Endocrine+Metabolic Flashcards

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1
Q

Describe the presentation and diagnosis of congenital adrenal hyperplasia

A
  • Presentation-2wk old w/vomiting, lethargy, mottling, dehydration, hypotension.
  • Dx-signs+sxs, +/- ambiguous genitalia (females), +/- newborn screen, BMP showing hyponatremia, hyperkalemia, and hypoglycemia
  • Because of the age this is someone you will do the full sepsis workup on
  • Girls often have ambiguous genitalia making them easier to diagnose, for this reason boys tend to present much sicker
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2
Q
  • What is the treatment of congenital adrenal hyperplasia?

- What labs should you obtain if a diagnosis of CAH has never been obtained?

A
  • IV fluids, 2mg/kg hydrocortisone (max 100mg), glucose bolus, tx hyperkalemia if present w/calcium gluconate +/- insulin, EKG
  • If diagnosis of CAH has never been made obtain serum cortisol and ACTH prior to giving hydrocortisone
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3
Q

What is the rule of 50 for glucose bolusing?

A
  • Just multiply up to 50
  • D10W=5mL/kg (infant)
  • D25W=2mL/kg (toddler)
  • D50W=1mL/kg (adolescent)
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4
Q

Describe the symptoms and estimated volume loss of mild, moderate, and severe dehydration

A
  • Mild~3-5% fluid loss~50mL/kg-normal exam but thirsty with dry mucous membranes
  • Moderate~6-9% fluid loss~100mLkg-decreased urine output and tears, cap refill >2 seconds, dry mucous membranes, tachycardia, weak pulse
  • Severe~>9% fluid loss~150mL/kg-AMS, sunken fontanelle in infant, decreased skin turgor, decreased BP
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5
Q

When should you obtain labs in dehydration and what labs should you consider obtaining?

A
  • Severe dehydration or moderate dehydration with a weird history or exam findings
  • Obtain glucose, BMP, urinalysis vs dipstick
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6
Q

How should you treat dehydration?

A
  • Tx nausea with zofran
  • trail oral rehydration of 50-100mL/kg over 2-4 hours
  • If fails oral rehydration give NS bolus and D10NS at maintenance
  • Indications to admit are PO intolerance and clinical exam
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7
Q

What are potential causes of hypoglycemia?

A

CAH, ketotic hypoglycemia (starvation state), beta blocker ingestion, insulin overdose, sepsis, ethanol ingestion, inborn errors of metabolism

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8
Q
  • How does hypoglycemia present?
  • What are additional labs you should consider obtaining?
  • What are treatment options for hypoglycemia?
A
  • Presentation-AMS, seizure, hypotonia, apnea
  • Addtl labs-Growth hormone, serum cortisol+ACTH, serum insulin
  • Tx options-Glucose bolusing, glucagon
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9
Q

What are potential causes of hyponatremia?

How does hyponatremia present?

A
  • Causes-SIADH, CAH, increased free water ingestion

- Sxs-lethargy, vomiting, respiratory failure, refractory seizures

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10
Q

How should hyponatremia be treated?

  • What are etiologies of hyponatremia?
  • When do you worry about complications of over aggressive correction?
A
  • if symptomatic give 5mL/kg 3% saline over 20 minutes and repeat BMP
  • Vomiting, SIADH, increased h20 consumption, improperly mixed formula, CAH, diarrhea
  • Do not correct Na faster than 8mEq in a 24 hour period as to avoid developing central pontine myelinolysis
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11
Q

What are causes of hypocalcemia?
What are sxs?
What is the treatment?

A
  • causes-given excess phosphate (fleets enemas, excess cow’s milk), infant of a diabetic mother, digeorge syndrome, Ricketts, chronic renal failure
  • Sxs-lethargy, poor feeding, jitteriness, seizures, vomiting
  • Tx-1mL/kg of calcium gluconate slow IV
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12
Q
  • Give a typical presentation of an inborn error of metabolism
  • What can be seen with labs and PE?
A

1mo w/ lethargy, seizure, vomiting with normal imaging. +/- hepatomegally, metabolic acidosis, peculiar odor

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13
Q
  • What is the initial workup and treatment of a suspected inborn error of metabolism?
  • What initial treatment will they often require?
A
  • WU-VBG, electrolytes, glucose, ammonia, lactate, UA, repeat glucose
  • Tx-IVF, replete glucose, tx seizures, give HCO3 if pH <7.1
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14
Q

What sxs should alert you to the possibility of DKA and what labwork should you obtain?

A
  • Sxs-abdominal pain, polyuria, polydypsia, N/V, recent weight loss
  • Workup-obtain loaded gas, UA, CBC, serum osms, HgbA1C, LFTs, Mg, Phos, amylase, lipase
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15
Q
  • How is the diagnosis of DKA made?

- What other lab work may be altered?

A
  • Glucose>200, ketonuria vs serum beta hydroxybutyrate, metabolic acidosis
  • may also see psuedohyponatremia 2/2 hyperosmolarity and hyperkalemia although total body K is down
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16
Q

What should everyone with DKA get?

How do you determine if you will be on the 2 bag system or giving subQ insulin?

A
  • Everyone should get a NS bolus!
  • if the serum bicarb>15 consider subQ insulin
  • if the serum bicarb<15 start the 2 bag system
17
Q

What is the total fluid rate when starting the 2 bag system for DKA?
What parameters determine what the bag compositions are?
What determines the starting insulin infusion rate?

A
  • 1.5 x maintenance
  • Bag compositions are determined by the serum K concentration, serum glucose
  • insulin infusion rate determined by blood glucose and the patient’s age
18
Q

What monitoring needs to be done for anyone with DKA?

What are potential complications of DKA?

A
  • hourly neuro checks, hourly glucose, q2 hourly loaded gases
  • cerebral edema, pulmonary edema vs ARDS, rhabdomyolysis, renal failure
19
Q

Give early and late diagnostic criteria for cerebral edema following DKA

A
  • Early-vomiting, HA, drowsy, diastolic BP>90

- Late-AMS, Cushing’s Triad, incontinence

20
Q

What are risk factors for cerebral edema following DKA?

A

pH<7.1, pCO2 <20, >50mL/kg of gluid given within the first hour, high initial BUN, failure of Na to rise after glucose falls

21
Q

How should cerebral edema in DKA be treated?

A

mannitol, hypertonic saline, restrict IVF to 2/3rd maintenance

22
Q
  • What is the cause of rickets and what children are most at risk?
  • How do children with Rickets present?
  • What will be seen on imaging/
  • How is it treated?
A
  • Although there are many causes of rickets, the most common cause is inadequate dietary intake of vitamin D. Children at risk include malnourished children, vegetarians, premature infants, children who are not exposed to sunlight (important in vitamin D synthesis), children who are on certain medications (e.g., furosemide, phenytoin or phenobarbital) and patients with malabsorptive or renal disease.
  • This inadequate bone mineralization is most clinically evident in areas of rapid bone growth, leading to widening and irregularities of the epiphyses and metaphyses of growing bones. This irregular growth bone growth frequently leads to the knobby chest wall (“rachitic rosary”) and splaying and flaring of the wrists.
  • In the ED, x-rays reveal these characteristic bone irregularities.
  • In vitamin D-deficiency rickets, the calcium and phosphate levels are normal or low, and treatment is with oral vitamin D supplements. In severe cases, calcium therapy may need to be initiated before starting vitamin D
23
Q
  • What are risk factors for neonates who will develop neonatal thyrotoxicosis?
  • What are symptoms of thyrotoxicosis?
  • How is it treated?
A
  • almost always seen in children whose mothers have Graves’ Disease. Overall, however, the incidence of hyperthyroidism in children is relatively low. The vast majority of pediatric patients with hyperthyroidism have Graves’ Disease
  • More severely affected neonates have are hypoactive, feed poorly, and are frequently constipated. These infants are frequently hypothermic, jaundiced, and have large posterior fontanelles. They later develop coarse facies and enlarged tongues if left untreated.
  • Treatment of thyroid storm includes beta-blockers in order to minimize sympathomimetic symptoms, antithyroid medications (e.g., propylthiouracil) FOLLOWED by iodine so that the iodine is not incorporated into the synthesis of new hormone, glucocorticoids (which decreases the peripheral conversion of T4 to T3), and cooling measures for hyperthermia.
24
Q

What is witch’s milk?

A

A white discharge from a neonate’s nipples (commonly known as “witch’s milk”) is normal and is due to the withdrawal from maternal estrogen

25
Q
  • How would you make the diagnosis of physiologic leukorrhea in an adolescent female?
  • Is any workup required
A
  • physiologic leukorrhea is white vaginal discharge not associated with itch or odor that can occur in a female that has never been sexually active undergoing puberty 6-12 months prior to menarche. So breast budding would have likely occurred at least 2 years prior to presentation.
  • No workup required if all of these conditions met and you trust that the child has never been sexually active
26
Q
  • What is the normal sequence of puberty in boys and girls?
  • At what age ranges does puberty typically start at?
  • How long does puberty typically take to complete?
A
  • The pubertal sequence of events is
    • Girls: Breast development → Pubic hair → Peak height velocity → Menarche.
    • Boys: Testicular enlargement → Pubic hair → Increased penile length → Peak height velocity.
  • Puberty starts between 8 and 13 years of age for girls and 9 and 14 years of age for boys.
  • Puberty takes about 4 years to complete but can vary anywhere from 1.5 to 8 years
27
Q

How is pubic hair tanner staged in boys and girls?

A

-Stage 1-No hair
-Stage 2-Straight, downy hair along labia or base of
penis
-Stage 3-Curlier, pigmented, coarser hair over mons
pubis
-Stage 4-Adult quality hair; does not extend to inner
thighs
-Stage 5-Adult hair with spread to inner thighs

28
Q

How is breast development tanner staged?

A
  • Stage 1-No breast buds; areola conforms to chest wall
  • Stage 2-Breast bud; areola widens
  • Stage 3-Enlargement and elevation of the breast contour beyond the areola
  • Stage 4-Areola/papilla form a mound above the breast contour
  • Stage 5-Adult female breast; areola in line with breast contour
29
Q

How is testicular and penile development tanner staged?

A
  • Stage 1-Testes volume <4 mL; penis child-like
  • Stage 2-Testes enlarge, volume 4-8 mL; scrotum reddens; penis no change
  • Stage 3-Testes enlarge, 10-15 mL; scrotum enlarges; penile length increases
  • Stage 4-Testes enlarge, 15-20 mL; scrotum enlarges; penis increases in length and circumference
  • Stage 5-Testes >25 mL; adult male genitals