An Unusual Case of Rapidly Progressive Hyperbilirubinemia
| Autor: | Kimberly M. Thornton, Michael F. Nyp, Lejla Music Aplenc, Gary L. Jones, Shannon L. Carpenter, Erin M. Guest, Steven M. Shapiro, Winston M. Manimtim |
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| Jazyk: | angličtina |
| Rok vydání: | 2013 |
| Předmět: | |
| Zdroj: | Case Reports in Pediatrics, Vol 2013 (2013) |
| Druh dokumentu: | article |
| ISSN: | 2090-6803 2090-6811 |
| DOI: | 10.1155/2013/284029 |
| Popis: | We present an unusual case of hyperbilirubinemia with rapid early progression leading to bilirubin encephalopathy in a term neonate. Despite early recognition and intervention, the total serum bilirubin reached a maximum level of 39 mg/dL at 32 hours of life. Prior to an emergent exchange transfusion, the patient’s diagnostic evaluation was significant for Coombs-negative microangiopathic hemolytic anemia and thrombocytopenia. Further testing revealed a deficiency of ADAMTS13 protein, or von Willebrand factor-cleaving protease, a finding diagnostic of congenital thrombotic thrombocytopenic purpura, or Upshaw-Schulman syndrome. This rare disease is often misdiagnosed, especially in the newborn period. |
| Databáze: | Directory of Open Access Journals |
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