Brachydactyly is a general term that refers to disproportionately short fingers and toes and forms a part of the group of limb malformations characterized by bone dysostosis. Brachydactyly usually occurs either as an ...Brachydactyly is a general term that refers to disproportionately short fingers and toes and forms a part of the group of limb malformations characterized by bone dysostosis. Brachydactyly usually occurs either as an isolated malformation or as a part of a complex malformation syndrome. Brachydactyly is classified as types A, B, C, D, or E;brachymetatarsus IV;Sugarman brachydactyly;or Kirner deformity. Various types of isolated brachydactyly are rare, except for types A3 and D. We describe a 15-year-old girl with isolated brachyphalangism of the basal finger bones of the little finger with symptoms of tenosynovitis. Tenosynovitis might be caused by growth deviation between the flexor digitorum superficialis and the flexor digitorum profundus. The patient responded very well to surgical treatment.展开更多
目的分析不同入路小骨窗开颅显微手术治疗基底节区高血压脑出血的效果。方法选取2019年1月至2022年3月丰城市人民医院收治的88例基底节区高血压脑出血患者作为研究对象,随机分为观察组与对照组,每组44例。两组均行小骨窗开颅显微手术,...目的分析不同入路小骨窗开颅显微手术治疗基底节区高血压脑出血的效果。方法选取2019年1月至2022年3月丰城市人民医院收治的88例基底节区高血压脑出血患者作为研究对象,随机分为观察组与对照组,每组44例。两组均行小骨窗开颅显微手术,对照组采用经颞叶皮质入路手术治疗,观察组采用经侧裂下Rolandic点-岛叶入路手术治疗,比较两组手术情况、血肿清除率、再出血率、术后并发症、术后1个月格拉斯哥昏迷量表(Glasgow coma score,GOS)分级情况及术后1、3、6个月的美国国立卫生研究院卒中量表(National Institute of Health stroke scale,NIHSS)评分及世界卫生组织生存质量测定量表(World Health Organization on quality of life brief scale,WHOQOL-BREF)评分。结果两组手术时间、术中出血量、引流管放置时间、行大骨瓣减压例数、住院时间比较差异均无统计学意义;观察组开始手术至颅内压下降时间长于对照组,差异有统计学意义(P<0.05)。观察组术后24 h血肿清除率明显高于对照组,差异有统计学意义(P<0.05);两组再出血率比较差异无统计学意义。观察组术后1个月预后良好率为81.82%,高于对照组的61.36%,差异有统计学意义(P<0.05)。术后1、3、6个月,观察组NIHSS评分均低于对照组,WHOQOL-BREF评分均高于对照组,差异有统计学意义(P<0.05)。观察组术后并发症发生率为6.82%,低于对照组的25.00%,差异有统计学意义(P<0.05)。结论经侧裂下Rolandic点-岛叶入路小骨窗开颅显微手术治疗基底节区高血压脑出血的效果显著,有助于提升血肿清除率,减少术后并发症发生率,促进术后神经功能的恢复,提高患者预后生存质量。展开更多
Keratocystic odontogenic tumors(KCOTs,previously known as odontogenic keratocysts) are aggressive,noninflammatory jaw lesions with a putative high growth potential and a propensity for recurrence.This article puts tog...Keratocystic odontogenic tumors(KCOTs,previously known as odontogenic keratocysts) are aggressive,noninflammatory jaw lesions with a putative high growth potential and a propensity for recurrence.This article puts together a summary of the serial studies related to KCOTs undertaken by the author’s research group in recent years.Intraosseous jaw cysts with a solely orthokeratinized lining epithe-lium have been suggested to differ from the typical KCOTs.We report 20 cases of such cyst type under the term of ’orthokeratinized odontogenic cyst(OOC)’.Apart from the presence of a keratinizing epithelial lining,the OOC lacks the other histological features of KCOT,exhibits little if any tendency to recur,has no apparent association with NBCCS,may be cured by simple enucleation,and may thus constitute its own clinical entity.Mutations in PTCH1 gene are responsible for NBCCS and are related in tumors associated with this syndrome.We have so far detected 26 PTCH1 mutations(2 mutations occurred twice) in 10 out of 34(29.4%) sporadic and 14 out of 16(87.5%) NBCCS-associated KCOTs.The 26 mutations consisted of 10 frameshift,2 nonsense,3 aberrant splicing,4 in-frame insertion/deletion/ duplication and 7 missense mutations.Two missense mutations in PTCH2 were also detected in 2 out of 15 NBCCS related KCOT patients.By contrast,no pathogenic mutation was detected in SMO.Thus,our data,together with reports from ther groups,indicate that defects of PTCH1 are involved in the pathogenesis of syndromic as well as sporadic KCOTs.The pathogenic role of PTCH2 requires further investigation.A series of in vitro studies on bone resorption of KCOTs and ameloblastomas were undertaken by this group.The results indicate that odontogenic lesions could promote bone resorption in vitro and it is likely to be related to some of the cytokines secreted by the lesions.展开更多
文摘Brachydactyly is a general term that refers to disproportionately short fingers and toes and forms a part of the group of limb malformations characterized by bone dysostosis. Brachydactyly usually occurs either as an isolated malformation or as a part of a complex malformation syndrome. Brachydactyly is classified as types A, B, C, D, or E;brachymetatarsus IV;Sugarman brachydactyly;or Kirner deformity. Various types of isolated brachydactyly are rare, except for types A3 and D. We describe a 15-year-old girl with isolated brachyphalangism of the basal finger bones of the little finger with symptoms of tenosynovitis. Tenosynovitis might be caused by growth deviation between the flexor digitorum superficialis and the flexor digitorum profundus. The patient responded very well to surgical treatment.
文摘目的分析不同入路小骨窗开颅显微手术治疗基底节区高血压脑出血的效果。方法选取2019年1月至2022年3月丰城市人民医院收治的88例基底节区高血压脑出血患者作为研究对象,随机分为观察组与对照组,每组44例。两组均行小骨窗开颅显微手术,对照组采用经颞叶皮质入路手术治疗,观察组采用经侧裂下Rolandic点-岛叶入路手术治疗,比较两组手术情况、血肿清除率、再出血率、术后并发症、术后1个月格拉斯哥昏迷量表(Glasgow coma score,GOS)分级情况及术后1、3、6个月的美国国立卫生研究院卒中量表(National Institute of Health stroke scale,NIHSS)评分及世界卫生组织生存质量测定量表(World Health Organization on quality of life brief scale,WHOQOL-BREF)评分。结果两组手术时间、术中出血量、引流管放置时间、行大骨瓣减压例数、住院时间比较差异均无统计学意义;观察组开始手术至颅内压下降时间长于对照组,差异有统计学意义(P<0.05)。观察组术后24 h血肿清除率明显高于对照组,差异有统计学意义(P<0.05);两组再出血率比较差异无统计学意义。观察组术后1个月预后良好率为81.82%,高于对照组的61.36%,差异有统计学意义(P<0.05)。术后1、3、6个月,观察组NIHSS评分均低于对照组,WHOQOL-BREF评分均高于对照组,差异有统计学意义(P<0.05)。观察组术后并发症发生率为6.82%,低于对照组的25.00%,差异有统计学意义(P<0.05)。结论经侧裂下Rolandic点-岛叶入路小骨窗开颅显微手术治疗基底节区高血压脑出血的效果显著,有助于提升血肿清除率,减少术后并发症发生率,促进术后神经功能的恢复,提高患者预后生存质量。
文摘Keratocystic odontogenic tumors(KCOTs,previously known as odontogenic keratocysts) are aggressive,noninflammatory jaw lesions with a putative high growth potential and a propensity for recurrence.This article puts together a summary of the serial studies related to KCOTs undertaken by the author’s research group in recent years.Intraosseous jaw cysts with a solely orthokeratinized lining epithe-lium have been suggested to differ from the typical KCOTs.We report 20 cases of such cyst type under the term of ’orthokeratinized odontogenic cyst(OOC)’.Apart from the presence of a keratinizing epithelial lining,the OOC lacks the other histological features of KCOT,exhibits little if any tendency to recur,has no apparent association with NBCCS,may be cured by simple enucleation,and may thus constitute its own clinical entity.Mutations in PTCH1 gene are responsible for NBCCS and are related in tumors associated with this syndrome.We have so far detected 26 PTCH1 mutations(2 mutations occurred twice) in 10 out of 34(29.4%) sporadic and 14 out of 16(87.5%) NBCCS-associated KCOTs.The 26 mutations consisted of 10 frameshift,2 nonsense,3 aberrant splicing,4 in-frame insertion/deletion/ duplication and 7 missense mutations.Two missense mutations in PTCH2 were also detected in 2 out of 15 NBCCS related KCOT patients.By contrast,no pathogenic mutation was detected in SMO.Thus,our data,together with reports from ther groups,indicate that defects of PTCH1 are involved in the pathogenesis of syndromic as well as sporadic KCOTs.The pathogenic role of PTCH2 requires further investigation.A series of in vitro studies on bone resorption of KCOTs and ameloblastomas were undertaken by this group.The results indicate that odontogenic lesions could promote bone resorption in vitro and it is likely to be related to some of the cytokines secreted by the lesions.
