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镰状细胞贫血患儿的急性胸综合征

Acute chest syndrome in children with sickle cell anemia.

作者信息

Koren A, Wald I, Halevi R, Ben Ami M

机构信息

Pediatric Department B, Central Emek Hospital, Afula, Israel.

出版信息

Pediatr Hematol Oncol. 1990;7(1):99-107. doi: 10.3109/08880019009034323.

DOI:10.3109/08880019009034323
PMID:2397172
Abstract

Vaso-occlusive crisis is the most common cause of morbidity in patients with sickle cell anemia (SCA). Those patients are frequently admitted with chest signs and symptoms suggesting an infective process. The term acute chest syndrome (ACS) is used to describe those chest episodes in SCA patients, and it is postulated that a primary bone vaso-occlusive crisis may be the cause of the acute chest syndrome in SCA patients. In this study we report 52 episodes of ACS in a group of 22 children with SCA. Chest pain, fever, and leukocytosis were a constant clinical finding. The hematological, radiological, and bacteriological studies are reported. There is a constant and significant fall in hemoglobin levels from 88 +/- 10 g/L (8.8 +/- 1.0 g/dl) to 68 +/- 15 g/L (6.8 +/- 5 g/dl). Unilateral or bilateral pulmonary basal infiltrations were found in 50 episodes. Pleural effusion was noticed in 60 episodes, and it was bilateral in three. No significant bacteriological findings were present.

摘要

血管闭塞性危象是镰状细胞贫血(SCA)患者发病的最常见原因。这些患者常因提示感染过程的胸部体征和症状而入院。“急性胸综合征”(ACS)一词用于描述SCA患者的那些胸部发作情况,据推测,原发性骨血管闭塞性危象可能是SCA患者急性胸综合征的病因。在本研究中,我们报告了一组22例SCA患儿中的52次ACS发作情况。胸痛、发热和白细胞增多是持续的临床发现。报告了血液学、放射学和细菌学研究结果。血红蛋白水平持续且显著下降,从88±10 g/L(8.8±1.0 g/dl)降至68±15 g/L(6.8±5 g/dl)。50次发作中发现单侧或双侧肺底部浸润。60次发作中发现胸腔积液,其中3次为双侧性。未发现明显的细菌学结果。

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