Short answer · Medically reviewed summary · Last updated: 2026-05-08
Congenital Nephrotic Syndrome Finnish Type (CNF) is classified under the ICD-10 system as code Q04.0 (Congenital nephrotic syndrome), while in the ICD-9 system, it is categorized under code 753.16. These codes are essential for medical billing and tracking the clinical progression of Congenital Nephrotic Syndrome Finnish Type in hospital and research registries. What is the clinical classification of Congenital Nephrotic Syndrome Finnish Type? Congenital Nephrotic Syndrome Finnish Type is a rare, autosomal recessive disorder characterized by massive proteinuria beginning in utero or shortly after birth.
ICD10 code of Congenital Nephrotic Syndrome Finnish Type and ICD9 code
ICD-10 and ICD-9 codes for Congenital Nephrotic Syndrome Finnish Type, with classification details for clinicians, coders and patients.
Congenital Nephrotic Syndrome Finnish Type (CNF) is classified under the ICD-10 system as code Q04.0 (Congenital nephrotic syndrome), while in the ICD-9 system, it is categorized under code 753.16. These codes are essential for medical billing and tracking the clinical progression of Congenital Nephrotic Syndrome Finnish Type in hospital and research registries.
What is the clinical classification of Congenital Nephrotic Syndrome Finnish Type?
Congenital Nephrotic Syndrome Finnish Type is a rare, autosomal recessive disorder characterized by massive proteinuria beginning in utero or shortly after birth. Because it is a specific genetic condition, clinicians use the ICD-10 code Q04.0 to distinguish Congenital Nephrotic Syndrome Finnish Type from other forms of childhood nephrosis, such as steroid-sensitive nephrotic syndrome.
How is Congenital Nephrotic Syndrome Finnish Type diagnosed?
Diagnosis typically involves a combination of clinical assessment and genetic testing. Key indicators include:
- Elevated alpha-fetoprotein (AFP) levels in maternal serum and amniotic fluid during pregnancy.
- Onset of massive proteinuria within the first three months of life.
- Mutation analysis of the NPHS1 gene, which encodes the protein nephrin, confirming the diagnosis of Congenital Nephrotic Syndrome Finnish Type.
Is Congenital Nephrotic Syndrome Finnish Type hereditary?
Yes, Congenital Nephrotic Syndrome Finnish Type is inherited in an autosomal recessive pattern. This means that both parents must be carriers of a mutation in the NPHS1 gene for their child to be affected, resulting in a 25% chance of recurrence in each pregnancy. Genetic counseling is strongly recommended for families affected by Congenital Nephrotic Syndrome Finnish Type to understand these inheritance risks.
Next steps
- Consult with a pediatric nephrologist to discuss long-term management, including potential nephrectomy and kidney transplantation.
- Connect with the 4 members of the DiseaseMaps.org community who are navigating life with Congenital Nephrotic Syndrome Finnish Type to share experiences.
- Request a referral to a clinical geneticist for comprehensive family testing and reproductive counseling.
Medical disclaimer: This information is for educational purposes only and should not replace professional medical advice, diagnosis, or treatment.
References
- Orphanet: Congenital nephrotic syndrome of Finnish type (ORPHA:653).
- NIH Genetic and Rare Diseases Information Center (GARD): Congenital nephrotic syndrome, Finnish type.
- OMIM (Online Mendelian Inheritance in Man): Nephrotic Syndrome, Congenital, Finnish Type; NPHS1 (Entry #256300).
- National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) resources on rare pediatric renal disorders.
