RESUMEN
Coronavirus (cov) disease 2019 pandemic caused by severe acute respiratory syndrome cov 2 has imposed significant demands on healthcare systems across the world. These demands were more significant on obstetrics and gynecology (obgyn) patients, who required services that had to continue despite the closure of other services. This paper describes the change management of an obgyn department at a tertiary health-care center. That experience resulted in a complete management shift in the institution and the formation of an infectious disease epidemic plan for respiratory infections. Description of the change management performed, difficulties encountered, and achievements obtained can assist other departments change management when they face similar situations.
RESUMEN
Familial hepatic veno-occlusive disease with immunodeficiency (VODI, OMIM: 235550) is a rare form of combined immune deficiency (CID) that presents in the first few months of life with failure to thrive, recurrent infections, opportunistic infections along with liver impairment. Herein, we are describing a Pakistani patient with a homozygous novel variant in the SP110 gene, presenting with classical phenotypic manifestations of VODI. He presented at the age of 3 months with opportunistic infections and later developed liver failure. Conclusion. Hepatic veno-occlusive disease with immunodeficiency is a rare cause of immunodeficiency, and this is the first case report from the Middle East in a patient of Pakistani origin. It is important to have a high suspicion for this disease, in patients presenting early life with a picture of CID and deranged liver function, as the earlier the diagnosis and treatment, the better the prognosis.
