Evidence map›Paper›PMID 38193991›Full record

ArticleClinical and experimental nephrology2024

A case series of Fabry diseases with CKD in Japan.

Oi Yusei, Hajime Nagasu, Naoki Nakagawa, Seigo Terawaki, Takahito Moriwaki, Seiji Itano, Seiji Kishi, Tamaki Sasaki, Naoki Kashihara, Takanobu Otomo

Abstract readMulticenter Study
In one paragraph

Article in Clinical and experimental nephrology, 2024. 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.

Oi YuseiDepartment of Health Informatics, Faculty of Health and Welfare Services Administration, Kawasaki University of Medical Welfare, Kurashiki, Okayama, Japan.
Hajime NagasuDepartment of Nephrology and Hypertension, Kawasaki Medical School, 577 Matsushima, Kurashiki, Okayama, 701-0192, Japan. hajimenagasu@kms-ndh.com.ORCID http://orcid.org/0000-0003-2549-4126
Naoki NakagawaDivision of Cardiology, Nephrology, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan.
Seigo TerawakiDepartment of Molecular and Genetic Medicine, Kawasaki Medical School, 577 Matsushima, Kurashiki, Okayama, 701-0192, Japan.
Takahito MoriwakiDepartment of Molecular and Genetic Medicine, Kawasaki Medical School, 577 Matsushima, Kurashiki, Okayama, 701-0192, Japan.
Seiji ItanoDepartment of Nephrology and Hypertension, Kawasaki Medical School, 577 Matsushima, Kurashiki, Okayama, 701-0192, Japan.
Seiji KishiDepartment of Nephrology and Hypertension, Kawasaki Medical School, 577 Matsushima, Kurashiki, Okayama, 701-0192, Japan.
Tamaki SasakiDepartment of Nephrology and Hypertension, Kawasaki Medical School, 577 Matsushima, Kurashiki, Okayama, 701-0192, Japan.
Naoki KashiharaDepartment of Nephrology and Hypertension, Kawasaki Medical School, 577 Matsushima, Kurashiki, Okayama, 701-0192, Japan.
Takanobu OtomoDepartment of Molecular and Genetic Medicine, Kawasaki Medical School, 577 Matsushima, Kurashiki, Okayama, 701-0192, Japan. otomo@med.kawasaki-m.ac.jp.

Funding

Wellcome Trust FC001048
6 · The paper itself

Abstract

backgroundIt is well known that kidney injury is vital organ damage in Fabry disease (FD). Renin-angiotensin system (RAS) inhibitors are known to reduce proteinuria in patients with chronic kidney disease (CKD) by dilating the glomerular export arteries and reducing intraglomerular pressure. This improvement in intraglomerular pressure, although lowering the glomerular filtration rate, is thought to prevent renal damage and be renoprotective in the long term. RAS inhibitors may be effective in FD patients with proteinuria to prevent the progression of kidney disease, however, the degree to which they are used in clinical practice is unknown.

methodsThe J-CKD-DB-Ex is a comprehensive multicenter database that automatically extracts medical data on CKD patients. J-CKD-DB-Ex contains data on 187,398 patients in five medical centers. FD patients were identified by ICD-10. Clinical data and prescriptions of FD patients between January 1 of 2014, and December 31 of 2020 were used for the analysis.

resultsWe identified 39 patients with FD from the J-CKD-DB-Ex including those with suspected FD. We confirmed 22 patients as FD. Half of the patients received RAS inhibitors. RAS inhibitors tended to be used in CKD patients with more severe renal impairment.

conclusionsThis case series revealed the actual clinical practice of FD patients with CKD. In particular, we found cases in which patients had proteinuria, but were not treated with RAS inhibitors. The database was shown to be useful in assessing the clinical patterns of patients with rare diseases.

Indexed as

Fabry DiseaseRenal Insufficiency, ChronicAdolescentAdultAgedAngiotensin-Converting Enzyme InhibitorsDatabases, FactualFemaleGlomerular Filtration RateHumansJapanMaleMiddle AgedProteinuriaRenin-Angiotensin SystemYoung AdultAngiotensin-Converting Enzyme InhibitorsFabry diseasesRAS inhibitorsReal-world dataSS-MIX2

Identifiers

PMID38193991
PMCPMC11033225

What OpenQuestion holds

Textmetadata
LicenceCC BY
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.