After eight months of follow-up, the patient improved significantly his clinical condition. At five months old, a gastrostomy tube was inserted to provide nutrition and hydroelectrolyte balance. Due to poor metabolic control, the patient demanded recurrent hospital admissions. It was started oral sodium and alkali replacement, hydrochlorothiazide and cation-exchange resin. 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).
The neonate remained with hypovolemia and severe hyperkalemia (11 mmol/L). Adrenal congenital hyperplasia was suspected and hormone-therapy started, although without improvement. Despite fluid and alkali replacement, the metabolic state did not recover. Laboratorial evaluation showed hyperkalemia (10mmol/L), hyponatremia (114 mmol/L) and metabolic acidosis (pH 7.23). A twelve-day-old term male neonate was admitted at emergency room of our institution with severe dehydration and without gastrointestinal losses.
We reported a case of life-threatening RTA-type IV manifested at neonatal period. Department of Pediatrics, Federal University of Minas Gerais, Belo Horizonte, Brazil.Īna Cristina Simoes e Silva, MD, PhD Avenida Bernardo Monteiro 1300, ap 1104, Bairro Funcionarios, 30150-281, Belo Horizonte, MG, Brazil.īy the lack of sensitivity to aldosterone effects (pseudohypoaldosteronism).
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