RESUMEN
We report a case of hypokalemic nephropathy associated with primary Sjögren's syndrome (SS). The patient presented with profound and persistent hypokalemia secondary to distal renal tubular acidosis (RTA). A renal biopsy exhibited tubular degeneration, marked interstitial fibrosis and intense macrophage infiltration. Hypokalemia has been reported to induce macrophage infiltration in experimental animal models but not in humans. This is the first report of intense tubulointerstitial macrophage infiltration in a patient with hypokalemic nephropathy associated with SS.
Asunto(s)
Hipopotasemia/complicaciones , Hipopotasemia/inmunología , Enfermedades Renales/complicaciones , Enfermedades Renales/inmunología , Túbulos Renales/inmunología , Túbulos Renales/patología , Macrófagos , Síndrome de Sjögren/complicaciones , Adulto , Femenino , HumanosRESUMEN
BACKGROUND: Thyrotoxic periodic paralysis (TPP) is characterized by episodes of neuromuscular weakness occurring in the context of hypokalemia and hyperthyroidism and has been predominantly described in Oriental populations. Whereas it is uncommon in Caucasians and Blacks, TPP does occur in individuals of Native American descent. The objective was to analyze the clinical, biochemical, and HLA characteristics of a group of Mexican mestizo patients with TPP. METHODS: The sample was comprised of 14 men with TPP diagnosed since January 1990, based on one or more episodes of flaccid paralysis, accompanied by hypokalemia and occurring in the context of clinical and biochemical hyperthyroidism. Eight were available for HLA testing. RESULTS: Hyperthyroidism was diagnosed before the development of periodic paralysis in five of the patients, whereas in six it occurred afterward. The severity of paralysis did not correlate with the degree of either hypokalemia or hyperthyroidism. An increased frequency of HLA-DR3 was found in Graves' patients without paralysis but not in those with paralysis, as compared to the general population. CONCLUSIONS: TPP is more common than previously thought in Mexicans, in whom it behaves as in other Native American groups. The lack of HLA-DR3 association in Graves' patients with TPP is interesting, but at the moment has no pathophysiological implications.
Asunto(s)
Etnicidad , Enfermedad de Graves/complicaciones , Antígenos HLA/análisis , Hipopotasemia/etnología , Parálisis/etnología , Tirotoxicosis/etnología , Adulto , Pueblo Asiatico/genética , Etnicidad/genética , Frecuencia de los Genes , Predisposición Genética a la Enfermedad , Enfermedad de Graves/sangre , Enfermedad de Graves/inmunología , Antígenos HLA/genética , Antígeno HLA-DR3/análisis , Antígeno HLA-DR3/genética , Humanos , Hipopotasemia/sangre , Hipopotasemia/etiología , Hipopotasemia/inmunología , Indígenas Norteamericanos/genética , Masculino , México/epidemiología , Persona de Mediana Edad , Parálisis/sangre , Parálisis/etiología , Parálisis/inmunología , Periodicidad , Potasio/sangre , Factores Sexuales , España/etnología , Hormonas Tiroideas/sangre , Tirotoxicosis/sangre , Tirotoxicosis/etiología , Tirotoxicosis/inmunología , Población Blanca/genéticaAsunto(s)
Complejo Antígeno-Anticuerpo/metabolismo , Creatina Quinasa/metabolismo , Hipopotasemia/diagnóstico , Inmunoglobulina A/metabolismo , Atrofia Muscular/diagnóstico , Femenino , Humanos , Hipopotasemia/inmunología , Persona de Mediana Edad , Atrofia Muscular/inmunología , Potasio/administración & dosificación , Potasio/sangreRESUMEN
A case of alpha-chain disease (alpha-CD) is described presenting as a malabsorption syndrome complicated by severe kalipenic nephropathy. Antibiotics and cyclophosphamide were effective in controlling the malabsorption and thr renal dysfunction but did not affect the basic pathological lesion. Eventually the disease became generalized, affected lymph nodes, liver and bone marrow and caused multiple intestinal perforations. Detailed histological studies failed to demonstrate the development of reticulum-cell sarcoma as a terminal complication of the disease and are suggestive of a single malignant plasmacytic cell process.