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Introduction: Patients with optic pathway gliomas (OPG) have good survival rates although their long-term quality of life can be affected by the tumor or treatment-related morbidity. This retrospective study sought to describe the clinical presentation and outcomes of children with OPG at a tertiary center in Mexico. Methods: Consecutive patients <18 years-of-age with newly diagnosed OPG between January 2002 and December 2020 at the Hospital Civil de Guadalajara Dr. Juan I. Menchaca in Guadalajara, Mexico were included. Results: Thirty patients were identified with a median age of six years. The most frequent clinical manifestations were loss of visual acuity (40%) and headaches (23%). Neurofibromatosis-1 was found in 23.3% of the patients. Surgery, either biopsy or resection, was done in 20 of 30 patients. Two patients died shortly after initial surgery. The 5-year event-free survival (EFS) was 79.3% ± 10.8% and the 5-year overall survival was 89.5% ± 6.9%. Lower EFS was associated with age less than 3 years, intracranial hypertension at presentation, and diencephalic syndrome. Patients who received surgery as first-line treatment had a 3.1 times greater risk of achieving a performance score of less than 90 points at 6 months after diagnosis (p=0.006). Of 10 patients with vision testing, 5 had improvement in visual acuity, 4 had no changes, and one patient showed worsening. Conclusion: Our data suggests that favorable outcomes can be achieved with OPG in low- and middle-income countries, although a high rate of surgical complications was described leading to a lower overall survival. These data can be used prospectively to optimize treatment at this institute and other middle-income countries through a comprehensive, multidisciplinary approach.
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Sweet syndrome (SS) is an acute febrile neutrophilic dermatosis that is histologically characterized by an infiltration of the dermis by neutrophils. A 12-year-old adolescent female patient recently diagnosed with acute promyelocytic leukemia presented with fever and was hospitalized for antibiotic management after 22 days of being treated with a treatment protocol based on daunorubicin, all-trans retinoic acid (ATRA), and prophylaxis with dexamethasone, the patient developed erythematous skin lesions located mostly on the extremities. Lesions evolved into painful subcutaneous nodules, and one lesion evolved into a 2.5-cm blister with a purple and necrotic base. A skin biopsy was performed and showed neutrophilic dermatosis which confirmed the diagnosis of SS. The patient's clinical features complied with criteria for differentiation syndrome complicated by shock. Two days after ATRA was suspended, the patient presented resolution of the fever and skin lesions. SS is a rare neutrophilic dermatosis secondary to an innate immune disorder classified into four categories: classical (idiopathic), para-inflammatory, paraneoplastic or pregnancy-related. SS has been described in patients with acute myeloid leukemia in adults secondary to the use of drugs such as ATRA or as a part of a paraneoplastic syndrome. SS can occur exceptionally in children with myeloid leukemia secondary to the use of drugs such as ATRA.
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Resumen Introducción: En Colombia, la criptococosis es una infección fúngica invasiva poco frecuente. Objetivo: Reportar el caso clínico de un niño con meningoencefalitis por Cryptococcus gattii que tenía como factor de riesgo una disfunción inmune asociada a cirrosis, un factor de riesgo descrito en adultos pero no reportado en niños. Materiales y métodos: Se realizó una revisión no sistemática de la literatura y un resumen de los aspectos más relevantes de la historia clínica. Resultados: Se presenta el caso de un paciente masculino de 16 años con antecedente de cirrosis quien consultó por un cuadro clínico de 8 días de evolución de fiebre, emesis persistente, cefalea frontal, fotofobia y tinitus. Al examen físico no presentaba alteraciones neurológicas. En el estudio del líquido cefalorraquídeo las pruebas moleculares directas fueron positivas para Cryptococcus neoformans/gattii y el cultivo reportó crecimiento de Cryptococcus gattii. Conclusión: En este caso se destaca la importancia de sospechar infecciones oportunistas en pacientes que tienen inmunodeficiencia no solo secundaria a las infecciones por VIH, sino también a la producida por otros factores que alteran la respuesta inmunológica como la cirrosis.
Abstract Introduction: In Colombia, cryptococcosis is a rare invasive fungal infection. Objective: To report the clinical case of a child with Cryptococcus gattii meningoencephalitis whose risk factor was immune dysfunction associated with cirrhosis, a risk factor described in adults which in the reviewed literature has not been reported in children. Materials and methods: A non-systematic review of the literature and a summary of the most relevant aspects of the clinical history were performed. Results: We present the case of a 16-year-old male patient with a history of cirrhosis who came for consultation with a clinical presentation of 8 days of fever, persistent emesis, frontal headache, photophobia and tinnitus. The physical examination did not present neurological alterations. In the study of cerebrospinal fluid, he presented positive direct molecular tests for Cryptococcus neoformans/gattii and the culture reported growth of Cryptococcus gattii. Conclusion: In this case, the importance of suspecting opportunistic infections in patients who have immunodeficiencies not only secondary to HIV infections, but also to those caused by other factors that alter the immune response such as cirrhosis is highlighted.
