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Nguyễn Phạm Anh Hoa, Trịnh Thị Thủy, Đõ Văn Đô, Bùi Thị Kim Oanh, Bạch Ly Na, Hoàng Thị Xuyến, Hoàng Vân Anh, Bùi Thị Hương Thùy, Nguyễn Thị Mai Thảo, Vũ Thị Hà, Ngô Văn Mạnh(1)

Hội chứng thận hư ở bệnh nhân Wilson: Nhân ba trường hợp được phát hiện tại Bệnh viện Nhi Trung ương

The syndrome of Wilson kedney disorder patients: Report on three cases treated in National Pediatric Hospital

Gan mật Việt Nam

2015

31

49-52

1859-431X

Wilson disease is a copper metabolism disorder, inherited in an autosomal recessive, causes excessive copper accumulation in the liver, brain, kidney, cornea… Therapy for Wilson disease treatment: copper-chelating agents (Dpenicillamine or Trientin), Zinc combined low copper diet. Some Wilson patients developed nephrotic syndrome after a period of treatment with D-Penicillamine, the cause of nephrotic syndrome can be caused by side effects of the drug D- Penicillamine on the kidneys or by the accumulation of copper which injury kidney of wilson's disease. A number of reports about the Wilson patients who developed nephrotic syndrome after a period of treatment with D-Peni-cillamine were treated by stopping 0penicillamine and switched to Trientin or steroids. The authors report three patients with Wilson's disease who developed nephrotic syndrome after a period of treatment with D-Penicillamine treatment.

TTKHCNQG, CVv 339