Coronary stenting for ST-elevation myocardial infarction vs. other indications in patients on oral anticoagulation: any difference in in-hospital management and outcome?

AIM: The aim of this paper was to compare the in-hospital management and outcome of patients on oral anticoagulation (OAC) undergoing coronary artery stenting (PCI-S) for ST-elevation myocardial infarction (STEMI) vs. other indications. METHODS: One hundred and sixteen patients on OAC at the time of PCI-S who were prospectively enrolled in a multi-center, observational registry, were evaluated. Patients were segregated according to whether PCI-S was performed for STEMI (group 1) or other indications, such as non ST-elevation acute coronary syndromes, stable angina, silent ischemia, etc. (group 2), and the pharmacological and procedural management, as well as the in-hospital outcome, were compared. RESULTS: No significant differences were observed in vascular access site, sheath size and type of stent implanted, nor was significantly different the use of glycoprotein IIb/IIIa inhibitors, and the use and dose of intravenous unfractionated heparin. Although not statistically different, the in-hospital occurrence of death (3.7% vs. 1.1%; OR 3.3; 95% confidence intervals [CI] 0.2-56.0), stent thrombosis (3.7% vs. 1.1%; OR 3.3; 95% CI 0.2-56.0) and major bleeding (7.4% vs. 2.2%; OR 3.4; 95% CI 0.4-25.9) was consistently about 3-fold higher in group 1. CONCLUSION: The in-hospital pharmacological and procedural management of OAC patients undergoing PCI-S for STEMI vs. other indications appears not different. Although not significantly different however, the in-hospital occurrence of major bleeding, as well as of death and stent thrombosis, appears substantially higher in patients treated for STEMI, warranting therefore further larger, prospective studies.

Exercise intensity of head-out water-based activities (water fitness).

The aims of this study were: (i) to measure the exercise intensity (EI) of the most common water-based exercises (WE) at different movement frequencies (f1 = 1.8-2.0 Hz; f2 = 2.0-2.2 Hz; f3 = 2.2-2.4 Hz) and at a standardize movement's amplitude; (ii) to measure EI during a combination (MIX) of these WE. Five WE were selected: "running raising the knees high" (S); "jumping moving the legs sideways" (SJ); "jumping moving the legs backward and forward" (FJ); "alternate forward kicks" (FK); "alternate sideways kicks" (SK). Twelve physically active women were asked to perform these WE at the three frequencies, as well as a combination (MIX) of the WE. EI increased significantly (p < 0.01) with increasing frequency; as an average, for all WE: VO2 ranged from 18 to 25 ml kg(-1) min(-1), HR from 102 to 138 bpm, RPE from 9.8 to 14.4 (at f1 and f3, respectively). In terms of % VO2max, EI ranged from 37 to 54% for S, was similar for SJ and FJ (31-43%) and for FK and SK (47-63%) at f1 and f3, respectively. Thus, a given EI can be attained either by changing the type of exercise and/or the frequency of the movement. The combination of exercises did not change (in terms of VO2, HR and RPE) the intensity of each exercise performed separately. These data can be utilized to control, in terms of exercise type and frequency, the intensity of a proposed water-based activity.

Left ventricular stroke work reserve as prognostic marker in severe heart failure.

AIM: We sought to determine whether an invasive approach based on Swan-Ganz catheterization, coupled with a pharmacologic stressor, might help stratify prognosis in patients with severe heart failure and uniformly depressed indices of cardiac function. METHODS: We studied 31 unselected consecutive patients with scalar doses of dobutamine (2.5-10 microg/kg/min) after baseline hemodynamic evaluation. Changes in stroke work index (SWI) from baseline to peak effect (stroke work reserve, SWR) were recorded, and patients classified as responders (SWR above the median) or non-responders to dobutamine (SWR below the median). One-year follow-up data were recorded. RESULTS: All patients completed the dobutamine challenge test without complications. Dobuta-mine increased SWI from 17+/-9 to 26+/-13 mg/ beat/m2 (P<0.0001 vs baseline), with a median increase of 6.4 g/beat/m2. Basal SWI was not related to stroke work reserve. The only predictor of response to dobutamine was a smaller left ventricular end-diastolic volume (135+/-28 vs 205+/-90 mL/m2; P=0.007). After 1 year, only 7 patients were alive, while 10 had successful transplantation. Transplant-free survival was 47% in responders vs 0% in non responders (P=0.007). At multivariate analysis, none of baseline hemodynamic parameters was predictive of survival. Only age and a SWR above the median were significant independent predictors of survival in this model. CONCLUSIONS: This study allows us to draw the following conclusions: 1) 1-year mortality in severe heart failure remains extremely high; 2) baseline hemodynamics dos not predict survival; 3) a positive response to dobutamine identifies a subgroup with significant lower mortality at 1 year; 4) this response is an independent predictor of survival and is more likely to occur in the presence of a less dilated left ventricle.

Laparoscopic nerve-sparing complete excision of deep endometriosis: is it feasible?

BACKGROUND: Little is known about the morbidity associated with laparoscopic complete excision of endometriosis in terms of urinary, digestive and sexual function. METHODS: We performed a prospective non-randomized study in 45 patients with laparoscopic complete excision of all detectable foci of endometriosis with segmental bowel resection using a non nerve-sparing technique (control group-group A n=20) and a nerve-sparing technique (case group-group B n=25). At initial gynaecological evaluation, and at follow-up details on dysmenorrhoea, pelvic pain, dyspareunia and dyschezia were evaluated using an interview-based questionnaire (10-point analogue rating scale: 0=absent, 10=unbearable). RESULTS: The mean (+/-SD) follow-up period was 15.3+/-10 months (range, 8.8-23 months) for group A and 3.5+/-2.1 months (range, 0.3-5.2 months) for group B. In the immediate postoperative course, in group A three women required blood transfusion vs seven women in group B (P=0.003). The median time to resume the voiding function was significantly shorter in group B (12.5 vs 3.0 days; P<0.01). At the time of follow-up a higher proportion of patients in group B were 'very satisfied' than those in group A (87.7% vs 59.0%, P=0.013). CONCLUSIONS: Laparoscopic nerve-sparing complete excision of endometriosis seems to be feasible and offers good results in terms of bladder morbidity reduction with apparently higher satisfaction than classical technique. Larger series with longer follow-up are needed to confirm our results.

Amino-terminal propeptide of type III procollagen is associated with restrictive mitral filling pattern in patients with dilated cardiomyopathy: a possible link between diastolic dysfunction and prognosis.

OBJECTIVE: To analyse the relation between restrictive mitral pattern, amino-terminal propeptide of type III procollagen (PIIINP), and prognosis in patients with dilated cardiomyopathy. DESIGN: Prospective cohort study of 106 patients with dilated cardiomyopathy. SETTING: Tertiary care centre. MAIN OUTCOME MEASURES: PIIINP concentration, echocardiographic variables, oxygen consumption, hospitalisation for heart failure, and cardiac mortality were evaluated in patients grouped by the presence of non-restrictive (group 1), reversible (group 2), and irreversible restrictive mitral pattern (group 3). RESULTS: Groups differed regarding left ventricular ejection fraction (group 1, mean (SD) 36 (6)%, group 2, 29 (8)%, group 3, 25 (6)%; p = 0.0001), left atrial ejection fraction (group 1, 0.47 (0.1)%, group 2, 0.43 (0.2)%, group 3, 0.26 (0.1)%; p < 0.0001), and PIIINP (p = 0.001). Multivariate analysis showed that PIIINP was related to mitral pattern (odds ratio 0.8, 95% confidence interval 0.23 to 1.4, p = 0.006) independently of left atrial and ventricular ejection fractions. After 21 months, survival was 88% and 34% (p = 0.0001) in patients with non-restrictive and irreversible restrictive mitral patterns, respectively. CONCLUSION: In patients with dilated cardiomyopathy, restrictive mitral pattern is associated with higher PIIINP and worse prognosis.

Relationship between exhaled air temperature and exhaled nitric oxide in childhood asthma.

Airway inflammation is a characteristic of asthma. Exhaled nitric oxide (eNO) has been demonstrated to be related to actual levels of airway inflammation in asthmatic patients. The purpose of this study was to investigate whether the temperature of exhaled air is related to eNO levels. Temperature of exhaled air and eNO were measured in 52 asthmatic children with a cross-sectional design. A significant relationship was demonstrated between eNO and temperature of peak and plateau exhaled air temperature. The relationship between both the peak and the plateau values and eNO was more evident when it was corrected for environmental temperature. These results suggest a relationship between exhaled nitric oxide and the temperature of exhaled air in asthmatic patients not treated with systemic steroids.

Prevalence of urinary incontinence in women with cystic fibrosis.

OBJECTIVE: To determine the prevalence of urinary incontinence (UI) in female patients (aged > or = 15 years) attending a cystic fibrosis (CF) centre, in whom stress UI could be common, as chronic coughing and sputum production are frequent symptoms associated with progressive lung disease in these patients. PATIENTS AND METHODS: An anonymous questionnaire was completed by 176 women with CF (mean age 24.6 years, SD 5.8) during routine assessments as outpatients. RESULTS: In all, 72 patients (41%) were classified as never incontinent; occasional UI was reported in 61 women (35%). Regular UI, occurring twice or more a month for at least two consecutive months in the last year, was reported in 43 patients (24%). Regular UI was associated with increasing age and a lower mean (SD) forced expiratory volume/s (of that predicted) than in women with no urinary symptoms, at 26.9 (6.5) years and 53.5 (23.5)%, and 23.1 (5.4) years and 65.5 (23.2)%, respectively (P < 0.01 and P < 0.05, respectively). All incontinent women recorded stress UI; coughing, laughing and physical activity were associated with UI in 92%, 33% and 21% of the patients, respectively. CONCLUSION: Stress UI is a common symptom in women with CF. As urine loss can be under-reported to the healthcare providers, women should be asked about incontinence as part of their routine follow-up. Pelvic floor muscle exercises are effective in treating stress UI and should be considered for those with CF and regular UI.

Reduced right ventricular ejection fraction as a marker for idiopathic dilated cardiomyopathy compared with ischemic left ventricular dysfunction.

BACKGROUND: Evidence for the role of right ventricular (RV) function is emerging in patients with heart failure of different etiologies. Studies conducted in dilated cardiomyopathy (IDC) showed a high prevalence of RV dysfunction unrelated to the severity of pulmonary hypertension. The aim of the study was to investigate the role of RV dysfunction in ischemic versus nonischemic patients. METHODS: A series of 153 patients with left ventricular (LV) dysfunction (defined as a LV ejection fraction <45%) of either ischemic (n = 61, coronary artery disease [CAD] group) or nonischemic (n = 92, IDC group) origin were studied invasively. Besides routine catheterization data, RV volumes and ejection fractions were obtained angiographically. Reference data were collected in a control group of healthy subjects. RV dysfunction was defined as a RV ejection fraction <35% and ventricular concordance as a <10% difference between RV and LV ejection fraction. The LV/RV end-diastolic volume ratio was calculated to assess the relative dilatation of the ventricular chambers. Hemodynamic and angiographic data were compared in the 2 groups by univariate and multivariate logistic regression analysis. RESULTS: Patients with IDC and CAD had comparable LV ejection fractions (29% +/- 3% vs 31% +/- 8%, P not significant) and mean pulmonary pressures (27 +/- 12 mm Hg vs 26 +/- 11 mm Hg, P not significant); the LV/RV end-diastolic volume ratio was identical in the 2 groups (1.26 +/- 0.4 vs 1.24 +/- 0.4, P not significant). RV ejection fraction was significantly lower in IDC compared with CAD (33% +/- 10 % vs 46% +/- 11%, P <.0001), with a prevalence of RV dysfunction in the IDC group of 65% compared with 16% in the CAD group (P <.0001); similarly, the prevalence of ejection fraction concordance was 74% versus 33%, respectively (P <.0001). At multivariate analysis, a low RV ejection fraction was a powerful independent predictor of IDC compared with CAD (odds ratio 0.91, 95% confidence interval 0.87-0.94, P <.0001). RV dysfunction had a positive predictive value of 75% and a negative predictive value of 78% for the diagnosis of IDC; for ventricular concordance, these values were 81% and 69%, respectively. The correlation between mean pulmonary artery pressure and RV ejection fraction was weaker in the IDC group compared with the CAD group (R(2) = 0.032, P =.047 and R(2) = 0.172,P <.0001, respectively). CONCLUSION: In the presence of LV dysfunction, a reduced RV ejection fraction is a powerful marker for IDC compared with CAD, independent of age, pulmonary hypertension, LV function, and ventricular dimensions. These findings support the concept that IDC is frequently characterized by a biventricular involvement and that the presence of RV dysfunction represents a distinguishing feature of this disease.

Failure of aldosterone suppression despite angiotensin-converting enzyme (ACE) inhibitor administration in chronic heart failure is associated with ACE DD genotype.

OBJECTIVES: The objective of this study was to assess whether the angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism influences the adequacy of the neurohormonal response to ACE inhibitors in patients with chronic heart failure (CHF). BACKGROUND: The renin-angiotensin-aldosterone system (RAAS) plays an important role in the pathophysiology of CHF, and aldosterone levels closely relate to outcome in patients with CHF. Angiotensin-converting enzyme inhibitors suppress the RAAS, but a significant proportion of patients exhibit elevated serum levels of aldosterone despite long-term administration of apparently adequate doses of these agents. METHODS: We prospectively studied 132 patients with CHF (ejection fraction <45%) receiving long-term therapy with ACE inhibitors for over six months. Patients taking aldosterone antagonists were excluded from the study. "Aldosterone escape" was defined as being present when plasma aldosterone levels were above the normal range in our laboratory (>42 nmol/L). Patients were then divided into two subgroups according to the presence (group 1) or absence (group 2) of aldosterone escape. Genotype analysis for the ACE I/D polymorphism was performed by polymerase chain reaction. RESULTS: The prevalence of aldosterone escape in our patients was 10% (13/132). The two groups of patients did not differ regarding the dose of ACE inhibitor, diuretics and their renal function. There was a statistically significant different distribution of genotypes between the two groups, with a higher proportion of DD genotype in group 1 compared with group 2 (62% vs. 24%, p = 0.005). CONCLUSIONS: Patients with CHF with aldosterone escape have a higher prevalence of DD genotype compared with patients with aldosterone within the normal limits. Angiotensin-converting enzyme gene polymorphism contributes to the modulation and adequacy of the neurohormonal response to long-term ACE-inhibitor administration in CHF.

Skeletal muscle mass independently predicts peak oxygen consumption and ventilatory response during exercise in noncachectic patients with chronic heart failure.

OBJECTIVES: We sought to assess whether skeletal muscle mass might be a predictor of peak oxygen consumption (Vo2) and relation of the ventilation to carbon dioxide production (VE/VCo2) slope in patients with chronic heart failure (CHF) independent of clinical conditions, neurohormonal activation and resting hemodynamics. BACKGROUND: A variety of abnormalities characterize skeletal muscle and contribute to exercise intolerance in patients with CHF. Skeletal muscle mass is a determinant of peak Vo2 both in healthy patients and in patients with CHF, but there are no reports on the independent predictive value of this parameter, which can be measured with great accuracy by whole-body dual energy X-ray absorptiometry (DEXA). The influence of skeletal muscle mass on VE/VCo2 slope is not known either. METHODS: We prospectively evaluated 120 consecutive noncachectic patients with CHF. Every patient underwent a cardiopulmonary exercise test, an echo-Doppler examination and an evaluation of neurohormonal activation and body composition as assessed by DEXA. RESULTS: At the univariate analysis, New York Heart Association (NYHA) class (p < 0.0001), age (p < 0.0001), male gender (p < 0.0001) and plasma renin (p < 0.0001) significantly related with peak Vo2. There was a significant correlation between lean mass and absolute peak Vo2 (r = 0.70, p < 0.0001) and VE/VCo2 slope (r = -0.27; p < 0.01). At the multivariate analysis, lean mass predicted peak Vo2 and VE/VCo2 slope independently of NYHA functional class, age, gender, neurohormonal activation and resting hemodynamics. CONCLUSIONS: Skeletal muscle mass is an independent predictor of peak Vo2 and VE/VCo2 slope in stable noncachectic patients with CHF. Future studies will determine whether an increase in skeletal muscle mass in the individual patient might result in an improvement in parameters of exercise capacity.

Low domestic exposure to house dust mite allergens (Der p 1) is associated with a reduced non-specific bronchial hyper-responsiveness in mite-sensitized asthmatic subjects under optimal drug treatment.

BACKGROUND: Airway inflammation in asthma causes symptoms, airflow limitation and bronchial hyper-responsiveness. The strategy of asthma management is to reduce airway inflammation by drug treatment and avoidance of triggers, including allergens. OBJECTIVE: We determined the effect of exposure to house dust mite (HDM) allergens on bronchial responsiveness in asthmatics sensitive to mites while under optimal drug treatment. METHODS: We studied 71 mild to moderate HDM-sensitive asthmatics. Drug treatment sufficient to keep asthma under control was administered to each patient for 1 year. Subjects were divided into two groups, according to the amount of Der p 1 in their bedrooms measured after standard HDM reduction measures: low Der p 1 exposure (0.64 +/- 0.5 microg/g dust) (Group 1, n = 34) and high Der p 1 exposure (12.5 +/- 11.4 microg/g) (Group 2, n = 37). Bronchial responsiveness to methacholine (PD20FEV1) was determined at the beginning and end of the study. RESULTS: In Group 1, PD20FEV1 increased 2.15-fold at the end of the study from 57 to 123 microg (P < 0.05), whereas in Group 2 no significant changes were observed. The subjects in Group 2 tended to increase the use of inhaled steroids and bronchodilators in the autumn months compared with subjects in Group 1, but the difference was not significant. CONCLUSION: This long-term study shows that exposure to lower levels of mite allergens in the bedroom is associated with a decrease of bronchial hyper-responsiveness in sensitized asthmatic subjects under optimal drug treatment.

Frequency, prognosis and predictors of improvement of systolic left ventricular function in patients with 'classical' clinical diagnosis of idiopathic dilated cardiomyopathy.

In patients with dilated cardiomyopathy (DCM) of different aetiologies, a variable frequency of improvement in the left ventricular (LV) systolic function has been reported, while in patients with a 'classic' idiopathic DCM, the frequency of improvement is still under debate, and clinical and haemodynamic predictors of recovery of the LV function are needed. The aim of the present study was to determine the frequency of improvement in the LV systolic function in idiopathic DCM and to identify predictors of reversibility of the impaired LV contractility. A sample of 98 consecutive patients with idiopathic DCM was retrospectively evaluated. Echocardiographic and Doppler measurements were directly taken from the routine echo-report. LV systolic function was assessed semiquantitatively using a score index (SFSI). According to the improvement in the LV systolic function, the patients were divided into group 1 patients with improvement, and group 2 patients without improvement. During a follow-up of at least 12 months, 19 patients (19%) showed an improvement, with a significant increase in the mean SFSI; all these group 1 patients survived without heart transplant; in group 2, 18 patients (23%) died and 3 (4%) received a heart transplant. Patients in group 1 had a significantly shorter duration of symptoms (P=0.0045), a younger age (P=0.006), a shorter DtE (P=0.04), a lower SFSI (P<0.01), a worse NYHA class (P<0.001) and more frequently had a history of hypertension (P<0.0001). The same variables were significant predictors of improvement at the univariate analysis. At the multivariate logistic regression analysis, a shorter duration of symptoms (P=0.02), a history of hypertension (P=0.003), and a worse NYHA class (P=0.01) were independent predictors of improvement. A relatively large percentage of patients with an idiopathic DCM will have a marked improvement in the LV systolic function. This is more likely to happen in the presence of a short duration of symptoms and a history of hypertension. After an improvement, the prognosis is excellent.

Preoperative autologous blood donation by elderly patients undergoing orthopaedic surgery.

BACKGROUND AND OBJECTIVES: To assess the feasibility of a programme of predeposit in elderly patients undergoing elective orthopaedic surgery. PATIENTS AND METHODS: We retrospectively studied 789 elderly patient candidates (> 65 years of age) for orthopaedic surgery (total hip and knee replacement and spinal surgery), who were undergoing a programme of preoperative autologous blood donation (PABD) in our city hospital between January 1990 and December 1998. RESULTS: Six hundred and eighty-eight patients (87.2%) were transfused with autologous blood; 128 (16.2%) also received allogeneic blood. Hip arthroplasty revision was characterized by the greatest blood consumption. The predeposit programme was discontinued in 96 patients (12.2%) because of the following complications: the onset of anaemia (11.0%); vasovagal reactions (0.5%); lack of venous access (0.4%); or cardiac complications (0.2%). No episodes of reaction to autologous transfusion were recorded. CONCLUSIONS: Our study confirms the feasibility of PABD in elderly patients undergoing elective orthopaedic surgery.

Additional value of pulmonary vein parameters in defining pseudonormalization of mitral inflow pattern.

BACKGROUND: An echocardiographic assessment of left ventricular (LV) diastolic dysfunction is still challenging when identifying a pseudonormal mitral pattern (PSE) in an unselected population. The present study analyzed and compared the accuracy of various parameters in correctly identifying a PSE pattern in patients with a broad range of ejection fraction (EF) and degree of mitral regurgitation. METHODS: Eighty-two patients with E/A > or = 1 and an invasive determination of left ventricular end-diastolic pressure (LVEDP) were enrolled in the study. Mitral E wave (E(max)) and A (A(max)) velocities, E (DTe) and A (DTa) deceleration times, pulmonary vein systolic and diastolic velocities, and time velocity integrals were measured. The different duration between mitral and pulmonary vein A wave (A'-A) also was calculated. E(max) and E/A during Valsalva maneuver were measured and expressed as percentage compared with baseline. LV end-diastolic (LVD), end-systolic (LVS), and EF were measured from the apical four-chambers view (area-length method). Left atrial end-systolic (LA(max)) and end-diastolic (LA(min)) were measured from the apical four- and two-chambers views (area-length method). Left atrial filling volume (LA(fill)) was the difference between LA(max) and LA(min). Mitral regurgitant volume was estimated by the following equation: MR(vol) = 6.18 + (1.01 * LA(fill)) - (0.783 * PVs %). RESULTS: Thirty-two patients (age: 55 +/- 21 years; 75% male) had LVEDP < or = 18 mmHg and were classified as normal mitral pattern (Group 1). Fifty patients (age: 57 +/- 22 years; 76% male) had LVEDP > 18 mmHg, and were classified accordingly as having PSE (Group 2). At logistic univariate analysis, DTa (0.005), LV EF (0.01), A'-A (< 0.0001) and % E/A (0.03) were the more powerful predictors of PSE. A'-A had the highest global accuracy in identifying PSE in patients with reduced (90%) and normal (88%) LV EF. CONCLUSION: A'-A has the highest accuracy in identifying PSE in an unselected population. This parameters should be implemented in routine echocardiography since it allows additional information about LV diastolic function assessment.

Diffusion model of pain language and quality of life in orofacial pain patients.

AIMS: To address the following questions: (1) Which words are preferred by different groups of orofacial pain patients to describe their pain experience? (2) Is it possible, based on such descriptions, to obtain a clinical differential diagnosis in these patients? (3) Is there any relationship between the verbal description of pain and self-rated quality of life (QOL)? (4) Can a pattern of modulation of pain language by affective variables (diffusion model) be recognized in orofacial pain patients, as it has in other chronic pain patients? and (5) If so, what might be the clinical usefulness of assessing pain language in these patients? METHODS: A total of 332 consecutive orofacial pain patients filled out an Italian Pain Questionnaire (the Italian analog of the McGill Pain Questionnaire) and were then divided into 6 diagnostic subgroups (sample 1) based on history and clinical findings. In a double-blind setting, the distribution of pain descriptors and indexes was statistically evaluated. From sample 1, a randomly selected sample of 121 patients (sample 2) also filled out a QOL categorical scale. The results of both tests in this sample were compared statistically. RESULTS: Some significant differences among diagnostic subgroups were found for choice of descriptors and for pain intensity. When a patient's pain description was compared to the corresponding self-evaluation of QOL, a self-perceived worsening of QOL revealed a good correlation with an increase in the number of words chosen, pain intensity, and affective and sensory pain descriptors. A similar significant association was found between self-assessed anxiety and/or depression and the same items. CONCLUSION: Although trends in patients' choice of descriptors were evident, differential diagnosis based on only a pain questionnaire was not possible in the different groups of orofacial pain patients examined in this study. The present study suggests the presence of a phenomenon of diffusion in the language of those patients who were experiencing a worsening of their QOL as a result of pain and consequent psychologic distress. This observation can be of clinical usefulness by enhancing the sensitivity of the clinician to the suffering and affective distress experienced by the patient, and it also can be helpful in refining the therapeutic approach for each individual patient.

Neonatal screening for cystic fibrosis: long-term clinical balance.

BACKGROUND/AIMS: Very few studies have been performed on the long-term clinical advantages of neonatal screening programs for cystic fibrosis (CF) and these have been inconclusive. This is a preliminary report of two observational cohort studies on this subject. METHODS: In the first study, CF patients born between 1973 and 1981 in northeastern Italy were split into 4 groups according to the modality of diagnosis: screening by meconium test (58 patients); meconium ileus (45 patients); symptoms and pancreatic insufficiency (PI; 75 patients), or symptoms and pancreatic sufficiency (PS; 19 patients). The patients were followed for up to 26 years by three CF centers sharing common treatment protocols. In the second study, two cohorts of CF patients born between 1983 and 1992 were compared. Patients from one cohort (126 patients) were born in the Veneto region, where a neonatal screening program had been established based on immunoreactive trypsinogen. Patients from the other cohort (152 patients) were born in Sicily, where an intensive program of early diagnosis by symptoms was implemented. The cohorts were comparable for CF incidence, CFTR genotypes, gender proportion and common treatment protocols. Statistical analyses were performed by Kaplan-Meier survival curves, a Cox proportional hazard model for survival and cross-sectional comparisons by 2-year periods for weight z score, height z score and body mass index. RESULTS: In the first study, the patients detected by newborn screening (PI) showed better survival and nutritional status compared to patients diagnosed through meconium ileus or symptom presentation with PI. PS patients diagnosed by symptoms showed the best outcome, but most of them had a mild genotype. In the second study, the Veneto cohort showed better outcome with regard to survival and nutritional status over 16 years of follow-up. CONCLUSIONS: Observational cohort studies cannot give definitive evidence of the clinical benefit of neonatal CF screening; however, data have been accumulated which strongly suggest a better clinical outcome for CF patients born in an area where a screening program is performed.

Reliability of sweat-testing by the Macroduct collection method combined with conductivity analysis in comparison with the classic Gibson and Cooke technique.

UNLABELLED: This study was to ascertain the reliability of sweat-testing by the Macroduct collection method combined with conductivity analysis (MCS) compared with the Gibson and Cooke technique (GCT). Sweat stimulation by pilocarpine iontophoresis was identical for both procedures, sweat being collected for 30 min on a filter paper on one forearm and in the coil of the Macroduct collector on the other. Chloride, sodium and potassium concentrations were chemically analysed both on paper-eluted and tube-collected sweat; the latter was also analysed using a conductivity analyser. Chemical analyses were compared with conductivity analyses. This prospective study was carried out on 318 subjects with MCS (118 CFs, 200 controls) and on 305 of them with the GCT (113 CFs, 192 controls). The pilocarpine iontophoresis produced adequate sweat in 96.4% of collections with GCT and in 90.9% with the MCS. Sensitivity and specificity of the Macroduct/conductivity system were comparable to the GCT. No patient detected by the GCT technique was considered negative by conductivity, but one GCT positive was "borderline" with the MCS. Six non-CF subjects identified as negative by the GCT (3.3%) were in the borderline range with the MCS. CONCLUSION: Sweat-testing by the MCS has acceptable sensitivity and specificity when performed by trained CF sweat-testing technicians. Additional studies will be required to find out if these results can be confirmed in small clinics and hospitals where testing is done infrequently. Wherever the MCS is used all positive or borderline results should be confirmed by the GCT at a reference Cystic Fibrosis Center.

Cardiac troponin I as diagnostic and prognostic marker in severe heart failure.

BACKGROUND: Cardiac cell death has been shown to occur in heart failure and has been implicated as one of the mechanisms responsible for progression of the disease. Cardiac Troponin I (cTnI) represents a highly sensitive marker for myocardial cell death. Based on previous studies reporting that cTnI may be detected in patients with heart failure, we evaluated the clinical correlates and prognostic implications of detectable cTnI in a consecutive series of patients with severe heart failure. METHODS: Thirty-four patients were examined. Upon admission, we measured serum levels of cTnI by conventional immunoenzymatic assay (Stratus Dade II). According to the results of this assay, patients were divided into 2 groups, based on the presence (cTnI+) or absence (cTnI-) of detectable cTnI. These 2 groups were compared by non-parametric analysis for their clinical characteristics, instrumental findings, and short-term outcome. RESULTS: The cTnI+ group included 10 patients (29%) with a mean serum cTnI of 0.7 +/- 0.3 ng/ml. Compared with the cTnI- group, these patients had significantly lower left ventricular ejection fractions (20% +/- 5% vs 26% +/- 7%, p = 0.023) and a trend for higher systolic pulmonary artery pressure (59 +/- 17 mm Hg vs 49 +/- 13 mm Hg, p = 0.08). In cTnI+ patients, the correlation between cTnI levels upon admission and ejection fraction was r = -0.530 (p = 0.11). We found ischemic etiology was equally present in the 2 groups, whereas we never found histologic signs of acute myocarditis. Other clinical characteristics (functional class, daily diuretic dose, need for intravenous inotropes) were not statistically different in the 2 groups. In cTnI+ patients who improved after admission, cTnI became undetectable after a few days; in patients with refractory heart failure who were hospitalized until death, cTnI persisted in detectable levels throughout the observation period. Using the Cox proportional hazard model, a positive cTnI was the most powerful predictor of mortality at 3 months (p = 0.013; hazard ratio 6.86; 95% confidence interval 1.32 to 35.4). CONCLUSIONS: These observations suggest that cTnI is detected in the blood of 25% to 33% of patients with severe heart failure; its presence may help to identify a high-risk sub-group who faces very poor short-term prognosis.