Introduction: Cataract may cause severe visual impairment in eyes with microphthalmos and uveal coloboma. Despite their similarities, distinguishing between these conditions is crucial for cataract surgery. We aimed to compare complications, nucleus hardness, surgical strategies, and outcomes of cataract surgery between microphthalmos and uveal coloboma. Methods: This retrospective comparative study included 19 eyes with microphthalmos and 20 with uveal coloboma. Preoperative, intraoperative, and postoperative data of eyes that underwent cataract surgery were analysed. Results: MO eyes had lower high-hardness nucleus rates (P=0.002) than UC. The most common preoperative complications in the microphthalmos and uveal coloboma groups were glaucoma (37.5%) and retinal detachment (15.4%), respectively. No significant differences in the phacoemulsification (P=0.45) or intraocular lens implantation (P>0.99) rates between the two groups. Extracapsular cataract extraction was performed in five eyes (25%), and posterior capsular rupture was the most common surgical complication (15%) in the uveal coloboma group. Combined surgery was mainly used to deal with high or unstable intraocular pressure (77%) in the microphthalmos group versus posterior pars plana vitrectomy (85.71%) in the uveal coloboma group to treat vitreoretinal pathologies. Corrected distance visual acuity was significantly improved in both microphthalmos (P=0.0005) and uveal coloboma (P<0.001) groups, while intraocular pressure was decreased (P=0.03) in the microphthalmos group. Conclusion: Eyes with microphthalmos and uveal coloboma exhibited distinct cataract grades and complications, necessitating varied surgical strategies; while cataract surgery has proven to be effective in improving the visual acuity in both groups. Our findings hold significant value for guiding clinical treatment decision-making.
This study was investigated the surgical outcomes of primary rhegmatogenous retinal detachment (RRD) with inferior retinal breaks (IRBs) that were repaired by 25-gauge pars plana vitrectomy (PPV) with air tamponade.This retrospective review included 81 consecutive patients who had RRD with IRBs and underwent PPV with air tamponade in our hospital from January 2017 to January 2020. The main outcomes were single surgery anatomical success (SSAS) rate, postoperative best-corrected visual acuity (BCVA), and complications.The patient population consisted of 29 women and 52 men (mean age, 52.12 years); the mean follow-up interval was 8.88 months. The mean number of affected quadrants was 1.65 (range, 1-4 quadrants) and the mean number of breaks was 3.25. A single break was present in 20 cases (24.7%); two to 10 breaks were present in 61 (75.3%) cases. The SSAS rate was 91.36% (74/81) and the final anatomical success rate was 96.30% (78/81). More than half of the patients had BCVA < 0.3 logarithm of the minimum angle of resolution at the last follow-up. Axial length and patient age were candidate risk factors for redetachment (axial length, p = 0.03; age, p = 0.002). Postoperative complications included macular epiretinal membrane formation in one patient, lens opacity in three patients, and clinically significant macular edema in one patient.PPV with air tamponade may be effective for the treatment of primary RRD with IRBs. Extensive preoperative discussion may be necessary for young patients and patients with particularly long axial length.
Objective: To observe the effects of intravenous methylprednisolone pulse (IVMP) therapy on the recovery of visual acuity and its influencing factors in patients with the relapse of aquaporin (AQP) 4 antibody positive neuromyelitis optica related optic neuritis (NMO-ON). Methods: Retrospective case series. Forty-eight eyes of 35 patients diagnosed as NMO-ON in the Neuro-ophthalmology Clinic of Beijing Tongren Hospital from September 2012 to April 2018 were included in this research. All patients were AQP4 antibody seropositive, and had clinical manifestations of acute optic neuritis, with a history of optic neuritis treated with glucocorticoids effectively. They received the treatment of IVMP 500 mg/d or 1 000 mg/d for 3 to 5 days. The post-treatment and pre-treatment visual acuities were compared. Improving four lines or more was considered as markedly effective, improving two or three lines as effective, and improving one line or no change or a decline as no effect. The impacts of age, visual acuity at onset, relapse rate and dosage on the acute exacerbation of NMO-ON were analyzed. Mann-Whitney U test and Kruskal-Wallis test were used for statistical analysis. Results: Among the 35 patients, there were 2 males and 33 females, aged from 15 to 73 years (median, 36 years). In the 48 eyes of recurrence, the treatment was effective 41.7% (20/48), effective 20.8% (10/48), and ineffective 37.5% (18/48). The IVMP therapy was effective in 25 of 34 eyes with one recurrence and 5 of 14 eyes with two or more recurrences, and the difference was statistically significant (Z=2.315, P=0.021). The efficacy in 13 eyes with preoperative visual acuity not lower than 0.05 (10/13) was better than 35 eyes with preoperative visual acuity lower than 0.05 (20/35), and the difference was statistically significant (Z=1.994, P=0.046). Different ages and doses (1 000 mg/d and 500 mg/d) made no significant difference in the efficacy (P=0.273,0.105). Conclusions: The IVMP therapy is effective for the NMO-ON relapse in patients who were AQP4 antibody seropositive. The effect of IVMP treatment at doses of 500 mg/d and 1 000 mg/d is similar. Furthermore, visual acuity less than 0.05 and more relapses reduce the efficacy in relapsed NMO-ON patients. (Chin J Ophthalmol, 2020, 56: 509-513).目的： 观察静脉注射甲泼尼龙冲击（IVMP）治疗对水通道蛋白4（AQP4）抗体阳性的复发性视神经脊髓炎相关视神经炎（NMO-ON）患者的视力恢复效果及其影响因素。 方法： 回顾性系列病例研究。纳入2012年9月至2018年4月在首都医科大学附属北京同仁医院神经眼科门诊确诊为复发性NMO-ON的35例（48只眼）发病患者。所有患者AQP4抗体检测均为阳性，具有急性视神经炎的临床表现，既往有视神经炎发病史且经糖皮质激素治疗有效，均给予500 mg/d或1 000 mg/d，共3~5 d的IVMP治疗，糖皮质激素序贯减量。治疗后1~2周视力较治疗前提高4行及以上为显效；提高2~3行为有效；变化在1行内或无变化或下降为无效。分析复发次数、治疗前视力、年龄以及糖皮质激素冲击剂量对复发性NMO-ON疗效的影响。采用Mann-Whitney U及Kruskal-Wallis检验进行统计学分析。 结果： 35例患者中男性2例，女性33例；发病年龄15~73岁，中位数36岁。在复发的48只眼中，治疗显效41.7%（20/48），有效20.8%（10/48），无效37.5%（18/48）。复发1次的患眼IVMP治疗总有效率为73.5%（25/34），与复发≥2次的患眼IVMP治疗总有效率（5/14）比较，差异有统计学意义（Z=2.315，P=0.021）。治疗前视力<0.05与≥0.05的患眼IVMP治疗总有效率分别为57.1%（20/35）、10/13，差异有统计学意义（Z=1.994，P=0.046）。不同年龄段间及IVMP剂量500 mg/d与1 000 mg/d间治疗效果比较，差异均无统计学意义（P=0.273，P=0.105）。 结论： IVMP治疗对复发性AQP4抗体阳性NMO-ON患者的视力恢复有效，采用剂量为500 mg/d与1 000 mg/d的IVMP治疗效果相近，IVMP治疗对视力<0.05及复发次数多的患者效果更差。（中华眼科杂志，2020，56：509-513）.
Objective: To compare the efficacy and safety of 2 low-dose rituximab regimens in the treatment of children with primary immune thrombocytopenia (ITP). Methods: A total of 90 ITP children admitted to the Hematology Oncology Center of Beijing Children's Hospital from January 2018 to March 2021 were enrolled in this prospective cohort study. In the single-dose group, rituximab was given with a single dose of 375 mg/m2 (maximum dose 600 mg). In the 4-dose group, rituximab was given with a dose of 100 mg weekly (if body weight of the patient ≥ 30 kg, increase dosage to 200 mg weekly) for 4 weeks. Wilcoxon Mann-Whitney test, Chi-square test and Fisher's exact test were used to analyze the difference in efficacy, safety and treatment burden between two groups. Results: Among the 90 children, 41 were male and 49 were female, and the age of medication was 6.8 (4.1,10.0) years. There were 27 cases in the single-dose group and 63 cases in the 4-dose group.There were no significant differences in overall response rate, complete response rate and partial response rate between the single-dose group and 4-dose group (41% (11/27) vs. 33% (21/63), 26% (7/27) vs. 19% (12/63), 15% (4/27) vs. 14%(9/63), χ2=0.45, 0.54, 0.00, all P>0.05). The single-dose group was earlier to get overall response than the 4-dose group (1 (1, 1) vs. 3 (2, 6) weeks, Z=-3.24, P=0.001). There were no significant differences in the sustained response rate, the overall response rate in 1 year, the complete response rate in 1 year, and the partial response rate in 1 year between the single-dose group and the 4-dose group (33% (9/27) vs. 30% (19/63), 30% (8/27) vs. 24% (15/63), 19% (5/27) vs. 14% (9/63), 11% (3/27) vs. 10% (6/63), χ2=0.09, 0.34, 0.04, 0.00, all P>0.05). There were no significant differences in the duration of overall response, recurrence rate within half a year and one year, recurrence time and rate of adverse events between the single-dose group and 4-dose group (all P>0.05). The number of hospitalizations, the duration of hospital stays and the dosage of the single-dose group were significantly lower than those of the 4-dose group (1 (1, 1) vs. 4 (4, 4) times, 5 (4, 7) vs. 8 (5, 8) d, 400 (250, 500) vs. 400 (400, 800) mg, Z=-8.67, -3.03, -4.05, all P<0.05). Conclusions: The single-dose rituximab regimen is comparable to 4-dose rituximab regimen in effectiveness and safety for treatment of children ITP, but more economical and convenient. The single-dose rituximab regimen is more suitable for the second-line treatment of children ITP.目的： 比较两种小剂量利妥昔单抗治疗方案治疗儿童原发性免疫性血小板减少症（ITP）的效果及安全性。 方法： 前瞻性队列研究。将2018年1月至2021年3月北京儿童医院血液肿瘤中心收治共90例ITP患儿纳入研究。单剂组利妥昔单抗用量为375 mg/m2（最大剂量600 mg），只用1次；四剂组利妥昔单抗用量为100 mg/（次·周）［体重≥30 kg者200 mg/（次·周）］，连续4周，每周1次。采用秩和检验、χ2检验和Fisher确切概率法比较分析组间疗效、安全性及治疗费用等指标的差异。 结果： 90例患儿中男41例、女49例，用药年龄6.8（4.1，10.0）岁，其中单剂组27例，四剂组63例，两组患儿在总有效率、完全有效率和部分有效率方面差异均无统计学意义［41%（11/27）比33%（21/63）、26%（7/27）比19%（12/63）、15%（4/27）比14%（9/63），χ2=0.45、0.54、0.00，均P>0.05］。单剂组起效时间显著快于四剂组［1（1，1）比3（2，6）周，Z=-3.24，P=0.001］。单剂组比四剂组在持续有效率、1年时总有效率、1年时完全有效率、1年时部分有效率方面差异无统计学意义［33%（9/27）比30%（19/63）、30%（8/27）比24%（15/63）、19%（5/27）比14%（9/63）、11%（3/27）比10%（6/63），χ2=0.09、0.34、0.04、0.00，均P>0.05］。两组在疗效持续时间、半年内复发率、1年内复发率、复发时间、不良反应率方面差异均无统计学意义（均P>0.05）。单剂组住院次数、住院天数和用药量显著低于四剂组［1（1，1）比4（4，4）次、5（4，7）比8（5，8）d、400（250，500）比400（400，800）mg，Z=-8.67、-3.03、-4.05，均P<0.05］。 结论： 两种小剂量利妥昔单抗方案治疗儿童ITP的效果及安全性没有差异，但单剂小剂量利妥昔单抗方案更为经济和方便，更适合用于儿童ITP的二线治疗。.
To report the results of rhegmatogenous retinal detachment (RRD) repair after pars plana vitrectomy (PPV) without operative use of heavy liquid, and utilizing air tamponade in selected cases.RRD patients without severity of proliferative vitreoretinopathy C2 or more underwent PPV without operative use of heavy liquid, and utilizing air tamponade were consecutively enrolled. Alternative postoperative facedown position or lateral position was required for 3-5d.Totally 36 eyes of 36 patients (24 males, 66.7%) aged 53.8±10.9y underwent this modified surgery. The mean number of retinal break was 2.1±1.3. Most of the eyes (29, 80.6%) had retinal detachment involving more than one quadrant. Twenty-two (61.1%) eyes with cataract had combined phacoemulsification and intraocular lens implantation. The mean follow up time was 4.6±1.8mo. Two eyes with retinal redetachment underwent a second retinal repair surgery with silicone oil tamponade, yielding the primary reattachment rate to 94.4% (34/36). Six (16.7%) eyes had intraocular pressure higher than 25 mm Hg. The visual acuity (logMAR) improved from 0.98±0.74 preoperatively to 0.52±0.31 postoperatively (P<0.001).The success rate of this modified retinal repair surgery is comparable with traditional surgery. This technique can be considered for certain retinal detachment patients, since its apparent advantages included lower surgical complications, reduced surgery expenditure, shorter time for postoperative facedown position, and avoiding silicone oil removal surgery.
Background: Personalized prophylaxies for moderate and severe hemophilia A and B are based on personal pharmacokenetics (PK), half-life(T1/2). While classical method for personal PK needs multiple blood collections, at least 5 points collections for children and 11points for adults, and expensive for testing and leads low compliance. Popular PK based on pupolar data can have a less blood collections but a rough data(range) is recommended for reference. One-chamber model may also have a less blood collection 2-3 collections for PK detection. Aims: Objective: To analyze and compare the half-life (T1/2) results of Factor VIII (FVIII) calculated by modified one-chamber model by “CAI’s” hemophilia pharmacokinetic calculation tool and WinNolin software (five-point sampling), non-av model, and to explore the reliability and clinical application value of “CAI’s” hemophilia pharmacokinetic calculation tool. Methods: A total of 30 patients with moderate and severe hemophilia A were treated with FVIII (50IU/Kg) after the 72-hour elution period. Peripheral blood samples were collected at five time points before and after FVIII injection (0h, 1h, 9h, 24h, 48h), and FVIII activity was detected by one-stage method. The T1/2 of FVIII was calculated by WinNolin software. Two of the three time points of FVIII activities (9h~24h, 9h~48h, 24h~48h) were used to calculate the FVIII T1/2 by “CAI’s” hemophilia A pharmacokinetic calculation tool, and the results were compared with those of WinNolin software, and the correlation analysis was conducted. Results: 1. 30 cases of T1/2 of FVIII calculated by WinNolin software was from 5.55 hs to 12.5 hs. 2. Compared with WinNolin software (five-point method, the results of T1/2 of FVIII caculated by “CAI’s” hemophilia A pharmacokinetic calculation tool, (the modified one-chamber model method) at 9h~24h, 9h~48h and 24h~48h showed that the detection periods with highist consistent T1/2 were 9h~48h, 24 out of 30, 80.0%, while 9h~24h 6 out of 30, 20.00%; 3. Correlation analysis shows that there is a good correlation between the T1/2 of the modified one-chamber model and the results of WinNolin’s calculation in all three time periods, but the correlation/consistency is the highest in the period of 9h~48h (see fig 1); 4. Analysis of the FVIII T1/2 results of 9h~24h, 9h~48h and 24h~48h we found that there were 8 out of 30 cases (26.67%) of T1/2 were basically stable at the same level, while 53.33% (16/30) of the children had significant changes at each time period. Image:Summary/Conclusion: 1. Compared with the T1/2 results by WinNolin’s method and modified ond-chambor modle (”CAI’s” tool), the most consistency of T1/2 of blood sampling was 9h~48h. However, for those T1/2 short than 7~8hs, the detection time should be adjusted (the later blood sample taking should be adjust to earlier); 2. Nearly 3/4 of the 30 cases, the T1/2 results fluctuated among three periods (9h~24h, 24h~48h, 9h~48h) in one case, suggesting a standardized test procedures such as specimen collection, sample transportation and detection ect needs to set up to ensure the stability and reliability of test results. 3. The classical pharmacokinetic test requires at least 5 points of blood sample collections, which is difficult to be popularized and applied in clinical practice. If a standardized and standardized testing process is established, the one-chamber model method is relatively simple and easy to be accepted and popularized by patients. Key words: hemophilia A, pharmacokinetics, children, one-chamber model, non-av-model
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