RESUMO
OBJECTIVES: Since 2005, a New Wilson Index (NWI) ≥11 is used as a predictor of death without transplantation in fulminant Wilson disease (WD). Plasma exchange is advocated as a new treatment modality. METHODS: We present a patient with fulminant WD treated with plasma exchange. All published cases applying plasma exchange for fulminant WD were reviewed systematically. RESULTS: A 14-year-old girl presented with hemolysis and fulminant liver failure. She had no encephalopathy; NWI was 14. As a bridge to transplantation plasma exchange was started immediately. Complete remission was achieved with plasma exchange and later chelation therapy with D-penicillamine. She is now at 3-year transplant-free survival. Literature review identified 37 patients presenting with fulminant WD and NWI ≥11 who were treated with plasma exchange. Seventeen of these patients (ie, 46%) recovered without transplantation. CONCLUSIONS: Multiple case reports and case series demonstrate transplant free survival after plasma exchange and subsequent chelation therapy, despite a NWI ≥11. Plasma exchange affects the clinical course and is a therapeutic option in children and young adults presenting with fulminant WD.
Assuntos
Degeneração Hepatolenticular , Falência Hepática Aguda , Transplante de Fígado , Troca Plasmática , Adolescente , Criança , Feminino , Degeneração Hepatolenticular/terapia , Humanos , Falência Hepática Aguda/terapia , Penicilamina , Adulto JovemRESUMO
Changes in coronal alignment in osteoarthritic knees evolving from extension to flexion remain poorly studied. Using an imageless computer-navigation system (Stryker©) we prospectively collected measurements of dynamic coronal pre-implant alignment during primary total knee arthroplasty. Coronal alignment of the osteoarthritic knee was determined at maxi- mal extension and 90° flexion. Measurements were subgrouped as varus (≤-3°), neutral (>-3°, <+3°) or valgus (≥+3°). Of 545 osteoarthritic knees (347 females), coronal alignment in extension was 261 (48%) varus, 197 (36%) neutral and 87 (16%) valgus. Varus extension alignment was more common in male versus female knees (p< .0001). Valgus ex- tension alignment was more common in female versus male knees (p= .002). In flexion, 174 (66%) of varus knees remained varus. Coronal alignment remained unchanged (within +3° ; -3°) in flexion versus extension in approximately half of the OA knees observed. This insight into a changing coronal deformity might contribute to a better understanding of osteoarthritic knee behaviour.
Assuntos
Artroplastia do Joelho/métodos , Osteoartrite do Joelho/fisiopatologia , Osteoartrite do Joelho/cirurgia , Amplitude de Movimento Articular/fisiologia , Cirurgia Assistida por Computador/métodos , Idoso , Idoso de 80 Anos ou mais , Fenômenos Biomecânicos , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
BACKGROUND: During ambulatory follow-up of patients with cerebral palsy (CP) systematic radiographic screening is required firstly to evaluate hip migration and development in the prevention of hip dislocation and secondly to analyse lower limb alignment and leg length. The Migration Percentage (MP) is a radiographic measurement used to describe the extent of femoral head lateralisation on conventional supine pelvic radiographs. Our goal was to assess the comparability of the MP measured on low radiation dose EOS® standing full-leg radiographs with that of conventional supine pelvic radiographs. METHODS: Patients presenting with CP were prospectively selected from our outpatient follow-up consultation at our institutions CP reference centre and underwent conventional supine pelvic and EOS® standing full-leg radiographs the same day for diagnostic and screening reasons. RESULTS: Out of 28 prospectively selected patients we included 21 (42 hips), of which 10 were female, with a mean age of 9.25 years and GMFCS levels of I, II and III. Seven out of 28 patients were excluded due to insufficient quality of radiographic images. The absolute differences in MP measured on both conventional supine pelvic and EOS® standing full-leg radiographs ranged between - 8 and 6% with an absolute mean difference of 0% (SD ±3.5) and were not statistically significant (p = 0.99). A Bland-Altman plot showed acceptable agreement between both measurements without proportional bias. CONCLUSION: There is no statistical significant difference between the Migration Percentage measured on conventional supine pelvic radiographs and EOS® standing full-leg radiographs in ambulant patients. These images use lower radiation doses and contain more radiographic information. TRIAL REGISTRATION: Approved by the Medical Research Ethics committee of the University Hospitals Leuven ( MP001492 ).
Assuntos
Paralisia Cerebral/complicações , Luxação do Quadril/diagnóstico por imagem , Criança , Feminino , Seguimentos , Luxação do Quadril/etiologia , Humanos , Extremidade Inferior/diagnóstico por imagem , Masculino , Ossos Pélvicos/diagnóstico por imagem , Estudos Prospectivos , Radiografia , Estudos Retrospectivos , Decúbito DorsalRESUMO
A growing number of patients undergoing total knee arthroplasty (TKA) is at working age and need to return to work (RTW) after surgery. The aim of this systematic review is to give an overview of the literature regarding RTW after TKA and beneficial and limiting factors influencing this process. A systematic search in four electronic databases was conducted in November 2019 to identify studies describing RTW after primary TKA in patients aged 65 years or younger. Study characteristics and data on work status before and after surgery were extracted. All studies were assessed for risk of bias. Fourteen studies published between 2009 and 2019 were included in this review, accounting for a total of 3,073 patients. The percentage of patients working after TKA ranged from 36 to 89%, and the fraction of patients working before and returning to work after surgery ranged from 40 to 98%. Mean time of RTW ranged from 7.7 to 16.6 weeks. Most important factors associated with a slower or no RTW were a more physical nature of employment and preoperative absence from work. The majority of patients undergoing TKA returned to work postoperatively. However, comparison between studies is seriously hampered by the wide variation regarding the definition and timeframe used to measure the work status. Therefore, standardized outcome measures for studies investigating RTW after TKA are warranted. We identify this review as level-I evidence (systematic review of level-I and level-II studies).