Search

While it has long been appreciated that up to 98% of men with cystic fibrosis (CF) are infertile due to a failure to develop, or early blockage of, the mesonephric ducts and vas deferens,1 the effect of CF on the fertility of women is less clear. Survival is now into the fourth decade of life and, with the largest growth in the CF population seen in the adult age groups,2 it is not surprising that the issues of fertility and reproduction are increasingly raised in adult clinics. Pregnancy is possible and increasing numbers of children are born to women with CF each year.3 Since the first pregnancy in 1960,4 management of all aspects of CF has improved and so the prognosis for mother and child has also improved. This paper reviews the pathophysiology of CF in the female reproductive tract, its effect on fertility and the ability to carry a pregnancy, and the outcome for mother and infant. CF is an autosomal recessive disease arising from abnormalities on the long arm of chromosome 7. Defective production of the cystic fibrosis transmembrane conductance regulator (CFTR) protein5—a complex channel regulating the passage of chloride and, indirectly, sodium and water across luminal cell membranes—results in thickened and desiccated secretions throughout the respiratory and alimentary tracts, sweat ducts, and the reproductive organs. The principal manifestations in adults are due to undernutrition resulting from pancreatic insufficiency and malabsorption, and respiratory failure due to chronic infection, bronchiectasis, and progressive lung destruction. Diabetes mellitus and cirrhosis are secondary manifestations resulting from pancreatic and biliary damage and are increasingly seen in older patients. CFTR is found in large quantities on the cervix6 and, although anatomically normal, histologically the columnar epithelium is distorted by mucus filled balloon or cygnet ring cells. The …

BACKGROUND: There has been increasing concern since 1979 about the emergence of Pseudomonas cepacia (Burkholderia cepacia) in patients with cystic fibrosis in the UK and elsewhere. Colonisation of the sputum has been shown to be associated with increased morbidity and mortality. Evidence suggests person to person transmission and some centres have segregated those colonised with B cepacia from other patients with cystic fibrosis. The outcome of patients colonised by B cepacia has been studied, together with the effects of strict segregation. METHODS: The outcome in 18 patients with sputum colonised by B cepacia was compared with that in age, sex, and severity matched controls with no evidence of B cepacia colonisation by a retrospective case note study. RESULTS: No difference between cases or controls were found in the 24 month period prior to colonisation by B cepacia in lung function, number of days in hospital, or outpatient visits. Colonisation led to an increased rate of loss of lung function and utilisation of hospital services. There was an increase in the numbers of transplants and deaths amongst the cases. Since 1992 there have been only three new cases of B cepacia colonisation and the incidence and prevalence of the organism has fallen dramatically since segregation commenced. CONCLUSIONS: B cepacia appears to be linked to the decline in colonised individuals. There was no evidence that colonisation occurred in patients declining for other reasons. B cepacia colonisation confers a worse prognosis than Pseudomonas aeruginosa alone. Segregation appears to limit the spread of the organism from infected individuals to other patients with cystic fibrosis.

The case history is presented of a patient with acute respiratory failure complicated by nasal obstruction resulting in intolerance of nasal ventilation. Urgent insertion of nasal stents permitted restoration of ventilation with resolution of breathlessness and stabilisation of arterial blood gases.

The medical management of a patient with Crohn's disease should take into account the activity, site and behaviour of disease, and should be discussed with the patient, and 5-aminosalicylates are a group of medications which have been commonly used. Sulfasalazine is a combination of 5-aminosalicylic acid and sulfapyridine which acts only as a carrier to the colonic site of action but can still cause systemic side-effects1 including lung disease. In mesalazine the specific sulfapyridine-related side-effects, especially pulmonary reactions, are avoided. However, we present a case of lung fibrosis which was associated with mesalazine in a Crohn's patient.

Introduction The association between gastro-oesophageal reflux (GOR) and various respiratory disorders including cystic fibrosis (CF) is widely acknowledged. Although the exact nature of this relationship is uncertain, it is thought that reflux aspiration is a contributing factor in lung dysfunction in these patients. Patients with GOR typically report symptoms such as heartburn, regurgitation, and occasionally atypical symptoms such as cough. It has been hinted that GOR in CF patients may be “silent” making detection and treatment often difficult. We piloted a cross-sectional survey to gain a better understanding of the perception of GOR and its impact on lung function. Methods Consenting patients completed two validated questionnaires regarding GOR symptoms which were scored and ranked (Gastro-oesophageal Reflux Symptom Questionnaire and Reflux Symptom Index). Patients were stratified by score and divided into two pilot groups; SGOR (symptomatic-highest scores) and AGOR (asymptomatic-lowest scores). Both groups underwent 24-h multiple intraluminal impedance/pH metry (MII) and high-resolution manometry (HRM) for positioning of MII probe. MII studies were assessed for the number of GOR events, proximal reflux events (PREs), and total proximal bolus exposure (TPBE). Patients remained on all current medication including PPIs. Clinical data were collected and compared retrospectively including most recent lung function results (FEV1). Result 53/110 patients completed questionnaires and 10 from each group were invited to undergo investigation—11 were completed successfully (age 18–55 years; four male, seven female; seven SGOR, four AGOR). There was little difference in the mean number of events between the groups (range 16–75, mean—SGOR 38, AGOR 42; (normal on PPI=48, off PPI=73)). There was no correlation between symptom scores (SS) and number of GOR events but there was a moderate negative correlation between the number of GOR events/FEV1. There was no significant difference in the mean number of PREs between the groups (range 1–31; mean SGOR=11, AGOR=22). TPBE ranged from 0.2–342 s. No correlation was shown between; number of PREs/SS, number of PREs/FEV1 and TPBE/FEV1. Conclusion This small pilot study indicated that CF patients demonstrate impaired awareness of GOR and supports the theory that GOR is associated with poorer lung function. While there was no significant difference in the number of PREs between the groups, levels were higher in the AGOR group. This suggests that CF patients have poor perception of proximal reflux, which could increase the likelihood of aspiration. Based on these findings we aim to continue the survey to gain a more comprehensive assessment of the perception of GOR in adults with CF.

Sarah Bache and Frank Edenborough uncover scores of health problems associated with making music