-
Hepatology Communications Oct 2023Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous rare congenital cholestatic liver disease. Disease progression might necessitate liver... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous rare congenital cholestatic liver disease. Disease progression might necessitate liver transplantation (LT). The aim of this study was to describe the outcome of PFIC1-4 patients after LT.
METHODS
Electronic databases were searched to identify studies on PFIC and LT. Patients were categorized according to PFIC type, genotype, graft type, age at LT, time of follow-up, and complications and treatment during follow-up.
RESULTS
Seventy-nine studies with 507 patients met inclusion criteria; most patients were classified as PFIC1-3. The median age at LT was 50 months. The overall 5-year patient survival was 98.5%. PFIC1 patients with diarrhea after LT were at significant risk of developing graft steatosis ( p < 0.0001). Meta-analysis showed an efficacy of 100% [95% CI: 73.9%-100%] for surgical biliary diversion to ameliorate steatosis and 94.9% [95% CI: 53.7%-100%] to improve diarrhea (n = 8). PFIC2 patients with bile salt export pump (BSEP)2 or BSEP3-genotype were at significant risk of developing antibody-induced BSEP deficiency (AIBD) ( p < 0.0001), which was reported in 16.2% of patients at a median of 36.5 months after LT. Meta-analysis showed an efficacy of 81.1% [95% CI: 47.5%-100%] for rituximab-based treatment regimens to improve AIBD (n = 18). HCC was detected in 3.6% of PFIC2 and 13.8% of PFIC4 patients at LT.
CONCLUSIONS
Fifty percent of PFIC1 patients develop diarrhea and steatosis after LT. Biliary diversion can protect the graft from injury. PFIC2 patients with BSEP2 and BSEP3 genotypes are at significant risk of developing AIBD, and rituximab-based treatment regimens effectively improve AIBD. PFIC3 patients have no PFIC-specific complications following LT.
Topics: Humans; Child, Preschool; Liver Transplantation; Rituximab; Carcinoma, Hepatocellular; Liver Neoplasms; Fatty Liver; Diarrhea; Cholestasis; Cholestasis, Intrahepatic; Steroid Metabolism, Inborn Errors
PubMed: 37756114
DOI: 10.1097/HC9.0000000000000286 -
Jornal de Pediatria 2023Meckel diverticulum (MD) is a common malformation of the digestive tract, often accompanied by serious complications. It is important to find safe and effective... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Meckel diverticulum (MD) is a common malformation of the digestive tract, often accompanied by serious complications. It is important to find safe and effective diagnostic methods for screening MD. The aim of this study was to evaluate the effectiveness of a technetium-99m (Tc-99m) scan for pediatric bleeding MD.
METHODS
The authors conducted a systematic review of studies published in PubMed, Embase, and Web of Science before 1 January 2023. Studies based on PICOS were included in this systematic review. The flow chart was made by PRISMA software. The quality of included studies was assessed by RevMan5 software (QUADAS-2: Quality Assessment of Diagnostic Accuracy Studies-2). The sensitivity, specificity, and other measurements of accuracy were pooled using Stata/SE 12.0 software.
RESULTS
Sixteen studies with 1115 children were included in this systematic review. A randomized-effects model was used for the meta-analysis because of significant heterogeneity. The combined sensitivity and specificity were 0.80 [Confidence Interval (95% CI, 0.73-0.86) and 0.95 (95% CI, 0.86-0.98)], respectively. The area under the curve (AUC) was 0.88 (95% CI, 0.85-0.90). Publication bias (Begg's test p = 0.053) was observed.
CONCLUSION
Tc-99m scan has high specificity, but moderate sensitivity, which is always influenced by some factors. Hence, the Tc-99m scan has some limitations in the diagnosis of pediatric bleeding MD.
Topics: Child; Humans; Technetium; Meckel Diverticulum; Radionuclide Imaging; Gastrointestinal Hemorrhage; Sensitivity and Specificity
PubMed: 37277097
DOI: 10.1016/j.jped.2023.03.009 -
Cureus Sep 2023The neuropeptide calcitonin gene-related peptide (CGRP) is an essential pathophysiological treatment for migraines. A unique class of medications called CGRP monoclonal... (Review)
Review
The neuropeptide calcitonin gene-related peptide (CGRP) is an essential pathophysiological treatment for migraines. A unique class of medications called CGRP monoclonal antibodies target CGRP and its receptor and have demonstrated promising benefits in the treatment and prevention of migraines. This study sought to identify and assess the quality of existing systematic reviews about the effectiveness of CGRP antibodies for preventing migraines, as well as systematically review and synthesize the evidence on these topics. This included the four Food and Drug Administration (FDA)-approved medications erenumab, galcanezumab, fremanezumab, and eptinezumab. The effectiveness and safety of these monoclonal antibodies in preventing migraines should also be examined in light of patient characteristics, and any gaps in the body of knowledge should be noted in order to suggest new lines of investigation. Data gathering included a thorough search of internet databases (PubMed, Cochrane Library, Web of Science, and Scopus) for relevant research released between 2018 and 2023. The findings imply that CGRP monoclonal antibodies are efficient and secure for preventing migraines and may be considered a first-line alternative for treating migraines and drug misuse. The results further imply that combination treatment with CGRP antibodies and onabotulinumtoxinA may enhance the prevention of migraine in adults. With suggestions for more studies to find and address these variables, the significance of genetic and epigenetic factors in the progression of pediatric patients' acute postoperative pain to chronic postsurgical pain is underlined. All four anti-CGRP monoclonal antibodies, erenumab, fremanezumab, galcanezumab, and eptinezumab, were shown to be safe and effective for the prevention of migraine when the research additionally looked at their individual effectiveness and safety. Additionally, the study discovered considerable variances in effectiveness amongst various groups. However, further investigation is required to establish the best time and dosage and the effect of patient characteristics on the effectiveness and safety of these medications.
PubMed: 37868560
DOI: 10.7759/cureus.45560 -
AIMS Neuroscience 2023Gut microbiomes play a role in developing and regulating autoimmune diseases such as multiple sclerosis (MS). We designed this systematic review to summarize the... (Review)
Review
BACKGROUND
Gut microbiomes play a role in developing and regulating autoimmune diseases such as multiple sclerosis (MS). We designed this systematic review to summarize the evidence of the effect of gut microbiota in developing pediatric-onset MS.
METHODS
PubMed, Scopus, EMBASE, Web of Science, Google Scholar, references of the references and conference abstracts were comprehensively searched by two independent researchers. The search was done on January 1, 2023. Data regarding the total number of patients, the name of the first author, publication year, country of origin, mean age, duration of the disease, body mass index (BMI), type of MS, Expanded Disability Status Scale (EDSS), age at disease onset and stool composition were extracted.
RESULTS
A literature search revealed 4237 published studies. After removing duplicates, we had 2045 records for evaluation. Twenty-three full texts were evaluated, and four case-control studies remained for systematic review. Three studies were conducted in the United States and one in the Netherlands. The number of participants in included studies ranged between 24 and 68. The mean age of patients at the time of study varied between 11.9 and 17.9 years, and the mean age at the onset of the disease ranged between 11.5 and 14.3 years. Most included patients were female. The results show that median richness (the number of unique taxa identified, which was provided by two studies) was higher in controls, and also Margalef index, which was reported by one study was higher in control group than the case group. The results of two studies also demonstrated that median evenness indexes (taxon distribution, Shannon, Simpson) were higher in control groups, as well as PD index (Faith's phylogenic diversity metric).
CONCLUSION
The result of this systematic review (including four studies) showed disruption of the microbiota-immune balance in pediatric-onset MS cases.
PubMed: 38188004
DOI: 10.3934/Neuroscience.2023031 -
BMC Medical Informatics and Decision... Nov 2023Patient safety is a central healthcare policy worldwide. Adverse drug events (ADE) are among the main threats to patient safety. Children are at a higher risk of ADE in... (Review)
Review
Identifying the data elements and functionalities of clinical decision support systems to administer medication for neonates and pediatrics: a systematic literature review.
BACKGROUND
Patient safety is a central healthcare policy worldwide. Adverse drug events (ADE) are among the main threats to patient safety. Children are at a higher risk of ADE in each stage of medication management process. ADE rate is high in the administration stage, as the final stage of preventing medication errors in pediatrics and neonates. The most effective way to reduce ADE rate is using medication administration clinical decision support systems (MACDSSs). The present study reviewed the literature on MACDSS for neonates and pediatrics. It identified and classified the data elements that mapped onto the Fast Healthcare Interoperability Resources (FHIR) standard and the functionalities of these systems to guide future research.
METHODS
PubMed/ MEDLINE, Embase, CINAHL, and ProQuest databases were searched from 1995 to June 31, 2021. Studies that addressed developing or applying medication administration software for neonates and pediatrics were included. Two authors reviewed the titles, abstracts, and full texts. The quality of eligible studies was assessed based on the level of evidence. The extracted data elements were mapped onto the FHIR standard.
RESULTS
In the initial search, 4,856 papers were identified. After removing duplicates, 3,761 titles, and abstracts were screened. Finally, 56 full-text papers remained for evaluation. The full-text review of papers led to the retention of 10 papers which met the eligibility criteria. In addition, two papers from the reference lists were included. A total number of 12 papers were included for analysis. Six papers were categorized as high-level evidence. Only three papers evaluated their systems in a real environment. A variety of data elements and functionalities could be observed. Overall, 84 unique data elements were extracted from the included papers. The analysis of reported functionalities showed that 18 functionalities were implemented in these systems.
CONCLUSION
Identifying the data elements and functionalities as a roadmap by developers can significantly improve MACDSS performance. Though many CDSSs have been developed for different medication processes in neonates and pediatrics, few have actually evaluated MACDSSs in reality. Therefore, further research is needed on the application and evaluation of MACDSSs in the real environment.
PROTOCOL REGISTRATION
(dx.doi.org/10.17504/protocols.io.bwbwpape).
Topics: Infant, Newborn; Humans; Child; Decision Support Systems, Clinical; Pharmaceutical Preparations; Medication Errors; Drug-Related Side Effects and Adverse Reactions; Patient Safety
PubMed: 37974195
DOI: 10.1186/s12911-023-02355-5 -
Acta Bio-medica : Atenei Parmensis Dec 2023The relationship between precocious or early puberty and its treatment has received significant research attention, yielding diverse outcomes. This short review aims to...
BACKGROUND
The relationship between precocious or early puberty and its treatment has received significant research attention, yielding diverse outcomes. This short review aims to comprehensively analyze and summarize research articles to elucidate the potential link between precocious or early pubertal onset (CPP) and crucial health factors.
METHODS
We conducted a systematic review of studies published from -January 2000 to March 2023, sourced from databases of Medline, PubMed, Google Scholar and Web of Science. We assessed the relationship between CPP and final adult height (FHt), bone health, reproductive function, body mass index, metabolic and cardiovascular abnormalities, and increased cancer risk.
RESULTS
Upon reviewing and analyzing selected studies, the following key findings emerged: (a) treating CPP in girls before age 6-7 and in boys before age 9 improves FHt; (b) bone mineral density (BMD) decreases during GnRHa treatment but normalizes afterward, with no lasting effects on peak bone mass during puberty; (c) GnRH treatment does not negatively affect menstrual cycles; however, untreated CPP increases the risk of premature or early-onset menopause; (d) the incidence of PCOS/hyperandrogenemia may be slightly elevated in women with a history of CPP, but overall reproductive function remains largely unaffected; (e) earlier thelarche and menarche may enhance susceptibility to breast carcinogenesis; (f) CPP contributes to an increased risk of obesity and type 2 diabetes in both genders; (g) early menarche may slightly increase the risk of coronary heart disease and ischemic strokes and (h) early pubertal timing increases the risk of depression and anxiety disorders.
CONCLUSION
Monitoring and early diagnosis of these conditions are of paramount importance for successful management.
Topics: Female; Humans; Male; Child; Diabetes Mellitus, Type 2; Gonadotropin-Releasing Hormone; Puberty, Precocious; Obesity; Puberty
PubMed: 38054666
DOI: 10.23750/abm.v94i6.15316 -
European Review For Medical and... Aug 2023Childhood obesity, which is currently at epidemic levels, is the most prevalent chronic condition affecting young people's health worldwide. Along with the rise in... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Childhood obesity, which is currently at epidemic levels, is the most prevalent chronic condition affecting young people's health worldwide. Along with the rise in juvenile obesity, illnesses like diabetes mellitus, hypertension, and fatty liver disease have become more prevalent in kids. Hence, through this systematic review and meta-analysis, we aimed to determine the lifestyle changes that would have the most impact on the incidence of childhood obesity.
MATERIALS AND METHODS
The databases of PubMed-MEDLINE, Web of Science, Cochrane, and Scopus were searched using keywords, such as "BMI", "childhood obesity", "lifestyle changes" and "nutritional intervention" and 482 documents were found overall after a thorough search of the online journals; 169 of them were first chosen. Only 58 original papers were left after 111 articles that were duplicates or exact copies of one another were eliminated.
RESULTS
12 studies were ultimately picked because they met the necessary inclusion and exclusion requirements. Reducing overall caloric intake and dietary factors specific to the child's parents were two of the most frequent impact factors on obesity levels, closely followed by physical activity levels and a sedentary lifestyle. The dietary intervention had the most positive results in modifying obesity-related dietary risk factors for obese children and adolescents in the majority of the studies.
CONCLUSIONS
An overall balanced diet, parental awareness pertaining to BMI and physical activity in children were the three major factors influencing a child's obesity levels. However, more studies are needed in this regard so as to ascertain a complete, holistic treatment plan that can further validate the implementation of our findings.
Topics: Child; Adolescent; Humans; Pediatric Obesity; Incidence; Life Style; Databases, Factual; Energy Intake
PubMed: 37667948
DOI: 10.26355/eurrev_202308_33424 -
Cureus Nov 2023Opioid-related fatalities are a leading cause of accidental death in the United States. Appendicitis is a common cause of abdominal pain in children and adolescents. The... (Review)
Review
Opioid-related fatalities are a leading cause of accidental death in the United States. Appendicitis is a common cause of abdominal pain in children and adolescents. The management of pain throughout the laparoscopic appendectomy (LA) in the pediatric population is a critical concern. This study aimed to evaluate trends in analgesic use and patient satisfaction following LA, with a focus on reducing the reliance on opioids for pain management. From 2003 to 2023, 18258 articles were filtered for all types of analgesic use with LA. The publications were screened using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, and 19 studies were included for analysis and review. The study included peer-reviewed experimental and observational studies involving individuals under 18 years. Pain management strategies varied across studies, involving a combination of analgesics, nerve blocks, and wound infiltrations. Analgesics such as acetaminophen, non-steroidal anti-inflammatory drugs (NSAIDs), and opioids were administered before and after surgery. Some studies implemented patient-controlled analgesia (PCA) pumps. Other studies explored non-pharmacological interventions like magnetic acupuncture. The results showed a reduction in the need for postoperative analgesics in patients treated with LA, particularly when using non-opioid medications and novel analgesic techniques. Pediatric patients who received gabapentin reported lower opioid use, shorter hospital stays, and high satisfaction rates. However, the reliance on opioids remained significant in some cases, particularly among patients with peritonitis who required more morphine. Pain management in pediatric patients is multifaceted, involving preoperative and postoperative analgesics, nerve blocks, and PCA pumps. Efforts to improve pain management following pediatric LA while reducing opioid reliance are essential in the context of the ongoing opioid epidemic. The findings from this study highlight the potential benefits of non-opioid analgesics, nerve blocks, and alternative methods for managing postoperative pain in <18 appendectomy patients. Further research and standardization of pain management protocols are needed to ensure optimal patient outcomes and minimize the risk of opioid-related complications.
PubMed: 38156159
DOI: 10.7759/cureus.49581 -
Cancer Treatment Reviews Nov 2023To analyze changes in recurrent/refractory osteosarcoma phase II trials over time to inform future trials in this population with poor prognosis. (Review)
Review
BACKGROUND/OBJECTIVE
To analyze changes in recurrent/refractory osteosarcoma phase II trials over time to inform future trials in this population with poor prognosis.
METHODS
A systematic review of trials registered on trial registries between 01/01/2017-14/02/2022. Comparison of 98 trials identified between 2003 and 2016. Publication search/analysis for both periods, last update on 01/12/2022.
RESULTS
Between 2017 and 2022, 71 phase-II trials met our selection criteria (19 osteosarcoma-specific trials, 14 solid tumor trials with and 38 trials without an osteosarcoma-specific stratum). The trial number increased over time: 13.9 versus 7 trials/year (p = 0.06). Monotherapy remained the predominant treatment (62% vs. 62%, p = 1). Targeted therapies were increasingly evaluated (66% vs. 41%, P = 0.001). Heterogeneity persisted in the trial characteristics. The inclusion criteria were measurable disease (75%), evaluable disease (14%), and surgical remission (11%). 82% of the trials included pediatric or adolescent patients. Biomarker-driven trials accounted for 25% of the total trials. The survival endpoint use (rather than response) slightly increased (40% versus 31%), but the study H/H hypotheses remained heterogeneous. Single-arm designs predominated over multiarm trials (n = 7). Available efficacy data on 1361 osteosarcoma patients in 58 trials remained disappointing, even though 21% of these trials were considered positive, predominantly those evaluating multi-targeted kinase inhibitors.
CONCLUSION
Despite observed changes in trial design and an increased number of trials investigating new therapies, high heterogeneity remained with respect to patient selection, study design, primary endpoints, and statistical hypotheses in recently registered phase II trials for osteosarcoma. Continued optimization of trial design informed by a deeper biological understanding should strengthen the development of new therapies.
PubMed: 37738712
DOI: 10.1016/j.ctrv.2023.102625 -
Children (Basel, Switzerland) Aug 2023This systematic review and meta-analysis assessed the quality of the peer-reviewed literature and evaluated the usefulness of eye-tracking technology in evaluating... (Review)
Review
This systematic review and meta-analysis assessed the quality of the peer-reviewed literature and evaluated the usefulness of eye-tracking technology in evaluating observers' perceptions of pediatric patients with orofacial clefts. PubMed, Science Direct, Wiley, and Web of Science were searched. Articles were screened in accordance with the Preferred Reporting Items for Systematic Review and Meta-analysis guidelines, and their methodological quality was assessed. Of the 10,254 identified studies, 12 were included. Eleven studies were cross-sectional, and one was a prospective cohort study. The main areas of interest analyzed were the eyes, nose, and mouth. Nine studies used assessment scales to analyze the link between perceived attractiveness and visualization patterns and measures. For the fixation duration outcome, six studies were eligible for inclusion in the meta-analysis. All studies reported on fixation duration in milliseconds and reported on a standard deviation. The meta-analysis demonstrated a significant difference in the measurements between the control groups and the patients with orofacial clefts. This might indicate the usefulness of eye-tracking technology as a metric for assessing the success of cleft repairs based on the perceptions of different populations. Future studies should be comprehensively reported on for comparability and reproducibility purposes.
PubMed: 37628424
DOI: 10.3390/children10081425