[Wolcott-Rallisson syndrome: diabetes mellitus and epiphyseal dysplasia]

Wolcott-Rallison syndrome [SWR] is a rare autosomal recessive disorder. This syndrome is characterized by early infancy insulin-dependent diabetes associated with spondylo-epiphyseal dysplasia, severe growth retardation and other frequent multisystemic manifestations: facial dysmorphia, hepatic and renal dysfunction, mental retardation, cardiovascular abnormalities and neutropenia. Death occurs early in the first ten years of life. The syndrome results from mutations in the gene encoding the eukaryotic translation initiation factor 2-a kinase 3 [EIF2AK3]. We report a family case of WRS in a Moroccan girl who was born from healthy consanguineous parents. Diabetes began at the age of 2 months. Later, she developed spondylo-epiphyseal dysplasia, hepatic dysfunction and she died at 3 year-old of a renal failure. A same case was identified in the siblings with similar characteristics. Through this case, we have to pay attention to the existence of neonatal diabetes which etiopathogeny is different from the child classical auto-immune type I diabetes

Acute renal failure in children: a 111 cases report

Acute renal failure [ARE] is not exceptional in child. The aim of this study was to analyze the epidemiology, etiologies and the prognosis of ARE in children in Casablanca. We report 111 children with ARE, treated from 1991 to 2001 in the department of nephrology and pediatric unit of Ibn Rochd Universitary Hospital of Casablanca. The children were separated into 3 groups: newborn [6 cases], infants [28 cases] and child [77 cases]. Oligoanuria was observed in 71 cases [63%]. Causes of ARE were: functional ARF in 16 cases [14.4%], organic ARF in 86 cases [77.4%] due to glomerulonephritis in 39 cases [45.3%], hemolytic-uremic syndrome in 21 cases and acute tubular necrosis in 19 cases [18.2%]. Obstructive ARF was observed in 9 cases. The treatment depended on the cause of ARF. Renal replacement therapy was necessary in 37 cases [33%]. Eighty three children [90.2%] recovered a normal renal function, 9 children [9.7%] involved to chronic renal failure. Death occurred in 14 cases [12.6%]. We conclude that ARE prognosis depends on the cause and the early treatment

rare cause of children's vascular cerebral ischemic strije: sickle cell disease

Ischemic cerebral infarctions are uncommun in childhood. Aetiologies are numerous. The etiologic diagnosis is difficult and this case further proves it. Amina, 9 years old, is admitted in Pediatrics 2 of Children's Hospital in Casablanca, for polyarthralgia evolving for one year, with right hemiplegia. Right hemiconvulsions had preceded the neurologic motor deficency. Cerebral scanner shows ischemic lesions confirming the ischemic stroke. Etiologic screening allowed elimination of systemic diseases, high blood pressure, congenital heart diseases and rythm disorders. Haemoglobin electrophoresis confirmed the homozygote sickle cell disease showing a high 84% rate of S haemoglobinemia. Split in four groups: cardiac, vascular, hematologic and metabolic diseases. But a large number stays idiopathic. The ischemic stroke is confirmed at once by cerebral scanner. Then in case of no evident aetiology, the first step involves monitoring of blood pressure and cardiac check-up, followed by hemoglobin electrophoresis. Appropriate questionning and well examination after stroke in children are very important. In our case, semiologic features of pain which were acute, none inflammatory and none mechanical, along with mongoloid facies, were suffisant to lead to the real diagnosis. The particularity of our patient is that she was not known for suffering from sickle cell disease, and it was the stroke that revealed it. It's difficult to suggest a plan for diagnosis evaluation for children who undergo an acute cerebral ischemic stroke, but a thorough questionning and examination can be helpful to make the etiologic diagnosis. However a first strategie is proposed