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General Medicine

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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Related Images
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Organization of the olfactory system in the nose and inhaled odorants.
Organization of the olfactory system in the nose and inhaled odorants.
Structure of the olfactory epithelium.
Structure of the olfactory epithelium.
Central projections of the olfactory system.
Central projections of the olfactory system.
Figure 8.19 Central olfactory pathways. Axons of the olfactory tract branch and enter many regions of the forebrain, including the olfactory cortex. The neocortex is reached only by a pathway that synapses in the medial dorsal nucleus of the thalamus.
Figure 8.19 Central olfactory pathways. Axons of the olfactory tract branch and enter many regions of the forebrain, including the olfactor...
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