Objective To investigate the clinical significance of gamete intrafallopian transfer (GIFT) under laparoscope. Methods The gonadotropin was adopted to induce superovulation and the spermatic fluid was managed with the swim-up method. After oviums were dislodged by puncture under laparoscope, gametes were transplanted by intubating the fallopian tube. Results Among a total of 19 patients, 4 were pregnant, the pregnancy rate being 21.1% (4/19). Three women had a delivery at term and 1 had an abortion. No serious complications were seen. Of the 15 unfertilized patients, normal pregnancy was observed within 6 months after surgery in 5 patients, the circle pregnancy rate being 5.6% (5/89). Conclusions GIFT under laparoscope is a practical assisted reproductive technique (ART) that may achieve a relatively high pregnancy rate. Some other pelvic diseases resulting in infertility can be treated simultaneously, which contributes to natural fertilization in the future.

Objective To study the clinicopathological features and prognosis of Gastric hepatoid adenocarcinoma (GHA) and aims to guide future clinical practice.Method We retrospectively studied the clinical data of 25 patients with pathologically confirmed GHA who were admitted to our hospital from January 2003 to December 2015.Results There were 19 males and 6 females.The average age was 61.2 years.The clinical manifestations were non-specific.Pathologically,majority of GHA presented with ulcerative type,poor differentiation and extensive vascular cancerous embolus.Preoperative liver metastases were found in 12 patients,and postoperative liver metastases in 15 patients.Conclusions GHA is a special primary gastric adenocarcinoma which possesses both hepatocellular carcinoma-like and adenocarcinoma-like histology.GHA is characterized by a significantly elevated serum AFP and early hepatic metastasis.GHA is therefore often misdiagnosed as primary hepatocellular carcinoma.The main treatment option remains to be surgical resection,and the prognosis is poor.

The effects of Lipoid of Mujingzi〔LP,Fruits of vitex neguando L Var cannabifo- lia (Sieb et Zucc) Hand mazz〕 on Smooth muscles are reported. The results were as follows:① In the isolated perfused mice lung-bronchi, LP caused significant increase of perfusion medium dropping through the lung-trachea, and the effect of LP was dose-dependent; ACh (Acetyl-choline)-induced contraction of the isolated mice trachea smooth muscles could be relaxed by LP. ② When LP was given introperitoneally(100~ 300 mg?kg-1) into guinea pig, the incubationperiod of asthma induced by acetycholine together with histamine was prolonged.The optimum dilated effect of LP on smooth muscles of trachea in mice can be concluded as the results of synergistic effects by 4 compositions from LP (Oleic acid,Linoleic acid, Palmitic acid and Stearic acid).

Objective To investigate the effect of selective bowel decontamination (SBD) on prognosis of 90％ hepatectomy in rats.Methods We adopted rat model of subtotal hepatectomy(90％,SHx),gentamicin + polymyxin + nystatinor saline of the same amount was administrated preoperatively.Liver damage makers,portal and systemic lipopolysaccharide(LPS),mucosal damage,signaling pathways and liver regeneration were investigated.Results We found that SHx resulted in significantly enhancedsystemic LPS.Inhibition of gastrointestinal gram-negative bacteria by SBD significantly reduced LPS levels and improved survival after SHx.SBD protected intestinal mucosa barrier,alleviated liver parenchymal damage and inflammation and promoted liver regeneration.Conclusion SBD is beneficial and necessary for extended heptactomy.

Objective To investigate the prevalence,clinical classification,treatment and prognosis of neonatal hyperphenylalaninemia(HPA) in Xuzhou area,China.Methods Infants born between July 1,2003 and July 1,2015 in Xuzhou area were investigated.Heel blood samples of neonates were collected at 72 hours after birth,and the concentration of blood phenylalanine(Phe) was determined by fluorescent quantitative method in Xuzhou Maternity and Child Health Care Station Neonatal Disease Screening Center.Differential diagnosis was performed in all 265 cases diagnosed as HPA by urinary pterin analysis and dihydropteridine reductase activity determination.The blood Phe concentration and mental development were followed up regularly in infants with HPA.Mutations of phenylalanine hydroxylase (PAH) gene were analyzed by gene sequencing.The relationship between blood Phe concentration and mental development was analyzed by Bivariate correlation analysis.Results (1) The prevalence of HPA in neonates in Xuzhou was 1/4 635.Among the 265 confirmed HPA cases,260 cases(98.11％) had PAH deficiency,including 90(33.96％) classical phenylketonuria(PKU),84(31.70％) mild PKU and 86(32.45％) mild HPA.The other five patients(1.89％) diagnosed with tetrahydrobiopterin (BH4) deficiency all had 6-pyruvoyl tetrahydropteim synthase(PTPS) deficiency.(2) Among the 265 HPA cases,26 cases refused any treatment,including five cases of PTPS deficiency and 21 cases of PKU.Of the five patients with PTPS deficiency,two died and the other three had normal mental and physical development.Twenty-one PKU patients who refused treatment had mental retardation of various degrees.Among 153 PKU patients who received medical treatment,three died and 12 were lost to follow-up.(3) For 138 PKU patients who received dietary treatment and follow-up,the ages at the last visit were two months to 12 years,116 of them had normal mental development,the remaining 22 patients had mental retardation,and a negative correlation was observed between mental development and the average Phe concentration.(4) Thirty-five patients with PAH deficiency underwent gene sequencing,and 22 kinds of mutations of PAH gene were detected.Conclusions The prevalence of HPA in Xuzhou area is higher than the average national level.With early diagnosis and standard treatment,most of PKU neonates can have normal mental development.Phe level control is an important factor for mental development.

Gastric hepatoid adenocarcinoma (GHA) is a rare but important sub-type of gastric adenocarcinoma characterized by high serum α-fetoprotein,early lymphatic and hepatic metastasis,and poor prognosis.Clinically,the presentation could be atypical,liver neoplasm could be the initial finding.With similar clinicopathological presentation as hepatocellular carcinoma (HCC),prompt and correct diagnosis can be a challenge,especially in endemic areas with a high incidence of HCC.Once diagnosed,surgical removal remains the treatment of choice.This review focus on advancement on the biological,histological and immunohistological features,and the clinicopathological presentation of GHA.

Objective To study the role of mesenchymal stem cells (MSC) in an animal model combining ischemia-reperfusion with 85％ liver resection.Methods Eight-week-old male SD rats received BM-MSC by tail vein and then underwent 30-min ischemia followed by 85％ liver resection.The survival rate was monitored for 7 days after surgery.Liver regeneration was assessed on day 2 after hepatectomy.Liver damage,liver cell apoptosis,and cytokine expression in the first 24 h after hepatectomy were also assessed.Results BM-MSC mostly homed to the spleen.Transplantation significantly inhibited myeloperoxidase [(19.9 ± 6.0) mg/g vs.(41.4 ± 10.2) mg/g] and downregulated proinflammatory cytokines.BM-MSC significantly reduced the ALT and AST levels [AST (1 475 ± 275) IU/L vs.(2 550 ± 441) IU/L,P ＜ 0.05;ALT (738 ± 101) IU/L vs.(1 113 ± 268) IU/L,P ＜ 0.05].The attenuation of liver injury was also verified histologically 24 h after surgery.Liver cell apoptosis was markedly reduced.Moreover,BM-MSC infusion significantly promoted remnant liver regeneration.As a result,the survival rate was improved by BM-MSC treatment in this model (95％ vs 70％,P ＜ 0.05).Conclusion In an animal model combining ischemia-reperfusion with 85％ liver resection,BM-MSC infusion attenuated liver injury and promoted hepatocyte regeneration,resulting in improved survival rate.

Objective: To estimate the levels of free carnitine and acylcarnitine in neonates, and summarize the incidence and clinical characteristics of carnitine absorption deficiency in Xuzhou. Methods: Between November 2015 and December 2017, 216 903 newborns were recruited with carnitine absorption deficiency screened via tandem mass spectrometry in Xuzhou.They were divided into different groups according to gestational age, birth body weight, blood collecting time and season, in which the group with gestational age <37 weeks was selected as the premature delivery group, and the group with gestational age 37-41^{+ 6} weeks as the normal gestational age group for gestational age analysis, while the group with the birth body mass <2 500 g was selected as low birth body mass group and the group with the birth body mass of 2 500-3 999 g as normal birth body mass group for body mass analysis.SPSS 16.0 software was used for data analysis to clarify the influence of the above factors on the detection of carnitine indexes by tandem mass spectrometry.DNA sequencing was performed to confirm the diagnosis and analyze the relevant pathogenic genotypes in children with positive screening, and these confirmed individuals were followed up. Results: There was no statistical difference in the levels of C3, C8 and C102 between preterm infants and normal body mass infants in the gestational age group(all P>0.05), but other carnitine levels had statistically significant differences(all P<0.05). The difference of C102 level between the different birth weight groups was not statistically significant(P>0.05), but that of other carnitine levels were statistically significant(all P<0.05). There was no significant difference in the level of C182 between different blood collection time(P>0.05). The levels of free carnitine and acylcarnitine between the different season groups had statistically significant differences(all P<0.05). Primary carnitine deficiency was diagnosed in 10 cases, including 7 cases of maternal carnitine absorption deficiency.The incidence in Xuzhou was approximately 121 690.The pathogenic genotype showed a specific regional distribution characteristic in Xuzhou, in which pathogenic mutation type c. 1400C >G was the most common one. Conclusions Some factors play a role in neonatal screening for carnitine absorption deficiency by tandem mass spectrometry including gestational age, birth body weight, blood collecting time and season.Pathogenic genes present a regional characteristic in Xuzhou and maternal carnitine absorption deficiency with a higher rate, the clinical attention should be paid to screening for maternal carnitine absorption deficiency.

Objective To investigate the characteristics of neonatal methylmalonic aciduria (MMA)regarding clinical manifestations,laboratory findings,gene mutations,treatments and prognosis.Methods Acylcamitine levels in blood samples of 207 308 neonates born from January 2016 to December 2017 in Xuzhou were detected by liquid chromatography tandem mass spectrometry and the abnormal results were further confirmed by detecting organic acids in urine samples with gas chromatography-mass spectrometry and gene sequencing analysis.Patients with isolated MMA were treated with dietary control and levocarnitine,while those complicated by homocysteinemia were treated with vitamin B12,levocarnitine,glycine betaine and calcium folinate.Clinical manifestations,laboratory findings,imaging features,genotypes,treatments and prognosis of patients with MMA were retrospectively analyzed.Paired sample t-test was applied for statistical analysis.Results MMA was eventually diagnosed in 12 patients,among which three were isolated MMA and nine were complicated by homocysteinemia.The three isolated MMA cases failed to response to vitamin B12 treatment without any symptoms on diagnosis.However,vitamin B12 was effective for the other nine patients,among which four had no clinical symptoms on diagnosis and five had manifestations such as slow response,recurrent vomiting,poor feeding,dyspnea,anemia and jaundice.Abnormal results of cranial MRI included bilateral basal ganglia damage,enlarged extracranial space,ventriculomegaly and changes in white matter.All patients underwent genetic analysis and three were found with MUT gene mutations and nine with MMACHC gene mutations.MUT gene mutations were classified into five types,including c.I106G＞A,c.1880A＞G,c.441T＞A,c.581C＞T and c.1741C＞T.Eight types of MMACHC gene mutations were identified,including c.609G＞A,c.658_660delAAG,c.482G＞A,c.1A＞G,c.567dupT,c.80A＞G,c.276+1G＞A and c.228_23 l delTGAC.Two mutations,c.276+lG＞A and c.228 23 ldelTGAC,were novel mutations.The most common mutation in MMACHC gene was c.609G＞A,followed by c.658_660delAAG and c.482G＞A.One of the isolated MMA patients died after refusing treatments and the other two showed significant decrease in serum propionylcarnitine,propionylcarnitine to acetylcarnitine ratio,serum homocysteine and methylmalonic acid and methylcitric acid in urine after treatment.Moreover,of the two patients who were alive at follow-up,one experienced normal growth and development and the other suffered from growth retardation.The ratio of propionylcamitine to acetylcarnitine and the levels of serum propionylcarnitine,serum homocysteine and methylmalonic acid and methylcitric acid in urine were significantly decreased in the nine patients with MMA complicated by homocystinuria after one month of treatment [0.88±0.35 vs 0.13±0.05,(7.12±1.90) μ mol/L vs (3.18±1.08) μ mol/L,(136.48±38.14) μ mol/L vs (34.41±17.33) μmol/L,103.51±69.62vs 5.35±2.15 and 7.95±6.88 vs 1.02±0.48,t=-6.166,-6.687,-12.941,-4.208 and-3.015,respectively,all P＜0.05].Two deaths,three asymptomatic and four psychomotor retardation patients were reported during follow-up.Conclusions Newborn screening with liquid chromatography tandem mass spectrometry is important for early diagnosis of MMA.MMACHC gene defects are the main causes of MMA in Xuzhou area and the predominant one is c.609G＞A mutation.Prognosis of MMA might be related to disease type,age of onset and patient's reactivity to vitamin B12.

Objective To investigate the clinical features and SLC22A5 gene mutation types in patients with primary carnitine deficiency(PCD).Methods The free carnitine(CO) and acylcarnitine levels in the blood of 210 908 neonates from newborn screening program and 576 children with suspected clinical inherited metabolic diseases were measured by using liquid chromatography tandem mass spectrometry method during September 2015 to December 2017,after that the SLC22A5 gene mutations were analyzed in the children with low CO level and the diagnosis was made.The clinical characteristics,laboratory findings,genotypes,treatment and prognosis were retrospectively analyzed in patients.Paired sample t test was used to compare the biochemical indexes of patients before and after the treatments.Results Ten children were diagnosed with PCD(9 cases from newborn screening program,1 case from clinical patients),and 7 children were diagnosed with maternal carnitine deficiency.After treatment with oral Levocarnitine,the free carnitine and acylcarnitine of the patients returned to the normal levels.The clinical symptoms disappeared in 1 patient out of clinical patients,and the other 16 patients from newborn screening program were asymptomatic and showed normal growth and development.Seventeen patients got genetic analysis,and 10 types of mutations were found,including c.1400C ＞ G,c.1462C ＞ T,c.797C ＞ T,c.95A ＞ G,c.92C ＞ T,c.1093A ＞ C,c.761G ＞ A,c.865C ＞ T,c.428C ＞ T,c.1195C ＞ T,among which two of them (c.1093A ＞ C and c.92C ＞ T) were novel mutations.The most common mutation of SLC22A5 gene was c.1400C ＞ G.Conclusions Liquid chromatography tandem mass spectrometry technology is sufficient to screen newborns and maternal carnitine deficiency,and the c.1400C ＞ G mutation is found at the highest frequency in Xuzhou area.If patients receive early treatment,they may have a good prognosis.