FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3173918872> ?p ?o ?g. }

• W3173918872 abstract "Epidermolysis bullosa (EB) is a heterogeneous group of inherited disorders characterized by a high degree of mucocutaneous fragility. This study aimed to describe the clinical and epidemiologic characteristics of patients with EB treated in Hospital Universitario La Paz, a national referral center for inherited EB. Observational, retrospective, single-center study. We included all cases with a clinical and molecular diagnosis of EB managed in the hospital’s dermatology department from January 2, 2000, to February 28, 2021. A total of 214 cases were studied. The median (interquartile range) age was 17 (8–32) years; 54.2% were women. One hundred thirty-five (63.1%) patients had dystrophic EB, 67 (31.3%) had EB simplex, 8 (3.7%) had junctional EB, and 3 (1.4%) had Kindler syndrome. One (0.5%) had EB acquisita. Over a third (35.5%) of the patients resided in Madrid. The most common clinical complications were pruritus (63.1%), local infections (56.5%), and pain (54.7%). The most serious ones were cardiomyopathy (in 5.6%) and squamous cell carcinoma (10.3%). Twenty-two patients (10.3%) died. Dystrophic EB was the most prevalent clinical form. The most prevalent complications were pruritus, pain, and infections. The most serious ones were cardiomyopathy and squamous cell carcinoma. This study is the first in Spain that explores strategies for improving the health status and quality of life of patients with EB. La epidermólisis bullosa (EB) es un grupo heterogéneo de trastornos hereditarios caracterizado por un aumento de la fragilidad mucocutánea. El objetivo del presente estudio es describir las características clínicas y epidemiológicas de los pacientes con EB atendidos en el Hospital Universitario La Paz, centro de referencia nacional para EB hereditaria. Estudio observacional, retrospectivo y unicéntrico. Se incluyeron todos los pacientes con diagnóstico clínico y molecular de EB atendidos en el servicio de Dermatología del Hospital Universitario La Paz desde el 1 de enero de 2000 hasta el 28 de febrero de 2021. Se registraron 214 pacientes, con una edad mediana de 17 años (RIQ: 8-32); el 54,2% fueron mujeres. Las formas clínicas correspondieron a EB distrófica con 135 (63,1%) casos, EB simple con 67 (31,3%) casos, EB juntural con 8 (3,7%), EB Kindler con 3 (1,4%) casos y EB adquirida con 1 (0,5%) caso. El 35,5% de los pacientes procedían de Madrid. Las complicaciones clínicas más frecuentes en nuestra serie fueron el prurito (63,1%), las infecciones locales (56,5%) y el dolor (54,7%). Las complicaciones más graves fueron las cardíacas (5,6%) y la aparición de CCE (10,3%). Fallecieron 22 pacientes (10,3%). La forma clínica predominante fue la EBDR. Las complicaciones más prevalentes fueron el prurito, el dolor y las infecciones, y las más graves, la miocardiopatía y el CCE. Es un estudio pionero realizado en nuestro país que permitirá́ implementar estrategias para mejorar la situación sociosanitaria de los pacientes con EB." @default.
• W3173918872 created "2021-07-05" @default.
• W3173918872 creator A5006542502 @default.
• W3173918872 creator A5013374406 @default.
• W3173918872 creator A5044982893 @default.
• W3173918872 creator A5066674831 @default.
• W3173918872 creator A5083409035 @default.
• W3173918872 creator A5084235164 @default.
• W3173918872 date "2021-07-01" @default.
• W3173918872 modified "2023-09-26" @default.
• W3173918872 title "Epidermolysis Bullosa in Spain: Observational Study of a Cohort of Patients Treated in a National Referral Center" @default.
• W3173918872 cites W1866957978 @default.
• W3173918872 cites W1966308555 @default.
• W3173918872 cites W1966829529 @default.
• W3173918872 cites W1969011049 @default.
• W3173918872 cites W1979418411 @default.
• W3173918872 cites W1989281051 @default.
• W3173918872 cites W1995568245 @default.
• W3173918872 cites W2006430422 @default.
• W3173918872 cites W2010098840 @default.
• W3173918872 cites W2019845831 @default.
• W3173918872 cites W2032108163 @default.
• W3173918872 cites W2046773115 @default.
• W3173918872 cites W2051308515 @default.
• W3173918872 cites W2061198092 @default.
• W3173918872 cites W2079623498 @default.
• W3173918872 cites W2081887077 @default.
• W3173918872 cites W2095040806 @default.
• W3173918872 cites W2095128966 @default.
• W3173918872 cites W2095145296 @default.
• W3173918872 cites W2162312651 @default.
• W3173918872 cites W2164249093 @default.
• W3173918872 cites W2171081618 @default.
• W3173918872 cites W24791593 @default.
• W3173918872 cites W2602137962 @default.
• W3173918872 cites W2986454132 @default.
• W3173918872 cites W3005172635 @default.
• W3173918872 cites W3025264127 @default.
• W3173918872 cites W3050672150 @default.
• W3173918872 doi "https://doi.org/10.1016/j.adengl.2021.07.012" @default.
• W3173918872 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/33984313" @default.
• W3173918872 hasPublicationYear "2021" @default.
• W3173918872 type Work @default.
• W3173918872 sameAs 3173918872 @default.
• W3173918872 citedByCount "0" @default.
• W3173918872 crossrefType "journal-article" @default.
• W3173918872 hasAuthorship W3173918872A5006542502 @default.
• W3173918872 hasAuthorship W3173918872A5013374406 @default.
• W3173918872 hasAuthorship W3173918872A5044982893 @default.
• W3173918872 hasAuthorship W3173918872A5066674831 @default.
• W3173918872 hasAuthorship W3173918872A5083409035 @default.
• W3173918872 hasAuthorship W3173918872A5084235164 @default.
• W3173918872 hasBestOaLocation W31739188721 @default.
• W3173918872 hasConcept C119060515 @default.
• W3173918872 hasConcept C126322002 @default.
• W3173918872 hasConcept C156662089 @default.
• W3173918872 hasConcept C16005928 @default.
• W3173918872 hasConcept C167135981 @default.
• W3173918872 hasConcept C187212893 @default.
• W3173918872 hasConcept C23131810 @default.
• W3173918872 hasConcept C2776135927 @default.
• W3173918872 hasConcept C2779134260 @default.
• W3173918872 hasConcept C2781207856 @default.
• W3173918872 hasConcept C512399662 @default.
• W3173918872 hasConcept C71924100 @default.
• W3173918872 hasConceptScore W3173918872C119060515 @default.
• W3173918872 hasConceptScore W3173918872C126322002 @default.
• W3173918872 hasConceptScore W3173918872C156662089 @default.
• W3173918872 hasConceptScore W3173918872C16005928 @default.
• W3173918872 hasConceptScore W3173918872C167135981 @default.
• W3173918872 hasConceptScore W3173918872C187212893 @default.
• W3173918872 hasConceptScore W3173918872C23131810 @default.
• W3173918872 hasConceptScore W3173918872C2776135927 @default.
• W3173918872 hasConceptScore W3173918872C2779134260 @default.
• W3173918872 hasConceptScore W3173918872C2781207856 @default.
• W3173918872 hasConceptScore W3173918872C512399662 @default.
• W3173918872 hasConceptScore W3173918872C71924100 @default.
• W3173918872 hasLocation W31739188721 @default.
• W3173918872 hasLocation W31739188722 @default.
• W3173918872 hasOpenAccess W3173918872 @default.
• W3173918872 hasPrimaryLocation W31739188721 @default.
• W3173918872 hasRelatedWork W2028132464 @default.
• W3173918872 hasRelatedWork W2053221007 @default.
• W3173918872 hasRelatedWork W2061253854 @default.
• W3173918872 hasRelatedWork W2313076522 @default.
• W3173918872 hasRelatedWork W2409884919 @default.
• W3173918872 hasRelatedWork W2789122854 @default.
• W3173918872 hasRelatedWork W3009277401 @default.
• W3173918872 hasRelatedWork W3162511055 @default.
• W3173918872 hasRelatedWork W4316080459 @default.
• W3173918872 hasRelatedWork W83978866 @default.
• W3173918872 isParatext "false" @default.
• W3173918872 isRetracted "false" @default.
• W3173918872 magId "3173918872" @default.
• W3173918872 workType "article" @default.