A giant ovarian cyst in a neonate with classical 21-hydroxylase deficiency with very high testosterone levels demonstrating a high-dose hook effect

Congenital adrenal hyperplasia (CAH) is a group of disorders affecting the adrenal steroid synthesis. The most common form, 21-hydroxylase deficiency (21-OHD), leads to decreased production of cortisol and aldosterone with increased androgen secretion. In classic CAH, glucocorticoid treatment can be life-saving and serves to bring the symptoms under control. However, the treatment challenge is to effectively control the excess androgen effect by using the lowest possible glucocorticoid dose. Previous studies suggested a relationship between ovarian cyst formation and adrenal androgen excess, but neonatal large ovarian cysts have been very rarely reported in newborns with CAH. Here, we present the unique case of a neonate with classical 21-OHD who underwent surgery for a giant (10x8x7 cm) unilateral solitary ovarian follicular cyst on the 2nd postnatal day. Hormonal evaluation of the patient revealed high-dose hook effect for serum testosterone levels for the first time by a two-site immunoradiometric assay. Possible mechanisms by which androgen excess may cause ovarian cyst formation are discussed.     

Acute presentation of autoimmune hepatitis in a patient with myasthenia gravis, thymoma, Hashimoto thyroiditis and connective tissue disorder

Myasthenia gravis is an antibody-mediated autoimmune disease at the neuromuscular junctions. It can be associated with many other autoimmune diseases. We report a case of acute presentation of autoimmune hepatitis with myasthenia gravis, thymoma, Hashimoto thyroiditis and connective tissue disorder.     

Urethral stromal tumor with pacemaker cell phenotype

Penile malignancies are rare in developed countries. The authors present a case of a penile urethral mesenchymal tumor occurring in a 51-year-old Caucasian male and displaying light microscopic, immunohistochemical, and ultrastructural features suggestive of a pacemaker cell type, combined with a lack of diagnostic features of any other established tumor category. The immunohistochemical profile was intensely positive for vimentin, PKC θ, and NSE and weakly positive to nonreactive for CD34 and smooth muscle actin, and entirely negative for CD117 (c-kit), S-100, and other markers. C-kit and PDGFRA gene analysis showed no mutations. Electron microscopy revealed tumor cells with plentiful cytoplasm and cytoplasmic processes/filopodia, both filled with intermediate filaments and occasional solitary focal densities. There were also prominent smooth endoplasmic reticulum cisternae, caveolae, neurosecretory granules, particularly concentrated in cytoplasmic processes, and synaptic-type structures. Poorly formed basal lamina, gap junctions, and intercellular collagen aggregates, consistent with skeinoid-type fibers, were also noted. Interstitial cells with potential pacemaker function have been recently described in the lower urinary tract, including the urethra, and this tumor may be related to this cellular phenotype.     

Influence of luting agent on the microleakage of all-ceramic crowns

PURPOSE: In this in-vitro study, microleakage of all-ceramic crowns was evaluated at enamel and dentin margins. MATERIALS AND METHODS: Forty maxillary central incisors were randomly divided into 4 groups (n = 10). While buccal and palatal margins were placed on enamel, mesial and distal margins were placed below the cementoenamel junction. In groups 1 to 3, IPS Empress 2 crowns were luted with Variolink 2/Syntac Classic (group 1), Bifix DC/Solobond Plus (group 2) and Calibra/Prime & Bond NT combinations (group 3), respectively. In the control group (group 4), porcelain-fused-to-metal crowns were luted with a zinc-phosphate cement. All specimens were subjected to 5000 thermocycles (at 5 degrees C to 55 degrees C; 30-s dwell time). After immersion in India ink for 48 h at 37 degrees C, the specimens were sectioned both buccolingually and mesiodistally. Each section was evaluated for microleakage under a stereomicroscope at 24X magnification. RESULTS: According to the Krukal-Wallis test, in all groups, there were significant differences in microleakage at the enamel margins (p = 0.001). Nevertheless, the margins finished in dentin showed no significant differences (p = 0.163). According to the Mann-Whitney U-test, statistically significant differences were observed in microleakage between groups 1 and 3 (p = 0.049), groups 1 and 4 (p = 0.001), groups 2 and 4 (p = 0.002), and between groups 3 and 4 (p = 0.045) at the enamel margin. In group 1, significantly greater microleakage was observed at the dentin margin compared to the enamel margin (p = 0.007). CONCLUSION: The adhesive luting technique demonstrated an excellent ability to minimize microleakage of all-ceramic crowns at the enamel margins. Water-based dentin bonding systems showed less microleakage than the water-free acetone-based dentin bonding system at the enamel margin.     

A comparison of the efficacy and safety of nonsteroidal antiinflammatory agents versus acetaminophen in the treatment of osteoarthritis: a meta-analysis

OBJECTIVE: To perform a meta-analysis comparing the efficacy and safety of recommended dosages of nonsteroidal antiinflammatory drugs (NSAIDs), including cyclooxygenase 2 inhibitors, versus acetaminophen in the treatment of symptomatic hip and knee osteoarthritis. METHODS: Medline and EMBASE searches were performed for original clinical trials directly comparing NSAIDs with acetaminophen. A standardized form was used to abstract all data, including outcome measures of pain at rest, walking pain, and dropouts due to adverse effects. Inverse-variance-weighted mean differences (WMDs) and 95% confidence intervals (95% CI) for pain measures were determined for treatment groups. Odds ratios (ORs) and 95% CIs were calculated for withdrawals due to adverse events. Results were compared using a random effects model. RESULTS: Seven articles met inclusion criteria with sufficient data for analysis. Participants had a mean age of 61.1 years and 71.1% were women. Test of heterogeneity was not significant for either rest (P = 0.73) or walking (P = 0.76) pain. The scores for overall pain at rest (WMD -6.33 mm on a 100-mm visual analog scale [VAS], 95% CI -9.24, -3.41) and walking pain (WMD -5.76 mm on a 100-mm VAS, 95% CI -8.99, -2.52) favored the NSAID-treated group. Although NSAIDs elevated the risk of withdrawals due to adverse events, the difference was not statistically significant (OR 1.45, 95% CI 0.93, 2.27). CONCLUSION: NSAIDs are statistically superior in reducing rest and walking pain compared with acetaminophen for symptomatic osteoarthritis. Safety, measured by discontinuation due to adverse events, was not statistically different between NSAID- and acetaminophen-treated groups.     

Primary Ovarian Malignant Melanoma Arising in Teratomatous Component of Mixed- Germ Cell Tumor in a Child: Case report

Primary ovarian malignant melanoma arising in teratomatous component of germ cell tumors is seen extremely rare with most reports being only of single cases and small series in reproductive aged woman and mostly from cystic teratoma, whereas information on pediatric presentation is sparse. This case is reported for being extremely rare tumor.