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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorVAISBICH, Maria Helena
dc.contributor.authorSATIRO, Carla Aline Fernandes
dc.contributor.authorROZ, Deborah
dc.contributor.authorNUNES, Debora de Almeida Domingues
dc.contributor.authorMESSA, Ana Carola H Lobo
dc.contributor.authorLANETZKI, Camila
dc.contributor.authorFERREIRA, Juliana Caires de Oliveira Achili
dc.date.accessioned2019-06-05T14:42:33Z-
dc.date.available2019-06-05T14:42:33Z-
dc.date.issued2018
dc.identifier.citationBRAZILIAN JOURNAL OF NEPHROLOGY, v.41, n.1, p.131-141, 2018
dc.identifier.issn2175-8239
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/32086-
dc.description.abstractAbstract Care for patients with chronic and rare diseases is complex, especially considering the lack of knowledge about the disease, which makes early and precise diagnosis difficult, as well as the need for specific tests, sometimes of high complexity and cost. Added to these factors are difficulties in obtaining adequate treatment when available, in raising patient and family awareness about the disease and treatment compliance. Nephropathic cystinosis is among these diseases. After more than 20 years as a care center for these patients, the authors propose a follow-up protocol, which has been used with improvement in the quality of care and consists of a multidisciplinary approach, including care provided by a physician, nurse, psychologist, nutritionist and social worker. In this paper, each field objectively exposes how to address points that involve the stages of diagnosis and its communication with the patient and their relatives or guardians, covering the particularities of the disease and the treatment, the impact on the lives of patients and families, the approach to psychological and social issues and guidelines on medications and diets. This protocol could be adapted to the follow-up of patients with other rare diseases, including those with renal involvement. This proposal is expected to reach the largest number of professionals involved in the follow-up of these patients, strengthening the bases for the creation of a national protocol, observing the particularities of each case.eng
dc.description.abstractResumo A assistência a pacientes com doenças crônicas e raras é complexa, principalmente pela falta de disseminação de conhecimento sobre a doença, o que dificulta o diagnóstico preciso e precoce, além da necessidade da realização de exames específicos, por vezes de alta complexidade e custo. Somam-se a esses fatores dificuldades na obtenção de tratamento adequado quando disponível, na conscientização do paciente e da família sobre a doença e na aderência ao tratamento. A cistinose nefropática está entre essas doenças. Após mais de 20 anos como centro de atendimento a esses pacientes, os autores propõem um protocolo de seguimento, o qual vem sendo empregado com melhora na qualidade da assistência e consiste de uma abordagem multidisciplinar, incluindo, principalmente, atendimento prestado por médico, enfermeiro, psicólogo, nutricionista e assistente social. Neste artigo, cada área expõe de maneira objetiva como abordar pontos que envolvem as etapas do diagnóstico e sua comunicação ao paciente e a seus familiares ou responsáveis, abrangendo as particularidades da doença e do tratamento, o impacto na vida do paciente e de sua família, a abordagem das questões psicológicas e sociais e orientações quanto a medicamentos e dietas. Considera-se que este protocolo poderia ser adaptado ao seguimento de pacientes portadores de outras doenças raras, incluindo aquelas com envolvimento renal. Com essa proposta, espera-se alcançar o maior número de profissionais envolvidos no seguimento desses pacientes, fortalecendo as bases para a criação de um protocolo nacional, observando-se as particularidades de cada caso.por
dc.language.isoengpor
dc.publisherSociedade Brasileira de Nefrologiaeng
dc.relation.ispartofBrazilian Journal of Nephrology
dc.rightsopenAccesseng
dc.subjectPatient Care Teameng
dc.subjectCystinosiseng
dc.subjectChildeng
dc.subjectTeenagereng
dc.subjectAdolescenteng
dc.subjectEquipe de Assistência ao Pacienteeng
dc.subjectCistinoseeng
dc.subjectCriançaeng
dc.subjectAdolescenteeng
dc.titleMultidisciplinary approach for patients with nephropathic cystinosis: model for care in a rare and chronic renal diseaseeng
dc.title.alternativeAbordagem multidisciplinar para pacientes com cistinose nefropática: modelo para atendimento em uma doença renal rara e crônicaeng
dc.typearticleeng
dc.rights.holderCopyright Sociedade Brasileira de Nefrologiaeng
dc.identifier.doi10.1590/2175-8239-jbn-2018-0139
dc.subject.wosUrology & Nephrologyeng
dc.type.categoryeditorial materialeng
dc.type.versionpublishedVersioneng
hcfmusp.description.beginpage131
hcfmusp.description.endpage141
hcfmusp.description.issue1
hcfmusp.description.volume41
hcfmusp.origemsciELO
hcfmusp.origem.idSCIELO:S0101-28002019000100131
hcfmusp.origem.id2-s2.0-85065656085
hcfmusp.relation.referenceAriceta G, 2015, NEPHROL DIAL TRANSPL, V30, P475, DOI 10.1093/ndt/gfu329eng
hcfmusp.relation.referenceBesouw M, 2010, MINERVA PEDIATR, V62, P307eng
hcfmusp.relation.referenceBesouw MTP, 2013, J PEDIATR-US, V163, P754, DOI 10.1016/j.jpeds.2013.03.078eng
hcfmusp.relation.referenceBesouw MTP, 2011, J PEDIATR-US, V159, P1004, DOI 10.1016/j.jpeds.2011.05.057eng
hcfmusp.relation.referenceBrodin-Sartorius A, 2012, KIDNEY INT, V81, P179, DOI 10.1038/ki.2011.277eng
hcfmusp.relation.referenceCentenaro Grizy Augusta, 2010, Ciênc. saúde coletiva, V15, P1881, DOI 10.1590/S1413-81232010000700102eng
hcfmusp.relation.referenceCohen C, 2015, ORPHANET J RARE DIS, V10, DOI 10.1186/s13023-015-0307-9eng
hcfmusp.relation.referenceColeman JE, 2000, PEDIATR NEPHROL, V14, P833, DOI 10.1007/s004670000318eng
hcfmusp.relation.referenceGuimaraes LPD, 2014, PEDIATR NEPHROL, V29, P1097, DOI 10.1007/s00467-013-2705-3eng
hcfmusp.relation.referenceOnis Mercedes de, 2007, Bull World Health Organ, V85, P660, DOI 10.2471/BLT.07.043497eng
hcfmusp.relation.referenceDohil R, 2005, PEDIATR NEPHROL, V20, P1786, DOI 10.1007/s00467-005-2027-1eng
hcfmusp.relation.referenceElenberg E, 1998, PEDIATR NEPHROL, V12, P365, DOI 10.1007/s004670050467eng
hcfmusp.relation.referenceEllingsen DG, 2007, HANDBOOK ON THE TOXICOLOGY OF METALS, 3RD EDITION, P529, DOI 10.1016/B978-012369413-3/50081-1eng
hcfmusp.relation.referenceFrisancho A R, 1990, Anthropometric Standards for the Assessment of Growth and Nutritional Statuseng
hcfmusp.relation.referenceGahl WA, 2002, NEW ENGL J MED, V347, P111, DOI 10.1056/NEJMra020552eng
hcfmusp.relation.referenceGreco M, 2010, PEDIATR NEPHROL, V25, P2459, DOI 10.1007/s00467-010-1641-8eng
hcfmusp.relation.referenceHarvey LJ, 2009, AM J CLIN NUTR, V89, p2009S, DOI 10.3945/ajcn.2009.27230Eeng
hcfmusp.relation.referenceHAYCOCK GB, 1982, ARCH DIS CHILD, V57, P934, DOI 10.1136/adc.57.12.934eng
hcfmusp.relation.referenceHogg RJ, 2003, PEDIATRICS, V111, P1416, DOI 10.1542/peds.111.6.1416eng
hcfmusp.relation.referenceLabbe A, 2014, MOL GENET METAB, V111, P314, DOI 10.1016/j.ymgme.2013.12.298eng
hcfmusp.relation.referenceLangman CB, 2016, KIDNEY INT, V89, P1192, DOI 10.1016/j.kint.2016.01.033eng
hcfmusp.relation.referenceLangman CB, 2012, CLIN J AM SOC NEPHRO, V7, P1112, DOI 10.2215/CJN.12321211eng
hcfmusp.relation.referenceLevtchenko E, 2003, CLIN NEPHROL, V60, P386eng
hcfmusp.relation.referenceNapolitano G, 2015, EMBO MOL MED, V7, P158, DOI 10.15252/emmm.201404223eng
hcfmusp.relation.referenceNesterova G, 2013, PEDIATR NEPHROL, V28, P51, DOI 10.1007/s00467-012-2242-5eng
hcfmusp.relation.referenceTown M, 1998, NAT GENET, V18, P319, DOI 10.1038/ng0498-319eng
hcfmusp.relation.referenceVaisbich MH, 2010, NEPHRON CLIN PRACT, V114, pC12, DOI 10.1159/000245065eng
hcfmusp.relation.reference2002, Dietary reference intakeseng
dc.description.indexMEDLINEeng
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hcfmusp.scopus.lastupdate2022-05-06-
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