Updated analysis: central venous access device infection rates in an expanded cohort of paediatric patients with severe haemophilia receiving prophylactic recombinant tissue plasminogen activator. Haemophilia 2016; 22 (1): 81-86
Central venous access devices (CVADs) are used in the care of paediatric haemophilic patients with difficult peripheral access, but their use is limited by complications such as infection. We previously published our experience with monthly recombinant tissue plasminogen activator (r-tPA) administration to CVADs of haemophilic patients as an intervention for infection prophylaxis, which suggested a 10-fold decrease in infection rate compared to published rates without r-tPA.This study was conducted to assess the CVAD infection rate in an expanded haemophilia cohort receiving r-tPA over an extended period.A retrospective review was performed on patients with haemophilia who received monthly r-tPA to CVADs, with data collected from January 1, 2008 to December 31, 2012. The data were merged with the previously reported data set (collected from June 1, 1998 to December 31, 2007).Over the entire observation period, there were 46350 CVAD days among 32 patients [26 severe factor VIII (FVIII) deficiency, six severe FIX deficiency]. Eight patients received immune tolerance therapy for inhibitors and 24 patients received prophylactic factor administration. No patients were HIV positive. Three infections were observed, with an overall infection rate of 0.06 infections per 1000 CVAD days.A low CVAD infection rate, similar to that observed in our previous study (0.04 per 1000 CVAD days), was observed in this expanded haemophilia cohort treated with prophylactic r-tPA, supporting the use of monthly r-tPA as CVAD infection prophylaxis in haemophilia patients.
View details for DOI 10.1111/hae.12772
View details for PubMedID 26248602
Neonatal Thrombocytopenia Neonatology: Clinical Practice and Procedures 2015
Bone marrow failure Rodacks Hematology: Clinical Principles and Applications 2015
Female Gender and Hispanic Ethnicity are Associated with Increased Risk of Subacute Methotrexate Encephalopathy Toxicology: Open Access 2015
Polycythemia Neonatology: Clinical Practice and Procedures 2015
Immune Thrombocytopenia in Children Less Than 1 Year of Age: A Single-institution 10-year Experience JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY 2013; 35 (5): 406-408
Immune thrombocytopenia (ITP) in children less than one year of age is less well characterized compared to ITP in toddlers and school-age children. We performed a 10-year retrospective review of ITP patients in this age-cohort at our institution. Diagnosis and classification were made according to the 2009 International Working Group criteria. Fourteen infants were identified. Their bleeding scores were Grades 1 to 2 (79%), Grade 3 (22%), Grades 4 to 5 (0%). Eight patients received treatment with a 75% response rate. Three patients (21%) developed chronic ITP. These observations suggest that ITP in very young patients is similar to typical childhood ITP.
View details for DOI 10.1097/MPH.0b013e3182580ab4
View details for Web of Science ID 000323224000025
View details for PubMedID 22767132
The Role of Oxidative Stress in Pediatric Immune Thrombocytopenia AMER SOC HEMATOLOGY. 2012
CLINICAL PRESENTATION AND MANAGEMENT OF SUBACUTE METHOTREXATE-INDUCED ENCEPHALOPATHY IN PATIENTS WITH PEDIATRIC MALIGNANCIES WILEY-BLACKWELL. 2012: 1050-1051
Chronic Oxidative Stress and Endothelial Dysfunction in Sickle Cell Disease Sickle Cell Disease: A New Vision for an Old Problem 2012
The role of vanin-1 and oxidative stress-related pathways in distinguishing acute and chronic pediatric ITP BLOOD 2011; 117 (17): 4569-4579
Pediatric immune thrombocytopenia (ITP) is usually self-limited. However, approximately 20% of children develop chronic ITP, which can be associated with significant morbidity because of long-term immunosuppression and splenectomy in refractory cases. To explore the molecular mechanism of chronic ITP compared with acute ITP, we studied 63 pediatric patients with ITP. Gene expression analysis of whole blood revealed distinct signatures for acute and chronic ITP. Oxidative stress-related pathways were among the most significant chronic ITP-associated pathways. Overexpression of VNN1, an oxidative stress sensor in epithelial cells, was most strongly associated with progression to chronic ITP. Studies of normal persons demonstrated VNN1 expression in a variety of blood cells. Exposure of blood mononuclear cells to oxidative stress inducers elicited dramatic up-regulation of VNN1 and down-regulation of PPAR?, indicating a role for VNN1 as a peripheral blood oxidative stress sensor. Assessment of redox state by tandem mass spectrometry demonstrated statistically significant lower glutathione ratios in patients with ITP versus healthy controls; lower glutathione ratios were also seen in untreated patients with ITP compared with recently treated patients. Our work demonstrates distinct patterns of gene expression in acute and chronic ITP and implicates oxidative stress pathways in the pathogenesis of chronic pediatric ITP.
View details for DOI 10.1182/blood-2010-09-304931
View details for Web of Science ID 000289984800022
View details for PubMedID 21325602
Development of Antibodies to Human Thrombin and Factor V in a Patient Exposed to Topical Bovine Thrombin PEDIATRIC BLOOD & CANCER 2010; 55 (6): 1195-1197
Bovine topical thrombin is commonly used for local hemostasis in pediatric surgery. Acquired inhibitors to coagulation factors, particularly to factor V and bovine thrombin, have been infrequently reported in the pediatric population. We report a 3-year-old male who developed a coagulopathy and clinical bleeding after cardiothoracic surgery, during which bovine topical thrombin was used for local hemostasis. Laboratory tests revealed elevated prothrombin, partial thromboplastin, and thrombin times, and a low factor V activity level. He was found to have both human-thrombin and factor V inhibitors, among the first reported cases of these combined inhibitors secondary to bovine topical thrombin. He was treated with intravenous immunoglobulin and steroids with a rapid and durable response.
View details for DOI 10.1002/pbc.22699
View details for Web of Science ID 000283757600028
View details for PubMedID 20979176