The prevalence of Selective Immunoglobulin M Deficiency (SIgMD) in Iranian volunteer blood donors.

BACKGROUND: Selective Immunoglobulin M Deficiency (SIgMD) is known as a rare primary immunodeficiency characterized by an isolated deficiency of serum IgM. Other immunoglobulin levels and T-cell immunity are usually normal; although IgE may be elevated. SIgMD can be asymptomatic or with various bacterial and viral infections. It can also be associated with autoimmune diseases or malignancies. In the present study, we report for the first time, the prevalence of SIgMD in Iranian healthy adult population. MATERIALS AND METHODS: A total of 3436 healthy donors were examined in the study; from August, 2006 to April, 2008. Serum IgM concentration was measured using the nephelometric method. We considered serum IgM less than 30 mg/dl as IgM deficiency. RESULTS: Among 3436 participants, 65% were male and 34% were female; aging from 17 to 72 years (38.18±10.78). Thirteen individuals were detected as IgM deficient subjects with the male to female ratio of 11/2, the prevalence of 0.37% and the frequency of 1/265. The mean serum IgM level was 24±4.56 (16-29 mg/dl) in these cases. Among 13 IgM-deficient subjects, 7 cases were available for evaluating the clinical manifestations. In addition to atopic dermatitis which was the most common symptom in these patients, others were allergic rhinitis, food allergy, urinary tract infection and skin fungal infection. Two patients had no history of infectious disease or atopic conditions. CONCLUSION: In the present study we could determine the prevalence of SIgMD in our adult population (0.37%). The most common comorbid condition was atopy. Neither severe or life-threatening infections, nor autoimmune diseases (based on their history; the antibody screening was not performed as part of this study) or malignancies were found in these patients. Further evaluation is recommended to elucidate the prevalence of SIgMD among patients with recurrent infections.

Memantine add-on in moderate to severe obsessive-compulsive disorder: randomized double-blind placebo-controlled study.

There is a growing body of evidence for the efficacy of memantine augmentation in patients with obsessive-compulsive disorder (OCD). However, to date, no double-blind study has addressed this issue. The objective of the present randomized double-blind placebo-controlled study was to evaluate efficacy and tolerability of memantine add-on treatment in patients with moderate to severe OCD. Forty-two patients with the diagnosis of OCD based on DSM-IV-TR who had a Yale-Brown Obsessive Compulsive Scale (Y-BOCS) score of ≥21 were randomly assigned to memantine (10 mg/day for the first week, and 20 mg/day for the rest of the trial) or placebo in addition to fluvoxamine for eight weeks. Patients were assessed using Y-BOCS every two weeks. Thirty-eight patients completed the study. Repeated measure ANOVA showed significant effect for time × treatment interaction in total scale [F (2.096, 75.470) = 5.280, P = 0.006] and obsession [F (2.340, 94.547) = 5.716, P = 0.002] and near significant effect for compulsion subscales [F (2.005, 79.179) = 2.841, P = 0.065]. By week eight, all patients in the memantine group and six (32%) patients in the placebo group [P value of Fisher's exact test <0.001] met the criteria for partial and complete response. At the end of the trial, 17 (89%) patients in the memantine group compared with six (32%) patients in the placebo group achieved remission (χ(2)(1) = 13.328, P < 0.001). Frequency of side-effects was not significantly different between the two groups. In summary, we showed that memantine add-on to fluvoxamine significantly improved short-term outcomes in patients with moderate to severe OCD.

Clinical and laboratory findings in Iranian patients with leukocyte adhesion deficiency (study of 15 cases).

Leukocyte adhesion deficiency type I (LAD I) is a rare, inherited, autosomal recessive, immunodeficiency disease caused by the combined loss of expression on the surface of leukocytes of the leukocyte integrins. We describe the clinical and laboratory findings for 15 patients with LAD I. The range of patients' ages was from 10 month to 14 years (median 4 years) and 93.3% of their parents had consanguineous marriages. The most commonly occurred manifestations were: recurrent infections (93.3%), poor wound healing (86%), oral ulcers (86%), and skin abscesses (80%). The most specific laboratory findings were defect in CD18 in all of 15 patients. The most common symptoms in these patients are poor wound healing and oral ulcer, so, the clinical physicians should pay special attention to these symptoms. Furthermore, because of considerable rate of consanguineous marriages in parents of LAD patients, we suggested more genetic studies on this disease and genetic consultation for these families.

Health-related quality of life in primary immune deficient patients.

The primary immunodeficiency (PI) disorders are abnormalities in development and maturation of the immune system. Individuals with PI disease may experience frequent infections, which limit their abilities to exhibit physical and psychological well-being secondary to their illness. In this survey we compared health-related quality of life of primary immune deficient patients with healthy children. The case-control study was designed for patients with PI disease who were referred to Children Medical Center in 2004-2005. Demographic information was taken and Pediatric Quality Of Life (PEDQOL) questionnaire were filled for 50 PI patients and 100 healthy children. The mean age in PI patients was 12.62+/- 3.65 (range from 8 to 18) years and in the control group was 11.04+/- 3.3 years. In PI patients 68% were male and 32% female .Most patients with PI disease had a diagnosis of common variable immunodeficiency (54%) or X-linked agammaglobulinemia (24%). Patients with PI disease had great limitations in physical functioning and psychological well-being (p<0.001 and p<0.001 respectively) compared with children without a chronic health condition. Patients had lower PEDQOL scores in all age groups compared with normal sample (p<0.001). Long duration of disease significantly correlated with low psychological score. (r =-3.23. P= 0.03) Children with PI disease experience poorer health related quality of life than healthy children, indicating more attention should be paid to early diagnosis and treatment of PI disease, as well as more attention to their social limitation. PI patients may need psychological consultation for better coping with their illness.