Dysfunction In Children Research Articles

Third-Line Therapeutic Interventions for Non-Neurogenic Bladder Dysfunction in Children.

The aim of this article is to review considerations and efficacy of third-line treatments for pediatric non-neurogenic bladder dysfunction, including Botulinum toxin A (BoTNA), Posterior Tibial Nerve Stimulation (PTNS), and Sacral Neuromodulation (SNM). Federal Drug Administration approval for use of beta-3-agonists in overactive detrusor activity in pediatric patients may provide an additional step prior to third-line therapies. New long-term data on pediatric SNM efficacy, complications, and revision rates will provide valuable information for counseling families. BoTNA offers a safe and efficacious treatment to decrease detrusor contractility and improve bladder capacity but is limited by the half-life of BoNTA agent. Percutaneous or transcutaneous PTNS offers improved voided volumes or cure in some patients but is time-intensive. SNM can be utilized in a variety of LUTD pathology with high success rate and cure but should consider cumulative anesthetic and fluoroscopic exposures for battery replacements and re-positioning for patient growth.

Endothelial Dysfunction in Children with Frequently Relapsing and Steroid-Dependent Nephrotic Syndrome

Background Children with frequently relapsing (FR) or steroid-dependent (SD) nephrotic syndrome (NS) show endothelial dysfunction and risk of endothelial injury during relapses, increasing the risk of accelerated atherosclerosis and adverse cardiovascular events. This study examines the plasma levels of markers of endothelial dysfunction [sE-selectin and vascular cell adhesion molecule-1 (VCAM-1)] in children aged 1–16 years with FRNS and SDNS in relapse. Materials and Methods Fifty-six children with FRNS and SDNS between 1 and 16 years were enrolled at the time of relapse and followed till six weeks of steroid-induced remission. Markers of endothelial dysfunction (sE-selectin and VCAM-1) in plasma were measured in these children and in an equal number of controls. Results Plasma sE-selectin and VCAM-1 levels were significantly raised during relapse, declined after six weeks of steroid-induced remission, and became comparable to controls (p < 0.0001). We found high serum total cholesterol and triglycerides levels during relapse that remained elevated even after steroid-induced remission as compared to controls (p < 0.0001). Raised levels of these markers confirm endothelial dysfunction in FRNS and SDNS patients. Conclusion Children with FRNS and SDNS had endothelial dysfunction during relapse, which was largely dependent upon disease activity.

Intravesical Botulin Toxin-A Injections for Neurogenic Bladder Dysfunction in Children: Summary Update on Last 10 Years of Research.

Neurogenic bladder dysfunction (NB) represents a challenge in pediatric urology. Intravesical botulin toxin-A (BTX-A) bladder injection is part of the armamentarium for the treatment of this condition, usually after failed first-line medical strategies and before the escalation to more invasive options such as neuromodulation or augmented cystoplasty in severe cases. However, there is still a lack of consensus about the appropriate treatment modality for the pediatric population. A review of the last 10 years' research was performed on the PubMed database by two authors. Articles doubly selected and meeting the inclusion criteria were collected and analyzed for their study type, demographics, neurological disease(s) at diagnosis, BTX-A treatment modality and duration, previous treatment, clinical and urodynamic parameters, adverse events, outcomes, and follow-ups. A total of 285 studies were initially selected, 16 of which matched the inclusion criteria. A cohort of 630 patients was treated with BTX-A at a median age of 9.7 years, 40% of which had a diagnosis of myelomeningocele. The results of the selected publications show the overall efficacy and safety of BTX-A injections in children and confirmed BTX-A as a valuable strategy for NB treatment in pediatric population. Nevertheless, up to now, the literature on this topic offers scarce uniformity among the published series and poor protocol standardization.

Preoperative Echocardiographically Derived Mean dP/dTic Predicts Early Post-operative Dysfunction in Children Undergoing Mitral Valve Surgery.

Mean dP/dtic is a quantitative measurement of ventricular function that can be obtained noninvasively by echocardiography. In adults with mitral regurgitation (MR), it has been shown to be a more sensitive predictor of postoperative left ventricular ejection fraction (EF). The utility of dP/dtic in pediatric congenital heart diseases with MR has been underexplored. Patients (0 to ≤ 19years) with MR who underwent mitral valve (MV) repair or replacement from 2015 to 2021 were included. Echocardiographically derived mean dP/dtic, Tei index, and EF were used to assess and compare ventricular function prior to, shortly after, and late after MV surgery. Study cohort included 61 patients (age 4.5 [IQR 0.14, 18.7] years, 89% MV repair, 11% MV replacement). Median time intervals between surgery and preoperative, early postoperative, and late postoperative echocardiograms were 6days, 6days, and 350days, respectively. Median EF was 62% (z-score -0.40) preoperatively, 56% (z-score -1.40) early postoperatively, and 61% (z-score -0.60) late postoperatively. Median dP/dtic was 1393 (IQR 1029, 1775) mmHg/s preoperatively, 1178 (IQR 886, 1946) mmHg/s early postoperatively, and 1270 (IQR 791, 1765) mmHg/s late postoperatively. Preoperative median dP/dtic correlated with early and late postoperative EF. Preoperative EF was not significantly correlated with early postoperative EF, but was correlated with late postoperative EF. Mitral valve intervention in pediatric patients is associated with an initial decline but subsequent recovery of systolic function. Non-invasively derived mean dP/dtic may offer advantages over other preoperative echocardiographic indices to predict postoperative systolic function.

1224 Signs & Symptoms Predictive of CSF Shunt Dysfunction in Children & Young People Presenting to ED

1224 Signs & Symptoms Predictive of CSF Shunt Dysfunction in Children & Young People Presenting to ED

Peripheral Microvascular Dysfunction in Children and Adults with Congenital Heart Disease: A Literature Review.

Although there is a continually growing number of patients with congenital heart disease (CHD) due to medical and surgical advances, these patients still have a poorer prognosis compared to healthy individuals of similar age. In patients with heart failure, microvascular dysfunction (MVD) has recently emerged as a crucial modulator of disease initiation and progression. Because of the substantial pathophysiological overlap between CHD and heart failure induced by other etiologies, MVD could be important in the pathophysiology of CHD as well. MVD is believed to be a systemic disease and may be manifested in several vascular beds. This review will focus on what is currently known about MVD in the peripheral vasculature in CHD. Therefore, a search on the direct assessment of the vasodilatory capacity of the peripheral microcirculation in patients with CHD was conducted in the PubMed database. Since there is little data available and the reported studies are also very heterogeneous, peripheral MVD in CHD is not sufficiently understood to date. Its exact extent and pathophysiological relevance remain to be elucidated in further research.

Cognitive Dysfunction in Children with Chronic Liver Diseases and Portal Vein Thrombosis

Cognitive Dysfunction in Children with Chronic Liver Diseases and Portal Vein Thrombosis

Endothelial Dysfunction Linked to Ventricular Dysfunction in Children With Sickle Cell Disease, a 3D Speckle Tracking Study.

Sickle Cell Disease (SCD) is not a hematologic disease that occurs in isolation; it results in multi-organ complications. There is growing evidence of vascular stiffness as its underlying cause. This study aimed to investigate the relationship between endothelial stiffness and LV dysfunction in SCD patients and to explore its pathophysiology, particularly regarding the depletion of vasodilators such as Nitric Oxide (NO). 32 patients with established criteria for SCD and 40 healthy control subjects were selected for this case-control study. Comprehensive clinical assessment and assessment of endothelial function using Brachial Flow-mediated dilation (FMD) were performed, along with serum NO measurement, which was followed by diagnosis and echocardiographic assessment using 3D speckle tracking echocardiography (STE) and tissue Doppler imaging (TDI). Collected SCD cases showed echocardiographic features of Systo-diastolic dysfunction with reduced FMD compared to controls, denoting endothelial dysfunction in those patients. LDH showed a marked elevation, while serum NO showed a significant reduction in cases compared with controls. We also noted a positive correlation between FMD on the one hand and measures of ventricular dysfunction and level of serum NO on the other hand, the latter proving that reduction of NO is responsible for reduced endothelial function. We present the first report to date to outline the role of vascular stiffness as measured by brachial FMD in the induction of left ventricular dysfunction in SCD. We recommend that more research be conducted regarding possible strategies to replenish serum NO stores to delay microvascular injury and, in turn, ventricular dysfunction in SCD.

Effects of L-Carnitine Supplementation on the Rate of Weight Gain and Biomarkers of Environmental Enteric Dysfunction in Children with Severe Acute Malnutrition: A Double-Blind Randomized Controlled Clinical Trial

BackgroundSevere acute malnutrition (SAM) is a major public health concern among low- and middle-income countries, where the majority of the children encountering this acute form of malnutrition suffer from environmental enteric dysfunction (EED). However, evidence regarding the effects of L-carnitine supplementation on the rate of weight gain and EED biomarkers in malnourished children is limited. ObjectivesWe aimed to investigate the role of L-carnitine supplementation on the rate of weight gain, duration of hospital stays, and EED biomarkers among children with SAM. MethodsA prospective, double-blind, placebo-controlled, randomized clinical trial was conducted at the Nutritional Rehabilitation Unit (NRU) of Dhaka Hospital, International Centre for Diarrheal Disease Research, Bangladesh. Children with SAM aged 9–24 mo were randomly assigned to receive commercial L-carnitine syrup (100 mg/kg/d) or placebo for 15 d in addition to standard of care. A total of 98 children with Weight-for-Length-z-score (WLZ) < −3 Standard deviation were enrolled between October 2021 and March 2023. Analyses were conducted on an intention-to-treat basis. ResultsThe primary outcome variable, “rate of weight gain,” was comparable between L-carnitine and placebo groups (2.09 ± 2.23 compared with 2.07 ± 2.70; P = 0.973), which was consistent even after adjusting for potential covariates (age, sex, Weight-for-Age z-score, asset index, and WASH practices) through linear regression [ß: 0.37; 95% confidence interval (CI): −0.63,1.37; P = 0.465]. The average hospital stay was ∼4 d. The results of adjusted median regression showed that following intervention, there was no significant difference in the EED biomarkers among the treatment arms; Myeloperoxidase (ng/mL) [ß: −1342.29; 95% CI: −2817.35, 132.77; P = 0.074], Neopterin (nmol/L) [ß: −153.33; 95% CI: −556.58, 249.91; P = 0.452], alpha-1-antitrypsin (mg/mL) [ß: 0.05; 95% CI: −0.15, 0.25; P = 0.627]. Initial L-carnitine (μmol/L) levels (median, interquartile range) for L-carnitine compared with placebo were 54.84 (36.0, 112.9) and 59.74 (45.7, 96.0), whereas levels after intervention were 102.05 (60.9, 182.1) and 105.02 (73.1, 203.7). ConclusionsAlthough our study findings suggest that L-carnitine bears no additional effect on SAM, we recommend clinical trials with a longer duration of supplementation, possibly with other combinations of interventions, to investigate further into this topic of interest.This trial was registered at clinicaltrials.gov as NCT05083637.

Endothelial Dysfunction in Children With Nephrotic Syndrome: A Cross-Sectional Study.

Background Children with nephrotic syndrome (NS) have a higher risk of cardiovascular morbidity. Studies on the evaluation of arterial stiffness and endothelial function and its predictive risk factors in these children are limited. Objective The primary objective of the study was to determine arterial stiffness by measuring carotid intimal medial thickness, flow-mediated dilatation, and physiological parameters in children with nephrotic syndrome to predict the risk of premature atherosclerosis as compared to controls. Participants A total numberof 33 children with NS in the age group of 2-14 years in remission and 39 healthy controls were enrolled in the study. Out of 33 children with nephrotic syndrome, five were infrequently relapsing NS, eight were frequently relapsing, 16 were steroid dependent, and four were steroid-resistant NS. Intervention Relevant history, physical examination, anthropometric measurements, and laboratory investigations were done. Carotid intimal medial thickness (cIMT), flow-mediated dilatation (FMD), and other physiological parameters were measured in both children with NS and control groups. Outcome Carotid intimal medial thickness (cIMT), flow-mediated dilatation (FMD), and other physiological parameters were compared between children with NS and healthy controls for detecting arterial stiffness and endothelial dysfunction. Results Dyslipidaemia was seen in more than 50% of children during remission. There was neither significant difference in mean cIMT in the common carotid artery nor FMD between the control and study groups. There was a trend of lower Reactive Hyperemia Index (RHI) in children with NS. Conclusion Dyslipidemia persists even during the remission phase in NS. No statistically significant difference is observed in cIMT and percentage proportionate change in FMD in both the study and control groups. Nevertheless, RHIis notably lower in children with NS. These findings need further validation in future studies.

Is Left Atrial Strain the Missing Part to Diagnose Diastolic Dysfunction in Children with Chronic Kidney Disease?

Is Left Atrial Strain the Missing Part to Diagnose Diastolic Dysfunction in Children with Chronic Kidney Disease?

Kiosk 2R-FB-10 - Left Ventricular Diastolic Dysfunction in Children with Atrial Septal Defect Is Likely Augmented by a Reduced Hydraulic Force

Kiosk 2R-FB-10 - Left Ventricular Diastolic Dysfunction in Children with Atrial Septal Defect Is Likely Augmented by a Reduced Hydraulic Force

Sonographically Determined Fecal Width: An Objective Indicator of Management of Bladder and Bowel Dysfunction in Children

ObjectivesTo test whether sonographically determined fecal width (SDFW) correlates with symptom improvement in a population of children with Bladder and Bowel Dysfunction (BBD) managed with Standard Urotherapy, even for those patients lacking initial bowel complaints, MethodsWe retrospectively analyzed 200 pediatric BBD patients managed with SU for at least 3 months. Self-reported symptom improvement (complete, partial, no response) following International Children’s Continence Society (ICCS) guidelines was tabulated. Patients with complex urologic diagnoses other than VUR were excluded. Pharmacotherapy choice, physical therapy (PT), UTI occurrence, and VUR status were tabulated. SDFW was recorded. Non-parametric ANOVA and parametric/non-parametric T-testing were used for analysis. ResultsPatients had a mean age of 9.5 years (4-12). 48 patients had no GI complaints at presentation. Urotherapy yielded complete, partial, and no responses in 14% (n=27), 33% (n=67), and 53% (n=106) of patients, respectively. The average SDFW for those patients with complete response (2.6cm) was smaller than the SDFW of those with a partial response (3.1cm) or no response (3.3cm) (p=0.0001). Non-compliance led to greater SDFW compared to compliant patients (3.7cm and 3.1cm respectively, p=0.0001). FW was unaffected by VUR, UTI, PT, or pharmacotherapy. ConclusionsSDFW correlates well with symptom improvement in pediatric patients managed for BBD, confirming our hypothesis. SDFW is reasonable as single objective parameter to identify successful management in patients with BBD, extending to those without bowel complaints at presentation.

Kiosk 2R-FB-07 - The Discriminatory Ability of Cardiac MRI Biomarkers to Differentiate Severity of Myocardial Dysfunction in Children

Kiosk 2R-FB-07 - The Discriminatory Ability of Cardiac MRI Biomarkers to Differentiate Severity of Myocardial Dysfunction in Children

Upper Esophageal Sphincter Dysfunction in Children with Type 1 Laryngeal Cleft after Failed Primary Cleft Repair.

Changes in pharyngeal and upper-esophageal-sphincter (UES) motor dynamics contribute to swallowing dysfunction. Children with type 1 laryngeal clefts can present with swallowing dysfunction and associated symptoms which may persist even after the initial endoscopic intervention. This study sought to characterize pharyngeal and esophageal motor function in children with type 1 laryngeal clefts who had persistent presenting symptoms after their initial therapeutic intervention. We retrospectively analyzed high-resolution esophageal manometry studies of children ≤ 18 years old with type 1 laryngeal clefts who had an esophageal manometry study performed for persistent symptoms after an initial repair. A total of 16 children were found to have significantly increased UES resting pressure, UES pre- and post-swallow maximum pressures, and duration of UES contraction during swallows in comparison to nine age-matched controls of children without pharyngeal anatomical abnormalities. There was no difference between UES residual pressures or pharyngeal dynamics between the two groups. UES resting and residual pressures did not correlate with VFFS in penetration and aspiration scores of children with type 1 laryngeal clefts status post repair. Our study is the first to identify specific changes in UES motor function in patients with type 1 laryngeal cleft post initial repair.

Personalized Pre-clinic Nursing Telemedicine Visit: An Efficient and Efficacious Approach for Bowel and Bladder Dysfunction in Children

To evaluate the effectiveness and efficacy of a Registered Nurse (RN) led educational pre-clinic telephone call on compliance and outcomes in children with bowel and bladder dysfunction (BBD). A retrospective chart review of a prospectively applied protocol in a single academic institution was performed for children aged 4-17 presenting with BBD. All children underwent a pre-clinic RN telemedicine visit where they were educated on pathophysiology of BBD, provided personalized urotherapy and bowel recommendations and instructed to complete pre-clinic questionnaires and voiding diaries. Patients were evaluated by a provider 4weeks following RN call. Data collected included compliance with forms, bowel management and need for imaging/testing, medications, and biofeedback. Patients were considered to improve with urotherapy alone if they were discharged from urology without the need for medications and/or biofeedback. In total, 277 patients completed an RN call and 224 patients attended a provider visit between December 2020 and June 2022. Mean age was 9.4years (3:1 Female to Male ratio). During the RN call, 154 (56%) patients had bowel management initiated. Of the 224 patients seen by a provider, 69% (n=154) had symptom improvement or resolution with urotherapy alone. Thirty-eight patients (17%) enrolled in biofeedback with 7 (3%) completing all 8 sessions. Thirty-two patients (14%) required medication for daytime bladder symptoms. Our novel RN-led pre-clinic telemedicine visit demonstrates excellent compliance and patient outcomes for children with BBD and can reduce the use of unnecessary imaging, medications, and time-consuming treatments such as biofeedback.

O-4 | Low Risk of Thyroid Dysfunction in Children

O-4 | Low Risk of Thyroid Dysfunction in Children <3 Years After Exposure to Iodine-Containing Contrast Agents in Cardiac Catheterization

A Study on Clinical Correlation and Outcome of Dengue Fever with Hepatic Dysfunction in Children

A Study on Clinical Correlation and Outcome of Dengue Fever with Hepatic Dysfunction in Children

Essential Fatty Acid Deficiency Associates with Growth Faltering and Environmental Enteric Dysfunction in Children.

Environmental enteric dysfunction (EED) is characterized by intestinal inflammation, malabsorption and growth-faltering in children with heightened exposure to gut pathogens. The aim of this study was to characterize serum non-esterified fatty acids (NEFA), in association with childhood undernutrition and EED, as potential biomarkers to predict growth outcomes. The study comprised a cohort of undernourished rural Pakistani infants (n = 365) and age-matched controls followed prospectively up to 24 months of age. Serum NEFA were quantified at ages 3-6 and 9 months and correlated with growth outcomes, serum bile acids and EED histopathological biomarkers. Serum NEFA correlated with linear growth-faltering and systemic and gut biomarkers of EED. Undernourished children exhibited essential fatty acid deficiency (EFAD), with low levels of linoleic acid and total n-6 polyunsaturated fatty acids, compensated by increased levels of oleic acid and increased elongase and desaturase activities. EFAD correlated with reduced anthropometric Z scores at 3-6 and 9 months of age. Serum NEFA also correlated with elevated BA and liver dysfunction. Essential fatty acid depletion and altered NEFA metabolism were highly prevalent and associated with acute and chronic growth-faltering in EED. The finding suggests that targeting early interventions to correct EFAD and promote FA absorption in children with EED may facilitate childhood growth in high-risk settings.

The Prevalence of Bladder and Bowel Dysfunction in Children with Cerebral Palsy and its Association with Motor, Cognitive, and Autonomic Function

ABSTRACT Purpose To describe the prevalence of bladder and bowel dysfunction (BBD) in 8–10-year-old children with cerebral palsy and its association with motor, cognitive, and autonomic dysfunction. Methods A cross-sectional, random sample study of parents of 8–10-year-old children with cerebral palsy. Tools: The Enuresis/Urinary Incontinence Parental Questionnaire, the Functional Independence Measure children’s version, the autonomic signs questionnaire, and the Gross Motor Function Classification System. Results 39 out of 59 parents consented to participate, whereas 25.64% reported complete continence. Of the 29 children with BBD, 21 (72.4%) had lower urinary tract symptoms and bowel problems. Only two of the children received conservative and noninvasive treatments. Lastly, motor, cognitive and autonomic impairments were associated with incontinence. Conclusions BBD is common in 8–10-year-old children with cerebral palsy at all levels of functioning. Most having both lower urinary tract symptoms and bowel problems.