Orthotopic liver transplantation in a patient with carbamyl phosphate synthetase deficiency and cystic fibrosis

A 15-year-old female with carbamyl phosphate synthetase deficiency, cystic fibrosis, and cystic fibrosis-related diabetes underwent orthotopic cadaveric liver transplantation. Metabolic control was maintained during the procedure with nutritional support and the use of intravenous sodium phenylacetate and benzoate. Her postoperative course was complicated by seizures and a transient decline in her pulmonary function tests, which returned to preoperative levels within one year of the transplant. Now, four years post-transplant, her quality of life has dramatically improved. There are only four Canadian centres with paediatric liver transplantation programs. However, expert medical care for adults with inborn error of metabolism is even more limited, suggesting that access to adult medical care is one of the many factors to be considered when liver transplantation is contemplated for patients with metabolically unstable conditions.     

Prognostic significance of HIV-associated oral lesions and their relation to therapy

The oral manifestations of HIV infection have been considered to be of value in assessing disease progression in the developed world. However, the potential use of oral lesions as prognostic markers in resource-poor countries has yet to be fully investigated. There is reasonably compelling evidence in the developed world for an association between oral lesions and viral load. However, the true nature of this association is less clear and there are few data available from the developing world. With the introduction of HAART, a change in prevalence of the oral manifestations of HIV infection has been observed, including regression of oral candidiasis, Kaposi's sarcoma and oral hairy leukoplakia. However, oral condylomata and herpes simplex virus infection appear to persist with HMRT therapy. Further research in partnership with resource-poor countries is required to document disease progression and the associated oral lesions in both adults and children.     

Treatment of lipomas assisted with tumescent liposuction

BACKGROUND: Lipoma is a common soft-tissue tumour of mature fat cells. Although surgical excision is effective, treatments that are less invasive and not associated with disfigurement of scar would be ideal for the treatment of lipomas. Recently, tumescent liposuction has been used for the treatment of lipomas. OBJECTIVE: To evaluate the efficacy of tumescent liposuction in lipoma treatment, we reviewed our experience of lipoma treatment by tumescent liposuction. METHODS: A total of 21 patients presenting with 31 lipomas were treated with tumescent liposuction. After liposuction, remaining stromas were removed by a haemostat through the small incision. Tumour size and post-operative complications were recorded before and after treatment. RESULTS: A total of 31 lipomas of 21 patients were treated by tumescent liposuction. The size of lipomas ranged between 1.2 and 11 cm (mean size, 4.1 cm). In 23 cases, there were no complications. However, remnant lipomas, bruise, haematoma and immediate dimpling were found as complications. CONCLUSION: Tumescent liposuctions with extracting remnant fat tissue and fibrous tissue through the opening for liposuction can be an effective treatment technique in lipoma treatment in the efficacy and cosmetic outcomes and this method can be a substitute for excision in treating large lipomas.     

A Review of Autism Spectrum Disorders (ASD) from A Perspective of Classical Chinese Medicine (CCM)

Introduction Autism is a complex developmental disability that typically appears during the first two years of man's life and is the result of a neurological disorder that affects the functioning of the brain,and the     

功能磁共振成像与弥散张量成像联合监测视觉通路异常在高压氧治疗前后的结构及功能变化

目的:联合应用血氧依赖功能磁共振成像(BOLD-fMRI)和弥散张量成像技术评价视觉通路异常高压氧患者在康复过程中脑功能和解剖结构的重组特点。方法:①临床资料:选择2006-01/2007-05解放军南京军区福州总医院收治的因视觉通路病变致单侧或双侧视觉障碍的16例患者为病变组,均接受2个标准大气压高压氧治疗3个疗程。以性别、年龄与病变组大致匹配的正常视力者12例作为正常对照组。②磁共振扫描及分析:两组以相同条件,采用黑白棋盘格变化刺激双眼,应用Signa xcite HD 1.5T双梯度16通道磁共振成像系统进行测试。BOLD-fMRI数据处理采用AFNI软件包进行,弥散张量成像应用日本东京大学影像计算和分析实验室开发的Volume-one 1.64下的dTV.II.R1软件进行数据处理。③观察指标:病变组高压氧干预前后BOLD-fMRI激活体数和弥散张量成像表现,并与正常对照组比较。结果:28例受试者均进入结果分析。①BOLD-fMRI激活体数值:病变组高压氧治疗前视皮质激活体数低于正常对照组(P<0.01),高压氧治疗后视皮质激活体数与正常对照组比较差异不显著(P>0.05),但高于高压氧治疗前(P<0.05)。②弥散张量成像示视放射部分各向异性值:病变组高压氧治疗前部分各向异性值低于正常对照组(P<0.05),高压氧治疗后与正常对照组比较差异不显著(P>0.05),且显著高于高压氧治疗前(P<0.05)。病变组中的6例视神经病变患者视放射显示完整,康复前后的视放射部分各向异性值与正常对照组比较无显著性差异(P>0.05),10例枕叶视中枢病变患者视放射纤维部分中断,视放射各向异性值低于视神经病变患者(P<0.05)。结论:BOLD-fMRI联合弥散张量成像能从功能和结构方面探讨视觉通路病变的发生、发展及康复过程,为脑功能的康复提供较为可靠的治疗依据。