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镰状细胞贫血无症状儿童的镰状细胞肾病及相关因素

Sickle Cell Nephropathy and Associated Factors among Asymptomatic Children with Sickle Cell Anaemia.

作者信息

Nnaji Uchenna Modestus, Ogoke Christian Chukwukere, Okafor Henrietta Uche, Achigbu Kingsley I

机构信息

Department of Paediatrics, Federal Medical Centre, Owerri, Nigeria.

Department of Clinical Neurophysiology & Child Neurology, Mother Healthcare Diagnostics & Hospital, Owerri, Nigeria.

出版信息

Int J Pediatr. 2020 Aug 14;2020:1286432. doi: 10.1155/2020/1286432. eCollection 2020.

DOI:10.1155/2020/1286432
PMID:32908550
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7474388/
Abstract

BACKGROUND

Sickle cell nephropathy (SCN) is a serious complication of sickle cell anaemia (SCA) with asymptomatic onset in childhood and possible progression to chronic kidney disease (CKD). In Southeast Nigeria, few studies have evaluated renal function in paediatric SCA patients for early detection of renal impairment and early intervention to reduce morbidity and mortality. Therefore, this study evaluated the renal function of paediatric SCA patients in a steady state based on glomerular filtration rate and urinalysis findings (proteinuria and haematuria).

METHODS

A cross-sectional study of consecutively recruited sixty haemoglobin SS (HbSS) children in a steady state and sixty age- and sex-matched haemoglobin AA (HbAA) controls aged 2-18 years was done. Renal function of HbSS subjects was evaluated using estimated glomerular filtration rate (eGFR) which was compared with healthy HbAA subjects. The prevalence of significant proteinuria and haematuria, its association with eGFR, and the effect of past sickle cell crisis (in the preceding 24 months) on renal function were also evaluated.

RESULTS

Mean eGFR was significantly higher in HbSS subjects than in the HbAA subjects ( = 0.001) and decreased with age. Significant proteinuria and haematuria were more prevalent in the HbSS group (3.4% and 6.7%, respectively) compared to the HbAA subjects (0% and 0%, respectively) ( = 0.496 and 0.119, respectively). No significant association was observed between eGFR and proteinuria ( = 1.000) or haematuria ( = 1.000). There was a positive correlation between eGFR and frequency of past painful crisis that required hospitalization ( = 0.138, = 0.295) and between eGFR and frequency of blood transfusion ( = 0.679, ≤ 0.001).

CONCLUSIONS

Asymptomatic paediatric HbSS (SCA) patients had higher mean eGFR indicating an increased risk of nephropathy. There was no association between eGFR and proteinuria or haematuria. Frequent sickle cell crises especially one requiring transfusion were positively correlated with hyperfiltration.

摘要

背景

镰状细胞肾病(SCN)是镰状细胞贫血(SCA)的一种严重并发症,在儿童期无症状起病,可能进展为慢性肾脏病(CKD)。在尼日利亚东南部,很少有研究评估小儿SCA患者的肾功能,以早期发现肾功能损害并进行早期干预,从而降低发病率和死亡率。因此,本研究基于肾小球滤过率和尿液分析结果(蛋白尿和血尿)评估了处于稳定状态的小儿SCA患者的肾功能。

方法

对连续招募的60名处于稳定状态的血红蛋白SS(HbSS)儿童和60名年龄及性别匹配的2至18岁血红蛋白AA(HbAA)对照儿童进行了横断面研究。使用估计肾小球滤过率(eGFR)评估HbSS受试者的肾功能,并与健康的HbAA受试者进行比较。还评估了显著蛋白尿和血尿的患病率、其与eGFR的关联以及过去镰状细胞危象(在之前24个月内)对肾功能的影响。

结果

HbSS受试者的平均eGFR显著高于HbAA受试者(P = 0.001),且随年龄降低。与HbAA受试者(分别为0%和0%)相比,HbSS组中显著蛋白尿和血尿更为普遍(分别为3.4%和6.7%)(P分别为0.496和0.119)。未观察到eGFR与蛋白尿(P = 1.000)或血尿(P = 1.000)之间存在显著关联。eGFR与过去需要住院治疗的疼痛性危象频率之间存在正相关(P = 0.138,r = 0.295),eGFR与输血频率之间也存在正相关(P = 0.679,r ≤ 0.001)。

结论

无症状的小儿HbSS(SCA)患者平均eGFR较高,表明肾病风险增加。eGFR与蛋白尿或血尿之间无关联。频繁的镰状细胞危象,尤其是需要输血的危象,与超滤呈正相关。

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