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Background: Functional constipation (FC) is one of the most common complaints in childhood. The aim of this study was the evaluation of the quality of life in children with chronic FC and their family members. Methods: In this study, 100 children from 2-18 years of age (57 males, 43 females) with chronic FC were selected. The Child Health Questionnaire - Parent Form 50 questionnaire was used for quality of life assessment in children and their family members. Quality of life scores were compared with 100 healthy children from 2-18 years of age. The questionnaire consists of 15 domains, each of which ranges in score from zero to 100. A higher score represents a better quality of life. Results: Quality of life scores in all 15 domains were significantly lower in children with FC than in healthy children (P

International journal of fuzzy systems : IJFS (2021). doi:10.1007/s40815-021-01128-4, Published by Chinese Fuzzy Systems Association, Taibei

Model for end-stage liver disease (MELD) is currently used for liver transplantation (LT) allocation, however, it is not a sufficient criterion.This current study aims to perform a hybrid neural network analysis of different data, make a decision tree and finally design a decision support system for improving LT prioritization.In this cohort follow-up-based study, baseline characteristics of 1947 adult patients, who were candidates for LT in Shiraz Organ Transplant Center, Iran, were assessed and followed for two years and those who died before LT due to the end-stage liver disease were considered as dead cases, while others considered as alive cases. A well-organized checklist was filled for each patient. Analysis of the data was performed using artificial neural networks (ANN) and support vector machines (SVM). Finally, a decision tree was illustrated and a user friendly decision support system was designed to assist physicians in LT prioritization.Between all MELD types, MELD-Na was a stronger determinant of LT candidates' survival. Both ANN and SVM showed that besides MELD-Na, age and ALP (alkaline phosphatase) are the most important factors, resulting in death in LT candidates. It was cleared that MELD-Na23, age53 and ALP257 IU/L were the best predictors of survival in LT candidates. An applicable decision support system was designed in this study using the above three factors.Therefore, Meld-Na, age and ALP should be used for LT allocation. The presented decision support system in this study will be helpful in LT prioritization by LT allocators.

Introduction: Inflammatory bowel disease (IBD) is a disorder of unknown etiology categorized into three groups including Crohn disease (CD), ulcerative colitis [UC], and intermediate colitis (IC). In addition to gastrointestinal (GI) symptoms, childhood IBD frequently present with extra GI manifestations. In present study, we aimed to determine extra GI symptoms in children with IBD in Iran. Methods: Children

Background and objectives Helicobacter pylori (H. pylori) infection has been assumed to have roles in various extra-digestive diseases. The current study was designed to evaluate the incidence of H. pylori infection in patients with cyclic vomiting syndrome and its possible role in the etiology of this disease. Design and setting In this case-control study, 120 cases with diagnoses of cyclic vomiting or abdominal migraine who were registered at the Gastroenterology Clinic at Shiraz University of Medical Sciences from 2010 to 2013 were enrolled. Materials and methods Primarily information regarding the patients' diseases were collected with a data gathering sheet, and fresh morning stool samples were collected from the patients and examined for H. pylori stool antigen with the H. pylori Ag EIA test kit. The results were compared with those of healthy children from the control group. Results A total of 120 patients with cyclic vomiting (47.5%) and abdominal migraine (52.5%) with a mean age of 7.1 ± 3.4 (range 2–16 years) and a male-to-female ratio of 1.6 were included. The HPs Ag tests were positive in only 7 (5.8%) patients in our case group, and the HPs Ag tests were positive in 13 (13%) of the children in the control group; this difference was statistically insignificant. Conclusion Our study did not support H. pylori infection as an etiological factor in CV or AM.

Background and Objective: This study investigates epidemiologic and practical information about the incidence and risk factors of childhood cancer in a population of Southern Iranian children. Materials and Methods: A total number of 300 cancer patients along with 600 age- and gender-matched healthy control were interviewed by a trained physician regarding their demographic characteristics, and major family-associated risk factors, childhood malignancies. Results: The average annual percentage change for cancers in the studied population is calculated as 45%. Our study indicated that possible risk factors which could contribute to the development of childhood cancer are maternal oral contraceptive pill use during pregnancy, exposure to radiation during pregnancy, parental smoking, residence near high voltage electricity lines, exposure to pesticides and fertilizers, patient allergy, contact with domestic animals and father's educational degree. Furthermore, new ecological risk factors such as air pollution due to nonstandard petroleum or toxic inhalant particles, nonhealthy food consumption, and satellite jamming are other predisposing factors. Conclusion: Our study reported a higher average annual percentage change of childhood cancers in our area, compared to the existing literature. In conclusion, detection and prevention of the consistent and possible new environmental risk factors such as nonstandard petroleum or satellite jamming from all around the country should be taking into consideration.

Background: Wilson disease (WD) is an autosomal recessive progressive degeneration of hepatolenticular tissue that causes the increase of copper deposition in the liver and other organs, with resultant hepatic, neurologic and psychological manifestations. WD is fatal if left untreated. The aim of the current study was to evaluate the clinical and Para-clinical findings in children with WD in Shiraz, Southern Iran.Patients and Methods: The Medical records of all children less than 18 years of age with definite diagnosis of WD, who were admitted in Nemazee Teaching Hospital from 2001 to 2009, or were under follow up at the Pediatric Hepatology Clinic affiliated to Shiraz University of Medical Sciences, were reviewed.Results: Overall, 70 patients with WD (41 males, 29 females) were studied. The mean age at the onset of diagnosis was 10.3±3.2 years and the most common first presentation in our patients was hepatic (90%). The most common biochemical abnormalities were increased urinary copper content, increased liver enzymes (92.9%), and increased prothrombin time (71.4%). Wilson index was ≥11 in 44.3% of the patients.Conclusion: WD is a rare and fascinating disorder that often poses a diagnostic and therapeutic challenge for the physician. Maintaining a high index of suspicion is critical in diagnosing this readily treatable disease and early treatment can decrease its mortality rate.

Background. Efforts have been made worldwide to improve awareness and treatment of primary immunodeficiency (PID). This has also gained momentum on the African continent albeit at a slower pace. Objective. This review reports on the current status of PID on the African continent regarding its prevalence, distribution, genetic mutations and challenges in diagnosis and treatment of affected patients. Method. We evaluated all studies published from the African continent in the field of PID dealing with prevalence, epidemiology, case reports and genetic findings. Results. The prevalence of PID on the African continent has been estimated to be as high as 902 631 individuals. PID still is mostly underdiagnosed in Africa and although progress has been made in parts of the continent many challenges still remain regarding awareness, diagnosis, registration and care of these patients. Conclusion. Given the unique genetic mutations reported in PID patients on the African continent and the feasibility of hematopoietic stem cell transplantation and gene therapy, increased awareness should be encouraged and new therapeutic options considered.

Background: Alanine aminotransferase (ALT) level is the most common diagnostic test used for detection of liver damage; however in the past few years some studies have questioned the normal aminotransferase range in adults and have suggested its revision. Objectives: In this study, we assessed the upper limit of ALT and aspartate transaminase (AST) in a healthy population of Iranian children. Patients and Methods: A total of 500 apparently healthy children, aged 1 - 15 years, were enrolled in the study. Each patient's serum AST, ALT, triglyceride, cholesterol, and fasting blood sugar were checked. Participants with low or high body mass indexes as well as abnormal lipid profiles and fasting blood sugars were excluded from the study. Results: A total of 340 subjects with a mean age of 9.2 3.5 years were included in the study. According to our study we reported the 95th percentile of ALT values, corresponding to the upper limit of normal in healthy individuals, as 21 U/L for children. Furthermore, the 95th percentile of AST values, corresponding to the upper limit of normal range in healthy children, was 29 U/L. Conclusions: A clearly lower cutoff of normality for liver enzyme values in children of our population was demonstrated in this study. However, age and gender were not found to be determining factors for upper limit of normal range for ALT in children.

Mutations in podocin (NPHS2) gene have the key role in the pathogenesis of steroid-resistant nephrotic syndrome (SRNS) in children, but data is scarce regarding their prevalence and natural course among different all ethnic groups. This study was aimed to demonstrate the spectrum of NPHS2 mutations in children with SRNS and to compare the clinical course of disease in patients with and without mutation.All 8 exons of NPHS2 were sequenced in 99 children, including 49 with SRNS and 50 with steroid-sensitive nephrotic syndrome (control group) by DNA sequencing.The prevalence rates of NPHS2 gene mutation among children with SRNS and SSNS were 31% and 4%, respectively. The prevalence rates of mutation among familial and sporadic forms were 57% and 26%, respectively. Thirty-three percent of the children experienced recurrence of primary disease after kidney transplantation, none of whom had a mutation. The clinical response to treatment was poorer in children with mutation in comparison with patients without mutation (12% versus 32%, respectively; odds ratio, 3.29, 95% confidence interval, 0.40 to 25.64). Patients with and without mutation could not be differentiated by demographic and histological features, glomerular filtration rate at onset, hypertension, progression to end-stage renal disease, and proteinuria.Mutations of NPHS2 gene are frequent among Iranian children with SRNS. Regarding similar clinical features in patients with and without mutation and poor response to pharmacotherapy in patients with mutation, a molecular approach might be necessary for different treatment plans and prediction of prognosis.

ObjectivesHelicobacter pylori (H. pylori) cure rates vary in different geographical regions because of differences in hosts as well as in H. pylori strains. In this study we evaluated the efficacy of different treatment regimens for eradication of H. pylori infection in children, in order to select a treatment regimen that is most effective with the least adverse effects and cost. Method Through a randomized clinical trial study we enrolled 120 pediatric patients (age ≤ 18 years) with H. pylori infection confirmed through histopathological examination of their upper endoscopic findings and positive rapid urease test. Patients were randomized into three groups: group A received omeprazole, amoxicillin, metronidazole, and bismuth subcitrate; group B received omeprazole, amoxicillin, and clarithromycin; and group C the most recent regime of omeprazole, amoxicillin-clavulanic acid, and metronidazole. Subjects were followed 6 weeks after completing the antimicrobial therapy and H. pylori eradication was assessed with urea breath test. Results A total of 117 patients with a mean age of 12 ± 4 years completed the study. Eradication rate was 91.9% in group A, compared with 82.1% in group B, and 80.5% in group C (P = 0.33). Conclusion Considering these data we suggest quadruple therapy as the first line of therapy for eradication of H. pylori infection in children in our geographic area (Iran).

We reviewed records of 35 pediatric liver transplant recipients who were operated at the Shiraz Organ Transplant Center between April 1998 and April 2005 to gather demographic data, primary diagnosis, duration of hospital stay, source of graft, mortality, and surgical (vascular, biliary, fluid collection) and medical (infection, respiratory, neurological, cardiovascular, and gastrointestinal) complications. Among 23 male and 12 female pediatric liver transplant recipients (mean age: 11.8+/-4.9 years) with a mean hospital stay duration of 23.3+/-20.3 days, the postoperative complications included biliary leakage (7.20%), biliary stricture (3.10%), biliary obstruction (3.10%), pleural effusion (9.26%), lung collapse (n=1) pulmonary hemorrhage (n=1), and vascular complications of portal and hepatic vasculature (n=10, 28.6%); and infections of the peritoneum, lung, wound site, and urinary tract (n=10; 28.6%). Acute cellular rejection was documented in 6 (17.1%) recipients. Overall, 13 (37.1%) children died.