Kallmann syndrome

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Kallmann syndrome

Reproductive system

Male and female reproductive system disorders

Precocious puberty

Delayed puberty

Klinefelter syndrome

Turner syndrome

Androgen insensitivity syndrome

5-alpha-reductase deficiency

Kallmann syndrome

Male reproductive system disorders

Hypospadias and epispadias

Bladder exstrophy

Priapism

Penile cancer

Prostatitis

Benign prostatic hyperplasia

Prostate cancer

Cryptorchidism

Inguinal hernia

Varicocele

Epididymitis

Orchitis

Testicular torsion

Testicular cancer

Erectile dysfunction

Male hypoactive sexual desire disorder

Female reproductive system disorders

Amenorrhea

Ovarian cyst

Premature ovarian failure

Polycystic ovary syndrome

Ovarian torsion

Krukenberg tumor

Sex cord-gonadal stromal tumor

Surface epithelial-stromal tumor

Germ cell ovarian tumor

Uterine fibroid

Endometriosis

Endometritis

Endometrial hyperplasia

Endometrial cancer

Choriocarcinoma

Cervical cancer

Pelvic inflammatory disease

Urethritis

Female sexual interest and arousal disorder

Orgasmic dysfunction

Genito-pelvic pain and penetration disorder

Mastitis

Fibrocystic breast changes

Intraductal papilloma

Phyllodes tumor

Paget disease of the breast

Breast cancer

Hyperemesis gravidarum

Gestational hypertension

Preeclampsia & eclampsia

Gestational diabetes

Cervical incompetence

Placenta previa

Placenta accreta

Placental abruption

Oligohydramnios

Polyhydramnios

Potter sequence

Intrauterine growth restriction

Preterm labor

Postpartum hemorrhage

Chorioamnionitis

Congenital toxoplasmosis

Congenital cytomegalovirus (NORD)

Congenital syphilis

Neonatal conjunctivitis

Neonatal herpes simplex

Congenital rubella syndrome

Neonatal sepsis

Neonatal meningitis

Miscarriage

Gestational trophoblastic disease

Ectopic pregnancy

Fetal hydantoin syndrome

Fetal alcohol syndrome

Reproductive system pathology review

Disorders of sex chromosomes: Pathology review

Prostate disorders and cancer: Pathology review

Testicular tumors: Pathology review

Uterine disorders: Pathology review

Ovarian cysts and tumors: Pathology review

Cervical cancer: Pathology review

Vaginal and vulvar disorders: Pathology review

Benign breast conditions: Pathology review

Breast cancer: Pathology review

Complications during pregnancy: Pathology review

Congenital TORCH infections: Pathology review

Disorders of sexual development and sex hormones: Pathology review

Amenorrhea: Pathology Review

Testicular and scrotal conditions: Pathology review

Sexually transmitted infections: Warts and ulcers: Pathology review

Sexually transmitted infections: Vaginitis and cervicitis: Pathology review

HIV and AIDS: Pathology review

Penile conditions: Pathology review

Assessments

Kallmann syndrome

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Flashcards

Kallmann syndrome

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Questions

USMLE® Step 1 style questions USMLE

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A 14-year-old boy presents to his family physician accompanied by his parent due to concerns regarding delayed puberty. The patient’s parent expresses concern about a lack of secondary male sex characteristics compared to his sibling of the same age. The patient has a reserved demeanor and answers questions with one-word answers. The patient gets mostly C’s and some D’s in school. He has been picked on since starting school for his inability to smell when other students intentionally break wind in his presence. On physical examination, height and weight are near the 60th percentile for his age. The patient has absent pubic hair and small testes. A deficiency of which of the following hormones is most directly responsible for this patient’s clinical presentation?  

External References

First Aid

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Follicle-stimulating hormone (FSH)

Kallmann syndrome p. NaN

Gonadotropin-releasing hormone (GnRH)

Kallmann syndrome p. NaN

Hypogonadism

Kallmann syndrome p. 663

Infertility

Kallmann syndrome p. 663

Kallmann syndrome p. 513, 663

Puberty

Kallmann syndrome and p. 663

External Links

Transcript

Contributors

Sam Gillespie, BSc

Kallmann syndrome is an endocrine disorder caused by a decrease in sex hormones, either testosterone in males or estrogen and progesterone in females. That decrease leads to a failure to start or complete puberty.

The syndrome is named after Dr. Franz Kallmann, the geneticist who first described it.

Normally, during fetal development there’s a region of the brain called the olfactory placode. Two groups of neurons emerge from that region.

The first group contains olfactory neurons that eventually help with sensing smells.

These neurons migrate down from the olfactory placode and get embedded in the cribriform plate, which is a bone plate that separates the nasal cavity from the brain, forming the olfactory bulb.

The second group contains neurons that release gonadotropin-releasing hormone.

And these neurons migrate through the cribriform plate, and settle in the hypothalamus.

The hypothalamic-pituitary-gonadal axis is a system of hormone signaling between the hypothalamus, pituitary gland, and gonads, either the testes or ovaries, to control sexual development and reproduction.

Gonadotropin-releasing hormone is released into the hypophyseal portal system, which is a network of capillaries connecting the hypothalamus to the hypophysis, or pituitary.

When gonadotropin-releasing hormone reaches the pituitary gland, it stimulates cells in the anterior pituitary, called gonadotrophs, to release gonadotropin hormones, luteinizing hormone and follicle-stimulating hormone into the blood.

Sources

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Harrison's Endocrinology, 4E" McGraw-Hill Education / Medical (2016)
  6. "Clinical Genetic Testing for Kallmann Syndrome" The Journal of Clinical Endocrinology & Metabolism (2013)
  7. "Congenital Hypogonadotropic Hypogonadism and Kallmann Syndrome: Past, Present, and Future" Endocrinology and Metabolism (2015)
Elsevier

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