Evidence map›Paper›PMID 39264505›Full record

ArticleJournal of clinical immunology2024

Successful Allogeneic Hematopoietic Cell Transplantation for Patients with IL10RA Deficiency in Japan.

Dan Tomomasa, Tasuku Suzuki, Ichiro Takeuchi, Kimitoshi Goto, Shin-Ichiro Hagiwara, Dai Keino, Satoshi Saida, Takashi Ishige, Takahiro Kudo, Katsuhide Eguchi and 10 more

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Article in Journal of clinical immunology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing 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

3 citing papers in PubMed.

  1. Article
  2. Article
  3. 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

20 authors.

Dan TomomasaDepartment of Pediatrics and Developmental Biology, Tokyo Medical and Dental University (TMDU), Tokyo, Japan.
Tasuku SuzukiDepartment of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
Ichiro TakeuchiCenter for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology, National Center for Child Health and Development, Tokyo, Japan.
Kimitoshi GotoDepartment of Hematology/Oncology, Osaka Women's and Children's Hospital, Osaka, Japan.
Shin-Ichiro HagiwaraDepartment of Pediatric Gastroenterology, Nutrition and Endocrinology, Osaka Women's and Children's Hospital, Osaka, Japan.
Dai KeinoDivision of Hematology/Oncology, Kanagawa Children's Medical Center, Yokohama, Japan.
Satoshi SaidaDepartment of Pediatrics, Kyoto University Hospital, Kyoto, Japan.
Takashi IshigeDepartment of Pediatrics, Gunma University Graduate School of Medicine, Gunma, Japan.
Takahiro KudoDepartment of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
Katsuhide EguchiDepartment of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Masataka IshimuraDepartment of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Yusuke MatsudaDepartment of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
Taizo WadaDepartment of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
Yoshiya ItoDivision of Clinical Medicine, The Japanese Red Cross Hokkaido College of Nursing, Hokkaido, Japan.
Motohiro KatoChildren's Cancer Center, National Center for Child Health and Development, Tokyo, Japan.
Yoji SasaharaDepartment of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
Tomohiro MorioDepartment of Pediatrics and Developmental Biology, Tokyo Medical and Dental University (TMDU), Tokyo, Japan.
Katsuhiro AraiCenter for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology, National Center for Child Health and Development, Tokyo, Japan.
Holm H UhligTranslational Gastroenterology Unit, Experimental Medicine, John Radcliffe Hospital, University of Oxford, Oxford, OX3 9DU, UK.
Hirokazu KaneganeDeparment of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan. hkanegane.ped@tmd.ac.jp.

Funding

MEXT/JSPS KAKENHI 22K07887
6 · The paper itself

Abstract

backgroundIL10RA (IL10 receptor subunit alpha) deficiency is an autosomal recessive disease that causes inflammatory bowel disease during early infancy. Its clinical course is often fatal and the only curative treatment is allogeneic hematopoietic cell transplantation (HCT). In Japan, only case reports are available, and there are no comprehensive reports of treatment outcomes.

methodsWe retrospectively analyzed patients with IL10RA deficiency in Japan.

resultsTwo newly identified and five previously reported patients were included in this study. Five patients underwent HCT; one untransplanted patient survived to age 14, and one died of influenza encephalopathy before transplantation. All five HCT recipients underwent HCT at the age before 2 years. They all were conditioned with fludarabine/busulfan- or fludarabine /melphalan-based regimens. The donor source was human leukocyte antigen haploidentical donor bone marrow (BM) for two patients and unrelated umbilical cord blood (CB) for two patients. One patient experienced graft failure with unrelated CB and required a second transplant with unrelated BM. All patients who underwent HCT survived and demonstrated an improved performance status.

conclusionIn cases of IL10RA deficiency, the need for transplantation should be promptly assessed, and early transplantation should be considered. (190/250).

Indexed as

Hematopoietic Stem Cell TransplantationTransplantation, HomologousAdolescentChildChild, PreschoolFemaleHumansInfantInflammatory Bowel DiseasesInterleukin-10 Receptor alpha SubunitJapanMaleRetrospective StudiesTransplantation ConditioningTreatment OutcomeInterleukin-10 Receptor alpha SubunitBusulfanFludarabineHematopoietic cell TransplantationIL10RA DeficiencyInborn Errors of ImmunityJapanMelphalan

Identifiers

PMID39264505

What OpenQuestion holds

Textmetadata
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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.