Factors affecting prepubertal growth in homozygous sickle cell disease.
Factors affecting prepubertal growth in homozygous sickle cell disease.
复制标题
影响纯合镰状细胞病青春期前生长的因素。
DOI：
10.1136/adc.74.6.502
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发表时间：
1996
影响因子：
5.2
通讯作者：
London
中科院分区：
文献类型：
--
作者：
A. Singhal;J. Morris;P. Thomas;G. Dover;D. Higgs;G. Serjeant;Chetwynd Road;London
目的：探讨血液学指标、社会经济地位和发病率在纯合子镰状细胞病（SS）青春期前生长中的作用。实验方法：在牙买加金斯顿西印度群岛大学医院镰状细胞诊所进行的一项队列研究中，连续记录了315名从出生到9岁患有SS疾病的儿童的身高、体重和血液学。结果：3 - 9岁之间的身高增长与7岁时的总血红蛋白呈正相关，男孩而不是女孩。7岁时达到的身高和体重与男孩的血红蛋白和胎儿血红蛋白呈正相关，但与女孩无关。只有血红蛋白和体重之间的相关性显示出显着的性别差异。偏相关分析表明，胎儿血红蛋白的影响占血红蛋白的影响，进一步的分析表明，身高与F网织红细胞计数（胎儿血红蛋白生产的措施）在两种性别，但不与F细胞的比例F网织红细胞（F细胞富集的措施）。生长与平均红细胞体积、网织红细胞比例计数、α地中海贫血、社会经济地位或发病率无显著相关。结论：患有SS疾病的男孩中胎儿血红蛋白浓度高与线性生长相关。据推测，在男孩中，低浓度的胎儿血红蛋白会增加溶血，从而增加红细胞生成的代谢需求，使他们面临更大的生长不良风险。SS疾病男孩和女孩之间的血液学和生长关系的差异决定了未来的生长分析要考虑性别因素。
OBJECTIVE: To investigate the role of haematological indices, socioeconomic status, and morbidity in prepubertal growth in homozygous sickle cell (SS) disease. METHOD: Height, weight, and haematology were serially recorded in a cohort study of 315 children with SS disease from birth to 9 years at the sickle cell clinic of the University Hospital of the West Indies, Kingston, Jamaica. RESULTS: Height increment between 3 and 9 years correlated positively with total haemoglobin at age 7 years in boys but not girls. Attained height and weight at age 7 years correlated positively with haemoglobin and fetal haemoglobin in boys but not girls. Only the correlation between haemoglobin and weight showed a significant gender difference. Partial correlation analysis suggested that the effect of haemoglobin was accounted for by the effect of fetal haemolglobin and further analysis indicated that height correlated with F reticulocyte count (a measure of fetal haemoglobin production) in both sexes but not with the ratio of F cells to F reticulocytes (a measure of F cell enrichment). Growth was not significantly related to mean red cell volume, proportional reticulocyte count, alpha thalassaemia, socioeconomic status, or morbidity. CONCLUSION: A high concentration of fetal haemoglobin in boys with SS disease is associated with greater linear growth. It is postulated that in boys, low concentrations of fetal haemoglobin increase haemolysis and hence metabolic requirements for erythropoiesis, putting them at greater risk of poor growth. Differences in the relationship of haematology and growth between boys and girls with SS disease dictate that future analyses of growth take gender into account.