Manejo de hemangioma ulcerado segmentario: combinación de tratamiento sistémico y tópico en dos casos pediátricos / Management of ulcers on a segmental hemangioma: A combination of systemic and topical therapies in two pediatric cases

Los hemangiomas infantiles (HI) son los tumores benignos más frecuentes de la infancia; la variante segmentaria es rara y se asocia con un mal pronóstico. Una de sus principales complicaciones es la ulceración durante la fase de crecimiento del tumor, a pesar de no presentar características macroscópicas compatibles con una lesión agresiva. El manejo en estos casos es dificultoso e impone la necesidad de asociar múltiples estrategias, algunas orientadas específicamente a impedir la proliferación del hemangioma y otras orientadas a la curación de la herida, el manejo del dolor y la prevención de la infección agregada. Presentamos dos casos a fin de comunicar nuestra experiencia respecto del manejo de dicha patología y su evolución final.

Infantile hemangiomas (IHs) are the most common benign tumors of childhood, and segmental ones are rare and associated with a poor prognosis. While these tumors look harmless, one of their main related complications is ulceration during tumor growth. The management in these cases is extremely challenging, requiring a combination of multiple approaches, some specifically aimed at preventing the proliferation of the hemangioma and others aimed at wound care, pain management, and prevention of further infection. Here we discuss two cases to narrate our experience on the management of this condition and its outcome.

Infantile hemangiomas: risk factors for complications, recurrence and unaesthetic sequelae

Abstract Background: Infantile hemangiomas (IH) occur in approximately 4% to 10% of the pediatric population. The identification of clinical subtypes and conditions that indicate increased risk for complications is essential for therapeutic success. Objectives: To identify risk factors for complications, recurrence and unaesthetic sequelae. Methods: Retrospective cohort of patients with infantile hemangiomas undergoing follow-up at the Dermatology Service of Universidade Federal de Ciências da Saúde de Porto Alegre, between 2006 and 2018. Results: 190 patients were included; 24% had some type of complication, ulceration being the most frequent, and 86% required treatment. On correlation, ulceration was statistically related to mixed IH (p = 0.004), segmental IH (p < 0.01) and location in the gluteal region (p = 0.001). The mean time of treatment with propranolol was 12.7 months. Patients with PHACES syndrome and segmental infantile hemangioma required longer treatment (p < 0.001 and p = 0.0407, respectively), as well as those who started treatment after five months of life (p < 0.0001). Recurrence occurred in 16.6% of the treated patients, all-female; 94% were located on the head and neck (mainly on the upper eyelid, cyrano, S3 segment, and with parotid involvement); 61% and 38.8% were of the mixed and deep subtypes, respectively. Approximately 1/3 of the patients had some unaesthetic sequelae. Study limitations: As this is a retrospective study, data and photos of some patients were lost. Conclusions: Mixed and segmental hemangiomas are risk factors for ulceration and sequelae. Recurrence occurs more often in females and segmental hemangiomas. Segmental infantile hemangioma and PHACES syndrome require a longer time of treatment. Specific protocols are required for infantile hemangiomas with a high risk of recurrence.

Una década de atención al paciente con miocardiopatía hipertrófica / A decade of care to patients with hypertrophic cardiomyopathy

Introducción: La miocardiopatía hipertrófica es una enfermedad derivada de una alteración genética autosómica dominante que produce un aumento de la masa del ventrículo izquierdo, obstructiva o no. Es la principal causa de muerte súbita en adultos jóvenes. Objetivo: Mostrar la prevalencia de la miocardiopatía hipertrófica en la práctica del Cardiocentro Pediátrico "William Soler" y sus formas de presentación. Métodos: Estudio observacional longitudinal retrospectivo de serie de casos. Se resumieron variables demográficas y clínicas en los ingresos realizados durante 10 años de los pacientes con diagnóstico de miocardiopatía hipertrófica y se analizaron según correspondieran con pruebas para variables cualitativas y cuantitativas. Resultados: Se identificaron 21 pacientes; 12 con características obstructivas y 9 no obstructivas. No hubo predominio de sexo. La media de edad de diagnóstico y de debut clínico de los pacientes con enfermedad obstructiva fue significativamente menor que las edades de los pacientes sin obstrucción del tracto de salida izquierdo. El diagnóstico fue posible en más de 50 por ciento de los casos por sospecha por soplo o por pesquisa. El tratamiento quirúrgico permitió la reducción significativa del gradiente en el tracto de salida izquierdo. El propranolol fue el betabloqueador más usado acorde a consensos internacionales. No hubo fallecidos en la serie estudiada. Conclusiones: La miocardiopatía hipertrófica tiene una baja prevalencia en la práctica cardiopediátrica. Los síntomas tempranos se corresponden con la variedad obstructiva. Su diagnóstico temprano y el tratamiento específico, permite garantizar mejor calidad y expectativa de vida a los portadores de esta afección(AU)

Introduction: Hypertrophic cardiomyopathy is a disease derived from autosomal dominant genetic alteration that causes an increase in the mass of the left ventricle, and can be obstructive or not. It is the leading cause of sudden death in young adults. Objective: Show the prevalence of hypertrophic cardiomyopathy and its forms of presentation in the practice of the "William Soler" Pediatric Cardiocenter. Methods: Retrospective, observational, longitudinal study of a case series. Demographic and clinical variables were summarized in the admissions made during 10 years of patients diagnosed with hypertrophic cardiomyopathy whom were analyzed as appropriate with qualitative and quantitative variable testing. Results: 21 patients were identified; 12 with obstructive characteristics and 9 with non-obstructive ones. There was no predominance of sex. The average diagnostic age and clinical onset of patients with obstructive disease was significantly lower than the ages of patients without obstruction of the left outflow tract. Diagnosis was possible in more than 50 percent of cases by suspicion due to a murmur or by investigation. Surgical treatment allowed a significant reduction in the gradient of the left outflow tract. Propranolol was the most widely used beta-blocker according to international consensus. There were no deaths in the series studied. Conclusions: Hypertrophic cardiomyopathy has a low prevalence in cardiopediatric practice. Early symptoms correspond to the obstructive variety. Early diagnosis and specific treatment ensure better quality and life expectancy for carriers of this condition(AU)

Linfangiomatosis pulmonar difusa con compromiso pleural y pericárdico. Reporte de un caso pediátrico / Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report

La linfangiomatosis pulmonar difusa es una enfermedad rara caracterizada por una marcada proliferación y dilatación de los vasos linfáticos en los pulmones, la pleura y el mediastino. Se desconoce la prevalencia, y la etiología no se comprende completamente.Una niña de 22 meses ingresó por poliserositis, con derrame pericárdico y pleural. Requirió pericardiocentesis y avenamiento pleural, y presentó drenaje de quilo (1,5-4 litros/día) sin respuesta al tratamiento médico (ayuno, nutrición parenteral y octreotide). Se realizó biopsia pulmonar. La anatomía patológica mostró hallazgos compatibles con linfangiomatosis difusa pulmonar. Comenzó tratamiento con sirolimus y propanolol, que disminuyeron las pérdidas por el drenaje pleural a la semana. Presentó buena evolución; suspendió aporte de oxígeno y se retiró el drenaje pleural. Se externó al cuarto mes de internación. El diagnóstico temprano de la linfangiomatosis pulmonar difusa es difícil de lograr, pero permite aplicar terapéuticas que evitan la progresión de enfermedad y disminuir la morbimortalida

Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood.A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization.Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality.

Tratamiento con propranolol en el síndrome de Kasabach-Merritt secundario a hemangioma hepático congénito. Caso clínico / Propranolol treatment in Kasabach-Merritt Syndrome secondary to congenital hepatic hemangioma. Clinical case

El hemangioma hepático es el tumor benigno de hígado más frecuente. Puede ser congénito o infantil, con diferentes evoluciones y complicaciones. La evolución clínica es muy variable, desde pacientes asintomáticos hasta cuadros de gravedad con insuficiencia cardíaca, síndrome de Kasabach-Merritt o síndrome compartimental. El diagnóstico se basa en la historia clínica y los estudios por imágenes, especialmente, la ecografía y el examen doppler en manos experimentadas. Resulta fundamental el diagnóstico diferencial con otras lesiones hepáticas, sobre todo, el hepatoblastoma. En los pacientes sintomáticos, el propranolol surge como primera línea terapéutica con buenos resultados y baja frecuencia de efectos adversos. Se presenta el caso de un recién nacido con hemangioma hepático asociado a síndrome de Kasabach-Merritt, con excelente respuesta y tolerancia al propranolol

Hepatic hemangioma is the most common benign liver tumor. It can be congenital or infantile with different outcomes and complications. The clinical manifestation varies from asymptomatic to severe conditions with heart failure, Kasabach-Merritt syndrome or compartment syndrome. Diagnosis depends on medical history and imaging studies, especially ultrasound and Doppler examination in experienced hands. Differential diagnosis is essential with other hepatic lesions, mainly hepatoblastoma. In symptomatic patients, propranolol emerges as the first line treatment with good results and low frequency of adverse effects. We present the case of a newborn with a hepatic hemangioma and Kasabach-Merritt syndrome with an excellent response and tolerance to propranolol.

Evaluation of Depressive and Anxious Behavior with the Use of Propranolol in Melanoma-Bearing Mice

HIGHLIGHTS: Tumor progression and anxiety and depression behaviors under evaluation during propranolol use in murine melanoma. Evaluation of anxiety and depression through forced swimming behavior tests, elevated plus maze, open field and marble-burying test.

Abstract Melanoma, a severe form of skin cancer, has rapid growth and has been prone to behavioral disorders that worsen the patient's prognosis and survival. Among these psychic disorders can occur anxiety and depression, in addition to cognitive deficit. In order to try to elucidate the neuropsychological disorders that occur in melanoma, the objective of this study was to evaluate propranolol in tumor progression and in anxious and depressive behaviors in an animal model with melanoma. B16F10 cells were injected into C57BL6/J mice subsequently treated with propranolol at doses of 1.43 mg/kg and 5.71 mg/kg and evaluated for tumor growth and in open field, forced swimming, elevated plus maze and marble-burying test at initial time and consolidated tumor. As a result, the group treated with propranolol at a dose of 5.71 mg/kg showed less tumor growth. In the initial behavioral tests, melanoma altered the animals' motility, but anxious behavior was not detected. Depressive behavior was detected in the forced swimming test in the two doses of the treatment used. When taking time with consolidated tumor, there was a reduction in the locomotor activity of the animals in the open field test, impairing the analysis of anxious and depressive behavior. The data suggest that there was a reduction in the progression of melanoma, there was no anxious behavior in the animals, only the depressive behavior and the use of propranolol did not improve the evaluated behavior.

Tirotoxicosis por enfermedad trofoblástica gestacional: revisión a partir de 3 casos / Thyrotoxicosis due to gestational trophoblastic disease: clinical cases

La enfermedad trofoblástica gestacional (ETG) es una complicación del embarazo poco común. Corresponde a un espectro de lesiones proliferativas del tejido trofoblástico: Mola Hidatiforme (MH) en sus formas parcial y completa, Coriocarcinoma, Tumor Trofoblástico y Tumor Trofoblástico Epiteloide. Los distintos tipos de ETG presentan en común la hipersecreción de gonadotrofina coriónica humana (hCG). La hCG es una hormona glicoproteica con una estructura muy similar a la TSH, por lo cual puede estimular la función tiroidea en condiciones fisiológicas y en algunas condiciones patológicas. La ETG puede cursar con hipertiroidismo, el cual puede variar en intensidad, desde una presentación asintomática con alteración leve de hormonas tiroideas a un cuadro de hipertiroidismo manifiesto. Se presentan 3 casos clínicos de pacientes con ETG, específicamente MH que evolucionaron con tirotoxicosis transitoria. Los casos presentaron un cuadro leve de hipertiroidismo con pocos síntomas asociados. La taquicardia fue el único síntoma en la mayoría de los casos. En todas las pacientes las hormonas tiroideas se normalizaron después del tratamiento de la ETG. Conclusión: Se debe tener presente la posibilidad de hipertiroidismo en toda paciente con ETG. Un alto nivel de sospecha permitirá identificar a aquellas pacientes que cursen con hipertiroidismo, permitiendo así un diagnóstico y tratamiento oportuno.

Gestational trophoblastic disease (GTD) is a rare complication of pregnancy. GTD includes a group of proliferative lesions of trophoblastic tissue: partial and complete hydatidiform mole, choriocarcinoma, epithelioid trophoblastic tumor, and placental site trophoblastic tumor. The different types of GTD have in common the hypersecretion of human chorionic gonadotropin (hCG). HCG is a glycoprotein hormone with a similar structure to TSH. In physiological and pathological conditions hCG can stimulate thyroid function. GTD can present with hyperthyroidism, which can vary in intensity, from an asymptomatic presentation with mild alteration of thyroid hormones to a manifest hyperthyroidism. We present 3 clinical cases of patients with GTD thyrotoxicosis. All cases presented mild hyperthyroidism. Tachycardia was the only symptom in most cases. In all patients thyroid hormones return to normal after treatment of GTD. Conclusion: In patients with GTD the possibility of hyperthyroidism should be kept in mind. A high level of suspicion will allow to identifying patients with hyperthyroidism.

Progressão da lesão periapical em animais sob condições de estresse e modulação inflamatória através de bloqueador adrenérgico e inibidor seletivo da recaptação da serotonina / Progression of periapical lesion in animals under stress conditions and inflammatory modulation through adrenergic blocker and selective serotonin reuptake inhibitor

O objetivo deste estudo foi analisar os efeitos do estresse crônico sobre a periodontite apical (PA) induzida em ratos, e avaliar os efeitos do uso da Fluoxetina (antidepressivo da classe dos inibidores da recaptação de serotonina) e do Propranolol (bloqueador beta-adrenérgico), associados ou não, na modulação inflamatória e na reabsorção óssea periapical de ratos estressados. Foram utilizados 40 ratos divididos em cinco grupos: Grupo controle não-estressado (NS); Grupo controle estressado com administração de solução fisiológica (SS); Grupo estressado com administração de Fluoxetina (SF); Grupo estressado com administração de Propranolol (SP); Grupo estressado com administração de Fluoxetina e Propranolol (SFP). Os animais dos grupos estressados foram submetidos ao protocolo de estresse crônico imprevisível durante 6 semanas e as respectivas medicações foram administradas diariamente, via gavagem, ao longo de todo o período experimental. A PA foi induzida em todos os grupos após 21 dias do início do protocolo de estresse e ao final da 6ª semana, os animais foram eutanasiados e as hemimandíbulas e hemimaxilas removidas. Posteriormente foram realizadas as seguintes análises: a) da massa corporal; b) dos níveis séricos de corticosterona por radioimunoensaio; c) dos níveis séricos hormonais e inflamatórios por ensaio Multiplex; e) histomorfométrica por coloração com hematoxilina e eosina; f) da estrutura óssea periapical através de microtomografia computadorizada (micro-CT); g) da expressão gênica de biomarcadores relacionados à atividade osteoclástica, citocinas inflamatórias e metaloproteinases na região periapical por RT-PCR. Ao final do experimento os animais estressados apresentaram menor ganho de massa corporal, níveis séricos de ACTH significativamente mais altos, atividade inflamatória mais intensa e maiores volumes de lesão periapical quando comparados aos animais do grupo controle NS. Os grupos tratados SF, SP e SFP apresentaram menores volumes de lesão periapical quando comparados ao grupo controle SS, e o grupo SP apresentou menor intensidade do infiltrado inflamatório. O teste de RT-PCR mostrou maior expressão de RANKL e TRAP no grupo controle SS, bem como maior expressão de IL-6, IL-10 e IL-17 e MMP-8 quando comparado ao grupo controle NS. Na comparação em relação ao grupo controle SS, o grupo SF apresentou maior expressão de OPG, e menor expressão de IL-6 e IL-17; o grupo SP apresentou maior expressão de OPG e menor expressão de IL-6, IL-10, IL-17, MMP-8 e MMP-13, e o grupo SFP apresentou menor expressão de RANKL, TRAP, IL-6, IL-10, IL-17, MMP-8 e MMP-13. Foi concluído que o estresse crônico influenciou negativamente a patogênese da PA e ambos os medicamentos avaliados, bem como sua associação, tiveram efeitos positivos na prevenção da perda óssea e modulação inflamatória.

The aim of this study was to analyze the effects of chronic stress on induced apical periodontitis (AP) in rats, and to evaluate the effects of the use of fluoxetine (antidepressant known as selective serotonin reuptake inhibitor), and of Propranolol (beta-adrenergic blocker), associated or not, in inflammatory modulation and periapical bone resorption in stressed rats. Forty rats were divided into five groups: Unstressed control group (NS); Stressed control group with saline solution administration (SS); Stressed group with administration of Fluoxetine (SF); Stressed group with administration of Propranolol (SP); Stressed group with administration of Fluoxetine and Propranolol (SFP). The animals in the stressed groups were submitted to the unpredictable chronic stress paradigm for 6 weeks and the respective medications were administered daily, via gavage, throughout the entire experimental period. AP was induced in all groups, 21 days after the beginning of the stress paradigm, and at the end of the 6th week, the animals were euthanized and the hemi-mandibles removed for the following analyses: a) body weight b) serum corticosterone levels by radioimmunoassay; c) hormone and inflammatory serum levels by Multiplex assay; e) histomorphometric staining with hematoxylin and eosin; f) the periapical bone structure through computerized microtomography; g) gene expression related to osteoclastic activity, inflammatory cytokines and metalloproteinases in the periapical region by RT-PCR. At the end of the experiment, the stressed animals showed lower body weight gain, significantly higher levels of ACTH, more intense inflammatory infiltrate and higher volumes of periapical lesion when compared to animals in the NS control group. The treated groups SF, SP and SFP had smaller volumes of periapical lesion when compared to the SS control group and the SP group had lower intensity of inflammatory infiltrate. The RT-PCR test showed higher expression of RANKL and TRAP in the stressed control group, as well as higher expression of IL-6, IL-10, IL-17 and MMP-8 when compared to the NS control group. In comparison with the SS control group, the SF group showed higher expression of OPG, and lower expression of IL-6 and IL-17; the SP group showed higher expression of OPG and lower expression of IL-6, IL-10, IL-17, MMP-8 and MMP-13 and the SFP group showed lower expression of RANKL, TRAP, IL-6, IL-10, IL-17, MMP-8 and MMP-13. It was concluded that chronic stress negatively influenced the pathogenesis of apical periodontitis and both medications evaluated, as well as its association, had positive effects in preventing bone loss and inflammatory modulation.

Experiencia en el uso de propranolol para el tratamiento de la taquicardia supraventricular en pacientes menores de un año / Experience using propranolol for the management of supraventricular tachycardia in patients younger than 1 year

Objetivo. Evaluar los resultados y efectos adversos de la terapia con propranolol en menores de un año con taquicardia supraventricular. Población y métodos. Menores de 1 año con taquicardia supraventricular documentada, que recibieron tratamiento y prevención con propranolol por vía oral. Se analizaron sexo y edad, cardiopatía congénita asociada, pre excitación ventricular en el electrocardiograma basal, recurrencia intratratamiento y efectos adversos. Resultados. Se identificaron 107 pacientes. El primer episodio de taquicardia supraventricular ocurrió a una edad mediana de 190 días. En 10 pacientes, se observó cardiopatía congénita asociada. El 23,3 % presentó pre excitación ventricular en el electrocardiograma basal. El rango de la dosis de propranolol fue de 2 a 5 mg/kg/día. En el 30,8 %, se observó recurrencia intratratamiento. En 2 pacientes, se suspendió la medicación por efectos adversos graves. Conclusión. El propranolol evitó la recurrencia en el 70 % de los casos. En 2 pacientes, fue necesario suspenderlo por efectos adversos graves

Objective. To assess the results and adverse events of propranolol therapy in infants younger than 1 year with supraventricular tachycardia. Population and methods. Infants younger than 1 year with documented supraventricular tachycardia who received oral treatment and prophylaxis with propranolol. Sex and age, associated congenital heart disease, ventricular preexcitation in the base line electrocardiogram, on-treatment recurrence, and adverse events were analyzed. Results. A total of 107 patients were identified. The first supraventricular tachycardia event occurred at a median age of 190 days. Associated congenital heart disease was observed in 10 patients. Ventricular preexcitation in the baseline electrocardiogram was detected in 23.3 %. Propranolol dose ranged from 2 to 5 mg/kg/day. On-treatment recurrence was observed in 30.8 %. Medication was discontinued in 2 patients due to severe adverse events. Conclusion. Propranolol prevented recurrence in 70 % of cases. It was discontinued in 2 patients due to severe adverse events.

Success in the use of oral propranolol in the treatment of infantile hemangioma in nasal tip - Report of two cases

Abstract Infantile hemangioma is the most common pediatric vascular tumor, with the following risk factors: low birth weight, prematurity, white skin, female gender, multiparity and advanced maternal age. The use of oral and topical beta-blockers, although recent, has emerged as the first line of treatment, with superior safety and efficacy to previously used therapies, such as corticosteroids and surgeries. This report describes two cases of nasal tip infantile hemangioma, treated with oral propranolol. Both presented excellent therapeutic responses.

Retinopatía del prematuro: actualización en detección y tratamiento / Retinopathy of the premature: update in screening and treatment

Resumen: La Retinopatía del Prematuro (RDP) es una alteración proliferativa de los vasos sanguíneos de la retina inmadura, que afecta principalmente a los recién nacidos de muy bajo peso (RNMBP) y de menor edad gestacional. El objetivo de esta revisión es describir a qué niño se debe efectuar la detección de esta enfermedad y analizar los recientes avances en su tratamiento. La detección de RDP está dirigida principalmente a los RNMBP y a < de 32 semanas de edad gestacional, pero también se ha propuesto un criterio según edad postmenstrual. Además de la fotocoagulación con láser, tratamiento estándar en la actualidad, se han desarrollado nuevas terapias, como los agentes anti factor de crecimiento vas cular endotelial (VEGF), que se han utilizado exitosamente en la retinopatía umbral, especialmente localizada en zona I, con menos efectos adversos y mejores resultados oculares a futuro. que la fo tocoagulación con láser. En los últimos años, se han realizado ensayos clínicos con propranolol oral como tratamiento de la RDP, principalmente en la etapa pre-umbral (etapa 2 o 3 en zona II ó III). Este bloqueador beta-adrenérgico puede prevenir la progresión de la retinopatía en RNMBP de etapa pre- umbral a umbral y/o evitar la necesidad de terapias invasivas, como la fotocoagulación con láser o la administración intravítrea de agentes anti-VEGF. La fotocoagulación con láser continúa siendo el tra tamiento de elección en la RDP. Los agentes anti-VEGF y el propranolol oral, evitarían la progresión de esta patología de etapa pre-umbral a umbral, y podrían complementar el tratamiento de la RDP.

Abstract: Retinopathy of Prematurity (ROP) is a proliferative disorder of the blood vessels of the immature retina, which affects mainly very-low-birth-weight infants (VLBW). The objective of this review is to describe to which infant the screening examination of this disease should be performed and to analy ze the recent advances in the treatment of this disease, which have emerged in the last decade. The detection of this disease is mainly focused on VLBW infants and newborns < 32 weeks of gestational age. In addition to laser photocoagulation, standard treatment today, new therapies have appeared, such as the anti-VEGF agents, which have been successfully used in the threshold ROP, especially located in zone I. This therapy is less harmful than laser photocoagulation and with better ocular results in the future. In recent years, oral propranolol has been used as a treatment for ROP in clinical trials, mainly in the pre-threshold stage (stage 2 or 3 in zone II or III). This drug is a beta-adrenergic blocker that can prevent the progression of retinopathy in pre-threshold to threshold stage and/or avoid the need for invasive therapies, such as laser photocoagulation or intravitreal administration of anti-VEGF agents. Laser photocoagulation continues to be the standard treatment for ROP. New treatments have emerged for ROP, such as anti-VEGF agents and oral propranolol, which could pre vent the progression of this disease from the pre-threshold to the threshold stage.

Clinical effect of propranolol in the treatment of respiratory hemangioma in infants and young children / 中国当代儿科杂志

OBJECTIVE@#To study the clinical effect of oral propranolol in the treatment of respiratory hemangioma in infants and young children.@*METHODS@#A retrospective analysis was performed from the chart review data of children with respiratory hemangioma treated by oral propranolol and diagnosed by bronchoscopy and laryngeal plain enhanced CT/MRI from November 2012 to December 2019.@*RESULTS@#A total of 20 children were enrolled. All children had improvement in the symptoms of laryngeal stridor and dyspnea after oral administration of propranolol for 1-2 days. The median treatment time was 10 months (range 6-12 months). The median follow-up time was 10 months (range 3-15 months). Of the 20 children, 19 (95%) achieved regression of tumor, and 1 (5%) experienced an increase in tumor size during reexamination at 6 months after drug withdrawal and had no recurrence after the treatment with an increased dose of propranolol for 6 months. Only 1 child (5%) had adverse reactions, and 1 child (5%) was still under treatment.@*CONCLUSIONS@#Oral propranolol can quickly relieve the symptoms such as dyspnea and achieve tumor regression, with few adverse events, and it is therefore an effective method for the treatment of respiratory hemangioma in infants and young children.

Efectividad del propranolol en el tratamiento de malformaciones vasculares cutáneas / Effectiveness of propranolol in treatment of cutaneous vascular malformations

Se realizó un estudio cuasi-experimental de series temporales para evaluar la efectividad del propranolol en el tratamiento de malformaciones vasculares cutáneas en 48 pacientes que asistieron a la consulta del Servicio de Dermatología del Hospital Central Universitario Dr. Antonio María Pineda durante el período febrero-julio 2018. Los resultados muestran que existen diferencias estadísticamente significativas (p <0.05; p0,0001) antes y después del primer mes de tratamiento con propranolol, las cuales se mantiene hasta los seis meses, con respecto al tamaño, color, consistencia y temperatura. Se espera que los resultados sirvan para proponer el uso de propranolol como una opción terapéutica no invasiva en el tratamiento de las malformaciones vasculares cutáneas(AU)

A quasi-experimental study of time series was carried out to evaluate the effectiveness of propranolol in the treatment of cutaneous vascular malformations in 48 patients attending the Dermatology Service of the Hospital Central Universitario Dr. Antonio Maria Pineda during the period February - July 2018. The results show that there are statistically significant differences (p <0.05; p0,0001) before and after treatment with propranolol starting one month post-treatment which are kept until six months, related to size, color, consistency and temperature of lesions. We hope that these results will encourage the use of propranolol as a non-invasive therapeutic option in the treatment of cutaneous vascular malformations(AU)

Effectiveness of fixed doses of propranolol in the treatment of hemangiomas regardless of child's weight gain: a case report / Efetividade do tratamento de hemangiomas utilizando doses fixas de propranolol em comprimido independentemente do ganho de peso da criança: relato de caso

ABSTRACT Objective: To present the outcomes of fixed doses of propranolol tablets for the treatment of hemangiomas. Case description: Two illustrative cases of hemangioma in infant patients younger than six months old are described. Treatments were started in 2010 and 2011 and were monitored until August 2017. Patients were treated with fixed doses, initially calculated based on the upper limit of 3 mg/kg/day and administrated in two daily doses rounded down to the nearest multiple of five milligrams. Dosage was not adjusted to patients' weight gain. The tablets were crushed and then diluted in a maximum amount of 3 mL of water. This procedure was necessary because propranolol was not available in oral solution in 2009, when dosages available in the Brazilian market were 10, 40 and 80 mg. Both patients presented significative improvement in the first 60 days and were in complete remission by the end of the treatment. Comments: It is possible to treat patients with Propranolol 10 mg tablets, even though the dosage is not as precise as when calculated according to patients' weight. The maintenance of a fixed dose, ignoring the patient's progressive weight gains, helps avoiding the rebound effect and decreases complications.

RESUMO Objetivo: Apresentar a experiência com a utilização de propranolol em doses fixas, em forma de comprimido, para o tratamento de hemangiomas. Descrição do caso: Dois casos ilustrativos de portadores de hemangiomas com menos de seis meses de idade são descritos. O início de tratamento ocorreu nos anos de 2010 e 2011 com seguimento até agosto de 2017. Os pacientes foram tratados com doses fixas iniciais calculadas com limite máximo de 3 mg/kg/dia, divididas em duas doses diárias, sempre com quantidades múltiplas de 5 mg. Os comprimidos de 10 mg ou a sua metade eram macerados e diluídos em 3 mL de água. As doses não foram mais alteradas. Esse uso foi decorrente da ausência da forma líquida de propranolol em 2009, quando começamos a utilizar esse tratamento, sendo então apenas disponíveis comprimidos de 10, 40 e 80 mg. Os pacientes obtiveram melhora acentuada nos primeiros 60 dias e remissão completa posteriormente. Comentários: É possível o uso de comprimidos de 10 mg, apesar de resultar numa dose não exata, como a calculada por kg/peso. A manutenção da mesma dose, mesmo com aumento progressivo de peso, pode evitar o efeito rebote e diminuir o índice de complicações.

Hemangioma infantil: diagnóstico y manejo / Infantile hemangioma: diagnosis and management

El hemangioma infantil es el tumor de partes blandas más frecuente de la infancia; aparece durante las primeras semanas de vida. La fase de crecimiento progresivo ocurre durante el primer año de edad y la fase involutiva, hasta los 7 años. Puede ser único o múltiple, afectar un segmento del cuerpo o asociarse a otras anomalías en otros órganos. El tratamiento de elección para los hemangiomas que amenazan la vida o la función es el propranolol bajo un adecuado monitoreo médico; sin embargo, existen otras alternativas terapéuticas tanto para detener su crecimiento como para las secuelas. El manejo integral del hemangioma infantil varía de acuerdo a su presentación en cada paciente, de ahí la importancia de conocer su comportamiento para un adecuado diagnóstico, tratamiento y pronóstico. Este artículo brinda un enfoque práctico para el diagnóstico del hemangioma infantil, así como pautas y recomendaciones para el tratamiento sobre la base de la literatura.Palabras clave: atenolol, diagnóstico clínico, hemangioma, propranolol

Infantile hemangioma is the most frequent soft tissue tumor of childhood. It appears during the first weeks of life with a progressive growth during the first year of age and a regression phase until the seventh year of age. It can be single or multiple, affect one segment of the body or be associated with other abnormalities in other organs. Propranolol is the treatment of choice for hemangiomas that threaten life or function under adequate medical monitoring, however there are other therapeutic alternatives both to stop the proliferative phase and for the sequels. The integral management of the infantile hemangioma varies according to the presentation in each patient, hence the importance of knowing its behavior for an adequate diagnosis, treatment and prognosis. This article provides a practical approach for the diagnosis of infantile hemangioma, as well as guidelines and recommendations for treatment based on the literature

A Case of Lumbar Infantile Hemangioma (SACRAL Syndrome) Accompanied by Spinal Anomaly and Dermal Sinus / 대한피부과학회지

Infantile hemangioma (IH) usually presents solely as a cutaneous manifestation, and rarely accompanies diverse anomalies such as spinal dysraphism. A 2-month-old girl presented with IH on her lumbar skin as a coin-sized red plaque with adjacent depressed skin and a child-palm-sized red plaque on her left ankle since birth. Considering the coexistence of IH and depressed skin on the midline in her lumbosacral area, magnetic resonance imaging of her spine was performed, which showed intraspinal/dermal vascular tumors with spina bifida occulta at the 12th thoracic vertebrae level. Furthermore, no neurologic deficits were observed. She has been taking oral propranolol with topical timolol to prevent neural complications and the lesions clinically improved. However, additional surgery for the intraspinal lesions was considered due to urination/defecation abnormalities since she was 13 months of age. In cases of midline IH, particularly with additional skin lesions, appropriate imaging studies to identify accompanying anomalies should be performed, and referrals to neurosurgical specialists should be considered.

Cost-Effectiveness of Rate- and Rhythm-Control Drugs for Treating Atrial Fibrillation in Korea

PURPOSE: Although the economic and mortality burden of atrial fibrillation (AF) is substantial, it remains unclear which treatment strategies for rate and rhythm control are most cost-effective. Consequently, economic factors can play an adjunctive role in guiding treatment selection. MATERIALS AND METHODS: We built a Markov chain Monte Carlo model using the Korean Health Insurance Review & Assessment Service database. Drugs for rate control and rhythm control in AF were analyzed. Cost-effective therapies were selected using a cost-effectiveness ratio, calculated by net cost and quality-adjusted life years (QALY). RESULTS: In the National Health Insurance Service data, 268149 patients with prevalent AF (age ≥18 years) were identified between January 1, 2013 and December 31, 2015. Among them, 212459 and 55690 patients were taking drugs for rate and rhythm control, respectively. Atenolol cost $714/QALY. Among the rate-control medications, the cost of propranolol was lowest at $487/QALY, while that of carvedilol was highest at $1363/QALY. Among the rhythm-control medications, the cost of pilsicainide was lowest at $638/QALY, while that of amiodarone was highest at $986/QALY. Flecainide and propafenone cost $834 and $830/QALY, respectively. The cost-effectiveness threshold of all drugs was lower than $30000/QALY. Compared with atenolol, the rate-control drugs propranolol, betaxolol, bevantolol, bisoprolol, diltiazem, and verapamil, as well as the rhythm-control drugs sotalol, pilsicainide, flecainide, propafenone, and dronedarone, showed better incremental cost-effectiveness ratios. CONCLUSION: Propranolol and pilsicainide appear to be cost-effective in patients with AF in Korea assuming that drug usage or compliance is the same.

Drug interactions in maternal intensive care: prevalence, risk factors, and potential risk medications / Interações medicamentosas em terapia intensiva materna: prevalência, fatores e medicamentos de risco

ABSTRACT Objective: To characterize severe potential drug interactions in maternal intensive care, and to determine their frequency, risk factors and potential risk medications. Methods: An observational and longitudinal study conducted between December 2014 and December 2015 in a maternal intensive care unit. Clinical data were collected and severe potential drug interactions were identified on pregnant inpatients. The drug interactions were classified by type, prevalence and exposure rate. A multivariate logistic regression model was used to identify the severe potential drug interactions and the related drugs (p<0.05). Results: A total of 95.1% of patients were exposed to, at least, one potential drug interaction; in that, 91.7% 33.9% were related to, respectively, moderate and severe potential drug interactions. The patients were exposed, on average, on 69.2% of days they were in the intensive care unit. The main drugs involved in more severe drug interactions were magnesium sulfate, metoclopramide, propranolol and diazepam. Conclusion: The severe potential drug interactions were observed in almost all patients of the study, and, approximately one third of those interactions were related to greater severity and resulted in exposure during long hospital stay. The higher number of prescribed drugs and its previous use of medications at home increase the occurrence of severe potential drug interactions.

RESUMO Objetivo: Caracterizar as interações medicamentosas potenciais graves em terapia intensiva materna, e determinar sua frequência, os fatores e os medicamentos de risco associados à ocorrência dessas interações. Métodos: Estudo observacional e longitudinal executado entre dezembro de 2014 a dezembro de 2015, conduzido em uma unidade de terapia intensiva materna. Foram coletados dados clínicos e identificadas interações medicamentosas potenciais graves de gestantes admitidas. As interações medicamentosas foram caracterizadas quanto ao tipo, à prevalência e à taxa de exposição. Um modelo multivariado de regressão logística foi utilizado para identificação de fatores associados à ocorrência de interações medicamentosas potenciais graves e os medicamentos implicados (p<0,05). Resultados: Um total de 95,1% das pacientes foi exposto a, no mínimo, uma interação medicamentosa potencial, com 91,7% delas envolvidas com interações medicamentosas potenciais moderadas e 33,9% com as interações graves. As pacientes ficaram expostas, em média, em 69,2% dos dias que estiveram sob terapia intensiva. Os principais medicamentos implicados em interações medicamentosas de maior gravidade foram sulfato de magnésio, metoclopramida, propranolol e diazepam. Conclusão: As interações medicamentosas potenciais graves ocorreram na maioria das pacientes avaliadas. Aproximadamente um terço das interações foram graves e levaram à maior exposição por um longo período de internação. Maior número de fármacos prescritos e uso prévio domiciliar de medicamentos elevam a ocorrência de interações medicamentosas potenciais graves.