2026 ICD-10-CM Diagnosis Code E34.328Other genetic causes of short stature

ICD-10-CM Codes›E00–E89›E20-E35›E34

ICD-10-CM E34.328
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E34.328 is a billable ICD-10-CM diagnosis code for other genetic causes of short stature. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 643 through 645. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified endocrine disorders.

Code Identity

ICD-10-CM Code
E34.328
Billable Status
Yes — Valid for Submission
Code Describes
Other genetic causes of short stature
Short Description
Other genetic causes of short stature
Same as the full description in the CMS dataset.
Parent Code
E34.32
Genetic causes of short stature

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE20-E35Disorders of other endocrine glands
CategoryE34Other endocrine disorders
This CodeE34.328Other genetic causes of short stature

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • 46,XX ovarian dysgenesis, short stature syndrome
  • AMeD syndrome
  • Asexual dwarfism
  • Autosomal recessive asexual dwarfism
  • Body height below reference range
  • Bone age finding
  • Congenital anomaly of endocrine ovary
  • Congenital hypoplasia of adrenal gland
  • Congenital stenosis of carotid artery
  • Constitutional aplastic anemia
  • Delayed bone age
  • Diabetes mellitus associated with genetic syndrome
  • Dolichocephalic dwarfism
  • DONSON-related microcephaly, short stature, limb abnormalities spectrum
  • Hereditary acantholytic dermatosis
  • Infantile dwarf
  • Insulin resistance
  • Internal carotid artery stenosis
  • Intrauterine growth restriction, short stature, early adult-onset diabetes syndrome
  • Keratosis follicularis, dwarfism, cerebral atrophy syndrome
  • Long narrow head
  • Malabsorption of glucose
  • Microcephalic osteodysplastic primordial dwarfism type II
  • Microcephalic osteodysplastic primordial dwarfism types I and III
  • Microcephalic primordial dwarfism Alazami type
  • Microcephalic primordial dwarfism Dauber type
  • Microcephalic primordial dwarfism due to ZNF335 deficiency
  • Microcephalic primordial dwarfism Montreal type
  • Microcephalic primordial dwarfism Toriello type
  • Microcephalic primordial dwarfism, insulin resistance syndrome
  • Microcephalus, hypergonadotropic hypogonadism, short stature syndrome
  • MIRAGE syndrome
  • Moyamoya angiopathy, short stature, facial dysmorphism, hypergonadotropic hypogonadism syndrome
  • Moyamoya disease
  • Multiple malformation syndrome, moderate short stature, facial
  • Osteodysplastic primordial dwarfism
  • Ovarian dysgenesis
  • Primordial dwarfism
  • Short stature co-occurrent and due to endocrine disorder
  • Short stature due to growth hormone secretagogue receptor deficiency
  • Short stature with delayed bone age due to thyroid hormone metabolism deficiency
  • Short stature, unique facies, enamel hypoplasia, progressive joint stiffness, high-pitched voice syndrome
  • Short stature, wormian bones, dextrocardia syndrome
  • Stenosis of intracranial carotid artery
  • X-linked asexual dwarfism

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Short stature due to ACAN gene variant
  • Short stature due to aggrecan deficiency
  • Short stature due to NPR-2 gene variant

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

  • - Dwarfism - See Also: Short, stature; - E34.328

    • - congenital - See Also: Short, stature; - E34.328

    • - infantile - See Also: Short, stature; - E34.328

External Cause of Injuries IndexGuidance

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

    • Deficiency, deficient
      • short stature homeobox gene (SHOX)
        • with
          • short stature (idiopathic)
    • Dwarfism
    • Dwarfism
      • congenital
    • Dwarfism
      • infantile
    • Short, shortening, shortness
      • stature (child) (hereditary) (idiopathic) NEC
        • due to
          • genetic causes
            • ACAN gene variant
    • Short, shortening, shortness
      • stature (child) (hereditary) (idiopathic) NEC
        • due to
          • genetic causes
            • aggrecan deficiency
    • Short, shortening, shortness
      • stature (child) (hereditary) (idiopathic) NEC
        • due to
          • genetic causes
            • NPR-2 gene variant
    • Short, shortening, shortness
      • stature (child) (hereditary) (idiopathic) NEC
        • due to
          • genetic causes
            • specified genetic cause NEC

Clinical ClassificationClinical

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

CCSR END015
Other specified and unspecified endocrine disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Moyamoya Disease

    a noninflammatory, progressive occlusion of the intracranial carotid arteries and the formation of netlike collateral arteries arising from the circle of willis. cerebral angiogram shows the puff-of-smoke (moyamoya) collaterals at the base of the brain. it is characterized by endothelial hyperplasia and fibrosis with thickening of arterial walls. this disease primarily affects children but can also occur in adults.

  • Metabolic Syndrome

    a cluster of symptoms that are risk factors for cardiovascular diseases and type 2 diabetes mellitus. the major components of metabolic syndrome include abdominal obesity; atherogenic dyslipidemia; hypertension; hyperglycemia; insulin resistance; a proinflammatory state; and a prothrombotic (thrombosis) state.

  • Insulin Resistance

    diminished effectiveness of insulin in lowering blood sugar levels: requiring the use of 200 units or more of insulin per day to prevent hyperglycemia or ketosis.

  • Insulin Resistance Syndrome

    a cluster of closely related metabolic abnormalities associated with insulin resistance that confer an increased risk of the development of type 2 diabetes and cardiovascular disease. these abnormalities may include obesity, high blood pressure, abnormal cholesterol levels, proteinuria, and/or polycystic ovary syndrome.

  • Insulin Resistance Measurement|INSULINR|Insulin Resistance|Insulin Resistance

    the determination of the insulin resistance (cells inability to respond to insulin) in a biological specimen.

  • Insulin Resistance

    decreased sensitivity to circulating insulin which may result in acanthosis nigicrans, elevated insulin level or hyperglycemia.

  • Insulin Receptor Mutation - Associated Insulin Resistance Syndromes

    insulin resistance caused by inactivating mutation(s) in the insr gene encoding the insulin receptor.

  • Hyperandrogenism, Insulin Resistance, Acanthosis Nigricans Syndrome|HAIR-AN Syndrome

    a condition characterized by hyperandrogenism, insulin resistance, and acanthosis nigricans, typically associated with obesity in teenage girls. it is considered to be a subtype of polycystic ovarian syndrome, but may occur in male individuals. etiology is unclear, but some cases may be associated with mutations affecting the tyrosine kinase domain of the insulin receptor.

  • Homeostatic Model Assessment of Insulin Resistance

    an assessment of beta-cell function and insulin resistance based on fasting blood glucose and insulin concentrations.

  • RNF213 wt Allele|ALK Lymphoma Oligomerization Partner on Chromosome 17 Gene|ALO17|C17orf27|Chromosome 17 Open Reading Frame 27 Gene|DKFZp762N1115|FLJ13051|KIAA1554|KIAA1618|MGC46622|MGC9929|MYMY2|MYSTR|Moyamoya Disease 2 Gene|NET57|Ring Finger Protein 213 wt Allele|hCG_1812857

    human rnf213 wild-type allele is located in the vicinity of 17q25.3 and is approximately 135 kb in length. this allele, which encodes e3 ubiquitin-protein ligase rnf213 protein, may play a role in the regulation of protein ubiquitination. a chromosomal translocation t(2;17)(p23;q25) of this gene with the alk gene is associated with anaplastic large cell lymphoma.

  • Moyamoya Disease 2|MYMY2

    an autosomally inherited subtype of moyamoya disease often presenting in childhood caused by mutation(s) in the rnf213 gene, encoding e3 ubiquitin-protein ligase rnf213.

  • Moyamoya Disease

    a rare inherited vascular disorder characterized by constriction of arteries at the base of the brain, resulting in the formation of collateral circulation in order to compensate for the constriction. the name "moyamoya" in japanese means "puff of smoke" and derives from the characteristic radiographic appearance of the collateral vessels.

  • Obesity-Associated Insulin Resistance

    insulin resistance associated with obesity, which may be attributed in part to impaired insulin signaling in target tissues, or impaired insulin-stimulated glucose transport due to reduced expression of the glucose transporter protein 4.

  • Insulin Resistant Diabetes Mellitus with Acanthosis Nigricans and Hyperandrogenism|Type A Insulin Resistance Syndrome

    a syndrome of insulin resistance caused by mutation(s) in the insr gene, encoding the insulin receptor. this condition is characterized by a clinical triad of hyperinsulinemia, acanthosis nigricans, and hyperandrogenism without lipodystrophy. this is the least severe of a spectrum of disorders; the other two conditions are rabson-mendenhall syndrome and donohoe syndrome.

Patient EducationClinical

Growth Disorders

Does your child seem much shorter - or much taller - than other kids his or her age? It could be normal. Some children may be small for their age but still be developing normally. Some children are short or tall because their parents are.

Read the full article at MedlinePlus

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

Code History & ChangesHistory

Replacement E34.328 replaces the following previously assigned code(s):

  • E34.3 - Short stature due to endocrine disorder
FY 2023AddedAdded to the ICD-10-CM code setEffective October 1, 2022.
FY 2024–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E34.328Overview

Is E34.328 (Genetic causes of short stature) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report other genetic causes of short stature on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does E34.328 group to?

When other genetic causes of short stature is the principal diagnosis on an inpatient stay, it groups to MS-DRG 643, 644, 645, with relative weights from 0.7683 to 1.6461 depending on complications. Higher weights mean higher Medicare reimbursement.

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.