Kallmann Syndrome and Idiopathic Hypogonadotropic Hypogonadism Treatment & Management

Updated: Oct 10, 2016
  • Author: Nicholas A Tritos, MD, DSc, MMSc, FACP, FACE; Chief Editor: George T Griffing, MD  more...
  • Print
Treatment

Medical Care

Evaluation and therapy can usually be implemented on an outpatient basis. Inpatient evaluation and treatment may be necessary for patients with congenital heart disease or acute adrenocortical insufficiency.

All postpubertal-age patients with Kallmann syndrome and idiopathic hypogonadotropic hypogonadism are candidates for gonadal steroid replacement therapy in the absence of specific contraindications. Additional therapies to restore fertility can be implemented on request.

Behavioral modification and psychological counseling may benefit individuals with hypothalamic amenorrhea. Such approaches should be offered before estrogen replacement therapy.

Medical therapies are used to treat associated conditions, including osteoporosis, adrenocortical insufficiency, congenital heart disease, and neurologic disorders.

Next:

Surgical Care

Assisted reproductive technologies, including in vitro fertilization (IVF), zygote intrafallopian transfer (ZIFT), and gamete intrafallopian transfer (GIFT), have been used successfully when male patients with Kallmann syndrome or idiopathic hypogonadotropic hypogonadism do not achieve adequate sperm counts on either GnRH or gonadotropin therapy.

Patients with Kallmann syndrome and congenital heart disease may need corrective surgery. A detailed description of the pertinent procedures is beyond the scope of this review.

Patients with cleft lip or palate also need surgical correction.

Previous
Next:

Consultations

Adult or pediatric specialists should be consulted, depending on the patient's age.

Consultations include the following:

  • Endocrinologist and reproductive endocrinologist
  • Cardiologist
  • Cardiothoracic surgeon and pediatric surgeon
  • Neurologist and psychiatrist
  • Otolaryngologist
  • Dermatologist
Previous
Next:

Diet

No dietary restrictions are required in the absence of congenital heart disease. Salt restriction (adult Na+ intake < 2 g/d) is advised for patients with congestive heart failure.

All patients must ensure an adequate calcium (1200-1500 mg/d) and vitamin D (800-1000 U/d) intake, especially if they are osteopenic. Dietary supplements may be necessary for patients to achieve these goals.

Previous
Next:

Activity

Routine activity restrictions are not necessary; however, patients with osteoporosis need to avoid high-impact sports and situations conducive to falls.

Activity restrictions are also appropriate in patients with certain forms of congenital heart disease or seizures.

Previous