Fatal Postpartum Hemorrhage in a Patient with Niemann-Pick Disease Type B
Background. Niemann-Pick Disease Type B (NPD B) is a rare lysosomal storage disorder resulting from an inherited deficiency of acid sphingomyelinase activity. Here, we report the case of a splenectomized patient with NPD B who died because of severe postpartum hemorrhage (PPH). Case Presentation. A...
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| Format: | Article |
| Language: | English |
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Wiley
2018-01-01
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| Series: | Case Reports in Obstetrics and Gynecology |
| Online Access: | http://dx.doi.org/10.1155/2018/9719374 |
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| author | Atakan Tanacan Abdullah Yalcin Canan Unal Seda Banu Akinci Mehmet Sinan Beksac |
| author_facet | Atakan Tanacan Abdullah Yalcin Canan Unal Seda Banu Akinci Mehmet Sinan Beksac |
| author_sort | Atakan Tanacan |
| collection | DOAJ |
| description | Background. Niemann-Pick Disease Type B (NPD B) is a rare lysosomal storage disorder resulting from an inherited deficiency of acid sphingomyelinase activity. Here, we report the case of a splenectomized patient with NPD B who died because of severe postpartum hemorrhage (PPH). Case Presentation. A 23-year-old nulliparous woman was admitted to intensive care unit (ICU) after cardiopulmonary arrest during urgent hysterectomy because of severe postpartum bleeding. The patient concealed her disease from her family and obstetricians during her pregnancy, and her NPD B diagnosis was revealed during her stay in ICU while searching for the cause of the splenectomy and severe bleeding. Unfortunately, she had a detrimental course with hypoxic brain injury leading to brain death. Conclusions. In conclusion, physicians should keep in mind that patients with a history of splenectomy and/or uncontrollable hemorrhage must be carefully evaluated for rare diseases like lysosomal storage diseases and that NPD B can cause mortality because of postpartum bleeding. Adult intensivists should be familiar with adult presentations of rare metabolic or genetic diseases as more and more children with metabolic or genetic diseases will survive to adulthood and will be admitted to and unfortunately will even die in the adult ICU. |
| format | Article |
| id | doaj-art-24331386195e479fa5d234e48cc22acf |
| institution | Kabale University |
| issn | 2090-6684 2090-6692 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Obstetrics and Gynecology |
| spelling | doaj-art-24331386195e479fa5d234e48cc22acf2025-02-03T01:12:18ZengWileyCase Reports in Obstetrics and Gynecology2090-66842090-66922018-01-01201810.1155/2018/97193749719374Fatal Postpartum Hemorrhage in a Patient with Niemann-Pick Disease Type BAtakan Tanacan0Abdullah Yalcin1Canan Unal2Seda Banu Akinci3Mehmet Sinan Beksac4Division of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University Medical Faculty, Ankara, TurkeyDepartment of Anaesthesiology and Critical Care Medicine, Hacettepe University Medical Faculty, Ankara, TurkeyDivision of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University Medical Faculty, Ankara, TurkeyDepartment of Anaesthesiology and Critical Care Medicine, Hacettepe University Medical Faculty, Ankara, TurkeyDivision of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University Medical Faculty, Ankara, TurkeyBackground. Niemann-Pick Disease Type B (NPD B) is a rare lysosomal storage disorder resulting from an inherited deficiency of acid sphingomyelinase activity. Here, we report the case of a splenectomized patient with NPD B who died because of severe postpartum hemorrhage (PPH). Case Presentation. A 23-year-old nulliparous woman was admitted to intensive care unit (ICU) after cardiopulmonary arrest during urgent hysterectomy because of severe postpartum bleeding. The patient concealed her disease from her family and obstetricians during her pregnancy, and her NPD B diagnosis was revealed during her stay in ICU while searching for the cause of the splenectomy and severe bleeding. Unfortunately, she had a detrimental course with hypoxic brain injury leading to brain death. Conclusions. In conclusion, physicians should keep in mind that patients with a history of splenectomy and/or uncontrollable hemorrhage must be carefully evaluated for rare diseases like lysosomal storage diseases and that NPD B can cause mortality because of postpartum bleeding. Adult intensivists should be familiar with adult presentations of rare metabolic or genetic diseases as more and more children with metabolic or genetic diseases will survive to adulthood and will be admitted to and unfortunately will even die in the adult ICU.http://dx.doi.org/10.1155/2018/9719374 |
| spellingShingle | Atakan Tanacan Abdullah Yalcin Canan Unal Seda Banu Akinci Mehmet Sinan Beksac Fatal Postpartum Hemorrhage in a Patient with Niemann-Pick Disease Type B Case Reports in Obstetrics and Gynecology |
| title | Fatal Postpartum Hemorrhage in a Patient with Niemann-Pick Disease Type B |
| title_full | Fatal Postpartum Hemorrhage in a Patient with Niemann-Pick Disease Type B |
| title_fullStr | Fatal Postpartum Hemorrhage in a Patient with Niemann-Pick Disease Type B |
| title_full_unstemmed | Fatal Postpartum Hemorrhage in a Patient with Niemann-Pick Disease Type B |
| title_short | Fatal Postpartum Hemorrhage in a Patient with Niemann-Pick Disease Type B |
| title_sort | fatal postpartum hemorrhage in a patient with niemann pick disease type b |
| url | http://dx.doi.org/10.1155/2018/9719374 |
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