特发性中枢性性早熟女童血清Kisspeptins水平变化及意义

目的 通过检测Kisspeptins在特发性中枢性性早熟(ICPP)女童血清中的水平,探讨其在ICPP发生发展过程中的作用.方法 以20名6～8岁Tanner Ⅰ期健康女童作为健康对照组,经生长发育评价结合骨龄、盆腔B超及促性腺激素释放激素激发试验结果选取20名6～8岁处于TannerⅡ期的ICPP女童作为实验1组,20名6～8岁处于TannerⅢ期的ICPP女童作为实验2组;用放射免疫法测定血清中黄体生成素(LH)水平;Western blot法检测3组女童血清中Kisspeptins水平.采用SPSS 12.0软件进行统计学分析.结果 实验1组血清Kisspeptins水平较健康对照组增加了0.86倍(P＜0.01),实验2组血清Kisspeptins水平较健康对照组增加了 1.68倍(P＜0.01);实验1组血清Kisspeptins水平与LH峰值、骨龄均呈正相关(r=0.42、0.38,Pa＜0.05);实验2组血清Kisspeptins水平与LH峰值、骨龄也均呈正相关(r=0.48、0.40,Pa＜0.05).结论Kisspeptins/G蛋白偶联受体54信号在女童ICPP的启动和发展过程中起重要作用.     

中医辨证治疗女童真性性早熟研究现状

目的了解中医对女童真性性早熟的认识,分析中医药治疗现状,总结现代中医药治疗儿童性早熟的优势。方法制定原始文献的纳入及排除标准,检索2006-01-01/2010-12-31中国知网数据库、重庆维普数据库、万方数据库并获取全文,从临床应用角度对中医药辨证治疗女童真性性早熟的方药进行归纳、整理、分析。结果符合纳入标准且不符合排除标准的25篇文献中,西医诊断标准主要参考儿科专著,中医诊断标准仍以证候诊断为主;女童真性性早熟中医辨证以阴虚火旺型最多,治疗上多采用知柏地黄丸加减;肝郁化火型以柴胡疏肝散化裁治疗;痰湿困脾型主要采用健脾燥湿药物灵活配伍治疗。结论中医辨证治疗女童真性性早熟疗效确切,值得临床中推广应用。     

青春期及青春期前和性早熟女孩瘦素水平及其意义

目的 测定正常青春期及青春期前特发性中枢性性早熟及单纯乳房发育患儿瘦素水平,研究瘦素水平与青春期的关系和影响因素。方法 在内分泌专科门诊就诊的女童71例,测定特发性中枢性性早熟、单纯乳房发育、正常未发育女童身高、体重,并据此计算体块指数,并按Tanner标准进行发育分期。采用ELISA法测定其血清瘦素及雌二醇。结果 中枢性性早熟女童瘦素水平高于单纯乳房发育及青春期前女童,差异有显著性（P〈0．001）;中枢性性早熟女童瘦素水平与正常青春期女童差异无显著性（P〉0．05）。瘦素与体块指数呈正相关,真性性早熟及正常青春期瘦素水平与雌二醇水平呈正相关。结论 在青春发育过程中一定的体脂及瘦素水平对维持青春期或第二性征和雌激素水平是必要的,瘦素可能与垂体一性腺轴的启动相关。     

运脾化痰通络法治疗女童真性性早熟30例疗效观察

目的观察运脾化痰通络法对女童真性性早熟治疗效果。方法 30例真性性早熟女童采用运脾化痰通络中药口服,3个月后观察患儿症状体征,血浆雌二醇和卵泡刺激素,促黄体生成激素,B超(子宫大小,卵巢、乳腺组织发育状况),X线(骨龄测算)等指标的变化。结果 30例病例中显效7例,有效18例,无效5例;总有效率83.33%。结论运脾化痰通络法对女童真性性早熟有明显的治疗作用,且患儿病程越短,年龄越小,疗效越好。     

儿童性早熟86例临床特点回顾性分析

目的分析性早熟儿童的临床特点,为进一步治疗提供依据。方法对86例性早熟患儿的临床资料进行回顾性分析,总结其临床特点及辅助检查结果。结果 86例性早熟患儿中,女童有82例,男童4例;17例为真性性早熟,19例为假性性早熟,50例为部分性性早熟;82例女童患者均有乳房发育,TannerⅡ~Ⅳ期,5例出现腋毛4例,6例有月经来潮,伴阴道分泌物增多;4例男童可见阴茎增大,其中3例睾丸增大,属于TannerⅢ期;17例真性性早熟患儿促性腺激素释放激素(GnRH)激发试验呈阳性,骨龄比生理年龄升高1~3年,假性性早熟及部分性性早熟患儿GnRH激发试验呈阴性,骨龄与生理年龄基本相符;患儿性早熟与环境及饮食等因素密切相关。结论儿童性早熟发病率较高,与环境及饮食均有很大的关系,值得医务工作者及家长的重视。     

内分泌系统疾病

023,47不同荆,促性腺激素释放激素类似物治疗女童特发性中枢性性早熟的临床观察/杨立础…//实用儿科临床杂志一2002,17(3)一200一201 以达菲林针10。陀/(28d·kg)肌注2次,随机改用40一50拜g/(28d·kg)。与随机改用80~100拜g/(28d·kg)治疗特发性中枢性性早熟女童各10例,每例共12次,疗程1年。起始年龄(8.3士0.9)岁;观察治疗前后两组性征、身高、骨龄及预测成年身高及性激素、生长激素变化。结果:两组治疗后性征均减退,性激素水平下降,骨龄增长受抑;两组预测成年身高改善相似。表1参4(李瑛) 023948先天性肾上腺皮质增生症的治疗与随访/祝国红…     

不同剂量促性腺激素释放激素类似物治疗女童特发性中枢性性早熟的临床观察

目的　观察不同剂量促性腺激素释放激素类似物 (GnRHa)缓释剂治疗特发性中枢性性早熟女童(ICPP)的疗效。方法　以达菲林针 1 0 0 μg/ (2 8d·kg)肌注两次 ,随机改用 40～ 50 μg/ (2 8d·kg) ;与随机改用 80～ 1 0 0 μg/ (2 8d·kg)治疗ICPP女童各 1 0例 ,每例共 1 2次 ,疗程 1年。起始年龄 (8.3± 0 .9)岁 ;观察治疗前后两组性征、身高、骨龄及预测成年身高及性激素、生长激素 (GH)变化。结果　两组治疗后性征均减退 ,性激素水平下降 ,骨龄增长受抑 ;两组预测成年身高改善相似。结论　小剂量达菲林治疗ICPP能有效抑制中枢性性早熟 ,改善患儿成年身高 ,较大剂量具有更实用、经济的优点     

内分泌系统疾病

060514特发性身材矮小/刘戈力∥中国实用儿科杂志.-2005,20(8).-454～456060515血清脱氢表雄酮水平与女性性早熟关系的探讨/蒋优君…∥浙江大学学报(医学版).-2005,34(4).-308～311,319采用ELISA法测定血清脱氢表雄酮(DHEA)及硫酸脱氢表雄酮(DHEAS)浓度,进行达菲林治疗前、后对比研究、随访。结果:1ICPP组血清Log(DHEA)·Log(DHEAS)与PT组、对照组比较差异有显著性意义;ICPP组与PT组无显著意义;Tanner期明显高于Tanner期。2经双变量相关分析,Log(DHEA)及Log(DHEAS)与身高、骨龄、卵巢容积呈正相关,与Log(LH峰值)、Log…     

无创正压通气辅助治疗重症手足口病临床观察

目的 探讨无创正压通气辅助治疗重症手足口病的疗效.方法 我院2009年4月至2010年8月收治的86例重症手足口病患儿按入院时间分为两组,A组(2009年4月至7月)常规综合治疗38例,B组(2010年4月至8月)常规综合治疗结合经鼻持续气道正压通气辅助治疗48例,比较两组总有效率.结果 A组38例,治愈25例,有效6例,无效7例,治愈率65.8％,总有效率81.5％.B组48例,治愈40例,有效7例,无效1例,治愈率83.3％,总有效率97.9％.B组治疗效果显著优于A组,差异有统计学意义(x2 =6.71,P＜0.05).结论 重症手足口病及早使用经鼻持续气道正压通气辅助治疗,对阻止重症病例向危重症发展有重要作用.     

血小板生成素和巨核细胞对特发性血小板减少性紫癜预后的影响

目的探讨血小板生成素(TPO)和巨核细胞计数对特发性血小板减少性紫癜(ITP)患儿预后的意义.方法采用夹心酶联免疫吸附法测定122例ITP患儿血清TPO水平,根据巨核细胞数分为A组(巨核细胞数≤35/6 cm2)与B组(巨核细胞数＞35/6 cm2),两组进行TPO水平比较,并观察各组疗效.结果 A和B组TPO水平分别为(368.33±39.47) ng/L,(141.48±11.28) ng/L.A组明显高于B组,有显著性差异(t=7.44 P＜0.01).A组治疗有效率为23.8%,明显低于B组84.2%,其差异有显著性(χ2=6.54 P＜0.05).A组有效5例TPO为(109.60±15.59) ng/L,无效16例TPO为(449.18±29.59) ng/L,其差异有显著意义(t=-6.23 P＜0.01).结论 ITP患儿血清TPO水平存在较大差异,TPO水平明显增高且无巨核细胞数增高者疗效差,可能提示预后不良.     

Comparison of patients with Kawasaki disease with retropharyngeal edema and patients with retropharyngeal abscess

Kawasaki disease with retropharyngeal edema (KD with RPE) is a rare complication, and it is diagnosed by neck CT. Most reported cases had a delayed diagnosis because those patients' conditions were misdiagnosed as retropharyngeal abscess (RPA). The purpose of this study was to differentiate KD with RPE from RPA. We performed a retrospective case–control study comparing children with KD with RPE to those with RPA hospitalized at the tertiary pediatric hospital in Tokyo between 2005 and 2011. The 39 patients revealing RPE on neck CT were divided into two groups: group A was classified as KD (n?=?21) and group B was classified as non-KD (n?=?18). Patients in group B were finally evaluated as having RPA clinically and were treated with antibiotic therapy. A significantly higher proportion of patients in group B complained of dysphagia (11 patients vs. 5 patients; p?=?0.0170) and neck pain (17 patients vs. 12 patients; p?=?0.0106). Neck CT revealed a ring enhancement (16 patients vs. no patients; p?<?0.0001) and mass effect in a greater proportion of patients in group B (11 patients vs. 1 patient; p?<?0.0003). Conclusion: Careful attention to manifestations and close analyses of CT imaging may allow clinicians to differentiate KD with RPE from RPA.     

Patients with moyamoya disease presenting with movement disorder

Moyamoya disease is a rare cerebrovascular disease characterized by idiopathic bilateral stenosis or occlusion of bilateral internal carotid arteries and the development of characteristic leptomeningeal collateral vessels at the base of the brain. Typical presentations include transient ischemic attacks or stroke, and hemorrhage. Presentation with movement disorders is extremely rare, especially in the pediatric population. The authors describe the cases of 4 children with moyamoya disease who presented with movement disorders. Among 446 patients (118 pediatric) with moyamoya disease surgically treated by the senior author, 4 pediatric patients had presented with movement disorders. The clinical records, imaging studies, surgical details, and postoperative clinical and imaging data were retrospectively reviewed. The initial presenting symptom was movement disorder in all 4 patients: chorea in 2, hemiballismus in 1, and involuntary limb shaking in 1. All the patients had watershed infarcts involving the frontal subcortical region on MR imaging. Additionally, 1 patient had a ganglionic infarct. Single-photon emission computed tomography studies showed frontoparietal cortical and subcortical hypoperfusion in all patients. Three patients had bilateral disease, whereas 1 had unilateral disease. All the patients underwent superficial temporal artery-middle cerebral artery bypass. Postoperatively, all 4 patients had complete improvement in their symptoms. The SPECT scans revealed normal perfusion in 3 patients and a small residual perfusion deficit in 1. Movement disorders are a rare presenting feature of moyamoya disease. Hypoperfusion of the frontal cortical and subcortical region was seen in all patients, and the symptomatology was attributed to ischemic dysfunction and imbalance in the cortical-subcortical-ganglionic-thalamic-cortical circuitry. Combined revascularization with superficial temporal artery-middle cerebral artery bypass and encephaloduroarteriosynangiosis leads to excellent results.     

Treatment with probucol of children with familial hypercholesterolaemia

Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2 +/- 1.45 mmol/l to 7.17 +/- 0.84 mmol/l (12.6%). This level was further reduced to 5.92 +/- 0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.