Histiocytosis X: Clinical Observation of 33 Cases with Special Referance to Occurrence of Hypothalamic-Pituitary Dysfunction

Abstract

Clinical pictures of hypothalmic-pituitary dysfunctuion of 33 cases of children with histjocytosis X were analysed. Some of clinical manifestations due to hypothalamic-pituitary lesions were observed in 15 children including one of 7 deceased children. Observed clinical manifestations were: diabetes insipidus in 11 cases, growth retardation in 10 cases, growth acceleration in 2 cases and precocius puberty in 2 cases. The marked female preponderance (male : female=10 : 1) in incidence of diabetes insipidus was observed. Of 11 cases with diabetes insipidus, 8 cases showed growth retardation below 2 standard deviations. The cases with diabetes insipidus showed a larger deviation from standard body height than ones without diabetes insipidus and this difference was statistically significant (P=5%). This result indicates that in histiocytosis X disease process involves rather wide region of hypothalamic-pituitary system.
Based on an analysis of our cases, the following procedures were proposed to prevent diabetes insipidus in histiocytosis X: 1. periodical measurement of body height and ability of urine concentration should be repeated for at least 3 years after onset of histiocytosis X; 2. cases which show decreased ability of urine concentration or female children with bone lesion (s) who have onset of histiocytosis X after one year of age and show unexplained growth retardation should have radiotherapy to the hypothalamic-pituitary region.