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原发性草酸血症合并家族性球形红细胞增多症病例中的血管病(作者译)

[Angiopathy in a case of primary oxalosis combined with familial spherocytosis (author's transl)].

作者信息

Samizadeh A, Beersiek F, Müller K M, Loew H, Losse H

出版信息

Dtsch Med Wochenschr. 1979 Jan 12;104(2):62-5. doi: 10.1055/s-0028-1103845.

DOI:10.1055/s-0028-1103845
PMID:365490
Abstract

An 18-year-old female with primary oxalosis, seen first when she was in advanced renal failure, developed a severe necrotizing angiopathy which began after a rapid decrease in renal function requiring chronic dialysis. Because of the severe angiopathy the preliminary diagnosis of an acute autoimmune vasculitis had been made. The correct diagnosis was revealed by renal biopsy and a renal transplantation performed. Soon after severe oxalosis led to failure of the renal transplant and death. The patient had also had familial spherocytosis, inherited from her father, while the oxalosis had been inherited from her mother. It is suggested that early transplantation at the onset of renal failure, as long as the blood oxalate level is still tolerably low, may give better results than have so far been reported.

摘要

一名18岁原发性草酸血症女性患者,初诊时已处于晚期肾衰竭,在肾功能迅速下降需要长期透析后,出现了严重的坏死性血管病。由于严重的血管病,初步诊断为急性自身免疫性血管炎。肾活检和肾移植明确了正确诊断。严重的草酸血症导致肾移植失败和患者死亡。该患者还患有从父亲遗传而来的家族性球形红细胞增多症,而草酸血症则从母亲遗传而来。建议在肾衰竭初期尽早进行移植,只要血草酸水平仍在可耐受的低水平,可能会比迄今报道的结果更好。

相似文献

1
[Angiopathy in a case of primary oxalosis combined with familial spherocytosis (author's transl)].原发性草酸血症合并家族性球形红细胞增多症病例中的血管病(作者译)
Dtsch Med Wochenschr. 1979 Jan 12;104(2):62-5. doi: 10.1055/s-0028-1103845.
2
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Dtsch Med Wochenschr. 1979 Feb 16;104(7):266.
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Renal homotransplantation in a patient with primary familial oxalosis.一名原发性家族性草酸尿症患者的肾同种移植
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