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Biomedical subjects

C F Doershuk

Publications and source records attributed to C F Doershuk.

At least 73 records · Page 4Linked to original sources
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

Comparison of measures of forced expiration.

Theoretical relationships among a number of parameters were derived for idealized timed vital capacity (TVC) and maximal expiratory flow-volume (MEFV) curves to determine a minimal set of independent parameters. Normal pediatric subjects and those with cystic fibrosis were studied to verify these relationships experimentally. The average flow over the middle half (FEF25-75%) of the forced vital capacity (FVC) and flows at various exhaled percentages of the FVC (FEF50%, FEF75%), as well as moments of the TVC and MEFV curves were computed. From the TVC moments, a mean transit time (MTT) and an index of dispersion (ID) were also calculated. The minimum information needed to detect pulmonary mechanical changes associated with obstructive lung disease requires at least two reproducible measures: one related to the mean slope (e.g., FEF25-75%/FVC or MTT) and the other to the shape (e.g;, ID) of the effort-independent portion of the MEFV curve.

Adolescent↗
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↗
PubMed1976

Course of cystic fibrosis in 95 patients.

The course of 95 patients with cystic fibrosis is presented. Survivors have a mean follow-up period of over 14 years (minimum: 13 years). Of 45 patients diagnosed prior to extensive irreversible pulmonary involvement, only one has died and none is disabled. In contrast, of the other 50 patients diagnosed after substantial irreversible pulmonary disease was present, 26 have died. Mortality and morbidity has been greater in females. Possible factors contributing to the improving prognosis include early diagnosis, aggressive management with comprehensive care, easy access to specialized care, and improved antimicrobial therapy.

Adolescent↗
PubMed1976

Course of cystic fibrosis in black patients.

Seventeen black patients showed typical sweat gland, gastrointestinal, and pulmonary manifestations of cystic fibrosis. There was an unusually high incidence of meconium ileus equivalent (35%). Three infant siblings of patients in this study had died of cystic fibrosis prior to referral of a family member to this center. One patient died at age four years after emotional problems interfered with the treatment program; status asthmaticus played a major role in his terminal respiratory failure. The remainder of the patients have had substantially less morbidity from pulmonary disease than a comparable, much larger group of white patients. Only one patient, age 28, who is married and employed full-time has severe pulmonary involvement as assessed by pulmonary function studies and chest roentgenogram. The 16 surviving patients have a mean age of 13 1/2 years after a mean follow-up period of 10 years. If black patients with cystic fibrosis survive infancy, they then, as a group, may have a relatively good prognosis.

Black People↗
PubMed1976

Cystic fibrosis.

Explore the source record for details and available documents.

Biological Transport↗
PubMed1976

Symptomatic hepatic disease in cystic fibrosis: incidence, course, and outcome of portal systemic hunting.

Fifteen (2.2%) of 693 patients with cystic fibrosis seen over an 18-year period developed clinical hepatic disease. In 13 patients all symptoms were secondary to portal hypertension. Ten had hypersplenism and 6 had variceal bleeding, including 3 who developed both conditions. All 5 patients who survived the initial episode of gastrointestinal bleeding underwent portal systemic shunting. A shunting procedure also was performed on 1 patients with hypersplenism but no variceal bleeding. No subsequent deterioration of intellectual function occurred in either the shunted or unshunted patients. Only 1 of the shunted patients showed progression of hepatic disease after surgery. These results suggest that portal systemic shunting is useful in the treatment of bleeding esophageal varices in cystic fibrosis. A sweat test to rule out cystic fibrosis should be included in the evaluation of any teenage or young adult patient with unexplained portal hypertension.

Adolescent↗
PubMed1975

Hyperreactivity to cow milk in young children with pulmonary hemosiderosis and cor pulmonale secondary to nasopharyngeal obstruction.

Six black infants and young children with high titers of milk precipitins were identified by screening the sera of 160 children with idiopathic chronic lung disease. None of the six had immunoglobulin deficiency, elevation of sweat chlorides, SS hemoglobin, or recurrent aspiration. All six children had typical manifestations of milk-induced pulmonary hemosiderosis: recurrent pulmonary infiltrates (6/6), hemosiderin-laden pulmonary macrophages (5/6), intermittent wheezing (5/6), eosinophilia (4/6), anemia (4/6), iron deficiency (4/4), failure to thrive (4/6), and elevated levels of serum IgE (4/4). Three children also had chronic rhinitis and eventually developed large adenoids, hypercapnia and acidosis during sleep, and right heart failure. Elimination of cow milk from the diet, symptomatic therapy, and adenoidectomy when indicated resulted in improvement of all six patients. Pulmonary hemosiderosis and some cases of upper airway obstruction with pulmonary hypertension appear to be two stages, early and delayed, of the same immunophysiologic process. Early dietary intervention may prevent the cardiovascular complications of this process.

Black or African American↗
PubMed1975

Open thoracotomy for pneumothorax in cystic fibrosis.

The results of open thoractomy and pleurectomy or pleural abrasion for 17 episodes of pneumothorax in patients with cystic fibrosis were compared with the results of observation, closed thoracostomy, and closed thoracostomy with sclerosing agents. Open thoracotomy had the advantage of insuring prompt resolution of the pneumothorax. A small incision minimized postoperative morbidity. The average hospital stay was 15 days after open thoracotomy and 19 days after closed thoracostomy, with or without sclerosing agents. Complications of all treatments were infrequent. A limited symptomatic recurrence was observed in 2 of 17 pneumothoraces successfully treated with open thoracotomy. Recurrence also occurred in 1 of 8 treated with observation, 3 of 9 treated with closed thoracostomy, and 1 of 14 treated with closed thoracostomy and sclerosing agents. A loss of vital capacity was usually noted 4 to 18 months after resolution, regardless of treatment. Forty per cent of patients in both operative and nonoperative groups survived 3 years. In our experience, open thoracotomy can be used safely for treatment of unresolved or recurrent pneumothorax and warrants further evaluation as a primary therapy for pneumothorax in patients with cystic fibrosis.

Cystic Fibrosis↗
PubMed1975

Studies on cor pulmonale in cystic fibrosis: I. Effects of diuresis.

The effects of acute diuresis in patients with cor pulmonale secondary to cystic fibrosis were studied. Both hemodynamic parameters and arterial blood gas changes were investigated. The major effect noted was a significant reduction of systemic venous pressure. Pulmonary artery and pulmonary artery wedge pressure either remained constant or declined slightly. No consistent changes were noted in cardiac output or arterial blood gases. Acute diuresis of moderate degree appears to be a safe and effective manner in which to treat the systemic venous congestion of cor pulmonale in this situation.

Acid-Base Equilibrium↗