Evidence map›Paper›PMID 40780791›Full record

ArticleJACC. Case reports2025

Atrioventricular Block in a Pompe Disease Patient Receiving Enzyme Replacement Therapy.

Kentaro Akagi, Shiro Baba, Koichi Matsuda, Takeshi Yoshida, Takuya Hirata, Atsushi Yokoyama, Fumiya Yoneda, Fumiaki Fukumura, Eitaro Kume, Junko Takita

Abstract readCase Reports
In one paragraph

Article in JACC. Case reports, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
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

1 citing paper in PubMed.

  1. 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

10 authors.

Kentaro AkagiDepartment of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan. Electronic address: k_akagi@kuhp.kyoto-u.ac.jp.
Shiro BabaDepartment of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Koichi MatsudaDepartment of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Takeshi YoshidaDepartment of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Takuya HirataDepartment of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Atsushi YokoyamaDepartment of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Fumiya YonedaDepartment of Cardiovascular Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Fumiaki FukumuraDepartment of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Eitaro KumeDepartment of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Junko TakitaDepartment of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundInfantile-onset Pompe disease (IOPD) is a lethal disorder, but enzyme replacement therapy (ERT) has dramatically improved the prognosis of these patients. CASE SUMMARY: An 18-year-old male patient, who had been diagnosed with IOPD based on decreased α-glucosidase activity in his infancy and received ERT, visited an emergency department at Kyoto University Hospital with poor complexion. An electrocardiogram showed extreme bradycardia and complete atrioventricular block (cAVB), and permanent pacemaker was implanted for cAVB. DISCUSSION: ERT has reduced the risk of death in patients with IOPD; on the other hand, severe cAVB in those patients needs to be recognized as a rare complication. TAKE-HOME MESSAGE: cAVB is a rare but critical complication in long-term survivors of IOPD and deserves attention.

Indexed as

bradycardiacardiac pacemakercardiomypathy

Identifiers

PMID40780791
PMCPMC12426544

What OpenQuestion holds

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Registered trials

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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.