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30. Clinical, immunological, and molecular analysis in a large cohort of patients with X-linked agammaglobulinemia: an Italian multicenter study

Author

Plebani, A, Soresina, A, Rondelli, R, Amato, Gm, Azzari, C, Cardinale, F, Cazzola, G, Consolini, R, De Mattia, D, Dell'Erba, G, Duse, M, Fiorini, M, Martino, Silvana, Martire, B, Masi, M, Monafo, V, Moschese, V, Notarangelo, Ld, Orlandi, P, Panei, P, Pession, A, Pietrogrande, Mc, Pignata, C, Quinti, I, Ragno, V, Rossi, P, Sciotto, A, Stabile, A, Italian Pediatric Group for XLA AIEOP, Plebani, A., Soresina, A., Rondelli, R., Amato, G. M., Azzari, C., Cardinale, F., Cazzola, G., Consolini, R., DE MATTIA, D., Dell'Erba, G., Duse, M., Fiorini, M., Martino, S., Martire, B., Masi, M., Monafo, V., Moschese, V., Notarangelo, L. D., Orlandi, P., Panei, P., Pession, A., Pietrogrande, M. C., Pignata, Claudio, Quinti, I., Ragno, V., Rossi, P., Sciotto, A., and Stabile, A.

Subjects
Lung Diseases, Adult, Male, Pediatrics, medicine.medical_specialty, Genetic Linkage, Agammaglobulinemia, Humans, Infant, Newborn, Protein-Tyrosine Kinases, Child, Child, Preschool, X Chromosome, Immunoglobulins, Intravenous, Cohort Studies, Chronic Disease, Follow-Up Studies, Adolescent, Mutation, clinical features, X-linked agammaglobulinemia, Immunology, Immunoglobulins, infections, intravenous immunoglobulin, BTK mutation, Sepsis, Immunopathology, Epidemiology, medicine, Agammaglobulinaemia Tyrosine Kinase, Immunology and Allergy, Preschool, Settore MED/38 - Pediatria Generale e Specialistica, BTK mutations, business.industry, Chronic sinusitis, Meningoencephalitis, Infant, medicine.disease, Newborn, agammaglobulinemia, business, Intravenous, Meningitis, Cohort study
Abstract

A questionnaire-based retrospective clinical and immunological survey was conducted in 73 males with a definite diagnosis of X-linked agammaglobulinemia based on BTK sequence analysis. Forty-four were sporadic and 29 familial cases. At December 2000, the patients' ages ranged from 2 to 33 years; mean age at diagnosis and mean duration of follow-up were 3.5 and 10 years respectively. After the mid-1980s all but 2 were on intravenous immunoglobulin (IVIG) substitution therapy, with residual IgG >500 mg/dl in 94% of the patients at the time of enrollment. Respiratory infections were the most frequent manifestation both prior to diagnosis and over follow-up. Chronic lung disease (CLD) was present in 24 patients, in 15 already at diagnosis and in 9 more by 2000. The cumulative risk to present at diagnosis with CLD increased from 0.17 to 0.40 and 0.78 when the diagnosis was made at the ages of 5, 10, and 15 years respectively. For the 9 patients who developed CLD during follow-up, the duration of follow-up, rather than age at diagnosis; previous administration of intramuscular immunoglobulin; and residual IgG levels had a significant effect on the development of CLD. Chronic sinusitis was present in 35 patients (48%), in 15 already at diagnosis and in 20 by 2000. Sistemic infections such as sepsis and meningitis/meningoencephalitis decreased over follow-up, probably due to optimal protection provided by high circulating IgG levels reached with IVIG.

Published
2002

31. Studio clinico multicentrico italiano sulla agammaglobulinemia X-recessiva (XLA)

Author

PLEBANI A., MARTIRE B., CARDINALE F., GAZZOLA G., DE MATTIA D., DUSE M., FIORE M., MARTINO S., MASI M., MONAFO V., PIETROGRANDE C., QUINTI I., RAGNO V., ROSSI P., STABILE A., MOSCHESE V., PIGNATA, CLAUDIO, Plebani, A., Martire, B., Cardinale, F., Gazzola, G., DE MATTIA, D., Duse, M., Fiore, M., Martino, S., Masi, M., Monafo, V., Pietrogrande, C., Pignata, Claudio, Quinti, I., Ragno, V., Rossi, P., Stabile, A., and Moschese, V.

Published
1999

37. X-linked agammaglobulinemia (XLA): An Italian Multicenter Clinical Study

Author

Plebani, A, Baldassarre, M, Cardinale, F, Cazzola, G, De Mattia, D, Duse, M, Flore, M, Martino, S, Masi, M, Monafo, V, Pietrogrande, C, Pignata, C, Quinti, I, Ragno, V, Rossi, P, Stabile, A, and Moschese, V

Subjects
Clinical study, Settore MED/38 - Pediatria Generale e Specialistica, Pediatrics, medicine.medical_specialty, business.industry, Immunology, Medicine, X-linked agammaglobulinemia, business, medicine.disease, Molecular Biology
Published
1998

38. CD40lbase: a database of CD40L gene mutations causing X-linked hyper-IgM syndrome

Author

Notarangelo, Ld, Peitsch, Mc, Abrahamsen, Tg, Bachelot, C., Bordigoni, P., Cant, Aj, Chapel, H., Clementi, M., Deacock, S., Saint Basile, G., Duse, M., Espanol, T., Etzioni, A., Fasth, A., Fischer, A., Giliani, S., Gomez, L., Hammarstorm, L., Jones, A., Kanariou, M., Kinnon, C., Klemola, T., Kroczek, Ra, Levy, J., Matamoros, N., Monafo, V., Paolucci, P., Reznick, I., Sanal, O., C. I. Edvard SMITH, Thompson, Ra, Tovo, P., Villa, A., Vihinen, M., Vossen, J., and Zegers, Bj

Subjects
Membrane Glycoproteins, X Chromosome, Databases, Factual, Genetic Linkage, X-linked hyper-IgM syndrome, CD40L, CD40 Ligand, hemic and immune systems, Ligands, Immunoglobulin M, Hypergammaglobulinemia, Mutation, Humans, CD40 Antigens
Abstract

X-linked hyper-IgM syndrome (X-HIM) is an immunodeficiency caused by mutations in the gene encoding the CD40 ligand (CD40L). A database (CD40Lbase) of CD40L mutations has now been established, and the resultant information, together with other mutations reported elsewhere in the literature, is presented here.

Published
1996

48. Serum IgG subclass concentrations in healthy subjects at different age: Age normal percentile charts.

Author

Plebani, A., Ugazio, A., Avanzini, M., Massimi, P., Zonta, L., Monafo, V., and Burgio, G.

Abstract

IgG subclass levels were determined in 448 normal children from 6 months to 18 years of age and in 141 healthy adults by radial immunodiffusion using monoclonal antibodies. Age-normal percentile values were calculated for each year of age up to 18 years for IgG1, IgG2, IgG3 and in adults for all four subclasses. The broad spread of IgG4 values in children did not permit calculation of reference values. [ABSTRACT FROM AUTHOR]

Published
1990
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50. IgM and IgD concentrations in the serum and secretions of children with selective IgA deficiency.

Author

Plebani, A., Mira, E., Mevio, E., Monafo, V., Notarangelo, L. D., avanzini, A., and Ugazio, A. G.

Subjects
JUVENILE diseases, SERUM, BLOOD plasma, ENZYME-linked immunosorbent assay, SALIVA, IMMUNOGLOBULINS
Abstract

Serum IgG and serum and secretory IgM, IgA and IgD levels were determined in 14 children with selective IgA deficiency and in 12 age and sex matched healthy controls. IgD was determined using a highly sensitive ELISA technique. In the healthy controls serum IgG, IgA and IgM were all in the age normal range, and serum IgA was significantly higher than secretory IgA with IgA in nasal secretions being significantly higher than in saliva. In the IgA deficient children serum IgG and IgM and secretory IgM were present in higher concentrations than in the controls but the difference was statistically significant only for serum IgG and salivary IgM. IgD was detectable in the serum and secretions of all patients and all but one control subject. Like IgM, serum IgD levels were significantly higher than secretory IgD levels and IgD was present in greater concentrations in nasal secretions than in saliva both in the patients and the controls, with no difference between the two groups. Thus, the data of this study show that white serum and secretory IgM levels are elevated in children with selective IgA deficiency, serum and secretory IgD are present in normal concentrations, supporting the hypothesis of a compensatory increase in IgM but not in IgD in such patients. [ABSTRACT FROM AUTHOR]

Published
1983

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