Juvenile dermatomyositis presenting with anasarca

Generalized edema can occur during the course of juvenile dermatomyositis. In this article, a 4-year-old boy with generalized nonpitting edema and proximal weakness is reported. Characteristic cutaneous lesions, laboratory tests, results of electromyography, and magnetic resonance imaging findings resulted in a diagnosis of juvenile dermatomyositis. He was treated with corticosteroids and methotrexate. It is concluded that the generalized edema may appear as the presenting cutaneous manifestation of juvenile dermatomyositis.     

Amphotericin B deoxycholate (d-AMB) use in cases with febrile neutropenia and fungal infections: lower toxicity with suitable premedication

In spite of the development of new antifungal drugs, amphotericin B deoxycholate (d-AMB) remains the gold standard in the treatment of severe fungal infections in immunosuppressed hosts. However, d-AMB is a toxic drug, the most important dose-limiting toxicities being nephrotoxicity and infusion-related allergic reactions. Lipid and liposomal formulations of d-AMB have relatively lower toxicity and are considered alternative choices. However, the routine use of these formulations is limited by their higher cost. Using retrospective analysis, we explored the incidence of nephrotoxicity and allergic reactions requiring the cessation of conventional d-AMB in 113 cases treated with the drug. In contrast to knowledge in the relevant literature, we did not detect significant toxicity, which would have required discontinuation of the d-AMB treatment. Mean serum creatinine levels were 0.72 +/- 0.25 and 0.84 +/- 0.31 mg dl(-1) before and after therapy, respectively. Although the difference between creatinine levels before and after d-AMB is statistically significant, the creatinine level increased twofold in only eight cases. Mean serum potassium levels were 3.8 +/- 0.54 and 3.6 +/- 0.7 mmol l(-1) before and after d-AMB respectively. Potassium levels below 3 mmol l(-1) were found in 7 and 17 cases before and after d-AMB respectively. Potassium levels were statistically lower in cases with fungal mucositis. Severe infusion-related allergic reactions were observed in three cases. Antihistamine and corticosteroid were added to the treatment in these cases. With these findings, we can conclude that d-AMB is a tolerable, low cost drug which can be safely used provided there is suitable premedication and monitoring of blood urea nitrogen, serum potassium and magnesium levels.     

Gravin gene expression in acute leukaemias: clinical importance and review of the literature

The aim of this study was to determine the expression of Gravin (a tumor suppressor gene belonging to the A kinase anchoring protein family) in samples of acute leukaemia and to explore its association with the prognosis. The study group consisted of 162 people (137 patients with acute leukaemia and 25 volunteers as control). Real-time quantitative PCR was used to determine the gene expression and beta Actin used as a control gene. The results were evaluated with Comparative Ct method. Gravin beta-Actin DeltaCt was statistically different between patients and controls (p value < 0.001). The Gravin expression was found to be decreased 11-fold when compared with controls and was found to be decreased in 106 cases (77.5%). There was an inverse correlation between gravin expression and overall survival. The Gravin expression was found to be decreased in samples of acute leukaemia and was associated with an inferior overall survival. Because of paradoxical results, there is a need for more studies exploring gravin isoforms and other related gene expressions.     

Effect of cervical length on second trimester pregnancy termination

AIM: To evaluate the value of sonographic measurement of cervical length as a predictor of abortion or delivery within 24 h by misoprostol in second trimester termination of pregnancy (TOP). MATERIAL AND METHODS: One hundred and sixty-three women underwent TOP between 14 and 26 weeks of pregnancy due to various indications. The primary outcome was abortion within 24 h. Cervical length was measured before transvaginal administration of misoprostol. The effects of cervical length, total misoprostol dose, parity, and gestational age at diagnosis on successful TOP were evaluated. Results: One hundred and sixty-three women were eligible who met the inclusion criteria. TOP occurred in 80.5% of patients within 24 h. Parous women had shorter prolonged induction to expulsion period over 24 h (14.1% vs 28.6%, P = 0.061). Total misoprostol dose and history of abortion were parameters that affected induction to delivery period (P = 0.002 and P = 0.041). Using an optimum cutoff of 36 mm, 58.2% sensitivity and 68.2% specificity were obtained. In addition, positive and negative predictive values were 85.36% and 33.3%, respectively. Pregnant women whose preinduction cervical length was shorter than 36 mm had a shorter induction time and needed a lower total misoprostol dose to achieve TOP than women with a cervical length longer than 36 mm (P = 0.027 and P = 0.011, respectively). Conclusion: Transvaginal measurement of cervical length before administration of prostaglandin analogue was not correlated with successful TOP within 24 h. It cannot be used as a predictor in light of our findings.     

Serum vascular endothelial growth factor,insulin-like growth factor-1 and aflibercept levels in retinopathy of prematurity

Japanese Journal of Ophthalmology - To evaluate the serum levels of vascular endothelial growth factor (VEGF) and insulin-like growth factor-1 (IGF-1) after intravitreal injection of aflibercept...     

A clinically isolated syndrome: A challenging entity

Abstract   Acute isolated neurological syndromes, such as optic neuropathy or transverse myelopathy, may cause diagnostic problems since they can be the first presentations of a number of diseases such as multiple sclerosis (MS) and collageneous tissue disorders. In the present study, particular systemic lupus erythematosus (SLE) and primary Sjogren syndrome (pSS) patients, who were followed up with the initial diagnosis of possible MS with no evidence of collagen tissue disorders for several years, are described. Five patients with the final diagnosis of SLE and five pSS patients are evaluated with their neurologic, systemic and radiologic findings. Over several years, all developed some systemic symptoms like arthritis, arthralgia, headache, dry mouth and eyes unexpected in MS. During the regular and close follow-up laboratory evaluations of vasculitic markers revealed positivity, leading to the final definite diagnosis of SLE or pSS. Patients with atypical neurological presentation of MS, a relapsing remitting clinical profile, or lack of response to the regular MS treatment should be evaluated for the presence of a connective tissue disease. Various laboratory tests, such as cerebrospinal fluid findings, autoantibodies profile, markers, cranial and spinal magnetic resonance imaging, can be helpful for the differential diagnosis. Lack of response to the regular multiple sclerosis treatment, even increasing rate of relapses can force the clinician for the differential diagnosis. In particular cases an accurate diagnosis can only be made after close follow-up.     

Alterations in ocular surface and corneal thickness in relation to metabolic control in patients with chronic renal failure

AIM: Ocular surface changes and ocular symptoms may be encountered in patients with chronic renal failure (CRF) undergoing haemodialysis. The ocular surface changes and its relationship with metabolic control in CRF patients were aimed to be emphasized in this study. METHODS: Thirty-eight CRF patients (75 eyes) undergoing haemodialysis were enrolled. Patients underwent a complete ocular examination together with Schirmer, tear break-up time tests, pachymetric measurements and conjunctival impression cytologies. Blood calcium, phosphate levels and total body volume changes after haemodialysis were recorded. RESULTS: The most common findings were conjunctival calcification with red eye (81.3%) and dry eye (62.7%, according to tear break-up time test) in 75 eyes of 38 patients. Impression cytologies were graded as 0 in 57.3% of eyes and 2-3 in 40% of eyes showing positive correlation with the extent of conjunctival calcification (R = 0.486, P = 0.0001). Serum calcium and phosphate levels were also positively correlated with the degree of conjunctival calcification (R = 0.684, P = 0.0001 and R = 0.428, P = 0.0001, respectively) as well as with the grades of impression cytology (R = 0.587, P = 0.0001 and R = 0.385, P = 0.0001, respectively). Furthermore, the mean corneal thickness decreased significantly (9.31 +/- 26.9 mum) following haemodialysis (Paired t-test, P = 0.002). CONCLUSION: Dry eye and irritational symptoms are major ocular symptoms in CRF patients. Serum calcium and phosphate levels seem to have a prognostic importance for the ocular findings and symptoms in patients with CRF.     

Survivin and EPR-1 expression in acute leukemias: prognostic significance and review of the literature

The aim of this study is to detect the biologic and/or prognostic significance of survivin (S) and effector protease receptor 1: EPR-1 (E) expression in acute leukemias (34 ALL and 40 AML) by using RT-PCR. S and E expressions were found in 83.8 and 20.3% of the cases, respectively. S was detected in 90%, 76.5% and E was detected in 17.5%, 23.5% of the cases with AML and ALL, respectively. There was a significant correlation between S and E (r=0.30 p=0.01). Mortality rate was higher in E(-) cases than E(+) cases (83.1 % versus 66.7%) (p=0.04). The median DFS and OS rates were shorter in S(+) and E(-) cases. In subgroup analysis, there was not a significant difference for OS between S(-) and S(+) cases and E(-) and E(+) cases in ALL group. The median OS rate was significantly longer in S(-) cases than S(+) cases, and longer in E(+) cases than E(-) in AML groups (p=0.04, 0.001, respectively). OS and DFS rates were longest in S(-) E(+) cases and shortest in S(+) E(-) cases (p=0.04 and 0.03, respectively). In multivariate analyses EPR1 negativity was found to be an independent poor risk factor for survival (OR: 2.4, p=0.02). In conclusion S expression is a bad prognostic indicator in cases with acute leukemia especially in AML. S negativity and E positivity show good clinical outcome in acute leukemias. E expression is important due to its property of the possible natural anti-sense of the S.     

Effect of Non‐Surgical Periodontal Treatment on Gingival Crevicular Fluid and Serum Endocan,Vascular Endothelial Growth Factor‐A,and Tumor Necrosis Factor‐Alpha Levels

Background: Periodontitis is a chronic inflammatory disease that occurs due to the interaction between pathogenic microorganisms and host defenses. Endocan is a proteoglycan secreted by endothelial cells under the control of inflammatory cytokines. Aims of the study are to determine serum and gingival crevicular fluid (GCF) endocan levels in the pathogenesis of periodontal diseases, supported with vascular endothelial growth factor (VEGF‐A) and tumor necrosis factor (TNF)‐alpha levels. This study additionally aims to evaluate correlation between GCF endocan levels, VEGF‐A, and TNF‐α levels with periodontal probing depth (PD). Methods: The study consists of two groups: group 1 (n = 20), healthy individuals; group 2 (n = 20), individuals with generalized chronic periodontitis (CP). Clinical measurements were recorded; GCF and serum samples were obtained from each participant before and 6 weeks after therapy. Levels of biomarkers were measured by enzyme‐linked immunosorbent assay. Intergroup comparisons of biochemical and clinical parameters were analyzed by Kruskal–Wallis/Bonferroni‐adjusted Mann–Whitney U test using statistical software. Results: Serum and GCF endocan, VEGF‐A, and TNF‐α levels were significantly higher in patients with CP than in healthy individuals (P <0.001) and decreased after treatment (P <0.03). A significant correlation was observed between GCF TNF‐α and PD (4 mm ≤ PD ≤5 mm and PD ≥6 mm). A significant relationship was found among GCF endocan and TNF‐α, VEGF‐A, CAL, and GI for all groups (P <0.05). Conclusions: Endocan and TNF‐α levels, both in GCF and serum, increased from health to periodontitis and decreased with non‐surgical periodontal treatment. Within the limits of the study, endocan may be considered as a potential inflammatory marker for periodontal disease.