intestinal disorder prion disease PrP systemic amyloidosis https://github.com/monarch-initiative/mondo/issues/6671 https://github.com/monarch-initiative/mondo/issues/6752 https://github.com/monarch-initiative/mondo/issues/6877 https://www.malacards.org/card/prp_systemic_amyloidosis UMLS:C4518776 MONDO:0018339 Prion protein (PrP) systemic amyloidosis, previously known as chronic diarrhea with hereditary sensory and autonomic neuropathy is an extremely rare autosomal dominant disorder reported in three British families, a Japanese and an Italian family (about 16 cases in total). Onset is usually in the fourth decade of life and the course lasts about 20 years. Reported clinical manifestations include diarrhea, nausea, autonomic failure (areflexia, weakness), neurogenic bladder and urinary infections. The disorder is caused by truncation mutations of the prion protein gene PRNP (20p13) leading to deposition of prion protein amyloid. Orphanet:397606 SCTID:733422008 chronic diarrhea with HSAN chronic diarrhea with hereditary sensory and autonomic neuropathy chronic diarrhoea with HSAN prion protein systemic amyloidosis GARD:0021632 chronic diarrhoea with hereditary sensory and autonomic neuropathy MEDGEN:1377371 hereditary neurological disease