Medicine and Health Sciences Commons^™

Anaesthetic Management Of Nesidioblastosis In Two Infants, Muhammad Saad Yousuf, Mohsin Nazir, Fauzia Khan

Department of Anaesthesia

Nesidioblastosis is the most common cause of non-transient, recurrent and persistent hypoglycaemia in neonates and infants. It is a disorder of diffuse proliferation of beta cells of the pancreas leading to hyperinsulinemia and hypoglycaemia. The main aim is to prevent the severe episodes of hypoglycaemia which can cause damage to the brain and/or mental retardation. In this case report we present two cases of nesidioblastosis and their perioperative anaesthetic course for near-total pancreatectomy. First case was a 7 months old female who had repeated episodes of convulsions since birth. Second case was a 4-month-old female child who again presented with …

Successful Reversal Of Furosemide-Induced Secondary Hyperparathyroidism With Cinacalcet., Tarak Srivastava, Shahryar Jafri, William E. Truog, Judith Sebestyen Vansickle, Winston M. Manimtim, Uri S. Alon

Manuscripts, Articles, Book Chapters and Other Papers

Secondary hyperparathyroidism (SHPT) is a rare complication of furosemide therapy that can occur in patients treated with the loop diuretic for a long period of time. We report a 6-month-old 28-weeks premature infant treated chronically with furosemide for his bronchopulmonary dysplasia, who developed hypocalcemia and severe SHPT, adversely affecting his bones. Discontinuation of the loop diuretic and the addition of supplemental calcium and calcitriol only partially reversed the SHPT, bringing serum parathyroid hormone level down from 553 to 238 pg/mL. After introduction of the calcimimetic Cinacalcet, we observed a sustained normalization of parathyroid hormone concentration at 27 to 63 pg/mL …

Fibromatosis Colli - An Infrequent Case Of Neonatal Neck Mass, Madhusudhan Krishnamoorthy, Ram Kumar Sharma Shanmugam, Shahrul Bin Hitam, Khairullah Bin Anuar, Nur Safira Binti Abdul Isa

Manipal Journal of Medical Sciences

Fibromatosis colli presents in 0.4% of infants as a firm palpable mass in the middle or lower thirds of the sternocleidomastoid muscle.1 The typical age of presentation ranges between two to four weeks of life. It is unilateral in 75% of cases and occurs due to fibrosis within the sternocleidomastoid muscle. Although no single entity has been able to explain the etiology, birth trauma is often attributable. Ultrasonography is the initial investigation of choice. This benign condition is often treated conservatively, rarely requiring surgical intervention. Here are two cases from our centre

Aeromedical Transport Of Critically Ill Infants Less Than 3 Months Of Age, Anil P. George, Akshay Sharma, Scottie B. Day

Pediatrics Faculty Publications

No abstract provided.

Precision Newborn Screening For Lysosomal Disorders, Melissa M. Minter Baerg, Stephanie D. Stoway, Jeremy Hart, Lea Mott, Dawn S. Peck, Stephanie L. Nett, Jason S. Eckerman, Jean M. Lacey, Coleman T. Turgeon, Dimitar Gavrilov, Devin Oglesbee, Kimiyo Raymond, Silvia Tortorelli, Dietrich Matern, Lars Mørkrid, Piero Rinaldo

Pathology and Laboratory Medicine Faculty Publications

Purpose: The implementation of newborn screening for lysosomal disorders has uncovered overall poor specificity, psychosocial harm experienced by caregivers, and costly follow-up testing of false-positive cases. We report an informatics solution proven to minimize these issues.

Methods: The Kentucky Department for Public Health outsourced testing for mucopolysaccharidosis type I (MPS I) and Pompe disease, conditions recently added to the recommended uniform screening panel, plus Krabbe disease, which was added by legislative mandate. A total of 55,161 specimens were collected from infants born over 1 year starting from February 2016. Testing by tandem mass spectrometry was integrated with multivariate pattern recognition …

Canadian Guidelines For Controlled Pediatric Donation After Circulatory Determination Of Death-Summary Report, Matthew J Weiss, Laura Hornby, Bram Rochwerg, Michael Van Manen, Sonny Dhanani, V Ben Sivarajan, Amber Appleby, Mary Bennett, Daniel Buchman, Catherine Farrell, Aviva Goldberg, Rebecca Greenberg, Ram Singh, Thomas A Nakagawa, William Witteman, Jill Barter, Allon Beck, Kevin Coughlin, Alf Conradi, Cynthia Cupido, Rosanne Dawson, Anne Dipchand, Darren Freed, Karen Hornby, Valerie Langlois, Cheryl Mack, Meagan Mahoney, Deepak Manhas, Christopher Tomlinson, Samara Zavalkoff, Sam D Shemie

Paediatrics Publications

OBJECTIVES: Create trustworthy, rigorous, national clinical practice guidelines for the practice of pediatric donation after circulatory determination of death in Canada.

METHODS: We followed a process of clinical practice guideline development based on World Health Organization and Canadian Medical Association methods. This included application of Grading of Recommendations Assessment, Development, and Evaluation methodology. Questions requiring recommendations were generated based on 1) 2006 Canadian donation after circulatory determination of death guidelines (not pediatric specific), 2) a multidisciplinary symposium of national and international pediatric donation after circulatory determination of death leaders, and 3) a scoping review of the pediatric donation after circulatory …

Disparities In Rate, Triggers, And Management In Pediatric And Adult Cases Of Suspected Drug-Induced Anaphylaxis In Canada, Sofianne Gabrielli, Ann E Clarke, Harley Eisman, Judy Morris, Lawrence Joseph, Sebastien La Vieille, Peter Small, Rodrick Lim, Paul Enarson, Michal Zelcer, Edmond S Chan, Chris Mill, Moshe Ben-Shoshan

Paediatrics Publications

Introduction

Data is sparse on drug-induced anaphylaxis (DIA) and there have not been studies assessing the differences in clinical characteristics and management of DIA between adults and children.

Objective

We assessed the percentage, diagnosis, and management of DIA among all anaphylaxis visits in three pediatric and one adult emergency departments (ED) across Canada.

Methods

Children presenting to the Montreal Children's Hospital (MCH), British Columbia Children's Hospital (BCCH), and Children's Hospital at London Health Sciences Center and adults presenting to Hôpital du Sacré-Coeur with anaphylaxis were recruited as part of the Cross-Canada Anaphylaxis Registry. A standardized data form documenting the reaction …

Comparative Trial Between Neonatal Intensive Care Incubator, Neonatal Laminar Flow And Radiant Warmer, J Perez, F Perez, Sergio Golombek, Augusto Sola

NYMC Faculty Publications

No abstract provided.

Multisite Evaluation Of The Bd Max Extended Enteric Bacterial Panel For Detection Of Yersinia Enterocolitica, Enterotoxigenic Escherichia Coli, Vibrio, And Plesiomonas Shigelloides From Stool Specimens., Patricia J Simner, Margret Oethinger, Kathleen A Stellrecht, Dylan R Pillai, Ram Yogev, Helene Leblond, Joel Mortensen

Articles, Abstracts, and Reports

The purpose of this study was to perform a multisite evaluation to establish the performance characteristics of the BD Max extended enteric bacterial panel (xEBP) assay directly from unpreserved or Cary-Blair-preserved stool specimens for the detection of Yersinia enterocolitica, enterotoxigenic Escherichia coli (ETEC), Vibrio, and Plesiomonas shigelloides The study included prospective, retrospective, and prepared contrived specimens from 6 clinical sites. BD Max xEBP results were compared to the reference method, which included standard culture techniques coupled with alternate PCR and sequencing, except for ETEC, for which the reference method was two alternate PCRs and sequencing. Alternate PCR was …

Legionellosis Following Water Birth In A Hot Tub In A Canadian Neonate, Michelle Barton, Brianna Mckelvie, Aaron Campigotto, Tara Mullowney

Paediatrics Publications

No abstract provided.

Characterization Of Pulmonary Metastases In Children With Hepatoblastoma Treated On Children's Oncology Group Protocol Ahep0731 (The Treatment Of Children With All Stages Of Hepatoblastoma): A Report From The Children's Oncology Group., Allison F. O'Neill, Alexander J. Towbin, Mark D. Krailo, Caihong Xia, Yun Gao, M. Beth Mccarville, Rebecka L. Meyers, Eugene D. Mcgahren, Greg M. Tiao, Stephen P. Dunn, Max R. Langham, Christopher B. Weldon, Milton J. Finegold, Sarangarajan Ranganathan, Wayne L. Furman, Marcio Malogolowkin, Carlos Rodriguez-Galindo, Howard M. Katzenstein

Department of Pediatrics Faculty Papers

Purpose To determine whether the pattern of lung nodules in children with metastatic hepatoblastoma (HB) correlates with outcome. Methods Thirty-two patients with metastatic HB were enrolled on Children's Oncology Group Protocol AHEP0731 and treated with vincristine and irinotecan (VI). Responders to VI received two additional cycles of VI intermixed with six cycles of cisplatin/fluorouracil/vincristine/doxorubicin (C5VD), and nonresponders received six cycles of C5VD alone. Patients were imaged after every two cycles and at the conclusion of therapy. All computed tomography scans and pathology reports were centrally reviewed, and information was collected regarding lung nodule number, size, laterality, timing of resolution, and …

Tnf-Α Promotes Nuclear Enrichment Of The Transcription Factor Tonebp/Nfat5 To Selectively Control Inflammatory But Not Osmoregulatory Responses In Nucleus Pulposus Cells., Zariel I. Johnson, Alexandra C. Doolittle, Joseph W. Snuggs, Irving M. Shapiro, Christine L. Le Maitre, Makarand V. Risbud

Department of Orthopaedic Surgery Faculty Papers

Intervertebral disc degeneration (IDD) causes chronic back pain and is linked to production of proinflammatory molecules by nucleus pulposus (NP) and other disc cells. Activation of tonicity-responsive enhancer-binding protein (TonEBP)/NFAT5 by non-osmotic stimuli, including proinflammatory molecules, occurs in cells involved in immune response. However, whether inflammatory stimuli activate TonEBP in NP cells and whether TonEBP controls inflammation during IDD is unknown. We show that TNF-α, but not IL-1β or LPS, promoted nuclear enrichment of TonEBP protein. However, TNF-α-mediated activation of TonEBP did not cause induction of osmoregulatory genes. RNA sequencing showed that 8.5% of TNF-α transcriptional responses were TonEBP-dependent and …

Integrating Nutrition Interventions Into An Existing Maternal, Neonatal, And Child Health Program Increased Maternal Dietary Diversity, Micronutrient Intake, And Exclusive Breastfeeding Practices In Bangladesh: Results Of A Cluster-Randomized Program Evaluation, Phuong Hong Nguyen, Sunny S. Kim, Tina Sanghvi, Zeba Mahmud, Lan Mai Tran, Sadia Shabnam, Bachera Aktar, Raisul Haque, Kaosar Afsana, Edward A. Frongillo Jr., Marie T. Ruel, Purnima Menon

Faculty Publications

Maternal undernutrition is a major concern globally, contributing to poor birth outcomes. Limited evidence exists on delivering multiple interventions for maternal nutrition simultaneously. Alive & Thrive addressed this gap by integrating nutrition-focused interpersonal counseling, community mobilization, distribution of free micronutrient supplements, and weight-gain monitoring through an existing Maternal, Neonatal, and Child Health (MNCH) program in Bangladesh. We evaluated the effect of providing nutrition-focused MNCH compared with standard MNCH (antenatal care with standard nutrition counseling) on coverage of nutrition interventions, maternal dietary diversity, micronutrient supplement intake, and early breastfeeding practices. We used a cluster-randomized design with cross-sectional surveys at baseline (2015) …

Effect Of Voxel Size On The Accuracy Of 3d Reconstructions With Cone Beam Ct., Delphine Maret, Norbert Telmon, Ove A. Peters, B. Lepage, J. Treil, J. M. Inglèse, A. Peyre, Jean-Luc Kahn, Michel Sixou

Ove Peters

OBJECTIVES: The various types of cone beam CT (CBCT) differ in several technical characteristics, notably their spatial resolution, which is defined by the acquisition voxel size. However, data are still lacking on the effects of voxel size on the metric accuracy of three-dimensional (3D) reconstructions. This study was designed to assess the effect of isotropic voxel size on the 3D reconstruction accuracy and reproducibility of CBCT data. METHODS: The study sample comprised 70 teeth (from the Institut d'Anatomie Normale, Strasbourg, France). The teeth were scanned with a KODAK 9500 3D® CBCT (Carestream Health, Inc., Marne-la-Vallée, France), which has two voxel …

Do Premature Babies Who Are Fed Thickened Liquids, Versus Those Fed Standard Feeds, Transition More Quickly To Full Oral Feeds? A Review Of Current Research And Evidence Based Practice, Donna J. Bisbee

Interprofessional Research and Innovations Council

Do Premature Babies who are fed Thickened Liquids, versus those fed standard feeds, transition more quickly to full oral feeds?

A Review of Current Research and Evidence Based Practice

Donna J. Bisbee, MA CCC-SLP, Speech Language Pathologist, MMC,

Department of Rehabilitation Medicine October, 2017

Background/literature : Babies born prematurely (prior to 37 weeks gestational age) experience a greater percentage of medical complications than full term babies. Chronic lung disease and gastro-esophageal reflux are particularly prevalent in this population and can contribute to delays and difficulties in learning to orally feed. Inability to efficiently and safely feed orally frequently contributes to …

Increased Birth Weight Is Associated With Altered Gene Expression In Neonatal Foreskin, Leryn J. Reynolds, Rebecca I. Pollack, Richard J. Charnigo, Cetewayo S. Rashid, Arnold J. Stromberg, Shu Shen, John O'Brien, Kevin J. Pearson

Pharmacology and Nutritional Sciences Faculty Publications

Elevated birth weight is linked to glucose intolerance and obesity health-related complications later in life. No studies have examined if infant birth weight is associated with gene expression markers of obesity and inflammation in a tissue that comes directly from the infant following birth. We evaluated the association between birth weight and gene expression on fetal programming of obesity. Foreskin samples were collected following circumcision, and gene expression analyzed comparing the 15% greatest birth weight infants (n = 7) v. the remainder of the cohort (n = 40). Multivariate linear regression models were fit to relate expression levels on differentially …

Does Breastfeeding Shape Food Preferences? Links To Obesity, Alison K. Ventura

Kinesiology and Public Health

The first 2 years of life have been recognized as a critical window for obesity prevention efforts. This period is characterized by rapid growth and development and, in a relatively short period of time, a child transitions from a purely milk-based diet to a more varied solid-food diet. Much learning about food and eating occurs during this critical window, and it is well-documented that early feeding and dietary exposures predict later food preferences, eating behaviors, and dietary patterns. The focus of this review will be on the earliest feeding experiences - breast- and formula-feeding - and the unique role of …

Therapeutic And Prognostic Implications Of Braf V600e In Pediatric Low-Grade Gliomas., Alvaro Lassaletta, Michal Zapotocky, Matthew Mistry, Vijay Ramaswamy, Marion Honnorat, Rahul Krishnatry, Ana Guerreiro Stucklin, Nataliya Zhukova, Anthony Arnoldo, Scott Ryall, Catriona Ling, Tara Mckeown, Jim Loukides, Ofelia Cruz, Carmen De Torres, Cheng-Ying Ho, Roger J Packer, Ruth Tatevossian, Ibrahim Qaddoumi, Julie H Harreld, James D Dalton, Jean Mulcahy-Levy, Nicholas Foreman, Matthias A Karajannis, Shiyang Wang, Matija Snuderl, Amulya Nageswara Rao, Caterina Giannini, Mark Kieran, Keith L Ligon, Maria Luisa Garre, Paolo Nozza, Samantha Mascelli, Alessandro Raso, Sabine Mueller, Theodore Nicolaides, Karen Silva, Romain Perbet, Alexandre Vasiljevic, Cécile Faure Conter, Didier Frappaz, Sarah Leary, Courtney Crane, Aden Chan, Ho-Keung Ng, Zhi-Feng Shi, Ying Mao, Elizabeth Finch, David Eisenstat, Bev Wilson, Anne Sophie Carret, Peter Hauser, David Sumerauer, Lenka Krskova, Valerie Larouche, Adam Fleming, Shayna Zelcer, Nada Jabado, James T Rutka, Peter Dirks, Michael D Taylor, Shiyi Chen, Ute Bartels, Annie Huang, David W Ellison, Eric Bouffet, Cynthia Hawkins, Uri Tabori

Paediatrics Publications

Purpose BRAF V600E is a potentially highly targetable mutation detected in a subset of pediatric low-grade gliomas (PLGGs). Its biologic and clinical effect within this diverse group of tumors remains unknown.

Patients and Methods A combined clinical and genetic institutional study of patients with PLGGs with long-term follow-up was performed (N = 510). Clinical and treatment data of patients with BRAF V600E mutated PLGG (n = 99) were compared with a large international independent cohort of patients with BRAF V600E mutated-PLGG (n = 180).

Results BRAF V600E mutation was detected in 69 of 405 patients (17%) with PLGG across a …

Harlequin Colour Change, Suneeta Khemani, Shirin Bahadur Ali, Sarfaraz Karim, Mohammad Ali Yezdan

Department of Paediatrics and Child Health

Harlequin colour change (HCC) is a rare, benign and transient colour change presenting in normal healthy neonates in initial days of life and resolves spontaneously. It is thought to be due to hypothalamic immaturity, leading to capillary bed dysregulation. Gravity might also play a role, as this is apparent on the dependent side. It is important for physicians to recognize this phenomenon so that they do not order any irrelevant investigations. Here, we present a case of a healthy neonate who had HCC, which was unilateral, well demarcated erythema, with patchy pallor. Baby was vitally stable at that time. This …

Reducing The Risk Of Hypothermia In Neonatal Infants, Monika Klett-Dunbar

Master's Projects and Capstones

Abstract

Global Aim: To improve infant thermoregulation within the first 24 hours of life of the high risk preterm and very low birth weight (VLBW) infant population in the NICU, thereby reducing rates of morbidity and mortality due to complications influenced by infant hypothermia. Project Aim: To reduce the rate of hypothermia at one hour and the first 24 hours of life in the target population of preterm neonates weighing less than 1500 grams with a gestational age less than 32 weeks from 9.5% to < 5% through the consistent use of a specific bundle of interventions applied in the labor and delivery setting, by December 2017. The aim of the current initiative is also to build on the success of the pilot program launched in 2015 by adding to the preliminary bundle of initiatives, and sustaining hard-won achievements by reinforcing education measures to the nursing and medical teams involved in the infant delivery and NICU admission process. Background: Hypothermia is a major factor in morbidity and mortality of low-birth-weight premature infants, …

The Oral Microbiome And Adverse Pregnancy Outcomes., Charles M. Cobb, Patricia J. Kelly, Karen B. Williams, Shilpa Babbar, Mubashir Angolkar, Richard J. Derman

Department of Obstetrics and Gynecology Faculty Papers

Significant evidence supports an association between periodontal pathogenic bacteria and preterm birth and preeclampsia. The virulence properties assigned to specific oral pathogenic bacteria, for example, Fusobacterium nucleatum, Porphyromonas gingivalis, Filifactor alocis, Campylobacter rectus, and others, render them as potential collaborators in adverse outcomes of pregnancy. Several pathways have been suggested for this association: 1) hematogenous spread (bacteremia) of periodontal pathogens; 2) hematogenous spread of multiple mediators of inflammation that are generated by the host and/or fetal immune response to pathogenic bacteria; and 3) the possibility of oral microbial pathogen transmission, with subsequent colonization, in the vaginal microbiome resulting from sexual …

Parents' Experiences And Perceptions When Classifying Their Children With Cerebral Palsy: Recommendations For Service Providers., Natalie V Scime, Doreen J Bartlett, Laura K Brunton, Robert J Palisano

Physical Therapy Publications

AIMS: This study investigated the experiences and perceptions of parents of children with cerebral palsy (CP) when classifying their children using the Gross Motor Function Classification System (GMFCS), the Manual Ability Classification System (MACS), and the Communication Function Classification System (CFCS). The second aim was to collate parents' recommendations for service providers on how to interact and communicate with families.

METHODS: A purposive sample of seven parents participating in the On Track study was recruited. Semi-structured interviews were conducted orally and were audiotaped, transcribed, and coded openly. A descriptive interpretive approach within a pragmatic perspective was used during analysis.

RESULTS: …

Active Auditory Experience In Infancy Promotes Brain Plasticity In Theta And Gamma Oscillations, Gabriella Musacchia, Silvia Ortiz-Mantilla, Naseem Choudhury, Teresa Realpe-Bonilla, Cynthia P. Roesler, April A. Benasich

All Faculty Scholarship

Language acquisition in infants is driven by on-going neural plasticity that is acutely sensitive to environmental acoustic cues. Recent studies showed that attention-based experience with non-linguistic, temporally-modulated auditory stimuli sharpens cortical responses. A previous ERP study from this laboratory showed that interactive auditory experience via behavior-based feedback (AEx), over a 6-week period from 4- to 7-months-of-age, confers a processing advantage, compared to passive auditory exposure (PEx) or maturation alone (Naïve Control, NC). Here, we provide a follow-up investigation of the underlying neural oscillatory patterns in these three groups. In AEx infants, Standard stimuli with invariant frequency (STD) elicited greater Theta-band …

Brain Biomarkers And Pre-Injury Cognition Are Associated With Long-Term Cognitive Outcome In Children With Traumatic Brain Injury, Amy A Wilkinson, Maureen Dennis, Nevena Simic, Margot J Taylor, Benjamin R Morgan, Helena Frndova, Karen Choong, Craig Campbell, Douglas Fraser, Vicki Anderson, Anne-Marie Guerguerian, Russell Schachar, Jamie Hutchison

Paediatrics Publications

BACKGROUND: Children with traumatic brain injury (TBI) are frequently at risk of long-term impairments of attention and executive functioning but these problems are difficult to predict. Although deficits have been reported to vary with injury severity, age at injury and sex, prognostication of outcome remains imperfect at a patient-specific level. The objective of this proof of principle study was to evaluate a variety of patient variables, along with six brain-specific and inflammatory serum protein biomarkers, as predictors of long-term cognitive outcome following paediatric TBI.

METHOD: Outcome was assessed in 23 patients via parent-rated questionnaires related to attention deficit hyperactivity disorder …

Two-Year Antibody Persistence In Children Vaccinated At 12-15 Months With A Measles-Mumps-Rubella Virus Vaccine Without Human Serum Albumin., Andrea A. Berry, Remon Abu-Elyazeed, Clemente Diaz-Perez, Maurice A. Mufson, Christopher J. Harrison, Michael Leonardi, Jerry D. Twiggs, Christopher Peltier, Stanley Grogg, Antonio Carbayo, Steven Shapiro, Michael Povey, Carmen Baccarini, Bruce L. Innis, Ouzama Henry

Manuscripts, Articles, Book Chapters and Other Papers

One combined measles-mumps-rubella (MMR) vaccine without Human Serum Albumin (HSA) is currently licensed in the USA (M-M-R II; Merck, USA) and another has been developed (Priorix™ [MMR-RIT, GSK, Belgium]). In this follow-up study, children from USA or Puerto Rico, who had received one dose of M-M-R II or MMR-RIT at 12-15 months of age in the primary study (NCT00861744), were followed-up for 2 y post-vaccination. Anti-measles and anti-rubella antibodies were measured using Enzyme-Linked Immunosorbent Assay (ELISA), and anti-mumps antibodies using ELISA and plaque reduction neutralization (PRN) assays. Serious adverse events (SAEs) were recorded during the entire follow-up. The according-to-protocol (ATP) …

Cesarean In The Second Stage: A Possible Risk Factor For Subsequent Spontaneous Preterm Birth., Vincenzo Berghella, Alexis C. Gimovsky, Lisa D. Levine, Joy Vink

Department of Obstetrics and Gynecology Faculty Papers

No abstract provided.

Brain Injury With Systemic Inflammation In Newborns With Congenital Heart Disease Undergoing Heart Surgery, R Pironkova, Joseph Giamelli, H Seiden, V Parnell, D Gruber, C Sison, K Ojamaa

NYMC Faculty Publications

The potential role of systemic inflammation on brain injury in newborns with congenital heart disease (CHD) was assessed by measuring levels of central nervous system (CNS)-derived proteins in serum prior to and following cardiac surgery. A total of 23 newborns (gestational age, 39±1 weeks) with a diagnosis of CHD that required cardiac surgery with cardiopulmonary bypass (CPB) were enrolled in the current study. Serum samples were collected immediately prior to surgery and 2, 24 and 48 h following CPB, and serum levels of phosphorylated neurofilament-heavy subunit (pNF-H), neuron-specific enolase (NSE) and S100B were analyzed. Systemic inflammation was assessed by measuring …

Lung Rest During Extracorporeal Membrane Oxygenation For Neonatal Respiratory Failure-Practice Variations And Outcomes., Deepthi Alapati, Zubair H. Aghai, Jobayer Hossain, Daniel R Dirnberger, Mark T. Ogino, Thomas H. Shaffer

Department of Pediatrics Faculty Papers

OBJECTIVE: Describe practice variations in ventilator strategies used for lung rest during extracorporeal membrane oxygenation for respiratory failure in neonates, and assess the potential impact of various lung rest strategies on the duration of extracorporeal membrane oxygenation and the duration of mechanical ventilation after decannulation.

DATA SOURCES: Retrospective cohort analysis from the Extracorporeal Life Support Organization registry database during the years 2008-2013.

STUDY SELECTION: All extracorporeal membrane oxygenation runs for infants less than or equal to 30 days of life for pulmonary reasons were included.

DATA EXTRACTION: Ventilator type and ventilator settings used for lung rest at 24 hours after …

Expanding The Genotypic Spectrum Of Bathing Suit Ichthyosis., Nareh V. Marukian, Rong-Hua Hu, Brittany G. Craiglow, Leonard M. Milstone, Jing Zhou, Amy Theos, Hande Kaymakcalan, Deniz A. Akkaya, Jouni J. Uitto, Hassan Vahidnezhad, Leila Youssefian, Susan J. Bayliss, Amy S. Paller, Lynn M. Boyden, Keith A. Choate

Department of Dermatology and Cutaneous Biology Faculty Papers

Importance: Bathing suit ichthyosis (BSI) is a rare congenital disorder of keratinization characterized by restriction of scale to sites of relatively higher temperature such as the trunk, with cooler areas remaining unaffected. Fewer than 40 cases have been reported in the literature. Bathing suit ichthyosis is caused by recessive, temperature-sensitive mutations in the transglutaminase-1 gene (TGM1). Clear genotype-phenotype correlations have been difficult to establish because several of the same TGM1 mutations have been reported in BSI and other forms of congenital ichthyosis. We identify novel and recurrent mutations in 16 participants with BSI.

Objective: To expand the genotypic spectrum of …

The Quest For Sustained Multiple Morbidity Reduction In Very Low-Birth-Weight Infants: The Antifragility Project., Joseph Kaempf, N M Schmidt, S Rogers, C Novack, M Friant, L Wang, N Tipping

Articles, Abstracts, and Reports

OBJECTIVE: Can a comprehensive, explicitly directive evidence-based guideline for all therapies that might affect the major morbidities of very low-birth-weight (VLBW) infants help a neonatal intensive care unit (NICU) further improve generally favorable morbidity rates? Can Antifragility principles of provider adaptive growth from stressors, enhanced infant risk assessment and adherence to effective therapies minimize unproven treatments and reduce all morbidities?

STUDY DESIGN: Prospectively planned observational trial in VLBW infants: control group born October 2011 to September 2013 and study group October 2013 to September 2015. Multi-disciplinary evidence-based review assigned all NICU treatments into one of four distinct categories: (1) always …