Membranoproliferative glomerulonephritis in a patient with X-linked agammaglobulinemia.

Immune complex and complement systems play an important role in membranoproliferative glomerulonephritis (MPGN). X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by severe hypogammaglobulinemia. We report the case of an XLA patient who developed MPGN during an intravenous immunoglobulin (IVIG) treatment.

An adolescent with pharyngeal-cervical-brachial variant of Guillain-Barré syndrome after cytomegalovirus infection.

A 15-year-old Japanese girl developed bulbar palsy and upper limb-dominant muscle weakness 2 weeks after the onset of an upper respiratory tract infection due to cytomegalovirus (CMV). Her symptoms resembled that seen in the pharyngeal-cervical-brachial variant (PCB) of Guillain-Barré syndrome (GBS). Although bulbar palsy usually continues for several months in PCB, her bulbar palsy was very mild and improved rapidly before intravenous immunoglobulin therapy was instituted.

Early onset distal muscular dystrophy with normal dysferlin expression.

A 7-year-old boy, who was noted to be a slow runner at the age of 2 years, had progressive muscle weakness and atrophy, preferentially affecting distal muscles. At 3 years of age, he had scoliosis and difficulty in standing on tip-toe. Serum creatine kinase was 1074IU/l.

Time interval of measles vaccination in patients with Kawasaki disease treated with additional intravenous immune globulin.

Unlabelled: A standard treatment with a dose of 2 g/kg of intravenous immune globulin (i.v.IG) is sometimes ineffective in some patients with Kawasaki disease (KD), who are commonly treated with additional i.