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Mitral valve dysfunction in the Marfan syndrome. Clinical and echocardiographic study of prevalence and natural history.

Although mitral regurgitation and fibromyxomatous thickening of the mitral leaflets have long been recognized as such, mitral valve prolapse has only recently been added as one of the pleiotropic features of the Marfan syndrome. The prevalence, age of onset, and natural history of mitral valve dysfunction in this condition are uncertain. Therefore, all patients in one clinic who met strict diagnostic criteria for the Marfan syndrome and who had clinical and echocardiographic examinations before age 22 years were reviewed. Of the 166 patients (84 males, aged 11.9 +/- 0.6 years [mean +/- SEM]; and 82 females, 11.0 +/- 0.6 years), 52 percent had auscultatory and 68 percent had echocardiographic evidence of mitral valve dysfunction, generally mitral valve prolapse. Prevalence did not differ between the sexes. Follow-up in 115 patients averaged five examinations over a mean of four years; 17 percent were followed for more than six years. Criteria for progression of mitral valve dysfunction were: (1) on auscultation, the appearance of new systolic clicks or apical systolic murmurs, a mitral regurgitant murmur increased by two grades, or appearance of congestive heart failure not due to aortic regurgitation; and (2) on echocardiography, the new appearance of mitral valve prolapse or abnormally increased left atrial dimension. Nearly half the patients met at least one criterion and one quarter had both auscultatory and echocardiographic evidence of progressive mitral valve dysfunction. Twice as many females demonstrated worse mitral valve function with time. Eight of the 166 patients either died as a result of mitral valve dysfunction or required mitral valve replacement. Severe mitral regurgitation developed in an additional 15 patients. Rupture of chordae tendineae was uncommon. Antibiotic prophylaxis was routine, and no cases of bacterial endocarditis of the mitral valve occurred. These results suggest that mitral valve dysfunction is extremely common in young patients with Marfan syndrome and usually presents as mitral valve prolapse. Serious mitral regurgitation develops in one of every eight patients by the third decade. Thus, the prevalence and natural history of mitral valve prolapse in the Marfan syndrome appear distinct from mitral valve prolapse associated with other conditions, including idiopathic or familial mitral valve prolapse.

Adolescent↗

Orthostatic hypotension due to hypertrophic cardiomyopathy and autonomic failure.

A case of orthostatic hypotension due to the combination of hypertrophic obstructive cardiomyopathy and autonomic failure is described. The interacting pathophysiologic features of the two disorders and response to therapy are discussed. The value of dynamic cardiac auscultation and Doppler echocardiography in patients with postural blood pressure changes is demonstrated.

Aged↗

Fetal acid-base balance in low-risk patients in labor.

The fetal acid-base balance from scalp blood in the early and late first stage of labor and from cord arterial blood was assessed in 120 low-risk patients only monitored electronically for 15 minutes at admission. The mean scalp blood pH values were 7.332 at a cervical dilatation of 5 cm and 7.335 at 10 cm cervical dilatation. A low pH value (less than 7.25) was found in 14 patients; five (4.2%) of them had fetal distress. Stethoscopic auscultation failed to identify these cases. Another nine patients had transient abnormal acid-base balance (a check value within 30 minutes was normal), and the fetal heart rate trace (obtained immediately after the sampling) showed no ominous changes in these cases. It is concluded that an abnormal fetal acid-base balance is not uncommon in a low-risk population in labor. However, a critical evaluation of the acid-base balance is necessary, since in most cases the abnormal acid-base balance is transient and of a respiratory, innocuous type without concomitant fetal distress.

Acid-Base Equilibrium↗

Experience with early postcesarean hospital dismissal.

OBJECTIVE: Our purpose was to assess early postcesarean hospital dismissal. STUDY DESIGN: A retrospective review was performed of all women receiving cesarean delivery over the most recent 6-month period in a busy private obstetrics practice that routinely dismisses its cesarean patients on postoperative day 2. Women who meet certain criteria (uncomplicated pregnancy, Pfannenstiel incision, uncomplicated surgery, no febrile morbidity, stable vital signs, ability to ambulate without assistance, ability to urinate without assistance, and auscultation of active bowel sounds) on postoperative day 2 are dismissed from the hospital. Outcomes were compared against women undergoing cesarean delivery during the 6 months immediately before the institution of the early dismissal program. RESULTS: Among 147 women undergoing cesarean deliveries, 117 (80%) met the criteria for early dismissal. When compared with controls (n = 93), there was no difference in outcomes. No one in the early dismissal group required readmission to the hospital. CONCLUSION: Among properly selected candidates, early postcesarean hospital admission is a reasonable option.

Adult↗

The effect of unsuspected carotid-cavernous fistula in enucleation.

An 18-year-old man with an unsuspected carotid-cavernous fistula underwent enucleation as the result of a severe localized perforating ocular trauma. Potentially lethal bleeding occurred that was difficult to control. Auscultation of the orbit should be performed in all cases before eye enucleation to avoid the possibility of overlooking a carotid cavernous fistula.

Adolescent↗

Surgical orthodontics and the temporomandibular joint. I. Superior repositioning of the maxilla.

Ten randomly selected adults who had undergone orthodontic treatment and isolated superior repositioning of the maxilla for vertical maxillary excess (VME) were evaluated clinically and radiographically (mean, 48.7 months postsurgery) for signs and symptoms of masticatory and temporomandibular joint dysfunction. The patients ranged from 18 years to 37 years of age (mean, 26.2 years) when evaluated. A three-part evaluation of the subjects was performed. This consisted of an anamnestic evaluation (previous medical history), a clinical examination, and a radiographic evaluation. The anamnestic evaluation revealed that, prior to surgery, facial pain was reported by one patient and was not present in any of the patients upon follow-up examination. We believed that the pain was not related to the masticatory musculature and/or the temporomandibular joint. No patients reported pain or sounds in their joints preoperatively, while 30 percent (3/10) of the patients related a history of temporomandibular joint sounds immediately after release of intermaxillary fixation, which subsequently was reported to have resolved in all instances without treatment. Clinical examination of the temporomandibular joints at the time of recall evaluated mandibular movements and the presence of pain or sounds during joint function. These examinations revealed that clinical measures of mandibular movements were somewhat reduced relative to normal. All patients were free of temporomandibular joint and masticatory muscle pain during function, upon contralateral masticatory loading, and upon palpation. Fifteen percent (3/20) of the joints examined demonstrated sounds (popping or crepitation) via auscultation. Masticatory loading in the contralateral premolar region did not induce noise in any of the joints examined. Cephalometric laminagraphic radiographs were obtained of each of the twenty temporomandibular joints with the mandible in three positions; maximum intercuspation, mandibular rest position, and maximal opening. Numerous anatomic relations were quantified from these radiographs. However, only three parameters (condylar position, movement, and evidence of arthrosis) were compared to normative data available in the literature. These comparative data suggested that persons who had undergone orthodontic treatment in conjunction with superior maxillary repositioning demonstrated (1) a relatively retropositional condyle within the fossa and (2) reduced condylar movement during maximal mandibular opening. Two of twenty temporomandibular joints demonstrated radiographic evidence of arthrosis; one condyle demonstrated articular surface erosions, and another exhibited articular surface sclerosis. The overall incidence of arthrosis was not much greater than normal, with 20 percent (4/20) of the joints demonstrating a reduced interarticular joint space. Overall, the clinical findings revealed a low incidence of pathologic masticatory muscle and temporomandibular joint symptoms and signs compared to normative data in the literature...

Adolescent↗

Stridor in childhood asthma.

Three asthmatic children are reported with shortness of breath due to stridor. The stridor was preceded by a deterioration in asthma control necessitating the introduction or increased dosage of a pressurized beclomethasone inhaler. The stridor mimicked asthmatic symptoms and was only differentiated by auscultation directly over the neck and by response to nebulized adrenaline. One child had a laryngeal foreign body which probably gained entry via a pressurized inhaler.

Administration, Inhalation↗

Comparison of the ID3 algorithm versus discriminant analysis for performing feature selection.

Having obtained disappointing results in a small medical data set despite the fact that our data seemed to be well suited for induction via ID3, we decided to compare the performance of ID3 to discriminant analysis. Performance was gauged by the percentage of correct classification in a second, independent data set. Examples were obtained from a cardiology project on the accuracy of auscultation. There were 107 examples in the first data set and 67 cases in the second. We found that ID3 and discriminant analysis performed equally poorly, with ID3 classifying only 60% of the second set correctly and discriminant analysis classifying 66% of the second set correctly. Also, the ID3 probability statistic for estimating the accuracy of ID3 for classifying further cases was markedly optimistic compared to our actual second data set results. Moreover, with an increase in sample size, ID3 seemed to break down, producing a large, complex decision tree of dubious generality, whereas discriminant analysis, with a larger sample size, used more independent variables but maintained its first set accuracy. These data suggest that there is a need for more sophisticated algorithms than ID3, even at the risk of giving up some computational efficiency.

Adult↗

[Analytical study and physiological consequences of a model of respiration].

The author, starting from the pattern established by Brocas and Cherruault (1973), develops new equations by considering the dynamics of breathing. He distinguishes space without exhanges, called 'blower' and space with exchanges, called 'exchanger'. The equations are studied and explicit solutions are found by an original method. The necessity for a neutral gas is proved. The author develops periodic functions for ventilation, for gas pressures in the air and in the blood and control functions such as differences of pressure between aerial- and blood-dissolved gas at the bottom of the alveola, consumption of O2 and excretion of CO2 and remaining CO2 in blood. The control functions oscillate around zero for the differences and around constants for the others. Examining the effects of frequency of breathing, blood flow, variations of transerval diffusivities, atmospheric pressure, variation of the dimensions connected with fever, infections, altitude and depth, sclerosis and even emotions, he again determines the pathologies, their auscultation symptoms and other aspects.

Biomechanical Phenomena↗

Evaluation of arthrographically assisted splint therapy in treatment of TMJ disk displacement.

Eighty-two patients with audible clicking were evaluated and treated with splints made by using arthrographic assistance. In the course of this study, it became apparent that the later the opening click, the earlier the closing click. It was not always possible to auscultate or palpate either an opening or a closing click in many patients with arthrographic findings of disk displacement with reduction. Since the opening click was the only audible sound in some patients, clinical judgment alone cannot be used to replace the displaced disk at an optimal mandibular position. The elimination of the opening click does not always signify recapture of the disk. Maxillomandibular and incisal relationships limit the amount of protrusion possible to recapture the displaced disk.

Adolescent↗

Renal arteriovenous fistula following nephrectomy.

Three cases of postnephrectomy renal arteriovenous fistula are described. A review of the literature shows the rarity of this complication since only 62 cases (including the author's cases) are known. Reported cases have been recognized after intervals up to forty years. The major complication is cardiac failure. Surgical treatment gives satisfactory results, but nonsurgical closure has now become possible. Early diagnosis is easy by auscultation of the loin which constantly reveals a continuous bruit.

Adult↗

Antepartum fetal assessment. A nurse-midwifery perspective.

This article provides an in-depth review of the most current antepartum fetal assessment techniques. Included in this review are both low- and high-technology methods, such as fetal movement counting, nonstress tests, vibroacoustic stimulation, auscultated acceleration tests, contraction stress tests, amniotic fluid index, biophysical profiles, and Doppler velocimetry. The interpretation of antepartum testing using screening test validity concepts is addressed, as is the current and emerging role of the nurse-midwife in fetal assessment. By integrating content on maternal and fetal physiology, including a critical review of current literature, together with relevant clinical information, including protocols, this article provides a useful guide to fetal assessment for nurse-midwives.

Embryonic and Fetal Development↗

The inhibitory effect of a selective alpha 2-adrenergic receptor antagonist on moderate- to severe-type asthma.

Midaglizole (DG-5128), a novel selective alpha 2-adrenergic receptor antagonist, was administered to 17 patients with moderate to severe asthma in a single-dose, double-blind, randomized, crossover study. All patients also continued their regular treatment regimens. FEV1 increased significantly (p less than 0.05), and respiratory resistance decreased significantly (p less than 0.05). Wheezing and dry rales on auscultation improved in patients receiving 200 mg of midaglizole, and their assessment of overall benefit was also significantly better (p less than 0.05). Blood pressure and heart rate were unaffected. However, plasma glucose levels fell slightly. This study demonstrated the bronchodilating effect of midaglizole in moderate to severe bronchial asthma. This selective alpha 2-adrenergic antagonist may prove to be a useful addition to current treatment regimens for asthma.

Adrenergic alpha-Antagonists↗

Effect of doxapram on postoperative pulmonary complications after upper abdominal surgery in high-risk patients.

In a double-blind randomised trial an infusion of doxapram, 2 mg per min for 6 h immediately after surgery and repeated on the first postoperative day, or the same volume of saline, was given to 39 patients who underwent upper abdominal surgery and who were at high risk of postoperative day, or the same volume of saline, was given to 39 patients who underwent upper abdominal surgery and who were at high risk of postoperative pulmonary complications. The patients were assessed pre-operatively and during the first 5 postoperative days by physical examination, spirometry, blood-gas analysis, and chest radiography. Postoperative pulmonary complications were defined as temperature over 38 degrees C for 2 days, abnormal auscultation, pathological radiography, and/or productive cough. Data from 16 patients per group were analysed. Significantly more patients in the placebo group had three criteria of postoperative pulmonary complication compared with the doxapram group (63% vs 19%). The doxapram group also had higher PaO2 postoperatively.

Abdomen↗

Does mitral valve prolapse cause nonspecific symptoms?

I studied the prevalence and symptoms of idiopathic mitral valve prolapse by auscultation in 972 consecutive patients in an adult general medical population. Forty-five patients (4.6%) had idiopathic mitral valve prolapse defined by a nonejection click with or without a late systolic murmur. The prevalence was not significantly different in men and women. The mean age (49.9 yr) and age distribution of patients with prolapse were similar to those of patients without prolapse (47.7 yr). The prevalence of dizziness (4.1% vs. 1.5%), fatigue (4.4% vs. 2.6%), and palpitations (4.4% vs. 1.3%), was not significantly greater in patients with or without prolapse. Atypical chest pain (13% vs. 4.3%) and chronic anxiety (8.8% vs. 2.9%) were more frequent (less than 0.05) in the patients with prolapse than in those without prolapse. Of the patients with prolapse, 29 were healthy without clinically identifiable diseases while 16 had medical diseases. In the group without prolapse, 184 patients were healthy and 707 had other diseases. When patients with isolated prolapse without other associated diseases were compared to healthy patients without prolapse, the prevalence of atypical chest pain (17.4% vs. 17.2%) and chronic anxiety (7.1% vs. 10.3%) were not significantly different. When patients with prolapse and other diseases were compared to patients without prolapse and other diseases, the prevalence of atypical chest pain (6.2% vs. 1.1%) and chronic anxiety (6.2% vs. 1.7%) was again not significantly different. Thirty-two patients without prolapse were suspected but not confirmed of having disease and were not included in this analysis. The results would have been unaltered by their inclusion in the diseased group without prolapse.

Age Factors↗

Re-evaluation of normal splitting of the second heart sound in patients with classical left bundle branch block.

To study the mechanism of normal splitting of the second heart sound in patients with classical left bundle branch block, we investigated 43 such patients and 15 normal controls, using electro-, phono- and echo-cardiography and comparing the relative timing of mechanical activity in the two ventricles. The splitting of the second heart sound is reversed in only two-thirds of the patients and normal in remaining one-third. Comparing patients with and without reversed splitting, there are no significant differences in left ventricular cavity size, heart rate, pre-ejection period and the distribution of age, gender, or aetiology. QRS duration is longer (P < 0.01) in patients with reversed splitting. Diastolic events of the left ventricle do not differ between groups. The onset of the left ventricular free wall motion is delayed compared with normal by a similar extent in the two groups. In patients with normal splitting, the onset of the right ventricular wall motion is also delayed, both with respect to normal and to those with reversed splitting to an extent similar to that seen in classical right bundle branch block. Normal splitting of the second heart sound associated with an electrocardiographic pattern of left bundle branch block therefore suggests bilateral block. This combination can be documented from the precise timing of the movement of the two ventricles by M-mode echocardiography and identified by simple auscultation.

Aged↗

Lung perforation by nasogastric feeding tubes.

Endotracheal misdirection of narrow bore nasogastric feeding tubes resulted in perforation of the lung, pneumothorax and hydrothorax in two intensive care patients. Both were intubated with cuffed endotracheal low pressure tubes, one patient was on respirator therapy with neuromuscular relaxation. Feeding tubes were inserted by experienced personnel with the assistance of a steel stylet without difficulties. Aspiration of fluid was misinterpreted as proof of correct positioning, the liquid being however pleural effusion and not gastric juice. Similarly auscultation of gurgling sounds in the upper epigastrium was not a reliable sign of intragastric position. Insertion of nasoenteric feeding tubes may be complicated by perforation of the upper gastrointestinal tract and lung in poorly responsive patients with cuffed endotracheal devices during neuromuscular blockage. In these patients a laryngoscope and forceps should be used to ensure free passage of the tube into the oesophagus. Röntgenographic confirmation of correct positioning of the tube immediately after insertion is mandatory.

Journal Article↗