&lt;p&gt;Cholestasis In Infants With Down Syndrome Is Not Due To Extrahepatic Biliary Atresia: A Ten-Year Single Egyptian Centre Experience&lt;/p&gt;

Kotb, Magd A.; Draz, Iman H.; Basanti, Christine William Shaker; Sorogy, Sally T. Mostafa El; Elkader, Hesham M Abd; Esmat, Haytham; Baky, Hend Abd El; Mosallam, Dalia

doi:10.2147/ceg.s216189

Cited by 5 publications

(5 citation statements)

References 17 publications

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“…UDCA has been reported to be ineffective and unsafe among neonates and children with cholestasis and liver disease and has been contraindicated among Egyptian children since 2018. 9-11, 47 Handy off-label use of UDCA has precluded double-blind prospective clinical trials of this drug in children with different ethnicities, and there is a lack of evidence to support its use among children.…”

Section: Discussionmentioning

confidence: 99%

Medical-grade Spore-free Natural Honey is an Effective Choleretic in Neonatal Cholestasis: A Pilot Single-center Trial

Kotb¹,

Satar²,

Badr³

et al. 2023

Gene Expr

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Background and objectives: Liver damage in cholestasis is multifactorial, yet bile acid-mediated hepatotoxicity is pivotal. Honey consumption has many physiological effects; it influences detoxification process (phase I, II and III), has antioxidant, anti-inflammatory, immune-stimulant, anti-ulcer, wound/burn healing effects and others. The bile acid ursodeoxycholic acid (UDCA) is currently used off-label to treat neonatal cholestasis. This study aimed to assess the effectiveness of spore-free natural honey to treat neonatal nonobstructive cholestasis.Methods: Thirty infants with cholestasis received spore-free natural honey. Their progression was compared to a control group with cholestasis (28 infants) and a historical group (31 infants) on UDCA. Results:The mean ± standard deviation (SD) follow-up duration was 29.68 ± 18.68 months. At presentation, the total bilirubin concentrations were 9.5 ± 5.9 mg/dL, 10.9 ± 7 mg/dL and 14 ± 9 mg/dL in the honey, control and UDCA groups, respectively (p = 0.064), and their direct bilirubin concentrations were 6 ± 4 mg/dL, 6.7 ± 4 mg/dL and 8.4 ± 6.9 mg/dL, respectively (p = 0.169). The final total bilirubin concentrations were 1.2 ± 1.7 mg/dL, 4.6 ± 8.2 mg/dL and 7.3 ± 8.9 mg/dL, (p = 0.006), and their final direct bilirubin concentrations were 0.8 ± 1 mg/dL, 2.77 ± 5.2 mg/dL and 5.1 ± 7.2 mg/dL (p = 0.008), with cure achievement in 25, 16 and 16 (p = 0.023), improvement in 3, 5 and 3 (p = 0.565), failure in 2, 3 and 10 (p = 0.016), and death in 0, 4 and 2, respectively (p = 0.095). None suffered from botulism or flaccid paralysis.Conclusions: Spore-free honey is effective in clearing cholestasis in neonates and infants. UDCA use in cholestasis in pediatric age should be abandoned as it is less effective and is associated with a worse outcome.

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Section: Discussionmentioning

confidence: 99%

Medical-grade Spore-free Natural Honey is an Effective Choleretic in Neonatal Cholestasis: A Pilot Single-center Trial

Kotb¹,

Satar²,

Badr³

et al. 2023

Gene Expr

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“…Other crucial determinants of phenotype seem to be the neutrophil elastase attack on aflatoxin damaged hepatocytes and cholangiocytes, as children with Down syndrome who are known to have neutrophil function defects do not develop biliary atresia at all (27). It seems that more factors interact to produce the BA Kotb disease variant and the very poor outcome of native livers despite early prompt portoenterostomy.…”

Section: Discussionmentioning

confidence: 99%

“…It is not clear if the constant ursodeoxycholic acid deleterious effect among Egyptian children is a racial susceptibility. Its use among children with liver disease should be strictly within institutionally approved research premise (16) with frequent regular assessment of lithocholic acid (27,(32)(33)(34).…”

Section: Discussionmentioning

confidence: 99%

P53 Mutation at Codon 249 is Uncommon in Neonatal Kotb Disease Biliary Atresia

Kotb,

Kelany,

Shehata

et al. 2023

Pediatric Sciences Journal

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Background: Hepatocellular carcinoma (HCC) is known to result from aflatoxin B1 that induces p53 mutation at codon 249. Aflatoxins are also known to cause The Kotb disease Biliary atresia (BA) variant which is characterized by congenital aflatoxicosis B1 in neonates with null glutathione S transferase M1. Aim of the Work:We aimed to search for the aflatoxin B1 induced HCC 249 codon p53 mutation among neonates with Kotb disease BA variant and their mothers. Patients and Methods: This study included 13 neonates and infants with confirmed BA who presented to Hepatology Clinic, New Children Hospital, Cairo University, Egypt during January-May 2019. All subjects and their mothers underwent detection of aflatoxins from peripheral blood. BA cases underwent detection of mutation from liver biopsy tissue as well. Results: The studied cohort with confirmed BA comprised 9 (69.2%) girls and 4 (30.8%) boys, with mean ages ± standard deviation (SD) at onset, presentation, diagnosis and portoenterostomy of 8.1±5.7 days, 44.8±11 days, 57±14.53 and 64.5±21.34 days respectively. All 13 and their mothers were found to have elevated blood levels of aflatoxin B1 with a mean of 8.56 ± 4.2ng/ml and 14.75±16.78ng/ml respectively. The mean ± SD duration of follow up was 259.1±141 days. None of the mothers had abnormal levels of bilirubin or liver aminotransferases. All samples tested negative for p53 mutation at codon 249 except for one infant who tested negative for the mutation in his whole blood and had heterozygous mutation in DNA from his liver tissue. None of the studied cohort or their mothers had HCC. Cholestasis resolved in 2 children, 7 had progressive course and 4 died. There was no correlation between outcome and neonate/maternal aflatoxin B1 level (p=0.299; p=0.443), age at portoenterostomy (p=0.93), hepatic fibrosis degree (p=0.56), or other lab or liver biopsy findings. Conclusion: p53 mutation at codon 249 is uncommon in infants with Kotb disease BA variant, despite the aflatoxicosis they suffer from. The cause remains to be studied. Screening for p53 mutation at codon 249 cannot be used as a diagnostic test for Kotb disease.

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“…The multifactorial etiology of BA is not limited to genetic susceptibility to immune dysregulation or detoxification defects such as GST M1 deficiency or fibrinogenesis. There are no reports of BA in children with Down syndrome ( 9 ); hence, it seems that there are genetic protective sentinels against BA that remain to be explored. This protective gene(s) might be the focus of future research and may be usable within gene therapy.…”

Section: The Multifactorial Etiology and Genetic Susceptibility To Bi...mentioning

confidence: 99%

Editorial: Elimination of biliary atresia

2023

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<p>Cholestasis In Infants With Down Syndrome Is Not Due To Extrahepatic Biliary Atresia: A Ten-Year Single Egyptian Centre Experience</p>

Cited by 5 publications

References 17 publications

Medical-grade Spore-free Natural Honey is an Effective Choleretic in Neonatal Cholestasis: A Pilot Single-center Trial

Medical-grade Spore-free Natural Honey is an Effective Choleretic in Neonatal Cholestasis: A Pilot Single-center Trial

P53 Mutation at Codon 249 is Uncommon in Neonatal Kotb Disease Biliary Atresia

Editorial: Elimination of biliary atresia

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