Adult North Star Network (ANSN): Consensus Guideline For The Standard Of Care Of Adults With Duchenne Muscular Dystrophy.

There are growing numbers of adults with Duchenne Muscular Dystrophy living well into their fourth decade. These patients have complex medical needs that to date have not been addressed in the International standards of care. We sought to create a consensus based standard of care through a series of multi-disciplinary workshops with specialists from a wide range of clinical areas: Neurology, Cardiology, Respiratory Medicine, Gastroenterology, Endocrinology, Palliative Care Medicine, Rehabilitation, Renal, Anaesthetics and Clinical Psychology. Detailed reports of evidence reviewed and the consensus building process were produced following each workshop and condensed into this final document which was approved by all members of the Adult North Star Network including service users. The aim of this document is to provide a framework to improve clinical services and multi-disciplinary care for adults living with Duchenne Muscular Dystrophy.

Ketoacidosis in Duchenne muscular dystrophy: A report on 4 cases.

The longer survival in Duchenne dystrophy can be associated with previously unrecognised medical issues, in particular cardiac and nutritional problems, which provide new challenges in the management of these patients. We describe a series of patients who have DMD presenting with severe acid base abnormalities due to ketoacidosis. The cases described all developed the abnormalities in the context of decreased dietary intake. In all cases, the acid-base derangement resolved with intravenous glucose infusion. We conclude that decreased calorific intake for any reason can lead to very severe acid-base derangement in patients with DMD. The treatment is relatively simple once the problem has been identified. An awareness of this potential complication is important for all clinicians involved in the acute care of these patients.

Modulation of protein kinase C-epsilon by phorbol esters in the monoblastoid U937 cell.

Expression of protein kinase C-epsilon was examined in the human monoblastoid U937 cell. This cell type contained the alpha, beta, and epsilon isoforms of protein kinase C (PKC). While PKC-epsilon content was slightly higher in the cytosolic than in the particulate fraction, the amount contained in the particulate fraction was higher than the alpha and beta isoforms which were predominantly localized to the cytosol. After an acute exposure to tetradecanoyl-13-phorbol acetate (TPA), PKC-epsilon translocated to the particulate fraction. Acute or chronic exposure to ionomycin did not alter content of the epsilon isoform. Longer exposures to TPA decreased PKC-epsilon in both cellular fractions. PKC-epsilon displayed a similar sensitivity to TPA-induced down-regulation as did PKC-beta while PKC-alpha was more resistant to this effect. After a 72-h exposure to 0.1 nM TPA, increases in the alpha and beta isoforms but not in PKC-epsilon were observed. However, 1,25-dihydroxy vitamin D3 and dibutyryl cyclic AMP which induce U937 differentiation enhanced PKC-epsilon expression.

Local hydration environments of amino acids and dipeptides studied by X-ray spectroscopy of liquid microjets.

The nitrogen K-edge spectra of aqueous proline and diglycine solutions have been measured by total electron yield near-edge X-ray absorption fine structure (NEXAFS) spectroscopy at neutral and high pH. All observed spectral features have been assigned by comparison to the recently reported spectrum of aqueous glycine and calculated spectra of isolated amino acids and hydrated amino acid clusters. The sharp preedge resonances at 401.3 and 402.6 eV observed in the spectrum of anionic glycine indicate that the nitrogen terminus is in an "acceptor-only" configuration, wherein neither amine proton is involved in hydrogen bonding to the solvent, at high pH. The analogous 1s --> sigma(NH) preedge transitions are absent in the NEXAFS spectrum of anionic proline, implying that the acceptor-only conformation observed in anionic glycine arises from steric shielding induced by free rotation of the amine terminus about the glycine CN bond. Anionic diglycine solutions exhibit a broadened 1s --> pi(CN) resonance at 401.2 eV and a broad shoulder resonance at 403 eV, also suggesting the presence of an acceptor-only species. Although this assignment is not as unambiguous as for glycine, it implies that the nitrogen terminus of most proteins is capable of existing in an acceptor-only conformation at high pH. The NEXAFS spectrum of zwitterionic lysine solution was also measured, exhibiting features similar to those of both anionic and zwitterionic glycine, and leading us to conclude that the alpha amine group is present in an acceptor-only configuration, while the end of the butylammonium side chain is fully solvated.

pH dependence of the electronic structure of glycine.

The carbon, nitrogen, and oxygen K-edge spectra were measured for aqueous solutions of glycine by total electron yield near-edge X-ray absorption fine structure (TEY NEXAFS) spectroscopy. The bulk solution pH was systematically varied while maintaining a constant amino acid concentration. Spectra were assigned through comparisons with both previous studies and ab initio computed spectra of isolated glycine molecules and hydrated glycine clusters. Nitrogen K-edge solution spectra recorded at low and moderate pH are nearly identical to those of solid glycine, whereas basic solution spectra strongly resemble those of the gas phase. The carbon 1s --> pi*(C=O) transition exhibits a 0.2 eV red shift at high pH due to the deprotonation of the amine terminus. This deprotonation also effects a 1.4 eV red shift in the nitrogen K-edge at high pH. Two sharp preedge features at 401.3 and 402.5 eV are also observed at high pH. These resonances, previously observed in the vapor-phase ISEELS spectrum of glycine, have been reassigned as transitions to sigma* bound states. The observation of these peaks indicates that the amine moiety is in an acceptor-only hydrogen bond configuration at high pH. At low pH, the oxygen 1s --> pi*(C=O) transition exhibits a 0.25-eV red shift due to the protonation of the carboxylic acid terminus. These spectral differences indicate that the variations in electronic structure observed in the NEXAFS spectra are determined by the internal charge state and hydration environment of the molecule in solution.

Total joint replacement preadmission programs.

Patients begin to formulate their expectations of the postoperative hospitalization during the preadmission program. The challenge is to better understand the factors patients consider when formulating judgments about the quality of preadmission education. For example, it may be that perceptions of the preadmission program are influenced by what patients believe about their postoperative pain and functional abilities. Specific attention needs to be given both preoperatively and postoperatively to instructing patients on realistic expectations for recovery. One other method of measuring patient outcomes is with the Health Status Profile (SF-36) (Response Healthcare Information Management, 1995). The SF-36 approach emphasizes the outcome of medical care as the patient sees it, in addition to a clinical evaluation of successful health care. This form is currently initiated in the physician's office and returned for scanning at the preadmission class. The patient then completes another SF-36 at 6 months and every year thereafter to compare measurable outcomes. Patients intending to have elective total joint replacements experience anxiety and require much support and education. An effective preadmission program is a major investment in a patient's recovery, as well as a unique marketing tool to customers. Preadmission programs can be viewed as an opportunity to enhance customer satisfaction. Preadmission clinics are an excellent means for nurses to improve the quality of patient care through patient education. the overall goal of preadmission testing programs is to ensure patient preparedness while increasing quality health care and overall customer satisfaction. To enhance program effectiveness, health care providers must lead collaborative efforts to improve the efficiency of systems.

The prognostic variables predictive of mortality in patients with an exacerbation of COPD admitted to the ICU: an integrative review.

INTRODUCTION: Chronic Obstructive Pulmonary Disease (COPD) frequently presents with an acute exacerbation (AECOPD). Debate exists as to whether these patients should be admitted to intensive care units (ICUs). An integrative review was performed to determine whether clinical variables available at the time of ICU admission are predictive of the intermediate-term mortality of patients with an AECOPD. METHODS: An integrative review was structured to incorporate a five-stage review framework to facilitate data extraction, analysis and presentation. The quality of the studies contributing to the integrative review was assessed with a novel scoring system developed from previously published data and adapted to this setting. RESULTS: The integrative review search strategy identified 28 studies assessing prognostic variables in this setting. Prognostic variables associated with intermediate-term mortality were low Glasgow Coma Scale (GCS) on admission to ICU, cardio-respiratory arrest prior to ICU admission, cardiac dysrhythmia prior to ICU admission, length of hospital stay prior to ICU admission and higher values of acute physiology scoring systems. Premorbid variables such as age, functional capacity, pulmonary function tests, prior hospital or ICU admissions, body mass index and long-term oxygen therapy were not found to be associated with intermediate-term mortality nor was the diagnosis attributed to the cause of the AECOPD. DISCUSSION: Variables associated with intermediate-term mortality after AECOPD requiring ICU admission are those variables, which reflect underlying severity of acute illness. Premorbid and diagnostic data have not been shown to be predictive of outcome. A scoring system is proposed to assess studies of prognosis in AECOPD.