Tryptophan Disorders

Question 1

Blue Diaper Syndrome

Answer 1

Indicanuria or Hartnup Disease

Question 2

Rare inherited disorder with increased amounts of tryptophan converted to indole. The indole is then reabsorbed from the intestine into the bloodstream and circulated to the liver, where it is converted to indican and then excreted in the urine.

Answer 2

Indicanuria or Hartnup Disease or Blue Diaper Syndrome

Question 3

Indican excreted in the urine is _____ until oxidized to the dye _____ by exposure to air

Answer 3

1. colorless
2. indigo blue

Question 4

Treatment of Indicanuria

Answer 4

proper dietary supplements, including niacin

Question 5

Urine test for Indicanuria

Answer 5

Ferric chloride test (violet-blue with chloroform)

Question 6

carcinoid tumors involving the argentaffin (enterochromaffin) cells develop, excess amounts of SEROTONIN are produced, resulting in the elevation of urinary _______ levels

Answer 6

5-HIAA (5-Hydroxyindoleacetic acid) or Argentaffinoma

Question 7

normal daily excretion of 5-HIAA

Answer 7

2 to 8 mg (more than 25 mg/24h - indication of argentaffin cell tumors)

Question 8

Urine test for Argentaffinoma

Answer 8

first morning/random
if 24-hour sample is used, it must be preserved with hydrochloric or boric acid

Nitrosonapthol test
- purple to black

Ferric chloride test
- blue-green

Plasma method: HPLC w/ fluorescence detection

Question 9

Serotonin is a major constituent of foods such as

Answer 9

bananas
pineapples
tomatoes