Cyst liver

from Wikipedia, the free encyclopedia
Classification according to ICD-10
Q44.6 Cyst liver
ICD-10 online (WHO version 2019)

As polycystic liver disease , including polycystic liver or cystic liver disease called, refers to a usually congenital malformation of the liver , in which this member having a plurality of liquid-filled cavities ( cysts is penetrated). The polycystic liver disease is the benign ( benign ) tumors counted.

In contrast to the very common liver cyst , which is usually a harmless incidental finding of the liver with a cyst, the cyst liver is a relatively rare serious disease.

Etiology and prevalence

Cystic liver disease can have genetic ( autosomal - dominant inheritance ) causes or it can occur sporadically. The exact prevalence is not known. The disease mostly affects women over the age of forty.

The common cause of cystic liver disease is autosomal dominant polycystic kidney disease (ADPKD). As a systemic disease , ADPKD also very often affects the liver, which is why cyst liver is diagnosed in around 75% of patients with ADPKD.

Polycystic Liver Disease

While most of the cyst liver diseases are caused by genetically determined ADPKD, there is an autosomal dominant cause of cyst liver that is independent of ADPKD. In this case, it is called polycystic liver disease ( PCLD ). The genetic background for PCLD are mutations on chromosome 19 , gene locus p13.2 – p13.1 or chromosome 6 , gene locus 21.

The PRKCSH gene, also known as the G19P1 or PCLD gene, is located on chromosome 19 in humans . This gene encodes the protein kinase C substrate 80K-H .

The SEC63 gene located on chromosome 6 codes for an endoplasmic-reticulum membrane complex.

PCLD is a very rare disease. The incidence of PCLD is less than 1 in 10,000.

Symptoms and diagnosis

The space required by the constantly growing cysts causes a feeling of pressure and pain in the upper abdomen , depending on the size of the liver . The cysts can press on the stomach and intestines. Those affected then often suffer from a lack of appetite and weight loss. Liver function is maintained for a long time even with strong penetration with cysts.

If symptoms are present, the diagnosis is made using imaging methods , usually ultrasound or computed tomography . Since the majority of polycystic liver diseases are genetic, a detailed family history also plays an important role.

therapy

A drug treatment of a cyst liver is currently not possible. Cure can only be guaranteed through surgery . There are currently three intervention methods available that are only used when the liver function is severely restricted by the cysts.

Open cyst uncovering

Prior to the development of laparoscopy developed by the Taiwanese doctor TY Lin and colleagues in 1968 open cyst was uncapping ( Engl. : De-roofing ), also open fenestration called the method of choice.

Laparoscopic cyst uncovering

The laparoscopic cyst uncapping is now in humans the drug of choice for treatment of polycystic liver disease. The minimally invasive procedure was first performed in 1991. After aspiration (suction) of the cyst fluid, the roof of the cysts is completely removed and the remaining cavity is filled with the body's own large mesh ( omentum majus ), which is sewn to the edge of the cyst. This suppresses the formation of new cysts.

Liver transplant

The last and by far the most complex measure for treating cyst liver is liver transplantation. It is usually only performed when the liver function has almost completely come to a standstill, which is the case when the liver is completely infected with cysts.

history

The cyst liver was first described in 1856 by the British doctor F. Bristowe as a side effect of the autosomal dominant polycystic kidney disease (ADPKD).

literature

Reference books

  • C. Thomas (Editor): Special Pathology. Schattauer Verlag, 1996, ISBN 3-794-52110-2 , p. 253.
  • W. Wermke: Sonographic differential diagnosis of liver diseases. Deutscher Ärzteverlag, 2006, ISBN 3-769-10433-1 .

Review article

Technical article

  • JP Grünfeld et al: Liver changes and complications in adults polycystic kidney disease. In: Adv Nephrol 14, 1985, pp. 1-20. PMID 2983516 .
  • B. Kremer and others: The cyst liver, an indication for liver transplantation or for decompressive resection? In: Zentralbl Chir 123, 1998, pp. 131-135. PMID 9556884 .
  • H. Sanchez et al .: Surgical Management of Nonparasitic Cystic Liver Disease. In: The American Journal of Surgery 161, 1991, pp. 118-119. PMID 1987844 .
  • TE Starzl et al: Liver transplantation for polycystic liver disease. In: Arch Surg 125, 1990, pp. 575-577. PMID 2331212 .
  • JN Vauthey et al: Clinical experience with adult polycystic liver disease. In: Br J Surg 79, 1992, pp. 562-565. PMID 1611453 .
  • DR Jeyarajah et al.: Liver and kidney transplantation for polycystic disease. In: Transplantation 66, 1998, pp. 529-532. PMID 9734499 .

Dissertations

Web links

Individual evidence

  1. ↑ a b c d R. T. Russell and CW Pinson: http://www.wjgnet.com/1007-9327/13/5052.asp Surgical management of polycystic liver disease. In: World J Gastroenterol 13, 2007, pp. 5052-5059.
  2. ↑ orphanet.org: Liver Disease , Polycystic Accessed November 2, 2008
  3. ^ IDA d'Agata et al .: Combined cystic disease of the liver and kidney. In: Sem Liver Dis 14, 1994, pp. 215-228. PMID 7939783
  4. ↑ a b J.Milutinovic et al.: Liver cysts in patients with autosomal dominant polycystic kidney disease. In: Am J Med 68, 1980, pp. 741-744. PMID 7377224
  5. ^ AC Ong and PC Harris: Molecular pathogenesis of ADPKD: the polycystin complex gets complex. In: Kidney Int . 67, 2005, pp. 1234-1247. PMID 15780076
  6. ↑ U. Faber: Long-term course in adult polycystic kidney degeneration after kidney transplantation. Dissertation, Heinrich Heine University Düsseldorf, 2000.
  7. ^ GT Everson et al: Polycystic disease of the liver. In: Hepatology 40, 2004, pp. 774-782. PMID 15382167
  8. ↑ JPH Drenth et al .: Germline mutations in PRKCSH are associated with autosomal dominant polycystic liver disease. In: Nature Genetics 33, 2003, pp. 345-348. PMID 12577059
  9. ^ A. Li et al.: Mutations in PRKCSH cause isolated autosomal dominant polycystic liver disease. In: Am. J. Hum. Genet. 72, 2003, pp. 691-703. PMID 12529853
  10. ↑ DM Reynolds et al.: Identification of a locus for autosomal dominant polycystic liver disease, on chromosome 19p13.2-13.1. In: Am. J. Hum. Genet. 67, 2000, pp. 1598-1604. PMID 11047756
  11. ↑ Protein Kinase C Substrates, 80-KD, Heavy Chain; PRKCSH.  In: Online Mendelian Inheritance in Man . (English), accessed November 2, 2008.
  12. ↑ S. Davila et al.: Mutations in SEC63 cause autosomal dominant polycystic liver disease. In: Nature Genetics 36, 2004, pp. 575-576. PMID 15133510
  13. ↑ E. Woollatt et al.: Human SEC63 endoplasmic reticulum membrane protein: map position 6q21. In: Chromosome Res 7, 1999, p. 77. PMID 10219736
  14. ↑ SEC63, S. CEREVISIAE, HOMOLOG OF.  In: Online Mendelian Inheritance in Man . (English), accessed November 2, 2008.
  15. ^ TY Lin et al: Treatment of non-parasitic cystic disease of the liver: a new approach to therapy with polycystic liver. In: Ann Surg 168, 1968, pp. 921-927. PMID 5684196
  16. ↑ charite.de: Minimally invasive surgery: liver cysts (no cyst liver is shown; the treatment of a solitary liver cyst is shown), viewed on November 20, 2014
  17. ↑ H. Lang et al .: Liver Transplantation in Patients with Polycystic Liver Disease. In: Transplant Proc 29, 1997, pp. 2832-2833. PMID 9365580
  18. ↑ J. Klupp et al.: Orthotopic liver transplantation for the therapy of advanced polycystic liver disease. In: Chirurg 67, 1996, pp. 515-521. PMID 8777881
  19. ^ F. Bristowe: Cystic disease of the liver associated with similar disease of the kidneys. In: Trans Pathol Soc Lond 7, 1856, pp. 229-234.