Diamond-Blackfan anemia(DBA),also known as congenital pure red cell aplasia,is a rare genetic disorder characterized by bone marrow failure,congenital anomalies,and severe red blood cell abnormalities.The rarity of the condition,and consequently limited patient pool and scarcity of research models,means that the pathogenic mechanisms associated with genetic mutations in DBA remain uncertain,and the clinical treatment options are limited.This review synthesizes the findings from zebrafish,mouse,and human cellular models of DBA mutations.We clarify the pathogenic mechanisms and monitor the progression of drugs into clinical trials,thereby aiding further in-depth explorations into the etiology and therapeutic advancements for DBA.

Diamond-Blackfan anemia(DBA),also known as congenital pure red cell aplasia,is a rare genetic disorder characterized by bone marrow failure,congenital anomalies,and severe red blood cell abnormalities.The rarity of the condition,and consequently limited patient pool and scarcity of research models,means that the pathogenic mechanisms associated with genetic mutations in DBA remain uncertain,and the clinical treatment options are limited.This review synthesizes the findings from zebrafish,mouse,and human cellular models of DBA mutations.We clarify the pathogenic mechanisms and monitor the progression of drugs into clinical trials,thereby aiding further in-depth explorations into the etiology and therapeutic advancements for DBA.

OBJECTIVETo assess the safety and clinical efficacy of transjugular intrahepatic portosystemic shunt (TIPS) with various stents for treating patients with cirrhosis and esophageal gastric varices bleeding.

METHODSOne hundred and five patients were stratified according to stent type: bare stent group, covered stent-grafts group, combined stents group. Rates of success, shunt insufficiency, rebleeding, patient survival, and major complications were observed. The shunt insufficiency rate, rebleeding rate, and survival rate were calculated by the life tables method, the Kaplan-Meier analytical curve, and the log-rank test; a p-value less than 0.05 was considered statistically significant.

RESULTSThe overall success rate of all TIPS for treating the esophageal gastric varices bleeding was 100%. The overall shunt insufficiency rates at 6-, 12-and 24-months post-TIPS were 8%, 9% and 16%, rebleeding rates were 2%, 6% and 17%, and survival rates were 100%, 97% and 94%. The shunt insufficiency rate was 26% in the bare stent group, 14% in the covered stent-grafis group, and 5% in the combined stents group (x2=1.00, P=0.61). The rebleeding rate was 33% in the bare stent group, 7% in the covered stent-grafts group, and 3%in the combined stents group (x2=1.69, P=0.43). The survival rate was 92% in the bare stent group, 93% in the covered stent-grafts group, and 100% in the combined stents group (x2=1.91, P=0.39). The shunt insufficiency rates were higher in patients with splenectomy than in those without splenectomy (30% vs.14%; x2=4.15, P=0.04). The intraperitoneal hemorrhage rates in the covered stent-grafis group and the combined stents group were significantly lower than that in the bare stent group (0% vs 0% vs 13%; x2=8.88, P=0.01).

CONCLUSIONSTIPS with an 8 mm stent effectively treated and prevented esophageal gastric varices bleeding in patients with cirrhosis. Intraperitoneal hemorrhaging caused by TIPS was significantly decreased in the covered stent-grafts group and combined stents group,which represented an improvement in safety of this treatment. However, the influence of covered stent-grafis and combined stents towards the clinical efficacy of TIPS needs further study.

Esophageal Diseases ; Esophageal and Gastric Varices ; Gastrointestinal Hemorrhage ; Humans ; Kaplan-Meier Estimate ; Liver Cirrhosis ; Portasystemic Shunt, Transjugular Intrahepatic ; Stents ; Survival Rate

OBJECTIVE:To provide the clinical departments with a reference for promoting rational drug use. METHODS:The inpatient prescriptions given by orthopaedics and burn departments from Jun. to Dec. 2013 were selected randomly. The number of the selected prescriptions by each department accounted for 2% of the total prescriptions given by the corresponding department in that month,including 4 921 cases by orthopaedics department and 1 391 cases by burn department. Statistical analysis of irrational prescriptions was made according to Chinese Pharmacopoeia,New Materia Medica,the package insert, and other relevant references. RESULTS:1 821 prescriptions given by orthopaedics department and 378 by burn department were found to be irrational,accounting for 37.00% and 27.17% respectively. The reasons of irrationality mainly included im-proper compatibility,improper route of administration,contraindication in and use with caution by the elderly and children,re-peated drug use,improper drug combination etc. CONCLUSIONS:The system of prescription review should be strengthened, and clinical staff training system and pharmaceatical knowledge information platform are established to promote rational use of drugs.

Objective To investigate the antagonistic effect of wogonin in combination with 5-fluorouracil (5-FU) on the proliferation of human Hep-G2 hepatocellular carcinoma cells .Methods Human hepatocellular Hep-G2 cells were divided into experimental group (wogonin group ,5-FU group ,wogonin + 5-FU group) and control group .MTT method was used to eval-uate tumor cell proliferation in vitro ,flow cytometry analysis was used to evaluate tumor cell apoptosis .Results The results showed that the wogonin inhibited the proliferation of tumor cells at the concentrations of 5 ,10 ,20 and 40 μmol/L after 24 h and 48 h treatment respectively (P<0.05);5-FU also inhibited the proliferation of tumor cells at the concentrations of 5 ,10 , 20 and 40 mg/L after 24 h and 48 h treatment respectively (P< 0.05) .However when wogonin was combined with 5-FU (wogonin+5-FU group) ,an antagonistic effect was observed on tumor cell proliferation (P<0.05) .When cells were treated by wogonin+5-FU for 48 h ,the combined index (CI) value slowed a dose-dependent antagonistic effect (P<0.05) .Conclusion Wogonin has anti-tumor effect .However when wogonin was combined with 5-FU ,an obvious antagonistic effect on 5-FU′s anti-tumor action was observed .The underlying mechanism deserves further study .