An Unusual Case of Rapidly Progressive Hyperbilirubinemia
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 transfusio...
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| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2013-01-01
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| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2013/284029 |
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| _version_ | 1832558500822122496 |
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| author | Kimberly M. Thornton Michael F. Nyp Lejla Music Aplenc Gary L. Jones Shannon L. Carpenter Erin M. Guest Steven M. Shapiro Winston M. Manimtim |
| author_facet | Kimberly M. Thornton Michael F. Nyp Lejla Music Aplenc Gary L. Jones Shannon L. Carpenter Erin M. Guest Steven M. Shapiro Winston M. Manimtim |
| author_sort | Kimberly M. Thornton |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-127c372db9424569a2aea96c847c7320 |
| institution | Kabale University |
| issn | 2090-6803 2090-6811 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-127c372db9424569a2aea96c847c73202025-02-03T01:32:07ZengWileyCase Reports in Pediatrics2090-68032090-68112013-01-01201310.1155/2013/284029284029An Unusual Case of Rapidly Progressive HyperbilirubinemiaKimberly M. Thornton0Michael F. Nyp1Lejla Music Aplenc2Gary L. Jones3Shannon L. Carpenter4Erin M. Guest5Steven M. Shapiro6Winston M. Manimtim7Division of Neonatology-Perinatology, Children’s Mercy Hospitals and Clinics, 2401 Gillham Road, Kansas City, MO 64108, USADivision of Neonatology-Perinatology, Children’s Mercy Hospitals and Clinics, 2401 Gillham Road, Kansas City, MO 64108, USASchool of Medicine, University of Missouri-Kansas City, 2411 Holmes Road, Kansas City, MO 64108, USASchool of Medicine, University of Missouri-Kansas City, 2411 Holmes Road, Kansas City, MO 64108, USASchool of Medicine, University of Missouri-Kansas City, 2411 Holmes Road, Kansas City, MO 64108, USASchool of Medicine, University of Missouri-Kansas City, 2411 Holmes Road, Kansas City, MO 64108, USASchool of Medicine, University of Missouri-Kansas City, 2411 Holmes Road, Kansas City, MO 64108, USADivision of Neonatology-Perinatology, Children’s Mercy Hospitals and Clinics, 2401 Gillham Road, Kansas City, MO 64108, USAWe 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.http://dx.doi.org/10.1155/2013/284029 |
| spellingShingle | Kimberly M. Thornton Michael F. Nyp Lejla Music Aplenc Gary L. Jones Shannon L. Carpenter Erin M. Guest Steven M. Shapiro Winston M. Manimtim An Unusual Case of Rapidly Progressive Hyperbilirubinemia Case Reports in Pediatrics |
| title | An Unusual Case of Rapidly Progressive Hyperbilirubinemia |
| title_full | An Unusual Case of Rapidly Progressive Hyperbilirubinemia |
| title_fullStr | An Unusual Case of Rapidly Progressive Hyperbilirubinemia |
| title_full_unstemmed | An Unusual Case of Rapidly Progressive Hyperbilirubinemia |
| title_short | An Unusual Case of Rapidly Progressive Hyperbilirubinemia |
| title_sort | unusual case of rapidly progressive hyperbilirubinemia |
| url | http://dx.doi.org/10.1155/2013/284029 |
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