---
abstract: 'Estimated incidence of Crigler-Najjar syndrome(CNS) is 1 case per 1,000,000 births(1 million). The overall prevalence of CN syndrome is unknown, with only several hundred people reported to have this disease. It is interestingly very rare to encounter a pregnant adult women with congenital jaundice. Pregnancy in CN type II patients is a diagnostic and a therapeutic challenge because of the high risk of bilirubin encephalopathy with serious neurological damage as life-threatening complications for the fetus. To date 8 pregnancy outcome have been reported from 5 women and we report the6 woman with a successful 9 th pregnancy outcome. We have discussed detail history, presentation and management during pregnancy and care of the new born.'
altloc:
- http://www.ojhas.org/issue43/2012-3-12.htm
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creators_id:
- shakuntala_pn@yahoo.com
- ~
- ~
- ~
creators_name:
- family: Shakuntala
given: PN
honourific: ''
lineage: ''
- family: Poornima
given: M
honourific: ''
lineage: ''
- family: Padmalatha
given: VV
honourific: ''
lineage: ''
- family: Rao
given: SR
honourific: ''
lineage: ''
date: 2012-10-25
date_type: published
datestamp: 2013-05-04 23:08:47
department: ~
dir: disk0/00/00/89/22
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editors_id:
- Kakkilaya BS
editors_name:
- family: Kakkilaya
given: Srinivas
honourific: Dr
lineage: ''
eprint_status: archive
eprintid: 8922
fileinfo: /style/images/fileicons/application_pdf.png;/8922/1/2012-3-12.pdf
full_text_status: public
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keywords: Crigler Najjer Syndrome type II; Unconjugated hyperbilurubinemia; Phenobarbitone; Phototherapy.
lastmod: 2013-05-04 23:08:47
latitude: ~
longitude: ~
metadata_visibility: show
note: ~
number: 3(12)
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pubdom: TRUE
publication: Online Journal of Health and Allied Sciences
publisher: Kakkilaya BS
refereed: TRUE
referencetext: "1.\tTukey RH, Strassburg CP. Human UDP-glucuronosyltransferases: metabolism, expression, and disease. Annu Rev Pharmacol Toxicol 2000;40: 581–616.\r\n2.\tCrigler JF Jr, Najjar VA: Congenital familial nonhemolytic jaundice with kernicterus; a new clinical entity. AMA Am J Dis Child 1952;83:259–260.\r\n3.\tArias M. Chronic unconjugated hyperbilirubinemia without overt signs of hemolysis in adolescents and adults. J Clin Invest (NY) 1962;41:2233–2245.\r\n4.\tGupta R, Parashar Y. Crigler-Najjar syndrome type II. Indian J Pediatr 2004;71:1043.\r\n5.\tTrotman BW, Shaw L, Roy-Chowdhury J, Malet PF, Rosato EF. Effect of Phenobarbital on serum and biliary parameters in a patient with Crigler-Najjar syndrome, type II and acquired cholestasis. Dig Dis Sci 1983;28:753–762.\r\n6.\tCosta E. Hematologically important mutations: bilirubin UDP-glucuronosyltransferase gene mutations in Gilbert and Crigler- Najjar syndromes. Blood Cells Mol Dis2006;36:77–80.\r\n7.\tSeppen J, Bosma PJ, Goldhoorn BG, Bakker CT, Chowdhury JR, Chowdhury NR, Jansen PL, Oude Elferink RP. Discrimination between Crigler-Najjar type I and II by expression of mutant bilirubin uridine diphosphate-glucuronosyltransferase. J Clin Invest 1994;94:2385–2391.\r\n8.\tRitter JK, Yeatman MT, Ferreira P, Owens IS. Identification of a genetic alteration in the code for bilirubin UDP-glucuronosyltransferase in the UGT1 gene complex of a Crigler-Najjar type I patient. J Clin Invest 1992;90:150–155.\r\n9.\tTaylor WG, Walkinshaw SA, Farquharson RG, Fisken RA, Gilmore IT: Pregnancy in Crigler-Najjar syndrome. Case report. Br J Obstet Gynaecol 1991;98:1290–1291.\r\n10.\tSmith JF, Baker JM. Crigler-Najjar disease in pregnancy. Obstet Gynecol 1994;84:670–672.\r\n11.\tIto T, Katagiri C, Ikeno S, Takahashi H, Nagata N, Terakawa N. Phenobarbital following phototherapy for Crigler-Najjar syndrome type II with good fetal outcome: a case report. J Obstet Gynaecol Res 2001;27:33–35.\r\n12.\tHolstein A, Plaschke A, Lohse P, et al. Successful photo- and phenobarbital ther- apy during pregnancy in a woman with Crigler–Najjar syndrome type II. Scand J Gastroenterol 2005;40:1124–1126.\r\n13.\tPinkee S, Renu A, Bharati M. Crigler-Najjar syndrome with pregnancy. J Obstet Gynecol India 2005;55:270–271.\r\n14.\tHolstein A, Bryan CS. Three consecutive pregnancies in a woman with Crigler–Najjar syndrome type II with good maternal and neonatal outcomes. Dig Liver Dis. 2010, doi:10.1016/j.dld.2010.08.003\r\n15.\tPassuello V, Puhl AG, Wirth S, Steiner E, Skala C, Koelbl H, Kohlschmidt N. Pregnancy Outcome in Maternal Crigler-Najjar Syndrome Type II: A Case Report and Systematic Review of the Literature. Fetal Diagn Ther 2009;26:121–126 DOI: 10.1159/000238122\r\n16.\tHannam S, Moriaty P, O’Reilly et al. Normal neurological outcome in two infants treated with exchange transfusions born to mothers with Crigler-Najjar Type 1 disorder.Eur J Pediatr 2009;168:427-429.\r\n17.\tWielsma S, Kerkmeijer L, Bekkers R, Pyman J, Tan J, Quinn M. Persistent trophoblast disease following partial molar pregnancy. Aust N Z J Obstet Gynaecol. April 2006;46(2):119–123."
relation_type: []
relation_uri: []
reportno: ~
rev_number: 9
series: ~
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status_changed: 2013-05-04 23:08:47
subjects:
- OJHAS
succeeds: ~
suggestions: ~
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title: Successful Pregnancy Outcome In Maternal Crigler Najjar Syndrome Type II.
type: journale
userid: 4338
volume: 11