Primary sclerosing cholangitis.
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Cholangitis, Sclerosing↗
Biomedical subjects
S C Robson
Publications and source records attributed to S C Robson.
Non-cirrhotic portal hypertension--a new entity in South Africa? A report of 6 cases.
Six patients with portal hypertension and well-preserved liver function as well as classic features of non-cirrhotic portal hypertension on histological examination of biopsy specimens are described. Three of these patients also had extrahepatic portal vein occlusion. All patients had varying degrees of portal hypertension and hypersplenism. Three patients underwent splenectomy and the remainder had sclerotherapy to control variceal bleeding. The overall prognosis is excellent if variceal bleeding can be controlled by appropriate measures. Hence recognition of this infrequently diagnosed entity is important for an accurate prognosis and appropriate therapeutic interventions.
Adult↗
Serial changes in pulmonary haemodynamics during human pregnancy: a non-invasive study using Doppler echocardiography.
1. Serial pulmonary haemodynamic investigations were performed in 13 women before conception, at monthly intervals throughout pregnancy, and then at 6 months after delivery. 2. Mean pulmonary artery pressure was calculated from pulsed Doppler pulmonary velocities. Pulmonary flow was measured by Doppler and cross-sectional echocardiography. These two measurements were used to calculate pulmonary vascular resistance. 3. Mean non-pregnant pulmonary artery pressure was 13.8 mmHg and no significant change was demonstrated during pregnancy. 4. Pulmonary flow increased from 4.88 to 7.19 litres/min during pregnancy. 5. Pulmonary vascular resistance decreased from 2.85 resistance units before pregnancy to 2.17 resistance units at 8 weeks gestation. Thereafter there was no further significant change, values returning to pre-pregnant levels by 6 months after delivery.
Adult↗
Primary biliary cirrhosis. A retrospective survey at Groote Schuur Hospital, Cape Town.
Primary biliary cirrhosis (PBC) or chronic non-suppurative destructive cholangiohepatitis is rare in southern Africa. Eight patients with this diagnosis were identified and fully investigated at Groote Schuur Hospital between 1980 and 1988. Seven patients were female, all were white or coloured, and their ages ranged from 49 years to 80 years. All patients presented with a history of malaise, fatigue, night sweats and pruritus, which had been present for 3 months--12 years before diagnosis of PBC. Initial misdiagnosis had resulted in unnecessary invasive investigations including laparotomies. Signs of chronic liver disease, such as xantholasmas, evidence of pruritus, the sicca syndrome or hepatomegaly, were invariably present. Marked elevation of serum alkaline phosphatase level and IgM were present in all cases. Antimitochondrial antibodies were positive in significant titre in 7 of the 8 patients. Liver biopsies demonstrated stage II-III disease in all patients. Therapy was chiefly supportive and symptomatic although most patients received immunosuppressive agents. Despite the late presentation, the subsequent course was similar to that seen elsewhere where patients are recognised earlier.
Aged↗
Retrospective survey of drug-induced liver disease at Groote Schuur Hospital, Cape Town--1983-1987.
In a survey of patients admitted to the medical and surgical wards of Groote Schuur Hospital during the 5-year period 1983-1987 38 patients with severe drug-induced hepatitis were identified. Fifty-three per cent of these reactions were caused by anti-tuberculosis drugs, 21% to phenytoin and 11% to methyldopa. Whereas 82% of the patients were jaundiced, only one-third had gastro-intestinal symptoms and/or fever and only 24% had a rash. Twenty-six per cent of patients were encephalopathic on admission. The overall mortality rate was 24%. Forty per cent of patients with hepatitis caused by anti-tuberculosis therapy died. Many patients had continued to receive therapy despite signs of liver disease. These findings underline the need for a high index of suspicion in the diagnosis of drug-induced liver disease and for early withdrawal of the offending agent(s).
Adolescent↗
Apparent liver blood flow during pregnancy: a serial study using indocyanine green clearance.
Serial measurements of apparent liver blood flow and cardiac output were performed in 12 women at 12-14, 24-26 and 36-38 weeks of pregnancy and then at 10-12 weeks after delivery. Apparent liver blood flow was calculated from indocyanine green clearance. Cardiac output was measured by Doppler and cross-sectional echocardiography at the aortic valve. Postnatal apparent liver blood flow was 1.81 l/min and no significant change was demonstrated during pregnancy. In contrast cardiac output decreased from 7.46 l/min at 36-38 weeks gestation to 4.90 l/min after delivery. Apparent liver blood flow accounted for 24% of cardiac output during pregnancy. This increased to 37% after delivery.
Adult↗
Common variable immunodeficiency in association with Turner's syndrome.
Common variable immunodeficiency is a heterogeneous syndrome which may occur at any age and may be associated with recurrent sinopulmonary and gastro-intestinal infections, atopic illness, autoimmune disorders and varying degrees of hypogammaglobulinaemia (1). The clinical syndrome is very similar to that described in X-linked agamma-globulinaemia but the mode of inheritance is unknown (2). In this communication, a patient with Turner's syndrome with X-isoX chromosomal pattern in conjunction with common variable immunodeficiency is reported.
Adolescent↗
Monocyte-macrophage release of IL-1 is inhibited by type-1 plasminogen activator inhibitors.
Interleukin-1 (IL-1) release from monocyte-macrophages (Mo) appears dependent on pericellular proteolysis mediated by plasmin. Thus plasminogen activator inhibitors (PAI) which bind the serine proteases responsible for the conversion of plasminogen to plasmin, may inhibit IL-1 release from Mo. We have examined the effect of purified PAI from a hepatoma cell line Hep G2, on IL-1 release from Mo with secondary effects on lymphocyte proliferation in vitro. Fast acting inhibitors of both urokinase (u-PA) and tissue plasminogen activator (two chain t-PA) were noted in harvest fluids of Hep G2 cells. These inhibitors were stable at pH 3 but lost activity at 45 degrees C. They were SDS-stable and migrated with Mr53 and 104 kDa. These properties conformed to characteristics of type-1 plasminogen activator inhibitor (PAI-1). Partially purified PAI-1 added to human Mo cultured on 125I fibrin layer both in the presence and absence of plasminogen inhibited secretion of IL-1 by Mo in response to LPS. This effect, however, did not correlate with the inhibition of plasminogen dependent fibrinolysis. This suggested a degree of sequestration and inaccessibility of membrane bound u-PA of LPS activated Mo to PAI-1. PAI-1, in addition, inhibited mitogen stimulated peripheral blood mononuclear cell (PBMC) proliferation at similar concentration ranges. This effect was abrogated by the addition of specific antisera to PAI-1. PAI-1 may be released as part of an acute phase response. In addition to influencing fibrinolysis, PAI-1 may constitute a negative feedback pathway on Mo IL-1 release and subsequent immune activation in vivo.
Acute-Phase Reaction↗
Streptococcus bovis bacteremia and endocarditis at Groote Schuur Hospital.
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Adolescent↗
The prevalence of hepatitis B virus in southern Africa.
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Africa, Southern↗
Hemodynamic changes during twin pregnancy. A Doppler and M-mode echocardiographic study.
Serial hemodynamic investigations were performed in 10 women with twin pregnancies at 20, 24, 28, 32, and 36 weeks' gestation and at 6 months after delivery. Cardiac output was measured by Doppler and cross-sectional echocardiography at the aortic, pulmonary, and mitral valves. Cardiac chamber size and ventricular function were investigated by M-mode echocardiography. The hemodynamic results were compared with those of 13 women with singleton pregnancies. Mean Doppler cardiac output was increased at 20 weeks of twin pregnancy (7.63 L/min) and showed no significant change during the remainder of pregnancy, but fell to 5.07 L/min after delivery. This increase was significantly greater than that recorded during singleton pregnancy, because of a relatively greater increase in heart rate. Twin pregnancy was associated with a significantly greater increase in left atrial dimension, but the increases in left ventricular dimensions, wall thickness, and function were comparable to those recorded in singleton pregnancy.
Cardiac Output↗
Haemodynamic changes associated with caesarean section under epidural anaesthesia.
Serial haemodynamic investigations were performed in 15 women delivered by elective caesarean section under epidural anaesthesia at 38-40 weeks gestation. Cardiac output was measured by Doppler and cross-sectional echocardiography at the aortic valve. No haemodynamic changes were demonstrable after attainment of surgical anaesthesia (T5 or above). Stroke volume increased 13% after delivery of the placenta and remained elevated until the end of the operation at which time cardiac output was 11% above pre-operative values. Stroke volume and cardiac output fell during the first postoperative day. Heart rate remained elevated at pre-operative values for 48 h after delivery. There was a fall in heart rate and cardiac output between the second and the sixth days after delivery. By 2 weeks after delivery cardiac output was 28% lower than pre-operative values. Compared with pre-operative values, diastolic blood pressure was lower on the first and second postnatal days.
Adult↗
Haemodynamic effects of breast-feeding.
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Adult↗
Isometric exercise in the denervated heart: a Doppler echocardiographic study.
The haemodynamic responses to isometric exercise of eight recipients of orthotopic heart transplants and eight healthy controls were studied. Each performed sustained exercise at 30% of maximal voluntary contraction for three minutes on a handgrip dynamometer. Cardiac output was measured by combined Doppler and cross sectional echocardiography before exercise and every 30 seconds during and after exercise. In the controls cardiac output and blood pressure increased significantly owing to an increase in heart rate with no change in stroke volume. In the transplant group cardiac output, heart rate, and stroke volume remained unchanged throughout exercise. In contrast with its response to dynamic exercise the denervated human heart is unable to increase cardiac output during isometric exercise. The pressor response that occurs is mediated via an increase in peripheral vascular resistance.
Adult↗
Serial study of factors influencing changes in cardiac output during human pregnancy.
Serial hemodynamic measurements were performed in 13 women on two occasions before conception and then at monthly intervals throughout pregnancy. Cardiac output (CO) was measured by Doppler and cross-sectional echocardiography at the aortic, pulmonary, and mitral valves. Cardiac chamber size and ventricular function were investigated by M-mode echocardiography. CO increased from a mean of 4.88 l/min before the conception to a maximum of 7.21 l/min at 32 wk, the increase being significant by 5 wk after the last menstrual period. Heart rate and left ventricular performance increased during the first trimester. Heart rate increased further during the second trimester, during which left atrial and left ventricular end-diastolic dimensions increased, suggesting an increase in venous return. Derived values of total peripheral vascular resistance fell during the first 20 wk. These changes were associated with a progressive increase in valve orifice area and left ventricular wall thickness during pregnancy.
Adult↗
Maternal hemodynamics after normal delivery and delivery complicated by postpartum hemorrhage.
Serial hemodynamic investigations were performed in 40 women at 38 weeks' gestation and then 1, 2, 6, 10, and 14 days after normal delivery. Cardiac output was measured by Doppler and cross-sectional echocardiography at the aortic valve. In 30 controls who received an average of 280 mL intravenous fluid during labor, the mean cardiac output remained elevated (7.15 L/minute) for 24 hours after delivery and then fell to 5.39 L/minute at 10 days as a result of decreases in both heart rate and stroke volume. Blood pressure fell during the first 2 days after delivery and thereafter increased to values not significantly different from those recorded at the end of pregnancy. In ten women who experienced postpartum hemorrhage, stroke volume decreased and heart rate increased, relative to the control group, during the first 48 hours after delivery. Blood pressure and cardiac output, however, were not significantly different from values in the control group.
Adult↗
Doppler echocardiographic estimation of cardiac output: analysis of temporal variability.
The temporal variability of combined cross-sectional and Doppler echocardiographic estimates of cardiac output was studied in 14 normal subjects. In each subject cross-sectional echocardiographs and Doppler velocities were recorded from the aortic, pulmonary and mitral valves. Recordings were repeated after 30-60 min and after 1-3 months to allow estimation of short-term and long-term temporal variability. A components of variance analysis showed that between-subject variability was significantly larger than within-subject variability for all measured and calculated variables. Long-term variability was larger than short-term variability for all variables except the mitral valve area. Calculation of flows from the three measurement sites were equally reproducible and the mean percentage error for CO measurements performed 1-3 months apart ranged from 6.4% to 7.4%. The 95% confidence intervals for Doppler measurements of CO at the three sites ranged from +/- 0.49 l min-1 to +/- 0.56 l min-1, suggesting that the temporal variability of flow measurements using the non-invasive Doppler method is sufficiently small for the method to be useful in serial haemodynamic studies.
Adult↗