Cardiovascular Involvement in Patients with Autosomal Dominant Polycystic Kidney Disease: A Review.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease with a prevalence of 1:400 to 1:1,000 in Caucasians. It is caused by mutations in the PKD1 gene located on chromosome 16p13.3 (in about 85% cases) as well as in the PKD2 gene on chromosome 4q13-23. In the Polish population, the disease is associated with PKD1 mutations in 84% of the ADPKD-affected families. PKD1 and PKD2 genes encode the proteins polycystin-1 (PC1) and polycystin-2 (PC2), respectively. The presence of kidney cysts is a characteristic feature in the ADPKD patients. But in the ADPKD patients, cardiovascular abnormalities, such as hypertension (HT) with higher systolic blood pressure (SBP) and diastolic blood pressure (DBP) values, higher left ventricular mass (LVM), intracranial (ICAN) and extracranial aneurysms, and cardiac valve defects, are significantly more common than in the general population. SUMMARY: According to the literature data, both higher LVM and vascular dysfunction already occur in children and young adults with normal renal function and without HT. Moreover, biventricular diastolic dysfunction, endothelial dysfunction, increased carotid intima-media thickness, and impaired coronary flow velocity reserve are present even in young patients with ADPKD who have normal HT and well-preserved renal function. In patients with ADPKD, hypertension has some specific features; in the youngest age group of children, the prevalence of hypertension is greater if their parents suffer from hypertension; in normotensive young ADPKD-diagnosed individuals, ambulant SBP and DBP values were significantly higher than in age- and gender-matched controls; hypertension appears at least 10 years earlier than spontaneous HT in general population. In adults, HT is often diagnosed before any substantial reduction in the GFR, and a lower nocturnal dip in BP in comparison to hypertensives in the general population. PKD1 and PKD2 gene products (PC1 and PC2 proteins) have been shown to assemble at the plasma membrane and to regulate calcium (Ca2+) entry. A defect in Ca2+ binding mediated by mutations in polycystin proteins is a hypothetical factor contributing to left ventricular mass increase. Altered intracellular Ca2+ handling contributes importantly to impaired contractility associated with heart failure. Impairment of intracellular Ca2+ homeostasis and mitochondrial function has been implicated in the development of LVH. KEY MESSAGES: It can be assumed that the cause of LVH in ADPKD patients is the natural course of this disease with developing HT and deteriorating kidney function, which may be influenced by the presence of PKD1- and PKD2-mutated gene products: PC1 and PC2 proteins.

Biological Activity of Pulcherrimin from the Meschnikowia pulcherrima Clade.

Pulcherrimin is a secondary metabolite of yeasts belonging to the Metschnikowia pulcherrima clade, and pulcherrimin formation is responsible for the antimicrobial action of its producers. Understanding the environmental function of this metabolite can provide insight into various microbial interactions and enables the efficient development of new effective bioproducts and methods. In this study, we evaluated the antimicrobial and antiadhesive action of yeast pulcherrimin, as well as its protective properties under selected stressful conditions. Classical microbiological plate methods, microscopy, and physico-chemical testing were used. The results show that pure pulcherrimin does not have antimicrobial properties, but its unique hydrophilic nature may hinder the adhesion of hydrophilic bacterial cells to abiotic surfaces. Pulcherrimin also proved to be a good cell protectant against UV-C radiation at both high and low temperatures.

New Antiadhesive Hydrophobic Polysiloxanes.

Intrinsic hydrophobicity is the reason for efficient bacterial settlement and biofilm growth on silicone materials. Those unwelcomed phenomena may play an important role in pathogen transmission. We have proposed an approach towards the development of new anti-biofilm strategies that resulted in novel antimicrobial hydrophobic silicones. Those functionalized polysiloxanes grafted with side 2-(carboxymethylthioethyl)-, 2-(n-propylamidomethylthioethyl)- and 2-(mercaptoethylamidomethylthioethyl)- groups showed a wide range of antimicrobial properties towards selected strains of bacteria (reference strains Staphylococcus aureus, Escherichia coli and water-borne isolates Agrobacterium tumefaciens, Aeromonas hydrophila), fungi (Aureobasidium pullulans) and algae (Chlorella vulgaris), which makes them valuable antibacterial and antibiofilm agents. Tested microorganisms showed various levels of biofilm formation, but particularly effective antibiofilm activity was demonstrated for bacterial isolate A. hydrophila with high adhesion abilities. In the case of modified surfaces, the relative coefficient of adhesion for this strain was 18 times lower in comparison to the control glass sample.

Supramolecular Interactions in Hybrid Polylactide Blends-The Structures, Mechanisms and Properties.

The conformation of polylactide (PLA) chains can be adjusted by supramolecular interactions (the formation of hydrogen bonds or host-guest complexes) with appropriate organic molecules. The structures formed due to those intermolecular interactions may act as crystal nuclei in the PLA matrix ("soft templating"). In this review, the properties of several supramolecular nucleating systems based on synthetic organic nucleators (arylamides, hydrazides, and 1,3:2,4-dibenzylidene-d-sorbitol) are compared to those achieved with biobased nucleating agents (orotic acid, humic acids, fulvic acids, nanocellulose, and cyclodextrins) that can also improve the mechanical properties of PLA. The PLA nanocomposites containing both types of nucleating agents/additives are discussed and evaluated in the context of their biomedical applicability.

Antimicrobial and Antibiofilm N-acetyl-L-cysteine Grafted Siloxane Polymers with Potential for Use in Water Systems.

Antibiofilm strategies may be based on the prevention of initial bacterial adhesion, the inhibition of biofilm maturation or biofilm eradication. N-acetyl-L-cysteine (NAC), widely used in medical treatments, offers an interesting approach to biofilm destruction. However, many Eubacteria strains are able to enzymatically decompose the NAC molecule. This is the first report on the action of two hybrid materials, NAC-Si-1 and NAC-Si-2, against bacteria isolated from a water environment: Agrobacterium tumefaciens, Aeromonas hydrophila, Citrobacter freundii, Enterobacter soli, Janthinobacterium lividum and Stenotrophomonas maltophilia. The NAC was grafted onto functional siloxane polymers to reduce its availability to bacterial enzymes. The results confirm the bioactivity of NAC. However, the final effect of its action was environment- and strain-dependent. Moreover, all the tested bacterial strains showed the ability to degrade NAC by various metabolic routes. The NAC polymers were less effective bacterial inhibitors than NAC, but more effective at eradicating mature bacterial biofilms.

[Singleton and twin pregnancies of PKU patients - individual variability of phenylalanine tolerance: experience of a single treatment center (Preliminary report)].

Phenylketonuria (PKU) is the autosomal recessive deficiency of phenylalanine hydroxylase resulting in the accumulation of phenylalanine (Phe) in blood and in the brain. Phe restriction in a patient's diet is determined depending on the amount of Phe intake which allows for stable blood Phe levels within the therapeutic range of 120-360µmol/L. In clinical practice the empirical determination of Phe tolerance relies on frequent assessment of blood Phe concentrations in relation to Phe intake from food records. Untreated maternal PKU may lead to maternal PKU syndrome in offspring. The objective of the study was to compare Phe tolerance during the course of singleton and multiple pregnancies of PKU patients. Case subjects and methods: The cases reviewed included three sets of classical PKU-affected Polish women on a low-phenylalanine diet during the course of singleton and twin pregnancies and their PKU-unaffected newborns. All the patients were under regular supervision of a metabolic dietitian to stabilize blood Phe levels and determine Phe tolerance. Data on pregnancy weight gain, the gestational age when the diet initiated, the percent of Phe assessments < 120 µmol/L and > 360 µmol/L, as well as offspring birth measurements were analyzed. RESULTS: The total increase in Phe tolerance and its pattern during the course of singleton and twin pregnancies differed remarkably in each patient. Three PKU women (Q383X/R408W, EX3DEL/EX3DEL, R281L/R408W) increased their Phe tolerance in singleton and twin pregnancies by 579%/468%, 674%/261%, and 427%/236%, respectively. During the last 10 weeks of singleton and twin pregnancy Phe tolerance showed an increase by 62%/149%, 33%/64%, and 37%/40%, respectively. The analysis of predictors for Phe tolerance showed that an individual's weight gain and the fetal weight gain as estimated from liveborn birth-weight data had no predictive capacity. CONCLUSIONS: Individual Phe tolerance in singleton pregnancies of PKU patients does not predict tolerance in twin pregnancy. Further research on the growing population of multiple pregnancy PKU patients is necessary to provide evidence-based guidelines to optimize the treatment of PKU in females of childbearing age.

Phenylketonuria patients' and their parents' acceptance of the disease: multi-centre study.

PURPOSE: Phenylketonuria (PKU) still poses a therapeutic challenge for patients and medical professionals. The aim of the study was to assess both patients' and their parents' acceptance of the disease. METHODS: The study included 218 PKU patients and 178 parents of PKU children who were enrolled in the study on the basis of questionnaire data. RESULTS: Regarding attitude towards the disease, our study demonstrated that 63 (28.9 %) PKU patients did not accept the disease. Patients who found accepting the disease difficult, more frequently perceived themselves as inferior/different in comparison with their peers. In total, 36 % of patients did not want their friends to be aware of their condition, while only 18 % of parents believed that their children's peers should not know about their disease. In total, 42 % of parents wanted to talk to other parents of PKU children and only 13 % to a doctor. Only 20 % of patients saw the need to discuss their condition with a doctor. In total, 8 % of children, regardless of age, and 14 % of parents preferred to talk to a psychologist. CONCLUSION: Our data demonstrated that disease acceptance played an essential role in patients' social integration. The study also indicated the need to overcome communication barriers between patients and their healthy peers and for patients to find the courage to be open about the disease. The importance of support groups for PKU families and the significance of strict cooperation between patients and their families with PKU treatment teams were also revealed.

[High plasma folate in patients with phenylketonuria]. / Hiperfolacynemia u chorych na fenyloketonurie.

UNLABELLED: Phenylketonuria is an inborn error of metabolism treated with a closely monitored low phenylalanine diet. Protein substitutes used for treatment are supplemented with vitamins and micronutrients. AIM: The aim of this study was to investigate plasma folic acid concentrations in children with phenylketonuria. MATERIALS AND METHODS: Retrospective analysis of medical records of 73 patients with phenylketonuria and 28 with mild hyperphenylalaninemia (on normal diet) was carried out. Intake of folic acid was calculated on the basis of protein substitute intake. Folate concentrations were analyzed according to their intake, and concentration of homocysteine and phenylalanine. RESULTS: In 76.7% patients with phenylketonuria intake of folic acid exceeded recommended dietary allowance. Serum folic acid concentrations above upper reference level were detected in 75.3% patients with phenylketonuria and only in 25% patients with hyperphenylalaninemia (p<0.0001). Strong positive correlation between daily intake of folic acid (with protein substitute) and concentration plasma folic acid (corr=0.55, p<0.0001) has been observed. CONCLUSIONS: Low phenylalanine diet using protein substitutes currently available in Poland predisposes to high concentration of plasma folic acid. The security of folic acid hipersupplementation in patients with phenylketonuria requires further detailed research.

Supramolecular self-assembly of linear oligosilsesquioxanes on mica--AFM surface imaging and hydrophilicity studies.

Linear oligomeric [2-(carboxymethylthio)ethylsilsesquioxanes] (LPSQ-COOH) adsorb spontaneously on muscovite mica and form smooth, well-ordered lamellar structures at the liquid-solid interface. Side carboxylic groups, having donor-acceptor character with regard to hydrogen bonds, are engaged both in multipoint molecule-to-substrate interactions and intermolecular cross-linking. The unique arrangement of silsesquioxane macromolecules, with COOH groups situated at the interface with air, produces highly hydrophilic surfaces of good thermal and solvolytic stability. Supramolecular assemblies of LPSQ-COOH were studied using atomic force microscopy (AFM), angle-resolved X-ray photoelectron spectroscopy (ARXPS) and attenuated total reflectance (ATR) FTIR spectroscopy. Comparative height profile analysis combined with ATR-FTIR studies of the spectral regions characteristic of carboxylic groups and C1s core level envelope by XPS confirmed specific interactions between LPSQ-COOH and mica.

Association of kidney and cysts dimensions with anthropometric and biochemical parameters in patients with ADPKD.

The aim of the study was to evaluate an association between kidney and cyst dimensions and anthropometric, clinical and biochemical parameters of autosomal dominant polycystic kidney disease (ADPKD) patients. Forty-nine adults, ADPKD-diagnosed patients aged 36 ± 11 years, and 50 healthy controls were included in the study. Oral glucose tolerance test (OGTT with 75 g of glucose) was performed and venous blood was collected to measure biochemical parameters and various ion concentrations. Ultrasound abdominal examinations were performed with special emphasis on kidney and cysts parameters. In the ADPKD group, mean kidney length correlated positively with age, systolic blood pressure (SBP), diastolic blood pressure (DBP), fasting glucose and glucose and C-peptide concentrations after 120 min of glucose intake and negatively with Mg(2+) concentration and glomerular filtration rate (eGFR). Multivariate analyses adjusted for age and gender showed that higher mean kidney length and maximal cyst diameter were significant predictors of higher SBP (p = 0.034 and 0.046, respectively) and DBP (p = 0.024 and 0.034, respectively), higher maximal cyst diameter was a significant predictor of higher OGTT 2-h C-peptide concentration (p = 0.033), higher mean cyst diameter was a significant predictor of lower eGFR (p = 0.039) and higher mean kidney length was a significant predictor of lower serum magnesium concentration (p = 0.043). In the ADPKD patients with normal GFR, mean kidney length and mean cyst diameter measured by ultrasonography are associated negatively with GFR and positively with blood pressure. Higher mean kidney length and cyst diameter might be indicators of disorders of glucose and magnesium metabolism which precede renal failure in patients with ADPKD.

Hybrid Bio-Based Silicone Coatings with Anti-adhesive Properties.

Hybrid polysiloxanes and polysilsesquioxanes grafted with naturally occurring bioactive phytochemicals: eugenol and linalool, were synthesized and investigated with regard to their structure and properties. The two series of materials, differing in the type of inorganic structure and the content of active groups, were coated onto the surface of glass plates, and their antibiofilm activities against bacteria Aeromonas hydrophila were assessed by luminometry and fluorescence microscopy. Bioactivity was correlated with specific properties of the hybrid coatings (chemical structure, surface free energy and adhesiveness). The functionalized polysilsesquioxanes exhibited the most favorable anti-adhesive effects. Cell adhesion after 6 days of incubation, expressed as RLU/cm2, was significantly reduced (44 and 67 for, respectively, Z-E-100 and Z-L-100, compared to 517 for the control glass carrier). The surface stickiness of polysiloxane films deteriorated their anti-adhesion properties, despite the presence of a large amount of bioactive species.

Autosomal dominant polycystic kidney disease and hypertension are associated with left ventricular mass in a gender-dependent manner.

BACKGROUND: The aim of this study was to compare echocardiographic parameters in patients with autosomal dominant polycystic kidney disease (ADPKD) and in controls with normal kidney function taking into account gender and the presence of hypertension. METHODS: 47 patients with ADPKD (age 36.3 ± 11.0 years) and 49 healthy controls (36.8 ± 9.2 years) were enrolled. M-mode echocardiography was performed in all subjects. Left ventricular hypertrophy (LVH) was diagnosed when the left ventricular mass index (LVMI) was greater than or equal to 125 g/m2 in males and 110 g/m2 in females. RESULTS: The prevalence of LVH was greater in ADPKD patients than in controls (13% vs. 2%; p=0.05). Among females, ADPKD patients demonstrated greater LVMI (87.9 ± 18.5 vs. 68.8 ± 15 g/m2, p=0.00009) than controls. There was a positive correlation between LVMI and blood pressure in ADPKD females (Rs=0.54, p=0.027 for systole blood pressure-SBP and Rs=0.50, p=0.0053 for diastole blood pressure-DBP) but not in males. CONCLUSION: Left ventricular mass is increased in ADPKD females with normal renal function. A positive correlation between SBP and DBP and LVMI was found in ADPKD females but not in ADPKD males.

Oxidative stress modulates the organization of erythrocyte membrane cytoskeleton.

BACKGROUND: Apart from their main role in transporting oxygen and carbon dioxide, erythrocytes play also an important role in organism antioxidative defence. Direct exposure to reactive oxygen species (ROS) results in shortening of their half-life, even by 50%. The presence of glucose, being the substrate in pentose phosphate pathway (PPP) cycle, is one of the factors that can have influence on the level of oxidative stress. The activity of PPP increases during oxidative stress. Glucose guarantees normal PPP functioning with the production of reductive equivalents in the amounts necessary to reproduction of glutathione--nonenzymatic free radical scavenger. In available literature there are no reports regarding the changes in protein contents of erythrocyte cytoskeleton exposed to t-butyl hydroperoxide in relation to glucose presence in incubation medium. MATERIAL/METHODS: Erythrocytes taken from 10 healthy subjects were used to assess the influence of generated free radicals on erythrocyte proteins and chosen parameters of oxidative stress. Erythrocytes were incubated in the solutions containing deferent concentrations of t-butyl hydroperoxide and glucose. Electrophoresis was performed on polyacrylamide gel in denaturating conditions. The contents of tryptophan in membranes was evaluated spectrofluorometrically. RESULTS/CONCLUSIONS: In vitro conditions oxidative stress leads to protein damage in erythrocyte cytoskeleton, both in proteins inside the cell as well as having contact with extracellular environment. In consequence, the amount of low-molecular proteins--mainly globin, which bind to cytoskeleton, increases. This process takes place independently of glucose presence in incubation medium. One of the element of protein cytoskeleton, tryptophan, also undergoes degradation. The decrease of its contents is higher during erythrocyte exposure to t-BOOH in environment containing glucose, what can suggest prooxidative influence of glucose in conditions in vitro.

Self-Healing Silsesquioxane-Based Materials.

This review is devoted to self-healing materials (SHM) containing polyhedral oligomeric silsesquioxanes (POSS) as building blocks. The synthetic approach can vary depending on the role POSS are expected to play in a given system. POSS (especially double-decker silsesquioxanes) can be grafted in side chains of a polymer backbone or used as segments of the main chain. Appropriate functionalization allows the formation of dynamic bonds with POSS molecules and makes them an active component of SHM, both as crosslinking agents and as factors that enhance the dynamics of macromolecules in the polymer matrix. The latter effect can be achieved by reversible release of bulky POSS cages or by the formation of separated inclusions in the polymer matrix through hydrophobic interactions and POSS aggregation. The unique properties of POSS-based self-healing systems make them interesting and versatile materials for various applications (e.g., repairable coatings, sealants, sensors, soft materials for tissue engineering, drug delivery, and wound healing).

Safety of extended treatment with sapropterin dihydrochloride in patients with phenylketonuria: results of a phase 3b study.

BACKGROUND: Phenylketonuria (PKU) results from impaired breakdown of phenylalanine (Phe) due to deficient phenylalanine hydroxylase (PAH) activity. Sapropterin dihydrochloride (sapropterin, Kuvan®) is the only US- and EU-approved pharmaceutical version of naturally occurring 6R-BH(4), the cofactor required for PAH activity. Sapropterin enhances residual PAH activity in sapropterin-responsive PKU patients and, in conjunction with dietary management, helps reduce blood Phe concentrations for optimal control. Approval was based on the positive safety and efficacy results of four international clinical studies, the longest of which was 22 weeks in duration. OBJECTIVE: To evaluate the safety of long-term treatment with sapropterin in PKU subjects who participated in previous Phase 3 sapropterin trials. METHODS: PKU-008 was designed as a Phase 3b, multicenter, multinational, open-label, 3-year extension trial to evaluate the long-term safety of sapropterin in patients with PKU who were classified as sapropterin responders and participated in prior Phase 3 sapropterin studies: 111 subjects aged 4-50 years completed prior studies and were subsequently enrolled in study PKU-008. Routine safety monitoring was performed at 3-month intervals and included adverse event reporting, blood Phe monitoring, clinical laboratory evaluations, physical examinations and vital sign measurements. RESULTS: Average exposure during PKU-008 was 658.7±221.3 days (range, 56-953; median, 595). The average total duration of participation in multiple studies (PKU-001, PKU-003, PKU-004, and PKU-008; or PKU-006 and PKU-008) was 799.0±237.5 days (range, 135-1151). The mean sapropterin dose was 16.2±4.7 mg/kg/day. Most adverse events were considered unrelated to treatment, were mild or moderate in severity, and were consistent with prior studies of sapropterin. No age-specific differences were observed in adverse event reporting. Three subjects discontinued treatment due to adverse events that were considered possibly or probably related to study treatment (one each of difficulty concentrating, decreased platelet count, and intermittent diarrhea). No deaths were reported. Of seven reported serious adverse events, one was considered possibly related to study treatment (gastroesophageal reflux). There were no laboratory or physical examination abnormalities requiring medical interventions. For most subjects, blood Phe concentrations were consistently within target range, confirming the durability of response in subjects undergoing extended treatment with sapropterin. CONCLUSION: Sapropterin treatment was found to be safe and well tolerated at doses of 5 to 20mg/kg/day for an average exposure of 659 days. This study supports the safety and tolerability of sapropterin as long-term treatment for patients with PKU.

Phase Structure and Properties of Ternary Polylactide/Poly(methyl methacrylate)/Polysilsesquioxane Blends.

Ternary blends of polylactide (PLA, 90 wt.%) and poly(methyl methacrylate) (PMMA, 10 wt.%) with functionalized polysilsesquioxanes (LPSQ-R) were obtained by solution blending. R groups in LPSQ containing hydroxyethyl (LPSQ-OH), methylglycolic (LPSQ-COOMe) and pentafluorophenyl (LPSQ-F5) moieties of different chemical properties were designed to modify PLA blends with PMMA. The effect of the type of LPSQ-R and their content, 1-3 wt.%, on the structure of the blends was studied with scanning electron microscopy (SEM) combined with energy dispersive spectroscopy (SEM-EDS), dynamic mechanical thermal analysis (DMTA) and Raman spectroscopy. Differential scanning calorimetry (DSC) and tensile tests also showed various effects of LPSQ-R on the thermal and mechanical properties of the blends. Depth-sensing indentation was used to resolve spatially the micro- and nano-scale mechanical properties (hardness and elastic behaviour) of the blends. The results showed clearly that LPSQ-R modulate the structure and properties of the blends.

Glucose metabolism parameters during an oral glucose tolerance test in patients with autosomal dominant polycystic kidney disease.

OBJECTIVE: The aim of the study was to estimate the pancreatic beta cell function and insulin resistance indexes in a group of Autosomal Dominant Polycystic Kidney Disease (ADPKD) patients with normal kidney function and no previous diabetes mellitus diagnosis. METHODS: A total of 49 adult patients with ADPKD aged 36 ± 11 years, and 50 healthy controls, all of Caucasian origin, were included in the study. Blood glucose, insulin and C-peptide concentrations were measured during an oral glucose tolerance test (OGTT with 75 g glucose) performed according to WHO recommendations in all subjects. RESULTS: The insulin/glucose ratio at the 30th and 120th minute of the OGTT and the insulinogenic index [(insulin at 30 min - insulin at 0 min)/glucose at 30 min] were significantly lower (p = 0.018, p = 0.031 and p = 0.013, respectively) in the ADPKD group. Four other indexes of beta cell function were lower with the borderline statistical significance (p = 0.054-0.076) than in controls. None of the calculated insulin sensitivity indexes differed between the study and control groups. CONCLUSIONS: Presence of ADPKD in patients with normal kidney function is associated with impaired beta cell function after an oral glucose load, without a significant decrease in insulin sensitivity.

[Is autosomal dominant polycystic kidney disease associated with asymptomatic bacteriuria?]. / Czy u chorych z autosomalna dominujaca wielotorbielowatoscia nerek wystepuje bakteriomocz bezobjawowy?

UNLABELLED: Urinary tract infections (UTIs) are a common complication in patients with autosomal dominant polycystic kidney disease (ADPKD). Asymptomatic bacteriuria (AB) is a factor predisposing to UTI in other populations. The aim of the study was to determine whether AB is more frequent in a group of ADPKD patients with normal kidney function and no diabetes than in a healthy control group. MATERIAL AND METHODS: A group of 49 ADPKD patients (19 men and 30 women) with normal kidney function (creatinine level < 1.35 mg/dl) and no diabetes, with an average age of 35.9 +/- 11.1 years was compared with a group of 50 healthy controls (22 men, 28 women) with similar age (36.7 +/- 9.2 years). All subject were evaluated using medical history and physical examination, urine culture, urinalysis (biochemical and morphological), kidney ultrasonography, oral glucose tolerance test (with 75 g of glucose) and serum creatinine testing. RESULTS: UTIs were significantly more frequent in medical history of ADPKD patients than in control group (26% vs. 0%, p < 0.001). Asymptomatic bacteriuria was found in 2% of ADPKD patients and in 4% of control group (p = 0.69). The presence of protein in urinalysis was observed in 6% of ADPKD and in none of the control patients. Other urine measurements like pH, specific gravity, and morphological examination were normal and did not substantially differ between groups. The kidneys of ADPKD patients were significantly longer than in control group (p < 0.001). CONCLUSIONS: Asymptomatic bacteriuria is not present more frequently in ADPKD patients with normal kidney function and no diabetes, than in healthy people.

Hybrid Fluorescent Poly(silsesquioxanes) with Amide- and Triazole-Containing Side Groups for Light Harvesting and Cation Sensing.

Hybrid polymers containing pyrene (Py) units bound to linear poly(silsesquioxane) (LPSQ) chains through flexible linkers containing heteroatoms (S, N, O) (LPSQ-triazole-Py and LPSQ-amide-Py) exhibit intense fluorescence emission, both in very diluted solutions (c = 10-8 mol/L) and in the solid state. The materials are thermally stable and exhibit good thin film forming abilities. Their optical and physicochemical properties were found to be strongly dependent on the structure of the side chains. Comparative studies with octahedral silsesquioxane (POSS) analogues (POSS-triazole-Py and POSS-amide-Py) emphasized the role of the specific double-strand architecture of the LPSQ backbone and distribution of side Py groups for their photo-luminescent properties. The new hybrid materials were tested as fluorescence energy donors to red-emitting dyes (Nile Red and Coumarine 6). All the silsesquioxanes studied were found to be able to transfer FL emission energy to Coumarin 6, irrespectively of their spatial structure. However, due to the differences in the wavelength range of FL emission, only LPSQ-triazole-Py were able to act as energy donors to Nile Red. The Py-grafted LPSQ may be also applied for development of soluble and highly emissive chemosensors. Their fluorescent nature was explored for the detection of Cu(II), Fe(III), Co(II), Ag(I), Hg(II), Mg(II), Ca(II), Pb(II) and Zn(II). The morphology of the side chains and hydrogen-bonding interactions influenced the sensing capacity of all the studied materials.

Metabolic syndrome components in patients with autosomal-dominant polycystic kidney disease.

BACKGROUND: The aim of this work was to compare the prevalence of the metabolic syndrome (MS) and its components between a group of autosomal-dominant polycystic kidney disease (ADPKD) patients with normal kidney function and no prior diagnosis of diabetes and healthy controls. METHODS: Forty-nine patients with ADPKD (age 35.9 +/- 11.1 years) with serum creatinine <1.35 mg/dl and 50 healthy controls (36.7 +/- 9.2 years) were enrolled for the study. Physical examination, basic laboratory measurements and oral glucose tolerance test were performed in all subjects. RESULTS: In the group of ADPKD patients, when compared to controls, the following values were significantly higher: waist-to-hip ratio (0.84 +/- 0.09 vs. 0.81 +/- 0.10, p = 0.046), systolic blood pressure (133.9 +/- 19.8 vs. 122.2 +/- 14.9 mm Hg, p = 0.0023), diastolic blood pressure (92.9 +/- 12.4 vs. 82.9 +/- 9.3 mm Hg, p < 0.0001), fasting glycemia (91.6 +/- 10.5 vs. 84.5 +/- 9.2 mg/dl, p = 0.04), and HbA(1C) (5.41 +/- 0.35 vs. 5.28 +/- 0.33%, p = 0.06, borderline significance). Adult Treatment Panel III criteria of MS were fulfilled by 14% of patients and 14% of controls, while International Diabetes Federation criteria were fulfilled by 22% of patients and 20% of controls, without any significant difference between the groups. CONCLUSION: The presence of ADPKD with normal kidney function is associated with components of MS such as hypertension, abdominal obesity and higher fasting glycemia.