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Specialty: Pediatrics
Condition: Sickle Cell Anemia

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Total 52 results found since Jan 2013.

Pediatric Sickle Cell Disease - Past Successes and Future Challenges.
Abstract Once a fatal disease of childhood, more than 95% of patients born today with sickle cell disease (SCD) in developed countries are expected to survive into adulthood, largely because of improvements in supportive and preventive care (newborn screening, penicillin prophylaxis, transcranial Doppler (TCD) screening). Hydroxyurea (HU) therapy, the only oral medication currently available to prevent SCD complications, has become more widespread over the past 20 years. The NHLBI recommends that HU be offered to all patients with HbSS beginning at nine months of age, and the recently published Abnormal TCD with T...
Source: Pediatric Research - October 4, 2016 Category: Pediatrics Authors: Meier ER, Rampersad A Tags: Pediatr Res Source Type: research

Annelizabeth ’s story: Care that feels like home, close to home
When you’re 5, it’s nice to have a place that feels like a second home. Where there are lots of hugs. And songs. And games. And you can curl up and watch “Frozen,” your favorite movie. For Annelizabeth Jean-Baptiste, a spunky Waltham kindergartener, that place is Boston Children’s Hospital at Waltham. Annelizabeth, or Annie (but never Anna, she says), first came to Boston Children’s at Waltham two weeks after she was born. Her mother Elcie wasn’t expecting that her fourth child would need special care. “It was a difficult pregnancy. I was very excited and relieved when she was born.” But that sense of rel...
Source: Thrive, Children's Hospital Boston - August 29, 2016 Category: Pediatrics Authors: Lisa Fratt Tags: Our Patients’ Stories Boston Children's at Waltham Dr. Rachael Grace sickle cell disease Source Type: news

Progress in pediatrics in 2015: choices in allergy, endocrinology, gastroenterology, genetics, haematology, infectious diseases, neonatology, nephrology, neurology, nutrition, oncology and pulmonology
AbstractThis review focuses key advances in different pediatric fields that were published in Italian Journal of Pediatrics and in international journals in 2015. Weaning studies continue to show promise for preventing food allergy. New diagnostic tools are available for identifying the allergic origin of allergic-like symptoms. Advances have been reported in obesity, short stature and autoimmune endocrine disorders. New molecules are offered to reduce weight gain and insulin-resistance in obese children. Regional investigations may provide suggestions for preventing short stature. Epidemiological studies have evidenced th...
Source: Italian Journal of Pediatrics - August 26, 2016 Category: Pediatrics Source Type: research

Primary and Secondary Stroke Prevention in Children With Sickle Cell Disease
Children with sickle cell disease (SCD) have numerous acute and chronic complications, including central nervous system (CNS) disease, which can be debilitating over their life span. Recognition of risk factors for CNS disease and overt CNS disease should be properly identified by primary care providers, including physicians, physician assistants, and nurse practitioners. Here, we discuss an emerging and important early indicator of CNS disease in the form of silent cerebral infarcts and review overt stroke in patients with SCD.
Source: Journal of Pediatric Health Care - July 12, 2016 Category: Pediatrics Authors: A. Kyle Mack, Alexis A. Thompson Tags: Article Source Type: research

Genetic determinants and stroke in children with sickle cell disease.
CONCLUSION: There is a high incidence of stroke in male children and in children with SCA. Coexistence with α-thal and haplotypes of the beta globin chain cluster did not show any significant association with stroke. The heterogeneity between previously evaluated populations, the non-reproducibility between studies, and the need to identify factors associated with stroke in patients with SCA indicate the necessity of conducting further research to demonstrate the relevance of genetic factors in stroke related to SCD. PMID: 27268459 [PubMed - as supplied by publisher]
Source: Jornal de Pediatria - June 3, 2016 Category: Pediatrics Authors: Rodrigues DO, Ribeiro LC, Sudário LC, Teixeira MT, Martins ML, Pittella AM, Junior IO Tags: J Pediatr (Rio J) Source Type: research

Transcranial Doppler ultrasound in children with stroke and cerebrovascular disorders
This article summarizes the basic physics and variables used during TCD, recent pediatric data published on the use of TCD in stroke and cerebrovascular disorders and how it may impact diagnosis and management, and some issues to be resolved so that TCD can be put into clinical practice. Recent findings: In sickle cell disease in children, TCD is the gold standard stroke prediction tool. Recent data suggest that TCD may provide important information in ischemic stroke because of other childhood arteriopathies such as moyamoya syndrome, transient or focal cerebral arteriopathy, and genetic/syndromic causes. TCD may also det...
Source: Current Opinion in Pediatrics - November 10, 2015 Category: Pediatrics Tags: NEUROLOGY: Edited by Robert C. Tasker Source Type: research

Structural causes of ischemic and hemorrhagic stroke in children: moyamoya and arteriovenous malformations
Purpose of review: Moyamoya and arteriovenous malformations represent, respectively, significant sources of ischemic and hemorrhagic stroke in children after the first year of life. Although rarely encountered in routine pediatric practice, the potential severe morbidity of these entities, coupled with the typical acuity of their presentation, merit ongoing awareness of current relevant diagnostic and therapeutic strategies. Recent findings: Mutations in RNF213, ACTA2, and GUCY are implicated in moyamoya. Several common pediatric conditions – trisomy 21, sickle-cell disease, and neurofibromatosis type I – demonstrate a...
Source: Current Opinion in Pediatrics - November 10, 2015 Category: Pediatrics Tags: NEUROLOGY: Edited by Robert C. Tasker Source Type: research

Management of sickle cell disease: out-patient and community aspects
Sickle Cell Disease (SCD) is the commonest inherited disorder in England, affecting 1 in 2000 births, the majority born in London and of Black African family origins. Newborn bloodspot screening for SCD has now been implemented across the whole of England. This review considers the neonatal screening pathway and aspects of out-patient and community care which should be delivered within comprehensive care networks. Morbidity and mortality during childhood has declined due to implementation of effective care programmes, which include infection and stroke prophylaxis.
Source: Paediatrics and Child Health - July 7, 2015 Category: Pediatrics Authors: Paul T. Telfer Tags: Symposium: haematology Source Type: research

From Bermuda to Boston for surgery to protect the brain of a boy with sickle cell disease
Calvin Steede, who lives in Bermuda, will never forget the day in 2011 when he saw the movie “Winnie the Pooh” with his mother and sister. The film ended, and suddenly the boy who likes to draw and play soccer couldn’t put on his backpack. His arms had stopped working. He couldn’t stand, and soon he couldn’t talk. Calvin, now 11, had suffered a minor stroke, a complication of sickle cell disease and the first step of a journey that would take him to Dana-Farber/Boston Children’s Cancer and Blood Disorders Center for minimally invasive surgery to protect his brain from future strokes. Sickle cell disease Sickle ...
Source: Thrive, Children's Hospital Boston - June 19, 2015 Category: Pediatrics Authors: Irene Sege Tags: Diseases & conditions Dana-Farber/Boston Children's Cancer and Blood Disorders Center moyamoya sickle cell disease Source Type: news

Screening of cerebral vasculopathy in sickle cell anemia children using transcranial Doppler.
Abstract Cerebral vasculopathy exposes patients to a high risk of stroke, a major complication of sickle cell disease (SCD) associated with a high risk of death and disability. Transcranial doppler (TCD) ultrasonography used to identify SCD patients at risk of stroke may contribute to significantly reducing morbidity and mortality in these patients by indicating appropriate treatment. From March 2008 to February 2013, we conducted systematic screening for cerebral vasculopathy using TCD in 572 SCD patients (including 375 SS, 144 SC, 26 S/β(0), and 27 S/β(+) thalassemia patients) aged 1-17 years in a compre...
Source: Archives de Pediatrie - January 27, 2015 Category: Pediatrics Authors: Dorie A, Guindo A, Saro YS, Touré BA, Fané B, Dembelé AK, Diallo DA Tags: Arch Pediatr Source Type: research

Direct mail does not improve screening in sickle cell disease
Because children with sickle cell disease have an increased risk for stroke, annual transcranial Doppler (TCD) screening is recommended for children diagnosed with sickle cell disease from ages 2 to 16 years, yet implementation has been limited. Can letters mailed directly to parents and primary providers improve compliance? To investigate this, Bundy et al mailed reminders to families and primary doctors of eligible children in one Medicaid managed care organization, and compared results with 6 other Medicaid managed care organizations serving as controls.
Source: The Journal of Pediatrics - December 18, 2014 Category: Pediatrics Authors: Paul G. Fisher Tags: The Editors' Perspective Source Type: research

Transcranial Doppler Screening of Medicaid-Insured Children with Sickle Cell Disease
Transcranial Doppler screening reduces the risk of stroke in children with sickle cell disease. We tested the effect of informational letters sent to parents and doctors of Medicaid-insured children on improving screening efficiency. The letters did not improve the low baseline screening rates, suggesting the need for more aggressive outreach. Hematologist visits were correlated with increased screening rates.
Source: The Journal of Pediatrics - October 14, 2014 Category: Pediatrics Authors: David G. Bundy, Michael T. Abrams, John J. Strouse, Carl H. Mueller, Marlene R. Miller, James F. Casella Tags: Clinical and Laboratory Observations Source Type: research

Headache does not equal stroke in sickle cell
Headache is a common complaint in children with sickle cell disease. Dowling et al conducted a planned secondary analysis from the Silent Infarct Transfusion Trial to determine whether recurrent headache or, more specifically, migraine were predictive of silent cerebral infarction in children with sickle cell disease. In a cross-sectional study of 872 children, about one-third had headaches and 15% had migraines. Asymptomatic cerebral infarcts were not more likely in patients with recurrent headaches or migraine. As a take-home point, the authors note that neuroimaging may not be needed for children with sickle cell diseas...
Source: The Journal of Pediatrics - April 16, 2014 Category: Pediatrics Authors: Paul G. Fisher Tags: The Editors' Perspectives Source Type: research