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/Title (Paucit ductulaire syndromatique chez l'enfant : propos d'une observation)
/Author (Deschamps Fabienne)
/Creator (Xerox 4112)
/ModDate (D:20180110153632+01'00')
/Subject (La paucit ductulaire syndromatique entre dans le cadre des cholestases intra-hpatiques de l'enfant. Elle a t individualise en 1975 par ALAGILLE. Dans certaines familles, on retrouve cette affection au niveau de plusieurs gnrations successives, ce qui permet d'apporter la notion de transmission autosomique dominante. Elle touche non seulement les canalicules biliaires intra-hpatiques, mais aussi dautres organes cibles. Parmi eux, nous pouvons retenir : un facis dysmorphique, une stnose de l'artre pulmonaire, des anomalies vertbrales avec un retard de croissance, un retard mental, un hypogonadisme, une atteinte oculaire, une atteinte rnale et des signes neurologiques. Le diagnostic est confirm par la biopsie hpatique. Le traitement reste symptomatique avec une supplmentation en vitamines liposolubles, un rgime enrichi en triglycrides chane moyenne et l'adjonction de cholrtique. Lvolution est en principe favorable.)
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2011-01-14T17:33:30+02:00
2018-01-10T15:36:32+01:00
Xerox 4112
Xerox 4112
application/pdf
Paucité ductulaire syndromatique chez l'enfant : à propos d'une observation
Deschamps Fabienne
La paucité ductulaire syndromatique entre dans le cadre des cholestases intra-hépatiques de l'enfant. Elle a été individualisée en 1975 par ALAGILLE. Dans certaines familles, on retrouve cette affection au niveau de plusieurs générations successives, ce qui permet d'apporter la notion de transmission autosomique dominante. Elle touche non seulement les canalicules biliaires intra-hépatiques, mais aussi d‘autres organes cibles. Parmi eux, nous pouvons retenir : un faciès dysmorphique, une sténose de l'artère pulmonaire, des anomalies vertébrales avec un retard de croissance, un retard mental, un hypogonadisme, une atteinte oculaire, une atteinte rénale et des signes neurologiques. Le diagnostic est confirmé par la biopsie hépatique. Le traitement reste symptomatique avec une supplémentation en vitamines liposolubles, un régime enrichi en triglycérides à chaîne moyenne et l'adjonction de cholérétique. L’évolution est en principe favorable.
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