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021 Journal of Nara Medical Association >
Please use this identifier to cite or link to this item:
http://hdl.handle.net/10564/439
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| Title: | AN ADULT CASE OF CONGENITAL ADRENAL HYPERPLASIA DUE TO 21-HYDROXYLASE DEFICIENCY ASSOCIATED WITH BILATERAL ADRENAL MYELOLIPOMA : AN 8-YEAR OBSERVATION OF CLINICAL CHARACTERISTICS DURING STEROID REPLACEMENT THERAPY |
| Authors: | Okamoto, Shingo
Izumi, Yukiko
Hosokawa, Akiko
Matsuo, Takeshi
Sugimoto, Yumi
Tanaka, Yasuyo
Ueshima, Masakazu
Ikenaka, Yasuhide
Kuriyama, Shigeki
Samma, Shoji
Kyumon, Masayuki
Okada, Hideto
Hirao, Yoshihiko
Okajima, Eigoro
Fukui, Hiroshi |
| Keywords: | congenital adrenal hypertrophy
21-hydroxylase deficiency
late onset type
myelolipoma |
| Issue Date: | 30-Apr-1998 |
| Publisher: | 奈良医学会 |
| Citation: | 奈良医学雑誌 Vol.49 No.2 p.147-155 |
| Abstract: | We describe the case of a 44-year-old man with congenital adrenal hyper-
plasia (CAH ; 21-hydroxylase deficiency) associated with bilateral adrenal myelolipoma.
While undergoing computed tomogaraphy(CT) for the evaluation of persisting flank pain
in May 1988, he was diagnosed incidentally as having a bilateral adrenal tumor. Histology
of the right extirpated tumor revealed myelolipoma of the adrenal gland, and the other side
of the tumor also was diagnosed as myelolipoma by the image diagnostic characteristics.
The left side tumor was not extirpated. During examination of hyper-reninism, plasma
renin activity (PRA) at 10.5ng/ml/h, he was diagnosed as having congenital adrenal
hyperplasia due to elevation of adrenocorticotropin (ACTH), progesterone, and 17 α-
hydroxyprogesterone (17 α-OHP). Hydrocortisone with dexamethasone supplemented
therapy was started, and the ACTH, progesterone, and 17 α-OHP were normalized, but
plasma PRA persisted at high levels. The PRA in this case did not show the degree of
sodium loss. During 8 years of observation of the left side myelolipoma by CT, the tumor
size did not increase. This case provides an interesting natural history of the clinical
characteristics of CAH that went undiagnosed for a long period and an interesting compli-
cation of bilateral adrenal myelolipoma. |
| URI: | http://hdl.handle.net/10564/439 |
| ISSN: | 04695550
13450069 |
| Appears in Collections: | 021 Journal of Nara Medical Association
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Files in This Item:
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| 147-155p.AN ADULT CASE OF CONGENITAL ADRENAL HYPERPLASIA DUE TO 21-HYDROXYLASE.pdf | | 6703Kb | Adobe PDF | View/Open |
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