2026 ICD-10-CM Diagnosis Code N07.9Hereditary nephropathy, not elsewhere classified with unspecified morphologic lesions

ICD-10-CM Codes›N00–N99›N00-N08›N07

ICD-10-CM N07.9
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

N07.9 is a billable ICD-10-CM diagnosis code for hereditary nephropathy, not elsewhere classified with unspecified morphologic lesions. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 698 through 700. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Nephritis; nephrosis; renal sclerosis.

Code Identity

ICD-10-CM Code
N07.9
Billable Status
Yes — Valid for Submission
Code Describes
Hereditary nephropathy, not elsewhere classified with unspecified morphologic lesions
Short Description
Hereditary nephropathy, NEC w unsp morphologic lesions
Parent Code
N07
Hereditary nephropathy, not elsewhere classified

Code Classification

ChapterN00–N99Diseases of the genitourinary system
SectionN00-N08Glomerular diseases
CategoryN07Hereditary nephropathy, not elsewhere classified
This CodeN07.9Hereditary nephropathy, not elsewhere classified with unspecified morphologic lesions

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • 10p partial monosomy syndrome
  • Autosomal dominant progressive nephropathy with hypertension
  • Carpal-tarsal osteolysis with nephropathy
  • Choreoathetosis
  • Chronic deafness
  • Congenital nephritis
  • Crome syndrome
  • Deafness, small bowel diverticulosis, neuropathy syndrome
  • Deletion of part of chromosome 10
  • Dent disease type 1
  • Dent disease type 2
  • Dent's disease
  • Diplegia
  • Disorder of zinc metabolism
  • Familial aplasia of the vermis
  • Hereditary angiopathy with nephropathy, aneurysms, and muscle cramps syndrome
  • Hereditary nephritis
  • Hereditary nephropathy
  • Hereditary sensory neuropathy
  • Hypoparathyroidism, deafness, renal disease syndrome
  • Idiopathic osteolyses
  • Inherited magnesium-losing nephropathy
  • Joubert syndrome
  • Joubert syndrome with renal defect
  • Magnesium-losing nephropathy
  • Marfanoid physique
  • Microcephalus, glomerulonephritis, marfanoid habitus syndrome
  • Nephrogenic syndrome of inappropriate antidiuresis
  • Nephropathy, deafness, hyperparathyroidism syndrome
  • Non-progressive hereditary glomerulonephritis
  • Parathyroid hyperplasia
  • Peripheral sensory neuropathy
  • Primary hyperparathyroidism
  • Progressive chorea
  • Progressive hereditary glomerulonephritis without deafness
  • Psychomotor regression, oculomotor apraxia, movement disorder, nephropathy syndrome
  • Sensory neuropathy
  • Severe oculo-renal-cerebellar syndrome
  • Spastic diplegia
  • Spastic paralysis
  • Spastic paraplegia, nephritis, deafness syndrome

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Nephropathy
      • hereditary NEC

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR GEN001
Nephritis; nephrosis; renal sclerosis
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Neonatal Severe Primary Hyperparathyroidism

    an autosomal recessive form of kenny-caffey syndrome that is secondary to mutation(s) in the tcbe gene that encodes tubulin-specific chaperone e; it is characterized by the following: hypoparathyroidism with hypocalcemia, marked growth retardation, craniofacial anomalies, absent diploic space, cortical thickening and medullary stenosis of long bones, and small hands and feet.

  • Primary Hyperparathyroidism

    hyperfunction of the parathyroid glands resulting in the overproduction of parathyroid hormone. it is caused by parathyroid adenoma, parathyroid hyperplasia, parathyroid carcinoma, and multiple endocrine neoplasia. it is associated with hypercalcemia and hypophosphatemia. signs and symptoms include weakness, fatigue, nausea, vomiting, constipation, depression, bone pain, osteoporosis, cystic bone lesions, and kidney stones.

  • Brachial Amyotrophic Diplegia|BAD|FAS|Flail Arm Syndrome|MIBS|Man-in-barrel Syndrome

    a neurodegenerative condition characterized by asymmetric weakness in the upper extremities resulting from segmental lower motor neuron dysfunction.

  • Diplegia

    paralysis affecting corresponding parts on both sides of the body.

  • Diplegia of Upper Limbs|Diplegia of upper limbs

    evidence of diplegia of the upper limbs.

  • Neurodevelopmental Disorder with Spastic Diplegia and Visual Defects|MRD19|Mental Retardation, Autosomal Dominant 19|NEDSDV

    an autosomal dominant condition caused by mutation(s) in the ctnnb1 gene, encoding catenin beta-1. it is characterized by severe intellectual disability, progressive spastic diplegia, visual impairment, and dysmorphic craniofacial features.

  • Quadriplegia|Bilateral Diplegia|Bilateral Diplegia|Quadriplegia, unspecified|Tetraplegia

    paralysis of all four limbs.

  • Spastic Diplegia|Little's Disease|Spastic diplegic cerebral palsy

    a type of cerebral palsy characterized by spasticity and hypertonia of the lower extremities bilaterally, particularly the legs, hips, and pelvis; this is the most common (70%) form of cerebral palsy.

  • Joubert Syndrome

    a rare genetic syndrome characterized by the hypoplasia or absence of the cerebellar vermis. signs and symptoms include rapid breathing (hyperpnea), sleep apnea, abnormal eye movements, mental retardation, and ataxia.

  • Joubert Syndrome 17|JBTS17

    an autosomal recessive subtype of joubert syndrome caused by mutation(s) in the cplane1 gene, encoding ciliogenesis and planar polarity effector 1.

  • Joubert Syndrome 3|JBTS3

    an autosomal recessive subtype of joubert syndrome caused by mutation(s) in the ahi1 gene, encoding jouberin.

  • Joubert Syndrome 4

    a rare genetic syndrome caused by mutations in the nphp1 gene. it is characterized by the hypoplasia or absence of the cerebellar vermis. signs and symptoms include rapid breathing (hyperpnea), sleep apnea, abnormal eye movements, mental retardation, and ataxia.

  • Joubert Syndrome 7|JBTS7

    an autosomal recessive sub-type of joubert syndrome caused by mutation(s) in the rpgrip1l gene, encoding a protein thought to function in programmed cell death. it is characterized by cerebellar and oculomotor apraxia, hypotonia and psychomotor delay, neonatal respiratory abnormalities, renal abnormalities, and retinal dystrophy.

  • Joubert Syndrome 9|JBTS9

    an autosomal recessive subtype of joubert syndrome caused by mutation(s) in the cc2d2a gene, encoding coiled-coil and c2 domain-containing protein 2a.

  • Neonatal Severe Primary Hyperparathyroidism

    a genetic condition caused by loss-of-function mutation(s) in the casr gene, encoding extracellular calcium-sensing receptor. it is characterized by severe hypercalcemia and metabolic bone disease occurring in the first six months of life.

Patient EducationClinical

Genetic Disorders

Genetic disorders are health conditions caused by changes (also called mutations or variants) in your genes. Genes are parts of DNA found in your cells that carry instructions for how your body grows, develops, and functions. Many genes tell your body how to make proteins, which are needed for your body to work properly.

The full article covers:

  • What are genetic disorders?
  • What causes genetic disorders?
  • What are the types of genetic disorders?
  • What are the different ways a genetic disorder can be inherited?
  • How are genetic disorders diagnosed?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert N07.9 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
583.9 Nephritis NOS
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About N07.9Overview

Is N07.9 (Hereditary nephropathy, not elsewhere classified) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report hereditary nephropathy, not elsewhere classified with unspecified morphologic lesions on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does N07.9 group to?

When hereditary nephropathy, not elsewhere classified with unspecified morphologic lesions is the principal diagnosis on an inpatient stay, it groups to MS-DRG 698, 699, 700, with relative weights from 0.6899 to 1.6544 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of N07.9?

Under the General Equivalence Mappings, hereditary nephropathy, not elsewhere classified with unspecified morphologic lesions converts to ICD-9-CM 583.9 (nephritis NOS). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.