Search

Background: Thromboembolic events are a known complication of Inflammatory Bowel Disease (IBD)especially during disease relapse, more commonly in deep veins of extremities and lung, and rarely as Cerebral Sinovenous Thrombosis (CSVT). Case Presentation: We describe an 11 year, old male patient with 3 months history of Ulcerative Colitis (UC) who presented as pseudotumor cerebri due to superior sagittal sinus thrombosis during an acute exacerbation of his colitis, that was successfully treated with heparin and then warfarin. Conclusion:In any known cases of UC presenting as acute severe headache, consider CSVT and request brain MRI and MRV to facilitate the diagnosis and early treatment. [ABSTRACT FROM AUTHOR]

Rosai-Dorfman disease (RDD) is a rare lymphoproliferative disorder with nodal and extranodal involvements. Here we report a case of RDD in a 15-year-old female who presented with epigastric pain, fatigue, Raynaud phenomenon in fingers, submandibular lymphadenopathy, proptosis, hepatosplenomegaly, and round shape painless patches on the extensor surfaces. Histological examination of the submandibular lymph nodes and skin biopsy demonstrated evidences of RDD. Patient was treated with prednisone and thereafter, with azathioprine. After one year, prednisone was discontinued and all of the symptoms and signs, except proptosis, were resolved. This report highlights the extranodal manifestations of RDD. The presentation, differential diagnosis, and treatment are discussed. [ABSTRACT FROM AUTHOR]

Practicing medicine according to the best evidence is gaining popularity in the medical societies. Although this concept, which is usually called Evidence Based Medicine (EBM) has been explained in many resources, it has not been addressed enough in pediatrics. In this review, we briefly explained Evidence Based Medicine approach and its applications in pediatrics in order to help the pediatricians to efficiently integrate EBM into their daily practice. [ABSTRACT FROM AUTHOR]

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. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background/aims: The current systematic review and meta-analysis study assessed the prevalence of celiac disease (CD) in Iran., Materials and Methods: Electronic databases, including MEDLINE, SCOPUS, Web of Science, Cochrane library Collaboration, and Iranian scientific databases, were searched from 1993 to 2013 for English and Persian articles. The following terms were used, alone or combined, "celiac (MeSH)," "ceoliac," "prevalence (MeSH)," and "Iran*." Heterogeneity was assessed using the I2 statistic with a cut-off value of 50%, and the Chi-square test was used to define a statically significant degree of heterogeneity with a p value of <0.10. The publication bias of literatures was assessed by visual examination of the funnel plot and Begger's funnel plot., Results: Meta-analysis was conducted on seven publications with 9,720 subjects. Overall, the pooled prevalence of CD among the Iranian population was 0.72% [95% confidence interval (CI): 0.62%-0.98%]. There was no significant heterogeneity among the studies (I2=4%, p=0.396). The pooled prevalence of CD on the basis of IgA-anti tissue transglutaminase (tTGA) and tTGA and duodenal biopsy positivity was 0.83% (95% CI: 0.69%-1.14%) and 0.79% (95% CI: 0.66%-1.09%), respectively. No significant publication bias was observed using the funnel plot and Begger's funnel plot., Conclusion: CD prevalence among the Iranian population was approximately similar to that of the American and European populations.

Zellweger syndrome (ZS) is a peroxisomal disorder with a multiple congenital anomalies, characterized by stereotypical facies, profound hypotonia, organ involvement including cerebral, retinal, hepatic, and renal. Herein, a 3-month-old female with ZS is presented who was referred because of increased liver enzymes (subclinical hepatitis), which was detected in work-up of her neck cyst, severe hypotonia, and abnormal facies. An increased concentration of very long chain fatty acid in lipid profile was detected. ZS should be considered in the list of differential diagnosis in infants with stereotypical phenotype, neurodevelopmental delay, and severe hypotonia in association with liver and other organs involvement.

Background/aims: Cystic fibrosis is the most common inherited lethal disease, which could be frequently identified late in regions 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 failure to thrive, recurrent respiratory infections, steatorrhea, metabolic alkalosis, and salty tasting skin should be considered as clinical 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.

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 m(2) 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 PaO(2) 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 PaO(2) 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.