A male neonate born to a healthy primigravida was noted to have jaundice and hepatosplenomegaly at birth. Laboratory investigations revealed pancytopenia, conjugated hyperbilirubinemia, vitamin K unresponsive coagulopathy, and elevated transaminases with low albumin. Investigations for TORCH infection, gestational alloimmune liver disease, and metabolic liver diseases were negative and bone marrow examination showed hemophagocytic activity. The presence of rash (Fig. 1) prompted us to do antinuclear, anti-Ro, and anti-La antibodies in both mother and baby, which were positive. Electrocardiogram and echocardiogram were normal. He was treated with steroids for neonatal lupus associated with macrophage activation syndrome. He made a complete recovery with resolution of jaundice, whereas his mother has been commenced on antilupus medications. Neonatal lupus commonly presents with congenital heart block, rash, and occurs due to the transplacental passage of maternal autoantibodies anti-Ro and anti-La (1). Pulmonary, hepatobiliary, and neurologic manifestations are increasingly being reported (Fig. 1).The spectrum of hepatobiliary disease ranges from fulminant liver failure to mild elevation of transaminases with or without cholestasis (2).The diagnosis is challenging in absence of maternal history for connective tissue diseases and other features of lupus may not be present. Most neonates will recover with conservative management; however; immunoglobulin therapy, steroids, and cyclosporine may be required in severe cases associated with macrophage activation syndrome (3,4).
Paediatric multisystem inflammatory syndrome: temporally associated with
SARS-COV-2 (PIMS-TS) is a well described rare but severe COVID-19
related syndrome. PIMS-TS have been reported in children from
geographical areas of high COVID-19 infection. Most children with
PIMS-TS require management in an intensive care unit with variable
respiratory involvement. Adults recovering from COVID-19 infection have
been reported to suffer from respiratory morbidity but such outcomes are
unknown in children. We present the first report of normal short term
respiratory outcomes as measured by spirometry in children with
SARS-COV-2 antibody positive, PIMS-TS syndrome managed at a specialist
children’s hospital in the UK.
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