Frontiers in Pediatrics最新文献

Infantile Epileptic Spasm Syndrome (IESS) is a group of infantile spasm syndromes of various etiologies that typically present in early infancy, predispose to refractory epilepsy, and leave intellectual disability. Ketogenic diet therapy (KDT) is a non-pharmacologic treatment modality for medically refractory IESS. Recent scientific evidence supported the efficacy, safety, and tolerability of KDT for the treatment of IESS. KDT not only reduces the frequency of seizures in infants with IESS, but also improve their cognition and long-term prognosis. Recently, it has also received increasing attention as a potential treatment for neurological disorders. This reviewed the recent research progress of KDTs for the treatment of IESS, and discussed the different types and the mechanisms of KDTs, the expansion of KDT applications, the influencing factors, and future research issues.

Introduction: Pressure ulcers are a significant concern in pediatric intensive care units, with prevalence rates ranging from 0.8% to 27%. They pose serious physical and psychological challenges, particularly in neonatal and pediatric intensive care units (NICU and PICU). This study explores nursing strategies, attitudes, and barriers toward pressure ulcer prevention in NICU and PICU settings.

Methods: Using a descriptive study design, data were collected from 80 nurses working in NICU and PICU through validated questionnaires, including a demographic profile and a pressure ulcer survey. Descriptive statistics were employed to calculate mean scores and percentages, while inferential statistics assessed associations between variables.

Results: The study revealed specific nursing strategies, along with significant barriers and attitudes toward pressure ulcer prevention in NICU and PICU. The total attitude mean score was 3.57, with the highest positive response (mean = 4.29) for "most pressure sores can be avoided", and the highest negative response (mean = 3.86) for "pressure sore prevention is a low priority for me". Among participants, 72.5% conducted risk assessments on all patients, 60% had written prevention care plans, and 76.3% implemented preventive strategies. Barriers such as staff shortages and time constraints were reported by 76.2% of nurses. Multivariate analysis indicated that nurses with more than 10 years of qualification (OR = 3.67) and permanent staff with over 10 years of employment (OR = 4.31) were significantly more likely to engage in preventive practices. The use of a pressure ulcer grading tool (OR = 2.49, P < 0.05) and participation in formal training (OR = 3.14, P < 0.05) were also positively associated with preventive practices.

Discussion: These findings underscore the importance of structured assessment tools, ongoing education, and the need to foster positive attitudes among nurses to effectively reduce pressure ulcer prevalence and enhance patient outcomes in NICU and PICU settings.

Background: Asparaginase is a critical component of chemotherapy for pediatric acute lymphoblastic leukemia (ALL), but its use is often complicated by asparaginase-associated pancreatitis (AAP). Genetic predispositions, such as variants in the SPINK1 gene, have been linked to an increased risk of pancreatitis. However, the role of genetic factors in relation to asparaginase treatment remains incompletely understood, partly because mutations in pancreatitis-causing genes are rarely found in pediatric ALL.

Case description: A four-year and three-month-old Chinese girl was admitted to our hospital due to fever for half a day, with no history of significant prior medical history. Initial blood tests revealed hematological abnormalities, including leukopenia, anemia, and thrombocytosis. Bone marrow aspiration identified 81.5% blast cells with B-lymphocyte morphology and immunophenotype, leading to a diagnosis of B-cell acute lymphoblastic leukemia (B-ALL). The patient began treatment under the CCCG-ALL-2015 protocol, which included PEG-asparaginase (PEG-asp). On day 10 of induction, she developed AAP, which was primarily characterized by severe epigastric pain and elevated serum amylase. Despite effective symptom management with analgesics and anti-inflammatory therapy, AAP recurred following administration of L-asparaginase (L-asp). Genetic analysis revealed a heterozygous SPINK1 c.194 + 2T>C variant (rs148954387), a well-known pathogenic variant associated with increased susceptibility to pancreatitis. Sanger sequencing confirmed that the SPINK1 variant was inherited from her asymptomatic mother. The patient's AAP was managed conservatively, and an asparaginase-free regimen ultimately achieved complete remission without recurrence of pancreatitis.

Conclusions: The identification of the SPINK1 c.194 + 2T>C variant, which is recognized as pathogenic, provides valuable information for understanding the heightened risk of AAP in our pediatric ALL patient. Our case underscores the potential role of genetic predisposition in the development of AAP and highlights the importance of considering genetic screening prior to asparaginase therapy in pediatric ALL patients to identify those at increased risk.

Background: One of the greatest challenges to conducting multicenter research studies in low and middle-income countries (LMICs) is the heterogeneity in regulatory processes across sites. Previous studies have reported variations in requirements with a lack of standardization in the Institutional Review Board (IRB) processes between centers, imposing barriers for approval, participation, and development of multicenter research.

Objectives: To describe the regulatory process, variability and challenges faced by pediatric researchers in LMICs during the IRB process of an international multicenter observational point prevalence study (Global PARITY).

Design: A 16-question multiple-choice online survey was sent to site principal investigators (PIs) at PARITY study participating centers to explore characteristics of the IRB process, costs, and barriers to research approval. A shorter survey was employed for sites that expressed interest in participating in Global PARITY and started the approval process, but ultimately did not participate in data collection (non-participating sites) to assess IRB characteristics.

Results: Of the 91 sites that sought IRB approval, 46 were successful in obtaining approval and finishing the data collection process. The survey was completed by 46 (100%) participating centers and 21 (47%) non-participating centers. There was a significant difference between participating and non-participating sites in IRB approval of a waiver consent and in the requirement for a legal review of the protocol. The greatest challenge to research identified by non-participating sites was a lack of research time and the lack of institutional support.

Conclusions: Global collaborative research is crucial to increase our understanding of pediatric critical care conditions in hospitals of all resource-levels and IRBs are required to ensure that this research complies with ethical standards. Critical barriers restrict research activities in some resource limiting countries. Increasing the efficiency and accessibility of local IRB review could greatly impact participation of resource limited sites and enrollment of vulnerable populations.

[This corrects the article DOI: 10.3389/fped.2024.1430921.].

Introduction: Providing adequate nutrition to preterm infants to achieve postnatal growth similar to intrauterine growth remains challenging due to the unpredictability of individual determinants.

Material and methods: We used a calculation program for infant incubators to compare the estimated heat balance with the caloric intake and growth rate in Very Low Birth Weight Infants (VLBWI).

Results and discussion: A group of 32 VLBWI was studied over a period of 14-28 days. An interrelationship between thermal equilibrium and growth rate was observed, with standardized incubator settings being unable to avoid periods of negative thermal balance and concomitantly poor growth rate.

Conclusion: Determining personalized incubator settings by means of a calculation program could help improve nutrition and growth in preterm infants.

Human milk oligosaccharides (HMOs) are a diverse group of structures and an abundant bioactive component of breastmilk that contribute to infant health and development. Preclinical studies indicate roles for HMOs in shaping the infant gut microbiota, inhibiting pathogens, modulating the immune system, and influencing cognitive development. In the past decade, several industrially produced HMOs have become available to fortify infant formula. Clinical intervention trials with manufactured HMOs have begun to corroborate some of the physiological effects reported in preclinical studies, especially modulation of the gut microbiota in the direction of breastfed infants. As more HMOs become commercially available and as HMOs have some shared mechanisms of action, there is a need to better understand the unique and differential effects of individual HMOs and the benefits of combining multiple HMOs. This review focuses on the differential effects of different HMO structural classes and individual structures and presents a scientific rationale for why combining multiple structurally diverse HMOs is expected to exert greater biological effects.

Objective: This study aims to investigate the surface electromyography (sEMG) characteristics of upper limb muscles in children with neglected Monteggia fracture after open reduction of radial head dislocation and ulna osteotomy and bone grafting internal fixation, and to understand the recovery of muscle activity in children after operation, provide reference for clinical rehabilitation.

Methods: A retrospective analysis was conducted on sixteen children with neglected Monteggia fracture who underwent ulna osteotomy at our hospital from January 2021 to August 2022. The biceps brachii, triceps brachii, flexor carpi radialis, and extensor carpi ulnaris muscle activities were recorded during grip strength tests, flexion and extension of elbow joint while holding a 1 kg dumbbell, as well as gripping tasks. The root mean square (RMS) values of sEMG signals, co-contraction ratio, and elbow joint function scores were compared between pre- and post-operation periods as well as between the affected side and unaffected side.

Results: The preoperative maximum grip strength, as well as the average RMS values of flexor carpi radialis and average RMS value of extensor carpi ulnaris on the affected side were significantly lower. After surgery, both the maximum RMS value of biceps brachii and maximum and average RMS value of extensor carpi ulnaris on the affected side remained lower. Prior to surgery, when performing elbow flexion and extension tests while holding a 1 kg dumbbell, both mean RMS values of biceps brachii and flexor carpi radialis on the affected side were smaller. However, after surgery, there was an increase in mean RMS values of biceps brachii on the affected side. Furthermore, postoperative elbow function scores were significantly higher than preoperative scores.

Conclusion: Open reduction of radial head dislocation combined with ulna osteotomy and bone grafting can achieve good functional activities in the treatment of neglected Monteggia fractures in children. The EMG activity of the extensor carnosus ulnalis muscle on the affected side related to grip strength was low, and the desired effect was not achieved within the expected time.

Objective: To study the cerebrospinal fluid (CSF) neurofilament light chain (NfL) in pediatric acquired demyelinating syndrome (ADS) and its association with factors of laboratory and imaging results.

Methods: We analyzed clinical data from children with ADS collected from May 2020 to January 2021 at the Department of Neurology of Guangzhou Women and Children's Medical Center. Enzyme-linked immunosorbent assays were used to detect the CSF NfL of patients.

Results: Thirty pediatric ADS patients (17 male, 13 female) were included in the study. The most frequent diagnosis was uncategorized ADS (36.7%, 11/30), followed by acute disseminating encephalomyelitis (ADEM) (23.3%, 7/30), myelin oligodendrocyte glycoprotein antibody-associated disorder (MOGAD) (20.0%, 6/30), NMO (6.7%, 2/30), multiple sclerosis (MS) (6.7%, 2/30), and neuromyelitis optic spectrum disorders (NMOSD) (6.7%, 2/30). The median CSF NfL for the first time was 7,425.28 pg/ml (interquartile range, 1,273.51, >10,000 pg/ml). CSF NfL increase over normal value (<290.00 pg/ml for people younger than 30 years old) was seen in 98.7% of patients. Patients were divided into uncategorized ADS, ADEM, MOGAD, and MS/NMO/NMOSD groups, with no significant difference in CSF NfL between each group. The CSF NfL positively correlated with the immunoglobulin (Ig) G (ρ = 0.473) and IgE (ρ = 0.366). However, the CSF NfL did not correlate with CSF white blood count and CSF protein. Furthermore, there was no significant difference between patients with oligoclonal bands positive and without. The CSF NfL negatively correlated with interferon γ (ρ = -0.501), CD45 ⁺ CD3⁺ T (ρ = -0.466), CD45 ⁺ CD3 ⁺ CD4⁺ T (ρ = -0.466), and CD45 ⁺ CD3 ⁺ CD8⁺ T cells (ρ = -0.521). However, it did not correlate with CD45 ⁺ CD19⁺ B cells. CSF NfL in patients with cerebral white matter lesions in MRI was higher than in patients without. Moreover, the CSF NfL positively correlated with the number of brain MRI locations (ρ = 0.362). Nine patients underwent multiple detections of CSF NfL, and their CSF NfL for the last detection was not significantly different from the first.

Conclusions: The CSF NfL increases significantly in pediatric ADS, and it can be a biomarker of neuro-axonal injury and a good indication of the extent of lesions.

Background: Fever is a common symptom in children, but despite existing guidelines, pediatricians may not fully apply recommendations. Fever of Unknown Origin (FUO) is generally referred to as an unexplained prolonged fever. However, a standardized FUO definition and management is missing.

Objective: To collect updated data on the approach to fever and FUO among Italian pediatricians.

Methods: A cross-sectional anonymous survey was conducted among a large sample of primary care and hospital pediatricians. The panel group formulated and proposed a practical FUO definition, using a modified Delphi approach. A 75% consensus was required to reach an agreement.

Results: Among 620 respondents, paracetamol was the first-choice antipyretic for 97.7% of participants, followed by ibuprofen; 38.4% prescribed antipyretics based on a specific body temperature rather than on child's discomfort, while physical methods were almost completely abandoned. Alternate treatment was recommended by 19.8% (123/620) of participants, 16.9% (105/620) would prescribe antipyretics to prevent adverse events following immunization. Regarding FUO diagnosis, 58.3% (362/620) considered as cut-off a body temperature above 38°C; the duration required was one week according to 36.45% (226/620) of participants, two weeks according to 35.32% (219/620). The FUO definition proposed by the expert panel reached 81% of consent. Large agreement was observed on first-level laboratory and instrumental investigations in the diagnostic evaluation of FUO, whereas more discrepancies arose on second and third-level investigations. Compared to what participants reported for the treatment of non-prolonged fever, a significant decrease in the prescription of paracetamol as first-choice drug in children with FUO was observed (80.5%; P < 0.0001). Interestingly, 39% of participants would empirically recommend antibiotics, 13.7% steroids, and 4.5% Nonsteroidal Anti-Inflammatory Drugs (NSAIDs) for persistent FUO.

Conclusion: Non-recommended behaviors in fever management persist among pediatricians, including alternating use of paracetamol and ibuprofen, and their prophylactic use for vaccinations. Our data confirm the variability in the definition, work-up, and management of FUO. We observed that in children with FUO paracetamol was significantly less commonly preferred than in non-prolonged fever, which is not supported by evidence. Our findings combined with evidence from existing literature underlined the need for future consensus documents.