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Inherited Metabolic Disorders

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Book cover Pediatric Hepatology and Liver Transplantation

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Abstract

Paediatric hepatologist has become increasingly involved in management of IMDs due to their better diagnostics, considerable clinical overlap with liver diseases and possibility of LT as long-term treatment option. This overview presents some clinical background for the commonest of the IMDs potentially managed by LT but also attempts to assess risk/benefit ratios in specific contexts. The potential benefits of minimising developmental delay, which is a common feature of medically treated IMDs, represent a strong drive to performing these elective operations earlier in childhood. However, surgical and perioperative issues need to be borne in mind in order to optimise outcome for this unique group of patients.

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Authors and Affiliations

  1. Kings College Hospital, London, UK

    Nedim Hadzic

  2. Paediatric Inherited Metabolic Disease, Evelina Children’s Hospital, London, UK

    Roshni Vara

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  1. Nedim Hadzic
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  2. Roshni Vara
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Correspondence to Nedim Hadzic .

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Editors and Affiliations

  1. Paediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII Bergamo, Bergamo, Italy

    Lorenzo D'Antiga

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Hadzic, N., Vara, R. (2019). Inherited Metabolic Disorders. In: D'Antiga, L. (eds) Pediatric Hepatology and Liver Transplantation. Springer, Cham. https://doi.org/10.1007/978-3-319-96400-3_16

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  • DOI: https://doi.org/10.1007/978-3-319-96400-3_16

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