Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Orbital bone infarction with subsequent orbital compression syndrome presenting as orbital cellulitis is reported in a child with sickle cell anemia. She deteriorated despite the use of antibiotics and improved after the surgical drainage of the collection. Radiographic findings, absence of sinusitis, hemorrhagic nature of the collection, and negative cultures all were consistent with orbital compression syndrome secondary to a vasoocclusive crisis. This condition needs to be differentiated from the more common orbital cellulitis secondary to sinusitis.
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PMID:Orbital compression syndrome presenting as orbital cellulitis in a child with sickle cell anemia. 2038 12

Review of a case of paraosseous extramedullary hematopoiesis (EMH) affecting the maxillary sinuses and retro-orbital spaces imitating bilateral orbital cellulitis. Maxillofacial EMH causes diagnostic/therapeutic challenges. This case report describes a 4-year-old African American male with sickle cell disease (HbSS) who presented with bilateral orbitofacial swelling. Diagnosis was made with imaging and confirmed with tissue sampling. Partial exchange transfusion was utilized to stop the progression of maxillofacial EMH and to treat the patient's chronic anemia. Follow-up MRI showed regression of orbital and retro-orbital involvement. Early treatment with conservative modalities and close observation may prevent need for more invasive treatments.
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PMID:Maxillofacial extramedullary hematopoiesis in a child with sickle cell presenting as bilateral periorbital cellulitis. 2480 68

Repeated vaso-occlusive crises (VOCs) are the hallmark of sickle cell disease (SCD). These repeated crises can lead to bone infarcts, necrosis, and, over time, degenerative changes in the bone marrow. Orbital complications in SCD patients are infrequent and usually present as orbital cellulitis. We report the appearance of orbital bone infarction intraoperatively in the case of an 18-year-old Saudi male patient who has been diagnosed with SCD and presented with severe headaches and generalized body aches. He was admitted with a case of SCD with acute VOC and started on the hospital sickle cell protocol. During the admission, the patient developed bilateral periorbital swelling and left inferior dystopia secondary to bilateral frontoparietal bone infarction, which was evident on the magnetic resonance imaging.
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PMID:Frontal Bone Infarctions Masquerading as Bilateral Orbital Cellulitis in a Patient with Sickle Cell Disease. 3254 29