The association of cystic nephroma with pulmonary sequestration: is it a coincidence or not?

Cystic nephroma (CN) is a rare, presumably benign, multilocular cystic renal tumor. Pulmonary sequestration (PS) also presents as cystic masses of non-functioning primitive lung tissue. We describe a 15-month-old girl with CN and PS. Although some rare associations of renal and pulmonary lesions have been reported, this is the first case report in the English literature that shows the association of CN with PS.     

Combining gastric and ileal segments,does it overcome segment-related complications? An experimental study on rats

Bladder augmentation has revolutionized the care of children with neurogenic bladder but it is associated with certain short- and long-term complications. Using the combination of gastric and ileal segments to balance effects of these segments might be a solution for complications. A total of 39 female Spraque-Dawley rats randomly divided into four groups: ileocystoplasty (11), gastrocystoplasty (9), ileogastrocystoplasty (11) and control (8). Serum/urine electrolytes and pH values, and serum creatinine levels and urine mucus concentration were measured. Kruskal–Wallis non-parametric variance analysis was performed to compare the groups and p < 0.05 was accepted as significant. Metabolic alkalosis with significantly lower urine pH was observed in gastrocystoplasty group. Gastroileal group showed similar results with the ileal group in all parameters. No stone formation was detected in the sham and gastric cystoplasty groups. Metaplastic and hyperplastic changes were observed in all segments surrounding urothelium. In conclusion, combination of gastric and ileal segments does not significantly reduce the rate of metabolic impairments, stone and mucus formation. Besides it is not associated with significant improvement in histological outcome since urine is still in contact with the gastrointestinal mucosa.     

Pretransplantation serum FT3 concentration in kidney recipients is useful to identify higher risk of graft failure

Background

Free triiodothyronine (FT₃) is a marker of comorbidity in end-stage renal disease and in many acute and chronic diseases. The objective of the present study was to investigate the effects of pretransplantation FT₃ concentration on graft function.

Materials and Methods

Between 2003 and 2008, the study enrolled 86 patients who underwent renal transplantation. Twenty-six patients were women, and 60 were men, with overall mean (SD) age of 38 (10) years. In all patients, serum FT₃, free thyroxine, and thyroid-stimulating hormone concentrations were determined before transplantation. Demographic data and laboratory values were evaluated at 2 years posttransplantation.

Results

The overall graft survival rate at 2 years was 82.1%. Pretransplantation serum FT₃ concentration was inversely correlated with 2-year serum creatinine concentration (r = −0.29; P = .01) and proteinuria (r = −0.37; P < .00). Linear regression analysis demonstrated that serum FT₃ (r² = 0.63; 95% confidence interval, 0.52-0.74; P = .00) was a statistically significant risk factor for increased serum creatinine concentration. No correlation was observed for thyroid-stimulating hormone or free thyroxine and posttransplantation data.

Conclusion

Patients with end-stage renal disease with low pretransplantation serum FT₃ concentration are at greater risk of subsequent graft failure. Measurement of pretransplantation serum FT₃ concentration could be a clinically useful method of identifying patients at increased risk of graft failure.     

Late onset hemorrhagic cystitis in a hematopoietic stem cell recipient: Treatment with intravesical hyaluronic acid

Çipe Erol F, Soygür T, Do?u F, Erdo?an Ö, Bozdo?an G, ?kincio?ullar? A. Late onset hemorrhagic cystitis in a hematopoietic stem cell recipient: Treatment with intravesical hyaluronic acid.
Pediatr Transplantation 2010: 14:E79–E82. © 2009 Wiley Periodicals, Inc. Abstract: HC is a common complication following HSCT. Risk factors include viral infections, cyclophosphamide and busulfan usage, pelvic irradiation, older age at transplantation, allogeneic HSCT and GvHD. The severity of HC ranges from mild hematuria to life‐threatening bleeding. Here, we present a seven‐and‐a‐half‐yr‐old boy with Wiskott‐Aldrich syndrome who experienced a late onset Grade III hemorrhagic cystitis following HSCT from his fully matched sibling. A Grade I GvHD localized to skin developed on day +11 and prednisolone therapy was given between the 11th and 22nd d. Myeloid and platelet engraftments were achieved +13 and +16 d, respectively. A gross hematuria began on the 21st post‐transplant day. The urine cultures for bacterial or fungal organisms were negative. Urine analysis by PCR revealed a CMV viruria. Following systemic ganciclovir treatment, urinary CMV became negative but hemorrhagic cystitis did not improve. Due to the probability of existing BK virus or adenovirus, two doses of cidofovir were administered intravesically. As he continued to have painful hematuria with large clot formations, two doses of intravesical hyaluronic acid were applied. Macroscopic hematuria resolved within four d after the second dose. Complete remission was achieved on day +77. Finally, intravesical administration of hyaluronic acid seems to be effective and safe and can be a promising treatment in patients suffering from severe and late onset HC.     

Fluoroscopically guided endoluminal balloon dilation of oesophageal stricture due to dystrophic epidermolysis bullosa in two sisters

Dystrophic epidermolysis bullosa is an inherited disorder with frequent oesophageal stricture formation. There is no satisfactory medical treatment of dysphagia however; endoluminal balloon dilation is a novel method with satisfactory results. Intrafamilial cases of dystrophic epidermolysis bullosa manifest variable clinical presentations. We report two sisters with dystrophic epidermolysis bullosa simultaneously presenting with dysphagia. Fluoroscopically guided endoscopic balloon dilation revealed almost complete resolution of dysphagia in both patients. Our cases represented a striking similarity in their clinical picture and response to treatment. Balloon dilation in these cases is a safe and effective approach.     

Effects of sevoflurane on QT dispersion and heart rate variability

The purpose of this study was to use estimates of corrected QT dispersion (QTcd) and heart rate variability (HRV) to assess the effects of sevoflurane, an inhalation agent used frequently in clinical practice, on autonomic cardiac function. This study was conducted prospectively and in a blind manner on 20 women between 38 and 51 y of age who were classified as American Society of Anesthesiologists stage I–II and whose treatment required total abdominal hysterectomy. Electrocardiograms were recorded by 12-lead Holter monitor for 5 min before sevoflurane induction and again for 5 min at 10 min after tracheal intubation. Data on the first recording were considered as baseline; those on the second recording were viewed as final data. The study was terminated at this point, and surgery was allowed to proceed. QTcd and HRV values were assessed by a cardiologist, who was blinded to all data. All parameters were expressed as a mean value ± standard deviation. Wilcoxon’s test was used to compare baseline and final data. Statistical significance was considered asP< .05. No significant changes were observed between baseline and final QTcd values and between low and high-frequency components (LF and HF) of HRV; nor were changes seen in the LF/HF ratio. With the patient under sevoflurane/nitrous oxide anesthesia, no significant changes were detected in QTcd, LF, and HF values, and in the LF/HF ratio, whereas a significant increase (P=.001) was seen in standard deviation of the R-R interval, which was used as a measure of cardiac autonomic tone.     

Epigenetics and pancreatic cancer:Pathophysiology and novel treatment aspects

An improvement in pancreatic cancer treatment represents an urgent medical goal.Late diagnosis and high intrinsic resistance to conventional chemotherapy has led to a dismal overall prognosis that has remained unchanged during the past decades.Increasing knowledge about the molecular pathogenesis of the disease has shown that genetic alterations,such as mutations of K-ras,and especially epigenetic dysregulation of tumor-associated genes,such as silencing of the tumor suppressor p16ink4a,are hallmarks of pancreatic cancer.Here,we describe genes that are commonly affected by epigenetic dysregulation in pancreatic cancer via DNA methylation,histone acetylation or miRNA(microRNA)expression,and review the implications on pancreatic cancer biology such as epithelial-mesenchymal transition,morphological pattern formation,or cancer stem cell regulation during carcinogenesis from PanIN(pancreatic intraepithelial lesions)to invasive cancer and resistance development.Epigenetic drugs,such as DNA methyltransferases or histone deactylase inhibitors,have shown promising preclinical results in pancreatic cancer and are currently in early phases of clinical development.Combinations of epigenetic drugs with established cytotoxic drugs or targeted therapies are promising approaches to improve the poor response and survival rate of pancreatic cancer patients.