Hepatopulmonary syndrome medical therapy: Difference between revisions

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==Overview==
==Overview==
There is no treatment for hepatopulmonary syndrome; the mainstay of therapy is supportive care.
There is no medical treatment for hepatopulmonary syndrome. The mainstay of treatment for hepatopulmonary syndrome is [[surgery]]. [[Liver transplantation|Orthotopic liver transplantation]] is the only available treatment for patients with hepatopulmonary syndrome. Supportive therapy for hepatopulmonary syndrome includes [[Oxygen therapy|oxygen therapy.]]
 
OR
 
Supportive therapy for hepatopulmonary syndrome includes [therapy 1], [therapy 2], and [therapy 3].
 
OR
 
The majority of cases of hepatopulmonary syndrome are self-limited and require only supportive care.
 
OR
 
hepatopulmonary syndrome is a medical emergency and requires prompt treatment.
 
OR
 
The mainstay of treatment for hepatopulmonary syndrome is [therapy].
 
OR
The optimal therapy for [malignancy name] depends on the stage at diagnosis.
 
OR
 
[Therapy] is recommended among all patients who develop hepatopulmonary syndrome.
 
OR
 
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
 
OR
 
Pharmacologic medical therapies for hepatopulmonary syndrome include (either) [therapy 1], [therapy 2], and/or [therapy 3].
 
OR
 
Empiric therapy for hepatopulmonary syndrome depends on [disease factor 1] and [disease factor 2].
 
OR
 
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].


==Medical Therapy==
==Medical Therapy==
*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
*There is no medical treatment for hepatopulmonary syndrome.<ref name="pmid18509123">Rodríguez-Roisin R, Krowka MJ (2008) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=18509123 Hepatopulmonary syndrome--a liver-induced lung vascular disorder.] ''N Engl J Med'' 358 (22):2378-87. [http://dx.doi.org/10.1056/NEJMra0707185 DOI:10.1056/NEJMra0707185] PMID: [https://pubmed.gov/18509123 18509123]</ref>
*Pharmacologic medical therapies for hepatopulmonary syndrome include (either) [therapy 1], [therapy 2], and/or [therapy 3].
*Empiric therapy for hepatopulmonary syndrome depends on [disease factor 1] and [disease factor 2].
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
===Disease Name===


* '''1 Stage 1 - Name of stage'''
<ref name="pmid11003635">Fallon MB, Abrams GA (2000) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=11003635 Pulmonary dysfunction in chronic liver disease.] ''Hepatology'' 32 (4 Pt 1):859-65. [http://dx.doi.org/10.1053/jhep.2000.7519 DOI:10.1053/jhep.2000.7519] PMID: [https://pubmed.gov/11003635 11003635]</ref><ref name="pmid14762853">Krowka MJ, Mandell MS, Ramsay MA, Kawut SM, Fallon MB, Manzarbeitia C et al. (2004) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=14762853 Hepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant database.] ''Liver Transpl'' 10 (2):174-82. [http://dx.doi.org/10.1002/lt.20016 DOI:10.1002/lt.20016] PMID: [https://pubmed.gov/14762853 14762853]</ref><ref name="pmid891282">Kennedy TC, Knudson RJ (1977) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=891282 Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis.] ''Chest'' 72 (3):305-9. [http://dx.doi.org/10.1378/chest.72.3.305 DOI:10.1378/chest.72.3.305] PMID: [https://pubmed.gov/891282 891282]</ref><ref name="pmid27326810">Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA et al. (2016) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=27326810 International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension.] ''Transplantation'' 100 (7):1440-52. [http://dx.doi.org/10.1097/TP.0000000000001229 DOI:10.1097/TP.0000000000001229] PMID: [https://pubmed.gov/27326810 27326810]</ref><ref name="pmid8101797">Krowka MJ, Dickson ER, Cortese DA (1993) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8101797 Hepatopulmonary syndrome. Clinical observations and lack of therapeutic response to somatostatin analogue.] ''Chest'' 104 (2):515-21. [http://dx.doi.org/10.1378/chest.104.2.515 DOI:10.1378/chest.104.2.515] PMID: [https://pubmed.gov/8101797 8101797]</ref><ref name="pmid15828054">Swanson KL, Wiesner RH, Krowka MJ (2005) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=15828054 Natural history of hepatopulmonary syndrome: Impact of liver transplantation.] ''Hepatology'' 41 (5):1122-9. [http://dx.doi.org/10.1002/hep.20658 DOI:10.1002/hep.20658] PMID: [https://pubmed.gov/15828054 15828054]</ref>
** 1.1 '''Specific Organ system involved 1'''
*** 1.1.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' 
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
*** 1.1.2 '''Pediatric'''
**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose) 
***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
****1.1.2.2 (Specific population e.g. '<nowiki/>'''''children < 8 years of age'''''')
***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose) 
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
** 1.2 '''Specific Organ system involved 2'''
*** 1.2.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
*** 1.2.2  '''Pediatric'''
**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)


* 2 '''Stage 2 - Name of stage'''
*The mainstay of treatment for hepatopulmonary syndrome is [[surgery]].
** 2.1 '''Specific Organ system involved 1 '''
*[[Liver transplantation|Orthotopic liver transplantation]] is the only available treatment for patients with hepatopulmonary syndrome.
**: '''Note (1):'''
*Supportive therapy for hepatopulmonary syndrome includes [[oxygen therapy]].
**: '''Note (2)''':
*Nevertheless, norfloxacin, pentoxifylline, inhaled N(G)-nitro-L-arginine methyl ester, nitric oxide inhalation and methylene blue injection, are among suggested and studied medical treatments for HPS. Most of them failed to show efficacy and hence, non of them yet approved as a standard treatment for HPS.<ref name="pmid20816858">Gupta S, Faughnan ME, Lilly L, Hutchison S, Fowler R, Bayoumi AM (2010) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=20816858 Norfloxacin therapy for hepatopulmonary syndrome: a pilot randomized controlled trial.] ''Clin Gastroenterol Hepatol'' 8 (12):1095-8. [http://dx.doi.org/10.1016/j.cgh.2010.08.011 DOI:10.1016/j.cgh.2010.08.011] PMID: [https://pubmed.gov/20816858 20816858]</ref><ref name="pmid18779471">Gupta LB, Kumar A, Jaiswal AK, Yusuf J, Mehta V, Tyagi S et al. (2008) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=18779471 Pentoxifylline therapy for hepatopulmonary syndrome: a pilot study.] ''Arch Intern Med'' 168 (16):1820-3. [http://dx.doi.org/10.1001/archinte.168.16.1820 DOI:10.1001/archinte.168.16.1820] PMID: [https://pubmed.gov/18779471 18779471]</ref><ref name="pmid18668653">Tanikella R, Philips GM, Faulk DK, Kawut SM, Fallon MB (2008) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=18668653 Pilot study of pentoxifylline in hepatopulmonary syndrome.] ''Liver Transpl'' 14 (8):1199-203. [http://dx.doi.org/10.1002/lt.21482 DOI:10.1002/lt.21482] PMID: [https://pubmed.gov/18668653 18668653]</ref><ref name="pmid12853200">Brussino L, Bucca C, Morello M, Scappaticci E, Mauro M, Rolla G (2003) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=12853200 Effect on dyspnoea and hypoxaemia of inhaled N(G)-nitro-L-arginine methyl ester in hepatopulmonary syndrome.] ''Lancet'' 362 (9377):43-4. [http://dx.doi.org/10.1016/S0140-6736(03)13807-X DOI:10.1016/S0140-6736(03)13807-X] PMID: [https://pubmed.gov/12853200 12853200]</ref><ref name="pmid11074903">Schenk P, Madl C, Rezaie-Majd S, Lehr S, Müller C (2000) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=11074903 Methylene blue improves the hepatopulmonary syndrome.] ''Ann Intern Med'' 133 (9):701-6. [http://dx.doi.org/10.7326/0003-4819-133-9-200011070-00012 DOI:10.7326/0003-4819-133-9-200011070-00012] PMID: [https://pubmed.gov/11074903 11074903]</ref><ref name="pmid11497151">Jounieaux V, Leleu O, Mayeux I (2001) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=11497151 Cardiopulmonary effects of nitric oxide inhalation and methylene blue injection in hepatopulmonary syndrome.] ''Intensive Care Med'' 27 (6):1103-4. PMID: [https://pubmed.gov/11497151 11497151]</ref>
**: '''Note (3):'''
*** 2.1.1 '''Adult'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
*** 2.1.2 '''Pediatric'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
***** Alternative regimen (2): [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '<nowiki/>'''''(Contraindications/specific instructions)''''''
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
** 2.2  '<nowiki/>'''''Other Organ system involved 2''''''
**: '''Note (1):'''
**: '''Note (2)''':
**: '''Note (3):'''
*** 2.2.1 '''Adult'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
*** 2.2.2 '''Pediatric'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
***** Alternative regimen (2): [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
**** Oral regimen
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)


==References==
==References==
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{{WH}}
{{WH}}
{{WS}}
{{WS}}
[[Category: (name of the system)]]
[[Category:Surgery]]
[[Category:Medicine]]
[[Category:Pulmonology]]
[[Category:Cardiology]]
[[Category:Gastroentrology]]
[[Category:Up-To-Date]]

Latest revision as of 18:03, 6 September 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]

Overview

There is no medical treatment for hepatopulmonary syndrome. The mainstay of treatment for hepatopulmonary syndrome is surgery. Orthotopic liver transplantation is the only available treatment for patients with hepatopulmonary syndrome. Supportive therapy for hepatopulmonary syndrome includes oxygen therapy.

Medical Therapy

  • There is no medical treatment for hepatopulmonary syndrome.[1]

[2][3][4][5][6][7]

  • The mainstay of treatment for hepatopulmonary syndrome is surgery.
  • Orthotopic liver transplantation is the only available treatment for patients with hepatopulmonary syndrome.
  • Supportive therapy for hepatopulmonary syndrome includes oxygen therapy.
  • Nevertheless, norfloxacin, pentoxifylline, inhaled N(G)-nitro-L-arginine methyl ester, nitric oxide inhalation and methylene blue injection, are among suggested and studied medical treatments for HPS. Most of them failed to show efficacy and hence, non of them yet approved as a standard treatment for HPS.[8][9][10][11][12][13]

References

  1. ↑ Rodríguez-Roisin R, Krowka MJ (2008) Hepatopulmonary syndrome--a liver-induced lung vascular disorder. N Engl J Med 358 (22):2378-87. DOI:10.1056/NEJMra0707185 PMID: 18509123
  2. ↑ Fallon MB, Abrams GA (2000) Pulmonary dysfunction in chronic liver disease. Hepatology 32 (4 Pt 1):859-65. DOI:10.1053/jhep.2000.7519 PMID: 11003635
  3. ↑ Krowka MJ, Mandell MS, Ramsay MA, Kawut SM, Fallon MB, Manzarbeitia C et al. (2004) Hepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant database. Liver Transpl 10 (2):174-82. DOI:10.1002/lt.20016 PMID: 14762853
  4. ↑ Kennedy TC, Knudson RJ (1977) Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis. Chest 72 (3):305-9. DOI:10.1378/chest.72.3.305 PMID: 891282
  5. ↑ Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA et al. (2016) International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation 100 (7):1440-52. DOI:10.1097/TP.0000000000001229 PMID: 27326810
  6. ↑ Krowka MJ, Dickson ER, Cortese DA (1993) Hepatopulmonary syndrome. Clinical observations and lack of therapeutic response to somatostatin analogue. Chest 104 (2):515-21. DOI:10.1378/chest.104.2.515 PMID: 8101797
  7. ↑ Swanson KL, Wiesner RH, Krowka MJ (2005) Natural history of hepatopulmonary syndrome: Impact of liver transplantation. Hepatology 41 (5):1122-9. DOI:10.1002/hep.20658 PMID: 15828054
  8. ↑ Gupta S, Faughnan ME, Lilly L, Hutchison S, Fowler R, Bayoumi AM (2010) Norfloxacin therapy for hepatopulmonary syndrome: a pilot randomized controlled trial. Clin Gastroenterol Hepatol 8 (12):1095-8. DOI:10.1016/j.cgh.2010.08.011 PMID: 20816858
  9. ↑ Gupta LB, Kumar A, Jaiswal AK, Yusuf J, Mehta V, Tyagi S et al. (2008) Pentoxifylline therapy for hepatopulmonary syndrome: a pilot study. Arch Intern Med 168 (16):1820-3. DOI:10.1001/archinte.168.16.1820 PMID: 18779471
  10. ↑ Tanikella R, Philips GM, Faulk DK, Kawut SM, Fallon MB (2008) Pilot study of pentoxifylline in hepatopulmonary syndrome. Liver Transpl 14 (8):1199-203. DOI:10.1002/lt.21482 PMID: 18668653
  11. ↑ Brussino L, Bucca C, Morello M, Scappaticci E, Mauro M, Rolla G (2003) Effect on dyspnoea and hypoxaemia of inhaled N(G)-nitro-L-arginine methyl ester in hepatopulmonary syndrome. Lancet 362 (9377):43-4. DOI:10.1016/S0140-6736(03)13807-X PMID: 12853200
  12. ↑ Schenk P, Madl C, Rezaie-Majd S, Lehr S, Müller C (2000) Methylene blue improves the hepatopulmonary syndrome. Ann Intern Med 133 (9):701-6. DOI:10.7326/0003-4819-133-9-200011070-00012 PMID: 11074903
  13. ↑ Jounieaux V, Leleu O, Mayeux I (2001) Cardiopulmonary effects of nitric oxide inhalation and methylene blue injection in hepatopulmonary syndrome. Intensive Care Med 27 (6):1103-4. PMID: 11497151

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