Dysfunction In Pediatric Patients Research Articles

Organizational challenges for partial heart transplantation.

Partial heart transplantation (PHT) has emerged as a new treatment strategy to correct unrepairable heart valve dysfunction in pediatric patients. PHT selectively replaces the dysfunctional components of the recipient's heart and spares the native ventricles. As a result, the transplant biology of PHTs differs from heart transplants. Notably, donor hearts that are unsuitable for whole heart transplantation can be used, graft preservation can be prolonged and immunosuppression levels can be lowered. These nuances of PHT transplant biology have important implications for organizational aspects of PHT clinical application.

Effects of cyclophosphamide administration on ovarian dysfunction in pediatric patients with connective tissue diseases: A systematic scoping review.

This systematic scoping review assess the effect of cyclophosphamide (CY) administration during childhood on ovarian function in patients with juvenile-onset connective tissue diseases. A MEDLINE database search was conducted using terms related to CY, juvenile-onset connective tissue diseases, and ovarian function. Studies were included if they met specific criteria. The search, conducted on 28 November 2023, yielded 3328 references. After a two-stage screening process, six observational studies on systemic lupus erythematosus patients were included. All studies had a high risk of confounding bias, as none adjusted for confounding variables. Two studies assessing clinical ovarian dysfunction found no clear difference between CY and non-CY groups. However, statistical differences were observed in hormonal profiles. Decreased ovarian reserve was more frequent in CY-exposed patients. Two studies showed significantly higher follicle-stimulating hormone (FSH) levels in the CY group, while one showed a trend towards higher FSH levels without statistical significance. This review suggested that CY use in childhood may not conclusively have clinically significant effects on ovarian function. Further investigation needed on CY's effect on hormonal levels, fertility, and pregnancy outcomes.

Permanent cutaneous vesicostomy: a pragmatic approach to safely manage lower urinary tract dysfunction in pediatric patients with chronic and life-limiting conditions and neuropathic bladders.

Lower urinary tract dysfunction (LUTD) in cerebral palsy (CP) and other neuromuscular diseases can present with chronic retention that leads to hydronephrosis, recurrent urinary tract infections (UTI), and stone formation. Whenever the conservative treatment of LUTD fails for any reason, it is considered to be complicated LUTD, in which a surgical approach is warranted. Cutaneous vesicostomy (CV) is a simple, well-tolerated, and potentially reversible procedure that protects the upper tracts. We describe our experience using CV for this complex population. Children with CP and other neuromuscular diseases admitted to pediatric long-term care units for palliative care between 2015 and 2019 were included in the study. They present multi-system involvement, polypharmacy, and Gross Motor Function Classification System levels of 4 or 5. We retrospectively studied this population's indications and results of CV. Of the 52 admitted patients, 18 presented LUTD with UTI (n:18; 100%), stones (n:5; 28%), progressive hydroureteronephrosis (n:3; 17%), or stones (n:2; 11%). Conservative initial management (catheterizations, prophylaxis antibiotics) was effective in half the cases. The remaining nine were defined as complicated LUTD and underwent CV. After a mean follow-up of 11.3 months, the follow-up showed improved hydronephrosis in all nine (100%) patients. Recurrent UTIs were no longer seen in eight of nine patients, although three patients required bladder irrigations; bladder stones did not recur after CV; the kidney stones needed further intervention. Revision of the CV was required in two (11%) cases at 12 and 24 months postoperatively due to stoma stenosis. CV is a relatively simple and effective procedure representing a pragmatic solution for managing complicated LUTD in complex long-term institutionalized pediatric palliative care patients with neuropathic bladders.

Comparing Video Head Impulse Testing With Rotary Chair in Pediatric Patients: A Controlled Trial.

To investigate the efficacy of video head impulse testing (VHIT) in detecting vestibular loss in pediatric patients with abnormal rotary chair testing, compared to a control group with normal rotary chair testing. Prospective, nonrandomized, controlled trial. Pediatric vestibular program at tertiary level children's hospital. Patients 3 to 18 years of age were recruited to undergo VHIT and rotary chair testing between September 2015 and November 2022. VHIT results were compared between an experimental group of 23 patients with vestibular symptoms and abnormal rotary chair testing results versus a control group of 14 patients without vestibular symptoms and with normal rotary chair testing results. The experimental group demonstrated reduced mean VHIT gain for the lateral semicircular canals (right = 0.83; left = 0.75) relative to the control group (right = 1.04, P = .005; left = 0.98, P < .001), but there was no significant difference between groups in superior and posterior canal responses. Among controls, there were no significant differences in mean gain values for each canal plane between age groups (3-7, 8-12, and 13-18 years) or between genders. A VHIT lateral canal gain value <0.85 and/or corrective saccades demonstrated 100% specificity and 100% positive predictive value for detecting vestibular loss on rotary chair, but only 69.6% sensitivity and 66.7% negative predictive value. VHIT is a useful test for detecting impairment of the lateral semicircular canals in children, though its sensitivity is limited relative to rotary chair. Its role in detecting vertical canal dysfunction in pediatric patients may be more limited and warrants further study.

Lower Urinary Tract Dysfunction in Pediatric Patients with Multiple Sclerosis: Diagnostic and Management Concerns.

Multiple sclerosis (MS) is increasing in the pediatric population and, as in adults, symptoms vary among patients. In children the first manifestations can sometimes overlap with acute neurological symptoms. Urological symptoms have not been much studied in childhood. We shared our experience with MS urological manifestation in children. This article is a retrospective evaluation of all children with MS, according to the Krupp criteria, who also present with urological symptoms. We collected demographic and clinical history, the MR localization of demyelinating lesions, urological symptoms, and exams. We report on six MS pediatric cases with urological manifestation. Urinary symptoms, characterized by urinary incontinence in five patients and urinary retention in one patient, appeared in a different time frame from MS diagnosis. Urodynamic exams showed both overactive and underactive bladder patterns. Treatment was defined according to lower urinary tract dysfunction, using clean intermittent catheterization, oxybutynin, and intradetrusor Onabotulinum Toxin-A injection. A low acceptance rate of invasive evaluation and urological management was observed. The MS diagnosis was traumatic for all our patients. We believe it is important to address urological care in young people from the time of diagnosis for prompt management; it could be useful to include a pediatric urologist in multidisciplinary teams.

Double Ventricular Response with Aberrant Conduction Leading to Ventricular Dysfunction.

Double ventricular response (DVR), where a single P wave results in two QRS complexes, is a rare presentation of dual AV node physiology. It has been associated with ventricular dysfunction in the setting of incessant tachycardia. We present the case of an otherwise healthy adolescent who had frequent DVR without tachycardia leading to left ventricular dysfunction. Slow pathway modification led to a significant reduction in ectopy and normalization of ventricular function. This highlights that DVR without tachycardia might lead to ventricular dysfunction in pediatric patients. Slow pathway modification with reduction of ectopy may be sufficient to restore ventricular function.

Hormonal Dysfunction in Paediatric Patients Admitted to Rehabilitation for Severe Traumatic Brain Injury: Analysis of the Associations with Rehabilitation Outcomes.

Traumatic brain injury is often accompanied by defects in hormone levels, caused by either peripheral gland dysfunctions or by an insufficient central stimulation of hormone production. The epidemiology of endocrinological defects after traumatic brain injury is quite well described, but the consequences of hormone defects are largely unknown, especially in paediatric patients undergoing neurological rehabilitation. Only one previous study reported on a cohort of 20 children with traumatic brain injury and found a low incidence of hormone defects and a correlation between some hormone levels and neurological recovery. In this study, we performed a retrospective chart review on patients affected by severe subacute traumatic brain injury. Their levels of cortisol, ACTH, IGF-1, TSH, free T4, free T3, and prolactin were collected and compared with reference ranges; we then used regression models to highlight any correlation among them and with clinical variables; last, we probed with regression models whether hormone levels could have any correlation with clinical and rehabilitation outcomes. We found eligible data from the records of 52 paediatric patients with markedly severe traumatic brain injury, as shown by an average GCS of 4.7; their age was 10.3 years, on average. The key results of our study are that 32% patients had low IGF-1 levels and in multiple regression models, IGF-1 levels were correlated with neurological recovery, indicating a possible role as a biomarker. Moreover, 69% of patients had high prolactin levels, possibly due to physical pain and high stress levels. This study is limited by the variable timing of the IGF-1 sampling, between 1 and 2 months after injury. Further studies are required to confirm our exploratory findings.

Telomere Dysfunction in Pediatric Patients with Differences/Disorders of Sexual Development.

Peripheral blood lymphocytes were sampled from 35 DSD patients (mean age: 3.3 years; range 0-18 years) admitted to two hospital centers in Dakar. Peripheral blood lymphocytes from 150 healthy donors were used as a control. Conventional cytogenetics, telomere, and centromere staining followed by multiplex FISH, as well as FISH with SRY-specific probes, were employed. Cytogenetic analysis identified 19 male and 13 female patients with apparently normal karyotypes, two patients with Turner syndrome, and one patient with Klinefelter syndrome. Additional structural chromosome aberrations were detected in 22% of the patients (8/35). Telomere analysis revealed a reduction in mean telomere lengths of DSD patients compared to those of healthy donors of similar age. This reduction in telomere length was associated with an increased rate of telomere aberrations (telomere loss and the formation of telomere doublets) and the presence of additional chromosomal aberrations. To the best of our knowledge, this study is the first to demonstrate a correlation between telomere dysfunction and DSDs. Further studies may reveal the link between telomere dysfunction and possible mechanisms involved in the disease itself, such as DNA repair deficiency or specific gene mutations. The present study demonstrates the relevance of implementing telomere analysis in prenatal tests as well as in diagnosed genetic DSD disorders.

Identification and treatment of persistent small airway dysfunction in paediatric patients with asthma: a retrospective cohort study

BackgroundAsthma is a common respiratory disease. In asthma, the small airways have more intensive inflammation and prominent airway remodelling, compared to the central airways. We aimed to investigate the predictive value of risk factors and the fractional concentration of exhaled nitric oxide (FeNO) for persistent small airway dysfunction (p-SAD), and compare the effects of different treatment modalities.MethodsThis retrospective cohort study included 248 children with asthma (aged 4–11 years). Binary logistic regression was used to analyse the risk factors for p-SAD. Correlations among FEV1/FVC, small airway function parameters, and FeNO levels in patients with asthma were analysed using Spearman’s rank correlation. The receiver operating characteristic curve and the Delong test were used to analyse the predictive value of FeNO for p-SAD. Differences in the treatment effects of inhaled corticosteroids (ICS) and ICS with a long-acting beta-agonist (ICS/LABA) on p-SAD were analysed using Fisher’s exact test.ResultsAsthmatic children with older age of receiving the regular treatment (OR 1.782, 95% CI 1.082–2.935), with younger age at the time of onset of suspected asthma symptoms (OR 0.602, 95% CI 0.365–0.993), with longer duration of using ICS or ICS/LABA (OR 1.642, 95% CI 1.170–2.305) and with worse asthma control (OR 3.893, 95% CI 1.699–8.922) had increased risk for p-SAD. Significant negative correlations of small airway function parameters with FeNO at a 200 mL/s flow rate (FeNO200), and the concentration of nitric oxide in the alveolar or acinar region (CaNO) were observed. The areas under the curve of FeNO200 (cut-off:10.5ppb), CaNO (cut-off:5.1ppb), and FeNO200 combined with CaNO were 0.743, 0.697, and 0.750, respectively, for asthma with p-SAD. After using ICS or ICS/LABA, switching to ICS/LABA was easier than continuing with ICS to improve small airway dysfunction (SAD) in the 8th month.ConclusionsPaediatric asthma with p-SAD is associated with older age at receiving regular treatment, younger age at the time of onset of suspected asthma symptoms, longer duration of using ICS or ICS/LABA, worse asthma control, and higher FeNO200 and CaNO levels, all of which can be combined with small airway function indicators to distinguish p-SAD from asthma. ICS/LABA improves SAD better than ICS alone.

Prevalence of hepatic dysfunction in paediatric patients with Fontan circulation

Prevalence of hepatic dysfunction in paediatric patients with Fontan circulation

Assessment of glymphatic dysfunction in pediatric idiopathic intracranial hypertension: insights from quantitative diffusivity and perivascular spaces analysis-a case-control study.

The impaired drainage of cerebrospinal fluid through the glymphatic system is thought to play a role in the idiopathic intracranial hypertension (IIH) pathophysiology. Limited data exist regarding the glymphatic system's involvement in pediatric patients with IIH. Therefore, the study's objective was to quantitatively evaluate alterations in parenchymal diffusivity and magnetic resonance imaging (MRI)-visible dilated perivascular spaces (PVS) as imaging indicators of glymphatic dysfunction in pediatric patients with IIH. Patients diagnosed with IIH in 2017-2022 in a single tertiary center (Sheba Medical Center, Israel) were retrospectively reviewed. Twenty-four pediatric patients were enrolled. All patients underwent clinical 3-T brain MRI. The control group included 24 age- and gender-matched healthy subjects with a normal-appearing brain on imaging. We used automatic atlas-based diffusion-weighted imaging analysis to determine regional diffusivity of the thalamus, caudate, putamen, globus pallidus, hippocampus, amygdala, and brain stem. PVS were evaluated using a semi-quantitative rating scale on T2-weighted images. Variables were compared using the Mann-Whitney test. Multivariate analysis of covariance was used to test for differences between controls and IIH patients. No significant differences in regional brain diffusivity were observed between individuals with IIH and healthy controls (P=0.14-0.91 for various brain regions). The number of visible PVS was comparable between patients with IIH and the control group across all evaluated sites (P=0.12-0.74 for various brain regions). Pediatric IIH patients exhibited similar patterns of parenchymal diffusivity and PVS compared to age-matched controls. These findings do not support the hypothesis that the glymphatic system may play a role in the pathophysiology of pediatric IIH, although previously postulated. However, employing more sophisticated magnetic resonance (MR) techniques could enhance the sensitivity in uncovering underlying glymphatic dysfunction. Further research is warranted to validate and explore this association in larger cohorts and investigate the underlying mechanisms involved in IIH.

Novel implementation of cardiac magnetic resonance first-pass perfusion imaging for semi-quantitatively evaluating microvascular dysfunction in paediatric patients with Duchenne muscular dystrophy.

The current study aimed to assess myocardial microcirculation dysfunction via cardiac magnetic resonance (CMR) first-pass perfusion imaging in children with Duchenne muscular dystrophy (DMD). In total, 67 children with DMD and 15 controls who underwent contrast-enhanced CMR first-pass perfusion imaging were enrolled in this study. CMR first-pass perfusion and late gadolinium enhancement (LGE) sequences were acquired. Further, the global, regional, and coronary artery distribution area perfusion indexes (PI), upslope (%BL), maximum signal intensity (MaxSI), time to maximum signal intensity (TTM), and baseline SI were analysed. The perfusion parameters of the LGE positive (+), LGE negative (-), and control groups were compared. Pearson correlation analysis was performed to assess the association between myocardial microcirculation and conventional cardiac function and LGE parameters. The LGE+ group had a significantly lower global and apical-ventricular MaxSI than the control group (all P < .05). The left anterior descending arterial (LAD), left circumflex coronary arterial (LCX), and right coronary arterial (RCA) segments of the LGE+ group had a lower upslope and MaxSI than those of the control group (all P < .05). The LAD segments of the LGE- group had a lower MaxSI than those of the control group (41.10 ± 11.08 vs 46.36 ± 13.04; P < .001). The LCX segments of the LGE- group had a lower PI and upslope than those of the control group (11.05 ± 2.84 vs 12.46 ± 2.82; P = .001; 59.31 ± 26.76 vs 68.57 ± 29.99; P = .002). Based on the correlation analysis, the upslope, MaxSI, and TTM were correlated with conventional cardiac function and LGE extent. Paediatric patients with DMD may present with microvascular dysfunction. This condition may appear before LGE and may be correlated with coronary artery blood supply and LGE extent. First-pass perfusion parameters may reveal the status of myocardial microcirculation and reflect the degree of myocardial injury at an earlier time in DMD patients. Perfusion parameters should be analysed not only via global or base, middle, and apical segments but also according to coronary artery distribution area, which may detect myocardial microvascular dysfunction at an earlier stage, in DMD patients with LGE-.

Prevalence and impact of antithyroid antibodies in children with immune thrombocytopenic purpura

Abstract Background Immune thrombocytopenia (ITP) is a common autoimmune condition characterized by isolated thrombocytopenia. ITP can present either as a primary disorder or secondary to other diseases, such as infections or altered immune states. The aim of this work was to evaluate the prevalence of antithyroid autoantibodies found at the time of diagnosis of childhood ITP and their influence on treatment response, in addition to prevalence of antiplatelet antibodies and thyroid dysfunction in pediatric patients with ITP. Patients and methods This prospective controlled study was carried out on 50 children with newly diagnosed primary ITP, and 50 apparently healthy children served as a control group. Serum levels of thyroid-stimulating hormone, free thyroxin and free triiodothyronine), anti-thyroglobin, anti-peroxidase antibodies, and antiplatelet antibodies were evaluated at diagnosis. Results Anti-thyroglobin, anti-peroxidase antibodies, and antiplatelet were significantly positive in children with ITP than the control group. Patients with positive antiplatelet, anti-thyroglobin, and anti-peroxidase antibodies show more resistance to effective medications in comparison to ITP patients with negative antibodies (P&lt;0.001). The relation of these antibody positivity and the clinical course of these groups was statistically significant. Conclusions Pediatric ITP patients are prone to develop autoantibodies such as antiplatelet antibodies. Also, anti-thyroglobin and anti-peroxidase antibodies were significantly higher in children with ITP with positive antiplatelet, and both show more resistance to effective medications with relatively good response to eltrombopag olamine in comparison to ITP patients with negative antiplatelet, anti-thyroglobin, and anti-peroxidase antibodies.

Right Ventricular Strain Analysis By Tissue Tracking Cardiac Magnetic Resonance Imaging In Pediatric Patients With End-Stage Renal Disease.

To investigate right ventricular (RV) volume and mass by cardiac magnetic resonance (CMR) and the added value of tissue tracking strain analysis as markers of RV dysfunction in pediatric patients with end-stage renal disease (ESRD) and preserved RV ejection fraction. Twenty-five children with ESRD and preserved RVEF (>50%) and 10 healthy control children were enrolled. Tissue tracking CMR was used to assess Global Longitudinal, circumferential (GCS), and radial short and long axes (GRS SAX and GRS LAX) RV strains in the patients group compared with controls. Correlations between strain parameters and other CMR parameters and clinical biomarkers were assessed. Binary logistic regression was used to test the independence of cofounders and detect their significance. RV end-diastolic volume and mass (RVMi) were significantly higher in patients (97.2±19.3 mL/m2 and 26.6±7gr/m2) than control (71±7.8 mL/m2 and 11.9±2 gr/m2, P values 0.000). All RV global strain parameters were significantly impaired in patients compared with control (all P values <0.05). RV Global Longitudinal was significantly correlated to LVEF (r=-0.416, P=0.039), LVEDVi (r=0.481, P=0.015), LVMi (r=0.562, P=0.004), and systolic blood pressure index (r=0.586, P=0.002). RV GRS (LAX) was significantly correlated to LV GCS (r=-0.462, P=0.020) and LV GRS (SAX) (r=0.454, P=0.023). GRS (SAX) and GCS demonstrated the highest diagnostic accuracy (area under curve: 0.82 and 0.81) to detect strain impairment. Univariate binary logistic regression with patients versus control as dependent variables identified LVMi, RV end-diastolic volume, RVMi, weight, body surface area, RV GCS, RV GRS (LAX), RV GRS (SAX), LV GCS, and LV GRS (SAX) as significantly correlated to patients with ESRD. When adjusted to other cofounders in the multivariable model, only RVMi remained as an independent significant cofounder (Odds ratio:0.395, P=0.046). RV global strain, volume, and mass by CMR are markers of RV dysfunction in ESRD pediatric patients with preserved RVEF.

Endothelin-1 gene polymorphism (G8002A) and endothelial monocyte-activating polypeptide II: Role in vascular dysfunction in pediatric patients with β-thalassemia major.

Endothelin-1 (ET-1), a potent endogenous vasoconstrictor, stimulates production of reactive oxygen species. Endothelial monocyte-activating polypeptide-II (EMAP-II) is a multifunctional polypeptide. To assess ET-1 gene polymorphism (G8002A) in pediatric patients with β-thalassemia major (β-TM) as a potential genetic marker for vascular dysfunction and its possible relation to EMAP II, oxidative stress and vascular complications. β-TM patients (n=95) without symptomatic cardiac or renal disease were compared with 95 healthy controls. Markers of hemolysis, serum ferritin, urinary albumin-to-creatinine ratio, serum EMAP II, malondialdehyde (MDA) and antioxidant enzymes; superoxide dismutase (SOD), glutathione peroxidase (GPx), reduced glutathione (GSH), glutathione reductase and catalase were measured. ET-1 gene polymorphism (G8002A) was determined using polymerase chain reaction‑restriction fragment length polymorphism. β-TM patients had significantly higher EMAP II than healthy controls. EMAP II was significantly higher among patients with cardiac disease, pulmonary hypertension (PH) risk, nephropathy, poor compliance to therapy and ferritin≥2500μg/L. There were significant correlations between EMAP II and transfusion index, LDH, ferritin and oxidative stress markers. The AA genotype of ET-1 gene polymorphism (G8002A) was significantly higher among β-TM patients than controls. The number of patients with cardiac disease, PH risk or nephropathy was significantly higher among AA genotype compared with GG and GA genotypes. Lactate dehydrogenase (LDH), serum ferritin, EMAP II, MDA, SOD and GPx were significantly higher in AA genotype. ET-1 gene polymorphism (G8002A) could be a possible genetic marker for prediction of increased susceptibility to cardiopulmonary and renal complications among pediatric patients with β-TM.

Renal Dysfunction in Pediatric Patients in Iraq With β-Thalassemia Major and Intermedia.

BackgroundWith optimum transfusion and chelation therapy, the survival of β-thalassemia patients and the incidence of various complications, including renal complications, have improved.ObjectivesTo investigate renal involvement in β-thalassemia patients using serum and urinary biochemical markers of glomerular and tubular dysfunction.MethodsThis case-control study included 69 β-thalassemia major (β-TM) patients, 23 β-thalassemia intermedia (β-TI) patients, and 100 healthy controls, all ranging from 1 to 16 years in age. Blood urea nitrogen (BUN), serum ferritin, serum and urinary levels of creatinine (Cr), uric acid (UA), calcium (Ca), phosphorus (Ph), magnesium (Mg), sodium (Na), and potassium (K), and the urinary albumin/creatinine ratio were evaluated.ResultsThe BUN level and the urinary Na/Cr, K/Cr, Ca/Cr, Mg/Cr, Ph/Cr, albumin/Cr, and UA/Cr ratios were significantly higher in the β-thalassemia patients than in the controls. In contrast, the serum Na, K, Ca, and Mg levels were significantly lower in the patients (P<0.05). An elevated urinary UA/Cr ratio was found in 61.9% of β-thalassemia patients, and an elevated urinary Ca/Cr, and urinary albumin/Cr ratio was found in 53.2%. An elevated Na/Cr ratio was found in 41.3%. The serum and urinary renal markers showed no significant differences between patients with β-TM and β-TI, except for microscopic hematuria, which was significantly higher in β-TI patients (34.8%) than in β-TM patients (13%), P>0.02. At an older age, high serum ferritin levels and deferoxamine therapy were associated with significant tubular and glomerular dysfunction in β-thalassemia patients.ConclusionsPediatric patients with β-thalassemia have significantly abnormal tubular and glomerular functions, necessitating early detection and monitoring to prevent/reverse renal function deterioration.

Persistent Liver Dysfunction in Pediatric Patients After Total Cavopulmonary Connection Surgery.

BackgroundStudies have reported early liver dysfunction (LD) after cardiac surgery is associated with short and long-term mortality. In this study, we aimed to investigate risk factors for persistent LD after total cavopulmonary connection (TCPC) surgery.MethodsThis is a retrospective case-control study. We defined persistent LD as LDs occurring between postoperative day 1 (POD1) and POD7 and sustaining at least on POD7, while transient LD as LDs occurring between POD1 and POD7 and recovering at least on POD7. Multivariable logistic regression analysis was applied and central venous pressure (CVP) was considered continuously or in quantiles.ResultsPostoperative LD occurred in 111 (27.1%) patients. Transient and persistent LD occurred in 65 (15.9%) and 46 (11.2%) patients, respectively. Aortic cross-clamping (ACC) (odds ratio [OR] 2.55, 95% CI 1.26–5.14) and postoperative CVP (OR 1.34, 95% CI 1.18–1.51) were risk factors for persistent LD, also identified for postoperative any LD and transient LD. Adding postoperative CVP to the model only including ACC significantly improved persistent LD prediction (△AUC 0.15, p = 0.002). Compared with CVP ≤ 14 mmHg, adjusted ORs and 95% CI of persistent LD for CVP of 14–16 and >16 mmHg were 3.11 (1.24, 7.81) and 10.55 (3.72, 29.93), respectively. Patients with persistent LD might have a longer length of mechanical ventilation (mean difference, 13.5 h) and postoperative hospital stay (mean difference, 7 days), and higher postoperative costs (mean difference, 6.7 thousand dollars) compared to those with transient LD.ConclusionsIntra-operative application of ACC and postoperative elevated CVP were independent risk factors for persistent LD in pediatric patients following TCPC surgery. Compared to patients with transient LD, patients with persistent LD might have a longer length of mechanical ventilation and postoperative hospital stay, and higher postoperative costs. We should pay more attention to patients with high postoperative CVP to prevent their persistent LD occurrence.

Pressure-Strain Loops, a Novel Non-invasive Approach for Assessment of Children with Cardiomyopathy.

Non-invasive myocardial work (MW) by left ventricular (LV) pressure-strain loops (PSL) is a novel method for assessing myocardial function while adjusting for afterload, yet pediatric data remain lacking. The aims of this study were to investigate the different patterns of LV PSL and non-invasive MW in pediatric patients with hypertrophic (HCM) and dilated cardiomyopathy (DCM) and their association with exercise tolerance. We included 110 pediatric subjects (mean age, 13 ± 4years, 35 DCM, 40 HCM, and 35 healthy controls). Standard and speckle-tracking echocardiography were performed. LV PSLs were generated, and global work index (GWI), MW efficiency (GWE), constructive work (GCW), and wasted work (GWW) were compared between groups. Regression analysis was used to assess the influence of ventricular function, dimensions, wall thickness, and wall stress on MW and to predict the association between MW and VO2 max as a surrogate of exercise capacity. Patients with DCM had significantly lower GWI compared to controls (GWI 479.6 ± 263.0 vs 1610.1 ± 211.0, P < 0.005). GWE was significantly reduced in DCM (79.3 ± 7.9 vs 95.2 ± 1.3, P < 0.005) due to significantly reduced GCW and increased GWW. HCM patients had significant reduction in GWI and GWE from normal (1237.7 ± 449.1 vs 1610.1 ± 211.0, P = 0.001 and 89.6 ± 4.9 vs 95.2 ± 1.3, P < 0.005, respectively), although less severe than with DCM. In a multivariate regression analysis, GWE had the highest association with VO2 max in both cohorts (DCM: β = 0.68, P = 0.001, HCM: β = 0.71, P = 0.007). Non-invasively assessed myocardial work and LV PSLs provide novel insights into the mechanisms of dysfunction in pediatric patients with cardiomyopathy with good prediction of clinical status and thus hold promise to further explore myocardial mechanistic with clinical relevance in different disease entities.

Microvascular dysfunction in pediatric patients with SARS-COV-2 pneumonia: report of three severe cases.

The coronavirus 19 (COVID-19) pandemic has affected hundreds of millions of people worldwide: in most of cases children and young people developed asymptomatic or pauci-symptomatic clinical pictures. However authors have showed that there are some categories of childhood more vulnerable to COVID-19 infection such as newborns or children with comorbidities. We report for the first time to the best of our knowledge about microvascular dysfunction in three pediatric clinical cases who developed COVID-19 infections with need of pediatric critical care. We found that sublingual microcirculation is altered in children with severe COVID-19 infection.Our findings confirmed most of data already observed by other authors in adult population affected by severe COVID-19 infection, but with distinct characteristics than microcirculation alterations previous observed in a clinical case of MIS-C.However we cannot establish direct correlation between microcirculation analysis and clinical or laboratory parameters in our series, by our experience we have found that sublingual microcirculation analysis allow clinicians to report directly about microcirculation dysfunction in COVID-19 patients and it could be a valuable bedside technique to monitor thrombosis complication in this population.

Characteristics of Oral Dysfunction in Pediatric Patients with Malocclusion

Characteristics of Oral Dysfunction in Pediatric Patients with Malocclusion