Elevated tricuspid regurgitant velocity as a marker for pulmonary hypertension in children with sickle cell disease: less prevalent and predictive than previously thought?

Although elevated tricuspid regurgitant velocity (TRV), an echocardiographic marker for pulmonary hypertension, has previously been tied to mortality in adult patients with sickle cell disease, recent data demonstrated that it correlates poorly with catheterization findings. We describe the largest echocardiographic evaluation of pediatric patients with sickle cell disease to date, specifically the results of a protocol whereby a TRV≥250 cm/s prompted further evaluation. We investigated if elevated TRV would independently identify patients at risk for increased morbidity.

Management of refractory pain in hospitalized adolescents with sickle cell disease: changing from intravenous opioids to continuous infusion epidural analgesia.

Background: Prolonged hospitalizations for sickle cell disease painful episodes are not uncommon, as analgesic options are often suboptimal.

Observations: Seven patients (15.4 ± 3.

The other side of abnormal: a case series of low transcranial Doppler velocities associated with stroke in children with sickle cell disease.

The prevalence of cerebrovascular events in sickle cell disease (SCD) can be as low as 10% by the age of 18 for overt cerebral infarction or strokes, up to 35% for silent cerebral infarction, and as high as 43/100 patient years for acute silent cerebral ischemic events. These events typically occur during childhood with a peak incidence between the age of 4 and 7 years. The cumulative risk of central nervous system events in SCD increases with age.

Concordant rhabdoid tumor of the kidney in a set of identical twins with discordant outcomes.

We report identical twin boys who each had stage IV rhabdoid tumor of the left kidney at the age of 5 months and 2 years, respectively. The 5-month-old boy, despite receiving chemotherapy, died of progressive disease at the age of 12 months. Following resection of the tumor, his twin brother was treated with 6 cycles of combination chemotherapy consisting of cisplatinum, doxorubicin, vincristine, cyclophosphamide, and actinomycin-D alternating with ifosfamide and etoposide.

Chemotherapeutic treatment of malignant chordoma in children.

Purpose: We describe the effect of multiagent chemotherapy for malignant chordoma. Previous reports of other patients with malignant chordoma treated with chemotherapy as well as other therapeutic interventions are reviewed.

Patients And Methods: We describe a 19-month-old girl with unresectable cervical chordoma metastatic to the lungs at diagnosis treated with multiagent systemic chemotherapy.