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  1. No Access

    Article

    Glomerular extracellular matrix and growth factors in diffuse mesangial sclerosis

    Diffuse mesangial sclerosis, isolated (IDMS) or observed in the context of Denys-Drash syndrome (DDS) due to WT1 mutation, is characterized by early onset nephrotic syndrome progressing to renal failure. A striki...

    Youxin Yang, Shao-yu Zhang, Mireille Sich, Agnès Béziau… in Pediatric Nephrology (2001)

  2. No Access

    Article

    Congenital Erythropoietic Porphyria: Prenatal Diagnosis and Autopsy Findings in Two Sibling Fetuses

    F. Daïkha-Dahmane, M. Dommergues, F. Narcy… in Pediatric and Developmental Pathology (2001)

  3. No Access

    Article

    Crescentic glomerulonephritis in hyper IgD syndrome

     The hyperimmunoglobulinemia D syndrome (HIDS) is a well-defined entity resembling familial Mediterranean fever. HIDS is a systemic inflammatory disease associated with stimulation of T-cell-mediated immunity....

    M. Tsimaratos, I. Koné-Paut, L. Daniel, M.-C. Gubler, B. Dussol… in Pediatric Nephrology (1999)

  4. No Access

    Article

    Collagen type III glomerulopathy: a new type of hereditary nephropathy

    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...

    M. C. Gubler, J. P. Dommergues, M. Foulard, A. Bensman, J. P. Leroy… in Pediatric Nephrology (1993)

  5. Article

    DELETIONS OF COLLAGEN TYPE 4 ALPHA 5 GENE IN 4 FAMILIES WITH ALPORT'S SYNDROME

    Alport's syndrome (AS) is manifested by progressive renal disease with typical basement membranes morphology and frequent association wtth ocular signs and deafness. This hereditary disease has been assigned t...

    M Sanak, R M Rad, G Dechennes, M C Gubler, S Heuertz, J P Grunfeld… in Pediatric Research (1992)

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  6. Article

    Immunogold studies of monomeric elements from the globular domain (NC1) of type IV collagen in renal basement membranes during experimental diabetes in the rat

    The protein A-gold immunocytochemical technique was applied to reveal the monomeric elements M1, M2* and M3 from the non-collagenous globular domain (NC1) of type IV collagen over various renal basement membranes...

    M. Desjardins, F. Gros, J. Wieslander, M. C. Gubler, M. Bendayan in Diabetologia (1990)

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  7. No Access

    Article

    Renal amyloidosis in juvenile chronic arthritis: evolution after chlorambucil treatment

    Over a 22-year period, eith patients affected with severe systemic or polyarticular juvenile chronic arthritis (JCA) developed systemic amyloidosis with nephrotic syndrome. They were treated with chlorambucil ...

    G. Deschênes, A. M. Prieur, F. Hayem, M. Broyer, M. C. Gubler in Pediatric Nephrology (1990)

  8. No Access

    Article

    Ocular changes in some progressive hereditary nephropathies

    Ocular involvement is a common feature of three herediatary nephropathies: cystinosis, nephronophthisis and Alport's syndrome. The follow-up of 25 cases of infantile cystinosis over a period of 26 years demons...

    J. L. Dufier, D. Orssaud, P. Dhermy, M. C. Gubler, M. F. Gagnadoux… in Pediatric Nephrology (1987)

  9. No Access

    Article

    Oligomeganephronic renal hypoplasia with tapetoretinal degeneration

    Bilateral renal hypoplasia with oligomeganephronia, associated with bilateral tapetoretinal degeneration was observed in a child; this association has been reported only once before. Light, ultrastructural and...

    A. Janin-Mercier, J. B. Palcoux, M. C. Gubler, M. de Latour, H. Dalens… in Virchows Archiv A (1985)

  10. No Access

    Chapter

    Loss of Ultrafiltration and Peritoneal Membrane Alterations in Children on CAPD

    Continuous ambulatory peritoneal dialysis (CAPD) is widely used as maintenance therapy in patients with end-stage renal disease (ESRD). CAPD was introduced as a treatment for children in 1978, and the use of C...

    P. Niaudet, R. Drachman, M.-C. Gubler, M. Broyer in CAPD in Children (1985)

  11. Article

    DEPAKENE (R) (DPA): a new aetiology for proximal tubulopathy

    A 9 year-old boy was referred on Nov. 1977 for a complete Fanconi syndrome. Prior history was unremarkable, except for a petit mal epilepsy treated with DPA (200 mg T.I.D.) since Oct. 1976. On Dec. 1976, he de...

    G Lenoir, J L Perignon, M C Gubler in Pediatric Research (1980)

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  12. Article

    FAMILIAL NEPHROSIS (FN)

    32 children with FN were studied, after exclusion of finnish type. They belonged to 17 families totalizing 39 affected subjects. When compared to sporadic nephrosis (N) FN showed a higher incidence of infantil...

    C Kleinknecht, G Gonzalez, M C Gubler, G Lenoir, M Broyer in Pediatric Research (1980)

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