Natural history of trisomy 18 and trisomy 13: II. Psychomotor development

Developmental data were abstracted from medical records on 50 trisomy 18 individuals ranging in age from 1 to 232 months and 12 trisomy 13 individuals ranging in age from 1 to 130 months. Data on the age when trisomy 18 and trisomy 13 children achieved developmental skills were collected from a larger group of 62 trisomy 18 individuals and 14 trisomy 13 individuals whose families filled out parent questionnaires. Developmental quotient (DQ), defined as developmental age divided by chronological age, averaged 0.18 for trisomy 18 and 0.25 for trisomy 13. There was a dramatic drop in DQ from infancy to later childhood. The highest DQs and the greatest variation in DQs were in the first 2–3 years of life. Developmental ages in 7 skill areas were significantly different, with daily living and receptive language having the highest values and motor and communication skills having the lowest. When chronological age was taken into account, there was no significant difference in DQs in the same 7 skill areas, although there was a trend that was similar to the pattern of differences with developmental age. Older children could use a walker, understand words and phrases, use a few words and/or signs, crawl, follow simple commands, recognize and interact with others, and play independently. Walking and some toileting skills were also reported for trisomy 13. Although individuals with trisomy 18 and trisomy 13 were clearly functioning in the severe to profound developmentally handicapped range, they did achieve some psychomotor maturation and always continued to learn. © 1994 Wiley-Liss, Inc.     

In vivo 13C saturation transfer effect of the lactate dehydrogenase reaction.

Lactate dehydrogenase (LDH, EC 1.1.1.27) catalyzes an exchange reaction between pyruvate and lactate. It is demonstrated here that this reaction is sufficiently fast to cause a significant magnetization (saturation) transfer effect when the 13C resonance of pyruvate is saturated by a continuous-wave (CW) RF pulse. Infusion of [2-(13)C]glucose was used to allow labeling of pyruvate C2 at 207.9 ppm to determine the pseudo first-order rate constant of the unidirectional lactate-->pyruvate flux in vivo. During systemic administration of GABAA receptor antagonist bicuculline, this pseudo first-order rate constant was determined to be 0.08+/-0.01 s-1 (mean+/-SD, N=4) in halothane-anesthetized adult rat brains. In 9L and C6 rat glioma models, the 13C saturation transfer effect of the LDH reaction was also detected in vivo. Our results demonstrate that the 13C magnetization transfer effect of the LDH reaction may be useful as a novel marker for utilizing noninvasive in vivo MRS to study many physiological and pathological conditions, such as cancer.     

骨髓间充质干细胞在丝素蛋白/骨形态发生蛋白中的生物学行为观察

目的 观察丝素蛋白与牛骨形态发生蛋白的载体系统与骨髓间充质干细胞的体外相容性.方法 将多孔丝素蛋白修剪成20×10×5 mm长方体状物,置入6孔板中;将粉状bBMP用PBS液溶解制成2mg/ml混悬液,0.5ml无菌移液管滴定于多孔丝素蛋白上(0.5毫升/个).骨髓间充质干细胞以107/ml接种到载体系统上,复合4小时后加入生长液继续体外培养,每1、4、8天时进行碱性磷酸酶、矿化结节染色观察以及扫描电镜和激光共聚焦显微镜观察;另外同时设立单纯丝素蛋白与细胞复合培养对照组进行观察.结果 倒置显微镜、扫描电镜和共聚焦显微镜下观察到细胞在两种材料上生长形态、活性正常,体外培养24小时时细胞已贴附材料上,呈匍匐状伸展,以后逐渐融合生长,8天时大量细胞成片状攀附在材料表面,并分泌有大量的网状细胞外基质,实验组可见结节状基质生成;碱性磷酸酶、矿化结节染色观察发现实验组细胞碱性磷酸酶和矿化结节染色阳性反应,对照组阴性.结论 丝素蛋白是一种良好细胞外基质材料,也是一种良好生长因子的缓释载体.     

短期用药对幽门螺杆菌检测结果的影响

目的：研究短期不规则用药对幽门螺杆菌(Hp)检测结果的影响。方法：136名慢性胃炎及消化性溃疡的住院患，根据病史分为服药组即Hp检查前一周内接受过铅剂、抑酸剂治疗的患，共四例。对照组即Hp检查前未接受过药物治疗的患考，共37例。Hp检查方法包括①血清学方法；②快速尿素酶试验(RUT)；③组织学方法；④13c—尿素呼气试验(13C—UBT)。结果：服药组RUT，组织学，13C—UBT方法Hp检出率分别为9．0％，21．2％，27．3％，均显低于对照组相应Hp检出率37．8％、40．5％、48．6％(P＜0．01，P＜0．05，P＜0．05)。两组血清学方法Hp IgG、IgM阳性率各为48．5％，51．5％和54．1％，54．1％(P＞0．05)。单独服用质子泵抑制剂组RUT，组织学方法Hp检出率4．0％，16．0％显低于对照组相应方法Hp检出率(P＜0．01，P＜0．05)。单独服用枸橼酸铋钾、H2受体拮抗剂患中各方法Hp检出率与对照组检出率无显性差异(P＞0．05)。单独服用枸橼酸铋钾、H2受体拮抗剂、质子泵抑制剂三组Hp感染率14．3％、15．0％、16．0％均显低于对照组Hp感染率46．0％(P＜0．05)。结论：Hp检查前短期使用铋剂、抑酸剂可导致Hp检出率下降，短期使用质子泵抑制剂可显降低RUT、组织学方法Hp检出率；各方法中快速尿素酶试验检出率最低。血清学方法检测Hp抗体不受药物影响。     

牦牛骨形态发生蛋白及其与陶瓷化异种骨复合修复兔桡骨缺损的实验研究

本实验采用yBMP和yBMP/CBB材料,修复1.5cm长的兔桡骨缺损。分yBMP和yBMP/CBB二个实验组。材料植入20天,缺损区均分别生成多量软骨及交织骨。40天,大量交织骨生成,其间出现骨髓组织。60天,板层骨增多。80天,板层骨进一步成熟,骨缺损修复。与之对照,空白组各期仅见两端形成交织骨。这表明:yBMP及yBMP/CBB对骨缺损的修复作用非常显著。     

Regeneration of periodontal ligament and cementum by BMP-applied tissue engineering

Previously, we demonstrated that the inductive properties of bone morphogenetic protein (BMP) highly depend on the nature of the carrier material used for implantation. In this paper, we show that administration of BMP incorporated in a fibrous collagen membrane can help to regenerate periodontal ligament and cementum both in cat canines and in monkey molars. The partially purified bovine BMP was combined with one or two layers of a fibrous collagen membrane. Although the single layer approach showed partial regeneration of periodontal defects, it also quite often led to ankylosis. The double layer technique in artificially prepared class III furcation defects in monkey molars gave favorable results. After 12 wk, not only the alveolar process but also the periodontal ligament and cementum had regenerated along the entire treated dentin surface. Collagen fibers were arranged more or less perpendicular to the surface of the new cementum. Ankylosis was not seen. It is concluded that the double-layer approach is superior to the single-layer technique in regenerating cementum.     

Bromine-76 and carbon-11 labelled NNC 13-8199, metabolically stable benzodiazepine receptor agonists as radioligands for positron emission tomography (PET)

NNC 13-8241 has recently been labelled with iodine-123 and developed as a metabolically stable benzodiazepine receptor ligand for single-photon emission computed tomography (SPECT) in monkeys and man. NNC 13-8199 is a bromo-analogue of NNC 13-8241. This partial agonist binds selectively and with subnanomolar affinity to the benzodiazepine receptors. We prepared ⁷⁶Br labelled NNC 13-8199 from the trimethyltin precursor by the chloramine-T method. Carbon-11 labelled NNC 13-8199 was synthesised by N-alkylation of the nitrogen of the amide group with [¹¹C]methyl iodide. Positron emission tomography (PET) examination with the two radioligands in monkeys demonstrated a high uptake of radioactivity in the occipital, temporal and frontal cortex. In the study with [⁷⁶Br]NNC 13-8199, the monkey brain uptake continued to increase until the time of displacement with flumazenil at 215 min after injection. For both radioligands the radioactivity in the cortical brain regions was markedly reduced after displacement with flumazenil. More than 98% of the radioactivity in monkey plasma represented unchanged radioligand 40 min after injection. The low degree of metabolism indicates that NNC 13-8199 is metabolically much more stable than hitherto developed PET radioligands for imaging of benzodiazepine receptors in the primate brain. [⁷⁶Br]NNC 13-8199 has potential as a radioligand in human PET studies using models where a slow metabolism is an advantage. Received 19 April and in revised form 10 June 1997     

高糖等因素对系膜细胞细胞周期调节负控蛋白P27的影响

目的探讨高糖不同浓度刺激或抑制剂对肾小球系膜细胞表达细胞周期负控蛋白P27的影响.方法不同浓度高糖(5.5 mmol/L和25mmol/L、内皮素-1(10-7mol/L)、白介素-13(10 ng/ml和100 ng/ml)作用于培养的系膜细胞.流式细胞仪检测系膜细胞P27表达水平.结果5.5mol/L浓度组在不同时间P27表达为5.10±0.94和3.84±0.81,较对照级明显降低(P＜0.001),而25 mmol/L浓度组在不同时间P27表达为26.82±3.15和30.88±3.68,较对照组明显升高.内皮素-1刺激14 h和18 h后P27的表达分别为14.76±1.49和12.18±1.30,与对照组比较有明显差异(P＜0.05,P＜0.05).IL-13 10 ng/ml浓度组和100 ng/ml浓度组P27表达为46.74±3.52和23.8±2.56,对对照组比较有明显差异(P＜0.001,P＜0.05),不同浓度组之间有显著差异(P＜0.01).结论细胞周期调节负控蛋白P27在调节系膜细胞增殖过程中起重要作用.     

Synthesis of JTT‐501 and its metabolite JTP‐20604 labelled with 13C

JTT‐501 specifically labelled with ¹³C was obtained via a four‐step synthesis at an isotopic enrichment level of 99% and in 14% overall chemical yield starting from 4‐hydroxy‐[ring‐U‐¹³C₆]benzaldehyde (3) . The hydrogenation of [¹³C₆]JTT‐501 over Pd/C gave [¹³C₆]JTP‐20604 in 90% chemical yield. Copyright © 2003 John Wiley & Sons, Ltd.     

Cerebral perfusion assessment by bolus tracking using hyperpolarized 13C.

Cerebral perfusion was assessed with 13C MRI in a rat model after intravenous injections of the 13C-labeled compound bis-1,1-(hydroxymethyl)-1-13C-cyclopropane-D8 in aqueous solutions hyperpolarized by dynamic nuclear polarization (DNP). Since the tracer acted as a direct signal source, several of the problems associated with techniques based on traditional dynamic susceptibility contrast (DSC) MRI contrast agents were avoided. Maps of cerebral blood flow (CBF), cerebral blood volume (CBV), and mean transit time (MTT) were calculated. The MTT was determined to be 2.8 +/- 0.8 sec. However, arterial partial-volume effects in the animal model prevented accurate absolute quantification of CBF and CBV. It was demonstrated that depolarization of the hyperpolarized 13C tracer via relaxation and the imaging sequence had little influence on CBF assessment when the time resolution of the imaging sequence was short compared to the MTT. However, CBV and MTT were increasingly underestimated as MTT or the depolarization rate increased if depolarization was not taken into account. With a modified bolus-tracking theory depolarization could be compensated for, assuming that the depolarization rate was known. Three separate compensation methods were investigated experimentally and by numerical simulations.