Infrequent association: massive inguinoscrotal bladder hernia, multiple scrotal lithiasis, and bilateral obstructive uropathy]

BACKGROUND AND AIMS OF THE STUDY: Surgical treatment of functional tricuspid regurgitation associated with advanced valvular disease remains controversial, mainly due to the difficulty in choosing between valve replacement or reconstructive surgery. Failure to correct tricuspid regurgitation during valvular surgery carries a poor prognosis, as reoperation may represent a significant risk to the patient. Thus correct tricuspid valve surgery is vital to achieve improved early and long-term clinical results. METHODS: A total of 142 patients underwent concomitant tricuspid annuloplasty with mitral valve replacement and their clinical outcome was assessed. RESULTS: Overall hospital mortality rate was 11.3%. Seven patients died during follow up. The overall actuarial survival rate for 10 years was 74.1 +/- 14.2%. Postoperatively, 75.8% of the patients had no residual tricuspid insufficiency, while 24.2% had first- or second-degree tricuspid insufficiency and were treated medically. All patients were in NHYA functional class I or II postoperatively. CONCLUSION: Clinically, it is important to assess the severity of functional tricuspid insufficiency before and/or during the operation. Bicuspidalization annuloplasty for functional tricuspid insufficiency has provided good results, with a low incidence of reoperation and significant clinical improvement. The technique could be used in most patients with functional tricuspid regurgitation.     

Right thoracotomy to approach mitral or tricuspid valve in reoperation

Four patients underwent mitral valve re-replacement, and required tricuspid annuloplasty in two, through a right thoracotomy. There was no major postoperative morbidity and recovery was full and uneventful. The indications for selection of this approach were isolated mitral valve disease or combined tricuspid and mitral valve disease, severe adhesion between heart and sternum on CT scan, intact aortic valve and no severe reduction of respiratory function. We recommend right thoracotomy to approach atrioventricular valves in selected situations in patients for reoperations.     

Neuropsychiatric correlates and treatment of lenticulostriatal diseases: a review of the literature and overview of research opportunities in Huntington's, Wilson's, and Fahr's diseases. A report of the ANPA Committee on Research. American Neuropsychiatric Association

This report reviews clinical neuropsychiatric findings and opportunities for research in Huntington's, Wilson's, and Fahr's diseases. Consistent, systematic methodology is lacking among neuropsychiatric studies in these lenticulostriatal diseases. Systematic cross-sectional and longitudinal assessments are needed to ascertain the prevalence of psychiatric disorders as a function of disease course. Preliminary synthesis of existing data suggests the following heuristic relationships in these diseases: depression with parkinsonian states; personality changes with caudate or putamen disease; psychosis, impulsivity, and sexual disorders with caudate disease; dementia and mania with caudate and pallidal diseases; and compulsions with pallidal disease. Correlation of neuropsychiatric findings with disease stage, clinical signs, and radiologic, metabolic, physiologic, and pathologic markers of disease will add to our understanding of these conditions.     

Mimics of Ebstein's anomaly

The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein's anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein's anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein's anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein's anomaly but were found to have other abnormalities of the tricuspid value or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein's anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein's anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein's anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein's anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (> or = 8 mm/m2) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein's anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (> or = 8 mm/m2) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein's anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein's anomaly had important surgical implications.     

Surgical treatment of combined valvular disease in 680 patients

Between 1982 and May 1993, we operated on 680 patients with combined valvular disease. Previous operations were including closed mitral commissurotomy in 87, and bioprosthetic valve replacement in mitral position in 15. The types of combined valve disease included mitral valve disease combined with functional tricuspid regurgitation (FTR) (245), with organic tricuspid disease (OTD) (10); mitral and aortic disease (258), combined with FTR (128), and with OTD (39). The early mortality was 4.7% in total 2.4% and 6.1% respectively in MVR or DVR plus tricuspid procedures. The main cause of early death was low cardiac output syndrome. The late mortality was 2.6% pt-yr, congestive heart failure and coagulant-related hemorrhage were the predominant causes of late death. We considered that FTR is the outcome of right ventricle decompensation, while tricuspid valve must be actively inspected intraoperatively, and must be corrected completely. OTD can be successfully repaired in most patients. Myocardial protection should be emphasized, and continuous perfusion of cold blood cardioplegia and controlled reperfusion of warm blood contained mannitol have marked effect.     

Angiotensin-converting enzyme (ACE) inhibitors revert abnormal right ventricular filling in patients with restrictive left ventricular disease

OBJECTIVES: Our aim was to determine mechanisms underlying abnormalities of right ventricular (RV) diastolic function seen in heart failure. BACKGROUND: It is not clear whether these right-sided abnormalities are due to primary RV disease or are secondary to restrictive physiology on the left side of the heart. The latter regresses with angiotensin-converting enzyme inhibition (ACE-I). METHODS: Transthoracic echo-Doppler measurements of left- and right-ventricular function in 17 patients with systolic left ventricular (LV) disease and restrictive filling before and 3 weeks after the institution of ACE-I were compared with those in 21 controls. RESULTS: Before ACE-I, LV filling was restrictive, with isovolumic relaxation time short and transmitral E wave acceleration and deceleration rates increased (p < 0.001). Right ventricular long axis amplitude and rates of change were all reduced (p < 0.001), the onset of transtricuspid Doppler was delayed by 160 ms after the pulmonary second sound versus 40 ms in normals (p < 0.001) and overall RV filling time reduced to 59% of total diastole. Right ventricular relaxation was very incoordinate and peak E wave velocity was reduced. Peak RV to right atrial (RA) pressure drop, estimated from tricuspid regurgitation, was 45+/-6 mm Hg, and peak pulmonary stroke distance was 40% lower than normal (p < 0.001). With ACE-I, LV isovolumic relaxation time lengthened, E wave acceleration and deceleration rates decreased and RV to RA pressure drop fell to 30+/-5 mm Hg (p < 0.001) versus pre-ACE-I. Right ventricular long axis dynamics did not change, but tricuspid flow started 85 ms earlier to occupy 85% of total diastole; E wave amplitude increased but acceleration and deceleration rates were unaltered. Values of long axis systolic and diastolic measurements did not change. Peak pulmonary artery velocity increased (p < 0.01). CONCLUSIONS: Abnormalities of RV filling in patients with heart failure normalize with ACE-I as restrictive filling regresses on the left. This was not due to altered right ventricular relaxation or to a fall in pulmonary artery pressure or tricuspid pressure gradient, but appears to reflect direct ventricular interaction during early diastole.     

A double-blind, placebo controlled, dose ranging study to evaluate the safety and biological efficacy of the lipid-DNA complex GR213487B in the nasal epithelium of adult patients with cystic fibrosis

Dramatic physiologic changes are part of normal human pregnancy. The physiologic alterations of pregnancy have the potential to affect chronic diseases, to unmask subclinical conditions, or to alter the presentation and course of newly acquired illnesses. An update in selected topics of obstetric medicine follows, focusing on clinical entities in which there have been significant advances in diagnosis or management. Additionally, reviews of selected medical disorders, such as HIV infection and asthma, that are rising in incidence in women of reproductive age are included.     

A modified repair technique for tricuspid incompetence in Ebstein's anomaly

OBJECTIVE: A modified technique for tricuspid valve repair in Ebstein's anomaly restructures the valve mechanism at the level of the true tricuspid anulus by using the most mobile leaflet for valve closure without plication of the atrialized chamber. Midterm results of this therapeutic approach for patients with Ebstein's anomaly and tricuspid valve incompetence are reported. METHODS: Between October 1988 and April 1997, the incompetent tricuspid valve was repaired with our technique in 19 patients (12 female, 7 male; 2 to 54 years, mean 21 years). The indication for operation was congestive heart failure of various degrees in all patients. Tricuspid incompetence was grade II in two patients, grade III in 14, and grade IV in three. Associated congenital malformations were simultaneously repaired (interatrial communication in 18, ventricular septal defect in two, pulmonary stenosis in two, mitral valve prolapse in one). Follow-up ranged between 10 and 103 months (median 28 months) and was complete for all patients. RESULTS: There were no operative deaths. One patient with active endocarditis and pulmonary abscess died 2 months after the operation of recurrent sepsis; there were no late deaths. During follow-up, New York Heart Association functional class improved from 2.8 before the operation to 1.9 without recurrent cyanosis, and tricuspid incompetence decreased from a mean grade of 3.1 to one of 0.9, without any echocardiographic deterioration of the tricuspid valve function or right ventricular dilation. CONCLUSIONS: Our technique allows tricuspid valve repair in patients with Ebstein's anomaly, even in cases usually reserved for primary valve replacement, without late functional deterioration.     

Spontaneous resolution of massive congenital tricuspid insufficiency

Four cases of congenital tricuspid insufficiency (CTI) are presented in whom spontaneous improvement occured. There was no evidence of Ebstein's disease in three of the four. All presented with similar clinical and laboratory findings including massive cardiomegaly, mild to moderate congestive heart failure, and moderate to severe desaturation. Catheterization data demonstrated severe TI and right-to-left atrial shunting. During the period of follow-up, up to 3.5 years, all symptoms have spontaneously disappeared. Clinical and angiocardiographic evidence for minimal residual TI have remained. Right atrial and right ventricular volume measurements were markedly abnormal initially and returned to normal or near normal. These measurements may be a useful method for quantitating the severity of tricuspid insufficiency. The course of the three infants with massive CTI (without Ebstein's disease) differs from previous reports and may be important both in formulating future management plans and in defining the etiology of the disorder.     

A further look at tricuspid annuloplasty

Is it necessary to replace the tricuspid valve or insert a tricuspid ring for pure tricuspid insufficiency, or is repair satisfactory? In 96 of 113 patients with pure tricuspid insufficiency the tricuspid valve was repaired by converting the incompetent three-leaflet valve into a two-leaflet one. To avoid liver damage in these critically ill patients, the inferior caval tie was omitted during the open-heart procedure. There have been 5 deaths in the last 51 consecutive operations. Three patients developed recurrent tricuspid insufficiency secondary to failure of the mitral repair or replacement. It is concluded that tricuspid repair for pure tricuspid insufficiency is a simple and excellent method for treating severe, pure tricuspid insufficiency.     

Inflammatory disorders of the heart. Pericarditis, myocarditis, and endocarditis

The emergency physician frequently sees patients with cardiac complaints. Too often, ischemic heart disease is overwhelmingly considered to the exclusion of other diagnostic possibilities such as inflammatory cardiac disorders. These disorders can cause considerable discomfort, have long-term implications, or lead to more serious cardiac disorders. Emergency physicians must have a practical understanding of the diagnostic evaluation and therapeutic approach to patients with inflammatory cardiac disorders.     

Unilateral lenticular infarcts: radiological and clinical syndromes, aetiology, and prognosis

OBJECTIVES: To analyse the clinical features induced by lenticular infarction found in 20 patients, and to analyse the radiological and clinical correlations. METHODS: Eight women and 12 men, mean age 73 years, were included in this study, which was carried out from 1 January 1994 to 30 November 1996. They were characterised by the onset of a lenticular infarction, shown by CT and MRI. A complete neurological and neurocognitive examination, and photon emission computed tomography (SPECT), were performed in all the patients and there was a long clinical follow up. RESULTS: Two distinct clinical syndromes were identified corresponding to the two anatomical areas of the lenticular nucleus: behavioural and cognitive disorders were associated with infarcts within the globus pallidus, whereas both motor disorders (dystonia) and cognitive disorders were associated with infarcts within the putamen. Outcome was excellent in all the patients for motor function, but slight cognitive disorders, problems with short term memory, and dysphasia persisted for several months. The size of the lesion did not explain these symptoms. By contrast, the slight reduction in cerebral blood flow found in the adjacent frontotemporal area may explain them by a deafferentation or a diaschisis phenomenon. CONCLUSION: It is possible to identify the clinical symptoms of a single lesion in the pallidus nucleus and in the putaminal nucleus, in which behavioural, cognitive, and movements disorders are important. After an acute and spectacular onset, outcome is in general excellent. A disease of the small arteries must be involved.     

Rat testicular extracellular superoxide dismutase: its purification, cellular distribution, and regulation

Rehabilitative measures for stroke are not generally based on basic neurobiological principles, despite evidence from animal models that certain anatomical and pharmacological changes correlate with recovery. In this report, we use functional magnetic resonance imaging (fMRI) to study in vivo human brain reorganization in a right handed patient with an acquired reading disorder from stroke. With phonological dyslexia, her whole-word (lexical) reading approach included inability to read nonwords and poor reading of function words. Following therapy, she was able to read nonwords and function words, and preferred a decompositional (sub-lexical) strategy in general. fMRI was performed during a reading task before and after treatment. Prior to therapy, her main focus of brain activation was in the left angular gyrus (area 39). After therapy, it was instead in the left lingual gyrus (area 18). This result suggests first that it is possible to alter brain physiology with therapy for acquired language disorders, and second, that two reading strategies commonly used in normal reading use distinct neural circuits, possibly reconciling several conflicting neuroimaging studies of reading.     

Left ventricular diastolic function: physiology, methods of assessment, and clinical significance

Diastole, that portion of the cardiac cycle that begins with isovolumic relaxation and ends with mitral valve closure, results in ventricular filling and involves both active (energy-dependent) and passive processes. The interactions between active processes (myocardial relaxation) that primarily influence early ventricular filling and passive processes, such as loading conditions, myocardial compliance, and valvular disease, are complex. Clinical methods to assess ventricular filling include cardiac catheterization, radionuclide angiography, and echocardiography. Any measurements of diastolic function must be made with an understanding of the determinants of ventricular filling and the limitations of the diagnostic test. Many cardiac disorders are characterized by elevated pulmonary venous pressures in the face of normal systolic ventricular function, which suggests a primary abnormality of diastolic function. Abnormalities in diastolic function have been observed in coronary artery disease, congestive heart failure (with and without systolic dysfunction), hypertrophic cardiomyopathy, hypertension, and in healthy elderly subjects. Identification of these abnormalities may be useful clinically, particularly in patients with symptoms of heart failure and normal systolic function. Data are not available to determine the optimal therapy for such patients, although evidence suggests that calcium channel blockers, beta blockers, and agents that reverse myocardial hypertrophy may be useful. This review briefly summarizes the physiology of diastole, the methods of clinical assessment of diastolic function, and the role of diastolic function in cardiovascular disease.     

Post-transcriptional block in oxytocin gene expression within the seminiferous tubules of the bovine testis

Immunodeficiency is frequently invoked as an ethiopathogenetic factor for many somatic diseases. On the other hand, stress, depression, and psychotic disturbances are associated with severe immunological disorders. Taking into account that the benzodiazepines (BZ) are the psychoactive drugs more widely used than any other to treat psychological disturbances, it seems important to elucidate the immuno-enhancing or immunosuppressant potential of such drugs. Our goal was easily reached, since 69% of the outpatients visiting our Institute are chronic BZ consumers and because neurochemical, hormonal, immunological, and psychiatric investigations are routinely performed on all of our patients. In the present study, immune function was investigated on two occasions: while the patient was on active medication and 15 days after discontinuation. We concluded that chronic consumption of BZ provokes significant immunological disorders that should be further investigated. Said disorders could not be linked to a pre-existing affective disease or psychosis, since we only selected those BZ users in whom psychiatric investigations ruled out a past or present history of major psychiatric disease.     

Postoperative oxygen therapy

Much has been published in the medical literature concerning adverse events relating to the surgical patient. Among the notable disorders requiring the expertise of the postanesthesia care unit nurse are the diagnosis and management of respiratory dysfunction acutely attributable to the effects of surgery and anesthesia. Inhalational and/or intravenous anesthetic agents contribute to pathophysiological alterations that lend to the development of hypoxemia in the postoperative period. When patients present with preexisting respiratory disease, their care is frequently more complex and challenging. This review session will address the oxygenation component of respiration and the perioperative influences that alter it as well as treatment considerations for normalizing oxygenation.     

Long-term outcomes of nonsurgical treatment in nonreducing anteriorly displaced disk of the temporomandibular joint

OBJECTIVE: To review the evidence supporting the biopsychosocial model in understanding patients with gastrointestinal disorders (GI). METHOD: Essay of personal experience and review of related literature through a MEDLINE search. RESULTS: Through clinical examples of three common gastrointestinal disorders, a case is made to refocus our understanding from a biomedical or disease-based model of illness to a biopsychosocial model. With the latter model, the psychosocial and biological predeterminants are seen to interact in the clinical expression of illness and disease. With gastroesophageal reflux disease, the evidence shows that stress can lead to amplification of heartburn symptoms that is independent of the degree of reflux. Functional gastrointestinal pain is "an illness without disease," where structural or physiological disturbance of the GI system does not exist. Rather, the symptoms are understood in terms of visceral hypersensitivity as modulated by central nervous system activity. With the Crohn's disease example, the clinical expression of the disorder is not explained by the degree of disease activity. Rather, the symptoms and impaired quality of life relate to preexisting psychosocial determinants. The observed association of stress with disease activation in Crohn's disease is explained by stress-related alterations in psychoimmunological function via the hypothalamic-pituitary-adrenal axis. CONCLUSIONS: Gastrointestinal disorders, as a model for other medical conditions, exemplify the important role of an integrated, biopsychosocial model of illness.     

Minimum requirements, accreditation, certification, quality: connections and problems. An editorial comment on "ISO 9000: guidelines for a total quality system in health"]

Two babies with duct-dependent cyanotic congenital heart disease were palliated with modified Blalock-Taussig shunts. One patient was diagnosed to have tetralogy of Fallot, and the other patient, tricuspid atresia type Ia. Early postoperative arterial oxygen saturations dropped significantly due to shunt thrombosis. Both patients were successfully treated with angioplasty and stent implantation.     

Granulocytes in the subarachnoid space of humans and rabbits with bacterial meningitis undergo apoptosis and are eliminated by macrophages

The sphingolipid storage disorders constitute a group of inherited metabolic disorders in which the structure of the stored sphingolipid and the corresponding genetic defect have been established. However, the pathological mechanism(s) behind the disorders has not been fully elucidated. Sphingolipids are known to be recognition molecules involved in intercell communication and altered expression might lead to dyscommunication. The impaired degradation and lysosomal accumulation of specific sphingolipids might influence the metabolism of other molecules and/or intracellular transport, which in turn might alter the distribution of these molecules. However, the progress of these diseases indicates that additional factors, besides the stored sphingolipid itself, might be involved. During the last decade, several sphingolipids have emerged as active participants in intracellular signalling processes such as growth control and apoptosis. Particular interest focused on the sphingolipid metabolites, ceramide and sphingosine, as potential mediators in intracellular events and an altered presence of these metabolites in sphingolipidoses cannot be ruled out. Some sphingolipids have been found to influence cytokine release and thereby might induce immunological processes, which are known to exist in at least one of the sphingolipidoses--Gaucher disease. These processes might already have a pathogenic effect during early development, before significant storage has occurred.