Changes In Pediatric Patients Research Articles

Investigation of brain diffusion changes in children with primary nephrotic syndrome

AimPrimary nephrotic syndrome, which occurs with the deterioration of kidney functions, can subsequently affect the brain with systemic immune activation, vasculopathy and ischemia. The main aim of this study was to investigate the effectiveness of Apparent Diffusion Coefficient (ADC) and Diffusion Weighted Imaging (DWI) in identifying and detecting brain changes in pediatric patients with primary nephrotic syndrome (PNS). Materials and MethodsThe study included 24 pediatric patients with PNS and 60 healthy children as a control group. The Apparent Diffusion Coefficient values of caudate nucleus, frontal cortex, thalamus, lentiform nucleus, anterior crus and posterior crus of the internal capsule, frontal and occipital white matter were measured quantitatively. ResultsThe ADC values of thalamus, occipital white matter, caudate nucleus and frontal cortex in the PNS group were significantly lower than in the control group (p<0.05 for all). No statistically significant difference was detected between the groups in respect of other brain locations. ConclusionsSystemic effects and possible complications of primary nephrotic syndrome may lead to diffusion changes in brain tissue. The decrease in ADC values in patients with PNS may be explained by decreased cerebral perfusion due to cerebral vasoconstriction and vasculopathy.

Cortical and subcortical structural changes in pediatric patients with infratentorial tumors.

This study aimed to detect supratentorial cortical and subcortical morphological changes in pediatric patients with infratentorial tumors. The study included 24 patients aged 4-18 years who were diagnosed with primary infratentorial tumors and 41 age- and gender-matched healthy controls. Synthetic magnetization-prepared rapid gradient echo images of brain magnetic resonance imaging were generated using deep learning algorithms applied to T2-axial images. The cortical thickness, surface area, volume, and local gyrification index (LGI), as well as subcortical gray matter volumes, were automatically calculated. Surface-based morphometry parameters for the patient and control groups were compared using the general linear model, and volumes between subcortical structures were compared using the t-test and Mann-Whitney U test. In the patient group, cortical thinning was observed in the left supramarginal, and cortical thickening was observed in the left caudal middle frontal (CMF), left fusiform, left lateral orbitofrontal, left lingual gyrus, right CMF, right posterior cingulate, and right superior frontal (P < 0.050). The patient group showed a volume reduction in the pars triangularis, paracentral, precentral, and supramarginal gyri of the left hemisphere (P < 0.05). A decreased surface area was observed in the bilateral superior frontal and cingulate gyri (P < 0.05). The patient group exhibited a decreased LGI in the right precentral and superior temporal gyri, left supramarginal, and posterior cingulate gyri and showed an increased volume in the bilateral caudate nucleus and hippocampus, while a volume reduction was observed in the bilateral putamen, pallidum, and amygdala (P < 0.05). The ventricular volume and tumor volume showed a positive correlation with the cortical thickness in the bilateral CMF while demonstrating a negative correlation with areas exhibiting a decreased LGI (P < 0.05). Posterior fossa tumors lead to widespread morphological changes in cortical structures, with the most prominent pattern being hypogyria. This study illuminates the neurological impacts of infratentorial tumors in children, providing a foundation for future therapeutic strategies aimed at mitigating these adverse cortical and subcortical changes and improving patient outcomes.

Non-IgE-mediated drug-induced hypersensitivity reactions in pediatrics.

Despite their prevalence and potential severity, non-IgE-mediated drug-induced hypersensitivity reactions (DHRs) are under-researched and poorly defined, particularly in children. Presentations range from mild cutaneous reactions to severe systemic diseases, with pathophysiological mechanisms and reliable diagnostic markers not well established. The lack of validated tests often leads to permanent drug restrictions, reliance on second-line drugs, and increased costs. Focusing on recent advancements and areas needing further research, this review aims to enhance children's recognition, diagnosis, and management of non-IgE-mediated DHRs. Recent studies have enhanced the understanding of immediate and delayed non-IgE-mediated drug reactions. Key findings include the Mas-related G protein-coupled receptor X2 in mast cells and the identification of HLA alleles linked to severe cutaneous adverse reactions, such as Stevens-Johnson syndrome and toxic epidermal necrolysis. Improved diagnostic techniques, including skin testing, show promise in identifying immediate and delayed non-IgE DHRs. Additionally, research highlights the impact of cofactors, drug metabolites, and co-infections on these DHRs and explores potential biomarkers for predicting reaction severity. Non-IgE-mediated DHRs are a significant cause of morbidity and treatment changes in pediatric patients. Recent research underscores their clinical presentations and mechanisms, paving the way for more precise diagnostic and therapeutic strategies to improve patient outcomes.

Electrocardiogram Abnormalities in Children With Lyme Arthritis.

Classically, Lyme disease follows a staged illness pattern with carditis occurring in early disseminated disease and arthritis in late-stage disease. A more comprehensive understanding of Lyme suggests that clinical stages may intersect. Little is known regarding the overlap of electrocardiogram (ECG) abnormalities in children with Lyme arthritis. This study aimed to estimate the prevalence of ECG changes in pediatric patients presenting with Lyme arthritis. In this retrospective, cross-sectional study was conducted at a tertiary care children's hospital in a Lyme endemic area; patients were identified based on Lyme testing performed from January 2012 to August 2022. Children diagnosed with Lyme arthritis by 2-tiered serology with ECGs obtained within 2 days of antibiotic initiation were included. A study cardiologist reviewed all ECGs for evidence of carditis defined as atrioventricular block, ST-T wave changes, QTc interval prolongation, accelerated junctional rhythm or right bundle branch block. Two hundred thirty-three patients were diagnosed with Lyme arthritis; 90 (38.6%) had ECGs completed. Five patients (5.6%) had ECG abnormalities: 3 were diagnosed with first-degree atrioventricular block, 1 with QTc prolongation, and 1 with ST-T wave changes. No clinical or laboratory features in patients with Lyme arthritis were associated with an increased likelihood of having an abnormal ECG. All patients with ECG abnormalities were treated with oral antibiotics, and none had clinically significant cardiac disease. ECG abnormalities in children with Lyme arthritis rarely occur and, when present, are not reflective of clinically significant cardiac disease. These results do not support routine screening ECGs on asymptomatic pediatric patients with Lyme arthritis.

Machine learning-enhanced HRCT analysis for diagnosis and severity assessment in pediatric asthma.

Chest high-resolution computed tomography (HRCT) is conditionally recommended to rule out conditions that mimic or coexist with severe asthma in children. However, it may provide valuable insights into identifying structural airway changes in pediatric patients. This study aims to develop a machine learning-based chest HRCT image analysis model to aid pediatric pulmonologists in identifying features of severe asthma. This retrospective case-control study compared children with severe asthma (as defined by ERS/ATS guidelines) to age- and sex-matched controls without asthma, using chest HRCT scans for detailed imaging analysis. Statistical analysis included classification trees, random forests, and conventional ROC analysis to identify the most significant imaging features that mark severe asthma from controls. Chest HRCT scans differentiated children with severe asthma from controls. Compared to controls (n = 21, mean age 11.4 years), children with severe asthma (n = 20, mean age 10.4 years) showed significantly greater bronchial thickening (BT) scores (p < 0.001), airway wall thickness percentage (AWT%, p < 0.001), bronchiectasis grading (BG) and bronchiectasis severity (BS) scores (p = 0.016), mucus plugging, and centrilobular emphysema (p = 0.009). Using AWT% as the predictor in conventional ROC analysis, an AWT% ≥ 38.6 emerged as the optimal classifier for discriminating severe asthmatics from controls, with 95% sensitivity, specificity, and overall accuracy. Our study demonstrates the potential of machine learning-based analysis of chest HRCT scans to accurately identify features associated with severe asthma in children, enhancing diagnostic evaluation and contributing to the development of more targeted treatment approaches.

Assessment of insulin dose changes in pediatric patients with type 1 diabetes mellitus starting on continuous subcutaneous insulin infusion

ObjectiveTo assess change in total daily dose (TDD) of insulin following a switch from subcutaneous (SC) injections to continuous subcutaneous insulin infusion (CSII) in pediatric patients with type 1 diabetes (T1D). Secondary objectives were to determine the change in %basal insulin, insulin to carbohydrate (I:C) ratios, insulin sensitivity factor (ISF), and HbA1c/IDAA1c. MethodsA retrospective chart review of patients < 18 years of age who transitioned from SC to CSII at the Alberta Children’s Hospital (Calgary, Alberta, Canada) between January 2019 and March 2022. ResultsThere was an increase of 0.04 units/kg/day in TDD from baseline vs 1–3 months later (p = 0.04, 95 % confidence interval (CI) [0.002, 0.072]). When stratified by age, a similar increase in TDD was observed in age 5–12 years only (p = 0.05, 95 % CI [0.0006, 0.8236]). There was a decrease in overall %basal insulin by 3 (44 % of TDD at baseline vs 41 % of TDD on CSII). (p = 0.02, 95 % CI [−5.5, −0.4]). No strengthening was seen in I:C ratios from baseline vs 1–3 months later. There was a significant strengthening of I:C ratios at all meals in the basal bolus group from 1–3 weeks to 1–3 months post-CSII; overall strengthening of ISF at both time points; and an overall HbA1c decrease −0.30 (p < 0.0001, CI [−0.45, −0.15]). Each extra year with diabetes was associated with a decrease in HbA1c by 0.07 % (p = 0.006). ConclusionsTDD of insulin was not found to be decreased post CSII initiation and patient characteristics should be considered when changing from SC to CSII. HbA1c was significantly improved post CSII.

Brochure intervention to manage bothersome taste changes in pediatric patients receiving cancer therapy.

Primary objective was to determine if a patient informational brochure describing potentially useful strategies could help manage specific taste changes. Secondary objective was to describe the specific strategies used and whether the strategies were perceived as being helpful. This single-center study included pediatric patients with cancer or hematopoietic cell transplant recipients receiving active treatment who experienced bothersome taste changes in the last month. Participants participated in baseline and follow-up interviews conducted 14-21 days apart. A brochure that listed 16 potentially helpful strategies was provided at baseline. At follow-up, we asked about brochure use and whether it helped. At both interviews, we asked about experienced taste changes, strategies used, and whether strategy helped. Of 100 enrolled participants, different (87%) and bad (72%) taste were most common at baseline. Following the brochure intervention, statistically significant reductions were observed in food tasting different, bad, bland, bitter, sour, and metallic. For most strategies, the proportion of patients who used specific strategies or found them helpful was not significantly different between baseline and follow-up. However, "eating foods you like" was considered helpful in significantly more participants who used the strategy in follow-up (72 out of 89, 80.9%) compared with baseline (55 out of 95, 57.9%; p=.008). Between visits, 81.2% looked at the brochure. Among participants, 53.1% found the brochure helpful, very helpful, or extremely helpful. A brochure that offered strategies to manage changes in taste helped participants cope with them. Further research should evaluate the brochure using randomized and multicenter trials.

Comparison of different induction techniques on hemodynamic changes in pediatric patients undergoing infra umbilical surgeries using laryngeal mask airway

Background: Post-induction hypotension associated with various agents is well-known in adults undergoing general anesthesia for various procedures. However, this phenomenon has not been addressed well in pediatric patients. We studied the incidence of postinduction hypotension in patients 1–5 years using inhalational induction with sevoflurane, intravenous induction with propofol, and co-induction with sevoflurane plus propofol. Aims and Objectives: To compare three different induction techniques in children 1–5 years undergoing various infra-umbilical surgeries under general anesthesia with laryngeal mask airway using sevoflurane induction, propofol induction, and sevoflurane plus propofol co-induction. Materials and Methods: Out of 150 patients observed in this study, 50 patients (group S) received inhalational induction with sevoflurane, 50 (group P) patients received intravenous induction with propofol, and 50 (group SP) patients received co-induction with propofol and sevoflurane. Results: Intravenous induction with propofol resulted in maximum hemodynamic changes Inhalational induction was responsible for significant changes in heart rate whereas co-induction with propofol (1–1.5 mg/kg) and sevoflurane 5% yielded minimum hemodynamic variations with least adverse effects. Conclusion: We conclude that co-induction with propofol (1–1.5 mg/kg) plus Sevoflurane 5% provided better hemodynamic stability with the least adverse effects.

Sleep effects of Ketogenic diet in pediatric patients with migraine: Preliminary data of a prospective study

Objective/backgroundKetogenic dietary therapies’ effects on sleep have been poorly investigated up to date. Preliminary results of a prospective study aimed at evaluating possible sleep changes in pediatric patients with migraine treated with classic ketogenic diet are presented. Patients/methodsIncluded patients were aged 14-18 years and had a diagnosis of chronic migraine. A customized classic ketogenic diet was drawn up for all patients and all participants underwent neurological, nutritional and subjective and objective sleep assessment at baseline and after three months of follow-up (standardized sleep questionnaires, polysomnography, actigraphy). ResultsThe majority of patients reported an improvement in migraine symptoms and quality of life. As far as sleep effects, a possible sleep stabilization was evidenced according to actigraphic data, and polysomnographic data showed a slight increase in total sleep time and sleep efficiency together with a reduction in waking time during night and a trend of NREM stage 1 decrease and REM increase. ConclusionsFuture analyses on a broader population are needed to shed light on the ketogenic dietary therapies’ effects on sleep and future research should be devoted to identify influence of possible individual and diet characteristics, and biochemical related changes.

Laboratory and Bleeding Frequency Changes in Pediatric Patients after Transitioning from Factor VIII to Emicizumab Prophylaxis -Real World Evidence in Patients with Severe Hemophilia a

Background: Emicizumab, a monoclonal antibody which functions as a bypassing agent by bridging activated factor IX and factor X in the absence of normal factor VIII levels, has recently been approved for routine prophylaxis against bleeding episodes in both adults and pediatric patients with hemophilia A. While complete blood counts are typically normal in patients living with hemophilia, it is generally accepted that some patients with unusually heavy bleeding or persistently long bleeds can have low hemoglobin. This anemia may be more common as a previous study revealed that patients with hemophilia had lower that average values for hemoglobin cross all ages and degrees of severity as evidenced by 31% of patients with values less than the third percentile, 46% less that then tenth percentile, and 83% less than the mean value. Real world impact on hemogram in pediatric patients who have been switched to Emicizumab has not been reported in the past after the medication was approved by FDA for hemophilia management. Methods: We performed a retrospective chart review of a cohort of 12 male pediatric patients with hemophilia A who were transitioned from factor VIII to emicizumab prophylaxis between May 2019 and May 2022 to determine changes in laboratory values and bleeding frequencies. Results: Hematologic metricies in severe hemophilia patients pre and post emicizumab switch :Complete blood counts before emicizumab transition revealed a mean hemoglobin 12.8 g/dL (range 10.4-15.7 g/dL), MCV 80.8 fL (range 67.2-99.1 fL), RDW 13.8% (range 11.8-16.6%), and platelets 297 bil/L (range 166-490 bil/L). Following emicizumab initiation, follow up blood counts demonstrated mean hemoglobin 14.8 g/dL (range 12.0-17.1 g/dL), MCV 83.3 fL (range 77.2-95.4 fL), RDW 12.9% (range 12.0-13.9%), and platelets 303 bil/L (range 199-514 bil/L). Mean hemoglobin was noted to have a significant increase after emicizumab was started (P&amp;lt;0.001). Both minor and breakthrough bleeding frequencies were noted to decrease in all patients. Bleeding Frequency and Overall Satisfaction : The patient /caregiver input to assess changes in bleeding frequency was obtained, after stable treatment with emicizumab was tolerated for at least 6 months. The bleeding frequencies of our cohort of patients on FVIII prophylaxis had a wide range with minor bleed frequencies (defined as any joint, soft tissue or other hemorrhage not requiring additional FVIII administration) occurring as often as daily to once yearly. Breakthrough bleeds that required additional episodic FVIII administration occurred 2-3x/monthly to once yearly. Following transition to emicizumab, patients had a reported an overall decrease in bleeding frequencies with minor bleeds averaging from 2x/month previously to never. 3 of these patients had breakthrough bleeding episodes requiring FVIII with 2 episodes attributed to trauma. Overall, patients' parents were asked to give a subjective verbal rating from 1 to 10 of their experience with emicizumab with 1 being worst and 10 being best. The mean verbal rating of our cohort of patients was 9.3. 11 of 12 patients remain of emicizumab prophylaxis as of May 2023 with one patient returning to FVIII prophylaxis due to pain at injection site. Conclusion: An increase in mean hemoglobin concentration in conjunction with reported decreased bleeding frequencies may suggest emicizumab facilitates an overall reduction in prolonged bleeding episodes. At our center, hemophilia A patients transitioned from factor VIII to emicizumab prophylaxis demonstrated a statistically significant increase in hemoglobin concentrations. This data combined with a notable reported decrease in minor and breakthrough bleeding frequencies in all patients suggests an overall reduction in prolonged bleeding episodes. Patients and their families appear to be very satisfied with emicizumab prophylaxis which may promote changes in the standard of care for hemophilia A patients.

A Prospective Study of Sensory Changes in Pediatric Patients After Minimally Invasive Repair of Pectus Excavatum With Cryoablation

BackgroundCryoablation during minimally invasive repair for pectus excavatum (MIRPE) reduces opioid use and hospital length of stay. Skin hypoesthesia of the chest wall also occurs. This study sought to determine the frequency, onset, duration, and location of sensory changes and neuropathic pain after cryoablation. MethodsA prospective study was conducted on patients aged ≤21 years undergoing MIRPE with cryoablation of T3 to T7 dermatomes bilaterally for 120 s at a single institution between March 2021 to December 2022. Patients underwent sensory testing of the chest wall and neuropathic pain surveys (S-LANSS) preoperatively and then postoperatively for 6 months. Incidence and duration of hypoesthesia and neuropathic pain were evaluated. ResultsOf 61 patients enrolled in the study, 45 completed evaluations at six months postoperatively. All patients had skin hypoesthesia on postoperative day (POD)1. The mean percentage of the treated anterior chest wall surface area (TACWSA) with hypoesthesia to cold stimulus was 52% (±29.3) on POD 0 and 55% (±19.7) on POD 1. Sensation returned over time, with hypoesthesia affecting 11.1% (±15.5) TACWSA at 6 months. At study completion 58% of patients (26/45) had complete return of sensation; hypoesthesia was found at: 1 dermatome 13% (2/45), 2 dermatomes 22% (11/45), and 3 dermatomes 4% (2/45). Neuropathic pain (S-LANSS ≥12) was documented in 16% (9/55) of patients at hospital discharge but decreased to 6.7% of patients at 6 months. ConclusionOnset of skin hypoesthesia after cryoablation occurred on POD0 and affected 52% of the TACWSA. All patients experienced return of sensation to varying degrees, with 58% experiencing normal sensation in all dermatomes by 6 months. The etiology of persistent hypoesthesia to select dermatomes is unknown but may be related to operative technique or cryoablation. Chronic neuropathic pain is uncommon. Level of EvidenceII. Type of StudyPrognosis Study.

The impact of the COVID-19 pandemic on parents seeking dental care for their children

Many changes in routine dental care were implemented to protect dentists and patients during the COVID-19 pandemic. Pediatric dentists face unique challenges in dealing with parental fears and behavioral changes in pediatric patients. Despite the meticulous infection control protocols that dental offices have implemented during the pandemic, they experienced significant reductions in routine recall. The aim of this study is to assess the impact of the SARS-CoV-2 pandemic on parents seeking dental care for their children at the Temple Kornberg School of Dentistry (TUKSoD). Parents who currently have their children treated at the TUKSoD were invited to participate in a questionnaire. One hundred and six parents completed the survey anonymously on paper. The survey focused on parents’ attitude changes towards dental care for their children post-pandemic. Bivariate analysis followed by a Two-way ANOVA test was used to calculate the association between the effect of the COVID-19 pandemic on parents of pediatric patients’ decision to seek dental care versus the age of the child. Our results showed that improved dental protocols increased the likelihood of parents bringing their children to the dentist during the COVID pandemic (p &lt; 0.001). The results help pediatric dentists to build trust among their patients during the pandemic. It will also help policymakers understand the pediatric patients’ parents’ concerns and assess their current safety measures. Ultimately, this study will give practitioners more insight into how to handle outbreaks of new COVID-19 variants that may present in the future.

Incidence and Related Factors of L-asparaginase-induced Hypersensitivity Reactions Requiring Treatment Changes in Pediatric Patients with Acute Lymphoblastic Leukemia

Incidence and Related Factors of L-asparaginase-induced Hypersensitivity Reactions Requiring Treatment Changes in Pediatric Patients with Acute Lymphoblastic Leukemia

Widefield Fluorescein Angiography Findings in Pediatric Patients with X-Linked Retinoschisis

To evaluate the retinal vasculature in pediatric patients with X-linked retinoschisis (XLRS). Retrospective consecutive case series. Pediatric patients with a diagnosis of XLRS who had undergone widefield fluorescein angiography (FA). The electronic medical records of pediatric patients with XLRS at a tertiary referral eye center were reviewed from January 2015 to December 2021. Fluorescein angiography images were reviewed for anomalies of the retinal vasculature. Vascular anomalies on FA were recorded, including capillary dropout/ischemia, terminal supernumerary vessels, vascular leakage, abnormal vascular loops, straightening of vessels, aberrant circumferential vessels, and neovascularization. In total, 29 eyes of 15 patients were included in the study (1 patient had a phthisical eye). On FA, the most common findings were capillary dropout/ischemia (21 of 29 eyes, 72.4%), terminal supernumerary vessels (21 eyes, 72.4%), abnormal vascular loops (20 eyes, 69%), and vascular leakage (17 eyes, 58.6%). Of the 17 eyes with leakage, the most posterior zone of involvement was zone 1 in 11 eyes (64.7%) and zone 2 in 6 eyes (35.3%). All eyes demonstrated ≥ 1 vascular anomaly on FA. Among the 29 eyes, 23 (79.3%) demonstrated peripheral bullous schisis or retinal detachment (RD) with a mean of 5.6 clock hours of involvement. The presence of either RD or bullous retinal schisis was associated with the incidence of capillary dropout (91.3% in schisis/RD eyes vs. 0% in nonschisis/RD eyes, P < 0.001). Among those with RD or bullous schisis, a higher degree of involvement correlated with more severe capillary dropout (Pearson 0.49, P=0.025). The present study demonstrates consistent vascular changes in pediatric patients with XLRS using widefield FA. Although the presence of capillary ischemia was associated with the severity of bullous schisis or RD, other vascular anomalies were observed in patients both with and without peripheral schisis. Although further research is needed to understand the etiology of these vascular anomalies, FA should be considered in the evaluation of these patients. Proprietary or commercial disclosure may be found after the references.

P322 Mycobiome signature in pediatric patients with Inflammatory Bowel Disease

Abstract Background Inflammatory bowel disease (IBD) encompasses dysregulation of mucosal immunity involving multiple factors including diet, medication, family history, and the gut microbiome. The symptoms of Pediatric IBD are different from an adult-onset disease. Based on the clinical conditions, IBD can be classified into three categories: Crohn’s disease (CD), Ulcerative colitis (UC), and IBD-unclassified (IBDU). The gut bacteriome alteration is widely reported in IBD. But the gut mycobiome link to the disease remains sparse. This study compared mycobiome profile changes in pediatric patients with IBD and healthy controls. Methods Stool samples were collected from patients with CD (n = 110), UC (n=64), IBDU (n=23), and healthy control subjects (n = 42). ITS-2 (Internal Transcribed Spacer) libraries were sequenced and analyzed using the QIIME pipeline to assess the gut mycobiome composition. Lefse analysis was performed to identify the signature members among the study groups. Results Ascomycota and Basidiomycota were the most common phyla among the study groups. Saccharomyces cerevisiae and Candida albicans were the most abundant species in the gut. Patients with IBDU and CD had significantly lower alpha and beta diversities compared to the controls. Conclusion Pediatric IBD is associated with reduced alpha and beta gut fungal microbiome diversity. Specific Candida taxa were found to be increased in abundance in the IBD samples. These results emphasize the potential importance of fungal microbiota signatures as biomarkers of pediatric IBD, supporting their possible role in disease pathogenesis.

Lipid profile peculiarities and matrix Gla protein concentration in Ukrainian pediatric patients with heterozygous familial hypercholesterolemia

Atherosclerotic changes in the vascular walls begin early in childhood, especially in association with familial hypercholesterolemia (FH). The process may be subclinical, which nevertheless requires therapeutic and preventive measures. Purpose - to evaluate baseline lipid profiles, the thickness of carotid intima-media complexes, blood pressure indices and the association with concentration changes of dephosphorylated-uncarboxylated matrix Gla protein (dp-uc MGP) as a marker of subclinical arterial lesions in different age groups of pediatric patients with FH. Materials and methods. Children with heterozygous FH (n=15), stratified by age and sex, were included in the study. The control group consisted of healthy peers (n=21). Blood samples were analyzed to determine levels of total (TC), low-density (LDL-C), very-low-density (VLDL-C), high-density (HDL-C), remnant (rC) and non-high-density (non-HDL-C) cholesterol, triglycerides (TG), apolipoproteins A1 (apoA1) and B (apoB), lipoprotein (a), and dp-uc MGP. The intima-media complex thickness of the common carotid artery and blood pressure were measured in all study subjects. The obtained data were processed using the accepted methods of medical statistics and SAS® OnDemand for Academics. Results. Lipid profile changes in pediatric patients with FH were characterized by high levels of LDL-C, non-HDL-C and lipoprotein (a) in the 5-9 years age group; in the 10-14 years age group - high levels of LDL-C, TG, rC, non-HDL-C and lipoprotein (a); in the 15-18 years age group - high levels of LDL-C, TG, non-HDL-C and lipoprotein (a). At the same time, the most marked dyslipidemia changes were evident in children aged 10-14 years in the FH group. apoA1 levels were significantly decreased in all FH children. Elevated levels of lipoprotein (a) (&gt;30 mg/dL) in FH children were found in all age groups, suggesting that elevated lipoprotein (a) levels can be used as a factor for cardiovascular risk stratification. Dp-uc MGP levels were significantly elevated in all age groups of FH children compared to healthy peers. Conclusions. A lipid profile examination is necessary to diagnose FH in children, along with family health history and cascade screening. As atherosclerotic changes at 5-18 years of age remain subclinical, and the instrumental tests available in routine medical practice are not sensitive enough to detect them, therefore, preventive or therapeutic measures cannot be initiated promptly. The evaluation of circulating matrix Gla protein in pediatric patients with FH can be used as a marker of vascular wall calcification, which may allow early preventive measures against microcalcification to be developed. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

IMPACT OF DIAGNOSTIC AND INTERVENTIONAL ENDOSCOPIC ULTRASONOGRAPHY IN CHILDREN.

Endoscopic ultrasonography is used in the diagnosis and treatment of digestive diseases in adults. In children, its use is limited due to a lack of available expertise. This study aimed to evaluate the clinical impact of endoscopic ultrasonography on diagnostic and therapeutic strategy changes in pediatric patients. Over ten years, this study retrospectively and consecutively analyzed children aged ≤18 years who underwent endoscopic ultrasonography because of inconclusive imaging or laboratory tests. The indications, results, occurrence of adverse events, and clinical impact of the procedures were analyzed. The clinical impact was classified as major (when the findings led to changes in diagnosis and management), minor (change in diagnosis but not in management), or none (no change in diagnosis or management). Overall, 107 children [77 (72%) of whom were female; mean age: 11.7 ± 4 years] underwent upper [102 (95.3%)] and lower [5 (4.7%)] endoscopic ultrasonography; 64 (58%) patients underwent diagnostic endoscopic ultrasonography, and 43 (42%) underwent interventional endoscopic ultrasonography. Endoscopic ultrasonography was used to investigate pancreaticobiliary, gastric, rectal, esophageal, duodenal, and mediastinal diseases in 81 (76%), 14 (13%), 5 (4.6%), 3 (2.8%), 2 (1.8%), and 2 (1.8%) patients, respectively. The clinical impact was significant in 81% of the children. Major and no clinical impact on pancreaticobiliary, gastrointestinal diseases, and mediastinal masses occurred in 50 (62%) and 13 (16%), 13 (54%) and 9 (37%), and 2 (100%) and 0 (0%) of the patients, respectively. This study evaluated the impact of diagnostic and interventional endoscopic ultrasonography in pediatric patients. When clinically and appropriately indicated, these procedures are safe and effective diagnostic or therapeutic interventions in pediatric patients with gastrointestinal or pancreaticobiliary disorders.

Ocular Parameter Changes in Pediatric Patients with Nephrotic Syndrome

Ocular Parameter Changes in Pediatric Patients with Nephrotic Syndrome

Early marker of ocular neurodegeneration in children and adolescents with type 1 diabetes: the contributing role of polymorphisms in mir146a and mir128a genes

BackgroundEarly ocular neurodegenerative signs of diabetic neuropathy (DN) can be found in children and adolescents with type 1 diabetes (T1D). No data are available on the potential role of polymorphisms in miRNAs genes in predisposing T1D subjects to these signs.AimsTo determine whether MIR146A rs2910164 and MIR128A rs11888095 polymorphisms are associated with early retinal and corneal neurodegenerative changes in pediatric patients with T1D.MethodsA total of 140 T1D children/adolescents underwent spectral domain-optical coherence tomography (SD-OCT) and in vivo confocal microscopy (IVCM) with measurement of retinal and corneal nerve fiber parameters. Risk factors for diabetes complications (diabetes duration, blood pressure, HbA1c) were recorded. Genotyping of rs2910164 and rs1188095 SNPs and genotype–phenotype association analysis were performed.ResultsThe C allele of rs2910164 in MIR146A was associated with higher values of IVCM parameters and minimum rim width (MRW) of the peripapillary region of optic nerve head measured in the retina, whereas the T allele of rs1188095 in MIR128A was associated with a significant impairment of them. Multiple regression analysis showed that MIR146A and MIR128A polymorphisms were significantly associated with corneal nerve fiber length (beta = 0.225 and − 0.204, respectively) and other IVCM parameters, independently from age, diabetes duration, HbA1c and systolic blood pressure percentile. Similar results were found for MRW (beta = 0.213 and − 0.286, respectively).ConclusionsThese results provide new insight into the genetic predisposition to DN showing that two polymorphisms in MIR146A and MIR128A genes could significantly contribute to the development of early ocular preclinical signs of DN.

Anaesthetic Management of 2 Micropreemies with Difficult Airway: Case Report and Review of Literature.

The infants are explicitly vulnerable to develop anaesthesia-related complications, with micropreemies being at the end of the spectrum because of their unique morphological and physiological features. Airway changes in pediatric patients are most exaggerated in these tiny infants and their immature lungs provide a little reserve, and therefore, securing airways can be challenging in this population. Moreover, many devices available for managing difficult airways in adults are not available for use in this miniature version. Videolaryngoscopes are a useful addition to anaesthesiologists’ armamentarium but for micropreemies, size of videolaryngoscope can be a limiting factor as no videolaryngoscope comes in “00” size. The technique of videolaryngoscope may need to be modified in these patients. Regional anaesthesia can be an invaluable component of their perioperative care to facilitate a smooth recovery. The successful management of these vulnerable neonates requires a multidisciplinary team approach to maintain perioperative homeostasis of their immature organ systems.