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血清铁蛋白与小儿输血依赖型地中海贫血生长状态的关系

Relationship between serum ferritin and growth status of pediatric transfusion dependent thalassemia.

作者信息

Cahyadi Andi, Ugrasena I Dewa Gede, Andarsini Mia Ratwita, Larasati Maria Christina Shanty, Jauhari Raden Muhammad Zulfan, Arumsari Diah Kusuma

机构信息

Department of Child Health, Faculty of Medicine, Universitas Airlangga/Dr. Soetomo General Academic Hospital, Surabaya, East Java, Indonesia.

出版信息

Caspian J Intern Med. 2023 Summer;14(3):425-432. doi: 10.22088/cjim.14.3.425.

Abstract

BACKGROUND

Growth retardation is a long-term complication in pediatric transfusion-dependent thalassemias (TDTs), presented as short-stature and upper body segment shortening. The cause of this condition was chronic hypoxia, iron overload, endocrinopathy, inadequate transfusion, and iron chelation. We analyze the relationship between ferritin level and growth status of pediatric TDTs.

METHODS

This was a cross-sectional study on pediatric TDTs aged 2-18 years old at Dr. Soetomo General Academic Hospital Surabaya, Indonesia conducted in 2020. They required blood transfusion every 2-4 weeks. We evaluated the ratio of upper/lower body segments, weight for age Z-score (WAZ), height for age Z-score (HAZ), and body mass index (BMI) Z-score, based on CDC growth chart as growth status parameters. Serum ferritin was checked every three months to determine iron overload and iron chelation (deferiprone, deferasirox and deferoxamine). We used Spearman correlation and Mann-Whitney U test to analyze between variables (α=0.05).

RESULTS

We enrolled 15/29 males with median age 10.5 years. Serum Ferritin had negative correlation with the ratio of upper/lower body segments (rho=-0.552; P=0.002), but not for HAZ (rho=-0.078; P=0.694), WAZ (rho=-0.186; P=0.342), BMI Z-score (rho=-0.089; P=0.653) especially if serum ferritin was above 2500 µ/L. In deferiprone group (n=8), the WAZ (P=0.034) and BMI Z-score (P=0.031) were lower; but the ratio of upper/lower body segments was greater (P=0.039) than the deferasirox group.

CONCLUSION

Growth retardation was more visible in pediatric TDTs with high ferritin and in deferiprone group. The height and the ratio of upper/lower body segments of the body were more affected.

摘要

背景

生长发育迟缓是小儿输血依赖型地中海贫血(TDT)的一种长期并发症,表现为身材矮小和上身段缩短。这种情况的原因是慢性缺氧、铁过载、内分泌病、输血不足以及铁螯合作用。我们分析了小儿TDT患者铁蛋白水平与生长状况之间的关系。

方法

这是一项于2020年在印度尼西亚泗水苏托莫综合学术医院对2至18岁小儿TDT患者进行的横断面研究。他们每2至4周需要输血一次。我们根据美国疾病控制与预防中心(CDC)生长图表评估上身段与下身段的比例、年龄别体重Z评分(WAZ)、年龄别身高Z评分(HAZ)和体重指数(BMI)Z评分,将其作为生长状况参数。每三个月检查一次血清铁蛋白,以确定铁过载和铁螯合情况(去铁酮、地拉罗司和去铁胺)。我们使用Spearman相关性分析和Mann-Whitney U检验来分析变量之间的关系(α=0.05)。

结果

我们纳入了15名男性/29名患者,中位年龄为10.5岁。血清铁蛋白与上身段与下身段的比例呈负相关(rho=-0.552;P=0.002),但与HAZ(rho=-0.078;P=0.694)、WAZ(rho=-0.186;P=0.342)、BMI Z评分(rho=-0.089;P=0.653)无相关性,尤其是当血清铁蛋白高于2500µ/L时。在去铁酮组(n=8)中,WAZ(P=0.034)和BMI Z评分(P=0.031)较低;但上身段与下身段的比例高于地拉罗司组(P=0.039)。

结论

在铁蛋白水平高的小儿TDT患者和去铁酮组中,生长发育迟缓更为明显。身高和上身段与下身段的比例受影响更大。

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