Asunto(s)

RESUMEN

A series of 22 patients with cystic fibrosis (CF) of similar clinical severity (9 with normal carbohydrate tolerance and 13 with insulin-treated fasting hyperglycemia) was examined with quantitative vitreous fluorophotometry. All of the CF patients studied had normal fundi on ophthalmoscopy, fundus photographs, and fluorescein angiography. Mean vitreous fluorescein concentration in the CF patients whose hyperglycemia was treated with insulin (11.79 ng/ml) was significantly higher than in CF patients with normal carbohydrate tolerance (6.98 ng/ml, P less than 0.005). Thus, CF patients with fasting hyperglycemia demonstrate a breakdown of the blood-retinal barrier. When CF patients with fasting hyperglycemia were compared with age- and sex-matched type I diabetics, there was no significant difference in mean vitreous fluorescein accumulation. Thus, breakdown of the blood-retinal barrier, one of the earliest detectable functional abnormalities that may be associated with the microangiopathy of diabetes mellitus, also occurs with equal frequency and severity in the diabetes secondary to pancreatic fibrosis associated with CF.

Asunto(s)

RESUMEN

Nasal polyposis complicated the course of fibrosis in 157 (26%) of 605 patients. Onset before age 5 years or after age 20 years was rare. Polyposis was the initial symptom of cystic fibrosis in 13 patients. Common symptoms included obstruction to nasal air flow, mouth breathing, epistaxis, and rhinorrhea. Intranasal and oral corticosteroids and antihistamines were ineffective in preventing recurrences but did occasionally afford symptomatic relief of obstruction. Nineteen (31%) of 62 patients who never had surgery had spontaneous and permanent disappearance of polyps. Simple polypectomy was an adequate procedure for patients with substantial nasal symptoms. There were no visual complications. Other surgical complications were rare. Children and adolescents with nasal polyps should have sweat tests by pilocarpine iontophoresis to rule out cystic fibrosis.

Asunto(s)

RESUMEN

Rectal prolapse occurred in 112 (18.5%) of 605 cystic fibrosis patients. In 48 patients prolapse preceded diagnosis of cystic fibrosis, but physicians (pediatricians, pediatric and general surgeons, and proctologists) rarely appreciated its importance as a symptom of this disease. Prolapses frequently cease with institution of pancreatic enzyme replacement therapy following diagnosis of cystic fibrosis. However, even when the disease remains undiagnosed, the symptom is often transient and frequently resolves at 3-5 yr of age. Prolapse occurring initially after cystic fibrosis is diagnosed rarely responds to manipulation of diet or enzyme doses. Many patients develop a method of reduction which involves voluntary abdominal, perineal, and gluteal muscles and does not require manual pressure on the prolapsed segment. Most patients do not need specific treatment for the prolapse. Surgery is rarely necessary. A sweat test should be obtained on any child who has had even a single episode of rectal prolapse.

Asunto(s)

RESUMEN

Failure of the right side of the heart with cardiac dilation and fluid retention occurred in 55 of 170 patients who died of cystic fibrosis; six patients survive. All had severe hypoxia, but 24% had normal PaCO2. Cardiac catheterization showed high mean pulmonary artery pressure and resistance. Pulmonary artery wedge pressure was greater than 12 mm Hg in 40% of the patients. Mean survival was eight months. Male survival was significantly better than female survival. Digitalis treatment was of no clear benefit. Tolazoline hydrochloride was also ineffective. Recent medical advances have not substantially affected prognosis.

Asunto(s)

RESUMEN

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.

Asunto(s)

RESUMEN

Lobar atelectasis occurred in 30 (4.1 per cent) and segmental atelectasis occurred in 6 (0.8 per cent) of 728 patients with cystic fibrosis. The right lung was involved in all 11 patients less than 5 years of age and in 18 of 26 episodes in 19 older patients. Five of the younger patients died within 5 years of the initial episode. Bronchoscopy (with or without local lavage) performed in addition to intensified medical therapy did not appear to improve the outcome. Future studies of therapeutic measures (including bronchoscopy) for treatment of atelectasis complicating cystic fibrosis must include appropriate control patients who receive only medical therapy.

Asunto(s)

RESUMEN

We studied the clinical and laboratory characteristics of seven patients with sweat chloride concentration consistently between 40 and 60 mEq/liter. Each has chronic Pseudomonas bronchitis, and all lack digestive symptoms. Laboratory findings indicate the preservation of exocrine pancreatic function. The patients include two of five children in one family and two of four in another. In a third family, one of five siblings has an intermediate sweat chloride concentration, but another has a typical fibrosis value (105 mEq/liter). One patient died of respiratory failure; results of an autopsy showed bronchiolectasis typical of cystic fibrosis, but minimal pancreatic changes. The data suggest a genetic basis for this variant of cystic fibrosis. These patients may be homozygous for a portion of a closely linked multigene cystic fibrosis locus or may have modifier genes that ameliorate the pancreatic and sweat lesions.

Asunto(s)

Asunto(s)

RESUMEN

Statistical discriminant analysis is applied to 41 concomitant variables obtained during the first year of study of 226 patients with cystic fibrosis. A discriminant function based on six variables is developed which can be used as a predictive index. This index estimates the probability of 5-year survival for an individual patient and can also be used to classify patients into one of two groups: (1) will live for 5 years or (2) will die within 5 years. Speculation A classification rule for cystic fibrosis is presented which correctly assigns 90% of the 5-year survivors and 84% of those who expire. Such a classification scheme is useful for clinical and research purposes.

Asunto(s)

RESUMEN

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.

Asunto(s)

RESUMEN

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.

Asunto(s)

RESUMEN

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.

Asunto(s)

RESUMEN

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.

Asunto(s)

Asunto(s)

RESUMEN

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.

Asunto(s)

RESUMEN

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.

Asunto(s)

RESUMEN

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.

Asunto(s)

Asunto(s)

Asunto(s)