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.

This study focuses on endoscopic and pathologic alterations of gastrointestinal (GI) disorders of Iranian patients with common variable immunodeficiency (CVID). Nineteen of 39 CVID patients (48%) had GI complaints. The most common symptom was chronic diarrhea (28%). In endoscopic examination of small intestines, 15 patients had no abnormal finding. Duodenal biopsy revealed villous atrophy in eight and nodular lymphoid hyperplasia in three patients. There was no statistically significant difference between patients with and patients without duodenal villous atrophy regarding the presence of chronic diarrhea, anemia, and absolute CD4+T cells. In three patients, biopsies of the colon showed chronic noncrypt-destructive colitis. GI problems pose a high morbidity to CVID patients and are second only to respiratory complications. CVID patients are at increased risk of infectious and inflammatory conditions in the GI tract. Early diagnosis of these complications improves the quality of life and well-being of patients.

The prevalence of asthma in Iran was similar to other Asian and European countries. However, repeated national surveys are required to determine the trend of asthma prevalence in Iran in comparison to other countries.

Adverse Drug Reactions (ADRs) and drug allergies- as a subset of ADRs- make a significant public health concern, complicating 5 to 15% of therapeutic drug courses. They may result in diminished quality of life, increased physician visits, health care costs, hospitalizations, and even death. The incidence of serious ADRs in hospitalized patients was estimated to be 6.7% and for fatal ADRs to be 0.32%, so recognizing and taking action on ADRs is an important aspect of medication management. Allergic reactions to drugs refer to those ADRs that involve immune mechanisms which account up to 15% of ADRs and can be identified as being a type I through IV immune reaction that the most common immunologic mechanism is IgE-mediated- type I reaction. Clinical manifestations of allergic reactions range from pruritus and rash to serious reactions such as systemic anaphylaxis and cardiovascular emergencies and they are responsible for 2-3% of hospitalized patients. Health professionals should be aware of the ADRs presenting clinical features and the risk factors and should be able to differentiate between allergic and non-allergic adverse drug reactions. This will lead to increased opportunities to review drug selection and prescribing practices affecting patients' outcome. This article will review the definition and estimated incidence, the features, classification and types of ADRs and drug allergies and related patents. It will highlight the role of detecting, reporting, and assessing suspected ADRs and drug allergies in the most clinically relevant drugs group. Priorities in the evaluation and management of the conditions of patients who have experienced allergic and non-allergic drug reactions also will be discussed.

No abstract

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.

This hot spot is flanked by "direct repeats" of nucleotides, which are previously shown to be associated with areas of recurrent small deletions.

Pediatric patients with leukocyte adhesion deficiency type-I (LAD-I) experience severe and recurrent lifethreatening bacterial infections with failure of pus formation and delayed wound healing. LAD-I is a rare inherited disease caused by mutation in the leukocyte CD18 integrin expression, resulting in defective adherence and migration of leukocytes, in particular neutrophilic granulocytes through the intravascular space. Hematopoietic SCT is the only curative treatment option available to patients with LAD-I. Since 2007, in a prospective trial, reduced-intensity conditioning regimen have been developed for 10 consecutive patients with LAD-I who were referred to our center. Based upon available data, it is the first time that such a number of patients affected by LAD-I have been treated with this regimen. This study attempts to show that reduced-intensity regimen leads to a favorable result in LAD-I patients even in those who have experienced comorbid complications. Following transplantation, some patients develop mixed chimerism, however, in our study mixed chimerism was not followed by transplant rejection.