LYW Endocrinology
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Created by:
AK1138 on September 16, 2011
Subjects:
pediatric boards, endocrinolgoy
Description:
Chapter 18: Is that an Orchidometer in your pocket?
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69 terms
Terms | Definitions |
|---|---|
 The presence of WHAT is required for formation of male external genitals in XY males? | Androgens |
| The presence of WHAT results in regression of female internal duct structure in XY males? | Mullerian inhibiting factor |
| How can you differientate between a micropenis and ambigious genitalia? | Micropenis will have a normally formed penis w/ normal positioned meatus and testes in the scrotal sac. Whereas with ambigious genitalia, you might not have either testes or the meatus in the proper spot. |
| If a female patient presents with clitoromegaly and palpable masses in the labial folds, what should you get? | A karotype, it is likely an XY patient with female phenotypical genitalia. Male pseudohermaphroditism, or testicular feminization |
| If a xy patient is diagnosed with pseudohermaphroditism, what else should you rule out? | 5-alpha-reductase deficiency (can't reduce testerone to DHT) or 17-ketosteroid-reductase deficiency (impaired conversion of androstenedione to testosterone). |
| What do the Mullerian (paramesonephric) ducts normally form? | The uterus, cervix, upper vagina and fallopian tubes |
| When are persistant Mullerian Ducts normally found in XY males? | When they have a hernia repair or cryptochidism repair and patient is noted to have fallopian tubes or a rudimentary uterus with otherwise normal genitalia (including testes) |
| What happen to the genitalia in testicular feminization/androgen insensitivity? | Inability to develop male external genitalia resulting in a blind ending vagina and no uterus, but they are genetically male (XY), peripheral receptors are resistant to testerosterone; testes often found in the inguinal canal |
| If a patient presents with a micropenis and hypoglycemia, what disease process should you be suspicious of ? | Be suspicious of panhypopituitarism. Need to also check to thyroid function. |
| What do you also need to be suspicious of if a phenotypical female presents of an x-linked disorder? | A genetic XY male who has androgen insensitivity. |
| A male patient who presents with in the newborn period with a shocky/septic picture, what disease process are you concerned about? | Congenital Adrenal Hyperplasia |
| If does the male genitalia look like in CAH? | Genitalia looks normal, but there can be excessive scrotal pigmentation |
| What does the female genitalia look like in CAH? | Ambigiuous, The boards may describe it as 'ruggated labia' or 'clitoral hypertrophy' |
| If the boards is presenting a patient with growth delay, h/o hirsutism and amenorrhea (without a history of no medical care), what is the underlying condition? | CAH (late onset form) |
| What is the most common cause of CAH? | 21-hydroxylase deficiency |
| If the CAH is the 'salt-wasting' variety, what labwork will be abnormal? | Hyperkalemia, hyponatremia and elevated 17-hydroxyprogesterone |
| What medication treats congenital CAH? | Hydrocortisone hemisuccinate |
| Rate of growth prior to puberty? | 5-6 cm/year |
| During puberty, what is the peak growth velocity in girls? | 9-10 cm/year |
| If a boy presents with pubic hair, axillary hair and an enlarged penis, what it is this? | r/o an adrenal tumor |
| When does peak velocity occur in girls? | About 1.5 years before menarche; once they receive menarche they are within 1-2 inches of their adult height |
| If a patient presents with a sharp decline in the height percentile, weight normal for age but high for height, or delayed bone age as much as 2 years, what should you be suspicious of? | Growth Hormone Deficiency (GHD) |
| If a child presents with growth delay but is otherwise asymptomatic, what should you be wary of? | It could be crohn's diease. Crohn's can present w/ growth retardation before some the GI issues. Check and ESR to r/o it out first. |
| Is constitutional Delay more common in males or females? | Males |
| If a patient shows a decrease in growth rate (usually in the teen years) with a delayed onset of buttery and bone age below chronological age, what is this? | Constitutional growth delay |
| What disorder should you consider in any short female? | Turner's syn (sometimes doesn't present until puberty) Growth hormone can help |
| If a kid presents with a delayed bone age and short height and might have cold intolerance or constipation, what should you be suspicious of? | Hypothyroidsim |
| Boy that is tall with learning difficulties and small testes, what disorder should you consider? | Klinefelter's synd |
| What is the average intelligence in Klinefelter's? | Normal |
| Kid with a cardiac defect, tall and lower body is longer in proportion to upper, what disorder should you consider? | Marfan's synd |
| If a kid presents with being tall, overweight, and advanced bone age, what disorder should you consider? | High caloric intake (exogenous obesity) |
| If an infant presents with poor feeding, jaundice, constipation, hypotonia, hoarse cry, macroglossia, umbilical hernia and/or enlarged anterior fontanelle, what disorder should you consider? | Congenital hypothyroid |
| What is Grave's disease caused by? | An IgG antibody, known as "thyroid-stimulating immunoglobulin" |
| If a kid presents with emotional liability, weight loss, sleep issues, bulging eyes, and/or lid lag, what disorder should you consider? | Grave's disease |
| What disease do you need to consider if a kid is presenting with Emotional liability and hyperactivity before deciding it's psych d/o? | Grave's disease |
| How do you treat Grave's disease? | PTU, radioactive iodine. Acute: propanolol |
| If you find a thyroid nodule in a kid, what do you do? | Ultrasound to determine size and location? |
| Which is a better, a cold or hot nodule in the thyroid? | A cold nodule is a bad sign. |
| If you are presented with a patient with a thyroid mass, what is the most informative test you can order? | A fine needle biopsy |
| IF you evaluate a child with acanthosis nigricans and is obese, what is the most likely lab finding? | Low HDL cholesterol |
| If exercise and nutrition doesn't help a type 2 diabetic child, what medication can you use? | Metformin |
| How can type 1 and type 2 DM be distinguished from each | Measuring autoantibioides against pancreatic beta cells, which are only present in type 1 |
| What are the five criteria for metabolic syndrome? | 1) high triglycerides 2) low HDL 3) hypertension, 4)hyperglycemia, (glu >100) 5) truncal obesity |
| What is the best way to check compliance long in a diabetic? | Hemoglobin A1c |
| What is the first step in treating DKA? | Fluid replacement, a bolus of 20 cc/kg of NS in the 1st hour |
| If the sodium falls with rehydration of DKA, what should you consider? | SIADH (or poor management). DKA is usually a sodium deficiency |
| Should you give K if the K value is normal in a present DKA patient? | Yes, they are overall depleted in K |
| When do you add glucose to the IVs in DKA? | When the sugars drop below 300 |
| When do you stop the IV insulin in DKA? | When the patient has stabilized, pH > 7.3 and bicarb >15 and sugar <300 |
| Why do you NOT want to rehydrate a DKA too quickly? | Don't want to cause cerebral edema! |
| How do you treat Hyperosmolar diabetic coma (patient w/ T2DM and high Osm and Glu)? | Same way as DKA |
| What are some causes that cause hypercalcemia? | Williams syndrome, Ingestion (Vit D & A intoxication, thiazides), Skeletal d/o (dysplasias & immobilization), Hyperparathyroidism (WISH) |
| How is the best way to treat hypercalcemia (especially if the kid is immobilized)? | High volume fluid, lasix and EKG monitoring |
| If a patient presents with painful muscles spasms, generalized seizures, vomiting, or prolonged QT interval on EKG, what disorder should you consider? | Hypocalcemia |
| What are the causes of Hypocalcemia? | Pseudohypoparathyroidism, Nutritional defiency, Immune deficiency (di-george), Nephrotic syndrome (lower albumin level), Kidney d/o |
| Hypocalcemia and hypophosphatemia correlate with what disorder? | Vitamin D deficiency |
| Hypocalcemia and hyperphosphatemia correlates with what disorder? | Hypoparathyroidism |
| A kid presenting bone pain, anorexia, decreased growth rate, widening of wrist and knees, bowed legs, enlarged costochondral junctions, what disorder should you consider? | Rickets |
| If a kid presents with delayed eruption of teeth, what disorder should you consider? | Rickets |
| Serum alkaline phosphatase levels are what in rickets? | ELEVATED |
| Vitamin D deficient rickets is most likely to occur when? | Inadequate Vitamin D intake (breast-fed baby, especially if mom is vegetarian) or Malabsorption, or lack of exposure to sunlight |
| What labs do you expect in Vitamin D rickets? | Elevated PTH, decreased serum calcium and phosphate, decreased 25-OH Vit D |
| How do you treat nutritional rickets? | With vit D and calcium |
| If you have a kid that appears to have Vit D defiency causing rickets, and doesn't improve with Vit D/Ca, what else should you suspect? | Vitamin D dependent rickets type 1 (autosomal recessive), |
| If labs show lows serum calcium and phosphate, normal 25-OH Vit D, but a very low 1,25-OH Vit D, what disorder should you consider? | Vit D dependent rickets, type 1 (Autosomal recessive) |
| How do you treat vitamin D dependent rickets, type 1 (autosomal recessive)? | Vitamin D2 and 1,25 dihydroxy Vit D |
| What is a common form of rickets in industrialized countries? | X-linked (Dominant) familial hypophosphatemia (excessive kidney losses of phosphate) |
| What is the treatment of X-linked familial hypophosphatemic rickets? | Phosphate supplement pulus 1,25 di-hydroxy-Vit D |
| What is the most specific risk factor for vitamin D deficient rickets in a premie? | Very low birthweight |
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