Autoimmune diseases and pregnancy: analysis of a series of cases

BACKGROUND: An autoimmune disease is characterized by tissue damage, caused by self-reactivity of different effector mechanisms of the immune system, namely antibodies and T cells. All autoimmune diseases, to some extent, have implications for fertility and obstetrics. Currently, due to available treatments and specialised care for pregnant women with autoimmune disease, the prognosis for both mother and child has improved significantly. However these pregnancies are always high risk. The purpose of this study is to analyse the fertility/pregnancy process of women with systemic and organ-specific autoimmune diseases and assess pathological and treatment implications. METHODS: The authors performed an analysis of the clinical records and relevant obstetric history of five patients representing five distinct autoimmune pathological scenarios, selected from Autoimmune Disease Consultation at the Hospital of Braga, and reviewed the literature. RESULTS: The five clinical cases are the following: Case 1-28 years old with systemic lupus erythematosus, and clinical remission of the disease, under medication with hydroxychloroquine, prednisolone and acetylsalicylic acid, with incomplete miscarriage at 7 weeks of gestation without signs of thrombosis. Case 2-44 years old with history of two late miscarriages, a single preterm delivery (33 weeks) and multiple thrombotic events over the years, was diagnosed with antiphospholipid syndrome after acute myocardial infarction. Case 3-31 years old with polymyositis, treated with azathioprine for 3 years with complete remission of the disease, took the informed decision to get pregnant after medical consultation and full weaning from azathioprine, and gave birth to a healthy term new-born. Case 4-38 years old pregnant woman developed Behcet's syndrome during the final 15 weeks of gestation and with disease exacerbation after delivery. Case 5-36 years old with autoimmune thyroiditis diagnosed during her first pregnancy, with difficult control over the thyroid function over the years and first trimester miscarriage, suffered a second miscarriage despite clinical stability and antibody regression. CONCLUSIONS: As described in literature, the authors found a strong association between autoimmune disease and obstetric complications, especially with systemic lupus erythematosus, antiphospholipid syndrome and autoimmune thyroiditis.

A questionnaire to evaluate the impact of chronic diseases: validated translation and Illness Effects Questionnaire (IEQ) reliability study

INTRODUCTION: Patients' perception about their health condition, mainly involving chronic diseases, has been investigated in many studies and it has been associated to depression, compliance with the treatment, quality of life and prognosis. The Illness Effects Questionnaire (IEQ) is a tool which makes the standardized evaluation of patients' perception about their illness possible, so that it is brief and accessible to the different clinical settings. This work aims to begin the transcultural adaptation of the IEQ to Brazil through the validated translation and the reliability study. METHODS: The back-translation method and the test-retest reliability study were used in a sample of 30 adult patients under chronic hemodialysis. The reliability indexes were estimated using the Pearson, Spearman, Weighted Kappa and Cronbach's alpha coefficients. RESULTS: The semantic equivalence was reached through the validated translation. In this study, the reliability indexes obtained were respectively: 0.85 and 0.75 (p < 0.001); 0.68 and 0.92 (p < 0.0001). DISCUSSION: The reliability indexes obtained attest to the stability of responses in both evaluations. Additional procedures are necessary for the transcultural adaptation of the IEQ to be complete. CONCLUSION: The results indicate the translation validity and the reliability of the Brazilian version of the IEQ for the sample studied.

The choroid plexus is modulated by various peripheral stimuli: implications to diseases of the central nervous system

The blood brain barrier (BBB) and the blood cerebrospinal fluid barrier (BCSFB) form the barriers of the brain. These barriers are essential not only for the protection of the brain, but also in regulating the exchange of cells and molecules in and out of the brain. The choroid plexus (CP) epithelial cells and the arachnoid membrane form the BCSFB. The CP is structurally divided into two independent compartments: one formed by a unique and continuous line of epithelial cells that rest upon a basal lamina; and, a second consisting of a central core formed by connective and highly vascularized tissue populated by diverse cell types (fibroblasts, macrophages and dendritic cells). Here, we review how the CP transcriptome and secretome vary depending on the nature and duration of the stimuli to which the CP is exposed. Specifically, when the peripheral stimulation is acute the CP response is rapid, strong and transient, whereas if the stimulation is sustained in time the CP response persists but it is weaker. Furthermore, not all of the epithelium responds at the same time to peripheral stimulation, suggesting the existence of a synchrony system between individual CP epithelial cells.

Epidemiological characteristics of congenital heart diseases in Londrina, Paraná South Brazil

OBJECTIVE: To determine the prevalence and other epidemiological characteristics of congenital heart diseases. METHODS: A retrospective population based study of children who were born in Londrina, from January '89 to December '98 (80,262 live births). Diagnoses were confirmed through autopsy, surgery, catheterization, or echocardiography. RESULTS: A total of 441 patients was as certained what corresponds to a prevalence of 5.494:1,000 live births. Ventricular septal defect was the commonest lesion. A small number of transpositions of the great vessels and of left ventricular hypoplasia was observed. A high propation of ventricular septal defect (28.3%) and atrioventricular septal defects (8.1%) occurred. Fifty-one (11.35%) affected children had syndromic diseases and 52 (12.01%) children had nonsyndromic anomalies. CONCLUSION: The prevalence of congenital heart diseases in Londrina is in accordance with that of other regions of the globe. This prevalence also may reflect the reality in the southern region of Brazil, because population characteristics are very similar in the 3 southernmost Brazilian states.

Clinical and pathological assessment of 82 patients with cardiovascular diseases undergoing autopsy at the Hospital das Clínicas of the Faculdade de Medicina de Botucatu from 1988 to 1993

OBJECTIVE: To evaluated the clinical diagnostic, efficiency for basic death causes in patients dying of circulatory disease and de relative frequency of those diseases. METHODS: Analysis of medical record data of 82 patients, ages from 16 to 84 years old (68 over 40 years old), whose died of circulatory disease and had undergone necropsy in the period from 1988 to 1993 years in the University Hospital of Medicine Faculty of Botucatu-UNESP, Br. RESULTS: The functional class of patients were III or IV, in 78%, and 81.7% needed urgent hospitalization. By the clinical judgment the death were by ischemic heart disease in 32 (21 acute myocardial infarction), Chagas'disease in 12, valvopathy in 11, cardiomyopathy in 7, heart failure with no specification of cardiopathy in 11 and other causes in 9. At the necropsy the death cause was ischemic heart disease in 34 patients, valvopathy in 10, Chagas'disease in 10, cardiomyopathy in 5, and heart failure with no specification of cardiopathy in 2.The concordance taxes were in thhe same order: 94,6%, 90,0%, 83.3%, 71.4% and 28.5%. CONCLUSION: There was a great efficiency of clinical diagnosis for death cause in a general university hospital. The ischemic heart disease were the main causes of death.

Trends in death from circulatory diseases in Brazil between 1979 and 1996

OBJECTIVE: To analyze the trends in mortality due to circulatory diseases in men and women aged > or = 30 years in Brazil from 1979 to 1996. METHODS: We analyzed population count data obtained from the IBGE Foundation and mortality data obtained from the System of Information on Mortality of the DATASUS of the Ministry of Health. RESULTS: Circulatory diseases, ischemic heart disease, and cerebrovascular disease were the major causes of death in men and women in Brazil. The standardized age coefficient for circulatory disease in men aged > or = 30 years ranged from 620 to 506 deaths/100,000 inhabitants and in women from 483 to 383 deaths/100,000 inhabitants for the years 1979 and 1996, respectively. In men, the mean coefficient for the period was 586.25 deaths with a significant trend towards a decrease (P<0.001) and a decline of 8.25 deaths/year. In women, the mean coefficient for the period was 439.58 deaths, a significant trend towards a decrease (P<0.001) and a rate of decline of 7.53 deaths/year. The same significant trend towards a decrease in death (P<0.001) was observed for ischemic heart disease and cerebrovascular disease. Risk of death from these causes was always higher for men of any age group (P<0.001). Cerebrovascular disease was the primary cause of death in women. CONCLUSION: Although circulatory diseases have been the major cause of mortality in men and women in the Brazilian population, with a greater participation by cerebrovascular diseases, a trend towards a decrease in the risk of death from these causes is being observed.

Trends in the risk of mortality due to cardiovascular diseases in five Brazilian geographic regions from 1979 to 1996

OBJECTIVE - To analyze the trends in risk of death due to cardiovascular diseases in the northern, northeastern, southern, southeastern, and central western Brazilian geographic regions from 1979 to 1996. METHODS - Data on mortality due to cardiovascular, cardiac ischemic, and cerebrovascular diseases in 5 Brazilian geographic regions were obtained from the Ministry of Health. Population estimates for the time period from 1978 to 1996 in the 5 Brazilian geographic regions were calculated by interpolation with the Lagrange method, based on the census data from 1970, 1980, 1991, and the population count of 1996, for each age bracket and sex. Trends were analyzed with the multiple linear regression model. RESULTS - Cardiovascular diseases showed a declining trend in the southern, southeastern, and northern Brazilian geographic regions in all age brackets and for both sexes. In the northeastern and central western regions, an increasing trend in the risk of death due to cardiovascular diseases occurred, except for the age bracket from 30 to 39 years, which showed a slight reduction. This resulted from the trends of cardiac ischemic and cerebrovascular diseases. The analysis of the trend in the northeastern and northern regions was impaired by the great proportion of poorly defined causes of death. CONCLUSION - The risk of death due to cardiovascular, cerebrovascular, and cardiac ischemic diseases decreased in the southern and southeastern regions, which are the most developed regions in the country, and increased in the least developed regions, mainly in the central western region.

Trends of the Risk of Death due to Circulatory, Cerebrovascular, and Ischemic Heart Diseases in 11 Brazilian Capitals from 1980 to 1998

OBJECTIVE: To assess the trends of the risk of death due to circulatory (CD), cerebrovascular (CVD), and ischemic heart diseases (IHD) in 11 Brazilian capitals from 1980 to 1998. METHODS: Data on mortality due to CD, CVD and IHD were obtained from the Brazilian Health Ministry, and the population estimates were calculated by interpolation with the Lagrange method based on census data from 1980 and 1991 and the population count of 1996. The trends were analyzed with the multiple linear regression method. RESULTS: CD showed a trend towards a decrease in most capitals, except for Brasília, where a mild increase was observed. The cities of Porto Alegre, Curitiba, Rio de Janeiro, Cuiabá, Goiânia, Belém, and Manaus showed a decrease in the risk of death due to CVD and IHD, while the city of Brasília showed an increase in CVD and IHD. The city of São Paulo showed a mild increase in IHD for individuals of both sexes aged 30 to 39 years and for females aged 40 to 59 years. In the cities of Recife and Salvador, a reduction in CD was observed for all ages and both sexes. In the city of Recife, however, an increase in IHD was observed at younger ages (30 to 49 years), and this trend decreased until a mild reduction (-4%) was observed in males ³ 70 years. CONCLUSION: In general, a reduction in the risk of death due to CD and an increase in IHD were observed, mainly in the cities of Recife and Brasília.

Electrocardiographic abnormalities in neurological diseases

OBJECTIVE: To evaluate electrocardiographic abnormalities in patients with neurologic diseases. METHODS: We studied 161 patients with neurologic disorders by analyzing the 12-lead electrocardiogram during the pathological process. An expert who did not know anything about the patients evaluated the traces. RESULTS: Neurological process included brain tumor (41%), stroke (27.3%), cerebral aneurysm (15.5%), subarachnoid hemorrhage (6.8%), subdural hemorrhage (5%), and head injury (4.4%). Electrocardiograms were normal in 61% of cases, and the most frequent abnormality was ventricular repolarization (23.7%). The presence of T waves (4.6%) and prolonged QT intervals (8.8%) was the most characteristic of brain injuries. CONCLUSION: We observed a lower incidence of electrocardiographic abnormalities than that described in the literature.

Ligation of the thoracic duct for the treatment of chylothorax in heart diseases

In children, chylothorax occurs mainly after cardiac and thoracic surgeries. One of the recommended postsurgery treatments is ligation of the thoracic tract, when all other conservative treatments have failed. We report 4 cases of chylothorax in patients who were successfully treated with this approach, which resulted in a decrease in pleural drainage without recurrent chylothorax.