畏食症患儿血清瘦素水平测定的临床意义

目的探讨畏食症患儿血清瘦素水平变化及其临床意义。方法采用放射免疫分析法对1-10岁畏食症患儿54例进行血清瘦素水平测定,随机选择正常同龄儿童46例作为对照组。结果畏食症患儿血清瘦素水平男(1.42±1.26)μg/L,女(2.19±1.52)μg/L,明显低于同年龄同性别正常小儿(P<0.001)。畏食症患儿血清瘦素水平与体质量指数(BMI)无明显相关性(r男=0.12,r女=0.26P均>0.05);正常小儿血清瘦水平与BMI呈显著正相关(r男=0.73,r女:0.81P均<0.01)。结论畏食症患儿血清瘦素水平与体脂含量无明显线性关系,可能在禁食和饥饿状态下,存在独立于体脂之外的调节瘦素分泌的因素。     

肥胖儿童血浆瘦素及可溶性瘦素受体质量浓度的变化

目的观察单纯性肥胖儿童血浆瘦素及可溶性瘦素受体质量浓度的变化,探讨其相互之间及与体重指数(BMI)之间的关系。 方法于2005-06北京儿童医院采用酶联免疫吸附试验(ELISA)对40例3~6岁的单纯性肥胖儿童及按性别、年龄1∶1配对的40例正常儿童进行了血浆瘦素及可溶性瘦素受体质量浓度的检测。 结果肥胖儿童血浆瘦素质量浓度(2226±230)μg/L较正常儿童(336±023)μg/L明显升高,可溶性瘦素受体质量浓度(10010±2460)μg/L较正常儿童(13231±3017)μg/L则明显降低(P<0001)。相关性分析显示,瘦素与可溶性瘦素受体水平之间呈负相关;BMI与瘦素呈正相关,而与可溶性瘦素受体呈负相关(P<005)。 结论学龄前肥胖儿童已存在明显的瘦素抵抗现象,而可溶性瘦素受体表达减少,可能参与了瘦素抵抗的发生。     

生长激素缺乏儿童血清胰岛素样生长因子-1和瘦素水平的变化及其相互关系

目的探讨生长激素缺乏(GHD)儿童血清胰岛素样生长因子1(IGF1)、瘦素水平的变化。方法用放射免疫法分别检测20例正常青春期前儿童和23例GHD患儿血清IGF1和瘦素的水平。结果GHD组血清IGF1水平(51.158±29.988)μg/L低于对照组(112.680±41.540)μg/L,两者有显著差异(t=5.619P<0.01);瘦素水平(6.002±2.204)μg/L高于对照组(4.523±2.204)μg/L,两者比较有显著差异(t=2.225P<0.05);但IGF1和瘦素之间无相关性(P>0.05)。结论IGF1和瘦素对GHD患儿生长发育的调节作用是相互独立的。     

儿童原发性肾病综合征外周血辅助性T淋巴细胞亚群的变化

目的 探讨辅助性T淋巴细胞(Th细胞)亚群紊乱与儿童原发性肾病综合征(PNS)发病的关系.方法 选择2000年6月至2005年6月青岛大学医学院附属医院儿科收治的初发PNS患儿35例为研究对象,以15名健康儿童为对照,采用流式细胞术检测35例初发PNS患儿外周血111细胞亚群,其中22例激素敏感者于缓解期进行复查.结果 初发PNS患儿激素治疗前外周血Th1、Th2细胞百分率分别为(7.2±3.6)%和(9.7±4.5)%,与正常对照组[分别为(13.5±5.3)%和(4.5±2.9)%]比较,PNS患儿Th1细胞明显减少而Th2细胞明显增多,PNS组Th1/Th2比值(0.79±1.02)显著低于对照组(3.71±1.92),差异均有统计学意义(P＜0.01);激素敏感PNS组治疗前外周血th1细胞百分率[(6.2±3.9)%]明显低于非激素敏感组[(9.0%±3.1)%](P＜0.05),而Th2细胞百分率[(9.9±3.7)%]和Th1/Th2比值(0.70±1.14)与非激素敏感组[分别为(9.4±5.2)%和(0.94±1.06)]比较差异无显著性(P＞0.05).激素敏感组患儿缓解期(隔日疗法6～8周后)与活动期比较,Th1细胞和Th1/Th2比值显著增高而Th2细胞明显下降(P＜0.01),而与对照组比较差异均无统计学意义(p＞0.05).结论 ,Th细胞亚群紊乱与儿童PNS的发病关系密切,检测初发PNS患儿外周血Th细胞亚群对预测激素疗效有一定帮助.     

儿童原发性肾病综合征瘦素及受体水平变化

目的检测儿童原发性肾病综合征（PNS）血清瘦素（leptin）、可溶性瘦素受体（sOBR）滴度,探讨外周组织瘦素受体（OBR）mRNA表达强弱对血清sOBR、leptin状态的影响。方法分别采用ELISA、RT-PCR法检测23例未经激素治疗的PNS患儿空腹血清、尿液leptin和sOBR水平、以及外周血单个核细胞（PBMC）上OBR基因表达水平,并与在年龄、性别、体重指数（BMI）均匹配的15例门诊正常体检儿童比较。结果空腹血清总leptin水平。肾病组与对照组相当,尿leptin。肾病组高于对照组;肾病组血清sOBR水平明显低于对照组,尿sOBR检测值两者差异无显著性;血清中代表游离leptin水平的游离leptin指数（FLI）肾病组高于对照组;PBMC上OBR基因表达强度。肾病组低于对照组。结论PNS患儿血清leptin总水平正常,sOBR水平降低,致使机体实际发挥生物学作用的游离leptin增多;其中血清sOBR减少与PNS患儿外周组织OBR基因表达减少有关。     

儿童原发性肾病综合征脂联素与其他脂代谢相关因素的探讨

目的 初步探讨儿童原发性肾病综合征(PNS)脂联素(ADPN)的变化,了解ADPN与PNS其他脂代谢相关因素的联系.方法 对71例PNS急性期患儿、37例缓解期患儿和35例健康儿童的血浆、尿液ADPN浓度进行定量分析,并将急性期患儿分为激素敏感组59例和激素耐药组12例,测定急性期患儿的体质指数(BMI)、肾小球滤过率(GFR)、血清白蛋白(Alb)、肌酐(Cr)、补体C3、三酰甘油(TG)、胆固醇(Tch)、高密度脂蛋白(HDL)、低密度脂蛋白(LDL)、脂蛋白aLP(a)、载脂蛋白A(apoA)、载脂蛋白B(apoB)以及24 h尿蛋白定量,并进行相关性分析.结果 急性组的血浆、尿ADPN(14.02 ± 3.44 μg/ml,4.71 ± 2.12 μg/ml)显著高于缓解期组(7.13 ± 5.07 μg/ml,2.50 ± 1.06 μg/ml)和正常对照组(7.17 ± 2.94 μg/ml,2.60 ± 1.06 μg/ml)(P ＜ 0.01).激素耐药组血浆、尿ADPN均显著高于激素敏感组(P ＜ 0.05).PNS急性期患儿血浆ADPN分别与尿ADPN、血清TG、Tch、LDL、LP(a)、apoB及24 h尿蛋白定量正相关(r ＝ 0.242 7 ～ 0.609 1,P均＜ 0.05),与血清Alb、HDL负相关(r ＝ -0.745 4、-0.489 7,P ＜ 0.01).结论 PNS患儿的血浆及尿液ADPN浓度明显升高.血浆和尿液ADPN浓度与其他脂代谢指标密切相关.     

足月新生儿脐血瘦素水平测定及其与胰岛素的关系

目的探讨瘦素对胎儿生长发育的影响及其与胰岛素的关系。方法采用放射免疫分析法对83例足月新生儿脐血瘦素、胰岛素水平进行测定,测量新生儿出生体质量、身长,记录性别及分娩方式,计算体质量指数(BMI)评估新生儿营养状况。结果1.足月新生儿脐血瘦素水平为(10.53±7.05)μg/L,其中男婴(9.01±4.53)μg/L;女婴(11.62±5.03)μg/L。剖宫产组瘦素水平(10.07±5.88)μg/L;阴道分娩组瘦素(13.55±12.00)μg/L。瘦素水平在性别之间无明显差异(t=1.934 P>0.05),分娩方式间亦无明显差异(t=-1.216 P>0.05)。2.新生儿脐血瘦素水平与BMI呈显著正相关(r=0.520 P<0.01)。瘦素水平在大于胎龄儿(LGA)、适于胎龄儿(AGA)、小于胎龄儿(SGA)组分别为(17.29±8.99)(、10.54±4.96)(、3.33±1.58)μg/L,3组间有明显差异,LGA组明显高于其他两组(F=16.5 P<0.01)。3.新生儿脐血瘦素水平与胰岛素呈显著正相关(r=0.436 P<0.01)。结论瘦素在胎儿宫内生长和发育过程中起重要的调节作用,与胰岛素关系密切。     

瘦素及可溶性瘦素受体在原发性肾病综合征患儿血脂升高中的作用

目的探讨瘦素(Leptin)及可溶性瘦素受体(sOBR)在儿童原发性肾病综合征(PNS)血脂升高中的作用。方法检测23例未经治疗的PNS患儿空腹血清血脂、血浆清蛋白、Leptin、sOBR、胰岛素及尿Leptin、sOBR水平,并与在年龄、性别、体质量指数(BMI)均匹配的15例正常儿童比较。结果肾病组血胆固醇(TC)、三酰甘油(TG)、低密度脂蛋白(LDL)、载脂蛋白B(apoB)水平增高,血浆清蛋白、胰岛素水平较对照组降低。空腹血清总Leptin水平肾病组与对照组相当,而尿Leptin水平肾病组高于对照组;血清sOBR水平肾病组降低,尿sOBR水平二者无差异;血清游离Leptin指数(FLI)肾病组高于对照组。肾病组游离Leptin指数(FLI)与血浆清蛋白、HDL、apoA呈正相关,与LDL、胰岛素呈负相关。结论PNS患儿血清sOBR减少,游离瘦素增多可能为一抗高脂血症的代偿机制,但其纠正PNS脂代谢紊乱的能力并不完全。     

糖皮质激素对肾病综合征患儿成骨细胞功能的影响

目的　糖皮质激素是治疗肾病综合征的首选药物。但糖皮质激素可抑制成骨细胞功能,导致骨质疏松。该研究通过检测成骨细胞不同分化阶段的生化指标:I型前胶原羧基端前肽(PICP)、骨钙素(BGP)和总碱性磷酸酶(AKP),探讨糖皮质激素对肾病综合征(NS)患儿成骨细胞功能的影响。方法　测定正常对照组(n=30),未治NS患儿(n=30)和激素治疗后NS患儿(每日泼尼松2mg/kg治疗4 ~8周,n=30)血清PICP、BGP及AKP水平。结果　未治NS患儿血清PICP165 ±56μg/L,BGP15 ±9ng/L水平明显低于正常对照组205 ±81μg/L, 19 ±12ng/L(均P<0. 05),而血清总AKP198 ±71U/L与正常对照组202 ±46U/L比较差异无显著性。激素治疗后NS患儿血清PICP85 ±56μg/L、BGP8±5ng/L、AKP104 ±59 U/L均明显低于未治NS患儿(P<0. 01)。结论　NS患儿本身存在骨合成障碍,大剂量糖皮质激素治疗可进一步抑制NS患儿的成骨细胞合成功能。     

肥胖儿童运动后血脂、瘦素、糖耐量、BMI改变及其意义

目的探讨运动对单纯性肥胖症儿童的治疗作用。方法采用假期集中训练形式,对32名诊断为肥胖症儿童进行3周的计划运动,于住院后第1天,检测胆固醇(Chol)、甘油三脂(TRG)、低密度脂蛋白(LDL?C)、胰岛素,测量BMI值,第2天作口服葡萄糖耐量试验(OGTT)。于结束运动疗程当天测量BMI;并以同样方式重复检测代谢指标。用SPSS软件处理数据,比较运动前后变化。结果Chol从(4.71±0.79)mmol/L→(3.62±0.69)mmol/L;TRG从(1.09±0.49)mmol/L→(0.85±0.36)mmol/L;LDL?C从(1.43±0.24)mmol/L→(1.07±0.25)mmol/L瘦素从(47.25±16.69)μg/L→(23.71±11.86)μg/L;BMI从(27.38±2.65)→(25.12±2.38)kg/m2。OGTT试验,运动前3例异常并空腹血糖增高,3例单纯OGTT异常(IGT);运动后仅2例OGTT异常(IGT)并空腹血糖升高,最后诊断2型糖尿病。结论适当运动锻炼能使肥胖儿童BMI降低,脂代谢改善,降低肥胖儿童并发心血管病的危险;适当运动还能改善机体血糖利用,纠正糖耐量异常,降低肥胖儿童患2型糖尿病的风险。     

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.     

Treatment with Probucol of Children with Familial Hypercholesterolemia

ABSTRACT. 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.     

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.     

Treatment of children with congenital cytomegalovirus infection with ganciclovir

BACKGROUND: Congenital cytomegalovirus (CMV) infection affects approximately 1% of live births in the US. Ten percent of these infants have symptoms at birth and another 10 to 15% acquire hearing loss or developmental problems. Congenital CMV is the most common cause of nonhereditary sensorineural hearing loss in children, and progressive hearing loss is common. To arrest the natural progression of congenital CMV, children referred to our center were treated with a prolonged course of ganciclovir. METHODS: Medical records of children with congenital CMV who were treated with ganciclovir were reviewed to tabulate their presenting symptoms, duration of treatment, audiologic and developmental assessments and complications. RESULTS: We treated nine children with symptomatic CMV with iv ganciclovir at a median age of 10 days (range, 3 days to 11 months). Findings at diagnosis included microcephaly (five of nine); petechiae (five of nine); thrombocytopenia (seven of nine); and intracranial calcifications (six of eight). Hearing loss was noted before therapy in five of nine. The median duration of iv and subsequent oral ganciclovir was 1 year and 0.83 year, respectively. Median follow-up was 2 years (range, 1 to 7 years). No child had progression of hearing loss; improvement occurred in two. Seven children had at least one complication of ganciclovir therapy: central venous catheter/site infection (six); catheter malfunction (three); and neutropenia (one). CONCLUSION: Of nine children none treated with ganciclovir for congenital CMV had detectable progressive hearing loss. Complications associated with iv therapy occurred frequently. Currently available oral analogues of ganciclovir may facilitate earlier and more prolonged therapy for children with symptomatic congenital CMV and should be subjected to randomized controlled trials.     

Treatment of Children with Advanced-Stage Lymphoblastic Lymphoma with Pegaspargase

Objective: To evaluate the feasibility of Pegaspargase instead of L-asparaginase to treat children with advanced-stage lymphoblastic lymphoma (LBL) on the Berlin-Frankfurt-Munster (BFM)-95 protocol. Methods: Fifty-four newly diagnosed patients with stage III or IV LBL and without any treatment were enrolled in this study. Pegaspargase took place of L-asparaginase in BFM-95. The complications and treatment responses of patients treated on the BFM-95 protocol and modified BFM-95 protocol were then evaluated respectively. Findings : For LBL patients treated with BFM-95 protocol or modified BFM-95 protocol, the complete response, event-free survival, overall survival were similar. Stage 4 myelosuppression was the most common complication in both groups. Besides that, among 31 patients receiving modified BFM-95 protocol, coagulation defects were the most common complication. In contrast, anaphylactic reaction was the most common complication in the other 23 patients receiving BFM-95 protocol. Conclusion: Modified BFM-95 protocol is available to children with advanced-stage LBL with an equal outcome and enhances its compliance and decreases the incidence of anaphylactic reaction, compared to BFM-95 protocol. Coagulation defects are the major complication and tolerable in modified one.Key Words: Pegaspargase, L-Asparaginase, Lymphoblastic Lymphoma, Chemotherapy     

Growth of infants with osteogenesis imperfecta treated with bisphosphonate

Background:  Osteogenesis imperfecta (OI) is a heritable bone disease characterized by bone brittleness and various degrees of growth disorder. Cyclic pamidronate therapy is reportedly useful to prevent bone fracture in OI and in infants with OI, but, it remains unclear how infants with OI grow during bisphosphonate therapy.
Methods:  Height and weight measurements of OI infants treated with cyclic pamidronate therapy were taken before and every 6 months during therapy until 18 months. Vertebral morphometry and the concavity index were analyzed using X-ray films taken simultaneously.
Results:  Among OI patients, those in the group for which the height z- score decreased tended to have more femur fractures than those of the group for which the height z- score increased. Morphometry of the lumbar spine showed that compression fractures occurred less during cyclic pamidronate therapy, by which the lumbar bone mineral density increased.
Conclusions:  Bisphosphonate preserved vertebral morphometry during 18 months after starting therapy in infants. Prevention of femur fracture during the infantile period might help prevent short stature; therapeutic strategies during infancy must better emphasize prevention of long bone fracture before the beginning of gait.