战后新生儿筛查的成功:嘌呤核苷磷酸化酶(PNP)缺乏症的国际合作案例。

IF 4 Q1 GENETICS & HEREDITY
Alessandra Bettiol, Roberta Damiano, Nataliia Mytsyk, Nataliia Samonenko, Gabriella Cericola, Carsten Speckmann, Nataliia Olkhovich, Renzo Guerrini, Giancarlo la Marca
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引用次数: 0

摘要

乌克兰的医疗保健系统在持续的新生儿筛查(NBS)中显示出非凡的弹性,超越了战争的挑战。在冲突中,一名乌克兰新生儿筛查出极为罕见的严重联合免疫缺陷(SCID)-嘌呤核苷磷酸化酶(PNP)缺乏症阳性。乌克兰通过实时荧光定量PCR成功对新生儿干血斑(DBS)进行了NBS检测,结果显示t细胞受体和kappa删除重组切除环(TREC/KREC)明显减少。由于与家人沟通困难,重新测试被推迟。新的DBS全外显子组测序证实了诊断。这名新生儿是异体造血干细胞移植(HSCT)的候选者,这是唯一能治愈的治疗方法。HSCT是一项复杂的手术,尽管乌克兰存在冲突,但仍在进行中。然而,由于心理社会压力,这家人在德国寻求医疗支持,6个月时成功进行了造血干细胞移植。作为与意大利合作计划的一部分,根据托斯卡纳SCID NBS建立的协议,使用串联质谱法在相同的DBSs上对PNP生物标志物进行量化,作为其诊断性能概念的证明。这一案例突出了维持预防和挽救生命的保健服务的重要性,并反映了国际伙伴关系在危机时期维护保健权方面的关键作用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

The Success of Newborn Screening Beyond War: An International Collaborative Case of Purine Nucleoside Phosphorylase (PNP) Deficiency.

The Success of Newborn Screening Beyond War: An International Collaborative Case of Purine Nucleoside Phosphorylase (PNP) Deficiency.

The Success of Newborn Screening Beyond War: An International Collaborative Case of Purine Nucleoside Phosphorylase (PNP) Deficiency.

Ukraine's healthcare system has shown remarkable resilience in continuing newborn screening (NBS), beyond the challenges of war. Amid the conflict, a Ukrainian newborn screened positive for an extremely rare severe combined immunodeficiency (SCID)-purine nucleoside phosphorylase (PNP) deficiency. Ukraine successfully carried out NBS on a neonatal dried blood spot (DBS) by real-time PCR, which showed remarkably reduced T-cell receptor and kappa-deleting recombination excision circles (TREC/KREC). Retesting was delayed due to communication difficulties with the family. Whole exome sequencing on a new DBS confirmed the diagnosis. The newborn was a candidate for allogeneic hematopoietic stem cell transplantation (HSCT), the only curative treatment. HSCT is a complex procedure still ongoing in Ukraine despite the conflict. However, due to the psychosocial strain, the family sought medical support in Germany, where HSCT was performed successfully at 6 months. As part of a collaborative initiative with Italy, PNP biomarkers were quantified on the same DBSs using tandem mass spectrometry, according to the protocols established for SCID NBS in Tuscany, serving as a proof of concept of its diagnostic performance. This case highlights the importance of sustaining preventive and life-saving healthcare services, and reflects the key role of international partnerships in upholding the right to healthcare in times of crisis.

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来源期刊
International Journal of Neonatal Screening
International Journal of Neonatal Screening Medicine-Pediatrics, Perinatology and Child Health
CiteScore
6.70
自引率
20.00%
发文量
56
审稿时长
11 weeks
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