[Inaugural diabetic acido-ketosis: rare endocrine complication in thalassemia child]

Diabetes is a sparse endocrine complication of f3 thalassemia major. We report the case of a 14year-old boy with beta thalassemia major, who was polytransfused without regular iron-chelation therapy. He was admitted to hospital with acidosic dyspnoea and severe deshydratation without signs of cardiac failure. Urine examination showed glucosuria and acetonuria. On laboratory exams, glycemia was 39 mmol/l with severe metabolic acidosis. The diagnosis was a diabetic acido-ketosis requiring insulin therapy. Diabetes was hardly controlled; serum ferritin level was 4500 mg/l. Serum ferritin level is the main risk factor for diabetes in patients with beta thalassemia. Thus, an adequate iron-chelation therapy can prevent this complication

[Severe acute liver failure. About 32 cases]

The current study was conducted to determinate clinical, biological and outcome characteristics of children hospitalized for severe acute liver failure [SALF] and to identify prognosis indicators in our patients. Patients hospitalized with SALF, between January 1995 and June 2005, were reviewed retrospectively. Thirty two patients, aged from 2 months to 13 years and 7 months were studied, 72 percent for them developed encephalopathy. The main causes of SALF were viral hepatitis in 31 percent of cases, toxic and drug induced liver disease in 28 percent of cases, inherited metabolic disorders in 31 percent of cases, auto-immune hepatitis in one cases and Budd Chiari syndrome in one case. The etiology of SALF remained undetermined in 16 percent of normal, serum bilirubin level above 300 micro mol/l and the grade of encephalopathy. In the absence of liver transplantation, a total of 11 patients died from sepsis or multiple organ failure. The spontaneous prognosis of severe acute liver failure is poor with an overall mortality. Preventive treatment of viral and toxic hepatitis is one of the most effective approaches