TRUE PRECOCIOUS PUBERTY ASSOCIATED WITH PHENYLKETONURIA

We studied a girl with phenylketonuria who demonstrated signs of preco cious puberty. At the age of 7.5 years she had premature telarche. Her height age was 7-9/12 years and her bone age was 9 years. Gonadotropi n responses to administration were consistent with precocious puberty. Elevated serum phenylalanine levels in this patient, due to poor comp liance with the phenylalanine restricted diet, may be related to the e arly onset of puberty.