Elevated serum prolactin concentrations in phenylketonuric patients on a 'loose diet'.

[classical phenylketonuria]

High levels of phenylalanine (Phe ) have been shown to reduce dopamine (DA ) and noradrenaline (NA ) production . We have therefore evaluated whether increased serum Phe concentrations lead to increased prolactin (PRL ) secretion in phenylketonuria .Forty -two patients with classical phenylketonuria were divided into two groups . Group A (n = 19 ) adhered very strictly to their special diet (Phe = 0 .09 +/- 0 .039 mmol /l ) and Group B (n = 23 ) were on a 'loose diet ' (Phe = 0 .77 +/- 0 .45 mmol /l ). Thirty healthy children of comparable age served as controls .PRL was measured by RIA , and Phe and tyrosine (Tyr ) with an amino-acid autoanalyser . Adrenaline (A ), noradrenaline (NA ), and dopamine (DA ) were measured by HPLC .Serum PRL in group B patients (1595 +/- 57 mU /l ) was significantly raised compared to Group A patients (243 +/- 57 mU /l ) and controls (201 +/- 55 mU /l ) (P < 0 .001 ). Serum DA , A and NA in group B were lower than in group A patients and controls . Serum Phe in group B patients was significantly correlated to serum PRL (r = 0 .59 ) and DA (r = -0 .41 ). Irregular menses were observed in 1 /10 women from group A but in 10 /13 in group B .High serum phenylalanine concentrations in phenylketonuric patients not strictly adhering to their diets are correlated with high serum PRL and low serum dopamine concentrations , and a high prevalence of menstrual irregularities .

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Diseases presenting "and a high prevalence of menstrual irregularities" symptom

classical phenylketonuria

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