Urine glycosaminoglycans of two patients (full brother and sister )with Sanfilippo's syndrome were analysed by electrophoresis, enzymatic or chemical treatment and ion-exchange chromatography. Heparin sulfate is the main glycosaminoglycan accouting for 70% and 58% of urine glycosaminoglycans in both patients, whereas only 29% and 42% of urine glycosaminoglycans are cnondroitin sulfate. Tbe C/O ratio urine heparin sulfate of the patients is 2.32, indicating that the hexuronic acid component parts of the patient's heparin sulfate are much the same as general heparin sulfate. However, the analysis of glycosaminoglycans with DEAE-Sephadex A-25 chromatography and electrophoresis in 0.1 M HCL showed that urine heparin sulfate of the patients arc heterogeneous, and that it covers, at least, two molecular types with different amount of sulfate and charge density.

Biliary atresia(BA) is a serious digestive system disease caused by the obliteration of intrahepatic and extrahepatic biliary system of infancy.Without prompt treatment,BA can cause biliary cirrhosis,and ultimately cause liver failure,leading to death.The early diagnosis of BA is crucial to the early treatment.At present,liver biopsy is considered to be the gold standard of diagnosis of BA,but this method has some shortcomings such as invasive,repeated sampling and subjectivity.In contrast,ultrasonography (US),magnetic resonance imaging (MRI) and hepatobiliary scintigraphy(HBS) gradually play important roles in clinical diagnoses of BA and become the hotspot of BA diagnoses.This paper focuses on reviewing the clinical applications of BA imaging diagnoses and the latest research progress.

The author believed that pathogenesis of IGT is yin asthenia,deficiency of both vital energy and yin,obstruct of phlegm and blood stasis,which result from innate deficiency,improper diet,over exertion and so on.According to these pathogenesis,therapeutic principle as supplementing qi and nourishing yin,invigorating vital energy and spleen,removing blood stasis and phlegm,and nourishing yin and kidney are established.Besides,acupuncture and massage have been applied as well.According to the literatures,good potentialities have been shown in traditional Chinese medicine intervening on IGT.

Objective To analyze the relationship between soluble brain proteoglycan (PG) and brain development. Methods SD rats were divided into three groups: new-born, young adult and near-old groups. Soluble brain PGs of each group were extracted with 4 mol?L -1 guanidine HCl and purified by DEAE-sephacel chromatography, their molecular sizes were identified by Sepharose 4B chromatography, and their GAG compositions were characterized by two dimensional electrophoresis. Results The extraction amounts of soluble brain proteoglycans were decreased with age; soluble brain PGs could be divided into four fractions according to molecule size. The percentage of the large size CSPG aggregate increased with age, but its HA declined. The percentage of the small sized fraction containing DSPG declined with age increase. Conclusion Soluble brain PGs change with age and are related to brain development.

Hepatocellular carcinoma (HCC) is one of the most common cancers in the worldwide.In recent years,the development of imaging techniques has facilitated the detection of HCC at early stages.However,HCC has a high frequency of portal vein invasion,which is reportedly observed in 11％ to 42％ of patients with HCC.Portal vein tumor thrombus(PVTT) is a crucial factor that can worsen the prognosis of HCC because it can be wide dissemination of tumor throughout the liver and cause a marked deterioration of hepatic function.It was considered that is not appropriate with surgical treatment in the past,which is treatment conservative or even give up,most of the patients died within a few months.Even if the patients have a chance to surgical resection,and the most of patients combined with TACE,portal vein stent implantation or chemotherapy postoperative,some patients can be more prolonged survival,however,due to the small tumor thrombus and micro metastases have exist before the surgery and can not be checked out.it's difficult to clean up in the surgery,easily lead to HCC recurrence postoperative.However,with the constant awareness the PVTT formation mechanism and scientific technology progress,more and more new treatment techniques is applied to clinical.This view focuses on the portal vein tumor thrombus formation mechanisms and Classic Transcatheter Arterial Chemoembolization and new treatment methods such as:Helical tomotherapy,CIK cell therapy (cytokine-induced killer cell therapy),Chemoembolization and stent combined with iodine-125 seed.

Mitofusin 2 (Mfn2) plays an important role in the process of mitochondrial fusion, and is involved in regulating mitochondrial function and morphological changes. Studies have found that Mfn2 has a tumor suppressor effect in a variety of malignant tumors and their cell lines, including cervical cancer, hepatocellular cancer, pancreatic cancer, breast cancer, and bladder cancer. The difference in the expression of Mfn2 in malignant tumor tissues suggests that Mfn2 may play an important role in the proliferation, apoptosis, invasion and migration of malignant tumor cells. As a cancer-related gene, Mfn2 provides new ideas for gene therapy of malignant tumors.

In order to understand the status of neonatal transport research at home and abroad,we summarized and analyzed the research progress of neonatal transport through the literature search.Thus we evaluated the current application of a variety of transport critical rating system.Intrauterine transport is considered the safest mode of transport,and promote intrauterine transport of high-risk mothers.It is suggested that the parents participate in the transshipment process and return the stable children to the local hospital for further treatment and promote the family-centered treatment mode.In transit,mobile ECMO,hypothermia and other advanced equipment in foreign countries have been applied.It is recommended to use the respiratory function monitor to monitor the respiration during transit.It can provide the parameters of respiratory wave,identify air leak,accidental release,spontaneous breathing.

Neovascular eye diseases,with highly complicated and refractory,are one group of the most difficult problems for domestic and overseas clinical oculists in recent years.Angiogenesis is a complex process participating with multiple factors and various ways,and its exact pathogenesis is unclear yet.Ocular neovascularization is the dynamic balance result between proangiogenic factors and antiangiogenic factors provided that intraocular microenvironment be under a physiological condition.Some pathological factors including ischemia,hypoxia and inflammation destroy the dynamic balance between cytokines leading to neovascularization.Currently studies indicated that numerous cytokines containing vascular endothelial growth factor (VEGF) and pigment epithelium derived factor (PEDF) are closely linked to ocular neovascularization,while it is more crucial to elucidate the molecular mechanism of numerous cytokines involved in ocular neovascularization,which can recognize the pathogenesis of neovascular eye disease more clearly.Research progress of pathological relevant cytokines of neovascular eye diseases,including VEGF,insulin-like growth factor 1 (IGF-1),angiogenin-2 (Ang-2),stromal cell derived factor 1 (SDF-1) and PEDF were reviewed in this paper.

0.05). Conclusion Both acanthopanasia and hoelen have no mutagenic toxicity founded on the genetic damage of somatic cells,and have significant antagonistic effects on the genetic damage induced by cadmium sulfate,and they are good antimutagents.

Objective To evaluate the veracity and consistency of the results obtained from three automated hematology an-alyzers. Methods The reproducibility tests were used to assess the precision,the verification tests were exerted to evaluate the accuracy and consistency of test parameters(WBC,RBC,Hb,Hct and Plt) of the three hematology analyzers. According to the 1/2 standard of American Clinical Laboratory Improvement Amendment (CLIA,88),the relative bias of the reference analyzer and other assessed analyzers were determined. Results The results obtained from the three analyzers showed ex-cellent precision and stability,the coefficients of variation (CV) were all less than 5%; compared with the results of LH750,the results of Gens 5diff and ACT-5diff showed excellent correlation,all coefficients of correlation(?) exceeded 0.975. The results of both RBC and Hct obtained from Gens 5diff and ACT-5diff exceeded allowable error; the results of WBC,Hb and Plt were in the allowable error range. Conclusion In the condition of the excellent precision and stability,system errors existed in the analyzer can be found by verification on time. Through being adjusted and calibrated,the hematology ana-lyzers can meet the requirements and these results are consistent.