Abstract:
:Cystic fibrosis (CF) is a multiorgan disease caused by mutation of the CF transmembrane conductance regulator (CFTR) gene. Obstructive lung disease is the predominant cause of morbidity and mortality; thus, most efforts to improve outcomes are directed toward slowing or halting lung-disease progression. Current therapies, such as mucolytics, airway clearance techniques, bronchodilators, and antibiotics, aim to suppress airway inflammation and the processes that stimulate it, namely, retention and infection of mucus plaques at the airway surface. New approaches to therapy that aim to ameliorate specific CFTR mutations or mutational classes by restoring normal expression or function are being investigated. Because of its sensitivity in detecting changes associated with early airway obstruction and regional lung disease, high-resolution CT (HRCT) complements pulmonary function testing in defining disease natural history and measuring response to both conventional and experimental therapies. In this review, perspectives on the genetics and microbiology of CF provide a context for understanding the increasing importance of HRCT and other imaging techniques in assessing CF therapies.
journal_name
Pediatr Radioljournal_title
Pediatric radiologyauthors
Moskowitz SM,Gibson RL,Effmann ELdoi
10.1007/s00247-005-1445-3keywords:
subject
Has Abstractpub_date
2005-08-01 00:00:00pages
739-57issue
8eissn
0301-0449issn
1432-1998journal_volume
35pub_type
杂志文章,评审abstract:BACKGROUND:Motion remains a major limitation of fetal magnetic resonance imaging (MRI). Some centers have required mothers to fast before MRI in an effort to reduce motion; however, there is no current literature that describes the effect of maternal diet on fetal activity. OBJECTIVE:The objective is to define associa...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-018-4254-1
更新日期:2019-01-01 00:00:00
abstract::Developmental dysplasia of the hip (DDH) is an important but poorly defined entity, the natural history of which is incompletely understood. The term encompasses a disease spectrum ranging from a stable hip with a mildly dysplastic acetabulum to complete hip dislocation. Much controversy surrounds the diagnosis, imagi...
journal_title:Pediatric radiology
pub_type: 杂志文章,评审
doi:10.1007/s00247-012-2429-8
更新日期:2012-12-01 00:00:00
abstract:BACKGROUND:Infantile myofibromatosis is the most common fibrous tumor of infancy. It can involve the skin, muscle, bone, and viscera. This uncommon entity is subdivided into solitary and multicentric forms, with or without visceral involvement. OBJECTIVE:To describe the imaging characteristics of extracranial myofibro...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-004-1357-7
更新日期:2005-04-01 00:00:00
abstract:BACKGROUND:Data suggest an increased risk of thyroid dysfunction following iodine-based contrast material (IBCM) in children. OBJECTIVE:To estimate the prevalence of thyroid stimulating hormone (TSH) abnormalities following exposure to a single dose of intravenous IBCM during computed tomography (CT) in young children...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-020-04881-0
更新日期:2020-11-17 00:00:00
abstract::Treatment-related neurotoxicity is a potentially life-threatening clinical condition that can represent a diagnostic challenge. Differentiating diagnoses between therapy-associated brain injury and recurrent disease can be difficult, and the immediate recognition of neurotoxicity is crucial to providing correct therap...
journal_title:Pediatric radiology
pub_type: 杂志文章,评审
doi:10.1007/s00247-016-3750-4
更新日期:2017-05-01 00:00:00
abstract::Extracranial hematoma without significant head trauma is uncommon. We discuss a 9-year-old girl who presented with sudden head swelling, bilateral proptosis, extraocular muscle palsy, and progressive visual disturbance after hair braiding. The diagnosis of a large subgaleal hematoma with extension into the superior as...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-009-1176-y
更新日期:2009-06-01 00:00:00
abstract::Hepatocyte-specific contrast media are gadolinium chelates that are taken up by hepatocytes and partially cleared via the biliary tree. The absence of lesional uptake of the contrast media in the hepatobiliary phase is a marker of either the absence of hepatocytes or of poorly functioning, neoplastic hepatocytes. Upta...
journal_title:Pediatric radiology
pub_type: 杂志文章,评审
doi:10.1007/s00247-018-4108-x
更新日期:2018-08-01 00:00:00
abstract::We report a rare case of atypical teratoid/rhabdoid tumor that presented with imaging findings similar to those of optic pathway glioma. The diagnosis of atypical teratoid/rhabdoid tumor was determined following surgical resection of the tumor by collective histologic and immunohistochemical staining, and cytogenetic ...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-008-0923-9
更新日期:2008-10-01 00:00:00
abstract:BACKGROUND:Influenza A (H1N1) can cause severe neurological complications. OBJECTIVE:The purpose of this study was to analyze clinical and MRI features of neurological complications after H1N1 infection in critically ill children. MATERIALS AND METHODS:We retrospectively analyzed clinical and neuroimaging findings in...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-013-2682-5
更新日期:2013-09-01 00:00:00
abstract::Prenatal imaging with high-resolution US and rapid acquisition MRI plays a key role in the accurate diagnosis of congenital chest masses. Imaging has enhanced our understanding of the natural history of fetal lung masses, allowing for accurate prediction of outcome, parental counseling, and planning of pregnancy and n...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-011-2171-7
更新日期:2012-01-01 00:00:00
abstract:BACKGROUND:Late-onset noninfectious pulmonary complications (LONIPCs) are life-threatening complications of bone marrow transplantation (BMT). Several pathological patterns are described in the literature with different prognoses, and with different relationships to graft-versus-host disease (GVHD). The role of high-re...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-008-0984-9
更新日期:2008-11-01 00:00:00
abstract::The practice of contrast-enhanced ultrasound in children is in the setting of off-label use or research. The widespread practice of pediatric contrast-enhanced US is primarily in Europe. There is ongoing effort by the Society for Pediatric Radiology (SPR) and International Contrast Ultrasound Society (ICUS) to push fo...
journal_title:Pediatric radiology
pub_type: 杂志文章,评审
doi:10.1007/s00247-013-2746-6
更新日期:2013-09-01 00:00:00
abstract:BACKGROUND:Keutel syndrome is a rare autosomal-recessive condition characterized by abnormal cartilage calcification. Neuroimaging findings associated with this condition have been randomly described in the literature. OBJECTIVE:To systematically evaluate the neuroimaging findings in a series of children with Keutel s...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-013-2768-0
更新日期:2014-01-01 00:00:00
abstract::The arterial resting flow, the arterial peak flow after 3 minutes of circulatory arrest to the leg, and the venous emptying rate were studied by strain gauge plethysmography in the calves of 85 children between 3 and 16 years of age. Most of the children had heart disease; therefore the values obtained can be regarded...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/BF00973363
更新日期:1975-01-24 00:00:00
abstract::Antenatal detection of mesoblastic nephroma by US is possible. Reviewing the literature, we found 19 previously reported cases, only 1 of which underwent prenatal MRI. We present a further case diagnosed by US and confirmed with MRI. The imaging findings and differential diagnoses are discussed. Early and correct dete...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s002470050033
更新日期:2000-03-01 00:00:00
abstract::Two different forms of dyssegmental dysplasia, mild and severe, can be distinguished on clinical, radiographic and chondro-osseous morphologic grounds. The milder Rolland-Desbuquois type is frequently characterised by survival beyond the newborn period. The lethal Silverman-Handmaker type has distinctive and more seve...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s002470050279
更新日期:1997-12-01 00:00:00
abstract:BACKGROUND:Shielding of the gonads is a dose-saving strategy in pediatric radiography and its use is the law in New York and the majority of other states. However, routine use of gonadal shields is controversial because of concerns that important diagnostic information can be obscured. OBJECTIVE:The purpose of this st...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-013-2700-7
更新日期:2013-10-01 00:00:00
abstract:BACKGROUND:Pediatric optic pathway gliomas are typically indolent but have a variable clinical course. Treatment is dictated by symptoms and changes on contrast-enhanced MRI examinations. Gadolinium retention in children has motivated parsimonious use of gadolinium-based contrast agents. OBJECTIVES:To determine survei...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-018-4154-4
更新日期:2018-09-01 00:00:00
abstract::In this article, the fourth author's name is misspelled. The correct spelling, as shown above, should be "Nadia F. Mahmood." ...
journal_title:Pediatric radiology
pub_type: 杂志文章,已发布勘误
doi:10.1007/s00247-019-04343-2
更新日期:2019-03-01 00:00:00
abstract::Primary liver malignancies are rare in children. Hepatoblastoma and hepatocellular carcinoma (HCC) together represent the overwhelming majority of cases. Overall survival of hepatoblastoma approaches 80% with multimodal treatment approaches that include chemotherapy, surgery and transplantation. However, there remains...
journal_title:Pediatric radiology
pub_type: 杂志文章,评审
doi:10.1007/s00247-018-4068-1
更新日期:2018-04-01 00:00:00
abstract:BACKGROUND:Despite regulations, insufficient information is provided to adult patients prior to their radiologic examinations. Information regarding paediatric patients has not been systematically studied. OBJECTIVE:To survey parents' experience and wishes for information in connection with their child's radiographic ...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-018-4300-z
更新日期:2019-02-01 00:00:00
abstract:OBJECTIVE:To assess the value of head ultrasound (HUS) in neurologically normal infants with macrocephaly and to develop a rational approach to radiological investigation of macrocephalic infants. MATERIALS AND METHODS:A retrospective analysis of infants with macrocephaly (seen by one pediatric neurologist at this ins...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s002470050315
更新日期:1998-03-01 00:00:00
abstract::The detailed radiographic features of triploidy, a fatal congenital disorder with 69 chromosomes, have not previously been reported. Radiographs of ten liveborn infants with chromosomally confirmed triploidy showed six findings highly suggestive of this diagnosis: harlequin orbits, small anterior fontanelle, gracile r...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/BF02386842
更新日期:1989-01-01 00:00:00
abstract::In hypertrophic pyloric stenosis (HPS), prompt pyloromyotomy is, in general, the treatment of choice. There has been no information available as to the natural history of the pyloric tumour. We present four infants with medically treated HPS who were followed by sonography to observe the anatomical changes that occur ...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s002470050325
更新日期:1998-03-01 00:00:00
abstract:BACKGROUND:Pyeloplasty is an established treatment for pelviureteric junction (PUJ) obstruction. The postoperative change in the size of the renal pelvis and the kidney parenchyma are variable. OBJECTIVE:To document the changes in renal pelvic size and renal parenchymal thickness following pyeloplasty and to establish...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s002470050342
更新日期:1998-04-01 00:00:00
abstract::The need for X-ray investigations of the urinary tract at the time of the first proven urinary infection, even in older girls, is stressed. This policy is the best way to detect chronic pyelonephritis and congenital anomalies of the urinary tract and provide timely and appropriate treatment. These conclusions are supp...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/BF00972109
更新日期:1977-10-28 00:00:00
abstract::Drainage problems due to catheter malpositioning are acutely life-threatening in patients undergoing extracorporeal membrane oxygenation. In order to reduce these complications we introduced sonographically guided catheter positioning. We compare the outcome in a group of patients with blind cannula positioning to tha...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/BF02011838
更新日期:1995-01-01 00:00:00
abstract::Sonographic studies on 52 children with suspected peripheral vascular disease examined between January 1988 and December 1989 were retrospectively reviewed. A technique for examining the jugular, subclavian veins, and superior vena cava (SVC) was developed and is described. The technique for examining the extremities ...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/BF02011718
更新日期:1991-01-01 00:00:00
abstract:BACKGROUND:Methylmalonic acidemia (MMA) is an autosomal-recessive inborn error of metabolism. OBJECTIVE:To recognize the CT and MR brain sectional imaging findings in children with MMA. MATERIALS AND METHODS:Brain imaging studies (47 MR and 5 CT studies) from 52 children were reviewed and reported by a neuroradiologi...
journal_title:Pediatric radiology
pub_type: 杂志文章,评审
doi:10.1007/s00247-008-0940-8
更新日期:2008-10-01 00:00:00
abstract:BACKGROUND:There are few data regarding the frequency and type of diagnostic errors made by radiology residents and fellows ("trainees"). However, increasing interest in reducing medical errors highlights the need to analyze which areas of medical knowledge are most problematic for physicians-in-training, including rad...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-004-1150-7
更新日期:2004-04-01 00:00:00