Bleeding In Pediatric Patients Research Articles

Susceptibility-Based Differentiation of Intracranial Calcification and Hemorrhage in Pediatric Patients.

Differential diagnosis of intracranial hemorrhage versus calcification on conventional magnetic resonance images (MRIs) is often challenging. Although computed tomography (CT) confirms calcification, phase information obtained during susceptibility-weighted imaging can be useful in distinguishing between 2 pathologies. Fourteen patients previously diagnosed to have hemorrhage or calcification with imaging were included in the study retrospectively. Phase shift values of hemorrhage and calcification were compared by using Student t test. The pathologies identified were tuberous sclerosis, Sturge-Weber syndrome, craniopharyngioma, congenital cytomegalovirus, subependymal hemorrhages, and hemorrhagic microembolic infarction. Calcifications appeared hypointense whereas hemorrhages were hyperintense on phase maps (left-handed magnetic resonance system). Statistical comparison of phase shift values yielded significant difference between hemorrhage versus calcification (P < .01). Phase maps were found to offer valuable data to differentiate 2 pathologies when used complementary to conventional magnetic resonance images. Considering the relatively higher risks of radiation exposure in children, susceptibility-weighted imaging with phase maps may help to waive radiation exposure from CT.

Central nervous system bleeding in pediatric patients with factor XIII deficiency: A study on 23 new cases

BackgroundFactor XIII (FXIII) deficiency is an extremely rare bleeding disorder, which has the highest incidence in Sistan and Baluchistan Province in Iran, compared to its overall incidence around the world. This disorder has different clinical manifestations ranging from mild bleeding tendency to lethal bleeding episodes including central nervous system (CNS) hemorrhage. The aim of this study was to evaluate the demographic data, pattern of CNS bleeding, and the role of plasminogen activator inhibitor-1 (PAI-1) (PAI-1) 4G/5G and thrombin activatable fibrinolysis inhibitor (TAFI) Thr325Ile polymorphisms in intracranial and extracranial hemorrhages in 23 new cases of FXIII-deficient subjects.MethodsThis case–control study was conducted on 23 FXIII-deficient patients with CNS bleeding episodes and 23 patients as the control group with FXIII deficiency but without any history of CNS bleeding. Initially, to confirm the molecular defect, both groups were evaluated for the most frequently reported mutation of FXIII (Trp187Arg mutation) in a previous study in Sistan and Baluchistan Province. Then, demographic data, clinical manifestations, and pattern of CNS bleeding were determined. Eventually, the patients were assessed for PAI-14G/5G and TAFI Thr325Ile polymorphisms.ResultsThe results of this study revealed that all the subjects (including the case and control groups) were homozygous for Trp187Arg mutation. Nineteen patients (82.6%) had intracranial hemorrhage (ICH) and four patients (17.4%) had extracranial hemorrhage (ECH). Intraparenchymal hemorrhage was the most common form of ICH (89.5%), and epidural hemorrhage was observed in two patients (10.5%). Anatomic regions in patients with intraparenchymal hemorrhage were temporal in six (35.3%), occipital in four (23.5%), diffused intraparenchymal in four (23.5%), temporal-occipital in two (11.8%), and subdural with temporal in one (5.9%) patient.We found that in the case group, 14 patients (60.8%) were homozygous for TAFI Thr325Ile polymorphism and 8 cases (34.7%) were heterozygous. In the control group, 4 (17.4%) and 13 (56.5%) patients were homozygous and heterozygous, respectively (P < 0.001 vs. P < 0.01).We also found that an equal number of patients (two individuals) in the case and control groups (8.7% in each group) were heterozygous for PAI-14G/5G polymorphism.ConclusionIt seems that PAI-14G/5G polymorphism does not have any effect on occurrence of ICH and ECH in patients with FXIII deficiency, while TAFI Thr325Ile is a strong genetic risk factor (odds ratio:14.9, 95% confidence interval: 7.4–31.1).

C0105: Plasminogen Activator Inhibitor-1 Polymorphism is Not Associated with Central Nervous System Bleeding in Pediatric Patients with Factor XIII Deficiency

C0105: Plasminogen Activator Inhibitor-1 Polymorphism is Not Associated with Central Nervous System Bleeding in Pediatric Patients with Factor XIII Deficiency

Decreased Mortality From Intracranial Hemorrhage In Pediatric Patients With Hemophilia

Introduction and Objective Intracranial hemorrhage (ICH) is one of the most significant bleeding complications in hemophilia with a reported 20% mortality rate. A recent study from the Centers for Disease Control and Prevention Universal Data Collection project demonstrated that prophylaxis in patients with severe hemophilia reduced the risk of intracranial hemorrhage but mortality remained high (19.6 %). However 95% of those ICH deaths occurred in subjects over 20 years of age. This was in contrast to prior studies that demonstrated much higher mortality rates in children. Additional studies are needed to confirm this shift in the age distribution of ICH mortality. The objective of this study was to determine the mortality rate from ICH in pediatric patients with hemophilia admitted to US tertiary care hospitals. Patients and methods This retrospective multicenter cohort study utilized data from 43 free-standing children’s hospitals that contribute data to the Pediatric Health Information System administrative database. Subjects were male, less than 21 years of age, with an International Classification of Diseases (ICD-9) diagnostic code for hemophilia A or B, with an admission between 1/1/2002-12/31/2011. ICD-9 discharge codes were used to identify admissions during which an ICH was diagnosed. Subjects were classified as having an inhibitor if they received bypassing agents (either FEIBA or recombinant factor VIIa). Summary statistics include frequencies and percentages for categorical variables and median and interquartile range for continuous variables. Odds ratios (OR) with 95% confidence intervals were calculated using multivariable logistic regression to evaluate the strength of association between mortality and the presence of ICH, an inhibitor and hemophilia type (A versus B). Result During the 10 year study period there were a total of 8,325 admissions in 3,133 individual male subjects with either hemophilia A or B. 80% (2,506) of the subjects had hemophilia A and 14.4% (451) had an inhibitor. The median number of admissions per subject was 1 (interquartile range 1-3). Overall mortality for the entire cohort was low at 0.7% (21 deaths). There were a total of 327 admissions with ICD-9 codes for ICH in 265 (8.5%) subjects. 12.8% (34) of subjects had more than 1 ICH event during the study period. The median age for ICH was 2.2 (interquartile range 0.6-7.3) years. The ICH mortality rate was 1.8% (6 deaths). This represents an 11 fold decrease from prior ICH mortality estimates of 20%. 50% (3) of subjects who died from ICH had an inhibitor. For the entire cohort, mortality was associated with the presence of ICH (5.6 OR (2.3-14)) and the presence of an inhibitor (3.1 OR (1.2-7.8)). Conclusion This retrospective cohort study utilizing an inpatient pediatric administrative database confirms that mortality from ICH in pediatric patients is significantly reduced from prior studies. In this cohort, mortality from ICH was quite low (1.8%), representing an 11 fold decrease from previous mortality estimates. Potential reasons for this reduction include the routine use of prophylaxis in pediatric patients with severe hemophilia, increased availability of factor concentrates and aggressive management of patients with hemophilia and suspected ICH. Disclosures: No relevant conflicts of interest to declare.

Clinical features and endovascular treatment of intracranial arteriovenous malformations in pediatric patients

The purpose of this study was to characterize clinical features and evaluate the clinical outcome of endovascular embolization treatment intracranial arteriovenous malformations in pediatric patients. A cohort of children (age ≤ 18 years) with arteriovenous malformations (AVMs) from 2000 to 2012 was included. Predictors studied included patient gender, age, and angioarchitectural features, including AVM location, nidus morphology and size, venous drainage, and associated aneurysms. Treatment method, complications and outcomes were recorded. The features of AVMs were evaluated before the treatment. One hundred twenty-seven children (77 males, mean age 13.2 years) were included; 90/127 (70.9 %) children were presented with hemorrhage. AVM size and deep venous drainage were independently associated with hemorrhage; 66/127 patients (52 %) treated with endovascular embolization. Complete obliteration at the end of all endovascular procedures was achieved in 14/66 patients (21.2 %), with an average of 78 % (range, 20-100 %) volume reduction. A mean of 2.9 (range, 1-9) feeding pedicles was embolized per patient. Overall, nine complications occurred in a total of 123 procedures (7.3 %). There was no procedure-related death in this study population. There was no significant difference between patients with and without complications in terms of AVM grade, demographic characteristics, or embolization features. AVM size and deep venous drainage were independently associated with hemorrhage in pediatric patients. Endovascular procedure is feasible and safe for pediatric AVMs, and complete embolization can be achieved in small AVMs, while large AVMs can be adequately reduced in size for additional microsurgery or stereotactic radiosurgery.

Safety and efficacy of recombinant activated factor VII for refractory hemorrhage in pediatric patients on extracorporeal membrane oxygenation: a single center review

Medically refractory hemorrhage in patients on ECMO (extracorporeal membrane oxygenation) support can have catastrophic complications. Recombinant-Activated Factor VII (rFVIIa; NovoSeven®) may provide lifesaving hemostasis; however, there are reports of catastrophic thrombosis related to its administration. This review attempts to add safety and efficacy data to existing literature regarding the use of rFVIIa for refractory hemorrhage in pediatric patients on ECMO support. Design/ A retrospective chart review was performed for all pediatric patients on ECMO who received rFVIIa for refractory hemorrhage from 2004 to 2009. Data was extracted for each refractory bleeding event, including patient blood loss and transfused blood products in the 6 hours before the first dose, between rFVIIa doses and in the 6 hours after the final dose. For purposes of data collection, a hemorrhagic event was defined as new onset hemorrhage or a hemorrhage occurring at least 12 hours after the most recent dose of rFVIIa. In total, seven patients aged 1 month to 15 years received rFVIIa for 14 different hemorrhagic events. There was no significant difference in blood loss or blood product transfusion associated with rFVIIa administration. There was one patient-related and one ECMO-related complication temporally associated with rFVIIa administration: decreased ECMO circuit oxygenator efficiency and the development of an intra-gastric clot requiring surgical evacuation. These data suggest limited efficacy for rFVIIa use for refractory hemorrhage in pediatric patients on ECMO support. There were two non-catastrophic complications temporally associated with its administration.

Readmissions for warfarin-related bleeding in pediatric patients after hospital discharge

Warfarin therapy in pediatric patients can be difficult to manage with bleeding as a primary adverse event. Therapy initiation can be difficult as doses to achieve therapeutic outcomes are being determined. Evaluation of readmission for bleeding in pediatric patients discharged on warfarin therapy may be useful to prevent adverse events. The Pediatric Health Information System (PHIS) was queried to identify all patients <19 years of age who were discharged from a pediatric hospital on warfarin therapy. Patients who were readmitted with bleeding in the first 30 days after discharge were identified and patient variables, hospital stay variables, and medications at discharge were identified. Univariate and multivariate analysis was performed to identify independent risk factors for bleeding readmission. A total of 4,883 patients met study criteria (56% male, mean age 10.1 + 5.9 years). The two most common indications for warfarin therapy were cardiac valve replacement (23.6%) and Fontan procedure (19.5%). Ninety-seven patients (1.99%) were readmitted with bleeding within 30 days of discharge [median time 9 days (IQR 5-16 days)]. Multivariate analysis identified Asian race (OR 4.0, P < 0.01); mitral valve replacement (OR 2.5, P < 0.01); escitalopram at discharge (OR 4.2, P = 0.02); levofloxacin at discharge (OR 8.3, P < 0.01); lansoprazole at discharge (OR 1.7, P = 0.047); and length of stay (OR 1.01, P = 0.047) as significant for bleeding readmission. Pediatric patients discharged on warfarin may be readmitted for bleeding within 30 days if risk factors are present. Risk factors include patient genetic profile, drug interactions, and indications with higher goal INR values.

Use of CT enterography for the diagnosis of lower gastrointestinal bleeding in pediatric patients

Diagnosis of lower gastrointestinal bleeding (LGIB) represents a significant diagnostic and therapeutic challenge for any physician. While CT enterography (CTE) has been applied in adults with occult LGIB, its use in children has been limited to evaluation of Crohn's disease. We reviewed 6 patients ages 4–15 who underwent CTE for LGIB at a tertiary pediatric institution. In sum, CTE appears to be a valuable tool for localizing the source of LGIB prior to surgical or endoscopic intervention. However, rapid lesion identification must be weighed against the increased radiation exposure and patient discomfort due to bowel distention associated with this diagnostic technique.

Subdural hemorrhage in pediatric patients with enlargement of the subarachnoid spaces

Enlargement of the subarachnoid spaces has been theorized as a risk factor for the development of subdural hemorrhage (SDH). As the finding of unexplained SDH in children often raises suspicion for nonaccidental trauma, the possibility of increased risk of SDH in children with enlargement of the subarachnoid spaces has important clinical, social, and legal implications. Therefore, the authors evaluated the frequency of SDH in a cohort of children with enlargement of the subarachnoid spaces. The authors identified children younger than 2 years of age who were diagnosed with enlargement of the subarachnoid spaces on MRI or CT scanning in a large primary care network between July 2001 and January 2008. The authors excluded children who had enlargement of the subarachnoid spaces diagnosed on imaging performed for trauma or developmental delay, as well as children with a history of prematurity, diagnosis of intracranial pathology, or metabolic or genetic disorders. Chart review recovered the following data: patient demographics, head circumference, history of head trauma, and head imaging results. For the subset of children with SDH, information regarding evaluation for other injuries, including skeletal survey, ophthalmological examination, and child protection team evaluation, was abstracted. There were 177 children with enlargement of the subarachnoid spaces who met the inclusion criteria. Subdural hemorrhage was identified in 4 (2.3%) of the 177 children. All of the children with SDH underwent evaluations for suspected nonaccidental trauma, which included consultation by the child protection team, skeletal survey, and ophthalmological examination. Additional injuries (healing rib fractures) were identified in 1 of 4 patients. None of the 4 children had retinal hemorrhages. Only the child with rib fractures was reported to child protective services due to concerns for abuse. Only a small minority of the patients with enlargement of the subarachnoid spaces had SDH. Evidence of additional injuries concerning for physical abuse were identified in a quarter of the children with enlargement of the subarachnoid spaces and SDH, suggesting that an evaluation for suspected nonaccidental trauma including occult injury screening should be performed in cases of SDH with enlargement of the subarachnoid spaces. In the absence of additional injuries, however, the presence of an unexplained SDH in the setting of enlargement of the subarachnoid spaces may be insufficient to support a diagnosis of nonaccidental trauma.

Evaluation and Management of Massive Endotracheal Hemorrhage in Pediatric Patients: A Case Report and Review of Literature

Massive hemoptysis is a frightening and life-threatening event in children. Prompt, aggressive evaluation and management are necessary. The most common cause of hemoptysis in a pediatric patient is infectious, but other various etiologies including tracheotomy related problems, cystic fibrosis, bronchiectasis, congenital anomalies of the cardiopulmonary vasculature and suction trauma must be considered as well. Presented is a report of a case of acute, massive endotracheal hemorrhage with multiple methods of management including balloon tamponade, highly selective embolization, and mainstem occlusion. This case is an addition to our previously reported case series of nine pediatric patients with massive pulmonary hemorrhage. Various diagnostic and management techniques of hemoptyis in pediatric patients are discussed with an extensive review of the literature.

Propranolol Safety Profile in Children

Data regarding the use of propranolol in pediatrics are limited despite its widespread use in adults. Since 1984, Propranolol has been used for the prevention of portal hypertensive hemorrhage in pediatric patients. Recently it has been also used for the management of hemangiomas in addition to other indications. The purpose of this review is to evaluate safety and efficacy of propranolol use in the pediatric population, highlighting the most important reported side effects, warnings and precautions.

Use of Recombinant Activated Factor VII for Controlling Refractory Postoperative Bleeding in Children Undergoing Cardiac Surgery With Cardiopulmonary Bypass

To review the efficacy, safety, and dose of recombinant activated factor VII in off-label management of refractory bleeding in pediatric patients with congenital heart disease undergoing cardiac surgery with cardiopulmonary bypass. A retrospective database analysis with medical records review. A single research hospital. Ninety pediatric patients with uncontrolled postoperative hemorrhage after cardiac surgery with cardiopulmonary bypass for congenital heart disease. Intravenous recombinant activated factor VII treatment according to institutional treatment protocol. The recombinant activated factor VII treatment was effective in reducing bleeding in 78 pediatric patients. The 12 patients who failed to respond had surgical sources of bleeding. The mean blood loss decreased from 51.04 mL/kg/2 h to 7.8 mL/kg/2 h (p < 0.001) in neonates, from 40.2 mL/kg/2 h to 7.7 mL/kg/2 h (p < 0.001) in infants, and from 29.1 mL/kg/2 h to 4.0 mL/kg/2 h in children (p < 0.001). The mean (standard deviation) total recombinant activated factor VII doses required to achieve hemostasis were 131.7 (69.8) μg/kg in neonates, 104.6 (36.0) μg/kg in infants, and 44.6 (15.3) μg/kg in children aged 1 to ≤18 years. There was no evidence of thrombosis in the first 24 hours after the administration of recombinant activated factor VII. Recombinant activated factor VII treatment reduced blood loss and transfusion requirements and prevented re-exploration in the majority (83.8%) of pediatric cardiac surgery patients. High doses were required to discontinue bleeding promptly in neonates, the majority of whom had hypoplastic left-heart syndrome. No treatment-related thrombotic events were observed.

Severe pelvic fracture-related bleeding in pediatric patients: does it occur?

Pediatric pelvic fractures are rare and less likely to cause hemodynamic instability than similar injuries in adult patients. The associated injuries are common, and they have a major impact on mortality. The aim of the present study was to evaluate the risk of life-threatening hemorrhage associated with unstable pelvic fractures in children. We identified retrospectively all pediatric pelvic fractures (ring and acetabulum) treated at Helsinki University Central Hospital during a 10-year period (1998-2007). Stable A-type fractures (fractures not involving the pelvic ring) were excluded. All available pre- and in-hospital medical records were reviewed. The collected data consisted of patient characteristics, mechanisms of injury, vital signs, laboratory tests, care given, other injuries diagnosed, and the 30-day survival rate. There were 71 (40 males) pediatric patients (median age 14, range 1-16years) with unstable pelvic fractures; 66 pelvic ring and 5 acetabulum fractures. The most common mechanism of injury was traffic accident (69%). Four patients had life-threatening bleeding. All had fracture of a mature pelvic ring, but the source of massive bleeding was pelvic ring fracture in only two patients (2.8% of all patients). No acetabulum fracture-related major pelvic bleeding was observed. One patient (age 16years) required emergency surgery and angioembolization for pelvic bleeding. No life-threatening pelvic bleeding was seen among patients with immature bony pelvis. Pelvic ring fractures were surgically treated in 25 patients. Two patients died from head injuries (overall mortality 2.8%), but there were no bleeding-related deaths. We conclude that life-threatening bleeding from pelvic or acetabular fractures in pediatric patients is rare (2.8%), and does not contribute to the overall mortality.

Minimizing bleeding associated with mechanical circulatory support following pediatric heart surgery☆

The use of extracorporeal membrane oxygenation (ECMO) to support patients with early postcardiotomy heart failure may be associated with catastrophic bleeding, making its use undesirable. However, postcardiotomy mechanical circulatory assistance is necessary in some patients to allow for myocardial recovery. We have assembled a centrifugal pump system (CPS) that does not require early systemic anticoagulation. This study compares postoperative bleeding in pediatric patients placed on standard ECMO versus CPS within 24h of cardiotomy. Between November 2002 and February 2007, 25 patients (age 0 days-1.72 years) received postcardiotomy mechanical support. Fourteen patients were placed on ECMO and 11 patients were placed on CPS within 24h of surgical repair. Retrospective analysis was performed of chest-tube drainage at multiple time points following initiation of mechanical support. Additional variables, including Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1) score, total time on mechanical support, 30-day mortality, activated clotting time, blood-product administration, circuit-related complications, and circuit changes were also analyzed. Patients on ECMO (0.30 ± 0.39 years) and CPS (0.40 ± 0.56 years) were of similar age (p = 0.64). Patients on ECMO (0.3 ± 0.1m(2)) and CPS (0.3 ± 0.1m(2)) had similar body surface areas (p = 0.46). Patients placed on CPS had significantly less chest-tube drainage during the first 4h of support. Activated clotting times appeared to be higher during the first 12h of ECMO versus CPS. There was no statistical difference between ECMO and CPS with respect to the following variables: RACHS-1 score, time on support, 30-day mortality, circuit-related complications, and circuit changes. Blood-product administration at 24h of support was significantly less (p = 0.04) for patients on CPS versus ECMO. Mechanical circulatory support can be provided without the complication of clinically significant bleeding if a specialized circuit is used. This has important implications for the decision to use mechanical support in the immediate postoperative period in the face of ventricular failure. In addition, early mechanical support can be used with a low incidence of circuit-related complications.

Recombinant-activated factor VII for control and prevention of hemorrhage in nonhemophilic pediatric patients

A total of 108 episodes among 103 nonhemophilic pediatric patients (nine newborns, 16 infants and 78 children) treated with recombinant factor-activated VII (rFVIIa) were evaluated retrospectively. These episodes were divided into two groups: group 1 included 86 occurrences for hemorrhagic control of ongoing massive bleeding due to thrombocytopenia and coagulopathy unresponsive to blood component therapy in patients with dengue hemorrhagic fever, life-threatening, intraoperative and postoperative bleeding; group 2 included 22 episodes for prevention of hemorrhage with invasive procedures in patients with chronic liver disease and associated coagulopathy, and patients without preexisting hemostatic disorder but at high risk due to their underlying diagnosis and required surgical intervention. The effective control of hemostasis response rate in group 1 was significantly lower than in group 2. The median total dose per kilogram of rFVIIa group 1 was twice that of group 2. The overall case-fatality rate related to bleeding or underlying conditions was 31.1% (32/103). Adverse events were observed in three patients (2.9%) receiving rFVIIa for control of intraoperative and postoperative bleeding in the setting of corrective cardiac surgery. These results support the safety and potential benefit of rFVIIa for control and prevention of hemorrhage in pediatric patients without congenital hemophilia.

Fatal Outcome of Recombinant Factor VIIa in Heart Transplantation With Extracorporeal Membrane Oxygenation

Recombinant activated factor VII (rFVIIa) has been approved for treatment of bleeding episodes in patients with hemophilia and in nonhemophilia patients with acquired antibodies against factor VIII or IX. The application of rFVIIa in nonapproved settings, as in cardiac surgery, has not been established. It raises concerns regarding its safety. We used rFVIIa in a patient with excessive nonsurgical bleeding on extracorporeal membrane oxygenation, which was established for early graft failure after heart transplantation and after 3 months of biventricular assist device support. After rFVIIa administration, cardiac thrombosis developed and caused the patient's death.

Post-tonsillectomy bleeding in children with von Willebrand disease: A single-institution experience

Objectives 1) Compare rates of post-tonsillectomy bleeding in pediatric patients with and without von Willebrand disease (vWD). 2) Identify factors that may increase the risk for post-tonsillectomy bleeding in children with and without vWD. Study Design Historical cohort study. Setting Tertiary care, university-based pediatric hospital. Subjects and Methods Medical records were examined for 99 patients with vWD and 99 patients without vWD younger than 18 years who underwent tonsillectomy with or without adenoidectomy from August 1997 to October 2005. Subjects were matched for age, year of surgery, type of surgery, and indication for surgery. Results Post-tonsillectomy hemorrhage occurred in eight of 99 (8%) vWD patients and in six of 99 (6%) non-vWD patients ( P = 0.58, odds ratio 1.36, 95% CI 0.45-4.08). A two-sample test of proportions demonstrated lower and upper limits of −0.051 and 0.092. Four of eight children with vWD and two of six non-vWD patients required surgical intervention for control of bleeding. Ninety-three of 99 vWD patients received desmopressin acetate (DDAVP) preoperatively. In patients with vWD who responded to DDAVP challenge, there was no increased likelihood of post-tonsillectomy bleeding compared with non-vWD patients. No significant difference in the number of bleeding events was noted on the basis of demographics, preoperative laboratories, or use of aminocaproic acid. Conclusion Children with vWD undergoing tonsillectomy have a postoperative bleeding rate similar to that of a matched group. However, the sample size was not sufficient to eliminate the possibility of a clinically important difference between the two groups.

Adult to pediatric living donor liver transplantation for portal cavernoma

Portal cavernoma (PC) is an important cause of non-cirrhotic portal hypertension with severe complications, such as variceal hemorrhage in pediatric patients. With the development of new surgical techniques, living donor liver transplantation (LDLT) has recently been recognized as a viable but challenging treatment option for PC. The purpose of the present study was to summarize the efficacy of LDLT in PC patients and to carry out a follow-up study of pediatric recipients. The primary indication for LDLT in our research was PC with severe variceal bleeding and liver function decompensation. Three patients were diagnosed with PC following evaluation with computed tomography angiography and abdominal color Doppler ultrasonography (CDU). Various surgical techniques, including jump bypass grafting for portal vein anastomosis, were carried out according to the range and degree of cavernous transformation within the splenic vein and superior mesenteric vein. Postoperative CDU confirmed the early integrity of the portal vein (PV) in each patient. PV rethrombosis occurred in one patient 7 days after LDLT, despite anticoagulation therapy with coumadin. Two of the three patients had no further episodes of variceal hemorrhage during the 2-year follow-up period. The present study is the first report of the successful use of LDLT to treat pediatric PC patients. We conclude that LDLT is effective for the majority of pediatric patients with PC.

Use of Recombinant Factor VIIa (NovoSeven) in Pediatric Cardiac Surgery

Significant post-operative bleeding can be encountered in a small population of pediatric surgical patients requiring cardiopulmonary bypass (CPB). Recombinant factor VIIa (Novo-Seven) has been advocated as a possible off-label rescue therapy for these individuals when conventional blood component therapy alone is inadequate. This study retrospectively evaluates rFVIIa administration for the treatment of severe bleeding in pediatric patients immediately after cardiac surgical procedures requiring CPB. The records of 15 patients receiving rFVIIa for excessive rates of bleeding refractory to conventional blood component therapy were studied. Blood product utilization, rates of blood loss, and evidence of pathologic sequelae were compared with matched historical controls in retrospective fashion. NovoSeven doses ranged from 76 to 282 μg/kg (group 1 &lt;30 kg) and 26 to 956 μg/kg (group 2 &gt;30 kg). Blood product administration patterns were not significantly different (p &gt; .05) in the intensive care unit (ICU) between patient groups receiving rFVIIa and those not treated. Bleeding rates (mL/kg/h) for the first 2 hours after admission to the ICU remained statistically unchanged but were significantly increased for those time periods &gt;3 hours in patients &lt;30 kg treated with NovoSeven. Patients &gt;30 kg did not exhibit statistical differences in the rate of bleeding or the administration of blood products compared with matched controls. A significant reduction in prothrombin time (p = .001) and partial thromboplastin time (p = .02) was noted in patients &lt;30 kg receiving rFVIIa. There were no pathologic sequelae directly attributed to the administration of rFVIIa in any patients treated. Trends in the improvement of bleeding disturbances were noted in the ICU in patients &lt;30 kg treated with rFVIIa, subsequent to blood component therapy. The rate of bleeding (mL/kg/h) was improved in patients &lt;30 kg for the first 2 hours in the ICU. For individuals &gt;30 kg, there was no apparent benefit from the administration of rFVIIa.

The Effectiveness of Recombinant Factor VIIa in the Treatment of Bleeding in Pediatric Patients: A Ten-Year Experience from 1997 to 2007.

Introduction: Recombinant factor VIIa (rFVIIa) has been used in the treatment of bleeding in Thailand since 1997.Methods: 125 patients receiving rFVIIa at the Faculty of Medicine, Ramathibodi Hospital and Regional Hospitals from 1997 to 2007 were retrospectively evaluated. Their age group ranged from newborn (n=11), infants (n=21), children (n=86) to adolescents (n=7). They were divided into 2 groups. Group 1 included 11 hemophiliacs with high inhibitor (A10, B1) exhibiting 90 episodes of bleeding or undergoing surgeries. The rFVIIa 100–200 μg/kg at 1–2 h interval for 1–4 doses combined with prednisolone, transamine, fibrin glue and dental splint were given. In cases of surgery, 100 μg/kg at 1–2 h interval was given in the first 24 h and extended to 3–4 h until the removal of stitches or 7–14 days after the surgical procedures. Sequential use of rFVIIa with non-activated or activated prothrombin complex concentrates were given to 9 patients. Group 2 included 114 non-hemophiliacs with or without pre-existing hemostatic disorder for controlling excessive bleeding unresponsive to conventional blood components or for preventing bleeding in invasive procedures. There were 122 episodes involving patients with Dengue Hemorrhagic Fever (n=38), massive bleeding (n=49), invasive procedure (n=23), Glanzmann thrombasthenia (n=6), congenital factor VII deficiency (n=5) and acquired aplastic anemia (n=1). For controlling excessive bleeding, 40–100 μg/kg of rFVIIa was given at 15–30 min interval for 1–4 doses until the bleeding significantly reduced, followed by 40–100 μg/kg at 2–4 h interval for 24–48 h until the bleeding was completely ceased. For preventing bleeding in invasive procedures, the similar regimen but with a lower fixed dose of 40–100 μg/kg was used. One to two doses were usually used. An exceptional high dose of 200–300 μg/kg was used in patients with Glanzmann thrombasthenia and a lower dose of 20–30 μg/kg at 6 h interval was used in patients with congenital factor VII deficiency.Results: The efficacy of controlling bleeding in hemophiliacs revealed effective (80/90, 88.9%), partially-effective (6/90, 6.7%) and ineffective (4/90, 4.4%) responses while the non-hemophiliacs revealed effective (81/122, 88.5%), partially-effective (14/122, 11.5%) and ineffective (27/122, 22.1%) responses. The case-fatality rates were 0% and 27.2% (31/114) in hemophiliacs and non-hemophiliacs, respectively. Three episodes of thrombosis (3/125, 2.4%) were found 3 children underwent cardiac surgery.Conclusion: rFVIIa has shown an effectiveness in the treatment of bleeding in pediatric patients with hemophiliacs and non-hemophiliacs.