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Biomedical subjects

C F Doershuk

Publications and source records attributed to C F Doershuk.

At least 19 recordsLinked to original sources
PubMed1992

The prognostic value of exercise testing in patients with cystic fibrosis.

BACKGROUND: Previous studies have shown female sex, impaired pulmonary function, older age, malnutrition, and colonization of the respiratory tract with Pseudomonas cepacia to be associated with a poor prognosis in patients with cystic fibrosis. We sought to determine the prognostic value of exercise testing in addition to the other prognostic factors. METHODS: A total of 109 patients with cystic fibrosis, 7 to 35 years old, underwent pulmonary-function and exercise testing in the late 1970s. They were followed for eight years to determine the factors associated with subsequent mortality. Survival rates were calculated with standard life-table methods. Cox proportional-hazards regression models were used to determine crude relative risks of mortality and relative risks adjusted for age, sex, body-mass index, forced expiratory volume in one second (FEV1) end-tidal partial pressure of carbon dioxide (PCO2) at peak exercise, and oxygen consumption at peak exercise (VO2 peak). RESULTS: Patients with the highest levels of aerobic fitness (VO2 peak, > or = 82 percent of predicted) had a survival rate of 83 percent at eight years, as compared with rates of 51 percent and 28 percent for patients with middle (VO2 peak, 59 to 81 percent of predicted) and lowest (VO2 peak, < or = 58 percent of predicted) levels of fitness, respectively. After adjustment for other risk factors, patients with higher levels of aerobic fitness were more than three times as likely to survive than patients with lower levels of fitness. Colonization with P. cepacia was associated with a risk of dying that was increased fivefold. Age, sex, body-mass index, FEV1, and end-tidal PCO2 at peak exercise were not independently correlated with mortality. CONCLUSIONS: Higher levels of aerobic fitness in patients with cystic fibrosis are associated with a significantly lower risk of dying. Although better aerobic fitness may simply be a marker for less severe illness, measurement of VO2 peak appears to be valuable for predicting prognosis. Further research is warranted to determine whether improving aerobic fitness through exercise programs will result in a better prognosis.

Adolescent↗
PubMed1992

Use of nonmedical treatment by cystic fibrosis patients.

To determine frequency, perceived effectiveness, and cost of religious, physical, and other nonmedical practices, we interviewed all (n = 402; 100% participation) of our cystic fibrosis patients/families [age, 0-45 (median, 18) years]. At least one type of nonmedical therapy was used by/on 264 (66%), two-thirds of which was religious. Most respondents perceived benefit (e.g., group prayer, 95%; religious articles, 81%; chiropractic, 69%). Only 17 (4.2%) invested over $200 lifetime (minimal compared with medical costs). These treatments, apparently used by most patients/families, rarely interfered with medical care. Physician openness and empathy toward patients' beliefs might enhance rapport and facilitate discussion of possible helpful or adverse effects of nonmedical treatment.

Adolescent↗
PubMed1992

The nasal response to exercise in patients with cystic fibrosis.

This study has evaluated the nasal response to exercise in patients with cystic fibrosis (CF), a genetic disease in which factors such as chronic lung disease and/or nasal polyposis might be anticipated to modify nasal function responses. Measurements of nasal resistance (NAR) by posterior rhinomanometry and specific airway resistance (sRAW) were made before and 1, 5, 10, and 30 min after a 4-min period of exhausting legwork exercise (50% predicted maximal) in 19 CF patients (aged 11-29 years) and 10 healthy subjects (aged 11-31 years). One minute after exercise, healthy subjects showed a 54 +/- 5% (mean +/- SEM; standard error of the mean) relative fall from baseline in NAR and CF patients showed a 31 +/- 8% relative fall from baseline (p < 0.05). There were no significant differences in the magnitude or pattern of recovery in NAR after exercise (1 to 30 min) between the groups, largely because of the variability in NAR responses in CF patients. Exercise did not result in significant changes in sRAW in either group. We also found that a history or presence of nasal polyposis does not significantly affect functional nasal responses to exercise. Our conclusion is that the CF genotype and its airway sequelae do not substantively affect the control of the nasal response to exercise.

Adolescent↗
PubMed1991

Medical and social factors as predictors of outcome in infant tracheostomy.

We examined the relative impact of infant tracheostomy in comparison to associated medical and social factors, on developmental outcome as part of a cross-sectional follow-up of 32 children. These children had no mental retardation, physical handicap, or severe neurological problems, but had a history of long-term tracheostomy in infancy, ranging from 3 to 146 months duration. Medical factors evaluated included prematurity, neurological status, severity of illness, and number of weeks hospitalized. Social factors included parental education and occupation. Outcome measures included IQ, language quotient, growth parameters, and behavioral competence. Correlation analyses, stepwise multiple regression analyses, and t-tests were used. Early medical illnesses were significant predictors of cognitive, language, and growth outcome. Severity of medical complications at birth and the presence of any neurological problem predicted 49% of the variance in IQ at follow-up. Social class was the only variable to predict behavioral outcome, accounting for 28% of the variance. For children without confounding medical conditions, tracheostomy had a negative impact on overall language and auditory comprehension. Once children with confounding medical risk factors were removed from the sample, children with history of infant tracheostomy exhibited significantly lower overall mean language scores (106 versus 120), and lower mean language comprehension scores (104 versus 119) than a matched comparison group.

Child Development↗
PubMed1990

Monocytes in cystic fibrosis: responsiveness to microbial stimuli.

Peripheral blood monocytes were obtained from 19 patients with cystic fibrosis (CF) and age-matched paired normal individuals. The oxidative metabolic response of these cells was measured by superoxide anion production before and after stimulation with Salmonella typhimurium or Pseudomonas aeruginosa lipopolysaccharide (LPS). CF monocytes showed slightly greater spontaneous superoxide anion production (14.1 +/- 2.1 SEM nanomoles superoxide anion/10(6) monocytes/180 min; n = 12) than normal monocytes (9.5 +/- 1.4; n = 12), P = 0.009. No differences between CF and normals were found in LPS-stimulated superoxide anion production (CF = 33.5 +/- 4.6, n = 13; normal = 33.8 +/- 4.2, n = 13). Furthermore, CF monocytes responded to both P. aeruginosa and S. typhimurium LPS stimulation as well as to recombinant interferon-gamma. Superoxide anion production of CF monocytes was comparable in autologous serum and in normal serum, and responses of patients colonized with P. aeruginosa and P. cepacia did not differ. We conclude that CF monocytes have a slightly increased metabolic level and, despite chronic infection, are capable of a further response to exogenous microbial stimuli.

Adolescent↗
PubMed1990

The spectrum of appendiceal disease in cystic fibrosis.

A retrospective review of 1,220 cystic fibrosis (CF) patients between 1965 and 1989 identified 60 patients who underwent appendectomy. Ten had appendectomy prior to referral and 16 had an incidental appendectomy (primarily meconium ileus). Among the remaining 34 patients, acute appendicitis was present in 19 (1.8% incidence). "Classic appendicitis"--acute abdominal pain shifting to the right lower quadrant (RLQ), focal RLQ tenderness, and elevated white blood cell (WBC) count--was present in 15. Four additional inflamed appendixes were removed in patients following incorrect preoperative diagnosis. Thirteen of these 19 were perforated. Complications included wound infection (2), pelvic abscess (1), ileal obstruction requiring ileostomy (1), and pelvic hematoma (1). There were no deaths. One patient with acute RLQ pain and tenderness had a normal appendix. Seven patients (mean, 20 years) had chronic, intermittent, focal RLQ pain and tenderness not originating periumbilically with a normal WBC count and temperature. At exploration, these appendixes were enlarged and tensely distended with inspissated mucus. Microscopic examination showed no inflammation. Appendectomy resulted in resolution of symptoms without complications. Four additional patients with intermittent RLQ pain and tenderness and a history of recurrent intussusception presented with ileocolic intussusception. Persistent postreduction symptoms in three and failure of reduction in the fourth necessitated celiotomy at which time tensely distended appendixes were removed. Appendectomy led to resolution of symptoms. Three additional enlarged noninflammed appendixes were incidentally removed in asymptomatic patients undergoing unrelated intraabdominal procedures. Appendiceal disease in CF patients represents a spectrum ranging from simple mucous distention to acute appendicitis with perforation. CF patients with pain secondary to a noninflamed distended appendix represent a distinct syndrome cured by appendectomy.(ABSTRACT TRUNCATED AT 250 WORDS)

Abdominal Pain↗
PubMed1990

Bronchial artery embolization to control hemoptysis in cystic fibrosis.

Severe hemoptysis in cystic fibrosis can be life-threatening because of acute blood loss or because it interferes with sustaining physical therapy of the chest. Hemoptysis was controlled in 19 of 20 cystic fibrosis patients by means of embolization with a combination of 250-590-microns particles of polyvinyl alcohol foam and absorbable gelatin pledgets. Repeat embolization was needed in eight patients to achieve or maintain effective hemostasis. One or more aberrant bronchial arteries were found in seven patients (35%), and a spinal artery branching from a vessel that also supplied bronchial circulation was present in 11 patients (55%). The data suggest that although embolization has a wider applicability than previously reported, care should be taken during angiography to evaluate the bronchial circulation because aberrant bronchial vessels and spinal arteries arising from bronchial circulation are common.

Adolescent↗
PubMed1990

Weight and menstrual function in patients with eating disorders and cystic fibrosis.

Many patients with eating disorders have menstrual dysfunction. In patients with anorexia nervosa, amenorrhea has been linked to weight loss. However, many patients with bulimia nervosa, even those of average or greater than average weight, also experience menstrual abnormalities, including amenorrhea. It was hypothesized that low weight per se is not responsible for menstrual dysfunction in patients with eating disorders. First, 16 patients with bulimia nervosa of average weight and 29 patients with cystic fibrosis of below average weight of similar menstrual age were compared. Of the patients with bulimia nervosa, 11 (73%) had had secondary amenorrhea at some time compared with 8 (28%) of the patients with cystic fibrosis (P less than .01). At the time of study, 6 (40%) of the patients with bulimia nervosa and 21 (78%) of the patients with cystic fibrosis were having regular cycles (P less than .01). Next 18 patients with anorexia nervosa were compared with 18 patients with cystic fibrosis matched for weight and menstrual age. Although 18 (100%) of the patients with anorexia nervosa had had secondary amenorrhea, only 6 (33%) of the patients with cystic fibrosis had amenorrhea. Although only 1 (6%) of the anorexia nervosa group was currently having regular cycles, 14 (78%) of the patients with cystic fibrosis were (P less than .0001). Closer approximation to ideal body weight was associated with regular menses only in the cystic fibrosis group. Exercise did not appear related to regularity of menstruation in any group. Body weight is not the major factor responsible for menstrual abnormalities in patients with eating disorders.

Adolescent↗
PubMed1988

Longterm histopathologic follow-up of bronchial arteries after therapeutic embolization with polyvinyl alcohol (Ivalon) in patients with cystic fibrosis.

We used light microscopy to examine, at autopsy, bronchial arteries in three patients with cystic fibrosis who died, respectively, 10, 16, and 28 months after bronchial artery embolization with barium sulfate-impregnated polyvinyl alcohol (PVA) to control hemoptysis. PVA was not identified beyond the midsegmental bronchus in any patient. Persistent focal fibrovascular occlusion was noted in two patients, and recanalized and/or partially obstructed vessels were associated with PVA in all. The histologic reaction to PVA included fibrosis, mild chronic inflammation, localized foreign body reaction, and, in two patients, focal calcification of PVA spicules. Within the inflammatory milieu were numerous macrophages containing BaSO4. Extensive vascular mural destruction and fibrosis associated with PVA were also observed. Both PVA and BaSO4 were also frequently present in the perivascular connective tissue. These findings indicate that, although longterm occlusion persists after therapeutic arterial embolization with PVA, focal recanalization also occurs. The extent of vascular mural injury following PVA embolization in humans has been previously underestimated by animal experiments. Finally, perivascular deposition of PVA represents a common reaction to diverse foreign body emboli in both systemic and pulmonary arteries.

Adolescent↗
PubMed1987

Recreational use of psychoactive drugs by patients with cystic fibrosis.

We assessed unprescribed psychoactive drug use in 173 adults with cystic fibrosis. Twenty (11%) regularly smoked tobacco. Cigarette smoking ranged from 1 to 30 years (2 to 60 pack-years). Alcohol was used by 60%, and marijuana by 20% of the patients. Pulmonary symptoms were often increased the day after alcohol ingestion. Alcohol occasionally caused nausea, vomiting, and headache if the patient was taking some cephalosporin derivatives (such as cefsulodine) or chloramphenicol. Marijuana often aggravated chronic pulmonary symptoms, although some patients reported transient relief during use. Comparison with a retrospectively selected control group did not show faster short-term pulmonary deterioration in the tobacco smokers. Physicians who deal with cystic fibrosis and other chronic illnesses should be cognizant of interactions of unprescribed and prescribed drugs. Recreational use of unprescribed psychoactive drugs should be considered if unexpected symptoms occur in older patients.

Adolescent↗
PubMed1987

Colonization of the respiratory tract with Pseudomonas cepacia in cystic fibrosis. Risk factors and outcomes.

Between 1981 and 1983, some 85 patients with cystic fibrosis at Rainbow Babies and Childrens Hospital, Cleveland, developed colonization or infection of the respiratory tract with Pseudomonas cepacia. Twenty-nine (34 percent) of the colonized patients died; four were female patients with fulminant bacteremia with P cepacia prior to death. Case-control studies showed that increasing severity of underlying cystic fibrosis, increasing age, having a sibling with cystic fibrosis who was colonized with P cepacia, and previous hospitalizations were associated with increased risk of colonization. In patients with mild cystic fibrosis, no differences in clinical outcome were seen during the period of study; however, patients colonized with P cepacia who had moderate or advanced cystic fibrosis were hospitalized longer and died sooner after colonization, compared with control subjects with similar severity of cystic fibrosis. The excess mortality associated with such colonization varied in magnitude and trend according to the patient's sex and severity of underlying cystic fibrosis, reflecting the combined influence of colonization with P cepacia, sex, and severity of cystic fibrosis on the mortality of the patients. The source and mode of transmission of P cepacia were not determined, but the data suggest a possible nosocomial source. The results of this investigation showed that colonization with P cepacia most often affected patients with moderate or advanced cystic fibrosis and was associated with an adverse clinical outcome in these patients.

Adolescent↗
PubMed1987

Correlates of psychosocial adjustment among young adults with cystic fibrosis.

Young adults with cystic fibrosis (CF) (n = 73) were found to have age-adequate psychological functioning. Psychological adjustment, as measured by the Psychological Screening Inventory (PSI) and the Tennessee Self-Concept Inventory (TSCI), correlated with coping style as measured by the Repression-Sensitization (R-S) scale. A repressive or avoidant coping style was associated with higher self-esteem, lower alienation and discomfort, and greater social activity, but not with employment status or school attendance. Severely ill patients were less likely to be employed or in school, and showed higher levels of psychological discomfort. Physicians' ratings of coping effectiveness were positively related to a repressive illness-related coping style. Prospective assessment of psychological functioning would yield useful information concerning the coping strategies used by young adults with CF to ameliorate the psychological distress associated with physical deterioration.

Adolescent↗
PubMed1986

Treatment and prognosis of symptomatic gallbladder disease in patients with cystic fibrosis.

Twenty-four (3.6%) of 670 patients with cystic fibrosis seen over a 25-year period developed symptomatic gallbladder disease. Only four patients were less than 16 years old. Four patients presented with unusual problems, including one with acute cholangitis and two with atonic gallbladder, one of whom required cholecystectomy. Another patient was found to have cholangiocarcinoma of the gallbladder when an exploratory laparotomy was performed to investigate biliary obstruction. Twenty patients had cholelithiasis, 15 of whom underwent cholecystectomy. Only one patient had substantial pulmonary difficulties postoperatively. Patients who presented with classic biliary colic had no further symptoms after cholecystectomy. One patient developed intrahepatic stones 6 years later and required a choledochoduodenostomy. As the pulmonary status of most cystic fibrosis patients will eventually deteriorate, we recommend that serious consideration be given to performing a cholecystectomy as soon as practical after the diagnosis of symptomatic cholelithiasis. Our experience indicates that surgery can be performed safely unless pulmonary status is already extremely compromised and the patient is in overt respiratory failure.

Adenoma, Bile Duct↗
PubMed1986

The interaction of 2 diseases: diabetes mellitus and cystic fibrosis.

We have reviewed the hospital records of 24 patients with cystic fibrosis-associated diabetes, and 2 groups of CF patients (1 with normal and the other with abnormal oral glucose tolerance tests) who did not develop symptomatic fasting hyperglycemia, to define the clinical characteristics of the diabetes and to study its effects on the progression of the pulmonary disease, changes in sputum organisms, and mortality. Although maximum blood glucoses ranged from 322 to 1160 mg/dl with a median of 579 mg/dl, only 1 of 24 diabetic patients developed ketoacidosis. This patient developed diabetes 12 years prior to the diagnosis of CF and may have had type 1 diabetes. In contrast, hypoglycemia was frequent and 4 patients were hospitalized with serious neurologic manifestations. Two patients were found to have diabetic retinopathy, 1 with macular edema required laser photocoagulation to improve vision, and the other had multiple microaneurysms. CF-associated diabetes did not influence the deterioration of clinical scores, chest x-ray scores, pulmonary function tests, the number of hospital admissions, the type of organisms found in the sputum, or mortality rates. The development of diabetes in our CF patients was not related to the severity of pulmonary dysfunction, clinical, or chest x-ray scores. Thus, although the development of diabetes is an additional encumbrance upon the already therapeutically burdened existence of a CF patient, it does not appear to affect the course of the disease. Despite the demonstration of diabetic retinopathy in this study, most patients with CF-associated diabetes still do not live long enough to develop microvascular complications from the diabetes.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1986

Pseudomonas cepacia: decrease in colonization in patients with cystic fibrosis.

The incidence and prevalence of Pseudomonas cepacia pulmonary colonization were noted to be increasing in patients with cystic fibrosis (CF). Previous work had indicated a greater prevalence of P. cepacia among siblings (with CF) of patients colonized by P. cepacia as well as an association of initial positive P. cepacia cultures with a hospitalization. Because of uncertainty regarding the source and mode of transmission, limited precautionary measures were instituted in 1983, including physical separation of hospitalized patients colonized with P. cepacia from non-colonized patients, reeducation of staff concerning basic infection control procedures, explanation to families regarding these precautionary efforts, and institution of separate summer camp sessions. Repeated environmental cultures throughout the hospital were negative for P. cepacia. Coincident with the institution of control measures, a sharp decline in incidence occurred (8.2% in 1983 versus 1.7% in 1984). These results are suggestive of patient-to-patient transmission. Because P. cepacia infections have been associated with shorter survival in some patients with CF, we will continue our current segregation measures.

Anti-Bacterial Agents↗