镰状细胞特征缺血性坏死1例报告。

Q2 Medicine BMC Hematology Pub Date : 2018-02-22 eCollection Date: 2018-01-01 DOI:10.1186/s12878-018-0098-z
William J Sanders
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引用次数: 7

摘要

背景:镰状细胞特征通常是患者血红蛋白分子组成与正常人略有不同的无症状表现。它类似于一种更严重的疾病,镰状细胞病,患者的血红蛋白发生突变,导致他们的红细胞在某些环境和内部状态下容易改变形状;这导致红细胞粘附在血管壁上,阻塞它们所经过的动脉腔,导致继发于缺血的下游效应。镰状细胞特征通常没有这些缺血效应。病例介绍:在本病例中,一位年轻的非裔美国女性患者以严重的右髋关节疼痛来到诊所。既往病史包括镰状细胞特征和哮喘。她多年来没有哮喘症状,也没有接受治疗。疼痛持续了几个月,服用消炎药也没有改善。髋关节内、外旋转伴有剧烈疼痛。双侧下肢的神经血管是完整的。股骨MRI显示股骨头2期或3期无血管性坏死,而x线检查无明显异常。数周不负重不成功;此后不久,患者接受了右股骨头核心减压术,并开始使用双磷酸盐。病人的病情暂时好转,但此后不久就退化了。在接下来的几个月里,她的血管坏死恶化。在这一点上,唯一的其他选择是做一个全髋关节置换术,但由于人工置换术的寿命,患者可能需要在她的一生中进行多次手术。结论:镰状细胞特征患者发生缺血性坏死的报道很少。这份手稿提出了这样一个案例,包括与它的管理有关的考验和磨难。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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A rare case of avascular necrosis in sickle cell trait: a case report.

Background: Sickle cell trait is usually an asymptomatic presentation of a patient with slightly different hemoglobin molecule makeup than normal. It is similar to a more serious disease, sickle cell disease, in which a person's hemoglobin is mutated in such a way that causes their red blood cells to easily change shape in certain environmental and internal states; this causes red blood cells to adhere to the walls and occlude the lumen of the arteries in which they travel, leading to downstream effects secondary to ischemia. Sickle cell trait does not have these ischemic effects, usually.

Case presentation: In this case, a young African American female patient presents to the clinic with severe right hip pain. Her past medical history includes sickle cell trait and asthma. She has not been symptomatic of her asthma for years and is not on therapy for it. The pain has lasted for several months and has not improved with anti-inflammatory medication. There is severe pain with internal and external rotation of the hip. The neurovascularity of the lower extremities is intact bilaterally. MRI of the femur shows stage 2 or 3 avascular necrosis of the femoral head, while X-rays of the femur are unremarkable. Non weight-bearing for several weeks was unsuccessful; shortly thereafter, the patient underwent core decompression of the right femoral head as well as starting bisphosphonates. The patient improved temporarily but regressed shortly thereafter. Her avascular necrosis worsened radiographically over the next several months. At this point, the only other option would be to do a total hip arthroplasty, but the patient may need several more throughout her lifetime due to the lifespan of the artificial replacement.

Conclusion: There have only been scarce reports of avascular necrosis in patients with sickle cell trait. This manuscript presents such a case and includes the trials and tribulations associated with its management.

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来源期刊
BMC Hematology
BMC Hematology Medicine-Hematology
CiteScore
4.10
自引率
0.00%
发文量
0
期刊介绍: BMC Hematology is an open access, peer-reviewed journal that considers articles on basic, experimental and clinical research related to hematology. The journal welcomes submissions on non-malignant and malignant hematological diseases, hemostasis and thrombosis, hematopoiesis, stem cells and transplantation.
期刊最新文献
Correction to: Rapid and reliable detection of α-globin copy number variations by quantitative real-time PCR Correction to: Patterns of bone marrow aspiration confirmed hematological malignancies in Eritrean National Health Laboratory. Correction to: The impact of helicobacter pylori eradication on platelet counts of adult patients with idiopathic thrombocytopenic purpura. Assessment of knowledge, attitude and practice and associated factors of blood donation among health care workers in Ethiopia: a cross-sectional study. Health-related quality of life of adolescents with sickle cell disease in sub-Saharan Africa: a cross-sectional study.
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