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Primary pigmented nodular adrenocortical disease
3 OMIM references -
3 associated genes
40 connected diseases
15 signs/symptoms
Disease Type of connection
Acrodysostosis
Acrodysostosis with multiple hormone resistance
Autosomal dominant striatal neurodegeneration
Carney complex
Familial atrial myxoma
Retinitis pigmentosa
Williams syndrome
Periventricular nodular heterotopia
Autosomal dominant nonsyndromic sensorineural deafness type DFNA
Autosomal recessive nonsyndromic sensorineural deafness type DFNB
Usher syndrome type 1
Giant cell glioblastoma
Gliosarcoma
Autosomal dominant nonsyndromic intellectual deficit
Acute myeloblastic leukemia with maturation
Acute myeloblastic leukemia without maturation
Acute myelomonocytic leukemia
Acute promyelocytic leukemia
Anophthalmia / microphthalmia - esophageal atresia
Classical homocystinuria
Colobomatous microphthalmia
Hereditary sensory and autonomic neuropathy type 2
Isolated anophthalmia - microphthalmia
Leber congenital amaurosis
Pigmented paravenous retinochoroidal atrophy
Pseudohypoaldosteronism type 2C
Pseudohypoaldosteronism type 2E
Septo-optic dysplasia
Chorioretinopathy, Birdshot type
Dedifferentiated liposarcoma
Familial melanoma
Well-differentiated liposarcoma
Blackfan-Diamond anemia
Early infantile epileptic encephalopathy
Familial isolated hyperparathyroidism
Familial parathyroid adenoma
Intellectual deficit, X-linked, Turner type
Multiple endocrine neoplasia type 1
Posterior polar cataract
Zollinger-Ellison syndrome
Synonym(s):
- PPNAD
Classification (Orphanet):
- Rare endocrine disease
- Rare genetic disease
- Rare infertility
Classification (ICD10):
- Endocrine, nutritional and metabolic diseases -
Epidemiological data:
Class of prevalence: <1 / 1 000 000
Average age onset: variable
Average age of death: any age
Type of inheritance: autosomal dominant
External references:
3 OMIM references -
No MeSH references
Gene symbol UniProt reference OMIM reference
PDE11A Q9HCR9604961
PDE8B O95263603390
PRKAR1A P10644188830
Very frequent
- Adrenal glands anomalies
- Cortico-adrenal hyperplasia / hypersecretion

Frequent
- Asthenia / fatigue / weakness
- Chronic arterial hypertension
- Diabetes mellitus
- Late puberty / hypogonadism / hypogenitalism
- Muscle hypotrophy / atrophy / dystrophy / agenesis / amyotrophy
- Muscle weakness / flaccidity
- Mutiple fractures / bone fragility
- Osteoporosis / osteopenia / demineralisation / osteomalacia / rickets
- Short stature / dwarfism / nanism
- Striae
- Thin skin
- Truncal obesity

Occasional
- Myopathy