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Biomedical subjects

C F Doershuk

Publications and source records attributed to C F Doershuk.

At least 37 records · Page 2Linked to original sources
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↗
PubMed1985

A morphologic and fluorophotometric analysis of the corneal endothelium in type I diabetes mellitus and cystic fibrosis.

Seven patients with type I diabetes mellitus (Group 1), seven with normoglycemic cystic fibrosis (Group 2), seven with hyperglycemic cystic fibrosis (Group 3), and ten age-matched control subjects underwent corneal fluorophotometry and quantitative specular microscopy. Group 1 had background microangiopathic retinopathy but no evidence of proliferative disease by fluorescein angiography. Significant increases in mean corneal endothelial permeability and mean pump rate occurred in Group 1, indicating a defect in the endothelial barrier function early in type I diabetes mellitus. Similar significant increases in mean corneal endothelial permeability and mean pump rate occurred in both cystic fibrosis groups. The greatest increase was found in Group 3, suggesting a primary defect in the endothelial barrier function in cystic fibrosis, aggravated by the hyperglycemic state. No morphologic abnormalities were noted in Group 1, but both cystic fibrosis groups had smaller mean cell areas than did the control group. There were significant differences in the morphologic and functional correlations between Groups 1 and 3, suggesting different mechanisms for the increased endothelial permeability in these two disorders.

Adult↗
PubMed1984

The alpha 2-adrenergic system of the platelet in cystic fibrosis.

The ability of norepinephrine to inhibit prostaglandin E1 (PGE1)-stimulated accumulation of adenosine 3':5' cyclic monophosphate (cyclic AMP) in intact washed platelets was determined in 12 patients with cystic fibrosis, 6 parents of patients with cystic fibrosis, and a total of 21 healthy age-matched controls. Patients with cystic fibrosis and their parents did not differ from their age-matched controls in basal or PGE1-stimulated levels of cyclic AMP, nor in the dose dependent inhibition of cAMP accumulation by norepinephrine. Moreover, binding sites for [3H]-dihydroergocryptine were present in normal numbers and had normal ligand affinity in platelet membranes from patients with cystic fibrosis. In all measures tested, the alpha 2-adrenergic system in the platelet was normal in cystic fibrosis.

Adolescent↗
PubMed1984

Effects of alpha-tocopherol on platelet membrane function in cystic fibrosis.

Patients with cystic fibrosis and their parents were reported to have abnormal platelet aggregation responses to prostaglandin E1. To determine whether this is a property of the platelets, we studied the adenosine 3':5'-cyclic monophosphate (cAMP) response of washed platelets to prostaglandin E1. The cAMP response to prostaglandin E1 was the same in platelets from obligate heterozygotes for cystic fibrosis and from those of healthy controls. Patients with cystic fibrosis who had deficient vitamin E levels (plasma alpha-tocopherol, less than 500 micrograms/dl) had significantly (p less than 0.01) reduced platelet cAMP response to prostaglandin E1 compared with patients who had sufficient vitamin E, and supplementation with water-miscible vitamin E in these patients resulted in significant increases in plasma alpha-tocopherol levels (p less than 0.01) and in cAMP response to prostaglandin E1 (p less than 0.05). Plasma alpha-tocopherol levels correlated significantly with platelet cAMP response to prostaglandin E1 in patients with cystic fibrosis (r = 0.58, p less than 0.05). However, plasma alpha-tocopherol level was unrelated to the lymphocyte and granulocyte cAMP response to prostaglandin E1 or to the platelet cAMP response to alpha 2-adrenergic stimulation. Our data suggest that patients with cystic fibrosis have no inherited defect in platelet cAMP response to prostaglandin E1. In patients who have sufficient vitamin E, cAMP responses to prostaglandin E1 are normal in all the formed elements of the blood.

Adolescent↗
PubMed1983

Early echocardiographic and pulmonary function findings in idiopathic scoliosis.

Thirty-six children and adolescents with early stages of idiopathic scoliosis underwent evaluation by echocardiography and pulmonary function testing. Mildly increased pulmonary vascular resistance was inferred from an elevated ratio of right preejection period to right ventricular ejection time, an increased right ventricular dimension, and a decreased left ventricular dimension. Since neither decreased arterial oxygen saturation nor increased end-tidal expired carbon dioxide partial pressure was seen, desaturation and hypoventilation should not account for these abnormalities. Pulmonary function parameters showed no distinct patterns of abnormality. Even though the patients were divided into two groups by severity of spinal curvature, the cardiopulmonary measures did not correlate with thoracic deformity. Billowing of the mitral leaflets, termed mitral valve prolapse, was demonstrated in 25% of the subjects. Our findings suggest that cardiopulmonary and thoracic changes in idiopathic scoliosis may develop in parallel and may be expressions of a common collagen defect. However, study of sleep and exercise arterial saturation may be required to rule out intermittent hypoxemia as a precipitating factor of cor pulmonale in scoliosis.

Adolescent↗