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Kallmann Syndrome

Tipsuda Junsanto-Bahri, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Kallmann syndrome (KS) is hypogonadotropic hypogonadism caused by deficient gonadotropin-releasing hormone (GnRH).

  • Patients present with incomplete or absent puberty, infertility, and...

DIAGNOSIS

HISTORY

  • Both genders will display delayed sexual maturation, but the classic presentations differ depending on gender.

    • Female patients: primary amenorrhea and history of multiple fractures

    • Male...

TREATMENT

  • There are multiple management approaches and individual patient considerations that must be taken into account in determining an appropriate course.

  • In chronic GnRH deficiency, patients may d...

ONGOING CARE

DIET

In addition to weight-bearing exercise, patients should have adequate calcium intake in order to achieve optimal bone density. 

PATIENT EDUCATION

  • Patients with autosomal dominant form o...

REFERENCES

1
Boehm U, Bouloux PM, Dattani MT, et al. Expert consensus document: European consensus statement on congenital hypogonadotropic hypogonadism—pathogenesis, diagnosis and treatment. Nat Rev E...

CODES

ICD10

E23.0 Hypopituitarism 

ICD9

253.4 Other anterior pituitary disorders 

SNOMED

  • 93559003 hypogonadism with anosmia (disorder)

  • 33927004 Hypogonadotropic hypogonadism (disorder)

  • 123953004 Idiopathic h...

CLINICAL PEARLS

  • KS represents a specific variant of hypogonadotropic hypogonadism resulting from failed migration of hypothalamic neurons from the olfactory placode during embryologic development.

  • KS s...

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