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Review
. 2020 Oct;35(10):1985-1990.
doi: 10.1007/s00467-020-04578-4. Epub 2020 May 6.

Congenital nephrotic syndrome: is early aggressive treatment needed? Yes

Tuula Hölttä  1 , Hannu Jalanko  2
Affiliations
Review

Congenital nephrotic syndrome: is early aggressive treatment needed? Yes

Tuula Hölttä et al. Pediatr Nephrol. 2020 Oct.

Abstract

Congenital nephrotic syndrome (CNS) was primarily considered one disease entity. Hence, one treatment protocol was proposed in the beginning to all CNS patients. Today, with the help of gene diagnostics, we know that CNS is a heterogeneous group of disorders and therefore, different treatment protocols are needed. The most important gene defects causing CNS are NPHS1, NPHS2, WT1, LAMB2, and PLCE1. Before active treatment, all infants with CNS died. It was stated already in the mid-1980s that intensive medical therapy followed by kidney transplantation (KTx) should be the choice of treatment for infants with severe CNS. In Finland, early aggressive treatment protocol was adopted from the USA and further developed for treatment of children with the Finnish type of CNS. The aim of this review is to state reasons for "early aggressive treatment" including daily albumin infusions, intensified nutrition, and timely bilateral nephrectomy followed by KTx at the age of 1-2 years.

Keywords: Albumin; Congenital nephrotic syndrome; NPHS1; Nephrectomy.

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Conflict of interest statement

The authors declare that they have no conflict of interest.

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