Autoimmune vasculitis in a child following tetralogy of Fallot repair in Uganda

nbsp nbsp nbsp Introduction nbsp Tetralogy of Fallot is one of the conotruncal abnormalities and is the most common cyanotic heart disease worldwide Conotruncal abnormalities have been closely associated with q deletion syndrome Children with q deletion syndrome often have thymic hypoplasia hypocalcaemia and have an increased susceptibility to viral fungal and bacterial infections due to poor T cell production Autoimmune vasculitis has not been well documented among individuals with Tetralogy of Fallot TOF though a few reports exist in association with Q deletion syndrome nbsp nbsp nbsp Case summary nbsp We report a case of an year old child post TOF repair who presented to the Uganda Heart Institute with persistent high grade fevers abdominal pain mucosal bleeds and hypocalcaemia hypokalemia hypomagnesaemia thrombocytopenia and elevated acute phase reactants ndash leukocytosis x neutrophil count x elevated C reactive protein mg l and elevated immunological tests C ANCA U ml She improved on intravenous methyl prednisolone nbsp nbsp nbsp Conclusion nbsp Children with Tetralogy of Fallot can still manifest with complications such as autoimmune disorders and severe infections given the association with q deletion syndrome Screening for autoimmune abnormalities and electrolytes especially calcium improves on their outcome We were not able to perform the FISH analysis for Fluorescent in Situ Hybridization to confirm the diagnosis of q deletion syndrome in KT