Supportive palliative care in an infant with RAG1-related Omenn syndrome and vaccine-associated paralytic poliomyelitis was unable to prevent death at 8 months of age.
Case Report (n=1)
No
Highlights the severe risk of vaccine-associated paralytic poliomyelitis from oral polio vaccine in infants with undiagnosed severe combined immunodeficiency in settings lacking newborn screening.
Severe combined immunodeficiency (SCID) is a life-threatening inborn error of immunity (IEI) that is potentially curable when diagnosed early but is associated with high morbidity and mortality when recognition is delayed. In settings without universal newborn screening (NBS), and where vaccination programs rely heavily on live vaccines, affected infants are at risk of preventable complications. We report an Indonesian girl, the first child of non-consanguineous parents, who at the age of three months, developed a generalized maculopapular rash, followed by febrile encephalopathy with acute flaccid paralysis and hepatomegaly at four months of age. Investigations revealed pan-hypogammaglobulinemia (IgG RAG1 variants. As the child received oral polio vaccine (OPV) at day 8 of life, vaccine-associated paralytic poliomyelitis (VAPP) was suspected, which was confirmed with positive enterovirus PCR in the cerebral spinal fluid; Sabin-like poliovirus serotype 1 was isolated from stool. Hematopoietic stem cell transplantation (HSCT) was discussed but the family opted for supportive palliative care. The child died of a febrile illness at 8 months of age. Although IEI was initially suspected, the use of limited flow cytometric diagnostic evaluation delayed the definitive diagnosis in the child. The lack of NBS for SCID, together with the continued usage of OPV in the routine childhood vaccination program in most lower to middle income countries is the perfect storm for VAPP in children born with IEIs in these settings. This case highlights that 1) there is an urgent need to strengthen diagnostic capabilities in resource-limited settings, 2) the transition from OPV to inactivated polio vaccine (IPV) is a public health priority, and 3) there are significant barriers to the implementation of SCID NBS in Southeast Asia. Addressing these systemic gaps is critical to improve survival outcomes for children with severe but treatable IEIs.
Chan et al. (Fri,) conducted a case report in Infant girl (5 months old) with RAG1-related Omenn syndrome presenting with vaccine-associated paralytic poliomyelitis (VAPP) following oral polio vaccine (OPV) administration in the context of severe combined immunodeficiency (SCID) (n=1). Supportive palliative care and immunosuppression (oral prednisolone 1 mg/kg/day, ciclosporin 5 mg/kg/day, IV immunoglobulin approx. 0.4g/kg every 3-4 weeks) was evaluated on Survival status and neurological recovery post diagnosis and initiation of supportive care in SCID infant with VAPP. Supportive palliative care in an infant with RAG1-related Omenn syndrome and vaccine-associated paralytic poliomyelitis was unable to prevent death at 8 months of age.