Elif Karakoç Aydıner

Experience with intravenous immunoglobulin in severe childhood atopic dermatitis

6. Shiohara T, Inaoka M, Kano Y. Drug-induced hypersensitivity syndrome (DIHS): a reaction induced by a complex interplay among herpesviruses and antiviral and antidrug immune responses. Allergol Int. 2006;55:1--8. 7. Picard D, Janela B, Descamps V, D’Incan M, Courville P, Jacquot S, et al. Drug reaction with eosinophilia and systemic symptoms (DRESS): a multiorgan antiviral T cell response. Sci Transl Med. 2010;2, 46ra62.

Combined T- and B-Cell Immunodeficiencies

The combined T and B cell immunodeficiencies are heterogenous disorders characterized by lack of T cell-mediated immunity associated with impaired B cell function.

Other Well-Defined Immunodeficiency Syndromes

Five broad and major groups of PID classification have been known as CID, PAD, phagocyte disorders, complement deficiencies, as well as other well-defined immunodeficiency syndromes. The former includes Wiskott-Aldrich syndrome (WAS), which presents as a clinical triad of eczema, thrombocytopenia, and recurrent infections. Patients exhibit a variety of laboratory abnormalities, including decreases in peripheral blood CD8+ T cells. A flow cytometric assay for the detection of Wiskott-Aldrich syndrome protein (WASP) protein in lymphocytes has recently been described and applied to the diagnostic evaluation of both WAS patients and carriers of WASP mutations [1].

Use of subcutaneous immunoglobulin in primary immune deficiencies.

AIM Immunoglobulin replacement therapy is required to reduce the frequency and severity of infections in patients with primary antibody deficiencies. Immunoglobulin G (IgG) can be administered intramuscularly, intravenously or subcutaneously. We aimed to evaluate the efficacy, dose adjustment and adverse events in subcutaneous immunoglobulin therapy by retrospectively presenting the records of 16 patients who received subcutaneous immunoglobulin therapy. MATERIAL AND METHODS The demographic findings, clinical and laboratory findings, subcutaneous immunoglobulin dosage and dose frequency, infusion time, area and methods, adverse events and frequency of infections were obtained from patient files and recorded. RESULTS Sixteen patients (seven female, nine male) aged between 0-33 years who were diagnosed with primary immune deficiency and treated with subcutaneous immunoglobulin were enrolled. All patients had been receiving intravenous imunoglobulin (5-10%) at a dose of 0.33-1.25 gr/kg/dose with two-four week intervals before subcutaneous immunoglobulin. Subcutaneous immunoglobulin (10%) was administered at a dose of 0.03-0.43 gr/kg/dose with one-two week intervals. No significant difference was found between serum through IgG levels before administration of intravenous imunoglobulin and steady state IgG levels during subcutaneous immunoglobulin therapy. When five patients whose serum through IgG levels were below 600 mg/dL were evaluated, however, a significant increase was found in steady state IgG levels with subcutaneous immunoglobulin therapy (p=0.043). In a ten-month follow-up period, seven infections were observed in four patients (three upper respiratory infectons, two lower respiratory tract infections and three acute gastroenteritis). No acute severe bacterial infection was observed. Local advers reaction was reported in only 10 of 180 infusions (6%). No serious adverse events were reported. All 16 patients were willing to continue IgG replacement therapy by subcutaneous administration. CONCLUSIONS Ig replacement therapy by subcutaneous route is an efficient, safe and easy option which is eligible for individual administration. Home therapy is feasible for patients with primary immune deficiency, if informed consent is obtained and sufficient education is ensured.

Ev Tozu Akarı Duyarlı Atopik Dermatitli Çocuklarda D Vitaminin Etkisi

Objective: Atopic dermatitis (AD) in studies conducted by the International Study of Asthma and Allergies in Childhood (ISAAC), children aged 6-7 years accounted for less than 2% in Iran, 16% in Japan and Sweden, and 13-14 years in Albania less than 1%, and over 15% in Northern Europe. House dust mite (HDM) sensitivity confers a distinct phenotype in AD. The current study aimed to determine the factors influencing the disease severity in HDMsensitized AD patients. Materials and Methods: Thirty-three patients with HDMsensitized AD were enrolled in the study. The medical charts were retrospectively evaluated for complaints, age at admission and diagnosis, delivery, gender, atopy in family, sensitizations, eosinophil counts, immunoglobulin and Vitamin D levels, treatment modalities and response to treatment. Results: The chief complaint was pruritus in 81.8%, rash in 45.5% and dryness in 9.1% of patients. Objective SCORAD (scoring atopic dermatitis) at admission was 45±15. Severe AD was detected in 21 patients according to their objective SCORAD index. Accompanying allergic diseases were allergic asthma (AA) in 71.9%, allergic rhinitis (AR) in 62.5% and food allergy in 27.3% of the patients. Sixteen (57.1%) patients showed decreased vitamin D levels. When familial atopy, type of delivery, and gender were set as grouping items, age at disease onset and diagnosis, laboratory parameters and objective SCORAD were similar in HDM-sensitized AD patients. On the other hand, patients with low vitamin D levels had significantly low levels of serum IgG and IgA and significantly high levels of specific IgE D1 (p=0.046, p=0.036, p=0.0023 respectively) when compared to those with normal vitamin D levels. In addition, serum vitamin D levels were found to have a strong negative correlation with specific IgE D1 levels (p=0.002, r= -0.617). Conclusion: HDM-sensitized AD has a severe and persistent phenotype. The correlation between low levels of vitamin D and high levels of D1-specific IgE in addition to lower levels of IgG and IgA in vitamin D deficient patients warrants further investigation of immunoregulatory effects of vitamin D in HDM-sensitized AD patients in larger sample sizes.