title: Clinical features and endocrine profile of Laron syndrome in Indian children.
abstract: BACKGROUND
  Patients with growth hormone (GH) insensitivity (also known as Laron syndome) have been reported from the Mediterranean region and Southern Eucador, with few case reports from India. We present here the clinical and endocrine profile of 9 children with Laron syndrome from India.
  
  
  METHODS
  Nine children diagnosed with Laron syndrome based on clinical features of GH deficiency and biochemical profile suggestive of GH resistance were studied over a period of 5 years from January 2008 to January 2013.
  
  
  CONCLUSIONS
  Age of presentation was between 2.5-11.5 years. All children were considerably short on contemporary Indian charts with mean (SD) height Z score -5.2 (1.6). However, they were within ± 2 SD on Laron charts. No child was overweight [mean (SD) BMI Z score 0.92 (1.1)]. All children had characteristic facies of GH deficiency with an added feature of prominent eyes. Three boys had micropenis and 1 had unilateral undescended testis. All children had low IGF-1 (<5 percentile) and IGFP-3 (<0.1 percentile) with high basal and stimulated GH [Basal GH mean (SD) = 13.78 (12.75) ng/ml, 1-h stimulated GH mean (SD) = 46.29 (25.68) ng/ml]. All children showed poor response to IGF generation test.
  
  
  CONCLUSIONS
  Laron syndrome should be suspected in children with clinical features of GH deficiency, high GH levels and low IGF-1/IGFBP-3. These children are in a state of GH resistance and need IGF-1 therapy.
authors.affiliation: Research Scientist, Hirabai Cowasji Jehangir Medical Research Institute, Jehangir Hospital, Pune, Maharashtra, India.
authors.forename: Supriya R
authors.initials: SR
authors.lastname: Phanse-Gupte
authors.affiliation: Consultant Pediatric and Adolescent Endocrinologist, Jehangir Hospital, Pune, Maharashtra, India.
authors.forename: Vaman V
authors.initials: VV
authors.lastname: Khadilkar
authors.affiliation: Deputy Director, Hirabai Cowasji Jehangir Medical Research Institute, Jehangir Hospital, Pune, Maharashtra, India.
authors.forename: Anuradha V
authors.initials: AV
authors.lastname: Khadilkar
keywords: Failure of IGF generation test; Laron syndrome; high basal GH