卵巢幼年型颗粒细胞瘤四例临床分析

目的 探讨卵巢幼年型颗粒细胞瘤的诊断,治疗和预后。方法 回顾性分析自1983年至2002年间北京协和医院收治的4例卵巢幼年型颗粒细胞瘤的临床资料。结果 卵巢幼年型颗粒细胞瘤的发生率极低,主要发生在青少年及儿童,临床表现为盆腔实性包块伴胸腹水,4例患者雌二醇水平均在正常范围,手术病理分期为Ⅰ期。确诊主要依据病理检查,所有患者接受手术 化学药物治疗（化疗）。其中,2例呈高核分裂相的患者病情很快恶化,分别于发病后10和14个月死亡;2例低分裂相的患者现无瘤生存分别为32和25个月。结论 卵巢幼年型颗粒细胞瘤的诊断主要依据病理检查,核分裂相高者预后不良,治疗以手术为主,辅以化疗可能改善预后。     

A fetus with trisomy 9p and trisomy 10p originating from unbalanced segregation of a maternal complex chromosome rearrangement t(4;10;9)

Complex chromosome rearrangements are only rarely seen in constitutional karyotypes. A case of prenatally detected trisomy 9p with trisomy 10p originating from adjacent segregation of a maternal complex chromosome rearrangement is reported. Ultrasound examination at 18 weeks of gestation showed cleft lip palate, club feet, structural anomalies of the cerebellum and cystic kidneys. Cytogenetic analysis of amnion cells revealed a female fetus with 47,XX,+der(9). FISH analyses together with parental karyotyping demonstrated the fetal additional chromosome to originate from malsegregation of a maternal complex chromosomal rearrangement. The mother is carrier of a balanced translocation t(4;10;9) (q12; p11;q13). Postmortem examination of the fetus showed nose anomalies, cleft lip palate, low set ears, club feet, lung anomalies, cystic kidney and aplasia of the uterus. Reporting of such rare cases is important in order to enable this information to be used for genetic counselling in similar situations.     

Effects of hedgehog proteins on tissue engineering of cartilage in vitro

The effects of three derivatives of the N-terminal signaling domain of hedgehog proteins on cartilage engineered in vitro were investigated, with specific focus on the ability to increase tissue growth rate and concentrations of major extracellular matrix components, that is, glycosaminoglycans (GAG) and collagen, and on the effects on morphological appearance of the tissue. Bovine articular chondrocytes were cultured on biodegradable polyglycolic acid (PGA) scaffolds with or without the addition of dipalmitoylated sonic hedgehog (dp-shh), dipalmitoylated indian hedgehog (dp-ihh), or sonic hedgehog dimer (shh-dimer) to medium with either 1% or 10% fetal bovine serum (FBS). All three hedgehog proteins dose-dependently increased construct weights (by up to 1.95-fold, dp-shh at 1,000 ng/mL) and the fraction of GAG over 4 weeks (by up to 2.7-fold, dp-shh at 1,000 ng/mL), as compared to control constructs. Dp-shh and dp-ihh elicited similar responses; a 10-fold higher concentration of nonacylated shh-dimer was necessary to reach comparable results. Positive hedgehog effects were more pronounced in medium containing 1% FBS than in medium containing 10% FBS; however, at either FBS concentration, cartilaginous tissues grown in the presence of hedgehog proteins appeared morphologically more mature. Hedgehog derivatives thus appear as promising candidates to improve the development and composition of engineered cartilage.     

Tissue oxygen reactivity and cerebral autoregulation after severe traumatic brain injury

OBJECTIVE: To study the relationship between arterial blood pressure, intracranial pressure, directly measured brain tissue oxygenation (PtiO2), and middle cerebral artery blood flow velocity in severely head-injured patients. DESIGN: Prospective study. SETTING: Neurosurgical intensive care unit.PATIENTS A total of 14 patients with severe head injury. INTERVENTIONS: Pharmacologic blood pressure manipulations using norepinephrine. MEASUREMENTS AND MAIN RESULTS: We assessed the magnitude of PtiO2 related to changes in cerebral perfusion pressure in 12 of the patients. We calculated in all the static rate of regulation, which is an index to describe the change of cerebrovascular resistance, using cerebral artery blood flow velocity in relation to changing cerebral perfusion pressure. Finally, we calculated the rate of change in PtiO2, which quantifies the percentage of change in PtiO2 divided by the percentage of change in cerebral perfusion pressure. It is a new marker for cerebral tissue oxygen regulation based on direct measurement of PtiO2. There was a plateau phase for the cerebral perfusion pressure-PtiO2 relation that was similar to the autoregulatory plateau seen in the relationship between cerebral perfusion pressure and cerebral artery blood flow velocity. The rate of change in PtiO2 demonstrated a significant correlation with the static rate of regulation (R = -.61, <.05). A decrease in intracranial pressure when arterial blood pressure increased from 70 to 90 mm Hg was strongly correlated with static rate of regulation (R =.79, <.001). CONCLUSIONS Cerebral tissue PO2 demonstrates a plateau phase similar to what is known about cerebral blood flow velocity, which suggests a close link between cerebral blood flow and oxygenation. Static cerebral autoregulation is significantly correlated with cerebral tissue oxygen reactivity.     

Restrictive Interatrial Communication with Protein-Losing Enteropathy and Coagulopathy in Hypoplastic Left Heart Syndrome After Norwood Palliation

The Norwood procedure is one option for neonates born with hypoplastic left heart syndrome. We describe a case of an infant with hypoplastic left heart syndrome, palliatively repaired with the Norwood procedure. The infant developed restriction of the interatrial communication, despite atrioseptectomy at the first stage of palliation. Consequently, a protein-losing enteropathy with severe coagulopathy developed which resolved after a repeat atrioseptectomy.     

Superoxide mediates the actions of angiotensin II in the central nervous system

Angiotensin II (Ang II) has profound effects in the central nervous system (CNS), including promotion of thirst, regulation of vasopressin secretion, and modulation of sympathetic outflow. Despite its importance in cardiovascular and volume homeostasis, angiotensinergic mechanisms are incompletely understood in the CNS. Recently, a novel signaling mechanism for Ang II involving reactive oxygen species (ROS) has been identified in a variety of peripheral tissues, but the involvement of ROS as second messengers in Ang II-mediated signaling in the CNS has not been reported. The hypothesis that superoxide is a key mediator of the actions of Ang II in the CNS was tested in mice using adenoviral vector-mediated expression of superoxide dismutase (AdSOD). Changes in blood pressure, heart rate, and drinking elicited by injection of Ang II in the CNS were abolished by prior treatment with AdSOD in the brain, whereas the cardiovascular responses to carbachol, another central vasopressor agent, were unaffected. In addition, Ang II stimulated superoxide generation in primary CNS cell cultures, and this was prevented by the Ang II receptor (Ang II type 1 subtype) antagonist losartan or AdSOD. These results identify a novel signaling mechanism mediating the actions of Ang II in the CNS. Dysregulation of this signaling cascade may be important in hypertension and heart failure triggered by Ang II acting in the CNS.