| 000 | 02498cam a2200181 4500 | ||
|---|---|---|---|
| 001 | NMDX7496 | ||
| 008 | 120401t2016 xxu||||| |||| 00| 0 eng d | ||
| 100 | _aRoberts-Harewood, M. | ||
| 240 | _aBritish Journal of Haematology | ||
| 245 | _aFat embolism syndrome in 3 patients with sickle cell disease | ||
| 260 | _c2016 | ||
| 500 | _aNMUH Staff Publications | ||
| 500 | _aEMBASE | ||
| 500 | _a173 | ||
| 520 | _a<span style="font-size: 10pt;">Fat embolism secondary to bone marrow necrosis may occur patients with sickle cell disease giving rise to respiratory, neurological and haematological complications - the so-called fat embolism syndrome (FES). FES is more common in non-SS genotypes. We present 3 cases of suspected or confirmed FES in adult patients with HbSC and review the clinical, radiological and histological findings. Case 1: A 53 year old man with HbSC was admitted with malaise, fevers and rigors. The following day he dropped his GCS and was transferred to ITU. MRI brain showed widespread small infarcts. He became pancytopenic. Bone marrow examination showed widespread necrosis. Despite supportive care including top up transfusions he retained a major neurological deficit. Case 2: A 50 year old woman with HbSC was admitted with abdominal and joint pain. That evening, she deteriorated with hypoxia, pyrexia and tachycardia. CT showed no major PE. She was given antibiotics and transferred to ITU where exchange transfusion was initiated. A drop in the platelet count was noted. She suffered a cardiac arrested and died. At post mortem pulmonary fat embolism was found. Case 3: A 56 year old man with HbSC was admitted with body pain and breathing difficulties and deteriorated within hours. Chest x-ray showed bilateral haziness despite antibiotics. He deteriorated further with a fall in GCS and acute kidney injury and was transferred to ITU before being exchange transfused. CT showed cerebral oedema. An MRI performed 3 days later showed numerous widespread small haemorrhagic foci. GCS remained low despite removal of sedation. Conclusion: Fat embolism is associated with major morbidity and mortality in sickle cell disease. The presentation may mimic a thromboembolic complication but is often characterized by a combination of venous and arterial infarction and has a characteristic radiological appearance when involving the brain.&nbsp;</span> | ||
| 700 | _aYardumian, A. | ||
| 856 | _uhttp://onlinelibrary.wiley.com/doi/10.1111/bjh.14019/epdf | ||
| 999 |
_c76374 _d76374 |
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