The second edition of the expert consensus on pediatric nutrition was formed based on a global update of pediatric nutrition guidelines or consensus worldwide, the management of congenital heart disease, and the results of multi-center clinical nutrition research for congenital heart disease following the first Chinese consensus edition of 2016. The consensus was also shaped by the results of three discussion sessions and two questionnaires conducted by the 13-member collaboration group. This process was informed by both clinical guidelines and expert consensus. The quality of literature, both in English and Chinese, and the level of recommendations were evaluated using the Grading of Recommendations Assessment, Development, and Evaluations (GRADE) system.
Objective:To explore the association between serum level of alpha-fetoprotein (AFP) at the end of first-line therapy and the prognosis of patients with hepatoblastoma (HB).Methods:From December 2007 to May 2022, the relevant clinical data were retrospectively reviewed for 72 HB patients aged under 18 year. There were 42 boys and 30 girls with a median diagnostic age at 27.8(0.3-150.3) month. And 69.4% of them were aged under 3 year. All of them completed a first-line therapy after a definite diagnosis. Two major outcome parameters were event-free survival (EFS) and overall survival (OS). Chi-square test, survival analysis and COX proportional hazards model were utilized for evaluating the risk factors of prognosis of HB patients.Results:Two, 14, 25 and 31 patients were classified into very-low-risk, low-risk, median-risk and high-risk groups. After a first-line therapy, 46/72 patients (63.9%) had serum level of AFP ≤10 ng/ml and 55/72 patients (76.4%) achieved complete remission (CR) as evaluated by imaging. Recurrence rate in individuals with AFP >10 ng/ml was significantly higher than those with AFP ≤10 ng/ml (65.4% vs 15.2%), 3-year event-free survival (3yr-EFS) was 31.4% and 80.7% ( P<0.01) and overall survival (OS) 63.0% and 96.6% ( P<0.01). For CR cases, 3yr-EFS/OS was higher among individuals with AFP≤10 ng/ml than those with AFP >10 ng/ml. Even without CR, 3yr-EFS was considerably higher in AFP ≤10 ng/ml group. Multivariate analysis indicated that AFP >10 ng/ml after a first-line therapy was an independent predictor. Conclusion:The prognosis of HB patients without serum AFP≤10 ng/ml after a first-line therapy remains poor and treatment should be intensified.
Currarino syndrome (CS) is a rare congenital malformation characterized by three major clinical features of sacral agenesis, anorectal malformations and presacral mass.It can be divided into complete, mild and minimal CS according to clinical features.Besides the above triad, some patients may suffer from some abnormalities of multi-systems.MNX1 gene is closely correlated with the disease.The authors searched and collected the clinical and genotypic profiles of 297 mutation carriers.A total of 145 different MNX1 pathogenic variants related to CS have been reported in the literature so far.And patient phenotypes and clinical characteristics were recorded.No significant correlation existed between CS type and the location of mutation sites.However, chromosomal variation such as microdeletion of MNX1 gene coded for more serious phenotype.The distribution of mutation sites was concentrated in coding region of the homeodomain in MNX1.Mutations within homeodomain may alter DNA binding specificity.Furthermore, a rare homozygous mutation carrier of MNX1 hinted at a priority for islet function in CS patients.Different phenotypes of individuals carrying the same mutation within the same family may implicate the involvements of other regulators in the pathogenic mechanism.A new clue was detected for candidate genes in CS pathogenesis through enrichment analysis of 491 proteins interacting with MNX1 in early human embryonic developmental process.More attention should be devoted to the developments of pancreas, sensory organs and reproductive structures.
Biliary atresia (BA) refers to a cholestatic disease involving intrahepatic and extrahepatic bile ducts.Its etiology has remained elusive.Kasai operation and liver transplantation are sequential treatments.Eventually BA children die from end-stage liver diseases under an age of 2 years if not treated promptly.They tend to require comprehensive postoperative managements, including standardized vaccination.Yet there is an ongoing controversy on the specific implementations.Therefore this review focused upon current vaccination efforts and summarized expert recommendations to provide rationales for standardized postoperative vaccination of BA children.
Objective:To explore the clinical features of accessory spleen in hereditary spherocytosis (HS) and track the prognosis of children with residual accessory spleen after splenectomy to improve the diagnosis and treatment of HS.Methods:The relevant clinical data were retrospectively reviewed for 54 HS children undergoing splenectomy from January 2017 to December 2021.There were 30 boys and 24 girls with an average operative age of (8.2±3.5) years.The imaging data and clinical characteristics of accessory spleen were recorded.The prognosis was evaluated by either telephone or WeChat, including postoperative severe infection and recurrence of hemolytic anemia.The followed-up results of children with residual accessory spleen were also analyzed.Results:Accessory spleen was detected by preoperative examination in 26(26/54, 48.1%) children, including 4(4/26, 15.4%) in splenic hilum.There were 16, 6 and 3 cases with 1, 2 and 3 accessory spleens respectively.A total of 37 accessory spleens were identified, predominantly in splenic hilum (30/37, 81.1%) and ienocolic ligament (5/37, 13.5%). Fifty children (50/54, 92.6%) were followed up with an average time of 2.8(1.5, 3.6) years and an average age of (11.1±3.4) years.One child (1/50, 2.0%) developed severe infections twice post-splenectomy.Seven cases (7/54, 13.0%) had residual accessory spleen post-splenectomy and were followed up with an average time of (3.7±0.9) years.No accessory spleen was found in 4 children during splenectomy.Accessory spleen was found in 1 child during operation but was not removed.Indirect bilirubin continued rising within 3 years post-splenectomy with an expanding volume of residual accessory spleen and another accessory spleen was found by ultrasound at 3 years post-splenectomy.However, bilirubin and accessory spleens stabilized after 3 to 5 years of follow-up.The remainders with residual accessory spleen were followed up without severe infection or recurrence of hemolytic anemia.Conclusions:Accessory spleen is prevalent in HS and it predominates in splenic hilum.A preoperative diagnosis of accessory spleen is rather challenging, requiring careful explorations and a complete removal.Jaundice after splenectomy in HS should be on a high alert for accessory spleen residue.Children with residual accessory spleen may have an immediate postoperative onset of hemolysis without anemia.Regular follow-ups are required.
