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Biomedical subjects

O Sanal

Publications and source records attributed to O Sanal.

At least 73 records · Page 4Linked to original sources
PubMed1984

Cell mediated immunity in patients with subacute sclerosing panencephalitis.

Cell mediated immunity was assessed on the basis of total lymphocytes (TL), total T lymphocytes (TTL) counts, delayed skin test responses and in vitro leucocyte migration inhibition test (LIF) production in 25 patients with SSPE, classified according to the clinical stages of the disease. The patients in stage I of the disease did not show any defect in cellular immunity while the patients in stage II showed decreased TL and TTL counts, more negative skin test responses to PHA, SKSD and PPD, and unresponsiveness to SKSD of LIF production. When the patient group was evaluated as a whole, only the TL counts and the skin test responses against SKSD differed from those in the controls. These results suggest that the defects in cellular immunity demonstrated in the patients with SSPE may be due to SSPE or the measles virus itself rather than to a genetic factor predisposing patients to SSPE.

Adolescent↗
PubMed1983

Defective lymphocyte locomotion in Chédiak-Higashi syndrome.

Various defects in leukocyte function have been reported in Chédiak-Higashi syndrome (CHS); however, to our knowledge, lymphocyte locomotor capacity has not been investigated. A 7-year-old boy with typical signs and symptoms of CHS showed impaired neutrophil, monocyte, and lymphocyte locomotion. Chemotaxis studies were performed by the micropore filter technique using modified Boyden chambers, and migration was measured by the leading front method. Treatment with ascorbic acid (vitamin C), 500 mg/day for five days, resulted in a significant increase in in vitro neutrophil and monocyte chemotaxis but failed to affect lymphocyte locomotion.

Ascorbic Acid↗
PubMed1982

The effect of concanavalin-A (Con A) stimulation on lymphocyte subpopulations.

It has been shown that suppressor T lymphocytes can be activated by plant mitogens as Con A. In order to see if there is any change in lymphocyte surface markers in addition to functional activity, lymphocyte subpopulations (E, EA gamma, EAC rosette forming cells) were determined in 18 healthy controls and 16 patients with ataxia-telangiectasia (AT) after 18-24 hour incubation of lymphocytes with or without Con A (10 micrograms/ml per 2 x 10(6) lymphocytes). Without any incubation, the percentage of EA gamma-RFC was higher and E-RFC was lower in AT patients than in controls. In healthy controls the percentage of EA gamma-RFC significantly increased after incubation with Con A, while the patient group did not show any significant difference. In contrast, the percentage of E-RFC cells in AT group showed significant increase after Con A incubation, while this difference was not significant in healthy controls. There was no significant difference in percentage of EAC-RFC with or without Con A incubation in healthy controls and AT patients. These differences between patient and control groups can be explained by the different features in circulating lymphocyte subpopulations.

Ataxia Telangiectasia↗
PubMed1981

Epstein-Barr-virus-carrying lymphoma in a patient with ataxia-telangiectasia.

An undifferentiated lymphocytic lymphoma of mesenteric lymph nodes occurred in a young boy with ataxia-telangiectasia. Two independent tests, Epstein-Barr virus (EBV)-cRNA/DNA hybridisation and EBV DNA/DNA reassociation kinetic analysis, showed 53 and 68 EBV genome equivalents per cell respectively, which was compatible with an EBV-genome-carrying tumour. Whether this was a polyclonal lymphoproliferation or a monoclonal tumour could not be determined owing to lack of suitable material. The presence of EBV genomes should be sought in lymphomas arising in ataxia-telangiectasia and other immunodeficiencies.

Abdominal Neoplasms↗
PubMed1980

Fanconi's aplastic anemia, analysis of 18 cases.

A total of 18 patients within the age range of 5-13 years, 12 male and 6 female, are diagnosed as having Fanconi's aplastic anemia on the basis of congenital abnormalities, pancytopenia, bone marrow hypoplasia, and chromosomal and hematologic analysis. The hereditary and familial basis of Fanconi's aplastic anemia was apparent in this series. Common abnormalities were growth retardation, café au lait spots, hyperpigmentations, microcephaly, phalange deformities, mental retardation, and hypogenitalism; chromosome abnormalities were detected in the majority of our cases. Mast cells were observed in the bone marrow in most of the patients. 1 case developed acute myelomonocytic leukemia.

Abnormalities, Multiple↗
PubMed1979

Epstein-Barr virus-related antibody patterns in ataxia-telangiectasia.

Epstein-Barr virus-related antibody titres were determined in twenty-seven patients with ataxia-telangiectasia (AT) and twenty-two healthy members of their families, in twenty-two patients with other diseases, among them ten with Behçet's disease and ten with various primary immune deficiencies, and fifteen healthy members of their families, and in twenty-three unrelated healthy controls. The AT patients showed an increased incidence (55.6%) of high antibody titres (greater than or equal to 1:320) to viral capsid antigen (VCA), and also a high incidence (48.2%) of antibodies to Epstein-Barr virus (EBV) induced early antigens (EA), but low titres (less than 1:10) of antibodies to the EBV-associated nuclear antigen (EBNA) in 44.2% of the cases. The geometric means of anti-VCA were three-to four-fold higher, and of anti-EBNA six-fold lower, than those of the control groups. The patients with the other diseases did not differ significantly from the controls except for a higher incidence of anti-EBNA titres of less than 1:10 (38.1% vs 4--5%). AT patients with low anti-EBNA titres tended to have more advanced T cell deficiencies than AT patients with moderate anti-EBNA titres, as detected by counts of total lymphocytes and E-rosetting cells, and skin test responses. The results support the hypothesis that a functioning T cell system is required to release EBNA from EBV genome-carrying cells for initial and maintained production of anti-EBNA.

Adolescent↗
PubMedDate not supplied

Alopecia universalis in a patient with common variable immunodeficiency.

A 12-year-old boy with common variable immunodeficiency (CVI) who developed severe alopecia is presented. His sister also had alopecia and recurrent infections and died of lung infection at the age of 7 years. The loss of hair in both children was total; the pathology of a scalp skin biopsy specimen was typical for alopecia areata. The boy was subjected to clinical and immunologic evaluation and the results were compatible with common variable immunodeficiency.

Alopecia Areata↗
PubMedDate not supplied

Neonatal tuberculosis.

We present an autopsy case of neonatal miliary tuberculosis in a 36-day-old baby that was born to a mother who had no symptoms. Histopathological examination of lungs revealed necrosis and numerous acid-fast bacilli. Mycobacterium tuberculosis hominis was isolated by guinea pig inoculation with lung tissue. Three months after dietary the mother was admitted with pleural effusion and endometrial biopsy showed a granulomatous inflammation compatible with tuberculosis. Intrauterine infection was considered and that was supported by retrospective findings of the endometrial biopsy. We also want to emphasize the importance of searching for genital tuberculosis even if the mother has no symptoms.

Autopsy↗
PubMedDate not supplied

Fc gamma receptor allotypes in children with bacterial meningitis. A preliminary study.

IgG2 antibody is the essential subclass to protect against encapsulated bacteria Fc gamma RIIa is the only Fc gamma receptor that interacts with human IgG2. The two genetically determined allotypes of human Fc gamma RIIa, Fc gamma RIIa-R131 and Fc gamma RIIa-H131 alleles have functionally different reactivities with IgG2 in vitro, and H/H-131 cells have markedly higher binding affinity for human IgG2. Homozygous Fc gamma RIIIb-NA1/NA1 PMNLs show higher phagocytic capacity than Fc gamma RIIIb-NA2/NA2 PMNLs. To evaluate in vivo significance of Fc gamma RIIa and Fc gamma RIIIb allotypes, we analyzed Fc gamma R allotypes in children with bacterial meningitis due to Haemophilus influenzae type b, Streptococcus pneumoniae and Neisseria meningitidis. Fc gamma RII and Fc gamma RIIIb polymorphisms were determined by using quantitative flow cytometry. Fc gamma RIIa were studied in 23 children with bacterial meningitis and 50 healthy Turkish controls, and Fc gamma IIIb in 18 and 43 such individuals, respectively. The distribution of Fc gamma RIIa in the healthy Turkish control group was found to be significantly different from that in the Chinese and Japanese population (p < 0.05), but similar to that of the white population in the USA and the Netherlands. No case (0%) had the Fc gamma RII-H/H-131 Fc gamma RIIIb-NA1/NA1 the corresponding figure in the controls was 4 (9.3%). Homozygous Fc gamma RIIa-H/H-131 phenotype was underrepresented with borderline significance (p: 0.057) in patients below two years of age in comparison with the healthy subjects and with patients with meningitis over two years of age (p: 0.059). Although the study needs to be conducted in a large series of patients in order to draw a firm conclusion, Fc gamma RIIa polymorphism may be a contributing factor to the increased susceptibility to meningitis with encapsulated bacteria in children below two years of age.

Adolescent↗
PubMedDate not supplied

Cyclic neutropenia complicated by renal AA amyloidosis.

Cyclic neutropenia is a rare disease characterized by regular cyclic fluctuations in the numbers of neutrophils. Patients with the disease suffer from recurrent infections at regular intervals of nearly three weeks. Recently, recombinant human granulocyte colony-stimulating factor (rhG-CSF) was reported to be an effective treatment for this disease. here we describe 17-year-old cyclic neutropenic female patient with a very rare association of renal amyloidosis of AA type who was under intermittent rhG-CSF treatment for the previous one and a half years. We conclude that although the disorder is usually benign, reactive amyloidosis may rarely develop in cases who remain untreated for a long period of time. However familial Mediterranean fever (FMF) type II should also be born in mind, particularly in predisposed populations.

Adolescent↗
PubMedDate not supplied

Gastric antral stricture in a patient with chronic granulomatous disease.

Chronic granulomatous disease (CGD) is a rare disorder of phagocytic cell oxidative metabolism. Patients have recurrent infections with catalase-positive organisms and granulomatous lesions throughout the body. Gastric antrum can be an occult site of involvement. We describe a four-year old boy with chronic granulomatous disease who was admitted with the complaints of persistent vomiting and weight loss. Gastric antral narrowing was diagnosed according to radiological findings. Treatment with steroid and antibiotics yielded a good clinical response in 15 days with a relief of the obstruction. This case report emphasizes the beneficial effect of this form of therapy in preventing life-threatening obstruction of vital organs in CGD.

Anti-Infective Agents↗
PubMedDate not supplied

Effects of intravenous immunoglobulin on clinical and immunological findings of patients with humoral immunodeficiency diseases.

We evaluated nine patients with humoral immunodeficiency (6 immunodeficiency with hyper-IgM, 2 X-linked agammaglobulinemia, 1 common variable immunodeficiency) who were being treated with intravenous immunoglobulins (IVIG). After the use of the IVIG regimen in a dose of 250-300 mg/kg/4 weeks for one year, the severity and frequency of infections, even in patients with chronic lung disease, decreased significantly. An improvement in pulmonary function tests was observed in four patients who had airway obstruction prior to IVIG therapy. Side effects such as chills and fever were observed in 21 of 91 infusions, particularly in the early months of therapy. Preinfusion administration of aspirin and diphenhydramine prevented these side effects. The inversion of the CD4+/CD8+ ratio was detected in most patients during both intramuscular gammaglobulins (IMIG) and IVIG therapy.

Adolescent↗
PubMedDate not supplied

IgG subclasses in symptomatic IgA deficiency.

We evaluated IgG subclass levels in 11 symptomatic patients (ages between 3 and 22; mean 7.5 years) with IgA deficiency, seven with selective IgA deficiency, and four with low IgA levels. All patients had experienced three or more episodes of sinopulmonary infections a year. Combined IgG2-IgG4 deficiency was detected in two patients, IgG2 deficiency in one patient and IgG4 deficiency in two patients. Elevated IgG1 and IgG3 levels were detected in most of the IgG subclass deficient and sufficient patients. It is known that gammaglobulin replacement therapy reduces the frequency of infections significantly in IgG subclass deficiency. Although immunization against IgA is a risk in IgA deficiency, these patients can be treated with gammaglobulins containing low IgA.

Adolescent↗
PubMedDate not supplied

Gene mapping using linkage analysis.

Linkage analysis is a recombinant technology used for gene mapping. If two genetic loci segregate together in a pedigree more often than by random chance, they are said to be linked, that is they lie close on the same chromosome. What makes the detection of linkage between two markers on a chromosome possible, are the recombination events which occurred during meiosis. Thus, information can be obtained for the location of a mutant gene by using markers with known locations. Since the first published reports of linkage over thirty years ago, the use of the "lod score" method to determine the chromosomal location of a disease gene has become widespread. With the continual production of new RFLPs, the mapping of new loci will continue to become faster and more efficient. The power of the linkage method has spawned the development of several new techniques which utilize marker data to graphically represent the most likely location of a disease gene. Such advances, however, can best be utilized only after initial chromosomal localization of the particular gene through the lod score method.

Chromosome Mapping↗
PubMedDate not supplied

IgG subclass deficiency in children with recurrent infections.

IgG subclass levels were studied in 12 children, aged between 2.5-12 years with recurrent respiratory tract infections. They did not have low IgG levels or IgA deficiency. We found combined deficiency of IgG2-IgG4 in one patient and selective IgG2 deficiency in another (16.6% of patients). These two patients had bronchiectasis due to recurrent severe pneumonia, however one patient with bronchiectasis had normal IgG subclass concentrations. Our IgG subclass-deficient patients who did not respond to prophylactic antibiotic therapy were given gammaglobulin therapy. IgG subclass deficiency should be considered in children with unexplained recurrent infections even in the presence of normal serum immunoglobulin levels.

Child↗