The management of childhood liver diseases in adulthood

Joshi, Deepak; Gupta, Nitika; Samyn, Marianne; Deheragoda, Maesha; Dobbels, Fabienne; Heneghan, Michael A.

doi:10.1016/j.jhep.2016.11.013

Cited by 31 publications

(22 citation statements)

References 134 publications

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“…This is too late for a benefit to be obtained from early surgery(11) and demonstrates the need for continued education and awareness of the importance of early diagnosis of neonatal liver disease.An electronic questionnaire on continuity of care in transition arrangements for children with liver disease reaching adult life, prepared in collaboration with the British Society for Gastroenterology, will be sent to adult gastroenterology and hepatology units who have >3 WTE hepatologists or who meet Level 2 training criteria. A draft document specifying training requirements for Physicians caring for young adults with liver disease has also been submitted to the Specialist Advisory Committee in Gastroenterology and the management of childhood liver disease including the transition period featured in a recent publication in the Journal of Hepatology(12) but no metric data is available at present on what arrangements are in place.…”

mentioning

confidence: 99%

Disease burden and costs from excess alcohol consumption, obesity, and viral hepatitis: fourth report of the Lancet Standing Commission on Liver Disease in the UK

Alexander²,

et al. 2018

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This report contains new and follow-up metric data relating to the eight main recommendations of the Lancet Standing Commission on Liver Disease in the UK, which aim to reduce the unacceptable harmful consequences of excess alcohol consumption, obesity, and viral hepatitis. For alcohol, we provide data on alcohol dependence, damage to families, and the documented increase in alcohol consumption since removal of the above-inflation alcohol duty escalator. Alcoholic liver disease will shortly overtake ischaemic heart disease with regard to years of working life lost. The rising prevalence of overweight and obesity, affecting more than 60% of adults in the UK, is leading to an increasing liver disease burden. Favourable responses by industry to the UK Government's soft drinks industry levy have been seen, but the government cannot continue to ignore the number of adults being affected by diabetes, hypertension, and liver disease. New direct-acting antiviral drugs for the treatment of chronic hepatitis C virus infection have reduced mortality and the number of patients requiring liver transplantation, but more screening campaigns are needed for identification of infected people in high-risk migrant communities, prisons, and addiction centres. Provision of care continues to be worst in regions with the greatest socioeconomic deprivation, and deficiencies exist in training programmes in hepatology for specialist registrars. Firm guidance is needed for primary care on the use of liver blood tests in detection of early disease and the need for specialist referral. This report also brings together all the evidence on costs to the National Health Service and wider society, in addition to the loss of tax revenue, with alcohol misuse in England and Wales costing £21 billion a year (possibly up to £52 billion) and obesity costing £27 billion a year (treasury estimates are as high as £46 billion). Voluntary restraints by the food and drinks industry have had little effect on disease burden, and concerted regulatory and fiscal action by the UK Government is essential if the scale of the medical problem, with an estimated 63 000 preventable deaths over the next 5 years, is to be addressed.

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confidence: 99%

Disease burden and costs from excess alcohol consumption, obesity, and viral hepatitis: fourth report of the Lancet Standing Commission on Liver Disease in the UK

Alexander²,

et al. 2018

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“…CA therapy allows affected children to reach adulthood in good health condition without the need for a liver transplantation. Provided a successful transition and a follow-up during adulthood performed by a specialized hepatologist, it is likely that CA therapy will guarantee to patients, including patients diagnosed at adult age, a normal quality of life throughout all their lifetime [ 28 – 30 ].…”

Section: Discussionmentioning

confidence: 99%

Cholic acid for primary bile acid synthesis defects: a life-saving therapy allowing a favorable outcome in adulthood

Gonzalès¹,

Matarazzo²,

Franchi‐Abella³

et al. 2018

Orphanet J Rare Dis

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BackgroundOral cholic acid (CA) replacement has been shown to be an effective therapy in children with primary bile acid synthesis defects, which are rare and severe genetic liver diseases. To date there has been no report of the effects of this therapy in children reaching adulthood. The aim of the study was to evaluate the long-term effectiveness and safety of CA therapy.MethodsFifteen patients with either 3β-hydroxy-Δ5-C27-steroid oxidoreductase (3β-HSD) (n = 13) or Δ4–3-oxosteroid 5β-reductase (Δ4–3-oxo-R) (n = 2) deficiency confirmed by mass spectrometry and gene sequencing received oral CA and were followed prospectively.ResultsThe median age at last follow-up and the median time of follow-up with treatment were 24.3 years (range: 15.3–37.2) and 21.4 years (range: 14.6–24.1), respectively. At last evaluation, physical examination findings and blood laboratory test results were normal in all patients. Liver sonograms were normal in most patients. Mean daily CA dose was 6.9 mg/kg of body weight. Mass spectrometry analysis of urine showed that excretion of the atypical metabolites remained low or traces in amount with CA therapy. Liver fibrosis scored in liver biopsies or assessed by elastography in 14 patients, after 10 to 24 years with CA therapy, showed a marked improvement with disappearance of cirrhosis (median score < F1; range: F0-F2). CA was well tolerated in all patients, including five women having 10 uneventful pregnancies during treatment.ConclusionsOral CA therapy is a safe and effective long-term treatment of 3β-HSD and Δ4–3-oxo-R deficiencies and allows affected children to reach adulthood in good health condition without the need for a liver transplantation.

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“…Furthermore, the successes of the discipline had led to new needs: a new generation of young adults are now coming to see our adult colleagues with entirely new diagnostic, psychosocial and therapeutic problems. This can only be solved with transition clinics, where paediatricians and adult physicians collaborate with regard to patients and diseases .…”

Section: Liver Transplantsmentioning

confidence: 99%

The coming of age of a young subspecialty: paediatric hepatology

Németh

2017

Acta Paediatrica

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Paediatric hepatology dates from the 1970s and it is the youngest of the organ-specific subspecialties. As then there have been impressive achievements in the fields of anatomical, metabolic, immunological and neoplastic diseases, and the advent of modern molecular biology has resulted in a marked increase in exact diagnoses. Liver transplants provided enormous stimulus for the discipline. Due to changing morbidity patterns, the discipline faces new challenges, such as environment-and lifestyle-induced liver diseases, but different forms of chronic viral hepatitis are diminishing. Conclusion: High levels of competence require good clinical research, optimal results and a high degree of centralisation.

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The management of childhood liver diseases in adulthood

Cited by 31 publications

References 134 publications

Disease burden and costs from excess alcohol consumption, obesity, and viral hepatitis: fourth report of the Lancet Standing Commission on Liver Disease in the UK

Disease burden and costs from excess alcohol consumption, obesity, and viral hepatitis: fourth report of the Lancet Standing Commission on Liver Disease in the UK

Cholic acid for primary bile acid synthesis defects: a life-saving therapy allowing a favorable outcome in adulthood

The coming of age of a young subspecialty: paediatric hepatology

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