Mannosidosis is an inherited autosomal recessive mucopolysaccharidosis. Patients affected accumulate mannose-rich compounds in various tissues and excrete an increased quantity of oligosaccharides with mannose as a component. A case of type II mannosidosis with end-stage renal failure is reported. The patient, after 6 years of regular hemodialysis treatment, received a kidney transplant. At the time this article was written, the graft was functioning well and thesaurismotic renal deposits had not been observed. The clinical course of mannosidosis was silent and the patient's quality of life was good. Although the risk of recurrence could not be excluded, it seems that renal transplantation can be safely offered to patients affected with mannosidosis type II, in the rare setting of chronic renal failure.
Renal transplantation in a case of mannosidosis.
STRATTA, Piero
1996-01-01
Abstract
Mannosidosis is an inherited autosomal recessive mucopolysaccharidosis. Patients affected accumulate mannose-rich compounds in various tissues and excrete an increased quantity of oligosaccharides with mannose as a component. A case of type II mannosidosis with end-stage renal failure is reported. The patient, after 6 years of regular hemodialysis treatment, received a kidney transplant. At the time this article was written, the graft was functioning well and thesaurismotic renal deposits had not been observed. The clinical course of mannosidosis was silent and the patient's quality of life was good. Although the risk of recurrence could not be excluded, it seems that renal transplantation can be safely offered to patients affected with mannosidosis type II, in the rare setting of chronic renal failure.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
