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Biomedical subjects

R C Stern

Publications and source records attributed to R C Stern.

At least 127 records · Page 7Linked to original sources
PubMed1978

Lymphocyte responsiveness to Pseudomonas aeruginosa in cystic fibrosis: Relationship to status of pulmonary disease in sibling pairs.

Lymphocyte proliferative responses to Pseudomonas aeruginosa and Staphylococcus aureus were evaluated in six sibling pairs with cystic fibrosis. In each pair, one sibling had advanced clinical disease, whereas the other sibling was in good clinical condition. Three in this latter group had no clinically apparent Pseudomonas bronchitis. In all cases, the average responses to Pseudomonas isolated from each sibling pair were lower in the sibling with advanced clinical disease. This difference was not observed in the responses to Staphylococcus. Normal plasma or plasma from patients with CF in good clinical condition does not restore the responses in patients with advanced clinical disease. However, plasma from patients with low or no responses to Pseudomonas inhibits the responses of responding siblings. A progressive specific lymphocyte unresponsiveness to Pseudomonas may play an important role in the increasing destructiveness of chronic pulmonary Pseudomonas infection in cystic fibrosis.

Adolescent↗
PubMed1978

Abnormal platelet aggregation in severe combined immunodeficiency disease with adenosine deaminase deficiency.

The platelets from a boy with adenosine deaminase deficiency-severe combined immunodeficiency disease (ADA-SCID) showed markedly subnormal aggregation in response to collagen and ADP. In contrast with normal platelets, ATP, AMP and adenosine had little effect in inhibiting ADP-induced aggregation of ADA-SCID platelets. These observations suggest that altered adenosine metabolism exists in ADA-SCID platelets.

Adenosine Deaminase↗
PubMed1978

Treatment and prognosis of lobar and segmental atelectasis in cystic fibrosis.

Lobar atelectasis occurred in 30 (4.1 per cent) and segmental atelectasis occurred in 6 (0.8 per cent) of 728 patients with cystic fibrosis. The right lung was involved in all 11 patients less than 5 years of age and in 18 of 26 episodes in 19 older patients. Five of the younger patients died within 5 years of the initial episode. Bronchoscopy (with or without local lavage) performed in addition to intensified medical therapy did not appear to improve the outcome. Future studies of therapeutic measures (including bronchoscopy) for treatment of atelectasis complicating cystic fibrosis must include appropriate control patients who receive only medical therapy.

Adolescent↗
PubMed1978

Cystic fibrosis: the prognosis for five-year survival.

Statistical discriminant analysis is applied to 41 concomitant variables obtained during the first year of study of 226 patients with cystic fibrosis. A discriminant function based on six variables is developed which can be used as a predictive index. This index estimates the probability of 5-year survival for an individual patient and can also be used to classify patients into one of two groups: (1) will live for 5 years or (2) will die within 5 years. Speculation A classification rule for cystic fibrosis is presented which correctly assigns 90% of the 5-year survivors and 84% of those who expire. Such a classification scheme is useful for clinical and research purposes.

Adolescent↗
PubMed1978

Pulmonary function and morbidity in 40 adult patients with cystic fibrosis.

Pulmonary function and cardiopulmonary complications were studied in a group of 40 patients with cystic fibrosis who reached the age of 25 years. Mean values for vital capacity (VC), functional residual capacity, residual volume (RV), the ratio of RV over total lung capacity (RV/TLC), conductance, and the ratio of the forced expiratory volume in one second over VC were abnormal. There was a variable pattern of progression from patient to patient. The men differed from the women only in that they had a significantly larger TLC and inspiratory capacity than the women. The resultant preservation of VC may have an advantage for survival in those patients in whom it is observed. Pseudomonas aeruginosa was encountered with increasing frequency with age. Massive hemoptysis did not result in early death. The occurrence of rightsided heart failure secondary to cor pulmonale, with or without respiratory failure, was a poor prognostic sign.

Adolescent↗
PubMed1977

The effect of early diagnosis and treatment in cystic fibrosis: a seven-year study of 16 sibling pairs.

Data on 16 sibling pairs with cystic fibrosis were analyzed to test the hypothesis that early treatment of this condition improves prognosis. Younger siblings' conditions were diagnosed before 1 year of age, usually before the onset of pulmonary disease. Older siblings' conditions were diagnosed after 1 year of age and after the onset of pulmonary disease. Although the sibling pairs received similar treatment, comparison at 7 years of age showed that the younger siblings had significantly better chest roentgenogram scores, total clinical scores, residual lung volumes, and ratios of residual volume to total lung volume. Younger siblings also required fewer hospital admissions to control their lung disease. The results suggest that, in general, early initiation of therapy is beneficial for patients with cystic fibrosis.

Child↗
PubMed1977

Cellular immunity to bacteria: impairment of in vitro lymphocyte responses to Pseudomonas aeruginosa in cystic fibrosis patients.

Lymphocyte responses to the mitogens phytohemagglutinin and concanavalin A and to Streptococcus pyogenes, Staphylococcus aureus, Haemophilus influenzae, and Pseudomonas aeruginosa were evaluated in patients with cystic fibrosis and in normal individuals. Lymphocyte proliferation in vitro was stimulated by gentamicin-killed whole bacteria, and the proliferative response was measured by [3H]thymidine incorporation. The in vitro lymphocyte responses to antibiotic-killed bacterial reached maximum thymidine incorporation after 5 days in culture and followed a unimodal dose-response curve for each of the bacteria studied. A significant specific incapacity to respond to P. aeruginosa was detected in cystic fibrosis patients with advanced clinical disease.

Antibodies, Bacterial↗
PubMed1977

Human tracheobronchial secretions: development of mucous glycoprotein and lysozyme-secreting systems.

Baseline rates for secretion of mucous glycoprotein were similar similar (680--830 microgram/g tissue/24 hour) for cultured tracheal epithelium from newborns of 26--32 weeks' gestation, full term newborns, and older children. Addition of methacholine to culture medium augmented secretory rates of glycoprotein from all tissue sources 3--5 fold. The overall composition of secreted mucous glycoproteins changed little with increasing age. A trend toward less sulfation and toward increased sialic acid and fucose content was noted in secreted glycoproteins from explants of older subjects. Histochemical observations of stored glycoprotein in tracheal tissue, which was subsequently used for organ culture experiments, confirmed that a modest, but consistent sulfate to sialic acid shift occurs during early life. In contrast, baseline secretory rates for lysozyme from tracheal epithelium of preterm infants were one-half as large as rates from epithelium of full term babies and were refractory to cholinergic stimulation. Stimulation of lysozyme secretion by a cholinergic agonist was achieved in all cases by 40 weeks' gestation. We conclude that basal glycoprotein secretion and the mechanism for glycoprotein response to cholinergic stimulation have developed by the earliest age of viability, but that lysozyme secretion is deficient and is unresponsive to cholinergic stimulation in tracheal tissue from preterm newborns.

Age Factors↗
PubMed1977

Cystic fibrosis diagnosed after age 13. Twenty-five teenage and adult patients including three asymptomatic men.

Cystic fibrosis was diagnosed after age 13 in 25 patients. All had an elevated sweat chloride and either a sibling with cystic fibrosis or typical pulmonary infection or digestive symptoms caused by exocrine pancreatic deficiency. Fourteen had long-standing pulmonary or digestive symptoms. In contrast, four of eight patients whose symptoms began after age 13 presented with biliary cirrhosis. Three male patients were asymptomatic at diagnosis. Opacification of all paranasal sinuses was found in all patients examined radiologically. At diagnosis, pulmonary-function testing showed obstructive changes in 19 patients and sputum cultures showed Pseudomonas aeruginosa in 15 patients. Delayed menarche in five of seven female patients and infertility in the asymptomatic male patient (two of whom were found to have aspermia) could have led to earlier diagnosis. Teenagers and young adults with long-standing pulmonary or digestive symptoms, unexplained cirrhosis, aspermia, or a sibling with cystic fibrosis should be sweat-tested by pilocarpine iontophoresis.

Adolescent↗
PubMed1977

Normal plasma arginine esterase and the Hageman factor (factor XII) -prekallikrein-kininogen system in cystic fibrosis.

Previous investigators have suggested that the biological activity of plasma prekallikrein is defective in cystic fibrosis. In contrast, no such difference was demonstrable between normal and cystic fibrosis plasma. Esterolytic activity for the synthetic substrate p-toluene sulfonyl-arginine methyl ester (TAMe) evolved normally in cystic fibrosis plasma treated with chloroform and ellagic acid, a measure of generation of plasma kallikrein. Additionally, plasma prekallikrein (Fletcher factor) and high molecular weight kininogen (Fitzgerald factor), a substrate of plasma kallikrein, were normal. Thus, the concept that cystic fibrosis is associated with abnormalities in the plasma kallikrein-kinin system could not be supported.

Adolescent↗
PubMed1976

Enzyme replacement therapy for adenosine deaminase deficiency and severe combined immunodeficiency.

To evaluate their role as a form of replacement therapy, frozen irradiated red blood cells were administered to a child with adenosine deaminase deficiency associated with severe combined immunodeficiency disease. In vitro lymphocyte responses to mitogens and allogeneic cells were restored. Subsequently, a "thymus shadow" appeared, and immunoglobulin synthesis was demonstrated. Frozen irradiated plasma, which alone had no effect on lymphocytes numbers or responses, promoted lymphocytosis when given with frozen irradiated red blood cells. The patient received the transfusions with or without irradiated plasma at four-week intervals and remained free of infection for 17 months. The patient's lymphocyte adenosine triphosphate levels were elevated before therapy, which consistently reduced them without altering the lymphocyte adenosine deaminase activity. Enzyme replacement therapy may provide a way to treat patients with adenosine deaminase deficiency associated with severe combined immunodeficiency disease who do not have histocompatible bone-marrow donors.

Adenosine Deaminase↗
PubMed1976

Intellectual and social development following prolonged isolation.

A patient with severe combined immunodeficiency required protective isolation for the first year of life. He was discharged after an effective treatment program was developed and has remained well. Developmental testing at 14 and 16 months revealed near age level social and intellectual functioning. Optimal nursing care, frequent and prolonged maternal contact, and the patient's adaptive capacities permitted rapid adjustment to normal life following prolonged isolation.

Child Development↗