http://hdl.handle.net/10564/1522 1998-02 Fri, 10 Apr 2026 15:38:36 GMT 2026-04-10T15:38:36Z http://hdl.handle.net/10564/426 タイトル: A CASE OF OCCULT ECTOPIC CORTICOTROPIN (ACTH) SYNDROME MAITAINING REMISSION STATE OVER 4 YEARS AFTER BILATERAL ADRENALECTOMY 著者: Okamoto, Shingo; Hokaze, Yohichi; Matsumoto, Makoto; Izumi, Yukiko; Hosokawa, Akiko; Saka, Munehisa; Yoshida, Katsunori; Hirao, Yoshihiko; Kuriyama, Shigeki; Fukui, Hiroshi 抄録: A long-lasting state of remission of over 4 years of occult ectopic cor- ticotropin (ACTH) syndrome in a 68-year-old female is reported. She presented with general malaise, muscle atrophy and weakness of extremities, general petechiae and hypokalemia. Although ectopic ACTH syndrome was suspected due to marked hypercor- tisolism and ACTH elevation, the source of ACTH secretion could not detected by any diagnostic technique. Because of that effective suppression of plasma cortisol levels by torilostane, a 3 β-hydroxy-steroid-dehydrogenase inhibitor, could not obtained, so bilateral adrenalectomy was performed. After the operation, supplemental therapy of hydrocor- tisone and Florinef, 9 α-fluorohydrocortisone, was initiated and the plasma ACTH levels decreased gradually. At the 6th postoperative month, the levels of ACTH remained within the normal range and her general status markedly improved. At the 4th postoperative year, the plasma ACTH still maintained normal levels with no recurrence. Cases of occult ectopic ACTH syndrome which remain in remission for a long period after bilateral adrenalectomy are rare and interesting. Bilateral adrenalectomy may be the first choice of treatment for occult ectopic ACTH syndrome with the support of chemical therapy. Fri, 27 Feb 1998 15:00:00 GMT http://hdl.handle.net/10564/426 1998-02-27T15:00:00Z http://hdl.handle.net/10564/425 タイトル: A CASE OF CUSHING'S SYNDROME ASSOCIATED WITH ISOLATED ADRENOCORTICOTROPIN (ACTH) DEFICIENCY AFTER THE REMOVAL OF CORTISOL-PRODUCING ADRENAL ADENOMA 著者: Okamoto, Shingo; Umemoto, Norie; Nakatani, Yoshihiro; Hosokawa, Akiko; Izumi, Yukiko; Nishiyama, Toshimasa; Sakamoto, Takemi; Kuriyama, Shigeki; Tsumatani, Kenichi; Hirao, Yoshihiko; Fukui, Hiroshi 抄録: A 45-yr-old woman with Cushing's syndrome who could not be withdrawn from postoperative hydrocortisone supplement for over 3 years after the removal of an adrenocortical adenoma is presented. This was diagnosed as associating with isolated ACTH deficiency. She had suffered from persistent severe general fatigure since 5 years prior. However, after 2 years, obesity, hypertension and moon face developed, while the malaise improved. She was then diagnosed as having Cushing's syndrome and underwent the extirpation of right adrenal adenoma. The dosage of supplementary hydrocortisone was gradually reduced after surgery. However the hydrocortisone dose could not be reduced lower than 10mg/day after several trials of dose reduction as severe general fatigue would ensue. The pituitary function tests at postoperative 2 years and a half-year, revealed an association of isolated ACTH deficiency. It was unknown whether the isolated ACTH deficiency arose before or after the surgery. In the present report, we discuss the association of isolated ACTH deficiency and Cushing's. syndrome. Fri, 27 Feb 1998 15:00:00 GMT http://hdl.handle.net/10564/425 1998-02-27T15:00:00Z http://hdl.handle.net/10564/424 タイトル: A CASE OF TYPE II POLYGLANDULAR AUTOIMMUNE DISEASE (SCHMIDT'S SYNDROME) ASSOCIATED WITH A MYOPATHY OF UNKNOWN ETIOLOGY 著者: Okamoto, Shingo; Umemoto, Norie; Kumata, Keiichi; Murata, Kenya; Izumi, Yukiko; Nishiyama, Toshimasa; Sakamoto, Takemi; Kuriyama, Shigeki; Fukui, Hiroshi 抄録: A 44-year-old female with Schmidt's syndrome associated with a myopathy of unknown etiology is reported. The myopathy, characterized by general muscle weakness and extensive atrophy and loss of muscle fibers, developed since the age of 23 y and progressed. The myopathy could not be diagnosed by immuno-histochemistry nor ser- ological examination. Skin pigmentation developed since 40 y. At 44 y, she was diagnosed with Schmidt's syndrome due to the association of autoimmune Addison's disease and Hashimoto's disease. Although the supplemental therapy of hydrocortisone and thyroxin improved adrenal insufficiency and hypothyroidism, the therapy could not improve the muscle weakness of the myopathy. This is the first case of Schmidt's syndrome with a myopathy of unknown etiology like this case. Fri, 27 Feb 1998 15:00:00 GMT http://hdl.handle.net/10564/424 1998-02-27T15:00:00Z http://hdl.handle.net/10564/423 タイトル: A MALE CASE OF KALLMANN'S SYNDROME : FERTILITY INDUCED BY GONADOTROPIN (hCG/hMG) THERAPY 著者: Okamoto, Shingo; Mayumi Mimura, Mayumi; Moch, Tadao; Sakamoto, Takemi; Izumi, Yukiko; Matzui, Yuhji; Hosokawa, Akiko; Kuriyama, Shigeki; Fukui, Hiroshi 抄録: A 24-year-old male patient with Kallmann's syndrome who fathered two children after gonadotropin therapy is reported here. He was diagnosed with Kallmann's syndrome because of hypothalamic hypogonadism associated with anosmia. The gonadotropin therapy was initiated which involved treatment with human chorionic gonadotropin (hCG) and human menopausal gonadotropin (hMG). After 3 years of treatment, his secondary sexual characteristics developed to near the adult level and sperm were detected in his semen. Although pulsatile luteinizing hormone releasing hormone (LH -RH) injection using a mini-pump was tried for 4 months, it did not maintain the plasma testosterone at normal levels. At 29 y the patient was switched to gonadotropin therapy. His sperm count reached 6×10(6/mm3, and his wife became pregnant ; they had a female baby. His second child was born when the patient was 32 y. The sequence of the KAL gene was normal in all 14 exons of the patient's DNA. Here we describe the clinical benefits of gonadotropin therapy and LH-RH therapy. Fri, 27 Feb 1998 15:00:00 GMT http://hdl.handle.net/10564/423 1998-02-27T15:00:00Z