Variable Common Immune Deficiency (VCID) is a very heterogeneous condition both clinically and immunologically. It is a group of molecular abnormalities responsible for a defect in antibody production leading to hypog...Variable Common Immune Deficiency (VCID) is a very heterogeneous condition both clinically and immunologically. It is a group of molecular abnormalities responsible for a defect in antibody production leading to hypogammaglobulinemia often associated with autoimmune and/or lymphoproliferative manifestations. Late Onset Combined Immune Deficiency (LOCID) is a type of Variable Common Immune Deficiency (VCID) defined by a defect in antibody production (IgG and IgA ± IgM type), profound CD4 T-cell lymphopenia and frequent opportunistic infections. LOCID has been considered as a distinct entity from VCID due to its particular clinical and immunological profile.展开更多
Devic’s neuromyelitis optica (NMO) is a rare inflammatory disease of the central nervous system that results in optic neuropathy and myelitis. Optic neuritis represents the mode of entry into the disease in more than...Devic’s neuromyelitis optica (NMO) is a rare inflammatory disease of the central nervous system that results in optic neuropathy and myelitis. Optic neuritis represents the mode of entry into the disease in more than two thirds of cases. It is a rare entity in children. There is no effective treatment at present, but some molecules can be used, such as corticosteroids, immunosuppressants and plasma exchange. The prognosis in children is generally favorable. Devic’s neuromyelitis is a condition of unknown etiopathogeny which is functionally critical and requires early and appropriate treatment. We report the case of a 12-year-old girl who presented to emergency with a headache and decreased visual acuity, whose investigations led to the diagnosis of Devic’s neuromyelitis optica.展开更多
文摘Variable Common Immune Deficiency (VCID) is a very heterogeneous condition both clinically and immunologically. It is a group of molecular abnormalities responsible for a defect in antibody production leading to hypogammaglobulinemia often associated with autoimmune and/or lymphoproliferative manifestations. Late Onset Combined Immune Deficiency (LOCID) is a type of Variable Common Immune Deficiency (VCID) defined by a defect in antibody production (IgG and IgA ± IgM type), profound CD4 T-cell lymphopenia and frequent opportunistic infections. LOCID has been considered as a distinct entity from VCID due to its particular clinical and immunological profile.
文摘Devic’s neuromyelitis optica (NMO) is a rare inflammatory disease of the central nervous system that results in optic neuropathy and myelitis. Optic neuritis represents the mode of entry into the disease in more than two thirds of cases. It is a rare entity in children. There is no effective treatment at present, but some molecules can be used, such as corticosteroids, immunosuppressants and plasma exchange. The prognosis in children is generally favorable. Devic’s neuromyelitis is a condition of unknown etiopathogeny which is functionally critical and requires early and appropriate treatment. We report the case of a 12-year-old girl who presented to emergency with a headache and decreased visual acuity, whose investigations led to the diagnosis of Devic’s neuromyelitis optica.
