Objective In order to understand whether recombinant human growth hormone(r-hGH) can improve the idiopathic short stature(ISS) boys' final adult height(FAH).Methods We measured 16 cases diagnosed as ISS boys FAH, divided into 2 groups, of which 9 cases were not given r-hGH treatment, treatment group ,7 patients received r-hGH treatment, a dose of 0.1 ～ 0.13 U· kg-1·d-1, before going to sleep subcutaneous injection, duration of treatment was 6 ～ 20 months ( 11.9 ± 5.2 months).Results The treatment group compared with the average FAH FAH observation group had significantly improved the average(t =2.219,P<0.05).Conclusion r-hGH can improve the ISS boys FAH, but early treatment, the longer the course the more obvious effects.

Objective To investigate the clinical effect of IFNα combined with mannan peptide in treatment of patients with HBeAg-positive chronic hepatitis B ( CHB ). Methods Eighty HBeAg-positive CHB patients with HBV DNA quantity ranging from 10 to 10 eopies/mL were enrolled and randomized into the treatment group and the control group ( n = 40 for each ). Patients in treatment group were given daily subcutaneous injection of IFNα-2b 5,000,000 U for 52 weeks, and received mannan peptide 10 mg per intravenous injection or 2. 5 mg per intramuscular injection for a total of 2 to 3 treatment courses (12 weeks for each). The control group received only IFNα-2b treatment. Liver function, serum markers of hepatitis B, HBV DNA quantity and blood tests were performed before the treatment and at 2, 4, 8, 16, 26 and 52-week during the treatment; and the adverse effects were recorded. Results The rates for ALT normalization, negative HBsAg, negative HBeAg, HBeAg seroconversion and negative HBV DNA were 91. 8% , 17. 5% , 52. 5% , 27. 5 % and 47. 5% at 52nd week in the treatment group, while those in the control group were 80. 0% , 12. 5% , 30. 0% , 10. 0 % and 25. 0% , respectively. There were significant differences in HBeAg-negative, HBeAg-seroeonversion and HBV DNA-negative rates between two groups (χ2 = 4. 178, 4.021 and 4.381, P < 0. 05 ) , and these indexes in the treatment group were increased to 57. 5% , 30. 0% and 50. 0 respectively at 52nd week after drug withdraw. White blood cells began to be elevated at 4th week and were restored to the normal levels at 8th week in the treatment group, while the count in the control was lower than the normal value even at 52nd week of the treatment with the average of (3.45±1. 18)×109/L. Conclusion Alpha-interferon combined with mannan peptide therapy is effective for patients with HBeAg-positive CHB, which may restore the declined peripheral WBC counts induced by interferon and improve the compliance.

Objective To investigate some improvements in the surgical techniques of adult-to-adult living donor liver transplantation( A-A LDLT) without the middle hepatic vein(MHV) for hepat-ic vein reconstruction. Methods The retrospective analysis was made on the clinical data of 11 recipi-ents who underwent the operation in A-A LDLT including the hepatic vein reconstructed in right liver lobe without MHV from June 2007 to January 2008. The key techniques included reconstructing out-flow of graft on shaping the tips of vena cava and right hepatic veins, cadaveric vein allografts stored in 4℃ UW solution within 7d being used for significant-sized hepatic vein reconstruction such as tributa-ries of the middle hepatic vein from V5, V8 and right inferior hepatic vein. Results 10 cases success-fully underwent reconstruction of outflow of graft on shaping the tips of vena cava and right hepatic veins and the outflow reconstruction ratio of V5, V8 and right inferior hepatic vein was 81. 8% (9/11), 7 one-vein reconstruction, 1 two-vein reconstruction and 1 three-vein reconstruction. 1 recipient died of renal failure and pulmonary infection 14 days after operation without venous outflow obstruc-tion. Doppler ultrasonography showed no thrombosis and the blood flowed smoothly in the right he-patic vein of other 8 recipients during the 9th to 15th mouth of follow-up. The cumulative patency rates of these 8 survivals for interposition vein grafts were 100% (11/11), 72. 7 %(8/11), 54. 5%(6/11) and 36. 5%(4/11) in 1, 3, 6 and 9 mouths, respectively. The regeneration of paramedian sectors was equivalent. Conclusion Shaping the tips of vena cava and right hepatic veins and using cadaveric vein allografts in adult-to-adult right lobe living donor liver transplantation for hepatic vein reconstruc-tion are both safe,simple and effective methods.This approach can be recommended.

Objective: To understand and analyze the prevalence of viral hepatitis in railway passenger occupational population in Jiangxi province, and to explore its epidemiological characteristics, so as to provide basis for the prevention and control of viral hepatitis in railway occupational population in the future. Methods: The test results of anti-hepatitis A virus (HAV)-IgM, anti-HAV-IgG, hepatitis B surface antigen (HBsAg), anti-hepatitis B surface antibody (anti-HBs), anti-hepatitis C virus (HCV), anti-hepatitis E virus (HEV)-IgM and anti-HEV-IgG in some passenger train attendants of Nanchang Railway Bureau from 2013 to 2015 were collected and analyzed by SPSS 19.0 and Excel 2007 software. Results: The positive rate of anti-HAV-IgG was 90.6%-98.7% from 2013 to 2015, the positive rate of HBsAg was 6.6%-15.1%, the positive rate of anti-HCV was 0.2%-1.4%, the positive rate of anti-HEV-IgG was 16.1%-24.9%; the positive rate of anti-HEV-IgG was significantly different between men and women, and the positive rate of anti-HEV-IgG was 29.5%, 30.5%, 22.5% between 2013 and 2015. The positive rate of male was higher than that of female (22.3, 22.5, 13.8) (χ²=3.934, P=0.047; χ² =4.363, P=0.037; χ²=6.755, P=0.009), and there was no significant difference between male and female in the surveillance results of other types of viral hepatitis. Conclusions The positive rate of intestinal transmitted hepatitis was high in the population and low in the acute phase. The positive rate of anti-HCV was low in the population with extraintestinal transmitted hepatitis. The positive rate of HBsAg was high in the population with extraintestinal transmitted hepatitis.

OBJECTIVE: To introduce peratrial device closure of secundum atrial septal defects (ASD) under the guidance of transesophageal echocardiography (TEE) without cardiopulmonary bypass (CPB) in children, and to summarize the clinical experiences. METHODS: A total of 115 children with secundum ASD (the occlusion group) underwent peratrial device closure of atrial septal defects through a small sternotomy under TEE guidance without cardiopulmonary bypass. Children were followed up closely for 1-13 months. Another 59 children (the bypass group) had closed atrial septal defects under cardiopulmonary bypass during the same period. The differences in the operation duration, convalescence and complication between the 2 groups were compared. RESULTS: Except 1 patient was operated under the CPB, the rest 114 patients in the occlusion group were successfully closed by the occluders. The duration of the operation, mechanical ventilation, intensive care and hospitalization, and the rate of blood-transfusion in the occlusion group were significantly lower than those in the bypass group (P<0.01), with no difference in complications in the 2 groups (P>0.05). CONCLUSION Minimally invasive peratrial device closure of ASD without CPB is a relatively simple, safe and effective operation under the guidance of TEE for children. The short and mid-term clinical outcomes are promising. Long-term follow-up is indispensable.
Child ; Child, Preschool ; Echocardiography, Transesophageal ; methods ; Female ; Follow-Up Studies ; Heart Septal Defects, Atrial ; diagnostic imaging ; surgery ; Humans ; Infant ; Male ; Minimally Invasive Surgical Procedures ; methods ; Septal Occluder Device ; Ultrasonography, Interventional

OBJECTIVE: To elucidate the preoperative, intraoperative, and postoperative utility of echocardiographic technology in occlusion of ventricular septal defect (VSD) via small chest incision. METHODS: We performed occlusion of VSD via small chest incision in 446 children. Before surgery, a multiple-section transthoracic echo-cardiogram (TTE) was employed to evaluate various parameters (including the size, position, and type) of the VSD region as well as the condition of neighboring tissues. During surgery we reassured the size of the occluder, meanwhile, guiding placement of the occluder by transesophageal echocardiogram (TEE). Patients also received postoperative follow-ups at regular intervals. RESULTS: Out of the 446 VSD cases, the defects of 412 patients were successfully closed; a 92.4% successful rate. All patients received follow-ups one year after operation. The occluders had stayed firmly and echoed clearly. No notable residual shunt or valve regurgitation was discovered. CONCLUSION During occlusion of VSD via small chest incision, echo-cardiogram technology (including TTE and TEE) can play a vital role by helping screen cases preoperatively, by guiding the closure intraoperatively, and by evaluating therapeutic efficacy postoperatively, providing relatively accurate and accountable results at all stages.
Child ; Child, Preschool ; Echocardiography, Transesophageal ; methods ; Female ; Heart Septal Defects, Ventricular ; diagnostic imaging ; surgery ; Humans ; Infant ; Male ; Minimally Invasive Surgical Procedures ; methods ; Prosthesis Design ; Septal Occluder Device ; Treatment Outcome ; Ultrasonography, Interventional

OBJECTIVE: To explore the effect of unidirectional valved patch (UVP) for congenital heart disease (CHD) with severe pulmonary hypertension (PH). METHODS: We retrospectively analyzed the treatment of 37 CHD patients with severe PH by UVP in the operation, and summarized its short-term to mid-term effect to find an optimum therapeutic regimen. RESULTS: Before the operation, the ECG showed that the mean pulmonary artery pressure (MPAP) ranged 65-72 mmHg, and the cardiac catheterization showed the pulmonary artery pressure ranged 80-120 mmHg, P(P)/P(A) ranged 0.8-1.05,PVR ranged 8.5-19.2 (under oxygen inhalation 6.8-14.6) wood unit.After the operation, P(P)/P(A) ranged 0.4-0.72 on weaning-off CPB. Postoperative ECG showed the MPAP ranged 32-48 mmHg. No pulmonary hypertension crisis occurred and no patient died. Mechanical ventilation time ranged from 32 h to 8 d and the SaO₂ ranged 93%-96% at rest after the extubation.The right-to-left shunt situations by ECG were as follows:22 cases had shunt 5 d after the operation, 11 cases had shunt 1 month after the operation,4 cases 3 months after the operation, and none 1 year after the operation but one patient lost follow-up.However,there were no long-term follow-up data: 12 patients had a 1-year follow-up, 5 patients had a 3-year follow-up, and most patients had just 3-month follow-up. CONCLUSION UVP can decrease the operative risk in CHD with severe PH at perioperative period. The short-term to mid-term effect is satisfactory, while long-term effect remains uncertain.
Adolescent ; Cardiac Surgical Procedures ; methods ; Child ; Female ; Heart Defects, Congenital ; complications ; surgery ; Heart Septal Defects, Ventricular ; complications ; surgery ; Humans ; Hypertension, Pulmonary ; etiology ; surgery ; Male ; Pericardium ; transplantation ; Prosthesis Implantation ; Retrospective Studies ; Young Adult

OBJECTIVE: To discuss the preoperative, intraoperative, and postoperative application of echocardiography in mini-invasive surgical device closure of secundum atrial septal defects, including those special and difficulty-occluded defects. METHODS: We performed mini-invasive surgical device closure of secundum atrial septal defects on 287 patients. Before the surgery, transthoracic echocardiography was applied for screening; during the surgery we reassessed the sizes of the defects and their remaining margins, designated the suitable occluders, and guided the placement of the occluders by multiplane transesophageal echocardiography. The patients were postoperatively followed up at regular intervals by multiplane transesophageal echocardiography (MTEE) which was employed to assess the therapeutic efficacy. RESULTS: Out of the 287 atrial septal defects, 276 (96.17%) were successfully closed. There were 37 porous defects and 23 cases with short posterior-inferior margin of defects. Follow-ups at intervals showed the occluders stayed firmly and echoed clearly. No notable residual shunt or valve regurgitation was observed. CONCLUSION Echocardiography plays a vital and reliable role in mini-invasive surgical device closure of secundum atrial septal defects, especially those special and difficulty-occluded defects.
Adolescent ; Child ; Child, Preschool ; Echocardiography, Transesophageal ; methods ; Female ; Heart Septal Defects, Atrial ; surgery ; Humans ; Infant ; Male ; Minimally Invasive Surgical Procedures ; methods ; Septal Occluder Device ; Ultrasonography, Interventional

OBJECTIVE: To discuss the suitable types of ventricular septal defects for asymmetric occluders, and elucidate the critical role of echocardiography in choosing occluders, guiding successful occlusion and avoiding injury during operation. METHODS: We retrospectively studied 179 patients with ventricular septal defects who received minimal-invasive surgical device closure with asymmetric occluder. We analyzed the types, size and morphology of ventricular septal defects suitable for asymmetric occluders. The therapeutic efficiency was evaluated by follow-ups. RESULTS: Out of the 179 successful cases treated with asymmetric occluders, 86.59% had perimembranous ventricular septal defects, and double-committed sub-arterial ventricular septal defects accounted for 13.41%. In general, the size of occluders to be selected was the maximum diameter of the defects plus 2-3 mm. Follow-ups showed that occluders were placed and fixed properly. No severe residual shunt, valve regurgitation or heart block were discovered. CONCLUSION Application of asymmetric occluders expands the range of indications for occlusion via small chest incision. Accurate echocardiography helps to improve the safety and successful rate of the surgery.
Adolescent ; Child ; Child, Preschool ; Echocardiography, Doppler, Color ; Female ; Heart Septal Defects, Ventricular ; diagnostic imaging ; surgery ; Humans ; Infant ; Male ; Minimally Invasive Surgical Procedures ; instrumentation ; methods ; Retrospective Studies ; Septal Occluder Device

Objective:To analyze the clinical characteristics and genetic features of SMC1A gene related disorders. Methods:The data of 5 children with SMC1A gene variants were collected from Children′s Hospital of Fudan University from February 2018 to January 2022. The clinical features, electroencephalogram (EEG), brain imaging and gene testing results were summarized. Results:Among the 5 patients, 4 are females and 1 is male. Two female cases are siblings. One boy had dysmorphic features, consisting of bilateral ptosis, synophrys, a short nose and upturned nasal tip. He also had patent foramen ovale plus atrial septal defect, unilateral cryptorchidism and microcephaly. Three cases had microcephaly. Two girls had patent foramen ovale, and 2 girls had microcephaly. Four cases had epilepsy, and age at seizure onset ranged from 2 to 52 months. Multiple seizure types were observed, including bilateral tonic clonic seizures in 2 patients, and focal seizures in 3 patients. The seizures in 3 cases were in cluster. All patients had developmental delay, including 1 patient with mild and 4 patients with moderate to severe developmental delay. Three patients had slow background activity in EEG. Interictal EEG showed abnormal discharges in 4 patients, including focal discharges in 3 cases and generalized discharges in 1 case. Brain magnetic resonance imaging was normal in 3 patients and showed mild cortical thickening in 1 case. All cases harbored 4 SMC1A gene variants, including 2 missense variants and 2 frameshift variants (c.580_587del, c.2699delG, c.3362G>A, c.1486C>T). Three cases harbored heterozygous SMC1A variants and 2 cases carried somatic mosaic SMC1A variants with 17.5% and 88.1% mosaicism in peripheral blood. The follow-up lasted for 3 months to 4 years. The epilepsy was refractory in 2 cases. During the follow-up, all cases had very slow developmental progress or developmental retardation. All cases had different levels of growth retardation. The scores of Cornelia de Lange syndrome (CdLS) phenotypes in 5 cases were 2-6. One case had the combined phenotypes of atypical CdLS and developmental epileptic encephalopathy (DEE). The phenotype was atypical CdLS in 1 case and DEE in 1 case. The phenotypes of 2 cases with SMC1A missense variants were mild, manifesting as non-refractory epilepsy and moderate to severe developmental delay. Conclusions:All of cases with SMC1A gene variants have developmental delay. Most of the patients have clusters of seizures and some dysmorphisms. The phenotypes of SMC1A gene related disorders are diverse. Except CdLS and DEE, there are some patients with mild phenotype due to missense variants of SMC1A gene.