Literature DB >> 33502802

Genetic skin disorders: The value of a multidisciplinary clinic.

James Clayton Parker1, Sneha Rangu2, Katheryn Lynn Grand3, Elizabeth Joyce Bhoj1,3, Leslie Castelo-Soccio1,2, Sarah E Sheppard3.   

Abstract

Genodermatoses are inherited disorders with skin manifestations and can present with multisystem involvement, resulting in challenges in diagnosis and treatment. To address this, the expertise of dermatology and clinical genetics through a multidisciplinary clinic (Genodermatoses Clinic) were combined. A retrospective cohort study of 45 children seen between March 2018 and February 2019 in the Genodermatoses Clinic at The Children's Hospital of Philadelphia was performed. Patient demographics, referral information, genetic testing modality, diagnoses, and patient satisfaction scores were evaluated to assess the clinic's impact. The majority of patients (42.2%) were referred from Dermatology and 86.7% were referred for diagnosis. Two-thirds of the patients were recommended genetic testing, and subsequently 73.3% completed testing. Nearly three-quarters, 26 out of 36 patients (72.2%), of our undiagnosed patients received a clinical and/or molecular diagnosis, which is imperative in managing their care. Twenty-two individuals pursued genetic testing. In eight individuals (36%), molecular testing was diagnostic. However, in two individuals the molecular diagnosis did not completely explain the phenotype. However, there are still obstacles to genetic testing, such as cost of testing and insurance barriers. Almost all (91.4%) rated the Genodermatoses Clinic as "Very Good," the top Press Ganey score. High patient satisfaction scores suggest a positive impact of the Genodermatoses clinic, emphasizing the importance to increase support for the clinical and administrative time needed for patients with genodermatoses.
© 2021 Wiley Periodicals LLC.

Entities:  

Keywords:  genetic skin disorder; genodermatosis; multidisciplinary clinic

Mesh:

Year:  2021        PMID: 33502802      PMCID: PMC8683660          DOI: 10.1002/ajmg.a.62095

Source DB:  PubMed          Journal:  Am J Med Genet A        ISSN: 1552-4825            Impact factor:   2.802


  13 in total

1.  [Molecular diagnostics in genodermatoses].

Authors:  Martin Laimer; Johann W Bauer; Roland Lang
Journal:  Hautarzt       Date:  2015-03       Impact factor: 0.751

Review 2.  A multistep approach to the diagnosis of rare genodermatoses.

Authors:  Iliana Tantcheva-Poór; Vinzenz Oji; Cristina Has
Journal:  J Dtsch Dermatol Ges       Date:  2016-10       Impact factor: 5.584

3.  What Is Known About the Psychodermatology Clinic Model of Care? A Systematic Scoping Review.

Authors:  Stephanie Zhou; Ilya Mukovozov; An-Wen Chan
Journal:  J Cutan Med Surg       Date:  2017-07-10       Impact factor: 2.092

4.  Genetic Testing in Dermatology: A Survey Analyzing Obstacles to Appropriate Care.

Authors:  Devorah R Shagalov; Georgina M Ferzli; Temima Wildman; Sharon A Glick
Journal:  Pediatr Dermatol       Date:  2016-09-22       Impact factor: 1.588

5.  Experiences From a Combined Dermatology and Rheumatology Clinic: A Retrospective Review.

Authors:  Michael Samycia; Collette McCourt; Kam Shojania; Sheila Au
Journal:  J Cutan Med Surg       Date:  2016-05-04       Impact factor: 2.092

6.  Further delineation of the phenotypic spectrum of nevus comedonicus syndrome to include congenital pulmonary airway malformation of the lung and aneurysm.

Authors:  Sarah E Sheppard; Anna Smith; Katheryn Grand; Jennifer Pogoriler; Adam I Rubin; Erica Schindewolf; Mark P Fitzgerald; Julie Moldenhauer; Pablo Laje; William Peranteau; Elizabeth Bhoj; Patrick McMahon; Leslie Castelo-Soccio
Journal:  Am J Med Genet A       Date:  2020-01-21       Impact factor: 2.802

7.  The value of diagnostic testing for parents of children with rare genetic diseases.

Authors:  Deborah A Marshall; Karen V MacDonald; Sebastian Heidenreich; Taila Hartley; Francois P Bernier; Meredith K Gillespie; Brenda McInnes; A Micheil Innes; Christine M Armour; Kym M Boycott
Journal:  Genet Med       Date:  2019-06-26       Impact factor: 8.822

8.  Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology.

Authors:  Sue Richards; Nazneen Aziz; Sherri Bale; David Bick; Soma Das; Julie Gastier-Foster; Wayne W Grody; Madhuri Hegde; Elaine Lyon; Elaine Spector; Karl Voelkerding; Heidi L Rehm
Journal:  Genet Med       Date:  2015-03-05       Impact factor: 8.822

Review 9.  Future of Rare Diseases Research 2017-2027: An IRDiRC Perspective.

Authors:  Christopher P Austin; Christine M Cutillo; Lilian P L Lau; Anneliene H Jonker; Ana Rath; Daria Julkowska; David Thomson; Sharon F Terry; Béatrice de Montleau; Diego Ardigò; Virginie Hivert; Kym M Boycott; Gareth Baynam; Petra Kaufmann; Domenica Taruscio; Hanns Lochmüller; Makoto Suematsu; Carlo Incerti; Ruxandra Draghia-Akli; Irene Norstedt; Lu Wang; Hugh J S Dawkins
Journal:  Clin Transl Sci       Date:  2017-10-23       Impact factor: 4.689

10.  International Cooperation to Enable the Diagnosis of All Rare Genetic Diseases.

Authors:  Kym M Boycott; Ana Rath; Jessica X Chong; Taila Hartley; Fowzan S Alkuraya; Gareth Baynam; Anthony J Brookes; Michael Brudno; Angel Carracedo; Johan T den Dunnen; Stephanie O M Dyke; Xavier Estivill; Jack Goldblatt; Catherine Gonthier; Stephen C Groft; Ivo Gut; Ada Hamosh; Philip Hieter; Sophie Höhn; Matthew E Hurles; Petra Kaufmann; Bartha M Knoppers; Jeffrey P Krischer; Milan Macek; Gert Matthijs; Annie Olry; Samantha Parker; Justin Paschall; Anthony A Philippakis; Heidi L Rehm; Peter N Robinson; Pak-Chung Sham; Rumen Stefanov; Domenica Taruscio; Divya Unni; Megan R Vanstone; Feng Zhang; Han Brunner; Michael J Bamshad; Hanns Lochmüller
Journal:  Am J Hum Genet       Date:  2017-05-04       Impact factor: 11.025

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