Making artificial bile ducts in vitro for repairing and replacing diseased bile ducts is an important concept in tissue engineering. This study printed a tubular composite scaffold using polycaprolactone (PCL) through the current 3D printing method. It served as a matrix for the organoid cells of the bile duct to proliferation, migration, and differentiation. The PCL scaffold full of bile duct-like organ cells can achieve the effect of bionics, replacing the original bile duct to perform its proper function. In order to enrich the performance of the tubular scaffold, hydrogels were also used in this study. Applying a layer of gelatin methacryloyl (GelMA) hydrogel with an appropriate thickness on the outer layer of the PCL scaffold not only protects and supports the scaffold, but also improves the biocompatibility of the printed bile duct. In addition, ultrasmall superparamagnetic iron oxide (USPIO) nanoparticles dispersed in GelMA served as the contrast agent to monitor the repair of the lesion site and the degradation of the bile duct in real time by magnetic resonance imaging (MRI). In this study, a tubular composite scaffold that could reconstruct bile duct function and possess a real-time MRI imaging property was constructed by 3D printing. After 13 days of the co-culture of bone marrow derived stem cells (BMSCs), the survival rate of the BMSCs was greater than 95%, and the coverage of the BMSCs was as high as 90%. At the same time, the compression modulus of the stent could reach 17.41 kPa and the Young’s modulus could reach 5.03 kPa. Thus, the mechanical properties of it can meet the needs of human implantation. USPIO can achieve MRI imaging in situ and nondestructively monitor the degradation of the stent in the body. In summary, PCL/GelMA/USPIO bile duct scaffolds are beneficial to the proliferation of cells on the scaffolds and can be used to construct biologically active artificial bile ducts.
Fetal ventriculomegaly development leads to neurological, motor, and/or cognitive impairment, and is presently diagnosed based on the width of the atrium in the lateral ventricle. But in this study, we have tried to assess the relationship between the development of calcarine sulcus and width of fetal lateral ventricles, to assess if calcarine sulcus can also be used for fetal ventriculomegaly diagnosis. We conducted a retrospective analysis of the magnetic resonance imaging (MRI) data from 45 subjects with isolated mild fetal ventriculomegaly (IMVM). The calcarine sulcus development was divided into three categories based on the depth; Grade 1 (undeveloped), Grade 2 (underdeveloped), and Grade 3 (fully developed), and its correlation with fetal ventriculomegaly was analyzed based on Spearman's partial rank correlation test. Based on this analysis, the width of left and right lateral ventricles showed significant downward trend with the calcarine sulcus maturation [undeveloped (Left 13.88 ± 2.70 mm, Right 14.27 ± 3.13 mm) → underdeveloped (Left 12.95 ± 1.93 mm, Right 11.93 ± 2.24 mm) → fully developed (Left 11.06 ± 2.10 mm, Right 10.42 ± 2.10 mm)] (F = 5.12, P = 0.01; F = 10.72, P = 1.73 × 10 ). In addition, significant correlations were also observed between the width of the lateral ventricles and the maturity of the calcarine sulcus (Spearman's rank correlation coefficient; -0.47 for the left lateral ventricles and -0.56 for the right, both P < 0.001). Overall, our data indicated a negative correlation between the fetal morphological development of calcarine sulcus and the width of lateral ventricles in subjects having isolated fetal ventriculomegaly.
Objective
This study aimed to summarize the magnetic resonance imaging (MRI) and computed tomography (CT) features of the central nervous system (CNS) in children with Hoyeraal‐Hreidarsson syndrome.
Methods
The imaging and clinical data of four children diagnosed with Hoyeraal‐Hreidarsson syndrome by clinical and laboratory tests in the Guangzhou Women and Children's Medical Center were gathered and analyzed retrospectively. The clinical manifestations and CNS imaging features of Hoyeraal‐Hreidarsson syndrome were summarized based on our results and a literature review.
Results
Our results showed that delayed development, skin pigmentation, nail/toenail dystrophy, thrombocytopenia, and anemia are the most observed clinical presentations of Hoyeraal‐Hreidarsson syndrome. Important findings on CNS imaging showed that all patients had cerebellar hypoplasia, delayed myelination, hydrocephalus, brain atrophy, and calcification. The gene mutations in all cases were consistent with those of dyskeratosis congenita, including TINF2 mutations in three cases and DKC1 mutations in one case.
Conclusion
Hoyeraal‐Hreidarsson syndrome is a severe variant of dyskeratosis congenita. Both DKC1 and TINF2 mutations can lead to the phenotypes of Hoyeraal‐Hreidarsson syndrome. In our study, CNS imaging revealed that cerebellar hypoplasia has an important diagnostic value for Hoyeraal‐Hreidarsson syndrome while delayed myelination, calcification of the parenchyma, brain atrophy, and hydrocephalus are also important findings on CNS imaging. Combining imaging features with clinical and laboratory indicators can assist the diagnosis of Hoyeraal‐Hreidarsson syndrome.
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