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.
Authors
- Carsten Speckmann (ORCID: https://orcid.org/0000-0002-6217-1556)
- Renzo Guerrini (ORCID: https://orcid.org/0000-0002-7272-7079)
- Nataliia Mytsyk (ORCID: https://orcid.org/0009-0005-4287-1570)
- Giancarlo la Marca (ORCID: https://orcid.org/0000-0003-3319-7260)
- Roberta Damiano (ORCID: https://orcid.org/0000-0002-0150-728X)
- Alessandra Bettiol (ORCID: https://orcid.org/0000-0002-2292-1412)
- Nataliia Samonenko (ORCID: https://orcid.org/0000-0002-6927-3301)
- N. V. Olkhovych (ORCID: https://orcid.org/0000-0001-6468-2782)
- Gabriella Cericola
Institutions
- University Medical Center Freiburg (DE)
- Meyer Children's Hospital (IT)
- Children's Specialized Hospital (US)
- University of Florence (IT)
Publication Details
- Journal
- International Journal of Neonatal Screening
- Published
- 2025-09-16
- DOI
- https://doi.org/10.3390/ijns11030079
- Primary Topic
- Immunodeficiency and Autoimmune Disorders
- Type
- article
- Field-Weighted Citation Impact
- 0.00