Healthy Children Research Articles

Frequency and severity of hyponatremia in healthy children with acute illness.

Hyponatremia can occur in the acute phase of any illness through various mechanisms. However, the frequency and severity of hyponatremia are not well known across a broad range of illnesses including medical and surgical diseases and trauma. The present, retrospective chart review was conducted at Tokyo Metropolitan Children's Medical Center from 2018 to 2019. Included were healthy children aged < 16years with an acute illness, who were urgently admitted, and had their serum sodium level measured on arrival. In total, 2717 patients were urgently admitted and had their serum sodium level measured. Of these, 1890 were included. Hyponatremia was found in 260 patients (13.8%). The most common hyponatremic disease was type 1 diabetes mellitus (69%) followed by acute infectious encephalopathy (60%), pyogenic arthritis (60%), and Kawasaki disease (51%). Kawasaki disease, seizure, urinary tract infection, acute appendicitis, lower respiratory tract infection, and acute gastroenteritis were associated with a significantly lower serum sodium value than cases of fracture comprising a control group. Conversely, acute bronchial asthma exacerbation (3%), anaphylaxis (0%), intussusception (0%), acute scrotal disease (0%), head injury (1%), and fracture (0%) were very infrequently associated with hyponatremia. The present study determined the frequency and severity of hyponatremia in various, acute, pediatric illnesses, including medical and surgical diseases and trauma. Despite reports of respiratory distress and pain inducing vasopressin secretion, hyponatremia was rarely observed on arrival in patients with acute bronchial asthma exacerbation, anaphylaxis, intussusception, acute scrotal diseases, head injury, or fracture.

Plasma-derived extracellular vesicles miR-335–5p as potential diagnostic biomarkers for fusion-positive rhabdomyosarcoma

BackgroundRhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma, with embryonal (ERMS) and alveolar (ARMS) representing the two most common histological subtypes. ARMS shows poor prognosis, being often metastatic at diagnosis. Thus, the discovery of novel biomarkers predictive of tumor aggressiveness represents one of the most important challenges to overcome and may help the development of tailored therapies. In the last years, miRNAs carried in extracellular vesicles (EVs), small vesicles of endocytic origin, have emerged as ideal candidate biomarkers due to their stability in plasma and their tissue specificity.MethodsEVs miRNAs were isolated from plasma of 21 patients affected by RMS and 13 healthy childrens (HC). We performed a miRNA profile using the Serum/Plasma Focus microRNA PCR panels (Qiagen), and RT-qPCR for validation analysis. Statistically significant (p < 0.05) miRNAs were obtained by ANOVA test.ResultsWe identified nine EVs miRNAs (miR-483-5p, miR-132-3p, miR-766-3p, miR-454-3p miR-197-3p, miR-335-3p, miR-17-5p, miR-486-5p and miR-484) highly upregulated in RMS patients compared to HCs. Interestingly, 4 miRNAs (miR-335-5p, miR-17-5p, miR-486-5p and miR-484) were significantly upregulated in ARMS samples compared to ERMS. In the validation analysis performed in a larger group of patients only three miRNAs (miR-483-5p, miR-335-5p and miR-484) were differentially significantly expressed in RMS patients compared to HC. Among these, mir-335-5p was significant also when compared ARMS to ERMS patients. MiR-335-5p was upregulated in RMS tumor tissues respect to normal tissues (p = 0.00202) and upregulated significantly between ARMS and ERMS (p = 0.04). Furthermore, the miRNA expression correlated with the Intergroup Rhabdomyosarcoma Study (IRS) grouping system, (p = 0.0234), and survival (OS, p = 0.044; PFS, p = 0.025). By performing in situ hybridization, we observed that miR-335-5p signal was exclusively in the cytoplasm of cancer cells.ConclusionWe identified miR-335-5p as significantly upregulated in plasma derived EVs and tumor tissue of patients affected by ARMS. Its expression correlates to stage and survival in patients. Future studies are needed to validate miR-335-5p as prognostic biomarker and to deeply elucidate its biological role.

Life Quality of Pediatric Patients with Central Nervous System Infections: A 1-Year Follow-up

Background: Central nervous system (CNS) infections can lead to long-term motor and cognitive complications in children. Objectives: We aimed to investigate the prevalence of attention deficit-hyperactivity disorder (ADHD) and assess the quality of life in children with CNS infections. Methods: A longitudinal study was conducted on 94 children (aged 6 - 15 years) with CNS infection symptoms who were admitted to referral hospitals in Isfahan, Iran. Parents completed questionnaires assessing quality of life and neurological deficits at 6 and 12 months after discharge. All patients were evaluated by the corresponding physician for a final assessment. Results: Attention deficit-hyperactivity disorder symptoms were diagnosed in 30 patients, with viral encephalitis being the most common infection. The quality of life in children with CNS infections was significantly lower compared to that of healthy children. Conclusions: Central nervous system infections negatively impact the quality of life in children. Screening for ADHD and managing affected children can help improve outcomes. Further research and interventions are needed to mitigate long-term complications.

A method and approach for evaluating coparenting events during couples group interventions.

When interventionists stimulate productive father-mother dialogues around coparenting, there are numerous potential benefits for families. Families stand to benefit from more positive involvement of fathers with both coparents and children, key contributors to healthy child developmental outcomes. In this report, we introduce a new strategy and rating system for helping practitioners and supervisors assess the nature and quality of coparenting-related dialogues and conversations in the context of couples group interventions. The system derives from analysis of 24 relationship-enhancement groups, 13 enrolling English-speaking couples and 11 enrolling Spanish-speaking couples, all parents of young children. All groups were co-led by a male-female team explicitly trained to focus on marital and parenting themes and supervised to address couples issues - not coparenting issues explicitly. All co-leaders spoke the native language of group participants. We documented how frequently coparenting events occurred, and how the nature and quality of events varied within and across groups. Overall, in both English- and Spanish-speaking groups expressly assembled to focus on marital and parenting issues, coparenting events occurred relatively infrequently. At the same time, both mothers and fathers appeared motivated to raise and discuss issues associated with their coparenting, and extended discussions about coparenting issues broached by the parents blossomed in approximately 37% of all instances. Process-oriented (rather than didactic) co-leader responses appeared especially helpful in scaffolding prolonged coparenting discussions. We propose that use of the system as a training, supervision and self-assessment tool can help clinicians become more consciously aware of how well their interventions succeed in promoting and scaffolding coparenting conversations during group interactions.

Childhood Hypertension And Obesity In a Developing Country Setting: A Survey Among School Age Children In Calabar, Nigeria

Background Childhood hypertension is silently rising and gradually becoming a serious public health burden and concern due to its attendant significant morbidity and mortality. It is oftenneglected and rarely reported.Also, childhood obesity is increasingly assuming epidemic proportion with a consequent predisposition to cardio-metabolic diseases. Aim To determine the prevalence of childhood hypertension in association with obesity among apparently healthy school age children in Calabar Study Design/Setting A cross-sectional descriptive study conducted among randomly selected school-age children, in Calabar, Nigeria. Methods/Subjects This cross-sectional study was conducted among children, aged 6 – 12 years old who were selected from public and private primary schools through multi-stage sampling. Blood pressure and body mass index (BMI) were categorized based on the updated 2004 fourth Report on the Diagnosis, Evaluation and Treatment of High Blood Pressure in Children and Adolescents and International Obesity Trust Fund (IOTF) respectively. Results A total of 374 children were enrolledwith a male to female ratio of 1.0: 1.3 and mean (SD) age of 9.48 (1.42) years. Mean (SD) systolic blood pressure was 95.9 ±8.8mmHg and the mean (SD) BMI was 16.6 ± (2.6) kg /m2. Hypertension was observed in 9 (2.4%) out of 374 children. Hypertension occurred more in the adolescents 6(3.2%) than in the preadolescents 3(1.6%). Similarly, hypertension was higher in females 8(3.7%) than in males 1(0.6%) With regards to obesity,15 (4%) were obese with a female: male ratio of 1.5: 1. Obesity was higher in adolescents [11 (5.8%)] than in pre-adolescents [4(4%)] Conclusion The prevalence of hypertension was 2.4% and it was commoner in females than in males who were more obese. Obesity was strongly associated with increasing BP. Awareness should be created among health care professionals to regularly check for hypertension and overweight among school children for early diagnosis and treatment.

Aortic valve function and aortic dimensions in obese and overweight patients with bicuspid aortic valve.

Bicuspid aortic valve is the most common CHD and commonly associated with activity restrictions that may lead to a sedentary lifestyle known to increase obesity risk. It is unknown whether obesity is associated with changes in aortic dimensions or aortic valve function in young people with bicuspid aortic valve. This study investigates whether overweight and obese children with bicuspid aortic valve have worse aortic valve function or increased aortic dimensions compared to healthy weight children with bicuspid aortic valve. This was a single centre retrospective cohort study comprised of patients 5 to 25 years old with a diagnosis of bicuspid aortic valve between 1 January, 2019 and 31 December, 2020. Patients were classified as healthy weight or overweight/obese. Values for aortic dimensions as well as peak and mean aortic valve gradients were obtained from echocardiogram reports. About 251 patients were analysed. Demographics were similar between groups. When indexed to height, the aortic valve annulus (1.28 ± 0.14 vs. 1.34 ± 0.15, p = 0.001) and sinotubular junctions (1.44 ± 0.21 vs. 1.49 ± 0.24, p = 0.038) were larger in the overweight/obese group, with no differences in aortic root or ascending aorta sizes. The obese/overweight group had a higher peak aortic valve gradient (23.03 ± 1.64 mmHg vs. 16.17 ± 1.55 mmHg, p = 0.003) compared to the healthy weight group. Healthy weight patients did not have larger aortic dimensions compared to the overweight/obese patients. There was evidence of worsening aortic valve stenosis in overweight/obese patients compared to those at a healthy weight.

Quality of Life and Mental Development of Children with Acute Respiratory Infections Undergoing Hospital Treatment

Preserving the health of the younger generation is a priority multidisciplinary task. For a more comprehensive and individual assessment of the functioning of the child's body against the background of acute viral infections, a technique for determining the quality of life based on subjective perception was used, on the basis of which patients are classified into the first or second classes of quality of life. The paper examines the interrelationships of health, quality of life and mental development of preschool children with acute respiratory infections being treated in the pediatric department. Some children were found to have a reduced quality of life, not only in the group of children with acute respiratory infections, but also in the group of healthy children. There were significantly more patients with grade 2 quality of life in the main group. According to the results of correlation analysis using Spearmanʼs rank correlation coefficient, it was found that the quality of life is interrelated with psycho-emotional and the intellectual development of the child. Based on the study of the quality of life of patients in the pediatric department, it is possible to implement an individual approach to assessing the condition of the child as a whole and against the background of the disease, for high-quality further treatment and monitoring, for the organization of preventive measures.

Safety and efficacy of COVID-19 vaccines in children and adolescents with cancer.

Children with cancer have a higher morbidity and mortality due to COVID-19. Vaccination of children with cancer is important. In this study, we aimed to investigate the effectiveness and side effects of the COVID-19 vaccines in children and adolescents with cancer. Fifty-eight patients from four centers were included in the study. Antibodies to the SARS-CoV-2 spike protein levels were measured. Vaccine-related complaints were recorded. There were 33 male and 25 female patients. The mean age was 16.9±2.3 years. In 58.6% of cases, the diagnosis was hematological malignancies. Twenty patients were currently under treatment, while 38 had completed the treatment. Forty-eight patients received chemotherapy ± radiotherapy, 13 received immunotherapy, and 3 underwent stem cell transplantation. CoronoVac© and BNT162b2© vaccines were administered in 24% and 76%, respectively. The mean antibody level was lower in patients who received CoronaVac© than that of BNT162b2©, although the difference was not significant. The levels were within the protective limits in both groups. No significant difference was found in antibody levels according to diagnostic subgroups, treatment status, type of treatment, line of treatment, disease status and time between vaccines and measurement of antibody level. The most common side effects were pain at the injection site (37.9%) and malaise/weakness (17.2%), which were similar for both vaccines. Our study showed that both mRNA and inactivated vaccines elicit an immune response in children with cancer. However, the seroconversion rate is significantly higher in mRNA vaccines. Side effects were similar to those seen in healthy children.

Clinical characteristics and serum 25-hydroxyvitamin D levels in children with febrile seizures in China

The aim of this study was to evaluate the serum level of 25-hydroxyvitamin D (25(OH)D) in children with febrile seizures (FS) in Luzhou, Sichuan Province, China, and in particular its association with gender and age. This should inform possible strategies for supplementation with vitamin D, and hence for prevention of FS in the local pediatric population. The Febrile seizures group consisted of 747 children hospitalized with FS at the Southwest Medical University Affiliated Hospital from January 2020 to January 2024. The healthy control group was comprised of 750 children aged from 0 to 8 years who underwent health checkups during this period. The serum 25(OH)D level was analyzed in relation to gender and age to explore its association with FS. The median serum vitamin D level in the FS group (28.8 ng/mL; IQR 21.64, 33.64) was significantly lower than in the healthy control group (37.51 ng/mL; IQR 31.05, 37.51). The incidence of vitamin D deficiency in the FS group was 10.8%, which was significantly higher than in the healthy control group (P < 0.05). In addition, the serum vitamin D level in children with FS varied in different age groups, with significantly lower levels observed in older children (P < 0.05). ROC curve analysis revealed that a serum vitamin D level of 35.28 ng/mL showed 60.0% sensitivity and 84.7% specificity for predicting FS (P < 0.05). In this study cohort, the serum vitamin D level in children with FS was at the lower limit of the physiological range, and significantly lower than in healthy children. Furthermore, this level decreased with age in children with FS. Regular supplementation with vitamin D for 6 months after birth and outdoor sun exposure for more than 2 h per day can improve the serum vitamin D level in children with FS.

Lymphocyte phenotyping in untreated children patients with chronic allergic asthma

This study aimed to isolate and phenotype lymphocytes in untreated children patients with chronic allergic asthma. To reach such aim the study involved (25) patients from children (17 male and 9 female) whom their ages where between (3-10) years, in addition to (15) apparently healthy children (9 male and 6 female) in the same ages involved as control group. The data demonstrated that there was a significant increase in the mean percentages of T-lymphocytes (CD3+ cells) in the peripheral blood of patients (66.75±0.29)**, in comparison with control group (43.58±0.19), a significant increase in the mean percentages of T-helper lymphocytes (CD4+ cells) in the peripheral blood of patients (51.14±0.55), in comparison with control group (39.17±0.23) and the mean percentages of B-lymphocytes (CD20+ cells) was also increased significantly in the peripheral blood of patients (29.63±0.20) when it compared with the mean percentages of the same cells in control group (18.60±0.80). Besides a significant decrease in the mean percentages of T-suppressor lymphocytes (CD8+ cells) in the peripheral blood of patients (11.31±0.05), in comparison with control group (16.42±0.15). Finally the results of this study showed a significant increase in the mean percentages of the ratio of (CD4+ cells/CD8+ cells) in the peripheral blood of patients (55.34±0.41), in comparison with control group (31.25±0.09).

Characteristics of gut flora in children who go to bed early versus late

Investigating the characteristics of the gut flora in children who go to bed early versus late. The study sample consisted of 88 healthy children aged 2–14 years, with an equal number of boys and girls. The researchers collected faecal samples from all participants and sequenced the genome of their gut flora. Findings indicate that beta diversity was statistically significant at the genus level for both the early and late sleeper groups (P = 0.045). Furthermore, alpha diversity indicators, including Simpson’s index (P = 0.0011) and Shannon’s index (P = 0.0013), exhibited higher values at the genus level. The differences observed in terms of species diversity, abundance, and metabolic pathways offer potential avenues for implementing pharmacological interventions aimed at addressing sleep disorders in children.

7135 Changes in 11-oxygenated androgens in healthy prepubertal children from ages 4, 6, to 8 years: a prospective cohort study

Abstract Disclosure: Y. Lee: None. K. Lee: None. J. Kim: None. Y. Lim: None. J.I. Kim: None. B. Kim: None. Y. Hong: None. J. Song: None. C. Shin: None. Y. Lee: None. Background: 11-oxygenated androgens (11-oxyandrogens) have been recognized for their important role in adrenarche or androgen excess conditions. However, the trajectory during childhood remains understudied. Herein, we examined the longitudinal changes in 11-oxyandrogens among healthy prepubertal children. Methods: In an ongoing prospective cohort study, 172 prepubertal children with available blood samples at the ages of 4, 6, and 8 years were included. Serum concentrations of 14 adrenal androgens and precursors, including dehydroepiandrosterone (DHEA), DHEA-sulfate (DHEAS), androstenedione (A4), testosterone (T), 11β-hydroxyandrostenedione (11OHA4), 11-ketoandrostenedione (11KA4), 11β-hydroxytestosterone (11OHT), and 11-ketotestosterone (11KT) were assessed using liquid chromatography-tandem mass spectrometry. Repeated measures ANOVA was used to analyze the changes in adrenal androgens across age groups. Linear mixed model analysis was used to evaluate factors associated with adrenal hormone levels during 4 to 8 years of age. Results: Among 172 prepubertal children (107 boys, 65 girls), 11OHA4 was the predominant 11-oxyandrogen, exceeding concentrations of DHEA or A4. Serum 11KT level was higher than testosterone level from 6 years of age. Across age 4, 6, and 8 years, 11-oxyandrogens showed an increasing trend. Trajectories of 11OHA4, 11KA4, and 11KT significantly differed by sex (p for interaction &amp;lt; 0.05). Serum levels of 11OHA4 and 11KT consistently increased from 4, 6, to 8 years in both sexes, while 11OHT increased during 6-8 years (p &amp;lt; 0.05 for all). Serum 11KA4 levels increased during 4-6 years in boys and 6-8 years in girls (p &amp;lt; 0.05 for all). Girls showed higher levels of 11OHA4, 11KA4 at 4 years, 11OHT at 6 years, and 11KT at 4 and 6 years. Regarding classic adrenal androgens, DHEA, DHEAS, and A4 also exhibited increases in different patterns between sexes (p for interaction &amp;lt; 0.05) during 4-8 years. Girls had higher serum DHEA and A4 levels at 4 and 6 years, and DHEAS levels at 4 years (p &amp;lt; 0.05 for all). In boys, lower gestational age, lower birth weight z-score, and higher body mass index (BMI) z-score were correlated with elevated levels of DHEA and DHEA-S. Additionally, a higher BMI z-score was associated with increased levels of A4, 11OHT, and 11KT (p &amp;lt; 0.05 for all, multivariable models). In girls, a higher BMI z-score was linked to elevated DHEA levels, while a higher birth weight z-score was associated with increased A4 levels (p &amp;lt; 0.05 for all, multivariable models). Conclusion: In prepubertal children, 11-oxyandrogens showed increasing patterns similar to classic androgens, with sex-differences. Some 11-oxyandrogens were more predominant than classic androgens. This study suggests the importance of 11-oxyandrogens as biomarkers for zona reticularis development and adrenarche in prepubertal children. Presentation: 6/3/2024

7814 Non-fasting and Fasting Mixed Meal Tolerance Tests Equally Capture the Triglyceride Response in Participants With and Without MASLD

Abstract Disclosure: L.A. Gonzalez Ramirez: None. Z. He: None. J.N. Binongo: None. H.E. Huneault: None. A. Ramirez Tovar: None. J.A. Welsh: None. M.B. Vos: Consulting Fee; Self; Boehringer Ingelheim, Prosciento, Target Real World Solutions, Intercept Pharmaceuticals, INC, Novo Nordisk. Grant Recipient; Self; Target Real World Solutions. Mixed meal tolerance tests (MMTTs) are functional assessments that may identify metabolic dysregulation earlier than fasting tests during the progression to metabolic dysfunction-associated steatotic liver disease (MASLD). Although standards to conduct MMTTs in adults are readily available, the knowledge gap for children is quite large. Furthermore, conducting this type of study in children is challenging because they are less tolerant of long fasting intervals, long periods of testing, and early morning visits that interrupt school attendance. The objective of this study was to assess if conducting an MMTT in a non-fasting state will equally identify variance in the metabolic response of triglycerides (TG) between healthy children and those living with MASLD when compared to a 12-hour fasting MMTT. We collected blood samples every two hours in our 2-day randomized crossover feeding study. During each visit, 9 children with MASLD and 10 healthy controls aged 10 to 18 y underwent 1 standardized MMTT after a 12-hour overnight fasting (breakfast), followed by 2 standardized MMTTs in a non-fasting state (lunch and dinner). Participants were randomized to receive a glucose or fructose beverage at visit 1 and the other at visit 2. For uniformity, we only used data from the glucose beverage visit. We found that TG levels were statistically higher in the MASLD participants when compared to the non-MASLD group at each time point (p= 0.01). The maximum TG metabolic response peak for each MMTT was higher and more delayed in the MASLD group compared to the non-MASLD group. Within groups, postprandial TG level patterns were not significantly different when comparing breakfast, lunch, and dinner. Although TG levels varied throughout the day, the pattern after every meal was similar. These findings suggest that it is possible to identify differences in the TG metabolic response between children living with MASLD and healthy participants in the postprandial state. The results from our study also support the use of non-fasting MMTTs when examining TG metabolism in children, though further research to validate these findings is needed. Presentation: 6/1/2024

Health-related quality of life of toddlers during and after cancer treatment

BackgroundThere is a knowledge gap regarding health-related quality of life (HRQOL) in childhood cancer patients below 2 years of age. The aim of this study was therefore to compare HRQOL of young children during and after treatment for cancer, to healthy controls, and to investigate effects of biopsychosocial factors. ProcedureThe study is based on data from an online monitoring program. Parent-proxy reports of HRQOL in 205 children aged 12–24 months were compared to 108 healthy children. The parents filled out the TNO-AZL Preschool Quality of Life questionnaire for young children, which consists of 12 subscales, in addition to a parental distress thermometer. ResultsParticipants undergoing treatment had less favorable HRQOL than children after treatment for eight of the subscales. They also had less favorable HRQOL than healthy children for seven scales, while the only significant difference between children off treatment and healthy controls was for motor functioning. For ten subscales, there were significant relationships between biopsychosocial variables and HRQOL outcomes. Parental distress and treatment with immunotherapy were the variables most frequently associated with lower HRQOL. ConclusionsParental distress should be considered when monitoring young childhood cancer patients. Treatment with immunotherapy is likely to be a marker of disease severity and might represent other underlying factors affecting HRQOL. The association between immunotherapy and HRQOL therefore needs more research. While there was a significant difference depending on treatment status, it cannot be concluded that this represents an improvement in HRQOL after end of treatment.

TREC and KREC values in patients with congenital heart defects — neonatal screening data

Relevance. Children with congenital heart disease (CHD) are at high risk of infectious complications with unfavorable outcomes, which is associated with inadequate immune responses. TREC and KREC are recognized biomarkers of T- and B-cell lymphopoiesis.Objective of the study: to evaluate the number of circular DNA segments — TREC and KREC in children with congenital heart defects.Materials and methods. The study used data from neonatal screening for primary immunodeficiencies in the Stavropol region from January 1, 2023, to June 30, 2024. An analysis of TREC and KREC was conducted in 43 newborns with CHD compared to healthy infants.Results. The levels of TREC and KREC in children with CHD were lower than in healthy children. A correlation was established between TREC levels and absolute lymphopenia, as well as the development of infectious complications. A decrease in KREC was identified in infants with CHD and genetic syndromes. It was shown that a TREC level of less than 650 copies per 105 cells may be a predictor of the development of infectious complications in newborns with CHD.Conclusions. The reduction in the number of TREC and KREC copies in neonatal screening allows for the identification of children with CHD at high risk of infection, opening potential opportunities for preventive therapy.

7345 Study on the correlation between the levels of myokines and metabolic abnormality in obese children

Abstract Disclosure: L. Ouyang: None. M. Li: None. F. Yang: None. Objective: There are limited drug options to treat childhood obesity. Studies have found that myokines play an important role in the mechanism of exercise in the treatment of obesity. However, the correlation between myokines and metabolic abnormalities in obese children is still unclear. The purpose of this study is to analyze the correlation between the levels of myokines and metabolic abnormalities in obese children to provide the basis for the future application of myokines in childhood obesity. Methods: 41 obese children who visited the pediatric clinic of our hospital from March to October 2022 were enrolled, and 11 healthy non-obese children who had a regular physical examination in the pediatric clinic during the same period were enrolled. Basic information, anthropological indicators and biochemical indicators were collected. Human myokine magnetic bead panel was used to detect serum myokine levels including irisin, FGF21, BDNF, LIF, MSTN, IL 6, and IL 15. Difference analysis, rank sum test and Spearman analysis were used to conduct the correlation between the levels of myokines and metabolic abnormalities in obese children. Results: We found that the BMI-SDS and body fat rate of obese children with abnormal metabolism was higher than those with normal metabolism status(p&amp;lt;0.05). Children with abnormal glucose metabolism had an increased risk of abnormal lipid metabolism and hyperuricemia(p &amp;lt; 0.05), and children with abnormal lipid metabolism had an increased risk of hyperuricemia and hypertension(p&amp;lt;0.05). The obese children with abnormal glucose and lipid metabolism had an increased risk of abnormal purine metabolism. The levels of leukemia inhibitory factor and brain-derived neurotrophic factor in obese children were significantly higher than those in non-obese children (p&amp;lt;0.05), and the level of fibroblast growth factor 21 in obese children with abnormal metabolism was significantly higher than that in children with normal metabolism (p&amp;lt;0.05), respectively. There was a significant positive correlation between BMI-SDS and the levels of irisin, leukemia inhibitory factor and brain-derived neurotrophic factor (r=0.361, 0.368, 0.343, p=0.009, 0.007, 0.013, respectively). There was a significant positive correlation between irisin, BDNF, LIF, MSTN and IL 6, and between FGF21 and irisin, MSTN, IL 6 in children (p &amp;lt; 0.05). Conclusions: With the increase in BMI, the risk of metabolic abnormalities in obese children increases. Obese children with one kind of metabolic abnormality are prone to merge with other metabolic abnormalities. The levels of leukemia inhibitory factor, fibroblast growth factor 21, irisin, brain-derived neurotrophic factor, and other myokine in obese children and obese children with abnormal metabolism are different from those in non-obese children and obese children with normal metabolism respectively, and there is interaction between myokines. Presentation: 6/1/2024

6703 Age-Related Reference Intervals for 11-Oxygenated Androgens in Children

Abstract Disclosure: L. IJsselstijn: None. R. Zellerer: None. R. Oude Engberink: None. B. van Zelst: None. S.A. van den Berg: None. Context: 11-Oxygenated androgens have emerged as major components of several disorders of androgen excess. Levels of 11-keto androstenedione (11KA4), 11β-hydroxy androstenedione (11OHA4), 11-keto testosterone (11KT) and 11β-hydroxy testosterone (11OHT) have been mainly studied in adults and in relation to various disorders. Although it is known that 11-oxygenated androgens are pivotal in adrenarche and pubarche, well established reference intervals for children are lacking. Objective: To establish age-related reference intervals for 11-oxygenated androgens in children. Methods: Serum samples were collected from apparently healthy children with ages ranged 0-18 years (n=186, 108 boys and 78 girls). Concentrations of 11KA4, 11OHA4, 11KT and 11OHT were determined by isotope dilution mass spectrometry. Before measurement the serum was deproteinized and purified by solid-phase extraction. The lower limit of quantitation for 11KA4, 11KT and 11OHT were 3.0 ng/dL (0.10 nmol/L), and for 11OHA4 9.1 ng/dL (0.30 nmol/L). Results: For 11KA4 a difference between boys and girls was observed until the age of 6. In girls 11KA4 was undetectable where in boys concentration up to 9.61 ng/dL (0.32 nmol/L) were measured. From the age of 6 an increase of 11KA4 was observed with increasing age. From the age of 13 concentrations stabilized at 4.21-28.5 ng/dL (0.14-0.95 nmol/L) without a difference between boys and girls. 11OHA4 was detectable at all ages and showed no differences between boys and girls, except for an apparently slightly higher concentration for boys until the age of 4. The concentration 11OHA4 increased with age in an almost linear way. At the age of 18 concentrations were observed of 60.5- 296.4 ng/dL (2.00-9.80 nmol/L). For 11KT a similar relation between the concentration and age was observed until the age of 14. At the age of 14 a plateau seems to be reached at concentrations of 14.5-74.4 ng/dL (0.48-2.46 nmol/L), which slightly decreased from the age of 17. 11OHT was undetectable in both boys and girls up to the age of 9 years. After the age of 9 the concentration range increased by age up to 30.4 ng/dL (1.00 nmol/L). From the age of 12 11OHT was detectable in all girl samples, where as in boys samples with undetectable concentrations were present at all ages. Conclusions: Age-related reference ranges were established for 11KA4, 11OHA4, 11KT and 11OHT. Overall no differences were observed between boys and girls, except for 11KA4 at a young age. The concentrations measured at the age of 18 correspond to the adult values known from literature for all 4 11-oxygenated androgens. Presentation: 6/1/2024

First diagnosis of severe coarctation of the aorta necessitation percutaneous intervention during pregnancy, a case report

Abstract Background Coarctation of the aorta (CoA) is a common congenital heart defect that affects about 3-4 in every 10,000 live births. Despite clear signs on clinical examination, the diagnosis is sometimes not made until adulthood. An increasing number of patients with CoA are reaching child-bearing age. Unrepaired CoA or severe recurrent stenosis during pregnancy is a significant concern, as it poses a high risk of maternal and foetal complications and even death. Case summary A 21-year-old women was referred to the cardiology department at 17 weeks’ gestation for management of arterial hypertension and unexplained systolic murmur. She had been diagnosed with hypertension elsewhere the year before presentation, but unfortunately this remained unexplored. She had been started on labetalol early in the pregnancy. Clinical examination showed a loud systolic heart murmur extending from parasternal to subclavicular and scapular areas. Pulses in the lower extremities were very weak and blood pressure was slightly elevated with a significant gradient between the upper and lower extremities. Echocardiography showed remarkable absence of pulsatile flow in the abdominal aorta and narrowing just distal to the subclavian artery with typical diastolic tail pattern on suprasternal imaging. Cardiac magnetic resonance confirmed the presence of a severe coarctation distal to the subclavian artery and presence of multiple collaterals allocating this patient in the extremely high-risk category with a risk of up to up 40-100% of maternal cardiac event during pregnancy. An extensive multidisciplinary team meeting was convened. After initial medical optimisation, increased claudication and signs of placental hypoperfusion necessitated an endovascular procedure under general anaesthesia at 23 weeks’ gestation. A Bentley BeGraft Plus stent (16x38 mm) was successfully placed. Postoperative ultrasound showed biphasic placental perfusion and normalisation of blood pressure and ankle-brachial indices. At 36 weeks’ gestation, the patient gave birth to a healthy child. Conclusion CoA should be considered in any young patient with arterial hypertension. Altered maternal haemodynamics during pregnancy resulted in severe symptomatic CoA and reduced placental flow necessitating percutaneous intervention during pregnancy. A multidisciplinary pregnancy heart team is essential for optimal treatment management in these high-risk patients.

6568 A Novel Case of Liddle Syndrome in Siblings: Insights into a Rare Genetic Mutation

Abstract Disclosure: A.B. Humrickhouse: None. M. Peltsverger: None. Introduction: Liddle syndrome (LS) is an inherited form of hypertension caused by a mutation in the epithelialsodium channel (ENaC). This condition is characterized by hypertension (HTN), hypokalemia,hyporeninemia, and metabolic alkalosis, although clinical presentation can vary. Asymptomaticearly-onset HTN is common, and if left undiagnosed, it can lead to advanced renal disease,cardiovascular complications, stroke, and even sudden death. Treatment typically involves theadministration of ENaC blockers such as amiloride and triamterene. We present a novel case ofLS in two siblings resulting from a missense mutation in the SCNN1B gene. Case Presentation: The patient is a 24-year-old Hispanic female who presented for evaluation of poorly controlledHTN and severe hypokalemia of 2.9 mmol/L during the first trimester of pregnancy. She wasdiagnosed with HTN at age eighteen. The family history was notable for resistant HTN in herfather and younger brother. Biochemical evaluation revealed normal plasma-free metanephrinesand 24-hour urine-free cortisol. Low plasma renin activity (0.530 ng/mL/hr) and aldosterone(&amp;lt;1.0 ng/dL) were detected on multiple tests. The urine-free cortisol-to-cortisone ratio was &amp;lt;0.5,excluding the diagnosis of apparent mineralocorticoid excess syndrome. The diagnosis of LS wasconfirmed by genetic analysis, which identified a novel heterozygous mutation, c.1859 A&amp;gt;G(Tyr620Cys), in the SCNN1B gene, which had not been reported in the literature to date. Thesame mutation was identified in her brother. The patient was treated with a high dose ofamiloride (15 mg daily), which controlled her HTN and hypokalemia throughout her pregnancy. Discussion: LS is an autosomal dominant disorder with genetic heterogeneity. It is characterized by HTN,low plasma aldosterone and renin activity, and increased potassium excretion. This leads tohypokalemia and metabolic alkalosis. LS is caused by germline mutations found in theSCNN1A, SCNN1B, and SCNN1G genes, which encode the alpha, beta, and gamma subunits ofthe ENaC channel. These mutations lead to enhanced sodium reabsorption in the kidneys,independent of aldosterone. Our patient was diagnosed with hypertension at the age of 18.However, the diagnosis of LS was not made until six years later when she presented during herfirst trimester of pregnancy. Treatment with amiloride effectively controlled her hypertension andhypokalemia, which allowed her to deliver a healthy child. Amiloride was also prescribed for thepatient's brother. A combination of biochemical analysis and genetic testing enables a betterunderstanding of the various clinical presentations, ensuring appropriate treatment andprevention of end-organ damage. Genetic counseling is essential for families affected by LS. Presentation: 6/1/2024

Balancing nutrition and budgets: Socio-ecological impacts on nutritional environments of families with low incomes

BackgroundResearch on childhood nutritional environments, which are predictive of a variety of child and family health and wellbeing outcomes, has primarily focused on parenting behaviors and the home food environment. However, broader social and community factors play an important role in shaping nutritional environments during early childhood. Food security is a key example that is closely linked to nutrition-related health disparities, which can arise from constrained financial resources and material deprivations. Understanding parent and caregiver lived experiences related to social and community components of family nutritional environments in the context of economic constraints could help to support multi-level nutritional practices that are optimal for healthy child development. MethodSemi-structured in-depth interviews were conducted using a narrative approach with parents (n = 21) of pre-school aged children living near or below the poverty line. Participants were recruited from social and community service sites pertinent to maternal and child health in Philadelphia where health disparities are highly concentrated. Analysis was conducted using MAX QDA analytic software following a systematic approach for thematic analysis in narrative research. ResultsThree themes describe the feeding and eating environments in the home and community context: 1) Food Procurement and Meal Planning; 2) Family Togetherness; 3) Creativity, Variety, and Flexibility. Creative decision making and food procurement strategies responsive to child preferences were balanced by shopping to save and visiting multiple stores for the best foods and savings. Logistical (e.g., transportation, childcare) and emotional supports facilitated food shopping habits and routines, while lack of trusted childcare, reliable transportation, and social support barriers challenged feeding and eating decisions. ConclusionStrategies for improving cost and time-efficiencies could help support health-promoting nutritional environments of families with food insecurity. Building social and community capacity and facilitating connections to practical supports that include trustworthy childcare and peer support may be particularly important for this population.