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time, but less than I year; the second eye deteriorated within a period of 5 months.

A characteristic of functional or hysterical visual

loss is the apparent failure to be inconvenienced or

“significantly incapacitated” by apparently very

poor measurable visual function. This case

empha-sizes that this feature may be seen in organic visual loss and thus the importance of aggressively

pursu-ing investigations when even minor physical signs

are present.

The patient, his mother, and brothers are all

het-eroplasmic for the 11 778 mutation. It has been sug-gested recently that the relative proportions of

mu-tant and wild-type mtDNA determine the risk of

developing the disease.7 The patient has a higher

proportion of mutant mtDNA in his white blood cells

than either of his brothers, which may help to explain the intrafamilial variation in the clinical expression of the disease. However, uncertainty remains regarding

the proportion of mutant mtDNA in the primarily

affected tissue (optic nerve), given the potential van-ability of mutant-wild-type ratios in different hetero-plasmic tissues of the body. The clinical significance

of carrying the mutation is uncertain, and an

mdi-vidual can remain asymptomatic even to a late age.

Heteroplasmy cannot fully explain the variability of

clinical expression; other factors, such as exposure to

environmental toxins and the mitochondrial and

nu-clear genetic background, may alter the clinical

ex-pression of the disease.#{176} Genetic counseling is, therefore, very difficult.

Patients with LHON may have associated systemic

involvement. Neunologic manifestations such as

en-cephalopathy and movement disorders8’11’12 have

been documented in some families, and cardiac

con-duction defects (especially preexcitation syndromes) have been noted in many pedigrees.13’14

Electnocar-diographic studies, therefore, may be important in

asymptomatic cases. The clinical presentation and

any associated systemic features of patients with

LHON may be related to the type of mutation

present in the mitochondnial DNA.3 Although the

magnetic resonance imaging scan was normal in this

patient, intraorbital lesions of the optic nerve are

commonly imaged in patients with LHON.15

In summary, this case illustrates two clinical

points: first, that LHON may appear in childhood

and, when it does, that it may be associated with

remarkably little functional defect; second, because

of variable expression of the mutation in family

members, it may present more commonly without a

family history than had been recognized. Leber’s

he-reditary optic neuropathy can now be diagnosed

de-finitively by means of mitochondnial DNA analysis.

In children with unexplained acute or subacute

visual loss, who do not have structural abnormalities on brain and orbital imaging, testing for the presence

of the mtDNA 11 778 mutation and other mutations

commonly associated with LHON may be helpful.

C. M. MOORMAN, FCOPHTH

J.

S. ELSTON, FCOPHTH

Oxford Eye Hospital Oxford, England

P. MA-I-I-HEWS, MD, DPHIL,

Genetics Laboratory and University Dept of Neurology

Oxford University Oxford, England

REFERENCES

I. Nikoskelainen EK, Savontous ML, Wanne OP. Katila MJ, Nummela KU.

Leber’s optic neuroretinopathy. a maternally inherited disease: a geneo-logic study in four pedigrees. Arch Ophthalmol. 1987;105:665-671

2. Brown MD, Voljavec AS, LoU MT. et al. Leber’s hereditary optic

neur-opathy: a model for mitochondrial neurodegenerative diseases. FASEB

I. 1992;6:2791-2799

3. Newman NJ, Lott MT. Wallace DC. The clinical characteristics of pedi-grees of Leber’s hereditary optic neuopathy with the 11 778 mutation. Am JOphthalmol. 1991;111:750-762

4. Seedorf T. The inheritance of Leber’s disease: a genealogical follow-up study. Acta Ophthalmol. 1985;63:135-145

5. Stone EM, Cuppings JM, Kardon RH, Donelson J. Mae 111 positively

detects the mitochondrial mutation associated with type I Leber’s hereditary optic neuropathy. Arch Ophthamol. 1990;108:1417-1420

6. Anderson 5, Bankier AT, Barrell BC, et al. Sequence and organization of the human mitochondrial genome. Nature. 1981;290:457-465

7. Harding AE. Neurologic disease and mitochondrial genes. Trends Neurosci. 1991;14:132-138

8. Howell N, Kubacka I, Xu M, McCullough DA. Leber hereditary optic

neuropathy: involvement of the mitochondrial NDI gene and evidence

for an intragenic suppressor mutation. Am IHum Genet. 1991;48:935-942 9. Vilki J, Otto J, Savontaus ML, Avion P. Nikoskelainen EK. Optic atrophy

in Leber’s hereditary optic neuroretinopathy is probably determined by

an x-thromome gene closely linked to Dx 57. Am I Hum Genet.

1991;48:486-491

10. Schoffner JM, Wallace DC. Oxidative phosphorylation diseases: disor-ders of two genomes. Ado Hum Genet. 1990;19:267-330

11. Wallace DC. A new manifestation of Leber’s disease and a new expla-nation for the agency responsible for its unusual pattern of inheritance.

Brain. 1970;93:121-132

12. Larsson N, Anderson 0, Holme E, Oldfors A, Wahlstrom J. Leber’s

hereditary optic neuropathy and complex 1 deficiency in muscle. Ann

Neurol. 1991;30:701-708

13. Rose FC, Bowden AN, Bowden PMA. The heart in Leber’s atrophy.

Br JOphthalmol. 1970;54:388

14. Nikoskelainen E, Wanne 0, Dahi M. Pre-excitation syndrome and Leb-er’s hereditary optic neuroretinopathy. Lancet. 1985;1:969

15. Kermode AG, Moseley IF, Kendall BE, et al. Magnetic resonance

imag-ing in Leber’s optic neuropathy. I Neurol Neurosurg Psychiatry. 1989; 52:671-674

Spectrum

of Limb

Disruption

Defects

Associated

With

Chorionic

Villus

Sampling

ABBREVIATION. CVS, chorionic villus sampling.

A number of recent publications have suggested

an increased incidence of transverse limb

anoma-lies,1’2 the hypoglossia-hypodactylia syndrome,1 and

cavernous hemangiomas3 in infants born to mothers

who had undergone chonionic villus sampling (CVS)

in the first trimester of pregnancy. We previously

reported 4 CVS-related cases of transverse limb

anomalies2 and since have examined 10 additional

cases. The purpose of this report is to describe the

spectrum of abnormalities observed in these 14 cases. We believe that the findings in these patients support

Received for publication Oct 19, 1992; accepted Dec 16, 1992.

Reprint requests to (B.K.B.) Center for Medical and Reproductive Genetics, Humana Hospital-Michael Reese, 2929 S Ellis Aye, Chicago, IL60616-3390.

PEDIATRICS (ISSN 0031 4005). Copyright © 1993 by the American

Acad-emy of Pediatrics.

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(2)

an association between CVS and limb abnormalities

by

demonstrating that the type of anomaly

associ-ated with CVS is a very specific one that is uncom-monly observed in the general population.

METHODS

Cases 1 through 4 (Table) were ascertained through routine

surveillance of pregnancy outcome data on 436 patients

undergo-ing CVS at Humana Hospital-Michael Reese and have been

pre-viously reported.2 Case 5 was identified subsequently in the same patient population and involved a patient who initially reported no abnormalities in her infant but later came to attention because

of increased awareness of the link between CVS and digital

anom-alies. Case 6 was ascertained at the Craniofacial Center at the

University of Illinois College of Medicine, where she was

exam-med by one of the authors (B. K. B.). Cases 7 through 14 were

referred for evaluation because of the authors’ known interest in

the possible link between CVS and limb abnormalities. Patients 6

through 14 all had mothers who had undergone CVS at other

centers in the United States. A complete medical history and pre-natal and family history were obtained for each patient. Prenatal records related to the CVS procedure and relevant medical records

on the affected infants were obtained. Cases 1 through 8, 10, 11,

and 14 were examined by one of the authors. In the other 3 cases,

photographs were reviewed. Radiographs of the extremities were

obtained in 9 cases. Patients 1 and 6 had nuclear magnetic

reso-nance imaging of the brain.

RESULTS

The findings in the 14 cases are summarized in the

Table.

Limb Anomalies

All patients in this series have transverse disrup-tion defects of the limbs involving absence or short-ening of the digits. Nails are absent in all except the least severely affected digits, in which nail hypopla-sia may be observed. One of the patients had

unilat-era! absence of the metacarpals (case 10) and one

(case 6) had hypoplasia of the left foot, although all

bones except the middle and distal phalanges were

present radiographically. None of the patients had

any shortening of the long bones. There was no clear evidence in any case of amniotic bands.

Representa-tive photographs of the typical digital anomalies

observed are seen in the Figure. Hemangiomas

Seven of the 14 patients described had one or more

cavernous hemangiomas. None required medical

in-tervention, although several were of cosmetic

signif-icance. None of the patients had evidence of any

visceral hemangiomas. Other Anomalies

One of the 14 patients (case 6) had hypog!ossia and met the criteria for the diagnosis of the

hypoglossia-hypodactylia syndrome. One patient (case 2) had

strabismus requiring surgical intervention. None of

the patients had any other anomalies. All have

ex-hibited normal growth and psychomotor

develop-ment up to their present ages of 6 months to 4 years.

Prenatal and Family Histories

All 14 mothers in this series underwent CVS

be-cause of advanced maternal age. All were 35 years of

age or older at the time of delivery. There was no

family history in any case of major or minor limb

anomalies, nail hypoplasia, or cavernous hemangio-mas.

One

patient (case 1) used topical isotretinoin

during early gestation; there was no other history of

exposure to any potential teratogens. Specifically, all 14 patients denied use of cocaine during pregnancy. None of the patients had a history of diabetes

melli-tus or chronic hypertension. None had a history of

uterine anomalies, trauma during pregnancy, or any

other pregnancy complications. Eleven patients were

white, 2 were African-American, and I was Asian.

CVS Procedures

Twelve of the 14 mothers had their CVS

proce-dures at 9 to 9.5 weeks’ gestation; the other two

procedures were performed at 10 to 11 .5 weeks.

Eleven of the procedures were transcervical; three

were transabdominal. Four of the transcervical

pro-TABLE. Patients With CVS-Related Limb Anomalies*

Patient Limb Anomalies Hemangiomas Other Type of GA,+ wk Age at Last

Anomalies CVS Examination

1 Adactyly R hand; hypodactyly digits 2-4 Lhand and 3 None TC 9.5 19 mo

all digits R foot; L foot normal

2 Hypodactyly all digits L hand and both feet and digits 1 Strabismus TC 10.5 18 mo

2-5 R hand

3 Hypodactyly 3rd and 4th digits R hand, all digits L 0 None TC 11.5 14 mo

hand and L foot, digits 1-4 R foot

4 Hypodactyly digits 2 and 3 L hand; rest normal 0 None TA 9.5 19 mo

5 Hypodactyly digits 2 and 3 R foot; nail hypoplasia 0 None TC 9.5 21 mo

digit 3 L hand

6 Hypodactyly digits 1-3 L hand, digits 2-4 R hand, all 3 Hypoglossia TC 9.0 14 mo

digits R foot; L foot smaller than R

7 Hypodactyly all digits both hands 0 None TA 9.5 24 mo

8 Hypodactyly digits 3-5 R hand, all digits L hand and 2 None TC 9.5 18 mo

L foot, digit 2 R foot

9 Adactyly all digits both hands 1 None TC 9.0 5 mo

10 Adactyly all digits L hand 0 None TC 9.5 3 y

11 Hypodactyly all digits both feet 2 None TC 9.0 3 y

12 Hypodactyly digits 2 and 3 L hand and L foot; R hand 1 None TC 9.5 10 mo

and foot normal

13 Adactyly all digits L hand 0 None TA 9.5 30 mo

14 Hypodactyly all digits both feet 0 None TC 9.0 4 y

(3)

Fig 1. Representative photographs of CVS-related digital anomalies. A: Hands of patient 9. The fingers of both hands are represented only

by tiny nubbins. B: Hands of patient 3. All digits on the left hand are shortened, with no nails. The third and fourth digits of the right hand

are minimally shortened. There is no nail on the third and a hypoplastic nail on the fourth digit. C: Hands of patient 2. All digits except the right thumb are shortened, with no nails. D: Feet of patient 3. The toes of the left foot are all represented by tiny nubbins. All toes on the right foot are shortened; a nail is present on the fifth toe only. E: Feet of patient 8. All digits on the left foot are short, with no nails. The second toe on the right foot is slightly shortened, with a hypoplastic nail.

cedures were performed using a COOK OB/GYN

(Spencer, IN) catheter, four with a Trophocan

(Concord/Portex, Keene, NH) catheter, two with

an-other type of catheter not specifically designed for

CVS, and one with an unknown type of catheter. Two

of the transabdominal procedures were performed

using a 20-gauge needle and the third with an

18-gauge needle. In 12 cases, an adequate sample was

obtained with one sampling attempt. In 1 case, two

attempts were required. In I case, a single sampling attempt yielded a sample insufficient for analysis so

an amniocentesis was performed 6 weeks later. The

karyotypes were normal in all 14 cases.

DISCUSSION

Since publication of the results of two large collab-orative studies comparing CVS with amniocentesis

in 1989, CVS has been offered with increasing

fre-quency across the United States to women at

in-creased risk of having a fetus with a chromosomal

anomaly or other detectable genetic disorder. Both of

these early studies assessing CVS focused primarily on short-term risks, such as fetal loss, and did not

provide detailed data on birth defects observed in

the infants subsequently delivered. Only recently

have concerns been raised regarding the possibility

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(4)

that CVS might be associated with an increased risk of certain congenital anomalies.

Isolated case reports describing CVS-exposed

in-fants with limb defects were published in 19866 and

I989. Serious concerns regarding a possible link to

limb anomalies were first voiced, however, when

Firth et a!’ reported a series of 289 infants exposed to

transabdominal CVS between 56 and 66 days’

gesta-tion, among whom 5 had severe limb abnormalities.

Four of the 5 had the hypoglossia-hypodactylia

syn-drome, while the fifth had a unilateral

amputation-type defect. The authors suggested that the early

timing of the procedure in these cases might have

been a factor in the occurrence of the abnormalities. Subsequent to this report, a number of letters to the editor appeared suggesting that a similar increased incidence of limb defects had been observed in Italy,8 China,9 and Germany.’#{176} In 1992, we published our

experience with a series of 394 CVS patients, of

whom 4 gave birth to infants with a very similar type of limb anomaly.2

Once these reports began to appear, data on birth

defects from the National Institute of Child Health

and Human Development Collaborative CVS Study

Group were analyzed and reported by Mahoney.11 In

a combined series of 9588 pregnancies exposed to

CVS, a total of 8 infants with limb defects were

re-ported. In comparison with the general population

frequency of all types of limb defects of

approxi-mately 1 in 2000 as reported in the British Columbia

Registry,12 this was interpreted as demonstrating no

increased frequency. It should be noted, however,

that 6 infants in this CVS-exposed cohort had

iso-lated terminal transverse defects. Transverse limb de-fects of all types occur with an estimated frequency of I .8 per 10 yyj,12 while defects of the fingers occur

in I in 7016 and defects of the toes in I in 43 354

births.’3

The cases reported in this series do not shed any

light on the frequency with which limb disruption defects occur as a result of CVS in early pregnancy,

because many of them were ascertained by virtue of

this association. The earlier reports suggest that such

defects are a rare, albeit real, complication of the

procedure. What is striking about the cases reported in this series is the remarkable similarity of the

ab-normalities observed among the individual cases.

They are clearly not representative of the entire

spec-trum of limb malformations observed in the general

population but reflect a very specific type of disrup-tion defect rarely seen in otherwise normal infants not exposed to CVS. Recognition of this specific type of abnormality may not only alert the pediatrician

examining an infant with this type of defect to ask

mothers whether they had a CVS procedure, but it

may ultimately help to elucidate the mechanism

leading to these abnormalities. It is also of

signifi-cance that 12 of the 14 mothers in this series had a

CVS procedure performed prior to 10 weeks’

gesta-tion. Although most CVS centers in the United States

have historically offered CVS at 9 to 12 weeks of

pregnancy, analysis of the experience from the two

US collaborative studies revealed that only 11 % were performed at or before 9#{189}weeks’ gestation.’1 Our

observation would, therefore, support previous

sug-gestions that the risk of CVS-related anomalies is

greatest when the procedure is performed prior to 10

weeks. In fact, many centers now offering CVS in the

United States restrict the procedure to patients who are at 10 weeks’ gestation or beyond.

We have previously suggested that the limb

abnor-malities associated with CVS have a vascular basis,

and the spectrum of defects observed in the present series of patients is entirely consistent with this. By its nature, CVS results in transection of villi with disruption of fetal villous vessels. It is likely that a

small amount of fetal bleeding occurs in almost all

cases into the sampling site or maternal circulation. Since the total blood volume of the embryo is small at this stage of gestation, this may be sufficient in some cases to result in hypoperfusion and anoxia of distal fetal structures, such as distal limbs. Alternatively, we believe that there could be vasoactive substances

released in response to the placental injury which

lead to vasoconstriction or hemorrhage in the fetus. Quintero et al’4 have recently described fetal

hemor-rhagic lesions visualized by embryoscopy following

CVS at 9#{189}weeks’ gestation. Indeed, a vascular

eti-ology for many limb defects was suggested long

be-fore there were data suggesting a relationship

be-tween CVS and limb anomalies. Hoyme et a115

described four cases of isolated limb reduction

de-fects in which fetal vascular occlusion could be dem-onstrated. In addition, limb reduction defects as well

as brainstem infarcts can be produced in animal

models by reducing blood flow in the uterine vessels.

16,17

It is of interest that half of the patients reported

here had one or more cavernous hemangiomas in

contrast to a reported frequency of 8% to 10% in the

general population.’8 In a previous study comparing

major and minor anomalies in children born to

moth-ers following CVS and amniocentesis, an increased

frequency of superficial cavernous hemangiomas

was noted in the CVS group.3 Further investigation of this possible association is clearly warranted.

CONCLUSION

A clear pattern of limb-disruptive defects

associ-ated with CVS in early gestation is supported by the

cases described. The frequency with which this

oc-curs and specific factors contributing to its occur-rence are still largely unknown. Pediatricians should be aware of this association when examining infants

with limb and digital anomalies. Mothers

consider-ing prenatal diagnosis should be aware that limb

abnormalities, and perhaps cavernous

hemangio-mas, may be a rare complication of the CVS

proce-dune.

BARBARA K. BURTON, MD

Depts of Pediatrics and Obstetrics and Gynecology

University of Illinois College of Medicine

Chicago

Michael Reese Hospital and Medical Center Chicago

CHARLENE J. SCHULZ, MS

Michael Reese Hospital and Medical Center

LAURENCE I. BURD, MD

(5)

University of Illinois College of Medicine

Michael Reese Hospital and Medical Center

REFERENCES

1. Firth HV, Boyd PA, Chamberlain P. et al. Severe limb abnormalities after chorion villus sampling at 56-66 days’ gestation. Lancet. 1991;1:762-763 2. Burton BK, Schulz CJ, Burd LI. Limb anomalies associated with

chori-onic villus sampling. Obstet Gynecol. 1992;79:726-730

3. Kaplan P, Normandin JJ, Wilson GN, et al. Malformations and minor

anomalies in children whose mothers had prenatal diagnosis: compar-ison between CVS and amniocentesis. Am JMed Genet. 1990;37:366-370 4. Rhoads GG, Jackson LG, Schlesselman SE, et al. The safety and efficacy

of chorion villus sampling for early prenatal diagnosis of cytogenetic abnormalities. N Engl I Med. 1989;320:609-617

5. Canadian Collaborative CVS-Amniocentesis Clinical Trial Group. Mul-ticentre randomized clinical trial of chorion villus sampling and amnio-centesis. Lancet. 1989;1:1-6

6. Planteydt HT, van de Vooren MJ, Verweij H. Amniotic bands and

mal-formation in child born after pregnancy screened by chononic villus

biopsy. Lancet. 1986;2:756-757

7. Chistiaens GCML, Van Baarlen J, Huber J, Leschot NJ. Fetal limb

con-striction: a possible complications of CVS. Prenat Diagn. 19899:67-71

8. Mastroiacovo P. Cavalcanti DP. Limb-reduction defects and chorion

villus sampling. L.ancet. 1991;337:1091

9. Hsieh F-J, Chen D, Tseng L-H, et al. Limb-reduction defects and chorion villus sampling. Lancet. 1991;337:1091-1092

10. Miny I, Holzgreve W, Horst J, Lenz W. Limb abnormalities and

chori-onic villus sampling. Lancet. 1991;337:1423-1424

11. Mahoney MJ. Limb abnormalities and chorionic villus sampling. Lancet.

1991337:1422-1423

12. Froster-Iskenius VG, Baird PA. Limb reduction defects in over one mil-lion consecutive live births. Teratology. 1989;39:127-135

13. Froster UG, Baird PA. Limb reduction defects and chorionic villus sam-pling. Lancet. 1992;339:66

14. Quintero RA, Romero R, Mahoney MJ, et al. Fetal haemorrhagic lesions after chorionic villus sampling. Lancet. 1992339:193

15. Hoyme HF, Jones KL, Van Allen MI, Saunders BS, Benirschke K.

Vas-cular pathogenesis of transverse limb reduction defects. JPediatr. 1982; 101:839-843

16. Brent RL. Relationship between uterine vascular clamping, vascular

disruption syndrome and cocaine teratogenicity. Teratology. 1990;41: 757-760

17. Webster WS, Lipson AH, Brown-Woodman PDC, Osborn RA. Moebius unmasked: pathogenesis of the Moebius syndrome in an animal model. Teratology. 1988;38:199

18. Jacobs AH, Walton RG. The incidence of birthmarks in the neonate.

Pediatrics.1976;58:218-222

A Simplified

Behavioral

Treatment

for

Trichotillomania:

Report

of Two

Cases

Trichotillomania is a behavioral disorder character-ized by habitual hair pulling resulting in alopecia.

Although once considered extremely rare, a recent

survey study of college freshmen suggests a lifetime

prevalence of more than 3% in females and more

than I % in males.1

The recent literature on trichotillomania has

em-phasized the pharmacologic treatment of adolescents

and adults. Some studies have reported decreased

hair pulling in response to treatment with the

anti-obsessional medications clomipramine and

fluoxet-me.2’3 However, Christenson and colleagues4 failed

Received for publication Jun 11, 1992; accepted Feb 3, 1993.

Reprint requests to (N.J.B.) Children’s Seashore House, 3405 Civic Center Blvd. Philadelphia, PA 19104.

PEDIATRICS (ISSN 0031 4005). Copyright © 1993 by the American

Acad-emy of Pediatrics.

to demonstrate benefit from fluoxetine, and in a

pre-liminary report Rothbaum and found greater

decreases in hair pulling with a behavioral interven-tion than with clomipramine.

A number of behavioral interventions for trichotil-lomania have been described.6 One well-supported

behavioral intervention involves treating thumb

sucking when hair pulling co-occurs, 7h1 but hair

pulling in young children is not always accompanied

by

thumb sucking. Previous studies of behavioral

treatments for hair pulling alone have been limited in

the following ways: they have used procedures such

as hypnotherapy, which are unfamiliar to most

pedi-atricians12; they have used multicomponent (8 to 13

components) interventions difficult to apply in most pediatric settings 1314; they have not used experi-mental methodologies to assess the efficacy of

treat-ment15-18; and in only one case18 have they treated a

preschool-age child. In this paper single-subject

re-search methodologies are used to demonstrate the

efficacy of a simplified behavioral treatment for tri-chotillomania in two children. We believe this treat-ment could be used successfully by pediatricians.

CASE 1

A 3-year-old girl was seen for chronic hair pulling of I year’s duration. The hair pulling had resulted in alopecia on the crown of

her head (approximately a 13 X 8-cm patch). The hair pulling

occurred mostly during the onset of sleep and during sedentary

activities. She did not exhibit other significant behavior problems and was described by her parents as happy, compliant, and highly intelligent. The parents had tried several procedures to stop the hair pulling with no success. These included scolding, hats, edible rewards for not pulling, and spanking.

The treatment for hair pulling involved three components: (1)

Increased nurturing (time-in) was provided by asking each parent to increase physical touching by 50 touches per day and play with

their daughter at least 10 minutes per day, providing frequent

praise and avoiding questions, commands, or criticisms. In

addi-lion, the usual bedtime routine was extended by 15 minutes. (2)

The child was placed in time-out for 3 minutes contingent on

observed hair pulling. (3) Response prevention was used to help

the child limit her hair pulling. The child agreed to select a pair of loose-fitting cotton socks (hand socks) to be placed over her hands if she was observed pulling her hair in the time-out chair. She also wore the hand socks to bed at night.

Three days per week one parent observed the child for the

presence or absence of hair pulling during 5 high-risk one-hour

time intervals. The percentage of intervals during which hair

pull-ing occurred was recorded for each day. During 25 one-hour

in-tervals the child was observed by both parents, and there was 92%

interobserver agreement on the presence or absence of hair

pull-ing.

A within-series withdrawal of treatment (ABAB) design was

used to assess the effects of treatment (Fig 1). During baseline hair

pulling occurred in 76% of observed intervals. During the first

treatment phase hair pulling rapidly declined, averaging 22% of

observed intervals. During withdrawal of treatment hair pulling

returned to baseline levels. When the treatment was reintroduced

hair pulling quickly decreased, with no hair pulling observed for

seven consecutive data points collected over more than 2 weeks.

The use of hand socks at bedtime was stopped after 2 months, but

it was recommended that the parents continue the increased

time-in. At 6-month follow-up hair pulling was still at zero levels, and

at 2-year follow-up the child was reported to have a full head of

hair approximately 14 inches in length.

CASE 2

A 9-year-old girl was seen for chronic hair pulling of 8 months’

duration. The hair pulling occurred both at home and in school. It

had resulted in alopecia of the scalp on the left side and the crown

of the head (approximately 40% bald), and the absence of

(6)

1993;91;989

Pediatrics

BARBARA K. BURTON, CHARLENE J. SCHULZ and LAURENCE I. BURD

Spectrum of Limb Disruption Defects Associated With Chorionic Villus Sampling

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1993;91;989

Pediatrics

BARBARA K. BURTON, CHARLENE J. SCHULZ and LAURENCE I. BURD

Spectrum of Limb Disruption Defects Associated With Chorionic Villus Sampling

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American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143. Copyright © 1993 by the

been published continuously since 1948. Pediatrics is owned, published, and trademarked by the

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References

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