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  • 1
    Electronic Resource
    Electronic Resource
    Paediatric Castleman disease: report of seven cases and review of the literature (1999)
    Springer
    European journal of pediatrics 158 (1999), S. 631-637 
    Details
    ISSN: 1432-1076
    Keywords: Key words Castleman disease ; Lymphoproliferative disorder
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Castleman disease is a distinct lymphoproliferative disorder of unknown origin. Seven new cases in children are reported here and 76 cases from the paediatric literature are reviewed. The disease has been reported in 46 females and 37 males, their age ranging from 2 months to 17 years. The disease was localized in 72 cases and multicentric in 11 cases. The hyalinovascular type was more frequently encountered (54%) than the plasma cell type (24%). Laboratory abnormalities were more often associated with the plasma cell type and were mainly represented by anaemia and hypergammaglobulinaemia. Treatment of the localized tumour consisted of surgical excision, whereas treatment of the multicentric form was medical, comprising prednisone and other immunosuppressor drugs. The disease in the paediatric population seems to have a more favourable course than in adults. Conclusion The paediatric features of the disease suggest that Castleman disease in this population could represent an earlier form of the pathology or even suggest a benign lymphoproliferative disorder.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310051166
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  • 2
    Electronic Resource
    Electronic Resource
    Renal Granulomatous sarcoidosis in childhood: a report of 11 cases and a review of the literature (1999)
    Springer
    European journal of pediatrics 158 (1999), S. 154-159 
    Details
    ISSN: 1432-1076
    Keywords: Key words Sarcoidosis ; Child ; Granulomatous interstitial nephropathy ; Renal failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leucocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. Light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1–11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. Conclusion Renal failure, proteinuria, leucocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310051038
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  • 3
    Electronic Resource
    Electronic Resource
    Mastoiditis, meningitis and venous sinus thrombosis caused by Fusobacterium necrophorum (1994)
    Springer
    European journal of pediatrics 153 (1994), S. 339-341 
    Details
    ISSN: 1432-1076
    Keywords: Key words:Fusobacterium necrophorum– Anaerobic meningitis – Mastoiditis – Venous sinus thrombosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. The authors report a case of septic venous sinus thrombosis (VST) and meningitis occurring as an early complication of mastoiditis caused by Fusobacterium necrophorum. CT scan was normal, and cerebral angiography was required to diagnose the VST. The evolution was favourable with appropriate antimicrobial therapy and steroids.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01956414
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  • 4
    Electronic Resource
    Electronic Resource
    Myelodysplasia in childhood may be a polyclonal disease (1997)
    Springer
    Hematology and cell therapy 38 (1997), S. 325-330 
    Details
    ISSN: 1279-8509
    Keywords: Myelodysplastic syndromes ; Clonality ; Mitochondrial cytopathies ; Malformative syndromes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Myelodysplasia in childhood can be associated with constitutional abnormalities. Two main situations can be observed: constitutional diseases such as Down’s Syndrome may be the first step of a malignant stem cell transformation leading to monoclonal hematopoiesis. However, in other situations such as mitochondrial cytopathies or other polymalformative syndromes, myelodysplasia may simply be the hematological expression of a multi-tissue constitutional disease. In such cases, the bone marrow karyotype is usually found to be normal and, in affected females, clonality studies show a polyclonal pattern. Clonality assessment should be, when possible, a mandatory step before any major therapeutic decision during the course of childhood myelodysplasia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s00282-996-0325-0
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  • 5
    Electronic Resource
    Electronic Resource
    Induction chemotherapy followed by allogeneic bone marrow transplantation or aggressive consolidation chemotherapy in childhood acute myeloblastic leukemia: A prospective study from the French Society of Pediatric Hematology and Immunology (SHIP) (1997)
    Springer
    Hematology and cell therapy 38 (1997), S. 169-176 
    Details
    ISSN: 1279-8509
    Keywords: Acute myeloblastic leukemia ; Child ; Bone marrow transplantation ; Chemotherapy ; Cytarabine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In the LAME89/91 protocol, children with acute myeloid leukemia (AML) who achieved complete remission (CR) after induction chemotherapy, were treated either with allogeneic bone marrow transplantation (BMT) if they had an HLA-compatible related donor or with chemotherapy including high-dose cytarabine. The objectives of this study were to describe the overall results of this strategy and to compare the two post-remission arms. Two hundred and thirty-one children were enrolled in the protocol. Induction chemotherapy consisted of a combination of cytarabine and mitoxantrone. A CR was achieved in 204 children (88%). Fifty-one of them had an HLA-identical sibling donor and were eligible for BMT. These 51 patients, as well as two additional children who had a one antigen HLA-mismatched father, received BMT during first CR. Consequently, 53 patients were analysed in the BMT group and 151 in the chemotherapy group. With a mean follow up duration in the study of 38 ± 2 months, overall event-free survival (EFS) was 47 ± 7% at 4 years for the 231 patients entered into the protocol. The 4-year disease-free survival (DFS) was 53 ± 8% for the 204 patients who achieved complete remission after induction therapy. The 4-year probability of relapse was 28 ± 14% in the BMT group and 47 ± 9% in the chemotherapy group (p = 0.02). The risk of therapy-related death was 6.2% for BMT and 8.1% for chemotherapy. DFS was 68 ± 14% in the BMT group and 48 ± 9% in the chemotherapy group (p = 0.02). We conclude that allogeneic BMT from a matched sibling donor is the treatment of choice for reducing the relapse risk and for increasing DFS in children with AML in first CR.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s00282-996-0169-7
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  • 6
    Electronic Resource
    Electronic Resource
    Collagen type III glomerulopathy: a new type of hereditary nephropathy (1993)
    Springer
    Pediatric nephrology 7 (1993), S. 354-360 
    Details
    ISSN: 1432-198X
    Keywords: Hereditary nephropathy ; Collagen type III glomerulopathy ; Type III collagen ; Familial haemolytic uraemic syndrome ; Nail-patella syndrome ; Glomerular extracellular matrix
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A new type of hereditary glomerulopathy was observed in ten children presenting with early and progressive glomerular symptoms, often associated with hypertension. Light microscopy showed a diffuse increase in the mesangial matrix and generalized widening of the capillary walls. Electron-microscopic examination of renal tissue, after phosphotungstic acid treatment, revealed the presence of fibrillar collagen within the mesangial matrix and the subendothelial aspect of the glomerular basement membrane, adjacent to normal lamina densa. Immunohistochemical studies identified the fibrillar collagen not usually present within the glomerular extracellular matrix as type III collagen. Clinical and family studies ruled out the diagnosis of nail-patella syndrome, an autosomal dominant disorder with typical extrarenal symptoms, which is also characterized by the presence of fibrillar collagen within the glomerular basement membranes. The poor renal outcome, the possible extrarenal haematological and pulmonary involvement and the transmission as an autosomal recessive trait strongly suggest that collagen type III glomerulopathy is a new type of hereditary disease. From the high incidence of superimposed haemolytic uraemic syndrome in patients or their siblings, it may be hypothetized that collagen type III glomerulopathy is the underlying defect in some of the familial cases of haemolytic uraemic syndromes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00857536
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