Delayed puberty

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Delayed puberty

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

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Delayed puberty

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Delayed puberty

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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?  

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Transcript

Content Reviewers

Rishi Desai, MD, MPHYifan Xiao, MD

Robyn Hughes, MScBMC

Contributors

Tina Collins

Sam Gillespie, BSc

Puberty is the time in an individual’s life when they physically become sexually mature and able to have children. Generally speaking, it’s considered delayed if puberty hasn’t started for a female by age 13 and for a male by age 14.

The hypothalamic (HYpo-tha-lamb-ic)-pituitary-gonadal (Go-nad-al) axis is a system of hormonal signaling between the hypothalamus, pituitary gland, and gonads, the gonads are either the testes or the ovaries, and this will control sexual development and reproduction. Gonadotropin (Go-nad-ah-tro-pin) -releasing hormone is released into the hypophyseal (high-poth-ah-see-al) portal system, which is a network of capillaries connecting the hypothalamus to the hypophysis (high-pof-o-sis), or pituitary. When gonadotropin(Go-nad-ah-tro-pin) -releasing hormone reach the pituitary gland, it stimulates cells in the anterior pituitary, called gonadotrophs (Go-nad-a-trofs), to release gonadotropin hormones: luteinizing hormone and follicle-stimulating hormone which then enter the blood. These gonadotropin hormones then stimulate the gonads to produce sex specific hormones. These are estrogen and progesterone in females and testosterone is the major sex specific hormone in males.

Early on in male development, testosterone helps the external sex organs to differentiate into male genitals and causes the testes to descend from the abdomen into the scrotal sac. Beginning at puberty, the Leydig cells of the testes respond to the luteinizing hormone by converting more cholesterol into testosterone. In addition, the Sertoli cells of the testes respond to follicle-stimulating hormone by producing more sperm. The major sex specific hormones in women are estrogen and progesterone, and they are produced by the ovarian follicles that are scattered on the ovaries. Each ovarian follicle is made up of a ring of granulosa and theca cells surrounding a primary oocyte at its core. Beginning at puberty, theca cells respond to luteinizing hormone by producing androstenedione, an androgen. Then, the granulosa cells respond to follicle stimulating hormone by converting the androstenedione into estrogen and progesterone.

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. "Pediatric Endocrinology Update: An Overview" Hormone Research in Paediatrics (1998)
  6. "Pathology or Normal Variant: What Constitutes a Delay in Puberty?" Hormone Research in Paediatrics (2014)
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