Endocrine & Female Reproductive Flashcards
Type 1 Diabetes Mellitus
Pathophys
- -T cell mediated autoimmune destruction of pancreatic beta cells –> low insulin
- -hyperglycemia –> osmotic diuresis, glucosuria
Features
- -polyuria, polydipsia
- -polyphagia, weight loss
- -Most initially present with diabetic ketoacidosis.
- What type of acidosis?
- What urine finding?
What is the main tx?
Type 1 DM
Pathophys
- -T cell mediated autoimmune destruction of pancreatic beta cells
- -low insulin, catabolic state
- -hyperglycemia –> osmotic diuresis, glucosuria
Features
- -polyuria, polydipsia
- -polyphagia, weight loss
- diabetic ketoacidosis
- -hyperglycemia, ketonuria, increased AG, low HCO3, acidosis, high serum osmolality
Tx
- -insulin
- -diabetic ketoacidosis: insulin, rehydration
Complications associated with maternal hyperglycemia
What is the name of the complication that features neurologic impairment and hypoplasia of the pelvis and lower extremities?
Complications associated with maternal hyperglycemia
Metabolic
–hypoglycemia
Size
–macrosomnia, LGA
Cardiovascular
- -cardiomegaly
- -VSD, ASD, transposition
Respiratory
–tachypnea, RDS
Heme
- -polycythemia
- -renal vein thrombosis (due to polycythemia)
GI
–small left colon syndrome
Neuro
–caudal regression syndrome
Precocious Puberty
Central v Peripheral
- -which one has high FSH and LH?
- -which one has low FSH and LH?
- -which one is characterized by low LH levels before and after GnRH stimulation test?
Precocious Puberty
Central Precocious Puberty
- -early activation of H-P-O axis
- -high FSH and LH
- needs brain imaging
- Tx: GnRH analog
Peripheral Precocious Puberty
- -gonadal/adrenal release of sex hormones
- -low FSH and LH
- LH levels do NOT increase in response to GnRH test
- -CAH, ovarian cyst
Congenital Adrenal Hyperplasia
MoI?
Congenital Adrenal Hyperplasia
MoI: AR
21-alpha hydroxylase deficiency
Pathophys: shunting to androgen synthesis
- -masculinized external genitalia in females
- -salt-wasting (classic, infantile presentation)
- -precocious puberty, normal electrolytes (late-onset)
Labs
- -increased 17-OH progesterone
- -low aldosterone
Tx
- -hydrocortisone
- -fludrocortisone (salt-wasting)
Refeeding Syndrome
What is the main hormonal derangement?
Refeeding Syndrome
refeeding –> increased insulin –> cellular uptake of P, K, Mg, thiamine
- -low serum P –> decreased ATP/energy
- -low serum K, Mg –> arrhythmia, CHF, seizures
- -low serum thiamine –> Wernicke’s
Diabetic Ketoacidosis in T1DM
High or low?
- -plasma osmolality
- -plasma Na
Features
- -hyperglycemia
- -polyphagia
- -AG metabolic acidosis
- -polyuria, polydipsia
- -blurry vision
- -coma
Pathophysiology of Diabetic Ketoacidosis in T1DM
- low insulin –> FA oxidation –> ketone acids
- -AG metabolic acidosis - low insulin –> hyperglycemia
- -osmotic diuresis –> polyuria
- -dehydration –> polydipsia - hyperglycemia, diuresis –> increased plasma osmolality –> H2O shifts from ICF to ECF
- -coma
- absent uterus/upper vagina; cryptorchid testes; breasts present; minimal axillary/pubic hair; 46 XY
- absent/rudimentary uterus/upper vagina; normal ovaries; breasts present; normal axillary/pubic hair; 46 XX
- abnormal vagina; normal uterus and ovaries; breasts present; normal axillary/pubic hair; 46 XX
- normal vagina and uterus; streak ovaries; normal axillary/pubic hair; 45 X
Androgen-Insensitivity Syndrome
- -XL mut of androgen receptor
- -peripheral tissues unresponsive to androgens
Mullerian Agenesis Syndrome
–failure of mullerian duct system to differentiate into uterus, cervix, and upper vagina
Transverse Vaginal Septum
Turner Syndrome
Ovarian Tumors in Younger Women
secretes estrogen; presents with precocious puberty (eg, breast enlargement, pubic hair growth, growth spurt)
Granulosa Cell Tumor
- -secretes estrogen
- -“isosexual” precocious puberty
NB:
1. Sertoli-Leydig cell tumors secrete androgens
- Serous cystadenomas, mature teratomas, and dysgerminomas do NOT secrete estrogen or androgens
