Ali Salavati

Gastrointestinal Manifestations in Patients with Common Variable Immunodeficiency

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.

Diagnostic Accuracy and Clinical Utility of Noninvasive Testing for Coronary Artery Disease

BACKGROUND Computed tomography coronary angiography (CTCA) has become a popular noninvasive test for diagnosing coronary artery disease. OBJECTIVE To compare the accuracy and clinical utility of stress testing and CTCA for identifying patients who require invasive coronary angiography (ICA). DESIGN Observational study. SETTING University medical center in Rotterdam, the Netherlands. PATIENTS 517 patients referred by their treating physicians for evaluation of chest symptoms by using stress testing or ICA. INTERVENTION Stress testing and CTCA in all patients. MEASUREMENTS Diagnostic accuracy of stress testing and CTCA compared with ICA; pretest probabilities of disease by Duke clinical score; and clinical utility of noninvasive testing, defined as a pretest or posttest probability that suggests how to proceed with testing (no further testing if < or =5%, proceed with ICA if between 5% and 90%, and refer directly for ICA if > or =90%). RESULTS Stress testing was not as accurate as CTCA; CTCA sensitivity approached 100%. In patients with a low (<20%) pretest probability of disease, negative stress test or CTCA results suggested no need for ICA. In patients with an intermediate (20% to 80%) pretest probability, a positive CTCA result suggested need to proceed with ICA (posttest probability, 93% [95% CI, 92% to 93%]) and a negative result suggested no need for further testing (posttest probability, 1% [CI, 1% to 1%]). Physicians could proceed directly with ICA in patients with a high (>80%) pretest probability (91% [CI, 90% to 92%]). LIMITATIONS Referral and verification bias might have influenced findings. Stress testing provides functional information that may add value to that from anatomical (CTCA or ICA) imaging. CONCLUSION Computed tomography coronary angiography seems most valuable in patients with intermediate pretest probability of disease, because the test can distinguish which of these patients need invasive angiography. These findings need to be confirmed before CTCA can be routinely recommended for these patients.The role of computed tomography coronary angiography (CTCA) for diagnosing coronary artery disease is uncertain. Weustink and colleagues compared the accuracy of stress testing and CTCA with that o...

Clinical, Immunological and Molecular Characteristics of 37 Iranian Patients with X-Linked Agammaglobulinemia

Background: X-linked agammaglobulinemia (XLA) is a hereditary immunodeficiency characterized by an early onset of recurrent bacterial infections, a profound deficiency of all immunoglobulin isotypes and a markedly reduced number of peripheral B lymphocytes. Eighty-five percent of the patients with this phenotype have mutations in Bruton’s tyrosine kinase (BTK) gene. Methods: To provide an informative outlook of clinical and immunological manifestations of XLA in Iran, 37 Iranian male patients with an age range of 1–34 years, followed over a period of 25 years, were studied. Twenty-four of the 37 patients were screened for BTK gene mutation using PCR-SSCP followed by direct sequencing. BTK protein expression assay was done by flow cytometry in 9 families. Results: All patients first presented with infectious diseases, the most common of which were respiratory tract infections. Eighteen different mutations were identified, 13 of which were novel: IVS1+5G>C, 1896G>A, 349delA, 1618C>T, 1783T>C, 2084A>G, 1346delT, 1351delGAG, 587A>G, IVS14–1G>A, IVS3+2T>C, 1482G>A, 1975C>A. Conclusion: The fact that we found a great number of novel mutations in a relatively limited number of patients underlines the heterogeneity of BTK mutations in the Iranian population. The large number of new mutations indicates that extended studies in this region would be rewarding.

Dual-source computed tomography angiography for diagnosis and assessment of coronary artery disease: Systematic review and meta-analysis

BACKGROUND Development of an accurate test for noninvasive assessment of coronary arteries has been highly desirable. OBJECTIVES We performed a systematic review of diagnostic accuracy of the dual-source computed tomography (DSCT) in the diagnosis of coronary artery disease (CAD). METHODS Eight medical databases were searched for articles published from January 2005 through March 2011. Studies compared DSCT coronary angiography (DSCT-CA) and invasive coronary angiography, as the reference standard, in consecutive patients with suspected or known CAD, and relevant data were extracted by 2 independent reviewers. Summary diagnostic accuracies were calculated, and the effect of covariates on the diagnostic performance was evaluated by meta-regression. RESULTS Twenty-five studies were included. In per-patient analysis (n = 2303), pooled sensitivity was 99% [95% confidence interval (CI), 97%-99%] with specificity of 89% (95% CI, 84%-92%). The summary positive (+LR) and negative (-LR) likelihood ratios were 8.6 (95% CI, 6.4-11.6) and 0.02 (95% CI, 0.01-0.03), respectively. In per-segment analysis (n = 32,615), pooled sensitivity was 94% (95% CI, 92%-96%) with specificity of 97% (95% CI, 96%--98%). Summary +LR and -LR were 30.2 (95% CI, 22.1-43.5) and 0.06 (95% CI, 0.04-0.08), respectively. CONCLUSIONS DSCT-CA seems to be robust to elevate heart rates while maintaining a high level of diagnostic performance.

Vitamin D insufficiency among children and adolescents living in Tehran, Iran.

Vitamin D is important for calcium absorption and skeletal growth. Vitamin D insufficiency (VDI) is a prevalent health problem in children. A study was performed to determine the prevalence of VDI in healthy children living in Tehran, Iran. In a cross-sectional study, 963 students (424 boys and 539 girls) aged 7-18 years were selected by random sampling. Serum 25-hydroxyvitamin D (25-OHD), calcium, alkaline phosphatase and phosphorus were measured. VDI was defined as serum 25-OHD <20 ng/ml. Prevalence of VDI was 53.6% in girls and 11.3% in boys. VDI in female students was about five times more common than males (p < 0.000001). VDI in children and adolescent girls is a health problem not only for these age-groups but also for the next generation to come. Encouraging girls to have more sun exposure, fortification of foods and prescription of supplemental vitamin D are recommended.

Evaluation of a New Antigen for Diagnosis of Helicobacter pylori Infection in Stool of Adult and Children

Background:  Nowadays, there is an increasing interest in noninvasive methods to diagnose Helicobacter pylori infection. Indeed, they can profitably replace endoscopy in predicting the diagnosis. The stool antigen test for H. pylori is a noninvasive immunoassay to diagnose active infection with this bacterium in human fecal samples. The aim of this study was detection of alkyl hydroperoxide reductase protein (AhpC) antigen by immunoblotting in stool samples for diagnosis of H. pylori.

Diagnosis of Helicobacter pylori infection by invasive and noninvasive tests

Although several invasive and noninvasive tests have been developed for the diagnosis of Helicobacter pylori infection, all of the tests have their limitations. We conducted a study to investigate and compare the suitability of rapid urease test (RUT), serology, histopathology and stool antigen tests with polymerase chain reaction (PCR) for detection of H. pylori, and correlate the diagnostic methods with PCR. Eighty nine patients (61 adults, 28 children) referred to the Firoozgar Hospital and Children Medical Center Hospital for diagnostic upper gastrointestinal endoscopy entered to the study and noninvasive tests such as immunoassay for serological antibodies against H. pylori and detection of its antigen in feces were measured. The biopsies were utilized for histological examination, RUT and PCR. The H. pylori statuses were evaluated by the positivity of ureC PCR in biopsy specimens and 53 subjects had H. pylori positive result. Histopathology showed high overall performance in adults and children with sensitivity and specificity 100% and 90%, respectively. Sensitivity, specificity, and accuracy for stool antigen test were 87.8%, 75% and 82%, respectively. Correlation of RUT, serology (IgG), histopathology and stool antigen tests with PCR were 0.82, 0.32, 0.91 and 0.63, respectively. In conclusion, the RUT and histopathology are as accurate as the PCR of biopsy and stool antigen test can consider as appropriate noninvasive test for detection of H. pylori infection.

Invasive aspergillosis in chronic granulomatous disease : report of 7 cases

Keywords Chronicgranulomatousdisease.CGD.InvasiveaspergillosisChronic granulomatous disease (CGD) is an inherited dis-order of the NADPH oxidase complex characterized byrecurrent bacterial and fungal infections. The underlyingdefect is an inability to generate reactive oxygen intermedi-ates and activation of intracellular proteases, which are keyelements in host defense [5]. The incidence of fungalinfections in CGD has been reported to be 20%, withAspergillus spp accounting for 78% of them [2]. We reportseven CGD patients with invasive aspergillosis (IA) anddescribetheirclinicalpicture(Table1). The diagnostic criteriafor CGD were abnormal nitroblue tetrazolium test (NBT),and/or dihydrorhodamin 123 (DHR) assay. The X-linkedform of CGD was suspected by the pattern of inheritance,abnormal NBT or mosaic DHR pattern in mothers.The mycological diagnosis was determined by micros-copy and culture of clinical samples.The median age at onset of CGD was 5 months (range2–24). The fungal infection appeared at the median age of72 months. In P4, aspergillosis was the first presentation.All patients presented febrile with the mean erythrocytesedimentation rate (ESR) of 80 mm/hr. Uncertain responsesto antibiotics and infiltrations on X-ray or CT-scan were thefirst indication of a fungal infection.The sites of infection were lungs, chest wall, brain, liverand lymph nodes. In P2 and P6, costal osteomyelitisresulted from contiguous spread of pulmonary and hepaticinfections, respectively.In five patients A. fumigatus was isolated whereas P5was affected by A. flavus. Deoxycholate amphotericin B(1 mg/kg/day) was the first line treatment. Itraconazole (5–10 mg/kg/day) and flucytosine (100 mg/kg/day) were usedas adjunctive. Prophylactic itraconazole reduces the risk offungal infections in CGD [3] but none of our patientsreceived it.Surgical debridement was used for all but P2. Threepatients recovered, and discharged on oral itraconazole.Other patients died because of an uncontrolled infectiousprocess. In P6 amphotericin B therapy led to an initialimprovement, however; he deteriorated within two monthsand passed away.IA is the most important cause of mortality in CGD [1]and A. fumigatus is the most frequent inciting organism [7].All of our patients presented during the first decade of lifewhich is lower than a previous study [8]. Although X-linked CGD is thought to be more susceptible to IA [7], ourresults show that autosomal forms are prone as well.

Pubertal development in a random sample of 4,020 urban Iranian girls.

BACKGROUND Entering puberty is an important milestone of human growth and maturation associated with marked physiological and psychological changes. AIM To assess normal pubertal development in Iranian girls to define normal and precocious puberty. METHODS The present study included a cross-sectional sample of 4,020 normal Iranian girls living in Tehran, aged from 6-20 years. Pubertal assessment was made according to Tanner staging. The mean ages of attaining secondary sexual characteristics were estimated using probit analysis. Weight and height were measured and body mass index (BMI) was calculated. RESULTS The mean age at breast bud stage (B2) was 10.15, pubic hair stage (PH2) was 10.48, and menarche was at 14.54 years. The mean age at attainment of puberty was compared with those of other populations. CONCLUSION Girls of the present study started pubertal development at an age similar to other populations but reached menarche later. Differences between the present study and other studies can be attributed to various genetic, ethnic, geographical, and nutritional factors.

Echocardiographic abnormalities and their correlation with bronchiectasis score in primary antibody deficiencies.

Background Primary antibody deficiencies are characterized by defective antibody production and recurrent infections. Patients usually present with recurrent respiratory tract infections with consequent chronic pulmonary damage and bronchiectasis, which could potentially influence cardiac function. Our aim was to assess noninvasively the cardiac complications due to pulmonary disease in patients with primary antibody deficiency. Methods A cross-sectional series of patients with primary antibody deficiency syndromes from our referral immunology center were recruited. Individuals undergoing high-resolution computed tomography (HRCT) and transthoracic echocardiography were reviewed. Results Thirty primary immunodeficient patients aged 5–55 years of age (21 males and 9 females) were enrolled in this study. Half of the patients (50%) were found to have bronchiectasis in HRCT imaging. In echocardiographic examination, 20 patients (67%) had at least one abnormality; among which pulmonary hypertension was the most common (33%). Patients with bronchiectasis had higher pulmonary artery pressures and HRCT bronchiectasis score was strongly correlated with pulmonary artery pressure (regression R = 0.59, P value = 0.001). Conclusion Echocardiographic evaluation of right ventricular function and noninvasive estimation of pulmonary artery pressure could have an important diagnostic role in the follow-up and therapeutic management of patients with primary immune deficiency.