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  • 1
    Electronic Resource
    Electronic Resource
    An atypical paediatric case of malignant atrophic papulosis (Köhlmeier-Degos disease) (1990)
    Springer
    European journal of pediatrics 149 (1990), S. 457-458 
    Details
    ISSN: 1432-1076
    Keywords: Occlusive angiopathy ; Malignant atrophic papulosis ; Köhlmeier ; Degos disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A new case of malignant atrophic papulosis (Köhlmeier-Degos disease) is reported. Vascular symptoms began at 17 months of age with cerebral ischaemia and progressive involvement of fingers and toes with torpid ulcers and apical necrotic amputations. At 6 years of age he developed chronic intestinal ischaemia with malabsorption and a new cerebral attack; in spite of anti-aggregant therapy the disease progressed and he died 7 months after diagnosis from a third cerebral ictus. Since the typical skin lesions of the disease were absent, the diagnosis was made on the basis of a pathological pattern of an occluded biopsed artery. The elder brother presents clinical and instrumental vascular involvement without cutaneous lesions and could be slightly affected.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01959394
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  • 2
    Electronic Resource
    Electronic Resource
    Azathioprine in paediatric inflammatory bowel disease: an Italian multicentre survey (2002)
    Oxford, UK : Blackwell Science Ltd
    Alimentary pharmacology & therapeutics 16 (2002), S. 0 
    Details
    ISSN: 1365-2036
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Aim : To assess the efficacy and safety of azathioprine in a paediatric population with inflammatory bowel disease.Patients and methods : One hundred and twenty-three Italian children treated with azathioprine were studied retrospectively. The treatment duration and causes of its discontinuation, side-effects and variation in corticosteroid dose were assessed.Results : The mean age at inflammatory bowel disease diagnosis was 9.8 ± 3.6 years, and at the start of azathioprine therapy 11.8 ± 4.3 years. The mean duration of treatment was 19 ± 16 months. Fifty patients (41%) stopped treatment due to surgery (12%), prolonged remission (11%), non-response (7%), severe side-effects (7%) and poor compliance (3%). Of the 73 patients (59%) remaining on azathioprine, 11 had never been treated with corticosteroids, 27 were able to stop them and 35 were still on a very low daily dose (91% 〈 0.3 mg/kg). The difference in the daily corticosteroid dose between the beginning of azathioprine treatment (1 ± 0.6 mg/kg) and the conclusion of the study (0.18 ± 0.16 mg/kg) was statistically significant. Side-effects were recorded in 48 of the 123 patients (39%), but only eight required discontinuation of azathioprine.Conclusions : Azathioprine was efficacious in 70% of patients, but ineffective in 20% and induced severe toxicity in 7%. Corticosteroids were stopped or markedly reduced in 62% of patients, but they were never given in 9%.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2036.2002.01269.x
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  • 3
    Electronic Resource
    Electronic Resource
    The use of ciclosporin in paediatric inflammatory bowel disease: an Italian experience (2002)
    Oxford, UK : Blackwell Science Ltd
    Alimentary pharmacology & therapeutics 16 (2002), S. 0 
    Details
    ISSN: 1365-2036
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Aim : To asses the efficacy and safety of ciclosporin in a paediatric population with inflammatory bowel disease.Patients and methods : Twenty-three Italian children treated with ciclosporin were studied retrospectively. The indications for treatment were severe unresponsive colitis, chronic active colitis or severe fistulizing Crohn's disease. The treatment duration, follow-up and causes of drug discontinuation were assessed.Results : Sixteen patients were treated intravenously for a mean time of 10 ± 7 days (1–24 days) and 19 orally for a mean time of 133 days (17–660 days). The mean follow-up of all patients was 13.2 months. Ciclosporin was totally ineffective, being discontinued for surgery, in nine of 23 patients (39%); it was discontinued for partial response in three patients (13%). During treatment, clinical remission was achieved in eight children (35%) and maintained after drug withdrawal in four (17%). In severe unresponsive colitis, urgent colectomy was avoided in 12 (85%) of 14 patients who tolerated the drug. Side-effects appeared in six of 23 patients (26%), and three (13%) required ciclosporin to be discontinued due to neurotoxicity.Conclusions : Ciclosporin shows disappointing long-term results in the treatment of refractory inflammatory bowel disease, but can play an important role in preventing urgent surgery in unresponsive severe colitis. Severe side-effects can occur.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2036.2002.01308.x
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  • 4
    Electronic Resource
    Electronic Resource
    Superconducting rhenium as absorber for thermal detectors
    Amsterdam : Elsevier
    Nuclear Instruments and Methods in Physics Research Section A: 315 (1992), S. 260-262 
    Details
    ISSN: 0168-9002
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Physics
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0168-9002(92)90712-D
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  • 5
    Electronic Resource
    Electronic Resource
    Response to infliximab is related to disease duration in paediatric Crohn's disease (2003)
    Oxford, UK : Blackwell Science Ltd
    Alimentary pharmacology & therapeutics 18 (2003), S. 0 
    Details
    ISSN: 1365-2036
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background : Infliximab is an effective therapy in adult patients with refractory and fistulizing Crohn's disease. Experience in children is still limited.Aim : To evaluate the experience in 22 children and adolescents treated with infliximab with refractory and/or fistulizing Crohn's disease, and to compare duration of response in children between early Crohn's disease and late Crohn's disease.Methods : The experience in 22 children and adolescents treated with a total of 73 infusions was evaluated retrospectively. Treatment indication was refractory Crohn's disease in 9/22 patients, fistulizing Crohn's disease in 7/22 patients and both these conditions in 6/22. All patients with refractory Crohn's disease had late Crohn's disease (〉 1 year), whereas 6/13 patients with fistulas had early disease (〈 1 year).Results : Mean Paediatric Crohn's Disease Activity Index (PCDAI) decreased from 41.2 to 16.2 at 4 weeks (P 〈 0.01), and to 15.4 at 18 weeks (P 〈 0.01). Mean PCDAI at 18 weeks in children with early Crohn's disease and late Crohn's disease was 5.5 and 18.1, respectively (P 〈 0.05). Complete closure of fistulas was obtained in 5/6 children with early Crohn's disease and in 2/7 children with late Crohn's disease. Immediate adverse reactions were observed in two children.Conclusions : Infliximab is a highly effective treatment in children and adolescents with both severe refractory or fistulizing Crohn's disease. Children with early Crohn's disease have a higher chance of prolonged response to infliximab than children with late Crohn's disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2036.2003.01672.x
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  • 6
    Electronic Resource
    Electronic Resource
    Cystic lymphangioma of the spleen (1982)
    Springer
    Pediatric radiology 12 (1982), S. 94-95 
    Details
    ISSN: 1432-1998
    Keywords: Cystic lymphangioma of the spleen ; Computed tomography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The spleen is the least common intra-abdominal site for the development of cystic disease. A lymphangioma of the spleen was first reported in 1885 by Fink; since that time 47 cases of lymphangioma or lymphangectasia have been reported. Up to now, no CT study has been published. This report concerns a case of cystic lymphangioma of the spleen consisting of a large splenic cyst with associated multiple small subcapsular cysts and lymphangectasia. The utility of CT in diagnosis is presented.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00972442
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  • 7
    Electronic Resource
    Electronic Resource
    A new syndrome with cerebro-oculo-skeletal-renal involvement (1990)
    Springer
    Pediatric radiology 20 (1990), S. 612-614 
    Details
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on an infant male who presented with microcephaly of prenatal onset, schizencephaly, decorticated disturbance of the neurological function, congenital optic atrophy, abnormal eye movements and nystagmus. In addition, he had a skeletal dysplasia with predominant acromelic involvement and a renal disease characterized by both nephritic and nephrotic changes. The natural history of his condition included severe postnatal failure to thrive, lack of development of psychomotor milestones, intractable seizures, terminal renal insufficiency with early death. Such spectrum of phenotypic abnormalities has never been reported before and we suggest that it may represent a new syndromic entity. The differential diagnosis with the oculo-skeletal-renal syndromes, with the osteodysplastic primordial dwarfism of the Taybi-Linder type and with the Hutterite cerebro-osteo-nephrodysplasia, is discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02129073
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