1/5. association of autoimmunity with IgG2 and IgG4 subclass deficiency in a growth hormone-deficient child.An association between humoral immune deficiency and childhood autoimmune disease has been previously established. We describe a 7-year-old male with severe autoimmune disease, recurrent infections, a marked deficiency of IgG2 and IgG4, and an inability to respond to polysaccharide antigens. This child was also found to have isolated growth hormone (GH) deficiency. Laboratory results included a positive anti-smooth muscle antibody, a positive Raji-cell assay for immune complexes, and normal levels of IgG, IgM, and IgA. IgG subclasses revealed an IgG1 of 1225 (normal for age, 280-1120 mg/dl), IgG2 of less than 10 (30-630 mg/dl), IgG3 of 36 (40-250 mg/dl), and IgG4 of less than 4 (11-620 mg/dl). No increase in antibody titer was noted to either Pneumovax or unconjugated haemophilus influenzae vaccine. Numbers of circulating B cells (CD19) were markedly diminished (less than 0.5%). liver biopsies have shown chronic active hepatitis. Somatomedin C was 0.28 U/ml (normal for age, 0.5-2.06 U/ml). Challenge with either L-dopa or clonidine produced a peak GH response of 2.3 ng/ml (normals = greater than 7 ng/ml). Children with autoimmune disorders should be evaluated for IgG subclass deficiencies and ability to make antibody in response to antigen challenge regardless of the serum immunoglobulin levels. Growth failure in immune-deficient children should not be assumed to be due to chronic illness or recurrent infections. Other etiologies for growth failure should be sought.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/5. Combined IgG2, IgG4 and iga deficiency: low C1q concentrations and the presence of excess C1r and C1s in an adult patient with recurrent pneumococcal infections.The complement (C) profile was investigated in an adult patient with combined IgG2, IgG4 and iga deficiency and recurrent pneumococcal infections. The analysis revealed no gross impairment of the classic and alternative pathways of C activation. However, the concentrations of circulating C1q were persistently decreased, and the sera contained an excess of C1r-C1s complexes, resembling the C1 aberrations previously found in children with recurrent acute otitis media. The concentrations of C4 in the patient were persistently low. This could be ascribed to partial C4 deficiency with lack of C4A variants. The patient's IgG and IgM antibody responses to pneumococcal capsular polysaccharides and to other bacterial carbohydrate antigens were very poor. Interestingly, pneumococcal C-polysaccharide (CPS) could be detected in serum obtained during infection-free periods. Since CPS has been shown to bind C1q without causing C1 activation, the possibility was considered that the C1 aberrations in serum were due to circulating CPS. After administration of intramuscular gammaglobulin to the patient, the serum C1q levels were observed to return to normal.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
3/5. Continuous ambulatory gamma globulin infusion in the home setting: a treatment proposal for a high-risk patient.As infusion therapies increasingly move from the hospital to the home setting, home I.V. therapy nurses must frequently decide which therapies are appropriate for home delivery. These practitioners must find innovative, cost-effective, and, most important, safe methods of providing new I.V. therapies in the home. A medically complex patient with a strong desire to maintain the most normal lifestyle possible was introduced to our infusion team. We accomplished our goal--to institute and maintain home therapy on this patient--by designing a treatment proposal that outlined specific guidelines, criteria, and recommendations for all aspects of the infusion care. This proposal can serve as a model for any experimental or unusual treatment being considered for home therapy.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
4/5. Desquamative interstitial pneumonia and antigen-antibody complexes in two infants with congenital rubella.Desquamative interstitial pneumonia was observed in two infants with the late-onset congenital rubella syndrome. In both infants this unusual lung disease was associated with circulating immunoglobulin m complexes and interstitial pulmonary deposits of IgM by immunofluorescence. Both infants had igg deficiency. The first child recovered with a reduction in IgM complex levels and synthesis of rubella-specific IgG. The second infant died during the acute phase of his illness at which time there were high serum concentrations of IgM complexes and slightly increased levels of IgG complexes. Delayed maturation of the immune response in congenital rubella may predispose to persistent antigenemia and pulmonary deposition of rubella antigen-containing IgM complexes resulting in an acute form of interstitial pneumonia.- - - - - - - - - - ranking = 9keywords = complex (Clic here for more details about this article) |
5/5. Inherited deficiency of the third component of complement associated with recurrent pyogenic infections, circulating immune complexes, and vasculitis in a Dutch family.A family is described in which 3/11 children showed a homozygous deficiency of C3, and both parents and six other children had subnormal levels of C3. The three children with selective C3 deficiency suffered repeatedly from bacterial infections, whereas the parents and the other siblings were clinically healthy. During infectious episodes the patients showed a maculopapular skin rash, and at such times immune complexes were present in the serum. biopsy specimens of the skin lesions showed the picture of leukocytoclastic vasculitis.- - - - - - - - - - ranking = 5keywords = complex (Clic here for more details about this article) |