Objective: To retrospectively study the early compli-cations of excision with hepaticoenterostomy for in-fants and children with choledochal cysts.Methods: We analyzed 16 patients with early posto-perative complicatio...Objective: To retrospectively study the early compli-cations of excision with hepaticoenterostomy for in-fants and children with choledochal cysts.Methods: We analyzed 16 patients with early posto-perative complications out of 173 patients with con-genital choledochal cysts aged 27 days to 14 years(mean 2.4 years) who had undergone excisional pro-cedures and biliary tract reconstruction.Results: The early complications included bile leak-age (10 patients), abdominal wall dehiscence (3),and hepatic failure, pancreatic juice leakage andpostoperative intussusception (each in 1) respective-ly. Three patients died from bile leakage and 1 frompostoperative hepatic failure. No statistical differen-ces were observed between the procedures of biliarytract reconstruction with jejunal segment interposi-tion hepaticoduodenostomy and Roux-en-Y hepatico-jejunostomy (P】0.75). The morbidity was signifi-cantly higher in infants below 1 year than in children(P【0.005). Prevention and treatment of the com-plications were discussed.Conclusion: Bile leakage and abdominal wall dehis-cence are major early postoperative complications.The morbidity of cholechal cysts is higher in infantsthan in children. Exploratory laparotomy should notbe delayed when biliary leakage with diffuse peritoni-tis appears. The "tension suture in the fascial spaceof the abdominal wall" is useful to prevent and treatwound dehiscence.展开更多
Background Hirschsprung's disease(HSCR)is one of the most common congenital digestive tract malformations and can cause stubborn constipation or gastrointestinal obstruction after birth,causing great physical and ...Background Hirschsprung's disease(HSCR)is one of the most common congenital digestive tract malformations and can cause stubborn constipation or gastrointestinal obstruction after birth,causing great physical and mental pain to patients and their families.Studies have shown that more than 20 genes are involved in HSCR,and most cases of HSCR are sporadic.However,the overall rate of familial recurrence in 4331 cases of HSCR is about 7.6%.Furthermore,familial HSCR patients show incomplete dominance.We still do not know the penetrance and genetic characteristics of these known risk genes due to the rarity of HSCR families.Methods To find published references,we used the title/abstract terms"Hirschsprung"and"familial"in the PubMed data-base and the MeSH terms"Hirschsprung"and"familial"in Web of Science.Finally,we summarized 129 HSCR families over the last 40 years.Results The male-to-female ratio and the percentage of short segment-HSCR in familial HSCR are much lower than in sporadic HSCR.The primary gene factors in the syndromic families are ret proto-oncogene(RET)and endothelin B receptor gene(EDNRB).Most families show incomplete dominance and are relevant to RET,and the RET mutation has 56%pen-etrance in familial HSCR.When one of the parents is a RET mutation carrier in an HSCR family,the offspring's recurrence risk is 28%,and the incidence of the offspring does not depend on whether the parent suffers from HSCR.Conclusion Our findings will help HSCR patients obtain better genetic counseling,calculate the risk of recurrence,and provide new insights for future pedigree studies.展开更多
Background To determine whether ex utero intrapartum treatment (EXIT) is an appropriate approach for managing fetuses antenatally diagnosed with giant congenital omphaloceles. Methods We retrospectively reviewed patie...Background To determine whether ex utero intrapartum treatment (EXIT) is an appropriate approach for managing fetuses antenatally diagnosed with giant congenital omphaloceles. Methods We retrospectively reviewed patients with omphaloceles who underwent either an EXIT procedure or a traditional repair surgery. Basic and clinical parameters including gender, gestational age, birth weight, maternal blood loss, operative times and operative complications were analyzed. During the 6–12-month follow-ups, postoperative complications including bowel obstruction, abdominal infections, postoperative abdominal distension were monitored, and survival rate was analyzed. Results A total of seven patients underwent the EXIT procedure and 11 patients underwent the traditional postnatal surgery. We found no differences in maternal age, gestational age at diagnosis, gestational age at delivery and birth weight between the two groups. In the EXIT group, the average operation time for mother was 68.3 ± 17.5 minutes and the average maternal blood loss was 233.0 ± 57.7 mL. The operation time in the EXIT group (22.0 ± 4.5 minutes) was shorter than that in the traditional group (35 ± 8.7 minutes), but the length of hospital stay in the EXIT group (20.5 ± 3.1 days) was longer than that in the traditional group (15.7 ± 2.5 days,P < 0.05). During the follow-up, one patient in the EXIT group had an intestinal obstruction, one developed abdominal compartment syndrome and one died in the traditional group. Conclusions In our experience, EXIT is a safe and effective procedure for the treatment of giant congenital omphaloceles. However, more experience is needed before this procedure can be widely recommended.展开更多
Background:This study was undertaken to retrospectively analyze the accuracy of different methods in differentiating biliary atresia from neonatal jaundice.Methods:A search was made in MEDLINE,and the Web of Science f...Background:This study was undertaken to retrospectively analyze the accuracy of different methods in differentiating biliary atresia from neonatal jaundice.Methods:A search was made in MEDLINE,and the Web of Science for relevant original articles published in English;methodological quality of the included studies was also assessed.Two reviewers extracted data independently.Studies were pooled,summary receiver operating characteristics curve and diagnostic odds ratio(DOR)with corresponding confi dence intervals were calculated.Results:For diagnosis of biliary atresia,ultrasonography(US),hepatic scintigraphy(HBS),and magnetic resonance cholangiography(MRCP)had a pooled sensitivity of 74.9%(range:70.4%-79.1%),93.4%(range:90.3%-95.7%)and 89.7%(range:84.8%-93.4%),a specificity of 93.4%(range:91.4%-95.1%),69.2%(range:65.1%-73.1%)and 64.7%(range:58.0%-71.0%),a positive likelihood ratio of 12.16(range:6.41-23.08),3.01(range:2.15-4.20)and 3.10(range:1.59-6.06),a negative likelihood ratio of 0.23(range:0.13-0.38),0.13(range:0.06-0.25)and 0.16(range:0.06-0.44),DOR of 72.56(range:27.34-192.58),29.88(range:12.82-69.64)and 32.48(range:8.22-128.29),with an area under the curve of 0.96,0.91,and 0.92,and Q value of 0.90,0.85,and 0.85,respectively.Conclusions:US,HBS and MRCP can be very useful for the diagnostic work-up of neonatal cholestasis.To improve the sensitivity and specificity,several additional measures can be used.展开更多
文摘Objective: To retrospectively study the early compli-cations of excision with hepaticoenterostomy for in-fants and children with choledochal cysts.Methods: We analyzed 16 patients with early posto-perative complications out of 173 patients with con-genital choledochal cysts aged 27 days to 14 years(mean 2.4 years) who had undergone excisional pro-cedures and biliary tract reconstruction.Results: The early complications included bile leak-age (10 patients), abdominal wall dehiscence (3),and hepatic failure, pancreatic juice leakage andpostoperative intussusception (each in 1) respective-ly. Three patients died from bile leakage and 1 frompostoperative hepatic failure. No statistical differen-ces were observed between the procedures of biliarytract reconstruction with jejunal segment interposi-tion hepaticoduodenostomy and Roux-en-Y hepatico-jejunostomy (P】0.75). The morbidity was signifi-cantly higher in infants below 1 year than in children(P【0.005). Prevention and treatment of the com-plications were discussed.Conclusion: Bile leakage and abdominal wall dehis-cence are major early postoperative complications.The morbidity of cholechal cysts is higher in infantsthan in children. Exploratory laparotomy should notbe delayed when biliary leakage with diffuse peritoni-tis appears. The "tension suture in the fascial spaceof the abdominal wall" is useful to prevent and treatwound dehiscence.
基金National Natural Science Foundation of China(82071685 to FJX)Clinical Research Pilot Project of Tongji Hospital(2019YBKY026 to FJX)+2 种基金Provincial Key Research and Development Program(2020BCB008 to FJX)Science and Technology Innovation Base Platform(2020DCD006 to FJX)Project of Shenzhen San Ming(SZSM201812055 to FJX).
文摘Background Hirschsprung's disease(HSCR)is one of the most common congenital digestive tract malformations and can cause stubborn constipation or gastrointestinal obstruction after birth,causing great physical and mental pain to patients and their families.Studies have shown that more than 20 genes are involved in HSCR,and most cases of HSCR are sporadic.However,the overall rate of familial recurrence in 4331 cases of HSCR is about 7.6%.Furthermore,familial HSCR patients show incomplete dominance.We still do not know the penetrance and genetic characteristics of these known risk genes due to the rarity of HSCR families.Methods To find published references,we used the title/abstract terms"Hirschsprung"and"familial"in the PubMed data-base and the MeSH terms"Hirschsprung"and"familial"in Web of Science.Finally,we summarized 129 HSCR families over the last 40 years.Results The male-to-female ratio and the percentage of short segment-HSCR in familial HSCR are much lower than in sporadic HSCR.The primary gene factors in the syndromic families are ret proto-oncogene(RET)and endothelin B receptor gene(EDNRB).Most families show incomplete dominance and are relevant to RET,and the RET mutation has 56%pen-etrance in familial HSCR.When one of the parents is a RET mutation carrier in an HSCR family,the offspring's recurrence risk is 28%,and the incidence of the offspring does not depend on whether the parent suffers from HSCR.Conclusion Our findings will help HSCR patients obtain better genetic counseling,calculate the risk of recurrence,and provide new insights for future pedigree studies.
基金supported by Grants from the National Natural Science Foundation of China(Nos.81270441,81401240)
文摘Background To determine whether ex utero intrapartum treatment (EXIT) is an appropriate approach for managing fetuses antenatally diagnosed with giant congenital omphaloceles. Methods We retrospectively reviewed patients with omphaloceles who underwent either an EXIT procedure or a traditional repair surgery. Basic and clinical parameters including gender, gestational age, birth weight, maternal blood loss, operative times and operative complications were analyzed. During the 6–12-month follow-ups, postoperative complications including bowel obstruction, abdominal infections, postoperative abdominal distension were monitored, and survival rate was analyzed. Results A total of seven patients underwent the EXIT procedure and 11 patients underwent the traditional postnatal surgery. We found no differences in maternal age, gestational age at diagnosis, gestational age at delivery and birth weight between the two groups. In the EXIT group, the average operation time for mother was 68.3 ± 17.5 minutes and the average maternal blood loss was 233.0 ± 57.7 mL. The operation time in the EXIT group (22.0 ± 4.5 minutes) was shorter than that in the traditional group (35 ± 8.7 minutes), but the length of hospital stay in the EXIT group (20.5 ± 3.1 days) was longer than that in the traditional group (15.7 ± 2.5 days,P < 0.05). During the follow-up, one patient in the EXIT group had an intestinal obstruction, one developed abdominal compartment syndrome and one died in the traditional group. Conclusions In our experience, EXIT is a safe and effective procedure for the treatment of giant congenital omphaloceles. However, more experience is needed before this procedure can be widely recommended.
文摘Background:This study was undertaken to retrospectively analyze the accuracy of different methods in differentiating biliary atresia from neonatal jaundice.Methods:A search was made in MEDLINE,and the Web of Science for relevant original articles published in English;methodological quality of the included studies was also assessed.Two reviewers extracted data independently.Studies were pooled,summary receiver operating characteristics curve and diagnostic odds ratio(DOR)with corresponding confi dence intervals were calculated.Results:For diagnosis of biliary atresia,ultrasonography(US),hepatic scintigraphy(HBS),and magnetic resonance cholangiography(MRCP)had a pooled sensitivity of 74.9%(range:70.4%-79.1%),93.4%(range:90.3%-95.7%)and 89.7%(range:84.8%-93.4%),a specificity of 93.4%(range:91.4%-95.1%),69.2%(range:65.1%-73.1%)and 64.7%(range:58.0%-71.0%),a positive likelihood ratio of 12.16(range:6.41-23.08),3.01(range:2.15-4.20)and 3.10(range:1.59-6.06),a negative likelihood ratio of 0.23(range:0.13-0.38),0.13(range:0.06-0.25)and 0.16(range:0.06-0.44),DOR of 72.56(range:27.34-192.58),29.88(range:12.82-69.64)and 32.48(range:8.22-128.29),with an area under the curve of 0.96,0.91,and 0.92,and Q value of 0.90,0.85,and 0.85,respectively.Conclusions:US,HBS and MRCP can be very useful for the diagnostic work-up of neonatal cholestasis.To improve the sensitivity and specificity,several additional measures can be used.
