Hand-Schueller-Christian disease with occult diabetes insipidus, cardiac failure and renal dysfunction

Autor: Hiroaki Aoyama, Kazuyasu Endo, Tokihisa Kimura, Masahiro Ohta, Kaoru Yoshinaga, Hirofumi Osaki, Masaru Shoji, Kazutoshi Sato, Keishi Abe, Tadasu Yamamoto, Minoru Inoue, Kozo Ota
Rok vydání: 1990
Předmět:
Zdroj: Japanese Journal of Medicine. 29:405-410
ISSN: 1881-123X
0021-5120
DOI: 10.2169/internalmedicine1962.29.405
Popis: A 60-year-old man was diagnosed as Hand-Schüller-Christian disease due to the triad of exophthalmus, decalcification of the bone, and diabetes insipidus. He had xanthogranuloma on the face and a nuchal region, and unusual complications of ADH-resistant diabetes insipidus due to renal dysfunction, and chronic cardiac failure. Urine osmolality was hypotonic, but urine volume was within the normal limit, despite the presence of central diabetes insipidus. Hypophyseal, adrenal and thyroid function were not remarkable. The skin biopsy showed the infiltration of eosinophilic granuloma cells. Treatment with vincristine was effective to regress the xanthogranuloma. Diabetes insipidus was not treated because of the absence of polyuria and polydipsia.
Databáze: OpenAIRE