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Development of psychological and intellectual performance in transplanted sickle cell disease patients: A prospective study from pretransplant period to 5years after HSCT.
CONCLUSION: At the end of follow-up, the patients improved their physical and psychological well-being. This allowed them to build projects for the future and to manifest the desire of becoming an adult. Bone marrow transplantation in this cohort of children with SCD and severe cerebral vasculopathy is associated with improved performance as measured by the Wechsler scale. PMID: 23769628 [PubMed - as supplied by publisher]
Source: Archives de Pediatrie - June 11, 2013 Category: Pediatrics Authors: Bockenmeyer J, Chamboredon E, Missud F, Benkerrou M, Holvoët L, Ithier G, Lescoeur B, Yakouben K, Ouachée-Chardin M, Rohrlich PS, Duval M, Baruchel A, Dalle JH Tags: Arch Pediatr Source Type: research

Freedom in waiting: A ventricular assist device gives Aidan’s family independence
Aidan When Aidan was just 8 months old, his parents Patrick and Tavina received shocking news—their son had hypertrophic cardiomyopathy, a rare but serious disease that affects the muscle of the heart. Given his young age and severity of his condition, the early prognosis was bleak. “Things didn’t look good at first,” remembers Patrick. “We were steeling ourselves for the real possibility that Aidan wouldn’t make it to see his first birthday.” But, heart condition or not, little Aidan was a fighter, and with minimal medical management his condition stabilized. Over the years, he grew ...
Source: Thrive, Children's Hospital Boston - July 3, 2013 Category: Pediatrics Authors: Tripp Underwood Tags: All posts Heart conditions Our patients’ stories Christina VanderPluym Heart Center heart transplant hypertrophic cardiomyopathy MD VAD ventricular assist device Source Type: news

Idaho mom goes the distance to help teen with scoliosis
“You want a hospital with the best technology and the best nurses and doctors in the country. You expect that, and you expect it to be clean. Boston Children’s has all of that,” says Lisa Findlay, a mom from Hayden, Idaho. “What made the difference was how much everyone loves these kids. Everyone who walked into Aaron’s room, from the surgeons to nurses’ aides to janitors and child life specialists, was on a mission to help Aaron.” From the time he was born, Aaron encountered one medical challenge after another. He was born with hypoplastic left heart syndrome (HLHS), a serious congenital heart defect. By...
Source: Thrive, Children's Hospital Boston - April 2, 2015 Category: Pediatrics Authors: Lisa Fratt Tags: Orthopedics Our patients’ stories Source Type: news

Cerebral vasculopathy in children with sickle cell disease: Key issues and the latest data.
Abstract Cerebral vasculopathy is a common and severe complication of sickle cell disease in children. The pathophysiology consists of progressive damage to the basal intracranial arteries and cerebral microcirculation, while chronic anemia worsens exposure to cerebral hypoxia. It results in stroke and subclinical or poorly symptomatic ischemic lesions. Many clinical, biological, and radiological risk factors have been identified. The prevention strategy through systematic transcranial Doppler screening of large-vessel vasculopathy has revolutionized the management of this disease and has greatly decreased the ris...
Source: Archives de Pediatrie - December 15, 2017 Category: Pediatrics Authors: Corvest V, Blais S, Dahmani B, De Tersant M, Etienney AC, Maroni A, Ormières C, Roussel A, Pondarré C Tags: Arch Pediatr Source Type: research

Low CD8 T‐cells in neonates and infants prior to surgery and healthcare‐associated infections: a prospective observational study.
Cytotoxic T‐lymphocytes (CD8 T‐cells) are a powerful component of the adaptive immune system. They are involved in the eradication of intracellular infections, tumours, and contribute to rejection of organ transplants; and autoimmunity [1]. Several studies in humans and animals have demonstrated that altered host defence mechanisms after major surgery or trauma are an important risk in the development of postsurgical healthcare‐associated infections [2‐4]. These and other studies have demonstrated decreased counts and/or response of T‐lymphocytes to mitogenic activation in patients following general surgery, blun...
Source: Pediatrics International - April 1, 2013 Category: Pediatrics Authors: Stefan Grosek, Ziva Petrin, Andreja Natasa Kopitar, Tanja Gmeiner Stopar, Mirjana Petreska, Janez Primozic, Janez Erzen, Jan Grosek, Alojz Ihan Tags: Clinical Investigations Source Type: research

Predictors of Mortality in Pediatric Patients on Venoarterial Extracorporeal Membrane Oxygenation*
Objectives: Currently, there are no established echocardiographic or hemodynamic predictors of mortality after weaning venoarterial extracorporeal membrane oxygenation in children. We wished to determine which measurements predict mortality. Design: Over 3 years, we prospectively assessed six echo and six hemodynamic variables at 3–5 circuit rates while weaning extracorporeal membrane oxygenation flow. Hemodynamic measurements were heart rate, inotropic score, arteriovenous oxygen difference, pulse pressure, oxygenation index, and lactate. Echo variables included shortening/ejection fraction, outflow tract Doppler-derive...
Source: Pediatric Critical Care Medicine - November 1, 2014 Category: Pediatrics Tags: Extracorporeal Support Source Type: research

Liver or Combined Liver-Kidney Transplantation for Patients with Isolated Methylmalonic Acidemia: Who and When?
The hereditary disorders of vitamin B12 (cobalamin) and methylmalonic acid metabolism comprise a major group of organic acid disorders that are collectively common inborn errors of metabolism.1 Affected patients are medically fragile and suffer multisystemic complications, such as lethal metabolic instability, metabolic stroke, pancreatitis, end-stage renal failure, growth impairment, osteopenia, optic nerve atrophy, and neurocognitive delay.2 The frequency of these complications and their precipitants, long-term sequelae, and optimal treatment regimens remain ill-defined.
Source: The Journal of Pediatrics - April 13, 2015 Category: Pediatrics Authors: Jennifer L. Sloan, Irini Manoli, Charles P. Venditti Tags: Editorial Source Type: research

Thrombophilia testing in a tertiary paediatric hospital: Indications, outcomes and appropriateness
ConclusionWith improvements in thrombophilia testing practices at the Royal Children's Hospital AU$29 645.40 could be saved over 3 years. While there are improvements to be made in this area, in the overall context of the hospital's pathology testing budget, review of other areas such as inappropriate use of low‐cost, high‐volume tests may be of greater value in reducing the cost of pathology testing.
Source: Journal of Paediatrics and Child Health - May 15, 2015 Category: Pediatrics Authors: Aryanto Sudarmana, Paul Monagle Tags: Original Article Source Type: research

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

What Causes Hyperammonemia?
Discussion Reye’s syndrome (RS)is named for Dr. Douglas Reye who along with Drs. G. Morgan and J. Baral described encephalopathy and fatty accumulation and degeneration in children in a 1963 Lancet article. RS usually affects children but can occur at all ages. All organs can be affected but the liver and brain are primarily affected causing liver failure and encephalopathy as toxic metabolites (especially ammonia) accumulate, and intracranial hypertension and cerebral edema occurs. As the ammonia levels begin to rise (> 100 mg/dL) patients lose their appetite, have nausea and emesis and mental status changes whic...
Source: PediatricEducation.org - February 20, 2017 Category: Pediatrics Authors: pediatriceducationmin Tags: Uncategorized Source Type: news

Brain abnormalities in children and adolescents with chronic kidney disease.
CONCLUSION: Cerebral white matter abnormalities, including white matter injury, are under-recognized in pediatric CKD patients. Brain imaging studies through progression of CKD are needed to determine the timing of white matter injury and any potentially modifiable risk factors. PMID: 29967532 [PubMed - as supplied by publisher]
Source: Pediatric Research - July 2, 2018 Category: Pediatrics Authors: Matsuda-Abedini M, Fitzpatrick K, Harrell WR, Gipson DS, Hooper SR, Belger A, Poskitt K, Miller SP, Bjornson BH Tags: Pediatr Res Source Type: research