Use of daytime phototherapy for Crigler–Najjar disease

Nydegger, A; Bednarz, Andrew; Hardikar, Winita

doi:10.1111/j.1440-1754.2005.00642.x

Cited by 7 publications

(5 citation statements)

References 10 publications

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“…In vitro analysis of the resultant truncated protein showed no enzyme activity 7. By the age of 8 years, her average serum bilirubin was 337 μmol/L, and her treatment regime consisted of 8 hours per day in a purpose‐built phototherapy unit 8…”

Section: Patient and Methodsmentioning

confidence: 99%

Cell-mediated rejection results in allograft loss after liver cell transplantation

et al. 2008

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Liver cell transplantation in humans has been impeded by invariable loss of the graft. It is unclear whether graft loss is due to an immune response against donor hepatocytes. Transplantation with ABO-matched liver cells was performed in a patient with Crigler-Najjar type 1. After successful engraftment, there was a gradual loss of graft function. Solid-phase enzyme immunoassay testing and cell-complement cytotoxicity assays detecting preformed antibodies directed toward class I and/or class II human leukocyte antigen (HLA) molecules were negative. In contrast, a striking host alloresponse to either the HLA-B39 or C7 antigen was found, suggesting that a vigorous response to a defined mismatched HLA antigen contributed to graft loss in our patient. This study provides evidence that a T-cell-mediated immune mechanism could be responsible for human liver cell transplant graft loss. This finding warrants confirmation in future liver cell transplants in humans. Liver Transpl 14:688-694, 2008.

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Section: Patient and Methodsmentioning

confidence: 99%

Cell-mediated rejection results in allograft loss after liver cell transplantation

et al. 2008

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“…This technique involves exposure to light and heat 10-12 h per day [26] . -Phenobarbital: inducts the residual UGT1A1 activity present in CNS type II patients.…”

Section: Discussionmentioning

confidence: 99%

Pregnancy Outcome in Maternal Crigler-Najjar Syndrome Type II: A Case Report and Systematic Review of the Literature

Passuello

Puhl²,

Wirth³

et al. 2009

Fetal Diagn Ther

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Objective: To report a case of maternal Crigler-Najjar syndrome (CNS) type II in pregnancy, systematically review the literature for similar case reports, and to evaluate whether pregnancy is safe in patients with the disease. Data sources included the PubMed and uptodate databases. Results: A 37-year-old mother with CNS type II was treated with phenobarbital during her pregnancy and her bilirubin levels were monitored. Her newborn had mild direct hyperbilirubinemia, did not require any treatment and his postnatal follow-up showed normal growth and development as well as normal hearing. Conclusion: CNS type II is rare, and only a few pregnancies with this condition have been reported. Maternal treatment with phenobarbital lowers the unconjugated bilirubin and avoids fetal and newborn sequelae.

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“…Bilirubin levels over 171 μmol/L are an indication for phototherapy in term infants without risk factors, while the threshold is 4 mg/dL (68.4 μmol/L) in infants with high risk for kernicterus (preterm, low birth weight) ( 33 ). This technique involves the patient’s exposure to light for 10–12 h, even 14 h, per day ( 19 , 34 , 35 ). Oral calcium supplementation makes phototherapy more efficient ( 36 ).…”

Section: Discussionmentioning

confidence: 99%

Type II Crigler-Najjar syndrome: a case report and literature review

He,

Geng,

Zhu

et al. 2024

Front. Med.

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BackgroundCrigler-Najjar syndrome (CNS) is caused by mutations in uridine 5′-diphosphate glucuronyltransferase (UGT1A1) resulting in enzyme deficiency and hyperbilirubinemia. Type II CNS patients could respond to phenobarbital treatment and survive. This study presents a rare case of type II CNS.Case summaryThe proband was a 29-year-old male patient admitted with severe jaundice. A hepatic biopsy showed bullous steatosis of the peri-central veins of the hepatic lobule, sediment of bile pigment, and mild periportal inflammation with normal liver plate structure. The type II CNS was diagnosed by routine genomic sequencing which found that the proband with the Gry71Arg/Tyr486Asp compound heterozygous mutations in the UGT1A1 gene. After treatment with phenobarbital (180 mg/day), his bilirubin levels fluctuated between 100 and 200 μmol/L for 6 months and without severe icterus.ConclusionType II CNS could be diagnosed by routine gene sequencing and treated by phenobarbital.

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Use of daytime phototherapy for Crigler–Najjar disease

Cited by 7 publications

References 10 publications

Cell-mediated rejection results in allograft loss after liver cell transplantation

Cell-mediated rejection results in allograft loss after liver cell transplantation

Pregnancy Outcome in Maternal Crigler-Najjar Syndrome Type II: A Case Report and Systematic Review of the Literature

Type II Crigler-Najjar syndrome: a case report and literature review

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