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Vol 19, No 5
Vol 19, No 5 May 2023 ISSN 1708-8569
Review articles
Original articles
Research letter
Multigenerational birth cohort study in China: importance, necessity and beyond
  Sai-Li Ni, Wei He, Jia-Kai Zhang, Fei Yang, Guan-Nan Bai, Die Li, Wei-Ze Xu, Jia-Bin Li, Qiang Shu, Shan-Kuan Zhu
  [Abstract] [Full Text] [PDF]  
Review articles:
Biliatresone: progress in biliary atresia study
  Jia-Jie Zhu, Yi-Fan Yang, Rui Dong, Shan Zheng
Background: Biliary atresia (BA) is one of the main causes of neonatal end-stage liver disease. Without timely diagnosis and treatment, most children with BA will develop irreversible liver fibrosis within the first two months. While current theorized causes of BA include viral infection, immune disorders, and genetic defects, the comprehensive etiology is still largely unknown. Recently, biliatresone attracted much interest for its ability to induce BA in both zebrafish and mice, so we summarized the latest progress of biliatresone research in BA and tried to answer the question of whether it could provide further clues to the etiology of human BA.
Data sources: We conducted a PubMed search for any published articles related to the topic using search terms including ※biliary atresia§, ※biliatresone§, ※GSH§, and ※HSP90§. Relevant data were extracted from the original text or supplementary materials of the corresponding articles.
Results: Biliatresone had shown its unique toxicity in multiple species such as zebrafish and mice, and pathogenic factors involved included glutathione (GSH), heat shock protein 90 (HSP90) and the related pathways. In combination with epidemiological evidence and recent studies on the intestinal flora in biliary atresia, a new pathogenic hypothesis that the occurrence of biliary atresia is partly due to biliatresone or its structure-like compounds depositing in human body via vegetables or/and the altered intestinal flora structure can be tentatively established.
Conclusions: Based on the existing evidence, we emphasized that GSH and HSP90 are involved in the development of BA, and the maternal diet, especially higher vegetable intake of Asian women of childbearing age, accompanied by the altered intestinal flora structure, may contribute to the occurrence of biliary atresia and the higher incidence in the Asia group. However, the evidence from large sample epidemiological research is necessary.
  [Abstract] [Full Text] [PDF]  
Sphingosine phosphate lyase insufficiency syndrome: a systematic review
  Zahra Pournasiri, Abbas Madani, Fatemeh Nazarpack, John A. Sayer, Zahra Chavoshzadeh, Fatemeh Nili, Paulina Tran, Julie D. Saba, Mahnaz Jamee
Background: Sphingosine-1-phosphate lyase insufficiency syndrome (SPLIS) or nephrotic syndrome type-14 is caused by biallelic mutations in SGPL1. Here, we conducted a systematic review to delineate the characteristics of SPLIS patients.
Methods: A literature search was performed in PubMed, Web of Science, and Scopus databases, and eligible studies were included. For all patients, demographic, clinical, laboratory, and molecular data were collected and analyzed.
Results: Fifty-five SPLIS patients (54.9% male, 45.1% female) were identified in 19 articles. Parental consanguinity and positive family history were reported in 70.9% and 52.7% of patients, respectively. Most patients (54.9%) primarily manifested within the first year of life, nearly half of whom survived, while all patients with a prenatal diagnosis of SPLIS (27.5%) died at a median [interquartile (IQR)] age of 2 (1.4每5.3) months (P = 0.003). The most prevalent clinical feature was endocrinopathies, including primary adrenal insufficiency (PAI) (71.2%) and hypothyroidism (32.7%). Kidney disorders (42, 80.8%) were mainly in the form of steroid-resistant nephrotic syndrome (SRNS) and progressed to end-stage kidney disease (ESKD) in 19 (36.5%) patients at a median (IQR) age of 6 (1.4每42.6) months. Among 30 different mutations in SGPL1, the most common was c.665G > A (p.Arg222Gln) in 11 (20%) patients. Twenty-six (49.1%) patients with available outcome were deceased at a median (IQR) age of 5 (1.5每30.5) months, mostly following ESKD (23%) or sepsis/septic shock (23%).
Conclusion: In patients with PAI and/or SRNS, SGPL1 should be added to diagnostic genetic panels, which can provide an earlier diagnosis of SPLIS and prevention of ESKD and other life-threatening complications.
  [Abstract] [Full Text] [PDF]  
Original articles:
Early recombinant human growth hormone treatment improves mental development and alleviates deterioration of motor function in infants and young children with Prader每Willi syndrome
  Ruo-Qian Cheng, Yan-Qin Ying, Zheng-Qing Qiu, Jun-Fen Fu, Chun-Xiu Gong, Yan-Ling Yang, Wei Shi, Hui Li, Ming-Sheng Ma, Chang-Yan Wang, Min Liu, Jia-Jia Chen, Chang Su, Xiao-Ping Luo, Fei-Hong Luo, Wei Lu
Background: Recombinant human growth hormone (rhGH) therapy has shown to improve height and body composition in children with Prader每Willi syndrome (PWS), the evidence of early rhGH treatment on motor and mental development is still accumulating. This study explored the time effect on psychomotor development, anthropometric indexes, and safety for infants and young children with PWS.
Methods: A phase 3, single-arm, multicenter, self-controlled study was conducted in six sites. Patients received rhGH at 0.5 mg/m2/day for first four weeks, and 1 mg/m2/day thereafter for up to 52 weeks. Motor development was measured using Peabody Developmental Motor Scales-second edition, mental development using Griffiths Development Scales-Chinese (GDS-C). Height standard deviation score (SDS), body weight SDS, and body mass index (BMI) SDS were also assessed.
Results: Thirty-five patients were enrolled totally. Significant improvements were observed in height, body weight, and BMI SDS at week 52; GDS-C score showed significant improvement in general quotient (GQ) and sub-quotients. In a linear regression analysis, total motor quotient (TMQ), gross motor quotient (GMQ), and fi ne motor quotient were negatively correlated with age; however, treatment may attenuate deterioration of TMQ and GMQ. Changes in GQ and locomotor sub-quotient in < 9-month group were signifi cantly higher than ≡ 9-month group. Mild to moderate severity adverse drug reactions were reported in six patients.
Conclusion: Fifty-two-week treatment with rhGH improved growth, BMI, mental development, and lessened the deterioration of motor function in infants and young children with PWS. Improved mental development was more pronounced when instituted in patients < 9 months old.
  [Abstract] [Full Text] [PDF]  
Nusinersen for spinal muscular atrophy types II and III: a retrospective single-center study in South Korea
  Hui Jin Shin, Ji-Hoon Na, Hyunjoo Lee, Young-Mock Lee
Background: This study investigated the efficacy and safety of nusinersen, an antisense oligonucleotide, in patients with spinal muscular atrophy (SMA) types II (OMIM: 253,550) or III (OMIM: 253,400), including those with severe scoliosis or requiring respiratory support via mechanical ventilation.
Methods: Data from 40 patients with genetically confirmed SMA who were treated with nusinersen at our institute from March 2019 to April 2022 were retrospectively analyzed. Of these, 30 patients with an age of onset < 3 years and not on permanent ventilation were selected. Clinical and genetic characteristics were investigated, and motor function was evaluated based on the Hammersmith Functional Motor Scale-Expanded (HFMSE) score.
Results: The mean age of symptom onset was 1.2 years. Most patients were diagnosed with SMA type II (27/30, 90%). Nusinersen was administered via computed tomography-guided or direct intrathecal injection in 87% (26/30) and 13% (4/30) of the patients, respectively. At the 6-, 14-, 22-, and 26-month follow-ups, 72%, 71%, 88%, and 86% of patients showed motorimprovement, respectively, with mean changes in HFMSE scores of 2.10, 2.88, 4.21, and 5.29, respectively. Multivariable analysis showed that the use of noninvasive ventilation was associated with poorer outcomes of motor function.
Conclusions: Patients with SMA type II or III who received nusinersen treatment showed significant improvement in motor function. A longer treatment duration led to a higher number of patients with improved motor function. No significant side effects of nusinersen were observed. Patients with SMA, even those with severe scoliosis or on respiratory support, can be safely treated using nusinersen.
  [Abstract] [Full Text] [PDF]  
Relationship between early nutrition and deep gray matter and lateral ventricular volumes of preterm infants at term-equivalent age
  Felicia Toppe, Tobias Rasche, Christel Weiss, Alexandra Schock, Ursula Felderhoff-M邦ser, Hanna M邦ller
Background: The survival of preterm infants has improved over the last decade, but impaired brain development leading to poor neurological outcomes is still a major comorbidity associated with prematurity. The aim of this study was to evaluate the effect of nutrition on neurodevelopment in preterm infants and identify markers for improved outcomes.
Methods: Totally 67 premature infants with a gestational age of 24每34 weeks and a birth weight of 450每2085 g were included. Clinical parameters and documented diet were collected from medical records. The nutritional analysis comprised the protein, fat, carbohydrate, and energy intake during different time spans. Brain development was assessed by determining deep gray matter (DGM; basal ganglia and thalamus) and lateral ventricular (LV) volumes as measured on cerebral magnetic resonance imaging scans obtained at term-equivalent age (TEA), and potential associations between nutrition and brain volumetrics were detected by regression analysis.
Results: We observed a negative correlation between mean daily protein intake in the third postnatal week and MRI-measured DGM volume at TEA (P = 0.007). In contrast, head circumference at a corrected age of 35 weeks gestation (P < 0.001) and mean daily fat intake in the fourth postnatal week (P = 0.004) were positively correlated with DGM volume. Moreover, mean daily carbohydrate intake in the first postnatal week (P = 0.010) and intraventricular hemorrhage (P = 0.003) were revealed as independent predictors of LV volume.
Conclusion: The study emphasizes the importance of nutrition for brain development following preterm birth.
  [Abstract] [Full Text] [PDF]  
Impact of the COVID-19 kindergarten closure on overweight and obesity among 3- to 7-year-old children
  Xiang Long, Xing-Ying Li, Hong Jiang, Lian-Di Shen, Li-Feng Zhang, Zheng Pu, Xia Gao, Mu Li
Background: Knowledge on the impact of the temporary kindergarten closure policy under COVID-19 in 2020 on childhood overweight and obesity is inadequate. We aimed to examine differences in rates of overweight and obesity from 2018 to 2021 among kindergarten children aged 3每7 years.
Methods: Overweight was defined as body mass index (BMI) > 1 standard deviation (SD) for age and sex, and obesity was defined as BMI > 2 SD for age and sex. Generalized linear mixed modeling was used for analysis.
Results: A total of 44,884 children and 71,216 growth data points from all 57 public kindergartens in Jiading District, Shanghai, China were analyzed. The rates of obesity from 2018 to 2021 were 6.9%, 6.6%, 9.5%, and 7.3% in boys and 2.8%, 2.8%, 4.5%, and 3.1% in girls, respectively. The rates of overweight from 2018 to 2021 were 14.3%, 14.3%, 18.2%, and 15.3% in boys and 10.6%, 10.9%, 13.9%, and 11.6% in girls. The rates of obesity and overweight among kindergarten children in 2020 were significantly higher than those in 2018, 2019, and 2021. Compared to 2020, the odds ratios of the obesity rate in 2018, 2019, and 2021 were 0.67 [95% confidence interval (CI) = 0.58每0.77, P < 0.001], 0.72 (95% CI = 0.64每0.80, P < 0.001) and 0.81 (95% CI = 0.72每0.92, P = 0.001), respectively. The odds ratios of the overweight rate in 2018, 2019, and 2021 were 0.75 (95% CI = 0.69每0.82, P < 0.001), 0.78 (95% CI = 0.72每0.84, P < 0.001), and 0.89 (95% CI = 0.81每0.97, P = 0.008), respectively, compared to 2020.
Conclusions: The rates of overweight and obesity significantly increased among kindergarten children in 2020 after the 5-month kindergarten closure. It was critical to provide guidance to caregivers on fostering a healthy lifestyle for children at home under public health emergencies.
  [Abstract] [Full Text] [PDF]  
Altered gut microbiota composition in children and their caregivers infected with the SARS-CoV-2 Omicron variant
  Yi-Zhong Wang, Jian-Guo Zhou, Yan-Ming Lu, Hui Hu, Fang-Fei Xiao, Ting Ge, Xing Wang, Lu Zheng, Lian-Hu Yu, Jun Le, Hui Yu, Guang-Jun Yu, Qiang Xia, Ting Zhang, Wen-Hao Zhou
Background: Gut microbiota alterations have been implicated in the pathogenesis of coronavirus disease 2019 (COVID-19). This study aimed to explore gut microbiota changes in a prospective cohort of COVID-19 children and their asymptomatic caregivers infected with the severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) Omicron variant.
Methods: A total of 186 participants, including 59 COVID-19 children, 50 asymptomatic adult caregivers, 52 healthy children (HC), and 25 healthy adults (HA), were recruited between 15 April and 31 May 2022. The gut microbiota composition was determined by 16S rRNA gene sequencing in fecal samples collected from the participants. Gut microbiota functional profiling was performed by using Phylogenetic Investigation of Communities by Reconstruction of Unobserved States (PICRUSt) software.
Results: The gut microbiota analysis of beta diversity revealed that the fecal microbial community of COVID-19 children remained far distantly related to HC. The relative abundances of the phyla Actinobacteria and Firmicutes were decreased, whereas Bacteroidetes, Proteobacteria, and Verrucomicrobiota were increased in COVID-19 children. Feces from COVID-19 children exhibited notably lower abundances of the genera Blautia, Bifidobacterium, Fusicatenibacter, Streptococcus, and Romboutsia and higher abundances of the genera Prevotella, Lachnoclostridium, Escherichia-Shigella, and Bacteroides than those from HC. The enterotype distributions of COVID-19 children were characterized by a high prevalence of enterotype Bacteroides. Similar changes in gut microbiota compositions were observed in asymptomatic caregivers. Furthermore, the microbial metabolic activities of KEGG (Kyoto Encyclopedia of Genes and Genomes) and COG (cluster of orthologous groups of proteins) pathways were perturbed in feces from subjects infected with the SARS-CoV-2 Omicron variant.
Conclusion: Our data reveal altered gut microbiota compositions in both COVID-19 children and their asymptomatic caregivers infected with the SARS-CoV-2 Omicron variant, which further implicates the critical role of gut microbiota in COVID-19 pathogenesis.
  [Abstract] [Full Text] [PDF]  
Retroperitoneal kidney transplantation with liver and native kidney mobilization: a safe technique for pediatric recipients
  Juliano Riella, Raphealla Ferreira, Marina M. Tabbara, Phillipe Abreu, Lucas Ernani, Marissa Defreitas, Jayanthi Chandar, Jeffrey J. Gaynor, Javier Gonz芍lez, Gaetano Ciancio
Background: Pediatric kidney transplant (KT) using larger, deceased or living donor adult kidneys can be challenging in the pediatric population due to limited space in the retroperitoneum. Liver and native kidney (L/NK) mobilization techniques can be used in smaller and younger transplant recipients to aid in retroperitoneal placement of the renal allograft. Here, we compare the clinical outcomes of pediatric retroperitoneal KT with and without L/NK mobilization.
Methods: We retrospectively analyzed pediatric renal transplant recipients treated between January 2015 and May 2021. Donor and recipient demographics, intraoperative data, and recipient outcomes were included. Recipients were divided into two groups according to the surgical technique utilized: with L/NK mobilization (Group 1) and without L/NK mobilization (Group 2). Baseline variables were described using frequency distributions for categorical variables and means and standard errors for continuous variables. Tests of association with the likelihood of using L/NK mobilization were performed using standard 2 tests, t tests, and the log-rank test.
Results: Forty-six pediatric recipients were evaluated and categorized into Group 1 (n = 26) and Group 2 (n = 20). Recipients in Group 1 were younger (6.7 ㊣ 0.8 years vs. 15. 3 ㊣ 0.7, P < 0.001), shorter (109.5 ㊣ 3.7 vs. 154.2 ㊣ 3.8 cm, P < 0.001) and weighed less (21.4 ㊣ 2.0 vs . 48.6 ㊣ 3.4 kg, P < 0.001) than those in Group 2. Other baseline characteristics did not differ between Groups 1 and 2. One urologic complication was encountered in Group 2; no vascular or surgical complications were observed in either group. Additionally, no stents or drains were used in any of the patients. There were no cases of delayed graft function or graft primary nonfunction. The median follow-up of the study was 24.6 months post-transplant. Two patients developed death-censored graft failure (both in Group 2, P = 0.22), and there was one death with a functioning graft (in Group 2, P = 0.21).
Conclusions: Retroperitoneal liver/kidney mobilization is a feasible and safe technique that facilitates implantation of adult kidney allografts into pediatric transplant recipients with no increased risk of developing post-operative complications, graft loss, or mortality.
  [Abstract] [Full Text] [PDF]  
Research letter:
New epidemiological trends of respiratory syncytial virus bronchiolitis during COVID-19 pandemic
  Emanuele Castagno, Irene Raffaldi, Francesco Del Monte, Silvia Garazzino, Claudia Bondone
  [Abstract] [Full Text] [PDF]  
World Journal of Pediatric Surgery
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