Publications by authors named "Kristy Pahl"

Article Synopsis
  • Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare vascular tumors in children, and this study aimed to identify effective first-line therapies due to the lack of clinical trials.
  • The study included 159 patients, revealing high treatment response rates (>70%) to both sirolimus and vincristine, with no significant differences in effectiveness over time.
  • The findings suggest personalized treatment approaches are essential, as both medications showed similar outcomes, and the low mortality rate (1.3%) indicates improved management of these tumors.
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Background: A rise in hospital-acquired venous thromboembolism (HA-VTE) in children has led to increased awareness regarding VTE prophylaxis and risk assessment. Despite no consensus exists regarding these practices in pediatrics.

Objective: To describe common practices in VTE prophylaxis, VTE risk assessment models, and anticoagulation dosing strategies in pediatric hospitals that are members of the Children's Hospital Acquired Thrombosis (CHAT) Consortium.

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Vascular anomalies are a group of disorders divided into two distinct subtypes: vascular tumors and vascular malformations. Vascular tumors are proliferative in nature, while malformations are nonproliferative. Simple, localized vascular malformations refer to a group of malformations that are localized to a single area of involvement.

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Infantile hemangiomas (IHs) are common vascular lesions which are benign but can cause significant functional and cosmetic morbidity. Since the fortuitous discovery of propranolol being effective to treat IH over a decade ago, the therapy and prognosis for children with IH have improved dramatically. Oral propranolol (as well as other oral beta-blockers and topical timolol) are safe and effective treatments, and have now supplanted other therapies.

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Given the limited information on the coagulation abnormalities of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in pediatric patients, we designed a systematic review to evaluate this topic. A comprehensive literature search was conducted for "SARS-CoV-2," "coagulopathy," and "pediatrics." Two authors independently screened the articles that the search returned for bleeding, thrombosis, anticoagulant and/or antiplatelet usage, and abnormal laboratory markers in pediatric patients with SARS-CoV-2, and the authors then extracted the relevant data.

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Background: Thrombosis in the neonatal population is rare, but increasing. Its incidence and management are not well understood.

Objectives: To investigate the incidence, associated factors, and management of thrombosis in the neonatal intensive care unit (NICU) population.

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Notch expression has been shown to be aberrant in brain arteriovenous malformations (AVM), and targeting Notch has been suggested as an approach to their treatment. It is unclear whether extracranial vascular malformations follow the same patterning and Notch pathway defects. In this study, we examined human extracranial venous (VM) (n = 3), lymphatic (LM) (n = 10), and AV (n = 6) malformations, as well as sporadic brain AVMs (n = 3).

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Objective: To evaluate the accuracy of the clinical Curaçao criteria in the diagnosis of hereditary hemorrhagic telangiectasia (HHT) in children and adolescents.

Study Design: This was a retrospective, multicenter chart review of 673 patients evaluated between 2002 and 2016; 290 were eligible for the study. Genetic testing for a pathogenic mutation was considered the gold standard against which the clinical Curaçao criteria were compared.

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Background: Vascular anomalies are a heterogeneous group of disorders seen in children and adults. A standard nomenclature for classification has been offered by the International Society for the Study of Vascular Anomalies. Its application is important for communication among the multiple specialties involved in the care of patients and for planning treatment, as well as for research and billing.

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Sickle cell disease is a severe hemoglobinopathy caused by mutations in the beta globin genes. The disorder has protean manifestations and leads to severe morbidity and early mortality. Acute chest syndrome (ACS) is a common complication and in the USA is the leading cause of death in patients with sickle cell disease.

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Background: RUNX1 (AML1) amplification in patients with B-cell acute lymphoblastic leukemia (B-ALL) has been associated with poor survival for unclear reasons. Our anecdotal experience suggests that children with B-ALL and RUNX1 amplification might be predisposed to thrombosis.

Procedure: We performed a retrospective cohort study of children with B-ALL treated from 2008 to 2014 at the North Carolina Children's Hospital.

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