摘要:

SummaryThe relationship between the activity of the cystic fibrosis serum ciliary dyskinesia factor, detected by the rabbit tracheal bioassay, and clinical status of the patient has been investigated in children, 1–24 years old, with cystic fibrosis. No significant correlation was found between the amount of serum factor activity and age, clinical status assessed by the Schwachman score, pulmonary function (vital capacity, functional residual capacity, total lung capacity, residual volume, maximum breathing capacity, maximal midinspiratory flow, and maximal midexpiratory flow) or blood gas levels (pCO2, pO2). The activity showed no significant relationship to serum galactosyltransferase activity in children with cystic fibrosis.SpeculationThe substance responsible for the ciliary dyskinesia serum factor activity detected in children with cystic fibrosis may not be directly involved in the clinical syndrome or in its pathophysiology.

ISSN:0031-3998    

出版商:OVID    

年代:1979

数据来源: OVID

摘要:

SummaryJejunal biopsies obtained from 45 children referred for a variety of clinical conditions were examined histologically and by standard immunofluorescence methods. The number of plasma cells was recorded per “mucosal tissue unit.” The results showed that most of the immunoglobulin A (IgA) counts in infants under 3 yr with normal mucosa were low and that there was a rise in the number of IgA plasma cells starting after this age. Despite this, under pathologic conditions (partial villous atrophy with increased inflammatory cell infiltration - PVA) a significant rise in IgA plasma cells occurred in all age groups. Patients with coeliac disease tended to show the highest IgA as well as IgM plasma cell counts. In all other patients, IgM counts did not change significantly with age or histologic abnormalities.SpeculationChildren in the first few months of life are as capable as older children of increasing the number of IgA plasma cells in their jejunal mucosa. However, though immunocompetent, their gut, on first contact, will be lacking in primary immune responses to the various antigens, and, therefore, may be relatively unprotected.

ISSN:0031-3998    

出版商:OVID    

年代:1979

数据来源: OVID

摘要:

SummaryStrains ofEscherichia coliwere opsonized in human colostrum via heat stable opsonins and the classic complement pathway, but colostrum lacked capacity to opsonizeE. colivia the alternative pathway. There was no bacteriostatic activity against serum sensitiveE. colistrains, although specific antibodies against the strains were present. Neutrophils suspended in colostrum had normal chemotaxis and this was not altered by treating the colostrum with HCI.SpeculationPossibly the migration of neutrophils to the intestinal wall and lumen in response to cytotaxic factors may be enhanced by colostrum. Milk derived opsonins may enhance phagocytosis. Human breast milk may, thus, play a role in the defense against bacteria in the gut of the nursed infant and locally in the mammary gland by promoting neutrophil chemotaxis and phagocytosis.

ISSN:0031-3998    

出版商:OVID    

年代:1979

数据来源: OVID

摘要:

SummaryPlasma renin activity was determined in 25 healthy, full-term, newborn infants aged 1 day to 9 weeks. High values were found, the mean level at 1–2 days of life (24.8 ± 8.4 ng/ml/hr, SE) being significantly higher than the mean levels at 7–9 days (5.8 ± 1.5) and at 4–9 weeks (8.1 ± 1.3) (P< 0.05). No correlation was found between plasma renin activity and systolic blood pressure, hematocrit, creatinine clearance, serum sodium, or serum potassium. Plasma renin activity (log values) was inversely correlated with sodium intake (r= −0.58) or with urinary sodium (r= −0.44), and positively with urinary osmolality (r= 0.67). The correlations reached higher coefficients if only infants aged ≤ 9 days were considered.In addition, vasopressin was measured by radioimmunoassay in the urine. The daily excretion was lower in newborn infants (9.4 ± 1.6 ng/m2/day, SE, at 1–2 days of postnatal life) than in healthy children (37.1 ± 5.6), and was significantly correlated with creatinine clearance (r= 0.69), but not with urinary osmolality.SpeculationThe postnatal changes of plasma renin activity were significantly correlated only with parameters of sodium balance and with urinary osmolality, so that one can speculate that early after birth, the renin-angiotensin system plays a preponderant role not only in sodium homeostasis, but also in water balance.

ISSN:0031-3998    

出版商:OVID    

年代:1979

数据来源: OVID

摘要:

SummaryWe report some properties of propionyl CoA carboxylase (PCC) partially purified from cultured human fibroblasts obtained from controls and several patients with propionic acidemia. A series of steps (Triton X-100 treatment, high speed centrifugation, ammonium sulfate precipitation, and density gradient centrifugation) led to 100− to 300− fold purification of control enzyme. Control PCC had a molecular weight of nearly 700,000, contained biotin, demonstrated a pH optimum at 8.0–8.5, was activated by potassium, and followed Michaelis-Menten kinetics for each of its substrates. It was distinguished from acetyl CoA carboxylase immunologically as well as by differential purification.Each of seven lines from patients with propionic acidemia had clearly detectable PCC activity which was less than 5% of that in control lines. Although yields were poor and purification less extensive than in control lines, mutant PCC was enriched 2− to 40-fold by the same procedures employed for the control enzyme. Mutant enzyme had a pH optimum, ionic requirements, and substrate Km's similar to those of control PCC, but was distinctly more labile to both cold and heat. These findings suggest that the markedly reduced activity of PCC in these patients results from a mutation in the PCC structural gene locus or loci which leads to the synthesis of altered enzyme protein molecules.SpeculationOur results suggest that human propionyl CoA carboxylase has properties very similar to those of other mammalian propionyl CoA carboxylases. Further chemical studies with control enzyme and that from patients with propionic acidemia may help define the basis for the two major genetic complementation groups reported among propionyl CoA carboxylase deficient patients.

ISSN:0031-3998    

出版商:OVID    

年代:1979

数据来源: OVID

摘要:

SummaryIncorporation of labeled glucose and fatty acid residues into saturated phosphatidylcholine was significantly reduced in lung slices from 27.5 days of gestation fetal rabbits during 90 min incubation in the presence of 100 μU/ml insulin. When14C-glucose was used as substrate, incorporation into both phosphatidylcholine and saturated phosphatidylcholine was reduced by insulin. This occurred despite an increase in overall glucose utilization by the lung from 11.3 ± 3.9 to 16.3 ± 5.2 nmole/g tissue in the presence of insulin (P< 0.05). A decrease in incorporation of fatty acid residues into saturated phosphatidylcholine was also observed when14C-paImitate was used as substrate, from 102 ± 4 to 90 ± 5 nmole palmitate/g tissue (P< 0.01). In the presence of insulin, there were significant reductions of both substrates appearing in lysophosphatidylcholine, a precursor of saturated phosphatidylcholine. There was no significant change in incorporation of glucose residues into glycogen or lactate under these conditions.SpeculationHyperinsulinemia appears to be responsible for respiratory distress syndrome (RDS) in infants of diabetic mothers (IDM) and infants of gestational diabetic mothers (IGDM). Control of the maternal diabetic state to a degree which inhibits development of fetal hyperinsulinism reduces the incidence of RDS in IDM and IGDM by removing excess quantities of the factor, insulin, that is responsible for inhibition of surfactant production.

ISSN:0031-3998    

出版商:OVID    

年代:1979

数据来源: OVID

摘要:

SummarySalivary aldosterone measurements can be used to study aldosterone secretion and metabolism noninvasively. Salvia and plasma aldosterone concentration were highly correlated during all periods of study; during adrenocorticotrophic hormone (ACTH) administration, a relatively greater amount of aldosterone appeared in saliva; during dexamethasone administration, a relatively smaller amount of aldosterone appeared in saliva.As plasma cortisol increased with ACTH administration there was an increased relative amount of aldosterone in saliva. DOC (11-deoxycorticosterone) was found in saliva only during ACTH administration. Corticosterone was identified during baseline periods. Salivary aldosterone concentrations were independent of flow.The data indicate that saliva is an accessible bodily fluid which may be used to monitor the changes in nonprotein bound plasma steroid hormone concentration in children.SpeculationSaliva is an accessible bodily fluid which may be used to monitor the changes in nonprotein bound plasma aldosterone concentration and other steroidal hormones.

ISSN:0031-3998    

出版商:OVID    

年代:1979

数据来源: OVID

摘要:

SummaryLung lavage samples from rats treated in a chronic fashion with reserpine had mean increases of 133, 170, and 120% in the total protein, lipid, and carbohydrate contents, respectively, when compared with those of untreated control animals, when these values were expressed in terms of body weight. An acute single ip injection of the β-adrenergic agent isoproterenol (10 mg) increased the glycoprotein content of pulmonary lavage fluid from control rats approximately 15–19%, but only 9–10% in those from reserpine treated rats. By contrast, pilocarpine (10 mg), administered in the same manner, caused a 15% increase in the total protein, carbohydrate, and lipid content of lavage samples from control rats and increased these same constituents in the lavage samples of reserpine treated rats 98, 102, and 80%, respectively. The increased total carbohydrate content in the lavage samples of the treated animals was not associated with changes in the percent distribution of neutral sugars, amino sugars, or sialic acid. The ratio of these various sugar components did not change in the lavage samples of control or reserpine treated rats upon stimulation with either pilocarpine or isoproterenol. The increased total lipid content fround in the lavage samples from the treated rats probably results from an increase in phospholipids. A decrease in phospholipid content occurred in the lavage samples of reserpine treated rats upon stimulation, while the opposite was observed in those of control animals. Chronic treatment of rats with reserpine, thus, appears to induce an enhanced production of glycoproteins in the airways and to interfere with phospholipid metabolism in the lung. In addition, the drug treatment enhances the secretory response to pilocarpine in comparison with the responses of control animals. The enhanced response or hypersecretion of glycoproteins is a quantitative one and does not seem to involve alterations in the ratios or distribution of the various sugar components. This disturbance in the secretory function of the respiratory tract, a target organ which is prominently involved in cystic fibrosis (CF) together with alterations in other exocrine glands which resemble those of CF patients, makes the reserpine treated rat a useful model for the further study of possible pathogenetic mechanisms in CF.SpeculationThe experimental animal model for CF developed by the chronic administration of reserpine to rats has been found to have changes in the protein content of lung lavage samples, in addition to morphologic and secretory alterations in the salivary glands and the pancreas. Further analysis of the organic composition of lung lavage samples shows that the protein, carbohydrate, and lipid contents are significantly increased after chronic reserpine administration and that the response to stimulation with pilocarpine is enhanced in the treated animals when compared to that of untreated control rats. It therefore appears that chronic reserpine administration causes a hypersecretion of glycoproteins and changes in lipid metabolism in the respiratory tract of the rat and that, in conjunction with previous findings in other exocrine glands, these effects on lung function make the reserpine treated rat a useful tool for the study of the pathologic disturbance seen in the major exocrine glands affected in CF.

ISSN:0031-3998    

出版商:OVID    

年代:1979

数据来源: OVID

摘要:

SummaryThe importance of intracellular calcium (Ca) in secretion and transmembrane ion movement led us to study Ca in cells from patients with cystic fibrosis (CF) which is a lethal genetic exocrinopathy. Skin fibroblasts from patients with CF, obligate heterozygotes (HZ), and age- and sex-matched controls (C) were used in matched pair experiments measuring45Ca exchange into and efflux from the cells over time. CF cell lines and HZ cell lines exhibit increased45Ca exchange when compared with their respective controls (P< 0.005). The magnitude of this difference (approximately 30%) is not reduced when cells are washed with lanthanum chloride after the exchange period. This difference is likely attributable to an altered capacity of one or more of the intracellular Ca sequestering organelles. Further evidence for this explanation was seen in46Ca efflux experiments in which CF cells retained a higher percent of their initial 0-time45Ca than did C cells late in the efflux period (P< 0.05). The finding of an altered Ca pool size in both CF and particularly HZ cells suggests that altered Ca metabolism is related to the basic gene defect in CF.SpeculationIncreased intracellular Ca in CF cells, while necessarily secondary to the basic gene defect, may influence cellular metabolism sufficiently to be a basis for many events in the pathogenesis of the disease. The presence of the Ca pool size alteration in cells from obligate heterozygotes is evidence that this phenomenon is closely related to the basic gene defect.

ISSN:0031-3998    

出版商:OVID    

年代:1979

数据来源: OVID

摘要:

SummaryIgG-coated liposomes containing glucose oxidase (GO) in order to provide a means of generating H2O2were prepared. Leukocytes from patients with chronic granulomatous disease (CGD) which are lacking a means of generating H2O2, ingested the IgG-coated liposomes and metabolic oxidative deficiencies of glucose-1-14C oxidation and iodination were normalized. Both of these activities have been shown to depend upon the availability of H2O2within polymorphonuclear leukocytes. Improvement in the capacity to killStaphylococcus aureusby chronic granulomatous disease leukocytes were observed under similar conditions. Thus, it is possible to restore the oxidative metabolic capacities to CGD leukocytes by the introduction of glucose oxidase containing liposomes to these cells.SpeculationIt is possible to restore the oxidative metabolic capacities and to obtain moderate improvement in the bactericidal functionsin vitroof CGD leukocytes by providing the cells ingestible liposomes containing GO. Further studies might define more efficient methods for introducing oxidase activity into these cells, or employing drugs that generate H2O2.

ISSN:0031-3998    

出版商:OVID    

年代:1979

数据来源: OVID