Digital Commons Network^™

Endoscopic Mucosal Resection: Tips And Tricks For Gastrointestinal Trainees, Tessa Herman, Bryant Megna, Kumar Pallav, Mohammad Bilal

Faculty, Staff and Student Publications

Endoscopic mucosal resection (EMR) is a minimally invasive, specialized endoscopic technique that is useful in resecting superficial gastrointestinal lesions that are unable to be removed by standard polypectomy. This is typically accomplished using a submucosal injection that lifts the lesion away from the muscularis propria to allow for safe resection of the polyp, either via piecemeal or en bloc resection. Over the years, several techniques exist to perform EMR including conventional EMR or hot EMR, cold EMR and underwater EMR. Despite its established advantages and safety over conventional techniques such as surgery, the adoption of endoscopic resection (and thus the …

Are There Sex Differences In Interactive Associations Of Environmental Exposure To Lead (Pb), Mercury (Hg), And Manganese (Mn) With Gst Genes (Gstp1, Gstt1, And Gstm1) In Relation To Asd In Jamaican Children?, Hiba T Zwiya, Maureen Samms-Vaughan, Jan Bressler, Minjae Lee, Courtney Byrd-Williams, Manouchehr Hessabi, Megan L Grove, Sydonnie Shakespeare-Pellington, Katherine A Loveland, Mohammad H Rahbar

Faculty, Staff and Student Publications

Background: Male preponderance is well-established in children with autism spectrum disorder (ASD). Glutathione S-transferase (GST) genes play a crucial role in suppressing oxidative stress triggered by environmental stressors.

Objective: To determine whether the association between metals and ASD differs by sex and by GST genes.

Methods: Using data from 344 pairs of sex-and age-matched cases and controls, we assessed the association of each metal with ASD or ASD severity, by applying conditional logistic regression (CLR) or general linear models (GLM). Sex was assessed as an effect modifier in separate GST genetic models.

Results: For Pb exposure, using a recessive model …

Advancing Asms With Lc-Ms/Ms For The Discovery Of Novel Pdcl2 Ligands From Dna-Encoded Chemical Library Selections, Qiuji Ye, Hassane Belabed, Yong Wang, Zhifeng Yu, Murugesan Palaniappan, Jian-Yuan Li, Stacey A Kalovidouris, Kevin R Mackenzie, Mingxing Teng, Damian W Young, Yoshitaka Fujihara, Martin M Matzuk

Faculty, Staff and Students Publications

BACKGROUND: A safe, effective, and reversible nonhormonal male contraceptive drug is greatly needed for male contraception as well as for circumventing the side effects of female hormonal contraceptives. Phosducin-like 2 (PDCL2) is a testis-specific phosphoprotein in mice and humans. We recently found that male PDCL2 knockout mice are sterile due to globozoospermia caused by impaired sperm head formation, indicating that PDCL2 is a potential target for male contraception. Herein, our study for the first time developed a biophysical assay for PDCL2 allowing us to screen a series of small molecules, to study structure-activity relationships, and to discover two PDCL2 binders …

Geospatial Distribution Of Obsessive-Compulsive Disorder Specialists: Understanding Access As A Function Of Distance, Insurance Status, And Neighborhood Socioeconomic Status, Abiodun O Oluyomi, Sophie C Schneider, Catherine Christian, Juan M Alvarez, Orri Smárason, Wayne K Goodman, Eric A Storch

Faculty, Staff and Students Publications

Obsessive-compulsive disorder is an impairing psychiatric condition affecting 1-2% of adults and youth. Cognitive-behavioral therapy with exposure and response prevention (CBT) is an efficacious intervention but requires specialty training and access is often limited. While certain factors are associated with treatment access, one key barrier that has not been explored is the geographic availability of OCD treatment providers. Using integrated geographically-referenced data, we examined the geographic distribution of OCD CBT specialty providers across the state of Texas, with particular attention to the relationship to neighborhood socioeconomic disadvantage, insurance status, and rural versus urban status. We found that specialist providers are …

Closure Of Refractory Gastrocutaneous Fistula With Endoscopically Guided Percutaneous Suturing With The Use Of Miniature Biopsy Forceps, Rogelio Garcia, Juan D Gomez Cifuentes, Fuad Aloor, Tara Keihanian, Marvin Ryou, Wasif M Abidi

Faculty, Staff and Students Publications

A PEG tube is an ideal option for enteral nutrition in patients with impaired swallowing or for nutrition optimization. After PEG tube removal, the tract usually closes spontaneously. However, persistent gastrocutaneous fistula (GCF) is a rare but well-known adverse event of long-term PEG tube use.¹ Despite the evolution of endoscopic techniques over the last decade, such as over-the-scope clips (OTSCs), endoscopic suturing, and biosynthetic glue plaque, the management of GCF remains challenging.2, 3, 4, 5, 6 Although endoscopic suturing results in full-thickness closure of the gastric wall, persistent leakage from the cutaneous site may …

Realizing The Promise Of Dolutegravir In Effectively Treating Children And Adolescents Living With Hiv In Real-World Settings In 6 Countries In Eastern And Southern Africa, Jason Michael Bacha, Sandile Dlamini, Florence Anabwani, Judith Gwimile, Jacqueline Balungi Kanywa, John Farirai, Menard Bvumbwe, Mabene Tsotako, Teresa Steffy, Diane Nguyen, Jose Euberto Mendez-Reyes, Peter Elyanu, Heather Haq

Faculty, Staff and Students Publications

BACKGROUND: Despite encouraging results from clinical trials and in high-income countries, large-scale data on the effectiveness and safety of dolutegravir (DTG) in children and adolescents living with HIV (CALHIV) are lacking in low- and middle-income countries (LMICs).

METHODS: Retrospective analysis was performed among CALHIV 0-19 years old and weighing greater than or equal to 20 kg who received DTG from 2017 to 2020 at sites in Botswana, Eswatini, Lesotho, Malawi, Tanzania and Uganda to determine effectiveness, safety and predictors of viral load suppression (VLS) among CALHIV using DTG, including through single drug substitutions (SDS).

RESULTS: Among 9419 CALHIV using DTG, …

Predictors, Disparities, And Facility-Level Variation: Sglt2 Inhibitor Prescription Among Us Veterans With Ckd, L Parker Gregg, David J Ramsey, Julia M Akeroyd, Shehrezade A Jafry, Michael E Matheny, Salim S Virani, Sankar D Navaneethan

Faculty, Staff and Students Publications

RATIONALE & OBJECTIVE: Sodium/glucose cotransporter 2 (SGLT2) inhibitors are recommended for type 2 diabetes mellitus (T2DM) in patients with chronic kidney disease (CKD) or atherosclerotic cardiovascular disease (ASCVD). We evaluated factors associated with SGLT2 inhibitor prescription, disparities by race and sex, and facility-level variation in prescription patterns.

STUDY DESIGN: Retrospective cohort.

SETTING & PARTICIPANTS: A national sample of US veterans with comorbid T2DM, CKD, and ASCVD with a primary care visit between January 1 and December 31, 2020.

EXPOSURE: Race, sex, and individual Veterans Affairs (VA) location.

OUTCOME: SGLT2 inhibitor prescription.

ANALYTICAL APPROACH: Multivariable logistic regression assessed associations of race …

Vincristine/Irinotecan/Temsirolimus Upfront Window Treatment Of High-Risk Hepatoblastoma: A Report From The Children’S Oncology Group Ahep0731 Study Committee, Patrick A Thompson, Marcio H Malogolowkin, Wayne L Furman, Jin Piao, Mark D Krailo, Nadia Chung, Lindsay Brock, Alexander J Towbin, Elizabeth B Mccarville, Milton J Finegold, Sarangarajan Ranganathan, Stephen P Dunn, Max R Langham, Eugene D Mcgahren, Gregory M Tiao, Christopher B Weldon, Allison F O'Neill, Carlos Rodriguez-Galindo, Rebecka L Meyers, Howard M Katzenstein

Faculty, Staff and Students Publications

Background: Survival for children with metastatic hepatoblastoma (HB) remains suboptimal. We report the response rate and outcome of two courses of vincristine/irinotecan/temsirolimus (VIT) in children with high-risk (HR)/metastatic HB.

Procedures: Patients with newly diagnosed HB received HR window chemotherapy if they had metastatic disease or a serum alpha-fetoprotein (AFP) level less than 100 ng/mL. Patients received vincristine (days 1 and 8), irinotecan (days 1-5), and temsirolimus (days 1 and 8). Cycles were repeated every 21 days. Responders had either a 30% decrease using RECIST (Response Evaluation Criteria in Solid Tumors) criteria OR a 90% (>1 log10 decline) AFP decline …

Prematurity Blunts Protein Synthesis In Skeletal Muscle Independently Of Body Weight In Neonatal Pigs, Marko Rudar, Jane K Naberhuis, Agus Suryawan, Hanh V Nguyen, Marta L Fiorotto, Teresa A Davis

Faculty, Staff and Students Publications

Background

Postnatal growth failure in premature infants is associated with reduced lean mass accretion. Prematurity impairs the feeding-induced stimulation of translation initiation and protein synthesis in the skeletal muscle of neonatal pigs. The objective was to determine whether body weight independently contributes to the blunted postprandial protein synthesis.

Methods

Preterm and term pigs that were either fasted or fed were stratified into quartiles according to birth weight to yield preterm and term groups of similar body weight; first and second quartiles of preterm pigs and third and fourth quartiles of term pigs were compared (preterm-fasted, n = 23; preterm-fed, n …

The Systemic Availability Of Indispensable Amino Acids From Orally Ingested Algal And Legume Protein In Young Children At Risk Of Environmental Enteric Dysfunction, Nirupama Shivakumar, Sindhu Kashyap, Farook Jahoor, Sarita Devi, Thomas Preston, Tinku Thomas, Anura V Kurpad

Faculty, Staff and Students Publications

Background: The digestion and absorption of ingested protein may be reduced in children with environmental enteric dysfunction (EED), reducing systemic amino acid availability for protein synthesis with resultant growth faltering. This has not been directly measured in children with EED and associated growth faltering.

Objectives: To evaluate the systemic availability of algal (spirulina) and legume (mung bean) indispensable amino acids (IAAs) in children with EED.

Methods: Indian children (18-24 mo) from urban slums were assigned to EED (n = 24) or no-EED (control, n = 17) groups based on the lactulose rhamnose test, where the lactulose rhamnose ratio cutoff for …

Diabetes Technology Meeting 2022, Jingtong Huang, Andrea M Yeung, Ashley Y Dubord, Howard Wolpert, Peter G Jacobs, Wei-An Lee, Andjela Drincic, Elias K Spanakis, Jennifer L Sherr, Priya Prahalad, Alexander Fleming, Victoria C Hsiao, Tejaswi Kompala, Rayhan A Lal, Maya Fayfman, Barry H Ginsberg, Rodolfo J Galindo, Andreas Stuhr, J Geoffrey Chase, Bijan Najafi, Umesh Masharani, Jane Jeffrie Seley, David C Klonoff

Center on Aging Staff Publications

Diabetes Technology Society hosted its annual Diabetes Technology Meeting from November 3 to November 5, 2022. Meeting topics included (1) the measurement of glucose, insulin, and ketones; (2) virtual diabetes care; (3) metrics for managing diabetes and predicting outcomes; (4) integration of continuous glucose monitor data into the electronic health record; (5) regulation of diabetes technology; (6) digital health to nudge behavior; (7) estimating carbohydrates; (8) fully automated insulin delivery systems; (9) hypoglycemia; (10) novel insulins; (11) insulin delivery; (12) on-body sensors; (13) continuous glucose monitoring; (14) diabetic foot ulcers; (15) the environmental impact of diabetes technology; and (16) spinal …

The Predictive Performance Of Contemporary Guideline Recommendations For Helicobacter Pylori Testing In A United States Population, Theresa Nguyen Wenker, Frederick B Peng, Ikenna Emelogu, Niharika Mallepally, Fasiha Kanwal, Hashem B El-Serag, Mimi C Tan

Faculty, Staff and Students Publications

BACKGROUND: The Houston Consensus Conference and American College of Gastroenterology (ACG) have recommended Helicobacter pylori screening in United States populations with specific risk factors. However, the performance of these guidelines in clinical practice is not known.

METHODS: We identified consecutive patients undergoing upper endoscopy with gastric biopsies for any indication in a safety-net hospital in Houston, TX during January 2015-December 2016. We tested the association between the presence of H pylori (histopathology, stool antigen, urea breath test, immunoglobulin G serology, or prior treatment) and H pylori risk factors using logistic regression models, reported as odds ratios and 95% confidence intervals …

Oropharyngeal Teratoma: Five-Month-Old Presenting With Failure To Thrive And Severe Obstructive Sleep Apnea., Anna Lawrence, Melissa Gener, Shao Jiang, Jill M. Arganbright

Manuscripts, Articles, Book Chapters and Other Papers

Oropharyngeal teratomas are an extremely rare congenital tumor. They are often diagnosed prenatally and can cause significant airway obstruction and feeding difficulties at birth. We present a five-month-old female that was diagnosed with a palatal teratoma that presented with failure to thrive, difficulty feeding, and eventually with severe obstructive sleep apnea. We present a five-month-old term, otherwise healthy female who became stridulous after an episode of the respiratory syncytial virus at one month old. At three months old, an otolaryngologist diagnosed mild laryngomalacia with no mass identified, and no surgical intervention was recommended. Due to continued poor weight gain, at …

Association Of Chronic Pancreatitis Pain Features With Physical, Mental, And Social Health, Dhiraj Yadav, Robert L Askew, Tonya Palermo, Liang Li, Dana K Andersen, Minxing Chen, William E Fisher, Evan L Fogel, Christopher E Forsmark, Phil A Hart, Mohamed O Othman, Stephen J Pandol, Walter G Park, Mark D Topazian, Stephen K Van Den Eeden, Santhi Swaroop Vege, Yunlong Yang, Jose Serrano, Darwin L Conwell, Consortium For The Study Of Chronic Pancreatitis, Diabetes, Pancreatic Cancer (Cpdpc)

Faculty, Staff and Students Publications

BACKGROUND AND AIMS: Pain is a cardinal symptom of chronic pancreatitis (CP). Using Patient-Reported Outcomes Measurement Information System (PROMIS) measures, we characterized physical and mental health and symptom profiles of a well-defined cohort of individuals with CP and compared them with control subjects. Among patients with CP, we also examined associations between pain (intensity, temporal nature) and PROMIS symptom profiles and the prevalence of clinically significant psychological comorbidities.

METHODS: We analyzed baseline data in 488 CP patients and 254 control subjects enrolled in PROCEED (Prospective Evaluation of Chronic Pancreatitis for Epidemiologic and Translational Studies), an ongoing longitudinal cohort study. Participants …

Resolved Peristomal Erosive Papulonodular Dermatitis Mimicking Nevoid Hyperkeratosis Of The Nipple And Areola, Christa M. Tomc, Daniel A. Nguyen, Joseph S. Susa, Stephen E. Weis

HCA Healthcare Journal of Medicine

Introduction

Nevoid hyperkeratosis of the nipple and areola (NHKNA) is a rare cutaneous entity with a distinct clinical and histological presentation. The type II form of this condition can result from various dermatoses, such as irritant contact dermatitis. Erosive papulonodular dermatitis is a chronic irritant dermatitis that often occurs in areas of occlusion and maceration, such as peristomal skin. Pseudoverrucous papules and nodules are a variant of erosive papulonodular dermatitis and have a non-specific histologic pattern of reactive hyperplasia.

Case Presentation

We present a case of a patient with resolved peristomal erosive papulonodular dermatitis who presented status-post ileostomy reversal with …

Transpyloric Feed In Reflux-Associated Apnea In Preterm Newborns: A Prospective Study, Tamoghna Biswas, Tapas K. Sabui, Somosri Roy, Rakesh Mondal, Shubhabrata Majumdar, Sudipta Misra

HCA Healthcare Journal of Medicine

Background

The etiological correlation between gastroesophageal reflux (GER) and apnea is controversial. We conducted a prospective interventional study designed to address the controversy.

Methods

Preterm neonates with apnea at a tertiary care center, who had clinical features of GER without any other comorbidities likely to cause apnea, were included in the study. The enrolled neonates underwent continuous transpyloric tube feeding for 72 hours. The primary outcome measure was the difference in the number of apneic episodes pre- and post-initiation of nasoduodenal (ND) feeding. Secondary outcome measures included the incidence of necrotizing enterocolitis, other gastrointestinal disturbances, and mortality.

Results

Sixteen preterm …

An Overview Of Risk Factors For Androgenetic Alopecia Among Women At Pasar Ambacang, Kuranji, Padang, Lidya Aprilia Sugma, Satya Wydya Yenny, Roslaili Rasyid

Journal of General - Procedural Dermatology and Venereology Indonesia

Background: Androgenetic alopecia (AGA) is a patterned hair loss due to chronic and progressive miniaturization of hair follicles. Hair represents femininity and self-confidence in women. Therefore, AGA may affect the quality of life and psychology in women. This study aims to describe the risk factors for AGA in women living in Pasar Ambacang Sub-district, Kuranji District, Padang City.

Method: This was a descriptive, cross-sectional study that included 40 women with AGA. Interviews with subjects were conducted. This study was approved by the Health Research Ethics Committee at Universitas Andalas (252/UN.16.2/KEPK-FK/2021)

Results: AGA was mostly found on patients …

Single Leg Depth Jump Kombinasi Core Plank Knee To Elbow Terhadap Power Otot Tungkai Atlet, Maya Triyanita, Siti Sardianti Dwi Tirta

Jurnal Fisioterapi Terapan Indonesia

Power limit training is defined as the movement to get a jump on athletes in doing the maximum jump smash. Badminton requires leg muscle explosive power because it is the muscle's ability to overcome loads and with a high speed of contraction. This study aims to determine the effect of the single leg depth jump exercise, the combination of the knee to elbow core plank, on increasing leg power in badminton athletes.

This study uses an experimental design method. This research was conducted on badminton athletes using a total sampling technique of 13 people, namely the Pliometric Simgle Leg Dept …

Co-Targeting Fasn And Mtor Suppresses Uveal Melanoma Growth, Anna Han, Dzmitry Mukha, Vivian Chua, Timothy J. Purwin, Manoela Tiago, Bhavik Modasia, Usman Baqai, Jenna Aumiller, Nelisa Bechtel, Emily Hunter, Meggie Danielson, Mizue Terai, Philip B. Wedegaertner, Takami Sato, Solange Landreville, Michael A. Davies, Stefan Kurtenbach, William J. Harbour, Zachary T. Schug, Andrew E. Aplin

Department of Pharmacology, Physiology, and Cancer Biology Faculty Papers

Uveal melanoma (UM) displays a high frequency of metastasis; however, effective therapies for metastatic UM are limited. Identifying unique metabolic features of UM may provide a potential targeting strategy. A lipid metabolism protein expression signature was induced in a normal choroidal melanocyte (NCM) line transduced with GNAQ (Q209L), a driver in UM growth and development. Consistently, UM cells expressed elevated levels of fatty acid synthase (FASN) compared to NCMs. FASN upregulation was associated with increased mammalian target of rapamycin (mTOR) activation and sterol regulatory element-binding protein 1 (SREBP1) levels. FASN and mTOR inhibitors alone significantly reduced UM cell growth. Concurrent …

Challenges In Diagnosing Staphylococcal Scalded Skin Syndrome In Children With Kwashiorkor: A Case Report, Christie Hamdali, Sondang Sirait, Adria Rusvita, Bawono Bhakti

Journal of General - Procedural Dermatology and Venereology Indonesia

Staphylococcal scalded skin syndrome (SSSS) is a type of exfoliating skin disease with high incidence in children. This condition can cause serious morbidity and even mortality with certain comorbid diseases such as malnutrition. Malnutrition could mask the diagnosis of SSSS because of its similarity to numerous differential diagnoses. Accuracy of clinical diagnosis is then essential to determine the proper initial management. This case report presents a 6-month-old male baby with staphylococcal scalded skin syndrome and kwashiorkor. The patient presented with complaints of multiple erythematous plaques and widespread vesicular lesions that subsequently ruptured, resulting in erosions. Physical examination revealed edema, coarse …

Co-Targeting Fasn And Mtor Suppresses Uveal Melanoma Growth, Anna Han, Dzmitry Mukha, Vivian Chua, Timothy J Purwin, Manoela Tiago, Bhavik Modasia, Usman Baqai, Jenna L Aumiller, Nelisa Bechtel, Emily Hunter, Meggie Danielson, Mizue Terai, Philip B Wedegaertner, Takami Sato, Solange Landreville, Michael A Davies, Stefan Kurtenbach, J William Harbour, Zachary T Schug, Andrew E Aplin

Faculty, Staff and Student Publications

Uveal melanoma (UM) displays a high frequency of metastasis; however, effective therapies for metastatic UM are limited. Identifying unique metabolic features of UM may provide a potential targeting strategy. A lipid metabolism protein expression signature was induced in a normal choroidal melanocyte (NCM) line transduced with GNAQ (Q209L), a driver in UM growth and development. Consistently, UM cells expressed elevated levels of fatty acid synthase (FASN) compared to NCMs. FASN upregulation was associated with increased mammalian target of rapamycin (mTOR) activation and sterol regulatory element-binding protein 1 (SREBP1) levels. FASN and mTOR inhibitors alone significantly reduced UM cell growth. Concurrent …

Role Of Glucose Metabolic Reprogramming In Breast Cancer Progression And Drug Resistance, Pan Lei, Wenzhou Wang, Marisela Sheldon, Yutong Sun, Fan Yao, Li Ma

Faculty, Staff and Student Publications

The involvement of glucose metabolic reprogramming in breast cancer progression, metastasis, and therapy resistance has been increasingly appreciated. Studies in recent years have revealed molecular mechanisms by which glucose metabolic reprogramming regulates breast cancer. To date, despite a few metabolism-based drugs being tested in or en route to clinical trials, no drugs targeting glucose metabolism pathways have yet been approved to treat breast cancer. Here, we review the roles and mechanisms of action of glucose metabolic reprogramming in breast cancer progression and drug resistance. In addition, we summarize the currently available metabolic inhibitors targeting glucose metabolism and discuss the challenges …

Commentary On The Obesity Epidemic: A Family Medicine Perspective, Mediha B. Chaudhry, Katherine Goth

Transformative Medicine

No abstract provided.

Targeting The Vulnerabilities Of Oncogene Activation, Christina Huang, Jack L. Arbiser

Student Papers, Posters & Projects

No abstract provided.

Randomized Intervention And Outpatient Follow-Up Lowers 30-D Readmissions For Patients With Hepatic Encephalopathy, Decompensated Cirrhosis, Antoinette Pusateri, Kevin Litzenberg, Claire Griffiths, Caitlin Hayes, Bipul Gnyawali, Michelle Manious, Sean G Kelly, Lanla F Conteh, Sajid Jalil, Haikady N Nagaraja, Khalid Mumtaz

Faculty, Staff and Student Publications

BACKGROUND: We previously reported national 30-d readmission rates of 27% in patients with decompensated cirrhosis (DC).

AIM: To study prospective interventions to reduce early readmissions in DC at our tertiary center.

METHODS: Adults with DC admitted July 2019 to December 2020 were enrolled and randomized into the intervention (INT) or standard of care (SOC) arms. Weekly phone calls for a month were completed. In the INT arm, case managers ensured outpatient follow-up, paracentesis, and medication compliance. Thirty-day readmission rates and reasons were compared.

RESULTS: Calculated sample size was not achieved due to coronavirus disease 2019; 240 patients were randomized into …

Spatial Single-Cell Sequencing Of Meiosis I Arrested Oocytes Indicates Acquisition Of Maternal Transcripts From The Soma, Kenneth A Trimmer, Peisen Zhao, Jacob Seemann, Shin-Yu Chen, Sudip Mondal, Adela Ben-Yakar, Swathi Arur

Faculty, Staff and Student Publications

Maternal RNAs are stored from minutes to decades in oocytes throughout meiosis I arrest in a transcriptionally quiescent state. Recent reports, however, propose a role for nascent transcription in arrested oocytes. Whether arrested oocytes launch nascent transcription in response to environmental or hormonal signals while maintaining the meiosis I arrest remains undetermined. We test this by integrating single-cell RNA sequencing, RNA velocity, and RNA fluorescence in situ hybridization on C. elegans meiosis I arrested oocytes. We identify transcripts that increase as the arrested meiosis I oocyte ages, but rule out extracellular signaling through ERK MAPK and nascent transcription as a …

Detrimental Effects Of Pcsk9 Loss-Of-Function In The Pediatric Host Response To Sepsis Are Mediated Through Independent Influence On Angiopoietin-1., Mihir R. Atreya, Natalie Z. Cvijanovich, Julie C. Fitzgerald, Scott L. Weiss, Michael T. Bigham, Parag N. Jain, Adam J. Schwarz, Riad Lutfi, Jeffrey Nowak, Geoffrey L. Allen, Neal J. Thomas, Jocelyn R. Grunwell, Torrey Baines, Michael Quasney, Bereketeab Haileselassie, Matthew N. Alder, Patrick Lahni, Scarlett Ripberger, Adesuwa Ekunwe, Kyle R. Campbell, Keith R. Walley, Stephen W. Standage

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Sepsis is associated with significant mortality. Yet, there are no efficacious therapies beyond antibiotics. PCSK9 loss-of-function (LOF) and inhibition, through enhanced low-density lipoprotein receptor (LDLR) mediated endotoxin clearance, holds promise as a potential therapeutic approach among adults. In contrast, we have previously demonstrated higher mortality in the juvenile host. Given the potential pleiotropic effects of PCSK9 on the endothelium, beyond canonical effects on serum lipoproteins, both of which may influence sepsis outcomes, we sought to test the influence of PCSK9 LOF genotype on endothelial dysfunction.

METHODS: Secondary analyses of a prospective observational cohort of pediatric septic shock. Genetic variants …

Hyperinsulinemic Hypoglycemia And Growth Hormone Deficiency Secondary To 20p11 Deletion., Erica Wee, John Herriges, Kavitha Dileepan, Sarah Tsai, Joseph Alaimo, Emily Paprocki

Manuscripts, Articles, Book Chapters and Other Papers

Hypoglycemia is concerning for neurological complications in infants and children. Determining the cause of hypoglycemia is essential in providing appropriate treatment. Hyperinsulinism and growth hormone deficiency are known causes of hypoglycemia but are not commonly found together. We report a 4-month-old boy who presented with severe hypoglycemia and was found to have both hyperinsulinism and growth hormone deficiency. Treatment with both recombinant human growth hormone and diazoxide led to blood glucose normalization. Subsequently, he was found to have a genetic diagnosis of 20p11.22p11.21 deletion. 20p11 deletions have been associated with hypopituitarism, most commonly seen in growth hormone deficiency causing hypoglycemia. …

Perinatal Palliative Care In Sub-Saharan Africa: Recommendations For Practice, Future Research, And Guideline Development., Mahlet Abayneh, Sharla Rent, Peter Odion Ubuane, Brian S. Carter, Solomie Jebessa Deribessa, Betelehem B. Kassa, Atnafu Mekonnen Tekleab, Stephanie K. Kukora

Manuscripts, Articles, Book Chapters and Other Papers

Worldwide, sub-Saharan Africa has the highest burden of global neonatal mortality (43%) and neonatal mortality rate (NMR): 27 deaths per 1,000 live births. The WHO recognizes palliative care (PC) as an integral, yet underutilized, component of perinatal care for pregnancies at risk of stillbirth or early neonatal death, and for neonates with severe prematurity, birth trauma or congenital anomalies. Despite bearing a disproportionate burden of neonatal mortality, many strategies to care for dying newborns and support their families employed in high-income countries (HICs) are not available in low-and-middle-income countries (LMICs). Many institutions and professional societies in LMICs lack guidelines or …

Prevalence Of Iron Deficiency In Patients With Inherited Bleeding Disorders, Thomas Cochran, Brian R. Lee, Shannon L. Carpenter

Posters

Background: Patients with bleeding disorders have greater propensity for blood loss and therefore may have a higher prevalence of iron deficiency (ID) when compared to the general population. However, current literature focuses on adolescent females and few studies have assessed the overall prevalence of ID in children with inherited bleeding disorders. Aims: This study aims to identify the prevalence of ID in children with an inherited bleeding disorder. Methods: A retrospective analysis of children with any inherited bleeding disorder seen in Children’s Mercy Hospital’s Hemophilia Treatment Center between 2010 and 2020 was performed. Iron deficiency was defined by recently published …