[Uremic and haemolytic syndrome in children: Study of 17 cases]

In spite of its rarity, the haemolytic and uremic syndrome [HUS] constitutes the first aetiology of acute renal insufficiency [ARI] in child. The aim of this work is to analyze clinical and evolutive aspects of the HUS in child. We studied retrospectively 17 cases of HUS in child enrolled in the paediatrics' department of Sahloul Hospital during eight years period [1996 to 2003]. It is about four boys and 13 girls [sex - ratio= 0.3] aged three months to nine years [mean age: 32 months]. Typical HUS was observed in eight child and atypical HUS in the nine others which three presenting a familial form and one associated with steroid resistant nephrotic syndrome. Diagnosis of HUS was established on the classic triad of the disease [anaemia, thrombopenia and ARI] and/on by the histology. Extra-renal manifestations [neurological or digestive involvement] were observed in 11 patients. A blood transfusion was indicated in 13 patients presenting severe anaemia. Peritoneal dialysis was indicated for nine patients while three others required haemodialysis because renal insufficiency had evolved quickly to the end stage. Thirteen cases of HUS [eight typical and five atypical] have received plasma therapy during two to five days. The short-term evolution was favourable with recuperation of normal renal function in seven cases [five with typical SHU and two with atypical SHU]. Three children developed terminal renal insufficiency and were currently in haemodialysis. Five patients [four cases of atypical HUS and one case of typical HUS] died of the continuations of the ARI and/or nosocomial infection. The HUS remains a serious illness because of the risk of complications that can occur to short and long-term. Currently, the specific treatment is only recommended in patients presenting an atypical form of HUS

[Cardiac amyloidosis revealing multiple myeloma]

Amyloidosis is a rare disease characterized by an extracellular accumulation of a protein polysaccharide complex [Amyloid]. Cardiac involvement is considered as a major prognostic factor. We report the case of two women, hospitalized for heart failure. The diagnosis of cardiac amyloidosis was suggested by echocardiography left ventricular concentric hypertrophy and typical amyeloid infiltration with hyperechoic. shiny and granite-like aspect of the interventricular septum. The histological confirmation was obtained by gastric biopsy in the first case and biopsy of the salivary glands in the second revealing an amyloidosis AL. This cardiac amyloidosis was secondary to multiple myeloma: monoclonal Gammopathy with immunoglobulin Lambda in the first and Kappa in the second, and the presence of a plasmocyte infiltration in the sternal puncture. Amyloidosis is a rare pathology, the cardiac involvement is frequent in the type AL and can occur with or without clinical manifestations. Echocardiography should be systematic in patients with confirmed amyloidosis