肾病患儿红细胞补体受体Ⅰ活性及血浆脂质过氧化物水平改变及临床意义

对２５例肾病综合征（ＮＳ）患儿的红细胞补体受体Ⅰ（ＣＲ－Ⅰ）活性及其调节因子、血浆脂质过氧化物（ＬＰＯ）水平进行检测。结果显示ＮＳ患儿红细胞Ｃ３ｂ受体花环率（ＲＢＣ－Ｃ３ｂＲＲ）、红细胞免疫复合物花环率（ＲＢＣ－ＩＣＲ）、红细胞免疫粘附肿瘤细胞能力（ＲＢＣ－ＣａＲ）、红细胞免疫促进因子（ＲＦＥＲ）均低于对照组（Ｐ＜００１），红细胞免疫抑制因子（ＲＦＩＲ）及血浆ＬＰＯ高于对照组（Ｐ＜００１）。提示ＮＳ患儿存在原发性红细胞免疫功能低下，并且与红细胞免疫调节功能紊乱及血浆ＬＰＯ水平有关     

新生儿感染性疾病红细胞免疫功能及SOD的变化

对５种临床常见的新生儿感染性疾病（败血症、肺炎、脐炎、臀红、鹅口疮）的红细胞Ｃ３ｂ受体花环率（ＲＢＣ－Ｃ３ｂＲＲ）、免疫复合物花环率（ＲＢＣ－ＩＣＲ）、血清中红细胞免疫粘附促进因子（ＲＦＥＲ）及抑制因子（ＲＦＩＲ）以及红细胞超氧化物歧化酶（ＳＯＤ）的活性进行了检测，结果表明红细胞免疫功能及ＳＯＤ的变化与感染的严重程度及疾病的不同阶段密切相关。     

围产期窒息和缺氧缺血性脑病红细胞免疫粘附功能及机体免疫状态的研究

目的探讨围产期窒息及缺氧缺血性脑病（ＨＩＥ）时机体免疫状态,特别是红细胞免疫状态的变化与ＨＩＥ发病、病理变化及病情演变的关系。方法应用红细胞酵母菌花环试验、间接免疫荧光流式细胞仪检测法、单向免疫扩散法对围产期窒息及ＨＩＥ患儿红细胞免疫粘附（ＲＣＩＡ）功能,血Ｔ细胞亚群、血清免疫球蛋白及补体进行检测。结果窒息及ＨＩＥ组红细胞Ｃ３ｂ受体花环（Ｅ－Ｃ３ｂＲＲ）较对照组明显降低（Ｐ＜００５和＜００１）,且随着病情加重降低更为明显。在病情恢复期Ｅ－Ｃ３ｂＲＲ较急性期明显增高（Ｐ＜００５）,但仍低于正常组（Ｐ＜００５）。与正常组比较,窒息及ＨＩＥ组ＣＤ３＋、ＣＤ４＋明显降低（Ｐ＜００５）,ＣＤ８＋、ＣＤ４＋／ＣＤ８＋无显著变化。ＨＩＥ组血清ＩｇＧ、ＩｇＡ、ＩｇＭ及补体Ｃ３水平明显降低,且Ｅ－Ｃ３ｂＲＲ与ＣＤ８＋呈负相关,与ＣＤ４＋／ＣＤ８＋及ＩｇＭ呈正相关。结论围产期窒息及ＨＩＥ时红细胞及白细胞免疫功能均低下,且两者之间存在显著的相关性,ＲＣＩＡ功能的变化参与了ＨＩＥ的病理生理过程,可间接反映ＨＩＥ时脑损伤的程度及病情演变过程。     

系膜增生性肾小球肾炎红细胞和白细胞免疫粘附功能及其相关性的研究

为从红细胞免疫角度探讨系膜增生性肾小球肾炎（ＭｓＰＧＮ）患儿的免疫发病机制，用郭峰法检测了３１例ＭｓＰＧＮ的红细胞和白细胞免疫粘附功能，并进行比较研究。结果提示：（１）作为检测红细胞免疫粘附功能的红细胞Ｃ３ｂ受体花环经（ＲＣＲ）和肿瘤红细胞花环率（ＴＲＲ）均较对照组明显降低，红细胞免疫复合物花环率（ＲＩＣＲ）差异不显著。（２）作为检测白细胞免疫功能的肿瘤料花环率（ＴＮＲ）和肿瘤淋巴细胞花环率（ＴＬ     

围生期新生儿红细胞免疫功能探讨

本文利用红细胞Ｃ３ｂ受体花环（ＲＢＣ－Ｃ３ｂＲＲ）与红细胞免疫复合物花环（ＲＢＣ－ＩＣＲ）试验对１００例围生期新生儿红细胞免疫功能进行测定。发现出生第１天围生期儿ＲＢＣ－Ｃ３ｂＲＲ明显增高，第２天开始下降并持续稳定至第７天，低出生体重儿ＲＢＣ－ＩＣＲ增高；分娩时情况不能使红细胞免疫功能发生改变。     

小儿某些血液病红细胞免疫粘附功能的检测及其意义

本文测定急性白血病（ＡＬ）、慢性再生障碍性贫血（ＣＡＡ）、特发性血小板减少性紫癜（ＩＴＰ）和过敏性紫癜（ＨＳＰ）等患儿红细胞免疫粘附功能，结果表明，ＡＬ、ＣＡＡ和ＩＴＰ红细胞Ｃ３ｂ受体花环率显著低于对照组（Ｐ〈０．０１），ＨＳＰ则明显高于对照组（Ｐ〈０．０１），各组红细胞免疫复合物花环率无明显变化。随着病情好转，Ｃ３ｂ受体花环率趋于正常。因此，红细胞免疫粘附功能检测对上述血液病的发病和病情观察有一     

川崎病患儿红细胞补体Ⅰ活性及IgE变化

本文检测了１７例川崎病（ＫＤ）患者红细胞补体Ⅰ（ＣＲ－Ⅰ）活性及其调节因子、血清ＩｇＥ水平,并对上述指标进行探讨和分析。对象和方法一、对象　１７例ＫＤ患儿,男１１例,女６例,年龄６ｍｏ～５ａ,符合川崎病诊断标准［１］。１５例健康儿作对照组,平均年龄３ａ５ｍｏ。二、方法　红细胞ＣＲ－Ⅰ活性及其调节因子测定　红细胞Ｃ３ｂ受体花环率（ＲＢＣ－Ｃ３ｂＲＲ）、红细胞免疫复合物花环率（ＲＢＣ－ＩＣＲ）、红细胞免疫促进因子（红细胞Ｃ３ｂ受体花环促进率,ＲＦＥＲ）、红细胞免疫抑制因子（红细胞Ｃ３ｂ受体花环抑制率…     

贫血患儿红细胞免疫功能观察

为了解贫血患儿的红细胞免疫功能,对 ８５例贫血患儿（缺铁性贫血 ２９例、急性白血病 ２６例、地中海贫血 １８例、再生障碍性贫血 １２例）进行了红细胞 Ｃ３ｂ受体花环率（ＲＢＣ－Ｃ３ｂＲＲ）和红细胞免疫复合物花环率（ＲＢＣ－ＩＣＲ）测定。结果显示所有贫血患儿ＲＢＣ－Ｃ３ｂＲＲ均显著低于对照组儿童（Ｐ＜０．０１）,缺铁性贫血和再生障碍性贫血患儿 ＲＢＣ－ＩＣＲ与对照组比较无明显差异（Ｐ＞０．０５）,急性白血病患儿 ＲＢＣ－ＩＣＲ高于对照组（Ｐ＜０．０５）,地中海贫血患儿 ＲＢＣ－ＩＣＲ则显著高于对照组（Ｐ＜０．０１）。贫血患儿容易感染可能与红细胞免疫功能低下有关。     

新生儿脐血红细胞免疫功能的检测

对新生儿脐血红细胞免疫功能进行检测，发现：（１）足月新生儿脐血组红细胞Ｃ３ｂ受体花环率和和红细胞免疫复合物花环率均明显高于正常儿童组和成人组，而后两者则无差异。（２）男女新生儿脐血组ＲＢＣ－Ｃ３ｂＲＲ和ＲＢＣ－ＩＣＲ两者的均无差异。     

癫痫患儿红细胞免疫功能的研究

目的 探讨红细胞免疫粘附功能与癫痫发作的关系。方法 采用酵母菌花环试验法对７０例癫痫患儿及３０例健康儿童（对照组）进行红细胞（Ｃ３ｂ（ＲＢＣ－Ｃ３ｂ）受体花环率及红细胞免疫复合物ＲＢＣ－ＩＣ）花环率检测,并进行比较。结果（１）癫痫组ＲＢＣ－Ｃ３ｂ受体花环率明显地姐;ＲＢＣ－ＩＣ花环率明显高于对照组;（２）使用抗癫痫药物组患儿ＲＢＣ－Ｃ３ｂ受体花环率明显高于未使用抗癫痫药物组,ＲＢＣ－ＩＣ花环率两组     

Contribution of sarcoplasmic reticulum Ca²+ release and Ca²+ transporters on sarcolemmal channels to Ca²+ transient in fetal mouse heart

Sarcoplasmic reticulum (SR) Ca release has been shown not to be the predominant mechanism responsible for excitation-contraction (E-C) coupling in fetal myocytes. However, most of the studies have been conducted either on primary cultures or acutely isolated cells, in which an apparent reduction of ryanodine receptor density have been reported. We aimed to elucidate the contribution of SR Ca release and Ca transporters on sarcolemmal channels to Ca transients in fetal mouse whole hearts. On embryonic day 13.5, ryanodine significantly reduced the amplitude of the Ca transient to 27.2 ± 4.4% of the control, and both nickel and SEA0400 significantly prolonged the time to peak from 84 ± 2 ms to 140 ± 5 ms and 129 ± 6 ms, respectively, whereas nifedipine did not alter it. Therefore, at early fetal stages, SR Ca release should be an important component of E-C coupling, and T-type Ca channel and reverse mode sodium-calcium exchanger (NCX)-mediated SR Ca release could be the predominant contributors. Using embryonic mouse cultured cardiomyocytes, we showed that both nifedipine and nickel inhibited the ability of NCX to extrude Ca from the cytosol. From these results, we propose a novel idea concerning E-C coupling in immature heart.     

卡维地洛对心力衰竭时兰尼碱受体的作用

目的探讨卡维地洛对心力衰竭（heart failure,HF）（简称心衰）时兰尼碱受体／钙释放通道（ryanodine receptor,RyR）的作用。方法采用腹主动脉缩窄术建立幼鼠心衰模型,术后6周随机分为2组：心衰组和卡维地洛治疗组。另设假手术对照组。卡维地洛灌胃给药,术后每日观察幼鼠的呼吸、皮毛颜色、活动量、体重等发育状况。4周后行高频超声检测。超速离心分离肌质网（SR）,荧光分光光度仪检测钙离子（Ca^2＋）的重吸收和渗漏。结果与假手术组（n=20）比较,HF组（n=20）幼鼠左室舒张末期内径（LVEDD）（P〈0．05）、左室收缩末期内径（LVESD）、室间隔舒张末期厚度（IVSTd）、室间隔收缩末期厚度（IVSTs）、左室后壁舒张末期厚度（LVPWTd）、左室后壁收缩末期厚度（LVPWTs）均明显升高（P〈0．01）,短轴缩短率（FS）、射血分数（EF）均明显降低（P〈0．01）;与HF组比较,卡维地洛治疗组（n=20）LVEDD（P〈0．05）、LVESD、IVSTd、IVSTs、LVPWTd、LVPWTs均明显降低（P〈0．01）,FS、EF均明显升高（P〈0．01）。若分别向含有三组SR的缓冲液中仅加入SRCa^2＋-ATP酶抑制剂-毒胡萝卜内酯（thapsigargin）,HF组较假手术组、卡维地洛治疗组有明显的Ca2＋渗漏（P〈0．01）;若毒胡萝卜内酯和FKBP抑制剂-FK506一起加入三组SR缓冲液中,假手术组、卡维地洛治疗组、HF组均出现明显的Ca2＋渗漏（P〈0．01）;与仅加入毒胡萝卜内酯比较,假手术组和卡维地洛治疗组的Ca2＋渗漏明显增多（P〈0．01）,HF组的Ca2＋渗漏未见明显增加（P〉0．05）。结论HF时心肌Ca2＋渗漏明显,卡维地洛通过恢复HF时心肌RyR FKBPl2．6的结合,从而抑制Ca2＋的渗漏。提高心脏功能并有效抑制心室重塑。     

A randomized, masked study of triiodothyronine plus thyroxine administration in preterm infants less than 28 weeks of gestational age: hormonal and clinical effects

A randomized, placebo-controlled, masked study was conducted of the responses of thyroid parameters, cortisol, and the cardiovascular system to a single dose of triiodothyronine (T(3)) 24 h after birth, followed by a daily dose of thyroxine (T(4)) during 6 wk to infants <28 wk gestational age. Thirty-one infants were assigned to three groups: 1) group A: T(3) 24 h after birth plus daily T(4) during 6 wk; 2) group B: placebo T(3) and T(4) during 6 wk; and 3) group C: placebo T(3) and placebo T(4). T(4), free T(4), T(3), free T(3), reverse T(3), thyroid-stimulating hormone, and cortisol were measured in cord blood and on days 1, 3, 7, 14, 21, 42, and 56. Data on pulse rate, blood pressure, and cumulative dose of inotropic agents were collected. T(3) (0.5 microg/kg) resulted in a plasma increase until day 3. Thereafter, plasma T(3) levels were comparable between the groups. T(4), free T(4), and reverse T(3) were increased in groups A and B during the period of T(4) administration. Thyroid-stimulating hormone suppression was of shorter duration in group A. T(3) and T(4) administration did not have any effect on cortisol levels. We did not find any effects of T(3) or of T(4) administration on the cardiovascular system. A single injection of T(3) (0.5 microg/kg) given 22-26 h after birth only leads to a 2-d increase of T(3) levels and does not have effects on the cardiovascular system. This study does not support the use of T(3) according to our regimen in preterm infants.     

Vitamin D metabolite levels in normal children

Vitamin D metabolite levels were measured in 174 normal children throughout the year. 25-hydroxyvitamin D3 (25(OH)D3) and 24,25-dihydroxyvitamin D3 (24,25(OH)2D3) levels showed a seasonal variation; both levels were higher in summer than in winter (p less than 0.001 for both). There was a fall in the 1,25-dihydroxyvitamin D (1,25(OH)2D) level in August and September (p less than 0.001), which coincided with a rise in mean 25(OH)D3 and 24,25(OH)2D3 levels. An inverse correlation was seen between 1,25(OH)2D and 24,25(OH)2D3 levels (r = -0.233; p less than 0.01). 25(OH)D3 levels increased with age only for winter values (less than 3 years, 11.70 +/- 3.98 ng/ml; 3-11 years, 18.38 +/- 1.65 ng/ml; greater than 11 years, 23.60 +/- 4.60 ng/ml) while 1,25(OH)2D and 24,25(OH)2D3 levels did not show an age-related difference. Intake of multivitamins had an interesting effect on 25(OH)D3 and 24,25(OH)2D3 levels in the winter but not in the summer; the endogenous metabolite levels were lower in the vitamin supplemented children [25(OH)D3: 23.05 +/- 7.35 versus 15.77 +/- 5.51 ng/ml, p less than 0.001; 24,25(OH)2D3: 2.30 +/- 1.11 versus 1.66 +/- 0.88 ng/ml, p less than 0.05]. Children studied in the winter who were not receiving supplemental vitamins were older than those who did receive the vitamins (7.26 +/- 2.64 versus 5.42 +/- 3.17 years; p less than 0.01). Sixteen of the children had both winter and summer measurements. Their 25(OH)D3 and 24,25(OH)2D3 levels showed the same seasonal variation as the overall group data, while their 1,25(OH)2D levels showed no consistent pattern.(ABSTRACT TRUNCATED AT 250 WORDS)     

Infantile sialic acid storage disease: the fate of biosynthetically labeled N-acetyl-(3H)-neuraminic acid in cultured human fibroblasts

N-acetyl-(3H)-mannosamine[(3H)-ManNAc] was used as a precursor for the metabolic labeling of N-acetyl-(3H)-neuraminic acid [(3H)-NANA] in cultured fibroblasts of a patient with infantile sialic acid storage disease (ISSD). The metabolic fate of free and bound (3H)-NANA, isolated by high-performance liquid chromatography, was followed under pulse-chase labeling conditions. Nonsaturable accumulation of free (3H)-NANA was observed in ISSD, while the metabolic flux from (3H)-ManNAc to NANA-glycoconjugates was unaffected. Accumulated free (3H)-NANA could not effectively be chased from ISSD cells although N-acetyl-(3H)-hexosamines [(3H)-HexNAc] were appearing in the chase medium. These metabolites could arise from (3H)-NANA bound to glycoconjugates which were cleaved at normal rates in ISSD. The finding that free (3H)-NANA was markedly increased relative to its major products (3H)-HexNAc is suggestive for an impaired degradation and reutilization of (3H)-NANA due to trapping in a metabolically unaccessible pool. In titration experiments with digitonin a clear-cut increase in the latency of labeled NANA relative to a cytoplasmic marker enzyme was evident in ISSD. The release of (3H)-NANA, however, followed closely the digitonin-induced release of the lysosomal enzyme beta-hexosaminidase. This is suggestive for a lysosomal location of the stored material.     

Maternal diet with diverse omega-6/omega-3 ratio affects the brain docosahexaenoic acid content of growing chickens

Eggs with diverse omega-6/omega-3 ratio produced by feeding breeder hens a wheat-soybean meal-basal diet containing 5% (wt/wt) sunflower oil (H(omega)6), 5% fish oil (H(omega)3) or 2.5% sunflower oil plus 2.5% fish oil (M(omega)3omega6) were incubated. The hatched chicks were fed a docosahexaenoic acid (DHA)-deficient diet up to 6 weeks of age. The fatty acid composition of chick brain was determined on 0, 2, 4 and 6 weeks and brain weight was taken on day 0 and day 42. The omega-6/omega-3 ratios were 37.12, 4.21 and 0.98 for the maternal diet; 28.36, 2.83 and 0.89 for the egg yolk; 1.94, 0.48 and 0.18 for hatched chick brain (p < 0.05). At 2 weeks of age, the omega-6/omega-3 ratios were 1.88, 0.81 and 0.60 for chicks hatched from hens fed H(omega)6, M(omega)3omega6 and H(omega)3 diets, respectively. The brain DHA contents at 0 and 2 weeks of age were Homega3 > M(omega)3omega6 < H(omega)6 (p < 0.05) and at 4 and 6 weeks of age H(omega)3 = M(omega)3omega6 > H(omega)6. Dietary C18:3omega3 in the starter and finisher diet did not increase brain DHA (p > 0.05). The significant increase in the content of C22:5omega3 at 6 weeks of age in group 1 birds with a concomitant reduction in DHA suggests a weak delta-4 desaturation but an effective delta-6 and delta-5 desaturation similar to human infants. Considering the role of DHA in early brain development and growth, the maternal supply of DHA during growth might be of importance when fed a DHA-deficient neonatal diet.     

An influenza Epidemic among Institutionalized Children at the Time of Replacement from A(H3N2)to A(H1N1): Observation of 32 Cases Including 3 Cases of Probable Simultaneous Infection with A(H1N1) and A(H3N2)

An influenza epidemic occurred in Fukuoka, southern part of Japan in January, 1978 in which both influenza virus A(H1N1) and A (H3N2) were isolated. Thirty-two institutionalized children with influenza were studied at the time of this shift from A (H3N2) to A (H1N1). Fourteen virus strains were isolated. Thirteen strains belonged to influenza virus A (H1N1) (A/USSR/92/77-like strain) and one a mixed strain of A (H1N1) and A (H3N2) (A/Texas/77-like strain). The hemagglutination inhibition (H1)tests of paired sera indicated that of the 32 Children, 27 Showed a significant increasein HI antibody titers for influenza virus A (H1N1). 2 for A (H3N2) and the remaining 3, including the case from which the mixed virus strain was isolated, for both A (H1N1) and A (H3N2). These offers 3 cases were thought to be probably infected simulataneously with both influenza virus A (H1N1) and A (H3N2). Clinical manifestations due to influenza virus A (H1N1) were moderate and the 3 cases of probable simultaneous infection with both influenza virus A (H1N1) and A (H3N2) did not show two clinical episodes but rather a single episode with clinical manifestations similar to the cases with single virus infection.     

FMS样酪氨酸激酶3靶向RNA干扰对急性单核细胞白血病细胞株THP-1增殖和凋亡的影响

目的探讨短发夹状干扰RNA（shRNA）诱导FMS样酪氨酸激酶3（FLT3）的靶向抑制对急性单核细胞白血病（AMOL）细胞株THP-1增殖、凋亡的影响。方法设计并体外转录合成FLT3靶向shRNA（FLT3-shRNA）,转染THP-1细胞。以半定量逆转录PCR（RT-PCR）、流式细胞法（FCM）检测细胞FLT3在mRNA、蛋白水平表达,细胞计数试剂8（CCK-8）检测细胞增殖活力,FCM法检测细胞周期,DNA梯度条带（DNA Ladder）和Annexin V—FITC染色法分析细胞凋亡。结果合成的FLT3-shRNA 15nmol／L转染48h对FLT3 mRNA和蛋白的抑制率分别达（72．95±2．07）％、（65．39±5．57）％。shRNA干扰后细胞增殖活力受到抑制,48h抑制率达（36．66±3．67）％,72h达（35．56±0．73）％。转染48h细胞周期出现G0／G1期到S期的阻滞,FLT3-shRNA组G0／G1期的细胞百分比（65．71±4．47）％明显高于对照组,S期（25．11±2．70）％低于对照组。FLT3-shRNA作用48h有明显的凋亡条带出现,Annexin V-FITC检测细胞的早期凋亡率（18．59±2．07）％明显高于对照组。结论由shRNA诱导的FLT3靶向干扰可有效的抑制THP-1细胞增殖、诱导凋亡,显示其在儿童AMOL治疗中具有潜在的价值。     

Prenatal trisomy 21 screening using the Lens culinaris agglutinin-reactive alpha-fetoprotein ratio

For the purpose of improving the clinical efficacy of alpha-fetoprotein (AFP)-L3% in prenatal screening for trisomy 21, we calculated the multiple of the median (MoM) of AFP-L3% (L3 MoM) and the ratio of L3 MoM to AFP MoM (L3 MoM/AFP MoM) in maternal serum. Maternal serum samples from 1822 women (maternal age 37.3 +/- 3.8 years, and weeks of gestation 16.0 +/- 1.0; mean +/- SD) with unaffected pregnancies and 28 women (37.6 +/- 4.6 years, 16.6 +/- 3.1) pregnant with of trisomy 21 fetuses were obtained. The AFP concentration and AFP-L3% in maternal serum were measured using a liquid-phase binding assay. The areas under the receiver operating characteristic curves (AUCs) of AFP MoM, AFP-L3%, L3 MoM, and L3 MoM/AFP MoM were 0.750, 0.868, 0.949 and 0.946, respectively. The AUCs of L3 MoM and L3 MoM/AFP MoM were significantly higher than AFP-L3% (P < 0.05) and AFP MoM (P < 0.0005). However, no statistical difference was observed between the AUCs of L3 MoM and L3 MoM/AFP MoM. In conclusion, the L3 MoM should be an effective replacement for AFP-L3% in prenatal trisomy 21 screening.     

Nephrogenic diabetes insipidus,cystinosis, and vitamin D.

We describe a patient with early diagnosed cystinosis who presented with nephrogenic diabetes insipidus in addition to proximal tubular dysfunction. Another feature in this patient was abnormally low serum concentration of 24,25 dihydroxy vitamin D3 (24,25(OH)2D3) with normal 25 hydroxy vitamin D3 (25(OH)D3) and relatively low 1,25 dihydroxy vitamin D3 (1,25(OH)2D3).