scholarly journals JNETS clinical practice guidelines for gastroenteropancreatic neuroendocrine neoplasms: diagnosis, treatment, and follow-up: a synopsis

2021 ◽  
Author(s):  
Tetsuhide Ito ◽  
Toshihiko Masui ◽  
Izumi Komoto ◽  
Ryuichiro Doi ◽  
Robert Y. Osamura ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Clinical Practice Guidelines ◽  
Practice Guidelines ◽  
Somatostatin Receptor ◽  
Multidisciplinary Treatment ◽  
Clinical Manifestations ◽  
Treatment Strategies ◽  
Neuroendocrine Neoplasms ◽  
Gastroenteropancreatic Neuroendocrine Neoplasms ◽  
Vhl Disease

AbstractNeuroendocrine neoplasms (NENs) are rare neoplasms that occur in various organs and present with diverse clinical manifestations. Pathological classification is important in the diagnosis of NENs. Treatment strategies must be selected according to the status of differentiation and malignancy by accurately determining whether the neoplasm is functioning or nonfunctioning, degree of disease progression, and presence of metastasis. The newly revised Clinical Practice Guidelines for Gastroenteropancreatic Neuroendocrine Neoplasms (GEP-NENs) comprises 5 chapters—diagnosis, pathology, surgical treatment, medical and multidisciplinary treatment, and multiple endocrine neoplasia type 1 (MEN1)/von Hippel–Lindau (VHL) disease—and includes 51 clinical questions and 19 columns. These guidelines aim to provide direction and practical clinical content for the management of GEP-NEN preferentially based on clinically useful reports. These revised guidelines also refer to the new concept of “neuroendocrine tumor” (NET) grade 3, which is based on the 2017 and 2019 WHO criteria; this includes health insurance coverage of somatostatin receptor scintigraphy for NEN, everolimus for lung and gastrointestinal NET, and lanreotide for GEP-NET. The guidelines also newly refer to the diagnosis, treatment, and surveillance of NEN associated with VHL disease and MEN1. The accuracy of these guidelines has been improved by examining and adopting new evidence obtained after the first edition was published.

Clinical and subclinical findings in heterozygous ABCC6 carriers: results from a Belgian cohort and clinical practice guidelines

2021 ◽  
pp. jmedgenet-2020-107565
Author(s):  
Lukas Nollet ◽  
Laurence Campens ◽  
Julie De Zaeytijd ◽  
Bart Leroy ◽  
Dimitri Hemelsoet ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Diastolic Dysfunction ◽  
Clinical Practice Guidelines ◽  
Practice Guidelines ◽  
Clinical Manifestations ◽  
Ectopic Calcification ◽  
Imaging Data ◽  
Variable Expression ◽  
Increased Risk

BackgroundBiallelic pathogenic variants in the ATP-binding cassette subfamily C member 6 (ABCC6) gene cause pseudoxanthoma elasticum, a multisystemic ectopic calcification disorder, while heterozygous ABCC6 variants are associated with an increased risk of cardiovascular and cerebrovascular disease. As the prevalence of pathogenic ABCC6 variants in the general population is estimated at ~1%, identifying additional ABCC6-related (sub)clinical manifestations in heterozygous carriers is of the utmost importance to reduce this burden of disease. Here, we present a large Belgian cohort of heterozygous ABCC6 carriers with comprehensive clinical, biochemical and imaging data. Based on these results, we formulate clinical practice guidelines regarding screening, preventive measures and follow-up of ABCC6 carriers.MethodsThe phenotype of 56 individuals carrying heterozygous pathogenic ABCC6 variants was assessed using clinical (eg, detailed ophthalmological examinations), biochemical, imaging (eg, cardiovascular and abdominal ultrasound) and genetic data. Clinical practice guidelines were then drawn up.ResultsWe found that ABCC6 heterozygosity is associated with distinct retinal alterations (‘comet-like’) (24%), high prevalence of hypercholesterolaemia (>75%) and diastolic dysfunction (33%), accelerated lower limb atherosclerosis and medial vascular disease, abdominal organ calcification (26%) and testicular microlithiasis (28%), though with highly variable expression.ConclusionIn this study, we delineated the multisystemic ABCC6 heterozygosity phenotype characterised by retinal alterations, aberrant lipid metabolism, diastolic dysfunction and increased vascular, abdominal and testicular calcifications. Our clinical practice guidelines aimed to improve early diagnosis, treatment and follow-up of ABCC6-related health problems.

Clinical practice guidelines and recommendations for the management of patients with systemic lupus erythematosus: a critical comparison

Rheumatology ◽  
2020 ◽  
Vol 59 (12) ◽  
pp. 3690-3699 ◽  
Author(s):  
Margarida Oliveira ◽  
Sergio Palacios-Fernandez ◽  
Ricard Cervera ◽  
Gerard Espinosa
Keyword(s):  
Clinical Practice ◽  
Clinical Practice Guidelines ◽  
Practice Guidelines ◽  
Lupus Erythematosus ◽  
Clinical Judgment ◽  
Methodological Quality ◽  
Clinical Manifestations ◽  
High Quality ◽  
Clinical Patient ◽  
Quality Evidence

Abstract Objective SLE has a great clinical heterogeneity and low prevalence, thus making the development of recommendations or clinical practice guidelines (CPG) based on high-quality evidence difficult. In the last few years, several CPG appeared addressing the management of the disease. The aim of this review is to critically compare the recommendations made in the most recent CPG and to analyse and compare their methodological quality. Methods The Appraisal of Guidelines for Research and Evaluation (AGREE) II tool was used to compare the methodological quality of each of the CPG. Results Most CPG agreed in the general management and first-line treatment recommendations where there is higher quality evidence and disagreed in refractory disease treatment where there is lack of quality evidence. Also, the CPG are agreed in whether a patient should be treated regarding the most severe clinical manifestation or taking into account the treatment that best serves all clinical manifestations. The majority of the appraised CPG scored high-quality ratings, especially for scope and purpose and clarity of presentation, while they were of less quality when assessing applicability of each CPG. Conclusion CPG should aid, but not replace, the health professional’s clinical judgment in daily clinical patient management.

scholarly journals Systemic lupus erythematosus: state of the art on clinical practice guidelines

RMD Open ◽  
2018 ◽  
Vol 4 (Suppl 1) ◽  
pp. e000793 ◽  
Author(s):  
Farah Tamirou ◽  
Laurent Arnaud ◽  
Rosaria Talarico ◽  
Carlo Alberto Scirè ◽  
Tobias Alexander ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Practice ◽  
Disease Management ◽  
Clinical Practice Guidelines ◽  
Practice Guidelines ◽  
Lupus Erythematosus ◽  
Unmet Needs ◽  
Wide Spectrum ◽  
Clinical Manifestations ◽  
Systemic Lupus

Systemic lupus erythematosus (SLE) is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsing-remitting course. The aim of the present work was to identify current available clinical practice guidelines (CPGs) for SLE, to provide their review and to identify physicians’ and patients’ unmet needs. Twenty-three original guidelines published between 2004 and 2017 were identified. Many aspects of disease management are covered, including global disease management, lupus nephritis and neuropsychiatric involvement, management of pregnancies, vaccinations and comorbidities monitoring. Unmet needs relate with disease management of some clinical manifestations and adherence to treatment. Many patient’s unmet needs have been identified starting with faster diagnosis, need for more therapeutic options, guidelines on lifestyle issues, attention to quality of life and adequate education.

Clinical practice guidelines for the management of constipation in adults. Part 1: Definition, aetiology and clinical manifestations

2017 ◽  
Vol 40 (3) ◽  
pp. 132-141 ◽  
Author(s):  
Jordi Serra ◽  
Juanjo Mascort-Roca ◽  
Mercè Marzo-Castillejo ◽  
Silvia Delgado Aros ◽  
Juan Ferrándiz Santos ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Clinical Practice Guidelines ◽  
Practice Guidelines ◽  
Clinical Manifestations

High Flow Oxygen Therapy and the Pressure to Feed Infants With Acute Respiratory Illness

2020 ◽  
Vol 5 (4) ◽  
pp. 1006-1010
Author(s):  
Jennifer Raminick ◽  
Hema Desai
Keyword(s):  
Clinical Practice ◽  
Clinical Practice Guidelines ◽  
Practice Guidelines ◽  
Oxygen Therapy ◽  
Respiratory Illness ◽  
High Flow ◽  
Oral Feeding ◽  
Respiratory Support ◽  
Acute Respiratory Illness ◽  
High Flow Oxygen

Purpose Infants hospitalized for an acute respiratory illness often require the use of noninvasive respiratory support during the initial stage to improve their breathing. High flow oxygen therapy (HFOT) is becoming a more popular means of noninvasive respiratory support, often used to treat respiratory syncytial virus/bronchiolitis. These infants present with tachypnea and coughing, resulting in difficulties in coordinating sucking and swallowing. However, they are often allowed to feed orally despite having high respiratory rate, increased work of breathing and on HFOT, placing them at risk for aspiration. Feeding therapists who work with these infants have raised concerns that HFOT creates an additional risk factor for swallowing dysfunction, especially with infants who have compromised airways or other comorbidities. There is emerging literature concluding changes in pharyngeal pressures with HFOT, as well as aspiration in preterm neonates who are on nasal continuous positive airway pressure. However, there is no existing research exploring the effect of HFOT on swallowing in infants with acute respiratory illness. This discussion will present findings from literature on HFOT, oral feeding in the acutely ill infant population, and present clinical practice guidelines for safe feeding during critical care admission for acute respiratory illness. Conclusion Guidelines for safety of oral feeds for infants with acute respiratory illness on HFOT do not exist. However, providers and parents continue to want to provide oral feeds despite clinical signs of respiratory distress and coughing. To address this challenge, we initiated a process change to use clinical bedside evaluation and a “cross-systems approach” to provide recommendations for safer oral feeds while on HFOT as the infant is recovering from illness. Use of standardized feeding evaluation and protocol have improved consistency of practice within our department. However, further research is still necessary to develop clinical practice guidelines for safe oral feeding for infants on HFOT.

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