Primary epithelioid sarcoma of the vulva in pregnancy

Primary epithelioid sarcoma (ES) of the vulva is an extremely rare tumor, and to date, only 20 cases have been reported. We report the second case of vulvar ES presenting in pregnancy. A 25-year-old woman, at 6 weeks of gestation, was diagnosed as having vulvar sarcoma and was referred to our hospital. Immunohistochemical studies revealed that the tumor cells were positive for cytokeratin, epithelial membrane antigen (EMA), and wimentin. The tumor cells did not stain for S-100, SMA, HMB-45, desmin, LCA, CD-34, or estrogen and progesterone receptors. The tumor showed diffuse and strong staining for CD-117 (c-kit). After the curettage, she underwent local wide excision and left inguinal lymph node dissection and received external-beam radiation therapy. At 16 months of follow-up the patient was well without any disease recurrence. The appropriate treatment of vulvar ES is not clear, and the role of chemotherapy is disappointing; however, it might be worth investigating the role of Imatinib in c-kit overexpressing vulvar ES.     

Subureteral dextranomer/hyaluronic acid copolymer injection for vesicoureteral reflux in transplant candidates

BACKGROUND AND PURPOSE: Endoscopic subureteral injection of tissue-augmenting substances has become an alternative to antibiotic prophylaxis and open surgery for the management of vesicoureteral reflux (VUR). Several injectable materials have been tried for this purpose. In this study, we tried to determine the efficacy of dextranomer/hyaluronic acid copolymer (Dx/HA) injection for the treatment of VUR in renal-transplant candidates. PATIENTS AND METHODS: A total of 21 transplant candidates (29 ureteral units; 13 females, 8 males) with a mean age of 20.2 years (range 14-26 years) underwent endoscopic correction of VUR with Dx/HA. Diagnosis of VUR was made by voiding cystourethrography. The efficacy of the treatment was assessed with voiding cystourethrography at 3 months and 1 year postoperatively. Renal transplantation with living related donor organs was performed in 11 of the 21 patients. RESULTS: Endoscopic treatment was performed without complication in all cases. Higher success rates were obtained in patients with low-grade reflux, the overall success rate in the series being 82.7%. The mean follow- up after renal transplantation was 21.8 months (range 5-45 months). In one patient, reflux recurred after renal transplantation and was treated successfully by a repeat Dx/HA injection. The urine cultures of all patients remained sterile. CONCLUSION: Transplant candidates with VUR can be treated with Dx/HA, which cured the majority of our patients after one or two treatments with few low side effects. Endoscopic subureteral injection of Dx/HA has become an alternative treatment for VUR in transplant candidates. Long-term results are needed before making a final statement about its value.     

Labor induction at term: a comparison of the effects of 50 microg and 25 microg vaginal misoprostol

PURPOSE OF INVESTIGATION: To compare the effects of 50 microg of vaginal misoprostol with 25 microg for labor induction at term. METHODS: One hundred and forty-seven pregnant women with indications for labor induction and cervical Bishop's score of < or = 6 were randomly assigned to receive either 50 microg (n = 74) or 25 microg (n = 73) of vaginal misoprostol every four hours until either a Bishop's score of > or = 8 or adequate uterine contraction frequency had been achieved. Induction-to-vaginal-delivery time was considered the primary outcome measure. RESULTS: Mean induction-to-vaginal-delivery time was significantly shorter in the 50-microg group than in the 25-microg group (526 +/- 141 min vs 745 +/- 218 min, respectively); oxytocin was administered to 65.8% of the patients in the 25-microg group and to 35.1% in the 50-microg group (p < .05). The incidence of tachysystole was significantly higher in the 50-microg group than in the 25-microg group (12% vs 2.7%, p < .05). We found no statistically significant difference between the two groups with respect to the rate of primary cesarean section, incidence of hyperstimulation syndrome, or neonatal outcome (p > .05). CONCLUSION: Fifty micrograms of vaginally administered misoprostol is an effective and inexpensive means of inducing labor at term. Uterine tachysystole may be associated more frequently with a 50-microg dose of vaginal misoprostol than with a 25-microg dose. Clinicians must accurately document the frequency and intensity of uterine contractions before every 50-microg dose of misoprostol is administered.     

Familial systemic lupus erythematosus: the role of genetic and environmental factors

OBJECTIVE: To examine the contribution of genetic and environmental factors to disease occurrence in 26 families with two or more members affected with systemic lupus erythematosus (SLE). METHODS: Genetic and environmental factors were examined by HLA-A, B, C/DR typing and by determining the presence of lymphocytotoxic antibodies (LCA) in patients and their consanguineous and non-consanguineous relatives. RESULTS: No association between SLE and HLA-A, B, C antigens was found. There was, however, a significant association with HLA-DR2 in white subjects with SLE. The most striking finding was that HLA sharing was increased among the affected members, suggesting genetic similarities. Seven of 14 sib pairs (50%) who had concordant SLE were HLA identical as opposed to an expected 25%. Another interesting finding was that 15/18 (83%) patients with SLE and 11/22 (50%) consanguineous relatives had LCA, while 1/9 (11%) spouses, and 2/42 (5%) healthy controls had these antibodies. CONCLUSION: Genetic factors have a role in the development and expression of SLE. Environmental factors may trigger the disease in genetically susceptible hosts.     

Chronic Cigarette Smoking Affects Left and Right Ventricular Long-Axis Function in Healthy Young Subjects: A Doppler Myocardial Imaging Study

Objective: Cigarette smoking is one of the major risk factors for coronary artery disease. However, chronic smoking has additional cardiac adverse effects independent of coronary atherosclerosis. We assessed the possible effects of chronic smoking on left and right ventricular (LV, RV) long-axis function using Doppler myocardial imaging (DMI). Methods: Forty otherwise healthy smokers (mean age = 26 ± 3 years) and 40 age-matched nonsmoking controls enrolled. Standard echocardiography protocol was followed by DMI. Peak systolic (V_S), early (V_E) and late diastolic (V_A) velocities, peak systolic strain (S), and strain rate (SR) were measured from septal, lateral, and RV free walls. Results: The baseline characteristics of two groups were similar. There were significant differences regarding Doppler myocardial velocity, S, and SR indices. Septal V_E (P < 0.04), S (P < 0.0001) and SR (P < 0.02) were significantly reduced in smokers. For both lateral and RV free wall, V_S (P < 0.003, P < 0.002, respectively), V_E (P < 0.0001, P < 0.002, respectively), S (P < 0.0001 for both), and SR (P < 0.0001, P < 0.002, respectively) were significantly reduced in smokers. There were significant correlations between the amount of smoking and septal, lateral, and RV free wall S and SR. Conclusions: Chronic cigarette smoking causes alterations in long-axis systolic and diastolic functions of right and left ventricles in healthy young subjects. These changes can be accurately detected with Doppler myocardial velocity and SR imaging.     

The relationship of the methylenetetrahydrofolate reductase C677T gene polymorphism in Turkish type 2 diabetic patients with and without nephropathy

BACKGROUND: Poor glycaemic control, hypertension and duration of diabetes are risk factors for the development of diabetic nephropathy, but there may be genetic factors. Recently, a common C to T mutation at nucleotide position 677 of the MTHFR gene (MTHFR677C > T) has been reported to be correlated with hyperhomocysteinemia and the severity of coronary artery disease as macroangiopathy. We aim to investigate Turkish type 2 diabetic patients with/without diabetic nephropathy and healthy group and examine the contribution of the MTHFR gene polymorphism to the development of diabetic nephropathy. METHODS: DNA was extracted from peripheral leukocytes of the subjects. Genotyping of the MTHFR C677T polymorphism for all individuals was performed by melting curve analysis of the generated amplicons after real-time online PCR. RESULTS: This genotype distribution did not differ between control subjects and type 2 diabetic patients in which 6.8% were TT, 43.7% were CT and 49.5% were CC (chi2 = 0.201, p > 0.05). The frequency of the mutant T allele was 23.4% in diabetic patients with nephropathy versus 33.0% in those without nephropathy. The genotype frequencies were TT, 2.1%; CT, 46.6%; CC, 55.3% in diabetic patients with nephropathy versus TT, 10.7%; CT, 44.6%; CC, 44.6% in those without nephropathy. CONCLUSIONS: The MTHFR genotype and allele frequencies were not different between diabetic patients with and without nephropathy (chi2 = 3, 386, p > 0.005; chi2 = 2.320, p > 0.005, respectively). Therefore, we conclude that the MTHFR gene polymorphism is not associated with the development of diabetic nephropathy in Turkish type 2 diabetic patients.