Karin M. Unsinn

Sensitivity of lung clearance index and chest computed tomography in early cf lung disease

It is widely accepted that CF lung disease starts before clinical symptoms become apparent or spirometry deteriorates. Computed chest tomography (CT) is the reference method for identifying structural changes in CF; however, radiation exposure limits its use as a monitoring tool. It has been suggested that the Lung Clearance Index (LCI) measured by Multiple Breath Washout (MBW) for assessing ventilation inhomogeneity is a more sensitive surrogate marker than spirometry allowing non-invasive monitoring of CF lung disease. The aim of this study was to prospectively investigate the diagnostic accuracy of the LCI in comparison to CT in CF patients with early lung disease and normal FEV(1) (>80% pred.). MBW and ultra-low-dose CT were performed in 34 patients (6-26 years). LCI was abnormal in 76.5% subjects. LCI and CT correlated significantly in 82.3%. LCI was related to presence and extent of structural lung changes observed on CT with a sensitivity of 88%. Diagnostic accuracy of the LCI for detecting CF lung disease in patients with normal FEV(1) was good when compared to CT. Results indicate that structural changes are unlikely if a normal LCI is measured. We speculate that serial measurements of the LCI for assessing ventilation inhomogeneity may help to identify early structural lung disease and help to reduce the individual cumulative radiation dose. The LCI may be a suitable surrogate marker for monitoring progression of CF lung disease and effect of treatment in both, clinical care and research settings.

Tracking Lung Clearance Index and chest CT in mild cystic fibrosis lung disease over a period of three years

INTRODUCTION Lung disease remains the main cause of morbidity and mortality in patients with Cystic Fibrosis (CF). To detect lung disease before clinical symptoms become apparent, sensitive tools are essential. Spirometry is used for monitoring, but the FEV1 remains frequently normal throughout childhood. The Lung Clearance Index (LCI) calculated from Multiple Breath Washout (MBW) was introduced at the CF centre Innsbruck in 2007 for assessing ventilation inhomogeneity in patients with mild lung disease. We hypothesized that LCIs in 2007 are of prognostic value for the presence or absence of structural lung changes in later years. METHODS Between 2007 and 2010 MBW, spirometry and ultra-low-dose HR-CT were prospectively tracked in 36 patients (6-53 years) with a mean FEV1 ≥ 80% predicted in 2007. RESULTS At study start the majority of patients had abnormal CT scores and LCI results. While CT and spirometry remained largely stable throughout the study, LCI results slightly improved but still correlated with CT scores in 2010. LCI results in 2007 correlated with CT scores in 2010 while FEV1 did not. In 86% the LCI value in 2007 was indicative for the presence or absence of structural lung changes in 2010. CONCLUSION The LCI is a sensitive tool for detecting and tracking pulmonary changes. Extended structural changes are unlikely if the LCI is normal. The LCI has the potential to be used for monitoring the progression of early CF lung disease and assessing the effect of treatment in both clinical care and research settings.

Segmental bowel-wall thickening on abdominal ultrasonography: an additional diagnostic sign in Kawasaki disease

Abdominal complaints are a frequent early feature of Kawasaki disease, an acute systemic febrile vasculitis of childhood. The only well known correlating morphological finding on abdominal US is gallbladder hydrops. We report two boys with Kawasaki disease who showed segmental thickening of the small-bowel wall on abdominal US. These changes are thought to result from bowel-wall oedema due to vasculitis of the supplying vessels. We conclude that in an acutely ill febrile child with the finding of segmental bowel-wall thickening on abdominal US, Kawasaki disease should be considered in the differential diagnosis. The search for gallbladder hydrops and segmental bowel-wall thickening on abdominal US may serve as an important diagnostic tool in Kawasaki disease, supporting early recognition and prompt treatment.

Imaging in Pancreas and Intestinal Transplantation

Successful pancreas transplantation is currently the only known therapy that establishes an insulin-independent euglycemic state with normalization of glycosylated hemoglobin levels. Insulin-secreting cells are part of the pancreatic islets, which are predominantly located in the tail (Williams 1995). In the majority of cases, pancreas transplantation is performed together with the kidney from the same donor as simultaneous pancreas-kidney transplantation in patients with coexisting end-stage diabetic nephropathy, and less frequently as sequential pancreas after kidney transplantation or pancreas transplantation alone. In all modifications of pancreas transplantation the recipient’s native pancreas is left untouched. The first human pancreas transplantation was performed at the University of Minnesota in 1966 (Kelly et al. 1967). Since then, pancreas graft survival has improved consistently, especially in the last decade, thanks to refined surgical techniques and the introduction of better immunosuppressive regimens including tacrolimus and mycophenolate mofetil, which have decreased technical and immunological failure rates. In total, over 90% of pancreas transplantations are performed as simultaneous pancreas-kidney transplantations from the same donor, with the remaining cases classified as sequential pancreas after previous kidney transplantation, and rarely as pancreas transplantation alone. Today the international pancreas transplant registry reports a 1-year patient survival rate of greater than 95%, a 1-year pancreas graft survival rate of greater than 80% for simultaneous pancreas-kidney transplantation, and of nearly 80% for pancreas after previous kidney transplantation and pancreas transplantation alone (Gruessner and Sutherland 2001; International Pancreas Transplant Registry 2004). Although the majority of transplant centers formerly used exocrine bladder drainage to divert pancreatic juice, an increasing proportion of simultaneous pancreasukidney transplantations are being performed with the more physiologic enteric drainage and either systemic or portal endocrine drainage.