Biliary atresia (BA) is characterized of progressive inflammation and obstruction with fibrosis in biliary system and presents symptoms with obstructive jaundice in neonatal period,if its treatment is not ineffectively may lead to progressive liver fibrosis,end-stage liver failure and die within 2 years.Kasai procedure is the major method in BA treatment,but not all cases have postoperative jaundice clearance and survive with native liver,it is need to optimize the choice of surgical time and methods,postoperative protocol with medicine,prevention and treatment of complications for improving the postoperative therapeutic effect.In this paper,the current option of clinical research and statue in Kasai procedure were summarized.

Objective To compare oral with intravenous Prednisolone after hepatoportoenterostomy in improving biliary drainage and survival with the native liver.Methods Forty-nine cases of biliary atresia were conducted from January to December in 2012.Participants were randomized to receive oral Prednisolone (group A) or intravenous Prednisolone (group B) on the fifth day of posthepatoportoenterostomy (the other treatments were same,including intravenous injection of Cefoperazone for 2 weeks, choleretic treatment, etc.).The initial dose was 4 mg/(kg · d), and it was reduced by half per 3 days, until 1 mg/(kg · d).Then Prednisolone was administered orally with 1 mg/(kg · d)for 3 months.The primary end points were the total bilirubin level(TBIL) and the percentage of cholangitis in 6 months of posthepatoportoenterostomy.Secondary outcome was survival with native liver in 24 months after hepatoportoenterostomy.Results The ages of group A and group B were(98 ±33) d and(105 ±36) d,TBIL of them were (158.36 ± 55.22) μmol/L and (160.11 ±43.12) μmol/L,and there were no statistical differences between the 2 groups before surgery (t =-0.663,-0.123, all P ＞ 0.05).In the first 2 months after operation, the mean TBIL value of group A was higher than that of group B (in the first month after surgery ,the TBIL quartiles P25 ,P50 ,P75 of group A were 105 μmol/L, 152 μmol/L, 236 μmol/L, and the quartiles of group B were 40 μmol/L, 74 μmol/L, 99 μmol/L.In the second month after surgery,the TBIL quartiles of group A were 67 μmol/L, 103 μmol/L, 144 μmol/L,and the quartiles of group B were 39 μmol/L,64 μmol/L, 196 μmol/L) (all P ＜ 0.05).The morbidity of cholangitis which occurred in group A(56.0％ ,60.0％) was higher than that in group B (25.0％, 29.2％) (x2 =4.871,4.705, all P ＜ 0.05).However,from the 3rd to 6th months,there was no statistical difference between group A and group B(all P ＞ 0.05).Transplant-free survival was 44.0％ in group A and 47.7％ in group B 24 months after operation, and there was no statistical difference(x2 =1.007 ,P ＞ 0.05).Conclusions Therapy of intravenous Prednisolone following portoenterostomy may be more beneficial in bile drainage than oral method,but it's of no use to improve survival.

Objective To evaluate the curative effectiveness and safety of human chorionic gonadotropin(HCG) treatment on children with microphallic hypospadias.Methods A total of 48 consecutive children with microphalic hypospadias were enrolled in the study,and the children were randomized into the experiment group(HCG treatment) and the control group with the research randomizer.The patients in experiment group were treated with HCG prior to surgery,and the control group did not received any hormone therapy preoperatively.All children in the experiment group and the control group underwent hypospadias repair by using transverse preputial island flap (Duckett technique) urethroplasty.Penile length,diameter of glans penis,bone age,serum testosterone level,and secondary effects were recorded before and after therapy in the experiment group.Postoperative complications were assessed with respect to fistulas,urethral strictures,diverticula,meatal stenosis,and glanular dehiscence in both groups.Results (1)Mean penile length and diameter of the experiment group increased significantly by (1.08±0.47) cm (t=-5.196,P<0.05) and (0.31±0.06) cm and there was a significant difference between before and after treatment (t=-5.080,P<0.05).(2)Urethrocutaneous fistulas were observed in 8 patients in the control group compared to 2 patients in the experiment group with a statistically significant difference (χ2=4.547,P<0.05).There was a significant difference between the overall reoperation rates of control group (9 patients) and the experiment group (3 patients,χ2=4.000,P<0.05).The penile tissue of the patients in the experiment group was soft and able to be easily separated and released during the operation and the flap had more blood supply.Conclusions Pretreatment with HCG therapy prior to hypospadias repair is beneficial to children with microphallic hypospadias.Significant penile growth was seen in the children treated with HCG and there was no obvious side effect.Moreover,pretreatment with HCG is beneficial to decrease the complications and reoperation rates of hypospadias repair which proves to be effective and safe.

Objective:To explore the effect of personalized psychological intervention based upon acceptance and commitment therapy on paternal donors and spouses after pediatric liver transplantation.Methods:From July 2017 to November 2019, a total of 160 parental donors and spouses undergoing pediatric liver transplantation were selected as research objects.Through a random number table, 40 donors and 40 liver donor spouses were assigned into control and experimental groups.The control group received routine psychological intervention while the experimental group had personalized psychological intervention with acceptance and commitment therapy based upon conventional psychological intervention.Symptom checklist-90, numeric rating scale, chronic pain acceptance questionnaire-8, length of stay, hospitalization cost, complication rate, initial postoperative ambulation time and hospital satisfaction were employed as evaluation tools.Results:After interventions, SCL-90 score, pain assessment score and initial ambulation time of donor group were significantly lower than those of control group( P<0.05). Hospitalization satisfaction and chronic pain acceptance were significantly higher than those of control group( P<0.05). SCL-90 score was significantly lower in liver spouse observation group than that in control group and hospitalization satisfaction higher than control group( P<0.05). Conclusions:Personalized psychological intervention based upon acceptance and commitment therapy can help parental donors and spouses to improve their mental health, help donors to recover sooner and boost hospitalization satisfaction.

Objective:To investigate the effect of instantaneous flow rate on the consistency of diagnostic accuracy of severe degenerative mitral regurgitation (DMR) using proximal isovelocity surface area (PISA).Methods:From June 2019 to June 2021, 75 patients with DMR who underwent echocardiography in Department of Echocardiography of Zhongshan Hospital, Fudan University were prospectively enrolled. The instantaneous flow rate of DMR during the systolic phase was calculated using M-mode PISA(PISA M-mode), and a time-integrated curve was plotted. Regurgitant volume (RVol) and effective regurgitant orifice area (EROA) were calculated by traditional PISA (PISA max), pair PISA (PISA pair), and PISA M-mode, respectively. RVol acquired from cardiac magnetic resonance (CMR) volumetric method in 22 patients of the enrolled patients. The correlation and consistency of RVol acquired between the three PISA methods and CMR were compared. Agreement of diagnostic accuracy of severe mitral regurgitation (sMR) acquired between the three PISA methods and multi-parameter algorithm by American Society of Echocardiography (ASE) was analyzed using Cohen′s Kappa analysis. Results:The curve of instantaneous flow rate of DMR showed unimodal pattern with the peak at mid-late systolic phase. The correlation of RVol acquired between PISA methods and CMR was moderate for PISA max and PISA pair ( r=0.77, 0.80, both P<0.001), whereas PISA M-mode presented strong correlation with CMR ( r=0.87, P<0.001). RVol acquired from PISA max was larger than that of CMR[(69.1±37.1) ml vs (49.0±29.0)ml, P=0.002]. Both PISA max and PISA pair were shown moderate agreement of diagnostic accuracy of sMR with ASE multi-parameters algorithm (RVol: κ=0.496, 0.525, both P<0.001; EROA: κ=0.570, 0.578, both P<0.001), while PISA M-mode presented strong agreement (RVol: κ=0.867 and EROA: κ=0.802, both P<0.001). Conclusions:Based on the unimodal pattern of instantaneous flow rate in patients with DMR, PISA max may significantly overestimate RVol, exposing a significant proportion of patients with DMR to unnecessary MR surgery. PISA M-mode presents better correlation and consistency with CMR on the quantification of RVol compared with PISA max and PISA pair, and may improve the diagnostic accuracy of quantification of sMR using PISA.

Objective To summarize the diagnosis and treatment experience of adenine phosphoribosyltransferase deficiency after kidney transplantation. Methods Clinical data of 1 patient with adenine phosphoribosyltransferase deficiency after kidney transplantation were retrospectively analyzed. Clinical characteristics, diagnosis, treatment and prognosis of adenine phosphoribosyltransferase deficiency were summarized by literature review. Results Renal biopsy showed that salt crystallization was found in most renal tubule lumen and positive results were observed under polarized light microscopy. After allopurinol, hemodialysis and anti-crystallization treatment, the graft function was gradually recovered. After postoperative 1-year follow-up, the patient's renal function was properly recovered. Conclusions Adenine phosphoribosyltransferase deficiency after kidney transplantation may lead to delayed graft function or graft dysfunction. Early detection, diagnosis and treatment may delay disease progression and improve renal function.

Objective: To understand the psychological experience of the pediatric living donor liver transplantation who is the child`s parent, identify the problems and gains that donors face in the event of donor liver, help donors to solve their difficulties to promote early recovery of donors and children, and help potential donors better understand the surgical. Methods: The phenomenological methodology in qualitative research was used in the study, and the Colaizzi 7-step data analysis method was used for analysis the interview recording. Results: Total of four themes are summarized. Preoperative psychological path: excitement, impatience, anxiety. Postperative growth: grateful, regain hope, hard work and proactive. Postnatal negative emotions: fear and jealousy. Family and social support. Conclusion Medical staff should strengthen psychological care and health education, use narrative care and other methods to mobilize the donor′s positive psychological experience, while providing perioperative quality care, strengthen social and family support, and help donors to restore physical and mental health.