Biliary stricture after cholecystectomy poses difficult management problems to surgeons because of high and stable incidence.In contrast to malignant stricture,benign stricture requires durable repair.Repeated operations may not only increase the suffering of the patient,but also reduce the likelihood of a better outcome. A 56-year-old woman with biliary stricture after cholecystectomy who had undergone several operations in other hospitals was admitted to Chinese PLA General Hospital.Computed tomography (CT) scan showed a dilated biliary tree and localized the level of ductal obstruction in the hepatic hilar stricture.In addition,CT identified fluid collections in the left upper quadrant and no artery injury was detected. Ultrasound-guided percutaneous abdominal drainage was performed to control the abdominal infection. Magnetic resonance cholangiopancreatography classified the injury as Bismuth Ⅲ.The patient with bile leakage and severe abdominal infection was treated with antibiotics before the final operation.On June 1,2012,the patient received Roux-en-Y hepaticojejunostomy.After operation,the patient recovered smoothly without severe complications,such as bile leakage,cholangitis and recurrent stricture.Liver function of the patient was back to normal and T tube drainage was pulled out at the end of 3 months of follow up.

Gluconobacter oxydans is known to oxidize glucose to gluconic acid (GA), and subsequently, to 2-keto-gluconic acid (2KGA) and 5-keto-gluconic acid (5KGA), while 5KGA can be converted to L-(+)-tartaric acid. In order to increase the production of 5KGA, Gluconobacter oxydans HGI-1 that converts GA to 5KGA exclusively was chosen in this study, and effects of carbon sources (lactose, maltose, sucrose, amylum and glucose) and nitrogen sources (yeast extract, fish meal, corn steep liquor, soybean meal and cotton-seed meal) on 5KGA production were investigated. Results of experiment in 500 mL shake-flask show that the highest yield of 5KGA (98.20 g/L) was obtained using 100 g/L glucose as carbon source. 5KGA reached 100.20 g/L, 109.10 g/L, 99.83 g/L with yeast extract, fish meal and corn steep liquor as nitrogen source respectively, among which the optimal nitrogen source was fish meal. The yield of 5KGA by corn steep liquor is slightly lower than that by yeast extract. For the economic reason, corn steep liquor was selected as nitrogen source and scaled up to 5 L stirred-tank fermentor, and the final concentration of 5KGA reached 93.80 g/L, with its maximum volumetric productivity of 3.48 g/(L x h) and average volumetric productivity of 1.56 g/(L x h). The result obtained in this study showed that carbon and nitrogen sourses for large-scale production of 5KGA by Gluconobacter oxydans HGI-1 were glucose and corn steep liquor, respectively, and the available glucose almost completely (85.93%) into 5KGA.
Bioreactors ; Carbon ; chemistry ; Culture Media ; chemistry ; Fermentation ; Gluconates ; metabolism ; Gluconobacter oxydans ; metabolism ; Industrial Microbiology ; Nitrogen ; chemistry

Objective To evaluate the clinical efficacy of super-selective prostatic arterial embolization(PAE) for the treatment of benign prostatic hyperplasia(BPH).Methods From February 2012to March 2015,a total of 17 patients with BPH who failed in medical treatment,or unwilling to accept surgery were selected for PAE as the study group.The mean age was 73 years (range 61-84 years) and the mean prostatic volume was 64.6 ml (ranging 50-85 ml).The study group underwent super selective arterial embolization.The internal iliac artery angiography was performed and the main blood vessel of prostate was showed.The femoral artery was punctured under local anesthesia and X-ray monitoring,a F4-5 Cobra catheter was inserted,and then the Embosphere microspheres were implanted.A total of 40 patients who underwent transurethral resection of the prostate(TURP) were selected as the control group.The mean age was 70 years (ranging 59-87 years).The mean prostatic volume was 68.7 ml(ranging 55-90 ml).All cases were followed up for 1 year.The changes of prostatic volume (PV),international prostate symptom score(IPSS),quality of life (QOL),pre-and post-treatment peak urinary flow (Qmax) were evaluated.Results For the 17 patients who underwent PAE,the PV decreased from (64.6 ± 10.2) ml to(42.0 ± 7.5) ml,the IPSS decreased from 23.9 ±4.9 to 13.1 ±3.5,QOL decreased from 4.1 ±0.7 to 2.1 ±0.7,and Q increased from (9.5 ± 3.7) ml/s to(21.8 ± 4.2) ml/s,which were statistically significant (P ＜ 0.05) compared with the pre-treatment parameters.The post-operative parameters of the control group were also significantly improved compared with the preoperative parameters (P ＜ 0.05).Conclusions PAE is safe and effective in treating BPH,especially for those failing in medical treatment,or unwilling to accept surgery.

Benign biliary stricture is a challenging problem in hepatobiliary surgery. Benign biliary stricture is associated with major portal vein variation, which is not be found in literatures. A male patient with benign biliary stricture was admitted to the Chinese PLA General Hospital in March, 2010.The stricture was located in the hilar confluence with intrahepatic biliary dilation and hepatolithiasis. The result of computed tomography showed that the hilar biliary confluence was compressed by the left portal vein and right anterior portal vein. The patient was cured after receiving gallbladder interposition, choledocholithotomy and T tube drainage. We suggested that the benign hilar biliary stricture due to portal vein variation may be named as biliary nut-craker syndrome.

Objective To evaluate surgical treatment for different types of tumor of the suprarenal infrahepatic segment of the inferior vena cava (IVC).Methods A retrospective analysis was made on 8cases of IVC leiomyosarcoma at the suprarenal infrahepatic IVC segment.According to the extent of tumor invasion and collateral circulations,operations included tumor resection plus inferior vena cava reconstruction or tumor resection plus right nephrectomy.Results Tumors were completly resected in all patients.Renal function recovered after operation.During a follow up of 24 mons,7 cases had no recurrence.One case with recurrence after 12 months underwent successful resection and is doing well after a follow-up of 12 months.Conclusion CT and inferior vena cavography can determine the extent of tumor invasion and collateral circulations helping select appropriate surgical aproach before the operation resulting in less renal injury.

Objective:To investigate the clinical characteristics and pathogenic mutations of propionic acidemia.Methods:Clinical data of two patients with propionic acidemia admitted to the Obstetrics and Gynecology Hospital of Nanjing Medical University from May 2017 to June 2018 were collected. Genomic DNA was extracted from the peripheral blood of the patients and their parents. Inherited disease panel based on Ion Torrent semiconductor sequencing technology was performed to detect gene mutations, and those with suspected pathogenic mutations were verified by Sanger sequencing. Descriptive statistical analysis was used for data analysis.Results:Case 1 was suspected of sepsis and admitted to the Obstetrics and Gynecology Hospital of Nanjing Medical University due to "drowsiness and milk rejection" on the second day after birth. Tandem mass spectrometry suggested the level of propionyl carnitine and its ratios to acetylcarnitine and free carnitine were increased. Urine gas chromatography-mass spectrometry showed elevated 3-hydroxypropionic acid and methylcitric acid. Genetic analysis revealed that the infant carried c.331C>T (p.R111X)/c.1228C>T (p.R410W) compound heterozygous mutations in the PCCB gene. The infant was diagnosed with propionic acidemia and treated with a special diet with an L-Carnitine supplement but died of sudden coma and vomiting without precipitating factors at three months of age. Case 2 presented with sudden vomiting, drowsiness, and anergia on the admission at five-months old. Tandem mass spectrometry showed increased propionyl carnitine level and its ratios. Compound heterozygous mutations of c.146delG (p.G49EfsX16)/c.1253C>T (p.A418V) in the PCCB gene were identified in the patient, of which c.146delG (p.G49EfsX16) was a de novo mutation and was evaluated as a pathogenic mutation. The patient was on a special diet with an L-Carnitine supplement, but with disobedience. Followed up to the age of three years and eight months, the child was severely underdeveloped. Conclusions:Neonates with clinically suspected sepsis may have propionic acidemia, and tandem mass spectrometry and genetic testing should be performed as soon as possible to confirm or rule out the diagnosis. Further investigations on the pathogenesis and function of the new mutation are still needed.

Objective:To investigate the clinical features, treatment methods of metastatic liver leiomyosarcoma (MLL).Methods:The characteristics of 5 cases of MLL were reviewed and summarized. Among them, 4 cases were derived from retroperitoneal leiomyosarcoma and 1 case was derived from uterine leiomyosarcoma. In terms of metastasis time, 1 case was simultaneous liver metastasis and 4 cases were heterochronous liver metastases, all of which were more than 2 years away from the primary tumor resection.Results:One patient died within 2 years after the diagnosis of MLL, and this patient had not undergone surgical treatment for liver metastases. Of the 4 patients who had had the metastasis resection, 2 patients died within 3 years. Currently, 2 patients are alive, 1 patient after 36 months, and 1 patient after 29 months.Conclusion:MLL occurs mostly years after the primary tumor is removed. Currently, surgical treatment is considered to be the main treatment method for MLL, which can significantly prolong the patients survival.

Gastric schwannoma is a tumor originating from mesenchymal tissue. The clinical incidence is relatively rare, accounting for 6.3% of all gastric stromal tumors. The tumor is more likely to occur in the body of the stomach and usually originates from the gastric submucosal nerve. Most gastric schwannomas do not have any clinical symptoms. Imaging examination can play a diagnostic role, but the diagnosis still requires pathological examination, especially S-100 protein which is the gold standard for the diagnosis of gastric schwannomas. Gastric schwannoma usually needs to be distinguished from gastrointestinal stromal tumors and gastrointestinal autonomic nerve tumors. In terms of treatment, complete surgical resection is the first choice.

Objective:To analyze the detection of neonatal inherited metabolic diseases in Nanjing.Methods:We researched the results of 175 767 newborns by tandem mass spectrometry from December 2013 to July 2018. Amino acids, acylcarnitines, and succinylacetone were detected by non-derivatized tandem mass spectrometry to screen the abnormity of newborn amino acid, organic acid, or fatty acid oxidation metabolism disease. Gene panels based on high throughput sequencing technology were carried out to detect gene mutation of positive neonates. Descriptive statistics were used to analyze all the data.Results:The positive rate of primary screening was 2.1% (3 691/175 767), 3 598 of 3 691 positive cases were recalled. At last, 62 cases of the inherited metabolic disease were diagnosed. Among them, there were 35 cases of amino acid metabolism disease, 12 cases of organic acid metabolism disorder, and 15 cases of fatty acid metabolism defect. The total incidence of neonatal inherited metabolic disease was 0.035 3%, among which amino acid metabolic diseases were 0.019 9%, organic acid metabolic diseases were 0.006 8%, and fatty acid metabolic diseases were 0.008 5%. The diseases with the highest incidence were phenylalanine hydroxylase deficiency (0.015 9%), methylmalonic acidemia (0.005 1%), and primary carnitine deficiency (0.005 1%). Among 62 children, 51 (82.2%) were diagnosed by gene diagnosis (including 17 cases of phenylalanine hydroxylase deficiency and 34 cases of other inherited metabolic diseases). Another 11 children with phenylalanine hydroxylase deficiency refused gene diagnosis. Two pathogenic mutations were found in 17 children with phenylalanine hydroxylase deficiency. Two pathogenic mutations were found in 29 of the other 34 children with inherited metabolic disease, which were from their parents, while only one pathogenic mutation was found in the other five children, of which two cases with hypermethioninemia were autosomal dominant inheritance.Conclusions:Neonatal inherited metabolic diseases with high incidence in Nanjing are phenylalanine hydroxylase deficiency, methylmalonic acidemia, and primary carnitine deficiency. Some cases screened by tandem mass spectrometry only showed abnormal screening indicators. No specific clinical symptoms were found during follow-up, and further follow-up was needed.

OBJECTIVE: To analyze the clinical features and genetic variants in four neonates with very long chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency. METHODS: Neonates with a tetradecenoylcarnitine (C14:1) concentration at above 0.4 μmol/L in newborn screening were recalled for re-testing. Four neonates were diagnosed with VLCAD deficiency by MS-MS and genetic testing, and their clinical features and genotypes were analyzed. RESULTS: All cases had elevated blood C14:1, and the values of first recalls were all lower than the initial test. In 2 cases, the C14:1 had dropped to the normal range. 1 case has remained at above 1 μmol/L after the reduction, and the remainder one case was slightly decreased. In total eight variants of the ADACVL genes were detected among the four neonates, which included 5 missense variants and 3 novel variants (p.Met344Val, p.Ala416Val, c.1077+6T>A). No neonate showed salient clinical manifestations. CONCLUSION Above findings have enriched the spectrum of ADACVL gene mutations and provided a valuable reference for the screening and diagnosis of VLCAD deficiency.
Acyl-CoA Dehydrogenase/genetics* ; Acyl-CoA Dehydrogenase, Long-Chain ; Congenital Bone Marrow Failure Syndromes ; Genetic Testing ; Humans ; Infant, Newborn ; Lipid Metabolism, Inborn Errors ; Mitochondrial Diseases ; Muscular Diseases ; Tandem Mass Spectrometry