Analysis of pregnancy outcomes, disease progression, and risk factors in patients with undifferentiated connective tissue disease / 北京大学学报(医学版)

OBJECTIVE@#To investigate the fetal and maternal outcomes, risk factors of disease progression and adverse pregnancy outcomes (APOs) in patients with undifferentiated connective tissue disease (UCTD).@*METHODS@#This retrospective study described the outcomes of 106 pregnancies in patients with UCTD. The patients were divided into APOs group (n=53) and non-APOs group (n=53). The APOs were defined as miscarriage, premature birth, pre-eclampsia, premature rupture of membranes (PROM), intrauterine growth restriction (IUGR), postpartum hemorrhage (PPH), and stillbirth, small for gestational age infant (SGA), low birth weight infant (LBW) and birth defects. The differences in clinical manifestations, laboratory data and pregnancy outcomes between the two groups were compared. Logistic regression analysis was performed to analyze the risk factors for APOs and the progression of UCTD to definitive CTD.@*RESULTS@#There were 99 (93.39%) live births, 4 (3.77%) stillbirths and 3 (2.83%) miscarriage, 20 (18.86%) preterm delivery, 6 (5.66%) SGA, 17 (16.03%) LBW, 11 (10.37%) pre-eclampsia, 7 (6.60%) cases IUGR, 19 (17.92%) cases PROM, 10 (9.43%) cases PPH. Compared with the patients without APOs, the patients with APOs had a higher positive rate of anti-SSA antibodies (73.58% vs. 54.71%, P=0.036), higher rate of leukopenia (15.09% vs. 3.77%, P=0.046), lower haemoglobin level [109.00 (99.50, 118.00) g/L vs. 124.00 (111.50, 132.00) g/L, P < 0.001].Multivariate Logistic regression analysis showed that leucopenia (OR=0.82, 95%CI: 0.688-0.994) was an independent risk factors for APOs in UCTD (P=0.042). Within a mean follow-up time of 5.00 (3.00, 7.00) years, the rate of disease progression to a definite CTD was 14.15%, including 8 (7.54%) Sjögren's syndrome, 4 (3.77%) systemic lupus erythematosus (SLE), 4 (3.77%) rheumatoid arthritis and 1 (0.94%) mixed connective tissue disease. Multivariate Cox proportional risk regression analysis showed that Raynaud phenomenon (HR=40.157, 95%CI: 3.172-508.326) was an independent risk factor for progression to SLE.@*CONCLUSION@#Leukopenia is an independent risk factor for the development of APOs in patients with UCTD. Raynaud's phenmon is a risk factor for the progression of SLE. Tight disease monitoring and regular follow-up are the key measures to prevent adverse pregnancy outcomes and predict disease progression in UCTD patients with pregnancy.

Clinical Features of Neuromyelitis Optica Spectrum Disorders with Connective Tissue Diseases / 中国医学科学院学报

Objective To investigate the clinical features of neuromyelitis optica spectrum disorders(NMOSD)with connective tissue diseases(CTD). Methods Clinical data of 16 NMOSD-CTD patients and 54 NMOSD patients admitted to the Second Affiliated Hospital of Fujian Medical University from January 2015 to February 2020 were collected.The initial symptom,intracranial lesion,spinal cord lesion,laboratory examination and treatment response were compared between the two groups. Results The incidence of Sjögren's syndrome(SS)was the highest(10/16,62.5%)in NMOSD-CTD group.The NMOSD-CTD group had significantly higher positive rate of aquaporin-4 immunoglobulin G(AQP4-IgG)in serum or cerebrospinal fluid(100% vs. 70.2%,P=0.009),higher positive rates of serum anti-nuclear antibodies,anti Sjögren's syndrome A antibodies and anti-Ro52 autoantibodies(P0.05).Conclusions NMOSD is often complicated with CTD,and SS is the most common one.The positive rate of serum or cerebrospinal AQP4-IgG and the seropositivity of several other autoantibodies in NMOSD-CTD patients were higher than those in NMOSD patients.Neurological impairment in NMOSD-CTD patients were severer,which should arouse attention of clinicians.

Absenteísmo-doença no serviço público municipal da Prefeitura Municipal de Vitória / Sickness-absenteeism among municipal civil servants in Vitória, Brazil

Contexto: O conhecimento sobre as doenças que levam ao afastamento laboral constitui uma ferramenta importante para a elaboração de medidas que possam tornar o ambiente de trabalho mais seguro e agradável para o trabalhador, reduzindo o índice de absenteísmo-doença. Objetivo: Estudar as causas de absenteísmo-doença entre os servidores públicos da Prefeitura Municipal de Vitória (Espírito Santo, Brasil) no ano de 2012. Métodos: Estudo epidemiológico retrospectivo e descritivo. Os dados foram fornecidos pelo Serviço de Medicina Ocupacional da Prefeitura Municipal de Vitória. Resultados: As doenças dos aparelhos respiratório e osteomuscular e do tecido conjuntivo foram as principais causas de absenteísmo-doença entre os servidores municipais em 2012. No período avaliado, foram concedidas 14.271 licenças para tratamento de saúde com duração média de 10,2 dias. O percentual de absenteísmodoença encontrado foi de 4,79%. Conclusão: Este trabalho demonstrou que as doenças dos aparelhos respiratório e osteomuscular e do tecido conjuntivo foram as principais causas de absenteísmo-doença no serviço público da Prefeitura Municipal de Vitória.

Context: Knowing the diseases that lead to leave of absence is important for the development of measures capable of creating the conditions for a safer and more pleasant working environment for the workers, which may reduce sickness-absenteeism index. Objective: To identify the causes of sickness-absenteeism among municipal civil servants in Vitória (Espírito Santo, Brazil) in 2012. Methods: This is a descriptive and retrospective epidemiological study based on information disclosed by the Office of Occupational Medicine of the municipal government of Vitória. Results: Respiratory, musculoskeletal, and connective tissue disorders were the main causes of absenteeism among civil servants in 2012. During this period, 14,271 sick leaves were granted, with an average duration of 10.2 days. The percentage of absenteeism found was 4.79%. Conclusion: The main causes of sickness-absenteeism among municipal civil servants in Vitória were diseases of the respiratory and musculoskeletal systems and connective tissue.

Aortic Pathologies and Pregnancy: A Special Focus on Connective Tissue Disorders.

Pregnancy represents a physiological status which, due to clear and typical hormonal and hemodynamic changes, is frequently accompanied by a high morbidity of aortic structures, in all of its segments. Such morbidity might become patent when a pregestational situation exists, especially with the mother suffering from connective tissue disorders. Nevertheless, morbid occurrences that will be met only during pregnancy are well known, with particular pathophysiological and etiological theories, as well as a diversity of treatments proposed in such an unusual setting. The authors discuss the main pathologies of aorta that are seen during pregnancy, from a theoretical point of view, and from a historical perspective as well. A special focus is made to the connective tissue disorders, and the theoretical considerations are illustrated with images of dissecting aneurysms of aorta.

Doenças do trabalho e benefícios previdenciários relacionados à saúde, Bahia, 2000 / Work-related diseases and health-related compensation claims, Northeastern Brazil, 2000 / Enfermedades del trabajo y beneficios de la previsión relacionados a la salud, Bahia (Brasil), 2000

OBJETIVO: Estimar a contribuição das doenças relacionadas ao trabalho nos afastamentos por problemas de saúde em geral e ocupacionais. MÉTODOS: Foram analisados dados sociodemográficos, ocupacionais e de saúde referentes a 29.658 registros dos benefícios por incapacidade temporária concedidos por agravos à saúde pelo Instituto Nacional do Seguro Social, no Estado da Bahia, em 2000. Foram considerados casos todos os diagnósticos clínicos constantes da CID-10, com exceção das causas externas e fatores que influenciam o contato com os serviços de saúde. A vinculação do diagnóstico com a ocupação baseou-se no código CID-10 e se a espécie do benefício era "acidentária". RESULTADOS: Dentre os benefícios, 3,1 por cento foram concedidos para doenças do trabalho: 70 por cento eram doenças do sistema osteomuscular e do tecido conjuntivo e 14,5 por cento do sistema nervoso. No geral, benefícios concedidos numa freqüência maior que o dobro da esperada foram: para tenossinovites na indústria da transformação (Razão de Proporção-RP=2,70), síndrome do túnel do carpo na intermediação financeira (RP=2,43) e transtornos do disco lombar no ramo de transporte, correio e telecomunicações (RP=2,17). Entretanto, não foi estabelecido nexo causal para estas doenças, nesses ramos de atividade, em percentual significativo de benefícios. CONCLUSÕES: Os resultados sugerem a existência de possíveis fatores de risco ocupacionais para enfermidades nesses ramos de atividade, como também o sub-registro da vinculação das patologias com o trabalho, camuflando a responsabilidade das empresas e a perspectiva de prevenção pela reorganização do trabalho.

OBJECTIVE: To estimate the contribution of work-related diseases to sick leaves due to general and occupational health problems. METHODS: Sociodemographic, occupational and health data from 29,658 records of temporary disability benefits, granted on account of health problems by the Instituto Nacional do Seguro Social (National Institute of Social Security) in the state of Bahia (Northeastern Brazil), were analyzed. All constant ICD-10 clinical diagnoses were taken into consideration, except for those referring to external causes and factors that influence contact with health services. The link between diagnosis and occupation was based on the ICD-10 code and whether the type of compensation was due to a "work-related accident/disease" or not. RESULTS: From all the benefits, 3.1 percent were granted due to work-related diseases: 70 percent were musculoskeletal system and connective tissue diseases, while 14.5 percent were related to the nervous system. In general, benefits granted at more than two times the expected frequency were as follows: tenosynovitis in the manufacturing sector (Proportion Ratio-PR=2.70), carpal tunnel syndrome in the financial intermediation sector (PR=2.43), and lumbar disc degeneration in the transportation, postal service and telecommunications sectors (PR=2.17). However, no causal connection could be established for these diseases, in these activity sectors, in a significant percentage of benefits. CONCLUSIONS: Results suggest the existence of possible occupational risk factors for diseases in these fields of activity, as well as the underreporting of the link between diseases and work, thus disguising the responsibility of companies and the perspective of prevention through work reorganization.

OBJETIVO: Estimar la contribución de las enfermedades relacionadas al trabajo en las licencias por problemas de salud en general y ocupacionales. MÉTODOS: Fueron analizados datos sociodemográficos, ocupacionales y de salud referentes a 29.658 registros de los beneficios por incapacidad temporal concedidos por agravamientos a la salud por el Instituto Nacional do Seguro Social, en el estado de Bahia (Nordeste de Brasil), en 2000. Fueron considerados como casos todos los diagnósticos clínicos constantes de CID-10, con excepción de las causas externas y factores que influencian el contacto con los servicios de salud. La vinculación del diagnóstico con la ocupación se baso en el código CID-10 y si la especie de beneficio era "accidentaria". RESULTADOS: De los beneficios, 3,1 por ciento fueron concedidos para enfermedades de trabajo: 70 por ciento eran enfermedades del sistema osteomuscular y del tejido conjuntivo y 14,5 por ciento del sistema nervioso. En general, beneficios concedidos en una frecuencia mas grande que el doble de lo esperado fueron: para tenossinovites en la industria de transformación (Razón de Proporción-RP=2,70), síndrome del túnel del carpo en la intermediación financiera (RP=2,43) y trastornos del disco lumbar en el ramo del transporte, correo y telecomunicaciones (RP=2,17). Sin embargo, no fue establecido nexo causal para estas enfermedades, en estos ramos de actividad, en porcentual significativo de beneficios. CONCLUSÕES: Los resultados sugieren la existencia de posibles factores de riesgo ocupacionales para enfermedades en estos ramos de actividad, como también el sub-registro de la vinculación de las patologías con el trabajo, camuflando la responsabilidad de las empresas y la perspectiva de premención por la reorganización del trabajo.

Enfermedades reumáticas y del tejido conectivo en niños de Santiago, Chile / Rheumatic and connective tissue diseases in children at Santiago, Chile

Se describen los resultados de una encuesta sobre enfermedades reumatológicas infantiles en pacientes de 15 años o menos de edad de los consultorios públicos y privados de reumatología pediátrica del área metropolitana de Santiago, cuyo diagnóstico hubiese quedado definido en el curso del año 1991. La población cubierta por dichos servicios fue estimada en 1.571.171 menores de 15 años. Durante 1991 hubo 2.807 consultas de reumatología infantil, de las cuales 478 (17 por ciento) correspondieron a nuevas consultas, entre las cuales se comprobaron 135 casos nuevos de enfermedades del tejido conectivo (incidencia estimada de 8.6 casos por 100.000). La artritis reumatoidea fue la afección más frecuente entre ellas (incidencia estimada 5,6 casos por 100.000)

Bloqueio atrioventricular fetal / Fetal atrioventricular block

Objetivo - Estudar a etiopatogenia e evolução clínica dos fetos portadores de bloqueio átrioventricular (BAY) com o objetivo de identificar fatores que afetam a história natural dessa entidade. Métodos - Ecocardiografia fetal associada ao modo M e doppler foi realizada em 600 gestantes entre 18 a 40 semanas por diversas indicações. Em 13 fetos (2,1%) diagnosticou-se BAV. Foram analisados o tipo de bloqueio, presença de cardiopatia congênita associada à síndrome de isomerismo atrial esquerdo, assim como a positividade para doença materna do tecido conectivo, clínica ou laboratorial. Resultados - BAV isolado foi encontrado em 6 fetos (5 casos de BAV total e 1 caso de BAV 2° grau 2:1). Neste grupo, 5 gestantes apresentaram positividade para anticorpos antinúcleo. Bloqueio associado à cardiopatia foi encontrado em 7 casos, sendo em 6 associados à síndrome de isomor fismo atrial esquerdo (5 casos de BAV total e 1 caso de BAV 2° grau 2:1). Conclusão - Os achados deste estudo concordam com dados de literatura sobre a possível relação causa-efeito entre anticorpos maternos, característicos da doença do tecido conectivo e o bloqueio cardíaco fetal isolado. Da mesma forma, concordam com a literatura a péssima evolução dos fetos portadores de bloqueio e anormalidade cardíaca estrutural, associada à síndrome do isomor fismo atrial esquerdo. Formas efetivas de terapia fetal não estão estabelecidas

Purpose - To study the pathogenesis, evolution and prognosis of the complete heart block of the fetus. Methods - Bidimensional echo cardiography associated to M-mode and doppler was performed in 600 patients. All cases of congenital heart block were referred because the fetuses presented hydrops, bradycardia and/or cardiac malformation suspected by routine ultrasound.Results - Isolated heart block was found in 6 fetuses (5 cases of complete type and 1 case of 2nd degree type 2:1). Heart block associated with complex cardiac disease and left atrial isomerism was found in 6 fetuses with no survivors (5 cases of complete type and 1 case of 2nd degree type 2:1). Heart block associated with átrioventricular discordante wasfound in 1 case. Conlusion - The findings of this study agree the literatura about the relation between maternal anti-RO antibodies and isolated complete heart block. We also found a poor prognosis in the group with heart block and complex cardiac malformations

Hospitalización por causa reumatológica: 20 años de experiencia en el Hospital Nacional Cayetano Heredia 1970 - 1989

Se realizó una evaluación epidemiológica retrospectiva de las enfermedades reumatológicas atendidas en los servicios de hospitalización de medicina del Hospital Nacional Cayetano Heredia entre 1970-1989. Se hicieron 2,500 admisiones a 2080 pacientes (1.23 hospitalizaciones por pacientes), 104 pacientes nuevos por año. La relación mujer/varón promedio fue 1.8/1. La edad promedio fue 37.8 años, con rango de 12 a 99 años, el 25.3 por ciento tuvo entre 20-29 años. El tiempo de hospitalización promedio fué 23.3 días. Los grupos de enfermedades atendidos más frecuentemente fueron: el I-Enfermedades Difusas del Tejido Conectivo, 52.5 por ciento; el IV-Reumatismos asociados a infección, 26 por ciento; el III-Enfermedad degenerativa artícular, 4.9 por ciento y el II-Artritis asociadas a espondilitis, 3.6 por ciento. La tasa de mortalidad global fué 91/29249; 0.31 por ciento, la relativa de 91/2028; 4.48 por ciento