[The calciphylaxis syndrome as a complication of chronic kidney failure. 6 case reports and literature review].
Calciphylaxis is a rate complication of unknown pathogenesis in patients with end stage renal disease. It is characterized by calcification of tunica media of small arteries associated with intimal fibrosis and thrombus formation which leads to the development of skin and subcutaneous tissue necrosis. Superinfection of skin lesions is a common consequence of this syndrome which may lead to the sepsis. The prognosis of this condition in serious. We performed a retrospective study of 6 subjects (4 men and 2 women) in the age of 35 to 59 years. We followed the parameters of calcium-phosphate metabolism, presence of calciphylaxis risk factors and the effect of parathyreoidectomy. Five patients were on hemodialysis, one had a kidney transplant. Skin and subcutaneous tissue necrosis were present in all subjects. The serum levels of parathormone were either high, normal or low, levels of calcium were normal or slightly elevated and phosphate levels were high or normal. Calcium was substituted before calciphylaxis development in 5 patients, calcitriole in 3 of therm. Five patients underwent parathyroidectomy. Three patients died (all of sepsis), one patient had the lower into amputation for infected lesions and the remaining two achieved regression. Our findings do support the hypothesis that calcium and calcitriole administration participates in development of calciphylaxis. Fatal prognosis of the once infections skin lesions was also proved.
Válek
M
M
Interní oddĕlení Strahov 1. LF UK a VFN, Praha. m.valek@seznam.cz
Sulková
S
S
Schestauberová
E
E
Svára
F
F
Mann
H
H
Polakovic
V
V
D000328:Adult; D002115:Calciphylaxis / Q000209:etiology*; D005260:Female; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications*; D008297:Male; D008875:Middle Aged