Decreased respiratory performance of children and adolescents with myelomeningocele who use a wheelchair - preliminary data.

Myelomeningocele (MMC) is a neural tube defect that often causes spinal cord injury at the thoracolumbar region, as well as sensory and motor paralysis in the lower limbs. This leads to continuous use of a wheelchair and, consequently, a sedentary lifestyle, predisposition to muscle weakness, cardiovascular and respiratory disorders, obesity, and structural alterations in the spine. We assessed the respiratory function and shoulder strength of MMC participants who were wheelchair-users and had no respiratory complaints and compared them to healthy children and adolescents. MMC (n=10) and healthy (n=25) participants of both genders with a mean age of 12.45 years (SD=2.1) were assessed for weight, height, respiratory performance, and isometric peak for shoulder flexors, extensors, abductors, and adductors, using an isokinetic dynamometer. Medullary lesion, functional levels, and abnormal curvatures of the spine were assessed for MMC participants. The level of spinal cord injury for the majority of the MMC participants was high lumbar and they had scoliosis. MMC showed lower values for forced vital capacity, forced expiratory volume at the first second, forced expiratory flow (25-75%), maximal voluntary ventilation, and isometric peak for shoulder flexors and adductors compared to healthy participants. This indicated a decreased vital capacity, respiratory muscle endurance, and shoulder muscle strength.

Decreased respiratory performance of children and adolescents with myelomeningocele who use a wheelchair - preliminary data

Myelomeningocele (MMC) is a neural tube defect that often causes spinal cord injury at the thoracolumbar region, as well as sensory and motor paralysis in the lower limbs. This leads to continuous use of a wheelchair and, consequently, a sedentary lifestyle, predisposition to muscle weakness, cardiovascular and respiratory disorders, obesity, and structural alterations in the spine. We assessed the respiratory function and shoulder strength of MMC participants who were wheelchair-users and had no respiratory complaints and compared them to healthy children and adolescents. MMC (n=10) and healthy (n=25) participants of both genders with a mean age of 12.45 years (SD=2.1) were assessed for weight, height, respiratory performance, and isometric peak for shoulder flexors, extensors, abductors, and adductors, using an isokinetic dynamometer. Medullary lesion, functional levels, and abnormal curvatures of the spine were assessed for MMC participants. The level of spinal cord injury for the majority of the MMC participants was high lumbar and they had scoliosis. MMC showed lower values for forced vital capacity, forced expiratory volume at the first second, forced expiratory flow (25-75%), maximal voluntary ventilation, and isometric peak for shoulder flexors and adductors compared to healthy participants. This indicated a decreased vital capacity, respiratory muscle endurance, and shoulder muscle strength.

Case Report: Aortic Valve Replacement After JARVIK 2000 Left Ventricular Assist Device Implantation in Long-Time Survivor With Severe Aortic Valve Regurgitation.

BACKGROUND: There are limited clinical records in the literature regarding aortic valve replacement in left ventricular assist device (L-VAD) patients. Previously we had two cases of severe aortic valve regurgitation in patients with L-VAD support treated with Corvalve prosthesis insertion and Amplatzer closure procedure. Both patients died a few days after the procedure from complications not related to the procedure itself. PATIENT HISTORY: The patient was a male with previous coronary artery bypass graft surgery in 2001 that was complicated with postischemic dilated cardiomyopathy with severe heart failure (ejection fraction [EF], 20%). Cardiac resynchronization therapy was biventricular-pacemaker and cardiac defibrillator implantation in 2009 for recurrent ventricular arrhythmia. L-VAD implantation (Jarvik 2000) with graft apposition in descending thoracic aorta through left thoracotomy access and retro-auricolar cable was performed in October 2013. In 2015 the patient underwent surgical aortic valve replacement with bioprothesis due to progressive worsening of the aortic valve regurgitation. The Jarvik 2000 outflow was occluded with vascular ball occluder inserted via right axillary artery under fluoroscopy before CEC installation. The recovery was without major complications. DISCUSSION: Long-time survivors with Jarvik 2000 are increasing in number and such late complication is expected to become a main future issue. Our previous experience with the interventional approach was delusive. Due to the fatal consequences in similar patients with nonsurgical approaches, we opted for surgical aortic valve replacement. At the moment, the international literature does not describe safe approaches regarding aortic valve replacement in patients with Jarvik 2000 L-VAD. This case shows that surgical valve replacement could be managed with success according to the described specific technique.

Longitudinal Strain Stress-Echo Evaluation of Aged Marginal Donor Hearts: Feasibility in the Adonhers Project.

BACKGROUND: The Adonhers (aged donor heart rescue by stress-echo protocol) Project was created to resolve the current shortage of donor hearts. One of the great limits of stress echo is the operator dependency. Speckle-tracking echocardiography (STE), offering a quantitative objective analysis of myocardial deformation, may help to overcome this limit. This study aimed to verify feasibility of a stress-strain echo analysis in selection of aged donor hearts for heart transplant. METHODS: From February 2014 to October 2015, 22 marginal candidate donors (16 men) ages 58 ± 4 years were initially enrolled. After legal declaration of brain death, all marginal donors underwent bedside echocardiography, with baseline and (when resting echocardiography was normal) dipyridamole (0.84 mg/kg in 6 minutes) stress echo. In all patients, left ventricular (LV) longitudinal myocardial deformation was obtained by STE in the 4-, 2-, and 3-chamber views, obtaining the average global longitudinal strain (GLS). GLS was assessed at baseline and at the peak of stress echo. RESULTS: Baseline echocardiography showed wall motion abnormalities in 9 patients (excluded from donation). Stress echocardiography was performed in the remaining 13 patients. Results were normal in 8, who were uneventfully transplanted in marginal recipients. Stress results were abnormal in 5 (excluded from donation). STE was obtained in all cases (100% feasibility) and ΔGLS was significantly different between normal and pathological stress-echo (+13.2 ± 5.2 versus -6.1% ± 3.1%, P = .0001, respectively). CONCLUSIONS: STE showed an excellent feasibility in analysis of LV myocardial longitudinal strain at baseline and at the peak of stress echo of marginal heart donors. Further experience is needed to confirm STE as a valuable additional mean to better interpret stress echo in marginal donors.

A new mathematical model based on clinical and laboratory variables for the diagnosis of Sjögren's syndrome.

Sjögren's syndrome (SS) is a systemic autoimmune disease that mainly affects exocrine glands. A diagnosis of SS in its early stages has a potential clinical relevance, but it is difficult and cannot be made solely on clinical grounds. Several sets of diagnostic criteria have been proposed, but none has met with a general consensus. Minor salivary gland has been judged to be the "gold standard" for the diagnosis of SS. However, it is a painful procedure and has a small but significant proportion of both false positive and false negative results. The aim of our study was to develop a simple mathematical score that uses clinical and laboratory variables for diagnosing SS, thereby reducing the need of minor salivary gland. The following variables were included in the model: ANA, SS-A/SS-B, Schirmer's Test/BUT, C3/C4, serum gammaglobulin levels. One hundred consecutive individuals reporting clinical syndromes consistent with a sicca syndrome were included in the study. The application of our multifactorial mathematical model has shown a high predictive value for SS vs controls or vs patients with other autoimmune disorders (Sensitivity 93%, Specificity 100%), with an estimated minor salivary gland reduction of 77%. We conclude that our mathematical model can be considered a useful non-invasive approach for diagnosing Sjogren's Syndrome and recommend its validation on a larger scale.

Perioperative use of tirofiban hydrochloride (Aggrastat) does not increase surgical bleeding after emergency or urgent coronary artery bypass grafting.

BACKGROUND: The platelet glycoprotein IIb/IIIa inhibitor tirofiban hydrochloride improves outcome in patients with acute coronary syndrome. Nevertheless, a considerable number of patients require emergency or urgent coronary artery bypass grafting and may be at increased risk of postoperative bleeding after treatment with this molecule. The aim of this study is to evaluate the incidence of bleeding complications among patients undergoing bypass grafting after treatment with tirofiban. METHODS: We investigated the influence of the molecule on postoperative bleeding after cardiac surgery, comparing 2 groups of patients undergoing emergency or urgent coronary artery bypass grafting: group A (n = 20) received tirofiban, and group B (n = 68) received conventional therapy with intravenous heparin up until the operation. A total of 88 patients underwent coronary artery bypass surgery within 2 hours of ceasing the hemodynamic study. Clinical outcome, chest tube outputs, bleeding complications, transfusion requirements, platelet and hemoglobin counts, and clinical complications were examined. RESULTS: Bleeding differences were noted between the 2 groups at 8, 16, and 24 hours postoperatively. The incidence of blood, platelet, and fresh frozen plasma transfusions was higher in the control group. Postoperative thrombocytopenia was preserved in group A (199.5 +/- 70.4 vs 150.6 +/- 33.4 10(3)/mL, P <.01). No significant differences were noted between the 2 groups in the incidence of perioperative myocardial infarction, but significant differences were noted in enzyme levels, length of stay in the intensive care unit, and length of stay in the hospital. No deaths were observed. Hospital morbidity was increased in group B because of factors that were not apparently linked with tirofiban infusion. CONCLUSIONS: Patients may safely undergo coronary artery bypass surgery after treatment with tirofiban hydrochloride. This molecule, administered in the immediate preoperative period, has no adverse clinical effects and does not seem to negatively influence the incidence of perioperative myocardial infarction. Although extracorporeal circulation can modify platelet numbers and function, our ongoing data could show significant reduction in the loss of platelets induced by cardiopulmonary bypass, minor postoperative bleeding, and a minor transfusion requirement in general.

The properties of the pacemaker current I(F)in human ventricular myocytes are modulated by cardiac disease.

The pacemaker current I(f)is present in ventricular myocytes from the human failing heart where it may contribute to arrhythmogenesis. The role of cardiac disease in the modulation of I(f)expression is still uncertain. We studied the functional expression and properties of I(f)in human ventricular myocytes isolated from control donor hearts or from explanted failing hearts of patients with ischemic and dilated cardiomyopathy. In patch-clamped cells, I(f)was elicited by hyperpolarization. Membrane capacitance (C(m)) was significantly higher in dilated cardiomyopathy than in control or ischemic cardiomyopathy. I(f)was present in all ischemic and dilated cardiomyopathy tested cells and in 76% of control cells. In ischemic and dilated cardiomyopathy, I(f)amplitude measured at -120 mV was significantly greater than in control. However, I(f)density (i.e. current normalized to C(m)) was significantly higher in ischemic cardiomyopathy (2.0+/-0.2 pA/pF) than in dilated cardiomyopathy (1.2+/-0.1 pA/pF) or control (1.0+/-0.1 pA/pF). In diseased hearts, the activation curve was significantly shifted to more positive values compared to control. The slope of the fully-activated I-V relations was greater in ischemic cardiomyopathy than in dilated cardiomyopathy or control (P<0.05) while the intercept with the x -axis (V(rev)) was similar. In conclusion, I(f)is overexpressed in human ventricular myocytes from failing hearts; its functional expression seems related to the etiology of the disease, being higher in ischemic than in dilated cardiomyopathy, and not to the degree of cell hypertrophy.

Latrogenic injury to the longthoracic nerve: an underestimated cause of morbidity after cardiac surgery.

After heart surgery, complications affecting the brachial plexus have been reported in 2% to 38% of cases. The long thoracic nerve is vulnerable to damage at various levels, due to its long and superficial course. This nerve supplies the serratus anterior muscle, which has an important role in the abduction and elevation of the superior limb; paralysis of the serratus anterior causes "winged scapula," a condition in which the arm cannot be lifted higher than 90 degrees from the side. Unfortunately the long thoracic nerve can be damaged by a wide variety of traumatic and nontraumatic occurrences, ranging from viral or nonviral disease to improper surgical technique, to the position of the patient during transfer to a hospital bed. Our patient, a 62-year-old man with triple-vessel disease, underwent myocardial revascularization in which right and left internal thoracic arteries and the left radial artery were grafted to the right coronary, descending anterior, and obtuse marginal arteries, respectively. Despite strong recovery and an apparently good postoperative course, the patient sued for damages due to subsequent winging of the left scapula. In this instance, the legal case has less to do with the cause of the lesion (which remains unclear) than with failure to adequately inform the patient of possible complications at the expense of the nervous system. The lesson is that each patient must receive detailed written and oral explanation of the potential benefits and all conceivable risks of a procedure.

Electrophysiologic effects of lercanidipine on repolarizing potassium currents.

Blockade of cardiac repolarizing potassium channels by drugs may result in QT-interval prolongation, eventually degenerating into "torsades de pointes," a life-threatening arrhythmia. Lercanidipine (LER) is a recently introduced lipophilic calcium antagonist with no cardiodepressant activity and long-lasting antihypertensive action. Its chemical structure is characterized by the presence of a diphenylpropylaminoalkyl group, which is present in some of the drugs that have been reported to cause QT-interval prolongation. Our previous data demonstrated that LER blocks L-type calcium channels without affecting sodium current; however, no data are available concerning its effects on cardiac potassium channels. Transient outward (I(to)), delayed rectifier (I(K)), background currents, and action potential (AP) profile were measured from patch-clamped ventricular myocytes isolated from rat, guinea pig, or human hearts using enzymatic dissociation procedures. LER did not affect I(K) (and I(Kr)) density and activation curve in guinea pig myocytes; the reversal potential of the background current (I(K1)) and its slope were not changed by the drug. Maximal diastolic potential (MDP) and duration of the AP measured at -60 mV (APD(-60)) were not significantly changed. I(to) density and activation curves measured in rat myocytes were similar in the absence and presence of 1 or 10 microM LER. Finally, the effect of LER was tested in human ventricular myocytes: superfusion with 1 microM LER did not affect MDP and APD(-60). I(to) density and the midpoint of activation and inactivation curves were similar in the absence and presence of LER. In conclusion, our data demonstrate that LER does not affect repolarizing potassium currents and action potential profile recorded from guinea pig, rat, and human ventricular myocytes. It is unlikely that LER could cause QT prolongation in vivo.

[Reconstruction of the aortic root: midterm results]. / Ricostruzione della radice aortica: risultati a medio termine.

BACKGROUND: The aim of this study was to assess mid-term results of aortic reconstruction. METHODS: According to Dr. David's technique, 20 patients (17 males, 3 females, median age 63 years) were examined, who underwent surgery between September 1996 and August 1999. Indications for operation were aortic insufficiency with ascending aorta aneurysm in 19 patients, and acute Stanford type A dissection in 1 patient. In every patient the native valve was preserved and suspended inside a tubular prosthesis on which the coronaries were reimplanted. RESULTS: No patient died in hospital. Follow-up (mean 13 months) was complete for all patients. One patient died of extracardiac causes. Seventeen of 19 survivors are in NYHA functional class I, the remaining 2 in class II. Nineteen patients underwent echocardiography which showed moderate aortic regurgitation in 1, mild in 6 and absent or trivial in 12. There were no instances of thromboembolism. CONCLUSIONS: These results are encouraging and have brought us to consider aortic reconstruction a valid alternative to traditional aortic root replacement. Dr. David's technique is reproducible and reliable in the long term.

Predictor of the rate of CD4 lymphocyte loss in HIV-1-seropositive asymptomatic hemophiliacs by in vitro immunoglobulin synthesis.

The objective of the present study was to evaluate the prognostic utility in determining the risk of AIDS progression in HIV-1-infected asymptomatic hemophiliacs by in vitro immunoglobulin (Ig) synthesis. With this aim, a cohort of 28 HIV-1-seropositive hemophiliacs were studied. All showed the number of CD4 lymphocytes higher than 400 positive cells/mm3. In all cases the spontaneous and pokeweed mitogen-induced in vitro production of Ig by peripheral blood lymphocytes was evaluated at the beginning of the study and the ratio stimulated/spontaneous (Stim/Spon) synthesis was calculated. At the same time, the absolute CD8+ cell count, IgA serum immunoglobulin, p24 HIV-1 antigenemia, and beta2 microglobulin were calculated. These data were monitored during the 4-year follow-up of patients and compared with the stimulated/spontaneous Ig synthesis ratio to evaluate the predictive significance on the progression of HIV infection. According to the stimulated/spontaneous Ig synthesis ratio, hemophilic patients were separated into two categories. Group I included 12 subjects with a Stim/Spon ratio higher than 2 (the lowest value of normal controls) and group II included 16 cases with a ratio lower than 2. As control, in 36 HIV-1-negative hemophiliac individuals the stimulated/spontaneous Ig ratio ranged between 2 and 42; mean +/- SEM, 12.9 +/- 1.8. At the end of the 4-year follow-up, group I patients showed a CD4 count and clinical staging consistent with those of the first evaluation; in contrast group II demonstrated a significant decrease in CD4 lymphocytes and deterioration of clinical conditions. Our results show that a low Stim/Spon Ig ratio when the CD4 lymphocyte count was still normal appears to predict the depletion of this lymphoid subset and progression to AIDS before T CD8, IgA immunoglobulin, p24 HIV-1 antigenemia, and beta2 microglobulin abnormalities. In this setting, the stimulated/spontaneous Ig ratio may represent a useful tool for clinical decisions in HIV-1-infected hemophiliacs.

Normative beliefs of health behavior professionals regarding the psychosocial and environmental factors that influence health behavior change related to smoking cessation, regular exercise, and weight loss.

PURPOSE: To examine the degree of consensus among health behavior change professionals regarding the personal and environmental factors they believe most strongly influence health behavior decisions related to smoking cessation, regular exercise, and weight loss. DESIGN: A factorial survey design was implemented. This method combines the positive elements from simple sample surveys and factorial experiment designs. A total of 44 independent psychosocial and environmental variables are used to randomly construct vignettes, or short stories, to collect dependent variable data. SUBJECTS: A probability sample of 311 health behavior change professionals was selected from the Society for Public Health Education, Inc., the Society of Behavioral Medicine, and faculty from the 1986 Harvard University Symposium on Health Promotion in the Work Place. MEASURES: Judgment ratings on the probability that the person described in each vignette would initiate the behavior in question. RESULTS: Multivariate analysis indicates that the multidimensional model explained approximately one half of the variance in the judgments across the three health behaviors (smoking cessation, R2 = .52; weight loss, R2 = .49; and regular exercise, R2 = .49). CONCLUSIONS: These data suggest a high degree of consensus among the sample regarding the personal and environmental factors that influence health behavior judgments. Here, the subjects perceive both behavioral intentions and self-efficacy as the most powerful determinants of judgments to initiate weight loss, regular exercise, and smoking cessation.

Warm heart surgery eliminates diaphragmatic paralysis.

Since January 1992, we adopted a new method of myocardial protection: warm blood cardioplegia with continuous ante-retrograde combined delivery during normothermic cardiopulmonary bypass, (CPB) instead of cold blood intermittent cardioplegia plus topical ice slush in hypothermic CPB. We have compared postoperative chest X-rays of 50 patients who underwent elective coronary artery bypass with normothermic CPB to postoperative chest X-rays, of 50 patients operated upon with hypothermia. In the cold group transitory diaphragmatic paralysis, as well as pleural effusions and thoracentesis related to the hypothermia, and topical cooling, were statistically increased over that of warm group. The data suggest that topical cooling with slush ice is responsible for phrenic nerve injury and that warm heart surgery has no associated incidence of diaphragmatic injury.

[Endometriosis of the sigmoid: 2 new cases and a review of the literature]. / Endometriosi del sigma: due nuove osservazioni e revisione della letteratura.

Endometriosis of the sigmoid colon is a pathology which is not frequently reported but may be manifested with symptoms in the form of intestinal occlusions and/or subocclusions and rectorrhagia. Diagnosis is difficult because there are no typical radiological and endoscopic findings. The authors report two cases of sigmoid endometriosis which were manifested by recurrent subocclusive attacks and rectorrhagia. Moreover, they stress the rarity of the site and review the literature in order to summarise the etiopathogenetic hypotheses and anatomopathological aspects. They also focus on clinical symptoms and on endoscopic and radiological findings. Lastly, they examine the problems of differential diagnosis and the therapeutic options.

[Immunostimulation in heart surgery: thymostimulin. Preliminary data]. / Immunostimolanti in cardiochirurgia: timostimolina. Dati preliminari.

The immunodepressive effect of surgical procedures is well known. Immunotherapy with thymic hormones has used a long time to prevent surgical immunitary damage. In cardiac surgery, we can observe a hard immunitary depression caused by the cardiopulmonary bypass (CBP). In this paper we evaluate the response to immunotherapy, with an extractive thymic hormone: thymostimulin at the dosage of 1 mg/kg/day; 20 patients of a group of 40 underwent CBP. Immunological monitoring was obtained with the "multitest CMI", a disposable skin test to evaluate changes of lymphocytes, especially T cells. Results shows a significant increase of response in patients treated with thymostimulin versus control. A larger sample and a deeper immunitary assessment will be provided in the future.