[Objective] We explore the diagnosis of Smith-Magenis syndrome and its clinical features of children,to raise the domestic awareness of this disease.[Methods] In this study,the child received peripheral blood chromosome microarray analysis,blood routine and urine routine,growth hormone provocation test,insulin-like growth factor Ⅰ and insulin-like growth factor binding protein Ⅲ test,cortisol (8a) test,prolactin test,adrenocorticotropic hormone test,thyroid function test,liver and kidney function test,blood biochemistry test,fasting insulin test,2-hour plasma glucose test,the antibodies and antigens test of hepatitis B.The bone age measurement and the pituitary gland MRI were also performed.We use the above figures to diagnose Smith-Magenis syndrome,assess and observe the condition of the child in Smith-Magenis syndrome.[Results] In this case,the chromosomal microarray analysis revealed a deletion of about 3.6Mb fragments in the chr17p11.2 region,including main functional gene RAI1,which was associated with Smith-Magenis syndrome.According to the clinical manifestations and the result of chromosome microarray analysis,the diagnosis of children with Smith-Magenis syndrome was made clear.[Conclusion] Genetic tests are the standard for diagnosing Smith-Magenis syndrome.When children have special facial features combined with multiple system disorders,early genetic examination is conducive to early diagnosis,and can reduce the time and economic cost.

OBJECTIVETo optimize the method in the Chinese Pharmacopoeia for determining Salviae Miltiorrhizae Radix et Rhizoma.

METHODTanshinone II(A) and salvianolic acid B were selected as the index in optimization of the sample preparation method of Salviae Miltiorrhizae Radix et Rhizoma in Chinese Pharmacopoeia. Orthogonal test was used to optimize the extraction process of Salviae Miltiorrhizae Radix et Rhizoma, and concentration of contents were detected by high performance liquid chromatography method. A detection of using methanol-water (85: 15) at wavelength of 270 nm was employed for tanshinone II(A) and a detection of using methanol-acetonitrile-formic acid-water (30:10:1: 59) at wavelength of 286 nm was employed for salvianolic acid B.

RESULTThe optimized extraction process of tanshinone II(A) and salvianolic acid B was: extracted by 90% methanol and reflux twice (0.5 h each time) at 75 degrees C, extracted by 70% methanol and reflux twice (1.5 h each time) at 75 degrees C, respectively.

CONCLUSIONOptimized extraction and determination methods could be used to reflect the content of tanshinone II(A) and salvianolic acid B in Salviae Miltiorrhizae Radix et Rhizoma more accurately and efficiently.

Benzofurans ; analysis ; Chromatography, High Pressure Liquid ; Diterpenes, Abietane ; analysis ; Rhizome ; chemistry ; Salvia miltiorrhiza ; chemistry ; Temperature

BACKGROUNDData on the epidemiology of hypertension in Chinese non-dialysis chronic kidney disease (CKD) patients are limited. The aim of the present study was to investigate the prevalence, awareness, treatment, and control of hypertension in the non-dialysis CKD patients through a nationwide, multicenter study in China.

METHODSThe survey was performed in 61 tertiary hospitals in 31 provinces, municipalities, and autonomous regions in China (except Hong Kong, Macao, and Taiwan). Trained physicians collected demographic and clinical data and measured blood pressure (BP) using a standardized protocol. Hypertension was defined as systolic BP ≥ 140 mmHg and/or diastolic BP ≥ 90 mmHg, and/or use of antihypertensive medications. BP < 140/90 mmHg and < 130/80 mmHg were used as the 2 thresholds of hypertension control. In multivariate logistic regression with adjustment for sex and age, we analyzed the association between CKD stages and uncontrolled hypertension in non-dialysis CKD patients.

RESULTSThe analysis included 8927 non-dialysis CKD patients. The prevalence, awareness, and treatment of hypertension in non-dialysis CKD patients were 67.3%, 85.8%, and 81.0%, respectively. Of hypertensive CKD patients, 33.1% and 14.1% had controlled BP to < 140/90 mmHg and < 130/80 mmHg, respectively. With successive CKD stages, the prevalence of hypertension in non-dialysis CKD patients increased, but the control of hypertension decreased (P < 0.001). When the threshold of BP < 130/80 mmHg was considered, the risk of uncontrolled hypertension in CKD 2, 3a, 3b, 4, and 5 stages increased 1.3, 1.4, 1.4, 2.5, and 4.0 times compared with CKD 1 stage, respectively (P < 0.05). Using the threshold of < 140/90 mmHg, the risk of uncontrolled hypertension increased in advanced stages (P < 0.05).

CONCLUSIONSThe prevalence of hypertension Chinese non-dialysis CKD patients was high, and the hypertension control was suboptimal. With successive CKD stages, the risk of uncontrolled hypertension increased.

Adult ; Aged ; Awareness ; Female ; Humans ; Hypertension ; complications ; epidemiology ; therapy ; Male ; Middle Aged ; Prevalence ; Renal Insufficiency, Chronic ; complications

Multi-drug resistance (MDR) of cancer cells is a major cause of failure in chemotherapy. To the majority of anti-cancer drugs, tumor cells are able to generate a multi-drug resistance; but there is no common views on the mechanism of MDR. This review summarizes the use of drug delivery system based on nanoparticles to overcome MDR in recent years. Three kinds including non-modified, ligand-modified and multifunctional drug delivery systems are described. Especially, the mechanism of reversing MDR based on nanoparticles is covered. Through efficiently offsetting and antagonizing the action of pumping drugs out of the tumor cells, drug delivery system based on nanoparticles can increase the concentration of the drug in tumors, while reduce the side effects on normal cells and overcome multi-drug resistance. The use of drug-loaded nanoparticles, which combines nanotechnology with the strategy of active and passive targeting administration, has shown significant prospect improving cancer therapy.
Animals ; Antineoplastic Agents ; administration & dosage ; therapeutic use ; Drug Delivery Systems ; methods ; Drug Resistance, Multiple ; Drug Resistance, Neoplasm ; Humans ; Nanoparticles ; therapeutic use ; Neoplasms ; drug therapy

OBJECTIVETo investigate the effects and the mechanisms of stanozolol (ST) on the proliferation, maturation and differentiation of in vitro cultured growth plate chondrocyte isolated from gonadotropin releasing hormone analogue (GnRHa)-treated adolescent rats, to study if ST mediates the proliferation of chondrocytes via the estrogen receptor alpha (ERalpha), androgen receptor (AR) and/or insulin-like growth factor-1 receptor (IGF-1R) and interactions of the two receptor and IGF-1R receptor signaling pathway, to investigate the mechanism of the biological effects in ST promoting bone growth/maturity at molecular level.

METHODThe rats were weaned at the end of 3 weeks and intramuscular injection of triptorelin of GnRHa preparations, qow x 2 was started. The rats were sacrificed at the end of 7 weeks, and then the tibiae growth plates were taken out with sterile procedure. The chondrocytes were obtained by two-time enzyme digestion method, and the experiments were carried out with the primary chondrocytes. Immunohistochemical staining of proliferating cell nuclear antigen (PCNA) and Western blot analysis were applied.

RESULTThe results of PCNA demonstrated that stanozolol enhanced the proliferation of the chondrocytes, time-course studies showed that the proliferation were maximally stimulated by stanozolol after 2 days of incubation and decreased again after longer periods of incubation. The expression of p-ERalpha, p-IGF-1R and p-extracellular-signal regulated kinase 1/2 (ERK1/2) increased with the incubation period of ST treatment, and reached the peak value at a certain time, and then gradually decreased. The expression of p-ERalpha, p-IGF-1R and p-ERK1/2 increased with the elevation of ST concentration, and reached the peak value at 10(-9) - 10(-8) mol/L, then gradually decreased. ST induced-p-ERalpha expression was partially blocked by ERalpha and mitogen-activated protein kinase kinase inhibitors. ST induced-p-IGF-1R expression was partially blocked by ERalpha and IGF-1R inhibitors. ST induced-p-ERK1/2 expression was partially blocked by mitogen-activated protein kinase kinase and IGF-1R inhibitors.

CONCLUSIONAs an androgen derivation, ST exerts its biological effects of promoting proliferation of the long bone growth plate chondrocytes via activating the classic ERalpha receptor pathway and mitogen-activated protein kinase pathway, and at the same time, by activation of IGF-1R. Both IGF-1R and ERalpha can promote "cross-talk" of two systems' receptor signal through mitogen-activated protein kinase signal pathway.

Androgens ; pharmacology ; Animals ; Cells, Cultured ; Chondrocytes ; cytology ; drug effects ; metabolism ; Estrogen Receptor alpha ; metabolism ; Female ; Growth Plate ; drug effects ; metabolism ; Mitogen-Activated Protein Kinase 3 ; metabolism ; Rats ; Receptor Cross-Talk ; Receptor, IGF Type 1 ; metabolism ; Signal Transduction ; drug effects ; Stanozolol ; pharmacology

OBJECTIVETo study the relationship between the changes of intestinal mucosal tumor necrosis factor alpha (TNF-alpha), plasma diamine oxidase (DAO) values and the degree of mucosal injuries in young rat model of colitis and thereby to explore if plasma DAO could be used as a potential index for monitoring intestinal mucosal injury.

METHODSOne hundred and four healthy young male Sprague-Dawley (SD) rats aged 5-6 weeks were randomly divided into three groups: zero time group (n = 8), model group (n = 48) and control group (n = 48). The model and control groups were further divided into 24 h, 72 h, 1 week, 2 weeks, 3 weeks and 4 weeks subgroups, respectively, with 8 rats in each. The rats in model group were given 2, 4, 6-trinitrobenzene sulfonic acid (TNBSA) via enema to induce colitis, while the rats in the control group were given normal saline (NS) solution in the same way and those in zero time group were not treated. TNF-alpha and DAO were measured by immunohistochemical technique and spectrophotometry.

RESULTSThe most serious enteric mucosal injury was seen 24 hours after giving TNBSA. Plasma DAO and TNF-alpha decreased as the intestinal mucosal injury was alleviated.

CONCLUSIONSPlasma DAO values may be used as a marker for intestinal mucosal injury. TNF-alpha is a factor for causing mucosal injury. Young rat colitis model can be used to study intestinal mucosal injury.

Amine Oxidase (Copper-Containing) ; blood ; Animals ; Biomarkers ; blood ; metabolism ; Colitis ; blood ; chemically induced ; metabolism ; pathology ; Disease Models, Animal ; Enema ; Immunohistochemistry ; Intestinal Mucosa ; metabolism ; pathology ; Male ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Severity of Illness Index ; Spectrophotometry ; Time Factors ; Trinitrobenzenesulfonic Acid ; Tumor Necrosis Factor-alpha ; metabolism

Child, Preschool ; Humans ; Male ; Nevus, Blue ; pathology ; Skin Neoplasms ; pathology

OBJECTIVEWith the development of endoscopic therapy in children, endoscopic electrocoagulation polypectomy had gradually replaced surgery and became an important method to resect gastrointestinal polyps in children. Simple electrocoagulation polypectomy could often bring some complications of gastrointestinal bleeding and perforation because of incomplete electrocoagulation or mechanical incision, especially in gastrointestinal thick-pedunculated polyps which always have thick nutrient blood vessel. Hemoclips can successfully interdict arteriovenous blood because it can clamp tissue firmly without causing necrosis around the target area. Based on its good mechanical hemostasis, hemoclips are not only widely used in treating bleeding like from ulcer, tumor and variceal ligation but also used in removal of thick-pedunculated gastrointestinal polyps in adults. This paper describes the application of endoscopic electrocoagulation with metal hemoclips to remove thick-pedunculated intestinal polpys in children for the first time, sums up the experience and evaluates its efficacy and safety.

METHODSBetween October, 2001 and December, 2002, 5 cases with thick-pedunculated intestinal polpys were presented. The age of the patients ranged from 3 to 5 years. The clinical features were gastrointestinal bleeding or abdominal pain. The longest course of disease was 2 years. Enough preparations for alimentary tract were necessary for polypectomy. The procedures were performed under general anesthesia in order to avoid the risk of bleeding aspiration. Endoscopy was performed in the standard fashion. The apparatus included electronic colonic endoscope (XQ 200, Fuji Corp, Tokyo, Japan), snare (XQ200, Fuji Corp, Tokyo, Japan), impeller of the clip (HX-5QR-1) and hemoclip (MD850) which could be passed through the biopsy channel of endoscope. The clip was completely covered with a hood avoiding any injury to the mucous membrane. The pedicel with diameter of more than 1.0 cm underwent endoscopic electrocoagulation polypectomy with hemoclips. The clip contacted polyps in upright direction. One or more hemoclips were selected to clamp the proximal basement of the pedicel in terms of the pedicel diameter. Turning of the red colour of polyps to purple suggested that hemoclip interdicted arteriovenous blood effectively. The clip was then shut off and electrocoagulation polypectomy was followed. Six polyps were observed and removed.

RESULTSSix polyps including 2 transverse colon polyps and 4 descending colon polyps were resected. Pathological results showed that 3 were juvenile polyps and the other 3 adenomatous polyps. All the polyps were completely resected. The diameter of pedicel were 1.2 - 2.2 cm. The head and pedicel of the biggest polyp was about 5 cm x 5 cm and 2.2 cm, respectively, and five clips were used in order to remove it. No complications of bleeding and perforation were observed in these children. All hemoclips were expelled from intestines within one week. The symptoms of these patients disappeared.

CONCLUSIONMechanical hemostasis with hemoclips successfully interdicted arteriovenous blood of thick-pedunculated polyps. Hemoclips can successfully prevent the complications of bleeding and perforation. The clipping brings about a new method in endoscopic therapy. Endoscopic electrocoagulation polypectomy with hemoclips is a simple, safe and effective method to treat thick-pedunculated gastrointestinal polyps in children and it is a valuable tool in polypectomy for children.

Child ; Child, Preschool ; Endoscopy ; methods ; Humans ; Intestinal Polyps ; surgery ; Surgical Instruments ; Treatment Outcome

OBJECTIVEEndoscopic sclerotherapy has emerged as an effective treatment for bleeding esophageal varices in adults and children but the long-term outcome is poorly defined in children. The present study aimed to study the long-term effect of endoscopic sclerotherapy in children with portal hypertension.

METHODSFifteen patients (age 3 to 14 years) with esophageal variceal bleeding underwent endoscopic injection treatments with 1% Aethoxy-sclerol since 1996. All subjects continued to receive the therapy by repeated intra and extravariceal endoscopic sclerotherapy at intervals of 3 - 4 weeks until the varices disappeared, and received regular endoscopic follow-up.

RESULTSFifteen patients had totally 43 injections, and were followed up from 40 to 86 months (mean 66 months) by endoscopy. Two patients received 2 injections and 5 received 3 before eradication of varices. The mean time needed for varices eradication was 3 to 6 months. Recurrence of varices and bleeding was seen in 3 patients who had duodenal ulcer.

CONCLUSIONEndoscopic sclerotherapy is a safe and effective treatment for pediatric esophageal varices.

Adolescent ; Child ; Child, Preschool ; Duodenal Ulcer ; complications ; Esophageal and Gastric Varices ; etiology ; therapy ; Esophagoscopy ; Gastrointestinal Hemorrhage ; etiology ; therapy ; Humans ; Hypertension, Portal ; complications ; Injections, Intralesional ; Polyethylene Glycols ; administration & dosage ; Recurrence ; Reoperation ; Sclerosing Solutions ; administration & dosage ; Sclerotherapy ; Time Factors ; Treatment Outcome

OBJECTIVEData on traffic accident from year 2000 to 2002 were collected and testified. Epidemiological study was carried out to find the main risk factors of traffic accident.

METHODSThe spatial distribution was conducted by means of Geographic Information System (GIS) and were marked on Shanghai digitalized map with different layers by different colors.

RESULTSResults showed that during 2000, 2001 and 2002, 69,669 , 68,894, and 47,088 traffic accident episodes occurred in Shanghai, resulting in 1747, 1724, 1557 deaths respectively with direct economic lost: 2.1, 2.4, 3.0 billion RMB. The main risk factors causing traffic accident deaths would include drinking alcohol before driving, fatigue and speeding. The leading causes of death were head injury and complex injury of body. GIS showed that there was a homocentric circle with more traffic accidents in downtown city, but more deaths in the urban city. Disease burden caused by traffic accident was very heavy.

CONCLUSIONIn Shanghai, traffic accident has great impact on social and economic issues thus should be controlled effectively. Focus should be layed on control of traffic accident in downtown area and to decrease the number of deaths related to traffic accident in the urban area.

Accidents, Traffic ; Age Factors ; China ; epidemiology ; Female ; Humans ; Male ; Risk Factors ; Sex Factors ; Wounds and Injuries ; epidemiology ; etiology