KEGG   DISEASE: Pigmented micronodular adrenocortical disease
Entry
H00260                      Disease                                
Name
Pigmented micronodular adrenocortical disease
  Supergrp
Cushing syndrome [DS:H01431]
Description
Primary pigmented micronodular adrenocortical disease (PPNAD) is a form of ACTH-independent adrenal hyperplasia resulting in endogenous Cushing's syndrome.
Category
Endocrine disease
Brite
Human diseases [BR:br08402]
 Endocrine and metabolic diseases
  Adrenal gland diseases
   H00260  Pigmented micronodular adrenocortical disease
Human diseases in ICD-11 classification [BR:br08403]
 05 Endocrine, nutritional or metabolic diseases
  Endocrine diseases
   Disorders of the adrenal glands or adrenal hormone system
    5A70  Cushing syndrome
     H00260  Pigmented micronodular adrenocortical disease
Network
nt06310 CRH-ACTH-cortisol signaling
Gene
(PPNAD1) PRKAR1A [HSA:5573] [KO:K04739]
(PPNAD2) PDE11A [HSA:50940] [KO:K13298]
(PPNAD3) PDE8B [HSA:8622] [KO:K18437]
Other DBs
ICD-11: 5A70.Y
ICD-10: E27.5
MeSH: C566469 C566472
OMIM: 610489 610475 603390
Reference
  Authors
Powell AC, Stratakis CA, Patronas NJ, Steinberg SM, Batista D, Alexander HR, Pingpank JF, Keil M, Bartlett DL, Libutti SK
  Title
Operative management of Cushing syndrome secondary to micronodular adrenal hyperplasia.
  Journal
Surgery 143:750-8 (2008)
DOI:10.1016/j.surg.2008.03.022
Reference
  Authors
Stratakis CA
  Title
Cushing syndrome caused by adrenocortical tumors and hyperplasias (corticotropin- independent Cushing syndrome).
  Journal
Endocr Dev 13:117-32 (2008)
DOI:10.1159/000134829
Reference
  Authors
Bourdeau I, Stratakis CA
  Title
Cyclic AMP-dependent signaling aberrations in macronodular adrenal disease.
  Journal
Ann N Y Acad Sci 968:240-55 (2002)
DOI:10.1111/j.1749-6632.2002.tb04339.x
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