Volume : 7, Issue : 3, March - 2018

Laron dwarfism-a rare cause of severe short stature

Arindam Ray, Soumik Goswami, Rahul Valsaraj, Riyas R. S. , Nilanjan Sengupta

Abstract :

<p>&nbsp;</p> <p class="MsoNormal" style="margin-left:.5in;text-align:justify;text-justify:&#10;inter-ideograph"><span lang="EN-GB" style="font-size:8.5pt;line-height:107%;&#10;font-family:&quot;Arial&quot;,&quot;sans-serif&quot;;color:#404040;background:#D1D1D1">Patients with Growth Hormone Insensitivity have characteristic phenotypic features and severe short&nbsp;stature. The underlying basis is mutations in the growth hormone receptor gene. We present a 12-year-old girl evaluated for short stature (-4.8&nbsp; SDS ) without Turner stigmata . She has sparse hair, depressed nasal idge, mid-facial hypoplasia, high pitched voice. Biochemical analyses revealed normal GH levels with low serum insulin-like growth factor-1(IGF-1). IGF1 generation test revealed growth hormone insensitivity. The massive expense incurred in the diagnosis and treatment with suboptimal therapeutic response necessitates a judicious approach in this regard in a resource limited setup.<o:p></o:p></span></p>