Archives of Iranian Medicine
Volume:15 Issue: 6, Jun 2012

Several studies have shown the prevalence of celiac disease (CD) to be around 1% in Iran, which is similar to the worldwide prevalence. There is scant information on occult CD in apparently healthy school age children. This study, as the first such study in Iran, aims to determine the prevalence of occult CD in healthy Iranian school age children. In this cross-sectional study, we screened healthy school age children for CD by serum IgA and IgA anti-tissue transglutaminase antibody (tTG) levels. Measurement of these antibodies was by enzyme linked immunosorbent assay. A recheck of positive tTG tests was performed and patients who tested positive underwent endoscopic duodenal biopsies. The biopsy samples were scored according to the Marsh classification by an experienced pathologist. A total of 634 children (314 males, 320 females; mean age: 12.8 years) were included in the study. All children and/or their parents completed a questionnaire and children underwent an initial physical examination to determine study eligibility. Positive serum tTG was noted in 3 (0.5%; 2 females) out of 634 patients. Duodenal biopsies were consistent with CD in these 3 subjects. The mean age of patients with CD was 14.3 years (range: 12–17 years). The female to male ratio was 2:1. These cases had no signs and symptoms, but a gluten-free diet was recommended according to pathologic changes in their small bowels and results of the tTG test. The prevalence of occult CD in these children is 0.5%, which is half of the prevalence of CD in Iranian adults. The anti-tTG concentration at initial serological CD screening is highly informative in determining occult cases of CD. The question is whether all non-symptomatic cases should be treated with a gluten-free diet or not.

Although fast food consumption has drastically increased in Iran in recent years; there is a paucity of data in relation to the association between fast food consumption, dietary intake, and cardiovascular risk factors. This study aims to determine fast food consumption status among young and middle-aged Iranian adults, and to assess its impact on dietary intake and cardiovascular disease (CVD) risk factors. This cross-sectional population-based study was conducted on 1944 young and middle-aged adults (840 men and 1104 women), who participated in the Tehran Lipid and Glucose Study (2006–2008). We collected dietary data by using a validated 168 item, semi-quantitative food frequency questionnaire. Total fast food consumption was calculated by summing up weekly consumption of the most commonly consumed fast foods in Iran. Mean consumption of fast food was 161g/week (95% CI: 147–175) for young adults and 108 g/week (95% CI: 101–115) for middle-aged adults. Mean dietary intakes of energy, fat, saturated fat, cholesterol, sodium, meat, and soft drinks increased significantly (P < 0.05), while carbohydrate and refined grain consumption decreased (P < 0.01) across tertiles of fast food in both age groups. In young adults, dietary energy density and protein intake increased significantly (P < 0.01) where as intake of non-starchy vegetables and carotenoids decreased (P < 0.05). In middle-aged adults dietary intakes of fiber, folate, calcium, and fruits significantly decreased across fast food tertiles (P < 0.05). After adjustment for confounders, there was an association between fast food consumption and body mass index (BMI; β = 0.104; P < 0.01) and waist circumference (WC; β= 0.083; P < 0.01) in young adults, and serum triglycerides (β = 0.072; P < 0.05), high density lipoprotein cholesterol (HDL-C; β = -0.051; P < 0.05), and atherogenic index of plasma (β = 0.056; P < 0.05) in middle-aged adults. The results show that increased consumption of fast foods is associated with poor dietary intake and some of the CVD risk factors in Iranian adults.

Acute lymphoblastic leukemia (ALL) is a cancer of the white blood cells most commonly found in childhood with a peak incidence at 2–5 years of age. The ubiquitin degradation pathway facilitates degradation of damaged proteins and regulates the growth and stress response. This pathway is activated in various cancers, including ALL. It has been previously reported that the newly characterized human gene UBE2Q2, a putative member of the ubiquitin-conjugating enzyme family, is over-expressed in the tumor mass and invasive epithelium in head and neck squamous cell carcinoma and breast cancer. Here, we have used quantitative reverse transcriptase polymerase chain reaction (RT-PCR) to assess expression of the UBE2Q2 gene in bone marrow samples of 20 children with ALL. Whole blood samples of 20 normal children were used as control specimens. RT-PCR revealed the expression of UBE2Q2 mRNA in 80% of the bone marrow samples from ALL patients as well as in 85% of leukemic normal peripheral blood cells. According to the results of quantitative RT-PCR, the levels of UBE2Q2 mRNA expression in the bone marrow cells of 11 out of the 20 children with ALL (55%) were significantly higher (> 2–47 fold) than those in blood cells of normal children. Our data suggest that the newly characterized human gene, UBE2Q2, may have implications for the pathogenesis of ALL and could be used for molecular diagnosis purposes in the future.

In Iran, the number of stray dogs in cities and villages necessitates epidemiologic investigations. This study has undertaken an epidemiologic survey in Ilam, Iran with regard to animal bites during 1999–2009. This was a cross-sectional descriptive study. The data due to animal bites was collected from patients and analyzed. The number of animal bites reported was 4420, which included 3032 men (68.3%) and 398 women (31.7%). Most animal bites were reported in the 10–19 year-old (1172, 26%) age group. The feet were the most commonly attacked body part, which was reported in 3177 cases (71.8%). Most bites were from dogs (3942 cases, 89.15%). Of cases, 3419 (77.3%) resided in rural areas while 1001 (22.7%) were urban residents. The number of patients with incomplete vaccinations was 3596 (81.3%) compared to 824 (18.7%) completely vaccinated patients. The present study showed a high incidence of animal bites in Ilam, which necessitates the importance of rabies prevention and control. It is recommended that the sanitation authorities provide for and implement measures to determine beneficial ways to avoid and control rabies infection in this part of Iran.

Mutations in the human aristaless-related homeobox (ARX) gene are amongst the major causesof developmental and neurological disorders. They are responsible for a wide spectrum of phenotypes, including nonsyndromic X-linked intellectual disability (NS-XLID), and syndromic (XLIDS) forms such as X-linked lissencephaly with abnormal genitalia (XLAG), Partington syndrome (PRTS), and X-linked infantile spasm syndrome (ISSX). The recurrent 24 bp duplication mutation, c.428_451dup(24 bp), is the most frequent ARX mutation, which accounts for ~40% of all cases reported to date. We have screened the entire coding sequences of the ARX gene in 65 Iranian families with intellectual disabilities in order to obtain the relative prevalence of ARX mutations.At first these families were screened for the most recurrent mutation, the c.428_451dup(24 bp). For samples with negative results, single strand conformation polymorphism (SSCP) analysis was performed. We identified one family with the c.428_451dup(24 bp) duplication. Three shifts (one shift in exon 5 and two shifts in exon 4) were also identified among the total families. According to the results of the sequencing analysis, two shifts were not associated with any mutation and the other one was a c.1347C>T (p.G449G) substitution in exon 4. Hence, we suggest that molecular analysis of ARX mutations as a second cause of XLID should be considered as routine diagnostic procedure in any male who presents with either NS-XLID or XLIDS.

Evaluation of estrogen (ER) and progesterone (PR) receptors is important in the management and prognosis of breast cancer patients. Immunohistochemistry (IHC) is currently the worldwide accepted methodology for detection of ER/PR receptors in breast carcinomas. However, technical artifacts may alter the results. Since most authorities believe that there are no true ER-negative/PR-positive breast tumors, therefore we hypothesized that technical artifact in IHC might cause ER-negative/PR positive cases. The clinical records of 2432 patients treated by surgery at six community hospitals for different histologic subtypes of breast carcinoma were reviewed. Among them, 43 (1.8%) patients reported as ER-negative/PR-positive were re-evaluated in a reference laboratory. Expressions of ER and PR were evaluated by IHC on the same paraffin block used for the initial testing. The repeat study showed that of the 43 patients with the initial results of ER-negative/PR-positive, 24 (55.8%) were ER-positive/PR-positive, 15 (34.9%) were ER-negative/PR-negative, and 4 (9.3%) were ER-positive/PR-negative. In none of the 43 cases were the initial results (ER-negative/PR-positive) confirmed. Technical artifacts in IHC may alter ER/PR results in breast carcinomas. The technical factors affecting steroid receptor IHC ought to be properly controlled to provide reliable results.

The aim of this study was to determine the prevalence of musculoskeletal disorders (MSDs) and ergonomic hazards and their relationship among Iranian physicians who work in teaching hospitals. A self-administered questionnaire was provided to 405 physicians in four teaching hospitals. The questionnaire had three major parts: the first part gathered individual and work-related data, the second was a modified version of the Standardized Nordic questionnaire for musculoskeletal symptoms, and the last part evaluated the duration of exposure to ergonomic hazards at work. Knee pain (19.8%) was the most common complaint among physicians, followed by low back (15.1%) and neck pain (9.8%). A total of 169 physicians (41.7%) reported symptoms in at least one part of their bodies. Prolonged sitting, standing, and neck flexion were the most common reported ergonomic hazards among participants. Multiple logistic regression analysis reported statistically significant associations for the outcomes of knee pain and symptoms in any part of the body with the work-related factors of years of employment and work hours per shift. The prevalence of musculoskeletal complaints among physicians was low, less than other health care workers, but similar to those reported in the general population. These musculoskeletal complaints were, however, associated with traditional work-related and ergonomic factors.

Liver cirrhosis is associated with different types of electrophysiological changes, including QT prolongation, which may adversely affect long-term prognosis of these patients. The aim of this study is to evaluate the effect of orthotopic liver transplantation (LT) on corrected QT (QTc) interval and QT dispersion (QTd) in cirrhotic patients of various etiologies. We enrolled 249 patients with end-stage liver disease between 2004 and 2009 at Shiraz Transplant Research Center, Shiraz, Iran. The QTc interval and QTd were measured by 12 lead ECGs for baseline and at 3 months after LT. Mean QTc interval and mean QTd were calculated. A QTc interval above 440 ms was considered abnormal. Within 3 months following surgery, 6 patients died. There were 105 patients (43.2%) with prolonged QTc before transplantation; in 91 (86.6%) patients, the mean QTc normalized after transplantation (baseline: 490.9 ± 45.74 ms; post-transplantation: 385 ± 48.74 ms; P < 0.0001). Fourteen patients (13.3%) had evidence of some shortening of the QTc interval although the QTc remained above the upper limit of normal. Prolongation of the QTc interval in cirrhotic patients was independent of the etiology of cirrhosis. A normal QTc was seen in 138 patients (56.7%) before transplantation, of which 4 (2.9%) developed prolonged QTc after transplantation. The mean QTd decreased significantly after transplantation (baseline: 30 ± 20 ms; post-transplantation: 30 ± 10 ms; P < 0.0001). Many cirrhotic patients have prolonged QTc intervals before LT regardless of disease etiology. In the majority of patients this value returns to normal after LT, suggesting that liver cirrhosis has independent unfavorable, but reversible electrophysiological effects.

Early-onset multiple sclerosis (EOMS) is defined as the first presentation of symptoms in childhood (before the age of 16 years). EOMS occurs in about 0.4% to 10.5% of multiple sclerosis (MS) patients. In this retrospective population-based study we aimed to describe the clinical/paraclinical details and frequency of epileptic seizures in Iranian EOMS patients registered with the Isfahan Multiple Sclerosis Society (IMSS) from April 2003 to July 2010. EOMS cases were extracted from the Isfahan total MS cohort and included 3522 patients. A total of 117 EOMS patients (19 males and 98 females) with a mean age at onset of 14.2 ± 2.0 years (range: 7–16 years) were extracted from our database (3.3% of the total cohort). Of cases, ten (one male and nine females) had experienced at least two epileptic seizures, providing a crude prevalence of 8.5%. The frequency of epilepsy in EOMS patients (3.3%, 10/117) was significantly greater (P < 0.001) than that of non-EOMS cohort (2.0%, 71/3405). Epileptic seizures occurred before MS onset in two patients, after MS onset in seven, and at MS onset in one as the presenting symptom of the disease. Our findings mostly indicate an excessive prevalence of epileptic seizures in Iranian EOMS patients (8.5%), which is higher than any other report concerning seizures or epileptic seizures in a large MS series. These findings might be supportive of increased prevalence of epileptic seizures in EOMS.

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by hamartomatous involvement of multiple organs such as the skin, central nervous system, kidneys, lungs, and heart. A linkage has been found with a locus on the long arm of chromosome 9 (9q34) and with a locus on the short arm of chromosome 16 (16p13). TSC has a birth incidence of 1/6000. Children with TSC are almost universally born with normal kidneys, but cystic disease and angiomyolipomas develop with increasing age. Angiomyolipomas, renal cysts, and renal cell carcinoma are classical features of renal involvement in TSC. Renal complications are the most common cause of death in adult TSC patients, thus renal involvement has a crucial importance on the course of this disease. We present a 27-year-old patient previously diagnosed as tuberous sclerosis complex and referred with acute renal failure and polycystic kidney disease.

Compartment syndrome is a rare, devastating complication of coronary artery bypass grafting (CABG) and intra-aortic balloon pump (IABP). Prompt diagnosis is based on symptoms and signs and is paramount for limb rescue. This report describes a CABG patient with IABP in whom receiving continuous analgesia-sedation obscured the symptoms of compartment syndrome.