Biomedical subjects
Publications and source records attributed to F Ersoy.
The clinical and immunologic features of six patients with X-linked agammaglobulinemia (XLA) are presented. The most common presenting manifestations were respiratory and gastrointestinal tract infections. On admittance to the hospital, one patient had a history of recurrent meningitis, another had a dermatomyositis-like syndrome, and still another had a history of recurrent arthritis.
A four-year-old girl with hyper-IgE syndrome is presented. She had a coarse facial appearance, pruritic dermatitis, recurrent skin abscesses, pulmonary infection, spontaneous bone fractures, and an elevated serum IgE concentration. She has been treated with cimetidine, ascorbic acid and trimethoprim-sulfamethoxazole for the last two years and there has been no evidence of a severe infection.
Eight patients with Hyper-IgM syndrome were subjected to clinical and immunological evaluation. There were seven males and one female. All the patients had recurrent pyogenic infections; one had lymphoid hyperplasia with centrally necrotic granulomas, and one had gingivitis with neutropenia. Isohemagglutinin titers were either high or normal in all the patients and five had group 0 blood. The percentage of IgM-bearing cells were normal in five patients. The percentage of T cells were normal in all the patients, helper T cells were decreased in two patients, and suppressor T cells were increased in four patients. These results suggest that at least in some patients, the imbalances of T cell subsets may play a role in the pathogenesis of the disease rather than it being attributed to an intrinsic B cell defect.
The clinical and immunological features of 14 patients including 12 with selective and two with partial IgA deficiency are presented. One patient was asymptomatic, six patients had allergic diseases, three patients had hematologic disorders and the remaining patients had chronic-recurrent infections. The IgG and IgM serum concentrations were high in four and two patients, respectively. Delayed hypersensitivity skin tests were applied to seven patients, and were found positive in all. E-rosette forming cells were within the normal range. In vitro lymphoblastic transformation with PHA was normal in all the patients, except for one. Two patients had no sIgA+ B cells. The percentage of CD+4 T cells was decreased in one patient and the percentage of CD+8 T cells was increased in another, both of whom had a low CD4/CD8 ratio. The heterogeneity of clinical and immunological features in these patients suggests that various mechanisms may be responsible for the immunopathogenesis of IgA deficiency.
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We researched complement activation of fifteen patients who had open heart surgery and on ten patients who had closed heart surgery. Our results showed that the complement system was partially activated by the anaesthesia and partially by tissue damage. This activation was aggravated when plasma contacted the pump-oxygenator system, continued in the intensive-care unit and became normal in the 24th hour after the operation. Complement activation occurred both via the alternative and classical pathways but the alternative pathway was activated more than the classical with increase in bypass time. Pulmonary sequestration of leucocytes which occurred due to the complement activation and the complement derived inflammatory mediators could have contributed to the pathogenesis of the post-pump syndrome.
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Bare lymphocyte syndrome (BLS) is a rare disorder characterized by deficient expression of human leukocyte antigens (HLA antigens) and combined immunodeficiency to various degrees. Recurrent severe infections especially due to opportunistic organisms are common. Here, we present two patients with BLS who lack both class I and II antigens (Type III). They had the typical clinical and immunologic findings of BLS. The first patient showed marked improvement in pulmonary symptoms resulting from cytomegalovirus infection by means of gancyclovir treatment. However, intramuscular injections of interferon-alpha (IFN-alpha) had no beneficial effect in either the expression of HLA antigens or the clinical status. The second patient died of septicemia while being prepared for bone marrow transplantation.
A 2-month-old SCID patient with mesangial sclerosis of the kidney is presented. Although the ADA status of the patient is unknown, the parents' RBC-ADA levels seemed to be in the heterozygote range.
Immunoglobulin G (IgG) subclass levels were determined in 393 serum samples of 329 healthy children from birth to 16 years of age, 24 healthy adults and 20 pairs of mother-cord blood by radial immunodiffusion. Age-normal percentile values for IgG1, IgG2 and IgG3 were calculated for age-groups up to 16 years and for adults. The broad range of IgG4 values in children did not permit calculation of reference values.
The value of IgM-enriched immunoglobulin therapy in 44 preterm infants with neonatal sepsis was evaluated in a prospective randomized study. All infants received antibiotic therapy and fresh plasma and/or whole blood transfusions. Twenty randomly-chosen infants were allocated to receive 5 ml/kg/d of IgM-enriched immunoglobulin intravenously for three days. Although the mortality rate in preterm infants whose gestational ages were 31-34 weeks in the immunotherapy group was slightly lower than in the control group, the general mortality rate from sepsis in the control group (9/24) and in the immunotherapy group (6/20) showed no statistically significant difference (37.5% vs 30.0%, p < 0.05).