Hitherto undescribed venous vacuolar myopathy without mucoid degeneration in the varicose saphenous vein of a child

Varicose vein abnormalities involving the lesser saphenous vein of a 7-year-old boy are presented. The histopathology of the vein wall revealed a vacuolar degeneration without mucoid deposits of the muscle cells, which has not been described previously in the literature in congenital varices.     

Effect of creatine and pioglitazone on Hk-2 cell line cisplatin nephrotoxicity

Cisplatin is a chemotherapeutic agent, which is used in the treatment of various solid organ cancers, and its main dose limiting side effect of cisplatin is nephrotoxicity. The aim of this study is to investigate the role of pioglitazone and creatine on cisplatin nephrotoxicity in vitro. Real-time cell analyzer system (RTCA) was used for real-time and time-dependent analysis of the cellular response of HK-2 cells following incubation with cisplatin and combination with creatine or pioglitazone hydrochloride. First, half-maximal inhibitory concentrations (IC50) of cisplatin, creatine and pioglitazone were calculated by RTCA system. Afterwards creatine and pioglitazone was administered with serial dilutions under RTCA system. IC50 dose for cisplatin was 7.69?M?×?10^?5 at 24th hour and 3.93?M?×?10^?6 at 48th hour. IC50 dose for pioglitazone was 1.61?M?×?10^?3 at 24th hour and 2.85?M?×?10^?4 at 48th hour. Although cells were treated the dose of 40,225?mM creatine, IC50 dose could not been reached. Neither pioglitazone nor creatine had additional protective effect in any dose. Consequently, beneficial effect of creatine and pioglitazone on cisplatin-induced cell death could not be found. Further studies and clinical trials are needed to evaluate the effect of different doses of these drugs in cisplatin-induced nephrotoxicity.     

Eculizumab therapy in a patient with dense-deposit disease associated with partial lipodystropy

Background

Dense deposit disease (DDD) (also known as membranoproliferative glomerulonephritis type II) in childhood is a rare glomerulonephritis with frequent progression to end-stage renal disease (ESRD) and a high recurrence after kidney transplantation. The pathophysiologic basis of DDD is associated with the uncontrolled systemic activation of the alternative pathway (AP) of the complement cascade.

Case-diagnosis/treatment

A 14-year-old girl presented with edema and nephrotic range proteinuria. Blood tests showed hypoalbuminemia, nephrotic range proteinuria, normal renal function, and a low C3 level. Renal biopsy confirmed the diagnosis of crescentic DDD. Complement analysis revealed strong AP activation (low C3), positive C3 nephritic factor (C3NeF), and a decreased complement factor H (CFH) levels with CFH polymorphisms. Therapy with eculizumab was considered after the failure of corticosteroid and plasmapheresis to modulate the ongoing massive proteinuria and persistence of low serum C3 levels. There was a marked clinical and biochemical response following the administration of eculizumab.

Conclusions

Our case emphasizes the efficacy of eculizumab in the management of crescentic DDD in a patient with a normal renal function, in a short follow-up period. Considering previously reported cases, it appears that eculizumab represents a promising new approach which may prevent progression to ESRD in a subset of patients with DDD.     

Results of intravitreal triamcinolone acetonide in patients with macular edema secondary to branch retinal vein occlusion

Background Macular edema is one of the most common causes of visual loss in patients with retinal vein oclusions. Intravitreal corticosteroids and intravitreal anti-vascular endothelial growth factors are modalities of treatment for macular edema secondary to branch retinal vein occlusion (BRVO). Objective To present the results of intravitreal triamcinolone acetonide therapy in patients with macular edema secondary to BRVO. Setting A retrospective clinical interventional study included 32 patients with macular edema secondary to BRVO. Method The records of 32 eyes of 32 patients who received 4 mg/0.1 mL injection of intravitreal triamcinlone for macular edema secondary to BRVO were evaluated. Patients with visual acuity <0.40 logMAR (logarithm of the minimum angle of resolution), central macular thickness (CMT) >260 μm and no neovascularization at baseline were included. Patients with diabetes mellitus, a history of intravitreal anti vascular endothelial growth factor injection, grid laser photocoagulation and ischemic BRVO were excluded. The re-injections were performed in cases with increased CMT >100 μm or vision loss of five or more letters. Results The mean follow-up was 12 ± 1.9 months. The visual acuity increased from 0.58 ± 0.16 at baseline to 0.25 ± 0.11 logMAR (P < 0.001). The CMT decreased from 490 ± 107 μm at baseline to 266 ± 90 μm at 12 months (P < 0.001). Both cataract and glaucoma developed in 18.75 % patients. Conclusion Intravitreal triamcinolone, due to absence of systemic side effects, can be used with confidence for treatment macular edema secondary to BRVO. However the main disadvantages of intravitreal triamcinolone injection are elevation of intraocular pressure and formation of cataract.     

Do Proinflammatory Cytokine Levels Predict Serious Complication Risk of Infection in Pediatric Cancer Patients?

Determination of risk of severe bacterial infection complication in children with cancer is important to diminish the cost of hospitalization and therapy. In this study, children with cancer (leukemia excluded) were evaluated for risk of severe infection complication, success of therapy and the relation between clinical and inflammatory parameters during neutropenic fever attacks. Children who fulfilled the criteria of neutropenic fever with cancer were enrolled in the study. During admission, together with clinical and laboratory parameters; interleukin-6, interleukin-8, soluble tumor necrosis factor receptor II, and soluble interleukin 2 reseptor ve procalcitonin levels were detected. Empirical therapy was started with piperacillin/tazobactam and relation between the inflammatory cytokine levels and therapy response parameters were evaluated. The study population included 31 children and 50 neutropenic attacks were studied. In 48% of the attacks, absolute neutrophile count was >100/mm³ and infectious agents were shown microbiologically in 12% of the attacks. In the study group with piperacillin/tazobactam monotherapy, the success rate without modification was 58%. In the therapy modified group mean duration of fever, antibiotherapy and hospitalization were significantly longer than the group without modification. Inflammatory cytokines’ levels during admission (interleukin-6, interleukin-8, soluble tumor necrosis factor reseptor II) were higher in patients with fever >3 days and in multiple regression analysis, it has been shown that they have a determinative role on fever control time. Other cytokines did not show any significant relationship with risk of severe bacterial infection complication and success of therapy.     

Sealer Penetration into Dentinal Tubules in the Presence or Absence of Smear Layer: A Confocal Laser Scanning Microscopic Study

Introduction

The aim of this study was to test the dentinal tubule penetration of AH26 (Dentsply DeTrey, Konstanz, Germany) and MTA Fillapex (Angelus, Londrina, PR, Brazil) in instrumented root canals obturated by using cold lateral compaction or warm vertical compaction techniques in either the presence or absence of the smear layer.

Methods

Forty-five extracted single-rooted human mandibular premolar teeth were used. The crowns were removed, and the root canals were instrumented by using the Self-Adjusting File (ReDent-Nova, Ra'anana, Israel) with continuous sodium hypochlorite (2.6%) irrigation. Final irrigation was either with 5% EDTA or with sodium hypochlorite. The canals were dried and obturated by using rhodamine B–labeled AH26 or MTA Fillapex in combination with the cold lateral compaction or the warm vertical compaction technique. After setting, the roots were sectioned horizontally at 4-, 8-, and 12-mm distances from the apical tip. On each section, sealer penetration in the dentinal tubules was measured by using confocal laser scanning microscopy.

Results

Regardless of the usage of EDTA, MTA Fillapex, compared with AH26, was associated with greater sealer penetration when used with the cold lateral compaction technique, and, conversely, AH26, compared with MTA Fillapex, was associated with greater sealer penetration when used with the warm vertical compaction technique (P < .05). Removal of the smear layer increased the penetration depth of MTA Fillapex used with the cold lateral compaction technique (P < .05); however, it had no significant effect on the penetration depth of AH26.

Conclusions

Greater sealer penetration could be achieved with either the MTA Fillapex–cold lateral compaction combination or with the AH26–warm vertical compaction combination. Smear layer removal was critical for the penetration of MTA Fillapex; however, the same did not hold for AH26.     

Effects of pirfenidone on experimental head injury in rats

Traumatic brain injury (TBI) continues to be a significant public healthcare concern. Neuroinflammation that occurs in the secondary phase of TBI leads to cognitive and physical dysfunction. A number of therapeutic modalities have been evaluated in an attempt to find a suitable treatment. The only drug approved for the treatment of idiopathic pulmonary fibrosis, pirfenidone, has been evaluated for its antifibrotic, anti-inflammatory, and anti-oxidant properties for various disorders, but this is the first study to examine its effects in an experimental TBI model. Twenty-four Wistar rats were randomly divided into three groups: control, trauma, and pirfenidone. The two latter groups underwent experimental diffuse cortical injury mimicking TBI. Neurological assessment was performed using the Garcia test, histological analysis was performed to examine neuroprotective and anti-inflammatory effects, and biochemical analyses of neuron-specific enolase (NSE), S-100B, caspase-3, and thiobarbituric acid reactive substances were performed. The pirfenidone group had a better Garcia test score (P=0.001), an increased anti-inflammatory effect (P<0.001), and an enhanced neuroprotective effect (P=0.007) along with decreased NSE, S100B, and TBARS levels compared to the trauma group. However, pirfenidone did not show a beneficial effect on caspase-3 levels. Pirfenidone may help decrease mortality and morbidity rates after TBI through its anti-inflammatory and antioxidant effects.     

Cognition of the mothers of patients with Duchenne muscular dystrophy

Duchenne muscular dystrophy (DMD) has been found to be associated with cognitive impairment. However, few studies have addressed cognitive impairment among mothers of children with DMD. In the present study, the neuropsychological profiles of both carrier mothers (C-Ms) and noncarrier mothers (NC-Ms) were examined, and the findings were compared with healthy control mothers (HC-Ms). There were 90 participants, consisting of 31 C-Ms, 24 NC-Ms, and 35 HC-Ms, each of whom completed a neuropsychological test battery. C-Ms had poorer cognition performance in attention, working memory, immediate verbal memory, visuospatial skills, and executive functions than NC-Ms, and HC-Ms. This study provides evidence that there may be cognitive impairment in mothers of patients with DMD. The cognitive impairment of C-Ms has similarities to that seen in children with DMD.