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1.
BMJ Open Respir Res ; 5(1): e000264, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29616140

RESUMO

Introduction: High prevalence of obstructive lung disease has been reported in patients undergoing surgical correction of thoracic scoliosis. Airway narrowing due to spine morphology is analysed as a contributing factor. Methods: Preoperative surgical planning CTs of 34 patients with right-sided thoracic scoliosis (age: 17.6±9.0) were retrospectively analysed and compared with 15 non-scoliotic controls (age: 16.3±5.1). Three-dimensional models of spine and airway lumen were reconstructed. Based on thoracic sagittal profile, patients were divided into hypokyphosis (HypoS: <10°), normal kyphosis (NormS: ≥10° and <40°) and hyperkyphosis (HyperS: ≥40°) groups. Lumen area of bronchi, bifurcation angles and minimum spine-airway distance were measured. Pulmonary function tests were correlated to scoliosis, kyphosis and lumen area. Results: Loss of kyphosis led to proximity between bronchus intermedius (BI) and spine. HypoS (NormS) had lumen area reductions in the right main bronchus of 29% (19%), BI of 45% (23%), right middle lobar bronchus of 46% (32%) and right lower lobe bronchus (RLL7) of 66% (37%), respectively (P<0.05). The lower right superior segmental bronchus was reduced across all scoliotic groups (P<0.05). Airways were displaced caudal by 0.65±0.45 vertebra in patients with scoliosis. Loss of kyphosis correlated negatively with forced expiratory volume in 1 s/forced vital capacity (FEV1/FVC), FVC/(FVC predicted) and FEV1/(FEV1 predicted) (P<0.01). Lumen area of trachea, right upper lobar bronchus, BI and RLL7 correlated negatively with FEV1/FVC. BI and RLL7 narrowing were strong predictors of FVC and FEV1 loss (P<0.001). Conclusions: Right-sided main stem airways are narrowed in HypoS and NormS. Loss of kyphosis leads to narrowing of BI and its trifurcation. FEV1/FVC correlated negatively with airway narrowing, implying an obstructive element to lung function impairment in patients with scoliosis and hypokyphosis.

2.
Eur Spine J ; 26(8): 2103-2111, 2017 08.
Artigo em Inglês | MEDLINE | ID: mdl-27554347

RESUMO

PURPOSE: To compare measurements of motor evoked potential latency stimulated either magnetically (mMEP) or electrically (eMEP) and central motor conduction time (CMCT) made pre-operatively in conscious patients using transcranial and intra-operatively using electrical cortical stimulation before and after successful instrumentation for the treatment of adolescent idiopathic scoliosis. METHODS: A group initially of 51 patients with adolescent idiopathic scoliosis aged 12-19 years was evaluated pre-operatively in the outpatients' department with transcranial magnetic stimulation. The neurophysiological data were then compared statistically with intra-operative responses elicited by transcranial electrical stimulation both before and after successful surgical intervention. MEPs were measured as the cortically evoked compound action potentials of Abductor hallucis. Minimum F-waves were measured using conventional nerve conduction methods and the lower motor neuron conduction time was calculated and this was subtracted from MEP latency to give CMCT. RESULTS: Pre-operative testing was well tolerated in our paediatric/adolescent patients. No neurological injury occurred in any patient in this series. There was no significant difference in the values of mMEP and eMEP latencies seen pre-operatively in conscious patients and intra-operatively in patients under anaesthetic. The calculated quantities mCMCT and eCMCT showed the same statistical correlations as the quantities mMEP and eMEP latency. CONCLUSIONS: The congruency of mMEP and eMEP and of mCMCT and eCMCT suggests that these measurements may be used comparatively and semi-quantitatively for the comparison of pre-, intra-, and post-operative spinal cord function in spinal deformity surgery.


Assuntos
Potencial Evocado Motor , Monitorização Neurofisiológica Intraoperatória/métodos , Assistência Perioperatória/métodos , Escoliose/cirurgia , Estimulação Magnética Transcraniana , Adolescente , Criança , Estimulação Elétrica , Feminino , Humanos , Masculino , Escoliose/fisiopatologia , Resultado do Tratamento , Adulto Jovem
3.
Med. clín (Ed. impr.) ; 146(11): 497-505, jun. 2016. tab
Artigo em Espanhol | IBECS | ID: ibc-152132

RESUMO

La paniculitis mesentérica es una entidad caracterizada por inflamación crónica e inespecífica del mesenterio. Existe poca información y a menudo confusa sobre sus características, así como sobre la actitud a seguir una vez que se evidencia en una prueba de imagen. Nos proponemos describir las características epidemiológicas, clínicas, analíticas, radiológicas y patológicas de los pacientes con paniculitis mesentérica publicados en la literatura, así como las entidades posiblemente causales o asociadas a la paniculitis mesentérica, según la opinión de los autores de cada estudio. Por último, revisaremos las diferentes opciones terapéuticas utilizadas y la respuesta a las mismas. Para ello se realizó una búsqueda bibliográfica en las principales bases de datos médicas, seleccionando aquellos artículos con información sobre estos aspectos, recogiendo dicha información en una base de datos que se almacenó en el paquete estadístico SPSS para su posterior análisis y resumen (AU)


Mesenteric panniculitis is a condition characterized by chronic nonspecific inflammation of the mesentery. There is little and often confusing information about its characteristics and the approach to take once it has been demonstrated by an imaging test. We propose to describe the epidemiological, clinical, laboratory, radiological and pathological features of the patients with mesenteric panniculitis reported in the literature, as well as possible disorders causal or associated with mesenteric panniculitis, in the opinion of the authors of each study. Finally, we will review the different therapeutic options used and the response to them. To that end a literature search was performed from the main medical databases selecting ítems with information on these aspects. This information was collected on a database stored in SPSS software for further analysis and summary (AU)


Assuntos
Humanos , Masculino , Feminino , Paniculite Peritoneal/epidemiologia , Paniculite Peritoneal/patologia , Paniculite Peritoneal/etiologia , Mesentério/patologia , Mesentério , Mesentério/cirurgia , Necrose Gordurosa/diagnóstico , Necrose Gordurosa/patologia , Necrose Gordurosa , Fibrose/diagnóstico , Fibrose/patologia , Fibrose , Prevalência , Incidência , Glucocorticoides/administração & dosagem , Glucocorticoides/farmacologia , Glucocorticoides/uso terapêutico , Progressão da Doença , Diagnóstico por Imagem/instrumentação , Diagnóstico por Imagem/métodos , Diagnóstico por Imagem
4.
Med Clin (Barc) ; 146(11): 497-505, 2016 Jun 03.
Artigo em Espanhol | MEDLINE | ID: mdl-26971978

RESUMO

Mesenteric panniculitis is a condition characterized by chronic nonspecific inflammation of the mesentery. There is little and often confusing information about its characteristics and the approach to take once it has been demonstrated by an imaging test. We propose to describe the epidemiological, clinical, laboratory, radiological and pathological features of the patients with mesenteric panniculitis reported in the literature, as well as possible disorders causal or associated with mesenteric panniculitis, in the opinion of the authors of each study. Finally, we will review the different therapeutic options used and the response to them. To that end a literature search was performed from the main medical databases selecting ítems with information on these aspects. This information was collected on a database stored in SPSS software for further analysis and summary.


Assuntos
Paniculite Peritoneal , Saúde Global , Humanos , Paniculite Peritoneal/diagnóstico , Paniculite Peritoneal/epidemiologia , Paniculite Peritoneal/etiologia , Paniculite Peritoneal/terapia , Prevalência , Fatores de Risco
5.
Ann Rheum Dis ; 74(3): e14, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24534757

RESUMO

OBJECTIVES: To perform fine mapping of the PXK locus associated with systemic lupus erythematosus (SLE) and study functional effects that lead to susceptibility to the disease. METHODS: Linkage disequilibrium (LD) mapping was conducted by using 1251 SNPs (single nucleotide polymorphism) covering a 862 kb genomic region on 3p14.3 comprising the PXK locus in 1467 SLE patients and 2377 controls of European origin. Tag SNPs and genotypes imputed with IMPUTE2 were tested for association by using SNPTEST and PLINK. The expression QTLs data included three independent datasets for lymphoblastoid cells of European donors: HapMap3, MuTHER and the cross-platform eQTL catalogue. Correlation analysis of eQTLs was performed using Vassarstats. Alternative splicing for the PXK gene was analysed on mRNA from PBMCs. RESULTS: Fine mapping revealed long-range LD (>200 kb) extended over the ABHD6, RPP14, PXK, and PDHB genes on 3p14.3. The highly correlated variants tagged an SLE-associated haplotype that was less frequent in the patients compared with the controls (OR=0.89, p=0.00684). A robust correlation between the association with SLE and enhanced expression of ABHD6 gene was revealed, while neither expression, nor splicing alterations associated with SLE susceptibility were detected for PXK. The SNP allele frequencies as well as eQTL pattern analysed in the CEU and CHB HapMap3 populations indicate that the SLE association and the effect on ABHD6 expression are specific to Europeans. CONCLUSIONS: These results confirm the genetic association of the locus 3p14.3 with SLE in Europeans and point to the ABHD6 and not PXK, as the major susceptibility gene in the region. We suggest a pathogenic mechanism mediated by the upregulation of ABHD6 in individuals carrying the SLE-risk variants.


Assuntos
Peptídeos e Proteínas de Sinalização Intracelular/genética , Desequilíbrio de Ligação/genética , Lúpus Eritematoso Sistêmico/genética , Monoacilglicerol Lipases/genética , Proteínas do Tecido Nervoso/genética , Proteínas Serina-Treonina Quinases/genética , RNA Mensageiro/genética , Processamento Alternativo , Estudos de Casos e Controles , Mapeamento Cromossômico , Cromossomos Humanos 1-3 , Grupo com Ancestrais do Continente Europeu/genética , Predisposição Genética para Doença , Haplótipos , Humanos , Polimorfismo de Nucleotídeo Único
6.
J Rheumatol ; 41(3): 453-7, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24488423

RESUMO

OBJECTIVE: To compare the prevalence and disability of headache in patients with systemic lupus erythematosus (SLE) with the general population and to assess the role of chronic psychological stress (CPS) in headache development. METHODS: One hundred seventy patients with SLE and 102 control subjects matched for age, sex, and level of education were included in this multicenter, cross-sectional study. CPS, headache-related disability, and chronic analgesic intake (CAI) were evaluated in all participants. RESULTS: No statistical differences in the prevalence of headache between both groups were observed but headache disability was significantly higher in patients with SLE. In addition, a higher average score in the Cohen Perceived Stress Scale (CPSS) and a higher prevalence of patients with CAI were observed in patients with SLE. In multivariate analysis, CPSS score was positively (OR 1.09; 95% CI: 1.03-1.14; p = 0.001) and CAI negatively (OR 0.43; 95% CI: 0.19-0.99; p = 0.049) associated with headache in patients with SLE. CONCLUSION: Despite the prevalence of headache in patients with SLE and the general population being similar, headache-related disability may be higher in patients with SLE. Moreover, CPS might play a role in the pathogenesis of SLE headache, whereas CAI might have a protective effect against it.


Assuntos
Cefaleia/epidemiologia , Cefaleia/etiologia , Lúpus Eritematoso Sistêmico/complicações , Estresse Psicológico/complicações , Adulto , Estudos Transversais , Feminino , Cefaleia/psicologia , Humanos , Lúpus Eritematoso Sistêmico/psicologia , Masculino , Pessoa de Meia-Idade , Prevalência , Estresse Psicológico/psicologia
7.
Eur Spine J ; 23(12): 2751-7, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-24469884

RESUMO

PURPOSE: To evaluate the outcome and complications of a novel technique for the treatment of progressive thoracolumbar kyphosis in children with mucopolysaccharidosis (MPS). METHODS: The medical records and spinal imaging of four consecutive paediatric patients who underwent a single stage anteroposterior spinal fusion with segmental pedicle screw instrumentation were reviewed. RESULTS: Patients underwent spinal deformity correction at the mean age of 3 years (2.4-3.7) with mean clinical follow-up of 3.2 years (2.1-4.5) and mean postoperative radiographic follow-up was 2.4 years (0.8-3). Preoperative kyphosis was corrected from a mean angle of 65º (63º-70º) to 6.5º (-12º-13º). Vertebral subluxation at the apex of the deformity was corrected from an average 64% (56-83%) to 12% (0-24%). Spinal cord monitoring with somatosensory evoked potentials (SSEP) was successfully obtained and stable throughout surgery. No instrumentation failure, loss of correction or junctional problems occurred at final follow-up. CONCLUSIONS: Anterior and posterior spinal arthrodesis with segmental pedicle screw instrumentation is a safety and reliable technique for the treatment of severe thoracolumbar kyphosis in children with MPS. This technique achieves excellent correction of the deformity with adequate decompression of the spinal canal. The fusion is limited to the thoracolumbar junction and interferes minimally with the longitudinal growth of the thorax. No neurological complications or intraoperative spinal cord monitoring events occurred. No loss of correction or junctional kyphosis was observed.


Assuntos
Luxações Articulares/cirurgia , Cifose/cirurgia , Mucopolissacaridoses/complicações , Parafusos Pediculares , Fusão Vertebral/métodos , Pré-Escolar , Gerenciamento Clínico , Progressão da Doença , Feminino , Humanos , Luxações Articulares/complicações , Cifose/complicações , Masculino , Estudos Retrospectivos , Fusão Vertebral/instrumentação , Resultado do Tratamento
8.
Spine (Phila Pa 1976) ; 39(1): E60-3, 2014 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-24153165

RESUMO

STUDY DESIGN: Case report. OBJECTIVE: To report a case of a true-positive isolated somatosensory evoked potential (SSEP) loss with preservation of motor evoked potential (MEP) response during scoliosis correction. SUMMARY OF BACKGROUND DATA: Combined intraoperative monitoring uses SSEPs and MEPs to decrease the probability of observing false-negative events. In combination, SSEPs and MEPs have become a standard of care for spinal deformity surgery. However, literature review reveals several cases of false-negative response with combined SSEPs and MEPs, raising the contention that intraoperative monitoring does not reliably identify all isolated selective spinal cord dysfunction. METHODS: A 15-year-old female patient with a 65° right thoracic adolescent idiopathic scoliosis underwent correction and posterior spinal fusion with segmental pedicle screw instrumentation. After capture and derotation of the left concave rod, left-sided irreversible SSEP loss occurred whereas MEPs remained unchanged. After excluding systemic factors, anesthetic causes, or technical fault, deformity correction was released and instrumentation removed. No cortical breach was reported during pedicle screw removal. RESULTS: Postoperatively, no clinical sensory or motor deficit was present; computed tomography demonstrated a burst left pedicle at T10 with the medial pedicle wall fragment in direct contact with the dorsal spinal cord. Magnetic resonance imaging excluded cord edema or other evidence of injury. Three days after surgery, intraoperative monitoring showed delayed latencies and amplitudes of the left SSEP. An uneventful reinsertion of instrumentation and correction excluding the left T10 pedicle screw was performed 7 days after the initial surgery. CONCLUSION: This case report provides evidence of selective posterior spinal cord dysfunction with sparing of the anterior columns immediately after a correction maneuver and emphasizes the importance of simultaneous SSEP and MEP monitoring. To the authors' knowledge, there is no previous report of a true-positive isolated SSEP loss with preservation of MEP response during scoliosis correction. LEVEL OF EVIDENCE: N/A.


Assuntos
Potencial Evocado Motor/fisiologia , Potenciais Somatossensoriais Evocados/fisiologia , Escoliose/cirurgia , Medula Espinal/fisiopatologia , Vértebras Torácicas/cirurgia , Adolescente , Feminino , Humanos , Monitorização Intraoperatória , Escoliose/fisiopatologia , Fusão Vertebral/instrumentação , Fusão Vertebral/métodos , Resultado do Tratamento
9.
Clin Rheumatol ; 33(4): 567-73, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24096638

RESUMO

Internet has become a widely used tool by patients seeking information on different diseases. The information regarding lupus patients' Internet use is scarce. This study aims to explore the attitudes and practices of lupus patients in southern Spain, regarding Internet use to find health-related information. A survey was carried out including 150 patients from six Andalusian Hospitals. To search for information, 67.3 % of the patients used Internet. The proportion of female Internet users was higher (69.3 vs 46.2 %), particularly those belonging to a patients' association (81.8 vs 32.7 %), and are regular users of Internet (80.2 vs 44.4 %); 37.5 % thought the information found in the Internet was of little use or not useful at all, and 58 % of the respondents stated that the information found caused them concern while for 27 %, it was a relief. Most patients preferred the information given by their physicians (63.6 %); 33.9 % considered that the information from both sources was complementary, and 2.5 % preferred the information obtained from the Internet. A percentage of 85.3 of the patients would like their physicians to provide them with information on high-quality sites regarding their illness. Lupus patients make frequent use of the Internet to look for information on their disease. Considering this, and because better-informed patients follow more precisely the indications given by the physician, medical staff should collaborate in the development of high-quality sites for the patient to have additional sources of information.


Assuntos
Comportamento de Busca de Informação , Internet , Lúpus Eritematoso Sistêmico , Adulto , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Distribuição por Sexo , Espanha , Inquéritos e Questionários
10.
Mol Biol Rep ; 40(8): 4851-6, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23645042

RESUMO

To determine whether the IL2/IL21 region, a general autoimmunity locus, contributes to the observed variation in response to rituximab in patients with systemic lupus erythematosus as well as to analyze its influence in a cohort including other autoimmune diseases. rs6822844 G/T polymorphism at the IL2-IL21 region was analyzed by TaqMan assay in 84 systemic lupus erythematosus (SLE) and 60 different systemic autoimmune diseases Spanish patients receiving rituximab. Six months after the first infusion patients were classified, according to the EULAR criteria, as good responders, partial responders and non-responders. A statistically significant difference was observed in GG genotype frequency between responder (total and partial response) (83.56%) and non-responder (45.45%) SLE patients (p=0.010, odds ratio (OR)=6.10 [1.28-29.06]). No association with the response was evident in the group of patients with autoimmune diseases other than lupus. Furthermore, when both groups of patients were pooled in a meta-analysis, a reduced statistical significance of the association was observed (p=0.024, OR=3.53 [1.06-11.64]). Our results show for a first time that IL2-IL21 region seems to play a role in the response to rituximab in SLE patients but not in other autoimmune diseases.


Assuntos
Anticorpos Monoclonais Murinos/farmacologia , Doenças Autoimunes/tratamento farmacológico , Interleucina-2/genética , Interleucinas/genética , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Farmacogenética/métodos , Anticorpos Monoclonais Murinos/uso terapêutico , Doenças Autoimunes/genética , Doenças Autoimunes/imunologia , Regulação da Expressão Gênica , Genótipo , Humanos , Lúpus Eritematoso Sistêmico/genética , Lúpus Eritematoso Sistêmico/imunologia , Razão de Chances , Polimorfismo de Nucleotídeo Único/genética , Rituximab , Espanha
11.
DNA Cell Biol ; 31(12): 1671-7, 2012 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-23075294

RESUMO

Rituximab is being used as treatment for systemic autoimmune diseases. The objective of this study was to determine whether the genetic variant in the Fc gamma-receptor III a (FCGR3A) gene, 158F/V, contributes to the observed variation in response to rituximab in patients with systemic autoimmune diseases. DNA samples from 132 Spanish patients with different systemic autoimmune diseases receiving rituximab were genotyped for FCGR3A-158F/V (rs396991) gene polymorphism using the TaqMan(®) allelic discrimination technology. Six months after infusion with rituximab we evaluated the response to the drug: 61% of the patients showed a complete response, partial 27% and 12% did not respond to the treatment. A statistically significant difference was observed in V allele frequency between responder (38%) and nonresponder (16%) patients (p=0.01; odds ratio [OR]=3.24, 95% confidence interval [CI] 1.17-11.1). Rituximab was also more effective in V allele carriers (94%) than in homozygous FF patients (81%): p=0.02; OR=3.96, 95% CI 1.10-17.68. These results suggest that FCGR3A-158F/V (rs396991) gene polymorphism play a role in the response to rituximab in autoimmune diseases. Validation of these findings in independent cohorts is warranted.


Assuntos
Anticorpos Monoclonais Murinos/uso terapêutico , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/genética , Polimorfismo de Nucleotídeo Único , Receptores de IgG/genética , Alelos , Estudos de Coortes , Grupo com Ancestrais do Continente Europeu , Feminino , Frequência do Gene , Homozigoto , Humanos , Infusões Intravenosas , Masculino , Receptores de IgG/metabolismo , Rituximab
14.
DNA Cell Biol ; 31(9): 1486-91, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22734797

RESUMO

Rituximab has become a pivotal treatment for systemic autoimmune diseases. The aim of this study was to determine whether the genetic variant -174 IL-6 contributes to differences in the response to rituximab in patients with systemic autoimmune diseases, including systemic lupus erythematosus (SLE), inflammatory myopathies, anti-neutrophil cytoplasmic antibody-mediated vasculitis, systemic sclerosis, Sjöegren's syndrome, rheumatoid arthritis, and autoimmune hemolytic anemia. DNA samples from 144 Spanish patients with different systemic autoimmune diseases receiving rituximab were genotyped for -174 IL-6 (rs1800795) gene polymorphism using the TaqMan(®) allelic discrimination technology. Six months after the first infusion with rituximab, we evaluated the response to the drug: 60.4% of the patients showed a complete response, partial 27.8%, and 11.8% did not respond to the treatment. The CC genotype frequency was significantly increased in nonresponders with respect to responders (23.5% vs. 7.1%, respectively; p=0.049; odds ratio (OR)=4.03, 95% confidence intervals (CI) 0.78-16.97). According to the genotype distribution, rituximab was effective in 69.2% of the CC carriers, 91.9% of the CG carriers, and 88.4% of the GG carriers. A similar trend was observed when SLE patients were analyzed separately (27.3% carried CC homozygosis in nonresponders and 6.9% in responders; p=0.066; OR=5.10, 95% CI 0.65-31.73). Rituximab was effective in 62.5% of the CC carriers, 88.9% of the GC carriers, and 90% of the GG carriers. These results suggest that -174 IL-6 (rs1800795) gene polymorphism plays a role in the response to rituximab in systemic autoimmune diseases. Validation of these findings in independent cohorts is warranted.


Assuntos
Anticorpos Monoclonais Murinos/uso terapêutico , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/genética , Interleucina-6/genética , Polimorfismo de Nucleotídeo Único , Feminino , Frequência do Gene/genética , Humanos , Masculino , Rituximab , Resultado do Tratamento
15.
Semin Arthritis Rheum ; 41(6): 789-800, 2012 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-22169458

RESUMO

OBJECTIVE: To investigate the incidence of clinical and immunological characteristics of a large cohort of Spanish patients with scleroderma (SSc) and identifying factors associated with particular organ manifestations assessed by a nationwide cross-sectional analysis. METHODS: We classified SSc patients in 4 subsets using a modification of LeRoy and Medsger classification that included: "prescleroderma" (pre-SSc), limited cutaneous SSc (lcSSc), diffuse cutaneous SSc (dcSSc), and SSc sine scleroderma (ssSSc). Fourteen Spanish centers participated in patient recruitment. On January 2008, the database included 916 consecutive Spanish SSc patients, 801 women (87.4%) and 115 men (12.6%), all of whom fulfilled the classification criteria proposed by LeRoy and Medsger. Epidemiological, clinical, and laboratory data were collected according to a standard protocol. Mean age at diagnosis was 51.2 ± 15.1 years and mean age at disease onset was 44.9.0 ± 15.8 years. lcSSc was the most frequent subset (61.8%) followed by dcSSc (26.5%), ssSSc (7.5%), and preSSc (4%) subsets. Gender ratios were as follows: dcSSc subset, 200 women and 43 men (4.7:1); lcSSc subset, 503 women and 63 men (ratio 7.9:1), and ssSSc subset, 62 women and 7 men (ratio 8.9:1). Digital ulcers, interstitial lung disease (ILD), musculoeskeletal and esophageal involvement, and scleroderma renal crisis were more frequent in dcSSc than lcSSc and ssSSc subsets. The incidence of pulmonary arterial hypertension assessed by echocardiography was similar in all subsets but mean estimated systolic pulmonary arterial pressure was higher in ssSSc than in lcSSc subset (47.3 ± 23.9 mm Hg vs 39.6 ± 19.2 mm Hg; P < 0.03). Cardiac involvement was identified more frequently in ssSSc than in dcSSc and lcSSc subsets (49.3% vs 32.5% and 31.1%, respectively; P = 0.015 and P = 0.004 for both comparisons). Acro-osteolysis (8.2% vs 2.4%, P = 0.049), calcinosis (19.8% vs 7.2%, P < 0.05), and sicca syndrome (37.5% vs 14.5%, P < 0.0001) were more frequent in lcSSc than in ssSSc subsets. The frequency of clinical manifestations related to the presence of anticentromere antibodies or antitopoisomerase I antibodies was very similar to that identified in patients categorized to lcSSc and dcSSc, respectively. However, in multivariate studies, the ranking of the variables according to their overall explanatory effect on the model showed that the contributory effect of the antibody status was not greater than that of the clinical categorization into lcSSc and dcSSc for the majority of disease manifestations, but, in important manifestations, as ILD, absence of anticentromere antibodies was an independent predictor factor. CONCLUSIONS: The classification of SSc into dcSSc, lcSSc, and ssSSc subsets is the one that most closely reflects the natural history of the disease, as they presented clear clinical differences. The immunological profile helps to define important visceral alteration as ILD. Finally, to improve early diagnosis of SSc, patients with preSSc should be considered both to trace the true evolution of the disease and to define which patients could benefit from therapeutic measures able to prevent the appearance of visceral involvements.


Assuntos
Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Adulto , Idoso , Estudos Transversais , Feminino , Humanos , Incidência , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Masculino , Pessoa de Meia-Idade , Fibrose Pulmonar/complicações , Fibrose Pulmonar/diagnóstico , Sistema de Registros , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/imunologia , Espanha/epidemiologia
16.
Spine (Phila Pa 1976) ; 36(26): 2299-303, 2011 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-21494191

RESUMO

STUDY DESIGN: Prospective, intraoperative force measurement in consecutive lengthening procedures in a series of growing-rod patients undergoing lengthening. OBJECTIVE: The purpose of this study was to measure the forces and amount of distraction over time in early onset scoliosis patients treated with growing rods. SUMMARY OF BACKGROUND DATA: Growing rods are one of the current techniques used in the treatment of early onset scoliosis, and the goal of the growing-rod technique is to achieve deformity correction, maintaining spinal growth at the same time. Gradual stiffening or spontaneous fusion of the spine can interfere with the ability to lengthen. In addition, diminished acquired length with serial distraction are common observations and need to be evaluated and quantified. METHODS: Distraction forces were measured prospectively during 60 consecutive lengthening procedures in 26 patients. All patients had single submuscular rod constructs with side-to-side connectors. For each measurement, output from a transducer on a dedicated pair of distraction calipers was recorded at zero load status and the force was then recorded at every 1 mm lengthening; length was obtained at each event and was recorded in millimeters. RESULTS: The force required to distract the spine doubled at the 5th lengthening procedure (mean 368 N ± 54 N), and the distraction force was significantly higher at the fifth lengthening compared with the previous lengthening (P <0.01). Mean length achieved at each distraction decreased over time such that by the fifth lengthening, consistently 8 mm or less was achieved. CONCLUSION: Distraction forces increase significantly after repeated lengthening of growing-rod constructs, and the length obtained at each procedure exhibits a decreasing trend.


Assuntos
Osteogênese por Distração/métodos , Escoliose/cirurgia , Coluna Vertebral/cirurgia , Criança , Pré-Escolar , Humanos , Osteogênese por Distração/instrumentação , Estudos Prospectivos , Coluna Vertebral/crescimento & desenvolvimento , Fatores de Tempo , Resultado do Tratamento
17.
Spine (Phila Pa 1976) ; 35(25): 2252-8, 2010 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-21102301

RESUMO

STUDY DESIGN: Retrospective clinical and radiologic review of consecutive series of patients treated with single submuscular growing rods from a single center with a minimum of 2-year follow-up. OBJECTIVES: To describe the surgical technique and methods used to minimize complications and to report on the outcomes of a large consecutive series of patients treated with single submuscular growing rods for scoliosis in the immature spine from a single center. SUMMARY OF BACKGROUND DATA: Previous studies have reported on the safety and efficacy of single and dual growing-rod constructs; however, these studies have been of small patient numbers with varying results. METHODS: Between 1999 and 2007, 88 patients underwent the insertion of a single, submuscular growing-rod construct for scoliosis. A clinical and radiologic review of these 88 consecutive patients with a minimum of 2-year follow-up was conducted. Diagnoses include idiopathic, neuromuscular, syndromic, and congenital. Data include Cobb angle measurements, T1-S1 heights, number, and frequency of lengthening as well as complications. RESULTS: The patients underwent single submuscular growing-rod insertion at an average age of 7.0 years. The mean follow-up period was 42 months. Twenty-eight patients had a simultaneous apical fusion. Growing-rod lengthening was performed on an average at 9-month intervals. The average initial Cobb angle was 73° (range: 40-117) and improved to 44° (range: 9-90) at final follow-up. T1-S1 height gain was 3.37 cm; this translates to 1.04 cm growth/yr. No significant difference was noted between those who had undergone apical fusion and those without. Complications noted in this series include 8 incidences of superficial infection and 3 of deep infection, proximal junctional kyphosis in 2 patients requiring early fusion, 31 rod fractures, 10 cases of proximal anchor failure, and 6 distal anchor failures. Thirty patients within study group have reached definitive fusion. CONCLUSION: Favorable outcomes have been demonstrated in this large single-center series of growing-rod constructs used to treat scoliosis in the growing spine. Their safety and efficacy in controlling spinal deformity and allowing spinal growth along with an acceptable rate of complications would support the continued use of single growing-rod constructs as a scoliosis management option.


Assuntos
Fixadores Internos/efeitos adversos , Escoliose/cirurgia , Fusão Vertebral/efeitos adversos , Fusão Vertebral/instrumentação , Coluna Vertebral/cirurgia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Estudos Retrospectivos , Fusão Vertebral/métodos , Coluna Vertebral/crescimento & desenvolvimento , Resultado do Tratamento
18.
Ann Rheum Dis ; 69(12): 2083-9, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-20833738

RESUMO

BACKGROUND: Long-term immunosuppressive treatment does not efficiently prevent relapses of lupus nephritis (LN). This investigator-initiated randomised trial tested whether mycophenolate mofetil (MMF) was superior to azathioprine (AZA) as maintenance treatment. METHODS: A total of 105 patients with lupus with proliferative LN were included. All received three daily intravenous pulses of 750 mg methylprednisolone, followed by oral glucocorticoids and six fortnightly cyclophosphamide intravenous pulses of 500 mg. Based on randomisation performed at baseline, AZA (target dose: 2 mg/kg/day) or MMF (target dose: 2 g/day) was given at week 12. Analyses were by intent to treat. Time to renal flare was the primary end point. Mean (SD) follow-up of the intent-to-treat population was 48 (14) months. RESULTS: The baseline clinical, biological and pathological characteristics of patients allocated to AZA or MMF did not differ. Renal flares were observed in 13 (25%) AZA-treated and 10 (19%) MMF-treated patients. Time to renal flare, to severe systemic flare, to benign flare and to renal remission did not statistically differ. Over a 3-year period, 24 h proteinuria, serum creatinine, serum albumin, serum C3, haemoglobin and global disease activity scores improved similarly in both groups. Doubling of serum creatinine occurred in four AZA-treated and three MMF-treated patients. Adverse events did not differ between the groups except for haematological cytopenias, which were statistically more frequent in the AZA group (p=0.03) but led only one patient to drop out. CONCLUSIONS: Fewer renal flares were observed in patients receiving MMF but the difference did not reach statistical significance.


Assuntos
Azatioprina/uso terapêutico , Imunossupressores/uso terapêutico , Nefrite Lúpica/tratamento farmacológico , Ácido Micofenólico/análogos & derivados , Adulto , Azatioprina/efeitos adversos , Ciclofosfamida/uso terapêutico , Quimioterapia Combinada , Feminino , Seguimentos , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/efeitos adversos , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/efeitos adversos , Ácido Micofenólico/uso terapêutico , Prednisolona/uso terapêutico , Prevenção Secundária , Resultado do Tratamento , Adulto Jovem
19.
Arthritis Rheum ; 62(11): 3404-14, 2010 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-20669283

RESUMO

OBJECTIVE: Costimulatory receptor CD226 plays an important role in T cell activation, differentiation, and cytotoxicity. This study was undertaken to investigate the genetic association of CD226 with susceptibility to systemic lupus erythematosus (SLE) and to assess the functional implications of this association. METHODS: Twelve tag single-nucleotide polymorphisms (SNPs) in CD226 were typed in 1,163 SLE patients and 1,482 healthy control subjects from Europe or of European ancestry. Analyses of association were performed by single-marker Cochran-Mantel-Haenszel meta-analysis, followed by haplotype analysis. Gene expression was analyzed by quantitative real-time polymerase chain reaction analyses of RNA from peripheral blood mononuclear cells, and by fluorescence-activated cell sorter analysis. To study the functional impact of the associated variants, luciferase reporter constructs containing different portions of the 3'-untranslated region (3'-UTR) of the gene were prepared and used in transfection experiments. RESULTS: A 3-variant haplotype, rs763361;rs34794968;rs727088 (ATC), in the last exon of CD226 was associated with SLE (P = 1.3 × 10(-4) , odds ratio 1.24, 95% confidence interval 1.11-1.38). This risk haplotype correlated with low CD226 transcript expression and low CD226 protein levels on the surface of CD4+ and CD8+ T cells and natural killer T (NKT) cells. NK cells expressed high levels of CD226, but this expression was independent of the haplotype. Reporter assays with deletion constructs indicated that only the presence of rs727088 could account for the differences in the levels of luciferase transcripts. CONCLUSION: This study identified an association of CD226 with SLE in individuals of European ancestry. These data support the importance of the 3'-UTR SNP rs727088 in the regulation of CD226 transcription both in T cells and in NKT cells.


Assuntos
Regiões 3' não Traduzidas/genética , Antígenos de Diferenciação de Linfócitos T/genética , Lúpus Eritematoso Sistêmico/genética , Linfócitos T/imunologia , Regiões 3' não Traduzidas/imunologia , Alelos , Antígenos de Diferenciação de Linfócitos T/imunologia , Diferenciação Celular/genética , Diferenciação Celular/imunologia , Grupo com Ancestrais do Continente Europeu/genética , Feminino , Estudos de Associação Genética , Predisposição Genética para Doença , Genótipo , Haplótipos , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Polimorfismo de Nucleotídeo Único
20.
Spine (Phila Pa 1976) ; 34(17): 1808-14, 2009 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-19644332

RESUMO

STUDY DESIGN: Retrospective study with clinical and radiologic evaluation of 15 patients with congenital kyphosis or kyphoscoliosis who underwent anterior instrumented spinal fusion for posterolateral or posterior hemivertebra (HV). The management of congenital kyphosis has been described in the literature using a variety of techniques. The presentation of patients at diagnosis is discussed. The question of when to begin treatment is reviewed. The pitfalls in the management and how to avoid these are discussed. The different published techniques are reviewed. We present our own techniques and our results of treatment of congenital kyphosis in very young children. OBJECTIVE: To evaluate the safety and efficacy of early surgical anterior instrumented fusion with partial preservation of the HV in the treatment of progressive congenital kyphosis in children below the age of 3. We discuss the management of patients presenting with neurologic compromise. We aim to systematically review the literature and to present our own experience in the management of these deformities, so that the issues common to treating physicians may be explored. SUMMARY OF BACKGROUND DATA: A variety of treatments have been described in the literature for the treatment of congenital kyphosis due to HV. We report the results of our technique. METHODS: Between 1997 and 2005 we have treated 15 consecutive patients with progressive congenital kyphosis with anterior instrumented fusion and strut grafting. Thirteen patients had a single posterolateral HV and 2 patients had a single posterior HV. Of the 15 patients in the study, 5 were girls and 10 boys. Mean age at surgery was 22 months (range, 8-33). Mean follow-up period was 6.8 years. Thirteen HV were located in the thoracolumbar junction (T10-L2) and 2 in the thoracic spine. RESULTS: The average operating time of procedure was 150 minutes (range, 130-210 minutes). The average blood loss was 180 mL (range, 100-330 mL), equivalent to a mean external blood volume loss of 15% (range, 11%-24%).Preoperative segmental Cobb angle averaging 34 degrees at last follow-up. Compensatory coronal cranial and caudal curves were corrected by 50%. The angle of segmental kyphosis averaged 39 degrees (range, 20 degrees-80 degrees) before surgery and 21 degrees (range, 11 degrees-40 degrees) at last follow-up. This represents a 43% of improvement of the segmental kyphosis, and a 64% of improvement of the segmental scoliosis at last follow-up. One case with initial kyphosis of 80 degrees continued to progress and required revision anterior and posterior surgery. There were no neurologic complications.


Assuntos
Procedimentos Neurocirúrgicos/instrumentação , Procedimentos Neurocirúrgicos/métodos , Doença de Scheuermann/cirurgia , Coluna Vertebral/anormalidades , Coluna Vertebral/cirurgia , Distribuição por Idade , Transplante Ósseo/métodos , Pré-Escolar , Feminino , Humanos , Lactente , Fixadores Internos , Masculino , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/fisiopatologia , Complicações Pós-Operatórias/prevenção & controle , Hemorragia Pós-Operatória/etiologia , Hemorragia Pós-Operatória/prevenção & controle , Procedimentos Cirúrgicos Reconstrutivos/instrumentação , Procedimentos Cirúrgicos Reconstrutivos/métodos , Estudos Retrospectivos , Doença de Scheuermann/congênito , Doença de Scheuermann/patologia , Distribuição por Sexo , Fusão Vertebral/instrumentação , Fusão Vertebral/métodos , Coluna Vertebral/patologia , Resultado do Tratamento
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