Rheumatic chorea: relationship to systemic manifestations and response to corticosteroids.

Objective: To describe Sydenham chorea among children in a cohort of patients with rheumatic fever (RF).

Study Design: An existing database was used to identify demographic characteristics, clinical manifestations, and therapy in persons with RF identified in Salt Lake City, Utah, from 1985 through January 2002.

Results: Of 584 cases in the database, 537 (91%) were new-onset RF (median age of 10 years) and 177 (33%) had chorea.

Cerebral sinovenous thrombosis in children: another reason to treat iron deficiency anemia.

Iron deficiency anemia is a rare cause of cerebral sinovenous thrombosis in children. We report three cases of cerebral sinovenous thrombosis and iron deficiency anemia treated at Primary Children's Medical Center in Salt Lake City, Utah, between 1998 and 2001. The children were 9, 19, and 27 months old at the time of admission.

Leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia (LACH) in two brothers: a novel syndrome?

We report a pair of brothers who have leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia. Both presented initially with seizures in the early postnatal period. They have significant developmental delay, and brain MRIs demonstrate leukoencephalopathy, characterized by profound hypomyelination.