Abstract
The extent of neuroendocrine impairment in 6 cases of disseminated histiocytosis X {HX} & response to hGH therapy in 3 cases was evaluated. Four males & 2 females with biopsy-proven bone & soft tissue involvement, X̄ age at onset was 2.3 yrs. Each required chemotherapy & prednisone, 4 skull irradiation. All exhibited neurogenic diabetes insipidus {DI}. LH & FSH levels averaged 2.9 & 3.4 mIU/ml prepuberally & rose at puberty in 1 patient. ACTH & cortisol did not respond in 2 cases. Stature was mildly to severely impaired, averaging 2.33 SD below the X̄. Basal X̄ GH level was 1.52ng/ml. GH deficiency was established with hypoglycemic & arginine stimulation. These cases were treated with hGH 0.08U/kg 3× weekly. Growth rates averaged 3.77cm/yr prior to hGH therapy & 5.41cm/yr with hGH. Growth rates in the 3 cases without GH deficiency averaged 4.42cm/yr. T4 levels were normal in all cases {X̄ 7.5ug/dl}. TSH & PRL secretion were stimulated with 100ug TRH {IV}. TSH rose from basal X̄ of 4.2 {R3-6}mIU/ml to a peak X̄ of 21 {R9-42}mIU/ml at 30 min. PRL rose from a basal X̄ of 8.7 {R4-20 ng/ml to a peak X̄ of 49 {R28-84}ng/ml at 30 min. This data suggests that HX has a selective effect on hypothalamicpituitary function. TSH & prolactin responses to TRH were normal in all cases. This does not support the hypothesis that HX extensively infiltrates the hypothalamus. Response to hGH, while modest, exceeded growth rates of HX cases without GH deficiency.
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Brown, D., Bonner, R., Aceto, T. et al. Histiocytosis X: Neuroendocrine Testing & Growth Hormone Response. Pediatr Res 15, 1561 (1981). https://doi.org/10.1203/00006450-198112000-00156
Issue date:
DOI: https://doi.org/10.1203/00006450-198112000-00156
