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Biomedical subjects

C P Simeón

Publications and source records attributed to C P Simeón.

14 recordsLinked to original sources

Mortality and prognostic factors in Spanish patients with systemic sclerosis.

OBJECTIVE: To determine survival and mortality in a cohort of Spanish patients with scleroderma (systemic sclerosis, SSc) and to analyse whether survival is influenced by demographic, clinical or immunological variables or the extent of skin involvement. METHODS: The study included 79 patients diagnosed with SSc and taking part in a study to determine the extent of sclerosis, visceral involvement and immunological alterations. We studied the number of observed and expected deaths (the expected number being based on age- and sex-specific rates in the background population) and derived standardized mortality ratios with their 95% confidence intervals (CI). Cumulative survival after onset of the first symptom was estimated according to the Kaplan-Meier method. The Cox method was used to identify the prognostic factors. RESULTS: The mortality rate was 0.0249 deaths per person-year. Survival at 15 yr was 0.62 (95% CI 0.410-0.778). The standardized mortality ratio was 429.4% (95% CI 222-750). On crude analysis, lung involvement [forced vital capacity (FVC) <70%, pulmonary hypertension], SSc renal crisis, an active capillaroscopic pattern, pericardial effusion and age over 60 yr at diagnosis were associated with shorter survival. On multivariate analysis, only age at diagnosis over 60 yr, FVC <70% and SSc renal crisis were independent prognostic factors. CONCLUSIONS: The mortality rate associated with SSc showed a four-fold increase compared with the background population. Lung involvement and sclerodermal renal crisis were found to be independently associated with reduced survival.

Adolescent↗

Morphologic capillary changes and manifestations of connective tissue diseases in patients with primary biliary cirrhosis.

The objective of this study was to investigate the presence of nailfold capillary abnormalities and extrahepatic signs of connective tissue disease in patients with primary biliary cirrhosis (PBC), as compared to patients with other chronic liver diseases. We evaluated 22 patients with PBC and 15 patients with other chronic liver diseases as a control group. Nailfold capillaroscopy was performed by two observers blinded to clinical findings using a Wild M3 stereomicroscope with an Intralux 5000 Volpi cold light lamp. We detected nailfold capillary abnormalities in 20 out of 22 (91%) PBC patients. Twelve of these 20 patients (54%) showed capillary alterations characteristic of systemic sclerosis. In the control group only two out of 15 patients (13%) presented alterations and in both cases they were a non-specific type. The presence of nailfold capillary abnormalities was significantly greater in PBC patients than in the control group (P < 0.001). Eleven out of the 22 PBC patients (50%) had extrahepatic signs of connective tissue disease and most of them were related to systemic sclerosis; patients with other chronic liver diseases did not present rheumatic manifestations (P < 0.001). In PBC patients there was a significant association between systemic sclerosis capillary pattern and rheumatic manifestations (P < 0.03). The high prevalence of nailfold capillary abnormalities characteristic of systemic sclerosis in patients with PBC and the correlation with sclerodermal manifestations suggests that this capillaroscopic finding could be a useful indicator to investigate rheumatic manifestations in these patients.

Adult↗

[Scleroderma and pregnancy: obstetrical complications and the impact of pregnancy on the course of the disease].

BACKGROUND: To describe the outcome of the pregnancy in patients with scleroderma. PATIENTS AND METHODS: Patients with scleroderma and control group were included in this retrospective study. Two groups were different in pregnant patients with scleroderma: pregnancy before scleroderma (A1) and pregnancy after scleroderma (A2). The presence of clinical problems during pregnancy and the outcome of scleroderma were collected in a questionnaire. Differences in the frequencies of complications were analyzed using the U Mann-Whitney, the chi-square or Fisher's exact test when necessary. RESULTS: The frequency of global fetal complications was increased in patients group, but there was no significantly increased frequency when variables were analyzed independently: number of births, miscarriages, fetal deaths, preterm births and low weight full term babies. There was no increased frequency of renal crisis, hypertension or eclampsia. Differences between diffuse and limited subsets were no observed. Improvement of scleroderma was seen in only 3 patients and worsening of skin thickening was experienced by 2 patients. CONCLUSIONS: The pregnant scleroderma patients are a group with high risk pregnancies and therefore well-supervised pregnancies are necessary.

Adult↗

Comprehensive noninvasive assessment of cardiac involvement in limited systemic sclerosis.

OBJECTIVE: To assess cardiovascular abnormalities in patients with limited systemic sclerosis (SSc), using noninvasive cardiac techniques. METHODS: Sixty-three patients with limited SSc were prospectively evaluated with Doppler echocardiography and thallium-201 perfusion scintigraphy after a cold-stress test and radionuclide ventriculography. RESULTS: In the patients with limited SSc, there was a significantly high prevalence of abnormal left- and right-diastolic function parameters (P = 0.001 and P = 0.0002, respectively), thickening of papillary muscles (46%; P = 0.003), and mild mitral regurgitation (49%; P < 0.0001), compared with controls. Systolic pulmonary arterial hypertension was detected in 9 patients (14%), and pericardial effusion in 11 patients (18%). In 64% of patients with limited SSc, an ischemic response was detected on the thallium cold-stress scan; similarly, an ischemic response was detected in 57% of patients with primary Raynaud's phenomenon (P < 0.0001 versus controls). CONCLUSION: Although the frequency of cardiovascular symptoms was low in patients with limited SSc, a significant rate of cardiovascular abnormalities was found by noninvasive cardiac techniques.

Adult↗

[Study of the capillary microscopy changes in scleroderma and their association with organ disease, clinical manifestations and disease progression].

BACKGROUND: The changes of nailfold capillaries detected by capillary microscopy is a characteristic frequently observed in systemic sclerosis. The relationship between the different capillary microscopic changes and clinical manifestation, organic disease and disease progression were studied. METHODS: Sixty-three patients were studied in whom capillary microscopy was performed and organic disease was determined by analytical and morphological examination. The presence of association between the capillary microscopic signs and clinical manifestation and disease progression were investigated by the chi-square and Fisher tests. RESULTS: Capillary microscopic alterations were found in 95% of the patients. The extense capillary loss was associated as statistically significant to the diffuse form of scleroderma. Statistically significant associations were not found between the different capillary microscopic signs and disease progression and organic disease (global and individual per each organ). CONCLUSIONS: Although capillary microscopic changes are present in most scleroderma patients they are not useful in the evaluation of organ involvement of the disease. The presence of extense capillary loss is linked to worse prognosis in its association to the diffuse form of scleroderma.

Adolescent↗

Platelet activation, endothelial cell dysfunction in the absence of anticardiolipin antibodies in systemic sclerosis.

The presence of anticardiolipin antibodies (aCL), von Willebrand factor activity and platelet function was studied in 35 patients with systemic sclerosis (SSc) scleroderma and 22 healthy controls. aCL positivity was observed in no patient with SSc or controls, whereas beta-thromboglobulin and platelet factor 4 levels were significantly higher in patients with SSc (p less than 0.001 and p less than 0.002, respectively). Furthermore, plasma from patients with SSc had a greater degree of aggregation to adenosine diphosphate 1 microM (p less than 0.05) but not to collagen or arachidonic acid. The plasma of patients with scleroderma also had increased von Willebrand factor activity compared with controls (p less than 0.001). We conclude that aCL appears not to play a central role in the pathogenesis of vascular and hemostatic alterations in SSc.

Adult↗

[Platelet function study in primary Raynaud's phenomenon and Raynaud's phenomenon associated with scleroderma].

We evaluated platelet function in 59 patients with Raynaud's phenomenon (RP): 24 had primary RP (PRP) and in 35 RP was associated with diffuse scleroderma (DS). In the group with PRP there were 10 males and 14 females, with a mean age of 43 +/- 12 years and a time of evolution of 5 +/- 5 years. In the group with RP associated with DS there were 31 females and 4 males with a mean age of 53 +/- 12 years and a time of evolution of 9 +/- 7 years. The control group consisted of 20 healthy individuals (14 males and 6 females with a mean age of 40 +/- 12 years). In all patients and controls beta-thromboglobulin (BTG) and platelet factor 4 (PF4) levels were measured in plasma, and platelet aggregation was evaluated in the presence of adenosine diphosphate (ADP), collagen and arachidonic acid. The patients with RP associated with DS had BTG and PF4 higher than those with PRP and controls (p less than 0.02). The BTG/PF4 ratio was also significantly greater in patients with DS (p less than 0.005). The platelets from patients with DS had a greater aggregation with ADP (1 microM and 0.5 microM) than those from PRP (p less than 0.03). We concluded that patients with RP associated with DS had in vivo activation and a greater aggregation of platelets, in contrast with the absence of these findings in the group with PRP and in controls.

Adult↗