Splenic function is not maintained long-term after partial splenectomy in children with sickle cell disease.

Background: Partial splenectomy (PS) may allow preservation of splenic function in cases where splenectomy is indicated for hematologic diseases; however, the long-term outcomes are uncertain. We investigated the long-term outcomes of PS in patients with sickle cell disease (SCD).

Methods: A single-institution retrospective chart review was performed for children with SCD who underwent PS from 1997 to 2017.

A Quality Improvement Intervention to Decrease Postoperative Opioid Prescriptions in Pediatric Oncology Patients.

Purpose: This quality improvement initiative aimed to minimize opioid prescribing after oncologic pediatric surgery.

Methods: Retrospective surgical data collected at a pediatric cancer hospital from July 2016 to June 2018 included hospitalization details, oral morphine equivalents prescribed, unplanned visits/calls because of pain, and parental/patient satisfaction with pain control. The quality improvement initiative promoted opioid prescription at discharge on the basis of prior inpatient requirements and education regarding nonopioid analgesia.

Neutropenia at the time of subcutaneous port insertion may not be a risk factor for early infectious complications in pediatric oncology patients.

Background: The risk of infection associated with subcutaneous port (SQP) placement in patients with neutropenia remains unclear. We reviewed the rate of early infectious complications (<30 days) following SQP placement in pediatric oncology patients with or without neutropenia [absolute neutrophil count (ANC) <500/mm].

Methods: Baseline characteristics and infectious complications were compared between groups using univariate and multivariate analyses.

Overall Survival and Renal Function of Patients With Synchronous Bilateral Wilms Tumor Undergoing Surgery at a Single Institution.

Objectives: Wilms tumor is the most common renal cancer in children. Approximately 5% of children with Wilms tumor present with disease in both kidneys. The treatment challenge is to achieve a high cure rate while maintaining long-term renal function.

Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG).

Background: Sickle-cell anaemia is associated with substantial morbidity from acute complications and organ dysfunction beginning in the first year of life. Hydroxycarbamide substantially reduces episodes of pain and acute chest syndrome, admissions to hospital, and transfusions in adults with sickle-cell anaemia. We assessed the effect of hydroxycarbamide therapy on organ dysfunction and clinical complications, and examined laboratory findings and toxic effects.

The effects of hydroxycarbamide and magnesium on haemoglobin SC disease: results of the multi-centre CHAMPS trial.

In a phase-II multi-centre double-blinded trial, we evaluated haematological effects of oral hydroxycarbamide (HC) and magnesium (Mg) in patients with HbSC, aged 5-53 years old. Subjects were randomized to HC + placebo, Mg + placebo, HC + Mg, or placebo + placebo. The primary endpoint was the proportion of hyperdense red blood cells after 8 weeks.

Recruitment of infants with sickle cell anemia to a Phase III trial: data from the BABY HUG study.

Background: Protocol-eligible subjects may not be candidates for research participation or may decline. To determine factors that affected accrual, we evaluated enrollment in BABY HUG, a multi-center, randomized, placebo-controlled Phase III trial of hydroxyurea (HU) in infants with sickle cell anemia.

Methods: An anonymized registry of potential subjects served as the primary source of data.

Phase I study of magnesium pidolate in combination with hydroxycarbamide for children with sickle cell anaemia.

In sickle cell anaemia, red cell dehydration increases intracellular HbS concentration and promotes sickling. Higher erythrocyte magnesium reduces water loss through negative regulation of membrane transporters. Hydroxycarbamide (also known as hydroxyurea) reduces sickling partly by increasing intracellular HbF.

Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study.

The long-term efficacy and toxicity of hydroxyurea for infants are undefined, and its role in preventing organ dysfunction is unknown. Short-term feasibility of hydroxyurea administration, toxicities, hematologic effects, and effect on spleen function in infants with sickle cell anemia (SCA) were reported (Hydroxyurea Safety and Organ Toxicity [HUSOFT] trial). These infants completing 2 years of hydroxyurea therapy (20 mg/kg/d) were offered study extension with dose escalation to 30 mg/kg/d.

Brain imaging findings in pediatric patients with sickle cell disease.

Purpose: To determine prevalence of imaging abnormalities in the brain of children with sickle cell disease (SCD) and to identify clinical and methodological factors that influence prevalence estimate.

Materials And Methods: Magnetic resonance (MR) imaging and MR angiographic findings for 185 patients with SCD examined at St Jude Children's Research Hospital since 1993 were reviewed. At least two readers independently reviewed images.

The effect of hydroxyurea on vasculopathy in a child with sickle cell disease.

We report serial CNS findings in a girl with sickle cell disease and stroke. Religious considerations precluded transfusion and bone marrow transplantation; therefore, she received single-agent hydroxyurea therapy for almost 6 years. MR angiography showed that vascular patency improved, although diffuse cerebral atrophy slowly worsened.

Hydroxyurea therapy associated with declining serum levels of magnesium in children with sickle cell anemia.

Objective: To obtain quantitative serum levels of total and ionized magnesium (Mg(2+)) in children with homozygous sickle cell anemia (SCA) undergoing therapy with hydroxyurea.

Study Design: Five children, ages 11 to 14 years with homozygous SCA, were enrolled in a dose-escalating trial of hydroxyurea over an 18-month period. Serum levels of total and ionized magnesium together with ionized K(+), Na(+), and Ca(2+) were measured before hydroxyurea and every 6 months during hydroxyurea therapy.