Current Pediatric Reviews最新文献

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.

Introduction: Understanding the delayed effects of COVID-19, often called "long COVID-19," is essential for creating effective treatment protocols and support systems for affected children. This study was conducted to determine the delayed effects of COVID-19 in hospitalized children.

Methods: This cross-sectional study is descriptive-analytical and was conducted through a census of 137 hospitalized children diagnosed with COVID-19 in Jiroft, Iran, from 2021 to 2023. Data were collected using a checklist from the patients' medical records and through telephone interviews with the children's parents. The data were analyzed using descriptive and inferential statistics at a significance level of p < 0.05.

Results: The average age of the children was 7.7 years, with a majority being boys, and a small percentage having underlying medical conditions. Most children were hospitalized for three days. Fatigue was the most common delayed complication, affecting 21.1% of cases, while seizures were the least common at 1.4%. Other notable complications included cough, rhinorrhea, and nausea, with significant relationships observed between age and issues like loss of smell and decreased appetite. Underlying diseases were linked to higher rates of cough and shortness of breath.

Discussion: Fatigue was the most common delayed complication of COVID-19 in hospitalized children. Younger children and those with underlying health conditions were particularly vulnerable to these delayed effects.

Conclusion: This study underscores the need for personalized follow-up care for pediatric patients recovering from COVID-19. Comprehensive monitoring and support programs are crucial for addressing the specific complications observed in this population, thereby ensuring improved long-term outcomes.

Introduction: Vaccination is crucial because it protects individuals and communities from infectious diseases, reducing the spread and potential for outbreaks. As mothers are often the primary caregivers of children, they must be well-informed about childhood vaccinations through credible sources. The study investigated the disparities in knowledge, attitudes, and practices regarding immunization among mothers of children aged 0-5 years residing in Ras Al Khaimah. In the qualitative part of the study, the main barriers and challenges to immunization in the UAE were identified, as perceived by different stakeholders.

Methodology: This study employed a mixed-method approach to examine childhood vaccination practices and attitudes among Emirati and expatriate mothers in Ras Al Khaimah, UAE, between January and April 2024. Two hundred and seven mothers were interviewed using a structured questionnaire, and 14 stakeholders were interviewed using an in-depth interview guide.

Results: Key findings revealed that 95.1% of mothers vaccinated their children, but 18.3% admitted to delays. Expatriate mothers demonstrated higher knowledge of vaccine benefits and fewer concerns about safety compared to Emirati mothers. The themes generated from in-depth interviews- namely, natural immunity, peer influence, and healthcare system challenges-were significant factors influencing vaccination decisions. Cultural norms and systemic issues, such as delays in vaccine administration, also affected vaccination uptake.

Discussion: Despite high vaccination rates, there was a persistent lack of awareness and concerns about side effects among both groups. This gap indicates a need for more targeted educational initiatives to address misconceptions and promote vaccine safety. The study's limited interviews, cross-sectional design, and sample may not fully represent all UAE demographic groups.

Conclusion: Despite the high vaccination rates, there is a persistent lack of awareness and concerns regarding side effects. This study highlights the importance of culturally tailored communication and increased advocacy by healthcare providers to address concerns about vaccination and prevent misconceptions among the diverse population in the UAE.

Aims: The similarity between mucopolysaccharidosis (MPS) patients and juvenile idiopathic arthritis (JIA) leads to ineffective and needless anti-rheumatic treatment and delayed initiation of enzyme-replacement therapy, the early start of which is crucial for diminishing the disease progression and yielding better outcomes Background: The prevalence of joint involvement in the attenuated forms of MPS leads to the JIA misdiagnosis, especially in patients without distinctive facial dysmorphic features.

Objective: Our study aimed to compare mild forms of MPS and JIA and create a diagnostic score helping to differentiate both conditions.

Methods: 41 patients with mild forms of different types of MPS and 255 JIA patients with polyarthritis were included in the retrospective study. The routine clinical and laboratory features were used for the comparison analysis.

Results: The main features of the MPS cohort were younger age at the disease onset, lower weight and height lower inflammation, and higher number of affected joints compared to JIA patients and involvement in the organs and system, specific for MPS. The majority of the patients had similar C-reactive protein levels. At least two extra-articular features with polyarticular involvement were the main discriminating factors for both conditions. The sum (>38) of the following criteria:ESR< 12 mm/h (38 points), growth delay (height ≤ -2.0 SD; 20 points), age of joint involvement < 1.0 years (24 points), male sex (15 points), and involvement of both elbows with limited range of motion (29 points) can help in the differentiation If a patient had no extra-articular features, typical for MPS. The specificity and sensitivity of this model are 91.0% and 92.7% respectively.

Conclusion: This diagnostic algorithm might increase the suspicion of MPS and should be added to routine testing not miss the mild form of MPS inside JIA. Early diagnosis of mild cases allows for the initiation of treatment in patients with MPS at an earlier stage, which can significantly improve their daily functioning and quality of life.

Objective: To explore the prevalence and characteristics of infantile idiopathic hypotonia in the literature and investigate a potential association with vestibular dysfunction in young children.

Methods: A scoping review was conducted following the Joanna Briggs Institute (JBI) guidelines and Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) standards. Literature was searched across MEDLINE, EMBASE, and Web of Science. Google Scholar, GreyNet International, and trial registers were searched for grey literature. Eligible articles were required to report features of children with idiopathic hypotonia or a predefined synonym.

Results: Of 4,174 screened articles, 16 met the inclusion criteria. Eleven studies focused on various underlying causes of hypotonia and comprised 1150 hypotonic children, of which 24% remained undiagnosed. The remaining studies delved into the characteristics of idiopathic hypotonic children and described different developmental challenges in these children.

Discussion: Clumsiness and poor coordination were commonly reported, and therapy, aiming to enhance muscle strength, coordination, and balance, was often recommended, yet none of the included articles explored vestibular dysfunction. Conclusion Despite diagnostic advancements, the underlying cause of hypotonia remains unidentified in some children. While similarities exist between hypotonic children and children with vestibular dysfunction, none of the reviewed studies explored vestibular dysfunction, warranting further investigation.

Netherton syndrome (NS), also known as Comèl-Netherton syndrome, is a rare disorder of cornification resulting from pathogenic variants in the Kazal type 5 (SPINK5) gene encoding serine protease inhibitor LEKTI. NS is characterized by the triad of congenital ichthyosiform erythroderma (CIE)/ichthyosis linearis circumflexa (ILC), trichorrhexis invaginata (TI), and atopic diathesis. Due to the severity of pathogenesis and variability in clinical presentations, the management of NS poses significant therapeutic challenges, which often require a multidisciplinary approach. Current treatment modalities predominantly consist of topical interventions and supportive measures. With an improved understanding of NS pathophysiology, emerging literature has suggested novel systemic therapeutic options for NS, which include repurposed biologics like targeted inhibitors and intravenous immunoglobulins, but there remains a paucity of literature regarding their clinical outcomes. Although often cited among dermatologists and allergists, the condition is rare in Hong Kong and Singapore, and most physicians have not managed any case. This review suggests that NS may be underestimated clinically. We aim to raise awareness for clinicians in the specific clinical characteristics and pathophysiology of NS to decide tailor-made treatment plans and improve patient outcomes.

Introduction: The Federal Maternal and Child Health Bureau defines Children with special health care needs (CSHCN) as those who have, or are at increased risk for, chronic physical, developmental, behavioral, or emotional conditions, and require health and related services beyond what is generally required by other children. More than half of the entire Italian internationally adopted children (IAC) population has special needs. This monocentric retrospective study aims to describe the demographic features and prevalence of several chronic conditions in a large cohort of IAC.

Methods: Between 1 January 2009 and 31 October 2023, we consecutively enrolled all IAC referred to Anna Meyer Children's University Hospital, in Florence. This process followed a standardized operative protocol developed internationally. Then, univariate logistic regression analyses were performed.

Results: Among 2694 IAC, 315 children (11.89%) were found to be affected by a chronic condition. Asia appears to have 101/561 (18.00%) prevalence of diseases in the total number of IAC from this region of origin, followed by Eastern Europe with 135/1030 (13.11%), Latin America 63/598 (10.54%) and Africa 16/435 (3.68%). The countries of origin with higher prevalence of chronic conditions were China (57.75%), India (15.89%), Russia (15.44%), and Ukraine (13.79%). Children adopted from China and India have a high prevalence of malformations (p = 0.016), while children adopted from Russia accounted for 69.77% of the total fetal alcohol syndrome affected in our cohort. Mental and behavioral disorders appear significantly more prevalent in children from Latin America (p = 0.015), whereas endocrinological disorders predominate in children from Africa (p = 0.014). High rates of precocious puberty were recorded in Asia (p = 0.018) and Africa (p = 0.001) in comparison with the other regions of origin.

Conclusion: A revision of the current definition of special needs for adoptive purposes is recommended. Italian screening of the adopted child could be tailored considering the country of origin, and the study of mental health should definitively become part of it.

Background: Intrauterine parvovirus B19 infection usually causes fetal anemia, hydrops, and stillbirth. Less commonly, central nervous system injuries have also been described.

Case presentation: We report an atypical case of severe brain injury with diffuse malformation of cortical development and severely immature gyration in a preterm infant with profound fetal anemia and hydrops due to congenital parvovirus B19 infection. The fetus was treated with multiple intrauterine transfusions. Postnatal magnetic resonance imaging of the brain showed extensive encephalomalacia with severe volume loss of both hemispheres, associated with a deranged, markedly immature gyration.

Conclusion: This case provides the first description of severe delay in brain maturation in intrauterine parvovirus B19 infection and represents evidence of a possible teratogenic effect of parvovirus B19 on the brain tissue.

The literature review presents data from a limited number of available studies conducted over the last two decades on immunological deficiency in congenital heart defects (CHDs), which is the cause of frequent infectious complications before and after cardiac surgery. Several studies based on screenings at various levels indicate the presence of primary and secondary immunodeficiency in CHDs, in particular about 13 genetic syndromes in which CHD is combined with immunodeficiency. The available data suggests a greater severity of immunological disorders in patients with critical CHDs, cyanotic CHDs, and conotruncal defects with T-cell dysfunction and deficiency of immunoglobulins (especially the IgG class, mainly IgG4) than in patients with shunts and obstructive defects. To identify defects in the T- and B-cell components of the immune system, quantification of the DNA of T-cell receptor excision circles (TRECs) and K-deleting recombination excision circles (KRECs)-by-products of the maturation of T- and B-cell receptors- has proven helpful in the world practice of neonatal screening. It allows the evaluation of a number of functionally mature T- and B-cells. In Russia, however, its widespread use started only in 2023. Data on the use of this assay in infants with CHDs are represented by isolated case reports. In Russia, a combination of CHD and primary immunodeficiency was found in 37% of cases in the Sverdlovsk Region. We conducted our own study of 200 children with CHD; 5% of cases were syndromic forms of CHD. 48.5% of children were admitted to the cardiac surgery clinic in critical condition. A decrease in the TREC level was detected in 23.5% of cases, including all children with syndromic CHD. In the group of patients with immunological disorders, there were significantly more children with cyanotic CHD, children admitted in critical condition, and children with conotruncal defects. Infectious complications in the postoperative period (sepsis, pneumonia, tracheobronchitis, postoperative wound infection) were observed significantly more often in 47 children with reduced TREC levels compared to children with normal TREC levels (P = .00000, in 36% and 3.6%, respectively). The analysis of publications confirms the prognostic value of TREC and KREC screening for targeted preoperative preparation to reduce postoperative complications and decrease the risk of mortality in CHDs.

In preterm infants, necrotizing enterocolitis (NEC) is one of the most devastating complications seen in the neonatal intensive care unit (NICU), with both short- and long-term sequelae. Outcomes reported for mono- (versus di-) chorionic twins suggest an increased incidence in the monochorionic twins, usually attributed to abnormal placental vascular connections. Same sex concordance and zygosity data suggest shared genetic and environmental factors as important contributing factors to the pathogenesis of NEC. This concept is further supported by fecal microbiome data from preterm twins. However, there is also some uncertainty in these observations because studies that controlled for gestational age did not show significant differences between singletons and twins in the occurrence of NEC. This mini-review was undertaken as a critical appraisal of the published literature in terms of twin studies to establish the genetic relevance vis-àvis NEC. There is a need for further evaluation of genetic and confounding factors arising from the environment and the gut microbiome in these infants using cohorts with larger sample sizes.