SUMMARY Hemophilia A is aninherited bleeding disorder, lack of coagulation factorⅧ( FⅧ) , and if combined with intracranial malignant tumor, the operation risk is very high. Department of Pediatric Sur-gery in Peking University First Hospital used coagulation factor replacement therapy, succeeded in the operation of 2 cases of intracranial malignant tumor with hemophilia A in children, with no abnormal bleeding events. The establishment of the multi subject cooperation group before operation, good preoper-ative preparation, enough alternative factors, and close postoperative monitoring, are the key to the suc-cessful treatment.

Objective To detect stunned myocardium using low-dose dobutamine stress (DBS)combined with two-dimensional speckle tracking imaging (2D-STI),and to evaluate the difference and characteristics of mechanical parameters in different conditions between stunned myocardium and adjacent normal myocardium.Methods The acute myocardium ischemia/reperfusion (I/R) of anterior wall of left ventricle(LV) was induced with 60 minutes ligation of left anterior descending coronary artery (LAD),and with reperfusion of 120 minutes in 10 open-chest beagle dogs.Dobutamine was administered continuously via vein with two different dose of 5 μg · kg-1 · min-1 and 10 μg · kg-1 · min-1.At baseline,post ischemia/reperfusion,after the first DBS and the second,the gray-scale dynamic images of three cardiac cycle of left ventricular short axis at the levels of mitral annulus,papillary muscle and apex were acquired.The systolic peak of circumferential strain (CS),radial displacement (RD) of eighteen segments of subendocardium and subepicardium were analyzed by Speckle tracking workstation.LV end-systolic dimension(LVIDS),LV end-diastolic dimension (LVIDD),LV eject fraction (LVEF),heart rate (HR),aortic valve forward flow velocity time integral(AV-VTI) were also measured.Results ①There was no statistical significance (P ＞0.05) under the conditions of baseline,post I/R,the first DBS and the second for LVIDD,LVIDS,LVEF,HR and AV-VTI.②Compared with baseline,the peak systolic subendocardium and subepicardium CS,RD decreased significantly at the apex and middle of short-axis anterior wall of LV under the conditions of post I/R;The parameters of the first DBS was significantly increased compared with the I/R (P ＜ 0.05) ; There was no difference between the two DBS and baseline(P ＞0.05).The peak systolic subendocardium and subepicardium CS,RD at the bottom segment of LV were no statistical significance under the four conditions.③There was no difference for anterolateral wall and anterior septum of LV at the level of papillary muscle under the four conditions.Conclusions ①Low-dose DBS combined with two-dimensional speckle tracking imaging can detect stunned myocardium accurately.② After the occurrence stunning myoeardium,the peak systolic subendocardium RD decreased slightly,subepicardium RD was normal,the subendocardium CS of the surrounding myocardium was still normal,and the peak systolic subepicardium CS decreased to some degree.

Objective To investigate regional ventricular long-axis velocity and adjacent intraventricular flow velocity using dual-pulse wave(PW) Doppler echocardiography synchronously,and to analyze the coupling relationship between regional ventricular long-axis strain and adjacent intraventricular pressure gradients in normal cases using dual-PW Doppler echocardiography.Methods Eighty-six normal subjects aged 19～66 years [mean age (37.49 ± 11.97)years] underwent routine echocardiography and dualPW Doppler echocardiographic exam.Total 18 segments were sampled using dual-PW Doppler (PW/TDI mode) images at basal,mid and apical levels on three standard apical views,and electrocardiogram was recorded simultaneously.Peak early diastolic velocity (emax),peak late diastolic velocity (amax),peak early mitral diastolic inflow velocity (Emax),regional peak early diastolic velocity (Em),regional peak late diastolic velocity (Am) and regional peak early diastolic inflow velocity (E) were measured simultaneously in the same one cardiac cycle.Myocardial variations of left ventricular segments were observed.Em,Am and E of different levels from different ventricular wall were analyzed.The co-relationship between regional ventricular long-axis strain and adjacent intraventricular pressure gradients were calculated and analyzed.Results ①Regional myocardial velocity and regional peak early diastolic inflow velocity in normal subjects declined from basal to apical segments gradually at the same ventricular wall (P ＜ 0.01).② Emax was correlated with emax(r =0.418,P ＜0.001),and E was correlated with Em (posterior septum,r =0.610,P ＜0.001 ；anterior wall,r =0.499,P ＜0.001).③There was a medium correlation between normal longaxis strain in segments and adjacent intraventricular pressure gradients (the global,r =0.412,P ＜0.001 ；posterior septum,r =0.319,P ＜0.005).Conclusions The acquirement of E and Em by dual-PW Doppler showed downtrend of segmental wall movement and regional blood flow from basal segment to apex.Emax is correlated with e E is correlated with Em,and long-axis strain at segments is correlated with adjacent intraventricular pressure gradients partly.Dual-PW Doppler might provide a potential method for the more precise evaluation of left ventricular fluid-solid coupling during diastole.

Objective To summarize the experience of the ventriculoperitoneal shunt in treating children with methylmalonic aciduria combined with hydrocephalus,and to assess the clinical value.Methods From September 2012 to May 2016,a total of 12 patients with methylmalonic aciduria combined with hydrocephalus in Peking University First Hospital were enrolled,including 7 boys and 5 girls.All the 12 patients underwent ventriculoperitoneal shunt.Drug therapy was performed after surgery.The clinical manifestations and imaging findings were used as the basis for adjusting the pressure of the diverter valve appropriately.The clinical condition of patients were evaluated retrospectively.The patients' clinical symptoms,signs,imaging materials,surgical complications and postoperative prognosis were analyzed.Results All the cases were followed up for 3 to 36 months,no death case and no serious postoperative complications of hydrocephalus occurred.Clinical symptoms of intracranial hypertension were relieved or disappeared.The head circumference progressive enlargement stopped.The anterior fontanelle tension decreased significantly.Setting-sun sign of eyes disappeared.One out of 4 cases with convulsion and epilepsy was relieved after the operation.Seven cases of poor vision or vision loss,postoperative visual acuity were improved though not recover to normal eyesight.One case of the children with hearing loss,postoperative hearing recovered.During the follow-up period,the head CT showed that the ventricle was narrowed significantly,interstitial brain edema improved obviously.Conclusion Ventriculoperitoneal shunt is a effective method for treating children with methylmalonic aciduria combined with hydrocephalus,which is beneficial for patients with these diseases.

Aim To study the effects of Shenfu soup onthe ISO -induced myocardial ischemia injury model ofrats and the influence of apelin level changes on myocardial ischemia.Methods The myocardial ischemiainjury model of rats was established by the subcutaneous injection of ISO.For 1 5 days,rats in Shenfugroups were given by gavage 3,6 and 1 2 g · kg -1Shenfu soup and rats in SF injection group were given6.67 g·kg -1 Shenfu injection,rats in sham group andmodel group were given the same volume of distilledwater.Rats were subcutaneously multipoint injectedISO for 5 days on the eleventh administrating day.Theeffects of Shenfu soup on myocardial morphology,serum myocardial enzyme levels,apelin mRNA level inserum and apelin protein level in myocardial tissue,and relationship between myocardial enzyme and apelinexpression were evaluated. Results Shenfu soupcould reduce the degree of myocardial tissue necrosisand compared with model group,CK values and LDHvalues of rats in middleand highdose groups were significantly lower (P <0.05 or P <0.01 ).However,the apelin mRNA level in myocardial tissue and apelinprotein level in serum of the rats in middleand highdose groups compared with model group had increased(P <0.05,P <0.01 ).Meanwhile,serum levels ofapelin and the expression of myocardial enzyme CK andLDH were negatively correlated.Conclusion Shenfusoup has a protective effect on myocardial ischemia injury in rats induced by ISO and the mechanism is involved with the promotion of apelin mRNA and proteinexpression,the inhibition of myocardial enzyme production and the improvement of myocardial ischemia.

Objective To evaluate left intra-ventricular diastolic blood flow patterns and fluid dynamics in pre diabetics using vector flow mapping(VFM),and to explore the value of left intra-ventricular hydromechanics characteristics assessment of intracardiac flow by VFM with more sensitive indexes.Methods Forty four patients with type 2 diabetes,twenty-eight pre-diabetes patients and thirty-three normal volunteers underwent VFM to determine the left ventricular(LV) diastolic blood flow patterns and fluid dynamics.The two-dimensional color Doppler flow images data were acquired in standerd apical fourchamber view during 3 complete cardiac cycles.These images were analyzed using a dedicated off-line workstation.At different level (mitral annulus,papillary muscl and apex level),the diastole blood flow parameters of LV including flow velocity and volume,velocity gradients (△V),pressure gradients (△P),diastole flow volume,vortex intensity ( Ⅵ),vortex area ( VA),vortex radius (VA) were derived.Thedifferences of the above parameters were compared among the three groups.The correlationship between diastolic function parameters and LV hydromechanics indexes were analyzed.Results ① Compared to normal group and pre-diabetics group,the LV diastolic function parameters E/A and e/a in diabetics group were lower( P ＜0.01),with no differences for E/e( P ＞0.05).There were no significant diffrences of these indices between diabetics group and pre-diabetics group( P ＞0.05).②)Compared to normal group,the intraventricular peak flow velocity at basal level,△V and △P in diabetics group and pre-diabetics group were lower( P ＜0.01),but there were no significant differences of these indices between diabetics group and prediabetics group( P ＞ 0.05).③Compared to normal group,the diastolic positive volume at basal level in diabetics group and pre-diabetics group was lower ( P ＜0.01 ),but there was no significant differences of these indices between diabetics group and pre-diabetics group ( P ＞ 0.05).④ Compared to the normal group,the Ⅵ in diabetics and pre-diabetics group was decreased,but there were no significant differences ofthese indices between diabetics group and pre-diabetics group( P ＞0.05).The VA and VR in diabetics and pre-diabetics group were increased,but there were no significant differences of these indices between diabetics group and pre-diabetics group ( P ＞ 0.05).⑤ There were no correlationship between diastolic function parameters and LV hydromechanics indexes.Conclusions There is the same LV diastolic blood flow patterns and fluid dynamics existed in pre-diabetics as diabetics.VFM could be used as a new methods to demonstrate the early left intra-ventricular fluid dynamics damages in pre-diabetics patients.

ObjectiveTo evaluate the myocardium damage of myocardial contrast echocardiography (MCE) and the impact on regional left ventricular mechanics by imaging and pathology techniques.Methods Eleven open-chest animal models of Beagle were employed to collect the short-axis views of mitral annular,papillary muscle and apical level of three complete cardiac cycles using gray-scale imaging at the baseline and blank irradiation 5 min later(transmitting frequency 1.7/3.4 M Hz,mechanical index 1.0).After that,5 ml of SonoVue was gotten a shot by intravenous bolus injection,the heart was exposed by ultrasonic wave for 5 min continuously,and then the same images collected were at the point-in-time irradiation immediately,20 min,40 min and 60 min later.The short-axis circumferential strain and strain rate,radial strain and strain rate were measured and calculated by EchoPAC multi-parameter workstation,meanwhile including conventional of cardiac function.All the dogs were killed after the experiments and the myocardium was harvested for HE staining and observed with transmission electron microscope for the tissue microstructure.Results①Conventional parameters of cardiac function:there was no significant difference before and after MCE in the heart rate,blood pressure,ejection fraction,left ventricular end-systolic volume and end-diastolic volume,stroke volume,cardiac output,mitral flow spectrum prior to the E/A,tissue Doppler mitral annular e/a and E/e ( P ＞0.05).During the whole process of experiment,the dogs’ vital signs were stable.②Compared the segmental circumferential strain and strain rate,radial strain and strain rate between baseline and each treatment groups,these parameters had a trend of increase in most segments,but the difference was not statistically significant in most segments (P ＞0.05).③HE staining at the light microscope demonstrated a small amount of myocardial cell infiltration of inflammatory cells near the outer membrane (21.2％),and very few part of the muscle fibers dissolved and fractured (6.1％).④The section of transmission electron microscope showed all the structures were normal except that a small amount of endothelial cells were mild swelling,some red blood cells were leaked,some myofilaments were regional dissolved,the relevance ratio were 28.6 ％,42.9 ％ and 21.4 ％ respectively,and the abnormal area was less than 10％ of the entire field of vision.ConclusionsMCE has no significant impact for the global function of heart and the regional mechanical state,and furthermore there is no serious pathological damage on the myocardium.

Objective:The clinical manifestations, types of gene mutations, therapeutic effects and prognostic characteristics of 15 children with cblC type methylmalonic acidemia (MMA) and hydrocephalus were analyzed to improve the clinical understanding of the disease, so as to provide a basis for the treatment of the disease.Methods:From April 2015 to January 2019, 15 patients with MMA and hydrocephalus in Department of Pediatric Surgery, Peking University First Hospital were enrolled, and all gene detection showed clbC type.All the 15 patients underwent ventriculoperitoneal shunt.After surgery, the clinical manifestations and imaging findings were applied as the basis to adjust the pressure of the diverter valve appropriately.Clinical data and gene mutation characteristics of 15 children with cblC type and hydrocephalus were retrospectively analyzed, and the therapeutic effects and prognosis were summarized and analyzed as well.Results:There were 8 males (53.3%) and 7 females (46.7%), aged from 2 to 33 months.All the cases were followed up from 11 to 55 months, without death case and serious postoperative complications of hydroce-phalus.The head circumference of 3 cases (20.0%) was in the normal range, 1 case (6.7%) was greater than the normal range, and 11 cases (73.3%) were less than the normal range.Four patients (26.7%) were transferred to the pediatric intensive care unit after surgery.c.609G>A mutation was the most common in this study, with 7 cases (46.7%) of c. 609G>A homozygous mutation, and 5 cases (33.3%) of c. 609G>A heterozygous mutation.Clinical symptoms of intracranial hypertension were relieved or disappeared.The head circumference progressive enlargement was stopped.The anterior fontanelle tension greatly decreased, all " setting-sun" sign of eyes disappeared, and vision loss and hearing loss were better compared with the pre-operation.Four cases (26.7%) displayed normal intelligence and exercise, and 11 cases (73.3%) were left with mild to severe psychomotor retardation.During the follow-up pe-riod, the head CT showed that the ventricle was remarkably narrowed, and interstitial brain edema obviously improved.Conclusions:Ventriculoperitoneal shunt in the treatment of cblC type MMA with hydrocephalus has positive effects.The head circumference of most cblC type MMA with hydrocephalus is less than the normal range.c.609 G>A is the most common mutation in cblC type MMA with hydrocephalus.Perioperative " metabolic crisis" can result in serious complications.

Objective:To study the safety and feasibility of anatomic left hemihepatectomy via cranial-dorsal approach in the treatment of left hepatolithiasis.Methods:Clinical data of 47 patients with left intrahepatic bile duct stones who underwent cranial-dorsal approach laparoscopic anatomic left hemihepatectomy in Hunan People's Hospital from October 2016 to June 2022 were retrospectively analyzed, including 15 males and 32 females, aged (56.45±1.37) years old. The operative time, intraoperative blood loss, postoperative liver function and complications were analyzed. Patients were followed up by telephone and outpatient review.Results:All 47 patients successfully underwent laparoscopic surgery without conversion. The median operative time was 260 (range, 160-440) min. The median intraoperative blood loss was 100 ml (range, 20-400 ml). The total bilirubin, alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels were 15.7 (11.7, 21.9) μmol/L, 126.6 (91.7, 168.5) U/L, and 151.1 (98.0, 212.4) U/L on postoperative day (POD) 1, respectively, and decreased to 12.6 (9.6, 16.2) μmol/L, 97.9 (60.7, 156.9) U/L, 54.2 (40.0, 104.1) U/L on POD 3, respectively. The median postoperative hospital stay was 7 (range, 4-24) d. Postoperative abdominal effusion and infection occurred in one patient, and the complication rate was 2.1% (1/47). Postoperative CT review found residual stones in common bile duct in one patient [2.1% (1/47)]. No stone recurrence or death occurred during postoperative follow-up.Conclusion:Anatomic left hemihepatectomy via cranial-dorsal approach is a safe and feasible surgery for the treatment of left hepatolithiasis.