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1.
BMC Pediatr ; 19(1): 461, 2019 11 26.
Artigo em Inglês | MEDLINE | ID: mdl-31771531

RESUMO

BACKGROUND: We have recognized 15 children with jSLE and the antecedent of IgA vasculitis (HSP). This association is not broadly present in the literature. AIM: To know the age and gender distribution of children with IgA vasculitis (HSP), compare it to our IgA vasculitis (HSP) + jSLE cases, and identify prognostic factors to develop jSLE within our case series, IgA vasculitis (HSP) vs. IgA vasculitis (HSP) + jSLE. METHODS: A systematic review was carried out to know the age and gender distribution of children with IgA vasculitis (HSP). The information obtained plus data from 110 children with IgA vasculitis (HSP) from the Instituto Nacional de Pediatría were used to compare groups and identify prognostic factors. We performed a case-control study in patients < 18 years, consisting of 15 cases retrospectively identified with IgA vasculitis (HSP) + jSLE, and 110 IgA vasculitis (HSP) control subjects. RESULTS: The information of 12,819 IgA vasculitis (HSP) subjects from the systematic review and 110 IgA vasculitis (HSP) controls was obtained and compared to our 15 IgA vasculitis (HSP) + jSLE cases. The mean age of IgA vasculitis (HSP) was 7.1-years vs. 10.4-years of IgA vasculitis (HSP) + jSLE at the HSP diagnosis. Female to male ratio of IgA vasculitis (HSP) was 1:1.33 vs. 1:0.25 of IgA vasculitis (HSP) + jSLE. Patients with IgA vasculitis (HSP) + jSLE had lower levels of Hemoglobin (Hb) compared to patients with IgA vasculitis (HSP) 109 g/L vs. 141 g/L. For the development of jSLE, we found older age and lower levels of Hb as prognostic factors with OR [95% CI]: 1.37 [1.06, 1.89] and 5.39 [2.69, 15.25], respectively. CONCLUSION: IgA vasculitis (HSP) + jSLE patients are older and have lower levels of Hb than patients with IgA vasculitis (HSP). It is necessary to confirm these findings through a prospective study.

2.
Rev. salud pública ; 19(4): 506-510, jul.-ago. 2017. tab, graf
Artigo em Espanhol | LILACS-Express | ID: biblio-903137

RESUMO

RESUMEN Objetivo Determinar la confiabilidad entre dos observadores y el cambio mínimo detectable de los estudios de neuroconducción para diagnosticar el síndrome de túnel carpiano. Métodos Se estudiaron 69 pacientes remitidos para estudio electrofisiológico por sospecha de síndrome de túnel carpiano. A los pacientes, se les realizaron dos exámenes, dos días diferentes, por dos evaluadores. Se evaluaron las latencias sensitivas y motoras de los nervios mediano y cubital; todos fueron clasificados como negativos, incipientes, leves, moderados, severos o extremos. Se calculó la variación relativa entre ensayos, el coeficiente de correlación intraclase, el índice de kappa, el límite de acuerdo y el cambio mínimo detectable. Resultados La variación relativa entre ensayos de la latencia motora del nervio mediano fue de -6,8 % a 15,9 % con coeficiente de correlación intraclase de 0,98 para la diferencia con la latencia del nervio cubital. El cambio mínimo detectable fue de 0,4ms. La variación relativa entre ensayos de la latencia sensitiva del nervio mediano fue -5,0 % a 11 % con coeficiente de correlación intraclase de 0,95 para la diferencia con el nervio cubital. El cambio mínimo detectable fue de 0,2ms. La clasificación ele trofisiológica coincidió en 93 % de los casos, índice de kappa de 0,89. Conclusiones Las latencias sensitivas y motoras del nervio mediano así como la diferencia de estas con el nervio cubital son medidas confiables. El cambio mínimo detectable obtenido en nuestro estudio le sirve al clínico para establecer si los cambios de las latencias en estudios consecutivos o después del tratamiento son significativos.


ABSTRACT Objective To determine the reliability of neuroconduction studies by comparing two observers and detecting minimum changes when diagnosing carpal tunnel syndrome. Methods Sixty-nine patients referred for electrophysiological study due to suspected carpal tunnel syndrome were studied. The patients underwent two examinations, performed by two evaluators, on two different days. Sensory and motor latencies of the median and ulnar nerves were evaluated; all were classified as negative, incipient, mild, moderate, severe or extreme. Relative interval variation, intraclass correlation coefficient, kappa index, limit of agreement and minimum detectable change were estimated. Results The relative variation of motor nerve latency of the median nerve was -6.8% to 15.9%, with intraclass correlation coefficient of 0.98 for the difference of median-ulnar nerve latency. The minimum detected change was 0.4ms, while the relative interval variation of sensory latency of the median nerve was -5.0% to 11%, with intraclass correlation coefficient of 0.95 for difference with the ulnar nerve. The minimum detectable change was 0.2ms. Electrophysiological classification agreed in 93% of the cases, with a kappa index of 0.89. Conclusions Sensory and motor latencies of the median nerve, as well as the difference between them and the ulnar nerve, are reliable measures. The minimum detectable change obtained in our study helps clinicians to establish whether changes in latencies in consecutive or post-treatment studies are significant.

3.
Rev Salud Publica (Bogota) ; 19(4): 506-510, 2017.
Artigo em Espanhol | MEDLINE | ID: mdl-30183855

RESUMO

OBJECTIVE : To determine the reliability of neuroconduction studies by comparing two observers and detecting minimum changes when diagnosing carpal tunnel syndrome. METHODS : Sixty-nine patients referred for electrophysiological study due to suspected carpal tunnel syndrome were studied. The patients underwent two examinations, performed by two evaluators, on two different days. Sensory and motor latencies of the median and ulnar nerves were evaluated; all were classified as negative, incipient, mild, moderate, severe or extreme. Relative interval variation, intraclass correlation coefficient, kappa index, limit of agreement and minimum detectable change were estimated. RESULTS : The relative variation of motor nerve latency of the median nerve was -6.8% to 15.9%, with intraclass correlation coefficient of 0.98 for the difference of median-ulnar nerve latency. The minimum detected change was 0.4ms, while the relative interval variation of sensory latency of the median nerve was -5.0% to 11%, with intraclass correlation coefficient of 0.95 for difference with the ulnar nerve. The minimum detectable change was 0.2ms. Electrophysiological classification agreed in 93% of the cases, with a kappa index of 0.89. CONCLUSIONS : Sensory and motor latencies of the median nerve, as well as the difference between them and the ulnar nerve, are reliable measures. The minimum detectable change obtained in our study helps clinicians to establish whether changes in latencies in consecutive or post-treatment studies are significant.


Assuntos
Síndrome do Túnel Carpal/diagnóstico , Nervo Mediano/fisiopatologia , Condução Nervosa , Nervo Ulnar/fisiopatologia , Adulto , Síndrome do Túnel Carpal/fisiopatologia , Eletrofisiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Reprodutibilidade dos Testes
4.
Carbohydr Polym ; 130: 141-8, 2015 Oct 05.
Artigo em Inglês | MEDLINE | ID: mdl-26076610

RESUMO

A multi-reactive polysaccharide-based inisurf (acting both as initiator and stabilizer) has been designed for the first time from dextran with the aim of preparing dextran-covered nanoparticles with covalent linkage between core and coverage. This inisurf was used for polymerizing butyl acrylate in miniemulsion by AGET-ATRP. Both hydrophobic phenoxy groups and initiator groups (bromoisobutyryl ester) were introduced within hydrophilic dextran chain, conferring it amphiphilic and macroinitiator characters. Amphiphilic properties of dextran inisurfs have been evidenced as well as their ability to stabilize the direct miniemulsion of n-butyl acrylate. After optimization of polymerization conditions with model studies, assays were successfully realized with dextran-based inisurfs. Because of their amphiphilic character, inisurfs migrated at oil/water interface and initiated polymerization from bromoisobutyryl ester groups. Therefore graft copolymers were produced at oil/water interface, due to the multifunctional character of these inisurfs and constituted the particle inner core with covalent links to the dextran coverage.


Assuntos
Dextranos/química , Emulsões/química , Polimerização , Interações Hidrofóbicas e Hidrofílicas , Cinética , Espectroscopia de Ressonância Magnética , Nanopartículas/química , Propriedades de Superfície , Tensoativos/química
5.
Arthritis Care Res (Hoboken) ; 66(1): 34-9, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23983057

RESUMO

OBJECTIVE: To evaluate agreement among musculoskeletal pediatric specialists in assessing radiographic joint damage in juvenile idiopathic arthritis (JIA). METHODS: Two pediatric rheumatologists, 2 pediatric radiologists, and 2 pediatric orthopedic surgeons evaluated independently 60 radiographs of both wrists and hands of children with polyarticular-course JIA. Films were scored using an adapted and simplified version of the Larsen score, ranging from 0-5. Study radiographs were selected from 568 films used in a previous study aimed to validate an adapted pediatric version of the Sharp/van der Heijde (SHS) score. To enable comparison of specialists' scores with the adapted SHS score, the 60 radiographs were divided into 6 classes of severity of damage based on quintiles of the adapted SHS score. Agreement was evaluated in terms of absolute agreement and through weighted kappa statistics. RESULTS: The pediatric radiologists tended to assign lower scores and to provide more frequently scores of 0 than did the other specialists. Weighted kappa for the 3 pairs of specialists ranged from 0.67-0.69, indicating substantial agreement. Absolute agreement ranged from 51.3-55.7%, depending on the pair of specialists examined. Both absolute and weighted kappa concordance between specialists' scores and the adapted SHS score were poorer for the pediatric radiologist than for the other specialists. CONCLUSION: We observed fair agreement in the assessment of radiographic damage among pediatric specialists involved in the care of children with JIA. The radiologists tended to be more reserved than the rheumatologists and orthopedic surgeons in labeling radiographs as damaged or in considering changes as important.


Assuntos
Artrite Juvenil/diagnóstico por imagem , Articulação da Mão/diagnóstico por imagem , Pediatria , Índice de Gravidade de Doença , Especialização , Articulação do Punho/diagnóstico por imagem , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Lactente , Masculino , Ortopedia , Radiografia , Radiologia , Reprodutibilidade dos Testes , Reumatologia
6.
Rev Alerg Mex ; 60(1): 38-40, 2013.
Artigo em Espanhol | MEDLINE | ID: mdl-24008068

RESUMO

Kawasaki disease is an acute, self-limiting vasculitis of unknown origin, characterized by fever, palms and soles edema, cervical lymphadenopathy, strawberry tongue, and non-exudative conjunctivitis. It is a multisystemic vasculitis that affects predominantly infants and young children. The most feared complication is the development of coronary aneurysms that occurs up to 25% of untreated patients; however there are reports of extra coronary involvement. Herein we present the case of a 2 year-old girl who had a severe symptomatology and persistent fever despite intravenous gammaglobulin. Two years later she presented right hemiparesia and headache, with data from CAT and MRI suggestive of brain mass and deviation of the midline, secondary to left frontoparietal haemorrhage that was treated with a craniotomy. She was discharged on prednisone, ASA and rehabilitation.


Assuntos
Hemorragias Intracranianas/etiologia , Síndrome de Linfonodos Mucocutâneos/complicações , Pré-Escolar , Feminino , Humanos
7.
J Clin Immunol ; 32(2): 207-11, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-22119934

RESUMO

OBJECTIVES: Chronic granulomatous disease is a rare phagocyte disorder characterized by an increased susceptibility to infections and inflammatory complications. We describe two patients with chronic granulomatous disease (CGD) complicated by macrophage activation syndrome (MAS) (secondary hemophagocytic lymphohistiocytosis) treated with intravenous immunoglobulin (IVIG). METHODS: A report of two cases of CGD complicated by MAS who were successfully treated with IVIG was made, and a comparison was made with ten other cases reported in the literature. RESULTS: MAS is a severe potentially fatal complication of CGD. Most cases are associated with Burkholderia cepacia and leishmaniasis infection. The treatment of these patients varies between centers, and one example is the use of the HLH-2004 protocol. IVIG could be an effective first line option for this complication in CGD patients. CONCLUSIONS: The exaggerated inflammatory response characteristic of CGD patients could play a role in the development of this complication. IVIG appears to be a safe and effective first line treatment in these patients.


Assuntos
Doença Granulomatosa Crônica/complicações , Imunoglobulinas Intravenosas/uso terapêutico , Síndrome de Ativação Macrofágica/complicações , Síndrome de Ativação Macrofágica/terapia , Adolescente , Criança , Pré-Escolar , Doença Granulomatosa Crônica/genética , Humanos , Lactente , Masculino , Resultado do Tratamento
8.
Rev Alerg Mex ; 59(1): 37-40, 2012.
Artigo em Espanhol | MEDLINE | ID: mdl-24007932

RESUMO

Henoch-Schönlein Purpura (HSP) and Kawasaki disease (KD) are the most frequent systemic vasculitis in childhood. Both diseases are clearly distinct and easily distinguishable. Despite their high frequency, the coexistence of both diseases in the same patient is very rare. The diagnosis of these two diseases is based on clinical features, but sometimes it may be difficult, since signs and symptoms can be atypical and occasionally there are overlapping features among different forms of vasculitis. We present a 5 year-old boy who showed KD and three years later he developed HSP. We discuss similarities and differences between these two systemic vasculitic diseases and make a review of the literature of the few cases reported where KD and PHS have coexisted. Although rare, these two diseases can be present in the same patient and should be treated accordingly.


Assuntos
Síndrome de Linfonodos Mucocutâneos , Púrpura de Schoenlein-Henoch , Humanos , Vasculite Sistêmica , Vasculite
9.
Pediatrics ; 128(6): e1633-5, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22106083

RESUMO

Kimura disease is an uncommon chronic inflammatory condition of unknown etiology and is characterized by painless subcutaneous nodules, usually affecting the head and neck, eosinophilia, and markedly elevated immunoglobulin E levels. Several reports have described the main modalities of treatment; both corticosteroids and surgery have provided good results, but occasionally corticosteroids cannot be tapered as the disease flares up. We report here the case of an 8-year-old boy diagnosed with Kimura disease who was successfully treated with 1 dose of intravenous immunoglobulin as a steroid-sparing agent.


Assuntos
Hiperplasia Angiolinfoide com Eosinofilia/tratamento farmacológico , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Criança , Humanos , Masculino
10.
Clin Exp Rheumatol ; 28(3): 424-33, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-20497629

RESUMO

OBJECTIVES: To evaluate change over time and level of agreement of renal-specific and multi-dimensional measures in juvenile systemic lupus erythematosus (SLE) with renal disease. METHODS: An analysis was made of 205/557 children with baseline 24-hour proteinuria >or=0.5 g. Data were collected at baseline, 6-, 12- and 24-month intervals. Using the Systemic Lupus International Collaborating Clinics (SLICC) renal index (change in proteinuria and urine sediment) as gold standard, responsiveness and discriminative ability analyses were used to identify key renal and multi-dimensional disease activity and damage measures for the evaluation of response to therapy. We also evaluated the kappa agreement between SLICC renal index and PRINTO/ACR juvenile SLE criteria (change in proteinuria, physician and parents evaluations, disease activity, health related quality of life [HRQOL]). RESULTS: Children with renal disease compared to children without renal disease, had a lower female rate and higher disease activity/response rate (p-values <0.01) but similar damage levels. Large responsiveness (standardised response mean >or=0.8) and statistical significant discriminative ability with the SLICC renal index 4 levels of response (improved, partially improved, stable and worsened) were observed for renal specific measures (proteinuria, urine sediment, renal sub-scores, p<0.0001) and for multi-dimensional variables (disease activity level and physician evaluation p<0.001). Agreement between the SLICC renal index and PRINTO/ACR criteria was moderate (0.57; 95% confidence intervals: 0.44-0.71). CONCLUSIONS: We propose to incorporate multi-dimensional measures (physician and parents' evaluations, disease activity and HRQOL), in addition to renal specific measures, in future clinical trials in juvenile SLE with renal involvement.


Assuntos
Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/fisiopatologia , Proteinúria/diagnóstico , Proteinúria/fisiopatologia , Índice de Gravidade de Doença , Biomarcadores , Criança , Ensaios Clínicos como Assunto/métodos , Ensaios Clínicos como Assunto/normas , Bases de Dados Factuais , Monitoramento de Medicamentos/métodos , Monitoramento de Medicamentos/normas , Nível de Saúde , Humanos , Rim/fisiologia , Estudos Longitudinais , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Proteinúria/tratamento farmacológico , Qualidade de Vida , Padrões de Referência , Urina
11.
Eur J Hum Genet ; 15(3): 336-41, 2007 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-17228327

RESUMO

A regulatory single nucleotide polymorphism (SNP) PD1.3G/A located on programmed cell death 1 (PDCD1) gene, was shown to be involved in susceptibility to systemic lupus erythematosus (SLE) in Swedish, European American, and Mexican cases. However, association to childhood-onset SLE has not been analyzed. The aim of this study was to investigate the association of PDCD1 polymorphisms and haplotypes with susceptibility to childhood-onset SLE in Mexican population. Three PDCD1 SNPs, PD1.3G/A, PD1.5C/T, PD1.6G/A, were analyzed in 250 childhood-onset SLE Mexican patients and 355 healthy controls in a case-control association study. Polymorphisms were genotyped by TaqMan technology. Stratification analysis was performed on the SLE cohort to investigate the SNP association with renal disorder. In addition, haplotypes were constructed with these three SNPs. The PD1.3A allele was significantly associated to childhood-onset SLE (P=0.0019, odds ratio (OR) 2.73, 95% confidence interval (95% CI) 1.35-5.56). The other PDCD1 SNPs did not show association. A total of 155 patients (62%) had nephritis, and no association was observed with PDCD1 SNPs. The ACG haplotype (PD1.3A, PD1.5C, PD1.6G) included almost all PD1.3A alleles, and it was more frequent in SLE patients (5.5%) than in controls (2.1%) (P=0.003; OR 2.73, 95% CI 1.37-5.46). The haplotype structure in Mexican controls was significantly different from those reported in Spanish and Swedish. Our results support association of the PD1.3A SNP to susceptibility of childhood-onset SLE in Mexican population and does not show association to lupus nephritis in this age group.


Assuntos
Antígenos CD/genética , Proteínas Reguladoras de Apoptose/genética , Predisposição Genética para Doença , Lúpus Eritematoso Sistêmico/genética , Polimorfismo Genético , Idade de Início , Estudos de Casos e Controles , Criança , Feminino , Humanos , Lúpus Eritematoso Sistêmico/epidemiologia , Nefrite Lúpica/genética , Masculino , Receptor de Morte Celular Programada 1
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