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青铜色婴儿综合征:铜卟啉组织浓度升高的证据。

The bronze baby syndrome: evidence of increased tissue concentration of copper porphyrins.

作者信息

Rubaltelli F F, Da Riol R, D'Amore E S, Jori G

机构信息

Department of Paediatrics, University of Padova School of Medicine, Italy.

出版信息

Acta Paediatr. 1996 Mar;85(3):381-4. doi: 10.1111/j.1651-2227.1996.tb14040.x.

Abstract

A case regarding a newborn infant with severe Rh haemolytic disease, who presented with the bronze baby syndrome and eventually died, is reported. The postmortem examination showed marked extramedullary haematopoiesis in the liver and spleen, heavy hepatic haemosiderosis and mild intralobular cholestasis. The porphyrin content, which was assayed in different tissues, was very high in the liver, suggesting that the increased erythropoiesis seen in Rh haemolytic disease leads to an increased synthesis of porphyrins as by-products of haem synthesis. Phototherapy causes photodestruction, sensitized by bilirubin, of porphyrins (mainly copper porphyrins), yielding brown photoproducts.

摘要

本文报告了一例患有严重Rh溶血病的新生儿病例,该患儿出现青铜色婴儿综合征并最终死亡。尸检显示肝脏和脾脏有明显的髓外造血,肝脏有严重的含铁血黄素沉着症和轻度小叶内胆汁淤积。在不同组织中检测的卟啉含量在肝脏中非常高,这表明Rh溶血病中所见的红细胞生成增加导致作为血红素合成副产物的卟啉合成增加。光疗会导致胆红素致敏的卟啉(主要是铜卟啉)发生光破坏,产生棕色光产物。

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