Dumping syndrome after combined pyloroplasty and fundoplication.
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Biomedical subjects
Publications and source records attributed to C M Kneepkens.
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A fatal case of fulminant hepatic failure that occurred in the neonatal period is reported in a premature infant born after 27 4/7-weeks' gestation. Immediately after birth the infant had severe hypoxia and hypotension resulting from birth asphyxia, hypovolemic shock, and septicemia. At autopsy, histological appearance of the liver showed virtually total hepatocellular necrosis without features of fibrosis. Although the exact cause of hepatocellular injury cannot be fully ascertained, it is assumed that hypoxia and hypotension must have been the predominant factors leading to massive hepatic necrosis.
Lipid peroxidation has gained increasing interest in recent years as one of the more prominent features of free radical-induced damage in biology. The study of lipid peroxidation might increase our understanding of the etiology and pathophysiology of a great number of diseases. Ethane and pentane are among the numerous end-products of lipid peroxidation and although they represent only a small and possibly variable proportion of the total amount of peroxidized polyunsaturated fatty acids, their determination in head space or exhaled breath enables accurate assessment of oxidative stress both in vitro and in vivo. To date, the number of studies utilizing the hydrocarbon breath test as a marker of lipid peroxidation in humans is small. Technical difficulties are among the main reasons for the limited use of this method. An appropriate washout period, the use of the right materials, the scrupulous avoidance of air contamination, adequate preinjection concentrations of the samples, and a sensitive gas chromatographic technique enable the accurate and reproducible measurement of hydrocarbons in human breath. The hydrocarbon breath test provides a noninvasive and extremely sensitive instrument for the assessment of oxidative stress status in adults as well as in children.
Two neonates with intestinal obstruction and two children (aged 1 and 4 years) with severe constipation since birth are reported in whom stenosis of the distal rectum was found. In association with the rectal anomaly, three of them had a presacral tumour (teratoma in two, hamartoma in one) and all had a deformed sacrum. An embryological hypothesis to explain this association has been postulated by Currarino, after whom this triad has been named. Two patients were related (father and daughter). The role of hereditary factors in the occurrence of the syndrome has been reported before. Operative treatment of the rectal stenosis was necessary in all patients. Preoperative diverting colostomy was performed in three cases, followed by a posterior sagittal approach to excise the rectal stenosis and the presacral mass. In one case, persistent cerebrospinal fluid leakage required re-exploration for closure of a tear in a congenitally abnormal dural sac. The fourth patient had undergone a low anterior resection in the past via the abdominal route and needed rectal dilatation afterwards for some time. The final result in all patients appears satisfactory, although follow-up is short. Most cases of this triad have been reported in children but a number of patients have been diagnosed only as adults. Recognition of this triad should imply a careful search for neural crest malformations. Operative treatment to correct all soft tissue anomalies leads to good results.
A 2 1/2-year-old boy presented severely underweight and with an extremely distended abdomen. He was born prematurely and at age 6 months had an incarcerated inguinal hernia, which had been treated conservatively. Laboratory investigations at the time of presentation showed evidence of bacterial overgrowth. Barium studies revealed a fistula between the jejunum and sigmoid colon. Resection of the fistula was followed by complete recovery. We suggest that the incarcerated hernia underlies this fistula. Until now, this series of events has not been published.
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Apple juice contains fructose and sorbitol, substances that have been shown to be incompletely absorbed by most people. As this might have clinical consequences, especially in young children, we investigated the absorption of the carbohydrate content of apple juice in apple juice consuming toddlers with chronic nonspecific diarrhoea as compared to controls, using the breath hydrogen (H2) test. Incomplete absorption of the carbohydrates from 250 ml of apple juice, as indicated by a maximum breath H2 increase of greater than or equal to 20 parts per million (ppm), was found in all nine patients (mean +/- SEM 57 +/- 8 ppm), and in five out of eight controls (22 +/- 7 ppm) (P less than 0.01). Six patients were retested with apple juice "enriched" with glucose, which is known to improve fructose absorption. The maximum breath H2 increase as well as the area under the breath H2 curve decreased significantly. It was thus estimated that fructose accounted for 80% of the incomplete absorption and sorbitol for 20%. Elimination of apple juice from the diets of the nine patients resulted in normalisation of both the frequency and the consistency of the stools. Incomplete absorption of the carbohydrates, particularly fructose, from apple juice seems to be quite common, and may contribute to chronic diarrhoea in young children.
A 14 year old Turkish girl was diagnosed to have hepatitis A according to symptoms and positive serology. She had a clinical and biochemical relapse eight weeks after the first manifestation of the disease. Relapsing hepatitis A is very rare but it does not alter the usual favourable outcome.
Dumping syndrome is rarely seen in children. We studied ten children with symptoms following various types of gastric surgery. All had abnormal blood glucose and breath hydrogen responses after oral glucose challenge, indicating reduction of both glucose tolerance and glucose absorption, as compared to controls. Glucose tolerance was best expressed as the difference between peak and subsequent lowest blood glucose level, the upper limit of normal (mean + 3 SD) being 5.9 mmol/l. In eight children with persisting symptoms we tested the effects of glucomannan, a highly hygroscopic tasteless fibre, on glucose tolerance and glucose absorption. Glucomannan significantly improved glucose tolerance, but did have no overall effect on glucose absorption. Four children tested glucomannan added to the main meals. Breath hydrogen excretion, as established with the daytime breath hydrogen profile, decreased significantly in two, but complaints increased in three children. Glucomannan does not appear to be suitable for the treatment of dumping syndrome in children as the side effects seem to overrule the beneficial effects.
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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.
Transient hyperphosphatasemia of infancy. Striking transient increases in serum alkaline phosphatase in nine new cases are reported. The etiology is still unknown. The patients were 6.5 months to 30 months of age and none showed any clinical evidence of hepatic or skeletal disease. Values became normal within 12 weeks in all patients. It is important to know this condition to avoid unnecessary and expensive diagnostic procedures. Though hyperphosphatasemia does not seem to give any problems we recommend a brief diagnostic protocol in order to recognize transient hyperphosphatasemia of infancy.
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.