Journal of pediatric hematology/oncology
-
J. Pediatr. Hematol. Oncol. · May 1996
Case ReportsAtypical PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenitis) in a young girl with Fanconi anemia.
To describe a case of atypical, severe, periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA syndrome) in a patient with Fanconi anemia. Important aspects about the PFAPA syndrome and Fanconi anemia are reviewed. ⋯ In patients with underlying hematologic disease such as Fanconi anemia, PFAPA syndrome may be associated with severe clinical problems in contrast to otherwise normal children with the disorder.
-
J. Pediatr. Hematol. Oncol. · Feb 1996
Comparative StudyDetection of bone lesions in Langerhans cell histiocytosis: complementary roles of scintigraphy and conventional radiography.
This research was undertaken to determine the relative sensitivity of scintigraphic and radiographic bone survey examinations in detecting bone lesions in various regions of the skeleton in patients with a histopathologic diagnosis of Langerhans cell histiocytosis (LCH). ⋯ RNSS has a greater value in detecting sites of bone involvement with LCH than reported previously. RNSS is more sensitive than XRSS in detecting histiocytic lesions in the ribs, spine and pelvis and less sensitive in identifying lesions in the skull.
-
J. Pediatr. Hematol. Oncol. · Feb 1996
Newborn screening for sickle cell disease: 4 years of experience from California's newborn screening program.
In this article we describe the success of a unique newborn screening program for sickle cell disease and other hemoglobinopathies. We will present and discuss 4 years of experience from the California Newborn Hemoglobinopathy Screening Program. ⋯ The prevalence and ethnicity data presented here demonstrate the ineffectiveness of targeted screening and justify universal screening. Had targeted screening been performed in California during the past 4 years, 58 nonblack infants with sickle cell disease would have gone undiagnosed, and 6,921 nonblack infants with sickle cell trait would not have been identified.
-
J. Pediatr. Hematol. Oncol. · Nov 1995
Clinical TrialCyclophosphamide dose escalation in combination with vincristine and actinomycin-D (VAC) in gross residual sarcoma. A pilot study without hematopoietic growth factor support evaluating toxicity and response.
The Intergroup Rhabdomyosarcoma Study (IRS) initiated an escalating-dose cyclophosphamide (Cyc) pilot without hematopoietic growth factor (HGF) support in combination with vincristine (Vcr) and actinomycin-D (Amd), known as VAC, to establish a Cyc dose with myelotoxicity comparable to an ifosfamide (Ifos), Vcr, and Amd combination regimen (VAI). A Cyc dose equivalent to Ifos was to be determined when comparable myelotoxicity was achieved. ⋯ Myelotoxicity using 2.2 g Cyc/m2 in a single intravenous infusion was dose limiting in this VAC pilot without HGF. In the first year and overall, myelotoxicity is comparable to that with VAI using Ifos at 9.0 g/m2. An ongoing IRS-IV randomized trial of VAC and VAI should provide a comparison of the efficacy of Ifos and Cyc in children and adolescents with embryonal or alveolar rhabdomyosarcoma and undifferentiated soft-tissue sarcomas.
-
J. Pediatr. Hematol. Oncol. · Nov 1995
Long-term central venous access in patients with sickle cell disease. Incidence of thrombotic and infectious complications.
Central venous access devices (CVAD) have been used with increasing frequency in recent years among pediatric patients. We retrospectively reviewed our experience in 25 children and young adults with sickle cell disease (SCD) over a 4 1/2 year period in an attempt to define occurrence rates of perioperative complications, thrombosis requiring catheter removal, and infectious episodes. ⋯ The occurrence of thrombosis requiring catheter removal and infection in our population of patients with SCD was comparable to that reported in patients with malignant disease, cystic fibrosis and acquired immune deficiency syndrome. CVAD represents an effective, reliable, and reasonably safe means of establishing and maintaining venous access for a selective group of children and young adults with SCD who have limited peripheral venous access and require intravenous therapies.