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Portal hypertension, noncirrhotic(NCPH1)

MedGen UID:
934702
•Concept ID:
C4310735
•
Disease or Syndrome
Synonyms: NCPH1; PORTAL HYPERTENSION, NONCIRRHOTIC, 1
 
Related genes: GIMAP5, DGUOK
 
Monarch Initiative: MONDO:0024193
OMIM®: 601465; 617068
OMIM® Phenotypic series: PS617068

Clinical features

From HPO
Portal hypertension
MedGen UID:
9375
•Concept ID:
C0020541
•
Disease or Syndrome
Increased pressure in the portal vein.
See: Feature record | Search on this feature
Esophageal varix
MedGen UID:
5027
•Concept ID:
C0014867
•
Disease or Syndrome
Extreme dilation of the submucusoal veins in the lower portion of the esophagus.
See: Feature record | Search on this feature
Hepatomegaly
MedGen UID:
42428
•Concept ID:
C0019209
•
Finding
Abnormally increased size of the liver.
See: Feature record | Search on this feature
Splenomegaly
MedGen UID:
52469
•Concept ID:
C0038002
•
Finding
Abnormal increased size of the spleen.
See: Feature record | Search on this feature
Elevated circulating hepatic transaminase concentration
MedGen UID:
116013
•Concept ID:
C0235996
•
Finding
Elevations of the levels of SGOT and SGPT in the serum. SGOT (serum glutamic oxaloacetic transaminase) and SGPT (serum glutamic pyruvic transaminase) are transaminases primarily found in the liver and heart and are released into the bloodstream as the result of liver or heart damage. SGOT and SGPT are used clinically mainly as markers of liver damage.
See: Feature record | Search on this feature
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Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

ACG Clinical Guideline: Disorders of the Hepatic and Mesenteric Circulation.
Simonetto DA, Singal AK, Garcia-Tsao G, Caldwell SH, Ahn J, Kamath PS
Am J Gastroenterol 2020 Jan;115(1):18-40. doi: 10.14309/ajg.0000000000000486. PMID: 31895720
Surgical management of portal hypertension in children.
de Ville de Goyet J, D'Ambrosio G, Grimaldi C
Semin Pediatr Surg 2012 Aug;21(3):219-32. doi: 10.1053/j.sempedsurg.2012.05.005. PMID: 22800975
Advances in the diagnosis and treatment of chronic granulomatous disease.
Seger RA
Curr Opin Hematol 2011 Jan;18(1):36-41. doi: 10.1097/MOH.0b013e32834115e7. PMID: 21076296

Recent clinical studies

Etiology

EASL-EASD-EASO Clinical Practice Guidelines on the management of metabolic dysfunction-associated steatotic liver disease (MASLD).
European Association for the Study of the Liver (EASL); European Association for the Study of Diabetes (EASD); European Association for the Study of Obesity (EASO)
J Hepatol 2024 Sep;81(3):492-542. Epub 2024 Jun 7 doi: 10.1016/j.jhep.2024.04.031. PMID: 38851997
Porto-sinusoidal vascular disorder.
De Gottardi A, Sempoux C, Berzigotti A
J Hepatol 2022 Oct;77(4):1124-1135. Epub 2022 Jun 9 doi: 10.1016/j.jhep.2022.05.033. PMID: 35690264
Causes and Management of Non-cirrhotic Portal Hypertension​.
Gioia S, Nardelli S, Ridola L, Riggio O
Curr Gastroenterol Rep 2020 Sep 17;22(12):56. doi: 10.1007/s11894-020-00792-0. PMID: 32940785Free PMC Article
Porto-sinusoidal vascular disease: proposal and description of a novel entity.
De Gottardi A, Rautou PE, Schouten J, Rubbia-Brandt L, Leebeek F, Trebicka J, Murad SD, Vilgrain V, Hernandez-Gea V, Nery F, Plessier A, Berzigotti A, Bioulac-Sage P, Primignani M, Semela D, Elkrief L, Bedossa P, Valla D, Garcia-Pagan JC; VALDIG group
Lancet Gastroenterol Hepatol 2019 May;4(5):399-411. doi: 10.1016/S2468-1253(19)30047-0. PMID: 30957754
Non-cirrhotic portal hypertension - diagnosis and management.
Khanna R, Sarin SK
J Hepatol 2014 Feb;60(2):421-41. Epub 2013 Aug 23 doi: 10.1016/j.jhep.2013.08.013. PMID: 23978714

Diagnosis

EASL-EASD-EASO Clinical Practice Guidelines on the management of metabolic dysfunction-associated steatotic liver disease (MASLD).
European Association for the Study of the Liver (EASL); European Association for the Study of Diabetes (EASD); European Association for the Study of Obesity (EASO)
J Hepatol 2024 Sep;81(3):492-542. Epub 2024 Jun 7 doi: 10.1016/j.jhep.2024.04.031. PMID: 38851997
Porto-sinusoidal vascular disorder.
De Gottardi A, Sempoux C, Berzigotti A
J Hepatol 2022 Oct;77(4):1124-1135. Epub 2022 Jun 9 doi: 10.1016/j.jhep.2022.05.033. PMID: 35690264
Causes and Management of Non-cirrhotic Portal Hypertension​.
Gioia S, Nardelli S, Ridola L, Riggio O
Curr Gastroenterol Rep 2020 Sep 17;22(12):56. doi: 10.1007/s11894-020-00792-0. PMID: 32940785Free PMC Article
Porto-sinusoidal vascular disease: proposal and description of a novel entity.
De Gottardi A, Rautou PE, Schouten J, Rubbia-Brandt L, Leebeek F, Trebicka J, Murad SD, Vilgrain V, Hernandez-Gea V, Nery F, Plessier A, Berzigotti A, Bioulac-Sage P, Primignani M, Semela D, Elkrief L, Bedossa P, Valla D, Garcia-Pagan JC; VALDIG group
Lancet Gastroenterol Hepatol 2019 May;4(5):399-411. doi: 10.1016/S2468-1253(19)30047-0. PMID: 30957754
Non-cirrhotic portal hypertension - diagnosis and management.
Khanna R, Sarin SK
J Hepatol 2014 Feb;60(2):421-41. Epub 2013 Aug 23 doi: 10.1016/j.jhep.2013.08.013. PMID: 23978714

Therapy

Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies.
Dana J, Debray D, Beaufrère A, Hillaire S, Fabre M, Reinhold C, Baumert TF, Berteloot L, Vilgrain V
J Hepatol 2022 Feb;76(2):420-434. Epub 2021 Oct 20 doi: 10.1016/j.jhep.2021.09.042. PMID: 34678405
Treatment of portal vein thrombosis: an updated narrative review.
Caiano LM, Riva N, Carrier M, Gatt A, Ageno W
Minerva Med 2021 Dec;112(6):713-725. Epub 2021 Apr 9 doi: 10.23736/S0026-4806.21.07526-1. PMID: 33832217
Therapeutic pipeline in nonalcoholic steatohepatitis.
Vuppalanchi R, Noureddin M, Alkhouri N, Sanyal AJ
Nat Rev Gastroenterol Hepatol 2021 Jun;18(6):373-392. Epub 2021 Feb 10 doi: 10.1038/s41575-020-00408-y. PMID: 33568794
Portopulmonary hypertension.
Mukhtar NA, Fix OK
J Clin Gastroenterol 2011 Sep;45(8):703-10. doi: 10.1097/MCG.0b013e31820656bd. PMID: 21325952
Letter: Arsenic and non-cirrhotic portal hypertension.
Datta DV
Lancet 1976 Feb 21;1(7956):433. doi: 10.1016/s0140-6736(76)90282-8. PMID: 55707

Prognosis

EASL-EASD-EASO Clinical Practice Guidelines on the Management of Metabolic Dysfunction-Associated Steatotic Liver Disease (MASLD).
European Association for the Study of the Liver (EASL); European Association for the Study of Diabetes (EASD); European Association for the Study of Obesity (EASO)
Obes Facts 2024;17(4):374-444. Epub 2024 Jun 7 doi: 10.1159/000539371. PMID: 38852583Free PMC Article
EASL-EASD-EASO Clinical Practice Guidelines on the management of metabolic dysfunction-associated steatotic liver disease (MASLD).
European Association for the Study of the Liver (EASL); European Association for the Study of Diabetes (EASD); European Association for the Study of Obesity (EASO)
J Hepatol 2024 Sep;81(3):492-542. Epub 2024 Jun 7 doi: 10.1016/j.jhep.2024.04.031. PMID: 38851997
Idiopathic Portal Hypertension.
Hernández-Gea V, Baiges A, Turon F, Garcia-Pagán JC
Hepatology 2018 Dec;68(6):2413-2423. doi: 10.1002/hep.30132. PMID: 30066417
Non-cirrhotic portal hypertension - diagnosis and management.
Khanna R, Sarin SK
J Hepatol 2014 Feb;60(2):421-41. Epub 2013 Aug 23 doi: 10.1016/j.jhep.2013.08.013. PMID: 23978714
Portopulmonary hypertension.
Mukhtar NA, Fix OK
J Clin Gastroenterol 2011 Sep;45(8):703-10. doi: 10.1097/MCG.0b013e31820656bd. PMID: 21325952

Clinical prediction guides

EASL-EASD-EASO Clinical Practice Guidelines on the Management of Metabolic Dysfunction-Associated Steatotic Liver Disease (MASLD).
European Association for the Study of the Liver (EASL); European Association for the Study of Diabetes (EASD); European Association for the Study of Obesity (EASO)
Obes Facts 2024;17(4):374-444. Epub 2024 Jun 7 doi: 10.1159/000539371. PMID: 38852583Free PMC Article
EASL-EASD-EASO Clinical Practice Guidelines on the management of metabolic dysfunction-associated steatotic liver disease (MASLD).
European Association for the Study of the Liver (EASL); European Association for the Study of Diabetes (EASD); European Association for the Study of Obesity (EASO)
J Hepatol 2024 Sep;81(3):492-542. Epub 2024 Jun 7 doi: 10.1016/j.jhep.2024.04.031. PMID: 38851997
Porto-sinusoidal vascular disorder.
De Gottardi A, Sempoux C, Berzigotti A
J Hepatol 2022 Oct;77(4):1124-1135. Epub 2022 Jun 9 doi: 10.1016/j.jhep.2022.05.033. PMID: 35690264
Idiopathic Portal Hypertension.
Hernández-Gea V, Baiges A, Turon F, Garcia-Pagán JC
Hepatology 2018 Dec;68(6):2413-2423. doi: 10.1002/hep.30132. PMID: 30066417
Portopulmonary hypertension.
Mukhtar NA, Fix OK
J Clin Gastroenterol 2011 Sep;45(8):703-10. doi: 10.1097/MCG.0b013e31820656bd. PMID: 21325952

Recent systematic reviews

Prevalence of hepatic encephalopathy in patients with non-cirrhotic portal hypertension: A systematic review and meta-analysis.
Giri S, Singh A, Angadi S, Kolhe K, Roy A
Indian J Gastroenterol 2023 Oct;42(5):642-650. Epub 2023 Aug 17 doi: 10.1007/s12664-023-01412-1. PMID: 37589913
Maternal and perinatal outcome in pregnancies complicated with portal hypertension: a systematic review and meta-analysis.
Pal K, Sadanandan DM, Gupta A, Nayak D, Pyakurel M, Keepanasseril A, Maurya DK, Nair NS, Keepanasseril A
Hepatol Int 2023 Feb;17(1):170-179. Epub 2022 Jul 8 doi: 10.1007/s12072-022-10385-w. PMID: 35802227
Systematic review and meta-analysis: impact of anti-viral therapy on portal hypertensive complications in HBV patients with advanced chronic liver disease.
Kong Y, Lv T, Li M, Zhao L, Meng T, Wu S, Wei W, Zhang Q, Chen S, You H, Lens S, Yoshiji H, Francque S, Tsochatzis E, Sarin SK, Mandorfer M, Jia J; BAVENO Cooperation: an EASL consortium
Hepatol Int 2022 Oct;16(5):1052-1063. Epub 2022 Sep 9 doi: 10.1007/s12072-022-10369-w. PMID: 36083440
Effectiveness of Terlipressin on Modulation of Portal Vein Pressure after Hepatic Resections in Non-Cirrhotic Patients. A Systematic Review and Meta-Analysis of Randomised Controlled Trials.
Gavriilidis P, Roberts KJ, Angelis N, Memeo R, Pai M, Saverio SD, Askari A, Sutcliffe RP
Chirurgia (Bucur) 2020 Nov-Dec;115(6):707-714. doi: 10.21614/chirurgia.115.6.707. PMID: 33378629
Management of acute non-cirrhotic and non-malignant portal vein thrombosis: a systematic review.
Hall TC, Garcea G, Metcalfe M, Bilku D, Dennison AR
World J Surg 2011 Nov;35(11):2510-20. doi: 10.1007/s00268-011-1198-0. PMID: 21882035

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