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  • 1
    ISSN: 1432-1076
    Keywords: Lysinuric protein intolerance ; Erythroblastophagocytosis ; Interstitial lung disease ; Renal disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three patients with lysinuric protein intolerance are reported. The first patient displayed severe haemolytic anaemia, bone marrow erythroblastophagocytosis, renal tubular disease and interstitial lung disease. Despite treatment with citrulline and low-protein diet, this child died at the age of 18 months. The second patient is now 24 years old and has chronic interstitial lung disease and focal renal glomerulosclerosis. The third patient, now 5 years old, has severe chronic interstitial lung disease. A 6-month treatment with prednisone was ineffective in the second and third patients.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Neuroradiology 31 (1990), S. 512-522 
    ISSN: 1432-1920
    Keywords: Occult spinal dysraphism ; Lipoma ; Tethered cord ; Spinal cord ; Caudal regression syndrome ; Developmental mass lesions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We present a retrospective study of occult spinal dysraphism in 47 children aged 0 to 14 years, all studied with plain X-rays, 60% with CT and myelo-CT, and 40% with MR. We consider the classification and grading of these malformations, clinical, neuroradiological patterns, and indications for surgery. In the light of our findings and of the published data MR emerges as the key investigation. Only in a few cases of great anatomical complexity is it now necessary to perform CT and myelo-CT as well. A case in point is when the conus and thickened filum terminale are inextricably bound together and can no longer be considered separate structures. We propose the term “neurofibrous structure” to define the conus-thickened-filum-terminale unit when these structure are no longer distinguishable.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Isolated oesophageal leiomyomatosis in children is very rare. The association between leiomyomatosis of oesophagus and female genital tract and an Alport like hematuric nephritis has been recognized as a distinct entity by Garcia-Torres and Guarner [1]. Since then few other cases of this syndrome have been observed. Congenital cataracts and neurosensory deafness have been added to the clinical spectrum. Autosomal dominant inheritance is suggested by the familial cases. We report a patient who presents, as a new finding, leiomyomatosis of the rectum.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 23 (1993), S. 202-203 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A case of vein of Galen aneurysm, studied with a fast gradient refocusing pulse MRI sequence, is presented. Fast MRI sequences allowed the acquisition of additional hemodynamic information that was not available with either routine MRI or angiography.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cranial CT and/or MRI imaging of 8 patients with mucopolysaccharidosis (MPS) was retrospectively evaluated. Two patients had MPS IH, 1 had MPS IS, 1 had MPS IVA and 4 had MPS IV. CT and MRI showed thickening of dura mater at the cranio-cervical junction, causing narrowing of the subarachnoid space, in all the patients examined. Spinal cord compression was detected in 4 patients. Other findings were: white matter alterations, mild to severe hydrocephalus, skull dysplasia and odontoid dysplasia. White matter alterations were evident as large areas and as multiple dispersed spots of prolonged T1 and T2 value. Reduced gray/white matter contrast was demonstrated on T2-weighted MRI images. It is important to examine the cranio-cervical junction carefully for thickening of dura mater in all patients with mucopolysaccharidosis examined by CT or MRI, because of the generally progressive clinical course of MPS. In patients with symptomatic cord compression, surgical intervention should be considered.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on a 8-month-old boy with AIDS, born of an asymptomatic mother with positive HTLV-III serology. He was hospitalized in the Intensive Care Unit because of anemia, fever and hepatosplenomegaly. Chest X-ray showed pneumonia and subsequent blood cultures were positive for Candida albicans. After 3 days of Amphotericine B treatment, the patient was transferred to Infectious Disease Department. After 30 days of hospitalization, the patient developed a rapid neurological impairment evolving into coma. CT scan showed a round, ringshaped low density lesion with hypershaped low density lesion with hyper-dense and enhancing haemorragic centre in the left basal ganglia and a smaller hypodense lesion on the right. There was also evidence of cortical atrophy and mild ventricular dilatation. Such lesions are more commonly described in children with AIDS and congenital cytomegalic inclusion virus (CMV) encephalitis. In this case toxoplasma cysts were shown microscopically reinforcing the contention that in patients with AIDS, toxoplasma gondii infection may occur with atypical manifestation.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 23 (1993), S. 177-180 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In treating cases of malignancy, the use of chemotherapy carries a high risk of lower respiratory tract infections, especially fungal pneumonopathy. This complication is a major cause of mortality and is often difficult to diagnose because of non-specific clinical or radiological changes, but the early recognition of invasive fungal disease is imperative. CT is an important non-invasive method for the detection and evaluation of opportunistic fungal infections. In these patients an improved survival rate can be achieved when early detection by CT leads to the prompt institution of high-dose antifungal therapy. We illustrate the spectrum of CT findings of invasive pulmonary aspergillosis encountered in children with cancer. These patients had previously been treated with highdose chemotherapy with or without bone marrow rescue, and underwent radiological examinations because of clinical evidence of pneumonopathy. Representative cases demonstrate the clinical applications of CT in the evaluation and management of invasive fungal disease.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In this study of 14 patients with anorectal anomalies CT and MRI were employed for preoperative assessment. The use of a pressure enhanced water soluble enema via the colostomy proved to be an extremely efficient method for showing a fistula. MRI studies were enhanced by the use of vaseline oil and in one case this technique was used prior to surgery to provide important information by injecting through a perineal fistula. CT and axial MRI proved to be more valuable than sagittal MRI which is only useful for the length of the atretic segment. The authors consider that a combined approach using pressure enhanced water soluble enema and MRI will provide the most valuable preoperative information to plan a successful operative approach and enable an accurate prognostic evaluation of continence in these difficult and complex 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 Intraabdominal calcifications are an unusual radiographic finding in the newborn and can easily be misinterpreted as meconium peritonitis. The authors refer to a patient with anorectal malformation (ARM), intraluminal calcifications and other anomalies. Careful differentiation of the type of intraabdominal calcifications can suggest the possibility of other initially unsuspected anomalies.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Relapsing polychondritis is very rare in children. The diagnosis must be based on a combination of clinical and pathologic features. CT is very useful for an accurate and rapid assessment of laryngo-tracheo-bronchial involvement and the typical finding is lumen narrowing by wall thickening and collapse of the supporting cartilaginous structures. The role of MR imaging should be complementary to CT.
    Type of Medium: Electronic Resource
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