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多次输血的成年β地中海贫血患者出现关节积液和紫癜——坏血病诊断的临床指征

Joint effusions and purpura in multiply-transfused adult beta-thalassemia- clinical pointers to diagnosis of scurvy.

作者信息

Prakash A, Pandey A K

机构信息

Department of Obstetrics and Gynecology, Lady Hardinge Medical College and Smt. Sucheta, Kriplani Hospital, New Delhi, India.

Department of Medicine, Lady Hardinge Medical College and Smt. Sucheta Kriplani Hospital, New Delhi, India.

出版信息

Kathmandu Univ Med J (KUMJ). 2013 Oct-Dec;11(44):360-2. doi: 10.3126/kumj.v11i4.13485.

DOI:10.3126/kumj.v11i4.13485
PMID:24899338
Abstract

Periodic transfusions and effective chelation have ensured that thalassemics survive in to adulthood but their life is punctuated by peculiar problems in adulthood. Three cases of scurvy are being reported presenting uniquely as purpura, right hip joint effusion and right knee joint effusion with haemorrhage in prepatellar and retropatellar bursae, respectively over an 18 month period (2009-2010). The first two cases did give a history of gum bleed. None had any coagulation disturbance or transfusion-transmitted infections or connective tissue disorder. All the three cases responded dramatically to vitamin C supplementation. It is imperative to keep in mind that recurrent blood transfusions are associated with a state of subclinical vitamin C deficiency and overt scurvy may manifest as cumulative number of transfusions increase, as in adult thalassemics.

摘要

定期输血和有效的螯合疗法已确保地中海贫血患者能活到成年,但他们成年后的生活却被一些特殊问题所困扰。本文报告了3例坏血病病例,在18个月期间(2009 - 2010年),分别独特地表现为紫癜、右髋关节积液以及右膝关节积液并伴有髌前和髌后滑囊出血。前两例患者有牙龈出血史。所有患者均无凝血功能障碍、输血传播感染或结缔组织疾病。所有3例患者补充维生素C后均有显著疗效。必须牢记,反复输血与亚临床维生素C缺乏状态相关,随着输血累积次数增加,如成年地中海贫血患者,可能会出现明显的坏血病症状。

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Pediatr Radiol. 2017 Feb;47(2):214-220. doi: 10.1007/s00247-016-3726-4. Epub 2016 Oct 24.
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[Scurvy. A rare differential diagnosis of rheumatic diseases].[坏血病。一种罕见的风湿性疾病鉴别诊断]
Z Rheumatol. 2016 Mar;75(2):166-72. doi: 10.1007/s00393-015-0030-x.