All Articles

Safa Gul, Anna Suessman, and Raj Warrier

University of Queensland Ochsner Clinical School.

Autoimmune hemolytic anemia is uncommon in children. We report a term male infant with autoimmune hemolytic anemia who was found to be positive for cytomegalovirus. He received steroids and two transfusions during his hospital course due to low hemoglobin levels. He did not show any signs of cardiovascular collapse. His cytomegalovirus viral count decreased without antiviral medications. He was discharged on steroids for about 3 months. We contrast this case with that of another infant male with autoimmune hemolytic anemia after a viral upper respiratory infection. In this contrasting case, the patient also had low hemoglobin levels and had no signs of cardiovascular collapse and was treated with steroids alone. In this case report we demonstrate that steroids are a suitable first line treatment for autoimmune hemolytic anemia in cases where hemoglobin is low but there are no signs of cardiovascular collapse.

Jasmine Al-Zahiri^1*, Arun Nair², Maksuda Akter³, Amal Hamud², Nermin Elmakawey⁴, Hossam Abdel Aziz⁵, Tabitha Watts⁶

¹University of Jordan, Faculty of Medicine

²Royal College of Surgeons in Ireland – Medical University of Bahrain

³American International Medical University

⁴Faculty of Medicine, Benha University

⁵University Faculty of Medicine, Silver Cross Hospital

⁶John H. Stroger, Jr. Hospital of Cook County

The increasing prevalence of childhood obesity is a global public health concern with metabolic syndrome (MetS) at its forefront. Due to the rising levels of pediatric obesity within the past few years, 18.4% of children were classified as obese and 5.2% as severely obese in the United States in 2016. It is imperative to investigate pharmacological options for the treatment of pediatric MetS. Conservative management, namely the incorporation of dietary intervention and physical activity, has been the only adopted treatment approach for pediatric MetS while pharmacological and surgical (bariatrics) methods have only been reserved for high-risk severe cases. The use of HMG-CoA reductase inhibitors known as statins for the treatment of pediatric MetS has been fairly controversial. Statin therapy would be primarily used in pediatric metabolic syndrome as a means to reduce LDL levels to further prevent adverse cardiometabolic events. Nonetheless, the predominant concern for the use of statin therapy in children was that of adverse effects. This article will delve into the use of statin therapy in the pediatric population and how this relates to the prevention of cardiometabolic adverse events in pediatric metabolic syndrome.

Sulochana Manandhar^1,2*, Puja Amatya³, Stephen Baker⁴ and Abhilasha Karkey^1,2

¹Oxford University Clinical Research Unit, Patan Academy of Health Sciences, Kathmandu, Nepal

²Centre for Tropical Medicine and Global Health, Medical sciences division, Nuffield Department of Medicine, University of Oxford, Linacre College, Oxford, UK

³Department of Pediatrics, Patan Academy of Health Sciences, Patan Hospital, Kathmandu, Nepal

⁴Cambridge Institute of Therapeutic Immunology & Infectious Disease (CITIID), Department of Medicine, University of Cambridge, Cambridge, UK

Fuat Bulut^1*, Alev Cumbul², Basak Ballica³

^1*Department of Otorhinolaringology, Private Corlu Reyap Hospital, Rumeli University, Istanbul

²Istanbul Yeditepe University Faculty of Medicine, Istanbul

³Istanbul Bahcesehir University Faculty of Medicine, Istanbul

Introduction: In pediatric patients with a history of recurrent chronic tonsillitis in both their mother and father, they may show an excessive immune response due to genetic inheritance.

Method: Family history in immune defense mechanism predispose to tonsil disorders. Biofilm formation theory explains that the underlying factor of recurrent/ chronic tonsillitis formation is the bacterial biofilm on the surface of the tonsils.

Results: Although recurrent chronic tonsillitis is a clinical diagnosis, patient's symptoms and signs should be monitored carefully. The history of each patient and family members should be taken carefully. Since the clinical presentation of PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) syndrome can be confused with pediatric patients with a history of recurrent chronic tonsillitis in both parents, must follow a multidisciplinary approach before starting a treatment.

Conclusions: Family history determines the severity of tonsillar damage with immune response in pediatric patients with a history of recurrent chronic tonsillitis in both their mother and father.

Zhe Amy Fang¹, Meena Nathan², Sirisha Emani², Sitaram Emani², Juan C. Ibla³*

¹Department of Anesthesia and Pain Medicine, Hospital for Sick Children, Toronto, Ontario, Canada

²Department of Cardiac Surgery, Boston Children’s Hospital and Harvard Medical School, Boston, MA, USA

³Division of Cardiac Anesthesia, Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA

Thromboelastography, a point of care graphical representation of the activation of coagulation and fibrin polymerization process, assists clinicians in making diagnostic and transfusion-related decisions in the perioperative setting. There is growing interest in applying this technology to pediatric cardiac surgery and this article reviews the currently available evidence for the use of thromboelastography (TEG) and thromboelastometry (ROTEM) in this population. A few studies exist on the use of TEG/ROTEM to accurately guide transfusion and positively impacting patient outcomes, indicating a need for additional studies to validate its utility during pediatric cardiac surgery.

Darrell O. Ricke¹*, Nicole Gherlone², Philip Fremont-Smith¹, Philip Tisdall³, Maurice Fremont-Smith²

¹MIT Lincoln Laboratory, USA

²Frank H. Netter MD School of Medicine – Quinnipiac University, USA

³Medical School Companion LLC, USA

Multisystem Inflammatory Syndrome in Children (MIS-C) is appearing in infants, children, and young adults in association with COVID-19 (coronavirus disease 2019) infections of SARS-CoV-2. Kawasaki Disease (KD) is one of the most common vasculitides of childhood. KD presents with similar symptoms to MIS-C especially in severe forms such as Kawasaki Disease Shock Syndrome (KDSS). The observed symptoms for MIS-C and KD are consistent with Mast Cell Activation Syndrome (MCAS) characterized by inflammatory molecules released from activated mast cells. Based on the associations of KD with multiple viral and bacterial pathogens, we put forward the hypothesis that KD and MIS-C result from antibody activation of mast cells by Fc receptor-bound pathogen antibodies causing a hyperinflammatory response upon second pathogen exposure. Within this hypothesis, MIS-C may be atypical KD or a KD-like disease associated with SARS-CoV-2. We extend the mast cell hypothesis that increased histamine levels are inducing contraction of effector cells with impeded blood flow through cardiac capillaries. In some patients, pressure from impeded blood flow, within cardiac capillaries, may result in increased coronary artery blood pressure leading to aneurysms, a well-known complication in KD.

María Recuero Pradillo¹*, Beatriz Moreno Torres¹, Carlos Quimbayo-Arcila¹, Esperanza Carabias Lopez¹, Laura Sánchez Sánchez², Ángel Pantoja², Pedro Rafael Altungy Labrador³, Juan Ruiz Martín¹

¹Department of Pathology. Hospital Virgen de la Salud, Toledo, Spain

²Department of Pediatrics and Neonatology. Hospital Virgen de la Salud, Toledo, Spain

³Department of Research. Complutense University of Madrid, Spain

Noura Al Hassani¹, Abdul-Kader Souid²*

¹Department of Pediatrics, Tawam Hospital, Alain, United Arab Emirates (UAE)

²Department of Pediatrics, College of Medicine and Health Sciences - UAE University, Alain, UAE

One-third of children and adolescents are currently classified as overweight or obese. This epidemic childhood disease is the origin of adult obesity and its associated cardiovascular diseases. Dyslipidemia is also well-recognized risk of cardiovascular disease that often starts in childhood. Breasfeeding lowers the risks of obesity and dyslipidemia later in life and it should be encouraged. Type 1 diabetes (T1D) is an immune-mediated destruction of the pancreatic islets. It is often associated with other autoimmune disorders, such as chronic lymphocytic thyroiditis and celiac disease. Children with T1D require insulin to ‎prevent fatty acid oxidation regardless of their blood glucose level. Exercise can induce hypoglycemia in a child with T1D, as glucose utilization and insulin sensitivity increase. Thus, children with T1D need to check their blood glucose before, during, and after the activity. Type 2 diabetes (T2D) represents an insulin deficiency due to obesity and genetic susceptibility; glucose is overproduced by the liver and underused by the muscles. Thus, healthy diet and regular exercise are the recommended remedies for this condition.

Eric W. Reynolds¹*, Cynthia S. Bell², Debbie Grider³

¹University of Texas Health Science Center at Houston, McGovern Medical School, Department of Pediatrics, Division of Neonatology Houston, TX

²University of Texas Health Science Center at Houston, McGovern Medical School, Department of Pediatrics, Houston, TX

³University of Kentucky, Department of Pediatrics, Division of Neonatology, Lexington, KY

Introduction: During nonnutritive suck, infants must intermittently swallow. When a swallow occurs, it must interact with respiration in 2 main ways. We have previously labeled HOW the interaction occurs as “swallow-breath interaction” (SwBr), and WHERE in the respiratory cycle the swallow occurs as “phase of respiration incident to swallow” (POR). We have described SwBr and POR in preterm infants with and without bronchopulmonary dysplasia and term infants with neonatal abstinence syndrome.

Objective: The objective of this work is to describe SwBr and POR in term infants (TRM) and compare those findings to our previous study of low-risk preterm (LRP) infants.

Method: Suckle, swallow, nasal airflow and chest movement were recorded during nonnutritive suck in 12 TRM infants, collecting 94 swallows. SwBr and POR for each swallow were characterized by our previously described method. Generalized estimating equations were developed to relate the 3 types of SwBr and 5 types of POR to gender, birth weight, gestational age, postmenstrual age (PMA), and weeks post-first nipple feed. The percentages of SwBr and POR were compared to 16 LRP infants, with 176 swallows over 35 encounters.

Results: TRM infants had more swallows with attenuated respiration (AR) with advancing weeks post-first nipple feed and fewer swallows occurring with obstructive apnea (OA) in males and with increasing birth weight. More swallows occurred at mid-expiration (ME) with increasing gestational age, PMA, and male gender and at mid-inspiration (MI) with increasing weeks post-first nipple feed. Fewer swallows occurred at MI in males. Infants in the LRP group studied before 35 weeks PMA were different from TRM infants but become indistinguishable from TRM infants as PMA approached 40 weeks. SwBr and POR in LRP infants progress towards improved feeding efficiency and safety. These results are similar to studies of nutritive feeding.

Conclusion: SwBr and POR during nonnutritive suck in LRP infants become more like TRM infants with advancing PMA. Because the same brainstem centers are activated in both nutritive and nonnutritive suck, investigation of swallow during nonnutritive suck may provide similar information as nutritive feeding with easier analysis.

Tiffanie Bourgeois¹, Catie Griffith¹, Ema-Chanel Johnson¹*, Betty Leblanc¹, Brooke Melancon¹

¹Northwestern State University of Louisiana in Shreveport

This review’s purpose is to identify barriers on adherence of treatment guidelines in the management of pediatric Acute Otitis Media (AOM). The American Academy of Pediatrics (AAP) and American Academy of Family Physicians (AAFP) released revised AOM practice guidelines in 2013. These guidelines address diagnosis and management of AOM in healthy pediatric patients from six months to 12 years of age. AOM is the most common reason children are prescribed antibiotics¹. Previous and continued antibiotic overuse presents increasing problems pertaining to antibiotic resistance, overall children’s health, and healthcare costs². This guideline recommends treatment should be based on presenting signs and symptoms with severity being key in treatment, while conserving antibiotic use. This guideline includes diagnostic criteria, promotes the use of pneumatic otoscopy, and provides appropriate treatment regimens based on findings. Despite this, there is evidence that these guidelines are not being followed in multiple disciplines that provide care to this population.

A comprehensive review of the literature obtained from several databases, produced 650 articles after inclusion and exclusion criteria was applied. In order to include the highest possible level of research, the articles were individually reviewed, and 19 articles were included in this review. Major barriers identified included factors regarding providers, parents, AOM severity and complications, concerns for follow-up care, and other clinical related factors. This literature review identified and compares these multidisciplinary barriers in hope of understanding reasons for the lack of guideline adherence and possibly help facilitate behavioral changes to improve patients’ wellbeing.

Rajesh Parmar¹, Hardik Patel², Sarat K. Dalai¹*

¹Institute of Science, Nirma University, Ahmedabad, Gujarat, India

²Center for Global Infectious Disease Research, Seattle Children’s Research Institute, Seattle, Washington

Whole sporozoite vaccination (WSV) is considered a gold standard for inducing and providing sterile protection against Plasmodium infection. Multiple doses of immunizations with radiation-attenuated sporozoites (RAS) is essential for establishing complete sterile protection against Plasmodium infection in mice as well as in humans. In our recently published article¹, we have shown that the pattern of vaccination with RAS determines the degrees of protection in mice and frequent immunization with RAS in optimum time duration helps in generating minimum threshold liver-stage (LS) specific CD8⁺ T cell memory responses leading to sterile protection. Further, we have shown that the alterations in successive RAS immunization could possibly affect the induction of sterile protection. In summary, animals receiving four successive doses generated 100% sterile protection. However, three successive doses with the same parasite inoculum as four doses, could induce sterile protection in ∼50% mice. Interestingly, mice immunized with the same 3 doses, but with longer gap, could not survive the challenge.

Shilpa Aroskar

MGM Hospital, Vashi, Navi Mumbai, India

Elizabeth Englander

Bridgewater State University

Developmental factors and cyberbullying have not been a major focus in the field; most strikingly, the experiences that young children have with technology have been studied far less, relative to their adolescent peers. Prevalence estimates comparing younger and older children are problematic for several reasons; first, researchers have no consensus definition of cyberbullying, and second, prevalence estimates vary so widely that drawing conclusions is difficult. Access to technology is only another factor among several that likely influences the prevalence of cyberbullying, and it appears to vary with age. Access to cell phones and digital technology in adolescence is related to both victim availability and prevalence of cyberbullying itself. Among younger students, those under 12 years old, one study has found that cell phone ownership increases the risk of being both a cyberbully and a cyberbully/victim significantly. One factor that may mediate the impact of cell phone ownership is education on the appropriate and accurate use of digital technology. This type of education has been neglected in elementary schools, but evidence suggests it may be helpful in reducing cyberbullying.

Whitney B. Eldridge

Mednax Incorporated, Sunrise Florida

Kratom is a legal, widely-available herbal supplement with opioid-like properties increasingly used by those with opioid dependence to self-treat opioid withdrawal. Kratom binds to opioid receptors and can induce withdrawal, dependence, and toxicity. Classification of Kratom as an opioid is controversial. The search for non-opioid alternatives for the treatment of opioid dependence has intensified in the current opioid epidemic. Kratom is heavily advertised as one such safe non-opiate alternative and has been used by pregnant women with chronic opioid use resulting in neonatal abstinence syndrome. Kratom cannot be detected on routine toxicology screening. As kratom use becomes more widespread, pediatric populations will likely be impacted and pediatricians should familiarize themselves with its pharmacology and adverse effects to appropriately counsel parents and care for kratom-exposed patients. This article reviews kratom’s pharmacology, uses, potential benefits as a therapeutic, and risks for pediatric patients.

Moustafa Hamchou¹, Bahjat M. Sahari¹, Adnan R. Swid¹, Abdul-Kader Souid²*

¹Department of Surgery, Tawam Hospital, Alain City, United Arab Emirates

²College of Medicine and Health Sciences - UAE University, Alain City, United Arab Emirates

Wa Xian^1,2*, Marcin Duleba³, Yutao Qi³, Rajasekaran Mahalingam³, Matthew Vincent⁴, Frank McKeon³

¹Institute of Molecular Medicine, McGovern Medical School of University of Texas Health Science Center, Houston, Texas 77030, USA

²Department of Biochemistry and Molecular Biology, University of Texas McGovern Medical School, Houston, Texas 77030, USA

³Department of Biology and Biochemistry, University of Houston, Houston, Texas 77204, USA

⁴Tract Pharmaceuticals, Inc., Marlborough, Massachusetts 01752, USA

A robust and reliable culture system of adult stem cells is essential for applying the cutting-edge technologies of drug screening, gene editing, and genomics to stem cell research necessary for breakthroughs in this field. In addition, personalized regenerative medicine based on autologous transplantation requires our ability to clone and expand the numbers of these stem cells in vitro. In comparison to the 3D "organoid" culture system that shows limited ability to propagate stem cells as the majority of cells are differentiated or transit amplifying cells, ground-state stem cell culture system is a novel technology that permits long-lived adult stem cells to maintain immaturity, self-renewal capacity, multi-potency and genomic stability despite long-term culturing in a 2D system. The robustness, reliability and easy-to-use features of this new technology bypass the deficiencies of 3D organoid culture systems and provided unlimited stem cell sources for research, therapeutic use, and drug discovery.

Sania Amr^1,2 and Christopher A. Loffredo³

¹Department of Epidemiology and Public Health, University of Maryland School of Medicine

²Marlene and Stuart Greenebaum Comprehensive Cancer Center, University of Maryland School of Medicine, Baltimore, Maryland

³Lombardi Comprehensive Cancer Center, Georgetown University, Washington DC

Hepatocellular carcinoma (HCC) is on the rise worldwide and in the US, and despite emerging treatment modalities, its overall prognosis remains poor; therefore, there is a need for preventing its development globally. The major factors contributing to HCC development, namely, infections with hepatitis B and hepatitis C viruses, alcoholic cirrhosis, aflatoxin-contaminated food, non-alcoholic fatty liver disease, metabolic syndrome, and smoking are modifiable; and prevention intervention can start in childhood.

Vaccination for hepatitis B, screening for and treatment of hepatitis C and intravenous drug users, education to avoid fatty liver, alcoholism, and substance use were shown to be effective ways to curb HCC incidence. A focus on reducing early childhood adversity and training young children to make healthy decisions has been strongly recommended as a prevention strategy to reduce most of HCC risk factors.

Lilly Bayouth^1*, Shannon W. Longshore²

¹Department of Surgery, Brody School of Medicine at East Carolina University, 600 Moye Blvd, Greenville, NC, USA

²Division of Pediatric Surgery, Brody School of Medicine at East Carolina University, 600 Moye Blvd MA, 207, Greenville, NC, USA

Emma Barry*, Susan M Smith

HRB Centre for Primary Care Research, Department of General Practice, Royal College of Surgeons in Ireland, Dublin, Ireland

Hannelore Ehrenreich^1,2*, Luise Poustka^3#

¹Clinical Neuroscience, Max Planck Institute of Experimental Medicine, Göttingen, Germany

²Cluster of Excellence and DFG Research Center for Nanoscale Microscopy and Molecular Physiology of the Brain (CNMPB), Göttingen, Germany

³Department of Child and Adolescent Psychiatry and Psychotherapy, University Medical Center, Göttingen, Germany

Vugar Nabi Nabyev¹, Hakan Seneran², Mehmet Cemalettin Aksoy^3*

¹Acibadem Ankara Hospital, Department of Orthopedics, Ankara, Turkey

²Selcuk University Faculty of Medicine, Department of Orthopedics, Selcuklu/Konya, Turkey

³Hacettepe University, Faculty of Medicine, Department of Orthopedics, Ankara, Turkey

The congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive disease caused by mutations in NTRK1 gene (neurotrophic tyrosine kinase receptor 1) located in chromosome 1q21-22, encoding the tyrosinase domain receptor high affinity nerve growth factor. It is characterized by anhidrosis, insensitivity to painful stimuli and mental retardation. Given their low prevalence and the few reported cases, it is important to know its main features to be considered in the differential diagnosis in pediatric practice. The therapeutic approach of CIPA remains unclear. The preventive approach remains the only possible treatment of CIPA. Early surgical treatment for long bone fractures to prevent pseudo arthrosis and to allow early weightbearing decreasing the risk of further osteopenia. The choice of appropriate antibiotics and surgical debridement in cases of infection might prevent further destruction of joints.

Lawrence M. Benedict¹, Deeksha Sarma¹, Achintya Moulick^1,2, Randy Stevens^1,2, Vicki Mahan*

¹Drexel University College of Medicine, PA, USA

²Department of Pediatric Cardiothoracic Surgery, St. Christopher’s Hospital for Children, Philadelphia, PA, USA

Cardiac risk factors in childhood are often overlooked in clinical practice, however cardiac risk factors can start before the child is even born. Maternal factors including diet, genetics, and smoking during gestation can all impact the long-term cardiac health of the offspring. Atherosclerosis starts as early as fetal life and can continue to develop in children with risks including high cholesterol. Current guidelines for screening of cholesterol in children, while improving in recent years, still allows years of damage to accumulate before identifying those at risk. Additionally, intervention for cholesterol and other known risks in children and adolescents are often avoided or started later than necessary for optimal cardiac health. Non-pharmacological approaches like diet and exercise for cholesterol and health management can be implemented very early in life, while many pharmacological options are approved for use in certain conditions as early as ages 8 to 10. Combating cardiac disease reaching endemic levels in the developed world will take an aggressive approach in management starting with identification early in life and utilizing the appropriate tools available, both medical and lifestyle.

Sudha Rathna Prabhu*

The Tamil Nadu Dr MGR Medical University, Chennai, Tamil Nadu, India

Congenital hypothyroidism (CH) is one of the most common preventable and treatable cause of intellectual impairment in children. Among known etiologies dysgenesis due to abnormal anatomical development and dyshormonogenesis as a result of deranged physiological functioning of fetal thyroid gland. account for at least 90 % of causes of CH. While review of literature report thyroid dysgenesis as the most common etiology of CH currently focus is on iodine status adequacy in women before and during pregnancy and after delivery and lactation. Worldwide reports of previously iodine sufficient countries declared presently as iodine insufficient and recent publications of several Indian states consuming lesser amounts of iodized salt seem to indicate that iodine related pathophysiological factors are emerging as predominant etiologies of CH. The most vulnerable pregnant women and newborns are prone to be affected with iodine imbalances leading to pregnancy and perinatal related complications. Analysis of demographic, biochemical, clinical and statistical data by Indian Council of Medical Research (ICMR) in a multi-centric pilot study on congenital hypothyroidism has clearly shown that dyshormonogenesis is a leading cause of CH in neonates born in India. The need of the hour is to consider mandatory newborn screening for CH in all live newborns and further conduct country wise ethnic and culture oriented research studies with special emphasis on iodine status, genetic predispositions and lifestyle changes impact on all vulnerable populations and design effective early therapeutic strategies for management of CH to prevent intellectual impairment.

Juan R Malagelada^1*, Carolina Malagelada¹

¹Digestive System Research Unit, University Hospital Vall d’Hebron, Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (Ciberehd), Department of Medicine, Universitat Autònoma de Barcelona, Barcelona, Spain

Paolo Indolfi^1*, Serena Picazio², Silverio Perrotta¹, Francesca Rossi¹, Andrea Pession³, Martina Di Martino¹, Elvira Pota¹, Daniela Di Pinto¹, Roberto Rondelli³, Cristiana Indolfi¹, Fiorina Casale¹

^1*UOSD Hemato-Oncology, Department of Woman and Children, and General and Specialized Surgery, University of Campania “Luigi Vanvitelli”, Naples, Italy

²AIL, Section “Valentina Picazio”, Caserta, Italy

³Pediatric Hemato-Oncology Unit, University of Bologna, Italy

Introduction: Childhood cancer is relatively uncommon and the European age-standardized rate was 164 new case per million per year among 0 to 15 years old children (95% CI 158-170). We analyzed data collected in the Mod. 1.01 of the AIEOP (Association Italian Pediatric Hematology-Oncology) over 25 years (1990-2014) about patients with malignant disease diagnosed in 90 towns of the “Ground of fires” (56 in Napoli and 34 in Caserta province).

Methods: Thanks to the wide monitored population we defined 5 time trends: 1990-94; 1995-99; 2000-04; 2005-09; and 2010-14. We calculated the ratio between observed cases (O) and expected (E) numbers of cases among the children from “Terra dei fuochi”.

Results: 2037 cancer patients (0-15 years) were registered in the AIEOP database: 1144 males (56.1%) and 893 females (43.8%).

Conclusions: It is note-worthy to underline that our cases needed to be integrated with the Hospital Informative System and Register of births, marriage and deaths to have a better collection of observed cases. This is a limit of our study. The data analysis do not show significant differences in number of observed cases and type of diseases respect to our previous epidemiological study on cancer cases registered in Campania.