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2009 ESC/ERS pulmonary hypertension guidelines and connective tissue disease.

2009 ESC/ERS pulmonary hypertension guidelines and connective tissue disease.

Allergol Int. 2011 Dec;60(4):419-24

Authors: Nakanishi N, ,

Abstract
Pulmonary hypertension was defined as mean pulmonary artery pressure ≥ 25 mmHg at the 4th World Symposium on Pulmonary Hypertension. In 2009, the European Society of Cardiology and European Respiratory Society jointly created guidelines for practical pulmonary hypertension classifications and treatments based on the discussions at the 4th World Symposium. This classification is characterized by division into five groups: Pulmonary arterial hypertension (PAH); Pulmonary hypertension due to left heart disease; Pulmonary hypertension due to lung disease and/or hypoxia; Chronic thromboembolic pulmonary hypertension; and Pulmonary hypertension with unclear and/or multifactorial mechanisms. PAH is a common and fatal complication of connective tissue disease (CTD), but pulmonary hypertension in CTD consists of PAH, pulmonary hypertension caused by myocardial involvement, pulmonary veno-occlusive disorder, pulmonary hypertension due to interstitial lung disease. PAH has been studied widely in SSc and the estimated prevalence of 7-12%. Treatment of CTD associated PAH (CTD-PAH) consists of general therapeutic options and specific treatment. Specific treatment of CTD-PAH patients is targeted to produce vasodilatation. Calcium channel blockers (CCBs) are indicated in cases where a sufficient decrease in pulmonary arterial pressure is seen in vasoreactivity testing. If vasoreactivity is absent in CTD-PAH patients, the treatment consists of the endothelin receptor antagonists, the prostacyclin analogues and phosphodiesterase-type 5 inhibitors. Few data are available to support the use of immunosuppression in CTD-PAH. However, some case reports suggested that a minority of CTD-PAH patients could benefit from immunosuppressive therapy. The treatment of CTD-PAH patients may differ from the treatment of idiopathic PAH.

PMID: 22015568 [PubMed - indexed for MEDLINE]

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Appendix to dialysis centre guidelines: recommendations for the relationship between outpatient haemodialysis centres and reference hospitals. Opinions from the Outpatient Dialysis Group. Grupo de Trabajo de Hemodiálisis Extrahospitalaria.

Appendix to dialysis centre guidelines: recommendations for the relationship between outpatient haemodialysis centres and reference hospitals. Opinions from the Outpatient Dialysis Group. Grupo de Trabajo de Hemodiálisis Extrahospitalaria.

Nefrologia. 2011 Nov 25;31(6):664-9

Authors: Berdud I, Arenas MD, Bernat A, Ramos R, Blanco A,

Abstract
INTRODUCTION: The different clinical guidelines backed by the Spanish Society of Nephrology (SEN) attempt to homogenise the monitoring of renal patients. However, this effort to homogenise treatment has been obstructed in the case of renal replacement therapy patients on haemodialysis due to, among other reasons, the existence of several different dialysis providers, with private centres located in many cities, each with their own reference hospitals and different criteria for treatment based on the existing outsourcing services agreements with the public health service, which also differ between regions. A good relationship between a private dialysis centre and its reference hospital would lead to equal treatment for all dialysis patients, at least at that particular town. The SEN, through the efforts of the Grupo de Trabajo de Hemodiálisis Extrahospitalaria (Outpatient Haemodialysis Group), has prioritised a close relationship and good communication between reference hospitals and dialysis centres in order to guarantee proper continuity of the health care given to these patients. STRATEGIES FOR IMPROVEMENT: Conditions for referring patients from one centre to another. A patient that starts a haemodialysis programme should be referred from a reference hospital with a definitive vascular access for optimising treatment, with a full report updated within 24-48 hours before the transferral, including essential information for providing proper nephrological treatment: primary pathology, recent viral serology (including hepatitis B and C virus [HBV and HCV] and human immunodeficiency virus [HIV]), parameters for anaemia and calcium-phosphorus metabolism, and ions, date of the first session of dialysis, and the number and dates of blood transfusions received. Furthermore, patients referred from the dialysis centre to the hospital, whether for programmed visits or emergency hospitalisation, should be accompanied by an updated report indicating the primary diagnoses, recent events, viral serology and laboratory analyses, updated haemodialysis and treatment regimens used, and the reason for transferral to the hospital. A single, digital clinical history that is accessible by both institutions would facilitate this situation, although this option is not completely available to all centres and hospitals. There are also legal issues to resolve in this aspect. Continued care for dialysis patients. Good communication between dialysis centres and hospitals is fundamental for achieving a proper level of care for dialysis patients, and not only with the nephrology department. The interconsultations of dialysis patients at each private centre, as well as the requests for diagnostic tests, should be able to be requested by the centre directly. The results and reports from these interconsultations should also be sent to the centre. It would also be best if the reference hospitals and their private dialysis centres shared common treatment protocols. These protocols should include basic aspects of the treatment of renal patients (anaemia, mineral metabolism, vascular accesses including catheter infections, etc., and laboratory tests), transplant protocols, complementary tests, and other components specific to each area. Not only would this generalise and unify the approach taken with dialysis patients regardless of where they are treated, it would also facilitate access to data on all patients regarding clinical trials and research studies. Access to medication. Dialysis patients require medications that are only given in the hospital setting, which is normally provided by the reference hospital, as per the agreement between institutions. It would also be recommendable that any other medications not included in the agreement (antibiotics, urokinase, nutritional supplements, etc.) be dispensed in a similar manner. Access to kidney transplant. The management of the transplant waiting list, once a patient starts renal replacement therapy, should be controlled from the dialysis centre, as in any other procedure. As such, the nephrologists from each centre should be familiar with the existing protocols and new developments in this context, and should participate in meetings with nephrology and urology departments in each hospital. The transplant protocol at each town/region should be followed for all patients, whether dialysis is undergone in a hospital or private centre. Characteristics of the work at dialysis centres. The doctor attending patients at each dialysis centre must be a specialist in nephrology. This complicated issue must be a requirement for agreements within the regional health system in order to guarantee a proper and equitable treatment of patients that receive dialysis in private centres. Only in the case of an absence of a nephrologist should a general practitioner be used, and this doctor must have adequate training in haemodialysis. This training should also be standardised. Over 75% of nephrologists that work at these centres are alone during the workday, and 40% never see another colleague during the whole shift. The administrators of these centres should seek out protocols that provide professional contact, both with the hospital staff and nephrologists from other centres, which would facilitate an exchange of ideas. Training. The nephrologists at each centre have the right and the obligation to perform research and to continuously expand their training, so as to develop and improve health care provision. Since the majority of patients in haemodialysis programmes are treated in outpatient centres that depend on reference hospitals, we might suggest a minimal rotation of nephrology residents in private outpatient dialysis centres, once accreditation has been given for providing this training.

PMID: 22130281 [PubMed - indexed for MEDLINE]

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Canadian Thoracic Society 2011 guideline update: diagnosis and treatment of sleep disordered breathing.

Canadian Thoracic Society 2011 guideline update: diagnosis and treatment of sleep disordered breathing.

Can Respir J. 2011 Jan-Feb;18(1):25-47

Authors: Fleetham J, Ayas N, Bradley D, Fitzpatrick M, Oliver TK, Morrison D, Ryan F, Series F, Skomro R, Tsai W,

Abstract
The Canadian Thoracic Society (CTS) published an executive summary of guidelines for the diagnosis and treatment of sleep disordered breathing in 2006⁄2007. These guidelines were developed during several meetings by a group of experts with evidence grading based on committee consensus. These guidelines were well received and the majority of the recommendations remain unchanged. The CTS embarked on a more rigorous process for the 2011 guideline update, and addressed eight areas that were believed to be controversial or in which new data emerged. The CTS Sleep Disordered Breathing Committee posed specific questions for each area. The recommendations regarding maximum assessment wait times, portable monitoring, treatment of asymptomatic adult obstructive sleep apnea patients, treatment with conventional continuous positive airway pressure compared with automatic continuous positive airway pressure, and treatment of central sleep apnea syndrome in heart failure patients replace the recommendations in the 2006⁄2007 guidelines. The recommendations on bariatric surgery, complex sleep apnea and optimum positive airway pressure technologies are new topics, which were not covered in the 2006⁄2007 guidelines.

PMID: 21369547 [PubMed - indexed for MEDLINE]

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Canadian guideline for safe and effective use of opioids for chronic noncancer pain: clinical summary for family physicians. Part 1: general population.

Canadian guideline for safe and effective use of opioids for chronic noncancer pain: clinical summary for family physicians. Part 1: general population.

Can Fam Physician. 2011 Nov;57(11):1257-66, e407-18

Authors: Kahan M, Mailis-Gagnon A, Wilson L, Srivastava A,

Abstract
OBJECTIVE: To provide family physicians with a practical clinical summary of the Canadian Guideline for Safe and Effective Use of Opioids for Chronic Non-Cancer Pain, developed by the National Opioid Use Guideline Group.
QUALITY OF EVIDENCE: Researchers for the guideline conducted a systematic review of the literature on the effectiveness and safety of opioids for chronic noncancer pain, and drafted a series of recommendations. A panel of 49 clinicians from across Canada reviewed the draft and achieved consensus on 24 recommendations.
MAIN MESSAGE: Screening for addiction risk is recommended before prescribing opioids. Weak opioids (codeine and tramadol) are recommended for mild to moderate pain that has not responded to first-line treatments. Oxycodone, hydromorphone, and morphine can be tried in patients who have not responded to weaker opioids. A low initial dose and slow upward titration is recommended, with patient education and close monitoring. Physicians should watch for the development of complications such as sleep apnea. The optimal dose is one which improves function or decreases pain ratings by at least 30%. For by far most patients, the optimal dose will be well below a 200-mg morphine equivalent dose per day. Tapering is recommended for patients who have not responded to an adequate opioid trial.
CONCLUSION: Opioids play an important role in the management of chronic noncancer pain, but careful prescribing is needed to limit potential harms. The new Canadian guideline provides much-needed guidance to help physicians achieve a balance between optimal pain control and safety.

PMID: 22084455 [PubMed - indexed for MEDLINE]

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Canadian guideline for safe and effective use of opioids for chronic noncancer pain: clinical summary for family physicians. Part 2: special populations.

Canadian guideline for safe and effective use of opioids for chronic noncancer pain: clinical summary for family physicians. Part 2: special populations.

Can Fam Physician. 2011 Nov;57(11):1269-76, e419-28

Authors: Kahan M, Wilson L, Mailis-Gagnon A, Srivastava A,

Abstract
OBJECTIVE: To provide family physicians with a practical clinical summary of opioid prescribing for specific populations based on recommendations from the Canadian Guideline for Safe and Effective Use of Opioids for Chronic Non-Cancer Pain.
QUALITY OF EVIDENCE: Researchers for the guideline conducted a systematic review of the literature, focusing on reviews of the effectiveness and safety of opioids in specific populations.
MAIN MESSAGE: Family physicians can minimize the risks of overdose, sedation, misuse, and addiction through the use of strategies tailored to the age and health status of patients. For patients at high risk of addiction, opioids should be reserved for well-defined nociceptive or neuropathic pain conditions that have not responded to first-line treatments. Opioids should be titrated slowly, with frequent dispensing and close monitoring for signs of misuse. Suspected opioid addiction is managed with structured opioid therapy, methadone or buprenorphine treatment, or abstinence-based treatment. Patients with mood and anxiety disorders tend to have a blunted analgesic response to opioids, are at higher risk of misuse, and are often taking sedating drugs that interact adversely with opioids. Precautions similar to those for other high-risk patients should be employed. The opioid should be tapered if the patient’s pain remains severe despite an adequate trial of opioid therapy. In the elderly, sedation, falls, and overdose can be minimized through lower initial doses, slower titration, benzodiazepine tapering, and careful patient education. For pregnant women taking daily opioid therapy, the opioids should be slowly tapered and discontinued. If this is not possible, they should be tapered to the lowest effective dose. Opioid-dependent pregnant women should receive methadone treatment. Adolescents are at high risk of opioid overdose, misuse, and addiction. Patients with adolescents living at home should store their opioid medication safely. Adolescents rarely require long-term opioid therapy.
CONCLUSION: Family physicians must take into consideration the patient’s age, psychiatric status, level of risk of addiction, and other factors when prescribing opioids for chronic pain.

PMID: 22084456 [PubMed - indexed for MEDLINE]

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The diagnosis of mild cognitive impairment due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease.

The diagnosis of mild cognitive impairment due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease.

Alzheimers Dement. 2011 May;7(3):270-9

Authors: Albert MS, DeKosky ST, Dickson D, Dubois B, Feldman HH, Fox NC, Gamst A, Holtzman DM, Jagust WJ, Petersen RC, Snyder PJ, Carrillo MC, Thies B, Phelps CH

Abstract
The National Institute on Aging and the Alzheimer’s Association charged a workgroup with the task of developing criteria for the symptomatic predementia phase of Alzheimer’s disease (AD), referred to in this article as mild cognitive impairment due to AD. The workgroup developed the following two sets of criteria: (1) core clinical criteria that could be used by healthcare providers without access to advanced imaging techniques or cerebrospinal fluid analysis, and (2) research criteria that could be used in clinical research settings, including clinical trials. The second set of criteria incorporate the use of biomarkers based on imaging and cerebrospinal fluid measures. The final set of criteria for mild cognitive impairment due to AD has four levels of certainty, depending on the presence and nature of the biomarker findings. Considerable work is needed to validate the criteria that use biomarkers and to standardize biomarker analysis for use in community settings.

PMID: 21514249 [PubMed - indexed for MEDLINE]

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The diagnosis of dementia due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease.

The diagnosis of dementia due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease.

Alzheimers Dement. 2011 May;7(3):263-9

Authors: McKhann GM, Knopman DS, Chertkow H, Hyman BT, Jack CR, Kawas CH, Klunk WE, Koroshetz WJ, Manly JJ, Mayeux R, Mohs RC, Morris JC, Rossor MN, Scheltens P, Carrillo MC, Thies B, Weintraub S, Phelps CH

Abstract
The National Institute on Aging and the Alzheimer’s Association charged a workgroup with the task of revising the 1984 criteria for Alzheimer’s disease (AD) dementia. The workgroup sought to ensure that the revised criteria would be flexible enough to be used by both general healthcare providers without access to neuropsychological testing, advanced imaging, and cerebrospinal fluid measures, and specialized investigators involved in research or in clinical trial studies who would have these tools available. We present criteria for all-cause dementia and for AD dementia. We retained the general framework of probable AD dementia from the 1984 criteria. On the basis of the past 27 years of experience, we made several changes in the clinical criteria for the diagnosis. We also retained the term possible AD dementia, but redefined it in a manner more focused than before. Biomarker evidence was also integrated into the diagnostic formulations for probable and possible AD dementia for use in research settings. The core clinical criteria for AD dementia will continue to be the cornerstone of the diagnosis in clinical practice, but biomarker evidence is expected to enhance the pathophysiological specificity of the diagnosis of AD dementia. Much work lies ahead for validating the biomarker diagnosis of AD dementia.

PMID: 21514250 [PubMed - indexed for MEDLINE]

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Diagnostic evaluation and management of chronic thromboembolic pulmonary hypertension: a clinical practice guideline.

Diagnostic evaluation and management of chronic thromboembolic pulmonary hypertension: a clinical practice guideline.

Can Respir J. 2010 Nov-Dec;17(6):301-34

Authors: Mehta S, Helmersen D, Provencher S, Hirani N, Rubens FD, De Perrot M, Blostein M, Boutet K, Chandy G, Dennie C, Granton J, Hernandez P, Hirsch AM, Laframboise K, Levy RD, Lien D, Martel S, Shoemaker G, Swiston J, Weinkauf J, ,

Abstract
BACKGROUND: Pulmonary embolism is a common condition. Some patients subsequently develop chronic thromboembolic pulmonary hypertension (CTEPH). Many care gaps exist in the diagnosis and management of CTEPH patients including lack of awareness, incomplete diagnostic assessment, and inconsistent use of surgical and medical therapies.
METHODS: A representative interdisciplinary panel of medical experts undertook a formal clinical practice guideline development process. A total of 20 key clinical issues were defined according to the patient population, intervention, comparator, outcome (PICO) approach. The panel performed an evidence-based, systematic, literature review, assessed and graded the relevant evidence, and made 26 recommendations.
RESULTS: Asymptomatic patients postpulmonary embolism should not be screened for CTEPH. In patients with pulmonary hypertension, the possibility of CTEPH should be routinely evaluated with initial ventilation/perfusion lung scanning, not computed tomography angiography. Pulmonary endarterectomy surgery is the treatment of choice in patients with surgically accessible CTEPH, and may also be effective in CTEPH patients with disease in more ‘distal’ pulmonary arteries. The anatomical extent of CTEPH for surgical pulmonary endarterectomy is best assessed by contrast pulmonary angiography, although positive computed tomography angiography may be acceptable. Novel medications indicated for the treatment of pulmonary hypertension may be effective for selected CTEPH patients.
CONCLUSIONS: The present guideline requires formal dissemination to relevant target user groups, the development of tools for implementation into routine clinical practice and formal evaluation of the impact of the guideline on the quality of care of CTEPH patients. Moreover, the guideline will be updated periodically to reflect new evidence or clinical approaches.

PMID: 21165353 [PubMed - indexed for MEDLINE]

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Guidelines for the diagnosis and treatment of primary aldosteronism–the Japan Endocrine Society 2009.

Guidelines for the diagnosis and treatment of primary aldosteronism–the Japan Endocrine Society 2009.

Endocr J. 2011 Sep 30;58(9):711-21

Authors: Nishikawa T, Omura M, Satoh F, Shibata H, Takahashi K, Tamura N, Tanabe A,

Abstract
The Japan Endocrine Society (JES) attempted to develop guidelines for the diagnosis and treatment of primary aldosteronism (PA). The Task Force Committee (TFC) was composed of a chair, selected by the JES, and additional experts. Systematic reviews of available evidence for Japanese patients were used to recommend the key treatment and prevention. We have evaluated the methods of screening, confirmatory tests and imaging, plus adrenal vein sampling (AVS). Consensus was guided by systematic review of evidence and discussion during each annual meeting of the JES, plus its related meetings, and by e-mail communication. The drafts prepared by TFC were reviewed successively by the members of Research on Intractable Diseases provided by the Japanese Ministry of Health, Labour and Welfare, and in comments from the JES’s councilors. At each stage of review, TFC received written comments and incorporated suggested changes. In conclusion, all patients with hypertension should be screened for PA, because of the high prevalence of cardiovascular disease and the current low case-detection rate in Japan. Case detection can be performed in hypertensive patients and those with hypokalemia by determining the aldosterone/renin ratio, and the diagnosis of PA can be confirmed by two of three confirmatory tests. The presence of a unilateral aldosterone-producing adenoma should be established/excluded by AVS by an experienced radiologist, optimally followed by laparoscopic adrenalectomy. In contrast, patients with bilateral adrenal hyperplasia, or those unsuitable for surgery, are optimally treated medically with mineralocorticoid receptor antagonists.

PMID: 21828936 [PubMed - indexed for MEDLINE]

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EMQN best practice guidelines for the laboratory diagnosis of osteogenesis imperfecta.

EMQN best practice guidelines for the laboratory diagnosis of osteogenesis imperfecta.

Eur J Hum Genet. 2012 Jan;20(1):11-9

Authors: van Dijk FS, Byers PH, Dalgleish R, Malfait F, Maugeri A, Rohrbach M, Symoens S, Sistermans EA, Pals G

Abstract
Osteogenesis imperfecta (OI) comprises a group of inherited disorders characterized by bone fragility and increased susceptibility to fractures. Historically, the laboratory confirmation of the diagnosis OI rested on cultured dermal fibroblasts to identify decreased or abnormal production of abnormal type I (pro)collagen molecules, measured by gel electrophoresis. With the discovery of COL1A1 and COL1A2 gene variants as a cause of OI, sequence analysis of these genes was added to the diagnostic process. Nowadays, OI is known to be genetically heterogeneous. About 90% of individuals with OI are heterozygous for causative variants in the COL1A1 and COL1A2 genes. The majority of remaining affected individuals have recessively inherited forms of OI with the causative variants in the more recently discovered genes CRTAP, FKBP10, LEPRE1,PLOD2, PPIB, SERPINF1, SERPINH1 and SP7, or in other yet undiscovered genes. These advances in the molecular genetic diagnosis of OI prompted us to develop new guidelines for molecular testing and reporting of results in which we take into account that testing is also used to ‘exclude’ OI when there is suspicion of non-accidental injury. Diagnostic flow, methods and reporting scenarios were discussed during an international workshop with 17 clinicians and scientists from 11 countries and converged in these best practice guidelines for the laboratory diagnosis of OI.

PMID: 21829228 [PubMed - indexed for MEDLINE]

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