提高化疗强度对伴IKZF1基因缺失儿童急性淋巴细胞白血病预后的影响

目的 总结伴IKZF1基因缺失儿童急性淋巴细胞白血病（ALL）的临床特征并观察提高化疗强度对其预后的影响。方法 2015年12月至2018年2月间确诊并按照中国儿童白血病协作组-ALL 2008（CCLG-ALL 2008）方案规范治疗的ALL患儿共278例,根据有无IKZF1基因缺失将其分为IKZF1基因缺失组和IKZF1基因正常组,IKZF1基因缺失组均接受CCLG-ALL 2008高危（HR）方案治疗,IKZF1基因正常组则按临床危险度分型接受不同强度化疗,比较两组的临床特征及无事件生存（EFS）率。结果 278例患儿中共24例（8.6%）检出IKZF1基因外显子大片段缺失。IKZF1基因缺失组初诊时WBC ≥ 50×10⁹/L、BCR-ABL1融合基因阳性、诱导缓解治疗第15天微小残留病≥ 10%、微小残留病-HR、临床危险度-HR所占比例均高于IKZF1基因正常组（P < 0.05）。IKZF1基因缺失组3年EFS率（76%±10%）低于IKZF1基因正常组（84%±4%）,但差异无统计学意义（P=0.282）;其中,IKZF1基因缺失组-非HR（实际按CCLG-ALL 2008 HR方案化疗）的预计3年EFS率为82%±12%,低于IKZF1基因正常组-非HR（86%±5%）,但差异无统计学意义（P=0.436）。结论 伴IKZF1基因缺失的儿童ALL早期治疗反应更差,提高化疗强度可能改善其预后。     

双眼自发性晶状体悬韧带松弛致继发闭角型青光眼一例

双眼自发晶状体悬韧带松弛,导致前房波动,进行性近视加重,继发闭角型青光眼的病例在临床比较少见。本病例报道了1例25岁女性双眼自发晶状体悬韧带松弛不伴有晶状体脱位及形态异常,出现双眼前房波动,近视加重,最终继发双眼闭角型青光眼。经过局部抗炎、散瞳处理和"PHACO IOL(Phacoemulsification intraocular lens)植入 前段玻璃体切除术"将IOL置于睫状沟后,患者眼压得到控制、视力基本矫正至正常。眼科学报2007;23:243-246.     

Retinal tear and raised intraocular pressure following unintentional intraocular botulinum toxin type A injection

CASE REPORT: A 67-year-old woman with a 30-year history of dysthyroid orbitopathy and diplopia had an inadvertent injection of botulinum toxin type A into the vitreous cavity. Full pupil mydriasis and an intraocular pressure (IOP) of 50 mm Hg in the right eye were noted after the injection. An attack of acute angle closure was initially suspected despite a patent peripheral iridotomy (PI). The episode resolved with topical and intravenous IOP-lowering agents. Postoperative retinal examination revealed a retinal tear, which was successfully treated with laser photocoagulation. Visual acuity returned to baseline within 2 weeks, and the results of further retinal examinations remained stable. COMMENTS: This case supports the animal studies and human case reports that have demonstrated no retinal toxicity after intraocular injections of botulinum toxin type A. Temporary mydriasis and elevated IOP that may occur after injection do not appear to be detrimental and can be treated with topical and intravenous IOP-lowering agents. Transiently elevated IOP after injection is most likely due to volume increase, though acute angle closure with occlusion of the PI might be considered.     

Long-term Therapeutic Outcome of Acute Primary Angle Closure in Japanese

Purpose

To investigate the long-term clinical course of acute primary angle closure (APAC) and acute primary angle-closure glaucoma (APACG) in Japanese patients.

Methods

We retrospectively reviewed our records of 66 consecutive APAC or APACG eyes observed between February 1992 and December 2003 (mean follow-up, 42.1 months). Immediately after the diagnosis, all patients had received similar medications to halt the acute attack. Subsequently, laser iridotomy or surgical peripheral iridectomy and/or laser iridoplasty were conducted. If intraocular pressure (IOP) control was poor under maximum tolerable ocular hypotensive agents, trabeculectomy with adjunctive mitomycin C (MMC) was undertaken.

Results

After laser therapy, the probability of success, defined as an IOP of <21?mmHg with or without medications, was 81.2% ± 6.2%. In the ten eyes that were trabeculectomized, the probability of success based on the same criterion was 40.0% ± 29.7%. Multivariate analysis revealed that the degree of synechial angle closure (P = 0.029) and the preexistence of chronic glaucomatous optic neuropathy (P = 0.015) significantly influenced the need for subsequent filtering surgery.

Conclusions

Without the intervention of filtering surgery, 84.6% of eyes with APAC or APACG maintained IOP control with or without antiglaucoma medications. However, APAC and APACG eyes that eventually received trabeculectomy were predisposed to an uncontrollable IOP, even with the intraoperative application of MMC. The severity of APAC or APACG in Japanese may be affected by an underlying creeping angle closure.?Jpn J Ophthalmol 2007;51:353–359 © Japanese Ophthalmological Society 2007

     

白内障手术后急性高眼压快速处理法

目的:探讨白内障手术后急性高眼压的快速处理。方法:对于手术前无青光眼史、眼压正常的白内障患者,手术后眼压增高者,应用表面麻醉,开睑,1mL消毒注射器压迫辅助切口后唇,房水自然流出,眼压随即下降。结果:共处理白内障手术后高眼压67例,放水1次眼压恢复正常者65例,两次放水眼压恢复正常者1例,4次1例。其中6例放水后前房出血,经半卧位休息及全身应用止血药后3～5d出血吸收。无其它并发症。结论:应用辅助切口放出房水治疗白内障手术后高眼压,无应用高渗脱水剂引起肾功能障碍的风险,治疗后眼压随即下降,角膜恢复透明,高眼压临床症状立即消失,减少了高眼压对手术眼视力的损害,且无特殊并发症,经济实用,可以在临床推广应用。     

急性高眼压后大鼠视网膜谷氨酰胺合成酶mRNA表达的变化

目的观察急性高眼压后大鼠视网膜谷氨酰胺合成酶（GS）mRNA的表达变化。方法前房灌注加压至110mmHg，维持60min。提取视网膜总RNA，以RT—PCR半定量GSmRNA。结果大鼠前房加压解除后4h，GSmRNA的表达较对照眼开始升高（P〈0．05），至24h达高峰（P〈0．01），36h降低（P〈0．05），72h（P〉0．05）恢复至正常水平。结论急性高眼压后大鼠视网膜GSmRNA的表达呈现明显升高过程，可能引起GS表达上调，促进谷氨酸转化为谷氨酰胺，从而减轻视网膜神经节细胞（RGCs）的损伤。     

急性视网膜坏死综合征的临床观察

目的:探讨急性视网膜坏死综合征(acute retinal necrosis ARN)临床诊断、治疗的有效方法.方法:回顾性分析我院2003-09/2006-07以来8例11眼ARN患者的临床表现、检查、诊治及预后.结果:8例患者中除1例1眼无眼前段表现外,其余7例(10眼)都表现为不同程度的眼前段炎症、玻璃体炎、坏死性视网膜炎和闭塞性视网膜血管炎.8例中1例(1眼)HSV-1阳性.治疗后视力提高共5眼(46%),最终视力≤0.1者7眼(64%).随访期内10眼(91%)病变控制.结论:ARN的诊断以临床表现为主.早期足量地抗病毒并联合应用糖皮质激素、预防性视网膜光凝、必要时玻璃体手术是控制病变的关键.     

Acute anterior uveitis in primary care

Acute anterior uveitis is an important ocular disease of considerable interest to therapeutically and non‐therapeutically qualified optometrists. This review examines the role of optometrists in the primary care setting and gives guidelines for appropriate care of patients with anterior uveitis. Diagnosis and differentiation from other forms of anterior segment inflammation are the initial requirement. In parallel, possible medical conditions associated with acute anterior uveitis must be considered, with appropriate referral to medical practitioners. In uncomplicated cases of recurrent acute anterior uveitis, optometrists can initiate topical treatment and monitor resolution of inflammation, while being aware of possible complications of both the disease and its treatment. It is especially important in new attacks of anterior uveitis to liaise with the patient’s general practitioner about medical investigation for underlying disease. Atypical, complicated or severe anterior uveitis should be promptly referred for specialist care.     

SURGICAL OPTIONS IN ACUTE DIVERTICULITIS: VALUE OF SlGMOlD RESECTION IN DEALING WITH THE SEPTIC FOCUS

The outcome of 78 patients hospitalized with acute diverticulitis was analysed to determine the indications for emergency operation and to examine the safety of sigmoid resection. Forty patients improved on medical therapy, but four relapsed soon after discharge. Forty-two (55%) patients had emergency operations for non-resolution of clinical sepsis (25). persistent symptoms or mass (14) and fistula (three). Operations performed were: sigmoid resection and primary anastomosis (23) with covering colostomy (six), Hartmann's operation (10). drainage with/without colostomy (seven) and right hemicolectomy (two). Findings at operation were: abscess (19). phlegmon (14). generalized peritonitis (six), colovesical fistula (three) and chronic diverticulitis (three). Complications included wound infections (six), respiratory complications (five) and one death. Six patients having drainage of the sigmoid inflammation alone as the first operation required subsequent resection, with prolonged and often complicated hospitalization. Minimal morbidity and shorter hospitalization were achieved when sigmoid resection was performed at the initial procedure.     

兔急性肾功能衰竭时胰腺形态与功能及自由基、一氧化氮的变化

目的：探讨家兔急性肾功能衰竭（ARF）时胰腺的变化及机制。 方法： 60只家兔均分为4组。ARF模型1组：皮下注射1%HgCl2（1.3 mL/kg）；ARF模型2组：肌肉注射50%甘油（10 mL/kg）；以等量生理盐水代替HgCl2或甘油分别作为对照1、2组。24 h后，经Aeroset型全自动生化分析仪测定血清BUN、Cre及胰淀粉酶（AMY）活性；以放射免疫法检测血清胰岛素（INS）水平；制作胰腺病理切片，观察其组织等变化；制备10%胰匀浆，检测自由基及一氧化氮（NO）水平的变化。 结果： ARF时胰腺细胞有空泡变性、坏死等改变。ARF模型1、2组血清AMY活性均显著高于相应对照组，血清INS水平显著低于相应对照组（P<0.05，P<0.01）；胰匀浆MDA、NO2-/NO3-含量及NOS活性高于对照组，SOD活性低于对照组（P<0.05，P<0.01）。 结论： 兔急性肾功能衰竭时，胰腺可发生形态及功能变化，其机制可能与自由基损伤及NO升高有关。