Department of Endocrinology, Lianshui County People's Hospital, Huai'an 223400, Jiangsu Province, China.
Department of Ophthalmology, The Affiliated Huai'an Hospital of Xuzhou Medical University, Huai'an Second People's Hospital, Huai'an 223001, Jiangsu Province, China.
J Healthc Eng. 2022 Feb 16;2022:7414165. doi: 10.1155/2022/7414165. eCollection 2022.
In this paper, we have carefully investigated the clinical phenotype and genotype of patients with Johanson-Blizzard syndrome (JBS) with diabetes mellitus as the main manifestation. Retinal vessel segmentation is an important tool for the detection of many eye diseases and plays an important role in the automated screening system for retinal diseases. A segmentation algorithm based on a multiscale attentional resolution network is proposed to address the problem of insufficient segmentation of small vessels and pathological missegmentation in existing methods. The network is based on the encoder-decoder architecture, and the attention residual block is introduced in the submodule to enhance the feature propagation ability and reduce the impact of uneven illumination and low contrast on the model. The jump connection is added between the encoder and decoder, and the traditional pooling layer is removed to retain sufficient vascular detail information. Two multiscale feature fusion methods, parallel multibranch structure, and spatial pyramid pooling are used to achieve feature extraction under different sensory fields. We collected the clinical data, laboratory tests, and imaging examinations of JBS patients, extracted the genomic DNA of relevant family members, and validated them by whole-exome sequencing and Sanger sequencing. The patient had diabetes mellitus as the main manifestation, with widened eye spacing, low flat nasal root, hypoplastic nasal wing, and low hairline deformities. Genetic testing confirmed the presence of a c.4463 T > C (p.Ile1488Thr) pure missense mutation in the UBR1 gene, which was a novel mutation locus, and pathogenicity analysis indicated that the locus was pathogenic. This patient carries a new UBR1 gene c.4463 T > C pure mutation, which improves the clinical understanding of the clinical phenotypic spectrum of JBS and broadens the genetic spectrum of the UBR1 gene. The experimental results showed that the method achieved 83.26% and 82.56% F1 values on CHASEDB1 and STARE standard sets, respectively, and 83.51% and 81.20% sensitivity, respectively, and its performance was better than the current mainstream methods.
本文详细研究了以糖尿病为主要表现的 Johanson-Blizzard 综合征(JBS)患者的临床表型和基因型。视网膜血管分割是检测许多眼部疾病的重要工具,在视网膜疾病的自动筛查系统中起着重要作用。针对现有方法中小血管分割不充分和病理性误分割的问题,提出了一种基于多尺度注意力分辨率网络的分割算法。该网络基于编解码器架构,在子模块中引入注意力残差块,增强特征传播能力,减少不均匀光照和低对比度对模型的影响。在编码器和解码器之间添加跳跃连接,并去除传统的池化层,以保留足够的血管细节信息。使用两种多尺度特征融合方法,并行多分支结构和空间金字塔池化,在不同感受野下实现特征提取。我们收集了 JBS 患者的临床资料、实验室检查和影像学检查,提取相关家族成员的基因组 DNA,并通过全外显子组测序和 Sanger 测序进行验证。患者以糖尿病为主要表现,伴有眼距增宽、扁平鼻梁、鼻翼发育不良、发际线低等畸形。基因检测证实 UBR1 基因存在 c.4463 T > C(p.Ile1488Thr)纯错义突变,为新的突变位点,致病性分析表明该位点为致病性。该患者携带新的 UBR1 基因 c.4463 T > C 纯突变,提高了对 JBS 临床表型谱的认识,拓宽了 UBR1 基因的遗传谱。实验结果表明,该方法在 CHASEDB1 和 STARE 标准集上分别达到了 83.26%和 82.56%的 F1 值,分别达到了 83.51%和 81.20%的敏感性,性能优于当前主流方法。