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The authors report 4 cases in 2 different families of a syndrome characterized by nephrotic syndrome of early onset (during the first 2 years of life) and microcephaly. Such an association was previously reported in 5 cases. In 4 it was familial. The study of families suggests an autosomal recessive transmission. Microcephaly was associated with psychomotor retardation, sometimes dysmorphic facies and various neurologic abnormalities. The nephrotic syndrome was characterized by its early onset and prognostic severity. However, the renal histologic lesions were heterogeneous: either minimal glomerular changes with focal and segmental hyalinosis or mesangial sclerosis, or, so-called "microcystic dysplasia". This heterogeneity does not suggest a single genetically determined disorder.
J Gaudelus, and
G Leverger, and
G Rault, and
M Nathanson, and
J L Giorno, and
L Boccon-Gibod, and
M Levy, and
M Broyer
July 2015,
Journal of nephropathology,
J Gaudelus, and
G Leverger, and
G Rault, and
M Nathanson, and
J L Giorno, and
L Boccon-Gibod, and
M Levy, and
M Broyer
December 1995,
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J Gaudelus, and
G Leverger, and
G Rault, and
M Nathanson, and
J L Giorno, and
L Boccon-Gibod, and
M Levy, and
M Broyer
January 1983,
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J Gaudelus, and
G Leverger, and
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M Nathanson, and
J L Giorno, and
L Boccon-Gibod, and
M Levy, and
M Broyer
March 1973,
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J Gaudelus, and
G Leverger, and
G Rault, and
M Nathanson, and
J L Giorno, and
L Boccon-Gibod, and
M Levy, and
M Broyer
September 1971,
Pediatrie,
J Gaudelus, and
G Leverger, and
G Rault, and
M Nathanson, and
J L Giorno, and
L Boccon-Gibod, and
M Levy, and
M Broyer
September 1975,
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J Gaudelus, and
G Leverger, and
G Rault, and
M Nathanson, and
J L Giorno, and
L Boccon-Gibod, and
M Levy, and
M Broyer
January 1969,
Bulletin de la Federation des societes de gynecologie et dobstetrique de langue francaise,
J Gaudelus, and
G Leverger, and
G Rault, and
M Nathanson, and
J L Giorno, and
L Boccon-Gibod, and
M Levy, and
M Broyer
December 1970,
Nihon Naibunpi Gakkai zasshi,
J Gaudelus, and
G Leverger, and
G Rault, and
M Nathanson, and
J L Giorno, and
L Boccon-Gibod, and
M Levy, and
M Broyer
June 1964,
Medecine dans le monde,
J Gaudelus, and
G Leverger, and
G Rault, and
M Nathanson, and
J L Giorno, and
L Boccon-Gibod, and
M Levy, and
M Broyer
January 2000,
Nephrologie,
