Morte, óbito, falecimento (falecer+mento), ou passamento (passar+mento), são termos usados para denominar o processo irreversível de cessamento das atividades biológicas necessárias à caracterização e manutenção da vida em um sistema orgânico. Os processos que seguem-se à morte geralmente são os que levam à decomposição dos sistemas. Sob condições ambientais específicas, processos distintos podem segui-la, a exemplo aqueles que levam à mumificação natural ou a fossilização de organismos. A morte encefálica às vezes é usada como uma definição legal de morte.
Introdução
O que você precisa saber de cara
Necrose avascular primária é uma condição rara que causa morte óssea, afetando principalmente a cabeça femoral. Manifesta-se com dor, rigidez e limitação de movimento no quadril, podendo levar à osteoartrite e dificuldade nas atividades diárias.
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 27 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 73 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
3 genes identificados com associação a esta condição.
May inhibit the adenylyl cyclase-stimulating activity of guanine nucleotide-binding protein G(s) subunit alpha which is produced from the same locus in a different open reading frame
Cell membraneCell projection, ruffle
Non-selective calcium permeant cation channel involved in osmotic sensitivity and mechanosensitivity (PubMed:16293632, PubMed:18695040, PubMed:18826956, PubMed:22526352, PubMed:23136043, PubMed:29899501). Activation by exposure to hypotonicity within the physiological range exhibits an outward rectification (PubMed:18695040, PubMed:18826956, PubMed:29899501). Also activated by heat, low pH, citrate and phorbol esters (PubMed:16293632, PubMed:18695040, PubMed:18826956, PubMed:20037586, PubMed:219
Cell membraneApical cell membraneCell junction, adherens junctionCell projection, ciliumEndoplasmic reticulum
Brachyolmia 3
A form of brachyolmia, a clinically and genetically heterogeneous skeletal dysplasia primarily affecting the spine and characterized by a short trunk, short stature, and platyspondyly. BCYM3 is an autosomal dominant form with severe scoliosis with or without kyphosis, and flattened irregular cervical vertebrae.
Type II collagen is specific for cartilaginous tissues. It is essential for the normal embryonic development of the skeleton, for linear growth and for the ability of cartilage to resist compressive forces
Secreted, extracellular space, extracellular matrix
Spondyloepiphyseal dysplasia congenital type
Disorder characterized by disproportionate short stature and pleiotropic involvement of the skeletal and ocular systems.
Variantes genéticas (ClinVar)
2,180 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
21 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Necrose avascular primária
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
🟢 Recrutando agora
2 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
0 ensaios clínicos encontrados.
Publicações mais relevantes
Treatment for primary avascular necrosis of the lower limb in childhood.
This is a protocol for a Cochrane Review (intervention). The objectives are as follows: To investigate the benefits and harms of surgical, non-surgical, and pharmacological interventions for paediatric primary lower limb avascular necrosis (AVN). This is a common review protocol, outlining the approach for four separate Cochrane reviews for paediatric primary lower limb AVN. To assess the benefit and harm of surgical interventions for paediatric primary lower limb AVN on function, pain, patient-reported outcomes, and radiological outcomes. To assess the benefit and harm of non-surgical interventions for paediatric primary lower limb AVN on function, pain, patient-reported outcomes, and radiological outcomes. To assess the benefit and harm of pharmacological interventions for paediatric primary lower limb AVN on function, pain, patient-reported outcomes, and radiological outcomes. To assess the comparative effectiveness of surgical, non-surgical, and pharmacological interventions for paediatric primary lower limb AVN on function, pain, patient-reported outcomes, and radiological outcomes. The secondary objective for all four of the reviews is to evaluate health equity by assessing the effects across different geographical regions, gender, and age groups.
Assessment, diagnostic criteria, and outcome measures for non-femoral head lower limb avascular necrosis in children and adolescents: a scoping review.
Avascular necrosis is a condition that results from disruption of the blood supply to bone. It can develop idiopathically or secondary to disease or corticosteroid use. To facilitate consistent clinical and research practice, this review aimed to identify methods used to assess, diagnose, and determine outcomes of non-femoral head (non-FH) lower limb avascular necrosis in children and adolescents. We conducted a scoping review up to January 2024, searching Ovid Medline, Embase, CINAHL, and Scopus databases. Studies were included if they had 10 or more individuals aged 0-17 yr with non-FH lower limb avascular necrosis, and included assessment, diagnostic criteria, or outcome measures. Measures identified were grouped according to the International Classification of Functioning, Disability, and Health framework of impairments of body function and structure, activity limitations, and participation restrictions. Following full-text screening, 31 studies met the inclusion criteria: 24 of these studies were retrospective (77%). Twenty-three studies involved secondary avascular necrosis (74%) and 8 studies involved primary avascular necrosis (26%). MRI and radiographs were most frequently used for diagnosis. Impairments were predominantly assessed via patient report, and use of validated measures was limited. There was also limited consideration of activity limitations and participation restrictions in both assessment and outcome measures. Where present, these were patient reported. The findings highlight a strong focus on impairments despite a need to consider the conditions impact on activity limitations and participation restrictions. Obtaining consensus on assessments and outcome measures, with increased use of validated measures to improve rigor, would facilitate collation of results in future research.
Clinical and Radiological Results of Hemiarthroplasty and Total Shoulder Arthroplasty for Primary Avascular Necrosis of the Humeral Head in Patients Less Than 60 Years Old.
Total shoulder arthroplasty (TSA) and hemiarthroplasty (HA) have shown good clinical outcomes in primary avascular necrosis of the humeral head (PANHH) both in short and long terms. The purpose of this study was to assess the complications, the clinical and radiological outcomes of shoulder arthroplasty in young patients with PANHH. One hundred and twenty-seven patients aged under 60 years old and suffering from PANHH were operated with arthroplasty. Patients were assessed clinically and radiographically before surgery with a minimum of 2 years of follow up (FU). HA was performed on 108 patients (85%). Two patients were revised for painful glenoid wear after 2 and 4 years. TSA was performed on 19 patients (15%). Five TSA had to be revised for glenoid loosening (n = 4) or instability (n = 1). Revision rate was 26% with TSA and 2% with HA. There were no significant differences between HA and TSA in terms of clinical outcomes. With a mean FU of 8 years, HA and TSA improved clinical outcomes of patients with PANHH. HA revisions for painful glenoid wear were rare (2%). The revision rate was excessively high with TSA (26%).
Publicações recentes
Treatment for primary avascular necrosis of the lower limb in childhood.
Assessment, diagnostic criteria, and outcome measures for non-femoral head lower limb avascular necrosis in children and adolescents: a scoping review.
Clinical and Radiological Results of Hemiarthroplasty and Total Shoulder Arthroplasty for Primary Avascular Necrosis of the Humeral Head in Patients Less Than 60 Years Old.
Primary avascular necrosis of the halluces in a ballet dancer.
Avascular necrosis and its relation to lipid and purine metabolism.
📚 EuropePMC3 artigos no totalmostrando 3
Treatment for primary avascular necrosis of the lower limb in childhood.
The Cochrane database of systematic reviewsAssessment, diagnostic criteria, and outcome measures for non-femoral head lower limb avascular necrosis in children and adolescents: a scoping review.
JBMR plusClinical and Radiological Results of Hemiarthroplasty and Total Shoulder Arthroplasty for Primary Avascular Necrosis of the Humeral Head in Patients Less Than 60 Years Old.
Journal of clinical medicineAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Necrose avascular primária.
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Necrose avascular primária
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Treatment for primary avascular necrosis of the lower limb in childhood.
- Assessment, diagnostic criteria, and outcome measures for non-femoral head lower limb avascular necrosis in children and adolescents: a scoping review.
- Clinical and Radiological Results of Hemiarthroplasty and Total Shoulder Arthroplasty for Primary Avascular Necrosis of the Humeral Head in Patients Less Than 60 Years Old.
- Primary avascular necrosis of the halluces in a ballet dancer.
- Avascular necrosis and its relation to lipid and purine metabolism.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:399302(Orphanet)
- MONDO:0018379(MONDO)
- GARD:21664(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q55788017(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
