Nasal potential difference in young children is feasible - Report from a national referral center

IF 3.5 3区医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

Respiratory medicine Pub Date : 2025-04-18 DOI:10.1016/j.rmed.2025.108097

Michael Cohen , Mordechai Slae , Ayelet Gamliel , David Shoseyov , Malena Cohen-Cymberknoh , Oded Breuer , Galit Livnat , Liron Birimberg-Schwartz , Michael Wilschanski

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引用次数: 0

Abstract

Background

–Nasal Potential Difference (NPD) is an established diagnostic tool for CF. However, standardized values for very young patients are lacking.

Aim

– To evaluate the feasibility of performing NPD testing in young children.

Methods

– We modified the standard NPD protocol for young children by shortening the infusion time per solution to 2 min. No sedation was required.

Results

– Fifty-five children aged 6 days to 3 years were enrolled from 2010 to 2024, with 50 completing the modified NPD protocol, 2 failing to tolerate the procedure, and 3 having inconclusive results. Fourty-eight results were normal, and 2 abnormal. In the normal group, the basal potential difference (PD) averaged −17 ± 6 mV, amiloride response was 7 ± 3 mV, and chloride-free + isoproterenol response was −9 ± 5 mV. The two abnormal results were observed in a 12-month-old with failure to thrive and a 6-day-old with meconium plug, presenting with basal PD of −30 mV and −28 mV respectively, with no cAMP response. CF was diagnosed in the 12-month-old based on clinical symptoms and sweat test of 70 meq/L. The 6-day-old, is being followed for mild asthma with normal repeat sweat testing. None of the children with normal NPD results were diagnosed with CF.

Conclusion

–NPD testing is feasable in very young children. This protocol could be valuable for investigating cases of CF screened positive indeterminate diagnosis (CFSPID) or CF metabolic syndrome (CFMS), and for pre- and post-testing in clinical trials. Further validation studies are warranted.

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幼儿鼻电位差是可行的-来自国家转诊中心的报告

背景：鼻电位差（NPD）是CF的诊断工具。然而，对于非常年轻的患者，缺乏标准化的值。目的：评估在幼儿中进行NPD测试的可行性。方法：我们修改了儿童标准NPD方案，将每次溶液的输注时间缩短至2分钟。不需要镇静。结果：从2010年到2024年，55名6天至3岁的儿童入组，其中50名完成了修改后的NPD方案，2名无法耐受该手术，3名结果不确定。48例正常，2例异常。正常组基底电位差（PD）平均为- 17±6 mV，阿米洛利反应为7±3 mV，无氯+异丙肾上腺素反应为- 9±5 mV。这两种异常结果分别发生在12个月大的发育不全和6天大的胎便堵塞，分别表现为- 30 mV和- 28 mV的基础PD，无cAMP反应。在12个月时根据临床症状和70 meq/L汗液试验诊断CF。这个6天大的孩子正在接受轻度哮喘的随访，并进行正常的重复汗液测试。结论：NPD测试在幼童中是可行的。该方案可用于调查CF筛查阳性不确定诊断（CFSPID）或CF代谢综合征（CFMS）的病例，以及临床试验的前后测试。进一步的验证研究是必要的。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Respiratory medicine 医学-呼吸系统

CiteScore

7.50

自引率

0.00%

发文量

199

审稿时长

38 days

期刊介绍： Respiratory Medicine is an internationally-renowned journal devoted to the rapid publication of clinically-relevant respiratory medicine research. It combines cutting-edge original research with state-of-the-art reviews dealing with all aspects of respiratory diseases and therapeutic interventions. Topics include adult and paediatric medicine, epidemiology, immunology and cell biology, physiology, occupational disorders, and the role of allergens and pollutants. Respiratory Medicine is increasingly the journal of choice for publication of phased trial work, commenting on effectiveness, dosage and methods of action.