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Inspissated bile syndrome

MedGen UID:
473009
•Concept ID:
C0270217
•
Disease or Syndrome
Synonyms: Inspissated Bile Syndrome; Perinatal Jaundice due to Inspissated Bile Syndrome; Perinatal Jaundice Related to Inspissated Bile Syndrome
SNOMED CT: Inspissated bile syndrome (1295333008)

Definition

A syndrome characterized by persistent jaundice, hemolytic anemia, and hepatomegaly in a newborn. It is characterized by the presence of inspissated bile plugs in the bile ducts. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVInspissated bile syndrome

Professional guidelines

PubMed

Laparoscopic cholecystostomy and bile duct lavage for treatment of inspissated bile syndrome: a single-center experience.
Yeh ML, Chang PC
World J Pediatr 2012 Feb;8(1):88; author reply 88. doi: 10.1007/s12519-012-0339-3. PMID: 22282387
Laparoscopic cholecystostomy and bile duct lavage for treatment of inspissated bile syndrome: a single-center experience.
Gao ZG, Shao M, Xiong QX, Tou JF, Liu WG
World J Pediatr 2011 Aug;7(3):269-71. Epub 2011 Aug 7 doi: 10.1007/s12519-011-0321-5. PMID: 21822994
Diagnostic utility of hepatobiliary scintigraphy with 99mTc-DISIDA in neonatal cholestasis.
Spivak W, Sarkar S, Winter D, Glassman M, Donlon E, Tucker KJ
J Pediatr 1987 Jun;110(6):855-61. doi: 10.1016/s0022-3476(87)80396-7. PMID: 3585600

Recent clinical studies

Etiology

Parameters that help to differentiate biliary atresia from other diseases.
Hayashida M, Matsuura T, Kinoshita Y, Esumi G, Yoshimaru K, Yanagi Y, Takahashi Y, Taguchi T
Pediatr Int 2017 Dec;59(12):1261-1265. doi: 10.1111/ped.13392. PMID: 28802084
Inspissated bile syndrome; Safe and effective minimally invasive treatment with percutaneous cholecystostomy in neonates and infants.
Bollu BK, Dawrant MJ, Thacker K, Thomas G, Chenapragadda M, Gaskin K, Shun A
J Pediatr Surg 2016 Dec;51(12):2119-2122. Epub 2016 Sep 20 doi: 10.1016/j.jpedsurg.2016.09.053. PMID: 27712892
Surgical treatment of inspissated bile syndrome using a 2-stage pure laparoscopic approach: a case report.
Lieber J, Piersma FE, Sturm E, Schäfer JF, Fuchs J, Szavay PO
J Pediatr Surg 2012 Dec;47(12):e47-50. doi: 10.1016/j.jpedsurg.2012.09.043. PMID: 23217917
Obstructive jaundice in neonates.
Wani BN, Jajoo SN
Trop Gastroenterol 2009 Oct-Dec;30(4):195-200. PMID: 20426278
Inspissated bile syndrome in a neonate treated with cefotaxime: sonographic aid to diagnosis, management, and follow-up.
Miloh T, Rosenberg HK, Kochin I, Kerkar N
J Ultrasound Med 2009 Apr;28(4):541-4. doi: 10.7863/jum.2009.28.4.541. PMID: 19321684

Diagnosis

Diseases of bile duct in children.
Eiamkulbutr S, Tubjareon C, Sanpavat A, Phewplung T, Srisan N, Sintusek P
World J Gastroenterol 2024 Mar 7;30(9):1043-1072. doi: 10.3748/wjg.v30.i9.1043. PMID: 38577180Free PMC Article
Parameters that help to differentiate biliary atresia from other diseases.
Hayashida M, Matsuura T, Kinoshita Y, Esumi G, Yoshimaru K, Yanagi Y, Takahashi Y, Taguchi T
Pediatr Int 2017 Dec;59(12):1261-1265. doi: 10.1111/ped.13392. PMID: 28802084
Obstructive jaundice in neonates.
Wani BN, Jajoo SN
Trop Gastroenterol 2009 Oct-Dec;30(4):195-200. PMID: 20426278
Congenital biliary tract disease.
Karrer FM, Hall RJ, Stewart BA, Lilly JR
Surg Clin North Am 1990 Dec;70(6):1403-18. doi: 10.1016/s0039-6109(16)45291-6. PMID: 2247822
The clinical spectrum of anomalous pancreatobiliary junction.
Kochhar R, Nagi B, Chawla S, Das K, Rao KL, Mitra SK, Mehta SK
Surg Endosc 1989;3(2):83-6. doi: 10.1007/BF00590906. PMID: 2772805

Therapy

Association of N-acetylcysteine and glucagon during percutaneous cholangiography in the treatment of inspissated bile syndrome.
Berrani H, Vasies I, Cron J, Bachy B, Le Dosseur P, Mouterde O
Arch Pediatr 2015 Mar;22(3):300-2. Epub 2015 Jan 19 doi: 10.1016/j.arcped.2014.12.007. PMID: 25612875
Inspissated bile syndrome in a neonate treated with cefotaxime: sonographic aid to diagnosis, management, and follow-up.
Miloh T, Rosenberg HK, Kochin I, Kerkar N
J Ultrasound Med 2009 Apr;28(4):541-4. doi: 10.7863/jum.2009.28.4.541. PMID: 19321684
Biliary infusion therapy in the inspissated bile syndrome of cystic fibrosis.
Evans JS, George DE, Mollit D
J Pediatr Gastroenterol Nutr 1991 Jan;12(1):131-5. doi: 10.1097/00005176-199101000-00024. PMID: 2061768
A prospective analysis of cholestasis in infants supported with extracorporeal membrane oxygenation.
Shneider B, Cronin J, Van Marter L, Maller E, Truog R, Jacobson M, Kevy S
J Pediatr Gastroenterol Nutr 1991 Oct;13(3):285-9. doi: 10.1097/00005176-199110000-00008. PMID: 1791505

Prognosis

Diseases of bile duct in children.
Eiamkulbutr S, Tubjareon C, Sanpavat A, Phewplung T, Srisan N, Sintusek P
World J Gastroenterol 2024 Mar 7;30(9):1043-1072. doi: 10.3748/wjg.v30.i9.1043. PMID: 38577180Free PMC Article
Surgical treatment of inspissated bile syndrome using a 2-stage pure laparoscopic approach: a case report.
Lieber J, Piersma FE, Sturm E, Schäfer JF, Fuchs J, Szavay PO
J Pediatr Surg 2012 Dec;47(12):e47-50. doi: 10.1016/j.jpedsurg.2012.09.043. PMID: 23217917
Inspissated bile syndrome in a neonate treated with cefotaxime: sonographic aid to diagnosis, management, and follow-up.
Miloh T, Rosenberg HK, Kochin I, Kerkar N
J Ultrasound Med 2009 Apr;28(4):541-4. doi: 10.7863/jum.2009.28.4.541. PMID: 19321684
The spectrum of surgical jaundice in infancy.
Davenport M, Betalli P, D'Antiga L, Cheeseman P, Mieli-Vergani G, Howard ER
J Pediatr Surg 2003 Oct;38(10):1471-9. doi: 10.1016/s0022-3468(03)00498-6. PMID: 14577070
Duodenal tube test in the diagnosis of biliary atresia.
Larrosa-Haro A, Caro-López AM, Coello-Ramírez P, Zavala-Ocampo J, Vázquez-Camacho G
J Pediatr Gastroenterol Nutr 2001 Mar;32(3):311-5. doi: 10.1097/00005176-200103000-00015. PMID: 11345182

Clinical prediction guides

A 6-Year-Old Child With Citrin Deficiency and Advanced Hepatocellular Carcinoma.
Wang L, Wang L, Zhu S, Zhang M, Dong Y, Wang FS
Pediatrics 2019 Jan;143(1) doi: 10.1542/peds.2018-1931. PMID: 30591617
Predictive value of bile duct dimensions measured by ultrasound in neonates presenting with cholestasis.
Fitzpatrick E, Jardine R, Farrant P, Karani J, Davenport M, Mieli-Vergani G, Baker A
J Pediatr Gastroenterol Nutr 2010 Jul;51(1):55-60. doi: 10.1097/MPG.0b013e3181d1b226. PMID: 20543724
Expression of the interferon-induced Mx proteins in biliary atresia.
Al-Masri AN, Flemming P, Rodeck B, Melter M, Leonhardt J, Petersen C
J Pediatr Surg 2006 Jun;41(6):1139-43. doi: 10.1016/j.jpedsurg.2006.02.022. PMID: 16769349
Duodenal tube test in the diagnosis of biliary atresia.
Larrosa-Haro A, Caro-López AM, Coello-Ramírez P, Zavala-Ocampo J, Vázquez-Camacho G
J Pediatr Gastroenterol Nutr 2001 Mar;32(3):311-5. doi: 10.1097/00005176-200103000-00015. PMID: 11345182
Extraintestinal rotavirus infections in children with immunodeficiency.
Gilger MA, Matson DO, Conner ME, Rosenblatt HM, Finegold MJ, Estes MK
J Pediatr 1992 Jun;120(6):912-7. doi: 10.1016/s0022-3476(05)81959-6. PMID: 1317419

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