ABSTRACT
Pulmonary alveolar proteinosis is a rare disease characterized by extensive radiological and tomographic pulmonary lesions and a variable clinical picture ranging from pulmonary insufficiency to spontaneous remission. Among its three described forms, the so called Idiopathic or Adult form is responsible for more than 80% of published cases. It's physiopathology depends on an autoimmune process directed against the GM-CSF (Granulocite Macrophage- Colony Stimulating Factor) that induces a functional defect of the macrophage with consequent intraalveolar accumulation of surfactant. Pulmonary and extrapulmonary infections, are common, often with unusual pathogens. Diagnosis can be made from the combination of clinical, radiological and bronchoalveolar lavage data, although sometimes histopathologic material is needed for confirmation. Total pulmonary lavage is currently the treatment of choice. Mortality is usually low and related mostly to infectious complications. We hereby describe our experience with two patients.
Subject(s)
Pulmonary Alveolar Proteinosis/diagnosis , Female , Humans , Male , Middle Aged , Pulmonary Alveolar Proteinosis/diagnostic imaging , Pulmonary Alveolar Proteinosis/pathology , Radiography , Young AdultABSTRACT
One case of xanthogranulomatous pyelonephritis diagnosed following nephrectomy is described, considering its outstanding characteristics such as male adolescent, right kidney, involvement with normal function and without pathological history for which there is no explanation to date. This disease usually occurs in women 60 years or older, suggesting a renal tumor. In 50% of the cases, urine cultures are positive for Escherichia coli or Proteus mirabilis; in accordance with different reports renal tissue cultures are positive in more than 90%. Malnutrition, calcification, urolithiasis and renal failure with hydronephrosis are common findings. The left kidney is more frequently involved. The etiology is unclear: many hypotheses are discussed, with malnutrition and peroxidase deficit as important causes.
Subject(s)
Pyelonephritis, Xanthogranulomatous/diagnosis , Adolescent , Diagnosis, Differential , Humans , Male , Nephrectomy , Pyelonephritis, Xanthogranulomatous/etiology , Pyelonephritis, Xanthogranulomatous/pathologyABSTRACT
Se describe 1 caso de pielonefritis xantogranulomatosa que fue diagnosticado luego de nefrectomía y con algunas características destacables como ser, adolescente varon, riñón derecho afectado y funcionante, sin antecedentes previos patológicos, de las que no encontramos explicación en la revisión bibliográfica realizada. La pielonefritis xantogrnaulomatosa se observa usualmente en mujeres en la sexta década de la vida y la presunción sugiere tumor renal. El 50% de los casos se asocia a cultivos de orina positivos para Escherichia coli o Proteus mirabilis según diferentes series; en más del 90% de los casos, el cultivo del tejido renal afectado es bacteriológicamente positivo. Desnutrición, urolitiasis, calcificación y riñón no funcionante, con hidronefrosis, son los hallazgos comunes en esta enfermedad. El riñón ezquierdo es el más frecuentemente comprometido. La etiología es poco clara y se discuten varias hipótesis, siendo la desnutrición y la deficiencia de peroxidasas las que tienen mayor aceptación
Subject(s)
Humans , Adolescent , Male , Pyelonephritis, Xanthogranulomatous/diagnosis , Diagnosis, Differential , Pyelonephritis, Xanthogranulomatous/etiology , Pyelonephritis, Xanthogranulomatous/pathologyABSTRACT
Se describe 1 caso de pielonefritis xantogranulomatosa que fue diagnosticado luego de nefrectomía y con algunas características destacables como ser, adolescente varon, riñón derecho afectado y funcionante, sin antecedentes previos patológicos, de las que no encontramos explicación en la revisión bibliográfica realizada. La pielonefritis xantogrnaulomatosa se observa usualmente en mujeres en la sexta década de la vida y la presunción sugiere tumor renal. El 50% de los casos se asocia a cultivos de orina positivos para Escherichia coli o Proteus mirabilis según diferentes series; en más del 90% de los casos, el cultivo del tejido renal afectado es bacteriológicamente positivo. Desnutrición, urolitiasis, calcificación y riñón no funcionante, con hidronefrosis, son los hallazgos comunes en esta enfermedad. El riñón ezquierdo es el más frecuentemente comprometido. La etiología es poco clara y se discuten varias hipótesis, siendo la desnutrición y la deficiencia de peroxidasas las que tienen mayor aceptación (AU)
Subject(s)
Humans , Adolescent , Male , Pyelonephritis, Xanthogranulomatous/diagnosis , Pyelonephritis, Xanthogranulomatous/etiology , Pyelonephritis, Xanthogranulomatous/pathology , Diagnosis, DifferentialABSTRACT
One case of xanthogranulomatous pyelonephritis diagnosed following nephrectomy is described, considering its outstanding characteristics such as male adolescent, right kidney, involvement with normal function and without pathological history for which there is no explanation to date. This disease usually occurs in women 60 years or older, suggesting a renal tumor. In 50
of the cases, urine cultures are positive for Escherichia coli or Proteus mirabilis; in accordance with different reports renal tissue cultures are positive in more than 90
. Malnutrition, calcification, urolithiasis and renal failure with hydronephrosis are common findings. The left kidney is more frequently involved. The etiology is unclear: many hypotheses are discussed, with malnutrition and peroxidase deficit as important causes.
