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21-羟化酶缺陷患者晨尿孕三醇和 17-羟孕酮显著相关。

First Morning Pregnanetriol and 17-Hydroxyprogesterone Correlated Significantly in 21-Hydroxylase Deficiency.

机构信息

Division of Endocrinology and Metabolism, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.

Department of Pediatrics, Oita University Faculty of Medicine, Oita, Japan.

出版信息

Front Endocrinol (Lausanne). 2022 Jan 24;12:808254. doi: 10.3389/fendo.2021.808254. eCollection 2021.

Abstract

BACKGROUND

Biochemically monitoring 21-hydroxylase deficiency (21-OHD) is challenging. Serum/blood 17-hydroxyprogesterone (17OHP) measurements are normally used for this purpose. Urinary pregnanetriol (PT), a urinary metabolite of 17OHP, may also be used. Based on auxological data, we previously reported that the optimal first morning PT value fell in the range of 2.2-3.3 mg/gCr (95% confidence interval of the mean) and 0.59-6.0 mg/gCr (10 - 90 percentile) for monitoring 21-OHD treatment. No report thus far has directly compared the first morning urinary PT value with the 17OHP value at various times during the day.

OBJECTIVE

To explore the correlation between the first morning urinary PT value before glucocorticoid administration and the serum/blood 17OHP value at three time points, namely, before and two and four hours after glucocorticoid administration.

DESIGN

This was a prospective study done at two children's hospitals.

METHODS

In total, 25 patients with 21-OHD aged 3-25 years were recruited. Their urinary PT levels and 17OHP levels were measured for three days within a total period of one week. The first morning PT value was collected on all three days. Dried blood spots and serum were used to measure 17OHP.

RESULTS

The range for the first morning PT value for all the samples (n=69) was 0.10-56.1 mg/gCr. A significant, positive correlation was found between the first morning PT and 17OHP values before medication (r=0.87, p<0.01), and weaker correlation was observed between the first morning PT and 17OHP values after medication.

CONCLUSIONS

The first morning PT correlated more significantly with 17OHP before the morning medication. Measuring the first morning PT value may be more practical and useful for monitoring 21-OHD biochemically.

摘要

背景

生化监测 21-羟化酶缺乏症(21-OHD)具有挑战性。通常使用血清/血液 17-羟孕酮(17OHP)测量值来进行此目的。尿孕三醇(PT),17OHP 的尿代谢产物,也可用于此目的。根据生长发育数据,我们之前报告称,最佳的第一个早晨 PT 值落在 2.2-3.3mg/gCr(平均值的 95%置信区间)和 0.59-6.0mg/gCr(10-90 百分位)范围内,用于监测 21-OHD 治疗。迄今为止,尚无报告直接比较在一天中的不同时间点时,第一个早晨的尿 PT 值与血清/血液 17OHP 值之间的相关性。

目的

探讨糖皮质激素给药前第一个早晨尿 PT 值与血清/血液 17OHP 值在三个时间点(即给药前和给药后两小时和四小时)之间的相关性。

设计

这是在两家儿童医院进行的一项前瞻性研究。

方法

共招募了 25 名年龄在 3-25 岁的 21-OHD 患者。在一周的总时间内,连续三天测量他们的尿 PT 水平和 17OHP 水平。所有三天均采集第一个早晨 PT 值。使用干血斑和血清测量 17OHP。

结果

所有样本(n=69)的第一个早晨 PT 值范围为 0.10-56.1mg/gCr。第一个早晨 PT 与药物前的 17OHP 值之间存在显著的正相关关系(r=0.87,p<0.01),而与药物后 17OHP 值之间的相关性较弱。

结论

第一个早晨 PT 与早晨药物前的 17OHP 相关性更显著。测量第一个早晨 PT 值可能更实用且有助于生化监测 21-OHD。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/71ab/8820395/fccc307f8207/fendo-12-808254-g001.jpg

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