摘要:

A boy with ornithine transcarbamylase (OTC) deficiency was relatively symptom free for 9 months and then developed an acute episode with liver failure and metabolic imbalance. Subsequently there was severe cerebral atrophy.Liver ornithine transcarbamylase activity was 3% of the normal mean. Of considerable interest was the finding of an accelerated breakdown of liver peroxisomes during the acute phase.

ISSN:0031-3998    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Employing a perifusion technique, the activity of hormone-sensitive lipase (HSL) in human adipose tissue and isolated adipocytes was found to be significantly stimulated by 5 μM epinephrine (5-fold,P< 0.001) or 0.5 mM theophylline (2-fold,P< 0.03). The stimulatory effect of both, however, was not cumulative; instead, theophyilline appeared to blunt the epinephrine effect. The two effectors together produced a 3-fold increase in activity over basal (P< 0.01). Basal lipolysis was highly and significantly correlated with epinephrine and/or theophylline stimulated lipolysis in the tissue as well as in the isolated cells.The activity of the enzyme was examined in cultured human adipocytes using a cell-free system. The basal activity of HSL in the 20,000 xgsupernatant (S20) fraction of cultured cells grown in fat-enriched medium was significantly higher than the value in the same fraction of cells grown in regular medium (6.06 ± 1.49versus2.78 ± 0.89 nmole glycerol/min/mg protein, mean ± S.D.,P< 0.01), and was similar in the S20fractions of cells grown in the enriched medium and the original tissue (6.06 ± 1.49versus5.44 ± 2.73 nmole glycerol/min/mg protein, mean ± S.D.,P> 0.83). When the tissue and cells were stimulatedin vitrobefore fractionation, the HSL activity in the S20fraction of the original tissue increased 4-fold over basal (P< 0.001), whereas that in the cultured cells increased 2-fold (P< 0.01) regardless of the culture medium employed. In the S20fractions derived from basally incuhated samples, but not in those derived from the epinephrine-stimulated samples, the HSL enzyme was slightly but significantly (P< 0.002) activated by exogenous addition of ATP, cyclic AMP, and protein kinase.The data suggest that the activity of HSL is retained in cultured human adipocytes and could be enhanced under conditions of culture which favor lipid accumulation and adipose conversion.

ISSN:0031-3998    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Application of a Harpenden caliper to a neonate's skinfold results in an exponential decline of the skinfold thickness, stabilizing within 50–60 sec. In order to elucidate the meaning of this decline, simultaneous measurements of skinfold thickness (SFT, by Harpenden caliper connected to a chart recorder), corrected bromide space (CBS, in ml/kg), plasma volume (ml/kg, by T-1824 dilution) and interstitial water (IW, ml/kg = CBS-plasma volume) were obtained in 18 term and 18 preterm neonates. Skinfold thickness was measured at the midtricipital (MT) and subscapular (SS) sites. The magnitude of SFT decline was estimated as the difference between 0–60 sec readings expressed in % of 0 second reading (%ΔSFT). The rate of SFT decline was estimated as the slope of the semilogarithmic plot from 4–20 sec after caliper application (SΔSFT).The 36 neonates whose birthweights ranged from 620–3700 g and whose gestational ages ranged from 27–41 wk were studied within 12 h of birth. Mean CBS, IW, %ΔMT, %ΔSS, SΔMT and SΔSS were higher in preterm than in term neonates (P< 0.001). Mean plasma volume was higher in preterm neonates (P= 0.009), but the difference disappeared after exclusion of six polycythemic term neonates from the calculations. The magnitude of SFT decline correlated well with both CBS (%ΔMTversusCBS:r= 0.71 and %ΔSSversusCBS:r= 0.71) and IW (%ΔMTversusIW:r= 0.71 and %ΔSSversusIW:r= 0.70). The rate of decline correlated moderately but highly significantly with both CBS (SΔMTversusCBS:r= 0.50 and SΔSSversusCBS:r= 0.42) and IW (SΔMTversusIW:r= 0.51 and SΔSSversusIW:r= 0.43). Exculsion of five neonates less than 30 wk in gestation improved the correlations with both %ΔSFT and SΔSFT.These data suggest that the decline in SFT measurements after caliper application results from the expression of subcutaneous IW from the skinfold and that both the amount of water expressed and the rate of its expression increase linearly with the amount of extracellular and IW in the body.

ISSN:0031-3998    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Monocytes and polymorphonuclear neutrophils of human colostrum were identified using cytochemical staining procedures. The ability of colostral phagocytes to function in antibody dependent cellular cytotoxicity was found to be satisfactory at high effector/target cells ratio (20:1) Colostral monocytes showed a diminished cytotoxicity when compared to monocytes of adult or neonatal peripheral blood at low effector/target cells ratio (1:1). This could be due to the extensive fat ingestion by these cells or other inhibitory factors found in colostrum.

ISSN:0031-3998    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Previous studies have shown that platelets exhibit a H2O2producing, NADH-dependent system that is activated by interaction with particulate material. Current evidence suggests that this system could be critically involved in the generation of chemotactic factor(s). In the present studies, chemotactic activity for polymorphonuclear leukocytes of supernatants derived from zymosan-stimulated human platelets has been evaluated using an agarose gel technique. Supernatants of opsonized zymosan-stimulated platelets showed significant chemotactic activity (migration index = 300 ± 50), in comparison with supernatants prepared from platelet suspensions stimulated with nonopsonized-zymosan (migration index = 15 ± 15) or resting platelet supernatants (migration index = 15 ± 15). Furthermore, a marked increase in chemotactic activity of the opsonized zymosan-treated platelet supernatants was demonstrated after the additon of NADH (migration index = 525 ± 100). The inclusion of specific inhibitors of the cycloxygenase and lipoxygenase pathways resulted in a marked reduction of chemotactic activity, which was restored in the presence of NADH. Further, the addition of superoxide dismutase completely abolished the chemotactic response induced by NADH. These data suggest that platelets are the source of chemotactic factor(s) derived from the activation of a superoxide generating system.

ISSN:0031-3998    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Peptic-tryptic-cotazym (PTC) digests were obtained, simulatingin vivoprotein digestion, from albumin, globulin, gliadin and glutenin preparations from hexapiod (bread) wheat as well as from diploid (monococcum) and tetraploid (durum) wheat gliadins. The digest frombreadwheat gliadins reversibly inhibitedin vitrodevelopment and morphogenesis of small intestine from 17-day-old rat fetuses, whereas all the other digests (obtained both from nongliadin fractions and from gliadins from other wheat species) were inactive.The PTC-digest frombreadwheat gliadins was also able to prevent recovery of and to damage thein vitrocultured small intestinal mucosa from patients with active coeliac disease (gluteninduced entheropathy). The PTC-digest fromdurumwheat gliadins caused a much less adverse effect on this human pathologic tissue culture system.

ISSN:0031-3998    

出版商:OVID    

年代:1982

数据来源: OVID

ISSN:0031-3998    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Fasting pancreatic glucagon was observed in Jamaican infants during malnutrition and subsequent recovery. Rehabilitation in two groups of children with isocaloric diets rich either in carbohydrate or fat produced no differences in the rate of weight gain. During malnutrition, plasma pancreatic glucagon concentration was 104 ± 11 (n= 20) pg/ml (mean ± S.E.) significantly lower than during recovery when the maximum value was 180 ± 24 (n= 13) pg/ml during the later recovery phase. After clinical recovery glucagon levels declined to 127 ± 13 (n= 15) pg/ml. Plasma insulin followed a similar pattern, increasing significantly during catch-up growth and declining after recovery. Slower rates of growth were associated with the simultaneous decline in the concentrations of both hormones after clinical recovery.

ISSN:0031-3998    

出版商:OVID    

年代:1982

数据来源: OVID

ISSN:0031-3998    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Red cell membrane proteins were investigated in two unrelated children with congenital dyserythropoietic anemia (CDA) I and two siblings with CDA II. The CDA I patients displayed globin chain synthesis imbalance, with reduction of the non α/α ratio. One of the CDA II patients presented the reverse alteration. Whenever globin chain synthesis was unbalanced, the membranep-nitrophenylphosphatase had an abnormally biphasic kinetics, consistent with substrate excess inhibition, as is observed in α- or β-thalassemic syndromes. One CDA I patient displayed a decrease of electrophoretic band 4.1 along with an ectopic phosphorylated protein at the level of band 4.2. In CDA II patients, band 3 was strikingly narrower than in controls. In CDA II and, to a lesser extent, in CDA I, thein vitroendogenous phosphorylation of band 2 + 2.1 was sharply reduced.

ISSN:0031-3998    

出版商:OVID    

年代:1982

数据来源: OVID