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Pediatric liver transplantation (PLT) is a life-saving procedure for children with end-stage liver disease. However, post-transplant monitoring, particularly the diagnosis and prognosis of complications such as allograft fibrosis, remains challenging. Traditionally, liver biopsy has been the gold standard for assessing allograft fibrosis, despite its invasive nature and inherent procedural risks. Recently, shear wave ultrasound elastography (SWE) has seen increasing use as a noninvasive method to provide real-time, quantitative measurements of liver stiffness as a proxy for fibrosis. In this review, the latest developments in the use of SWE as a method to assess allograft fibrosis post-PLT are presented, including the current technical challenges associated with its clinical implementation., (© 2024 Wiley Periodicals LLC.)

Background: Gerascophobia, or excessive fear of aging, is thought to be caused by a mixture of cognitive, experiential, and physiological factors acting on a person at particular time points. Measurement tools for evaluating geraschophobia remain insufficiently developed, despite this commonplace fear's distress., Objective: The objective of the current study was to develop and validate the Gerascophobia or Excessive Fear of Aging Scale (GEFAS) and analyze its psychometric properties., Methods: Three successive investigations were carried out with a total of 1594 participants. Exploratory and confirmatory factor analyses were conducted to evaluate the construct validity of the GEFAS. Convergent and discriminant validity was assessed by examining relationships with measures of depression, anxiety, stress, death anxiety, psychosocial illness, and life satisfaction. Multiple linear regression was used to investigate factors that predict fear of aging., Results: The GEFAS demonstrated high reliability (Cronbach alpha >0.8). Factor analysis supported a single-factor solution for the scale (fitness indices CFI, TLI, NNFI, NFI, RFI, and MFI all > 0.95; RMSEA=0.06. Significant positive correlations were found between fear of aging and depression (r = 0.270; p < 0.01), anxiety (r = 0.311; p < 0.01), stress (r = 0.285; p < 0.01), death anxiety (r = 0.600; p < 0.01), and psychosocial illness (r = 0.243; p < 0.01). A significant inverse correlation was observed with life satisfaction. Gender differences were also noted, with women exhibiting greater fear of aging than men (p < 0.01; d = 0.488)., Conclusion: The GEFAS fills a significant gap in the psychological/psychiatric literature as a useful tool for evaluating the fear of aging. The study concludes that the excessive fear of aging contributes to poor mental health., Competing Interests: Declaration of competing interest None., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Trichohepatoenteric syndrome (THES) is a rare genetic disorder inherited in an autosomal recessive manner. THES primarily leads to neonatal enteropathy, typically manifesting as severe, persistent diarrhea, distinctive facial features such as frontal bossing and a broad flat nasal bridge, woolly and fragile hair, immunodeficiency resulting in recurrent infections, failure to thrive (FTT), and liver complications including fibrosis or cirrhosis. This multisystem disorder is linked to mutations in the tetratricopeptide repeat domain 37 (TTC37) gene, also known as superkiller complex (SKIC) protein 3, responsible for THES type 1, and the Ski2-like ribonucleic acid (RNA) helicase (SKIV2L) gene, also known as SKIC2, responsible for THES type 2. This case report describes two far-related pediatric patients from Bahrain diagnosed with THES type 1. Although both patients exhibited typical symptoms of this syndrome, the first patient had more severe symptoms. Diagnostic genetic evaluations confirmed THES. Patient 1 had three homozygous variants in the TTC37 gene, while Patient 2 had two variants in the same gene. Both of our patients had a novel homozygous variant. The treatment focused on supportive care and infection management. Despite these efforts, patients did not reach their growth potential. This report underscores the necessity for early identification of THES to facilitate appropriate management and genetic counseling., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. The Research and Research Ethics Committee issued approval 21-140224. This study was conducted in accordance with the principles of Helsinki declaration, and it was ethically approved by the Research and Research Ethics Committee, Salmaniya Medical Complex, Government Hospitals, Kingdom of Bahrain (IRB number: 21-140224, approval date: February 14, 2024). Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Isa et al.)

Vanishing white matter (VWM) disease is an autosomal recessive disorder caused by mutations in the gene EIF2B encoding the subunits 1-5 of eukaryotic initiation factor 2B. Although rare, with a reported prevalence of 1:80,000 (0.001%), it was considered as one of the most common leukodystrophies. However, the worldwide incidence and prevalence of this disease are not clear. In Bahrain, of 21 patients who were diagnosed with leukodystrophy, two patients were found to have VWM disease accounting for 9.5%. Vaccinations and infections were the trigger factors for this disease to manifest. Rapid neurological deterioration, loss of developmental milestones, and seizure disorders are the main presentations in both patients. Magnetic resonance imaging (MRI) showed the classical radiological changes of demyelination and leukodystrophy. Patient 1 had associated ulcerative colitis, a finding that was not reported before. Patient 1's condition progressed to a vegetative stage, while patient 2 passed away, reflecting the poor disease outcome. In patient 2, a novel homozygous missense mutation was found in the EIF2B3 gene (c.25G>A, p.Ala9Thr). In this report, we present in detail the prevalence of VWM disease among cases with leukodystrophy, patients' characteristics, clinical presentations, radiological findings, associated diseases, genetic results, and clinical outcomes in the main tertiary hospital in Bahrain between 1998 and 2024. Moreover, we conducted a thorough literature review on this rare condition., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. The Research and Research Ethics Committee, Salmaniya Medical Complex, Government Hospitals, Kingdom of Bahrain issued approval IRB number: 148221121, dated November 22, 2021. This study was conducted in accordance with the Declaration of Helsinki and was ethically approved by the Research and Research Ethics Committee, Salmaniya Medical Complex, Government Hospitals, Kingdom of Bahrain. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Alsahlawi et al.)

Introduction Children with cystic fibrosis (CF) have lipid maldigestion due to pancreatic insufficiency, which causes malabsorption of fat-soluble vitamins. The primary objective of this study was to assess the prevalence of vitamin E deficiency among children with CF. The secondary objective was to examine the correlation between vitamin E levels with demographic data, laboratory findings, and the number of pulmonary exacerbations. Furthermore, the study aimed to identify potential predictors of vitamin E deficiency in this population. Methods A prospective cohort study was conducted from July 1, 2017, to April 30, 2019. Medical records of children diagnosed with CF at the Department of Pediatrics, Salmaniya Medical Complex, Bahrain were reviewed. Patients who didn't receive fat-soluble vitamin supplementation for at least three days were recruited for the study. Light-protected blood samples were tested for vitamin E and D levels and fasting serum cholesterol levels. Patients with vitamin E deficiency were compared with those without regarding demography, laboratory results, and number of pulmonary exacerbations. Results Of 109 patients with CF, 35 (32.1%) fulfilled the inclusion criteria. Eighteen (51.4%) were males. The mean age was 6.8 ± 4.7 years. Eleven (31.4%) patients were symptomatic. Vitamin E and D were deficient in nine (25.7%) and 28/34 (82.4%) patients, respectively. Cholesterol was low in 29 (82.9%). The mean vitamin E level in the deficient group was significantly lower (3.2 ± 1.7 mg/L) than that (10.3 ± 3.1 mg/L) of the vitamin E-sufficient group (P < 0.0001). A significant negative correlation was noted between vitamin E levels and white blood cell (WBC) count (r = -0.408; P = 0.015). However, no correlation was found between vitamin E levels and cholesterol, vitamin D levels, or the number of pulmonary exacerbations. Vitamin E-deficient patients had lower weight at presentation (P = 0.045), hemoglobin level (P = 0.001), and salbutamol use (P = 0.022), but higher reticulocyte percentage (P = 0.034) and WBC count (P = 0.001) compared to the vitamin E-sufficient group. Conclusion Vitamin E deficiency is common among patients with CF in Bahrain and may increase the risk of hemolytic anemia. This deficiency did not seem to affect the frequency of pulmonary exacerbations. Management of vitamin E deficiency in patients with CF should be hastened to avoid irreversible complications., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. The Research Committee for Government Hospitals, Salmaniya Medical Complex, Kingdom of Bahrain issued approval 56230522. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Isa et al.)

Introduction Wilson disease (WD) is a rare inherited autosomal recessive disorder caused by a mutation in the ATP7B gene. This mutation affects copper metabolism, leading to the accumulation of copper in the liver, brain, cornea, and other tissues. If not treated, WD can lead to significant morbidities. This study aimed to display the prevalence, clinical presentations, diagnosis, treatment, and outcome of WD in Bahrain. Methods This is a retrospective cohort study of patients diagnosed with WD in the Department of Pediatrics, Salmaniya Medical Complex, Manama, Kingdom of Bahrain, from March 2002 to July 2024. The diagnosis of WD was based on clinical presentations, laboratory markers, radiological imaging, and genetic testing. Results Up to July 2024, eight patients from four families were diagnosed with WD in Bahrain. Accordingly, the prevalence of WD was 1.7 patients per 100,000 (0.002%). Parental consanguinity was noted in all families. Males were equal to females (n=4, 50% each). The mean age at presentation was 13 ± 3.6 years, ranging from 9 to 21 years. Three patients (37.5%) presented with hepatic manifestations, two (25%) with neurological manifestations, and three (37.5%) were detected upon family screening. Kayser Fleischer (KF) rings were found in two (25%) patients via slit lamp examination. Of the seven patients with available data, five (71.4%) had low serum copper, six (85.7%) had low serum ceruloplasmin, and six (85.7%) had high 24-hour urinary copper. Genetic testing was positive for the  ATP7B  gene variants in all patients, of which four members of one family had a novel mutation. Abdominal ultrasound revealed hepatic cirrhosis in four (50%) patients, increased hepatic echogenicity in five (62.5%), splenomegaly in three (37.5%) patients, and ascites in one (12.5%) patient. Brain magnetic resonance imaging (MRI) was positive in three (50%) out of six patients. All patients received copper chelating therapy. Six (75%) patients survived; one of them underwent living-related liver transplantation due to rapidly progressive neurological symptoms. Two (25%) patients died due to hepatic failure and encephalopathy. Conclusion WD is a rare disorder in Bahrain. This study presents eight patients with WD from four families. Five patients were symptomatic at presentation, while three were diagnosed upon genetic family screening. Hepatic manifestations were the most common presentation, followed by behavioral changes and neurological symptoms. Two patients had KF rings, while three had classical brain MRI findings. Genetic testing served as a confirmatory diagnostic tool that revealed a diversity of variants causing the disease, of which one variant was a novel mutation. Despite oral chelating therapy, morbidity and mortality remain high in patients with WD., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. The Research and Research Ethics Committee, Government Hospitals, Manama, Kingdom of Bahrain. issued approval 11140223, 14th February 2023. This study was conducted in accordance with the principles of Helsinki Declaration. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Isa et al.)

Introduction Detection of gynecological cancers preoperatively is imperative for practitioners for optimal patient management and outcome. This study aimed to estimate the incidence of unexpected malignancy (UM) in patients who underwent hysterectomy or myomectomy for presumed benign indications and to detect the predictive factors of UM. Methods A retrospective analytical study that included patients who underwent hysterectomy or myomectomy for benign indications from January 1st, 2016, to December 31st, 2020, was conducted at the Department of Obstetrics and Gynecology at Salmaniya Medical Complex, Bahrain. The main outcome was the overall incidence of UM and the incidence of each malignancy. Characteristics of UM were compared with benign pathologies. Fisher's exact and Pearson's chi-square tests were used to compare categorical variables and the Mann-Whitney U test or student's t-test for continuous variables. Binary logistic regression was used to identify the predictors of occurrence of UM. Confidence interval (CI) was set at 95%. A probability value (p-value) less than 0.05 was considered statistically significant. Results Out of 513 patients who underwent hysterectomy or myomectomy, 379 (73.9%) fulfilled the inclusion criteria, 314 (82.8%) hysterectomies and 65 (17.2%) myomectomies. The overall incidence of UM was 1.3% (n=5/379), 1.3% (n=4/314) among hysterectomies and 1.5% (n=1/65) among myomectomies. Three (0.8%) pre-malignant pathologies were identified: one (0.26%) smooth muscle tumor of unknown malignant potential, leiomyoma with bizarre nuclei, and mucinous borderline tumor of endocervical type of ovary each. The types of UM were sarcomas in three (0.26%) patients (two (0.5%) leiomyosarcoma and one (0.26%) endometrial stromal sarcoma) and endometrial adenocarcinoma and ovarian cancer in one (0.26%) patient each. No significant difference was found between the characteristics of UM and benign pathologies. Conclusion Although this study demonstrated a low incidence of UM among both hysterectomies and myomectomies, the age at the diagnosis of our patients with UM was as young as 34 years of age, and sarcomas were the most common type of UM. Disconcertingly, none of the studied independent variables had significantly predicted the occurrence of UM., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Research and Research Ethics committee, Salmaniya Medical Complex, Government Hospitals issued approval 2100123. This study conforms to the provisions of the Declaration of Helsinki in 1995 (revised in Edinburgh 2000) and was approved by the Research and Research Ethics Committee, Salmaniya Medical Complex, Government Hospitals (Institutional Review Board number: 2100123). Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Sabt et al.)

Cowden syndrome (CS) is a rare autosomal dominant genodermatosis disorder. This disease is characterized by the development of several hamartomata lesions in a variety of tissues from all three embryonic layers. The most well-known hamartomata are those of the gastrointestinal system, which represent one of the major criteria for the diagnosis of CS. Yet, the most frequent initial presenting symptom of the disease is thought to be mucocutaneous symptoms such as trichilemmomas, acral keratosis, and oral papilloma. Early diagnosis and management are essential to improving the quality of life for patients with CS as this disorder predisposes them to cancers such as thyroid, breast, gastrointestinal, and endometrial cancers. This report presents a rare case of CS in a Bahraini child who presented with macrocephaly and had numerous intestinal polyposis. Genetic testing using whole exome sequencing confirmed the diagnosis, identifying a pathogenic de novo phosphatase and tensin homolog gene ( PTEN ) variant (Chr10 NM&#95;000314.8: c.17&#95;18del p.(Lys6Argfs*4)) in a heterozygous state. This variant has been confirmed by Sanger sequencing., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Research and Research Ethics Committee, Goverment Hospitals, Manama, Bahrain issued approval 66-050624. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Isa et al.)

Background: Non-alcoholic fatty liver disease (NAFLD) is highly prevalent in children and adolescents, particularly those with obesity. NAFLD is considered a hepatic manifestation of the metabolic syndrome due to its close associations with abdominal obesity, insulin resistance, and atherogenic dyslipidemia. Experts have proposed an alternative terminology, metabolic dysfunction-associated fatty liver disease (MAFLD), to better reflect its pathophysiology. This study aimed to develop consensus statements and recommendations for pediatric MAFLD through collaboration among international experts., Methods: A group of 65 experts from 35 countries and six continents, including pediatricians, hepatologists, and endocrinologists, participated in a consensus development process. The process encompassed various aspects of pediatric MAFLD, including epidemiology, mechanisms, screening, and management., Findings: In round 1, we received 65 surveys from 35 countries and analyzed these results, which informed us that 73.3% of respondents agreed with 20 draft statements while 23.8% agreed somewhat. The mean percentage of agreement or somewhat agreement increased to 80.85% and 15.75%, respectively, in round 2. The final statements covered a wide range of topics related to epidemiology, pathophysiology, and strategies for screening and managing pediatric MAFLD., Conclusions: The consensus statements and recommendations developed by an international expert panel serve to optimize clinical outcomes and improve the quality of life for children and adolescents with MAFLD. These findings emphasize the need for standardized approaches in diagnosing and treating pediatric MAFLD., Funding: This work was funded by the National Natural Science Foundation of China (82070588, 82370577), the National Key R&D Program of China (2023YFA1800801), National High Level Hospital Clinical Research Funding (2022-PUMCH-C-014), the Wuxi Taihu Talent Plan (DJTD202106), and the Medical Key Discipline Program of Wuxi Health Commission (ZDXK2021007)., Competing Interests: Declaration of interests M.-H.Z. has received honoraria for lectures from AstraZeneca, Hisky Medical Technologies, and Novo Nordisk and consulting fees from Boehringer Ingelheim and serves as a consultant for Eieling Technology. L.A.B. has received speaker fees from Novo Nordisk and is on the Steering Committee of ACTION Teens, a multicountry study of adolescent obesity sponsored by Novo Nordisk. All fees received are directed to L.A.B.’s hospital research cost center. M.K. is a consultant for Mirum and Medison Pharma. A.M. has received honoraria from AstraZeneca, Boehringer Ingelheim, Novo Nordisk, and Janssen. N.B.H.N. has received honoraria for lectures and the role of advisory board member (Adolescents with Obesity) from Novo Nordisk. C.M. is chairman of the board and shareholder of DeFaire Medical AB, scientific advisor and shareholder of Oriflame Wellness AB, scientific advisor of Itrim AB, scientific advisor of and speaker for Novo Nordisk, scientific advisor of Rhythm, and speaker for Astra Zeneca, Semper, and Redeye. C.D.B. has received an independent research grant from Echosens. C.G. has acted as speaker for Merck, Gilead, Abbvie, and Novo Nordisk; has served as an advisory board member for Merck, Novo Nordisk, and Gilead; and has received unrestricted research funding from Theratechnologies Inc. M.T.A.-L. has obtained a grant from Novo Nordisk and has acted in an advisory capacity for MSD and Novo Nordisk and as speaker for Novo Nordisk, Gilead, and Abbie. J.M.P. has received consulting fees from Boehringer Ingelheim, MSD, and Novo Nordisk; speaking fees from Gilead, Intercept, and Novo Nordisk; travel expenses from Gilead, Rubió, Pfizer, Astellas, MSD, CUBICIN, and Novo Nordisk; educational and research support from Madrigal, Gilead, Pfizer, Astellas, Accelerate, Novartis, Abbvie, ViiV, and MSD; and funds from European Commission/EFPIA IMI2 853966-2, IMI2 777377, H2020 847989, and ISCIII PI19/01898. J.-G.F. has received honoraria for lectures from Sanofi, Echosens, Abbott, Novartis, AstraZeneca, Hisky Medical Technologies, and Novo Nordisk; consulting fees from Allergan, Echosens, Novartis, and Terns; and research grants from Sanofi. S.S.S. serves on Mirum and Alberio advisory boards., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Cyclin-dependent kinase 13 (CDK13)-related disorder is a rare autosomal dominant disease caused by pathogenic variants in the CDK13 gene. This disorder was found to be related to several clinical features, including structural cardiac anomalies, developmental delay, anomalies of the corpus callosum, and a variety of facial dysmorphisms. In addition, feeding difficulties and neonatal hypotonia might also present. The diagnosis of this disorder is based on molecular genetic testing to detect the causative pathogenic variants. Here, we report a case of a one-year-old girl from Yemen, residing in Bahrain, with a CDK13-related disorder who was found to have an unusual association of abdominal situs inversus along with multiple structural cardiac anomalies, including atrial septal defect, ventricular septal defect, patent ductus arteriosus, interrupted inferior vena cava, bilateral superior vena cava, mild coarctation of the aorta, dilated coronary sinuses, and mild regurgitation in the tricuspid valve. Moreover, facial dysmorphism including medial epicanthal folds, posteriorly rotated ears, and a depressed nasal bridge was also noted. Further assessment showed a delay in reaching developmental milestones, including speech and motor delay. The patient also presented with recurrent episodes of upper respiratory tract infections, acute bronchiolitis, and lobar pneumonia which required admission to the intensive care unit and ventilation. The last infection episode was at the age of one year. Thereafter, the patient underwent cardiac repair of the ventricular septal defect followed by no more infection episodes until the age of one year and two months. The diagnosis of CDK13 was confirmed by a whole exome sequencing test which demonstrated a novel missense variant in exon 14 of the CDK13 gene as a variant of uncertain significance in a heterozygous state., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Isa et al.)

Background: Inflammatory bowel disease (IBD) is a chronic gastrointestinal disease that causes anorexia, malabsorption, and increased energy requirements. Childhood IBD can significantly impact nutritional status and future health., Objective: This study aimed to analyze the nutritional status of patients with pediatric IBD at presentation and during follow-up and to identify predictors of nutritional outcome., Methods: This retrospective cohort study reviewed the medical records of children diagnosed with IBD in the Pediatric Department, Salmaniya Medical Complex, Bahrain, 1984 - 2023. Demographic data, clinical characteristics, and anthropometric data were collected. World Health Organization growth standards were used to interpret nutritional status., Results: Of the 165 patients, 99 (60%) had anthropometric data at presentation, and 130 (78.8%) had follow-up data. Most patients were males (64.6%) and had Crohn's disease (CD) (56.2%), while 43.8% had ulcerative colitis (UC). The median age at presentation was 10.9 years and the mean follow-up duration was 12.6 years. At presentation, 53.5% of the patients were malnourished, that decreased to 46.9% on follow-up. Thinness was reduced from 27.3% at presentation to 12.1% at follow-up (p = 0.003). There was an increased tendency to normal weight on follow-up (59.6%) compared to time of presentation (46.5%), p = 0.035. Overweightness showed a non-significant increase from 26.3% at presentation to 28.3% at follow-up (p = 0.791). Children with IBD were more likely to become obese when they grow up to adulthood (2.3% versus 20.5%, respectively, p < 0.001). Weight-for-age, and height-for-age at presentation were higher among CD compared to UC, but body mass index (BMI) at follow-up was higher among UC patients (p < 0.05). Thinness at follow up was associated with very early-onset disease (p = 0.02), lower weight and BMI at presentation (p < 0.001 each), younger age at follow-up (p = 0.002), pediatric age group (p = 0.023), lower hematocrit (p = 0.017), and higher C-reactive protein (p = 0.007). Overweight at follow up was associated with increased weight and BMI at presentation (p < 0.001 each), longer disease duration (p = 0.005), older age (p = 0.002), and azathioprine intake (p = 0.026). Considering follow-up duration, univariate analysis exhibited that Bahraini nationality, post-diagnosis disease duration, age at follow-up, occurrence of diarrhea, height, and BMI at presentation were factors that decreased liability to abnormal nutritional status, while CD, history of weight loss, perianal disease, and skin rash, and intake of prednisolone expressed increased liability of abnormal nutritional status (p < 0.05)., Conclusion: Pediatric IBD is associated with a high incidence of malnutrition. Thinness is more prominent at presentation, while overweight is higher on follow-up. Multiple risk factors aggravating abnormal nutritional status were highlighted. Accordingly, nutritional counseling should be prioritized in a multidisciplinary approach., (© 2024. The Author(s).)

Background: Liver transplantation (LT) is a life-saving procedure for patients with end-stage liver disease and has become the standard and most effective treatment method for these patients. There are many indications for LT that vary between countries and settings. The outcome of LT depends on the available facilities and surgical expertise, as well as the types of liver graft donors available., Aim: To assess the clinical characteristics of patients from Bahrain who underwent LT overseas, and analyze factors affecting their survival., Methods: In this retrospective cohort study, we reviewed the medical records and overseas committee registry information of all pediatric and adult patients who were sent overseas to undergo LT by the Pediatric and Medical Departments of Salmaniya Medical Complex and Bahrain Defence Force Hospital via the Overseas Treatment Office, Ministry of Health, Kingdom of Bahrain, between 1997 and 2023. Demo graphic data, LT indication, donor-recipient relationship, overseas LT center, graft type, post-LT medications, and LT complications, were collected. Outcomes measured included the overall and 5-year LT survival rate. Fisher's exact, Pearson χ 2 , and Mann-Whitney U tests were used to compare the pediatric and the adults' group in terms of clinical characteristics, donor-recipient relationship, medication, complications, and outcome. Survival analysis was estimated via the Kaplan-Meier's method. Univariate and multivariate analyses were used to detect predictors of survival., Results: Of the 208 eligible patients, 170 (81.7%) were sent overseas to undergo LT while 38 (18.3%) remained on the waiting list. Of the 170 patients, 167 (80.3%) underwent LT and were included in the study. The majority of the patients were Bahraini (91.0%), and most were males (57.5%). One-hundred-and-twenty (71.8%) were adults and 47 (28.3%) were children. The median age at transplant was 50.0 [interquartile range (IQR): 14.9-58.4] years. The main indication for pediatric LT was biliary atresia (31.9%), while that of adult LT was hepatitis C-related cirrhosis (35.0%). Six (3.6%) patients required re-transplantation. Most patients received a living-related liver graft (82%). Pediatric patients received more living and related grafts than adults ( P = 0.038 and P = 0.041, respectively), while adult patients received more cadaveric and unrelated grafts. Most patients required long-term immunosuppressive therapy after LT (94.7%), of which tacrolimus was the most prescribed (84.0%), followed by prednisolone (50.7%), which was prescribed more frequently for pediatric patients ( P = 0.001). Most patients developed complications (62.4%) with infectious episodes being the most common (38.9%), followed by biliary stricture (19.5%). Tonsilitis and sepsis ( n = 12, 8.1% for each) were the most frequent infections. Pediatric patients experienced higher rates of infection, rejection, and early poor graft function than adult patients ( P < 0.001, P = 0.003, and P = 0.025, respectively). The median follow-up time was 6.5 (IQR: 2.6-10.6) years. The overall survival rate was 84.4%, the 5-year survival rate, 86.2%, and the mortality rate, 15.6%. Younger patients had significantly better odds of survival ( P = 0.019) and patients who survived had significantly longer follow-up periods ( P < 0.001)., Conclusion: Patients with end-stage liver disease in Bahrain shared characteristics with those from other countries. Since LT facilities are not available, an overseas LT has offered them great hope., Competing Interests: Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article., (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)

Johanson-Blizzard syndrome (JBS) is a rare hereditary autosomal recessive disorder caused by a mutation in the ubiquitin protein ligase E3 component n-recognin 1 (UBR1) gene. This syndrome is characterized by the following typical clinical features: hypoplasia or aplasia of the alae nasi, congenital scalp defects, sensorineural hearing loss, hypothyroidism, growth retardation, psychomotor retardation, imperforate anus, genitourinary anomalies, and atypical hair patterns. Here, we describe a case of a 12-year-old girl with JBS of consanguineous parents. During the last trimester of pregnancy, a congenital abnormality affecting the nose was detected. Immediately after birth, the clinical examination revealed dysmorphic features in the form of hypoplastic alae nasi, microcephaly, mild hypotelorism, and cutis aplasia on the scalp. The genetic testing of the patient showed a novel sequence change mutation of the UBR1 gene (1bp duplication causing a frameshift), while both parents were carriers for this mutation. Moreover, a diagnosis of pancreatic insufficiency and subclinical hypothyroidism was made based on clinical presentation and laboratory results. The patient was started on pancreatic enzyme replacement therapy and fat-soluble vitamins, minerals, and antioxidant syrup. Further assessment revealed hypotonia, growth impairment, delay in reaching developmental milestones, and bilateral profound sensorineural hearing loss, which was managed with bilateral cochlear implantation. In addition, the patient underwent multiple craniofacial reconstructive surgeries. This case report highlights the importance of early diagnosis and multidisciplinary care of patients with JBS., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Isa et al.)

Background: Fever is a common cause of medical consultation and hospital admission, particularly among children. Recently, the United Kingdom's National Institute for Health and Care Excellence (NICE) updated its guidelines for assessing fever in children under five years of age. The efficient assessment and management of children with fever are crucial for improving patient outcomes., Aim: To evaluate fever assessment in hospitalized children and to assess its adherence with the NICE Fever in under 5s guideline., Methods: We conducted a retrospective cohort review of the electronic medical records of children under five years of age at the Department of Pediatrics, Salmaniya Medical Complex, Bahrain, between June and July 2023. Demographic data, vital signs during the first 48 h of admission, route of temperature measurement, and indications for admission were gathered. Fever was defined according to the NICE guideline. The children were divided into five groups according to their age (0-3 months, > 3-6 months, > 6-12 months, > 12-36 months, and > 36-60 months). Patients with and without fever were compared in terms of demography, indication for admission, route of temperature measurement, and other vital signs. Compliance with the NICE Fever in the under 5s guideline was assessed. Full compliance was defined as > 95%, partial compliance as 70%-95%, and minimal compliance as ≤ 69%. Pearson's χ 2 , Student's t test, the Mann-Whitney U test, and Spearman's correlation coefficient (r s ) were used for comparison., Results: Of the 136 patients reviewed, 80 (58.8%) were boys. The median age at admission was 14.2 [interquartile range (IQR): 1.7-44.4] months, with the most common age group being 36-60 months. Thirty-six (26.4%) patients had fever, and 100 (73.6%) were afebrile. The commonest age group for febrile patients (> 12-36 months) was older than the commonest age group for afebrile patients (0-3 months) ( P = 0.027). The median weight was 8.3 (IQR: 4.0-13.3) kg. Patients with fever had higher weight than those without fever [10.2 (IQR: 7.3-13.0) vs 7.1 (IQR: 3.8-13.3) kg, respectively] ( P = 0.034). Gastrointestinal disease was the leading indication for hospital admission ( n = 47, 34.6%). Patients with central nervous system diseases and fever of unknown etiology were more likely to be febrile ( P = 0.030 and P = 0.011, respectively). The mean heart rate was higher in the febrile group than the afebrile group (140 ± 24 vs 126 ± 20 beats per minute, respectively) [ P = 0.001 (confidence interval: 5.8-21.9)] with a positive correlation between body temperature and heart rate, r = 0.242, n = 136, P = 0.004. A higher proportion of febrile patients received paracetamol ( n = 35, 81.3%) compared to the afebrile patients ( n = 8, 18.6%) ( P < 0.001). The axillary route was the most commonly used for temperature measurements ( n = 40/42, 95.2%), followed by the rectal route ( n = 2/42, 4.8%). The department demonstrated full compliance with the NICE guideline for five criteria: the type of thermometer used, route and frequency of temperature measurement, frequency of heart rate measurement, and use of antipyretics as needed. Partial compliance was noted for two criteria, the threshold of fever at 38 °C or more, and the respiratory rate assessment in febrile patients. Minimal compliance or no record was observed for the remaining three criteria; routine assessment of capillary refill, temperature reassessment 1-2 h after each antipyretic intake, and refraining from the use of tepid sponging., Conclusion: This study showed that fever assessment in hospitalized children under five years of age was appropriate, but certain areas of adherence to the NICE guideline still need to be improved., Competing Interests: Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article., (©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.)

Infantile-onset multisystem neurologic, endocrine, and pancreatic disease (IMNEPD) is a rare autosomal recessive multisystemic disease with a prevalence of < 1/1 000 000. The wide spectrum of symptoms and associated diseases makes the diagnosis of this disease particularly challenging. Here, we report a 12-year-old Bahraini male who presented with the core clinical features of IMNEPD including intellectual disability, global developmental delay, sensorineural hearing loss, endocrine dysfunction, and exocrine pancreatic insufficiency. The diagnosis was confirmed by genetic testing using whole exome sequencing. This is the first reported case of IMNEPD from Bahrain and was found to have a novel homozygous peptidyl-tRNA hydrolase 2 (PTRH2) gene mutation (NM&#95;001015509.2: c.370del p.(Glu124Lysfs*4)). Moreover, we conducted an extensive literature review with an emphasis on the variable clinical spectrum and genotypes of previously reported patients in comparison to our case., (Copyright © 2023, Oman Medical Journal.)

Background: Incidence of body contouring surgeries (BCS) rose significantly to overcome problems resulted from post-Bariatric Surgery (BS). We aimed to evaluate satisfaction level and quality of life (QOL) in patients' post-BCS., Methods: In this retrospective prospective study, patients who underwent BCS in Plastic Surgery Department, Salmaniya Medical Complex, Bahrain, in 2017-2018, were enrolled. Demographic and anthropometric data were collected. BS-group's QOL and satisfaction level were assessed using a questionnaire., Results: Of 929 plastic surgery admissions, 316 (34%) were for BCS (249 patients). Fifty-eight (28%) patients underwent 82 BS were recruited, mostly females (n=42, 72.4%). The mean age was 37.4±9.6 years. Excess abdominal skin was the most area of concern (n=50, 86.2%). Median pre-BCS body mass index was 26.9 (interquartile range: 25.6-29.8) kg/m 2 . Most patients were overweight (n=26, 44.8%). Abdominoplasty was the commonest BCS (n=172, 50.6%). This was also the case in 82 BCS in post-BS group (n=38, 46.3%). In post-BS group, post-operative complications were noted in 25/82 (30.5%) patients with wound problems being the most frequent (n=14, 17.1%). Most patients rated their experience as better in all questionnaire domains and most (n=45, 54.9%) rated their satisfaction level as excellent. Older age gave better overall satisfaction ( P <0.001) while employed patients had better overall QOL ( P =0.012) and self-confidence ( P =0.048). Females had better satisfaction with body appearance ( P <0.001) while those underwent abdominoplasty or breast surgeries had lower physical activity ( P =0.042)., Conclusion: This study showed improvement in patient's QOL post-BCS with excellent overall satisfaction, findings that could be affected by age, sex, and occupation., Competing Interests: The authors declare that there is no conflict of interests.

Background Neuromuscular blocking agents (NMBAs) are employed during general anesthesia induction for endotracheal intubation and to facilitate specific surgeries requiring muscle relaxation. However, residual neuromuscular blockade (RNMB) can lead to respiratory complications in post-anesthesia care units (PACUs). This study investigates RNMB incidence in PACUs and its association with postoperative airway and respiratory issues. Methods A prospective observational study on patients undergoing general anesthesia with NMBAs was conducted at the Department of Anesthesia, Salmaniya Medical Complex, Bahrain, over six months (April to September 2023). Train-of-four (TOF) ratios were calculated using an acceleromyograph upon PACU arrival. Data on demographics, perioperative variables, and postoperative complications were recorded. Results Among 82 patients, 30 (36.6%) had RNMB upon PACU arrival. RNMB incidence declined: 17.1% at 10 minutes, 6.1% at 20 minutes, and 2.4% at 30 minutes, resolving by 40 minutes. Demographics and procedure duration showed no correlation with RNMB. Postoperative respiratory complications affected 23.2% of patients, notably higher in those with RNMB (p = 0.001). Among patients with TOF <90% at PACU arrival, 46.7% experienced complications compared to 9.6% with TOF ≥90% (p<0.001). Participants without RNMB had a significantly higher weight (p = 0.046). Airway support was required for 30% of patients, all with TOF <90% (p<0.001). Conclusion This study emphasizes the importance of assessing and monitoring neuromuscular function to detect and prevent RNMB in PACUs. RNMB presence correlated with an increased susceptibility to postoperative respiratory complications. Regular quantitative neuromuscular monitoring is advisable in clinical practice to proactively mitigate RNMB incidence and its complications., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Bucheery et al.)

Introduction: Since its emergence, the COVID-19 pandemic has had a radical effect on different aspects of health worldwide. The burden created by the COVID-19 pandemic on healthcare systems has also involved educational and academic activities among healthcare professionals., Objectives: This study aimed to explore the effect of the COVID-19 pandemic on the participation of doctors and nurses in educational and academic activities in Bahrain., Methods: A cross-sectional survey was conducted through an online questionnaire that was distributed among 204 healthcare providers (HCPs) in the Kingdom of Bahrain., Results: Out of 204 HCPs, 110 (53.9%) were doctors and 94 (46.1%) were nurses. A total of 154 responses were received (100 (64%) doctors and 54 (35.1%) nurses) with an overall response rate of 75.5%. Seventy-four (74%) doctors and 32 (59.2%) nurses stated that their overall academic activities had decreased since the start of the pandemic, yet this was not statistically significant (P=0.059). Unlike nurses, doctors attended more webinars 54 (54%) and online courses 47 (47%), compared to the time before the pandemic (P=0.022, P=0.014, respectively)., Conclusion: The COVID-19 pandemic had a negative impact on educational and academic activities among healthcare workers. However, it created an opportunity to expand the use of electronic and online methods in those activities., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Ali et al.)

Background: Several countries, including Bahrain, used wastewater surveillance for disease activity monitoring. This study aimed to determine the presence of SARS-CoV-2 in untreated wastewater and to correlate it with the disease spread., Methods: A retrospective review was conducted for all wastewater samples tested for SARS-CoV-2 in public health laboratories from November 2020 to October 2022. Samples were collected weekly between February and October 2022 from different areas across Bahrain. Real-time polymerase chain reaction was used to test for the presence of SARS-CoV-2 in wastewater, and the results were correlated with the number of COVID-19 cases in the same area., Results: Of 387 wastewater samples, 103 (26.6%) samples tested positive for SARS-CoV-2. In late 2020, of 42 samples collected initially, four (9.5%) samples tested positive for SARS-CoV-2 in the four locations that hosted COVID-19 isolation facilities. Between February and October 2022, 345 specimens of wastewater were tested, and 99 (28.7%) were positive. The highest detection rate was in February, June, and July (60%, 45%, and 43%, respectively), which corresponded to COVID-19 peaks during 2022, and the lowest detection rate was in August and September (11% and 0%, respectively), corresponding to the low number of COVID-19 cases., Conclusion: The detection rate of SARS-CoV-2 in wastewater samples from Bahrain was high and was significantly correlated with the number of reported COVID-19 cases. Wastewater surveillance can aid the existing surveillance system in monitoring SARS-CoV-2 spread., Competing Interests: No financial or non‐financial benefits have been received or will be received from any party directly or indirectly related to this article's subject., (© 2023 The Authors. Influenza and Other Respiratory Viruses published by John Wiley & Sons Ltd.)

Hyaline fibromatosis syndrome (HFS) is a rare autosomal recessive disorder characterized by the deposition of hyaline material in the skin, soft tissues, and bones. In this report, we discuss a case of a six-month-old male with HFS who presented with faltering growth, chronic diarrhea, multiple joint contractures, joint stiffness, hyperpigmented skin over bony prominences, gingival hypertrophy, patent foramen ovale, and symmetric periventricular hyperintensities on brain MRI. The diagnosis of HFS was confirmed by skin biopsy and genetic testing, which identified a homozygous mutation in the anthrax toxin receptor 2 (ANTXR2) gene. The patient was managed symptomatically with nutritional support, physiotherapy, analgesics, and regular dental care. He also received intralesional corticosteroid therapy, which significantly decreased the size of the skin nodules. His hyperpigmented skin and gingival hypertrophy remained stable, and the patent foramen ovale was managed conservatively. This case report highlights the importance of early diagnosis and management of HFS and the benefits of involving a multidisciplinary team to improve the quality of life of affected individuals., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Albusta et al.)

Background: Children like to discover their environment by putting substances in their mouths. This behavior puts them at risk of accidentally ingesting foreign bodies (FBs) or harmful materials, which can cause serious morbidities., Aim: To study the clinical characteristics, diagnosis, complications, management, and outcomes of accidental ingestion of FBs, caustics, and medications in children., Methods: We conducted a retrospective cohort study of all children admitted for accidental ingestion to the Department of Pediatrics, Salmaniya Medical Complex, Bahrain, between 2011 and 2021. Demographic data, type of FB/harmful material ingested, and investigations used for diagnosis and management were recorded. The patients were divided into three groups based on the type of ingested material (FBs, caustics, and medications). The three groups were compared based on patient demographics, socioeconomic status (SES), symptoms, ingestion scenario, endoscopic and surgical complications, management, and outcomes. The FB anatomical location was categorized as the esophagus, stomach, and bowel and compared with respect to symptoms. The Fisher's exact, Pearson's χ 2 , Mann-Whitney U , and Kruskal-Wallis tests were used for comparison., Results: A total of 161 accidental ingestion episodes were documented in 153 children. Most children were boys ( n = 85, 55.6%), with a median age of 2.8 (interquartile range: 1.8-4.4) years. Most participants ingested FBs ( n = 108, 70.6%), 31 (20.3%) ingested caustics, and the remaining 14 (9.2%) ingested medications. Patients with caustic ingestion were younger at the time of presentation ( P < 0.001) and were more symptomatic ( n = 26/31, 89.7%) than those who ingested medications ( n = 8/14, 57.1%) or FBs ( n = 52/108, 48.6%) ( P < 0.001). The caustic group had more vomiting ( P < 0.001) and coughing ( P = 0.029) than the other groups. Most FB ingestions were asymptomatic ( n = 55/108, 51.4%). In terms of FB location, most esophageal FBs were symptomatic ( n = 14/16, 87.5%), whereas most gastric ( n = 34/56, 60.7%) and intestinal FBs ( n = 19/32, 59.4%) were asymptomatic ( P = 0.002). Battery ingestion was the most common ( n = 49, 32%). Unsafe toys were the main source of batteries ( n = 22/43, 51.2%). Most episodes occurred while playing ( n = 49/131, 37.4%) or when they were unwitnessed ( n = 78, 57.4%). FBs were ingested more while playing ( P < 0.001), caustic ingestion was mainly due to unsafe storage ( P < 0.001), and medication ingestion was mostly due to a missing object ( P < 0.001). Girls ingested more jewelry items than boys ( P = 0.006). The stomach was the common location of FB lodgment, both radiologically ( n = 54/123, 43.9%) and endoscopically ( n = 31/91, 34%). Of 107/108 (99.1%) patients with FB ingestion, spontaneous passage was noted in 54 (35.5%), endoscopic removal in 46 (30.3%), laparotomy in 5 (3.3%) after magnet ingestion, and direct laryngoscopy in 2 (1.3%). Pharmacological therapy was required for 105 (70.9%) patients; 79/105 (75.2%) in the FB group, 22/29 (75.9%) in the caustic group, and 4/14 (28.8%) in the medication group ( P = 0.001). Omeprazole was the commonly used ( n = 58; 37.9%) and was used more in the caustic group ( n = 19/28, 67.9%) than in the other groups ( P = 0.001). Endoscopic and surgical complications were detected in 39/148 (26.4%) patients. The caustic group had more complications than the other groups ( P = 0.036). Gastrointestinal perforation developed in the FB group only ( n = 5, 3.4%) and was more with magnet ingestion ( n = 4) than with other FBs ( P < 0.001). In patients with FB ingestion, patients aged < 1 year ( P = 0.042), those with middle or low SES ( P = 0.028), and those with more symptoms at presentation ( P = 0.027) had more complications. Patients with complications had longer hospital stays ( P < 0.001) than those without., Conclusion: Accidental ingestion in children is a serious condition. Symptomatic infants from middle or low SES families have the highest morbidity. Prevention through parental education and government legislation is crucial., Competing Interests: Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article., (©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.)

Bile acid synthesis disorders (BASD) are a group of rare autosomal recessive disorders. Of the nine different versions, BASD type 4 is characterized by a gene mutation in alpha-methylacyl-CoA racemase (AMACR), which is located on chromosome 5p13. These disorders generally present with a normal gamma-glutamyl transferase with cholestasis, absence of pruritis, and malabsorption of fat, which can lead to fat-soluble vitamin deficiencies. In adulthood, patients usually develop neurological sequelae. Initial testing can be done through the measurement of urine metabolites; however, confirmation of the diagnosis is achieved through whole exome sequencing. Treatment involves supplementation of oral cholic acid and modification of diet. Only 23 patients with this disease have been described. Here, we report two cases of siblings from a family in Bahrain with a novel AMACR mutation and a unique association with autoimmune antibodies alongside a literature review., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Isa et al.)

Background: Chronic constipation is common among children worldwide. Constipation includes functional constipation (FC) and organic constipation (OC). The early recognition of the causes of childhood constipation and its subsequent complications is important., Purpose: This study aimed to evaluate the prevalence and causes of childhood constipation and compare the clinical characteristics, treatment, and outcomes of children with FC versus OC to identify the predictive factors., Methods: This retrospective cross-sectional study analyzed children with FC or OC diagnosed in pediatric gastroenterology clinics, Salmaniya Medical Complex, Bahrain, 2017-2021. The Rome IV criteria were used to define FC., Results: A total of 7,287 gastroenterology appointments were attended by 4,346 children during the study period. Of the 639 children (14.7%) with constipation, 616 (96.4%) were included in the study. Most patients had FC (n=511, 83%), whereas 17% (n=105) had OC. FC was more common in females than in males. Children with OC were younger (P<0.001) and had lower body weights (P<0.001), more stunted growth (P<0.001), and more associated diseases (P= 0.037) than those with FC. Enuresis was the most associated disease (n=21, 3.4%). Organic causes included neurological, allergic, endocrine, gastrointestinal, and genetic diseases. Allergies to cow milk protein were the most common (n=35, 5.7%). The presence of mucus in the stool was more common in OC than in FC (P=0.041), but no other symptoms or physical findings differed. A total of 587 patients (95.3%) received medication, among which lactulose was commonly prescribed (n=395, 64.1%). There were no intergroup differences in nationality, sex, body mass index, seasonal variation, laxative type, or treatment response. A good response was observed in 114 patients (90.5%)., Conclusion: Chronic constipation represented a significant proportion of outpatient gastroenterology visits. FC was the most common type. Young children with a low body weight, stunted growth, mucus in the stool, or associated diseases should be assessed for an underlying organic cause.

Background: Alagille syndrome (ALGS) is an autosomal dominant disease caused by JAG1 or NOTCH2 mutation. It is diagnosed by the presence of three out of five features: characteristic facies, posterior embryotoxon, peripheral pulmonary stenosis, vertebral defects, and interlobular bile duct paucity. This study aimed to review the prevalence, clinical presentations, diagnosis, treatment, and outcome of patients with ALGS., Materials and Methods: This is a retrospective review of patients with ALGS at the Pediatric Department, Salmaniya Medical Complex, Bahrain, between August 1994 and October 2022. The diagnosis was based on clinical, laboratory, radiological, histopathological, and genetic findings., Results: Five patients were found to have ALGS. The prevalence of ALGS in Bahrain was 1.04 patients per 100,000 (0.001%). Four were Bahraini and three were females. Median birth weight was 2.3 (2.3-2.5) kg. All patients presented at the time of birth with low birth weight, cholestatic jaundice, clay-colored stool, heart murmur, and dysmorphic facial features. All had congenital heart diseases, two had butterfly vertebrae, and one had posterior embryotoxon. All had elevated liver enzymes and normal abdominal ultrasound. Three had positive hepatobiliary iminodiacetic acid scan and one had bile duct paucity in liver biopsy. Three had intraoperative cholangiogram. Four were positive for JAG1 mutation. All received ursodeoxycholic acid and fat-soluble vitamins. Two required liver transplantation., Conclusion: ALGS is a rare disorder in Bahrain. Diagnosis is challenging as the disease can be associated with or misdiagnosed as biliary atresia. Patients with ALGS are at high risk of morbidity either by unnecessary intraoperative cholangiogram or unavoidable liver transplantation., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Advanced Biomedical Research.)

Background: Severe acute respiratory tract infection (SARI) is a major global health threat. This study aimed to examine risk factors associated with poor outcomes in patients with SARI., Methods: All patients who met World Health Organization's (WHO) SARI case definition and were admitted to Salmaniya Medical Complex from January 2018 to December 2021 were included. Epidemiological and virological data were obtained and analyzed., Results: Of 1159 patients with SARI included, 731 (63.1%) patients were below 50 years, and 357 (30.8%) tested positive for viral pathogens. The most prevalent virus was Flu-A ( n  = 134, 37.5%), SARS-CoV2 ( n  = 118, 33%), RSV ( n  = 51, 14.3%), Flu B ( n  = 49,13.7%), other viruses ( n  = 3, 0.8%), and combined infection ( n  = 2, 0.6%). Six hundred fifty-eight (56.8%) patients had comorbidities, mainly diabetes ( n  = 284, 43%) and heart disease ( n  = 217, 33%). 183 (16%) patients were admitted to ICU, 110 (9%) needed mechanical ventilation, and 80 (7%) patients died.The odds of ICU admission were higher for patients with hematological (OR 5.9, 95% CI 3.1-11.1) and lung diseases (OR 2.7, 95% CI 1.6-4.6). The odds of mechanical ventilation were higher among patients with lung disease (OR 3.1, 95% 1.7-5.5). The mortality odds were higher among patients above 50 (OR 2.4, 95% CI 1.4-4.1) and chronic kidney disease (OR 2.5, 95% CI 1.1-5.2)., Conclusions: Being 50 years or above or having kidney, lung, or heart diseases was associated with worse SARI outcomes. Efforts and actions in developing better strategies to vaccinate individuals at high risk and early diagnosis and treatment should help in reducing the burden of SARI., Competing Interests: No financial or non‐financial benefits have been received or will be received from any party directly or indirectly related to this article's subject., (© 2023 The Authors. Influenza and Other Respiratory Viruses published by John Wiley & Sons Ltd.)

Background: Gastrointestinal bleeding (GIB) is a serious health problem worldwide, particularly during childhood. This can be an alarming sign of an underlying disease. Gastrointestinal endoscopy (GIE) is a safe method for the diagnosis and treatment of GIB in most cases., Aim: To determine the incidence, clinical presentation, and outcomes of GIB in children in Bahrain over the last two decades., Methods: This was a retrospective cohort review of the medical records of children with GIB who underwent endoscopic procedures in the Pediatric Department at Salmaniya Medical Complex, Bahrain, between 1995 and 2022. Demographic data, clinical presentation, endoscopic findings, and clinical outcomes were recorded. GIB was classified into upper (UGIB) and lower (LGIB) GIB according to the site of bleeding. These were compared with respect to patients' sex, age, and nationality using the Fisher's exact, Pearson's χ 2 , or the Mann-Whitney U tests., Results: A total of 250 patients were included in this study. The median incidence was 2.6/100000 per year (interquartile range, 1.4-3.7) with a significantly increasing trend over the last two decades ( P < 0.0001). Most patients were males ( n = 144, 57.6%). The median age at diagnosis was 9 years (5-11). Ninety-eight (39.2%) patients required upper GIE alone, 41 (16.4%) required colonoscopy alone, and 111 (44.4%) required both. LGIB was more frequent ( n = 151, 60.4%) than UGIB ( n = 119, 47.6%). There were no significant differences in sex ( P = 0.710), age ( P = 0.185), or nationality ( P = 0.525) between the two groups. Abnormal endoscopic findings were detected in 226 (90.4%) patients. The common cause of LGIB was inflammatory bowel disease (IBD) ( n = 77, 30.8%). The common cause of UGIB was gastritis ( n = 70, 28%). IBD and undetermined cause for bleeding were higher in the 10-18 years group ( P = 0.026 and P = 0.017, respectively). Intestinal nodular lymphoid hyperplasia, foreign body ingestion, and esophageal varices were more common in the 0-4 years group ( P = 0.034, P < 0.0001, and P = 0.029, respectively). Ten (4%) patients underwent one or more therapeutic interventions. The median follow-up period was two years (0.5-3). No mortality was reported in this study., Conclusion: GIB in children is an alarming condition, whose significance is increasing. LGIB, commonly due to IBD, was more common than UGIB, commonly due to gastritis., Competing Interests: Conflict-of-interest statement: All the Authors have no conflict of interest related to the manuscript., (©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.)

Introduction Inflammatory bowel diseases (IBD) are chronic diseases that can affect nutrient absorption leading to micronutrient deficiencies and biochemical abnormalities.This study aimed to assess certain serum micronutrients and nutritionally related biochemical markers levels in patients with pediatric IBD and to compare the actual levels and the prevalence of micronutrients deficiencies and biochemical abnormalities between patients with Crohn's disease (CD) and those with ulcerative colitis (UC). Methods A retrospective cross-sectional study reviewing medical records of patients with IBD was conducted in the pediatric department, Salmaniya medical complex, Bahrain, from 1 January 1984 to 31 December 2021. Demographic data and laboratory results related to micronutrients and biochemical markers including full blood count, total protein, albumin, globulin, iron, ferritin, folic acid, vitamin B12, calcium, phosphorous, magnesium, and vitamin D levels were collected upon presentation before starting the treatment. Nutritional deficiencies were compared based on sex, nationality, type of IBD, age at presentation, disease duration, weight at diagnosis, and inflammatory markers levels including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Results Of 157 patients with pediatric IBD, 117 (74.5%) were included. Sixty-six (56.4%) patients were males. Sixty-six (56.4%) had CD and 51 (43.6%) had UC. No patient had indeterminant colitis. The mean age at presentation was 10.8±3.8 years. Most patients had one or more micronutrient deficiencies (n=110, 94%). Anemia was a common finding (n=79/116, 68.1%), with iron deficiency anemia (IDA) being predominant. Low iron levels were noted in 64/77 (83.1%) patients with a median of 5.0 (2.0-9.3) µmol/L (normal range, 11.6-31.3); isolated iron deficiency (ID) in 11/18 (61.1%) and IDA in 53/59 (89.8%) patients. Vitamin D deficiency was the second most common (n=45/61, 73.8%). Serum albumin, ferritin, calcium, phosphorous, and magnesium were deficient in 29.2%, 27.8%, 31.7%, 12.5%, and 10%, respectively. One patient had vitamin B12 deficiency while none had folate deficiency. Patients with CD had significantly lower serum iron (5.4±5.6 versus 8.1±6.09 µmol//L, p=0.02) and lower serum protein (71.7±8.7 versus 75.4±9.9 g/L, p=0.043) but higher serum ferritin (45 (19-110.2) versus 21.3 (10.3-51.2) µg/L, p=0.046) compared to those with UC. Elevated ESR was noted in 62/105 (59.1%) patients while high CRP was found in 67/104 (64.4%). Patients with low iron had higher ESR (28 (17-47) versus 14 (10-33) mm/h, p=0.028) and higher CRP (13.3 (1.6-42) versus 1.8 (0.9-4.6) mg/L, p=0.019) levels compared to those with normal levels. Conclusion Patients with pediatric IBD are at risk of multiple micronutrient deficiencies and biochemical abnormalities. Iron and vitamin D deficiencies are the most frequent. Patients with CD are more prone to have lower serum iron and protein levels than those with UC. ID was associated with elevated inflammatory markers., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Isa et al.)

Objectives: Though the prevalence of biliary atresia (BA) in the Middle East is low, its role in causing life-threatening liver diseases is disproportionately high. This study aimed to assess the prevalence, clinical presentations, diagnosis, surgical interventions, and long-term outcomes including survival rate, and to analyze prognostic factors affecting the survival of infants with BA in Bahrain., Methods: A retrospective cohort review of cases of infants diagnosed with BA between January 1993 and December 2021 at Salmaniya Medical Complex, Bahrain, was conducted. Demographic, clinical, laboratory, imaging, and histopathological data were analyzed. Outcomes measured included 5-year native liver survival (NLS) and overall liver survival (OLS) rates., Results: Out of 27 infants diagnosed with BA, 26 were included in the study. The overall prevalence was 0.007% and was significantly higher among Bahraini (0.006%) compared to the non-Bahraini population (0.001%), p < 0.001. Most patients had clay-colored stool (19 of 22; 86.4%) and hepatomegaly (18; 81.8%). Kasai procedure was performed in 23 (88.5%) patients and primary liver transplant (LT) in three. Ten (38.5%) infants required LT after the Kasai procedure. The median age at diagnosis was 56 days with interquartile range (IQR) of 30-90, and the median age at the Kasai procedure was 61 days (IQR = 56-90). NLS and OLS rates were 53.8% and 65.4%, respectively. The overall mortality rate was 34.6%. Six of the 13 (46.2%) patients who were subjected to Kasai procedure alone died awaiting LT donors. Infants with lower birth weight and older age at diagnosis had significantly better odds of survival ( p < 0.029 and p < 0.044, respectively)., Conclusions: BA is uncommon among infants in Bahrain. Despite effective treatment options, late diagnosis can lead to significant morbidity and mortality. Our study found a relatively low prevalence of NLS (53.8%) and OLS (65.4%). Since the majority of the diagnosed patients had presented with clay-colored stools, implementing an infant stool card for screening may be beneficial in raising parental awareness and detecting BA early. Early detection and treatment using novel therapies are likely to improve the survival rates., (The OMJ is Published Bimonthly and Copyrighted 2023 by the OMSB.)

Acute liver failure (ALF) in children is a rare life-threatening condition. ALF is caused by different etiologies. The most common causes are drug-induced liver injury, infections, and metabolic diseases. Other rare causes of ALF are genetic disorders including spinocerebellar ataxia-21 (SCAR21). Herein, we describe the first Bahraini child who was diagnosed with a novel homozygous mutation in the SCYL1 gene. He was admitted to the hospital twice by the age of two and five years due to acute hepatic failure triggered by a febrile illness. Drug-induced, infectious causes, and metabolic diseases were excluded. The liver function then gradually recovered. The patient had delayed gross motor development as he started to walk at 20 months of age. After the first episode of ALF, he had progressive difficulty in walking leading to frequent falls and ending with a complete inability to walk. A whole-exome sequencing (WES) test revealed that the patient has previously unreported autosomal recessive pathogenic non-sense variation c.895A>T (p.Lys299Ter) in exon 7 of the SCYL1 gene in a homozygous status. It is confirmed that the pathogenicity of this variant in the SCYL1 gene was associated with SCAR21 disease., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Isa et al.)

Background: Inflammatory bowel disease (IBD) is a chronic inflammatory immune-mediated condition that affects the gastrointestinal system and alters bone growth and bone mineral density (BMD). Here we aimed to study the prevalence and predictors of a low BMD in pediatric patients with IBD., Methods: This retrospective cross-sectional analytical study included pediatric patients with IBD in whom BMD was evaluated using dual energy X-ray absorptiometry of the total body and lumbar spine. Osteoporosis was defined as a BMD Z-score ≤-2, osteopenia as -2 to -1, and normal as >-1. Clinical and laboratory findings were compared between patients with and without osteoporosis., Results: Of the 48 patients, 30 (62.5%) were males, 35 (72.9%) had Crohn's disease, and 13 (27.1%) had ulcerative colitis. The mean age at diagnosis was 9.9±2.8 years. The median age at the time of the BMD scans was 11.9 (interquartile range, 9.9-14.3) years. Total body BMD scans identified 13 (27.1%) and 16 (33.3%) patients with osteoporosis and osteopenia, respectively. Spinal BMD scans revealed that 17 (39.5%) and 14 (32.6%) patients had osteoporosis and osteopenia, respectively. A low body mass index (BMI) Z-score (p=0.038), ileocolonic disease location (p=0.008), and a low calcium level (p=0.008) were significant predictors of osteoporosis on the total body BMD scans. A low BMI Z-score (p=0.039), decreased hemoglobin level (p=0.018), low calcium level (p=0.033), and infliximab use (p=0.019) were significant predictors of osteoporosis on the spinal BMD scans., Conclusions: This study showed a high prevalence of low BMD among pediatric patients with IBD. A low BMI, ileocolonic disease location, low hemoglobin and calcium levels, and infliximab use were significantly associated with osteoporosis.

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive genetic disorder. It is caused by a defect in the action of the cilia lining multiple organs of the body, including the lungs, the sinuses, hepatobiliary and reproductive organs. In general, the estimated prevalence of this condition is one in 15,000-20,000 individuals. It is characterized by the triad of chronic sinusitis, bronchiectasis, and situs inversus, which occurs in 50% of the cases. It can be associated with other diseases too. Genetic studies can aid in confirming the diagnosis of this condition. A high degree of suspicion about PCD among pediatricians, neonatologists, otorhinolaryngologists, and pulmonologists is essential to make early referrals of patients before they develop irreversible lung damage. Hence, early diagnosis and appropriate treatment are very important. Multicenter collaborations might improve the quality of treatment and patient outcomes. Here, we discuss a case of PCD with a unique association with type IIIb jejunal atresia, and developmental delay secondary to vitamin B12 deficiency. Moreover, the patient was found to have a novel DNAH9 gene mutation in a compound heterozygous state. This is the first case of this rare disease to be reported from Bahrain. This case report is also associated with an extensive literature review., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Isa et al.)

17q12 deletion syndrome is a rare autosomal dominant inherited condition. It results from de novo mutation and can occur without a family history. Hepatocyte nuclear factor-1 beta (HNF1B) and LIM homeobox 1 (LXH1) genes are the most common genes to be deleted in this syndrome. It has unique clinical characteristics involving multiple systems in the body. The most common presentations are usually renal involvement and maturity-onset diabetes of the young type 5 (MODY5). Genetic study is the golden tool to diagnose patients with this syndrome. Our case presents the unique clinical features of 17q12 deletion syndrome along with a literature review., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Isa et al.)

Background: Respiratory syncytial virus (RSV) is a prevalent cause of lower respiratory tract infections. It may be associated with hepatocellular involvement, as indicated by increased liver enzymes aspartate aminotransferase and alanine transaminase (ALT)., Aim: To evaluate the rate of increased liver enzyme levels in children with acute bronchiolitis and correlate them with clinical, laboratory, and radiological variables., Methods: The study was a retrospective review of the medical records of children who presented with acute bronchiolitis when admitted to the Pediatric Department, Salmaniya Medical Complex, the Kingdom of Bahrain, between 2019 and 2020. We collected the demographic data, the clinical presentation, the laboratory and radiological findings, and the clinical outcomes. We compared the patients with elevated liver enzymes to those with normal levels at the time of presentation and at follow-up., Results: We included 166 (57.8%) of 287 patients with acute bronchiolitis who fulfilled the inclusion criteria. Ninety-three (56%) patients were males. The median age at presentation was 3.4 (interquartile range 1.1 to 12.4) mo. Fifty-four (28%) patients tested positive for RSV, which was confirmed in 15 of them (28%) by PCR. Laboratory findings of 161 patients tested at presentation showed high ALT levels in 14 (8.7%) patients and normal ALT in 147 (91.3%). Coagulation profiles were measured in 46 (27.7%) of 166 patients. High prothrombin time was present in 15 (32.6%), a high international normalized ratio was present in 13 (28.3%), and high activated partial thromboplastin time was present in three (6.5%). Thrombin time was elevated in nine (27.3%) of 33 patients. Five (21.7%) of 23 patients with available radiological data had hepatomegaly; one of them had findings suggestive of fatty infiltration. High ALT had a significant association with lengthy hospital stays ( P < 0.05) and positive urine culture ( P < 0.05). Seventy (42.2%) patients had documented follow-up with liver function tests over a median follow-up period of 10.2 (IQR, 2.4-23.3) mo. Total serum protein and serum globulin levels were normalized at the follow-up time, with a significant P value of < 0.05., Conclusion: This study showed a low prevalence of liver function involvement in patients with acute bronchiolitis with a benign course. However, there was a rising trend in ALT during follow-up. Prolonged hospital stay and positive urine cultures were associated with elevated liver enzymes., Competing Interests: Conflict-of-interest statement: The authors declare no conflict of interest for this article., (©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.)

Introduction: Urinary tract infections (UTIs) are fairly common in infants. Early diagnosis and prompt treatment are crucial to avoid complications. However, providing appropriate antibiotic treatment is a challenge to any clinician considering the rise in the urinary pathogens that are resistant to the commonly used antibiotics especially with the emergence of extended-spectrum beta-lactamase (ESBL)-producing organisms. Consequently, this leaves the physician with limited options of antibiotic choices. This study aims to investigate the local resistance patterns of uropathogens causing UTIs in hospitalized infants in Bahrain and to provide guidance for the preferred choice of empirical antibiotic treatment., Methods: A retrospective cross-sectional study with an analysis of the medical records of infants from birth to one year of age admitted with UTIs at Salmaniya Medical Complex between June 2015 and June 2017 was conducted., Results:  Data were obtained from 117 term infants (no preterm infants were included), with 80 (68.4%) being male. A total of 106 (90.6%) patients were less than six months of age. The most frequently isolated organisms were Escherichia coli found in 66 (49.6%) patients, followed by Klebsiella in 43 (32.3%). Forty-eight (36%) were ESBL-producing organisms (31 and 17 of E. coli and Klebsiella , respectively). The highest antibiotic resistance of E. coli was found against cephalosporin (55.5%), while penicillin resistance was only 19.4% and co-trimoxazole was 14.4%. Resistance rates of non- E. coli organisms were highest against cephalosporin (44.6%), followed by nitrofurantoin (19.1%) and penicillin (18.5%). The overall resistance to cephalosporin antibiotics was highest against cephalothin (a first-generation cephalosporin) (54.9%). The second highest resistance rate was demonstrated against cefuroxime (a second-generation cephalosporin) (50.5%), followed by ceftriaxone (a third-generation cephalosporin) (45.1%). Lower resistance rates were found against cefotaxime (22.3%), followed by ceftazidime (33.3%). A resistance rate of zero was found against cefepime (a fourth-generation cephalosporin). Aminoglycosides and nitrofurantoin demonstrated the lowest resistance rates among all cultured uropathogens. Among all cultures, 3.9% of the E. coli were resistant to aminoglycosides, while non- E. coli organisms showed zero resistance to aminoglycosides. Regarding the nitrofurantoin antibiotic, 19% of non- E. coli bacteria showed resistance, while only 1.7% of E. coli were resistant to this antibiotic. Zero resistance to carbapenem was found in E. coli organisms (both ESBL and non-ESBL), compared to 1.9% resistance by non- E. coli organisms (both ESBL and non-ESBL combined)., Conclusion: E. coli is the predominant uropathogen causing UTI in infants with high rates of ESBL organisms in our community. Since the highest resistance was found against cephalosporins, aminoglycosides and nitrofurantoin are the preferred empirical antibiotic options for the treatment of UTIs. For infants with ESBL organisms, carbapenem antibiotics represent a good treatment option., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Mohammed et al.)

Results: Out of 555 records, 404 neonates were included. Among those, 209 (51%) were males and 275 (68.1%) were Bahraini. The median indirect bilirubin level at presentation was 218 (interquartile range, 174-270) μ mol/L. ABO incompatibility was the commonest risk factor for neonatal indirect hyperbilirubinemia ( n = 152, 37.6%) followed by glucose-6-phosphate dehydrogenase (G6PD) deficiency ( n = 130/400, 32.5%). Age (>25 years) was the commonest maternal risk factor ( n = 331, 81.9%) followed by cesarean delivery ( n = 137, 33.9%). Neonates with ABO incompatibility had a significantly higher mean indirect bilirubin level compared to those with other risk factors (234.9 ± 68.5 versus 225 ± 82.2 mmol/L, respectively) ( P = 0.04). Phototherapy use significantly increased along with the rise of bilirubin level ( P < 0.0001). Intravenous immunoglobulins (IVIG) and exchange transfusion were used in 44 (10.9%) and 14 (3.5%) patients, respectively. Neonates who received IVIG had significantly higher bilirubin levels than those who did not ( P = 0.005). Male newborns ( P = 0.008), Bahrainis ( P = 0.001), those with reticulocytosis ( P = 0.001), and those who received IVIG ( P = 0.001) were more prone to have associated risk factors., Conclusion: ABO incompatibility, G6PD deficiency, and older maternal age were the commonest neonatal and maternal risk factors for developing neonatal indirect hyperbilirubinemia. Bahraini, male newborns, reticulocytosis, and IVIG use were associated with these factors. Early detection of such factors through screening can aid in immediate management to prevent serious complications of this common condition., Competing Interests: No potential conflict of interest relevant to this article was reported., (Copyright © 2022 Hasan M. Isa et al.)

Objective The objective is to assess the overall prevalence of maternal and neonatal pregnancy-related complications, and to compare their frequency among women with sickle cell trait (SCT) and those with normal hemoglobin patterns to examine the association between SCT and maternal and neonatal outcomes. Methods A retrospective cross-sectional case-control study was conducted by reviewing medical data from the labor room registry book and electronic database. All pregnant ladies delivered in Salmaneya medical complex from January 1 to December 31, 2019 were included. Patients were divided into two groups based on the presence or absence of SCT. Adverse maternal and neonatal outcomes were compared between the two groups.  Results Out of the 5,067 pregnant women reviewed, 934 (18.4%) were included (460 (49.3%) with SCT and 474 (50.7%) with healthy controls). Overall, maternal, and neonatal complications were noted in 40.8% (n=381) and 16.3% (n=152), respectively. In univariate analysis, patients with SCT were significantly more likely to underwent cesarean sections compared to healthy women, 28.7% (n=132) vs. 21.7% (n=103), respectively (P=0.044) and to have more intrauterine fetal death (3% [n=14] vs. 0.2% [n=1], respectively (P<0.0001). No significant differences were found between the two groups in terms of the occurrence of the pregnancy-related hypertensive disorder, gestational diabetes, small for gestational age, and preterm delivery.  Conclusion The result of this large, retrospective cross-sectional, case-control study shows that pregnant women with SCT were associated with an increase in intrauterine fetal death in comparison with pregnant women with normal hemoglobin. There were no differences found in pregnancy-related hypertensive disorder, gestational diabetes, small for gestational age, and preterm delivery. This result will emphasize the requirement of additional studies to scrutinize these findings and to determine whether there may be a benefit of a unique antenatal surveillance guideline for such patients., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Buhusayyen et al.)

Ectopia cordis is a rare type of malformation where the heart is not located normally. It may be partially or completely located outside the thoracic cavity and can be associated with other congenital abnormalities. It results from failure of maturation of midline mesoderm and ventral body formation during embryogenic formation. The exact etiology remains unknown. The literature review reveals the prognosis for infants with ectopia cordis is very poor. Here, we are reporting the first case of a fetus that was prenatally diagnosed with ectopia cordis that was associated with omphalocele documented in our country. Considering the poor prognosis for the fetus, conservative management during the prenatal period was chosen., Competing Interests: The authors declare that there are no conflicts of interest., (Copyright © 2022 Shereen M. Mohamed et al.)

Introduction Linear growth impairment (LGI) is one of the complications of pediatric inflammatory bowel disease (IBD). This study aimed to assess the linear growth of patients with pediatric IBD and to detect the frequency and the possible risk factors of LGI. Methods A retrospective cross-sectional review of medical records of patients with pediatric IBD was conducted in the pediatrics department, Salmaniya Medical Complex, Bahrain, from 1984 to 2019. Demographic and anthropometric data were gathered. World Health Organization (WHO) standards and references were used to define LGI. According to WHO, stunting and severe stunting were defined as length/height for age of <-2 standard deviations and <-3 standard deviations from age and sex-specific reference means, respectively. To determine the possible risk factors for LGI, stunted patients were compared with normal height patients in regard to demographic data, clinical presentations, and treatment used. Results Out of 130 patients with pediatric IBD, 88 (67.7%) had anthropometric data available. Fifty-five (62.5%) were males. Forty-seven (53.4%) had Crohn's disease and 41 (46.6%) had ulcerative colitis. The mean age at presentation was 10.7±3.8 years. The median age at the time of growth measurement was 14.2 (interquartile range=12.1-24.4) years. Fifteen (17%) patients were stunted, and seven (46.7%) of those stunted patients were severely stunted. Weight at presentation was lower in stunted patients (21.6±5.9 kilograms) compared to normal height patients (31±13.4 kilogram) (p=0.048). Sex, delivery type, birth weight, height at presentation, age at presentation, age at growth measurements, IBD type, disease duration, presence of extraintestinal manifestations, and prednisolone and biologic therapy use were not significant factors of stunting. Conclusion Patients with pediatric IBD have a high prevalence of LGI compared to the general population. Low weight at disease presentation is the only significant risk factor for LGI. This might indicate that IBD as a disease by itself is having the main negative impact on linear growth., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Isa et al.)

Background: Caudal epidural block (CEB) is a commonly performed neuraxial block to provide effective pain relief and analgesia in pediatric patients undergoing infraumbilical surgery., Aims: This study aimed to compare the effectiveness of adding CEB to general anesthesia (GA) in terms of intra- and postoperative pain management., Design: Prospective, randomized case-controlled trial study., Setting: Operation theater, and postoperative recovery rooms at Salmaniya Medical Complex, Bahrain., Materials and Methods: A total of 74 patients aged 2 months to 6 years with American Society of Anesthesiologists physical status classification I were recruited over a 6-month period between December 2019 and May 2020. Patients were allocated into two groups (Group A, with CEB) or (Group B, without CEB). Both groups were compared based on hemodynamic stability, pain scores, level of sedation, analgesia need, and parental satisfaction., Statistical Analysis: Data were analyzed using SPSS program. Categorical and numerical variables of both the groups were compared., Results: Patients with CEB had better hemodynamic stability during the surgical procedure based on heart rate ( P = 0.039). Pain intensity scores were less in patients with CEB than those without ( P < 0.001). Fentanyl consumption was lower in Group A compared to Group B at the end of surgery ( P = 0.002). They were also ambulated earlier and discharged sooner than those without CEB. Parental satisfaction was 92.1% in Group A compared to 63.9% in Group B ( P = 0.012)., Conclusions: Adding CEB to GA for intraoperative and perioperative pain control in pediatric patients undergoing infraumbilical surgery makes it more effective, safe, and with better parental satisfaction., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Anesthesia: Essays and Researches.)

Results: Of 287 patients, 229 (79.2%) were included. 132 (57.6%) were males. Median presentation age was 3.7 (interquartile range (IQR), 1.27-12.33) months. Median CRP level was 10.4 (IQR, 2.8-35.1) mg/L. CRP was high in 167 (72.9%) patients. 17.6% (33/187 patients) had confirmed bacterial coinfection. Respiratory syncytial virus (RSV) was detected in 84 (36.7%) patients. Mean CRP level was higher in RSV-negative compared to RSV-positive patients, 31.3 ± 44.3 versus 21.5 ± 27.7 mg/L, respectively ( P = 0.042). Respiratory viral serology profile was positive in 34.7% (17/49 patients). 66.9% (107/160 patients) had positive chest X-ray. Antibiotics were used in 78.1% (179/227 patients). Thirteen (5.7%) patients required intensive care, five (2.2%) had surgical intervention, four (1.8%) required endotracheal intubation, and four (1.8%) died. Patients with high CRP were older at presentation ( P < 0.0001) and had more fever ( P < 0.0001) and cough ( P = 0.002), but lower hemoglobin level ( P < 0.0001) compared to those with normal CRP. Fever ( P = 0.016) and hemoglobin level ( P = 0.002) were independent factors., Conclusion: Most children with acute bronchiolitis had high rate of elevated CRP values that did not correlate with the rate of bacterial coinfection. High CRP levels were found in older children, those presented with more fever and cough, and had a lower hemoglobin level despite that those factors were previously reported to be associated with disease severity and bacterial coinfection. This study also showed a high overall rate of antibiotic prescriptions in mostly viral disease., Competing Interests: No potential conflict of interest relevant to this article was reported., (Copyright © 2022 Hasan M. Isa et al.)

Objectives: To identify the prevalence of non-alcoholic fatty liver disease (NAFLD) among patients with type 2 diabetes mellitus (T2DM) in Bahrain and to assess the risk factors for the same., Methods: This retrospective cross-sectional study investigated a random sample of patients who were treated for T2DM during 2018 at non-communicable disease clinics in primary health centers in Bahrain. Cases of 382 patients who underwent abdominal ultrasonography were selected for the study. The collected patients' data were statistically analyzed. Prevalence of NAFLD among T2DM patients and the possible risk factors were assessed., Results: The mean age of the study population (N = 382) was 59.0±12.0 years. The majority (61.5%) were women. Hypertension (57.9%) was the most prevalent associated condition. Most patients were either overweight (30.5%) or obese (58.3%). Fatty liver was found in 68.1% patients based on ultrasound imaging. Elevated alanine aminotransferase was found in 75 (21.0%) out of 357 (93.5%) patients who were tested for the same. The significant risk factors identified for fatty liver were female ( p = 0.013), high body mass index (BMI) ( p < 0.001), high waist circumference ( p = 0.011), and high triglyceride levels ( p = 0.043). Binary logistic regression identified BMI as an independent risk factor for fatty liver ( p = 0.005)., Conclusions: The prevalence of NAFLD among patients with T2DM in Bahrain is high, and comparable to the levels reported in other studies. Female and high BMI, waist circumference, and triglyceride level are risk factors for NAFLD, while BMI is an independent risk factor., (The OMJ is Published Bimonthly and Copyrighted 2022 by the OMSB.)

Background: Plastic surgery is the most diverse specialty. It deals with a wide spectrum of abnormalities in different genders, age groups and body parts. Data on clinical characteristics of patients admitted in the burn and plastic surgery unit have been reported from our center last in 1993., Methods: This retrospective cross-sectional study conducted during 1-year from 2017 to 2018 in the burn and plastic surgery unit, Salmaniya Medical Complex, Bahrain. Seven hundred seventy-four patients (929 admissions) were enrolled. Indications of admissions, demographic data including gender, nationality and age were gathered. Different types of burns were categorized., Results: Out of 16,492 surgical admissions, 929 (5.6%) admissions were for burn and plastic surgery. Nine hundred-twelve (98.2%) admissions for 766 patients were included. Burn injuries were the main indication with a total of 345 (37.8%) admissions for 337 (44%) patients. Three hundred eighty-eight (50.7%) patients were males. Five hundred fifty-eight (72.8%) patients were nationals. Most were in the age group of 30-39 years old (24.9%). On comparison, burn injuries were more in males (n=241, 71.5%), nationals (n=175, 51.9%), younger in age (mean age, 23.8±19.6 years) and in pediatric age group (n=122, 36.2%) specifically, (All P <0.0001). Scalded burn was the commonest type (n=184/317, 58%)., Conclusion: Burn and plastic surgery is a significant part of surgical admissions. Burn injuries were the most frequent reason of admissions. Patients with burn injuries were mainly males, nationals and children. Scalded burn was the most frequent type in our center., Competing Interests: The authors declare that there is no conflict of interests.

Purpose: This study aimed to investigate the efficacy of human-derived umbilical cord mesenchymal stem cells (HDUMSC) and human-derived umbilical cord mesenchymal stem cells expressing erythropoietin (HDUMSC-EPO) to rescue total degenerated retina in a rat model., Methods: The study included four treatment groups, namely negative control using normal saline (HBSS) injection, positive control using sodium iodide 60 mg/kg (SI), SI treated with HDUMSC, and SI treated with HDUMSC-EPO given via subretinal and intravenous routes, to test the efficacy of retinal regeneration following SI-induced retinal degeneration. Retinal function in both phases was tested via electroretinography (ERG) and histological staining examining the outer nuclear layer (ONL)., Results: There was a statistically significant result (P < 0.05) in the SI treated with HDUMSC-EPO only when comparing day 11 (mean = 23.6 μv), day 18 (mean = 25.2 μv), day 26 (mean = 26.3 μv), and day 32 (mean = 28.2 μv) to the b-wave ERG on day 4 rescue injection day (mean = 12.5 μv). The SI treated with HDUMSC-EPO showed significant improvement in b-wave ERG readings in the Sprague-Dawley (SD) rat but did not restore baseline readings prior to degeneration (day 0). Both treated groups' ONL thicknesses did not show significant changes compared to the negative control group (HBSS) following rescue therapy., Conclusion: Total retinal degeneration following intravenous SI injection was observed at 60 mg/kg. SI treated with HDUMSC and HDUMSC-EPO showed no regenerative potential compared to baseline in SI-induced total retina degeneration on ERG or histology, whereas SI treated with HDUMSC-EPO group showed a substantial increase in b-wave ERG amplitude over time., Competing Interests: None

Background: This study aimed to develop an expert consensus regarding the epidemiology, diagnosis, and management of cow's milk protein allergy (CMPA) in the Middle East., Methods: A three-step modified Delphi method was utilized to develop the consensus. Fifteen specialized pediatricians participated in the development of this consensus. Each statement was considered a consensus if it achieved an agreement level of ≥ 80%., Results: The experts agreed that the double-blind placebo-controlled oral challenge test (OCT) should be performed for 2-4 weeks using an amino acid formula (AAF) in formula-fed infants or children with suspected CMPA. Formula-fed infants with confirmed CMPA should be offered a therapeutic formula. The panel stated that an extensively hydrolyzed formula (eHF) is indicated in the absence of red flag signs. At the same time, the AAF is offered for infants with red flag signs, such as severe anaphylactic reactions. The panel agreed that infants on an eHF with resolved symptoms within 2-4 weeks should continue the eHF with particular attention to the growth and nutritional status. On the other hand, an AAF should be considered for infants with persistent symptoms; the AAF should be continued if the symptoms resolve within 2-4 weeks, with particular attention to the growth and nutritional status. In cases with no symptomatic improvements after the introduction of an AAF, other measures should be followed. The panel developed a management algorithm, which achieved an agreement level of 90.9%., Conclusion: This consensus document combined the best available evidence and clinical experience to optimize the management of CMPA in the Middle East., (© 2021. The Author(s).)

Background: Celiac disease (CD) is a chronic autoimmune enteropathy. It results from genetic predisposition and exposure to gluten-containing food. The prevalence and presentation of CD vary among populations., Purpose: This study aimed to describe the prevalence and clinical characteristics of CD in children in Bahrain., Methods: We retrospectively reviewed the medical records of children diagnosed with CD in the pediatric department, Salmaniya Medical Complex, Bahrain, in 1988-2018. Their clinical, biochemical, serological, and histopathological findings were documented. Adherence to the recommended gluten-free diet (GFD) was assessed., Results: Of 86 patients with CD, 67 were included. The CD prevalence was 0.02%. A significant increase in prevalence in the last decade was observed (P<0.0001). Thirty-eight patients (56.7%) were males. The median (interquartile range) age at presentation was 4.45 (1.5-7.3) years. A family history of CD was positive in 13 out of 43 patients (30.2%). Pallor and failure to thrive were the most common presentations. The most frequent associated disease was iron-deficiency anemia in 23 patients (69.7%). Positive serology was found in 32 of 45 patients (71.1%). Marsh-Oberhuber type III was found in 16 of 35 patients (45.7%). Seropositive patients were significantly older (P=0.025) and had more severe duodenal histology (P=0.002). Adherence to GFD was poor in 27 patients (64.3%)., Conclusion: This study revealed a significant increase in CD prevalence over the last decade. Atypical presentations were frequent. Most patients had poor adherence to GFD.

Background: Coronavirus 2019 disease (COVID-19) is a highly infectious disease prompting extreme containment measures, including lockdown, travel restrictions, social distancing, and stringent personal hygiene. This study investigates the depression level and coping responses toward the lockdown, referred as the movement control order (MCO) during COVID-19 pandemic in Malaysia and its impact on quality of life., Method: This cross-sectional study was conducted from April to May 2020. The outcomes were assessed using the Depression, Anxiety and Stress Scale-21, Coping Orientation to Problems Experienced Inventory, and World Health Organisation Quality of Life-BREF Scale (WHOQOL-BREF) in both English and validated Malay versions., Results: Mild-to-severe depression was found in 28.2% (n = 149) of the 528 respondents. Respondents with mild-to-severe depression were significantly younger (33.09 ± 10.08 versus 36.79 ± 12.47 years), without partner (71.8% versus 45.6%), lived in the red zone (85.9% versus 71.0%), and had lower household income as defined in the category of B40 (51.7% versus 39.3%) compared to those without depression (all p < 0.01). The avoidant coping score was significantly higher (25.43 ± 5.69 versus 20.78 ± 5.65), while the religious coping score was significantly lower (5.10 ± 2.07 versus 5.94 ± 2.11) among those with mild-to-severe depression compared to those without depression (both p < 0.001). Respondents with mild-to-severe depression also had significantly lower mean score in each domain of WHOQOL-BREF compare to those without depression [(physical health, 13.63 ± 2.66 versus 16.20 ± 2.11), (psychological, 12.5 ± 2.79 versus 16.10 ± 2.14), (social relationships, 12.17 ± 3.49 versus 15.28 ± 2.93), environment (14.50 ± 2.39 versus 16.21 ± 2.14), all p < 0.001] after controlling for age, marital status, zone, household income, and coping scores., Conclusion: COVID-19 lockdown had adverse mental health effects. Our study highlighted that approximately one in three individual experienced mild-to-severe depression during the nationwide MCO. The varied impact of the pandemic on mental health could be due to different population characteristics and coping strategies used. Identifying those at higher risk to develop depression during MCO for COVID-19 pandemic could help mental healthcare service providers to plan services for those susceptible, thereby mitigating the pandemic's effect on quality of life.

Introduction: Teaching disaster response medicine (DRM) to medical students requires considerable resources. We evaluate the effectiveness of e-learning in teaching emergency disaster response (ELITE-DR), a novel initiative, in educating medical students of the cognitive aspect of DRM. Methods: A prospective cross-sectional study among pre-clinical year medical students was carried out to determine their knowledge on DRM and perception regarding the ELITE-DR initiative using a validated online questionnaire. A three-part self-learning video covering the principles and medical management of DRM were distributed before answering the questionnaire served as the training. Results: A total of 168 students participated in the study. Their overall knowledge showed a significant increase in between pre-and-post-interventions. Recall and simple decision-making knowledge aspects were better than complex decision-making knowledge. It appeared that participants assimilate knowledge better from visual rather than audio stimuli. Participants with high perception-scores demonstrated better knowledge-scores. However, e-learning was not preferred as a substitute for face-to-face (F2F) teaching. Conclusion: ELITE-DR shows promise in teaching DRM. Simple recall and comprehension levels of knowledge were well-served through this technique. However, for more complex decision-making knowledge, a different approach might be required. ELITE-DR offers flexibility, accessibility, and personalized learning. The content presentation is improved by using several different visual stimuli. This approach is useful for cognitive aspect learning, but it should not replace standard F2F teaching., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Saiboon, Zahari, Isa, Sabardin and Robertson.)

Foramina transversaria are bilateral landmarks of human cervical vertebrae. Morphometric analysis of foramina transversaria is valuable in both clinical and academic fields. This study aims to build a novel normative database of the dimensions of foramina transversaria in Jordanian population and to correlate these data with gender and age. For this purpose, axial computer tomographic images of cervical vertebrae of 329 normal individuals were obtained from the Radiology Department at King Abdulla University Hospital. Cases were subdivided into adolescence, youth, and adulthood groups for both genders. Antero-posterior and transverse diameters were measured bilaterally at all cervical vertebral levels. Statistical analysis of measured dimensions was performed using Statistical Package of Social Sciences software. The results showed that the overall mean antero-posterior diameter of foramina transversaria was 6.09 ± 0.60mms and 5.94 ± 0.62mms on the left and on the right sides, respectively. The corresponding mean transverse diameter measured 4.76 ± 0.51mms and 4.62 ± 0.52mms on the left and the right sides, respectively. Foramina transversaria were significantly larger in males compared to females (p = 0.00). Age-related differences were statistically significant for both antero-posterior and transverse diameters in favor of adult age-group, irrespective of vertebral level. The relevance of these findings is discussed.

An international, expert led consensus initiative was set up by the Collaborative Ocular Tuberculosis Study (COTS) group to develop systematic, evidence, and experience-based recommendations for the treatment of ocular TB using a modified Delphi technique process. In the first round of Delphi, the group identified clinical scenarios pertinent to ocular TB based on five clinical phenotypes (anterior uveitis, intermediate uveitis, choroiditis, retinal vasculitis, and panuveitis). Using an interactive online questionnaires, guided by background knowledge from published literature, 486 consensus statements for initiating ATT were generated and deliberated amongst 81 global uveitis experts. The median score of five was considered reaching consensus for initiating ATT. The median score of four was tabled for deliberation through Delphi round 2 in a face-to-face meeting. This report describes the methodology adopted and followed through the consensus process, which help elucidate the guidelines for initiating ATT in patients with choroidal TB.

Blindness and vision loss contribute to irreversible retinal degeneration, and cellular therapy for retinal cell replacement has the potential to treat individuals who have lost light sensitive photoreceptors in the retina. Retinal cells are well characterized in function, and are a subject of interest in cellular replacement therapy of photoreceptors and the retinal pigment epithelium. However, retinal cell transplantation is limited by various factors, including the choice of potential stem cell source that can show variability in plasticity as well as host tissue integration. Dental pulp is one such source that contains an abundance of stem cells. In this study we used dental pulp-derived mesenchymal stem cells (DPSCs) to mitigate sodium iodate (NaIO 3 ) insult in a rat model of retinal degeneration. Sprague-Dawley rats were first given an intravitreal injection of 3 × 10 5 DPSCs as well as a single systemic administration of NaIO 3 (40 mg/kg). Electroretinography (ERG) was performed for the next two months and was followed-up by histological analysis. The ERG recordings showed protection of DPSC-treated retinas within 4 weeks, which was statistically significant (* P ≤ .05) compared to the control. Retinal thickness of the control was also found to be thinner (*** P ≤ .001). The DPSCs were found integrated in the photoreceptor layer through immunohistochemical staining. Our findings showed that DPSCs have the potential to moderate retinal degeneration. In conclusion, DPSCs are a potential source of stem cells in the field of eye stem cell therapy due to its protective effects against retinal degeneration., (Copyright © 2019. Published by Elsevier B.V.)