Biomedical subjects
Publications and source records attributed to D A Applegarth.
alpha-Galactosidase from human placenta was actively taken up by cultured fibroblasts from patients with Fabry's disease whereas the enzyme from the fig was not absorbed. alpha-Glucosidase from two microbiological sources. A. fumigatus and A. niger, was not taken up by cultured fibroblasts from patients with Pompe's disease.
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We report the identification of delta 1-pyrroline 3-hydroxy 5-carboxylic acid (hydroxy PCA) in a previously reported patient with hyperprolinemia Type II. This compound had been called pseudo PCA in a previous report.
1. Between 1971 to 1977, 74,521 urines, collected on filter paper and mailed in, were screened by the Metabolic Screening Program of the Children's Hospital. These represented 45.9% of live births in B.C. hospitals were the program has been available. The mean age of the infants was 4.4 weeks. Urines were examined by chromatography with ethyl acetate-pyridine-water for sugars. 1423 (2.13%) had an abnormal pattern necessitating a repeat urine card. A persistent abnormality was noted in 167 (0.22%) and from these a liquid urine sample was obtained for two dimensional amino acid chromatography and/or a repeat sugar chromatography. 2. In 47 (0.06%) of these a definite metabolic abnormality was confirmed. These included cases of Iminoglycinuria (8), Hartnup trait (4), Nonketotic hyperglycinemia (2), Histidinemia (1), Cystathioninuria (5), Argininosuccinic aciduria (1), Maple Syrup Urine Disease (1), Diabetes Mellitus (1), Renal glycosuria (1) and Persistent galactosuria (3). 201 infants had a slight increase of cystine and/or lysine, and 19 of these were documented to be heterozygous for cystinuria.
From data collected at the Children's Hospital, Vancouver, during a two year period, 1976 - 1977, we have determined a range of values for children aged 3 months to 6 years for 24 plasma amino acids and for children aged 3 months to 10 years for 18 cerebrospinal fluid amino acids. A total of 26 plasma and 21 cerebrospinal fluid samples were accepted for our reference population. Six of the children in the plasma group were also in the cerebrospinal fluid group, making a total of 39 children in the study.
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We have assessed the vitamin B6 status of 40 nonpregnant women of reproductive age, 30 pregnant women, 20 postpartum, not depressed, women and 24 postpartum, depressed women by means of the erythrocyte glutamate-oxaloacetate transaminase activation test (alpha EGOT). The level of mental depression was evaluated in the nonpregnant controls, the postpartum controls and the postpartum, depressed patients by the Beck Depression Inventory and the Depression Adjective Check Lists. The results of the alpha EGOT did not indicate any significant differences between the postpartum, depressed patients and any of the control groups. The Beck and Depression Adjective Check Lists scores were significantly higher in the postpartum depressed patients than in the postpartum controls or nonpregnant controls. On the basis of this study, there is no evidence for vitamin B6 deficiency in women suffering from postpartum depression.
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Culture medium from fibroblasts of cystic fibrosis patients and controls was examined for the ability to inhibit (calcium plus magnesium)-activated ATPase and (sodium plus potassium)-activated ATPase. The ATPase systems used were both a solubilised preparation from dog-fish and a membrane associated preparation from human erythrocyes. Contrary to other reports the medium from cystic fibrosis fibroblasts did not inhibit ATPase activity.