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25 Cards in this Set
- Front
- Back
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1. Biliary atresia?
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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.
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2. Kasai procedure?
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a. An operative procedure in which a bowel loop forms a duct to allow bile to drain from a liver with biliary atresia.
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3. Rickets?
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a. Poor mineralization of growing bone or of osteoid tissue.
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4. How does liver failure affect nutrition absorption?
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a. A person w/liver failure has poor bile salt secretion, resulting in poor fat-soluble vitamin absorption, including Vit D.
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5. Effect of Vit D deficiency?
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a. Occasionally reduced serum calcium levels
b. Markedly elevated alk phos c. Poor bone mineralization d. ↑’d risk of fractures. |
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6. Tx of children w/Vit D deficiency from liver failure and ascites and adverse effect of the treatment?
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a. Loop diuretics, which often cause urinary calcium losses
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7. Tx of Rickets?
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a. Goal of restoring normal bone mineralization consists of High vitamin D doses and calcium supplementation.
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8. Nutritional rickets?
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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. |
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9. More common causes of rickets in US?
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a. Liver or renal failure and a variety of biochemical abnormalities in calcium and phosphorous metabolism.
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10. Most common form of non-nutritional rickets?
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a. Familial, primary hypophosphatemia.
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11. Inheritance of Familial, primary hypophosphatemia?!?!?!?!?!?!?
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a. X-linked dominant.
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12. Pathophys of Familial, primary hypophosphatemia?
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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.
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13. Lab evidence of Familial, primary hypophosphatemia?
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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 |
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14. Physical Presentation of Familial, primary hypophosphatemia?
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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. |
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15. Additional rickets findings more specific to calcium-deficient rickets?
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1. Myopathy
2. Rachitic rosary 3. Pectus deformities 4. Tetany. b. These are usually not seen in Familial, primary hypophosphatemia- rickets. |
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16. Teeth defect in Familial, primary hypophosphatemia-rickets vs. calcium deficient-rickets?
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a. Familial, primary hypophosphatemia: Intraglobular dentin deformities.
b. Calcium-deficient rickets: enamel defects. |
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17. Radiologic findings in rickets?
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a. Course-appearing trabecular bone
b. Widening, fraying, and cupping of the metaphysis of the proximal and distal tibia, distal femur radius, and ulna. |
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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)?
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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) |
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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: ↑ |
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20. Causes of Malabsorption of Vit D?
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a. Celiac
b. Cystic Fibrosis c. Steatorrhea |
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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:↑ |
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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:↑ |
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23. 2 anticonvulsive drugs that cause Vit D problems?
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a. Phenobarbital and Phenytoin.
b. Pts have reduced levels of 25(OH)D possibly as a result of ↑d cytochrome P450 activity. |
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24. Tx of Vit D deficiency from Phenobarbital and Phenytoin?
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a. Vitamin D2 and adequate dietary calcium.
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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:↑ |
