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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:
338525
•Concept ID:
C1848701
•
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

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
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
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
Noncirrhotic portal hypertension.
Da BL, Koh C, Heller T
Curr Opin Gastroenterol 2018 May;34(3):140-145. doi: 10.1097/MOG.0000000000000433. PMID: 29465431
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

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
Noncirrhotic portal hypertension.
Da BL, Koh C, Heller T
Curr Opin Gastroenterol 2018 May;34(3):140-145. doi: 10.1097/MOG.0000000000000433. PMID: 29465431
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

Diagnosis and management of ectopic varices in portal hypertension.
Tranah TH, Nayagam JS, Gregory S, Hughes S, Patch D, Tripathi D, Shawcross DL, Joshi D
Lancet Gastroenterol Hepatol 2023 Nov;8(11):1046-1056. Epub 2023 Sep 9 doi: 10.1016/S2468-1253(23)00209-1. PMID: 37683687
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

Prognosis

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.
Sarin SK, Khanna R
Clin Liver Dis 2014 May;18(2):451-76. doi: 10.1016/j.cld.2014.01.009. PMID: 24679506
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
Idiopathic noncirrhotic portal hypertension.
Schouten JN, Garcia-Pagan JC, Valla DC, Janssen HL
Hepatology 2011 Sep 2;54(3):1071-81. Epub 2011 Jul 21 doi: 10.1002/hep.24422. PMID: 21574171
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

Performance of non-invasive tests and histology for the prediction of clinical outcomes in patients with non-alcoholic fatty liver disease: an individual participant data meta-analysis.
Mózes FE, Lee JA, Vali Y, Alzoubi O, Staufer K, Trauner M, Paternostro R, Stauber RE, Holleboom AG, van Dijk AM, Mak AL, Boursier J, de Saint Loup M, Shima T, Bugianesi E, Gaia S, Armandi A, Shalimar, Lupșor-Platon M, Wong VW, Li G, Wong GL, Cobbold J, Karlas T, Wiegand J, Sebastiani G, Tsochatzis E, Liguori A, Yoneda M, Nakajima A, Hagström H, Akbari C, Hirooka M, Chan WK, Mahadeva S, Rajaram R, Zheng MH, George J, Eslam M, Petta S, Pennisi G, Viganò M, Ridolfo S, Aithal GP, Palaniyappan N, Lee DH, Ekstedt M, Nasr P, Cassinotto C, de Lédinghen V, Berzigotti A, Mendoza YP, Noureddin M, Truong E, Fournier-Poizat C, Geier A, Martic M, Tuthill T, Anstee QM, Harrison SA, Bossuyt PM, Pavlides M; LITMUS investigators
Lancet Gastroenterol Hepatol 2023 Aug;8(8):704-713. Epub 2023 Jun 5 doi: 10.1016/S2468-1253(23)00141-3. PMID: 37290471
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
Portal hypertensive gastropathy.
Negreanu L, Buşegeanu C, Trandafir D, Dragomir P, Udeanu M, Fierbinţeanu-Braticevici C, Andronescu D
Rom J Intern Med 2005;43(1-2):3-8. PMID: 16739861

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
Noninvasive differentiation of porto-sinusoidal vascular disorder from cirrhosis: a systematic review.
Giri S, Singh A, Roy A, Patel RK, Tripathy T, Angadi S
Abdom Radiol (NY) 2023 Jul;48(7):2340-2348. Epub 2023 Apr 29 doi: 10.1007/s00261-023-03927-9. PMID: 37119294
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
Surgical shunts for extrahepatic portal vein obstruction in pediatric patients: a systematic review.
Zielsdorf S, Narayanan L, Kantymyr S, Barbetta A, Kwon Y, Etesami K, Genyk Y, Emamaullee J
HPB (Oxford) 2021 May;23(5):656-665. Epub 2020 Dec 30 doi: 10.1016/j.hpb.2020.11.1149. PMID: 33388243
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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