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Emergency lobectomy for massive hemoptysis in cystic fibrosis
J
THoRAc CARDIOVASC SURG
86:409-411, 1983
Emergency lobectomy for massive hemoptysis in cystic fibrosis Massive hemoptysis occurs in 50% to 70% of patients with cystic fibrosis and carries an immediate mortality of up to 32%. Cystic fibrosis is regarded by many as a disease in which thoracic operations are ill-advised. A 21-year-old woman with mild cystic fibrosis presented with unrelenting massive hemoptysis. was not helped by medical management. and underwent a successful left upper lobectomy. We suggest that thoracotomy does have a role in the management of massive hemoptysis in selected patients with cystic fibrosis. On the basis of our case and all other cases reported in the English literature. we suggest criteriafor determining which patients are suitable candidates for emergency thoracotomy.
Donald K. Porter, Captain, USAF, MC,* Marvin J. Van Every, Captain, USAF, MC,** and John W. Mack, Jr., Lieutenant Colonel, USAF, MC,** Lackland Air Force Base. Texas
h e survival rate of patients with cystic fibrosis has steadily improved such that 69% to 84% of patients are expected to survive to the age of 20 years. I With prolonged survival, pulmonary complications such as hemoptysis are being seen with increased frequency. Minor hemoptysis has been noted in 60% of patients with cystic fibrosis and massive hemoptysis, defined as a 300 ml blood loss within 24 hours, in 5% to 7%.2 Although massive hemoptysis is classically managed by surgical intervention;" certain authors have recommended not treating massive hemoptysis complicating cystic fibrosis with emergency thoracotomy." Other authors feel emergency thoracotomy may have a role in the treatment of such patients.' No one, however, has reviewed the available literature and formulated recommendations for surgical therapy for this select population of patients. To date, only one case of emergency thoracotomy for massive hemoptysis in cystic fibrosis has been formally presented in the English literature," Seven other cases have been superficially discussed in papers reviewing other aspects of massive hemoptysis in cystic fibrosis F~om
Wilford Hall USAF Medical Center (AFSC), Lackland Air Force Base, Texas.
The opinions expressed herein are those of the authors and do not necessarily represent official USAF policy. Received for publication Jan. 7. 1983. Accepted for publication Feb. 2, 1983. Address for reprints: Captain Donald K. Porter, Pulmonary Medicine Service (SGHMMP), Wilford Hall Medical Center, Lackland Air Force Base, Texas 78236. 'Pulmonary Disease Service. Department of Medicine. "Department of Surgery.
patients. 7• 9. ,o In this report, we present a case of surgically treated massive hemoptysis in a patient with cystic fibrosis. Using the nine cases as a data base, we suggest criteria to select which patients would be acceptable candidates for emergency thoracotomy. Case report A 21-year-old white woman presented with a chief complaint of hemoptysis. In 1975, a diagnosis of cystic fibrosis had been made by means of sweat chloride testing at the National Institutes of Health (NIH). She had an uncomplicated course until May, 1979, when the first episode of hemoptysis occurred. It quickly subsided after a course of antibiotics. In July, 1980, she had a second episode of hemoptysis which resolved spontaneously. In December, 1980, the patient was admitted for treatment for significant hemoptysis (one-half cup of blood per day for 19 days) and a left upper lobe infiltrate. Bronchoscopic examination revealed the left upper lobe to be the source of bleeding, and conservative therapy with sedation and antibiotic therapy was successful. During an outpatient visit in September, 1981, the patient had pulmonary function tests revealing a forced vital capacity (FVC) of 4.69 L (III % of predicted), a forced expiratory volume in I second (FEV,) of 3.11 L (91% of predicted), and an FEV1/FVC of 66%, consistent with mild flow obstruction. ShwachmanKulczycki" clinical scoring of 85 placed her in the "good" category. In October, 1981, she again presented with significant hemoptysis estimated by the patient at 300 ml (Fig. 1). Bronchoscopy was immediately performed during which 500 ml of blood was suctioned from the left upper lobe. During bronchoscopy a Fogarty balloon catheter was inflated in the left upper lobe bronchus, and this successfully blocked the bleeding site. The patient was transferred to the medical intensive care unit, sedated, and begun on intravenous gentamicin for sputum cultures growing Pseudomonas aeruginosa. On the second hospital day, she had another unrelenting episode of massive hemoptysis at a rate exceeding 200 ml in 30 minutes. She underwent a left thoracotomy, left upper lobec-
409
410 Porter, Van Every, Mack
The Journal of Thoracic and Cardiovascular Surgery
Fig. I. Posteroanterior chest roentgenograms . Baseline film (left) is dated September, 1981. Preoperative film (center), dated October, 1981, reveals an infiltrate in the left upper lobe. Postoperative film (right) is dated March, 1982.
tomy, and lavage of remaining bronchi to extract residual blood clots. The patient had an uneventful postoperative course and was discharged from the hospital on the sixth postoperative day. Pathologic examination of the resected lobe revealed extensive bronchiectasis and lung abscesses. Presently the patient is doing well with no change in exercise tolerance and no further episodes of hemoptysis. Postoperative pulmonary function tests reveal an FVC of 4.10 L (97% of predicted), an FEY, of 2.12 L (65% of predicted) , and an FEY,/FVC of 52%, consistent with moderate obstruction.
Discussion In 1970, Levitsky, Lapey, and di Sant'Agnese" reported the first case of surgically treated massive hemoptysis in cystic fibrosis. The hemorrhage was localized to the right lung, but the patient had an FVC of only 30% of the predicted value. A ventilationperfusion scan revealed that the right lung was contributing only 20% of the entire lung function; the patient underwent a successful right pneumonectomy with no postoperative change in pulmonary function testing, exercise tolerance, or arterial blood gases. Holsclaw, Grand, and Shwachman' reported, as an addendum to their paper, two cases of patients with cystic fibrosis and Shwachman-Kulczycki clinical scores greater than 55 who underwent successful right upper lobectomy. A third patient with a clinical score of 41 died following right middle and lower lobectomy. Fellows and associates" 10 described four patients with cystic fibrosis and massive hemoptysis who were treated with lobectomy . Two of the four died after the operation, but both had Shwachman-Kulczycki clinical scores of less than 55, consistent with moderate-to-severe cystic disease.
Our patient had a clinical score of 85 and pulmonary function tests which revealed sufficient pulmonary reserve to allow lobectomy. The usual medical management, which consists of bed rest, light sedation, and intravenous antibiotics active against Pseudomonas and/or Staphylococcus species had clearly failed. Endobronchial Fogarty balloon tamponade" was successfully performed but did not prevent a recurrence of lifethreatening hemoptysis. Bronchial artery angiography and embolization, as discussed by Fellows and colleagues," was not available. This modality has been successful, however, and should be considered in patients who are obviously not surgical candidates. These reports suggest that the Shwachman-Kulczyucki clinical score may have predictive value in determining risk of operation. A score of over 55 carries an excellent prognosis (5/5 survived) whereas a score of less than 55 predicts a poor outcome (1/4 survived). This correlation between clinical score and mortality exists because of the worsening physical condition and pulmonary function that corresponds with decreasing clinical scores. The Shwachman-Kulczycki II clinical scoring system combines four criteria, specifically, the activity level of the patient, the physical examination, the nutritional status of the patient, and the chest roentgenographic fmdings, to arrive at a number which grades the severity .of a patient's cystic fibrosis. Generally, patients with clinical scores of 55 or less have dyspnea with routine activities of daily living, frequent productive cough, accessory muscle use during quiet respiration, are below
Volume 86 Number 3
Massive hemoptysis in cystic fibrosis
September. 1983
ideal body weight, and have areas of emphysematous change and/or atelectasis on chest roentgenograms. Pulmonary function tests have been compared to the Shwachman-Kulczycki scoring system. Beier and ass0elates" showed that clinical scores of 55 or less correlated with FVCs less than 50% to 60% of predicted values, and FEV! values that were less than 50% to 60% of the FVC. The residual volume-total lung capacity ratios in these patients exceeded 35%, a fact suggesting that air trapping was the reason for reduced FVCs. Godfrey and Mearns" showed that Shwachman-Kulczycki clinical scores of 55 or less correlated with maximal voluntary ventilation values of less than 45% of predicted values. These data confirm that patients with Shwachman-Kulczycki clinical scores of 55 or less have severe pulmonary function abnormalities. The 75% operative mortality seen to date in patients with clinical scores of 55 or less is due in part to their marginal physical condition and severe pulmonary function abnormalities. On the other hand, patients with clinical scores better than 55 have the physical condition and pulmonary function to withstand emergency lobectomy or pneumonectomy with low perioperative mortality. Admittedly, the number of cases (n = 9) is much too small to allow firm conclusions, but the trend is suggestive. Data from other groups'? reveal that the operative mortality for all types of patients undergoing emergency lobectomy or pneumonectomy for massive hemoptysis ranges from 0% to 36% with a mean mortality of
18%. Stern and associates" have suggested that the mortality of emergency thoracotomy is higher than the mortality of massive hemoptysis in cystic fibrosis patients. They recommended that such patients be managed only by medical means. Conversely, Holsclaw, Grand, and Shwachman,? using medical management alone, found that massive hemoptysis in patients with cystic fibrosis carried a 32% mortality within 48 hours of admission. Both groups agreed, however, that massive hemoptysis can occur in patients having from "mild" to "severe" disease, as determined by the Shwachman-Kulczycki scoring system. Furthermore, it appears that patients with clinical scores above 55 are not protected from the risk of death from massive hemoptysis. The study of Holsclaw, Grand, and Shwachman? included one patient with a clinical score of 60, indicating "mild" cystic fibrosis and presumably good pulmonary function, who died of asphyxiation soon after admission. Obviously, the mortality of massive hemoptysis in cystic fibrosis is not clearly defined. However, it appears that even patients with mild disease can die of massive hemoptysis and that the operative mortality is less than the mortality of massive hemoptysis in patients with
41 1
Shwachman-Kulczycki scores greater than 55 and adequate pulmonary function. We propose, therefore, the following criteria to select patients for emergency thoracotomy: (1) life-threatening hemoptysis in excess of 600 rnl in 24 hours, (2) accurate localization of the bleeding source, (3) Shwachman-Kulczycki clinical scores better than 55, (4) adequate pulmonary function testing, defined as an FVC, an FEV" and/or maximal voluntary ventilation of greater than 50% of predicted values, and (5) failure of medical therapy. If the patient fails to meet these criteria, we recommend nonsurgical intervention in an attempt to control massive hemoptysis. Repeated attempts at endobronchial tamponade or bronchial artery embolization may be the wiser course of action.
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11
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REFERENCES Shwachman H, Redmond A, Khaw KT: Studies in cystic fibrosis. Report of 130 patients diagnosed under three months of age over a twenty year period. Pediatrics 46:335-343, 1970 diSant'Agnese PA, Davis PB: Cystic fibrosis in adults. Am J Med 66:121-132, 1979 Garzon AA, Cerruti M, Gourin A, Karlson KE: Pulmonary resection for massive hemoptysis. Surgery 67:633638, 1970 Gourin A, Garzon AA: Operative treatment of massive hemoptysis. Ann Thorac Surg 18:52-60, 1974 Garzon AA, Gourin A: Surgical management of massive hemoptysis. Ann Surg 187:267-271, 1978 Stern RC, Wood RE, Boat TF, Matthews LW, Tucker AS, Doersheck CF: Treatment and prognosis of massive hemoptysis in cystic fibrosis. Am Rev Respir Dis 117:825828, 1978 Holsclaw DS, Grand RJ, Shwachman H: Massive hemoptysis in cystic fibrosis. J Pediatr 76:829-838, 1970 Levitsky S, Lapey A, diSant'Agnese PA: Pulmonary resection of life threatening hemoptysis in cystic fibrosis. JAMA 213:125-127, 1970 Fellows KE, Styol L, Schuster S, Khaw KT, Shwachman H: Selective bronchial angiography in patients with cystic fibrosis and massive hemoptysis. Radiology 114:551-556, 1975 Fellows KE, Khaw KT, Schuster S, Shwachman H: Bronchial artery embolization in cystic fibrosis. Technique and long-term results. J Pediatr 95:959-963, 1979 Shwachman H, Kulczycki LL: Long-term study of one hundred five patients with cystic fibrosis. Am J Dis Child 96:6-15, 1958 Gottlieb LS, Hillberg R: Endobronchial tamponade therapy for intractable hemoptysis. Chest 67:482-483, 1975 Beier FR, Reuzetti AD, Mitchel M, Watanake S: Pulmonary pathophysiology in cystic fibrosis. Am Rev Respir Dis 94:430-440, 1966 Godfrey S, Mearns M: Pulmonary function and response to exercise in cystic fibrosis. Arch Dis Child 46:144-151, 1971
THoRAc CARDIOVASC SURG
86:409-411, 1983
Emergency lobectomy for massive hemoptysis in cystic fibrosis Massive hemoptysis occurs in 50% to 70% of patients with cystic fibrosis and carries an immediate mortality of up to 32%. Cystic fibrosis is regarded by many as a disease in which thoracic operations are ill-advised. A 21-year-old woman with mild cystic fibrosis presented with unrelenting massive hemoptysis. was not helped by medical management. and underwent a successful left upper lobectomy. We suggest that thoracotomy does have a role in the management of massive hemoptysis in selected patients with cystic fibrosis. On the basis of our case and all other cases reported in the English literature. we suggest criteriafor determining which patients are suitable candidates for emergency thoracotomy.
Donald K. Porter, Captain, USAF, MC,* Marvin J. Van Every, Captain, USAF, MC,** and John W. Mack, Jr., Lieutenant Colonel, USAF, MC,** Lackland Air Force Base. Texas
h e survival rate of patients with cystic fibrosis has steadily improved such that 69% to 84% of patients are expected to survive to the age of 20 years. I With prolonged survival, pulmonary complications such as hemoptysis are being seen with increased frequency. Minor hemoptysis has been noted in 60% of patients with cystic fibrosis and massive hemoptysis, defined as a 300 ml blood loss within 24 hours, in 5% to 7%.2 Although massive hemoptysis is classically managed by surgical intervention;" certain authors have recommended not treating massive hemoptysis complicating cystic fibrosis with emergency thoracotomy." Other authors feel emergency thoracotomy may have a role in the treatment of such patients.' No one, however, has reviewed the available literature and formulated recommendations for surgical therapy for this select population of patients. To date, only one case of emergency thoracotomy for massive hemoptysis in cystic fibrosis has been formally presented in the English literature," Seven other cases have been superficially discussed in papers reviewing other aspects of massive hemoptysis in cystic fibrosis F~om
Wilford Hall USAF Medical Center (AFSC), Lackland Air Force Base, Texas.
The opinions expressed herein are those of the authors and do not necessarily represent official USAF policy. Received for publication Jan. 7. 1983. Accepted for publication Feb. 2, 1983. Address for reprints: Captain Donald K. Porter, Pulmonary Medicine Service (SGHMMP), Wilford Hall Medical Center, Lackland Air Force Base, Texas 78236. 'Pulmonary Disease Service. Department of Medicine. "Department of Surgery.
patients. 7• 9. ,o In this report, we present a case of surgically treated massive hemoptysis in a patient with cystic fibrosis. Using the nine cases as a data base, we suggest criteria to select which patients would be acceptable candidates for emergency thoracotomy. Case report A 21-year-old white woman presented with a chief complaint of hemoptysis. In 1975, a diagnosis of cystic fibrosis had been made by means of sweat chloride testing at the National Institutes of Health (NIH). She had an uncomplicated course until May, 1979, when the first episode of hemoptysis occurred. It quickly subsided after a course of antibiotics. In July, 1980, she had a second episode of hemoptysis which resolved spontaneously. In December, 1980, the patient was admitted for treatment for significant hemoptysis (one-half cup of blood per day for 19 days) and a left upper lobe infiltrate. Bronchoscopic examination revealed the left upper lobe to be the source of bleeding, and conservative therapy with sedation and antibiotic therapy was successful. During an outpatient visit in September, 1981, the patient had pulmonary function tests revealing a forced vital capacity (FVC) of 4.69 L (III % of predicted), a forced expiratory volume in I second (FEV,) of 3.11 L (91% of predicted), and an FEV1/FVC of 66%, consistent with mild flow obstruction. ShwachmanKulczycki" clinical scoring of 85 placed her in the "good" category. In October, 1981, she again presented with significant hemoptysis estimated by the patient at 300 ml (Fig. 1). Bronchoscopy was immediately performed during which 500 ml of blood was suctioned from the left upper lobe. During bronchoscopy a Fogarty balloon catheter was inflated in the left upper lobe bronchus, and this successfully blocked the bleeding site. The patient was transferred to the medical intensive care unit, sedated, and begun on intravenous gentamicin for sputum cultures growing Pseudomonas aeruginosa. On the second hospital day, she had another unrelenting episode of massive hemoptysis at a rate exceeding 200 ml in 30 minutes. She underwent a left thoracotomy, left upper lobec-
409
410 Porter, Van Every, Mack
The Journal of Thoracic and Cardiovascular Surgery
Fig. I. Posteroanterior chest roentgenograms . Baseline film (left) is dated September, 1981. Preoperative film (center), dated October, 1981, reveals an infiltrate in the left upper lobe. Postoperative film (right) is dated March, 1982.
tomy, and lavage of remaining bronchi to extract residual blood clots. The patient had an uneventful postoperative course and was discharged from the hospital on the sixth postoperative day. Pathologic examination of the resected lobe revealed extensive bronchiectasis and lung abscesses. Presently the patient is doing well with no change in exercise tolerance and no further episodes of hemoptysis. Postoperative pulmonary function tests reveal an FVC of 4.10 L (97% of predicted), an FEY, of 2.12 L (65% of predicted) , and an FEY,/FVC of 52%, consistent with moderate obstruction.
Discussion In 1970, Levitsky, Lapey, and di Sant'Agnese" reported the first case of surgically treated massive hemoptysis in cystic fibrosis. The hemorrhage was localized to the right lung, but the patient had an FVC of only 30% of the predicted value. A ventilationperfusion scan revealed that the right lung was contributing only 20% of the entire lung function; the patient underwent a successful right pneumonectomy with no postoperative change in pulmonary function testing, exercise tolerance, or arterial blood gases. Holsclaw, Grand, and Shwachman' reported, as an addendum to their paper, two cases of patients with cystic fibrosis and Shwachman-Kulczycki clinical scores greater than 55 who underwent successful right upper lobectomy. A third patient with a clinical score of 41 died following right middle and lower lobectomy. Fellows and associates" 10 described four patients with cystic fibrosis and massive hemoptysis who were treated with lobectomy . Two of the four died after the operation, but both had Shwachman-Kulczycki clinical scores of less than 55, consistent with moderate-to-severe cystic disease.
Our patient had a clinical score of 85 and pulmonary function tests which revealed sufficient pulmonary reserve to allow lobectomy. The usual medical management, which consists of bed rest, light sedation, and intravenous antibiotics active against Pseudomonas and/or Staphylococcus species had clearly failed. Endobronchial Fogarty balloon tamponade" was successfully performed but did not prevent a recurrence of lifethreatening hemoptysis. Bronchial artery angiography and embolization, as discussed by Fellows and colleagues," was not available. This modality has been successful, however, and should be considered in patients who are obviously not surgical candidates. These reports suggest that the Shwachman-Kulczyucki clinical score may have predictive value in determining risk of operation. A score of over 55 carries an excellent prognosis (5/5 survived) whereas a score of less than 55 predicts a poor outcome (1/4 survived). This correlation between clinical score and mortality exists because of the worsening physical condition and pulmonary function that corresponds with decreasing clinical scores. The Shwachman-Kulczycki II clinical scoring system combines four criteria, specifically, the activity level of the patient, the physical examination, the nutritional status of the patient, and the chest roentgenographic fmdings, to arrive at a number which grades the severity .of a patient's cystic fibrosis. Generally, patients with clinical scores of 55 or less have dyspnea with routine activities of daily living, frequent productive cough, accessory muscle use during quiet respiration, are below
Volume 86 Number 3
Massive hemoptysis in cystic fibrosis
September. 1983
ideal body weight, and have areas of emphysematous change and/or atelectasis on chest roentgenograms. Pulmonary function tests have been compared to the Shwachman-Kulczycki scoring system. Beier and ass0elates" showed that clinical scores of 55 or less correlated with FVCs less than 50% to 60% of predicted values, and FEV! values that were less than 50% to 60% of the FVC. The residual volume-total lung capacity ratios in these patients exceeded 35%, a fact suggesting that air trapping was the reason for reduced FVCs. Godfrey and Mearns" showed that Shwachman-Kulczycki clinical scores of 55 or less correlated with maximal voluntary ventilation values of less than 45% of predicted values. These data confirm that patients with Shwachman-Kulczycki clinical scores of 55 or less have severe pulmonary function abnormalities. The 75% operative mortality seen to date in patients with clinical scores of 55 or less is due in part to their marginal physical condition and severe pulmonary function abnormalities. On the other hand, patients with clinical scores better than 55 have the physical condition and pulmonary function to withstand emergency lobectomy or pneumonectomy with low perioperative mortality. Admittedly, the number of cases (n = 9) is much too small to allow firm conclusions, but the trend is suggestive. Data from other groups'? reveal that the operative mortality for all types of patients undergoing emergency lobectomy or pneumonectomy for massive hemoptysis ranges from 0% to 36% with a mean mortality of
18%. Stern and associates" have suggested that the mortality of emergency thoracotomy is higher than the mortality of massive hemoptysis in cystic fibrosis patients. They recommended that such patients be managed only by medical means. Conversely, Holsclaw, Grand, and Shwachman,? using medical management alone, found that massive hemoptysis in patients with cystic fibrosis carried a 32% mortality within 48 hours of admission. Both groups agreed, however, that massive hemoptysis can occur in patients having from "mild" to "severe" disease, as determined by the Shwachman-Kulczycki scoring system. Furthermore, it appears that patients with clinical scores above 55 are not protected from the risk of death from massive hemoptysis. The study of Holsclaw, Grand, and Shwachman? included one patient with a clinical score of 60, indicating "mild" cystic fibrosis and presumably good pulmonary function, who died of asphyxiation soon after admission. Obviously, the mortality of massive hemoptysis in cystic fibrosis is not clearly defined. However, it appears that even patients with mild disease can die of massive hemoptysis and that the operative mortality is less than the mortality of massive hemoptysis in patients with
41 1
Shwachman-Kulczycki scores greater than 55 and adequate pulmonary function. We propose, therefore, the following criteria to select patients for emergency thoracotomy: (1) life-threatening hemoptysis in excess of 600 rnl in 24 hours, (2) accurate localization of the bleeding source, (3) Shwachman-Kulczycki clinical scores better than 55, (4) adequate pulmonary function testing, defined as an FVC, an FEV" and/or maximal voluntary ventilation of greater than 50% of predicted values, and (5) failure of medical therapy. If the patient fails to meet these criteria, we recommend nonsurgical intervention in an attempt to control massive hemoptysis. Repeated attempts at endobronchial tamponade or bronchial artery embolization may be the wiser course of action.
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4 5 6
7 8
9
10
11
12 13
14
REFERENCES Shwachman H, Redmond A, Khaw KT: Studies in cystic fibrosis. Report of 130 patients diagnosed under three months of age over a twenty year period. Pediatrics 46:335-343, 1970 diSant'Agnese PA, Davis PB: Cystic fibrosis in adults. Am J Med 66:121-132, 1979 Garzon AA, Cerruti M, Gourin A, Karlson KE: Pulmonary resection for massive hemoptysis. Surgery 67:633638, 1970 Gourin A, Garzon AA: Operative treatment of massive hemoptysis. Ann Thorac Surg 18:52-60, 1974 Garzon AA, Gourin A: Surgical management of massive hemoptysis. Ann Surg 187:267-271, 1978 Stern RC, Wood RE, Boat TF, Matthews LW, Tucker AS, Doersheck CF: Treatment and prognosis of massive hemoptysis in cystic fibrosis. Am Rev Respir Dis 117:825828, 1978 Holsclaw DS, Grand RJ, Shwachman H: Massive hemoptysis in cystic fibrosis. J Pediatr 76:829-838, 1970 Levitsky S, Lapey A, diSant'Agnese PA: Pulmonary resection of life threatening hemoptysis in cystic fibrosis. JAMA 213:125-127, 1970 Fellows KE, Styol L, Schuster S, Khaw KT, Shwachman H: Selective bronchial angiography in patients with cystic fibrosis and massive hemoptysis. Radiology 114:551-556, 1975 Fellows KE, Khaw KT, Schuster S, Shwachman H: Bronchial artery embolization in cystic fibrosis. Technique and long-term results. J Pediatr 95:959-963, 1979 Shwachman H, Kulczycki LL: Long-term study of one hundred five patients with cystic fibrosis. Am J Dis Child 96:6-15, 1958 Gottlieb LS, Hillberg R: Endobronchial tamponade therapy for intractable hemoptysis. Chest 67:482-483, 1975 Beier FR, Reuzetti AD, Mitchel M, Watanake S: Pulmonary pathophysiology in cystic fibrosis. Am Rev Respir Dis 94:430-440, 1966 Godfrey S, Mearns M: Pulmonary function and response to exercise in cystic fibrosis. Arch Dis Child 46:144-151, 1971