Regulatory T cell levels in children with asthma.

Natural regulatory T (nTreg) cells are described by expression of a specific transcription factor, FOXP3, on CD4+CD25+ cells. They play very important roles in the suppression of allergic reactions and disorders. The aim of this study was to obtain peripheral blood Treg levels among atopic asthmatic patients before and during inhaled steroid treatment and to observe the effect of these cells on the pathogenesis and treatment of asthma.

Primary immune deficiency disease awareness among a group of Turkish physicians.

Primary immunodeficiencies (PIDs) are a relatively common occurrence in countries where consanguineous marriages are widespread. A principal factor leading to misdiagnosis and ensuing complications can be the lack of knowledge and proper evaluation. The aim of this study was to assess PID awareness and the identification of diagnostic criteria leading to correct diagnosis.

Zinc and selenium status in pediatric malignant lymphomas.

Ninety-six untreated patients with malignant lymphoma (ML), 81 Hodgkin's disease, and 15 Burkitt's lymphoma were studied for zinc (Zn) status, and 21 patients also had selenium (Se) status analysis. Plasma and hair Zn and Se levels were measured by atomic absorption spectrophotometry. Chronic Zn and Se deficiencies (low plasma and low hair Zn and Se levels together) were found to be associated with ML in Turkish children.

Acute myeloid leukemia in Turkish children with Fanconi anemia. One center experience in the period between 1964-1995.

Objective: Fanconi's anemia(FA) is an autosomal recessive disorder characterized by a progressive pancytopenia,variable congenital abnormalities and an increased risk for the development of acute myeloid leukemia (AML). The objective of this study is to evaluate AML in the patients with FA diagnosed and followed-up in the Department of Pediatric Hematology at Ankara University School of Medicine in the period between 1964-1995.

Methods: A total of 39 patients within the age range 2-14 years( mean 8.

[TGF-Beta1-915G/C and TNF-alpha-308G/A polymorphisms in children with asthma].

TGF-Beta1 is a pro and antiinflammatory cytokine and plays an important role in airway remodelling in asthma. TNF-alpha is a proinflammatory cytokine that involved in the pathogenesis of asthma. Cytokine production is under genetic control, and certain single nucleotide polymorphisms (SNPs), which lead to allelic variants of cytokine genes are associated with higher or lower cytokine production in vitro.

Peripheral blood lymphocyte subsets in children with frequent upper respiratory tract infections.

It is a common and well-known fact that infants and preschool children undergo frequent episodes of upper respiratory tract infections. The majority of these children do not have a recognized immunodeficiency. The aim of the present study was to evaluate the effects of frequent upper respiratory tract infections on cellular immunity, using peripheral blood lymphocyte subsets and activation markers as defining parameters.

An unconditioned bone marrow transplantation in a child with purine nucleoside phosphorylase deficiency and its unique complication.

Purine nucleoside phosphorylase deficiency is a rare immunodeficiency syndrome characterized by recurrent infections, neurological dysfunction, and autoimmunity. Early diagnosis and hematopoietic stem cell transplantation may reverse the dismal prognosis in PNP deficiency. This report presents a new PNP deficiency case successfully transplanted without a conditioning regimen from an HLA-identical family donor, who developed a complication of disseminated BCG infection.

Stable mixed chimerism after hematopoietic stem cell transplantation in Wiskott-Aldrich syndrome.

Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency disease characterized by thrombocytopenia, eczema, impaired cellular and humoral immunity, and increased susceptibility to malignancy and autoimmunity. The only curative treatment for WAS is hematopoietic stem cell transplantation, especially in the presence of a matched sibling donor or matched unrelated donor. Here, we report the case of a 2.

A novel mutation for TAP deficiency and its possible association with Toxoplasmosis.

We describe two siblings (a male patient and his older sister) with a novel mutation in the peptide transporter associated to antigen processing (TAP). The index case presented with not only granulomatous skin lesions and recurrent sino-pulmonary infections, often associated with this deficiency, but also a severe pulmonary toxoplasmosis. His toxoplasmosis and skin lesions were successfully treated.

A case of Wiskott-Aldrich syndrome with de novo mutation at exon 4.

Wiskott-Aldrich syndrome (WAS) is an X-linked disorder characterized by thrombocytopenia, eczema and immunodeficiency. Clinical features of the disease are highly varied; therefore, the diagnosis is sometimes difficult, especially in solitary cases or cases with milder forms of the disease. However, the identification of the WASP gene has made possible a definite WAS diagnosis for these cases.

Granulocyte transfusions in children with chronic granulomatous disease and invasive aspergillosis.

The transfusion of granulocytes to restore host defenses in severely granulocytopenic patients or in patients with defective granulocyte functions has been studied for more than 60 years. However, inadequate dosage of cells and inconsistent efficacy has limited the usage of these transfusions. Recently, the use of mobilizing agents such as granulocyte colony stimulating factors and dexamethasone has renewed interest in these treatment modalities.

Peripheral blood lymphocyte subsets in healthy Turkish children.

Immunophenotyping of peripheral blood lymphocyte subpopulations is essential for the diagnosis and follow-up of children with immunodeficiencies and other immune disorders. The relative size and absolute number distributions (median and 5-95%) of lymphocyte subsets, including cord blood (Coulter, EPICS-XL) were examined by flow cytometry in 190 healthy subjects from birth to 18 years of age with a view to obtaining normal reference values for Turkish children of the following age groups: cord blood (n:29), birth to 1 year (n:41), 1 to 2 years (n:30), 2 to 6 years (n:30), 6 to 10 years (n:30), and 10 to 18 years (n:30). The relative size of CD2+, CD3+CD16-56-, CD3+CD8+ T lymphocytes increased while the relative size and absolute counts of those together with CD3+CD4+ and CD19+, CD20+ B lymphocytes decreased with age.

Transient hypogammaglobulinemia of infancy and early childhood: outcome of 30 cases.

Tansient hypogammaglobulinemia of infancy (THI) is characterized by an abnormal delay in the onset of immunoglobulin synthesis. In the present study, clinical and immunological data and outcome of 30 patients with THI are presented. Between 1995-2001, 464 patients with frequent upper and lower airway infections admitted to the Pediatric Immunology and Allergy outpatient clinic; 30 of them (aged between 6-46 months) were diagnosed as THI.

Evaluation of nutritional status and factors related to malnutrition in children on CAPD.

Objective: The aim of this study was to investigate the nutritional status of children on continuous ambulatory peritoneal dialysis (CAPD) and to relate it to the dose of dialysis and serum levels of inflammatory cytokines and insulin-like growth factor-1 (IGF-1).

Patients: 17 CAPD patients (8 girls, 9 boys; mean age 13.1 +/- 3.

Circulating adhesion molecule levels in childhood asthma.

The present study aims at comparing the levels of circulating forms of E-selectin, ICAM-1 and VCAM-1 in 10 patients with acute asthma, 10 stable atopic asthmatics, 10 nonatopic stable asthmatics and 10 age-matched healthy children. sE-selectin levels of patients with acute asthma were found to be significantly higher than that of the other three groups. The insignificant rise in sICAM-1 levels was attributed to the usage of inhaler corticosteroids.

Is immune system influenced by adenotonsillectomy in children?

Objective: Tonsils and adenoids are lymphoid tissues that are located in the pharynx and play an important role against invading antigens of the upper respiratory tract. The present study analyses serum immunoglobulin levels and peripheral blood (PB) lymphocyte subsets in children, 24-48 h prior to and 4-6 weeks after adenotonsillectomy, in order to determine early effects of adenotonsillectomy on the immune system.

Methods: The study population consists of 15 children (aged 4-10 years) who underwent adenotonsillectomy because of adenoidal hypertrophy and chronic tonsillitis and 15 age-matched healthy children without a history of adenotonsillectomy.

Endocrinologic Late Effects of Chemoradiotherapy in Pediatric Acute Leukemia.

Unlabelled: This study intends to describe growth and endocrine disorders secondary to chemotherapy among long-term survivors of pediatric acute leukemia. Sixteen patients including 14 ALL and 2 AML entered the study. Four were females and 12 were males with the mean age of 17.

Trace Element Analyses (Zinc and Selenium) in Pediatric Malignant Lymphomas.

Ninetysix untreated patients with Malignant lymphoma's, 81 Hodgkin's disease and 15 Burkitt's lymphoma were studied for zinc (Zn) and selenium (Se) status. Plasma and hair Zn, and Se levels were measured by atomic absorption spectrophotometry. Chronic Zn and Se deficiencies (low plasma and low hair Zn and Se levels together) were found to be associated with Malignant lymphoma's in Turkish children.

Characteristics of "Early Type-I Pattern" (0-6 years) Hodgkin's Disease in Turkish Children.

The clinico-epidemiologic characteristics of 54 children with HD in 0-6 years of age group were retrospectively analyzed. This group represented 27% of 200 HD cases observed in our center and was named as early type-I pattern HD. The association of EBV with HD was also shown by serologic and immunohistochemical methods (LMP1) in these very young Turkish patients.

Recurrent arterial thrombosis in a child: primary antiphospholipid antibody syndrome.

Antiphospholipid antibody syndrome (APS) is characterized by the association of recurrent arterial or venous thrombosis or recurrent fetal wastage and the presence of circulating antiphospholipid antibodies, detected as anticardiolipin antibodies or lupus anticoagulant. The authors report an 8-year-old girl, who presented with central retinal artery occlusion and live do reticularis and was diagnosed as APS. Despite the proper anticoagulant treatment she had several cerebral ischemic events and died 29 months after the diagnosis.