[Moyamoya disease associated with kidney angiodysplasia in a child]. / Maladie de Moya-Moya associée à une angiodysplasie rénale chez un enfant.

INTRODUCTION: Moyamoya disease is a progressive, chronic occlusive vascular disease of the circle of Willis arteries leading to the development of collateral circulation to compensate the occlusion. CASE REPORT: An 11-year-old girl presented an abrupt loss of consciousness and a right-sided motor deficit. Clinical examination found hypertension with 220/120mmHg arterial blood pressure. Brain magnetic resonance imaging showed a large left hemispheric ischemic lesion. Cerebral arteriography demonstrated an abnormal anastomotic vascular network with fine arteriolar ends in several territories, with a "wisp of smoke" aspect arguing in favor of moyamoya disease. Renal arteriography revealed dysplasia of the upper polar branches of the right kidney. CONCLUSION: Moyamoya disease is a rare cause of stroke in children. Its association with renal angiodysplasia is unusual and may be responsible for malignant hypertension. Cognitive impairment and social dependence have recently been recognized as an important unresolved social issue. Affected children require medical-surgical, social, and psychological care.

[Iliofemoral cutaneous mucormycosis with endopelvic extension in an immunocompetent child]. / Mucormycose cutanée ilio-fémorale avec extension endo-pelvienne chez un enfant immunocompétent.

Mucormycosis is a rare opportunistic fungal infection with clinical polymorphism and is rapidly extensive and destructive. It is caused by fungi of the mucorales group in the environment and generally arises in the context of immunosuppression. Often difficult and late, diagnosis is based on mycological and histological examination. We report the case of a 10-year-old patient admitted for a pruritic erythematous scaly eruption located in the right inguinal area associated with satellite lymphadenopathy and lymphedema of the right lower limb. The histological study of the cutaneous biopsy revealed a granulomatous reaction with filaments. The mycological examination of the collection of the cutaneous lesion showed mucorales filaments and a stump of Absidia corymbifera was isolated. Abdomino-pelvic CT showed muscular extension with vascular and ureteral englobement. The diagnosis of cutaneous mucormycosis was made. Immunological investigations were normal. Treatment included itraconazole for 3months followed by IV amphotericin B for 1month, with favorable clinical and radiological progression. Mucormycosis is an uncommon fungal infection whose cutaneous localization is rare. It occurs exceptionally in immunocompetent patients and is clinically manifested by a vesicular and pustular rash progressing to ulceration. The diagnosis is confirmed by mycological and histological studies. Treatment consists of antifungal therapy associated with surgical excision of necrotic and infected tissue.

Linkage analysis of families with severe childhood autosomal recessive muscular dystrophy in Morocco indicates genetic homogeneity of the disease in north Africa.

It has been previously shown in Tunisian and Algerian families that the locus for SCARMD maps to the proximal part of 13q, and in Algerian families that the disease is associated with deficiency of the 50 kDa dystrophin associated glycoprotein (50DAG). We have tested this linkage in six families from Morocco where this disease is also prevalent. In one family the 50DAG was tested and found to be negative in a muscle biopsy. Our results showed similar linkage in this country, with statistical tests indicating genetic homogeneity between the three Maghreb countries.