A Chinese adolescent girl with Fechtner-like syndrome

We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded to intravenous immunoglobulin and pulsed methylprednisolone. This case illustrates that life-threatening haemorrhage can occur in patients with Fechtner syndrome. Although there was no effective treatment reported in the literature, high dose steroid and immunoglobulin seemed to be useful in our patient. Our patient also had nephritic-nephrotic syndrome with renal insufficiency, which is unusual in adolescent female patients.     

Serum and Cerebrospinal Fluid Pharmacokinetics of Intravenous and Oral Lamivudine in Human Immunodeficiency Virus-Infected Children

We studied the pharmacokinetics of intravenously and orally administered lamivudine at six dose levels ranging from 0.5 to 10 mg/kg of body weight in 52 children with human immunodeficiency virus infection. A two-compartment model with first-order elimination from the central compartment was simultaneously fitted to the serum drug concentration-time data obtained after intravenous and oral administration. The maximal concentration at the end of the 1-h intravenous infusion and the area under the concentration-time curve after oral and intravenous administration increased proportionally with the dose. The mean clearance of lamivudine (± standard deviation) in the children was 0.53 ± 0.19 liter/kg/h (229 ± 77 ml/min/m² of body surface area), and the mean half-lives at the distribution and elimination phases were 0.23 ± 0.18 and 2.2 ± 2.1 h, respectively. Clearance was age dependent when normalized to body weight but age independent when normalized to body surface area. Lamivudine was rapidly absorbed after oral administration, and 66% ± 25% of the oral dose was absorbed. Serum lamivudine concentrations were maintained above 1 μM for ≥8 h of 24 h on the twice daily oral dosing schedule with doses of ≥2 mg/kg. The cerebrospinal fluid drug concentration measured 2 to 4 h after the dose was 12% (range, 0 to 46%) of the simultaneously measured serum drug concentration. A limited-sampling strategy was developed to estimate the area under the concentration-time curve for concentrations in serum at 2 and 6 h.     

额叶内侧面损伤的临床特点及救治

目的 总结17例额叶内侧面损伤的临床特点及救治经验。方法 对我科1999年7月至2003年4月收治的额叶内侧面损伤患者进行回顾性分析。结果 手术治疗1例，恢复良好，保守治疗16例，其中恢复良好12例，中残2例，死亡2例，全组死亡率11．77％。结论 额叶内侧面损伤一般意识障碍较轻，但易直接发生枕骨大孔疝，严密观察病情及动态CT检查，颅内压监测，不拘泥传统手术指征，及时抓住手术时机，可有效降低致残率及死亡率。     

Paired kidney analysis of tacrolimus and cyclosporine microemulsion-based therapy in Chinese cadaveric renal transplant recipients

Few studies used paired kidneys for comparison between tacrolimus and cyclosporine in renal transplantation. Most of the published data used whole blood trough levels for drug monitoring. However, the use of limited sampling strategy and abbreviated formula to estimate the 12-h area under concentration-time curve (AUC(0-12)) allowed better prediction of drug exposure. Sixty-six first cadaveric renal transplant recipients receiving paired kidneys were randomized to receive either tacrolimus-based (n = 33) or cyclosporine microemulsion (Neoral)-based therapies (n = 33). Abbreviated AUC(0-12) was used for drug monitoring and dose titration. Mean follow-up duration was 2.8 +/- 2 years. The patient and graft survival were comparable. Fewer incidence of acute rejection was observed in tacrolimus group (15% vs. 27.3%) though the difference was not significant (P = 0.23). The absolute value and the rate of decline of creatinine clearance were both significantly better in tacrolimus-treated patients. Prevalence of hypertension, post-transplant diabetes mellitus, infection, and malignancy were similar in both groups. Prevalence of hypercholesterolemia (11/33 vs. 4/33) and gum hypertrophy (6/33 vs. 1/33) was more common in cyclosporine-treated patients (P = 0.04 in both parameters). This was the first prospective, randomized study with paired kidney analysis showing the renal function was significantly better in tacrolimus-treated patients than in cyclosporine-treated patients.     

Pten基因敲除对过氧化物酶家族表达和活性氧水平的影响

目的:探讨Pten基因敲除后对过氧化物酶家族(Peroxiredoxins,Prdxs)水平和活性氧水平的影响.方法:采用Western印迹和化学/荧光发光分析法分别检测了在Pten / MEF和Pten-/-MEF细胞中PRDXs的表达和细胞内活性氧水平.结果:Western印迹结果显示,与Pten / MEF细胞相比,Pten-/-MEF细胞PRDX Ⅰ,Ⅱ,Ⅴ,Ⅵ蛋白水平下调,PRDX Ⅲ不变,PRDX Ⅳ上调.DCFH探针标记后流式结果显示Pten-/-MEF 细胞活性氧荧光值显著高于对照Pten / MEF细胞(P＜0.05).结论:Pten基因敲除引起数种PRDXs表达下调,细胞内活性氧水平增高.     

α-地中海贫血种植前基因诊断妊娠成功

【目的】探讨种植前基因诊断的临床应用。【方法】对 1例夫妇均为东南亚缺失型α 地中海贫血携带者 ,经超排取卵、卵母细胞单精子显微注射受精及胚胎细胞活检 ,吸取的单个卵裂球采用针对α SEA基因的 gap PCR引物和荧光探针用荧光定量PCR技术进行诊断 ,将诊断为不致病的 3个胚胎进行宫腔内移植。【结果】胚胎移植后 7周B超诊断单胎妊娠 ,18周羊膜腔穿刺产前诊断证实为不致病胎儿。【结论】应用成熟的显微操作技术进行胚胎细胞活检 ,活检的单个卵裂球用荧光定量PCR技术进行诊断 ,获得国内首例α 地中海贫血胚胎种植前基因诊断后妊娠成功。     

人胃癌组织中fibulin-5的表达及其临床意义

目的探讨fibulin-5在人胃癌组织中的表达及其临床意义。方法采用免疫组织化学染色结合图像分析法，对32例胃癌组织、癌旁组织及正常胃组织中fibulin-5的表达进行半定量分析。结果fibulin-5在胃癌组织中的表达强于癌旁组织和正常组织（P〈0．05），且表达强度与癌组织的分化程度有关，低分化癌组织的fibulin-5表达强（P〈O．05）。结论fibulin-5的表达可能与胃癌组织细胞的恶性转化及增殖有关。     

Preparation and identification of monoclonal antibodies against Penicillium marneffei]

OBJECTIVE: To prepare and identify monoclonal antibodies (mAbs) against Penicillium marneffei. METHODS: Recombinant mannoprotein1 (MP1) of Penicillium marneffei was used to immunize BALB/c mice, and anti-MP1 mAbs were obtained by means of hybridoma. Screening and identification of the mAbs were subsequently performed with indirect enzyme-linked immunosorbent assay and Western blotting, respectively. RESULTS: Four hybridomas producing antibodies against Penicillium marneffei were obtained, and the IgG isotypes of the 4 mAbs were identified as IgG1 with affinity constants (K) of 8.2x10(-9), 4.7x10(-9), 6.5x10(-9) and 2.7x10(-9), respectively. Western blotting demonstrated specific recognition of Aspergillus fumigatus MP1 by the obtained mAbs. CONCLUSION: The 4 hybridomas producing anti-MP1 mAbs with high specificity and affinity can be of significant value in the diagnosis of Penicilliosis marneffei infections.