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[PMID]: 28323974 [Au] Autor: Webb EA; Balasubramanian M; Fratzl-Zelman N; Cabral WA; Titheradge H; Alsaedi A; Saraff V; Vogt J; Cole T; Stewart S; Crabtree NJ; Sargent BM; Gamsjaeger S; Paschalis EP; Roschger P; Klaushofer K; Shaw NJ; Marini JC; Högler W [Ad] Endereço: Department of Endocrinology and Diabetes, Birmingham Children's Hospital, Birmingham B4 6NH, United Kingdom. [Ti] Título: Phenotypic Spectrum in Osteogenesis Imperfecta Due to Mutations in TMEM38B: Unraveling a Complex Cellular Defect. [So] Source: J Clin Endocrinol Metab;102(6):2019-2028, 2017 Jun 01. [Is] ISSN: 1945-7197 [Cp] País de publicação: United States [La] Idioma: eng [Ab] Resumo: Context: Recessive mutations in TMEM38B cause type XIV osteogenesis imperfecta (OI) by dysregulating intracellular calcium flux. Objectives: Clinical and bone material phenotype description and osteoblast differentiation studies. Design and Setting: Natural history study in pediatric research centers. Patients: Eight patients with type XIV OI. Main Outcome Measures: Clinical examinations included bone mineral density, radiographs, echocardiography, and muscle biopsy. Bone biopsy samples (n = 3) were analyzed using histomorphometry, quantitative backscattered electron microscopy, and Raman microspectroscopy. Cellular differentiation studies were performed on proband and control osteoblasts and normal murine osteoclasts. Results: Type XIV OI clinical phenotype ranges from asymptomatic to severe. Previously unreported features include vertebral fractures, periosteal cloaking, coxa vara, and extraskeletal features (muscular hypotonia, cardiac abnormalities). Proband lumbar spine bone density z score was reduced [median -3.3 (range -4.77 to +0.1; n = 7)] and increased by +1.7 (1.17 to 3.0; n = 3) following bisphosphonate therapy. TMEM38B mutant bone has reduced trabecular bone volume, osteoblast, and particularly osteoclast numbers, with >80% reduction in bone resorption. Bone matrix mineralization is normal and nanoporosity low. We demonstrate a complex osteoblast differentiation defect with decreased expression of early markers and increased expression of late and mineralization-related markers. Predominance of trimeric intracellular cation channel type B over type A expression in murine osteoclasts supports an intrinsic osteoclast defect underlying low bone turnover. Conclusions: OI type XIV has a bone histology, matrix mineralization, and osteoblast differentiation pattern that is distinct from OI with collagen defects. Probands are responsive to bisphosphonates and some show muscular and cardiovascular features possibly related to intracellular calcium flux abnormalities. [Mh] Termos MeSH primário: Coxa Vara/fisiopatologia Canais Iônicos/genética Osteoblastos/fisiologia Osteoclastos/fisiologia Osteogênese Imperfeita/fisiopatologia Fraturas da Coluna Vertebral/fisiopatologia [Mh] Termos MeSH secundário: Adolescente Adulto Animais Densidade Óssea Cálcio/metabolismo Osso Esponjoso/diagnóstico por imagem Osso Esponjoso/patologia Estudos de Casos e Controles Contagem de Células Diferenciação Celular Criança Pré-Escolar Coxa Vara/etiologia Ecocardiografia Feminino Perfilação da Expressão Gênica Genótipo Cardiopatias/diagnóstico por imagem Cardiopatias/etiologia Cardiopatias/fisiopatologia Heterozigoto Seres Humanos Lactente Recém-Nascido Canais Iônicos/metabolismo Vértebras Lombares/diagnóstico por imagem Masculino Camundongos Microscopia Eletrônica Hipotonia Muscular/etiologia Hipotonia Muscular/fisiopatologia Mutação Tamanho do Órgão Osteoblastos/citologia Osteoclastos/citologia Osteogênese Imperfeita/complicações Osteogênese Imperfeita/diagnóstico por imagem Osteogênese Imperfeita/genética Fenótipo Reação em Cadeia da Polimerase Via Transcriptase Reversa Índice de Gravidade de Doença Análise Espectral Raman Fraturas da Coluna Vertebral/etiologia Adulto Jovem [Pt] Tipo de publicação: JOURNAL ARTICLE [Nm] Nome de substância: 0 (Ion Channels); 0 (TMEM38B protein, human); 0 (TRIC-B protein, mouse); SY7Q814VUP (Calcium) [Em] Mês de entrada: 1709 [Cu] Atualização por classe: 170911 [Lr] Data última revisão: 170911 [Sb] Subgrupo de revista: AIM; IM [Da] Data de entrada para processamento: 170322 [St] Status: MEDLINE [do] DOI: 10.1210/jc.2016-3766

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[PMID]: 28154993 [Au] Autor: Kutzner KP; Freitag T; Donner S; Kovacevic MP; Bieger R [Ad] Endereço: Department of Orthopaedic Surgery and Traumatology, St. Josefs Hospital Wiesbaden, Beethovenstr. 20, 65189, Wiesbaden, Germany. kkutzner@joho.de. [Ti] Título: Outcome of extensive varus and valgus stem alignment in short-stem THA: clinical and radiological analysis using EBRA-FCA. [So] Source: Arch Orthop Trauma Surg;137(3):431-439, 2017 Mar. [Is] ISSN: 1434-3916 [Cp] País de publicação: Germany [La] Idioma: eng [Ab] Resumo: INTRODUCTION: The principle of implanting a calcar-guided short stem consists of an individual alignment alongside the medial calcar providing the ability of reconstructing varus and valgus anatomy in a great variety. However, still, there are broad concerns about the safety of extensive varus and valgus positioning in regard to stability, bony alterations, and periprosthetic fractures. MATERIALS AND METHODS: 216 total hip arthroplasties using a calcar-guided short stem (optimys, Mathys Ltd.) in 162 patients were included. Depending on postoperative CCD angle, hips were divided into five groups (A-E). Varus- and valgus tilt and axial subsidence were assessed by "Einzel-Bild-Roentgen-Analyse"(EBRA-FCA, femoral component analysis) over a 2-year follow-up. The incidence of stress-shielding and cortical hypertrophy as well as clinical outcome [Harris Hip Score (HHS)] were reported. RESULTS: Postoperative CCD angles ranged from 117.9° to 145.6° and mean postoperative CCD angles in group A-E were 123.3°, 128.0°, 132.4°, 137.5°, and 142.5°, respectively. After 2 years, the mean varus/valgus tilt was -0.16°, 0.37°, 0.48°, 0.01°, and 0.86°, respectively (p = 0.502). Axial subsidence after 2 years was 1.20, 1.02, 1.44, 1.50, and 2.62 mm, respectively (p = 0.043). No periprosthetic fractures occurred and none of the stems had to be revised. Rates of stress-shielding and cortical hypertrophy as well as HHS showed no significant difference between the groups. CONCLUSIONS: Valgus alignment results in increased subsidence but does not affect the clinical outcome. There is no difference in stress shielding and cortical hypertrophy between the groups. The authors recommend long term monitoring of valgus aligned stems. [Mh] Termos MeSH primário: Artroplastia de Quadril/métodos Coxa Valga/epidemiologia Coxa Vara/epidemiologia Prótese de Quadril Osteoartrite do Quadril/cirurgia Complicações Pós-Operatórias/epidemiologia [Mh] Termos MeSH secundário: Idoso Coxa Valga/diagnóstico por imagem Coxa Valga/fisiopatologia Coxa Vara/diagnóstico por imagem Coxa Vara/fisiopatologia Feminino Necrose da Cabeça do Fêmur/cirurgia Quadril/cirurgia Luxação Congênita de Quadril/cirurgia Articulação do Quadril/cirurgia Seres Humanos Masculino Meia-Idade Osteoartrite/cirurgia Fraturas Periprotéticas/epidemiologia Complicações Pós-Operatórias/diagnóstico por imagem Complicações Pós-Operatórias/fisiopatologia Período Pós-Operatório Desenho de Prótese Radiografia Radiologistas Estudos Retrospectivos Resultado do Tratamento [Pt] Tipo de publicação: JOURNAL ARTICLE; OBSERVATIONAL STUDY [Em] Mês de entrada: 1705 [Cu] Atualização por classe: 170524 [Lr] Data última revisão: 170524 [Sb] Subgrupo de revista: IM [Da] Data de entrada para processamento: 170204 [St] Status: MEDLINE [do] DOI: 10.1007/s00402-017-2640-z

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[PMID]: 27768218 [Au] Autor: Murphy CG; Bonnin MP; Desbiolles AH; Carrillon Y; AÑ—t Si Selmi T [Ad] Endereço: Department of Orthopaedics, Center Orthopédique Santy, Lyon - France. [Ti] Título: Varus will have varus; a radiological study to assess and predict varus stem placement in uncemented femoral stems. [So] Source: Hip Int;26(6):554-560, 2016 Nov 10. [Is] ISSN: 1724-6067 [Cp] País de publicação: Italy [La] Idioma: eng [Ab] Resumo: BACKGROUND: Varus inclination of the uncemented stem is not necessarily a technical error. The proximal femoral anatomy of hips with a coxa vara deformity frequently predisposes varus inclination. METHODS: We reviewed a series of 200 patients undergoing primary uncemented THA with the Corail® hip system. Preoperative data were based on patient demographics, diagnosis, and radiographic information (preoperative templating-CT measurements), and compared postoperative alignment for each stem and type of stem used. Proximal femoral traits which can alert surgeons, when templating preoperatively, to potential varus alignment were noted. RESULTS: All stems were inserted either in neutral or varus alignments. Low neck shaft angle is strongly predictive of increased varus stem alignment (p<0.001). Stems inserted with higher varus alignment were associated with the preoperative morphological traits associated with coxa vara hip deformities - increased femoral offset (p<0.001), greater trochanteric overhang (p<0.001), greater trochanteric height (p<0.046), and a lower canal flare index (p<0.046). CONCLUSIONS: Varus stem alignment is neither unexpected nor necessarily a technical failure when using this particular uncemented stem system. Coxa vara deformities, due to a combination of morphological traits, are more likely to be inserted with higher varus alignment than hips with normal or higher neck shaft angles. Surgeons need to be aware of this when carrying out preoperative templating and intraoperative assessment, in order to prevent over-compensation for offset, length or stability. [Mh] Termos MeSH primário: Artroplastia de Quadril Coxa Vara/diagnóstico por imagem Coxa Vara/cirurgia Prótese de Quadril Artropatias/diagnóstico por imagem Artropatias/cirurgia [Mh] Termos MeSH secundário: Idoso Cimentação Coxa Vara/etiologia Feminino Fêmur/diagnóstico por imagem Fêmur/cirurgia Seres Humanos Artropatias/complicações Masculino Meia-Idade Estudos Retrospectivos Tomografia Computadorizada por Raios X Resultado do Tratamento [Pt] Tipo de publicação: JOURNAL ARTICLE [Em] Mês de entrada: 1707 [Cu] Atualização por classe: 170707 [Lr] Data última revisão: 170707 [Sb] Subgrupo de revista: IM [Da] Data de entrada para processamento: 161030 [St] Status: MEDLINE [do] DOI: 10.5301/hipint.5000412

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[PMID]: 27751317 [Au] Autor: Patil DV; Phadke MS; Pahwa JS; Dalal AB [Ad] Endereço: Resident, KEMH - Seth GS medical College and KEM Hospital, Parel, Mumbai, India. Electronic address: devendrapatil161185@gmail.com. [Ti] Título: Brothers with constrictive pericarditis - A novel mutation in a rare disease. [So] Source: Indian Heart J;68 Suppl 2:S284-S287, 2016 Sep. [Is] ISSN: 0019-4832 [Cp] País de publicação: India [La] Idioma: eng [Ab] Resumo: Familial constrictive pericarditis is extremely rare. We report a case of two brothers both suffering constrictive pericarditis along with having multiple painless joint deformities. Genetic workup confirmed the clinical diagnosis of camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome CACP syndrome and also revealed a rare mutation in the causative gene. [Mh] Termos MeSH primário: Artropatia Neurogênica/genética Coxa Vara/genética DNA/genética Deformidades Congênitas da Mão/genética Mutação Pericardite Constritiva/genética Proteoglicanas/genética Doenças Raras Irmãos Sinovite/genética [Mh] Termos MeSH secundário: Adolescente Artropatia Neurogênica/diagnóstico Artropatia Neurogênica/metabolismo Cateterismo Cardíaco Criança Coxa Vara/diagnóstico Coxa Vara/metabolismo Análise Mutacional de DNA Ecocardiografia Testes Genéticos Deformidades Congênitas da Mão/diagnóstico Deformidades Congênitas da Mão/metabolismo Seres Humanos Masculino Linhagem Pericardite Constritiva/diagnóstico Pericardite Constritiva/metabolismo Proteoglicanas/metabolismo Sinovite/diagnóstico Sinovite/metabolismo [Pt] Tipo de publicação: CASE REPORTS; JOURNAL ARTICLE [Nm] Nome de substância: 0 (PRG4 protein, human); 0 (Proteoglycans); 9007-49-2 (DNA) [Em] Mês de entrada: 1706 [Cu] Atualização por classe: 170902 [Lr] Data última revisão: 170902 [Sb] Subgrupo de revista: IM [Da] Data de entrada para processamento: 161019 [St] Status: MEDLINE

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[PMID]: 27748313 [Au] Autor: Vutukuru R; Reddy KK [Ad] Endereço: Department of Orthopaedics & Traumatology, Osmania General Hospital, Afzalgunj, Hyderabad 500 012, Telangana, India. [Ti] Título: Pathognomonic acetabular cysts in camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome. [So] Source: Indian J Med Res;143(6):834-835, 2016 Jun. [Is] ISSN: 0971-5916 [Cp] País de publicação: India [La] Idioma: eng [Mh] Termos MeSH primário: Artropatia Neurogênica/diagnóstico Coxa Vara/diagnóstico Deformidades Congênitas da Mão/diagnóstico Articulação do Joelho/fisiopatologia Sinovite/diagnóstico [Mh] Termos MeSH secundário: Artropatia Neurogênica/diagnóstico por imagem Artropatia Neurogênica/fisiopatologia Criança Coxa Vara/diagnóstico por imagem Coxa Vara/fisiopatologia Deformidades Congênitas da Mão/diagnóstico por imagem Deformidades Congênitas da Mão/fisiopatologia Seres Humanos Articulação do Joelho/diagnóstico por imagem Masculino Líquido Sinovial/metabolismo Sinovite/diagnóstico por imagem Sinovite/fisiopatologia [Pt] Tipo de publicação: CASE REPORTS; JOURNAL ARTICLE [Em] Mês de entrada: 1706 [Cu] Atualização por classe: 170817 [Lr] Data última revisão: 170817 [Sb] Subgrupo de revista: IM [Da] Data de entrada para processamento: 161018 [St] Status: MEDLINE [do] DOI: 10.4103/0971-5916.192082

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[PMID]: 27583115 [Au] Autor: Elzohairy MM; Khairy HM [Ad] Endereço: Faculty of Medicine, Zagazig University Hospitals, Sharkia, Egypt. [Ti] Título: Fixation of Intertrochanteric Valgus Osteotomy with T Plate in Treatment of Developmental Coxa Vara. [So] Source: Clin Orthop Surg;8(3):310-5, 2016 Sep. [Is] ISSN: 2005-4408 [Cp] País de publicação: Korea (South) [La] Idioma: eng [Ab] Resumo: BACKGROUND: Although the valgus subtrochanteric osteotomy is considered as a standard surgical treatment for coxa vara, there is no consensus on the optimal method of fixation and osteotomy technique. Fixation of the osteotomy has been achieved by various methods including external fixation and internal fixation with pins and cerclage and a variety of plates. The aim of this study is the evaluation of the results of developmental coxa treated by Y intertrochanteric valgus osteotomy fixed with a T-buttress plate compared with other methods of fixation in the literature. METHODS: Eighteen corrective valgus intertrochanteric femoral osteotomies were performed in 18 patients (18 hips) for treatment of unilateral developmental coxa vara deformity and fixed with a T plate. There were 12 males and 6 females. The right hip was affected in 10 patients and the left hip in 8 patients. Clinically, patients were evaluated by Larson hip score. Radiographically, anteroposterior view of the pelvis and frog leg lateral views of the affected hip were taken preoperatively and compared with the findings at the final follow-up. RESULTS: The average follow-up was 29 months (range, 24 to 36 months). Clinical results showed improvement of the mean Larson hip score from 57.8 to 97.0 (p < 0.001). Radiological results showed that all osteotomies were completely united in 2.4 months (range, 2 to 3 months) with the achievement of the planned correction angle. The average correction of Hilgenreiner's epiphyseal angle improved from 78.2° to 27.8° (p < 0.001) at the final follow-up. The femoral neck shaft angle was improved from 93.7° to 129.9° (p < 0.001) at the final follow-up. Shortening of the affected limb was corrected from 2.8 cm to 1.3 cm (p < 0.001) at the last follow-up. No major serious complications were recorded in the present study. CONCLUSIONS: Intertrochanteric valgus osteotomy of the proximal femur fixed with a T plate may be efficient for treatment of developmental coxa vara. With careful planning, it can result in a low complication rate and insignificant or minimal recurrence rate. [Mh] Termos MeSH primário: Placas Ósseas Coxa Vara/cirurgia Colo do Fêmur/cirurgia Fixação Interna de Fraturas Osteotomia [Mh] Termos MeSH secundário: Criança Pré-Escolar Feminino Seguimentos Fixação Interna de Fraturas/instrumentação Fixação Interna de Fraturas/métodos Fixação Interna de Fraturas/estatística & dados numéricos Seres Humanos Masculino Osteotomia/métodos Osteotomia/estatística & dados numéricos [Pt] Tipo de publicação: JOURNAL ARTICLE [Em] Mês de entrada: 1703 [Cu] Atualização por classe: 170306 [Lr] Data última revisão: 170306 [Sb] Subgrupo de revista: IM [Da] Data de entrada para processamento: 160902 [St] Status: MEDLINE [do] DOI: 10.4055/cios.2016.8.3.310

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[PMID]: 27224999 [Au] Autor: Peters B; Schuurs-Hoeijmakers JH; Fuijkschot J; Reimer A; van der Flier M; Lugtenberg D; Hoppenreijs EP [Ad] Endereço: Department of Paediatrics, Radboud University Nijmegen Medical Centre, Radboud umc. Geert Grooteplein Zuid 10, Nijmegen, 6525 GA, The Netherlands. Brmpeters@gmail.com. [Ti] Título: Protein-losing enteropathy in camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome. [So] Source: Pediatr Rheumatol Online J;14(1):32, 2016 May 25. [Is] ISSN: 1546-0096 [Cp] País de publicação: England [La] Idioma: eng [Ab] Resumo: BACKGROUND: Camptodactyly-arthropathy-coxa vara-pericarditis (CACP, OMIM: #208250) syndrome is a rare autosomal recessive disease that can be difficult to recognise not only because of its wide clinical variability but also because of its clinical resemblance to juvenile idiopathic arthritis (JIA). PRG4 is the only gene so far known to be associated with CACP syndrome. Children with CACP syndrome lack the glycoprotein lubricin due to recessive mutations in PRG4. Lubricin serves as a lubricant in joints, tendons and visceral cavities (pleural cavity, pericardium) and inhibits synovial proliferation. Children with CACP syndrome suffer from congenital camptodactyly, arthropathy, coxa vara and sometimes pericarditis. This report concerns a child with CACP syndrome complicated by protein-losing enteropathy (PLE), caused by constrictive pericarditis and so contributes to knowledge of the presentation of CACP syndrome. CASE PRESENTATION: A 10- year-old girl with consanguineous parents suffered from congenital camptodactyly and progressive swollen and painful joints. Her father and his sister had similar childhood-onset joint complaints. Laboratory tests showed no signs of inflammation but showed persistent low protein- and IgG- levels, indicating a secondary immunodeficiency. Increased alpha antitrypsin clearance confirmed PLE. Abdominal ultrasound with Doppler showed hepatomegaly and portal hypertension. Echocardiography suggested constrictive pericarditis. However, heart catheterization could not confirm this. Ultrasound and X-ray examination of the joints combined with a puncture of the synovial fluid were performed. These results, combined with the clinical presentation and the consanguinity, suggested CACP syndrome. Due to excessive enteral protein losses, the patient was treated with Cotrimoxazol prophylaxis and immunoglobulin supplements. These supplements were inadequate to achieve normal IgG values. As constrictive pericarditis with subsequent PLE was the best explanation for the excessive IgG losses, pericardiectomy was performed with good results. Genetic testing in our patient was complicated but revealed a pathogenic mutation within the repeat sequence in exon 7 of the PRG4 gene. CONCLUSION: PLE resulting from constrictive pericarditis can be a complication of CACP syndrome. As serious complications can arise from the resulting secondary immunodeficiency, we recommend regular evaluation of clinical symptoms of constrictive pericarditis and PLE in children with CACP syndrome. [Mh] Termos MeSH primário: Artropatia Neurogênica/complicações Coxa Vara/complicações Deformidades Congênitas da Mão/complicações Enteropatias Perdedoras de Proteínas/complicações Sinovite/complicações [Mh] Termos MeSH secundário: Artropatia Neurogênica/genética Criança Consanguinidade Coxa Vara/genética Feminino Deformidades Congênitas da Mão/genética Homozigoto Seres Humanos Enteropatias Perdedoras de Proteínas/genética Sinovite/genética [Pt] Tipo de publicação: CASE REPORTS; JOURNAL ARTICLE [Em] Mês de entrada: 1703 [Cu] Atualização por classe: 170321 [Lr] Data última revisão: 170321 [Sb] Subgrupo de revista: IM [Da] Data de entrada para processamento: 160527 [St] Status: MEDLINE [do] DOI: 10.1186/s12969-016-0093-5

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[PMID]: 27125086 [Au] Autor: Savin L; Mihailescu D; Grierosu C; Botez P [Ti] Título: EFFECT OF TRANEXAMIC ACID USE ON POSTOPERATIVE BLOOD LOSS IN TOTAL KNEE ARTHROPLASTY. [So] Source: Rev Med Chir Soc Med Nat Iasi;120(1):137-41, 2016 Jan-Mar. [Is] ISSN: 0048-7848 [Cp] País de publicação: Romania [La] Idioma: eng [Ab] Resumo: AIM: To monitor the effect of tranexamic acid use (Exacyl) on postoperative bleeding (aspiration), autologous blood transfusion (autotransfusion), allogeneic blood transfusion and postoperative anemia (difference between preoperative and immediate postoperative hemoglobin levels) in total knee arthroplasty. MATERIALS AND METHODS: This retrospective observational study was conducted on a sample of 457 patients who underwent endoprosthetic knee arthroplasty in the interval January 1, 2008-July 3O, 2014 at the Traumatology-Orthopedics Clinic of the Iasi Rehabilitation Hospital. The mean age of the study group was 66 years (range 32-84 years), 74.4% were female, 39.8% presented varus and 6.1% valgus malalignment. The study group was subdivided into two groups: Group 1 served as controls and underwent surgery without the administration of tranexamic acid and Group 2 received tranexamic acid. RESULTS: In group 1 the average amount of blood lost was significantly higher than in group 2, 1168.94 ml vs. 452.9 ml (p < 0.001). The use of a cell saver was required in 70% of group 1 patients with an average amount of auto transfused blood of 480.70 ml, significantly more frequent and in greater amounts than in group 2 (below 10%) with an average of 15.05 ml (p < 0.001). In this study tranexamic acid had not a statistically significant favorable effect on blood transfusion requirements and anemia syndrome. CONCLUSION: Tranexamic acid has a favorable effect on postoperative blood loss and its associated complications, with a favorable impact on both early clinical and functional recovery. [Mh] Termos MeSH primário: Antifibrinolíticos/administração & dosagem Artroplastia do Joelho Hemorragia Pós-Operatória/prevenção & controle Ácido Tranexâmico/administração & dosagem [Mh] Termos MeSH secundário: Adulto Idoso Idoso de 80 Anos ou mais Artroplastia do Joelho/efeitos adversos Coxa Valga/cirurgia Coxa Vara/cirurgia Feminino Seres Humanos Masculino Meia-Idade Estudos Retrospectivos Resultado do Tratamento [Pt] Tipo de publicação: JOURNAL ARTICLE; OBSERVATIONAL STUDY [Nm] Nome de substância: 0 (Antifibrinolytic Agents); 6T84R30KC1 (Tranexamic Acid) [Em] Mês de entrada: 1606 [Cu] Atualização por classe: 160429 [Lr] Data última revisão: 160429 [Sb] Subgrupo de revista: IM [Da] Data de entrada para processamento: 160430 [St] Status: MEDLINE

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[PMID]: 26242281 [Au] Autor: Pruszczynski B; Mackenzie WG; Rogers K; White KK [Ad] Endereço: Department of Orthopedics and Pediatric Orthopedics, Medical University of Lodz, Lodz, Poland. [Ti] Título: Spinal Cord Injury After Extremity Surgery in Children With Thoracic Kyphosis. [So] Source: Clin Orthop Relat Res;473(10):3315-20, 2015 Oct. [Is] ISSN: 1528-1132 [Cp] País de publicação: United States [La] Idioma: eng [Ab] Resumo: BACKGROUND: Spinal cord injury is a rare complication after lower extremity surgery in children with skeletal dysplasia and thoracic kyphosis. We encountered two patients who had this complication, from among 51 (39 from Nemours/Alfred I. duPont Hospital for Children and 12 from Seattle Children's Hospital) who underwent lower extremity surgery during an 8.5-year period (June 2004 to December 2012). Because spinal cord injury is a devastating complication likely not known to most physicians treating patients with skeletal dysplasias, we sought to examine factors that may contribute to this rare complication. CASE DESCRIPTION: We performed a retrospective review of two patients with skeletal dysplasia who had paraplegia develop after extremity surgery. Outcome measures included operative time, vital signs, and postsurgery recovery of neurologic deficit. MR images were reviewed. Two patients were found-an 8.5-year-old boy with spondyloepiphyseal dysplasia congenita with a 76°-thoracic kyphosis apex at T4 and a 6.5-year-old boy with mucopolysaccharidosis type 1-H with an 80°-thoracic kyphosis apex at T2. Bilateral proximal femoral osteotomies or bilateral innominate and proximal femoral osteotomies had been performed. The spinal cord injuries occurred at the apex of the kyphosis as determined by clinical examination and MRI assessment. In both patients, the mean arterial blood pressure decreased below 50 mm Hg and might be a factor in the etiology of the paralysis. The first patient recovered motor function in 5 months; the second had no recovery. LITERATURE REVIEW: Paraplegia is extremely rare after nonspine operations. Many factors contribute to the risk for a spinal cord event: low mean arterial pressure, duration of the surgery, position on the operating table, the kyphotic spine deformity, or unappreciated vascular disease. Motor-evoked potentials and somatosensory-evoked potentials together potentially provide high sensitivity and specificity for predicting a postoperative neurologic deficit. CLINICAL RELEVANCE: Based on our two patients with skeletal dysplasia and a literature review of patients with hyperkyphosis undergoing extremity surgery, the surgeon must be aware of the risk of spinal cord injury. Careful preoperative assessment possibly including MRI of the spine is recommended. Mean arterial pressure should be maintained at a safe level; neuromonitoring should be considered. [Mh] Termos MeSH primário: Coxa Vara/complicações Coxa Vara/cirurgia Cifose/complicações Osteotomia Complicações Pós-Operatórias/etiologia Traumatismos da Medula Espinal/etiologia [Mh] Termos MeSH secundário: Criança Seres Humanos Masculino Estudos Retrospectivos [Pt] Tipo de publicação: CASE REPORTS; JOURNAL ARTICLE; REVIEW [Em] Mês de entrada: 1512 [Cu] Atualização por classe: 161019 [Lr] Data última revisão: 161019 [Sb] Subgrupo de revista: AIM; IM [Da] Data de entrada para processamento: 150806 [St] Status: MEDLINE [do] DOI: 10.1007/s11999-015-4437-0

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[PMID]: 26110218 [Au] Autor: Kushare I; Wiltfong RE; Klingele KE [Ad] Endereço: aDepartment of Orthopedic Surgery, Nationwide Children's Hospital bDepartment of Orthopaedics, The Ohio State University, Columbus, Ohio, USA. [Ti] Título: Acute, unstable slipped capital femoral epiphysis with associated congenital coxa vara. [So] Source: J Pediatr Orthop B;24(6):511-4, 2015 Nov. [Is] ISSN: 1473-5865 [Cp] País de publicação: United States [La] Idioma: eng [Ab] Resumo: Slipped capital femoral epiphysis in patients younger than 10 years is rare and is often associated with some identifiable metabolic or endocrinologic abnormality. We present a case of a 5-year-old girl with an acute, unstable, severe slipped capital femoral epiphysis associated with congenital coxa vara and its surgical management. This association has not been described in previous literature. Surgical treatment is proposed and described. [Mh] Termos MeSH primário: Coxa Vara/congênito Escorregamento das Epífises Proximais do Fêmur/etiologia [Mh] Termos MeSH secundário: Doença Aguda Pré-Escolar Coxa Vara/complicações Coxa Vara/diagnóstico por imagem Feminino Seres Humanos Procedimentos Ortopédicos/métodos Radiografia Escorregamento das Epífises Proximais do Fêmur/diagnóstico Escorregamento das Epífises Proximais do Fêmur/cirurgia [Pt] Tipo de publicação: CASE REPORTS; JOURNAL ARTICLE [Em] Mês de entrada: 1607 [Cu] Atualização por classe: 161125 [Lr] Data última revisão: 161125 [Sb] Subgrupo de revista: IM [Da] Data de entrada para processamento: 150626 [St] Status: MEDLINE [do] DOI: 10.1097/BPB.0000000000000209

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