SummaryBiotin responsive multiple carboxylase deficiency was suspected in a third trimester conceDtus on the basis of enzvmatic confirmation in fibroblasts cultured from an earlier sibling who suffered a demise in the immediate neonatal period. Maternal urinary organic acid profile was normal throughout the final 4 wk of pregnancy. Oral administration of biotin, 10 mg/day to the mother resulted in a 100-fold increase in urinary biotin excretion within 7 days. Urinary biotin excretion over the subsequent 2 wk decreased steadily, suggesting either decreased maternal absorption or increased fetal sequestration.After the birth of nonidentical twins, cord blood and urinary organic acid profiles of the infants were normal. However, cord blood biotin concentration was 4 to 7-fold that of normal newborns.
Fourteen insulinopenic diabetics were treated with a closedloop Biostator Glucose Controller for 24-48 hours followed by 7-10 days of continuous subcutaneous insulin infusion (CSII) in 12 patients. Percent distribution of pre-meal insulin during optimal control with CSII was predicted by the Biostator (r=0.66, slope = 0.89 x intercept=0.24, P<.001). control during closed-loop feadback, expressed as mean of pre-meal, peak and two hour post meal blood glucose was correlated with HbAlC prior to control (r=0.67, P<.Ol).During CSII insulin requirements decreased significantly 19f 4.4% MfSE, (Pc.025). The decrease in insulin was accompanied by good glucose control: lllf6.9 vs 100f4.3 mgldl. In the 3 patients displaying the largest decrease in insulin requirements (25-44%) during CSII, initial HbAIC (13.5f1.3%) was greater than in patients whose requirement decreased less (HbAIC 10f.86%, P<.05).Conclusions 1) Distribution of insulin for-programming of CSII can be established with a closed-loop insulin delivery system and may shorten the time required to achieve optimal metabolic control. 2) Metabolic control during closed-loop insulin delivery is influenced by the previous state of control as assessed by HbAlC levels. 3) Over 7-10 days of tight metabolic control with CSII, insulin requirements decreased, with the greatest decrease occurring in patients who have been in poor control. Although lipolysis and ketogenesis increase in fasting GHdeficient children, growth hormone is required for these processes to occur optimally. EFFECT OF HUMAN GROWTH FAT-DERIVED FUELS DURING 24-HOUR STARVATION INCHILDREN. Joseph I. Wolfsdorf, &do1 lah Sade_g-hk ' l 9 l -, and Gris Senior. Tufts University School of Medicine, New Enqland Medical Center, Boston.~uring prolonged fasting, adults show a gradual rise in the blood levels of free fatty acids (FFA) and betahydroxybutyrate (BOHB) . In children, by contrast, ketonuria is frequently present after a relatively short fast and is often viewed as an indication of a pathological process. Despite the importance of fat-derived fuels, there is a surprising scarcity of information concerning blood levels of FFA and ketones in fasting children of various ages. We studied the metabolic response to a 24-hour fast in 23 normal children (mean age=8.3, range 1.9-16.7 years). TIME FFA* BOHR* GLUCOSE* hrs mM mM --mM mean'!'^.^. .27'0.70 There was a rapid and progressive rise in FFA and BOHB concomitant with a fall in glucose. The concentrations achieved at 24 hours were comparable to those seen in fasting adults after about one week. There was an inverse correlation (r=0.701; p<0.001) between fasting BOHB levels and age.We conclude that moderate ketonemia and ketonuria in children after a relatively brief period of food deprivation is a normal physiologic response. The assessment of ketonuria should depend on quantitative blood assays interpreted according to age. A 25y.o. G4P3Ab. woman who had previously delivered at least one and possibly two infants affected with a holocarboxylase synthetase defi...
The general disposition of [14C]-Pranolium Chloride (SC-27761), a potential anti-arrhythmic agent, has been studied in the beagle dog, baboon and rhesus monkey. The compound was moderately absorbed from the gastro-intestinal tract of the three species at 5 mg/kg. There was appreciable inter-animal variation in the amount of absorption, and the absorption was dose-dependent in the monkey. After i.v. dosage the radioactivity was largely cleared via the kidneys. The initial elimination half-lives for Pranolium in the dog and primate were between 0.6 to 3.1 hours after i.v. dosage, but could not be determined after oral dosage. Less than 1% of the dose was localised in monkey fetal tissues, two hours after an i.v. dose was given to pregnant female rhesus monkeys, and the highest concentrations of radiolabel were detected in fetal liver. Pranolium was found to be extensively metabolised and 1-naphthol was identified as a major metabolite. Pranolium was excreted in urine both unchanged and as conjugates, but 1-naphthol was excreted largely as conjugates.
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