Biomedical subjects
Publications and source records attributed to V S Hubbard.
Large abscess cavities are not commonly encountered in patients with cystic fibrosis (CF). One such patient is reported in whom an abscess developed secondary to aspiration of sand and salt water. Material obtained at bronchoscopy revealed anaerobes as well as the aerobic organisms previously cultured from her sputum. Rapid improvement occurred after institution of antibiotic therapy specifically directed against the anaerobic organisms.
Ultrasound examination of 13 patients with cystic fibrosis revealed ovarian cysts more than 3 cm in size in 46 per cent. All cysts were unilateral, unilocular, and transitory. An autopsy study of 23 patients with cystic fibrosis showed that the measured diameter of the largest ovarian follicle was greater in these patients than in a similar group of age-matched controls (P less than 0.05). The relationship of these ovarian changes to cystic fibrosis is discussed.
Historically, it has been thought that the patient with cystic fibrosis (CF) has a voracious appetite. It is now becoming apparent that many patients do not have caloric intakes which meet their full energy requirements. Our study of CF patient, utilizing, a five-day written record of food consumptions and direct observation, supports this finding and points out the need for further nutritional counseling in providing optimal care for these patients. The cause of increased nutrient requirements and considerations for individualization of counseling are briefly discussed.
The phospholipid fatty acid composition of erythrocytes from patients with cystic fibrosis with pancreatic insufficiency, patients with cystic fibrosis with normal pancreatic enzyme function, and normal subjects was determined. Increased percentages of palmitoleic acid and total eicosatrienoic acid and a decreased percentage of linoleic acid were observed only in the cystic fibrosis patients with pancreatic insufficiency.
Explore the source record for details and available documents.
A patient with the clinical syndrome of cystic fibrosis characterized by chronic pulmonary disease, infection with mucoid Pseudomonas aeruginosa, sinusitis, nasal polyposis, abnormal pancreatic bicarbonate response to secretin stimulation, but normal levels of trypsin and chymotrypsin in the duodenal drainage, and a sibling with autopsy-documented cystic fibrosis, is described. Sweat chloride ranged from 20 to 44 meq/liter and sweat sodium from 36 to 55 meq/liter. Immunoglobulin deficiency, alpha 1-antitrypsin deficiency, tuberculosis and abnormalities of ciliary ultrastructure were excluded. Review of sweat electrolytes in 213 patients with cystic fibrosis revealed that patients with normal pancreatic enzyme release have significantly lower sweat sodium and chloride concentrations (p < 0.0005) than do patients with pancreatic insufficiency. Chronic pulmonary disease, pancreatic insufficiency and elevated levels of sweat electrolytes comprise the classic diagnostic triad for cystic fibrosis. The expression of these features may be variable, but the sweat test remains the cardinal laboratory confirmation of the diagnosis. Over 98 percent of patients with cystic fibrosis have sweat chloride values greater than 60 meq/liter, 1 to 2 percent between 50 and 60 meq/liter, and only about one in 1,000, like our patient, less than 50 meq/liter. Patients with cystic fibrosis with borderline sweat chloride values frequently have chronic pulmonary disease but intact pancreatic enzyme release. In such patients, family history, ancillary clinical features and systemic exclusion of other syndromes assume special diagnostic importance.
An echocardiographic scoring system was developed on the basis of an observed sequence of echo abnormalities, beginning with RV hypertrophy, through RV dilation, to abnormal RV systolic time intervals, noted to be associated with the progressive pulmonary disease of cystic fibrosis. This score correlated significantly with both the Shwachman-Kulczycki (r = 0.87, P < 0.001) and Taussig-NIH (r = 0.86, P < 0.001) clinical scoring systems, the Brasfield chest roentgenogram score (r = 0.86, P < 0.001), and pulmonary function test results. The scoring of echos appears to be useful for the early detection and systematic quantitation of the cardiac effects of the progressive pulmonary disease. Preliminary sequential echo studies suggest that this system provides a method for assessing the progression of cardiac disease and evaluating prognosis in individual patients, and may prove to be useful in monitoring therapeutic interventions for cor pulmonale.
The effectiveness and safety of the use of cimetidine as an adjunct to pancreatic enzymes was evaluated in eight patients with cystic fibrosis having pancreatic insufficiency. Cimetidine (300 mg, three times a day) was given for a 6-week period during which the patients demonstrated a significant weight gain (P < 0.05) and reduction of steatorrhea (P < 0.01). Data suggesting an early correction of the abnormal fatty acid composition of plasma lipids were observed. No side effects were apparent during the clinical trial. These results demonstrate the beneficial effects of the use of cimetidine in patients with cystic fibrosis and indicate the need for long-term clinical trials.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
An episode of severe small intestinal hemorrhage occurred in a cystic fibrosis patient having pancreatic insufficiency and receiving timed-release aspirin therapy for disabling hypertrophic pulmonary osteoarthropathy. The increased acidity of small intestinal contents due to decreased bicarbonate secretion observed in patients with pancreatic insufficiency may alter the luminal environment and result in mucosal erosions and/or ulcerations in association with the presence of aspirin. Thus, physicians should be aware of the possibility that timed-release aspirin causes small intestinal hemorrhage in such patients.
Many epidemiological studies show that alcohol-derived calories added to food intake of men and women in amounts of 0-25% of total energy do not appreciably alter the average daily intake of other macronutrients (carbohydrate, fat, and protein). With such lack of evidence for caloric compensation, alcohol and its calories seem to make little contribution to metabolic energy, body weight, or body composition (as indicated by the body mass index, BMI). In fact, a major study by Colditz et al. (Am. J. Clin. Nutr. 54:49-55; 1991) reported a clear inverse relationship between alcohol intake and BMI for women! Research on alcohol metabolism has left unresolved some apparent contradictions regarding the effect of alcohol on caloric control, appetite and satiation, and body mass and composition. To resolve those apparent contradictions, the National Institute on Alcohol Abuse and Alcoholism cosponsored with the National Institute of Diabetes and Digestive and Kidney Diseases and the Beltsville Human Nutrition Research Center of the U.S. Department of Agriculture an all-day workshop titled "Alcohol and Calories: A Matter of Balance" on January 27, 1993. The workshop included sessions on calorimetry and body mass maintenance, alcohol metabolism, thermoregulation, and an overview of energy balance. This report provides summaries of the four discussion sessions at the workshop.
The percentage fatty-acid composition of the various plasma-lipid fractions from cystic-fibrosis patients with and without pancreatic insufficiency, obligate heterozygotes, and normal subjects was determined. Only the cystic-fibrosis patients with pancreatic insufficiency had significantly abnormal fatty-acid composition. This general observation did not correlate with vitamin-E deficiency, age, sex, or severity of the disease. Thus the altered fatty-acid composition of the different plasma-lipid fractions appears to be a secondary consequence of the pancreatic insufficiency commonly associated with cystic fibrosis and not a direct metabolic defect related to the cystic-fibrosis gene itself.
Explore the source record for details and available documents.
Explore the source record for details and available documents.