The role of antral histopathology in diagnosing paediatric celiac disease.

The aim of this study was to evaluate the diagnostic potential of gastric antrum histology in children suspected of having celiac disease (CD). The present retrospective study was performed on 224 patients who were suspected of having CD and had several duodenal and one gastric antrum biopsies. They were divided into 2 groups based on the definite diagnosis of CD. The statistical analysis was performed using SPSS version 22 software. Receiver operating characteristic (ROC) curves were drown and the area under the curves (AUCs) was calculated. Article authorized by the Editor-in-Chief. Based on MARSH criteria, out of 224 patients, 124 were diagnosed as definite CD and 100 patients comprised the non-celiac group. The AUC for the mean of all pathological tests was estimated to be 0.90 (p < 0.001). The pooled AUC for the combination of 3 pathologic findings with the highest AUCs (cell, crypt, and gland size) was estimated to be 0.89 (p < 0.001). We observed that the histological changes we found in the gastric antrum were identical to those found in the duodenum of paediatric CD patients. Because providing a biopsy from the gastric antrum is easier than getting multiple biopsies from the duodenum, we suggest using the criteria mentioned in this study in other studies with larger sample sizes.

Risk Factors of Nonsurgical Management Failure in Pediatric Intussusception Patients With Delayed Presentation.

OBJECTIVES: The present study aimed to investigate the time-related predicting factors of the ultrasound-guided hydrostatic reduction (USGHR) failure in pediatric patients with ileocolic intussusception and delayed presentation. METHODS: The present retrospective study included pediatric patients diagnosed with ileocolic intussusception who presented to our hospital with the related symptoms started 48 hours ago or greater duration during 2018-2020. The patients with spontaneous reduction were excluded from the study. Afterward, the participants with failed and successful USGHR were compared in terms of age, sex, symptom duration, and ultrasound findings using the χ 2 and logistic regression tests. RESULTS: A total of 103 children were included in the present study. The mean symptom duration was 4.13 ± 2.39 days, with a range of 2-14 days. Moreover, 47.6% of the patients had a successful reduction. In addition, there was a significant relationship between failed USGHR and the factors of symptom duration, free peritoneal fluid, entrapped fluid between intussuscepted loops, the size of the invaginated segment, and malperfusion of the intussuscepted bowel loops detected using the Doppler ultrasound ( P < 0.05). However, there was no significant relationship between failed USGHR and the factors of the primary location of intussusception and the presence of intussuscepted lymph nodes ( P > 0.05). CONCLUSIONS: The presence of entrapped fluid between the intussuscepted loops, free peritoneal fluid, and the length of the intussuscepted segments were all associated with USGHR failure in our study. Therefore, determining these predictors may help anticipate failure of reduction.

Prognostic Value of Porta-Hepatis Lymphadenopathy in Children with Hepatitis A.

Background: The present study aimed to investigate the prognostic value of porta-hepatis lymphadenopathy (PHL) in children with hepatitis A virus. Methods: The present prospective cohort study included 123 pediatric patients with a definite diagnosis of hepatitis A who were divided into two groups based on the presence or absence of PHL in their abdominal ultrasound: Group A included the patients with a porta-hepatis lymph node of >6 mm in diameter, whereas the patients with a porta-hepatis lymph node of <6 mm in diameter were classified in Group B. The patients were also classified based on the presence or absence of para-aortic lymphadenopathy: Group C had bisecting para-aortic lymph nodes, whereas Group D did not have such findings in their ultrasound. Afterward, the groups were compared in laboratory investigation results and hospital stay. Results: According to our results, Group A (n = 57) was significantly higher in aspartate and alanine aminotransferase and alkaline phosphatase levels compared to Group B (P < 0.05), whereas these two groups were not significantly different in the hospital stay. Furthermore, except bilirubin, all laboratory test results were significantly higher in Group C (n = 3) than in Group D. However, there was no significant correlation between the patients' prognosis with the absence or presence of porta-hepatis or para-aortic lymphadenopathy. Conclusion: We concluded that there was no significant relationship between porta-hepatis or para-aortic lymphadenopathy and the prognosis of the children with hepatitis A. However, ultrasound findings can help determine the disease severity in pediatric patients with hepatitis A.

The effects of prebiotic supplementation on weight gain, diarrhoea, constipation, fever and respiratory tract infections in the first year of life.

AIM: This study was conducted to evaluate the effect of prebiotics on some common clinical ailments in healthy term infants. METHODS: Sixty healthy-term, breastfed (BF) infants were included. Along with these infants, 120 healthy-term formula-fed infants were randomly assigned to either the prebiotic formula (PF, n = 60) or regular formula (RF, n = 60) groups. Ready-to-use prebiotic-supplemented formula containing galacto-oligosaccharides and polydextrose (ratio 1:1) was used. RESULTS: At 2 months of age, PF infants demonstrated significantly higher weight gain than BF and RF. At 6 months of age, bodyweight was significantly higher in the RF group compared to BF and PF groups (P < 0.05). Similar results were seen at 8, 10 and 12 months of age. At 10 months of age, the duration of diarrhoea was significantly shorter in PF-fed compared to the RF (P = 0.03) group. A significant difference was found between PF and RF (P < 0.0001) and BF and RF groups (P = 0.002) for diarrhoea duration. Means of constipation episodes per year were 0.03 ± 0.18, 0.433 ± 0.77 and 0.1 ± 0.30 for the BF, RF and PF groups, respectively, with significant difference found between BF and RF (P = 0.006) and PF and RF (P = 0.02). The means of episodes of respiratory tract infections per year for BF, RF and PF groups were 1 ± 0.69, 1.6 ± 0.88 and 1 ± 0.58, respectively (P = 0.01). CONCLUSION: Prebiotic-supplemented and regular formula were similar to breast milk regarding prophylactic effects for diarrhoea, constipation and respiratory tract infections in the first year of life. Prebiotic-supplemented formula may be an appropriate substitution for breast milk when breast milk in unavailable.

Impact of Intravenous Trehalose Administration in Patients with Niemann-Pick Disease Types A and B.

BACKGROUND AND AIMS: Niemann-Pick disease (NPD) types A (NPA) and B (NPB) are caused by deficiency of the acid sphingomyelinase enzyme, which is encoded by the SMPD1 gene, resulting in progressive pathogenic accumulation of lipids in tissues. Trehalose has been suggested as an autophagy inducer with therapeutic neuroprotective effects. We performed a single-arm, open-label pilot study to assess the potential efficacy of trehalose treatment in patients with NPA and NPB patients. METHODS: Five patients with NPD type A and B were enrolled in an open-label, single-arm clinical trial. Trehalose was administrated intravenously (IV) (15 g/week) for three months. The efficacy of trehalose in the management of clinical symptoms was evaluated in patients by assessing the quality of life, serum biomarkers, and high-resolution computed tomography (HRCT) of the lungs at the baseline and end of the interventional trial (day 0 and week 12). RESULTS: The mean of TNO-AZL Preschool children Quality of Life (TAPQOL) scores increased in all patients after intervention at W12 compared to the baseline W0, although the difference was not statistically significant. The serum levels of lyso-SM-509 and lyso-SM were decreased in three and four patients out of five, respectively, compared with baseline. Elevated ALT and AST levels were decreased in all patients after 12 weeks of treatment; however, changes were not statistically significant. Pro-oxidant antioxidant balance (PAB) was also decreased and glutathione peroxidase (GPX) activity was increased in serum of patients at the end of the study. Imaging studies of spleen and lung HRCT showed improvement of symptoms in two patients. CONCLUSIONS: Positive trends in health-related quality of life (HRQoL), serum biomarkers, and organomegaly were observed after 3 months of treatment with trehalose in patients with NPA and NPB. Although not statistically significant, due to the small number of patients enrolled, these results are encouraging and should be further explored.

Novel UBR1 gene mutation in a patient with typical phenotype of Johanson-Blizzard syndrome.

Johanson-Blizzard syndrome is a rare autosomal recessive disorder, characterized by exocrine pancreatic deficiency and a wide range of other abnormalities. We present here an infant with failure to thrive, exocrine pancreatic deficiency, short stature and developmental delay, cutis aplasia on the scalp, aplasia of alae nasi, hypospadias, hypothyroidism, myxomatous mitral valve, and patent ductus arteriosus. Molecular studies revealed a novel homozygous nonsense mutation in exon 38 of the UBR1 gene, which confirmed the diagnosis of Johanson-Blizzard syndrome. It should be acknowledged that the combination of exocrine pancreatic insufficiency and nasal wing hypo-aplasia is pathognomonic for this syndrome. Prompt diagnosis and exact monitoring of the patients with JBS are required to avoid further complications.

Discharge against medical advice in paediatric patients.

OBJECTIVES: Discharge against medical advice (DAMA) from the hospital may negatively impact patients' well-being. The present study aimed to investigate the main reasons for DAMA among parents of children admitted to the paediatric ward of the Ali Ebne Abitaleb Hospital in Zahedan, Iran. METHODS: Participants in this case-control study included 130 children who had been admitted to the hospital's paediatric ward. Participants were divided into two equal groups: (1) those with incomplete treatment and (2) those who stayed in the hospital until the completion of their management and followed regular discharge procedures. A self-administered questionnaire was utilised for data collection. RESULTS: Participants included 130 children aged <1-18 years with an average of 3.3 ± 3.7 years; 51.5% (67) were girls, and 48.5% (63) were boys. The results showed a significant relationship between DAMA and the father's level of education (p < 0.05), length of hospitalization (p < 0.001), and duration of treatment (p = 0.027). No significant correlation was found for other factors (p > 0.05). CONCLUSION: This study found that the key reasons for DAMA were as follows: lack of satisfaction with physicians and hospital staff, family disturbance due to the presence of other children at home, inadequate economic situation, and being away from home. Providing professional education to parents and expressing the benefits and disadvantages of refusing complete treatment may help parents make better decisions.

Clinical presentation of cystic fibrosis at the time of diagnosis: a multicenter study in a region without newborn screening.

BACKGROUND/AIMS: Cystic fibrosis is the most common inherited lethal disease, which could be frequently identified late in regions without newborn screening. There are dramatically better outcomes in the early diagnosis of cystic fibrosis patients. This study aimed to evaluate the spectrum of manifestations of cystic fibrosis at first admission leading to diagnosis. MATERIALS AND METHODS: This study was performed in a multi-referral pediatrics center in Iran. Data of patients with cystic fibrosis at the time of diagnosis were recorded based on a checklist denoting demographic characteristics, clinical and laboratory features. All of the patients had two documented sweat chloride tests. RESULTS: One hundred and ninety seven patients with cystic fibrosis were enrolled in this study. Among them, 119 patients (74%) were less than six months and 34 patients (21%) were between 6 and 12 months of age. The most common clinical findings were failure to thrive, recurrent pulmonary infections, and steatorrhea in 178 (90%), 139 (71%), and 135 (69%) patients, respectively. The most common radiologic abnormality was hyperaeration. In patients with salty tasting skin, steatorrhea, metabolic alkalosis, radiologic findings, and liver function abnormalities, the mean age at the time of diagnosis was significantly low than in the subjects without these findings. CONCLUSION: This study suggests that some conditions such as failure to thrive, recurrent respiratory infections, steatorrhea, metabolic alkalosis, and salty tasting skin should be considered as clinical screening tools for cystic fibrosis, especially in regions with high rate of cystic fibrosis. In these regions, awareness and clinical suspicion of medical professionals are crucial for early diagnosis of cystic fibrosis patients in the pre-diagnostic period.