Cross-cultural adaptation, reliability, and validity of the Turkish version of the Exercise Therapy Burden Questionnaire for individuals with chronic diseases.

OBJECTIVE: Chronic diseases, which caused 36 million deaths in 2008, are the most common cause of death worldwide. Exercise is one of the non-pharmacological treatment methods. Although exercise benefits are well known, more than half of the population does not exercise due to the burden of exercise. The objectives of the current study were to evaluate the Turkish version of the Exercise Therapy Burden Questionnaire (ETBQ-T) and to investigate its reliability and validity. METHODS: A total of 100 participants (female: 69, male: 31) who were diagnosed with at least one chronic disease participated in the translation validity and reliability analysis of the study. Cross-cultural adaptation of the ETBQ-T was performed according to Beaton's guidelines. The ETBQ-T, the European Quality of Life 5 Dimensions (EQ-5D), pain, satisfaction, and self-efficacy were applied for convergent validity. The ETBQ-T was retested to examine its reliability after 7 days. RESULTS: The internal consistency and reliability were excellent (intraclass correlation coefficient=0.959; Cronbach's α=0.919). The standard error of measurement was reported as 5.35. The minimum detectable difference was also demonstrated at 1.35. The ETBQ-T had a good correlation with pain (r=0.545, p<0.001), satisfaction (r=-501, p<0.001), and self-efficacy (r=-0.579, p<0.001). However, the correlation of the ETBQ-T with EQ-5D (r=0.340, p=0.001) was weak. A factor was extracted, accounting for 58.289% of the total variation. There were no floor or ceiling effects. CONCLUSIONS: The ETBQ-T is a reliable and valid tool to evaluate the exercise burden in the Turkish population with chronic disease.

Early onset neonatal thrombocytopenia is associated with severe intraventricular hemorrhage in very preterm infants: A retrospective cohort study.

BACKGROUND: The role of platelet function in the development of intraventricular hemorrhage is still a subject of debate. In this study, we aimed to determine whether there is an association between platelet indices in the first week of life and severity of intraventricular hemorrhage in very preterm infants. MATERIALS AND METHODS: Preterm infants born < 30 weeks of gestation in our hospital were retrospectively evaluated. Platelet parameters, including platelet counts, mean platelet volume, platelet distribution width, and platelet mass were retrieved at two different time points: the initial value on the first day of life and the value closest to the end of the first week of life. The infants were categorized according to the findings of cranial ultrasonography as; no intraventricular hemorrhage, mild or severe intraventricular hemorrhage. RESULTS: Totally, 1051 infants were evaluated. The mean gestational age and birth weight for the entire cohort were 27.9±1.6 weeks and 1058±247 g, respectively. Infants in the severe intraventricular hemorrhage group had significantly lower gestational age (p < 0.001) and birthweight (p < 0.001) compared to other two groups. Furthermore, there were significant differences in platelet count and platelet mass between the groups at two time intervals. However, logistic regression analysis revealed that only platelet count of < 100×109/L on the first postnatal day was independently associated with the severity of intraventricular hemorrhage. CONCLUSION: There is an association between platelet count of < 100×109/L on the first postnatal day and severe intraventricular hemorrhage in very preterm infants.

Sulfatase-2 from Cancer Associated Fibroblasts: An Environmental Target for Hepatocellular Carcinoma?

Introduction: Heparin sulphate proteoglycans in the liver tumour microenvironment (TME) are key regulators of cell signalling, modulated by sulfatase-2 (SULF2). SULF2 overexpression occurs in hepatocellular carcinoma (HCC). Our aims were to define the nature and impact of SULF2 in the HCC TME. Methods: In liver biopsies from 60 patients with HCC, expression and localization of SULF2 were analysed associated with clinical parameters and outcome. Functional and mechanistic impacts were assessed with immunohistochemistry (IHC), in silico using The Cancer Genome Atlas (TGCA), in primary isolated cancer activated fibroblasts, in monocultures, in 3D spheroids, and in an independent cohort of 20 patients referred for sorafenib. IHC targets included αSMA, glypican-3, ß-catenin, RelA-P-ser536, CD4, CD8, CD66b, CD45, CD68, and CD163. SULF2 impact of peripheral blood mononuclear cells was assessed by migration assays, with characterization of immune cell phenotype using fluorescent activated cell sorting. Results: We report that while SULF2 was expressed in tumour cells in 15% (9/60) of cases, associated with advanced tumour stage and type 2 diabetes, SULF2 was more commonly expressed in cancer-associated fibroblasts (CAFs) (52%) and independently associated with shorter survival (7.2 vs. 29.2 months, p = 0.003). Stromal SULF2 modulated glypican-3/ß-catenin signalling in vitro, although in vivo associations suggested additional mechanisms underlying the CAF-SULF2 impact on prognosis. Stromal SULF2 was released by CAFS isolated from human HCC. It was induced by TGFß1, promoted HCC proliferation and sorafenib resistance, with CAF-SULF2 linked to TGFß1 and immune exhaustion in TGCA HCC patients. Autocrine activation of PDGFRß/STAT3 signalling was evident in stromal cells, with the release of the potent monocyte/macrophage chemoattractant CCL2 in vitro. In human PBMCs, SULF2 preferentially induced the migration of macrophage precursors (monocytes), inducing a phenotypic change consistent with immune exhaustion. In human HCC tissues, CAF-SULF2 was associated with increased macrophage recruitment, with tumouroid studies showing stromal-derived SULF2-induced paracrine activation of the IKKß/NF-κB pathway, tumour cell proliferation, invasion, and sorafenib resistance. Conclusion: SULF2 derived from CAFs modulates glypican-3/ß-catenin signalling but also the HCC immune TME, associated with tumour progression and therapy resistance via activation of the TAK1/IKKß/NF-κB pathway. It is an attractive target for combination therapies for patients with HCC.

Monitoring of near-infrared spectrum values during packed red blood cell transfusion in pediatric intensive care unit.

OBJECTIVES: Packed red blood cell (PRBC) transfusion is one of the most common treatment options in pediatric intensive care unit (PICU) which targets a better cerebral oxygenation. This study aimed to show the cerebral near-infrared spectroscopy (cNIRS) changes during PRBC transfusions in PICU. MATERIAL AND METHODS: In this prospective observational study, changes in regional cerebral tissue oxygen saturation (rSO2) in pediatric patients, who required PRBC transfusion were monitored. All the cNIRS and related values were classified as baseline values. The same values were measured and calculated at the end of transfusion and named as 4th-hour values. Further measurements and calculations were made three hours later and named as 7th-hour values. Changes in cNIRS, cerebral tissue fractional oxygen extraction (CTFOE), cNIRS variability index (cNIRS-VI) were compared using Friedman test. RESULTS: A total of 53 PRBC transfusions were monitored. Baseline haemoglobin increased from 6.3 (5.9, 6.7) gr/dL to 8.6 (8.4, 9) gr/dL at the 7th-hour. cNIRS values improved during transfusion (P=0.012), with a concomitant decrease in cNIRS-VI and CTFOE values (P<0.001 and P=0.017 consecutively) CONCLUSION: Our study revealed that there is an increase in cNIRS and related values after transfusion compared to baseline values in critically ill children admitted to a PICU. Age of PRBC did not have an effect on delta-cNIRS or post-transfusion hemoglobin values. There is a moderate correlation between the baseline cNIRS values and delta-cNIRS value after the transfusion.

Comparison of three different noninvasive ventilation strategies as initial respiratory support in very low birth weight infants with respiratory distress syndrome: A retrospective study.

BACKGROUND: There is inadequate evidence regarding which noninvasive ventilation (NIV) is superior for initial respiratory support of preterm infants with respiratory distress syndrome. OBJECTIVES: To compare the failure of noninvasive ventilation (NIV) and neonatal outcomes between nasal continuous positive airway pressure (NCPAP), bi-level positive airway pressure (BiPAP), and nasal intermittent positive pressure ventilation (NIPPV) as the initial respiratory support with less invasive surfactant administration (LISA) in very low birth weight (VLBW) infants. METHODS: Medical records of 419 VLBW infants born at 26-30weeks' gestation who did not require intubation in the delivery room and were initially supported with either NCPAP (n=221), BiPAP (n=101), or NIPPV (n=97) were retrospectively reviewed. The LISA approach was preferred in cases of surfactant requirement. The primary outcome was the failure of NIV within the first 72h of life. Failure of NIV was defined as the persistence or recurrence of one or more of the following: hypoxemia, respiratory acidosis, more than one episode of apnea requiring bag and mask ventilation or more than six episodes of apnea requiring stimulation over a 6-h period. Data were analyzed using univariate and multivariate logistic regression analysis. RESULTS: Failure of NIV within the first 72h of life was significantly higher in the NCPAP group (29.4%) compared with the BiPAP (12.9%) or NIPPV (12.4%) group (P<0.001). However, the BiPAP and NIPPV groups were not different in terms of NIV failure (P=0.91). Multivariable logistic regression analysis showed that antenatal steroid administration (OR: 0.49, 95% CI: 0.27-0.90; P=0.02) and gestational age˂28weeks (OR: 2.03, 95% CI: 1.18-3.49; P=0.01) were independent factors that influence failure of NIV within the first 72h of life. CONCLUSION: Compared with NCPAP, the use of NIPPV/BiPAP strategies for initial respiratory support can reduce the need for invasive ventilation in infants born at 26-30weeks' gestation.

Effect of kidney ischemia/reperfusion injury on proliferation, apoptosis, and cellular senescence in acute kidney injury in mice.

INTRODUCTION: Kidney ischemia/reperfusion injury (IRI) is the leading cause of acute kidney injury (AKI). Kidney IRI demonstrated apoptosis of epithelial cells in acute phase followed by proliferation of interstitial cells in chronic episode, and cellular senescence may contribute to development of AKI, however, its occurrence within acute or chronic episodes is still not completely understood. METHODS: Kidney IRI was performed with bilateral pediculus clamping in Swiss Background mice (3 months, 30-40g). Mice were euthanised on day one (I/R1, n=6), day eight (I/R8, n=6), and day twelve (I/R12, n=6) to exam acute and chronic episodes. Sham operation procedure was performed in the control. Tubular injury was assessed based on periodic acid- Schift (PAS) staining. Reverse transcriptase PCR (RT-PCR) was done to quantify mRNA expression of Bax, Bcl-2, and p16. Immunohistostaining (IHC) was performed to examine localisation of apoptosis (p53) and proliferation (Bcl-2). RESULTS: RT-PCR analysis showed upregulation of mRNA expression of Bcl-2, Bax, and p16 (p<0.05). The data showed that ischemia/reperfusion induces upregulation of Bax (p=0.20), Bcl-2 (p=0.45), p16 (p=0.18). Apoptosis and proliferation occurred in the epithelial cells in acute episodes, but occurred in interstitial areas in chronic episodes. CONCLUSIONS: Ischemia/reperfusion injury induces upregulation proliferation, apoptosis, and cellular senescence in acute kidney injury. Apoptosis reached its peak on day 1, proliferation on day 8, and cellular senescence on day 12.

Validity and reliability study of the Turkish version of SACRAH (Score for Assessment and quantification of Chronic Rheumatic Affections of the Hands).

Rheumatic diseases cause deformities in the hands and affect daily living activities. Therefore, assessment of hand disabilities is important in rheumatic disease. The aim of this study was to test the validity and reliability of the Turkish version of the A Score For Assessment and Quantification of Chronic Rheumatic Affections of the Hands (SACRAH). A translation and back-translation of the SACRAH were performed, according to the Beaton guidelines. Patients who were between 18-65 years old, who were literate in Turkish, who had rheumatic disease diagnosis and whose hands were affected, were included in the study. Patients who were using a splint during daytime were excluded from the study. They completed the Turkish version of Disabilities of the Arm, Shoulder and Hand Questionnaire (DASH-T) once and the final version of the SACRAH Questionnaire twice with a 7 days' interval. The internal consistency (Cronbach's α) and reliability (test-retest reliability) of the questionnaire were assessed. Besides, correlations between SACRAH and DASH-T scores were analyzed using the Spearman correlation coefficient. One hundred and twenty patients participated in the study. The Turkish version of the SACRAH met set criteria of reliability and validity. Internal consistency was excellent (Cronbach's α=0.88) and test-retest reliability were very good (r=0.73). SACRAH showed a positive and statistically significant correlation with DASH-T scores (r=0.83, p<0.001). Our results show that the Turkish version of the SACRAH has excellent test-retest reliability and validity. As a result of this study we determined that SACRAH is a valid and reliable instrument for assessing functional status and subjective manual function in Turkish-speaking patients.

Fibulin Levels in Autosomal Dominant Polycystic Kidney Disease and Its Relationship to Arterial Stiffness.

BACKGROUND: The objective of the present study was to evaluate fibulin 1 levels in different stages of patients with autosomal dominant polycystic kidney disease (ADPKD) and investigate possible connections between fibulin-1 and arterial stiffness. METHODS: For this cross-sectional study, we included 74 patients with ADPKD (mean age, 50.92 ± 15.70 years) and 32 healthy controls (mean age, 49.53 ± 7.32 years). Patients with ADPKD were classified based on CKD epidemiology collaboration (CKD-EPI) equation assessments of estimated glomerular filtration rate (eGFR). Blood levels of fibulin 1 and creatinine levels were analyzed. We measured brachial artery PWV (baPWV), augmentation index (AIx), and pulse pressure (PP) for the assessment of arterial stiffness and systolic and diastolic blood pressures (SBP and DBP, respectively). RESULTS: Fibulin 1 was significantly higher in the patient group (p < 0.001). SBP, DBP, MAP, PP, and baPWV levels were also significantly higher in the patient group. A statistically significant positive correlation was found between fibulin 1 and creatinine (r = 0.377, p = 0.001). No significant correlation was found between the fibulin 1 levels and age, SBP, DBP, MAP, baPWV, and AIx. CONCLUSIONS: Plasma concentrations of fibulin 1 increased in patients with ADPKD. Arterial stiffness measured by baPWV increased in patients with ADPKD, but it was not related to fibulin 1 levels.

Sulfatase-2: a prognostic biomarker and candidate therapeutic target in patients with pancreatic ductal adenocarcinoma.

BACKGROUND: Pancreatic ductal adenocarcinoma (PDAC) is the fifth most common cause of cancer death in the UK. Its poor prognosis is attributed to late detection and limited therapeutic options. Expression of SULF2, an endosulfatase that modulates heparan sulfate proteoglycan 6-O-sulfation and is reportedly tumourigenic in different types of cancer, was investigated. METHODS: SULF2 expression was determined immunohistochemically in archival surgical resection tissue sections from 93 patients with a confirmed histological diagnosis of PDAC between 2002 and 2008 followed for a median of 9 years. Relationships with clinico-pathological parameters and patient survival were explored. RESULTS: The majority of PDACs showed positive SULF2 staining in tumour cells and intratumoural or tumour-adjacent stroma. Greater than 25% SULF2-positive tumour cells was present in 60% of cancers and correlated with tumour stage (P=0.002) and perineural invasion (P=0.024). SULF2 intensity was scored moderate or strong in 81% of cancers and positively correlated with vascular invasion (P=0.015). High SULF2 expression, defined as >50% SULF2-positive tumour cells and strong SULF2 staining, was associated with shorter time to radiological progression (P=0.018, HR 1.98, CI 1.13-3.47). Similarly, by multivariate analysis, high SULF2 expression was independently associated with poorer survival (P=0.004, HR 2.10, CI 1.26-3.54), with a median survival of 11 months vs 21 months for lower PDAC SULF2. CONCLUSIONS: Elevated SULF2 in PDAC was associated with advanced tumour stage, vascular invasion, shorter interval to radiological progression and shorter overall survival. SULF2 may have roles as a prognostic biomarker and as a therapeutic target for patients with PDAC.

Histochemical and immunohistochemical studies of the gonads and paramesonephric ducts of male and female hatchlings of loggerhead sea turtles (Caretta caretta).

The sex of neonatal sea turtles is difficult to determine, because neonates lack heteromorphic sex chromosomes and dimorphic external characteristics; internal dimorphic morphology is defined at hatching. We used histochemical staining and made measurements in the gonads and paramesonephric ducts (PD) of both sexes to determine structural differences in female and male loggerhead sea turtle (Caretta caretta) hatchlings. We detected differences in the gonads and PD between the sexes including the amounts of mucopolysaccharides, collagen and elastic fibers. We determined that the thickness of the gonadal cortex and the diameter of the PD lumen are reliable sex-specific characteristics. We also assessed immunolocalization of aromatase, an enzyme complex that converts androgens to estrogens, and found differences in the localization and intensity of aromatase immunostaining in the gonads and PD of female and male hatchlings. Comprehensive studies of the sexual differences of sea turtles are important for conservation programs.

Soluble Klotho levels in diabetic nephropathy: relationship with arterial stiffness.

OBJECTIVE: In this cross-sectional study, we investigate the relationship between soluble Klotho (s-Klotho) levels, markers of bone mineral metabolism and arterial stiffness in 109 diabetic nephropathy patients (median age 61.00± 9.77 years) and 32 healthy controls (median age 49.23 ± 7.32 years). PATIENTS AND METHODS: Blood samples were collected to measure the levels of s-Klotho, and FGF23, serum creatinine, Calcium (Ca), Phosphorus (P), 25-hydroxyvitamin D3 (25hD) and parathyroid hormone (PTH). Pulse wave velocity (PWV) and blood pressure were also measured using a combined monitor. RESULTS: s-Klotho, FGF23 and PTH levels were significantly higher and 25hD was significantly lower in the patients than in controls (p < 0.001). Systolic blood pressure, pulse pressure and PWV were also significantly higher in the patients (p < 0.001). s-Klotho, FGF23 and 25hD levels significantly varied between sub-groups according to CKD stages, defined according to the CKD epidemiology collaboration equation. A strong positive correlation was found between s-Klotho and FGF23 (r = 0.768, p = 0.001) levels, but not with other bone mineral metabolism, blood pressure or arterial stiffness parameters. Creatinine levels significantly differed (p = 0.009) between three s-Klotho-level sub-groups, with the high creatinine levels in the sub-group with the lowest s-Klotho levels and estimated glomerular filtration rate (eGFR). CONCLUSIONS: There was no correlation between eGFR and s-Klotho levels. Arterial stiffness increased in CKD but was not related to s-Klotho or FGF23 levels. Among all parameters, FGF23 levels had the greatest effect on s-Klotho levels.

Simultaneous oroantral communication closure, sinus-lifting, and particulate bone grafting and immediate dental implant perforation.

The aim of this paper is to introduce an alternative technique for the treatment of oroantral communication (OAC). Closure of OAC, reconstruction of the posterior maxilla by sinus-lifting procedure with a particulate xenograft, and implant insertion were performed in the same operation. A lateral antral approach was used in the sinus elevations. The sinus membranes were elevated gently around the perforation area, and then a barrier membrane was used to close the perforation. Care was taken not to extend the perforation. Next, the maxillary sinus was filled with a particulate xenograft, and an implant was inserted simultaneously. Forty-seven and 40 months of clinical and radiographic follows-up revealed healthy and functional implants in the teeth area.

Design and synthesis of biphenyl and biphenyl ether inhibitors of sulfatases.

Inhibitors of sulfatase-2 are putative anticancer agents, but the discovery of potent small molecules targeting this enzyme has proved challenging. Based on molecular modelling, two series of sulfatase-2 inhibitors have been developed with biphenyl and biphenyl ether scaffolds judiciously substituted with sulfamate, carboxylate and other polar groups (e.g. amino). Inhibition of aryl sulfatase A and B was also determined. The biphenyl ether derivatives were less selective for sulfatase-2 over aryl sulfatase B than the biphenyl series. All biphenyl ether derivatives inhibited aryl sulfatase A, whereas only amino derivatives inhibited aryl sulfatase B significantly. In the biphenyl series few derivatives exhibited activity against aryl sulfatase B. The trichloroethylsulfamate group was identified as a new pharmacophore enabling potent inhibition of all of the sulfatases studied.

Increased oxidative stress in diabetic nephropathy and its relationship with soluble Klotho levels.

BACKGROUND: In the present study, we aimed to assess the relationship between the levels of soluble Klotho (s-Klotho) and oxidative stress markers in diabetic nephropathy patients with different stages of chronic kidney disease (CKD) and albuminuria levels. METHODS: We enrolled 109 patients with type 2 diabetes (mean age, 61.63 ± 9.77 years) and 32 healthy controls (mean age, 49.53 ± 7.32 years) between January and June 2014.  Patients were classified into three groups based on their urinary albumin/creatinine ratio (UACR). Blood samples were collected to measure the levels of s-Klotho, serum creatinine, calcium, phosphorus, 25-hydroxyvitamin D3, and parathyroid hormone (PTH). We used the total oxidant status (TOS), total antioxidant status (TAS), ischemia-modified albumin (IMA), and ischemia-modified albumin ratio (IMAR) values to measure the oxidative status. Moreover, the oxidative stress index (OSI) was estimated as the percentage ratio of TOS/TAS values. RESULTS: The TOS, TAS, and OSI values were significantly greater in the diabetic nephropathy patients compared to controls (p <0.001). When patients were classified based on their UACR, we noted that the TOS, OSI, and IMA values did not significantly differ, although the TAS (p <0.001), and IMAR (p =0.002) values significantly differed between the groups. The s-Klotho levels also significantly differed (p =0.031) between the groups. These s-Klotho levels exhibited a significant positive correlation with TOS (r =0.186, p =0.034) and OSI (r =0.207 p =0.018), but showed no correlation with the estimated glomerular filtration rate; UACR; HbA1c, calcium, phosphorus, and PTH levels; and TAS, IMA, and IMAR values. CONCLUSION: Oxidative stress is greater in patients with diabetic nephropathy, and the TOS was positively correlated with s-Klotho levels in diabetic patients. The therapeutic reduction of oxidative stress in patients with diabetic nephropathy could improve the renal and cardiovascular outcomes. Hippokratia 2016, 20(3): 198-203.

SERUM 25-HYDROXYVITAMIN D(3) LEVELS IN TYPE 2 DIABETIC PATIENTS WITH NORMO-, MICRO-, AND MACROALBUMINURIA.

BACKGROUND: The aim of this study was to observe the differences in serum 25-hydroxyvitamin D(3) levels in nondiabetic healthy control subject and type 2 diabetic patients, and to investigate the differences in serum 25-hydroxyvitamin D(3) levels in type 2 diabetic patients with normo-, micro- and macroalbuminuria. PATIENTS AND METHODS: Total 140 nondiabetic healthy controls and 384 type 2 diabetic patients (156 normoalbuminuric, 152 microalbuminuric and 76 macroalbuminuric) were included in the study. 25-hydroxyvitamin D(3) levels were measured in sera with the method of electrochemiluminescence using modular immunoassay analyzer. RESULTS: Vitamin D deficiency was detected in 70.85% and 22.9% of type 2 diabetic patients and nondiabetic healthy controls, respectively. Serum 25-hydroxyvitamin D(3) levels were significantly lower in type 2 diabetic patients compared to nondiabetic healthy controls (16.4 ± 9.5 ng/mL vs. 28.2 ± 11.6 ng/mL, p=0.0001). Serum 25-hydroxyvitamin D(3) levels were lower in albuminuric and nonalbuminuric diabetic patients (14.3 ± 7.9 ng/mL vs. 19.6±10.9 ng/mL, respectively, p=0.013). Serum 25-hydroxyvitamin D(3) levels were 19.6 ± 10.9 ng/mL in normoalbuminuric, 14.9 ± 8.8 ng/mL in microalbuminuric and 12.9 ± 5.8 ng/mL in macroalbuminuric diabetic patients. While lower serum 25-hydroxyvitamin D(3) levels were detected both in microalbuminuric (p=0.028) and macroalbuminuric diabetic patients (p=0.014) compared to normoalbuminuric diabetic patients, 25-hydroxyvitamin D(3) levels did not change significantly between microalbuminuric and macroalbuminuric diabetic patients (p=0.67). Serum 25-hydroxyvitamin D(3) levels correlated negatively with urinary albumin excretion (r=-0.24, p=0.016) in patients with diabetes mellitus. CONCLUSION: Findings of the present study demonstrated reduced serum 25-hydroxyvitamin D(3) levels which were significantly related with albuminuria in type 2 diabetic patients.

Interleukin-6 and C-reactive protein load in pre-storage and post-storage white blood cell-filtered red blood cell transfusions in premature infants.

OBJECTIVE: Leukocyte contamination during blood transfusion can cause many adverse effects. Filtration can be performed either at bedside during the transfusion or as pre-storage filtration. Pre-storage filtration is superior to bedside filtration because leukocytes are removed prior to storage, thus preventing further adverse effects associated with the storage of these cells. METHODS AND MATERIALS: One hundred and six infants were randomised into two groups: pre-storage filtration (group 1, n = 53) and bedside filtration (group 2, n = 53). C-reactive protein (CRP) and interleukin-6 (IL-6) levels were analysed within 24 h prior to the transfusion and 24 h after completion of the transfusion. RESULTS: In group 1, pre-transfusion median CRP and IL-6 levels were 2·95 (0·73-10·25) mg L(-1) and 8·59 (3·45-20·55) pg L(-1) , respectively, and post-transfusion median CRP and IL-6 levels were 2·28 (0·44-12·87) mg L(-1) and 6·62 (2·18-27·87) pg L(-1) , respectively. In group 2, pre-transfusion median CRP and IL-6 levels were 1·30 (0·40-7·84) mg L(-1) and 4·40 (2-17·12) pg L(-1) , respectively, and post-transfusion median CRP and IL-6 levels were 3·50 (0·50-7·85) mg L(-1) and 8·30 (3·48-23·75) pg L(-1) , respectively. There were no differences between pre-storage and post-storage leukoreduction average IL-6 and CRP levels in either group (P > 0·05 for both). Packed red blood cell (PRBC)-related necrotizing enterocolitis was detected in one infant in group 2. CONCLUSIONS: Because leukocytes in PRBC transfusions can be associated with many undesirable effects, leukoreduction is the best choice to prevent those effects. However, this method is still controversial. We demonstrated that using pre-storage and post-storage leukoreduction methods in erythrocyte transfusions did not change CRP or IL-6 levels, which are indicators of acute-phase response.

Regioselective sulfamoylation at low temperature enables concise syntheses of putative small molecule inhibitors of sulfatases.

Regioselective sulfamoylation of primary hydroxyl groups enabled a 5-step synthesis (overall yield 17%) of the first reported small molecule inhibitor of sulfatase-1 and 2, ((2S,3R,4R,5S,6R)-4,5-dihydroxy-2-methoxy-6-((sulfamoyloxy)methyl)tetrahydro-2H-pyran-3-yl)sulfamic acid, which obviated the use of hydroxyl protecting groups and is a marked improvement on the reported 9-step synthesis (overall yield 9%) employing hazardous trifluoromethylsulfonyl azide. The sulfamoylation methodology was used to prepare a range of derivatives of 1, and inhibition data was generated for Sulf-2, ARSA and ARSB.

Bardet-Biedl syndrome with vulva carcinoma presented with acute renal failure: a case report.

BACKGROUND: Bardet-Biedl syndrome is a rare disorder characterized by retinal dystrophy, obesity, kidney dysfunction, polydactyly, hypogonadism and cognitive impairment. It can be accompanied by systemic findings such as malignancy, hypertension, diabetes mellitus, constitutional and functional disorders of urogenital system and liver fibrosis. CASE REPORT: A 35-year-old woman with Bardet-Biedl syndrome was referred to our outpatient nephrology clinic with dysuria, acute renal failure, and urinary tract infection. A sized 2 x 1 cm mass between labia major and minor was noted, while CT scan showed a lesion that encompassed uterus and extended to the posterior side of the bladder in the left adnexal region and a 3 cm lesion in the liver. Excisional biopsy of the mass revealed a well-differentiated, squamous cell carcinoma. Dysuria resolved with insertion of urinary catheter after bougie dilatation and the patient was referred for radiotherapy. CONCLUSION: It should be kept in the mind that renal failure may develop due to constitutional urogenital anomalies such as vulva carcinoma. This can be an important cause of morbidity and mortality in patients with Bardet-Biedl syndrome.Hippokratia 2015; 19 (2):176-178.