The Risk of the Next Child Getting Affected by Chronic Granulomatous Disease in Families with at Least One Autosomal Recessive CGD Child.

Chronic granulomatous disease (CGD) is a rare primary immunodeficiency disorder more common in autosomal recessive (AR) than X-linked in Iran. This study aimed to assess whether having a child with AR-CGD would increase the likelihood of the next child being affected by CGD. Ninety-one families with at least one child affected by AR-CGD entered this study. Out of the 270 children, 128 were affected by AR-CGD. We used a cross tab for the odds ratio (OR) calculation, in which exposure to a previously affected child and the next child's status were evaluated. This study illustrated that the chances of having another child afflicted with AR-CGD are significantly increased if the previous child had AR-CGD (OR=2.77, 95% CI=1.35-5.69).Althoug h AR disorders affect 25% of each pregnancy, we showed that the chance that the next child would be affected by CGD, given that the previous child was affected, is 2.77 times greater than in families with a normal child. It is recommended to warn families with one or more affected children to evaluate the risk of CGD in their subsequent pregnancies with prenatal diagnosis.

Contribution of survivin to the immune system, allergies and autoimmune diseases.

In addition to malignancies, survivin (a member of the apoptosis inhibitor family) has been implicated in the pathogenesis of inflammatory disorders, including autoimmune and allergic diseases. Survivin is constantly expressed in the proliferating hematopoietic progenitor cells, and it is re-expressed in the mature cells of the innate and adaptive immunity, upon activation. Survivin enhances the expression of co-stimulatory molecules and MHC class II molecules in dendritic cells, and promotes the lifespan of macrophages, neutrophils, and eosinophils, while suppressing natural killer (NK) cell activity. Survivin has been implicated in T cell maturation, T cell expansion, effector CD4+ T cell differentiation, maintenance of memory CD4+ T and CD8+ T cells, as well as antibody production. Upregulated expression of survivin was indicated in the T cells as well as various samples collected from allergic patients. Survivin can contribute to the pathogenesis of allergic diseases via the promotion of the Th2 polarization, promoting IL-4 expression, compromising activation-induced cell death (AICD) in Th2 cells, and preventing apoptosis of eosinophils, as well as, amplification of eosinophilia. Moreover, survivin can interfere with clonal deletion of autoreactive T and B cells, as well as suppress Treg cell development and activity supporting the development of autoimmune diseases. This review discusses the role of survivin in immunity, allergy and autoimmunity as well as provides evidence that survivin may be considered as a novel therapeutic target for the treatment of allergic and autoimmune diseases.

Allergic asthma manifestations in human and seropositivity to Toxocara, a soil-transmitted helminth of carnivores: A case-control study and scoping review of the literature.

Asthma is a common respiratory disease affecting humans. Helminth parasites, including Toxocara species, have been implicated as predisposing factors of asthma. However, various studies present different findings on asthma-Toxocara association. Herein, we investigated the association of asthma manifestations with Toxocara seropositivity in a case-control setting on 248 participants (147 women and 101 men), with 124 healthy individuals as the control group and 124 patients known to have asthma based on the medical records of asthma clinics of Kerman University of Medical Sciences. Consequently, we presented a scoping review of all previous studies carried out on this topic, summarizing current findings and existing knowledge on this issue. Of 248 participants, 31 (12.5%) were Toxocara-seropositive, of which 19 (15.3%) were in the patient group and 12 (9.7%) in the control group. A significant relationship was found between asthma severity and age in Toxocara-seropositive individuals (P < 0.04). We found no significant relationship between asthma and Toxocara seropositivity. We identified 7,724 related records in three major scientific databases, NCBI PubMed, Scopus, and Google Scholar. The review of the literature showed that there are 80 published articles on asthma-Toxocara relationship with contradictory findings. More than half of the studies were performed in only four countries, namely, Brazil, the Netherlands, the United States, and Iran. The study population in 70% of the studies were children, and few studies investigated asthma-Toxocara association in adults. The most common study designs for investigating the association of asthma and Toxocara seropositivity were cross-sectional (35.0%), case-control (27.5%), and animal experimental (12.5%) studies. This study found no significant relationship between asthma manifestations and toxocariasis in a case-control setting. However, a scoping review of the current literature suggests that further experimental and field longitudinal cohort studies are required to elucidate the nature of asthma-Toxocara interaction in humans.

Echinococcosis in immunocompromised patients: A systematic review.

BACKGROUND: Human echinococcoses are the infection caused by the larval stages of different species of the genus Echinococcus, mostly E. granulosus and E. multilocularis. There is no aggregated information on the nature and characteristics echinococcosis in patients with immunodeficiency. This study presents a systematic review of the current literature published on the status of echinococcosis in immunocompromised individuals. METHODS: An electronic search of related articles in four major databases (PubMed, Scopus, Web of Science and Google Scholar) was performed up to November 2021. All related studies meeting the inclusion criteria were assessed for qualitative analysis. Data available on different characteristics of the diseases were extracted. The data were subsequently categorized into two subgroups: Cystic Echinococcosis (CE) and Alveolar Echinococcosis (AE). RESULTS: Twenty-eight articles related to the existence of echinococcosis in immunocompromised hosts were included. HIV/AIDS was found as the most frequent condition in immunocompromised CE patients. Most of the CE cases with immunodeficiency were female (66.4%). The dominant stages of the cysts were CE2 and CE3. Surgery was performed for 76.2% of the patients. A high mortality rate of 23.8% was recorded in CE patients. Malignancies was the dominant condition in AE patients. CONCLUSION: Findings of the present study can potentially improve our understanding of the impact of immunodeficiency syndromes on echinococcoses and contribute to an improved diagnosis, treatment and quality of care in immunocompromised patients suffering from cystic and alveolar echinococcosis.

Epidemiology, Sociodemographic Factors and Comorbidity for Allergic Rhinitis, Asthma, and Rhinosinusitis Among 15 to 65-year-Old Iranian Patients.

Background: It is well established that upper and lower airways are often clumped together when diagnosing and treating a disease. This study was designed to determine the prevalence of upper and lower airway diseases and to assess the effect of sociodemographic factors on the prevalence and the comorbidity of these disorders. Methods: This cross-sectional population-based study included patients with ages ranging between 15 to 65 years, who were referred to allergy outpatient clinics in various provinces of Iran from April to September 2020. A modified global Allergy and Asthma European Network (GA2LEN) screening questionnaire was filled out by local allergists of the 12 selected provinces in Iran. Information about the patients and sociodemographic factors was also recorded. Statistical analysis was done by univariate statistical analyses and multiple logistic regressions in SPSS software Version 26. Results: Out of 4988 recruited patients, 1078 (21.6%) had the symptoms of allergic rhinitis (AR) and 285 (5.7%) met the criteria of asthma. The prevalence of acute rhinosinusitis (ARS) and chronic rhinosinusitis (CRS) was 21.6 % and 22%, respectively. The highest prevalence of AR and ARS was in Tehran with the arateof of 33.9% each. Asthma was more prevalent in Khuzestan (14.2%) and CRS in Baluchestan (57.5%). Our analysis showed that the patients with asthma were most likely to have other allergic diseases as well-CRS (OR = 4.8; 95% CI, 2.02- 5.82), AR (OR= 2.5, 95% CI, 2.10-3), ARS (OR = 1.8; 95% CI, 2.10-3), followed by eczema (OR = 1.4; 95% CI, 1.13-1.67).We found that those individuals with CRS were most likely to have painkiller hypersensitivity (OR= 2.1; 95% CI, 1.21-3.83). Furthermore, smoking has been found more than 1.5 folds in patients with ARS. After adjusting variables, there was no correlation between education, occupation, and ethnicity with the studied diseases. Conclusion: Rhinosinusitis is a common condition among Iranian patients. This study confirmed that inflammation of the upper and lower airways can occur simultaneously. Gender, education, occupation, and ethnicity were found to be irrelevant in the development of either AR, asthma, ARS, or CRS.

Prevalence and Molecular Subtyping of Blastocystis from Patients with Irritable Bowel Syndrome, Inflammatory Bowel Disease and Chronic Urticaria in Iran.

BACKGROUND: Blastocystis is a parasite that colonizes in the human intestine. Its clinical features include diarrhea, abdominal pain, or urticarial and irritable bowel syndrome (IBS). Spite of being significant genetic diversity and numerous subtypes within the genus there were no associations between its subtypes and symptomatology. MATERIALS AND METHODS: Aim of this project was subtyping of the protozoa in 184 Iranian people with history of IBS/IBD (n = 74) or chronic urticaria (n = 59) and individuals referred to general clinic (n = 51). Microscopic and molecular examinations used for identifying and subtyping of Blastocystis. RESULTS: Overall, frequency of the parasite was 24.46% while, 29.41% of people who referred to general clinic, 20.27%, and 25.42% of IBS/IBD and urticarial cases were infected, respectively. Subtyping result showed that 28.89% of all people were infected with Blastocystis sp. while the prevalence of ST3, ST2 and ST1 were 22.22%, 22.22%, and 17.78%, respectively. Blastocystis sp., was identified in most IBS/IBD cases (46.7%) followed with ST2 and ST3 (13.3 and 13.3, respectively). Whereas, in chronic urticaria group ST2(33.3%) was the major subtype and most individuals in control group were infected with ST3 (33.3%). Pearson's Chi Square test showed no significant differences between the parasite or subtype prevalence and diseases (p > 0.05). CONCLUSION: Given significant factors have effect on clinical signs including host or parasite genetics, microbiota, as well as environmental factors, it seems that further studies are needed to find out different markers of host susceptibility to diverse parasite genotypes in patients with irritable bowel syndrome or urticaria.

Genetic and molecular findings of 38 Iranian patients with chronic granulomatous disease caused by p47-phox defect.

One of the components of NADPH oxidase is p47-phox, encoded by NCF1 gene. This study aims to find new genetic changes and clinical features in 38 Iranian patients with autosomal recessive chronic granulomatous disease (AR-CGD) caused by NCF1 gene defect. Patients who had abnormal NBT and DHR-1,2,3 assay with loss of p47-phox in Western blotting were included in this study. After recording demographic and clinical data, PCR amplification was performed followed by direct sequencing for all exons and exon-intron boundaries. The most common form of CGD in Iran was AR-CGD due to consanguinity marriages. Among patients with AR-CGD, NCF1 deficiency was found to be more common than other forms. Cutaneous involvements (53%), pulmonary infections (50%) and lymphadenopathy (29%) were more prevalent than other clinical manifestations of CGD. Mutation analysis of NCF1 gene identified five different mutations. Homozygous delta GT deletion (c.75_76delGT) was the most frequent mutation and was detected in more than 63% of families. Six families had a nonsense mutation in exon 7 (c.579G > A). Two novel mutations were found in exon 4 in two families, including a missense mutation (c.328C > T) and a nine-nucleotide deletion (c.331_339delTGTCCCCAC). Genetic detection of these mutations may result in early diagnosis and prevention of possible complications of the disease. This could be useful for timely decision-making for haematopoietic stem cell transplantation and for carrier detection as well as prenatal diagnosis of next children in the affected families. Our findings might help to predict outcomes, raise awareness and help effective treatment in these patients.

Anthropometric Parameters in Asthmatic Children and the Relationship of Childhood Asthma with Height, Weight and Body Mass Index.

Background: Asthma as a chronic disease may affect the growth process. The aim of this study was to investigate the anthropometric indices in 2-18 years old children with asthma and compare them with the control group. Patients and Methods: In a case-control study, 150 asthmatic children with age of 2-18 years as case group and 300 age- and sex-matched healthy children as control group were randomly included. The height, weight, and body mass index (BMI) of both group measured by the standard method and Z score was calculated. Data were analyzed using SPSS, chi-square and analysis of variance. Results: Totally, 290 boys (64.4%) and 160 girls (35.6%) with mean age of 6.58±2.82 years were evaluated. Case group had significantly lower height compared to the healthy control group (117.00±0.17 cm vs. 121.00±0.15 cm respectively, P=0.025). No significant differences were detected in weight (23.13±9.75 kg vs. 24.62±10.36 kg, P=0.145) and BMI (16.32±3.10 kg/m2 vs. 16.28±3.16 kg/m2, P=0.900) between case and control groups, respectively. There were no significant relationships between normal and abnormal Z scores of height, weight and BMI in case and control group (P>0.05). Conclusion: Despite 4 cm difference between the age of two groups, no differences in height, weight ad BMI between two groups may be due to good control of the disease in the case group or lack of significant growth related effect of asthma.

Imbalanced Expression of Th2 and Treg Cell-related Parameters in Peripheral Blood Mononuclear Cells in Patients with Allergic Asthma.

BACKGROUND: The imbalance between Th2 and Treg cells plays fundamental role in the pathogenesis of allergic asthma. The current study aimed at assessing the expression of some Th2 and Treg cell-related parameters in patients with allergic asthma. MATERIAL AND METHODS: The serum and peripheral blood mononuclear cell (PBMC) samples were collected from 30 patients with asthma and 36 healthy subjects. The serum levels of transforming growth factor (TGF)-ß, interleukin (IL)-4, as well as the expression levels of GATA3 and FOXP3 genes in PBMCs were determined by the enzyme-linked immunosorbent assay (ELISA) and real-time polymerase chain reaction (PCR), respectively. The PBMCs were cultured for 48 hours with/without phytohemagglutinin (PHA) stimulation. The TGF-ß and IL-4 levels in supernatants were also determined. RESULTS: The serum levels of IL-4, the expression level of GATA3, and GATA3/FOXP3 ratio in patients with asthma were significantly higher than healthy subjects (P <0.002, P <0.001, and P <0.004, respectively). The FOXP3 expression did no differ between the two groups. The serum level of TGF-ß as well as its secretion profile in non-stimulated and stimulated PBMCs isolated from patients with asthma were significantly higher than those of the controls (P <0.03, P <0.001, and P <0.001, respectively). The serum TGF-ß levels in severe asthma were significantly higher than moderate asthma; whereas the TGF-ß secretion by PHA-stimulated PBMCs isolated from moderate asthma was higher than that of severe pattern of the disease (P <0.001 and P <0.05, respectively). The GTAT3/FOXP3 expression ratio in moderate asthma was significantly higher than severe form (P <0.04). CONCLUSION: The results confirmed a Th2 cell-biased pattern and possible contribution of TGF-ß in allergic asthma. TGF-ß may have different expression patterns in moderate and severe asthma and the two forms of the disease may have differences in some main immunological parameters.

Survey on the prevalence of allergic rhinitis and its effect on the quality of high school students' life.

INTRODUCTION: Allergic rhinitis (AR) is a common airway disease. In order to study the prevalence of AR in high school students in Kerman, the Score for Allergic Rhinitis (SFAR) was used and the quality of life in the students affected by rhinitis was evaluated using the SF-36 questionnaire. MATERIALS AND METHODS: This was a cross-sectional, analytical, descriptive study, based on the SFAR scale. Quality of life in students with AR was evaluated using the SF-36 questionnaire. RESULTS: From 1511 students who completed the SFAR questionnaire, 291 (52.6%, girls; 47.4%, boys) had AR. Domestic dust was the most common cause of the disease. The most common symptoms of AR were rhinorrhea (76.6%), epiphora (76.3%), nasal congestion (64.3%), and itching (54.3%). According to the ARYA scale, (Allergic Rhinitis and its Impact on Asthma), 41.9% of students had moderate-to-severe rhinitis and 58.1% had mild rhinitis. A total of 43.1% of patients with moderate-to-severe rhinitis had a persistent condition and 56.9% had an intermediate condition. Results of the SF-36 questionnaire among students with AR showed a significant difference in physical functioning and bodily pain in comparison with healthy students. CONCLUSION: The results of this study show that the prevalence of AR among Kerman high school students is 19.3%. Because of the effect of this disease on the life quality of high school students in terms of both physical functioning and bodily pain, efforts should be made to reduce allergen levels as far as possible.

Inheritance pattern and clinical aspects of 93 Iranian patients with chronic granulomatous disease.

BACKGROUND: Chronic granulomatous disease (CGD) is a rare immunodeficiency due to a genetic defect in one of the NADPH-oxidase components. We studied CGD inheritance forms (autosomal recessive (AR) or X-linked (XL)) and AR-CGD subtypes in Iran. METHODS: Clinical and functional investigations were conducted in 93 Iranian CGD patients from 75 families. RESULTS: Most of the patients were AR-CGD (87.1%). This was related to consanguineous marriages (p = 0.001). The age of onset of symptoms and diagnosis were lower in XL-CGD compared with AR-CGD (p < 0.0001 for both). Among AR-CGD patients, p47phox defect was the predominant subtype (55.5%). The most common clinical features in patients were lymphadenopathy (65.6%) and pulmonary involvement (57%). XL-CGD patients were affected more frequently with severe infectious manifestations. CONCLUSIONS: Although XL-CGD is the most common type of the disease worldwide, only 12 patients (12.9%) were XL-CGD in our study. The relatively high frequency of AR-CGD is probable due to widely common consanguineous marriages in Iran.

Cutaneous manifestations of primary immunodeficiency diseases in children.

Primary immunodeficiency diseases (PIDs) are rare but include severe conditions found predominantly in children, Most PIDs have cutaneous manifestations that may be important as early diagnostic features. The purpose of this study was to determine the frequency and nature of cutaneous alterations associated with PIDs. This article is a cross-sectional study at the department of allergy and clinical immunology of children's medical center conducted between December 5, 2001 and April 20, 2002. The subjects included pediatric patients with a diagnosis of PID and dermatological diagnoses were made by a dermatologist. Two hundred and ten patients were studied They consisted of 68 cases of humoral deficiency, 22 cases of cellular and combined deficiencies, 57 cases of phagocytic defects and 63 cases of other PIDs. In 67 cases (31.8%) the cutaneous alterations preceded and were the basis for clinical immunological diagnosis. Overall cutaneous alterations were infections in 99 cases and eczematous dermatitis in 27 cases. Our findings support the results of other studies that most PIDs have cutaneous features which being their typical aspects are highly suggestive for the diagnosis of PIDs.

Adverse reactions of prophylactic intravenous immunoglobulin infusions in Iranian patients with primary immunodeficiency.

BACKGROUND: Although long-term intravenous immunoglobulin infusion is an effective treatment for children with antibody deficiencies, it can be complicated by systemic adverse reactions. OBJECTIVE: To evaluate the adverse reactions of intravenous immunoglobulin therapy in patients with primary immunodeficiency. METHODS: Seventy-one immunodeficient patients receiving intravenous immunoglobulin were evaluated during a 7-year period (1995-2002) at Children's Medical Center in Tehran, Iran. Immunological diagnoses were as follows: common variable immunodeficiency (31 patients), X-linked agammaglobulinemia (25 patients), IgG subclass deficiency (5 patients), hyper-IgM syndrome (2 patients), and ataxia-telangiectasia (8 patients). RESULTS: One hundred fifty-two cases (12.35%) of adverse reactions occurred following 1,231 infusions in 35 patients. The most frequent immediate adverse reactions were mild reactions (131 infusions), including chills, fever, flushing, muscle pains, nausea, headache, and anxiety. Moderate reactions, such as vomiting, chest pain, and wheezing, occurred in 19 infusions. Two patients experienced severe adverse reactions. The highest proportion (23.06%) of reaction to injection was in patients with common variable immunodeficiency. CONCLUSIONS: Intravenous immunoglobulin is a well tolerated medical agent for patients with antibody deficiency. However, to prevent occurrence of immediate adverse reactions during infusion in these patients, physicians should perform a detailed history and proper physical examination and check the titer of anti-IgA.

Mortality in primary immunodeficient patients, registered in Iranian primary immunodeficiency registry.

Primary immunodeficiencies (PID) are a group of disorders, characterized by an unusual susceptibility to infections. Delay in diagnosis results in increased morbidity and mortality in affected patients. The purpose of this study was to determine the mortality rate of Iranian immunodeficient patients referred to Children Medical Center Hospital affiliated to Tehran University of Medical Sciences over a period of 20 years.In this study, records of 235 (146 males, 89 females) patients with immunodeficiency who were diagnosed and followed in our center, during 22 years period (1979-2001) were reviewed. The diagnosis of immunodeficiency was based on the standard criteria. The cause of death was determined by review of death certificates.Antibody deficiency was the most common diagnosis made in our patients. The overall five-year survival rate was 22.7% in our studied patient group; this was greatest in antibody deficiency. During the 22 year period of study, 32 patients died. As some of the patients could not be located, the true mortality rate ranged between 13.6% and 17.5%. The main leading cause of death were lower respiratory tract involvement in 14 cases (44%). The most common pathogenic microorganisms causing fatal infections were psudomonas and staphylococcus in 9 cases (28.1%) followed by E. coli in 7 (21.9%), tuberculosis in 13 (40.6%) and salmonella in 1 (3.1%).Based on our study, delay in diagnosis in patients with PID results in tissue and organ damage and several complications. Mortality and morbidity are increased in undiagnosed patients.

Neutropenia in patients with primary antibody deficiency disorders.

Neutropenia is characterized by decrease in the absolute number of circulating neutrophils and an increase susceptibility to infections. The current study was performed in order to explain the clinical and laboratory findings of patients with antibody deficiency disorders associated neutropenia. The patients' records of 19 neutropenic cases out of 207 patients with antibody deficiencies, who had been referred to Children's Medical Center and enrolled in Iranian primary immunodeficiency registry, were reviewed. Nineteen cases (14 male and 5 female), with a mean age of 10.7+/-5.7 years, were associated with neutropenia (9.2%). The disorders with associated neutropenia were Hyper IgM syndromes (3 of 8), Common variable immunodeficiency (13 of 109), and X-linked agammaglobulinemia (3 of 45). The median age for the onset of disease and diagnosis age were 15 months (1-134) and 3.8 years (6 months-13 years), respectively. The most common infections during the course of illness were pneumonia (13 cases), diarrhea (12 cases), oral candidiasis (9 cases), otitis media (6 cases), sinusitis (6 cases), cutaneous infections (5 cases), and abscess (5 cases). Other less frequent infections were: conjunctivitis, oral ulcers, meningitis, and osteomyelitis. Three neutropenic patients died because of recurrent infections. Neutropenia may occur in any of the primary immunodeficiency disorders. Persistent or severe infections always pose a supposition, which deserves further evaluation for detecting an underlying immune deficiency syndrome and neutropenia, since a delay in diagnosis may result in a serious organ damage or even death of the patient.

Gastrointestinal manifestations of patients with chronic granulomatous disease.

Chronic Granulomatous Disease (CGD) represents a group of inherited disorders of phagocytic system, manifesting recurrent infections at different sites. The present study was accomplished in order to determine the gastrointestinal manifestations of CGD patients. Fifty-seven patients (38 males and 19 females) with CGD, who had been referred to three immunodeficiency referral centers in Iran, were studied during a 24-year period (1980-2004). The median age at the time of study was 14.5 years old (1-56 years). The median onset age of symptoms was 5 months (1 month- 13.75 years), and that of diagnostic age was 5 years (2 months- 54.1 years), with a diagnostic delay of 33 months, on average. Seven patients were presented with acute diarrhea, 3 with oral candidiasis, and 2 with liver abscesses as the first chief complaints. Twenty-four cases (42.1%) had been complicated by gastrointestinal manifestations during their course of the disease. Of those, 12 cases (21.1%) had diarrhea, 7 (12.3%) oral candidiasis, 5 (8.8%) hepatitis, 4 (7.0%) hepatic abscess, and 2 cases (3.5%) gastric outlet obstruction. Also, failure to thrive was detected in 6 patients (10.5%). Four patients died (7%). CGD should be excluded in any patient with gastrointestinal manifestations especially chronic diarrhea, hepatic abscess, and gastric outlet obstruction.

Two related cases of primary complement deficiencies.

Primary complement deficiencies are rare and two related patients are reported here. The first patient is a 41- year- old man with eighteen episodes of pneumococcal meningitis and other purulent infections. The serum C3 level was checked at three separate times, showing that this was a primary C3 deficient case; other immunological tests were however normal. This patient now takes prophylactic antibiotics and the meningitis has not recurred, but he does have glomerulonephritis. The second case is a 40 - year-old woman with repeated episodes of orofacial and laryngeal edema and dyspnea. The serum C1INH levels were 4.3 to 7 mgldL which were very low compared with normal healthy subjects (C1INH was 40-50 mg/dL in ten normal controls) and C4 was lower than normal but other immunological tests were normal. Other causes of angioederna such as lymphoproliferative disorders were excluded. She had hereditary angioedema without a family background. The condition may be due to genetic mutation. The angioedema was controlled with Danazol and Stanasol. As our patients are related, this may suggest a genetic relationship between these two disorders.

Adverse effects of intravenous immunoglobulin therapy in patients with antibody deficiency.

Long-term intravenous immunoglobulin (IVIG) infusion is an effective treatment for children with humoral immunodeficiencies, already be complicated by systemic adverse effects. In order to determine the adverse effects of intravenous immunoglobulin in patients with antibody deficiency, 45 immunodeficient patients receiving intravenous immunoglobulin were studied during a 36 month period at Children's Medical Center. The investigated group included 25 patients with common variable immunodeficiency, 14 patients with X-linked agammaglobulinemia and 6 patients with IgG subclass deficiency. A total of fifty adverse effects occurred through 955 infusions (5.2%). The most frequent immediate adverse effects were mild (40 infusions out of 955) in 22 cases, including: chills, flushing, fever, nausea and headache. Three patients experienced moderate effects (10 infusions out of 955) such as rash, severe headache, joint pain and chest tightness. None of the effects was anaphylactic type. It can be concluded that intravenous immunoglobulin is generally a well-tolerated medical agent for patients with antibody deficiency, but all patients should be monitored by a physician who is familiar with its indications, risks, adverse effects and their appropriate management.