Evidence map›Paper›PMID 10603098›Full record

ReviewEuropean journal of pediatrics1999

Liver transplantation for glycogen storage disease types I, III, and IV.

D Matern, T E Starzl, W Arnaout, J Barnard, J S Bynon, A Dhawan, J Emond, E B Haagsma, G Hug, A Lachaux and 2 more

Open access · greenAbstract readReview
In one paragraph

Review in European journal of pediatrics, 1999. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 50 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
50citing papers in PubMed, 2 pooled it
3.5field-weighted citation impact, top 8% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

50 citing papers in PubMed, 2 syntheses or guidelines pooled it, 187 citations in OpenAlex.

  1. Guideline
  2. Pooled it
  3. Review
  4. Review
  5. Article
  6. Article
  7. Review
  8. Glycogen storage diseases.Nature reviews. Disease primers · 2023
    Review
  9. Glycogen storage diseases: An update.World journal of gastroenterology · 2023
    Review
  10. Article
  11. Article
  12. Article
  13. Article
  14. Article
  15. Review
  16. Review
  17. Article
  18. Article
  19. Liver fibrosis during clinical ascertainment of glycogen storage disease type III: a need for improved and systematic monitoring.Genetics in medicine : official journal of the American College of Medical Genetics · 2019
    Article
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

12 authors at 11 institutions in 4 countries.

D MaternDepartment of Pediatrics, Duke University Medical Center, P.O. Box 3528, Durham, NC 27710, USA.
T E Starzl
W Arnaout
J Barnard
J S Bynon
A Dhawan
J Emond
E B Haagsma
G Hug
A Lachaux
G P Smit
Y T Chen
University of Groningen · NLCedars-Sinai Medical Center · USCincinnati Children's Hospital Medical Center · USDuke Medical Center · USDuke University Hospital · USHôpital Edouard Herriot · FRThe King's College · USUniversity of Alabama at Birmingham · USUniversity of California, San Francisco · USUniversity of Pittsburgh · USVanderbilt University Medical Center · US

Funding

ORTHOTOPIC LIVER TRANSPLANTATIONR01DK029961 · NIDDK · UNIVERSITY OF PITTSBURGH AT PITTSBURGH · PI STARZL, THOMAS E · 1986 to 2001
$876k
MOLECULAR MECHANISM IN TYPE III GLYCOGEN STORAGE DISEASER01DK039078 · NIDDK · DUKE UNIVERSITY · PI CHEN, YUAN-TSONG YT · 1987 to 2002
$695k
NIDDK NIH HHS DK39078NIDDK NIH HHS R01 DK029961
6 · The paper itself

Abstract

unlabelledGlycogen storage disease (GSD) types I, III, and IV can be associated with severe liver disease. The possible development of hepatocellular carcinoma and/or hepatic failure make these GSDs potential candidates for liver transplantation. Early diagnosis and initiation of effective dietary therapy have dramatically improved the outcome of GSD type I by reducing the incidence of liver adenoma and renal insufficiency. Nine type I and 3 type III patients have received liver transplants because of poor metabolic control, multiple liver adenomas, or progressive liver failure. Metabolic abnormalities were corrected in all GSD type I and type III patients, while catch-up growth was reported only in two patients. Whether liver transplantation results in reversal and/or prevention of renal disease remains unclear. Neutropenia persisted in both GSDIb patients post liver transplantation necessitating continuous granulocyte colony stimulating factor treatment. Thirteen GSD type IV patients were liver transplanted because of progressive liver cirrhosis and failure. All but one patient have not had neuromuscular or cardiac complications during follow-up periods for as long as 13 years. Four have died within a week and 5 years after transplantation. Caution should be taken in selecting GSD type IV candidates for liver transplantation because of the variable phenotype, which may include life-limiting extrahepatic manifestations. It remains to be evaluated, whether a genotype-phenotype correlation exists for GSD type IV, which may aid in the decision making.

conclusionLiver transplantation should be considered for patients with glycogen storage disease who have developed liver malignancy or hepatic failure, and for type IV patients with the classical and progressive hepatic form.

Indexed as

Liver TransplantationAdolescentAdultChildFemaleGlycogen Storage Disease Type IGlycogen Storage Disease Type IIIGlycogen Storage Disease Type IVHumansLiver DiseasesMaleNeutropeniaPrognosis

Identifiers

PMID10603098
PMCPMC3006437
OpenAlexW1983230618

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.