CHEST - GENERAL. COMPLICATIONS. TRANSPLANTATION

1953 di Sant’Agnese PA. Bronchial obstruction with lobar atelectasis and emphysema in cystic fibrosis of pancreas. Pediatrics 1953; 2:178-190. [PubMed]
This paper describes the classical right upper lobe collapse so characteristic of CF and notes bronchoscopy was usually not effective in expanding the collapsed lobe (however, only rigid bronchoscopes were available at that time). Of 211 children with CF 10% presented with collapse of one or more lobes. In the discussion there was still a lingering suggestion that vitamin A deficiency “may contribute but not a major cause”.
Harry Shwachman agreed that atelectasis worsens the prognosis but also noted “The hypothesis suggesting an imbalance of the autonomic nervous system which may affect many systems and organs is attractive”. So there was still no clear explanation for the various manifestations of the condition; some suspected the autonomic nervous system. With regard to the association of the pancreatic lesions and the pulmonary complications, di Sant’Agnese concludes that “a satisfactory understanding of this problem has not yet been attained” which seemed to sum up the situation.

1957 White R Jr, Dent JH, Derbes VJ. Asthmatic states caused by mucoviscidosis. J Louisiana St Med S 1957; 109:299-302. [PubMed]
Thirty eight of 66 children with CF from New Orleans had some degree of wheezing. The authors comment “It has been the common experience of mature pediatricians that administration of epinephrine in such circumstances has led to prompt, albeit transient, alleviation”. The mechanical factors that cause impairment of pulmonary function in asthma – bronchospastic contraction, edema of the mucous membrane and excessive secretion and retention of mucus, are present both in CF and asthma. The authors' message from their paper being - “it is the responsibility of all physicians who treat wheezing children to exclude this disorder (i.e. cystic fibrosis)”.

1966 Paxton DG, Scott EP. Pneumothorax: An unusual complication in fibrocystic disease. Am J Dis Child 1966; 111:311-312. [PubMed]
The authors did not find previous reports of pneumothorax in the American literature – only in text books. However, prior to this report there had been sporadic reports of pneumothorax in CF in the literature from 1947 (Fanconi G, Metaxas-Buhler M. Helv Paediat Acta 1947; 2:289-295). Also Doershuk (1964) had one example in 96 patients.
This present report is of sudden onset of left pneumothorax in a 12 year old girl with CF whose chest showed “far advanced changes” at the age of 7.5years (figure 24). With a tube and drainage the lung expanded in 2 days (figure 25); she was discharged in 6 days but died 4 months later from her pulmonary disease.
Pneumothorax is a complication which occurs in those with more advanced chest damage and is now very rare in children with CF. For example in the 2004 UK CF Registry there were no children reported as having pneumothorax and only one in an adult with cystic fibrosis.

1970 Holsclaw DS, Grand RJ, Shwachman H. Massive haemoptysis in cystic fibrosis. Pediatrics 1970; 75:829-838. [PubMed]
First report dealing primarily with 29 episodes of haemoptysis – a serious complication as 13 of the 19 patients died within six months. Treatment was with transfusions and supportive care and surgical resection in one patient (also reported by Lenvitsky S, et al, JAMA 1970; 213:125-127).
Arterial embolisation had not been described as yet and the technique certainly improved the prognosis - for example Stern RC et al, (Am Rev Resp Dis 1977; 117:825) had no deaths in 38 patients although 48% had recurrent episodes. Also a series of 13 patients treated by bronchial artery embolisation was reported from Shwachman’s unit (Fellows KE et al, J Pediatr 1979; 95:959-963 below).

1979 Fellows KE, Kon Taik Khaw, Schuster S, Shwachman H. Bronchial artery embolisation in cystic fibrosis; technique and long term results. J Pediatr 1979; 93:959-963. [PubMed]
This technique was first reported in other diseases (Wholey MH et al, JAMA 1976; 236: 2501) and in two patients with CF in a series of 104 with various conditions (Remy J et al, Radiology 1977; 122:33). In this present series major bleeding ceased in 12 of the 13 patients with CF although five had minor recurrences. The authors advised the procedure should be limited to life-threatening episodes (also Holsclaw DS et al, 1970; Stern et al, 1977 both above)

1980 Wood RE, Sherman JM. Pediatric flexible bronchoscopy. Ann Otol Rhinol Laryngol 1980; 89:414-416. [PubMed]
The first report of the use of the paediatric flexible fibreoptic bronchoscope in children by Robert Wood who pioneered the technique. He described a prototype flexible paediatric bronchoscope that he had used to perform both diagnostic and therapeutic procedures on pediatric patients ranging from infants of 840 g to children aged 14 years. Flexible bronchoscopy, with appropriate instrumentation and careful attention to physiological requirements of the patient, was found to be safe and effective in young patients. Wood correctly forecast that with this instrument, the indications for bronchoscopy in children would be considerably expanded. (Also there is an excellent review of flexible bronchoscopy by Wood RE. J Pediatr 1988; 112:1-6; also Wood RE. “Pediatric flexible bronchoscopy: The inside story”. In: Doershuk CF, (Ed.). Cystic Fibrosis in the Twentieth Century. Cleveland: AM Publishing Ltd, 2001:112-119.With permission). Expansion continues and it has even been suggested that every infant identified by newborn screening should be bonchoscoped to obtain cultures of the lower airways (Hilliard TN et al. Arch Dis Child 2007; 92:898-899).

1985 (1990) Yacoub MH, Banner NR, Khaghani A, FitzGerald M, Madden B, Tsang V, Hodson M. Heart-lung transplantation for cystic fibrosis and subsequent domino heart transplantation. J Heart Transplant 1990; 9:459-467. [PubMed]
A major advance, for those who had reached the end stages of their disease (an FEV1 predicted of less than 30%), was the successful introduction of heart-lung transplantation in 1985, pioneered by Sir Magdi Yacoub (figure 29) and his team in London (Yacoub et al, 1990; Scott et al, 1988 below) and mr John Wallwork and his team in Cambridge. The possibility of successful treatment in what were previously the terminal stages of the condition had a major influence on both prognosis and the treatment of severely affected individuals.
The first results of heart-lung transplantations were quite remarkable and were related both to surgical skills, concentrated medical expertise in assessment and after care and also to more successful immunosuppressive therapy to prevent rejection of the transplanted organs. Later double lung transplants became more popular (Pasque et al, 1990 below) and are now the most commonly performed operation. Living donor lung transplants have proved successful in some centres and will be an obvious choice for some families in the absence of suitable donor organs (Cohen & Starnes, 2001 below).

1987 Penketh A, Higenbottam T, Hakim M, Wallwork J. Heart lung transplantation in patients with end stage lung disease. BMJ 1987; 295:311-314. [PubMed]
The cardiothoracic surgical team at Papworth, Cambridge UK under the leadership of Mr John Wallwork (figure 41.1) report preliminary results of heart-lung transplantation in seven patients one of whom had CF. Six of the seven patients were well after four to 33 months. Papworth became one of the major UK transplant centres for people with cystic fibrosis. Further experience was published in 1989 where five of 33 patients referred benefited over 18 months (Smyth RL et al. Arch Dis Child 1989; 64: 1225-1229). The first heart lung transplants for CF were performed by Mr. Magdi Yacoub in 1985 in London at Harefields Hospital (Yacoub et al, 1990 below).

1988 Jones K, Higenbottam T, Wallwork J. Successful heart-lung transplantation for cystic fibrosis. Chest 1988; 93:644-5. [PubMed]
Report of one of the first successful heart lung transplants in CF in October 1985 by Mr Wallwork’s team at Papworth Hospital, Cambridge. Sixteen months after heart-lung transplantation, the FEV1 of a young woman, who had been in the terminal stages of CF, has risen from 16 percent (0.6 L) to 77 percent of her predicted value. She had returned to work.
These first reports of heart lung transplantation for a condition which was uniformly fatal were quite dramatic and excited a great deal of hope and interest in the CF community. John Wallwork and Magdi Yacoub were leaders in this field in the UK for the next few years. (also Penketh et al above; Scott J et al. Heart lung transplantation for cystic fibrosis. Lancet 1988; ii: 192-194 reporting five of six patients with CF survived heart lung transplantation at Papworth Hospital). Later Prof John Dark and Prof Paul Corris in Newcastle developed a major transplant service.

1990 Pasque MK, Cooper JD, Kaiser LR, Haydock DA, Triantafillou A, Trulock EP. Improved technique for bilateral lung transplantation: rationale and initial clinical experience. Ann Thorac Surg 1990; 49:785-791. [PubMed]
The first bilateral lung transplantation in CF was performed in Toronto in 1988 and 17 were carried out between 1988 and 1991 (Ramirez JC et al. J Thorac Cardiovac Surg 1992; 103:287-293. [PubMed] ). This improved operation was done through a transverse thoracosternotomy and involves sequential replacement of the two lungs. Positive features included separate bronchial anastomoses to reduce ischemic airway complications and elimination of the need for total cardiopulmonary bypass. Three patients were reported, one had CF, all recovered without complications and post operative function was excellent. The double lung transplant operation gradually replaced heart-lung transplants for people with cystic fibrosis.

1990 Yacoub M, Banner NR, Khaghani A, Fitzgerald M, Madden B, Tsang V, Smyth R, Hodson ME. Heart lung transplantation for CF and subsequent domino cardiac transplantation. J Heart Transplantation 1990; 9:459-67. [PubMed]
Between September 1984 and October 1988, 27 patients underwent combined heart-lung transplantation for treatment of end-stage respiratory disease caused by CF: survival was 78% at 1 year and 72% at 2 years. Lung function was greatly improved after transplantation, and long-term survivors achieved an excellent quality of life. Lymphoproliferative disorders developed in two patients; these disorders regressed after a reduction in immunosuppression. Two patients required retransplantation: one because of obliterative bronchiolitis and the other because of recurrent respiratory infections associated with a moderate tracheal stenosis and severe deterioration in lung function. A modification of the technique used for heart-lung transplantation allowed 20 hearts from cystic fibrosis patients to be used for subsequent heart transplantation.
This report by Mr Yacoub and his team, who performed the first heart lung transplantations in people with CF at Harefields Hospital, London in 1984. There were also reports from Mr Wallwork’s unit at Papworth, Cambridge (Penketh et al, 1987 above; Jones et al, 1988 above and later from Professors Dark and Paul Corrie from Newcastle)

1991 Hodson ME, Madden BP, Steven MH, Tsang VT, Yacoub MH. Non-invasive mechanical ventilation of CF patients – the bridge to transplantation. Eur Resp J 1991; 4:524-7. [PubMed]
This technique of non-invasive ventilation was developed as a direct result of attempting to prolong the survival of people with CF awaiting a heart-lung transplant at the Brompton Hospital – prior to the possibility of heart-lung transplantation assisted ventilation was usually regarded as inappropriate for people with CF in respiratory failure as their prognosis was uniformly bad. The case histories of six patients with CF using nasal ventilation while awaiting heart-lung transplantation are reviewed; four patients did well. This method of ventilation proved to be a useful bridge to transplantation when a patient suddenly deteriorates. This is also a very cost effective method of ventilation and does not encroach on conventional Intensive Care Unit facilities.

1991 Warner JO. Heart-lung transplantation: all the facts. Arch Dis Child 1991; 66:1013-1017. [PubMed]
John Warner, then paediatrician at the Brompton Hospital, London, wrote this cautionary article on heart lung transplantation as it applied to children in the UK and called for open discussion on the subject. His article was published alongside two papers on heart-lung transplantation in children from Great Ormond Street (Whitehead B et al. Arch Dis Child 1991;66;1018-1021 & Whitehead B et al, Arch Dis Child 1991; 66:1022-1026). Of 27 children referred to GOS for assessment of suitability for heart-lung transplant, 10 (37%) were actually transplanted. Six were still alive from three months to three years after the operation but two thirds of the cohort had died at various stages during referral, assessment, and transplant.
Warner maintained that while the transplant has offered miraculous new life to a few children, many more have experienced increased and unnecessary suffering. Planning of transplant programmes must take all facts into account. Also he emphasised that the possibility of heart-lung transplant must not deter further efforts to control chronic lung diseases medically and must not influence appropriate terminal care.
These were early days for transplantation (the first adults had only received transplants in 1984), and although there were some similar views, the latest being in 2008, the outlook for children having transplants continued to improve although as for older patients shortage of donor organs remained a problem.
Figure 24: Sonny Laing’s CF was not diagnosed until she was 18 months old when she was referred to Leeds by which time her lungs were irreparably severely damaged. She was the youngest child with CF to receive a heart-lung transplant at Great Ormond Street, London when she was 5 years old. In the figure she is 15 years old, very well, active and a keen gymnast.

1992 Madden BP, Hodson ME, Tsang V, Radley-Smith R, Khaghani A, Yacoub MY. Intermediate term results of heart-lung transplantation for cystic fibrosis. Lancet 1992; 339:1583-1587. [PubMed]
Between 1984 and 1991 79 patients with CF had heart-lung transplantations in London by Mr Yacoub’s team with a 69% survival to 1 year, 52% to 2 yrs and 49% to 3 years. Cumulative probability of development of obliterative bronchitis was 17%, 23% and 48% at 1, 2, and 3 years. These were encouraging results but the authors noted serious shortage of donor organs – which in 2008 was still a major problem in the UK (Penketh et al, 1987 above; Yacoub et al, 1990 above; Scott et al, 1988 above for first reports from Brompton and Harefields and Cambridge

2000 Goldstein AB, Goldstein LS, Perl MK, Haug MT, Arroliga AC, Stillwell PC. Cystic fibrosis patients with and without central nervous system complications following lung transplantation. Pediatr Pulmonol 2000; 30:203-206). [PubMed]
Eleven of 21 patients (52%) with CF had CNS events after lung transplantation: eight had seizures, five severe headaches, three had strokes, and one had a confusional episode. The authors could not identify any predictive risk factors but considered cyclosporine toxicity to be the major cause of the CNS complications. Despite the high rate of CNS events, the overall prognosis for these patients was good, and their 6-month survival was not affected.

A surprisingly high percentage of people with CF have neurological problems after lung transplantation. CNS complications are not common in CF but Arnold-Chiari malformations have been reported (Needleman JP et al. Pediatr Pulmonol 2000; 30:490 above).

2001 Cohen RG, Starnes VA. Living donor lung transplantation. World J Surg 2001; 25: 244-250. [PubMed].
Since 1993 a total of 101 living-donor bilateral lung transplants had been performed with acceptable results. Though most recipients were patients with CF who were rapidly deteriorating, the indications for live-donor lung transplantation had been expanded to include some CF patients in a more elective setting, as well as select patients with other end-stage pulmonary diseases. One-year Kaplan-Meier recipient survival is 72%. Seventy-six percent of deaths occur within the first 2 months after transplantation. There has been no donor mortality and 83% had no problems.

This is a remarkable series which does not appear to have been replicated anywhere else, although there are further publications on the subject from Starnes group reporting further experience (Backhus LM. Et al. J Heart Lung Transpl 2005; 24:2086-90) [PubMed]. Eighty-seven transplants were performed on 84 adult recipients from 1993 through 2003 -  76 had cystic fibrosis. Starnes had been publishing on the subject of living donor lung transplants since 1996. The technique has not become popular in the UK.

2001 Sood N. Paradowski LJ. Yankaskas JR. Outcomes of intensive care unit care in adults with cystic fibrosis. Am J Resp Crit Care 2001; 163:335-338. [PubMed]
Mechanical ventilation has been discouraged in CF because of poor outcomes, but improved survival and the availability of lung transplantation have increased the indications for care of CF patients in the intensive care unit (ICU). We studied the outcomes of all CF patients admitted to the University of North Carolina Hospitals Medical ICU from January 1990 through December 1998. Seventy-six patients, ranging in ages from 17 to 45 yr (mean: 27 yr), and of whom 53% were female, had 136 admissions for exacerbations of CF with respiratory failure (RF, n = 65), hemoptysis (n = 33), antibiotic desensitization (n = 30), pneumothorax (n = 3), or other reasons (n = 5). Eighty-six percent of the patients with hemoptysis and all of those with desensitization and pneumothorax were alive 1 yr after ICU discharge. Of the 42 patients with RF, 37 (88%) required assisted ventilation. Twenty-three (55%) of the patients with RF survived to ICU discharge and 19 (45%) died. Seventeen (40%) of the patients with RF received lung transplants and 14 were alive 1 yr later. Without transplantation, three (7%) of the patients with RF were alive and three (7%) were dead 1 yr later. Sex, body mass index, and respiratory bacteria did not correlate with survival. We conclude that ICU care for adults with CF who have reversible complications is appropriate and effective. Ventilatory support is appropriate for some transplant candidates.

This is a useful indication of the fate of people with CF admitted to an ICU in a major CF Centre at the present time. Undoubtedly the policy of not admitting to an ICU or ventilating people with CF in no longer valid.

2005 Davies JC, Davies M, McShane D, Smith S, Chadwick S, Jaffe A, Farley R, Collins L, Bush A, Scallon M, et al. Potential difference measurements in the lower airway of children with and without cystic fibrosis. Am J Respir Crit Care Med 2005; 171:1015-1019. [PubMed]      
The potential difference measurements were made via a bronchoscope from the lower respiratory tracts of children as young as 1 year. Tracheal baseline values were significantly higher in children with CF than those without, although this was not the case more distally. In airways between the third and seventh generation, perfusion with a zero chloride solution containing isoprenaline led to a significant change in potential difference in children without CF, whereas no change was seen in those with CF. This measure provided a reliable distinguishing test between the two disease groups This is the first report of this technique in children.

Jane Davies (figure 7) is the senior paediatrician with the UK Gene Therapy Consortium at the Royal Brompton Hospital, London. Airway potential measurements are one of the end point measurements used by the Gene Therapy Consortium in their gene therapy trials which started in 2009.  The trials involve patients down to the age of 12 years. Eventually younger children will be involved in trials of either gene therapy or protein based therapies.

2006 Hafen GM. Ukoumunne OC. Robinson PJ. Pneumothorax in cystic fibrosis: a retrospective case series. Arch Dis Child 2006; 91:924-925.[PubMed] Pneumothorax is a known complication in cystic fibrosis (CF), associated with poor outcome. Records of CF patients with pneumothorax at the Royal Children's Hospital, Melbourne between 1990 and 2004 were reviewed, and the characteristics, sputum culture results, lung function, treatment, and outcome for the 11 patients who had pneumothoraces were described.

Although a small series, this is a useful report as pneumothorax is now rare in children with CF.

2006 Vidal V. Therasse E. Berthiaume Y. Bommart S. Giroux MF. Oliva VL. Abrahamowicz M. du Berger R. Jeanneret A. Soulez G. Bronchial artery embolization in adults with cystic fibrosis: impact on the clinical course and survival. J Vasc Intervent Radiol 2006; 17:953-958 [PubMed]
Although bronchial artery embolization (BAE) is effective in the acute control of recurrent or major hemoptysis in adults with cystic fibrosis, outcomes after embolization are not well known. Of 297 patients with cystic fibrosis hospitalized from 1990 to 2004, 30 patients (mean age, 26.7+/-9.2 years) presented with major or persistent hemoptysis that required 42 BAE sessions. These patients were compared with a control group of 27 patients without hemoptysis requiring embolization who were matched for age, sex, and forced expiratory volume in 1 second (FEV1). Hemoptysis stopped within 24 hours after BAE in 96.6% of patients (n=29), and there were no major complications. The change in the slope of FEV1 after the BAE or matching date was significantly worse in the embolization group (P=.0007). At last follow-up, nine and one patients, respectively, had undergone lung transplantation in the BAE and control groups (P=.002). The 5-year survival rates without lung transplantation were 31% and 84%, respectively, in the BAE and control groups (hazard ratio, 5.95; P=.002). Sixty-two percent of patients were free of hemoptysis 5 years after BAE. The number of collateral arteries was the only factor associated with the risk of death or recurrent hemoptysis (P=.001). Despite the effectiveness of embolization in controlling recurrent or major hemoptysis, adults with cystic fibrosis who have undergone BAE for hemoptysis are at much higher risk of respiratory function aggravation, death, and the need for lung transplantation than those who have not undergone BAE for hemoptysis. They are more likely to die or to undergo lung transplantation than to present with recurrent major hemoptysis

This is useful extensive experience of treating haemoptysis in people with CF. As to be expected the patients with haemoptysis had more severe chest involvement and generally a worse prognosis but the procedure was successful and free of major complications with regard to the controlling the bleeding.

2006 Slieker MG. van Gestel JP. Heijerman HG. Tramper-Stranders GA. van Berkhout FT. van der Ent CK. Jansen NJ. Outcome of assisted ventilation for acute respiratory failure in cystic fibrosis. Intens Care Med 2006; 32:754-758. [PubMed]
To assess outcome of assisted ventilation in cystic fibrosis (CF) patients with acute respiratory failure (ARF), to identify risk factors associated with poor outcome and to compare long-term outcome of CF children who were mechanically ventilated for ARF with unventilated CF controls. A retrospective cohort study in two large CF centres in the Netherlands. Thirty-one CF patients required assisted ventilation for ARF between January 1990 and March 2005. All five children (under 2 years of age) and seven adults (27%) survived. CF patients younger than 2 years old, who are ventilated because of ARF, have a good prognosis and their long-term outcome seems identical to unventilated CF controls. ARF in adult CF patients still is associated with high mortality, especially among patients with acute on chronic respiratory failure.

The resort to assisted ventilation has changed over the years now there is the possibility of transplantation; prior to the mid Eighties before the first successful transplantations ventilation was regarded as inappropriate.

2006 Montgomery GS. Sagel SD. Taylor AL. Abman SH. Effects of sildenafil on pulmonary hypertension and exercise tolerance in severe cystic fibrosis-related lung disease. Pediatr Pulmonol 2006; 41:383-385. [PubMed]
Cystic fibrosis (CF) patients with advanced lung disease are at risk for developing pulmonary vascular disease and pulmonary hypertension, characterized by progressive exercise intolerance beyond the exercise-limiting effects of airways disease in CF. We report on a patient with severe CF lung disease who experienced clinically significant improvements in exercise tolerance and pulmonary hypertension without changing lung function during sildenafil therapy.

Sildenafil has been reported to have a favourable effect on the DF508 mutation although no clinical trial have bee reported (by 2011). Here the drug is used to treat pulmonary hypertension which is common in severe CF lung disease.

2007 Hilliard TN, Sukhani S, Francis J, Madden N, Rosenthal M Balfour-Lynn I, Bush A, Davies JC. Bronchoscopy following diagnosis with cystic fibrosis. Arch Dis Child 2007; 92:898-899. [PubMed]
The authors recently changed their practice and performed bronchoscopy following a diagnosis of cystic fibrosis. On a retrospective review of 25 children, Pseudomonas aeruginosa was detected in bronchoalveolar lavage for the first time in five children (20%) and Staphylococcus aureus in four (16%). Lavage culture was positive in eight of the 18 children without respiratory symptoms. The authors suggest that these findings highlight the potential of bronchoscopy following diagnosis, even in asymptomatic children.

Whether to recommend bronchoscopy in an asymptomatic screened infant with CF is dependent on many factors, not least, where the infant was born and the facilities and skill available for paediatric respiratory investigation. Also, the treatment policy of the unit responsible for the care of the infants. Also there is the potential danger of infecting an, as yet uninfected, infant with the instrument if sterilisation has been faulty; also hospitalisation does present a definite infection risk to infants with CF. If the infants were started on prophylactic flucloxacillin from diagnosis (as is recommended by the UK CF Trust's expert Antibiotic Group 2009), it is very unlikely that S. aureus would have been cultured. Also units where screening has been routine for many years, such as Leeds, have managed to achieve very low levels of chronic Pseudomonas infection using only frequent throat cultures, cough swabs and serum antibody levels to recognise and treat early P. aeruginosa infection. If an infant with CF had repeatedly negative upper respiratory tract cultures and negative Pseudomonas antibody levels it would be very unlikely there would be positive bronchial cultures. So “bronchoscopy for all at diagnosis, although it may be decided is desirable, is definitely a policy that needs careful discussion before being applied generally.

The recent multicentre study completed in 2009 comparing regular bronchoscopy with routine care led by Claire Wainwright of Brisbane does not show a significant advantage for those who have regular bronchoscopies.

2007 Corno V, Dezza MC, Lucianetti A, Codazzi D, Carrara B, Pinelli D, Parigi PC, Guizzetti M, Strazzabosco M, Melzi ML, Gaffuri G, Sonzogni V, Rossi A, Fagiuoli S, Colledan M. Combined double lung-liver transplantation for cystic fibrosis without cardio-pulmonary by-pass. Am J Transpl2007; 7:2433-2438. [PubMed]
The authors performed sequential bilateral single lung-liver transplantation - a therapeutic option for patients with end stage lung and liver disease. They performed the operation in three young men affected by CF. All the recipients had respiratory failure and portal hypertension with hypersplenism. The three recipients are alive with a median follow-up of 670 days (range 244-1,533). The operation is a complex but effective procedure for the treatment of end stage lung disease due to CF.

2008 McLaughlin AM. McGrath E. Barry R. Egan JJ. Gallagher CG. Treatment of lobar atelectasis with bronchoscopically administered recombinant human deoxyribonuclease in cystic fibrosis? Clin Respir J 2008; 2:123-126. 20298317. [PubMed] The objective of this study was to describe our experience in which rhDNase (Pulmozyme) was administered by bronchoscopic instillation into atelectatic lobes in five adults with CF. This method was successful in treating lobar atelectasis, which was resistant to conventional therapy with antibiotics and physiotherapy. In all but one of the cases we described, administration of DNase in this manner resulted in a radiographic and clinical improvement of the atelectasis. We recommend that respiratory physicians consider this as a second line treatment in the management of atelectasis.

Although not the first to use this treatment for atelectasis, confirmation of the success of this treatment is useful for clinicians faced with resistant atelectasis.

2009 McDermott S. Barry SC. Judge EE. Collins S. de Jong PA. Tiddens HA. McKone EF. Gallagher CC. Dodd JD. Tracheomalacia in adults with cystic fibrosis: determination of prevalence and severity with dynamic cine CT. Radiology 2009; 252:577- 586. [PubMed]
To determine the prevalence and severity of tracheomalacia in adults with cystic fibrosis (CF) by using dynamic cine multidetector computed tomography (CT) and to correlate these findings with pulmonary function test (PFT) results and the severity of parenchymal lung disease. Tracheomalacia was demonstrated in 24 (69%) patients and no control subjects during forced expiratory maneuvers (P = .001) and in 10 (29%) patients and one (10%) control subject during coughing. There was no correlation between tracheal cross-sectional luminal reduction and either predicted FEV(1) or CT Bhalla score. Tracheomalacia depicted at dynamic cine multidetector CT is a highly prevalent finding in adults with CF.

Clinically tracheomalacia has been observed on occasion but the present study demonstrates how common is the condition. It is interesting that similar tracheal abnormalites occur commonly in CF pigs in particular some degree of maldevelopment of the tracheal cartilage.