Ueber eine neue Synthese der Propionsäure

n/

The Volumetric Contraction of Dental Gypsum Materials on Setting

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/67364/2/10.1177_00220345530320030801.pd

Epidemiology of paediatric renal stone disease: a 22-year single centre experience in the UK

BACKGROUND: Whilst still rare, the incidence of paediatric stone disease is increasing in developed countries and it is important to evaluate the aetiology. We set up a dedicated renal stone service for children combining medical and surgical expertise in 1993 and now have a large case series of children to investigate the epidemiology. METHODS: A retrospective hospital note review of children presenting with kidney stones during the last 22 years (1993-2015) was conducted. All patients had a comprehensive infective and metabolic screen and were classified as metabolic, infective or idiopathic stone disease. RESULTS: Five hundred eleven patients (322 male) were reviewed. The median age of presentation was 4.4y for males (1 m-16.6y) and 7.3y (1-18.5y) for females with a median height and weight on the 25th centile for male and on 10th and 25th for female, respectively. One hundred seventy five (34%) had an underlying metabolic abnormality, 112 (22%) had infective stones and 224 (44%) were classified as idiopathic. Of the 175 patients with a metabolic abnormality: 91 (52%) had hypercalciuria (76 persistent and 15 transient), 37 (21%) hyperoxaluria, 38 (22%) cystinuria, 3 (2%) abnormalities in the purine metabolism and the remainder other metabolic abnormalities. Bilateral stones occurred in 27% of the metabolic group compared to 16% in the non-metabolic group (OR 0.2, p < 0.05). Urinary tract infection was a common complication (27%) in the metabolic group. CONCLUSIONS: In this paper, we present the largest cohort of paediatric stone disease reported from a developed country giving details on both, clinical and laboratory data. We show that in the majority of the patients there is an identifiable underlying metabolic and/or infective aetiology emphasizing the importance of a full work up to provide adequate treatment and prevent recurrence. Moreover, we show that stone disease in children, in contrast to the adult population, does not seem to be associated with obesity, as children have a weight below average at presentation

Glutathione depletion and increased apoptosis rate in human cystinotic proximal tubular cells

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 (6.8nmol GSH/mg protein vs 11.8nmol 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 (2.4nmol GSH/mg protein vs 7.2nmol GSH/mg protein; P<0.001). Hypoxic stress led to increased complexI and complexIV activities in control but not in cystinotic PTCs. ATP levels were significantly reduced in cystinotic PTCs after hypoxic stress (12.2nmol/mg protein vs 26.9nmol/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 complexI/IV activities. Apoptotic rate is also increased, and these mechanisms may contribute to cellular dysfunction in cultured, human cystinotic PTC

Innseiling til Borg havn – modellering av mudrings- og deponeringsoperasjoner - Spredning av finpartikulært materiale

Rapporten viser resultatene av en diagnostisk modellering av mudring og deponering ved en utdypning av innseilingen til Borg havn. I den pågående designfasen er det gjennomført modellering av turbiditet for å forutsi virkningen og omfanget av operasjonene. Turbiditetssimuleringene er gjort ved hjelp av SINTEFs partikkelbaserte DREAM-modell. DREAM-modellen bruker et detaljert datasett for strøm generert av SINTEFs numeriske 3D-modell SINMOD. Datasettet dekker de hydrodynamiske forholdene i innseilingen til Borg havn fra 1. april til 8. oktober 2013. Borg havneområde inneholder både forurenset og ikke-forurenset sediment og stein. Basert på omfattende feltmålinger ble lokalitetene av de forskjellige bunnmaterialene bestemt for to usikkerhetsnivåer (konfidensintervall). På grunn av de sensitive forholdene i Borg havneområde og den planlagte store mudringen, ble egnede og validerte mudringsteknikker brukt i simuleringen. Turbiditetsverdier ble tatt fra internasjonal litteratur, felt- og laboratoriemålinger og tilpasset DREAM-modellen. Forventede effekter er bestemt med et akseptabelt nøyaktighetsnivå. En indikasjon på innvirkningen på sårbare områder er gitt. Det er lagt vekt på at en god og miljørettet ledelse av mudringsarbeidene krever et grundig operativt overvåkingsprogram under utførelsen av arbeidene.publishedVersio

Fainting Fanconi syndrome clarified by proxy: a case report

BACKGROUND: Rare diseases may elude diagnosis due to unfamiliarity of the treating physicians with the specific disorder. Yet, advances in genetics have tremendously enhanced our ability to establish specific and sometimes surprising diagnoses. CASE PRESENTATION: We report a case of renal Fanconi syndrome associated with intermittent hypoglycemic episodes, the specific cause for which remained elusive for over 30 years, despite numerous investigations, including three kidney and one liver biopsy. The most recent kidney biopsy showed dysmorphic mitochondria, suggesting a mitochondrial disorder. When her son presented with hypoglycemia in the neonatal period, he underwent routine genetic testing for hyperinsulinemic hypoglycemia, which revealed a specific mutation in HNF4A. Subsequent testing of the mother confirmed the diagnosis also in her. CONCLUSION: Modern sequencing technologies that test multiple genes simultaneously enable specific diagnoses, even if the underlying disorder was not clinically suspected. The finding of mitochondrial dysmorphology provides a potential clue for the mechanism, by which the identified mutation causes renal Fanconi syndrome

Challenges and Opportunities offered by Flight Certification of Rotorcraft by Simulation

Newly developed aircraft must obtain a type certificate from the responsible aviation regulatory authority. This certificate testifies that the type of aircraft meets the safety requirements set by the authority. The compliance demonstration itself is the lengthiest and most expensive part of the certification process. The driving factor for the cost and duration of the compliance demonstration is the amount of ground and flight testing required. Moreover, certain certification flight test activities, particularly those involving demonstrations of control system or engine failures, can be classified as high-risk in terms of flight safety. The ROtorcraft Certification by Simulation (RoCS) project aims to explore the possibilities, limitations, and guidelines for best practices for the application of flight simulation to demonstrate compliance to the airworthiness regulations related to helicopters and tiltrotors. The paper presents the main objectives of the project and then introduces to some of the approaches that will be employed to achieve these goals