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Biomedical subjects

J Fernandes

Publications and source records attributed to J Fernandes.

At least 91 records · Page 5Linked to original sources
PubMed1984

The dietary treatment of children with type I glycogen storage disease with slow release carbohydrate.

The effect of ingestion of uncooked cornstarch (2 g/kg body weight) in water, uncooked starch (1 g/kg) added to a meal, and glucose (2 g/kg) in water, was studied in eight patients with type IA glycogen storage disease (GSD) and one patient with type IB GSD. Blood glucose concentrations were determined at 30-min intervals during each tolerance test; blood lactate, blood insulin, and expiratory hydrogen were determined at 60-min intervals. The glucose levels remained in the normal range (greater than or equal to 1.8 mM) during approximately 6.5-9.0 h, 3.5-6.5 h, and 2.25-4.0 h during the three tolerance tests, respectively. The lactate levels differed markedly for the different tests per patient, and for the same type of test between the patients. Blood insulin concentrations after starch administration did not exceed values of 50 mU/liter above fasting levels and were markedly lower than those after glucose administration (maximum levels of 280 mU/liter). The expiratory hydrogen excretion did not increase or only slightly increased after cornstarch administration (less than 20 ppm).

Adolescent↗
PubMed1984

Carbohydrate malabsorption in children with severe protein energy malnutrition.

To determine the incidence of carbohydrate malabsorption, particularly lactose malabsorption in Basotho children with severe PEM during treatment with a dry skim milk (DSM)-sucrose-oil mixture, 105 children with PEM were submitted to a Hydrogen Breath Test (HBT) after administration of the mixture. Carbohydrate malabsorption occurred more frequently in children with kwashiorkor (28/58) than in those with marasmus (5/33), marasmic kwashiorkor (3/15) and healthy controls (8/34). The positive HBT appeared to be due to lactose malabsorption in at least two thirds of the children with kwashiorkor as it turned negative when the challenge was repeated with a lactosefree mixture. In controls malabsorption of carbohydrate (usually lactose) appeared at the age of 22 months, nearly a year later than in PEM. Diarrhoea occurred in 23 children with PEM and particularly in those with carbohydrate malabsorption. In 20 children Giardia was found in the stools without any observable effect on carbohydrate malabsorption, however. The findings support the cautious use of physiological doses of lactose in the treatment of severe PEM.

Animals↗
PubMed1983

The effects of colestipol hydrochloride on serum lipoprotein lipid and apolipoprotein B and A-I concentrations in children heterozygous for familial hypercholesterolemia.

The effects of colestipol hydrochloride on serum lipoprotein lipid and apolipoprotein B and A-I concentrations in children heterozygous for familial hypercholesterolemia. Acta Paediatr Scand, 72:81, 1983.--Colestipol hydrochloride was administered to 28 children with familial hypercholesterolemia type II-A, and its effects on serum lipoproteins were tested against a placebo in a cross-over design. All children consumed a diet low in cholesterol and high in linoleic acid. Colestipol therapy resulted in a 15.7% decrease in serum very low plus low density lipoproteins and in a 13.5% decrease in serum apolipoprotein B. High density lipoprotein cholesterol, serum apolipoprotein A-I and serum triglycerides remained unaltered.

Adolescent↗
PubMed1983

[Hypoglycemia].

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Child↗
PubMed1982

Trace metals in cystic fibrosis.

Serum zinc and copper concentration, 24 hrs urinary zinc and copper excretion, plasma selenium and red blood cell glutathione peroxidase activity were measured in 13 cystic fibrosis patients aged 6 to 15 years. The mean serum zinc value +/- S.D. (17.3 mumol/l +/- 4.6) did not differ from that of the control group (17.9 mumol/l +/- 3.1). Urinary zinc excretion in 12 out of 13 patients was within the normal range (1.53-13.8 mumol/24 hrs). The mean serum copper +/- S.D. (23.8 mumol/l +/- 4.2) was not significantly elevated as compared to the value found in the control group (19.2 mumol/l +/- 3.5), but 4 children, including 1 with documented portal hypertension, showed an urinary copper excretion greater than 0.94 mumol/24 hrs (normal: 0.16-0.80 mumol/24 hrs). Mean plasma selenium +/- S.D. (0.84 mumol/l +/- 0.25) was significantly reduced as compared to the control group (1.0 mumol/l +/- 0.15) (p less than 0.05). The correlation between selenium concentration and RBC glutathione peroxidase activity was significant (p less than 0.01). A negative correlation was also found between plasma selenium and 24 hrs faecal fat excretion (p less than 0.05). It is concluded that CF children with severe dysfunction of the exocrine pancreas are at increased risk to develop symptoms of subclinical or manifest zinc and/or selenium deficiency. Appropriate supplementation should therefore systematically be considered.

Adolescent↗
PubMed1981

Interval breath hydrogen test in glucose-galactose malabsorption.

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.

Air↗
PubMed1981

Left ventricular function in end-stage renal disease: echocardiographic classification.

We used echocardiography (ECHO) to detect pericardial effusions and assess left ventricular (LV) function in 39 patients with end-stage renal disease (ESRD). Pericardial effusions were present in 24 patients (62%). Thirty-one patients (79%) had concentric hypertrophy and 20 patients (51%) had decreased LV compliance. The majority of patients with concentric hypertrophy and decreased LV compliance had normal LV internal dimensions and contractility. Six patients (15%) had ECHO results compatible with congestive cardiomyopathy. An echocardiographic distinction between congestive heart failure, decreased LV compliance, and salt and water overload in ESRD has allowed an appropriate therapeutic decision as to whether to administer cardiac glycosides or increase ultrafiltration as a treatment for pulmonary congestion. ECHO is a safe, convenient, and noninvasive method of assessing serial LV function, and it can be used to document progressive deterioration of LV function in ESRD.

Adolescent↗
PubMed1981

The effect of a virtually cholesterol-free, high-linoleic-acid vegetarian diet on serum lipoproteins of children with familial hypercholesterolemia (type II-A).

The effect of a virtually cholesterol-free, high-linoleic-acid vegetarian diet and a high-linoleic-acid "normal" diet with a moderate cholesterol content was tested in 39 children heterozygote for hypercholesterolemia type II-A. The diets were administered in an outpatient cross-over design of two periods of 10 weeks each and the serum lipoproteins were analyzed at the end of the two 10-week periods. The vegetarian diet induced a decrease in serum concentrations of LDL-II total and free cholesterol and of apo-B, by an average of 10%, whereas HDL cholesterol and apo-A-I decreased by 4%. The disproportionately large change in LDL compared to the small change in HDL was interpreted as an antiatherogenic effect of the vegetarian diet.

Adolescent↗
PubMed1981

Cimetidine as an adjunct to oral enzymes in the treatment of malabsorption due to cystic fibrosis.

The effects of two doses orally administered Cimetidine on gastric and acid production and on intestinal absorption in 13 patients with cystic fibrosis who were treated with pancreatin supplementation were studied. Although both doses of Cimetidine reduced the acid secretion significantly, no reduction in the steatorrhea and creatorrhea or increase in weight were found, although less pancreatin seemed to be inactivated while on cimetidine. It is concluded that pancreatic extract supplementation alone is not sufficient to normalise the digestion in cystic fibrosis.

Administration, Oral↗
PubMed1981

[Malabsorption].

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Bile Acids and Salts↗
PubMed1981

[Diagnosis of carbohydrate malabsorption].

After a review of causes and symptoms of sugar malabsorption and the usual diagnostic methods, the application is described of a recently developed procedure with high specificity and sensitivity: the hydrogen breath test. Examination of a large number of children shows that its sensitivity is higher than that of the procedures used so far, that lactose malabsorption is present in over 30% of the children with recurrent abdominal pain and/or diarrhoea; that in contrast to the prevailing opinion, malabsorption of sucrose in children is rare.

Biopsy↗