IRANIAN JOURNAL OF MEDICAL SCIENCES (IJMS)
Year:2009 | Volume:34 | Issue:2|Papers Count:13

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' and co-workers were the first to describe graft-versus-host-disease and its treatment, and the graftversus- leukemia effect in human. The first BMT for β- thalassemia major was performed successfully by Thomas and colleagues in Seattle, in 1981. In the same year another patient with b-thalassemia major underwent BMT in Pesaro, Italy, by Lucarelli and others Since then, several hundred transplantations have been performed worldwide, mostly 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. Because of high graft failure and high rates of graftversus- host-disease 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.

Background: Accurate and rapid assessment of allograft function is essential in renal transplant recipients in order to detect allograft rejection and to monitor drug nephrotoxicity. We aimed to evaluate the usefulness of cystatin C as a marker of kidney allograft function in the early post-transplant period and to compare this value with that of conventional serum creatinine concentration.Methods: Twenty four patients scheduled for kidney transplantation at the Kidney Transplant Center of Ghaem Hospital, Mashhad, Iran from September 2006 to November 2007, were sequentially enrolled into the present study. Serum creatinine and cystatin C concentrations and urine output were measured daily after transplantation for 3 weeks or until discharge from the hospital.Results: On the 3rd postoperative day, with a cut-off value of 75 mL/min for glomerular filtration rate, areas under the receiver operating characteristic (ROC) curves were 0.926 for creatinine (P=0.021) and 0.815 for cystatin C (P=0.088). At this point creatinine was more sensitive and specific than cystatin C in estimating glomerular filtration rate. On the 7th day after transplantation, areas under ROC curves were 0. 893 for creatinine (P=0.066) and 1.000 for cystatin C (P=0.017). Therefore, cystatin C was more sensitive and specific than creatinine in estimating glomerular filtration rate. In two patients with acute rejection and arterial thrombosis, serum cystatin C concentrations increased earlier than serum creatinine.Conclusion: There is a correlation between creatinine and cystatin C early after kidney transplantation. Serum creatinine levels seem to be more sensitive and specific for detecting transitory changes in renal function in the 1st week after transplantation. After the 1st week after transplantation, cystatin C was more sensitive and specific than serum creatinine concentration.

Background: Many different techniques have been described to treat undescended testes. We aimed to study a new technique of two-stage orchiopexy with total inguinal canal mobilization to prevent cord damage and testicular atrophy.Methods: Between March 2002 and July 2005, we operated on 16 testes with short spermatic cord. In the first stage, the testes were anchored to the pubic tubercle and 6 months later (second stage) they were fixed in the scrotum through total inguinal canal mobilization. The patients were followed up by pre and postoperative physical examination, sonography of scrotum, and serum testosterone level measurement for 6-12 months.Results: Two of the 16 testes developed atrophy but 14 had volume increase. Mean testicular volumes of preoperative and 1 and 6 months postoperative testes were 0.69±0.22 ml, 0.84±0.26 ml, and 1.06±0.33 ml respectively. The volume increase was not only significant 1 (P=0.012) and 6 (P=0.002) months after the second operation compared with the first operation but also a significant increase in the volume was noted between the 1st and 6th month after the operation (P=0.003). All testes were in scrotum after 6- 12 months and the patients had normal serum testosterone level for their ages before and after the operation.Conclusion: Two-stage orchiopexy with total inguinal canal mobilization prevents vascular damage in the second stage of operation.

Background: Bipolar mood disorder type 1 is one of the most serious psychiatric disorders. We aimed to assess the efficacy of a short-term family–focused treatment for patients with bipolar mood disorder type 1 in a one-year follow-up period.Methods: Sixty patients with bipolar mood disorder and acute mania episodes who referred to Ibn-e-Sina Psychiatric Hospital in Mashhad were recruited. Half of them were randomly assigned to the psycho-education group. Their available adult family members received a psycho-educational training session before the patients' discharge from the hospital. All patients received concurrent appropriate pharmacotherapy and psychotherapy. All the patients were evaluated by a blind home-visit team every 3 months for a period of one year. The evaluation included the number of psychiatric visits, patients' adherence, and relapse status, number of re-hospitalizations, and time to relapse.Results: Fifty-seven patients completed the trial. In the experimental group, there were four patients with relapse (13.79%) and in the control group nine patients (31.58%) had relapse of the disorder (P=0.006). The mean time of taking medications in the education group was 11.41 months. This time was 9.14 months in the control group (P<0.001). There was also a significant difference between the two groups in terms of frequency of psychiatric visits (P<0.001).Conclusion: Short–term family-focused psycho-education is an effective adjunct to pharmacotherapy for bipolar mood disorder. Further studies are needed to evaluate the efficacy and cost-effectiveness of long-term family-focused psycho educational treatment for patients with bipolar mood disorder.

Background: Developmental delay is one of the most common problems of children referred to pediatric neurology clinics. While there are reports on rehabilitation and its effects, limited studies are available to delineate pharmacotherapy of such children. Because many children with developmental delay have abnormal findings in electroencephalography, we aimed to treat a group of these children, who were seizure free with sodium valproate to find the effect of sodium valproate in improving the developmental delay.Methods: We included patients referred to Mofid Children’s Hospital for developmental delay who had no organic or brain structural diseases, genetic or metabolic disorders, or intrauterine TORCH infection; however, the patients had abnormal electroencephalograms (without seizure). After clinical, para clinical, and neuroimaging evaluations, the patients were divided into two groups; those receiving treatment with sodium valproate and rehabilitation (experimental group, 25 patients), and those having only rehabilitation (control group, 25 patients). The patients were followed up and assessed at 6, 12, and 18 months after initiation of the study. The data obtained were analyzed using SPSS software.Results: All patients in the experimental group had normal electroencephalograms after 18 months of treatment. Differences in the scores of developmental quotient in both groups, before and after treatment were significant.Conclusion: Sodium valproate along with rehabilitation was very effective in the improvement of speech, mental, and behavioral development.

Background: Induced abortion is the medical or surgical termination of pregnancy before fetal viability. It has maternal or fetal indications. The aim of the present study was to evaluate the efficacy of the combination of methotrexate and misoprostol for termination of the pregnancy in the first trimester. Methods: This analytic study was performed on 100 women at the first trimester of pregnancy (<14th week), during 2004- 2006. Each woman received intramuscular methotrexate (50 mg/m2) and intravaginal misoprostol (800 mg) 72 hours after methotrexate administration. The second dose of misoprostol was administered if abortion did not occur after 24 hours. Abdominal ultrasonography was performed 7 days after abortion.Results: Of the 100 women, 76 had gestational age>9 weeks and 24 has gestational age<9 weeks. Eighty one women (81%) had successful medical abortion and 19% women required curettage. Fifty nine patients with gestational age>9 weeks and 22 patients with gestational age<9 weeks had complete abortion. Failure rate was higher in missed abortion. Required dose of misoprostol and duration of conceptus expulsion were higher in pregnancies with missed abortion.Conclusion: Combination therapy with methotrexate and misoprostol represents a safe and effective alternative to invasive methods for termination of the pregnancy in the first trimester.

Background: Pseudo exfoliation syndrome is one of the most common identifiable causes of open angle glaucoma. It has unknown etiology and pathogenesis. Infection, possibly viral, is one of the proposed pathogenic mechanisms in this condition. In the present study the presence of herpes simplex virus (HSV) in specimens of anterior lens capsule of patients with pseudo exfoliation syndrome has been assessed.Methods: The presence of HSV- DNA was searched by using polymerase chain reaction method in specimens of anterior lens capsule (5 mm diameter) of 50 patients with pseudo exfoliation syndrome (study group) and 50 age-matched patients without the disease (control group) who underwent cataract or combined cataract and glaucoma surgery during a one-year (2006-2007) period in Khalili Hospital, Shiraz, Iran. The results were compared statistically with Chi square test and independent samples t test using SPSS software (version 11.5).Results: HSV type I DNA was detected in 18% of the patients in the study group compared with 2% in the control group (Chi square test, P=0.008). The difference between the ranges of intraocular pressure in the two groups was not statistically significant.Conclusion: The presence of HSV type I DNA suggests the possible relationship between the virus and pseudo exfoliation syndrome. It may be a treatable etiology in this multi-factorial disorder and may help to future management of patients; especially to prevent some of the complications in this syndrome.

Background: Crinum purpurascens is a herbaceous plant belonging to the Amaryllidaceae family. We aimed to evaluate the antisalmonellal properties of the leaf extracts and fractions of C. purpurascens, and the toxicity of the most active extract.Methods: Three extracts and three fractions were prepared from the leaves of Crinum purpurascens Herb. (Amaryllidaceae) and tested for their antisalmonellal activities and toxicity profile. The antibacterial activity was determined using agar diffusion, agar dilution, and broth dilution techniques. Phytochemical screening of the various extracts and fractions was performed. The toxicity profile of the CH2Cl2/MeOH (1:1) extract was studied.Results: All the extracts and fractions, except hexane fraction, showed antimicrobial activity against Salmonella typhi, Salmonella paratyphi A and Salmonella paratyphi B. The CH2Cl2/MeOH (1:1) extract showed the highest activity. The minimum inhibitory concentration values were 2.50 mg/ml against S. typhi, and 1.88 mg/ml against S. paratyphi A and S. paratyphi B. The minimum bactericidal concentration values were 7.50 mg/ml against S. typhi and 3.75 mg/ml against S. paratyphi A and S. paratyphi B. Mice administered high doses of extract exhibited reduced reaction to noise, locomotion, reactivity and reaction to pinch, and losses in body weight. Additionally, the rats that received high doses of the extract showed increase in liver, spleen and kidney to body weight ratios, and decrease in total protein concentrations of the liver and lung, and in hematocrit value.Conclusion: C. purpurascens leaf extract contains antisalmonellal principle (s) and at high doses, may have a depressant or sedative effect on the central nervous system and analgesic activity. Also, it may be anorexiant, hepatotoxic, and nephrotoxic.

Normal hemostasis requires balanced regulation of prothrombotic and antithrombotic factors. Inherited alteration of factor V and prothrombin gene, the G20210A mutation, increases the resistance of factor V to degradation and booster production of prothrombin respectively. These alterations can increase hypercoagulability leading to thrombotic consequences. We aimed to assess the frequencies of these mutations in a group of the population of southern Iran. In total, 198 healthy volunteers with the age range of 1-64 years were selected and screened for factor V Leiden and prothrombin mutations using polymerase chain reaction and restriction fragment length polymorphism techniques. The carrier frequencies for factor V Leiden and prothrombin mutation in the studied cohort were 4.1% and 3.07%, respectively. In the studied area, the allele frequency of factor V is higher than the prothrombin G20210A mutation (0.0204 v 0.0153). According to the data and Hardy-Weinberger equation, the total risk of thrombosis caused by homozygosity and heterozygosity of factor V Leiden, prothrombin G20210A mutation and compound heterozygosity of these mutations are about 1 in 500 individuals.

Neurofibromatosis type 2 is an inherited disease. The main manifestation of the disease is the development of symmetric, non-malignant brain tumors in the region of the cranial nerve VIII usually as schwannoma. We report here a 20-year-old woman with primary intracranial malignant nerve sheath tumor located in the left cerebellopontine angle. Histologically, the tumor showed malignant spindle cells in fascicular pattern with focal S100 positivity on immunohistochemistry. A subtotal surgical resection was performed followed by adjuvant radiotherapy. This is the first case of primary intracranial malignant nerve sheath tumor associated with neurofibromatosis type 2 reported in the literature. The prognosis of this potentially aggressive neoplasm is poor.

The placental adhesive disorders such as placenta accreta and placenta percreta are the rare causes of serious obstetric hemorrhages. They are associated with high maternal morbidity and mortality. Placenta percreta is usually diagnosed in the third trimester of pregnancy as a massive postpartum hemorrhage when an attempt to remove the placenta reveals lack of a cleavage plane. Here we report an unusual presentation of placenta percreta as hemoperitoneum and hemorrhagic shock in the third trimester of pregnancy. A 33-year-old woman was admitted to hospital at the 35th weeks of gestation with abdominal pain and hemorrhagic shock. Laparotomy was immediately performed because of intra-abdominal bleeding. Upon inspection, a posterior laceration of the uterus was noted. Pathologic investigation of the uterus showed placenta percreta. The patient recovered uneventfully. Spontaneous rupture of the uterus can be occured in the absence of uterine trauma. In the differential diagnosis of a pregnant woman presenting with hypotension, abdominal pain, and fetal death, rupture of the uterus caused by placenta percreta should be considered. Rapid diagnosis, blood transfusion, and emergency laparotomy are the key steps in successful management.

Pilonidal disease consists of a hair-containing sinus or abscess occurring most frequently in intergluteal cleft. This disease is generally benign. Although very uncommon entity, it seems reasonable to be aware of possible malignancy in longstanding cases. We report a case of squamous cell carcinoma in a 52- year-old man, with a prolonged history of pilonidal disease and ulceration since 3 months before referring to our clinic. We excised the cyst, and the pathologic evaluation reported moderate differentiated squamous cell carcinoma. So, reoperation on the lesion site to excise a 2-cm margin was performed and the defect was covered with Limberg cutaneus flap. We recommend early excision of pilonidal cysts to prevent possible malignant degeneration. Histological examination of the excised materials to prevent missing rare malignant cases is recommended.

Mycosis fungoides is the most common type of cutaneous T cell lymphoma with a reported incidence of 0.5 cases per 100,000 people per year. The cause of mycosis fungoides is still unknown; however genetic and environmental factors have been implicated as possible pathologic factors.