RESUMO
Banked unrelated umbilical cord blood matched at 5 of 6 human leukocyte antigen loci was used to reconstitute the immune system in 2 brothers with X-linked lymphoproliferative syndrome and 1 boy with X-linked hyperimmunoglobulin-M syndrome. Pretransplant cytoreduction and posttransplant graft-versus-host prophylaxis were given. Hematopoietic engraftment and correction of the genetic defects were documented by molecular techniques. Two years after transplantation, all 3 patients have normal immune systems. These reports support the wider use of banked partially matched cord blood for transplantation in primary immunodeficiencies.
Assuntos
Sangue Fetal/citologia , Transplante de Células-Tronco Hematopoéticas/métodos , Transtornos Linfoproliferativos/terapia , Ligante de CD40/genética , Criança , Pré-Escolar , Análise Mutacional de DNA/métodos , Doença Enxerto-Hospedeiro/prevenção & controle , Humanos , Lactente , Transtornos Linfoproliferativos/genética , Transtornos Linfoproliferativos/imunologia , Masculino , Linfócitos T/imunologiaRESUMO
Chronic recurrent multifocal osteomyelitis is characterized by recurrent episodes of painful swollen lesions of the bone and overlying skin with radiographic changes and an elevated sedimentation rate. It resembles infectious osteomyelitis but with negative findings on bacterial culture and no response to antibiotics. We treated a 13-year-old girl with interferon gamma for 3 months. She had 11 episodes of chronic recurrent multifocal osteomyelitis in 2 1/2 years before therapy and has had none in the 15 months since therapy, an outcome suggesting a favorable therapeutic response.
Assuntos
Interferon gama/uso terapêutico , Osteomielite/terapia , Adolescente , Biópsia , Criança , Doença Crônica , Feminino , Humanos , Inflamação , Osteomielite/diagnóstico por imagem , Osteomielite/patologia , Psoríase , Radiografia , Proteínas Recombinantes , RecidivaAssuntos
Animais , Cricetinae , Endocitose/fisiologia , Endossomos/fisiologia , Proteínas de Ligação ao GTP/fisiologia , Fusão de Membrana/fisiologia , Trifosfato de Adenosina/metabolismo , Células CHO , Proteínas de Transporte/fisiologia , Linhagem Celular , Sistema Livre de Células , Cricetulus , Proteínas de Ligação ao GTP/genética , Rim , Mesocricetus , Fagocitose , Proteínas rab5 de Ligação ao GTPAssuntos
Animais , Cricetinae , Endocitose , Endossomos , Fusão de Membrana/fisiologia , Proteínas de Ligação ao GTP/fisiologia , Trifosfato de Adenosina , Linhagem Celular , Sistema Livre de Células , Células CHO , Cricetulus , Rim , Mesocricetus , Fagocitose , Proteínas de Ligação ao GTP/genética , Proteínas de Transporte/fisiologiaRESUMO
An infant boy with a congenital immunodeficiency had fatal disseminated measles after administration of a live attenuated measles vaccine. This rare complication was confirmed with molecular virologic techniques. Although efforts to expand availability of vaccinations are critically important, caution is warranted in children with potentially severe immunologic dysfunction.
Assuntos
Vacina contra Sarampo/efeitos adversos , Sarampo/etiologia , Imunodeficiência Combinada Severa/complicações , Insuficiência de Crescimento/etiologia , Humanos , Lactente , Masculino , Sarampo/patologia , Vacinas Atenuadas/efeitos adversosRESUMO
The effect of intravenously administered lipids (intralipid) on immunologic function, complement, and coagulation was prospectively studied over 1 year in 15 children. The mean age of the children was 52.4 +/- 37.9 months; they had received total parenteral nutrition for an average of 3 years. Immunoglobulins (IgA, IgM, IgG), coagulation studies (platelets, prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen, fibrinogen degradation products, factor V) and components of complement (C3, C4, and CH100) were analyzed. Activation of monocytes by opsonized zymosan was measured by chemiluminescence and compared with that of normal control subjects. The clinically stable children had normal monocyte activation and normal complement levels. The PT and PTT values were significantly increased but improved with increased intralipid dose; other coagulation factors were normal. Acutely sick children, however, had decreased fat tolerance with significantly increased serum triglyceride levels and PT and PTT values; their monocyte activation and complement factors remained normal. These data indicate that the dose of intralipid should be lowered during acute illnesses; we suggest close monitoring of PT and PTT values and of serum triglyceride and cholesterol levels to avoid the fat overload syndrome.