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PROXIMAL RENAL TUBULAR ACIDOSIS IN TETRALOGY OF FALLOT
Author(s) -
RODRIGUEZSORIANO J.,
VALLO A.,
CHOUZA M.,
CASTILLO G.
Publication year - 1975
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1975.tb03902.x
Subject(s) - medicine , acidosis , metabolic acidosis , hematocrit , bicarbonate , tetralogy of fallot , cardiology , acid–base homeostasis , endocrinology , heart disease
Rodriguez‐Soriano, J., Vallo, A., Chouza, M. and Castillo, G. (Department of Paediatrics, Hospital Infantil de la Seguridad Social, Bilbao, Spain). Proximal renal tubular acidosis in tetralogy of Fallot. Acta Paediatr Scand, 64:671, 1975.–A 9‐year‐old girl presented with tetralogy of Fallot and moderate metabolic acidosis. Despite a Blalock's fistula there was evidence of chronic hypoxia with cyanosis, clubbing of fingers and toes and very elevated blood hematocrit values. Renal acidification and bicarbonate titration demonstrated the existence of proximal renal tubular acidosis: renal bicarbonate threshold was low (18 mmoles/1) and normal urinary acidification was present at subthreshold serum bicarbonate levels. Following corrective heart surgery, blood acid‐base values and renal reabsorption of bicarbonate became normal. A causal relationship between extracellular fluid volume expansion dependent on the high hematocrit and proximal renal tubular acidosis is suggested.