Nodular regenerative hyperplasia
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; Medical liver disease; Synonym 3
Overview
Nodular regenerative hyperplasia is described as a form of non-cirrhotic portal hypertension. Nodular regenerative hyperplasia is associated with renal transplants, bone marrow transplants and vasculitides.
Historical Perspective
- Nodular regenerative hyperplasia was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
- In [year], [gene] mutations were first identified in the pathogenesis of nodular regenerative hyperplasia.
- In [year], the first [discovery] was developed by [scientist] to treat/diagnose nodular regenerative hyperplasia.
Classification
- Nodular regenerative hyperplasia may be classified according to [classification method] into [number] subtypes/groups:
- Other variants of nodular regenerative hyperplasia include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
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 [gene name] gene/Mutation in [gene name] has been associated with the development of nodular regenerative hyperplasia, involving the [molecular pathway] pathway.
- On gross pathology findings of nodular regenerative hyperplasia, may include:
- Diffuse nodularity
- On microscopic histopathological analysis findings of nodular regenerative hyperplasia, may include:
- 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:
- Azathioprine
- Thioguanine
- Mercaptopurine
- Didanosine
- Stavudine
- Isoplatin
- Vitamin A
- Methotrexate
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:
- Hepatocellular carcinoma
- Cirrhosis
- Peptic ulcer
- Metastatic disease
Epidemiology and Demographics
- The prevalence of nodular regenerative hyperplasia is approximately [number or range] per 100,000 individuals worldwide.
- In [year], the incidence of nodular regenerative hyperplasia was estimated to be [number or range] cases per 100,000 individuals in [location].
Age
- Patients of all age groups may develop nodular regenerative hyperplasia.
- Nodular regenerative hyperplasia is more commonly observed among patients aged [age range] years old.
- Nodular regenerative hyperplasia is more commonly observed among [elderly patients/young patients/children].
Gender
- Nodular regenerative hyperplasia affects men and women equally.
- [Gender 1] are more commonly affected with nodular regenerative hyperplasia than [gender 2].
- The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
Race
- There is no racial predilection for nodular regenerative hyperplasia.
- Nodular regenerative hyperplasia usually affects individuals of the [race 1] race.
- [Race 2] individuals are less likely to develop 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.
Natural History, Complications and Prognosis
- The majority of patients with nodular regenerative hyperplasia remain asymptomatic for [duration/years].
- Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
- If left untreated, [#%] of patients with nodular regenerative hyperplasia may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
- Common complications of nodular regenerative hyperplasia include [complication 1], [complication 2], and [complication 3].
- Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with nodular regenerative hyperplasia is approximately [#%].
Diagnosis
Diagnostic Criteria
- 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
- <120 U/L: <3 times ULN
- Alkaline phosphatase (<345 U/L: <3 times ULN)
- Clinical, radiologic or endoscopic signs of portal hypertension, such as:
- Ascites
- Splenomegaly
- Abdominal venous collaterals
- Varices
- Portal hypertensive gastropathy
- Liver biopsy showing nodularity with minimal or no fibrosis
Symptoms
- Nodular regenerative hyperplasia may be initially asymptomatic.
- Symptoms of nodular regenerative hyperplasia may include the following:
- Fatigue
- Weight loss
- Abdominal distension
- Nausea
- Hematemesis
Physical Examination
- Patients with nodular regenerative hyperplasia usually appear [general appearance].
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
- [finding 5]
- [finding 6]
Laboratory Findings
- There are no specific laboratory findings associated with nodular regenerative hyperplasia.
- A [positive/negative] [test name] is diagnostic of nodular regenerative hyperplasia.
- An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of nodular regenerative hyperplasia.
- Other laboratory findings consistent with the diagnosis of nodular regenerative hyperplasia include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
Imaging Findings
- There are no [imaging study] findings associated with nodular regenerative hyperplasia.
- [Imaging study 1] is the imaging modality of choice for nodular regenerative hyperplasia.
- On [imaging study 1], nodular regenerative hyperplasia is characterized by [finding 1], [finding 2], and [finding 3].
- [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
- Nodular regenerative hyperplasia may also be diagnosed using [diagnostic study name].
- Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
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 [medical therapy 1] and [medical therapy 2].
- [Medical therapy 1] acts by [mechanism of action1].
- Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
Surgery
- Surgery is the mainstay of therapy for nodular regenerative hyperplasia.
- [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of nodular regenerative hyperplasia.
- [Surgical procedure] can only be performed for patients with [disease stage] nodular regenerative hyperplasia.
Prevention
- There are no primary preventive measures available for nodular regenerative hyperplasia.
- Effective measures for the primary prevention of nodular regenerative hyperplasia include [measure1], [measure2], and [measure3].
- Once diagnosed and successfully treated, patients with nodular regenerative hyperplasia are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].