Necrose é o estado de morte de um tecido ou parte dele em um organismo vivo. A necrose é a morte de um grupo de células, ocorrendo a perda da permeabilidade, possui resposta inflamatória, células ficam tumeficadas. A necrose é sempre um processo patológico e desordenado de morte celular causado por fatores que levam à lesão celular irreversível e consequente morte celular. Alguns exemplos destes fatores são hipóxia/isquemia, agentes químicos tóxicos ou agentes biológicos que causem dano direto ou desencadeiem resposta imunológica danosa, como fungos, bactérias e vírus. A necrose pode ser diferenciada em vários tipos, e cada um está associado a determinado tipo de agente lesivo e determinadas características teciduais após a necrose.
Introdução
O que você precisa saber de cara
Nécrose avascular secundária é uma condição rara caracterizada pela morte de tecido ósseo devido à interrupção do suprimento sanguíneo. Manifesta-se com dor, sangramento gengival, anorexia, osteoporose e atraso de crescimento, podendo levar a complicações neurológicas e articulares.
Encontrou um erro ou informação desatualizada? Sugira uma correção →
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
+ 29 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 87 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
4 genes identificados com associação a esta condição.
Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramides/GlcCers (such as beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine) into free ceramides (such as N-acylsphing-4-enine) and glucose (PubMed:15916907, PubMed:24211208, PubMed:32144204, PubMed:39395789, PubMed:9201993). Plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed:27378698). Through the production of ceramides, participates in the PKC-activ
Lysosome membrane
Gaucher disease
An autosomal recessive lysosomal storage disease due to deficient activity of lysosomal beta-glucocerebrosidase, and characterized by accumulation of glucosylceramide in the reticulo-endothelial system. GD is a multisystem disease historically divided into three main subtypes on the basis of the presence of neurologic involvement, age at onset and progression rate: type 1 is the non-neuropathic form, type 2 is the acute neuropathic form with early onset and rapid neurologic deterioration, type 3 is the chronic neuropathic form with slow progression of neurologic features. GD shows a marked phenotypic diversity ranging from adult asymptomatic forms, at the mild end, to perinatal lethal forms at the severe end of the disease spectrum. Formal diagnosis of Gaucher disease is based on the measurement of glucocerebrosidase levels in circulating leukocytes and molecular genetic analysis.
Acts as a lysosomal receptor for glucosylceramidase (GBA1) targeting (Microbial infection) Acts as a receptor for enterovirus 71
Lysosome membrane
Epilepsy, progressive myoclonic 4, with or without renal failure
A form of progressive myoclonic epilepsy, a clinically and genetically heterogeneous group of disorders defined by the combination of action and reflex myoclonus, other types of epileptic seizures, and progressive neurodegeneration and neurocognitive impairment. EPM4 is an autosomal recessive form associated with renal failure in some cases. Cognitive function is preserved.
Plasma glycoprotein that binds a number of ligands such as heme, heparin, heparan sulfate, thrombospondin, plasminogen, and divalent metal ions. Binds heparin and heparin/glycosaminoglycans in a zinc-dependent manner. Binds heparan sulfate on the surface of liver, lung, kidney and heart endothelial cells. Binds to N-sulfated polysaccharide chains on the surface of liver endothelial cells. Inhibits rosette formation. Acts as an adapter protein and is implicated in regulating many processes such a
Secreted
Thrombophilia due to histidine-rich glycoprotein deficiency
A hemostatic disorder characterized by a tendency to thrombosis.
Most important serine protease inhibitor in plasma that regulates the blood coagulation cascade (PubMed:15140129, PubMed:15853774). AT-III inhibits thrombin, matriptase-3/TMPRSS7, as well as factors IXa, Xa and XIa (PubMed:15140129). Its inhibitory activity is greatly enhanced in the presence of heparin
Secreted, extracellular space
Antithrombin III deficiency
An important risk factor for hereditary thrombophilia, a hemostatic disorder characterized by a tendency to recurrent thrombosis. Antithrombin-III deficiency is classified into 4 types. Type I: characterized by a 50% decrease in antigenic and functional levels. Type II: has defects affecting the thrombin-binding domain. Type III: alteration of the heparin-binding domain. Plasma AT-III antigen levels are normal in type II and III. Type IV: consists of miscellaneous group of unclassifiable mutations.
Variantes genéticas (ClinVar)
535 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
10 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 secundária
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
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.
[Translated article] Clinical results of total hip arthroplasty assisted by robotic arm in Spain: Preliminary study.
Clinical, radiological and functional results of the first Spanish series of patients undergoing total hip arthroplasty assisted by Mako® (Stryker) robotic arm at the Hospital Clínico San Carlos (HCSC) in Madrid. Prospective and descriptive study analyzing the first 25 patients who underwent robotic-assisted THA at the HCSC, with a minimum follow-up of 4 months. Demographics, imaging studies (Mako® processing, Rx and CT), clinical parameters, functionality (modified Harris) and associated complications were evaluated. Average age was 67.2 years (min 47, max 88), being 56% male population sample. 88% involves primary coxarthrosis, 4% post-traumatic coxarthrosis, 4% secondary avascular necrosis and 4% secondary femoroacetabular impingement. Average surgery time was 116.9min (min 92, max 150). The average time of the first five surgeries was 122.6min, and, regarding the last five interventions, it was 108.2min. Found medical intraoperative complications were four intraoperative markers loss. Average admission time was 4.4days (min 3, max 7), with an average postoperative haemoglobin decrease of 3.08±1.08g/dl, requiring a transfusion in 12% of the cases. Three medical complications have been registered in the meantime of the admission, with a relevant case of a confusional syndrome and a fall, which resulted in a non-displaced AG1 periprosthetic fracture. The analysis of the positioning of registered implants with Mako® system shows 40.55±1.53 acetabular inclination degrees and 12.2±3.6 acetabular anteversion degrees. The postoperative image study carried out on patients, are consistent with Mako® results, as it shows an acetabular inclination of 41.2±1.7 in Rx, as well as acetabular anteversion of 16±4.6 in CT. Hip length variance ranges depending on preoperative values of 3.91mm (SD: 3.9; min -12, max 3) to 1.29mm (SD: 1.96) after surgery registered with Mako®, with an increase of an average hip length of 5.64mm (SD: 3.35). Rx simple study results show a postoperative difference between both hips of 0.5±3.08mm, which is consistent with Mako® results. Native femoral offset was stable after surgery with a showing difference both pre and post operative of the intervened hip of 0.1mm (SD: 3.7), registered with Mako®. Preoperatory modified Harris punctuation was 41.6±13.3, improving to postoperative values of 74.6±9.7 after four months since the surgery. No complications were registered in immediate postoperative (4 months). Total hip arthroplasty robot-assisted achieves an adequate precision and repeatability of the implant positioning and the postoperative hip dysmetry without showing an increase of associated complications to the technique applied. Surgery time, complications and functional results in a short-time period are similar to conventional techniques applied to great series previously published.
Clinical results of total hip arthroplasty assisted by robotic arm in Spain: Preliminary study.
Clinical, radiological and functional results of the first Spanish series of patients undergoing total hip arthroplasty assisted by Mako® (Stryker) robotic arm at the Hospital Clínico San Carlos (HCSC) in Madrid. Prospective and descriptive study analyzing the first 25 patients who underwent robotic-assisted THA at the HCSC, with a minimum follow-up of 4months. Demographics, imaging studies (Mako® processing, Rx and CT), clinical parameters, functionality (modified Harris) and associated complications were evaluated. Average age was 67.2years (min 47, max 88), being 56% male population sample. 88% involves primary coxarthrosis, 4% post-traumatic coxarthrosis, 4% secondary avascular necrosis and 4% secondary femoroacetabular impingement. Average surgery time was 116.9min (min 92, max 150). The average time of the first five surgeries was 122.6min, and, regarding the last five interventions, it was 108.2min. Found medical intraoperative complications were four intraoperative markers loss. Average admission time was 4.4days (min 3, max 7), with an average postoperative hemoglobin decrease of 3.08±1.08g/dL, requiring a transfusion in 12% of the cases. Three medical complications have been registered in the meantime of the admission, with a relevant case of a confusional syndrome and a fall, which resulted in a non-displaced AG1 periprosthetic fracture. The analysis of the positioning of registered implants with Mako® system shows 40.55±1.53 acetabular inclination degrees and 12.2±3.6 acetabular anteversion degrees. The postoperative image study carried out on patients, are consistent with Mako® s results, as it shows an acetabular inclination of 41.2±1.7 in Rx, as well as acetabular anteversion of 16±4.6 in CT. Hip length variance ranges depending on preoperative values of 3.91mm (SD: 3.9; min -12, max 3) to 1.29mm (SD: 1.96) after surgery registered with Mako®, with an increase of an average hip length of 5.64mm (SD: 3.35). Rx simple study results show a postoperative difference between both hips of 0.5±3.08mm, which is consistent with Mako® results. Native femoral offset was stable after surgery with a showing difference both pre and post operative of the intervened hip of 0.1mm (SD: 3.7), registered with Mako®. Preoperatory modified Harris punctuation was 41.6±13.3, improving to postoperative values of 74.6±9.7 after four months since the surgery. No complications were registered in immediate postoperative (4month). Total hip arthroplasty robot-assisted achieves an adequate precision and repeatability of the implant positioning and the postoperative hip dysmetry without showing an increase of associated complications to the technique applied. Surgery time, complications and functional results in a short-time period are similar to conventional techniques applied to great series previously published.
[99m Tc] Tc-MDP bone SPECT/CT diagnosing unstable slipped capital femoral epiphysis with secondary AVN in a patient with misleading knee pain.
Bone scan is highly sensitive whole-body imaging with relative low radiation in patients with non-localized skeletal symptoms. Patient is 12-year-old boy with Down syndrome, suffering recent claudication and exacerbated left knee pain unable to walk even with crutches. Three-dimensional Single photon emission computed tomography/Computed tomography (SPECT/CT) detected left slipped capital femoral epiphysis (SCFE) and secondary Avascular necrosis (AVN).
Fixation of anterolateral distal tibial fractures: the anterior malleolus.
The anterior tibial rim with the anterolateral tibial tubercle provides attachment to the anterior tibiofibular syndesmosis. It may be considered an anterior or "fourth" malleolus. Fixation of a displaced anterior tibial fragment in the treatment of malleolar fractures aims at providing a bone-to-bone fixation of the anterior tibiofibular ligament and restoring the integrity of the tibial incisura. Displaced intra-articular fragments of the anterior tibia; fractures involving the tibial incisura; fractures with intercalary fragments; impaction of the anterior tibial plafond; syndesmotic avulsions producing instability or preventing reduction of the distal fibula into the tibial incisura. Critical local soft tissues preventing an anterolateral approach; missing consent to surgery by the patient; overall critical general condition preventing surgery to the extremities. Anterolateral approach over the tibial tubercle. Identification and mobilization of the anterior tibial fragment without dissecting the anterior syndesmosis. Reduction of the anterior tibial fragment with a pointed reduction clamp. Fixation of extra-articular avulsion fractures (type 1) with suture anchor. Screw fixation of larger fragments involving the joint surface and incisura (type 2). Disimpaction, realignment of the joint surface, bone grafting as needed and plate fixation of impaction fractures of the anterolateral tibial plafond (type 3). Mobilization with partial weight bearing (15-20 kg) in a special boot (ankle foot orthosis) or cast for 6-8 weeks depending on the overall malleolar fracture pattern, bone quality and patient compliance. Few studies report the results of anterior tibial fractures in adults. Failure to fix displaced fragments frequently leads to nonunions. Overlooked Chaput fractures have been reported to result in malpositioning of the distal fibula in the tibial incisura leading to incongruity of the ankle mortise requiring revision surgery. Secondary avascular necrosis of the anterolateral tibial plafond may develop after joint impaction. OPERATIONSZIEL: Die Tibiavorderkante mit dem Tuberculum anterius tibiae dient als Ansatz für das Lig. tibiofibulare anterius. Sie kann als vorderer oder „vierter“ Knöchel bezeichnet werden. Ziel der Osteosynthese dislozierter Fragmente ist die knöcherne Stabilisierung der vorderen Syndesmose und die Wiederherstellung der Incisura tibiae für die distale Fibula. Dislozierte Gelenkfrakturen der Tibiavorderkante, Frakturen mit Verwerfung der vorderen Tibiainzisur, Frakturen mit Intermediärfragment(en), Impression des vorderen Tibiaplafonds, instabile Avulsionen des Lig. tibiofibulare anterius oder in den Syndesmosenspalt eingeschlagene Fragmente. Kritische Weichteilverhältnisse im Zugangsbereich, Ablehnung der Operation durch den Patienten, kritischer Allgemeinzustand des Patienten. Anterolateraler Zugang über dem Tuberculum anterius tibiae. Identifikation und Mobilisierung des Tibiavorderkantenfragments ohne Dissektion des Lig. tibifibulare anterius. Reposition des Fragments mit einer spitzen Repositionszange. Fixierung extraartikulärer Avulsionsfrakturen mit Nahtanker. Schraubenosteosynthese größerer Fragmente mit Beteiligung der Gelenkfläche und Inzisur. Anhebung, Wiederherstellung der Gelenkfläche, Spongiosaplastik, wenn erforderlich, und anteriore Plattenosteosynthese von Impressionsfrakturen des vorderen Tibiaplafonds. Mobilisation im Verbandsstiefel oder Castverband mit Teilbelastung (15–20 kg) für 6–8 Wochen je nach Frakturmuster, Knochenqualität und Patientencompliance. Nur wenige Studien berichten über Resultate nach der Osteosynthese von Tibiavorderkantenfrakturen bei Erwachsenen. Bei ausbleibender Fixierung dislozierter Fragmente werden Pseudarthrosen beschrieben. Bei der Versorgung von Sprunggelenkfrakturen übersehene Tibiavorderkantenfragmente können zu einer Fehlplatzierung der distalen Fibula in der Incisura fibularis tibiae mit nachfolgender Inkongruenz der Knöchelgabel und der Notwendigkeit von Revisionsoperationen führen. Sekundäre avaskuläre Nekrosen des anterolateralen Tibiaplafonds können nach Impaktionsverletzungen entstehen.
Publicações recentes
Selective Femoral Resurfacing for Bilateral Knee Osteonecrosis: A Bone-Preserving Alternative to Total Knee Arthroplasty.
Assessment, diagnostic criteria, and outcome measures for non-femoral head lower limb avascular necrosis in children and adolescents: a scoping review.
[Translated article] Clinical results of total hip arthroplasty assisted by robotic arm in Spain: Preliminary study.
[(99m) Tc] Tc-MDP bone SPECT/CT diagnosing unstable slipped capital femoral epiphysis with secondary AVN in a patient with misleading knee pain.
Clinical results of total hip arthroplasty assisted by robotic arm in Spain: Preliminary study.
📚 EuropePMC3 artigos no totalmostrando 6
Assessment, diagnostic criteria, and outcome measures for non-femoral head lower limb avascular necrosis in children and adolescents: a scoping review.
JBMR plus[Translated article] Clinical results of total hip arthroplasty assisted by robotic arm in Spain: Preliminary study.
Revista espanola de cirugia ortopedica y traumatologia[99m Tc] Tc-MDP bone SPECT/CT diagnosing unstable slipped capital femoral epiphysis with secondary AVN in a patient with misleading knee pain.
Clinical case reportsClinical results of total hip arthroplasty assisted by robotic arm in Spain: Preliminary study.
Revista espanola de cirugia ortopedica y traumatologiaFixation of anterolateral distal tibial fractures: the anterior malleolus.
Operative Orthopadie und Traumatologie[Fractures of the anterolateral tibial rim : The fourth malleolus].
Der UnfallchirurgAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Necrose avascular secundária.
É de uma associação que acompanha esta doença? Fale com a gente →
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Necrose avascular secundária
Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.
Tire suas dúvidas
Perguntas, dicas e experiências compartilhadas aqui na página
Participe da discussão
Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.
Fazer loginDoenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
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.
- Assessment, diagnostic criteria, and outcome measures for non-femoral head lower limb avascular necrosis in children and adolescents: a scoping review.
- [Translated article] Clinical results of total hip arthroplasty assisted by robotic arm in Spain: Preliminary study.
- Clinical results of total hip arthroplasty assisted by robotic arm in Spain: Preliminary study.
- [99m Tc] Tc-MDP bone SPECT/CT diagnosing unstable slipped capital femoral epiphysis with secondary AVN in a patient with misleading knee pain.
- Fixation of anterolateral distal tibial fractures: the anterior malleolus.
- Selective Femoral Resurfacing for Bilateral Knee Osteonecrosis: A Bone-Preserving Alternative to Total Knee Arthroplasty.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:399169(Orphanet)
- MONDO:0018374(MONDO)
- GARD:21659(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q55788011(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
