An endocrinopathy characterized by dysfunction of the pituitary-adrenal axis and alopecia universalis: supporting the entity of a triple H syndrome.
We demonstrate the rare disorder of triple H syndrome in a 25-year-old man. He was pointed out as having short stature, at -5.9 s.d., and diagnosed as GH deficient at 6 years old. Approximately a year ago, he noticed systematic hair loss. He lost body weight by 7 kg during the last half year. He was admitted to Jichi Medical School Hospital because of unconsciousness. Physical findings showed disturbance of consciousness with Japan Coma Scale I-3. He had emaciation and alopecia universalis. Laboratory findings showed plasma glucose was as low as 1.11 mmol/l. GH and ACTH deficiency with hypoadrenocorticism were clarified. His intelligence was in the low normal range with a WAIS IQ of 70, and anterograde amnesia was suggested in the presence of a little, but not significant, morphological change in the hippocampus on a magnetic resonance imaging scan. Replacement by a physiological dose of hydrocortisone normalized plasma glucose, and restored body weight and growth of hair during the 7 month therapeutic period. The present finding strongly supports a clinical entity of triple H syndrome, including ACTH deficiency, alopecia universalis and anterograde amnesia, and that there may be some variation of the triad among the subjects.
Ichiki
K
K
Division of Endocrinology and Metabolism, Department of Medicine, Jichi Medical School, Tochigi 329-0498, Japan.
Nakamura
T
T
Fujita
N
N
Honda
K
K
Hiraga
T
T
Ishibashi
S
S
Ishikawa
S
S
D000311:Adrenal Glands / Q000503:physiopathology*; D000309:Adrenal Insufficiency / Q000150:complications; D000324:Adrenocorticotropic Hormone / Q000172:deficiency; D000328:Adult; D000505:Alopecia / Q000150:complications*; D000647:Amnesia / Q000150:complications; D001786:Blood Glucose / Q000032:analysis; D004614:Emaciation; D004700:Endocrine System Diseases / Q000175:diagnosis* / Q000503:physiopathology*; D006624:Hippocampus / Q000473:pathology; D019382:Human Growth Hormone / Q000172:deficiency; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D007360:Intelligence; D008279:Magnetic Resonance Imaging; D008297:Male; D010902:Pituitary Gland / Q000503:physiopathology*; D013577:Syndrome; D015431:Weight Loss
D001786:Blood Glucose; D019382:Human Growth Hormone; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone