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Biomedical subjects

J E Collins

Publications and source records attributed to J E Collins.

At least 127 records · Page 7Linked to original sources

Study of liver metabolism in glucose-6-phosphatase deficiency (glycogen storage disease type 1A) by P-31 magnetic resonance spectroscopy.

Liver metabolism of two patients (aged 15 and 23 yr) was studied by P-31 magnetic resonance spectroscopy at 1.9 tesla. The P-31 spectra of liver showed the resonances of phosphomonoesters (including sugar phosphates), inorganic phosphate (Pi), phosphodiesters (e.g. glycerophosphorylcholine, glycerophosporylethanolamine), and ATP. These resonances were quantified by expressing their peak areas in mM (assuming that ATP concentrations in normal liver is 2.5 mM) or as a ratio relative to the area of the phosphodiester resonance. After an overnight fast liver phosphomonoesters in patients were 2.6 and 1.6 AU, respectively (controls 1.1 +/- 0.5, mean +/- 2 SD, n = 17). At the same time liver Pi was decreased in patients to 1.3 and 1.0, respectively (controls 1.8 +/- 0.8). Based on chemical shift measurements the increase in phosphomonoesters could be attributed to accumulation of sugar phosphates (mainly glycolytic intermediates). After 1 g/kg oral glucose, hepatic sugar phosphates decreased in patients by 64 and 40%, respectively, and reached normal levels (on the absolute intensity scale); whereas liver Pi increased by 130 and 40%, respectively. Liver Pi levels remained elevated in both patients 30 min after ingestion of glucose. Liver sugar phosphates and Pi did not change in control subjects (n = 4) after glucose. In contrast to some previous reports, we have found accumulation of glycolytic intermediates in the liver of glucose-6-phosphatase-deficient patients during fasting. In these patients high levels may enhance the activity of residual glucose-6-phosphatase thus increasing hepatic glucose production and reducing the degree of hypoglycemia during fasting.(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Oral↗

Characterization of canine adenovirus type 1 isolated from American black bears.

Viruses recovered from tissues taken at necropsy from American black bears were examined by use of immunofluorescence with polyclonal and monoclonal antibodies, virus neutralization with monoclonal antibodies, and restriction endonuclease analyses of the viral genomes. With these techniques, viruses were determined to be canine adenovirus type 1. Seronegative dogs that were inoculated with the virus had clinical signs typical of infectious canine hepatitis, suggesting that the virus, which was virulent for bears, was not a vaccinal strain, but a wild strain of canine adenovirus type 1.

Adenoviridae Infections↗

Study of hereditary fructose intolerance by use of 31P magnetic resonance spectroscopy.

The effect of fructose on liver metabolism in patients with hereditary fructose intolerance (HFI) and in heterozygotes for HFI was studied by 31P magnetic resonance spectroscopy (31P-MRS). In patients with HFI (n = 5) ingestion of small amounts of fructose was followed by an increase in sugar phosphates and decrease in inorganic phosphate (Pi) in the liver that could be detected by 31P-MRS. 31P-MRS could be used to diagnose fructose intolerance and to monitor the patients' compliance with a fructose-restricted diet. In heterozygotes (n = 8) 50 g fructose given orally led to accumulation of sugar phosphates and depletion of Pi in the liver. Fructose also induced a larger increase in plasma urate in heterozygotes than in control subjects. The effect of fructose on liver Pi and plasma urate was most pronounced in heterozygotes with gout (n = 3). Heterozygosity for HFI may predispose to hyperuricaemia.

Carbohydrate Metabolism, Inborn Errors↗

Reactions of would-be helpers whose offer of help is spurned.

An experiment was conducted to test the proposition that rejection of help by a needy recipient constitutes a negative expectancy violation for the would-be helper. The helper will react to this self-threat by expressing negative affect, unfavorable evaluations of the recipient, low attraction to the recipient, and causal attributions for the rejection that cast the recipient in an unflattering light. To test these hypotheses, college freshmen were prompted to offer rules for word construction to a same-sex recipient (a confederate) who was described as needing remediation on vocabulary and who "failed" a practice task. After rejecting/accepting the help, the recipient failed or succeeded on a comparable task. The results were consistent with these predictions. Dispositionally high expectations of interpersonal success served to amplify helpers' reactions to rejection/acceptance.

Adult↗

Effect of insulin on leucine kinetics in maple syrup urine disease.

Leucine turnover was measured using [1-14C] L-leucine in three patients with classical maple syrup urine disease. There was measurable leucine oxidation although it was lower than in normal adults. Leucine production rate was greater than normal in all three patients with an increased rate of incorporation of leucine into protein and increased protein catabolism. These fluxes were both relatively insensitive to exogenous insulin.

Child↗

Hepatic glycogen storage disease.

The management of patients with glycogen storage disease is based on an understanding of the biochemistry. The outlook for many patients has improved so that they can now grow up normally.

Glycogen Storage Disease↗

Experimental infection and abortion of pregnant guinea pigs with a unique spirillum-like bacterium isolated from aborted ovine fetuses.

Study was made of the pathogenicity of a spirillum-like, anaerobic, gram-negative bacterium, originally isolated from aborted lambs, for pregnant guinea pigs. Reproducible conditions for propagation and preservation of the bacterium were determined as requisite for the preparation of cultures for animal inoculation. A preliminary experiment was done with 10 pregnant guinea pigs to test for an infective dose of organisms that would produce abortion. High-passage cultures (n = 50) were used to inoculate these guinea pigs intraperitoneally. Six of 10 guinea pigs aborted, and the organism was cultured from fetal tissues of 5 guinea pigs. Isolates from 3 of the 6 guinea pigs were propagated through 4 passages on blood agar and used to infect 3 groups, each of 5 guinea pigs. A 4th group of 5 guinea pigs was inoculated with the original culture. Three of 5 animals in the first 3 groups, which had been given the low-passage cultures from the preliminary trial, and 2 of 5 guinea pigs in the 4th group, which had been given the original culture, aborted. Antibody against the spirillum was detected in 19 of 30 inoculated guinea pigs. The major microscopic lesions were acute suppurative placentitis and splenitis. This bacterium retained pathogenic properties sufficient to cause infection, abortion, and microscopic lesions in two-thirds of the guinea pigs, in spite of high in vitro passage. The organism has unique ultrastructures, and its genus and species are yet to be determined.

Abortion, Veterinary↗

Porcine abortion caused by Bacillus sp.

Bacillus sp is known to cause abortion in cattle and sheep, but it is rarely reported to cause abortion in swine. Bacillus sp was isolated from aborted fetuses, and lesions compatible with a bacterial infection were detected in 4 of 962 aborted porcine fetuses examined. The infection apparently results in sporadic abortions and is not likely to cause economically significant reproductive failures within a herd.

Abortion, Septic↗

Infection of gnotobiotic pigs with an Escherichia coli O157:H7 strain associated with an outbreak of hemorrhagic colitis.

Gnotobiotic pigs inoculated with an Escherichia coli O157:H7 strain isolated from a human with hemorrhagic colitis developed anorexia, lethargy, and watery diarrhea. Bacteria diffusely colonized the cecum and colon surfaces and the crypt epithelium. At bacterial attachment sites, microvilli were effaced, and epithelial cells were irregularly shaped, rounded, or detached. Submucosa, lamina propria, and mesentery were markedly edematous and contained many inflammatory cells.

Adhesiveness↗

Circulating immune complexes and immunoglobulin A rheumatoid factor in patients with mesangial immunoglobulin A nephropathies.

Circulating immune complexes (CIC) containing IgA and C3 were elevated in 48% of IgA nephropathy patients; IgA1 was the predominant subclass. IgA1-IgG CIC were detected in 44%, IgA2-IgG CIC in 7%, and IgM-IgA1 CIC in 16% of the patients. No IgM-IgA2 CIC were detectable. Sucrose gradient ultracentrifugation indicated that IgG-IgA1 CIC were predominantly of intermediate (13-19S) size whereas IgA1-C3 CIC sedimented from 11S to 19S. At acid pH, isolated CIC revealed the presence of substantial amounts of 7S IgA. One third of the patients had elevated serum IgA rheumatoid factor (RF) of both polymeric and monomeric forms despite normal levels of IgM-RF; 87% of patients with elevated IgA-RF had IgA1-IgG CIC. These results indicate that the IgA1 component of CIC in patients with IgA nephropathy is not necessarily of mucosal origin and suggest that a portion of these CIC consists of IgA RF immunologically complexed with autologous IgG.

Antigen-Antibody Complex↗

Abortion in sheep caused by a nonclassified, anaerobic, flagellated bacterium.

Twenty-eight pregnant ewes were inoculated IV with approximately 6 X 10(8) nonclassified, anaerobic, flagellated bacteria (NAFB) that had been isolated from an aborted lamb. Abortion occurred in 3 of the ewes and 1 ewe gave birth to a weak lamb. The remaining 24 ewes and 3 other ewes inoculated orally with NAFB did not develop clinical signs of illness. Suppuration and vasculitis were seen in the placentas of the 3 aborted lambs, 1 of which had necropurulent hepatitis indistinguishable from that usually attributed to Campylobacter fetus infection. The NAFB was isolated from fetal placenta, abomasal content, or internal organs of 2 aborted lambs and the weak lamb. A morphologically similar organism was seen in the abomasal content of the other aborted lamb, but the organism did not grow on bacteriologic culture medium. Therefore, in susceptible pregnant ewes, NAFB can cause fetal placentitis and hepatitis and subsequent birth of weak lambs or abortion.

Abortion, Septic↗

Ovine abortion associated with an anaerobic bacterium.

An anaerobic, slightly curved, tapered, corrugated, rod-shaped bacterium with O to greater than 12 flagella at each pole was isolated from 2 aborted lambs with focal hepatic necrosis. The organism stained faintly Gram-negative, but stained better by the Giemsa method. It was pleomorphic in culture, ranging from filaments containing granules to faintly staining spheroids.

Abortion, Veterinary↗

Biological potency of porcine, bovine and human insulins in the rabbit bioassay system.

Potency determination of porcine, bovine and human insulins relative to the International Standard in the pharmacopoeial rabbit bioassay system requires that the log-dose response curves are parallel. Furthermore, the same relative potency should be obtained independent of how the hypoglycaemic response is defined. The results of 508 rabbit blood glucose assays have been analyzed by new multivariate statistical methods. No deviations from parallelism of the log-dose response curves were detected. However, the potencies showed significant variation depending on the blood sampling times. Pure porcine and human (semisynthetic and biosynthetic) insulin potencies decreased by 12% and 18%, respectively, from the 30-min to the 2.5-h response, whereas bovine insulin potencies increased by 9%. Since the standard is a 52:48 mixture of bovine and porcine insulins, these results could be due to porcine and human insulins having a quicker onset and shorter duration of hypoglycaemic effect than bovine insulin. This was confirmed in assays of bovine relative to porcine insulin and by direct comparison of mean blood glucose curves. It is concluded that there is a response time-dependent variation in potency when the test and standard insulin have a different species composition. Hence, pure species insulin standards - a porcine, a bovine and a human standard - are needed for assay of the three insulins.

Animals↗

Is the sweat test infallible in cystic fibrosis?

The diagnosis of cystic fibrosis is straight forward if the clinical findings and sweat sodium are considered together. We describe 3 patients where the diagnosis has proved difficult as both the clinical features and sweat sodium have varied between normal and abnormal.

Cystic Fibrosis↗

The dietary management of inborn errors of metabolism.

Many inborn errors have now been described that can be treated by alterations in diet. Such treatment requires an understanding of both the biochemistry of the defect and of normal nutritional requirements. The principal strategies are cofactor therapy, steps to prevent accumulation of toxic metabolites and the replacement of essential nutrients that are deficient as a result of the metabolic block. It is essential to make sure that any diet used for more than a brief period is complete and capable of sustaining normal growth and development. The treatment of disorders of carbohydrate and amino acid metabolism including organic acidaemias and disorders of the urea cycle and of fat oxidation are discussed.

Acute Disease↗