Pediatric Reports最新文献

Background: Wernicke Encephalopathy (WE), a neurological disorder often linked to alcohol use, can also occur under non-alcoholic conditions, including in pediatric populations.

Methods: This systematic review examines 88 pediatric WE cases reported over the past 30 years, encompassing diverse etiologies such as cancer (25 cases), gastrointestinal diseases (19), malnutrition (17), psychiatric disorders (13), obesity surgery (5), renal disease (4), COVID-19 (2), PICU complications (1), hyperemesis gravidarum (1), and a genetic mutation (1).

Results: Prodromal symptoms included nausea (60%) and vomiting (55%). In total, 37% of the patients received parenteral nutrition without thiamine before WE diagnosis, often progressing to Wernicke-Korsakoff syndrome (WKS). Key findings revealed the classic triad of WKS, eye movement disorders (80%), mental status changes (75%), and ataxia (63%), with MRI demonstrating high diagnostic sensitivity (85%). Treatment varied widely; higher parenteral thiamine doses correlated with faster recovery and better outcomes, while insufficient dosages led to adverse effects. Full remission was achieved in 61% of cases, with improved outcomes in more recent reports due to refined dosing protocols.

Conclusions: These findings underscore the importance of early recognition of nausea and vomiting as predictors of pediatric WE and the critical need to incorporate thiamine in parenteral nutrition for children. Optimal dosing remains vital for recovery, particularly in severe cases.

Mediastinal hemangiomas, particularly those of thymic origin, are rare phenomena. Due to its rarity, this pathologic condition is not characterized as related to the angiogenic profile of hemangioma endothelial cells. The diagnosis is challenging clinically and radiologically, and biopsies may not yield a definitive answer. Surgical resection offers the material for histologic diagnosis, relieves symptoms, and has a favorable long-term prognosis for such benign tumors. Sometimes, such benign tumors may have aggressive behavior and repeated recurrences but the causes responsible for this unpredictable evolution are not actually known. A case of intrathymic hemangioma diagnosed in a 16-year-old girl is presented here. We focused equally on a multidisciplinary approach to this challenging diagnosis but also on the characterization of the hemangioma endothelial cells profile not previously performed for such type of vascular anomalies. To define an antibodies panel for the evaluation of intrathymic hemangiomas may help in the full characterization of this rare vascular lesion, and subsequently focus on the new therapeutic targets which may be applied for cases with aggressive behavior.

A limp is an abnormal, uneven or laboured gait typically resulting from pain, weakness, or structural deformity involving the hip, lower limb, spine or abdominopelvic abnormalities. Limps in children are common and have diverse causes that can be benign to life-threatening including trauma, congenital malformations, and neoplastic diseases. Diagnosis involves identifying gait abnormality thoroughly examining history and physical exam, assessing tenderness and range of motion, and completing targeted lab and radiographic studies. We present an imaging review of various usual and unusual causes of limp in different age groups such as in toddlers (1-3 years), children (4-10 years), and adolescents (11-16 years) with a comprehensive literature review.

Inguinal hernia repair (IHR) is a common surgical procedure among neonates and infants; the time of surgery is one of the major factors affecting its outcomes. Our systematic review aims to evaluate the effects of surgical timing on outcomes in inguinal hernia repairs in the newborn and infant population to establish evidence-based guidelines for optimal surgical timing. A systematic search was performed in PubMed, MEDLINE, and Web of Science databases, following PRISMA guidelines. Studies evaluating neonates and infants undergoing IHR with outcomes of recurrence, complications, and postoperative recovery were included. Data were collaboratively extracted, including patient demographics, surgical approaches, perioperative complications, and long-term outcomes. Early repair (0-28 days of life) decreased the risk of hernia incarceration but also increased the risk of preoperative complications. Delayed repair (29 days to 1 year of life) showed fewer preoperative complications but increased the risk of incarceration. The outcomes were affected by variables including patient maturity and comorbidities, along with hernia severity. Neonates with a high risk for incarceration are best treated with early repair, while stable infants can be managed safely with delayed repair. More randomized trials are needed to develop standardized guidelines that balance the associated risks of neonatal versus infant repair strategies to maximize benefits.

Background/objectives: Anterior crossbite is characterized by a reverse sagittal relationship between the maxillary and mandibular incisors. Early treatment of an anterior crossbite is advocated to avoid periodontal and traumatic consequences on lower dentition along with growth disturbances in the anterior maxilla and mandible. The present case series describes the usage of a sagittal expansion screw integrated with a removable maxillary plate or fixed appliance to correct an anterior upper single dental crossbite and outlines the clinical rationale and the protocol associated with this appliance system.

Methods: A total of four children presenting anterior crossbite were treated using a sagittal expansion screw integrated into a removable plate or a fixed appliance.

Results: All patients were successfully treated in less than 4 months. Overjet and overbite were normalized by the end of treatment. The orthodontic treatment received positive feedback from parents and caregivers, who seek a rapid improvement in their children's aesthetics and function. No significant discomfort or speech difficulties were reported by the parents or patients.

Conclusions: The present case series would suggest that a sagittal expansion screw, integrated into both a removable and fixed appliance, can represent a valuable and versatile treatment option for correcting an anterior crossbite.

Background/objectives: This study aimed to assess and compare the rates of medication error (ME) using the PediSTAT application compared to the conventional method of calculating the correct dose and determining the appropriate route of medication administration for common pediatric emergencies.

Methods: A prospective cross-sectional study design was used for the study. Data were collected using a questionnaire that was distributed to certified paramedics holding a bachelor's degrees or higher and working in Riyadh City, Saudi Arabia. Alternate simple random sampling was used to recruit the participants into two groups using the same questionnaire: the PediSTAT group and the conventional method group. The questionnaire contained four pediatric emergency vignettes: cardiac arrest, asthma exacerbation, seizures, and hypoglycemia.

Results: A total of 63 participants agreed to the study. Almost 80% of them were males, 81% held bachelor's degrees, and 87% were certified in pediatric resuscitation courses. The findings of the study showed that the use of the PediSTAT application increased accuracy and reduced the risk of ME for common pediatric emergencies. This was shown to be statistically significant for asthma medication dose (p-value < 0.001, 95% CI 0.034-0.352), midazolam dose (p-value = 0.012, 95% CI 0.030-0.764), and hypoglycemia medication dose (p-value < 0.001, 95% CI 0.046, 0.452).

Conclusions: The study findings supported the use of standardized precalculated applications such as PediSTAT, which was shown to reduce the risk of ME in prehospital care for pediatric emergencies.

Low birth weight (LBW) is a significant concern not only because of its association with perinatal outcomes, but also because of its long-term impact on future health. Despite the physical differences among individuals of different ethnicities, the definition of LBW remains the same for all ethnicities. This study aimed to explore and discuss this issue. We compiled national data from several countries and found that maternal height was negatively correlated with LBW incidence. We discovered the INTERGROWTH-21st chart may not be suitable for the Japanese population, as the Japanese birth weight chart differs from the INTERGROWTH-21st chart. Researchers have reported different LBW cutoff values used to assess adverse perinatal outcomes for different countries. However, there is currently no definition of LBW independent of the mother's country of origin that can be used for predicting the risk of adverse health outcomes. Therefore, the current era of personalized healthcare may be the perfect time to establish a standard definition of LBW which is independent of the mother's country of origin. Considering the future of healthcare, it seems an apt time to discuss the development of a more meaningful definition of LBW that can be applied across ethnicities. Further research is needed to investigate the cutoff values of LBW in every ethnicity.

The aim of our study was to investigate the role of ABCB1 polymorphism in the pharmacokinetics of carbamazepine (CBZ) in children. The study enrolled 47 Serbian pediatric epileptic patients on CBZ treatment. Genotyping for ABCB1 1236CABCB1 1236CABCB1 + 0.0176 × CYP1A2 + 0.000151 × DD where SEX has a value of 1 if male and 0 if female, ABCB1 has a value of 1 if C-G-C/T-T-T and 0 if any other ABCB1 diplotype, CYP1A2 has a value of 1 if -163A/A and 0 if -163C/C or C/A, and DD is the total CBZ daily dose (mg/day). The presence of the ABCB1 1236T-2677T-3435T haplotype is associated with an increased clearance of CBZ in pediatric epileptic patients.

Objectives: Intravenous midazolam is widely used for sedation in critically ill children. Sometimes, these children develop a paediatric delirium (PD). Our aim was to determine the relationship between midazolam serum concentration and the development of new PD in critically ill children.

Design: Prospective observational pilot study.

Setting: Paediatric Intensive Care Unit (PICU), Groningen, the Netherlands.

Patients: All children admitted to the PICU from October-December 2019 who received continuous midazolam administration.

Interventions: None.

Measurements and main results: Twenty-five percent (n = 7) of the included patients (n = 28) developed new PD. In most patients, PD occurred following midazolam dose reduction. The median cumulative midazolam dose was higher in patients who developed PD compared to those without PD. We analysed 104 blood samples to determine the midazolam concentrations. To determine whether patients had PD, the Sophia Observation withdrawal Symptoms-Paediatric Delirium (SOS-PD) score was used. Patients suffering PD (n = 7) had a lower median midazolam concentration on that day compared with the day prior to PD detection. Analysis of the active metabolites, 1-hydroxymidazolam and 1-hydroxymidazolam glucuronide, showed similar results.

Conclusions: PD may be linked to a sudden and significant reduction in the midazolam concentration in critically ill children. Further investigation in larger patient populations is necessary to validate our findings.

Background/objectives: Level 1 autism spectrum disorder (ASD) is a neurodevelopmental condition characterised by challenges in social and communication skills. Despite these difficulties, individuals with level 1 ASD often exhibit average intelligence and typical language development. Improving socialisation skills in this population requires tailored approaches that address their specific needs and include targeted strategies. This study aims to evaluate the effectiveness of a structured social skills training programme for adolescents and young adults with level 1 ASD.

Methods: Participants diagnosed with level 1 ASD, regardless of gender, were consecutively recruited from an outpatient clinic. The intervention involved activities from the Social Skills, Autonomy, and Awareness Module, specifically designed for adolescents and young adults. Sessions were conducted fortnightly, lasting 1.5 to 3 h each, over 17 months. Adaptive behaviour was assessed using the Vineland Adaptive Behaviour Scales (VABS) at baseline and after completing the programme. Data were analysed with SPSS version 22.0 (SPSS Inc., Chicago, IL, USA). Statistical methods included automatic clustering to identify optimal clusters and Pearson's Chi-square and Fisher's exact tests to evaluate variable distributions among the clusters.

Results: A total of 31 participants (77.4% female) with a mean age of 20.1 years (SD = 7.0) were included in the study. Two distinct clusters emerged. Cluster 1 (n = 8) had significantly higher mean ages and baseline Vineland II socialisation scores than Cluster 2 (n = 23). Both clusters demonstrated significant improvements in social skills following the intervention.

Conclusions: This study highlights distinct profiles within individuals with level 1 ASD, showing a clear link between age and social skill development. The intervention improved social skills for most participants, regardless of the age at which treatment began. For some individuals, alternative or augmented treatment strategies may be necessary to achieve optimal results.