Loss of CD55 in eculizumab-responsive protein-losing enteropathy
A Kurolap, O Eshach-Adiv, T Hershkovitz… - … England Journal of …, 2017 - Mass Medical Soc
A Kurolap, O Eshach-Adiv, T Hershkovitz, T Paperna, A Mory, D Oz-Levi, Y Zohar, H Mandel…
New England Journal of Medicine, 2017•Mass Medical Soc… We followed a large consanguineous Muslim–Arab family that included six patients who
had protein-losing enteropathy associated with hypercoagulability (Fig. S1A in the
Supplementary Appendix, available with the full text of this letter at NEJM.org). Disease
onset was between 10 months and 3 years of age, and the patients presented with
generalized edema, severe hypoalbuminemia (reaching 0.5 g per deciliter), diarrhea, and
abdominal complications, with repeated thrombotic events occurring in three of them …
had protein-losing enteropathy associated with hypercoagulability (Fig. S1A in the
Supplementary Appendix, available with the full text of this letter at NEJM.org). Disease
onset was between 10 months and 3 years of age, and the patients presented with
generalized edema, severe hypoalbuminemia (reaching 0.5 g per deciliter), diarrhea, and
abdominal complications, with repeated thrombotic events occurring in three of them …
CD55 (complement decay-accelerating factor) inhibits the alternative and classical arms of the complement pathway. Three patients with protein-losing enteropathy and a genetic variant predicted to result in loss of function of CD55 had a response to eculizumab.
The New England Journal Of Medicine以上显示的是最相近的搜索结果。 查看全部搜索结果