Comparison of robot-assisted single-port-plus-one pyeloplasty vs. laparoscopic single-port pyeloplasty in the treatment of ureteropelvic junction obstruction in children.

Objective: To compare the efficacy of robot-assisted single-port-plus-one pyeloplasty (RSPY) and laparoscopic single-port pyeloplasty (LSPY) in the treatment of children with ureteropelvic junction obstruction (UPJO). Methods: The clinical data of 47 children who underwent surgery for UPJO at the Department of the Pediatric Surgery of the Fujian Provincial Hospital from October 2020 to September 2022 were analyzed retrospectively. Of these 47 children, 27 received RSPY while 20 underwent LSPY. The baseline data, operation time, intraoperative anastomosis time, intraoperative blood loss, postoperative hospital stay, complications, total cost, preoperative and postoperative renal parenchymal thickness (PT), anteroposterior renal pelvis diameter (APD), and differential renal function (DRF) of the two groups were compared to evaluate the clinical efficacy of the two surgical methods. Results: The results showed that both surgical techniques were successful and no patient transitioned to open surgery. There was no significant difference between the two groups in baseline data, intraoperative blood loss, complications, APD, and PT 6 months after surgery. There was also no significant difference in APD, PT, and DRF 12 months after surgery (all P > 0.05). Compared with the LSPY group, the RSPY group had shorter operation time [(153.04 ± 14.44) vs. (189.90 ± 32.59) min, t = -5.24, P < 0.05], less intraoperative anastomosis time [(68.81 ± 16.80) vs. (97.45 ± 11.99) min, t = -6.49, P < 0.05], shorter postoperative hospital stay [(5.96 ± 1.34) vs. (9.00 ± 1.33) d, t = -7.68, P < 0.05], but higher total cost [(57,390 ± 7,664) vs. (30,183 ± 4,219) yuan, t = 14.32, P < 0.05]. Conclusion: Compared with LSPY, RSPY achieves equivalent efficacy in the treatment of UPJO in children and has certain advantages by shortening the operation time, intraoperative anastomosis time, and postoperative hospital stay. However, its cost burden is heavy, and appropriate cases need to be selected for popularization and application.

Analysis of the Clinical Characteristics of Spontaneous Bile Duct Perforation in Children.

Objective: This study aimed to explore the etiology, clinical features, diagnosis, and treatment of spontaneous bile duct perforation (SBDP) in children. Methods: The clinical data of children with SBDP who were admitted to Wuhan Children's Hospital between January 2014 and January 2020 were retrospectively analyzed. Results: In all, 28 cases of children with SBDP (male, 28.6%; female, 71.4%; male-to-female ratio, 1:2.5; average age, 2.15 years) were analyzed. The most common symptoms were fever (85.7%), nausea and vomiting (78.6%), and abdominal distension (67.9%). Among the 28 patients, 26 (92.9%) had elevated hypersensitive C-reactive protein, 24 (85.7%) had an increased neutrophil percentage, and 22 (78.6%) had raised peripheral blood leukocyte counts. Moreover, 19 patients (67.9%) showed increased serum total bilirubin levels, and 5 (17.9%) showed an elevated conjugated bilirubin level. Abdominal CT examination revealed that the gallbladder wall of patients was thickened with edema, accompanied by gallbladder stenosis and gallbladder mucosa enhancement; furthermore, ascites was found in the abdominal cavity and lesser omental bursa. Twenty-two patients underwent abdominal paracentesis, and 20 (90.9%) of them were exposed to bile-based ascites. Among the 28 patients, four recovered with conservative treatment, whereas the others (85.7%) were surgically treated. Of the twenty-four patients undergoing surgery, the perforation site was found at the union of the hepatic and cystic ducts in 12 patients (50%), no perforation site was observed in 9 patients (37.5%), and a common hepatic duct was observed in 3 patients (12.5%). All 24 patients underwent stage I surgery, and temporary biliary drainage was performed because of severe abdominal inflammation. Cholangiography and enhanced CT revealed an abnormal location of the pancreatic duct joining the bile duct in 64.3% patients. Following surgery, 15 patients underwent hepaticojejunostomy. Subsequently, 3-month to 6-year follow-up (median, 30 months) indicated that the patients recovered well with no serious complications. Conclusion: SBDP in children may be associated with pancreaticobiliary malunion (PBM) and congenital weakness of the bile duct wall. However, the clinical manifestations of this condition lack specificity; this limitation can be assisted through diagnosis via abdominal CT and by performing abdominal paracentesis. Once SBDP diagnosis is confirmed, the patient should follow the principles of individualized treatment.

Risk factors of recurrent acute pancreatitis after conservative treatment in pediatric population: a single-center study.

Background: Although complete resolution and recovery occurs in most children with an initial attack of acute pancreatitis (AP), a subset of children may progress to recurrent AP (RAP). RAP has serious effects to the individual and the socioeconomic burden. The aim of this project was to identify the independent risk factors for pediatric RAP so as to provide evidence for its prevention, early diagnosis and treatment. Methods: A retrospective cohort study of children discharged from Tianjin Children's Hospital from June 2017 to January 2020 was performed. Demographic and clinical variables, treatment strategies, clinical course and outcomes were collected. Independent risk factors of RAP were identified using the logistic regression model. Results: Of the total 96 enrolled children, 30 (31.3%) developed RAP during the follow-up period. The majority (27/30, 90%) of the children with AP developed RAP within 6 months of their first AP attack. The presence of systemic inflammatory response syndrome (SIRS) [odds ratio (OR)=6.652, 95% confidence interval (CI) 1.989 to 22.247], fasting time (OR=1.267, 95% CI 1.104 to 1.583), whether meet all three AP diagnostic criteria (OR=7.438, 95% CI 1.346 to 41.103) and abnormal amylase/lipase value on the seventh day of hospitalization (OR=3.601, 95% CI 0.972 to 13.342) were independent risk factors of RAP in children. Conclusions: Most children who developed RAP had progressed within 6 months after their first episode of AP. RAP was more common in children who met all three AP diagnostic criteria at initial attack and in children with SIRS, long fasting time and abnormal amylase/lipase value on the seventh day of hospitalization.

Biliary atresia in twins'population: a retrospective multicenter study in mainland China.

AIM: We evaluated the demographic of biliary atresia (BA) children from twins family and aimed to investigated what it can add to the twins' literature and our understanding of the disease. METHODS: This study contains 11 medical centers in mainland China and the medical record of twins with BA was retrospectively analyzed from January 2012 to December 2018. Follow-up was carried out by out-patient review and questionnaire. RESULTS: The study included 19 twin pairs in whom there was discordance for BA. Sixteen (84.2%) affected twin underwent Kasai Procedure (KP); median age at KP was 78 (49-168) days. There were ten affected twins that became jaundice-free at 3 months post-KP, and eight occurred with different degrees of cholangitis post-KP. Six affected twins received Liver Transplantation (LT) successfully. The 2 year native liver survival rate and the 2 year overall survival rate of affected twins were 61.1 and 94.4%, respectively. There were three affected monozygotic (MZ) twins and one healthy co-twin with BA-associated congenital malformations, all of which were cardiac malformations. The number of virus infection of affected MZ twins was significantly more (p = 0.04) than affected dizygotic (DZ) twin. CONCLUSIONS: Discordance for BA in 19 pairs of twins supported that BA may be related to genetic phenotype or penetrance. The difference in genetic background between MZ and DZ affects the susceptibility of the host to virus infection. High acceptance of KP (84.2%) in our study implied a high motivation for treatment for twins with BA. Delays of KP (78 days) in affected twin may be related to the postnatal gradual onset and the late diagnosis.

Investigation into multi-centre diagnosis and treatment strategies of biliary atresia in mainland China.

BACKGROUND: Biliary atresia (BA) is an obstructive hepatobiliary disease which manifests during infancy. Kasai portoenterostomy (KPE) is the preferred operation for BA, supplemented with glucocorticoids, antibiotics, and choleretic agents. A great deal of research has been carried out regarding diagnosis, operation, and adjuvant therapies of BA, but no consensus had been reached. To understand the variation in diagnosis and treatment strategies of BA across mainland China and to help achieve a unified treatment strategy in the future, this investigation was carried out. METHODS: This investigation was conducted via electronic questionnaire. The centres were divided into three groups based on their annual caseload: low (0-20)-, mid (21-40)-, and high (≥ 41)-volume group. Differences in the clinical practice among three groups were analyzed by Chi-square test and considered statistically significant at P < 0.05. RESULTS: 41 Centres from 26 different administrative regions were involved. The average age at KPE was mainly 51-60 days (39%, 16/41) and 61-70 days (32%, 13/41). The annual caseload was 0-20 patients in 17 centres, 21-40 patients in 11 centres, and > 40 patients in 13 centres. Preoperative ultrasound and intraoperative cholangiography were performed in all centres. Low-volume centres had a high proportion of MRI (P = 0.005), while the high-volume group had a high proportion of LSM (P = 0.015). Open KPE without liver mobilisation is the most common surgical procedure (71%, 29/41). Open KPE without liver mobilisation was more commonly used in low-volume group (P = 0.044), and laparoscopic KPE was mainly used in high-volume group (P = 0.011). The spur anti-reflux intestinal valve was performed in more than half of the centres (51%, 21/41). The length of the Roux-en-Y loop was ≥ 30 cm in the majority of centres (78%, 32/41). Glucocorticoids and antibiotics were used in most centres (90%, 37/41; 100%, 41/41) with marked variations in type, administration, dose, and duration. Oral ursodeoxycholic acid (UDCA) was used in 38 centres, in varying doses of 10-20 mg/kg/day. The duration of oral UDCA was over a year in 19 centres. CONCLUSION: Mainland China has a large number of patients with biliary atresia. Diagnostic and surgical methods vary from centre to centre and are related to its caseload. In most centres, KPE is supplemented with glucocorticoids, antibiotics, and choleretic agents without a standard regimen.

Case report: maple syrup urine disease with a novel DBT gene mutation.

BACKGROUND: Maple syrup urine disease (MSUD) is a potentially life-threatening metabolic disorder caused by decreased activity of the branched-chain α-ketoacid dehydrogenase (BCKD) complex. Mutations in four genes (BCKDHA, BCKDHB, DLD and DBT) are associated with MSUD. Here, the presenting symptoms and clinical course of a case of MSUD with a novel DBT gene mutation are described. CASE PRESENTATION: We describe an infant with MSUD with the DBT gene mutation who had drowsiness and poor appetite as well as abnormal findings upon head magnetic resonance imaging (MRI), plasma amino acid analysis and urine organic acid analysis. Genetic testing revealed that both parents had the heterozygous mutation c.1132C > T (p.378X) in chr1:100672078, and the patient had the homozygous mutations c.1132C > T (p.378X) in chr1:100672078. Once diagnosed with MSUD, the patient's disease was controlled with a diet of BCAA-free enteral formula and thiamine. CONCLUSION: The mutation c.1132C > T (p.378X) is a novel DBT gene mutation that is associated with MSUD and always has mild clinical manifestations. After timely BCAA-free nutrition and supplementation with thiamine for the patient, the plasma levels of BCAAs reached a safe level, the abnormal range of the multiple intracranial abnormalities was significantly smaller than before, and the symptoms of drowsiness and poor appetite disappeared.