Iranian Journal of Medical Sciences
Volume:34 Issue: 1, Mar 2009

During the last two decades conventional therapy has improved the prognosis of thalassemia. However, despite such improvement it still remains a progressive disease with treatment-related complications such as hepatitis, liver fibrosis, and cardiac disease. Bone marrow transplantation (BMT) can prevent or delay progression of the aforementioned complications. The importance of clinical research in the field of BMT was recognized with the award of the 1990 Nobel Prize in Physiology and Medicine to E. Donnall Thomas, one of the pioneers of BMT in humans. George Mathe'' was a pioneer in the early development of clinical BMT. Mathe'' et al. were the first to describe graft-versus-host-disease (GVHD) and its treatment, and the graft-versus- leukemia (GVL) effect in human. The first BMT for β-thalassemia major was performed successfully by Thomas et al. in Seattle, in 1981. In the same year another patient with β-thalassemia major underwent BMT in Pesaro, Italy, by Lucarelli et al. Since then, several hundred transplantations have been performed worldwide, the majority of these in Italy. From 1991 through 2007 BMT have been performed on 497 (Tehran=342, Shiraz=155) blood transfusion dependent patients with thalassemia major in Iran, with disease-free survival of 71-77% respectively. Due to high graft failure and GVHD rates, BMT from alternative donors should be restricted to patients who have poor life expectancies because they cannot receive adequate conventional treatment or because of alloimmunization to minor blood antigens. Beginning in the early 1980s, it was shown that umbilical cord blood contained high levels of hematopoietic progenitor cells.

Beta thalassemia is a common inherited disease, resulting from one or more of 200 different mutations in the beta-globin gene. Qazvin province has attracted migrations of several different populations due to industrialization during the past five decades. The aim of this study was to define the molecular spectrum of beta-thalassemia mutations in Qazvin province. Ethylen diamin acetic acid-containing venous blood samples were collected from 100 patients with transfusion-dependent beta-thalassemia from the department of Pediatrics in Qods hospital. Age, sex, history, and consanguinity between the parents were recorded by reviewing the patients’ files. DNA was isolated from leukocytes using the standard procedure. Amplification refractory mutation system (ARMS) technique was used for molecular detection of mutations. Direct sequencing analysis was applied for DNA samples when no mutation was detected with ARMS. Of the 200 chromosomes investigated, 11 types of mutations were identified by ARMS technique while direct sequencing revealed the remaining alleles (9 types of mutations). Total 20 different mutations discovered by this two-step approach. Abundant alleles (IVS II-1, IVS I-10, FSC 8/9) accounted for 59.3% of the mutations. IVS II-1 with a frequency of 31.3 % was the most common while HbS, Cd 74/75 and Cd 15, each with a frequency of 0.55%, had the least frequencies. Beta thalassemia mutations are very heterogeneous in Qazvin province. Extensive ethnic and genetic admixture has resulted in unexpectedly high number of different mutations, most of them similar to that of north and north-western provinces of Iran. Different mutations in this region suggest migration of chromosomes from distant places and genetic admixture.

For more than four decades clomiphene citrate has been the first line of the treatment for ovulatory disorders. The aim of this study was to compare the effects of letrozole and clomiphene citrate on ovulation and pregnancy rate in patients with polycystic ovary syndrome. In this prospective double-blind study, 100 patients with polycystic ovary syndrome were randomized into two equal groups. The first group received letrozole, 5mg daily (per oral) and the second group received clomiphene, 100mg daily during the 3rd-7th days of the menstrual cycles. Intramuscular human chorionic gonadotropin (hCG) (10, 000 IU) was administered to trigger ovulation when at least one mature follicle (≥ 18mm) was developed. Ovulation occurred in 30 patients (60%) of the letrozole group and in 16 patients (32%) of the clomiphene group, which showed a statistically significant difference (P=0. 009). The mean number of follicles with diameter >14 mm on the day of administration of hCG was 1. 06±0. 95 in the letrozole group and 1. 14±1. 17 in the clomiphene group, which showed non-significant difference (P=0. 962). No difference was found in the endometrial thickness between the two groups. A non-significant increase in pregnancy rate was observed in the letrozole group (26% v 14% P=0. 21). پ Ovulation rate was higher in letrozole group and administration of letrozole was associated with a non-significant increase in pregnancy rate.

Identifying the sources of variation in QTc measurements is important for preventing arrhythmias during and after hemodialysis. The present study was designed to determine the correlation between the type of hemodialysis buffer and the changes in QTc interval in patients on chronic hemodialysis. Fifty-nine patients on chronic hemodialysis who referred in winter 2007 to hemodialysis centers of Ghaem and Hashemi Nejad hospitals, in Mashhad, Iran, were divided into two groups according to their last dialysate buffer: acetate or bicarbonate. Electrocardiography, arterial blood gas parameters, serum K+, Na+, ionized calcium, and albumin levels were measured prior to and after hemodialysis in all patients. All arterial blood gas parameters and serum electrolytes concentrations were increased except K+ levels that were significantly decreased with hemodialysis. PCO2 and QTc intervals were slightly increased in all patients, however this increase was not statistically significant. We found that the type of dialysate affected the QTc interval, HCO3, base excess, base excess of extra cellular fluid, and base buffer changes with no effect on ionized calcium, pH, PCO2, and serum albumin concentration. QTc interval was prolonged by using bicarbonate and shortened by using acetate dialysate buffer. We found no correlation between the variations of QTc interval and serum electrolytes or arterial blood gas parameters in either group. Bicarbonate buffer use in hemodialysis prolonged QTc interval and acetate buffer shortened it. This effect is independent of serum electrolytes and pH changes during hemodialysis. The effect of bicarbonate buffer is probably due to more tolerability of ultra filtration, more effective edema reduction and augmented body electro-conductivity.

Angiotensin converting enzyme (ACE) upregulation in stromal cells of joints affected by rheumatoid arthritis may lead to higher tissue angiotensin II that is a vasoconstrictor and mitogen factor. To date, the role of angiotensin II on regulating blood flow in inflamed joints has not been studied. Acute and chronic joint inflammation was induced in rabbits by intra-articular injection of carrageenan and antigen-induced arthritis method, respectively. The ACE level of synovial fluid and the response of joint blood flow to angiotensin II, angiotensin II receptor antagonist, and the role of nitric oxide (NO) in modulation of the effects of angiotensin II on joint blood vessels were examined. The synovial fluid level of ACE was significantly increased during the process of inflammation and angiotensin II increased joint vascular resistance dose-dependently in both acute and chronically inflamed joints. The angiotensin 1 receptor antagonist losartan completely blocked the vasoconstrictor effect of angiotensin II on joint blood vessels and induced vasodilatation. Nitric oxide synthase inhibitor N-omega -nitro L- arginine methyl ester (L-NAME) increased joint vascular resistance and augmented vascular response of inflamed joints to angiotensin II. Angiotensin II receptors in joint blood vessels are angiotensin -1 subtype, and inflammation significantly increases the activity of synovial fluid ACE. Nitric oxide plays a significant role on regulating joint blood flow and in modulation of angiotensin 1 receptor-mediated vasoconstriction of inflamed joint blood vessels.

Cylophosphamide is used alone or in combination with other drugs for treatment of neoplastic diseases. Hemorrhagic cystitis is a major potential toxicity and dose limiting side effect of cyclophosphamide. The aim of this study was to evaluate the effects of lycopene compared with some antioxidants for the prevention of cyclophosphamide induced hemorrhagic cystitis in rats. In this study, male Sparague-Dawley rats divided into 17 groups of six animals. Group 1 received saline (10 ml/kg, i.p) as normal control, group 2 received cyclophosphamide (200 mg/kg, i.p) as a single dose, groups 3-10 received Mesna (40 mg/kg, i.p), N-acetylcysteine (100 mg/kg i.p), dithiotheritol (50 mg/kg, i.p), L-carnitine (200 and 400 mg/kg, i.p), grape seed extract (500 mg/kg i.p) and lycopene (0.1 and 0.5 mg/kg, i.p) alone. Groups 11-17 received Mesna (40 mg/kg, i.p), N-acetylcysteine (100 mg/kg, i.p), dithiotheritol (50 mg/kg, i.p), L-carnitine (400 mg/kg, i.p), grape seed extract (500 mg/kg, i.p) and lycopene (0.1 and 0.5 mg/kg, i.p), 5 minutes before, and 2 and 6 hours after administration of 200 mg/kg cyclophosphamide. Pathological and biochemical analysis was evaluated 24 hours after cyclophosphamide administration. Mesna and N-acetylcysteine resulted in some but not full protection against cyclophosphamide toxicity compared to the controls. Lycopene (0.1 and 0.5 mg/kg) was efficient in protecting the bladder from cyclophosphamide induced hemorrhagic cystitis. However, dithiotheritol, L- carnitine and grape seed extract did not prevent hemorrhagic cystitis. Our results suggest that pre and co- treatment of lycopene (0.1 and 0.5 mg/kg) with cyclophosphamide may have therapeutic potential to inhibit the hemorrhagic cystitis by cyclophosphamide.

Although the physician-patient relationship is of special significance for a proper diagnosis, few studies have been done to find out how successful these interactions are across various medical specialties. Common physician knowledge measured by a questionnaire tended to view fields such as psychiatry more successful in achieving patient satisfaction than other specialties. However, the validity of such assumptions has rarely been assessed scientifically. The current study was designed to find out whether medical specialties with greater mental/emotional orientation, such as psychiatry, are more successful in achieving patient satisfaction than specialties with a stronger manual orientation, such as surgery. A total of 27 physicians were randomly selected from different medical orientations. They were requested to use their common-sense to rate the specialties under study depending on whether they were more mentally oriented or manually inclined. They were also asked to indicate which groups of specialties are likely to be more successful in achieving patient satisfaction from clinical interactions. Another sample of 561 patients was selected from nine different medical specialty clinics based on a quota sample method. Patients were asked to complete a 15-item Communication Satisfaction Questionnaire following their clinical interviews with their physicians. The results obtained from the patients did not fully corroborate the results of the physicians'' questionnaire, which predicted greatest patient satisfaction from psychiatrists. Our results showed that pediatricians and gynecologists were more successful in achieving patient satisfaction (P<0.001) than psychiatrists. Patients’ satisfaction with different medical specialties is different from physicians’ common-sense assumptions. Patients were more satisfied with pediatricians and gynecologists rather than psychiatrists.

Burns are among the most common traumas in developing countries, which consume large amounts of medical resources. It is important to find an appropriate material for dressing of burn wounds that improves healing and is readily available, easily applicable, and economical. In a single-blind randomized controlled clinical trial from March to October 2006, 211 patients with less than 20% burn were enrolled into two groups. The first group contained 104 patients with average burn of 11.90± 3.80% of total body surface area (TBSA) for whom amnion dressing was used. The second group composed of 107 patients with average burn of 12.30± 4.14% of TBSA treated with routine silver sulfadiazine dressing. Amniotic membrane usage was accompanied by acceleration in wound healing, less need for skin graft, and less pain. The mean healing time in superficial parts of burn wounds in the amnion group was significantly shorter than the control group (9.50±2.13 v 14.30±2.60 days; P value < 0.01). The extent of the wound with granulation tissue which needed skin graft was less in the amnion group (2.10 ± 2.21% v 4.2 0±1.44%; P value < 0.01). Widespread use of amniotic membrane dressing is recommended for limited burn wound management.

The purpose of the current study was to determine whether infiltration of bupivacaine in the incision site of midline laparotomy reduces postoperative pain and opioid consumption. Fifty-six, 30-60 year-old patients who were undergoing midline laparotomy were enrolled in the present study. The patients were randomly assigned into two groups of control (group C, n = 28) or bupivacaine (group B, n= 28). Just before suturing, the incision sites were infiltrated by 20 ml epineprinated bupivacaine 0.25% (group B) or 20 ml normal saline as placebo (group C). The patients were asked to score their pain at 6, 24, and 48 hours after surgery. Demographic characteristics of the patients were similar in the two groups. There was no significant difference in the mean of visual analogue scale pain scores measured over time between the two groups. There was a significant difference in post operative meperidine consumption between the two groups, and in the bupivacaine group, meperidine request was less (90.53±13.36 mg in bupivacaine group v127.5±23.14 mg in the control group, P<0.05). After midline laparotomy, incisional site infiltration with 20 ml epineprinated bupivacaine 0.25% causes a significant decrease in postoperative meperidine consumption

Congenital fusion of the maxilla and the mandible is a rare condition. The extent of the problem ranges from simple mucosal adhesion (synechia) to severe bony fusion (syngnathia). Here we present a patient with zygomatico-mandibular fusion who was treated at neonatal and infancy periods at three different stages without tracheostomy. Having had recurrence for two times, porcine grafting was performed as a novel procedure to increase the chance of success. Ultimately the patient had uneventful outcome for 6 months after the last operation

Rheumatoid arthritis is a chronic systemic inflammatory disease that affects approximately 0.5-1% of the world population. The current approach to this disease is to start an intensive treatment without delay once the disease has developed. Various studies in the literature have shown that combination of disease modifying antirheumatic drugs such as sulfasalazine and chloroquine offers a more advantageous treatment. Although these drugs may cause central nervous system adverse effects such as serious psychiatric problems including mania and psychosis, these symptoms have been reported to occur only infrequently. The present case study reports a female patient who was hospitalized due to bipolar affective disorder-mixed episode. She had been receiving 250 mg/day chloroquine for 9 months for rheumatoid arthritis without exhibiting any adverse psychiatric effects. However, upon receiving a combination of 250 mg/day chloroquine and 2 gr/day sulfasalazine, she developed serious psychiatric symptoms.

Echinococcus granulosus is considered the major cause of human hydatid cysts. Usually the duration of cyst formation is 10-20 years. This period shortens significantly upon rupture of a primary cyst. The literature describes low incidence of primary involvement of ovary as a site of hydatid cyst formation. Our case is the first report on ovarian hydatid cyst in Iran. A 60-year-old woman was presented with abdominal pain in the left lower quadrant area. Paraclinical data were suggestive of neoplasia and preoperative diagnosis was ovarian tumor. During laparotomy, multiple cysts resembling hydatid cysts were observed in the left ovary. Pathological examination confirmed the diagnosis of hydatid cyst. Although there is a small possibility of secondary ovarian echinococcal disease, it is more probable for this case to be primary infection, as the patient had developed ovarian hydatid cysts 15 years after hepatic involvement and recurrence after 30 months is very uncommon.