ACUTE SPLENIC SEQUESTRATION IN HOMOZYGOUS SICKLE-CELL DISEASE - NATURAL-HISTORY AND MANAGEMENT
ACUTE SPLENIC SEQUESTRATION IN HOMOZYGOUS SICKLE-CELL DISEASE - NATURAL-HISTORY AND MANAGEMENT
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DOI：
10.1016/s0022-3476(85)80125-6
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发表时间：
1985-01-01
影响因子：
5.1
通讯作者：
SERJEANT, GR
中科院分区：
文献类型：
--
作者：
EMOND, AM;COLLIS, R;SERJEANT, GR
在308名出生时诊断为纯合子镰状细胞病的儿童中，89名在10年内经历了132次临床显著的急性脾隔离症（ASS）发作。初次发病年龄3月龄。到6岁。生存曲线分析的时间间隔，直到第一次攻击表明，累积概率为0.225的2年，0.265的3年，0.297的5岁。13起事件是致命的，11起发生在首次发作期间，所有事件都发生在输血之前。49%的第一次袭击幸存者复发，并且后续事件之间的间隔越来越小。呼吸道症状与132例事件中的52例相关，但血培养细菌分离株较少，肺炎球菌疫苗或青霉素预防不能预防ASS。高胎儿血红蛋白水平保护免受ASS的攻击。旨在早期诊断ASS的父母教育计划之后，ASS的发病率从平均4.6/100患者-年增加到11.3/100患者-年，这可能反映了对并发症的认识增加。在同一时期，死亡率从每100起事件中29.4起下降到每100起事件中3.1起。结果的改善可能是由于医疗管理的改善和ASS的早期检测。
Of 308 children with homozygous sickle cell disease diagnosed at birth, 89 experienced 132 clinically significant attacks of acute splenic sequestration (ASS) over a 10-yr period. The age at first attack ranged from 3 mo. to 6 yr. Survival curve analysis of the interval until first attack indicated a cumulative probability of 0.225 by 2 yr, 0.265 by 3 yr, and 0.297 by 5 yr of age. Thirteen events were fatal, 11 during the first attack, and all before tranfusion could be instituted. Recurrences occurred in 49% of survivors of the first attacks, and there were diminishing intervals between subsequent events. Respiratory symptoms were associated with 52 of 132 events, but bacterial isolates on blood culture were less frequent, and ASS was not prevented by pneumococcal vaccine or penicillin prophylaxis. A high fetal Hb level protected against attacks of ASS. A parental education program aimed at early diagnosis of ASS was followed by an increase in the incidence rate for ASS from a mean of 4.6 per 100 patient-yr to 11.3 per 100 patient-yr, probably reflecting increased awareness of the complication. During the same periods, the fatality rate fell from 29.4 per 100 events to 3.1 per 100 events. The improvement in outcome is likely to have resulted from improvement in medical management and earlier detection of ASS.