Van wyk Grumbach and café au lait: an unusual association

A Case of Van Wyk-Grumbach Syndrome

In boys precocious puberty is defined as the development of secondary sexual characters before 9 year of age. In girls, the development of breasts (thelarche) before 7.5 year, the development of pubic hairs (pubarche) before 8.5 year or menarche before the age of 9.5 year is considered precocious [1]. Hypothyroidism is usually associated with delayed puberty and rarely with precocious puberty [...

An unusual presentation of girl with down syndrome: Van-Wyk Grumbach syndrome

Van Wyk-Grumbach syndrome is a rare disease characterized by precocious puberty associated with prolonged hypothyroidism and multicystic enlarged ovaries. A9 year-old girl with Down syndrome visited our hospital for early menarche. At birth, she showed subclinical hypothyroidism (11.8 mg/dL of thyroxine (T4) and 6.05 mIU/mL of thyroid stimulating hormone (TSH)), but she had not been followed up...

An unusual presentation of acquired hypothyroidism: the Van Wyk-Grumbach syndrome.

The association in young females of long-standing primary hypothyroidism, isosexual precocious pseudopuberty and multicystic enlarged ovaries was first described in 1960 by Van Wyk and Grumbach. Since then, sporadic case reports have contributed to clarifying the key features of this syndrome. The unique elements that lead to this diagnosis are FSH-dominated sexual precocity combined with a del...

A child with axillary freckling and café au lait spots.

Pulmonary stenosis, café-au-lait spots, and dull intelligence.

Of the many abnormalities that have been reported in association with multiple caf&-au-lait spots, congenital cardiac anomalies are extremely rare. The purpose of this paper is to report three families in which there were children with pulmonary valvular stenosis, who were mentally dull and also had cafe-au-lait spots inherited as an autosomal dominant, as in von Recklinghausen's disease (VRD)....