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Biomedical subjects

M Duse

Publications and source records attributed to M Duse.

At least 55 records · Page 3Linked to original sources
PubMed1984

Serum IgG levels and complement activity in hypogammaglobulinaemic patients under substitution therapy.

Haemolytic activity of the classical and alternative pathways of complement as well as serum levels of C1q, Factor B, Factor H, C3, C4, C3d,g and IgG were determined in 15 hypogammaglobulinaemic patients on immunoglobulin replacement therapy. Alternative pathway activity (AP) and C1q were defective in the presence of low IgG levels and normalized on achievement of normal IgG levels; for both variables the correlation with serum IgG was highly significant. Classical pathway activity (CP), C3, C4 and Factor H serum levels were normal independently of IgG levels; Factor B and C3d, g serum levels were elevated in hypogammaglobulinaemic patients regardless of IgG levels. The present report supports the hypothesis that IgG serum levels influence complement function.

Adolescent↗
PubMed1983

Chronic idiopathic thrombocytopenia treated with immunoglobulin.

Twenty five children with chronic idiopathic thrombocytopenic purpura followed from 6-96 months in 7 Italian paediatric departments were treated with high dose immunoglobulin according to a multicentre protocol. Positive responses were observed in 20 of 25 patients (80%) and negative responses in 5 of 25 (20%). On previous steroid treatment 7 of 10 positive responders were steroid resistant and 13 of 15 were steroid dependent. Within four weeks of beginning treatment 16 of 20 patients (80%) relapsed, while 4 of 20 (20%) maintained normal platelet values after 4-12 months' follow up. Statistical analysis of the platelet count on day five of treatment enabled us to divide positive responders into three groups: good, intermediate, and poor. The possible mode of action and clinical application of high dose immunoglobulin are discussed.

Adolescent↗
PubMed1981

Defective Aspergillus killing by neutrophil leucocytes in a case of systemic aspergillosis.

A persistent defect of Aspergillus killing was observed in the neutrophils of a 6-year-old patient with a systemic A. fumigatus infection which was highly refractory to anti-mycotic therapy. Aspergillus phagocytosis in vitro was normal, but nearly 80% of the ingested organisms (versus 30% in the controls) survived intracellularly during the 2-hr assay period. The patient's neutrophils showed a subnormal frequency of nitroblue tetrazolium reduction and a subnormal hexose monophosphate shunt activation in response to phagocytosis. The metabolic responsiveness, however, was clearly superior to that of chronic granulomatous disease neutrophils tested for comparison. The immune status of the patient and the following properties of his neutrophils were found to be normal: random and chemotactic motility, killing of S. aureus and C. albicans, and the contents of several granula enzymes. Our findings suggest the existence of neutrophil factors or functions which are required for killing Aspergillus, but not S. aureus and C. albicans.

Aspergillosis↗
PubMed1980

Selective IgA deficiency: clinical and immunological evaluation of 50 pediatric patients.

Fifty children with IgA deficiency were folllowed for 1 to 4 years from 1975 to 1978. Thirty-five had complete deficiency of serum IgA (less than 2.5 IU/ml) and 15 partial deficiency (serum IgA below the 10th centile for age). Patients with another associated immunodeficiency, such as ataxia-telangiectasia, were not included. Most children with complete deficiency of IgA had recurrent respiratory and/or gastrointestinal infections, about half with onset in the first year of life, while partial deficiency of IgA has probably little if any importance for anti-infectious immunity but is important in the pathogenesis of atopy. Atopic diseases were frequent in both groups. Chromosomal abnormalities were found in 2 patients: trisomy 21 in one and in the other a ring chromosome 18. No important defects in cellular immunity were detected but some isolated, borderline abnormalities were often present.

Child↗
PubMed1980

Immunodeficiency in Down's syndrome: low levels of serum thymic factor in trisomic children.

The activity of thymus-dependent serum factor SF was significantly lower in 18 children wit Down's syndrome (DS) than in 14 matched controls. The percentage of circulating T lymphocytes forming E-rosettes was also low in DS. Together with the previous finding of immature of T lymphocytes in peripheral blood, the present data suggest that the basic immune defect of DS is failure in differentiation of peripheral post-thymic precursors to fully immunocompetent T lymphocytes resulting from lack of thymic hormonal factors.

Child, Preschool↗
PubMed1979

A rapid unfavorable outcome of Wegener's granulomatosis in early childhood.

Wegener's Granulomatosis was suspected in a 27-month-old female with a nodular, necrotizing lesion of the nose, diffuse subcutaneous nodules, and erythematous desquamation of the entire body. From 20 months of age on she had a purulent nasal discharge, recurrent infections of the upper and lower respiratory tract, a Coombs positive anemia, and enlargement of the spleen and liver. Treatment with azathioprine and corticosteroids produced transient improvement but three months later a dramatic relapse occurred. Cyclophosphamide was substituted for azathioprine but 10 days later the patient died and the autopsy confirmed the diagnosis of Wegener's Granulomatosis. The early age of onset of the disease may explain the unfavorable outcome, despite treatment with cytotoxic agents.

Adrenal Cortex Hormones↗
PubMed1978

Immunodeficiency in Down's syndrome. Titres of "natural" antibodies to E. coli and rabbit erythrocytes at different ages.

"Natural" antibody titres to E. coli O antigens of different serotypes and to rabbit red blood cells were determined in 86 subjects with Down's syndrome and 79 mentally retarded but chromosomally normal controls ranging in age from 10 months to 52 years. Subjects in the two groups were matched for sex, age and socio-environmental conditions. Titres of both antibodies, assessed by haemagglutination, were significantly lower in subjects with DS in the 1 to 5 year old group. E. coli antibodies transiently increased to normal values in subjects with DS during the second 5 years of life, thereafter rapidly declining to levels significantly lower than those observed in controls. The titres of antibodies to rabbit erythrocytes in subjects with Down's syndrome showed a more variable course transiently approaching normal values in the 7-10 year group and after 20 years of age. These data are interpreted as further evidence for the existence of a congenital immunodeficiency in Down's syndrome.

Adolescent↗
PubMed1977

Immunodeficiency in Down's syndrome: relationship between presence of human thyroglobulin antibodies and HBsAg carrier status.

The relationship between the presence of hepatitis B surface antigen (HBsAg) and antibodies to human thyroglobulin (HTgAb) has been studied in 110 subjects with Down's syndrome (DS) from 4 months to 50 years of age and in 122 controls carefully matched for sex, age and socio-environmental conditions. The overall percentage of HBsAg carriers was 22.7 in DS and 6.6 in controls and that of HTgAb-positive subjects was 41.8 in DS and 19.7 in controls. In DS the frequency of HTgAb-positive subjects was very high, even in the youngest age groups in which the percentage of HBsAg carriers was relatively low; the latter thereafter showed a marked increase with age. A positive association between the presence of HBsAg and HTgAb was found only in the oldest age group of DS subjects. It is thus concluded that in DS the high frequency of HTgAb cannot be attributed to chronic hepatitis B virus infection. On the contrary, the presence of HTgAb might well represent an early "marker" of immunodeficiency and increased susceptibility to infection with hepatitis B virus.

Adolescent↗
PubMedDate not supplied

Western blot technique in the serological evaluation of three LAV/HTLV III-infected Italian families.

In order to confirm suspected LAV/HTLV III infection, serological evaluation of patients is of utmost importance. ELISA is currently being employed on a large scale for screening, but like the immunofluorescence assay, it has a variable rate of possible non-specific positivity. On the other hand, the Western Blot (WB) technique can detect antibodies to different viral proteins. In this paper we are reporting the serological patterns of three LAV/HTLV III-infected families. In particular, their viral protein-specific antibody patterns are described. With the exception of one child, all the patients tested showed seropositivity in both ELISA and WB. In the one child mentioned above, ELISA and immunofluorescence positivity were due to non-specific binding. Two out of three children tested showed a close correlation between a severe clinical course and the absence of p25-specific IgM. In contrast, one child showing a switch from IgM to p25-specific IgG antibodies had a favorable clinical course. We observed a family in which vertical transmission of LAV/HTLV III from the mother to her neonate seems not to have happened; the child was seronegative and healthy at the age of one. At birth, this neonate had LAV/HTLV III-specific IgG corresponding to the mother's pattern, but it lacked viral-specific IgM. Its mother had transmitted the viral infection to her first child, who died of AIDS. Preliminary suggestions are made about the detection of different specific antibodies and clinical features; the utility of WB is emphasized.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

Primary immunodeficiencies: milestones in the history of pediatric immunology.

Pediatric immunology is a recent and important branch of pediatrics. Besides development of vaccines, the discovery of primary immunodeficiencies has represented a major contribution in the history of pediatric immunology. Characterization of these disorders as inborn errors of immunity has been crucial for understanding the functional organization and ontogeny of the immune system. Thus, progress in the study of immunodeficiency diseases has contributed to progress in pediatric immunology as a whole. As a result of these advances and a major biotechnology breakthrough, new therapeutic strategies have been devised. The benefits of these strategies extend far beyond the area of immunodeficiencies; they permit better care of infants and give new therapeutic approaches to other inherited disorders.

History, 20th Century↗