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2.
J Arthroplasty ; 2024 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-38754706

RESUMO

BACKGROUND: This review aimed to determine outcomes following megaprostheses in non-oncological indications for knee arthroplasty, including range of motion (ROM) and patient-reported outcome measures of function, pain, and quality of life (QoL). METHODS: A search of MEDLINE, Embase, and Cochrane via Ovid and PubMed between January 2003 and June 2023 was conducted. Studies reporting function, pain, ROM, and/or QoL in non-oncological patients who have received knee megaprostheses were included. Studies with sample sizes (n ≤ 5) were excluded. The risk of bias was assessed using the Downs and Black Quality Checklist for Health Care Intervention Studies. Central tendency measures (mean or median) were reported at each time point, and dispersion measures were extracted and reported whenever data were available. RESULTS: A total of 30 studies (involving 1,294 megaprostheses) were included. Of which, 14 of 30 studies reviewed patients who had mixed indications; 14 of 30 looked at fracture only; 1 of 30 focused on distal femur nonunion; and 1 of 30 focused on patients who had periprosthetic infections. The average patient follow-up time was 40.1 months (range, 1.0 to 93.5). Most studies presented a high risk of bias (27 of 30), while a few (3 of 30) presented a low risk of bias. Improvements from preoperative baseline were observed in 85.7% of studies that reported baseline and follow-up data for function (12 of 14), 100.0% pain (4 of 4), 90.9% ROM (10 of 11), and 66.6% QoL (2 of 3). CONCLUSIONS: Favorable function, pain, ROM, and QoL outcomes following knee megaprostheses in non-oncological patients were observed. Heterogeneity in outcome measures and follow-up periods prevented the pooling of data. Future comparative studies are warranted to enhance the body of evidence relating to knee megaprostheses in non-oncological patients.

3.
Bone Joint J ; 106-B(5): 425-429, 2024 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-38689572

RESUMO

Chondrosarcoma is the second most common surgically treated primary bone sarcoma. Despite a large number of scientific papers in the literature, there is still significant controversy about diagnostics, treatment of the primary tumour, subtypes, and complications. Therefore, consensus on its day-to-day treatment decisions is needed. In January 2024, the Birmingham Orthopaedic Oncology Meeting (BOOM) attempted to gain global consensus from 300 delegates from over 50 countries. The meeting focused on these critical areas and aimed to generate consensus statements based on evidence amalgamation and expert opinion from diverse geographical regions. In parallel, periprosthetic joint infection (PJI) in oncological reconstructions poses unique challenges due to factors such as adjuvant treatments, large exposures, and the complexity of surgery. The meeting debated two-stage revisions, antibiotic prophylaxis, managing acute PJI in patients undergoing chemotherapy, and defining the best strategies for wound management and allograft reconstruction. The objectives of the meeting extended beyond resolving immediate controversies. It sought to foster global collaboration among specialists attending the meeting, and to encourage future research projects to address unsolved dilemmas. By highlighting areas of disagreement and promoting collaborative research endeavours, this initiative aims to enhance treatment standards and potentially improve outcomes for patients globally. This paper sets out some of the controversies and questions that were debated in the meeting.


Assuntos
Neoplasias Ósseas , Condrossarcoma , Humanos , Antibioticoprofilaxia , Neoplasias Ósseas/terapia , Neoplasias Ósseas/cirurgia , Condrossarcoma/terapia , Oncologia , Ortopedia , Infecções Relacionadas à Prótese/terapia , Infecções Relacionadas à Prótese/etiologia , Reoperação
4.
J Robot Surg ; 18(1): 206, 2024 May 08.
Artigo em Inglês | MEDLINE | ID: mdl-38717705

RESUMO

As uptake of robotic-assisted arthroplasty increases there is a need for economic evaluation of the implementation and ongoing costs associated with robotic surgery. The aims of this study were to describe the in-hospital cost of robotic-assisted total knee arthroplasty (RA-TKA) and robotic-assisted unicompartmental knee arthroplasty (RA-UKA) and determine the influence of patient characteristics and surgical outcomes on cost. This prospective cohort study included adult patients (≥ 18 years) undergoing primary unilateral RA-TKA and RA-UKA, at a tertiary hospital in Sydney between April 2017 and June 2021. Patient characteristics, surgical outcomes, and in-hospital cost variables were extracted from hospital medical records. Differences between outcomes for RA-TKA and RA-UKA were compared using independent sample t-tests. Logistic regression was performed to determine drivers of cost. Of the 308 robotic-assisted procedures, 247 were RA-TKA and 61 were RA-UKA. Surgical time, time in the operating room, and length of stay were significantly shorter in RA-UKA (p < 0.001); whereas RA-TKA patients were older (p = 0.002) and more likely to be discharged to in-patient rehabilitation (p = 0.009). Total in-hospital cost was significantly higher for RA-TKA cases (AU$18580.02 vs $13275.38; p < 0.001). Robotic system and maintenance cost per case was AU$3867.00 for TKA and AU$5008.77 for UKA. Patients born overseas and lower volume robotic surgeons were significantly associated with higher total cost of RA-UKA. Increasing age and male gender were significantly associated with higher total cost of RA-TKA. Total cost was significantly higher for RA-TKA than RA-UKA. Robotic system costs for RA-UKA are inflated by the software cost relative to the volume of cases compared with RA-TKA. Cost is an important consideration when evaluating long term benefits of robotic-assisted knee arthroplasty in future studies to provide evidence for the economic sustainability of this practice.


Assuntos
Artroplastia do Joelho , Custos Hospitalares , Tempo de Internação , Procedimentos Cirúrgicos Robóticos , Humanos , Procedimentos Cirúrgicos Robóticos/economia , Procedimentos Cirúrgicos Robóticos/métodos , Artroplastia do Joelho/economia , Artroplastia do Joelho/métodos , Masculino , Feminino , Idoso , Pessoa de Meia-Idade , Estudos Prospectivos , Tempo de Internação/economia , Tempo de Internação/estatística & dados numéricos , Custos Hospitalares/estatística & dados numéricos , Duração da Cirurgia , Resultado do Tratamento
5.
Bone Jt Open ; 5(4): 260-268, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38555947

RESUMO

Aims: Custom triflange acetabular components (CTACs) play an important role in reconstructive orthopaedic surgery, particularly in revision total hip arthroplasty (rTHA) and pelvic tumour resection procedures. Accurate CTAC positioning is essential to successful surgical outcomes. While prior studies have explored CTAC positioning in rTHA, research focusing on tumour cases and implant flange positioning precision remains limited. Additionally, the impact of intraoperative navigation on positioning accuracy warrants further investigation. This study assesses CTAC positioning accuracy in tumour resection and rTHA cases, focusing on the differences between preoperative planning and postoperative implant positions. Methods: A multicentre observational cohort study in Australia between February 2017 and March 2021 included consecutive patients undergoing acetabular reconstruction with CTACs in rTHA (Paprosky 3A/3B defects) or tumour resection (including Enneking P2 peri-acetabular area). Of 103 eligible patients (104 hips), 34 patients (35 hips) were analyzed. Results: CTAC positioning was generally accurate, with minor deviations in cup inclination (mean 2.7°; SD 2.84°), anteversion (mean 3.6°; SD 5.04°), and rotation (mean 2.1°; SD 2.47°). Deviation of the hip centre of rotation (COR) showed a mean vector length of 5.9 mm (SD 7.24). Flange positions showed small deviations, with the ischial flange exhibiting the largest deviation (mean vector length of 7.0 mm; SD 8.65). Overall, 83% of the implants were accurately positioned, with 17% exceeding malpositioning thresholds. CTACs used in tumour resections exhibited higher positioning accuracy than rTHA cases, with significant differences in inclination (1.5° for tumour vs 3.4° for rTHA) and rotation (1.3° for tumour vs 2.4° for rTHA). The use of intraoperative navigation appeared to enhance positioning accuracy, but this did not reach statistical significance. Conclusion: This study demonstrates favourable CTAC positioning accuracy, with potential for improved accuracy through intraoperative navigation. Further research is needed to understand the implications of positioning accuracy on implant performance and long-term survival.

7.
Haematologica ; 109(4): 1220-1232, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-37794800

RESUMO

Multiple myeloma (MM) is an incurable disease of the bone marrow (BM) characterized by the uncontrolled proliferation of neoplastic plasma cells. While CD8+ T cells have an established role in disease control, few studies have focused on these cells within the MM tumor microenvironment (TME). We analyzed CD8+ T cells in the BM and peripheral blood (PB) of untreated patients with MM and non-myeloma controls using flow cytometry, mass cytometry and single-cell RNA sequencing, using several novel bioinformatics workflows. Inter-tissue differences were most evident in the differential expression of Granzymes B and K, which were strongly associated with two distinct subsets of CD8+ T cells delineated by the expression of CD69, accounting for roughly 50% of BM-CD8+ T cells of all assessed cohorts. While few differences were observable between health and disease in the BM-restricted CD8CD69+ T-cell subset, the CD8+CD69- T-cell subset in the BM of untreated MM patients demonstrated increased representation of highly differentiated effector cells and evident compositional parallels between the PB, absent in age-matched controls, where a marked reduction of effector cells was observed. We demonstrate the transcriptional signature of BM-CD8+ T cells from patients with MM more closely resembles TCR-activated CD8+ T cells from age-matched controls than their resting counterparts.


Assuntos
Mieloma Múltiplo , Humanos , Mieloma Múltiplo/patologia , Linfócitos T CD8-Positivos/metabolismo , Subpopulações de Linfócitos T/patologia , Medula Óssea/patologia , Análise de Célula Única , Microambiente Tumoral
8.
Bone Jt Open ; 4(11): 846-852, 2023 Nov 08.
Artigo em Inglês | MEDLINE | ID: mdl-37935246

RESUMO

Aims: Tenosynovial giant cell tumour (TGCT) is a rare benign tumour of the musculoskeletal system. Surgical management is fraught with challenges due to high recurrence rates. The aim of this study was to describe surgical treatment and evaluate surgical outcomes of TGCT at an Australian tertiary referral centre for musculoskeletal tumours and to identify factors affecting recurrence rates. Methods: A prospective database of all patients with TGCT surgically managed by two orthopaedic oncology surgeons was reviewed. All cases irrespective of previous treatment were included and patients without follow-up were excluded. Pertinent tumour characteristics and surgical outcomes were collected for analysis. Results: There were 111 total cases included in the study; 71 (64%) were female, the mean age was 36 years (SD 13.6), and the knee (n = 64; 57.7%) was the most commonly affected joint. In all, 60 patients (54.1%) had diffuse-type (D-TGCT) disease, and 94 patients (84.7%) presented therapy-naïve as "primary cases" (PC). The overall recurrence rate was 46.8% for TGCT. There was a statistically significant difference in recurrence rates between D-TGCT and localized disease (75.0% vs 13.7%, relative risk (RR) 3.40, 95% confidence interval (CI) 2.17 to 5.34; p < 0.001), and for those who were referred in the "revision cases" (RC) group compared to the PC group (82.4% vs 48.9%, RR 1.68, 95% CI 1.24 to 2.28; p = 0.011). Age, sex, tumour volume, and mean duration of symptoms were not associated with recurrence (p > 0.05). Conclusion: Recurrence rates remain high even at a tertiary referral hospital. Highest rates are seen in D-TGCT and "revision cases". Due to the risks of recurrence, the complexity of surgery, and the need for adjuvant therapy, this paper further supports the management of TGCT in a tertiary referral multi-disciplinary orthopaedic oncology service.

9.
Cancers (Basel) ; 15(3)2023 02 02.
Artigo em Inglês | MEDLINE | ID: mdl-36765897

RESUMO

Diffuse-type tenosynovial giant cell tumors' (D-TGCTs) intra- and extra-articular expansion about the knee often necessitates an anterior and posterior surgical approach to facilitate an extensive synovectomy. There is no consensus on whether two-sided synovectomies should be performed in one or two stages. This retrospective study included 191 D-TGCT patients from nine sarcoma centers worldwide to compare the postoperative short-term outcomes between both treatments. Secondary outcomes were rates of radiological progression and subsequent treatments. Between 2000 and 2020, 117 patients underwent one-stage and 74 patients underwent two-stage synovectomies. The maximum range of motion achieved within one year postoperatively was similar (flexion 123-120°, p = 0.109; extension 0°, p = 0.093). Patients undergoing two-stage synovectomies stayed longer in the hospital (6 vs. 4 days, p < 0.0001). Complications occurred more often after two-stage synovectomies, although this was not statistically different (36% vs. 24%, p = 0.095). Patients treated with two-stage synovectomies exhibited more radiological progression and required subsequent treatments more often than patients treated with one-stage synovectomies (52% vs. 37%, p = 0.036) (54% vs. 34%, p = 0.007). In conclusion, D-TGCT of the knee requiring two-side synovectomies should be treated by one-stage synovectomies if feasible, since patients achieve a similar range of motion, do not have more complications, but stay for a shorter time in the hospital.

10.
Cancer Med ; 12(1): 368-378, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-35603739

RESUMO

BACKGROUND: Mesenchymal chondrosarcoma (MCS) is an ultra-rare sarcoma that follows a more aggressive course than conventional chondrosarcoma. This study evaluates prognostic factors, treatments (surgery, chemotherapy, and radiation), and outcomes in an Australian setting. METHODS: We collected demographics, clinicopathological variables, treatment characteristics, and survival status from patients with MCS registered on the national ACCORD sarcoma database. Outcomes include overall survival (OS) and progression-free survival (PFS). RESULTS: We identified 22 patients with MCS between 2001-2022. Median age was 28 (range 10-59) years, 19 (86%) had localised disease at diagnosis of whom 16 had surgery (84%), 11 received radiation (58%), and 10 chemotherapy (53%). Ten (52%) developed recurrence and/or metastases on follow-up and three patients with initial metastatic disease received surgery, radiation, and chemotherapy. At a median follow-up of 50.9  (range 0.4-210) months nine patients had died. The median OS was 104.1 months (95% CI 25.8-182.3). There was improved OS for patients with localised disease who had surgical resection of the primary (p = 0.003) and those with ECOG 0-1 compared to 2-3 (p = 0.023) on univariate analysis. CONCLUSIONS: This study demonstrates contemporary Australian treatment patterns of MCS. The role of chemotherapy for localised disease remains uncertain. Understanding treatment patterns and outcomes help support treatment decisions and design of trials for novel therapeutic strategies.


Assuntos
Neoplasias Ósseas , Condrossarcoma Mesenquimal , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Criança , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Condrossarcoma Mesenquimal/cirurgia , Neoplasias Ósseas/patologia , Austrália/epidemiologia , Estudos de Coortes , Estudos Retrospectivos
11.
Knee Surg Sports Traumatol Arthrosc ; 31(3): 1098-1105, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36446908

RESUMO

PURPOSE: Joint dynamics following Total Knee Arthroplasty (TKA) may influence patient-reported outcome. Simulations allow many knee alignment approaches to a single patient to be considered prior to surgery. The simulated kinematics can be matched to patient-reported outcome to predict kinematic patterns most likely to give the best outcome. This study aims to validate one such previously developed algorithm based on a simulated deep knee bend (the Dynamic Knee Score, DKS). METHODS: 1074 TKA patients with pre- and post-operative Computerised Tomography (CT) scans and 12-month post-operative Knee Injury and Osteoarthritis Outcomes (KOOS) Scores were identified from the 360 Med Care Joint Registry. Landmarking and registration of implant position was performed on all CT scans, and each of the achieved TKAs was computationally simulated and received a predictive outcome score from the DKS. In addition, a set of potential alternative surgical plans which might have been followed were simulated. Comparison of patient-reported issues and DKS score was evaluated in a counter-factual study design. RESULTS: Patient-reported impairment with the knee catching and squatting was shown to be 30% lower (p = 0.005) and 22% lower (p = 0.026) in patients where the best possible DKS result was the one surgically achieved. Similar findings were found relating attainment of the best tibial slope and posterior femoral resection DKS plans to patient-reported difficulty straightening the knee (40% less likely, p < 0.001) and descending stairs (35% less likely, p = 0.006). CONCLUSION: The DKS has been shown to correlate with presence of patient-reported impairments post-TKA and the resultant algorithm can be applied in a pre-operative planning setting. Outcome optimization in the future may come from patient-specific selection of an alignment strategy and simulations may be a technological enabler of this trend. LEVEL OF EVIDENCE: III (Retrospective Cohort Study).


Assuntos
Artroplastia do Joelho , Prótese do Joelho , Osteoartrite do Joelho , Humanos , Estudos Retrospectivos , Articulação do Joelho/cirurgia , Osteoartrite do Joelho/cirurgia , Medidas de Resultados Relatados pelo Paciente , Fenômenos Biomecânicos
12.
Hip Int ; 33(5): 905-915, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36408844

RESUMO

BACKGROUND: Reconstructive procedure following resection of large pelvic tumours around the hip joint remains a complex challenge. METHODS: This study presents a retrospective case series of patients presenting with benign or malignant pelvic tumour for which an internal hemipelvectomy including the hip joint and subsequent reconstruction with a custom designed 3-dimensional printed titanium pelvic implant (3DPPI) has been performed between August 2013 and January 2018. RESULTS: 15 consecutive patients with a median age of 33.9 years (IQR 26.4-72.2) and a median BMI of 20.7 kg/m2 (IQR 19.0-33.3) were reviewed after median follow-up of 33.8 months (IQR 24.0-78.1). The majority of patients presented with a malignant tumour as their principal diagnosis (n = 13, 86.7%). The median surgical time was 5.5 hours (IQR 4.5-8.5) and median peri-operative blood loss was 5000 ml (IQR 2000-10000). The median MSTS score at follow-up was 63.3% (IQR 51.7-86.7%). The median NRS in rest was 0.0 (IQR 0.0-5.0), the median NRS during activity was 2.0 (IQR 0.5-7.0) and the median HOOS-PS was 76.6% (IQR 67.9-91.0). 4 patients had implant-specific complications (n = 4, 26.6%); 1 hip dislocation (Henderson type 1a), 3 structural complications (type 3a), 1 deep infection (type 4a) and 1 local tumour recurrence (type 5b). At follow-up, 4 out of 15 implants were classified as a failure, resulting in an implant survival rate of 73.3%. CONCLUSIONS: Acceptable peri-operative outcomes, functional results, complication rates and short-term implant survival can be achieved in a cohort of complex patients undergoing 3DPPI reconstruction after hemipelvectomy including the acetabulum.


Assuntos
Artroplastia de Quadril , Neoplasias Pélvicas , Humanos , Pré-Escolar , Criança , Acetábulo/diagnóstico por imagem , Acetábulo/cirurgia , Acetábulo/patologia , Titânio , Artroplastia de Quadril/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento , Complicações Pós-Operatórias/etiologia , Neoplasias Pélvicas/diagnóstico por imagem , Neoplasias Pélvicas/cirurgia , Impressão Tridimensional
13.
Vasc Endovascular Surg ; 56(8): 779-783, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35750487

RESUMO

Persistent sciatic arteries are rare congenital abnormalities, with an incidence between .01-.6%. Whilst most patients with the pathology are asymptomatic during their younger years, up to 48% of patients will suffer aneurysmal degeneration of the persistent sciatic artery and present with claudication or lower limb ischaemia (from distal embolisation of mural thrombus). Due to the rare nature of persistent sciatic artery aneurysms, optimal management of the pathology is yet to be determined. Both endovascular and open techniques have been described in the management of this pathology, however hybrid management approaches are reported infrequently. The authors present a case of a persistent sciatic artery aneurysm successfully treated in a 69-year-old male utilising a hybrid approach with a femoropopliteal bypass (below knee), Amplatzer plug occlusion of the aneurysm and subsequent resection of the aneurysm.


Assuntos
Aneurisma , Idoso , Aneurisma/diagnóstico por imagem , Aneurisma/cirurgia , Artérias/cirurgia , Humanos , Isquemia/cirurgia , Extremidade Inferior/irrigação sanguínea , Masculino , Resultado do Tratamento
14.
Histopathology ; 78(7): 932-942, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33128780

RESUMO

AIMS: NTRK-rearranged sarcomas are emerging as a distinct class of sarcomas of particular importance in the era of targeted therapy. The aim of this study was to use array comparative genomic hybridisation (aCGH) to explore the cytogenetic profile of six adult soft tissue sarcomas harbouring NTRK gene fusions. METHODS AND RESULTS: aCGH was performed on six adult soft tissue sarcomas with proven NTRK rearrangements [NTRK1, n = 1 (TPM3-NTRK1); NTRK2, n = 1 (MTMR2-NTRK2); NTRK3, n = 4 (two ETV6-NTRK3; two with unknown partners). The morphological patterns of these cases included inflammatory myofibroblastic tumour-like, fibrosarcoma/malignant peripheral nerve sheath tumour-like, and Ewing sarcoma-like. On the basis of the number of chromosomal copy number variations (CNVs), ranging from two to 15 per sample, NTRK-associated sarcomas could be subdivided into two groups: one with a relatively simple karyotype (n = 2; median of three genomic alterations), and those with a more complex karyotype (n = 4; median of 11 genomic imbalances). Recurrent chromosomal CNVs included gains at chromosomes 6p, 1q, 7 (whole chromosome), and 12p, and losses at chromosomes 10q, 13q, 19q, and 9p. CONCLUSIONS: NTRK-rearranged sarcomas constitute a heterogeneous group of tumours that can show a relatively simple or a complex karyotype. Although there were some, but inconsistent, associations between karyotype complexity and morphology, our study showed that a more complex karyotype in this group of tumours appeared to correlate with more aggressive clinical behaviour. Gains at chromosome 6p and 1q were the most common recurrent genomic alterations, being present in 67% of the samples (4/6), followed by gains at chromosome 7, which were present in 50% of the samples (3/6).


Assuntos
Proteínas de Fusão Oncogênica/genética , Receptor trkB/genética , Sarcoma , Adulto , Idoso , Aberrações Cromossômicas , Feminino , Fusão Gênica , Rearranjo Gênico , Genômica/métodos , Humanos , Masculino , Glicoproteínas de Membrana/genética , Pessoa de Meia-Idade , Terapia de Alvo Molecular , Receptor trkA/genética , Sarcoma/genética , Sarcoma/patologia
15.
Blood Adv ; 4(19): 4593-4604, 2020 10 13.
Artigo em Inglês | MEDLINE | ID: mdl-32986791

RESUMO

CD8+CD57+ terminal effector T (TTE) cells are a component of marrow-infiltrating lymphocytes and may contribute to the altered immune responses in multiple myeloma (MM) patients. We analyzed TTE cells in the bone marrow (BM) and peripheral blood (PB) of age-matched controls and patients with monoclonal gammopathy of undetermined significance (MGUS), smoldering MM (SMM), and newly diagnosed (ND) MM using flow cytometry, mass cytometry, and FlowSOM clustering. TTE cells are heterogeneous in all subjects, with BM containing both CD69- and CD69+ subsets, while only CD69- cells are found in PB. Within the BM-TTE compartment, CD69- and CD69+ cells are found in comparable proportions in controls, while CD69- cells are dominant in MGUS and SMM and predominantly either CD69- or CD69+ cells in NDMM. A positive relationship between CD69+TTE and CD69-TTE cells is observed in the BM of controls, lost in MGUS, and converted to an inverse relationship in NDMM. CD69-TTE cells include multiple oligoclonal expansions of T-cell receptor/Vß families shared between BM and PB of NDMM. Oligoclonal expanded CD69-TTE cells from the PB include myeloma-reactive cells capable of killing autologous CD38hi plasma cells in vitro, involving degranulation and high expression of perforin and granzyme. In contrast to CD69-TTE cells, oligoclonal expansions are not evident within CD69+TTE cells, which possess low perforin and granzyme expression and high inhibitory checkpoint expression and resemble T resident memory cells. Both CD69-TTE and CD69+TTE cells from the BM of NDMM produce large amounts of the inflammatory cytokines interferon-γ and tumor necrosis factor α. The balance between CD69- and CD69+ cells within the BM-TTE compartment may regulate immune responses in NDMM and contribute to the clinical heterogeneity of the disease.


Assuntos
Gamopatia Monoclonal de Significância Indeterminada , Mieloma Múltiplo , Mieloma Múltiplo Latente , Medula Óssea , Humanos , Plasmócitos
16.
Skeletal Radiol ; 49(7): 1161-1166, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31960075

RESUMO

Blau syndrome (BS) is a rare autosomal dominant familial granulomatous inflammatory disease presenting in early childhood with dermatitis, arthritis and uveitis. Early-onset sarcoidosis represents the sporadic form, and both are characterised by mutations in the CARD15/NOD2 gene on chromosome 16. We describe a 38-year-old man with known BS who presented for orthopaedic review following right-sided patellar dislocation. MRI of the injured knee demonstrated diffuse synovitis and prominent fatty tissue resembling lipoma arborescens with evidence of recent patellar dislocation. Synovectomy was performed and confirmed granulomatous synovitis. Knee imaging findings are described for the first time. Combining distinct morphological bone changes with synovitis which resembles lipoma arborescens and histology which includes sarcoidal-type granulomatous synovitis should lead the radiologist and pathologist to consider the diagnosis of BS.


Assuntos
Artrite/complicações , Artrite/diagnóstico por imagem , Joelho/diagnóstico por imagem , Sarcoidose/complicações , Sarcoidose/diagnóstico por imagem , Sinovite/diagnóstico por imagem , Sinovite/etiologia , Uveíte/complicações , Uveíte/diagnóstico por imagem , Adulto , Artrite/cirurgia , Diagnóstico Diferencial , Humanos , Imageamento por Ressonância Magnética , Masculino , Sarcoidose/cirurgia , Sinovite/complicações , Sinovite/cirurgia , Uveíte/cirurgia
18.
Front Immunol ; 10: 1596, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31428081

RESUMO

Multiple Myeloma (MM) is preceded by the clinically stable condition monoclonal gammopathy of undetermined significance (MGUS). Critical immune events that discriminate MGUS from newly diagnosed MM (ND)MM patients remain unknown, but may involve changes in the regulatory T cell (Treg) compartment that favor myeloma growth. To address this possibility, we used mass cytometry and the unsupervised clustering algorithm Flow self-organizing map (FlowSOM) to interrogate the distribution of multiple subsets within CD25+CD127low/negTreg in matched bone marrow (BM) and peripheral blood (PB) of MGUS and NDMM patients. Both mass cytometry and flow cytometry confirmed a trend toward prevalence of CD39-Treg within the Treg compartment in BM and PB of NDMM patients compared to CD39-Treg in MGUS patients. FlowSOM clustering displayed a phenotypic organization of Treg into 25 metaclusters that confirmed Treg heterogeneity. It identified two subsets which emerged within CD39-Treg of NDMM patients that were negligible or absent in CD39-Treg of MGUS patients. One subset was found in both BM and PB which phenotypically resembled activated Treg based on CD45RO, CD49d, and CD62L expression; another subset resembled BM-resident Treg based on its tissue-resident CD69+CD62L-CD49d- phenotype and restricted location within the BM. Both subsets co-expressed PD-1 and TIGIT, but PD-1 was expressed at higher levels on BM-resident Treg than on activated Treg. Within BM, both subsets had limited Perforin and Granzyme B production, whilst activated Treg in PB acquired high Perforin and Granzyme B production. In conclusion, the use of mass cytometry and FlowSOM clustering discovered two discrete subsets of CD39-Treg which are discordant in MGUS and NDMM patients and may be permissive of myeloma growth which warrants further study. Understanding the regulatory properties of these subsets may also advance MGUS and MM diagnosis, prognosis, and therapeutic implications for MM patients.


Assuntos
Apirase/imunologia , Gamopatia Monoclonal de Significância Indeterminada/imunologia , Mieloma Múltiplo/imunologia , Linfócitos T Reguladores/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Medula Óssea/imunologia , Feminino , Citometria de Fluxo/métodos , Humanos , Imunofenotipagem/métodos , Antígenos Comuns de Leucócito/imunologia , Masculino , Pessoa de Meia-Idade , Receptor de Morte Celular Programada 1/imunologia , Receptores Imunológicos/imunologia
19.
Asia Pac J Clin Oncol ; 15(1): 38-47, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29806089

RESUMO

AIM: Fluorescence in situ hybridization (FISH) is an important ancillary tool for the classification of bone/soft tissue (BST) tumors. The aim of this study was to evaluate the contribution of FISH to the final classification of common BST entities in the molecular pathology department of the Royal Prince Alfred Hospital (RPAH), which is one of the most important referral centers for the management of sarcomas in Australia. METHODS: All routine diagnostic FISH tests performed on BST formalin-fixed paraffin embedded (FFPE) tissue specimens at the RPAH in a 5-year period (February, 2010-November, 2015) were reviewed. FISH analyses presented in this study include commercial break-apart probes (SS18, FUS, DDIT3, FUS, USP6, PDGFB, TFE3 and ALK) and a single enumeration (MDM2) probe. RESULTS: There were 434 interpretable FISH assays on BST samples including MDM2 (n=180), SS18 (n=97), FUS (n=64), DDIT3 (n=37), USP6 (n=30), PDGFB (n=13), TFE3 (n=8) and ALK (n=5). Discrepancies between the histopathological diagnosis and the FISH results were seen in 12% of the cases. In this subset of discordant cases, FISH contributed to the re-classification of 7% of cases originally diagnosed as synovial sarcoma (SS18) and 6% of adipocytic neoplasms (MDM2) based on the presence or absence of the expected gene alteration. CONCLUSION: Our study confirms that paraffin FISH is a sensitive and specific ancillary tool in the diagnosis of BST neoplasms when used in the appropriate clinicopathological context. These findings highlight the need for further ancillary molecular tools in the diagnosis and characterization of challenging cases.


Assuntos
Variações do Número de Cópias de DNA , Rearranjo Gênico , Hibridização in Situ Fluorescente/métodos , Proteínas de Neoplasias/genética , Neoplasias de Tecidos Moles/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Austrália/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias de Tecidos Moles/epidemiologia , Neoplasias de Tecidos Moles/genética , Centros de Atenção Terciária , Adulto Jovem
20.
Int Orthop ; 43(6): 1283-1295, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30219968

RESUMO

PURPOSE: The purpose of the study is to determine the effectiveness of semi-active and active robotic hip and knee arthroplasty on post-operative patient-reported outcomes of function, pain, quality of life and satisfaction with surgery. METHODS: PubMed, Medline, Embase and CENTRAL were searched. Included were comparative studies investigating the effectiveness of semi-active or active robotic hip or knee arthroplasty compared to any other surgical intervention on function, pain, quality of life and satisfaction with surgery. Risk of bias and the strength of the evidence were assessed using the Downs and Black tool and the GRADE system, respectively. Relative risks, mean differences and 95% CI were calculated using random-effects models. RESULTS: Fourteen studies involving 1342 patients were included. All studies compared robotic to conventional surgery, with active robotic surgery evaluated in total hip or knee arthroplasty and semi-active robotic surgery in total hip or unicompartmental knee arthroplasty. Most studies presented some risk of bias, and the strength of evidence was rated as low to very low quality. Random-effects meta-analyses showed that post-operative functional outcomes were comparable between active robotic and conventional total hip and knee arthroplasty at the short-, medium- and long-term follow-up. No significant difference in pain, quality of life and satisfaction with surgery were reported in individual studies. CONCLUSIONS: This systematic and meta-analyses indicates that functional outcomes for patients undergoing active robotic total hip and knee arthroplasty were comparable to conventional surgery. Whether semi-active or active robotic hip or knee arthroplasty is effective in improving post-operative pain, quality of life and satisfaction with surgery is unclear. PROSPERO Registration Number: CRD42017059932.


Assuntos
Artroplastia de Quadril , Artroplastia do Joelho , Procedimentos Cirúrgicos Robóticos , Artroplastia de Quadril/efeitos adversos , Artroplastia do Joelho/efeitos adversos , Humanos , Dor Pós-Operatória/etiologia , Medidas de Resultados Relatados pelo Paciente , Qualidade de Vida
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