Abstract
Growth without GH has been well described: in utero;with obesity and hyperinsulinism following surgery for craniopharyngeoma or hypothalamic tumors(Frasier;Kenny,1968);temporarily, with hyperprolactinemia in optic nerve hypoplasia(Costin, 1985); in acromegaloidism(Ashcraft, 1983);with obesity, low RIA-IGF-I but normal IGF-bioassay(Geffner, 1986);in a single child before removal of a craniopharyngeoma (Wit, 1988);the “invisible” GH:normal receptor binding and bioactivity but absent detectability by RIA(Bistritzer, 1988).
The patient: a boy with optic nerve hypoplasia has now been followed for 16.5 yrs., growing along the 3rd to 10th percentile of Zürich standards with only little bone age delay. GH-deficiency was proven at 13 mos. and 9 yrs. of age. Insulin and prolactin were normal. Obesity never existed: BMI 21.2;puberty occurred spontaneously at 13 yrs. of age. ACTH-and ADH-insufficiencies appear to develop gradually recently.
Results:(ng/ml) IGF-I-RIA:19(71-431);IGF-I-RRA:36(52-325);IGF-II-RIA: 522(441-1003);IGF-II-RRA:468(407-1227);IGFBP-3:1457(2119-4262). IGF-bioassay:0.41 U/ml(0.90±0.26).
There was no “burst promoting activity”, when the patient's serum was incubated with erythroid progenitor cells(in contrast to Geffner's patient).
Conclusion: this patient has grown for 16 yrs. without GH and IGF-I with low-normal IGF-II. The low IGFBP-3 supports GH-deficiency, while low IGF-I by bioassay excludes enough free IGF. He differs from all similar patients and demonstrates our ignorance of some growth types.
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Petrykowski, W., Blum, W., Ranke, M. et al. 16 YEARS OF NORMAL GROWTH WITHOUT GROWTH HORMONE(GH), WITHOUT IGF-I AND IGFBP-3 AND WITHOUT OBESITY. Pediatr Res 33 (Suppl 5), S62 (1993). https://doi.org/10.1203/00006450-199305001-00355
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DOI: https://doi.org/10.1203/00006450-199305001-00355