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Biomedical subjects

D J Strieder

Publications and source records attributed to D J Strieder.

12 recordsLinked to original sources
PubMed1990

Does lithium carbonate affect the ion transport abnormality in cystic fibrosis?

Lithium is known to affect several aspects of cellular regulation which may be related to ion channel function in epithelial cells. To determine whether the ion transport abnormality in cystic fibrosis (CF) is affected by lithium with resultant changes in clinical status, 36 CF patients, 12-37 years old, were enrolled in a 14 week, double-blind, placebo-controlled trial. Eighteen patients were randomly assigned to receive lithium carbonate for 10 weeks. At the end of therapy their average serum lithium concentration was 0.56 +/- 0.06 mmol (SEM) per liter. Their sweat chloride concentration fell from 92.1 +/- 4.8 mmol per liter to 87.4 +/- 4.0 mmol per liter after 10 weeks of therapy (P = 0.07) and rose to 94.4 +/- 3.5 mmol per liter 4 weeks after end of therapy (P less than 0.001 compared to results at end of therapy). Their forced vital capacity (FVC) fell from 72 +/- 5.3% of predicted to 66 +/- 5.1% of predicted after 4 weeks of therapy (P less than 0.01), and their forced expiratory volume in one second (FEV1) fell from 56 +/- 5.5% of predicted to 51 +/- 5.5% of predicted after 4 weeks of therapy (P less than 0.01). In a non-blind assessment, performed 19 weeks after the end of therapy, their FVC and FEV1 had risen and were not significantly different from baseline. Sweat chloride, FVC, and FEV1 remained unchanged in the placebo group throughout the period of study.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1979

Lung function in children with sickle cell anemia.

Lung volumes and expiratory flows were measured in 12 children with sickle cell anemia and 12 height-matched black control subjects. Diffusing capacity of the lung for CO, pulmonary capillary blood volume, the membrane component of diffusing capacity, arterial blood gases on breathing room air and 100 per cent O2 were measured in the subjects with sickle cell anemia. The lung volumes and expiratory flows of subjects with sickle cell anemia were no different from those of the control subjects. Diffusing capacity for CO was maintined in the noraml range despite the severe anemia by increases in pulmonary capillary blood volume and the membrane component of diffusing capacity. All subjects with sickle cell anemia had mild hypoxemia and abnormal increases in calculated shunt. Pulmonary function in children with sickle cell anemia appears to be determined by their race and anemia.

Adolescent↗
PubMed1979

Lung function in adolescents receiving high-dose methotrexate.

Methotrexate (MTX) has been implicated as a cause of interstitial pneumonitis and/or fibrosis, but the mechanism by which the drug causes these processes is not known. The purpose of this study was to determine whether patients receiving high-dose MTX developed a consistent decrease in pulmonary function, which would implicate a role for total dose of MTX received in the pathogenesis of the lung toxicity. Pulmonary function studies, including spirometry, plethysmography, and diffusing capacity at two levels of alveolar PO2, were performed in 38 adolescents treated for osteogenic sarcoma. The patients were divided into three groups including 12 patients (group 1) studied before and during therapy, 15 patients (group 2) studied during therapy, and 11 patients (group 3) studied after completion of treatment. While total dose received at the time of the study varied from 0 to 256 gm/sq m, pulmonary function showed no change, with one exception. A mild restrictive defect and decrease in diffusing capacity due to unilateral pleural and diaphragmatic disease, whose relationship to MTX therapy is uncertain, developed in one patient. MTX, when administered in high dose to young patients by the described protocol, causes no dose-related decrease in pulmonary function.

Adolescent↗
PubMed1977

Hypersensitivity to pancreatic extracts in parents of patients with cystic fibrosis.

Because immediate hypersensitivity reactions can occur in individuals exposed to powdered pancreatic extracts, 36 patients with cystic fibrosis and 51 patents of such patients wwer studied for evidence of sensitization. Sensitivity to the extracts as evidence by history and skin testing was infrequent in the children with cystic fibrosis. However, skin testing for immediate hypersensitivity with either crude pancreatic extracts or inactivated trypsin correlated well in their patents with a history of clinical symptoms. IgE mediation of these reactions in sensitized individuals was demonstrated by antigen-induced histamine release from leukocytes, passive transfer studies, and immediate response to inhalation challenge.

Adult↗
PubMed1977

The lung following repair of congenital diaphragmatic hernia.

To determine the effects of the pulmonary hypoplasia present at birth in infants with congenital diaphragmatic hernia upon subsequent development of the lung, 19 patients who had undergone surgical repair before the age of one year were studied at ages 6 to 18 years. Total lung capacity and vital capacity averaged 99% of predicted value. Diffusing capacity for carbon monoxide was normal. Forced expiratory volume in one second averaged 89% of predicted value and 80% of vital capacity. Total respiratory system conductance and maximum expiratory flow volume curves obtained during air and helium-oxygen breathing were normal. Xenon 133 radiospirometry performed in nine patients revealed equal distribution of lung volumes on the two sides. Ventilation to the hernia side was reduced in only two patients. Blood flow to the hernia side was reduced in all nine patients. Chest radiographs supported the physiologic observations. These findings are consistent with the persistence of a reduction in the number of branches or generations of pulmonary arteries and bronchi on the side of the hernia. Since a substantial part of the vascular resistance resides in peripheral vessels, this developmental abnormality influences the distribution of pulmonary blood flow, although it has little effect on tests reflecting airway resistance or the distribution of ventilation.

Adolescent↗
PubMed1977

Sleep apnea in a child with the pickwickian syndrome.

A 3-year-old girl with a history of excessive weight gain from birth presented with obesity, somnolence, and cyanosis, characteristic of the Pickwickian syndrome. Obesity was familial and exogenous without endocrine or neurologic anomaly. Respiratory center sensitivity to carbon dioxide was normal. Excessive somnolence was due to the obesity, which during sleep caused airway obstruction, apnea, and awakening, finally resulting in sleep deprivation. The sleep apneas and the daytime somnolence disappeared with weight reduction, showing that obesity alone had been responsible for the disorder.

Apnea↗
PubMed1976

Periodic apnea, exercise hypoventilation, and hypothalamic dysfunction.

Periodic apnea and exercise hypoventilation were observed in a 14-year-old boy. Hyperphagia, obesity, serum hyperosmolality without diabetes insipidus or appropriate thirst, and retardation of growth and sexual development indicated a hypothalamic disorder. Neurologic evaluation was normal except for electroencephalographic changes induced by apnea. Pulmonary function tests, resting arterial blood gases in the wakeful state, and ventilatory response to inhaled CO2 were also normal. Acute hypoxemia and respiratory acidosis occurred with apnea during sleep and with insufficient ventilation during exercise. The central origin of sleep apneas was shown by esophageal pressure monitoring. The hypothalamic dysfunction and exercise hypoventilation distinguish this patient from others with obesity and periodic apnea.

Adolescent↗
PubMed1975

Immediate hypersensitivity to hog trypsin resulting from industrial exposure.

Clinical, immunologic and psysiologic studies were undertaken to establish the role of hog trypsin dust in four cases of occupational asthma and to define the mechanism of this respiratory disease. Objective evidence was obtained that these patients, but not their asymptomatic co-workers, were allergic to trypsin, and that this fact accounted for their respiratory symptoms. Direct skin testing, passive transfer of IgE antibodies, antigen-mediated histamine release from peripheral blood leukocytes and airway obstruction in response to inhalation challenge indicated IgE-mediated, Type I hypersensitivity. These effects were induced by inactivated trypsin and were therefore independent of tryptic enzymatic activity. The data suggested that periodic skin testing for immediate sensitivity to organic dust would be an inexpensive and convenient screening procedure for early detection and prevention of some types of occupational asthma.

Aerosols↗
PubMed1975

Exercise tolerance after repair of tetralogy of Fallot.

Twelve patients who had undergone complete repair of tetralogy of Fallot at ages ranging from 8 to 27 years were studied for exercise tolerance several years later. Work performance ranged from 58 to 130% of that predicted. Increased physiological dead space and alveolar-arterial Po2 difference at exercise suggested pulmonary vascular disease, which was confirmed in 1 patient by pulmonary angiography. Excercise tolerance decreased with increasing age at time of repair, underlining the desirability of early corrective operation.

Acid-Base Equilibrium↗