ABSTRACT
Kawasaki disease is an acute, self-limiting vasculitis of unknown origin, characterized by fever, palms and soles edema, cervical lymphadenopathy, strawberry tongue, and non-exudative conjunctivitis. It is a multisystemic vasculitis that affects predominantly infants and young children. The most feared complication is the development of coronary aneurysms that occurs up to 25% of untreated patients; however there are reports of extra coronary involvement. Herein we present the case of a 2 year-old girl who had a severe symptomatology and persistent fever despite intravenous gammaglobulin. Two years later she presented right hemiparesia and headache, with data from CAT and MRI suggestive of brain mass and deviation of the midline, secondary to left frontoparietal haemorrhage that was treated with a craniotomy. She was discharged on prednisone, ASA and rehabilitation.
Subject(s)
Intracranial Hemorrhages/etiology , Mucocutaneous Lymph Node Syndrome/complications , Child, Preschool , Female , HumansABSTRACT
Neonatal lupus has a rare incidence, distinct from systemic lupus erythematosus. This is an acquired autoimmune disease associated with maternal antibodies to proteins Ro / La (SSA /SSB), transferred by the placenta; it represents the prototype of passive transfer of antibodies from mother to child. The disease can affect the skin, heart, and rarely, the hepatobiliary or hematologic systems. Congenital complete heart block is the most severe form of neonatal lupus. In clinical practice it is important to distinguish in utero a complete from an incomplete atrioventricular block (AV) in order to render prompt care. We present the case of a new born female, who was diagnosed with an atrio-ventricular block at 26 weeksí gestation. When the baby was delivered at 38 weeksí gestation, she presented bradycardia (54 xí). On the suspicion of neonatal lupus, we required antinuclear antibodies, anti-Sm, anti-RNP, anti-SS-A and anti-SS-B, which were positive. A bicameral pacemaker was placed uneventfully.
