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Biomedical subjects

F Ersoy

Publications and source records attributed to F Ersoy.

At least 55 records · Page 3Linked to original sources

Selective IgA deficiency with unusual features: development of common variable immunodeficiency, Sjögren's syndrome, autoimmune hemolytic anemia and immune thrombocytopenic purpura.

We report on a girl with selective IgA deficiency and persistently low complement component 4 (C4) levels compatible with heterozygous C4 deficiency. Deterioration of her serum immunoglobulin levels and transition to common variable immunodeficiency were observed within a 5 year follow-up. She also developed Sjögren's syndrome, autoimmune hemolytic anemia and immune thrombocytopenic purpura. While these abnormalities have been described before in various combinations, to our knowledge, they have not been reported in a single individual.

Anemia, Hemolytic, Autoimmune↗

The gingival crevicular fluid interleukin-1 beta and tumour necrosis factor-alpha levels in patients with rapidly progressive periodontitis.

Cytokines are believed to play an important role in the pathogenesis of periodontal diseases. In the present study, gingival crevicular fluid (GCF) levels of two important cytokines, interleukin 1-beta (IL-1 beta) and tumour necrosis factor-alpha (TNF-alpha) and, in addition, serum IL-1 beta levels, were determined in patients with severe and rapid periodontal breakdown by use of ELISA. While IL-1 beta was detected in all of the GCF samples studied, TNF-alpha could only be detected in about half the samples. The mean GCF IL-1 beta level was 38.45 +/- 13.99 pg/mL, and the mean TNF-alpha level was 3.20 +/- 1.39 pg/mL, respectively. The GCF IL-1 beta levels also presented a strong positive correlation with the mean pocket depths. Although weak, both of the cytokines also presented correlations with the presence of bleeding on probing. Additionally GCF samples contained increased IL-1 beta levels when compared with the serum samples suggesting local production mechanisms. The findings of the present study suggest that these cytokines may be involved in the pathogenesis of periodontal diseases (IL-1 beta being more significant), and also may help in defining the active phase of periodontal breakdown.

Adult↗

Oral findings, treatment and follow-up of a case with major aphthous stomatitis (Sutton's disease).

Major aphthous stomatitis (Sutton's disease) is a clinical variant of recurrent aphthous stomatitis, which is noted for its high morbidity. Since the etiology of the disease is not clear, many therapies have been attempted. However, the controversial results hinder the adoption of a single mode of management. We present a 13-year-old boy with Sutton's disease, who was successfully treated with a combination of burst systemic prednisone (1 mg/kg/day for five days, thereafter half dose on alternate days for one week) and topical triamcinolone (four rinses a day). He continued the mouth rinses with the same interval. At the end of the first month, significant healing was observed and gradual tapering was recommended on the condition that the ulcers were well-controlled. The maintenance of steroid rinse once a day provided a symptom-free period of one year. Neither any withdrawal signs nor side affects were observed. Therefore, we think that this regimen should be considered as the treatment of choice in Sutton's disease along with a close follow-up even in childhood.

Adolescent↗

Periodical gingival bleeding as a presenting symptom of periodontitis due to underlying cyclic neutropenia. Case report.

A 13-year-old boy presenting with spontaneous and periodical gingival bleeding accompanied by fever was thoroughly examined in order to determine the underlying aetiology of the gingival bleeding. Following repeated blood tests, the patient was diagnosed as having cyclic neutropenia. Thus, it was suggested, gingival bleeding, especially when spontaneous and periodical, should be carefully investigated to eliminate the possibility of cyclic neutropenia.

Adolescent↗

Immunoglobulin A levels in serum and saliva of patients treated with phenytoin.

A study was conducted to compare IgA levels in serum and saliva obtained from phenytoin-treated epileptic patients (PHT-TEPs) and a control group and to examine the correlation between IgA levels and clinical parameters. Eighteen epileptic patients treated with phenytoin and 18 periodontally healthy individuals with no systemic disease were included in the study. Clinical parameters were recorded, and samples of serum and saliva were obtained from each individual. IgA levels were determined by the radial immunodiffusion technique. Serum IgA levels were significantly lower in PHT-TEPs. No difference was found in salivary IgA levels between the PHT-TEP and control groups. Weak negative correlations were found between serum IgA level and gingival overgrowth index (GOI), and between salivary IgA level and GOI. None of the clinical parameters was significantly correlated with IgA level in the PHT-TEP group.

Adolescent↗

Myeloperoxidase activity in peripheral blood, neutrophil crevicular fluid and whole saliva of patients with periodontal disease.

Mean levels of myeloperoxidase (MPO) activity were determined in samples of gingival crevicular fluid (GCF), whole saliva and peripheral blood neutrophils from patients with rapidly progressive periodontitis (RPP) and adult periodontitis (AP) using a spectrophotometric method. The mean neutrophil MPO activity in the RPP group was 563.1 +/- 137.05 U/l x 10(6)/ml, that in the AP group was 483.3 +/- 88.81 U/l x 10(6)/ml, and that in the control group was 220.6 +/- 26.7 U/l x 10(6)/ml. The mean GCF MPO activity in the RPP group was 15.13 +/- 2.34 U/mg, which was significantly higher than in the other two groups. The mean whole saliva MPO activity in the RPP group was 0.14 +/- 0.04 U/ml, that in the AP group was 0.11 +/- 0.02 U/ml, and that in the control group was 0.05 +/- 0.06 U/ml. MPO activity detected in the samples was significantly increased in the patient groups when compared to the healthy subjects. The highest MPO activity was found in the RPP group. The present findings suggest a relationship between MPO activity and the pattern and severity of periodontal breakdown. Also the increased MPO activity in periodontally diseased patients can be attributed to the increased number of neutrophils, the degranulation of these cells and also their hyperactive state in the presence of chronic antigenic stimulation.

Adolescent↗

Neutrophil chemotaxis and periodontal status in Down's syndrome patients.

Along with clinical parameters, chemotaxis and random migration of neutrophils were evaluated in 15 patients with Down's syndrome (DS) and 15 healthy subjects. Signs of more severe gingival inflammation were present in the DS group. The random migration and chemotaxis of neutrophils were significantly decreased in comparison with the control group. In DS, the pathological status was attributed to these impaired host defense factors besides the existing bacterial plaque.

Adolescent↗

Complement component deficiencies and infection: C5, C8 and C3 deficiencies in three families.

Three families are described with complement component deficiencies. In one family, five children had C5 deficiency; in a second family, two children had C8 deficiency and one child in a third family had C3 deficiency. The index cases were identified during screening of patients with recurrent pyogenic infections, recurrent meningitis and meningococcaemia. Two of the five C5 deficient patients had recurrent meningitis and meningococcaemia, two had recurrent respiratory tract infections and otitis and one was healthy. One of the C8 deficient patients had meningitis, meningococcaemia and pneumonia, whereas his sibling with the same deficiency was healthy. The patient with C3 deficiency had four episodes of meningitis and recurrent otitis.

Child↗

Ataxia-telangiectasia: linkage analysis of chromosome 11q22-23 markers in Turkish families.

To further pinpoint the location of the genes for ataxia-telangiectasia on the long arm of chromosome 11, we performed linkage analysis and analysis of recombinants of genetic haplotypes on 14 Turkish families with ataxia-telangiectasia, 12 of which were consanguineous. These studies used more than 25 polymorphic genetic markers spanning a region of the long arm of chromosome 11 that is larger than 50 cM. Seven markers gave significant LOD scores to AT: CJ5, DRD2, CJ208, S144, CD3E, PBGD, and S147, as did haplotypes created with pairs of markers DRD2/CJ5 and S144/CJ208, giving recombination fractions (theta) of 0.00, 0.00, 0.05, 0.08, 0.03, 0.09, 0.07, 0.00, and 0.06, respectively. Monte Carlo analysis of these 14 Turkish families indicated the best location for a single AT gene to be within a 6 cM sex-averaged (3 cM male-specific) interval defined by STMY and CJ77; this was three times more likely than the next most likely location (peak III) at the DRD2 locus. The analysis also revealed a peak (peak II) between S147 and S133, which may represent the complementation group D gene. Recombinant analysis of haplotypes also localized an AT locus to the STMY-CJ77 interval. Taken together, these results suggest that at least two distinct AT loci exist (ATA and ATD) at 11q22-23, with perhaps a third locus, ATC, located very near to the ATA gene. This genetic heterogeneity further complicates plans to isolate the major ATA and ATC genes and to begin identifying AT carriers in the general population.

Ataxia Telangiectasia↗

Clinical and immunological characteristics of patients with rheumatoid arthritis and periodontal disease.

Various clinical and immunological parameters were determined in patients with RA and patients with adult periodontitis. There were no significant differences between the two groups with regard to the number of missing teeth, although pocket depths and gingival inflammation scores were significantly increased in the periodontitis group (p less than 0.05). Salivary IgM levels were below the minimum detectable level in both groups, and no significant differences were evident between the two groups with regard to salivary IgG and IgA levels. However, serum IgG, IgA and IgM levels in RA patients were significantly higher than in periodontitis patients (p less than 0.05), indicating enhanced humoral immunity. The present findings suggest that RA patients are not a risk group for advanced periodontal problems in comparison with age- and sex-matched systemically healthy controls.

Adult↗

The incidence of non-classical 21-hydroxylase deficiency in hirsute adolescent girls.

Non-classical adrenal hyperplasia due to 21-hydroxylase enzyme deficiency (NC21OHD) causes hirsutism, acne and menstrual irregularities in women. Clinically, patients with NC21OHD may be indistinguishable from other hyperandrogenic women, as they all present with similar symptoms. An elevated response of cortisol precursors like 17 alpha-hydroxyprogesterone (17-OHP) to ACTH stimulation is a valuable diagnostic criteria. In this study, 32 hirsute adolescent girls, aged 13-19 years, underwent i.v. adrenocorticotrophic hormone (ACTH) (Synacthen 0.25 mg) stimulation test. The results were compared with those of the controls. The plasma levels of 17 alpha-hydroxyprogesterone, cortisol, dehydroepiandrosterone sulphate (DHEA-S), androstenedione, testosterone, follicle stimulating hormone (FSH), luteinizing hormone (LH) and prolactin were established before, and 60 min after the infusion of ACTH to both patients and controls. Six patients demonstrated an increase in both the 17 alpha-hydroxyprogesterone levels and the 17 alpha-hydroxyprogesterone/cortisol ratio on ACTH stimulation, almost twice that of the mean +/- 2SD in the control group and ten times that in one patient. Six patients with abnormal elevation of 17 alpha-hydroxyprogesterone were considered heterozygotes for 21-hydroxylase enzyme deficiency, and one patient was presumed to have NC21OHD. Human leukocyte antigen (HLA) analysis supported these diagnoses. In this study, the incidence of NC21OHD in hirsute adolescent girls in our population was investigated, and NC21OHD was found in only one of 32 patients.

17-alpha-Hydroxyprogesterone↗

Familial Behçet's disease.

OBJECTIVES: To highlight the frequency, clinical features and histocompatibility antigen types of the familial form of Behçet's disease. METHODS: Twenty-seven cases with familial Behçet's disease in 12 families were evaluated according to clinical features, sites of involvement, HLA-A and HLA-B typing. A review of the literature is presented. RESULTS: The frequency of familial form of Behçet's disease was found to be 8.7% among 137 patients studied. Vascular involvement was 7.4% (2/27) in the familial group while it was 28.8% (36/125) in patients without the familial form of the disease (p < 0.01). HLA-B51(5) and HLA-A2 were positive in 68% and 75% in 16 familial cases studied, respectively. CONCLUSIONS: Familial Behçet's disease, which constitutes a small group of patients with Behçet's disease, may represent a clinically heterogeneous subtype of this entity. Although lower frequency of vascular complications was observed in this study, it is not possible to indicate the precise frequency of vascular and ocular complications of the familial form of Behçet's disease. The frequencies of HLA-A2 and HLA-B51(5) positivity are higher than the previously reported non-Behçet's controls from Turkey.

Adolescent↗

Localization of an ataxia-telangiectasia gene to chromosome 11q22-23.

Ataxia-telangiectasia (AT) is a human autosomal recessive disorder of childhood characterized by: (1) progressive cerebellar ataxia with degeneration of Purkinje cells; (2) hypersensitivity of fibroblasts and lymphocytes to ionizing radiation; (3) a 61-fold and 184-fold increased cancer incidence in white and black patients, respectively; (4) non-random chromosomal rearrangements in lymphocytes; (5) thymic hypoplasia with cellular and humoral (IgA and IgG2) immunodeficiencies; (6) elevated serum level of alphafetoprotein; (7) premature ageing; and (8) endocrine disorders, such as insulin-resistant diabetes mellitus. A DNA processing or repair protein is the suspected common denominator in this pathology. Heterozygotes are generally healthy; however, the sensitivity of their cultured cells to ionizing radiation is intermediate between normal individuals and that of affected homozygotes. Furthermore, heterozygous females are at an increased risk of breast cancer. These findings, when coupled with an estimated carrier frequency of 0.5-5.0%, suggest that (1) as many as one in five women with breast cancer may carry the AT gene and that (2) the increased radiation sensitivity of AT heterozygotes may be causing radiation therapists to reduce the doses of radiation used for treating cancer in all patients. To identify the genetic defect responsible for this multifaceted disorder, and to provide effective carrier detection, we performed a genetic linkage analysis of 31 families with AT-affected members. This has allowed us to localize a gene for AT to chromosomal region 11q22-23.

Ataxia Telangiectasia↗

Studies on lymphocyte cell surface in ataxia-telangiectasia.

Lymphocyte surface proteins of patients with ataxia-telangiectasia were separated by polyacrylamide gel electrophoresis and compared by autoradiography. The patients lacked one of the two main bands (Band I at the origin). The second main band (Band II) was absent in some cases. All patients had one or two additional bands of smaller molecular weight than Band II except one case who had no band detectable. In the patients, alkaline phosphatase, total ATPase and Mg2+. ATPase were increased but 5'-nucleotidase was normal. The results suggest abnormality in the plasma membranes of the patients' lymphocytes.

5'-Nucleotidase↗

Immune response to Epstein-Barr virus (EBV) in ataxia-telangiectasia: EBV-specific antibody patterns and their relation to cell-mediated immunity.

Epstein-Barr virus (EBV)-specific antibody titers were investigated in 60 patients with ataxia-telangiectasia (AT) and 22 healthy members of their families. In addition, we studied 36 patients with primary immunodeficiencies, Behçet disease, and other conditions and 61 unrelated healthy controls. Twenty-seven AT patients were examined sequentially at intervals varying from 2 months to 8 years. The AT patients showed an increased incidence (66.6%) of high antibody titers (greater than or equal to 1:320) to viral capsid antigen (VCA) and also a high incidence (35%) of antibody titers to early antigens (EA), but low titers (less than 1:10) of antibodies to the EBV-associated nuclear antigen (EBNA) in 35% of the patients. The geometric mean titers (GMT) of antibodies to VCA were five to six times higher; those of anti-EBNA were five times lower in AT patients as compared with control groups. In serial determinations, anti-VCA and anti-EBNA titers remained constant with the exceptions of two patients who developed ALL and Hodgkin lymphoma. The patients with other diseases did not differ significantly from the controls, with the exception of lower titers (less than 1:10) of anti-EBNA (52.8%). AT patients with low anti-EBNA titers tended to have more advanced T-cell deficiencies than those with moderate anti-EBNA titers, as detected by total lymphocyte and E-rosetting cell counts and skin test responses. The percentage of patients with low serum IgA levels was found to be higher in the low anti-EBNA group than in the moderate anti-EBNA group (44.5 vs 20%).

Adolescent↗