[Paroxysmal Nocturnal Hemoglobinuria: A Rare but Treatable Cause of Acute Kidney Injury. Case Report].

Raimundo Contreras, Gonzalo P Méndez, José Mujica, Carlos Padilla, María E Selamé, Alondra Frías
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Abstract

Early recognition of acute kidney injury is essential to prevent progression to chronic kidney disease. We present the case of a 19-year-old man with multiple emergency department visits for fatigue, abdominal pain, and intermittent dark urine. Upon admission, he had pancytopenia with elements suggestive of hemolysis and acute kidney injury. Thrombotic thrombocytopenic purpura was suspected, so he was treated with dexamethasone and plasmapheresis. The complementary study ruled out this suspicion, and plasmapheresis was suspended. The renal histology showed an acute kidney injury consistent with paroxysmal nocturnal hemoglobinuria (PNH), and bone marrow biopsy excluded marrow infiltration. The diagnosis of PNH was confirmed by flow cytometry. He was treated with prednisone, anticoagulation, iron, and folic acid supplementation. He progressed with a favorable clinical and laboratory response.

阵发性夜间血红蛋白尿:一种罕见但可治疗的急性肾损伤原因。病例报告)。
早期识别急性肾损伤是必不可少的,以防止进展为慢性肾脏疾病。我们提出的情况下,19岁的男子多次急诊科就诊疲劳,腹痛,间歇性暗尿。入院时,他有全血细胞减少,伴有溶血和急性肾损伤。怀疑为血栓性血小板减少性紫癜,给予地塞米松和血浆置换治疗。补充研究排除了这种怀疑,血浆置换暂停。肾脏组织学显示急性肾损伤与阵发性夜间血红蛋白尿(PNH)一致,骨髓活检排除骨髓浸润。流式细胞术证实PNH的诊断。给予强的松、抗凝、铁和叶酸补充治疗。他的临床和实验室反应良好。
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