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Background. Ocular extraintestinal manifestations (O-EIMs) are one of the most well-known EIMs in patients with inflammatory bowel disease (IBD). This study aimed to identify the frequency of O-EIMs in children with IBD, referred to Mofid Children's Hospital, Tehran, Iran, during 2014 to 2019. Methods. Children with IBD, younger than the age of 18 years, who were referred to an ophthalmologist, were included in this study. Results. Ninety-six patients with IBD were examined. Four patients had ocular manifestation of IBD. Two patients had complications due to treatment of IBD. The mean age of the patients was 11.25 ± 4.17 years (range: 5.5-17 years). The O-EIMs included 3 (50.0%) cases of anterior uveitis, 1 (16.7%) case of episcleritis, and 2 (33.3%) cases of posterior subcapsular cataract. Conclusion. O-EIMs are important in children with IBD. Therefore, it is recommended that annual screening for ocular complications be performed in all children with IBD. [ABSTRACT FROM AUTHOR]

Background: Lead exposure is one of the most menacing of environmental exposures, particularly in children. Children are more susceptible to the effects of lead which manifest in many organ systems, including interference with mental and motor development. Lead poisoning can cause colicky abdominal pain. In this study, the authors sought to evaluate the prevalence of elevated blood lead level (BLL) and its contributing factors among pediatric patients presenting with abdominal pain. An epidemic of lead poisoning in adults was previously uncovered, and thus a concern for pediatric lead poisoning was raised.Methods: Pediatric patients presenting to two pediatric clinics in Tehran with abdominal pain were eligible for enrollment in a descriptive prospective cross-sectional study. A predesigned questionnaire was filled for each patient by their consenting parents. The questionnaire queried demographic information, environmental, social, and other relevant parameters for lead exposure. After completion of the questionnaire, biometrics were obtained, and a blood sample was taken from each patient for measurement of BLL and complete blood count.Results: A total of 187 patients were enrolled in the study. Of them, almost 20% had BLL ≥ 5 µg/dL. Univariate analysis showed that age (p = 0.002, OR 3.194, CI 95% 1.504-6.783), weight (p = 0.009, OR 2.817, CI 95% 1.266-6.269), height (p = 0.003, OR 3.155, CI 95% 1.443-6.899), and playing with both plastic and cotton toys (p = 0.03, OR 2.796, CI 95% 1.072-7.295) were significant predictors of high BLLs. Maternal level of education correlated with blood lead concentrations (p = 0.048, OR 2.524, CI 95% 1.006-6.331).Conclusions: A clinically significant number of cases of abdominal pain may have high BLLs. Specific attention should be paid to children presenting with abdominal pain, especially due to the detrimental effects of lead on their mental and motor development. [ABSTRACT FROM AUTHOR]

Aim: This multicenter study is the first one on Iranian children with very early onset ulcerative colitis (UC) and one of the few studies about the effect of biological therapy in children with UC under 7 years of age. Background: Children with very early onset inflammatory bowel disease (IBD) are diagnosed before 6 years of age Methods: The current study was performed on 14 children under 7 years of age with severe UC. Children with severe UC whose therapy with corticosteroid and azathioprine as conventional treatment had failed were treated with infliximab (IFX) and later with adalimumab (ADA). Results: Among the total 14 participants, 6 (43%) patients were female. Mean patient age was 4.9 years (range = 3-7 years), mean age at diagnosis was 3.4 years (range = 1.5-6 years), and mean duration of illness was 1.5 years. At the end of 54 weeks of therapy with IFX, 2 (14%) patients were in remission, 2 (14%) patients were mild, and 4 (29%) patients were moderate, with no secondary treatment failure (during the maintenance phase). A total of 6 (43%) patients had primary treatment failure (no response after 14 weeks of therapy). These patients were treated with ADA. At the end of 52 weeks of therapy, 3 (50%) of those 6 (100%) patients were referred for colectomy, 1 (17%) was in remission, and 2 (33%) patients had mild severity. Conclusion: The current study has shown that IFX is a safe and effective therapy for children with very early onset UC. ADA may be effective in the treatment of children with UC who are refractory to IFX. [ABSTRACT FROM AUTHOR]

Aim: Description of the inflammatory bowel disease natural history in Tehran province. Background: Inflammatory bowel disease (IBD) is a non-homogeneous disorder with an unpredictable natural history that impairs a patient's quality of life over the course of their life. As a result, providing evidence for efficient patient management is critical. Methods: In this case series study, 198 IBD patients who were visited in our clinic at least three times routinely from Oct 2015 to May 2020 were included. Then, two panel-based approaches, the Multi-State Model (MSM) and random-effect ordered logistic, were used to deduce the clinical course of IBD, which included remission, mild, moderate to severe, and surgical states. Results For ulcerative colitis (UC), women had a slightly poorer condition for remission but better for moderate to severe and a faster transition from moderate to severe to mild (HR=1.490, 95% CI: 1.02-2.16) compared to men. For Crohn's disease (CD), they had a better condition for remission but a slightly poorer condition for the severe state and higher transition from mild to moderate to severe (HR=1.221, 95% CI: 0.4713.22) than men. Oral 5-ASA had better efficacy in people with remission and/or mild states but not for those with moderate to severe states, especially in CD (mild to moderate to serve, HR=1.526, 95% CI: 0.59-3.89). Immunosuppressive drugs were better for patients with lower disease severity, especially with UC (mild to remission, HR=1.258, 95% CI: 0.75-2.09). Conclusion: Panel approaches have the potential efficacy to tackle the unpredictable clinical course of IBD (UC/CD). Hence, we highly recommend that our findings be included into the Iranian routine clinical environment of IBD and/or that related studies be conducted in Iran and other regions to gain a better understanding of the natural history of IBD. [ABSTRACT FROM AUTHOR]

Introduction: According to the World Health Organization, the incidence of severe acute hepatitis of unknown origin in children is increasing worldwide. However, there are no known causes and treatments to deal with this disease. This study presents three children with severe acute hepatitis of unknown origin in a hospital medical center in Iran. Case Presentation: Two 12-year-old children and a six-year-old child were admitted to the hospital with jaundice. Laboratory tests showed markedly elevated liver enzymes and total and direct bilirubin in these patients. However, hepatitis B surface antigen (HBsAg), hepatitis C virus (HCV) antibodies (Ab), hepatitis A virus (HAV) IgM, HEV IgM, Epstein-Barr virus (EBV) IgM, anti-microsomal Ab, anti-liver kidney (LKM Ab), anti-smooth muscle antibodies (ASMA), anti-nuclear antibodies (ANA), and total IgG were all negative. After excluding the probable infectious causes of acute hepatitis and per the progressive course of fulminant hepatic failure, intravenous methylprednisolone was started for two of three cases during hospitalization. These children were discharged from the hospital in good general condition, and the liver function tests gradually decreased and approximated to near normal during the follow-up visits. Conclusions: General practitioners and pediatricians are advised to examine the underlying causes and consider severe acute hepatitis of unknown origin when visiting children under 16 years with severe jaundice and very high transaminases. [ABSTRACT FROM AUTHOR]

Background: The projection studies are imperative to satisfy demands for health care systems and proper response to the public health problems such as inflammatory bowel disease (IBD).Methods: To accomplish this, we established an illness-death model based on available data to project the future prevalence of IBD in Asia, Iran in particular, separately from 2017 to 2035. We applied two deterministic and stochastic approaches.Results: In 2035, as compared to 2020, we expected a 2.5-fold rise in prevalence for Iran with 69 thousand cases, a 2.3-fold increment for North Africa and the Middle East with 220 thousand cases, quadrupling of the prevalence for India with 2.2 million cases, a 1.5-fold increase for East Asia region with 4.5 million cases, and a 1.6-fold elevation in prevalence for high-income Asia-Pacific and Southeast Asia regions with 183 and 199 thousand cases respectively.Conclusions: Our results showed an emerging epidemic for the prevalence of IBD in Asia regions and/or countries. Hence, we suggest the need for immediate action to control this increasing trend in Asia and Iran. However, we were virtually unable to use information about age groups, gender, and other factors influencing the evolution of IBD in our model due to lack of access to reliable data. [ABSTRACT FROM AUTHOR]

Background: Celiac disease is an immune-mediated systemic disease, in which gluten ingestion causes different symptoms. HLADQ2 is positive in 90% - 95% of celiac patients. HLA has been considered to inhibit antibody production against the hepatitis B virus vaccine. Considering that celiac disease affects about 1% of the population, this phenomenon could be a significant factor in immunization programs. On the other hand, HLA-DQ2 positive patients not only are at the risk of HBV infection themselves but also have the potential to be an important source of HBV dissemination in the world. Objectives: This study was carried out to evaluate the responsiveness of celiac-affected children to HBV vaccination in Iran. Methods: This case-control prospective study was performed on 62 Iranian children (31 children with confirmed celiac disease before introducing a gluten-free diet and 31 healthy children). HBS Ab (antibody against hepatitis B virus surface antigen) titer was checked in the patients and compared between the two groups. Results: It was shown that 67.7% of cases and 64.5% of controls had HBS Ab titer above 10 mIU/mL, but the difference was not signifi- cant statistically. After matching for the time interval from the last HBV vaccination, it was observed that although non-significantly, celiac disease can decrease the chance for anti-HBS production up to 30%. Conclusions: This study did not confirm non-responsiveness against the HBV vaccine in celiac disease in children. This may be due to genetic factors or type of vaccine. Moreover, in our study, responsiveness was assessed qualitatively in the two groups (HBS Ab > 10 mIU/mL was considered as a responder). [ABSTRACT FROM AUTHOR]

Background: Effective and safe procedural sedation is necessary for percutaneous liver biopsy in children. There are a number of different protocols for this purpose. The current study investigated ketamine and DPT cocktail (meperidine (Demerol®) + promethazine (Phenergan®) + chlorpromazine (Thorazine®)). Methods: The current cohort of 80 Iranian children aimed at investigating percutaneous liver biopsy. Each of the 2 study groups (ketamine and DPT) included 40 patients. Both groups were matched by age (number of participants under and above 7 years old). The current study evaluated the efficacy of 2 protocols by CHEOPS (children's hospital of Eastern Ontario pain scale) and visual analogue scale (VAS) pain scoring system and sedation scoring A,B,C, and D. Results: Ketamine group was sedated and recovered much more rapidly than the DPT receiving patients. Also, they had significantly less pain during the biopsy. The most common side effect of ketamine was vomiting (27%); in the other group, transient hypotension and tachycardia were more common. Conclusions: Ketamine is a safe and effective choice for procedural sedation in percutaneous liver biopsy in children. [ABSTRACT FROM AUTHOR]

This study aims to investigate the association between the MIND index (Mediterranean- Dietary approaches to Stop Hypertension diet Intervention for Neurodegenerative Delay) and attention-deficit hyperactivity disorder (ADHD) in the Iranian children. It builds upon existing research that highlights the role of dietary antioxidants in alleviating psychological disorders, cognitive impairments, and memory deficits. Additionally, previous studies have separately explored the beneficial effects of the Mediterranean and DASH diets on these issues. A case-control study was undertaken in Iran, involving a sample of 360 children and adolescents aged 7–13 years. Participants were divided into two groups, namely the case group (n = 120) and the control group (n = 240), with age and sex being matched between the groups. The Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV-TR) was employed for the diagnosis of ADHD. The MIND diet score was computed using the food intake data acquired from the Food Frequency Questionnaire (FFQ) completed by the subjects. The mean ± SD for the age and BMI of the study population was 8.76 ± 1.64 years and 16.90 ± 3.58 kg/m2, respectively. The mean score of MIND in this study was 27.93. After adjustment for potential confounder in the final model, subjects in highest compared to the lowest quartile of MIND diet score had significantly lower odds of ADHD (OR = 0.59, 95% CI 0.37–0.83; P-trend = 0.019). This study provides valuable evidence suggesting that adherence to the MIND diet is associated with decreased odds of ADHD. [ABSTRACT FROM AUTHOR]

Patients with very early-onset inflammatory bowel disease (VEO-IBD) may present because of underlying monogenic inborn errors of immunity (IEI). Strong differences have been observed in the causes of monogenic IBD among ethnic populations. This multicenter study was carried out on 16 Iranian patients with VEO-IBD. We reviewed clinical and basic immunologic evaluation including flow cytometry and immunoglobulin levels. All patients underwent clinical whole exome sequencing (WES). Sixteen patients (8 females and 8 males) with a median age of 43.5 months were enrolled. The median age at the onset of symptoms was 4 months. Most patients (12, 75%) had consanguineous parents. Chronic non-bloody diarrhea (13, 81.3%) and perianal diseases including perianal abscess (6, 37.5%), anal fissure (6, 37.5%), or anal fistula (2, 12.5%) were the most common manifestations. WES identified a spectrum of genetic variants in 13 patients (81.3%): IL10RB (6, 37.5%), MVK (3, 18.8%), and CASP8, SLC35C1, G6PC3, and IKBKB in 1 patient, respectively. In 3 patients (18.7%), no variant was identified. Flow cytometry identified a spectrum of abnormalities that helped to assess the evidence of genetic diagnosis. At the end of the survey, 3 (18.8%) patients were deceased. This high rate of monogenic defects with a broad spectrum of genes reiterates the importance of investigating IEI in patients with infantile-onset IBD., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Immunology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: The incidence of acute pancreatitis in children is increasing, but causes and diagnostic and therapeutic methods are various in different centers. The aim of this study was to investigate the common causes and routine diagnostic and therapeutic methods of acute pancreatitis in children in a pediatric gastrointestinal referral center and its accordance with existing guidelines., Methods: In this retrospective, cross-sectional study, a total of 60 children with a diagnosis of acute pancreatitis, were studied., Results: The most common causes of acute pancreatitis were systemic and metabolic diseases and medications. CT scan was performed for 36% of patients, but 31% of patients, for whom a CT scan was performed had no clear indication of CT scan. Only half of the patients received fluid 1.5 times their maintenance in the first 24 h. Antibiotic therapy was performed for 48% of patients but medical indications for antibiotic treatment were found in only 34% of cases. During the COVID-19 pandemic, the relative incidence of acute pancreatitis was increased., Conclusions: In children with systemic and metabolic disease and using anticonvulsant drugs, it is important to consider the incidence of this disease. In clinical education, the risks of radiation due to unnecessary CT scans and inappropriate prescription of antibiotics need to be emphasized. More research should be done to study the association between COVID-19 and acute pancreatitis., (© 2022. The Author(s).)

Background: The aim of this study was to evaluate the serum level of matrix metalloproteinase 7 (MMP7) in infants with cholestasis and the diagnostic values of this biomarker to differentiate biliary atresia (BA) from other causes of cholestasis., Methods: This multi-center study is conducted during 2 years in Mofid children's hospital and Children's Medical Center, Pediatrics Center of Excellence Tehran, Iran. 54 infants with cholestasis were enrolled in this study with a control group consists of 41 healthy infants with the same age. Serum samples were taken from all these patients to assess serum levels of MMP7, Gamma-glutamyl Transferase (GGT). For each biomarker, we calculated the sensitivity and specificity and other statistical characteristics., Results: There were 89 subjects, 22 patients with BA, 32 patients with non-BA cholestasis and 41 subjects as control group. The mean serum MMP7 levels in BA, non-BA cholestasis and control group was 15.91 ng/ml ± 6.64, 4.73 ng/ml ± 2.59 and 0.49 ng/ml ± 0.33, respectively. The best cut-off point is calculated 7.8 ng/ml for MMP7 and 434.5 U/L for GGT. The area under curve (AUC) for these two markers are 0.988 ± 0.008 and 0.854 ± 0.052, respectively. The sensitivity and specificity of MMP7 to differentiate biliary atresia from nonbiliary atresia cholestasis in our study was 95.5% and 94.5%, respectively. The sensitivity and specificity of GGT was 77.3% and 77.8%, respectively. These results show that the MMP7 has more sensitivity and specificity in differentiation., Conclusion: MMP7 demonstrated good accuracy to differentiate biliary atresia from other causes of cholestasis., (© 2022. The Author(s).)

We describe a cohort of 25 Iranian patients with infantile inflammatory bowel disease (IBD), 14 (56%) of whom had monogenic defects. After proper screening, patients were referred for whole exome sequencing (WES). Four patients had missense mutations in the IL10 RA , and one had a large deletion in the IL10 RB . Four patients had mutations in genes implicated in host:microbiome homeostasis, including TTC7A deficiency, and two patients with novel mutations in the TTC37 and NOX1 . We found a novel homozygous mutation in the SRP54 in a deceased patient and the heterozygous variant in his sibling with a milder phenotype. Three patients had combined immunodeficiency: one with ZAP-70 deficiency (T + B + NK - ), and two with atypical SCID due to mutations in RAG1 and LIG4 . One patient had a G6PC3 mutation without neutropenia. Eleven of the 14 patients with monogenic defects were results of consanguinity and only 4 of them were alive to this date.

LPS-responsive beige-like anchor protein (LRBA) deficiency is a monogenic primary immunodeficiency characterized by a heterogeneous spectrum of clinical manifestations associated with immune dysregulation. In this study, we reported clinical, immunologic, and genetic evaluation of two Iranian patients from unrelated families, both suffering from recurrent respiratory tract infections, failure to thrive, interstitial lung disease, autoimmune cytopenia, and hypogammaglobulinemia. Pulmonary abscess in one patient and persistent enteropathy in another were also observed. Further investigations revealed causative mutations in the exon (c.2166_2766del) and intron (c.4730-3 T > G) of the LRBA gene. These results may provide further elucidation of the clinical phenotypes and responsible genetic factors of LRBA deficiency.

Background: OObesity is a complex disorder influenced by various genetic and environmental factors. It has been shown that gut microbiota, which colonizes gastrointestinal tract, has a substantial role as an environmental factor in the pathophysiology of obesity. Since the composition of gut microbiota alters with regard to different criteria, such as ethnicity, geographical location, diet, lifestyle, age, and gender, we aimed to determine firmicutes/bacteroidetes (F/B) ratio and the abundance of important gut microbiota members, Akkermansia muciniphila, Faecalibacterium prausnitzii, Roseburia, Bifidobacterium, and Prevotella in Iranian obese and normal weight individuals, for the first time., Methods: In this study, 50 normal and 50 obese subjects were recruited and classified based on their BMI into normal weight and obese groups. Stool samples were collected. Following DNA extraction from the samples, quantitative PCR was conducted based on 16s rDNA universal primers. Finally, the correlation between the bacterial abundance and obesity was analyzed by statistical analyses., Results: We observed a significant increase of F/B ratio in the obese group, compared to the normal weight group (p = 0.002). Although A. muciniphila (p = 0.039) and Bifidobacterium (p = 0.049) abundance significantly decreased, the abundance of F. prausnitzii (p = 0.046) significantly elevated with BMI increase in the studied groups., Conclusion: Owing to the importance of the gut microbiota composition in obesity development, determination and targeted restoration of gut microbiota pattern could be valuable in the control and treatment of obesity in certain populations.

Background: Malnutrition in hospitalized patients causes problems in treatment and increases hospitalization duration. The aim of this research was to determine the prevalence of malnutrition in hospitalized children., Methods: Children aged 1 month to 18 years (n = 1186) who were admitted to medical and surgery wards of Mofid children's hospital from November 2015 to February 2016, entered the study. We measured different anthropometric variables in patients with malnutrition. Also, nutritional counseling was performed and three months follow-up was done., Results: Patient data were registered in questionnaires particularly for children 2 years old and less. 597 children under 2 years of age and 607 children over two years entered the study. The data analysis was done by SPSS version 22.0 (Chicago, IL, USA). The t test inferential method was used in comparing variables. P values less than 0.05 were considered statistically significant. Based on the body mass index (BMI) Z score, and in accordance with the World Health Organization (WHO) cut-off, among children over 2 years, 9% were diagnosed as overweight or obese, 54% were within the normal range and 37% were underweight at time of admission. In the underweight group, 43% were mildly, 21.2% were moderately and 35.8% were severely underweight. Based on the weight for length Z score in patients less than 2 years of age at time of admission, 6% were overweight, 60% were in normal range and 34% were underweight. Among children with malnutrition, 21% had mild, 3.0% had moderate and 10% had severe malnutrition. No significant meaningful relation was found between prevalence of malnutrition and severity of illness. In the moderate to severe undernutrition group, nutritionist counseling was done. Comparison of BMI and weight, before and after admission (the baseline and the follow up visits), was done by means of repeated measurements. Comparison of the patient's weight at time of admission with weight at 1, 2 and 3 months after the first nutritional consultation showed statistically meaningful difference (P value < 0.05)., Conclusion: Growth indices need to be evaluated in every hospitalized child. Nutritional consultation is useful in children with malnutrition. The main purpose of early diagnosis of malnutrition is to prevent its progression, and also to design a useful, applicable and cost-effective nutritional intervention for malnutrition treatment., (© 2018 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.)