Laron syndrome: A case report

Silva, K.S.H de; Jayasena, Yaa

doi:10.4038/sljch.v40i4.3848

Cited by 3 publications

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References 6 publications

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“…[ 1 ] Many more cases have been reported from the Mediterranean region, Eucador, and recently from India and Sri Lanka, with a preponderance in consanguineous families. [ 3 4 ] Our observations over a period of 5 years in a tertiary care pediatric endocrine clinic setting have shown that 1 in 100 children tested for GH deficiency is diagnosed with Laron syndrome (unpublished data). We have described the clinical and biochemical profile of Laron syndrome amongst Indian children in a scenario wherein treatment remains unavailable.…”

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confidence: 93%

Clinical features and endocrine profile of Laron syndrome in Indian children

Phanse-Gupte

Khadilkar

2014

Indian J Endocr Metab

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Introduction: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.Material and 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.Results and Discussion: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.Conclusion: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.

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Section: Introductionmentioning

confidence: 93%

Clinical features and endocrine profile of Laron syndrome in Indian children

Phanse-Gupte

Khadilkar

2014

Indian J Endocr Metab

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Growing up different in Neolithic China: A contextualised case study and differential diagnosis of a young adult with skeletal dysplasia

Halcrow

Miller

Snoddy

et al. 2020

International Journal of Paleopathology

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Clinical profile of Laron dwarfism – experience from a tertiary care institute in Chennai

Sethuraman

Venkatasamy

2023

Journal of Pediatric Endocrinology and Metabolism

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Objectives Laron dwarfism is a rare genetic disorder first reported among Israeli jewish children, subsequently about 350 cases cases have been reported worldwide. We aim to describe the clinical profile of nine children with Laron dwarfism from Institute of Child Health, Chennai. Methods Analysis of case records from 2010 to 2018. Results Male:female ratio is 6:3. Mean age of the children at the time of diagnosis was 3 years. All children were extremely short, and mean height Z score (SD) was −7.7(0.8). All children had characteristic facies with no hypoglycaemic episodes. Microcephaly was present in four children out of which two had developmental delay. Three out of six boys had micropenis. All children had low insulin like growth factor-1 (IGF-1) and high basal growth hormone (GH) with a mean (SD) of 39.6 (11.2) ng/mL. Conclusions Suspicion of Laron syndrome should be high when child presents with features of Growth Hormone Deficiency (GHD) with extreme stunting.

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Laron syndrome: A case report

Cited by 3 publications

References 6 publications

Clinical features and endocrine profile of Laron syndrome in Indian children

Clinical features and endocrine profile of Laron syndrome in Indian children

Growing up different in Neolithic China: A contextualised case study and differential diagnosis of a young adult with skeletal dysplasia

Clinical profile of Laron dwarfism – experience from a tertiary care institute in Chennai

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