Abstract
Pituitary dwarfism was confirmed by clinical, auxologic and two GH provocative tests in 135 children (M=86, F= 49, M:F=1.8:1). Pituitary Imaging (MRI) was obtained in 36. These children could be classified into Group I - Idiopathic, 103 cases (76.4%), with 76 (74%) nonfamilial (NFGHD) and 27 (26%) familial (FGHD) from 17 families; Group II - Miscellaneous organic causes, 18 (13.3%); Group III - GHR syndrome -Laron Type, in 14 (10.3%), M:F= 8:6, from 5 families, with a mean basal GH 16.26±13.56 ng/ml and mean peak of 52.35±22.84 ng/ml (normal 20.1 ± 1.36 ng%). The mean peak GH in Group I was 1.52 ± 1.36 ng/ml. In FGHD, inheritance was autosomal recessive except in one with dominant transmission, with no difference in consanguinity (21%) in FGHD and NFGHD. The pituitary vertical coronal height on MRI in 10 FGHD (3.01 ± 0.55 mm.) and 20 NFGHD (2.76 ± 0.84) did not differ (p> 0.05) but none of the FGHD had stalk section or ectopic neurohypophysis while as both were seen in 8 and 6 cases respectively in NFGHD and H/o abnormal delivery obtained in 6 of these 20. The mean pituitary height in GHR, 4.6 ± 1.6 mm. was significantly larger (P < 0.001) with normal morphology. Low IGF-I and GH-BP % RSB (courtesy Prof. Laron) varying from 0 to 100% in 6, suggest defect ranging from receptor to postreceptor levels. This study shows the high incidence of familial GHD and GHR in this country with significant differences in MRI.
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Desai, M., Colaco, P., Choksi, C. et al. GROWTH HORMONE DEFICIENCY (GHD) AND GROWTH HORMONE RESISTANCE (GHR) IN INDIAN CHILDREN. Pediatr Res 33 (Suppl 5), S33 (1993). https://doi.org/10.1203/00006450-199305001-00182
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DOI: https://doi.org/10.1203/00006450-199305001-00182