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  • 1
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  Effective treatment of haemophilic arthropathy requires a detailed evaluation of joint integrity. Methodological assessment of magnetic resonance imaging (MRI) scores are needed to assure reproducibility of measurements when comparing results of clinical trials conducted in different centres. We compared the reliability of two MRI scoring systems for assessment of haemophilic arthropathy: one progressive system that displays the most severe change and one additive system that depicts osteochondral and soft tissue-related changes. A total of 47 1.5 T MRI examinations of knees (n = 21) and ankles (n = 26) of 42 haemophilic boys, age range, 22 months to 18 years, performed at different centres (Toronto, n = 20, Europe, n = 12 and Denver, n = 15) were independently reviewed by four radiologists at two occasions. Twenty-two examinations were from children 〈9 years and 25 from children ≥9. Sagittal and coronal gradient-echo (MPGR, 3D FLASH with fat saturation, GRASS) images were obtained. The MRI examinations of the ankle and knee studies presented with osteochondral abnormalities in 38.5% and 23.8% of the cases respectively. The two scoring systems demonstrated an excellent inter-reader [progressive, 0.88; additive (A, e, s and h components), 0.86] and intra-reader [progressive, 0.92; additive (A, e, s and h components), 0.93] reliability using intraclass correlation coefficients (ICCs). Although ICCs were slightly higher for knees when compared with ankles, and for older children when compared with younger children, all values fell within excellent inter- and intra-reader reliability categories. The two MRI scoring systems demonstrated a comparable reliability. This result constitutes the basis for further development of a combined MRI scoring system for assessment of haemophilic arthropathy, which incorporates progressive and additive components.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  Routine infusions of factor VIII to prevent bleeding, known as prophylaxis, and other intensive therapies are being more broadly applied to patients with haemophilia. These therapies differ widely in replacement product usage, cost, frequency of venous access and parental effort. In order to address residual issues relating to recommendations, implementation, and evaluations of prophylaxis therapy in persons with haemophila, a multinational working group was formed and called the International Prophylaxis Study Group (IPSG). The group was comprised of haemophilia treaters actively involved in studies of prophylaxis from North America and Europe. Two expert committees, the Physical Therapy (PT) Working Group and the Magnetic Resonance Imaging (MRI) Working Group were organized to critically assess existing tools for assessment of joint outcome. These two committees independently concluded that the WFH Physical Examination Scale (WFH PE Scale) and the WFH X-ray Scale (WFH XR Scale) were inadequately sensitive to detect early changes in joints. New scales were developed based on suggested modifications of the existing scales and called the Haemophilia Joint Health Score (HJHS) and the International MRI Scales. The new scales were piloted. Concordance was measured by the intra-class correlation coefficient of variation. Reliability of the HJHS was excellent with an inter-observer co-efficient of 0.83 and a test-retest value of 0.89. The MRI study was conducted using both Denver and European scoring approaches; inter-reader reliability using the two approaches was 0.88 and 0.87; test-retest reliability was 0.92 and 0.93. These new PT and MRI scales promise to improve outcome assessment in children on early preventive treatment regimens.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 30 (2000), S. 262-264 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Mucolipidosis type III (ML-III) is a lysosomal storage disease often presenting with joint involvement. We report the MRI appearance of the hips in two siblings with ML-III showing abnormal signal intensity within the hips with increased synovial thickness. Although the etiology is uncertain this may reflect a fibrous response to ML-III.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 30 (2000), S. 546-550 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Purpose. To evaluate the utility of routine follow-up computed tomography (CT) and/or ultrasound (US) scans in children with blunt hepatic trauma initially managed non-operatively. Materials and methods. Review of the records of 66 children with proven blunt liver injury on initial CT scan, who were initially managed non-operatively during the period January 1991 to December 1996. Follow-up CT and US studies were analyzed and correlated with clinical outcome. Results. Of the 66 children, 30 were not followed with any imaging study, 26 were followed with US only, 7 with CT only and 3 with US and CT. Disappearance of the liver lesion(s) was seen in 25 patients (range: 6 days – 14 months) and decrease in size was noted in 10. In one patient, who developed abdominal and right shoulder pain 10 days after presentation with subsequent hemoglobin drop, CT showed contrast medium extravasation into a hepatic hematoma from portal vein injury that required surgery. Conclusion. Our series suggests that in asymptomatic patients, US and CT follow-up studies do not provide the additional information needed for patient management. Therefore, we believe that in asymptomatic children with blunt hepatic trauma who are clinically stable, routine follow-up imaging studies are of very limited value.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 30 (2000), S. 471-479 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Objectives. Traumatic diaphragmatic injuries (DI) in infants and children are uncommon and are often associated with multiple severe injuries. Delayed presentation can be life threatening due to organ herniation and strangulation. We present the imaging findings in a relatively large population of children who experienced this rare injury.¶Methods. Medical records of all patients admitted to our Trauma Service from 1977 to 1998 with DI were retrospectively reviewed recording imaging, clinical and surgical or autopsy findings.¶Results. Of sixteen patients with DI (7 females, 9 males; age 3 weeks to 15 years), 14 suffered from blunt trauma secondary to high-energy impact, and 2 from penetrating injuries. Unilateral DI occurred equally on each side, with one bilateral injury. Associated injuries, present in 81 %, included severe head injuries, visceral, mesenteric and vascular injuries and multiple fractures. Six patients died from multiple organ failure (3), head injury (2), and shock (1). Findings in the initial chest X-ray suggested the diagnosis in 13 (81 %) of 16 injuries, and CT demonstrated irregularity and thickening of the diaphragm in 4 out of 7.¶Conclusions. Plain film findings suggested the diagnosis in most; CT and MR were useful adjuncts. High index of suspicion and awareness of the mechanism of injury can lead to prompt diagnosis, early repair, and decreased morbidity and mortality.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 23 (1993), S. 341-344 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The purpose of this study was to compare the concurrent use of chloral hydrate (CH), intramuscular Nembutal (IMN) and intravenous Nembutal (IVN) for sedation. Data was collected on all pediatric outpatients requiring sedation for CT over a 2.5-year period. During this period, 2178 outpatients required sedation of whom, 1324 (60.8%) received IMN, 710 (32.6%) IVN and 110 (5%) CH. The overall success rate was 97% and was similar in all three groups. IVN however, produced betterquality sedation than IMN or CH. The IVN group received a significantly lower dose of Nembutal than the IMN group (p=0.001). Patients receiving IVN had a significantly lower induction time (p=0.0001) and total examination time (p=0.001) than IMN or CH. There was an increased occurrence of desaturation in patients sedated with IVN, especially in those with airway obstruction. IVN sedation permits faster, more efficient and better-quality sedation for outpatient CT scanning than IMN or CH in patients requiring intravenous contrast but may not be appropriate for departments that perform relatively few sedations or lack proper resuscitation facilities.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 23 (1993), S. 1-5 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract While trauma is still the leading cause of death in the pediatric age range, it is surprising how little the CT appearances of pediatric chest injury have been investigated in the literature. We have reviewed the CT findings of blunt chest trauma in 44 children for whom chest CT examinations were requested to investigate the extent of intrathoracic injury. We noted a propensity for pulmonary contusions to be located posteriorly or posteromedially, and for them to be anatomically nonsegmental and crescentic in shape. This is possibly attributable to the relatively compliant anterior chest wall in children. The CT appearances of other major thoracic injuries are described, including pulmonary lacerations, pneumothoraces, malpositioned chest tubes, mediastinal hematomas, aortic injury, tracheobronchial injury, hemopericardium, and spinal injuries with paraspinal fluid collections. Children demonstrating findings incidental to the actual injury yet important to the subsequent therapy are also presented. We conclude that, in the event of clinically significant blunt chest trauma, the single supine chest examination in the trauma room is insufficient to adequately identify the extent of intrathoracic injury. With the exception of concern for aortic injury for which aortography is indicated, a dynamically enhanced CT scan of the thorax should be performed as clinically significant findings may result in altered therapy.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 23 (1993), S. 525-528 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A new constellation of spinal changes are observed in homozygous beta-thalassemia major (HBT) patients receiving deferoxamine (DF), an iron-chelating drug used in combination with transfusion therapy in certain anemic syndromes. In a retrospective study of 22 HBT patients who were receiving DF therapy, morphological deformities (decreased spinal height, increased thoracic kyphosis, vertebral flattening and elongation anteriorly, and disk calcification) were found in 16 of 22 patients. These changes are believed to be caused by interference with spinal growth-plate development. Investigation of DF-dose correlation supports the conclusion that the spinal changes were DF-induced. Spinal changes observed in DF-treated patients differ both morphologically and pathogenetically from earlier reprots of vertebral deformities occurring as a sequel to compensatory marrow hyperplasia in poorly transfused patients.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Progressive noninfectious anterior vertebral fusion (PNAF) is a rare but well-defined entity of unknown cause. We report the MRI findings in a 5-year-old in whom PNAF was associated with a lipoma of the filum terminale.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 23 (1993), S. 597-600 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Metastatic choriocarcinoma is unusual in infancy and considered to be due to metastases from the placenta. Only fifteen cases have been previously reported. We present an unusual case of hepatic, pulmonary, and axillary lymph node metastases from choriocarcinoma in a 3-month-old female. Several of the lesions had large vascular channels identified by computed tomography and sonography with color and duplex Doppler.
    Type of Medium: Electronic Resource
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