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Background: Little is known about the long-term social and professional outcomes in adults after pediatric kidney replacement therapy (KRT). In this study, we described social and professional outcomes of adults after kidney failure during childhood and compared these outcomes with the general population. Methods: We sent a questionnaire to 143 individuals registered in the Swiss Pediatric Renal Registry (SPRR) with KRT starting before the age of 18 years. In the questionnaire, we assessed social (partner relationship, living situation, having children) and professional (education, employment) outcomes. Logistic regression models adjusted for age at study and sex were used to compare outcomes with a representative sample of the Swiss general population and to identify socio-demographic and clinical characteristics associated with adverse outcomes. Results: Our study included 80 patients (response rate 56%) with a mean age of 39 years (range 19–63). Compared to the general population, study participants were more likely to not have a partner (OR = 3.7, 95%CI 2.3–5.9), live alone (OR = 2.5, 95%CI 1.5–4.1), not have children (OR = 6.8, 95%CI 3.3–14.0), and be unemployed (OR = 3.9, 95%CI 1.8–8.6). No differences were found for educational achievement (p = 0.876). Participants on dialysis at time of study were more often unemployed compared to transplanted participants (OR = 5.0, 95%CI 1.2–21.4) and participants with > 1 kidney transplantation more often had a lower education (OR = 3.2, 95%CI 1.0–10.2). Conclusions: Adults after pediatric kidney failure are at risk to experience adverse social and professional outcomes. Increased awareness among healthcare professionals and additional psycho-social support could contribute to mitigate those risks. [ABSTRACT FROM AUTHOR]

Breast cancer risk continues to increase post menopause. Anti-estrogen therapies are available to prevent postmenopausal breast cancer in high-risk women. However, their adverse effects have reduced acceptability and overall success in cancer prevention. Natural products such as hops (Humulus lupulus) and three pharmacopeial licorice (Glycyrrhiza) species have demonstrated estrogenic and chemopreventive properties, but little is known regarding their effects on aromatase expression and activity as well as pro-proliferation pathways in human breast tissue. We show that Gycyrrhiza inflata (GI) has the highest aromatase inhibition potency among these plant extracts. Moreover, phytoestrogens such as liquiritigenin which is common in all licorice species have potent aromatase inhibitory activity, which is further supported by computational docking of their structures in the binding pocket of aromatase. In addition, GI extract and liquiritigenin suppress aromatase expression in the breast tissue of high-risk postmenopausal women. Although liquiritigenin has estrogenic effects in vitro, with preferential activity through estrogen receptor (ER)-β, it reduces estradiol-induced uterine growth in vivo. It downregulates RNA translation, protein biosynthesis, and metabolism in high-risk women's breast tissue. Finally, it reduces the rate of MCF-7 cell proliferation, with repeated dosing. Collectively, these data suggest that liquiritigenin has breast cancer prevention potential for high-risk postmenopausal women. [ABSTRACT FROM AUTHOR]

Background: Little is known about health-related quality of life (HRQoL) in adults after kidney failure during childhood. In this study, we analyzed HRQoL of adults after pediatric kidney failure in Switzerland and investigated socio-demographic and clinical factors associated with HRQoL. Methods: In this cohort study, we sent questionnaires to 143 eligible patients registered in the Swiss Pediatric Renal Registry with continuous kidney replacement therapy starting before the age of 18 years. We assessed HRQoL using the Short-Form 36 version 1, compared HRQoL scores between our sample and the Swiss general population, and used linear regression models to examine socio-demographic and clinical factors associated with HRQoL. Results: We included 79 patients (response rate 55%) with a mean age of 38.6 years (range 19.4–63.1). Compared to the general population, HRQoL scores were lower for physical functioning (− 12.43, p < 0.001), role physical (− 13.85, p = 0.001), general health (− 14.42, p < 0.001), vitality (− 4.98, p = 0.035), and physical HRQoL (− 6.11, p < 0.001), but we found no difference in mental HRQoL (− 0.13, p = 0.932). The socio-demographic factors—lower education, unemployment, and not being in a relationship—were associated with lower HRQoL. The only clinical factor associated with HRQoL was the type of kidney disease. Patients with acquired kidney diseases had lower mental HRQoL than patients with congenital anomalies of the kidney and urinary tract (− 11.4, p = 0.007) or monogenetic hereditary diseases (− 9.5, p = 0.018). Conclusions: Adults after pediatric kidney failure in Switzerland have lower physical, but similar mental HRQoL compared to the general population. Subgroups may require special attention with regard to their HRQoL. [ABSTRACT FROM AUTHOR]

Colorimetric and electrochemical (bio)sensors are commonly employed in wearable platforms for sweat monitoring; nevertheless, they suffer from low stability of the sensitive element. In contrast, mass-(bio)sensors are commonly used for analyte detection at laboratory level only, due to their rigidity. To overcome these limitations, a flexible mass-(bio)sensor for sweat pH sensing is proposed. The device exploits the flexibility of piezoelectric AlN membranes fabricated on a polyimide substrate combined to the sensitive properties of a pH responsive hydrogel based on PEG-DA/CEA molecules. A resonant frequency shift is recorded due to the hydrogel swelling/shrinking at several pH. Our device shows a responsivity of about 12 kHz/pH unit when measured in artificial sweat formulation in the pH range 3–8. To the best of our knowledge, this is the first time that hydrogel mass variations are sensed by a flexible resonator, fostering the development of a new class of compliant and wearable devices. [ABSTRACT FROM AUTHOR]

Background: Infants with a body weight of less than 10 kg are often not considered to be suitable candidates for renal transplantation (RTx). The objective of this study was to evaluate this arbitrary weight threshold for pediatric RTx.Methods: We conducted a multicenter, retrospective, match-controlled cohort study on infants weighing less than 10 kg at time of engrafting (low-weight group [LWG], n = 38) compared to a matched control group (n = 76) with a body weight of 10-15 kg, using data from the first 2 years post-transplant derived from the CERTAIN Registry.Results: Patient survival was 97 and 100% in the LWG and control groups, respectively (P = 0.33), and death-censored graft survival was 100 and 95% in the LWG and control groups, respectively (P = 0.30). Estimated glomerular filtration rate at 2 years post-transplant was excellent and comparable between the groups (LWG 77.6 ± 34.9 mL/min/1.73 m2; control 74.8 ± 29.1 mL/min/1.73 m2; P = 0.68). The overall incidences of surgery-related complications (LWG 11%, control 23%; P = 0.12) and medical outcome measures (LWG 23%, control 36%, P = 0.17) were not significantly different between the groups. The medical outcome measures included transplant-related viral diseases (LWG 10%, control 21%; P = 0.20), acute rejection episodes (LWG 14%, control 29%; P = 0.092), malignancies (LWG 3%, control 0%; P = 0.33) and arterial hypertension (LWG 73%, control 67%; P = 0.57).Conclusions: These data suggest that RTx in low-weight children is a feasible option, at least in selected centers with appropriate surgical and medical expertise. [ABSTRACT FROM AUTHOR]

The objective of the study was the development of an abridged risk-stratified imaging algorithm for the management of children with unilateral ureteropelvic junction obstruction (UPJO). Data on timing, frequency and duration of diagnostic imaging in children with unilateral UPJO was extracted retrospectively. Based on these findings, an abridged imaging algorithm was developed without changing the intended management by the clinicians and the outcome of the individual patient. The potential reduction of imaging studies was analysed and stratified by risk and management groups. The reduction in imaging studies, seen for ultrasound (US) and functional imaging (FI), was 45% each. On average, this is equivalent to 3 US and 1 FI studies less for every patient within the study period. The change was more pronounced in the low-risk groups. Progression of UPJO never occurred after 2 years of age and all secondary surgeries were carried out until the age of 3.Conclusions: Although our findings need to be validated by further prospective research, the developed imaging algorithm represents a risk-stratified approach towards less imaging studies in children with unilateral UPJO, and a follow-up beyond 3 years of age should be considered only in selected cases at the discretion of the clinician. What is Known: • ultrasound and functional imaging represent an integral part of therapeutic decision-making in children with unilateral ureteropelvic junction obstruction • imaging studies cannot accurately assess which patients are in need of surgical intervention, therefore close, serial imaging is preferred What is New: • a new, risk-stratified imaging algorithm was developed for the first 3 years of life • applying this algorithm could lead to a considerable reduction of imaging studies, and also the associated risks and health-care costs. [ABSTRACT FROM AUTHOR]

Unlabelled: Congenital anomalies of the kidney and urinary tract are common findings on fetal ultrasound. The aim of this prospective observational study was to describe outcome and risk factors in 115 patients born 1995-2001. All prenatally diagnosed children were stratified into low- and high-risk group and followed postnatally clinically and by imaging at defined endpoints. Risk factors were evaluated using odds ratios. Neonatal diagnosis included pelvi-ureteric junction obstruction (n = 33), vesicoureteral reflux (n = 27), solitary mild pelvic dilatation (postnatal anteroposterior diameter 5-10 mm; n = 25), and further diagnosis as primary obstructive megaureter, unilateral multicystic dysplastic kidney, renal dysplasia and posterior urethral valves. In 38 children with prenatal isolated hydronephrosis, ultrasound normalized at median age of 1.2 years (range 0.1-9). Surgery was performed in 34 children at median age of 0.4 years (0.1-10.8). Persistent renal anomalies without surgery were present in 43 children and followed in 36 for median time of 16 years (12.2-18). Oligohydramnios and postnatal bilateral anomalies were significantly associated with surgery and impaired renal function.Conclusion: The majority of children had a favourable postnatal outcome, in particular children with prenatally low risk, i.e. isolated uni- or bilateral hydronephrosis. Oligohydramnios and postnatal bilateral anomalies were risk factors for non-favourable outcome.What Is Known: • In congenital anomalies of the kidney and urinary tract significantly poorer outcome is known in patients with bilateral renal hypoplasia or solitary kidney associated with posterior urethral valves. • Other factors as proteinuria and vesicoureteral reflux were associated with a higher risk of progression to chronic renal failure in these patients. What is New: • Unlike other studies giving us above-mentioned information, we included all patients with any kind of prenatally diagnosed congenital anomalies of the kidney and urinary tract. Our study shows long-term follow up (median 16 years, range 12.2-18 years), especially in patients not needing surgery, but with persistent anomalies. • During postnatal long-term follow up (median 2.2 years, range 0.1-18 years) one third each showed normalization, need of surgery or persistence of anomalies without need of surgery. Our study revealed a good prognosis in the majority of these children, in particular with prenatally low risk, i.e. isolated uni- or bilateral hydronephrosis, and revealed oligohydramnios and postnatal bilateral anomalies as risk factors for a non-favourable outcome, defined as need of surgery, persistent anomalies with impaired renal function, end stage renal failure or death. [ABSTRACT FROM AUTHOR]

Unlabelled: The number of reports on baclofen intoxication has increased in recent years. We report a 15-year-old boy who was referred in a state of deep coma (Glasgow Coma Scale = 3). On clinical examination, he showed sinus bradycardia with normal blood pressure. On admission to the hospital, he presented intermittent short episodes of generalized tonic-clonic seizures. While results of imaging procedures and initial toxicological screening (including standard HPLC analysis and urine test) were negative, a nonconvulsive status epilepticus was diagnosed by electroencephalography (EEG). Identification of baclofen as causative agent was possible after the boy's father reported abusive baclofen intake. Subsequent toxicological target analysis of blood and urine samples confirmed the excessive intake of baclofen and showed a typical elimination pattern with a secondary release. Following 112 h of mechanical ventilation, the boy rapidly regained consciousness and recovered normal neurological behavior.Conclusions: The present case demonstrates the importance of considering baclofen overdosage in cases of severe coma in combination with an abnormal EEG pattern and sinus bradycardia with normal blood pressure levels, in particular as the substance is popular in internet reports promoting baclofen as a rather harmless "fun drug." Furthermore, it underlines the difficulty to identify baclofen as a causative agent without anamnestic information. Nevertheless, by reviewing existing literature on oral baclofen overdosage, it is possible to picture a nearly specific pattern of clinical symptoms in baclofen intoxication. [ABSTRACT FROM AUTHOR]

Cystinosis is a rare multisystemic progressive disorder mandating lifelong medical treatment. Knowledge on the intellectual and motor functioning, health-related quality of life and psychosocial adjustment in children with cystinosis is limited. We have investigated nine patients (four after renal transplantation) at a median age of 9.7 years (range 5.3–19.9 years). Intellectual performance (IP) was analysed with the Wechsler Intelligence Scale for Children-III (seven children) and the Kaufman Assessment Battery for Children (two children). Motor performance (MP) was evaluated using the Zurich Neuromotor Assessment Test, and quality of life (QOL) was studied by means of the Netherlands Organization for Applied Scientific Research Academical Medical Center Child Quality of Life Questionnaire. Psychosocial adjustment was assessed by the Child Behavior Checklist. The overall intelligence quotient (IQ) of our patient cohort (median 92, range 71–105) was significantly lower than that of the healthy controls ( p = 0.04), with two patients having an IQ < 85. Verbal IQ (93, range 76–118) was significantly higher than performance IQ (90, range 68–97; p = 0.03). The MP was significantly below the norm for pure motor, pegboard and static balance, as well as for movement quality. The patients’ QOL was normal for six of seven dimensions (exception being positive emotions), whereas parents reported significant impairment in positive emotions, autonomy, social and cognitive functions. Significant disturbance was noted in terms of psychosocial adjustment. Based on the results from our small patient cohort, we conclude that intellectual and motor performance, health-related QOL and psychosocial adjustment are significantly impaired in children and adolescents with cystinosis. [ABSTRACT FROM AUTHOR]

Knowledge of health-related quality of life (QOL) and psychosocial adjustment (PA) in children after renal transplantation (RTPL) is limited. QOL and PA were evaluated by standardized tests in patients after RTPL. Thirty-seven children of median age 14.5 years (range 6.5–17 years) were investigated a mean 4.5 years (range 0.5–12.8 years) after RTPL. Child- and parent-rated QOL was evaluated with the Child Quality of life Questionnaire of The Netherlands Organization for Applied Scientific Research Academical Medical Centre (TNO-AZL). PA was assessed by the Child Behaviour Checklist (CBCL) providing parental reports of a child’s behaviour. In patients’ self-ratings, the QOL dimension physical complaints ( P < 0.0005) scored significantly better than that of healthy controls, whereas the dimension positive emotional functioning was impaired ( P = 0.02). Parents rated motor functioning ( P = 0.002), autonomy ( P = 0.01), cognition ( P = 0.04) and positive emotions ( P < 0.0005) as significantly impaired. Parents also assessed PA significantly ( P = 0.02) impaired with regard to internalizing behaviour. Dialysis duration, young age at RTPL, living-related donation, steroid treatment, adverse family relationships and maternal distress had a significantly negative impact on QOL and PA ( P < 0.05). Patients rated QOL higher than did healthy controls. Parents evaluated their children’s QOL and PA more pessimistically than did the patients themselves. Both illness-related variables and family environment played an important role. [ABSTRACT FROM AUTHOR]

Comprehensive information on neurodevelopmental outcome in children and adolescents with chronic kidney disease is still limited. Intellectual performance (IP) and motor performance (MP) were systematically assessed in 27 patients at a median age of 14.1 years (range 6.5–17) and 6 years (range 0.5–12.7) after renal transplantation (RTPL). IP was analyzed with the Wechsler Intelligence Scale for Children-III (WISC-III) in 25 patients and by the Kaufman Assessment Battery for Children in two patients. MP was evaluated by the Zurich Neuromotor Assessment. Median full- scale intelligent quotient (FSIQ) was 97 (range 49–133). Twenty-one patients had an FSIQ ≥ 85 (i.e. ≥ mean–1 standard deviation). The five patients with neurological comorbidity had a median FSIQ of 81 (range 49–101). Verbal IQ (VIQ) (median 104; range 50–146) was significantly ( p < 0.01) higher than performance IQ (PIQ) (median 88; range 48–117). The PIQ was significantly lower compared with controls ( p < 0.007), and patients scored significantly lower compared with controls in five of 11 subtests of the Wechsler Scale. All MP tasks were significantly ( p < 0.01) lower than in controls, and also in children without neurological comorbidity. Socioeconomic status was significantly correlated with FSIQ ( p = 0.03). IP after RTPL was within the normal range for the majority of children. PIQ was lower compared with VIQ, and MP was significantly affected in all children after RTPL. [ABSTRACT FROM AUTHOR]

Steroid withdrawal (SW) after paediatric renal transplantation (RTPL) is controversial. Selective late SW has been performed in our unit since 1995. The safety and effects of SW were analysed retrospectively in 47 patients undergoing RTPL between 1995 and 2004. Initial immunosuppression consisted of cyclosporine A, azathioprine or mycophenolate mofetil and steroids. Criteria for SW were: (1) stable renal function, (2) time interval after RTPL ≥ 1 year, (3) no rejection or time interval after last rejection ≥ 1 year and (4) good compliance. SW was performed in 30 patients at an age of 13.5 years (range 4.5–18.5) and 2.2 years (range 1–6.6) after RTPL. After SW, one patient experienced a steroid-sensitive rejection. Follow-up after SW (1.3 year; range 0.25–7.5) showed maintained renal function: glomerular filtration rate at SW and currently was 82 (65–128) and 82 (42–115) ml/min per 1.73 m2, respectively. The number of patients on antihypertensive treatment did not significantly change (at SW: n = 15; currently: n = 11). Height and body mass index (BMI) remained stable: Median standard deviation score (SDS) for height/BMI at SW and currently was −1.1/0.2 and −0.8/0.1, respectively. Selective late SW was safe regarding renal function and had no significant effect on blood pressure and growth. [ABSTRACT FROM AUTHOR]

We have determined levels of glutathione (GSH), ATP, mitochondrial complex activity and apoptosis rate in proximal tubular cells (PTCs) exfoliated from urine in cystinotic ( n=9) and control ( n=9) children. Intracellular GSH was significantly depleted in cystinotic PTCs compared with controls (6.8 nmol GSH/mg protein vs 11.8 nmol GSH/mg protein; P<0.001), but there were no significant differences in mitochondrial complex activities or ATP levels under basal conditions. Cystinotic PTCs showed significantly increased apoptosis rate. After PTCs had been stressed by hypoxia, there was further depletion of GSH in cystinotic and control PTCs (2.4 nmol GSH/mg protein vs 7.2 nmol GSH/mg protein; P<0.001). Hypoxic stress led to increased complex I and complex IV activities in control but not in cystinotic PTCs. ATP levels were significantly reduced in cystinotic PTCs after hypoxic stress (12.2 nmol/mg protein vs 26.9 nmol/mg protein; P<0.001). GSH depletion occurs in this in vitro model of cystinotic PTCs, is exaggerated by hypoxic stress and may contribute to reduced ATP and failure to increase complex I/IV activities. Apoptotic rate is also increased, and these mechanisms may contribute to cellular dysfunction in cultured, human cystinotic PTCs. [ABSTRACT FROM AUTHOR]

En bloc renal transplantation (EBT) from infant donors is an option for children with end-stage renal failure. Owing to potential complications, EBT is not performed in all paediatric nephrology centres. We evaluated the perioperative and long-term course of five children undergoing EBT. Primary diagnosis was atypical (diarrhoea-negative) haemolytic uraemic syndrome ( n=2), interstitial nephropathy (two siblings) and branchio-oto-renal syndrome ( n=1). Recipient and donor ages ranged between 5.9 and 11.1 years and 0.3 and 2.5 years, respectively. Follow-up time after EBT was 2.1–13.2 years. Perioperative complications included (1) a renal artery thrombosis, with immediate intraoperative reconstruction and primary non-functioning of the graft, with recovery after 10 days, and (2) a vesico-ureteric obstruction, successfully managed with temporary insertion of a JJ-catheter. All grafts had good long-term function. Absolute glomerular filtration rate (GFR; millilitres/minute) increased in all patients, whereas relative GFR (millilitres/minute per 1.73 m2 body surface area) remained stable during the follow-up period in all but one. Kidney size increased significantly, with maximal growth during the first year after EBT; magnetic resonance imaging (MRI) showed normal structure and vasculature. EBT is a safe and effective option for young children with end-stage renal failure. Absolute GFR and graft size increase and adapt to the children’s growing body mass. [ABSTRACT FROM AUTHOR]

Despite the fact that Manus Province is the most geographically isolated province of Papua New Guinea, research shows that the acculturation process, common among minority indigenous groups around the globe, has had detrimental effects on maintaining knowledge and customs. Analysis of ethnobotanical knowledge among the indigenous population of Manus was undertaken in an attempt to compare, contrast, and draw conclusions regarding factors in maintaining indigenous knowledge of the local flora. Four levels of knowledge (plants recognized, identified, identified as useful, and identified as medicinally useful) were used to examine the relationships between knowledge of the local flora and physical, geographical, and social variables of informants. Results showed significant differences in ethnobotanical knowledge based on informant gender, age, cultural subclass, and habitation locality. Low levels of medicinal plant knowledge were found among the Manus due to acculturation, supporting the theory that medicinal plant knowledge is particularly vulnerable to loss. [ABSTRACT FROM AUTHOR]

The renal Fanconi syndrome (FS) is characterised by generalised proximal tubular dysfunction. Cystinosis is the most common genetic cause of the FS and results from defective function of cystinosin, due to mutations of theCTNSgene leading to intralysosomal cystine accumulation. Despite these advances in our understanding of the molecular basis of cystinosis, the mechanisms of proximal tubular cell (PTC) dysfunction are still unknown. We have further characterised an in vitro model of cultured cells exfoliated in patients’ urine. We cultured cells from 9 cystinosis children, 4 children with Lowe syndrome and 8 controls. PTC phenotype and homogeneity were studied by lectin staining, immunocytochemistry (using ZO-1 as an epithelial marker) and enzyme cytochemistry (using?-glutamyltransferase as a PTC marker). All cultured cells showed PTC phenotype. Cystinosin was stained using anti-cystinosin antibody and co-localised to the lysosomes with LAMP-2 antibody. Additionally, we have demonstrated significantly elevated intracellular cystine levels in cystinotic cell lines (13.8±2.3 nmol 1/2 cystine/mg protein,P<0.001) compared with controls. We believe this in vitro model will allow further investigation of cystinosis and other types of the FS. [ABSTRACT FROM AUTHOR]

Living-related kidney transplantation (LRKT) is an option for children with end-stage renal failure. In addition to medical concerns, there is uncertainty about the psychological impact of living-related donation on parent donors and families. A survey was conducted on the decision making process and medical and psychosocial consequences of LRKT. Between 1992 and 1999, 20 parents donated a kidney for their child. A questionnaire including 24 items was sent to parent donors and their partners. Nineteen parents and partners responded; the median time after LRKT was 3 years. Donors and partners reported an independent decision making process with no significant influence of partners, relatives, or hospital staff. Partners were more concerned about medical problems than donors themselves (P<0.02). Donors and partners cited no medical problems except sustained pain. Both reported an improved personal relationship towards the transplanted child. Donors and partners also cited an improved personal relationship. The vast majority (18/19) of couples still supported the decision for organ donation. In conclusion, there was a high degree of satisfaction with the decision making process in LRKT. The great majority of donors and partners did not report negative medical or psychological consequences. The relationship between donor, partner, and recipient child improved after LRKT. [ABSTRACT FROM AUTHOR]

Infections jeopardize children on immunosuppression after organ transplantation. Immunization is protective in healthy children. The aims of this study were to analyze the rate and efficacy of immunization in 62 children undergoing dialysis and renal transplantation (RTPL) between 1987 and 2000. The analysis was based on clinical findings, vaccination certificates, and measurement of specific serum antibodies. A member of the renal unit administered vaccinations. All 62 patients were immunized against diphtheria, tetanus, pertussis, poliomyelitis, measles, mumps, rubella, and hepatitis B. Since introduction in 1991 and 1995, 44 and 42 children were also vaccinated against influenza and Hemophilus influenzae type b, respectively. Of 16 patients with a negative history, 14 were given varicella vaccine; 16 children on peritoneal dialysis (PD) or with nephrotic syndrome were immunized against Streptococcus pneumoniae. All vaccinated patients had detectable serum antibodies against measles, mumps, rubella, varicella, hepatitis B, H. influenzae, and S. pneumoniae. There were 3 infections despite vaccination; 1 patient developed varicella after RTPL and 1 patient on PD had 2 episodes of peritonitis caused by H. influenzae and S. pneumoniae. In conclusion, monitoring and administration of the vaccines by the renal team enabled a high immunization rate. Whether vaccines, as documented by antibody titers, or by the low prevalence in the general population promoted the low prevalence of infections remains open, as there were at least a few vaccination failures. [ABSTRACT FROM AUTHOR]

Rats were exposed to ethyl benzene at 0, 300, 400 and 550 ppm for 8 hours/day for 5 consecutive days. Three to six weeks after the exposure, auditory function was tested by measuring compound action potentials (CAP) in the frequency range of 1-24 kHz and 2f1-f2 distortion product otoacoustic emissions (DPOAEs) in the frequency range of 4-22.6 kHz. In addition, outer hair cell (OHC) loss was quantified by histological examination. The lowest concentration ethyl benzene had no effect on any of the above measures. At 400 ppm, auditory thresholds were increased by 15 and 16 dB at 12 and 16 kHz, respectively, and at 550 ppm by 24, 31, and 22 dB at 8, 12, and 16 kHz, respectively. DPOAE amplitude growth with stimulus level was affected only after 550 ppm at 5.6, 8, and 11.3 kHz. OHC loss was found in two of the five examined locations in the cochlea. At 400 ppm, 25% OHC loss was found at the 11- and 21-kHz region. The highest concentration evoked 40% and 75% OHC loss at the 11- and 21-kHz location, respectively. Thus, the mid-frequency region of rats is affected after exposure to relatively low concentrations of ethyl benzene (400-550 ppm). These results indicate that ethyl benzene is one of the most potent ototoxic organic solvents known today. [ABSTRACT FROM AUTHOR]

Lactoferrin is a multifunctional immunoregulatory protein, stored in specific granules of neutrophil granulocytes, from which it is released following cell activation. As activated neutrophils play a crucial role in the destruction of synovial joints in rheumatoid arthritis, we evaluated lactoferrin concentration in synovial fluid and sera from 21 patients with rheumatoid arthritis and 11 patients with osteoarthritis. We also measured lactoferrin levels in sera from 12 healthy controls. Lactoferrin was measured by a solid-phase inhibition immunoassay. Median lactoferrin levels were significantly higher in synovial fluid from rheumatoid arthritis than from osteoarthritis patients ( P=0.0002). In contrast, no significant difference was found between serum lactoferrin from patients with rheumatoid arthritis or osteoarthritis compared with normal controls. In patients with rheumatoid arthritis, lactoferrin concentrations were higher in synovial fluid than in sera ( P=0.036). In both rheumatoid arthritis and osteoarthritis no correlation was found between serum and synovial fluid lactoferrin ( P=0.51 and P=0.5, respectively). In synovial fluid from patients with rheumatoid arthritis, lactoferrin concentrations correlated with neutrophil granulocyte count ( P<0.0001), but neither serum nor synovial lactoferrin levels correlated with disease activity ( P=0.32 and P=0.25, respectively). In conclusion, lactoferrin is a reliable marker of neutrophil activation at sites of inflammation in rheumatoid synovitis, but does not represent a marker of disease activity. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Assessment of body composition is of primary importance in the management of celiac adolescents. We aimed to evaluate body composition by dual-energy x-ray absorptiometry and bioelectrical impedance in celiac adolescents on a gluten-free diet to investigate whether impedance may provide an alternative method to assess nutritional status in these patients. METHODS: We studied body composition in 43 adolescents affected by celiac disease on a gluten-free diet for > 1 yr and 30 healthy subjects. Fat, fat-free, and bone masses were assessed by dual-energy x-ray absorptiometry. Fat and fat-free masses were also assessed by bioelectrical impedance. All anthropometric measurements were performed according to standard procedures. RESULTS: All patients had a significantly lower body weight, height, fat-free mass, bone mineral density (p < 0.001), and body mass index (p < 0.01) compared with controls. In contrast, parameters predicting fat compartment (sum of skinfolds and fat mass) did not differ from those of controls. No significant difference was found between patients strictly adherent to a gluten-free diet and patients partially compliant. Compared with dual-energy x-ray absorptiometry measurements, bioelectrical impedance showed a high accuracy to estimate fat-free mass (R² = 0.97) and limited accuracy for fat mass (R² = 0.75). Furthermore, impedance was more reliable for estimating hydration of soft tissue underlying the fat-free mass changes. CONCLUSIONS: In adolescents with celiac disease, after a mean of 1 yr of gluten-free diet all the parameters assessing body compartments, except fat mass, were affected, compared with healthy controls. Bioelectrical impedance holds promise tor routine assessment of body composition changes in celiac adolescents on a gluten-free diet. [ABSTRACT FROM AUTHOR]

Vascular grafts are artificial conduits properly designed to substitute a diseased blood vessel. However prosthetic fail can occur without premonitory symptoms. Continuous monitoring of the system can provide useful information not only to extend the graft's life but also to optimize the patient's therapy. In this respect, various techniques have been used, but all of them affect the mechanical properties of the artificial vessel. To overcome these drawbacks, an ultrathin and flexible smart patch based on piezoelectric Aluminum Nitride (AlN) integrated on the extraluminal surface of the prosthesis is presented. The sensor can be conformally wrapped around the external surface of the prosthesis. Its design, mechanical properties and dimensions are properly characterized and optimized in order to maximize performances and to avoid any interference with the graft structure during its activity. The sensorized graft is tested in vitro using a pulsatile recirculating flow system that mimics the physiological and pathological blood flow conditions. In this way, the ability of the device to measure real-time variations of the hemodynamics parameters has been tested. The obtained high sensitivity of 0.012 V Pa−1 m−2, joint to the inherent biocompatibility and non-toxicity of the used materials, demonstrates that the device can successfully monitor the prosthesis functioning under different conditions, opening new perspectives for real-time vascular graft surveillance. [ABSTRACT FROM AUTHOR]

3-Methylglutaconic aciduria is frequently found during urine organic acid analysis and is widely regarded as a marker of a mitochondrial disorder, the clinical features of which are very heterogeneous. We describe two siblings with 3-methylglutaconic aciduria in whom renal ultrasonography showed echogenic medullae consistent with nephrocalcinosis. One patient also developed medullary cysts. In both children renal function was normal and neither had any plasma or urinary evidence of tubulopathy. The presence of nephrocalcinosis and medullary cysts in patients with 3-methylglutaconic aciduria adds to the heterogeneous clinical presentation of this group of disorders. [ABSTRACT FROM AUTHOR]

Reports on the case of a young boy diagnosed with hyperammonaemic encephalopathy. Symptoms experienced by the patient; Result of the micturition cystogram; Types of concurrent pathogenetic mechanisms known to cause hyperammonaemia secondary to urinary tract infection.

Background: There is limited information about psychological distress in adults who underwent kidney replacement therapy (KRT) during childhood. This study aimed to describe psychological distress in adults after KRT during childhood in comparison to the Swiss general population and to evaluate associations with sociodemographic and clinical characteristics.We sent a questionnaire to 143 people from the Swiss Pediatric Renal Registry (SPRR), who were alive, over 18 years old, started KRT before the age of 18 years, and were German speakers. We measured psychological distress using the Brief Symptom Inventory 18 (BSI-18) and evaluated the Global Severity Index 18 (GSI-18), reflecting the overall level of distress, and the three subscales: depression, somatization, and anxiety. We compared levels of psychological distress to normal data from the Swiss general population and used regression models to identify associations with sociodemographic and clinical characteristics.Eighty persons with a mean age of 39 years (SD 10.1) responded to the questionnaire (response rate 56%). Overall, the GSI-18 and all subscales of the BSI-18 were similar. Unemployed participants (25%) reported higher levels of somatization and were more likely to experience psychological distress. Participants using psychotropic drugs (14%) reported higher levels of overall psychological distress (10%), depression (13%) and somatization (9%).Adults after KRT during childhood showed good long-term psychological well-being. These results are encouraging and underline the favorable outcome of these patients. So besides the excellent somatic outcome, these patients can achieve a psychological healthy life after diagnosis of chronic kidney disease.A higher resolution version of the Graphical abstract is available as Supplementary informationA higher resolution version of the Graphical abstract is available as Supplementary informationMethods: There is limited information about psychological distress in adults who underwent kidney replacement therapy (KRT) during childhood. This study aimed to describe psychological distress in adults after KRT during childhood in comparison to the Swiss general population and to evaluate associations with sociodemographic and clinical characteristics.We sent a questionnaire to 143 people from the Swiss Pediatric Renal Registry (SPRR), who were alive, over 18 years old, started KRT before the age of 18 years, and were German speakers. We measured psychological distress using the Brief Symptom Inventory 18 (BSI-18) and evaluated the Global Severity Index 18 (GSI-18), reflecting the overall level of distress, and the three subscales: depression, somatization, and anxiety. We compared levels of psychological distress to normal data from the Swiss general population and used regression models to identify associations with sociodemographic and clinical characteristics.Eighty persons with a mean age of 39 years (SD 10.1) responded to the questionnaire (response rate 56%). Overall, the GSI-18 and all subscales of the BSI-18 were similar. Unemployed participants (25%) reported higher levels of somatization and were more likely to experience psychological distress. Participants using psychotropic drugs (14%) reported higher levels of overall psychological distress (10%), depression (13%) and somatization (9%).Adults after KRT during childhood showed good long-term psychological well-being. These results are encouraging and underline the favorable outcome of these patients. So besides the excellent somatic outcome, these patients can achieve a psychological healthy life after diagnosis of chronic kidney disease.A higher resolution version of the Graphical abstract is available as Supplementary informationA higher resolution version of the Graphical abstract is available as Supplementary informationResults: There is limited information about psychological distress in adults who underwent kidney replacement therapy (KRT) during childhood. This study aimed to describe psychological distress in adults after KRT during childhood in comparison to the Swiss general population and to evaluate associations with sociodemographic and clinical characteristics.We sent a questionnaire to 143 people from the Swiss Pediatric Renal Registry (SPRR), who were alive, over 18 years old, started KRT before the age of 18 years, and were German speakers. We measured psychological distress using the Brief Symptom Inventory 18 (BSI-18) and evaluated the Global Severity Index 18 (GSI-18), reflecting the overall level of distress, and the three subscales: depression, somatization, and anxiety. We compared levels of psychological distress to normal data from the Swiss general population and used regression models to identify associations with sociodemographic and clinical characteristics.Eighty persons with a mean age of 39 years (SD 10.1) responded to the questionnaire (response rate 56%). Overall, the GSI-18 and all subscales of the BSI-18 were similar. Unemployed participants (25%) reported higher levels of somatization and were more likely to experience psychological distress. Participants using psychotropic drugs (14%) reported higher levels of overall psychological distress (10%), depression (13%) and somatization (9%).Adults after KRT during childhood showed good long-term psychological well-being. These results are encouraging and underline the favorable outcome of these patients. So besides the excellent somatic outcome, these patients can achieve a psychological healthy life after diagnosis of chronic kidney disease.A higher resolution version of the Graphical abstract is available as Supplementary informationA higher resolution version of the Graphical abstract is available as Supplementary informationConclusions: There is limited information about psychological distress in adults who underwent kidney replacement therapy (KRT) during childhood. This study aimed to describe psychological distress in adults after KRT during childhood in comparison to the Swiss general population and to evaluate associations with sociodemographic and clinical characteristics.We sent a questionnaire to 143 people from the Swiss Pediatric Renal Registry (SPRR), who were alive, over 18 years old, started KRT before the age of 18 years, and were German speakers. We measured psychological distress using the Brief Symptom Inventory 18 (BSI-18) and evaluated the Global Severity Index 18 (GSI-18), reflecting the overall level of distress, and the three subscales: depression, somatization, and anxiety. We compared levels of psychological distress to normal data from the Swiss general population and used regression models to identify associations with sociodemographic and clinical characteristics.Eighty persons with a mean age of 39 years (SD 10.1) responded to the questionnaire (response rate 56%). Overall, the GSI-18 and all subscales of the BSI-18 were similar. Unemployed participants (25%) reported higher levels of somatization and were more likely to experience psychological distress. Participants using psychotropic drugs (14%) reported higher levels of overall psychological distress (10%), depression (13%) and somatization (9%).Adults after KRT during childhood showed good long-term psychological well-being. These results are encouraging and underline the favorable outcome of these patients. So besides the excellent somatic outcome, these patients can achieve a psychological healthy life after diagnosis of chronic kidney disease.A higher resolution version of the Graphical abstract is available as Supplementary informationA higher resolution version of the Graphical abstract is available as Supplementary informationGraphical abstract: There is limited information about psychological distress in adults who underwent kidney replacement therapy (KRT) during childhood. This study aimed to describe psychological distress in adults after KRT during childhood in comparison to the Swiss general population and to evaluate associations with sociodemographic and clinical characteristics.We sent a questionnaire to 143 people from the Swiss Pediatric Renal Registry (SPRR), who were alive, over 18 years old, started KRT before the age of 18 years, and were German speakers. We measured psychological distress using the Brief Symptom Inventory 18 (BSI-18) and evaluated the Global Severity Index 18 (GSI-18), reflecting the overall level of distress, and the three subscales: depression, somatization, and anxiety. We compared levels of psychological distress to normal data from the Swiss general population and used regression models to identify associations with sociodemographic and clinical characteristics.Eighty persons with a mean age of 39 years (SD 10.1) responded to the questionnaire (response rate 56%). Overall, the GSI-18 and all subscales of the BSI-18 were similar. Unemployed participants (25%) reported higher levels of somatization and were more likely to experience psychological distress. Participants using psychotropic drugs (14%) reported higher levels of overall psychological distress (10%), depression (13%) and somatization (9%).Adults after KRT during childhood showed good long-term psychological well-being. These results are encouraging and underline the favorable outcome of these patients. So besides the excellent somatic outcome, these patients can achieve a psychological healthy life after diagnosis of chronic kidney disease.A higher resolution version of the Graphical abstract is available as Supplementary informationA higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]