Page last updated: 2024-11-02

phenobarbital and Crigler Najjar Syndrome

phenobarbital has been researched along with Crigler Najjar Syndrome in 37 studies

Phenobarbital: A barbituric acid derivative that acts as a nonselective central nervous system depressant. It potentiates GAMMA-AMINOBUTYRIC ACID action on GABA-A RECEPTORS, and modulates chloride currents through receptor channels. It also inhibits glutamate induced depolarizations.
phenobarbital : A member of the class of barbiturates, the structure of which is that of barbituric acid substituted at C-5 by ethyl and phenyl groups.

Research Excerpts

ExcerptRelevanceReference
"Several mutations of bilirubin uridine diphosphate-glucuronosyltransferase gene (UGT1A1) have been reported in patients with unconjugated hyperbilirubinemia."8.12Gilbert or Crigler-Najjar syndrome? Neonatal severe unconjugated hyperbilirubinemia with P364L UGT1A1 homozygosity. ( Civeriati, D; Cozzi, L; Degrassi, I; Nebbia, G; Nuti, F; Paolella, G, 2022)
"Crigler-Najjar syndrome (CN) type II is a congenital disease with unconjugated hyperbilirubinemia due to a deficiency of uridine 5'-diphospho-glucuronosyltransferase 1A1."7.83Reduction of hyperbilirubinemia with hypericum extract (St. John's Wort) in a patient with Crigler-Najjar syndrome type II. ( Hammann, F; Haschke, M; Krähenbühl, S; Kummer, O, 2016)
" We report the case of a Gly71-->Arg/Tyr486-->Asp homozygous mother of East Asian descent, who was treated with phototherapy during embryogenesis and with phenobarbital during the rest of the pregnancy."7.73Successful photo-and phenobarbital therapy during pregnancy in a woman with Crigler-Najjar syndrome type II. ( Egberts, EH; Holstein, A; Lohse, P; Plaschke, A, 2005)
"The effect of phenobarbital treatment on bilirubin metabolism and bile secretion was studied in a patient with Crigler-Najjar syndrome, type II and acquired cholestasis."7.66Effect of phenobarbital on serum and biliary parameters in a patient with Crigler-Najjar syndrome, type II and acquired cholestasis. ( Malet, PF; Rosato, EF; Roy-Chowdhury, J; Shaw, L; Trotman, BW, 1983)
"A 37-year-old mother with CNS type II was treated with phenobarbital during her pregnancy and her bilirubin levels were monitored."4.85Pregnancy outcome in maternal Crigler-Najjar syndrome type II: a case report and systematic review of the literature. ( Koelbl, H; Kohlschmidt, N; Passuello, V; Puhl, AG; Skala, C; Steiner, E; Wirth, S, 2009)
"Several mutations of bilirubin uridine diphosphate-glucuronosyltransferase gene (UGT1A1) have been reported in patients with unconjugated hyperbilirubinemia."4.12Gilbert or Crigler-Najjar syndrome? Neonatal severe unconjugated hyperbilirubinemia with P364L UGT1A1 homozygosity. ( Civeriati, D; Cozzi, L; Degrassi, I; Nebbia, G; Nuti, F; Paolella, G, 2022)
" Patients with Crigglar-Najjar syndrome type II respond to phenobarbital therapy which decreases their chances of getting bilirubinemia by 60-70% in 3 weeks."3.88Crigler-Najjar Syndrome Type II Diagnosed in a Patient with Jaundice Since Birth. ( Ameer, A; Attiq, H; Imran, M; Liaqat, A; Shahid, A, 2018)
"Crigler-Najjar syndrome (CN) type II is a congenital disease with unconjugated hyperbilirubinemia due to a deficiency of uridine 5'-diphospho-glucuronosyltransferase 1A1."3.83Reduction of hyperbilirubinemia with hypericum extract (St. John's Wort) in a patient with Crigler-Najjar syndrome type II. ( Hammann, F; Haschke, M; Krähenbühl, S; Kummer, O, 2016)
" We report the case of a Gly71-->Arg/Tyr486-->Asp homozygous mother of East Asian descent, who was treated with phototherapy during embryogenesis and with phenobarbital during the rest of the pregnancy."3.73Successful photo-and phenobarbital therapy during pregnancy in a woman with Crigler-Najjar syndrome type II. ( Egberts, EH; Holstein, A; Lohse, P; Plaschke, A, 2005)
"The effect of phenobarbital treatment on bilirubin metabolism and bile secretion was studied in a patient with Crigler-Najjar syndrome, type II and acquired cholestasis."3.66Effect of phenobarbital on serum and biliary parameters in a patient with Crigler-Najjar syndrome, type II and acquired cholestasis. ( Malet, PF; Rosato, EF; Roy-Chowdhury, J; Shaw, L; Trotman, BW, 1983)
"Orlistat treatment increases fecal fat excretion and decreases plasma unconjugated bilirubin (UCB) concentrations in Gunn rats, the animal model for CN disease."2.73Orlistat treatment of unconjugated hyperbilirubinemia in Crigler-Najjar disease: a randomized controlled trial. ( Hafkamp, AM; Nelisse-Haak, R; Oude Elferink, RP; Sinaasappel, M; Verkade, HJ, 2007)
"Clinically apparent jaundice is unusual in patients with beta-thalassemia major."1.36Persistent jaundice in an infant with homozygous beta thalassemia due to co-inherited Crigler-Najjar syndrome. ( Aggarwal, V; Aneja, S; Fabiano, C; Sammarco, P; Seth, A; Sharma, S, 2010)
"Phenobarbital treatment of Gunn recipients of jejunal transplants from Wistar rats normalizes serum bilirubin levels."1.29The use of jejunal transplants to treat a genetic enzyme deficiency. ( Burgos, AA; Jaffe, BM; Martinez-Noack, M, 1996)
"The recognition that Gilbert's syndrome is a quite heterogeneous entity will allow a better understanding of the mode of inheritance of this disorder; its relationship to Crigler-Najjar type II disease also awaits further definition."1.27Familial unconjugated hyperbilirubinemia syndromes. ( Reichen, J, 1983)

Research

Studies (37)

TimeframeStudies, this research(%)All Research%
pre-199011 (29.73)18.7374
1990's9 (24.32)18.2507
2000's7 (18.92)29.6817
2010's9 (24.32)24.3611
2020's1 (2.70)2.80

Authors

AuthorsStudies
Cozzi, L1
Nuti, F1
Degrassi, I1
Civeriati, D1
Paolella, G1
Nebbia, G1
Aronson, SJ1
Veron, P1
Collaud, F1
Hubert, A1
Delahais, V1
Honnet, G1
de Knegt, RJ1
Junge, N1
Baumann, U1
Di Giorgio, A1
D'Antiga, L1
Ginocchio, VM1
Brunetti-Pierri, N1
Labrune, P1
Beuers, U1
Bosma, PJ2
Mingozzi, F1
Liaqat, A1
Shahid, A1
Attiq, H1
Ameer, A1
Imran, M1
Gailite, L1
Rots, D1
Pukite, I1
Cernevska, G1
Kreile, M1
Shi, X1
Aronson, S1
Khan, AS1
Kummer, O1
Hammann, F1
Haschke, M1
Krähenbühl, S1
Rubio Gómez, I1
López González, MF1
Ibáñez Alcalde, MM1
Cañabate Reche, F1
Passuello, V1
Puhl, AG1
Wirth, S1
Steiner, E1
Skala, C1
Koelbl, H1
Kohlschmidt, N1
Aggarwal, V1
Seth, A1
Sharma, S1
Aneja, S1
Sammarco, P1
Fabiano, C1
Holstein, A2
Bryan, CS1
Liu, WL1
Li, F1
He, ZX1
Jiang, HY1
Ai, R1
Chen, XX1
Huang, K1
Plaschke, A1
Lohse, P1
Egberts, EH1
Torres, M1
Bruguera, M1
Maruo, Y1
Topaloglu, AK1
Takahashi, H2
Mori, A1
Iwai, M1
Duzovali, O1
Yamamoto, K1
Matui, K1
Sato, H1
Takeuchi, Y1
Hafkamp, AM1
Nelisse-Haak, R1
Sinaasappel, M2
Oude Elferink, RP1
Verkade, HJ1
Reichen, J1
Trotman, BW1
Shaw, L1
Roy-Chowdhury, J1
Malet, PF1
Rosato, EF1
Younossi, ZM1
Foroozan, P1
Rubaltelli, FF1
Novello, A1
Zancan, L1
Vilei, MT1
Muraca, M2
Jaffe, BM1
Burgos, AA1
Martinez-Noack, M1
Ciotti, M1
Werlin, SL1
Owens, IS1
Jansen, PL2
Ito, T1
Katagiri, C1
Ikeno, S1
Nagata, N1
Terakawa, N1
Lee, WS1
McKiernan, PJ1
Beath, SV1
Preece, MA1
Baty, D1
Kelly, DA1
Burchell, B1
Clarke, DJ1
Plöchl, E1
Hohenwallner, W1
Wehinger, H1
Gollan, JL2
Huang, SN2
Billing, B1
Sherlock, S2
Stiehl, A1
Berk, PD1
Martin, JF1
Blaschke, TF1
Scharschmidt, BF1
Plotz, PH1
Volodin, NN1
Persico, M1
Romano, M1
Gentile, S1
Mori, Y1
Kimura, H1
Suehiro, K1
Naritomi, Y1
Maeda, Y1
Kusaba, T1
Ishibashi, D1
Pett, S1
Mowat, AP1
Duhamel, G1
Blanckaert, N1
Metreau, JM1
Préaux, AM1
Bouvry, M1
Fevery, J1
Berthelot, P1
Scheig, R1
Billing, BH1

Reviews

5 reviews available for phenobarbital and Crigler Najjar Syndrome

ArticleYear
Pregnancy outcome in maternal Crigler-Najjar syndrome type II: a case report and systematic review of the literature.
    Fetal diagnosis and therapy, 2009, Volume: 26, Issue:3

    Topics: Adult; Bilirubin; Crigler-Najjar Syndrome; Female; Hearing Disorders; Humans; Infant; Infant, Newbor

2009
[Crigler-Najjar syndrome].
    Gastroenterologia y hepatologia, 2005, Volume: 28, Issue:10

    Topics: Bilirubin; Calcium Phosphates; Chemotherapy, Adjuvant; Crigler-Najjar Syndrome; Diagnosis, Different

2005
Diagnosis and management of Crigler-Najjar syndrome.
    European journal of pediatrics, 1999, Volume: 158 Suppl 2

    Topics: Animals; Bilirubin; Child; Crigler-Najjar Syndrome; Female; Humans; Kernicterus; Liver Transplantati

1999
Phenobarbital following phototherapy for Crigler-Najjar syndrome type II with good fetal outcome: a case report.
    The journal of obstetrics and gynaecology research, 2001, Volume: 27, Issue:1

    Topics: Adult; Anticonvulsants; Crigler-Najjar Syndrome; Female; Humans; Infant, Newborn; Phenobarbital; Pho

2001
[Congenital nonhemolytic hyperbilirubinemias].
    Medizinische Klinik, 1977, Sep-16, Volume: 72, Issue:37

    Topics: Adolescent; Adult; Age Factors; Bilirubin; Child; Child, Preschool; Cholestasis; Chronic Disease; Cr

1977

Trials

1 trial available for phenobarbital and Crigler Najjar Syndrome

ArticleYear
Orlistat treatment of unconjugated hyperbilirubinemia in Crigler-Najjar disease: a randomized controlled trial.
    Pediatric research, 2007, Volume: 62, Issue:6

    Topics: Adolescent; Adult; Anti-Obesity Agents; Bilirubin; Child; Combined Modality Therapy; Crigler-Najjar

2007

Other Studies

31 other studies available for phenobarbital and Crigler Najjar Syndrome

ArticleYear
Gilbert or Crigler-Najjar syndrome? Neonatal severe unconjugated hyperbilirubinemia with P364L UGT1A1 homozygosity.
    Italian journal of pediatrics, 2022, Apr-18, Volume: 48, Issue:1

    Topics: Bilirubin; Crigler-Najjar Syndrome; Female; Gilbert Disease; Glucuronosyltransferase; Humans; Hyperb

2022
Prevalence and Relevance of Pre-Existing Anti-Adeno-Associated Virus Immunity in the Context of Gene Therapy for Crigler-Najjar Syndrome.
    Human gene therapy, 2019, Volume: 30, Issue:10

    Topics: Adolescent; Adult; Animals; Antibodies, Neutralizing; Antibodies, Viral; Bilirubin; Capsid; Child; C

2019
Crigler-Najjar Syndrome Type II Diagnosed in a Patient with Jaundice Since Birth.
    Journal of the College of Physicians and Surgeons--Pakistan : JCPSP, 2018, Volume: 28, Issue:10

    Topics: Adolescent; Crigler-Najjar Syndrome; Female; Gilbert Disease; Glucuronosyltransferase; Humans; Hyper

2018
Case report: multiple UGT1A1 gene variants in a patient with Crigler-Najjar syndrome.
    BMC pediatrics, 2018, 10-03, Volume: 18, Issue:1

    Topics: Adolescent; Crigler-Najjar Syndrome; Glucuronosyltransferase; Humans; Male; Mutation; Phenobarbital;

2018
A novel UGT1A1 gene mutation causing severe unconjugated hyperbilirubinemia: a case report.
    BMC pediatrics, 2019, 05-29, Volume: 19, Issue:1

    Topics: Adolescent; Bilirubin; Codon, Terminator; Crigler-Najjar Syndrome; DNA Mutational Analysis; Exons; F

2019
Reduction of hyperbilirubinemia with hypericum extract (St. John's Wort) in a patient with Crigler-Najjar syndrome type II.
    British journal of clinical pharmacology, 2016, Volume: 81, Issue:5

    Topics: Adult; Area Under Curve; Bilirubin; Crigler-Najjar Syndrome; Cytochrome P-450 CYP3A; Enzyme Inductio

2016
[Phenobarbital in Crigler-Najjar syndrome type 1: A therapeutic option].
    Anales de pediatria (Barcelona, Spain : 2003), 2016, Volume: 85, Issue:2

    Topics: Crigler-Najjar Syndrome; Humans; Infant, Newborn; Male; Phenobarbital

2016
Persistent jaundice in an infant with homozygous beta thalassemia due to co-inherited Crigler-Najjar syndrome.
    Pediatric blood & cancer, 2010, Volume: 54, Issue:4

    Topics: beta-Thalassemia; Crigler-Najjar Syndrome; Erythrocyte Transfusion; Fatal Outcome; GABA Modulators;

2010
Three consecutive pregnancies in a woman with Crigler-Najjar syndrome type II with good maternal and neonatal outcomes.
    Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver, 2011, Volume: 43, Issue:2

    Topics: Adult; Anticonvulsants; Cesarean Section; Crigler-Najjar Syndrome; Female; Humans; Infant, Newborn;

2011
Analysis of bilirubin UDP-glucuronosyltransferase gene mutations in an unusual Crigler-Najjar syndrome patient.
    Molecular medicine reports, 2012, Volume: 6, Issue:3

    Topics: Anticonvulsants; Crigler-Najjar Syndrome; DNA Mutational Analysis; Exons; Glucuronosyltransferase; H

2012
Successful photo-and phenobarbital therapy during pregnancy in a woman with Crigler-Najjar syndrome type II.
    Scandinavian journal of gastroenterology, 2005, Volume: 40, Issue:9

    Topics: Adult; Anticonvulsants; Crigler-Najjar Syndrome; Female; Follow-Up Studies; Humans; Infant, Newborn;

2005
Crigler-Najjar syndrome type II caused by a homozygous triple mutation [T-3279G, A(TA)7TAA, and H39D] of UGT1A1.
    Journal of pediatric gastroenterology and nutrition, 2006, Volume: 42, Issue:2

    Topics: Blotting, Western; Child; Crigler-Najjar Syndrome; Gene Expression Regulation, Enzymologic; Genetic

2006
Familial unconjugated hyperbilirubinemia syndromes.
    Seminars in liver disease, 1983, Volume: 3, Issue:1

    Topics: Animals; Bile Acids and Salts; Bilirubin; Crigler-Najjar Syndrome; Diagnosis, Differential; Disease

1983
Effect of phenobarbital on serum and biliary parameters in a patient with Crigler-Najjar syndrome, type II and acquired cholestasis.
    Digestive diseases and sciences, 1983, Volume: 28, Issue:8

    Topics: Adult; Bile; Cholestasis; Crigler-Najjar Syndrome; Humans; Hyperbilirubinemia, Hereditary; Liver Fun

1983
Treatment of Crigler-Najjar type II with small-dose phenobarbital.
    Digestive diseases and sciences, 1995, Volume: 40, Issue:3

    Topics: Adult; Atropine; Crigler-Najjar Syndrome; Drug Combinations; Female; Humans; Phenobarbital; Scopolam

1995
Serum and bile bilirubin pigments in the differential diagnosis of Crigler-Najjar disease.
    Pediatrics, 1994, Volume: 94, Issue:4 Pt 1

    Topics: Administration, Oral; Bile; Bile Pigments; Bilirubin; Chromatography, High Pressure Liquid; Crigler-

1994
The use of jejunal transplants to treat a genetic enzyme deficiency.
    Annals of surgery, 1996, Volume: 223, Issue:6

    Topics: Animals; Bilirubin; Crigler-Najjar Syndrome; Disease Models, Animal; Jejunum; Phenobarbital; Rats; R

1996
Delayed response to phenobarbital treatment of a Crigler-Najjar type II patient with partially inactivating missense mutations in the bilirubin UDP-glucuronosyltransferase gene.
    Journal of pediatric gastroenterology and nutrition, 1999, Volume: 28, Issue:2

    Topics: Bilirubin; Crigler-Najjar Syndrome; Fluid Therapy; Glucuronosyltransferase; Humans; Hyperbilirubinem

1999
Bile bilirubin pigment analysis in disorders of bilirubin metabolism in early infancy.
    Archives of disease in childhood, 2001, Volume: 85, Issue:1

    Topics: Bile Pigments; Bilirubin; Chromatography, High Pressure Liquid; Crigler-Najjar Syndrome; Diagnosis,

2001
[Treatment of Crigler-Najjar's congenital familial non-hemolytic jaundice with kernicterus].
    Wiener medizinische Wochenschrift (1946), 1978, Feb-15, Volume: 128, Issue:3

    Topics: Crigler-Najjar Syndrome; Developmental Disabilities; Epilepsy; Female; Humans; Hyperbilirubinemia; H

1978
Prolonged survival in three brothers with severe type 2 Crigler-Najjar syndrome. Ultrastructural and metabolic studies.
    Gastroenterology, 1975, Volume: 68, Issue:6

    Topics: Bile Acids and Salts; Bile Pigments; Bilirubin; Crigler-Najjar Syndrome; Electroencephalography; Glu

1975
[Treatment of various forms of icterus or cholestasis with phenobarbital (author's transl)].
    Zeitschrift fur Gastroenterologie, 1977, Volume: 15, Issue:6

    Topics: Adult; Anemia, Hemolytic; Cholangitis; Cholestasis; Crigler-Najjar Syndrome; Gilbert Disease; Humans

1977
Unconjugated hyperbilirubinemia. Physiologic evaluation and experimental approaches to therapy.
    Annals of internal medicine, 1975, Volume: 82, Issue:4

    Topics: Adult; Albumins; Animals; Bilirubin; Crigler-Najjar Syndrome; Ethosuximide; Female; Gilbert Disease;

1975
[Crigler-Najjar syndrome in an infant during the 1st year of life].
    Pediatriia, 1991, Issue:9

    Topics: Crigler-Najjar Syndrome; Follow-Up Studies; Humans; Infant; Male; Phenobarbital; Phototherapy; Time

1991
Responsiveness to phenobarbital in an adult with Crigler-Najjar disease associated with neurological involvement and skin hyperextensibility.
    Hepatology (Baltimore, Md.), 1991, Volume: 13, Issue:2

    Topics: Adult; Bilirubin; Chromatography, High Pressure Liquid; Crigler-Najjar Syndrome; Epilepsy, Tonic-Clo

1991
[A case of Crigler-Najjar syndrome of type II].
    Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine, 1991, Jan-10, Volume: 80, Issue:1

    Topics: Adolescent; Bilirubin; Crigler-Najjar Syndrome; Glucuronosyltransferase; Humans; Liver; Male; Phenob

1991
The differential diagnosis of Crigler-Najjar disease, types 1 and 2, by bile pigment analysis.
    Gastroenterology, 1991, Volume: 100, Issue:3

    Topics: Bile; Bile Acids and Salts; Bile Pigments; Bilirubin; Child; Crigler-Najjar Syndrome; Diagnosis, Dif

1991
Crigler-Najjar syndrome types I and II. Clinical experience--King's College Hospital 1972-1978. Phenobarbitone, phototherapy and liver transplantation.
    Molecular aspects of medicine, 1987, Volume: 9, Issue:5

    Topics: Child; Child, Preschool; Crigler-Najjar Syndrome; Female; Humans; Hyperbilirubinemia, Hereditary; In

1987
An unusual case of Crigler-Najjar disease in the adult. Classification into types I and II revisited.
    Journal of hepatology, 1985, Volume: 1, Issue:1

    Topics: Adult; Bile; Bile Acids and Salts; Bilirubin; Crigler-Najjar Syndrome; Glucuronosyltransferase; Huma

1985
Neonatal jaundice.
    American family physician, 1974, Volume: 10, Issue:4

    Topics: Albumins; Bile Ducts; Bilirubin; Breast Feeding; Crigler-Najjar Syndrome; Erythroblastosis, Fetal; E

1974
Proceedings: Prolonged survival in the Crigler-Najjar syndrome: ultrastructural and dietary studies.
    Gut, 1974, Volume: 15, Issue:10

    Topics: Bilirubin; Cell Membrane; Crigler-Najjar Syndrome; Endoplasmic Reticulum; Glucose; Golgi Apparatus;

1974