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BACKGROUND: Skeletal deformities (SD) in children and adolescents can lead to arthritic conditions, impairment of quality of life, and high treatment costs in the long term. However, comprehensive data on the prevalence of SDs in children and adolescents are limited and it remains therefore unclear whether there is a healthcare gap. "OrthoKids" is a project that addresses this evidence gap by implementing an orthopaedic screening for children and adolescents that supplements existing detection examinations within statutory standard care in Germany. OBJECTIVE: To detect SDs so that they can be treated as needed at an early stage. METHODS: The implementation of the supplementary orthopaedic screening will be evaluated through an exploratory cohort study that is set up in the German state Baden-Wuerttemberg. 20,000 children and adolescents aged 10 to 14 years will be recruited as a prospective cohort. A retrospective control cohort will be formed based on claims data provided by two cooperating statutory health insurances (SHIs). Participating children and adolescents receive a one-time orthopaedic screening. If at least one SD is diagnosed, treatment will be provided as part of the statutory standard care. Within the scope of the project, a follow-up examination will be performed after one year. An IT-platform will complement the study. The primary outcome measure is the point prevalence of scoliosis, genu varum/valgum, hip dysplasia, and flat feet. Secondary outcome measures are (i) the point prevalence of further less common SDs, (ii) health-related quality of life (HRQoL), (iii) sports ability based on activity (physical/athletic), physical constraints, and (sports) injuries, as well as (iv) monetary consequences of the orthopaedic screenings' implementation. Implementation determinants will be evaluated, too. DISCUSSION: If the supplementary orthopaedic screening proves to be viable, it could be considered as a supplementary examination for children and adolescents within the frame of SHI in Germany. This could relieve the burden of disease among children and adolescents with SDs. In addition, it could disburden SHIs in the medium to long term. TRIAL REGISTRATION: The OrthoKids study was registered in the German Clinical Trials Registry (Deutsches Register Klinischer Studien (DRKS)) on 26th July 2022 under the number 00029057.
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Ortopedia , Adolescente , Criança , Humanos , Estudos de Coortes , Alemanha/epidemiologia , Estudos Prospectivos , Qualidade de Vida , Estudos RetrospectivosRESUMO
PURPOSE: Missed monteggia-type injuries in children can result in chronic radial head dislocation with anatomic changes and osteoarticular remodeling of the radial head. In later stages, joint reconstruction is impossible and a functional radial head distalization can be a therapy option in symptomatic patients. METHODS: From 2010 to 2018, 46 patients (18 female and 28 male, mean age 11.8 (4-20)) with chronic radius head dislocation treated in our institution were retrospectively analyzed. A radial head distalization was performed in symptomatic patients at the time of ulna lengthening and angulation by use of an external ring fixator. We analyzed the surgical and radiographic data as well as the clinical outcome of the patients measured by DASH and Mayo Elbow score. RESULTS: 16 patients (6 female, 10 male) fulfilled the criteria for functional radial head distalization. Main reason was Monteggia injury in 11 cases, and radial head fracture in 5 cases. Average follow-up was 5.1 years (range 1-9, SD 2.1). Mean time from injury was 4.14 years (range: 4 months to 12 years, SD 3.5 years). Mean duration of external fixation was 106 days (range 56-182, SD 31.2), lengthening was 21.3 mm (range 12-42, SD 8.8). Average degree of sagittal angulation 14.8° (0-32°, SD 10.7°), coronal angulation 4.4° (0-25°, SD 7.3°). DASH score showed a good result with 2.4, and the MAYO Elbow Score was excellent (95/100). No secondary luxation of the radius head was detected. CONCLUSION: Radial head distalization with external ring fixator can be a therapy option for chronic radius head dislocations in symptomatic patients without losing stability of the elbow joint in contrast to radial head resection.
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Articulação do Cotovelo , Luxações Articulares , Fratura de Monteggia , Humanos , Masculino , Criança , Feminino , Rádio (Anatomia)/diagnóstico por imagem , Rádio (Anatomia)/cirurgia , Rádio (Anatomia)/lesões , Articulação do Cotovelo/diagnóstico por imagem , Articulação do Cotovelo/cirurgia , Fratura de Monteggia/diagnóstico por imagem , Fratura de Monteggia/cirurgia , Fratura de Monteggia/complicações , Estudos Retrospectivos , Ulna/cirurgia , Luxações Articulares/diagnóstico por imagem , Luxações Articulares/cirurgiaRESUMO
INTRODUCTION: Septic arthritis of the hip can appear isolated or concomitant with pelvic osteomyelitis. Delay in the diagnosis of a concomitant osteomyelitis increases the number of required surgeries and of possible complications. PURPOSE: This study aims to establish relevant factors in the diagnosis of concomitant osteomyelitis in cases with septic arthritis of the hip among paediatric patients. METHODS: The data were collected between 2005 and 2020. 41 pediatric patients with suspicion of septic arthritis of the hip joint, treated arthroscopically, were included. The following diagnostic test parameters were collected: ultrasound, MRI, X-Rays, blood samples, temperature, and incapacity to bear weight. The data were analysed with the sensitive analysis method using descriptive statistic. RESULTS: 41 patients were analyzed, with an average age of 6.04 y (7 months to 14 years), of which ten patients (24.39%) presented concomitant osteomyelitis. 6 out of ten patients needed secondary surgery. Regarding age, concomitant osteomyelitis was most common in the age group 4-14 years old. Average number of days of clinical symptoms before admission was 6.2 days. 36/41 cases showed CRP values higher than 2 mg/dl. 9/10 cases with concomitant osteomyelitis showed a CRP > 2 mg/dl, with an average value of 8.9 mg/dl. 22/41 patients underwent an MRI, of which nine cases presented a concomitant osteomyelitis. The probability of a child to have septic arthritis of the hip with adjacent osteomyelitis was analysed through a score based on four factors: impossibility to bear weight and/or hip pain in children in the non-walking age category, CRP > 2 mg/dl, age older than > 4 y, symptoms longer than 4 days. CONCLUSIONS: Children at the age of walking, with incapacity to bear weight, presenting symptoms longer than 4 days and a CRP > 2 mg/dl, should receive an MRI before surgery to exclude adjacent osteomyelitis. LEVEL OF EVIDENCE: IV.
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Artrite Infecciosa , Osteomielite , Criança , Humanos , Pré-Escolar , Adolescente , Recém-Nascido , Artroscopia/métodos , Radiografia , Imageamento por Ressonância Magnética , Artrite Infecciosa/complicações , Artrite Infecciosa/diagnóstico , Artrite Infecciosa/cirurgia , Osteomielite/complicações , Osteomielite/diagnóstico , Osteomielite/cirurgiaRESUMO
BACKGROUND: Despite recent progress in the field of genetics, sporadic late-onset (> 40 years) cerebellar ataxia (SLOCA) etiology remains frequently elusive, while the optimal diagnostic workup still needs to be determined. We aimed to comprehensively describe the causes of SLOCA and to discuss the relevance of the investigations. METHODS: We included 205 consecutive patients with SLOCA seen in our referral center. Patients were prospectively investigated using exhaustive clinical assessment, biochemical, genetic, electrophysiological, and imaging explorations. RESULTS: We established a diagnosis in 135 (66%) patients and reported 26 different causes for SLOCA, the most frequent being multiple system atrophy cerebellar type (MSA-C) (41%). Fifty-one patients (25%) had various causes of SLOCA including immune-mediated diseases such as multiple sclerosis or anti-GAD antibody-mediated ataxia; and other causes, such as alcoholic cerebellar degeneration, superficial siderosis, or Creutzfeldt-Jakob disease. We also identified 11 genetic causes in 20 patients, including SPG7 (n = 4), RFC1-associated CANVAS (n = 3), SLC20A2 (n = 3), very-late-onset Friedreich's ataxia (n = 2), FXTAS (n = 2), SCA3 (n = 1), SCA17 (n = 1), DRPLA (n = 1), MYORG (n = 1), MELAS (n = 1), and a mitochondriopathy (n = 1) that were less severe than MSA-C (p < 0.001). Remaining patients (34%) had idiopathic late-onset cerebellar ataxia which was less severe than MSA-C (p < 0.01). CONCLUSION: Our prospective study provides an exhaustive picture of the etiology of SLOCA and clues regarding yield of investigations and diagnostic workup. Based on our observations, we established a diagnostic algorithm for SLOCA.
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Ataxia Cerebelar , Atrofia de Múltiplos Sistemas , Ataxias Espinocerebelares , Degenerações Espinocerebelares , Humanos , Estudos Prospectivos , Ataxia Cerebelar/epidemiologia , Ataxia Cerebelar/etiologia , Ataxia Cerebelar/diagnóstico , Degenerações Espinocerebelares/complicações , Ataxias Espinocerebelares/complicações , Atrofia de Múltiplos Sistemas/complicações , Proteínas Cotransportadoras de Sódio-Fosfato Tipo IIIRESUMO
OBJECTIVE: Open reduction of congenital hip dislocations currently remains the standard treatment for those hip joints which are irreducible by closed means. The open reduction of the dislocated hip joint represents a relatively invasive surgical method. Thus, the goal was to develop a minimally invasive and safe procedure with a lower complication rate as an alternative to open reduction. This work presents the arthroscopically guided reduction of dislocated hip joints, first described in 2009, as a standardized surgical technique. INDICATIONS: Failed closed reduction for congenital hip dislocation. SURGICAL TECHNIQUE: Arthroscopic reduction of the dislocated femoral head using an arthroscopic two-portal technique, a high anterolateral and a medial subadductor portal. The arthroscope is inserted through the subadductor portal. The high anterolateral portal serves as working portal. Step-by-step identification and removal of obstacles to reduction such as the ligament of the femoral head, fat tissue, capsular constriction and psoas tendon. Reduction of the femoral head under arthroscopic control. POSTOPERATIVE TREATMENT: The hip joint is retained in a hip spica cast with the legs in human position. RESULTS: Arthroscopic hip reduction of 20 congenital hip dislocations: 13 girls and 3 boys with an average age at the time of operation of 5.8 months (3-9 months). All children had multiple, unsuccessful attempts of closed reduction by use of overhead traction, Pavlik harness or closed reduction and hip spica application. According to the Graf classification, there were 20 type IV hips. According to the radiological classification of Tönnis, there were 9 type 4, 7 type 3, and 4 type II grades. The obstacles to reduction were capsular constriction, hypertrophic ligament of the femoral head, and an extensively large pulvinar in the acetabulum. An inverted labrum was not seen in any of the cases. In contrast, in 2/3 of the cases, there was considerable retraction of the dorsal edge of the socket due to the ligament of the femoral head expanding right over it. In all cases, postreduction transinguinal ultrasound and MRI were used to check the femoral head position in the cast postoperatively. In all cases there was a deep reduction of the femoral head in the acetabulum. There were no intra- or postoperative complications such as bleeding, infections or nerve lesions. There were no cases of redislocation or decentering of the femoral head, which was also confirmed after an average follow-up of 15 months. The mean AC angle at follow-up was 24.5°. There was one coxa magna in the series and one avascular necrosis with a fragmented femoral head according to the Salter classification.
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Luxação Congênita de Quadril , Luxação do Quadril , Acetábulo , Criança , Feminino , Luxação do Quadril/diagnóstico por imagem , Luxação do Quadril/cirurgia , Luxação Congênita de Quadril/cirurgia , Articulação do Quadril/diagnóstico por imagem , Articulação do Quadril/cirurgia , Humanos , Lactente , Masculino , Resultado do TratamentoRESUMO
BACKGROUND: Little is known about outcome and settings adaptations after replacement of constant-voltage non-rechargeable implantable pulse generator (CV-nrIPG) by constant-current rechargeable IPG (CC-rIPG). OBJECTIVE: To determine the feasibility and safety of replacing a CV-nrIPG by a CC-rIPG in Parkinson's disease (PD) and the subsequent outcome. METHODS: A prospective cohort of thirty PD patients, whose CV-nrIPG was replaced by a CC-rIPG in University Hospital of Lyon between January 2017 and December 2018 (rIPG group) and 39 PD patients, who underwent the replacement of a CV-nrIPG by the same device in 2016 (nrIPG group), were enrolled in this study. Three surgeons performed the operations. Duration of hospitalization for the replacement as well as the number of in or outpatient visits during the first 3 months after the surgery were recorded. In the rIPG group, we compared preoperative DBS settings and the theoretical amplitude estimated using Ohm's law to the amplitude used at the end of follow-up. We assessed patients' and clinicians' opinion on the patient global functioning after the replacement using Clinical Global Impression score. RESULTS: Duration of hospitalization (P=0.47) and need for additional hospitalizations (P=0.73) or consultations (P=0.71) to adapt DBS parameters did not differ between the two groups. Neurological condition (CGI score) was considered as unchanged by both patients and neurologists. Final amplitude of stimulation using CC-rIPG was not predicted by Ohm's law in most cases. CONCLUSIONS: Replacing CV-nrIPG by CC-rIPG is safe and well tolerated but require neurological expertise to set the new parameters of stimulation.
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Doença de Parkinson , Estimulação Encefálica Profunda , Eletrodos Implantados , Estudos de Viabilidade , Humanos , Doença de Parkinson/terapia , Estudos ProspectivosRESUMO
Hepatocellular carcinoma (HCC) is one of the most common and most lethal cancers worldwide. Treatment of patients with HCC is complicated by the underlying liver disease in up to 80% of all cases. Interdisciplinary and multimodal treatment strategies are essential for successful therapy. Established therapies include surgical interventions (liver transplantation, resection), local ablative therapies (e.g., microwave or radiofrequency ablation), and locoregional therapies (e.g., transarterial chemoembolization [TACE] and selective internal radiotherapy [SIRT/TARE]). Moreover, there are emerging opportunities for systemic therapies. While the multityrosine kinase inhibitor sorafenib has been the only agent approved for patients with unresectable HCC for almost a decade, there are now additional systemic treatment options including the tyrosine kinase inhibitors (TKIs) lenvatinib, regorafenib, and cabozantinib as well as the VEGF-receptor inhibitor ramucirumab. To date, immune checkpoint inhibitor monotherapies have failed to show an overall survival benefit, but according to recent data combined immunotherapy/VEGF inhibition has shown superior activity in first-line treatment compared with sorafenib. The advent of numerous novel systemic agents offers a variety of combination opportunities. Combinations of systemic agents with locoregional treatments in palliative and potentially curative settings are currently being investigated. Today, treatment of patients with HCC is more challenging than ever owing to the multiple therapeutic options available, demanding strict multidisciplinary cooperation in the treatment selection process. There is an urgent need for clinical studies in order to further optimize the therapy sequence and to identify efficacious mono- and combination therapies.
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Carcinoma Hepatocelular , Quimioembolização Terapêutica , Neoplasias Hepáticas , Carcinoma Hepatocelular/terapia , Terapia Combinada , Humanos , Neoplasias Hepáticas/terapia , Transplante de FígadoRESUMO
Benign liver tumors form a heterogeneous group. The most frequent forms include simple cysts, hemangiomas, focal nodular hyperplasia and hepatocellular adenomas. They are often incidentally detected during routine sonography. The diagnosis of a liver tumor not uncommonly causes anxiety and insecurity in those affected, which is why a rapid and reliable diagnostic procedure should be carried out. Because some tumors, particularly hepatocellular adenomas, are of prognostic relevance due to the potential risk of malignant transformation, a correct classification should always be strived for. The type and extent of diagnostic clarification depend on the clinical and patient-related risk factors. This article describes the most important benign space-occupying lesions. The etiology, clinical manifestations and diagnostics as well as possible necessary treatment measures are presented.
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Cistos , Hiperplasia Nodular Focal do Fígado , Hemangioma , Hepatopatias , Neoplasias Hepáticas , Cistos/diagnóstico , Cistos/terapia , Diagnóstico Diferencial , Hiperplasia Nodular Focal do Fígado/diagnóstico , Hiperplasia Nodular Focal do Fígado/terapia , Hemangioma/diagnóstico , Hemangioma/terapia , Humanos , Hepatopatias/diagnóstico , Hepatopatias/terapia , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/terapiaRESUMO
PURPOSE: Children with sonographic grade IV hip dysplasia according to Graf and with failed conservative treatment usually need surgical reduction afterwards. Surgical reduction of the hip can lead to severe complications, the occurrence of residual acetabular dysplasia, osteonecrosis, redislocation and other postoperative complications. This paper investigates whether arthroscopic reduction is a promising alternative to open reduction. METHODS: We retrospectively examined 66 patients (78 hips) who were not older than two years at the first time of surgery. Arthroscopic reduction was performed on 17 children (19 hips) and open reduction on 49 children (59 hips). Patient records were used to determine redislocation, postoperative complication and residual dysplasia. Radiographs were used to determine Tönnis classification for osteonecrosis and pathological acetabular (AC) angle for residual dysplasia. We considered data up to a two-year follow-up. Statistical evaluation was performed with binary logistic regression. RESULTS: After arthroscopic reduction, 6% showed osteonecrosis, compared with 20% with open reduction (p = 0.334). Redislocation was not observed after arthroscopic reduction but for 29% after open reduction (p = 0.005). An improvement of femoral head coverage was achieved with residual dysplasia of 23.5% after arthroscopic reduction, compared with 62% after open reduction (p = 0.002). CONCLUSION: The arthroscopic procedure represents a meaningful alternative to the open procedure due to a lower complication rate, a safe setting, a lower rate of residual dysplasia, no observed redislocation and occurrence of osteonecrosis only once in the arthroscopic group of developmental dysplasia of the hip. The arthroscopic procedure should be tested in further studies and in other clinics in order to broaden the empirical base. LEVEL OF EVIDENCE: Level III (retrospective cohort study).
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BACKGROUND: Hip dysplasia is one of the most common disorders in paediatric orthopedics. With the development of ultrasound techniques treatment has changed, and earlier diagnosis and treatment is possible. So, for more than 20 years diagnosis and treatment has been focused on ultrasound. Many papers reporting new diagnostic techniques like MRI have been published. Arthroscopic reduction is a new surgical procedure. However, established surgical procedures continue to play an important role in the treatment. CURRENT TREATMENT: This paper presents new and established diagnostic techniques, conservative and surgical treatment options. Unfortunately, due to the great number of publications, not all aspects could be taken into account. In particular, we describe the diagnostic and treatment standards in German-speaking countries based on ultrasound hip screening. However, also international aspects of diagnostic and treatment options have been included.
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Luxação Congênita de Quadril , Luxação do Quadril , Artroscopia , Criança , Humanos , Ortopedia , Procedimentos de Cirurgia Plástica , UltrassonografiaRESUMO
In slipped capital femoral epiphysis (SCFE) atypical causes can be found in about 5% of cases. The patients' age differs from the typical age of SCFE patients of between age 10 and 16. There are numerous causes of atypical SCFE: different endocrine disorders, sequelae of chronic renal failure and effects of radiation and chemotherapy on the proximal femoral physis. The diagnostic and therapeutic procedures must be expanded to the search for and treatment of endocrinological disorders. Orthopaedic treatment follows the principles applied to patients with idiopathic SCFE. However, the specific treatment modalities which arise from different patient ages and bone qualities are taken into account.
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Escorregamento das Epífises Proximais do Fêmur , Progressão da Doença , Fêmur , Terapia de Reposição Hormonal , HumanosRESUMO
PURPOSE: Diseases such as osteogenesis imperfecta, fibrous dysplasia, hypophosphataemic rickets and others lead to soft and weak bones and long bone deformity in affected patients. As a consequence, these patients lose their walking capacity and functional abilities of the upper extremities as well. METHODS: In combination with bisphosphonate treatment and physical rehabilitation programmes surgical interventions are being applied to correct and stabilize the deformed and less mechanically resistant long bones. Intramedullary devices, ideally with an elongating telescopic mechanism, have proven to be the most suitable implants. RESULTS: The surgical correction and stabilization of deformed bones in weak bone diseases is very beneficial to the patients. Pain restriction, reduction of fracture events, minimization of consequences of traumatic events and falls have resulted in a significant functional improvement. The patients live on a better activity level with a much-improved individual independence. Despite a high complication and revision rate of the intramedullary rods the gain of quality of life outweighs these negative aspects by far. CONCLUSION: Orthopaedic surgical treatment for deformed bones in patients with weak bone diseases has changed the life of the patients and plays a very important role in the disease management protocols.This paper was written under the guidance of the Study Group Genetics and Metabolic Diseases of the European Paediatric Orthopaedic Society.
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The medial condyle fracture of the humerus is-in comparison to the lateral condyle fracture-a very rare Salter-Harrison IV-fracture of the elbow. In this prospective study 14 children were included and reviewed. One child had minimal displacement fracture type I, one child had type II, and 12 children had type III-fractures. One patient was treated conservatively by an upper arm cast; thirteen were surgically treated using open reduction and osteosynthetical treatment. Postoperatively the elbow was immobilized in 90° flexion and neutral position in a long-arm cast for 4-6 weeks. In 11 children the diagnosis was made immediately after trauma, in 3 children the fracture was overlooked initially. Medial condyle fractures may be difficult to diagnose in children younger than 6 years and the lesion may be mistaken for a simple avulsion of the medial epicondyle or even missed. The C-sign is a hint for a medial condyle fracture. The development of nonunion happens in consequence of failure to recognize the fractures. Results after an average follow-up of 36 months showed that children who were diagnosed immediately and received operative stabilization had very good functional and aesthetical results. Three children with delayed diagnosis of the fracture had open surgery with reposition and osteosynthetical fixation. In two of the overlooked cases a slight contracture and angular misalignment persisted. If in this injury the diagnosis is made without delay, an appropriate therapy is implemented and radiographical controls are performed until consolidation, good results can be expected. The main risk in medial condyle fractures of the humerus is to overlook them. This can lead to the development of a nonunion with joint malformations.
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Fraturas do Úmero/terapia , Adolescente , Placas Ósseas , Parafusos Ósseos , Moldes Cirúrgicos , Criança , Pré-Escolar , Diagnóstico Tardio , Feminino , Fixação Interna de Fraturas/métodos , Humanos , Fraturas do Úmero/diagnóstico , Masculino , Estudos Prospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Herpes simplex encephalitis (HSE) is the most frequent sporadic encephalitis in the world. In severe cases of HSE, the pathology usually progresses with an increase in intracranial pressure secondary to cerebral edema and/or hemorrhagic necrosis. Currently no high-power studies exist regarding the management of severe HSE and most of the papers reported in the literature are case reports. Decompressive craniectomy, effective in some cases of pharmaco-resistant intracranial hypertension (ICH) resulting from other causes, may be suggested in severe HSE, with several good results reported in the literature. CASE DESCRIPTION: The case of a 26-year-old man with severe HSE and a subsequent ICH is reported. In dealing with an ICH rebellious to conservative treatment, it was decided to perform a right decompressive hemicraniectomy, associated with a right temporal polectomy. The postoperative evolution was satisfactory, with normal neuropsychological tests and a Glasgow Outcome Scale of 1. CONCLUSION: Although herpes simplex encephalitis is sometimes devastatingly complicated by intracranial hypertension, its management lacks consensus and reliable data in the literature remains scarce. Surgical as well as conservative treatment, used together in a multimodal approach, may hold the key to a greater control of intracranial pressure, thus resulting in a better outcome. In this multimodal management, the window of opportunity where surgery may be considered is small, and must be discussed further and more precisely in future articles.
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Edema Encefálico/cirurgia , Encefalite por Herpes Simples/cirurgia , Encefalite por Herpes Simples/virologia , Hipertensão Intracraniana/cirurgia , Adulto , Edema Encefálico/diagnóstico , Craniectomia Descompressiva/métodos , Encefalite por Herpes Simples/diagnóstico , Humanos , Hipertensão Intracraniana/etiologia , Pressão Intracraniana/fisiologia , Masculino , Procedimentos Neurocirúrgicos/efeitos adversosRESUMO
The management of sporadic late-onset cerebellar ataxias represents a very heterogeneous group of patients and remains a challenge for neurologist in clinical practice. We aimed at describing the different causes of sporadic late-onset cerebellar ataxias that were diagnosed following standardized, exhaustive investigations and the population characteristics according to the aetiologies as well as at evaluating the relevance of these investigations. All patients consecutively referred to our centre due to sporadic, progressive cerebellar ataxia occurring after 40 years of age were included in the prospective, observational study. 80 patients were included over a 2 year period. A diagnosis was established for 52 patients (65%) corresponding to 18 distinct causes, the most frequent being cerebellar variant of multiple system atrophy (n = 29). The second most frequent cause was inherited diseases (including spinocerebellar ataxias, late-onset Friedreich's disease, SLC20A2 mutations, FXTAS, MELAS, and other mitochondrial diseases) (n = 9), followed by immune-mediated or other acquired causes. The group of patient without diagnosis showed a slower worsening of ataxia (p < 0.05) than patients with multiple system atrophy. Patients with later age at onset experienced faster progression of ataxia (p = 0.001) and more frequently parkinsonism (p < 0.05) than patients with earlier onset. Brain MRI, DaT scan, genetic analysis and to some extent muscle biopsy, thoracic-abdominal-pelvic tomodensitometry, and cerebrospinal fluid analysis were the most relevant investigations to explore sporadic late-onset cerebellar ataxia. Sporadic late-onset cerebellar ataxias should be exhaustively investigated to identify the underlying causes that are numerous, including inherited causes, but dominated by multiple system atrophy.
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Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/etiologia , Atrofia de Múltiplos Sistemas/complicações , Adulto , Idade de Início , Idoso , Encéfalo/diagnóstico por imagem , Canais de Cálcio/genética , Ataxia Cerebelar/genética , Ataxia Cerebelar/patologia , Eletromiografia , Feminino , Ataxia de Friedreich/complicações , Humanos , Estudos Longitudinais , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Atrofia de Múltiplos Sistemas/diagnóstico por imagem , Mutação/genética , Condução Nervosa/fisiologia , Exame Neurológico , Proteínas Proto-Oncogênicas c-sis/genética , Receptor beta de Fator de Crescimento Derivado de Plaquetas/genética , Receptores Acoplados a Proteínas G/genética , Receptores Virais/genética , Estudos Retrospectivos , Índice de Gravidade de Doença , Ataxias Espinocerebelares/complicações , Estatísticas não Paramétricas , Receptor do Retrovírus Politrópico e XenotrópicoAssuntos
Carcinoma Hepatocelular , Hepacivirus , Antivirais , Quimioprevenção , Humanos , Neoplasias HepáticasRESUMO
Hepatitis C infection represents a global health problem affecting â¼200 million chronically infected patients worldwide. Owing to the development of a fibrogenic and inflammatory micromilieu in the liver, hepatitis C virus (HCV)-infected patients are at a high risk of developing fibrosis, cirrhosis and hepatocellular carcinoma (HCC). The advent of direct-acting antiviral agents (DAAs), however, has spurred a revolution in the treatment of HCV patients with sustained viral response (SVR) rates exceeding 90% in real-life settings. Recent clinical trials suggest that these novel treatments will not only alter the epidemiology of HCV infection but also the incidence of HCV-induced complications including hepatic decompensation, liver transplantation and hepatocarcinogenesis. Here, we summarize data from clinical trials carried out in HCV patients with compensated and decompensated cirrhosis and analyze the impact of viral clearance on HCC development and treatment. Finally, we review and discuss current and future treatment options of HCV patients with HCC in pre- and post-transplantation settings.
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Antivirais/uso terapêutico , Carcinoma Hepatocelular/tratamento farmacológico , Hepatite C/tratamento farmacológico , Neoplasias Hepáticas/tratamento farmacológico , Carcinoma Hepatocelular/complicações , Carcinoma Hepatocelular/epidemiologia , Carcinoma Hepatocelular/virologia , Ensaios Clínicos como Assunto , Hepacivirus/patogenicidade , Hepatite C/complicações , Hepatite C/epidemiologia , Hepatite C/virologia , Humanos , Fígado/patologia , Fígado/virologia , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/epidemiologia , Neoplasias Hepáticas/virologiaRESUMO
BACKGROUND: Frequently, the first clinical sign of colorectal cancer is complete obstruction, which has to be considered an emergency situation. The treatment goal is to overcome ileus including reduction of the associated high morbidity and mortality. Therefore, alternative therapeutic options to emergency surgery have been sought in order to allow adequate preparation for elective surgery or stabilization of palliative patients and avoid colostoma. Therapeutic results of the placement of self-expanding metal stents (SEMS) are discussed in terms of a single-center, retrospective observational study. METHODS: In 35 patients with a clinically manifest stenosis of colonic cancer, it was attempted to insert SEMS to treat ileus as the first therapeutic step. Therapeutic results were investigated with regard to technical and clinical success, spectrum and rate of complications, and survival time, differentiating between a palliative and curative group of patients. RESULTS: Primary placement of a stent was achieved in 29 of 35 patients (82.9 %); 14 underwent the procedure with palliative and 15 with curative intention. Stent location was mainly the left hemicolon. In 2 of 15 patients (13.3 %), emergency surgery within 48 h was required because of complications, whereby in 13 patients (86.7 %), 6 patients (46.2 %) underwent elective open surgery and 7 patients (53.8 %) underwent a laparoscopic procedure. In all patients treated with curative intent, primary anastomosis was achieved, thus, avoiding a colostoma. Survival times of the palliative and curative patient groups were on average 7 and 28 months, respectively. CONCLUSION: In carefully selected cases, placement of SEMS in malignant stenosis of the left hemicolon with ileus can be considered a reasonable therapeutic alternative to emergency surgery since it allows surgical intervention with curative intention under more favorable conditions and also avoids a colostoma.
Assuntos
Doenças do Colo/terapia , Neoplasias Colorretais/terapia , Serviços Médicos de Emergência , Obstrução Intestinal/terapia , Stents , Idoso , Idoso de 80 Anos ou mais , Neoplasias Colorretais/complicações , Neoplasias Colorretais/mortalidade , Procedimentos Cirúrgicos Eletivos , Feminino , Alemanha , Humanos , Obstrução Intestinal/mortalidade , Laparoscopia , Masculino , Pessoa de Meia-Idade , Cuidados Paliativos , Retratamento , Estudos Retrospectivos , Fatores de Risco , Análise de SobrevidaRESUMO
OBJECTIVE: The treatment goal is to rapidly make a diagnosis and establish an effective arthroscopic therapy in order to allow the hip joint to develop normally throughout childhood with respect to anatomical and functional development. INDICATIONS: Coxarthritis suspected of being septic arthritis CONTRAINDICATIONS: No experience with arthroscopy, osteomyelitis of the dorsal femoral neck requiring an intervention. Relative contraindication: absence of cannulated systems. SURGICAL TECHNIQUE: Arthroscopic lavage of the hip joint, including revision of the femoral neck and debridement of osteomyelitis of the femoral neck with placement of an antibiotic carrier if necessary. The arthroscopy is performed using the two portal technique, placing the patient supine on a normal operation table (traction table not required). POSTOPERATIVE MANAGEMENT: Drain for 2-3 days with mobilization after removal of the drainage. A second look arthroscopy is not normally planned but may become necessary in cases without improvement of the clinical or laboratory test parameters and after follow-up magnetic resonance imaging (MRI). RESULTS: The two portal hip joint arthroscopy using an irrigation volume of 6-9 l is an efficient minimally invasive method to safely treat septic arthritis of the hip joint with or without concomitant femoral neck osteomyelitis. It is associated with low morbidity and offers all advantages of an arthroscopic procedure. Out of 23 children 19 could be sufficiently treated by a single arthroscopic lavage of the hip joint. In three patients an additional osseous component required a second intervention. Out of 23 children 22 achieved an excellent Harris hip score, with one girl only achieving a moderate outcome. The 22 children had an unrestricted hip function at follow-up and could return fully to previous activity levels.