Log in

Relevant bibliographies by topics / Crigler-Najjar / Journal articles

Journal articles on the topic 'Crigler-Najjar'

To see the other types of publications on this topic, follow the link: Crigler-Najjar.

Author: Grafiati

Published: 11 March 2023

Create a spot-on reference in APA, MLA, Chicago, Harvard, and other styles

Select a source type:

Consult the top 50 journal articles for your research on the topic 'Crigler-Najjar.'

Next to every source in the list of references, there is an 'Add to bibliography' button. Press on it, and we will generate automatically the bibliographic reference to the chosen work in the citation style you need: APA, MLA, Harvard, Chicago, Vancouver, etc.

You can also download the full text of the academic publication as pdf and read online its abstract whenever available in the metadata.

Browse journal articles on a wide variety of disciplines and organise your bibliography correctly.

1

Haque, Md Azizul, Laila Shamima Sharmin, Mohd Harun or Rashid, MA Alim, ARM Saifuddin Ekram, and Syed Ghulam Mogni Mowla. "Crigler-Najjar Syndrome Type 2 in a Young Adult." Journal of Medicine 12, no. 1 (January 21, 2011): 86–88. http://dx.doi.org/10.3329/jom.v12i1.6359.

Full text

Abstract:

Crigler-Najjar syndrome type 2 in an autosomal recessive congenital non-hemolytic hyperbilirubinemia caused by UDP-glucuronosyltransferase deficiency. Only a few hundred cases have been described in the literature so far. We are reporting Crigler-Najjar syndrome type 2 in an 18 year old female born out of consanguineous marriage. Keyword: Crigler-Najjar syndrome; UDP-glucuronosyltransferase; Bangladesh DOI: 10.3329/jom.v12i1.6359J Medicine 2011; 12 : 81-85

APA, Harvard, Vancouver, ISO, and other styles

2

Fatima, Bushra, Ayesha Ahmad, Tamkin Khan, and Rizwan Ahmad. "Crigler Najjar Syndrome [Type II] with Pregnancy: Case Report." International Journal of Human and Health Sciences (IJHHS) 4, no. 1 (October 31, 2019): 60. http://dx.doi.org/10.31344/ijhhs.v4i1.121.

Full text

Abstract:

Crigler-Najjar syndromes are rare, autosomal recessive disorders caused by mutations in the genes of bilirubin metabolism. The management of these pregnancies is controversial due to paucity of literature. We discuss here a successfully managed case of pregnancy with Crigler-Najjar Syndrome.International Journal of Human and Health Sciences Vol. 04 No. 01 January’20 Page : 60-62

APA, Harvard, Vancouver, ISO, and other styles

3

Radlovic, Nedeljko. "Hereditary hyperbilirubinemias." Srpski arhiv za celokupno lekarstvo 142, no. 3-4 (2014): 257–60. http://dx.doi.org/10.2298/sarh1404257r.

Full text

Abstract:

Inherited disorders of bilirubin metabolism involve four autosomal recessive syndromes: Gilbert, Crigler- Najjar, Dubin-Johnson and Rotor, among which the first two are characterized by unconjugated and the second two by conjugated hyperbilirubinemia. Gilbert syndrome occurs in 2%-10% of general population, while others are rare. Except for Crigler-Najjar syndrome, hereditary hyperbilirubinemias belong to benign disorders and thus no treatment is required.

APA, Harvard, Vancouver, ISO, and other styles

4

Torres, M., and M. Bruguera. "Síndrome de Crigler-Najjar." Gastroenterología y Hepatología 28, no. 10 (December 2005): 637–40. http://dx.doi.org/10.1016/s0210-5705(05)71530-2.

Full text

APA, Harvard, Vancouver, ISO, and other styles

5

RUBALTELLI, FIRMINO F., PIETRO GUERRINI, ELENA REDDI, and GIULIO JORI. "Tin-Protoporphyrin in the Management of Children With Crigler-Najjar Disease." Pediatrics 84, no. 4 (October 1, 1989): 728–31. http://dx.doi.org/10.1542/peds.84.4.728.

Full text

Abstract:

The Crigler-Najjar disease is a rare disorder originally described in 1952 and characterized by a severe unconjugated hyperbilirubinemia appearing in the first days of life and persisting throughout life. In 1969, Arias et al proposed to subdivide such patients into two groups. The first group (Crigler-Najjar disease type 1) consisted of the most severely jaundiced infants in whom bilirubin encephalopathy developed resulting in death, usually within the first year of life, and whose plasma bilirubin level did not decrease during treatment with phenobarbital. Their bile was described as virtually colorless and appeared to contain only a trace amount of bilirubin, all in the unconjugated form. In the second group (Crigler-Najjar disease type 2), bilirubin conjugates were detected in normally yellow bile. In these patients, the unconjugated hyperbilirubinemia was less severe and encephalopathy was absent.

APA, Harvard, Vancouver, ISO, and other styles

6

Šelih, Anja, and Manca Velkavrh. "CRIGLER- NAJJAR SYNDROME – CASE REPORT." Slovenska pediatrija, revija pediatrov Slovenije in specialistov šolske ter visokošolske medicine Slovenije 29, no. 2 (2022): 78–82. http://dx.doi.org/10.38031/slovpediatr-2022-2-04en.

Full text

APA, Harvard, Vancouver, ISO, and other styles

7

Huang, Ching-Shan, Nancy Tan, Sien-Sing Yang, Yung-Chan Sung, and May-Jen Huang. "Crigler-Najjar Syndrome Type 2." Journal of the Formosan Medical Association 105, no. 11 (2006): 950–53. http://dx.doi.org/10.1016/s0929-6646(09)60182-0.

Full text

APA, Harvard, Vancouver, ISO, and other styles

8

Güldütuna, Sükrettin, Ulrich Langenbeck, Karl Walter Bock, Andreas Sieg, and Ulrich Leuschner. "Crigler-Najjar syndrome type II." Digestive Diseases and Sciences 40, no. 1 (January 1995): 28–32. http://dx.doi.org/10.1007/bf02063937.

Full text

APA, Harvard, Vancouver, ISO, and other styles

9

MCDONAGH, ANTONY F. "Tin-protoporphyrin in the Management of Children With Crigler-Najjar Disease." Pediatrics 86, no. 1 (July 1, 1990): 151–52. http://dx.doi.org/10.1542/peds.86.1.151a.

Full text

Abstract:

To the Editor.— Rubaltelli et al1 recently described the use of tin-protoporphyrin (SnPp) to block bilirubin formation in a child with the potentially fatal Crigler-Najjar Type II disease, a genetic disorder characterized by an inability to conjugate and excrete bilirubin in the normal way. Although repeated administration of SnPp failed to suppress the plasma bilirubin concentration significantly in their patient, perhaps because of the buffering effect of a relatively large extravascular pool of bilirubin, it did appear to diminish the need for phototherapy, suggesting that SnPp might be a useful adjuvant to phototherapy in the treatment of Crigler-Najjar disease.

APA, Harvard, Vancouver, ISO, and other styles

10

Cahill, D. J., and C. F. McCarthy. "Pregnancy and the Crigler-Najjar syndrome." Journal of Obstetrics and Gynaecology 9, no. 3 (January 1989): 213. http://dx.doi.org/10.3109/01443618909151039.

Full text

APA, Harvard, Vancouver, ISO, and other styles

11

Weiß, Johannes. "Orphan Disease – Das Crigler-Najjar Syndrom." Zeitschrift für Gastroenterologie 52, no. 04 (April 15, 2014): 338. http://dx.doi.org/10.1055/s-0033-1362434.

Full text

APA, Harvard, Vancouver, ISO, and other styles

12

Alaman, Alizah, Munira Minaz Ali, Nazish Hussain Ali Pachani, and Anmol Minaz Ali. "Crigler-Najjar Syndrome: A Rare Fatality." International Journal of Women Empowerment 2, no. 1 (December 1, 2016): 22. http://dx.doi.org/10.29052/2413-4252.v2.i1.2016.22-30.

Full text

APA, Harvard, Vancouver, ISO, and other styles

13

Bhalchandra Deore, Amol. "Crigler najjar syndrome: A systematic outline." Indian Journal of Pharmacy and Pharmacology 6, no. 3 (October 15, 2019): 75–78. http://dx.doi.org/10.18231/j.ijpp.2019.017.

Full text

APA, Harvard, Vancouver, ISO, and other styles

14

Nazer, Hisham, Ali Al-Mehaidib, Souheil Shabib, and M. Ashraf Ali. "Crigler-Najjar syndrome in Saudi Arabia." American Journal of Medical Genetics 79, no. 1 (August 27, 1998): 12–15. http://dx.doi.org/10.1002/(sici)1096-8628(19980827)79:1<12::aid-ajmg4>3.0.co;2-k.

Full text

APA, Harvard, Vancouver, ISO, and other styles

15

Ilchenko, L. Yu, I. G. Fedorov, G. G. Totolyan, A. G. Tsvetkova, E. G. Gavrilenko, K. O. Mironov, and I. G. Nikitin. "HEREDITARY UNCONJUGATED HYPERBILIRUBINEMIA (COMBINATION OF CRIGLER-NAJJAR SYNDROME TYPE II AND GILBERT'S SYNDROME)." Hepatology and Gastroenterology 5, no. 1 (June 10, 2021): 79–84. http://dx.doi.org/10.25298/2616-5546-2021-5-1-79-84.

Full text

Abstract:

Background. Enzymopathic jaundices are manifested by intermittent hyperbilirubinemia, no changes in the structure of the liver, no hemolysis, Rh-conflict as well as cholestasis being noted. These jaundices include Crigler-Najjar syndrome type I, Crigler-Najjar syndrome type II and Gilbert's syndrome. They are characterized by an autosomal recessive inheritance due to the presence of mutations and polymorphisms in uridine 5'-diphosphate-glucuronosyltransferase gene (UGT1A1) leading to a decrease of the enzyme activity or to its complete loss. Objective. To demonstrate the peculiarities of diagnosis and treatment of a rare case of hereditary unconjugated hyperbilirubinemia - a combination of Crigler-Najjar syndrome type II and Gilbert's syndrome. Material and methods. Clinical observation of a patient G. aged 19, who was examined and treated at the Department of gastroenterology of a multidisciplinary hospital in Moscow in January 2021. Results. The patient G. has had icteric sclerae and skin since birth; he occasionally suffers from easy fatigability and general malaise. Physical examination revealed no changes (except for icteric discoloration). An increase in unconjugated bilirubin up to 270 μmol/L (median - 170 μmol/L) was detected. The molecular genetic study of UGT1A1 gene identified mutations in exon 4 Val378Asp (2002) and Arg108Cys as well as polymorphism 6/7TA in the promoter region, confirming the diagnosis of autosomal recessive inherited disease – a combination of Crigler Najjar syndrome type II and Gilbert's syndrome (heterozygous state), complicated by the development of hepatic encephalopathy stage 2. There was noted a significant decrease in unconjugated bilirubin up to 170.5 μmol/L, as well as improvement in general condition – reduced fatigue and weakness during the treatment with microsomal enzyme inducer (phenobarbital) and hyperammonemia corrector (ornithine aspartate). Conclusions. The use of molecular genetic analysis allows tailoring strategies for patient-specific disease diagnostics, treatment and prevention. The preservation of quality of life within satisfactory level is achieved through elimination of adverse effects provoking the development of this syndrome and through control of risk factors.

APA, Harvard, Vancouver, ISO, and other styles

16

Sagili, H., N. Pramya, D. Jayalaksmi, and R. Rani. "Crigler–Najjar syndrome II and pregnancy outcome." Journal of Obstetrics and Gynaecology 32, no. 2 (February 2012): 188–89. http://dx.doi.org/10.3109/01443615.2011.636158.

Full text

APA, Harvard, Vancouver, ISO, and other styles

17

Silva, E. Santos, R. Reding, J. de Ville, J. P. Buts, J. B. Otta, and E. M. Sokal. "105 LIVER TRANSPLANTATION FOR CRIGLER NAJJAR SYNDROME." Journal of Pediatric Gastroenterology and Nutrition 20, no. 4 (May 1995): 471. http://dx.doi.org/10.1097/00005176-199505000-00115.

Full text

APA, Harvard, Vancouver, ISO, and other styles

18

Arora, N., and S. Choudhary. "Pregnancy with Crigler–Najjar syndrome type II." Journal of Obstetrics and Gynaecology 29, no. 3 (January 2009): 242–44. http://dx.doi.org/10.1080/01443610802706043.

Full text

APA, Harvard, Vancouver, ISO, and other styles

19

TAYLOR, W. G., S. A. WALKINSHAW, R. G. FARQUHARSON, R. A. FISKEN, and I. T. GILMORE. "Pregnancy in Crigler-Najjar syndrome. Case report." BJOG: An International Journal of Obstetrics and Gynaecology 98, no. 12 (December 1991): 1290–91. http://dx.doi.org/10.1111/j.1471-0528.1991.tb15405.x.

Full text

APA, Harvard, Vancouver, ISO, and other styles

20

Jansen, P. L. M. "Diagnosis and management of Crigler-Najjar syndrome." European Journal of Pediatrics 158, S2 (January 1, 1999): S089—S094. http://dx.doi.org/10.1007/pl00014330.

Full text

APA, Harvard, Vancouver, ISO, and other styles

21

Robards, Christopher, and Sorin J. Brull. "The Anesthetic Implications of Crigler-Najjar Syndrome." Anesthesia & Analgesia 104, no. 2 (February 2007): 435–36. http://dx.doi.org/10.1213/01.ane.0000252462.70451.0e.

Full text

APA, Harvard, Vancouver, ISO, and other styles

22

Lodoso Torrecilla, B., E. Palomo Atance, C. Camarena Grande, M. ªC Díaz Fernández, L. Hierro Llanillo, A. de la Vega Bueno, E. Frauca Remacha, G. Muñoz Bartolo, and P. Jara Vega. "Síndrome de Crigler-Najjar: diagnóstico y tratamiento." Anales de Pediatría 65, no. 1 (July 2006): 73–78. http://dx.doi.org/10.1157/13090900.

Full text

APA, Harvard, Vancouver, ISO, and other styles

23

O'Reilly, C., and R. Dixon. "Crigler-Najjar Syndrome: Treatment at Home with Phototherapy." Scottish Medical Journal 33, no. 5 (October 1988): 335–36. http://dx.doi.org/10.1177/003693308803300509.

Full text

Abstract:

An infant became jaundiced in the neonatal period. The serum bilirubin failed to fall with phototherapy. A diagnosis of Crigler Najjar type 1 syndrome was made by exclusion and confirmed by liver biopsy. The infant has been successfully treated at home with phototherapy. Liver transplantation remains a therapeutic option.

APA, Harvard, Vancouver, ISO, and other styles

24

Sinha, R., S. Dalal, and K. Sodhi. "Differentiating Gilbert Syndrome from Crigler Najjar Syndrome Type 2 by Phenobarbitone Test." Journal of Nepal Paediatric Society 35, no. 1 (October 8, 2015): 82–84. http://dx.doi.org/10.3126/jnps.v35i1.10620.

Full text

Abstract:

Gilbert syndrome characterized by jaundice with intermittent elevations of indirect bilirubin, in the absence of haemolysis or underlying liver disease, has both autosomal dominant and recessive inheritance. Crigler-Najjar syndrome type II (CNS2) is a hereditary disorder of bilirubin metabolism characterized by unconjugated hyperbilirubinemia due to reduced and inducible activity of hepatic bilirubin glucuronosyltransferase (GT). We report 20 children between age 5 to 15 years with unconjugated hyperbilirubenemia who were given seven days of oral phenobarbitone (5mg/kg/day) and decrease in level of bilirubin was noted. There was only 30-40% reduction of bilirubin in Crigler Najjar Syndrome Type 2 compared to Gilberts Syndrome in which bilirubin level normalised. This case series highlights the importance of simple test to differentiate these two conditions. This test is also very helpful in a place where enzyme level and mutational study cannot be done.J Nepal Paediatr Soc 2015;35(1):82-84

APA, Harvard, Vancouver, ISO, and other styles

25

Naher, Bodhrun, Md Wahiduzzaman Mazumder, Sharmistha Ghosal, Bodhrun Naher, AZM Raihanur Rahman, and ASM Bazlul Karim. "Crigler Najjar Syndrome - A Rare case of Jaundice in Children." Northern International Medical College Journal 12, no. 1 (September 13, 2022): 515–17. http://dx.doi.org/10.3329/nimcj.v12i1.61594.

Full text

Abstract:

Crigler-Najjar syndrome (CNS) was first described in 1952 in Maryland, USA as congenital familial non-hemolytic jaundice with kernicterus by Crigler JF and Najjar VA.1 CNS is a rare genetic disorder characterized by abnormalities in bilirubin metabolism and evident by persistent increase of unconjugated bilirubin. During the first days of life, the syndrome clinically manifests as intense unconjugated hyperbilirubinemia without evidence of hemolysis. It consists of two types, type I and type II. Crigler-Najjar Syndrome is mostly autosomal recessive disorder, but variation may occur in the inheritance of CNS II.2 The key pathogenesis is defect in bilirubin conjugation due to complete or partial deficiency of uridine 5'-diphosphate-glucuronosyl transferase (UGT). This enzyme is required for the conjugation and further excretion of bilirubin from the body. In type I CNS the enzyme activity is completely absent and in type II there is partial absence of the enzyme. Therefore, Type I is more severe form and usually fatal with kernicterus at the age of 1-2 years.3-5 TypeII is less severe and has better prognosis. Patients with CN type II suffer from less jaundice, less neurological impairment, and show a fair response to phenobarbitone therapy (serum bilirubin levels decrease by at least 25%).6 Both males and females are equally affected by CNS. The incidence is approximately 1 in 750,000-1,000,000 in the general population.7 Here we report such a rare case. Northern International Medical College Journal Vol. 12 No.1 July 2020, Page 515-517

APA, Harvard, Vancouver, ISO, and other styles

26

Razek, Ahmed Abdel Khalek Abdel, Mohamed Ezz El Regal, Mortada El-Shabrawi, Mohamed Moustafa Abdeltawwab, Ahmed Megahed, Sherine Elzeny, Noha El Tantawi, and Saher Ebrahiem Taman. "Diffusion Tensor Imaging of Auditory Pathway in Patients With Crigler-Najjar Syndrome Type I: Correlation With Auditory Brainstem Response." Journal of Child Neurology 37, no. 2 (December 27, 2021): 119–26. http://dx.doi.org/10.1177/08830738211025865.

Full text

Abstract:

Aim: To evaluate the role of diffusion tensor imaging of the auditory pathway in patients with Crigler Najjar syndrome type I and its relation to auditory brainstem response. Methods: Prospective study was done including 12 patients with Crigler Najjar syndrome type I and 10 age- and sex-matched controls that underwent diffusion tensor imaging of brain. Mean diffusivity and fractional anisotropy at 4 regions of the brain and brainstem on each side were measured and correlated with the results of auditory brainstem response for patients. Results: There was significantly higher mean diffusivity of cochlear nucleus, superior olivary nucleus, inferior colliculus, and auditory cortex of patients versus controls on both sides for all regions ( P = .001). The fractional anisotropy of cochlear nucleus, superior olivary nucleus, inferior colliculus, and auditory cortex of patients versus controls was significantly lower, with P values of, respectively, .001, .001, .003, and .001 on the right side and .001, .001, .003, and .001 on left side, respectively. Also, a negative correlation was found between the maximum bilirubin level and fractional anisotropy of the left superior olivary nucleus and inferior colliculus of both sides. A positive correlation was found between the mean diffusivity and auditory brainstem response wave latency of the right inferior colliculus and left cochlear nucleus. The fractional anisotropy and auditory brainstem response wave latency of the right superior olivary nucleus, left cochlear nucleus, and inferior colliculus of both sides were negatively correlated. Conclusion: Diffusion tensor imaging can detect microstructural changes in the auditory pathway in Crigler Najjar syndrome type I that can be correlated with auditory brainstem response.

APA, Harvard, Vancouver, ISO, and other styles

27

Maruo, Yoshihiro, Mahdiyeh Behnam, Shinichi Ikushiro, Sayuri Nakahara, Narges Nouri, and Mansour Salehi. "Two Different UGT1A1 Mutations causing Crigler–Najjar Syndrome types I and II in an Iranian Family." Journal of Gastrointestinal and Liver Diseases 24, no. 4 (December 1, 2015): 523–26. http://dx.doi.org/10.15403/jgld.2014.1121.244.ugt.

Full text

Abstract:

Background: Crigler–Najjar syndrome type I (CN-1) and type II (CN-2) are rare hereditary unconjugated hyperbilirubinemia disorders. However, there have been no reports regarding the co-existence of CN-1 and CN-2 in one family. We experienced a case of an Iranian family that included members with either CN-1 or CN-2. Genetic analysis revealed a mutation in the bilirubin UDP-glucuronosyltransferase (UGT1A1) gene that resulted in residual enzymatic activity.Case report: The female proband developed severe hyperbilirubinemia [total serum bilirubin concentration (TB) = 34.8 mg/dL] with bilirubin encephalopathy (kernicterus) and died after liver transplantation. Her family history included a cousin with kernicterus (TB = 30.0 mg/dL) diagnosed as CN-1. Her great grandfather (TB unknown) and uncle (TB = 23.0 mg/dL) developed jaundice, but without any treatment, they remained healthy as CN-2. Results: The affected cousin was homozygous for a novel frameshift mutation (c.381insGG, p.C127WfsX23). The affected uncle was compound heterozygous for p.C127WfsX23 and p.V225G linked with A(TA)7TAA. p.V225G-UGT1A1 reduced glucuronidation activity to 60% of wild-type. Thus, linkage of A(TA)7TAA and p.V225G might reduce UGT1A1 activity to 18%–36 % of the wild-type. Conclusion: Genetic and in vitro expression analyses are useful for accurate genetic counseling for a family with a history of both CN-1 and CN-2. Abbreviations: CN-1: Crigler–Najjar syndrome type I; CN-2: Crigler–Najjar syndrome type II; GS: Gilbert syndrome; UGT1A1: bilirubin UDP-glucuronosyltransferase; WT: Wild type; TB: total serum bilirubin.

APA, Harvard, Vancouver, ISO, and other styles

28

Nampoothiri, Sheela, KarippothMohandas Nair, and Peter Lohse. "Crigler-Najjar syndrome type 2: Novel UGT1A1 mutation." Indian Journal of Human Genetics 18, no. 2 (2012): 233. http://dx.doi.org/10.4103/0971-6866.100776.

Full text

APA, Harvard, Vancouver, ISO, and other styles

29

HANSEN, THOR WILLY RUUD. "More to Be Learned From Crigler-Najjar Patients." Pediatrics 92, no. 1 (July 1, 1993): 184. http://dx.doi.org/10.1542/peds.92.1.184.

Full text

Abstract:

To the Editor.— In a recent study by Galbraith et al,1 the use of tin-mesoporphyrin in two patients with the Crigler-Najjar type I syndrome is described. The feasibility of this kind of treatment is documented, and the authors' comments on the size of the total-body bilirubin pool as well as their calculations on bilirubin production and clearance in these patients are illuminating. Personally, I am intrigued as much by aspects of the case reports, about which the authors do not comment, probably because these aspects are outside the focus of their paper.

APA, Harvard, Vancouver, ISO, and other styles

30

GALBRAITH, RICHARD A., GEORGE S. DRUMMOND, and ATTALLAH KAPPAS. "More to Be Learned From Crigler-Najjar Patients." Pediatrics 92, no. 1 (July 1, 1993): 184–85. http://dx.doi.org/10.1542/peds.92.1.184a.

Full text

Abstract:

In Reply.— We thank Dr Hansen for his comments on our study in Crigler-Najjar patients published recently in Pediatrics. A collaborative study of this rare genetic disease does seem in order as Dr Hansen indicates; to the extent that such a study might involve an examination of the efficacy of the heme oxygenase inhibitor tin-mesaporphyrin for ameliorating (with other therapeutic modalities) hyperbilirubinemia in this syndrome, we would be happy to collaborate with other investigators and to provide some resources for the study as required.

APA, Harvard, Vancouver, ISO, and other styles

31

HANSEN, THOR WILLY RUUD. "More to be Learned from Crigler-Najjar Patients." Pediatrics 89, no. 6 (June 1, 1992): 1268–69. http://dx.doi.org/10.1542/peds.89.6.1268b.

Full text

Abstract:

To the Editor.— In a recent study by Galbraith et al,1the use of tin-mesopor-phyrin in two patients with the Crigler-Najjar type I syndrome is described. The feasibility of this kind of treatment is documented, and their comments on the size of the total-body bilirubin pool as well as their calculations on bilirubin production and clearance in these patients are very interesting and illuminating. However, I am personally as much intrigued by aspects of the case reports, about which the authors do not comment, probably because it is outside the focus of their paper.

APA, Harvard, Vancouver, ISO, and other styles

32

Millichap, J. Gordon. "Cerebellar Symptoms in Crigler-Najjar Type I Disease." Pediatric Neurology Briefs 6, no. 7 (July 1, 1992): 56. http://dx.doi.org/10.15844/pedneurbriefs-6-7-12.

Full text

APA, Harvard, Vancouver, ISO, and other styles

33

Mitchell, Ellen, Sarangarajan Ranganathan, Patrick McKiernan, Robert H. Squires, Kevin Strauss, Kyle Soltys, George Mazariegos, and James E. Squires. "Hepatic Parenchymal Injury in Crigler-Najjar Type I." Journal of Pediatric Gastroenterology and Nutrition 66, no. 4 (April 2018): 588–94. http://dx.doi.org/10.1097/mpg.0000000000001843.

Full text

APA, Harvard, Vancouver, ISO, and other styles

34

Prager, Marie Csete, Kristen L. Johnson, Nancy L. Ascher, and John P. Roberts. "Anesthetic Care of Patients With Crigler-Najjar Syndrome." Anesthesia & Analgesia 74, no. 1 (January 1992): 162???164. http://dx.doi.org/10.1213/00000539-199201000-00031.

Full text

APA, Harvard, Vancouver, ISO, and other styles

35

Hassona, Yazan, Ala'a Al Haddad, and Ala'a Atef. "Oral findings in Crigler‐Najjar syndrome type I." Special Care in Dentistry 40, no. 6 (September 3, 2020): 611–12. http://dx.doi.org/10.1111/scd.12518.

Full text

APA, Harvard, Vancouver, ISO, and other styles

36

Shevell, Michael I., Annette Majnemer, and David Schiff. "Neurologic Perspectives of Crigler-Najjar Syndrome Type I." Journal of Child Neurology 13, no. 6 (June 1998): 265–69. http://dx.doi.org/10.1177/088307389801300605.

Full text

APA, Harvard, Vancouver, ISO, and other styles

37

YOUNG, TON-HO, YOU-CHEN CHAO, HUNG-SHUNG TANG, and WEN-SHENG HUANG. "Hepatobiliary Imaging in Crigler-Najjar Syndrome Type 2." Clinical Nuclear Medicine 23, no. 11 (November 1998): 780–81. http://dx.doi.org/10.1097/00003072-199811000-00017.

Full text

APA, Harvard, Vancouver, ISO, and other styles

38

MORI, TADASHI. "A case of II type Crigler-Najjar syndrome." Nihon Naika Gakkai Zasshi 80, no. 1 (1991): 102–3. http://dx.doi.org/10.2169/naika.80.102.

Full text

APA, Harvard, Vancouver, ISO, and other styles

39

Nydegger, Andy, Andrew Bednarz, and Winita Hardikar. "Use of daytime phototherapy for Crigler-Najjar disease." Journal of Paediatrics and Child Health 41, no. 7 (July 2005): 387–89. http://dx.doi.org/10.1111/j.1440-1754.2005.00642.x.

Full text

APA, Harvard, Vancouver, ISO, and other styles

40

Kagita, Atsushi, Yukihiko Adachi, Akira Kambe, Toshinori Kamisako, and Toshio Yamamoto. "Type II Crigler-Najjar syndrome with intrahepatic cholestasis." Journal of Gastroenterology 29, no. 2 (March 1994): 214–17. http://dx.doi.org/10.1007/bf02358686.

Full text

APA, Harvard, Vancouver, ISO, and other styles

41

Ambrosino, Giovanni, Sergio Varotto, Stephen C. Strom, Graziella Guariso, Elisa Franchin, Diego Miotto, Luciana Caenazzo, et al. "Isolated Hepatocyte Transplantation for Crigler-Najjar Syndrome Type 1." Cell Transplantation 14, no. 2-3 (February 2005): 151–57. http://dx.doi.org/10.3727/000000005783983250.

Full text

Abstract:

Crigler-Najjar syndrome type 1 (CN1) is an inherited disorder characterized by the absence of hepatic uridine diphosphoglucuronate glucuronosyltransferase (UDPGT), the enzyme responsible for the conjugation and excretion of bilirubin. We performed allogenic hepatocyte transplantation (AHT) in a child with CN1, aiming to improve bilirubin glucuronidation in this condition. A 9-year-old boy with CN1 was prepared with plasmapheresis and immunosuppression with prednisolone and tacrolimus. When a graft was made available, 7.5 × 109 hepatocytes were isolated and infused into the portal vein percutaneously. After 2 weeks phenobarbitone was added to promote the enzymatic activity of UDPGT of the transplanted hepatocytes. Nocturnal phototherapy was continued throughout the studied period. Total bilirubin was considered a reliable marker of allogenic cell function. There was no significant variation of vital signs nor complications during the infusion. Mean ± SD bilirubin level was 530 ± 38 μmol/L before and 359 ± 46 μmol/L after AHT (t-test, p < 0.001). However, the introduction of phenobarbitone was followed by a drop of tacrolimus level with increase of alanine aminotransferase (ALT) and increase of bilirubin. After standard treatment of cellular rejection bilirubin fell again but from then on it was maintained at a greater level. After discharge the patient experienced a further increase of bilirubin that returned to predischarge levels after readmission to the hospital. This was interpreted as poor compliance with phototherapy. Only partial correction of clinical jaundice and the poor tolerability to nocturnal phototherapy led the parents to refuse further hepatocyte infusions and request an orthotopic liver transplant. After 24 months the child is well, with good liver function on tacrolimus and prednisolone-based immunosuppression. Isolated AHT, though effective and safe, is not sufficient to correct CN1. Maintenance of adequate immunosuppression and family compliance are the main factors hampering the success of this procedure.

APA, Harvard, Vancouver, ISO, and other styles

42

Chaubal, Alisha Nitin, Ruchir Patel, Dhaval Choksi, Kaivan Shah, Meghraj Ingle, and Prabha Sawant. "Management of pregnancy in Crigler Najjar syndrome type 2." World Journal of Hepatology 8, no. 11 (2016): 530. http://dx.doi.org/10.4254/wjh.v8.i11.530.

Full text

APA, Harvard, Vancouver, ISO, and other styles

43

Raposo, Filipa, Margarida Reis Morais, Marina Pinheiro, Mariana Costa, Isabel Martinho, Sérgio Mendanha, and Miguel Salgado. "Síndrome de Crigler-Najjar tipo 2 – um caso atípico." Scientia Medica 24, no. 2 (June 24, 2014): 168. http://dx.doi.org/10.15448/1980-6108.2014.2.16572.

Full text

APA, Harvard, Vancouver, ISO, and other styles

44

Montenegro Miranda, Paula, and Piter Bosma. "Towards Liver-Directed Gene Therapy for Crigler-Najjar Syndrome." Current Gene Therapy 9, no. 2 (April 1, 2009): 72–82. http://dx.doi.org/10.2174/156652309787909508.

Full text

APA, Harvard, Vancouver, ISO, and other styles

45

Wolff, H., G. Otto, and H. Giest. "LIVER TRANSPLANTATION IN CRIGLER-NAJJAR SYNDROME A Case Report." Transplantation 42, no. 1 (July 1986): 84. http://dx.doi.org/10.1097/00007890-198607000-00018.

Full text

APA, Harvard, Vancouver, ISO, and other styles

46

Gridelli, B., A. Lucianetti, S. Gatti, M. Colledan, R. Benti, A. Bruno, L. N. Rossi, and L. R. Fassati. "Orthotopic liver transplantation for Crigler-Najjar type I syndrome." Transplantation Proceedings 29, no. 1-2 (February 1997): 440–41. http://dx.doi.org/10.1016/s0041-1345(96)00179-0.

Full text

APA, Harvard, Vancouver, ISO, and other styles

47

Tu, Zhen-Hua, De-Sheng Shang, Jin-Cai Jiang, Wu Zhang, Min Zhang, Wei-Lin Wang, Hai-Yan Lou, and Shu-Sen Zheng. "Liver transplantation in Crigler-Najjar syndrome type I disease." Hepatobiliary & Pancreatic Diseases International 11, no. 5 (October 2012): 545–48. http://dx.doi.org/10.1016/s1499-3872(12)60222-7.

Full text

APA, Harvard, Vancouver, ISO, and other styles

48

Perretti, A., G. Crispino, L. Marcantonio, S. Lenta, M. Caropreso, F. Manganelli, S. Scianguetta, R. Iorio, A. Iolascon, and P. Vajro. "Clinical Utility of Electrophysiological Evaluation in Crigler-Najjar Syndrome." Neuropediatrics 38, no. 4 (August 2007): 173–78. http://dx.doi.org/10.1055/s-2007-991147.

Full text

APA, Harvard, Vancouver, ISO, and other styles

49

Tabarki, Brahim, Monia Khalifa, Moncef Yacoub, Kalthoum Tlili, and Ahmed S. Essoussi. "Cerebellar symptoms heralding bilirubin encephalopathy in crigler-najjar syndrome." Pediatric Neurology 27, no. 3 (September 2002): 234–36. http://dx.doi.org/10.1016/s0887-8994(02)00425-3.

Full text

APA, Harvard, Vancouver, ISO, and other styles

50

Labrune, P., A. Myara, C. Hennion, J. P. Gout, F. Trivin, and M. Odievre. "Crigler-Najjar type II disease inheritance: A family study." Journal of Inherited Metabolic Disease 12, no. 3 (September 1989): 302–6. http://dx.doi.org/10.1007/bf01799221.

Full text

APA, Harvard, Vancouver, ISO, and other styles

We offer discounts on all premium plans for authors whose works are included in thematic literature selections. Contact us to get a unique promo code!