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PubMed This is a summary of 8 peer-reviewed journal articles Updated
Pediatric Nephrology

The Roadmap to Recovery: From Birth to Transplant

At a Glance

The treatment for Congenital Nephrotic Syndrome (CNF) follows a four-step roadmap: stabilizing the baby with albumin and nutrition, surgically removing the leaky kidneys (nephrectomy), using peritoneal dialysis, and finally performing a curative kidney transplant when the child reaches 10kg.

Back to Understanding Your Baby’s CNF Diagnosis

Navigating Congenital Nephrotic Syndrome (CNF) is a marathon, not a sprint. While the initial diagnosis in the NICU is frightening, medical teams follow a well-documented “roadmap” that transforms a life-threatening condition into a manageable one. This journey is organized into four distinct phases, all leading toward the ultimate goal: a kidney transplant [1].

Phase 1: Stabilization (Birth to Infancy)

The first goal is to stabilize your baby’s health and promote growth. Because the kidneys are “leaky,” your baby needs constant support to replace what is lost.

  • Daily Albumin Infusions: Your baby will receive intravenous albumin frequently to maintain blood volume and reduce swelling. To avoid daily needle pokes, the team will place a semi-permanent central IV line (like a Broviac line). Because these lines go directly to the heart, strict hygiene is absolutely critical to prevent severe, life-threatening infections [2][1].
  • Intense Nutrition: Growth is the most important “work” your baby has. They will need a high-protein, high-calorie diet, often delivered via a gastrostomy tube (G-tube) or feeding tube, to compensate for massive protein loss [1].
  • Medication Support: The team will provide levothyroxine to replace lost thyroid hormones and may use medications to help prevent infections and blood clots [3].

Phase 2: Surgery (The Strategic “Stop”)

At some point, the medical team may recommend a nephrectomy—the surgical removal of one or both kidneys [4].

  • Why remove the kidneys? While it sounds drastic, removing the kidneys is a strategic move to stop the massive “leak” of protein [2].
  • The Result: Once the “leaky” kidneys are gone, the swelling goes down and albumin infusions stop. However, removing both kidneys means your baby will no longer make urine (anuria). You will need to strictly monitor and restrict their fluid intake to prevent dangerous fluid overload between dialysis sessions. Additionally, because the massive protein leak has stopped, their feeding tube diet will be adjusted from high-protein to normal protein levels [5][4].

Phase 3: Dialysis (The Bridge to Transplant)

After the kidneys are removed, your baby will need renal replacement therapy to do the work the kidneys used to do.

  • Peritoneal Dialysis (PD): This is the most common method for infants. A small tube (catheter) is placed in the abdomen, and a special fluid cleans the blood while the baby sleeps [1].
  • Home Care: Many parents are trained to perform PD at home. While this allows the family to leave the hospital and establish a more “normal” routine, the dialysis catheter carries a severe risk of abdominal infection (peritonitis). You will be taught strict, sterile techniques that must be followed every day to keep your baby safe [1].

Phase 4: Kidney Transplant (The Ultimate Goal)

A kidney transplant is the definitive treatment for CNF [6].

  • The Milestone: Most centers look for the child to reach a weight of approximately 10kg (about 22 lbs)—which typically happens between 1.5 to 3 years of age—before performing a transplant [7].
  • The Outcome: Children with CNF generally have excellent long-term results after transplant. Once the new kidney is in place, the “Finnish type” disease does not come back [6].

Your Multidisciplinary Care Team

Managing CNF requires a large team. You should expect to work with:

  1. Pediatric Nephrologist: The lead doctor who manages kidney health and dialysis.
  2. Specialized Dietitian: Ensures your baby gets enough calories and protein to grow for surgery.
  3. Pediatric Surgeon: Performs the nephrectomy and places the dialysis catheter.
  4. NICU and Dialysis Nurses: The critical educators who will teach you how to manage G-tube feedings, central line hygiene, and home dialysis.
  5. Social Worker: Helps you navigate the emotional and financial toll of a long-term medical journey.

The emotional weight of seeing your baby with tubes and infusions is heavy. Remember that every tube and every infusion is a strategic step on the path toward a successful transplant and a healthy childhood [8][6].

Next: Life After Transplant

Common questions in this guide

Why does my baby need daily albumin infusions for CNF?
Because infants with CNF lose massive amounts of protein through their kidneys, frequent intravenous albumin infusions are given to replace this protein. This helps maintain a healthy blood volume, reduces dangerous swelling, and supports your baby's overall growth.
Why is a nephrectomy necessary for Congenital Nephrotic Syndrome?
Removing one or both kidneys stops the massive leak of protein that causes swelling and poor growth in infants with CNF. While it sounds drastic, this surgery stabilizes your child's health and transitions them to dialysis in preparation for a future kidney transplant.
What happens to my baby after their kidneys are removed?
Once both kidneys are removed, your baby will no longer produce urine and will require peritoneal dialysis to clean their blood. You will need to carefully monitor and restrict their daily fluid intake to prevent dangerous fluid overload between dialysis sessions.
When will my child be big enough for a kidney transplant?
Most medical centers require a child to reach a target weight of about 10 kilograms (22 pounds) before performing a kidney transplant. This milestone is typically reached between 1.5 to 3 years of age with the help of intensive nutrition and dialysis.
Will Congenital Nephrotic Syndrome come back after a kidney transplant?
No, children with CNF generally have excellent long-term results after a transplant. Once the new donor kidney is surgically in place, the specific 'Finnish type' disease does not return in the new organ.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What is the target weight our child needs to reach before the medical team considers a nephrectomy?
  2. 2.Which type of nephrectomy (unilateral or bilateral) do you anticipate will be best for my child?
  3. 3.Can we meet with the dietician and surgeon now to understand how the feeding tube and dialysis access will look?
  4. 4.How do you plan to monitor and prevent blood clots and infections while my child has a central line?
  5. 5.What are the specific requirements our child must meet to be placed on the kidney transplant waitlist?

Questions For You

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References

References (8)
  1. 1

    Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group.

    Boyer O, Schaefer F, Haffner D, et al.

    Nature reviews. Nephrology 2021; (17(4)):277-289 doi:10.1038/s41581-020-00384-1.

    PMID: 33514942
  2. 2

    Unilateral nephrectomy for young infants with congenital nephrotic syndrome of the Finnish type.

    Murakoshi M, Kamei K, Ogura M, et al.

    Clinical and experimental nephrology 2022; (26(2)):162-169 doi:10.1007/s10157-021-02141-5.

    PMID: 34581898
  3. 3

    Case Report: A Toddler With Anasarca Caused by Congenital Nephrotic Syndrome.

    Satekge TM, Kiabilua O, van Biljon G, et al.

    EJIFCC 2017; (28(2)):156-163.

    PMID: 28860961
  4. 4

    A Case of Congenital Nephrotic Syndrome with Crescents Caused by a Novel Compound Heterozygous Pairing of NPHS1 Genetic Variants.

    Goodman KN, Puapatanakul P, Barton KT, et al.

    Case reports in nephrology 2024; (2024()):5121375 doi:10.1155/2024/5121375.

    PMID: 38444459
  5. 5

    [A case of severe congenital nephrotic syndrome secondary to NPHS1 mutation].

    Quiros A, Lefèbvre C, Collard L, et al.

    Revue medicale de Liege 2020; (75(7-8)):544-547.

    PMID: 32779909
  6. 6

    Long-term outcome of congenital nephrotic syndrome after kidney transplantation in Japan.

    Hamasaki Y, Muramatsu M, Hamada R, et al.

    Clinical and experimental nephrology 2018; (22(3)):719-726 doi:10.1007/s10157-017-1508-4.

    PMID: 29185126
  7. 7

    Timing of renal replacement therapy does not influence survival and growth in children with congenital nephrotic syndrome caused by mutations in NPHS1: data from the ESPN/ERA-EDTA Registry.

    Hölttä T, Bonthuis M, Van Stralen KJ, et al.

    Pediatric nephrology (Berlin, Germany) 2016; (31(12)):2317-2325 doi:10.1007/s00467-016-3517-z.

    PMID: 27761660
  8. 8

    Physical performance capacity after pediatric kidney transplant and clinical parameters associated with physical performance capacity.

    Mäenpää H, Tainio J, Arokoski J, Jahnukainen T

    Pediatric nephrology (Berlin, Germany) 2023; (38(5)):1633-1642 doi:10.1007/s00467-022-05758-0.

    PMID: 36315277

This page outlines the general treatment roadmap for infants with Congenital Nephrotic Syndrome for educational purposes. Always consult your pediatric nephrologist and care team for medical decisions specific to your child.

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