Title:

Case Report: Hematemesis in a Young Man with Nodular Regenerative Hyperplasia (NRH) of the Liver

Learning Objective:

NRH is poorly understood with few cases reported and the majority of patients 25-60 years old when diagnosed. This patient was 20 with symptoms of portal hypertension already present.

Case Description:

This is a 20-year-old Hispanic male who presented to the emergency department (ED) with hematemesis, melena, and generalized weakness for the past 24 hours. Emergency Medical Services (EMS) was called and found the patient hypotensive with a systolic blood pressure in the 50s. He was subsequently taken to the hospital. In the hospital, a CTA was done that revealed large gastric varices. IR was consulted and he underwent successful obliteration of gastric varices via the gastric renal and splenorenal shunts. Outside medical records obtained from family members were reviewed which revealed the patient is suffering from nodular regenerative hyperplasia of unknown etiology. 
After the EGD the patient was transferred to the intensive care unit for further management. He was transfused with 2 units of pRBCs and transferred for a possible TIPS procedure.

Discussion:

Nodular regenerative hyperplasia (NRH) is a rare liver condition that is characterized by the transformation of liver parenchyma into small regenerative nodules distributed evenly throughout the liver. This transformation can ultimately lead to non-cirrhotic intrahepatic portal hypertension (NCIPH). NRH can be distinguished from other causes of NCIPH due to the presence of fibrous septa between the nodules. The mainstay management of NRH is the prevention and treatment of the complications related to the portal hypertension. 
The exact pathogenesis of NRH is still unknown, however, there are multiple hypotheses being studied. Although NRH can be associated with autoimmune disorder, hematological disease, and immunosuppressive medication, this was not the case for this patient. Like other cases of NRH, the treatment for this patient was managing the complications of portal hypertension.
NRH is a rare liver condition with an unknown etiology, commonly affecting those between the age 25-60 years old. However, in this case a 20-year-old male with NRH presented to the ED with symptoms of portal hypertension already evident needing to be treated immediately.

References:

Hartleb M, Gutkowski K, Milkiewicz P. Nodular regenerative hyperplasia: evolving concepts on underdiagnosed cause of portal hypertension. World J Gastroenterol. 2011 Mar 21;17(11):1400-9. doi: 10.3748/wjg.v17.i11.1400. PMID: 21472097; PMCID: PMC3070012.
LiverTox: Clinical and Research Information on Drug-Induced Liver Injury [Internet]. Bethesda (MD): National Institute of Diabetes and Digestive and Kidney Diseases; 2012-. Nodular Regenerative Hyperplasia. [Updated 2019 May 4].Available from: https://www.ncbi.nlm.nih.gov/books/NBK548385/
Lee, M., Izzy, M., Akki, A., Tanaka, K., & Kalia, H. (2017). Nodular Regenerative Hyperplasia. Journal of Investigative Medicine High Impact Case Reports. https://doi.org/10.1177/2324709617690742