Infantile Myofibromatosis With Cutaneous, Visceral, and CNS Involvement: A Multimodal Approach to Therapy.

BACKGROUND: Infantile myofibromatosis (IM) is a rare benign tumor of infancy. Cases with solitary and multicentric disease usually spontaneously regress, but multicentric disease with visceral involvement carries a poor prognosis. Few cases of multicentric disease with central nervous system (CNS) involvement have been reported, and none report survival. OBSERVATIONS: We present a newborn with multicentric IM with cutaneous, visceral, and CNS involvement. She was treated with vinblastine, methotrexate, and the novel addition of intrathecal methotrexate with treatment response after 1 year of therapy. CONCLUSIONS: Multicentric IM with CNS involvement can be successfully treated with a multimodal approach of chemotherapy with the addition of intrathecal methotrexate and surgery.

[Seroprevalence of Leptospira spp. in draft horses from indigenous communities in the Araucanía Region, Chile]. / Seroprevalencia de Leptospira spp. en equinos de tiro de comunidades indígenas mapuche de la Región de la Araucanía Chile.

In the Araucanía Region there are no studies on the seroprevalence of leptospirosis in horses. Serological samples from 100 draft horses from Mapuche communities of four communes in the region were analyzed using the microMAT technique. The seroprevalence was 35% for at least one serovar of Leptospira spp. being the most frequent serovars canicola (22%), grippotyphosa (21%), hardjo (13%) and pomona (10%). It was evident that the equine populations studied are exposed to infection by Leptospira spp. and reveal a potential risk of transmission to their owners.

Seroprevalencia de Leptospira spp. en equinos de tiro de comunidades indígenas mapuche de la Región de la Araucanía Chile / Seroprevalence of Leptospira spp. in draft horses from indigenous communities in the Araucanía Region, Chile

Resumen En la Región de la Araucanía no existen estudios sobre seroprevalencia de leptospirosis en equinos. Se analizaron muestras serológicas provenientes de 100 equinos de tiro de comunidades mapuche de cuatro comunas de la región mediante la técnica de microMAT. La seroprevalencia fue de 35% para al menos un serovar de Leptospira spp., siendo los serovares más frecuentes canicola (22%), grippotyphosa (21%), hardjo (13%) y pomona (10%). Se evidenció que las poblaciones equinas estudiadas están expuestas a la infección por Leptospira spp. y revelan un riesgo potencial de transmisión a sus propietarios.

Abstract In the Araucanía Region there are no studies on the seroprevalence of leptospirosis in horses. Serological samples from 100 draft horses from Mapuche communities of four communes in the region were analyzed using the microMAT technique. The seroprevalence was 35% for at least one serovar of Leptospira spp. being the most frequent serovars canicola (22%), grippotyphosa (21%), hardjo (13%) and pomona (10%). It was evident that the equine populations studied are exposed to infection by Leptospira spp. and reveal a potential risk of transmission to their owners.

Genetic markers for treatment-related pancreatitis in a cohort of Hispanic children with acute lymphoblastic leukemia.

PURPOSE: Treatment-related pancreatitis (TRP) is a serious complication occurring in children with acute lymphoblastic leukemia (ALL). Those affected are at high risk for severe organ toxicity and treatment delays that can impact outcomes. TRP is associated with asparaginase, a standard therapeutic agent in childhood ALL. Native American ancestry, older age, high-risk leukemia, and increased use of asparaginase are linked to pancreatitis risk. However, dedicated genetic studies evaluating pancreatitis in childhood ALL include few Hispanics. Thus, the genetic basis for higher risk of pancreatitis among Hispanic children with ALL remains unknown. METHODS: Cases of children with ALL treated in from 1994 through 2013 were reviewed and identified 14, all Hispanic, who developed pancreatitis related to asparaginase therapy. Forty-six controls consisting of Hispanic children treated on the same regimens without pancreatitis were selected for comparison. Total DNA isolated from whole blood was used for targeted DNA sequencing of 23 selected genes, including genes associated with pancreatitis without ALL and genes involved in asparagine metabolism. RESULTS: Non-synonymous polymorphisms and frameshift deletions were detected in 15 genes. Most children with TRP had variants in ABAT, ASNS, and CFTR. Notably, children with TRP harbored many more CFTR variants (71.4%) compared with controls (39.1%). Among these, V470M (rs213950) was most frequent (OR 4.27, p = 0.025). CONCLUSIONS: This is the first study of genetic factors in treatment-related pancreatitis in Hispanic children with ALL. Identifying correlative variants in ethnically vulnerable populations may improve screening to identify which patients with ALL are at greatest risk for pancreatitis.

Comparación entre los scores de Ripasa y Alvarado modificado en el diagnóstico de apendicitis aguda, 2015-2016 / Comparison between the scores of Ripasa and Alvarado modifieed in the diagnosis of acute appendicitis, 2015-2016

Objetivo: Determinar que score tiene mayor sensibilidad y especificidad entre el de RIPASA y el de ALVARADO modificado para corroborar el diagnóstico de Apendicitis Aguda en pacientes de 16 a 60 años de edad, Hospital Regional de Ica del 1 de octubre del 2015 al 30 de setiembre del 2016. Material y Metodos: Se realizó un estudio descriptivo, transversal y retrospectivo de 150 historias, se apoyo con los programas estadístico SPSS V 22.0; Microsoft Word 2013 y Microsoft Excel. Resultados: La sensibilidad, especificidad, VPP y VPN del score de RIPASA fue 88,7%, 77,8%, 98,4% y 30,4% respectivamente (para un score ≥ 7,5) y para el score de Alvarado fue 71,6%, 77,8%, 98,1% y 14,9% respectivamente (para un score ≥ 7), El área bajo la curva ROC del score de RIPASA fue de 0.848, superior a la de Alvarado de 0.81.Conclusiones: La efectividad diagnóstica del score de RIPASA fue 84.8% y del score de Alvarado Modificado de 81%, demostrando mayor exactitud como prueba diagnóstica. (AU)

Objetive: To determine which score has greater sensitivity and specificity between RIPASA and modified ALVARADO to corroborate the diagnosis of acute appendicitis in patients 16 to 60 years of age, Regional Hospital of Ica from October 1, 2015 to September 30 2016. Material And Methods: We carried out a descriptive, transversal and retrospective study of 150 histories, supported with the statistical programs SPSS V 22.0; Results: The sensitivity, specificity, VPP and NPV of the RIPASA score were 88.7%, 77.8%, 98.4% and 30.4%, respectively (for a score ≥ 7.5) And the Alvarado score was 71.6%, 77.8%, 98.1% and 14.9%, respectively (for a score ≥ 7). The area under the ROC curve of the RIPASA score was 0.848, higher To that of Alvarado of 0.81.Conclusions: The diagnostic effectiveness of the RIPASA score was 84.8% and the modified Alvarado score was 81%, demonstrating greater accuracy as a diagnostic test. (AU)

All-trans-retinoic acid and arsenic trioxide as initial therapy for acute promyelocytic leukemia.

Acute promyelocytic leukemia (APL) is a rare subtype of acute myeloid leukemia (AML). Treatment of pediatric APL is based on the combination of all-trans-retinoic acid (ATRA), an anthracycline and cytosine arabinoside. Arsenic trioxide (ATO) has been studied in adults with newly diagnosed or relapsed APL with excellent response rates both when used as a single agent or in combination with ATRA or ATRA plus chemotherapy. There is little data on combination therapy with ATRA and ATO in pediatric APL. We present a case of an adolescent male with APL who was treated using ATRA and ATO without conventional chemotherapy agents.

Intranasal granulocyte-macrophage colony-stimulating factor reduces the Aspergillus burden in an immunosuppressed murine model of pulmonary aspergillosis.

We demonstrated that intranasal granulocyte-macrophage colony-stimulating factor given to immunosuppressed mice infected with pulmonary aspergillosis resulted in a sixfold reduction in the lung fungal burden compared to the result for saline-treated mice (P = 0.045). These data suggest that lung-targeted immunotherapy may be complementary to antifungal agents and may improve patient responses.

Medical and non-medical barriers to outpatient treatment of fever and neutropenia in children with cancer.

BACKGROUND: A number of clinical trials have employed clinical criteria that can identify pediatric patients at low-risk for complicated episodes of fever and neutropenia (F&N) and have successfully treated low-risk patients in the outpatient setting. Despite this, inpatient management remains the standard of care. This trial tested the hypothesis that a strategy of initial hospitalization followed by continuation of therapy in the outpatient setting could be practically implemented in the majority of episodes. PROCEDURE: Patients presenting with F&N were initially evaluated to determine if they had high-risk clinical criteria that would exclude them from this approach. Eligible patients were then hospitalized and treated with iv antibiotics. On subsequent days the attending physician determined whether the patient had exhibited improvement and could continue therapy in the outpatient setting with oral antibiotics. Outpatients were seen three times weekly and continued antibiotics until recovery from F&N. RESULTS: Outpatient oral antibiotic therapy was practically implemented in less than one-quarter of episodes of pediatric F&N. Forty-nine percent of episodes were excluded from study by medical and social protocol exclusion criteria. One hundred five episodes were enrolled and among these 59 episodes included outpatient management. Common barriers to outpatient care included serious medical comorbidities, non-medical barriers including language and distance of residence from the medical center, and lack of interest. The average duration of outpatient care was 3.6 days following an average of 3.5 days of hospitalization. Ninety percent did not require rehospitalization. They experienced no complications. CONCLUSIONS: In only a minority of episodes can outpatient antibiotic management be implemented. Medical comorbidities and social barriers can make the transition to outpatient care difficult. However, initial hospitalization followed by oral antibiotic outpatient management appears safe and effective for low-risk patients who exhibit good responses to initial antibiotic therapy in hospital.