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Biomedical subjects

K M Hambidge

Publications and source records attributed to K M Hambidge.

At least 19 recordsLinked to original sources
PubMed1992

Decreased selenium intake and low plasma selenium concentrations leading to clinical symptoms in a child with propionic acidaemia.

A child with biotin-non-responsive propionic acidaemia treated with a propiogenic amino acid-restricted diet presented with an elevated blood mean corpuscular volume (MCV) of 93.1 fl, indicative of macrocytosis, and unusual hair texture with hypopigmentation. Plasma selenium concentration at this time was subnormal (45.9 micrograms/L), and calculated dietary selenium intake was 4.7 micrograms/day. After 4 months of selenium supplementation (50 micrograms/day) plasma selenium concentration normalized (97.7 micrograms/L) in conjunction with a reduced MCV (84.0 fl) and a dramatic improvement in hair growth, colour and length. Two additional periods off and on selenium supplementation, of varying time intervals, resulted in similar clinical changes. We conclude that these clinical changes were due to a deficient intake of dietary selenium.

Amino Acid Metabolism, Inborn Errors↗
PubMed1992

Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis.

To evaluate the impact of early pancreatic insufficiency on growth and nutritional status in cystic fibrosis, we studied 49 infants identified by a newborn screening program. Pancreatic insufficiency, determined by increased 72-hour fecal fat excretion, was present in 59% (23/39) of infants at diagnosis (7.0 +/- 0.8 weeks; mean +/- SEM). Before initiation of pancreatic enzyme replacement, growth and nutritional status of pancreatic-insufficient (n = 16) and pancreatic-sufficient (n = 13) infants were compared. Pancreatic-insufficient infants gained less weight from birth to diagnosis (13.4 +/- 3.4 vs 22.3 +/- 4.0 gm/day; p = 0.05), had decreased triceps skin-fold thicknesses (4.5 +/- 0.3 vs 6.1 +/- 0.4 mm; p less than 0.005), and had lower blood urea nitrogen (3.07 +/- 0.42 vs 4.62 +/- 0.65 mg/dl; p = 0.02) and albumin (2.99 +/- 0.14 vs 3.54 +/- 0.14 gm/dl; p less than 0.01) levels despite higher gross calorie (154 +/- 8 vs 116 +/- 13 kcal/kg per day; p less than 0.01) and protein intakes (2.81 +/- 0.21 vs 2.14 +/- 0.33 gm/kg per day; p = 0.03). Fecal nitrogen loss was correlated with fat loss (r = 0.79; p less than 0.001). Fat malabsorption was present in 79% (30/38) and 92% (33/36) of infants tested at 6 months and 12 months of age, respectively, indicating that pancreatic insufficiency persists and increases in frequency throughout infancy. We conclude that pancreatic insufficiency is prevalent in young infants with cystic fibrosis and has a significant impact on growth and nutrition.

Anthropometry↗
PubMed1992

The analysis of stable isotopes in urine to determine the fractional absorption of zinc.

We measured isotopic enrichment in urine after oral and intravenous administration of stable isotopes of zinc to determine fractional absorption (FA). 68Zn and 70Zn were administered orally and intravenously to four normal adults. Subsequently, urine and fecal samples were collected for 7 and 14 d, respectively, ashed, and passed through ion-exchange columns to separate zinc from other elements. Samples were analyzed by fast-atom-bombardment mass spectrometry. From 32 h onwards the enrichment of 68Zn and 70Zn in urine declined proportionately so that FA could be determined as follows: FA = enrichment (oral/iv) x dose (iv/oral). FA determinations from urine and feces (cumulative excretion) were, respectively, for subject ZK1, urine 0.79 +/- 0.03 and feces 0.70 +/- 0.01; ZK2, 0.79 +/- 0.05 and 0.69 +/- 0.02; ZK3, 0.26 +/- 0.01 and 0.25 +/- 0.01; and ZK4, 0.41 +/- 0.02 and 0.37 +/- 0.02. ZK1 and ZK2 received the oral isotope while fasting whereas ZK3 and ZK4 received the oral isotope with meals. FA of zinc can be determined by measurement of isotope enrichment in urine.

Absorption↗
PubMed1992

Zinc and diarrhea.

Malnutrition is a major factor in the etiology, management and prognosis of persistent diarrhea in young children. Apart from inadequate energy intake, deficiencies of several specific nutrients have been implicated. Zinc is a micronutrient that appears to be of special interest, at least in some communities. Zinc deficiency has been documented in otherwise normal children. The risk of deficiency, however, is enhanced by diarrhea which is associated with variable but sometimes gross increases in zinc losses in the feces. These losses could contribute to a vicious circle, as there is now evidence that mild as well as severe zinc deficiency states can contribute to the duration and severity of diarrheal disease. During rehabilitation, impaired zinc nutriture could be responsible for slow growth, especially if the rehabilitation diet is high in phytate, a recognized inhibitor of zinc absorption. Research should be directed to a better understanding of zinc metabolism and homeostasis during diarrhea disease, to the consequences of zinc deficiency and to the benefits to be derived from zinc supplementation programs.

Adult↗
PubMed1991

Sequential concentrations of copper and ceruloplasmin in serum from preterm infants with rickets and fractures.

Concentrations of copper (Cu) and ceruloplasmin in serum were measured serially in 49 preterm infants with mean (+/- SEM) birth weights of 979 +/- 33 g and gestational ages of 28.4 +/- 0.3 weeks at three, six, nine, and 12 months postpartum. Serial radiographic studies showed 17 infants with (group A) and 32 infants without (group B) rickets or fractures. Cu and ceruloplasmin concentrations in serum also were measured in 21 healthy term infants (group C) with birth weights 3668 +/- 98 g at three, six, and 12 months postpartum. Analyses of covariance of serial changes in these serum variables--taking into account such potential covariates as differences in gestational age, birth weight, initial weight and length, changes in weight and length during the study, the duration of parenteral nutrition, and increased enteral copper intake--showed both groups of preterm infants had significantly lower concentrations of Cu in serum up to age six months and ceruloplasmin up to age three months (P less than 0.001) when compared with term infants. By one year of age, Cu and ceruloplasmin concentrations in serum in all groups had increased significantly (P less than 0.001), into the adult range, and were not significantly different among groups. These data document a maturational lag in copper metabolism in small, preterm infants. Changes in concentrations of Cu and ceruloplasmin in serum were significantly correlated (r = 0.92, P less than 0.001) but were not significantly different between preterm infants with and without rickets or fractures at each age.

Aging↗
PubMed1990

Oxidant injury to hepatic mitochondrial lipids in rats with dietary copper overload. Modification by vitamin E deficiency.

To examine the role of oxidant damage to subcellular membranes in the pathogenesis of copper hepatotoxicity, the effects of dietary copper overload and varying states of vitamin E on biochemical, histological, and ultrastructural features of rat liver were investigated. Weanling male rats were pair-fed for 8 weeks on diets containing normal or high levels of copper in combination with either deficient, sufficient, or excessive vitamin E. Hepatic microsomes and mitochondria, isolated by differential centrifugation, showed similar enrichment and recovery among all experimental groups. Evidence of in vivo peroxidation of membrane lipids (generation of conjugated dienes and thiobarbituric acid reacting substances) was present in mitochondrial but not microsomal preparations from copper-overloaded rats. Serum aspartate aminotransferase, alanine aminotransferase, and cholylglycine (which were increased in all copper-overloaded rats), as well as mitochondrial thiobarbituric acid-reacting substances, were more elevated in vitamin E-deficient rats. In copper-overloaded rats, liver histology showed changes of acute and chronic hepatocyte injury with mild periportal fibrosis; electron microscopy showed abundant copper-containing lysosomes and dilated cristae of hepatocyte mitochondria, findings similar to those in the liver of humans with copper-overload disorders. These findings suggest that an oxidant injury to hepatocyte mitochondria may be one of the initiating factors in hepatocellular damage that leads to hepatic lesions in copper-overload states in humans.

Animals↗
PubMed1990

Treatment of Wilson's disease with triethylene tetramine hydrochloride (Trientine).

Penicillamine is the drug of choice for the treatment of Wilson's disease, whatever the stage of the illness. Toxic manifestations may preclude the use of this life-saving drug in some patients and discontinuation of penicillamine therapy usually leads to death. We report our experience with Trientine in seven patients, aged 13 to 33 years, with Wilson's disease who developed toxic manifestations with penicillamine that required discontinuation of therapy. These include two with nephrosis, one with neutropenia, two with thrombocytopenia, and one each with a SLE-like and a Henoch-Schonlein-like syndrome. The patients were treated for periods from 6 weeks to 16 years with a dose of 0.5 to 2 g/day. Trientine proved to be an effective alternative copper chelating agent in the treatment of Wilson's disease in patients with penicillamine-induced neutropenia, thrombocytopenia, SLE, and nephrosis. No serious untoward side effects were noted.

Administration, Oral↗
PubMed1990

The immune response to short-term nutritional intervention in advanced chronic obstructive pulmonary disease.

Nine patients with advanced chronic obstructive pulmonary disease (COPD) and recent weight loss resulting in a state of mild malnutrition were entered into a refeeding program at a clinical research center. They were divided into two groups, one using a hospital diet and the other a hospital diet with supplementation. Both groups of patients gained significant weight. Refeeding and weight gain were associated with a significant increase in absolute lymphocyte count and with an increase in reactivity to skin test antigens after 21 days of refeeding. Few changes occurred in large numbers of additional serum measurements during the study period. These preliminary observations suggest that dietary and supplementary refeeding may improve the immune responses in patients with COPD.

Aged↗
PubMed1990

Pre-breakfast plasma zinc concentrations: the effect of previous meals.

The aim of this study was to determine the effect of the time and size of the last meal of the previous day on pre-breakfast 0700 hours plasma zinc concentrations. Nine healthy women were each studied on four separate occasions when the same standard meal was taken either at 1800 hours (study 1); double meal at 1800 hours (study 2); at 2400 hours (study 3); or at 1800 hours and 2400 hours (study 4). Mean differences between 1800 hours (day 1) and 0700 hours (day 2) plasma zinc (mumol/L +/- SEM) were 1.6 +/- 0.3 for study one; 0.8 +/- 0.3 for study two; 0.1 +/- 0.3 for study three and 0.0 +/- 0.2 for study four. The differences for studies two, three and four were significantly less than the difference for study one (p less than 0.05). The difference for study four was also less than the difference for study two (p less than 0.05). Changes in time and size of the meals accounted for 59% of within-subject variation in 0700 hours plasma zinc concentrations. It is concluded that pre-breakfast 0700 hours plasma zinc concentrations are affected by the time and the size of the meal(s) consumed the previous evening.

Adult↗
PubMed1989

Maternal zinc and selenium status in pregnancies with a neural tube defect or elevated plasma alpha-fetoprotein.

Zinc and selenium status was assessed in 30 non-pregnant women, 69 women during normal pregnancy, six with a fetus with a neural tube defect (NTD) and 16 who had a raised plasma alpha-fetoprotein (AFP) but no detectable fetal abnormality. Plasma zinc and selenium concentrations were significantly reduced in the second trimester of normal pregnancy compared with non-pregnant levels. A significant decrease in concentrations of zinc in plasma and selenium in plasma and leucocytes was observed in women in the third trimester compared with women in the second trimester. Women with a fetal NTD and women with an unexplained elevation of plasma AFP had significantly lower leucocyte concentrations of zinc and of selenium. Mean values for plasma zinc, plasma and erythrocyte selenium, and for the activity of glutathione peroxidase in whole blood did not differ from those for normal pregnancy.

Erythrocytes↗
PubMed1989

Post-prandial and daily changes in plasma zinc.

The objective of this study was to determine changes in plasma zinc over a 24-hour period and the relationship of these changes to meals. Hourly plasma zinc concentrations were measured in 12 normal adult women for 24 consecutive hours. The subjects were fed standard meals at 0700 hours, 1200 hours and 1700 hours. A decline in plasma zinc started 1/2 to 1 1/2 hours after each meal, reaching a low point 3-4 hours after each meal. During the night there was a progressive increase in plasma zinc concentrations averaging (1.54 micrograms/dl)h-1 for 11 hours. The lowest value of the day at 2100 hours was 22% lower than the highest value at 0730 hours. These results indicate that meals are the primary factor and could be the only factor responsible for variations in an individual's plasma zinc during any one day.

Adolescent↗
PubMed1979

Zinc nutritional status of young middle-income children and effects of consuming zinc-fortified breakfast cereals.

The effects of consuming zinc-fortified ready-to-eat breakfast cereals were determined in a double-blind controlled study. The 96 healthy young children who participated (mean age 58 months) consumed either zinc-fortified cereal, providing 25% United States Recommended Dietary Allowance per 1 ounce serving (test children) or nonzinc-fortified cereals (controls) for a 9-month period. The test children were calculated on average to receive an additional 2.57 mg of zinc per day from this fortification program. This increment increased their mean daily zinc intake to a level that approached the Recommended Dietary Allowance (10 mg) of the National Academy of Sciences for children less than 10 years of age. By the end of the period, the test children (combined sexes) had a mean increment of plasma zinc that was 6.5 micrograms/dl greater than that of the control children (P less than 0.02). The test girls had a greater increment (28.5 micrograms/g) in hair zinc content than controls girls (P less than 0.05). There were no significant differences in other biochemical parameters including plasma copper and serum cholesterol. No significant differences in food intake or growth velocity were associated with the consumption of the zinc-fortified cereal. Multiple sex and time related differences occurred in plasma, hair, urine, and parotid saliva zinc concentrations that were unrelated to the type of cereal consumed.

Child↗
PubMed1978

Acrodermatitis enteropathica.

Acrodermatisis enteropathical has had a remarkable hist orical course beginning only 35 years ago, first passing through a period of empiric management with the 8-hydroxyquinoline drugs and culminating with the discovery of a biochemical effect in zinc absorption. This accomplishment has not only resulted in the cure of a serious and often fatal disease, but has opened the way to an understanding of the vital role played by the trace element zinc in many previously unrecognized areas of human physiology.

Acrodermatitis↗
PubMed1977

Pediatric kidney transplantation for cystinosis.

In an experience of more than 700 kidney transplants, three recipients are known to have had congenital cystinosis. A cadaver kidney transplant in a 10 yr old child with nephropathic cystinosis functioned well for 7 1/2 yr before it was rejected and the graft was free of recurrent cystinosis after that interval. This prolongation of life was associated with marked accumulation of cystine in the patient's thyroid gland, presenting as a mass in the neck. If additional longevity is achieved in this patient, abnormal intracellular cystine accumulation may be expected in other host tissues. Retransplantation was carried out with the father as donor, accepting the possibility of increased cystine levels in the retransplant. This patient's age of 18 yr identifies him as one of the longest known survivors of infantile nephropathic cystinosis.

Adolescent↗