Angiomyolipoma of the uterus associated with tuberous sclerosis: case report and review of the literature

BACKGROUND: Angiomyolipoma (AML) is a rare benign tumor that occurs most commonly in the kidney. Only a few cases have been described in the uterus. CASE: We describe a 32-year-old woman with tuberous sclerosis who presented with irregular bleeding. Preoperatively, the lesions were confused with leiomyoma on ultrasound. At laparotomy, multiple circumscribed subserosal and intramural purplish lesions on the corpus uteri were noted leading to a diagnosis of uterine angiomyolipoma with the histological and immunohistochemical findings. CONCLUSION: In contrast to other reported uterine angiomyolipoma cases, HMB-45 melanoma-specific antibody immunoreactivity is demonstrated in the present case. The histopathologic diagnosis as well as immunohistochemical analysis is discussed here with a review of the literature.     

Laparoscopic Decortication of Hilar Renal Cysts Using LigaSure

Background and Objectives:

In this study, we evaluated the safety and efficacy of using the LigaSure sealing system (Valleylab, Boulder, Colorado) for laparoscopic decortication of symptomatic hilar renal cysts.

Methods:

Seventeen patients underwent laparoscopic decortication of hilar renal cysts with the LigaSure system. Our study included only symptomatic, Bosniak type 1, simple and symptomatic renal cysts. The operative route, transperitoneal or retroperitoneal, was planned according to the location confirmed by computed tomography. The patients'' symptoms were preoperatively and postoperatively evaluated by the Wong-Baker visual pain scale. Operative measures and radiologic outcomes were prospectively evaluated.

Results:

The mean age of the patients was 56.4 years, and the mean follow-up period was 12.5 months. Preoperative computed tomography showed only a single cyst in 15 patients (88.2%) and showed two separate cysts in 2 cases (11.8%). The cysts were located in the perihilar region close to the vascular structure in all patients. A transperitoneal approach was used in 9 patients, and a retroperitoneal approach was used in 8 patients. The mean operative time and hospitalization time were 56.4 minutes and 1.2 days, respectively. Minor complications were observed in 3 patients. Symptomatic and radiologic success rates of 94.2% and 100%, respectively, were achieved.

Conclusion:

Laparoscopic decortication of symptomatic hilar renal cysts—first reported in the literature in this study—using the LigaSure sealing system is feasible, effective, and safe, even if the cyst is located in the perihilar area.     

Repeatability and variation of quantitative gait data in subgroups of patients with stroke

We aimed to determine the repeatability and variation of quantitative gait data in patients with stroke and to compare the subgroups in terms of gait variability. Time-distance and kinematic characteristics of gait were evaluated in 90 inpatients (30 women) with hemiparesis (mean+/-S.D. age 57.7+/-12.5 years and time since stroke 5.99+/-6.46 months). Subgroups were based on "gender", "side of paresis", "lesion type", "motor recovery level", "sensory status", "time since stroke" and "walking velocity". Repeatability was adequate to excellent in all stroke subgroups (ICC range 0.48-0.98). Walking velocity was the most repeatable gait parameter after stroke. Variation in step length was significantly higher in women than in men (CV 16% versus 9%, p<0.05). Slow walkers (walking velocity <0.34 m/s) had a higher variation than fast walkers in step length (CV 12.5% versus 7.5%, p<0.01), single support time (CV 11.9% versus 6.3%, p<0.05), peak hip extensions in stance (CV 11.5% versus 3.7%, p<0.01) and knee flexion in swing (CV 11.8% versus 6.5%, p<0.05). In our stroke patients, their age, time since injury, lesion characteristics, impaired proprioception or level of motor recovery had no effect on gait variability. For better interpretation of quantitative gait data, clinicians should consider that variation in step length, single support time, peak hip extension in stance and knee flexion in swing differs according to walking velocity after stroke.     

Comparision of preoperative and postoperative histopathology findings in patients undergoing thyroidectomy

Aim

To compare the histopathological findings on fine needle aspiration (FNA) biopsy of thyroid nodules and thyroidectomy surgical specimens, in order to determine the efficacy, specificity and sensitivity of FNA in the diagnosis of thyroid malignancy.

Methods

The data on a series of 86 patients who underwent thyroidectomy for thyroid nodules were evaluated retrospectively. The demographic characteristics and imaging findings were reviewed, and the histopathological findings on the preoperative FNA biopsy and the surgical thyroid specimen were compared.

Results

The FNA findings on the thyroid nodules were: 32.1% benign, 12.2% malignant, 2.7% suspicion of malignancy, 6% atypia of undetermined significance, 15.7% follicular lesion and/or suspicion of follicular neoplasm, and in 31.3% the aspirate was inadequate. The postoperative histopathological specimens were evaluated as 31 malignant (36%) and 55 benign (64%). Statistically significant correlation was demonstrated between the FNA and the postoperative histopathological findings (p<0.05). In this series, the overall sensitivity of FNA biopsy was 10%, the specificity 70.9%, the positive predictive value 16% and the negative predictive value 58%.

Conclusions

This study identified a high negative predictive value of FNA biopsy, and an increased rate of “nondiagnostic” and “microcarcinoma” outcomes, but the sensitivity of FNA in the preoperative diagnosis of thyroid malignancy was low.

     

Protective effect of resveratrol against renal oxidative stress in cholestasis

BACKGROUND/AIMS: This experimental study was designed to evaluate histological changes of the kidney and renal tissue levels of malondialdehyde (MDA), reduced glutathione (GSH), and nitric oxide (NO) and the effect of resveratrol on these metabolites after bile duct ligation in rats. METHODS: Secondary biliary cirrhosis was induced by bile duct ligation for 28 days. Swiss albino rats were divided into three groups. Group 1: Sham (n=7), Group 2: Bile duct ligation (n=7), Group 3: Bile duct ligation plus resveratrol (n=7). Bile duct ligation (BDL) plus resveratrol group received 10 mgr/kg dose of resveratrol intraperitoneally daily throughout 28 days. Kidney tissues were harvested to determine the tissue levels of MDA, GSH, and NO activity. Liver and kidney tissues were removed for light microscopic evaluation. RESULTS: Cholestasis was determined by biochemical and pathologic examination. In the resveratrol-treated rats, levels of MDA were significantly lower than those of the BDL group (p < 0.04). The levels of GSH in the resveratrol-treated rats were significantly higher than those in the BDL group (p < 0.01). The levels of NO in the resveratrol group were significantly lower than those in the BDL group (p < 0.01). CONCLUSION: The present study demonstrates that intraperitoneal administration of resveratrol in bile duct ligated rats maintains antioxidant defenses and reduces kidney oxidative damage. This effect of resveratrol may be useful in the preservation of renal oxidative stress in cholestasis.     

Multiscale analysis of SRY-positive 46,XX testicular disorder of sex development: Presentation of nine cases

46,XX testicular disorder of sex development (46,XX TDSD) is a relatively rare condition characterised by the presence of testicular tissue with 46,XX karyotype. The present study aims to reveal the phenotype to genotype correlation in a series of sex-determining region Y (SRY)-positive 46,XX TDSD cases. We present the clinical findings, hormone profiles and genetic test results of six patients with SRY-positive 46,XX TDSD and give the details and follow-up findings of our three of previously published patients. All patients presented common characteristics such as azoospermia, hypergonadotropic hypogonadism and an SRY gene translocated on the terminal part of the short arm of one of the X chromosomes. Mean ± standard deviation (SD) height of the patients was 164.78 ± 8.0 cm. Five patients had decreased secondary sexual characteristics, and three patients had gynaecomastia with varying degrees. Five of the seven patients revealed a translocation between protein kinase X (PRKX) and inverted protein kinase Y (PRKY) genes, and the remaining two patients showed a translocation between the pseudoautosomal region 1 (PAR1) of X chromosome and the differential region of Y chromosome. X chromosome inactivation (XCI) analysis results demonstrated random and skewed XCI in 5 cases and 1 case, respectively. In brief, we delineate the phenotypic spectrum of patients with SRY-positive 46,XX TDSD and the underlying mechanisms of Xp;Yp translocations.     

Internalized Stigma in Pediatric Psoriasis: A Comparative Multicenter Study

BackgroundInternalized stigma, adoption of negative attitudes and stereotypes of the society regarding persons'' illness, has not been studied previously in pediatric psoriasis patients.ObjectiveWe aimed to investigate the internalized stigma in pediatric psoriasis patients and to determine differences according to factors affecting internalized stigma compared to adult psoriasis patients.MethodsThis multicenter, cross-sectional, comparative study included 125 pediatric (55 female, 70 male; mean age±standard deviation [SD], 14.59±2.87 years) and 1,235 adult psoriasis patients (577 female, 658 male; mean age±SD, 43.3±13.7 years). Psoriasis Internalized Stigma Scale (PISS), Dermatology Life Quality Index (DLQI), Perceived Health Status (PHS), and the General Health Questionnaire (GHQ)-12 were the scales used in the study.ResultsThe mean PISS was 58.48±14.9 in pediatric group. When PISS subscales of groups were compared, the pediatric group had significantly higher stigma resistance (p=0.01) whereas adult group had higher scores of alienation (p=0.01) and stereotype endorsement (p=0.04). There was a strong correlation between mean values of PISS and DLQI (r=0.423, p=0.001). High internalized stigma scores had no relation to either the severity or localization of disease in pediatric group. However, poor PHS (p=0.007) and low-income levels (p=0.03) in both groups, and body mass index (r=0.181, p=0.04) in the pediatric group were related to high PISS scores.ConclusionInternalized stigma in pediatric patients is as high as adults and is related to poor quality of life, general health, and psychological illnesses. Unlike adults, internalized stigma was mainly determined by psoriasis per se, rather than disease severity or involvement of visible body parts, genitalia or folds.