Patients with Laron Syndrome Have Osteopenia/Osteoporosis

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An update on Laron syndrome.

Definition and epidemiology Laron syndrome is a hereditary disease where there is a primary resistance to growth hormone because of a polymorphic molecular defect in the growth hormone receptor. It is clinically and in many biochemical aspects undistinguishable from isolated growth hormone deficiency (IGHD), and is characterised by high circulating growth hormone concentrations and low serum IG...

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Growth curves for Laron syndrome.

Growth curves for children with Laron syndrome were constructed on the basis of repeated measurements made throughout infancy, childhood, and puberty in 24 (10 boys, 14 girls) of the 41 patients with this syndrome investigated in our clinic. Growth retardation was already noted at birth, the birth length ranging from 42 to 46 cm in the 12/20 available measurements. The postnatal growth curves d...

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Total and high molecular weight adiponectin are elevated in patients with Laron syndrome despite marked obesity.

OBJECTIVE Patients with Laron syndrome (LS; primary GH insensitivity) caused by molecular defects of the GH receptor gene, are characterized by dwarfism, profound obesity, and hyperlipidemia. The aim of the current study was to evaluate adiponectin levels in LS, as obesity is known to be associated with low adiponectin. DESIGN AND METHODS We studied nine untreated LS adult patients (5 males, ...

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The globe and orbit in Laron syndrome.

BACKGROUND AND PURPOSE Patients with LS have an inborn growth hormone resistance, resulting in failure to generate IGF-1. The purpose of this study was to evaluate the size of the eye and orbit in LS. MATERIALS AND METHODS We retrospectively reviewed the MR imaging of the brain in 9 patients with LS for the following parameters: axial diameter of the globe, interzygomatic distance, perpendicu...

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Facial morphometry of Ecuadorian patients with growth hormone receptor deficiency/Laron syndrome.

Facial morphometry using computerised image analysis was performed on patients with growth hormone receptor deficiency (Laron syndrome) from an inbred population of southern Ecuador. Morphometrics were compared for 49 patients, 70 unaffected relatives, and 14 unrelated persons. Patients with growth hormone receptor deficiency showed significant decreases in measures of vertical facial growth as...

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ژورنال

عنوان ژورنال: Journal of Bone and Mineral Research

سال: 1999

ISSN: 0884-0431

DOI: 10.1359/jbmr.1999.14.1.156