Role of HLA-DQ typing and antitissue transglutaminase antibody titres in diagnosing coeliac disease among Sudanese children with type 1 diabetes mellitus.

OBJECTIVE: The aim of the study was to determine the prevalence of coeliac disease (CD) and to recognise Human leukocyte antigen (HLA)-associated hereditary susceptibility to Sudanese CD patients with type 1 diabetes mellitus (DM1). DESIGN: Antitissue transglutaminase IgA (anti-TG IgA) was measured in the serum of 373 children affected with DM1 aged 1-19-year old and in 100 serum samples from non-diabetic control children. Histological examination was performed in 19 children seropositive for anti-TG IgA (17 DMI and 2 controls). Additionally, PCR-based analysis of Major histocompatibility complex, class II, DQ beta 1 (HLA-DQB1) genotyping was implemented in three study population groups as follows: group 1 (n=25) (+ve DM1 and +ve CD), group 2 (n=63) (-ve DM1 and +ve CD) and control group 3 (n=2) (+ve CD). RESULTS: Twenty-six Sudanese children with DM1 out of 373 (6.97%) were seropositive for anti-TG IgA. Duodenal biopsy revealed Marsh 2 and 3 in 13 out of 17 (76.47%) seropositive anti-TG IgA patients with DM1. Significant association (p<0.05) was detected between the level of anti-TG IgA autoantibodies (IU/mL) and Marsh stage. HLA DQ2 and DQ8 were found in 88% (22/25) and 8% (2/25) of examined patients with CD with DM1, respectively. CONCLUSIONS: Anti-TG IgA titre of greater than 10 times upper limit of normal (≥10× ULN) can be useful for detecting CD in children with type 1 diabetes without duodenal biopsy. HLA testing in children with DM1 appears to provide little added benefit given the high prevalence (96%) of HLA DQ2/DQ8 in children with DM1.

Pathologic causes of liver disease in Sudanese children: Results of 450 liver needle biopsies at a single children hospital.

The pathologic diagnoses of percutaneous 450 liver biopsies performed at the Gastroenterology Unit, Gaafar Ibnoof Specialized Children Hospital, Khartoum, Sudan during a five-year period (2005 to 2010) were reviewed. The cohort consisted of children aged between 1 month and 15 years, of whom 42% were less than 1 year of age. The male to female ratio was 1.4:1. The most common histological diagnosis was liver cirrhosis (26%), where no specific cause could be found, followed by neonatal hepatitis (20%), fatty liver (12%), billary atresia (10%), chronic hepatitis (8%), metabolic liver disease (6%), progressive intrahepatic cholestasis (5.5%), non-specific pathological changes (4.4%) and hepatocellular carcinoma in (4%). In conclusion, liver biopsy is a useful and practical tool for the appropriate diagnosis of pediatric liver diseases. Hepatocellular carcinoma has significantly higher prevalence in our pediatrics population.

Button battery induced traumatic tracheoesophageal fistula: Case report and review of literature.

Although accidental ingestions by children of various household chemicals and medicines are well described and the treatment is supported by protocols and hotlines, the ingestion of button batteries is less publicized, and the dangers are less understood by both parents and health care providers. We describe the case of a three-year-old girl, who presented to hospital with respiratory distress, cough, and fever; three weeks after the ingestion of a button battery. Endoscopic examination revealed impacted 20 mm disc battery, which was removed, and a tracheoesophageal fistula (TOF) in the upper third of the oesophagus associated with severe oesophagitis, and oesophageal ulcers. There was also evidence of oesophageal fungal infection, and severe hyperemic pan-gastritis and duodenitis. Parents were counseled and conservative management advised, but they opted for surgical repair of the TOF. The operation was done and the child survived, but she ended up with a tracheal stricture and recurrent chest infections. In conclusion, oesophageal button battery impaction places the patient at high risk for tracheoesophageal fistula. The key to successful therapy is prompt diagnosis and removal, which saves life and decreases morbidity. Because of the complications associated with this condition, patients should be managed at an institution with skilled personnel and facilities for advanced life support.

Liver Biopsy: is it Safe in Children?

Introduction: The blind liver biopsy technique has been widely used in Sudan as the availability of the ultra sound machines and the committed Pediatrics Radiologist were not always at hands. Liver biopsy is an essential tool in the diagnosis of liver diseases and subsequently; initiating the appropriate treatment. Objective: The aim of the study was to observe the safety of blind liver biopsy in our children. retrospectively. Using a standard percussion technique biopsy sites were chosen and through intercostals space blind liver biopsies were performed by TruCut biopsy needle. The study was conducted at Gafaar Ibn Oaf Specialized Children Hospital; Khartoum Sudan; over the last five years; between January 2005-January 2010. Results: The first biopsy sample was considered macroscopically adequate in 94.8of cases. A years of age and of these 8 (11.4) complained of pain at the biopsy site; external hemorrhage from the biopsy site was seen in 1 (0.6) case but no sign of internal hemorrhage was detected during the 24 hours follow up period. No child died following the procedure. Conclusions: Blind liver biopsy in the studied hospitalized children was found to be a safe procedure

Pattern of Liver Diseases in Sudanese Children

We aimed at determining the pattern and the incidence of liver disease in the Sudanese children referred to the Gastroenterology unit as Gaafar Ibn Oaf Specialized Children Hospital; which has not been studied before. Materials and Methods: In a cross-sectional study conducted over 5 years; 450 liver needle biopsies were sent to the pathology laboratory of our center. Slides were prepared from paraffinembedded blocks; stained by routine H et 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 patient were compared with other similar studies. Results: The male to female ratio was 1.5:1.The age range between 1 month and 15 years old and 42were less than 1 year old. The most common histological diagnosis was liver cirrhosis where no specific cause could be found (26) followed by neonatal hepatitis(20);fatty liver(12);Billary Atresia(10);chronic hepatitis(8); metabolic liver disease (6); Progressive Intrahepatic Cholestasis (5.5); non specific pathological changes (4.4) and Hepato Cellular Carcinoma in(4). Conclusion: A liver biopsy is a useful and practical tool for the appropriate diagnosis of pediatric liver diseases. We found that Idiopathic Liver Cirrhosis; Neonatal Hepatitis; Fatty Liver; Billary Atresia and Chronic Hepatitis in the stated order are the most prevalent histological diagnosis in Sudanese children. Hepatocellular Carcinoma is significantly high in our pediatrics population