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12 Cards in this Set

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Vitamin-D- deficiency rickets is due to:

S/S?

Biochemical hallmark? Other lab findings?
lack of sunlight AND dietary Vitamin D - genetic absence of renal 1-A-hydroxylase (converts 25(OH)D --> 1,25(OH2)D

bowed legs, large and soft cranium, skeletal deformities

low serum (OH)D
low P, elevated Alk Phos, PTH, normal serum Ca++
Explain how liver disease and anti-seizure meds affect vitamin D:

Deficiency in Vitamin-D resistant rickets, and lab findings:

Tx for vit-D dependent and resistant rickets?
decreased secretion of bile --> decreased ADEK absorption
decreased production, increased degradation of vitamin D

defect in vit-D receptor - low serum Ca++, P, elevated P, 1,25(OH)2D (active form)

dependent - vit D
resistant - Ca++ IV, PO
Defect and Lab values for XL low P rickets:

Treatment? Complications?
defect in PCT --> interferes with P absorption, vit D production

low serum P, high Alk Phos, normal Ca, PTH, 25(OH)D, 1,25(OH)2D, loss of phosphate in urine

PO4, Vit D

nephrocalcinosis, secondary hyper PTH
How is familial hypo-P rickets with calciuria different from XLH?

Tx?

Effect of renal osteodystrophy on vit D, phosphate:

Explain conection between secondary hyperPTH and renal osteodystrophy:
serum 1,25(OH2)D is elevated, patient has hypercalciuria

phosphate

decreased vit D production, PO4 clearance

low vit D --> decreased Ca++ absorption from gut, high serum [P]
Major S/S of OI:

Most common iatrogenic OP:

High risk of what fx?

Tx?
collagen I defect, recurrent fx, hearing loss, poor tooth growth, blue sclerae

steroid-induced - adults receiving long-term prednisone

collapsed vertebrae, hip fx

Ca, vit D, calcitonin, bisphosphonates
Function of bisphosphonates?

effective in what conditions?

recombinant PTH, tx of OP, not approved for children:
inhibits osteoclast action --> decreased bone resorption

hypercalcemia, post-menopause OP, Paget's, OI, steroid-induced osteopenia

Teriparatide (Fortero)
serum level = body storage of vit D:

serum level = active metabolite of vit D:

What promotes active vit D formation?

What decreases production?
25(OH)D - liver

1,25(OH)2D - kidney

high PTH, low P

high P, high Ca, decreased kidney tissue
Effect of low Ca, high P on PTH secretion:

High Ca, high 1,25(OH)2D effect:

effect of serum Mg++:

Connection between CaSR on tissues and PTH:
increases

decreases

mildly low Mg++ --> stimulates
very low Mg++ --> suppresses

CaSR senses low Ca++ --> stimulates PTH, high Ca++ --> inhibits PTH
Ca distribution in the serum:

pathology of tetany:

Carpopedal spasm almost always occurs when serum Ca++ is below:
45% albumin bound, 45% free, 10% complexed

decreased Ca++, Mg++ in fluids around neurons --> hyperexcitability

total <5.0, ionized <2.5
Eval for Ca++ disorders should include what serum tests?

What other diagnostic tests?
serum total/ionized Ca++, albumin, pH, phosphorus, alk phos, creatinine, Mg, PTG, inactive/active vit D

urine Ca/creatinine ratio, tubular reabsorption of P, skeletal survey XR, renal U/S, PTHrP, calcitonin, Vitamin A
Tx of hypocalcemia:

Tx of hypercalcemia:

Usual presentation, lab values of a kid with hypercalciuria:
Ca++ IV/PO, vit D. check serum Mg

fluids, Lasix, steroids, calcitonin, bisphosphonates

hematuria, dysuria, frequency wetting accidents, abd/back pain (most common cause of kidney stones)

Lab - urine Ca > 4 mg/kg/24h
Tx of hypercalciuria:

Why TZ diuretics?

Effect of K-citrate?
low Na+/high K+ diet, fluid intake, RDA protein/Ca, TZ diuretics, K citrate

increase Ca reabsorption in DCT

decreases Ca excretion, citrate pushes Ca to bones, increases Ca solubility in urine