RESUMEN
Acute postinfectious glomerulonephritis are defined by an acute nonsuppurative inflammatory insult predominantly glomerular. Its current incidence is uncertain because of the frequency of subclinical forms. The most common infectious agent involved is beta hemolytic streptococcus group A. Acute postinfectious glomerulonephritis is uncommon in adults, and its incidence is progressively declining in developed countries. Humoral immunity plays a key role in the pathogenesis of kidney damage. Complement activation by the alternative pathway is the dominant mechanism, but a third way (lectin pathway) has been recently identified. The classic clinical presentation is sudden onset of acute nephritic syndrome after a free interval from a streptococcal infection. Treatment is essentially symptomatic and prevention is possible through improved hygiene and early treatment of infections.
Asunto(s)
Glomerulonefritis/etiología , Glomérulos Renales/patología , Infecciones Estreptocócicas/complicaciones , Streptococcus pyogenes , Enfermedad Aguda , Adulto , Niño , Activación de Complemento , Femenino , Glomerulonefritis/inmunología , Glomerulonefritis/patología , Humanos , Masculino , Infecciones Estreptocócicas/inmunologíaRESUMEN
Hamartomas of the spleen or splenomas are uncommon benign lesions that predominantly occur in adults. We report a case of an 11-year-old girl with sickle cell anemia who had a single splenic 1.8 cm nodule incidentally found during splenectomy and histologically characterized by disorganized red pulp tissue without interspersed white pulp leading to the diagnosis of hamartoma. The association of hamartoma and hematological conditions is a very unusual condition in children.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Hamartoma/patología , Neoplasias del Bazo/patología , Niño , Femenino , Hamartoma/epidemiología , Hamartoma/cirugía , Humanos , Inmunohistoquímica , Incidencia , Esplenectomía , Neoplasias del Bazo/epidemiología , Neoplasias del Bazo/cirugíaAsunto(s)
Carcinoma de Células Pequeñas/patología , Carcinoma de Células Pequeñas/genética , Preescolar , Cromosomas Humanos Par 11 , Cromosomas Humanos Par 22 , Diagnóstico Diferencial , Fibroma Desmoplásico/genética , Fibroma Desmoplásico/patología , Humanos , Masculino , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Translocación GenéticaRESUMEN
Ehlers-Danlos syndrome (EDS), a heterogeneous disease of the connective tissues, is diagnosed by a triad of symptoms that include skin hyperextensibility, joint hypermobility and connective tissue fragility. Nephronophtisis (NPH) is an autosomal recessive interstitial nephritis leading to terminal renal insufficiency around puberty. The occurrence of these two rare diseases together is unusual. A review of the literature discloses no case of this association. We report here on a 16-year-old man with undiagnosed EDS, who was referred to our hospital because of renal insufficiency, history of polyuria and polydipsia. Renal ultrasound showed normal kidney size, with a lack of corticomedullary differentiation. Renal biopsy specimen disclosed chronic tubulointerstitial nephritis resembling NPH. Further evaluation identified hypermobile joints and hyperextensible skin, which led to the diagnosis of the EDS. These data suggest that patients with EDS need to be evaluated carefully for the presence of renal anomalies.
