Large Volume Paracentesis in Patients with Liver Cirrhosis Temporarily Diminishes Blood Cell Count.

Background: Large-volume paracentesis is the preferred treatment for patients with severe and refractory ascites. Several complications were reported during therapeutical paracentesis. However, there are very few published studies on the change in blood cell count after paracentesis. This study aimed to evaluate any changes in blood cell counts after ascites fluid drainage. Methods: This study was conducted on patients with severe ascites and chronic liver disease who underwent large-volume paracentesis at Namazi Hospital, in Shiraz, Iran, between March 2021 and February 2022. A data gathering form containing the patient's medical history, cause of cirrhosis, ascites fluid volume, as well as routine tests including primarily sodium, potassium, and basal creatinine, was filled out. Before and after the surgery, the total blood cell count was measured. Before the procedure, adjustment was made in the case of coagulopathy and albumin deficiency. The effect of factors such as the volume of drained fluid, splenomegaly, antibiotics, and steroid use was assessed on the changes in the number of blood cells. Using the JAMOVI 2.3.9 software, a paired t test and multiple regression were applied for statistical analysis (P<0.001). Results: The study included 37 patients. After the paracentesis procedure, the number of blood cells significantly decreased in all groups (P<0.001). The followings are the amounts of each type of blood cells before and after the procedure: Platelet=153837±91862 and 115648±69136, red blood cells=3.53±0.784 and 3.22±0.705, white blood cells=12.3±7.78 and 8.6±5.5. None of the study variables, including drained volume, splenomegaly, antibiotics, and steroid use, were significant predictors of the changes in the blood cell count after paracentesis (P>0.001). Conclusion: The findings of the present study showed that children with tense ascites who had large-volume paracentesis might experience a sharp drop in blood cell count after the procedure, which was a transient physiological condition.

Identification of a novel mutation in the ALDOB gene in hereditary fructose intolerance.

OBJECTIVES: Hereditary fructose intolerance (HFI) is caused by aldolase B enzyme deficiency. There has been no report about HFI from Iran and the type of mutations has not been reported in the Iranian population so far. CASE PRESENTATION: Herein we report a 2 year old girl presented with failure to thrive, hepatomegaly, and liver dysfunction. The primary impression has been hepatic glycogen storage disease type 1 or 6. This diagnosis was not confirmed by laboratory data and liver biopsy. Therefore, targeted-gene sequencing (TGS) covering 450 genes involved in inborn errors in metabolic diseases was performed. The results of TGS showed a rare novel homozygous pathogenic variant c.944del (p.Gly315ValfsTer15) in the ALDOB gene. CONCLUSIONS: This report introduces a novel variant that expands the mutational spectrum of the ALDOB gene in patients with HFI.

Do Serological Tests Eliminate the Need for Endoscopic Biopsy for the Diagnosis of Symptomatic Patients with Celiac Disease? A Retrospective Study with Review of Literature.

Background: Celiac disease is one of the most common genetic allergies worldwide. The prevalence of celiac disease in Iran is similar to or even higher than the global prevalence. Celiac disease is a chronic inflammatory disease that affects the small intestine. Affected patients are allergic to gluten protein that exists in some grains, such as wheat and barley. Methods: Serological endomysial IgA antibody (EMA-AB) and tissue transglutaminase IgA antibody (TTG-IgA) tests were performed on 114 patients aged the ages of 0-18 years with histopathological findings of celiac disease. The results of these tests were compared to the results of the histopathological study of the duodenal biopsy. Results: Based on the receiver operating characteristic (ROC) curve and a calculation of the TTG-IgA test's sensitivity and specificity, the best diagnostic limit for the TTG-IgA test is 144, which has the best sensitivity and specificity. At this value (cut-off), the test's sensitivity was 62%, and the specificity was 93.7%. For the endomysial test, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were 80%, 93%, 90%, and 75%, respectively. Conclusion: The diagnostic accuracy of the endomysial test is better than that of the TTG-IgA test in general for diagnosing patients with celiac disease. In the TTG-IgA test, false-positive cases are high due to a cut-off of 20, reducing the test's specificity. In these false-positive cases, the endomysial test helps in better diagnosis.

Rosa damascena together with brown sugar mitigate functional constipation in children over 12 months old: A double-blind randomized controlled trial.

ETHNOPHARMACOLOGICAL RELEVANCE: Rosa × damascena Herrm., known as damask rose, is a bushy shrub that is found abundantly in Fars province, Iran. This species has been used in Iranian traditional practices for the treatment of abdominal pain and constipation, as gastrointestinal diseases. Brown sugar (Saccharum officinarum L.) has also shown laxative effects in pediatric patients with functional constipation. AIM OF STUDY: This study aimed to compare the effects of Polyethylene Glycol (PEG) and a syrup made of R. damascena and brown sugar on the treatment of functional constipation in children aged above 12 months. MATERIALS AND METHODS: This double-blind randomized clinical trial was performed on 100 patients. One group received PEG and the other received an herbal syrup containing the decocted extract of 0.1 g R. damascena petals mixed with 0.85 g brown sugar per 1 mL. The patients were followed up for two and four weeks and their progresses were recorded. RESULTS: The cure rate was 100% in the R. damascena group and 91.7% in the control group. R. damascena and brown sugar syrup had an odds ratio of 1.09 in the treatment of functional constipation compared to PEG, but the difference was not statistically significant. The only adverse effect detected in the R. damascena group after four weeks was the bad taste of the medication that was too sweet. Nonetheless, this could be solved during the pharmaceutical processes. CONCLUSION: The R. damascena extract and brown sugar syrup can be used as an effective, safe, and inexpensive agent in the treatment of functional constipation.

Diagnostic Value of Immunoglobulin G Anti-Deamidated Gliadin Peptide Antibody for Diagnosis of Pediatric Celiac Disease: A Study from Shiraz, Iran.

Purpose: Screening serologic tests are important tools for the diagnosis of celiac disease (CD). Immunoglobulin (Ig)G anti-deamidated gliadin peptide (anti-DGP) is a relatively new autoantibody thought to have good diagnostic accuracy, comparable to that of anti-tissue transglutaminase (anti-tTG) antibody. Methods: Pediatric patients (n=86) with a clinical suspicion of CD were included. Duodenal biopsy, anti-tTG, and IgG anti-DGP antibody tests were performed. The patients were divided into CD and control groups based on the pathological evaluation of duodenal biopsies. The diagnostic accuracy of serological tests was determined. Results: IgA anti-tTG and IgG anti-DGP antibodies were positive in 86.3% and 95.4% of patients, respectively. The sensitivity, specificity, and diagnostic accuracy of the IgA anti-tTG test were 86.3%, 50.0%, and 68.6%, respectively, and those of the IgG anti-DGP test were 95.4%, 85.7%, and 90.7%, respectively. The area under the receiver operating characteristic (ROC) curve was 0.84 (95% confidence interval [CI], 0.74-0.91) for IgA anti-tTG test and 0.93 (95% CI, 0.86-0.97) for IgG anti-DGP test. The comparison of IgA anti-tTG and IgG anti-DGP ROC curves showed a higher sensitivity and specificity of the IgG anti-DGP test. Conclusion: IgG anti-DGP is a reliable serological test for CD diagnosis in children. High tTG and DGP titers in the serum are suggestive of severe duodenal atrophy. The combined use of IgA anti-tTG and IgG anti-DGP tests for the initial screening of CD can improve diagnostic sensitivity.

An evaluation of ascitic calprotectin for diagnosis of ascitic fluid infection in children with cirrhosis.

BACKGROUND: The most common infection in children with the hepatic disease with or without cirrhotic ascites is spontaneous bacterial peritonitis (SBP), which occurs in the absence of an evident intra-abdominal source of infection. The present study aims to assess the value of calprotectin in ascitic fluid in the diagnosis of ascitic fluid infection in children with liver cirrhosis. MATERIALS AND METHODS: In this cross-section study, 80 children with underlying liver disease who attended the Hepatology and Emergency Department in Shiraz University Hospitals were studied. All the patients were evaluated by a thorough history, clinical examination, laboratory investigations, diagnostic paracentesis with PMNLs count, and Calprotectin, which was measured in 1 mL ascitic fluid by ELISA. RESULTS: Thirty-five patients (43.75%) were diagnosed with ascitic fluid infection. Of these children 6 cases had positive ascitic fluid culture (SBP). Calprotectin was high in AFI patients with a statistically significant difference in AFI patients compared to non-AFI patients. The cut-off levels were 91.55 mg /L and the area under the curve was 0.971. So it can serve as a sensitive and specific diagnostic test for detection of AFI in children with underlying liver disease. CONCLUSION: Elevated ascitic calprotectin levels in cirrhotic patients are a diagnostic and reliable marker for the detection of AFI and are considered a surrogate marker for PMN.

Non-invasive monitoring associated with B lymphoma cells in post-transplant lymphoproliferative disorder (PTLD) patients: Systematic review.

BACKGROUND: One of the most severe side effects of solid-organ transplantation is posttransplant lymphoproliferative disease (PTLD). People with human immunodeficiency virus infection (HIV), an immunosuppressive disease comparable to HIV, have a higher chance of developing lymphoma when their peripheral blood contains elevated levels of the immunoglobulins kappa and lambda free light chains (FLCs). METHODS: This systematic review's objective was to monitor associated B lymphoma cells in PTLD patients. In order to find relevant studies published between 1/1/2000 and 1/9/2022, two independent researchers conducted searches (MT, AJ). A literature search of English language publications was conducted using MEDLINE through PubMed, EMBASETM through Ovid, the Cochrane Library, and Trip. In addition to Magiran and SID, we searched KoreaMed and LILACS for literature published in other languages. sFLC or PTLD, transplant, or Electrophoresis are terms used in the search strategy. RESULTS: A total of 174 studies were selected. After analyzing their correspondence with the required criteria, a final review of five studies was conducted. The manuscript presents current findings on the potential benefits of the clinical applicability of sFLCs in PTLD. While the preliminary results appear promising, the only consistent result is that early-onset PTLD is predicted within the first two years after transplant, a biomarker that could be used to diagnose the condition. CONCLUSIONS: Therefore, PTLD has been predicted by using the sFLCs. There have been contradictory results to date. Future research could include assessing the quantity of sFLCs and their quality in transplant recipients. In addition to PTLD and complications after transplantation, sFLCs may provide insight into other diseases. To confirm the validity of sFLCs, more studies are needed.

Evaluation of frequency of autoimmune hepatitis autoantibodies in children with type 1 diabetes / Frecuencia de anticuerpos de hepatitis autoinmune en niños con diabetes tipo I

Background: Diabetes mellitus type 1 (T1DM) is one of the childhood diseases with growing prevalence. Various accompanying autoimmune diseases were seen with type 1 diabetes. The most common autoimmune diseases with T1DM are autoimmune thyroiditis and celiac disease. In some reports, autoimmune hepatitis has been reported in association with DM-1. Objectives: The aim of this study was to evaluate autoimmune hepatitis autoantibodies in children with T1DM. Materials and methods: In this crosssectional study, 202 children with T1DM were evaluated (47.5% were males and 52.5% were girls). Liver enzymes, autoimmune hepatitis related autoantibodies such as anti-nuclear antibodies (ANA), anti-smooth muscle (ASMA) and anti liver and kidney microsomal antibodies (LKM-1) were measured. Liver ultrasound was done for participants and biopsy of liver was taken for children with increased echogenicity of the liver, hepatomegaly or elevated liver enzymes. Results analyzed by statistical software spss-16, Descriptive statistics and chi-square test, paired T-TEST. Level of less than 5% was considered statistically significant. Results: In 6 patients ANA and in 4 patients (2%) ASMA was positive,1 patient was ASMA positive but ANA negative. None of the patients were Anti LKM-1 positive. 3 patients had positive ANA and ASMA, and increased liver echogenicity on ultrasound simultaneously. Histological evaluation was showed that 2 patients had findings in favor of autoimmune hepatitis. Conclusion: Auto antibodies were positive in 10 cases. ANA was positive in 6 (2.97%) of all cases. ASMA was positive in 4 (1.98%) cases. Increased echogenicity was found in 3 cases. Histological evaluation showed 2 patients had biopsy confirmed autoimmune hepatitis. AIH-2 was not seen among our cases.

Antecedentes: La diabetes mellitus tipo 1 (DM1) es una de las enfermedades infantiles con mayor prevalencia. Se observaron varias enfermedades autoinmunes acompañantes con diabetes tipo 1. Las enfermedades autoinmunes más comunes con DM1 son la tiroiditis autoinmune y la enfermedad celíaca. En algunos reportes, se ha encontrado hepatitis autoinmune en asociación con DM-1. Objetivos: El objetivo de este estudio fue evaluar los autoanticuerpos de hepatitis autoinmunes en niños con DM1. Materiales y métodos: En este estudio transversal, se evaluaron 202 niños con DM1 (47,5% eran hombres y 52,5% eran niñas). Se midieron las enzimas hepáticas, los autoanticuerpos autoinmunes relacionados con la hepatitis, como los anticuerpos antinucleares (ANA), el músculo liso (ASMA) y los anticuerpos microsomales hepáticos y renales (LKM-1). Se realizó una ecografía hepática para los participantes y se tomó una biopsia del hígado para niños con mayor ecogenicidad del hígado, hepatomegalia o enzimas hepáticas elevadas. Los resultados fueron analizados por el software estadístico spss-16 usando estadística descriptiva y prueba de chi-cuadrado, T-TEST pareado. Se consideró estadísticamente significativo un nivel menor del 5%. Resultados: En 6 pacientes con ANA y en 4 pacientes (2%) ASMA fue positiva, 1 paciente fue ASMA positiva pero ANA negativa. Ninguno de los pacientes fue anti LKM-1 positivo. 3 pacientes tuvieron ANA y ASMA positivas, y aumentaron la ecogenicidad hepática en la ecografía simultáneamente. La evaluación histológica mostró que 2 pacientes tenían hallazgos a favor de la hepatitis autoinmune. Conclusión: Los autoanticuerpos fueron positivos en 10 casos. ANA fue positivo en 6 (2,97%) de todos los casos. La ASMA fue positiva en 4 (1,98%) casos. Se encontró mayor ecogenicidad en 3 casos. La evaluación histológica mostró que 2 pacientes tenían biopsia confirmada de hepatitis autoinmune. AIH-2 no fue visto entre nuestros casos.

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) / Complicaciones posteriores a dilatación endoscópica con balón en niños con estenosis esofágica. Experiencia de un centro de referencia en 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.

Antecedentes: La estenosis esofágica es una de las más importantes complicaciones de la ingesta de caústicos. Objetivo: El objetivo del estudio fue evaluar las complicaciones de la dilatación endoscópica con balón en niños con estenosis esofágica. Material y métodos: En este estudio retrospectivo se incluyeron 82 niños. Se incluyeron todos los niños que se sometieron a una dilatación endoscópica con balón por estenosis esofágica. La duración del estudio fue 14 años iniciando en el año 2001. La edad media de los pacientes fue 3,95 +/- 0,4 años (Min. 15 días, Máx. 14 años). La recolección de datos se realizó revisando las historias clínicas y con llamadas telefónicas. Los datos se analizaron con el Sistema SPSS. Resultados: En este estudio, 47% de los casos fueron varones y 53% fueron mujeres. La ingesta de caústicos fue la causa más frecuente de estenosis esofágica (33,7%). El síntoma más común fueron los vómitos (87,8%). Entre nuestros casos, 76,8% no presentaron molestias luego de la dilatación esofágica. El dolor torácico fue la molestia más común luego de la dilatación esofágica. La tasa de respuesta al tratamiento fue similar entre hombres y mujeres. Los niños pequeños tuvieron la mejor respuesta al tratamiento luego de la dilatación esofágica. Conclusiones: Entre nuestros casos, 76,8% no presentaron molestias luego de la dilatación esofágica. La perforación esofágica se presentó en 4,9% de los casos. El dolor torácico fue la complicación más común posterior a la dilatación.

Evaluation of frequency of autoimmune hepatitis autoantibodies in children with type 1 diabetes.

BACKGROUND: Diabetes mellitus type 1 (T1DM) is one of the childhood diseases with growing prevalence. Various accompanying autoimmune diseases were seen with type 1 diabetes. The most common autoimmune diseases with T1DM are autoimmune thyroiditis and celiac disease. In some reports, autoimmune hepatitis has been reported in association with DM-1. OBJECTIVES: The aim of this study was to evaluate autoimmune hepatitis autoantibodies in children with T1DM. MATERIALS AND METHODS: In this crosssectional study, 202 children with T1DM were evaluated (47.5% were males and 52.5% were girls). Liver enzymes, autoimmune hepatitis related autoantibodies such as anti-nuclear antibodies (ANA), anti-smooth muscle (ASMA) and anti liver and kidney microsomal antibodies (LKM-1) were measured. Liver ultrasound was done for participants and biopsy of liver was taken for children with increased echogenicity of the liver, hepatomegaly or elevated liver enzymes. Results analyzed by statistical software spss-16, Descriptive statistics and chi-square test, paired T-TEST. Level of less than 5% was considered statistically significant. RESULTS: In 6 patients ANA and in 4 patients (2%) ASMA was positive,1 patient was ASMA positive but ANA negative. None of the patients were Anti LKM-1 positive. 3 patients had positive ANA and ASMA, and increased liver echogenicity on ultrasound simultaneously. Histological evaluation was showed that 2 patients had findings in favor of autoimmune hepatitis. CONCLUSION: Auto antibodies were positive in 10 cases. ANA was positive in 6 (2.97%) of all cases. ASMA was positive in 4 (1.98%) cases. Increased echogenicity was found in 3 cases. Histological evaluation showed 2 patients had biopsy confirmed autoimmune hepatitis. AIH-2 was not seen among our cases.

Percutaneous endoscopic gastrostomy in children: a single center study at Tertiary hospital Iran.

INTRODUCTION: The aim of this study was to evaluate complications after percutaneous endoscopic gastrostomy among children who underwent percutaneous endoscopic gastrostomy in Nemazee hospital. MATERIALS AND METHODS: All children who underwent percutaneous endoscopic gastrostomy were included in the current study. Place of the study was department of pediatric gastroenterology of Nemazee children hospital of Shiraz university of medical sciences. Duration of the study was 5 year starting from 2008. All drugs such as aspirin, NSAIDS, and heparin were discontinued 1-7 days before procedures. All patients were kept NPO 6-8 hours before procedure according to the age. Single dose antibiotic was prescribed for all cases before procedure. During procedure, all patients were sedated using propofol and or midazolam. Some patients required intubation. RESULTS: Of 39 cases who underwent PEG, 4 (10.2%) patients showed complication. The most common indication for PEG insertion were neurologic problem (84.6%) and metabolic disease (10.2%). Of our patients, 84.6% of the cases had the weight below third percentile. CONCLUSION: The most common indication for percutaneous endoscopic gastrostomy was cerebral palsy. The complication rate in our study was 10.2%. Celulitis was the most common complication.

Effect of ursodeoxycholic acid and vitamin E in the prevention of liver injury from methotrexate in pediatric leukemia.

BACKGROUND/AIMS: Ursodeoxycholic acid (UDCA) and antioxidants such as vitamin E are considered to have a protective role in preventing chemotherapy-induced liver damage. The aim of this study was to assess the efficacy of these agents for hepatoprotection in pediatric patients with B-cell acute lymphoblastic leukemia (ALL), who were treated with methotrexate in their maintenance phase of treatment. MATERIALS AND METHODS: Eighty children with B-cell ALL were randomly divided into four groups. Group 1 was administered oral vitamin E (400 mg/day); group 2 was administered oral UDCA (15 mg/kg/day); group 3 was administered a combination of the two drugs; and group 4 served as a control group and was administered no drug except their chemotherapy drugs. Complete blood count, liver function test, liver ultrasonography, and liver fibroscan were requested, and the results were compared. RESULTS: Group 1 showed a slight increase in total bilirubin levels compared to baseline levels during the study (P=0.036). Group 2 showed a decline in aspartate aminotransferase and alanine aminotransferase levels during the study and at 6 months after discontinuing the drug; however, these differences were not statistically significant (P=0.051 and 0.083, respectively). None of the patients showed the evidence of significant fibrosis on liver fibroscan. Eight patients showed some evidence of mild-to-moderate fibrosis (F1, F2), but the results were not different between the groups as well as between pre- and post-study periods in each group. CONCLUSION: Low-dose methotrexate does not cause significant liver fibrosis in pediatric leukemia. UDCA and vitamin E have minimal roles in hepatoprotection among pediatric patients with ALL.

Percutaneous endoscopic gastrostomy in children: a single center study at Tertiary hospital Iran / Gastrostomía percutánea en niños: reporte de un hospital terciario de Iran

Introduction: The aim of this study was to evaluate complications after percutaneous endoscopic gastrostomy among children who underwent percutaneous endoscopic gastrostomy in Nemazee hospital. Materials and methods: All children who underwent percutaneous endoscopic gastrostomy were included in the current study. Place of the study was department of pediatric gastroenterology of Nemazee children hospital of Shiraz university of medical sciences. Duration of the study was 5 year starting from 2008. All drugs such as aspirin, NSAIDS, and heparin were discontinued 1-7 days before procedures. All patients were kept NPO 6-8 hours before procedure according to the age. Single dose antibiotic was prescribed for all cases before procedure. During procedure, all patients were sedated using propofol and or midazolam. Some patients required intubation. Results: Of 39 cases who underwent PEG, 4 (10.2%) patients showed complication. The most common indication for PEG insertion were neurologic problem (84.6%) and metabolic disease (10.2%). Of our patients, 84.6% of the cases had the weight below third percentile. Conclusion: The most common indication for percutaneous endoscopic gastrostomy was cerebral palsy. The complication rate in our study was 10.2%. Celulitis was the most common complication

Introducción: El objetivo de este estudio fue evaluar las complicaciones luego de una gastrostomía endoscópica percutánea (PEG) en niños realizada en el hospital Nemazee. Material y métodos: Se incluyeron al estudio todos los niños que se realizaron PEG en el hospital. El lugar del estudio fue el departamento de gastroenterología pediátrica del Hospital para niños Nemazee de la Universidad Shiraz de ciencias médicas. La duración del estudio fue cinco años, iniciando en el año 2008. Todas las drogas como aspirina, AINES y heparina fueron suspendidas entre 1 a 7 días previos al procedimiento. Todos los pacientes estuvieron entre 6 a 8 horas del examen en ayunas dependiendo de la edad. Se prescribió una dosis de antibioterapia profiláctica en todos los casos previo al procedimiento. Durante el procedimiento, todos los pacientes fueron sedados usando propofol y/o midazolam. Algunos pacientes necesitaron intubación. Resultados: De 39 casos que se sometieron a PEG, 4 (10,2%) tuvieron alguna complicación. La indicación más frecuente de PEG fueron los problemas neurológicos (84,6%) y luego las enfermedades metabólicas (10,2%). De nuestros pacientes, 84,6% de los casos estuvieron por debajo del tercer percentile. Conclusión: La indicación más común de gastrostomía endoscópica percutánea fue la parálisis cerebral. La tasa de complicación en nuestro estudio fue 10,2%. La celulitis fue la complicación más común

ASSOCIATION OF POTENTIAL CELIAC DISEASE AND REFRACTORY IRON DEFICIENCY ANEMIA IN CHILDREN AND ADOLESCENTS.

BACKGROUND: Celiac disease is an enteropathy caused by dietary gluten. The combination of serologic, genetic and histologic data has led to description of other categories of this disease. OBJECTIVE: There are a number of patients with iron deficiency anemia (IDA) that do not respond to iron treatment and may be repeated for many times, Therefore, we aimed to investigate celiac disease in this group. METHODS: In this cross sectional transverse prospective study from August 2011 to February 2013, in a Pediatric care clinic affiliated to Shiraz University of Medical Sciences, 184 children including 92 IDA patients who responded to treatment using iron supplement, 45 non-responding iron deficient patients, and 47 healthy individuals, with the maximum age of 18 years, with written consent from their parents, participated in serologic screening (with Anti-TTG antibody and anti-Endomysial antibody) for celiac disease. Patients with at least one positive serology test underwent multiple mucosal biopsy from bulb and duodenum. RESULTS: Among 184 participants, 19 (10.3%) subjects had positive serologic test for celiac disease, including 13 (28.9%) patients in the group with refractory IDA, 5 (5.4%) patients in the group with treated IDA, and 1 patient in the healthy group. The frequency of positive serologic test in the group with IDA resistant to treatment was prominently higher than the other two groups (P<0.001). Among the patients with positive serologic celiac test who underwent endoscopy and biopsy, no histologic evidence of celiac disease was seen. They were diagnosed as potential celiac disease. CONCLUSION: Frequency of potential celiac disease in patients with refractory IDA was higher than control the subjects. Therefore, we recommend serologic screening for early detection and minimizing the complications of celiac disease and repeated iron therapy for this group.

Potencial associação da doença celíaca com anemia ferropriva refratária em crianças e adolescentes / Association of potential celiac disease and refractory iron deficiency anemia in children and adolescents

ABSTRACT BACKGROUND: Celiac disease is an enteropathy caused by dietary gluten. The combination of serologic, genetic and histologic data has led to description of other categories of this disease. OBJECTIVE: There are a number of patients with iron deficiency anemia (IDA) that do not respond to iron treatment and may be repeated for many times, Therefore, we aimed to investigate celiac disease in this group. METHODS: In this cross sectional transverse prospective study from August 2011 to February 2013, in a Pediatric care clinic affiliated to Shiraz University of Medical Sciences, 184 children including 92 IDA patients who responded to treatment using iron supplement, 45 non-responding iron deficient patients, and 47 healthy individuals, with the maximum age of 18 years, with written consent from their parents, participated in serologic screening (with Anti-TTG antibody and anti-Endomysial antibody) for celiac disease. Patients with at least one positive serology test underwent multiple mucosal biopsy from bulb and duodenum. RESULTS: Among 184 participants, 19 (10.3%) subjects had positive serologic test for celiac disease, including 13 (28.9%) patients in the group with refractory IDA, 5 (5.4%) patients in the group with treated IDA, and 1 patient in the healthy group. The frequency of positive serologic test in the group with IDA resistant to treatment was prominently higher than the other two groups (P<0.001). Among the patients with positive serologic celiac test who underwent endoscopy and biopsy, no histologic evidence of celiac disease was seen. They were diagnosed as potential celiac disease. CONCLUSION: Frequency of potential celiac disease in patients with refractory IDA was higher than control the subjects. Therefore, we recommend serologic screening for early detection and minimizing the complications of celiac disease and repeated iron therapy for this group.

RESUMO CONTEXTO: A doença celíaca é uma enteropatia causada pelo glúten na dieta. A combinação de dados sorológicos, genéticos e histológicos proporcionou a descrição de outras categorias desta doença. OBJETIVO: Há pacientes com anemia por deficiência de ferro que não respondem ao tratamento com ferro mesmo que repetido por muitas vezes. O objetivo deste trabalho foi investigar a presença de doença celíaca nestes indivíduos. MÉTODOS: Realizado estudo prospectivo com cruzamento secional transversal, de agosto de 2011 a fevereiro de 2013, em uma clínica de cuidados pediátricos afiliados a Shiraz University Medical Sciences, com 184 crianças incluindo 92 pacientes com anemia por deficiência de ferro que responderam ao tratamento com ferro suplementar, 45 não respondedores e 47 indivíduos sadios, com idade máxima de 18 anos, todos com consentimento informado dos pais. Todos participaram da triagem sorológica (com anticorpos anti-TTG e anticorpo antiendomísio) para doença celíaca. Pacientes com pelo menos um teste de sorologia positiva foram submetidos a biópsia da mucosa múltipla do bulbo e duodeno. RESULTADOS: Entre os 184 participantes, 19 (10,3%) tinham teste sorológico positivo para doença celíaca, incluindo 13 (28,9%) pacientes no grupo com a anemia por deficiência de ferro refratária, 5 (5,4%) pacientes no grupo com anemia por deficiência de ferro tratados e respondedores e 1 paciente do grupo saudável. A frequência de teste sorológico positivo no grupo com anemia por deficiência de ferro resistente ao tratamento foi destacadamente maior do que os outros dois grupos (P<0,001). Entre os pacientes com teste sorológico positivo para doença celíaca submetidos a endoscopia e biópsia, não foi vista nenhuma evidência histológica de doença celíaca. Foram diagnosticados como potencial doença celíaca. CONCLUSÃO: Potencial frequência de doença celíaca em pacientes com anemia por deficiência de ferro refratária foi maior do que nos controles. Portanto, recomendamos testes sorológicos de triagem para a detecção precoce, minimizando as complicações da terapia de ferro repetidas para este grupo.

Rigler Sign in a Child With Posttransplant Lymphoproliferative Disease: A Sign That Should Not Be Missed.

Rigler sign is a double wall sign suggesting pneumoperitoneum and intestinal perforation, and it needs emergency surgical treatment. Early diagnosis of intestinal perforation by clinical symptoms, presence of Rigler sign in abdominal radiography, and then early surgical treatment can reduce mortality. Here, we report a patient with Crigler-Najjar syndrome who underwent liver transplant and then developed posttransplant lymphoproliferative disease and received chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone plus rituximab. She was referred to the emergency department due to abdominal distension with positive Rigler sign in abdominal radiography; intraoperative findings revealed intestinal perforation. Pediatricians and surgeons should be aware of Rigler sign so that it is diagnosed early and emergency surgical treatment can be performed.

Analysis of hospital records of children with hydatid cyst in south of Iran.

The clinical manifestations of hydatidosis are various and related to anatomic location. Defining frequent symptoms and signs of the disease is imperative for early management of it. The aim of this report was to analyse the clinical features of infected children with hydatid cysts located in different organs. In this study, medical charts of 57 children between 3 and 16 years of age with hydatid cyst admitted to Pediatric Wards of Nemazee Hospital were evaluated over a 12 year period (from 2003 to 2014, prospectively). All the epidemiologic, clinical, paraclinical and therapeutic data were collected. The frequencies of hydatidosis in males and females were 42.1 and 56.1%, respectively. Hydatid cysts were found in the liver and lungs in 59.6 and 33.3% patients respectively and 2 patients had an asymptomatic cyst in the heart with concomitant liver and lung cysts. The right upper quadrant pain (100%) was the most common symptom in the liver cysts. Phlegm (78.9%), Dyspnea (57.9%), acute (47.4%) and chronic cough (47.4%) were mostly seen in lung hydatid cysts. Some symptoms such as fever (68.4%) and weakness (59.6%) were the most common presenting symptoms in both groups. All children were treated through surgical approaches plus medical treatment. In the present report, liver was the most common site of involvement in children. Liver hydatidosis should be considered in children with upper quadrant pain and pulmonary hydatidosis in children complaining of phlegm and dyspnoea.

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.

Caustic ingestion in children in south of Iran: Retrospective study from Shiraz - Iran / Ingestión de cáusticos en niños del sur de Irán: Estudio retrospectivo de Shiraz - Irán

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.

La ingestión de cáusticos es una gran preocupación de salud tanto en países desarrollados como en vías de desarrollo, que puede llevar a lesiones esofágicas graves. La presentación clínica de la ingestión de cáusticos en niños varía desde asintomática hasta tener secuelas fatales, como perforación y/o estenosis. Objetivo: Debido a la ausencia de estudios en nuestra área, este estudio ha evaluado las manifestaciones clínicas, endoscópicas y las complicaciones de la ingesta de cáusticos en niños en el sur de Irán. Materiales y métodos: En estudio retrospectivo, revisamos 75 niños con ingesta de cáusticos que ingresaron al Nemazee Hospital of Shiraz University of Medical Science durante 6 años (2006-2011). Los signos y síntomas fueron recolectados para cada caso. Resultados: Los síntomas más frecuentes fueron disfagia, lesiones orales, vómitos y salivación. Las lesiones esofágicas se detectaron tanto en ingestión de ácido como de álcali, pero las lesiones gástricas fueron definitivamente más frecuentes con la ingestión de ácidos. Durante el periodo de seguimiento el 20% de los casos desarrolló estrechez esofágica. Conclusión: La disfagia, lesiones orales, vómitos y salivación fueron los hallazgos más comunes. La estrechez esofágica se encontró en el 20% de los casos durante los tres meses de seguimiento de los pacientes.