Polycystic liver disease: Difference between revisions
CSV import |
CSV import |
||
| Line 1: | Line 1: | ||
[[File:Von_Meyenburg_complex_low_mag.jpg|Von Meyenburg complex | {{SI}} | ||
{{Infobox medical condition | |||
| name = Polycystic liver disease | |||
| image = [[File:Von_Meyenburg_complex_low_mag.jpg|left|thumb|Polycystic liver disease]] | |||
| caption = Histological image of a [[Von Meyenburg complex]], which is associated with polycystic liver disease | |||
| synonyms = PCLD | |||
| pronounce = | |||
| specialty = [[Hepatology]] | |||
| symptoms = [[Abdominal pain]], [[bloating]], [[hepatomegaly]] | |||
| complications = [[Portal hypertension]], [[liver failure]] | |||
| onset = Usually [[adulthood]] | |||
| duration = [[Chronic (medicine)|Chronic]] | |||
| types = [[Autosomal dominant polycystic liver disease]] | |||
| causes = [[Genetic disorder]] | |||
| risks = Family history, [[polycystic kidney disease]] | |||
| diagnosis = [[Ultrasound]], [[CT scan]], [[MRI]] | |||
| differential = [[Polycystic kidney disease]], [[liver cysts]] | |||
| prevention = None | |||
| treatment = [[Liver resection]], [[aspiration]] of cysts, [[liver transplantation]] | |||
| medication = [[Somatostatin analogs]] | |||
| prognosis = Generally good, but varies | |||
| frequency = Rare | |||
}} | |||
'''Polycystic liver disease''' (PLD) is a rare genetic disorder characterized by the presence of multiple cysts scattered throughout the liver. These cysts can vary in size and number, and while they are generally benign, they can lead to complications and affect liver function. | |||
== Etiology == | == Etiology == | ||
PLD is primarily associated with two genetic conditions: [[autosomal dominant polycystic kidney disease]] (ADPKD) and [[autosomal dominant polycystic liver disease]] (ADPLD). In ADPKD, cysts are present in both the kidneys and the liver, whereas in ADPLD, cysts are confined to the liver. | PLD is primarily associated with two genetic conditions: [[autosomal dominant polycystic kidney disease]] (ADPKD) and [[autosomal dominant polycystic liver disease]] (ADPLD). In ADPKD, cysts are present in both the kidneys and the liver, whereas in ADPLD, cysts are confined to the liver. | ||
== Pathophysiology == | == Pathophysiology == | ||
The cysts in PLD arise from the biliary epithelium and are filled with fluid. The exact mechanism of cyst formation is not fully understood, but it is believed to involve abnormalities in the [[polycystin]] proteins, which play a role in cell signaling and the structural integrity of the biliary epithelium. | The cysts in PLD arise from the biliary epithelium and are filled with fluid. The exact mechanism of cyst formation is not fully understood, but it is believed to involve abnormalities in the [[polycystin]] proteins, which play a role in cell signaling and the structural integrity of the biliary epithelium. | ||
== Clinical Presentation == | == Clinical Presentation == | ||
Many individuals with PLD are asymptomatic and may not require treatment. However, symptoms can occur if the cysts become large or numerous enough to cause complications. Common symptoms include: | Many individuals with PLD are asymptomatic and may not require treatment. However, symptoms can occur if the cysts become large or numerous enough to cause complications. Common symptoms include: | ||
| Line 13: | Line 33: | ||
* Early satiety | * Early satiety | ||
* Nausea | * Nausea | ||
In severe cases, complications such as cyst infection, hemorrhage, or rupture can occur. | In severe cases, complications such as cyst infection, hemorrhage, or rupture can occur. | ||
== Diagnosis == | == Diagnosis == | ||
PLD is typically diagnosed through imaging studies such as [[ultrasound]], [[computed tomography]] (CT), or [[magnetic resonance imaging]] (MRI). These imaging modalities can reveal the size, number, and distribution of liver cysts. | PLD is typically diagnosed through imaging studies such as [[ultrasound]], [[computed tomography]] (CT), or [[magnetic resonance imaging]] (MRI). These imaging modalities can reveal the size, number, and distribution of liver cysts. | ||
== Treatment == | == Treatment == | ||
Treatment for PLD is generally conservative and focuses on managing symptoms. Options include: | Treatment for PLD is generally conservative and focuses on managing symptoms. Options include: | ||
| Line 25: | Line 42: | ||
* Sclerotherapy | * Sclerotherapy | ||
* Surgical intervention in severe cases, such as cyst fenestration or liver transplantation | * Surgical intervention in severe cases, such as cyst fenestration or liver transplantation | ||
== Prognosis == | == Prognosis == | ||
The prognosis for individuals with PLD varies. Many individuals live normal lives without significant liver dysfunction. However, in cases where PLD is associated with ADPKD, the prognosis may be influenced by the severity of kidney disease. | The prognosis for individuals with PLD varies. Many individuals live normal lives without significant liver dysfunction. However, in cases where PLD is associated with ADPKD, the prognosis may be influenced by the severity of kidney disease. | ||
== See also == | |||
== | |||
* [[Autosomal dominant polycystic kidney disease]] | * [[Autosomal dominant polycystic kidney disease]] | ||
* [[Autosomal dominant polycystic liver disease]] | * [[Autosomal dominant polycystic liver disease]] | ||
* [[Liver cyst]] | * [[Liver cyst]] | ||
* [[Liver transplantation]] | * [[Liver transplantation]] | ||
== See Also == | == See Also == | ||
* [[Hepatology]] | * [[Hepatology]] | ||
* [[Genetic disorders]] | * [[Genetic disorders]] | ||
* [[Cystic diseases]] | * [[Cystic diseases]] | ||
== References == | == References == | ||
{{Reflist}} | {{Reflist}} | ||
== External Links == | == External Links == | ||
{{Commons category|Polycystic liver disease}} | {{Commons category|Polycystic liver disease}} | ||
[[Category:Hepatology]] | [[Category:Hepatology]] | ||
[[Category:Genetic disorders]] | [[Category:Genetic disorders]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
[[Category:Medicine]] | [[Category:Medicine]] | ||
{{medicine-stub}} | {{medicine-stub}} | ||
Latest revision as of 15:50, 8 April 2025
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC
| Polycystic liver disease | |
|---|---|
 | |
| Synonyms | PCLD |
| Pronounce | |
| Specialty | Hepatology |
| Symptoms | Abdominal pain, bloating, hepatomegaly |
| Complications | Portal hypertension, liver failure |
| Onset | Usually adulthood |
| Duration | Chronic |
| Types | Autosomal dominant polycystic liver disease |
| Causes | Genetic disorder |
| Risks | Family history, polycystic kidney disease |
| Diagnosis | Ultrasound, CT scan, MRI |
| Differential diagnosis | Polycystic kidney disease, liver cysts |
| Prevention | None |
| Treatment | Liver resection, aspiration of cysts, liver transplantation |
| Medication | Somatostatin analogs |
| Prognosis | Generally good, but varies |
| Frequency | Rare |
| Deaths | N/A |
Polycystic liver disease (PLD) is a rare genetic disorder characterized by the presence of multiple cysts scattered throughout the liver. These cysts can vary in size and number, and while they are generally benign, they can lead to complications and affect liver function.
Etiology[edit]
PLD is primarily associated with two genetic conditions: autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (ADPLD). In ADPKD, cysts are present in both the kidneys and the liver, whereas in ADPLD, cysts are confined to the liver.
Pathophysiology[edit]
The cysts in PLD arise from the biliary epithelium and are filled with fluid. The exact mechanism of cyst formation is not fully understood, but it is believed to involve abnormalities in the polycystin proteins, which play a role in cell signaling and the structural integrity of the biliary epithelium.
Clinical Presentation[edit]
Many individuals with PLD are asymptomatic and may not require treatment. However, symptoms can occur if the cysts become large or numerous enough to cause complications. Common symptoms include:
- Abdominal pain or discomfort
- Bloating
- Early satiety
- Nausea
In severe cases, complications such as cyst infection, hemorrhage, or rupture can occur.
Diagnosis[edit]
PLD is typically diagnosed through imaging studies such as ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI). These imaging modalities can reveal the size, number, and distribution of liver cysts.
Treatment[edit]
Treatment for PLD is generally conservative and focuses on managing symptoms. Options include:
- Pain management with analgesics
- Aspiration of cyst fluid
- Sclerotherapy
- Surgical intervention in severe cases, such as cyst fenestration or liver transplantation
Prognosis[edit]
The prognosis for individuals with PLD varies. Many individuals live normal lives without significant liver dysfunction. However, in cases where PLD is associated with ADPKD, the prognosis may be influenced by the severity of kidney disease.
See also[edit]
- Autosomal dominant polycystic kidney disease
- Autosomal dominant polycystic liver disease
- Liver cyst
- Liver transplantation
See Also[edit]
References[edit]
<references group="" responsive="1"></references>
External Links[edit]
