Samira Humaira Habib
Bangladesh
Case Report
Kallmann’s Syndrome: A Rare Cause of Primary Amenorrhoea
Author(s): Samsad Jahan, Shahana Shermin, Samira Humaira Habib and Reefat NayerSamsad Jahan, Shahana Shermin, Samira Humaira Habib and Reefat Nayer
Kallmann syndrome is a very rare hereditary disease. It is characterized by hypogonadotropic hypogonadism in association with anosmia ot hyposmia, both of which occur as a result of impairment of olfactory axon development and failure of migration of gonadotropin-releasing hormone (GnRH) neurons. Mode of inheritance can be autosomal dominant, autosomal recessive, or X-linked. We report a case of Kallmann syndrome in an 18 year old girl who presented with primary amenorrhoea, poor sexual development with poor sense of smell and colour blindness. Plasma levels of LH, FSH and oestradiol were very low. The patient’s other pituitary hormone levels were normal. Chromosome analysis showed 46, XX karyotype. USG of lower abdomen confirmed presence of uterus and ovaries. MRI of brain showed olfactory bulbs to be present and there was no pituitary or hypothalamic lesion. We present this ca.. View More»