Arrhythmogenic right ventricular dysplasia: a sudanese experience

Arrhythmogenic right ventricular dysplasia cardiomyopathy [ARVDC] is a genetic cardiomyopathy characterized by ventricular arrhythmias and structural abnormalities of the right ventricle [RV].There were no reported studies on the presentation of Sudanese patients with ARVDC. This study aimed to describe patient characteristics and various manifestations of ARVDC among 4 patients seen at Shaab Teaching Hospital [STH] during the period from 2006-2010. This study was carried out in STH. STH is the central reference cardiology hospital in Sudan with the final diagnosis of ARVDC. Initially, none of those patients was diagnosed as having ARVDC. The first patient was treated as myocardial infarction and epilepsy. The second patient was diagnosed as Epilepsy. Both the third and fourth patients were treated for palpitations and the fourth patient was diagnosed as having heart failure as well. The first Patient died suddenly while the others stayed alive and have come for follow-up. They were prescribed anti-arrhythmic drugs, and showed remarkable improvement. The diagnosis of these cases based primarily on findings obtained from clinical assessment and simple laboratory tests, as neither MRI nor endomyocardial biopsy were available. Awareness of the protean presentations of ARVDC shouldn't be overlooked. Palpitation isn't always benign. Big heart shadows in X-rays may have many causes. T-wave changes not necessarily ischemia and Loss of consciousness isn't synonymous with epilepsy only. A national registry is a necessity

Liver biopsy: is it safe in children?

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. The aim of the study was to observe the safety of blind liver biopsy in our children. One hundred fifty consecutive liver biopsies in hospitalized children were evaluated 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. The first biopsy sample was considered macroscopically adequate in 94.8% of cases. A definitive histological diagnosis was possible in 99.1% of cases. seventy children were more than 5 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. 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. 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 paraffin-embedded blocks, stained by routine H and 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. The male to female ration was 1.5:1. The age range between 1 month and 15 years old and 42% were 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%], chornic hepatitis [8%], metabolic liver disease [6%], Progressive Intrahepatic Cholestasis [5.5], non specific pathological changes [4.4%] and Hepato Cellular Carcinoma in [4%]. 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

Result of eradication of esophageal varices in children with extra hepatic portal venous obstruction in Sudan

Extra hepatic Portal Vein Thrombosis is the main cause of Portal hypertension in children in Sudan, which is responsible for almost 30% of heamatemsis in children in Sudan. Treatment is targeted at the complications and includes primary and secondary prophylaxis against upper gastrointestinal bleeding [which results from the rupture of esophageal varices] which is usually a combination of endoscopic sclerotherapy and/or band ligation to eradicate the varices, Porto systemic shunting in selected cases, medical prophylaxis, and to support the child growth and development. In this study we looked beyond the eradications of the varices in these children to help us providing a better long term care plan for such an important problem. The Objectives are to find out the recurrence of esophageal varices, evolution of gastric varices, portal hypertensive gastropathy [PHG] and risk of rebleeding following esophageal variceal eradication in children with Extra hepatic Portal Vein Obstruction [EHPVO]. Between March 2005 and March 2010, children with extra hepatic portal venous obstruction [EHPVO] and bleeding from esophageal varices who referred to the Gastroentrology unit at Gafaar In Oaf Specialized Children Hospital, Khartoum, Sudan, and the Endoscopic unit of the Military hospital, Omderman, Sudan received endoscopic injection sclerotherapy [EIS] and /or Esophageal Varices Band Ligation [EVBL] until eradication. Surveillance endoscopy was performed initially at 3 months and subsequently at intervals of 6 months to one year to detect esophageal and gastric varices, and PHG. Gastric varices were classified as gastroesophageal [GOV] or isolated gastric varices [IGV]. Gastroesophageal varices included types GOV1 and GOV2 that extend along lesser and greater curvatures respectively. Patients who had recurrence of bleeding were evaluated by emergency upper gastrointestinal endoscopy. The therapeutic endoscopy procedures were done by the two authors. 113 of 133 children who achieved esophageal varices eradication were evaluated. Esophageal varices recurred in 40% cases. Primary gastric varices [before EIS/EVBL] were seen in 61% cases [GOV98% [83% GOV1, 15%GOV2] and IGV2%] and secondary gastric varices [after EIS/EVBL] in 28% [GOV 71% [47% GOV1, 24% GOV2] and IGV 29%]. Secondary gastric varices were distributes as 20% GOV1, 42% GOV2 and 87% IGV. Frequency of gastric varices before sclerotherapy or/and banding, at the last follow up showed decrease in GOV1 from 57to 39 [P=0.01], increase in GOV2 from 10 to 16 and increase in IGV from 1 to 10 [P<0.001]. PHG increased in frequency from 12% to 41% [P<0.001] and severity from one patient to 12 [P<0.001]. Eight cases had rebleeding from gastric varices [4 GOV1, 3 GOV2 and 1 IGV]. It was conclude that following esophageal variceal eradication in children with EHPVO a significant decrease in Gastroesophageal Varices, increase in Isolated Gastric Varices and increased frequency and severity of Portal hypertensive Gastropathy takes place. Small rebleeding risk persists from gastric varices irrespective of the type

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. 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 paraffin-embedded blocks, stained by routine H and 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. The male to female ratio was 1.5:1. The age range between 1 month and 15 years old and 42% were 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%]. 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 Carcinam is significantly high in our pediatrics population

Calciphylaxis: a diagnosis to be considered

Calciphylaxis, Calcific uremic artteriolopathy [CUA] is a rare but serious complication of end-stage renal disease [ESRD], though it can occur in non uremic. Its main presentation is skin changes and digital gangrene. We present a case of a 70 years old male who had a previous big toe amputation. He presented with acute heart failure syndrome, with fingers and toes gangrene which was mistakenly diagnosed as cyanosis. Following comprehensive assessment patient was diagnosed as having Calciphylaxis. Histological findings have shown intimal hypertrophy and diffuse calcification of the small arteries which further supported the diagnosis of CUA, calciphylaxis, complicating CRF

Causes of portal hypertension in Sudanese children

The etiological profile of Pediatric Portal hypertension in our hospital, Gafaar Ibn Oaf Specialized Children Hospital a tertiary referral children hospital for the whole of Sudan, showed that the commonest causes were extra hepatic portal venous obstruction [EHPVO] and liver cirrhosis. Cryptogenic liver cirrhosis was the most common cause of cirrhosis