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.

[Eosinophilic esophagitis: a rare cause of dysphagia]. / Œsophagite à éosinophiles: une cause rare de dysphagie.

Eosinophilic esophagitis is an unrecognized and emerging entity. Its incidence increases with allergic disorders. A 29-year-old man presented with a 4-year history of intermittent and paroxysmal dysphagia. The triad including allergy, young age, and impaction of foreign bodies, combined with a chronic dysphagia is almost pathognomonic of eosinophilic esophagitis. Endoscopic esophageal features can be diverse, so systematic esophageal biopsies are required. Diagnosis is established with the demonstration of an eosinophilic infiltrate with a cell count exceeding 15 eosinophils per high power field (×400). First line therapy includes swallowed topical corticosteroids and removal of an allergic cause, when it could be identified.

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.

[Foreign body perforation of the thoracic esophagus]. / Perforations de l'oesophage thoracique par corps étranger.

BACKGROUND: Esophageal perforation due to foreign body (FB) ingestion is an unusual occurrence. This study aims to define diagnostic difficulties of esophageal perforation by FB. PATIENTS AND METHODS: A chart review of patients on our service with FB esophageal perforation was carried out. Diagnosis of perforation was made by CT scan and/or esophagoscopy. Surgery was indicated when a FB could not be removed endoscopically or on a case-by-case basis according to clinical/laboratory, radiologic, and/or endoscopic findings. RESULTS: Seven patients (age range: 27 to 80 years) were admitted for esophageal FB perforation. All patients presented with dysphagia. Two patients presented with signs of sepsis more than 24 hours after FB ingestion. Perforation was diagnosed at initial evaluation in five cases (three by endoscopy, two by CT) and after FB extraction in two cases. Six patients underwent surgery (suture repair: n=4; esophageal exclusion: n=1; mediastinal drainage: n=1). Five surgeries were performed at the initial diagnosis and one after failure of medical management. Mortality was zero; one patient developed esophageal fistula. CONCLUSION: Diagnosis of FB esophageal perforation is difficult and is delayed in up to a quarter of patients. The perforation can be due to the FB itself or may be incurred during endoscopic extraction. Both CT and endoscopy are necessary for diagnosis and treatment. Most patients require surgical intervention.

[Spontaneous regression of hepatic and splenic involvements during sarcoidosis]. / Nodules hépato-spléniques d'évolution spontanément favorable au cours d'une sarcoïdose.

Splenic and hepatic involvements are frequent and usually clinically silent during systemic sarcoidosis. Imaging findings are extremely rare. We report hepatic and splenic nodules diagnosed on abdominal computed tomography with spontaneous regression.

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.

[Ruptured mesenteric aneurysm from Listeria monocytogenes]. / Rupture d'un anévrisme infectieux à Listeria monocytogenes de l'artère mésentérique inférieure.

Infections due to Listeria monocytogenes usually occur in pregnant women, in elderly or immunocompromised patients. We report the case of an immunosuppressed woman who had been hospitalized for a meningoencephalitis due to L. monocytogenes. She was operated on for an abdominal hemorrhagic shock due to a ruptured mycotic aneurysm of the inferior mesenteric artery. Arterial aneurysms due to this germ are rare and occur on large arteries. Only 17 cases have been previously described in the literature and just one concerning a mesenteric artery.

[Toxocariasis mimicking liver tumor]. / Toxocarose hépatique pseudotumorale.

INTRODUCTION: Toxocariasis is a common helminth infection that causes visceral larva migrans in humans. It has a wide and varied clinical spectrum. CASE: A 60-year-old woman consulted after 6 months of recurrent biliary pain with fever. Laboratory tests showed moderate disturbance of liver enzymes and hypereosinophilia (10 400/mm3). Imaging studies revealed a pseudotumor in hepatic segment IV, hyperechoic in abdominal ultrasound and low density in computed tomography. The diagnostic conclusion after ultrasound-guided liver biopsy was unspecific granulomatous hepatitis and eosinophilic infiltrate. The course of the antibody titer to Toxocara canis finally resulted in a diagnosis of Toxocara infection. Outcome was good following treatment with diethylcarbamazine. DISCUSSION: Some clinical features of liver toxocariasis can mimic tumors and may be interpreted histologically as granulomatous hepatitis, eosinophilic infiltrate of the hepatic portal vein, and/or necrotizing eosinophilic abscesses. Treatment is not yet well codified, but the symptomatic forms call for albendazole or diethylcarbamazine.

[Klinefelter's syndrome diagnosed three years after surgery for mediastinal teratoma]. / Diagnostic d'un syndrome de Klinefelter trois ans après chirurgie d'exérèse d'un tératome médiastinal.

INTRODUCTION: The association between some types of tumor and Klinefelter's syndrome (KS) is often missed and diagnosis of the latter delayed, as in this case report. CASE: We present the case of a 20 year-old patient for whom KS was not diagnosed until three years after thoracic surgery for a mediastinal teratoma. DISCUSSION: The association between KS and mediastinal germ-cell tumors is not coincidental; it illustrates the relation between aneuploidy and oncogenesis. In a young adult male, palpation of the scrotum to look for microorchidism is justified when these tumors are diagnosed because 25% occur in patients with KS.

[Hemolytic uremic syndrome as a complication of gemcitabine treatment: report of six cases and review of the literature]. / Syndrome hémolytique et urémique secondaire à la gemcitabine: à propos de six observations et revue de la littérature.

UNLABELLED: Hemolytic uremic syndrome is a rare condition during gemcitabine therapy. METHODS: We report six new cases of hemolytic uremic syndrome related to gemcitabine, three issued from a retrospective study of 136 consecutive patients treated with gemcitabine for which a systematic screening of this side effect has been performed and 29 cases with clinical data available identified in the literature in order to better characterised frequency and clinical presentation of this side effect. RESULTS: In our series, frequency of HUS is 2.2% and is higher than this previously reported (0.015%) or estimated with the data of clinical trials analysed (0.072 %). For 35 cases with clinical data available, the patients were always treated for a local advanced and/or metastatic disease. For our cases and for literature cases, at the time of diagnosis of hemolytic uremic syndrome, mean number of doses received (mean+/-standard deviation. Minimum/maximum)) (personal cases: 26.5+/-6.6. 16/36, literature cases: 21+/-11. 8/54), cumulative dose received (g/m2) (personal cases : 24.5+/-6.3. 16/31.6, literature cases: 21.7+/-12.4. 2.4/54) and duration of treatment (months) (personal cases: 8.2+/-1.9. 5.6/11, literature cases: 8.5+/-4.0. 3/18) are very closed and high individual variations observed for these factors are not consistent with a time and/or dose dependant toxicity. New-onset hypertension or exacerbation of underlying hypertension is the most common clinical manifestation, with mild anemia; thrombocytopenia is inconstant. The degree of severity of renal failure is highly variable. The existence of subacute clinical form with progressive worsening of the symptoms and biological form at the time of diagnosis suggest the interest of a systematic clinical and biological screening of this side effect, before each injection of gemcitabine. Early prognosis is linked to the evolution of hemolytic uremic syndrome and after hemolytic uremic syndrome healing, cancer progression. Treatment include gemcitabine discontinuation, antihypertensive drugs and if necessary fresh frozen plasma. CONCLUSIONS: Systematic clinical and biological screening of hemolytic uremic syndrome during gemcitabine therapy should allow to better know this complication, to recognize and treat it earlier with a potential positive impact for patients.

[Diabetes and amoebiasis: a high-risk combination]. / Le diabète et l'amibiase: une rencontre a haut risque.

Amoebiasis is the second most common parasitic disease in the world. It occurs mainly in developing countries. Many people in endemic countries are asymptomatic carriers. It results in severe disease that can be fatal in rare cases. The case described in this report illustrates the growing risk of exposure to amoebiasis for diabetic patients as travel to endemic countries becomes more and more frequent. In the patient described here amoebiasis led to amoeboma, a rare complication of the colonic presentation. Despite a clinical disease syndrome mimicking that of an occluding gut tumor, the patient was treated medically with drugs alone. Retrospective studies show that diabetics are at higher risk for severe complications after amoebic infection. Because of the high incidence and severity of concurrent diabetes and amoeba, prophylactic measures are necessary for diabetic patients traveling in developing countries.

Diabetes and Amoebiasis: a high risk encounter.

Amoebiasis is the second most common parasitic disease worldwIde. It occurs mainly in developing countries. A high percentage of people in countries where it is endemic are asymptomatic carriers. It results in severe disease that can be fatal in rare cases. Diabetics are at increased risk of exposure as travel to countries where it is endemic becomes more frequent, as indicated by the present case. This patient suffered from amoebiasis that produced an amoeboma which is most rare in cases of colonic amoebiasis. The clinical picture was that of an occluding gut tumor, but it was treated only with drugs. Retrospective studies show that diabetics are at increased risk of suffering severe complications after amoebic infection. The frequency and severity of this diabetes-amoeba association requires patients to take prophylactic measures, especially when travelling in developing countries.

Nontuberculous mycobacteria: incidence in Southwest Ireland from 1987 to 2000.

SETTING: The Southwest of Ireland (Counties Cork and Kerry) 1987-2000, average population 549,500. OBJECTIVE: Nontuberculous mycobacteria (NTM) cause significant morbidity worldwide and the study of epidemiology and characteristics helps in their prevention and treatment. This study was performed to determine the incidence of NTM disease in comparison to Mycobacterium tuberculosis (M. tuberculosis) and Mycobacterium bovis (M. bovis) in Southwest Ireland, over the above time period. DESIGN: A retrospective study was carried out in all human isolates of NTM, M. tuberculosis and M. bovis between 1987 and 2000, in the Southwest Region of Ireland. RESULTS: The mean incidence of NTM (0.4/100,000 population) has risen since 1995, principally of pulmonary Mycobacterium avium intracellulare complex (MAC). The annual incidence of M. tuberculosis in humans over 14 years in the same region was 971/100,000 population with a significant reduction since 1994 and M. bovis remained constant at 0.5/100,000 population. CONCLUSION: The increasing incidence of disease causing NTM noted in Southwest Ireland reflects global data and is surmised to be due to an ageing population, increased incidence related to chronic fibrotic lung disease, and environmental mycobacterial factors.