Child ; Exostoses, Multiple Hereditary ; genetics ; Female ; Humans ; Mutation ; N-Acetylglucosaminyltransferases ; genetics ; Pedigree

Objective To discuss the surgical treatment for huge hepatoblastoma in children,and the technique of hepatectomy without blockade of the blood supply to the remained liver lobes.Methods We reviewed 12 cases of huge hepatoblastorna who had been operated from July 2001 to January 2009 in our hospital.The mean age of the children was 3.2 years(range,11 months to 12 years).The diameter of the tumor was from 10 to 23 cm.3～7 cycles of chemotherapy was routinely administrated before operation.When the tumor reduced to a certain size that radical resection could be performed safely,regular hepatectomy was conducted.Hepatoblastoma resection without blocking the blood supply to the remained liver lobes was performed in every patient.Results The operations were successfully accomplished in all the 12 children.5 cases received right trihepatectomy (segment Ⅳ,Ⅴ～Ⅷ),4 cases received right hemihepatectomy(segment Ⅴ～Ⅷ),and the other 3 cases received Ⅳ,Ⅶ,Ⅷ segmentectomy,right Ⅴ,Ⅵ segmentectomy,and left hemihepatectomy respectively.The intraoperative hemodynamic parameters were stable,and there was no perioperative mortality.Postoperative chemotherapy wag routinely administrated.The follow-up period varied from 2 to 92 months.11 children survived and were disease free,among those 6 children have survived for more than 5 years.One child had brain and lung metastasis 5 months post operation,and died 7 months post operation. Conclusion Preoperative chemotherapy administrated to children with huge hepatoblastoma can reduce the tumor size and render tumor reseetable.Hepatoblastoma resection without blocking the blood supply to the remained liver lobes is a safe and feasible surgical technique.

Objective To evaluate the safety of tubeless micro-percutaneous nephrolithotomy (MPCNL). Methods Forty patients who met the criteria(single percutaneous tract, no serious bleeding or perforation in the collecting system and absence of insignificant residual fragments under B-US)were included. After the operation of stone fragmentation, all patients were randomly divided into 2 groups. Twenty patients underwent standard MPCNL(group A), 16 eases had stones in kidney and 4 in upper ureter. Longitudinal sizes of calculi were 1.3-8. 6 era(mean 3.4 era). Twenty patients in group B were with tubeless procedures, 14 patients had stones in kidney and 6 in upper ureter. Longi-tudinal sizes of calculi were 1.1-7. 9 era(mean 3.1 era). Double J stents were placed in all eases. Changes of hemoglobins, pain and complications were recorded and ststistieally analyzed. Results There were no differences in stone size(P=0. 23), operation time (98±29 rain vs 92±31 min, P=0. 63) between the 2 groups. The changes of hemoglobin had no significant difference between 2 groups[(3.3±1.6)% vs (3.1±1.6)%, P=0. 49]. Postoperative pain was assessed using a visual analog scale. The scores of both groups on the first postoperative day were 3.6±1.8(group A) and 3.3±1.5(group B,P=0. 66). On the third day the scores were 1.6±1.2 and 1.8±1.3(P=0. 62). Both groups had 1 patient suffered from mild fever. No transfuaion was performed and no renal colic happened in 2 groups. Conclustion Under certain circumstance, tubeless MPCNL is a safe procedure for patients with urolithiasis.

Objective To observe the effect of remifentanil combined with propofol to induce and sustain controlled hypotension in children during endoscopic sinus surgery(ESS). Methods Forty ASA Ⅰ children undergoing adenoidectomy in ESS were divided into control group and controlled hypotension group by random digits table with 20 cases in each group. No controlled hypotension in control group. Anesthesia was induced with propofol,remifentanil and atracurium, and maintained with continuous infusion of propofol 2 min until the target mean arterial pressure (MAP)(55 - 60 mm Hg, 1 mm Hg = 0.133 kPa) was reached,and MAP was maintained at this level during operation in controlled hypotension group. During 15 min before surgical procedure pharynx nasalis blood flow was measured and recorded with laser Dopper flowmetry continuously. The quality of the surgical field in term of blood loss and dryness was established at 15 min after operation starting. Results Controlled hypotension was induced within (2.5 ± 0.3 ) min, the infusion rate ofMAP and heart rate at 15 min after controlled hypotension and 15 min after operation starting were significantly lower than those at controlled hypotension instantly in controlled hypotension group and control group (P ＜ 0.05 ). The pharynx nasalis blood flow decreased at 15 min after controlled hypotension from baseline [(68.3 ± 8.3 )% vs. (99.8 ± 7.9 )%] (P ＜ 0.05 ). The operation time and the quality of the surgical field in term of blood loss and dryness in controlled hypotension group were better than those in control group [(21 ± 4) min vs. (32 ± 6) min and ( 1.8 ± 0.1 ) scores vs. (3.5 ± 0.5) scores] (P ＜ 0.05 ). The awakeextubate time was within 10 min in two groups, and there were no anesthesia related complications.Conclusion Remifentanil combined with propefol can induce and sustain controlled hypotension,reduce pharynx nasal is blood flow and provide good surgical conditions in children for ESS.

A 65-year-old woman presented with intermittent right hand numbness and elevated serum creatinine for more than 2 months. The histological examination of kidney biopsy showed renal arterioles occlusion and interstitial fibrosis. Pathological abnormality was originally considered as a part of systemic atherosclerosis. Thus, rosuvastatin 20 mg/d, fosinopril 10 mg/d, metoprolol 47.5 mg/d and aspirin 0.1g/d were administrated. No improvement of renal function was seen. Further Congo red staining was applied. Diffuse amorphous eosinophilic substance was deposited in interlobular artery and small arteriolar artery. Combined with the abnormal free light chain (FLC) level and ratio (serum κ 340 mg/L, κ/λ 10.932), the diagnosis of systematic light-chain amyloidosis was confirmed. The patient received 3 courses of chemotherapy regimen as BCD (bortezomib 2 mg d1, 8, 15, 22, cyclophosphamide 0.3 g d1, 8, 15, 22 and dexamethasone 40 mg d1, 8, 15, 22). A hematologic partial response was achieved and serum creatinine decreased to 180 μmol/L.

OBJECTIVE: To investigate the value of combination of thyroid imaging reporting and data system (TI-RADS) and enhanced flow (e-Flow) in the qualitative diagnosis of thyroid nodules; and to compare the diagnostic efficiency between e-Flow and color doppler flow imaging (CDFI. METHOD: The study chose 133 patients with 179 nodules (127 benign, 52 malignant nodules) who had per-operative ultrasound examination of their thyroid using grey-scale ultrasound, CDFI, e-Flow, and their noudles were graded by TI-RADS. Then the ultrasound diagnostic value were compared with their surgical pathology results by TI-RADS; TI-RADS and CDFI; TI-RADS and e-Flow, and diagnostic efficiency between e-Flow and CDFI were compared. RESULT: The results revealed that combination of TI-RADS and e-Flow had a higher sensitivity (94.23%, P < 0.05). There was no statistically significant difference between TI-RADS and the combination of TI-RADS and CDFI. Likewise, there was no significant difference between the combination of TI-RADS and e-Flow and the combination of TI-RADS and CDFI. The e-Flow showed a higher sensitivity, specificity and accuracy compare to the CDFI. CONCLUSION Combining e-Flow and TI-RADS could improve the sensitivity in qualitative diagnosis of thyroid nodules. Also, e-Flow has a better diagnostic efficiency of thyroid nodules.
Diagnosis, Differential ; Diagnostic Imaging ; Humans ; Sensitivity and Specificity ; Thyroid Nodule ; diagnosis ; diagnostic imaging ; Ultrasonography

Objective To investigate the diagnostic value of 64-slice CT angiography (CTA) to head and neck artery pathological changes.Methods One hundred patients with clinical suspicion of head and neck vascular lesions were retrospectively analyzed.Sixty four cases of lesions were detected by 64-slice CTA,and were checked by Digital Subtraction Angiography (DSA) within a week.First,the correlation of 64-slice CTA and DSA diagnosis of head and neck artery stenosis grade was counted.Then DSA was taken as the gold standard.The sensitivity,specificity,positive predictive value,and negative predictive value of 64-slice CTA to diagnose head and neck arteries stenosis were calculated.Results Of 64 cases of patients,2 cases of congenital vascular dysplasia,including 1 case with internal carotid artery in 2 cases,the remaining 60 patients had different degree of stenosis.The sensitivity,specificity,positive predictive value,and negative predictive value of 64-slice CTA was 95.34％,99.48％,97.62％,and 98.98％,respectively.Among 60 narrow patients,the plaque number was 205,including 88 soft plaques,56 mixed plaques,and 61 calcified plaque.Conclusions The 64-silce CTA has multiple advantages including rapid,noninvasiveness,high accuracy and safety,and high application value in the diagnosis of head and neck vascular lesions.It is worth promoting.

A secretive expression vector of Pichia pastoris system which can be used for the direct cloning of PCR products was constructed,and was verified through the expression of recombinant cellobiohydrolase II in Pichia pastoris.A randomly selected fragment was amplified with properly designed primers by PCR.The XhoI and Eam1105Ⅰ restriction sites were included in the 5'end of the fragment,and the Eam1105Ⅰ and XbaI restriction sites were included in its 3'end.The PCR amplified product was inserted into the P.pastoris expression plasmid pPICZ?A through XhoI and XbaI restriction sites and the resultant plasmid was digested with Eam1105Ⅰ,and lastly the big fragment was recovered,generating the P.pastoris expressive Tvector pPICZ?T.Then the cellobiohydrolase II of T.reesei was successfully expressed in P.pastoris with this expressive Tvector.Such results indicated that the constructed expression Tvector was convenient for PCR product cloning,and was effective for heterologous protein expression in P.pastoris.On the other hand,the application of the expression Tvector avoided the introduction of additional amino acids at the Nterminus of the expressed protein,which generally occurred when normal expression vectors were used in secretive expression system.

Objective To investigate the effect of reserpine,an efflux pumps inhibitor,on the activities of fluoroquinolones (FQNs) against Enterococci,and the distribution of efflux pump genes emeA and its correlation with the resistance of Enterococci.To elucidate the relationship between FQN resistance in Enterococci and active efflux.Methods One hundred isolates of enterococci were identified by VITEK microbe automatic system.The antibacterial agent susceptibility tests were performed by the disc diffusion method (K-B) in accordance with the CLSI standards.The minimum inhibitory concentration (MIC) of each FQN was tested by the agar dilution method,and the MIC changes were detected after adding reserpine.The distribution of emeA in 100 isolates of Enterococci was determined by PCR.Thex2 test was used to compare the differences of statistical results.Results After reserpine was used,three-FQN resistance in Enterococci was reduced.Ciprofloxacin,gatifloxacin and levofloxacin resistance was reduced from 42％ (42/100) to 28％ (28/100),from 30％ (30/100) to 17％ (17/100),and from 33％ (33/100) to 23％ (23/100),respectively.The positive rate of emeA gene in 100 strains of Enterococci was 55％ (55/100).There were 45 positive strains(72.6％) in 62 E.faecalis and 10 positive strains (26.4％) in 38 E.faecium.The positive rate of emeA gene in the resistant strains against ciprofloxacin,gatifloxacin and levofloxacin was 73.8％ (31/42),76.7％ (23/30),75.8％ (25/33),respectively,and the positive rate of emeA gene in the susceptible strains against above 3 antibacterials were 41.4％ (24/58),45.7％ (32/70),44.8％ (30/67).Efflux pump genes emeA in resistant strains is higher than the sensitive strains,with the statistically significant difference(x2 =13.02,8.13 and 8.57,P ＜ 0.005).Conclusions Reserpine could inhibit the active efflux of in FON Enterococci and reduce the MIC for drug-resistant strains in vitro.Multidrug-resistant efflux pump gene emeA was relevant to antimicrobial drug resistance in Enterococci.

OBJECTIVE To investigate the distribution of bacteria detected from blood culture of pediatric patients and to observe the antibiotic resistance of these bacteria. METHODS The BacT/Alert blood culture system was applied for culture.Species identification and antibiotic resistance tests were performed by the VITEK automicroscan system. RESULTS Coagulase-negative staphylococcus(CNS) and Staphylococcus aureus accounted for 67.2% and 7.8%, respectively.The proportion of Gram-negative bacteria was 14.4%.The resistant rate of CNS and S.aureus to oxacillin was 85% and 36%,respectively.The strains producing the extended spectrum beta-lactamases(ESBLs) of Gram-negative bacteria were often detected. CONCLUSIONS Gram-postive bacteria are the main pathogens detected from the blood culture of pediatric patients.Detection rate of CNS is the highest.Multiresistant strains are common.