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Biomedical subjects

G B Forbes

Publications and source records attributed to G B Forbes.

At least 91 records · Page 5Linked to original sources

3-methylhistidine excretion in myotonic dystrophy.

3-Methylhistidine (3-MH) excretion reflects the rate of muscle protein catabolism, since 3-MH occurs almost exclusively in muscle actin and myosin and is not reutilized or catabolized. We studied 3-MH excretion in 9 patients with myotonic dystrophy, 8 normals, and 10 disease controls with Duchenne dystrophy and other disorders. 3-MH excretion was expressed relative to muscle mass as determined by both urinary creatinine and total body potassium (40K method). Absolute 3-MH excretion was decreased in myotonic dystrophy patients but was normal when related to muscle mass. The finding of normal 3-MH excretion in myotonic dystrophy suggests that the muscle wasting in this disorder results from impaired anabolic processes rather than accelerated muscle destruction.

Adolescent↗

Iron absorption in the thalassemia syndromes and its inhibition by tea.

To determine the hemoglobin concentration at which iron absorption is minimal, five subjects with thalassemia major and one with thalassemia intermedia underwent a series of iron-absorption studies. The effect of tea as an inhibitor of non-heme iron absorption was also tested. Iron absorption increased as the hemoglobin concentration decreased, although iron absorption was much higher at any given hemoglobin level in the subject with thalassemia intermedia. In the subjects with thalassemia major, iron absorption averaged 10 per cent at hemoglobin concentrations between 9 and 10 and 2.7 per cent between 11 and 13 g per deciliter. The percentage of iron absorbed could be accurately predicted from the nucleated red-cell count (r = 0.91, P less than 0.001). Tea produced a 41 to 95 per cent inhibition of iron absorption. Since patients with thalassemia intermedia may absorb a large percentage of dietary iron, inhibitors of iron absorption, such as tea, may be useful in their management.

Adolescent↗

Steroid hormone toxicity in human fibroblasts does not correlate with high affinity receptor content.

Human diploid skin fibroblasts derived from normal individuals and those with the testicular feminization syndrome (TFM) have been shown to be killed to the same degree by dihydrotestosterone in spite of the absence of high affinity cellular androgen receptors in the TFM fibroblasts. Furthermore, several different normal fibroblast strains from various anatomical sites all showed similar amounts of androgen-induced cytotoxicity even though their respective receptor contents differed by as much as ten-fold. These results suggest that steroid-induced cytotoxicity in human fibroblasts is not correlated with receptor content, unlike murine lymphoid cells in which the receptor content has been shown to be closely related to their ability to survive hormone exposure.

Androgen-Insensitivity Syndrome↗

Loss of body nitrogen on fasting.

An analysis of the change in total body nitrogen during fasting shows that it declines exponentially, a small fraction being lost rapidly (t 1/2 of a few days), and the remainder being lost slowly (t 1/2 of many months). The obese faster loses N, and weight, at a slower relative rate than the nonobese; and the ratio of N loss to weight loss during an extended fast is inversely related to body fat content, being about 20 g/kg in the nonobese and about 10 g/kg in those with body fat burdens of 50 kg or more. The loss of body N on a low protein-calorie adequate diet can also be described in exponential terms, and this function allows an estimate to be made of the N requirement.

Adult↗

Liver damage due to perhexiline maleate.

Two middle-aged men, who had received perhexiline in recommended dosage, showed clinical and histological evidence of severe hepatic damage, and one of them died. Histological study of the livers showed a striking resemblance to alcoholic hepatitis.

Chemical and Drug Induced Liver Injury↗

Bone mineral turnover in a patient with osteogenesis imperfecta estimated by fluoride excretion.

A child with severe osteogenesis imperfecta was treated with NaF for 8 years, at the end of which time his iliac bone contained 29 mg F/g Ca. Urine F was assayed at intervals for 4.5 years after discontinuing treatment. After the first few days the decline in urinary F excretion can be described by a two component exponential function, with half-times of 5.4 months (10%) and 8.9 years (90%). The latter half-time value is of the same order of magnitude as those observed for F and other "bone-seeking" elements in normal subjects, which suggests that the turnover rate of bone mineral is normal in this disease. Three methods for estimating the attained body F burden at the end of NaF treatment--namely, metabolic balance, bone biopsy, and integration of the exponential function--yielded comparable values.

Bone and Bones↗

Enhanced dexamethasone resistance in cystic fibrosis cells: potential use for heterozygote detection and prenatal diagnosis.

Cultured skin fibroblasts from patients with cystic fibrosis (CF) are more resistant to dexamethasone toxicity than are normal cells. We now report that, when fibroblasts cultured from obligate CF heterozygotes are exposed to dexamethasone, they have an intermediate survival compared to normal and homozygous CF cells. When dexamethasone survival was tested on cells from four patients undergoing amniocentesis, cells from a woman at risk of producing a child with CF showed significant dexamethasone resistance, similar to that of fibroblasts derived from lnown CF homozygotes; the other amniotic cell specimens showed dexamethasone sensitivity similar to that of normal skin fibroblasts. These data suggest that the dexamethasone resistance previously observed in skin fibroblasts may also be useful in the prenatal diagnosis of CF.

Amniotic Fluid↗

Pediatrics.

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Breast Feeding↗

Comparison of hyperactive and emotionally-behaviorally disturbed children on the Devereux Child Behavior Rating Scale: a potential aid in diagnosis.

Comparison was made between Devereux Child Behavior Rating Scale (DCB) ratings of matched groups of hyperactive and emotionally-behaviorally disturbed children. The emotionally behaviorally disturbed group was chosen to represent a population with presenting symptoms similar to hyperactivity. Ratings of the two groups exceeded the normative population on all DCB factors. The hyperactive group received higher scores on 16 factors and was significantly higher than the emotionally-behaviorally disturbed group on 8 factors. The hyperactive group received the most extreme scores on behavioral dimensions generally described as characteristic of the hyperactive syndrome. The results suggested that the DCB can assist in differentiating hyperactive children from children with presenting symptoms similar to those of hyperactivity.

Child↗

Splenic lipidosis after administration of intravenous fat emulsions.

Spleens showing fatty infiltration and necrosis of the pulp were found at necropsy on several patients who had received intravenous fat emulsions during their terminal illnesses. The postmortem findings are described and the clinicopathological correlation is discussed with special reference to the phenomenon of creaming of the emulsion.

Adult↗

Nutrition and growth.

Longitudinal growth data on children who developed obesity during childhood reveal a distinct tendency for height gain to accelerate coincident with or after the onset of excessive weight gain. The magnitude of the relative height increment is related to the degree of overweight. Overnutrition accelerates growth, just as undernutrition retards it.

Adolescent↗