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The occurrence of rectosigmoid junction inflammatory myofibroblastic tumor (IMT) is uncommon in children. This is a rare form of mesenchymal tumor, belonging to the category of soft tissue tumors, and can be found at any anatomical site from the central nervous system to the gastrointestinal tract. Our patient was a 10-year-old male subject complaining of lack of defecation and constipation. The patient had decreased the frequency of defecation and constipation about two weeks before his referral and had not improved despite the use of laxatives. The abdomen was completely distended and there was no tenderness or guarding in the examination. Several airfluid levels are shown on the abdominal X-ray. In the ultrasound, free fluid was reported in the interlobular and pelvic spaces. The patient was transferred into the operating room. A tumor of the rectosigmoid junction was detected. Histopathologic studies showed evidence of IMT. IMT is a rare neoplasm of unknown origin, which may occur in various sites of the body. Complete surgical removal is usually curative, but early detection of recurrence is required. Treatment options include chemotherapy, radiation therapy, and immunotherapy. Further investigations are needed to improve the understanding and management of this rare tumor. [ABSTRACT FROM AUTHOR]

Context: The resistance of bacteria to commonly prescribed antibiotics is increasing both in developing as well as developed countries. Resistance has emerged even to newer, more potent antimicrobial agents. The present study was therefore undertaken to report resistance rates to antimicrobial agents in 2487 stool culture isolates in a tertiary care hospital between 1996 and 2000 and 2001 and 2005. Materials and Methods: During 1996 to 2005, 31776 fecal samples were collected from all patients having diarrhea aged> 1 month to 14 years old. Microbiology records were reviewed and information on each isolate regarding its antimicrobial susceptibility profile was collected and recorded. Statistical Analysis Used: The statistical analysis was performed using SPSS, Version 11.5 software. Results: Of the positive fecal cultures, 1329 (53.43%) of the isolates were Shigella spp and 700 (28.14%) of the isolates were Salmonella spp. Resistance to antimicrobial agents increased among most of the pathogens between 2001 and 2005. An increase in the rate of resistance was observed in Shigella spp for kanamycin (from 11 to 37%) and ceftazidime (from 1 to 9.9%) and among Salmonella spp. for nalidixic acid (from 9.2 to 42.3%) and ceftazidime (from 3 to 23.4%). Conclusions: Routine surveillance of antimicrobial susceptibilities to all classes of clinically used agents is necessary to detect resistance trends in different parts of world, detecting the emergence of new resistance mechanisms that guide infection control measures and public health guidelines; such trends may help in identifying outbreaks of resistant organisms. Such a check seems to be the best way to find appropriate antibiotic regimens

We aimed at determining the pattern of liver disease in the Iranian children referred to the Medical Center of Children affiliated with the Tehran University of Medical Sciences. Materials and Methods: In a cross-sectional study conducted over 2 years, 425 liver needle biopsies were sent to the pathology laboratory of our center. Slides were prepared from paraffin-embedded blocks, stained by routine H & E and special stains and were then reviewed. The frequency of each disorder, separately and in combination with the age group or gender of the patients was calculated and compared with other similar studies. Results: The male to female ratio was 1.42:1. The age range was between 1 month and 18 years old and 41.4% were less than 2 years old. The most common histological diagnosis was iron overload due to major thalassemia (17.5%) followed by biliary atresia (9.7%), no significant pathologic change (8.7%), neonatal hepatitis (8.7%), chronic hepatitis (8.5%), cirrhosis (6.5%), metabolic disease (5.5%) and progressive familial intrahepatic cholestasis (5%). Results of the hemosiderosis grading in patients with thalassemia revealed no or minimal, mild, medium, or marked increase in 10%, 27.1%, 10%, 21.4% and 31.5% of the cases, respectively and the degree of iron deposition rose in parallel with age and also the stage of fibrosis (p< 0.05). Conclusion: A liver biopsy is a useful and practical tool for the appropriate diagnosis of pediatric liver diseases. Also, we found that in non thalassemic children, biliary atresia, chronic hepatitis and neonatal hepatitis, in the stated order, are the most prevalent histologic diagnoses in Iranian pediatrics.

Background: During COVID-19 pandemic, most studies have focused on sampling technique in adults. Considering the need to be aware of the effectiveness and evaluation of sampling methods in children, we have motivated a search for introducing and performing sampling techniques, especially upper respiratory tract sampling in children. We systematically reviewed the literature to understand the performance of different sampling methods in children in COVID-19. Methods: We systematically reviewed PubMed, Google Scholar, medRxiv, and bioRxiv (last retrieval August 1st, 2021) for comparative studies of deferent sampling techniques by using the search keywords including: children, pediatric sampling, nasopharyngeal, COVID-19, oropharyngeal, swabs, SARS, CoV2. 8 relevant manuscripts were sourced from a total of 4852 search results. Results: Nasopharyngeal (NP) swabs testing significantly had higher positivity rate over oropharyngeal swab in detecting SARS-CoV-2. Nasal swab has a low sensitivity in detecting SARSCoV-2 in children when referred to the Nasopharyngeal Aspiration (NPA), whereas its specificity is high. Therefore, NPA can be as the gold standard for detection of SARS-CoV-2. Conclusion: Saliva is not a useful for diagnosing COVID-19 in children. Negative nasopharyngeal and oropharyngeal swabs do not rule out COVID-19 and in patients with strong clinical suspicion, and Bronchoalveolar lavage (BAL) can be helpful. [ABSTRACT FROM AUTHOR]

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.

Abstract Introduction To the best of our knowledge, the association between air travel and intra-tumoral hemorrhage in pediatric populations has never been described previously. Case presentation We report the case of a two-and-a-half-year-old Caucasian, Iranian boy with a hemorrhaging brain tumor. He had a posterior fossa midline mass and severe hydrocephalus. He had been shunted for hydrocephalus four weeks earlier and was subsequently referred to our center for further treatment. The hemorrhage occurred in an infra-tentorial ependymoma, precipitated by an approximately 700-mile air journey at a maximum altitude of 25,000 feet. Conclusions A pre-existing intra-cranial mass lesion diminishes the ability of the brain to accommodate the mild environmental disturbances caused by hypercarbia, increased venous pressure and reduced cerebral blood flow during long air journeys. This is supported by a literature review, based on our current knowledge of physiological changes during air travel.

Abstract 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.

Abstract 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.

Chronic granulomatous disease (CGD) is a rare genetic disorder of neutrophil activity, resulting in increased rate of recurrent infections with catalase-positive bacteria and fungi, as well as various autoimmune diseases such as sarcoidosis, rheumatoid arthritis, and discoid and/or systemic lupus erythematosus. Few reports have reported lupus erythematosus (LE) in patients with X-linked CGD (XL-CGD) and carriers, and very few in autosomal recessive CGD (AR-CGD). Here, we present 5 patients with CGD developing LE at different ages to emphasize on the importance of appropriate follow-up and treatment in patients with CGD with clinical signs and symptoms of autoimmune diseases and even in those with negative serologic results. [ABSTRACT FROM AUTHOR]

Objective: Henoch-Schonlein purpura (HSP) is a common vasculitis in children that can present with multi-organ involvement. The aim of this study is to investigate the correlation between direct immunofluorescence (DIF) results and the systemic involvements of the HSP in pediatric patients. Material and Methods: Those HSP patients with leukocytoclastic vasculitis on their biopsies who also had documented immunoglobulin/complement deposition by DIF were included in our study. Their demographic and laboratory data and clinical manifestations were recorded and analyzed. Results: Medical records of 95 patients (1.5–15 years old) were studied. 26.3% of the patients showed renal, 86.3% articular, and 70.3% gastrointestinal involvement. The risk of renal involvement was significantly higher in those with C3 deposition in their skin DIF. IgM deposition was mostly associated with articular involvement. Conclusion: Pediatric HSP patients who had C3 deposition in their skin DIF should be selected for further evaluation regarding HSP nephritis. [ABSTRACT FROM AUTHOR]

The article presents the case of a 47-year-old male with well-compensated cirrhosis (Na-MELD 18) and hepatoma secondary to autoimmune hepatitis to discuss the acute complication of liver transplantation called seventh-day syndrome (7DS) managed with ABO-incompatible (ABOi) transplant.

Background: Brain damage and mental retardation in children can be caused by either excess or depletion of certain amino acids. Up to now there is no reliable reference interval for free plasma amino acid in Iranian children. Objectives: The aim of this study was to provide the reference intervals of free plasma amino acid in healthy Iranian children using high performance liquid chromatography (HPLC) as an accurate method. Methods: 133 children referred to Children's Medical Center, Tehran, Iran for the annual checkup entered the study after filling out the informed consent form. Demographic data and blood samples after overnight fasting were collected. Plasma amino acid concentrations were determined by reverse phase HPLC (RP-HPLC) equipped with a fluorescence detector after precolumn derivatization of amino acids with o-phthalaldehyde (OPA). Results: Female children exhibited significantly higher plasma concentrations of taurine and lysine than male children. Other 20 amino acid values were not significantly different between two gender groups. Positive correlation was observed between age and taurine, leucine and lysine. Conclusions: Our data provided reference intervals of free plasma amino acids in healthy Iranian children. We believe our data can be used as a guideline for pediatricians to identify the significantly deviated amino acid values in children with medical issues such as genetic disorders and some acquired illnesses. Our data is also applicable for further investigations in the field of family medicine. [ABSTRACT FROM AUTHOR]

Objective:Angiotensin converting enzyme (ACE) converts angiotensin I into angiotensin II. The ACE gene shows an I/D polymorphism, which correlates with ACE concentrations. The aim of this study is to evaluate the distribution of the ACE I/D genotype in children with idiopathic nephrotic syndrome (INS) and healthy controls and study the effect of this polymorphism on clinical and pathologic findings.Methods:ACE gene I/D polymorphism of 104 patients with INS and 119 controls were determined.Results:The DD, ID, and II genotypes were found in 58.7%, 22.1%, and 19.2% of the patients, and in 79.8%, 2.5%, and 17.6% of controls, respectively (p> 0.05). The ID genotype was seen more frequently in patients resistant to treatment.Conclusion:The observed differences with previous reports suggest the influence of the genetic background on disease course. The ACE I/D gene polymorphism's role seems to be more important in renal disease progression than susceptibility. [ABSTRACT FROM PUBLISHER]

Background: Shigellosis is a diarrheal disease caused by Shigella spp. The majority of cases and deaths occur among children less than 5 years old. In severe cases, antibiotic therapy is recommended to lessen the risk of serious complications and death. Prevalence of different Shigella species and their antibiotic resistance patterns are changing over the time. Objectives: The current study aimed at assessing the changes in the prevalence of Shigella species and their antibiotic susceptibility among 0- to 14-year-old children referred to Children’s Medical Center, Tehran, Iran, from 2009 to 2014. Methods: The results of stool cultures were retrospectively analyzed to determine the prevalence of different Shigella species, their antibiotic susceptibility patterns and their changes in the largest university affiliated pediatrics center in Tehran. Results: Among 40 700 stool cultures, 507 cases were positive for Shigella spp. Most cases occurred among children under 5 years old. Shigellosis was more prevalent among males. The most common species were Shigella sonnei and Shigella flexneri. In general, they were most sensitive to cefotaxime and most resistant to cotrimoxazole. Resistance to cefotaxime and nalidixic acid increased annually. Resistance to ampicillin had a descending trend. Resistance to cotrimoxazole remained almost constant. Conclusions: The most common species of Shigella changed from Shigella flexneri to Shigella sonnei over the years. In comparison with the previous studies, resistance to nalidixic acid increased and resistance to ampicillin decreased. The prevalence and antibiotic resistance patterns of Shigella species, at different times and regions are changing. To make appropriate decisions on treatment, it is necessary to monitor the changes. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described in a group of patients with common variable immunodeficiency (CVID) in association with autoimmunity and/or inflammatory bowel disease (IBD)-like phenotype. We here describe a 10-year-old boy who experienced recurrent infections, mainly in the respiratory system, associated with thrombocytopenia and anemia. Immunological workup showed low numbers of B cells and low IgG, but normal IgM levels. In spite of therapeutic doses of antibiotics, antivirals, and antifungal agents, in addition to immunoglobulin replacement therapy, he developed disseminated involvement of both lungs with peripheral nodules; transbronchial lung biopsy revealed possible bronchiolitis obliterans organizing pneumonia (BOOP). Combined homozygosity mapping and exome sequencing identified a homozygous LRBA mutation in this patient (p.Asp248Glufs*2). Such clinical and immunological findings have not been described to date and illustrate the broad and variable clinical phenotype of human LRBA deficiency. [ABSTRACT FROM AUTHOR]

We presents an infant with several indurated plaques and nodules scattered on her body. She was brought to the hospital because of fever, runny nose and cough from one month ago. During the examination and investigation the plaques and nodules grabbed the attention of the clinicians and the skin biopsy and other lab works revealed the diagnosis of congenital leukemia. [ABSTRACT FROM AUTHOR]

Background. H. pylori infection leads to chronic gastritis in both children and adults. But recently, there are arising theories of its protective effect in diarrheal diseases. Aim. To explore the prevalence of H. pylori infection in children with bacterial diarrhea and compare it with healthy controls. Patients and Methods. Two matched groups consisted of 122 consecutive children, aged 24-72 months old, with acute bacterial diarrhea, who had Shigellosis (N = 68) and Salmonellosis (N = 54) as patients group and 204 healthy asymptomatic children as control group enrolled in this study. Results. The prevalence of H. pylori infection in healthy control children was significantly higher than in patients group, (odds ratio = 3.6, 95% CI: 1.33-9.5, P = 0.007). In our study, only 2/54 Salmonella infected patients and 3/68 of Shigellosis had evidence of H. pylori infection, while normal control children had 27/204 infected individuals. Conclusion. H. pylori infection may play a protective role against bacterial diarrhea in children. So it is important to consider all of the positive and negative aspects of H. pylori infection before its eradication. [ABSTRACT FROM AUTHOR]

Objective: Shiga toxin-producing E. coli (STEC) is a pathogenic E. coli that may cause hemolytic uremic syndrome (HUS) after diarrheal disease through Shiga toxins. Management of the patients with STEC infection is different from that of other diarrheal diseases due to increase in frequency of HUS after antibiotic administration. Few studies were conducted in Iran and epidemiology of STEC remains obscure; this necessitates examination of stools especially in young children for this bacterium. Methods: We determined the frequency of STEC in 947 E. coli strains isolated from diarrheal stools of children less than 14 years in Tehran with conventional culture methods and multiplex-PCR via determining the STX1 and STX2 genes, between October 2008 and September 2009. We also evaluated the association between stool exam findings and presence of STEC. Findings: Twenty seven (2.8%) of E. coli isolates were positive for STX1 or STX2 genes, most of which occurred in spring (P<0.05). There was no significant association between STEC positivity and stool exam findings. Eighteen out of 27 (66.7%) Shiga toxin positive samples were isolated from males and the rest from females. The most common STX-positive diarrheal samples showed loose consistency (P<0.017). Conclusion: Although the low frequency of STEC in our population indicates that it is not a major problem in our population, STEC should be regarded as an important infection because of its severe consequences. Further studies with greater sample size are needed to confirm our results. [ABSTRACT FROM AUTHOR]

Objective: Alpha1-antitrypsin deficiency (A1ATD) is the most important indication for liver transplantation in children. The gene frequencies vary in different ethnic groups. In the present study, we attempt to determine the frequencies of the most common defective alleles, Z and S, in Iranian children suffering from idiopathic neonatal cholestasis. Eighty-seven infants were typed for Z and S alleles. Methods: In a single center study, 87 consecutive liver biopsies from infants with cholestasis were reviewed and patients with neonatal cholestasis enrolled in the study and cases with confirmed biliary tract atresia excluded. Formalin fixed paraffin embedded blocks were used for DNA extraction. AAT genotype was determined by polymerase chain reaction (PCR) assay and amplification of the two most common deficiency variants, S and Z alleles, and then sequencing of PCR products. Findings: There were 48 (55.2%) males and 39 (44.8%) females, with a median age of 60 days. Out of 87 of the study subject, 2 (2.2%) were heterozygous for the S allele, and no ZZ, SS or MZ individual was found in the patients. No other polymorphism was found in the sequencing results. Conclusion: In comparison to other populations, AAT deficiency seems not to be an important etiologic factor for neonatal cholestatic liver disease in Iran; however, further studies are recommended to estimate the true mutant gene frequencies. [ABSTRACT FROM AUTHOR]

Objective: Exposure to environmental tobacco smoke (ETS) is one of the major factors of predisposing children to develop several hazardous health problems. We decided to investigate the association between nicotinine, one of the nicotine metabolites and esophagitis in children with gastroesophageal reflux disease (GERD). Methods: In a case control study 46 children suffering from esophagitis referred to endoscopy ward were recruited. The control group consisted of 45 healthy children. Urine samples were collected and urinary cotinine level (UCL) measured. Findings: The mean age of esophagitis and control groups were 5.11±2.93 and 6.72±2.8 respectively. Sixty children were passive smokers; 31 of them had non-smoker parents. In control group, 32 (71.1%) children and in esophagitis group 29 (63%) children had non-smoker parents. The mean value of UCL in patients suffering from esophagitis was significantly higher than those in normal group (P=0.04, 24.98±6.4 ng/ml vs. 15.16 ± 3.9 ng/ml). Considering 50ng/ml as a cutoff point for UCL, it was significantly higher in passive smoker group than in non smoker group (P=0.02). The mean cotinine level differed significantly in esophagitis and control group. Conclusion: Our results indicate the increased risk of developing esophagitis in children with ETS exposure. [ABSTRACT FROM AUTHOR]

Familial recurrent molar pregnancy is an exceedingly rare condition, in which complete hydatidiform moles are mostly diploid but biparental in origin and the outcome of subsequent pregnancies is likely to be a hydatidiform mole or other type of reproductive loss. We previously reported a case of familial molar pregnancy (family K) comprising five affected members (four sisters and one of their cousins) each with at least one hydatidiform mole (HM). In addition to the molar pregnancies, these patients have a total of three miscarriages and 8 normal pregnancies leading to healthy children; but the youngest member of this family has given birth to a boy with Down syndrome.Our second family (case S) includes two sisters with diploid biparental complete moles. They have a total of six molar pregnancies with no living child. Recently the younger sister had a partial molar pregnancy with apparently normal XX fetus accompanying diffuse molar changes of the placenta that led to preeclampsia and preterm delivery. Overall, these families have had 26 pregnancies including 12 molar pregnancies (complete or partial) and three abortions. We concluded that these families are predisposed to various genetic mutations, chromosomal abnormalities and clinical manifestations, which affect their offspring. Further studies of patients are needed to determine any relationship between a history of familial molar pregnancy and trisomy or other chromosomal abnormalities in offspring and genetic mutations in the products of conception to complete the puzzle and manage familial molar pregnancy. [ABSTRACT FROM AUTHOR]

Erythropoietic protoporphyria (EPP) is an inherited defect of mitochondrial ferrochelatase. This defect results in accumulation of protoporphyrin in erythrocytes, plasma, liver, and skin, which causes severe photosensitivity. Liver disease can occur in 1-4% of the patients with EPP, usually after at least a decade of photosensitivity. Herein, we describe a 1.5-year-old child with EPP with severe photosensitivity, heart abnormalities and early onset of cholestatic liver disease, whose clinical condition improved gradually after using ursodeoxycholic acid. It seems that liver disease in EPP patients is not limited to the late phases of the disease and could develop in childhood and early phases of EPP. Awareness among physicians has a major role in the early detection and prevention of mistreatment of EPP in case of its combination with other abnormalities. [ABSTRACT FROM AUTHOR]

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 µ/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. [ABSTRACT FROM AUTHOR]

Objective: Acute appendicitis is the most common cause of abdominal surgery in children. Similarity between signs and symptoms of appendicitis and other common pediatric illnesses, atypical manifestations of appendicitis in young children, and children's inability to give precise explanation for their symptoms contribute to considerable delay in proper diagnosis and increased rate of perforation. Current study reports the surgical and pathological findings of appendectomies in the largest Children's Hospital in Iran. It also evaluates whether common protocol for pathologic evaluation following appendectomy is beneficial. Methods: Pathologic reports of 947 appendectomies, performed with the presumptive diagnosis of acute appendicitis, were gathered. Correlation between surgical and pathologic findings was assessed. Demographic characteristics of patients between surgical and pathological subgroups were also compared. Findings: The mean age of participants was 6.9+3.5 years. Eighty seven (25.5%) children had abnormal pathological findings and normal surgical report. None of miscellaneous findings including appendicular carcinoid tumor 3 (0.3%), oxyuriasis 2 (0.2%), and mycobacterial infection 4 (0.5%) were recognizable during the surgery. Of all pathologically confirmed cases with perforated appendicitis, 9.7% were not detected during the surgery. Conclusion: In current study, acute appendicitis was the most common pathological diagnosis, however, high normal appendectomy rate along with noticeable proportion of surgically missed perforated appendicitis and unusual histopathologies strongly supported routine histological examination. [ABSTRACT FROM AUTHOR]

Background and Objectives: Infection with parvovirus may induce spontaneous abortion, non- immune hydrops fetalis, and intrauterine death in full term infants. The aim of this study was to determine the frequency of parvovirus B19 in paraffin-embedded formalin fixed placental tissues in lost fetuses by PCR method and comparison with its frequency in healthy full term neonates as controls. Materials and Methods: In a case control study, thirty-one formalin fixed paraffin embedded placental tissue from autopsies related to fetal losses and also the same number of control cases were collected and the PCR for internal control and target sequence were performed. Results: Thirty-one subjects and 31 controls were enrolled and three out of 31 cases were positive for parvovirus (9.7%) using PCR. Hydropic changes were noted in two of positive cases for parvovirus. Conclusion: Parvovirus B19 infection during pregnancy may cause red precursor cells damage resulting in anemia and congestive heart failure. We suggest that studies with greater sample sizes are carried out to determine the frequency and management of parvovirus B19 infection because of timely diagnosis and transfusion of severe hydropic fetuses can reduce the risk of fetal death. [ABSTRACT FROM AUTHOR]

Objective: Hirschsprung's disease (HD) is a complex disorder resulting from absence of ganglion cells in the bowel wall leading to functional obstruction and bowel dilatation proximal to the affected segment. The aim of our study was to evaluate rectal biopsies from constipated children in different age groups to see in which age it is more likely to encounter HD to avoid unnecessary rectal biopsy. Methods: Records of all children with chronic constipation undergoing a rectal biopsy to exclude HD were obtained from the files of Children's Medical Center in Tehran, Iran. A detailed retrospective demographic review, including age of beginning of signs and symptoms was made of all cases. Findings: Totally, 172 biopsies were taken from 168 children in a five year period, of which 127 cases (75%) had HD. The mean age of constipated patients at biopsy was 39 months and the mean age of patients with proven HD was 18 months. Males were affected more than females. Congenital anomalies associated with HD were found in 9.6%. In 85 (91%) cases constipation had begun in neonatal period. Conclusion: Our data supports previous studies that if constipation begins after the neonatal period, the child is unlikely to have HD. In neonates delay in meconium passage is the most important clinical sign of HD. [ABSTRACT FROM AUTHOR]