First Department of Pediatrics, School of Medicine, National and Kapodistrian University of Athens, Aghia Sofia' Children's Hospital, Athens, Greece.
Department of Cystic Fibrosis, 'Aghia Sofia', Children's Hospital, Athens, Greece.
J Eur Acad Dermatol Venereol. 2021 Aug;35(8):1717-1724. doi: 10.1111/jdv.17312. Epub 2021 Jun 9.
Aquagenic wrinkling of the palms (AWP) is an excessive and early palmar wrinkling occurring after Brief Immersion to Water (BIW), and has been reported as a frequent finding among cystic fibrosis (CF) patients.
To evaluate and assess the diagnostic performance of BIW test as an initial screening tool for CF diagnosis.
We measured AWP in CF patients, CF-heterozygotes (CF-het) and normal controls. The AWP parameters of palmar wrinkling, oedema, papules, pruritus and pain were assessed at 3, 7 and 11 min after a BIW test was performed for all the participants. Statistical analyses explored the progression of AWP in time for the three groups and assessed the diagnostic performance of BIW test as a diagnostic screening tool for CF.
A total of 250 individuals (100 CF patients, their 50 CF-het parents, 100 healthy controls) were included in the analysis. The average age in years (mean ± SD) was 10.4 ± 4.0 for CF, 35.9 ± 6.1 for CF-het and 10.5 ± 4.0 for controls. The rate of positives for AWP at 3 min among CF patients, CF-het and controls was 68%, 8% and 0%, respectively (P < 0.01). Kaplan-Meier analysis showed a clear trend towards earlier appearance of all five parameters in the direction controls < hetCF < CF (P values <0.01). The best diagnostic performance in detecting between CF patients and non-CF was achieved by the presence of papules and wrinkling at 7 min (sensitivity/specificity: 94.0%/98.3% and 100.0%/92.0%, respectively).
A strong association between AWP and CF was detected. AWP after BIW could be elicited easily and possibly can be used as an initial screening tool to assess if an individual with symptoms and signs that raise the likelihood of CF is a CF patient.
水诱导性手掌皱褶（AWP）是 Brief Immersion to Water（BIW）后手掌过度且早期出现的皱褶，已被报道为囊性纤维化（CF）患者的常见发现。
评估 BIW 测试作为 CF 诊断初始筛查工具的诊断性能。
我们测量了 CF 患者、CF 杂合子（CF-het）和正常对照者的 AWP。所有参与者均进行 BIW 测试后 3、7 和 11 分钟时评估手掌皱褶、水肿、丘疹、瘙痒和疼痛的 AWP 参数。统计分析探索了三组人群中 AWP 在时间上的进展情况，并评估了 BIW 测试作为 CF 诊断筛查工具的诊断性能。
共有 250 人（100 名 CF 患者、他们的 50 名 CF-het 父母和 100 名健康对照者）纳入分析。CF 患者、CF-het 和对照组的平均年龄（mean ± SD）分别为 10.4 ± 4.0 岁、35.9 ± 6.1 岁和 10.5 ± 4.0 岁。CF 患者、CF-het 和对照组在 3 分钟时 AWP 阳性率分别为 68%、8%和 0%（P < 0.01）。Kaplan-Meier 分析显示，所有 5 个参数在对照组<hetCF<CF 方向上的出现趋势更为明显（P 值均<0.01）。在区分 CF 患者和非 CF 患者方面，丘疹和皱褶在 7 分钟时的诊断性能最佳（灵敏度/特异性：94.0%/98.3%和 100.0%/92.0%）。
AWP 与 CF 之间存在很强的关联性。BIW 后诱发的 AWP 简单易行，可能可用作初始筛查工具，评估出现 CF 症状和体征的个体是否为 CF 患者。
