Mucopolysaccharidosis VII as cause of fetal hydrops in early pregnancy

We report on fetal hydrops presenting at 18 weeks of gestation and diagnosed as β-glucuronidase deficiency. The parents were first cousins and there were 2 previous similar fetal deaths. β-Glucuronidase was absent in cultured fetal fibroblasts and lymphoblasts but was normal in the tested relatives. The activities of other lysosomal enzymes were normal. © 1992 Wiley-Liss, Inc.     

Permissive Hypercapnia and Intravascular Oxygenator in the Treatment of Patients with ARDS

Abstract: This open clinical study was aimed at testing the hypothesis that an intravascular oxygenator (IVOX) may help to perform permissive hypoventilation in 10 patients with severe ARDS. After initial evaluation, we tried to reduce ventilator settings before and after IVOX implantation. Before IVOX, poor clinical tolerance and worsening oxygenation did not allow for a significant decrease in ventilator settings. With IVOX, peak inspiratory pressure (PIP) was reduced from 47 to 39 cm H₂O (p = 0. 005) and minute ventilation from 13 ± 3. 5 to 11 ± 3 L/min. CO₂ removal by IVOX allowed a significant decrease in Paco₂ from 66 ± 15 to 59 ± 13 mm Hg. Improvement of oxygenation with IVOX was not signify cant. Furthermore, interruption of oxygen flow through IVOX did not change oxygenation variables. Tolerance of the IVOX device was good, but insertion of the device was followed by a significant decrease in both cardiac index and pulmonary wedge pressure. In conclusion, IVOX improves tolerance of hypoventilation by limiting hypercapnia in ARDS patients. These preliminary results must be confirmed by a randomized controlled study     

Ontogenetic Development of 5-HT1D Receptors in Human Brain: An Autoradiographic Study

The pattern of pre- and postnatal appearance of 5-HT_1D receptors throughout the different areas of the human brain was studied by quantitative in vitro autoradiography, using [¹²⁵I]GTI (serotonin O -carboxymethyl-glycyl-[¹²⁵I]tyrosinamide) as a ligand. The anatomical distribution of 5-HT_1D receptors in neonatal, infant and children's brain was in good agreement with that observed in the adult, the basal ganglia and substantia nigra being the most intensely labelled areas. The development of these receptors throughout the human brain was mainly postnatal: low densities of [¹²⁵I]GTI binding sites were observed at the fetal/neonatal stage in most regions analyzed, in contrast with the high levels of labelling found in infant and children's brains. Indeed, in a number of regions, including the globus pallidus, substantia nigra and visual cortex, a peak of overexpression of 5-HT_1D receptors was observed in the first decade of life. Such overexpression could support a regulatory role for 5-HT_1D receptors in advanced periods of the CNS developmental process. Our results also indicate that the administration of drugs acting on 5-HT_1D receptors during the early postnatal period of life could result in modifications of their properties, as these receptors are already functional in this period.     

压力调节容量控制通气对肺损伤犬血流动力学、呼吸力学和呼吸功能的影响

目的 探讨压力调节容量控制(PRVC)通气模式对健康及急性肺损伤(ALI)模型犬的血流动力学、呼吸力学和呼吸功能的影响。方法 PRVC和间歇正压通气(IPPV)两种通气模式在呼气末压为0、0．5、1．0和1．5kPa时，分别测定健康犬及油酸诱发ALI犬的血流动力学、呼吸力学及血气参数。结果 健康犬PRVC较IPPV模式下除了在PEEP为0．5kPa时，气道峰压(PIP)、肺动态顺应性(Cdyn)和肺血管阻力(PVR)的影响差异有显著性，对血流动力学、呼吸力学及呼吸功能的影响差异均无显著性。ALI犬PRVC较IPPV模式下各PEEP水平的PIP、平均气道压(mPaw)、Cdyn、动脉血氧分压[pa(O2)]和氧耗(VO2)的影响均有显著意义，但两种通气模式对血流动力学的影响差异无显著性。结论 与IPPV相比，PRVC通气模式能降低PIP，增加Cdyn和提高pa(O2)，两模式对血流动力学的影响差异无显著性，PRVC更适用于ALI／ARDS的治疗。     

Restricted field of view magnetic resonance imaging of a dynamic time series.

A restricted field of view (rFOV) approach for imaging a dynamic time series of volumes of limited spatial extent within a larger subject is described. The shorter readout with rFOV-MRI can be exploited to either limit image artifacts or increase spatial resolution. To accomplish rFOV imaging of a multislice volume for a dynamic series, an outer volume suppression (OVS) preparation that saturates signal external to a cylinder through the subject is followed by slice-selective excitation and a spiral readout. The pass- and stopband efficiencies of the OVS in an agar gel phantom were 97% (+/-1.5%) and 3% (+/-1%), respectively. Profiles of the temporal signal-to-noise ratio (SNR) were measured in a phantom and an adult brain. The rFOV sequence reduced distortions from off-resonance signal and T2*-induced blurring compared to a conventional sequence. Sequence utility is demonstrated for high-resolution rFOV functional MRI (fMRI) in the visual cortex. The rFOV sequence may prove to be useful for other multislice dynamic and high-resolution imaging applications.     

氯喹对全肝缺血再灌注大鼠肺损伤的保护作用

目的 探讨氯喹对全肝缺血再灌注大鼠急性肺损伤（Au）的保护作用及其机制。方法 健康SD大鼠90只，雌雄不拘，体重300～350g，随机分为3组：假手术组（A组）、全肝缺血再灌注组（B组）、全肝缺血再灌注＋氯喹组（C组），每组30只。阻断肝门及肝上、肝下下腔静脉20min时开放血流，建立大鼠全肝缺血再灌注模型。C组于缺血前即刻经股静脉注射氯喹10mg／kg，A、B组给予等量生理盐水。于缺血20min、再灌注4h时每组分别处死10只大鼠，抽取门静脉血，测定血浆D-乳酸、内毒素（ETX）、肿瘤坏死因子-a（TNF-a）浓度，另外10只用于观察再灌注48h时大鼠生存率，并在电镜下观察肺组织超微结构的改变。结果 缺血再灌注可导致大鼠缺血期和再灌注期门静脉血D-乳酸、ETX、TNF-a浓度升高，大鼠生存率降低，肺组织超微结构严重受损，氯喹可减弱全肝缺血再灌注导致的上述改变。结论 氯喹对全肝缺血再灌注大鼠ALI有一定的保护作用，通过抑制磷脂酶A2激活，降低肠粘膜屏障通透性升高，降低肠黏膜内毒素移位。     

Blastomycosis in solid organ transplant recipients

BACKGROUND: Blastomyces dermatitidis, the etiologic agent of blastomycosis, causes severe disease and substantial mortality in those immunocompromised by acquired immunodeficiency syndrome or malignancy. In solid organ transplant recipients, the epidemiology, clinical features, and outcomes have not been fully described. METHODS: We conducted a retrospective case-series at the University of Wisconsin Hospital and Clinics. Case patients were solid organ transplant recipients with blastomycosis. RESULTS: From 1986 to 2004, we identified 11 cases of post-transplant blastomycosis with 64% occurring between 2000 and 2004. Onset of infection occurred a median of 26 months post transplantation with near equal distribution before and after the first year of transplantation. Rejection did not precede any case of post-transplant blastomycosis. Opportunistic co-infections were common, occurring in 36% of patients. Pneumonia was the most common clinical presentation and was frequently complicated by acute respiratory distress syndrome (ARDS). Extrapulmonary disease predominantly involved the skin and spared the central nervous system. The overall mortality rate was 36%; however, this increased to 67% in those with ARDS. None of the surviving patients relapsed or received routine secondary antifungal prophylaxis. CONCLUSION: Blastomycosis is an uncommon infection following solid organ transplantation that is frequently complicated by ARDS, dissemination, and opportunistic co-infection. After cure, post-transplant blastomycosis may not require lifelong antifungal suppression.     

山莨菪碱和无创面罩正压通气治疗急性呼吸窘迫综合征的临床观察

目的比较山莨菪碱联合无创正压面罩通气（654-2＋NIPPV）和传统机械通气（CMV）治疗急性呼吸窘迫综合征（ARDS）的临床效果，评估山莨菪碱联合无创正压通气在ARDS治疗中的作用。方法将各种原因所致ARDS患者42例随机分为654-2＋NIPPV组（21例）和CMV组（21例），在给予病因治疗同时分别实施654-2＋NIPPV和CMV。观察分析两组患者在治疗过程中动脉血气变化、并发症的发生率及治疗效果。结果654-2＋NIPPV组有8例（38．1％）治疗失败转为气管插管实行CMV，其中6例（28．6％）死亡。CMV组死亡7例（33．3％），两组病死率无显著差异（P〉0.05）。两组治疗有效的患者在分别接受654-2＋NIPPV和CMV治疗后1h和6h动脉血气有相似的显著改善。654-2＋NIPPV组患者机械通气时间和住院时间短于CMV组（P〈0.05）。654-2＋NIPPV组的并发症发生率低于CMV组（P〈0.05）。结论在经过选择的ARDS患者中，应用654-2＋NIPPV治疗的临床效果与CMV相似。实施654-2＋NIPPV可缩短机械通气和住院时间，减少并发症。654-2＋NIPPV可作为经过选择的ARDS患者首选的通气支持治疗手段。     

Pulmonary effects of closure of patent ductus arteriosus in premature infants with severe respiratory distress syndrome

The pulmonary effects of closure of a patent ductus arteriosus (PDA) were investigated in 11 premature infants with severe respiratory distress syndrome. Successful closure of a PDA did not improve the short-term severity of pulmonary disease (24 and 48 h after treatment) as judged by the arterial/alveolar oxygen tension ratio or the amount of ventilatory support. Also, pulmonary mechanics measured 24 h before, 24 and 48 h after treatment, were not statistically different.Conclusion Infants with severe respiratory disease requiring high ventilation pressure and high oxygen concentration, where structural changes in the lungs are already established, will rarely show short-term improvement in pulmonary disease as a result of closure of a PDA. The overall clinical condition may, however, improve as a result of reduced cardiovascular strain. Earlier treatment of a PDA could reduce the ventilation period and the possible risk of developing chronic lung disease.     

冷冻后人胚卵巢细胞团对hMG作用的内分泌与形态学反应

妊娠２０～２４周人胚胎卵巢经改进的超速冷冻仍具有内分泌功能。这种冷冻后的胚胎卵巢细胞团体外培养时，培养无数与细胞团的Ｅ２分泌能力呈负相关，形态学检查与此结果相吻合。冷冻后人胚胎卵巢的细胞团体外培养时培养位中人绝经期促性腺激素（ｈＭＧ）最适浓度为５０ＩＵ／Ｌ，培养的最适天数为１～３ｄ。这对人胚胎卵巢细胞团同种异体移植时受体促性腺激素水平内及培养时间的选择与设定有重要指导意义。