特发性钙尿症与细胞膜钙泵活性的研究

采用尿钙与肌酐比值（Ｃａ／Ｃｒ）筛查广东某镇６２３例学龄儿童特发性高钙尿症（ＩＨ）。对比值超过第９５百分位值再测２４小时尿钙。结果检出ＩＨ１６例。检测ＩＨ患儿血红红细胞膜钙泵活性，发现吸收型显高于正常对照组。提示ＩＨ患儿存在体内钙泵活性异常。ＩＨ患儿家族泌尿系结石发病率显正常对照组，提示ＩＨ可能与本地区泌尿系结石发病较高有关。     

小儿血尿与特发性高钙尿症

特发性高钙尿症(Idiopathic hypercalciuria,IH)在小儿单纯性血尿中较多见,尤其多见于非肾小球血尿的患儿,IH在小儿单纯性血尿中的发病率为28％～35％,若不予干预,2年～15年后72％的患儿有泌尿系结石形成。     

儿童特发性高钙尿症（讲座）

近年来儿童特发性高钙尿症(Idiopathie Hy-percaleiuria,IH)已引起广泛注意。美国Moore等1978年报道发病率为2.9～6.2%,Stapleton等1983年报道发病率为4.2%,国内魏珉等1991年报道北京儿童的发病率为1.7%。IH不仅是泌尿系钙性结石的主要原因,且是儿童常见泌尿系症状,如镜下血尿、发作性肉眼血尿、尿频尿急综合征、尿失禁、反复发作泌尿系感染和痉挛性腹痛的原因之一。     

小儿特发性高钙尿症13例报告

小儿特发性高钙尿症临床以血尿为主要表现,为小儿良性血尿的主要原因之一。根据钙负荷试验,可分为肾漏型和肠吸收亢进型,后者可分为Ⅰ型～Ⅲ型,其中Ⅱ、Ⅲ型在低钙饮食时,其尿钙<4mg/(kg·d),而Ⅲ型还伴有血磷下降,此外尚有报道为混合型者。现将我院自1992年～1999年2月明确为特发性高钙尿症13例报告如下。     

儿童特发性高钙尿症的临床代谢特征研究

目的　了解上海地区儿童特发性高钙尿症（ＩＨ）流行病学、临床分型、代谢特点及肾功能状况。方法　对上海地区６４０ 例６ ～１３ 岁正常儿童行尿钙／ 肌酐（Ｃａ／Ｃｒ） 比值、２４ 小时尿钙定量、钙负荷试验筛查ＩＨ;对筛查出的１６ 例ＩＨ儿童行血、尿生化及肾小球和肾小管功能检查。结果　尿Ｃａ／Ｃｒ 值呈偏态分布,与性别无关,但与年龄相关;９ 岁前和９ 岁后其第９５ 百分位值分别为０－２６ 和０－１７;ＩＨ 儿童检出率为２－５０ ％ ,以吸收型ＩＨ 为主;ＩＨ 与家族泌尿系结石的发生密切相关;１６ 例ＩＨ 儿童尿Ｎ－ 乙酰－ β－ Ｄ－ 氨基葡萄糖苷酶增高６ 例,尿转铁蛋白增高２例,尿微量白蛋白及ＩｇＧ 增高各１ 例,而尿常规均正常。结论　本地区儿童ＩＨ 并不少见,无症状性ＩＨ 可引起肾小管功能的损害。     

特发性高钙尿症家族发病情况及治疗随访

特发性高钙尿症（ＩＨ）是儿童期以血尿、泌尿系统结石为主要表现的少见病，其确切病因未明，某些患者具有明显的家族遗传性，国内报告甚少。我们对１９８６～１９９２年确诊的ＩＨ患者４８例进行了家系调查，以探讨遗传因素与ＩＨ发病的关系，并对治疗效果进行随访。对象...     

24例特发性高钙尿症远期随访

1986～1991年，我们对24例治疗2～5a的特发性高钙尿症（IH）患儿的血尿、泌尿系结石及体格发育、骨密度变化、肾功能检测等进行全面随访，分析如下。资料与方法一、病例选择：选择治疗随访＞2a的IH24例，男15例，女9例，均为血尿。血尿持续几天～3a／次，间歇3d～2mo。开始治疗年龄2～15a，平均6．8a。病程6mo～12a，平均8．6a。本组病人确诊前均测血钙、磷、碱性磷酸酶、钙磷乘积、二氧化碳结合力、肝肾功能，均正常。钙负荷试验结果，肠吸收型（aIH）14例，肾病型（rIH）10例，两型尿钙排出量无明显差异（P＞0．05）。二、治疗方法；…     

35例小儿特发性高钙尿症临床分析

特发性高钙尿症 (idiopathichypercalciuria ,IH )是一种与小儿单纯性血尿 ,尿路结石密切相关的疾病 ,临床表现多种多样。本文对 1996～ 2 0 0 1年我院儿科门诊和病房收治的表现为单纯性血尿、尿频、尿急、尿痛、遗尿、尿路结石等症状的患儿筛查随意尿尿钙和尿肌酐 ,计算尿钙 /     

小儿特发性高钙尿症与血尿：附85例报告

测定85例单纯性血尿患儿24小时尿钙定量,空腹尿Ca/Cr及钙负荷后尿Ga/Cr比值,24例为特发性高钙尿症(5.15±1.07mg/kg·24h),占单纯性血尿的28.2%,其中肾性16例,吸收性为8例。17例表现发作性肉眼血尿,其中12例伴镜下血尿:单纯镜下血尿7例。肾组织活检3例,钙染色沿小球囊壁及部分间质有钙盐沉积;另1例示系膜增生性肾炎。14例随访6～19月,尿钙测定13例恢复正常,1例仍高;血尿消失9例,仍有血尿5例。     

原发性高血压患儿红细胞膜三磷酸腺苷酶活性及血液黏度改变的意义

目的 探讨原发性高血压患儿红细胞膜Na 、K -ATP酶、Ca2 、Mg2 -ATP酶活性及血液黏度改变的意义.方法 对本院2004年11-12月坚持随访的50例原发性高血压患儿进行红细胞膜Na 、K -ATP酶、Ca2 、Mg2 -ATP酶活性(比色法)及血液黏度测定,并与30例健康儿童作对照,采用SPSS 12.0软件进行t检验及直线相关分析.结果 原发性高血压组患儿红细胞膜Na 、K -ATP酶、Ca2 、Mg2 -ATP酶活性[(6.12±1.30) μmolpi/(gHb·h)和(4.59±1.40) μmolpi/(gHb·h)]较健康对照组[(7.46±1.30) μmolpi/(gHb·h)和(5.81±1.20) μmolpi/(gHb·h)]显著降低(Pa＜0.01);血液黏度较健康对照组显著升高(Pa＜0.01).原发性高血压组Na 、K -ATP酶、Ca2 、Mg2 -ATP酶活性与血液黏度均呈负相关(P＜0.05,0.01).结论 Na 、K -ATP酶、Ca2 、Mg2 -ATP酶活性降低及血液黏度升高可能在儿童高血压发病机制中起重要作用.     

Plasma and Erythrocyte Concentrations of Mercaptopurine after Oral Administration in Children

Plasma and erythrocyte concentrations of 6-mercaptopurine (6-MP) were determined by gas chromatography-mass spectrometry. Eleven children (9 with acute lymphatic leukemia) were studied after oral intake of 6-MP doses ranging between 31 and 128 mg/m² body surface area. The concentrations of 6-MP in plasma were found to vary considerably between patients even after dose normalization to 75 mg/m². After dose normalization the mean peak plasma concentration was 0.68 μM (range 0.12-1.38) and the area under the plasma concentration-time curve (AUC) was 1.37 μM.h (range 0.12-3.04). The mean time taken to reach the peak concentration was 1.3 h (range 1-2), and the half-life of elimination was 1.8 h (range 0.6-2.5). No patient had detectable 6-MP concentrations 12 h after dose intake. The concentrations of 6-MP tended to be higher in erythrocytes than in plasma. The mean peak concentration in erythrocytes was 131% and the AUC 145% of that found in plasma. The mean half-life of elimination from erythrocytes was 2.0 h (range 0.7-2.8). These data indicate that 6-MP can pass through cell membranes rapidly to reach intracellular concentrations equal to or even higher than in plasma. In summary, marked interindividual differences in pharmacokinetics were found, probably due to highly variable bioavailability of oral 6-MP. Further studies are needed to determine whether measurements of plasma concentrations of 6-MP can be used to optimize maintenance treatment of childhood leukemia.     

Nephrocalcinosis, Hypercalciuria and Elevated Serum Levels of 1,25-Dihydroxyvitamin D in Children Possible Link to Vitamin D Toxicity

ABSTRACT. Ten children, age 1 1/2 to 14 years, had bilateral nephrocalcinosis and hypercalciuria, but normal serum calcium (Ca) and phosphate (P) concentrations. Patients with hypercalciuria were divided into absorptive (n=4) and renal (n=6) subgroups, and in the latter four patients had a primary Ca-leak and two had a P-leak. All the children had received intermittent high dose vitamin D prophylaxis during infancy. At the time of investigation all had normal serum levels of 25-hydroxyvitamin D, yet all but one had elevated values of 1, 25-(OH)₂D. Although the hypercalciuria was indistinguishable from the various known forms of idiopathic hypercalciuria, the previous clinical course and the pattern of bone mineral homeostasis suggest that both clinical features, namely nephrocalcinosis and hypercalciuria were related to vitamin D toxicity through various pathogenetic pathways.     

The Incidence of Erythrocyte Pi Antigen Retention in Children With Urinary Tract Infection

The erythrocyte P, antigen was observed in 91.7, 70.6, 56.7 and 62.5%, respectively, of children with upper urinary tract infection, symptomatic and asymptomatic urinary tract infections and cases responsive to initial therapy in which the abnormal urinary findings disappeared with the administration of antibiotics given despite the absence of bacteriuria. These values were significantly high (P<0.001, 0.001, 0.01, 0.001, respectively) compared with healthy controls (29.0%). In cases where initial antibiotic therapy was effective despite no significant bacteriuria, the presence of Gal cd– 4 Gal receptor in large quantity on the surface of the mucosa of the urinary tract was suggested, just as in the typical case of urinary tract infection with bacteria in the urine. No significant difference was observed in the incidence of P1 antigen in the peripheral blood of sick children according to the clinical symptoms and urinary findings in typical cases of urinary tract infection and cases responsive to initial antibiotic therapy.     

Relation of Serum and Erythrocyte Magnesium Levels to Blood Pressure and a Family History of Hypertension A Follow-up Study in Japanese Children, 12-14 Years Old

Abstract. Serum and erythrocyte magnesium concentrations (S-Mg, E-Mg) were measured in 122 junior high school students followed up for two years from 12 to 14 years of age, and the relationship to blood pressure and a family history of hypertension were investigated. The subjects who had high S-Mg and E-Mg levels at the first examination two years prior tended to show high levels after this follow-up. There were significant positive correlations between two intraindividual values of S-Mg and E-Mg. A similar tendency was found for blood pressure. Tracking phenomena were observed with these measures. The subjects who had high E-Mg levels at the first examination showed no blood pressure elevation during the two- year period. The subjects with a family history of hypertension [FH(+)] showed a higher degree of blood pressure rise during two years than those with no family history [FH(-)], with a significant difference in systolic blood pressure at the age of 14. E-Mg tended to be lower in the FH(+) group than in the FH(-) group with a significant difference in 14-year-old girls. These results suggest that a hereditary predisposition to hypertension is related to magnesium metabolism and that intracellular magnesium deficiency may influence blood pressure elevation in the FH(+) children.     

早期微量喂养对新生儿肺透明膜病临床康复的影响

目的 观察早期微量喂养在新生儿肺透明膜病(HMD)治疗中的效果.方法 收集2009年1月-2010年12月在本院NICU住院的HMD新生儿的临床喂养情况,分别统计实行早期微量喂养(早期微量喂养组)和常规喂养(常规喂养组)新生儿的一般资料,观察2组患儿达全量胃肠道营养时间、恢复出生体质量时间、胃肠功能紊乱发生率、血胆红素峰值、并发胆汁淤积比例等.采用t检验和x2检验进行统计学分析.结果 常规喂养组56例,早期微量喂养组48例,2组一般资料比较均无统计学差异.在达全量胃肠道营养时间、恢复出生体质量时间、血胆红素峰值、并发胆汁淤积等方面早期微量喂养组明显优于常规喂养组,差异均有统计学意义;2组患儿在住院天数及胃肠功能紊乱、吸人性肺炎、坏死性小肠结肠炎发生率方面差异均无统计学意义.结论 新生儿HMD实行早期微量喂养有利于患儿更早地过渡到全量胃肠道喂养,更好地恢复、增长体质量,减少胃肠外营养相关胆汁淤积的发生,无明显不良反应.     

铅中毒儿童血清锌、铜、铁、钙等元素含量变化

目的探讨铅中毒儿童体内锌、铜、铁、钙等元素含量变化。方法采用分层随机抽样方法进行抽样。在市区、农村、海岛共抽取540名儿童。对这些儿童进行血铅及其他元素(锌、铜、铁、钙等)含量测定,并分析这些元素含量在儿童铅中毒情况下有无变化。结果铅中毒组血锌水平(4.93±1.12)mg/L,明显低于非铅中毒组(t=2.282 P<0.05),其他元素含量两组无差异。多元逐步回归分析结果显示儿童体内血锌、血铜含量对儿童血铅水平有显著意义。结论补充适量锌制剂或通过膳食补锌可预防儿童铅中毒,减少铅对机体的毒性作用。     

高热惊厥患儿血钾、钠、氯、钙、糖变化的意义

目的探讨高热惊厥(FC)患儿血钾、钠、氯、钙、糖的变化及其临床意义。方法采用自动生化测定仪测定41例FC患儿(FC组)、30例发热患儿(发热组)和30例正常儿(正常组)的血钾、钠、氯、钙、糖水平,并进行比较分析。结果FC组血钾、钙水平与发热组之间无明显差异,但均明显低于正常儿(F=5.965,3.048 P<0.01,0.05);FC组血钠明显低于发热组和正常组;血糖则明显高于后两组(F=22.329,22.203 P均<0.001);发热组与正常组血钠、糖均无明显差异;血氯在3组间均无明显差异(F=0.867 P>0.05)。结论小儿FC存在低钾、低钠、低钙和高血糖。在常规治疗时应注意纠正电解质紊乱和高血糖等,以减少FC的复发、减轻脑组织和其他重要脏器的损害。