Two approaches for newborns with critical congenital heart disease: a comparative study

Background Prenatal diagnosis and planned peripartum care is an unexplored concept in China.This study aimed to evaluate the effects of the“prenatal diagnosis and postnatal treatment integrated model”for newborns with critical congenital heart disease.Methods The medical records of neonates(≤28 days)admitted to Fuwai Hospital were reviewed retrospectively from January 2019 to December 2020.The patients were divided into“prenatal diagnosis and postnatal treatment integrated group”(n=47)and“non-integrated group”(n=69).Results The age of admission to the hospital and the age at surgery were earlier in the integrated group than in the non-integrated group(5.2±7.2 days vs.11.8±8.0 days,P<0.001;11.9±7.0 days vs.16.5±7.7 days,P=0.001,respectively).The weight at surgery also was lower in the integrated group than in the non-integrated group(3.3±0.4 kg vs.3.6±0.6 kg,P=0.010).Longer postoperative recovery time was needed in the integrated group,with a median mechanical ventilation time of 97 h(interquartile range 51–259 h)vs.69 h(29–168 h)(P=0.030)and with intensive care unit time of 13.0 days(8.0–21.0 days)vs.9.0 days(4.5–16.0 days)(P=0.048).No significant difference was observed in the all-cause mortality(2.1 vs.8.7%,P=0.238),but it was significantly lower in the integrated group for transposition of the great arteries(0 vs.18.8%,log rank P=0.032).Conclusions The prenatal diagnosis and postnatal treatment integrated model could significantly shorten the diagnosis and hospitalization interval of newborns,and surgical intervention could be performed with a lower risk of death,especially for transposition of the great arteries.     

先天性巨结肠根治术后医源性直肠阴道瘘的再手术治疗CSCD

Objective To explore the causes of iatrogenic rectovaginal fistula after pull-through in Hirschsprung’s disease (HD) and summarize the experiences and efficacies of reoperation. Methods From December 2007 to December 2020 , retrospective review was conducted for clinical data of 6 HD children with iatrogenic rectovaginal fistula after pull-through. The surgical procedures for repairing rectovaginal fistula included transabdominal and transanal Soave , transperineal or transanal surgery. Results A total of six girls were included. Clinical manifestation was vaginal excretion. Injuries occurred in initial surgery (n =4) and during redo pull-through (n =2). The reasons for re-operation were postoperative abdominal hemorrhage and aganglionic segment residue. Rectovaginal fistula with anastomotic retraction and stenosis were successfully repaired by transabdominal and transanal Soave procedure in single time(n =4). The remaining two cases underwent simple local repair of rectovaginal fistula , including fistula closure after anal repair twice (n =1) and failed closure after five local (perineal/anal) repairs (n =1). Conclusion Rectovaginal fistula after pull-through in HD is a serious iatrogenic injury. It should be separated close to rectal submucosa/rectal wall to avoid vaginal injury. Transabdominal and transanal Soave procedure has a high success rate for repairing rectovaginal fistula and managing anastomotic retraction and stenosis. © 2022, Journal of Clinical Pediatric Surgery. All rights reserved.     

限制性后人字入路直肠肛门成形及外括约肌成形术治疗中低位无肛合并直肠会阴瘘

目的 针对中低位无肛合并直肠会阴瘘的患儿改良一种新的手术方法,使之较现行的方法如Pena、后切术更加简便、更符合生理以减少手术并发症.方法 切口采用限制性(即限制在3 cm以内)后人字切口,显露瘘管和直肠下端,在直肠下端的后方将肛门外括约肌复合体部分切开,将两侧的肌纤维环包于直肠下端.完成外括约肌成形术,在中线后切瘘管及直肠壁,于正常肛隐窝处完成肛门成形.结果 24例先天性无肛合并直肠会阴瘘(X线侧位片直肠盲端在P-C线或至Ⅰ线间)患儿进行了该手术,术后全部病例进行随诊:除1例5岁患儿术后便秘,全部患儿恢复自主排便,可控制,无失禁及溢粪.1例患儿术后肛门切口轻度感染经切开引流治愈.结论 该手术较Pe(n)a手术操作更加简便,局部创伤小,术后避免直肠回缩,减少了术后并发症,且术后不必扩肛;不同于传统的"后切术",术中进行肛门外括约肌成形,术后随诊疗效满意.

Abstract：

Objective To study the clinical outcomes of anorectoplasty and external anal sphincterplasty via posterior approach with Y-shaped incision to repair intermediate and low anorectal malformation complicated with rectal perineal fistula in children. Methods Twenty four patients with intermediate and low anorectal malformation complicated with rectal perineal fistula were enrolled in this study. Via the posterior approach, a Y-shaped incision (＜3 cm) was made to expose the lower rectum and fistula. The external anal sphincter was identified and dissected at the posterior aspect of the lower rectum. The external anal sphincterplasty was performed by overlapping the ends of the muscle around the lower rectum. The rectal perineal fistula was resected and anorectoplasty was performed. Results All surgeries were successfully performed. Voluntary bowel movement and fecal continence was restored on 23 patients after surgery. One 5 years old patient still had constipation. Mild incision infection was found on 1 patient, and was cured after opening the incision and draining the pus. No fecal incontinence or anastomotic stricture was noted. Conclusions Compared with Pena surgery, anorectoplasty and external anal sphincterplasty via posterior approach with Y-shaped incision is less complicated and invasive to repair intermediate and low anorectal malformation complicated with rectal perineal fistula. By using this procedure, better sphincter shape and fecal function is achieved on patients.     

腹腔镜辅助高位肛门直肠畸形成形术后中期随访研究

Objective To evaluate postoperative anorectal functions of patients with high-type anoreetal malformations who underwent laparoscopically assisted anorectal pull-through (LAARP). Methods From Jan 2002 to Dec 2005, LAARP was performed on 10 patients with high-type anorectal malformations. All the patients were followed up and their clinical data were compared with patients who underwent posterior sagittal anorectoplasty (PSARP) (n = 12) performed before 2002. All pa-tients underwent colostomy at newborn period. Anorectal functions were evaluated with Kelly's clinical scoring and anoreetal manometry. At follow-up, the age of patients ranged from 3 to 5 years and from 5 to 6 years in the LAARP group and in the PSARP group respectively. Results The age at evaluation in the LAARP group was significantly younger than that in the PSARP group (47. 2 ± 8. 3 months vs 74. 5 ± 9. 3 months, P<0. 05). Kelly's clinical score was 3. 7 ± 0. 9 vs 3. 4± 0. 8 for the LAARP group and PSARP group respectively. The results of resting anal pressure and high pressure zone length showed that no significant difference was noted between the two groups, while the positive rate of rec-toanal inhibitory reflex in the LAARP group was significantly higher than that in the PSARP group (80 % vs 41%, P<0. 05). Conclusions Midterm follow-up study revealed that satisfactory fecal con-tinence can be achieved in patients with high-type anorectal malformations treated with LAARP, which may be an effective alternative. However, long-term follow-up is needed to evaluate the anorec-tal functions of patients treated with LAARP or PSARP     

危重患儿血糖与胰岛素水平的临床分析

Objective To analyse the changes of blood glucose and insulin levels in children with critical illness,and to investigate the mechanism of hyperglycemia in critical illness.Methods Blood glucose and insulin levels were detected among 51 critically ill children hospitalized in our PICU from January to December,2007,which were compared with those of 15 healthy children.Results (1) All the patients had hyperglycemia after admission within 2Ah,septic shock patients showed the highest level with the maximum value of 27.30 mmol/L The dally mean blood glucose levels of the first 5 days after admission peaked on the admission day.(2) Within 24 h after admission,the blood insulin levels of patients with pulmonary infection,intracranial infection,septic shock and congenital heart disease were(17.65±16.85) mU/L,(13.45±7.33) mU/L,(16.24±12.41) mU/L,(6.75±3.22) mU/L respectively.The blood insulin levels of all the patients within the first 5 days after admission wrere higher than that of healthy children[(8.70±6.57) mU/L].According to blood glucose level on admission day,the patients were divided into normoglycemia and hyperglycemia group,and the blood insulin levels of the former and the letter were(5.44 ± 3.38) mU/L and (14.22±12.29) mU/L respectively.(3) The mean of PIM Ⅱscore of the patients averaged 12.96±16.82,and the mortality rate was 15.6%.The blood glucose level and the insulin level within 24 h after admission were(10.97±5.76) mmol/L and(49.46±90.35) mU/L in dead cases and(8.73±2.58) mmol/L and(11.91±11.24) mU/L for the survivals,and both the blood glucose level and insulin level had significant difference between the dead cases and survivals(P ＜0.05).(4) The scatter graphic analysis did not show significant linear relation between blood glucose and insulin,nor did it show significant linear relation between PIM Ⅱ and insulin levei,or blood glucose.Condusion Hyperglycemia and hyperinsulinemia are common in critical illness,which reflects indirectly the severity and prognosis of the disease.Hyperglycemia may be related to relative insulin insufficiency or insulin resistance;however,the definite relationship can not be confirmed until more reliable clinical data were available in the future.     

危重患儿血糖与胰岛素水平的临床分析

Objective To analyse the changes of blood glucose and insulin levels in children with critical illness,and to investigate the mechanism of hyperglycemia in critical illness.Methods Blood glucose and insulin levels were detected among 51 critically ill children hospitalized in our PICU from January to December,2007,which were compared with those of 15 healthy children.Results (1) All the patients had hyperglycemia after admission within 2Ah,septic shock patients showed the highest level with the maximum value of 27.30 mmol/L The dally mean blood glucose levels of the first 5 days after admission peaked on the admission day.(2) Within 24 h after admission,the blood insulin levels of patients with pulmonary infection,intracranial infection,septic shock and congenital heart disease were(17.65±16.85) mU/L,(13.45±7.33) mU/L,(16.24±12.41) mU/L,(6.75±3.22) mU/L respectively.The blood insulin levels of all the patients within the first 5 days after admission wrere higher than that of healthy children[(8.70±6.57) mU/L].According to blood glucose level on admission day,the patients were divided into normoglycemia and hyperglycemia group,and the blood insulin levels of the former and the letter were(5.44 ± 3.38) mU/L and (14.22±12.29) mU/L respectively.(3) The mean of PIM Ⅱscore of the patients averaged 12.96±16.82,and the mortality rate was 15.6%.The blood glucose level and the insulin level within 24 h after admission were(10.97±5.76) mmol/L and(49.46±90.35) mU/L in dead cases and(8.73±2.58) mmol/L and(11.91±11.24) mU/L for the survivals,and both the blood glucose level and insulin level had significant difference between the dead cases and survivals(P ＜0.05).(4) The scatter graphic analysis did not show significant linear relation between blood glucose and insulin,nor did it show significant linear relation between PIM Ⅱ and insulin levei,or blood glucose.Condusion Hyperglycemia and hyperinsulinemia are common in critical illness,which reflects indirectly the severity and prognosis of the disease.Hyperglycemia may be related to relative insulin insufficiency or insulin resistance;however,the definite relationship can not be confirmed until more reliable clinical data were available in the future.     

危重患儿血糖与胰岛素水平的临床分析

Objective To analyse the changes of blood glucose and insulin levels in children with critical illness,and to investigate the mechanism of hyperglycemia in critical illness.Methods Blood glucose and insulin levels were detected among 51 critically ill children hospitalized in our PICU from January to December,2007,which were compared with those of 15 healthy children.Results (1) All the patients had hyperglycemia after admission within 2Ah,septic shock patients showed the highest level with the maximum value of 27.30 mmol/L The dally mean blood glucose levels of the first 5 days after admission peaked on the admission day.(2) Within 24 h after admission,the blood insulin levels of patients with pulmonary infection,intracranial infection,septic shock and congenital heart disease were(17.65±16.85) mU/L,(13.45±7.33) mU/L,(16.24±12.41) mU/L,(6.75±3.22) mU/L respectively.The blood insulin levels of all the patients within the first 5 days after admission wrere higher than that of healthy children[(8.70±6.57) mU/L].According to blood glucose level on admission day,the patients were divided into normoglycemia and hyperglycemia group,and the blood insulin levels of the former and the letter were(5.44 ± 3.38) mU/L and (14.22±12.29) mU/L respectively.(3) The mean of PIM Ⅱscore of the patients averaged 12.96±16.82,and the mortality rate was 15.6%.The blood glucose level and the insulin level within 24 h after admission were(10.97±5.76) mmol/L and(49.46±90.35) mU/L in dead cases and(8.73±2.58) mmol/L and(11.91±11.24) mU/L for the survivals,and both the blood glucose level and insulin level had significant difference between the dead cases and survivals(P ＜0.05).(4) The scatter graphic analysis did not show significant linear relation between blood glucose and insulin,nor did it show significant linear relation between PIM Ⅱ and insulin levei,or blood glucose.Condusion Hyperglycemia and hyperinsulinemia are common in critical illness,which reflects indirectly the severity and prognosis of the disease.Hyperglycemia may be related to relative insulin insufficiency or insulin resistance;however,the definite relationship can not be confirmed until more reliable clinical data were available in the future.     

危重患儿血糖与胰岛素水平的临床分析

Objective To analyse the changes of blood glucose and insulin levels in children with critical illness,and to investigate the mechanism of hyperglycemia in critical illness.Methods Blood glucose and insulin levels were detected among 51 critically ill children hospitalized in our PICU from January to December,2007,which were compared with those of 15 healthy children.Results (1) All the patients had hyperglycemia after admission within 2Ah,septic shock patients showed the highest level with the maximum value of 27.30 mmol/L The dally mean blood glucose levels of the first 5 days after admission peaked on the admission day.(2) Within 24 h after admission,the blood insulin levels of patients with pulmonary infection,intracranial infection,septic shock and congenital heart disease were(17.65±16.85) mU/L,(13.45±7.33) mU/L,(16.24±12.41) mU/L,(6.75±3.22) mU/L respectively.The blood insulin levels of all the patients within the first 5 days after admission wrere higher than that of healthy children[(8.70±6.57) mU/L].According to blood glucose level on admission day,the patients were divided into normoglycemia and hyperglycemia group,and the blood insulin levels of the former and the letter were(5.44 ± 3.38) mU/L and (14.22±12.29) mU/L respectively.(3) The mean of PIM Ⅱscore of the patients averaged 12.96±16.82,and the mortality rate was 15.6%.The blood glucose level and the insulin level within 24 h after admission were(10.97±5.76) mmol/L and(49.46±90.35) mU/L in dead cases and(8.73±2.58) mmol/L and(11.91±11.24) mU/L for the survivals,and both the blood glucose level and insulin level had significant difference between the dead cases and survivals(P ＜0.05).(4) The scatter graphic analysis did not show significant linear relation between blood glucose and insulin,nor did it show significant linear relation between PIM Ⅱ and insulin levei,or blood glucose.Condusion Hyperglycemia and hyperinsulinemia are common in critical illness,which reflects indirectly the severity and prognosis of the disease.Hyperglycemia may be related to relative insulin insufficiency or insulin resistance;however,the definite relationship can not be confirmed until more reliable clinical data were available in the future.     

危重患儿血糖与胰岛素水平的临床分析

Objective To analyse the changes of blood glucose and insulin levels in children with critical illness,and to investigate the mechanism of hyperglycemia in critical illness.Methods Blood glucose and insulin levels were detected among 51 critically ill children hospitalized in our PICU from January to December,2007,which were compared with those of 15 healthy children.Results (1) All the patients had hyperglycemia after admission within 2Ah,septic shock patients showed the highest level with the maximum value of 27.30 mmol/L The dally mean blood glucose levels of the first 5 days after admission peaked on the admission day.(2) Within 24 h after admission,the blood insulin levels of patients with pulmonary infection,intracranial infection,septic shock and congenital heart disease were(17.65±16.85) mU/L,(13.45±7.33) mU/L,(16.24±12.41) mU/L,(6.75±3.22) mU/L respectively.The blood insulin levels of all the patients within the first 5 days after admission wrere higher than that of healthy children[(8.70±6.57) mU/L].According to blood glucose level on admission day,the patients were divided into normoglycemia and hyperglycemia group,and the blood insulin levels of the former and the letter were(5.44 ± 3.38) mU/L and (14.22±12.29) mU/L respectively.(3) The mean of PIM Ⅱscore of the patients averaged 12.96±16.82,and the mortality rate was 15.6%.The blood glucose level and the insulin level within 24 h after admission were(10.97±5.76) mmol/L and(49.46±90.35) mU/L in dead cases and(8.73±2.58) mmol/L and(11.91±11.24) mU/L for the survivals,and both the blood glucose level and insulin level had significant difference between the dead cases and survivals(P ＜0.05).(4) The scatter graphic analysis did not show significant linear relation between blood glucose and insulin,nor did it show significant linear relation between PIM Ⅱ and insulin levei,or blood glucose.Condusion Hyperglycemia and hyperinsulinemia are common in critical illness,which reflects indirectly the severity and prognosis of the disease.Hyperglycemia may be related to relative insulin insufficiency or insulin resistance;however,the definite relationship can not be confirmed until more reliable clinical data were available in the future.     

危重患儿血糖与胰岛素水平的临床分析

Objective To analyse the changes of blood glucose and insulin levels in children with critical illness,and to investigate the mechanism of hyperglycemia in critical illness.Methods Blood glucose and insulin levels were detected among 51 critically ill children hospitalized in our PICU from January to December,2007,which were compared with those of 15 healthy children.Results (1) All the patients had hyperglycemia after admission within 2Ah,septic shock patients showed the highest level with the maximum value of 27.30 mmol/L The dally mean blood glucose levels of the first 5 days after admission peaked on the admission day.(2) Within 24 h after admission,the blood insulin levels of patients with pulmonary infection,intracranial infection,septic shock and congenital heart disease were(17.65±16.85) mU/L,(13.45±7.33) mU/L,(16.24±12.41) mU/L,(6.75±3.22) mU/L respectively.The blood insulin levels of all the patients within the first 5 days after admission wrere higher than that of healthy children[(8.70±6.57) mU/L].According to blood glucose level on admission day,the patients were divided into normoglycemia and hyperglycemia group,and the blood insulin levels of the former and the letter were(5.44 ± 3.38) mU/L and (14.22±12.29) mU/L respectively.(3) The mean of PIM Ⅱscore of the patients averaged 12.96±16.82,and the mortality rate was 15.6%.The blood glucose level and the insulin level within 24 h after admission were(10.97±5.76) mmol/L and(49.46±90.35) mU/L in dead cases and(8.73±2.58) mmol/L and(11.91±11.24) mU/L for the survivals,and both the blood glucose level and insulin level had significant difference between the dead cases and survivals(P ＜0.05).(4) The scatter graphic analysis did not show significant linear relation between blood glucose and insulin,nor did it show significant linear relation between PIM Ⅱ and insulin levei,or blood glucose.Condusion Hyperglycemia and hyperinsulinemia are common in critical illness,which reflects indirectly the severity and prognosis of the disease.Hyperglycemia may be related to relative insulin insufficiency or insulin resistance;however,the definite relationship can not be confirmed until more reliable clinical data were available in the future.     

Resilience in Families with a Child with Cancer

The aim of this study was to identify and explore resilience factors associated with family adaption after a child had been diagnosed with cancer. Using a cross-sectional survey research design, parents (n = 26), and children (n = 25) from the same families independently completed six self-report questionnaires, as well as responded to an open-ended question about those qualities that helped their family through the period following the diagnosis. The most significant results came from the children's data. According to these results, connectedness within the family, the experience of control over life events, family routines, positive, and supportive communication, redefinition of crisis situations, and lastly, a passive appraisal of crisis situations, were positively linked to better family adaptation. The identified factors should be strengthened and developed in families finding themselves in a similar situation.     

Colonization with Clostridium difficile in Children with Cancer

Objective

Clostridium difficile is a gram-positive, anaerobic, spore-forming bacillus. Usually it does not cause disease unless a patient who is colonized with toxin-producing strains has been treated with antibiotics, particularly those that change the anaerobic flora of the large intestine.

Methods

We investigated in a prospective study intestinal colonization of C. difficile and its toxins in children with malignancy that used different antibiotics and cytotoxic drugs.

Findings

One hundred fifty-two patients were included in this prospective study. Stool samples were obtained within the first 48 hours after admission and cultured for C. difficile; cytopathic effect of C. difficile was detected on HELA cells, also ELISA test was performed for detection of toxins A and B. 25% of patients had positive culture for C. difficile; 36/38 (92%) revealed positive cytopathic effect on HELA cells. No significant relation was found between age, gender, history of antibiotic consumption and C. difficile positive culture and cytopathic effect on HELA cells. The only relation was seen between cotrimoxazol usage and cytopathic effect on HELA cells (P=0.03).

Conclusion

Although the rate of C. difficile colonization (25.6%) and toxigenic strains (23.7%) in admitted children in hematologic ward is high, the rate of ELISA positive test for toxin A+B was not correspond with culture and cytopatic effect on HELA cell. With respect to sensitivity and specificity of ELISA test, possibility for existence of toxin C with cytopathic effect is high in this type of patients.     

Treatment with Probucol of Children with Familial Hypercholesterolemia

ABSTRACT. Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2±1.45 mmol/l to 7.17±0.84 mmol/l (12.6%). This level was further reduced to 5.92±0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.     

Therapeutic experience with hepatoblastoma associated with trisomy 18

Trisomy 18 is often fatal, but patients with this disease can now have longer survival due to proactive treatment intervention. However, hepatoblastomas may develop in these patients. In this study, we report four cases of hepatoblastoma associated with trisomy 18. All of the patients had congenital heart disease and three had undergone intracardiac surgical repair. Tumor growth was relatively slow in all cases, and there were no problems with chemotherapy tolerability and surgical resection. Three of the patients are currently disease‐free and the fourth is alive with remaining of the tumor. These cases suggest that combined chemotherapy and surgical resection may be an option to treat hepatoblastoma associated with trisomy 18 when cardiac pulmonary function is relatively stable.     

Juvenile rheumatoid arthritis with myelofibrosis with myeloid metaplasia

Myelofibrosis with myeloid metaplasia is defined as a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extramedullary hematopoesis and varying degree of myelofibrosis. It may be idiopathic or secondary to a large number of conditions. Here is a rare case of myelofibrosis occurring in a patient with juvenile rheumatoid arthritis.     

Tyrosinemia associated with perinatal infection with cytomegalovirus.

A premature infant presented with elevated concentrations of tyrosine in blood and urine, evidence of hepatocellular damage, demineralization of the bones, and a renal Fanconi syndrome. This is the clinical picture found in hereditary tyrosinemia. The infant also had a perinatal infection with cytomegalovirus.