title: [Laron syndrome: Presentation, treatment and prognosis].
abstract: Laron syndrome is a rare cause of short stature due to an abnormality of growth hormone receptor (GHR). It is characterized by poor phenotype-genotype correlation and geographic predilection essentially in the Mediterranean rim, the Middle East and Indian subcontinent. This syndrome corresponds to an endogenous and exogenous complete insensitivity of GH and manifests by early hypoglycemia, an extremely severe short stature and dysmorphic features contrasting with high levels of circulating GH. To date, treatment with recombinant IGF1 is the only treatment option that has improved the terrible prognosis in these patients but does not actually realize the conditions for genuine replacement therapy.
authors.affiliation: Mohamed First University, Medical school, Mohamed VI hospital, department of endocrinology, Oujda, Maroc. Electronic address: hlatrech@hotmail.fr.
authors.forename: Hanane
authors.initials: H
authors.lastname: Latrech
authors.affiliation: Assistance publique-Hôpitaux de Paris, Necker-Enfants-Malades University Hospital, université Paris Descartes, centre des maladies endocriniennes rares de la croissance, pediatric endocrinology, gynecology and diabetology, 75015 Paris, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Polak
mesh_terms: D002648:Child; D006801:Humans; D046150:Laron Syndrome / Q000175:diagnosis* / Q000628:therapy*; D011379:Prognosis