I read with interest the recent review by Lu et al. (Gene therapy for pediatric genetic kidney diseases. Pediatr Discov. 2023; 1(1):e16) and congratulate the authors for their comprehensive review.1 The authors stated that PKHD1 is currently the only known disease-causing gene for autosomal recessive polycystic kidney disease (ARPKD). However, while PKHD1 is the primary gene implicated in ARPKD, it has been shown that DZIP1L is the second gene involved in the disease pathogenesis and is associated with a moderate form of ARPKD.2-4 The role of DZIP1L in ARPKD is also widely discussed in the review referenced by the authors to support their claim.5 Moreover, while it is a rarer cause of the disease, the DZIP1L gene has several advantages over PKHD1 in the development of gene therapy for ARPKD. In 2017, Lu et al. sequenced 743 patients presenting with suspected ARPKD or sporadic PKD. This investigation unveiled DZIP1L mutations as the causative factor underlying an ARPKD-like phenotype in 7 patients from 4 consanguineous families.2 The study also demonstrated that the DZIP1L protein localized to centrioles and the basal bodies of primary cilia, and ciliary trafficking of polycystin-1 and polycystin-2 was impaired in DZIP1L-mutant cells.2, 3 Concordantly, Hertz et al. recently reported 4 ARPKD patients with DZIP1L mutations from 3 consanguineous families.4 Given the extensive length of PKHD1, with its product named fibrocystin consisting of 4074 amino acids, it is not possible to package the cDNA of the gene into a single, or even dual, adeno-associated virus (AAV) vector, which is the most commonly used vector for in vivo gene delivery for inherited diseases.6 Moreover, PKHD1-associated ARPKD is often accompanied by congenital hepatic fibrosis, necessitating that any gene therapy developed for the disease would also target the biliary epithelium.3 Therefore, exceeding the packaging capacity of AAV vectors and the challenges of efficiently targeting both the liver and kidneys may hinder the progress of genomic medicines in treating PKHD1-associated ARPKD. Although being a rarer cause of the disease, the smaller size of the DZIP1L gene and its protein consisting of 767 amino acids offer a remarkable advantage for gene therapy applications by overcoming the AAV packaging limitations faced by PKHD1. Furthermore, liver involvement in the form of hepatosplenomegaly was reported for only one of the 11 patients with DZIP1L mutations, and the patients with truncating mutations showed no apparent hepatic complications.2, 4 While it is required to characterize more patients with diverse DZIP1L mutation profiles to establish phenotypic characteristics of DZIP1L-associated ARPKD, the available data suggest that liver involvement in DZIP1L-associated ARPKD might be less frequent and milder than PKHD1-associated ARPKD. Therefore, it might be sufficient for patients with DZIP1L mutations to target the kidneys using local delivery routes, such as retrograde ureteral
{"title":"Assessing the potential of <i>DZIP1L</i> gene in autosomal recessive polycystic kidney disease gene therapy","authors":"Fahreddin Palaz","doi":"10.1002/pdi3.44","DOIUrl":"https://doi.org/10.1002/pdi3.44","url":null,"abstract":"I read with interest the recent review by Lu et al. (Gene therapy for pediatric genetic kidney diseases. Pediatr Discov. 2023; 1(1):e16) and congratulate the authors for their comprehensive review.1 The authors stated that PKHD1 is currently the only known disease-causing gene for autosomal recessive polycystic kidney disease (ARPKD). However, while PKHD1 is the primary gene implicated in ARPKD, it has been shown that DZIP1L is the second gene involved in the disease pathogenesis and is associated with a moderate form of ARPKD.2-4 The role of DZIP1L in ARPKD is also widely discussed in the review referenced by the authors to support their claim.5 Moreover, while it is a rarer cause of the disease, the DZIP1L gene has several advantages over PKHD1 in the development of gene therapy for ARPKD. In 2017, Lu et al. sequenced 743 patients presenting with suspected ARPKD or sporadic PKD. This investigation unveiled DZIP1L mutations as the causative factor underlying an ARPKD-like phenotype in 7 patients from 4 consanguineous families.2 The study also demonstrated that the DZIP1L protein localized to centrioles and the basal bodies of primary cilia, and ciliary trafficking of polycystin-1 and polycystin-2 was impaired in DZIP1L-mutant cells.2, 3 Concordantly, Hertz et al. recently reported 4 ARPKD patients with DZIP1L mutations from 3 consanguineous families.4 Given the extensive length of PKHD1, with its product named fibrocystin consisting of 4074 amino acids, it is not possible to package the cDNA of the gene into a single, or even dual, adeno-associated virus (AAV) vector, which is the most commonly used vector for in vivo gene delivery for inherited diseases.6 Moreover, PKHD1-associated ARPKD is often accompanied by congenital hepatic fibrosis, necessitating that any gene therapy developed for the disease would also target the biliary epithelium.3 Therefore, exceeding the packaging capacity of AAV vectors and the challenges of efficiently targeting both the liver and kidneys may hinder the progress of genomic medicines in treating PKHD1-associated ARPKD. Although being a rarer cause of the disease, the smaller size of the DZIP1L gene and its protein consisting of 767 amino acids offer a remarkable advantage for gene therapy applications by overcoming the AAV packaging limitations faced by PKHD1. Furthermore, liver involvement in the form of hepatosplenomegaly was reported for only one of the 11 patients with DZIP1L mutations, and the patients with truncating mutations showed no apparent hepatic complications.2, 4 While it is required to characterize more patients with diverse DZIP1L mutation profiles to establish phenotypic characteristics of DZIP1L-associated ARPKD, the available data suggest that liver involvement in DZIP1L-associated ARPKD might be less frequent and milder than PKHD1-associated ARPKD. Therefore, it might be sufficient for patients with DZIP1L mutations to target the kidneys using local delivery routes, such as retrograde ureteral","PeriodicalId":498028,"journal":{"name":"Pediatric Discovery","volume":"114 2","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135372589","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Wei Liu, Xiaoke Dai, Hongxia Guo, Chengyan You, Min Du, Qiu Li
Abstract Although acute kidney injury (AKI) is a frequent postoperative complication after liver transplantation, renal function following living‐related liver transplantation (LRLT) in infants remains understudied. The aim of this study was to identify the prevalence of AKI and the impact of AKI on outcomes in infant recipients with biliary atresia. We retrospectively reviewed infants who underwent LRLT surgery between January 2018 and January 2021. The primary outcome was the risk of postoperative AKI occurrence in the first 7 postoperative days. A multivariate regression analysis model was used to investigate risk factors for AKI, and the incidence and impacts of AKI on outcomes were analyzed. A total of 98 patients were included in the analysis. AKI occurred in 59 patients (60.2%) within 7 days after surgery. Logistic regression analysis revealed that preoperative transfusion ( p < 0.05) and lower serum creatinine (SCr) ( p < 0.05) were independent risk factors for AKI. The incidence of serious complications was significantly higher in the AKI group than in the non‐AKI group ( p < 0.05). The postoperative mechanical ventilation time ( p < 0.05) and hospital stay ( p = 0.019) were significantly longer in the AKI group. There was no evidence of chronic kidney disease (CKD) in any surviving infants within 1 year after surgery. In conclusion, AKI is common in infant LRLT (60.2%), and preoperative transfusion and lower SCr levels were independently associated with AKI. AKI may be associated with the incidence of serious complications, prolonged use of ventilators, and hospital stays. No CKD occurred within 1 year.
{"title":"Acute kidney injury after living‐related liver transplantation in infants with biliary atresia: A retrospective study","authors":"Wei Liu, Xiaoke Dai, Hongxia Guo, Chengyan You, Min Du, Qiu Li","doi":"10.1002/pdi3.37","DOIUrl":"https://doi.org/10.1002/pdi3.37","url":null,"abstract":"Abstract Although acute kidney injury (AKI) is a frequent postoperative complication after liver transplantation, renal function following living‐related liver transplantation (LRLT) in infants remains understudied. The aim of this study was to identify the prevalence of AKI and the impact of AKI on outcomes in infant recipients with biliary atresia. We retrospectively reviewed infants who underwent LRLT surgery between January 2018 and January 2021. The primary outcome was the risk of postoperative AKI occurrence in the first 7 postoperative days. A multivariate regression analysis model was used to investigate risk factors for AKI, and the incidence and impacts of AKI on outcomes were analyzed. A total of 98 patients were included in the analysis. AKI occurred in 59 patients (60.2%) within 7 days after surgery. Logistic regression analysis revealed that preoperative transfusion ( p < 0.05) and lower serum creatinine (SCr) ( p < 0.05) were independent risk factors for AKI. The incidence of serious complications was significantly higher in the AKI group than in the non‐AKI group ( p < 0.05). The postoperative mechanical ventilation time ( p < 0.05) and hospital stay ( p = 0.019) were significantly longer in the AKI group. There was no evidence of chronic kidney disease (CKD) in any surviving infants within 1 year after surgery. In conclusion, AKI is common in infant LRLT (60.2%), and preoperative transfusion and lower SCr levels were independently associated with AKI. AKI may be associated with the incidence of serious complications, prolonged use of ventilators, and hospital stays. No CKD occurred within 1 year.","PeriodicalId":498028,"journal":{"name":"Pediatric Discovery","volume":"18 3","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136023086","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Abstract Pneumoperitoneum is one of the common acute abdominal emergencies that leads to surgical exploration in children, but not all pneumoperitoneum require emergency surgery. This study evaluated the diagnostic methods, predisposing risk factors, and therapeutic options in children with benign pneumoperitoneum for accurate diagnosis and reducing the risk of adverse surgical consequences. A total of 63 cases of pneumoperitoneum diagnosed by the radiographs were involved in this study and were divided into the surgical pneumoperitoneum and benign pneumoperitoneum groups, respectively, from 2009 to 2021. The general information, abdominal signs, laboratory examination, therapies and outcomes, total hospitalization time, and comorbidities were analyzed. Logistic regression analysis assessed the risk factors and protective factors of benign pneumoperitoneum. Promising biomarkers underwent receiver operating characteristic curve analysis. Children with surgical pneumoperitoneum were younger than those with nonsurgical pneumoperitoneum in the study. The C‐reactive protein (CRP) level in the surgical pneumoperitoneum group was higher than that in the other group, albumin (ALB) was lower than that in the benign pneumoperitoneum group, and the ratio of ALB to CRP can be used as an effective indicator to predict whether the pneumoperitoneum is benign or whether surgery is needed. Pulmonary injury, pneumonia, and blunt abdominal injury are the major causes in patients with benign pneumoperitoneum. The establishment and management of clinical criteria for the diagnosis of benign pneumoperitoneum in children will be important for the treatment and recovery of children diagnosed with pneumoperitoneum.
{"title":"Diagnosis, risk factors, and treatment of pediatric benign pneumoperitoneum: A single‐center retrospective study","authors":"Yueyue Liu, Quan Kang, Guobin Liu","doi":"10.1002/pdi3.36","DOIUrl":"https://doi.org/10.1002/pdi3.36","url":null,"abstract":"Abstract Pneumoperitoneum is one of the common acute abdominal emergencies that leads to surgical exploration in children, but not all pneumoperitoneum require emergency surgery. This study evaluated the diagnostic methods, predisposing risk factors, and therapeutic options in children with benign pneumoperitoneum for accurate diagnosis and reducing the risk of adverse surgical consequences. A total of 63 cases of pneumoperitoneum diagnosed by the radiographs were involved in this study and were divided into the surgical pneumoperitoneum and benign pneumoperitoneum groups, respectively, from 2009 to 2021. The general information, abdominal signs, laboratory examination, therapies and outcomes, total hospitalization time, and comorbidities were analyzed. Logistic regression analysis assessed the risk factors and protective factors of benign pneumoperitoneum. Promising biomarkers underwent receiver operating characteristic curve analysis. Children with surgical pneumoperitoneum were younger than those with nonsurgical pneumoperitoneum in the study. The C‐reactive protein (CRP) level in the surgical pneumoperitoneum group was higher than that in the other group, albumin (ALB) was lower than that in the benign pneumoperitoneum group, and the ratio of ALB to CRP can be used as an effective indicator to predict whether the pneumoperitoneum is benign or whether surgery is needed. Pulmonary injury, pneumonia, and blunt abdominal injury are the major causes in patients with benign pneumoperitoneum. The establishment and management of clinical criteria for the diagnosis of benign pneumoperitoneum in children will be important for the treatment and recovery of children diagnosed with pneumoperitoneum.","PeriodicalId":498028,"journal":{"name":"Pediatric Discovery","volume":"61 6","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136071060","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Abstract The aim of this retrospective study was to investigate the impact of early postoperative enteral nutrition on the short‐term prognosis of neonatal gastric perforation. The 63 neonates were divided into two groups based on enteral nutrition timing: the early enteral nutrition (EEN) group (≤15 days) and the late enteral nutrition (LEN) group (>15 days). The EEN group was additionally matched with the LEN1 group based on closely aligned gestational age (difference ≤6 days), birth weight (difference <250 g), and age of onset (<1 day). Data from the EEN, LEN, and LEN1 groups were compared and analyzed. No significant differences were observed among the groups in baseline characteristics such as gestational age, birth weight, age at hospital admission, cesarean section rate, and so on ( p > 0.05). Furthermore, preoperative comorbidities, clinical symptoms, and examination results were not significantly different between the three groups ( p > 0.05). However, the time required to achieve total enteral nutrition, the length of hospital stay, and fistula retention time were significantly less in the EEN group compared to the LEN groups ( p < 0.05). The EEN group also exhibited a shorter gastrointestinal decompression time than the LEN1 group, but other major postoperative outcome measures were not significantly different. In conclusion, our study suggests that early postoperative enteral nutrition (≤15 days) could reduce the time to total enteral nutrition, length of hospital stay, and fistula retention time, without increasing adverse prognosis rates.
{"title":"Effect of early postoperative enteral nutrition on the short‐term prognosis in neonatal gastric perforation","authors":"Ting Zhu, Yu Liu, Huan Wei, Shuo Tang, Xiaowen Li, Mengying Cui, Yuan Shi, Zheng‐Li Wang","doi":"10.1002/pdi3.34","DOIUrl":"https://doi.org/10.1002/pdi3.34","url":null,"abstract":"Abstract The aim of this retrospective study was to investigate the impact of early postoperative enteral nutrition on the short‐term prognosis of neonatal gastric perforation. The 63 neonates were divided into two groups based on enteral nutrition timing: the early enteral nutrition (EEN) group (≤15 days) and the late enteral nutrition (LEN) group (>15 days). The EEN group was additionally matched with the LEN1 group based on closely aligned gestational age (difference ≤6 days), birth weight (difference <250 g), and age of onset (<1 day). Data from the EEN, LEN, and LEN1 groups were compared and analyzed. No significant differences were observed among the groups in baseline characteristics such as gestational age, birth weight, age at hospital admission, cesarean section rate, and so on ( p > 0.05). Furthermore, preoperative comorbidities, clinical symptoms, and examination results were not significantly different between the three groups ( p > 0.05). However, the time required to achieve total enteral nutrition, the length of hospital stay, and fistula retention time were significantly less in the EEN group compared to the LEN groups ( p < 0.05). The EEN group also exhibited a shorter gastrointestinal decompression time than the LEN1 group, but other major postoperative outcome measures were not significantly different. In conclusion, our study suggests that early postoperative enteral nutrition (≤15 days) could reduce the time to total enteral nutrition, length of hospital stay, and fistula retention time, without increasing adverse prognosis rates.","PeriodicalId":498028,"journal":{"name":"Pediatric Discovery","volume":"6 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135889452","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Abstract Respiratory tract infections (RTIs) are common and frequently occurring diseases in children, posing a significant health threat to children worldwide. Viruses are the most important pathogens of childhood RTIs. Since the outbreak of Coronavirus Disease 2019 (COVID‐19), a series of nonpharmaceutical interventions (NPIs) have been widely implemented around the globe, and important changes have taken place in the spectrum of respiratory diseases and viruses in children. However, with relaxation of NPIs, there has been a “virus resurgence” in some areas, with multiple viral infectious diseases appearing simultaneously. This review comprehensively summarizes the changes observed in the spectrum of respiratory diseases and viruses in children in the context of the COVID‐19 pandemic, explores possible mechanisms, and presents reflections on the key points of diagnosis and treatment of RTIs in children in the post‐COVID‐19 era in light of recent advances in COVID‐19 in children.
{"title":"Current status and reflections on the diagnosis and treatment of respiratory tract infections in children in the COVID‐19 pandemic and post‐COVID‐19 era","authors":"Yuyi Tang, Luo Ren, Enmei Liu","doi":"10.1002/pdi3.33","DOIUrl":"https://doi.org/10.1002/pdi3.33","url":null,"abstract":"Abstract Respiratory tract infections (RTIs) are common and frequently occurring diseases in children, posing a significant health threat to children worldwide. Viruses are the most important pathogens of childhood RTIs. Since the outbreak of Coronavirus Disease 2019 (COVID‐19), a series of nonpharmaceutical interventions (NPIs) have been widely implemented around the globe, and important changes have taken place in the spectrum of respiratory diseases and viruses in children. However, with relaxation of NPIs, there has been a “virus resurgence” in some areas, with multiple viral infectious diseases appearing simultaneously. This review comprehensively summarizes the changes observed in the spectrum of respiratory diseases and viruses in children in the context of the COVID‐19 pandemic, explores possible mechanisms, and presents reflections on the key points of diagnosis and treatment of RTIs in children in the post‐COVID‐19 era in light of recent advances in COVID‐19 in children.","PeriodicalId":498028,"journal":{"name":"Pediatric Discovery","volume":"41 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135804236","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Xi Yang, Yingfu Chen, Rongxin Dai, Yunfei An, Xin Yan, Xiaodong Zhao, Xuemei Tang
Abstract Macrophage activation syndrome (MAS) is a rare, potentially life‐threatening condition in rheumatic diseases. The primary treatments consist of high‐dose corticosteroids and immunosuppressive drugs, although more recently, cytokine inhibitors like anakinra or tocilizumab (TCZ) have been reported. We present a case of a child with systemic juvenile idiopathic arthritis (sJIA). After receiving a single infusion of TCZ, the child developed progressive hypoxia and was subsequently transferred to the pediatric intensive care unit (PICU) after 4 days. An immediate postintubation chest X‐ray revealed a diffuse exudative lesion. Despite continuous efforts to provide mechanical ventilation and respiratory support, the patient's oxygen saturation continued to decline. Moreover, the patient developed hemodynamic compromise, necessitating the administration of norepinephrine. Eventually, vasopressin and dopamine were added to maintain stable hemodynamics. After an intensive but ineffective treatment, extracorporeal membrane oxygenation (ECMO) was initiated in the PICU after 16 h. The patient successfully recovered and was weaned off ECMO support after 60 h. Following discharge from the PICU, given the severe refractory clinical features, we made an attempt to readminister TCZ treatment. However, within half an hour of TCZ infusion, the patient experienced anaphylaxis characterized by palpitations and chest tightness, leading to the discontinuation of TCZ. TCZ, as a biological agent, is commonly used in the treatment of sJIA. Nonetheless, the occurrence of MAS and anaphylaxis following TCZ administration for sJIA may be more prevalent than previously recognized. Pediatric rheumatologists should exercise caution when initiating TCZ for active sJIA. Furthermore, we want to underscore the importance of life‐saving techniques such as ECMO for sJIA patients in emergency situations.
{"title":"Successful use of extracorporeal membrane oxygenation for life‐threatening macrophage activation syndrome after treatment with tocilizumab in an systemic juvenile idiopathic arthritis patient","authors":"Xi Yang, Yingfu Chen, Rongxin Dai, Yunfei An, Xin Yan, Xiaodong Zhao, Xuemei Tang","doi":"10.1002/pdi3.32","DOIUrl":"https://doi.org/10.1002/pdi3.32","url":null,"abstract":"Abstract Macrophage activation syndrome (MAS) is a rare, potentially life‐threatening condition in rheumatic diseases. The primary treatments consist of high‐dose corticosteroids and immunosuppressive drugs, although more recently, cytokine inhibitors like anakinra or tocilizumab (TCZ) have been reported. We present a case of a child with systemic juvenile idiopathic arthritis (sJIA). After receiving a single infusion of TCZ, the child developed progressive hypoxia and was subsequently transferred to the pediatric intensive care unit (PICU) after 4 days. An immediate postintubation chest X‐ray revealed a diffuse exudative lesion. Despite continuous efforts to provide mechanical ventilation and respiratory support, the patient's oxygen saturation continued to decline. Moreover, the patient developed hemodynamic compromise, necessitating the administration of norepinephrine. Eventually, vasopressin and dopamine were added to maintain stable hemodynamics. After an intensive but ineffective treatment, extracorporeal membrane oxygenation (ECMO) was initiated in the PICU after 16 h. The patient successfully recovered and was weaned off ECMO support after 60 h. Following discharge from the PICU, given the severe refractory clinical features, we made an attempt to readminister TCZ treatment. However, within half an hour of TCZ infusion, the patient experienced anaphylaxis characterized by palpitations and chest tightness, leading to the discontinuation of TCZ. TCZ, as a biological agent, is commonly used in the treatment of sJIA. Nonetheless, the occurrence of MAS and anaphylaxis following TCZ administration for sJIA may be more prevalent than previously recognized. Pediatric rheumatologists should exercise caution when initiating TCZ for active sJIA. Furthermore, we want to underscore the importance of life‐saving techniques such as ECMO for sJIA patients in emergency situations.","PeriodicalId":498028,"journal":{"name":"Pediatric Discovery","volume":"8 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135352009","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Abstract The microorganisms in the human body gradually change and maintain a dynamic balance with the development of physiology and pathology. Oral microbiota is one of the most important microbiota in human body. It is not only closely related to the occurrence and development of oral diseases, but also plays an important role in the overall health. In childhood, the population of oral microorganisms is relatively small, but with the growth of age and tooth development, the species and quantity of oral microorganisms are gradually increasing. Different oral diseases also have their corresponding main microorganisms, and these dominant microorganisms change at different stages of the disease. In this review, we summarized and compared the main pathogenic microorganisms of several common oral diseases in children and adults. In addition, the possible association and difference between adults and children of the main pathogenic microorganisms in different stages of the same or different diseases are also discussed in order to provide research data for the development and diagnosis of common oral diseases in children and adults.
{"title":"Comparison of the main pathogenic microorganisms of various common oral diseases in children and adults","authors":"Siqi Zhou, Tong‐Chuan He, Yuxin Zhang, Hongmei Zhang","doi":"10.1002/pdi3.35","DOIUrl":"https://doi.org/10.1002/pdi3.35","url":null,"abstract":"Abstract The microorganisms in the human body gradually change and maintain a dynamic balance with the development of physiology and pathology. Oral microbiota is one of the most important microbiota in human body. It is not only closely related to the occurrence and development of oral diseases, but also plays an important role in the overall health. In childhood, the population of oral microorganisms is relatively small, but with the growth of age and tooth development, the species and quantity of oral microorganisms are gradually increasing. Different oral diseases also have their corresponding main microorganisms, and these dominant microorganisms change at different stages of the disease. In this review, we summarized and compared the main pathogenic microorganisms of several common oral diseases in children and adults. In addition, the possible association and difference between adults and children of the main pathogenic microorganisms in different stages of the same or different diseases are also discussed in order to provide research data for the development and diagnosis of common oral diseases in children and adults.","PeriodicalId":498028,"journal":{"name":"Pediatric Discovery","volume":"61 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135482586","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Xinsi Chen, Kun Feng, Yu Zhang, Yongming Wang, Qianqian Zhao, Ya Hu, Kaizhen Liu, Hong Wei, Ziyu Hua
Abstract Bacterial meningitis (BM) is potentially life threatening in neonates, but the duration of antibiotic therapy is not well established. We aimed to compare the efficacy and safety among neonates suffering from BM of a relatively shortened duration of antibiotic treatment to the currently recommended course. We did a retrospective cohort study in neonates (gestational age [GA] or corrected GA ≥35 weeks) diagnosed with BM. Neonates in the study group were assigned to withdraw the antibiotics on condition that they were clinically stable after taking sufficient antibiotics with normal serological inflammatory biomarkers, whereas the cerebrospinal fluid (CSF) indicators remain abnormal. Neonates in the control group were treated until both serological and CSF indicators returned to normal as recommended. The incidence of recurrent infection after the discontinuation of antibiotics and adverse drug reactions (ADRs) during hospitalization was measured. A total of 233 neonates were enrolled, of whom 160 were assigned to a shortened antibiotic duration and 73 were treated according to the current guidelines. Twelve patients (7.5%) relapsed in the study group, whereas 4 (5.5%) relapsed in the control group ( χ 2 = 0.320, p = 0.572). The incidences of ADRs were similar in both groups ( p > 0.05). The study indicates that antibiotics might be safely discontinued in neonates (GA ≥35 weeks) diagnosed with BM who are clinically stable or improving after antibiotic treatment and feature normal serological inflammatory markers, no severe complications, and no evidence of systemic infection, even if CSF parameters are not completely normal.
细菌性脑膜炎(BM)对新生儿有潜在的生命威胁,但抗生素治疗的持续时间尚未确定。我们的目的是比较抗生素治疗时间相对较短的BM新生儿与目前推荐疗程的疗效和安全性。我们对诊断为BM的新生儿(胎龄[GA]或校正胎龄≥35周)进行了回顾性队列研究。实验组新生儿在服用足量抗生素后临床稳定,血清学炎症生物标志物正常,但脑脊液(CSF)指标仍异常的情况下停药。对照组患儿按建议治疗至血清学和脑脊液指标恢复正常。观察停药后复发感染发生率及住院期间药物不良反应(adr)。共有233名新生儿被纳入研究,其中160名被分配使用缩短的抗生素疗程,73名根据现行指南接受治疗。研究组12例(7.5%)复发,对照组4例(5.5%)复发(χ 2 = 0.320, p = 0.572)。两组不良反应发生率相似(p >0.05)。该研究表明,即使脑脊液参数不完全正常,诊断为BM的新生儿(GA≥35周)在抗生素治疗后临床稳定或改善,血清学炎症标志物正常,无严重并发症,无全身感染的证据,也可以安全停用抗生素。
{"title":"The evaluation of antimicrobial stewardship for bacterial meningitis in neonates","authors":"Xinsi Chen, Kun Feng, Yu Zhang, Yongming Wang, Qianqian Zhao, Ya Hu, Kaizhen Liu, Hong Wei, Ziyu Hua","doi":"10.1002/pdi3.30","DOIUrl":"https://doi.org/10.1002/pdi3.30","url":null,"abstract":"Abstract Bacterial meningitis (BM) is potentially life threatening in neonates, but the duration of antibiotic therapy is not well established. We aimed to compare the efficacy and safety among neonates suffering from BM of a relatively shortened duration of antibiotic treatment to the currently recommended course. We did a retrospective cohort study in neonates (gestational age [GA] or corrected GA ≥35 weeks) diagnosed with BM. Neonates in the study group were assigned to withdraw the antibiotics on condition that they were clinically stable after taking sufficient antibiotics with normal serological inflammatory biomarkers, whereas the cerebrospinal fluid (CSF) indicators remain abnormal. Neonates in the control group were treated until both serological and CSF indicators returned to normal as recommended. The incidence of recurrent infection after the discontinuation of antibiotics and adverse drug reactions (ADRs) during hospitalization was measured. A total of 233 neonates were enrolled, of whom 160 were assigned to a shortened antibiotic duration and 73 were treated according to the current guidelines. Twelve patients (7.5%) relapsed in the study group, whereas 4 (5.5%) relapsed in the control group ( χ 2 = 0.320, p = 0.572). The incidences of ADRs were similar in both groups ( p > 0.05). The study indicates that antibiotics might be safely discontinued in neonates (GA ≥35 weeks) diagnosed with BM who are clinically stable or improving after antibiotic treatment and feature normal serological inflammatory markers, no severe complications, and no evidence of systemic infection, even if CSF parameters are not completely normal.","PeriodicalId":498028,"journal":{"name":"Pediatric Discovery","volume":"80 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135483053","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Abstract Retinoic acid (RA) serves as a metabolic intermediate of vitamin A. It plays a crucial physiological role in regulating cell proliferation, differentiation, apoptosis, embryonic development, and immunomodulation. Once vitamin A enters the body in the form of retinol, it undergoes conversion into RA through the intestinal epithelium and liver. Subsequently, it interacts with retinoic acid receptors and retinoid X receptors within the cell nucleus, thereby regulating gene expression. Throughout liver development, RA exerts precise temporal control, stimulating liver growth, inducing RALDH2 expression in liver somatic epithelial cells, and influencing hepatocyte differentiation. Recent studies have consistently demonstrated the indispensable connection between RA deficiency and the development of liver diseases, including nonalcoholic fatty liver disease, chronic hepatitis, liver fibrosis, and liver tumors. Studying the mechanisms underlying the relationship between RA and disease can enhance our understanding and improve disease treatment. This paper provides a comprehensive review of the role of RA signaling in liver development and liver diseases.
{"title":"Retinoic acids and nuclear receptor signaling in liver development: Pathogenic roles in liver diseases","authors":"Wen Jia, Yang Bi","doi":"10.1002/pdi3.29","DOIUrl":"https://doi.org/10.1002/pdi3.29","url":null,"abstract":"Abstract Retinoic acid (RA) serves as a metabolic intermediate of vitamin A. It plays a crucial physiological role in regulating cell proliferation, differentiation, apoptosis, embryonic development, and immunomodulation. Once vitamin A enters the body in the form of retinol, it undergoes conversion into RA through the intestinal epithelium and liver. Subsequently, it interacts with retinoic acid receptors and retinoid X receptors within the cell nucleus, thereby regulating gene expression. Throughout liver development, RA exerts precise temporal control, stimulating liver growth, inducing RALDH2 expression in liver somatic epithelial cells, and influencing hepatocyte differentiation. Recent studies have consistently demonstrated the indispensable connection between RA deficiency and the development of liver diseases, including nonalcoholic fatty liver disease, chronic hepatitis, liver fibrosis, and liver tumors. Studying the mechanisms underlying the relationship between RA and disease can enhance our understanding and improve disease treatment. This paper provides a comprehensive review of the role of RA signaling in liver development and liver diseases.","PeriodicalId":498028,"journal":{"name":"Pediatric Discovery","volume":"43 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135536197","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Xin‐Kai Zhang, Xiang Li, Ming‐Yan Shi, Man Zhang, Pei‐Kang Wang, Hai‐Lun Yao, Xin Tan, Xiang Yu, Yu‐Fei Shao, Xing Liu
Abstract Growth hormone (GH) therapy has been used in patients with growth retardation and short stature due to various reasons. The safety of GH treatment is still debatable, one of which is the occurrence and development of scoliosis. In order to explore the effects of GH on the occurrence or progression of scoliosis, this review summarizes previous studies, summarizes the influence of GH treatment on the pathogenesis and progression of scoliosis, and analyzes the possible mechanism of GH in the pathogenesis and progression of scoliosis. Hopefully, it can provide a direction for future research.
{"title":"Impact of growth hormone on scoliosis","authors":"Xin‐Kai Zhang, Xiang Li, Ming‐Yan Shi, Man Zhang, Pei‐Kang Wang, Hai‐Lun Yao, Xin Tan, Xiang Yu, Yu‐Fei Shao, Xing Liu","doi":"10.1002/pdi3.26","DOIUrl":"https://doi.org/10.1002/pdi3.26","url":null,"abstract":"Abstract Growth hormone (GH) therapy has been used in patients with growth retardation and short stature due to various reasons. The safety of GH treatment is still debatable, one of which is the occurrence and development of scoliosis. In order to explore the effects of GH on the occurrence or progression of scoliosis, this review summarizes previous studies, summarizes the influence of GH treatment on the pathogenesis and progression of scoliosis, and analyzes the possible mechanism of GH in the pathogenesis and progression of scoliosis. Hopefully, it can provide a direction for future research.","PeriodicalId":498028,"journal":{"name":"Pediatric Discovery","volume":"33 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135718978","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}