Considerations For Pediatric Patients Research Articles

Derivation of a clinical decision rule for termination of resuscitation in non-traumatic pediatric out-of-hospital cardiac arrest

AimPrehospital termination of resuscitation (ToR) rules are used to predict medical futility in adult out-of-hospital cardiac arrest (OHCA), however, the available evidence for pediatric patients is limited. The primary aim of this study is to derive a Pediatric Termination of Resuscitation (PToR) prediction rule for use in pediatric non-traumatic OHCA patients. MethodsWe analyzed a retrospective cohort of pediatric OHCA patients within the CARES database over a 10-year period (2013 – 2022). We split the dataset into training and test datasets and fit logistic regressions with Least Absolute Shrinkage and Selection Operator (LASSO) to select predictor variables and estimate predictive test characteristics for the primary outcome of death and a secondary composite outcome of death or survival to hospital discharge with unfavorable neurologic outcome. ResultsWe analyzed a sample of 21,240 children where 2,326 (11.0%) survived to hospital discharge, and 1,894 (8.9%) survived to hospital discharge with favorable neurologic outcome. We derived a PToR rule for death demonstrating a specificity of 99.1% and a positive predictive value (PPV) of 99.8% and a PToR rule for death or survival with poor neurologic outcome with a specificity of 99.7% and PPV of 99.9% within the test dataset. ConclusionWe derived a clinical prediction rule with high specificity and positive predictive value in prehospital settings utilizing Advanced Life Support (ALS) providers which may inform termination of resuscitation considerations in pediatric patients. Further prospective and validation studies will be necessary to define the appropriateness and applicability of these PToR criteria for routine use.

Dual novel variants in CD40 leading to hyper IgM syndrome: a case report of a school-aged female with new-onset recurrent pneumonia

Introduction: Hyper IgM (HIGM) syndrome is an inborn error of immunity (IEI) that occurs due to defects in immunoglobulin class switch recombination (Ig-CSR). HIGM syndrome typically presents with recurrent infections in early childhood, and is often characterized on investigation with decreased IgG, IgA, and IgE titres, alongside normal or elevated IgM. A common cause of HIGM syndrome is a disruption to the CD40-CD40 ligand (CD40L) interaction that triggers Ig-CSR, of which variants in CD40 are much rarer than those in CD40L. We present a case of an 11-year-old female with HIGM syndrome caused by two novel variants of in the CD40 gene. Aim: To describe a case report of an eleven-year-old female with HIGM syndrome presenting with recurrent pneumonia. Methods: Data was collected retrospectively from the patient’s medical records. Laboratory investigations included quantitative immunoglobulins, quantitative B and T cell subsets, genetic testing using a primary immunodeficiency panel, and a functional assay for CD40 expression. Results: The proband is an 11-year-old female, who presented with recurrent pneumonia, otitis, and septic arthritis. Investigations revealed neutropenia, low IgA, elevated IgM and normal IgG, along with absent vaccine responses. She was identified to harbour two novel variants in CD40: an intronic variant c.52-13A>G p.(?) and a missense variant c.466T>C p.(Ser156Pro). Functional assay indicated low expression of CD40 compared to healthy control, confirming the diagnosis of CD40 deficiency. Conclusion: Class switch defects, such as CD40 deficiency, are rare but significant diagnoses within the spectrum of IEI. This case demonstrates that despite the absence of some clinical red flags for immunodeficiency in infancy, IEIs remain an important consideration in pediatric patients regardless of age. Increasing clinical awareness of IEI will lead to earlier diagnoses, initiation of appropriate treatment, and prevention of potential complications. Statement of novelty: We describe a patient with a late presentation of hyper IgM syndrome due to two novel variants in the CD40 gene, thus expanding the spectrum of CD40 gene variants.

Digital subtraction angiography of intrahepatic portal vein as the key visualization for mesoportal shunting in children with extrahepatic portal vein obstruction

Аn early consideration in pediatric patients with extrahepatic portal vein obstruction (EHPVO) is to be given for mesoportal shunt. A careful investigation is required to prove intrahepatic portal system patency. Conventional noninvasive imaging is not adequately reliable in assessing patency of the intrahepatic portal venous system. Retrograde portography in children brings additional invasive procedure. Antegrade, direct intraoperative digital subtraction angiography for mesoportal shunt feasibility assessment is poorly presented in literature. Aim - to improve the rate of success of mesoportal shunting in children with EHPVO by analyzing our own experience in intraoperative digital subtraction angiography of the intrahepatic branches of the portal vein for the final assessment of the mesoportal shunting feasibility. Materials and methods. 7 pediatric patients with EHPVO were selected for the study. Angiographies and surgeries in selected patients were performed within single center in a period from May 2022 to July 2023. Mean follow up of successful mesoportal shunting was 12.38±1.46 months. Results. All patients were males. Men age at surgery was 8.71±1.72 years. 5 (71.4%) patients manifested bleeding episodes as the first sign of portal hypertension. In all patients ultrasound revealed splenomegaly and suspected portal hypertension for reduced volumetric portal flow. All 7 patients had high grade. Liver function was normal in all patients, and in none thrombophilia was confirmed. CT scans suspected patent left intrahepatic branch (Rex-zone). Digital subtraction angiography approved mesoportal shunt feasibility in 4 (57.1%) patients. The follow up period was 13.5±2.9 months. Conclusions. Digital subtraction angiography of intrahepatic portal vein is effective visualization method to achieve with radiologic evidence of intrahepatic portal branches patency and make the decision on mesoportal shunting when favorable anatomy proved. 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.

Pediatric Cancer Drug Development: Leveraging Insights in Cancer Biology and the Evolving Regulatory Landscape to Address Challenges and Guide Further Progress.

The discovery and development of anticancer drugs for pediatric patients have historically languished when compared to both past and recent activity in drug development for adult patients, notably the dramatic spike of targeted and immune-oncology therapies. The reasons for this difference are multifactorial. Recent changes in the regulatory landscape surrounding pediatric cancer drug development and the understanding that some pediatric cancers are driven by genetic perturbations that also drive disparate adult cancers afford new opportunities. The unique cancer-initiating events and dependencies of many pediatric cancers, however, require additional pediatric-specific strategies. Research efforts to unravel the underlying biology of pediatric cancers, innovative clinical trial designs, model-informed drug development, extrapolation from adult data, addressing the unique considerations in pediatric patients, and use of pediatric appropriate formulations, should all be considered for efficient development and dosage optimization of anticancer drugs for pediatric patients.

Clinical Outcomes, Blood Utilization, and Ethical Considerations for Pediatric Patients in a Bloodless Medicine and Surgery Program.

Pediatric patients requesting bloodless care represent a challenging clinical situation, as parents cannot legally refuse lifesaving or optimal interventions for their children. Here, we report clinical outcomes for the largest series of pediatric inpatients requesting bloodless care and also discuss the ethical considerations. We performed a single-institution retrospective cohort study assessing 196 pediatric inpatients (<18 years of age) who requested bloodless care between June 2012 and June 2016. Patient characteristics, transfusion rates, and clinical outcomes were compared between pediatric patients receiving bloodless care and those receiving standard care (including transfusions if considered necessary by the clinical team) (n = 37,271). Families were informed that all available measures would be undertaken to avoid blood transfusions, although we were legally obligated to transfuse blood if the child's life was threatened. The primary outcome was composite morbidity or mortality. Secondary outcomes included percentage of patients transfused, individual morbid events, length of stay, total hospital charges, and total costs. Subgroup analyses were performed after stratification into medical and surgical patients. Of the 196 pediatric patients that requested bloodless care, 6.1% (n = 12) received an allogeneic blood component, compared to 9.1% (n = 3392) for standard care patients ( P = .14). The most common indications for transfusion were perioperative bleeding and anemia of prematurity. None of the transfusions were administered under a court order. Overall, pediatric patients receiving bloodless care exhibited lower rates of composite morbidity compared to patients receiving standard care (2.6% vs 6.2%; P = .035). There were no deaths in the bloodless cohort. Individual morbid events, length of stay, and total hospital charges/costs were not significantly different between the 2 groups. After multivariable analysis, bloodless care was not associated with a significant difference in composite morbidity or mortality (odds ratio [OR], 0.37; 95% confidence interval [CI], 0.12-1.11; P = .077). Pediatric patients receiving bloodless care exhibited similar clinical outcomes compared to patients receiving standard care, although larger studies with adequate power are needed to confirm this finding. There were no mortalities among the pediatric bloodless cohort. Although a subset of our pediatric bloodless patients received an allogeneic transfusion, no patients required a court order. When delivered in a collaborative and patient-centered manner, blood transfusions can be safely limited among pediatric patients.

Basics for Pediatric Brain Tumor Imaging: Techniques and Protocol Recommendations.

This review provides an overview of the current state of pediatric brain tumor imaging, emphasizing the role of various imaging sequences and highlighting the advantages of standardizing protocols for pediatric brain tumor imaging in diagnosis and treatment response evaluation. Basic anatomical sequences such as pre- and post-contrast 3D T1-weighted, T2-weighted, fluid-attenuated inversion recovery, T2*-weighted, and diffusion-weighted imaging (DWI), are fundamental for assessing tumor location, extent, and characteristics. Advanced techniques like DWI, diffusion tensor imaging, perfusion imaging, magnetic resonance spectroscopy, and functional MRI offer insights into cellularity, vascularity, metabolism, and function. To enhance consistency and quality, standardized protocols for pediatric brain tumor imaging have been recommended by expert groups. Special considerations for pediatric patients, including the minimization of anesthesia exposure and gadolinium contrast agent usage, are essential to ensure patient safety and comfort. Staying up-to-date with diagnostic imaging techniques can contribute to improved communication, outcomes, and patient care in the field of pediatric neurooncology.

Fanconi’s Anemia: A Review and Dental Considerations in Pediatric Patients

Fanconi’s anemia (FA) is an autosomal recessive disorder associated with many systemic problems. A thorough diagnosis of FA in dental clinical practice is important because this disease can compromise the growth and development of the entire stomatognathic system. However, the literature shows that each case has its own peculiarities, which hinder the diagnosis of FA. The present article presents manifestations of FA together with the management of pediatric dental problems associated with FA.

Spitz Nevus in a Nevus Spilus: Management Considerations in Pediatric Patients

Spitz Nevus in a Nevus Spilus: Management Considerations in Pediatric Patients

Psychiatric Considerations in Pediatric Patients With Brain Tumors.

Objective: Adolescents diagnosed with brain or central nervous system tumors face elevated levels of depression and anxiety compared to the general pediatric population. The objective of this review was to explore factors associated with elevated depression and anxiety symptoms in these patients, as well as various currently recommended interventions.Data Sources: Articles were searched in PubMed and filtered to select for the following MeSH keywords: brain neoplasm, mental health, behavioral symptom, mental disorder AND (child* OR adolescen* OR teen* OR youth OR pediatr*). Articles were limited to those published in English between 2000 and 2020.Study Selection: A total of 1,537 articles were retrieved, and 36 studies were reviewed. Articles were selected if they pertained to human subjects, specifically adolescents aged ≤ 18 years. Additionally, only articles about depression and anxiety were included. Symptoms of depression and anxiety before a brain tumor diagnosis or as symptoms of a possible brain tumor were excluded.Data Extraction: Articles were screened by title and abstract as they pertained to depression and anxiety in patients diagnosed with brain tumors in adolescence. Data extracted included information about risk factors associated with depression and anxiety, interventions, and pharmacologic considerations.Results: Factors associated with increased depressive and anxiety symptoms include intensity of cancer treatment, female sex, and brain tumor diagnosis. Pediatric brain tumor patients also experience elevated antidepressant prescription rates. When treating depression and anxiety in these patients, it is also important to focus on multifaceted interventions like behavioral and communication therapies, as the nature of brain tumors can affect psychological, social, and cognitive abilities.Conclusions: Depression and anxiety can have long-lasting effects on pediatric brain tumor patients and put them at risk for developing other psychosocial problems. Therefore, it is important to recognize factors contributing to depression and anxiety as well as potential interventions.

Perioperative Considerations for Pediatric Patients With Congenital Heart Disease Presenting for Noncardiac Procedures: A Scientific Statement From the American Heart Association

Continuous advances in pediatric cardiology, surgery, and critical care have significantly improved survival rates for children and adults with congenital heart disease. Paradoxically, the resulting increase in longevity has expanded the prevalence of both repaired and unrepaired congenital heart disease and has escalated the need for diagnostic and interventional procedures. Because of this expansion in prevalence, anesthesiologists, pediatricians, and other health care professionals increasingly encounter patients with congenital heart disease or other pediatric cardiac diseases who are presenting for surgical treatment of unrelated, noncardiac disease. Patients with congenital heart disease are at high risk for mortality, complications, and reoperation after noncardiac procedures. Rigorous study of risk factors and outcomes has identified subsets of patients with minor, major, and severe congenital heart disease who may have higher-than-baseline risk when undergoing noncardiac procedures, and this has led to the development of risk prediction scores specific to this population. This scientific statement reviews contemporary data on risk from noncardiac procedures, focusing on pediatric patients with congenital heart disease and describing current knowledge on the subject. This scientific statement also addresses preoperative evaluation and testing, perioperative considerations, and postoperative care in this unique patient population and highlights relevant aspects of the pathophysiology of selected conditions that can influence perioperative care and patient management.

Targeted muscle reinnervation in the pediatric population

Extremity amputation due to traumatic or oncologic etiology in the pediatric population confers reduced quality of life, psychosocial trauma, and chronic pain in the form of neuroma, and residual and/or phantom limb pain. Several techniques have been described with inconsistent efficacy, yet little is available prophylactically. The advent of targeted muscle reinnervation (TMR) has shown to have strong outcomes for this purpose in adults, affording aberrant nerves an adequate growth path. This study aims to describe our experiences with using TMR in a previously undescribed pediatric cohort. TMR consisted of isolation of major residual nerves following surgical amputation. Residual nerves undergo microsurgical coaptation to motor nerves innervating remaining regional stump-muscles. Patients are followed post-operatively for development of pain and prosthetic usage. Two subjects were identified. The first was a 17-year-old female who suffered from fulminant liver failure and subsequent bilateral lower extremity necrosis necessitating bilateral below knee amputation (BKA). She concomitantly underwent TMR to both limbs. The second was a 12-year-old male with Ewing's sarcoma of the femur who previously underwent femoral resection and allograft/autograft reconstruction. On recurrence, above knee amputation (AKA) was conducted with concomitant TMR. On 16- and 12-month follow-up, respectively, both patients demonstrate use of prosthetic devices and report no pain, without the usage of pain medications. TMR is feasible in the pediatric population. This intervention may warrant consideration in pediatric patients who are undergoing amputation for a variety of etiologies and can effectively serve to mitigate risk of neuroma formation and residual or phantom limb pain. • Pediatric targeted muscle reinnervation has yet to be described, and this article presents a proof-of-concept study. • Targeted muscle reinnervation may have success in the lower extremity when undertaken in the pediatric population. • Larger populations will uncover further applications.

A-266 Neuropsychological Profile of a 5-Year-Old Boy with Gould Syndrome

Abstract Objective: Gould syndrome (COL4A1/A2-related disorders) is an extremely rare, genetic disorder characterized by cerebral vasculature defects, ocular dysgenesis, myopathy, and cerebral cortical abnormalities. Many individuals with COL4A1 mutations exhibit seizures, stroke, visual field defects, and hydrocephalus. To date, research regarding neuropsychological sequelae of Gould syndrome has been sparse. Therefore, this presentation aims to present clinical considerations for pediatric patients with Gould syndrome. Method: Patient is a 5-year-old Hispanic boy born at 35-weeks following perinatal stroke, pre-term birth, and suspected cyanosis. After birth, genetic testing revealed a COL4A1p.G1234R gene mutation (i.e., Gould syndrome). Patient’s medical history is also notable for cerebral palsy, epilepsy, encephalopathy, hypertonia, global developmental delays with language regression, and attention-deficit/hyperactivity disorder. Previous brain MRIs have found decreased white matter density in both cerebral hemispheres. Abbreviated neuropsychological testing was completed during an inpatient pre-surgical epilepsy evaluation. Patient was non-verbal. Results: Non-verbal intellectual ability was Below Average (IQ=74). Receptive language was Average (SS=93). Further, bilateral fine-motor speed and dexterity and visual motor integration were Exceptionally Low. Standardized questionnaires revealed ongoing concerns related to attention, hyperactivity, and poor adaptive functioning. Diagnostic impressions included Global Developmental Delay. Conclusion: Gould syndrome can include a variety of neurological sequelae depending on neurological defects and incidents. These findings are consistent with the “early vulnerability” position of brain development, which proposes that earlier age at brain insult may result in greater impairments in cognitive functioning (Jacomb et al., 2018). Therefore, early intervention and targeted long-term interventions appears imperative to improve function outcomes for pediatric patients with Gould syndrome.

Antibody waning after immunosuppressive chemotherapy and immunomodulators, re-immunization considerations in pediatric patients with malignancy and chronic immune thrombocytopenic purpura

IntroductionImmunosuppressive chemotherapy increase the risk of vaccine-preventable infectious diseases in children; nevertheless, chemotherapy may result in delay or miss updated immunization schedules. The predictable antibody waning after incomplete primary immunization series may be intensified at the end of chemotherapy. This study aimed to investigate post-chemotherapy vaccine immunity waning at the end of immunosuppressive therapy in children with malignancy and hematologic disorders.Materials and methodsChildren with malignancies and hematologic disorders including chronic immune thrombocytopenic purpura (ITP) younger than 18 years old were enrolled from September 2015 to August 2019. Eligible patients who completed their treatment protocol for at least 6 months were recruited. The patient information, including sex, age at the date of diagnosis, number of chemotherapy sessions, underlying disease, and vaccination history, was taken by chart review using predefined questionnaires. The patient’s blood samples were obtained, and serum IgG antibody titer checked against diphtheria, tetanus, hepatitis B virus (HBV), mumps, measles, and rubella (MMR) were measured by enzyme-linked immunosorbent assay (ELISA).Results110 children receiving immunosuppressive chemotherapy were recruited. Forty-four (40%) of the children tested were girls and 66 (60%) were boys. The mean age of patients was 5.5 years with a range of 2 to 13 years. Of 110 studied children, 27.3% were seronegative for all antibodies. On average, patients undergo 19 episodes of chemotherapy. The mean chemotherapy sessions were significantly greater in children who were seronegative for all tested antibodies (mean: 36.2, 95% CI 33.16 to 39.24, p-value < 0.001). No statistically significant differences were observed regarding the patient’s sex and age between the seropositive and seronegative groups (p-value 0.513 and 0.060, respectively). Based on Poisson regression model analysis, the female gender was associated with 37% lower odds of seronegativity (incidence rate ratio (IIR): 0.63; [95% conf. interval: 0.39 to 1.01, p-value: 0.55]), while chemotherapy sessions 30 or more was associated with significant odds of seronegativity for all tested vaccines (IIR: 25.41; [95% conf. interval: 6.42 to 100.57, p-value < 0.001]).ConclusionOur results reemphasized planned catchup immunization in children undergoing immunosuppressive chemotherapy for malignancy, especially against tetanus, diphtheria, and hepatitis B at least 6 months after the end of chemotherapy sessions.

Pediatric subcutaneous allergen immunotherapy.

Subcutaneous immunotherapy (SCIT) is a widely used therapy for allergic rhinitis and asthma. It is a useful adjunct to standard medical management of these conditions that can lead to long-term benefits and possible resolution of symptoms. The benefits of SCIT, particularly for children, include avoiding prolonged use and side effects from medications, preventing new aeroallergen sensitizations, and reducing the risk of developing asthma. The primary risks of SCIT include local and systemic reactions. Standard schedules for SCIT include advancing through multiple doses usually in four vials (diluted to 1:1000) on a weekly basis; however, there are benefits of using accelerated schedules, especially for children who need to coordinate school and parent work schedules. Special considerations for pediatric patients include fear of needles, avoiding discomfort with injections, consent, optimal injection scheduling, and difficulty communicating about symptoms during reactions in very young children. Overall, SCIT can be a safe and beneficial therapy for children.

Respiratory physiology at high altitude and considerations for pediatric patients.

Over 150million people, including many children, live at high altitude (>2500m) with the majority residing in Asia and South America. With increases in elevation, the partial pressure of oxygen (pO2) is reduced, resulting in a hypobaric hypoxic environment. Fortunately, humans have evolved adaptive processes which serve to acclimate the body to such conditions. These mechanisms, occurring along a specific time course, result in tachypnea, tachycardia, diuresis, and hematopoiesis, and a shift in the oxygen dissociation curve favoring an increased affinity for oxygen. These, along with other physiological effects, including increased pulmonary vascular resistance, alterations in cerebral blood flow, and changes in sensitivity to opioids, must be considered when administering anesthesia at high altitudes. Susceptible individuals or those who ascend too quickly may outpace the body's ability to acclimate resulting in one or more forms of high-altitude sickness ranging from the milder acute mountain sickness to the more serious conditions of high-altitude pulmonary edema and cerebral edema, either of which can be life-threatening if not promptly recognized and treated. Since the adaptive mechanisms for acclimatization greatly affect the cardiopulmonary systems, patients with underlying health issues such as sleep apnea, congenital heart disease, and asthma may have susceptibilities and warrant special consideration. Clinicians should have an understanding of the physiologic adaptations, anesthetic considerations, and special concerns in these populations in order to offer the best care possible.

Anesthetic Considerations For Pediatric Patients: Challenges And Best Practices

Anesthetic Considerations For Pediatric Patients: Challenges And Best Practices

Anesthetic Considerations in Pediatric Patients Undergoing Cochlear Implantation Surgery

Anesthetic Considerations in Pediatric Patients Undergoing Cochlear Implantation Surgery

Anesthetic Considerations in Pediatric Patients With Acute Decompensated Heart Failure.

Acute decompensated heart failure (ADHF) in pediatrics is a significant cause for morbidity and mortality in children. Congenital heart disease and cardiomyopathy are the leading etiologies of ADHF. It is common for these children to undergo diagnostic, therapeutic, or surgical procedure under anesthesia, which may be associated with significant morbidity and mortality. The importance of preanesthetic multidisciplinary planning with all involved teams, including anesthesia, cardiology, intensive care, perfusion, and cardiac surgery, cannot be emphasized enough. In order to safely manage these patients, it is imperative for the anesthesiologist to understand the complex pathophysiological interactions between cardiopulmonary systems and anesthesia during these procedures. This review discusses the etiology, pathophysiology, clinical manifestations, and perioperative management of these patients.

SHEA Pediatric Leadership Council commentary: Inpatient visitor considerations for pediatric patients during the coronavirus disease 2019 (COVID-19) pandemic.

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Sickle Cell Disease: An Overview of Oral Health Considerations for Pediatric Patients

Aim: This literature review aims to update the evidence for orofacial manifestations and current treatment recommendations for children and adolescents with sickle cell disease.&#x0D; Background: Sickle cell disease is a frequent hemoglobinopathy and a life-threatening genetic disorder. The lifelong condition is characterized by chronic hemolytic anemia and vaso-occlusive crisis that may occur in a variable range of clinical presentations in different regions of the body, including the oral cavity.&#x0D; Review results: This review explored the most common orofacial alterations of pediatric patients with SCD. Dental caries is a common finding in SCD pediatric patients, especially in those who are socio-economically vulnerable. Moreover, malocclusions occur in high prevalence in SCD pediatric patients. Other oral health complications seen in SCD patients include periodontal inflammation, bone changes, infections, mental nerve neuropathy, facial overgrowth, delayed tooth eruption, dental anomalies, pulp necrosis, soft tissue alterations and salivary changes. Dental infections may trigger a vaso-occlusive crisis leading the patient to a higher probability on arriving in hospital emergency departments and in need for further hospital admission to deal with the correlated complications. Thus, preventive dental care and non-invasive dental procedures are the principal focus in SCD patients in order to avoid possible subsequent complications.&#x0D; Conclusion: The review showed that in pediatric patients with SCD the risk for orofacial manifestations and complications depends not only on the presence of SCD but also on other confounding factors such as oral hygiene, diet habits and social conditions. Moreover, more well-designed epidemiological studies are necessary to assess the real link between SCD disease and its impact on stomatognathic health.