THE BRONZE BABY SYNDROME - EVIDENCE OF INCREASED TISSUE CONCENTRATIONOF COPPER PORPHYRINS

Citation
Ff. Rubaltelli et al., THE BRONZE BABY SYNDROME - EVIDENCE OF INCREASED TISSUE CONCENTRATIONOF COPPER PORPHYRINS, Acta paediatrica, 85(3), 1996, pp. 381-384
Citations number
11
Categorie Soggetti
Pediatrics
Journal title
ISSN journal
08035253
Volume
85
Issue
3
Year of publication
1996
Pages
381 - 384
Database
ISI
SICI code
0803-5253(1996)85:3<381:TBBS-E>2.0.ZU;2-H
Abstract
A case regarding a newborn infant with severe Rh haemolytic disease, w ho presented with the bronze baby syndrome and eventually died, is rep orted. The postmortem examination showed marked extramedullary haemato poiesis 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 in creased erythropoiesis seen in Rh haemolytic disease leads to an incre ased synthesis of porphyrins as by-products of haem synthesis. Phototh erapy causes photodestruction, sensitized by bilirubin, of porphyrins (mainly copper porphyrins), yielding brown photoproducts.