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Biomedical subjects

I Tezcan

Publications and source records attributed to I Tezcan.

At least 55 records · Page 3Linked to original sources
PubMed1984

[Campylobacter jejuni gastroenteritis].

Acute gastroenteritis is a major cause of hospital admission with attendant morbidity and occasional mortality. Campylobacter jejuni recently has been recognized as a common cause of acute bacterial gastroenteritis in children and adults. Its frequency of isolation is comparable to and in many studies exceeds that of Salmonella isolations from diarrheal stools of hospitalized patients. Medical workers in many parts of the world confirmed that Campylobacter jejuni was found more commonly in feces in diarrheic than in non-diarrheic people. The feces is loose to watery and commonly contains blood and leukocytes. Although, Campylobacter jejuni has been isolated from feces and gallbladder of healthy and diarrheic animals, until now, to our knowledge, there is no report on the isolation of the microorganism from healthy or diarrheic subjects in Turkey. This paper reports three human infections found to have Campylobacter jejuni in their stools as part of our ongoing investigation of the incidence of diarrheal disease due to this organism.

Campylobacter fetus↗
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

Necrotizing otitis externa, otitis media, peripheral facial paralysis, and brain abscess in a thalassemic child after allogeneic BMT.

Severe infection is one of the major complications in the early and late post-bone marrow transplantation period. The authors report a thalassemic child who developed necrotizing otitis externa and otitis media, a very rare complication after bone marrow transplantation, and then peripheral facial nerve paralysis and brain abscess in the early period of bone marrow transplantation despite antibacterial and antifungal prophylaxis. Necrotizing otitis media is characterized by necrosis and sloughing of considerable areas in the middle ear and adjacent tissues and is an unusual disorder because of today's antibiotics. Granulocytopenia and background ear tissue exposed to previous repeated otitis media attacks may be the predisposing factors in this case. The authors conclude that the children with previous histories of recurrent otitis media should be prepared and monitored very carefully during bone marrow transplantation because of the risk of necrotizing otitis media, especially in the granulocytopenic period.

Anti-Bacterial Agents↗
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

Severe lymphopenia in tuberculosis. A mere coincidence or a significant association?

A variety of infectious agents can cause secondary immunodeficient states. We herein present a one-year-old patient, admitted to the hospital with severe lymphopenia, who was subsequently diagnosed as tuberculosis. After the antituberculosis (anti TB) therapy was started, the clinical condition and the immunologic findings of the patient improved. We have thus concluded that the transient lymphopenia of the patient was due to Mycobacterium tuberculosis. We suggest that immunodeficiency should be investigated more often in children with tuberculosis and that further studies will shed light on the pathogenesis of this aspect of the disease.

Acidosis↗
PubMedDate not supplied

Ultrastructural findings of bone marrow in a case with malignant osteopetrosis following successful allogeneic bone marrow transplantation.

A nine-month-old female patient suffering from malignant osteopetrosis was evaluated by light and transmission electron microscopic study before and following allogeneic bone marrow transplantation (BMT). Bone marrow specimens were obtained from iliac crest biopsies. Before BMT, the bone marrow had an irregular appearance and was filled with bridging bony trabeculae devoid of cells. Following BMT, the marrow had an almost normal appearance with no myelofibrosis and a relatively regular distribution of hematopoletic cells. The osteocytes were visible in their lacunae in the bone matrix. Presence of bone resorbing and bone forming cell together demonstrated that the bone was beginning to gain its normal dynamic structure. These findings were in accordance with the clinical, laboratory and radiological data which showed the beneficial effect of the therapy.

Bone Marrow↗
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

Allogeneic bone marrow transplantation for children with myelodysplastic syndrome.

Six children with myelodysplastic syndrome underwent allogeneic bone marrow transplantation (BMT) from their HLA-identical siblings. Ages ranged from six to 16 years. French-American British (FAB) diagnosis was refractory anemia with excess blasts (RAEB) in three, RAEB in transformation (RAEB-t) in one and chronic myelomonocytic leukemia (CMML) in two cases. Two patients had progressed to leukemia before BMT. All patients received busulfan and cyclophosphamide as a conditioning regimen. Antithymocyte globulin (ATG) was administered to two of them due to the multiple transfusion history. Graft versus host disease (GvHD) prophylaxis consisted of cyclosporine-methotrexate. Engraftment was documented in all patients except one who underwent a second infusion of bone marrow cells. She died in the early post-transplant period with pancytopenia and veno-occlusive disease of the liver. Two patients died from disease recurrence. Three patients are alive > 12 months post-transplant, two are in remission and one just relapsed at +16 months and is now being prepared for a second bone marrow transplant. The only significant factor for favorable outcome was short duration between diagnosis to transplant in the two patients in remission.

Adolescent↗
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

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↗
PubMedDate not supplied

Serum IgD concentrations in immunodeficiency diseases.

Serum IgD levels were determined in 66 patients with well-defined primary immunodeficiency diseases. The two major groups of patients consisted of those with ataxia-telangiectasia (38 patients) and those with selective IgA deficiency (11 patients). The ataxia-telangiectasia patients tended to have higher serum IgD levels while no significant difference was found in the serum IgD levels of the selective IgA deficiency patients when compared with the controls. No correlation was found between the IgD levels and the presence of frequent infections in both patient groups or the associated disorders present in the selective IgA deficiency patients. The percentage of low serum IgD phenotype in the normal subjects was similar to that described in the literature.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

X-linked agammaglobulinemia: clinical and immunologic evaluation of six patients.

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.

Agammaglobulinemia↗
PubMedDate not supplied

Hyper-IgE syndrome: a case report.

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.

Chemotaxis, Leukocyte↗
PubMedDate not supplied

Clinical and immunological aspects of hyper-IgM syndrome.

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.

Adolescent↗
PubMedDate not supplied

Bare lymphocyte syndrome with lack of HLA class I and II antigens. Presentation of two cases.

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.

Female↗