CME examination Identification No. 890-105

Instructions for Category 1 CME credit appear in the front advertising section. See last page of Contents for page number.

Questions 1-33, Itin PH, Pittelkow MR. JAM ACAD DERMATOL 1990;22:705-17.

Directions for questions 1-16: Give the single bestresponse.

1. Of the following, the statement that best character­izes clinically the hair of a patient with trichothio­dystrophy isa. the hair is thin and fineb. the hair is white and coarsec. the hair is brittled. the hair is broken off at the levelof the skinsur­

face to produce "black dots"e. the hair falls out easily

2. Of the following, the most characteristic chemicalalteration in the hair of a patient with trichothiodys­trophy isa. increased sulfur contentb. decreased sulfur contentc. increased iron contentd. decreased iron contente. increased lead content

3. Under polarizing microscopy,the most characteristicchange seen in the hair of patients with trichothio­dystrophy isa. a hollow central canalb. a central canal filled with debrisc. alternating light and dark bandsd. spotted darkening of hair colore. bulbous enlargements of the hair shaft

4. The low sulfur-brittle hair syndrome is transmittedas a(an)a. autosomal dominant traitb. autosomal recessive traitc. autosomal codominant traitd. sex-linked (X) traite. sex-linked (Y) trait

5. Hair color in tricothiodystrophy is usuallya. blondeb. whitec. redd. paler than normale. unaffected

6. Severe disorders associated with trichothiodystrophyare most oftena. also associated with abnormal pigmentation

718

b. hepatic/metabolicc. neuroectodermald. associatedwith pregnancye. associated with prematurity

7. orthe following, the most common abnormality re­ported occurring in association with trichothiodys­trophy isa. impairment of motor controlb. pyramidal signswith diminished muscle tonec. seizuresd. hyperreflexiae. mental retardation

8. Of the following, the most commonclinicallysignif­icant problem reportedoccurring in association withtrichothiodystrophy isa. photophobiab. conjunctivitisc. nystagmusd. cataracte. blindness

9. Of the following, the most commonclinicallysignif­icant problemreportedoccurring in associationwithtrichothiodystrophy isa. growth retardationb. protruding earsc. dental abnormalitiesd. receding chine. high-arched palate

10. Of the following, the most commonclinicallysignif­icant problemreported occurring inassociation withtrichothiodystrophy is:a. pruritusb. erythrodermac. ichthyosisd. generalized lichenificatione. alopecia universalis

11. Studies to date indicate that there is a relationbetween some cases of trichothiodystrophyanda. Bloomsyndromeb. Fanconi's syndromec. Gardner's syndromed. Ataxia/telangiectasiae. Xeroderma pigmentosum

Volume 22Number 5, Part 1May 1990

12. In trichothiodystrophy,a. urinary sulfur levels are usually elevatedb. urinary sulfur levels are usually decreasedc. serum sulfur levels are usually elevatedd. serum sulfur levels are usually decreasede. serumand urinary sulfur levelsare usuallynormal

13. In trichothiodystrophy,a. urinary cystine/cysteine levels are usually ele­

vatedb. urinary cystine/cysteine levels are usually de-

creasedc. serum cystine/cysteine levels are usuallyelevatedd. serumcystine/cysteine levels are usuallydecreasede. serumand urinary cystine/ cysteinelevels are usu­

ally normal14. The principalsymptomsand signs ofeach of the fol­

lowing involve primarily ectodermally derived tis­sues,excepta. Tay syndromeb. Sabinas syndromec. Amish brittle hair syndromed. Marinesco-Sjogren syndromee. IBIDS syndrome

15. ReducedDNA repair has beenfoundin patientswithtrichothiodystrophy who also havea. xerodermapigmentosum but not just photosensi­

tivityb. xerodermapigmentosumand photosensitivity but

not those without photosensitivityc. xeroderma pigmentosum, photosensitivity only,

and without photosensitivityd. lack of photosensitivity, but not in photosensitive

patientse. reduced DNA repair has not been found in

patients with trichothiodystrophy16. Characteristic clinical features of the BIDS syn­

drome includeeach of the following, excepta. brittle hairb. ichthyosisc. decreasedfertilityd. short staturee. each of the above is characteristic of this syn­

drome

Directionsfor questions 17-19: Select the one Let­tered item that is most closely related to each num­bered item.

a. BIDS syndromeb. IBIDS syndromec. PIBIDS syndromed. SIBIDS syndromee. ONMR syndrome

CME examination 719

17. Chronic neutropenia18. Photosensitivity19. Osteosclerosis

Directionsfor questions 20 and 21: SeLect the onelettered item that is most closely related to eachnumbered item.

a. Amish brittle hair syndromeb. Tay syndromec. Sabinas syndromed. Pollitt syndromee. Menke's syndrome

20. Trichorrhexis nodosa, mental and physical retarda­tion

21. Ichthyosiform erythroderma, hair shaft abnormali-ties, and mental and growth retardation

Directions for questions 22-24: SeLect the one Let­tered item that is most closely related to each num­bered item.

a. Trichothiodystrophyb. Trichothiodystrophy plus onychodystrophyc. Trichothiodystrophy plus onychodystrophy plus

mental retardationd. Trichothiodystrophy plus onychodystrophy plus

mental retardation plus growth retardatione. Trichothiodystrophy plus onychodystrophy plus

mental retardation plus growth retardation plusichthyosis

22. Amish brittle hair syndrome23. Sabinas syndrome24. Tay syndrome

Directions for questions 25-28: For each numbereditem choose the appropriate lettered item.

a. High-sulfur proteinsb. High-tyrosine proteinsc. Bothd. Neither

25. Matrix proteins of hair shafts26. Filamentous proteins of hair shafts27. Rich in cystine28. Rich in glycine

Directions for questions 29-31: For each numbereditem choose the appropriate lettered item.

a. Marinesco-Sjogren syndromeb. Siemenssyndromec. Bothd. Neither

29. Keratosis follicularis decalvans30. Oligophrenia, cerebellar ataxia, cataract, short stat­

ure, and unruly hair

CME examination

31. Photosensitivity, ichthyosis, intellectual impairment,decreased fertility, and short stature

Directions/or questions 32 and 33: Indicate correctanswers. All, some, or none 0/ the choices may becorrect.32. Most cases of trichothiodystrophy are associated

with intestinal malabsorption ofa. cystine/cysteineb. zinc

Answers to CME examination*

JDuma! of theAmerican Academy of

Dermatology

c. sulfurd. coppere. iron

33. Nail anomalies reported in association with tricho­thiodystrophy includea. splittingb. ridgingc. thickeningd. koilonychiae. yellow discoloration

Identification No. 890-104

April 1990 issue of the JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY

Questions 1-33, Cobb MW. JAM ACAD DERMATOL 1990;22:547-66.

1. a (p 550, C 1, pa 2)2. d (p 550, c 1, pa 2)3. d (p 551, C 2, pa 2)4. d (p 549, c 1, pa 2; pa 3)5. e (p 549, C 2, pa 2)6. e (p 548, c 2, pa 3)7. e (p 548, C 2, pa 3)8. c (p 556, C 2, pa 2)9. a (p 549, C 2, pa 1)

10. e (p 549, C 2, pa 1)11. a (p 549, C 2, pa 1)12. e (p 555, c 2, pa 3)13. e (p 555, C 2, pa 3)14. d (p 560, C 1, pa 3)15. e (p 559, C 2, pa 2)16. b (p 550, C 1, pa 2)17. a (p 555, C 2, pa 3)*p: page; c: column; pa: paragraph.

720

18. e19. a20. b21. a22. a23. b24. b25. b26. a27. a, b, C, d, C

28. a, b, c, d29. a. b, C, e30. None of

the answersis correct

31. c, e32. a, b, C, d, e33. a, b, C, d

(p 553, c 1, pa 2)(p 557, C I, pa 3~ 2, pa 1)(p 557, C 1, pa 3~ 2, pa 1)(p 557, C 1, pa 3~ 2, pa 1)(p 560, c 2, pa 2)(p 555, C 2, pa 4; p 560, C 2, pa 2)(p 555, C 2, pa 4; p 560, C 2, pa 2)(p 560, C 2, pa 2)(p 560, c 2, pa 2)(p 552, C 1, pa 2-p 553, C 2, pa 2)(p 555, C 1, pa 3)(p 555, c 1, pa 4)(p 557, C 2, pa 2)

(p 560, C I, pa 2)(p 560, C 2, pa 3-p 561, c I, pa 1)(p 562, C 2, pa 1)