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Biomedical subjects

J Fernandes

Publications and source records attributed to J Fernandes.

At least 55 records · Page 3Linked to original sources
PubMed1988

Dumping syndrome in children. Diagnosis and effect of glucomannan on glucose tolerance and absorption.

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.

Adolescent↗
PubMed1988

Long-term and short-term effects of dietary cholesterol and fats in the Mongolian gerbil.

In long-term and short-term experiments with Mongolian gerbils the effects of various dietary fats and different amounts of cholesterol on progeniture and liver pathology were studied. In contrast to palm kernel oil, with 8.75% (w/w) of sunflower seed oil we observed fewer litters and a decreased survival of the sucklings. On 0.2% of cholesterol we observed severe hypercholesterolemia and storage of cholesterol ester in the liver, ultimately leading to cirrhosis. On 0.05% for 6 months the gerbils stayed in good health. Progeniture was unaffected on this level of intake.

Animals↗
PubMed1987

Fat absorption in preterm infants fed a taurine-enriched formula.

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.

Dietary Fats↗
PubMed1987

Cytochrome c oxidase deficiency in subacute necrotizing encephalomyelopathy.

Two new patients with Leigh's syndrome (subacute necrotizing encephalomyelopathy) due to deficiency of cytochrome c oxidase are presented and their data are compared with those of the four Leigh's syndrome patients previously reported with this deficiency. It is not possible to distinguish between the various biochemical aetiologies of Leigh's syndrome on clinical grounds. Investigation of pyruvate metabolism and of the respiratory chain will reveal the enzymatic defect in some of the patients. It has now been firmly established that a relationship exists between Leigh's syndrome and deficiency of cytochrome c oxidase. There are, however, other syndromes which are also associated with a deficiency of this enzyme. In Leigh's syndrome, the enzyme deficiency has been reported in many organ systems and in cultured fibroblasts. In the liver, however, decreased, intermediate or normal values of cytochrome c oxidase activity have been found. Selective or more widespread involvement of organ systems, due to mutations of either the nuclear or the mitochondrial DNA encoding for different subunits of the enzyme molecule (some of which may be organ- or tissue-specific), could explain the clinical and biochemical heterogeneity of syndromes associated with a cytochrome c oxidase deficiency.

Brain↗
PubMed1987

Lactose malabsorption and giardiasis in Basotho school children.

The prevalence of primary, adult-type, lactose malabsorption was assessed by means of the hydrogen breath test after intake of 360 ml of full cream milk (approximately 18 g lactose) in 96 randomly selected Basotho school children, aged 5-15 years. Of 86 children who did not have diarrhoea in the previous week 82 (85%) were lactose malabsorbers, while 4 (5%) could not be classified because of undetectable hydrogen excretion. Milk intolerance presenting as diarrhoea was significantly (p less than 0.01) more common in children who associated previous abdominal complaints with milk intake and/or did not like milk. A negative hydrogen breath test was significantly (p less than 0.05) more often observed in children who had diarrhoea in the previous week. Giardia was present in 18 (19%) of 93 children. The incidence of giardiasis did not correlate with the presence of lactose malabsorption in children without diarrhoea in the previous week. However, milk intolerance presenting as diarrhoea was significantly (p less than 0.05) more common in children with giardiasis. The findings support the use of physiological quantities of milk in Basotho school children.

Adolescent↗
PubMed1987

Urinary excretion of lactate, 2-oxoglutarate, citrate, and glycerol in patients with glycogenosis type I.

Growth retardation and lactic aciduria are well-known abnormalities in patients with a deficiency of either glucose-6-phosphatase or glucose-6-phosphate translocase. In 19 patients with glucose-6-phosphatase and two patients with glucose-6-phosphate translocase, growth retardation was quantified by calculating the height standard deviation score. The urinary excretion of lactate and some other metabolites was quantified by calculating the lactate/creatinine, 2-oxoglutarate/creatinine, citrate/creatinine, and glycerol/creatinine ratios in urine. Significant correlations were found between the lactate/creatinine ratio, the 2-oxoglutarate/creatinine ratio, and height SD score. Urinary lactate appeared to respond promptly to changes of the diet, while urinary 2-oxoglutarate responded only slowly, as did growth itself. The citrate/creatinine ratio and the glycerol/creatinine ratio were within the normal range and varied little. It was concluded that the urinary 2-oxoglutarate excretion primarily reflects the severity of the disease as expressed in stunted growth. Thus, while urinary lactate levels are more suitable for monitoring the diet, urinary 2-oxoglutarate levels can be used as an indication for intensive treatment with hyperalimentation.

Adolescent↗
PubMed1986

Incomplete lactose absorption from breast milk during acute gastroenteritis.

The incidence and degree of incomplete lactose absorption was investigated in breast fed infants and children up to two years of age during acute gastroenteritis (GE). Lactose absorption was assessed in 50 patients by means of the hydrogen breath test (HBT), approximately 5.5 days after the admission to hospital. HBT detected incomplete lactose absorption of marked (lactose malabsorption) and probably mild degree in 8 and 6 patients respectively. Incomplete lactose absorption appeared to be transient in all 5 patients retested after discharge. HBT failed to identify 8 cases of lactose intolerance which were detected by investigation of the stools. In 31 breast fed controls of a similar age range incomplete lactose absorption of only mild degree was probably present in 2 and lactose intolerance in 1, which too was only detected by investigation of stools. During acute GE the use of HBT is appropriate to detect milder forms of incomplete lactose absorption than lactose intolerance. For the detection of lactose intolerance the measurement of pH and reducing substances in the stools remains the method of choice. The findings are in favour of the continuation of breast feeding during acute GE.

Acute Disease↗
PubMed1986

The daytime breath hydrogen profile in children with abdominal symptoms and diarrhoea.

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.

Adolescent↗
PubMed1986

Influence of age on the selenium status in Belgium and The Netherlands.

Plasma selenium concentration and glutathione peroxidase activity in red blood cells were determined in subjects from different age groups. The selenium level (mean +/- SD) found in infancy (0 to 6 months) was 2 +/- 0.6 micrograms/dl, with the lowest value of 1 microgram/dl observed in a 4-month-old infant. These levels were significantly lower (p less than 0.001) than the value of 9.5 +/- 1.1 micrograms/dl found in the adult group and 7.7 +/- 1.3 micrograms/dl found in the group of older children (2 to 15 yr). Younger children (6 to 24 months of age) had intermediate levels of 5 +/- 1.2 micrograms/dl. When the data were plotted on a logarithmic scale as a function of age, the figure shows clearly that the plasma selenium levels increase steadily with age throughout life after an initial drop at 60 to 90 days. There was a satisfactory correlation between the plasma selenium concentration and the enzyme glutathione peroxidase activity in the red blood cells (Spearman's p = 0.45, p less than 0.005). Although very low selenium values were observed, the enzyme glutathione peroxidase activity remained above 10 U/g hemoglobin (with only one exception) in all patients.

Adolescent↗
PubMed1985

The daytime breath hydrogen profile: technical aspects and normal pattern.

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.

Adolescent↗
PubMed1985

General aspects of nutrition, absorption and metabolism in patients with cystic fibrosis.

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.

Avitaminosis↗
PubMed1985

The serum apolipoprotein profile of patients with glucose-6-phosphatase deficiency.

Abnormal lipid transport is one of the more severe pathophysiological manifestations of glucose-6-phosphatase deficiency (glycogen storage disease, type I: GSD-I). To characterize further lipoprotein abnormalities in this inborn error of glycogen metabolism, we determined the levels of serum apolipoproteins (Apo) A-I, A-II, B, C-I, C-II, C-III, D, and E in 10 male and 12 female patients, 1-37 yr of age. Results showed that patients with GSD-I have a unique apolipoprotein profile characterized by normal or slightly decreased levels of ApoA-I and ApoA-II, reduced concentrations of ApoD, and significantly increased levels of ApoC-I and ApoC-II (p less than 0.01) and ApoB, ApoC-III, and ApoE (p less than 0.0001) in comparison with age- and sex-matched normolipidemic controls. However, there was some overlap of values in patients and controls for each of the lipid and apolipoprotein constituents with the exception of ApoC-III. This finding supported by the results of the logistic regression analysis showed that the concentration of ApoC-III is the best criterion for distinguishing patients with GSD-I from control subjects and the most characteristic feature of the deranged lipid transport system in this deficiency disease. It remains to be clarified, however, whether the ApoC-III concentrations in patients with GSD-I reflect the degree of other metabolic and clinical manifestations of this disease such as hyperlacticacidemia, hyperuricemia, and growth retardation.

Adolescent↗
PubMed1985

Sulphated lithocholic acid conjugates in serum from children with hepatic and intestinal diseases.

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.

Adolescent↗