[Selective involvement of the gastrointestinal tract in amyloidosis in a female patient with periodic disease and intact kidneys].
The authors describe a rare case of amyloidosis in a female patient suffering from periodic disease (PD) for 18 years without any clinico-laboratory signs of renal impairment but with marked clinical, (malabsorption, cachexia), endoscopic, x-ray and other manifestations of gastrointestinal amyloidosis. This case is of interest since patients suffering from amyloidosis due to PB develop malabsorption very rarely, namely in 2-3% of cases. As a rule, it develops in patients with pronounced chronic renal failure on hemodialysis or with a history of kidney transplantation. In this particular case, the patient demonstrated selective marked damage to the gastrointestinal tract, with the kidneys remaining practically intact. A possibility of the indicated variety of amyloidosis should be considered in specification of the genesis of persistent diarrhea in PB patients.
Arutiunian
V M
VM
Eganian
G A
GA
Khachatrian
V A
VA
D000293:Adolescent; D000686:Amyloidosis / Q000175:diagnosis* / Q000209:etiology; D002908:Chronic Disease; D003967:Diarrhea / Q000175:diagnosis / Q000209:etiology; D010505:Familial Mediterranean Fever / Q000150:complications / Q000175:diagnosis*; D005260:Female; D005767:Gastrointestinal Diseases / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D007668:Kidney / Q000502:physiology*
Izbiratel'noe porazhenie zheludochno-kishechnogo trakta pri amiloidoze u bol'noĭ periodicheskoĭ bolezn'iu s intaktnymi pochkami.