An unusual case of Legionellosis.

Legionella species is a common cause of community-acquired pneumonia. However disease due to L. pneumophila serogroup 13 is rare and has not previously been reported in Ireland. It may not be detected by routine Legionella antigen and antibody kits. Due to these limitations, early culture should be considered when legionellosis is suspected. The potential therapeutic benefit of quinolones in the management of this disease is also illustrated.

Sarcoidosis in Ireland: regional differences in prevalence and mortality from 1996-2005.

BACKGROUND: Sarcoidosis is a common multisystem disease of unknown cause and Ireland is among the countries with the highest reported prevalence of disease worldwide. Despite this, reports on the geographical distribution of disease and differences in mortality due to sarcoidosis within Northern Ireland (NI) and the Republic of Ireland (ROI) are currently lacking. OBJECTIVE: This study was performed to examine sarcoidosis prevalence and mortality in Ireland (NI and ROI) to specifically determine if geographical or temporal clusters of disease are present and if any differences in mortality exist between NI and ROI. DESIGN: A retrospective study, examining hospital discharge data for NI and ROI and data on deaths due to sarcoidosis, obtained from the relevant official government agencies. RESULTS: For 1996-2005, the prevalence of sarcoidosis was 28.13 per 100,000 for ROI compared with 11.16 per 100,000 for NI (p = 0.002). Two significant spatial clusters of disease were detected in the Northwest (Prevalence = 44.9 per 100,000) and also the Midlands region (32.1 per 100,000). Two lower-prevalence spatial clusters were also detected in the South and Southeast of ROI. Temporal clustering was also present throughout ROI and NI for the years 2000 to 2004, while space-time clustering was found in three regions, the West (ROI), the East (ROI) and Northeast (ROI and NI). The case fatality rate for ROI was 0.84%, and for NI was 1.44% (p = 0.03). CONCLUSION: Considerable heterogeneity in disease prevalence is evident in Ireland as significant spatial, temporal and space-time clusters of sarcoidosis are demonstrated in this study. Prevalence rates are also higher than that previously reported for Ireland and are comparable to those of Scandinavian countries. Although case-fatality is low in both ROI and NI, it is significantly lower in ROI. Further study is needed to investigate these findings and the creation of an all-island sarcoidosis registry would provide a mutually beneficial means of capturing this data more effectively.

Predictors of mortality in adults with cystic fibrosis.

Assessment of prognostic indicators in patients with cystic fibrosis (CF) is important. The study's aim was to assess the relative contribution of gender, genetics and microbiology on survival in adults with CF. Adult patients were studied from 1995 to 2005 and data collected included FEV(1) (%predicted), body mass index (BMI), genetics, and microbiology. Data was available on 183 patients in 1995. Forty-five patients died in the subsequent 10 years. Patients who died during the study had lower mean (SD) FEV(1) %predicted in 1995 when compared to those remaining alive, 41.5 (15.2)% versus 69.8 (23.2)% predicted, respectively, P<0.001 and they had lower mean (SD) BMI in 1995, 19.2 (3.3) kg/m(2) in comparison to those remaining alive, 20.7 (3.4) kg/m(2), P=0.008. The proportion of patients infected with Pseudomonas aeruginosa and Burkholderia cepacia complex was higher in the group who died during the study compared to those remaining alive, odds ratio 20.9 P<0.0001 and 7.1 P<0.0001, respectively. The presence of the Delta F508 homozygous mutation did not alter survival, P=0.3. Patients infected with either P.aeruginosa or B.cepacia complex had reduced survival compared to those without infection, P=0.01 and P<0.0001, respectively. FEV(1)% (P<0.0001), infection with P.aeruginosa (P=0.005) or B.cepacia complex (P=0.03) were the only significant predictors of mortality. This study demonstrates adults who died were more likely to have worse lung function and be infected with either P.aeruginosa or B.cepacia complex. FEV(1)% and infection with P.aeruginosa or B.cepacia complex were the most significant predictors of survival in adults with CF.

Farmer's lung is now in decline.

Farmer's lung incidence in Ireland was constant until 1996, even though hay making methods were revolutionised in late 1980's. We undertook this study to find out the incidence of farmer's lung in Ireland from 1982-2002 and its correlation with rainfall and the effect of changing farm practices. The primary cases of farmer's lung were identified from Hospital in Patients Enquiry (HIPE) unit of the national Economic & Social Research Institute (ESRI) Dublin. Rainfall data were obtained from Met Eireann whereas population, hay production and silage production were obtained from the Central Statistics Office, Dublin. As the farming population is in decline, we used the annual working unit (AWU), which reflects the true population at risk. An AWU is the equivalent of 1800 hours per farm worker per year. The incidence rates were constant from 1982-1996, but from 1997-2002 a marked decline was observed. There was strong positive correlation with hay production (r = 0.81) and strong negative correlation with silage production (r = -0.82). This study indicates that the incidence of farmer's lung is now in decline.

Pulmonary function in long-term survivors of thoracoplasty.

Pulmonary function was assessed in 15 patients who had undergone thoracoplasty (TPL) approximately 30 years previously. Relation was noted to extent of TPL performed, side of TPL, age at TPL, and degree of secondary scoliosis. Test results showed mixed restrictive and obstructive defects. Although extent of TPL did not correlate with current lung function values, the restrictive defect was probably caused by a mixture of (a) compression of lung by TPL and (b) fibrosis from old pulmonary tuberculosis. The obstructive defect might have been due to the TPL procedure itself or to chronic bronchitis related to (a) previous pulmonary TB or (b) cigarette smoking, since 14 subjects were former or current smokers. That Dsb was relatively preserved suggests that pulmonary hypertension as a late sequela of TPL may be reactive (secondary to hypoxemia and respiratory acidemia) rather than obliterative.

Subclinical anaemia of chronic disease in adult patients with cystic fibrosis.

Patients with chronic hypoxaemia develop secondary polycythaemia that improves oxygen-carrying capacity. Therefore, normal haemoglobin and haematocrit values in the presence of chronic arterial hypoxaemia in cystic fibrosis constitute 'relative anaemia'. We sought to determine the cause of this relative anaemia in patients with cystic fibrosis. We studied haematological indices and oxygen saturation in healthy volunteers (n=17) and in adult patients with cystic fibrosis (n=15). Patients with cystic fibrosis had lower resting arterial oxygen saturation when compared with normal volunteers (P<0.0001), and exercise led to a greater reduction in arterial oxygen saturation (P<0.0001). However, haemoglobin and haematocrit values in patients with cystic fibrosis did not significantly differ from normal volunteers. Serum iron (P=0.002), transferrin (P=0.02), and total iron-binding capacity (P=0.01) were lower in patients with cystic fibrosis. There were no significant differences in serum ferritin, percentage iron saturation, serum erythropoietin or red cell volume between the groups. The data presented demonstrate a characteristic picture of anaemia of chronic disease in adult patients with cystic fibrosis, except for normal haemoglobin and haematocrit values. Normal haemoglobin and haematocrit values in patients with cystic fibrosis appear to represent a combination of the effects of arterial hypoxaemia promoting polycythaemia, counterbalanced by chronic inflammation promoting anaemia of chronic disease.

Elevated serum angiotensin converting enzyme levels in metastatic ovarian dysgerminoma.

A case of a 32-year-old XY genotype female is described, presenting with mediastinal and abdominal lymphadenopathy and associated with an elevated serum angiotensin I converting enzyme (SACE) level. Lymph node histology showed a malignant dysgerminoma of ovarian origin. Combined chemotherapy led to a radiological regression of the lymphadenopathy and coincided with a decrease in SACE concentration. The authors suggest that SACE may be a marker for disseminated germinoma tumours and may be useful for monitoring treatment.

Fungal atopy in adult cystic fibrosis.

This study set out to estimate the prevalence of atopy to a variety of common ubiquitous fungi, including A. fumigatus, in cystic fibrosis (CF), and to evaluate the investigations by which the diagnosis was made. Particular attention was paid to the usefulness of skin testing and immunoassays in detecting which patients had simple fungal atopy, and which patients were at high risk of developing allergic bronchopulmonary mycoses. This cross-sectional study included 21 adult CF patients and 20 matched controls. Serum samples were taken for the measurement of total serum IgE and specific serum IgE to nine common fungi. Immediate hypersensitivity skin prick testing to each of the fungi was also performed. Simple fungal atopy was described in subjects fulfilling the following criteria: total serum IgE > 100 KU l(-1) with specific radioimmunoassay > or = grade 1 to at least one fungus and a positive skin prick test (SPT) > or = 3 mm to the same fungus. 'High risk' for developing allergic bronchopulmonary mycosis (ABPM) was described in subjects fulfilling the following criteria: total serum IgE > 200 KU l(-1) with specific radioimmunoassay > or = grade 2 to at least one fungus and a positive skin prick test (SPT) > or = 6 mm to the same fungus. The adult CF group had a significantly higher total SPT score (P=0.005) and mean total serum IgE (P<0.05) than controls. Forty-three percent of CF patients fulfilled the criteria for fungal atopy to at least a single fungus. Over half this group had an atopic tendency to more than one fungus. Nineteen percent of the CF group were at least 'high risk' of developing ABPM. Skin prick testing is a better marker of fungal atopy and a better predictor of those adult CF patients at higher risk of developing ABPM than specific radioimmunoassay serum testing. There is a high prevalence of fungal atopy in the adult CF population. Total serum IgE and skin prick testing are good predictors of fungal atopy and help predict those at risk of developing ABPM in CF.

The relationship between the tuberculin test and the Kveim test in sarcoidosis in Ireland.

The aim of this study was to correlate tuberculin reactivity with the Kveim test in sarcoidosis. Case records 1983-91 inclusive, of those patients with positive Kveim tests and recorded tuberculin tests in an acute regional general hospital serving south-west Ireland (counties Cork and Kerry) were reviewed. Of 39 patients with positive Kveim tests 28 (72%) were tuberculin negative to a test dose of 10 tuberculin units or more. The group was slightly though significantly younger (p < 0.05) in age than the corresponding group (n = 11; 28%) with positive tuberculin tests. The incidence of tuberculin reactivity in the presence of Kveim reaction is higher than that expected for sarcoidosis. Corresponding studies in Britain and Scandinavia show a considerably lower tuberculin positive rate, which may be due to a lower prevalence of tuberculosis in these countries. However the younger age of the tuberculin negative group in our study may reflect a continuing decline in tuberculosis prevalence in south-west Ireland.

Endometrial and pulmonary sarcoidosis.

A 33 year old woman with irregular menses due to endometrial sarcoidosis and a family history of sarcoidosis is described. Its relative increase in importance in the wake of the decline in the incidence of genital tract tuberculosis in Ireland is discussed.

Period prevalence of allergic bronchopulmonary mycosis in a regional hospital outpatient population in Ireland 1985-88.

The period of prevalence, 1988 inclusive, of Allergic Bronchopulmonary Aspergillosis (ABPA) and Allergic Bronchopulmonary Candiasis (ABPC), the two most frequently reported forms of allergic Bronchopulmonary Mycosis (ABPM), was investigated in an Irish regional hospital respiratory medicine outpatients, catchment area population 536,000. ABPM was defined by the presence of a majority of seven criteria: asthma, eosinophilia, elevated IgE, antibodies to Aspergillus Fumigatus or Candida Albicans, immediate skin test reaction to either fungus, culture of either fungus in sputum, and otherwise unexplained transient or permanent lung field x-ray abnormalities. New referrals were investigated for ABPM if they had asthma and eosinophilia, or asthma and lung field x-ray abnormalities. Fourteen patients with ABPM were identified from a total of 1390 new referrals, a period prevalence of just over one per cent. It is concluded that (1) ABPM is a relatively common disorder in an Irish regional hospital respiratory medicine outpatient population and (2) ABPC constitutes a higher proportion of this disorder than previously considered.

Farmer's lung in Ireland (1983-1996) remains at a constant level.

A prospective study was undertaken by the Departments of Respiratory Medicine and Medical Microbiology at the Cork University Hospital, a. to investigate the epidemiology of Farmer's Lung (F.L.) in the Republic of Ireland (pop. 3.5 million), with special reference to the South Western Region of this country (pop. 536,000) and b. to assess any relationship between the prevalence/incidence of F.L. with climatic factors in South West Ireland, between 1983 and 1996. F.L. incidence remained constant throughout the 13 yrs studied both on a national and a regional basis. A significant relationship was also found between total rainfall each summer and F.L. incidence and prevalence over the following yr (p < 0.005) in South-West Ireland. The persistence of F.L. in Ireland at a constant level suggests that farmers' working environment and farm practices need to be improved.

Laparoscopic cholecystectomy in adult cystic fibrosis.

Two female patients with Cystic Fibrosis, attending the Adult Regional Cystic Fibrosis centre at the Cork University Hospital, were investigated for upper abdominal pain and found to have gallstones at ultrasonography. Laparoscopic cholecystectomy was performed successfully and, without complication, in both patients.

Appropriateness of laboratory tests: requests for atypical pneumonia serology in a teaching hospital.

The cost of providing medical care is ever-increasing but the resources available are at best static. Major savings can be made by reducing inappropriate investigations. Using serological testing for organisms causing atypical pneumonia as an example, we examined the appropriateness of requests and also physicians' understanding of the test. Of 119 patients tested, only 3 had titres indicative of acute infection. Most patients were tested within 2 days of hospital admission, before receipt of results excluding more likely diagnoses. Forty-five patients had no current or recent respiratory symptoms, in whom infection was highly unlikely. Titres were most often requested by the least experienced members of the clinical team. Of 70 patients with an acute illness in whom a definitive diagnosis, bacteriological or otherwise, was not made, in only 9 was a convalescent specimen sent for follow-up titres. Most requests for serology for organisms causing atypical pneumonia were inappropriate. Furthermore, in the majority of cases the test was incorrectly used.

Pulmonary vascular occlusive disease presenting as sudden death.

We report a case of pulmonary vascular occlusive disease (also termed pulmonary veno occlusive disease) which caused the sudden death of an eleven-year-old girl. The post-mortem findings are described and the current theories of aetiology and pathogenesis of this unusual condition are reviewed. The clinical picture is characterized by pulmonary hypertension with marked increase of pulmonary arterial pressure but often normal or only slightly elevated wedge pressure. The pulmonary vasculature, especially the veins, show progressive fibrotic occlusion. The aetiology is unknown but an infective cause is considered most likely. Definitive diagnosis usually depends on an open lung biopsy or, more commonly, autopsy examination.

Human Mycobacterium bovis infection in the south-west of Ireland 1983-1992: a comparison with M. tuberculosis.

Epidemiological and bacteriological aspects of human Mycobacterium bovis disease were investigated in south-west Ireland (counties Cork & Kerry, population 536,000) over the years 1983-92 inclusive and compared to M. tuberculosis. Results showed a small, stable incidence of culture positive M. bovis human disease, mean annual incidence 0.56 per 100,000 population compared to a higher but declining incidence of culture positive M. tuberculosis (15.3 per 100,000 in 1983, 9.0 per 100,000 in 1992). Male patients were the majority, 63.4 per cent of M. bovis; 62.4% of M. tuberculosis (p = 0.03). Fifty three per cent of M. bovis cases (n = 30) were pulmonary, compared to 85% of M. tuberculosis (n = 626; p = 0.0001). M. bovis patients were older (p = 0.02), mean age 58.4 years (SD 18.9) compared to 48.5 (SD 22.2). The mycobacterial smear positive rate was similar in both groups taken as a whole. No rural-urban difference in incidence was found in either disease, suggesting in the case of M. bovis initial infection in childhood via contaminated milk in the pre-pasteurisation era.