Hepatopulmonary syndrome

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Hepatopulmonary syndrome

overview

Hepatopulmonary (hep-uh-toe-POOL-moe-nar-e) syndrome is a rare disorder that affects the lungs of people with advanced liver disease. Hepatopulmonary syndrome is caused by the expansion (dilatation) and increase in blood vessels in the lungs, making it difficult for red blood cells to absorb oxygen properly. As a result, the lungs cannot supply the body with enough oxygen, resulting in low oxygen levels (hypoxemia).

How exactly the liver disease is related to the lung disease is not yet known. A liver transplant is the only cure for hepatopulmonary syndrome.

Symptoms

Most people with hepatopulmonary syndrome have no symptoms. If symptoms occur, they may include:

  • Kurzatmigkeit, besonders beim Sitzen oder Stehen
  • Schlagen der Finger, bei dem sich die Fingerspitzen spreizen und runder als normal werden
  • Geplatzte Blutgefäße unter der Haut (Spinnenangiom)
  • Bläuliche Verfärbung der Lippen und der Haut (Zyanose)

Causes

Hepatopulmonary syndrome is caused when the blood vessels in and around the lungs widen (dilate), affecting the amount of oxygen that passes from the lungs into the bloodstream. What causes this abnormality remains unclear, and it is not known why some people with liver disease develop hepatopulmonary syndrome and others do not.

Treatment of hepatopulmonary syndrome

Sources:

  1. Kellermann RD, et al. Zirrhose. In: Conn’s Current Therapy 2019. Elsevier; 2019. https://www.clinicalkey.com. Abgerufen am 9. Dezember 2019.
  2. Ferri FF. Hepatopulmonales Syndrom. In: Ferri’s Clinical Advisor 2020. Elsevier; 2020. https://www.clinicalkey.com. Abgerufen am 9. Dezember 2019.
  3. Hepatopulmonales Syndrom. Nationale Organisation für seltene Erkrankungen. https://rarediseases.org/rare-diseases/hepatopulmonary-syndrome/. Abgerufen am 9. Dezember 2019.
  4. Hepatopulmonales Syndrom. Merck Manual Professional-Version. https://www.merckmanuals.com/professional/pulmonary-disorders/pulmonary-hypertension/hepatopulmonary-syndrome#. Abgerufen am 9. Dezember 2019.
  5. Braun AY. Allscripts EPSi. Mayo-Klinik. 14. November 2019.