Objective　To study the value and limitation of CT in the damage to multiple organs in children with Langerhans cell histiocytosis (LCH). Methods　Analysis of CT manifestations was performed in 13 cases of LCH proved by clinical features, laboratory data, and pathologic changes. Localized LCH was found in 4 cases, and disseminated LCH in 9 cases. All cases were examined by plain scan in craniocerebral, chest, liver, and spleen, and four cases by contrast-enhanced CT scan. Results　The male outnumbered female in this group. Cranial bones were involved in the sequence of temporal bone(8 cases), parietal bone (3 cases), occipital bone (2 cases), and frontal bone (1 case). CT findings were very sensitive in showing the early destructions of the bones. The characteristic manifestation on CT was multiple lytic lesions of the flat bones at any sites, without reactive sclerosis and soft tissue mass accompanied with the bony lesion. Imaging findings presented later than the clinical manifestations for LCH involving the hypothalamus/pituitary axis. Involvement of the lung at various stages by LCH could be demonstrated by CT scan. Conclusion　Imaging manifestations had no characteristics in LCH involvement. The sites of predilection and related CT manifestations could be suggestive of the diagnosis. In addition, CT findings can be helpful in the classification and evaluation of the therapy and prognosis in LCH.

Background Congenital aniridia is a rare bilateral hereditary ophthalmopathy which impact panocular.Researches showed that congenital aniridia can be caused by different mutation locus of PAX6 genes,and the mutations are multifarious.Objective This study was to detect and anaiyze the mutations of a Chinese family with congenital aniridia by using targeted sequence capture sequencing and direct Sanger sequencing.Methods This study was approved by Ethic Committee of the First Affiliated Hospital of Zhengzhou University and followed Declaration of Helsinki.Written informed consent was obtained from subjects or their custodians before any related medical examination.A cross-sectional study was performed.A Chinese congenital aniridia family was included at the First Affiliated Hospital of Zhengzhou University in March,2016.All the family members received systemic medical examinations including nervous system and oral glucose tolerance test and then the ocular examinations were carried out.The periphery blood of 10 ml was collected from the members for genomic DNA extraction.Targeted sequence capture sequencing was performed on the DNA of proband to screen out the suspicious mutant locus.The mutation was verified by comparing the Sanger direct sequencing results from all family members.Results A total of 3 generations of 9 members were included in this congenital aniridia pedigree,and the Ⅰ 1 was dead without eye abnormality.Three patients (Ⅱ2 and her children Ⅲ1,Ⅲ2) and 5 normal family members were determined,showing an autosomal dominant inheritance pattern.No abnormal signs were found in nervous system and oral glucose tolerance test in the families.The reduce of visual acuity,ocular hypertension (21 mmHg),absence of biocular iris,opacification of corneal stroma,horizontal nystagmus,hapoplasia of fovea were found in all the sufferers.In addition,the ptosis of the left eye,congenital cataract of the right eye in Ⅱ 2 patient as well as biocular cataract and subluxation of lenses also were exhibited.The c.183C＞A mutation of the PAX6 gene was screened out to be a possible pathogenic mutation.The result of Sanger direct sequencing in the families verified a co-segregation of this mutation with mutant phenotypes.Conclusions PAX6 gene c.183C ＞A,a rare mutation in Chinese population,is a virulence mutation site in this aniridia family.

Objective To prospectively investigate the characteristics and correlative influential factors for the changes in pulmonary artery hypertension (PAH)in chronic kidney disease (CKD) patients without end-stage renal disease.Methods Complete clinical and laboratory data of 133 outpatients without ESRD (CKD stage 2,3 and 4)in Beijing Hospital between January 2012 and January 2013 were retrospectively analyzed.Pulmonary artery systolic pressure(PASP)was assessed by echocardiography according to the guideline from the American Society of Echocardiography in 2010,and PASP more than 35 mmHg(1 mmHg=0.133 kPa)was diagnosed as PAH.Results PAH was found in twelve(9.0 ％) patients,including 7 females and 5 males,with mean age(82.9 ± 5.4 years).The prevalence of PAH was 2.3％,10.9％ and 17.6％ in the patients of CKD stage 2,3 and 4(P＜ 0.05) respectively.PASP could be calculated in 67 patients in whom tricuspid regurgitation was present.The mean PASP was (27.1 ± 5.4) mmHg,(31.0 ± 8.3) mmHg and (37.3 ± 13.8) mmHg in patients with CKD stage 2,3 and 4,respectively(P ＜ 0.05).Body mass index(BMI),eGFR and BUN showed significant differences between PAH group and non-PAH group with statistical significance (P＜0.05).Multivariate logistic regression analysis revealed that the lower BMI and the higher stage of CKD were independent risk factors for PAH (P ＜ 0.05).Conclusions PAH is not a rare complication in the CKD patients without end-stage renal disease.The incidence of PAH is significantly associated with lower BMI and the higher stage of CKD.Doppler echocardiography should be performed periodically to detect PAH in CKD patients in order to improve the prognosis of these patients.

Objective To observe the results of broth dilution method and disc diffusion method to test the synergistic effect of Reduning and cefoperazone sodium / sulbactam sodium(SCF) on extensive drug resistant Acinetobacter bauman (XDR-AB) in vitro environment ,and compare their compliance to guide the clinical medication .Methods A total of 12 strains of XDR-AB from infec-tion patients in our hospital in 2015 were collected ,the strain was sub cultured .Firstly ,observe the minimum inhibitory concentra-tion (MIC) of SCF and Reduning on XDR-AB alone and in combination by broth dilution method .And then judge the synergy effects through calculation .Secondly ,the inhibition ring diameter and the synergy effects was detected using the disc diffusion meth-od .Results The MIC of Reduning and SCF in combination on XDR-AB was declined compared with them alone .The Fractional in-hibitory concentration of Reduning and SCF in combination on XDR-AB were equal or less than 0 .5 ,they had synergistic effect on XDR-AB .The inhibition ring diameter of Reduning was 10 mm tested by disk diffusion method .Different strains of XDR-AB on SCF bacteriostatic annulus diameter difference ,5 strains were 15 mm ,3 strains were 16 mm ,and 4 strains were 17 mm .Reduning and SCF appeared synergistic effect according to the inhibition ring diameter expanded when they effected on XDR -AB in combina-tion .Conclusion In vitro ,Reduning combined with SCF on XDR-AB has good synergistic effect .Compared with broth microdilution checkerboard dilution method ,disk diffusion method is more simple and convenient ,but it has a certain subjective on judging re-sults ,which is better to operate by experienced person .

Objective To investigate the value of CT scans in differential diagnosis of thyroid neoplasm.Methods CT findings ofthyroid neoplasm in 29 cases including 8 cases thyroid adenoma,4 cases nodular goiter and 17 cases thyroid carcinoma identified by operation and pathology were analyzed retrospectively.Results The contour of thyroid adenoma was distinct,a completely enhanced ring in 5 cases and enhanced nodules in 3 cases were showed on contrast-enhanced scan.4 cases of nodular goiter showed homogenous cystic low density the lesions were localized and distinct in contour,the cystic wall could be seen.Thyroid carcinoma appeared unhomogenous density,the edges contour of lesion was not distinct and infiltration on adjacent tissue might be seen.Conclusion CT scan might play an important role in diagnosis and differential diagnosis of thyroid neoplasm.

Objective To observe the changes of cerebrovascular reactivity(CVR)in cerebral infarct, and ascertain the correlation between them. Methods Breath-holding index (BHI) was obtained with transcranial Doppler combined breath-holding test in 193 subjects with cerebral infarct and 113 controls. Results The BHI in cerebral infarct patients was significantly lower than that in controls(P=0.000),and CVR was a independent influential factor for cerebral infarct(P=0.000). Conclusion Impaired CVR is associated with cerebral infarct, which should be paid more attention to.

Objective: To learn the characteristics of adolescent pregnancy in Wenzhou from 2015 to 2019, so as to provide basis for protecting adolescent physical and mental health. Methods: Data of demographic characteristics, pregnancy and delivery outcomes of the women aged under 19 years ( adolescent pregnant ) and 20 to 34 years ( right-age pregnant ) who delivered in Wenzhou from 2015 to 2019 were collected from Wenzhou Maternal and Child Health Information Management System. The incidence of pregnancy complication, comorbidity and adverse delivery outcomes of adolescent pregnant women was compared with that of right-age pregnant women. Results: Among 536 753 parturients reported in Wenzhou from 2015 to 2019, there were 22 419 ( 4.18% ) of adolescent pregnancy and 430 163 ( 80.14% ) of right-age pregnancy. The average age of adolescent pregnant women was (18.11±0.82) years ( range, 10-19 years ). The majority of adolescent pregnant women were migrant population (19 437 cases, 86.70%), had an education level of junior high school and below ( 18 594 cases, 82.94% ), and had no occupation (19 192 cases, 85.61%). The incidence rates of anemia, gestational diabetes, postpartum hemorrhage, perineal laceration and premature delivery in adolescent pregnant women increased from 2015 to 2019 ( all P<0.05 ). The incidence rates of gestational diabetes, anemia, gestational hypertension, placental abruption, postpartum hemorrhage, perineal laceration, premature delivery, low birth weight, fetal death and stillbirth were 1.65%, 11.76%, 0.19%, 0.26%, 3.19%, 24.52%, 6.03%, 5.53%, 0.50% and 0.04% in adolescent pregnant women, which were significant different from 5.49%, 8.94%, 0.13%, 0.01%, 0.17%, 23.46%, 4.66%, 3.08%, 0.26% and 0.01% in the right-age pregnant women, respectively ( all P<0.05 ). Conclusions The adolescent pregnant population in Wenzhou are mainly migrant population with lower education level and no occupation. The incidence rates of pregnancy complication, comorbidity and adverse birth outcomes in adolescent pregnant population are higher than those in right-age pregnant population, and are increasing year by year.

Adult ; Heart Atria ; Heart Neoplasms ; complications ; Humans ; Intracranial Aneurysm ; etiology ; Magnetic Resonance Imaging ; Male ; Myxoma ; complications

Objective To explore the application value of multiphase dynamic contrast-enhanced MRI (MDCE-MRI) in the early chemoradiation efficacy in cervical cancer.Methods Twenty-one patients with cervical cancer received chemoradiation.Routine MR scan and MDCE-MRI scan were examined at different stages, including pre-treatment, 15 days,1 month and 2 months after treatment.According to volume change of the tumor after treatment, the cases were mainly divided into complete remission(CR), partial remission (PR).The correlation between volume change of cancer after treatment and the time-signal intensity curve (TIC),mean time to enhancement(MTE),time to peak(TTP),maximum slope of increase(MSI),maximum slope of decrease(MSD), negative enhancement integral (NEI) were analysed.Results After system chemoradiation for 2 months,4 of enrolled patients got complete remission and 17 partial remission.The reduction rate of the tumor diameter was negatively correlated to MSI values(r=-0.877,P<0.05) and positively related with NEI (r=0.819,P<0.05) before the treatment.15 days after the treatment, the diminished rate of the tumor diameter had active relation with change of TTP(r=0.765,P<0.05) and NEI(r=0.775,P<0.05).It indicated that MSI and NEI values before cervical cancer chemoradiation and the variation of TTP and NEI after treatment for 15 days could help predict the variation tendency of cancer diameter.Chemoradiation and therapy 1 month later,for all the parameters'' AUC values,the change rate of NEI were the largest one.When the critical value was not less than 96.46%, the sensitivity was 82.4%, specificity was 75%.TIC curve of type Ⅰ increased, type Ⅲ decreased to disappear gradually.Conclusion MDCE-MRI can be used to predict the efficacy of chemoradiation in cervical cancer patients, of which the MSI, TTP and NEI are more sensitive.

Objective:To summarize the midterm to long-term outcomes and experiences of endovascular treatment (ET) of spontaneous isolated superior mesenteric artery dissection (SISMAD).Methods:The clinical data of 31 SISMAD patients from Jan 2011 to Dec 2019 treated with ET was retrospectively analyzed.Results:Successful ET was achieved in 29 patients with a technical success rate of 93.5%. A total of 36 self-expandable bare stents were planted in 28 patients and plain old balloon angioplastry (POBA) was performed in 1 patient. Abdominal pain disappeared within 24 hours in 89.3% of the patients after stenting. The rate of perioperative complication was 3.2%. There was no SMA dissection rupture bleeding, nor perioperative death occurred. The mean follow-up time was 53.5 (range, 6 to 110) months. There was no dissecting aneurysm formation, no SMA rupture and bleeding, and no stent rupture during the follow-up. The post ET 1-year, 3-year, and 5-year free from reintervention rate were 100%, 100%, and 91.7%, respectively.Conclusions:ET for SISMAD is safe and effective with satisfactory perioperative and midterm to long-term outcomes.