Objective To analyze the variations of glycerol-3-phosphate dehydrogenase 1 like gene (GPD1-L) and address the association with sudden m anhood death syndrom e (SMDS). Methods The genom ic DNA was extracted from blood sam ples of the SMDS group and the norm alcontrolgroup.The exons, exon-in-tron boundaries and 3′-U TRs of coding region of GPD1-L w ere PCRam plified and DNAsequenced di-rectly to confirm the types of variations. The genotype frequency and allele frequency w ere analyzed statistically. Results There w ere tw ovariants in the SMDS group, c.465C>Tand c.*18G>T, the latter existed certain degree difference of genotype distribution and allele frequency betw een the SMDS group and the control group, but there was no statistically significant (P>0.05). Conclusion The relation be-tw een gene m utation of GPD1-L and the occurrence of Chinese SMDS deserves a further research.

Objective: To evaluate the safety and efficacy of one-stop treatment (iliac vein stenting and varicose vein surgery) for Cockett syndrome combined with varicose veins of lower limbs. Methods: There were 58 cases diagnosed as Cockett syndrome combined with varicose veins of lower limbs underwent one-stop treatment. The postoperative complications and with or without varicose veins recurrence were observed, venous clinical severity score (VCSS), Aberdeen varicose vein questionnaire (AVVQ) score and iliac vein stent patency rate of preoperative and postoperative followed up 1, 3, 6 and 12 months were recorded. Results: All the 58 patients underwent one-stop treatment successfully, and the technical success rate was 100%; 3 patients with active ulcer healed completely within 1 month after operation; there were no severe complications such as deep vein thrombosis and pulmonary embolism occurred. The VCSS and AVVQ scores at 1, 3, 6 and 12 months after operation were significantly lower than those of before surgery (all P<0.001). At 12 months follow-up, the patency rate of iliac vein stent was 100%, and there were no recurrence of varicose veins in lower limbs. Conclusion: The one-stop treatment for Cockett syndrome combined with varicose veins of lower limbs has good safety and effectiveness, and the short-term iliac vein stent patency rate is satisfactory, which has a certain promotion significance.

Objective To evaluate treatment outcomes in patients with Cockett syndrome complicating acute lower extremity deep vein thrombosis (DVT) either by direct stenting after taking angioplasty followed by catheter-directed thrombolysis or staged stenting after taking angioplasty and catheterdirected thrombolysis with urokinase.Methods From Jun 2015 to Jun 2017,35 Cockett syndrome patients with DVT were divided into group A (direct stenting after taking angioplasty followed by catheterdirected thrombolysis,n =15) and group B (staged stenting after taking angioplasty and catheter-directed thrombolysis with urokinase,n =20).The total urokinase dosage,the time of thrombolysis,and the score of thrombolysis rate were evaluated.After six months of follow-up,the difference between two groups of limb circumference,patency rate and PTS rate were compared.Results The technical success rate in both group was 100％.There was no fatal pulmonary embolism and massive bleeding during the perioperative period.During treatment,both groups showed significant improvement in limb swelling compared with that before operation.The differences in the total used dosages of urokinase and thrombolysis time were not statistically significant (P =0.47,P =0.51 respectively).Thrombolysis rates above grade Ⅱ in group A and group B were 93.33％ and 90％ (P =0.64),but stent placement was not satisfactory in 2 cases in group A.After six months of follow-up,there was no significant difference between two groups of limb circumference and PTS rate,but patency rate of the two groups was 85.2％ ± 2.4％ and 87.6％ ± 1.8％ respectively (P =0.02).Conclusions For the treatment of Cockett syndrome with DVT,the timing of stent implantation does not affect thrombolytic treatment process.However,the complete removal of the iliac vein thrombosis is beneficial to precise release of the stent and the stent implantation can better maintain iliac vein lumen patency.

As the place for gas exchange, the lungs are metabolically active, and their energy consumption are essential for regulating common cell functions and maintaining the unique function of lung tissues to synthesize pulmonary surfactants. The metabolic pathways of pulmonary cells mainly include glycolysis, pentose phosphate pathway, and tricarboxylic acid cycle. Recent studies have found that changes in pulmonary cells metabolism are closely related to a variety of lung diseases. Herein, we review the main pathways of pulmonary cells metabolism and the relationship between changes in cell metabolism and the four lung diseases of chronic obstructive pulmonary disease (COPD), asthma, idiopathic pulmonary fibrosis (IPF), and pulmonary hypertension (PH), to find new ways to treat lung diseases.

Objective To investigate the application of pedicled tunica vaginalis flap cover new urethra in redo and second-stage hypospadias repair.Methods Retrospective analysis was performed in 45 redo hypospadias repair cases,aged from 1 year 3 months to 9 years 4 months (median 5 years 3month).All patients were divided into urethral dehiscence after hypospadias repair (group A) and the second stage surgery of two-stage hypospadias repair (group B) based on medical history.According to the site of dehiscence,the group A were further divided into midshaft urethral dehiscence (A1) and glans and distal urethral dehiscence with urethral opening located on midshaft (A2).After new urethra was completed,the appropriate size of the pedicledtunica vaginalis flap was transferred to cover the new urethra.Results There was no scrotal hematoma occurred in all 45 cases,and somecomplications occurred,including scrotal swelling in 1 case,high-riding testicle in 2 cases,penile clockwise torsion in 1 case,urethrocutaneous fistula in 6 cases.All the 45 cases were followed up for 1 to 4 years,with mean of 1.8 years.One more urethrocutaneous fistula occurred in group A2 and 1 more urethrocutaneous fistula with urethral stricture occurred in group B.There was no penile curvature and urethral diverticulum occurred in all cases.Conclusions Pedicled tunica vaginalis flap could be used as waterproofing layer to cover new urethra in redo hypospadias repair and presented a low incidence rate of postoperative complications.

Objective To review the clinical characteristics of urethral prolapse in female children and summarize our experience of treatment.Methods A retrospective analysis of the clinical characteristics of 102 patients with urethral prolapse from January 2007 to December 2017 was conducted at The Children's Hospital of Zhejiang University School of Medicine.The age of the patients ranged from 8-156 months with an median of 80 months.The presenting symptoms in the 102 girls were:bleeding in 57 patients (55.9％),mass in 31 patients (30.4％),and dysuria/urinary frequency,urgent and pain in 14 patients (13.4％).In all,58 patients were managed conservatively with Sitz baths as their masses were small,39 underwent prolapse reduction under topical anesthesia and Sitz baths because their mass were large,and 5 patients were treated by excision of the prolapsed urethral mucosa with four-quadrant excisional technique because thrombosed urethral prolapse at first visit.Results A total of 89 patients were cured after conservative treatment (87.3％),8 patients were converted to surgical treatment because frequent recurrence with conservative treatment.No urethral stricture,active hemorrhage and recurrent were found in 13 patients after operation.Conclusions The most common clinical manifestations of urethral prolapse are urethral mass and bleeding.Most patients can be cured by conservative treatment.The patients whose symptoms were severe or suffered from frequent recurrence of urethral prolapse should be managed with surgical excision.

Objective To investigate the proper procedure for repairing different urethrocutaneous fistulas after primary urethroplasty for hypospadias.Method There were 101 cases,whose age ranged from 27 months to 171 months (mean 61 months),underwent urethrocutaneous fistula repairing secondary to the primary hypospadiasis urethroplasty from January 2010 to December 2015.The methods of the repairing were chosen mainly on the site and the size of the fistula,which included three types.The coronal fistula with a thin band of tissue stretching between the glans wings was classified as type Ⅰ (n =24).For the rest of the small fistulas at penis coronal ditch and penis body,the small fistula (diameter ＜ 3 mm) was classified as type Ⅱ (n =57) and the large fistula (diameter ≥3 mm) was classified as type Ⅲ(n =30).The urethroplasty was performed in the type Ⅰ cases.The ligation and transfixion was performed in type lⅡ cases.And the tension free repairing with continuous suture was performed in type Ⅲ cases.De-epithelization dartos fascia flap or tunica vaginalis flap covering was performed in all cases.After removing the catheter,all cases were followed-up at least 1 year.The successive operation was termed as no complication,such as urethral stricture,urethral diverticulum and urethrocutaneous fistula.Result Totally 111 fistulas were repaired by the methods described above.The mean followed-up duration was 32 months (ranging 12-48months).Total recurrence of fistula was 11.7％ (13/111) in different type fistulas,including 16.7％(4/24) in type Ⅰ,3.5％ (2/57) in type Ⅱ and 23.3％ (7/30) in type 1Ⅲ.Conclusions Different classification and treatment of urethrocutaneous fistula after urethroplasty by the site and size of fistula can improve the outcome of the operation.It is necessary to cover the tissue tightly to a new urethra during the operation.

Objective To investigate the effects and mechanisms of Zhenxin Anshen Prescription(composed of Os Draconis,Ostreae Concha,Lophatheri Herba,Drynariae Rhizoma,Poria)on mice with atopic dermatitis(AD)based on the calcium channel regulator 1(ORAI1)/nuclear factor of T-cells(NFAT)signalling axis.Methods Thirty-six BALB/c mice were randomly divided into a blank control group,a model group,a Cetirizine group(1.3 mg·kg-1)and a Zhenxin Anshen Prescription group(36.36 g·kg-1),with nine mice in each group.AD mouse model was established using 1-chloro-2,4-dinitrobenzene(DNCB)induction.The drug was administered by gavage once a day for 2 weeks.At the end of drug administration,the area of skin lesions was measured and the severity of skin lesions was scored;spleen mass was measured and spleen index was calculated;pathological changes of skin lesion tissues were observed by HE staining;interleukin 4(IL-4),IL-13 and thymic stromal lymphopoietin(TSLP)in serum were detected by ELISA;and the protein expression levels of ORAI1,calmodulin phosphatase A(CaN)and nuclear factor of T cells 2(NFAT2)were detected by Western Blot.Results Compared with the blank control group,the skin lesion score of mice in the model group was significantly increased(P＜0.01),the skin lesion area was significantly enlarged(P＜0.01);the thickness of the epidermis and dermis were significantly increased(P＜0.01),hyperkeratosis of the epidermis,hypertrophy of the stratum spinosum,and infiltration of inflammatory cells such as eosinophils and lymphocytes can be seen in the dermis;the splenic index and serum IL-4,IL-13,TSLP levels were significantly increased(P＜0.01);protein expression levels of CaN,NFAT2,ORAI1 were significantly increased in the skin lesion tissues(P＜0.01).Compared with the model group,the dermatitis score of mice in the Zhenxin Anshen Prescription group was significantly decreased(P＜0.01),the lesion area was significantly reduced(P＜0.01),the epidermal and dermal thicknesses(P＜0.01),the hyperkeratosis of epidermis was alleviated,the spinous layer was slightly hypertrophic,and there was a small amount of inflammatory cell infiltration in the dermis;the splenic index and the levels of serum IL-4,IL-13,and TSLP were significantly decreased(P＜0.01);the protein expressions levels of CaN,NFAT2,and ORAI1 in the skin lesion tissues were significantly decreased(P＜0.01).Conclusion Zhenxin Anshen Prescription can ameliorate dermatopathological injury in DNCB-induced AD mice,and the mechanism may be related to its ability to inhibit the protein expressions of ORAI1,CaN and NFAT2,reduce the levels of serum type 2 inflammatory factors TSLP,IL-4 and IL-13,and ameliorate cutaneous inflammation and itching through immunomodulation.

Objective To evaluate the feasibility of dual-source low-dose computed tomography (CT) of the paranasal sinus in children, with acquisition at an ultra-low tube voltage (70 kVp) combined with the Flash scan. Methods CT scans of the paranasal sinus were performed on 80 pediatric patients who were divided into two groups according to different protocols (70 kVp protocol with Flash scan mode and the iterative reconstruction, pitch 3, the experimental group (group A), n = 40; 80 kVp protocol with conventional spiral mode, pitch 1.5, control group (group B), n = 40). For each examination, the CT dose index (CTDIvol), dose-length product (DLP) and effective dose (ED) were estimated. The image noise, signal-to-noise ratio (SNR) and overall subjective diagnostic image quality were also evaluated. Results The images of these two groups were all satisfied for the clinical diagnosis. For radiation dose, the CTDIvol (mGy), DLP (mGycm) and ED (mSv) values of the 70 kVp protocol were significantly lower than those of the 80 kVp protocol [CTDIvol: 0.39 ± 0.004 vs1.57 ± 0.009 mGy, P < 0.001; DLP: 6.31 ± 0.52 vs 19.88 ± 2.01 mGycm, P < 0.001; ED: 0.024 ± 0.005 vs 0.079 ± 0.016 mSv, P < 0.001. Compared with those of the 80-kVp protocol, the image noise and the SNR_bone increased, the SNR_soft-tissue decreased. There was no statistical difference in the subjective scores of the two groups of image quality by the two physicians (P > 0.05). Conclusion When diagnosing the paranasal sinus in children, an ultralow tube voltage (70 kVp) combined with the Flash scan technique can reduce the radiation dose significantly while maintaining diagnostic image quality with clinically acceptable image noise.