Fig. 2.

Natural history of argininosuccinic aciduria. A Kaplan-Meier survival curves for all (solid line), early-onset (dashed line), late-onset (dashed dotted line) and screened (dotted line) patients. B Natural history of the systemic phenotype of argininosuccinic aciduria. Mean ± standard error of age of onset of each symptom from data of the whole cohort when information available: developmental delay (n = 7), abnormal LFTs (n = 8), hepatomegaly (n = 18), epilepsy (n = 15), brittle hair (n = 4), ataxia (n = 6), hypokalaemia (n = 2), high blood pressure (n = 1). Symptom frequency in the total population of patients studied is presented in brackets. ALT: plasma alanine aminotransferase activity. It was assumed that patients had normal blood pressure if hypertension was not specifically mentioned in medical records