Evaluation of the role of bile acids and serotonin as markers of pruritus in children with chronic cholestatic liver disease

Koofy, Nehal El; Yassin, Noha A.; Okasha, Sawsan; William, Hany; Elakel, Wafaa; Elshiwy, Yasmine

doi:10.1016/j.ajg.2021.04.001

Cited by 4 publications

(3 citation statements)

References 22 publications

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“…Itch in patients with chronic pruritus is decreased by treatment with selective serotonin reuptake inhibitors (SSRI) such as fluvoxamine and paroxetine [ 72 , 73 ]. However, serum serotonin levels do not correlate highly with the clinical severity of itch in children with chronic cholestatic liver disease [ 74 ]. The mechanisms of itch may differ between diseases and it is unclear whether elevated serotonin might contribute to the pruritus in SLS.…”

Section: Discussionmentioning

confidence: 99%

Untargeted Metabolomic Analysis of Sjögren–Larsson Syndrome Reveals a Distinctive Pattern of Multiple Disrupted Biochemical Pathways

et al. 2023

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Sjögren–Larsson syndrome (SLS) is a rare inherited neurocutaneous disease characterized by ichthyosis, spastic diplegia or tetraplegia, intellectual disability and a distinctive retinopathy. SLS is caused by bi-allelic mutations in ALDH3A2, which codes for fatty aldehyde dehydrogenase (FALDH) and results in abnormal lipid metabolism. The biochemical abnormalities in SLS are not completely known, and the pathogenic mechanisms leading to symptoms are still unclear. To search for pathways that are perturbed in SLS, we performed untargeted metabolomic screening in 20 SLS subjects along with age- and sex-matched controls. Of 823 identified metabolites in plasma, 121 (14.7%) quantitatively differed in the overall SLS cohort from controls; 77 metabolites were decreased and 44 increased. Pathway analysis pointed to disrupted metabolism of sphingolipids, sterols, bile acids, glycogen, purines and certain amino acids such as tryptophan, aspartate and phenylalanine. Random forest analysis identified a unique metabolomic profile that had a predictive accuracy of 100% for discriminating SLS from controls. These results provide new insight into the abnormal biochemical pathways that likely contribute to disease in SLS and may constitute a biomarker panel for diagnosis and future therapeutic studies.

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Section: Discussionmentioning

confidence: 99%

Untargeted Metabolomic Analysis of Sjögren–Larsson Syndrome Reveals a Distinctive Pattern of Multiple Disrupted Biochemical Pathways

et al. 2023

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“…At the time this study commenced, existing COAs to measure pruritus in clinical settings [ 40 ] had been developed primarily for specific dermatologic conditions (e.g., the Patient-Oriented SCORing Atopic Dermatitis [PO-SCORAD] [ 41 ]) or were primarily characterized in adult populations (e.g., the 5-D itch scale [ 28 ] and Visual Analog, Numerical Rating, and Verbal Rating Scales [ 31 ]). While these measures have been adapted for clinical use in pediatric patients with CLD [ 42 , 43 ], few scales have been specifically developed and validated in this patient population [ 11 ]. Limitations of existing scales designed for use in pediatric patients with CLD include lack of validation [ 33 , 44 ] or generation of clinician-reported scores only and/or assessment of pruritus without considering the burden on the patient more generally (i.e., the Whitington-itch scale/CSS) [ 35 , 45 ].…”

Section: Discussionmentioning

confidence: 99%

Development of the Patient- and Observer-Reported PRUCISION Instruments to Assess Pruritus and Sleep Disturbance in Pediatric Patients with Cholestatic Liver Diseases

Gwaltney¹,

Bean

Venerus

et al. 2022

Adv Ther

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Introduction: Understanding how patients experience their disease is a vital step in optimal disease management, and patient-and observerreported outcome (PRO and ObsRO, respectively) measures can add important details to clinical information that is obtained as novel treatments are developed. Instruments that measure meaningful symptoms and impacts from the perspective of pediatric patients with cholestatic liver disease or their caregivers are needed. This study aimed to identify salient concepts in pediatric cholestatic liver disease, develop novel PRO and ObsRO instruments, and establish the instruments' content validity. Methods: Relevant signs, symptoms, and impacts of cholestatic liver disease were Natalie Warholic, Lise Kjems, and Patrick Horn: Employees of Albireo Pharma at the time of the study. C. Gwaltney (&)

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“…Duodenal biliary TBA (dTBA), dTBA/sTBA ratio, and dTBA/ serum GGT ratio were lower in infants with biliary atresia than healthy infants 56 . Bile acids have not been shown to correlate with the severity of pruritis in childhood cholestatic disorders 57 . Serum primary BAs (especially GCDCA and TCDCA) are higher in cholestasis due to biliary atresia than due to other causes and may help in non-invasively distinguishing etiology of childhood cholestasis 58…”

Section: Pediatric Cholestatic Disordersmentioning

confidence: 98%

A Current Understanding of Bile Acids in Chronic Liver Disease

Farooqui

Elhence

Shalimar

2022

Journal of Clinical and Experimental Hepatology

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This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Evaluation of the role of bile acids and serotonin as markers of pruritus in children with chronic cholestatic liver disease

Cited by 4 publications

References 22 publications

Untargeted Metabolomic Analysis of Sjögren–Larsson Syndrome Reveals a Distinctive Pattern of Multiple Disrupted Biochemical Pathways

Untargeted Metabolomic Analysis of Sjögren–Larsson Syndrome Reveals a Distinctive Pattern of Multiple Disrupted Biochemical Pathways

Development of the Patient- and Observer-Reported PRUCISION Instruments to Assess Pruritus and Sleep Disturbance in Pediatric Patients with Cholestatic Liver Diseases

A Current Understanding of Bile Acids in Chronic Liver Disease

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