BACKGROUND: The objective of the study is to determine the efficacy, safety and feasibility of home treatment with low-molecular weight heparin (LMWH) in patients with symptomatic pulmonary embolism (PE). METHODS: All patients with PE on the home treatment programme between January 1998 and December 1999 were identified. Age, sex, length of hospital stay, risk factors for PE, ventilation perfusion (V/Q) lung scanning results, baseline investigations and treatment regimen were recorded. PE was defined as a clinical diagnosis supported by a high, intermediate or low probability V/Q lung scan. The efficacy of the treatment is defined as the event rate of recurrent PE. The safety outcome is defined as the rate of haemorrhage and the mortality that was related to the treatment during the 6-month follow-up period. RESULTS: Seventy patients were identified. Of these, 68 were hospitalised initially with a mean+/-S.D. length of hospital stay of 4.0+/-3.3 (range 0-17) days; their mean+/-S.D. age was 54.6+/-20.5 (range 19-87) years. Cardiopulmonary disease was the commonest pre-existing condition and 86% patients had at least one risk factor. All except one was haemodynamically stable. During the 6 months after discharge, 20 patients (28.6%) were readmitted at least once. One patient (1.4%) had symptomatic recurrent PE, one (1.4%) had haemoptysis and the remainder had unrelated pathology. There were no episodes of serious haemorrhage, and the two deaths that occurred in this period were not due to further PE. CONCLUSION: Early discharge home for stable patients with PE treated with LMWH (tinzaparin) is feasible, effective and safe, provided that patients are clinically stable, accessible by phone, receive daily administration of LMWH and have their international normalised ratio (INR) monitored.

Exhaled nitric oxide (NO) is thought to be a marker of asthmatic inflammation. Levels in cystic fibrosis (CF) are generally low. This study aimed to measure exhaled NO in CF patients at high risk of developing ABPA and patients at low risk. We studied nine patients at high risk of developing ABPA and 36 at low risk. The two groups were similar in age and spirometry. All patients in the high-risk group were taking oral or inhaled glucocorticoids, compared to 56% in the low-risk group (P=0.02). The exhaled NO levels were lower in the high-risk group than in the low-risk group (2.0 vs. 3.6 ppb), mean difference (95% CI) 1.6 (-3.6 to 0.4) ppb, P=0.001. On subgroup analysis of patients on oral glucocorticoids, the exhaled NO levels were significantly lower in patients with a high risk of developing ABPA (n=7) than patients with a low risk (n=8) (P=0.011). The number of patients who were on inhaled, but not oral glucocorticoids was too small to analyse usefully. Exhaled NO levels were lower in CF patients with a high risk of developing ABPA and on glucocorticoids. This may be because oral glucocorticoids exert a greater effect on exhaled NO than inhaled glucocorticoids. Alternatively, inducible nitric oxide synthase may be down-regulated by Aspergillus toxin.

BACKGROUND:Common variable immune deficiency (CVID) is prone to under-diagnosis and may not reach relevant specialists until late in life. Morbidity is most commonly due to acute-on-chronic respiratory infections leading to respiratory failure.AIM:To investigate respiratory complications, lung function and high-resolution computerized tomography scan (HRCT) findings and mortality in 47 patients with CVID.SETTING:A regional immunology unit (Birmingham Heartlands Hospital).DESIGN:Retrospective observational case-note study following the introduction of shared care between immunology and respiratory medicine.RESULTS:Age at diagnosis ranged from 5 to 72 years, with a median time from development of first symptoms to diagnosis of 4.0 years. There was delay in referral between chest physicians and immunologists, (median referral time between specialities >5 years). Forty-two patients had respiratory complications, due to bronchiectasis (n=32), asthma (n=7), recurrent chest infections (n=9) without concomitant evidence of structural lung damage, and granulomatous lung disease (n=2). Spirometry was abnormal in 10/39 patients (7 obstructive, 3 restrictive). Bronchiectasis was confirmed on chest radiograph (n=9) and HRCT (n=24). Despite the high prevalence of bronchiectasis, few patients had received instruction in physiotherapy and sputum culture results were sparse.DISCUSSION:To reduce the morbidity associated with CVID, there needs to be greater awareness of respiratory complications, particularly amongst physicians caring for such patients. Emphasis has been placed on adequate dosage of immunoglobulin, but early involvement by a respiratory physician is essential to monitor lung function and initiate optimal therapy, to minimize the occurrence and progression of lung damage.

Background - As women with cystic fibrosis are living longer, pregnancy is becoming increasingly common. The combined experience of pregnancies in women with cystic fibrosis from adult centres in the Midlands and North of England has been examined.Methods - A retrospective study of the case notes of 22 pregnancies in 20 patients with cystic fibrosis examined changes in lung function, body weight, and microbiological status during the course of pregnancy. Duration of pregnancy, birth weight, and maternal survival were amongst other variables studied. The relation between values before pregnancy and important outcome measures were examined.Results - Eighteen of 22 pregnancies were completed producing healthy, non-cystic fibrosis infants (12 female). Mothers lost 13% of FEV(1) and 11% of FVC during pregnancy, most of which was regained. Body weight changes were variable, but most mothers gained weight (mean weight gain 5.7 kg). Microbiological status remained unchanged. Six infants were preterm and two were light for dates. Four mothers died up to 3.2 years following delivery. Of the prepregnancy parameters examined, %FEV(1) showed the best correlation with maternal weight gain, gestation, birth weight, and maternal survival.Conclusions - Pregnancy was well tolerated by most mothers with cystic fibrosis although those with moderate to severe lung disease (%FEV(1) <60%) before pregnancy fared worse, producing preterm infants and suffering increased loss of lung function and mortality compared with mildly affected mothers. Prepregnancy %FEV(1) appears to be the most useful predictor of important outcome measures in pregnancies in women with cystic fibrosis.

Cystic fibrosis (CF) is the most common lethal autosomal recessive inherited disease in Caucasian European populations, affecting 1:2,500 children with a 1:25 carrier frequency. Nearly one third of the ∼7,500 patients in the United Kingdom are over 16 years of age, and as survival increases, the proportion is expected to further increase with fertility issues becoming increasingly important.Although the major manifestations of CF are in the lungs and gastrointestinal tract, up to 98% of men with CF are infertile because of obstruction or congenital bilateral absence of the vas deferens (CBAVD) resulting in azoospermia; this was first reported >30 years ago (1Kaplan E. Shwachman H. Perlmutter A.D. Rule A. Khaw K.T. Holsclaw D.S. Reproductive failure in males with cystic fibrosis.N Engl J Med. 1968; 279: 65-69Crossref PubMed Scopus (249) Google Scholar). Congenital bilateral absence of the vas deferens itself is a major cause of infertility in men, many of whom do not have true CF, but many of whom at least have one CF mutation. Over 750 gene mutations have been reported, with 70% of mutations being due to ΔF508, a three–base pair deletion in exon 10 of the CF gene, located at 7q31.The resulting defect in the CF transmembrane regulator protein, a complex chloride channel, causes abnormal control of ion and water transport across epithelial cells, resulting in tenacious secretions that obstruct glandular tissues in many organs. Histological studies of testicular tissues of males with CF and CBAVD show that spermatogenesis may be normal, and cases of normal fertility in CF have been described (2Barreto C. Pinto L.M. Duarte A. Lavinha J. Ramsay M. A fertile male with cystic fibrosis molecular genetic analysis.J Med Genet. 1991; 28: 420-421Crossref PubMed Scopus (21) Google Scholar). However, sperm numbers are often decreased with abnormal forms.Although much is known about the genetic basis of male infertility in cystic fibrosis, there is little information on patient knowledge and perception of reproductive issues. Osman et al. (3Osman L.M. Griffiths K. Fair A. Attitudes to fertility issues among adults with cystic fibrosis in Scotland.Thorax. 2000; 55: 672-678Crossref PubMed Scopus (74) Google Scholar) recently gave a questionnaire to 82 men and 54 women with CF and found that all but two men were aware that they were infertile and that men were unlikely to initiate discussion about infertility. We report our own findings here.One hundred CF males over 16 years of age, attending the Birmingham Adult Cystic Fibrosis Unit, were asked to complete a questionnaire designed by a panel including a CF specialist, a CF specialist nurse, a gynecologist, and a midwife trained in family planning and approved by the hospital ethics committee. Twenty-three questions inquired into partner status, use of contraception, knowledge of infertility, and opinions on fertility tests and treatment.Seventy-two patients (72%) completed the questionnaire in private at the clinic (n = 49) or at home (n = 23). Two patients refused to participate, and 26 did not reply. The mean age of the respondents was 24.6 years (range, 16 to 43 years). Forty of 72 (56%) sexually experienced patients had never received any advice on infertility or contraception. Of the men who had received advice on fertility, 24% had received information from their adult CF physician, and the remainder had recent information from parents, another family member, their adult CF nurse, general practitioner, fertility clinic, child CF doctor, or books. The majority (71%) felt that their adult CF physician or adult CF nurse (24%) was the most appropriate person to discuss fertility.Forty-one patients (57%) knew that it was rare for a CF man to father a child, and another 18 (25%) felt that it was much less common for a CF man to father a child compared with what was normal. Only five patients (7%) felt that their fertility would be normal. Seventeen patients (24%) had previously had a sperm count. Of these, 15 had no sperm, one did not know the result, and one said that sperm was present. A further 27 patients (38%) expressed a wish to have a sperm count.Forty-nine men (68%) wished to father a child either now or in the future. Nine patients (11%) had already tried to father a child. One patient had two children by natural means, and eight had tried to conceive without success for between 1 and 6 years. The man who had fathered two children by natural means knew that it is very rare for a CF man to father a child. He had not had a sperm count but had discussed assisted conception with his adult CF physician, and his partner had undergone genetic screening tests for CF carrier status. Two couples had attempted fertility treatment without success. Thirty-one patients (43%) said that they would consider it in the future.There recently have been advances in the treatment of male infertility in CF and CBAVD. Microepididymal sperm aspiration, followed by intracytoplasmic sperm injection and IVF, offers success rates of approximately 35% per cycle (4Rosenlund B. Sjoblom P. Dimitrakopoulos A. Hillensjo T. Epididymal and testicular sperm injection in the treatment of obstructive azoospermia.Acta Obstet Gynecol Scand. 1997; 75: 135-139Crossref Scopus (26) Google Scholar). It is important to consider the genetic implications of such treatment in men with CF (and those with CBAVD with a CF mutation). All children of CF fathers will be CF carriers. Screening of their partners for the 32 common CF gene mutations will detect approximately 90% of mutations in Caucasians, 97% of mutations in Ashkenazi Jews, and 45% in the African American population. In Caucasians, the risk of an affected child is reduced to less than 1:250 when the partner is negative for these common mutations. In addition, the couple would need to discuss how the shortened life span of one parent would affect their decision to have a child; this should be discussed in the context of the increasing success of lung transplantation.In conclusion, this survey shows that male CF patients are not well informed about infertility. Most patients wish to have a sperm count to confirm their infertility. A high proportion wish to consider assisted conception. Genetic counseling, screening of the partner for CF carrier status, and consideration of the implications of raising a child when one partner has a chronic disease are recommended. Cystic fibrosis (CF) is the most common lethal autosomal recessive inherited disease in Caucasian European populations, affecting 1:2,500 children with a 1:25 carrier frequency. Nearly one third of the ∼7,500 patients in the United Kingdom are over 16 years of age, and as survival increases, the proportion is expected to further increase with fertility issues becoming increasingly important. Although the major manifestations of CF are in the lungs and gastrointestinal tract, up to 98% of men with CF are infertile because of obstruction or congenital bilateral absence of the vas deferens (CBAVD) resulting in azoospermia; this was first reported >30 years ago (1Kaplan E. Shwachman H. Perlmutter A.D. Rule A. Khaw K.T. Holsclaw D.S. Reproductive failure in males with cystic fibrosis.N Engl J Med. 1968; 279: 65-69Crossref PubMed Scopus (249) Google Scholar). Congenital bilateral absence of the vas deferens itself is a major cause of infertility in men, many of whom do not have true CF, but many of whom at least have one CF mutation. Over 750 gene mutations have been reported, with 70% of mutations being due to ΔF508, a three–base pair deletion in exon 10 of the CF gene, located at 7q31. The resulting defect in the CF transmembrane regulator protein, a complex chloride channel, causes abnormal control of ion and water transport across epithelial cells, resulting in tenacious secretions that obstruct glandular tissues in many organs. Histological studies of testicular tissues of males with CF and CBAVD show that spermatogenesis may be normal, and cases of normal fertility in CF have been described (2Barreto C. Pinto L.M. Duarte A. Lavinha J. Ramsay M. A fertile male with cystic fibrosis molecular genetic analysis.J Med Genet. 1991; 28: 420-421Crossref PubMed Scopus (21) Google Scholar). However, sperm numbers are often decreased with abnormal forms. Although much is known about the genetic basis of male infertility in cystic fibrosis, there is little information on patient knowledge and perception of reproductive issues. Osman et al. (3Osman L.M. Griffiths K. Fair A. Attitudes to fertility issues among adults with cystic fibrosis in Scotland.Thorax. 2000; 55: 672-678Crossref PubMed Scopus (74) Google Scholar) recently gave a questionnaire to 82 men and 54 women with CF and found that all but two men were aware that they were infertile and that men were unlikely to initiate discussion about infertility. We report our own findings here. One hundred CF males over 16 years of age, attending the Birmingham Adult Cystic Fibrosis Unit, were asked to complete a questionnaire designed by a panel including a CF specialist, a CF specialist nurse, a gynecologist, and a midwife trained in family planning and approved by the hospital ethics committee. Twenty-three questions inquired into partner status, use of contraception, knowledge of infertility, and opinions on fertility tests and treatment. Seventy-two patients (72%) completed the questionnaire in private at the clinic (n = 49) or at home (n = 23). Two patients refused to participate, and 26 did not reply. The mean age of the respondents was 24.6 years (range, 16 to 43 years). Forty of 72 (56%) sexually experienced patients had never received any advice on infertility or contraception. Of the men who had received advice on fertility, 24% had received information from their adult CF physician, and the remainder had recent information from parents, another family member, their adult CF nurse, general practitioner, fertility clinic, child CF doctor, or books. The majority (71%) felt that their adult CF physician or adult CF nurse (24%) was the most appropriate person to discuss fertility. Forty-one patients (57%) knew that it was rare for a CF man to father a child, and another 18 (25%) felt that it was much less common for a CF man to father a child compared with what was normal. Only five patients (7%) felt that their fertility would be normal. Seventeen patients (24%) had previously had a sperm count. Of these, 15 had no sperm, one did not know the result, and one said that sperm was present. A further 27 patients (38%) expressed a wish to have a sperm count. Forty-nine men (68%) wished to father a child either now or in the future. Nine patients (11%) had already tried to father a child. One patient had two children by natural means, and eight had tried to conceive without success for between 1 and 6 years. The man who had fathered two children by natural means knew that it is very rare for a CF man to father a child. He had not had a sperm count but had discussed assisted conception with his adult CF physician, and his partner had undergone genetic screening tests for CF carrier status. Two couples had attempted fertility treatment without success. Thirty-one patients (43%) said that they would consider it in the future. There recently have been advances in the treatment of male infertility in CF and CBAVD. Microepididymal sperm aspiration, followed by intracytoplasmic sperm injection and IVF, offers success rates of approximately 35% per cycle (4Rosenlund B. Sjoblom P. Dimitrakopoulos A. Hillensjo T. Epididymal and testicular sperm injection in the treatment of obstructive azoospermia.Acta Obstet Gynecol Scand. 1997; 75: 135-139Crossref Scopus (26) Google Scholar). It is important to consider the genetic implications of such treatment in men with CF (and those with CBAVD with a CF mutation). All children of CF fathers will be CF carriers. Screening of their partners for the 32 common CF gene mutations will detect approximately 90% of mutations in Caucasians, 97% of mutations in Ashkenazi Jews, and 45% in the African American population. In Caucasians, the risk of an affected child is reduced to less than 1:250 when the partner is negative for these common mutations. In addition, the couple would need to discuss how the shortened life span of one parent would affect their decision to have a child; this should be discussed in the context of the increasing success of lung transplantation. In conclusion, this survey shows that male CF patients are not well informed about infertility. Most patients wish to have a sperm count to confirm their infertility. A high proportion wish to consider assisted conception. Genetic counseling, screening of the partner for CF carrier status, and consideration of the implications of raising a child when one partner has a chronic disease are recommended.

Hemoptyses are common in cystic fibrosis (CF) patients. They range from massive life-threatening (> 240 mL/24 hours) to recurrent minor streaking. Limited pulmonary reserve, potential concurrent chest infection, and the progressive nature of CF pose a high risk to this subgroup. Conservative management and selective bronchial artery embolization (BAE) control most acute episodes, but the recurrence rate is high. The possible need for lung transplantation in future makes an extrapleural approach for bronchial artery ligation desirable. The aim of this study was to assess the role of extrapleural bronchial artery ligation in the treatment of recurrent hemoptysis in CF patients. This is a retrospective analysis of four patients between 1986 and 1999 treated by extrapleural thoracotomy and ligation of bronchial arteries. Indications, surgical experience, and outcome are presented. Three patients underwent unilateral, and one patient bilateral extrapleural thoracotomy (in two separate sessions) for bronchial artery ligation. There were three men and one woman, with a mean age of 26.6 years (range 19-32 years). Indications were failure to stabilize the bronchial arterial catheter for BAE (three cases), recurrence after BAE previously controlled bleeding (one case), and communication with the right costocervical trunk signifying risk to the spinal circulation (one case). The mean follow-up was 68 months (range 3-144 months). There was one death in this series, a patient who was asphyxiated with hemoptysis, requiring ventilation preoperatively. He underwent successful extrapleural thoracotomy for bronchial artery ligation, with no further bleeding but succumbed to severe chest infection and multiorgan failure a few days later. Two patients had recurrent bleeding 12 and 36 months after surgery. Selective bronchial angiography proved the contralateral bronchial arteries to be the culprit. Extrapleural bronchial artery ligation is an effective method of controlling hemoptysis in CF, when BAE has failed. This approach minimizes pleural adhesions and is, therefore, desirable in the future consideration for lung transplantation. In this experience, muscle-sparing thoracotomy and postoperative epidural analgesia significantly improved the postoperative recovery.

Twenty-six adult cystic fibrosis patients were studied to compare nasal disease with their laboratory correlates including skin testing, immunoglobulin and Aspergillus fumigatus precipitin levels, saccharin testing and sputum cultures. Six patients were asymptomatic and all of these had negative skin tests, normal IgE levels and negative Aspergillus fumigatus precipitins. Thirteen patients had rhinitis, 12 had positive skin-testing for common allergens, 10 elevated IgE levels and nine positive Aspergillus fumigatus precipitins. Seven patients had polyps, all had normal IgE levels and negative Aspergillus fumigatus precipitins, six had positive skin testing for common allergens. There also appeared to be a relationship between Pseudomonas spp. colonization and positive skin testing.

Objective To identify pregnancies in women with cystic fibrosis and describe obstetric, infant and maternal medical outcomes in relation to the severity of maternal disease.Design Retrospective study, based on casenotes.Setting Eleven cystic fibrosis centres in the United Kingdom.Population Pregnant women with cystic fibrosis.Methods Single observer medical and obstetric casenote review categorising maternal cystic fibrosis (e.g. genotype, pancreatic, hepatic and diabetic status) and pre-pregnant severity (e.g, weight and lung function) and noting fetal outcome and maternal morbidity.Main outcome measures Completed pregnancies and pregnancy losses, fetal outcome: and complications, maternal morbidity, such as changes in weight, lung function, pulmonary infections during and after pregnancy. Relation of outcomes to severity of maternal cystic fibrosis.Results From 72 pregnancies identified, the outcomes were known for 69; there were 48 live births (70%) of which 22 were premature (46%); 14 therapeutic abortions (20%); and 7 miscarriages (10%). There were no stillbirths, neonatal or early maternal deaths. Three major fetal anomalies were seen, but no infant had cystic fibrosis. At the conclusion of our study three pregnancies were still continuing. Prematurity with increased fetal complications and maternal morbidity with infection, declining lung function and poor weight: gain were associated with poor pre-partum lung function.Conclusion Pregnancy occurs in women with cystic fibrosis of all degrees of severity. Outcomes for the infant are generally good but are variable for the mother. Predicting outcome on the basis of maternal severity is difficult but lung function appears to be the most significant determining factor. Pregnancy may be normal in women with normal lung function (forced expiratory volume > 80%). However, it may adversely affect mild and moderate lung disease due to cystic fibrosis and should be avoided in pulmonary hypertension, cor pulmonale and when forced expiratory volume < 50% predicted. Ideally, all pregnancies should be planned with prior counselling and monitored by dedicated cystic fibrosis teams, including obstetricians who are experienced in managing high risk pregnancies.

Excellent paediatric care has ensured that most children important group and exposing our patients to the vagaries of local initiatives and gross inequality of service between with cystic fibrosis survive into adulthood. 1 2 Centre based care from a multidisciplinary cystic fibrosis team has been regions. The current guidelines from the Royal College of Phys-shown to deliver a better prognosis 3 and continuing optimal supervision delivered from multidisciplinary adult cystic icians recommend 3–4 consultant sessions and 2–3 sessions from another senior doctor per week per 50 patients. 7 In fibrosis centres has seen patient survival reach beyond the fourth decade of life. Antenatal screening, selective or the adult cystic fibrosis units at Birmingham, Leeds, and Manchester one consultant currently has overall re-universal, will eventually reduce the proportion of paediatric patients further. 4 Cystic fibrosis is becoming pre-sponsibility for over 200 patients. Historically the care for adult patients with cystic fibrosis has been assumed largely dominantly an adult disease. The adult cystic fibrosis clinics in Birmingham, Leeds, and Manchester currently look by interested physicians in addition to their existing clinical workload, and some continue busy respiratory and general after over 600 patients whereas five years ago this combined figure was approximately 400. Each of the three clinics has medical practices. As total patient numbers have increased, individuals inevitably receive less personal attention, dean average annual net increase of 20 patients. Medical management has increased survival, not only spite the need for constant input from consultants and other experienced senior doctors, thus undermining the because care delivered by cystic fibrosis centres has improved with experience but also because there are more confidence of patients in the quality of care delivered. The accepted level of junior doctor support for every 50 treatment options to maintain patients in a good and stable clinical state. These include intensive use of oral and patients is five sessions of one registrar grade with a special interest in cystic fibrosis, in addition to the usual junior intravenous antibiotics, maximised nutrition with naso-gastric or gastrostomy feeding, and the prescription of staffing of a medical team. The recommended professional support is: one clinical nurse specialist, two physio-novel drugs such as DNase which can potentially increase survival further. Consequently many people with cystic therapists, one secretary, one part-time dietitian, and one part-time social worker. Based on these criteria, the levels fibrosis have a better quality of life, often with jobs, families, …

In patients with cystic fibrosis (CF), nasal intermittent positive pressure ventilation (NIPPV) is currently used as a short-term bridge to transplantation but its precise role has yet to be determined. Patients were offered a therapeutic trial of NIPPV when candidates for lung transplantation, with respiratory failure unresponsive to medical treatment. Twelve patients, six male of mean age of 26 +/- 1.4 years, had a trial of NIPPV. At recruitment the mean percentage predicted forced expired volume in one second (FEV1) was 15.1% +/- 1.2%, arterial carbon dioxide (PaCO2) 8.7 +/- 0.6 kPa, arterial oxygen (PaO2) with variable FiO2 7.4 +/- 0.6 kPa and arterial bicarbonate (HCO3-) 40.1 +/- 1.6 mmol l-1. Ten cases tolerated NIPPV for 1-15 months, mean 5.1 +/- 1.4 months, with subjective improvement in headache and quality of sleep. At 3 months, there was significant improvement in forced vital capacity, PaCO2 and arterial HCO3- and there was a reduction in the number of hospital inpatient days (P < 0.05). Subsequently three cases had lung transplantation, four died on the active list and three are awaiting organs. Two patients failed to tolerate NIPPV owing to abdominal bloating and increasing hypercapnia. In conclusion, NIPPV, if tolerated, was a useful adjunct in the treatment of CF patients with hypercapnic respiratory failure awaiting transplantation. Further prospective studies are required to determine the optimum time to commence NIPPV and to clarify its precise role.

Although the majority of patients with cystic fibrosis (CF) become chronically colonized with Pseudomonas aeruginosa, the mode of acquisition of infection remains unclear. Epidemiological studies using genotyping techniques suggest that person-to-person transmission of this organism may occur. All these studies have utilized sputum or throat swab samples. We have studied the regional microbiological flora of the lungs of five CF patients at post-mortem and genotyped P. aeruginosa strains found therein and in the major airway. We have shown that although in most cases major airway secretions accurately reflect the peripheral lung flora, in cases of multiple strain carriage, major airway cultures may not reflect all strains present in the periphery of the lung. This finding has implications for the interpretation of epidemiological studies that use genotyping of strains from sputum and throat swab samples to assess possible routes of transmission.

The case history is presented of a patient with common variable immunodeficiency in whom heart lung transplantation has been carried out with success. Transplantation was the only long term therapeutic option in this patient due to the progressive respiratory failure resulting from bronchiectasis, emphysema, and granulomatous lung disease.

Asbestos exposure may cause asbestosis, pleural plaques and benign pleural disease, and may pre-dispose to malignant mesothelioma and other neoplasms. The occurrence to two primary tumours in the same patient is rare, and the appearance of a pleural mesothelioma and another lung tumour is exceptional. The present case report describes a patient who, by standard immunohistochemistry, was thought to have mesothelioma at pleuro-pneumonectomy, and adenocarcinoma in the other lung at post-mortem 5 months later. Subsequent investigation using the MOC31 antibody demonstrated a single pathology of adenocarcinoma of the lung. The additional use of this antibody has important histopathological and legal implications.