Friday, January 5, 2018

Short stature with Intrauterine and postnatal growth restriction

OurD/d:
1. Silver Russel synd:short stature triangular face, microcephaly, unable to gain wt and ht( failure to thrive), asymmetric limb development, clinodactyly

2. Cornelia-de lange: short stature, hypertrichosis, long curved eye lashes, synorphys, aversion to eating, heart disease.

3. Primordial dwarfism
TURNER SYNDROME
+In turner untreated. Cases.....ht is 20cm less than normal(8inch)
AVG ht for turner in India is 4f6in....in west-4f8in

Growth hormone treatment started early at when ht falls less than 5th centile..initial dose 0.5mg/kg/day..
Short stature usually become evident  by 3 yr..so best result obtained if Gh Started less than 4-5 yes
Oxandrolone with GH  give better results in ht gain.....when given late in age
95.% turner have puberty delay

Estrogen replecemnt:. Ethynyl estradiol (lynoral) now not available
Progynova(estriol derivatives)....1mg /day

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