The prevalence of bleeding disorders among healthy pediatric patients with abnormal preprocedural coagulation studies.

BACKGROUND: We evaluated the prevalence of hemostatic disorders among pediatric patients with abnormal screening coagulation tests. PROCEDURE: We analyzed 48 consecutive referrals for abnormal prothrombin times, partial thromboplastin times, or closure times obtained as preprocedural screens. Patients were evaluated by uniform diagnostic testing. RESULTS: Seventeen patients (35%) had an isolated nonspecific inhibitor (NSI). Six patients (12.5%) presented with mildly low factor activity with a concomitant NSI. These deficiencies were of unclear clinical significance. One patient (2%) had a lupus anticoagulant. Only 9 patients (19%) had a possible or true mild bleeding disorder: 5 patients (10%) had isolated low von Willebrand factor levels, 2 patients (4%) had possible type I von Willebrand disease, and 2 (4%) had platelet aggregation disorders. In all patients, personal and family bleeding history had a positive predictive value of 45% for hemostatic disorders. CONCLUSIONS: The most common diagnosis among the patients referred to us for abnormal preoperative coagulation tests was a NSI, which is not associated with an increased risk of operative bleeding complications. Less than 20% had a possible or true mild bleeding disorder. Although certain bleeding disorders can be occult in children and are associated with perioperative bleeding risks, our study demonstrates the inherent limitations in making a laboratory diagnosis of a bleeding disorder in pediatric patients preoperatively. Our findings contribute to existing doubt about the usefulness of prothrombin times, partial thromboplastin times, and closure times to identify occult bleeding disorders in this population.

Severe neutropenia in children: a single institutional experience.

Severe neutropenia (SN) is a rare disorder in childhood. This study aimed to document the approach to diagnosis and treatment of children with SN in a single university-based children's hospital, determine the types of SN seen in a 4-year period, and determine outcomes of the subtypes of SN. Forty-five children with SN were identified between 2000 and 2004. Two patients had autoimmune, 3 congenital, 3 familial, 6 cyclic, and 31 idiopathic SN. The median age of the patients with idiopathic SN was 15 months (3 mo to 17 y). Thirteen patients with idiopathic SN received filgrastim and 18 were observed. The history of severe infection and hospitalization at presentation was significantly more common among the patients who received filgrastim than those observed, but was not different between the 2 groups during the follow-up period. SN resolved in 16 patients and persisted in 14 patients. One patient with idiopathic SN did not respond to filgrastim and died of sepsis while she was still neutropenic. In summary, the majority of patients with SN had idiopathic SN, the infection risk was variable, treatment was based on clinical judgment rather than absolute neutrophil count, and approximately half of the patients had complete recovery.

Different rates of clinical trial enrollment between adolescents and young adults aged 15 to 22 years old and children under 15 years old with cancer at a children's hospital.

BACKGROUND: Over the last 30 years significant strides have been made in cure rates for children with cancer, but these improvements have not been seen in adolescents and young adults. The reasons for this lack of progress are multifactorial, but it is clear greater cure rates are correlated with controlled clinical trials. Our objective was to see if pediatric patients over the age of 15 had a lower rate of clinical trial enrollment, and if so, why. METHODS: We retrospectively analyzed the clinical data on all patients with new oncology diagnoses at Children's Hospital of Pittsburgh (CHP) diagnosed over a 5-year period from July 2001 to June 2006. RESULTS: Six hundred and forty new oncology patients were seen at CHP over this time, 501 under 15 years old and 139 patients aged 15 to 22. Thirty-six percent of all patients were treated on a clinical trial, including 38% of the younger patients and 27% of the older patients (P=0.03). Fifty-seven percent in the older group were not enrolled on a clinical trial because one was not available versus 41% in the younger group (P=0.04). There were no significant differences between the age groups when other reasons were analyzed. CONCLUSIONS: A significantly lower proportion of adolescents and young adults patients (aged 15 to 22) were placed on a treatment trial than younger patients. The lack of an open clinical trial was the main reason for this deficit. Interventions to address this discrepancy need to be instituted on a national level.