Objective To observe the clinical efficacy of Santeng Tongbi pills on rheumatoid arthritis (RA),evaluate the safety and explore its mechanism. Methods Double-blind and double dummy design method was used. 107 cases of RA were randomly divided into Santeng Tongbi pills group (the treatment group,53 cases) and Zhengqing Fengtongning group (the control group,54 cases). The Ritchie index,health assessment questionnaire (HAQ),arthritis influenced index (AIMS),patinents' assessment to pain (VAS form),acute phase reactant,Chinese medical pattern grade,the hemorheology state and so on were retrospectively analyzed. Results There was significantly difference between RA patients and healthy people on the hemorheology state (P

Objective To explore the possible role of NROB1 gene played in regulating hypothalamic-pituitary-gonad axis(HPGA) by analyzing the clinical and molecular characteristics in a case of central precocious puberty(CPP) with NROB1 gene mutation. Methods Clinical characteristics and direct sequencing of NROB1 gene in the patient were analyzed. Results A 11-month-old boy with manifested premature puberty, enlargement of penis/testes, and penile erection, but without manifestations of adrenal insufficiency was reportd. Clinical diagnosis was adrenal hypoplasia congenita( AHC) with CPP. The NR0B1 gene sequencing revealed a novel mutation in exon 1 (913C> T). Conclusion NR0B1 gene mutation may lead to the development of CPP in the patient with AHC. However, the mechanism remains unclear and thus deserves further exploration.

Objective: To detect ED1 gene mutation in three hypohidrotic ectodermal dysplasia (HED) nuclear families. Methods: Peripheral blood samples were obtained from three different families of hypohidrotic ectodermal dysplasia. Genomic DNA was extracted. Polymerase chain reaction, direct sequencing and restriction enzyme reaction were performed to identify the mutations. Results: Different missense mutation in ED1 gene were found in each family: C412G, A1201G and C1375T. Two of the mutations had not been previously reported. Conclusion: Mutations in the ED1 gene are responsible for the phenotypes of HED of the patients in the family.

Objective To discuss the surgical management of von Hippel Lindau(VHL) syn drome.Methods The clinical data of four VHL patients who were clinically diagnosed from March1999 to October 2006 were reviewed.The first patient was a 56 year old man with the chief complaintof hypodynamia and low serum glucose as 2.37 mmol/L.CT scan showed three masses in the the leftkidney.He had a history of cerebral haemangioblastoma ten years before.The second case was a 57 year old woman with the chief complaint of lumbago in the left side.CT scan showed masses in theleft kidney,adrenal gland and panerease.The third case was a 39 year old woman with the chief com plaint of an accident mass in the left adrenal gland.She had the history of cerebellar hemangioblastoma9 years before,spinal hemangioblastoma resection 7 years before.The fourth case was a 41-year oldwoman,she was found brain tumors and cured by gamma radiation abalation.Bilateral renal masseswere found by B ultrasound one month later,CT scan showed four masses in the both kidneys,leftadrenal gland and pancreas.Results All 4 cases underwent surgical approach.The first case under went radical nephrectomy which pathological report was PEComa of kidney.The blood glucose wasnormal one week later.The second case underwent resection of the left adrenal gland,kidney,pancre atic body and tail and spleen.Pathological report was clear cell carcinoma,islet cell tumor and adrenal cyst.Three months later she was found spinal hemangioblastoma and refused treatment.The thirdcase underwent adrenalectomy in the left side and pathologieal report was adrenal pheoehromocytoma.There was not tumor reeurrenee during 2 years' follow up.The nephrectomy and adrenalectomy wasperformed for the last ease whose pathological report was clear celt carcinoma and pheochromoeytoma.Three weeks later,tumor enueleating of the right kidney was undertaken; the result was clear cellcancer.During the follow up for one year there was no relapse of tumor.Conclusions For VHL ac companied with multiple organ tumors,surgery resection is the proper approaeh when tumors of centralnervous system is large.Different approaches could be taken to deal with multiple tumors of VHL such aswatchful waiting,nephron sparing surgery.

Objective To explore the protective effect of Xuebijing on the endothelium and extracellular matrix in sepsisinduced acute kidney injury (AKI) rats for providing a new clinical treatment strategy.Methods The method of cecal ligation and puncture (CLP) was used to duplicate severe sepsis model.Thirty healthy male SD rats were randomly divided into 3 groups:Sham group (n=10),NS group (normal saline 4ml/kg,n=10),Xuebijing group (Xuebijing 4ml/kg,n=10).6h after CLP,the rats were sacrificed and their kidneys were resected and histopathological characteristic was observed by light microscopic and transmission electron microscopic techniques.The expressions ofET-1 mRNA,iNOS mRNA,MMP-9 mRNA,TIMP-1 mRNA in the renal tissues were measured semiquantitatively by reverse transcription-polymerase chain reaction (RT-PCR).Results The histopathological changes in renal tissue were observed by light microscope.The changes of renal glomerulus and renal tubuli in Xuebijing group were better than NS group.The ultrastructural changes in renal tissue were observed under electron microscope.Compared with NS group,ultrastructural changes of renal glomerulus and proximal convoluted tubule were smaller in Xuebijing group.The expressions of ET-1mRNA (0.631 ± 0.169vs 0.770 ± 0.154,P＜0.05),iNOS mRNA (0.507 ± 0.071 vs 0.587 ± 0.073,P＜0.05),MMP-9mRNA (0.641 ± 0.082 vs 0.742 ± 0.116,P＜0.05) and TIMP-1 mRNA (0.434 ± 0.052 vs 0.520 ± 0.049,P＜0.01) were significantly lower in XBJ group renal tissues than in NS group.The expressions of ET-1 mRNA(0.770 ± 0.154 vs 0.394 ± 0.105,P＜0.01),iNOS mRNA (0.587 ± 0.073 vs 0.326 ± 0.085,P＜0.01),MMP-9 mRNA (0.742 ± 0.116 vs 0.356 ± 0.055,P＜0.01) and TIMP-1 mRNA (0.520 ± 0.049 vs 0.351 ± 0.041,P＜0.05) in renal tissues were more significantly increased in NS group compared with sham group.Conclusions Xuebijing could reduce the levels of ET-1,iNOS,MMP-9 and TIMP-1 mRNA,protect the stability of endothelium and extracellular matrix,and reduce tissue injury in septic rats.

Objective:To investigate the effects and mechanisms of Xuebijing injection on early sepsis induced acute lung injury and explore a new therapy.Methods:Thirty healthy male Sprague-Dawley rats were randomly divided into 3 groups:Sham group(n=10),NS group (NS 4mL/kg n=10),Xuebijing Group(Xuebijing 4mL/kg n=10).Cecal ligation and puncture (CLP) was used to duplicate severe sepsis model.At 6h after CLP,the rats were sacrificed,the lungs were resected and histopathological characteristic was observed by transmission electron microscopy technique.The change of the lung wet/dry (W/D) weight ratio were studied.Meanwhile,the lungs were resected for detection of ET-1,iNOS,MMP-9,TIMP-1 mRNA with reverse transcription-polymerase chain raction (RT-PCR).Results:The changes of pulmonary alveoli and the interstitial edema as well as lung tissue in Xuebijing group were better than those of NS group.The change of lung wet/dry (W/D) weight ratio in septic rats showed was significantly increased at 6 h after CLP (vs Sham group:5.37± 0.12 vs 4.33± 0.06,P＜0.01).The lung W/D was significantly decreased in XBJ group (vs NS group:4.67± 0.09 vs 5.37± 0.12,P＜0.05).The expressions in XBJ group lung tissue of ET-1 (0.511 ± 0.111 vs 0.705± 0.122,P＜0.01),iNOS(0.45610.075 vs 0.548± 0.098,P＜0.05)、MMP-9 (0.617± 0.079 vs 0.732± 0.131,P＜0.05),TIMP-1 (0.438± 0.043 vs 0.515± 0.049,P ＜0.01) mRNA were significantly decreased than those in NS group.And the expressions of ET-1 (0.705± 0.122vs 0.400± 0.033,P＜0.01),iNOS (0.548± 0.098 vs 0.334± 0.027,P＜0.01),MMP-9(0.732± 0.131 vs 0.352± 0.061,P＜0.01),TIMP-1(0.515± 0.049 vs 0.365± 0.068,P＜0.01) mRNA in NS group were significantly increased compared with those in the sham group.Conclusions:Xuebijing could protect against sepsis induced acute lung injury,which might be related with the decrease ofET-1,iNOS,MMP-9 and TIMP-1 mRNA expressions.

Objective To evaluate the mid-term follow-up of the pereutaneous angioplasty of the occlusive acrtoiliac artery diseases.Methods The data of 30 patients who had distal abdominal aorta and bi-lateral iliac artery stenosis or occlusion and treated by percutaneous angioplasty were retrospectively reviewed.They are 24 males and 6 females,aged from 35 to 75 years (average 55 ± 10 years). The interventional procedures include wire-guided canalization,thrembolysis,balloon angioplasty and stents implantation.They were followed up by telephone,letter or visiting after discharged.Results Twenty-six patients with aarto-iliac are revascularized successfully.Three cases failed.Of them,one failed because only one side of iliac artery and aorta were successfully canalized while the eontralateral not,one failed to revascularize both the aorta and the iliac artery,the other one failed due to the ihac rupture and replaced by WallGraft implantation.Two cases had distal thrombosis during the procedure.One case was amputated due to the aggravated isebemia of the right lower extremity after the revascularization failed.Twenty-seven cases were followed uo after 1 to 112 months,averaged (41±9) months.One case showed that the iliac artery was uncanalized after 6 months.One case showed bilateral iliac artery occlusion during 25 months follow-up and re-occluded 3 months after balloon angioplasty and finally had stent implanation in bilateral iliac artery.The other patients had no aggravated symptom of recurrence.Conclusion The percutaneous angioplasty is a good choice for occlusive aorto-iliac artery disease,showing good recovery during mid-term follow-up.

Objective To emphasize the importance of embolization of nonbronchial systemic arteries in treatment of acute and life-threatening massive hemoptysis.Methods In a series of 146 patients with hemoptysis who underwent bronchial artery embolization,we found 12 cases whose blood supply were from 17 nonbronchial systemic arteries and hemoptysis was more than 300 ml blood within 24 hours.Embolic materials included absorbable gelatin sponge(GS),kelp micro gelatin(KMG),polyvinyl alcohol(PVA) particles and metal coils. Results In the 12 cases with 17 nonbronchial systemic arteries (4 were intercostal,3 internal mammary,3 thyrocervical trunk,3 inferior phrenic,1 left gastric,2 originated from the inferior aortic arch,and 1 originated from anterior abdominal aortic wall).Five cases were embolized by GS alone,2 cases by KMG,3 cases by GS+PVA,and 2 cases by GS+PVA+metal coils.Eight cases were performed embolization once,3 cases were performed twice and 1 case was performed three times.No significant complications developed related to embolization,except that 1 patient had transient eyesight decrease after embolization of thyrocervical trunk and 2 patients had chest pain after embolization of intercostal artery which resovled without any treatment.Conclusions During bronchial artery embolization for hemoptysis patients,all supplying artery should be searched and found.Even after successful embolization of bronchial arterys for hemoptysis patients,nonbronchial systemic arterial supply should still be taken into account.

[Objective] To observe the efficacy of metformin treatment on non-alcoholic fatty liver disease (NAFLD) in obese children.[Methods] A retrospective analysis was performed of 10 patients over 10 years old with NAFLD from July 10,2013 to August 23,2016.These patients were treated with metformin in pediatric endocrinology outpatient department of the First Affiliated Hospital of Sun Yat-sen University.The changes of liver ultrasonography,hepatic enzymes,blood lipids,blood glucose,insulin,HOMA-IR,BMI,and waist circumference height ratio were compared before and after treatment with metformin.[Results] There were 10 cases of NASH,including 5 boys and 5 girls.The short-term treatment of metformin reduced the levels of ALT,AST,and HOMA-IR for all 10 patients (P ＜ 0.01).ALT,gradually decreased with the course of treatment.Fasting insulin and waist circumference to height ratio also improved with the treatment (P ＜ 0.05);the changes of TG,BMI,and fast glucose were not obvious (P ＞ 0.05).[Conclusion] Metformin can effectively reduce liver enzymes and improve insulin sensitivity in children with NASH in short term,the improvement of TG and BMI in short term is not obvious.