Pediatrics Commons^™

Chronic Abdominal Pain & Colonic Non-Fixation Case Report, Brett Howe, Andreana Butter

Paediatrics Publications

Abnormalities of intestinal rotation and fixation occur in one out of 500 live births and usually present within the first month of life. We report a case of a 14-year-old girl with lifelong, chronic abdominal pain. Despite treatment by multiple specialists and psychiatrists, her pain remained severe. A barium enema revealed that her left colon was located along her right paracolic gutter, suggesting a form of malrotation/ non-fixation. Laparoscopy, followed by laparotomy, demonstrated a complete lack of fixation of her entire colon. Due to the mobility of the colon, as well as its redundant mesentery, her entire small bowel was …

Low Birth Weight Male Guinea Pig Offspring Display Increased Visceral Adiposity In Early Adulthood., Ousseynou Sarr, Jennifer A Thompson, Lin Zhao, Ting-Yim Lee, Timothy Regnault

Paediatrics Publications

Uteroplacental insufficiency (UPI)-induced intrauterine growth restriction (IUGR) predisposes individuals to adult visceral obesity. We postulated that low birth weight (LBW) offspring, from UPI-induced IUGR pregnancies, would display a visceral adipose lipogenic molecular signature involving altered gene expression, phosphorylation status of proteins of the lipid synthesis pathway and microRNA (miR) expression profile, occurring in association with increased visceral adiposity. Normal birth weight (NBW) and LBW (obtained by uterine artery ablation) male guinea pig pups were fed a control diet from weaning to 145 days and sacrificed. Despite being lighter at birth, LBW pups displayed body weights similar to NBW offspring at …

Diagnosis By Peritoneal Scintigraphy Of Peritoneal Dialysis–Associated Hydrothorax In An Infant, M. Chavannes, A. P. Sharma, R. N. Singh, R. H. Reid, G. Filler

Paediatrics Publications

No abstract provided.

Choline Kinase Beta Is Required For Normal Endochondral Bone Formation, Zhuo Li, Gengshu Wu, Roger B. Sher, Zohreh Khavandgar, Martin Hermansson, Gregory A. Cox, Michael R. Doschak, Monzur Murshed, Frank Beier, Dennis E. Vance

Paediatrics Publications

Background Choline kinase has three isoforms encoded by the genes Chka and Chkb. Inactivation of Chka in mice results in embryonic lethality, whereas Chkb-/- mice display neonatal forelimb bone deformations. Methods To understand the mechanisms underlying the bone deformations, we compared the biology and biochemistry of bone formation from embryonic to young adult wild-type (WT) and Chkb-/- mice. Results The deformations are specific to the radius and ulna during the late embryonic stage. The radius and ulna of Chkb-/- mice display expanded hypertrophic zones, unorganized proliferative columns in their growth plates, and delayed formation of primary ossification centers. The differentiation …

The Role Of The Insula In Speech And Language Processing, Anna Oh, Emma G. Duerden, Elizabeth W. Pang

Paediatrics Publications

Lesion and neuroimaging studies indicate that the insula mediates motor aspects of speech production, specifically, articulatory control. Although it has direct connections to Broca's area, the canonical speech production region, the insula is also broadly connected with other speech and language centres, and may play a role in coordinating higher-order cognitive aspects of speech and language production. The extent of the insula's involvement in speech and language processing was assessed using the Activation Likelihood Estimation (ALE) method. Meta-analyses of 42 fMRI studies with healthy adults were performed, comparing insula activation during performance of language (expressive and receptive) and speech (production …

Measuring Physicians' Productivity: A Three-Year Study To Evaluate A New Remuneration System, Guido Filler, Vanessa Burkoski, Gary Tithecott

Paediatrics Publications

PURPOSE: To evaluate a new assessment tool measuring physicians' academic productivity and its use in a performance-based remuneration system. METHOD: The authors developed an assessment tool based on existing tools to measure productivity. Yearly, from 2008 to 2011, physicians at the University of Western Ontario received a score of up to three points for each of four components (impact, application, scholarly activity, mentorship) in each of four domains (clinical practice, education, research, administration). Scores were weighted by the percentage of time physicians spent on tasks in each domain. Year 1 scores were a baseline. In Years 2 and 3, scores …

Pediatric Gastroesophageal Reflux Disease, Adina Mezrahi

The Science Journal of the Lander College of Arts and Sciences

This objective of this review is to present the known data in regards to Gastroesophageal Reflux Disease (GERD) in infants. Articles with relevant definitions, diagnosis and treatment options were evaluated. It is evident that much controversy exists in the diagnosis and treatment of this disease, and there is the question as whether this disease can be called GERD. Current ability to attribute the symptoms infants present with the disease is still difficult to clarify, despite the fact that as many as 60% of infants show symptoms of this disease. The current testing options have proven to be insufficient in concretely …

Adolescence And Sexuality, Joav Merrick, Ariel Tenenbaum, Hatim A. Omar

Pediatrics Faculty Publications

No abstract provided.

High Levels Of Dek Autoantibodies May Predict Early Flare Following Cessation Of Anti-Tnf Therapy In Juvenile Idiopathic Arthritis, Nirit Mor-Vaknin, Miguel Rivas, Maureen Legendre, Y. Yuanfang, Anne Johnson, Lawrence K. Jung, +12 Additional Authors

Pediatrics Faculty Publications

Introduction The nuclear oncoprotein DEK is a biochemically distinct, pro-inflammatory protein that is a chemoattractant for neutrophils and T-cells. High levels of DEK autoantibodies have been found in several autoimmune diseases including juvenile idiopathic arthritis (JIA), but their role in disease pathogenesis is unclear.

Objectives Since DEK and DEK autoantibodies can contribute to the development of immune complexes and joint inflammation, we suggest that DEK antibody levels can predict disease flare with discontinuation of anti-TNF therapy. Methods In 16 pediatric rheumatology centers, sera samples were collected from 137 children with polyarticular JIA on anti-TNF therapy. Therapy was stopped after 6 …

Is The Consistency More Important Than The Ingredients For Treatment? Case Report For Eosinophilic Esophagitis, Dan W. Parrish, Shashank Sharma, Santhosh Kumar

Surgery Publications

Eosinophilic Esophagitis seems to be an ever-changing disease, from patient-to-patient and from year-to-year in the same patient. Improvements in these patients’ lives have been made with the realization that elimination of dietary antigens helped with symptom relief and treatment with topical steroids assisted in eradication of esophageal eosinophils.

This case study has two very important aspects that should be emphasized. The first is the realization that it may be consistency of the steroid mixture rather than the ingredients that infer the effectiveness of the EoE treatment, and the second aspect is the willingness of the medical team to listen to …

Metabolic Effects Of Acute Thiamine Depletion Are Reversed By Rapamycin In Breast And Leukemia Cells, Shuqian Liu, Sumitra Miriyala, Mignon A. Keaton, Craig T. Jordan, Christina Wiedl, Daret K. St. Clair, Jeffrey A. Moscow

Pediatrics Publications

Thiamine-dependent enzymes (TDEs) control metabolic pathways that are frequently altered in cancer and therefore present cancer-relevant targets. We have previously shown that the recombinant enzyme thiaminase cleaves and depletes intracellular thiamine, has growth inhibitory activity against leukemia and breast cancer cell lines, and that its growth inhibitory effects were reversed in leukemia cell lines by rapamycin. Now, we first show further evidence of thiaminase therapeutic potential by demonstrating its activity against breast and leukemia xenografts, and against a primary leukemia xenograft. We therefore further explored the metabolic effects of thiaminase in combination with rapamycin in leukemia and breast cell lines. …

Comprehensive Analysis Of Human Cytomegalovirus Microrna Expression During Lytic And Quiescent Infection, Zhang-Zhou Shen, Xing Pan, Ling-Feng Miao, Han-Qing Ye, Stéphane Chavanas, Christian Davrinche, Michael Mcvoy, Min-Hua Luo

Pediatrics Publications

Background

Human cytomegalovirus (HCMV) encodes microRNAs (miRNAs) that function as post-transcriptional regulators of gene expression during lytic infection in permissive cells. Some miRNAs have been shown to suppress virus replication, which could help HCMV to establish or maintain latent infection. However, HCMV miRNA expression has not been comprehensively examined and compared using cell culture systems representing permissive (lytic) and semi-permissive vs. non-permissive (latent-like) infection.

Methods

Viral miRNAs levels and expression kinetics during HCMV infection were determined by miRNA-specific stem-loop RT-PCR. HCMV infected THP-1 (non-permissive), differentiated THP-1 (d-THP-1, semi-permissive) and human embryo lung fibroblasts (HELs, fully-permissive) were examined. The impact of …

Clinical Features And Genetic Analysis Of 20 Chinese Patients With X-Linked Hyper-Igm Syndrome, Lin-Lin Wang, Wei Zhou, Wei Zhao, Zhi-Qing Tian, Wei-Fan Wang, Xiao-Fang Wang, Tong-Xin Chen

Pediatrics Publications

X-linked hyper-IgM syndrome (XHIGM) is one type of primary immunodeficiency diseases, resulting from defects in the CD40 ligand/CD40 signaling pathways. We retrospectively analyzed the clinical and molecular features of 20 Chinese patients diagnosed and followed up in hospitals affiliated to Shanghai Jiao Tong University School of Medicine from 1999 to 2013. The median onset age of these patients was 8.5 months (range: 20 days–21 months). Half of them had positive family histories, with a shorter diagnosis lag. The most common symptoms were recurrent sinopulmonary infections (18 patients, 90%), neutropenia (14 patients, 70%), oral ulcer (13 patients, 65%), and protracted diarrhea …

Saving Time Under Pressure: Effectiveness Of Standardizing Pediatric Resuscitation Carts, Erich C. Maul Do, Mph, Philip Westgate Phd, Barbara Latham Msn

Erich C. Maul DO MPH

Platform presentation of original research

Decreased Langerhans Cell Responses To Il-36 Gamma: Altered Innate Immunity In Patients With Recurrent Respiratory Papillomatosis, J. Devoti, L. Hatam, A. Lucs, A. Afzal, A. Abramson, B. M. Steinberg, V. Bonagura

Journal Articles

Recurrent respiratory papillomatosis (RRP) is a rare, chronic disease caused by human papillomaviruses (HPVs) types 6 and 11 that is characterized by the polarization of adaptive immune responses that support persistent HPV infection. Respiratory papillomas express elevated mRNA levels of IL-36 gamma, a proinflammatory cytokine in comparison to autologous clinically normal laryngeal tissues; however there is no evidence of inflammation in these lesions. Consistent with this, respiratory papillomas do not contain T(H)1-like CD4(+) T-cells or cytotoxic CD8(+) T-cells, but instead contain a predominance of T(H)2-like and T regulatory cells (Tregs). In addition, papillomas also are infiltrated with immature Langerhans cells …

X-Linked Agammaglobulinemia Presenting As Polymicrobial Pneumonia, Including Pneumocystis Jirovecii, A. M. Jongco, J. D. Gough, K. Sarnataro, D. W. Rosenthal, J. Moreau, P. Ponda, V. R. Bonagura

Journal Articles

No abstract provided.

Blood Pressure Variability In Children With Primary Vs Secondary Hypertension, D. Leisman, M. Meyers, J. Schnall, N. Chorny, R. Frank, L. Infante, C. B. Sethna

Journal Articles

Increased blood pressure variability (BPV) is correlated with adverse cardiovascular (CV) events in adults. However, there has been limited research on its effect in the pediatric population. Additionally, BPV differences between primary and secondary hypertension (HTN) are not known. Children with primary and secondary HTN underwent 24-hour ambulatory blood pressure monitoring and echocardiography studies. BPV measures of standard deviation (SD), average real variability (ARV), and range were calculated for the 24-hour, daytime, and nighttime periods. Seventy-four patients (median age, 13.5 years; 74% boys) were examined, 40 of whom had primary HTN. Body mass index z score and age were independent …

Animal Models Of In Utero Exposure To A High Fat Diet: A Review, L. Williams, Y. Seki, P. M. Vuguin, M. J. Charron

Journal Articles

The incidence of metabolic disease, including type 2 diabetes and obesity, has increased to epidemic levels in recent years. A growing body of evidence suggests that the intrauterine environment plays a key role in the development of metabolic disease in offspring. Among other perturbations in early life, alteration in the provision of nutrients has profound and lasting effects on the long term health and well being of offspring. Rodent and non-human primate models provide a means to understand the underlying mechanisms of this programming effect. These different models demonstrate converging effects of a maternal high fat diet on insulin and …

Characteristics Of Avoidant/Restrictive Food Intake Disorder In Children And Adolescents: A "New Disorder" In Dsm-5, M. M. Fisher, D. S. Rosen, R. M. Ornstein, K. A. Mammel, D. K. Katzman, E. S. Rome, S. T. Callahan, J. Malizio, S. Kearney, B. T. Walsh

Journal Articles

Purpose: To evaluate the DSM-5 diagnosis of Avoidant/Restrictive Food Intake Disorder (ARFID) in children and adolescents with poor eating not associated with body image concerns. Methods: A retrospective case-control study of 8e18-year-olds, using a diagnostic algorithm, compared all cases with ARFID presenting to seven adolescent-medicine eating disorder programs in 2010 to a randomly selected sample with anorexia nervosa (AN) and bulimia nervosa (BN). Demographic and clinical information were recorded. Results: Of 712 individuals studied, 98 (13.8%) met ARFID criteria. Patients with ARFID were younger than those with AN (n = 98) or BN (n = 66), (12.9 vs. 15.6 vs. …

Anti-Inflammatory Actions Of Endogenous And Exogenous Interleukin-10 Versus Glucocorticoids On Macrophage Functions Of The Newly Born, K. Kasat, H. Patel, O. Predtechenska, I. Vancurova, D. Davidson

Journal Articles

OBJECTIVE: To determine whether specific macrophage immune functions of the newly born are insensitive to the actions of therapeutic levels of dexamethasone (DEX), previously measured in infants with bronchopulmonary dysplasia (BPD), compared with betamethasone (BETA) and exogenous or endogenous interleukin-10 (IL-10). STUDY DESIGN: Macrophages were differentiated from cord blood monocytes (N=18). A serial dose-response (around 10(-8)M), in vitro study was used to examine the effect of DEX, BETA and IL-10, on proinflammatory (PI) cytokine release, phagocytosis and respiratory burst. RESULT: Exogenous IL-10 (10(-8)M) significantly (P < 0.05) inhibited the endotoxin-stimulated release of IL-6, IL-8 and tumor necrosis factor by 63 to 82% with no significant effect by DEX and BETA. There was no inhibition by these three agents at 10(-8)M on phagocytosis and respiratory burst. Inhibition of endogenous IL-10 with a monoclonal antibody significantly increased endotoxin-stimulated cytokine release by at least fourfold. CONCLUSION: Macrophages were relatively insensitive to therapeutic levels of DEX and BETA with regard to PI cytokine release. This study provides rationale for translational and preclinical research using airway instillation of IL-10 for the treatment of BPD.