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Biomedical subjects

J D Stephens

Publications and source records attributed to J D Stephens.

At least 37 records · Page 2Linked to original sources

Management of the fetus with an abdominal wall defect.

Eleven fetuses with omphalocele and two with gastroschisis were diagnosed by prenatal ultrasonographic examination. Intact omphalocele was accurately distinguished from gastroschisis by detection of a membranous sac covering the herniated viscera, by liver protruding from the abdomen, and by the frequent association of major structural anomalies. Gastroschisis was characterized by the absence of these findings and the presence of bowel loops floating freely in the amniotic fluid. Nine of eleven fetuses with omphaloceles had other major malformations and did not survive. Two small omphaloceles and two gastroschises were correctly diagnosed as isolated defects, free from associated anomalies. All four underwent maternal transport and were successfully managed with good outcome. Prenatal ultrasonography allows rational decisions about perinatal management by distinguishing between omphalocele and gastroschisis and by screening for associated anatomic defects. Karyotype analysis should be a part of the prenatal workup. Most isolated defects can be followed and delivered normally near term. Accurate prenatal diagnosis allows maternal transport and in a few cases may alter the timing or mode of delivery.

Adolescent↗

Prenatal diagnosis of pseudo arylsulphatase A deficiency.

Prenatal diagnosis was performed on a pregnancy at risk for metachromatic leukodystrophy (MLD) in a family with the pseudo arylsulphatase A deficiency trait. Extracts of cultured amniotic fluid cells were deficient in arylsulphatase A indicating that the fetus was either affected with MLD or had the benign pseudodeficiency trait. In the cerebroside sulphate loading test, the at risk cells hydrolysed sulphatide like control cultured amniotic fluid cells implying that the fetus had pseudodeficiency. The pregnancy was carried to term and a male child was delivered. Placenta, urine and fibroblasts had very low activities of arysulphatase A. However, no sulphatide could be detected in urine and growing fibroblasts responded normally in the cerebroside sulphate loading test, suggesting pseudodeficiency. At 29 months, the infant is healthy and shows no stigmata of MLD. The prediction based on the results of the cerebroside sulphate loading test on cultured amniotic fluid cells appeared to be borne out.

Amniotic Fluid↗

Coronary haemodynamic effects of nifedipine. Comparison with glyceryl trinitrate.

The coronary haemodynamic effects of nifedipine and glyceryl trinitrate were compared in 22 patients undergoing investigations for suspected coronary artery disease. Myocardial blood flow was estimated by the coronary sinus thermodilution technique. In sinus rhythm nifedipine increased mean coronary sinus flow from 135 ml/min to 152 ml/min, and reduced arterio-coronary sinus oxygen difference from 12.4 to 10.96 ml/100 ml without causing a significant change in coronary vascular resistance or in myocardial oxygen consumption. Glyceryl trinitrate reduced mean coronary sinus flow from 165 to 111 ml/min, myocardial oxygen consumption from 19.2 to 11.9 ml/min, and arterio-coronary sinus oxygen difference from 11.7 to 10.9 ml/100 ml. There was a rise in coronary vascular resistance from 54 355 to 74 364 dynes s cm-5. During atrial pacing nifedipine reduced the arterio-coronary sinus oxygen difference from 11.99 to 11.0 ml/100 ml but had no significant effect on the other variables measured. Glyceryl trinitrate caused a fall in mean coronary sinus flow from 207 ml/min to 168 ml/min; myocardial oxygen consumption fell from 24 ml/min to 18 ml/min, while coronary vascular resistance rose from 41 714 to 51 234 dynes s cm-5. Direct comparison of the two drugs showed a significant difference in effects on coronary sinus flow and coronary vascular resistance in sinus rhythm. Both drugs appeared effective in relieving ischaemia as judged by a reduction of the incidence of pacing induced angina and an improvement in lactate status.

Blood Pressure↗

Rh isoimmunization following genetic amniocentesis.

A series of 8009 genetic amniocenteses were retrospectively examined to evaluate the relationship of the procedure to Rh isoimmunization. Of the 615 Rh negative women giving birth to Rh positive infants and estimated to be at risk, thirteen (2.1 per cent) were sensitized subsequently to the amniocentesis. Eleven of the sensitizations occurred early in the programs, and a combination of experience and ultrasound performed concurrently with the amniocentesis appear to have reduced the risk of isoimmunization to that of control data from the literature.

Amniocentesis↗

Combined exercise radionuclide and hemodynamic evaluation of left ventricular aneurysmectomy.

Twelve patients were studied by rest and exercise radionuclide ventriculography following left ventricular aneurysmectomy (LVA). Left ventricular filling pressure (LVFP) was also measured. Nine patients had been studied pre-LVA at rest and exercise before and after isosorbide dinitrate (ISDN). Resting ejection fraction (LVEF) improved after LVA (p less than 0.25), but exercise LVEF did not. End-diastolic volume (EDV) and resting LVFP also fell after LVA (p less than 0.25 for EDV, p less than 0.05 for LVFP) and although exercise LVFP fell (p less than 0.02), the values were abnormal in all patients. Ejection fraction of contractile segment (EFCS) from the resting radionuclide study pre-LVA was related to resting LVEF post-LVA (r = 0.71 p less than 0.02), although postoperative LVEF could not be predicted from preoperative EFCS in individual patients. Deterioration in LVEF and LVFP from rest to exercise post-LVA occurred both in patients with single-vessel occlusion and in those with multivessel coronary disease, irrespective of whether or not revascularization had been performed. Thus LVA is effective in improving resting ventricular function; exercise performance may remain abnormal even in patients without residual coronary disease.

Adult↗

Haemodynamic effects of oral salbutamol alone and in combination with sublingual isosorbide dinitrate in patients with severe congestive cardiac failure.

To examine possible augmentation of the effects of isosorbide dinitrate by salbutamol, haemodynamic measurements were made in 10 patients with severe chronic congestive cardiac failure who received isosorbide dinitrate 2.5 to 25 mg sublingually and salbutamol 4 to 12 mg orally, alone and in combination. Isosorbide dinitrate reduced mean left ventricular filling pressure from 29 to 18 mmHg and increased mean cardiac index from 1.7 to 2.0 1/min per m2, with no significant change in mean heart rate. Systemic arterial mean pressure fell from 85 to 72 mmHg. Salbutamol increased cardiac index from 1.8 to 2.2 1/min per m2. There was no significant change in left ventricular filling pressure, heart rate, or systemic arterial pressure. Compared with control, combined isosorbide dinitrate and salbutamol reduced left ventricular filling pressure from 27 to 19 mmHg and increased cardiac index from 1.8 to 2.7 1/min per m2, with no significant change in heart rate. Systemic arterial pressure fell from 82 to 75 mmHg. The reduction in left ventricular filling pressure by combined treatment was similar to that produced by isosorbide dinitrate alone, but the increase in cardiac index was significantly greater than that produced either by isosorbide dinitrate alone or salbutamol alone. Combined sublingual isosorbide dinitrate and oral salbutamol have an additive effect in improving left ventricular performance in patients with severe chronic congestive cardiac failure.

Aged↗

Radionuclide and hemodynamic assessment of left ventricular functional reserve in patients with left ventricular aneurysm and congestive cardiac failure. Response to exercise stress and isosorbide dinitrate.

The hemodynamic response to exercise stress was assessed in 17 patients with left ventricular (LV) aneurysm, demonstrated by contrast left ventriculography, and congestive cardiac failure (CCF) before and after sublingual isosorbide dinitrate (ISDN). Radionuclide ventriculography was performed at rest and during exercise in 14 patients. ISDN increased mean exercise LV stroke work index (LVSWI) from 31 to 39 g-m/m2 (p less than 0.001) and reduced mean exercise LV filling pressure from 38 to 25 mm Hg (p less than 0.001). Using the combined criteria of LVSWI and ejection fraction of the contractile section of the left ventricle (EFCS), no patient with rest EFCS of less than 40% achieved a good absolute response to exercise in LV performance with or without ISDN. Of the six patients with rest EFCS greater than or equal to 40% only three achieved a good response. While rest EFCS less than 40% indicates poor LV functional reserve, good LV functional reserve is not always indicated by rest EFCS greater than or equal to 40%.

Adult↗

In utero paternity testing utilizing fetal blood obtained by midtrimester fetoscopy.

To evaluate paternity in a case where both rape and conjugal coitus had occurred close to the time of conception, fetoscopy with fetal blood sampling was performed at 20 weeks gestation. Detailed blood group typing of the wife, husband, and fetus, and the presence of a very long Y chromosome in the last two, indicated a 99.9% chance that the fetus was fathered by the husband and only a 0.1% chance that it was fathered by "some other male Caucasian." The couple elected to continue the pregnancy. Neonatal testing verified the prenatal findings.

Adult↗