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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:: Adenosine, a signaling nucleoside, is controlled in part by the enzyme adenosine deaminase (ADA). There are rare reports on the role of adenosine levels and ADA in cystic fibrosis (CF) patients. Objectives:: The aim of this study was to assess serum ADA in CF patients in order to find whether the severity of lung disease in CF is related to significant changes of ADA or not. Patients and Methods:: Venous blood serum ADA was measured in CF patients (3-15 years) and 49 healthy children (3-15 years) referred to Children’s Medical Center. Classification of respiratory and gastrointestinal disease severity in CF patients as well as Body Mass Index (BMI) was performed. The results were compared with values obtained from healthy children matched for age and gender. Results:: This study included 49 children of both genders (20 females and 29 males) with CF (mean age: 6.36 ± 2.22 years). Mean serum ADA in CF patients group and control group was 9.38 ± 2.72 and 16.04 ± 1.27, respectively (P value = 0.001). Mean serum ADA in CF patients with normal BMI was higher than in patients with low BMI (P value = 0.002). Conclusions:: In this study the lower serum level of ADA was seen in CF patients compared to control group. The clinical symptoms, especially respiratory symptoms, in CF patients might be associated with reduction of serum ADA and rising serum adenosine; therefore, further studies on the use of ADA enzyme therapy in CF patients are highly recommended.

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.

Objective: Celiac disease is an autoimmune disorder in which the risk of autoimmune liver disease is high. Autoimmune hepatitis is a chronic and progressive entity and the risk of its being associated with other autoimmune disorders such as celiac disease is high also. The aim of this study was to determine the prevalence of celiac disease in patients with autoimmune hepatitis and vice versa. Methods: In a cross-sectional study children with autoimmune hepatitis underwent serological screening and endoscopy for celiac disease. In patients with celiac disease, serum aminotransferases were measured and, if abnormal, autoantibodies related to autoimmune hepatitis were checked and needle liver biopsy was performed. Findings: Of the 96 patients, 64 had autoimmune hepatitis and 32 celiac disease. Among patients with autoimmune hepatitis only three (4.7%) were compatible with celiac disease. In the group of patients with celiac disease, autoimmune hepatitis was confirmed in four (12.5%) cases. We consider important to state that 3.1% of this group had celiac hepatitis. Conclusion: Autoimmune liver disease is sometimes associated with latent celiac disease. Serological screening for celiac disease should be routinely done in patients with abnormal serum aminotransferases, particularly those with chronic liver disease. On the other hand, celiac disease is often accompanied by other autoimmune diseases, including autoimmune hepatitis.

Objective: Extrahepatic biliary atresia (EHBA) is one of the main causes of neonatal cholestasis. Its early diagnosis could increase the survival of the infants with early surgery. We evaluated the diagnostic accuracy of procalcitonin and apolipoprotein E (Apo-E) levels in infants with and without EHBA. Methods: This prospective study included 18 infants with EHBA and 15 infants with other causes of cholestasis. Blood samples were taken from each patient and different markers including procalcitonin and Apo-E levels were measured. ROC analysis was used to define sensitivity, specificity, positive and negative predictive value (PPV and NPV) for procalcitonin and Apo-E. Findings : There was a significantly positive correlation between Apo-E and SGOT (r=0.37, P=0.03), SGPT (r=0.38, P=0.02) and GGT (r=0.38, P=0.02), and an inverse correlation between procalcitonin and GGT (r=-0.45, P=0.01). Area under curve (AUC) for procalcitonin was 0.69 (P=0.05) with cut-point of 0.735 ng/ml. The sensitivity, specificity, PPV and NPV was 67%, 61%, 69% and 59%, respectively. AUC for Apo-E was 0.68 (P=0.06) for cut-point of 61.25 ng/ml with sensitivity, specificity, PPV and NPV of 67%, 67%, 71% and 67%, respectively. Conclusion: Both PCT and Apo-E have relatively good accuracy in diagnosing EHBA cases; we could not rely on these markers for diagnosis of EHBA, however, combinations of these biomarkers with other markers and imaging tests could improve their accuracy and may help to achieve a rapid and accurate diagnosis of EHBA.

Objective A variety of sign, symptoms and laboratory findings are more common in children with organic abdominal pains. This study was performed to evaluate the prevalence of organic and functional abdominal pains and relation of red flags to organic pains in 100 children with recurrent abdominal pain (RAP). Methods One hundred consecutive patients with RAP were enrolled in the study. A complete interview and physical examination was made for each patient, accompanied by a series of laboratory, clinical and para-clinical examinations. The data were recorded and analyzed. Logistic regression analysis was used to model and formulize correlations between sign, symptoms, and laboratory findings with organic and functional abdominal pain. Findings Among 100 patients (52% male, 48% female, Age: 9.29±3.17) diagnostic works up revealed organic pain for 57 patients. The most common symptoms of the patients included constipation, diarrhea, chest pain, cough, headache, vomiting, hematuria, and dysuria. Fecal incontinence, delayed puberty, organomegaly, jaundice, and family history of inflammatory bowel disease were reported in none of the patients with RAP. Fever, pain not located in periumbilical area, nocturnal pain, elevated erythrocyte sedimentation rate, weight loss, growth disorder, and abdominal tenderness were among the red flags which revealed diagnosis of organic pain in this study. Conclusion A series of red flags could increase likelihood of finding organic pain in children with RAP.

Objective Recurrent abdominal pain (RAP) by itself is one of the common reasons in child-aged patients to refer to a clinician. Some of these patients are presented with more serious features, so-called the “red flag”. The most important issue in management of RAP is to distinguish the type of it, whether it is functional or organic. In this study we aimed to assess the redundancy of red-flagged RAP with findings of esophago-gastro-deudonoscopy. Methods In a 2 year prospective study 150 consecutive children with RAP who showed red flags underwent esophago-gastro-deudonoscopy. The prevalence of each finding was recorded. Overall positive predictive value of predicting an endoscopic finding while having a red-flag was calculated. Findings Among all the patients, 126 cases showed at least a positive finding in their endoscopy that corresponded to the positive predictive value of 84% for predicting the presence of an endoscopic finding according to red flags. Interestingly, 20% of patients showed hiatus hernia when surveyed. Conclusion Comprehensive physical examination is needed to avoid performing esophago-gastro-deudonoscopy without indication in patients with recurrent abdominal pain.

Objective Wilson's disease (WD) is an autosomal recessive disease with genetic abnormality on chromosome 13 causing defect in copper metabolism and increased copper concentration in liver, central nervous system and other organs, which causes different clinical manifestations. The aim of this study was to determine the sensitivity of different clinical and paraclinical tests for diagnosis of Wilson's disease. Methods Paraffin blocks of liver biopsy from 41 children suspicious of WD were collected. Hepatic copper concentrations were examined with atomic absorption spectrophotometry (Australian GBC, model: PAL 3000). Fifteen specimens had hepatic copper concentration (dry weight) more than 250μg/g. Clinical and laboratory data and histologic slides of liver biopsies of these 15 children were reviewed retrospectively. Liver tissue was examined for staging and grading of hepatic involvement and also stained with rubeonic acid method for copper. Findings Patients were 5-15 years old (mean age=9.3 years, standard deviation=2.6) with slight male predominance (9/15=60%). Five (33%) patients were 10 years old. Three (20%) of them were referred for icterus, 8 (54%) because of positive family history, 2 (13%) due to abdominal pain and 2 (13%) because of hepatosplenomegaly and ascites. Serum AST and ALT levels were elevated at the time of presentation in all patients. In liver biopsy, histological grade and stage was 0-8 and 0-6 respectively, 2 (13%) had cirrhosis, 1 (7%) had normal biopsy and 12 (80%) showed chronic hepatitis. Hepatic copper concentrations were between 250 and 1595 μg/g dry weight. The sensitivity of various tests were 85% for serum copper, 83% for serum ceruloplasmin, 53% for urinary copper excretion, 44% for presence of KF ring and 40% for rubeonic acid staining on liver biopsies. Conclusion None of the tests stated in the article were highly sensitive for diagnosis of WD, so we suggest that diagnosis should be based on combination of family history, physical examination and different tests.

Objective Reinfection rate of Helicobacter pylori after successful eradication is low in developed countries. This study was performed to determine the reinfection rate of H. pylori during a follow up period of 12 months in Iranian children. Methods In this prospective study, children with H. pylori infection were treated with triple omeprazole based regimen. Patients with negative 13C urea breath test (UBT) performed after 8 weeks of therapy, were followed up by the same test after 1 year. Findings Thirty seven patients, aged 5 to 17 years, were studied. Among them 25 (67.5%) were boys. After eradication therapy of H. pylori, 34 patients had negative 13C UBT. Reinfection occurred in 5 (14.7%) patients. Reappearance or continuing symptoms after treatment were associated with higher rate of recurrence (P=0.042). Conclusion Recurrence rate of H. pylori was high in our children. Successful eradication significantly decreased complaints and further symptoms. Follow up and reevaluation of patients is necessary especially when there are symptoms after eradication.