ISSN - 0973-0958
Neonatal presentation of Renal Tubular Acidosis Type IV - a case report
Ana Cristina Simoes e Silva, Regina Maria Pereira, Frederico Jose de Carvalho Godinho, Sergio Veloso Brant Pinheiro.
Department of Pediatrics, Federal University of Minas Gerais, Belo Horizonte, Brazil.

by the lack of sensitivity to aldosterone effects (pseudohypoaldosteronism). We reported a case of life-threatening RTA-type IV manifested at neonatal period. A twelve-day-old term male neonate was admitted at emergency room of our institution with severe dehydration and without gastrointestinal losses. Laboratorial evaluation showed hyperkalemia (10mmol/L), hyponatremia (114 mmol/L) and metabolic acidosis (pH 7.23). Despite fluid and alkali replacement, the metabolic state did not recover. Adrenal congenital hyperplasia was suspected and hormone-therapy started, although without improvement. The neonate remained with hypovolemia and severe hyperkalemia (11 mmol/L). This evolution suggested a RTA-type IV, which was confirmed by elevated plasma aldosterone levels (33ng/dL, range 1-4ng/dl) with concomitant sodium urinary loss and low urinary potassium excretion (Na 38 mmol/L and K 0.7 mmol/L). It was started oral sodium and alkali replacement, hydrochlorothiazide and cation-exchange resin. Due to poor metabolic control, the patient demanded recurrent hospital admissions. At five months old, a gastrostomy tube was inserted to provide nutrition and hydroelectrolyte balance. After eight months of follow-up, the patient improved significantly his clinical condition. This case clearly illustrates the importance of early diagnosis and aggressive management for neonatal presenting forms of RTA.
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