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 case for its very rare occurrence in the eastern part of the world and the typical feature being normal MRI brain with functional defect of the olfactory pathway. Treatment was started with cyclic conjugated oestrogen and progestin. Our patient is now on regular follow-up to monitor response to treatment.
This study demonstrated that despite the increased operating time and blood loss, laparoscopy should be considered instead of laparotomy in cases of large uteri. Laparoscopically assisted vaginal hysterectomy can be performed safely for a large uterus.
Objective: To compare the efficacy of metformin and laparoscopic ovarian drilling to be used in anovulatory patients with polycystic ovarian syndrome (PCOS) for ovulation induction and pregnancy achievement. Materials and Methods: Setting and Design: A prospective clinical trial conducted in the outpatient department of Gynaecology and Obstetrics of BIRDEM hospital from August 2008 to August 2013. Patient(s): Three hundred and eight newly diagnosed patients with PCOS based on ESHRE/ASRM criteria. These patients were assigned to two groups: Group 1 (152 patients) received 500 mg of metformin three times a day; Group 2 (156 patients) received laparoscopic ovarian drilling. Main Outcome Measure(s): Rate of ovulation, pregnancy rate (PR), and live birth. Ovulation was monitored by serum progesterone, transvaginal sonography up to six cycles or till pregnancy occurred upto six months. Result(s): The ovulation rate was 68.4% in the metformin group, 59% in the laparoscopy treatment group. The pregnancy rate was (20.1% and 16.6%) and live birth rate was (18.4% and 15.4%) in metformin and laparoscopy treatment groups, respectively. The rate of spontaneous first trimester loss was three and two in metformin and laparoscopy group respectively. There were no ectopic pregnancies in metformin group and two in laparoscopy group. There was no second trimester pregnancy loss in metformin group, three in laparoscopy group. There were no multiple pregnancies in metformin group, two in lapa-* Corresponding author.
S. Jahan et al.OALibJ |
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