Abstract
In 1970, we presented the “A-type” of autosomal recessive IGHD characterized by early and severe growth retardation, typical face with vaulted forehead, a strong metabolic and growth response to exogenous hGH, and a tendency to form high GH antibodies which abolish this effect.
Recent events prompt us to report on these patients again:
1) Philipps et al (abstr.Ped.Res. 1981) carried out restriction endonuclease studies in our original family A, yielding abnormal DNA patterns of the GH genes. In other families with IGHD, they found a normal pattern.
2) One of our female patients of the same family (26 years, 118 cm) gave birth to a healthy girl (GA 40 weeks, 2370 g, 43 cm). GH antibodies were found not only in the mother (12 years after the end of hGH Rx) but also in the newborn. Five months later, no more antibodies were detectable.
3) In another family we succeeded for the first time to treat a 5 year-old boy with hGH over a period of 1.5 years without the appearance of HG antibodies so far. This is in contrast to his 10 year-old brother previously treated with a different hGH preparation who developped high titre, growth-inhibiting GH antibodies. It appears that certain hGH preparations are tolerated by these patients who presumably suffer from a prenatal GH deficiency which may cause a lack of immunotolerance for hGH.
Supported by the Swiss National Science Foundation Grant No. 3.901.077
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Illig, R., Zachmann, M. & Prader, A. “A-type” isolated growth hormone deficiency (IGHD). Pediatr Res 15, 1540 (1981). https://doi.org/10.1203/00006450-198112000-00033
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DOI: https://doi.org/10.1203/00006450-198112000-00033