Objective To study the arterial portography features and the impact on surgical treatment of children′s cavernous transformation of the portal vein (CTPV). Methods Angiographic findings of the arterial portography of 6 children with CTPV and its impact on surgical treatment were retrospectively analyzed. Results Abrupt occlusion of the portal vein at the porta hepatis was revealed in 5 out of 6 cases, and a masslike network of intertwined veins around the porta hepatis and many small irregular veins radiating from the network to the liver were demonstrated. Both hepatopetal and reverse flow of collateral venous pathways were defined. Valuable information of correlative vessels for surgical treatment was obtained from all 6 angiographic findings. ConclusionChildren′s CTPV have very characteristic angiographic findings, which can be very helpful for surgical management.

Objective To investigate the clinical features and bronchoalveolar lavage (BAL)therapy of postinfectious bronchiolitis obliterans (BO) in children. Method Ten children, who had post-infectious BO from February 2009 to February 2010, received BAL therapy, and were retrospectively analyzed. The data included pathology,chnical feature,chest HRCT scan, BALF cellular, levels of blood T cell subtypes and outcome of BAL therapy. Results Adenoviruses or mycoplasma pneumoniae were the most common etiologic agents (4/10, respectively). All patients presented persistent or recurrent dyspneic respirations and wheezing since the initial lung infection. The findings of HRCT included mosaic pattern of perfusion (6/10), accompanied by gas retention,bronchiectasis, atelectasis and bronchial wall thickening. The percentage of neutrophils in BALF was significantly increased in all cases (10/10). There were predominance of CD8+ T cell subtype (9/10) and lower ratio of CD4 +/CD8+ ( 10/10)in blood. Reduced symptoms and shortened hospital stay of BO in 9 of all 10 cases were observed after BAL therapy. Conclusions Severe adenovirus or mycoplasma pneunoniae bronchiolitis and/or pneumonia has higher risk for developing BO in children. Increased percentage of neutrophils in BALF and predominance of CD8 +T cell subtype may play an important role in the mechanism of BO. BAL therepy can reduce the respiratory symptoms of BO in children.

Objective To analyze the clinical epidemiology of primary hepatocellular carcinoma (PHC) in the Xinjiang region. Methods Clinical data of the patients with PHC were collected at First Affiliated Hospital of Xinjiang Medical University from 5 577 cases from January 2002 to December 2014, their gender, race, age, household distribution, hepatitis virus-positive rate were analyzed retrospectively. Results Among the 5 577 eases, the men/women gender ratio was 3.45∶1;the proportion of Han, Uighur, Kazakh, and other ethnic groups (Hui, Mongolian, Manchu, Xibo) was 79.67%, 9.86%, 4.55%, 3.31%and 2.61%, respectively. The Constituent ratio difference between Uighur and Han was significant (P<0.05);4 232 patients had hepatitis B surface antigen (HBsAg) detection, and 3 833 patients had HCV antibody (HCV-Ab) detection. HBsAg was positive in 2 560 cases (60.49%), HCV-Ab was positive in 490 cases (12.78%). Hepatitis B virus detection positive rate in Uygur was 35.52%, in Kazak was 40.00%, which was lower than the Han's (65.68%, P<0.05). Urban and rural population had 3589 cases (64.35%) and 1988 cases (35.65%). Conclusion An increased risk for PHC was found in hepatitis virus-positive patients, the Xinjiang Uygur and Kazak people had significantly lower prevalence of HBV infection than the Han's. Appropriate measures should be taken for clinical diagnosis, treatment and prevention of PHC.

Objective To investigate the relative percentage of normal cognitive function (NCF),age associated memory impairment (AAMI),mild cognitive impairment (MCI) and Alzheimer's disease (AD) in the elderly,and the correlation between cognitive function and ApoE genotypes.Methods A total of 2666 elderly people aged ≥65 years (2132 males and 534 females)were divided into 3 groups according age:65-74-year age group (925 cases),75-84 year age group (1054 cases) and 85-100-year age group (687 cases).ApoE genotypes were determined in the controls and patients with AAMI and MCI.The degrees of fundus arteriosclerosis were detected in all subjects except for patients with AD.Results There were 867 cases with NCF,860 cases with AAMI and 782 cases with MCI.The incidence of AAMI was higher in 65-74-year age group than in the other two groups (42.0％ vs.31.1,20.96).The incidences of MCI and AD were higher in 85-100-year age group than in the other groups (42.5％,13.3％).The major degrees of fundus arteriosclerosis were Ⅰ+,Ⅰ-Ⅱ°,Ⅱ in subjects with AAMI (34.7％,x2=10.02,P＜0.01) and were Ⅱ °/ Ⅱ + / Ⅲ° in subjects with MCI (34.9 ％,x2 =23.39,P＜0.001).The APOEε4 allele frequency was significantly higher in patients with MCI than in the controls (x2=8.31,P＜0.05).However,no significant differences in APOEε4 allele frequency were found between patients with AAMI and the controls.Conclusions The incidence of AAMI is highest in 65-74-year age group,while the incidences of MCI and AD are highest in 85-100-year age group.Compared to patients with AAMI,the more serious fundus arteriosclerosis and higher allele frequency of APOEε4 appear in patients with MCI.

Objective:To investigate the killing effect of radiation on echinococcus in vitro culture and its effect on the mRNA expression of growth arrest and DNA damage 45 alpha (Gadd45α) gene. Methods:Echinococcus from naturally infected sheep liver was cultured in vitro and divided into 7 groups. The echinococcus was irradiated with 6 MeV at doses of 0 (control), 20, 40, 60, 80, 100, and 120 Gy, respectively. The growth of echinococcus was observed under light microscope at 1, 3, 5 and 7 d after the radiation. The expression of Gadd45α mRNA in control, 20, 40 and 60 Gy groups of echinococcus was detected by RT-PCR technique at 7 d after the radiation. Results:The disintegration and exfoliation of echinococcus were observed under light microscope at 1, 3, 5 and 7 d after the radiation, and the death rate of echinococcus was positively correlated with the radiation dosages ( r = 0.81, P < 0.05). After the radiation at 7 d, compared with the control group (100.00 ± 0.00), the mRNA expression levels of Gadd45α in echinococcus of 20, 40, and 60 Gy groups were significantly increased (279.74 ± 80.08, 759.38 ± 160.98, 1 666.68 ± 316.36, P < 0.01), and it was positively correlated with the radiation dosages ( r = 0.93, P < 0.01). Conclusion:Radiation has a certain killing effect on echinococcus cultured in vitro, and there is a certain dose-effect relationship with the radiation dosages, and Gadd45α gene may be involved in the molecular mechanism of radiation-induced killing of echinococcus in vitro.

Objective:To investigate the clinical features and outcomes of adolescence-onset methylmalonic acidemia (MMA) and explore preventive strategies.Methods:This was a retrospective case analysis of the phenotypes, genotypes and prognoses of adolescence-onset MMA patients. There were 55 patients diagnosed in Peking University First Hospital from January 2002 to June 2023, the data of symptoms, signs, laboratory results, gene variations, and outcomes was collected. The follow-ups were done through WeChat, telephone, or clinic visits every 3 to 6 months.Results:Among the 55 patients, 31 were males and 24 were females. The age of onset was 12 years old (range 10-18 years old). They visited clinics at Tanner stages 2 to 5 with typical secondary sexual characteristics. Nine cases (16%) were trigged by infection and 5 cases (9%) were triggered by insidious exercises. The period from onset to diagnosis was between 2 months and 6 years. Forty-five cases (82%) had neuropsychiatric symptoms as the main symptoms, followed by cardiovascular symptoms in 12 cases (22%), kidney damage in 7 cases (13%), and eye disease in 12 cases (22%). Fifty-four cases (98%) had the biochemical characteristics of methylmalonic acidemia combined with homocysteinemia, and 1 case (2%) had the isolated methylmalonic acidemia. Genetic diagnosis was obtained in 54 cases, with 20 variants identified in MMACHC gene and 2 in MMUT gene. In 53 children with MMACHC gene mutation,1 case had dual gene variants of PRDX1 and MMACHC, with 105 alleles. The top 5 frequent variants in MMACHC were c.482G>A in 39 alleles (37%), c.609G>A in 17 alleles (16%), c.658_660delAAG in 11 alleles (10%), c.80A>G in 10 alleles (10%), c.567dupT and c.394C>T both are 4 alleles (4%). All patients recovered using cobalamin, L-carnitine, betaine, and symptomatic therapy, and 54 patients (98%) returned to school or work.Conclusions:Patients with adolescence-onset MMA may triggered by fatigue or infection. The diagnosis is often delayed due to non-specific symptoms. Metabolic and genetic tests are crucial for a definite diagnosis. Treatment with cobalamin, L-carnitine, and betaine can effectively reverse the prognosis of MMA in adolescence-onset patients.