Secondary Sjogren's syndrome and scleroderma presenting as renal tubular acidosis.

We report a case of distal renal tubular acidosis in a twenty year old female patient of scleroderma and secondary Sjogren's syndrome. This patient presented with two episodes of flaccid quadriparesis which were associated with hypokalaemia and was later found to have an underlying scleroderma with secondary Sjogren's syndrome.

Cleistanthus collinus poisoning.

Cleistanthus collinus is an extremely toxic plant poison. Cleistanthin A and B, the toxins of Cleistanthus collinus, are diphyllin glycosides which produce cardiac arrhythmias, urinary potassium wasting, hypoxia, metabolic acidosis and hypotension. We report ARDS, distal renal tubular acidosis and distributive shock secondary to inappropriate vasodilatation in a case following ingestion of its leaves.

Infantile cystinosis.

Ten patients of nephropathic cystinosis were admitted during the period 1995-2000. Their mean age was 12 months. The signs of failure to thrive and advanced rickets were seen in all patients. Other features included polyuria, polydipsia, pathologic fractures and deafness. Laboratory findings included glucosuria, hyposthenuria, hypocalcemia, proteinuria and azotemia. Therapy with phosphocysteamine showed marked clinical improvement.

Acidosis tubular renal / Renal tubular acidosis

Los transtornos de acidificación renal se manifiestan por síntomas y signos poco específicos, por lo que el pediatra debe sospechar el diagnóstico frente a un niño con dificultad en el progreso ponderal, anorexia, poliuria y que en los exámenes presenta acidosis metabólica hiperclómerica. Con estos antecedentes es importante la derivación oportuna al nefrólogo para la determinación del tipo de acidosis tubular renal y el tratamiento adecuado que repercutirá en el pronóstico vital del niño

Study of urinary acidification in patients with idiopathic hypocitraturia

Hypocitraturia (HCit) is one of the most remarkable features of renal tubular acidosis, but an acidification defect is not seen in the majority of hypocitraturic patients, whose disease is denoted idiopathic hypocitraturia. In order to assess the integrity of urinary acidification mechanisms in hypocitraturic idiopathic calcium stone formers, we studied two groups of patients, hypocitraturic (HCit, N = 21, 39.5 + or - 11.5 years, 11 females and 10 males) and normocitraturic (NCit, N = 23, 40.2 + or - 11.7 years, 16 females and 7 males) subjects, during a short ammonium chloride loading test lasting 8 h. During the baseline period HCit patients showed significantly higher levels of titratable acid (TA). After the administration of ammonium chloride, mean urinary pH (3rd to 8th hour) and TA and ammonium excretion did not differ significantly between groups. Conversely, during the first hour mean urinary pH was lower and TA and ammonium excretion was higher in HCit. The enhanced TA excretion by HCit during the baseline period and during the first hour suggests that the phosphate buffer mechanism is activated. The earlier response in ammonium excretion by HCit further supports other evidence that acidification mechanisms react promptly. The present results suggest that in the course of lithiasic disease, hypocitraturia coexists with subtle changes in the excretion of hydrogen ions in basal situations

Acidosis tubular renal tipo I como manifestación inicial de un síndrome de Sjögren / Tubular renal acidosis as the initial manifestation of Sjögren's syndrome

Se relata la historia clínica de una niña de 13 años con síndrome de Sjögren primario que se presentó como una acidosis tubular renal con nefrocalcinosis. La ecografía de parótida que evidenciaba áreas ecolúcidas, una biopsia de glándula salival con infiltrados linfocitarios y atrofia acinar y la existencia de una hermana que padece dicho síndrome, contribuyeron al diagnóstico

Paralisia periódica hipocaliêmica, distúrbio da funçäo renal e púrpura hiperglobulinêmia no curso de síndrome de Sjögren: a propósito de um caso / Paralysis, hypokalemic periodic, renal function disorder and purpura hyperglobulinemical in the Sjogren's syndrome: a propose of a case

Os autores apresentam o caso de uma paciente de 24 anos sofredora de síndrome de Sjöen com púrpura hiperglobulinêmica, acidose tubular renal, diabetes insípido nefrogênico e paralisia periódica hipocaliêmica, que motivara suas internaçöes. Apresentava ainda miocardiopatia chagásica e neurocisticercose. Discutem o diagnóstico e revisam os aspectos principais da síndrome de Sjöen

Acidosis tubular renal / Renal tubular acidosis

Se actualiza el concepto de ATR, haciéndose una diferenciación fisiopatológica y clínica de las formas proximal y distal. Se señala asimismo la distinta necesidad de bicarbonato para el tratamiento de las mismas. Se presenta ena clasificación de la ATR.