Publications by authors named "Janna M Journeycake"

The impact of residual symptoms following recovery from immune-mediated thrombotic thrombocytopenic purpura (iTTP) on activities of daily living during remission is not routinely discussed or evaluated by hematologists. This study used qualitative methodology to understand 3 issues from the patient's perspective: the most important symptoms during remission, the impact of these symptoms on their daily activities, and the effectiveness of communication with hematologists. Oklahoma and Ohio patients participated in either focus groups or individual interviews.

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We describe how infants and children with hereditary and acquired autoimmune thrombotic thrombocytopenic purpura (TTP) initially present and how they can be promptly diagnosed and effectively managed. These are uncommon disorders that are commonly misdiagnosed and can be rapidly fatal. TTP is caused by a severe deficiency of the plasma protease, A disintegrin and Metalloprotease with a ThromboSpondin type 1 motif, member 13 (ADAMTS13).

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Children with chronic illness often require prolonged or repeated venous access. They remain at high risk for venous catheter-related complications (high-risk patients), which largely derive from elective decisions during catheter insertion and continuing care. These complications result in progressive loss of the venous capital (patent and compliant venous pathways) necessary for delivery of life-preserving therapies.

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Background: Children with osteomyelitis are at risk for deep venous thrombosis (DVT). This study evaluates the characteristics of DVT among children to differentiate between those with and without osteomyelitis.

Methods: Children with DVT of any cause were studied between 2008 and 2016.

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Purpose: Central venous thrombosis (CVT) is a serious complication of long-term central venous access for parenteral nutrition (PN) in children with intestinal failure (IF). We reviewed thse incidence of CVT and possible risk factors.

Methods: Children with IF on home PN (2010-2014) with central venous imaging were reviewed.

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Childhood immune thrombocytopenia (ITP) is often considered a benign hematologic disorder. However, 30% of affected children will have a prolonged course and 5%-10% will develop chronic severe refractory disease. Until recently, the only proven therapeutic option for chronic severe ITP was splenectomy, but newer alternatives are now being studied.

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Age-group analyses were conducted of patients in the prophylactic platelet dose trial (PLADO), which evaluated the relation between platelet dose per transfusion and bleeding. Hospitalized patients with treatment-induced hypoproliferative thrombocytopenia were randomly assigned to 1 of 3 platelet doses: 1.1 × 10(11), 2.

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Background: Neonates and children differ from adults in physiology, pharmacologic responses to drugs, epidemiology, and long-term consequences of thrombosis. This guideline addresses optimal strategies for the management of thrombosis in neonates and children.

Methods: The methods of this guideline follow those described in the Methodology for the Development of Antithrombotic Therapy and Prevention of Thrombosis Guidelines: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines.

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Stroke is common in children with sickle cell anemia, but is rarely attributed to the traditional causes of stroke identified in other children. An 11-year-old girl with sickle cell anemia presented with severe headache and was found to have recurrent bilateral multifocal strokes in a cardioembolic pattern. Evaluation revealed the presence of a patent foramen ovale, antiphospholipid antibodies, and elevations in factor VIII and lipoprotein(a).

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Objective: May-Thurner syndrome is characterized by left common iliac obstruction secondary to compression of the left iliac vein by the right common iliac artery against the fifth-lumbar vertebra. This anatomic variant results in an increased incidence of left-sided deep venous thrombosis (DVT). Furthermore, while a preponderance of left-sided DVT has been demonstrated in women during pregnancy and oral contraceptive use, patients are not typically screened for this condition after developing a left-sided DVT.

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Objective: Subcutaneous administration of enoxaparin is often difficult in special populations, such as premature infants and critically ill children with severe edema. The difficulty achieving adequate anticoagulation in these patients has led to the employment of intravenous enoxaparin in some cases. However, little pharmacodynamic data are available for determining the appropriate dosing and monitoring (by anti-Factor Xa levels) of intravenous enoxaparin.

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Aspirin is used to prevent thromboembolism in children with heart disease without evidence supporting its efficacy. Studies in adults report a 5%-51% prevalence of aspirin resistance, yet the mechanisms involved are poorly understood. Our aims were to determine its prevalence in these children and to explore its possible mechanisms.

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Congenital platelet disorders represent a rare group of diseases classified by either a qualitative or quantitative platelet defect. This article outlines the historical, clinical, laboratory, and genetic features of various inherited platelet disorders with attention given to updated information on disease classification, diagnosis, and genotypes. A separate discussion regarding management addresses the difficulty in treatment strategies, particularly in patients who develop alloimmunization to platelets.

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Objective: To determine the prevalence and clinical features of deep vein thrombosis (DVT) complicating osteomyelitis during childhood.

Study Design: We retrospectively reviewed medical records of all patients with osteomyelitis admitted to Children's Medical Center Dallas between July 1, 2003 and December 31, 2004. Analysis was performed on patients with proximal upper or lower extremity, pelvic or vertebral osteomyelitis (a subgroup considered to be at highest risk for infection-related thrombosis).

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Purpose: Asymptomatic deep vein thrombosis (DVT) is a complication of central venous catheter (CVC) use in children with cancer, but its clinical significance is not well defined. Children with CVCs commonly experience two other CVC-related complications: occlusion and infection. The aim of this study was to determine the frequency of these two complications and their association with DVT.

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Deep vein thrombosis occurs in up to 50% of children with tunneled central venous catheters (CVCs). CVC-related deep vein thrombosis involving the upper extremity is usually asymptomatic but can result in post-thrombotic syndrome (swelling, pain, skin changes, and functional impairment). In a cohort of childhood cancer survivors evaluated clinically a mean of 7.

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Despite underlying illnesses, children have a greater chance to survive and are expected to live 6 to 8 decades following an episode of venous or arterial thrombosis. The disproportionate benefits of preventing thrombosis and its sequelae in pediatric patients are evident. Therefore, it is necessary to develop appropriate strategies for diagnosis and management of thromboembolic events in children and to understand their acute and long-term effects.

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Background: To determine the maximum-tolerated duration and dose-limiting toxicity of a daily schedule of orally administered cyclophosphamide and topotecan in pediatric patients with recurrent or refractory malignant solid tumors.

Methods: Patients received oral cyclophosphamide (50 mg/m2/dose) in the morning followed by topotecan (0.8 mg/m2/dose) 8-12 hr later for an escalating number of consecutive days (10, 14, and 17 days).

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Chronic idiopathic thrombocytopenic purpura (ITP) can be categorized as mild, moderately severe, or severe. Severe chronic ITP during childhood is a rare disorder characterized by clinically significant mucocutaneous hemorrhage, usually in the setting of marked thrombocytopenia. It can cause substantial morbidity and rarely mortality.

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Purpose: To retrospectively review the authors' institutional experience with arthroscopic synovectomy in young patients with hemophilia.

Patients And Methods: Patients with moderate or severe hemophilia seen in the authors' center were eligible to undergo synovectomy if they developed subacute or chronic synovitis that did not improve with prophylactic factor replacement. A single orthopedic surgeon performed all procedures.

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Use of central venous catheters has become standard in the treatment of many chronic conditions during childhood and for the acute treatment of critically ill infants and children. However, these catheters can be associated with numerous complications, including thrombosis at the tip or in the lumen causing difficulty with its overall function. Even more concerning is the occlusion of large veins into which the catheter is placed, which could predispose patients to pulmonary embolism or postthrombotic syndrome.

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