BACKGROUND: @entity1717 ( @entity1717 ) is a @entity74 with respect to genetics and response to therapy. Data on @entity1717 are sporadic in North African population. AIM: To characterize the clinical features and outcome of 12 Tunisian @entity1 with @entity1717 . METHODS: data of @entity1 diagnosed with @entity1717 during the period 1989-2007 were retrospectively analyzed. Diagnosis was considered whenever @entity3190 >=  10  UI/ml was concomitant to @entity4053 < 3mmol/l and/or high insulin to @entity413 ratio > 0.3 and/or positif glucagon test. Transient causes of @entity4053 , @entity2338 were excluded. RESULTS: There were nine @entity1 diagnosed at a median age of 17 months and three newborns. Permanent hyperammoniemia, found in one @entity1 , guided to @entity368 -sensitive hyperinsulinism. Seven @entity1 presented with @entity410 , two with psychomotor delay and one with recurrent malaises. Among 42 assays of plasmatic insulin, when in @entity4053 , 40% only were >=  10 U/ml. Three @entity1 resisted to @entity6335 and underwent subtotal pancreatectomy complicated by @entity6 in two cases and persistent @entity4053 in one @entity1 . Histological examination concluded to @entity1373 of pancreatic cells. @entity6335 was discontinued in four out the eight responders' @entity1 . Four @entity1 died, seven @entity1 developed variable degrees of @entity1032 and five suffered from @entity302 . CONCLUSION: Early onset forms were, as reported in the literature, mostly resistant to medical therapy. The high proportion of @entity16 is related to diagnosis delay or to a late surgery. We focus on the importance of a precocious diagnosis and @entity146 treatment of @entity4053 .

XXXX : review of 12 Tunisian cases.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient', 'infants'] @entity2338 :: ('MESH:D004393', 'Disease') :: ['adrenal and growth hormone deficiency'] @entity410 :: ('MESH:D012640', 'Disease') :: ['seizures'] @entity1032 :: ('MESH:D008607', 'Disease') :: ['mental retardation'] @entity6 :: ('MESH:D003920', 'Disease') :: ['diabetes mellitus'] @entity368 :: ('CHEBI:25017', 'Chemical') :: ['leucine'] @entity146 :: ('MESH:D001523', 'Disease') :: ['aggressive'] @entity6335 :: ('MESH:D003981', 'Chemical') :: ['Diazoxide', 'diazoxide'] @entity1373 :: ('MESH:D006965', 'Disease') :: ['diffuse hyperplasia'] @entity16 :: ('MESH:D009422', 'Disease') :: ['neurological sequelae'] @entity4053 :: ('MESH:D007003', 'Disease') :: ['hypoglycemia'] @entity1717 :: ('MESH:D044903', 'Disease') :: ['CHI', 'Congenital hyperinsulinism in infancy'] @entity3190 :: ('MESH:D006946', 'Disease') :: ['hyperinsulinemia'] @entity74 :: ('MESH:D030342', 'Disease') :: ['heterogeneous disorder'] @entity302 :: ('MESH:D004827', 'Disease') :: ['epilepsy'] @entity413 :: ('MESH:D005947', 'Chemical') :: ['glucose']
@entity1717 :: (MESH:D044903,Disease) :: ['Congenital hyperinsulinism']
