Short answer · Medically reviewed summary · Last updated: 2026-05-08
Neonatal Hemochromatosis (NH), now more commonly referred to as Gestational Alloimmune Liver Disease (GALD), does not have a unique, specific code in the ICD-10 or ICD-9 systems. Instead, clinicians typically use ICD-10 code K76.89 (Other specified diseases of liver) or ICD-9 code 573.8 (Other specified disorders of liver) to document this condition. What is the clinical nature of Neonatal Hemochromatosis? Neonatal Hemochromatosis is a severe form of liver disease characterized by iron overload in the liver and extrahepatic tissues.
ICD10 code of Neonatal Hemochromatosis and ICD9 code
ICD-10 and ICD-9 codes for Neonatal Hemochromatosis, with classification details for clinicians, coders and patients.
Neonatal Hemochromatosis (NH), now more commonly referred to as Gestational Alloimmune Liver Disease (GALD), does not have a unique, specific code in the ICD-10 or ICD-9 systems. Instead, clinicians typically use ICD-10 code K76.89 (Other specified diseases of liver) or ICD-9 code 573.8 (Other specified disorders of liver) to document this condition.
What is the clinical nature of Neonatal Hemochromatosis?
Neonatal Hemochromatosis is a severe form of liver disease characterized by iron overload in the liver and extrahepatic tissues. While historically thought to be a metabolic disorder, current evidence confirms that Neonatal Hemochromatosis is primarily driven by maternal alloantibodies that cross the placenta and attack the fetal liver. Our DiseaseMaps community currently supports 42 people with Neonatal Hemochromatosis, providing a vital space for families to share experiences regarding this complex diagnosis.
How is Neonatal Hemochromatosis diagnosed?
Diagnosis of Neonatal Hemochromatosis is typically based on clinical presentation and biochemical evidence of liver failure in a newborn. Key diagnostic markers include:
- Elevated serum ferritin levels (often exceeding 800 ng/mL).
- Evidence of extrahepatic siderosis, commonly confirmed via MRI or labial salivary gland biopsy.
- Clinical signs of liver dysfunction, such as coagulopathy, jaundice, and hypoglycemia.
- Exclusion of other neonatal infectious or metabolic liver diseases.
Is Neonatal Hemochromatosis hereditary?
Unlike classic hereditary hemochromatosis, Neonatal Hemochromatosis is not caused by a direct genetic mutation in the infant. Rather, it is an alloimmune process. However, the condition has a high recurrence rate in subsequent pregnancies (up to 60-80% if untreated), as the mother continues to produce the specific antibodies that triggered the Neonatal Hemochromatosis in the previous pregnancy.
Next steps
- Consult a pediatric hepatologist or a maternal-fetal medicine specialist if a history of Neonatal Hemochromatosis exists in your family.
- Discuss with your medical team the use of intravenous immunoglobulin (IVIG) as a potential preventative therapy in future pregnancies.
- Connect with the 42 members in the DiseaseMaps community to find support and shared insights.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment; always seek the advice of your physician with any questions regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD) on Neonatal Hemochromatosis.
- Orphanet: Portal for rare diseases and orphan drugs (ORPHA:399).
- OMIM (Online Mendelian Inheritance in Man): Entry #231100.
- The Children's Liver Disease Foundation.
