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Kallmann Syndrome

Elyas Parsa, Osteopathic Doctor and Wilmer E Garcia Ricardo, M.D. Reviewed 06/2022
 


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

  • Females: primary amenorrhea and history of multiple fractures

  • Males: present early in life with micropenis or cryptorchidism or delayed maturation at puberty; patients may or may not b...

TREATMENT

In chronic GnRH deficiency, patients may demonstrate pituitary hypoplasia or deficient GnRH receptors, either of which may cause them to be poorly responsive to exogenous GnRH. To determine ...

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, diagnos...

CODES

ICD10

E23.0 Hypopituitarism 

SNOMED

  • 93559003 hypogonadism with anosmia (disorder)

  • 33927004 Hypogonadotropic hypogonadism (disorder)

  • 123953004 Idiopathic hypogonadotropic hypogonadism (disorder)

  • 361116...

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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