RESUMO
We present the case of a healthy 13-year-old female adolescent who developed acute progressive swelling and pain in her right upper extremity that was secondary to an acute deep venous thrombosis of her right subclavian vein. Dynamic imaging revealed subclavian vein compression at the junction of the first rib and proximal third of the clavicle consistent with Paget-Schroetter syndrome, also known as effort-related thrombosis. The compressive etiology of her thrombus was most likely related to her cheerleading activity, in which she served as the pyramid base. The patient received multimodal therapy including anticoagulation, mechanical and site-directed thrombolysis, and a first rib resection. This case illustrates that frontline providers should have a high index of suspicion for an upper extremity thrombosis in pediatric patients who present with unilateral arm swelling.
Assuntos
Anticoagulantes/uso terapêutico , Veia Subclávia/patologia , Terapia Trombolítica/métodos , Trombose Venosa Profunda de Membros Superiores/diagnóstico , Adolescente , Feminino , Humanos , Flebografia , Costelas/cirurgia , Trombose Venosa Profunda de Membros Superiores/terapiaRESUMO
OBJECTIVE: To describe the off-label use of antithrombin concentrate in tertiary care pediatric hospitals across the US. STUDY DESIGN: This is a retrospective, multicenter, cohort study of 4210 admissions of children younger than 18 years of age who received antithrombin concentrate between 2002 and 2011 within the Pediatric Health Information System administrative database. An on-label admission was defined as an admission with an International Classification of Diseases diagnostic code for a primary hypercoagulable state; admissions without this code were classified as off-label. RESULTS: During the 10-year study period, off-label use of antithrombin concentrate increased 5-fold. Overall, 97% of study subjects received antithrombin off-label. Neonates younger than 30 days of age comprised the largest age group (45.7%) of use; 87% of patients had at least one complex chronic condition, with congenital heart/lung defects being the most prevalent primary diagnosis (36.3%). Extracorporeal membrane oxygenation was the most common procedure associated with antithrombin use (43.7%). CONCLUSIONS: The off-label use of antithrombin concentrate is increasing rapidly, particularly in critically ill children receiving extracorporeal membrane oxygenation, with few parallel studies to substantiate its safety or efficacy. Further preclinical and controlled clinical studies are critical to expanding our knowledge of this drug. In the meantime, antithrombin concentrate should be used judiciously by clinicians and following guidelines instated by hospitals.
Assuntos
Antitrombinas/uso terapêutico , Uso Off-Label , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Cardiopatias Congênitas/tratamento farmacológico , Hospitais Pediátricos , Humanos , Lactente , Recém-Nascido , Pneumopatias/congênito , Pneumopatias/tratamento farmacológico , Masculino , Estudos Retrospectivos , Centros de Atenção TerciáriaRESUMO
OBJECTIVE: To describe the off-label use of recombinant factor VIIa (rFVIIa) in tertiary care pediatric hospitals across the United States and to assess thrombotic events. STUDY DESIGN: A retrospective multi-center cohort study using the Pediatric Health Information System administrative database. Children 18 years of age or younger who received rFVIIa between 2000 and 2007 were included. A label admission was defined as an admission with an International Classification of Diseases diagnostic code for hemophilia or factor VII deficiency; admissions without these codes were classified as off-label. RESULTS: There were 4942 rFVIIa admissions, representing 3764 individual subjects; 74% (3655) of the admissions were off-label. There was a 10-fold increase in the annual rate of off-label admissions from 2000 to 2007 (from 2 to 20.8 per 10 000 hospital admissions, P < .001). The mortality rate in the off-label group was 34% (1258/3655). Thrombotic events occurred in 10.9% (399/3655) of the off-label admissions. CONCLUSIONS: The off-label use of rFVIIa in hospitalized children is increasing rapidly despite the absence of adequate clinical trials demonstrating safety and efficacy. Thrombotic events are common and mortality is high among patients receiving off-label rFVIIa. Further studies are warranted to determine whether these adverse events are attributable to rFVIIa.
Assuntos
Fator VIIa/efeitos adversos , Hemofilia A/tratamento farmacológico , Uso Off-Label , Trombose/epidemiologia , Adolescente , Criança , Pré-Escolar , Fator VIIa/uso terapêutico , Feminino , Seguimentos , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Proteínas Recombinantes/efeitos adversos , Proteínas Recombinantes/uso terapêutico , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Trombose/induzido quimicamente , Estados Unidos/epidemiologiaRESUMO
OBJECTIVE: To review the incidence of postoperative bleeding in children with type 1 von Willebrand disease (VWD) who were treated with a single institution protocol. STUDY DESIGN: We performed a retrospective study to determine the postoperative hemorrhage rate in pediatric patients with type 1 VWD who were treated via the Children's Hospital of Philadelphia institutional protocol. This protocol utilizes intravenous desmopressin (DDAVP), oral aminocaproic acid, and overnight observation. RESULTS: Between the years of 2000 to 2006, 41 children with type 1 VWD underwent an adenotonsillar procedure and were treated with this protocol. Seven patients (17%) experienced delayed (>24 hours after surgery) postoperative hemorrhage requiring intervention. Five of the 7 patients required cautery to control the bleeding, and the remaining 2 patients responded to DDAVP and aminocaproic acid alone. Older age and lower VW antigen levels were associated with postoperative hemorrhage (P = .05). CONCLUSIONS: Despite therapeutic intervention to decrease the risk of postoperative hemorrhage, the incidence of hemorrhage was higher in pretreated patients with type 1 VWD than in children without bleeding disorders. Further prospective studies are necessary to determine the optimal treatment to reduce bleeding complications in these patients.