Benign paroxysmal migraine variants of infancy and childhood: Transitions and clinical features

Introduction

Migraine variant disorders of childhood include benign paroxysmal torticollis of infancy (BPTI) and benign paroxysmal vertigo of childhood (BPVC). This study aimed to review our experience with BPTI and BPVC and determine the incidence of children transitioning between each of these disorders and to vestibular migraine (VM).

非酒精性脂肪肝炎患儿脂代谢紊乱及胰岛素抵抗研究

目的　探讨非酒精性脂肪肝炎(NASH)与脂代谢紊乱及胰岛素抵抗之间的关系。方法　对2 0 0 3年6～1 0月浙江大学医学院附属儿童医院收集的54例诊断为NASH的肥胖儿童及2 4例既无脂肪肝影像学改变也无肝转氨酶升高的单纯性肥胖儿童(对照组) ,进行血甘油三酯、胆固醇、空腹血糖/空腹血胰岛素比值(FGIR)的检测,分析NASH与高脂血症和胰岛素抵抗之间的关系。并对其中2 0例怀疑合并良性黑棘皮病的患儿行皮肤病理活检以确诊,分析NASH与良性黑棘皮病的关系。结果　54例NASH的患儿体重指数(BMI)为( 2 8 .1 0±4 .1 6) ,对照组BMI为( 2 3 . 91±1 . 88) ,二者相比,差异有显著性意义(t=5. 0 5,P <0 .0 1 )。NASH组中高脂血症及胰岛素抵抗(FGIR <7)的发生率分别为59. 2 6%和70 . 3 7% ,明显高于对照组(发生率为2 0 .83 % ,8 3 .3 % ,χ2 =9. 84,χ2 =2 5. 59,P <0 . 0 1 )。经相关分析,发现丙氨酸转氨酶(ALT)及天冬氨酸转氨酶(AST)与BMI、血胆固醇、血甘油三酯、FGIR呈显著相关(rs=0 . 41 3 ,0 . 2 9,0 . 3 79,-0 . 477,P <0 . 0 1 ;rs=0 . 3 590 3 4 .9,0 .3 4 8,-0 . 3 69,P <0. 0 1 )。且其中2 0例伴良性黑棘皮病(占3 7. 0 4% )。结论　NASH患儿存在严重的脂代谢紊乱及胰岛素抵抗。约1 /3以上NASH患儿合并良性     

181例柯兴氏综合征的临床病理

本文收集自1957至1984年181例柯兴氏综合征的肾上腺标本,对30例作了电镜观察。对88例用电子计算机作逐步回归分析,结果表明治疗方式、病理类型、紫纹和性征异常对预后影响较大。并对肾上腺皮腺癌诊断标准的掌握、皮质增生中腺瘤样结节和皮质腺瘤间可能存在的转化关系、肾上腺皮质增生的诊断标准以及柯兴氏综合征病例肾上腺病损的超微结构进行了讨论。     

PRIMARY MUCOSAL HYPERPLASIA OF THE GALLBLADDER

A case of primary mucosal hyperplasia of the gallbladder in a 55-year-old man is reported. Endoscopic ultrasonography demonstrated diffuse mucosal thickening of the gallbladder which was associated with anomalous union of the pancreatico-biliary duct system. Histologically, the mucosa showed villous and spongioid hyperplasia. The mucosa, which consisted of tall columnar cells that were morphologically similar to ordinary gallbladder epithelium, showed no metaplastic changes such as occurrence of mucous glands, Paneth cells, goblet cells, endocrine cells, or lysozyme immunoreactivity. These findings suggested that this disease was based on hyperplasia of ordinary gallbladder epithelium.     

Outcome analysis of benign vocal cord lesions by videostroboscopy, acoustic analysis and voice handicap index

Benign vocal cord lesions affect vibratory vocal fold function resulting in significant dysphonia. A prospective study of 30 patients with benign vocal fold lesions was undertaken at our centre to compare the pre and post operative voice using videostroboscopy, voice analysis and voice handicap index. The vibratory pattern of the cord improved as evidenced by the stroboscopic parameters-symmetry, mucosal wave and glottic closure (p<0.05). Voice analysis showed a trend towards normalcy but the values obtained did not attain statistical significance. Voice handicap index showed a reduction in the total and the subscales scores. Videostroboscopy along with acoustic analysis and voice handicap index are useful tools in the objective and subjective assessment of the effectiveness of treatment in patients with benign vocal cord lesions. Their routine use in a voice clinic is recommended.     

Intravascular papillary endothelial hyperplasia of the mouth: report of six cases and literature review

OBJECTIVE: Oral intravascular papillary endothelial hyperplasia (IPEH) is an uncommon, reactive vascular lesion with no specific clinical features. Microscopically two subtypes have been described: a pure and a mixed form. The importance of IPEH is in its resemblance to angiosarcoma. The aim of this study was to evaluate both the prevalence of IPEH in a consecutive series of oral vascular malformations and the clinico-pathological features. MATERIALS AND METHODS: Histological sections of 103 consecutive cases filed as vascular malformations were reviewed for histopathological criteria of IPEH. RESULTS AND CONCLUSION: We found six cases of oral IPEH, of which three were diagnosed previously. Five of these cases were in males, and the mean age was 58 years. It was usually described as a blue or reddish nodule. The lower lip mucosa was the most common site followed by the tongue and the upper lip. Histologically, thrombi were always present and five out of the six lesions appeared in a mixed form, while only one was in a pure form. Three cases were diagnosed at an early stage and in the other three, the lesions were well-established. In this series, IPEH associated with vascular malformation was more common than reported in previous studies. Lesions at early stages, especially in the mixed form, may be unnoticed.     

Nuclear and cell area measurements in the cytological evaluation of pleural effusions: a study of subjective assessments and morphometric measurements

Morphometric measurements of cellular area, nuclear area and nuclear:cytoplasmatic ratio were performed on single cells in pleural effusions from 15 patients with effusion caused by bronchial, ovarian, or mammary carcinoma. The results were compared with corresponding measurements on mesothelial cells in pleural effusions from 15 patients without malignant disease. Significant differences were found between the mean values from cellular area, nuclear area, and nuclear:cytoplasmatic ratios in mesothelial cells from benign effusion versus malignant cells from effusions associated with metastatic growth. Such morphometric measurements are, however, of little value in routine diagnostic work as more than 90% of the cells in the two groups showed similar values.     

新生儿先天性肾上腺皮质增生症筛查分析

目的探讨新生儿先天性肾上腺皮质增生症（CAH）筛查情况,为CAH的早期筛查、确诊和治疗提供依据。方法选择柳州市新生儿疾病筛查中心2010年9月至2012年12月进行CAH筛查的新生儿,采用时间分辨荧光免疫分析方法检测滤纸片17．羟孕酮（17-OHP）浓度,通过数据分析确立实验室筛查的切值。结果研究期间共筛查46592例新生儿,初筛阳性304例,确诊1例CAH。同一胎龄组中17-OHP浓度随体重增加而降低,同一体重组中早产儿17-OHP浓度大于足月儿,差异均有统计学意义（P〈0．05）。3327例早产儿97．5^th和99^th分位数值分别为40．1nmol／L和57．3nmoL／L,体重〈2500g早产儿切值定为40．0nmo]／L,体重≥2500g定为30．0nmol／L;足月儿中,体重〈2500g者97．5^th和99^th分位数值分别为20．9nmol／L和27．5nmol／L,体重2500～4000g 97．5^th和99^th分位数值分别为16．8nmol／L和21．1nmol／L,但初筛17-OHP浓度分布显示99．8％的新生儿筛查结果分布在O．0～30．0nmol／L,所以,足月儿无论体重大小17-OHP实验室筛查切值均定为30．0nmoL／L。结论确立17．OHP筛查切值,规范实验室管理,具有临床意义。     

导航鼻内镜下上颌窦良性骨性病变的治疗体会

目的 探讨经导航鼻内镜下切除上颌窦良性骨性病变的治疗体会。方法 回顾分析2005年10月至2010年1月治疗的上颌窦良性骨性病变8例的临床资料,术前行64排CT并三维重建,全部病例采用导航引导下手术切除,包括鼻内镜联合柯-陆氏径路及鼻内镜下经鼻下鼻道开窗径路。结果 3例为上颌窦内骨纤维异常增生症,5例为骨瘤,均在导航引导鼻内镜下成功切除了病变,其中6例经柯-陆氏径路切除病变,2例经鼻下鼻道开窗切除病变。随访半年以上,7例术后头痛、鼻塞、流脓涕、面部不适等症状逐渐消失,1例上颌窦内骨纤维异常增生症患者术后2月再次出现面部肿胀疼痛,保守治疗效果不佳,再次手术,发现上颌窦内有大量死骨,考虑为第1次手术后术腔未冲洗干净,导致上颌窦口堵塞,再次手术后症状消失。患者术后均未见复发。结论 影像导航系统可以准确迅速的确定病变位置,导航鼻内镜下上颌窦良性骨性病变切除是准确、安全、微创的手术方式。