Archives of Iranian Medicine
Volume:11 Issue: 3, May 2008

A specific chromosomal abnormality, the Philadelphia chromosome, is present in 90 – 95% of patients with chronic myeloid leukemia. The aberration results from a reciprocal translocation of chromosomes 9 and 22, creating a BCR-ABL fusion gene. There are two major forms of the BCR-ABL fusion gene, involving ABL exon 2, but including different exons of BCR gene. The transcript b2a2 or b3a2 codes for a p210 protein. Other fusion gene leads to the expression of an e1a2 transcript, which codes for a p190 protein. Other less common fusion genes are b3a3 or b2a3 (p203) and e19a2 (p230). The incidence of one or other rearrangement in chronic myeloid leukemia patients varies in different reports. In general, fusion transcripts are determined individually, a process which is labor- intensive in order to detect all major fusion transcripts. The objective of this study was to set up a multiplex RT-PCR assay for detection and to determine the frequency of different fusion genes in 75 Iranian patients with chronic myeloid leukemia. Peripheral blood samples were analyzed by multiplex RT-PCR from 75 adult Iranian chronic myeloid leukemia patients to detect different types of BCR-ABL transcripts of the t(9;22). All patients examined were positive for some type of BCR/ABL rearrangement. The majority of the patients (83%) expressed one of the p210BCR-ABL transcripts (b3a2, 62% and b2a2, 20%), while the remaining showed one of the transcripts of b3a3, b2a3, e1a2 or co-expression of b3a2 and b2a2. The rate of co-expression of the b3a2 and b2a2 was 5%. In contrast to other reports, we did not see any co-expression of p210/p190. Co-expression may be due to alternative splicing or to phenotypic variation, with clinical course different from classic chronic myeloid leukemia.

D2 dopamine receptor gene has been reported to be one of the most relevant candidate genes in schizophrenia. In this study, we investigated the association between TaqIA and TaqIB dopamine D2 receptor polymorphisms and psychopathology of schizophrenia. The study subjects were 38 acutely exacerbated schizophrenic patients who were all Iranian descent. The control population consisted of 63 healthy individuals with almost the same age as patients and were also of Iranian decent. The TaqIA and TaqIB genotypes, the A1 and A2 alleles, and the B1 and B2 were determined by restriction fragment length polymorphism of the amplified DNA fragments by polymerase chain reaction. For each polymorphism (A or B) the patients were categorized according to their genotype into three groups; i.e. the patients with alleles A1/A1, A1/A2, A2/A2; B1/B1, B1/B2, and B2/B2. No significant association was found between Taq1A or Taq1B gene polymorphisms and schizophrenia in patients compared to the controls. When study subjects were stratified according to their gender, the distribution of the A1/A1 genotype did was significantly different in both men and women (patients vs. controls). Our findings show that there is no genetic association between Taq1A and Taq1B gene polymorphisms and schizophrenia. Further clinical studies should be conducted to confirm and further evaluate these findings

There are few reports in the scientific literature on the factors taken into account by editors in deciding to accept or reject a scientific paper. The purpose of the present study was to investigate the effects of different factors on the journal editors’ decisions on whether to accept or reject the manuscripts submitted to their journals. We randomly selected the participants from the authors of original articles and case reports published in six medical journals, and sent them a questionnaire by e-mail. We analyzed the scores they gave to each of the 17 items of the questionnaire. One hundred and nineteen of the authors responded to our survey. The scores given by the respondents were analyzed comparing authors of developing and developed countries. Also, the results from authors of high- impact journals were compared with those with a low-impact factor. Multidimensional scaling was used to categorize the items based on their average scores. Highest scores were given to items addressing the quality of study performance, those addressing manuscript writing, and to the role of statistical significance of the results in the probability of studies getting published. Authors still believe in the existence of publication bias. They estimate its role to be comparable with the role of the quality of study performance and reporting. Our study also proves the presence of developing country bias, from the authors’ perspective.

Physical disability and depression in patients with chronic pain have been shown to be associated with pain intensity and pain self-efficacy beliefs. However, little is known about whether pain self-efficacy beliefs can predict depression and physical disability when this relationship is controlled for pain intensity and other related demographic variables The aim of the current study was to replicate and extend previous research on the relationship between pain-related beliefs, depression, and disability by examining these relationships in a heterogeneous sample of Iranian patients with chronic pain. A group of 430 patients with chronic pain participated in the study and completed questionnaires on demographic variables, pain intensity, pain self-efficacy beliefs, physical disability, and depression. Correlation analyses revealed that patients with higher education were less depressed and less physically disabled. Younger patients were more physically disabled. Pain intensity and pain self-efficacy beliefs were significantly related to physical disability and depression. In hierarchical multiple regression analyses, after controlling for patients’ background variables and pain intensity, pain self-efficacy beliefs accounted for significant variance in depression and physical disability over and above the effect of demographic variables and pain intensity. Patients with higher pain self-efficacy, compared to those with lower self-efficacy, were less depressed and less physically disabled. Pain self-efficacy was more strongly related to depression and physical disability than pain intensity and demographic variables. The findings of the present study suggest the importance of targeting pain self-efficacy beliefs for modification in treatment of patients with chronic pain

The treatment of recurrent anterior shoulder dislocation in patients who failed a supervised rehabilitation program is operative stabilization. Anatomical repair addressing the underlying pathology is the preferred method. We hypothesize that Bristow-Latarjet procedure is effective in all types of traumatic recurrent anterior shoulder dislocations, although in cases with Bankart lesion, Bankart operation is certainly preferred. Thirty-five shoulders on which a Bristow-Latarjet operation had been performed on account of traumatic recurrent anterior shoulder instability were followed up for an average of 24.6 months (range: 18 – 51). The clinical outcome was measured according to Walch-Duplay Rating Sheet for Anterior Instability of the Shoulder at the latest follow-up. The clinical outcome was excellent in 11 (31%) patients and good in 24 (69%) patients. Two patients had redislocation that were treated nonoperatively and remained symptom-free at the latest follow-up. Radial nerve palsy occurred in one patient that spontaneously recovered after nine weeks. Although the treatment of anterior shoulder instability in patients with Bankart lesion and intact capsular material (without excessive laxity) is certainly Bankart operation, we claim that in almost all types of anterior shoulder instability, especially in patients with large Hill-Sachs lesions, glenoid bone loss, or excessive capsular laxity, the Bristow-Latarjet operation is associated with good or excellent results and can make the patient satisfied.

Obesity continues to be an important public health problem worldwide. The objective of this study was to determine the association of body mass index and abdominal obesity with current marital status among the adult population of Iran. A nation-wide cross-sectional survey was conducted from December 2004 through February 2005.The subjects were selected by stratified probability cluster sampling through household family members in Iran. Weight, height, waist circumference, and marital status of 89,404 men and women aged 15 – 65 (mean: 39.2) years were recorded. Four classes of body mass index, i.e., <18.5, 18.5 – 24.9, 25 – 29.9, and ≥30 kg/cm2, and three marital status, i.e., currently-, formerly-, and never-married were used. Abdominal obesity was defined as waist circumference ≥102 cm in men and ≥88 cm in women. The prevalence of overweight was twofold higher in married men (OR: 2.24; 95% CI: 2.08 – 2.41) and women (OR: 2.36; 95% CI: 2.20 – 2.53) than never-married men and women, even when age, educational level, leisure time physical activity, smoking habits, and place of residence were controlled. The multivariate OR of obesity was increased about threefold in married men (2.82; 95% CI: 2.51 – 3.18) and women (3.64; 95% CI: 3.31 – 3.99). The prevalence of abdominal obesity was twofold higher among married men (2.02; 95% CI: 1.79 – 2.29) and about threefold higher among married women (2.87; 95% CI: 2.69 – 3.06). The marital status appears to influence the likelihood of developing overweight, obesity, and abdominal obesity in both men and women in Iran.

Incisional hernias are common and recurrence after repair has been reported in up to 44% of patients. Large incisional hernias of the abdominal wall represent substantial defect of supportive tissues. Twenty-nine patients with large incisional hernias underwent surgery from January 2003 through December 2005. Herein, we presented our experience in closure of large incisional hernias using a technique in which we combine a fascia with a prosthetic repair. The variables recorded were classified as patient-related (gender, age, obesity, cough, constipation, diabetes mellitus, glucocorticosteroid therapy, smoking habit, and abdominal surgical history) and operation-related factors (size of defect, recurrence, wound infection, hematoma, and duration of hospital stay). The repair was performed for 25 midline hernias and four large incisional hernias in the right subcostal region. Four patients were females and 25 were males with a mean age of 52 (range: 30 – 77) years. The mean size of fascial defect was 12.7x4.5 cm. The mean time of operation was three hours and 18 minutes. The mean hospital stay was six (range: four to ten) days. Two patients had recurrence during the follow-up period. The mean follow-up period was 16 (range: eight to 26) months. The combined three-layer fascia and mesh repair can be successfully used for large incisional hernias.

In the present study, the effect of dextromethorphan on antinociception and tolerance induced by water swim-stress in the formalin test was investigated. Swim- stress at 8ºC induces antinociception in both phases of the formalin test. Intraperitoneal administration of dextromethorphan (60 mg/kg) also induces antinociception in the second phase of the formalin test. The lower doses of dextromethorphan (1.25, 2.5, and 5 mg/kg) which did not induce antinociception alone, but did so in combination with swim- stress (40 second), showed antinociceptive effect in both phases of the test. Exposure to water swimming stress with a period of 20 sec, once daily for three days, altered swim- stress- induced antinociception in the formalin test, when tested on the fourth day. In these animals, exposure to either water swimming stress alone or water swimming stress in combination with dextromethorphan showed potentiation of antinociception induced by swim- stress up to 20 second and decreased the response induced by 40 and 60 second swim-stress, indicating a tolerance induction. Dextromethorphan (20 mg/kg) did not alter the changes induced by three days exposure to swim-stress. The results may indicate a possible involvement of n-methyl-d-aspartate receptor mechanism in the antinociception but not tolerance induced by swim- stress at 8ºC.

Smoking has not only enormous deleterious effects on cardiovascular, cerebral, and bronchial organs but also profoundly alters the function of all parts of the gastrointestinal tract through various mechanisms. Except the sole curiously observed benefit of smoking on the course of ulcerative colitis, it increases the prevalence of the common gastrointestinal diseases namely gastroesophageal acid reflux, peptic ulcer, and Crohn’s diseases. It also increases the incidence of cancer of oral cavity, esophagus, stomach, pancreas, and liver mostly in a dose-response relationship and worsens the prognosis of colon cancer. The cessation of smoking is associated with the reduced incidence of cancer in the reported organs, but its effect on the regression of benign disease is not generally studied. The physicians must be aware not only of the harmful effect of smoking on the cardiovascular and bronchial systems, but also about the detrimental consequences of life-long smoking on the gastrointestinal tract and the increase of its benign and malignant diseases.

Pharyngocutaneous fistula is a common and troublesome postoperative complication after total laryngectomy. The objective of this report was to determine the incidence, predisposing factors, and outcome of postlaryngectomy pharyngocutaneous fistula in patients operated on in our department and to describe the management of the complication.The medical records of 146 consecutive patients who underwent laryngeal surgery for squamous cell carcinoma of the larynx between 1990 and 2005 were assessed. All patients had similar preoperative/postoperative care. We studied a number of factors that could influence fistula formation such as age, gender, smoking, systemic disease, preoperative radiotherapy, previous tracheotomy, site of tumor, surgical procedure, positive surgical margins, type of closure (T vs. vertical), concurrent neck dissection, suture material, clinical stage, histologic grade, and experience of surgeon (consultant vs. resident). A pharyngocutaneous fistula was observed in 13% (19/146) of the patients within a mean time of 9.6 days from surgery. Spontaneous closure with local wound care was noted in 17 (89%) patients whereas a surgical closure was necessary in two. One patient required surgical closure by direct suture of the pharyngeal mucosa. Pectoralis major myocutaneous flap was used in another one. Our findings showed that fistula formation was significantly more common in patients who received previous radiotherapy or who had positive surgical resection margins or had a systemic disease. The mean healing time was 26 days. We concluded that pharyngocutaneous fistula remains a troublesome complication of the early postoperative period after total laryngectomy. There are many conflicting reports in the literature concerning the predisposing factors, but our data showed that the presence of systemic diseases, previous radiotherapy, and positive surgical margins can all be important predisposing factors, or at least underlying causes. Our experience confirmed that most fistulas can be successfully managed with conservative treatment

Cardiovascular diseases are the major causes of death in Iran. The aim of this study was to determine the prevalence of conventional risk factors for coronary artery disease in Lor migrating tribes in southern part of Iran. Two hundred six persons of Lor migrating tribes in Mamasani, southern Iran (age range, 21 – 80 years) were randomly enrolled in the study. Their serum total cholesterol, triglyceride, high- density lipoprotein, low- density lipoprotein, and fasting blood sugar were determined. Of the participants, 79.9% were smokers. The prevalence of hypertension was 37.4% (mostly grade 1), 3.6% had diabetes mellitus, and 14.7% had impaired fasting glucose. A cholesterol level of more than 240 mg/dL was found in 7.1% of the participants, 27.8% had a low- density lipoprotein level of more than 130 mg/dL, 38.9% had a high- density lipoprotein level of less than 40 mg/dL, and 25.2% had a triglyceride level of more than 200 mg/dL. Body mass index greater than 25 was found in 34.8% of the participants, and 57.1% had intermediate and high risk for coronary events. The prevalence of conventional risk factors for coronary artery disease in Lor migrating tribes in comparison with Tehran urban population was very high.

Infiltrative dermopathy is an uncommon manifestation of Graves'' disease. The most frequent location of infiltration is the lower extremities, especially the pretibial areas and on the dorsum of the foot. Rarely the hand, elbow, arm, and forearm are affected. We report a 66-year-old man with Graves'' disease of nine years duration who presented with dermopathy and nonpitting edema of the toe with no involvement of the pretibial portion since four years ago. Radiologic studies of the foot were normal, without subperiosteal bone formation or reaction. Skin biopsy was consistent with pretibial myxedema. This presentation of myxedema and limitation to a localized area for several years is very unusual.

Hereditary vitamin D-resistant rickets type or vitamin D-dependent rickets type II is a genetically determined and rare autosomal recessive disorder, most often caused by mutations in the vitamin D receptor gene. It usually presents with rachitic changes not responsive to vitamin D treatment and the circulating levels of 1,25 (OH)2 vitamin D-3 are elevated, differentiating it from vitamin D-dependent rickets type I. Alopecia capitis or alopecia totalis is seen in some families with vitamin D-dependent rickets type II. This is usually associated with a more severe phenotype. In this report, we present the clinical findings on a family which exhibited the typical clinical features of hereditary vitamin D-resistant rickets in two siblings. In addition, molecular analysis of the vitamin D receptor gene was performed by sequencing all coding exons. The cardinal findings in the index patient were alopecia totalis, renal tubular acidosis, mild generalized aminoaciduria, refractory rickets, high alkaline phosphatase, and hyperparathyroidism. Other routine biochemical tests were within normal limits, but 1+ glycine was detected in his urine. Skin biopsy results were compatible with alopecia areata. A previous child with similar phenotype was reported to be deceased at the age of 32 months. Mutation analysis of the vitamin D receptor gene by direct sequencing analysis of all coding exons showed a homozygous c.122GàA(p.Cys41Tyr) variant in exon 2 with several arguments pointing to a pathogenic effect. We should be aware of this very rare disease whenever we see a patient with refractory rickets and alopecia.

Neonatal diabetes mellitus, which is defined as hyperglycemia presenting within the first six weeks of life, is a rare disorder. It may result in transient or permanent disease. Pancreatic agenesis is a rare cause of neonatal diabetes.We report a neonate who was small for gestational age and presented with diabetes mellitus and signs of malabsorption because of pancreatic agenesis.

Barakat syndrome, also known as hypoparathyroidism, sensorineural deafness, and renal dysplasia syndrome is an extremely rare congenital disorder. Different etiologies are described for the syndrome but the definite pathophysiology remains unclear. Hereby, we present a case of Barakat syndrome who was diagnosed on the basis of clinical and molecular data.

Squamous cell carcinoma of the tongue is uncommon among children and teenagers. Most commonly, squamous cell carcinoma of the head and neck presents during the fifth and sixth decades of life in patients with a long history of tobacco and alcohol use. The rarity of this lesion in young patients implies that clinicians might not include it in the differential diagnoses. In general, carcinoma of the oral cavity in young people is reported to be particularly aggressive and carry a poor prognosis. A case of carcinoma of the tongue in a 13-year-old boy is presented in this report

The present article describes briefly the development of the theories regarding the circulation of blood in humans, from the time of Galen (second century C.E.) to the work of William Harvey (17th century C.E.).We shall summarize the views of Galen together with those of two prominent Iranian physicians of the Middle Ages (Razi and Ahwazi known in the West as Rhazes and Haly Abbas respectively) as well as that of Ibn-Nafis from Damascus (the discoverer of the pulmonary circulation) and the Spanish physician and cleric Michael Servetus and finally the definitive work of William Harvey, the English physician who described the mechanism of both the systemic and pulmonary circulation of blood in the human body.