CHEMICAL AND CLINICAL OBSERVATIONS DURING TREATMENT OF CHILDREN WITH PHENYLKETONURIA

PEDIATRICS ◽  
1958 ◽  
Vol 21 (6) ◽  
pp. 929-940
Author(s):  
Helen K. Berry ◽  
Betty S. Sutherland ◽  
George M. Guest ◽  
Barbara Umbarger

Three children with phenylketonuria between the ages of 2½ and 4½ years have been treated with three different diets low in content of phenylalanine during the past 18 months. In Diets I and III restriction of phenylalanine was accomplished by use of casein hydrolysates from which phenylalanine had been removed and other amino acids added. In Diet II intake of phenylalanine was limited by restriction of total intake of protein in the diet. Diets II and III were commercial preparations. All diets were fed in semisolid form combined with natural foods. After an initial period of hospitalization for regulation of intake of phenylalanine, treatment was carried out at home. Except during the early months of treatment intake of phenylalanine was not severely restricted. Nutritional disturbances were encountered as a result of apparent amino acid deficiencies in Diet I. Inadequate caloric intake and low intake of protein also contributed to poor nutritional status at one stage in the treatment. Biochemical abnormalities characteristic of phenylketonuria were improved when phenylalanine was restricted in the diets of the three children. All three showed improvement in motor ability, increased awareness, lengthened attention span, decreased tenseness and irritability. No change in mental status on objective testing was demonstrated. The improvement in behavior motor control, and ease of handling was such that the parents were unwilling for the children to receive diets with normal content of phenylalanine. This reaction of the parents should be taken into consideration whenever administration of a diet low in content of phenylalanine is considered.

2020 ◽  
Vol 28 (2) ◽  
pp. 298-318
Author(s):  
Roman Girma Teshome

The effectiveness of human rights adjudicative procedures partly, if not most importantly, hinges upon the adequacy of the remedies they grant and the implementation of those remedies. This assertion also holds water with regard to the international and regional monitoring bodies established to receive individual complaints related to economic, social and cultural rights (hereinafter ‘ESC rights’ or ‘socio-economic rights’). Remedies can serve two major functions: they are meant, first, to rectify the pecuniary and non-pecuniary damage sustained by the particular victim, and second, to resolve systematic problems existing in the state machinery in order to ensure the non-repetition of the act. Hence, the role of remedies is not confined to correcting the past but also shaping the future by providing reforming measures a state has to undertake. The adequacy of remedies awarded by international and regional human rights bodies is also assessed based on these two benchmarks. The present article examines these issues in relation to individual complaint procedures that deal with the violation of ESC rights, with particular reference to the case laws of the three jurisdictions selected for this work, i.e. the United Nations, Inter-American and African Human Rights Systems.


Author(s):  
Ihsan Sanusi

This article in principle wants to examine the history of the emergence of the conflict of Islamic revival in Minangkabau starting from the Paderi Movement to the Youth in Minangkabau. Especially in the initial period, namely the Padri movement, there was a tragedy of violence (radicalism) that accompanied it. This study becomes important, because after all the reformation of Islam began to be realized by reforming human life in the world. Both in terms of thought with the effort to restore the correct understanding of religion as it should, from the side of the practice of religion, namely by reforming deviant practices and adapted to the instructions of the religious texts (al-Qur'an and sunnah), and also from the side of strengthening power religion. In this case the research will be directed to the efforts of renewal by the Padri to the Youth towards the Islamic community in Minangkabau. To discuss this problem used historical research methods. Through this method, it is tested and analyzed critically the records and relics of the past. In analyzing the data in this research basically used approach or interactive analysis model by Miles and Huberman. In this analysis model, the three components of the analysis are data reduction, data presentation, and conclusion drawing or verification, the activity is carried out in an interactive form with the process of collecting data as a process that continues, repeats, and continues to form acycle.


Author(s):  
Rowland W Pettit ◽  
Jordan Kaplan ◽  
Matthew M Delancy ◽  
Edward Reece ◽  
Sebastian Winocour ◽  
...  

Abstract Background The Open Payments Program, as designated by the Physician Payments Sunshine Act is the single largest repository of industry payments made to licensed physicians within the United States. Though sizeable in its dataset, the database and user interface are limited in their ability to permit expansive data interpretation and summarization. Objectives We sought to comprehensively compare industry payments made to plastic surgeons with payments made to all surgeons and all physicians to elucidate industry relationships since implementation. Methods The Open Payments Database was queried between 2014 and 2019, and inclusion criteria were applied. These data were evaluated in aggregate and for yearly totals, payment type, and geographic distribution. Results 61,000,728 unique payments totaling $11,815,248,549 were identified over the six-year study period. 9,089 plastic surgeons, 121,151 surgeons, and 796,260 total physicians received these payments. Plastic surgeons annually received significantly less payment than all surgeons (p=0.0005). However, plastic surgeons did not receive significantly more payment than all physicians (p = 0.0840). Cash and cash equivalents proved to be the most common form of payment; Stock and stock options were least commonly transferred. Plastic surgeons in Tennessee received the most in payments between 2014-2019 (mean $ 76,420.75). California had the greatest number of plastic surgeons to receive payments (1,452 surgeons). Conclusions Plastic surgeons received more in industry payments than the average of all physicians but received less than all surgeons. The most common payment was cash transactions. Over the past six years, geographic trends in industry payments have remained stable.


2017 ◽  
Vol 210 (4) ◽  
pp. 307-308 ◽  
Author(s):  
Derek K. Tracy ◽  
Dan W. Joyce ◽  
Sukhwinder S. Shergill

Quitting smoking isn't easy, even with the advent of e-cigarettes. The NHS Stop Smoking Services (SSSs) were established in 2000, and have shown superior results to nicotine replacement alone, but are characterised by low, and dropping, attendance rates. Beneath the highlight figure of a halving of UK smoking prevalence over the past 40 years lies a direct £6 billion cost to the NHS and 80000 deaths each year, as well as recent concern that clinical commissioning groups are not renewing service funding. Given that the ‘health belief model’ is based upon a trigger changing behaviour, what will encourage attendance at SSSs, especially with evidence that smokers underestimate their own personal risk? Gilbert et al randomised over 4000 smokers across almost 100 general practices to receive either a standard generic advertisement of the SSS clinic, or an individually tailored risk letter and invitation to a no-commitment introductory SSS session. The hosting general practitioners (GPs) and SSS advisors were masked to the allocation. The personalised letter more than doubled the odds of attending the SSS, showing that a more proactive approach can help engagement. Interestingly, the intervention was more effective with men, who are typically less likely to attend and set quit dates.


2021 ◽  
Vol ahead-of-print (ahead-of-print) ◽  
Author(s):  
Nadja Capus ◽  
Kei Hannah Brodersen

Purpose Corporate foreign bribery can have devastating consequences on communities and states. Over the past decade, there have been several promising developments, both national and international, that might increase the chances of victim states to receive remediation for the harm they suffered from foreign bribery. In particular, awareness has risen that victim states must be considered and new innovative items have been added to the toolbox of prosecutors in the fight against corruption that is assumed to also improve victim states’ standing in these procedures. This study aims to assess whether indeed victim states receive compensation through these novel procedures. Design/methodology/approach This study uses the three case studies of Switzerland, France and England and Wales for a comprehensive empirical and normative analysis of settlement agreements between defendants and prosecution authorities and of court jurisprudence. Findings This study shows that although de jure, it seems warranted to order the payment of remedies to victim states within domestic criminal proceedings, in practice, this rarely happens. A number of legal and practical obstacles account for this situation. This study, therefore, calls for the formulation of international guidelines containing the obligation to inform victim states of ongoing criminal proceedings on corporate foreign bribery, and guidance on how to identify the victim of this crime, as well as the damage caused. Originality/value This is the first contribution to verify whether claims that settlement agreements, recently introduced in England and Wales and France (and similar procedures are available in Switzerland), are beneficial for victim states in their quest to receive compensation. As this study shows that this is – not yet – the case in practice, this study proposes solutions that could lead the way for remediation of the harm caused by corporate corruption – and thereby, ultimately, to a more just outcome.


Medicina ◽  
2022 ◽  
Vol 58 (1) ◽  
pp. 87
Author(s):  
Agnieszka Jodzis ◽  
Maciej Walędziak ◽  
Krzysztof Czajkowski ◽  
Anna Różańska-Walędziak

Background: Global access to social media has supposedly changed women’s awareness about the pharmacological and alternative methods of pain relief during vaginal delivery. The purpose of the study was to analyze changes in women’s preference and opinion about different forms of labor analgesia over the past decade. Materials and methods: The study was designed as an anonymous survey with questions about women’s knowledge and preference of different forms of pain relief in labor. The survey was conducted in 2010 and 2020, with data collected from 1175 women in 2010 and 1033 in 2020. Results: There were no differences between 2010 and 2020 in the proportion of women who wanted to receive analgesia in labor, at, respectively 67.9% of women in 2010 and 73.9% in 2020. About 50% of women chose epidural analgesia as the only efficacious method of pain relief in labor both in 2010 and 2020. There were no differences between the two time-points in the distribution of chosen methods of pain relief. In total, 92.3% of women in 2010 and 94.9% in 2020 thought that they should have the possibility of independent choice of analgesia method before the delivery (p < 0.04). Conclusions: A high proportion of Polish women choose EDA over other pharmacological and nonpharmacological methods of pain relief in labor, and this preference has not changed over the last decade. Increasing women’s knowledge about different methods of intrapartum pain relief may lead to wider use of nonpharmacological methods of pain relief.


Author(s):  
Marc Logarta ◽  
Anne McCready ◽  
Alan Cheng ◽  
Alexander Duller

Objectives Investigation of whether the duration of fasting preoperatively has an impact on pain and return to normal function after tonsillectomy To investigate the impact of the duration of preoperative fasting on pain and return to normal function after tonsillectomy. Design Retrospective study with surgeon blinded to the participants undergoing tonsillectomy with or without adenoidectomy Setting University teaching hospital, Sydney, Australia Participants A total of 174 children (n=174) ages 4-12 years old between February 2017 to April 2020 were enrolled. The children were randomised to receive instructions either from the staff at the hospital (n=90) or by the anaesthetist (n=84) in terms of exact pre-operative fasting. Main Outcome measures Fasting times, clear liquid caloric intake 2 hours prior to surgery and Parents Postoperative Pain Measures (PPPM) were documented and analysed. Secondary measures of analgesia usage, number of postoperative nausea or vomiting events were also analysed. Results Observed difference were noted between the instructed fasting period and the actual fasting period. Significant difference in PPPM scores was noted between the Hospital group and the juice group (p<0.001). No significant difference was noted at first to 3rd day (p=0.11) or by the 8th to 10th postoperative day (p=0.93). There was no difference in oxycodone usage between the two groups (p=0.52), or in postoperative nausea and vomiting (p=0.43) Conclusions Significantly lower PPPM scores were noted on day of surgery in the group that had fasting minimised. The effects appeared to trend in a positive manner but larger number of cases need to be performed for results to be of greater significance. Further research is required into the impact of caloric nutrition to enhance recovery after tonsillectomy.


2008 ◽  
Vol 7 (3) ◽  
pp. 106-106
Author(s):  
Chris Roseveare ◽  

There is no doubt that this Winter has been tough for those of us working in Acute Medical Units. At the time of writing I find myself in the depths of the post-Christmas blues; the combination of Bank Holidays, ward closures, junior doctor illness and huge admission surges have conspired to make January even more challenging than normal. No doubt the printing delays will have meant that, by the time you are reading this Editorial, these days will be a distant memory. Maybe this will serve as a useful reminder of the need for forward planning in time for next year, rather than breathing the usual sigh of relief as we steel ourselves for Easter. Perhaps a more imaginative colour coding system for our ‘alert’ system would be a start – simply alternating between ‘red alert’ (awful) and ‘black alert’ (even worse) can become quite tedious as the weeks pass. A few shades of maroon or magenta to remind us that ‘today is slightly better than yesterday’ might help break the monotony. We could even opt for a full rainbow spectrum to signify the hope that one day all of our efforts will be worthwhile….it’s important to stay positive in these difficult times! I frequently tell our students ‘When you hear hooves, think horses…not zebras’ – an alternative (and unoriginal) way to remind them that ‘common things occur commonly’. But of course not all chest pain is ischaemic in origin, and not every fever is caused by pneumonia – even in January. The case reports in this edition illustrate some of the more unusual causes of acute medical admission, and the importance of maintaining an open mind. Takotsubo cardiomyopathy is a condition which seems to have passed me by in my medical practice to-date; on reading this case I wondered how many patients I have inappropriately thrombolysed over the past 15 years. In the new world of Heart Attack Centres and urgent percutaneous intervention maybe we will find it is more common than was previously thought. Porphyrias and vasculitidies are occasionally sent to challenge us, but give us the opportunity to demonstrate the power of lateral thinking which distinguishes us as Physicians. As the authors remind us in their title – porphyria will only be diagnosed if it is considered in the differential; it is easy forget that the combination of psychiatric illness and abdominal pain does not always imply Irritable Bowel Syndrome! Cerebral Nocardiosis should not feature high up in the differential diagnosis when a 90 year old patient presents with a hemiparesis, even in the context of immune deficiency. The temptation to presume that the ‘ring enhancing lesion’ seen on his CT was neoplastic, must have been considerable for the team caring for this patient. The importance of a tissue diagnosis, even in this age group, is emphasised by the outcome of this case. I hope you enjoy this edition and hopefully we are now getting back on track with the scheduling. Please keep the submissions coming in; we are starting to receive some interesting pieces of research, which will be included over the next year pending review. If any more readers would like to volunteer to become editorial referees for future editions, please feel free to contact me directly on the email shown.


Author(s):  
Anthony Trollope
Keyword(s):  
The Past ◽  

Phineas Finn and Lady Laura Kennedy sat together discussing the affairs of the past till the servant told them that ‘My Lord’ was in the next room, and ready to receive Mr Finn. ‘You will find him much altered,’ said Lady Laura, ‘even more...


Author(s):  
Edward B. Foley

Each state already has the constitutional power to require that candidates win a majority of the popular vote to receive all of the state’s electoral votes. Each state could adopt the kind of runoff that New Hampshire used in the past, or instant runoff voting. There is no need for a multistate compact. If only two or three states had used runoffs, or instant runoff voting, in 2016—for example, Florida and Michigan, or the three Rust Belt states of Michigan, Wisconsin, and Pennsylvania—and if Clinton had won those runoffs, then she would have been president. In the future, it might be a Republican candidate who prevails in runoffs in pivotal states but would lose using plurality winner-take-all. States with ballot initiatives can use them to require majority rule for appointing electors as long as they leave the specific details to legislation.


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