Medicine and Health Sciences Commons^™

Directional Secretory Response Of Double Stranded Rna-Induced Thymic Stromal Lymphopoetin (Tslp) And Ccl11/Eotaxin-1 In Human Asthmatic Airways, Gustavo Nino, Shehlanoor Huseni, Geovanny F. Perez, Krishna Pancham, Humaira Mubeen, Aleeza Abeesi, Justin Wang, Stephen Eng, Anamaris M. Colberg-Poley, Mary C. Rose

Pediatrics Faculty Publications

Background

Thymic stromal lymphoproetin (TSLP) is a cytokine secreted by the airway epithelium in response to respiratory viruses and it is known to promote allergic Th2 responses in asthma. This study investigated whether virally-induced secretion of TSLP is directional in nature (apical vs. basolateral) and/or if there are TSLP-mediated effects occurring at both sides of the bronchial epithelial barrier in the asthmatic state.

Methods

Primary human bronchial epithelial cells (HBEC) from control (n = 3) and asthmatic (n = 3) donors were differentiated into polarized respiratory tract epithelium under air-liquid interface (ALI) conditions and treated apically with dsRNA (viral surrogate) …

Systems Biology Investigation Of Camp Modulation To Increase Smn Levels For The Treatment Of Spinal Muscular Atrophy., Sean G Mack, Daniel J Cook, Prasad Dhurjati, Matthew E R Butchbach

Department of Pediatrics Faculty Papers

Spinal muscular atrophy (SMA), a leading genetic cause of infant death worldwide, is an autosomal recessive disorder caused by the loss of SMN1 (survival motor neuron 1), which encodes the protein SMN. The loss of SMN1 causes a deficiency in SMN protein levels leading to motor neuron cell death in the anterior horn of the spinal cord. SMN2, however, can also produce some functional SMN to partially compensate for loss of SMN1 in SMA suggesting increasing transcription of SMN2 as a potential therapy to treat patients with SMA. A cAMP response element was identified on the SMN2 promoter, implicating cAMP …

Low Birth Weight Followed By Postnatal Over-Nutrition In The Guinea Pig Exposes A Predominant Player In The Development Of Vascular Dysfunction., Jennifer A Thompson, Ousseynou Sarr, Karolina Piorkowska, Robert Gros, Timothy Regnault

Paediatrics Publications

The association between intrauterine growth restriction (IUGR) and hypertension is well established, yet the interaction between IUGR and other pathogenic contributors remains ill-defined. This study examined the independent and interactive effects of fetal growth reduction resulting in low birth weight (LBW), and postnatal Western diet (WD) on vascular function. Growth reduction was induced in pregnant guinea pigs by uterine artery ablation. LBW and normal birth weight (NBW) offspring were randomly assigned to a control diet (CD) or a WD. In young adulthood, length-tension curves were generated in aortic rings and responses to methacholine (MCh) were evaluated in the carotid and …

Cyclooxygenase-2, Prostaglandin E2, And Prostanoid Receptor Ep2 In Fluid Flow Shear Stress-Mediated Injury In The Solitary Kidney., Tarak Srivastava, Uri S. Alon, Patricia A. Cudmore, Belal Tarakji, Alexander Kats, Robert E. Garola, R Scott Duncan, Ellen T. Mccarthy, Ram Sharma, Mark L. Johnson, Lynda F. Bonewald, Ashraf El-Meanawy, Virginia J. Savin, Mukut Sharma

Manuscripts, Articles, Book Chapters and Other Papers

Hyperfiltration subjects podocytes to increased tensile stress and fluid flow shear stress (FFSS). We showed a 1.5- to 2.0-fold increase in FFSS in uninephrectomized animals and altered podocyte actin cytoskeleton and increased synthesis of prostaglandin E2 (PGE2) following in vitro application of FFSS. We hypothesized that increased FFSS mediates cellular changes through specific receptors of PGE2. Presently, we studied the effect of FFSS on cultured podocytes and decapsulated isolated glomeruli in vitro, and on solitary kidney in uninephrectomized sv129 mice. In cultured podocytes, FFSS resulted in increased gene and protein expression of cyclooxygenase (COX)-2 but not COX-1, prostanoid receptor EP2 …

Developmental And Cultural Perspectives On Children’S Postoperative Pain Management At Home, Brooke N. Jenkins, Michelle A. Fortier

Psychology Faculty Articles and Research

Outpatient surgery is extremely common in children, and approximately 4 million children experience significant pain after surgery in the USA each year. Management of children's postoperative pain in the home setting is suboptimal and is impacted by characteristics of children and parents, as well as the larger family and cultural context. In particular, developmental status of the child, parental beliefs regarding pain expression and analgesic use in children, cultural values and language barriers can affect management of children's postoperative pain. Targeting the myriad barriers to children's pain management by capitalizing upon the use of tailored interventions may help bridge the …

Retrospective Study To Identify Predictors Of Withdrawal In Pediatric Intensive Care Patients On Long-Term Sedation, Lindsay Fakete Pharmd, Jenny Boucher Pharmd, Jd, Bcps, Scott Wheatley Md

Department of Pharmacy

No abstract provided.

Propranolol Therapy For Infantile Hemangioma Is Less Toxic But Longer In Duration Than Corticosteroid Therapy, Kathryn Sawa, Arjang Yazdani, Michael J. Rieder, Guido Filler

Paediatrics Publications

BACKGROUND: Infantile hemangioma is the most common benign, self-limiting tumour of childhood. Treatment is reserved for hemangiomas that obstruct vital structures or cause significant disfigurement. Traditionally, corticosteroids have been the medical treatment of choice. Since 2008, however, propranolol has been rapidly adopted as an effective pharmacological treatment for infantile hemangioma. Published data regarding the long-term side effects of propranolol are currently lacking. OBJECTIVE: To describe the long-term effects of propranolol and corticosteroids on anthropometric measurements (height, body mass index [BMI]) and blood pressure in children. METHODS: A prospective database analysis of all infantile hemangioma patient visits to the pediatric vascular …

The Health Initiative Program For Kids (Hip Kids): Effects Of A 1-Year Multidisciplinary Lifestyle Intervention On Adiposity And Quality Of Life In Obese Children And Adolescents -- A Longitudinal Pilot Intervention Study, Dirk E. Bock, Tracy Robinson, Jamie A Seabrook, Meghan Rombeek, Kambiz Norozi, Guido Filler, Ralf Rauch, Cheril L Clarson

Paediatrics Publications

BACKGROUND: Though recent data suggest that multidisciplinary outpatient interventions can have a positive effect on childhood obesity, it is still unclear which program components are most beneficial and how they affect quality of life (QoL). The aim of this study was to determine if a 1-year multidisciplinary, family-centered outpatient intervention based on social cognitive theory would be effective in (i) preventing further increases in BMI and BMI z-score, and (ii) improving QoL in obese children and adolescents.

METHODS: Obese children and adolescents 8-17 years of age and their families participated in this 1-year longitudinal pilot intervention study. The intervention consisted …

Integrative Therapy Use For Management Of Side Effects And Toxicities Experienced By Pediatric Oncology Patients, Shana Jacobs

Pediatrics Faculty Publications

Integrative Therapies (IT), otherwise known as Complementary and Alternative Medicine, are widely used among pediatric oncology patients, despite a paucity of available evidence. This review summarizes surveys that describe the prevalence of IT use by pediatric oncology patients, both during therapy and in survivorship, as well as the modalities being used. Additionally, the evidence that exists for specific treatments that appear to be efficacious in controlling specific symptoms is described. Finally, there are recommendations for practitioners on how to best counsel patients about IT use.

Newborn Care In Indonesia, Lao People’S Democratic Republic And The Philippines: A Comprehensive Needs Assessment, Els Duysburgh, Birgit Kerstens, Melissa Diaz, Vini Fardhdiani, Katherine Ann Reyes, Khamphong Phommachanh, Marleen Temmerman, Basil Rodriques, Nabila Zaka

Obstetrics and Gynaecology, East Africa

Background: Between 1990 and 2011, global neonatal mortality decline was slower than that of under-five mortality. As a result, the proportion of under-five deaths due to neonatal mortality increased. This increase is primarily a consequence of decreasing post-neonatal and child under-five mortality as a result of the typical focus of child survival programmes of the past two decades on diseases affecting children over four weeks of age. Newborns are lagging behind in improved child health outcomes. The aim of this study was to conduct a comprehensive, equity-focussed newborn care assessment and to explore options to improve newborn survival in Indonesia, …

Risk Factors Associated With Birth Asphyxia In Rural District Matiari, Pakistan: A Case Control Study, Farhana Tabassum, Arjumand Rizvi, Shabina Ariff, Sajid Bashir Soofi, Zulfiqar Ahmed Bhutta

Department of Paediatrics and Child Health

Background: During the past two decades there has been a sustained decline in child mortality; however, neonatal mortality has remained stagnant. Each year approximately 4 million babies are born asphyxiated resulting in 2 million neonatal deaths and intrapartum stillbirths. Almost all neonatal deaths occur in developing countries, where the majority is delivered at homes with negligible antenatal care and poor perinatal services.
Objectives: To identify socio-demographic and clinical risk factors associated with birth asphyxia in Matiari District of Sindh Province, Pakistan.
Method: A matched case control study was conducted in Matiari District with 246 cases and 492 controls. Newborn deaths …

Pupillometry: A Non-Invasive Technique For Pain Assessment In Paediatric Patients., Mark A. Connelly, Jacob T. Brown, Gregory L. Kearns, Rawni A. Anderson, Shawn D. St Peter, Kathleen A. Neville

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: Pupillometry has been used to assess pain intensity and response to analgesic medications in adults. The aim of this observational study was to explore proof of concept for the use of this technique in paediatric patients. Changes in pupil parameters before and after opioid exposure also were evaluated.

DESIGN AND SETTING: This was a single-centre, prospective study conducted at an academic paediatric medical centre.

PATIENTS: Children 9-17 years of age undergoing elective surgical correction of pectus excavatum were enrolled into a protocol approved by the human ethical committee (institutional review board).

INTERVENTIONS: Pupil size and reactivity were measured using …

Rare Presentation Of Pancreatitis Secondary To Intussusception Of Duodenal Duplication Cyst, A Pediatric Case Report, Valentina Shakhnovich, Jennifer Colombo, Amita A. Desai, Shawn D. St Peter

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Multi-Drug Resistant Gram Negative Infections And Use Of Intravenous Polymyxin B In Critically Ill Children Of Developing Country: Retrospective Cohort Study, Naveed-Ur-Rehman Siddiqui, Farah Naz Qamar, Humaira Jurair, Anwarul Haque

Department of Paediatrics and Child Health

Background: Patients in pediatric intensive care Units (PICU) are susceptible to infections with antibiotic resistant organisms which increase the morbidity, mortality and cost of care. To describe the clinical characteristics and mortality in patients with Multi-Drug Resistant (MDR) gram negative organisms. We also report safety of Polymyxin B use in these patients.
Methods: Files of patients admitted in PICU of Aga Khan University Hospital, from January 2010 to December 2011, one month to 15 years of age were reviewed. Demographic and clinical features of patients with MDR gram negative infections, antibiotic susceptibility pattern of isolates, discharge disposition and adverse effects …

Longer Gestation Among Children Born Full Term Influences Cognitive And Motor Development, Emma V. Espel, Laura M. Glynn, Curt A. Sandman, Elyssia Poggi Davis

Psychology Faculty Articles and Research

Children born preterm show persisting impairments in cognitive functioning, school achievement, and brain development. Most research has focused on implications of birth prior to 37 gestational weeks; however, the fetal central nervous system continues to make fundamental changes throughout gestation. Longer gestation is associated with reduced morbidity and mortality even among infants born during the period clinically defined as full term (37–41 gestational weeks). The implications of shortened gestation among term infants for neurodevelopment are poorly understood. The present study prospectively evaluates 232 mothers and their full term infants (50.4% male infants) at three time points across the first postnatal …

Here's The New Champ : Dr. Shirali Announces App For Infants With Heart Defects, Children's Mercy Hospital

Our Story Continues

News articles about the Cardiac High-Acuity Monitoring Program developed under Dr. Gigish Shirali's leadership which reduces mortality among children with single ventricle heart defects. This CHAMP app has since been used by other children's hospitals with continued success.

Corticosteroids And Pediatric Septic Shock Outcomes: A Risk Stratified Analysis, Sarah J. Atkinson, Natalie Z. Cvijanovich, Neal J. Thomas, Geoffrey L. Allen, Nick Anas, Robert J. Freishtat, +17 Additional Authors

Pediatrics Faculty Publications

Background

The potential benefits of corticosteroids for septic shock may depend on initial mortality risk.

Objective

We determined associations between corticosteroids and outcomes in children with septic shock who were stratified by initial mortality risk.

Methods

We conducted a retrospective analysis of an ongoing, multi-center pediatric septic shock clinical and biological database. Using a validated biomarker-based stratification tool (PERSEVERE), 496 subjects were stratified into three initial mortality risk strata (low, intermediate, and high). Subjects receiving corticosteroids during the initial 7 days of admission (n = 252) were compared to subjects who did not receive corticosteroids (n = 244). Logistic regression …

Heart Of The Matter, Children's Mercy Hospital

Our Story Continues

The Ward Family Heart Center at Children’s Mercy has received approval to begin performing pediatric heart transplants, making it the first healthcare facility in the Kansas City region to offer the life-saving procedure to pediatric patients.

Leading the Children’s Mercy heart transplant team are two nationally recognized experts:

James D. St. Louis, MD, is a board-certified thoracic and cardiac surgeon with more than 15 years of pediatric experience. Dr. St. Louis previously was the Director of Pediatric Cardiac Transplantation at the University of Minnesota Amplatz Children’s Hospital.

Aliessa P. Barnes, MD, is a board-certified pediatric cardiologist specializing in pediatric heart …

Subspecialty Surveillance Of Long-Term Course Of Small And Moderate Muscular Ventricular Septal Defect: Heterogenous Practices, Low Yield., Erik L. Frandsen, Aswathy V. House, Yunbin Xiao, David A. Danford, Shelby Kutty

Journal Articles: Pediatrics

BACKGROUND: No expert consensus guides practice for intensity of ongoing pediatric cardiology surveillance of hemodynamically insignificant small and moderate muscular ventricular septal defect (mVSD). Therefore, despite the well-established benign natural history of mVSD, there is potential for widely divergent follow up practices. The purpose of this investigation was to evaluate (1) variations in follow up of mVSD within an academic children's hospital based pediatric cardiology practice, and (2) the frequency of active medical or surgical management resulting from follow up of mVSD.

METHODS: We retrospectively reviewed records of 600 patients with isolated mVSD echocardiographically diagnosed between 2006 and 2012. Large …

Evidence-Informed Guidelines For Pediatric Pandemic Planning And Response, Ginny Sprang, James J. Clark, Miriam Silman, Phyllis W. Leigh, Candice M. Jackson, A. Scott Lajoie

Center on Trauma and Children Reports

From the executive summary:

Pandemic events are unpredictable and inevitable. When they occur, the impact is both all-encompassing and asymmetrical; each pandemic targets specific, vulnerable populations, but ultimately impacts individuals, families and communities throughout the world. Regardless of origin or circumstances, the next pandemic will certainly count infants, children, and adolescents among its most vulnerable targets. As evidenced by the 2009 H1N1 influenza pandemic, children may be at higher risk than populations more typically seen as susceptible to pandemic illness (the elderly, those with weakened immune systems, etc.). Children also can function as disease vectors, spreading the virus through their …

Atrx Is Required For Maintenance Of The Neuroprogenitor Cell Pool In The Embryonic Mouse Brain, Kieran Ritchie, L Ashley Watson, Benjamin Davidson, Yan Jiang, Nathalie G. Bérubé

Paediatrics Publications

Mutations in the alpha-thalassemia mental retardation X-linked (ATRX) gene cause a spectrum of abnormalities including intellectual disability, developmental delay, seizures, and microcephaly. The ATRX protein is highly enriched at heterochromatic repetitive sequences adjacent to the centromere, and ATRX depletion results in chromosome congression, segregation, and cohesion defects. Here, we show that Cre-mediated inactivation of Atrx in the embryonic mouse (Mus musculus) brain results in expansion of cerebral cortical layer VI, and a concurrent thinning of layers II-IV. We observed increased cell cycle exit during early-mid neurogenesis, and a depletion of apical progenitors by late neurogenesis in the Atrx-null neocortex, explaining …

The Translational Biology Of Remyelination: Past, Present, And Future, Robin J.M. Franklin, Vittorio Gallo

Pediatrics Faculty Publications

Amongst neurological diseases, multiple sclerosis (MS) presents an attractive target for regenerative medicine. This is because the primary pathology, the loss of myelin-forming oligodendrocytes, can be followed by a spontaneous and efficient regenerative process called remyelination. While cell transplantation approaches have been explored as a means of replacing lost oligodendrocytes, more recently therapeutic approaches that target the endogenous regenerative process have been favored. This is in large part due to our increasing understanding of (1) the cell types within the adult brain that are able to generate new oligodendrocytes, (2) the mechanisms and pathways by which this achieved, and (3) …

Vitamin A, Vitamin E, Iron And Zinc Status In A Cohort Of Hiv-Infected Mothers And Their Uninfected Infants, Jacqueline Pontes Monteiro, Maria Leticia Santos Cruz, Marisa Marcia Mussi-Pinhata, Roberta Garcia Salomao, Alceu Jordao Junior, Jennifer S. Read, Jose Henrique Da Silva Pilotto, Rachel Ann Cohen, Sonia Karolina Stoszek, George Kelly Siberry

Pediatrics Faculty Publications

Introduction

We hypothesized that nutritional deficiency would be common in a cohort of postpartum, human immunodeficiency virus (HIV)-infected women and their infants.

Methods

Weight and height, as well as blood concentrations of retinol, α-tocopherol, ferritin, hemoglobin, and zinc, were measured in mothers after delivery and in their infants at birth and at 6-12 weeks and six months of age. Retinol and α-tocopherol levels were quantified by high performance liquid chromatography, and zinc levels were measured by atomic absorption spectrophotometry. The maternal body mass index during pregnancy was adjusted for gestational age (adjBMI).

Results

Among the 97 women 19.6% were underweight. …

Coagulopathy In Newborns With Hypoxic Ischemic Encephalopathy (Hie) Treated With Therapeutic Hypothermia: A Retrospective Case-Control Study, Katie R. Forman, Yaser A. Diab, Edward C.C. Wong, Stephen Baumgart, Naomi L. Luban, An Nguyen Massaro

Pediatrics Faculty Publications

Background

Newborns with hypoxic ischemic encephalopathy (HIE) are at risk for coagulopathy due to systemic oxygen deprivation. Additionally, therapeutic hypothermia (TH) slows enzymatic activity of the coagulation cascade, leading to constitutive prolongation of routinely assessed coagulation studies. The level of laboratory abnormality that predicts bleeding is unclear, leading to varying transfusion therapy practices.

Methods

HIE infants treated with TH between 2008–2012 were included in this retrospective study. Initial, minimum (min) and maximum (max) values of International Normalized Ratio (INR), activated partial thromboplastin time (aPTT), fibrinogen (Fib) and platelet (PLT) count (measured twice daily during TH) were collected. Bleeding was defined …

Neonatal Magnesium Levels Correlate With Motor Outcomes In Premature Infants: A Long-Term Retrospective Cohort Study, Elizabeth Doll, Jacob Wilkes, Lawrence J. Cook, E. Kent Korgenski, Roger G. Faix, Bradley A. Yoder, Rajendu Srivastava, Catherine M.T. Sherwin, Michael G. Spigarelli, Erin A.S. Clark, Joshua L. Bonkowsky

Pediatrics Faculty Publications

OBJECTIVE:

Chronic neurological deficits are a significant complication of preterm birth. Magnesium supplementation has been suggested to have neuroprotective function in the developing brain. Our objective was to determine whether higher neonatal serum magnesium levels were associated with better long-term neurodevelopmental outcomes in very-low birth weight infants.

STUDY DESIGN:

A retrospective cohort of 75 preterm infants (<1500 g, gestational age <27 weeks) had follow-up for the outcomes of abnormal motor exam and for epilepsy. Average total serum magnesium level in the neonate during the period of prematurity was the main independent variable assessed, tested using a Wilcoxon rank-sum test.

RESULTS:

Higher average serum magnesium level was associated with a statistically significant decreased risk for abnormal motor exam (p = 0.037). A lower risk for epilepsy in the group with higher magnesium level did not reach statistical significance (p = 0.06).

CONCLUSION:

This …

Teaching Health Advocacy To Medical Students: A Comparison Study., Julia Belkowitz, Lee M Sanders, Chi Zhang, Gauri Agarwal, Daniel Lichtstein, Alex J Mechaber, Esther K Chung

Department of Pediatrics Faculty Papers

CONTEXT: Many encourage service learning and health advocacy training in medical student education, but related evaluation is limited.

OBJECTIVES: To assess (1) impact of a required community health advocacy training for medical students on student attitudes, knowledge, and skills; (2) student characteristics associated with higher advocacy knowledge and skills; and (3) perspectives of community-based organizations (CBOs).

DESIGN: Cross-sectional surveys.

SETTING: University of Miami Miller School of Medicine (UMMSM) Regional Medical Campus and main campus.

PARTICIPANTS: Medical students at both UMMSM campuses.

INTERVENTION: Required community health advocacy training for first- and second-year students including classroom experiences and hands-on project in partnership …

Technical Performance Score Is Associated With Outcomes After The Norwood Procedure., Meena Nathan, Lynn A. Sleeper, Richard G. Ohye, Peter C. Frommelt, Christopher A. Caldarone, James S. Tweddell, Minmin Lu, Gail D. Pearson, J William Gaynor, Christian Pizarro, Ismee A. Williams, Steven D. Colan, Carolyn Dunbar-Masterson, Peter J. Gruber, Kevin Hill, Jennifer Hirsch-Romano, Jeffrey P. Jacobs, Jonathan R. Kaltman, S Ram Kumar, David Morales, Scott M. Bradley, Kirk Kanter, Jane W. Newburger, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

Objectives: The technical performance score (TPS) has been reported in a single center study to predict the outcomes after congenital cardiac surgery. We sought to determine the association of the TPS with outcomes in patients undergoing the Norwood procedure in the Single Ventricle Reconstruction trial.

Methods: We calculated the TPS (class 1, optimal; class 2, adequate; class 3, inadequate) according to the predischarge echocardiograms analyzed in a core laboratory and unplanned reinterventions that occurred before discharge from the Norwood hospitalization. Multivariable regression examined the association of the TPS with interval to first extubation, Norwood length of stay, death or transplantation, …

Sofas And Infant Mortality., Lauren R. Rechtman, Jeffrey D. Colvin, Peter S. Blair, Rachel Y. Moon

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: Sleeping on sofas increases the risk of sudden infant death syndrome and other sleep-related deaths. We sought to describe factors associated with infant deaths on sofas.

METHODS: We analyzed data for infant deaths on sofas from 24 states in 2004 to 2012 in the National Center for the Review and Prevention of Child Deaths Case Reporting System database. Demographic and environmental data for deaths on sofas were compared with data for sleep-related infant deaths in other locations, using bivariate and multivariable, multinomial logistic regression analyses.

RESULTS: A total of 1024 deaths on sofas made up 12.9% of sleep-related infant …

Provider Cost Analysis Supports Results-Based Contracting Out Of Maternal And Newborn Health Services: An Evidence-Based Policy Perspective, Peter Hatcher, Shiraz Shaikh, Hassan Fazli, Shehla Zaidi, Atif Riaz

Community Health Sciences

Background

There is dearth of evidence on provider cost of contracted out services particularly for Maternal and Newborn Health (MNH). The evidence base is weak for policy makers to estimate resources required for scaling up contracting. This paper ascertains provider unit costs and expenditure distribution at contracted out government primary health centers to inform the development of optimal resource envelopes for contracting out MNH services.

Methods

This is a case study of provider costs of MNH services at two government Rural Health Centers (RHCs) contracted out to a non-governmental organization in Pakistan. It reports on four selected Basic Emergency Obstetrical …

Vascular Ehlers-Danlos Syndrome: A Rare Disorder Presenting With Focal Convulsions, Shakeel Ahmed, Syed Rehan Ali, Naila Nadeem, Muhammad Hamid

Department of Paediatrics and Child Health

Vascular Ehlers-Danlos Syndrome (VEDS), previously called Ehlers-Danlos syndrome type-IV, is a heterogeneous group of heritable connective tissue disorders characterized by thin, translucent skin, easy bruising, arterial, intestinal, and/or uterine fragility. There is large vessel involvement that leads to arterial rupture often preceded by aneurysm, arteriovenous fistulae, or dissection. Noninvasive imaging studies such as CT angiography and MR angiography are preferred as diagnostic studies for this condition. We are reporting a 4 years old girl who was presented with right sided unilateral convulsions and hypertension. CT angiogram showed stenosis with post-stenotic dilatation of coeliac and superior mesenteric arteries. There were extensive …