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Biomedical subjects

W Sherman

Publications and source records attributed to W Sherman.

At least 37 records · Page 2Linked to original sources
PubMed1992

A new transthyretin mutation associated with amyloid cardiomyopathy.

In transthyretin (TTR) a new mutation (TTR-Thr45) has been identified in a patient with familial amyloidosis characterized clinically by prominent cardiomyopathy and the absence of peripheral neuropathy. Comparative peptide mapping by high-performance liquid chromatography of the patient's plasma TTR together with normal TTR showed the presence of an abnormal tryptic peptide in the patient's TTR. The sequence of this peptide (peptide 6, residues 36-48) demonstrated the presence of a threonine-for-alanine substitution at position 45. This change can be explained by a single base change of adenine for guanine in the Ala-45 codon and was demonstrated directly by DNA sequence analysis of PCR-amplified exon 2 of the TTR gene; allele-specific oligonucleotide hybridization both in the patient and in fixed heart tissue from his aunt confirmed the base change. The TTR-Thr45 mutation is a new variant TTR found associated with cardiomyopathy.

Amino Acid Sequence↗
PubMed1991

Lymphoma, motor neuron diseases, and amyotrophic lateral sclerosis.

We studied 9 patients with motor neuron disease and lymphoma. The following several observations have not been recognized in the past: (1) Motor neuron syndromes are associated with either Hodgkin's disease or non-Hodgkin's lymphoma. (2) The syndromes are not restricted to lower motor neuron disorders; 8 of 9 patients had definite or probable upper motor neuron signs as well, qualifying for the diagnosis of amyotrophic lateral sclerosis. Corticospinal tracts were affected in both postmortem examinations. (3) The combination of motor neuron disease and lymphoma is often accompanied by paraproteinemia (3 of 7 patients studied), increased cerebrospinal fluid protein content (6 of 9 patients), and cerebrospinal fluid oligoclonal bands (3 of 9 patients). (4) In 2 patients, asymptomatic non-Hodgkin's lymphoma was found only because the discovery of paraproteinemia gave impetus to examine the bone marrow. (5) Patients with both upper and lower motor neuron signs (amyotrophic lateral sclerosis) may show physiological evidence of conduction block in peripheral nerves or autopsy abnormalities in peripheral nerves. The cause of this syndrome is not known. Both lymphoma and motor neuron disease could have a common cause, possibly a retroviral infection. The frequency of paraproteinemia suggests that an immunological disorder may play a role in the pathogenesis of the neurological disorder.

Aged↗
PubMed1991

Percutaneous coronary excimer laser-assisted balloon angioplasty: initial clinical and quantitative angiographic results in 50 patients.

The initial clinical experience and quantitative angiographic results of percutaneous coronary excimer laser-assisted balloon angioplasty are described for 55 lesions in 50 patients. With use of a xenon chloride (308 nm) excimer laser generator and 1.5 to 1.75 mm catheters, excimer laser angioplasty was attempted at 135 ns pulse width, 25 to 40 Hz repetition rate, 2 to 5 s laser delivery time and 30 to 60 mJ/mm2 energy fluence. Laser success (greater than 20% reduction in absolute percent diameter stenosis) was achieved in 41 (75%) of 55 lesions, with 100% subsequent balloon angioplasty success (less than 50% residual stenosis). By quantitative digital caliper technique, the percent diameter stenosis (mean +/- SE) was reduced from 81 +/- 1% to 50 +/- 3% after excimer laser angioplasty (p less than 0.001) and to 20 +/- 1% after balloon angioplasty (p less than 0.001); minimal luminal diameter increased from 0.56 +/- 0.04 to 1.46 +/- 0.08 mm (p less than 0.001) and 2.03 +/- 0.07 mm (p less than 0.001), respectively. By videodensitometric techniques, the percent area stenosis decreased from 86 +/- 2% to 54 +/- 3% after excimer angioplasty (p less than 0.001) and to 26 +/- 3% after balloon angioplasty (p less than 0.001). There were no perforations, need for emergency bypass surgery or deaths. The overall incidence of abrupt closure (3.6%), dissection (1.8%), embolization (1.8%), filling defect (6%), myocardial infarction (5.5%), side branch occlusion (3.6%) or spasm (3.6%) was infrequent and more related to subsequent balloon angioplasty than to the laser procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

Angioplasty, Laser↗
PubMed1990

Aortic valve tumor: an unusual cause of coronary obstruction during cardiac catheterization.

Coronary artery obstruction during cardiac catheterization is a rare but serious complication that has been reported to occur in 0.15 to 0.5% of cases. The most common causes of intraprocedural coronary occlusion include thromboembolism, air embolism, and coronary dissection. This report describes the angiographic findings of a patient who developed chest pain with electrocardiographic evidence of myocardial ischemia due to obstruction of the right coronary artery by an aortic valve tumor.

Aortic Valve↗
PubMed1990

Angiographic patterns of balloon inflation during percutaneous transluminal coronary angioplasty: role of pressure-diameter curves in studying distensibility and elasticity of the stenotic lesion and the mechanism of dilation.

There are few in vivo data concerning the mechanisms of balloon inflation during coronary angioplasty. To characterize how lesions dilate, videodensitometry was used to measure the diameter of the inflated balloon across 29 coronary lesions in 27 patients. Pressure-diameter curves for each lesion were derived with use of a standardized incremental inflation protocol in which pressures between 2 and 6 atm in 3 mm low profile balloons approximated normal vessel diameter. The diameter of coronary stenosis before and after angioplasty was also measured. Pressure-diameter curves showed that the most improvement in luminal caliber occurred at low inflation pressure. A distensibility factor was defined as the ratio of the amount of balloon inflation at 2 atm compared with the balloon diameter at 6 atm. Eccentric irregular lesions (n = 11) had a greater distensibility factor (0.49 +/- 0.17) than did lesions (n = 18) without this configuration (0.33 +/- 0.14) (p less than 0.02). The former were soft, presumably because of thrombus in these lesions. In addition, there were no differences in patterns of balloon inflation for lesions requiring additional inflation or for dilations resulting in an intimal crack or dissection after angioplasty. There was often a loss of luminal caliber when balloon diameter at 6 atm was compared with the diameter after angioplasty. This was defined as elasticity or recoil. There was a significant direct correlation between the amount of elasticity and the extent of balloon inflation at 6 atm (that is, lesions more fully dilated at 6 atm showed more elasticity).(ABSTRACT TRUNCATED AT 250 WORDS)

Angiography↗
PubMed1990

Cardiac amyloidosis: report of a patient heterozygous for the transthyretin isoleucine 122 variant.

Amyloid fibril material was extracted from autopsy material of a patient who died from progressive cardiac failure at age 64. He had enlarged heart on routine X-ray at age 47 and the first symptoms of cardiac failure at age 62. Fractionation of the fibril material resolved peptide fragments immunoreactive with anti-human transthyretin (TTR). One of the peptides was further purified by high-performance liquid chromatography (HPLC) and subjected to tryptic peptide mapping along with TTR isolated from the patient's serum. In both instances, sequencing procedures revealed, in addition to the normal peptide 12 (residues 105-126), an abnormal peptide with an isoleucine for valine substitution at position 122. This substitution has been described previously in a patient with systemic senile amyloidosis (SSA) homozygous for this variant. The results question whether SSA is a clinical entity related to TTR Ile 122 with phenotypic expression in the homozygous condition.

Amino Acid Sequence↗
PubMed1990

What happens to patients after five years of intensive case management stops?

Seventy-two patients who received five years of intensive case management services were transferred into mainstream community mental health center services with a much higher patient-to-staff ratio. At the end of a two-year follow-up, 91 percent of the patients were still receiving treatment. Compared with the previous five years, hospitalizations during the follow-up period increased, but not significantly so. Contacts with mainstream CMHC services increased significantly. Overall costs in constant 1979 dollars showed a nonsignificant decrease, dropping by about $1,500 per patient per year. The staff time and resources gained by the programmatic changes were used to treat a larger number of chronic patients seeking services.

Adult↗
PubMed1990

Motor neuron disease and amyotrophic lateral sclerosis: relation of high CSF protein content to paraproteinemia and clinical syndromes.

From 1984 to 1988, 11 of 120 patients (9%) with motor neuron disease (MND) had paraproteins detected by serum immunofixation electrophoresis (IFE), compared with 4 (3%) by cellulose acetate gels: 1 patient had progressive spinal muscular atrophy, 5 patients had amyotrophic lateral sclerosis (ALS), and 5 patients had ALS with probable upper motor neuron signs. Four of 5 patients (80%) with cerebrospinal fluid (CSF) protein content above 75 mg/dl had paraproteins, as did 6 of 30 with values above 50 mg/dl. Four of 14 patients with cerebrospinal oligoclonal bands (OCB) also had paraproteins. Two patients with ALS, CSF protein content above 75 mg/dl, and paraproteinemia had lymphoma. We conclude the following about patients with MND: high CSF protein content (especially above 75 mg/dl) or CSF OCB makes paraproteinemia more likely; some of these patients may have lymphoma; there is an inordinately high occurrence of paraproteinemia in MND; and IFE on agarose is more sensitive than electrophoresis on cellulose acetate in detecting paraproteins. Syndromes of paraproteinemia and high CSF protein are not restricted to the lower motor neuron but qualify as "ALS" with coexisting upper motor neuron signs.

Adult↗
PubMed1989

Balloon valvuloplasty in adult aortic stenosis: determinants of clinical outcome.

STUDY OBJECTIVE: To determine the clinical variables affecting outcome after balloon aortic valvuloplasty. DESIGN: Longitudinal follow-up of consecutive case series. PATIENTS: Consecutive sample of 36 patients with severe calcific aortic stenosis, and without active infection or left ventricular mural thrombus. INTERVENTIONS: Percutaneous transluminal dilatation of the aortic valve until the peak gradient was reduced by 50% or a maximal balloon size was used. Hemodynamic measurements taken before and after dilatation. MEASUREMENTS AND MAIN RESULTS: Thirty-three patients had a successful dilatation. Eighty-nine percent (95% confidence interval [CI]. 74% to 97%) improved symptomatically at 2 weeks, but by 26 weeks only 56% (CI, 35% to 76%) remained improved (P = 0.0078). Mortality rates were high at 8 (9%) and 26 (28%) weeks. Predictors of adverse events included left ventricular ejection fraction (P = 0.04, r = 0.46), pulmonary artery systolic pressure (P = 0.048, r = 0.65), pulmonary vascular resistance (P = 0.008, r = 0.69), and right ventricular end-diastolic pressure (P = 0.009, r = 0.43) at 8 weeks and all these factors except left ventricular ejection fraction at 26 weeks. These clinical outcomes were unrelated to other cardiac or pulmonary diseases. CONCLUSIONS: Symptomatic improvement is only temporary in many patients undergoing balloon aortic valvuloplasty, and the mortality rate in the mid-term follow-up period is high. Valve surgery remains the treatment of choice for aortic stenosis in the adult.

Aged↗
PubMed1989

Late thrombolytic therapy preserves left ventricular function in patients with collateralized total coronary occlusion: primary end point findings of the Second Mount Sinai-New York University Reperfusion Trial.

The change in left ventricular ejection fraction from preintervention to predischarge was prospectively assessed in 393 patients with acute myocardial infarction. Within 12 h of symptom onset (mean 6.3 +/- 2.7 h), patients were randomly assigned to a double-blind intracoronary infusion of streptokinase, nitroglycerin, both streptokinase and nitroglycerin or conventional therapy without acute cardiac catheterization. Treatment effects were also assessed in prospectively defined angiographic subsets. There was a significant interaction between streptokinase and nitroglycerin (p less than 0.01), resulting in an increase in ejection fraction of 3.9 percentage units in the combined treatment arm (p less than 0.001). Patients with collateral flow to a totally obstructed infarct-related artery showed a significant improvement over those without collateral flow in the streptokinase (5.4 +/- 2.5%) and streptokinase-nitroglycerin (10.6 +/- 2.7%) arms, but not in the nitroglycerin arm. Time to treatment did not influence the change in ejection fraction. In patients with initial subtotal occlusion, thrombolytic therapy was of no short-term benefit because ejection fraction increased by 6% in all three intervention arms. These findings indicate that relatively late thrombolytic therapy results in significant myocardial salvage in those patients with collateralized total coronary occlusion. This benefit is potentiated by concomitant nitroglycerin therapy.

Aged↗
PubMed1989

Determinants of collateral filling observed during sudden controlled coronary artery occlusion in human subjects.

Higher grades of collateral circulation limit the extent of myocardial ischemia observed during balloon inflation in patients with single vessel coronary disease undergoing coronary angioplasty. However, the grade of collateral filling during sudden coronary occlusion varies from patient to patient. To assess which characteristics may predict a high grade of collateral filling, baseline clinical and angiographic variables were correlated with the grade of filling during coronary occlusion in 67 patients (whose angina ranged from 1 week to 36 months in duration) undergoing left anterior descending or right coronary artery angioplasty. A second contralateral arterial catheter was used to assess the collateral filling that reached the vessel dilated before and during transient total occlusion by the angioplasty balloon. Thirty-six patients had a proximal stenotic lesion ranging in severity from 65 to 99%. On a 0 to 3 scale, mean collateral filling grade before inflation was 0.4 versus 1.8 during inflation (p = 0.001). All 19 patients with 95 to 99% stenosis had at least grade 2 collateral filling during inflation. In contrast, 18 of 21 patients with less than or equal to 80% stenosis had only grade 0 or 1 collateral filling during inflation. There were significant positive correlations between collateral grade during inflation and 1) baseline lesion severity (r = 0.76), 2) baseline collateral filling grade (r = 0.50), and 3) vessel dilated. There was no relation between collateral filling during inflation and age, gender, risk factors, duration of angina or proximal versus distal location of the lesion. Lesion severity was the only independent variable associated with collateral filling grade.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

Serial angiographic assessment of coronary artery obstruction and collateral flow in acute myocardial infarction. Report from the second Mount Sinai-New York University Reperfusion Trial.

In the Second Mt. Sinai-New York University Reperfusion Trial, in which change of ejection fraction was the primary end point, the following secondary end points were prospectively assessed by serial coronary angiography: patency of the infarct artery both before intervention and 10-14 days later, acute and delayed recanalization rates, presence or absence of collateral flow, and complication rates of acute interventional catheterization. We assigned 393 patients randomly to groups receiving acute cardiac catheterization and a double-blind intracoronary infusion of streptokinase (SK arm), both streptokinase and nitroglycerin (SK-NTG arm), nitroglycerin alone (NTG arm), or conventional therapy without acute catheterization (control arm). Prospective stratification was based on duration of infarct pain before randomization: group A, less than 2 hours; Group B, 2-12 hours. Baseline patency rates were comparable in patients studied within 6 hours (30%, 40 of 135) and those studied later (24%, 32 of 133). This finding refutes the hypothesis that spontaneous recanalization occurs frequently after 6 hours. The acute recanalization rates of the SK arm (60%, 40 of 67) and the SK-NTG arm (63%, 29 of 62) did not differ. During streptokinase infusion, more vessels recanalized in group A (81%, 22 of 27) than in group B (56%, 57 of 102) (p less than 0.01); this was due to a significant reduction of recanalization rates from 75% (48 of 64) to 45% (26 of 62) with treatment after 6 hours (p less than 0.01). Delayed recanalization, that is, patency at end point but not postintervention, was seen in 17% (17 of 100) of total occlusions treated with streptokinase. In group A, all total occlusions treated with streptokinase recanalized either acutely (20 of 22) or delayed (two of 22), whereas in group B, 23% (18 of 78) remained obstructed. The reocclusion rate in the SK arms was 17% (11 of 65). In the NTG arm, recanalization occurred during intervention in 4% (two of 47) and delayed in 45% (21 of 47). At end-point angiography, the patency rates of the NTG arm (62%, 41 of 66) and the control arm (58%, 36 of 62) were comparable; those of the SK arms were higher (75%, 105 of 140) (p less than 0.01). Total occlusion was associated with collateral flow in 33% (66 of 199) at baseline; the prevalence of collaterals did not increase with time to angiography, which indicates that they had developed before the index event.(ABSTRACT TRUNCATED AT 400 WORDS)

Angiography↗
PubMed1988

Precordial T-wave polarity changes in acute inferior infarction. A poor indicator of associated disease of the left anterior descending coronary artery.

T-wave polarity in V1 to V3 was examined in 70 patients with acute inferior wall myocardial infarctions (IWMI) as an indicator of significant disease in the left anterior descending coronary artery (LAD). Fifty-three percent (37/70) had precordial T-wave inversions, and of these 38 percent (14/37) had angiographically significant LAD disease. Conversely, 47 percent (33/70) had upright T waves in V1 to V3, and of these, 52 percent (17/33) had significant LAD disease. Likewise, 41 percent of the patients with ST segment depression in V1 to V3 had significant LAD disease, while 50 percent of those with isoelectric ST segments were similarly affected. Finally only 39 percent of those patients with both precordial ST segment depression and T-wave inversion had significant LAD disease. We conclude that neither anterior ST depression, T-wave inversion, nor a combination of both is a sensitive or specific indicator of LAD disease in patients with acute IWMI.

Aged↗