Hepatic Parenchymal Injury in Crigler‐Najjar Type I

Mitchell, Ellen; Ranganathan, Sarangarajan; McKiernan, Patrick; Squires, Robert H.; Strauss, Kevin A.; Soltys, Kyle; Mazariegos, George; Squires, James E.

doi:10.1097/mpg.0000000000001843

Cited by 23 publications

(20 citation statements)

References 47 publications

(54 reference statements)

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“…Liver fibrosis appears to develop in a proportion of CNS‐I patients over time, as suggested by Mitchell et al, who found pericentral, periportal, and mixed patterns of fibrosis in nine (41%) of 22 CNS‐I explants at the time of transplant and noted that patients with fibrosis were older (16.1 years) than those without (10.5 years; P = 0.02).…”

Section: Resultsmentioning

confidence: 63%

“…Of these, three were general review articles, one of which also included results of an international survey . Of the remaining 25 publications, eight were case series or case reports, 10 were retrospective cohorts, three were prospective cohorts, two were RCTs, and two were cross‐sectional studies . Nineteen primary publications were specific to CNS, while six comprised patients with various inherited metabolic disorders including CNS .…”

Section: Resultsmentioning

confidence: 99%

“…Phototherapy appears less effective with advancing age for a number of reasons, including increasing skin thickness, increasing body surface to weight ratio, and restricted hepatobiliary clearance of lumirubin . This waning efficacy inevitably increases the risk of neurological injury and is a major reason why adolescent and adult CNS‐I patients usually require liver transplantation.…”

Section: Resultsmentioning

confidence: 99%

“…2 Of the remaining 25 publications, eight were case series 22,25,35,[37][38][39] or case reports, 24,40 10 were retrospective cohorts, 21,23,26,28,30,31,34,36,41,43 three were prospective cohorts, 29,32,33 two were RCTs, 27,44 and two were cross-sectional studies. 10,42 Nineteen primary publications were specific to CNS, 10,21,22,24,25,27,[29][30][31]34,35,[37][38][39][40][41][42][43][44] while six comprised patients with various inherited metabolic disorders including CNS. 23,26,28,32,33,36 Most studies were conducted in Europe or the USA, with others in Saudi Arabia, Turkey, Japan, and Australia.…”

Section: Resultsmentioning

confidence: 99%

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Disease burden of Crigler–Najjar syndrome: Systematic review and future perspectives

Dhawan

Lawlor

Mazariegos

et al. 2019

J of Gastro and Hepatol

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Background and Aim Crigler–Najjar syndrome (CNS) results from biallelic mutations of UGT1A1 causing partial or total loss of uridine 5′‐diphosphate glucuronyltransferase activity leading to unconjugated hyperbilirubinemia and its attendant risk for irreversible neurological injury (kernicterus). CNS is exceedingly rare and has been only partially characterized through relatively small studies, each comprising between two and 57 patients. Methods A systematic literature review was conducted to consolidate data on the patient, caregiver, and societal burden of CNS. Results Twenty‐eight articles on clinical aspects of CNS were identified, but no published data on its humanistic or economic burden were found. In patients with complete UGT1A1 deficiency (type 1 CNS [CNS‐I]), unconjugated bilirubin levels increase 3–6 mg/dL/day during the newborn period and reach neurologically dangerous levels between 5 and 14 days of age. Phototherapy is the mainstay of treatment but poses significant challenges to patients and their families. Despite consistent phototherapy, patients with CNS‐I have worsening hyperbilirubinemia with advancing age. Liver transplantation is the only definitive therapy for CNS‐I and is increasingly associated with excellent long‐term survival but also incurs high costs, medical and surgical morbidities, and risks of immunosuppression. Conclusions Crigler–Najjar syndrome is associated with a substantial burden, even with existing standards of care. The development of novel disease‐modifying therapies has the potential to reduce disease burden and improve the lives of CNS patients and their families.

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Section: Resultsmentioning

confidence: 63%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Disease burden of Crigler–Najjar syndrome: Systematic review and future perspectives

Dhawan

Lawlor

Mazariegos

et al. 2019

J of Gastro and Hepatol

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“…Artificial intelligence programs have been used widely to prevent the ordering of related tests in the analytical phase. AST is one such test, which is commonly used to screen for liver diseases; its levels increase correspondingly with ALT levels [8,9]. There are only a very few conditions under which ALT is found to be within normal ranges and where AST is elevated above the normal limits, hemolysis being the most common of those [10].…”

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confidence: 99%

Elimination of clinical biochemistry laboratory tests through artificial intelligence programs to increase cost-effectiveness

Gönel¹

2018

Ann Clin Anal Med

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Aim: The aim of the study was to evaluate the efficacy of software programs such as ALIN IQ, to prevent the regular practice of unnecessary testing in the preanalytical phase, which could save millions of dollars. Material and Method: This study was conducted using data obtained from certain tests using the ALIN IQ software developed by Abbott Core Laboratory. The software implements algorithms defined by the information management system of the hospital to manage laboratory test ordering by clinicians. In the study, three tests-AST, direct bilirubin, and free PSA-were blocked by the software. Instead, the software suggested ALT, total bilirubin, and total PSA tests as preferences when ordered along with other tests sent to the c16000 biochemistry analyzer. Results: In this study, data on numbers of tests (ALT, AST, PSA) run over a one-month period were acquired from ALIN IQ software in a laboratory that runs 2,444,024 tests per year. Findings showed that 11,137 AST, 6,856 direct bilirubin, and 1,340 free PSA tests were ordered unnecessarily over the course of the month, and that avoiding these three tests (AST, direct bilirubin, and free PSA) provided savings of 77.96% from AST, 77.22% from direct bilirubin, and 72.45% from free PSA, corresponding to a projected 231,996 unnecessary tests per year. Discussion: Given the rise of global healthcare costs, reducing laboratory expenditures costing billions of dollars has been discussed in many articles, and different suggestions have been put forward. The present study showed that in addition to the measures set in place in the preanalytical phase, the costs of medical tests could also be reduced through the use of intelligent software programs in the analytical phase.

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Disorders of Bilirubin Metabolism

Chowdhury

2009

The Liver

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Hepatic Parenchymal Injury in Crigler‐Najjar Type I

Cited by 23 publications

References 47 publications

Disease burden of Crigler–Najjar syndrome: Systematic review and future perspectives

Disease burden of Crigler–Najjar syndrome: Systematic review and future perspectives

Elimination of clinical biochemistry laboratory tests through artificial intelligence programs to increase cost-effectiveness

Disorders of Bilirubin Metabolism

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