Medical Specialties Commons^™

Risk Factors Leading To Pulmonary Exacerbation In Patients With Cystic Fibrosis: A Systematic Review, Danish Abdul Aziz, Syeda Khadija Fatima, Hasan Nawaz Tahir

Department of Paediatrics and Child Health

Objective: To ascertain major risk factors associated with pulmonary exacerbation and pulmonary function decline in cystic fibrosis.
Methods: The systematic review was conducted at Aga Khan University, Karachi, in September 2018, and comprised electronic search of PubMed, Ovid, Science Direct and Cumulative Index of Nursing and Allied Health Literature databases of studies conducted from January 1990 to September 2018 which were categorised into 3 sets; 1990-98, 1999-2007 and 2008-18. Studies included for review focussed on articles with pulmonary exacerbation as the health outcome indicator, and had diagnosis of cystic fibrosis as the inclusion criteria, while risk factors were the exposure …

Management And Outcomes For Long-Segment Hirschsprung Disease: A Systematic Review From The Apsa Outcomes And Evidence Based Practice Committee, Akemi L Kawaguchi, Yigit S Guner, Stig Sømme, Alexandria C Quesenberry, L Grier Arthur, Juan E Sola, Cynthia D Downard, Rebecca M Rentea, Patricia A Valusek, Caitlin A Smith, Mark B Slidell, Robert L Ricca, Roshni Dasgupta, Elizabeth Renaud, Doug Miniati, Jarod Mcateer, Alana L Beres, Julia Grabowski, Shawn D St Peter, Ankush Gosain, American Pediatric Surgical Association Outcomes And Evidence-Based Practice (Oebp) Committee

Faculty, Staff and Student Publications

OBJECTIVE: Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD.

METHODS: Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed.

RESULTS: 66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method …

Primary Ciliary Dyskinesia: Ancestral Haplotypes Analysis Of The Rsph4a Founder Mutation In Puerto Rico, Wilfredo De Jesús-Rojas, Dalilah Reyes De Jesús, Angélica M Nieves, Ricardo A Mosquera, Juan C Martinez-Cruzado

Faculty, Staff and Student Publications

Genetic mutations in >50 genes, including RSPH4A, can lead to primary ciliary dyskinesia (PCD). RSPH4A mutations affect radial spokes, which alter the configuration of the ciliary ultrastructure and lead to chronic oto-sinopulmonary disease. The RSPH4A [c.921+3_6delAAGT] founder mutation was described as one cause of PCD without laterality defects in Puerto Rico. The average Puerto Rican genetic composition includes 64% European, 21% African ancestral, and 15% Native-American or Taino, a native tribe in the Caribbean at the start of the European colonization, genes. Due to the relatively elevated Taino ancestry on the island, it might have contributed to the endemicity of …

Are Body Surface Area Based Estimates Of Liver Volume Applicable To Children With Overweight Or Obesity? An In Vivo Validation Study., Chelsea Hosey-Cojocari, Sherwin S. Chan, Chance S. Friesen, Amie L. Robinson, Veronica Williams, Erica Swanson, Daniel O'Toole, Jansynn Radford, Neil J. Mardis, Trevor N. Johnson, J Steven Leeder, Valentina Shakhnovich

Manuscripts, Articles, Book Chapters and Other Papers

The liver is the primary organ responsible for clearing most drugs from the body and thus determines systemic drug concentrations over time. Drug clearance by the liver appears to be directly related to organ size. In children, organ size changes as children age and grow. Liver volume has been correlated with body surface area (BSA) in healthy children and adults and has been estimated by functions of BSA. However, these relationships were derived from "typical" populations and it is unknown whether they extend to estimations of liver volumes for population "outliers," such as children with overweight or obesity, who today …

Association Of Maternal Prenatal Selenium Concentration And Preterm Birth: A Multicountry Meta-Analysis, Nagendra Monangi, Huan Xu, Waqasuddin Khan, Furqan Kabir, Muhammad Imran Nisar, Aneeta Hotwani, Usma Mehmood, Ambreen Nizar, Javairia Khalid, Fyezah Jehan

Department of Paediatrics and Child Health

Background: Selenium (Se), an essential trace mineral, has been implicated in preterm birth (PTB). We aimed to determine the association of maternal Se concentrations during pregnancy with PTB risk and gestational duration in a large number of samples collected from diverse populations.
Methods: Gestational duration data and maternal plasma or serum samples of 9946 singleton live births were obtained from 17 geographically diverse study cohorts. Maternal Se concentrations were determined by inductively coupled plasma mass spectrometry analysis. The associations between maternal Se with PTB and gestational duration were analysed using logistic and linear regressions. The results were then combined using …

Cheyne-Stokes Respiration In A 17-Year-Old Boy Awaiting Heart Transplantation, Nooralam A Rai, Aliva De, Carin Lamm

The Texas Heart Institute Journal

Cheyne-Stokes respiration is a pattern of alternating central apnea and hyperpnea. It is well described in adults with congestive heart failure, but not in children.

We report the case of a 17-year-old boy whose systolic heart failure was complicated by Cheyne-Stokes respiration. He was given supportive therapy until heart transplant, after which his Cheyne-Stokes respiration clinically resolved. Clinicians should be aware of this uncommon condition in pediatric and adolescent patients who have advanced heart failure and irregular breathing.

Renal Involvement And Its Treatment In Pediatric Patients With Proteinase-3 Anti-Neutrophil Cytoplasmic Antibody Positive Granulomatosis With Polyangiitis: A Case Series, Xiaoyan Wu, Elizabeth Mancuso, Isabel Armendi, David Krasinski, Lin Liu, Shauna Tarsi, Wayne R Waz, Rabheh Abdul-Aziz, Ewa Elenberg

Faculty, Staff and Students Publications

We describe three pediatric patients between the ages of 10 and 14 years old who were diagnosed with granulomatosis with polyangiitis (GPA) between 2014 and 2019. Each case involves variations in presentation, symptomatology, diagnostics, and induction and maintenance therapy regimens. Patient 1 presented with significant renal involvement, hypertensive emergency, and focal alveolar hemorrhage, a rare presentation of GPA that causes up to 60% mortality.Patient 2 presented with minimal renal involvement and a diffuse petechial rash, which is the most common cutaneous presentation of GPA. Finally, patient 3 presented with significant renal involvement and later on with symptoms of idiopathic intracranial …

Mortality In Children Treated With Maintenance Peritoneal Dialysis: Findings From The International Pediatric Peritoneal Dialysis Network Registry., Sophie Ploos Van Amstel, Marlies Noordzij, Dagmara Borzych-Duzalka, Nicholas C. Chesnaye, Hong Xu, Lesley Rees, Il-Soo Ha, Zenaida L. Antonio, Nakysa Hooman, William Wong, Karel Vondrak, Yok Chin Yap, Hiren Patel, Maria Szczepanska, Sara Testa, Monica Galanti, Jameela A. Kari, Charlotte Samaille, Sevcan A. Bakkaloglu, Wai-Ming Lai, Luisa Fernanda Rojas, Mabel Sandoval Diaz, Biswanath Basu, Alicia Neu, Bradley A. Warady, Kitty J. Jager, Franz Schaefer

Manuscripts, Articles, Book Chapters and Other Papers

RATIONALE & OBJECTIVE: Research on pediatric kidney replacement therapy (KRT) has primarily focused on Europe and North America. In this study, we describe the mortality risk of children treated with maintenance peritoneal dialysis (MPD) in different parts of the world and characterize the associated demographic and macroeconomic factors.

STUDY DESIGN: Prospective cohort study.

SETTING & PARTICIPANTS: Patients younger than 19 years at inclusion into the International Pediatric Peritoneal Dialysis Network registry, who initiated MPD between 1996 and 2017.

EXPOSURE: Region as primary exposure (Asia, Western Europe, Eastern Europe, Latin America, North America, and Oceania). Other demographic, clinical, and macroeconomic (4 …

Classical Findings Of Infantile Hepatic Hemangiomas, Senayit Demie, Michael Bossak

HCA Healthcare Journal of Medicine

Introduction

Hemangiomas are benign vascular tumors that are common during infancy. They are most commonly noted as superficial bright red lesions on the skin but can also be found deeper as subcutaneous lesions. Patients with multifocal cutaneous hemangiomas are at risk of visceral involvement with the liver being most commonly affected. Most hemangiomas can be monitored clinically as they are self-limiting. Despite this, hepatic hemangiomas can have serious complications including large arteriovenous shunts leading to cardiac compromise as well as severe hepatomegaly which can cause abdominal compartment syndrome, impaired ventilation and renal vein compression.

Clinical Findings

A six-month-old female, born …

The Role Of The Pharmacist In The Treatment Of Infantile Botulism, Rachel E. Huntsman, Abigail Schauble

HCA Healthcare Journal of Medicine

Infantile botulism is a potentially devastating disease caused by ingestion of Clostridium botulinum spores through food products or dust particles. The toxin produced by the spores can lead to descending paralysis requiring hospitalization for supportive care which sometimes includes mechanical ventilation. Human Botulism Immune Globulin-Intravenous (BIG-IV or BabyBIG) from the Infant Botulism Treatment and Prevention Program (IBTPP) has been shown to greatly improve outcomes. A previously healthy 5-month-old infant was admitted to her regional hospital for poor feeding and lethargy. When the weakness progressed and she had trouble with protecting her airway, she was intubated and transferred to our institution. …

Nucleated Red Blood Cells As A Biomarker For Mortality In Neonates Following Cardiac Surgery, Kurt D. Piggott, Syeda Maqsood, Cynthia L. Warner, Timothy Pettitt, Shengping Yang, Jason Turner, Amira Soliman, Casey Norlin, Latasha Lewis, Ajay Bhatia

School of Medicine Faculty Publications

Introduction: Nucleated red blood cells (NRBCs) are immature red cells that under normal conditions are not present in the peripheral circulation. Several studies have suggested an association between elevated NRBC and poor outcome in critically ill adults and neonates. We sought to determine if elevations in NRBC value following cardiac surgery and following clinical events during the hospital stay can be used as a biomarker to monitor for mortality risk in neonates post-cardiac surgery. Materials and methods: We constructed a retrospective study of 264 neonates who underwent cardiac surgery at Children's Hospital, New Orleans between 2011 and 2020. Variables included …

Higher Entropy Observed In Sars-Cov-2 Genomes From The First Covid-19 Wave In Pakistan, Najia Karim Ghanchi, Asghar Nasir, Kiran I. Masood, Syed Hani Abidi, Syed Faisal Mahmood, Akber Kanji, Safina Abdul Razzak, Waqasuddin Khan, Saba Shahid, Maliha Yameen, Ali Raza, Javaria Ashraf, Zeeshan Ansar Ahmed, Mohammad Buksh Dharejo, Nazneen Islam, Zahra Hasan, Rumina Hasan

Department of Pathology and Laboratory Medicine

Background: We investigated the genome diversity of SARS-CoV-2 associated with the early COVID-19 period to investigate evolution of the virus in Pakistan.
Materials and methods: We studied ninety SARS-CoV-2 strains isolated between March and October 2020. Whole genome sequences from our laboratory and available genomes were used to investigate phylogeny, genetic variantion and mutation rates of SARS-CoV-2 strains in Pakistan. Site specific entropy analysis compared mutation rates between strains isolated before and after June 2020.
Results: In March, strains belonging to L, S, V and GH clades were observed but by October, only L and GH strains were present. The …

Atypical Idiopathic Intracranial Hypertension Presenting As Cyclic Vomiting Syndrome: A Case Report, Nafee T Talukder, Amanda H Clorfeine, Moira K Black, Shade B Moody

Faculty, Staff and Student Publications

BACKGROUND: Idiopathic intracranial hypertension is a disorder of increased intracranial pressure in the absence of cerebrospinal outflow obstruction, mass lesion, or other underlying cause. It is a rare phenomenon in prepubertal children and is most typically found in women of childbearing age. The classic presentation consists of headaches, nausea, vomiting, and visual changes; however, children present more atypically. We report a case of idiopathic intracranial hypertension in an otherwise healthy, 4-year-old child with atypical symptoms resembling those of cyclic vomiting syndrome.

CASE PRESENTATION: A 4-year-old Caucasian, otherwise healthy, male child presented to our emergency department with episodic intermittent early-morning vomiting …

Poverty And Paternal Education Associated With Infant Safe Sleep Intentions In A Peri-Urban Community In Ecuador, Hartley Feld, Janeth Ceballos Osorio, Marisol Bahamonde, Thomas Young, Pablo Boada, Mary Kay Rayens

Nursing Faculty Publications

Ecuador’s annual mortality rate from SIDS is 0.4 per 100 000 people, 4 times higher than neighboring countries Peru, Bolivia, and Brazil. Modifying the infant sleep environment toward safe practice has been demonstrated to be the most effective risk reduction strategy in reducing mortality from SIDS and little is known about sleep practices in Ecuador. The purpose of this study is to describe baseline infant sleep intentions of pregnant women in a peri-urban, low resource community in Ecuador. We also aim to identify demographic and psychosocial factors associated with suboptimal sleep practices in this context to develop long-term strategies to …

A Rare Entity: Case Report Of Recurrent Hypertrophic Pyloric Stenosis, Astrid A. Gleaton, Kartikey Pandya

Journal of Maine Medical Center

Introduction: Hypertrophic pyloric stenosis (HPS) is well known in pediatric surgery and has good outcomes after pyloromyotomy.1 Emesis in the immediate postoperative period occurs in 40% of patients with HPS and is attributed to gastroparesis from prolonged gastric distention.2 Emesis presenting weeks to months after pyloromyotomy prompts an evaluation that often yields pathology unrelated to HPS. Because recurrent HPS is rare, making this diagnosis highlighted the importance of practicing broad differential diagnoses, ruling out most common causes, and including obscure etiologies.

Clinical Findings: A 3-week-old male born after full-term gestation with a history of HPS underwent an uncomplicated laparoscopic pyloromyotomy. …

Implementing A Clinical Practice Guideline For Pediatric Appendicitis Safely Reduced Health Care Use And Improved Antimicrobial Stewardship, Jack Vernamonti, Robin Cotter, Jennifer Jubulis, Kartikey Pandya

Journal of Maine Medical Center

Introduction: Appendicitis is the most common emergency surgical disease in children. Those with perforated appendicitis have a more complicated and varied course. Through a clinical practice guideline (CPG), we sought to reduce computed tomography scans, laboratory draws, and exposure to broad-spectrum antibiotics without adversely affecting length of stay, hospital readmission, or repeat antibiotic administration.

Methods: Electronic records were retrospectively reviewed before and after CPG implementation, and data was collected in REDCap.

Results were reported as mean or percent incidence, and statistical analysis was done using a Student’s t-test, Mann-Whitney U test, or Pearson’s χ2 with P < .05 considered significant. Results: One hundred patients with a perforated appendix (50 before and 50 after CPG implementation) were included in our analysis. Length of stay (4.98 vs 4.46 days; P = .25), hospital readmission rate (10% vs 14%; P = .54), and additional antibiotic administration (2% vs 4%; P = .56) did not change. We observed no difference in the Pediatric Appendicitis Score (9 vs 9; P = .48) and a trending increase in evaluation at an outside hospital (56% vs 74%; P = .06). Rates of computed tomography scans did not differ overall (50% vs 40%; P = .31), but showed a decreasing trend at our institution (30% vs 12%; P = .06). We also found fewer post-operative laboratory studies (90% vs 38%; P < .01) and patients who received broad-spectrum intravenous antibiotics (92% vs 18%; P < .01).

Discussion: Through implementing the …

Restrictive Versus Liberal Transfusion Thresholds In Very Low Birth Weight Infants: A Systematic Review With Meta-Analysis, Peng Wang, Xing Wang, Haidong Deng, Linjie Li, Weelic Chong, Yang Hai, Yu Zhang

Department of Medicine Faculty Papers

BACKGROUND: To assess the efficacy and safety of restrictive versus liberal red blood cell transfusion thresholds in very low birth weight infants.

METHODS: We searched MEDLINE, EMBASE, and Cochrane database without any language restrictions. The last search was conducted in August 15, 2020. All randomized controlled trials comparing the use of restrictive versus liberal red blood cell transfusion thresholds in very low birth weight (VLBW) infants were selected. Pooled risk ratio (RR) for dichotomous variable with 95% confidence intervals were assessed by a random-effects model. The primary outcome was all-cause mortality.

RESULTS: Overall, this meta-analysis included 6 randomized controlled trials …

Deep Learning Applications In Neuro-Oncology, Adnan Khan, Hamza Ibad, Kaleem Ahmed, Zahra Hoodbhoy, Muhammad Shahzad Shamim

Medical College Documents

Deep learning (DL) is a relatively newer subdomain of machine learning (ML) with incredible potential for certain applications in the medical field. Given recent advances in its use in neuro-oncology, its role in diagnosing, prognosticating, and managing the care of cancer patients has been the subject of many research studies. The gamut of studies has shown that the landscape of algorithmic methods is constantly improving with each iteration from its inception. With the increase in the availability of high-quality data, more training sets will allow for higher fidelity models. However, logistical and ethical concerns over a prospective trial comparing prognostic …

Glycosaminoglycans As Biomarkers For Mucopolysaccharidoses And Other Disorders, Paige Amendum, Shaukat Khan, Seiji Yamaguchi, Hironori Kobayashi, Yasuhiko Ago, Yasuyuki Suzuki, Betul Celik, Estera Rintz, Jobayer Hossain, Wendi Xiao, Shunji Tomatsu

Department of Pediatrics Faculty Papers

Glycosaminoglycans (GAGs) are present in proteoglycans, which play critical physiological roles in various tissues. They are known to be elevated in mucopolysaccharidoses (MPS), a group of rare inherited metabolic diseases in which the lysosomal enzyme required to break down one or more GAG is deficient. In a previous study, we found elevation of GAGs in a subset of patients without MPS. In the current study, we aim to investigate serum GAG levels in patients with conditions beyond MPS. In our investigated samples, the largest group of patients had a clinical diagnosis of viral or non-viral encephalopathy. Clinical diagnoses and conditions …

Cognitive And Behavioral Domains That Reliably Differentiate Normal Aging And Dementia In Down Syndrome, Jordan P. Harp, Lisa M. Koehl, Kathryn L. Van Pelt, Christy L. Hom, Eric Doran, Elizabeth Head, Ira T. Lott, Frederick A. Schmitt

Neurology Faculty Publications

Primary care integration of Down syndrome (DS)-specific dementia screening is strongly advised. The current study employed principal components analysis (PCA) and classification and regression tree (CART) analyses to identify an abbreviated battery for dementia classification. Scale- and subscale-level scores from 141 participants (no dementia n = 68; probable Alzheimer’s disease n = 73), for the Severe Impairment Battery (SIB), Dementia Scale for People with Learning Disabilities (DLD), and Vineland Adaptive Behavior Scales—Second Edition (Vineland-II) were analyzed. Two principle components (PC1, PC2) were identified with the odds of a probable dementia diagnosis increasing 2.54 times per PC1 unit increase and by …

Cohort Profile: The Alliance For Maternal And Newborn Health Improvement (Amanhi) Biobanking Study, Fahad Aftab, Salahuddin Ahmed, Aneeta Hotwani, Muhammad Ilyas, Mohammad Javaid, Fyezah Jehan, Farah Khalid, Usma Mehmood, Muhammad Imran Nisar, Muhammad Sajid

Department of Paediatrics and Child Health

No abstract provided.

The Effect Of Severe Intraventricular Hemorrhage On The Biorhythms Of Feeding In Premature Infants, Ira H. Gewolb, Babatunde T. Sobowale, Frank L. Vice, Abhijit R. Patwardhan, Nino Solomonia, Eric W. Reynolds

Biomedical Engineering Faculty Publications

Background: Suck-swallow rhythmicity and the integration of breathing into infant feeding are developmentally regulated. Neurological injury and breathing abnormalities can both impact feeding in preterm infants.

Objective: To determine the effects of neurologic injury independent of effects of disordered breathing on feeding biorhythms in premature infants.

Methods: Low-risk preterm infants (LRP), infants with Grade 3–4 Intraventricular Hemorrhage (IVH), those with bronchopulmonary dysplasia (BPD), and those with both BPD and IVH (BPD+IVH) were identified. Forty-seven infants, 32–42 weeks Postmenstrual Age (PMA) were evaluated on one or more occasions (131 studies). Of these, 39 infants (81 studies) were performed at >35 weeks …

Comparing Growth Velocity Of Hiv Exposed And Non-Exposed Infants: An Observational Study Of Infants Enrolled In A Randomized Control Trial In Zambia, Obvious Nchimunya Chilyabanyama, Roma Chilengi, Natasha Makabilo Laban, Masuzyo Chirwa, Michelo Simunyandi, Luiza Miyanda Hatyoka, Innocent Ngaruye, Najeeha Talat Iqbal, Samuel Bosomprah

Department of Paediatrics and Child Health

Background: Impaired growth among infants remains one of the leading nutrition problems globally. In this study, we aimed to compare the growth trajectory rate and evaluate growth trajectory characteristics among children, who are HIV exposed uninfected (HEU) and HIV unexposed uninfected (HUU), under two years in Zambia.
Method: Our study used data from the ROVAS II study (PACTR201804003096919), an open-label randomized control trial of two verses three doses of live, attenuated, oral RotarixTM administered 6 &10 weeks or at 6 &10 weeks plus an additional dose at 9 months of age, conducted at George clinic in Lusaka, Zambia. Anthropometric measurements …

Identifying The Content And Context Of Pain Within Paediatric Rheumatology Healthcare Professional Curricula In The Uk: A Summative Content Analysis., Rebecca Rachael Lee, Janet E. Mcdonagh, Mark Connelly, Sarah Peters, Lis Cordingley

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The curriculum for professionals working in paediatric rheumatology should include pain but it is unclear to what extent this currently occurs. The aim of this study was to identify pain-related curriculum content and the context in which pain is presented in educational and training documentation for healthcare professionals in this clinical speciality.

METHODS: Core curricula documents from UK based professional organisations were identified in partnership with healthcare professionals. Documents were analysed using a summative content analysis approach. Key pain terms were quantified and weighted frequencies were used to explore narrative pain themes. Latent content was interpreted qualitatively to explore …

Moving Beyond Size: Vorticity And Energy Loss Are Correlated With Right Ventricular Dysfunction And Exercise Intolerance In Repaired Tetralogy Of Fallot., Yue-Hin Loke, Francesco Capuano, Vincent Cleveland, Jason G. Mandell, Elias Balaras, Laura J. Olivieri

Pediatrics Faculty Publications

BACKGROUND: The global effect of chronic pulmonary regurgitation (PR) on right ventricular (RV) dilation and dysfunction in repaired Tetralogy of Fallot (rTOF) patients is well studied by cardiovascular magnetic resonance (CMR). However, the links between PR in the RV outflow tract (RVOT), RV dysfunction and exercise intolerance are not clarified by conventional measurements. Not all patients with RV dilation share the same intracardiac flow characteristics, now measurable by time resolved three-dimensional phase contrast imaging (4D flow). In our study, we quantified regional vorticity and energy loss in rTOF patients and correlated these parameters with RV dysfunction and exercise capacity.

METHODS: …

Rumination Syndrome In Children And Adolescents: A Mini Review., Marc Martinez, Sandeep Rathod, Hunter J. Friesen, John Rosen, Craig A. Friesen, Jennifer Verrill Schurman

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Rumination syndrome involves recurrent regurgitation of food and is believed to be underdiagnosed with patients experiencing long delays in diagnosis. It can be associated with significant social consequences, high rates of school absenteeism, and medical complications such as weight loss. The primary aims of the current review are to assess the literature regarding prevalence, pathophysiology, and treatment outcomes with a focus on neurotypical children and adolescents.

Results: Population studies in children/adolescents, 5 years of age or older, range from 0 to 5.1%. There are fewer studies in clinical settings, but the prevalence appears to be higher in patients with …

Ability Of Video Telemetry To Predict Unplanned Hospital Admissions For Single Ventricle Infants., Doaa Aly, Lori Erickson, Hayley S. Hancock, Johnathan Apperson, Monica Gaddis, Girish S. Shirali, Suma Goudar

Manuscripts, Articles, Book Chapters and Other Papers

Background: Our Cardiac High Acuity Monitoring Program (CHAMP) uses home video telemetry (HVT) as an adjunct to monitor infants with single ventricle during the interstage period. This study describes the development of an objective early warning score using HVT, for identification of infants with single ventricle at risk for clinical deterioration and unplanned hospital admissions (UHA).

Methods and Results: Six candidate scoring parameters were selected to develop a pragmatic score for routine evaluation of HVT during the interstage period. We evaluated the individual and combined ability of these parameters to predict UHA. All infants with single ventricle monitored at home …

Phylogenetic And Drug-Resistance Analysis Of Hiv-1 Sequences From An Extensive Paediatric Hiv-1 Outbreak In Larkana, Pakistan, Syed Hani Abidi, George Makau Nduva, Dilsha Siddiqui, Wardah Rafaqat, Syed Faisal Mahmood, Amna Rehana Siddiqui, Apsara Ali, Aneeta Hotwani, Rashida Abbas Ferrand, Fatima Mir

Department of Biological & Biomedical Sciences

Introduction: In April 2019, an HIV-1 outbreak among children occurred in Larkana, Pakistan, affecting more than a thousand children. It was assumed that the outbreak originated from a single source, namely a doctor at a private health facility. In this study, we performed subtype distribution, phylogenetic and drug-resistance analysis of HIV-1 sequences from 2019 outbreak in Larkana, Pakistan.
Methods: A total of 401 blood samples were collected between April-June 2019, from children infected with HIV-1 aged 0-15 years recruited into a case-control study to investigate the risk factors for HIV-1 transmission. Partial HIV-1 pol sequences were generated from 344 blood …

Concentrations Of Lead, Mercury, Arsenic, Cadmium, Manganese, And Aluminum In The Blood Of Pakistani Children With And Without Autism Spectrum Disorder And Their Associated Factors, Mohammad H Rahbar, Shahnaz H Ibrahim, Syed Iqbal Azam, Manouchehr Hessabi, Fatima Karim, Sori Kim, Jing Zhang, Nasreen Gulzar Ali, Katherine A Loveland

Faculty, Staff and Student Publications

BACKGROUND: Autism Spectrum Disorder (ASD) is a neurodevelopmental disorder with early onset in utero or childhood. Environmental exposure to six metals (Pb, Hg, As, Cd, Mn, Al) is believed to be associated with ASD directly or interactively with genes.

METHODS: We enrolled 30 ASD cases, age 2-12 years old, and 30 age- and sex-matched typically developing (TD) controls in Karachi, Pakistan. We assessed associations of ASD status with various factors using Conditional Logistic Regression models. We also used General Linear Models to assess possible interaction of blood Mn and Pb concentrations with the three GST genes in relation to ASD …

Proof Of Concept: The Use Of Renal Biomarkers In Critically Ill Pediatric Patients For Therapeutic Drug Monitoring, Christopher Lee Shaffer

Theses & Dissertations

Acute kidney injury (AKI) is a serious and common complication in critically ill pediatric patients. The incidence of pediatric AKI continues to increase, especially in patients who undergo surgical correction of congenital heart defects. Serum creatinine and urine output are the most commonly used tools to assess renal function, with international guidelines standardizing AKI-definitions based upon these parameters. However, changes in serum creatinine can occur 24 hours or later after a renal insult event, delaying the diagnosis and potential interventions to reverse injury. It is critical to identify endogenous renal biomarkers within the pediatric population that are both timely and …