Intravenous glycerol therapy should not be used in patients with unrecognized fructose-1,6-bisphosphatase deficiency

BACKGROUND: In Asian countries, glycerol solution that contains fructose (5%) is often used for management of brain edema. However, glycerol and fructose may cause severe hypoglycemia and metabolic acidosis in patients with fructose-1,6-bisphosphatase (FBPase) deficiency, even under stable conditions. The aim of the present study was to determine whether glycerol solution was used for brain edema during acute metabolic decompensation of hypoglycemia and metabolic acidosis in patients with unrecognized FBPase deficiency in Japan and to examine a long-term prognosis of the patients who had this kind of severe metabolic decompensation with or without glycerol therapy. METHODS: A retrospective study of 20 children with FBPase deficiency was conducted, based on their medical records. RESULTS: Six of the 20 children were given glycerol solution for the presence or possibility of brain edema during acute metabolic decompensation of hypoglycemia and metabolic acidosis; two of the six patients administered with glycerol were given dialysis. In four patients treated with glycerol alone without dialysis, two had no brain edema before glycerol administration but it developed later after the administration. These four patients treated with glycerol alone died or developed severe neurological complications. Fourteen patients who were not treated with glycerol solution had no brain edema and showed good prognosis. CONCLUSIONS: Glycerol solution, which contains fructose in Asian countries including Japan, should not be used as an osmotic agent for treatment of brain edema in patients who have hypoglycemia and retention-type metabolic acidosis, until FBPase deficiency is ruled out by measuring blood concentration of lactate.     

糖皮质激素受体--虚证相关蛋白之一?

以已有实验与临床研究为依据，提出糖皮质激素受体(GR)是虚证相关蛋白之一的理论假说。分析了进一步深入研究论证的途径：①改进糖皮质激素受体检测方法；②应用酵母双杂交检测技术，了解GRβ与GRα之间的相互作用，并分析这些作用特征与阴虚、阳虚之间的关系；②运用良好的前瞻性设计，对临床虚证患者进行大量、反复的检测观察与统计分析。指出对该假说的研究论证，很有可能在蛋白质(受体)水平为中医证的微观诊断提供一个符合中医基本理论，具有现代科学依据的检测指标，并有可能发现能上调GR含量的药物。     

1314例育龄妇女缺铁性贫血发病现状调查

目的 :研究育龄妇女缺铁性贫血 (IDA)的发病现状及营养状况与IDA的关系。方法 :检测青岛市城市及农村 1314例妊娠期和非妊娠期育龄妇女的外周血红蛋白浓度 (Hb)、平均红细胞体积 (MCV)、平均红细胞血红蛋白量 (MCH)、血清铁蛋白 (SF) ,并进行个人问卷调查 ,应用营养计算软件评价膳食营养。结果 :1314例育龄妇女IDA发病率为 2 0 .4 0 % ,其中非孕妇的发病率高于孕妇。农村育龄妇女的发病率明显高于城市。孕妇中孕≥ 2 8周组发病率最高 (4 0 % ) ,与其他孕周组相比差异有高度显著性 (P <0 .0 1)。营养评价结果显示非孕妇组热量、维生素A、E、B1、B2 、PP、C的缺乏率高于孕妇组(P <0 .0 0 1)。结论 :农村育龄妇女及孕 2 8周以上的孕妇为IDA高发人群。应加强对农村育龄妇女的膳食营养宣教及指导 ,对孕晚期的孕妇及时采取有针对性的干预措施。     

HBV-YMDD变异患者与中医虚实辨证关系初探

目的 :初步探讨服用拉米夫定后HBV -YMDD变异患者中医辨证虚证、实证之间的关系。方法 :对服用拉米夫定半年以上的乙型慢性病毒性肝炎患者采用联合基因集团上海博华基因技术有限公司出品的迈科锐乙型肝炎病毒突变检测基因芯片 ,进行HBV -YMDD变异检测。出现HBV -YMDD者 ,根据中医基础理论 ,将其分为实证、虚证 ,来探讨HBV -YMDD变异患者中医虚、实辨证的关系。结果 :HBV -YMDD变异患者中实证变异率(38/ 4 8)明显高于虚证 (10 / 4 8) ,具有统计学意义。结论 :HBV -YMDD变异与虚、实辨证之间具有相关性。     

PATHOLOGY OF LONG-CHAIN 3-HYDROXYACYL-COA DEHYDROGENASE DEFICIENCY CAUSED BY THE G1528C MUTATION

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency is a recently discovered disorder affecting the mitochondrial-oxidation of fatty acids. There have been few reports of the pathologic findings in-oxidation defects. We examined pathologic specimens from 16 patients with this disorder (11 patients were homozygous for the common mutation G1528C, 5 patients were siblings with a similar clinical presentation). Autopsies were performed on all 15 patients who died, and liver biopsy specimens were available from 8 patients. Hepatomegaly and steatosis of the liver, found in every patient, were often combined with fibrosis or cirrhosis. Cardiomegaly and accumulation of fat in the myocardium, renal tubules, and skeletal muscle were found in many patients. A detailed neuropathologic examination was performed on six patients, and brain specimens obtained at autopsy were examined in four others. In general, neuropathologic findings were mild and unspecific, but vacuolization was detected in the deep gray matter and in the cerebellum and brain stem nuclei of five patients. In one patient the vacuolization was prominent; in the other four it was milder and more focal. The vacuoles seemed to be either in the neuropil or associated with swollen hydropic cells. The uniform pattern of histopathologic changes facilitates the diagnostics in this severe disorder, allowing opportunities for therapy and prenatal diagnosis.     

复方蚁酒的抗炎补肾阳作用研究

复方蚁酒能明显抑制二甲苯、琼脂引起的小鼠耳、足肿胀、抑制蛋清引起的大鼠足肿胀和佐剂性关节炎，并能对抗氢化考的松引起的肾阳虚症，同时还具有增强免疫功能的作用。     

二子阳宝的药理实验研究

二子阳宝能明显提高去势雄性小鼠副性器官和提肛肌指数；明显增加性交配功能；并能增强肾阳虚小鼠的耐缺氧，抗疲劳能力。     

Cardiac tamponade due to systemic lupus erythematosus in a 7-year-old boy with selective IgG subclass deficiency

Systemic lupus erythematosus (SLE) was observed in a 7-year-old boy with IgG₂ and IgG₄ subclass deficiencies who had been treated with immunoglobulin (100–200 mg/kg/4 weeks) since 2 years of age. The mother and the half-brother displayed the same deficiency. Serum IgG mainly consisted of IgG₁ (98.9%) during the acute phase of SLE due to transient IgG₃ deficiency. While he had no common manifestations of SLE such as arthritis or nephropathy, he developed cardiac tamponade due to massive pericardial effusion. Conclusion The clinical features of SLE in the present case such as the development of cardiac tamponade and the absence of renal involvement may result from the markedly imbalanced IgG subclass distribution among auto-antibodies. Received: 20 May 1997 and in revised form: 7 October 1997 / Accepted: 21 October 1997     

成人生长激素缺乏症诊治进展（综述）

介绍成人生长激素缺乏症诊断与治疗方面的一些进展,强调动态试验对于诊断的作用及注意事项,与替代治疗相关的问题涉及剂量、疗程、对代谢的影响及治疗的安全性等.