Biomedical subjects
Publications and source records attributed to C M Bijleveld.
Four cases of extrahepatic biliary atresia are reported. Each of these infants presented with haemorrhagic phenomena rather than with prolonged jaundice. The increased bleeding tendency was due to a vitamin K deficiency, probably caused by cholestasis-induced malabsorption. Therefore extrahepatic biliary atresia should be considered in each infant with a bleeding diathesis.
An adapted cow's milk infant formula without or with extra taurine (350 mumol/l) was fed to four and five infants, respectively. The infants, born after 28-32 weeks gestation, and initially fed with a starting formula for preterms, were switched to one of the two above-mentioned formulae at approximately the 16th day of life. Each infant was studied during 4 consecutive weeks. The faecal excretion of fat, energy and total bile acids was determined from 3-day stool collections each week. The addition of taurine to the infant formula neither improved the uptake of fat and energy nor changed the faecal bile acid excretion. Growth velocity was similar in both groups of infants. Based on these results there is no rationale for adding taurine to adapted cow's milk infant formula to obtain a better fat absorption.
We performed a prospective study on 375 patients with liver disease, 60% female, for whom orthotopic liver transplantation (OLT) was considered during 1977-1985. Fifty-four per cent had cirrhosis, 8.5% congenital/hereditary disorders, 25% malignant tumour, 6% benign tumour, 2% Budd-Chiari syndrome, 1.5% acute hepatic failure, 3% other diagnoses, and 10% were under 15 years of age. As of July 1st, 1985, 99 patients (47 chronic active/inactive cirrhosis (CAC/CIC), 28 primary biliary cirrhosis (PBC), five hepatocellular carcinoma (HCC), 19 other diagnoses) were accepted for OLT (median age 40 years, 10% under age 15). By that date, 45 patients (median age 42), had had an OLT (20 CAC/CIC, 15 PBC, three biliary atresia, two HCC, five other diagnoses). Fifty-four per cent (201 patients) were rejected for transplantation. The primary reasons for rejection were: no indication (11%), age (5%), other surgical procedures possible (3%), severe liver failure (14%), extrahepatic spread of liver tumour (11%), cardiovascular or pulmonary problems (2%), severe hepatic bone disease (1%), and miscellaneous (7%). Thirty per cent of the patients with CAC/CIC, 38% with PBC, 88% with HCC and 71% with biliary atresia were rejected. In the CAC/CIC, PBC and biliary atresia patients severe liver failure was the most frequent reason for rejection (62%, 50% and 60%, respectively). In HCC, extrahepatic tumour spread was the most frequent reason (72%) for rejection. In this category only two patients (7%) ultimately underwent liver transplantation.
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The daytime breath hydrogen profile (DBHP) enables the study of breath hydrogen (BH) excretion in children under normal dietary and environmental circumstances. We studied the DBHP in 43 children with abdominal pain and (or) diarrhoea in order to evaluate its use in the detection of carbohydrate malabsorption (CHM). The results were compared to those of the lactose BH test. The DBHP was abnormal in 16 patients (37%), 8 of whom also had an abnormal lactose BH test. Five other patients with an abnormal lactose BH test had a normal DBHP. In 7 out of 10 children with an abnormal DBHP, the recorded abdominal symptoms coincided with a sharp increase in BH excretion. Abnormal DBHPs were most frequently found in children with functional abdominal complaints and with giardiasis. Our findings indicate that CHM is more frequently encountered in children with abdominal symptoms than can be detected by the lactose BH test. The DBHP offers new possibilities in the investigation of gastrointestinal conditions by correlating the symptoms directly to the effect induced by CHM.
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A method has been developed for easy sampling of duodenal bile acids. For this purpose Entero-Test was used, an encapsulated nylon thread originally used to estimate enteral parasites. This capsule is swallowed by a fasting subject and one end of the thread is taped at a corner of the month. Four hours after swallowing the thread, it is withdrawn and bile acids are eluted with buffer. The solution is applied to a Sep-Pak C18 cartridge to extract bile acids, which are subsequently analyzed by capillary gas-liquid chromatography and liquid chromatography. In vitro analyses showed that there was no preferential binding to the thread of any bile acid and that binding was pH-independent. A high correlation (r = 0.98) was found between direct analyses of bile and analyses by Entero-Test after in vitro incubation. The values obtained by the Entero-Test were similar to those of duodenal bile simultaneously collected with the normal intubation technique (r = 0.99). Duodenal bile acid composition showed a daily variation. In 11 healthy volunteers the following bile acid composition of unstimulated duodenal juice was found (mean +/- SD; %): choleate 44 +/- 12 (glycine/taurine ratio 1.8), chenodeoxycholate: 29 +/- 6 (G/T ratio 2.3); deoxycholate: 25 +/- 11 (G/T ratio 5.7), lithocholate: 1, ursodeoxycholate: less than 1. The described technique turned out to be an easily applicable method for determination of duodenal bile acids in man. This enables longitudinal studies concerning the factors that determine the bile acid pool composition and its relevance to various diseases.
A method is described for breath sampling which can be used for breath hydrogen estimations not only in clinical practice, but also at home. Sampling of end-expiratory air is performed using a 10-ml syringe with a side hole. The samples are transferred to 3-ml vacuum tubes, which can be stored and mailed without significant loss of hydrogen. The hydrogen concentration is estimated gas chromatographically using 0.4 ml of sampled air. This method was used to assess the breath hydrogen pattern under normal circumstances: the daytime breath hydrogen profile. Fourteen children sampled their breath at 30-min intervals during one full day, and recorded diet and activity. The normal daytime breath hydrogen profile showed a typical pattern. Morning values were low, but the evening values were markedly increased in half of the children. These patterns differed markedly from those registered in three children with carbohydrate malabsorption. The daytime breath hydrogen profile, which is easy to perform and applicable at home, might provide valuable additional information in the investigation of children with suspected carbohydrate malabsorption.
It is important to determine the energy losses and the possible deficiencies of vitamins and trace elements for the adequate treatment of the patient. The energy loss presumably can be assessed best by direct calorimetry, supplemented by the assay of faecal fat, nitrogen and of breath hydrogen. Restriction of energy losses is feasible by prescribing a high-protein diet with a normal fat content, preferably polyunsaturated fats, in combination with pancreatin. Deficiencies of vitamins and trace elements can be prevented by timely supplementation.
Sulphated lithocholic acid conjugates (SGLC) were measured in the sera of 268 children with various hepatic and intestinal disorders. Two groups were distinguished: (I) SGLC concentration less than or equal to 1.2 mumol/l, n = 198, and (II) SGLC concentration greater than 1.2 mumol/l, n = 70. In 28 patients of the latter group the SGLC concentration was less than 25% of the concentration of glycocholic acid (GC) in the same serum sample. This group (IIA) consisted predominantly of patients with cholestasis, as characterized by high serum bile acid levels and deviating liver function tests. The rest of the group (IIB), with SGLC levels exceeding 25% of the GC concentration and relatively low serum bile acid concentrations, showed no clear cholestatic symptoms. A postprandial increase in serum SGLC (delta SGLC) greater than 1.0 mumol/l was found in only 1 of 32 patients of group I (3%), in 1 of 6 patients of group IIA (17%), but in 9 of 11 patients of group IIB (81%). delta SGLC did not correlate with delta GC in the same test, which indicated that a general hepatic bile acid clearance defect was not responsible. In two patients with intermittent cholestasis, the distinct postprandial rise in serum SGLC that was always found during anicteric periods could be prevented by adding cholestyramine to the test meal. We conclude that elevated serum concentrations of SGLC develop during the course of cholestasis but may also be caused by influx of this bile acid from the intestine. Because of its hepatotoxic properties, SGLC may be involved in the initiation or perpetuation of specific cholestatic phenomena.
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Generalized edema resulting from severe protein-losing enteropathy occurred in three patients 12, 15, and 17 months after the Fontan operation. One patient originally had tricuspid atresia and the other two, univentricular heart disease. At operation a conduit had been inserted between the right atrium and pulmonary artery. Apart from the protein loss, the patients were in good health. The cardiac catheterization data obtained 0.8 to 2.4 years (median 1.3 years) after operation in the three patients with protein-losing enteropathy were compared with those of 18 patients in whom Fontan's operation had been performed because of tricuspid atresia (eight patients) or univentricular heart disease (10 patients). All had atriopulmonary connections. The mean right and left atrial pressures and systemic blood flows measured by dye dilution in the patients with and without protein-losing enteropathy did not differ. However, the patients with protein-losing enteropathy had a higher diastolic right atrial pressure. Since maximal antegrade flow in the superior vena cava after Fontan's operation occurs during atrial diastole, these observations suggest that an increase in diastolic right atrial pressure may result in protein-losing enteropathy because of impairment of blood flow and therefore congestion in the superior vena cava, subclavian vein, and thoracic duct.
A simple test is described for the diagnosis of monosaccharide malabsorption in infancy caused by a congenital defect of glucose and galactose transport. Increased hydrogen (H2) excretion in expired air after ingestion of sugar was used to diagnose this condition in an infant with severe diarrhoea after breast feeding. Abnormal amounts of H2 were excreted after oral administration of glucose and galactose, but not after fructose. A carbohydrate free diet supplemented with fructose resulted in rapid weight gain and disappearance of diarrhoea. The diagnosis of glucose-galactose malabsorption was confirmed by 14C-glucose transport studies on a jejunal mucosal biopsy specimen. These findings indicate that interval breath H2 estimation in mixed expired air is a non-invasive, reliable procedure for detection of monosaccharide malabsorption in infancy.
Disturbances of the excretion and reabsorption of bile acids are discussed. Disturbances in bile acid metabolism can be found after terminal ileum resections, in contaminated small bowel syndrome, Crohn's disease localized in terminal ileum, ileostoma, non-specific diarrhoea of infancy, bulky small bowel contents, coeliac disease, cystic fibrosis, prematurity, intra- and extrabiliary obstruction. They can cause or increase malabsorption. Total bile acid and cholylglycine in serum and duodenal fluid can be used in diagnosing malabsorption. Estimation of serum bile acids and triglycerides after an oral fat loading test may provide information about the localization of small intestinal dysfunction.
The ability of ultrasound to detect biliary obstruction, bile leakage and generalized ductal changes after orthotopic liver transplantation (OLT) was compared to cholangiography. Cholangiography was considered to be the gold standard. Adequate opacification of the biliary tree was achieved in 139 cholangiograms. Biliary obstruction, intermediate or large bile leakage, and generalized ductal changes were diagnosed with cholangiography in 15% (21/139), 14% (20/139), and 16% (22/139), respectively. Normal ultrasound findings could not exclude biliary stricture, generalized ductal changes, or bile leakage, and fluid collections were not correlated with bile leakage. Abnormal ultrasound findings were highly predictive of the cholangiographic diagnosis of biliary obstruction or generalized ductal changes (specificity of 98% and 100%, respectively). An irregular appearance of the bile ducts and increased periductal echogenicity proved to be characteristic features for generalized ductal changes.
BACKGROUND: Selective angiography involves increased risk for children and may cause vascular complications. The aim of this study was to determine whether selective angiography is still necessary for viewing the portal vein and hepatic artery. METHODS: Doppler ultrasound, abdominal aortography, and selective angiography were performed and interpreted independently in 62 children (median age = 1 year 3 months), with end-stage liver disease, who were candidates for orthotopic liver transplantation. RESULTS: Selective angiography agreed with the Doppler ultrasound findings of patency, flow direction, and diameter of the extrahepatic portal vein in 84%, 73%, and 79% of the children, respectively. Important additional information was obtained from selective angiography in only five of the 62 children (8%). Selective angiography showed an anomaly of the hepatic artery in 21 of the 62 children (33%). In 18 out of these 21 children (85%), an anomaly of the hepatic artery was already visible on the abdominal aortogram. CONCLUSION: Selective angiography did not play any significant role in the detection of an anomaly of the hepatic artery and should only be done if the Doppler ultrasound findings of the portal vein are inconclusive or if abdominal aortography cannot provide reliable information about the hepatic artery. For the evaluation of the portal vein and hepatic artery, we recommend Doppler ultrasound and abdominal aortography, a less invasive angiographic procedure.