Fat embolism syndrome in 3 patients with sickle cell disease (Record no. 76374)

MARC details
000 -LEADER
fixed length control field 02498cam a2200181 4500
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control field NMDX7496
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fixed length control field 120401t2016 xxu||||| |||| 00| 0 eng d
100 ## - MAIN ENTRY--PERSONAL NAME
Personal name Roberts-Harewood, M.
240 ## - UNIFORM TITLE
Uniform title <a href="British Journal of Haematology">British Journal of Haematology</a>
245 ## - TITLE STATEMENT
Title Fat embolism syndrome in 3 patients with sickle cell disease
260 ## - PUBLICATION, DISTRIBUTION, ETC. (IMPRINT)
Date of publication, distribution, etc. 2016
500 ## - GENERAL NOTE
General note NMUH Staff Publications
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General note EMBASE
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General note 173
520 ## - SUMMARY, ETC.
Summary, etc. &lt;span style="font-size: 10pt;"&gt;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.&amp;nbsp;&lt;/span&gt;
700 ## - ADDED ENTRY--PERSONAL NAME
Personal name Yardumian, A.
856 ## - ELECTRONIC LOCATION AND ACCESS
Uniform Resource Identifier <a href="http://onlinelibrary.wiley.com/doi/10.1111/bjh.14019/epdf">http://onlinelibrary.wiley.com/doi/10.1111/bjh.14019/epdf</a>
Holdings
Withdrawn status Lost status Damaged status Not for loan Collection code Home library Current library Shelving location Date acquired Total Checkouts Date last seen Price effective from Koha item type
        Staff publications for NMDX Ferriman information and Library Service (North Middlesex) Ferriman information and Library Service (North Middlesex) Shelves 07/06/2022   07/06/2022 07/06/2022 Book
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