Search results for "Disease management"
showing 10 items of 168 documents
Recommendations for clinical monitoring of patients with acid sphingomyelinase deficiency (ASMD)
2018
Abstract Background Acid sphingomyelinase deficiency (ASMD), a rare lysosomal storage disease, results from mutations in SMPD1, the gene encoding acid sphingomyelinase (ASM). As a result, sphingomyelin accumulates in multiple organs including spleen, liver, lung, bone marrow, lymph nodes, and in the most severe form, in the CNS and peripheral nerves. Clinical manifestations range from rapidly progressive and fatal infantile neurovisceral disease, to less rapidly progressing chronic neurovisceral and visceral forms that are associated with significant morbidity and shorter life span due to respiratory or liver disease. Objectives To provide a contemporary guide of clinical assessments for di…
[Management of patients with lymphatic diseases and lipoedema during the COVID-19 pandemic. Recommendations of the Spanish Group of Lymphology].
2020
Resumen La pandemia de COVID-19 es un desafío para el manejo de las patologías no COVID como la enfermedad linfática y el lipedema. La Telemedicina puede evitar la propagación del coronavirus. Se necesita un sistema que nos ayude a determinar la prioridad clínica y la selección de la asistencia presencial o telemática para cada paciente y la forma de realizarlas durante la pandemia. El Grupo Español de Linfología (GEL) ha realizado un documento de consenso con recomendaciones basadas en la bibliografía y experiencia clínica, como guía de práctica clínica en el manejo de anomalías linfáticas y lipedema durante la pandemia de COVID-19. Estas recomendaciones deben adaptarse a las característic…
European ADPKD Forum multidisciplinary position statement on autosomal dominant polycystic kidney disease care
2018
Autosomal dominant polycystic kidney disease (ADPKD) is a chronic, progressive condition characterized by the development and growth of cysts in the kidneys and other organs and by additional systemic manifestations. Individuals with ADPKD should have access to lifelong, multidisciplinary, specialist and patient-centred care involving: (i) a holistic and comprehensive assessment of the manifestations, complications, prognosis and impact of the disease (in physical, psychological and social terms) on the patient and their family; (ii) access to treatment to relieve symptoms, manage complications, preserve kidney function, lower the risk of cardiovascular disease and maintain quality of life;…
Management of acute promyelocytic leukemia: updated recommendations from an expert panel of the European LeukemiaNet
2019
Abstract Since the comprehensive recommendations for the management of acute promyelocytic leukemia (APL) reported in 2009, several studies have provided important insights, particularly regarding the role of arsenic trioxide (ATO) in frontline therapy. Ten years later, a European LeukemiaNet expert panel has reviewed the recent advances in the management of APL in both frontline and relapse settings in order to develop updated evidence- and expert opinion–based recommendations on the management of this disease. Together with providing current indications on genetic diagnosis, modern risk-adapted frontline therapy, and salvage treatment, the review contains specific recommendations for the …
Redefining outcomes in immune TTP: an international working group consensus report
2021
Abstract Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a potentially fatal thrombotic microangiopathy caused by autoantibody-mediated severe deficiency of ADAMTS13. Standardized definitions of response, exacerbation, remission, and relapse were initially proposed in 2003 and modified by the International Working Group for TTP in 2017. These definitions, which have been widely used in clinical practice and research, are based primarily on the platelet count and are benchmarked against the timing of discontinuation of therapeutic plasma exchange (TPE). They do not incorporate ADAMTS13 activity or the temporizing effects on the platelet count of caplacizumab, a novel anti–von W…
Attitudes towards insulin initiation in type 2 diabetes patients among healthcare providers: A survey research
2016
Aims: To describe the views of healthcare providers about starting insulin in patients with type 2 diabetes and to determine the specific factors that contribute to delay insulin initiation. Methods: Two-phases observational descriptive study. In the quantitative phase we conducted a cross-sectional survey of a sample of 380 healthcare professionals (general practitioners (GPs), endocrinologists, internists and nurses). In the qualitative phase, a discussion group reviewed the results of the survey to propose solutions. Results: In poorly controlled patients, 46% of GPs vs. 43.2% of internists and 31.3% of endocrinologists waited 3-6 months before starting insulin, and 71.4% of GPs vs. 66.7…
Guidelines of the Italian societies of gastroenterology on the diagnosis and management of coeliac disease and dermatitis herpetiformis
2022
Introduction: Coeliac disease and dermatitis herpetiformis are immune-mediated diseases triggered by the consumption of gluten in genetically predisposed individuals. These guidelines were developed to provide general practitioners, paediatricians, gastroenterologists, and other clinicians with an overview on the diagnosis, management and follow-up of coeliac patients and those with dermatitis herpetiformis.Methods: Guidelines were developed by the Italian Societies of Gastroenterology. Following a systematic literature review, the Grading of Recommendations Assessment, Development and Evaluation methodol-ogy was used to assess the certainty of the evidence. Statements and recommendations w…
Prognosis after surgery for multiple endocrine neoplasia type 1-related pancreatic neuroendocrine tumors: Functionality matters
2021
Contains fulltext : 245221.pdf (Publisher’s version ) (Open Access) BACKGROUND: Metastasized pancreatic neuroendocrine tumors are the leading cause of death in patients with multiple endocrine neoplasia type 1. Aside from tumor size, prognostic factors of pancreatic neuroendocrine tumors are largely unknown. The present study aimed to assess whether the prognosis of patients with resected multiple endocrine neoplasia type 1-related nonfunctioning pancreatic neuroendocrine tumors differs from those with resected multiple endocrine neoplasia type 1-related insulinomas and assessed factors associated with prognosis. METHODS: Patients who underwent resection of a multiple endocrine neoplasia ty…
Conservative vs. Surgical Management of Post-Traumatic Epidural Hematoma: A Case and Review of Literature
2015
Patient: Male, 30 Final Diagnosis: Acute epidural hematoma Symptoms: — Medication: — Clinical Procedure: Observation Specialty: Neurosurgery Objective: Unusual clinical course Background: Trauma is the leading cause of death in people younger than 45 years and head injury is the main cause of trauma mortality. Although epidural hematomas are relatively uncommon (less than 1% of all patients with head injuries and fewer than 10% of those who are comatose), they should always be considered in evaluation of a serious head injury. Patients with epidural hematomas who meet surgical criteria and receive prompt surgical intervention can have an excellent prognosis, presumably owing to limited unde…
Addressing the impact of SARS-CoV-2 infection in persons with congenital bleeding disorders: The Italian MECCOVID-19 study
2021
congenital bleeding disorders