Abstract：
BACKGROUND:Urea cycle disorders encompass several enzyme deficiencies that can result in cerebral damage, with a wide clinical spectrum from asymptomatic to severe. OBJECTIVE:The goal of this study was to correlate brain MRI abnormalities in urea cycle disorders with clinical neurological sequelae to evaluate whether MRI abnormalities can assist in guiding difficult treatment decisions. MATERIALS AND METHODS:We performed a retrospective chart review of patients with urea cycle disorders and symptomatic hyperammonemia. Brain MRI images were reviewed for abnormalities that correlated with severity of clinical neurological sequelae. RESULTS:Our case series comprises six urea cycle disorder patients, five with ornithine transcarbamylase deficiency and one with citrullinemia type 1. The observed trend in distribution of brain MRI abnormalities as the severity of neurological sequelae increased was the peri-insular region first, extending into the frontal, parietal, temporal and, finally, the occipital lobes. There was thalamic restricted diffusion in three children with prolonged hyperammonemia. Prior to death, this site is typically reported to be spared in urea cycle disorders. CONCLUSION:The pattern and extent of brain MRI abnormalities correlate with clinical neurological outcome in our case series. This suggests that brain MRI abnormalities may assist in determining prognosis and helping clinicians with subsequent treatment decisions.
journal_name
Pediatr Radioljournal_title
Pediatric radiologyauthors
Bireley WR,Van Hove JL,Gallagher RC,Fenton LZdoi
10.1007/s00247-011-2253-6subject
Has Abstractpub_date
2012-04-01 00:00:00pages
455-62issue
4eissn
0301-0449issn
1432-1998journal_volume
42pub_type
杂志文章abstract：:Details of a female infant, who was born after 29 weeks gestation and who died within minutes of birth, are presented. The infant was hydropic, showed macroglossia and had very short limbs with normal sized hands and feet. Apart from a preductal aortic coarctation the pathological findings were confined to the skeleto...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF02388725
更新日期：1985-01-01 00:00:00
abstract：:A case of duodenal diaphragm diagnosed by ultrasound is presented. The case was associated with situs inversus. ...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF02388285
更新日期：1987-01-01 00:00:00
abstract：:The case of a 12 year old girl with a hamartoma of the liver is reported. A preoperative liver scan showed accumulation of technetium-99m sulfur colloid in the tumor with isotopic and contrast arteriography demonstration of a hypervascular intrahepatic mass. Although these diagnostic findings are not pathognomonic of ...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF00975347
更新日期：1976-04-14 00:00:00
abstract：:A 4-13 year follow up study of 29 patients with juvenile rheumatoid arthritis into adulthood revealed several structural deformities of the hips: coxa magna, short femoral neck, subluxations associated with large cyst-like erosions of femur and acetabulum near the ligamentum teres and a flattened, wide femoral head (w...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF01002300
更新日期：1984-01-01 00:00:00
abstract：:The use of a central venous catheter (CVC) has become commonplace in the care of children with a wide variety of medical and surgical problems. Complications resulting from the insertion of these catheters are well recognized and can be life-threatening. When a temporary CVC or other catheter is inserted into the cent...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-007-0674-z
更新日期：2008-03-01 00:00:00
abstract：:Radiation-induced injuries from fluoroscopic procedures in pediatric patients have occurred, and young patients are at greatest risk of many radiation-induced neoplasms. Some fluoroscopists have been injured from their use of fluoroscopy, and they are known to be at risk of radiation-induced neoplasm when radiation is...
journal_title：Pediatric radiology
pub_type： 杂志文章,评审
doi：10.1007/s00247-006-0187-1
更新日期：2006-09-01 00:00:00
abstract：OBJECTIVE:The purpose of this paper is to review and categorize the CT appearances of primary and secondary intraperitoneal neoplasms in children. MATERIALS AND METHODS:We retrospectively reviewed the CT images of 14 cases of intraperitoneal neoplasms. They were reviewed for bowel wall thickening, mesenteric nodules, ...
journal_title：Pediatric radiology
pub_type： 杂志文章,多中心研究
doi：10.1007/s002470050363
更新日期：1998-05-01 00:00:00
abstract：:The original version of this article contained an error. Under the heading 'Limitations', the sentence 'Another limitation was the decision to exclude one reader from interobserver analysis. ...
journal_title：Pediatric radiology
pub_type： 杂志文章,已发布勘误
doi：10.1007/s00247-018-4219-4
更新日期：2018-10-01 00:00:00
abstract：:The relationship between image noise and radiation dose was investigated in computed tomography (CT) images of a pediatric abdomen phantom. A protocol which provided a minimum absorbed dose consistent with acceptable image noise criteria was determined for a fourth generation CT scanner. It was found that pediatric ab...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF02387506
更新日期：1986-01-01 00:00:00
abstract：:Spontaneous reduction of a presumed ileo-colonic intussusception was demonstrated by ultrasound examination followed by a barium enema. No premedication or anesthetic had been given to the child. This case illustrates the possible natural history of intussusception rarely confirmed by imaging studies. Intussusception ...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF02013178
更新日期：1990-01-01 00:00:00
abstract：:Congenital solitary nonparasitic cysts of the liver (CSNCL) are extremely rare causes of abdominal masses in newborns and infants. We describe a cystic abdominal mass virtually all extrahepatic in a newborn that proved to be a CSNCL. ...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF02013009
更新日期：1992-01-01 00:00:00
abstract：BACKGROUND:It has been suggested that increased hepatic and intramuscular fat is associated with insulin resistance, and that increased pancreatic fat is related to impaired insulin secretion. OBJECTIVE:We postulated that in obese nondiabetic teenagers insulin levels would be directly related to increases in intramusc...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-005-1413-y
更新日期：2005-06-01 00:00:00
abstract：:A case is presented in which chronic renal failure led to intense visceral calcification, mainly to the lungs and heart. The discovery of cardiac calcifications on plain chest radiographs is exceedingly rare in renal patients. Punctate calcific deposits with an almost homogeneous distribution throughout the cardiac mu...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF02386824
更新日期：1986-01-01 00:00:00
abstract：:Gorham's disease may develop in any region of the skeleton, but shows a predilection for the bones of the shoulders and pelvic girdles. Less frequently, the disease may involve the spine, and it is the proximity to the spinal cord which may worsen the patient's outcome. We report the clinical outcomes of two children ...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF02012113
更新日期：1994-01-01 00:00:00
abstract：:Radiographic differentiation between cystic fibrosis and asthma presenting in teenagers and young adults can be difficult. Many patients with a late presentation of cystic fibrosis display minimal changes on a chest radiograph. However, a large majority (90%) of cystic fibrosis patients with an essentially normal PA c...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF02388758
更新日期：1985-01-01 00:00:00
abstract：:A case of the Hajdu-Cheney syndrome in a 15 year old boy is described. The manifestations were: 1. isiopathic progressive acro-osteotions; 2. mutiple cranio-skeletal abnormalities; and 3. polycystic kidneys complicated by a rapidly progressive glomerulonephritis. ...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF00973535
更新日期：1977-09-01 00:00:00
abstract：OBJECTIVE:To evaluate the effectiveness of lateral decubitus high-resolution CT (HRCT) in detecting air trapping in children. MATERIALS AND METHODS:HRCT scans of 21 children with heterogeneous lung attenuation caused by air trapping (n = 10) or with infiltrative lung disease (n = 11) were reviewed retrospectively. The...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-001-0622-2
更新日期：2002-03-01 00:00:00
abstract：BACKGROUND:Our center has developed a multidisciplinary approach to percutaneous endovascular thrombolysis with the goal of improving outcomes in children with thrombosis. There is little data describing the safety and efficacy of endovascular thrombolysis and the frequency of post-thrombotic syndrome after thrombolysi...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-014-3209-4
更新日期：2015-04-01 00:00:00
abstract：BACKGROUND:Typical flow velocity profiles in the extraparenchymal pulmonary veins (PVs) demonstrate two major antegrade flow waves: a biphasic systolic wave (S), with S1 and S2 peaks and a monophasic early diastolic wave (D). Flow reversal during atrial systole (A) is common. There is agreement that the forward diastol...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-011-2227-8
更新日期：2012-02-01 00:00:00
abstract：BACKGROUND:Recently a 320-detector-row CT (MDCT) scanner has become available that allows axial volumetric scanning of a 16-cm-long range (50 cm field of view) in a single 0.35-s rotation. For imaging neonates and small children, volume scanning is potentially of great advantage as the entire scan range can be acquired...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-009-1436-x
更新日期：2010-03-01 00:00:00
abstract：:An infant with complex cyanotic congenital heart disease was recently encountered whose radiographs seemed to show enhancement of pericardium, peritoneal mesothelium and body wall fascial planes without enhancement of the liver or spleen after very large doses of intravenous contrast. Although patterns of postcontrast...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-011-2249-2
更新日期：2012-06-01 00:00:00
abstract：BACKGROUND:Image-guided percutaneous core needle biopsy (PCNB) of soft-tissue lesions is a minimally invasive technique that can provide a definitive diagnosis for treatment. Anatomical and age considerations in children require judicious use of safe percutaneous biopsy techniques. There are, however, limited data on t...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-015-3364-2
更新日期：2015-09-01 00:00:00
abstract：BACKGROUND:Resistivity index (RI) of the pericallosal artery as is commonly measured during head ultrasound (US) examination in neonates. Some studies have shown that RI measured with gentle compression of the fontanelle provides additional information in cases of neonatal brain anomalies. OBJECTIVE:The purpose of thi...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-019-04347-y
更新日期：2019-05-01 00:00:00
abstract：BACKGROUND:Pleural effusion at presentation in Hodgkin lymphoma has been associated with inferior outcome but has not been systematically evaluated. OBJECTIVE:To determine whether pleural effusion at presentation in children with Hodgkin lymphoma is a primary indicator of poor prognosis or secondary to associated fact...
journal_title：Pediatric radiology
pub_type： 杂志文章,随机对照试验
doi：10.1007/s00247-018-4197-6
更新日期：2018-11-01 00:00:00
abstract：:Ectopic thyroid gland is a well-known developmental anomaly. We present a child with a unique anatomical variant of this anomaly, namely double ectopic thyroid. The purpose of this report is to demonstrate the scintigraphic findings and to emphasize the importance of performing a thyroid scan in every case of thyroid ...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-002-0787-3
更新日期：2002-12-01 00:00:00
abstract：BACKGROUND:Paediatric non-accidental injury is a considerable health problem, and imaging plays a fundamental role in its assessment. Since the introduction of joint guidelines published by the Royal College of Radiologists (RCR) and Royal College of Paediatrics and Child Health (RCPCH) in 2008, there has been a concer...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-014-3063-4
更新日期：2014-12-01 00:00:00
abstract：BACKGROUND:Takayasu's arteritis (TA) is a chronic idiopathic inflammatory disease affecting primarily the aorta, its proximal branches and the pulmonary arteries. OBJECTIVES:To retrospectively review the angiograms of children with TA so as to describe the patterns of vascular involvement. PATIENTS AND METHODS:Twenty...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-002-0817-1
更新日期：2003-04-01 00:00:00
abstract：:Foreign bodies, whether ingested, aspirated or retained in the soft tissues, are a particular hazard to pediatric patients. Ornamentation associated with the winter holidays is an uncommon source of foreign bodies in children, and many of these foreign bodies have a distinct appearance on imaging. Knowledge of these a...
journal_title：Pediatric radiology
pub_type： 杂志文章,评审
doi：10.1007/s00247-014-3192-9
更新日期：2014-12-01 00:00:00
abstract：:In a group of 70 premature and newborn infants, treated with artificial ventilation, 24.3% developed bronchopulmonary dysplasia (BPD). Only in a very few cases did the typical radiological stages, as described by Northway, succeed each other in a chronological order. It is impossible to differentiate BPD stage I or II...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/BF00973978
更新日期：1977-03-17 00:00:00
abstract：BACKGROUND:Inferior vena cava (IVC) filter placement in children has been described in literature, but there is variability with regard to their indications. No nationally representative study has been done to compare practice patterns of filter placements at adult and children's hospitals. OBJECTIVE:To perform a nati...
journal_title：Pediatric radiology
pub_type： 杂志文章
doi：10.1007/s00247-017-4001-z
更新日期：2018-02-01 00:00:00
