Syndrome In Pediatric Patients Research Articles

Current Approach to the Evaluation and Management of Abdominal Compartment Syndrome in Pediatric Patients

Current Approach to the Evaluation and Management of Abdominal Compartment Syndrome in Pediatric Patients

Genetic Susceptibility to Hepatic Sinusoidal Obstruction Syndrome in Pediatric Patients Undergoing Hematopoietic Stem Cell Transplantation.

Sinusoidal obstruction syndrome (SOS) is a well-recognized and potentially life-threatening complication of hematopoietic stem cell transplantation (HSCT). SOS arises from endothelial cell damage and hepatocellular injury mostly due to the transplantation conditioning regimens but also to other patient, disease, and treatment-related factors. Understanding risk factors associated with the development of SOS is critical for early initiation of treatment or prophylaxis. The knowledge about genetic contribution is limited; few studies investigated so far selected a set of genes. To get more comprehensive insight in the genetic component, we performed an exome-wide association study using genetic variants derived from whole-exome sequencing. The analyses were performed in a discovery cohort composed of 87 pediatric patients undergoing HSCT following a busulfan-containing conditioning regimen. Eight lead single-nucleotide polymorphisms (SNPs) were identified after correction for multiple testing and subsequently analyzed in a validation cohort (n=182). Three SNPs were successfully replicated, including rs17146905 (P=.001), rs16931326 (P=.04), and rs2289971 (P = .03), located respectively in the UGT2B10, BHLHE22, and KIAA1715 genes. UGT2B10 and KIAA1715 were retained in a multivariable model while controlling for nongenetic covariates and previously identified risk variants in the GSTA1 promoter. The modulation of associations by conditioning regimens was noted; KIAA1715 was dependent on the intensity of the conditioning regimen, whereas the effect of UGT2B10 was equally applicable to all of them. Combined effect of associated loci was also observed (P=.00006) with a genotype-related SOS risk of 9.8. To our knowledge, this is the first study addressing the genetic component of SOS at an exome-wide level and identifying novel genetic variations conferring a higher risk of SOS, which might be useful for personalized prevention and treatment strategies.

Acquired von Willebrand Syndrome in Pediatric Extracorporeal Membrane Oxygenation Patients: A Single Institution's Experience.

1) Describe the prevalence of acquired von Willebrand syndrome in pediatric patients undergoing extracorporeal membrane oxygenation deemed to be at increased risk for the disease in our institution, 2) discuss the challenges of testing for acquired von Willebrand syndrome diagnosis, 3) describe the characteristics of the patient population found to have acquired von Willebrand syndrome and their outcomes, and 4) discuss the potential implications of acquired von Willebrand syndrome on bleeding complications. Retrospective chart review. PICU and cardiovascular ICU in a single institution. All PICU and cardiovascular ICU extracorporeal membrane oxygenation patients 0-18 years old who underwent screening for acquired von Willebrand syndrome between January 2014 and December 2016. Humate P administration to a small subset of acquired von Willebrand syndrome positive subjects. Laboratory data of identified patients were analyzed. The diagnosis of acquired von Willebrand syndrome was made based on decreased ristocetin cofactor activity to von Willebrand factor antigen ratio and/or abnormal multimer analysis. Clinical data were extracted from the chart and through the Pediatric Extracorporeal Membrane Oxygenation Outcome Registry to describe the demographics, comorbidities, and outcomes of this patient population. In the 2 years, 29 patients had laboratory testing performed for surveillance and in cases of clinical bleeding. Of these, 23 (79%) were positive by criteria. No significant difference in mortality rate was found between patients with acquired von Willebrand syndrome versus without. We also did not find a significant difference in the blood product utilization or bleeding complications between patients with acquired von Willebrand syndrome versus without. Humate P was administered in 39% of patients (9/23) who were positive for acquired von Willebrand syndrome, but no significant difference was seen in blood product utilization or bleeding complications between acquired von Willebrand syndrome patients receiving Humate P versus those who did not. Acquired von Willebrand syndrome is a common but under recognized disorder in pediatric extracorporeal membrane oxygenation patients. The clinical implications of this disorder on bleeding and its potential treatments are still unclear.

Analysis of 30 cases of inherited cardiac arrhythmia syndrome in children

Objective: To analyze and summarize the diagnosis and treatment experience of common inherited cardiac arrhythmia syndrome in pediatric patients, and explore the most appropriate therapy. Methods: A retrospective review identified 30 pediatric cases (19 males, 11 females) diagnosed with long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), hypertrophic cardiomyopathy (HCM), arrhythmogenc right ventricular cardiomyopathy (ARVC) from January 2008 to December 2018 in the Pediatric Cardiology Department, Guangdong Provincial People's Hospital. Data obtained included the diagnosis, treatment and follow-up outcome. Results: The most common inherited cardiac arrhythmia syndromes were LQTS (n=14) including 1 case with epilepsy, CPVT (n=5), HCM (n=7), ARVC (n=1), and BrS (n=3). Twenty-seven cases were admitted to hospital due to syncope, whereas the remaining 3 cases of BrS had not presented with syncope before admission. The average onset age of inherited arrhythmia was (10.0±3.3) years. Genetic testing was performed on 20 patients. The median follow-up time was 40 months. Among 15 patients who underwent implantable cardioverter defibrillator (ICD) and survived, 2 patients had frequent ICD discharge. One patient underwent radiofrequency ablation, and the other one received left cardiac sympathetic denervation and an increased ICD defibrillation threshold, and the number of ICD discharge was significantly reduced. Among 10 patients who received drug therapy, 4 patients including two patients who discontinued treatment without advices died. Two patients whose parents refused treatment died, 1 case diagnosed with unexplained sudden cerebral death, and the remaining 2 cases without indication for drug therapy survived without any treatment. Conclusions: Mortality rate is high in pediatric patients with inherited cardiac arrhythmia and syncope. The therapeutic effect of drugs are not satisfactory, ICD implantation is the most effective treatment to prevent sudden cardiac death currently, but the postoperative frequent discharge should be brought to the forefront and handled in time.

A Novel and Simple Algorithm Using Surface Electrocardiogram That Localizes Accessory Conduction Pathway in Wolff-Parkinson-White Syndrome in Pediatric Patients.

The location of the accessory pathway (AP) can be precisely identified on surface electrocardiography (ECG) in adults with Wolff-Parkinson-White (WPW) syndrome. However, current algorithms to locate the AP in pediatric patients with WPW syndrome are limited. To propose an optimal algorithm that localizes the AP in pediatric patients with WPW syndrome. From 1992 to 2016, 180 consecutive patients aged below 18 years with symptomatic WPW syndrome were included. After the exclusion of patients with non-descriptive electrocardiography (ECG), multiple APs, congenital heart diseases, non-inducible tachycardia, and those who received a second ablation, 104 patients were analyzed retrospectively. Surface ECG was obtained before ablation and evaluated by using previously documented algorithms, from which a new pediatric algorithm was developed. Previous algorithms were not highly accurate when used in pediatric patients with WPW syndrome. In the new algorithm, the R/S ratio of V1 and the polarity of the delta wave in lead I could distinguish right from the left side AP with 100% accuracy. The polarity of the delta wave of lead V1 could distinguish free wall AP from septal AP with an accuracy of 100% in left-side AP, compared to 88.6% in leads III and V1 for right-side AP. The overall accuracy was 92.3%. This simple, novel algorithm could differentiate left from right AP and septal from free wall AP in pediatric patients with WPW syndrome.

OR54: The Reve Study, Preliminary Results. A Monocentric Single-Arm Study to Characterize the Long-Term Safety and Efficacy of GLP-2 Analog (Teduglutide) in the Management of Short Bowel Syndrome in Pediatric Patients on Home-Parenteral Nutrition

OR54: The Reve Study, Preliminary Results. A Monocentric Single-Arm Study to Characterize the Long-Term Safety and Efficacy of GLP-2 Analog (Teduglutide) in the Management of Short Bowel Syndrome in Pediatric Patients on Home-Parenteral Nutrition

Incidence, Risk Factors, and Timing of Compartment Syndrome in Pediatric Patients with Supracondylar Fractures of the Humerus

Incidence, Risk Factors, and Timing of Compartment Syndrome in Pediatric Patients with Supracondylar Fractures of the Humerus

Relationship between adenoid hypertrophy and sleep apnea in children

It was decided to organize this work on the assumption that it is necessary for all dental surgeons to familiarize themselves with the structure and alterations in oral anatomy from childhood, with attention to the possibility of adenoid hypertrophy (AH) and obstructive sleep apnea syndrome (OSAS) to be present, and these professionals can assist in orthodontic treatment. The objective of this literature review was to observe if there is a correlation between adenotonsillar hypertrophy and sleep apnea in children and which treatment were adopted. The following databases were searched: LILACS, MEDLINE, PubMed, Cochrane, Web of Science and SciELO, from January 2014 to August 2018. It is concluded that there is a correlation between hypertrophy of the adenoid glands and apnea of the sleep in children. Hypertrophy presents as the main cause of obstructive sleep apnea syndrome in pediatric patients and its treatment may be surgical, removal of the adenoid, drug or orthodontic glands.

Current Approach to the Evaluation and Management of Acute Compartment Syndrome in Pediatric Patients.

Acute compartment syndrome is an emergent condition caused by increased pressure within a closed compartment. The most common etiology is fractures, but there a number of atraumatic causes cited in the literature. Acute compartment syndrome occurs most frequently in the anterior compartment of the lower leg, followed by the volar forearm. Patients may present with severe pain, pain with passive stretch, swelling, paresthesias, numbness, weakness, decreased pulses, and delayed capillary refill. No finding in isolation can exclude the diagnosis. Direct measurement of the intracompartmental pressure is the most important diagnostic test. Treatment involves removal of compressive dressings and surgical consultation for emergent fasciotomy.

Current Approach to the Evaluation and Management of Acute Compartment Syndrome in Pediatric Patients.

Current Approach to the Evaluation and Management of Acute Compartment Syndrome in Pediatric Patients.

Quantitative Dynamic Thoracic MRI: Application to Thoracic Insufficiency Syndrome in Pediatric Patients.

Background Available methods to quantify regional dynamic thoracic function in thoracic insufficiency syndrome (TIS) are limited. Purpose To evaluate the use of quantitative dynamic MRI to depict changes in regional dynamic thoracic function before and after surgical correction of TIS. Materials and Methods Images from free-breathing dynamic MRI in pediatric patients with TIS (July 2009-August 2015) were retrospectively evaluated before and after surgical correction by using vertical expandable prosthetic titanium rib (VEPTR). Eleven volumetric parameters were derived from lung, chest wall, and diaphragm segmentations, and parameter changes before versus after operation were correlated with changes in clinical parameters. Paired analysis from Student t test on MRI parameters and clinical parameters was performed to detect if changes (from preoperative to postoperative condition) were statistically significant. Results Left and right lung volumes at end inspiration and end expiration increased substantially after operation in pediatric patients with thoracic insufficiency syndrome, especially right lung volume with 22.9% and 26.3% volume increase at end expiration (P = .001) and end inspiration (P = .002), respectively. The average lung tidal volumes increased after operation for TIS; there was a 43.8% and 55.3% increase for left lung tidal volume and right lung tidal volume (P < .001 for both), respectively. However, clinical parameters did not show significant changes from pre- to posttreatment states. Thoracic and lumbar Cobb angle were poor predictors of MRI tidal volumes (chest wall, diaphragm, and left and right separately), but assisted ventilation rating and forced vital capacity showed moderate correlations with tidal volumes (chest wall, diaphragm, and left and right separately). Conclusion Vertical expandable prosthetic titanium rib operation was associated with postoperative increases in all components of tidal volume (left and right chest wall and diaphragm, and left and right lung tidal volumes) measured at MRI. Clinical parameters did not demonstrate improvements in postoperative tidal volumes. © RSNA, 2019 Online supplemental material is available for this article. See also the editorial by Paltiel in this issue.

Neurodevelopmental outcomes of the West syndrome in pediatric patients: The first report from the Middle-East

BackgroundThis study aimed to investigate the clinical characteristics and neurodevelopmental outcomes of children with West syndrome (WS) by using the Bayley-III scale of infant development, as the first report from the Middle-East. MethodsBetween January 2013 and February 2016, we prospectively enrolled 67 consecutive patients with a confirmed diagnosis of WS from Isfahan, Iran. Cognition, language and motor outcomes of the studied subjects were evaluated with the Bayley Scales of Infant and Toddler Development-Third Edition (Bayley-III). ResultsOverall, 67 cases, including 34 (50.7%) boys and 33 (49.3%) girls (a male/female ratio of 1.03), were enrolled for the study. The mean age was 26.7 ± 12.9 months. Among the subjects, 50 (74.6%) patients had symptomatic WS, and 17 (25.4%) patients had cryptogenic WS. “Severe delay” was found in 76.9% of the patients regarding cognitive evaluation, 67.7% for language and communication abilities, and, 81.5% for motor function. The patients with cryptogenic WS were significantly more likely to have more favorable outcomes in motor (p = 0.035), cognitive (p = 0.035) and receptive language (p = 0.043) in comparison to those who had symptomatic WS. The patients with controlled seizures were significantly more likely to have more favorable outcomes in motor (p = 0.027) and cognition (p = 0.011) as compared to those with uncontrolled seizures. ConclusionWS was associated with poor neurodevelopmental outcome in our study. Severe developmental delay was associated with two major factors: (i) presence of a specific underlying abnormality (symptomatic WS) and(ii) persistent seizures as a result of the former.

Identification of Low Cardiac Output Syndrome at the Bedside: A Pediatric Cardiac Intensive Care Unit Survey

Low cardiac output syndrome is a transient constellation of signs and symptoms that indicate the heart's inability to supply sufficient oxygen to tissues and end-organs to meet metabolic demand. Because the term lacks a standard clinical definition, the bedside diagnosis of this syndrome can be difficult. To evaluate concordance among pediatric cardiac intensive care unit nurses in their identification of low cardiac output syndrome in pediatric patients after cardiac surgery. An anonymous survey was distributed to 69 pediatric cardiac intensive care unit nurses. The survey described 10 randomly selected patients aged 6 months or younger who had undergone corrective or palliative cardiac surgery at a freestanding children's hospital in a tertiary academic center. For each patient, data were presented corresponding to 5 time points (0, 6, 12, 18, and 24 hours postoperatively). The respondent was asked to indicate whether the patient had low cardiac output syndrome (yes or no) at each time point on the basis of the data presented. The response rate was 46% (32 of 69 nurses). The overall Fleiss k value was 0.30, indicating fair agreement among raters. When the results were analyzed by years of experience, agreement remained only slight to fair. Regardless of years of experience, nurses have difficulty agreeing on the presence of low cardiac output syndrome. Further research is needed to determine whether the development of objective guidelines could improve recognition and facilitate communication between the pediatric cardiac intensive care unit nurse and the medical team.

The prevalence of Klippel-Feil syndrome in pediatric patients: analysis of 831 CT scans.

To evaluate the prevalence of Klippel-Feil syndrome (KFS) in pediatric patients obtaining cervical CT imaging in the emergency room (ER). We evaluated CT scans of the cervical spine of pediatric patients treated in the ER of a Level I Trauma Center from January 2013 to December 2015. Along with analysis of the CT scans for KFS, the following demographics were collected: age, sex, race and ethnicity. Mechanism of injury was also established for all patients. If KFS was present, it was classified using Samartzis classification as type I (single level fusion), type II (multiple, noncontiguous fused segments) or type III (multiple, contiguous fused segments). Of the 848 cervical CTs taken for pediatric ER patients during the study period, 831 were included. Of these patients, 10 had KFS, a prevalence of 1.2%. According to Samartzis classification, 9 were type I and 1 type III. The average age of patients with KFS was 16.02 years (10-18 years), with 8 males (80%) and 2 females (20%). Three had congenital fusions at vertebral levels C2-C3, two at C3-C4, three at C5-C6, one at C6-C7, and one with multiple levels of cervical fusion. The prevalence of KFS amongst 831 pediatric patients, who underwent cervical CT imaging over a 3-year period, was 1.2% (approximately 1 in 83). The most commonly fused spinal levels were C2-C3 and C5-C6. The prevalence of KFS in our study was higher than previously described, and thus warrants monitoring.

Vitamin D Insufficiency in Overweight and Obese Children and Adolescents.

Excessive body weight and obesity in childhood and adolescence are becoming more and more important unfavorable factors that entail extremely adverse consequences and require close attention of physicians of any specialty. Along with the high prevalence of obesity and metabolic syndrome in pediatric patients, children and adolescents in the majority of countries are diagnosed with vitamin D deficiency. Among the non-calcaemic effects of vitamin D, a significant role is played by its impact on the hormonal regulation of glucose metabolism and the synthesis of adipokines by fat tissue. The review presents literature data indicative of a close pathogenic relationship between vitamin D insufficiency and impaired tissue insulin sensitivity. It demonstrates the role of vitamin D insufficiency in immune reactions resulting in development of subclinical inflammation in fat tissue infiltrated with macrophages and lymphocytes. It also shows the role of adipokines, immune system cells and pro-inflammatory cytokines produced by them in the pathogenesis of obesity, as well as the function of vitamin D as an endocrine and paracrine regulator of the process of inflammation in adipose tissue. The relationships between the principal adipokines (leptin, adiponectin, resistin) are revealed in the presence of normal vitamin D content and in vitamin D deficiency. The carbohydrate and lipid metabolism parameters in overweight children and adolescents with vitamin D insufficiency are analyzed. A high prevalence of vitamin D insufficiency in overweight and obese children and adolescents (increasing along with the severity of obesity) is demonstrated. The review also presents the current recommendations for the correction of vitamin D insufficiency and underlines the need for higher cholecalciferol doses to achieve serum calcifediol targets in overweight and obese children and adolescents.

A case series of cyclic vomiting syndrome in paediatric patients at a tertiary care hospital in Chennai, Tamil Nadu, India

Background: Cyclic vomiting syndrome is an increasingly recognized disorder with sudden, repeated episodes of severe nausea, vomiting, and physical exhaustion that occur with no apparent cause. It is more common in children than adults. However, we don’t often see a diagnosis of cyclic vomiting syndrome being made. Hereby we report 7 cases of cyclical vomiting.Methods: This is a retrospective study. Medical records of 5 years were review and consecutive paediatric patients discharged with a diagnosis of cyclic vomiting syndrome were identified, data collected and analysed.Results: Total of 7 cases were found, all of which were adolescents. The mean age was 13.28. 57.14% (4) were females and 42.86% (3) were boys. 42.86% (3) presented with complications. 14.28% (1) had an association with menstrual cycles and 14.28% (1) had an association with psychological stress.Conclusions: Cyclic vomiting syndrome should be kept in mind when a child presents with multiple episodes of vomiting, especially when repeated admissions have been made for the same. Early diagnosis and treatment can improve the quality of life.

Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support.

Bleeding signs can become life-threatening complications in patients on mechanical circulatory support (MCS). Clinical phenotyping and comprehensive analyses of the cause of bleeding are, therefore, essential, especially when risk-stratifying patients during MCS workup. We conducted coagulation analyses and determined von Willebrand factor (VWF) parameters in a paediatric cohort on temporary extracorporeal life support, extracorporeal membrane oxygenation or long-term ventricular assist device support. We carried out an observational single-centre study including 30 children with MCS (extracorporeal life support, n = 13; extracorporeal membrane oxygenation, n = 5; and ventricular assist device, n = 12). We also assessed the acquired von Willebrand parameters of each study participant: collagen binding capacity (VWF:CB), the ratio of collagen-binding capacity to VWF antigen (VWF:CB/VWF:Ag) and high-molecular-weight VWF multimers. We also documented bleeding events, transfusion requirement, haemolysis parameters and surgical interventions. All children developed AVWS (acquired von Willebrand syndrome) during MCS, usually during the early postoperative course. They presented no AVWS after device explantation. We detected a loss of high-molecular-weight VWF multimers, decreased VWF:CB/VWF:Ag ratios and reduced VWF:CB levels. Twenty of the 30 patients experienced bleeding complications; approximately 53% of them required surgical revision. There were no deaths due to bleeding during support. The AVWS prevalence in paediatric patients on MCS is 100% regardless of the types of devices tested in this study. The bleeding propensity of AVWS patients widely varies.

A cost comparison of rasburicase versus dialysis in the management of children with acute leukaemia and lymphoma at a South African centre

Background: Tumour lysis syndrome is a common complication of haematological malignancies and has historically been managed with hyperhydration, urine alkalinisation and allopurinol with renal dialysis reserved for patients in acute renal failure. Rasburicase has been shown to drastically reduce the need for dialysis; however, its use is limited in developing countries owing to its cost and availability.Aim: This retrospective analysis aimed to compare the cost to state per patient of rasburicase compared to dialysis in the management and prevention of tumour lysis syndrome in paediatric patients presenting with haematological malignancies admitted to Red Cross War Memorial Children’s Hospital (RCWMCH).Setting: Red Cross War Memorial Children’s Hospital.Methods: Patients from two consecutive 35 month periods, before and after the availability of rasburicase at RCWMCH, were grouped according to treatment modality, and the cumulative costs of hospitalisation, dialysis and drug administration were compared.Results: The groups were comparable in mean age and gender. The mean total length of hospital stay was 10.04 days shorter for the rasburicase group than the dialysis group with the average cost per patient in the rasburicase group being R40 989.64 lower than the dialysis group.Conclusion: The use of rasburicase results in a significant per patient cost saving when compared to dialysis, which often requires intensive care admission, and results in extended hospitalisation. The study supports the continued use of rasburicase as an essential adjunct in the management and prevention of tumour lysis syndrome, reaffirming its use as a cost-effective and efficient drug.

Posterior Reversible Encephalopathy in Nephrotic Syndrome: Case Report

: Posterior Reversible Encephalopathy Syndrome is a potentially life-threatening complication of nephrotic syndrome in pediatric patients. It is characterized by the presence of vasogenic edema in the parietal and occipital region of the brain that leads to the acute and sudden onset of unconsciousness, epileptic episodes, headache and visual disturbances. Multiple factors can predispose an individual with nephrotic syndrome to PRES such as- uncontrolled hypertension, administration of drugs (cyclosporine, tacrolimus), reduced serum albumin levels, anascara, disturbed body fluid status and renal insufficiency. PRES in pediatric patients with the nephrotic syndrome has been rarely reported. Here, we report a case of a 13-year-old boy with nephrotic syndrome who was presented with cellulitis in left knee and developed acute proteinuria and review of literature on PRES occurrence in nephrotic syndrome.Key words: Posterior reversible encephalopathy syndrome, Nephrotic syndrome, Pediatric, Tacrolimus, Calcineurin inhibitor.

Mercury Intoxication as a Rare Cause of Membranous Nephropathy in a Child

In adults, membranous nephropathy is the second most common cause of nephrotic syndrome. In contrast, minimal change disease and focal segmental glomerulosclerosis constitute the most common forms of nephrotic syndrome in children, while membranous nephropathy accounts for<5% of cases. In adults, causes of membranous nephropathy include autoantibodies directed against phospholipase A2 receptor and thrombospondin type 1 containing 7A, various infections, environmental toxicities, autoimmune disorders, malignancies, and other secondary forms. The most common causes of secondary membranous nephropathy in children are infections, autoimmune diseases, and neoplasia. We discuss an unusual presentation of new-onset membranous nephropathy due to mercury toxicity in a 14-year-old male with reflux nephropathy. This case underscores the importance of a high index of suspicion for uncommon causes of nephrotic syndrome in pediatric patients with membranous nephropathy.