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Genetic Skin Diseases HELP
Based on 20,927 articles published since 2010

These are the 20927 published articles about Skin Diseases, Genetic that originated from Worldwide during 2010-2020.
+ Citations + Abstracts
Pages: 1 · 2 · 3 · 4 · 5 · 6 · 7 · 8 · 9 · 10 · 11 · 12 · 13 · 14 · 15 · 16 · 17 · 18 · 19 · 20
1 Guideline Consensus on the therapeutic management of atopic dermatitis - Brazilian Society of Dermatology. 2019

Aoki, Valeria / Lorenzini, Daniel / Orfali, Raquel Leão / Zaniboni, Mariana Colombini / Oliveira, Zilda Najjar Prado de / Rivitti-Machado, Maria Cecília / Takaoka, Roberto / Weber, Magda Blessmann / Cestari, Tania / Gontijo, Bernardo / Ramos, Andrea Machado Coelho / Silva, Claudia Marcia de Resende / Cestari, Silmara da Costa Pereira / Souto-Mayor, Silvia / Carneiro, Francisca Regina / Cerqueira, Ana Maria Mosca de / Laczynski, Cristina / Pires, Mario Cezar. ·Department of Dermatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil. · Dermatology Service, Irmandade Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, RS, Brazil. · Dermatology Service, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil. · Dermatology Service, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. · Dermatology Service, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil. · Medical Dermatology Residency Program, Instituto de Ensino e Pesquisa, Hospital Sírio-Libanês, São Paulo, SP, Brazil. · Clinic of Dermatology, Department of Medicine, Faculdade de Medicina da Santa Casa de São Paulo, São Paulo, SP, Brazil. · Dermatology Service, Universidade do Estado do Pará, Belém, PA, Brazil. · Dermatology Service, Hospital Municipal Jesus, Rio de Janeiro, RJ, Brazil. · Dermatology Outpatient Clinic, Faculdade de Medicina do ABC, Santo André, SP, Brazil. · Dermatology Service, Hospital do Servidor Público Estadual, São Paulo, SP, Brazil. ·An Bras Dermatol · Pubmed #31166406.

ABSTRACT: BACKGROUND: Atopic dermatitis is a highly prevalent inflammatory and pruritic dermatosis with a multifactorial etiology, which includes skin barrier defects, immune dysfunction, and microbiome alterations. Atopic dermatitis is mediated by genetic, environmental, and psychological factors and requires therapeutic management that covers all the aspects of its complex pathogenesis. OBJECTIVES: The aim of this article is to present the experience, opinions, and recommendations of Brazilian dermatology experts regarding the therapeutic management of atopic dermatitis. METHODS: Eighteen experts from 10 university hospitals with experience in atopic dermatitis were appointed by the Brazilian Society of Dermatology to organize a consensus on the therapeutic management of atopic dermatitis. The 18 experts answered an online questionnaire with 14 questions related to the treatment of atopic dermatitis. Afterwards, they analyzed the recent international guidelines on atopic dermatitis of the American Academy of Dermatology, published in 2014, and of the European Academy of Dermatology and Venereology, published in 2018. Consensus was defined as approval by at least 70% of the panel. RESULTS/CONCLUSION: The experts stated that the therapeutic management of atopic dermatitis is based on skin hydration, topical anti-inflammatory agents, avoidance of triggering factors, and educational programs. Systemic therapy, based on immunosuppressive agents, is only indicated for severe refractory disease and after failure of topical therapy. Early detection and treatment of secondary bacterial and viral infections is mandatory, and hospitalization may be needed to control atopic dermatitis flares. Novel target-oriented drugs such as immunobiologicals are invaluable therapeutic agents for atopic dermatitis.

2 Guideline Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology. 2019

Santi, Claudia Giuli / Gripp, Alexandre Carlos / Roselino, Ana Maria / Mello, Danielle Santana / Gordilho, Juliana Olivieri / Marsillac, Paula Figueiredo de / Porro, Adriana Maria. ·Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil. · Department of Dermatology, Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil. · Department of Medical Clinics, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brazil. · Department of Dermatology, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil. ·An Bras Dermatol · Pubmed #31166405.

ABSTRACT: Bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita are subepidermal autoimmune blistering diseases whose antigenic target is located at the basement membrane zone. Mucous membrane pemphigoid and epidermolysis bullosa acquisita can evolve with cicatricial mucosal involvement, leading to respiratory, ocular and/or digestive sequelae with important morbidity. For each of these dermatoses, a literature review covering all therapeutic options was performed. A flowchart, based on the experience and joint discussion among the authors of this consensus, was constructed to provide treatment orientation for these diseases in Brazil. In summary, in the localized, low-risk or non-severe forms, drugs that have immunomodulatory action such as dapsone, doxycycline among others may be a therapeutic option. Topical treatment with corticosteroids or immunomodulators may also be used. Systemic corticosteroid therapy continues to be the treatment of choice for severe forms, especially those involving ocular, laryngeal-pharyngeal and/or esophageal mucosal involvement, as may occur in mucous membrane pemphigoid and epidermolysis bullosa acquisita. Several immunosuppressants are used as adjuvant alternatives. In severe and recalcitrant cases, intravenous immunoglobulin is an alternative that, while expensive, may be used. Immunobiological drugs such as rituximab are promising drugs in this area. Omalizumab has been used in bullous pemphigoid.


Anonymous3780972. · ·Arerugi · Pubmed #30541970.

ABSTRACT: -- No abstract --

4 Guideline Consensus Recommendations on Adjunctive Topical Management of Atopic Dermatitis. 2018

Del Rosso, James Q / Harper, Julie / Kircik, Leon / Albon, Glynis / Berson, Diane / Hebert, Adelaide / Day, Doris. · ·J Drugs Dermatol · Pubmed #30365587.

ABSTRACT: Atopic dermatitis (AD) is well-recognized as a very common chronic and relapsing pruritic skin disorder affecting both children and adults worldwide. The adverse effects on the quality of life of affected individuals and their families is well-established. The pathophysiology of AD is complex, leading to interindividual variations in clinical presentation and severity. The chronicity of AD, characterized by periods of exacerbation and remission, supports a strong need to develop measures that can effectively and safely prolong remissions between flares of the disease. This article provides an overview of AD including prevalence, severity, and disease course/progression, succinct summaries of pathophysiology and medical management, and discussion of epidermal barrier dysfunction and skin microbiome shifting associated with AD. Additional emphasis is placed on adjunctive topical skin barrier approaches that may prolong disease-free remissions. Results from a panel of dermatologists queried about adjunctive approaches to AD, using a modified-Delphi approach, are also discussed. J Drugs Dermatol. 2018;17(10):1070-1076.






5 Guideline Oral propranolol in the treatment of proliferating infantile haemangiomas: British Society for Paediatric Dermatology consensus guidelines. 2018

Solman, L / Glover, M / Beattie, P E / Buckley, H / Clark, S / Gach, J E / Giardini, A / Helbling, I / Hewitt, R J / Laguda, B / Langan, S M / Martinez, A E / Murphy, R / Proudfoot, L / Ravenscroft, J / Shahidullah, H / Shaw, L / Syed, S B / Wells, L / Flohr, C. ·Department of Paediatric Dermatology, Great Ormond Street Hospital for Children, London, U.K. · Royal Hospital for Children, Glasgow, U.K. · Queen Alexandra Hospital, Portsmouth, U.K. · Chapel Allerton Hospital, Leeds Teaching Hospitals NHS Trust, Leeds, U.K. · Birmingham Children's Hospital, Birmingham, U.K. · Paediatric Cardiology Division, Great Ormond Street Hospital for Children, London, U.K. · Leicester Royal Infirmary, Leicester, U.K. · Department of Paediatric Otolaryngology, Great Ormond Street Hospital for Children, London, U.K. · Chelsea and Westminster Hospital, London, U.K. · London School of Hygiene and Tropical Medicine, London, U.K. · Sheffield Teaching Hospitals, Sheffield, U.K. · The Royal Free London Hospital, London, U.K. · Nottingham Children's Hospital, London, U.K. · Derby Hospitals, Derby, U.K. · Unit of Population-Based Dermatology Research and Department of Paediatric Dermatology, St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust and King's College London, London, U.K. ·Br J Dermatol · Pubmed #29774538.

ABSTRACT: BACKGROUND: Infantile haemangiomas (IH) are the most common vascular tumours of infancy. Despite their frequency and potential complications, there are currently no unified U.K. guidelines for the treatment of IH with propranolol. There are still uncertainties and diverse opinions regarding indications, pretreatment investigations, its use in PHACES (posterior fossa malformations-haemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe) syndrome and cessation of treatment. OBJECTIVES: To provide unified guidelines for the treatment of IH with propranolol. METHODS: This study used a modified Delphi technique, which involved an international treatment survey, a systematic evidence review of the literature, a face-to-face multidisciplinary panel meeting and anonymous voting. RESULTS: The expert panel achieved consensus on 47 statements in eight categories, including indications and contraindications for starting propranolol, pretreatment investigations, starting and target dose, monitoring of adverse effects, the use of propranolol in PHACES syndrome and how to stop treatment. CONCLUSIONS: These consensus guidelines will help to standardize and simplify the treatment of IH with oral propranolol across the U.K. and assist in clinical decision-making.

6 Guideline Consensus-based European guidelines for treatment of atopic eczema (atopic dermatitis) in adults and children: part I. 2018

Wollenberg, A / Barbarot, S / Bieber, T / Christen-Zaech, S / Deleuran, M / Fink-Wagner, A / Gieler, U / Girolomoni, G / Lau, S / Muraro, A / Czarnecka-Operacz, M / Schäfer, T / Schmid-Grendelmeier, P / Simon, D / Szalai, Z / Szepietowski, J C / Taïeb, A / Torrelo, A / Werfel, T / Ring, J / Anonymous1860944. ·Department Dermatology and Allergy, Ludwig-Maximilian University, Munich, Germany. · Klinik Thalkirchner Straße, Munich, Germany. · Department of Dermatology, Centre Hospitalier Universitaire CHU Nantes, Nantes, France. · Department of Dermatology and Allergy, Christine Kühne-Center for Allergy Research and Education, University Bonn, Bonn, Germany. · Pediatric Dermatology Unit, Departments of Dermatology and Pediatrics, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland. · Department Dermatology, Aarhus University Hospital, Aarhus, Denmark. · European Federation of Allergy and Airways Diseases Patients' Associations (EFA), Global Allergy and Asthma Patient Platform (GAAPP), Konstanz, Germany. · Department of Dermatology, University of Gießen and Marburg GmbH, Gießen, Germany. · Department of Psychosomatics and Psychotherapy, University of Gießen and Marburg GmbH, Gießen, Germany. · Department of Medicine, Section of Dermatology, University of Verona, Verona, Italy. · Pediatric Pneumology and Immunology, Universitätsmedizin Berlin, Berlin, Germany. · Centro di Specializzazione Regionale per lo Studio e la Cura delle Allergie e delle Intolleranze Alimentari presso l'Azienda Ospedaliera, Università di Padova, Padova, Italy. · Department of Dermatology, Medical University, Poznan, Poland. · Dermatological Practice, Immenstadt, Germany. · Allergy Unit, Department of Dermatology, University of Zurich, Zurich, Switzerland. · Christine Kühne Center for Allergy Research and Education CK-CARE, Davos, Switzerland. · Department Dermatology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland. · Department of Dermatology, Heim Pál Children's Hospital, Budapest, Hungary. · Department of Dermatology, Venereology and Allergology, Wroclaw Medical University, Wroclaw, Poland. · Department of Dermatology and Pediatric Dermatology, Hôpital St André, Bordeaux, France. · Department of Dermatology, Hospital Niño Jesus, Madrid, Spain. · Department Dermatology and Allergy, Hannover Medical School, Hannover, Germany. · Department Dermatology and Allergy Biederstein, Technische Universität München, Munich, Germany. ·J Eur Acad Dermatol Venereol · Pubmed #29676534.

ABSTRACT: This guideline was developed as a joint interdisciplinary European project, including physicians from all relevant disciplines as well as patients. It is a consensus-based guideline, taking available evidence from other guidelines, systematic reviews and published studies into account. This first part of the guideline covers methods, patient perspective, general measures and avoidance strategies, basic emollient treatment and bathing, dietary intervention, topical anti-inflammatory therapy, phototherapy and antipruritic therapy, whereas the second part covers antimicrobial therapy, systemic treatment, allergen-specific immunotherapy, complementary medicine, psychosomatic counselling and educational interventions. Management of AE must consider the individual clinical variability of the disease; highly standardized treatment rules are not recommended. Basic therapy is focused on treatment of disturbed barrier function by hydrating and lubricating topical treatment, besides further avoidance of specific and unspecific provocation factors. Topical anti-inflammatory treatment based on glucocorticosteroids and calcineurin inhibitors is used for flare management and for proactive therapy for long-term control. Topical corticosteroids remain the mainstay of therapy, whereas tacrolimus and pimecrolimus are preferred in sensitive skin areas and for long-term use. Topical phosphodiesterase inhibitors may be a treatment alternative when available. Adjuvant therapy includes UV irradiation, preferably with UVB 311 nm or UVA1. Pruritus is targeted with the majority of the recommended therapies, but some patients may need additional antipruritic therapy. Antimicrobial therapy, systemic anti-inflammatory treatment, immunotherapy, complementary medicine and educational intervention will be addressed in part II of the guideline.

7 Guideline 2018 update of the EULAR recommendations for the management of Behçet's syndrome. 2018

Hatemi, Gulen / Christensen, Robin / Bang, Dongsik / Bodaghi, Bahram / Celik, Aykut Ferhat / Fortune, Farida / Gaudric, Julien / Gul, Ahmet / Kötter, Ina / Leccese, Pietro / Mahr, Alfred / Moots, Robert / Ozguler, Yesim / Richter, Jutta / Saadoun, David / Salvarani, Carlo / Scuderi, Francesco / Sfikakis, Petros P / Siva, Aksel / Stanford, Miles / Tugal-Tutkun, Ilknur / West, Richard / Yurdakul, Sebahattin / Olivieri, Ignazio / Yazici, Hasan. ·Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey. · Musculoskeletal Statistics Unit, The Parker Institute, Bispebjerg and Frederiksberg Hospital & Department of Rheumatology, Odense University Hospital, Copenhagen, Denmark. · Department of Dermatology, Catholic Kwandong University International St. Mary's Hospital, Incheon, Korea. · Department of Ophthalmology, Pitié-Salpêtrière Hospital, Sorbonne University, Paris, France. · Division of Gastroenterology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey. · Centre for Clinical and Diagnostic Oral Sciences, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, and the London Behçet's Centre, Barts Health London, London, UK. · Department of Vascular Surgery, Pitié-Salpêtrière Hospital, Sorbonne University, Paris, France. · Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey. · Department of Rheumatology, Immunology and Nephrology, Asklepios Clinic Altona, Hamburg, Germany. · Rheumatology Institute of Lucania (IRel) and the Rheumatology Department of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, Potenza and Matera, Italy. · Department of Internal Medicine, Hospital Saint-Louis, Paris, France. · National Behcet's Syndrome Centre of Excellence, Aintree University Hospital, Liverpool, UK. · Institute for Haematopathology Hamburg, Hamburg, Germany. · Department of Inflammation-Immunopathology-Biotherapy, Sorbonne Universités, UPMC Univ Paris 06, Paris, France. · INSERM, Paris, France. · CNRS, Paris, France. · Department of Internal Medicine and Clinical Immunology, Centre de Référence des Maladies Auto-Immunes et Systémiques Rares, Centre de Référence des Maladies Auto-Inflammatoires, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France. · Division of Rheumatology, Azienda USL-IRCCS di Reggio Emilia, University of Modena and Reggio Emilia, Modena and Reggio Emilia, Italy. · Patient Research Partner, Catania, Italy. · First Department of Propaedeutic and Internal Medicine & Rheumatology Unit, National Kapodistrian University of Athens Medical School, Athens, Greece. · Department of Neurology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey. · Department of Ophthalmology, St. Thomas' Hospital, London, UK. · Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey. · Patient Research Partner, Member of the UK Behcet's Syndrome Society and Director of Behcets International, London, UK. · Rheumatology Institute of Lucania (IRel) and the Rheumatology Department of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, and the Basilicata Ricerca Biomedica (BRB) Foundation, Potenza and Matera, Italy. ·Ann Rheum Dis · Pubmed #29625968.

ABSTRACT: Several new treatment modalities with different mechanisms of action have been studied in patients with Behçet's syndrome (BS). The aim of the current effort was to update the recommendations in the light of these new data under the auspices of the European League Against Rheumatism (EULAR) Standing Committee for Clinical Affairs. A task force was formed that included BS experts from different specialties including internal medicine, rheumatology, ophthalmology, dermatology, neurology, gastroenterology, oral health medicine and vascular surgery, along with a methodologist, a health professional, two patients and two fellows in charge of the systematic literature search. Research questions were determined using a Delphi approach. EULAR standardised operating procedures was used as the framework. Results of the systematic literature review were presented to the task force during a meeting. The former recommendations were modified or new recommendations were formed after thorough discussions followed by voting. The recommendations on the medical management of mucocutaneous, joint, eye, vascular, neurological and gastrointestinal involvement of BS were modified; five overarching principles and a new recommendation about the surgical management of vascular involvement were added. These updated, evidence-based recommendations are intended to help physicians caring for patients with BS. They also attempt to highlight the shortcomings of the available clinical research with the aim of proposing an agenda for further research priorities.

8 Guideline Diagnosis and management of moderate to severe adult atopic dermatitis: a Consensus by the Italian Society of Dermatology and Venereology (SIDeMaST), the Italian Association of Hospital Dermatologists (ADOI), the Italian Society of Allergy, Asthma and Clinical Immunology (SIAAIC), and the Italian Society of Allergological, Environmental and Occupational Dermatology (SIDAPA). 2018

Calzavara Pinton, Piergiacomo / Cristaudo, Antonio / Foti, Caterina / Canonica, Giorgio W / Balato, Nicola / Costanzo, Antonio / DE Pità, Ornella / DE Simone, Clara / Patruno, Cataldo / Pellacani, Giovanni / Peris, Ketty / Girolomoni, Giampiero. ·Department of Dermatology, Spedali Civili of Brescia, University of Brescia, Brescia, Italy - piergiacomo.calzavarapinton@unibs.it. · San Gallicano Dermatological Institute, IRCCS, Rome, Italy. · Unit of Dermatology, Department of Biomedical Science and Human Oncology, University of Bari, Bari, Italy. · Personalized Medicine Asthma & Allergy Clinic, Humanitas University, Clinical and Research Hospital, Rozzano, Milan, Italy. · Section of Dermatology, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy: 6 Unit of Dermatology, Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, Italy. · Unit of Dermatology, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy. · Clinical Pathology, Cristo Re Hospital, Rome, Italy. · Department of Dermatology, Sacro Cuore Catholic University, Rome, Italy. · Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy. · Section of Dermatology, Department of Medicine, University of Verona, Verona, Italy. ·G Ital Dermatol Venereol · Pubmed #29237258.

ABSTRACT: Atopic dermatitis (AD) is a chronic immune-mediated inflammatory skin disease, currently recognized as a systemic disease possibly burdened by various comorbidities, including, but not limited to, other allergic conditions. Management guidelines issued by American and European dermatology and allergy scientific societies are available. However, some discrepancies exist in these guidelines, and some aspects of the management process, including diagnosis and severity assessment, as well as therapy duration and switch criteria, are not fully clarified by existing guidelines. Moreover, biologics such as dupilumab have now entered the therapeutic scenario of moderate-to-severe AD, offering a great opportunity to treat effectively and safely in need AD patients. For all these reasons, four Italian dermatology and allergy scientific societies joined to provide practical guidance for the management of moderate-to-severe adult AD suitable for the Italian clinical practice. Through a modified Delphi procedure, consensus was reached by 63 Italian dermatologists and allergists experienced in the management of adult AD on 14 statements covering five AD areas of interest, i.e. diagnosis, severity definition, current systemic therapies, eligibility criteria to biologic treatments, and comorbidities, with the aim to define treatment goals and improve adult AD management. The potential usefulness of a multidisciplinary approach is also underlined, given the complexity of AD and its comorbidities.

9 Guideline Xeroderma pigmentosum clinical practice guidelines. 2017

Moriwaki, Shinichi / Kanda, Fumio / Hayashi, Masaharu / Yamashita, Daisuke / Sakai, Yoshitada / Nishigori, Chikako / Anonymous6930914. ·Department of Dermatology, Osaka Medical College, Takatsuki, Japan. · Division of Neurology, Kobe University Graduate School of Medicine, Kobe, Japan. · Department of Brain Development and Neural Regeneration, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan. · Division of Otolaryngology-Head and Neck Surgery, Kobe University Graduate School of Medicine, Kobe, Japan. · Division of Rehabilitation Medicine, Kobe University Graduate School of Medicine, Kobe, Japan. · Division of Dermatology, Kobe University Graduate School of Medicine (Chairperson at Xeroderma pigmentosum clinical practice guidelines revision committee), Kobe, Japan. ·J Dermatol · Pubmed #28771907.

ABSTRACT: Xeroderma pigmentosum (XP) is a genetic photosensitive disorder in which patients are highly susceptibe to skin cancers on the sun-exposed body sites. In Japan, more than half of patients (30% worldwide) with XP show complications of idiopathic progressive, intractable neurological symptoms with poor prognoses. Therefore, this disease does not merely present with dermatological symptoms, such as photosensitivity, pigmentary change and skin cancers, but is "an intractable neurological and dermatological disease". For this reason, in March 2007, the Japanese Ministry of Health, Labor and Welfare added XP to the neurocutaneous syndromes that are subject to government research initiatives for overcoming intractable diseases. XP is one of the extremely serious photosensitive disorders in which patients easily develop multiple skin cancers if they are not completely protected from ultraviolet radiation. XP patients thus need to be strictly shielded from sunlight throughout their lives, and they often experience idiopathic neurodegenerative complications that markedly reduce the quality of life for both the patients and their families. Hospitals in Japan often see cases of XP as severely photosensitive in children, and as advanced pigmentary disorders of the sun-exposed area with multiple skin cancers in adults (aged in their 20-40s), making XP an important disease to differentiate in everyday clinical practice. It was thus decided that there was a strong need for clinical practice guidelines dedicated to XP. This process led to the creation of new clinical practice guidelines for XP.

10 Guideline Late Effects Screening Guidelines after Hematopoietic Cell Transplantation for Inherited Bone Marrow Failure Syndromes: Consensus Statement From the Second Pediatric Blood and Marrow Transplant Consortium International Conference on Late Effects After Pediatric HCT. 2017

Dietz, Andrew C / Savage, Sharon A / Vlachos, Adrianna / Mehta, Parinda A / Bresters, Dorine / Tolar, Jakub / Bonfim, Carmem / Dalle, Jean Hugues / de la Fuente, Josu / Skinner, Roderick / Boulad, Farid / Duncan, Christine N / Baker, K Scott / Pulsipher, Michael A / Lipton, Jeffrey M / Wagner, John E / Alter, Blanche P. ·Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, University of Southern California, Los Angeles, California. Electronic address: adietz@chla.usc.edu. · Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, Maryland. · Division of Hematology/Oncology and Stem Cell Transplantation, Hofstra Northwell School of Medicine, Feinstein Institute for Medical Research, Cohen Children's Medical Center, New Hyde Park, New York. · Division of Bone Marrow Transplantation and Immune Deficiency, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio. · Willem-Alexander Children's Hospital, SCT Unit, Leiden University Medical Center, Leiden, The Netherlands. · Blood and Marrow Transplant Program, University of Minnesota, Minneapolis, Minnesota. · Hospital de Clinicas, Federal University of Parana, Curitiba, Brazil. · Service d'hémato-immunologie,Université Paris 7, Hôpital Robert-Debré, Paris, France. · Section of Paediatrics, Department of Paediatric Haematology, St Mary's Hospital, Imperial College Healthcare NHS Trust, London, United Kingdom. · Great North Children's Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust and Northern Institute of Cancer Research, Newcastle University, Newcastle upon Tyne, United Kingdom. · Bone Marrow Transplant Service, Department of Pediatrics, Memorial Sloan Kettering Cancer Center, Division of Pediatric Hematology/Oncology, New York Presbyterian Hospital, Weill Cornell Medical College, New York, New York. · Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, Massachusetts. · Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, Washington. · Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, University of Southern California, Los Angeles, California. ·Biol Blood Marrow Transplant · Pubmed #28533057.

ABSTRACT: Patients with inherited bone marrow failure syndromes (IBMFS), such as Fanconi anemia (FA), dyskeratosis congenita (DC), or Diamond Blackfan anemia (DBA), can have hematologic manifestations cured through hematopoietic cell transplantation (HCT). Subsequent late effects seen in these patients arise from a combination of the underlying disease, the pre-HCT therapy, and the HCT process. During the international consensus conference sponsored by the Pediatric Blood and Marrow Transplant Consortium on late effects screening and recommendations following allogeneic hematopoietic cell transplantation for immune deficiency and nonmalignant hematologic diseases held in Minneapolis, Minnesota in May 2016, a half-day session was focused specifically on the unmet needs for these patients with IBMFS. This multidisciplinary group of experts in rare diseases and transplantation late effects has already published on the state of the science in this area, along with discussion of an agenda for future research. This companion article outlines consensus disease-specific long-term follow-up screening guidelines for patients with IMBFS.

11 Guideline Quality of life measurement in atopic dermatitis. Position paper of the European Academy of Dermatology and Venereology (EADV) Task Force on quality of life. 2017

Chernyshov, P V / Tomas-Aragones, L / Manolache, L / Marron, S E / Salek, M S / Poot, F / Oranje, A P / Finlay, A Y / Anonymous2600888. ·Department of Dermatology and Venereology, National Medical University, Kiev, Ukraine. · Aragon Health Sciences Institute, University of Zaragoza, Zaragoza, Spain. · Dermatology, Dali Medical, Bucharest, Romania. · Department of Dermatology, Aragon Health Sciences Institute (IACS), Alcañiz Hospital, Alcañiz, Spain. · University of Hertfordshire, Hatfield, UK. · Department of Dermatology, ULB-Erasme Hospital, Brussels, Belgium. · Dermicis Skin Hospital, Alkmaar, and (Kinder)huid, Rotterdam, The Netherlands. · Division of Infection and Immunity, Department of Dermatology and Wound Healing, School of Medicine, Cardiff University, Cardiff, UK. ·J Eur Acad Dermatol Venereol · Pubmed #27879008.

ABSTRACT: There is a need for researchers to have easy reference to the wide spectrum of different types of quality of life (QoL) instruments that can be used in atopic dermatitis (AD). Previous reviews on QoL in AD do not cover the full spectrum of QoL measures used in studies on AD. This study, on behalf of the European Academy of Dermatology and Venereology (EADV) Task Force on QoL, contains information on instruments available for health-related QoL and family QoL assessment in AD including information on validation, experience of QoL assessment in AD for different purposes, peculiarities of QoL assessment in different age groups, expert analysis of available instruments including data on limitations of their use and recommendations of the Task Force.

12 Guideline Clinical Practice Guidelines for the Management of Atopic Dermatitis 2016. 2016

Saeki, Hidehisa / Nakahara, Takeshi / Tanaka, Akio / Kabashima, Kenji / Sugaya, Makoto / Murota, Hiroyuki / Ebihara, Tamotsu / Kataoka, Yoko / Aihara, Michiko / Etoh, Takafumi / Katoh, Norito / Anonymous5010864. ·Department of Dermatology, Graduate School of Medicine, Nihon Medical School, Tokyo, Japan. · Division of Skin Surface Sensing, Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. · Department of Dermatology, Hiroshima University Graduate School of Biomedical Sciences, Hiroshima, Japan. · Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan. · Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. · Department of Dermatology, Graduate School of Medicine, Osaka University, Suita, Japan. · Department of Dermatology, Keio University School of Medicine, Tokyo, Japan. · Department of Dermatology, Osaka Prefectural Medical Center for Respiratory and Allergic Diseases, Habikino, Japan. · Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, Kanagawa, Japan. · Division of Dermatology, Tokyo Teishin Postal Services Agency Hospital, Tokyo, Japan. · Department of Dermatology, Kyoto Prefectural University of Medicine Graduate School of Medical Science, Kyoto, Japan. nkatoh@koto.kpu-m.ac.jp. ·J Dermatol · Pubmed #27076388.

ABSTRACT: Atopic dermatitis (AD) is a disease characterized by relapsing eczema with pruritus as a primary lesion. Most patients have an atopic predisposition. The definitive diagnosis of AD requires the presence of all three features: (i) pruritus; (ii) typical morphology and distribution of the eczema; and (iii) chronic and chronically relapsing course. The current strategies to treat AD in Japan from the perspective of evidence-based medicine consist of three primary measures: (i) the use of topical corticosteroids and tacrolimus ointment as the main treatment for the inflammation; (ii) topical application of emollients to treat the cutaneous barrier dysfunction; and (iii) avoidance of apparent exacerbating factors, psychological counseling and advice about daily life. The guidelines present recommendations to review clinical research articles, evaluate the balance between the advantages and disadvantages of medical activities, and optimize medical activity-related patient outcomes with respect to several important points requiring decision-making in clinical practice.

13 Guideline ETFAD/EADV Eczema task force 2015 position paper on diagnosis and treatment of atopic dermatitis in adult and paediatric patients. 2016

Wollenberg, A / Oranje, A / Deleuran, M / Simon, D / Szalai, Z / Kunz, B / Svensson, A / Barbarot, S / von Kobyletzki, L / Taieb, A / de Bruin-Weller, M / Werfel, T / Trzeciak, M / Vestergard, C / Ring, J / Darsow, U / Anonymous2730862. ·Department of Dermatology and Allergy, Ludwig-Maximilian-University, Munich, Germany. · Department of Dermatology (Pediatric Dermatology and Hair), Dermicis Skin clinic, Alkmaar and Rotterdam, The Netherlands. · Department of Dermatology, Aarhus University Hospital, Aarhus, Denmark. · Department of Dermatology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland. · Heim Pal Childrens Hospital, Budapest, Hungary. · Dermatologikum, Hamburg, Germany. · Department of Dermatology, University Hospital UMAS, Malmö, Sweden. · Department of Dermatology, University of Nantes, Nantes, France. · Service de Dermatologie et Dermatologie Pédiatrique, Centre de référence pour les maladies rares de la peau, INSERM 1035, Université de Bordeaux, Talence, France. · Department of Dermatology, University of Utrecht, Utrecht, The Netherlands. · Department of Dermatology, Hautklinik Linden, MHH, Hannover, Germany. · Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Gdansk, Poland. · Department of Dermatology and Allergy Biederstein, Technische Universität München, Munich, Germany. · Christine Kühne Center for Allergy Research and Education (CK-CARE), Davos, Switzerland. · ZAUM - Center of Allergy & Environment, Munich, Germany. ·J Eur Acad Dermatol Venereol · Pubmed #27004560.

ABSTRACT: Atopic dermatitis (AD) is a clinically defined, highly pruritic, chronic inflammatory skin disease of children and adults. The diagnosis is made using evaluated clinical criteria. Disease activity is best measured with a composite score assessing both objective signs and subjective symptoms, such as SCORAD. The management of AD must consider the clinical and pathogenic variabilities of the disease and also target flare prevention. Basic therapy includes hydrating topical treatment, as well as avoidance of specific and unspecific provocation factors. Anti-inflammatory treatment of visible skin lesions is based on topical glucocorticosteroids and the topical calcineurin inhibitors tacrolimus and pimecrolimus. Topical calcineurin inhibitors are preferred in sensitive locations. Tacrolimus and mid-potent steroids are proven for proactive therapy, which is long-term intermittent anti-inflammatory therapy of the frequently relapsing skin areas. Systemic anti-inflammatory or immunosuppressive treatment is indicated for severe refractory cases. Biologicals targeting key mechanisms of the atopic immune response are promising emerging treatment options. Microbial colonization and superinfection may induce disease exacerbation and can justify additional antimicrobial treatment. Systemic antihistamines (H1R-blockers) may diminish pruritus, but do not have sufficient effect on lesions. Adjuvant therapy includes UV irradiation, preferably UVA1 or narrow-band UVB 311 nm. Dietary recommendations should be patient specific and elimination diets should only be advised in case of proven food allergy. Allergen-specific immunotherapy to aeroallergens may be useful in selected cases. Psychosomatic counselling is recommended to address stress-induced exacerbations. 'Eczema school' educational programmes have been proven to be helpful for children and adults.

14 Guideline [Not Available]. 2016

Werfel, Thomas / Aberer, Werner / Ahrens, Frank / Augustin, Matthias / Biedermann, Tilo / Diepgen, Thomas / Fölster-Holst, Regina / Gieler, Uwe / Heratizadeh, Annice / Kahle, Julia / Kapp, Alexander / Nast, Alexander / Nemat, Katja / Ott, Hagen / Przybilla, Bernhard / Roecken, Martin / Schlaeger, Martin / Schmid-Grendelmeier, Peter / Schmitt, Jochen / Schwennesen, Thomas / Staab, Doris / Worm, Margitta / Anonymous2460853. ·Deutsche Dermatologische Gesellschaft. · Österreichische Gesellschaft für Dermatologie und Venerologie. · Gesellschaft für Pädiatrische Allergologie und Umweltmedizin e.V. · Arbeitsgemeinschaft Gesundheitsökonomie und Evidenzbasierte Medizin der Deutschen Dermatologischen Gesellschaft. · Arbeitsgemeinschaft Dermatologische Forschung. · Arbeitsgemeinschaft Berufs- und Umweltdermatologie der Deutschen Dermatologischen Gesellschaft. · Arbeitsgemeinschaft Pädiatrische Dermatologie der Deutschen Dermatologischen Gesellschaft. · Deutsche Gesellschaft PM, APD. · Wissenschaftliche Dokumentation und Redaktion. · Deutscher Allergie- und Asthmabund e.V. · Deutsche Gesellschaft für Allergologie und Klinische Immunologie. · Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e.V. (Moderation). · Berufsverband der Kinder- und Jugendärzte e.V. · Deutsche Gesellschaft für Kinder- und Jugendmedizin e.V. · Arbeitsgemeinschaft Allergologie der Deutschen Dermatologischen Gesellschaft. · Berufsverband Deutscher Dermatologen e.V. · Schweizerische Gesellschaft für Dermatologie und Venerologie. · Deutsches Netzwerk Versorgungsforschung e.V. · Deutscher Neurodermitis Bund e.V. · Arbeitsgemeinschaft Neurodermitisschulung e.V. · Deutsche Kontaktallergiegruppe e.V. ·J Dtsch Dermatol Ges · Pubmed #26713660.

ABSTRACT: -- No abstract --

15 Guideline [Recommendations for the management of the child with allergic diseases at school]. 2015

Anonymous6430830 / Saranz, Ricardo J / Lozano, Alejandro / Mariño, Andrea / Boudet, Raúl V / Sarraquigne, María Paula / Cáceres, María Elena / Bandín, Gloria / Lukin, Alicia / Skrie, Víctor / Cassaniti, María Cristina / Agüero, Claudio / Chorny, Marta / Reichbach, Débora S / Arnolt, Roque Gustavo / Cavallo, Aldo. · ·Arch Argent Pediatr · Pubmed #25996328.

ABSTRACT: Allergic diseases cause great impact on the health related quality of life in children and adolescents, resulting in increased school absenteeism and deficiencies in school performance. Although the bibliographic framework on allergic diseases is wide, in our country, there are no guidelines for proper management of the allergic child at school. It is necessary to establish guidelines for coordinated action among the educational community, the families, the pediatrician, the health team and governmental and non-governmental authorities. This position paper aims to provide information about the impact of allergic diseases on school activities, establish standards of competence of the various stakeholders at school and consider the legal framework for the intervention of the school staff about the child with allergies at school.

16 Guideline Phototherapy for atopic dermatitis. 2015

Dogra, Sunil / Mahajan, Rahul / Anonymous1560817. ·Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. ·Indian J Dermatol Venereol Leprol · Pubmed #25566890.

ABSTRACT: BACKGROUND: The aim of these guidelines is to review the available published literature regarding the effectiveness of phototherapy and photochemotherapy in atopic dermatitis and put forward recommendations regarding their use in atopic dermatitis. MATERIALS AND METHODS: A literature search was performed to collect data from PubMed, EMBASE, and the Cochrane Library published till March 2014. Keywords used were "phototherapy", "photochemotherapy", "NB-UVB", "BBUVB", "PUVA", "UVA1", "atopic dermatitis", and "atopic eczema". Systematic reviews, meta-analysis, national guidelines, randomized controlled trials, prospective open label studies, and retrospective case series in English literature mentioning use of above-mentioned keywords were reviewed. RESULTS: Six hundred and eighty eight studies were evaluated, 38 of which fulfilled the criteria for inclusion in the guidelines. CONCLUSIONS AND RECOMMENDATIONS: Both UV1 and narrow-band UVB are effective in significantly decreasing the eczema severity although UV1 may be preferred in acute flares and narrow-band UVB in chronic eczema, especially in adults (Level of evidence 1+, Grade of recommendation A). Among various doses of UVA1, medium dose UVA1 may be preferred over others as its efficacy is similar to high dose and better than low dose UVA1 phototherapy. Narrow-band UVB is preferred to broad-band UVB (Level of evidence 1+, Grade of recommendation A). Medium-dose UVA1 is similar in efficacy to narrow-band UVB (Level of evidence 1+, Grade of recommendation A). In children, despite its efficacy, narrow-band UVB phototherapy should be used only as a second line therapy due to its potential for long-term adverse effects (Level of evidence 2+, Grade of recommendation B).

17 Guideline Pain care for patients with epidermolysis bullosa: best care practice guidelines. 2014

Goldschneider, Kenneth R / Good, Julie / Harrop, Emily / Liossi, Christina / Lynch-Jordan, Anne / Martinez, Anna E / Maxwell, Lynne G / Stanko-Lopp, Danette / Anonymous2590818. ·Pain Management Center, Department of Anesthesiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. Kenneth.goldschneider@cchmc.org. · Lucille Packard Children's Hospital, Department of Anesthesia (by courtesy, Pediatrics), Stanford University, Stanford, California, USA. julieg@stanford.edu. · Helen and Douglas Hospices, Oxford and John Radcliffe Hospital, Oxford, USA. emilyharrop@yahoo.com. · University of Southampton, Southampton, UK. Christina.Liossi@gosh.nhs.uk. · Great Ormond Street Hospital for Children NHS Trust, London, UK. Christina.Liossi@gosh.nhs.uk. · Pain Management Center and Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. Anne.Lynch-Jordan@cchmc.org. · National Paediatric Epidermolysis Bullosa Centre, Great Ormond Street Hospital NHS Foundation Trust, London, UK. Anna.Martinez@gosh.nhs.uk. · Department of Anesthesiology and Critical Care, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. MAXWELL@email.chop.edu. · James M. Anderson Center for Health Systems Excellence, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. Danette.Stanko@cchmc.org. ·BMC Med · Pubmed #25603875.

ABSTRACT: BACKGROUND: Inherited epidermolysis bullosa (EB) comprises a group of rare disorders that have multi-system effects and patients present with a number of both acute and chronic pain care needs. Effects on quality of life are substantial. Pain and itching are burdensome daily problems. Experience with, and knowledge of, the best pain and itch care for these patients is minimal. Evidence-based best care practice guidelines are needed to establish a base of knowledge and practice for practitioners of many disciplines to improve the quality of life for both adult and pediatric patients with EB. METHODS: The process was begun at the request of Dystrophic Epidermolysis Bullosa Research Association International (DEBRA International), an organization dedicated to improvement of care, research and dissemination of knowledge for EB patients worldwide. An international panel of experts in pain and palliative care who have extensive experience caring for patients with EB was assembled. Literature was reviewed and systematically evaluated. For areas of care without direct evidence, clinically relevant literature was assessed, and rounds of consensus building were conducted. The process involved a face-to-face consensus meeting that involved a family representative and methodologist, as well as the panel of clinical experts. During development, EB family input was obtained and the document was reviewed by a wide variety of experts representing several disciplines related to the care of patients with EB. RESULTS: The first evidence-based care guidelines for the care of pain in EB were produced. The guidelines are clinically relevant for care of patients of all subtypes and ages, and apply to practitioners of all disciplines involved in the care of patients with EB. When the evidence suggests that the diagnosis or treatment of painful conditions differs between adults and children, it will be so noted. CONCLUSIONS: Evidence-based care guidelines are a means of standardizing optimal care for EB patients, whose disease is often times horrific in its effects on quality of life, and whose care is resource-intensive and difficult. The guideline development process also highlighted areas for research in order to improve further the evidence base for future care.

18 Guideline Consensus on the use of cyclosporine in dermatological practice. Italian Consensus Conference. 2014

Altomare, G / Ayala, F / Bardazzi, F / Bellia, G / Chimenti, S / Colombo, D / Flori, M L / Girolomoni, G / Micali, G / Parodi, A / Peris, K / Vena, G A / Anonymous1430806. ·IRCCS Galeazzi, University of Milan, Milan, Italy - giampiero.girolomoni@univr.it. ·G Ital Dermatol Venereol · Pubmed #25213388.

ABSTRACT: Cyclosporine A (CsA) efficacy and safety have been proven in various dermatoses both in adults and in children even as long-term treatment. Over the last 25 years, Italian dermatologists have gathered relevant experience about CsA treatment for psoriasis and atopic dermatitis. This paper has been developed by an Italian Consensus Conference and it is aimed at providing recommendations based on real-world clinical experience in adult patients, consistent with efficacy and safety data arising from the scientific literature. The paper is mainly focused on the analysis of the optimal therapeutic schemes for psoriasis and atopic dermatitis, in terms of doses and treatment duration, according to individual characteristics and to the severity of the disease. Moreover, it overviews ideal management, taking into account pharmacological interactions, influence of comorbidities, and the most common adverse events related to CsA treatment.

19 Guideline Japanese Guideline for Atopic Dermatitis 2014. 2014

Katayama, Ichiro / Kohno, Yoichi / Akiyama, Kazuo / Aihara, Michiko / Kondo, Naomi / Saeki, Hidehisa / Shoji, Shunsuke / Yamada, Hidekazu / Nakamura, Koichiro / Anonymous1040805. ·Department of Dermatology, Course of Integrated Medicine, Graduate School of Medicine, Osaka University, Osaka, Japan. · Chiba Rosai Hospital, Chiba, Japan. · National Hospital Organization, Sagamihara National Hospital, Kanagawa, Japan. · Department of Dermatology, Yokohama City University, Kanagawa, Japan. · Department of Pediatrics, Graduate School of Medicine, Gifu University, Gifu, Japan. · Department of Dermatology, Nippon Medical School, Tokyo, Japan. · National Hospital Organization, Tokyo National Hospital, Tokyo, Japan. · Department of Dermatology, Nara Hospital Kinki University Faculty of Medicine, Nara, Japan. · Department of Dermatology, Saitama Medical University, Saitama, Japan. ·Allergol Int · Pubmed #25178178.

ABSTRACT: Given the importance of appropriate diagnosis and appropriate assessment of cutaneous symptoms in treatment of atopic dermatitis, the basics of treatment in this guideline are composed of (1) investigation and countermeasures of causes and exacerbating factors, (2) correction of skin dysfunctions (skin care), and (3) pharmacotherapy, as three mainstays. These are based on the disease concept that atopic dermatitis is a inflammatory cutaneous disease with eczema by atopic diathesis, multi-factorial in onset and aggravation, and accompanied by skin dysfunctions. These three points are equally important and should be appropriately combined in accordance with the symptoms of each patient. In treatment, it is important to transmit the etiological, pathological, physiological, or therapeutic information to the patient to build a favorable partnership with the patient or his/her family so that they may fully understand the treatment. This guideline discusses chiefly the basic therapy in relation to the treatment of this disease. The goal of treatment is to enable patients to lead an uninterrupted social life and to control their cutaneous symptoms so that their quality of life (QOL) may meet a satisfactory level. The basics of treatment discussed in this guideline are based on the "Guidelines for the Treatment of Atopic Dermatitis 2008" prepared by the Health and Labour Sciences Research and the "Guidelines for the Management of Atopic Dermatitis 2012 (ADGL2012)" prepared by the Atopic Dermatitis Guidelines Advisory Committee, Japanese Society of Allergology in principle. The guidelines for the treatment of atopic dermatitis are summarized in the "Japanese Guideline for the Diagnosis and Treatment of Allergic Disease 2013" together with those for other allergic diseases.

20 Guideline Canadian Cardiovascular Society position statement on the management of thoracic aortic disease. 2014

Boodhwani, Munir / Andelfinger, Gregor / Leipsic, Jonathon / Lindsay, Thomas / McMurtry, M Sean / Therrien, Judith / Siu, Samuel C / Anonymous6930795. ·Division of Cardiac Surgery, University of Ottawa Heart Institute, Ottawa, Ontario, Canada. Electronic address: mboodhwani@ottawaheart.ca. · Department of Pediatrics, University of Montreal, Montreal, Québec, Canada. · Department of Radiology, University of British Colombia, Vancouver, British Colombia, Canada. · Division of Vascular Surgery, University Health Network, Toronto, Ontario, Canada. · Division of Cardiology, University of Alberta, Edmonton, Alberta, Canada. · Division of Cardiology, McGill University, Montreal, Québec, Canada. · Division of Cardiology, Western University, London, Ontario, Canada. ·Can J Cardiol · Pubmed #24882528.

ABSTRACT: This Canadian Cardiovascular Society position statement aims to provide succinct perspectives on key issues in the management of thoracic aortic disease (TAD). This document is not a comprehensive overview of TAD and important elements of the epidemiology, presentation, diagnosis, and management of acute aortic syndromes are deliberately not discussed; readers are referred to the 2010 guidelines published by the American Heart Association, American College of Cardiology, American Association for Thoracic Surgery, and other stakeholders. Rather, this document is a practical guide for clinicians managing adult patients with TAD. Topics covered include size thresholds for surgical intervention, emerging therapies, imaging modalities, medical and lifestyle management, and genetics of TAD. The primary panel consisted of experts from a variety of disciplines that are essential for comprehensive management of TAD patients. The methodology involved a focused literature review with an emphasis on updates since 2010 and the use of Grading of Recommendations Assessment, Development, and Evaluation methodology to arrive at specific recommendations. The final document then underwent review by a secondary panel. This document aims to provide recommendations for most patients and situations. However, the ultimate judgement regarding the management of any individual patients should be made by their health care team.

21 Guideline [Atopic dermatitis: national consensus 2013]. 2014

Anonymous2670795. · ·Arch Argent Pediatr · Pubmed #24862815.

ABSTRACT: -- No abstract --

22 Guideline Guidelines of care for the management of atopic dermatitis: section 3. Management and treatment with phototherapy and systemic agents. 2014

Sidbury, Robert / Davis, Dawn M / Cohen, David E / Cordoro, Kelly M / Berger, Timothy G / Bergman, James N / Chamlin, Sarah L / Cooper, Kevin D / Feldman, Steven R / Hanifin, Jon M / Krol, Alfons / Margolis, David J / Paller, Amy S / Schwarzenberger, Kathryn / Silverman, Robert A / Simpson, Eric L / Tom, Wynnis L / Williams, Hywel C / Elmets, Craig A / Block, Julie / Harrod, Christopher G / Begolka, Wendy Smith / Eichenfield, Lawrence F / Anonymous5560793. ·Department of Dermatology, Seattle Children's Hospital, Seattle, Washington. · Department of Dermatology, Mayo Clinic, Rochester, Minnesota. · Department of Dermatology, New York University School of Medicine, New York, New York. · Department of Dermatology, University of California, San Francisco, California. · Department of Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, Canada. · Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. · Department of Dermatology, Case Western University, Cleveland, Ohio. · Department of Dermatology, Wake Forest University Health Sciences, Winston-Salem, North Carolina. · Department of Dermatology, Oregon Health and Science University, Portland, Oregon. · Department of Biostatistics and Epidemiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania. · Kaplan-Amonette Department of Dermatology, University of Tennessee Health Science Center, Memphis, Tennessee. · Private practice, Fairfax, Virginia. · University of San Diego, San Diego, California; Division of Pediatric and Adolescent Dermatology, Rady Children's Hospital, San Diego, California. · Center of Evidence-based Dermatology, Nottingham University Hospitals National Health Service Trust, Nottingham, United Kingdom. · Department of Dermatology, University of Alabama at Birmingham, Birmingham, Alabama. · National Eczema Association, San Rafael, California. · American Academy of Dermatology, Schaumburg, Illinois. · American Academy of Dermatology, Schaumburg, Illinois. Electronic address: wsmithbegolka@aad.org. ·J Am Acad Dermatol · Pubmed #24813298.

ABSTRACT: Atopic dermatitis is a chronic, pruritic inflammatory dermatosis that affects up to 25% of children and 2% to 3% of adults. This guideline addresses important clinical questions that arise in atopic dermatitis management and care, providing recommendations based on the available evidence. In this third of 4 sections, treatment of atopic dermatitis with phototherapy and systemic immunomodulators, antimicrobials, and antihistamines is reviewed, including indications for use and the risk-benefit profile of each treatment option.

23 Guideline Expert panel recommendations for the use of anti-tumor necrosis factor biologic agents in patients with ocular inflammatory disorders. 2014

Levy-Clarke, Grace / Jabs, Douglas A / Read, Russell W / Rosenbaum, James T / Vitale, Albert / Van Gelder, Russell N. ·St. Luke's Cataract and Laser Institute, Tarpon Springs, Florida. · Departments of Ophthalmology and Medicine, the Mount Sinai Medical School, New York, New York; Department of Epidemiology, the Johns Hopkins University Bloomberg School of Public Health, Baltimore, Maryland. · Department of Ophthalmology, University of Alabama at Birmingham School of Medicine, Birmingham, Alabama. · Departments of Ophthalmology and Medicine, Division of Rheumatology, Oregon Health & Science University, Portland, Oregon; Department of Ophthalmology, Legacy Devers Eye Institute, Portland, Oregon. · Department of Ophthalmology, Moran Eye Institute, University of Utah School of Medicine, Salt Lake City, Utah. · Departments of Ophthalmology, Pathology, and Biological Structure, University of Washington School of Medicine, Seattle, Washington. Electronic address: russvg@u.washington.edu. ·Ophthalmology · Pubmed #24359625.

ABSTRACT: TOPIC: To provide recommendations for the use of anti-tumor necrosis factor α (TNF-α) biologic agents in patients with ocular inflammatory disorders. CLINICAL RELEVANCE: Ocular inflammatory diseases remain a leading cause of vision loss worldwide. Anti-TNF-α agents are used widely in treatment of rheumatologic diseases. A committee of the American Uveitis Society performed a systematic review of literature to generate guidelines for use of these agents in ocular inflammatory conditions. METHODS: A systematic review of published studies was performed. Recommendations were generated using the Grading of Recommendations Assessment, Development, and Evaluation group criteria. RESULTS: Numerous studies including controlled clinical trials have demonstrated that anti-TNF-α biologic agents (in particular infliximab and adalimumab) are effective in the treatment of severe ocular inflammatory disease. Based on these studies, the expert panel makes the following recommendations. CONCLUSIONS: Infliximab and adalimumab can be considered as first-line immunomodulatory agents for the treatment of ocular manifestations of Behçet's disease. Infliximab and adalimumab can be considered as second-line immunomodulatory agents for the treatment of uveitis associated with juvenile arthritis. Infliximab and adalimumab can be considered as potential second-line immunomodulatory agents for the treatment of severe ocular inflammatory conditions including posterior uveitis, panuveitis, severe uveitis associated with seronegative spondyloarthropathy, and scleritis in patients requiring immunomodulation in patients who have failed or who are not candidates for antimetabolite or calcineurin inhibitor immunomodulation. Infliximab and adalimumab can be considered in these patients in preference to etanercept, which seems to be associated with lower rates of treatment success.

24 Guideline Best practice guidelines on clinical management of acute attacks of porphyria and their complications. 2013

Stein, Penelope / Badminton, Mike / Barth, Julian / Rees, David / Stewart, M Felicity / Anonymous2820756. ·Department of Medicine, Addenbrooke's Hospital, Cambridge CB2 0QQ, UK. ·Ann Clin Biochem · Pubmed #23605132.

ABSTRACT: The British and Irish Porphyria Network guidelines describe best practice in the clinical assessment, investigation and management of acute porphyria attacks and their complications, including severe attacks with neuropathy. Acute attacks of porphyria may occur in acute intermittent porphyria (AIP), variegate porphyria (VP) and hereditary coproporphyria (HCP). Aminolaevulinic acid dehydratase deficiency porphyria (ADP) is a very rare autosomal recessive porphyria; only six cases substantiated by mutation analysis have yet been described in the literature. Urinary porphobilinogen (PBG) is always raised in an acute attack due to AIP, VP or HCP and this analysis is essential to confirm the diagnosis. A positive result in a qualitative or semi-quantitative screening test must be followed by PBG quantitation at the earliest opportunity. However in a severely ill patient, treatment should not be delayed. Removal of precipitating factors, effective analgesia and control of symptoms with safe medication, attention to nutrition and fluid balance are essential. The indications for use of intravenous haem arginate are set out, together with advice on its administration. A small proportion of acute porphyria patients develop recurrent attacks and management options that may be considered include gonadotrophin-releasing hormone analogues, 'prophylactic' regular haem arginate infusion or ultimately, liver transplantation.

25 Guideline Therapeutic patient education in children with atopic dermatitis: position paper on objectives and recommendations. 2013

Barbarot, Sébastien / Bernier, Claire / Deleuran, Mette / De Raeve, Linda / Eichenfield, Lawrence / El Hachem, May / Gelmetti, Carlo / Gieler, Uwe / Lio, Peter / Marcoux, Danielle / Morren, Marie-Anne / Torrelo, Antonio / Stalder, Jean Francois / Anonymous7440751. ·Department of Dermatology, Nantes University Hospital, Nantes, France. sebastien.barbarot@chu-nantes.fr ·Pediatr Dermatol · Pubmed #23461685.

ABSTRACT: Poor adherence is frequent in patients with atopic dermatitis (AD), leading to therapeutic failure. Therapeutic patient education (TPE) helps patients with chronic disease to acquire or maintain the skills they need to manage their chronic disease. After a review of the literature, a group of multispecialty physicians, nurses, psychologists, and patients worked together during two international workshops to develop common recommendations for TPE in AD. These recommendations were structured as answers to nine frequently asked questions about TPE in AD: What is TPE and what are its underlying principles? Why use TPE in the management of AD? Who should benefit from TPE in AD? How can TPE be organized for AD? What is the assessment process for TPE in AD? What is the evidence of the benefit of TPE in AD? Who are the people involved in TPE? How should TPE be funded in dermatology? What are the limits of the TPE process?