RESUMEN
Las inmunodeficiencias primarias más frecuentes son las humorales, en las que predominan las deficiencias de anticuerpos, y entre éstas las inmunodeficiencias con timoma son raras. En este trabajo se presenta un paciente masculino de 3 años de edad, sin antecedentes familiares de inmunodeficiencia, que a los 21 días de nacido presentó varicela afebril. A partir de un año de edad comenzó a presentar episodios frecuentes de infecciones de vías respiratorias altas (faringoamigdalitis, rinitis purulenta), y bajas como neumonías, o reacciones alérgicas a las picadas de insectos que se infectaban secundariamente. Los estudios inmunológicos revelaron una disminución de las concentraciones séricas de IgG, IgA, (IgG 7.4 g/l, IgA 0.52 g/l), IgM normal (IgM 1g/l) y niveles elevados de IgE (>200u/ml). El estudio radiológico mostró en el mediastino anterior una masa relacionada con el timo. El paciente recibió tratamiento con factor de transferencia, gammaglobulina y se realizó la timectomia por videotoracoscopía. El estudio histológico evidenció un timoma linfocítico benigno. Los linfocitos T CD2+, CD3+, CD8+ estaban disminuidos en el preoperatorio, mientras que en el post operatorio los linfocitos CD2+, CD4+ estaban disminuidos y los CD8+ aumentados
The most frequent primary immunodeficiency is the humoral, where the deficiency of antibodies prevails; among these, the immunodeficiency with thymoma is rare. In this work, a 3- yearold male is presented, without family antecedents of immunodeficiency, which 21 days after birth presented Chicken pox without fever. In the first year of age, he began to present frequent episodes of infections of the upper and the lower respiratory tract: tonsillitis, purulent rhinitis, and pneumonias. He also presented an allergic reaction to insect bites that became infected secondarily. The immunologic studies revealed a decrease of the seric concentrations of IgG, IgA (IgG 7.4 g/l, IgA 0.52 g/l), normal IgM values (IgM 1 g/l) and high levels of IgE (> 200u/ ml). The amount of CD2+, CD3+ and CD8+ T lymphocytes were diminished pre surgery. After surgery, the levels of CD2+, and CD4+ lymphocytes were diminished and those of CD8+ lymphocytes increased. The radiological study showed an anterior, mediastinal mass related to the thymus. The patient received treatment with transfer factor, gammaglobulin and underwent resection of the thymoma for videothoracoscopy. The histology study evidenced a benign lymphocytic thymoma
Asunto(s)
Masculino , Preescolar , Humanos , Timoma/diagnóstico , Síndromes de Inmunodeficiencia/complicaciones , Neoplasias del Timo/diagnóstico , Timoma/tratamiento farmacológico , Timoma/inmunología , Timoma/cirugía , Síndromes de Inmunodeficiencia/diagnóstico , Síndromes de Inmunodeficiencia/inmunología , Timectomía/métodos , Globinas/uso terapéutico , Biomarcadores/sangre , Neoplasias del Timo/tratamiento farmacológico , Neoplasias del Timo/cirugíaRESUMEN
OBJECTIVE: To report on a female patient with metachronous bilateral Wilms' tumor with a long survival. METHODS: An 11-year-old girl underwent total nephrectomy when she was 4 years old for Wilms' tumor of the right kidney. She received cobalt therapy and chemotherapy, and the evolution was good for three years, at which time a control US scan detected a tumor in the left kidney that was diagnosed as Wilms' tumor. She underwent a partial nephrectomy at the Hospital Pediátrico de Centro Habana (Cuba) and received chemotherapy. RESULTS/CONCLUSION: A recent US scan and a descending pyelogram showed completely normal findings and growth of the remaining renal parenchyma. The patient is alive and well 7 years and 8 months later.
Asunto(s)
Neoplasias Renales/diagnóstico , Neoplasias Primarias Múltiples/diagnóstico , Tumor de Wilms/diagnóstico , Niño , Femenino , Humanos , SobrevivientesRESUMEN
OBJECTIVE: To report on a 6-year-old patient with an infected simple renal cyst. METHODS/RESULTS: A 6-year-old girl was admitted on three occasions for acute infectious syndrome and right lumboabdominal pain. The ultrasound scans showed a simple cyst in the right kidney which contained cellular elements. The patient was treated with antimicrobials on each occasion and the symptoms disappeared a few days later. On her third admission surgery was decided due to the recurrent clinical symptoms, a persistently high erythrocyte sedimentation rate and the presence of intracystic cellular elements, which raised the suspicion of intracystic abscess or tumor. The patient was submitted to surgery; the cyst was drained, resected and the internal wall marsupialized. The postoperative period was unremarkable and the patient is asymptomatic 10 months after surgery. CONCLUSION: The case described herein is uncommon; to our knowledge, a similar case has not been reported in the literature.