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M Purpura

Publications and source records attributed to M Purpura.

At least 19 recordsLinked to original sources
PubMed1993

Down's syndrome and celiac disease: the prevalence of high IgA-antigliadin antibodies and HLA-DR and DQ antigens in trisomy 21.

Patients with Down's syndrome (DS) or celiac disease (CD) have altered immune systems. Autoimmune diseases have been described in both conditions; the coexistence of DS and CD has been occasionally reported, but a clear relationship has not been definitely established. In this study we determined IgA antigliadin antibodies (IgA-AGA) in 155 children with DS, and the results were compared with those of the control groups formed by 320 children affected by upper-respiratory tract infections and 115 children with gastrointestinal symptoms but with normal jejunal mucosa. High IgA-AGA levels were found in 26% of DS patients, in 1% of the first control group and in 10% of the second control group. Such differences are statistically significant. Twenty-one DS patients with high IgA-AGA levels and gastrointestinal symptoms underwent jejunal biopsy, and total villous atrophy was found in seven of them (33.33%). HLA-DR and -DQ antigens were also determined in 75 DS patients (20 with high and 55 with normal IgA-AGA levels), and the percentages of the different phenotypes were compared in the two groups and with those of a control group. No statistically significant difference was found, but DR3, DR7, and DQ2 alleles were always present in DS patients with jejunal atrophy. Our study confirms the data reported in the literature about higher levels of IgA-AGA in DS patients and the relatively high incidence of CD in this group of patients.

Adolescent↗
PubMed1992

HLA haplotypes and susceptibility to Graves' disease.

Graves' disease is a polygenic disease in which the HLA cluster could play a role. The purpose of our study is to identify HLA haplotypes in a family with closely related susceptibility to Graves' disease and foresee the risk of disease in the youngest daughter. The family studied had included the father (47 years), mother (46 years) and 3 daughters (18, 17 and 13 years). The mother and 2 eldest daughters were affected by Graves' disease. HLA-A, -B, -C, -DR and -DQ were performed with standard microlymphotoxicity techniques. A mother's role in passing susceptibility to Graves' disease to daughters is undisputed; it seems to be due to the B35 HLA allele. Also, the third daughter (at 15 years) has an HLA B35 allele, and actually has an incipient humoral hyperthyroidism.

Adolescent↗
PubMed1992

[Chronic exposure to inhalation anesthetics and immunity].

The existence of a professional disease caused by exposure to general anesthetics has been reported by a number of studies, but opinions still differ as to the effective implications of this type of exposure. The aim of the present study was to analyse alterations in cellular and humoral immunity in anesthetists regularly exposed to general anesthetics. Regression analysis showed that IgA and NK lymphocytes were significantly increased whereas total T lymphocytes reduced in parallel with age and length of service. It can therefore be supposed that the increase in IgA might suggest chronic liver disease, while alterations in the lymphocyte populations, even if closely associated to chronic exposure leading to the hypothesis of a cause-and-effect relationship, do not alter immune system functions since they are similar to those that occur with ageing.

Adult↗
PubMed1991

Protection against malaria morbidity: near-fixation of the alpha-thalassemia gene in a Nepalese population.

We have previously reported that the Tharu people of the Terai region in southern Nepal have an incidence of malaria about sevenfold lower than that of synpatric non-Tharu people. In order to find out whether this marked resistance against malaria has a genetic basis, we have now determined in these populations the prevalence of candidate protective genes and have performed in-vitro cultures of Plasmodium falciparum in both Tharu and non-Tharu red cells. We have found significant but relatively low and variable frequencies of beta-thal, beta S, G6PD (-), and Duffy (a-b-) in different parts of the Terai region. The average in-vitro rate of invasion and of parasite multiplication did not differ significantly in red cells from Tharus versus those from non-Tharu controls. By contrast, the frequency of alpha-thalassemia is uniformly high in Tharus, with the majority of them having the homozygous alpha-/alpha-genotype and an overall alpha-thal gene (alpha-) frequency of .8. We suggest that holoendemic malaria has caused preferential survival of subjects with alpha-thal and that this genetic factor has enabled the Tharus as a population to survive for centuries in a malaria-holoendemic area. From our data we estimate that the alpha-thal homozygous state decreases morbidity from malaria by about 10-fold. This is an example of selection evolution toward fixation of an otherwise abnormal gene.

Animals↗
PubMed1990

Genetic polymorphisms in the Croatian ethno-linguistic minority of Italy.

Phenotype and gene frequencies of twelve genetic markers (ABO, RH, MNS, ACP1, ESD, PGD, PGM1, PGM2, HB, ALB, CP and HP) of the three Croatian communities (Acquaviva Collecroce, Montemitro and S. Felice del Molise) living in Southern Italy are reported. From the comparison with the surrounding Italian population and with Balkan populations, some instances of still incomplete genetic admixture can be inferred (ABO*A and O, ESD, PGD and PGM1).

Adolescent↗
PubMed1989

HLA-alloimmunization in pregnancy.

The authors analyzed the correlations between the presence of anti-HLA antibodies and fetal neonatal problems. There are specific data in the literature on the protective effect of anti-HLA antibodies against abortion. Nevertheless our study shows a significant correlation between their presence and perinatal problems.

Abortion, Habitual↗
PubMed1986

HLA-DR3 antigen linkage in patients with hypertrophic obstructive cardiomyopathy.

In order to investigate if genetic factors could be involved in the pathogenesis of hypertrophic obstructive cardiomyopathy, we determined HLA-A, HLA-B, HLA-C, and HLA-DR specificities in 12 Italian patients affected with the disease and in healthy family members of one of them. HLA-DR3 was found in 50% of patients as compared to 17.1% of normal control subjects (p = 0.023, relative risk = 4.82). The two relatives also had HLA-DR3 antigen and, in addition, showed equivocal signs of hypertrophy at echocardiographic examination. Thus hypertrophic obstructive cardiomyopathy is associated with genes in the HLA-DR region, and immunogenetic factors could be involved in the pathogenesis of the disease. Furthermore, the minimal target organ abnormalities in "healthy" relatives could represent a subclinical stage of the disease.

Adult↗
PubMed1986

Standard conditioning regimen and T-depleted donor bone marrow for transplantation in chronic myeloid leukemia.

Between January 1984 to June 1985, 18 Ph1 positive chronic myeloid leukemia (CML) patients in chronic phase (CP) underwent allogeneic bone marrow transplantation (BMT) from HLA identical and MLC negative siblings. The median age was 32.5 yr and median disease duration of CML at time of BMT was 19.3 months. The pretransplant conditioning regimen consisted of cyclophosphamide (CTX) (120 mg/kg) and 10.20 Gy total body irradiation (TBI) at 6 doses of 1.7 Gy each, administered in 3 daily fractions over 2 days at a dose rate of 15-20 cGy/min. To prevent graft-vs-host disease (GvHD) we used methotrexate (MTX) in one patient and cyclosporin-A (CYA) in the other 17 patients. In addition to CYA, given until day +365, 10 patients received donor marrow depleted of T cells with CAMPATH-1. The residual marrow lymphocytes were always less than 1%. The rate of engraftment was significantly correlated with the number of nucleated cells infused. Neither GvHD nor graft failure were observed among CAMPATH-1 patients. In this group one cytogenetic and one hematologic relapse occurred. The overall actuarial survival at 24 months is 78%. Of the 10 patients treated with donor marrow depleted of T cells, 9 are alive after a median follow-up of 9 months (range 5-18), with an actuarial survival of 90%. Of the other 8 patients transplanted with untreated marrow, 5 are alive after a median follow-up of 19.3 months (range 3.7-24) and the actuarial survival is 63.8%. This pilot study seems to demonstrate that T-cell depletion of donor bone marrow with CAMPATH-1 is effective to prevent GvHD, while the risk of graft failure can be avoided using a "standard" conditioning regimen including a fractionated TBI with a fast dose rate and a prolonged administration of CYA at the maximum tolerable dosage. While the high frequency of relapses suggests the employ of more aggressive anti-leukemic conditioning regimens in CAMPATH-1 treated marrow recipients.

Adolescent↗
PubMed1985

Discordant sex in one of three monozygotic triplets.

A case is reported of monozygotic triplets, discordant for phenotypic sex, in which the female presented at birth with the features of Turner's syndrome. Chromosomal analyses showed homogeneous 46,XY karyotypes in the lymphocytes of the three sibs, while a 45,X non-mosaic chromosome constitution was detected in skin fibroblasts of the female triplet. It is suggested that mitotic non-disjunction or anaphase lag occurring early during embryonic development accounted for the occurrence of monosomy X in one cell line of the affected triplet. Previous observations of monozygotic twin pairs discordant for chromosome constitutions are reviewed.

Blood Grouping and Crossmatching↗
PubMed1980

Blood groups and histocompatibility antigens in habitual abortion.

Forty-six couples with at least two consecutive abortions were examined. The morphological and the functional clinical check-ups were constantly negative. In all the couples a karyotype analysis was carried out including an investigation of C and/or G bands. The phenotypes of ABO, Rh, MNSs and HLA-systems were also determined. No significant difference was observed in the distribution of ABO phenotypes between males and females, or between subjects with abortions and controls. Regarding the Rh system, the most important findings are the absence of phenotypes with the E allele in double dose, the reduction of the frequency of the CCDee phenotype and the increase in the frequency of the ccDEe phenotype. Concerning MNSs system, an increase in the frequency of the phenotypes with the S allele in double dose is observed. Females with habitual abortions show a higher incidence of Bw35 as compared both to males and to the controls. No significant differences were observed for other antigens. The persistence of a genetic disequilibrium both in the Rh and the MNSs systems suggests that the selection might act against certain antigenic combinations, independently from the state of materno-foetal compatibility. Though preliminary, our data seem to give some support to this hypothesis. They also suggest that Bw35 antigen may be important in human reproduction.

Abortion, Habitual↗
PubMed1979

Auto-immune haemolytic anaemia in childhood: follow-up in 29 cases.

We describe 29 children with auto-immune haemolytic anaemia (AIHA). The patients could be followed up for long periods of time. The auto-antibodies, responsible for the AIHA, are described as well as the associated diseases that could be diagnosed, the clinical course and some clinical aspects of special importance. Finally, the treatment given to these patients and its results are discussed.

Anemia, Hemolytic, Autoimmune↗