Pathobiology of the Hepatic Glycogen Storage Diseases

Pathobiology of the Hepatic Glycogen Storage Diseases Purpose of the Review The purpose of this study is to review nificantly improved their outcome and improved metabolic the pathobiology of the hepatic glycogen storage diseases control can prevent many long-term complications. (GSD) and recent developments in their management. Recent Findings Increasingly, noninvasive mutation detec- Keywords Glycogen storage disease Inherited metabolic . . . tion has replaced biological and tissue samples as the first disorder Dietary treatment Metabolic control Hepatic . . line diagnostic method. For type 1 GSD, continuous sub- adenomata Hepatocellular carcinoma Liver transplantation cutaneous monitoring has transformed patient monitoring. There is evidence that improved metabolic control can prevent complications such as adenoma formation and Introduction renal dysfunction. Improved understanding of the patho- physiology of hepatic adenomata may allow rationaliza- Short-term blood glucose control is mediated by the liver via tion of their management. Hepatocellular carcinoma only glucose-6-phosphate (G6P). Postprandially, the liver imports develops in established adenomata, offering an option for glucose to form G6P which is used for glycogen synthesis. prevention. For type 111 GSD, intrinsic liver disease and The process is then reversed resulting in hepatic glucose ex- myopathy become the major clinical concerns after child- port by dephosphorylation of G6P by glucose-6-phosphatase hood. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Current Pathobiology Reports Springer Journals

Pathobiology of the Hepatic Glycogen Storage Diseases

Loading next page...
 
/lp/springer_journal/pathobiology-of-the-hepatic-glycogen-storage-diseases-xmxUw9PcmQ
Publisher
Springer US
Copyright
Copyright © 2017 by Springer Science+Business Media New York
Subject
Medicine & Public Health; Pathology
eISSN
2167-485X
D.O.I.
10.1007/s40139-017-0146-6
Publisher site
See Article on Publisher Site

Abstract

Purpose of the Review The purpose of this study is to review nificantly improved their outcome and improved metabolic the pathobiology of the hepatic glycogen storage diseases control can prevent many long-term complications. (GSD) and recent developments in their management. Recent Findings Increasingly, noninvasive mutation detec- Keywords Glycogen storage disease Inherited metabolic . . . tion has replaced biological and tissue samples as the first disorder Dietary treatment Metabolic control Hepatic . . line diagnostic method. For type 1 GSD, continuous sub- adenomata Hepatocellular carcinoma Liver transplantation cutaneous monitoring has transformed patient monitoring. There is evidence that improved metabolic control can prevent complications such as adenoma formation and Introduction renal dysfunction. Improved understanding of the patho- physiology of hepatic adenomata may allow rationaliza- Short-term blood glucose control is mediated by the liver via tion of their management. Hepatocellular carcinoma only glucose-6-phosphate (G6P). Postprandially, the liver imports develops in established adenomata, offering an option for glucose to form G6P which is used for glycogen synthesis. prevention. For type 111 GSD, intrinsic liver disease and The process is then reversed resulting in hepatic glucose ex- myopathy become the major clinical concerns after child- port by dephosphorylation of G6P by glucose-6-phosphatase hood.

Journal

Current Pathobiology ReportsSpringer Journals

Published: Jun 6, 2017

There are no references for this article.

You’re reading a free preview. Subscribe to read the entire article.


DeepDyve is your
personal research library

It’s your single place to instantly
discover and read the research
that matters to you.

Enjoy affordable access to
over 18 million articles from more than
15,000 peer-reviewed journals.

All for just $49/month

Explore the DeepDyve Library

Search

Query the DeepDyve database, plus search all of PubMed and Google Scholar seamlessly

Organize

Save any article or search result from DeepDyve, PubMed, and Google Scholar... all in one place.

Access

Get unlimited, online access to over 18 million full-text articles from more than 15,000 scientific journals.

Your journals are on DeepDyve

Read from thousands of the leading scholarly journals from SpringerNature, Elsevier, Wiley-Blackwell, Oxford University Press and more.

All the latest content is available, no embargo periods.

See the journals in your area

DeepDyve

Freelancer

DeepDyve

Pro

Price

FREE

$49/month
$360/year

Save searches from
Google Scholar,
PubMed

Create lists to
organize your research

Export lists, citations

Read DeepDyve articles

Abstract access only

Unlimited access to over
18 million full-text articles

Print

20 pages / month

PDF Discount

20% off