- Email: [email protected]
242 Pneumothorax in patients with cystic fibrosis
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Poster Sessions / Journal of Cystic Fibrosis 16S1 (2017) S63–S174
immediate success in 81%. Mean follow-up time was 22 and 35 months for the cases and controls, respectively. Of the 16 patients embolized: 3 had recurrences within 24 h (2 major, requiring new BAE), 11 had late recurrences (4 major requiring BAE and 1 surgery), and 2 died. Of the 5 non-embolized patients: 1 died, 1 had minimal recurrence, and 3 had serious recurrences (1 embolization and 1 lung transplant). Risk factors of massive hemoptysis were increased by previous mild repeated hemoptysis (HR 24.7, p = 0.002), and by the presence of ABPA (HR 3.4l, p = 0.04). The relative risk of hemoptysis was increased in patients with impaired FEV1 (FEV1 < 45%, HR 21, 45 < FEV1 < 70%, p = 0.03). During follow-up 5 cases (including 3 in the acute phase) and 1 control died from infectious respiratory failure. In the 4 years, the number of deaths and lung transplantations was higher in cases compared to controls (HR 7.1, p = 0.0004). Conclusion: Massive hemoptysis is associated with more altered respiratory function, leading to mortality or lung transplant within 4 years, and with the presence of ABPA. 240 Descriptive analysis of atelectasis as a pulmonary complication in cystic fibrosis M. Martínez-Redondo1, C. Prados Sánchez1, F. García-Río1, M.E. Quintana-Gallego2, S. Castillo3, A. Salcedo Posadas4, R.M. Girón5, M.T. Martínez Martínez6, L. Máiz7, C. Martín8, J. Costa9. 1La Paz University Hospital, Pulmonology, Madrid, Spain; 2Virgen del Rocío University Hospital, Pulmonology, Sevilla, Spain; 3Clinical Hospital of Valencia, Pulmonology, Valencia, Spain; 4Niño Jesús - Gregorio Marañón University Hospital, Pulmonology, Madrid, Spain; 5La Princesa University Hospital, Pulmonology, Madrid, Spain; 6Doce de Octubre University Hospital, Pulmonology, Madrid, Spain; 7Ramón y Cajal University Hospital, Pulmonology, Madrid, Spain; 8 Miguel Servet University Hospital, Pulmonology, Zaragoza, Spain; 9San Juan de Dios Hospital, Pulmonology, Barcelona, Spain Objective: to analyze atelectasis as a complication of cystic fibrosis (CF). Methods: a retrospective study has been performed on CF units of Spain. Cases of CF with atelectasis were included until Dec 2015 and controls of the same sex and d.o.b. ± 3 years. The study variables were demographic data, respiratory infections, pulmonary complications, comorbidities associated with CF, pulmonary function, diagnostic imaging, medical and bronchoscopic treatment and subsequent clinical and radiological improvement. Data were collected in different follow-up periods, following the ethical standards of each center. The SSPS version 13 was used. Comparison of variables: Student-t, Mann-Whitney and Chi-square tests according to their distribution. Relationship analysis: Chi-square test. Results: 38 patients had atelectasis (4–50 yrs), 23 were women (60.5%) and 15 were men (39.5%) with a mean age of 24.58 ± 11.9 yrs, compared to 39 controls (5–47 yrs), 26 were women (66.7%) and 13 were men (33.3%), with a mean age of 25.08 ± 1.84 yrs. Mutations: 20 homozygous F508del (26%), 37 heterozygous F508del (37%), 8 F508del/unknown (10.4%), 12 others (15.6%), p = 0.348. Location: 28 first lobar atelectasis (13 LUL) and 4 2nd lobar atelectasis (2 RUL, 2 LUL, 2 RML and 1 RLL). Pulmonary complications: ABPA was only statistically significant ( p = 0.006), presenting in 10 patients [9 cases (23.7%)]. Chronic colonization in last registry: there are no statistically significant differences between cases and controls. At the time of atelectasis (30 cases): P. aeruginosa 55.3%, MSSA 34.2%, A xylosoxidans 7.9%, NTM 7.9%, MRSA 5.3%, S. maltophila 5.3%, TB 0%, others 31.6%. There were no significant differences between the treatments before and after atelectasis: reinforcement of therapies to reduce sputum viscosity and inflammation. Respiratory function tests are detailed in Poster Figure 2. Conclusions: • Atelectasis cause a decline in lung function. • Prior ABPA could favor later atelectasis.
241 Atelectasis as a poor prognosis factor in cystic fibrosis M. Martínez-Redondo1, C. Prados Sánchez1, F. García-Río1, M.E. Quintana-Gallego2, S. Castillo3, A. Salcedo Posadas4, R.M. Girón5, M.T. Martínez Martínez6, L. Máiz Carro7, C. Martín8, J. Costa9. 1La Paz University Hospital, Pulmonology, Madrid, Spain; 2Virgen del Rocío University Hospital, Pulmonology, Sevilla, Spain; 3Clinical Hospital of Valencia, Pulmonology, Valencia, Spain; 4Niño Jesús - Gregorio Marañón University Hospital, Pulmonology, Madrid, Spain; 5La Princesa University Hospital, Pulmonology, Madrid, Spain; 6Doce de Octubre University Hospital, Pulmonology, Madrid, Spain; 7Ramón y Cajal University Hospital, Pulmonology, Madrid, Spain; 8Miguel Servet University Hospital, Pulmonology, Zaragoza, Spain; 9San Juan de Dios Hospital, Pulmonology, Barcelona, Spain Objectives: The aim of this study was to analyze pulmonary atelectasis in cystic fibrosis (CF) as a poor prognostic factor. Methods: A retrospective study has been performed on cystic fibrosis units of Spain. Cases of cystic fibrosis with atelectasis have been included until December 2015 and controls of the same sex and date of birth ± 3 years. The study variables were demographic data, respiratory infections, pulmonary complications, comorbidities associated with cystic fibrosis, pulmonary function, diagnostic imaging of atelectasis, medical and bronchoscopic treatment and subsequent clinical and radiological improvement. Data were collected in different follow-up periods, following the ethical standards of each center. The SSPS version 13 program was used. For the comparison of variables, Student-t, Mann-Whitney and Chi-square tests were used according to their distribution. For the relationship analysis, the Chi-square test was used. Results: in our series, 38 patients had atelectasis (between 4 and 50 years), which 23 were women (60.5%), with a mean age of 24.58 ± 11.9 years, compared to 39 controls (between 5 and 47 years), which 26 were female (66.7%) with a mean age of 25.08 ± 11.84 years. 4 cases died (10.5%) at the ages of 26, 30, 31 and 33 years and 1 control (2.6%) at 5 years ( p = 0.171). 3 cases with unresolved atelectasis after the treatment were transplanted with a FEV1 of 18%, 21% and 31% of predicted. Regarding the respiratory function tests, there was no significance in the overall annual decline compared to the annual decline after the first and second atelectasis in ml/year, % of predicted/year and z-score/year and exacerbations in the last year and between the previous and subsequent year of the first and second atelectasis, with the exception of the subgroup analysis (Poster Figure 1). Conclusion: Atelectasis may cause significant worsening of lung function and exacerbations in certain subgroups of patients. 242 Pneumothorax in patients with cystic fibrosis E. Gudumac1, S. Sciuca1, L. Balanetchi2, R. Selevestru1. 1Mother and Child Institute, Chisinau, Republic of Moldova; 2Emergency Hospital for Children, Cluj-Napoca, Romania Aim: Evaluation of pleural-pulmonary complications in patients with cystic fibrosis (CF) with pulmonary damage. Methods: The study included 70 patients with CF, who were monitored at Cystic Fibrosis Center, during 18 years. CF diagnosis was confirmed by sweat test (“Macroduct”, USA), mutation of CFTR. Imaging pulmonary monitoring done by thoracic radiographs and lung CT, at stages of medical supervision. Results: In 8.5% cases were found pulmonary complications with pneumothorax develop in CF patients with chronic lung infections. CF patients with pleural-pulmonary complications age is 16.2 ± 2.5 years, from 10 to 24 years. Index of death for this group of CF patients is very high and reaches 66.7% and the average age of death is 15.5 ± 3.2 years, from 10 to 21 years. In 2 cases pneumothorax caused a total collapse of the lung, 4 patients achieved partial pneumothorax, with prevalence of right lung (66.7%). Molecular research has confirmed F508del mutation in the majority of patients (83.3%) – 3 cases F508del/F508del, 1 case F508del/ 2789+5G>A, 1 case F508del/X. All patients with pneumothorax was
Poster Sessions / Journal of Cystic Fibrosis 16S1 (2017) S63–S174
diagnosed with chronic pulmonary infection with Ps. aeruginosa, associated in one case with B. cepacia. Conclusion: Pneumothorax is a serious complication in chronic lung infections in patients with CF, has a high index of nefavorable evolution, and risk factors is CFTR genotype F508del and pulmonary infection with Ps. aeruginosa. 243 Chronic rhino-sinusitis and nasal polyposis among patients with cystic fibrosis E. Hatziagorou1, E. Kouroukli1, L. Nousia1, M. Galogavrou1, V. Zacharia2, H. Karasmanis2, A. Skouras2, J. Tsanakas1. 1School of Medicine University of Thessaloniki, Cystic Fibrosis Unit, Hippokration Hospital, Thessaloniki, Greece; 2 Hippokration Hospital, Dept of Otorhinolaryngology-Head and Neck Surgery, Thessaloniki, Greece Objective: To evaluate sinonasal disease and nasal polyposis among patients with CF. Method: CF patients attending a CF clinic underwent prospective evaluation of chronic rhinosinusitis. They completed a questionnaire on sinonasal symptoms, SNOT-2- (Sino- Nasal- Outcome-Test) and subsequently underwent nasoendoscopy. Abnormalities identified were quantified using endoscopic (Lund-Kennedy) scoring system. The relationship between symptoms of chronic rhino-sinusitis (CRS) and endoscopic findings was assessed. Results: One hundred and ten CF patients (n = 110, mean age 11 ± 5 years) were studied. Main symptoms were nasal congestion (68.75%), thick nasal discharge/post- nasal discharge (56.25%), facial pain/pressure (12.5%) and hyposmia/anosmia (12.5%). Fourty-seven CF patients (42.7%) complained for symptoms of CRS. Seventy-seven CF patients (70%) had evidence of CRS on naso-endoscopy including 14 patients (12.7%) with nasal polyps. Mean age of presentation of polyps was 12.2 (±6) years. There was a significant correlation between sinonasal symptoms (SNOT-2) and the Lund-Kennedy endoscopic score. All patients with nasal polyps received nasal steroids for a mean period of 5.5 months with good response. Eight (57.1%) received oral steroids for 12 days and 4 patients underwent polypectomy. One patient relapsed 1.5 years later. Conclusions: Nasal polyposis is a common clinical feature among patients with CF. ENT evaluation should be performed as part of annual review. Conservative management of chronic rhino-sinusitis is the most common practice. 244 Otorhinolaryngological manifestations in patients with cystic fibrosis. Review of cases in a third level hospital M. Domínguez Begines1, I. González Marquez1, M.E. Quintana-Gallego1, M.J. Moreno Varela1, L. Carrasco Hernández1, M.P. Gaboli1, I. Delgado-Pecellín1. 1Hospital Universitario Virgen del Rocío, Seville, Spain Introduction: Cystic fibrosis (CF) is a systemic disease whose most relevant manifestation affects the respiratory system. However, although otorhinolaryngological pathology (ENT) is quite frequent, it is not always considered in these patients. Objective: To study the prevalence and characteristics of the ENT pathology in patients with CF. Methods: Retrospective and descriptive study of CF patients (n = 225) in a CF Reference Unit. Variables analyzed: type of ENT pathology, age at diagnosis, sex, symptoms, imaging tests, treatment, relapses and associated mutations. Results: 34% of patients had ENT involvement. The most prevalent pathologies were nasosinusal polyposis (61%) and chronic rhinusinusitis (31%), 16% of patients presented other pathologies, such as chronic otitis media (4 patients), vocal cord nodules (4 patients), mucoceles (2 patients), branchial cyst (1 patient) and external auditory canal agenesis (1 patient). 23% of them presented polyposis and chronic rhinosinusitis simultaneously. The average age at diagnosis was 18.51 (±9) years old. There were no differences between genders. 82% reported pre-diagnosis symptoms (mainly nasal congestion, rhinorrhea and headache). Image tests (CT scan) were performed on 87% of affected patients. 100% received treatment with topical steroids and 55% required surgery (nasosinusal endoscopic
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surgery). 32% of operated patients presented recurrences. The most frequent associated mutation was F508del in homocygosis (24% of patients). Conclusion: We have verified that the ENT pathology is a frequent entity in CF patients. The most prevalent pathologies are nasosinusal polyposis and chronic rhinosinusitis. They usually appear during youth. In addition to physical examination, CT scan can be performed for diagnostic confirmation and preoperative study, as almost half of the patients require surgical treatment. The most frequent associated mutation is F508del/F508del. 245 The effect of genotype on left ventricular function in patients with cystic fibrosis M. Pallin1, D. Keating1, D.M. Kaye2, T. Kotsimbos1, J. Wilson1. 1Alfred Health, Cystic Fibrosis Service, Melbourne, Australia; 2Alfred Health, Cardiology Department, Melbourne, Australia Objectives: To investigate the importance of genotype on cardiac function in patients with cystic fibrosis. Methods: Retrospective review of cardiac indices measured by transthoracic echocardiogram (TTE) performed during standard CF care or lung transplant assessment. Comparisons were made between genotype groups determined by functional class of identified CFTR allele mutations. Results: Of 615 patients treated by the service between 2000 and 2015, 192 had a TTE performed of which 100 had CFTR genotype testing with at least 1 CF-causing mutation identified. Seventy-nine had 2 functional class 1–3 CFTR mutations (severe genotype) and 21 had only 1 functional class 1–3 mutation (mild genotype). Patients with a severe genotype were younger (31.1 ± 10.0 versus 44.0 ± 16.8 years; p < 0.0001), had a tendency toward lower left ventricular ejection fraction (60.5 ± 6.3 versus 63.5 ± 4.9%; p = 0.15) and had a significantly lower left ventricular fractional shortening (33.3 ± 6.6 versus 36.9 ± 6.3%; p < 0.05), left atrial area (14.9 ± 3.6 versus 18.0 ± 4.2 cm2; p < 0.01) and left atrial volume (39.9 ± 18.7 versus 51.0 ± 18.7 mL; p < 0.05). There were no significant differences observed in FEV1 (45.2 ± 22.6% versus 51.8 ± 25.1% predicted; p = 0.26) or right ventricular systolic pressure (32.9 ± 9.4 mmHg versus 35.8 ± 9.4 mmHg; p = 0.33) between patients with a severe and mild genotype. Univariate analysis of fractional shortening using Pearson correlation identified a linear association with tricuspid annular plane systolic excursion (r = 0.2726, p < 0.05) but not left atrial area, left atrial volume or FEV1. Conclusion: CF patients with a more severe genotype had a lower left ventricular fractional shortening than their counterparts with a mild genotype. This was not related to severity of lung disease or vascular volume contraction. 246 Musculoskeletal symptoms in a cystic fibrosis population R. Narang1, A. Higton1, C. Orchard1, M. Lloyd1. 1Frimley Health NHS Foundation Trust, Frimley, United Kingdom Objectives: Cystic fibrosis (CF) is the commonest life-limiting autosomal recessive disorder in Caucasians. Musculoskeletal pain is a common symptom but poorly understood and possibly under-recognised. Using our CF clinic cohort we aimed to assess the prevalence and distribution of musculoskeletal pain in these patients. Methods: A questionnaire covering patients’ experience of musculoskeletal symptoms was distributed to all 121 patients attending our unit. Results: 28 patients responded. The median age was 32.5 years (IQR 26.8– 36.0 years). 25 (89%) reported joint pain or swelling with 8 (29%) patients describing early morning stiffness lasting ≥30 minutes. 18 (64%) were female of whom 17 (94%) reported joint pain, compared to 80% of males. The median painful joint count in those aged ≥30 was similar to those aged <30 years (4.0 [1.0–6.3] vs 4.0 [1.8–6.3]). 14 (50%) patients reported predominantly medium-large joint involvement whilst 4 (14%) reported mostly small joint involvement. 14 (50%) reported back symptoms. 13 (46%) patients reported that their symptoms affected activities of daily living. All patients underwent anti-nuclear (ANA) profiling. 11 (39%) patients were ANA positive (≥1:160) and of these 10 (91%) patients reported joint pain or swelling. There did not appear to be a correlation between ANA positivity and impairment on activities of daily living.
Poster Sessions / Journal of Cystic Fibrosis 16S1 (2017) S63–S174
immediate success in 81%. Mean follow-up time was 22 and 35 months for the cases and controls, respectively. Of the 16 patients embolized: 3 had recurrences within 24 h (2 major, requiring new BAE), 11 had late recurrences (4 major requiring BAE and 1 surgery), and 2 died. Of the 5 non-embolized patients: 1 died, 1 had minimal recurrence, and 3 had serious recurrences (1 embolization and 1 lung transplant). Risk factors of massive hemoptysis were increased by previous mild repeated hemoptysis (HR 24.7, p = 0.002), and by the presence of ABPA (HR 3.4l, p = 0.04). The relative risk of hemoptysis was increased in patients with impaired FEV1 (FEV1 < 45%, HR 21, 45 < FEV1 < 70%, p = 0.03). During follow-up 5 cases (including 3 in the acute phase) and 1 control died from infectious respiratory failure. In the 4 years, the number of deaths and lung transplantations was higher in cases compared to controls (HR 7.1, p = 0.0004). Conclusion: Massive hemoptysis is associated with more altered respiratory function, leading to mortality or lung transplant within 4 years, and with the presence of ABPA. 240 Descriptive analysis of atelectasis as a pulmonary complication in cystic fibrosis M. Martínez-Redondo1, C. Prados Sánchez1, F. García-Río1, M.E. Quintana-Gallego2, S. Castillo3, A. Salcedo Posadas4, R.M. Girón5, M.T. Martínez Martínez6, L. Máiz7, C. Martín8, J. Costa9. 1La Paz University Hospital, Pulmonology, Madrid, Spain; 2Virgen del Rocío University Hospital, Pulmonology, Sevilla, Spain; 3Clinical Hospital of Valencia, Pulmonology, Valencia, Spain; 4Niño Jesús - Gregorio Marañón University Hospital, Pulmonology, Madrid, Spain; 5La Princesa University Hospital, Pulmonology, Madrid, Spain; 6Doce de Octubre University Hospital, Pulmonology, Madrid, Spain; 7Ramón y Cajal University Hospital, Pulmonology, Madrid, Spain; 8 Miguel Servet University Hospital, Pulmonology, Zaragoza, Spain; 9San Juan de Dios Hospital, Pulmonology, Barcelona, Spain Objective: to analyze atelectasis as a complication of cystic fibrosis (CF). Methods: a retrospective study has been performed on CF units of Spain. Cases of CF with atelectasis were included until Dec 2015 and controls of the same sex and d.o.b. ± 3 years. The study variables were demographic data, respiratory infections, pulmonary complications, comorbidities associated with CF, pulmonary function, diagnostic imaging, medical and bronchoscopic treatment and subsequent clinical and radiological improvement. Data were collected in different follow-up periods, following the ethical standards of each center. The SSPS version 13 was used. Comparison of variables: Student-t, Mann-Whitney and Chi-square tests according to their distribution. Relationship analysis: Chi-square test. Results: 38 patients had atelectasis (4–50 yrs), 23 were women (60.5%) and 15 were men (39.5%) with a mean age of 24.58 ± 11.9 yrs, compared to 39 controls (5–47 yrs), 26 were women (66.7%) and 13 were men (33.3%), with a mean age of 25.08 ± 1.84 yrs. Mutations: 20 homozygous F508del (26%), 37 heterozygous F508del (37%), 8 F508del/unknown (10.4%), 12 others (15.6%), p = 0.348. Location: 28 first lobar atelectasis (13 LUL) and 4 2nd lobar atelectasis (2 RUL, 2 LUL, 2 RML and 1 RLL). Pulmonary complications: ABPA was only statistically significant ( p = 0.006), presenting in 10 patients [9 cases (23.7%)]. Chronic colonization in last registry: there are no statistically significant differences between cases and controls. At the time of atelectasis (30 cases): P. aeruginosa 55.3%, MSSA 34.2%, A xylosoxidans 7.9%, NTM 7.9%, MRSA 5.3%, S. maltophila 5.3%, TB 0%, others 31.6%. There were no significant differences between the treatments before and after atelectasis: reinforcement of therapies to reduce sputum viscosity and inflammation. Respiratory function tests are detailed in Poster Figure 2. Conclusions: • Atelectasis cause a decline in lung function. • Prior ABPA could favor later atelectasis.
241 Atelectasis as a poor prognosis factor in cystic fibrosis M. Martínez-Redondo1, C. Prados Sánchez1, F. García-Río1, M.E. Quintana-Gallego2, S. Castillo3, A. Salcedo Posadas4, R.M. Girón5, M.T. Martínez Martínez6, L. Máiz Carro7, C. Martín8, J. Costa9. 1La Paz University Hospital, Pulmonology, Madrid, Spain; 2Virgen del Rocío University Hospital, Pulmonology, Sevilla, Spain; 3Clinical Hospital of Valencia, Pulmonology, Valencia, Spain; 4Niño Jesús - Gregorio Marañón University Hospital, Pulmonology, Madrid, Spain; 5La Princesa University Hospital, Pulmonology, Madrid, Spain; 6Doce de Octubre University Hospital, Pulmonology, Madrid, Spain; 7Ramón y Cajal University Hospital, Pulmonology, Madrid, Spain; 8Miguel Servet University Hospital, Pulmonology, Zaragoza, Spain; 9San Juan de Dios Hospital, Pulmonology, Barcelona, Spain Objectives: The aim of this study was to analyze pulmonary atelectasis in cystic fibrosis (CF) as a poor prognostic factor. Methods: A retrospective study has been performed on cystic fibrosis units of Spain. Cases of cystic fibrosis with atelectasis have been included until December 2015 and controls of the same sex and date of birth ± 3 years. The study variables were demographic data, respiratory infections, pulmonary complications, comorbidities associated with cystic fibrosis, pulmonary function, diagnostic imaging of atelectasis, medical and bronchoscopic treatment and subsequent clinical and radiological improvement. Data were collected in different follow-up periods, following the ethical standards of each center. The SSPS version 13 program was used. For the comparison of variables, Student-t, Mann-Whitney and Chi-square tests were used according to their distribution. For the relationship analysis, the Chi-square test was used. Results: in our series, 38 patients had atelectasis (between 4 and 50 years), which 23 were women (60.5%), with a mean age of 24.58 ± 11.9 years, compared to 39 controls (between 5 and 47 years), which 26 were female (66.7%) with a mean age of 25.08 ± 11.84 years. 4 cases died (10.5%) at the ages of 26, 30, 31 and 33 years and 1 control (2.6%) at 5 years ( p = 0.171). 3 cases with unresolved atelectasis after the treatment were transplanted with a FEV1 of 18%, 21% and 31% of predicted. Regarding the respiratory function tests, there was no significance in the overall annual decline compared to the annual decline after the first and second atelectasis in ml/year, % of predicted/year and z-score/year and exacerbations in the last year and between the previous and subsequent year of the first and second atelectasis, with the exception of the subgroup analysis (Poster Figure 1). Conclusion: Atelectasis may cause significant worsening of lung function and exacerbations in certain subgroups of patients. 242 Pneumothorax in patients with cystic fibrosis E. Gudumac1, S. Sciuca1, L. Balanetchi2, R. Selevestru1. 1Mother and Child Institute, Chisinau, Republic of Moldova; 2Emergency Hospital for Children, Cluj-Napoca, Romania Aim: Evaluation of pleural-pulmonary complications in patients with cystic fibrosis (CF) with pulmonary damage. Methods: The study included 70 patients with CF, who were monitored at Cystic Fibrosis Center, during 18 years. CF diagnosis was confirmed by sweat test (“Macroduct”, USA), mutation of CFTR. Imaging pulmonary monitoring done by thoracic radiographs and lung CT, at stages of medical supervision. Results: In 8.5% cases were found pulmonary complications with pneumothorax develop in CF patients with chronic lung infections. CF patients with pleural-pulmonary complications age is 16.2 ± 2.5 years, from 10 to 24 years. Index of death for this group of CF patients is very high and reaches 66.7% and the average age of death is 15.5 ± 3.2 years, from 10 to 21 years. In 2 cases pneumothorax caused a total collapse of the lung, 4 patients achieved partial pneumothorax, with prevalence of right lung (66.7%). Molecular research has confirmed F508del mutation in the majority of patients (83.3%) – 3 cases F508del/F508del, 1 case F508del/ 2789+5G>A, 1 case F508del/X. All patients with pneumothorax was
Poster Sessions / Journal of Cystic Fibrosis 16S1 (2017) S63–S174
diagnosed with chronic pulmonary infection with Ps. aeruginosa, associated in one case with B. cepacia. Conclusion: Pneumothorax is a serious complication in chronic lung infections in patients with CF, has a high index of nefavorable evolution, and risk factors is CFTR genotype F508del and pulmonary infection with Ps. aeruginosa. 243 Chronic rhino-sinusitis and nasal polyposis among patients with cystic fibrosis E. Hatziagorou1, E. Kouroukli1, L. Nousia1, M. Galogavrou1, V. Zacharia2, H. Karasmanis2, A. Skouras2, J. Tsanakas1. 1School of Medicine University of Thessaloniki, Cystic Fibrosis Unit, Hippokration Hospital, Thessaloniki, Greece; 2 Hippokration Hospital, Dept of Otorhinolaryngology-Head and Neck Surgery, Thessaloniki, Greece Objective: To evaluate sinonasal disease and nasal polyposis among patients with CF. Method: CF patients attending a CF clinic underwent prospective evaluation of chronic rhinosinusitis. They completed a questionnaire on sinonasal symptoms, SNOT-2- (Sino- Nasal- Outcome-Test) and subsequently underwent nasoendoscopy. Abnormalities identified were quantified using endoscopic (Lund-Kennedy) scoring system. The relationship between symptoms of chronic rhino-sinusitis (CRS) and endoscopic findings was assessed. Results: One hundred and ten CF patients (n = 110, mean age 11 ± 5 years) were studied. Main symptoms were nasal congestion (68.75%), thick nasal discharge/post- nasal discharge (56.25%), facial pain/pressure (12.5%) and hyposmia/anosmia (12.5%). Fourty-seven CF patients (42.7%) complained for symptoms of CRS. Seventy-seven CF patients (70%) had evidence of CRS on naso-endoscopy including 14 patients (12.7%) with nasal polyps. Mean age of presentation of polyps was 12.2 (±6) years. There was a significant correlation between sinonasal symptoms (SNOT-2) and the Lund-Kennedy endoscopic score. All patients with nasal polyps received nasal steroids for a mean period of 5.5 months with good response. Eight (57.1%) received oral steroids for 12 days and 4 patients underwent polypectomy. One patient relapsed 1.5 years later. Conclusions: Nasal polyposis is a common clinical feature among patients with CF. ENT evaluation should be performed as part of annual review. Conservative management of chronic rhino-sinusitis is the most common practice. 244 Otorhinolaryngological manifestations in patients with cystic fibrosis. Review of cases in a third level hospital M. Domínguez Begines1, I. González Marquez1, M.E. Quintana-Gallego1, M.J. Moreno Varela1, L. Carrasco Hernández1, M.P. Gaboli1, I. Delgado-Pecellín1. 1Hospital Universitario Virgen del Rocío, Seville, Spain Introduction: Cystic fibrosis (CF) is a systemic disease whose most relevant manifestation affects the respiratory system. However, although otorhinolaryngological pathology (ENT) is quite frequent, it is not always considered in these patients. Objective: To study the prevalence and characteristics of the ENT pathology in patients with CF. Methods: Retrospective and descriptive study of CF patients (n = 225) in a CF Reference Unit. Variables analyzed: type of ENT pathology, age at diagnosis, sex, symptoms, imaging tests, treatment, relapses and associated mutations. Results: 34% of patients had ENT involvement. The most prevalent pathologies were nasosinusal polyposis (61%) and chronic rhinusinusitis (31%), 16% of patients presented other pathologies, such as chronic otitis media (4 patients), vocal cord nodules (4 patients), mucoceles (2 patients), branchial cyst (1 patient) and external auditory canal agenesis (1 patient). 23% of them presented polyposis and chronic rhinosinusitis simultaneously. The average age at diagnosis was 18.51 (±9) years old. There were no differences between genders. 82% reported pre-diagnosis symptoms (mainly nasal congestion, rhinorrhea and headache). Image tests (CT scan) were performed on 87% of affected patients. 100% received treatment with topical steroids and 55% required surgery (nasosinusal endoscopic
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surgery). 32% of operated patients presented recurrences. The most frequent associated mutation was F508del in homocygosis (24% of patients). Conclusion: We have verified that the ENT pathology is a frequent entity in CF patients. The most prevalent pathologies are nasosinusal polyposis and chronic rhinosinusitis. They usually appear during youth. In addition to physical examination, CT scan can be performed for diagnostic confirmation and preoperative study, as almost half of the patients require surgical treatment. The most frequent associated mutation is F508del/F508del. 245 The effect of genotype on left ventricular function in patients with cystic fibrosis M. Pallin1, D. Keating1, D.M. Kaye2, T. Kotsimbos1, J. Wilson1. 1Alfred Health, Cystic Fibrosis Service, Melbourne, Australia; 2Alfred Health, Cardiology Department, Melbourne, Australia Objectives: To investigate the importance of genotype on cardiac function in patients with cystic fibrosis. Methods: Retrospective review of cardiac indices measured by transthoracic echocardiogram (TTE) performed during standard CF care or lung transplant assessment. Comparisons were made between genotype groups determined by functional class of identified CFTR allele mutations. Results: Of 615 patients treated by the service between 2000 and 2015, 192 had a TTE performed of which 100 had CFTR genotype testing with at least 1 CF-causing mutation identified. Seventy-nine had 2 functional class 1–3 CFTR mutations (severe genotype) and 21 had only 1 functional class 1–3 mutation (mild genotype). Patients with a severe genotype were younger (31.1 ± 10.0 versus 44.0 ± 16.8 years; p < 0.0001), had a tendency toward lower left ventricular ejection fraction (60.5 ± 6.3 versus 63.5 ± 4.9%; p = 0.15) and had a significantly lower left ventricular fractional shortening (33.3 ± 6.6 versus 36.9 ± 6.3%; p < 0.05), left atrial area (14.9 ± 3.6 versus 18.0 ± 4.2 cm2; p < 0.01) and left atrial volume (39.9 ± 18.7 versus 51.0 ± 18.7 mL; p < 0.05). There were no significant differences observed in FEV1 (45.2 ± 22.6% versus 51.8 ± 25.1% predicted; p = 0.26) or right ventricular systolic pressure (32.9 ± 9.4 mmHg versus 35.8 ± 9.4 mmHg; p = 0.33) between patients with a severe and mild genotype. Univariate analysis of fractional shortening using Pearson correlation identified a linear association with tricuspid annular plane systolic excursion (r = 0.2726, p < 0.05) but not left atrial area, left atrial volume or FEV1. Conclusion: CF patients with a more severe genotype had a lower left ventricular fractional shortening than their counterparts with a mild genotype. This was not related to severity of lung disease or vascular volume contraction. 246 Musculoskeletal symptoms in a cystic fibrosis population R. Narang1, A. Higton1, C. Orchard1, M. Lloyd1. 1Frimley Health NHS Foundation Trust, Frimley, United Kingdom Objectives: Cystic fibrosis (CF) is the commonest life-limiting autosomal recessive disorder in Caucasians. Musculoskeletal pain is a common symptom but poorly understood and possibly under-recognised. Using our CF clinic cohort we aimed to assess the prevalence and distribution of musculoskeletal pain in these patients. Methods: A questionnaire covering patients’ experience of musculoskeletal symptoms was distributed to all 121 patients attending our unit. Results: 28 patients responded. The median age was 32.5 years (IQR 26.8– 36.0 years). 25 (89%) reported joint pain or swelling with 8 (29%) patients describing early morning stiffness lasting ≥30 minutes. 18 (64%) were female of whom 17 (94%) reported joint pain, compared to 80% of males. The median painful joint count in those aged ≥30 was similar to those aged <30 years (4.0 [1.0–6.3] vs 4.0 [1.8–6.3]). 14 (50%) patients reported predominantly medium-large joint involvement whilst 4 (14%) reported mostly small joint involvement. 14 (50%) reported back symptoms. 13 (46%) patients reported that their symptoms affected activities of daily living. All patients underwent anti-nuclear (ANA) profiling. 11 (39%) patients were ANA positive (≥1:160) and of these 10 (91%) patients reported joint pain or swelling. There did not appear to be a correlation between ANA positivity and impairment on activities of daily living.