Medical Specialties Commons^™

Allergic Reaction In Hem/Onc Patient, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Office Of Equity Diversity Report Annual Report Fy2020, Children's Mercy Hospital

Equity & Diversity Annual Reports

A report on equity and diversity at Children's Mercy Hospitals & Clinics, in the care it provides, the people it serves, and in its workforce. Specifically discusses the Equity & Diversity Leadership Structure, Employee Demographics, Patient Demographics, lasting impressions from FY20, and the future of healthcare providers.

Fit Clinical Decision-Making: Brain Abscess In A Nonfenestrated Fontan Patient, Bethany Runkel, Natalie Shwaish, Geetha Raghuveer, William Drake

Posters

No abstract provided.

Fit Clinical Decision-Making: Isolated Polymorphic Ventricular Tachycardia In A Pediatric Patient: An Unusual Presentation Of Acute Mycocarditis, Natalie S. Shwaish, Bethany Runkel, Lindsey Malloy-Walton

Posters

No abstract provided.

Two Months Of Global Health In Maseru, Lesotho, Rachel Hildebrand

Posters

Describes her experience working with infants and children at the Center of Excellence and Adolescents at Queen Mamohato Memorial Hospital in Maseru, Lesotho.

Global Health Immersive Elective In The Philippines, Lisa Godfrey

Posters

Describes her experiences working at Fabella Memorial NICU and the Philippines General Hospital.

Philippines: An Int-Erupted Experience, Susamita Kesh

Posters

Describes her experience working in the NICU of the Fabella Memorial Hospital in Philippines.

Fit Clinical Decision-Making: Uhl's Anomaly: An Uncommon Cause Of Fetal Cardiomegaly, Bethany Runkel, Atif Ahmed, Tara Swanson, Maria Kiaffas

Posters

No abstract provided.

Hypomorphic Si Genetic Variants Are Associated With Childhood Chronic Loose Stools., Bruno P Chumpitazi, Jeffery Lewis, Derick Cooper, Mauro D'Amato, Joel Lim, Sandeep Gupta, Adrian Miranda, Natalie Terry, Devendra Mehta, Ann Scheimann, Molly O'Gorman, Neelesh Tipnis, Yinka Davies, Joel Friedlander, Heather Smith, Jaya Punati, Julie Khlevner, Mala Setty, Carlo Di Lorenzo

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: The SI gene encodes the sucrase-isomaltase enzyme, a disaccharidase expressed in the intestinal brush border. Hypomorphic SI variants cause recessive congenital sucrase-isomaltase deficiency (CSID) and related gastrointestinal (GI) symptoms. Among children presenting with chronic, idiopathic loose stools, we assessed the prevalence of CSID-associated SI variants relative to the general population and the relative GI symptom burden associated with SI genotype within the study population.

METHODS: A prospective study conducted at 18 centers enrolled 308 non-Hispanic white children ≤18 years old who were experiencing chronic, idiopathic, loose stools at least once per week for >4 weeks. Data on demographics, GI …

An Unexpected Global Health Experience In Manila, Susan Mcanany

Posters

Describes her experience in the NICU of the Fabella Memorial Hospital in Manila, Philippines.

Global Health Elective In Santiago Atitlan, Guatemala, Sarah Greenberg

Posters

Describes her experience working in Hospitalito Atitlan in Santiago Atitlan, Guatemala.

Fit Clinical Decision-Making Unusual Echocardiographic Findings Of Myocarditis Mimicking An Aortic Runoff Lesion, Sarah Studyvin, Christine Symes, Barbara A. Pahud, Nitin Madan

Posters

No abstract provided.

A Simple Technique For The Management Of Refractory Gastrostomy Site Complications A Technical Innovation In Gastrostomy Tube Site Revision, Rebecca M. Rentea, Wendy Jo Svetanoff, Charlene Dekonenko, Courtney Pisano, Charles L. Snyder

Manuscripts, Articles, Book Chapters and Other Papers

Introduction

Longstanding gastrostomy sites are prone to leakage and excoriation secondary to protrusion of gastric mucosa into the tract, dilation of the site over time, and development of refractory granulation tissue. Surgical revision with creation of a new gastric and skin exit site can be difficult. We describe a technique to re-site a gastrostomy tube (GT) utilizing the existing GT site.

Materials and methods

For one patient with gastrostomy site refractory complications, we re-sited the GT using the existing site. A clamp was placed through the old GT site and was palpated through the abdominal wall. An incision over the …

Pentalogy Of Cantrell, Muhammad Khawar Sana, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

Pentalogy of Cantrell (POC) is a collection of five congenital midline birth anomalies that present a distinctive challenge for clinicians and surgeons. Those five defects are of the heart, pericardium, diaphragm, sternum, and abdominal wall. This condition has been divided into two categories, complete or partial. Complete, as the name indicates, refers to the presence of all five defects, while others may present with only partial defects. It is also referred to as thoracoabdominal ectopia cordis, a condition where the heart is covered by an omphalocele-like membrane. Ectopia cordis (EC) is often found in fetuses with POC. Infants usually have …

Retroperitoneal Hematoma, Christopher Mondie, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

Retroperitoneal hematoma is defined as bleeding into the retroperitoneal space. This clinical entity is often occult and under-recognized by clinicians and is a cause of significant morbidity and mortality. Often patients do not manifest clinically apparent signs and symptoms until a substantial amount of blood loss has occurred. It is not uncommon for patients to present in frank hemorrhagic shock due to an underlying retroperitoneal hematoma. The retroperitoneal space lies directly posterior to the peritoneal cavity. An organizational schema dividing the retroperitoneal space into three different “zones” is widely prevalent in the surgical literature. The central-medial zone (Zone I) falls …

Rna Sequencing Of Human Peripheral Nerve In Response To Injury: Distinctive Analysis Of The Nerve Repair Pathways., Andrew S. Welleford, Jorge E. Quintero, Nader El Seblani, Eric Blalock, Sumedha Gunewardena, Steven M. Shapiro, Sean M. Riordan, Peter Huettl, Zain Guduru, John A. Stanford, Craig G. Van Horne, Greg A. Gerhardt

Manuscripts, Articles, Book Chapters and Other Papers

The development of regenerative therapies for central nervous system diseases can likely benefit from an understanding of the peripheral nervous system repair process, particularly in identifying potential gene pathways involved in human nerve repair. This study employed RNA sequencing (RNA-seq) technology to analyze the whole transcriptome profile of the human peripheral nerve in response to an injury. The distal sural nerve was exposed, completely transected, and a 1 to 2 cm section of nerve fascicles was collected for RNA-seq from six participants with Parkinson's disease, ranging in age between 53 and 70 yr. Two weeks after the initial injury, another …

Morgagni Hernia, Wendy Jo Svetanoff, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

A Morgagni hernia is one of four types of diaphragmatic hernias; the other types include a Bochdalek hernia, where the defect is posterolateral, a hiatal hernia, where the defect is at the esophageal hiatus, and a paraesophageal hernia, where the defect is located adjacent to the esophageal hiatus. The Morgagni hernia, in which the defect is found in an anterior and retrosternal location, was first described by Morgagni in 1769. It is rarer than the other type of congenital diaphragmatic hernia (Bochdalek hernia) and comprises only 2% to 5% of all congenital diaphragmatic hernias. Morgagni hernias tend to be less …

Ambiguous Genitalia And Disorders Of Sexual Differentiation, Khawar T. Mehmood, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

The birth of an infant with ambiguous genitalia generates difficult multiple medical, surgical, ethical, psychosocial, and physical issues for patients and their parents. Phenotypic sex results from the differentiation of internal ducts and external genitalia under the influence of hormones and other additional factors. When discordance occurs among three process es (chromosomal, gonadal, phenotypic sex determination), a DSD is the result. Terminology such as hermaphrodite, pseudo-hermaphrodite, and intersex, are considered to be pejorative and dated. These terms have been replaced by the term disorders of sexual development (DSD) by the consensus statement on management of intersex disorders. Disorders of sexual …

Ileal Atresia, Obiyo O. Osuchukwu, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

Ileal and jejunal atresias are usually described together as jejunoileal atresia (JIA). JIA is a common cause of intestinal obstruction in neonates. It is seen in 1 in 5000 to 1 in 14000 live births. Intestinal atresia can occur in any location on the small bowel as a solitary or even multiple lesions. Distally located atresia usually presents with delayed symptoms compared to proximal ones. Occasionally, JIA is associated with other malformations such as cardiac anomalies, gastroschisis, and cystic fibrosis. Evaluation can be initiated before birth with prenatal diagnosis using ultrasound findings of evidence of intestinal obstruction reported in 29% …

Gastroschisis, Rebecca M. Rentea, Vikas Gupta

Manuscripts, Articles, Book Chapters and Other Papers

Gastroschisis is a paraumbilical, full-thickness abdominal wall defect associated with protrusion of the bowel through the defect. It is rarely associated with genetic conditions. A membrane does not cover the bowel exposed in utero and, as a result, may be matted, dilated, and covered with a fibrinous inflammatory rind. Infants have a high proportion of intrauterine growth restriction. Diagnosis is often made on the 20-week ultrasound with free-floating bowel loops in the uterine cavity. Maternal serum alpha-fetoprotein (AFP) is elevated in pregnancies with gastroschisis. Compared with other abdominal wall defects diagnosed prenatally such as omphalocele, only 10 percent of cases …

Patent Urachus, Kayla B. Briggs, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

atent urachus refers to one condition in a rare spectrum of disorders referred to as urachal anomalies. These conditions result from the failure of the involution of normal embryologic tissues that serve to empty the fetal bladder. The location and amount of persistent tissue dictate the presenting symptoms. Some of these urachal anomalies are obvious at birth, while others are more subtle and not diagnosed until adulthood or only incidentally discovered after imaging is obtained for other reasons. Historically, surgical resection of urachal anomalies was routinely undertaken, given the potential for malignancy in retained ectopic tissue. Early surgical resection has …

Oophorectomy, Ashli A. Lawson, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

Oophorectomy is the surgical removal of the ovary and can be unilateral or bilateral. Hysterectomy is the most common major gynecologic surgery, but salpingo-oophorectomy remains a frequent concomitant surgery. Surgery for ovarian pathology alone is still often encountered in females of all ages. While surgical removal of one ovary does not grossly affect the hormonal milieu of a patient, bilateral oophorectomy will render a patient infertile and menopausal with ensuing physiologic changes and risks. Forward-thinking discussions should be held with the patient before undertaking an oophorectomy.

Torsion Of An Accessory Spleen In A Child With Biliary Atresia Splenic Malformation Syndrome., David A. Simon, Nathan R. Fleishman Md, Pamala Choi, Jason D. Fraser, Ryan T. Fischer

Manuscripts, Articles, Book Chapters and Other Papers

Torsion of an accessory spleen is an exceedingly rare cause of abdominal pain in pediatric patients. The diagnosis is frequently challenging as presentation is variable and diagnostic imaging can be aspecific. The current case describes an unusual presentation of a torted accessory spleen in a 5-year-old girl with biliary atresia splenic malformation syndrome who initially presented with non-specific abdominal symptoms and fever. The diagnosis was made following fine-needle aspiration of a suspected intraabdominal abscess. The case highlights the diagnostic challenge of accessory splenic torsion and stresses the importance of its inclusion on the differential diagnosis of pediatric patients, especially those …

Incidence Of Initial Renal Replacement Therapy Over The Course Of Kidney Disease In Children., Derek K. Ng, Matthew B. Matheson, Bradley A. Warady, Susan R. Mendley, Susan L. Furth, Alvaro Muñoz

Manuscripts, Articles, Book Chapters and Other Papers

The Chronic Kidney Disease in Children Study, a prospective cohort study with data collected from 2003 to 2018, provided the first opportunity to characterize the incidence of renal replacement therapy (RRT) initiation over the life course of pediatric kidney diseases. In the current analysis, parametric generalized gamma models were fitted and extrapolated for RRT overall and by specific treatment modality (dialysis or preemptive kidney transplant). Children were stratified by type of diagnosis: nonglomerular (mostly congenital; n = 650), glomerular-hemolytic uremic syndrome (HUS; n = 49), or glomerular-non-HUS (heterogeneous childhood onset; n = 216). Estimated durations of time to RRT after …

Anticipation, Accompaniment, And A Good Death In Perinatal Care., Bryanna S. Moore, Brian S. Carter, Bryan Beaven, Katie House, Joel House

Manuscripts, Articles, Book Chapters and Other Papers

The ethics of perinatal care, and the experiences of families who receive such care, remains a nascent area of inquiry. It can be hard to see how existing "good death" constructs apply to the experiences of fetal patients and their families. In this paper, we explore two themes raised by a case at our fetal health center: anticipation and accompaniment. In this case, a mother presented to our fetal health center; her unborn son, our fetal patient, was diagnosed with life-threatening hypoplastic left heart syndrome and endocardial fibroelastosis. The parents were told that their son's life expectancy, upon birth, was …

Transanal-Only Swenson-Like Pull-Through For Late Diagnosed Hirschsprung Disease., Rebecca M. Rentea, Devin R. Halleran, Hira Ahmad, Elias Maloof, Richard J. Wood, Marc A. Levitt

Manuscripts, Articles, Book Chapters and Other Papers

Hirschsprung disease (HD) is an obstructive colonic process usually diagnosed in the neonatal period. A small subset of cases are diagnosed late, present with severe constipation without enterocolitis and have low rectosigmoid disease. A transanal-only pull-through is a well-described approach but in the newborn period risks a situation whereby the transition zone is higher than the sigmoid. We present our experience with the unique patient population of older HD patients in whom the transition zone was reliably reachable via a single-stage transanal approach, performed in prone position. Patients between 2 and 6 years of age with a rectal or sigmoid …

Dr. Ahmed Abdelmoity To Receive Endowed Chair, Children's Mercy Hospital

Our Story Continues

Former Children’s Mercy CEO Rand O’Donnell, PhD, and his wife, Melva, are establishing an endowed chair position that will be awarded to Ahmed Abdelmoity, MD, FAAP, Division Director of Child Neurology, Section Chief of Epilepsy and Neurophysiology. Dr. Abdelmoity will become our 31st endowed chair at Children’s Mercy.

Unraveling Complexity About Childhood Obesity And Nutritional Interventions: Modeling Interactions Among Psychological Factors., Keith Feldman, Gisela M B Solymos, Maria Paula De Albuquerque, Nitesh V. Chawla

Manuscripts, Articles, Book Chapters and Other Papers

As the global prevalence of childhood obesity continues to rise, researchers and clinicians have sought to develop more effective and personalized intervention techniques. In doing so, obesity interventions have expanded beyond the traditional context of nutrition to address several facets of a child's life, including their psychological state. While the consideration of psychological features has significantly advanced the view of obesity as a holistic condition, attempts to associate such features with outcomes of treatment have been inconclusive. We posit that such uncertainty may arise from the univariate manner in which features are evaluated, focusing on a particular aspect such as …

An Electronic Medical Records-Based Approach To Identify Idiosyncratic Drug-Induced Liver Injury In Children., Tracy L. Sandritter, Jennifer Goldman, Clayton J Habiger, James F. Daniel, Jennifer Lowry, Ryan T. Fischer

Manuscripts, Articles, Book Chapters and Other Papers

Drug-induced liver injury (DILI) is the leading cause of liver failure in the United States and the most common cause of drug recall. As opposed to the recognized direct toxicity of super-therapeutic acetaminophen or chemotherapeutic agents in children, limited data exists for pediatric populations on the incidence of idiosyncratic DILI (iDILI) that may develop independently of drug dose or duration of administration. To improve the detection of adverse drug reactions at our hospital, we utilized electronic medical records-based automated trigger tools to alert providers of potential iDILI. Clinical criteria concerning for iDILI were defined as serum ALT > 5x or serum …

Msto1 Mutations Cause Mtdna Depletion, Manifesting As Muscular Dystrophy With Cerebellar Involvement., S Donkervoort, R Sabouny, P Yun, L Gauquelin, K R Chao, Y Hu, I Al Khatib, A Töpf, P Mohassel, B B Cummings, R Kaur, D Saade, S A Moore, L B Waddell, M A Farrar, J K Goodrich, P Uapinyoying, S H S Chan, A Javed, M E Leach, P Karachunski, J Dalton, L Medne, A Harper, C Thompson, Isabelle Thiffault, S Specht, R E Lamont, Carol J. Saunders, H Racher, F P Bernier, D Mowat, N Witting, J Vissing, R Hanson, Keith A. Coffman, Meagan K. Hainlen, J S Parboosingh, A Carnevale, G Yoon, R E Schnur, Care4rare Canada Consortium, K M Boycott, J K Mah, V Straub, A Reghan Foley, A M Innes, C G Bönnemann, T E Shutt

Manuscripts, Articles, Book Chapters and Other Papers

MSTO1 encodes a cytosolic mitochondrial fusion protein, misato homolog 1 or MSTO1. While the full genotype-phenotype spectrum remains to be explored, pathogenic variants in MSTO1 have recently been reported in a small number of patients presenting with a phenotype of cerebellar ataxia, congenital muscle involvement with histologic findings ranging from myopathic to dystrophic and pigmentary retinopathy. The proposed underlying pathogenic mechanism of MSTO1-related disease is suggestive of impaired mitochondrial fusion secondary to a loss of function of MSTO1. Disorders of mitochondrial fusion and fission have been shown to also lead to mitochondrial DNA (mtDNA) depletion, linking them to the mtDNA …