A comparative study on ascetic fluid biochemical markers in cirrhotic children with and without spontaneous bacterial peritonitis: A cross-sectional observation

Background: Cirrhotic ascites is frequently complicated with spontaneous bacterial peritonitis (SBP). Objectives: Here, we assessed the diagnostic validity of some inflammatory markers in children with SBP. Methods: This cross-sectional study was performed in the Pediatric Gastroenterology Ward of Namazi hospital of Shiraz. SBP was considered as ≥ 250 polymorphonuclear cell (PMN) /µL ascetic fluid (AF). Results: Out of 150 liver cirrhotic patients with ascites, 41 (27.3) were diagnosed with SBP. Mean WBC count and PMN per µL of AF were significantly higher in SBP patients than non-SBP (P < 0.0001). The highest specificity for the diagnosis of SBP was recorded for AF smear test (100). Positive AF smear and culture tests rendered the highest positive predictive values (PPV, 100 and 88.2 respectively). The highest negative predictive value (NPV) was related to AF leukocyte esterase test (94.2). The highest area under the curve (AUC) for diagnosis of SBP belonged to WBC count (0.956), total cell count (0.817), and LDH concentration (0.814) of AF. A cut off value of 290 WBC per µL AF resulted in respective 95.1 and 88.1 sensitivity and specificity. Trespass of WBC count and lactate dehydrogenase (LDH) level of AF higher than cutoff values (290 cells/µL, and 175 (u/L), respectively) resulted in 82.9, 92.7, 81, and 93.5 sensitivity, specificity, PPV and NPV, respectively. Conclusions: Total cell and WBC counts of AF strongly correlated with ascetic PMN count. Ascetic LDH, alone or in combination with WBC count of AF can be used as a potential surrogate for PMN counts in diagnosis of SBP. © 2019, Journal of Comprehensive Pediatrics

Dietary compliance in Iranian children and adolescents with celiac disease

Maryam Taghdir,1 Naser Honar,2 Seyed Mohammad Mazloomi,3 Mojtaba Sepandi,4 Mahkameh Ashourpour,1 Musa Salehi5 1Student Research Committee, Department of Clinical Nutrition, School of Nutrition and Food Sciences, Shiraz University of Medical Sciences, Shiraz, Iran; 2Department of Pediatric Gastroenterology and Hepatology, Shiraz University of Medical Sciences, Shiraz, Iran; 3Nutrition Research Center, Department of Food Hygiene and Quality Control, School of Nutrition and Food Sciences, Shiraz University of Medical Sciences, Shiraz, Iran; 4Department of Epidemiology and Biostatistics, Baqyiatallah University of Medical Sciences, Tehran, Iran; 5Nutrition Research Center, Department of Clinical Nutrition, School of Nutrition and Food Sciences, Shiraz University of Medical Sciences, Shiraz, Iran Introduction: Celiac disease (CD) is caused due to intake of gluten, a protein component in wheat, barley, and rye. The only treatment currently available for CD is strict lifetime adherence to a gluten-free diet (GFD) which is a diet that excludes wheat, barley, and rye. There is limited information on barriers to following a GFD. The present study aimed to investigate the compliance with a GFD, barriers to compliance, and the impact of compliance on the quality of life (QOL) in Iranian children and adolescents suffering from CD.Methods: In this cross-sectional study, a total of 65 known cases of CD (both males and females), diagnosed in Namazi Hospital, a large referral center in south of Iran, selected by census were studied in 2014. Dietary compliance was assessed using a questionnaire. A disease-specific QOL questionnaire for children with CD (the celiac disease DUX [CDDUX]) was used. Comparisons between categorical variables were performed using chi-square test.Results: Sixty-five patients, 38 females (58.5%) and 27 (41.5%) males, were surveyed. Mean (&plusmn; standard deviation [SD]) age of the respondents was 11.3 (&plusmn;3.8) years. Dietary compliance was reported by 35 (53.8%) patients. The mean (&plusmn; SD) CDDUX score was higher in dietary-compliant patients (33.5 [&plusmn;19.4] vs 26.7 [&plusmn;13.6], respectively, P=0.23). The score of CDDUX in parents of patients in dietary-compliant group was more than the noncompliant patients (28.1 [&plusmn;13.5] vs 22.1 [&plusmn;14], respectively, P=0.1). Barriers to noncompliance were poor or unavailability (100%), high cost (96.9%), insufficient labeling (84.6%), poor palatability (76.9%), and no information (69.23%).Conclusion: Approximately half of the patients with CD reported dietary compliance. Poor or unavailability was found to be the most important barrier contributing to noncompliance. The QOL was better in compliant patients. Proposed strategies to improve compliance are greater availability of gluten-free products, better food labeling, and better education about the diet and condition. Keywords: child, gluten enteropathy, adherence, gluten-free diet, quality of lif

Caustic ingestion in children in south of Iran. Retrospective study from Shiraz - Iran

Caustic ingestion is a major health concern in both developed and developing countries, that may lead to serious esophageal injury. The clinical presentation of caustic ingestion in children vary from asymptomatic to serious and fatal sequelae, such as perforation and stricture formation. OBJECTIVE: Due to the lack of a comprehensive study in our area, this study has evaluated clinical and endoscopic manifestations and complications of caustic ingestion in children in south of Iran. MATERIALS AND METHODS: In this retrospective study, we reviewed 75 children with caustic ingestion who admitted in Nemazee Hospital of Shiraz University of Medical Science during 6 years (2006-2011). Sign and symptoms were recorded for each case. RESULTS: The most common symptoms were dysphagia, oral lesions, vomiting, and drooling. Esophageal injuries were detected in both acid and alkali ingestion, but gastric injuries was significantly more in acid ingestion. During follow up period, 20 of all cases developed esophageal stricture. CONCLUSION: Dysphagia, oral lesions, vomiting, and drooling were the most common findings.Esophageal stricture was found in 20 of cases during 3 months of follow up

Complications After endoscopic balloon dilatation of esophageal strictures in children. Experience from a tertiary center in Shiraz - Iran (Nemazee Teaching Hospital)

BACKGROUND: Esophageal stricture is one of the most important complication of the caustic ingestion. OBJECTIVE: The aim of this study was to evaluate complications of balloon dilatation among children with esophageal stenosis. MATERIAL AND METHODS: In this retrospective study 82 children were included. Children who underwent balloon dilatation for esophageal stenosis were included in our study. Duration of study was 14 year starting from 2001. Mean age of the cases was 3.95+/-0.4 year (Min: 15 days, Max: 14 year). Chart review and telephone calling were the methods of data collection. Data was analyzed using SPSS. RESULTS: In this study, 47 of the patients were male and 53 of the cases were female. Caustic ingestion (33.7) was the most common etiology for the esophageal stricture. Vomiting (87.8) was the most common presenting symptom. Among our cases, 76.8 had no compliant after esophageal dilatation. Chest pain was the most common compliant after esophageal dilatation. Response rate was similar among boys and girls. Toddler age had the best treatment response after esophageal dilatation. CONCLUSION: Among our cases, 76.8 had no post procedural compliant after esophageal dilatation. Esophageal perforation was seen in 4.9 of the cases. Chest pain was the most common post dilatation complication

Complications After endoscopic balloon dilatation of esophageal strictures in children. Experience from a tertiary center in Shiraz – Iran (Nemazee Teaching Hospital)

BACKGROUND: Esophageal stricture is one of the most important complication of the caustic ingestion. OBJECTIVE: The aim of this study was to evaluate complications of balloon dilatation among children with esophageal stenosis. MATERIAL AND METHODS: In this retrospective study 82 children were included. Children who underwent balloon dilatation for esophageal stenosis were included in our study. Duration of study was 14 year starting from 2001. Mean age of the cases was 3.95±0.4 year (Min: 15 days, Max: 14 year). Chart review and telephone calling were the methods of data collection. Data was analyzed using SPSS. RESULTS: In this study, 47 of the patients were male and 53 of the cases were female. Caustic ingestion (33.7) was the most common etiology for the esophageal stricture. Vomiting (87.8) was the most common presenting symptom. Among our cases, 76.8 had no compliant after esophageal dilatation. Chest pain was the most common compliant after esophageal dilatation. Response rate was similar among boys and girls. Toddler age had the best treatment response after esophageal dilatation. CONCLUSION: Among our cases, 76.8 had no post procedural compliant after esophageal dilatation. Esophageal perforation was seen in 4.9 of the cases. Chest pain was the most common post dilatation complication

Evaluation of Candida Infection after Six Months of Transplantation in Pediatric Liver Recipients in Iran

Background: Liver transplantation (LT) is the standard treatment of end-stage liver diseases (ESLD). Invasive fungal infection is one of the important causes of morbidity and mortality after transplantation.Objective: To determine the incidence of late-onset (after 6 months of LT) Candida infection in recipients.Methods: A retrospective study was conducted to evaluate 50 pediatric patients after LT for 8 years at the LT Unit of Nemazee Hospital affiliated to Shiraz University of Medical Sciences, Shiraz, Iran. We followed the patients until 6 months post-LT for episodes of Candida infection proven by culture.Results: One recipient (2%) developed late-onset esophageal candidiasis with improvement after intravenous amphotricin therapy but finally expired with a diagnosis of post-transplant lymphoproliferative disorder (PTLD).Conclusions: The incidence of late-onset Candida infection is not significant in pediatric liver recipient, but it still remains a significant problem. Control of Candida colonization would reduce the risk of invasive fungal infections and possibly more fatal complications

Targeting p53 in Vivo: A First-in-Human Study With p53-Targeting Compound APR-246 in Refractory Hematologic Malignancies and Prostate Cancer.

PURPOSEAPR-246 (PRIMA-1MET) is a novel drug that restores transcriptional activity of unfolded wild-type or mutant p53. The main aims of this first-in-human trial were to determine maximum-tolerated dose (MTD), safety, dose-limiting toxicities (DLTs), and pharmacokinetics (PK) of APR-246. PATIENTS AND METHODSAPR-246 was administered as a 2-hour intravenous infusion once per day for 4 consecutive days in 22 patients with hematologic malignancies and prostate cancer. Acute myeloid leukemia (AML; n = 7) and prostate cancer (n = 7) were the most frequent diagnoses. Starting dose was 2 mg/kg with dose escalations up to 90 mg/kg.ResultsMTD was defined as 60 mg/kg. The drug was well tolerated, and the most common adverse effects were fatigue, dizziness, headache, and confusion. DLTs were increased ALT/AST (n = 1), dizziness, confusion, and sensory disturbances (n = 2). PK showed little interindividual variation and were neither dose nor time dependent; terminal half-life was 4 to 5 hours. Tumor cells showed cell cycle arrest, increased apoptosis, and upregulation of p53 target genes in several patients. Global gene expression analysis revealed changes in genes regulating proliferation and cell death. One patient with AML who had a p53 core domain mutation showed a reduction of blast percentage from 46% to 26% in the bone marrow, and one patient with non-Hodgkin's lymphoma with a p53 splice site mutation showed a minor response. CONCLUSIONWe conclude that APR-246 is safe at predicted therapeutic plasma levels, shows a favorable pharmacokinetic profile, and can induce p53-dependent biologic effects in tumor cells in vivo