Evidence mapPaperPMID 40764872Full record

ArticleCEN case reports2025

A case of Frasier syndrome achieved complete remission by cyclosporine A for steroid-resistant nephrotic syndrome.

Yasuhiro Inaba, Akinori Miyazono, Takuro Mitsunobu, Yuko Seki, Kandai Nozu, China Nagano, Yasuhiro Okamoto

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Article in CEN case reports, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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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.

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4 · The record

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5 · Who and what money

Authors and funding

7 authors.

Yasuhiro InabaDepartment of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. y-i-0602@outlook.jp.ORCID http://orcid.org/0000-0001-9411-4786
Akinori MiyazonoDepartment of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
Takuro MitsunobuDepartment of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
Yuko SekiDepartment of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
Kandai NozuDepartment of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.
China NaganoDepartment of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.
Yasuhiro OkamotoDepartment of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

In pediatric steroid-resistant nephrotic syndrome (SRNS), causative genetic abnormalities are now being identified. Immunosuppressive therapy is generally believed to be ineffective in treating Frasier syndrome, which is associated with genetic abnormalities. Recently, the efficacy of cyclosporine A (CyA) via a non-immunological mechanism has been reported. However, all of these reports have shown partial remission. In the present study, we report a case of nephrotic syndrome in a 1-year-old patient who was treated with CyA for SRNS. Complete remission was confirmed for 6 months, and the patient is currently in partial remission. Following confirmation of complete remission, hereditary nephrotic syndrome was not actively investigated; however, delayed secondary sexual characteristics' development led to the diagnosis of Frasier syndrome at the age of 13 years. Thus, immunosuppressive agents may have some efficacy in treating Frasier syndrome, particularly given that our patient achieved complete remission, indicating that it is worth considering administration of immunosuppressive agents for Frasier syndrome management.

Indexed as

CyclosporineImmunosuppressive AgentsNephrotic SyndromeAdolescentDrug ResistanceHumansInfantMaleRemission InductionTreatment OutcomeCyclosporineImmunosuppressive AgentsCyclosporin AFrasier syndromeHereditary nephrotic syndromeWT1 gene abnormality

Identifiers

PMID40764872
PMCPMC12605836

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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 Socratic graph.