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Biomedical subjects

L J Dooren

Publications and source records attributed to L J Dooren.

At least 19 recordsLinked to original sources
PubMed1991

Expression of HLA-DQ antigens in the small-intestinal mucosa of patients with coeliac disease.

Studies at the DNA and product level of B-cell lines of coeliac patients have shown a strong association between coeliac disease and the HLA-DQ alpha 2.3 and HLA-DQ beta 2.7 alleles. The monoclonal antisera SFR20-DQ alpha 5 and XIII-358.4, which specifically react with HLA-DQ alpha 2.3 and with HLA-DQ beta 2.3 and -DQ beta 2.7, respectively, have been used to detect the expression of these specificities in the small-intestinal mucosa of 7 coeliac patients and 11 non-coeliac persons. An immunoperoxidase technique on frozen tissue sections of jejunal biopsy specimens was used. Positive specimens showed immunoperoxidase staining of lymphocytes and histiocytes in the lamina propria. The epithelial cells showed no immunoperoxidase staining. Positive results at the intestinal level correlated with the HLA typing of the patients and controls. The distribution found for the HLA-DQ alleles in the intestinal mucosa makes the role of a HLA-DQ alpha/beta dimer as gliadin receptor at the epithelial cell less probable, but it is compatible with the hypothesis that these DQ molecules are involved in the regulation of the intestinal immune response to gluten.

Adult↗
PubMed1990

Prevention of infection and graft-versus-host disease by suppression of intestinal microflora in children treated with allogeneic bone marrow transplantation.

The effect of suppression with antimicrobial agents of the intestinal microflora of paediatric bone marrow graft recipients on severe bacterial and fungal infections and on moderate to severe acute graft-versus-host disease was studied retrospectively. Data on 65 cases of bone marrow transplantation for either severe bone marrow failure or leukaemia, performed in a strict protective environment with either complete or selective gastrointestinal decontamination, were evaluated. All bone marrow grafts were from HLA-identical siblings and were not depleted of T-lymphocytes. Twenty percent of the recipients had one or more episodes of septicaemia during the granulocytopenic period after transplantation, mostly due to gram-positive bacteria. Only five children died due to infection, in each case caused by a microorganism originating from the endogenous flora. Complete gastrointestinal decontamination was superior to selective gastrointestinal decontamination in preventing infectious complications (p less than 0.001). The same was the case for the prevention of acute graft-versus-host disease of grade II or higher, which was observed in 7 of 40 (17.5%) completely decontaminated children versus 9 of 18 (50%) selectively decontaminated children evaluable for graft-versus-host disease (p less than 0.01). It is concluded that complete gastrointestinal decontamination in a strict protective environment is a feasible and very effective method for preventing severe infections and acute graft-versus-host disease after allogeneic bone marrow transplantation in children and adolescents; it resulted in a low transplantation-related mortality of 26% and a good quality of survival in 69% of the graft recipients.

Adolescent↗
PubMed1988

[Combined immunologic deficiency].

In the last 20 years 32 patients with severe combined immunodeficiency (SCID) were treated at our department. The clinical and immunological findings in these patients are presented. In connection with these patients, the recent WHO classification on combined immunodeficiency diseases and the pathogenesis in several forms of SCID, as far as is known, are discussed. SCID appears to represent a very heterogeneous group of disorders, which is affirmed by the findings in several of our patients. At this moment bone marrow transplantation (BMT) is the only way to cure patients with SCID. Without this treatment the prognosis of these patients is very poor. After BMT complete recovery is achieved for the majority of patients, even in the absence of a genotypically HLA identical donor. Besides the therapeutic aspects of SCID the recent developments with regard to carrier-detection and antenatal diagnosis are briefly discussed.

Blood Transfusion↗
PubMed1987

Natural killer (NK)-cell activity in sorted subsets of peripheral blood mononuclear cells from patients with severe combined immunodeficiency.

Natural killer-cell activity for K562 target cells was measured in 13 patients with severe combined immunodeficiency before bone marrow transplantation. Both unseparated peripheral blood mononuclear cells and sorted cell subsets (B73.1 positive, B73.1 negative, OKT3 positive, OKT3 negative) were tested. Heterogeneity of natural killer-cell activity was observed, the level of which did not correlate with the usual subdivision of severe combined immunodeficiency, as defined by a scientific group on immunodeficiency for the WHO. In those patients in whom natural killer-cell activity was observed in unseparated cells, testing of this function in sorted cell subsets revealed some unexpected findings. In three of four patients, B73.1-negative cells showed remarkable natural killer-cell activity. In addition, in one of these patients, no activity was observed in the B73.1-positive cells.

Antibodies, Monoclonal↗
PubMed1987

Correction of lysosomal enzyme deficiency in various organs of beta-glucuronidase-deficient mice by allogeneic bone marrow transplantation.

The correction of lysosomal enzyme deficiency was investigated for various organs of beta-glucuronidase-deficient C3H/Rij mice after allogeneic bone marrow transplantation from an enzymatically normal donor strain (C57BL/Rij). In the hemopoietic organs, the enzyme level increased to levels found in donor mice. In lung, kidney, liver, and peripheral nervous tissue, a significant increase in enzyme activity was seen to levels intermediate between those of donor and recipient. Increased enzyme activity was maintained throughout the observation period of 150 days. In skeletal muscle tissue, enzyme levels tended to be higher in recipient mice, but this increase was not significant for all data points. Bone marrow transplantation failed to significantly affect enzyme activity in central nervous system tissue. These data suggest that beneficial effects expected from bone marrow transplantation for lysosomal enzyme deficiencies depend on the type of tissue involved in the disease. In diseases severely affecting the central nervous system, cure may not be expected from bone marrow transplantation alone, whereas in diseases with only minimal central nervous system involvement, alleviation or prevention of clinical symptoms may occur.

Animals↗
PubMed1987

Transient elevation of serum IgE after allogeneic bone-marrow transplantation.

Serum IgE levels were followed longitudinally twice a week for up to 100 days in 60 children treated for leukemia, severe aplastic anemia or severe combined immunodeficiency: 52 underwent allogeneic bone-marrow transplantation and 8 immunosuppressive treatment. In 55 of 58 treatment periods which could be analyzed (95%), a transient sharp increase of serum IgE was detected, irrespective of the initial diagnosis and mode of treatment. A second IgE peak was recorded in 16% of evaluable treatment periods. In the transplanted leukemia and aplastic anemia patients, the rise of serum IgE levels occurred at the same time, i.e. at a mean of 14 days after transplantation; it occurred significantly later in children grafted for severe combined immunodeficiency. In children who received immunosuppression for the treatment of severe aplastic anemia, IgE elevations were always seen within 2 weeks after institution of therapy. No relation was found between either the occurrence of clinically acute graft-versus-host disease or infections after treatment, and the time of onset of IgE elevations. It is suggested that the phenomenon of IgE peaks in the population of patients investigated was due to disturbance of T-cell regulation, i.e. a temporary impairment of T-suppressor cell activity.

Adolescent↗
PubMed1987

[AIDS in children].

Infection with the human immunodeficiency virus type I in children causes variable clinical symptoms. It can lead to asymptomatic disease as well as to acquired immunodeficiency syndrome. In this review we describe the clinical picture, the immunological abnormalities, the mode of transmission, and the preventive measures in case of HIV-I infection.

Acquired Immunodeficiency Syndrome↗
PubMed1979

Cellular immunity in patients with the Wiskott-Aldrich syndrome before and after administration of transfer factor: a follow-up study.

Four patients with Wiskott-Aldrich syndrome were treated with transfer factor (TF), in an attempt to improve their clinical condition. Before and during treatment, cellular reactivity was followed in vivo (delayed type skin reactivity) and in vitro (lymphocyte transformation). In all patients positive skin reactions were occasionally observed during TF treatment. The lymphocyte reactivity in vitro to phytohaemagglutinin (PHA) was slightly diminished, the responding capacity of the mixed lymphocyte culture (MLC) severely impaired and the response to bacterial, viral and fungal antigens absent before and during TF treatment. No clinical improvement was observed after large doses of TF. No correlation between skin test reversal and TF administration was found, and lymphocyte transformation in vitro did not improve. Subsequently, a double blind trial, in which we compared the effect of TF and placebo, was carried out in these patients, which revealed no effect of TF therapy. It is concluded that the results of treatment with TF in these patients are highly controversial.

Clinical Trials as Topic↗
PubMed1977

Localized scleroderma-like lesions after bone marrow transplantation in man. A chronic graft versus host reaction.

Localized scleroderma-like skin lesions which developed in two children, from 8 to 10 months after successful bone marrow transplantation for aplastic anaemia, showed histopathological features resembling those of scleroderma. This finding, like the animal models described in the literature, provides additional support for the auto-immune nature of scleroderma.

Anemia, Aplastic↗
PubMed1975

IgG - subclass abnormalities in primary immunodeficiency diseases.

IgG-subclass levels were determined in the sera of 45 patients suffering from various types of immunodeficiency disease. Imbalances of IgG-subclass concentrations were found in 25 cases. IgG-4 levels were most frequently more depressed than the levels of the other subclasses. The percentage distribution of IgG-subclasses was normal in the remaining 20 sera. In a patient with autosomal recessive alymphocytic agammaglobulinemia, subclass concentrations drastically increased after bone marrow transplantion showing transient peaks of restricted heterogeneity. Normal IgG-subclass levels were reached at the end of the observation period.

Adult↗