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- W2969359787 abstract "Abstract Primary macronodular adrenal hyperplasia (PMAH) is an adrenal cause of Cushing's syndrome, the clinical and the pathological features of which were described for the first time by Marvin Kirschner et al. in 1964 [1] . At that time, the authors suggested the following as an explanation for this heterogeneous adrenal disorder: a more tenable hypothesis would be that multinodular hyperplasia with macronodules represents an abnormal outcome of adrenal stimulation, with eventual concomitant, possibly localized, loss of adrenal secretory efficiency. Over the 55 years since PMAH's identification, our knowledge has advanced to clarify the mechanisms involved in the pathophysiology of this disorder. Up to now, diverse molecular events have been proposed to explain the enhanced cortisol secretion, cell proliferation, and development of macronodules that occur in PMAH. Nevertheless, the precise sequence of events and molecular mechanisms underlying in this condition remaining incompletely elucidated. Recent studies indicate that PMAH is more genetically determined than previously thought according to the bilateral nature of the disease. In addition, the description of familial forms, with an autosomal dominant pattern suggested an inherited genetic etiology for PMAH. The purpose of this review is to summarize the primary insights concerning the molecular and cellular regulatory mechanisms of PMAH." @default.
- W2969359787 created "2019-08-29" @default.
- W2969359787 creator A5059864016 @default.
- W2969359787 creator A5061844889 @default.
- W2969359787 date "2019-10-01" @default.
- W2969359787 modified "2023-09-23" @default.
- W2969359787 title "Molecular and cellular regulation of primary macronodular adrenal hyperplasia" @default.
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- W2969359787 doi "https://doi.org/10.1016/j.coemr.2019.08.007" @default.
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