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Hatcher posted an update 9 months ago
Inherited optic neuropathy (ION), which can be characterized by isolated optic atrophy or together with systemic manifestations, is a rare optic nerve dysfunction with multiple genetic patterns. Genetic testing plays the key roles in the diagnosis, genetic counseling and treatment of ION. Currently, most ophthalmologists are unfamiliar with how to interpret the genetic testing results correctly. This article describes the genetic characteristics of ION and explains the main points on genetic reports interpretation to help ophthalmologists getting familiar with this relatively rare field and to promote the clinical application of gene testing. (Chin J Ophthalmol, 2021, 57 326-330).Cerebral/central visual impairment (CVI) has a wide range of features owing to central visual pathway disorders. It is currently the most common type of visual impairment in children in developed countries and is increasing in the developing world. The major cause of CVI is hypoxic-ischemic encephalopathy in premature children. CVI occurs from the postgeniculate visual pathway to the occipital lobe and results in severe visual impairment and even blindness. CVI may also lead to visual cognitive disability, walk difficulty, delayed social interaction and autism spectrum. These children are less likely to live independently, and this will impose huge costs on families and society. Puromycin in vivo Therefore, much more care and attention should be paid to CVI. (Chin J Ophthalmol, 2021, 57 321-325).Endovascular aortic arch repair provides treatment opportunity for patients with aortic arch dissection and aneurysm who are intolerant to open surgery. The aortic arch branches provide the blood flow for brain, the revascularization of these branches is part and parcel of the endovascular aortic arch repair. The anatomical configuration, high-speed blood flow and long access from femoral artery increase the difficulty of endovascular aortic arch repair. Debranch technique, combined with chimney, scallop, and fenestration, have partially simplified the endovascular aortic arch repair. The dedicated endografts for aortic arch is becoming a research focus. In the design of aortic arch endografts, fenestrated and branched stent-grafts are the two main strategies. A variety of innovative concepts have been applied in the design of aortic arch endografts, including modular and integrated design, inner branch and outer branch design, single branch and multi-branch design, etc. Today, these procedures of complex endovascular aortic arch repair still need to be limited to experienced centers. Endovascular aortic arch repair showed favorable short-term outcomes through the development of strict surgical plans, as well as effective teamwork. Long-term efficacy and safety in larger participants need further investigation.Hepatectomy represents the first choice of treatment for primary and secondary liver malignancies.However,post-hepatectomy liver failure(PHLF) remains a significant cause of morbidity and mortality after liver resection.Inadequate remnant liver volume and function are the determining factors of PHLF.Therefore,preoperative assessment of hepatic functional reserve is of paramount importance for prevention of PHLF.There are two main conventional assessment systems of preoperative hepatic functional reserve.The first is remnant liver volume(RLV) and its derivative indicators such as residual liver volume ratio,standardized residual liver volume ratio,RLV/body weight,and RLV/body surface area,calculated by imaging-based methods such as CT,that assess whether the volume of residual liver meets the requirements of safe hepatectomy.However,RLV is not an appropriate indicator of functional liver remnant,since the function of liver within equal volume among different persons is unequal due to the underlying liver diseaasing functional liver than traditional methods which just assess the increasing liver volume.The multimodality treatment has significantly increased local control of locally advanced rectal cancer, with a superior oncologic efficacy and reduced local recurrence rate from 35% to less than 10%, and the proportion of patients receiving “watch and wait” strategy or delaying surgery increased as well. However, distant relapse is still the leading cause of cancer-related death without improved long-term survival outcomes. To improve treatment compliance and overall survival benefits, a novel strategy that delivered upfront chemotherapy prior to surgery, which is termed total neoadjuvant therapy (TNT), has been proposed. TNT has two major patterns, including induction and consolidation therapy; the former treatment pattern requires systemic chemotherapy before neoadjuvant chemoradiotherapy, while consolidation therapy refers to additional cycles of chemotherapy between neoadjuvant chemoradiotherapy and surgery. As a radiosensitizer, upfront chemotherapy not only reduces gross tumor volume, but targets occult micro-metastatic disease at an early stage. Several clinical trials have also reported that TNT achieves better local control of disease with a promising treatment compliance. And organ preservation rate is supposed to increase with an improved pathologic or clinical complete response rate. Besides, there existed no established consensus regarding to specific patterns and chemotherapy regimens and doses, which results in remarkable differences among studies. In conclusion, the exact oncologic efficacy and survival benefits of total neoadjuvant therapy still need clinical trials to confirm.Objective To investigate the safety and clinical efficacy of osteotomy after halo pelvic traction in severe scoliosis accompanied with split cord malformation. Methods The clinical data of 14 patients with severe scoliosis accompanied with split cord malformation admitted to the Department of Spinal Surgery, Guizhou Orthopedic Hospital from August 2015 to August 2019 were retrospectively analyzed.There were 6 males and 8 females, aged (19.8±5.0) years (range13 to 34 years). All patients received spinal orthopedic surgery after halo pelvic traction for 3 to 7 weeks.The data of traction time, height, Cobb angle in the main curved coronal plane and sagittal plane, lung function and nutritional status of the patient were collected before and after the treatment. Paired t test was used to compare the evaluation indexes. Results The traction time of the 14 patients was (35.2±8.3)days (range20 to 49 days), and the height of them increased from (156.7±7.6)cm (range141 to 166 cm) before traction to (167.0±6.4)cm (range154 to 177 cm) after traction(t=-10.