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)

Chronic leg ulcers in sickle cell anemia occur mainly around the ankles. The prevalence is up to 75% in adults. These ulcers are painful and debilitating, yet medical treatment is unsuccessful. Surgical treatment consists of repeated skin grafting procedures, which eventually deplete the patient's available donor sites. Thus, in vitro generation of human epidermal autograft for potential treatment of leg ulcers in sickle cell anemia was initiated. However, because epidermal cells need a supporting matrix in order to be applied to the ulcer, the authors decided to assess the independent effect of a collagen matrix alone in promoting healing of sickle cell leg ulcers. Collistat (Hellistex, Inc) dressing was applied directly to the ulcers. The ulcers were reviewed every 2 weeks, and the dressing reapplied every 4 weeks. Collagen matrix caused the chronic ulcers to heal completely in fewer than 3 months. This encouraging performance of collagen alone should be greatly enhanced by the in vitro epidermal autograft.
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PMID:Rapid healing of sickle cell leg ulcers treated with collagen dressing. 276 90

Characterization of the bone phenotype of 24-week-old female transgenic sickle cell disease (SCD), sickle cell trait (SCT) revealed significant reductions in bone mineral density and bone mineral content relative to control with a further significant decreased in SCD compared with SCT. By microcomputed tomography, femur middiaphyseal cortical area was significantly reduced in SCT and SCD. Cortical thickness was significantly decreased in SCD vs control. Diaphysis structural stiffness and strength were significantly reduced in SCT and SCD. Histomorphometry showed a significant increase in osteoclast perimeter in SCD and significantly decreased bone formation in SCD and SCT compared with control with a further significant decrease in SCD compared with SCT. Collagen-I mRNA was significantly decreased in tibiae from SCT and SCD and osterix, Runx2, osteoclacin, and Dmp-1 mRNA were significantly decreased in tibiae of SCD compared with control. Serum osteocalcin was significantly decreased and ferritin was significantly increased in SCD compared with control. Igf1 mRNA and serum IGF1 were significantly decreased in SCD and SCT. IGF1 protein was decreased in bone marrow stromal cells from SCT and SCD cultured in osteogenic media. Crystal violet staining revealed fewer cells and significantly reduced alkaline phosphatase positive mineralized nodules in SCT and SCD that was rescued by IGF1 treatment. We conclude that reduced bone mass in SCD and SCT mice carries architectural consequences that are detrimental to the mechanical integrity of femoral diaphysis. Furthermore reduced IGF1 and osteoblast terminal differentiation contributed to reduced bone formation in SCT and SCD mice.
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PMID:Loss of Bone in Sickle Cell Trait and Sickle Cell Disease Female Mice Is Associated With Reduced IGF-1 in Bone and Serum. 2717 84