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Biomedical subjects

V Monafo

Publications and source records attributed to V Monafo.

At least 55 records · Page 3Linked to original sources
PubMed1982

Pulmonary hypertrophic osteoarthropathy in a child with late-onset agammaglobulinemia.

A case of hypertrophic osteoarthropathy is described in an 8-year-old child with late-onset agammaglobulinemia, bronchiectasis and clubbing of the fingers and toes. The child presented with pain of recent onset in the legs, ankles, and knees and a diagnosis of hypertrophic osteoarthropathy was made on the basis of the radiologic findings. Therefore the differential diagnosis of bone and joint pain in agammaglobulinemic subjects must take into account hypertrophic osteoarthropathy.

Agammaglobulinemia↗
PubMed1982

Different role of secretory IgA in the pathogenesis of RAST-positive and RAST-negative atopic dermatitis.

Secretory-IgA (SIgA) concentrations were determined in whole saliva, unstimulated or stimulated by lemon juice, of thirty-eight children with atopic dermatitis, which comprised three adolescents, sixteen with IgE detected by RAST to one or more common allergen and twenty-two without specific IgE by RAST. There were thirty healthy controls matched for age and sex. The mean amount of total IgE was significantly greater in the RAST-positive than in the RAST-negative group. The mean SIgA concentration in unstimulated saliva of the RAST-positive atopic dermatitis group was less than that of the RAST-negative atopic dermatitis group and control groups, through the mean concentrations of SIgA of stimulated saliva were not significantly different in the three groups. It is suggested that the pathogenesis of atopic dermatitis may differ in children with or without specific-IgE antibodies; in those who were RAST-positive deficient exclusion of allergen by the intestinal barrier contributed to the pathogenesis, but not in those who were RAST-negative.

Adolescent↗
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↗
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↗
PubMed1975

Rapidly reversible depression of delayed hypersensitivity in two children with visceral leishmaniasis.

Two cases of visceral leishmaniasis in children are described. In both "in vitro" lymphocyte response to mitogens and allogeneic cells was initially markedly depressed, but returned to normal within a few days after six days of treatment with N-methylglucamine antimonate; in one the candidin skin test reverted from negative to positive after treatment and in the other a circulating serum factor was found which strongly inhibited phytohemagglutinin--and mixed--responsiveness of normal lymphocytes, but disappeared after therapy.

Amino Sugars↗
PubMedDate not supplied

Infectious mononucleosis fifty years after the discovery of the Paul-Bunnell test.

The Paul-Bunnell test, now 50 years old, is still of fundamental importance in the diagnosis of infectious mononucleosis, even though various immunologic methods have been developed in clinical practice to identify constituents of the Epstein-Barr virus (EBV). The demonstration of sheep red cell agglutinins in infectious mononucleosis (Paul-Bunnell) was in fact the first observation of the presence of heterophil antibodies in this viral disease which was later shown to evoke a polyclonal antibody response to a wide spectrum of autoantigens as well. The selective tropism of EBV for B lymphocytes and the reactivity of T lymphocytes to these infected B lymphocytes are the pathophysiological elements characteristic of infectious mononucleosis, and are the reason for this massive antibody response.

Antibodies, Heterophile↗
PubMedDate not supplied

Cyproterone acetate treatment in precocious puberty.

Seven girls with precocious puberty, idiopathic in 6 and associated with the McCune Albright syndrome in 1, were treated with 70 mg/M2/die of cyproterone acetate (CPA) for 11 to 36 months. Before and during treatment clinical parameters (weight, height, bone age, height velocity, prediction of adult height and pubertal development) were evaluated and plasma hormone assays in basal conditions (LH, FSH, 17 beta-estradiol, progesterone, testosterone, PRL, ACTH and cortisol) and after stimulation (LH, FSH, cortisol, GH) were carried out to assess the efficacy and eventual side effects of CPA. The regression observed in the clinical signs of puberty was considered satisfactory and the increase observed in the developmental quotient indicated an improved prognosis for adult height. No symptoms of adrenal insufficiency were observed but an adrenal suppressive effect of CPA was evident from the response to insulin hypoglycemia observed in 4 patients.

Child↗
PubMedDate not supplied

Transient beneficial effect of GH replacement therapy and topical GH application on skin ulcers in a boy with prolidase deficiency.

A diagnostic examination for short stature in a boy with chronic ulcers of the feet due to prolidase deficiency, a rare disorder associated with intractable ulcers of the skin, led to the diagnosis of growth hormone (GH) deficiency. Replacement treatment with r-hGH associated with the topical application of a GH-containing ointment when the boy was 13 years old resulted in complete but transitory healing of the ulcers, which can probably be attributed to the growth-promoting effects of GH on dermal connective tissue.

Adolescent↗
PubMedDate not supplied

Prolidase deficiency: biochemical study of erythrocyte and skin fibroblast prolidase activity in Italian patients.

BACKGROUND AND METHODS: Prolidase deficiency (PD), a rare, autosomally inherited disorder causing iminodipeptiduria is associated with a number of clinical manifestations, the principle feature being chronic skin ulceration. The enzyme prolidase cleaves iminodipeptides containing C-terminal prolyl or hydroxyprolyl residues and is important in the final stages of protein catabolism. We report clinical and biochemical findings in 8 Italian patients with proven prolidase deficiency. There was considerable heterogeneity in age at onset of symptoms (varying from 3-17 years), mental retardation and clinical manifestations (asymptomless to very severe). Prolidase activity was determined in hemolysates of patient erythrocytes and cultured dermal fibroblasts. RESULTS: Prolidase activity was found to be deficient, especially against gly-pro. Erythrocyte and fibroblast enzyme was also separated into two forms, a major isoform (I) and a minor one (II) by fast protein liquid chromatography, and activity against different iminodipeptide substrates was tested. Isoform I activity was markedly reduced in all patients as compared to normal controls, while isoform II activity appeared to be unaltered. CONCLUSIONS: We were unable to find any correlation between degree of enzyme activity loss and severity of symptoms.

Adolescent↗
PubMedDate not supplied

[Granulocyte disorders].

The various "in vitro" tests for evaluating polymorphonuclear leucocyte function in children with recurrent infections are described and the different clinical conditions caused or accompanied by defects in polymorphonuclear function are summarized briefly. The necessity of correct interpretation of the results of the laboratory tests used in the diagnostic evaluation of patients with suspected immunodeficiency is stressed.

Agranulocytosis↗