73 research outputs found
Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)
No full textAbstract To test the association between bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD) and long-term clinical outcome and to identify risk factors for severe outcomes, a dataset comprising 504 patients from the international registry study ARegPKD was analyzed for characteristics and complications of patients with very early (≤ 3 months; VEBNE) and early (4–15 months; EBNE) bilateral nephrectomies. Patients with very early dialysis (VED, onset ≤ 3 months) without bilateral nephrectomies and patients with total kidney volumes (TKV) comparable to VEBNE infants served as additional control groups. We identified 19 children with VEBNE, 9 with EBNE, 12 with VED and 11 in the TKV control group. VEBNE patients suffered more frequently from severe neurological complications in comparison to all control patients. Very early bilateral nephrectomies and documentation of severe hypotensive episodes were independent risk factors for severe neurological complications. Bilateral nephrectomies within the first 3 months of life are associated with a risk of severe neurological complications later in life. Our data support a very cautious indication of very early bilateral nephrectomies in ARPKD, especially in patients with residual kidney function, and emphasize the importance of avoiding severe hypotensive episodes in this at-risk cohort.Universität zu Köln http://dx.doi.org/10.13039/501100008001Marga und Walter Boll-Stiftung http://dx.doi.org/10.13039/501100011566Bundesministerium für Bildung, Wissenschaft und Forschung http://dx.doi.org/10.13039/501100013699Bundesministerium für Bildung und Forschung http://dx.doi.org/10.13039/501100002347European Society for Paediatric NephrologyProjekt DEA
Rural implementation of Girls’ Programming Network (GPN)
No full textA small-scale pilot study was conducted in north-west Tasmania to investigate adolescent girls’ willingness to participate in informal STEM education, through exploring their perceptions and experiences of computer science (CS), and their future aspirations. This pilot study was funded by an Inspiring Australia Public Science Event Grant, enabling the delivery of two local Girls’ Programming Network (GPN) workshops by the two Sydney-based GPN co-founders. The aim of this case study was to determine the viability of establishing an ongoing and sustainable northwest Tasmanian GPN, which would provide rural adolescent girls with opportunities to explore programming, connect them with mentors and role models, and show them what potential career opportunities exist beyond family and local contexts. Qualitative methods of data collection comprised focus group interviews and artefact elicitation with the participants, and individual interviews with the program co-founders. The results indicate that single-sex informal CS education opportunities are valued by adolescent girls, and that typical gender stereotypes can be changed due to the experience of engaging in them. The success of the pilot study indicates that establishing the GPN in a rural area to provide adolescent girls with free and fun CS experiences is worthwhile and viable
Aetiology, course and treatment of acute tubulointerstitial nephritis in paediatric patients : A cross-sectional web-based survey
No full textBackground Acute tubulointerstitial nephritis (TIN) is a significant cause of acute renal failure in paediatric and adult patients. There are no large paediatric series focusing on the aetiology, treatment and courses of acute TIN. Patients, design and setting We collected retrospective clinical data from paediatric patients with acute biopsy-proven TIN by means of an online survey. Members of four professional societies were invited to participate. Results Thirty-nine physicians from 18 countries responded. 171 patients with acute TIN were included (54% female, median age 12 years). The most frequent causes were tubulointerstitial nephritis and uveitis syndrome in 31% and drug-induced TIN in 30% (the majority of these caused by non-steroidal anti-inflammatory drugs). In 28% of patients, no initiating noxae were identified (idiopathic TIN). Median estimated glomerular filtration rate (eGFR) rose significantly from 31 at time of renal biopsy to 86 mL/ min/1.73 m2 3-6 months later (p<0.001). After 3-6 months, eGFR normalised in 41% of patients (eGFR ≥90 mL/ min/1.73 m2), with only 3% having severe or end-stage impairment of renal function (<30 mL/min/1.73 m2). 80% of patients received corticosteroid therapy. Median eGFR after 3-6 months did not differ between steroid-treated and steroid-untreated patients. Other immunosuppressants were used in 18% (n=31) of patients, 21 of whom received mycophenolate mofetil. Conclusions Despite different aetiologies, acute paediatric TIN had a favourable outcome overall with 88% of patients showing no or mild impairment of eGFR after 3-6 months. Prospective randomised controlled trials are needed to evaluate the efficacy of glucocorticoid treatment in paediatric patients with acute TIN.Peer reviewe
Make at home or abroad? Manufacturing reshoring through a GPN lens: A Norwegian case study
No full textThe explorative paper investigates the drivers for the emerging trend of manufacturing reshoring from low- to high-cost locations. To date research on the reshoring phenomenon has been dominated by micro-level analyses of firms in supply chain management and reported in international business literature. The paper introduces reshoring as a research topic to the economic geography research field, arguing that it connects with the broader topic of regional development. To provide a better understanding of the reshoring phenomenon and to test the applicability of the global production network (GPN) framework in the analysis of the phenomenon, the authors analyse the reshoring of nine of Norwegian manufacturing firms. With the multiscalar lens provided by the GPN framework, the authors find that the implementation of advanced manufacturing technologies is a driver for manufacturing reshoring, but only when matched with key regional assets such as automation knowledge and competence, key human capital, and region-specific manufacturing competence. Additionally, reshoring decisions are influenced by extra-regional factors such as changes in the global economy and market fluctuations. Furthermore, the paper provides a refined conceptualization of strategic coupling processes by including acts of disinvestments and reinvestments performed by actors within global production networks. Accordingly, the authors advocate a more nuanced understanding, defined as partial coupling processes, in contrast to the predominant understanding of coupling processes as ruptures. This refined conceptualization provides enhanced analytical purchase when studying the reshoring phenomenon, as it illuminates the complexity of firms’ production and sourcing strategies and the resulting implications for the economic landscape.publishedVersion2020 The Author(s). Published by Elsevier Ltd. This is an open access article under the CC BY license (http://creativecommons.org/licenses/BY/4.0/)
An exploratory study on developing a geriatric pharmacy network for medication management via telehealth consultation / Nor Elyzatul Akma Hamdan
No full textBackground: The need for social distancing and disruption of healthcare systems appears to be opening a window of opportunity for greater exposure to the usage of technology in geriatric care. In response to maintain continuity of care and fill in the gap due to the impact of the COVID-19 pandemic, the geriatric pharmacy network (GPN) was developed. The GPN refers to a public-private partnership between Hospital Pharmacists with Academic Pharmacists and Community Pharmacists in Malaysia. Objective: The first phase of this study aims to explore the pharmacists' views on the impact of the COVID-19 pandemic on pharmacist-patient consultations and their views on the feasibility of conducting telehealth to maintain continuity of care in geriatric medication therapy adherence clinics (GMTAC). The second phase aims to elucidate the mechanism for developing a public-private partnership via GPN as an alternative strategy to expand and sustain GMTAC services through telehealth consultations. Methods: This study used a qualitative study design. The first phase of the study involved semi-structured interviews with pharmacists experienced in GMTAC. The second phase of the study involved focus group discussions with the key stakeholders involved in the GMTAC, such as the physicians, staff nurses, and patients. In addition, the key stakeholders involved in the GPN, such as community pharmacists and academic pharmacists, were also invited to this focus group discussion. Both interviews and focus group discussions were collected via an online platform. Data were transcribed verbatim and analysed using a framework analysis method. The NVivo 12 software was used to manage the data. Results: Semi-structured interviews and focus group discussions were conducted with 11 pharmacists and 21 stakeholders, respectively. Three themes emerged on the impact of the COVID-19 pandemic on pharmacist-patient consultation: (1) management of GMTAC services, (2) inefficient communication and (3) the pandemic's effect on emotion, behaviours, and psychology. As for the feasibility of conducting telehealth: (1) utility, (2) acceptance and (3) readiness are the three main themes that emerged. Finally, the focus group discussion resulted in a wide range of themes that help to elucidate the mechanism of developing GPN, categorized into short and long-term goals. Conclusion: The formation of GPN will offer tremendous benefits and has the potential to address the gap caused by the COVID-19 pandemic. The utilization of telehealth is feasible to be implemented to maintain continuity of care, but the challenges and barriers must be addressed. The development of GPN will serve as an alternative strategy to expand the GMTAC services, but the short and long-term goals must be achieved to ensure successful implementation of GPN
Chronic Effect of Gabapentin on Liver function in Adult Male Rats
No full textGabapentin (GPN) is a new antiepileptic agent currently in used as add-on therapy in adult patients suffering from partial seizures. The extent of liver damage at different dosage and long term treatment with GPN is not yet clear. Therefore this study was undertaken to find out the possibility of liver damage by this drug. Adult male (Wistar) rats of 180-220 g were administered intraperitoneally with GPN (20 or 100 mg/kg) for 45 days. After the experimental period, the liver function tests were carried out in control and experimental groups. The activity of liver enzymes, with 20 mg/kg of GPN were not significantly different from the control group but, the serum levels of aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, lactate dehydrogenase, direct bilirubin and total bilirubin were enhanced significantly with 100 mg/kg of GPN. Total protein and albumin decreased in this group as compared with control animals. The histopathology of the liver parenchymal cells also showed minute foci of necrosis in a few rats treated with high dose of GPN, whereas, at therapeutic dose the histopathology and biochemical indices showed almost normal values. At therapeutic dose GPN is a safer drug with regards to liver function and hepatocellular damage as compared with other antiepileptic drugs
Refining genotype–phenotype correlations in 304 patients with autosomal recessive polycystic kidney disease and PKHD1 gene variants
No full textRefining genotype-phenotype correlations in 304 patients with autosomal recessive polycystic kidney disease and <i>PKHD1</i> gene variants
No full textAutosomal recessive polycystic kidney disease (ARPKD) is a severe disease of early childhood that is clinically characterized by fibrocystic changes of the kidneys and the liver. The main cause of ARPKD are variants in the PKHD1 gene encoding the large transmembrane protein fibrocystin. The mechanisms underlying the observed clinical heterogeneity in ARPKD remain incompletely understood, partly due to the fact that genotype-phenotype correlations have been limited to the association of biallelic null variants in PKHD1 with the most severe phenotypes. In this observational study we analyzed a deep clinical dataset of 304 patients with ARPKD from two independent cohorts and identified novel genotype-phenotype correlations during childhood and adolescence. Biallelic null variants frequently show severe courses. Additionally, our data suggest that the affected region in PKHD1 is important in determining the phenotype. Patients with two missense variants affecting amino acids 709-1837 of fibrocystin or a missense variant in this region and a null variant less frequently developed chronic kidney failure, and patients with missense variants affecting amino acids 1838-2624 showed better hepatic outcome. Variants affecting amino acids 2625-4074 of fibrocystin were associated with poorer hepatic outcome. Thus, our data expand the understanding of genotype-phenotype correlations in pediatric ARPKD patients and can lay the foundation for more precise and personalized counselling and treatment approaches.sponsorship: We thank the German Society for Pediatric Nephrology (GPN) and the ESCAPE Network for their support. We thank Mr Mathias Burgmaier (Aachen) and Mr Samuel Kilian (Heidelberg) for support in conducting statistical analysis. ML was supported by grants of the GPN, the European Society for Paediatric Nephrology (ESPN), the German PKD foundation, the Koeln Fortune program, the GEROK program of the Medical Faculty of University of Cologne, and the Marga and Walter Boll-Foundation. FS, CB, and ML are supported by the German Federal Ministry of Research and Education (BMBF grant 01GM1515 and 01GM1903). KB was supported by the Koeln Fortune program and the GEROK program of the Medical Faculty of University of Cologne, as well as the Marga and Walter Boll-Foundation. This work was generated within the European Reference Network for Rare Kidney Disorders (ERKNet). The work was supported by the working groups "CAKUT" and "Inherited Kidney Diseases" of the ESPN. (German Society for Pediatric Nephrology (GPN), ESCAPE Network, European Society for Paediatric Nephrology (ESPN), German PKD foundation, Koeln Fortune program, GEROK program of the Medical Faculty of University of Cologne, Marga and Walter Boll-Foundation, German Federal Ministry of Research and Education (BMBF)|01GM1515, German Federal Ministry of Research and Education (BMBF)|01GM1903, working group "CAKUT" of the ESPN, working group "Inherited Kidney Diseases" of the ESPN)status: Publishe
Glossopharyngeal neuralgia treated by Gamma Knife radiosurgery: safety and efficacy through long-term follow-up
No full textOBJECTIVEGlossopharyngeal neuralgia (GPN) is a rare and disabling condition. Just as for trigeminal neuralgia, Gamma Knife radiosurgery (GKRS) is increasingly proposed as a therapeutic option for GPN. The purpose of this study was to assess long-term safety and efficacy of GKRS for this indication.METHODSFrom 2007 to 2015, 9 patients (4 male and 5 female) underwent a total of 10 GKRS procedures. All of the patients presented with GPN that was refractory to all medical treatment, and all had a long history of pain. One patient had previously undergone surgical microvascular decompression. In 5 cases, a neurovascular conflict had been identified on MRI. For the GKRS procedure, the glossopharyngeal nerve was localized on MRI and CT under stereotactic conditions. The target was located at the glossopharyngeal meatus of the jugular foramen. The dose administered to the nerve was 80 Gy in 3 procedures and 90 Gy in the others. Follow-up was planned for 3, 6, and 12 months after the procedure and annually thereafter.RESULTSEight patients experienced an improvement in their pain. The median length of time from GKRS to symptom improvement in this group was 7 weeks (range 2–12 months). At the first follow-up, 6 patients were pain-free (pain intensity scores of I–III, based on an adaptation of the Barrow Neurological Institute scoring system for trigeminal neuralgia), including 4 patients who were also medication-free (I). One patient had partial improvement (IV) and 2 patients had no change. The mean duration of follow-up was 46 months (range 10–90 months). At the last follow-up 6 patients remained pain-free (pain scores of I–III), including 4 patients who were pain free with no medication (I). No side effect was observed.CONCLUSIONSBecause of its safety and efficacy, GKRS appears to be a useful tool for treatment of GPN, including first-line treatment.</jats:sec
Characterization of Sensorineural Hearing Loss in Children with Alport Syndrome
No full textMost adults with Alport syndrome (AS) suffer from progressive sensorineural hearing loss. However, little is known about the early characteristics of hearing loss in children with AS. As a part of the EARLY PRO-TECT Alport trial, this study was the first clinical trial ever to investigate hearing loss in children with AS over a timespan of up to six years Nine of 51 children (18%) had hearing impairment. Audiograms were divided into three age groups: in the 5–9-year-olds, the 4-pure tone average (4PTA) was 8.9 decibel (dB) (n = 15) in those with normal hearing and 43.8 dB (n = 2, 12%) in those with hearing impairment. Among the 10–13-year-olds, 4PTA was 4.8 dB (healthy, n = 12) and 41.4 dB (hearing impaired, n = 6.33%). For the 14–20-year-olds, the 4PTA was 7.0 dB (healthy; n = 9) and 48.2 dB (hearing impaired, n = 3.25%). On average, hearing thresholds of the hearing impaired group increased, especially at frequencies between 1–3 kHz. In conclusion, 18% of children developed hearing loss, with a maximum hearing loss in the audiograms at 1–3 kHz. The percentage of children with hearing impairment increased from 10% at baseline to 18% at end of trial as did the severity of hearing loss
- …
