Medical Specialties Commons^™

Gastroesophageal Reflux Disease: A General Overview, Julia Fashner

HCA Healthcare Journal of Medicine

Gastroesophageal reflux disease (GERD) varies in presentation and the patient’s symptoms of regurgitation in the throat or epigastric pain do not necessarily correlate with the severity of their disease. This general overview of GERD will include information on guidelines and diagnostic testing; lifestyle, medical and surgical management; and GERD in special populations. The pathophysiology of GERD is multifactorial, and a step-wise approach will assist physicians in making the diagnosis as GERD has a significant financial burden to the U.S. healthcare system.

Effect Of Maternal Retinol Status At Time Of Term Delivery On Retinol Placental Concentration, Intrauterine Transfer Rate, And Newborn Retinol Status, Melissa K. Thoene, Haley Haskett, Jeremy Furtado, Maranda Thompson, Matthew Van Ormer, Corrine K. Hanson, Ann Anderson-Berry

Journal Articles: Pediatrics

Retinol (vitamin A) is essential, so the objective of this Institutional Review Board approved study is to evaluate retinol placental concentration, intrauterine transfer, and neonatal status at time of term delivery between cases of maternal retinol adequacy, insufficiency, and deficiency in a United States population. Birth information and biological samples were collected for mother-infant dyads (n = 260). Maternal and umbilical cord blood retinol concentrations (n = 260) were analyzed by HPLC and categorized: deficient (≤0.7 umol/L), insufficient (>0.7-1.05 umol/L), adequate (>1.05 umol/L). Intrauterine transfer rate was calculated: (umbilical cord blood retinol concentration/maternal retinol concentration) × …

Peripubertal Serum Concentrations Of Organochlorine Pesticides And Semen Parameters In Russian Young Men., Ramy Abou Ghayda, Oleg Sergeyev, Jane S Burns, Paige L Williams, Mary M Lee, Susan A Korrick, Luidmila Smigulina, Yury Dikov, Russ Hauser, Lidia Mínguez-Alarcón

Department of Pediatrics Faculty Papers

BACKGROUND: Epidemiologic literature on the relation of organochlorine pesticides (OCPs) with semen quality among adult men has been inconclusive, and no studies have prospectively explored the association between peripubertal serum OCPs and semen parameters in young men.

OBJECTIVE: To evaluate prospective associations of peripubertal serum concentrations of hexachlorobenzene (HCB), β-hexachlorocylohexane (β-HCH), and p,p'-dichlorodiphenyldichloroethylene (p,p'-DDE) with semen parameters among young Russian men.

METHODS: This prospective cohort study included 152 young men who enrolled in the Russian Children's Study (2003-2005) at age 8-9 years and were followed annually until young adulthood. HCB, β-HCH, and p,p'-DDE concentrations were measured at the CDC by …

Gaining A “Foothold” On The Diagnosis Of Leishmaniasis, Brent M. Troy, Rebecca Hart, Navjyot Vidwan, Bethany Hodge

Journal of Refugee & Global Health

Pediatric refugees and immigrants may present with unusual diagnoses due to their extensive migration and potential harsh conditions in travel. Trauma and family separation add to the difficulty of obtaining a history of exposures. We report a case of one of the more commonly neglected tropical diseases, Leishmaniasis. A 15-year-old male refugee patient presented to the hospital with ulcerative lesions to his legs. His migration history was extensive, starting in Central Africa with travel to South America, followed by migration through Central America to Texas. The patient developed ulcerative lesions on his legs, and he was brought to the children’s …

The Intertwined Relationship Between Malnutrition And Poverty, Faareha Siddiqui, Rehana Salam, Zohra S. Lassi, Jai K. Das

Woman and Child Health

Despite social and economic development, the burden of malnutrition across the globe remains unacceptably high. A vital relationship exists between nutritional status, human capital, and economic standing. Malnutrition adversely affects the physiological and mental capacity of individuals; which in turn hampers productivity levels, making them and their respective countries more susceptible to poverty. A two-way link exists between malnutrition and poverty, creating a vicious cycle with each fueling the other. Malnutrition produces conditions of poverty by reducing the economic potential of the population and likewise, poverty reinforces malnutrition by increasing the risk of food insecurity. The aim of the paper …

Correction To: Epidemiology Of Patients Presenting To A Pediatric Emergency Department In Karachi, Pakistan, Nadir Ijaz, Matthew Strehlow, N Ewen Wang, Elizabeth Pirrotta, Areeba Tariq, Naseeruddin Mahmood, Swaminatha Mahadevan

Department of Paediatrics and Child Health

An amendment to this paper has been published and can be accessed via the original article.

The Slc25a42 Transcript Is A Biomarker For Fetal Reprogramming In Response To Placental Insufficiency In Preterm Newborns Under 32 Weeks Gestation-A Pilot Study., Fu-Sheng Chou, Pei-Shan Wang

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Timing of medical delivery of preterm newborns exposed to placental insufficiency is largely determined by umbilical artery blood flow and maternal clinical manifestations. There is a lack of tools to properly assess fetal body response to placental insufficiency before or upon delivery. Yet, short- and long-term comorbidities associated with placental insufficiency and the consequential intrauterine growth restriction may be a result of fetal response following prolonged stress. This study aims to establish a procedure to investigate fetal/neonatal transcriptional response to placental insufficiency as part of an initiative to identify cost-effective biomarkers for assessing fetal response to placental insufficiency.

Methods: …

Exploring The Influence Of Socio-Cultural Factors And Environmental Resources On The Health Related Quality Of Life Of Children And Adolescents After Congenital Heart Disease Surgery: Parental Perspectives From A Low Middle Income Country, Laila Ladak, Robyn Gallagher, Babar Hasan, Khadija Awais, Ahmed Abdullah, Janice Gullick

Department of Paediatrics and Child Health

Background: Health related quality of life (HRQOL) is an important indicator of long-term well-being, influenced by environmental factors such as family, culture, societal norms and available resources. This study aimed to explore parental perspectives on the influence of socio-cultural factors and environmental resources on the HRQOL of children and adolescents after congenital heart disease (CHD) surgery.
Methods: Using a descriptive, qualitative design, semi-structured interviews of children/adolescents who had CHD surgery in this low-middle income country (LMIC) were collected between July to December 2017. There were 20 families enrolled, which included 18 parent dyads (mother and father) and two single mothers, …

Not All Free Air In The Nicu Is Necrotizing Enterocolitis., Paul Holtrop, Paras Khandar, Nathan Novotny

Articles

When a tiny preterm baby in our neonatal intensive care unit (NICU) has an intestinal perforation, it is always necrotizing enterocolitis (NEC), right? Not so fast, my friend. Many of these preterm infants who perforate have free air from spontaneous intestinal perforation (SIP) and not NEC. As it turns out, not all free air in the NICU is NEC. How can you tell the difference and does it matter?

It can be challenging to tell the difference, but SIP tends to occur earlier in life, often within the first week, whereas NEC usually occurs slightly later. One analysis of a …

A Pragmatic Randomized Controlled Trial Of Multi-Dose Oral Ondansetron For Pediatric Gastroenteritis (The Dose-Age Study): Statistical Analysis Plan., Anna Heath, Juan David Rios, Sarah Williamson-Urquhart, Petros Pechlivanoglou, Martin Offringa, Christopher Mccabe, Gareth Hopkin, Amy C Plint, Andrew Dixon, Darcy Beer, Serge Gouin, Gary Joubert, Terry P Klassen, Stephen B Freedman

Paediatrics Publications

BACKGROUND: Acute gastroenteritis is a leading cause of emergency department visits and hospitalizations among children in North America. Oral-rehydration therapy is recommended for children with mild-to-moderate dehydration, but children who present with vomiting are frequently offered intravenous rehydration in the emergency department (ED). Recent studies have demonstrated that the anti-emetic ondansetron can reduce vomiting, intravenous rehydration, and hospitalization when administered in the ED to children with dehydration. However, there is little evidence of additional benefit from prescribing ondansetron beyond the initial ED dose. Moreover, repeat dosing may increase the frequency of diarrhea. Despite the lack of evidence and potential adverse …

Spotlight Commentary: Why We Need To Pay Attention To Toxicity Associated With Herbal Medicines, Elena Y. Enioutina, Kathleen M. Job, Catherine M.T. Sherwin

Pediatrics Faculty Publications

No abstract provided.

Specialized Nutritious Food Combined With Cash Transfers And Social And Behavior Change Communication To Prevent Stunting Among Children Aged 6 To 23 Months In Pakistan: Protocol For A Cluster Randomized Controlled Trial, Gul Nawaz Khan, Sumra Kureishy, Shabina Ariff, Atif Habib, Asra Abeer Usmani, Areeba Mubarik, Masawar Hussain, Naveed Akbar, Pablo Rodriguez De Castro, Alba Cecilia Garzon, Saskia De Pee, Sajid Bashir Soofi

Department of Paediatrics and Child Health

Background: In Pakistan, the prevalence of stunting in children younger than 5 years has remained above global critical levels over the past two decades, with the stunting rate being 40.2% in 2018. Children living in rural areas and in the poorest households suffer the most from stunting across the country-43.2% in rural areas and 51.4% in the lowest wealth quintile. As a continuing public health concern, it is essential that stunting prevention is a national priority in order to ensure human capital development, especially among the poorest households.
Objective: The primary objective of this study is to determine the effect …

The Community-Level Interventions For Pre-Eclampsia (Clip) Cluster Randomised Trials In Mozambique, Pakistan, And India: An Individual Participant-Level Meta-Analysis, Peter Von Dadelszen, Zulfiqar Ahmed Bhutta, Sumedha Sharma, Jeffrey Bone, Joel Singer, Hubert Wong, Mrutyunjaya B. Bellad, Shivaprasad S. Goudar, Tang Lee, Rahat Qureshi, Clip Trials Working Group

Woman and Child Health

Background: To overcome the three delays in triage, transport and treatment that underlie adverse pregnancy outcomes, we aimed to reduce all-cause adverse outcomes with community-level interventions targeting women with pregnancy hypertension in three low-income countries.
Methods: In this individual participant-level meta-analysis, we de-identified and pooled data from the Community-Level Interventions for Pre-eclampsia (CLIP) cluster randomised controlled trials in Mozambique, Pakistan, and India, which were run in 2014-17. Consenting pregnant women, aged 12-49 years, were recruited in their homes. Clusters, defined by local administrative units, were randomly assigned (1:1) to intervention or control groups. The control groups continued local standard of …

Ethically Addressing Vaccine Hesitancy In Pediatric Populations, Tommie L. James Iii

Theses and Graduate Projects

Vaccines are highly regarded in the scientific and medical community for their efficacy in the prevention of disease; yet the prevalence of misinformation and mistrust surrounding vaccines is growing in communities around the world. Specifically observed in the United States (US) as an increase in vaccination exemption rates, this trend of vaccine hesitancy is leading to an increasing number of parents and caregivers to either refuse or delay vaccinations from the recommended schedule. As a result, an increasing number of outbreaks of diseases such as measles and pertussis, thought to be on the verge of elimination, have spiked. While health …

Discrete Left Ventricle Outflow Tract Obstruction In Children: Incidence And Predictors Of Recurrence. A Multi-Center Study, Gadah M. Alqurashi, Rema S. Almohanna, Kamal Mk Ayoub, Arwa A. Alkhuraiji, Najla A. Almasoud, Amjad R. Alsubaie, Alaa M. Althubaiti, Abdullah A. Al Sehly

Journal of the Saudi Heart Association

Objectives: The purpose of this study is to measure the incidence of recurrence of discrete subaortic stenosis (DSS) after primary resection in two major cardiac centers in Saudi Arabia and to identify risk factors associated with recurrence.

Methods: Data on 234 patients who were diagnosed with DSS and underwent surgical resection between 1999 and 2018 were retrospectively reviewed. Patient demographics as well as echocardiographic, surgical, and pathological data were compared between patients with recurrence and non-recurrence.

Results: The overall recurrence incidence after primary resection was 44.87% (N=105). Most patients were male (59%). The median age at the …

Interstage Home Monitoring For Infants With Single Ventricle Heart Disease: Education And Management: A Scientific Statement From The American Heart Association., Nancy A. Rudd, Nancy S. Ghanayem, Garick D. Hill, Linda M. Lambert, Kathleen A. Mussatto, Jo Ann Nieves, Sarah Robinson, Girish S. Shirali, Michelle M. Steltzer, Karen Uzark, Nancy A. Pike, American Heart Association Council On Cardiovascular And Stroke Nursing; Council On Lifelong Congenital Heart Disease And Heart Health In The Young; Council On Arteriosclerosis, Thrombosis And Vascular Biology; Council On Clinical Cardiology; And Council On Lifestyle And Cardiometabolic Health

Manuscripts, Articles, Book Chapters and Other Papers

This scientific statement summarizes the current state of knowledge related to interstage home monitoring for infants with shunt-dependent single ventricle heart disease. Historically, the interstage period has been defined as the time of discharge from the initial palliative procedure to the time of second stage palliation. High mortality rates during the interstage period led to the implementation of in-home surveillance strategies to detect physiologic changes that may precede hemodynamic decompensation in interstage infants with single ventricle heart disease. Adoption of interstage home monitoring practices has been associated with significantly improved morbidity and mortality. This statement will review in-hospital readiness for …

Reducing Emotional Distress For Childhood Hypoglycemia In Parents (Redchip): Protocol For A Randomized Clinical Trial To Test A Video-Based Telehealth Intervention., Susana R. Patton, Andrew Mcconville, Arwen M. Marker, Alexandra D. Monzon, Kimberly A A. Driscoll, Mark A. Clements

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Despite the introduction of new insulin analogs, insulin pumps, and continuous glucose monitoring (CGM), young children with type 1 diabetes mellitus (T1D) remain vulnerable to episodes of hypoglycemia because of their unpredictable eating and activity patterns and high degree of insulin sensitivity. Caregivers and young children living with T1D learn to fear hypoglycemia because it is uncomfortable, unpredictable, and dangerous. Up to 60% of caregivers of young children with T1D report moderate to severe levels of fear of hypoglycemia, and caregiver fear of hypoglycemia relates to lower quality of life for families and suboptimal child glycemic control. Yet, until …

Circus In Motion: A Multimodal Exergame Supporting Vestibular Therapy For Children With Autism, Oscar Peña, Franceli L. Cibrian, Monica Tentori

Engineering Faculty Articles and Research

Exergames are serious games that involve physical exertion and are thought of as a form of exercise by using novel input models. Exergames are promising in improving the vestibular differences of children with autism but often lack of adaptation mechanisms that adjust the difficulty level of the exergame. In this paper, we present the design and development of Circus in Motion, a multimodal exergame supporting children with autism with the practice of non-locomotor movements. We describe how the data from a 3D depth camera enables the tracking of non-locomotor movements allowing children to naturally interact with the exergame . A …

Development Of An Lc-Esi-Ms/Ms Method For Determination Of A Novel Pyrrolomycin (Mp-1) And Application To Pre-Clinical Adme Studies, Wafaa N. Aldhafiri

Theses & Dissertations

A rapid, selective, and sensitive liquid chromatography coupled with tandem mass spectrometry (LC-MS/MS) method was developed and validated for quantitation of a novel Pyrrolomycin (MP-1) in mouse plasma. MP-1 was extracted from plasma utilizing a structural analog (PL-3) as the internal standard (IS). Analyte separation was achieved using a Waters Acquity UPLC®BEH C18 column (1.7 µm, 100 x 2.1 mm) protected with Acquity UPLC C18 guard column. Mobile phase consisted of 0.1% acetic acid in water (10%) and methanol (90%) at a total flow rate of 0.25 mL/min. The mass spectrometer was operated at unit resolution in the multiple reaction …

Pediatric Sjs-Ten: Where Are We Now?, Michele Ramien, Jennifer Goldman

Manuscripts, Articles, Book Chapters and Other Papers

Stevens-Johnson syndrome and toxic epidermal necrolysis are rare severe blistering skin reactions triggered by medications or infections. Over the last 5 to 10 years, a number of important publications have advanced understanding of these diseases and their response to treatment. Importantly, a subset of patients with disease triggered by infection has been identified as having Mycoplasma pneumoniae-induced rash and mucositis, suggesting a reconsideration of the diagnostic paradigm. We present an update on pediatric Stevens-Johnson syndrome and toxic epidermal necrolysis in the broader context of cutaneous adverse drug reactions and focus on challenges and recent advances in diagnosis, management, and prevention.

Management Practices For West Syndrome In South Asia: A Survey Study And Meta-Analysis, Priyanka Madaan, Prem Chand, Kyaw Linn, Jithangi Wanigasinghe, Mimi Lhamu Mynak, Prakash Poudel, Raili Riikonen, Amit Kumar, Pooja Dhir, Sandeep Negi, Jitendra Kumar Sahu

Department of Paediatrics and Child Health

Objectives: Considering the dearth of literature on West syndrome (WS) from South Asian countries, this study aimed to evaluate the management practices in South Asia by an online survey and meta-analysis.
Methods: An online questionnaire was sent to 223 pediatric neurologists/pediatricians in India, Pakistan, Myanmar, Sri Lanka, Bhutan, Nepal, and Bangladesh. Their responses were evaluated and supplemented by a meta-analysis.
Results: Of 125 responses received (response rate: 56%), around 60% of responders observed male preponderance and an approximate lead-time-to-treatment (LTTT) of 4-12 weeks. The commonest etiology observed was a static structural insult (88.6% of responders). Most commonly used first-line drug …

Fatal Case Of Newborn Lassa Fever Virus Infection Mimicking Late Onset Neonatal Sepsis: A Case Report From Northern Nigeria, Taofik Oluwaseun Ogunkunle, Surajudeen Oyeleke Bello, Chinwe Immaculata Anderson, Rashida Musa, Rasaq Olaosebikan, Abdulazeez Imam

Department of Pharmacology and Experimental Therapeutics Faculty Papers

BACKGROUND: Lassa fever is a zoonotic viral infection endemic to the West Africa countries. It is highly fatal during pregnancy and as such reports of neonatal onset Lassa fever infections are rare in scientific literature. We report a fatal case of Lassa fever in a 26-day-old neonate mimicking the diagnosis of late-onset neonatal sepsis.

CASE PRESENTATION: The patient is a 26-day-old neonate who was admitted with a day history of fever, poor feeding, pre-auricular lymphadenopathy and sudden parental death. He was initially evaluated for late onset neonatal sepsis. He later developed abnormal bleeding and multiple convulsions while on admission, prompting …

Stress And Negative Affect As Mediators In The Association Between Parental Social Support And Lung Function In Adolescents With Asthma, Amber Osorno, Eric Sternlicht, Pornchai Tirakitsoontorn, Azucena Talamantes, Anchalee Yuengsrigul, Zeev N. Kain, Brooke Jenkins

SURF Posters and Papers

Asthma is the leading chronic condition amongst children in the United States as 7.5% of children are diagnosed with asthma. Studies have shown that positive social support is associated with positive asthma management. A strong social support system predicts good management of asthma symptoms; however, current literature has not yet examined how social support impacts lung function as opposed to merely symptom management. Stress and negative affect have been revealed to be associated with worse asthma control, as well as exacerbation of symptoms. Stressful situations, such as the death of a family member, unemployment, and familial tensions lead to worsening …

The Spectrum Of Primary Immunodeficiencies At A Tertiary Care Hospital In Pakistan, Sonia Qureshi, Fatima Mir, Samina Junejo, Khalid Saleem, Samreen Zaidi, Abdullah B. Naveed, Khalil Ahmad, Farah Naz Qamar

Department of Paediatrics and Child Health

Background: Primary Immunodeficiency Disorders (PIDs) are well-known disorders in the West. but the recognition and diagnosis of these disorders is challenging in developing countries. We present the spectrum of PIDs seen at a tertiary care center in Pakistan, identified using clinical case definitions and molecular methods.
Methods: A retrospective chart review of children suspected to have PID was conducted at the Aga Khan University Hospital (AKUH) Karachi, Pakistan from 2010 to 2016. Data on demographics, clinical features, family history of consanguinity, sibling death, details of laboratory workup done for PID and molecular tests targeted panel next generation sequencing (NGS) or …

Patient Experience In A Pediatric Emergency Department During Covid-19, Beth L. Emerson Md, Erika Setzer

Patient Experience Journal

The COVID-19 pandemic has changed many dynamics in healthcare in the United States. This study explores an increase in patient experience (PE) scores in a pediatric emergency department. Visits were analyzed before and after March 8, 2020, corresponding with the first local case of COVID-19. Changes in the patient population and characteristics of survey responders were analyzed. Overall, the number of daily visits decreased (113 vs 36/day) and survey response rate decreased (3.7 vs 2.8%, p = 0.03), but PE scores increased (87.21 to 93.73, p = 0.002). Comparatively, an increase in patients with higher acuity levels by Emergency Severity …

Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From The International Kawasaki Disease Registry., Brian W Mccrindle, Cedric Manlhiot, Jane W Newburger, Ashraf S Harahsheh, Therese M Giglia, Frederic Dallaire, Kevin Friedman, Tisiana Low, Kyle Runeckles, Mathew Mathew, Andrew S Mackie, Nadine F Choueiter, Pei-Ni Jone, Shelby Kutty, Anji T Yetman, Geetha Raghuveer, Elfriede Pahl, Kambiz Norozi, Kimberly E Mchugh, Jennifer S Li, Sarah D De Ferranti, Nagib Dahdah

Paediatrics Publications

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD. Methods and Results A 34-institution international registry of 1651 patients with KD who had CAAs (maximum CAA

Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From The International Kawasaki Disease Registry., Brian W. Mccrindle, Cedric Manlhiot, Jane W Newburger, Ashraf S. Harahsheh, Therese M. Giglia, Frederic Dallaire, Kevin Friedman, Tisiana Low, Kyle Runeckles, Mathew Mathew, Andrew S. Mackie, Nadine F. Choueiter, Pei-Ni Jone, Shelby Kutty, Anji T. Yetman, Geetha Raghuveer, Elfriede Pahl, Kambiz Norozi, Kimberly E Mchugh, Jennifer S. Li, Sarah D De Ferranti, Nagib Dahdah, International Kawasaki Disease Registry

Manuscripts, Articles, Book Chapters and Other Papers

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD. Methods and Results A 34-institution international registry of 1651 patients with KD who had CAAs (maximum CAA Z score ≥2.5) was used. Time-to-event analyses were performed using the Kaplan-Meier method and Cox proportional hazard models for risk factor analysis. In patients with CAA Z scores ≥10, …

Ewing Sarcoma Family Of Tumors-Derived Small Extracellular Vesicle Proteomics Identify Potential Clinical Biomarkers., Glenson Samuel, Jennifer Crow, Jon B. Klein, Michael L. Merchant, Emily Nissen, Devin C. Koestler, Kris Laurence, Xiaobo Liang, Kathleen Neville, Vincent S. Staggs, Atif Ahmed, Safinur Atay, Andrew K. Godwin

Manuscripts, Articles, Book Chapters and Other Papers

Purpose: Ewing Sarcoma Family of Tumors (ESFT), the second most common pediatric osseous malignancy, are characterized by the pathognomonic chromosomal EWS-ETS translocation. Outside of tumor biopsy, no clinically relevant ESFT biomarkers exist. Additionally, tumor burden assessment at diagnosis, monitoring of disease responsiveness to therapy, and detection of disease recurrence are limited to radiographic imaging. To identify new, clinically relevant biomarkers we evaluated the proteome of a subset of ESFT-derived small extracellular vesicles (sEVs).

Materials and methods: We performed the first high quality proteomic study of ESFT-derived sEVs from 5 ESFT cell lines representing the most common EWS-ETS fusion types and …

Fosl1 Is A Novel Mediator Of Endotoxin/Lipopolysaccharide-Induced Pulmonary Angiogenic Signaling., Christopher R. Nitkin, Sheng Xia, Heather Menden, Wei Yu, Min Xiong, Daniel P. Heruth, Shui Qing Ye, Venkatesh Sampath

Manuscripts, Articles, Book Chapters and Other Papers

Systemic sepsis is a known risk factor for bronchopulmonary dysplasia (BPD) in premature infants, a disease characterized by dysregulated angiogenesis and impaired vascular and alveolar development. We have previoulsy reported that systemic endotoxin dysregulates pulmonary angiogenesis resulting in alveolar simplification mimicking BPD in neonatal mice, but the underlying mechanisms remain unclear. We undertook an unbiased discovery approach to identify novel signaling pathways programming sepsis-induced deviant lung angiogenesis. Pulmonary endothelial cells (EC) were isolated for RNA-Seq from newborn C57BL/6 mice treated with intraperitoneal lipopolysaccharide (LPS) to mimic systemic sepsis. LPS significantly differentially-regulated 269 genes after 6 h, and 1,934 genes after …

Hearing Loss In Mucopolysaccharidoses: Current Knowledge And Future Directions., Jeremy Wolfberg, Keerthana Chintalapati, Shunji Tomatsu, Kyoko Nagao

Department of Pediatrics Faculty Papers

Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders caused by a deficiency of one of the enzymes involved in the degradation of glycosaminoglycans. Hearing loss is a common clinical presentation in MPS. This paper reviews the literature on hearing loss for each of the seven recognized subtypes of MPS. Hearing loss was found to be common in MPS I, II, III, IVA, VI, and VII, and absent from MPS IVB and MPS IX. MPS VI presents primarily with conductive hearing loss, while the other subtypes (MPS I, MPS II, MPS III, MPS IVA, and MPS VII) can present with …