Clinical Findings of Frank Deficiency

The clinical findings of frank biotin deficiency in adults, older children, and infants are similar. Typically, the findings appear gradually after weeks to several years of egg-white feeding or parenteral nutrition. Thinning of hair and progression to loss of all hair, including eyebrows and lashes, has been reported. A scaly (seborrheic), red (eczematous) skin rash was present in the majority; in several, the rash was distributed around the eyes, nose, mouth, and perineal orifices. These cutaneous manifestations, in conjunction with an unusual distribution of facial fat, have been termed 'biotin deficiency facies.' Depression, lethargy, hallucinations, and paraesthe-sia of the extremities were prominent neurologic symptoms in the majority of adults. The most striking neurologic findings in infants were hypotonia, lethargy, and developmental delay.

The clinical response to administration of biotin has been dramatic in all well-documented cases of

Table 3 Foods relatively rich in biotin

Food

ng biotin/g food

serving size (g)

ßg biotin/serving

Chicken liver, cooked

1872.00

74

138.00

Beef liver, cooked

416.00

74

30.80

Egg, whole, cooked

214.00

47

10.00

Peanuts, roasted, salted

175.00

28

4.91

Egg, yolk, cooked

272.00

15

4.08

Salmon, pink, canned in water

59.00

63

3.69

Pork chop, cooked

45.00

80

3.57

Mushrooms, canned

21.60

120

2.59

Sunflower seeds, roasted, salted

78.00

31

2.42

Chili

5.20

441

2.29

Hot dog, chicken and pork, cooked

37.00

56

2.06

Egg, white, cooked

58.00

35

2.02

Banana pudding

10.20

170

1.73

Strawberries, fresh

15.00

111

1.67

biotin deficiency. Healing of the rash was striking within a few weeks, and growth of healthy hair was generally present by 1 or 2 months. Hypotonia, lethargy, and depression generally resolved within 1 or 2 weeks, followed by accelerated mental and motor development in infants. Pharmacological doses of biotin (e.g., 1-10 mg) have been used to treat most patients.

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