Evidence map›Paper›PMID 38783919›Full record

ArticleFrontiers in pediatrics2024

Clinical analysis and quality of life survey of hemophilia B patients in the central and western regions of China.

Wen Wang, Li Xu, Jingsheng Wu, Weiyong Liu, Jiao Jin, Jing Huang, Zhongjin Xu, Yali Huang, Bai Li, Yufeng Liu and 4 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Frontiers in pediatrics, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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

0 citing papers in PubMed.

No citing paper in PubMed yet.

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

14 authors.

Wen WangDepartment of Pediatrics, Huazhong University of Science and Technology Tongji Medical College Tongji Hospital, Wuhan, China.
Li XuDepartment of Hematology, The Second People's Hospital of Anhui Province, Hefei, China.
Jingsheng WuDepartment of Hematology, The First Affiliated Hospital of University of Science and Technology of China, Hefei, China.
Weiyong LiuDepartment of Ultrasound, The First Affiliated Hospital of University of Science and Technology of China, Hefei, China.
Jiao JinDepartment of Pediatrics, The Affiliated Hospital of Guizhou Medical University, Guiyang, China.
Jing HuangDepartment of Pediatrics, The Affiliated Hospital of Guizhou Medical University, Guiyang, China.
Zhongjin XuDepartment of Hematology, Jiangxi Provincial Children's Hospital, Nanchang, China.
Yali HuangDepartment of Hematology, Jiangxi Provincial Children's Hospital, Nanchang, China.
Bai LiDepartment of Pediatrics, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Yufeng LiuDepartment of Pediatrics, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Qing ZhangHaemophilia Diagnosis and Treatment Center, Department of Hematology and Oncology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China.
Min ZhouHaemophilia Diagnosis and Treatment Center, Department of Hematology and Oncology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China.
Jie PengDepartment of Hematology, Xiangya Hospital Central South University, Changsha, China.
Qun HuDepartment of Pediatrics, Huazhong University of Science and Technology Tongji Medical College Tongji Hospital, Wuhan, China.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Objective: To study the current status of hemophilia B (HB) patients in the central and western regions of China. Methods: This cross-sectional, multicenter study was conducted in seven provinces in the central and western regions of China from April 2019 to June 2023. Samples were collected for the factor IX activity, inhibitor screen, and gene mutation. Furthermore, the status of six index joints and quality of life (QoL) were assessed. Results: A total of 185 HB patients (mild 15, moderate 75, and severe 95) with a median age of 12.17 years were enrolled. 30.3% (56/185) of patients had a family history of HB. 34.6% (64/185) of HB patients had diagnostic delay and 38.5% (69/179) experienced treatment delay. The incidence of inhibitors was 6.1% (11/179). We identified 123 genetic variants in this study, with missense mutations being the most common. 84.0% (89/106) of HB mothers were genetically identified as carriers, with 27.7% (13/47) of carriers having clotting factor levels less than 0.40 IU/ml. 71.4% (132/185) of HB patients had a history of joint hemorrhage, with a rate of target joint in these patients was 64.4% (85/132). Lower extremity joints were most often affected in patients. The Hemophilia Joint Health Score (HJHS) score was significantly positively correlated with the Hemophilia Early Arthropathy Detection with Ultrasound in China (HEAD-US-C) ( Conclusions: One-third of HB patients had delay in diagnosis and treatment, and the incidence of inhibitors was 6.1%. Target joints were present in nearly half of HB patients. Missense was the main mutation type. 84.0% of mothers of HB patients in this study were found to be carriers. HEAD-US-C and HJHS can complement each other in the evaluation of joint status and give a valid basis for early clinical management. Early detection and preventive treatment, as well as reducing high-intensity replacement therapy and inhibitor generation, can effectively improve the QoL of patients.

Indexed as

carriersgene mutationhemophilia Bjoint statusquality of life

Identifiers

PMID38783919
PMCPMC11111977

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.