Digital Commons Network^™

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

Manuscripts, Articles, Book Chapters and Other Papers

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

Intervention For Cognitive Reserve Enhancement In Delaying The Onset Of Alzheimer's Symptomatic Expression (Increase), A Randomized Controlled Trial: Rationale, Study Design, And Protocol, Daniela C. Moga, Brooke F. Beech, Erin L. Abner, Frederick A. Schmitt, Riham H. El Khouli, Ashley I. Martinez, Lynne Eckmann, Mark Huffmyer, Rosmy George, Gregory A. Jicha

Pharmacy Practice and Science Faculty Publications

BACKGROUND: The course of Alzheimer's disease (AD) includes a 10-20-year preclinical period with progressive accumulation of amyloid β (Aβ) plaques and neurofibrillary tangles in the absence of symptomatic cognitive or functional decline. The duration of this preclinical stage in part depends on the rate of pathologic progression, which is offset by compensatory mechanisms, referred to as cognitive reserve (CR). Comorbid medical conditions, psychosocial stressors, and inappropriate medication use may lower CR, hastening the onset of symptomatic AD. Here, we describe a randomized controlled trial (RCT) designed to test the efficacy of a medication therapy management (MTM) intervention to reduce inappropriate …

Clinical Characteristics Of Children With Epilepsy Managed At An Urban Hospital In Africa: A Retrospective Study, Pauline Samia, Arwen Barr, Subira B. Levi, Kirsten A. Donald, Jo M. Wilmshurst, Charles R. Newton

Paediatrics and Child Health, East Africa

Background: Most children with epilepsy reside in resource-limited regions such as sub-Saharan Africa, where the majority of studies have been conducted in rural areas with limited investigations. Medical records from children with epilepsy seen at an urban hospital in Kenya were examined to provide a comprehensive description of epilepsy in children from this hospital.
Methods: A retrospective observational study was conducted which involved reviewing medical records of 426 epilepsy patients (260 males and 166 females) aged 0 - 18 years, seen in Nairobi, Kenya between February 2011 and December 2014. Results: The most frequent age at presentation; documented in 29% …

Cholangiocarcinoma Presenting As Humoral Hypercalcemia Of Malignancy: A Case Report And Literature Review, Burak Erdinc, Preethi Ramachandran, Ruchi Yadav, Sonu Sahni, Gardith Joseph

Touro College of Osteopathic Medicine (New York) Publications and Research

Humoral hypercalcemia of malignancy (HHM) is most commonly encountered in squamous cell carcinoma (SCC) of different organs, and It is characterized by elevated parathyroid hormone-related peptide (PTHrP) levels. It may be seen as a manifestation of cholangiocarcinoma (CCC) at presentation and later in the course of the disease. However, HHM due to intrahepatic cholangiocarcinoma is a rare association and is associated with a poor prognosis. We herein report a case of hypercalcemia presenting as the first manifestation of an underlying rare variant of intrahepatic cholangiocarcinoma. Our patient is a 57-year-old male who presented to the emergency room with severe symptoms …

Effect Of Hospital Volume On Outcomes Of Total Hip Arthroplasty: A Systematic Review And Meta-Analysis, Syed Hamza Mufarrih, Muhammad Owais Abdul Ghani, Russell S. Martins, Nada Qaisar Qureshi, Sayyeda Aleena Mufarrih, Azeem Tariq Malik, Shahryar Noordin

Department of Biological & Biomedical Sciences

Background: A shift in the healthcare system towards the centralization of common yet costly surgeries, such as total hip arthroplasty (THA), to high-volume centers of excellence, is an attempt to control the economic burden while simultaneously enhancing patient outcomes. The "volume-outcome" relationship suggests that hospitals performing more treatment of a given type exhibit better outcomes than hospitals performing fewer. This theory has surfaced as an important factor in determining patient outcomes following THA. We performed a systematic review with meta-analyses to review the available evidence on the impact of hospital volume on outcomes of THA.
Materials and methods: We conducted …

Point-Of-Care Versus Central Testing Of Hemoglobin During Large Volume Blood Transfusion., Justin Herman, Brian Park, Bharat K. Awsare, Frances Mae West, Denine Crittendon, Lilah Evans, Mary Harach, Julie Karp, Alexis R. Peedin, Marianna Lanoue, Barbara Goldsmith, Eugene P. Warnick, Michael Baram

Division of Pulmonary and Critical Care Medicine Faculty Papers

BACKGROUND: Point-of-care (POC) hemoglobin testing has the potential to revolutionize massive transfusion strategies. No prior studies have compared POC and central laboratory testing of hemoglobin in patients undergoing massive transfusions.

METHODS: We retrospectively compared the results of our point-of-care hemoglobin test (EPOC®) to our core laboratory complete blood count (CBC) hemoglobin test (Sysmex XE-5000™) in patients undergoing massive transfusion protocols (MTP) for hemorrhage. One hundred seventy paired samples from 90 patients for whom MTP was activated were collected at a single, tertiary care hospital between 10/2011 and 10/2017. Patients had both an EPOC® and CBC hemoglobin performed within 30 min …

Collagen Fibrils And Proteoglycans Of Peripheral And Central Stroma Of The Keratoconus Cornea - Ultrastructure And 3d Transmission Electron Tomography, Aljoharah Alkanaan, Robert J. Barsotti, Omar Kirat, Adnan Khan, Turki Almubrad, Saeed Akhtar

PCOM Scholarly Papers

Keratoconus (KC) is a progressive corneal disorder in which vision gradually deteriorates as a result of continuous conical protrusion and the consequent altered corneal curvature. While the majority of the literature focus on assessing the center of this diseased cornea, there is growing evidence of peripheral involvement in the disease process. Thus, we investigated the organization of collagen fibrils (CFs) and proteoglycans (PGs) in the periphery and center of KC corneal stroma. Three-dimensional transmission electron tomography on four KC corneas showed the degeneration of microfibrils within the CFs and disturbance in the attachment of the PGs. Within the KC corneas, …

Patient Safety Escape Room: A Graduate Medical Education Simulation For Event Reporting., Gretchen Diemer, Rebecca Jaffe, Dimitrios Papanagnou, Xiao Chi Zhang, Jillian Zavodnick

Department of Emergency Medicine Faculty Papers

Introduction: Although residents are on the front lines of patient care, they enter few formal patient safety reports on the adverse events and near misses they witness. Demonstrating the rationale and mechanics of reporting may improve this.

Methods: We designed and implemented an escape room patient safety simulation to incorporate active learning, gamification, and adult learning theory into intern patient safety onboarding. Interns from all sponsoring institution programs participated, identifying, mitigating, and reporting a range of patient safety hazards. Props and faculty time were the major resources required.

Results: One hundred twenty interns participated in this simulation in June 2018. …

Comparison Of Three Different Techniques Of Inverted Internal Limiting Membrane Flap In Treatment Of Large Idiopathic Full-Thickness Macular Hole., Fariba Ghassemi, Hassan Khojasteh, Alireza Khodabande, Lauren A. Dalvin, Mehdi Mazloumi, Hamid Riazi-Esfahani, Masoud Mirghorbani

Wills Eye Hospital Papers

Purpose: To evaluate and compare three different techniques of inverted internal limiting membrane (ILM) flap in the treatment of large idiopathic full-thickness macular hole.

Methods: In a comparative interventional case series, 72 eyes from 72 patients with large (> 400 µm) full-thickness macular hole were randomly enrolled into three different groups: group A - hemicircular ILM peel with temporally hinged inverted flap; group B - circular ILM peel with temporally hinged inverted flap; and group C - circular ILM peel with superior inverted flap. Best-corrected visual acuity (BCVA), anatomical closure rate, and ellipsoid zone (EZ) or external limiting membrane (ELM) …

Is It T3 Thyrotoxicosis? A Case Of Falsely Elevated Tri-Iodothyronine (T3) Levels Leading To A Diagnosis Of Multiple Myeloma, Nanik Ram, Saira Furqan, Sibtain Ahmed

Section of Diabetes, Endocrinology and Metabolism

We are presenting a case of falsely elevated T3 levels in a patient due to interference from monoclonal immunoglobulins. A 56-year-old, clinically euthyroid man referred to the endocrinology clinic of the Aga Khan university, Karachi Pakistan, for possible T3 thyrotoxicosis after thyroid function tests revealed total T3 >12.32 nmol/L (reference range 0.6-2.79), normal TSH, and total T4 level. There was a mismatch in clinical and laboratory parameters and preliminary laboratory results were suggestive of thyroid binding globulin abnormalities. Further evaluation in this context unmasked multiple myeloma. The presence of monoclonal immunoglobulins can lead to assay interference and spurious results. To …

Chloroquine Induction Par-4 And Treatment Of Cancer, Vivek M. Rangnekar

Radiation Medicine Faculty Patents

Described herein are methods for treating cancer in a subject in need thereof by administering chloroquine, or a salt or prodrug thereof, optionally with another agent that promotes Par-4 production to induce prostate apoptosis response-4 (Par-4) production by host cells, particularly non-cancerous host cells, to promote apoptosis in cancer cells, including androgen insensitive prostate cancer cells.

Chloroviruses, James L. Van Etten, Irina V. Agarkova, David D. Dunigan

Nebraska Center for Virology: Faculty Publications

Chloroviruses are large dsDNA, plaque-forming viruses that infect certain chlorella-like green algae; the algae are normally mutualistic endosymbionts of protists and metazoans and are often referred to as zoochlorellae. The viruses are ubiquitous in inland aqueous environments throughout the world and occasionally single types reach titers of thousands of plaque-forming units per ml of native water. The viruses are icosahedral in shape with a spike structure located at one of the vertices. They contain an internal membrane that is required for infectivity. The viral genomes are 290 to 370 kb in size, which encode up to 16 tRNAs and 330 …

Evaluation Of A Technology-Based Survivor Care Plan For Breast Cancer Survivors: Pre-Post Pilot Study., Talya Laufer, Bryan Lerner, Anett Petrich, Anna Quinn, Leah Ernst, Alicin Roop, Janet Knoblauch, Nick C. Leasure, Rebecca J. Jaslow, Sarah Hegarty, Amy Leader, Andrea Barsevick

Department of Medical Oncology Faculty Papers

BACKGROUND: As of 2016, almost 16 million individuals were cancer survivors, including over 3.5 million survivors of breast cancer. Because cancer survivors are living longer and have unique health care needs, the Institute of Medicine proposed a survivor care plan as a way to alleviate the many medical, emotional, and care coordination problems of survivors.

OBJECTIVE: This pilot study for breast cancer survivors was undertaken to: (1) examine self-reported changes in knowledge, confidence, and activation from before receipt to after receipt of a survivor care plan; and (2) describe survivor preferences for, and satisfaction with, a technology-based survivor care plan. …

Pbrm1 Acts As A P53 Lysine-Acetylation Reader To Suppress Renal Tumor Growth., Weijia Cai, Liya Su, Lili Liao, Zongzhi Z Liu, Lauren Langbein, Essel Dulaimi, Joseph R Testa, Robert G Uzzo, Zhijiu Zhong, Wei Jiang, Qin Yan, Qing Zhang, Haifeng Yang

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

p53 acetylation is indispensable for its transcriptional activity and tumor suppressive function. However, the identity of reader protein(s) for p53 acetylation remains elusive. PBRM1, the second most highly mutated tumor suppressor gene in kidney cancer, encodes PBRM1. Here, we identify PBRM1 as a reader for p53 acetylation on lysine 382 (K382Ac) through its bromodomain 4 (BD4). Notably, mutations on key residues of BD4 disrupt recognition of p53 K382Ac. The mutation in BD4 also reduces p53 binding to promoters of target genes such as CDKN1A (p21). Consequently, the PBRM1 BD4 mutant fails to fully support p53 transcriptional activity and is defective …

Mta3 Represses Cancer Stemness By Targeting The Sox2ot/Sox2 Axis, Liang Du, Lu Wang, Jinfeng Gan, Zhimeng Yao, Wan Lin, Junkuo Li, Yi Guo, Yuping Chen, Fuyou Zhou, Sai-Ching Jim Yeung, Robert P Coppes, Dianzheng Zhang, Hao Zhang

PCOM Scholarly Papers

Cancer cell stemness (CCS) plays critical roles in both malignancy maintenance and metastasis, yet the underlying molecular mechanisms are far from complete. Although the importance of SOX2 in cancer development and CCS are well recognized, the role of MTA3 in these processes is unknown. In this study, we used esophageal squamous cell carcinoma (ESCC) as a model system to demonstrate that MTA3 can repress both CCS and metastasis in vitro and in vivo. Mechanistically, by forming a repressive complex with GATA3, MTA3 downregulates SOX2OT, subsequently suppresses the SOX2OT/SOX2 axis, and ultimately represses CCS and metastasis. More importantly, MTA3

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

Manuscripts, Articles, Book Chapters and Other Papers

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

Virtual Surgical Planning In Subscapular System Free Flap Reconstruction Of Midface Defects., Brian P. Swendseid, Dylan F. Roden, Swar Vimawala, Tony Richa, Larissa Sweeny, Richard A. Goldman, Adam Luginbuhl, Ryan N. Heffelfinger, Shachika Khanna, Joseph Curry

Department of Otolaryngology - Head and Neck Surgery Faculty Papers

OBJECTIVES: Reconstruction of the midface has many inherent challenges, including orbital support, skull base reconstruction, optimizing midface projection, separation of the nasal cavity and dental rehabilitation. Subscapular system free flaps (SF) have sufficient bone stock to support complex reconstruction and the option of separate soft tissue components. This study analyzes the effect of virtual surgical planning (VSP) in SF for midface on subsite reconstruction, bone segment contact and anatomic position.

MATERIALS AND METHODS: Retrospective cohort of patients with midface defects that underwent SF reconstruction at a single tertiary care institution.

RESULTS: Nine cases with VSP were compared to fourteen cases …

Sex Differences In 10-Year Ischemic Cardiovascular Disease Risk Prediction In Chinese Patients With Prediabetes And Type 2 Diabetes., Lihong Yang, Anne Fish, Yuanyuan Zhu, Xiaodan Yuan, Jianing Li, Xiaoyun Wang, Li Yuan, Zhumin Jia, Chao Liu, Yunchen Xu, Qingqing Lou

Nursing Faculty Works

No abstract provided.

Impact Of Motor Therapy With Dynamic Body-Weight Support On Functional Independence Measures In Traumatic Brain Injury: An Exploratory Study, Emily F. Anggelis, Elizabeth Salmon Powell, Philip M. Westgate, Amanda C. Glueck, Lumy Sawaki

Physical Medicine and Rehabilitation Faculty Publications

BACKGROUND: Contemporary goals of rehabilitation after traumatic brain injury (TBI) aim to improve cognitive and motor function by applying concepts of neuroplasticity. This can be challenging to carry out in TBI patients with motor, balance, and cognitive impairments.

OBJECTIVE: To determine whether use of dynamic body-weight support (DBWS) would allow safe administration of intensive motor therapy during inpatient rehabilitation and whether its use would yield greater improvement in functional recovery than standard-of-care (SOC) therapy in adults with TBI.

METHODS: Data in this retrospective cohort study was collected from patients with TBI who receive inpatient rehabilitation incorporating DBWS (n = …

Children’S Cancer Pain In A World Of The Opioid Epidemic: Challenges And Opportunities, Michelle Fortier, Sun Yang, Michael T. Phan, Daniel Tomaszewski, Brooke N. Jenkins, Zeev N. Kain

Psychology Faculty Articles and Research

The opioid crisis in the United States has grown at an alarming rate. Children with cancer are at high risk for pain, and opioids are a first‐line treatment in this population. Accordingly, there is an urgent need to optimize pain management in children with cancer without contributing to the opioid crisis. This report details opportunities for this optimization, including clinical practice guidelines, comprehensive approaches to pain management, mobile health, and telemedicine. It is vital to balance appropriate use of analgesics with efforts to prevent misuse in order to reduce unnecessary suffering and minimize unintended harms.

Clinical Significance Of Obstructive Sleep Apnea In Patients With Acute Coronary Syndrome In Relation To Diabetes Status., Xiao Wang, Jingyao Fan, Yunhui Du, Changsheng Ma, Xin-Liang Ma, Shaoping Nie, Yongxiang Wei

Department of Emergency Medicine Faculty Papers

Objective: The prognostic significance of obstructive sleep apnea (OSA) in patients with acute coronary syndrome (ACS) according to diabetes mellitus (DM) status remains unclear. We aimed to elucidate the association of OSA with subsequent cardiovascular events in patients with ACS with or without DM.

Research design and methods: In this prospective cohort study, consecutive eligible patients with ACS underwent cardiorespiratory polygraphy between June 2015 and May 2017. OSA was defined as an Apnea Hypopnea Index ≥15 events/hour. The primary end point was major adverse cardiovascular and cerebrovascular events (MACCEs), including cardiovascular death, myocardial infarction, stroke, ischemia-driven revascularization, or hospitalization for …

Mutation Signature Analysis Identifies Increased Mutation Caused By Tobacco Smoke Associated Dna Adducts In Larynx Squamous Cell Carcinoma Compared With Oral Cavity And Oropharynx., Andrew P. South, Nicoline Y Den Breems, Tony Richa, Uche Nwagu, Tingting Zhan, Shiv Poojan, Ubaldo E. Martinez-Outshoorn, Jennifer M. Johnson, Adam J. Luginbuhl, Joseph Curry

Kimmel Cancer Center Papers, Presentations, and Grand Rounds

Squamous cell carcinomas of the head and neck (HNSCC) arise from mucosal keratinocytes of the upper aero-digestive tract. Despite a common cell of origin and similar driver-gene mutations which divert cell fate from differentiation to proliferation, HNSCC are considered a heterogeneous group of tumors categorized by site of origin within the aero-digestive mucosa, and the presence or absence of HPV infection. Tobacco use is a major driver of carcinogenesis in HNSCC and is a poor prognosticator that has previously been associated with poor immune cell infiltration and higher mutation numbers. Here, we study patterns of mutations in HNSCC that are …

Sexting Among Adolescents: Examining The Association Between Sexting And Self-Esteem, Hanaah Fannin

Physician Assistant Studies | Student Capstone Presentations

The primary objective of this study is to provide a systematic review of the published literature, examining the association between sexting and self-esteem in adolescents, and to identify gaps in existing knowledge to provide recommendations for future research.

Methods To Impair Hematologic Cancer Progenitor Cells And Compounds Related Thereto, Craig Jordan

Internal Medicine Faculty Patents

Primitive or progenitor hematologic cancer cells have been implicated in the early stages and development of leukemia and malignant lymphoproliferative disorders, including acute myelogenous leukemia (AML), chronic myelogenous leukemia (CML) and chronic lymphoid leukemia (CLL). Interleukin-3 receptor alpha chain (IL-3Ra or CD123) is strongly expressed on progenitor hematologic cancer cells, but is virtually undetectable on normal bone marrow cells. The present invention provides methods of impairing progenitor hematologic cancer (e.g., leukemia and lymphomic) cells by selectively targeting cells expressing CD123. These methods are useful in the detection and treatment of leukemias and malignant lymphoproliferative disorders. Also provided are compounds useful …

Sleep Aspects And Sleep Disorders In Children And Adolescents, Sameh Morkous Md, Faan

Department of Pediatrics

No abstract provided.

Genetic Variations In The Dopamine Reward System Influence Exercise Reinforcement And Tolerance For Exercise Intensity, Kyle D. Flack, Christopher Pankey, Kelsey Elise Ufholz, Luann Johnson, James N. Roemmich

Dietetics and Human Nutrition Faculty Publications

Background: Exercise is a reinforcing behavior and finding exercise highly reinforcing is characteristic of habitual exercisers. Genotypes related to dopamine metabolism moderate the reinforcing value of behaviors, but genetic moderators of exercise reinforcement have not been established.

Purpose: Determine whether singular nucleotide polymorphisms (SNPs) that moderate central reward pathways and pain neurotransmission are associated with exercise reinforcement, tolerance for exercise intensity, and usual physical activity.

Methods: Adults (n = 178) were measured for the reinforcing value of exercise relative to sedentary activities (RRV_exercise), minutes of moderate-to-vigorous physical activity (MVPA) and completed the Preference for and Tolerance …

Safety Of Endoscopic Retrograde Cholangiopancreatography (Ercp) In Pregnancy: A Systematic Review And Meta-Analysis, Mohamed Azab, Shishira Bharadwaj, Mahendran Jayaraj, Annie S. Hong, Pejman Solaimani, Mohamad Mubder, Hyeyoung Yeom, Ji Won Yoo, Michael L. Volk

School of Medicine Faculty Publications

Background/Aims: Endoscopic retrograde cholangiopancreatography (ERCP) is a technically challenging procedure rarely associated with severe postprocedure complications. Hormonal changes during pregnancy promote cholelithiasis, but there are limited clinical data available on the outcomes of ERCP in pregnant women. ERCP techniques without irradiation were recently introduced as potential alternative. We performed a systematic review and meta-analysis to assess the safety of ERCP in pregnancy and to compare outcomes of radiation versus nonradiation ERCP. Materials and Methods: A systematic search of PubMed, Medline/Ovid, Web of Science, and Google Scholar through April 18th, 2018 using PRISMA and MOOSE guidelines identified 27 studies reporting the …

Multitex Rct - A Multifaceted Intervention Package For Protection Against Cotton Dust Exposure Among Textile Workers - A Cluster Randomized Controlled Trial In Pakistan: Study Protocol, Asaad Ahmed Nafees, Sara De Matteis, Muhammad Masood Kadir, Peter Burney, David Coggon, Sean Semple, Paul Cullinan

Community Health Sciences

Background: In the Pakistani textile industry the prevalence of workplace respiratory illnesses, including byssinosis, is high. The MultiTex RCT study aims to determine the effectiveness of a multifaceted intervention package in reducing dust levels in cotton mills, decreasing the frequency of respiratory symptoms among cotton textile workers, and improving their lung function.
Methods/design: We will conduct a cluster-randomized controlled trial at 28 textile mills in Karachi. The intervention will comprise: training in occupational health for all workers and managers reinforced by regular refresher sessions; the formation of workplace committees to draw up, agree and promote a health and safety plan …

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

Manuscripts, Articles, Book Chapters and Other Papers

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

Treatment Of Dental Complications In Sickle Cell Disease., Priti Mulimani, Samir K. Ballas, Adinegara Bl Abas, Laxminarayan Karanth

Cardeza Foundation for Hematologic Research

BACKGROUND: Sickle cell disease is the most common single gene disorder and the commonest haemoglobinopathy found with high prevalence in many populations across the world. Management of dental complications in people with sickle cell disease requires special consideration for three main reasons. Firstly, dental and oral tissues are affected by the blood disorder resulting in several oro-facial abnormalities. Secondly, living with a haemoglobinopathy and coping with its associated serious consequences may result in individuals neglecting their oral health care. Finally, the treatment of these oral complications must be adapted to the systemic condition and special needs of these individuals, in …