• Corpus ID: 35121663

Congenital Nephrotic Syndrome of Finnish Type . A Report of Two Cases

  title={Congenital Nephrotic Syndrome of Finnish Type . A Report of Two Cases},
  author={Carmen Duicu and {\'E}va Kiss and B. Claudia and Nescu and Virginia Bodescu and Zsuzsanna Mor{\'e}h and Emőke Horv{\'a}th},
Congenital nephrotic syndrome of the Finnish type ( CNSF) is a rare autosomal recessive disease with high infant mortality without aggressive treatment. CNSF is characterized by massive in utero proteinu ria and nephrosis at birth. The majority of cases are cause d by genetic defects in the components of the glome rular filtration barrier, especially nephrin and podocin. Conge ital nephrotic syndrome (CNS) may also be a part o f a more generalized syndrome or caused by a perinatal infec tion… 

Figures and Tables from this paper


Congenital nephrotic syndrome of the Finnish type.
A 4-month-old white male infant admitted for evaluation and treatment of nephrotic syndrome of the Finnish type, with a male infant with anasarca and a poor general condition, was observed.
Congenital nephrotic syndrome: a clinico-pathologic study of thirty children.
In the developing countries, the management of patients with congenital nephrosis may have to be different from that in the developed countries in view of the high cost of medical management, poor outcome, high risk of serious complications, and high mortality rate.
Nephrotic syndrome in the 1st year of life
  • R. Habib
  • Medicine, Biology
    Pediatric Nephrology
  • 2004
The early onset of the nephropathy, its familial incidence and its association with Drash syndrome suggests an antenatal dysgenetic process.
[Congenital and infantile nephrotic syndrome].
Renal function deteriorates necessitating a dialysis-transplantation program between age five and eight and proteinuria does not recur after renal transplantation.
[The Finnish-type congenital nephrotic syndrome. Comments on 2 cases].
  • I. Sabau, E. Potencz
  • Medicine
    Revista de pediatrie, obstetrica si ginecologie. Pediatria
  • 1989
The authors described the clinical, biologic and histologic particularities of two cases of congenital nephrotic syndrome, Finnish type, which presented microcystic dilatation of the proximal tubuli contorti.
Cardiac malformations associated with the congenital nephrotic syndrome
It is concluded that the cardiac status of all patients with CNS should be reviewed regularly by a paediatrician, with a low threshold for referral to a cardiologist, as flow murmurs due to chronic anaemia may obscure cardiac pathology.
Congenital nephrotic syndrome of the Finnish type in Italy: a molecular approach.
This study provides further evidence that loss of function of the nephrin gene is the main cause of congenital nephrotic syndrome of the Finnish type in Italian patients.
Successful treatment of Finnish congenital nephrotic syndrome with captopril and indomethacin.
Management of congenital nephrotic syndrome
Management of CNS with captopril and indomethacin therapy in combination with unilateral nephrectomy achieves significant improvements in plasma albumin and reduces the need for albumin infusions and time in hospital, while growth is maintained.
Multiple cardiac malformation and congenital nephrotic syndrome in two siblings from Iran
Congenital nephrotic syndrome is a rare disease in Iran but co morbidity with cardiac malformation in two consecutive male siblings with pulmonic stenosis, closed VSD, severe PS with small ASD and