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Fahad Al-Saif,1 Ahmed Alhumidi,2 Rama Ayed Alhallaf1 1Dermatology Department, 2Pathology Department, College of Medicine, King Saud University, Riyadh, Saudi Arabia Abstract: Dyschromatosis symmetrica hereditaria (DSH) is a rare genodermatosis characterized by various sizes of both hyper- and hypopigmented macules arranged in reticulated patterns on the face and the dorsal aspects of the extremities. There are also cutaneous and extracutaneous abnormalities, but they are rare. As far as we know, DSH associated with immune-mediated conditions has not been reported. We report the first case of DSH, which is associated with cutaneous lupus erythematosus and hyperthyroidism. Keywords: dyschromatosis, genodermatosis, lupus, reticulated, autoimmune

Pyridoxine HCl (vitamin B6; Pyr-H3) complexes with the formula [M(Pyr-H)(H2O)4].Cl·nH2O [M= V(III), Ru(III); n = 0.1], [Pt(Pyr-H)(H2O)(Cl)].Cl, [Se(Pyr-H3)(O)(OH)2], and [Au(Pyr-H)(H2O)2]·Cl are synthesized by the reactions of Pyr-H3 with metal chlorides [V(III), Ru(III), Pt(II), Se(IV), and Au(III)] at 60°C in methanol-water 50: 50 v/v. The accumulated data of the elemental analysis, conductivity measurements, mass, FT-IR, 1H, and 13C NMR, UV-Vis spectroscopy and magnetic moments support elucidated stoichiometry, structures and chelation of the complexes. Thermogravimetric analysis of the synthesized solid complexes is carried out for determining their thermal stability, number of crystalline water molecules and decomposition steps. According to the physicochemical analyses Pyr-H3 reacts with metal ions as a bidentate ligand via both phenolate oxygen and oxygen of the CH2OH group. The antimicrobial and anticancer tests reveal that complexes demonstrate higher antibacterial activity than free Pyr-H3 chelate. According to the cytotoxic results of Pt(IV) and Au(III) complexes in vitro based on human hepato cellular carcinoma (HepG-2) and human breast cancer (MCF-7) tumor cell lines, the former one exhibits promising activity. [ABSTRACT FROM AUTHOR]

Physical spectroscopic characteristics of Co(II), Ni(II), Cr(III), and Fe(III) cholyltaurine adducts have been studied. IR and Raman spectra confirmed that the cholyltaurine compound acted as a mononegative bidentate chelating agent. Nitrogen atom of the NH group and O of deprotonated sulphonate group acted as chelation sites towards the metal ions. UV-Vis spectra and magnetic moments block measurements confirmed that all complexes had the octahedral geometry. Water, Cl, and NO acted as secondary chelates. The positron annihilation lifetime (PAL) technique was applied to investigate the structural changes of cholyltaurine ligand and its complexes. The results indicated that the average defect density in the studied ligand and its complexes was efficiently correlating with their molecular weight. [ABSTRACT FROM AUTHOR]

Linear IgA bullous dermatosis is a rare sulfone-responsive subepidermal blistering disorder of unknown etiology in which smooth linear deposits of IgA are found in the basement membrane zone. Chronic bullous dermatosis of childhood is equivalent to linear IgA disease of adulthood and is characterized by an abrupt onset of large, widespread and tense bullae on a normal or erythematous base. In this case, we describe an unusual presentation of chronic bullous dermatosis in a 14-month-old Saudi girl. Histopathological examination revealed subepidermal cell poor blisters with linear deposition of IgA, IgG, IgM, and C3 along the dermoepidermal junction. The unusual clinical, histopathological and immunofluorescence findings in this patient are discussed, with an account on the differential diagnosis in such cases along with a detailed review of the relevant literature.

Background: Incense burning such as scented candles are commonly used in Arabian Gulf regions as it is thought to produce relaxing effects on people's mood. This study is conducted to examine the prevalence of scented candles' usage, extent of exposure and its effects on individuals' health based on symptoms prevalence in young university students., Material and Method: A cross-sectional study was conducted on university students from different regions in Saudi Arabia. Data was collected in March 2020 using an online questionnaire survey adapted from The European Community Respiratory Health Survey-II (ECRHS-II). Inclusion criterion for recruitment was students with non-smoking status. Descriptive statistics were used to report demographic data on the extent of exposure to scented candles (in terms of frequency and duration) and the presence of symptoms. Multiple logistic regression analysis was used to assess the relationship between scented candles exposure and respiratory and other health-related problems., Results: The prevalence of scented candles usage was 65.7% (472/718) among the respondents. However, its pervasiveness was significantly higher in females than in male respondents (74.9% vs. 28.4%; p = 0.0001). Among the scented candle users, 34.8% of the respondents used the scented candles more than 4 times a month and 40.2% of the respondents lit the scented candles for 20-40 min. A total of 117 (24.8%) respondents reported health-related problem and the top three health problems were headache 72 (15.2%), shortness of breath 42 (8.9%) and cough 37 (7.8%). The scented candle usage 5-6 times a week showed significantly lower wheezing (OR = 0.10, 95%CI 0.02-0.54, p = 0.008). The duration of more than 60 min of scented candle exposure showed higher occurrence of headache 1.42 times (95% CI = 0.68-2.96), sneezing 1.29 times (95% CI = 0.42-4.00) and wheezing 1.23 times (95% CI = 0.48-3.13), though the association was not significant., Conclusion: The results show that scented candle usage is more prevalent among female university students in Saudi Arabia. The common health-related problems associated with scented candle exposure were headache, shortness of breath and coughing., (© 2023. The Author(s).)

Non-alcoholic fatty liver disease (NAFLD) is a common chronic condition caused by the accumulation of fat in the liver. NAFLD may range from simple steatosis to advanced cirrhosis, and affects more than 1 billion people around the world. To date, there has been no effective treatment for NAFLD. In this study, we evaluated the expression of 4 candidate NAFLD biomarkers to assess their possible applicability in the classification and treatment of the disease.Twenty-six obese subjects, who underwent bariatric surgery, were recruited and their liver biopsies obtained. Expression of 4 candidate biomarker genes, PNPLA3, COL1A1, PPP1R3B, and KLF6 were evaluated at gene and protein levels by RT-qPCR and enzyme-linked immunosorbent assay (ELISA), respectively.A significant increase in the levels of COL1A1 protein (P = .03) and PNPLA3 protein (P = .03) were observed in patients with fibrosis-stage NAFLD compared to that in patients with steatosis-stage NAFLD. However, no significant differences were found in abundance of PPP1R3B and KLF6 proteins or at the gene level for any of the candidate.This is the first study, to our knowledge, to report on the expression levels of candidate biomarker genes for NAFLD in the Saudi population. Although PNPLA3 and PPP1R3B had been previously suggested as biomarkers for steatosis and KLF6 as a possible marker for the fibrosis stage of NAFLD, our results did not support these findings. However, other studies that had linked PNPLA3 to fibrosis in advanced NAFLD supported our current finding of high PNPLA3 protein in patients with fibrosis. Additionally, our results support COL1A1 protein as a potential biomarker for the fibrosis stage of NAFLD, and indicate its use in the screening of patients with NAFLD. Further studies are required to validate the use of COL1A1 as a biomarker for advanced NAFLD in a larger cohort.

The health care system is one of the key areas where people with mental illnesses could experience stigma. Clinicians can hold stigma attitudes during their interactions with patients with mental illness. To improve the quality of mental health services and primary care, evidence-based practices should be disseminated and implemented. In this study, we evaluated the attitudes of health care providers in Bahrain toward people with mental illness and adoption of evidence-based practice using the Opening Minds Stigma Scale for Healthcare Providers (OMS-HC) and Evidence-Based Practice Attitude Scale (EBPAS). We conducted a cross-sectional study across 12 primary health care centers and a psychiatric hospital (the country's main mental health care facility). A self-report questionnaire was distributed among all health care providers. A total of 547 health care providers participated, with 274 from mental health services and 273 from primary care services. Results of the OMS-HC indicated differences between both main groups and subgroups. Regression model analysis reported significant outcomes. There was no statistical difference found between both groups in EBPAS scores. A weak but statistically significant negative association was reported between both scales. Participants showed varying stigma attitudes across different working environments, with less stigma shown in mental health services than in primary care services. Providers who were more open to adopting evidence-based practices showed less stigma toward people with mental illness. Comparing our findings with previous research showed that health care providers in Bahrain hold more stigma attitudes than other groups studied. We hope that this study serves as an initial step toward future campaigns against the stigma of mental illness in Bahrain and across the region., Competing Interests: The authors have declared that no competing interests exist.

Objective: Becker's nevus (BN) is a benign cutaneous hamartoma. Although different types of lasers have been used to treat the pigmentary part of BN, to date, no definite treatment modality has been determined. The objective of this study is to evaluate the efficacy and safety of erbium: yttrium aluminum garnet (Er: YAG) laser treatment among Saudi patients with BN., Patients and Methods: A series of 10 patients (skin phototypes III-V) with BN underwent treatment with an Er: YAG laser. Biopsies were taken from all patients to confirm the diagnosis. Treatment consisted of using a 2940 nm Er: YAG laser (fluence 700 mj/cm 2 , short pulse, 3 mm spot size). Each patient underwent one session with three to five passes (the endpoint of passes was pinpoint bleeding). Clinical outcome was assessed using digital photographic method, before each treatment session and after the final visit. Clinical assessment scores were determined at each follow-up visit., Results: Seven patients completed the study. All patients noticed a significant reduction in pigmentation. Five of the patients had good improvement, and the remaining two had moderate improvement. The mean reepithelialization time was 7 days, and post-treatment erythema was seen in all patients (mean follow-up of 6 weeks). Post-inflammatory hypopigmentation was observed in all patients. No untoward sequelae were observed during the study or follow-up period., Conclusion: Er: YAG laser therapy can be an efficient modality for reducing the pigmentary part of BN. No scar formation, significant dyspigmentation, or recurrences were observed after 1 year.

Background: Hepatic resection is a major surgical procedure. Data on outcomes of hepatectomy in Saudi Arabia are scarce., Objective: To measure morbidity and mortality and assess predictors of outcome after hepatectomy., Design: Descriptive study., Setting: Tertiary care center in Saudi Arabia with well established hepatobiliary surgery unit., Patients and Methods: All patients undergoing liver resection in our institute during 2006-2014. Data were analyzed by Kaplan-Meier survival analysis., Main Outcomes Measure(s): Postoperative morbidity and 90-day mortality. Secondary outcomes were risk factors associated with increased morbidity and mortality., Results: Data on 77 resections were collected; 56 patients (72.7%) had a malignant etiology, mainly colorectal liver metastases and hepatocellular carcinoma (45.5% and 14.3% respectively). Complications developed following 30 resections (39.0%), with the majority being Clavien grades I-III. In the univariate analysis, predicting factors were the total bilirubin level preoperatively, operative time, extent of resection (i.e., major resection), use of epidural anesthesia, and postoperative liver dysfunction. In the multivariate analysis, the Schindl liver dysfunction score showed the strongest correlation with the development of complications (P=.006). The 90-day postoperative mortality was 5.2% (4/77 patients); 3 patients fulfilled the 50:50 liver dysfunction criteria. Significant predictors were concurrent intra-abdominal surgery, postoperative liver dysfunction, and multiple complications., Conclusion: Factors that predicted development of complications were elevated total bilirubin level preoperatively, operative time, extent of the resection, use of epidural anesthesia and a postoperative need for blood transfusion. Liver resection is a safe and feasible option at our center., Limitations: The small number of indications for resection and consequent reduction in variety of risk factors limited ability to make inferences. Additionally, only a handful of cases were performed laparoscopically.

Dermatopathia pigmentosa reticularis (DPR) is a very rare autosomal dominant ectodermal dysplasia caused by mutations in keratin 14 and characterized by the triad of generalized reticulate hyperpigmentation, nonscarring alopecia, and onychodystrophy. We report two Saudi brothers with DPR that had normal hair shafts, as assessed by scanning and transmission electron microscopy.

Objectives: Immunogenetic factors are known to play a role in the pathogenesis of alopecia areata (AA). This study aimed at investigating the association between AA with the polymorphisms of interleukin-4 (IL-4) promoter and receptor (IL-4R) genes., Patients and Methods: This work is a case-control study that was conducted on 76 AA patients from Qassim region, Saudi Arabia. Patients were compared with 93 normal healthy controls from the same locality. Genomic DNA was extracted and processed using real-time PCR amplification for characterization of IL-4 -590 T>C and IL-4R Q551R A>G gene polymorphisms., Results: Cases of AA showed a higher frequency of the IL-4 -590 CC homozygous genotype compared with controls (63.2 vs. 53.8%, P>0.05) with a lower frequency of the TT genotype (5.3 vs. 10.8%); yet, both were statistically nonsignificant (P>0.05). Regarding the IL-4R Q551R A>G polymorphism, cases and controls showed nearly equal frequencies of all variants, that is, with no significant difference. Although the frequency of the IL-4 C and the IL-4R A alleles was higher among cases than among controls (78.9 vs. 71.5% and 78.8 vs. 72.6%, respectively), this was also statistically nonsignificant (P>0.05). Comparing case subgroups in terms of their age of onset, sex, disease severity, consanguinity, and family history showed no statistically significant difference regarding the studied genetic variant., Conclusion: IL-4 -590 and IL-4R Q551R gene polymorphisms are not associated with the susceptibility and the clinical pattern of AA in Saudi patients. We recommend further research studies involving the estimation of cytokines both in the serum and in the local skin lesions or in cultured skin cells to figure out whether Th1 or Th2 pathways play a specific role in the pathogenesis of AA.

The success of a pediatric kidney transplantation program can only be judged by reviewing its results. We aim to audit our short-term outcome of pediatric kidney transplantation at the King Fahad Specialist Hospital-Dammam. A retrospective chart review was performed to collect data about recipient demographics, etiology of end-stage kidney disease, type of dialysis, type of donor and outcome. Between September 2008 and April 2012, 35 pediatric kidney trans-plantations (<16 year) were performed of a total of 246 kidney transplants (14.2%). The mean age was 8.1 years, with a mean weight of 23.3 kg, and there were 21 (60%) boys in the study. Kidney dysplasia/hypoplasia was the most common etiology (51.4%). Preemptive kidney transplantation was performed in six (17%) patients. Peritoneal dialysis was the most common mode of dialysis [24 (69%) children]. Living donation was the source of kidney allografts in 13 (37%) cases. During a mean follow-up of 1.5 years, one patient died and one graft was lost due to kidney vein thrombosis. The one year patient and graft survival rates were 97% and 94%, respectively. Efforts should now be focused on achieving optimal long-term results. There is also a need to encourage preemptive transplantation and living donation in this population.

Background: Vitiligo is a depigmenting skin disorder in which genetic factors play an important role., Objective: To examine the association of CYP2C9 (*) 1/(*) 2/(*) 3 gene polymorphism with vitiligo., Methods: In this case controlled study, 95 Saudi patients with vitiligo (50 men and 45 women), with a mean age of 27.3 years, were analyzed. Patients were compared to 86 healthy controls from the same locality (76 men and 10 women), with a mean age of 20.1 years. In all participants, DNA was extracted and processed for characterization of 2C9 (*) 1/(*) 2/(*) 3 gene variants using real time-polymerase chain reaction., Results: Vitiligo patients have a significantly higher CYP2C9 (*) 3 allele carriage rate compared to controls (32.7% versus 4.7%, p=0.00, odds ratio=9.9, 95% confidence interval=3.3~29.6). On the other hand, frequencies of CYP2C9 (*) 2 genotypes and alleles did not show any significant difference between vitiligo cases and controls. When the frequencies of CYP2C9 genotypes were compared among subgroups of age, gender, family history, and disease patterns, the cases with positive consanguinity had significantly higher frequencies of homozygous genotypes than others (p=0.029)., Conclusion: CYP2C9 (*) 3 allele carriage is probably associated with vitiligo susceptibility.

Background: Alpha-fetoprotein (AFP) levels for the diagnosis of hepatocellular carcinoma (HCC) may vary by geographical region and racial background. No data exists for this test in the Middle Eastern population. In addition, there is limited data on the impact of virological status on AFP levels., Methods: In a multicenter, case-control study involving 206 cases, 199 cirrhotic and 197 chronic hepatitis controls, we assessed the utility of AFP in the diagnosis of HCC (sensitivity, specificity, positive (PPV) and negative (NPV) predictive values, and positive likelihood ratios (LR). PPV and NPV were evaluated for three additional HCC prevalence rates (5, 10, and 20%)., Results: The best discriminating AFP value was 11.7 ng/ml. The sensitivity ranged from 32 to 79.5% at different AFP levels with the specificity increasing sequentially from 47.7 to 98.5%. Sensitivity of AFP at the best cut-off level for hepatitis C virus (HCV), hepatitis B virus (HBV) and non-viral etiology for HCC was 73.7, 65.6, and 59.5%, respectively. Specificity at this level for HCV, HBV, and non-viral etiology was 36.6, 30.1, and 29.4%, respectively. AFP cut-off levels of 102, 200, and 400 ng/ml showed similar sensitivity (39.8, 35.9, and 32%, respectively) and specificity (96, 98.5, and 98.5% respectively). Positive LR for AFP at >11.7, >20, >102, >200, >400 ng/ml were 2.8, 3.3, 9.9, 23.8, and 21.2, respectively., Conclusions: In cirrhotic patients, AFP has a poor screening and diagnostic value for HCC. Underlying viral etiology fails to influence the diagnostic accuracy of this test. An AFP level greater than 100 ng/ml has a high degree of specificity and may be used as a confirmatory test.

Although insulin independence is maintained in most islet recipients at 1 yr after transplant, extended follow-up has revealed that many patients will eventually require insulin therapy. Previous studies have shown that islet autografts are prone to chronic failure in large animals and humans, suggesting that nonimmunological events contribute to islet graft functional decay. Early intervention with therapies that promote graft stability should provide a measurable benefit over time. In this study, the efficacy of the long-acting glucagon-like peptide-1 analog liraglutide was explored in a porcine marginal mass islet autograft transplant model. Incubation with liraglutide enhanced porcine islet survival and function after prolonged culture. Most vehicle-treated (83%) and liraglutide-treated (80%) animals became insulin independent after islet autotransplantation. Although liraglutide therapy did not improve insulin independence rates or blood glucose levels after transplant, a significant increase in insulin secretion and acute-phase insulin response was observed in treated animals. Surprisingly, no evidence for deterioration of graft function was observed in any of the transplanted animals over more than 18 months of follow-up despite significant weight gain; in fact, an enhanced response to glucose developed over time even in control animals. Histological analysis showed that intraportally transplanted islets remained highly insulin positive, retained alpha-cells, and did not form amyloid deposits. This study demonstrates that marginal mass porcine islet autografts have stable long-term function, even in the presence of an increasing metabolic demand. These results are discrepant with previous large animal studies and suggest that porcine islets may be resistant to metabolic failure.

Purpose: Whereas intussusception is relatively common in children, it is clinically rare in adults. The condition is usually secondary to a definable lesion. This study was designed to review adult intussusception, including presentation, diagnosis, and optimal treatment., Methods: A retrospective review of 22 cases of intussusception occurring in individuals older than aged 18 years encountered at two university-affiliated hospitals in Winnipeg between 1989 and 2000. The 22 cases were divided to benign and malignant enteric, ileocolic, colonic lesions respectively. The diagnosis and treatment of each case were reviewed., Results: There were 22 cases of adult intussusception. Mean age was 57.1 years. Abdominal pain, nausea, and vomiting were the commonest symptoms. There were 14 enteric, 2 ileocolic, and 6 colonic intussusceptions. Eighty-six percent of adult intussusception was associated with a definable lesion. Twenty-nine percent of enteric lesions were malignant. All ileocolic lesions were malignant. Of colonic lesions, 33 percent were malignant and 67 percent were benign. All cases required surgical interventions except one., Conclusions: Adult intussusception is a rare entity and requires a high index of suspicion. Our review supports that small-bowel intussusception should be reduced before resection if the underlying etiology is suspected to be benign or if the resection required without reduction is deemed to be massive. Large bowel should generally be resected without reduction because pathology is mostly malignant.

Background: Linear IgA bullous dermatosis of childhood is a rare autoimmune bullous disease that mainly affects preschool-aged children. Dapsone is considered the first-line therapy with prompt response from most patients. However, it may be contraindicated in certain conditions such as glucose-6-phosphate dehydrogenase deficiency., Objective: We sought to assess the efficacy of flucloxacillin in the treatment of linear IgA bullous dermatosis., Methods: This is an observational study in which all confirmed cases of linear IgA bullous dermatosis (by both histological and immunofluorescence studies) will be treated with flucloxacillin. Flucloxacillin will be continued according to the response or otherwise will be discontinued after 8 weeks in the case of resistance., Results: We describe 7 patients with linear IgA bullous dermatosis of childhood treated with flucloxacillin. In 4 cases, it induced complete remission within 3 to 4 months of starting therapy with no relapses. In the other 3 cases, it successfully controlled the disease but with prompt relapse on discontinuation of the treatment., Limitations: This is a case series study with a small number of patients., Conclusion: Flucloxacillin may be considered among the first alternative therapies for linear IgA bullous dermatosis of childhood. Further evaluation of the efficacy and safety of the long-term use is required.

Islet transplantation is being offered increasingly for selected patients with unstable type 1 diabetes. Percutaneous transhepatic portal access avoids a need for surgery, but is associated with potential risk of bleeding. Between 1999 and 2005, we performed 132 percutaneous transhepatic islet transplants in 67 patients. We encountered bleeding in 18/132 cases (13.6%). In univariate analysis, the risk of bleeding in the absence of effective track ablation was associated with an increasing number of procedures (2nd and 3rd procedures with an odds ratio (OR) of 9.5 and 20.9, respectively), platelets count <150,000 (OR 4.4), elevated portal pressure (OR 1.1 per mm Hg rise), heparin dose > or =45 U/kg (OR 9.8) and pre-transplant aspirin (81 mg per day) (OR 2.6, p = 0.05). A multivariate analysis further confirmed the cumulative transplant procedure number (p < 0.001) and heparin dose > or =45 U/kg (p = 0.02) as independent risk factors for bleeding. Effective mechanical sealing of the intrahepatic portal catheter tract with thrombostatic coils and tissue fibrin glue completely prevented bleeding in all subsequent procedures (n = 26, p = 0.02). We conclude that bleeding after percutaneous islet implantation is an avoidable complication provided the intraparenchymal liver tract is sealed effectively.

Sirolimus (SRL) has been used for most islet recipients over the past 5 years. It provides balanced immunosuppression in combination with low-dose calcineurin inhibitors, while avoiding corticosteroids. This regimen decreases the risk of nephrotoxicity, neurotoxicity and diabetogenicity. SRL has also been used selectively in clinical liver and kidney transplantation. A number of common side effects including anemia, leucopenia, thrombocytopenia, hypercholesterolemia, mouth ulceration, joint pain, extremity edema and impaired wound healing have been associated with the use of SRL. As SRL is used more frequently, evidence has been gathered on its rare but severe side effects. We report 2 patients who underwent islet transplantation and developed symptomatic small bowel ulceration that resolved after complete withdrawal of SRL. Although small bowel ulceration is rare, it can potentially progress to more serious complications if not treated adequately. Our experience highlights an uncommon but potentially serious adverse effect of high-dose SRL in islet recipients.