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25 Cards in this Set

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  • Back
1. Biliary atresia?
a. A congenital condition affecting approximately 1 in 16,000 live births in which the liver’s bile ducts become blocked and fibrotic, resulting in reduced bile flow into the bowel.
2. Kasai procedure?
a. An operative procedure in which a bowel loop forms a duct to allow bile to drain from a liver with biliary atresia.
3. Rickets?
a. Poor mineralization of growing bone or of osteoid tissue.
4. How does liver failure affect nutrition absorption?
a. A person w/liver failure has poor bile salt secretion, resulting in poor fat-soluble vitamin absorption, including Vit D.
5. Effect of Vit D deficiency?
a. Occasionally reduced serum calcium levels
b. Markedly elevated alk phos
c. Poor bone mineralization
d. ↑’d risk of fractures.
6. Tx of children w/Vit D deficiency from liver failure and ascites and adverse effect of the treatment?
a. Loop diuretics, which often cause urinary calcium losses
7. Tx of Rickets?
a. Goal of restoring normal bone mineralization consists of High vitamin D doses and calcium supplementation.
8. Nutritional rickets?
a. Results form inadequate dietary D or lack of sunlight exposure.
b. Occasionally seen in dark-skinned infants who do not receive vit D supplementation or in breast-fed infants not exposed to sunlight.
9. More common causes of rickets in US?
a. Liver or renal failure and a variety of biochemical abnormalities in calcium and phosphorous metabolism.
10. Most common form of non-nutritional rickets?
a. Familial, primary hypophosphatemia.
11. Inheritance of Familial, primary hypophosphatemia?!?!?!?!?!?!?
a. X-linked dominant.
12. Pathophys of Familial, primary hypophosphatemia?
a. Phosphate reabsorption is defective, and conversion of 25 (OH)D to 1,25(OH)2D in the proximal tubules of the kidney is abnormal.
13. Lab evidence of Familial, primary hypophosphatemia?
a. Low serum 1,25 D
b. Low-normal serum calcium
c. Moderately low serum phosphate
d. Elevated Serum alk phos levels
e. Hyperphosphaturia
f. No evidence of hyperparathyroidism
14. Physical Presentation of Familial, primary hypophosphatemia?
a. Children at the age of walking present w/smooth lower-extremity bowing (as compared to angular bowing of calcium-deficient rickets)
b. Waddling gait
c. Genu Varum, Genu valgum, coxa vara, and short stature.
15. Additional rickets findings more specific to calcium-deficient rickets?
1. Myopathy
2. Rachitic rosary
3. Pectus deformities
4. Tetany.
b. These are usually not seen in Familial, primary hypophosphatemia- rickets.
16. Teeth defect in Familial, primary hypophosphatemia-rickets vs. calcium deficient-rickets?
a. Familial, primary hypophosphatemia: Intraglobular dentin deformities.
b. Calcium-deficient rickets: enamel defects.
17. Radiologic findings in rickets?
a. Course-appearing trabecular bone
b. Widening, fraying, and cupping of the metaphysis of the proximal and distal tibia, distal femur radius, and ulna.
18. 6 Causes of Calcium deficiency w/secondary hyperparathyroidism (Vit D deficiency or low 25 (OH) D w/o stimulation of 1,25(OH)2D production)?
1. Lack of Vit D (diet, sunlight, congenital)
2. Malabsorption of D
3. Hepatic disease
4. Anticonvulsive drugs
5. Renal Osteodystrophy
6. Vitamin D-dependent type 1 (distal)
19. How would the following values present w/Lack of Vitamin D (lack of exposure to sunlight; dietary deficiency vitamin D, congenital):
a. Serum Calcium:
b. Serum Phosphorous:
c. Serum Alkaline phosphatase:
d. Urine Amino Acids:
1. Serum Calcium: N or ↓
2. Serum Phosphorous:↓
3. Serum Alkaline phosphatase: ↑
4. Urine Amino Acids: ↑
20. Causes of Malabsorption of Vit D?
a. Celiac
b. Cystic Fibrosis
c. Steatorrhea
21. How would the following values present w/malabsorption of Vit D:
a. Serum Calcium:
b. Serum Phosphorous:
c. Serum Alkaline phosphatase:
d. Urine Amino Acids:
1. Serum Calcium: N or ↓
2. Serum Phosphorous:↓
3. Serum Alkaline phosphatase:↑
4. Urine Amino Acids:↑
22. How would the following values present w/Hepatic disease:
a. Serum Calcium:
b. Serum Phosphorous:
c. Serum Alkaline phosphatase:
d. Urine Amino Acids:
1. Serum Calcium: N or ↓
2. Serum Phosphorous:↓
3. Serum Alkaline phosphatase:↑
4. Urine Amino Acids:↑
23. 2 anticonvulsive drugs that cause Vit D problems?
a. Phenobarbital and Phenytoin.
b. Pts have reduced levels of 25(OH)D possibly as a result of ↑d cytochrome P450 activity.
24. Tx of Vit D deficiency from Phenobarbital and Phenytoin?
a. Vitamin D2 and adequate dietary calcium.
25. How would the following values present w/Anticonvulsive drugs:
a. Serum Calcium:
b. Serum Phosphorous:
c. Serum Alkaline phosphatase:
d. Urine Amino Acids:
1. Serum Calcium: N or ↓
2. Serum Phosphorous:↓
3. Serum Alkaline phosphatase:↑
4. Urine Amino Acids:↑