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婴儿期网织红细胞增多与镰状细胞贫血患者生命最初 3 年中住院次数增加有关。

Increased reticulocytosis during infancy is associated with increased hospitalizations in sickle cell anemia patients during the first three years of life.

机构信息

Molecular Medicine Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, United States of America.

出版信息

PLoS One. 2013 Aug 7;8(8):e70794. doi: 10.1371/journal.pone.0070794. eCollection 2013.

Abstract

OBJECTIVE

Among older children with sickle cell anemia, leukocyte counts, hemoglobin, and reticulocytosis have previously been suggested as disease severity markers. Here we explored whether these blood parameters may be useful to predict early childhood disease severity when tested in early infancy, defined as postnatal ages 60-180 days.

STUDY DESIGN

Data from fifty-nine subjects who were followed at Children's National Medical Center's Sickle Cell Program for at least three years was retrospectively analyzed. Comparisons were made between white blood cell counts, hemoglobin and reticulocyte levels measured at ages 60-180 days and the clinical course of sickle cell anemia during infancy and childhood.

RESULTS

A majority of subjects had demonstrable anemia with increased reticulocytosis. Only increased absolute reticulocyte levels during early infancy were associated with a significant increase in hospitalization during the first three years of life. Higher absolute reticulocyte counts were also associated with a markedly shorter time to first hospitalizations and a four-fold higher cumulative frequency of clinical manifestations over the first three years of life. No significant increase in white blood cell counts was identified among the infant subjects.

CONCLUSIONS

These data suggest that during early infancy, increased reticulocytosis among asymptomatic SCA subjects is associated with increased severity of disease in childhood.

摘要

目的

在患有镰状细胞贫血的较大儿童中,白细胞计数、血红蛋白和网织红细胞曾被认为是疾病严重程度的标志物。在此,我们探索了这些血液参数是否可用于预测婴儿期后 60-180 天(即新生儿期后)的早期疾病严重程度。

研究设计

回顾性分析了在儿童国家医疗中心镰状细胞项目中至少随访 3 年的 59 名受试者的数据。比较了 60-180 天龄时测量的白细胞计数、血红蛋白和网织红细胞水平与婴儿期和儿童期镰状细胞贫血的临床病程。

结果

大多数患者表现为贫血伴网织红细胞增多。只有在婴儿早期绝对网织红细胞水平升高与生命前 3 年住院率显著增加相关。绝对网织红细胞计数较高还与首次住院时间明显缩短和生命前 3 年临床表现的累积频率增加 4 倍相关。在婴儿受试者中未发现白细胞计数显著增加。

结论

这些数据表明,在婴儿早期,无症状 SCA 患者的网织红细胞增多与儿童期疾病严重程度增加有关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa27/3737358/ae403903616f/pone.0070794.g001.jpg

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