[Show abstract][Hide abstract] ABSTRACT: Lung transplantation (LTx) is an accepted therapy in children with end-stage lung diseases. Pediatric-specific experience is considered important in pediatric LTx. We present our institutional experience and its outcome since the year 2000, asking whether different treatment strategies produce comparable outcomes in pediatric lung transplant recipients at our predominantly adult center. This is a retrospective analysis of children and adolescents aged ≤20 years, undergoing LTx between January 2001 and December 2013. Minimum follow-up was 12 months. Primary endpoints were re-transplantation or death. We performed 33 lung transplant procedures in 29 patients. Survival 1 month post-operatively was 96.6%, at 3 months 93.1% and at 12 months 82.8%, respectively. At the end of our follow up, 72.4% of our pediatric cohort was still alive — median post-transplant survival was 59 months (range 0–159). 72.4% of the children were transplanted with support of extracorporeal membrane oxygenation (ECMO), size-reduced donor grafts were used in 69.0%. The differences between post-transplant survival of the “non-ECMO-group” versus the “ECMO-group” (137 vs. 28 months, P=0.7) and “full size” versus “size-reduced bilateral transplants” (61 vs. 28 months, P = 0.7) were not significant, though. There were no anastomotic complications, also not in size-reduced lungs. Our results are well comparable to the international data and show excellent short- and mid-term outcomes. We advocate ECMO-bridge to be considered as a valuable treatment option to prolong time on the waiting list in highly selected patients, as well as size reduction and lobar transplants as a strong strategy to increase the donor pool and reduce donor-recipient size-mismatches. Pediatr Pulmonol.
Full-text · Article · Jan 2016 · Pediatric Pulmonology
[Show abstract][Hide abstract] ABSTRACT: Elevation of the mean pulmonary arterial pressure to ≥25 mm Hg within the low-pressure system of the pulmonary circulation is defined as pulmonary hypertension. Pulmonary hypertension may be the consequence of various clinical and pathophysiological entities. Many of these conditions, however, result in a final common pathway of pathogenesis. This pathway is characterised by the triad of excessive vasoconstriction, microthrombosis and remodelling of pulmonary arteries. Remodelling is arguably the most important factor: its complex pathogenesis is not completely understood and no specific treatment directly targets vascular remodelling. This article aims to review the current understanding of the pathogenesis of pulmonary hypertension and to give insights in future developments in this evolving field.
No preview · Article · Oct 2015 · Schweizerische medizinische Wochenschrift
[Show abstract][Hide abstract] ABSTRACT: Cystic fibrosis (CF) is one of the most common genetic disorders. Mutations of the cystic fibrosis transmembrane regulator causes dysfunction of epithelial membranes within the gastrointestinal and respiratory system. Patients with CF are known to be at risk for gastrointestinal malignancies, and lung transplantation further increases this risk. We report a case series of three CF patients who developed adenocarcinoma of the gastrointestinal tract in the posttransplant setting. One of these case histories describes a gastric cancer, which is a novel and to date unreported observation. These data emphasise the importance of checking CF patients for the development of abdominal complications following lung transplantation.
No preview · Article · Aug 2015 · Schweizerische medizinische Wochenschrift
[Show abstract][Hide abstract] ABSTRACT: History and admission findings: Phenylketonuria is the most common genetic disease in amino acid metabolism. We report the case of a 22-year old patient with phenylketonuria and psychological symptoms. After early treatment, phenylalanine levels had been controlled and were within target area. Investigations: Clinical interview and psychometrics showed atypical eating disorder and anxiety disorder. Diagnosis, treatment and course: Possible toxic effects and psychological factors may play a role in pathogenesis. Conclusions: Most likely the frequency of eating disorders and anxiety disorders in phenylketonuria is underestimated.
No preview · Article · Jul 2015 · DMW - Deutsche Medizinische Wochenschrift
[Show abstract][Hide abstract] ABSTRACT: Levels of microRNAs (miRNAs) are increasingly assessed in biological fluids, for example, in samples obtained by bronchoalveolar lavage (BAL). "Post-collection kinetics" of miRNA expression levels, however, have not been investigated to date. In these experiments, we analyzed the dynamic expression profile of 5 different miRNAs (miR-17, miR-19b, miR-20b, miR-125a, and miR-223-3p) in BAL within the first 24 h following collection by routine bronchoscopy. miRNAs were quantified 0, 1, 4, 8, and 24 h after collection in samples that were kept at 4 °C or at room temperature. The expression of all five miRNAs was found to remain stable between the first 8 h after collection. 24 h after collection miRNAs faced substantial alterations in their expression profile. These data emphasize that BAL samples intended for further miRNA analysis can be handled at room temperature within the first 8 h after bronchoscopy.
No preview · Article · Mar 2015 · Beiträge zur Klinik der Tuberkulose
[Show abstract][Hide abstract] ABSTRACT: We investigated the prevalence of bronchial asthma in patients with Tako-Tsubo Syndrome (TTS).
This retrospective case-series study was conducted in a primary care hospital in Zurich, Switzerland. Data of all patients with newly diagnosed TTS (2002 - 2012) were assessed electronically by the use of ICD-10. Asthma prevalence was compared to published epidemiologic data.
Bronchial asthma is characterized by airway inflammation and, during attack, release of endogenous catecholamines. Sympathomimetic drugs are the mainstay of treatment for asthma patients. Likewise, catecholamine mediated diffuse microvascular myocardial dysfunction seems to be of critical importance for the development of TTS.
20 cases of TTS were identified. 90% were female, showed a median age of 70±13y [25y - 90y], an apical and/or midventricular ballooning pattern with preserved basal function and a median initial LVEF of 34±9% [25% - 55%]. 65% of patients underwent coronary angiography to rule out significant coronary artery disease. Hypertension was present in 45% of patients, 35% were smokers, none was suffering from diabetes. Prevalence of asthma in patients with TTS was significantly higher compared to the normal population (25% vs. 7%, p=0.012). In 30% of the TTS patients an iatrogenic cause for development of TTS was identified.
Prevalence of asthma was significantly higher in patients with TTS compared to epidemiologic data from an age-matched population. Phenotypes of patients developing obstructive ventilatory disease and TTS might share common pathogenic mechanisms beyond the use of bronchodilatators. In addition, we identified other iatrogenic etiologies in patients with TTS.
Preview · Article · Feb 2015 · The Open Cardiovascular Medicine Journal
[Show abstract][Hide abstract] ABSTRACT: Lung transplant recipients require life-long profound immunosuppression, making them prone to respiratory tract infections (RTIs), in particular viral infections, during the winter season. Since RTIs may have severe consequences, of which acute or chronic allograft dysfunction is feared most, early diagnosis and treatment are recommended. Patients monitor lung function daily at home and are instructed to contact the transplant center in case of signs and symptoms suspicious of RTIs. We then obtain nasopharyngeal swabs for viral and bacteriological examination
and initiate pre-emptive treatment with antivirals, broadband antibiotics, and sometimes intravenous immunoglobulins. Treatment duration is guided by virology sampling results. As preventive measures, we provide vaccination against seasonal influenza for patients and household contacts and recommend specific daily hygiene measures.
[Show abstract][Hide abstract] ABSTRACT: Molecular biomarkers are becoming increasingly significant in the workup of lung carcinoma patients. They assist in diagnosis, selecting the most adequate therapy and determining prognosis. Obtaining blood based biomarkers or volatile markers in exhaled breath may provide a less invasive method in the future. For the time being, bronchoscopy is still the method of choice to obtain specimen and assess tissue based biomarkers. The techniques how specimen are collected and processed for analysis are of paramount importance.