Endocrine Problems in Childhood Flashcards
What are the most important pubertal stages (Tanner Stages)?
- B2 in a girl
- T 3-4ml in a boy
These are the earliest signs of puberty and when present will usually progress onwards
When is early and delayed puberty indicated?
Boys:
• Early < 9 yrs (rare)
• Later > 14yrs (common, esp. in CDGP)
Girls:
• Early < 8yrs
• Late > 13yrs
What is the relationship between constitutional delay of growth (CDGP) and puberty?
- Boys mainly
- Family history in dad or brothers (difficult to obtain!)
- Bone age delay
- Need to exclude organic disease
Name some other causes of delayed puberty
- Gonadal dysgenesis (Turner 45X, Klinefelter 47XXY)
- Chronic disease (Crohn’s, asthma)
- Impaired HPG axis (septo-optic dysplasia, craniopharyngioma, Kallman’s syndrome)
- Peripheral (cryptorchidism, testicular irradiation)
What is thelarche?
Onset of secondary (postnatal) breast development, usually occurring at the beginning of puberty in girls
What is premature thelarche?
Breast tissue develops early—sometimes in the first two years of life, sometimes later, and occasionally even in the neonatal period—and then persists until the true onset of puberty
What is pubarche and adrenarche?
Pubarche is the appearance of sexual hair. Adrenarche is the onset of androgen-dependent body changes such as growth of axillary and pubic hair, body odor, and acne.
What is premature pubarche/adrenarche?
Variant of pubertal development in which pubic and/or axillary hair develop in a girl before age 8 or in a boy before age 9. The serum levels of adrenal androgens are normal, and there is no androgen- induced growth spurt.
What is the name used for early sexual development of PV bleeding?
Premature menarche
What are two secondary sexual characteristics of early sexual development?
- Exaggerated adrenarche
* Precocious pseudopuberty
What is central precocious puberty?
Early pubertal development
• Breast development in girls
• Testicular enlargement in boys
- Growth spurt
- Advanced bone age
What do you look for in the history of central precocious puberty?
- Acne, greasy skin/hair, body odour
- Voice change
- Mood swings
- Vaginal discharge/ bleeding
- Growth- acceleration?
- Neuro problems: Perinatal history, Headaches, Radiotherapy
- Drugs
- FHx: Parental pubertal timing
What is used for the examination of central precocious puberty?
Girls:
• Usually idiopathic
• Pituitary imaging
Boys:
• Look for underlying cause, i.e. brain tumor?
Also: • Fundi/neuro • BP • Skin • Pubertal assessment/genital exam - clitoromegaly?
What is the treatment of central precocious puberty?
GnRH
What is precocious pseudopuberty?
Incomplete puberty in which some of the milestones of puberty do not appear or fail to achieve the usual synchronicity. Development occurs despite low or prepubertal levels of FSH and LH. Ovarian or adrenal androgens may produce virilization in girls.
What are the features of precocious pseudopuberty?
- Gonadotrophin independent (low/prepubertal levels of LH and FSH)
- Abnormal sex steroid hormone secretion
- Virilasing or feminasing
- Clinical picture: secondary sexual characteristics
What is FSH?
Follicle-stimulating hormone
What is LH?
Luteinising hormone
What is the aetiology of precocious pseudopuberty?
- Feminising
- Adrenal/ovarian/testes tumour
- McCune-Albright syndrome
- Virilising
- Adrenal/ovarian/testes tumour
- Congenital adrenal hyperplasia (21-OHase def)
- Testotoxicosis
- Drugs
How is the sex of the baby determined if they have ambiguous genitalia?
- Do not guess the sex of the baby!
- Multidisciplinary approach (paed endo, surg, neonatologist, geneticist, psychologist)
- Exam: gonads?/ internal organs
- Karyotype
- Exclude Congenital Adrenal Hyperplasia! - risk of adrenal crisis is first 2 weeks of life
What are the causes of congenital hypothyroidism?
- Athyreosis/ hypoplastic/ ectopic
* Dyshormonogenic
How is congenital hypothyroidism detected and treated?
- Newborn screening
* Start treatment within first 2 weeks
What are causes of acquired hypothyroidism?
• Autoimmune (Hashimoto’s) thyroiditis - most common
• FH of thyroid/autoimmune disorders
• Childhood issues:
- Lack of height gain
- Pubertal delay (pr precocity)
- Poor school performance (but work steadily)
What are the boundaries of overweight and obesity in children?
• Overweight (BMI > 85th centile or SD
> 1.04)
• Obesity (BMI > 97.5th centile or SD > 2)
