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左腎發育不全併發高血壓及溶血性尿毒症候羣之一病例報告

Left Renal Hypoplasia with Hypertension and Hemolytic-Uremic Syndorme Report of a Case

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摘要


單側腎發育不全併發溶血性尿毒症候羣在小兒是少見的疾病。本病例爲6歲8個月女孩,臨床上有惡性高血壓及溶血性尿毒症候羣之三個特征:微小血管病變性溶血性貧血,血小板減少症,腎病變,X光檢查發現左腎發育不全。死後經病理檢查證實爲左腎發育不全並發溶血性尿毒症候羣。因此病例之發生與腎發育不全及惡性高血壓有密切之關係。

關鍵字

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並列摘要


A six years and eight months old girl was admitted to the Department of Pediatrics, Municipal Chung-Hsin Hospital of Taipei because of pallor for six months and abdominal pain for one week. Review of history revealed that the patient had had renal disease for two months before the admission. Hypertension, proteinuria and mild microscopic hematuria were noted then. Laboratory examination showed anemia, poikilocytosis, anisocytosis with burr, helmet and fragmented red cells, thrombocytopenia, proteinuria and increased plasma urea nitrogen. The most striking pathological findings in this case were multiple disseminated hyaline thrombi in microvasculatures, bilateral cortical necrosis of the kidneys, left renal hypoplasis, and malignant nephrosclerosis.

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