Un caso de Síndrome de Sjõgren primario complicado con acidosis tubular renal tipo 1 / Sjõgren syndrome associated with renal tubular acidosis type I

Primary Sjõgren's Syndrome complicated with a renal tubular acidosis type 1 and hypocalcemic paralysis, as the principal clinical manifestation, is uncommon. Although the initial manifestations of the nephropathy are not well understood, it is believed that the invasion of mononuclear cells and the high level of circulating antibodies, play an important role in the pathogenesis of the disease. We present a patient with hypocalcemic paralysis as an initial manifestation of a latent Sjõgren's disease. The glandular biopsy was normal, suggesting a mayor participation of an immunological humoral factor in the renal lesion.

Renal tubular acidosis

The clinical laboratory and X-Ray findings of 16 patients with distal [type-1] renal tubular acidosis diagnosed at Mayo and Sheikh Zayed Hospital, Lahore during an eight year period [1984 to 1992] are presented. Twelve patients [75%] were males and four females [25%]. Their ages ranged between 1.5 and 41 years [mean age 12.8 years]. Eight patients [50%] had radiologically evident bone disease in the form of rickets or osteomalacia. Two patients had pathological fractures. Growth retardation was noted in 10 patients [63%]. Polyuria and polydipsia were noted in 6 patients [31%]. Nephrocalcinosis and or nephrolithiasis was evident in 12 patients [75%]. Muscle weakness were noted in 7 patients [44%]. Biochemical derangements observed were hyperchloremic metabolic acidosis mean blood pH = 7.259 +/- 0.055, but urine pH remained alkaline in all patients during all grades of metabolic acidosis with a mean value of 6.69 [range 5.8 to 8.0]. Serum Chloride level was mean 111 +/- 5.288 mmol/l. Hypocalcemia, hyponatremia and hypokalemia was noted with mean values of 8.44 +/- 1.24 mg/dl, 134 +/- 3.56 mmol/l and 3.68 +/- 0.53. Treatment with alkali therapy resulted in general well being, reduced bone pains, muscle weakness and improved growth significantly

Acidosis tubular renal en niños: revisión de 174 casos / Renal tubular acidosis in children :review of 174 cases

Se estudiaron 174 pacientes con diagnóstico de Acidosis Tubular Renal, quienes asistieron regularmente a la Consulta del Servicio de Nefrología del Hospital de Niños "J.M. de los Ríos", durante el año 1985. Las manifestaciones clínicas más frecuentes fueron infección urinaria, hematuria, retraso pondo estatural, dolor abdominal y trastornos hidroelectrolíticos..