ABSTRACT
La hipertensión arterial (HTA) grave en pediatría responde fundamentalmente a causas secundarias. Presentamos una paciente adolescente de 14 años con HTA grave, alcalosis metabólica e hipopotasemia, secundaria a un tumor de células yuxtaglomerulares productor de renina, diagnosticado luego de dos años de evolución de HTA.
Severe arterial hypertension (HTN) in pediatrics is mainly due to secondary causes. Here we describe the case of a 14-year-old female adolescent with severe HTN, metabolic alkalosis, and hypokalemia, secondary to a renin-secreting juxtaglomerular cell tumor diagnosed after 2 years of HTN progression.
Subject(s)
Humans , Female , Adolescent , Hypertension/etiology , Hypokalemia/complications , Kidney Neoplasms/complications , Kidney Neoplasms/diagnosis , Renin/metabolism , Juxtaglomerular Apparatus/metabolism , Juxtaglomerular Apparatus/pathologyABSTRACT
Abstract Basaloid follicular hamartoma is a benign, superficial malformation of hair follicles that can be mistaken both clinical and histopathologically for basal cell carcinoma. Basaloid follicular hamartoma has been linked to a mutation in the PTCH-1 gene, which is part of the same pathway involved in Gorlin-Goltz syndrome. Here we present a 9-year-old patient with an asymptomatic congenital lesion on the forehead, which increased in size over the years. Histopathology showed a basaloid follicular hamartoma associated with follicular mucinosis and inflammation. Gorlin-Goltz syndrome was ruled out by clinical examination.
Subject(s)
Humans , Child , Skin Neoplasms , Carcinoma, Basal Cell , Mucinosis, Follicular , Hamartoma/complications , InflammationABSTRACT
Introducción: El liquen plano (LP) es una enfermedad inflamatoria crónica, de etiología desconocida, que puede afectar la piel, los anexos y las mucosas. Objetivo: Describir las características epidemiológicas y clínicas y el tratamiento realizado en los pacientes con diagnóstico clínico e histopatológico de LP, valorados en el Servicio de Dermatología del Hospital de Pediatría "Prof. Dr. Juan P. Garrahan" en los últimos 33 años. Diseño:Estudio descriptivo, retrospectivo y transversal. Materiales y métodos: Se revisaron las historias clínicas de los pacientes con diagnóstico histopatológico de LP atendidos en el Hospital desde agosto de 1987 hasta febrero de 2020 y se analizaron las variables epidemiológicas, clínicas y de tratamiento. Resultados: Se incluyeron datos de 26 pacientes, 18 de los cuales eran varones (69,3%). La media de edad en el momento del diagnóstico fue de 9,6 años. Presentaron la variedad de LP clásico 24 pacienes (93%). El compromiso ungueal y de la mucosa bucal se observó en 2 pacientes (7,7%). Ninguno tuvo asociación a fármacos ni a infecciones. En cuanto al tratamiento recibido, 4 pacientes (15,4%) tuvieron pérdida del seguimiento; 13 (50%) realizaron tratamiento tópico; 6 (23%), tratamiento sistémico y 3 (11,5%), tratamiento combinado. El promedio de seguimiento fue de 6 meses y se obtuvo una respuesta favorable en el 68% de los pacientes. Conclusiones: El LP es una patología de baja frecuencia en la infancia. No se encontró asociación a medicamentos ni a enfermedades infecciosas como se describe en la población adulta. Su curso es crónico y, en los pacientes que tuvieron un adecuado seguimiento, se obtuvo una buena respuesta al tratamiento indicado.
Introduction: Lichen planus (LP) is a chronic, inflammatory disease of unknown etiology, which can affect skin, adnexal tissues and mucosa. Objective: to describe the clinical, epidemiological characteristics and the treatment received by patients with clinical and histopathological diagnosis of LP, seen at the Dermatology Service of Pediatric Hospital "Prof. Dr. Juan P. Garrahan" in the last 33 years. Design: Descriptive, retrospective and cross-sectional study. Materials y methods: We reviewed the clinical history of patients with histopathological diagnosis of LP that were treated at our hospital from August 1987 to February 2020 and analysed the epidemiological, clinical and treatment variables. Results: Data of 26 patients were included in this study 18 of which are males (69.3%). The mean age of diagnosis was 9.6 years. 24 patients (93%) presented the classical type of LP. Ungual and mucosal compromise were observed in 2 patients (7.7%) and none had associations with infections or drugs. Regarding treatment, 4 patients (15.4%) did not continue follow-ups, 13 patients (50%) had topical treatment, 6 (23%) had systemic treatment and 3 patients (11.5%) received combined treatment. The average follow-up time was 6 months and 68% of patients had favorable outcome. Conclusions: LP is an uncommon pathology in children. During this study we did not find any etiologic relations with use of drugs or infectious diseases as is described in de adult population. Its course is chronic and in the patients that had a proper follow-up a good response to the indicated treatment was obtained.