Use of recombinant factor VIIa prior to lumbar puncture in pediatric patients with acute leukemia

The persistence of abnormal coagulation test results after standard treatment with fresh frozen plasma (FFP) poses significant problems in children with acute leukemia requiring a diagnostic lumbar puncture and intrathecal chemotherapy. We report the prophylactic use of a single dose of 90 microg/kg recombinant activated factor VII (rFVIIa) in three children and the rapid correction of abnormal coagulation test results previously not corrected by FFP. Administration of rFVIIa was useful in avoiding a delay of diagnostic lumbar punctures and intrathecal chemotherapy. Hemorrhagic complications and adverse effects of rFVIIa were not observed. Prospective evaluation of this indication and dose appears warranted. (c) 2005 Wiley-Liss, Inc.     

Use of recombinant factor VIIa (rFVIIa) to control intraoperative bleeding in pediatric brain tumor patients

Surgical bleeding during the resection of brain tumors in children may be related to tumor vascularity, pathology, and location. Despite improvements in neurosurgical technique, neuroanesthesia, and blood product replacement, bleeding can be life-threatening in these surgeries. We report eight pediatric patients in whom recombinant factor VIIa (rFVIIa) was used to control intraoperative bleeding during surgical resection of pediatric brain tumors. rFVIIa should be considered as a method to control intraoperative bleeding that is unresponsive to conventional interventions. Additional studies are needed to determine optimal patient selection and drug dosing, efficacy and safety.     

Use of recombinant factor VIIa for bleeding in children with Glanzmann thrombasthenia

Glanzmann thrombasthenia is a very rare inherited platelet function disorder in which bleeding may be extremely difficult to stop. Recombinant factor VIIa is one of the alternative treatments for bleeding. The authors report here their experience with the use of factor VIIa, which may be useful for arresting bleeding in Glazmann thrombasthenia.     

Use of recombinant factor VIIa for refractory hemorrhage during extracorporeal membrane oxygenation.

OBJECTIVE: To describe the outcome and treatment of two patients with recombinant factor VIIa (rFVIIa) for severe hemorrhage associated with extracorporeal membrane oxygenation (ECMO). DESIGN: Case report. SETTING: A 38-bed pediatric intensive care unit and 20-bed pediatric cardiac intensive care unit at a tertiary care children's hospital. Patient: Two patients with life-threatening hemorrhagic complications associated with ECMO requiring massive transfusion of blood products. INTERVENTIONS: Administration of repeated doses of rFVIIa at 90 microg/kg/dose. MEASUREMENT AND MAIN RESULTS: Patient 1 was an 11-yr-old male with a dilated cardiomyopathy who had undergone an orthotopic heart transplant treated with venoarterial ECMO postoperatively for right ventricular dysfunction. Patient 2 was a 13-yr-old male treated with venoarterial ECMO for cardiopulmonary failure from necrotizing staphylococcal pneumonia. Both patients had severe hemorrhage from the cannulation sites and thoracostomy tubes requiring massive transfusion to maintain intravascular blood volume and replace clotting factors. Both patients were treated with rFVIIa every 2-4 hrs and attained hemostasis. Patient 1 was administered three doses and Patient 2 was administered ten doses. No evidence of abnormal thrombus formation was noted in their respective ECMO circuits. CONCLUSIONS: The efficacy of rFVIIa in reducing intractable bleeding postcardiac surgery and in other coagulopathic states is being investigated. Despite theoretical concerns of thrombosis, these cases illustrate that there may be a role for the cautious use of rFVIIa in treating severe and intractable hemorrhage associated with ECMO.     

Successful use of recombinant factor VIIa for management of severe menorrhagia in an adolescent with an acquired inhibitor of human thrombin

Antibodies directed against human thrombin are exceedingly rare, having only been reported in adult patients with underlying diseases. Consensus on the most appropriate management has not yet been reached. A 12-y-old girl presented with intractable menorrhagia several days after an acute infectious episode. Laboratory tests revealed disturbed clotting tests: prothrombin index 17%, activated partial thromboplastin time >150 s, thrombin time >120 s, and failure to achieve correction with a normal pooled plasma. Further studies demonstrated the presence of an antibody directed against human thrombin. Viral serology revealed a 1/128 titre for adenovirus. Massive haemorrhage was unresponsive to standard treatments, but intravenous administration of recombinant factor VIIa resulted in a successful outcome. Conclusion: This is the first report of an anti-human thrombin antibody associated with severe bleeding in a child. Recombinant factor VIIa could represent a novel therapeutic approach for such patients.     

Spontaneous liver hemorrhage during laparotomy for necrotizing enterocolitis: a potential role for recombinant factor VIIa

Necrotizing enterocolitis remains a serious condition in very low birth weight infants, particularly in those infants who require surgery. Perioperative hemorrhage is a potentially fatal complication in this population. We describe our experience in 4 premature infants with necrotizing enterocolitis who received recombinant factor VIIa to manage life-threatening intraoperative hemorrhage.     

Novel applications of ultrasound in pediatric emergency medicine

A new field, termed emergency ultrasound (EUS), has recently been established. The past decade saw rapid development in the field of EUS in adult patients, especially as performed by emergency medicine physicians. Ultrasound imaging offers several advantages over traditional radiographic techniques, many of which are especially relevant to patients in the pediatric emergency department. Recent literature has documented increased use of EUS for pediatric patients. This review will examine basic principles of ultrasound relevant to pediatric emergency medicine physicians. Emphasis will be placed on understanding the instrument and its limitations. In addition, we will review recent developments in this field. It is our goal that the reader will gain an understanding of the strengths and limitations of this instrument and will therefore be in a position to plan their own program in EUS in pediatrics. Furthermore, it is hoped that this review will serve as an impetus for innovative research, to refine and extend the indications of this modality to benefit patients in the pediatric emergency department.     

Management of coagulopathy with recombinant factor VIIa in a neonate with echovirus type 7

A 5-day-old newborn presented with neonatal enteroviral infection. The patient's hospital course was complicated by acute liver dysfunction, renal insufficiency, fluid overload, respiratory failure, hypertension, catheter related thrombosis, Klebsiella pneumoniae sepsis, intracerebral and intraventricular hemorrhage, and disseminated intravascular coagulation (DIC). Administration of fresh frozen plasma (FFP) and cryoprecipitate failed to control the patient's hemostasis and led to significant fluid overload. Recombinant activated factor VII (rFVIIa, Novoseven NovoNordisk, Bagsvaerd, Denmark) was given to the neonate as a bolus (rFVIIa at 60-80 microg/kg body weight), followed by a continuous infusion (2.5-16 microg/kg/hr). Recombinant activated factor VII controlled hemostasis, until the patient's liver function recovered. The patient's blood product requirement significantly decreased and his fluid overload resolved. Administration of rFVIIa appears to have stabilized the coagulation process. The patient appears to have fully recovered from the infection's complications.     

Single-center experience: use of recombinant factor VIIa for acute life-threatening bleeding in children without congenital hemorrhagic disorder

Coagulopathy is an important cause of mortality in critically ill children. Traditional therapies to correct coagulopathy lead to great time delays and cause fluid overload in patients. The authors report the effectiveness and safety of the activated recombinant factor VII (rFVIIa) administration in a series of 13 nonhemophiliac children with acute, life-threatening bleeding. In this retrospective study, the records of the patients who were not diagnosed with congenital hemorrhagic disorder and were administered rFVIIa due to any other reason in Ege University Faculty of Medicine, Department of Pediatrics, between February 2002 and February 2007 were reviewed retrospectively. Thirteen nonhemophiliac patients with acute life-threatening bleeding and ages ranging from 2 days to 15 years received rFVIIa over a 5-year period. Three patients were diagnosed with hemaphagocytic lymphohistiocytosis, 4 with prematurity, sepsis, and disseminated intravascular coagulation (DIC), 5 with sepsis, multiple organ dysfunction syndrome, and DIC, and 1 with acute liver failure. Severe bleeding resulted from pulmonary (n = 3), lower gastrointestinal system (n = 2), esophagus varices (n = 1), pulmonary and gastrointestinal system (n = 4), pulmonary, gastrointestinal system, and intracranial hemorrhage (n = 1), and gastrointestinal system and intracranial hemorrhage (n = 2). Median frequency of rFVIIa administration was 3 per patient (range 2-15) and median dose of rFVIIa was 90 microg/kg, ranging from 60 to 135 microg/kg each administration. All of the patients were given fresh frozen plasma and if necessary platelet transfusion (n = 10) or fibrinogen concentrate (n = 3) before administration of rFVIIa. In 6 patients, lack of success to control bleeding by conventional methods was the only cause to start rFVIIa. In 7 patients, the need for volume restriction was also a significant contributing factor in deciding to start rFVIIa. Median PT was 32.9 s (range: 19-65) before rFVIIa administration and it was decreased to 11.6 s (range: 10.7-12.8), 2-3 h after rFVIIa infusion. Bleeding was stopped completely in 10 patients at least for 24 h and decreased in 3 patients 30-45 min after rFVIIa administration. Two patients had thrombotic complications attributed to rFVIIa administration. No other complication was observed in the other patients. In this retrospective study, rFVIIa was found to be effective at controlling severe hemorrhagic symptoms of different etiologies in children without congenital hemorrhagic disorder. In addition to the rapid control of bleeding, administration of this agent improved fluid balance and led to a reduction in blood product requirements in critically ill children. However, survival was still poor (23%), and 2/13 (15.4%) patients developed venous and arterial thrombosis within 3 h of treatment. The authors emphasize that in acquired, acute life-threatening bleeding, simultaneous administration of rFVIIa with conventional treatment may contribute to patient survival. However, the risk of thromboembolism should be considered before this treatment is given.     

The use of recombinant factor VIIa in a patient with Noonan syndrome and life-threatening bleeding.

OBJECTIVE: To present a case report of a patient with Noonan syndrome who developed life-threatening gastrointestinal bleeding shortly after cardiac surgery that was successfully treated with recombinant factor VIIa. DESIGN: Case report. SETTING: Pediatric intensive care unit of a children's hospital. PATIENT: Ten-month-old with Noonan syndrome and massive gastrointestinal bleeding resulting in severe hypovolemic shock. INTERVENTIONS: Recombinant factor VIIa was used in this patient's severe bleeding associated with Noonan syndrome after no other supportive measures were successful. MEASUREMENTS AND MAIN RESULTS: Recombinant Factor VIIa significantly decreased the patient's bleeding and allowed his hypovolemic shock to improve. Ultimately, the patient made a complete recovery. CONCLUSIONS: Noonan syndrome has a constellation of both cardiac and noncardiac malformations including an increased risk of bleeding, and recombinant factor VIIa is an important agent in the treatment of significant bleeding.     

小儿反流性疾病动力学改变及其治疗

小儿反流性疾病主要指胃内容反流入食管 ,称为胃食管反流 (ＧＥＲ) ;十二指肠内容反流入胃 ,称为十二指肠胃反流 (ＤＧＲ)。在ＤＧＲ的基础上 ,胃十二指肠内容反流入食管称为十二指肠胃食管反流 (ＤＧＥＲ) ,又称碱性胃食管反流 (ＡＧＥＲ)。1　ＧＥＲ1 .1　传统观念认为ＧＥＲ是由食管远端功能障碍引起的胃内容反流入食管。目前认为这一概念并不全面 ,ＧＥＲ可由多种因素引起 ,其中包括抗反流机制下降和反流物中攻击因子 (如氢离子 )增强。小儿ＧＥＲ较成人常见 ,但绝大多数为生理性反流 ,60 %在生后 1 2～ 1 8个月内呕吐症状消失 ,30 %持…     

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??Pediatric functional cardiovascular diseases involve a series of dysfunctions which can affect children’s physical and mental health. They include vasovagal syncope??postural tachycardia syndrome??orthostatic hypotension??orthostatic hypertension and beta-adrenoceptor hyperfunction??etc. Since the above-mentioned diseases have various therapeutic response??with the research progress of the disease mechanisms and prognosis??great progress has been made in individualized management of pediatric functional cardiovascular diseases. In the future??great attention should be paid to the individualized diagnosis and treatment of the diseases so as to improve the diagnostic and therapeutic technology.     

The assessment and management of the respiratory complications of pediatric neuromuscular diseases

Respiratory complications are a major cause of morbidity and mortality in the pediatric neuromuscular diseases. Weakness of the muscles of respiration results in shallow breathing and ineffective cough, making patients vulnerable to atelectasis, pneumonia, and tracheal obstruction by retained respiratory tract secretions. Assessment of the risk of respiratory complications includes evaluating the patient's history and respiratory physical examination and measuring pulmonary function and gas exchange. Treatment options include methods of assisted cough and mechanical ventilation. This review emphasizes the use of noninvasive respiratory aids to optimize duration and quality of life in these potentially fatal diseases.