Frontiers in Pediatrics最新文献

Objective: Capillary leak syndrome (CLS) is an urgent problem in postoperative patients, is challenging to diagnose early, and has a poor prognosis. We investigated a quick and convenient diagnostic indicator of secondary CLS in children after cardiopulmonary bypass (CPB).

Methods: We conducted this single-center, observational, prospective study in the Department of Critical Care Medicine at the Children's Hospital of Chongqing Medical University. All the data were collected within 24 h after cardiopulmonary bypass (CPB). The secondary CLS risk factors were determined using univariate and multivariate logistic regression analysis, and the cut-off point of secondary CLS was found by receiver operating characteristic (ROC) analysis.

Results: Our study included two hundred four pediatric patients in the PICU after cardiopulmonary bypass (CPB). 42.65% (87/204) of patients were diagnosed with secondary CLS. The incidence of acute kidney injury (AKI) was 36.76% (75/204), and the mortality was 5.39% (11/204). Logistic analysis indicated that a pulmonary exudation on chest radiograph, a high thoracic fluid content (TFC) and a higher vasoactive inotropic score (VIS) were independent risk factors for secondary CLS [odds ratio [OR] 23.62, 95% confidence interval [CI] 7.20-90.41, p < 0.001; OR 1.08, 95% CI 1.02-1.16, p = 0.010; OR 1.06, 95% CI 1.01-1.14, p = 0.049; respectively]. According to the ROC analysis, the cut-off point for the TFC was 52 (Ω^-1).

Conclusions: The TFC plays a key role in the early prediction of secondary CLS in children after CPB, and this novel indicator may help clinicians initiate intensive treatment as early as possible.

Objective: To analyze etiologies and management of postoperative intestinal obstruction following surgery (exeision of the dilated bile duet and Roux-enY hepaticojejunostomy) for congenital biliary dilatation (CBD) in children.

Methods: A single-institution retrospective review was conducted on 475 patients who underwent Roux-en-Y hepaticojejunostomy following complete excision of the dilated bile duct. Among the cohort, nine patients underwent reoperation for intestinal obstruction. The perioperative data of these cases were thoroughly analyzed.

Results: The cohort (8F:1M) developed obstruction 20 days-8.8 years postoperatively. Primary etiologies included internal hernias (Petersen's:2, transverse mesocolic:3, Brolin's:1), biliary-jejunal loop torsion (1), and adhesions (2). Three patients underwent redo biliary-enteric anastomosis secondary to Roux-en-Y loop necrosis. Cross-sectional imaging in children with internal hernia or Roux-en-Y volvulus demonstrated distended, fluid-filled biliary-jejunal loops at the porta hepatis. Surgical indications for intestinal obstruction included peritoneal signs, aggravated abdominal pain, and failure of conservative treatment. Two children with intestinal obstruction had abnormal liver function tests preoperatively.

Conclusion: Internal hernias (particularly within the internal hernia triangle) are the predominant cause of post-CBD surgery obstruction. Cross-sectional imaging shows high diagnostic sensitivity. Given the higher likelihood of internal hernia as a cause of post-CBD surgery obstruction and its rapid progression to Roux limb necrosis, early surgical intervention should be considered.

Background: Pediatric stone disease, once considered rare, has gained significant attention over the past decade owing to its rapidly increasing incidence. Despite this surge, a comprehensive evaluation of this burden is lacking.

Objectives: This study aimed to estimate the burden of pediatric stone disease, stratified by age and sex, at the global, regional, and national levels from 1990 to 2021.

Methods: Data on the global incidence, deaths, and disability-adjusted life years (DALYs) related to pediatric stone disease from 1990 to 2021 were collected. The estimated annual percentage change (EAPC) quantified the disease trends over this period. Additionally, the relationship between disease burden and factors such as age and sociodemographic index (SDI) levels was analyzed. A Bayesian Age-Period-Cohort (BAPC) model was employed to project the future burden from 2022 to 2041.

Results: In 2021, there were 3,289,663 cases of pediatric stone disease worldwide (95% UI: 1,724,296 to 5,384,797), resulting in 66 deaths (95% UI: 43 to 94) and 14,230 disabilities (95% UI: 9,264 to 21,569). Regionally, South Asia reported the highest incidence, mortality, and DALYs based on the Global Burden of Disease (GBD) classifications. Age-standardized morbidity (ASIR) and age-standardized mortality (ASDR) are highest in Eastern Europe, while age-standardized mortality (ASMR) is 0 in all regions of the world. At the country level, India recorded the highest incidence, mortality, and DALYs for pediatric stone disease in 2021. Armenia had the highest ASIR, while 28 countries, including Afghanistan, Armenia, and Brazil, reported the highest ASMR. Armenia and Kazakhstan recorded the highest ASDR. The disease burden was most pronounced among children aged 15-19 years, with boys being more affected than girls. These findings have significant global implications. Projections indicate that by 2041, the burden of pediatric stone disease will decline, although boys will continue to be more affected than girls.

Conclusion: From 1990 to 2021, the global burden of pediatric stone disease, adjusted for age, has decreased. However, regional variations persist, with some areas experiencing an increase in burden. This underscores the importance of ongoing monitoring to effectively reduce the overall impact of pediatric stone diseases.

Objective: Hepatopulmonary fusion (HPF) is a rare congenital malformation, frequently associated to right-sided congenital diaphragmatic hernia (CDHR). The presence of HPF often leads to a fatal outcome. The most effective approach to managing this condition remains uncertain due to the limited number of documented cases in the literature.

Study design: This case presents a 11-day old full-term female neonate with HPF associated to CDHR. The definitive diagnosis of HPF was made during surgery for CDHR. Our team opted for a simple repair of the diaphragmatic defect and no attempts were made to separate the liver from the right lung.

Results: Our approach was successful, as our patient not only survived the procedure but also showed favorable cardiorespiratory adaptation, consistent growth, and regular neurodevelopment, according to follow-up data, available at six months of life.

Conclusion: The adopted surgical management strongly suggests that when the diagnosis is made intraoperatively and detailed knowledge of the vascularization is lacking, partial separation of the viscera, preserving the medial hepatopulmonary fusion and suturing the diaphragm, is the successful approach.

[This corrects the article DOI: 10.3389/fped.2024.1514079.].

Objective: This study aimed to evaluate the clinical value of ultrasonography in the management of disorders of sex development (DSDs).

Methods: Ultrasonographic appearance and clinical data of 82 cases with DSD were reviewed retrospectively.

Results: In total, there were 54 cases with the male phenotype and 28 cases with the female phenotype. All 12 cases with ovotesticular DSD were confirmed by surgery or pathological examination. Furthermore, 2 of 12 cases with ovotesticular DSD were misdiagnosed by ultrasonography.

Conclusions: Ultrasonography can not only evaluate the internal sex organs in pediatric patients, but also estimate the type, location, size, and morphology of the gonads, which provides important imaging evidence for clinical diagnosis and treatment.

Introduction: Advancements in prenatal care have underscored the importance of understanding postnatal outcomes and prognosis in sacrococcygeal teratoma (SCT). However, giant SCTs continue to pose surgical challenges and are associated with increased morbidity and mortality. A clear, objective threshold for defining a "large" tumor relative to the patient's size remains undefined. This study aimed to establish objective tumor size indicators for predicting surgical outcomes and prognosis.

Methods: Data from 97 patients diagnosed with and surgically treated for SCT at Asan Medical Center from 2000 to 2021 was retrospectively reviewed. The tumor volume/birthweight ratio (VWR) and tumor length/height at birth ratio (LHR) were measured. Surgical outcomes and prognosis were evaluated based on these measures, including surgical complications, concomitant surgeries, long-term complications, and recurrence.

Results: After surgery, 38 patients experienced short-term complications, 18 required additional operations, 16 developed long-term complications, and 14 experienced tumor recurrence. Both VWR and LHR correlated with short and long-term complications, additional surgeries, and recurrence.

Discussion: The small patient population limited the determination of precise cut-off values; however, a significant difference was observed between groups stratified by the most predictive cut-off values. Both objective tumor size indicators were significantly associated with prognosis and surgical outcomes. Notably, both indicators exhibited comparable predictive capabilities without discrepancies.

Background: Cholestatic hepatopathy is common in infants. While many studies link gut microbiota to liver and gallbladder diseases, the relationship between infantile cholestatic hepatopathy (ICH) and gut microbiota remains unclear.

Methods: We collected stool samples from 19 healthy controls and 33 infants with ICH aged ≤3 months, then determined the intestinal microbiota by 16S rDNA sequencing. The differences of microbiota structure and functional between the two groups were analyzed.

Results: Alpha-diversity analysis showed that the Chao1 and ACE indexes were significantly higher in the ICH group than control group (p < 0.05). LEfSe analysis showed that 18 bacteria taxa, including Streptococcus, Streptococcaceae, and Staphylococcales, enriched significantly in the ICH group, and 3 bacteria taxa were enriched in the control group. At the genus level, the relative abundance of Streptococcus, Escherichia-Shigella, and Lactobacillus in ICH group was higher than control group (p < 0.05). The Receiver Operating Characteristic (ROC) analysis demonstrated that Streptococcus was highly valuable in distinguishing ICH from healthy controls. Moreover, functional prediction analysis identified 59 metabolic pathways potentially associated with ICH.

Conclusion: Gut microbiota dysbiosis is associated with infantile cholestatic hepatopathy, and Streptococcus can be used as an essential biomarker to identify ICH.

Introduction: Differences of sex development (DSD) encompass several rare diagnoses with medical and social implications. If a child is suspected of having DSD, timely and comprehensive information to the family is crucial for an undisturbed parent-child relationship and a good outcome. Providing information and competent care for a child with DSD is challenging for medical staff and parents, especially at the beginning of care, when many diagnostic results are still pending. The Empower-DSD information management program provides a structured multidisciplinary care and information exchange for children and their parents in the first 8-12 weeks after presenting to a specialized DSD center.

Methods: From June 2020 to August 2022, 51 families completed the structured care pathway in 4 DSD centers in Germany as part of the government-funded Empower-DSD study. The program was evaluated with a quantitative and a qualitative approach. Diagnosis, age of child, total duration, number of appointments, and completed elements of care were documented. Semi-structured guided interviews with parents, peers and professionals were used to explore expectations and the experience of the involved stakeholders.

Results: Care elements were documented in 11 children with congenital adrenal hyperplasia (CAH) and 28 children with other DSD-diagnoses (chromosomal DSD; 46, XY-DSD; 46, XX-DSD) with a mean age of 1.8 years (0-18 years). In total 45 people were interviewed. The information management program alleviated stress and uncertainties for parents and encouraged a trusting relationship with the DSD team. Professionals rated the developed materials as a valuable tool to provide consistent and thorough care. Parents underlined the importance of the early access to specialized DSD teams, a clear and open communication and the reassuring attitude of professionals in DSD care. Parents and professionals stated that the program required time and resources and would prefer an individualized approach instead of a predefined duration.

Conclusion: The structured, multidisciplinary support within the first weeks after a DSD diagnosis was perceived to be of high quality by all stakeholders involved. Information on the nature of the decision-making process and peer narratives could be added to the information material.

Objective: This study aims to analyze and summarize the clinical characteristics of small intestinal adenomyosis.

Methods: A retrospective study was conducted on children with small intestinal adenomyosis at our center from 2014 to 2024. The age of onset, gender, clinical symptoms, auxiliary examination results, treatment plans, and pathological characteristics of the tumors were recorded and analyzed.

Results: Six cases of small intestinal adenomyosis were analyzed. The male-to-female ratio was 5:1. The median age was 19 months, with two-thirds of the affected children being under 2 years old. Two cases were identified in newborns during the treatment of intestinal malrotation. The remaining patients developed symptoms of intussusception. Preoperative ultrasound identified pathological lead points in two cases. Among the six cases, one case's adenomyoma was located in the jejunum, while the rest was located in the ileum, with tumors ranging from 25 to 140 cm from the ileocecal region. All six patients underwent resection of the tumor segments in the small intestine followed by anastomosis; postoperative prognosis was favorable. Of the six pathological results, glandular-like structures were found in five tumors, with three cases lined with cuboidal or columnar epithelium and one case lined with stratified or squamous epithelium.

Conclusions: Small intestinal adenomyosis is observed to be more prevalent in male, with onset ages ranging from six days to four years. It frequently coexists with recurrent intussusception, making reduction with air or water enema challenging. Abdominal ultrasound typically shows tumors at the leading edge of the intussusception, with compromised blood flow signals and multiple small cystic or honeycomb-like anechoic structures. Surgery is the primary treatment and generally results in a favorable prognosis.