Lung Transplantation for Primary Ciliary Dyskinesia and Kartagener Syndrome: A Multicenter Study.

Primary ciliary dyskinesia, with or without situs abnormalities, is a rare lung disease that can lead to an irreversible lung damage that may progress to respiratory failure. Lung transplant can be considered in end-stage disease. This study describes the outcomes of the largest lung transplant population for PCD and for PCD with situs abnormalities, also identified as Kartagener's syndrome. Retrospectively collected data of 36 patients who underwent lung transplantation for PCD from 1995 to 2020 with or without SA as part of the European Society of Thoracic Surgeons Lung Transplantation Working Group on rare diseases. Primary outcomes of interest included survival and freedom from chronic lung allograft dysfunction. Secondary outcomes included primary graft dysfunction within 72 h and the rate of rejection ≥A2 within the first year. Among PCD recipients with and without SA, the mean overall and CLAD-free survival were 5.9 and 5.2 years with no significant differences between groups in terms of time to CLAD (HR: 0.92, 95% CI: 0.27-3.14, p = 0.894) or mortality (HR: 0.45, 95% CI: 0.14-1.43, p = 0.178). Postoperative rates of PGD were comparable between groups; rejection grades ≥A2 on first biopsy or within the first year was more common in patients with SA. This study provides a valuable insight on international practices of lung transplantation in patients with PCD. Lung transplantation is an acceptable treatment option in this population

Pediatric abdominal applications of multidetector-row CT

The introduction of multidetector technology of computed tomography (CT) into clinical practice has increased using CT as modality of choice for most body parts. In spite of some disadvantages of CT such as radiation exposure and using iodinated contrast medium, some facilities gathered by multidetector computed tomography (MDCT) such as faster scanning time and high-resolution imaging technique have improved pediatric CT applications. Less using intravenous and oral contrast medium, less sedation rate, decreased radiation exposure are very practical advantages of MDCT in abdominal imaging of pediatric population. In this review, technical details of these advantages of MDCT for pediatric population and some examples of improved imaging and diagnostic capabilities arising from MDCT for pediatric abdominal applications will be presented. (C) 2004 Published by Elsevier Ireland Ltd

Multidetector row CT of the kidneys

In this article, the value of multiple detector-row computed tomography (MDCT) in renal applications is discussed, with emphasis on clinical uses. The superiority of application techniques of MDCT renders it one of the most distinguished modality in detection of congenital anomalies, tumors, transplants, trauma, and arterial diseases of the kidney. (C) 2004 Elsevier Ireland Ltd. All rights reserved

Complete bronchial rupture in a child: report of a case

Tracheobronchial rupture due to blunt chest trauma is a rare and serious injury in children. The diagnosis is usually difficult and may be overlooked because of the variability of symptoms and findings. Fiberendoscopy is useful in children with stable tracheal or bronchial ruptures. However, in the emergency situation, fiberendoscopy may not be appropriate, and thoracotomy and primary anastomosis may be the best option

Acute torsion of a wandering spleen: imaging findings

Wandering spleen is a rare entity characterized by incomplete fixation of the spleen by lienorenal and gastrosplenic ligaments. It can migrate to the lower abdomen or pelvis and can be congenital or acquired. We report a case of torsion of a wandering spleen for which there was correlative imaging by ultrasonography, Doppler ultrasonography, computed tomography, magnetic resonance imaging, and angiography. To our knowledge, this is the first reported case in which all these modalities were used in the diagnostic evaluation