We describe a case of a 9-yr-old child with sickle cell disease complicated by abdominal vaso-occlusive crisis and priapism. Both complications were successfully treated with a combination of epidural local anesthetics and morphine.
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Factors Associated with Microalbuminuria among Children with Sickle Cell Disease in a Tertiary Centre in South-South Nigeria.
Niger Med J
January 2025
Department of Haematology and Blood Transfusion, Rivers State University Teaching Hospital & Faculty of Basic Clinical Sciences, Rivers State University, Nigeria.
Background: Microalbuminuria, an early indicator of kidney damage in Sickle Cell Disease (SCD) patients, is linked to a heightened risk of chronic kidney disease (CKD) in adulthood. This study investigates the determinants of microalbuminuria in paediatric SCD patients in South-South Nigeria.
Methodology: This cross-sectional study was conducted over six months at the Rivers State University Teaching Hospital, Nigeria, involving 60 children with [HbSS genotype, SCD] in a steady state.
Screening for sickle cell disease by point-of-care tests in Italy: pilot study on 1000 at risk children.
Eur J Pediatr
January 2025
Department of Woman, Child and General and Specialized Surgery, University of Campania 'Luigi Vanvitelli', Via Luigi De Crecchio 4, Naples, Italy.
Unlabelled: Sickle cell disease (SCD) is a global health problem causing premature deaths and preventable severe chronic complications. A priority goal to improve outcomes both in the short and long term is the screening for early diagnosis and access to specialized care. In Italy, as in other countries, no systematic national screening program is available.
View Article and Find Full Text PDFSystemic Inflammatory Diseases in Children with Sickle Cell Disease: A French Multicenter Observational Study on Diagnostic and Therapeutic Issues.
Pediatr Blood Cancer
January 2025
Pediatrics Department, Jean Verdier Hospital, APHP, Bondy, France.
Background: Systemic inflammatory diseases (SIDs) have been reported in patients with sickle cell disease (SCD), but clinical data in children are scarce.
Objectives: To identify clinical and laboratory features at diagnosis of SID in children with SCD and to describe their evolution.
Methods: Data from children with SCD and SIDs were retrospectively collected in a French multicenter study from 1991 to 2018.
Strengthening advanced therapy for sickle cell disease in Africa: experience from sickle cell disease centre in Dar es Salaam, Tanzania.
BMJ Glob Health
January 2025
Sickle Cell Programme, Department of Haematology and Blood Transfusion, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania.
Despite progress in healthcare services for individuals living with sickle cell disease (SCD) in Africa, substantial gaps remain in advanced treatments for SCD. To help address this burden, Tanzania has established one of the largest single-centre SCD programmes in the world and developed an advanced therapy programme for SCD focused on patient engagement and advocacy, clinical activities involving exchange blood transfusion (ExBT) and haematopoietic stem cell transplant (HSCT), gene therapy (GT) preparedness, and enabling partnerships. This report describes the programme's genesis, structure and progress achieved.
View Article and Find Full Text PDFChallenges of Engaging Primary Care Providers in Specialized Telementoring Education About Sickle Cell Disease for Sickle Cell Specialists: Results from the Sickle Cell Disease Training and Mentoring Program for Primary Care Providers (STAMP) Project ECHO.
AJPM Focus
February 2025
Department of Medicine, University of Alabama, Bermingham, Alabama.
Introduction: Sickle cell disease (SCD) is an inherited blood disorder affecting approximately 100,000 individuals in the U.S. A lack of knowledgeable providers, particularly for adult patients, has led to a significant number of adults without access to high-quality care.
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