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  • 1
    ISSN: 1432-0533
    Keywords: Key words Creutzfeldt-Jakob disease ; New variant ; Neuropathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Clinical data and autopsy findings in a case ¶of new variant Creutzfeldt-Jakob disease (vCJD) are reported. this case, the first histologically confirmed case described outside the United Kingdom, very much resembles the cases described by Will et al. [(1996) Lancet 347 : 921–925] and Zeidler et al. [(1997) Lancet 350 : 903–908, 908–910]. Neuropathological studies failed to reveal any conspicuous clues that could be relevant for understanding the pathophysiology of the disease. For epidemiological surveillance, neuropathologists should scrutinize suspected cases keeping in mind the possibility of vCJD.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1573-2568
    Keywords: CHRONIC INTESTINAL PSEUDOOBSTRUCTION ; CHILDREN ; SURGERY ; NUTRITIONAL SUPPORT
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Our aim was to collect a large number of casesto characterize clinical presentation, outcome, andprognosis of chronic intestinal pseusoobstruction inchildren. We conducted a retrospective multicenter study that included children treated forchronic intestinal pseusoobstruction defined asrecurrent episodes of intestinal obstruction with nomechanical obstruction, excluding Hirschsprung'sdisease. In all, 105 children, 57 boys and 48 girls, werestudied, including five familial forms. Prenataldiagnosis was made in 18 patients. Eighty patients wereless than 12 months old at onset; the disease began at birth for 37 patients. The most frequentsigns were abdominal distension, vomiting, andconstipation. Megacystis was noted in myopathies (7cases), neuropathies (10 cases) and unclassified forms(13 cases). For all but three cases (two patientswith CMV infection, one with Munchhausen-by-proxysyndrome), the associated diseases and disorders couldnot account for chronic intestinal pseusoobstruction as a secondary disorder. At least onefull-thickness biopsy from the digestive tract wasstudied for 99 patients. The diagnosis recorded wasvisceral neuropathy in 58 cases, visceral myopathy in 17cases, and uncertain or normal biopsy results in 24cases. Seventy-eight children were fed intravenously,and only 18 were able to be fed orally throughout theirillness. Seventy-one patients underwent surgery during their illness, and 217 surgicalprocedures, a mean of 3 per patient, were performed.Ostomy was the most performed procedure. Follow-upcontinued in 89 patients for 3 months to 16 years (mean85 months). Forty-two patients were still fed byparenteral (39 patients) or enteral nutrition (3patients) at the time of the study. Eleven patients diedbetween the age of 1 month and 14 years 7months.
    Type of Medium: Electronic Resource
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