[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.

[Vascular calcification in pseudoxanthoma elasticum in children]. / Calcifications vasculaires dans le pseudoxanthome élastique chez l'enfant.

INTRODUCTION: Vascular calcifications are associated with several diseases that affect vascular connective tissue and skin and cause considerable morbidity and mortality. The prototype of these conditions is pseudoxanthoma elasticum. We report, in this study, 4 pediatric cases of vascular calcifications diagnosed as elastic pseudoxanthoma. OBSERVATIONS: These 4 children were 2-11 years old and presented variable clinical features. Vascular involvement and arterial hypertension was observed in all patients, skin involvement in 2 cases, gastrointestinal involvement in 2 cases, neurological impairment in one case, and cardiac involvement in one case. Demonstration of ABCC6 gene mutations provided diagnostic confirmation in all cases. CONCLUSION: Pseudoxanthoma elasticum is a rare genetic disease, which can lead to many complications. Appropriate knowledge and early diagnosis are essential.

[Not isolated ventricular compaction in an infant]. / Non compaction ventriculaire isolée chez un nourrisson.

UNLABELLED: Not isolated ventricular compaction cardiomyopathy is a rare deasese described both in children than in adults. It due to the interruption of the embryogenic compaction process of the normal myocardium. We report a pediatric observation of noncompaction of the left ventricle. CASE REPORT: This is a 4-month girl who was admitted to an array of heart failure with systolic murmur to FM. Chest radiography showed cardiomegaly. The ECG showed repolarization disorder. The echocardiography objectified dilated left cavities with thickened wall and anechoic areas (sinusoidal), a mitral regurgitation GII and minimal tricuspid regurgitation. CONCLUSION: NCVI is a rare cause of heart failure in infants. The management is based on guidelines for various clinical symptomatology. Its discovery in children should lead to screening of first-degree relative.

[Pseudoxanthoma elasticum: A rare cause of gastrointestinal bleeding in children]. / Pseudo-xanthome élastique : une cause rare d'hémorragies digestives chez l'enfant.

INTRODUCTION: Pseudoxanthoma elasticum (PXE) is a rare autosomal recessive connective tissue disorder, characterized by calcification and progressive fragmentation of elastic fibers. Gastrointestinal lesions are rare in children and may be responsible for serious complications. This article reports two pediatric cases of PXE revealed by gastrointestinal bleeding. CASE REPORTS: An 11-year-old boy and a 12-year-old girl were hospitalized for gastrointestinal bleeding. Digestive endoscopy showed hemorrhagic gastroenteritis bulbitis in the first case and it was normal in the second. Abdominal ultrasound showed diffuse linear calcifications in both cases. The diagnosis of PXE was retained based on the presence of vascular disease in both patients, a skin lesion in the girl, and an ocular lesion in the boy. The genetic study confirmed the diagnosis of PXE identifying two ABCC6 mutations in the composite state in the boy: the c.2263G> A (p.G755R) mutation on exon 18 and the c.3421C> T (pR1141X) mutation on exon 24 and the 4021G> A (R1164Q) mutation in the homozygous state of ABCC6 exon 24 in the girl. CONCLUSION: Digestive manifestations are unusual ; however, pseudoxanthoma elasticum should be considered in all cases of gastrointestinal bleeding for no apparent reason. Early diagnosis allows prevention and measures to control the risk factors and limit the progression of complications.

[Anomalous origin of the left coronary artery from the pulmonary artery: report of 4 cases]. / Anomalie de naissance de la coronaire gauche à partir de l'artère pulmonaire: à propos de quatre observations.

Ahomalous origin of the left coronary artery from the pulmonary artery is a rare but severe congenital cardiopathy that often causes myocardial infraction within the first months of birth, often with congestive heart failure. We report four cases, three boys and one girl. The appearance of the first symptoms is early, usually between the second and the fifth month. The chest X-ray showed cardiomegaly. Echocardiography showed dilated and hypokinesis cardiomyopathy. In all patients the diagnosis was suspected because the standard electrocardiogram showed a constant deep Q wave in leads I and aVL. Angiography with coronarography was realized in only two cases confirming the diagnosis. The left coronary was directly reimplanted into the aorta in one child at 12 months, he has improved throughout six years. Two infants died after four and six years; one child had a spontaneous progressive recovered normal ventricular function because he had an important collateral circulation from right coronary artery. In conclusion, this rare ischemic myocardiopathy is serious because it's life threatening. The indirect signs given by the electrocardiogram and the echocardiography are of a big diagnosis aid. The prognosis depends on the precocity of the chirurgical reimplantation.

[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.

[Psoriasis in infants about five cases]. / Le psoriasis chez le nourrisson à propos de cinq cas.

INTRODUCTION: Psoriasis is a skin disease very common. The diagnosis is clinical with a scaly erythema. Given the increasing frequency of cases of psoriasis of the child, we are interested in studying the role of this disease especially in infants. We report five cases of psoriasis collected in units of pediatric dermatology at Children's Hospital of Rabat. RESULTS: It is about a girl and four boys ranging in age between 41 days and 2 years. There is in three cases a family history of autoimmune disease, in two cases a notion of family atopée. Three cases among the five cases have plaque psoriasis, a case of psoriasis vulgaris, and one case of psoriatic diaper rash. All cases in our series were treated with emollients and topical corticosteroids. The outcome was favorable in five cases. CONCLUSION: Psoriasis is a multifactorial disease complex, multiple mechanisms, not well known, with combination of genetic, immunological and environmental factors. Treatment is in the light with corticosteroids, anti-inflammatory and immunomodulators obtained by genetic engineering techniques raises considerable hope in severe forms.

[Recurrent flaccid paralysis indicative of acute intermittent porphyria in a child]. / Paralysie flasque récidivante révélatrice d'une porphyrie aiguë intermittente chez un enfant.

UNLABELLED: Porphyrias are inherited metabolic diseases characterized by accumulation and increased excretion of porphyrins due to enzyme deficiencies involved in the biosynthesis of heme. Porphyrias are monogenic autosomal and mostly dominant disorders. CASE REPORT: A 10-year-old boy was diagnosed with acute intermittent porphyria on the basis of recurrent flaccid paralysis with red urine. Confirmation was obtained by measurement of urinary porphyrin precursors and chromatography of porphyrins. CONCLUSION: Acute intermittent porphyria is a severe form of hereditary porphyria with severe neurological complications during an acute episode. Specific investigations should be performed early and urgently in the presence of suggestive signs in a child.

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.