尼日利亚伊图阿扎拉大学教学医院肾病综合征患儿的止血谱。

Haemostatic profile of children with nephrotic syndrome attending University of Nigeria Teaching Hospital Ituku-Ozalla, Nigeria.

机构信息

Department of Paediatrics, College of Medicine, University of Nigeria Enugu Campus, PMB 40001, Enugu, Nigeria.

Department of Haematology and Blood Transfusion, College of Medicine, University of Nigeria Enugu Campus, Enugu, Nigeria.

出版信息

BMC Nephrol. 2022 Aug 4;23(1):274. doi: 10.1186/s12882-022-02894-5.

DOI:10.1186/s12882-022-02894-5

PMID:35927678

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9351170/

Abstract

BACKGROUND

Haemostatic derangements are thought to be due to an imbalance between hepatic synthesis of pro-coagulants and urinary losses of anticoagulants.

OBJECTIVES

This study evaluated the coagulation profile of Nigerian children with nephrotic syndrome and examined the relationship between coagulation variables, disease state and steroid responsiveness.

METHODS

A cross- sectional hospital based study on evaluation of coagulation profile of children with nephrotic syndrome compared with their age- and gender- matched controls.

RESULTS

The median fibrinogen level in subjects and controls was the same (2.9 g/L). Sixteen of 46 (35%) children with nephrotic syndrome had hyperfibrinogenaemia. The median fibrinogen level of children in remission was 2.3 g/L and differed significantly when compared with those of children in relapse (p = 0.001). The median APTT of children with nephrotic syndrome was 45.0 s and differed significantly compared with those of controls (42.0 s) (p value = 0.02). The median prothrombin time in children with and without nephrotic syndrome were 12.0 and 13.0 s respectively, (p = 0.004). About 90% of children with nephrotic syndrome had INR within reference range. Thrombocytosis was found in 15% of children with nephrotic syndrome. The median platelet count in children with new disease was 432 × 10cells/mm and differed significantly when compared with those of controls (p = 0.01). INR was significantly shorter in children with steroid resistant nephrotic syndrome (SRNS) (median 0.8 s; IQR 0.8 -0.9 s) compared with controls (median 1.0 s; IQR 1.0 -1.1 s) (p = 0.01). Steroid sensitivity was the strongest predictor of remission in children with nephrotic syndrome; steroid sensitive patients were 30 times more likely to be in remission than in relapse (OR 30.03; CI 2.01 - 448.04).

CONCLUSION

This study shows that the haemostatic derangements in childhood nephrotic involve mostly fibrinogen, APTT, PT, INR and platelet counts. Antithrombin levels are largely unaffected. Variations in fibrinogen, APTT, PT and INR values may be due to the heterogeneous nature of the disease.

摘要

背景

人们认为止血紊乱是由于肝合成促凝物与尿中抗凝物丢失之间的失衡所致。

目的

本研究评估了尼日利亚肾病综合征患儿的凝血谱，并研究了凝血变量与疾病状态和类固醇反应性之间的关系。

方法

一项基于医院的横断面研究，评估肾病综合征患儿的凝血谱，并与年龄和性别匹配的对照组进行比较。

结果

患儿和对照组的中位纤维蛋白原水平相同（2.9g/L）。46 例肾病综合征患儿中有 16 例（35%）存在高纤维蛋白原血症。缓解期患儿的中位纤维蛋白原水平为 2.3g/L，与复发期患儿的纤维蛋白原水平差异有统计学意义（p=0.001）。肾病综合征患儿的 APTT 中位数为 45.0s，与对照组相比差异有统计学意义（42.0s）（p 值=0.02）。有和无肾病综合征患儿的凝血酶原时间中位数分别为 12.0s 和 13.0s（p=0.004）。约 90%的肾病综合征患儿的 INR 在参考范围内。肾病综合征患儿中发现血小板增多症 15%。新发疾病患儿的血小板计数中位数为 432×10cells/mm，与对照组相比差异有统计学意义（p=0.01）。与对照组相比，类固醇抵抗性肾病综合征（SRNS）患儿的 INR 明显缩短（中位数 0.8s；IQR 0.8-0.9s）（p=0.01）。在肾病综合征患儿中，类固醇敏感性是缓解的最强预测因素；与复发相比，类固醇敏感的患者缓解的可能性高 30 倍（OR 30.03；CI 2.01-448.04）。