Frontiers in Pediatrics最新文献

Background: Pediatric thrombosis is a relatively rare but severe condition in the field of pediatrics, with far-reaching consequences. Recent studies have indicated a rising incidence of this disease in children over the years. Additionally, the pharmacological treatment of thrombotic diseases in children faces numerous challenges. Due to significant physiological differences between children and adults, guidelines for the prevention and treatment of thrombotic diseases in adults cannot be directly applied to pediatric patients.

Purpose: A systematic review of the existing evidence-based medical literature should be conducted to propose pharmacological prevention and treatment recommendations for pediatric thrombotic diseases. Developing a comprehensive and practical pharmacotherapy guideline for the prevention and treatment of pediatric thrombotic diseases is essential to enhancing the rational use of medications in managing these conditions in children.

Methods: The guideline development followed the World Health Organization's (WHO) Handbook for Guideline Development. This involves systematically searching and extensively collecting data on common medication issues in the prevention and treatment of pediatric thrombosis nationwide. The Delphi method was used to survey experts and identify the clinical issues to be included. Subsequently, a systematic literature review was conducted to evaluate existing primary studies, systematic reviews, and guidelines or consensus statements from professional organizations. The quality of the evidence was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. The Delphi method was employed again to reach a consensus on the recommendations and evidence levels. This process was culminated in the development of the Guideline for Pharmacological Management of Thrombotic Diseases in Children.

Results: During the guideline development process, a total of 29 clinical issues were collected and evaluated by 78 experts in clinical pharmacy and clinical medicine. Through two rounds of surveys, 13 clinical issues were selected. Under the supervision of two methodologists, 13 clinical pharmacotherapy recommendations were formulated.

Conclusion: By conducting a comprehensive assessment of the feasibility and safety of clinical practices, the guideline provides specific anticoagulant medication recommendations for pediatric healthcare professionals. This will help enhance the prevention and treatment of pediatric thrombosis, promoting more standardized and effective medical practices.

Purpose: To understand the status of vitamin D deficiency in girls with growth and developmental variations, as well as the impact of COVID-19 on their vitamin D levels, and to provide reference for the prevention and treatment of vitamin D deficiency in children.

Methods: A retrospective analysis was conducted on 1,345 instances of girls with growth and developmental variations who visited our pediatric endocrinology department and completed vitamin D detection. A total of 279 girls with complete data were included in this study. Among them, 246 girls were classified into four groups based on different growth and developmental variations: early puberty group, menarche group, obesity group, short stature group, and 33 healthy girls served as the control group. Besides, the girls were divided into pre-epidemic and post-epidemic groups by the occurrence of the COVID-19 epidemic. Vitamin D were measured in all girls. The 25(OH)D <20 ng/ml was used as the standard for vitamin D deficiency.

Results: The levels of vitamin D in the early puberty group, menarche group, obesity group, short stature group, and control group were as follows: 20.23 ± 5.90 ng/ml, 17.85 ± 5.69 ng/ml, 21.31 ± 8.99 ng/ml, 27.90 ± 12.27 ng/ml, and 29.42 ± 12.65 ng/ml, respectively. The levels of vitamin D in the early puberty group, menarche group, and obesity group were significantly lower than those in the control group (P < 0.05). The individual vitamin D deficiency rates in the aforementioned groups were 52.07%, 73.91%, 59.46%, 30.95%, and 30.30%, respectively. The vitamin D levels in the pre-epidemic and post-epidemic groups were 20.48 ± 6.22 ng/ml and 22.50 ± 9.74 ng/ml, respectively (P > 0.05).

Conclusion: Girls with early puberty, menarche, and obesity have a certain deficiency of vitamin D levels, and appropriate vitamin D treatment should be provided clinically. Girls with short stature and healthy girls also have certain levels of vitamin D deficiency, and awareness of prevention should be strengthened.

Introduction: Neonates with congenital diaphragmatic hernia (CDH) have an associated high mortality and morbidity. The European CDH EURO consortium has developed guidelines for initial and perioperative ventilatory management. There are, however, no recommendations on how to wean these patients from the ventilator. Extubation failure is more frequent in this group of patients than in other neonates. The aim of this study was to describe patient characteristics and risk factors for failed extubation and to evaluate predictive factors for successful weaning.

Methods: We performed a retrospective study in a single centre tertiary pediatric intensive care unit in Stockholm, Sweden. CDH-patients (n = 38), aged 0-28 days, with extubation events were identified from 2017 to 2019. Eight patients (21.1%) needed reintubation within 24 h after the first extubation attempt. Patient demographics, surgical repair with patch, oxygenation saturation index (OSI), rapid shallow breathing index (RSBI), ventilatory settings, fluid balance and sedation on the day of extubation were recorded.

Results: Patients in the failed extubation group (FE) had lower birth weight (p < 0.05), surgical patch repair (p < 0.05), longer length of stay in intensive care (p < 0.05), longer time on the ventilator (p < 0.05) and other comorbidities (p < 0.001). Using logistic regression we identified OSI, RSBI and inspiratory pressure (Pinsp) as factors predicting a successful extubation, AUCROC 0.95 (95% CI: 0.87 to 1.00). Patients in the FE-group had significantly more often pulmonary hypertension requiring treatment (p < 0.05), a higher fraction of inspired oxygen (FiO₂) (p < 0.05) and hypercapnia (p < 0.001) prior to extubation and an oxygen demand exceeding 40% two hours after extubation (p < 0.05).

Conclusion: Useful predictors of successful extubation in CDH patients are OSI, RSBI and Pinsp. Low birth weight, patch repair and comorbidity also appear to be important factors. Prospective studies are required to confirm findings in the present study.

Cardiac iron overload affects approximately 25% of patients with β-thalassemia major, which is associated with increased morbidity and mortality. Two mechanisms are responsible for iron overload in β-thalassemia: increased iron absorption due to ineffective erythropoiesis and blood transfusions. This review examines the mechanisms of myocardial injury caused by cardiac iron overload and role of various clinical examination techniques in assessing cardiac iron burden and functional impairment. Early identification and intervention for cardiac injury and iron overload in β-thalassemia have the potential to prevent and reverse or delay its progression in the early stages, playing a crucial role in its prognosis.

Introduction: Currently, there is no established guideline for the application of ERCP in children with choledochal cyst. This study aimed to investigate the safety and effectiveness of ERCP in managing preoperative complications of choledochal cyst in children, as well as the timing for definitive surgery following ERCP.

Methods: We conducted a retrospective review of medical records for 68 pediatric patients who presented with complications of choledochal cyst, including pancreatitis and biliary obstruction combined with cholangitis. All patients underwent ERCP treatment followed by definitive surgery. The primary outcomes assessed included treatment efficacy, post-ERCP complication, and the impact of ERCP on definitive surgical procedures.

Results: Among the 68 patients studied, 41 presented with pancreatitis, while the remaining patients had biliary obstruction and cholangitis. Sixty-five patients successfully completed their treatments, with 64 experiencing alleviation of symptoms. Significant improvements were observed in serum amylase levels and liver function tests following ERCP. Post-ERCP complications occurred in three cases, including one case of pancreatitis and two cases of infection. The median interval between ERCP and surgery was 11 days. There was no significant difference in primary outcomes, such as surgical duration, rate of minimally invasive surgery, conversion to open surgery, intraoperative bleeding volume, intraoperative blood transfusion, postoperative complications, or average length of hospital stay, between the early surgery group (≤2 weeks) and the late surgery group (>2 weeks).

Conclusions: ERCP was proved to be a safe and effective intervention for alleviating preoperative complications in pediatric patients with choledochal cyst. Early definitive surgery following ERCP did not significantly impact the perioperative outcomes of pediatric patients.

Introduction: Information regarding children with Down syndrome (DS) in the Pediatric Intensive Care Unit (PICU) is limited and conflicting. We aimed to investigate the association between DS and clinical outcomes in pediatric patients admitted to the PICU at the Instituto Nacional de Salud del Niño (National Institute for Child Health, INSN for the acronym in Spanish) and to assess nutritional status within the study cohort.

Methods: This study involved the secondary analysis of a database. We included patients consecutively admitted to INSN, a tertiary care children's hospital in Lima, Peru. We collected demographic data, clinical characteristics, and nutritional status using standardized tables. The outcomes assessed included mortality, length of stay, duration of mechanical ventilation (DMV), ventilator-free days (VFD), and healthcare-associated infections (HAI). We applied Cox regression and Poisson regression analyses to explore the relationship between Down syndrome and clinical outcomes, providing both crude and adjusted results.

Results: A total of 525 children (average age 71.3 months, range 1-218 months) were analyzed. Children with DS were younger and had a higher prevalence of comorbidities, congenital heart disease, and underweight. Both crude bivariate and multivariate analyses demonstrated that children with DS had higher mortality rates, increased frequency of HAIs, longer DMV, longer PICU stay, and fewer VFD. Adjusted multivariate analysis revealed that children with DS had a significantly higher risk of developing HAIs (RR 2.95; 95% CI 1.10, 7.87) and longer DMV (RR 1.43; 95% CI 1.24, 1.65). Among the 525 children, underweight was associated with increased risk of mechanical ventilation (MV) use and longer DMV.

Discussion: Critically ill children with DS are at increased risk of developing HAIs and longer DMV. In all 525 children, underweight is associated with higher risk of MV use and longer duration of MV.

Atypical hemolytic uremic syndrome (aHUS) is a form of thrombotic microangiopathy (TMA) characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, and is caused by overactivation of the alternative complement pathway. A 13-year-old Japanese boy with an unremarkable medical history developed symptoms of TMA following coronavirus disease 2019 (COVID-19) infection with mild respiratory symptoms. He was eventually diagnosed with aHUS with a gain-of-function C3 variant. He improved with supportive therapy and plasma exchange, and did not require anti-C5 antibody therapy. In the literature, more than 20 cases of de novo or relapsed aHUS have been described following COVID-19. It has been shown that the complement lectin pathway can be activated by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike and N proteins, and the alternative pathway can be activated by the SARS-CoV-2 spike protein. The current case highlights the possibility that COVID-19, even when respiratory symptoms are not severe, can trigger aHUS.

Background: One of the most common measures of society's health is mortality among neonates. Developing and developed countries still differ significantly in neonatal mortality rates. While there are about 18 neonatal deaths worldwide for every 1,000 live births during the first month of life, less is known about neonatal mortality in developing countries, especially in East Africa. Understanding the extent of mortality during the post-neonatal period and its determinants is crucial for developing appropriate policies and strategies that could help solve the issue. Thus, the aim of this study was to identify the prevalence of post-neonatal mortality in East African countries and the factors that are associated with it.

Methods: Secondary data analysis was conducted using data from the most recent Demographic and Health Surveys, which included 11 East African countries between 2014 and 2022. A weighted sample of 225,635 live births had been used in the study. STATA/SE 14 was used for data analysis. The multilevel mixed-effects logistic regression model was applied to determine the factors associated with post-neonatal mortality. In the multilevel logistic regression model, significant factors were deemed to be associated with post-neonatal mortality at p-values <0.05. The data were interpreted using the adjusted odds ratio (AOR) and confidence interval (CI). The best-fit model has been found to be the one with the lowest deviance and highest logliklihood ratio.

Results: In East Africa, post-neonatal mortality was found to be 15 per 1,000 live births. Pregnancy type (AOR = 3.09, 95% CI: 2.30, 4.13), birth weight (AOR = 1.58, 95% CI: 1.25, 2.01), maternal age (AOR = 1.58, 95% CI: 1.32, 1.90), maternal education (AOR = 1.82, 95% CI: 1.14, 2.92), tetanus shots prior to delivery (AOR = 1.23; 95% CI: 1.06-1.42), birth order (AOR = 5.68, 95% CI: 4.48, 7.24), those born in Uganda (AOR = 1.33, 95% CI: 1.03, 1.73), and Burundi (AOR = 1.48, 95% CI: 1.11, 1.98) had the highest odds of post-neonatal death.

Conclusion: According to this study, post-neonatal mortality is higher in developing countries, particularly in East Africa. It was discovered that factors at the individual and community levels associated with post-neonatal mortality. Consequently, focus should be paid to babies born to mothers in the lowest age group, those born of multiple pregnancies, without formal educations, who did not receive tetanus shots prior to birth, and who were born in the first birth order.

Liver-related abnormalities are commonly observed in patients with congenital heart disease, and these may lead to secondary manifestations such as pruritus. Odevixibat is an ileal bile acid transporter inhibitor under investigation for the treatment of cholestatic liver diseases. Here, we describe the effects of odevixibat treatment in a pediatric patient with congenital heart disease and severe cholestatic pruritus. A 2-year-old male with Kleefstra syndrome, hypoplastic left heart syndrome, and a history of Giessen procedure and biventricular correction surgery presented to the pediatric cardiology and hepatology outpatient clinics at University Children's Hospital Bonn. Portal hypertension was evident on imaging, and the patient was experiencing severe itching attacks that did not respond to treatment with naltrexone, ursodeoxycholic acid, dimetindene, or rifampicin. Sleep and quality of life were poor. Treatment with odevixibat was initiated off label due to refractory pruritus and elevated serum bile acids. Improvements in pruritus and sleep occurred rapidly with odevixibat and were sustained for the duration of treatment. The patient's serum bile acids decreased from 111 μmol/L before treatment with odevixibat to 24 μmol/L within 1 month of initiating therapy. Relief from pruritus had positive effects on psychomotor development and quality of life. Mild diarrhea lasting 2 days was reported by the patient's mother. In this case report, odevixibat was effective and well tolerated. Together with those of previous studies in patients with progressive familial intrahepatic cholestasis and Alagille syndrome, these results suggest that odevixibat warrants further study as a potential treatment option for patients with cholestatic pruritus of diverse etiologies.

Objective: To investigate the efficacy and potential adverse events of Tuina therapy for idiopathic constipation in children with cerebral palsy (CP).

Methods: A total of 60 CP children with idiopathic constipation were enrolled and randomly divided into Tuina and control groups. The control group was treated with basic treatment and 12 sessions of lactulose oral solution, whereas the Tuina group received basic treatment and 12 sessions of infantile Tuina treatment. The following parameters were compared: the Bristol stool form scale (BSFS), the Constipation Assessment Scale (CAS) and the improvement in constipation. In addition, adverse effects were recorded.

Results: At 4 weeks after the final treatment, the percentage of infants whose constipation improved was 23 (76.7%) in the Tuina group and 21 (70.0%) in the control group (P = 0.771). Initially, the CAS score, weekly bowel movements and proportion of infants with bowel evacuation ≥2 h were comparable between the two groups (P > 0.05). At 4 weeks after the final treatment, the CAS score, weekly bowel movements and proportion of infants with bowel evacuation ≥2 h all significantly improved (P < 0.05) compared with those in the initial situation. However, no difference was found in either group at 4 weeks after the final treatment. No serious adverse reactions (such as diarrhea, abdominal pain, vomiting, subcutaneous redness, skin breakage, or syncope) were recorded.

Conclusions: Tuina was as effective as medical care in addition to basic treatment for both groups. The results of this study suggest that Tuina, as a nonpharmacological therapy, may be helpful as an alternative treatment for constipation. More advanced research and large-sample studies should be conducted.