FETAL HEMOGLOBIN AND CLINICAL SEVERITY OF HOMOZYGOUS SICKLE-CELL DISEASE IN EARLY-CHILDHOOD
FETAL HEMOGLOBIN AND CLINICAL SEVERITY OF HOMOZYGOUS SICKLE-CELL DISEASE IN EARLY-CHILDHOOD
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DOI：
10.1016/s0022-3476(81)80529-x
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发表时间：
1981-01-01
影响因子：
5.1
通讯作者：
SERJEANT, GR
中科院分区：
文献类型：
--
作者：
STEVENS, MCG;HAYES, RJ;SERJEANT, GR
纯合子镰状细胞病在出生后2年内的临床特征与6个月时胎儿Hb [HbF]水平的关系被查平均HbF水平显着较低的儿童表现为早期可触及的脾肿大，指状细胞炎，急性脾隔离症[ASS]和那些谁死了。在HbF水平较低的患者中，发生趾炎和ASS的风险显著更大。由于早期脾肿大本身可能增加ASS、感染和死亡的风险，因此在早期脾肿大组中进一步分析了HbF与这些特征的关系。低HbF可能对ASS的病因学有直接影响，但对感染或死亡的任何影响可能是通过其与可触及脾脏外观的关系介导的。高HbF对趾炎风险的保护作用被证明与其发病机制的公认理论一致。早期HbF测定可能有助于识别婴儿期严重并发症的高危患者。
The relationship of the clinical features of homozygous sickle cell disease in the first 2 yr of life to the level of fetal Hb [HbF] at age 6 mo. was investigated. Mean HbF levels were significantly lower in children manifesting early palpable splenomegaly, dactylitis, acute splenic sequestration [ASS] and in those who died. The risks of dactylitis and ASS were significantly greater in patients with lower HbF levels. Since early splenomegaly itself may increase the risks of ASS, infection and death, the relationship of HbF to these features was further analyzed within the early splenomegaly group. A low HbF may have a direct effect on the etiology of ASS, but any effect on infection or death is probably mediated via its relationship with the appearance of a palpable spleen. A protective effect of a high HbF on the risk of dactylitis was demonstrated coincident with the accepted theory of its pathogenesis. Early HbF determinations may be of value in identifying patients at high risk of serious complication during infancy.