PMID- 35187382
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220223
IS  - 2472-1972 (Electronic)
IS  - 2472-1972 (Linking)
VI  - 6
IP  - 3
DP  - 2022 Mar 1
TI  - Coexistence of Renin-independent Aldosterone Secretion and Multiple Endocrine 
      Neoplasia Type 1 Within a Family.
PG  - bvac009
LID - 10.1210/jendso/bvac009 [doi]
LID - bvac009
AB  - Primary aldosteronism (PA) is a state of renin-independent aldosterone secretion 
      that can range from subclinical to overt. Some normotensive individuals for whom 
      PA screening is not routinely recommended are reported to fulfill the loading 
      test criterion used for the diagnosis of PA. Multiple endocrine neoplasia type 1 
      (MEN1) is an autosomal dominant disorder characterized by the development of 
      various endocrine tumors. Cases of PA associated with MEN1 have been reported; 
      however, there has been no previous report on renin-independent aldosterone 
      secretion within a family with MEN1. Herein, we present the case of a 
      normotensive family presenting with both MEN1 and renin-independent aldosterone 
      secretion. A 49-year-old man was admitted to our hospital for PA evaluation owing 
      to the plasma aldosterone concentration/plasma renin activity ratio being greater 
      than the screening cut-off value; the patient was normotensive. The patient had a 
      history of left nephrectomy and adrenalectomy for left renal carcinoma and 
      adrenal tumor at the age of 39 years. Subsequently, he was diagnosed with MEN1 
      concurrent with primary hyperparathyroidism, insulinoma, and novel MEN1 gene 
      mutations (c.655-5_655-4insC and c.818delC). The loading tests for PA 
      confirmation, including saline infusion, and furosemide upright and captopril 
      challenge tests, yielded positive findings, confirming a case of 
      renin-independent aldosterone secretion. The patient's mother, brother, and 
      sister were also genetically or clinically diagnosed with MEN1. All of them were 
      also normotensive and confirmed to have renin-independent aldosterone secretion. 
      The coexistence of renin-independent aldosterone secretion and MEN1 within this 
      family suggests a relationship between the 2 entities.
CI  - (c) The Author(s) 2022. Published by Oxford University Press on behalf of the 
      Endocrine Society.
FAU - Obata, Yoshinari
AU  - Obata Y
AUID- ORCID: 0000-0002-3443-3635
AD  - Department of Diabetes and Endocrinology, Osaka Police Hospital, Osaka, Japan.
FAU - Takayama, Kana
AU  - Takayama K
AD  - Department of Diabetes and Endocrinology, Osaka Police Hospital, Osaka, Japan.
FAU - Maruo, Yumiko
AU  - Maruo Y
AD  - Department of Diabetes and Endocrinology, Osaka Police Hospital, Osaka, Japan.
FAU - Yamaguchi, Hiroki
AU  - Yamaguchi H
AD  - Department of Diabetes and Endocrinology, Osaka Police Hospital, Osaka, Japan.
FAU - Fujii, Kohei
AU  - Fujii K
AD  - Department of Metabolic Medicine, Graduate School of Medicine, Osaka University, 
      Osaka, Japan.
FAU - Hata, Sonyun
AU  - Hata S
AD  - Diabetes Center, National Hospital Organization Osaka Medical Center, Osaka, 
      Japan.
FAU - Togawa, Yuri
AU  - Togawa Y
AD  - Department of Internal Medicine, Japan Community Healthcare Organization, Osaka 
      Hospital, Osaka, Japan.
FAU - Sanda, Azusa
AU  - Sanda A
AD  - Department of Internal Medicine, Japan Community Healthcare Organization, Osaka 
      Hospital, Osaka, Japan.
FAU - Kosugi, Motohiro
AU  - Kosugi M
AD  - Department of Diabetes and Endocrinology, Osaka Police Hospital, Osaka, Japan.
FAU - Hazama, Yoji
AU  - Hazama Y
AD  - Department of Diabetes and Endocrinology, Osaka Police Hospital, Osaka, Japan.
FAU - Yasuda, Tetsuyuki
AU  - Yasuda T
AUID- ORCID: 0000-0002-1128-8834
AD  - Department of Diabetes and Endocrinology, Osaka Police Hospital, Osaka, Japan.
LA  - eng
PT  - Case Reports
DEP - 20220129
PL  - United States
TA  - J Endocr Soc
JT  - Journal of the Endocrine Society
JID - 101697997
PMC - PMC8852679
OTO - NOTNLM
OT  - independent aldosterone secretion
OT  - multiple endocrine neoplasia type 1
OT  - renin
EDAT- 2022/02/22 06:00
MHDA- 2022/02/22 06:01
PMCR- 2022/01/29
CRDT- 2022/02/21 06:06
PHST- 2021/07/09 00:00 [received]
PHST- 2022/02/21 06:06 [entrez]
PHST- 2022/02/22 06:00 [pubmed]
PHST- 2022/02/22 06:01 [medline]
PHST- 2022/01/29 00:00 [pmc-release]
AID - bvac009 [pii]
AID - 10.1210/jendso/bvac009 [doi]
PST - epublish
SO  - J Endocr Soc. 2022 Jan 29;6(3):bvac009. doi: 10.1210/jendso/bvac009. eCollection 
      2022 Mar 1.