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Background: Benign esophageal strictures are not rare. Over the past two decades, endoscopic balloon dilatation (EBD) has been used to treat them. Objectives: The purpose of this study was to identify the most common causes of benign esophageal stricture in children determine the success rate of endoscopic balloon dilatation. Methods: Children younger than 16 years with benign esophageal strictures referred to the endoscopy department during one year (2016 - 2017) were enrolled. After obtaining written consent from parents, endoscopy balloon dilatation was performed with two types of balloon catheters. Response to treatment was evaluated based on clinical symptoms and was classified according to the Vantrappen table score. Results: In this study, thirty-one (31) children participated including 19 (61%) boys and 12 (39%) girls. The mean age was 5.1 ± 3.9 years. The most common causes of esophageal stricture were: achalasia (45%), esophageal atresia (19%), stenosis due to the caustic ingestion (19%), another congenital stenosis (16%). Overall, 27 children (87.1%) had a good response to treatment. In children with stenosis due to caustic ingestion, the inappropriate response was higher than the rest (33%). However, only in 4 (12.9%) patients, balloon dilatation failed. No complications were observed. Conclusions: Achalasia, esophageal atresia, and caustic ingestion are the most common cause of benign esophageal stricture in the children. EBD is an effective and safe treatment in these children, even in cases of previous surgery and recurrence. If this procedure is performed by an expert using appropriate balloon catheters, no complications will be created.

Background: Effective and safe procedural sedation is necessary for percutaneous liver biopsy in children. There are a number of different protocols for this purpose. The current study investigated ketamine and DPT cocktail (meperidine (Demerol®) + promethazine (Phenergan®) + chlorpromazine (Thorazine®)). Methods: The current cohort of 80 Iranian children aimed at investigating percutaneous liver biopsy. Each of the 2 study groups (ketamine and DPT) included 40 patients. Both groups were matched by age (number of participants under and above 7 years old). The current study evaluated the efficacy of 2 protocols by CHEOPS (children’s hospital of Eastern Ontario pain scale) and visual analogue scale (VAS) pain scoring system and sedation scoring A,B,C, and D. Results: Ketamine group was sedated and recovered much more rapidly than the DPT receiving patients. Also, they had significantly less pain during the biopsy. The most common side effect of ketamine was vomiting (27%); in the other group, transient hypotension and tachycardia were more common. Conclusions: Ketamine is a safe and effective choice for procedural sedation in percutaneous liver biopsy in children.

Background: Chronic abdominal pain in children is a common disorder. For an accurate diagnosis of its cause, sometimes invasive diagnostic procedures such as endoscopy should be performed. Objectives: The purpose of the study was to evaluate the fecal calprotectin in children with upper gastrointestinal signs and symptoms and to compare it with endoscopic findings. Methods: A total of 131 children aged 1-14 years with upper gastrointestinal symptoms were enrolled during 2012 - 2013 at two centers. One hundred and twenty patients underwent endoscopy and biopsy. Before endoscopy, the level of calprotectin was measured by the enzyme-linked immunosorbent assay test in stool samples and the results were compared with the endoscopic and pathology findings. Results: Of the 120 children included in this study, 71 (59.2%) were males and 49 (40.8%) females with a mean age of 93.6 months. Of the 112 patients in whom biopsies were taken, 16 had esophagitis, 89 chronic gastritis (79.5%) and 57 colonization with Helicobacter pylori. Conclusions: There was a statistically significant correlation between fecal calprotectin and gastritis and severity of H. pylori infection. Fecal calprotectin level measurement can avoid unnecessary endoscopies and is also useful for evaluation of therapy response.

Introduction To define the clinical features, biochemical and histological findings and outcome of three forms of autoimmune hepatitis. Materials and Methods In a cross sectional study between November 2001 to January 2008 in Tehran and Mashhad university of medical sciences, 61 children who diagnosed as AIH (40 girls and 21 boys) have been analyzed for their clinical, serological, and histological data. Variables analyzed included age, sex, clinical presentation, hepatic function, immunoglobulins, autoimmunity markers, histology and clinical evolution. According to the auto-antibodies profile, AIH patients were classified as type I AIH (ANA or smooth-muscle antibodies or both positive), type II (anti-LKM-1 positive) and type III (antibody negative). Results We analyzed data of 61 children with AIH. 51 patients (median age: 10 (0.7-14) years had anti-nuclear and/or smooth muscle antibody (ANA/ASMA or both), 5 (median age: 7 (2-8) years) had liver/kidney microsomal antibody (LKM-1). 5 patients had no detectable autoantibody. At presentation: 60% had jaundice or symptoms of acute hepatitis; LKM-1 positive were younger. Interface hepatitis was seen in 100% and fibrosis+/- cirrhosis were found in 70%. Frequency of AIH was 2:1 in girls. Type 1 was the most frequent diagnosis (83%) and was more frequent in older children. Relapse and treatment failure were common in type II. Conclusion AIH may have different clinical presentation in children. Althout most of cases may have high inflammatory activity or cirrhosis, good response to treatment and remission may accur.

Girifl ve Amac: Kistik fibrozis en s›k karfl›lafl›lan kal›t›msal olumcul hastal›kt›r ve yenidoan taramas›n›n yap›lmad›¤› bolgeler- de hastalar gec tespit edilebilir. Erken tan› konulan kistik fibrozis hastalar›n›n sonuclar› dramatik olarak daha iyidir. Bu cal›fl- mada kistik fibrozis hastalar›nda ilk baflvuruda tespit edilen tan›ya goturen bulgular›n›n incelenmesi amaclanm›flt›r. Gerec ve Yontem: Bu cal›flma ‹ran'da birden cok pediatrik referans merkezinde gerceklefltirildi, hastalar›n tan› an›ndaki demografik, kli- nik ve labaratuvar verileri kontrol listesi ile kay›t alt›na al›nd›. Tum hastalar›n 2 adet ter klor testi mevcuttu. Bulgular: Kistik fibrozis tan›s› olan 197 hasta cal›flmaya dahil edildi. Bu hastalardan, 119'unun (%74) yafl› 6 aydan kucuktu ve 34 hastan›n (%21) yafl› 6-12 ay aras›ndayd›. En s›k karfl›lafl›lan klinik bulgular buyume gerilii (n=178; %90) , tekrarlayan akcier enfeksiyonlar› (n=139; %71) ve steatore (n=135; %69) olarak tespit edildi. En s›k karfl›lafl›lan radyolojik anormallik akcierlerde afl›r› havalan- mayd›. Terde tuz tad›, steatore, metabolik alkaloz, radyolojik anormallikler ve karacier testlerinde anormallik olan hastalar›n or- talama tan› yafl› dierlerine gore dufluk bulundu. Sonuc: Bu cal›flma sonuclar›na gore buyume gerilii, tekrarlayan solunum sis- temi enfeksiyonu, steatore, metabolik alkaloz ve tuzlu cilt tad› kistik fibrozis ile s›k karfl›lafl›lan bolgelerde klinikte uyar›c› olmal›- d›r. Bu bolgelerde, dikkat ve klinik fluphe ile hastalar›n erken tespit edilmesi mumkun olabilir. Anahtar kelimeler: Kistik fibrozis, tan›, klinik bulgular, tarama Background/aims: Cystic fibrosis is the most common inherited lethal disease, which could be frequently identified late in regi- ons 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 failu- re to thrive, recurrent respiratory infections, steatorrhea, metabolic alkalosis, and salty tasting skin should be considered as clini- cal 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.

Introduction Poor weight gain is one of the most important mortality hazards in cystic fibrosis (CF) patients. The mechanisms that may hinder body weight regulation are not completely understood. Leptin and its role in fat mass could be related to control of weight gain in CF patients. As the previous data are conflicting, we aimed to investigate serum leptin level in Iranian CF children compared to a control group. Material and methods Forty-three CF patients aged from 3 to 120 months and 43 age-matched controls were enrolled. Patients were recruited from the outpatient clinic of the Children's Medical Center Hospital. Controls were visited in the general outpatient clinic for an annual check-up. Both groups were divided into three subgroups based on age: 3 to 12 months, 13 to 48 months, and 49 to 120 months. Body mass index (BMI) was calculated for all the participants. Serum leptin levels were measured applying a solid phase enzyme-linked immunosorbent assay (ELISA). Results Leptin levels and BMI values were significantly different between patients and controls (p = 0.02, p < 0.001, respectively) but only patients aged 13–48 months had significantly higher levels of leptin than age-matched controls (p = 0.016). Overall male patients’ mean leptin level was significantly higher than in female patients (p = 0.032) and male controls (p < 0.001). Conclusions Leptin level in our patients was significantly higher than controls. It seems that leptin levels during infancy are higher than in adult patients. Further studies are required on specific genotypes, gender and age to reveal the probable correlation with BMI and leptin levels in CF patients from different ethnic groups.

Background. Eosinophilic esophagitis is an inflammatory condition where there is a dense infiltration of eosinophils typically exceeding fifteen cells per high power field. Familial Mediterranean fever is an autosomal recessive disorder characterized by brief, acute, and self-limited episodes of fever and polyserositis that recur at irregular intervals. Case Presentation. A three-year-and-nine-month-old Iranian girl was admitted to our center. The patient’s parents complained of a history of abdominal pain, poor appetite, and poor weight gain from 1.5 years ago and episodes of food impaction after starting solid foods. Eosinophilic esophagitis was diagnosed based on histology. Because of continuing abdominal pain after treatment of eosinophilic esophagitis, the episodic nature of disease, and the presence of fever with pain, screening for familial Mediterranean fever mutation was performed and the patient was found to be heterozygote for Mediterranean fever. Conclusion. We have reported a case of eosinophilic esophagitis coexisting with familial Mediterranean fever which has not been described previously.

INTRODUCTION: Cystic fibrosis (CF) is a common genetic disorder as a result of mutation in the CF transmembrane conductance regulator gene. Weight loss in CF patients seems to be multifactorial and metabolic factors, inflammation, recurrent infections and poor appetite are possible candidates. Ghrelin affects energy hemostasis by stimulating growth hormone secretion, glucose and lipid metabolism alteration and inhibition of the inflammatory system. Data on the role of ghrelin in energy deficiency in CF patients are sparse and controversial. The study was done to compare the plasma levels of the acylated form of ghrelin in CF patients with healthy participants. MATERIAL AND METHODS: Thirty cystic fibrosis patients (aged 1-168 months) and thirty healthy matched participants were enrolled in the study. Plasma ghrelin and albumin levels were measured and body mass index (BMI) was calculated as well. RESULTS: Plasma levels of acylated ghrelin in CF patients were significantly higher than the control group (mean 25-75%: 14.5 vs. 2.7, p = 0.032). Body mass index measurements in CF patients were significantly lower than the control group (p < 0.001). Using regression analysis there was no statistically significant correlation between plasma ghrelin levels and serum albumin, weight, height or BMI values in CF patients and controls. CONCLUSIONS: The acylated ghrelin levels are increased in CF. So plasma levels of acylated ghrelin could be used as an indicator of food uptake and energy balance in them. Further studies should be established to find out the exact role of factors affecting energy metabolism.

Introduction Infection is a major cause of morbidity and mortality in patients with cystic fibrosis (CF). Antimicrobial resistance of the bacterial spp. particularly methicillin resistance in Staphylococcus aureus has caused a lot of attention. The aim of this study was to describe the prevalence of S. aureus, Pseudomonas aeruginosa and Burkholderia cepacia-complex as well as their antimicrobial susceptibility patterns in CF patients in an Iranian referral pediatrics Hospital. Material and methods From March 2011 until February 2012, 172 samples were collected at the Children Medical Center (CMC), an Iranian referral hospital in Tehran, Iran. Sputum specimens were cultured for the following bacterial pathogens: P. aeruginosa, S. aureus, B. cepacia complex. Antimicrobial susceptibility was performed according to the Clinical Laboratory Standards Institute recommendations. Results In our study, 54% of the patients (n = 93) harbored at least once S. aureus, 30% (n = 52) P. aeruginosa, and 2% (n = 3) Burkholderia cepacia. In 40 patients (23%), none of these organisms was grown. An increasing colonization rate of P. aeruginosa in the second decade of life was found. In contrast, the colonization rate of S. aureus was constant in both decades of life. Methicillin resistant S. aureus (MRSA) was detected in 40 isolates (43%). Among MRSA, no resistance against vancomycin, linezolid and quinupristin/dalfopristin occurred. The susceptibility of P. aeruginosa isolates to meropenem, imipenem, doripenem, levofloxacin and polymixin B were more than 90%. Conclusion The prevalence of MRSA has been rising. Since its impact on clinical outcomes, optimal prevention and treatment strategies are unclear, further studies to expand the knowledge about the infection control strategies and MRSA treatment are highly recommended.

Objective: To study the clinical presentation, histology and colonoscopic features of lower gastrointestinal polyps in Iranian children. Material and Methods: Medical reports of children with colorectal polyps were retrospectively reviewed from 1996 to 2005 at the Children’s Medical Center Hospital, Iran. A total of 563 cases were studied. Data related to age, sex, family history, signs and symptoms, the size, location, polyp types and associated lesions were collected and analyzed. Results: The mean age of children was 5.66 ± 2.88 years (range 2 months to 17 years), with a male-to-female ratio of 1.61:1.0. The highest incidence was between ages 2 and 10 years (85.1%). Rectal bleeding was the presenting symptom in 78.5% cases. The polyps were solitary in 94% of cases. A majority of polyps (86.3%) were juvenile and 86.7% located in the rectosigmoid area. Three percent of cases had a positive family history. One case of Turcot syndrome was also identified. Conclusion: Juvenile polyps remain the most common polyps in Iranian children. Although the presence of a solitary polyp in the rectosigmoid colon is more prevalent, in a significant number of cases they are multiple and located in proximal parts. Polyps must be removed even when asymptomatic because of their probable neoplastic potential.

Dear Editor, Cystic Fibrosis (CF), characterized by abnormal transport of chloride and sodium ion across the epithelium, leads to thick, viscous secretions (1). The hallmark signs and symptoms of cystic fibrosis are salty tasting skin, poor growth and poor weight gain despite a normal food intake, accumulation of thick, sticky mucus, frequent chest infections, and coughing or shortness of breath. Males can be infertile due to congenital obstruction of vas deferens. Symptoms often appear in infancy and childhood, such as bowel obstruction due to meconium ileus in newborn babies (2, 3). Cystic fibrosis may be diagnosed by many different methods including newborn screening, sweat testing, and genetic testing (4). The prognosis for cystic fibrosis has improved due to earlier diagnosis through screening, better treatment and access to health care (5). This disease is specified by very wide spectrum of clinical manifestations for which many differential diagnoses have to be considered. We studied 300 patients of CF in Children’s Medical Center in Tehran, during 2006 to 2012. Necessary information including age, sex, and symptoms of patients such as cough, hemoptysis, recurrent pulmonary infection, nasal polyposis, recurrent sinusitis, intestinal obstruction, intussusception, reflux, rectal prolapse, pancreatitis, fatty diarrhea, edema and hypo-albuminemia, anemia, vomiting and dehydration attack, cirrhosis, biliary atresia, portal hypertension, salty skin, failure to thrive (FTT), clubbing, coagulation disorder, bone fracture and nyctalopia was entered in the checklists. First manifestation of each patient was also noted. The main complaint of patients was isolated FTT and this complication had occurred in 74 (24.7%) patients. In addition, FTT with another complication was observed in 28 (9.3%) patients at the beginning of the disease; in other words, isolated failure to thrive or FTT associated with other symptoms of the disease was seen as first clinical presentation in about one-third of the patients (that is, 102 patients). The most common complication next to FTT was pulmonary infection which was seen in 47 (15.7%) patients. Other common first clinical presentations of cystic fibrosis include: attacks of vomiting and dehydration in 45 (15%), cough in 25 (8.3%), intestinal obstruction (meconium ileus) in 23 (7.7%), fatty diarrhea in 18 (6%), FTT with fatty diarrhea in 11 (3.7%), edema and Hypo-albombinemia in 10 (3.3%), and biliary atresia in 6 (2%) patients. Other symptoms including anemia, salty skin, coagulation disorders, gastro esophageal reflux, and sinusitis were seen in less than five patients. In 30 (10%) patients, two of the mentioned complications were observed simultaneously. At onset of disease, among a total of 300 patients, 182 (60.7%) had gastrointestinal symptoms, and 72 (24%) patients had respiratory tract symptoms. CF may be diagnosed after a long period of illness, by different symptoms and laboratory tests (6). In a similar study by Fallahi et al. in Iran, prevalence of first clinical presentations of CF patients before the diagnosis was as follow: gastro intestinal symptoms in 152 (62.6%), respiratory symptoms in 93 (38.3%), isolated FTT or in combination with other symptoms in 34.9%, diarrhea in 28.8%, vomiting in 15.6%, cough in 30% and respiratory distress in 23.9% patients (6). As mentioned before, the first complication in 7.7% of our patients was ileus meconium which is similar to results of other studies (4). According to this study, gastrointestinal symptoms were the most common symptoms associated with CF disease. In a study by McCloskey more than two thirds of patients presented with respiratory symptoms including chronic sino-pulmonary disease, nasal polyps or recurrent respiratory tract morbidity. This study tried to bring up the most common presenting symptoms of CF. With more attention to the symptoms of this disease, the diagnosis could be established earlier and upcoming complications prevented.

Iron deposition in liver is a major finding in thalassemic patients and because of direct iron toxicity to liver it is associated with several consequences for example liver fibrosis. Liver biopsies from 63 patients were evaluated, 40 (63.5%) were male and 20 (36.5%) were female. The mean age of the patients was 8.01 ± 3.7 and the age range was from 1.8 to 15 years. Histologic grading and staging was performed for each case according to modified HAI (Hepatitis Activity Index) system. Iron scoring was performed according to Sindram & Marx and MTK1-3 scoring systems. The mean (SD) dry weight (dw) of liver specimens was 1.34 (0.11) mg (range 0.20 to 3.80 mg). The mean (SD) of hepatic iron concentration was 230.9 (121.2)μmol/g dry weight. The relationship between the variables HIC, HII (hepatic iron index) and all histological gradings of iron (S&M and MTK1-3) was very strong. The relationship between the HIC and staging by HAI method was good. Significant differences were identified between the mean HIC in scores 1&2 of all histological iron scorings (S&M and MTK1-3), but no significant differences identified between mean HIC in other adjacent scores in all histological iron scorings (S&M and MTK1, 2 and 3). New scoring system introduced by us in this study which considered size and density of iron granules as well as zone of iron deposition was very much the same as simple Sindram and Marx classification.

Dear Editor, Neonatal cholestasis (NC) is defined as prolonged elevation of serum levels of conjugated bilirubin beyond two weeks of life. The two most common causes of NC are biliary atresia (BA) and idiopathic neonatal hepatitis (INH). Early and accurate differentiation of these two entities is very important and determines the prognosis and curability of cholestasis. BA requires surgical intervention as soon as possible; whereas, INH needs medical management (1-3). There is no single non-invasive test that can definitely differentiate these two entities. The gold standard for the diagnosis of BA is intraoperative cholangiography. Liver biopsy is the investigation with high diagnostic usefulness in NC. Its diagnostic accuracy for NC has been more than 90% in several studies (1, 4-6). Alagille identified four clinical features that, although nonspecific, supported the correct diagnosis of intrahepatic or extrahepatic cholestasis in 82% of the cases. These clinical variables included stool color within 10 days of admission, age at onset of acholic stools, birth weight, and the features of hepatic involvement, specifically the presence of hepatomegaly and consistency of the liver in palpation (6). We conducted a retrospective study between March 1996 and March 2007 to review medical records of infants with NC. Patients were all admitted in Children’s Hospital Medical Center affiliated to Tehran University of Medical Sciences, which is the major tertiary pediatric referral center in Iran. We reviewed medical records of 250 infants with NC. Infants were excluded if they did not have liver biopsy results, definite diagnosis or complete data on their medical records. Ultimately, 88 infants with BA confirmed by exploratory laparotomy or INH confirmed by liver biopsy were enrolled into the study. In this study, the gold standard tests were liver biopsy and exploratory laparotomy in INH and BA cases, respectively. The sensitivity, specificity and predictive values of HIDA scan and Alagille criteria were evaluated in comparison with liver biopsy and surgical findings. Chi-square test was used for comparison and P < 0.05 was taken as significant. There were 88 infants with NC, including 49 (55.7%) boys and 39 (44.3%) girls. Of these, 63 (37 boys, 26 girls) were diagnosed to have INH and the remaining 25 (12 boys, 13 girls) had BA, according to liver biopsy (P = 0.361). Laparotomy confirmed liver biopsy results in all 25 cases of BA. It means that liver biopsy had 100% accuracy in diagnosing BA. Thirty-three patients had HIDA scan. Among the 23 cases with a positive scan for BA (Figure 1), 14 of them also had BA in liver biopsy (P = 0.031). Among 15 patients with a positive liver biopsy for INH, HIDA scan had shown INH in only two cases (P = 0.15). Figure 1. HIDA scan report of a patient with biliary atresia Among 18 cases who had all four criteria of Alagille accommodated with INH, 14 (77.8%) cases had INH in liver biopsy (P = 0.0514). Of the five patients who had all four Alagille criteria accommodated with BA, in two (40%) cases, liver biopsy showed BA (P = 0.554). So the accuracy of Alagille criteria in our study is lower than what is reported in the pediatric GI textbooks. HIDA scan had a diagnostic accuracy of 60% and 11% for BA and INH, respectively. In conclusion, there is not a single imaging test or clinical feature that has sufficient sensitivity and specificity to differentiate BA from INH, but according to our study hepatobiliary scintigraphy had a good sensitivity for diagnosing BA and the presence of acholic stools and hepatomegaly is significantly higher in infants with BA. So, scintigraphy and clinical features can help in the assessment of any infant with NC to arrive at a timely diagnosis for appropriate management. Since percutaneous liver biopsy is highly accurate in diagnosing BA, in developing countries such our country, using this modality is cost-effective, and it can preclude unnecessary surgical exploration.

Abetalipoproteinemia (ABL), or Bassen-Kornzweig syndrome, is a rare autosomal recessive disorder of lipoprotein metabolism, characterized by fat malabsorption, hypocholesterolemia retinitis pigmentosa, progressive neuropathy and acanthocytosis from early infancy. We describe the clinical and molecular characterization of a 6-month-old infant born of consanguineous, apparently healthy parents from Iran. The patient was hospitalized because of failure to thrive, greasy stool and vomiting. The patient's serum lipid profile, the clinical phenotype and the duodenal histology suggested the clinical diagnosis of ABL. The MTP gene analysis by direct sequencing revealed a novel homozygous mutation (c.1586 A > G-H529R). The parents were heterozygotes for the same mutation and interestingly the father showed a lipid profile characterized by a slight reduction of total and LDL-cholesterol plasma levels.

Aim of Study: Chronic gastritis is defined as the presence of chronic mucosal inflammatory changes leading eventually to mucosal atrophy and epithelial metaplasia. This condition constitutes a background for dysplasia and thereby carcinoma. Detection of exact histopathology of inflammatory process is necessary in biopsy specimen. We designed the current study to determine the value of taking more sections in small gastric biopsies for better histopathologic evaluation. Materials and Methods: Gastric biopsy specimen of children who suffered from gastrointestinal (GI) symptoms was sent in 10% formalin to our laboratory. After routine processing, three slides with several sections on them were taken from the specimen: t0 he first was named the superficial section, the second was stained by Giemsa and the third was named deep section (further sections after this slide will diminish in size). The slides were not taken exactly consecutively but several sections were discarded between them. The purpose of this study is to compare the superficial and deep sections for detection of inflammatory processes. Results: In 1062 specimens the results of superficial section and deep section were the same (87.1%) and in 158 specimens the results were different. In 88 (7.2%) specimens deep section was diagnostic. The difference was seen usually as normal tissue in superficial sections but presence of lymphoid follicle in deep sections. The difference between superficial and deep sections was statistically significant. Although obtaining more sections will put an economic burden on the laboratory, we propose that in small gastric biopsies, it is helpful in better evaluation of histopathological changes.

Introduction Several conditions are considered in differential diagnosis of neonatal cholestasis. Of these the most important is extrahepatic biliary atresia (EHBA), while prompt diagnosis and surgical correction of obstruction can ameliorate clinical symptoms, provides long term survival for about one fourth of patients and serves as an important bridge to transplantation for many others. From histopathologic standpoint, features of EHBA overlaps with other diagnoses and so ancillary tests such as immunohistochemical staining for CD56 is suggested by some authors as a helpful tool in differential diagnosis. Hereby we wanted to examine this staining in our center which is a referral children hospital and to prove its efficacy in our problematic cases. Materials and Methods By retrospective review of pathology records during 2000 to 2006 in Markaze Tebbi Koodakan (children hospital related to Tehran University of Medical Sciences), we selected 17 cases of EHBA as patients and 12 cases with other diagnoses as controls, both with some degree of bile ductular proliferation in liver biopsies. EHBA cases were all proved by surgery. Four of control cases also underwent surgery but proved to have open ducts by intra-operative cholangiography. Long term follow up and other tests ruled out EHBA in other 8 cases. Hematoxylin-Eosin stains of paraffin blocks were studied again and freshly prepared sections were immunostained for CD56. Results Bile ducts and proliferating bile ductules were strongly positive for CD56 in 6 of 17 cases of EHBA. In 7 out of 17, positivity were seen in more than two thirds of portal tracts. In controls, one case showed strong positivity and 6 out of twelve showed positivity in more than two thirds of portal tracts. The intensity and distribution of CD56 staining did not differ significantly between two groups. Discussion Despite findings of previous studies, we have shown that CD56 staining can not help as an ancillary test in differential diagnosis of neonatal cholestasis and perhaps other markers should be tested in this regard.

Introduction Extra-hepatic biliary atresia (EHBA) is an important cause of neonatal cholestasis. Several infectious agents have been proposed as etiologic factors such as Rotavirus and Reovirus. There is limited data on the role of Epstein Barr virus (EBV) infection in EHBA, so we decided to study the presence of EBV virus in a series of 16 proven EHBA cases by Chromogenic in situ hybridization (CISH) technique. Methods In the current study a total of 16 liver wedge biopsies of proven cases of EHBA were selected in a period of 4 years. CISH staining for EBV-encoded RNA (EBER) transcript was performed. Results The review of H&E-stained slides of liver biopsies revealed fibrosis and marked ductular proliferation. In CISH-stained slides, EBV trace was observed in hepatocytes in two cases and in biliary epithelium in one case of EHBA. Discussion Considering the association of hepatitis with the Epstein-Barr virus in later life, it is likely that EBV hepatitis and its complications occur in the neonatal/perinatal period. Since EHBA is a relatively rare disease, a similar study on wedge biopsies of this number of proven cases of EHBA has not been performed to date. Current observation proposes the need for a study of larger series and employing other methods for confirming the etiologic role of EBV in EHBA cases.

AIM: To study the effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome. METHODS: Garlic powder in a capsule form was given to 15 children with hepatopulmonary syndrome (confirmed by contrast echocardiography) at the dosage of 1 g/1.73 m2 per day. Patients were evaluated clinically and by arterial blood gas every four weeks. RESULTS: The garlic capsule was administered to 15 patients with hepatopulmonary syndrome. There were 10 boys and 5 girls with a mean age of 9.4 ± 3.9 years. The underlying problems were biliary tract atresia (4 patients), autoimmune hepatitis (4 patients), cryptogenic cirrhosis (4 patients) and presinusoidal portal hypertension (3 patients). Eight patients (53.3%) showed an increase of 10 mmHg in their mean arterial oxygen pressure. The baseline PaO2 was 65.6 ± 12.1 mmHg in the responder group and 47.1 ± 11.2 mmHg in non-responder group. At the end of treatment the mean PaO2 in responders and non-responders was 92.2 ± 7.75 mmHg and 47.5 ± 11.87 mmHg, respectively (P < 0.01). CONCLUSION: Garlic may increase oxygenation and improve dyspnea in children with hepatopulmonary syndrome.