AIM Oral rehabilitation of a child with Johanson-Blizzard syndrome (JBS). BACKGROUND JBS is an extremely rare inherited disorder characterized by unusually small nose that appears 'beak shaped' due to the absence (aplasia) or underdevelopment (hypoplasia) of the nostrils (nasal alae), abnormally small, malformed primary (deciduous) teeth and misshapen or absent secondary (permanent) teeth, he...

INTRODUCTION PU is an option to manage complex and/or recurrent urethral strictures and is necessary after urethrectomy and/or penectomy. PU is generally assumed to be the last option before abandoning the urethral outlet. METHOD Between 2001 and 2013, 51 patients underwent PU. Mean age (± standard deviation) was 60 ± 15 years. Only 13 patients (25.5%) did not undergo previous urethral interv...

BACKGROUND Early diagnosis of ventilator-associated pneumonia (VAP) is necessary to reduce morbidity and improve survival of critically ill patients in the ICU. The purpose of the present study is to examine the performance of macroscopic bronchoscopic findings and cytological analysis of bronchoalveolar lavage fluid (BALF) as an early diagnostic tool for VAP, either alone or in combination wit...

Éva Á. Csató, Gunilla Gren-Eklund, Lars Johanson, Birsel Karakoç (eds): Turcologica Upsaliensia: An Illustrated Collection of Essays. Leiden and Boston: Brill 2020. 267 pages, illustrated, with a fold-out map. Hardback €119.00. ISBN 978-90-04-43570-4.

1. Andersson U., Forsgren M., Holm U. [2015], Balancing Subsidiary Influence in the Federative MNC: A Business Network View, in: M. Forsgren, Holm, J. Johanson (eds.), Knowledge, Networks and Power, Palgrave Macmillan, London. Google Scholar

CONTEXT Johanson-Blizzard syndrome (JBS) is a rare autosomal recessive syndrome characterized by dysmorphic nasal alae, ectodermal abnormalities, exocrine pancreatic insufficiency and early growth failure. Most patients are diagnosed by clinical criteria prenatally or in early infancy. Nonsense, frame shift and splice-site mutations of the ubiquitin ligase gene (UBR1) lead to early loss of acin...