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Pediatrics Review andEducation Program

CONTENTS

ARTICLES

379 Chronic Nonspecific Diarrhea

Robert H. Judd

388 Movement Disorders in Childhood

Michael R. Pranzatelli

395 Back to Basics: Fluids and Electrolytes-

Clinical Aspects

Nicholas Jospe and Gilbert Forbes

405 Index of Suspicion

Gregory P. Connei�s’, Angela E. Liii, Juan A. Rivera

408 Consultation with the Specialist:

Congenital Stridor

Linda Brodskv

POINT-COUNTERPOINT

404 Acute Asthma Therapy

IN BRIEF

385 Reactions to Iodinated Contrast Media

386 Microcephaly

COVER

Each of our 1996 issues of Pediatrics in Review will feature a work of art sub-

mitted to our cover art contest this past year. We received more than 200 en-

tries and have chosen 12 to appear on our covers-four from each of three

age groups: 5 to 7 years, 8 to 10 years, and 11 to 15 years. The entrants were

asked to submit a drawing of what they like to do best. Most of the entries

will be displayed by the American Academy of Pediatrics at various sites.

This month’s work, by 6-year-old Taylor Woolwine, is of him playing basket-

ball with friends in physical education class. Taylor lives in Mechanicsville,

MD; his pediatrician is Pablo A. Dublin, Jr, MD.

ANSWER KEY

1.A; 2. D; 3. D; 4. B: 5. C; 6. D; 7. C; 8. E; 9. D; 10. B; 11. D; 12. B; 13. E; 14. C; 15. D.

IROSSI SUPPOPTINGI PEDIATRiC�EDUCATION

Printed in USA.

Pediatrics in ReviewVol.17 No.11November1996EDITORRobert J. HaggertyUniversity of RochesterSchool of Medicine and DentistryRochester, NY

Editorial Office:Department of PediatricsUniversity of RochesterSchool of Medicine and Dentistry601 Elmwood Aye, Box 777Rochester, NY 14642

ASSOCIATE EDITORLawrence F. NazarianPanorama Pediatric GroupRochester, NY

CONSULTING EDITOREvan Chamey, Worcester, MA

EDITOR, IN BRIEFHenry M. Adam, Bronx, NY

ASSOCIATE EDITOR, IN BRIEF

Tina L. Cheng, Washington, DC.

EDITORIAL CONSULTANT

Victor C. Vaughan, III, Stanford, CA

EDITORIAL BOARDHoover Adger Jr., Baltimore, MDRussell W. Chesney, Memphis, TNPeggy Copple, Tucson, AZJames J. Corrigan Jr., New Orleans, LABrahm Goldstein, Portland, ORJohn L. Green, Rochester, NYWalter Huurman, Omaha, NEJohn Kattwinkle, Charlottesville, VAKathi Kemper, Seattle, WAJohn T. McBride, Rochester, NYLawrence C. Pakula, Timonium, MDKenneth B. Roberts, Worcester, MABradley M. Rodgers, Charlottesville, VAAllen W. Root, Tampa, FLLawrence Schachner, Miami, FLGail Shapiro, Seattle, WAFrank R. Sinatra, Los Angeles, CAMartin T. Stein, LaJolla, CAPaul N. Thiessen, Vancouver, BCJon Tingelstad, Greenville, NCTerry Yamauchi, Little Rock, AR

EDITORIAL ASSISTANTSydney Sutherland

PUBLISHERAmerican Academy of PediatricsOlle Jane Z. Sahler, MD, Director

Department of EducationJo A. Largent, Director

Division of Medical JournalsDeborah Kuhlman, Copy Editor

PEDIATRICS IN REVIEW (ISSN 0191-9601) is ownedand controlled by the American Academy of Pediatrics.Itis published monthly by the American Academy ofPediatrics, 141 Northwest Point Blvd, P0 Box 927, ElkGrove Village, IL 60009-0927.

Statements and opinions expressed in Pediatrics inReview are those of the authors and not necessarilythose of the American Academy of Pediatrics or itsCommittees. Recommendations included in this pub-lication do not indicate an exclusive course of treat-ment or serve as a standard of medical care.

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The printing and production of Pediatrics in Review ismade possible, in part, by an educational grant fromRoss Products Division,Abbott Laboratories.

I I IN BRIEF �

Reactions to lodinated Contrast MediaPIR QUIZ

1 . The most common cause of chronic

diarrhea in the 6- to 36-month-old

child is:A. Chronic nonspecific diarrhea.B. Disaccharidase deficiency.

C. Enteric infection.D. Malabsorption.

E. Protein intolerance.

2. Which of the following features is

most characteristic of chronic non-

specific diarrhea?

A. Abdominal pain.B. Fever.

C. Flatulence.D. Normal growth.

E. Vomiting.

3. Which of the following interventions

is most appropriate in the manage-

ment of a child who has chronic non-

specific diarrhea?A. Increase dietary fructose.B. Increase total fluid intake.

C. Reduce dietary fiber.D. Reduce dietary sorbitol.

E. Reduce fat intake.

4. The most valuable laboratory test toestablish the diagnosis of chronic

nonspecific diarrhea is:

A. D-xylose absorption test.

B. Fresh stool specimenexamination.

C. Intestinal biopsy.D. Senim albumin measurement.E. Sweat test.

5. The stools of a patient who has chron-

ic nonspecific diarrhea are character-ized by each of the following, except

that the stools

A. Are not passed during sleep.B. Are watery.

C. Contain blood.D. Contain undigested food.

E. Occur most frequently in the

morning.

Pediatrics in Review Vol. 17 No. 11 November 1996 385

Reaction to Radiographic Contrast Media.Walker AC, Carr DH, Br J Radiol.

1986;59:53 1-536

Adverse Reaction to Ionic And Non-IonicContrast Media. Katayama H,

Yamaguchi K, Kozuka T, et al. Radiology.

1990; 175:621-628

Pretreatment With Corticosteroids toPrevent Adverse Reactions to Non-Ionic

Contrast Media. Lasser EC, Berry CC.

AiR. 1994;162:523-5l6

Pretreatment With Corticosteroids to

Alleviate Reactions to Intravascular

Contrast Media. Lasser EC, Berry CC,

Tamer LB. N EngI J Med. 1987;317:845-

849

A Review ofThe Toxicity of Non-Ionic

Contrast Agents in Children. Cohen

MD. Invest Radiol. 1993;28:587-593

Comparison of the Rates ofAdverse DrugReactions. Wolf GL, Mishkin MM, Roux

SG, et al. InvestRadiol. 199l;26:404-410The Allergic Theory of Radiocontrast Agent

Toxicity. Brasch RC, Caldwell JL. Invest

Radiol. 1976;2:347-356

Intravascular Contrast Media-the Past,Present and Future. Grainger RG. Br J

Radiol. 1982;55:l-18

The exact mechanism responsible for

adverse reactions to iodinated con-trast administered intravenously dur-ing radiologic evaluation is not

known. However, through work thathas been performed both in the labo-

ratory and in clinical trials, we doknow that the types of reactions arevariable and the underlying etiologiesmultifactorial. Moreover, reactions

can be prevented in many casesthrough careful choice of contrastagent and pretreatment with cortico-steroids and antihistamines.

Adverse contrast reactions can beseparated into two broad categories:chemotoxic and anaphylactoid.

Chemotoxic reactions are dose-dependent and related to direct chem-ical effects of the contrast medium.Pain at the injection site and flushing

belong in this category; a combina-lion of hypertonicity and calcium

binding results in vasodilatation.Nausea and vomiting likely are

chemotoxic reactions, but that

remains controversial. Reactions thatare not dose-dependent and appearsimilar to allergic hypersensitivity areclassified as anaphylactoid. Urticaria,bronchospasm, and angioneuroticedema are examples.

In general, investigators categorizereactions according to their level of

severity. Nausea, vomiting, diaphore-

sis, limited urticaria, and pruritushave been considered minor reactions

and usually require no treatment.Moderate reactions include facial

edema, extensive urticaria, broncho-

spasm, or laryngeal spasm not requir-ing intubation. Although treatmentmay be necessary, these reactionsusually are self-limited. Severe reac-

tions include hypotension, cardio-vascular collapse, bronchospasm,laryngeal edema requiring intubation,

and any reaction severe enough towarrant hospitalization. Patients at

increased risk for adverse reactionsinclude those who have a history of

reaction to contrast, major allergies,and asthma.

Although many reactions to iodi-

nated contrast appear similar to thoserelated to allergic hypersensitivity,they have not been proven to be anti-

gen/antibody-mediated. In 1976,Brasch demonstrated a higher rate of

binding of radiolabeled contrastmedia in the globulin fraction ofpatients who had reactions. He also

identified the specific antibodies tocontrast media in rabbits. Yet, both

Lasser and Can were unable to detectany evidence of specific immuno-globulin (Ig)E or 1gM related to con-

trast media in either animal or humanreactors. Iodinated contrast has beenshown to have a direct effect on mast

cells and basophils, causing releaseof histamine. Robertson and cowork-

ers demonstrated elevated plasmahistamine levels in 80% of patientswho received contrast. They were

unable to correlate these levels withclinically apparent adverse reactions.

Others have demonstrated histaminerelease along with bradykinin andfibrin split products through activa-tion of the compliment system,which has been shown experimental-ly to produce symptoms similar to

anaphylactic shock.Since 1929, when Moses Swick

introduced the first intravenous con-trast agent into clinical practice,much work has been done to decrease

the incidence of reactions. One majorstrategy has been to reduce the

chemotoxicity of contrast media. In1968, Alm#{244}npostulated that thehyperosmolality of contrast was the

major cause of reactions. Since then,

394 Pediatrics in Review Vol. 17 No. 11 November /996

NEUROLOGYMovement Disorders

retested for neuroblastoma at inter-

vals if the first evaluation is negative.

Periodic reassessment may be mdi-cated in secondary dyskinesias forwhich no etiology has been found.

SUGGESTED READINGAfihi AK. Basal ganglia: functional anatomy

and physiology. J Child Neurol. 1994;9:

249-260, 352-361

Campbell M, Grega DM, Green WA, Ct al.

Neuroleptic-induced dyskinesias in chil.

dren. CliiiNeumpharmacol. 1983;6:

207-222

Cosgrove AP, Cony IS. Graham HK. Botulinum

toxin in the management of the lower limb

in cerebral palsy. Dcv Med Child Neural.

1994;36:386-396

Fahn S. Janakovic J. Practical management of

dystonia. Neural Cliii.1984:2:555-569

Janakovic J, Brin MF. Therapeutic uses of botu-

linum toxin. NEnglfMed. l991;324:

1186-1194

Kiessling LS, Marcotte AC, Culpepper L.

Antineuronal antibodies in movement disor-

ders. Pediatrics. I 993;92:39-43

Klawans HL, Brandabur MM. Chorea in child-

hood. In: Pranzatelli MR. ed. Movement

disorders. Pediatr Ann. 1993;22: I 3-68

Leckman iF, Cohen Di. Descriptive and diag-

nostic classification of tic disorders. In:

Cohen Di, Bruun RD. Leckman iF, eds.

Tourette ‘S S%’iidroine �uid Tic Disorders. Clinical

Understanding and Treatment. New York,

NY: John Wiley & Sons; 1988: 4-19

Marsden CD. Investigation of dystonia. Ads’

Neurol. l988;50:35-44

Mastaglia FL. latrogenic (drug-induced) disor-

ders of the nervous system. In: Aminoff Mi,

ed. Neurology and General Medicine. New

York, NY: Churchill Livingstone; 1989:

505-532

Pranzatelli MR. The immunopharmacology of

the opsoclonus-myoclonus syndrome. CliiiNeuropharmacol. 1996; 19:1-47

Pranzatelli MR. Update on pediatric movement

disorders. Ads’ Pediatr 1995:42:415-463

Pranzatelli MR. Mott SH, Pavlakis SG, et al.

Clinical spectrum of secondary parkinson-

ism in childhood: a reversible disorder.

Pediatr Neural. 1994; 10:131-140

Singer HS, Brown J, Quaskey 5, et al.The

treatment of attention-deficit hyperactivity

disorder in Tourette’s syndrome: a double-

blind placebo-controlled study with cloni-

dine and desipramine. Pediatrics. 1995:95:

74-81

The Tourette Syndrome Classification Study

Group. Definitions and classification of tic

disorders. Arc/i Neural. l993;50: 1013-1016

Weiner Wi, Lang AE. Gilles de Ia Tourette syn-

drome. In: Movement Disorders: A

Comprehensive Sun’ev� Mount Kisco, NY:

Futura Publishing Co. mc; 1989:531-568

PIR QUIZ

6. Which one of the following state-

ments regarding tic disorders is true?

A. Behavior modification is animportant component of therapy.

B. Girls are more affected than

boys.

C. Magnetic resonance imaging

frequently demonstrates the

etiologic abnormality.

D. Movements are exaggerated with

anxiety.

E. Voluntary activities requiring

concentration worsen abnormal

movements.

7. A 2-month-old child presents with

nonrhythmic, spontaneous, multidirec-

tional, chaotic eye movements andjerky movements of extremities thatwere first noticed 1 month ago. He

appears interactive during these move-ments. Which one of the following

conditions is most likely associatedwith these symptoms?

A. Epilepsy partialis continua.B. Infantile spasms.

C. Neuroblastoma.

D. Sandifer syndrome.

E. Tay-Sachs disease.

8. Which of the following is associated

with Gilles de la Tourene syndrome?

A. Brief periods of loss of

consciousness.

B. Gastroesophageal reflux with

torticollis.

C. Infantile spasms.D. Laboratory evidence of prior

streptococcal infection.

E. Obsessive/compulsive behavior.

9. Immunomodulation with intravenousimmune globulin (IVIG) should be

considered in:A. Drug-induced dyskinesia.

B. Gilles de la Tourette syndrome.

C. Infantile spasms.

D. Rheumatic chorea.

E. Sandifer syndrome.

10. A 4-year-old child presents with facial

distortion, torticollis, and opisthotonusfollowing accidental ingestion of

prochlorperazine. Which one of the

following drugs is most appropriate

for treatment?A. Clonidine.

B. Diphenhydramine.

C. Halopendol.D. Lorazepam.

E. Phenobarbital.

FLUIDS & ELECTROLYTESClinical Aspects

PIR QUIZ

11. The most correct statement regarding

oral rehydration therapy (ORT) is:

A. All rehydration solutions contain

equivalent amounts of glucose

and electrolytes.B. Commercial “Gatorade” with a

sodium concentration of

20 mEq/L is an acceptable oral

rehydration solution.

C. Continuing diarrhea is a contra-

indication to the use of ORT.

D. Oral rehydration solutions should

be given at a rate of 100 mLikg

over 6 hours for moderate

dehydration.

E. Use of ORT is limited to patients

older than 12 months of age.

A. Intravenous administration of

0.45% saline in glucose at a rateof 20 mL’kg over 12 hours.

B. Intravenous administration of 3%

saline calculated to raise serumNa� by Ito 2 mEq/hr over8 hours.

C. Intravenous furosemide 2 mg/kg

to promote increased extracellu-larosmolarity by water loss.

D. Intravenous phenobarbital in aloading dose of 15 to 25 mg/kg

followed by intravenous fluids

containing 0.6% NaCl.

14. A 4-year-old girl is admitted to the

hospital with a history of repeatednonbilious vomiting for 2 days. There

is no history of fever or diarrhea. Onphysical examination, dehydration is

estimated to be moderate. The girl is

somnolent but recognizes her parents.

Her breathing is slightly labored.

Admission serum electrolytes are:

Na�, 134 mEq/L; Cl-, 98 mEq/L;

HCO#{231}, 14 mEq/L; and BUN,

42 mg/dL. Arterial pH is 7.25. By use

of the formula for calculation, the

anion gap is estimated to approximate:

A. 4mEq/L.B. l2mEq/L.

C. 22mEqIL.

D. 34mEq/L.

12. An 18-month-old boy is admitted to

the hospital with a history of diarrhea

and vomiting for 3 days. On physical

examination he is unresponsive and

has occasional twitching of the armsand legs. Dehydration is estimated to

be 10%. Admission serum electrolytes

are: Na’, ll5mEqIL;C1,83mEq/L;

HCO3, 15 mEq/L; and BUN, 44 mg/dL.On a randomly obtained urine, the

specific gravity is 1.022 and Na� is

12 mEq/L. The most appmpriate

immediate management for this

patient is:

13. All of the following statements about

hypernatremic dehydration are true

except:

A. An initial fluid infusion of

20 mlJkg of body weight is need-

ed to restore effective plasmavolume.

B. Correction of elevated serum

sodium should proceed at a rateof 10 mEq/L or less over each

24 hours.

C. Daily maintenance requirementsof sodium at 3 mEq/kg are needed.

D. Intracellular osmolarity andextracellular osmolarity are bal-

anced due to the generation ofintracellular “idiogenic osmoles.”

E The presence of excess body

sodium mandates use of dcc-trolyte-free solutions for intitial

rehydration.

15. For the patient in question 14, the

combination of the clinical picture and

the estimated anion gap is most con-

sistent with the diagnosis of:

A. Acetaminophen intoxication.

B. Acute renal tubular necrosis.

C. Acute respiratory alkalosis.

D. Diabetic ketoacidosis.

E. Meningitis with inappropriate

antidiuretic hormone.

404 Pediatrics in Review Vol. 17 No. 11 November 1996

POlNT-COUNTERPOINT�-�

Acute Asthma Therapy

A reader noted: “I am surprised tofind no mention of inhaled steroids inthe article by Drs. Murphy and Kelly

‘Advances in Management of AcuteAsthma in Children’ (July 1996).Their article is the only one I havecome across that avoids mentioningthe use of inhaled corticosteroids inchildren during the last 4 to 5 yearswhen there seems to be a concerted

effort to get pediatricians to usethem.”

Drs. Kelly and Murphy respond:“We appreciate your concerns about

our lack of discussion of inhaled cor-

ticosteroids. However, our charge for

the article was to review the currenttreatment of acute exacerbations ofasthma, including exercise-induced

bronchospasm. As inhaled cortico-steroids currently are not indicatedfor the treatment of acute exacerba-tions or for protection against exer-cise-induced asthma, we did notinclude them in our discussion. As

you pointed out, their use is beingpromoted for childhood asthma andas early intervention to alter the

course of the disease. This is receiv-ing quite a lot of interest. However,

there are still unanswered questionsabout inhaled corticosteroids in chil-

dren that would take an entire mono-graph to outline and would be suit-

able for an article in Pediatrics in

Review. Until that time, might wesuggest a recent article that providesan excellent summary of the issuessurrounding inhaled steroid use:Kamada, Szefler SJ, Martin RJ, et al.

Issues in the use of inhaled glucocor-ticoids: The Asthma ClinicalResearch Network. Am J Respir Crit

Care Med. l996;l53:l739-1748.”