2024 ICD-10-CM Diagnosis Code N04.8

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• Congenital nephrotic syndrome
• Drash syndrome
• Finnish congenital nephrotic syndrome
• Glomerular disease due to Finnish type congenital nephrotic syndrome
• Nephrotic syndrome with proliferative glomerulonephritis

Clinical Information

• Congenital Nephrotic Syndrome

  a rare autosomal recessive inherited nephrotic syndrome that is present in the first week of life. it manifests with edema and proteinuria and usually has a poor prognosis.

• Congenital Nephrotic Syndrome - Cytomegalovirus Associated|Cytomegalovirus Associated Congenital Nephrotic Syndrome

  nephrotic syndrome associated with a cytomegalovirus infection, most commonly presenting in the first three months of life.

• Congenital Nephrotic Syndrome - Diffuse Mesangial Sclerosis|Diffuse Mesangial Sclerosis|Diffuse Mesangial Sclerosis

  nephrotic syndrome within the first three motnhs of life, characterized initially by increased mesangial matrix, with or without hypertrophy and hyperplasia of podocytes, and eventual glomerular sclerosis.

• Congenital Nephrotic Syndrome - Focal Segmental Glomerulosclerosis|Congenital FSGS|Congenital FSGS

  nephrotic syndrome within the first three months of life, characterized by scarring of the glomerulus, in which only part of the glomerulus is involved, and less than 50% of the glomeruli are affected.

• Congenital Nephrotic Syndrome - Infection Associated|Infection Associated Congenital Nephrotic Syndrome

  nephrotic syndrome presenting within the first three months of life, and which is associated with an infectious process.

• Congenital Nephrotic Syndrome - Rubivirus Associated|Rubivirus Associated Congenital Nephrotic Syndrome

  nephrotic syndrome associated with rubella, most commonly presenting in the first three months of life.

• Congenital Nephrotic Syndrome - Toxoplasma Associated|Toxoplasma Associated Congenital Nephrotic Syndrome

  nephrotic syndrome associated with toxoplasmosis, most commonly presenting in the first three months of life.

• Congenital Nephrotic Syndrome - Treponema Pallidum Associated|Treponema Pallidum Associated Congenital Nephrotic Syndrome

  nephrotic syndrome associated with syphilis, most commonly presenting in the first three months of life.

• Nephrotic Syndrome - NPHS1 Associated|Congenital Nephrotic Syndrome - Finnish Type

  nephrotic syndrome attributed to mutation(s) in the nphs1 gene, which encodes the protein nephrin, and most commonly presents during the first three months of life.

N04.8 is grouped with Diagnostic Related Groups - MS-DRG Mapping

Diagnostic Related Groups (DRGs) are a classification system used to group together diagnosis codes for the purpose of reimbursement and healthcare management. DRGs are used to standardize the way hospitals and other providers are paid for inpatient services. In this system patients are grouped together based on their principal diagnosis in areas referred to as Major Diagnostic Categories (MDC). Once a patient is assigned a particular diagnostic category, providers receive a predetermined payment rate for the services rendered. This reimbursement rate is often fixed and is meant to cover the cost of care for that patient. Reimbursement is also affected by the relative weight of a diagnostic related group based on the severity of a patient's illness and the associated cost of care during hospitalization.

Diagnostic related groups encourage healthcare providers to focus on quality and efficiency in patient care while managing costs effectively. The diagnosis code is present in the following groups for version MS-DRG V41.0 applicable from 10/01/2023 through 09/30/2024.

MS-DRG	MS-DRG Title	MCD	Relative Weight
698	OTHER KIDNEY AND URINARY TRACT DIAGNOSES WITH MCC	11	1.6544
699	OTHER KIDNEY AND URINARY TRACT DIAGNOSES WITH CC	11	1.0208
700	OTHER KIDNEY AND URINARY TRACT DIAGNOSES WITHOUT CC/MCC	11	0.7083

The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.

Convert N04.8 to ICD-9-CM

• ICD-9-CM Code: 581.89 - Nephrotic syndrome NEC
  Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education

Kidney Diseases

You have two kidneys, each about the size of your fist. They are near the middle of your back, just below the rib cage. Inside each kidney there are about a million tiny structures called nephrons. They filter your blood. They remove wastes and extra water, which become urine. The urine flows through tubes called ureters. It goes to your bladder, which stores the urine until you go to the bathroom.

Most kidney diseases attack the nephrons. This damage may leave kidneys unable to remove wastes. Causes can include genetic problems, injuries, or medicines. You have a higher risk of kidney disease if you have diabetes, high blood pressure, or a close family member with kidney disease. Chronic kidney disease damages the nephrons slowly over several years. Other kidney problems include:

• Cancer
• Cysts
• Stones
• Infections

Your doctor can do blood and urine tests to check if you have kidney disease. If your kidneys fail, you will need dialysis or a kidney transplant.

NIH: National Institute of Diabetes and Digestive and Kidney Diseases

[Learn More in MedlinePlus]

Code History

• FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.