Evidence map›Paper›PMID 30555131›Full record

ReviewJournal of atherosclerosis and thrombosis2019

Liver Transplantation for Homozygous Familial Hypercholesterolemia.

Yasushi Ishigaki, Naoki Kawagishi, Yutaka Hasegawa, Shojiro Sawada, Hideki Katagiri, Susumu Satomi, Shinichi Oikawa

Open access · diamondAbstract readReview
In one paragraph

Review in Journal of atherosclerosis and thrombosis, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
27citing papers in PubMed, 2 pooled it
4.0field-weighted citation impact, top 6% 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

27 citing papers in PubMed, 2 syntheses or guidelines pooled it, 64 citations in OpenAlex.

  1. Pooled it
  2. Combined heart and liver transplantation: an updated systematic review.Annals of the Royal College of Surgeons of England · 2022
    Pooled it
  3. Review
  4. Review
  5. Article
  6. Liver transplantation for co-existing biliary atresia and familial hypercholesterolemia.Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology · 2024
    Article
  7. Familial hypercholesterolemia.Indian heart journal · 2024
    Review
  8. Article
  9. Review
  10. Review
  11. Article
  12. 2023 China Guidelines for Lipid Management.Journal of geriatric cardiology : JGC · 2023
    Article
  13. Article
  14. Review
  15. Review
  16. 2023 Chinese guideline for lipid management.Frontiers in pharmacology · 2023
    Review
  17. Review
  18. Review
  19. Article
  20. Review
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

7 authors at 4 institutions in 1 country.

Yasushi IshigakiDivision of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Iwate Medical University.
Naoki KawagishiDivision of Transplantation Surgery, JCHO Sendai Hospital.
Yutaka HasegawaDivision of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Iwate Medical University.
Shojiro SawadaDepartment of Metabolism and Diabetes, Tohoku University Graduate School of Medicine.
Hideki KatagiriDepartment of Metabolism and Diabetes, Tohoku University Graduate School of Medicine.
Susumu SatomiThe Division of Advanced Surgical Science and Technology, Graduate School of Medicine, Tohoku University.
Shinichi OikawaDiabetes and Lifestyle-related Disease Center, Fukujuji Hospital, Japan Anti-Tuberculosis Association.
Tohoku University · JPIwate Medical University · JPJapan Anti Tuberculosis Association · JPSendai Shakai Hoken Hospital · JP

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Pharmacological treatments to decrease low-density lipoprotein (LDL) cholesterol (LDL-C) have limited effects on patients with homozygous familial hypercholesterolemia (HoFH). Since LDL receptors are located mainly in the liver, liver transplantation is considered to be the only way to correct the hepatic cholesterol metabolism abnormalities in HoFH. Liver transplantations, including those combined with heart transplantation, for HoFH have been increasing since 1984, making this a globally established therapeutic option for HoFH. Plasma LDL-C is reported to be dramatically lowered, by 80%, after transplantation, with the rapid regression of cutaneous and tendinous xanthomas. However, long-term cardiovascular benefits remain unclear. The major concerns about liver transplantation include surgical complications, the need for lifelong immunosuppressive therapy, and rejection. In addition, organ transplantations from deceased donors are extremely rare in Japan. We experienced two pediatric siblings with HoFH who received living-donor liver transplantations from their heterozygous parents. Their plasma LDL-C levels decreased immediately and stabilized at approximately 200 mg/dL. Both developed normally with the administration of lipid-lowering medications and have been free of severe problems for more than 10 years, to date, since transplantation. In Japan, where the shortage of deceased donors is critical, the combination of living-donor liver transplant from a heterozygous donor, that is, usually a parent, and medication is regarded as a valid therapeutic option for HoFH. Further studies and clinical experience are required to establish liver transplantation as a safe and effective treatment for HoFH.

Indexed as

HomozygoteHumansHyperlipoproteinemia Type IILiver TransplantationPrognosisCholesterol metabolismFamilial hypercholesterolemiaLiver transplantation

Identifiers

PMID30555131
PMCPMC6365147
OpenAlexW2904411616

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-SA
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.