High-resolution Spectral Domain Optical Coherence Tomography Findings in Multifocal Vitelliform Macular Dystrophy

We describe the abnormalities seen in the mid periphery and posterior pole of two patients with multifocal vitelliform macular distrophy as evaluated by high-definition spectral domain optical coherence tomography (HD-OCT). In patient 1, HD-OCT scans revealed, in the central area, a thicker and more reflective layer compared with the normal macula, located between the retinal pigment epitelium and the interface of the inner segment /outer segment, corresponding to the Verhoeff‘s membrane. Moreover, HD-OCT macular scans, as well as C-scans, revealed a slight hyper-reflective lesion just above an area of reduced reflectivity between the photoreceptor layer (interface of the inner segment and outer segment) and the Verhoeff‘s membrane. In patient 2, on HD-OCT macular scans, the layer corresponding to the interface of inner segment and outer segment of the photoreceptor, and the Verhoeff's membrane, appeared disrupted, whereas the retinal pigment epithelium layer appeared preserved. On the other hand, in both patient 1 and 2, the clinically evident vitelliform lesions outside the macular area appeared on HD-OCT scans either as small focal hyper-reflective lesions at the level of the retinal pigment epithelium/photoreceptor complex, either as a more pronounced diffuse thickening of the retinal pigment epithelium/photoreceptor complex, facing the deposition of lipofuscin reported on the histopathologic examination. These new findings would help in a further understanding of multifocal vitelliform macular distrophy.     

Outcomes for patients with infantile idiopathic scoliosis by casting table type

Purpose:Serial casting is an effective treatment for infantile idiopathic scoliosis. The most common casting table types are Mehta, Risser, and spica tables. We compared major curve correction between patients with infantile idiopathic scoliosis treated using pediatric hip spica tables versus Risser or Mehta tables.Methods:In this multicenter retrospective study, we included 52 children younger than 3 years (mean ± standard deviation age, 1.6 ± 0.68 years) treated with ≥2 consecutive casts for infantile idiopathic scoliosis between September 2011 and July 2018. We compared major curve angle (measured using the Cobb method) before and after treatment and improvement in curve angle between the spica tables group (n = 12) and the Risser or Mehta tables group (n = 40). The primary outcome was the difference in percentage correction of the major curve according to radiographs taken after first casting and at final follow-up.Results:The mean major curve was 47° ± 18° before casting. A median of six casts (range: 2–14) were applied. Mean follow-up after treatment initiation was 22 months (range: 7–86 months). At baseline, the major curve was significantly larger in the spica tables group (58°) than in the Risser or Mehta tables group (43°) (p = 0.01). We found no differences in the percentage curve correction in the spica tables group versus Risser or Mehta tables group after first casting or at final follow-up.Conclusion:Serial casting was associated with substantial major curve correction in patients with infantile idiopathic scoliosis. Curve correction did not differ between patients treated with a spica table versus a Risser or Mehta table.Level of Evidence:Level III, retrospective cohort study     

Historical and pathological overview of Castleman disease

Castleman disease consists of several lymphoproliferative subtypes that share some histological features in the lymph nodes. On the other hand, numerous clinical findings and etiologies make the disease challenging to understand. The origin of the disease is the hyaline vascular-type unicentric Castleman disease (UCD), first reported by Benjamin Castleman et al. in 1954. Although UCD is characterized by localized lesions and lack of symptoms, multicentric Castleman disease (MCD) with multiple lesions and systemic symptoms was reported by Frizzera in 1983. MCD is further divided according to KSHV/HHV8 infection status. In KSHV/HHV8-related MCD, viral infection signals lead to excessive cytokine production, and cause clinical and pathologic abnormalities. Some cases of plasma cell-type KSHV/HHV8-negative MCD can be found in association with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes), which is a paraneoplastic syndrome. The others are idiopathic MCD, which are currently considered a heterogeneous group of diseases with overlapping pathological and clinical features. In this article, we summarize the historical evolution of Castleman disease to help understand the disease concept. We also review the latest ideas and definitions of the subtypes within the MCD spectrum and summarize the histopathological findings.     

Atrial Fibrillation-triggered Ventricular Fibrillation in a Patient with Early Repolarization Syndrome

A 54-year-old man with early repolarization syndrome (ERS) implanted with an implantable cardioverter-defibrillator (ICD) developed persistent atrial fibrillation (AF) three years after the implantation. Similarly, the remote monitoring system begun frequently detecting ventricular fibrillation (VF) and polymorphic ventricular tachycardia (PVT). Longer RR intervals were repeatedly observed just before the initiation of PVT/VF. Catheter ablation for AF successfully diminished both the PVT and VF events.     

紫癜汤抑制ITP模型小鼠血小板破坏的作用机制研究

[目的] 紫癜汤对特发性血小板减少性紫癜(ITP)模型小鼠脾脏巨噬细胞(MΦ)凋亡及微观结构的影响,探讨该方临床使用的作用机制.[方法] 将48只BALB/C小鼠随机分为4组:正常组、模型组、泼尼松组、紫癜汤组,除正常组外,其余各组采用免疫法腹腔注射豚鼠抗小鼠血小板血清(GP-APS)建立ITP小鼠模型,观察小鼠外周血小板、脾中巨噬细胞的微观结构及其凋亡情况.[结果] 治疗后,两药物组均能提高ITP小鼠血小板计数,与用药前比较差异有统计学意义;紫癜汤组酝椎的凋亡率提高,与模型组比较,疗效优于醋酸泼尼松(P<0.05);两药均能改善酝椎的微观结构,紫癜汤优于醋酸泼尼松.[结论] 紫癜汤通过改善ITP模型小鼠脾中巨噬细胞的微观结构及促进巨噬细胞的凋亡起到治疗作用.     

特发性身材矮小儿童成骨细胞的功能状况

目的通过检测特发性身材矮小(ISS)儿童血清骨碱性磷酸酶(BAP)及骨钙蛋白(OC)水平,探讨其成骨细胞的功能状况。方法ISS组36例及健康对照组儿童50例。ISS组分为青春期前组及青春期组。测各组儿童身高、体质量、体质量指数,采用CHN法评定左侧手、腕部骨化指标,计算骨龄。采用ELISA法测其血清BAP及OC水平。结果青春期前,ISS儿童血清BAP及OC水平为(79.90±25.96)U/Lvs(60.96±18.46)μg/L,健康对照组为(152.17±35.36)U/Lvs(76.16±28.03)μg/L;青春期,ISS儿童血清BAP及OC水平为(108.33±35.20)U/Lvs(63.82±24.81)μg/L,健康对照组为(156.30±35.29)U/Lvs(104.92±28.26)μg/L。青春期前及青春期ISS组血清BAP及OC水平均明显低于健康对照组,存在显著性差异(Pa<0.01)。结论ISS儿童以成骨细胞为中心环节的骨塑造和再造能力均较健康生长发育少年儿童差。     

开角型青光眼静态视野黄斑光阈值的改变

目的 观察开角型青光眼患者静态视野黄斑光阈值改变，探讨测定黄斑光阈值与青光眼早期视功能损害的关系。方法 采用Dicon全自动静态阈值视野计，分别对89例开角型青光眼(早期青光眼45例，中晚期青光眼44例)和正常人40名黄斑光阈值进行测定。结果 早期青光眼患者黄斑光阈值明显高于正常对照组，中晚期患者高于早期患者，3组数据差异有统计学意义。结论 测定黄斑光阈值对于青光眼早期视功能损害的评价有一定的临床意义。     

儿童特发性室性心动过速的临床诊治特点探讨

目的 探讨儿童特发性室性心动过速(IVT)的临床诊治特点。方法 对18例诊断为IVT的患儿，就其临床表现、心电图特征、部分患儿的运动试验(Brace方案)检查结果及临床治疗转归进行分析。结果 1．儿童IVT主要起源于右心室流出道及左心室间隔；2．37％右室流出道IVT患儿有明屁的心悸主诉，且与运动、情绪、感染等有关：90％左室IVT。有心悸、面色苍白；3．右室流出道IVT常伴有频发室性早搏，VT多数为非持续性VT，运动试验VT的诱发率高；左室IVT均呈持续性VT发作，能为心房或心室的程序刺激所诱发；4．腺苷、β受体阻滞剂、钙通道阻滞剂能较好地防治，VT的发生；5．大多数儿童，VT具有很好的预后，射频消融能根治IVT。结论 儿童IVT是发生于无器质性心脏病证据或致心律失常因素的一种VT，预后良好。有部分病人在疾病的某个过程需要药物的防治．其中大部分可以发展为不用药，甚至痊愈。对于症状严重、发作频繁的患儿，射频消融可使IVT得以根治。     

A quantitative and semi-automatic measurement of transverse sinus stenosis improves idiopathic intracranial hypertension diagnostic accuracy

We propose a new reliable transverse sinus stenosis (TSS) index based on magnetic resonance venography (MRV) for the diagnosis of idiopathic intracranial hypertension (IIH). Our quantitative semi-automatic measurement analysis based on segmentation and cross-sectional TS diameter from 48 IIH patients and controls matched for age and sex, had a good inter-observer agreement (κ = 0.729) compared to a visual examination (κ = 0.467). A cut-off point ≥ 2 discriminate IIH patients from controls, with a sensitivity and specificity of 100%.     

Disturbed hypothalamic-pituitary axis in idiopathic recurring hypersomnia syndrome

Disturbed function of the hypothalamic-pituitary axis at adrenal and thyroid levels was found during an episode of hypersomnia in the idiopathic recurring hypersomnia syndrome. These endocrinological abnormalities, abolished ACTH and cortisol responses to insulin-induced hypoglycemia and absent TSH response to TRH, normalised thereafter in the symptom-free interval. These data support the hypothesis that Kleine-Levin syndrome is related to an intermittent hypothalamic dysfunction.