M. A. Amani , N. Medjadi , B. Benlazaar

Department of Endocrinology and Diabetology 1ST November Hospital Oran, Algeria
Author    Correspondence author
International Journal of Clinical Case Reports, 2016, Vol. 6, No. 23   doi: 10.5376/ijccr.2016.06.0023
Received: 22 Feb., 2016    Accepted: 28 Mar., 2016    Published: 05 Sep., 2016

This is an open access article published under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

M. A. Amani, N. Medjadi, and B. Benlazaar, 2016, Kallmann Syndrome with Panhypopituitarism and Uterine Agenesis, International Journal of Clinical Case Report, 6(23): 1-3 (doi: 10.5376/ijccr. 2016.06.0023).

We report the case of a female patient of 27 years with no particular family history and who consulted for primary amenorrhea. The clinical examination revealed a female morphotype with eunuchoid appearance. The patient was immature and had anosmia. Laboratory tests: FSH- 0.67mIU/L, LH-0.05mIU/L, Estradiol- 17pg/ml (gonadotropin deficiency), basic cortisol-264.72nmol /L, ACTH-6pg/ml (adrenocorticotropic deficiency), FT4-6.20 pmol/L, TSHus-3.11?IU/L (thyreotropic deficiency), PRL = 4.02ng/ml. The Karyotype was 46XX. The hypothalamic pituitary MRI revealed an agenesis of the olfactory bulbs (Fig.1). An abdominal ultrasound performed to search an associated renal malformation and completed with a pelvic MRI found a uterine agenesis (Fig.2), atrophic ovaries (right ovary-1cm³ and left ovary-2cm³, fig.3 and fig 4 respectively) and a malrotation of a small right kidney (fig.5). Our treatment consisted in a hormonal replacement therapy.

 

Kallmann syndrome; Panhypopituitarism; Uterine agenesis; Renal malformation