Current Pediatric Reviews最新文献

The intestine plays a central role in the immune system, continuously interacting with antigens, dietary components, and the microbiota. Intestinal immune processes are increasingly recognized for their influence on the development of both local and systemic diseases, with long-term effects on health and disease progression. This review provides an overview of intestinal development, encompassing its maturation from conception, temporal changes, regenerative capacity, interactions with the microbiota, and involvement in disease. Early life, particularly critical periods such as pregnancy and lactation, may represent a "window of opportunity," establishing lasting conditions that either increase disease risk or confer protection in adulthood. Understanding the regulatory factors, regional and temporal variations, and existing knowledge gaps is essential for guiding clinical practice, as well as for the prevention and treatment of diseases.

Pierson syndrome (PS) is a rare autosomal recessive disorder, primarily characterized by (1) congenital nephrotic syndrome, (2) ocular abnormalities, and (3) neurodevelopmental deficits. It is caused by mutations in the LAMB2 gene, which encodes the laminin β2 chain-a protein subunit that is part of a specific group of proteins known as laminins. These proteins are present in the glomerular basement membrane, neuromuscular junctions, and ocular structures. Although PS exhibits a wide spectrum of phenotypic presentations, the prognosis remains poor, with most patients not surviving beyond early childhood. Despite its rarity, PS is clinically significant due to its potential to cause end-stage kidney disease early in life. This review consolidates the latest insights into the etiopathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of PS.

Introduction: Total parenteral nutrition (TPN) is essential for growth in very-low-birthweight (VLBW) infants. The worldwide variation in TPN dosing strategies warrants investigation. This study compared clinical outcomes of aggressive, rapid-increase, and standard TPN dosing strategies in VLBW infants.

Methods: A systematic review and network meta-analysis were conducted following the PRISMA NMA guideline. Searches were performed in PubMed, Scopus, Web of Science, CINAHL, CENTRAL, and ProQuest. Dosing strategies were classified as aggressive (higher starting dose), rapid-increase (standard start with rapid escalation), and standard (NICE-based). Outcomes were analyzed using a Frequentist model in RStudio v4.4.1.

Results: Nine randomized controlled trials were included. Compared with aggressive and standard strategies, the rapid-increase strategy was associated with a shorter time to regain birth weight (MD = -1.43 days; 95% CI -2.82 to -0.05; P-score = 0.80). The rapid-increase strategy was also associated with a shorter length of hospitalization (MD = -0.38 days; 95% CI -6.56 to 5.80; P-score = 0.54). Regarding safety outcomes, the rapid-increase strategy had the lowest proportions of mortality (Prop = 0.043), retinopathy (Prop = 0.124), and sepsis (Prop = 0.141), but a higher proportion of patent ductus arteriosus (PDA) (Prop = 0.508).

Discussion: The rapid-increase approach demonstrated the most favorable balance between efficacy and safety outcomes among the included trials, although the small number of studies is a limitation.

Conclusion: Rapid-increase TPN, using the recommended starting dose but achieving maintenance more quickly, may offer clinical advantages for VLBW infants. Further long-term studies are needed to confirm developmental and metabolic impacts.

Introduction: ACE inhibitors are commonly prescribed in children. Anecdotally, captopril- induced cough is not thought to occur in children as frequently as in adults.

Methods: We performed a retrospective cohort study in 100 paediatric cardiology patients taking regular ACE inhibitors (ACE-I). Telephone interviews and questionnaires were used to ask patients and their families about their experience of ACE-I-related cough symptoms.

Results: Of the 100 patients, 15% reported symptoms of captopril-related cough. Only 1% required a change in medication due to their cough.

Discussion: Captopril-related cough appears to be less significant in children than in adult populations. The reason for this is unclear, but it may be related to differences in ACE expression between adult and paediatric lungs.

Conclusion: The results suggest that cough associated with ACE-I use may be more prevalent in children than previously thought; however, it is generally well tolerated and rarely necessitates a change in management.

Introduction: Hirschsprung's (HIRSH-sproongz) disease is a multifactorial disorder characterized by the failure of enteric nervous system (ENS) development and is associated with loss-of-function variants, primarily in the RET gene. Rearranged during transfection (RET) expression is tightly regulated within a complex gene regulatory network (GRN) involving many transcription factors, such as GATA2. Aberrant DNA methylation in GATA2 may indicate a reduced enhancer activity, resulting in the silencing of RET and contributing to the failure of ENS development in HIRSH. However, the epigenetic mechanisms underlying these processes have yet to be established.

Method: We analyzed the GATA2 mRNA expression and DNA methylation levels in colonic tissues from the HIRSH patients and controls using quantitative polymerase chain reaction (qPCR) and methylation-specific quantitative PCR (MSP-qPCR). HIRSH tissues, including aganglionic and ganglionic segments, were obtained during pull-through surgery. In addition, control tissues were collected from patients with anorectal malformation (ARM) undergoing definitive surgery, and no clinical evidence of ENS issues was found.

Results: Our study contained 27 unrelated HIRSH patients (20 males and seven females) and 20 controls (12 males and eight females). GATA2 expression was significantly increased in HIRSH than in control colon (ganglionic: 11.78 ± 0.97 vs. 12.59 ± 0.46; p=0.049; aganglionic: 11.94 ± 0.63 vs. 12.59 ± 0.46; p=0.030). In addition, the percentage of methylation was higher in the HIRSH patients than in the control (60.71 ± 2.94% [ganglionic] vs. 79.55 ± 1.79% [aganglionic] vs. 40.43 ± 3.67% [control]).

Discussion: Overexpression of GATA2 in our subjects may indicate a compensatory mechanism for the loss of other TFs within RET-EDNRB or other HIRSH-associated GRN, as an in vitro study has shown that loss of GATA1 in mouse embryonic stem cells results in a marked increase of GATA2 expression, implying potential interchangeability or compensatory mechanism of GATA TFs in RET GRN. Several mechanisms on how specific DNA hypermethylation pathways can lead to upregulated gene expression have been proposed, including facilitating transcription in gene bodies by suppressing cryptic promoters and protecting the borders of promoters or enhancers against unwanted expansion or contraction.

Conclusions: We demonstrated aberrant expression and methylation of GATA2 in the HIRSH patients. The findings of this study also indicate that GATA2 hypermethylation is associated with increased GATA2 expression, contradicting the established inverse association between DNA methylation and transcriptional activity. This may indicate a distinctive interplay between the epigenetics of GATA2 and RET GRN in the context of HIRSH.

Atypical Pneumonia (AP) is any type of pneumonia not caused by one of the common microorganisms, such as Streptococcus pneumoniae. The most common etiologic microorganisms are intracellular bacteria and viruses, including Chlamydia pneumoniae and Mycoplasma pneumoniae. These microorganisms have been difficult to culture. The pandemic of COVID-19 has changed the management of these APs as a result of the widespread usage of multiplexed PCR tests. We have audited 7 anonymized cases of AP to illustrate the utility of these multiplexed PCR-based tests, which can aid the diagnosis and prompt treatment of several AP cases. In conclusion, AP can be readily diagnosed with a multiplexed PCR test, so that efficacious treatment can be initiated without delay. Chlamydia and Bordetella diseases are readily diagnosed even with NPS specimens. Macrolides and doxycycline are readily available oral medications for treating AP in children. Doxycycline is efficacious for macrolide-resistant mycoplasma disease and does not have the side effects of tetracycline in the young pediatric population.

Introduction: To study internet health information-seeking behavior and its determinants among caregivers in a tertiary Pediatric Outpatient Department (OPD) in Eastern India.

Methods: A cross-sectional study was conducted between September and December 2022 at the Pediatric Outpatient Department of the All India Institute of Medical Sciences (AIIMS), Deoghar, India. A 13-item validated questionnaire was administered through face-to-face interviews, capturing demographic information and internet health information-seeking behavior. Statistical analyses, including multivariable logistic regression, identified significant determinants.

Results: Outpatient visits were primarily for acute diseases (39.3%), followed by chronic disease monitoring (19.5%) and acute follow-ups (19.3%). Approximately 34.4% of caregivers sought health information online. Internet health information-seeking behavior was significantly associated with higher educational attainment and visit reasons. Caregivers with higher secondary education or graduate degrees were 7.5 and 7.6 times more likely, respectively, to seek health information online. Those attending for acute or acute follow-up visits had 2.2- and 3.5-times higher odds, respectively. The multivariable model explained 32.4% variability and had a predictive accuracy of 74.1%.

Discussion: The relatively low prevalence of online health information-seeking highlights regional gaps in digital health literacy. Education level and visit type were key predictors, underscoring the need for targeted guidance. Findings are limited by self-reporting and single-center design but offer direction for integrating digital support into pediatric care.

Conclusion: One-third of caregivers utilized the internet for children's health information, with higher education and acute visit reasons as key determinants.

The birth of a baby is a significant milestone for both parents, marking a major life transition into parenthood. In high-risk situations, such as preterm delivery, this event disrupts functional adaptation and greatly impacts parents' ability to recognize and effectively respond to their newborn's physiological and developmental needs. Positive interactions and sensitive responses to an infant's needs enable parents to foster development, supporting regulatory processes and establishing healthy social interaction patterns. The Newborn Behavioral Observation (NBO) Early Intervention is a prevention-focused follow-up program initiated shortly after birth during a developmental window when the infant's brain is highly receptive to environmental influences, maximizing its impact on infant development. Recent applications of the NBO intervention in at-risk contexts have shown promising effects on both infant development and the parent-infant relationship. This educational mini-review aims to summarize the main findings and future prospects of using the NBO to support the transition to parenthood from birth through the first months of life, particularly in high-risk environments such as intensive care units.

Pain management in the first 1000 days of life is crucial for neurodevelopmental outcomes and the quality of life in extremely preterm newborns, who often undergo mechanical ventilation. The use of drugs like midazolam in neonatal care offers advantages such as sedation and muscle relaxation, but can also lead to potential adverse effects. Morphine, a common opioid analgesic, is used in neonatal care for its dual analgesic and sedative properties. Uncertainties regarding short-term pulmonary outcomes and concerns about neurological effects in preterm infants remain. Fentanyl, a synthetic opioid, is a first-line drug for rapid analgesia in various clinical scenarios. Adrenergic agonists and antioxidant substances, dexmedetomidine and melatonin, have shown the potential to be the first analgesic compounds suitable for both clinical trials and clinical use. Dexmedetomidine, an adrenergic agonist, stands out for its opioid-sparing effects and neuroprotective properties. Its efficacy in reducing cumulative morphine administration and supporting mechanical ventilation in certain conditions has been demonstrated. Melatonin has emerged as a neurohormone with potential analgesic properties, and various studies indicate its benefits in reducing pain-related oxidative stress and inflammation. In this educational review, we analyze and discuss pain assessment and management in patients subjected to mechanical ventilation, with a focus on pharmacological management during neonatal intubation, navigating the complexities of balancing effective analgesia and sedation while considering potential risks and outcomes in extremely preterm newborns. The positive effects of new drugs such as melatonin and dexmedetomidine on mechanically ventilated newborns mainly consist of improvements in pain management and amelioration of pulmonary function.