Nodular regenerative hyperplasia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and Keywords: NRHL; Non-cirrhotic portal hypertension; NRH

Overview

Nodular regenerative hyperplasia is described as a rare form of non-cirrhotic portal hypertension. Nodular regenerative hyperplasia is associated with solid organ transplant (eg. renal transplants, bone marrow transplants) and chronic use of medications. Nodular regenerative hyperplasia may be classified into 2 subtypes: pre-sinusoidal and sinusoidal.[1] The pathogenesis of nodular regenerative hyperplasia is characterized by arterial hypervascularity secondary to loss of hepatic vein radicles and loss of central venule in the hepatic lobule. Nodular regenerative hyperplasia is a rare disease. The estimated incidence of nodular regenerative hyperplasia is approximately 0.34 cases per 100,000 individuals. Nodular regenerative hyperplasia is more commonly observed among patients between 25 and 65 years old.[2] The majority of patients with nodular regenerative hyperplasia may be initially asymptomatic. Early clinical features include fatigue, weight loss, and abdominal distension. If left untreated, the majority of patients with nodular regenerative hyperplasia may progress to develop acute hepatic failure and death. The diagnosis of nodular regenerative hyperplasia is made with the following diagnostic criteria: latency of more than 6 months, minimal or no elevations in serum ALT, clinical, radiologic or endoscopic signs of portal hypertension, and liver biopsy.

Historical Perspective

  • Nodular regenerative hyperplasia was first described by Steiner in 1959.[3]

Classification

  • Nodular regenerative hyperplasia may be classified into 2 subtypes:[1]
  • Pre-sinusoidal
  • Sinusoidal

Pathophysiology

  • The pathogenesis of nodular regenerative hyperplasia is characterized by arterial hypervascularity secondary to loss of hepatic vein radicles and loss of central venule in the hepatic lobule.
  • The RASSF1A gene has been associated with the development of nodular regenerative hyperplasia, involving the proapoptotic pathway.[4]
  • On gross pathology findings of nodular regenerative hyperplasia, may include:[4]
  • Diffuse nodularity
  • On microscopic histopathological analysis findings of nodular regenerative hyperplasia, may include:[4]
  • Diffuse hepatic micronodular transformation in groups without fibrous septa between the nodules
  • "Plump" hepatocytes surrounded by atrophic ones
  • No fibrosis

Causes

  • Common causes of nodular regenerative hyperplasia, may include:
  • Solid organ transplantation
  • Chronic use of medications, such as:

Differentiating nodular regenerative hyperplasia from other Diseases

  • Nodular regenerative hyperplasia must be differentiated from other diseases that cause fatigue, hematemesis, and weight-loss such as:[2]

Epidemiology and Demographics

  • Nodular regenerative hyperplasia is a rare disease.
  • The prevalence of nodular regenerative hyperplasia is approximately 31 cases per 100,000 individuals in the United Kingdom.
  • The estimated incidence of nodular regenerative hyperplasia is approximately 0.34 cases per 100,000 individuals.

Age

  • Nodular regenerative hyperplasia is more commonly observed among patients aged between 25 and 65 years old.[2]
  • Nodular regenerative hyperplasia is more commonly observed among adults and elderly patients

Gender

  • Nodular regenerative hyperplasia affects men and women equally.

Race

  • There is no racial predilection for nodular regenerative hyperplasia.

Risk Factors

  • Common risk factors in the development of nodular regenerative hyperplasia are recurrent vascular and infectious complications such as in cystic fibrosis, common variable hypogammaglobulinemia, and chronic granulomatous disease.

Screening

Natural History, Complications and Prognosis

  • The majority of patients with nodular regenerative hyperplasia may be initially asymptomatic
  • Early clinical features include fatigue, weight loss, and abdominal distension.
  • If left untreated, the majority of patients with nodular regenerative hyperplasia may progress to develop acute hepatic failure and death.
  • Nodular regenerative hyperplasia severity may be classified by the Child-Pugh score.
  • Common complications of nodular regenerative hyperplasia, may include:[5]
  • Prognosis is generally poor, and the mean survival rate of patients with nodular regenerative hyperplasia is approximately 8.1 years.[5]

Diagnosis

Diagnostic Criteria

  • The diagnosis of nodular regenerative hyperplasia is made with the following diagnostic criteria:[6]
  • Latency of more than 6 months
  • Minimal or no elevations in serum ALT
  • Alkaline phosphatase (<345 U/L: <3 times ULN)
  • Clinical, radiologic or endoscopic signs of portal hypertension, such as:[6]
  • Liver biopsy showing nodularity with minimal or no fibrosis

Symptoms

  • Symptoms of nodular regenerative hyperplasia may include the following:[6]
  • Fatigue
  • Weight loss
  • Abdominal distension
  • Nausea
  • Hematemesis

Physical Examination

  • Patients with nodular regenerative hyperplasia may be well-appearing, lethargic, or confused.
  • Physical examination of the abdomen may be remarkable for:

Inspection

  • Caput medusae
  • Appearance of distended and engorged superficial epigastric veins

Auscultation

  • Positive liver scratch test for enlarged liver size.
  • Cruveilhier-Baumgarten murmur
  • A venous hum in patients with portal hypertension

Percussion

  • Dull percussion

Palpation

  • Muehrcke nails
  • Terry nails, or "luekonychia"

Laboratory Findings

  • Laboratory findings consistent with the diagnosis of nodular regenerative hyperplasia, may include:[7]
  • Abnormal AST/ALT ratio
  • Less than <345 U/L: <3 times upper limit of normal
  • Decreased levels of vitamin B12

Imaging Findings

  • Imaging studies useful for the diagnosis of nodular regenerative hyperplasia, may include:[2]
  • Ultrasound ( doppler ultrasound)
  • CT angiography
  • MRI angiography
  • Ultrasound is the imaging modality of choice for nodular regenerative hyperplasia
  • On ultrasound, findings of nodular regenerative hyperplasia, may include:
  • May resemble the ring-shaped coral
  • Usually smaller than 3 mm
  • Round isoechoic lesions
  • Thin hyper-echoic rim

Other Diagnostic Studies

  • Nodular regenerative hyperplasia may also be diagnosed using biopsy.
  • Liver biopsy confirms the diagnosis of nodular regenerative hyperplasia
  • On biopsy, findings of nodular regenerative hyperplasia, may include:[2]
  • Diffuse fine nodularity of the liver
  • Nodule size between 1-3 mm
  • Mild hepatomegaly

Treatment

Medical Therapy

  • There is no treatment for nodular regenerative hyperplasia; the mainstay of therapy is supportive care.
  • The mainstay of therapy for nodular regenerative hyperplasia is acute management of complications, such as variceal bleeding. [2]

Surgery

  • Surgery is the mainstay of therapy for nodular regenerative hyperplasia.
  • Surgical resection is usually performed for patients with persistent pain or for lesions that are suspicious on radiological findings.

Prevention

  • There are no primary preventive measures available for nodular regenerative hyperplasia.

References

  1. 1.0 1.1 Louwers LM, Bortman J, Koffron A, Stecevic V, Cohn S, Raofi V (2012). "Noncirrhotic Portal Hypertension due to Nodular Regenerative Hyperplasia Treated with Surgical Portacaval Shunt". Case Rep Med. 2012: 965304. PMC 3432362Freely accessible. PMID 22956964. doi:10.1155/2012/965304. 
  2. 2.0 2.1 2.2 2.3 2.4 2.5 Hartleb M, Gutkowski K, Milkiewicz P (2011). "Nodular regenerative hyperplasia: evolving concepts on underdiagnosed cause of portal hypertension". World J. Gastroenterol. 17 (11): 1400–9. PMC 3070012Freely accessible. PMID 21472097. doi:10.3748/wjg.v17.i11.1400. 
  3. STEINER PE (1959). "Nodular regenerative hyperplasia of the liver". Am. J. Pathol. 35: 943–53. PMC 1934844Freely accessible. PMID 13834213. 
  4. 4.0 4.1 4.2 Nodular regenerative hyperplasia. Libre Pathology https://librepathology.org/wiki/Medical_liver_disease#Nodular_regenerative_hyperplasia Accessed on April 12, 2015
  5. 5.0 5.1 Morris JM, Oien KA, McMahon M, Forrest EH, Morris J, Stanley AJ, Campbell S (2010). "Nodular regenerative hyperplasia of the liver: survival and associated features in a UK case series". Eur J Gastroenterol Hepatol. 22 (8): 1001–5. PMID 20075739. doi:10.1097/MEG.0b013e3283360021. 
  6. 6.0 6.1 6.2 Nodular regenerative hyperplasia http://livertox.nih.gov/Phenotypes_nodular.html Accesed on April 12, 2016
  7. Seijo S, Lozano JJ, Alonso C, Reverter E, Miquel R, Abraldes JG, Martinez-Chantar ML, Garcia-Criado A, Berzigotti A, Castro A, Mato JM, Bosch J, Garcia-Pagan JC (2013). "Metabolomics discloses potential biomarkers for the noninvasive diagnosis of idiopathic portal hypertension". Am. J. Gastroenterol. 108 (6): 926–32. PMID 23419380. doi:10.1038/ajg.2013.11. 

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