原发甲状腺功能低下患儿治疗前后生长激素的变化

目的　观察原发甲状腺功能低下患儿 (甲低 )治疗前后生长激素 (GH)的变化。方法　用放射免疫法测定患儿治疗前后空腹T3、T4 、甲状腺刺激激素 (TSH)、卵泡刺激素 (FSH)、黄体生成素 (LH)、催乳素 (PRL)、生长激素 (GH) ,并做头颅CT或MRI。结果　治疗前T3、T4 明显下降 ,TSH、FSH、LH、PRL明显上升 ,GH刺激试验示GH分泌不足 ,头颅CT或MRI示垂体微腺瘤。用甲状腺片治疗后T3、T4 、TSH、FSH、LH、PRL渐正常 ,并于 3、6个月复查头颅CT或MRI示垂体微腺瘤消失 ,GH刺激试验示GH分泌正常。治疗前后各组数据有显著差异 ,与治疗前比较P <0 .0 1。结论　原发甲低患儿可出现暂时GH下降及垂体假性微腺瘤     

GnRHa激发试验对性早熟的诊断价值探讨

目的　探讨GnRH类似物 (GnRHa)曲普瑞林 (triptorelin)激发试验对于性早熟的诊断价值。方法　将因乳房发育提前而就诊的 95例女孩 (均于就诊时行 0、30和 6 0minGnRHa激发试验 )分为 3组 :真性性早熟(CPP)组 5 0例、单纯性乳房早发育 (PT)组 32例、外周性性早熟 (PPP)组 13例。以 33例健康的青春前期女孩作为对照。比较各组血清促卵泡激素 (FSH)、促黄体生成激素 (LH)激发值和基值、FSH和LH的最大反应 (ΔFSH和ΔLH)及峰值之比 (峰LH/FSH ,免疫化学发光法测定 )。绘制诊断CPP的受试者工作特性曲线 ,确定具有较高诊断灵敏度和特异度的峰LH和峰LH/FSH的截断值。结果　PPP、PT、CPP和对照组的FSH基值以及LH基值均有明显的重迭。PPP组 3个时相的血清FSH及LH浓度无差异。PT、CPP组 3个时相的血清FSH及LH浓度均有显著差异 (P =0 0 0 )。PT组ΔFSH (8 76± 3 74 )IU/L显著高于ΔLH(2 5 7± 1 5 6 )IU/L(P =0 0 0 )。CPP组ΔLH(2 3 2 3± 19 16 )IU/L显著高于ΔFSH(10 6 0± 4 79)IU/L(P =0 0 0 )。PT组和CPP组的ΔFSH无差异 ,CPP组ΔLH和峰LH/FSH(1 6 6± 1 2 6 )显著高于PT组 (0 2 8± 0 11) (P =0 0 0 )。以LH峰值≥5 0 5IU/L为前提 ,诊断CPP的灵敏度和特异度分别为 98 0 %和 90 6 %。结论　PPP组     

胰岛素样生长因子1及胰岛素样生长因子结合蛋白3与胎儿宫内生长发育的关系

目的　通过测定新生儿脐血中胰岛素样生长因子 1(IGF 1)和胰岛素样生长因子结合蛋白 3(IGF BP 3)水平 ,研究其与出生体质量、身长及胎盘质量等生长参数间的关系 ,探讨影响胎儿宫内生长发育的内分泌因素。方法　将新生儿根据出生体质量与胎龄的关系分为适于胎龄儿 (AGA)组与小于胎龄儿 (SGA)组 ,分别测定两组出生时身长、体质量和胎盘质量 ,同时取新生儿脐血用免疫放射法测定IGF 1和IGFBP 3含量。结果　1) 10 5例新生儿中 79例AGA和 2 6例SGA ,其出生时身长、体质量和胎盘质量 3个参数比较均存在显著差异(P <0 .0 0 1) ,AGA组显著高于SGA组 (P <0 .0 0 1) ;2 )两组脐血IGF 1和IGFBP 3比较 ,AGA组IGF 1和IGF BP 3水平均高于SGA组 (P <0 .0 1和P <0 .0 0 1) ;3)出生时身长、体质量和胎盘重质 3个生长参数均与IGF 1和IGFBP 3呈显著正相关 (P均 <0 .0 0 1)。结论　 1.出生时身长、体质量和胎盘质量可用来评价AGA和SGA的生长发育。 2 .AGA脐血中IGF 1和IGFBP 3明显高于SGA。 3.胎儿自身分泌IGF 1和IGFBP 3与上述生长参数密切相关 ,其对胎儿的生长发育起重要调节作用。IGF 1和IGFBP 3可作为胎儿宫内生长发育的指标     

性早熟女性患儿血清IGF-1和IGFBP-3质量浓度检测及临床价值

目的　探讨女性特发性中枢性性早熟(ICPP)及乳房早发育患儿血清胰岛素样生长因子 1 (IGF- 1 )和胰岛素样生长因子结合蛋白 3 (IGFBP -3 )的关系及临床意义。方法　以放射免疫法测定于2 0 0 0年5月至2 0 0 4年1月在暨南大学医学院第二附属医院就诊的2 2例ICPP及2 8例乳房早发育女孩血清IGF- 1和IGFBP -3的水平,并以2 5名正常青春发育期女孩及3 0名未发育女孩作为对照,以IGF- 1、IGFBP- 3为诊断指标,对ICPP进行诊断试验评价。结果　ICPP女性患儿血清IGF -1、IGFBP- 3水平均明显高于乳房早发育及未发育女孩(P <0 .0 1 ) ,而与正常青春发育女孩差别无显著性意义(P >0 .0 5)。IGF- 1 >2 69 .1 4mg/L对诊断ICPP的灵敏度、特异度、阳性预测值、准确度分别为95% ,96% ,95% ,96% ;IGFBP -3 >3 53 6 42mg/L对诊断ICPP的灵敏度、特异度、阳性预测值、准确度分别为72 % ,96% ,94% ,86%。结论　ICPP女性患儿血清IGF 1、IGFBP- 3水平明显增高,IGF -1、IGFBP -3对鉴别ICPP与乳房早发育具有临床意义。     

内分泌系统疾病

311 ,72326特发性垂体性生长激素缺乏症下丘脑一垂体一靶腺轴完整性探讨/沈永年一//临床儿科杂志一1996,14(6)一363一365 对25例典型的垂体性GH缺乏症进行下丘脑一垂体一甲状腺、肾上腺和性腺轴功能评价。经TRH、ITT和LHRH激发试验结果表明:单一GH缺乏症3例,占12%,多种垂体激素缺乏22例,占88%。其’中垂体TSH、ACTH、F’SH或LH储备和分泌功能低下分别’为一4(56%)、3(12%)和20(8(〕%)例。伴随TSH和FSH/LH同时受累n例(44 .0%),TSH和ACTH同时受累2例(8%),TSH、ACTH和FSH/LH同时受累1例(4%)。了解垂体其它激素不足,及时…     

危重新生儿甲状腺功能状态探讨

目的　 探讨危重新生儿甲状腺功能的变化。 方法 　对 3 0例危重新生儿 (危重组 )及 18例同期出生的正常新生儿(对照组 )于第 3～ 7天 (其中 18例危重新生儿于恢复期复查 )取静脉血检测T3、T4 、FT3、FT4 、rT3和TSH。 结果 　危重新生儿T3、T4 、FT3、FT4 均显著低于对照组 (P <0 0 1或P <0 0 5 ) ,rT3显著高于对照组 (P <0 0 1) ,TSH无显著差异 (P >0 0 5 ) ,恢复期危重新生儿T3、T4 、FT3、FT4 可基本恢复正常 ,但仍略低于对照组 (P >0 0 5 )。 结论 　危重症新生儿甲状腺功能损害严重 ,疾病恢复后甲状腺功能自行恢复 ,无需甲状腺素治疗     

早期营养干预对宫内生长迟缓大鼠胰岛素样生长因子和小肠发育及生长追赶的影响

目的　探讨早期营养干预对宫内生长迟缓 (IUGR)幼鼠血清胰岛素样生长因子 (IGF)和小肠发育及生长追赶的影响。方法　将 2 4只IUGR新生雌鼠和 8只正常新生雌鼠随机分为 4组 :IUGR模型组 ,IUGR低蛋白组 ,IUGR高蛋白组 ,正常对照组。于生后 4周时检测各组大鼠血清IGF1、胰岛素样生长因子结合蛋白 3(IGFBP3)浓度、体重、身长和小肠重量、长度及肠黏膜组织结构变化。结果　 4周时IUGR高蛋白组血IGF1、IGFBP3和小肠黏膜绒毛高度、吸收表面积均显著高于对照组和IUGR模型组 (P <0 .0 5或P <0 .0 1) ;小肠重量、长度和体重、身长与对照组比较无显著性差异 (P均 >0 .0 5 )。结论　生后头 4周予高蛋白饮食早期营养干预IUGR幼鼠 ,可通过促进小肠发育达到满意的体格生长追赶。血清IGF1是反映生长追赶的灵敏指标     

中枢神经系统感染患儿脑脊液和血清胰岛素样生长因子Ⅱ水平的变化及意义

目的　探讨中枢神经系统 (CNS)感染患儿脑脊液 (CSF)中胰岛素样生长因子 Ⅱ (IGF Ⅱ )水平的变化及其与CSF中葡萄糖浓度、蛋白质浓度和白细胞计数的关系。方法　 45例CNS感染患儿分为 2组 ,其中细菌性脑膜炎 (BM) 2 4例、病毒性脑炎 (VE) 2 1例 ,取 15例无神经系统疾病患儿的CSF作为对照组。用免疫放射分析法 (IRMA)测定CSF中IGF Ⅱ的浓度。结果　 1.BM组CSF中IGF Ⅱ浓度 64 .5 6± 49.61ng/ml显著高于VE组 3 1.41± 7.16ng/ml(P <0 .0 1)和对照组 2 7.19± 6.85ng/ml(P <0 .0 1) ;2 .CSF中IGF Ⅱ浓度与CSF中葡萄糖浓度呈负相关 (r =- 0 .5 61　P <0 .0 1) ,与CSF中蛋白浓度呈正相关 (r =0 .83 9　P <0 .0 0 1)。结论　1.IGF Ⅱ参与CNS感染的病理生理过程 ;2 .IGF Ⅱ可能参与CNS感染时CSF中葡萄糖和蛋白浓度变化的调节 ;3 .测定CSF中IGF Ⅱ浓度可对BM和VE的鉴别诊断提供帮助。     

儿童垂体功能减退症

垂体是维持生命的重要器官,包括垂体前叶和垂体后叶,垂体前叶也称"腺垂体",由5种细胞分泌6种激素,分别为生长激素(GH)、促肾上腺皮质激素(ACTH)、促甲状腺素(TSH)、促黄体生成激素(LH)、促卵泡刺激激素(FSH)及催乳素(PRL);垂体后叶为神经垂体,分泌血管加压素.垂体前叶激素TSH、ACTH、LH、FSH促使其相应腺体(靶器官)分泌三碘甲状腺原氨酸(T3)及甲状腺素(T4)、肾上腺皮质激素、性激素包括睾酮(T)和雌二醇(E2),GH、PRL则直接与相应的细胞受体蛋白相结合发挥其各自的生理效应.     

夜间尿促性腺激素对判断儿童促性腺激素释放激素类似物治疗后下丘脑-垂体-性腺轴功能的价值

目的 探讨免疫化学发光法( ICMA)检测夜间12 h尿促性腺激素对判断儿童促性腺激素释放激素类似物(GnRHa)治疗后下丘脑-垂体-性腺轴(HPGA)功能状态的价值.方法 患儿12例,其中中枢性性早熟4例(女,年龄7.3 ～9.8岁),青春期预测终身高矮小8例(男3例,女5例;年龄8.8～12.3岁).在GnRHa治疗前及治疗3个月后检测其夜间12 h尿黄体生成素(LH)和卵泡刺激素(FSH).结果 GnRHa治疗前后患儿夜间12 h尿液LH/Cr分别为(339.14±264.02) IU·mol -1和( 43.39±36.65) IU·mol-1(t=3.727,P=0.003),FSH/Cr分别为(1841.59±1287.46) IU·mol -1和( 348.20±165.22) IU·mol-1(t =3.968,P=0.002),当尿LH/Cr＞ 56.44 IU·mol-1同时又有FSH/Cr＞ 604.97 IU·mol-1时,其灵敏度及特异性分别为91.7％及100.0％;而仅当FSH/Cr＞ 604.97 IU·mol-1时,诊断HPGA未被抑制状态具有93.8％的灵敏度及85.7％的特异性;仅当LH/Cr＞ 56.44 IU·mol-1时,则其具有91.7％的灵敏度及83.3％的特异性.结论 夜间12 h尿液LH/Cr和FSH/Cr可用来判断GnRHa治疗后HPGA功能状态.     

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.