Bhuyan U N, Bagga A, Srivastava R N
Department of Pathology, All-India Institute of Medical Sciences, New Delhi.
Nephron. 1994;66(3):302-6. doi: 10.1159/000187827.
We observed 12 boys and 1 girl, 5-15 years old, presenting with acute renal failure (ARF) and severe hypertension. They constituted 4.9% of all patients with ARF. There was no preceding diarrheal or respiratory prodrome. Clinical and laboratory findings were indicative of hemolytic uremic syndrome (HUS) in 4, but obscure in 9 others. Renal biopsies revealed variably severe occlusive thrombotic microangiopathy (TMA) affecting predominantly interlobular arteries in 8 and both arteries and glomeruli in 5 cases. Glomerular crescents and cortical necrosis were not seen. Following supportive therapy, 8 progressed to or died of uremia; 2 showed persistent proteinuria, 1 moderate hypertension and 2 complete recovery. Our observations indicate that renal TMA without a prodromal illness and typical features of HUS may present with ARF, proteinuria and severe hypertension, and is associated with high mortality.
我们观察了12名男孩和1名女孩,年龄在5至15岁之间,他们均表现为急性肾衰竭(ARF)和严重高血压。他们占所有急性肾衰竭患者的4.9%。之前没有腹泻或呼吸道前驱症状。临床和实验室检查结果显示,4例符合溶血尿毒综合征(HUS),但另外9例情况不明。肾活检显示,8例有不同程度的严重闭塞性血栓性微血管病(TMA),主要累及小叶间动脉,5例动脉和肾小球均受累。未见肾小球新月体和皮质坏死。经过支持治疗,8例进展为尿毒症或死于尿毒症;2例持续蛋白尿,1例中度高血压,2例完全康复。我们的观察表明,无前驱疾病且无典型溶血尿毒综合征特征的肾性血栓性微血管病可能表现为急性肾衰竭、蛋白尿和严重高血压,且死亡率很高。