Nodular regenerative hyperplasia
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Nodular regenerative hyperplasia | |
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Synonyms | NRH |
Pronounce | N/A |
Specialty | N/A |
Symptoms | Portal hypertension, splenomegaly, ascites, esophageal varices |
Complications | Liver failure, portal hypertension |
Onset | Variable |
Duration | Chronic |
Types | N/A |
Causes | Unknown, associated with autoimmune diseases, hematological disorders, medications |
Risks | Autoimmune diseases, hematological disorders, certain medications |
Diagnosis | Liver biopsy, imaging studies |
Differential diagnosis | Cirrhosis, hepatic fibrosis, focal nodular hyperplasia |
Prevention | N/A |
Treatment | Symptomatic management, portal hypertension treatment |
Medication | N/A |
Prognosis | Variable, depends on complications |
Frequency | Rare |
Deaths | N/A |
Nodular Regenerative Hyperplasia
Nodular Regenerative Hyperplasia (NRH) is a rare liver condition characterized by the diffuse transformation of normal hepatic parenchyma into small regenerative nodules without significant fibrosis. This condition can lead to non-cirrhotic portal hypertension and is often associated with various systemic diseases and medications.
Pathophysiology
NRH is believed to result from uneven blood flow within the liver, leading to atrophy of some hepatocytes and compensatory hyperplasia of others, forming nodules. Unlike cirrhosis, NRH does not involve significant fibrosis, which distinguishes it from other nodular liver diseases.
Causes
The exact cause of NRH is unknown, but it is associated with several conditions and factors, including:
- Autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis.
- Hematological disorders like myeloproliferative neoplasms.
- Use of certain medications, including chemotherapy agents and immunosuppressants.
- HIV/AIDS and other chronic infections.
Clinical Presentation
Patients with NRH may be asymptomatic or present with signs of portal hypertension, such as:
Diagnosis
The diagnosis of NRH is challenging and often requires a combination of clinical, laboratory, and imaging studies. A definitive diagnosis is usually made through a liver biopsy, which shows nodular transformation without significant fibrosis.
Imaging
Imaging studies such as ultrasound, CT scan, and MRI may show nodular liver surface and signs of portal hypertension but are not specific for NRH.
Histology
Liver biopsy is the gold standard for diagnosis, revealing nodular regenerative changes in the absence of fibrosis.
Treatment
There is no specific treatment for NRH. Management focuses on treating the underlying condition and complications of portal hypertension. This may include:
- Beta-blockers for variceal bleeding prevention.
- Diuretics for ascites management.
- Endoscopic interventions for variceal bleeding.
Prognosis
The prognosis of NRH varies depending on the underlying cause and the presence of complications. Patients with well-controlled underlying conditions and minimal portal hypertension may have a favorable outcome.
See Also
External Links
- [American Liver Foundation](https://www.liverfoundation.org)
- [National Institute of Diabetes and Digestive and Kidney Diseases](https://www.niddk.nih.gov)
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Contributors: Prab R. Tumpati, MD