Unilateral tibial agenesia with preaxial polysyndactyly and renal disorder in two patients: a new syndrome?

Left tibial agenesis, polysyndactyly with talipes equinovarus deformity and Grade IV vesicoureteral reflux of the right kidney are described in a 40-day-old male and an unrelated 1-month-old male, is also reported with right tibial agenesis, polysyndactyly with talipes equinovarus deformity and right kidney agenesis and left Grade V vesicoureteral reflux. No other pathology was recorded. Follow up at 1 year and 3 years, respectively, revealed normal motor and mental development. As this combination has been unpublished before, we believe that this a new syndrome.

Factors influencing the duration of exclusive breastfeeding in a group of Turkish women.

This study was conducted to evaluate the influence of demographic characteristics, hospital practices, maternal psychosocial factors, and knowledge about infant feeding and breast milk on duration of breastfeeding. The mothers of 91 healthy, term infants delivered at a university hospital between June 1998 and December 1998, and first seen in the well-child unit within 10 days of delivery, participated in the study. Forty-nine (54%) infants were exclusively breastfed at 4 months of age. Cox regression analysis showed a negative association between formula supplementation during the hospital stay and duration of exclusive breastfeeding. The median age for starting non-breast milk liquids was 1 month for those who received formula in the hospital and 3 months for those who did not (P = .001). The hospital practices were more predictive of the duration of exclusive breastfeeding in this study group than mothers' knowledge of infant feeding or psychosocial factors. Thus, hospital practices should be reevaluated.

Modification of Christensen urease test as an inexpensive tool for detection of Helicobacter pylori.

About half the world population is infected with Helicobacter pylori. Most live in developing countries where clinical studies face the constraints of high costs of imported rapid diagnostic tests. In this work, we describe and validate a simple local urease test (LUT) to determine the presence of the bacterium in gastric biopsies, and report the incidence of infection among symptomatic patients in Caracas, Venezuela. Statistical comparison of LUT and CLOtest (Delta West, Bentley, Australia) (N = 216 patients) showed that the probability of 95% agreement between the two test was 0.936. Overall incidence of infection determined by the LUT was 65% (N = 229), and it was higher in patients from public (72%; N = 153) than from private (50%; N = 76) hospitals (p = .001). Therefore, the incidence of infection differs in two socioeconomic groups that coexist in the same city. LUT may represent an affordable tool in clinical studies needed to identify social factors that increase the risk of infection by H. pylori.

Failure of transesophageal echocardiography to visualize a large mitral prosthesis vegetation detected solely by transthoracic echocardiography.

Transesophageal echocardiography yields a highly accurate diagnosis in the detection of valvular vegetations. We describe a case in which omniplane transesophageal echocardiography failed to demonstrate a large vegetation protruding from a Starr-Edwards mitral prosthesis, toward the left ventricular outflow tract. The vegetation could be detected solely by transthoracic echocardiography.

Echocardiographic assessment of prosthetic valve endocarditis.

Prosthetic valve endocarditis is still a very serious complication, carrying an incidence of death between 30 and 70% in some series. Therefore early and accurate diagnosis is crucial. Early (less than 60 days post surgery) endocarditis is usually a fulminant disease, where staphylococcal infection is most common. Late prosthetic endocarditis resembles more closely other forms of the disease. Conventional echocardiography is useful in the evaluation of prosthetic valve function, but it is very limited in the demonstration of infective lesions, primarily because of acoustic shadowing. Transoesophageal echocardiography (TE) enables high resolution imaging of the heart without chest wall interference, and viewing of the heart from the posterior (atrial, low pressure) side, where most of the vegetations are expected to be found in both mitral and tricuspid positions. It also enables better visualization of the left ventricular outflow tract, where aortic prosthetic vegetations tend to be present. Furthermore, transoesophageal echocardiography allows accurate diagnosis of some of the common complications of endocarditis: abscess/cavity formation; mycotic aneurysm; prosthetic valve dehiscence and regurgitation. In spite of these advantages, limitations should be recognized. Struts are commonly seen on transoesophageal echocardiography following surgery and should not be confused with vegetations. Similarly, normal prosthetic regurgitation should not be confused with paravalvar leakage. Nevertheless, transoesophageal echocardiography, when expertly used, changes the possibility for early and more accurate diagnosis of prosthetic valve endocarditis dramatically. Transoesophageal echocardiography should be included among the major criteria in the diagnosis and follow-up of prosthetic valve endocarditis.

[Usefulness of the PCR technique (polymerase chain reaction) in the follow-up of patients infected with hepatitis C virus. Preliminary communication]. / Utilidad de la Técnica de PCR (reacción en cadena de la polimerasa) en el seguimiento de pacientes infectados con el virus de la hepatitis C. Comunicación preliminar.

UNLABELLED: In the study we show the usefulness of PCR (polymerase chain reaction) to follow patients with chronic hepatitis, infected with hepatitis C virus (HCV) of Centro Médico de Caracas. The study included 14 patients: 12 anti-HCV positive, 1 with chronic autoimmune hepatitis and 1 classified as non B-non C hepatitis. The patients were divided in 3 groups: Group 1 (5 pretreatment patients, anti-HCV+), 4 with increase in ALT and PCR positive, 1 with normal ALT and PCR negative. Group 2 (7 treated with recombinant interferon alpha 2b), 4 without normalization of ALT and PCR positive, 3 with normalization of ALT and PCR negative. Group 3 (control) 2 patients anti-HCV negative and PCR negative. Two posttreatment patients could be genotyped: one patient was infected with 1a and showed an early relapse with treatment and the other was infected with genotype 1b, which is reported to be more refractory to antiviral treatment. CONCLUSIONS: the results show a 100% correlation between biochemical markers of HCV infected patients and the presence of viral RNA detected by PCR. the usefulness of determination of genotype to assess any prognostic value of this parameter in Venezuela is discussed.

Decreased thickening of normal myocardium with transient increased wall thickness during stress echocardiography with atrial pacing.

Stress echocardiography is used increasingly in the evaluation of coronary artery disease. The echocardiographic evaluation of ischemia is based on stress-induced changes in wall motion and wall thickening of the ischemic segments. Studies have demonstrated that left ventricular volumetric changes may induce changes in wall thickness. The aim of the study was to evaluate whether significant changes in ventricular volume, wall thickness, and wall thickening occur during stress echocardiography with atrial pacing. Seven German Landrace female pigs were studied 4 weeks after the induction of a small myocardial infarction. Echocardiographic measurements were conducted in noninfarcted segments on the short-axis view at baseline and during atrial pacing at 120, 150, and 180 beats/min. End-diastolic circumferential area decreased from 12.3 +/- 2.0 cm2 at baseline to 8.9 +/- 1.9 cm2 at 180 beats/min of atrial pacing (p < 0.01). Mean wall thickness (interventricular septal plus posterior wall thickness divided by 2) increased markedly and progressively from 6.7 +/- 0.6 mm at baseline to 9.8 +/- 1.0 mm at 180 beats/min (p < 0.01). The increase in wall thickness correlated inversely with end-diastolic area (r = -0.57; p < 0.01). Percent systolic thickening decreased from 38.9 +/- 12.0 at baseline to 14.9 +/- 7.4 at 180 beats/min of atrial pacing (p < 0.01). The decrease in percent wall thickening correlated with the increase in wall thickness (r = -0.71; p < 0.01). In conclusion, this study shows that a marked increase in wall thickness (pseudohypertrophy) and decrease in percent systolic thickening are observed during rapid atrial pacing in normal myocardium and do not indicate stress-induced left ventricular dysfunction.

Persistence of normal right ventricular Doppler filling pattern early after tricuspid valve excision.

Two patients with acute tricuspid bacterial endocarditis in which a normal right ventricular Doppler filling pattern was demonstrated early following valvectomy are reported. After surgery, on pulsed Doppler examination, the sample volume positioned at the right atrioventricular level revealed a normal M-shaped filling pattern. A pathologic monophasic pattern was documented a few months later. Our findings suggest that early after surgery the preserved gradient throughout ventricular diastole leads to a passive and active filling similar to normal. Only at a later stage is the grossly dilated right atrium unable to maintain active filling, and the 'A' wave disappears despite the fact that sinus rhythm is maintained. Tricuspid valve diastolic motion represents a product of the several factors that determine atrioventricular gradient, but the valve itself appears not to be involved in the generation of a normal filling pattern.

Additional ST segment elevation during the first hour of thrombolytic therapy: an electrocardiographic sign predicting a favorable clinical outcome.

OBJECTIVES: The aim of this study was to investigate the significance of further ST elevation that occurs during the 1st h of thrombolytic therapy before the expected resolution. BACKGROUND: Early resolution of ST segment elevation is commonly accepted as a marker of clinical reperfusion during thrombolytic therapy for acute myocardial infarction. Using frequent electrocardiographic recordings, we observed in some patients further ST elevation that occurred during hour 1 of thrombolysis before the expected resolution. METHODS: To investigate the significance of this pattern, we classified 177 consecutive patients with a first acute myocardial infarction into two groups: Group A, 98 patients with ST elevation > or = 1 mm above the initial ST elevation during the 1st h of thrombolytic therapy, and Group B, 79 patients without this finding. RESULTS: Although the presence or absence of additional ST elevation was not associated with a clinical or prognostic difference in patients with a first inferior or posterior acute myocardial infarction, its presence indicated a more favorable clinical outcome and prognosis in patients with anterior infarction. Among the patients with anterior infarction the 65 patients in Group A had a higher ejection fraction (44 +/- 9% vs. 35 +/- 11%, p < 0.01), less heart failure (15% vs. 35%, p = 0.02) and a lower in-hospital mortality rate (0% vs. 8%, p = 0.04) than did the 37 patients from Group B. CONCLUSIONS: Additional ST elevation early during thrombolytic therapy in patients with anterior infarction suggests a favorable clinical outcome and thus may be indicative of successful reperfusion.

Effects of the dipyridamole test on left ventricular function in coronary artery disease.

The dipyridamole stress test is used with thallium-201 to detect areas of inhomogeneity of blood flow that point to coronary artery disease (CAD). It is unclear whether dipyridamole produces inhomogeneous perfusion only or whether it actually decreases net flow in the obstructed vessels and produces true ischemia. It is also unclear what effect dipyridamole has on global and segmental left ventricular function. Therefore, ejection fraction, segmental wall motion and ventricular volume equivalents were measured before and after dipyridamole in 113 patients and 32 normal subjects. Ejection fraction responded in an abnormal fashion in 98 patients (87%), decreasing from 49 +/- 11% to 43 +/- 13% (p less than 0.0001), whereas it increased in 29 normal subjects (90%) from 57 +/- 6% to 64 +/- 10% (p less than 0.0001). Wall motion worsened distinctly in 75 patients (66%), and pressure/volume ratio deteriorated in 72%. The effect of dipyridamole lasted between 10 and 25 minutes, but was promptly reversed by aminophylline. These findings indicate that dipyridamole generally induces true ischemia in CAD. Furthermore, the degree of dysfunction is related to the angiographically assessed severity of CAD. The shortness of breath (seen in 10% of patients) may be partially explained by the findings, and it seems advisable to give aminophylline to every patient in order to promptly correct left ventricular dysfunction.

[Effect of tourniquet occlusion on peripheral blood pooling and ventricular function].

Rotating tourniquets are traditionally part of the treatment of acute pulmonary edema. However, their effectiveness has been questioned. A radioisotope technique was therefore used to evaluate directly the increments in the blood volume of the leg after venous occlusion using a pressure of 60 mmHg in 26 patients with left ventricular (LV) dysfunction following myocardial infarction. The increment in mean radionuclide count at serial 15-second intervals (reflecting the blood volume in the leg distal to the occlusion) increased significantly from the pre-occlusion value by 46 +/- 26% (p less than 0.0005). Thus satisfactory trapping of blood is achieved even in LV failure. However, mean ejection fraction decreased slightly but significantly from 0.23 +/- 0.10 to 0.21 +/- 0.10 (p less than 0.05), a decrease observed in 18 of the 26 patients. LV end-diastolic and end-systolic volume equivalents tended to decrease slightly, but not in all patients. Mean stroke volume and cardiac output equivalents were reduced by 14% (p less than 0.0005), while calculated peripheral resistance increased significantly. The present study fails to support the hypothesis that preload reduction by tourniquets improves LV function.

The paradox of sudden slowing of idioventricular rhythm during exercise in complete heart block: modulation of automatic focus, concealed reentry, or Wedensky inhibition?

As a rule, exercise enhances the rate of discharge of idioventricular rhythms in complete atrioventricular (AV) block. The opposite effect, a sudden drop in the rate of discharge with subsequent resetting of the firing rate, was observed in an 18-year-old boy with chronic complete AV block. Two mechanisms for this paradoxical response to exercise are proposed: (1) delay in discharge of the automatic pacemaker focus by successful penetration of electrotonic potentials from nonconducted P waves across the area of block in the AV conduction system and into the pacemaker focus; and (2) discharge of the pacemaker focus by concealed reentrant extrasystoles. Wedensky inhibition is ruled out because of the resetting of the firing rate.

Increased intensity of the murmur of hypertrophic obstructive cardiomyopathy with carotid sinus pressure.

In a prospective study murmurs increased in intensity with carotid sinus pressure in 18 of 26 patients with hypertrophic obstructive cardiomyopathy (HOCM) (sensitivity, 69.2 percent for the 26 patients, 85.7 percent for the 21 patients in whom heart rate and blood pressure decreased with carotid sinus pressure). On the other hand, the murmur remained constant or decreased in all but one of 104 patients with valvular aortic stenosis, mitral insufficiency, hypertrophic nonobstructive cardiomyopathy, and systolic murmurs of miscellaneous origins (specificity, 99 percent; positive predictive value, 94.7 percent). Catheterization, indirect arterial pressure tracings, and echocardiographic studies indicated that carotid sinus pressure-induced bradycardia was associated with increased left ventricular outflow tract obstruction. The carotid sinus pressure-induced increase in the murmur is probably multifactorial: decreased aortic pressure and impedance; increased contractility immediately on sudden slowing of heart rate; further increase in obstruction as the mitral valve systolic anterior movement is enhanced; and delayed vasodilatation maintaining the obstruction even after return of heart rate to precarotid sinus pressure values. An increase in a systolic murmur with carotid sinus pressure is characteristic of HOCM.

Prolonged hemodynamic effect of a slow-release nitroglycerin ointment.

Nitroglycerin (NTG) ointment has been shown to be effective in the treatment of angina pectoris and congestive heart failure. Its duration of action is usually 4 to 6 hours. This study presents data that show that a new slow-release NTG ointment produces hemodynamic improvement over at least 24 hours. Twenty patients with coronary artery disease were tested with serial gated equilibrium radionuclide ventriculography before and at various stages of continuous, once-a-day use of slow-release NTG ointment and 4 days after cessation of therapy. NTG ointment significantly (p less than 0.005) decreased left ventricular end-diastolic and end-systolic volumes both at rest (23% and 33%) and during handgrip exercise (22% and 32%) when examined after continuous usage of at least 24 hours. Ejection fraction increased 21% at rest, from 0.42 +/- 0.15 to 0.51 +/- -0.18, p less than 0.0005). The ratio of peak systolic pressure to end-systolic volume increased 85% at rest (p less than 0.05) and 54% during exercise (p less than 0.01). All values had returned to baseline 4 days after cessation of treatment. Thus, slow-release NTG ointment may be useful in the treatment of angina pectoris and congestive heart failure on a once-a-day basis.

Unusual QRS morphology associated with transvenous pacemakers. The pseudo RBBB pattern.

Eleven patients with implanted pervenous pacemakers were found to have unusual QRS morphology resembling right bundle branch block (RBBB) on the 12-lead electrocardiogram. In nine patients, the tip of the electrode catheter was established with certainty to be in the right ventricular apex. In eight of the nine, the standard leads showed a left bundle branch block pattern (LBBB), whereas only the precordial leads V1 and V2 resembled RBBB. In only one of the nine was the RBBB pattern also seen in the standard leads. In all nine, recording the precordial leads one intercostal space below the usual space eliminated the RBBB pattern in V1-V2 and resulted in inscription of a QS complex, whereas recording the leads one space higher than usual enhanced the height of the R wave. This is explained by the marked superior and slight anterior orientation of the main QRS complex in right ventricular pacing. It is suggested that the pattern of RBBB in V1-V2 + LBBB in lead 1 be named pseudo RBBB pattern since it does not represent left prior to right ventricular activation.