Adolescent and paeds Flashcards
Commonest cause of precocious puberty in girls is :
a) CAH
b) Ovarian
c) Adrenals
d) Tumours
e) Idiopathic
e) Idiopathic
17 year old sexually active on COCP 12 months with no problem now complains of vaginal spotting, BHCG negative. What next?
a) add 1.25 mg conjugated oestrogen to pill
b) change to higher dose oestrogen pill
c) change to higher dose progesterone pill
d) swab for genital tract infection
d) swab for genital tract infection
- A woman had an amniocentesis to exclude Downs syndrome and was found to have a normal 46 XY fetus. When the child was delivered it had normal female external genitalia. What is the most likely explanation?
a) lab error
b) mullerian agenesis (MRHS)
c) androgen insensitivity
c) androgen insensitivity
- Regarding transverse vaginal septum choose the correct option.
a) occurs at the junction of lower 2/3 and upper 1/3 of vagina
b) occurs commonly with abnormalities of upper Mullerian system
c) associated with inutero DES exposure
d) autosomal recessive inheritance
e) treated with Tompkin’s procedure
c) associated with inutero DES exposure
- An 18 yr old girl presents with primary amenorrhoea, no breast development and scant pubic hair. Examination shows normal vagina and vulva with a hypoplastic cervix and uterus. The karyotpye is 46 XY. The most likely diagnosis is:
a) pure gondal dysgenesis -
b) androgen insensitivity -
c) Noonan’s syndrome
d) CAH
a) pure gondal dysgenesis -
- You are performing a laparotomy for a ruptured right ectopic pregnancy in a 17 yo. A 10cm right ovarian cyst is noted. Left ovary appears normal. Optimal management is:
a) RSO
b) Aspiration of cyst
c) Cystectomy
d) Cystectomy and biopsy of other ovary
e) R oopherectomy
c) Cystectomy
- A 15 yr old girl presents with pelvic pain and an US shows a 4 cm ovarian cyst. What is the commonest cause?
a) Dermoid cyst
b) Follicular cyst
c) Corpus luteal cyst
d) Serous adenoma
e) Endometrioma
b) Follicular cyst
- 6 yo child with persistant PV bleeding. Next step.
a) EUA
b) Report re sexual abuse
c) Rectal exam to exclude foreign body
d) US pelvis
e) Swabs and antibiotics
a) EUA
- A child is born with ambiguous genitalie. What is the first test to perform?
a) U and E’s
b) Karyotype
c) Choose girl’s name
d) Check corticosteroids
a) U and E’s
CAH – congenital adrenal hyperplasia - Life threatening. Electrolyte imbalance from the ‘salt-losing’ type of CAH can appear within a few days of birth, and occurs in 75% those with virilizing adrenal hyperplasia. Salt-losing crisis develops 5-15 days after birth, with a refusal to feed, FTT, apathy, vomiting, then addisonian like crisis
- 14 yo bought by mother because of lack of periods. Which will be most helpful in establishing diagnosis?
a) Tanner stage 3 brest development
b) Mild obesity
c) Waist:hip ratio 0.6
d) Acanthosis nigricans
a Tanner stage 3 brest development
- A 160 cm normotensive 15 yo girl has been progressively virilized since age 7. Now shaving face regularly. OE clitoromegaly, posterior scrotal fusion, cervix present, no adnexal masses, tanner 2 breast, ammenorrhoeic. Karyotype most likely to be:
a) XX
b) XX/XY
c) XO
d) XXY
e) XY
a XX
A YES. Normal female karyotype. Likely late-onset CAH, this is the nonclassic type of 21-hydroxylaste def, represents a partial def, producing a late-onset, milder hyperandrogenemia. Typically presents at or just after puberty
- A tall eunachoid 18 yo girl is found to have 46 XY karyotype. OE sparse pubic and axillary hair, tanner stage 2 breasts, normal vagina and vulva, hypoplastic cervix and uterus. Likely cause of intersexd:
a) true gonadal dysgenesis
b) true hermaphrodite
c) testicular feminisation / AIS
a) true gonadal dysgenesis ( has mullerian structures but decreased testosterone and AMH)
a) true gonadal dysgenesis ( has mullerian structures but decreased testosterone and AMH)
b) true hermaphrodite ( mixed gonadal dysgenesis ) –dysgenetic testis on one side and streak gonad on the other
c) testicular feminisation / AIS ( no mullerian structures
The following are all causes of precocious puberty except:
a. astrocytoma
b. Frolich’s syndrome
c. Neruofibromatosis
b. Frolich’s syndrome
A IS. 20-30% of central PP cases are due to an intracranial mass lesion. Commonest are hypothalamic harmatomas, optic nerve gliomas, suprasellar arachnoid cysts, hydrocephalus, germinomas, and other sellar/suprasellar lesions. Central PP secondary to a astrocytoma also occurs.
B NOT. Usually presents with delayed puberty
C IS. NF type 1 is associated with PP, in those with optic chiasm tumours. NF is an inherited disorder in which nerve tumours (neurofibromas) form in the skin, subcut tissue, and nerves and spinal cord
Frolich’s syndrome
• Rare childhood metabolic disorder
• Characterised by feminine obesity, growth retardation, retarded sexual development, atrophy or hypoplasia of gonads, altered secondary sexual characteristics
• Usually assoc with tumours of the hypothalamus
McCune-Albright syndrome
• Genetic disease that affects the bones and colour of the skin
• Can get café-au-lait spots, but this different to neurofibromatosis
For the patient described give the most likely clinical findings:
- Turners Syndrome, 18 yrs old, no treatment
- AIS 18 yrs old
a) absent uterus, breasts underdeveloped
b) absent uterus, breasts developed
c) uterus present, breasts undeveloped
d) uterus present, breasts developed
c) uterus present, breasts undeveloped
Turner’s
• Gonadal dysgenesis, get streak ovaries and poor breast development
• No testes thus no AMH thus will get mullerian structures thus uterus present
14: b) absent uterus, breasts developed
- 13 yo girl presents with heavy menstrual bleeding for 6 months. What is the next most appropriate step?
a) combined OCP
b) hysteroscopy, D& C
c) cyclical progesterone
d) coagulation profile
e) expectant management
d) coagulation profile