Ecchordosis physaliphora （EP） is a benign congenital hamartoma of the ectopic notochordal remnant, accounting for 0.5-2% of all autopsies, and found in 1.5% of all magnetic resonance imaging （MRI） of the brain. It...Ecchordosis physaliphora （EP） is a benign congenital hamartoma of the ectopic notochordal remnant, accounting for 0.5-2% of all autopsies, and found in 1.5% of all magnetic resonance imaging （MRI） of the brain. It commonly occurs along the midline within the retroclival and the prepontine cistern. EP has a slow growth rate and can vary in size from a few mm to 2 cm. Giant symptomatic EPs are extremely rare, and only four cases have been reported in the literature No cases of multiple EPs or EPs with metastasis have been reported,展开更多
The intervertebral disc(IVD)comprises a gelatinous inner core(nucleus pulposus;NP)and concentric rings(annulus fibrosus;AF).The NP,an important structure for shock absorption in the vertebrate spinal motion segment,ca...The intervertebral disc(IVD)comprises a gelatinous inner core(nucleus pulposus;NP)and concentric rings(annulus fibrosus;AF).The NP,an important structure for shock absorption in the vertebrate spinal motion segment,can be traced back to the notochord in ontogenetic lineage.In vertebrates,the notochord undergoes mucinoid changes,and had been considered vestigial until recently.However,observed correlations between IVD degeneration and back pain in humans have renewed interest in the IVD in biomedical fields.Beyond its mechanical contribution to development,the notochord is also an essential signaling center,which coordinates formation of the neural tube and somites.The pertinent signaling molecules,particularly TGF-b and bone morphogenetic proteins(BMPs),continue to play roles in the adult tissues and have been utilized for tissue regeneration.Genetic factors are major determinants of who will develop IVD degeneration and related back pain,and seem to correlate better with disc degeneration and back pain than do external forces on the spine.In summary,the spinal column is a landmark development in evolution.Genes directing the development of the IVD may also contribute to its maintenance,degeneration,and regeneration.Likewise,structural genes as well as genes responsible for maintenance of the structure are related to IVD degeneration.Finally,genes responsible for inflammation may play a dual role in exacerbating degeneration or facilitating repair responses depending on the context.展开更多
Aim: To describe the clinical, anatomical, therapeutic aspects and propose a diagram of a new type of a complete urethral duplicity in classification of Effman. Material and Methods: This was a case report of a 14-yea...Aim: To describe the clinical, anatomical, therapeutic aspects and propose a diagram of a new type of a complete urethral duplicity in classification of Effman. Material and Methods: This was a case report of a 14-year-old patient who suffered from birth of incontinence occurring while sitting with concept of preservation of normal urination. The patient was carrying two urethral meatus, one continent and the other incontinent. The two urethral meatus were easy to catheterize. The imagery was non-contributory. Resection of accessory urethra helped to make the patient continent. Informed consent was obtained from the patient’s parents for the surgical management, the anonymous use of record and photographs for scientific aims. Results: The diagnosis of urethral duplicity was retained. An explanatory approach is reported, a new scheme is proposed to improve the classification of Effman. Conclusion: The urethral duplicity is a rare urogenital abnormality whose etiology and pathophysiology are not fully understood and has not finished delivering the secret of his classification.展开更多
Due to the rare occurrence of chordomas extra-axially, these lesions have not earned a great deal of consideration in the clinical and radiographic differential diagnoses of extra-axial paraspinal lesions. We describe...Due to the rare occurrence of chordomas extra-axially, these lesions have not earned a great deal of consideration in the clinical and radiographic differential diagnoses of extra-axial paraspinal lesions. We describe a case of a patient with a surgically resected extra-axial chordoma and review the radiologic characteristics of chordomas as an entity in the spectrum of extra-axial lesions. A 47-year-old man presented to our institution with four months of intermittent parethesias, pain, and subjective weakness in the left leg. MR imaging of the lumbar spine demonstrated a large heterogeneously- enhancing paraspinal lesion extending from the left L3-4 neural foramen into the psoas muscle. The patient underwent a CT-guided needle biopsy demonstrating features consistent with a chordoma. He then underwent a left retroperitoneal approach and en bloc resection of the lesion from the vertebral column. Pathology confirmed chordoma as the diagnosis. EACs are a rare but important consideration in the diagnosis of extra-axial lesions of the central nervous system.展开更多
The nasopharynx is an unusual site for extraosseous chordoma. The characteristic histology and immunohistochemistry confirmed the diagnosis. The behaviour of this tumour is locally aggressive. We report a case of naso...The nasopharynx is an unusual site for extraosseous chordoma. The characteristic histology and immunohistochemistry confirmed the diagnosis. The behaviour of this tumour is locally aggressive. We report a case of nasopharyngeal chordoma in an elderly patient with literature review.展开更多
Xenopus tropicalis embryos were exposed for 48 hr to the mixtures of 5 μg Sn/L triphenyltin （TPT）, which is a well-known endocrine disruptor, and 0.25-5 μg/L 9-cis retinoic acid （9c-RA）, which is the natural lig...Xenopus tropicalis embryos were exposed for 48 hr to the mixtures of 5 μg Sn/L triphenyltin （TPT）, which is a well-known endocrine disruptor, and 0.25-5 μg/L 9-cis retinoic acid （9c-RA）, which is the natural ligand of retinoid X receptor. The phenotypes induced by combined exposure were more variable than those resulting from single exposure to either TPT or 9c-RA. The prominent phenotypes included underdeveloped head structures, abnormal eyes, narrow fins, enlarged proctodaeum, etc. Especially, combined exposure induced unexpected notochord malformations, which ranged from small swellings of the surface of the tails to the extension and extrusion of notochord out of the posterior tails. Compared with the 5 μg Sn/L TPT-treated group, the index of fin deficiency was not affected, and the index of axis deficiency was significantly increased with increasing RA concentrations in the mixtures. Our results suggest that combined exposure to TPT and 9c-RA induced not only more variable phenotypes of malformations than exposure to single compound but also some new and unexpected phenotypes.展开更多
Klippel-Feil syndrome(KFS) is defined by congenital cervical vertebral spine fusion and is seen with a wide spectrum of dental manifestations and craniofacial profiles. Previous studies on lateral cephalograms have do...Klippel-Feil syndrome(KFS) is defined by congenital cervical vertebral spine fusion and is seen with a wide spectrum of dental manifestations and craniofacial profiles. Previous studies on lateral cephalograms have documented an association between fusion of the cervicalvertebrae and deviations in the craniofacial profile in non-syndromic patients with severe malocclusion. To our knowledge, no previous studies have described the craniofacial profile including the cranial base of KFS patients on lateral cephalograms. Therefore KFS and its craniofacial and dental manifestations were described according to existing literature and additionally the craniofacial profile and cranial base was analysed on lateral cephalograms of two patients with KFS. According to the literature the dental manifestations of KFS-patients included oligodontia, overjet, cross bite, open bite and deep bite. The craniofacial profile was clinically described as reduced lower facial height, midfacial hypoplasia, and mandibular prognathia. The analyses of the two lateral cephalograms showed increased mandibular inclination, increased vertical jaw-relationship, increased jaw angle and maxillary retrognathia. The cranial base was normal in both cases. The sagittal jaw relationship and mandibular prognathia varied between the two cases. The literature review and the analyses of the two lateral cephalograms have shown that deviations in the occipital and cervical spine field as KFS were associated with deviations in the teeth and craniofacial profile.展开更多
A recent study published in Science Advances1 showed the influence of Yap on notochord formation and NT(neural tube)patterning in vertebrate embryonic development,and conducted an in-depth study from the perspective o...A recent study published in Science Advances1 showed the influence of Yap on notochord formation and NT(neural tube)patterning in vertebrate embryonic development,and conducted an in-depth study from the perspective of biomechanical signal mechanotransduction.In addition,this study also explored the possible complex interaction between mechanical signals and gene expression.Together,this study provides new insights into the development mechanism of early vertebrate embryos.展开更多
Intercellular communication of notochord cells during their differentiation was studied by microinjection of a fluorescent dye, Lucifer Yellow. Close correlation existed between the incidences of dye coupling and quan...Intercellular communication of notochord cells during their differentiation was studied by microinjection of a fluorescent dye, Lucifer Yellow. Close correlation existed between the incidences of dye coupling and quantitative evaluation of gap junctions. High incidences of dye coupling and of gap junctions occurred at a stage when notochord cells were active in the change of cell shape and cell arrangement. With the subsidence of cell movements, both dye coupling and gap junctions were reduced to lower levels. It was, therefore, suggested that intercellular communication via gap junctions played an important role in the coordination of notochord cell movements.Gap junctions of altered configuration occurred in notochord cells in late tailbud stage. The comparison of incidences of dye coupling at this stage with those at other stages strongly suggested that the gap junctions of altered configuration functioned just as those of generalized type.展开更多
Transcription factor Brachyury,a protein containing 435 amino acids,has been widely investigated and reported in notochord differentiation and nucleus pulposus development.The crucial functions and underlying mechanis...Transcription factor Brachyury,a protein containing 435 amino acids,has been widely investigated and reported in notochord differentiation and nucleus pulposus development.The crucial functions and underlying mechanisms by Brachyury are discussed in this paper,which suggests Brachyury can be developed into a potential novel target for the therapy of intervertebral disc degeneration.展开更多
文摘Ecchordosis physaliphora （EP） is a benign congenital hamartoma of the ectopic notochordal remnant, accounting for 0.5-2% of all autopsies, and found in 1.5% of all magnetic resonance imaging （MRI） of the brain. It commonly occurs along the midline within the retroclival and the prepontine cistern. EP has a slow growth rate and can vary in size from a few mm to 2 cm. Giant symptomatic EPs are extremely rare, and only four cases have been reported in the literature No cases of multiple EPs or EPs with metastasis have been reported,
基金Jason W.Ashley,PhD is supported by the University of Pennsylvania Postdoctoral Opportunities in Research and Teaching(PENN-PORT)fellowship funded by the National Institute of General Medical Sciences Institutional Research and Career Development Award(IRACDA5 K12 GM081259-09)+2 种基金Yejia Zhang,MD,PhD has been supported by the Eunice Kennedy Shriver National Institute of Child Health and Human Development(NICHD,1K08 HD049598)This work is supported,in part,by research grants from the Department of Veterans Affairs(VA RR&D I01 RX001321 and VA1I21RX001896)the Research Grants Committee of Hong Kong(T-12-708/12N).
文摘The intervertebral disc(IVD)comprises a gelatinous inner core(nucleus pulposus;NP)and concentric rings(annulus fibrosus;AF).The NP,an important structure for shock absorption in the vertebrate spinal motion segment,can be traced back to the notochord in ontogenetic lineage.In vertebrates,the notochord undergoes mucinoid changes,and had been considered vestigial until recently.However,observed correlations between IVD degeneration and back pain in humans have renewed interest in the IVD in biomedical fields.Beyond its mechanical contribution to development,the notochord is also an essential signaling center,which coordinates formation of the neural tube and somites.The pertinent signaling molecules,particularly TGF-b and bone morphogenetic proteins(BMPs),continue to play roles in the adult tissues and have been utilized for tissue regeneration.Genetic factors are major determinants of who will develop IVD degeneration and related back pain,and seem to correlate better with disc degeneration and back pain than do external forces on the spine.In summary,the spinal column is a landmark development in evolution.Genes directing the development of the IVD may also contribute to its maintenance,degeneration,and regeneration.Likewise,structural genes as well as genes responsible for maintenance of the structure are related to IVD degeneration.Finally,genes responsible for inflammation may play a dual role in exacerbating degeneration or facilitating repair responses depending on the context.
文摘Aim: To describe the clinical, anatomical, therapeutic aspects and propose a diagram of a new type of a complete urethral duplicity in classification of Effman. Material and Methods: This was a case report of a 14-year-old patient who suffered from birth of incontinence occurring while sitting with concept of preservation of normal urination. The patient was carrying two urethral meatus, one continent and the other incontinent. The two urethral meatus were easy to catheterize. The imagery was non-contributory. Resection of accessory urethra helped to make the patient continent. Informed consent was obtained from the patient’s parents for the surgical management, the anonymous use of record and photographs for scientific aims. Results: The diagnosis of urethral duplicity was retained. An explanatory approach is reported, a new scheme is proposed to improve the classification of Effman. Conclusion: The urethral duplicity is a rare urogenital abnormality whose etiology and pathophysiology are not fully understood and has not finished delivering the secret of his classification.
文摘Due to the rare occurrence of chordomas extra-axially, these lesions have not earned a great deal of consideration in the clinical and radiographic differential diagnoses of extra-axial paraspinal lesions. We describe a case of a patient with a surgically resected extra-axial chordoma and review the radiologic characteristics of chordomas as an entity in the spectrum of extra-axial lesions. A 47-year-old man presented to our institution with four months of intermittent parethesias, pain, and subjective weakness in the left leg. MR imaging of the lumbar spine demonstrated a large heterogeneously- enhancing paraspinal lesion extending from the left L3-4 neural foramen into the psoas muscle. The patient underwent a CT-guided needle biopsy demonstrating features consistent with a chordoma. He then underwent a left retroperitoneal approach and en bloc resection of the lesion from the vertebral column. Pathology confirmed chordoma as the diagnosis. EACs are a rare but important consideration in the diagnosis of extra-axial lesions of the central nervous system.
文摘The nasopharynx is an unusual site for extraosseous chordoma. The characteristic histology and immunohistochemistry confirmed the diagnosis. The behaviour of this tumour is locally aggressive. We report a case of nasopharyngeal chordoma in an elderly patient with literature review.
基金supported by the National Natural Science Foundation of China(No.21277049)the State Key Laboratory of Estuarine and Coastal Research(No.2012KYYW04)
文摘Xenopus tropicalis embryos were exposed for 48 hr to the mixtures of 5 μg Sn/L triphenyltin （TPT）, which is a well-known endocrine disruptor, and 0.25-5 μg/L 9-cis retinoic acid （9c-RA）, which is the natural ligand of retinoid X receptor. The phenotypes induced by combined exposure were more variable than those resulting from single exposure to either TPT or 9c-RA. The prominent phenotypes included underdeveloped head structures, abnormal eyes, narrow fins, enlarged proctodaeum, etc. Especially, combined exposure induced unexpected notochord malformations, which ranged from small swellings of the surface of the tails to the extension and extrusion of notochord out of the posterior tails. Compared with the 5 μg Sn/L TPT-treated group, the index of fin deficiency was not affected, and the index of axis deficiency was significantly increased with increasing RA concentrations in the mixtures. Our results suggest that combined exposure to TPT and 9c-RA induced not only more variable phenotypes of malformations than exposure to single compound but also some new and unexpected phenotypes.
文摘Klippel-Feil syndrome(KFS) is defined by congenital cervical vertebral spine fusion and is seen with a wide spectrum of dental manifestations and craniofacial profiles. Previous studies on lateral cephalograms have documented an association between fusion of the cervicalvertebrae and deviations in the craniofacial profile in non-syndromic patients with severe malocclusion. To our knowledge, no previous studies have described the craniofacial profile including the cranial base of KFS patients on lateral cephalograms. Therefore KFS and its craniofacial and dental manifestations were described according to existing literature and additionally the craniofacial profile and cranial base was analysed on lateral cephalograms of two patients with KFS. According to the literature the dental manifestations of KFS-patients included oligodontia, overjet, cross bite, open bite and deep bite. The craniofacial profile was clinically described as reduced lower facial height, midfacial hypoplasia, and mandibular prognathia. The analyses of the two lateral cephalograms showed increased mandibular inclination, increased vertical jaw-relationship, increased jaw angle and maxillary retrognathia. The cranial base was normal in both cases. The sagittal jaw relationship and mandibular prognathia varied between the two cases. The literature review and the analyses of the two lateral cephalograms have shown that deviations in the occipital and cervical spine field as KFS were associated with deviations in the teeth and craniofacial profile.
文摘A recent study published in Science Advances1 showed the influence of Yap on notochord formation and NT(neural tube)patterning in vertebrate embryonic development,and conducted an in-depth study from the perspective of biomechanical signal mechanotransduction.In addition,this study also explored the possible complex interaction between mechanical signals and gene expression.Together,this study provides new insights into the development mechanism of early vertebrate embryos.
文摘Intercellular communication of notochord cells during their differentiation was studied by microinjection of a fluorescent dye, Lucifer Yellow. Close correlation existed between the incidences of dye coupling and quantitative evaluation of gap junctions. High incidences of dye coupling and of gap junctions occurred at a stage when notochord cells were active in the change of cell shape and cell arrangement. With the subsidence of cell movements, both dye coupling and gap junctions were reduced to lower levels. It was, therefore, suggested that intercellular communication via gap junctions played an important role in the coordination of notochord cell movements.Gap junctions of altered configuration occurred in notochord cells in late tailbud stage. The comparison of incidences of dye coupling at this stage with those at other stages strongly suggested that the gap junctions of altered configuration functioned just as those of generalized type.
基金This work was supported by the Gusu Health Talent Project of Suzhou(Grant No.GSWS2020056)“333”Talent Project of Jiangsu Province(Grant No.BRA2017057).
文摘Transcription factor Brachyury,a protein containing 435 amino acids,has been widely investigated and reported in notochord differentiation and nucleus pulposus development.The crucial functions and underlying mechanisms by Brachyury are discussed in this paper,which suggests Brachyury can be developed into a potential novel target for the therapy of intervertebral disc degeneration.
