Practical Strategies

in Pediatric Diagnosis
and Therapy
Practical Strategies
in Pediatric Diagnosis
and Thera~vsecond Edition
Edited by
Robert M. Kliegman, MD
Professor and Chair
Department of Pediatrics
Medical College of Wisconsin
Pediatrician-in-Chief
'Pam and Les Muma Chair in Pediatrics
Children's Hospital of Wisconsin
Milwaukee, Wisconsin

Larry A. Greenbaum, MD, PhD

Associate Professor
Department of Pediatrics
Medical College of Wisconsin
Children's Hospital of Wisconsin
Milwaukee, Wisconsin

Patricia S. Lye, MD
Associate Professor
Department of Pediatrics
Medical College of Wisconsin
Children's Hospital of Wisconsin
Milwaukee, Wisconsin

ELSEVlER
SAUNDERS
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PRACTICAL STRATEGIES I N PEDIATRIC DIAGNOSIS AND THERAPY ISBN:&721M131-5

Second Edi~ion
copyright 0 2004,1996, Elsevier Inr. A l l rights rerervrd.

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II NOTICE

Medicine i s an ever-changing field. Smdard safety precavlionr must be followed, b a as new mearch and I
I clinical experience broaden &r knowledge. changesin UeatrnePand drug therapy may become nceerrary or
I appropriate. Readen are advised to chcck h c most cumnl pmducl informalion provided by the manufaclurm
oftach drug to be admininccedl o verify the recommended dose. the methd and dwalion af adminisrralion,
and convd~ndiea~.on~ I t or the nrpanrtbol~ryd t h c licenvd prescriber, relying on clpenrnce and houlcdge
of the plrienl. lo dctemune dolager and the bcrl m m e n l for each tndniaral paucnl. Nclther the publirhcr
nor thc authors assumes any l~ab!lttyfor an) %"jury andlor damage to penonr or pmpeny xiring fmm thir

Firs1Edirion copyrighted 1996.

Library of Cong- Calaloging-in-PublicdtionDala

Racusal svdlegier in pediawic diaporir and therapy I[ediled by] RobenM. Kliegman.
Larry A. Greenbaum. Parricia S. Lye.-2nded.
p. ; cm.
Includes biblio~aphlcalreferences and index.
ISBNC-7216-9131-5
I.PediaUicr-Decision making. 2. Pediarricr. I.Kliegman. Roben. U. Greenbaum.
Larry A. 1U. Lye. Pamcia S
[DNLM: 1. Pediatrics. WS 200 P895 2004)
RJ47.P724 2004
618.92--dc22

Errcurwe Publtrlner h i m Flrtcltri

Eduorr; Wettdy Buckwaiter CatfmullDana Lampxrellu
D~t~rlop,nenlol
Sdnto, Pmjer! Mmogrr. Robin 6 Davtr
Book Designer: Gene H&r

Printed in the Unired Slalcr of America

Last dizil i s the print number: 9 8 7 6 5 4 3 2 1

This book is dedicated to those master clinician-educators
who have inspired us with their clinical wisdom,
enthusiasm, empathy, and insight. At no time in the history of
pediatrics have these adaptable master clinician-educators
been needed more to inspire.young students and residents and
to provide encouragement and clinical guidance to the
practicing pediatrician.

In this light, we dedicaterthis edition to the memory of

Dr. David A. Lewis, Associate Professor of Pediatrics,
Director of Residency Training, Pediatric Cardiologist, and
master clinician at the Children's Hospital of Wisconsin.
His teaching will be missed by us all.
 Contributors

Uri S. Alon. M D R. Alexander Blackwwd, MD. PhD

Rofessor of Pediatrics. University of Missouri at Kansas City Associate Professor of ~ediatric.~
and Pedialric Infectious Diseases.
Schml of Med~cine:Pediluic Ncphrologist and Dimtor. Bone and University of Michigan Medical School. Ann Arbor. Michigan
Mineral Disorders Clinic. Ch~ldrcn'sMercy H o s v ~ d Kansas
. City.
Missouri
Acid-Enre ond Elrrrmlyre Dirrurboncer
Andrew Blea~el,MBBS, PhD
Staff Specialist, Neurology and Neurophysiology, Westmead
R. Stephen S. Amato, MD,PhD Hospital and Children's Hospital at Werunead. Sydney. Auslralia
Clinical Professor of Pediatrics.
~~~.Tufts
~- Univenitv
-~ ~
~.School of
Medicine, Boston. Massachusem; Chief, Pediamcs Service and
Pomryrmal Dirordrrr

Director. Medical Genetics. Eastern Maine Medical Center. Bangor.

.
Maine Laurence A. Boxer, MD
Gymorphology Henrv and Mala Dorfman Familv Professor in Pediatric
~ e m ~ t o l o ~ ~ l ~ nUniversity
c o l o ~ ~of, ~ i c h i ~ aMedical
n School;
Director. Pediatric HematologylOncology. C. S. Matt Children's
Stephen C. Amsoff, MD Hospital. Ann Arbor. ~ i c h i &
Professor and Chair. Depanment of Pediatrics, Temple University Recurrent Infection
Schml of Medicinc; Temple University Children's Medical Center.
Philadelphia. Pennsylvania
F n v r o f U n b o ~ aOrigin
Ben H. Brouhard, MD
Professor of Pediauics and Asscciare Dean, Case Western Reserve
University School of Medicinc; Executive Vice Resident of Medical
Jane P. Balm& MD Affain and Chief of StaK, MeuoHealth System. Cleveland. Ohio
Hemrurio
Clinical Associate Professor. Ohio Slate University; Pediatric
Gas@centemlo~isL Columbus Children's Hospital. Columbus. Ohio
Gale R. Buntein, MD, MPH
Medical Officer, Division of HIVIAIDS Prevenlion. Centers for
Sharon Bartash, MD Disease Conuol and Prevention. Atlanta, Georgia
Seruolly Trammined Direores
Associate Professor of Pediatrics, Universiry of Wtsconsin School of
Medicine; Chief, Division of Pediavic Nephrology. University of
Wisconsin Children's Hospital. Madison. W~sconsin -ma1 Chadha, MD
H?penemion
Assisrant Professor of Pediatrics. Wrginia Commonwealfh University
School of Medicine: Chair. Section of Pediatric Neohroloev. Vireinia
Commonwealth Untverslty Medtcal Cenler. ~ichmhnd,V&nta
Stuart Berger, MD Ac,d.Barc and Ekccmlgre D~r,l,rboncer
Professor of Pediamcs. Medical College of W~sconsin; Medical
Director. Henna Hean Center, Children's Hospital of W~sconsin.
Milwaukee. Wisconsin John C. Chandler, MD
H c o n Foilare Pediatric Surgeon. Children's Hospital of the Greenville Hospital
System. Greenville. South Carolina
Abdorr,inol Masrer
Brian W. Berman, MD
Professor of Pediauics, Case Western Reserve University School of
Medicine; Vice Chair for Community-Physician Affairs and Chief, Bruce H. Cohen, MD
Division of Ccncral Acadcmic Pediauics. Rainbor?. Babies and Staff, S=c!ic: nf Pediatric Neurology. Depanment of Neurology
Children's Hospital. Cleveland. Ohio Cleveland Clinic Foundation. Cleveland, Ohio
Lyrnpliodenopor1,y: Pallor ond Auemio Hendocl,ar in Cl~ildl~ood

David J. Beste, M D Roberr J. Cunningham 111, MD

Medical Director, Speech and Audiology. Children's Hospital of Chajr, Medical Subspecialty Pediavics and Head. Section Of
Wisconsin. Milwaukee. Wisconsin Pediatric Nepiuology. Cleveland Clinic Foundation. Cleveland. Ohio
Neck Morrer b Cl>ildl~ood Proreinurio

vii
 Leona Cuttler, MD Lany A. Crcenbaurn. MD, PhD
C:ISCWestern Reserve Universitv Schwl of
Professor of Pcdiatrics.. . Associate Professor. Dcpanment of Pediauics. Medical College of
Medicine: Chief. Division of Endocrinology. ~ i a b e t e s , and Wisconsin; Children's Hospital Of Wisconsin. Milwaukee.
Metabolism. Rainbow Babies and Children's Hospital. Cleveland. Wirconsin
Ohio Drlirivnn ond Coma
Shorr Srolsre

Marjorie Greenlield. MD
Jack S. Elder, MD ~ss&ialeProfessor of Reoroductive Biolorv. -, - .-
. Case Western
~ ..
~ .. Q -~ N ~
R...C
Professor of Pediatrics and Caner Kissell Professor of Urology. Case ~niversitySchool of ~ e d i c i n e :Associate Professor of Obsletrics
Western Rcserve University School of Medicine; Director. Pediatric and Gynecology and Pediatrics. University Hospitals of Cleveland.
Urology. Rainbow Babies and Children's Hospital. Cleveland. Ohio levela and. oh&
Ararrr o ~ t dCl~rnnicScmrel Stwlling: Anjbiy#rrrr,r Ceniioli~ Mcrrs!mrol Pmblrzrzr and Vugbtol Bleedzng

Susan Feigelman, MD Ajay Cupta, MD

Associate Professor of Pediatrics. University of Maryland Schwl of Staff. Section of Pediatric Neurology and Epilepsy. Department of
Medicine. Baltimore. Maryland Neumlogy, Cleveland Clinic Foundation. Cleveland. Ohio
Failure to Tlzn'l,ew d Molnn,ritivn Heodochrr in Childhood

Thomas Ferkol. MD Peter L. Havens. MD

Associate ~mfes'sorof Pediatrics. Washington Univenity School of Profasor of ~ebiatricsand Epidemiology. Medical College of
Medicine; Director. Cystic Fibrosis Center. St. Louis Children's Wisconsin, Consultant in infectious Diseases. Children's Hospital of
Hospital. St. Louis. Missouri Wisconsin. Milwaukee. Wisconsin
Rerpiroro? Dirrrrrr Me,,insis,nur and Menlngirir

Michele A. Fmmmelt. M D
Associate Professor of Pediatrics. Medical College of Wisconsin: .
leffrev S. Hvams.. MD
Professor of Pediatrics. Univers~ly of Connecticut School of
Children's Hospital of Wisconsin. Milwaukee. Wisconsin Medeine. Fmington. Connecttcut. Head. Diviston of Digestive
C?onosir
Dtscases. Connecticut Ch~ldren's Medical Center. Hariford.
Connecticut
Corrminrerrinol Bleeding
Peter C. Fmmmelt. MD
Associate ~rofesso; of Pediatrics. Medical College of Wsconsin;
Director of Pediauic Echocardiography, Children's Hospital of
David M. Jaffe, MD
Wisconsin. Milwaukee. Wisconsin
Dana Brown Professor of Pedialrics, Washington University School
of Medicine: Director, Division of Emergency Services, St. Louis
Childnn's Hospital. St. Louis. Missouri
Fever n.irhour Focus
Michael W. L. Gauderer. MD
Professor of Sur~erv. University of Soulh Carolina Schwl of
Medtctne ~ d ~ u n cProfessor
t of R~oengtnrenng. Clemson
Un~verstly. Chtcf. Pedlatnc Surgery. Children's Hospital of 0Ic Candice E. Johnson, hlD, PhD
Grecnvtllc Hosp~ralS)stcrn. Grecnv~llc.Sou01 Carollnn Professor of Ped~ntnc,. Untvcrrrty of Colorado School of Mcdtclne.
Atrendane
-Phvsictan
. Ch~ldren'cHosrr~tal.Lknver. Coloildo
Dy,r,,"a

Mitchell E. Geffner, XlD

Professor. Univcrstly of Southern Callfomia Keik School of -
Hueh F. .lohnston.
- MD
.
Medicine:. Phvslc~anand Director of Fcllowsl~tnTrainine.
-. Divtsion ~ ~~
Professor, Depanrnents of Psychiatry and Educational Psychology.
University of Wisconsin Medical School: University of Wisconsin
of Endocrinology, Diabetes, and Metabolism, Childrens Hospital
Los Angeles. Los Angeles. California Hos~italand Clinics. Madison. Wisconsin
Disorders of P~rbmy

Manju E. George, MD Virginia Keane, im

Resident in Dermatolopy.
- University of Kansas Medical Center. Associate Professor of Pediatrics. University of Maryland School of
Kansas City. Kansas Medicine. Baltimore. Maryland
RorAer orld Skbr Leriorrr Foilurc ro Tlzrivf oud Molnurri!io,r

William M. Gershan, MD cirolyn M. Kercsmar, MD

Associate Professor of Pediatrics, Medical College of Wisconsin; Professor of Pediatrics. Case Western Reserve University School af
Pedirtric Pulmonologist. Children's Hospital of Wisconsin, Medicine; Director. Children's Asthma Cenicr. Rainbow Babies and
Milwaukee. Wisconsin Children's Hospital. Cleveland, Ohio
Cozagh Rcrpirotor). Disiress
 ~obeA M. Kliegman, MD Amy Jo Nopper, M D
Professor and Chair, Department of Pediatrics. Medical College o f Associate Professor of Pediatric Dermatology. University of
Wisconsin; Pediatrician-in-Chief and Pam and Les Muma Chair in Missouri-Kansas City School of Medicine; Chief. Section of
Pediatrics. Children's Hospital of Wisconsin. Milwaukee. Wisconsin Pediatric Dermatology. Children's Mercy Hospital. Kansas City.
Ainmy Obrrrucrion in Children; Acute and Chmnic A b d o m i ~ Poin
l Missouri
Rarhrr and Skin LerioJlr

Subra Kugathasan, M D
Associate Professor of Pediatrics. Medical College o f Wisconsin; Susan R. Orenslein. M D
Children's Hospital of Wisconsin. Milwaukee. Wisconsin hfessor of Pediatrics. Division of Pediatric Gastroenterology.
Diarrhea University of Pittsbursh School of Medicine: Children's Hospital of

Roberl M. Lembo, M D
Associate Professor of Clinical Pediatrics and Director. Medical
Education. Department of Pediatrics. New York University School of Michael J. Painter, M D
Medicine; Anending Physician. Bellewe Hospital Center. New York. Professor of Neurolorv and Pediatrics. Division o f c h i l d Neuroloev.
New York University of ~ i t t s b u ~
School
~ h of ~ e d i c i n e Children's
; ~os~itaihf
Fmrrond Raria Piasbureh. Pittsburgh. Pennsylvania
~ ? ~ o r o nand
i a ~oknkr;;rr

David A. Lewis.,~~~
~ ~~ ~~ ~ MD
~ ~~

Associate h f e s s o r of Pediatrics, Division o f Pediatric Cardiology: Cynthia G. Pan, M D

Director. Graduate Medical Education F'ro~ram.Medical College of - Associate Professor of Pediatrics. Medical College o f Wisconsin;
Wisconsin. Milwaukee. Wsconsin f ~ e c e o ; e d l -.
Medical Director. Dialvsis Unit and Neohroloav. Children's Hosoital
of Wisconsin. Milwaukee. Wisconsin
Pol?uria and Urino? I,?conrinence

Gregory S. Liplak, MD, MPH

hfessor of Pediatrics.. Universitv o f . Rochester Medical Center. Andrew N. Pelech, M D
Anendig Physician. Svong Memorial Hospital, Rochester. New York Asmiate ~rofesso; of Pedialrics. Division of Cardiology, Medical
Mcnrol Rerordorion and Dweloprnenrnl Dirobiliry College of Wisconsin; Children's Hospital of Wisconsin. Milwaukee.
Wisconsin
Heon So,,ndr and Mnmrnrr
Patricia S. Lve. M D
Associate &feisor. Depanment o f Pediatrics. Medical College o f
Wisconsin; Children's Hospital of Wisconsin. Milwaukee. W~sconsin John M. Pelen, DO
Assislant Professor o f Pediatrics, Division of Pediatric
Gastroenteroloev. Universitv o f Pittsbureh School o f Medicine:
Children's ~ o s p i t aof
l ~ittsdurgh.~itlsburgh.Pennsylvania
Saleem I. hlalik, MD Vomiring and Regurg;ra,ion
Associate Director, Comprehensive Epilepsy Center, Cook
Children's Hospital. Fort Wonh. Texas
H?poronio ond Wr.brrrr Emory M. Pelrack. M D
~ssociatec l i n i c a l Professor. Depanment of Pediatrics. Case
Western Reserve University School of Medicine; President, Pelrack
KeUy W. Maloney, MD Consuldng. Inc.. Cleveland. Ohio
Assistant Professor of Pediatrics, Medical College o f Wisconsin, 77,e Irrimbie Infan,
Milwaukee, W~sconsin
Splenornegol?
Philip A. Pizzo, M D
Professor of Pediatrics and of Microbiology and Immunology; Dean.
Andrea C. S. McCoy, M D Stanford University School of Medicine, Stanford. California
Associate Professor o f Pediatrics. Temple University School o f F e ~ r and
r Neurmperria
Medicine and Temple University Children's Medical Center.
Philadelphia. Pennsylvania
F m r of Unhoxla Origin Robert M. Reece, M D
Clinical Professor of Pediatrics, Tufts University School of
Medicine; Visiting Professor of Pediatrics, Danrnouth Medical
Daniel W. McKenney, M D School: Director of Child Protection Program, The Floating
Associate Professor of Pediatrics, Nephrology and Hypenension Hospital for Children at New England Medical Center. Boston.
Division. University of Louisville. Louisviile, Kentucky Massachusetts
Re~lolFailure Cl~ildAblrse

James J. Nocton, M D Michael .I.Rivkin. M D

Associate Professor of Pediarrics. Medical College of Wisconsin; Awriate Rofcssor of Neurology. Hanard Med.cd Sctlmll. Aucnd.n:
Director, Pediatric Residency Training Program. Children's Hospital Physician. Depanment of Neurolog). Dlrector. Develupmenlal
of Wisconsin. Milwaukee, Wisconsin Neuro~maelncL ~ h o r ~ l nCn~ldrcn's
n. Ilosn~ml.Rurron. Marrachu\r.n,
Arrlwilis
 Mark S. Ruttum, MD Francisso A. Sylvester, MD
Professor of Opl~thalmology.Medical College of Wisconsin; Chief Associate Professor of Pediavics. University of Connecticut School
of Pediatric Ophthalmology. Children's Hospital of Wisconsin. of Medicine: Pediavic Gasvoenteroloeist. Connecticut Children's
Milwaukee. Wisconsin Medical ~ e n l e rHanford.
,
Cor,minrerrinal Bleeding
Connecticut -
Eye Di.ro"icrs

John R. Schrcibcr, MD, MPH Robert R. Tanz, MD

Professor of Pediatrics and Pathology. Case Western Reserve Professor and Director of Medical Education. Deoanrnent of ~ ~

University School of Medicine; Chief. Division of Infectious Pedt~trics.Nonh*erlem University Fe~nbcrgSchool of Medtcine.
Diseases. Allergy. Immunology. and Rheumatology. Rainbow Babies Attending Phys;c,an. Division of General Academic Pediatrics.
and Children's Hospital. Cleveland. Ohio
L~,"pl,u,l~,,,,p<,rl,~
Cnildren's Memorial Hosrr~tal.
Sore Tlmor
-
. Chicaro. llltnots

J. Paul Scolt. MD John G. Thome@ MD

Professor. Medical Colleee of Wisconsin: Attendina Physician.
Children's Hospital of isc cons in. Milwaukee. isc cons in
Bleeding and T l ~ m a ~ b o r i . ~
SLadord T. Shulman. MD
Professor of Pediatrics. Nonhwestern University Feinberg Schwl of
Medicine; Chief. Division of Infectious Diseases. Children's
Memorial Hospital. Chicago. Illinois
Som T1,mot
G a r w S. Sieman. MD
~rofeisor.~ i n h w e s t e r nUniversity Feinberg School of Medicine;
Director, Adolescent Medicine. Evanston Nonhwestern Healthcare,
Evanston. Illinois
Cherr Pain
Mark L. Splaingard, MD
Pmfessor of Pediatrics. Medical College of Wisconsin: Director of
Pediatric Pulmonary Care. Children's Hospital of Wisconsin.
Milwaukee, W~sconsin
Apnca and Sudden lnfnnr Deorl! Syndmme
Professor of Onhopaedic Surgery. Medical College of Wisconsin;
Chief. Pediatric Orthopaedic Surgery and Medical Director,
Onhopaedic Surgery. Children's Hospiul of Wisconsin. Milwaukee,
Wisconsin
Back Pain in Cltildmn and Adolcscentr
George H. Thompson, MD
Professor of Orlhopaedic Surgery and Pediatrics. Case Western
Reserve University School of Medicine: Director. Pediatric
Onhapaedics. Rainbow Babies and Children's Hospiml, Cleveland,
Ohio
Cair Dirrurbonces
George F. Van Hare. M D
Assoclatc Professo# of Pedmtncs. Stanford Unlvcrslry School
of Medtc~nc.Dtrcctor. Pedtatnc Anhythrnta Center. Lucrle Packard
Chtldrcn's Hosplral at Sunford Untverslty Medncal Center. Dlroctor.
Pdtarnc hrrhythnua Center. Untvrrsnty of Caloforn~a.San Franclcco.
Ch~ldren'sHoso~lal.San Franctsco. Cal~forn8a
Polpirarionr and krrhyrhmiar

Kristine C . Williams, MD, MPH

Charles A. Stanley, MD Instructor of Pediatrics. Division of Pediatric Emergency Medicine.
Professor of Pediatrics. University of Pennsylvania School of Washington University School of Medicine. St. Louis, Missouri
Fever virhour Focu
Medicine; Chief, Division of Endocrinology. Children's Hospital of
Philadelphia. Philadelphia, Pennsylvania
H ~ p o g l ~ c ~ ~ ~ ~ i ~
Martha S. Wright, MD
Associate Professor of Pediatrics. Case Western Reserve University
Rita Stellen, MD School of Medicine: Associate Director. Pediatric Emcraencv - .
Staff, Department of Pediatric Gastroenterology. Cleveland Clinic Medicine. Rainbow Babies and Children's HospitaWniversity
Faundation, Cleveland. Ohio Hospitals of Cleveland. Cleveland, Ohio
Consriporion

Frederick J. Suchv. Elaine Wyllie, MD

-.MD Head. Section of Pediatric Neurolo~yand Pediatric Epilepsy,
Professor and Chair. Department of Pedi;~trics.Mount Sinai School of
Medicine; Pediatrician-in-Chief. Mount Sinai Hospital. New York, Cleveland Clinic Foundation. ~ ~ e v e ~ aOhio
nd:
Pom.\~s,nalDisorders
New York
Hepororr!egaly

Robert \\'yllie, ML)

Chair. Department of Pediatric Gastroenterology, Cleveland Clinic
William J. Swifl, MD Foundation. Cleveland. Ohio
.-
Professor Emeritus of Child and Adolcscenl Psychiaq, University Conr!iporion
of Wisconsin Medical School and Wisconsin Psychiatric Institute
and Clinic, Madison. Wisconsin: Regional Medical Ofticer and
Psychiatrist, U.S. Depanment of St;\te. Pretoria. South Africa
Unurual Beldoviorr
 Preface
Most children's hospitals and pediatric residency training programs
have multiple educational conferences. such as professor rounds.
patient management conference. clinicopathologic conference, and
senior resident intake rounds. I n these highquality learning activi-
ties, experienced master clinician-educators lead a discussion o f a
particular patient-based issue, permitting the minces to see how a
master clinician thinks through diagnostic or therapeutic challenges.
The advice given is derived from the knowledge accumula~edover
many y e m o f clinical experience and careful analysis of the medical
literature. The synthesis o f the facts o f the case wilh lhe clinician's
practical experience and knowledge o f the literature ohen resulIs i n
the diagnosis and the appropriate treatment strategy. These master
clinician-educators provide wisdom that gives clarity to confusing
clinical cases and helps to reconcile discrepancies between practice
and lheory.
In addition, master clinician-educators focus on the imponance
o f adetailed history and a complete physical examination. The chief
complaint directs [ h i questioning during the history. wherea5 the
physical examination focuses on clues obtained by the history.
Laboratory and other studies are then employed to suppart the
diagnosis. not to make the diagnosis.
The goal o f this b ~ is kto put into a written text the oral teach-
ing rounds-based approaches toward clinical problem solving o f the
many expert clinician-educators who present at teaching confer-
ences. The combination o f clinical experience and evidenced-based
smtegies will provide guidance in developing a diffecential diagno-
sis, h e n a specific diagnosis. and finally the appropriate therapy o f
common pediatric problems. This book is manged i n chapters that
cover specific chief complaints, mirroring clinical practice. Patients
do not usually present with a chief complaint of cystic fibrosis;
rather. they may present with a cough, respiratory distress, or chronic
diarrhea.
This text is intended to help the reader begin wilh a specific chief
complaint that may encompass many disease entities. I n a user-
friendly. well-tabulated. and illustrated approach, the text will help
lhe reader differentiate between the many disease states causing a
common chief complaint. The inclusion of many original tables and
figures should help the reader identify distinguishing features o f
diseaws and work throueh a diaenostic andlor theraautic amroach
to the problem usingdecGion trees. Modified. adapt&, and & A w e d
aiwork and tables from other outstanding sources have been added
as well. The combination o f all o f these lust rations and tables will
help provide a quick visual guide to the differential diagnosis or
treatment of the various diseases under discussion.
We greatly appreciate the hard work of our contributing authors.
Writing a chapter in this type o f format is quite different from
writing i n the format o f a disease-based book. I n addition, we greatly
appreciate the efforts o f Judy Reeher of Elsevier, whose patience
and expertise contributed to the publication o f this book. Wc are all
also greatly appreciative o f Carolyn Redman o f the Depmment o f
Pediarrics at the Medical College o f Wisconsin, whose editorial
assistance and organization has made this edition a reality. The
authors also wish to make a special acknowledgment to Dr. Brendan
M. Reilly, for his courtesy and assistance. Finally, we acknowledge
the support and, at times, sacrifice of our families: Sharon. Jonalhan.
Rachel, Alison, and Matthew Kliegman: Jordan. Harry, and Irene
Greenbaum; and Dale, Erin. John, and Therese Lye, whose
underslanding helped make the time and effort put into l h i s book
meaningful.
 Contents

Section One 14. Acute and Chronic Abdominal Pain ...........................249

Respiratory Disorders R& M. Kliegman
15. Diarrhea ...................................................................... 27 I
%bra Kugah-

Rabm R. Tan2 and Sunford T Shvlman 16. Vomiting and Regurgitation ........................................ 291
Saran R. Orcnrtcin and John M. Pclrrr

William hl. Gcnhan 17. Gastrointestinal Bleeding ........................................... 323

3. Respiratory Distress ......................................................43 Fmcirco A. Sylvcrler and JelTrcy S. Hyamr

Camlyn ht. Krrcrmar and Thomar Fcrkol 18. Hepatomegaly ........................................................... 333
4.. Earache ........................................................................... 70 Frcdctick I. Suchy

F%"icia 5. Lye 19. Splenomegaly .............................................................. 345

5. Airnay Obsrructibn in Children .................................... 82 Kelly W Maloney
Robm M. K l i c p a n 20. Jaundice 53
6. Apnea and Sudden Infant Death Syndrome ................. 95 1- P Bdi",
Mark L. Splaingard . .
................................................................ 373
21. Const~pat~on
Rita SleKen md Rokn Wyllic
Section Two
Cardiac Disorders 107 22. Abdominal Masses ..................................................... 383
m a e l W L. Caudcrcr and John C. Chandlcr

7. Palpitations and Arrhythmias .................................. 109 Section Four

h a e F Vm H m Genitourinary Disorders
8. Heart Failure ............................................................ 122
SRlM Bcrgrr
23. Dysuria ...................................................................... 397
9. Chest Pain ................................................................... 148
W i E c E. lohnron
Carry S. Sigman . .
24. Protelnuna ...................................................................413
10. Cyanosis ..............:.................................................... 163
Rokben I. Cunningham 111
Michrlc A. Frommrl~and Prtcr C. Frommclc
25. Hematuria .................................................................. 423
I I. Heart Sounds and Murmurs ........................................ 178
BcnH. Brouhard
Andrcw N. Pclrch

12. Hypertension ............................................................... I 26. Renal Failure 33

Daniel W McKcnney
Shamn Bmorh
27. Acid-Base and Electrolyte Disturbances ....................447
section Three Vlmd Chrdha and Uri S.Alon
>astrointestinal Disorders 231 28. Acute and Chronic Scrotal Swelling .......................... 465
Jack S. Elder

3 . Failure to Thrive and Malnutrition ............................. 233 29. Sexually Transmitted Diseases ......................... -- ......-.475
Vlrginia Kcanc md Swan Feiplman Gals R. Bunlrin
riii
 1
X ~ V Conre,,r.v
I
30. Menstrual Problems and Vaginal Bleeding ................ 495 Section Eight
Mar~czvc(irccnficld Hematologic Disorders
. .
31. Ambiguous Gen~lalla.................................................. 517
Jack S. Elder 47. Lymphadenopathy ..................................................... 861
luhn R. S~hreibsrand Btirn W Bemnn
Section Five 48. Pallor and Anemia ......................................................873
Developmental~PsychiatricDisorders 533
Bnrn \I:B c m r n
-- .
..
.
49. Neck Masses i n Childhood ......................................... 895
32. Mental Retardation and Developmental David I. BIIL
Disability ..................................................................... 535
50. Bleeding and Thrombosis ........................................... 909
Cregari S. Liplak
J. Paul Scou
33. Dysmorphology ................................ ....................... 559
-.- I
R. Sccphcn S. Amala
Section Nine
34. The Irritable Infant .................................................... 577 Infectious Disorders
Emoy M. Pcmck -

35. Unusual Behaviors ...................................................... 585

5 1. Recurrent Infection ..................................................... 931
Willim I. Swill md Hugh F lohnrlon
Laurrsrc A. Boxer and R. Alerrndcr Blsckwmd
36. Child Abuse .......................................................... 6 1
52. Meningismus and Meningitis ..................................... 955
Roben M. Rerrr
Peter L. Havens

53. Bites ............................................................................ 973

Section Six
Manha S. Wright
Neurosensory Disorders 63 1
54. Fever of Unknown Origin ........................................... 987
Andrea C. S. McCoy and Stephen C. AmnoR
37. Headaches in Childhood ............................................ 633
55. Fever and Rash ........................................................... 997
Ajay Gupw md BWCC H. Cohcn
Rakn hl. Lembo
38. Hypotonia and Weakness............................................ 651
56. Rashes and Skin Lesions .......................................... 1017
Salecm I. Malik and Michacl I. Pllnlcr
Amy Jo Noppcr and Mrnju E. Georgc
39. Paroxysmal Disorders 73
57. Fever without Focus ................................................ 1059
Andrew Blc-1 and Elaine Wyllie
Knrdnc G. Williams and David M, JrRc
40. Delirium and Coma .................................................... 705
58. Fever and Neutro~enia............................................. I071
L m y A. Grccnbaum
Philip A. Pin*
41. Stroke in Childhood ................................................... 727
Michael I. Riven
Section Ten
42. Syncope and Dizziness ............................................... 743 Endocrine/Metabolic Disorders 1085
David A. Lewis

43. Eye Disorders ............................................................. 765

59. Disorders of Puberty ................................................ I087
Mark 4. Rvllurn
Milrhell E.GeKner

Section Seven 60. Shon Stature .............................................................1103

Orthopedic Disorders Lconaculller
61. Hypoglycemia......................................................... 1 2 1
.. Charles A. Sranlc"
44. Anhr~lls....................................................................... 801
62. Polyuria and Urinary Incontinence ...........................1133
1amcr I. Norlo"
Cynlhir G. Prn
45. Gait Disturbances ....................................................... 823
Gmwc H. Thompson
Index 1141
46. Back Pain in Children and Adolescents ..................... 845
~ o h nG. momca
 1 Sore Throat

Roben R. Tanz Stanford T. Shulman

..
Sore Ihrual is a common chief com~laint.Each war aooroximalel\~ The e#,~emviruser (coxsackievirus and echovirusl can cause sore
20 million patients in the United States visit physicians because of throat. especially in the summer. High fever is common, and the
lhroat complaints. The majority of these illnesses are nonbacterial throat is slightly red: tonsillar exudate and cervical adenopathy are
and ncitllsr necesritale nor i r e alleviated by antibiotic therapy unusual. Symptoms resolve within a few days. Enlemvimses can
(Tables 1-1 lo 1-3).Acutes1reptococcalpharyngitis, however, warmnts
accurate diagnosis and therapy lo prevent serious suppurative
..
and nonsu~vurati\~ecom~licalions.Funhennore. life-threatenine
infectious complications o f streptococcal and nonstreptococcal
- Table 1-1. Etiology of Sore Throat
orophqngeal infeclions may manifest with mouth pain, pharynpilis. Inleclion
par&har\-"zeal soace infectious extension, and ainrav obskction Bacterial (see Tables 1-2. 1-3)
lar iV (see Tables I-?. 1-3)
Fungal (see Table 1-31
Neutropenic mucositis (invasive anaerobic mouth flora)
VIRAL PHARYNGITIS Tonsillitis
Epiglottitis
Most episodes o f pharyngitis are caused by viruses (see Tables 1-2 U\ulilis
and 1-3). 11 i s dificylt to clinically diainguish between viral and Patonsillar abscess (quinsy sore throat)
bacterial pharyngitis with a very high degree of precision. but cenain Reuopharyngcal abscess (prevenebral space)
clues may help the physician. Accompanying symptoms o f conjunc- Ludwig angina (submandibular space)
rivitis. rhiniris, croup, or l a ~ n g i t i are
r common with viral infection Lateral pharyngeal space cellulitis-abscess
but rare in bacterial pharyngitis. Buccal space cellulitis
Many viral agenu can produce p h q n g i t i s (see Tables 1-2 Suppurative thyroiditis
and 1-31. Some cause distinct clinical syndromes that are readily Lcrnierre disease (septic jugular thrombophlebitis)
diagnosed without laborator? testing (see Tables 1-1. 1-4. and 1-6). Vincent angina (mixed anaerobic
I n .Dhmnzilis
. - caused b\. . .parainfluenza and influenza viruses. hcreria-gingivitis-pharyogitis)
rhinoviruses, coronaviruses. and respiralory syncylial virus (RSV). Imilation
the symptoms of coryza and cough often overshadow sore throat.
which is generally mild. Influenza virus may cause high fever. C i p e t t e smoking
cough, headache, malaise, myalgias. and cervical adenopathy in addi- Inhaled iniranrs
tion to pharyngilis. In young children, croup or bronchiolitis may Reflux esophagitis
develop. RSV is associated with bronchiolitis, pneumonia, and croup Chemical toxins (caustic agents)
Paraquat ingestion
in ,vounz children. RSV infection in older children is usuallv
tinguishable from a simple upper respiratory tract infection.
, indis-
Smoz-
Pharynpiris i s not a prominent findin. o f RSV infection i n either aoe
goup. i'arainfluenzi \,ituses arc ass&iated with croup and broncci-
olitis; minor sore throat and signs of pharyngitis are common at the
I Dry hot air
Ha foods. liquids
Other
outset but rapidly resolve. Infections caused by parainfluenza, Tumor, including Kaposi sarcoma, leukemia
influenza. and RSV are often seen in seasonal (winter1 .
. evidemics.
Ade,ro!.inrrescan cause upper and lower respirator?.tract disease.
Wegener granulamatosis
Sarcoidosis
ranging from ordinar?. colds to severe pneumonia. The incubation Glossopharyngeal neuralgia
ceriod of adenovirus infection i s 2 to 4 davs. , ..
U o w r res~irarorvtract Foreign body
infection typically produces fever, erythema o f the pharynx. and fol- Stylotyoid syndrome
licular hyperplasia of the tonsils, together with exudate. Enlagement Beh~etdisease
of the cervical lymph nodes occurs frequently. When conjunctivitis Kawasaki syndrome
occurs in association with adenoviral pharyngitis. the resulting Posterior pharyngeal trauma-pseudodiveniculum
syndrome i s called pho,?rzgocor~rr,8~1i1~nl fever Phar! ngitis may last Pneumomediastinum
as long as 7 days and does not respond lo antibiotics. There are many Hematoma
adeno\,ims serolypes. adenovirus infections may therefore develop Systemic lupus erytliematosus
i n children more than once. Laboratoly studies ma) reveal a leuko- Bullous pemphigoid
cytosis and an elevaled erylhrocyte sedimentation rate. Outbreaks Syndrome of periodic fever, aphthous stomatitis.
have been associated with swimming pools and contamination in pharyngitis. cervical adenitis (PFAPA)
health care workers.
 lasis less than 7 days, but rcverc pain may impair fluid intake
Table 1-2. lnleetlous Etiology of Pharyngitis and necessitate medicdl support.
Coxsackievirus A16 causes hond-foor-tnourh direurs. Vcsicles
Definite Causes can occur throughout the oropharynx: hey arc painful. and
Srmpmcoccrtr pyogenes (Group A streptococci) they ulcerate. Vesicles also develop on the palms, soles. and,
Covt,ebocreriunt diphrl~erioe less often, on thc trunk orcxtremilies. Fever is present in most
Arco,~obacreriumhoentolyrica!n cases. but many childrcn do not appcar seriously ill. This
Neisrerio go,torrhome disease lasts less than 7 days.
Epstein-Barr virus
Paminfluenzn viruses (types 1-4) Primary infection caused by hcrpes simplex virus (HSV) usually
Ipfluen7.a viruses produces high fever with acute pirr~it~osronzoriris, involving vesicles
Rhinoviruses (which become ulcers) throughout the anterior portion or the mouth.
Coronavirus including the lips. There is sparing of the posterior pharynx in hcrpes
Adenovirus (types 3.4. 7. 14. 21. others) gingivoston~atitis: the infection usually occurs in young children.
Respiratory syncytial virus Hi~h fever is comnian. oain i s imense. and intake of oral fluids is
Herpes sinlplex virus (types 1. 2) of& impaired, which may lead to dehydradon. In addition. HSV
may manifest in adolescents with pharyngitis. Approximately 35%
Probable Causes of new-onset HSV-positive adolescent uatients have hemetic lesions:
Group C areptococci most patients with^^^ pharyngilis cannot be distinguished from
Group G sueptococci patients with other causes of pharyngitis. The classic syndrome of
Chlnt,~jdioprteurnoniae henpetic gingivostomatitis in infants and toddlers lasts up to 2 weeks:
Cltlan~jdiorrachornoris data on the course of more benign HSV pharyngilis are lacking. The
Mycoplarmo pneurnoniae differential diagnosis of vesicular-ulcerating oral lesions i s noted in
Table 1-6. A common cause of a local and large lesion of unknown
-, is aohthous stomatitis (Fie.
etiolocv . - 1-1). . Some children have a
cause meningitis, rash, and two specific syndromes that involve the combination of periodic fever (recuncnt at preolctable fixed ttnleq,.
oropharynx: ~phtho~ stom3til1~.
s pharyng~t~s. and cervical adenttis (PFAPA). this
syndrome is idiopathic and may respond to oral prednisone or
Herpnagino is characterized by distinctive discrete. painful. gray- cimctidine. PFAPA usually begins before the age of 5 years and is
white papulovesicuiar lesions distributed over the posterior characterized by high fever lasting 4 to 6 days. occurring every 2 10
oropharynx (Table 1.6). The vesiclcs are I to 2 mm i n diame- 8 weeks, and resolving spontaneously.
ter and are initially surrounded by a halo of erythema before Infants and toddlers with measles often have prominent oral
they ulcerate. Fever may reach 39.SC. The illness generally findings early in the course of the disease. I n addition to high fever.
cough, coryza. and conjunctivitis. the pharynx may be intensely and
diffusely erythematous. without tonsillar enlarge men^ or exudate.
. . -
I
The oresence of Koolik roorr., . the oathaenomonic white or blue-
Table 1-3. Additional Potential Palhogens Associated
white enanthem of measles, on the buccal mucasa near the mandibu-
mlh Sore Throat
lar molars provides evidence of the correct diagnosis before the rash
Bacteria develops
Fuzobocreriurn necrophorurn (Lemierre disease)
Neisserio meningiridis
Yersinio enremcolirico INFECTIOUS MONONUCLEOSIS
Tularemia (orpharyngeal)
1 Yersinio perrir PATHOGENESIS
B ~ c i l l u onrlzrocis
r
1 Clzla,n?din psirroci Acute exudative pharyngitis commonly occurs with infectious
Secondary syphilis mononucleosis caused by primary infection with Epstein-Ban virus
Mjcobocrerism roberculoris (EBV) (Table 1-7). Mononucleosis is a febrile, systemic. self-limited
Lyme disease lymphopraliferative disorder that is usually associated with
Cotyteboderitr~nulcerans hepatosplenomegaly and generalizedlymphadenopathy. The pharyn-
Lcprospira species gitis may be mild or severe. with significant tonsillar hypenrophy
Mjcoplosna hotninis (possibly producing ainvay obstruction), erythema, and impressive
tonsillar exudates. Regional lymph nodes may be particularly
Virus
enlarged and slightly tender.
Coxsackievirus A, B Infectious mononucleosis usually occurs in adolescents and
Cylomegalo\~irus young adults; EBV infection is generally milder or subclinical in
Viral hemorrhagic fevers preadolescent children. I n United States high school and college
Human immunodeficiency virus students, attack rater are 200 to 800 per IO0,WO population per year.
(HIV) (primary infection) EBV i s transmitted primarily by saliva.
Human herpcrvirur 6
Measles :
Varicella CLINICAL FEATURES
Rubqlla
After a 2- to 4.week incubation period. patients with infectious
Fungus mononucleosis usually experiencean abrupt onset of malaise. fatigue.
Cottdida species fever, and headache, ~01lo;ved closely by pharyn,ottis.The 1onsii;are
Histoplasmosis enlarged with exudates and cervical adenopathy. More generalized
Cryptococcosis adenopathy with hepatosplenomegaly often follows. Fever and
pharyngitis typically last I to 3 weelt, while lymphadenopathy and
 ~ing
Table 1-4. U i s t i ~ ~ ~ a i s lFedurns a f I'nr;1pharyngcal-Upper Resplrnlory lkacl lnleclions

Submandibular Portanginal
1
Relmphur)mgei~l Spacc 1.alrml Lpryngolmchn- Seprlr*
I'critonsillnr Ahsccs (I.udwlg Pharyngcnl Maslir~~lnr hmnchitis RnrlcrinI (Lemierre
A htcc~s ICclluliIls) Ancinnl* bare* SPRCC* Eni~l~llllis (Cmuol Trarhcillr D'iwnse)

Etiology Gnrup A . S ~ ~ ~ J ~ / ~ , V / 0r:d

~ ~ :tni\ernhcs'
~ ~ ~ ~ C ~ ~ Orid
, ~ ;an;~cmhcr' Om1 ;~n;temhcs' f i . P;m!influcnr;~ M,>m.wlln Ftrrobarreri~~rn
b l r c l l l u ~ ~(1nll
~ ~ ~ i . ,,II~.IIL ori!l ~,~~,,cII:,,c virus; influenn~ caarrl~alir, nccmpb~mnx
;tt~;tcrohes' i~!~~lcrnhes! grollp A lypc h wlcnn-vinms .7. ~,!,rc,,.s,
st~c~~l~~c~~cci, i ~ s ~spir.!nlry
d 11, i#fjltw>txc
"st~pl~~~c~live sylnuylii!l vims l y p hor
i~tl~~~itis'' 1c.i.i contrnun nonlypable
Am Tcms Ininncy. prclcens, Tcens Tcenn Teces 2-5 yr 3 n r n r 3 yr 3-10 yr Tccns
lCC,,S
,~cc~,~i,>,,:,lly
Manifestoliens U ~ C
l~liliitlC ~ ~ S 01 Pcvcr, dy.ipneu. Rver. dysphagi!t. Scverc p:!in. I'ain. pmlninenl Suddcn-onret Low-grdde levcr. Prior history of Prior
l>haryngilis, saidor, dysphagia, odynophsgia. lever, trismur. lrismus. fcver high fcvcr, harkingcough. cmup wilh pharyngirir
lollowed by drooling, stiff stiff neck. dysphagin. Swelling not "IOX~C " hosrscncrr- sndden onxt with suddm-
sudden worsening neck. pain. dyrpnea: airway cdcmolous always evidcnl appearance. aphonia. of rerpimlary onset fever.
111unilateral cervical abslruclion. appearing. mumed voice, stridor: mild dislrcs, high chills.
r~dynophagia. adenaputhy. swollen langue painful lateral snriely. pain, rrtnclions: fever. "laxic" odynophagia.
~rirtnus,hol swelling of :and floor of fncial Gawl or rctmclions. ndiognph appeanncc. neck pain.
IIC"UK> (~txIllle~l) portericlr ~noulh(tcndcr) nccl rwrlling dysph;tgia. SIIOVS ho:!~ncss. scptic
voice. drooling. pharyngcrl rpuce Mumcd voics (induralion) dmling. "nccplc sign" nridor. thmmbo-
rlisplneemenl Descending Mny lead la ruidor, silling of subglonic barking phlebitis
of uvula mediartinilir (me) Lemicne up. leaning narmwing on cough. tripod of inlernal
Lateral neck disease forward tripod anemposterior rilling jugular vein
radiograph posilian. neck view position: with septic
reveal^ swollen cherry-red radiograph emboli
retropharyngeal SWOIICO ar per emup (e.8.. lungs.
prevcncbral space: cpiglottir plus ngged joinu).
infanlr. > I x Usually no1 tracheal bacaremia
width of adjacent home 01 air column
vcnebral body coughing
(>2-7 mm): leens. Lawn1risk
> '13 x width of radiograph
vertebral body shows "thumb
(>I-7 mml sign" of
CT dislinguishcs ~woll~n
Cell~litiS epiglottis
from abscess
Trtalrnenl Penicillin for Airway Ainvsy Penicillin. Penicillin. Aiway Airway Ainvay Clindamycin.
abscess and management. managcmenl clindamycin. eiindamycin, msnagemcnt managcmenl managcmcnt pnicillin.or
cellulitis nafciliin. Penicillin. ampicillin- ompicillin (intabation). (rare) (frequent cefaxitin
Aspiration for ceftrirxone. cliodamycin. suibrclam rulbaclam cenriaronc Cool misl, inrubation)
;~bsccss(needle ampicillin- ampicillin- Surgieol drainage nccmic Cefvirronc
or i ;and D) sulbac~am sulbnetam uruolly rcquircd cpincphrinc. with or
Necdlc is preferred Surgical drainage Rarely surgical dcrametharanc wilhoul
if an abscess drainage narcillin

TPepro~lmp,ococct,.~,
Fwolmrr~riutn.Bocremider (urnally rn~lonimgcniclu).
.Ohcn odoniogenic: chcck lor loolh ubrbe~r,curicr, lrndrr IeeL.
CT.computed lomoqruphy.
 6 Secriort One 0 Rerpirmor). Di.,order.~
Tabla 1-5. "Red Flags"Associa1ed wilh Sore Thmat
Fever > 2 weeks
Duration o f sore throat > 2 weeks
Trismus
Drooling
Cyanosis
Hemorrhage
Asymmetric lonsillar swelling or asymmetric cervical
adenopathy
Res~irarorvdistress (airwav obstmclion or ~nebmonia)
I Suspicion o f paraphnryngcal space infcclion
Susp~cton
" ' ofd~phthena(bull neck. uvula paralysis. hick
membrane) .
Apnea
Severe. unremitting pain
"Hol potato" voice
Chest or neck pain
Weight loss
hepatosplenomegaly subside over 3 to 6 weeks. Malaise and lethargy
can persist for several months, possibly leading to impaired school
or work performance.
DIAGNOSIS
L a b o r a t o studies
~ .. . .
of diaenostic value include atv~icallvrn~hocvtosis: .
these lymbhocytes are $marily EBV-specific, c y t o t o x i c ~ lympho-
cytes that represent a reactive response to EBV-infected B lympho-
eyles. A modest elevalion o f serum transaminase lcvels, reflecting
E B V hepatitis, is common. Tests useful for diagnosis include detec-
tion of heteraphile antibodies that react with bovine erythrocytes
(most often detected by B e monospot test) and specitic antibody
-
aeainst E B V viral cawid antieen .(VCA). . -
.. earlv antieen .(EA).
.. and
n k l e a r antigen (EBN~.). Acute infectious mononucleosis i s usually
associated w i h a positive heterophile test result and antibody to
V C A and E A (Fig. 1-2).
The findings o f acute exudative pharyngitis together with
hepatomegaly, splenomegaly, and generalized lymphadenopathy
suggest infectious mononucleosis. Early in the disease and i n cases
-
without liver or spleen enlareement, differentiation from other
causes of pharyngitis, including sveptococcalpharyngitis. i s difficult.
Indeed, a small number o f patients with infectious mononucleosis
have a throat culture positive for group A streptococci. Serologic
evidence o f mononucleosis should be sought when splenomegaly or
orher features are present or i f symptoms persist beyond 7 days.
TREATMENT
Palxcn~rw ~ l hrnfect~ousmononuclcostsrequuc supportive lreatmcnt
Con~co,rerotd%m3y k tndl~atcofor aculc llfc threalcn~ngcondttlons.
such as airway obstruction caused by enlarged tonsils.
G R O U P A STREPTOCOCCAL INFECTION
In the c\,aluarion of a patient with sore throat, the primary concern is
usually accurats diaenoiii and treatment of ~Larvngitiscausedbv
group^ sneplococci, which accounts for aboit 15%bf all episode;
of pharyngitis. The sequelae of group A streptoc~~cal phuryngitis,
especially acute rheumatic fever and acute glomemlonephrilis. atone
time resulted i n considerable morbidiw and moiialilv in lhc Uniled
Srates and continue lo do so in other pans of the world. Prevenlionof
acute rheumatic fever i n panicular depends an timcly diagnosis of
streptococcal pharyngitis a~ldprompt antibiotic treatment.
.-
Group A streptococci are characlerized by lhe presence of group
Aearbohydrate in the cell wail, and they are further distinguished by
w e r a l kinds of cell wall protein antigens (M. R:T). These protein
antigens are useful for studies of epidemiology and pathogenesis.
EPIDEMIOLOGY
Gmup A streptococcal pharyngitis has been endemic i n the United
States: epidemics occur sporadically. Episodes peak in the late
winter and early spring; rates of group A streptococcal pharyngitis
are hiehest amona children aged 5 lo II vears old.
ad
o f gro;p A strepto~occiin cla~sroomsand among family
members, especially in crowded living conditions, i s common.
Transmission occurs primarily by inhalation o f organisms in large
dmolels or bv direcl contact withresoiratorv secrelionr. Pets do n i t
~ ~~
appear to be a frequent reservoir. Untreated sireptococcal pharyngi-
tis is particularly contagious early i n the acule illness and for the firs
-
2 weiks after ihe oreanism has been acouired. Antibiotic theraov
eRect~velyprevenls dlseaTe lransm~sstonW~thtn24 hoJr, of Instltu.
-,
urn of therapy wtlh pnoclll!n. 11 i s difficult lo isolate groupA strep
tococci from with acute streptococcal phbngitis, and
infected children can return to school.
Molecular epidemiology studies o f streptococcal pharyngitis
have shown that numerous distinct strains of - erour,
. A sueprococci
circulate simultaneously i n the community during the peak season.
"DNA-fingerprinting" techniques funher demonstrate that children
w i h streptococcal pharyngitis serve as a community reservoir for
swains that cause invasive discase (e.g, sepsis, sweptococcal toxic
shak syndrome, cellulitis, necrotizing fasciilis) in the same peognphic
area and season.
CLINICAL FEATURES
Tkclassic patient with acute streptococcal pharyngitis has a sudden
onset of fever and sore throat. Headache, malaise, abdominal pain.
n a w a . and vomiling occur frequently. Cough, rhinorrhea, conjunc-
tivitis, stridor, diarrhea, and hoarseness are distinctly unusual and
sugest a v i a l etiology.
Examination of the patient reveals marked pharyngeal erythema.
Petechiae may be noted on the palate, but they can also occur in viral
pharyngitis (see Table 1-7). Tonsils are enlarged, symmeuic, and red,
with patchy exudates on their surfaces. The papillae o f the tongue
may be red and swollen: hence the designation "strawberry tongue."
Anterior cervical lymph nodes are ohen tender and enlarged.
Combinations of these signs can be used to assist in diagnosis; i n
panicular, tonsillar exudates in association wilh fever, palatal
petechiae, and tender anterior cervical adenitis suongly suggest
infection with group A streptococci. However, other diseaes can
produce this constellation of findings. Some or all of these classic
characteristics may be absent i n patients with streptococcal pharyn-
gitis. Younger children o f t m have coryza with crusting below the
nares, more generalized adenopathy, and a more chronic course, a
syndrome called streprococcosis.
.
When rash accomoanies. -
the illness. accurate clinical diaenosis is
easier. Scorler fever: so-called because o f the characteristic fine,
diffuse red rash, is essentially pathognomonic for infection with
group A sweptococci. Scarlet fever i s rarely seen in children .younoer-
Fhanj ).ears'oid or in adults.
--
SCARLET FEVER
The nsh of scarlet fever i s caused by infection with a swain of group A
streptococci that contains a bacteriophage encoding for producdon
of an erythrogenic (redness-producing) toxin, usually erythrogenic
(or pyrogenic) exotoxin A. Scarlet fever i s simply group A strepto-
coccal pharyngitis with a rash and should be sxplained as such to
patients and their families. Aithough patients with the srreprococcol
1 Table 1-6. Vcsicalar-Ulcen~lincB r u ~ t i o n osf the M o u t h nnd Phnrvnx I
Recurrent
Scsritying
Syslcmi~ lnnnmmslory Ulcemtivr
I.apur Ibwel Slomalilis
Ihnd-hot- Lrylhemslosur Dlsenrr Aphlhovs Bchset Vincent (Sultan
(;ingivostnmntilis Herpanginn Moulh Dlscss~ Chlebnpnx (Slag) (IUD) Stomstlllr Disearc Slamslitis D&)

el in lo^ Hcrpcs ailnpler Coxrockicvirus Curssekicvirus V;bricelln- Unkm,wn: Unknown: Unknown Unknown: Unknown; Unknown
virus (HSV) I A. B: cchovirus A, caxrlckic- roslcr virus iauloimmunc ilulnimmun~ vaculilis or
or HSV virus B anaerobic
(wmly) (mrely) bacteria
Location Ulccritlivc vcriclcs Anariur I';buccs T<mgac,huce;~l Tuttgae. Or:tl, n;nsol Lips. luegae. A in IBD On1 (similar Gingiva: Tongue:
ol' ph:lrynx. (lo~~rils),safl 111umsi8. gingiva. IIIUCUSB: huuc~l lo IBD): ulceration buccal
tongue. and pnlalc (uvula), prlulc, pill~as, buccsl prlale. mucost, genital at b m of mucora
palalc less oflen soles. mucosn. philryns. onlpharynr ulccm lelh
plus lesions of phitrynr rnlerior lnarkd hucc;tl
~ U C O C U ~ ~ ~ ~ O U S oral cavity LU~~~COUS ~UCOSB
(perioral) lesions;
m~gin mnk > face
Age . LesPthan 5 yr 3-10 yr 1 yr-teens Any age Any age Any age Tcenn and Teens,adull- T-; if Tens
adullhood h d . younger.
acasionally Iconrider
<I0 yr immune-
deficiency
and blmd
dyrcmia
Manit&ations Ftver,mouth pain, Fever, sore Painful bilateral Fever, prurili~ Renal, cemrsl Multiple Similar to Painful Fever. &P.
toxic, fetid throat. ~e6i~le.l~ EU1B"eOUS nervous mcurrcnees: IBD ulccralionr, bleeding large.
breah, drooling. alynaphagia, fever vclicles. ryrlem. painful (heal gums; painful
anorexia. cervical summer out- painful oral &ti$. ulecralion~ without Bay ulcers-
lymphadenopathy: bre*; 6-12 lesions CU~~ICOYS. 1-2 mm, but warring): membrane tion,:
cracked. swollen lesions (2 to hematologic, may be uvritis. rrlqsing;
hemorrhagic 4 mm papule) olhcr organ 5-15 mm anhralgia. searring
gums; secondary +vesicle + involvement; anhritis. with
ipoculation ul~eration: ulcers lower dinortion
possible (tinges,, headache. minimally to of mucara
eye, skin); myalgiar moderately inlertinal
reactivation with painful: may ulceration
long lslency be painless (similar to
(any age) BD):
Rcumncel;
spontaneous
remissions
Treatment Avoid dehydration; Avoid Avoid Avoid Specific therapy Specific Topical Topical Oral Topical
acyclovir if dehydration; dehydration: dehydmlion. for SLE themPY conico- canim- hyginc: mnico.
immunc- rarely. secondary for LBD ~l~roidr; rtcroidr; leua- ~temids.
compromised secondary infeclion: mu11 oral cyelinc and-
rscptie acyclovir i f exclude (viscous) wash gaicr:
meningitis or immuna- SLE. IBD, lidanine must mlc
myacarditis compromircd human out
immuna- dig-
deficiency nancy by
V~NS (HIV). biopsy
Beh$ct
disease
 Secrion One
Figure 1-1. Aphthour stomotitis ('conker sore'). (ham Reilly BM: Sore
throot. In Proclical Strategies in Outpatient Medicine. 2nd ed.
Philadelphia. WB Sounders. 1991 .I
toxic shock ryndrome are infected with group A streptococci that
produce erythrogenic toxin A, most infections with p u p A strepto-
cocci are not associated with unusual severity (Table 1-8).
Streptococcal toxic shock syndrome is usually associated with a
orimarv
' cutaneous rather than a oharvngeal focus o f infection.
Thgrash o f scarlet fever has a.tex&r'like sandpaper and blanches
with pressure. I t usually begins on the face, but afler 24 hours i t
becomes generalized. The face, especially the cheeks. is red, and the
~ ~
.. .
area around the mouth often aowars oale i n cornoarison icimumoral
pallor). Accentunl~ono f erythema occurs i n flexor slun creases, espe-
ciall) In the antccubil31 fossac (Pastid's lines). The erythema begins
to fdde wilhin a few days. Desquamation begins within a week o f
- grounds have not proved very useful. Using clinical criteria alone.
.LIL, rn0"lhS
TIME FOLLOWING ONSET OF ILLNESS
Figure 1-2. Typicol humon serologic rerponre to Epsein.Borr viwr infec-
Ibn. At time ofclinical prerenlolion luruolly 2 ID7 week aher exposure],
ontiviral copsid antigen IVCAI rerponre moy consist of IgM and IgG
antibdies; onli-early onligen lEAj response is ohen present: ond
anli-nucleor onligen IEBNAI rr uruolly negative. The IgM onti-VCA
rerponre vruolly subsides within 2 to 4 months, ond the anti-EA rerponre
uruoll, disoppeois i;.i:l,i,? 2 to 6 monlhr. [Dolo lrom Andiman WA.
McCcrlhy P. Marlo.::i-r ill, e: 01: Clinicol, viraiogic, ond serologic evi-
dence oi Ep~tetn-Bar,wrur inisction in association with childhwd pneu-
monio. J Pedioti 198 1 :99880886: Fleisher G. Heole W. Henle G. el 01:
Primary inlection with Ebnein-Boir virus in infan~sin the United Staler:
Clinicol and serological obrervalionr. J Infect Dir 1979:139:553-558;
Brown NA: The Eprlein-Borr virus l~nlecliourmononucieorir. B-lymphoprm
lilero~~vedisorderrl. lo Feigin RD. Cherry JD [edr]: Texlbook of Pediotric
lnlec~~o~rDireorer. 2nd ed. Philodelphia. WB Sounders. 1987.1
RI
T d e 1-7. Manifestations of Infeclious Mononucleosis
(Eslcin-Barr Virus)
Camon
F m r (1-2 weeks)
Lymphadenopathy (bilateral, minimally tender, primarily
arvical nodes with axillary, inguinal, epitrochlear.
supraclavicular nodes)
Tonrillopharyngitis (exudative)
Splcnomegaly *
Hepatomegaly
Elevated liver enzymes (transaminases)
Malaise
Fatigue
Lcs Common
Rash (spontaneous or associated with ampicillin or
albpurinol)
Ompharyngeal petechiae
Jaundice
Eyelid edema
AMominal pain
Thmmbocytopenia-purpura
Hemolytic or aplastic anemia-pallor
Severe upper aimay obstruction
Mcoingoencephalitis
Guillain-Barrt syndrome
Bell palsy (seventh cranial nerve)
Hemophagocytic syndrome
X - l i e d lymphoproliferative disorder (Duncan syndrome)
Lymphoproliferative disorder i n immunocompromised hosts
Splenic rupture
Glomerulonephritis
Orchitis
onset on the face and progresses downward, often resembling that
seen after a mild sunburn. On occasion, sheetlike desquamation
occurs around the free margins o f the fingernails and is usually more
coarse lhan the desquamation seen with Kawasaki disease. The
differential diagnosis o f scarlet fever includes Kawasaki disease.
measles and staphylococcal toxic shock syndrome (Table 1-9).
DIAGNOSIS
Although signs and symptoms may suongly suggest acute strepto-
coccal pharyngitis. laboratory diagnosis is highly recommended,
. - 1-31.
even for oatients with scarlet fever (Fie. . Scorine -svstems
. for
diagnosing acute group A strcplococcal pharyngitis on clinical
physicians overestimate the likelihood that patients have streptococ-
cal infection. The throat culture has traditionally been used to
diagnose streptococcal pharyngitis. Plating a swab o f the posrerior
pharynx and tonsils an sheep blood agar. identifying P-hemolytic
colonies,and testing them for the presence o f sensitivity to a baciuacin-
impregnated disk i s the "gold standard" diagnostic test, bur i t lakes
24 to 48 hours to obtain results. There are a number of rapid
diagnoaic tests that take less than 15 minutes. These "rapid strep"
tests detect the presence of the cell wall group A carbohydrate anti-
gen after acid extraction of organisms obtained by throat swab.
Rapid suep tests are highly rpec;Jc (generally >95R). with [he
throat culture used as the standard. Unfortunately. the ser,riliviry o f
most of these rapid tests can be considerably lower. I n comparison to
hos~ital or reference laboratorv throat culture results. the sensitivities
of these tact are generally 80% to 85% and can be lower However,
when bolh throat cultures and rapid tests performed in physicians'
oftices arecompared with cultures performed in reference kbbatorier.
 Sore lhmar 9
Tssting patients for semlogic evidence of an antibody response to
Table 1-8. Characlerislier o f Severe lnvprlve andlor exuaDcllular products of Erouv A stre~tocaviis not usefcl for
Toxigenic G m u p A Slreptoroecsl Infection diapcsing acula pharyngcir. ~ecausc.it generally takes several
Posilive Cullure Sites weeks for antlbody levels l o rise, streplococcal antibody tens are
validonly for deermining past infection. Spcc~ficantibodies include
Blood anlislrc~iolvsin0 (ASO), anti-DNase B. and antihvalumnidase
Soft tissue abscess (~~T').'when antibddy te;;ing is desired i order to e/aluate a pos-
Synovial fluid sible post-sueptococcal illness, more than one o f these tests should
I Ez:(neal fluid
Surgical wound
- be performed to improve sensitivity

Cellulitis aspirate TREATMENT

Clinical Manifeslations Labornlory Manifeslalions Treatment begun within 9 days of the onset of gmup A streptococcal
Fever Leukocytosis pharyngitis is effective i n preventing acute rheumatic fever. Therapy
Toxic Shock* Lymphopenia docs not appear to affect the risk of the other nonsuppuralive
Confusion Thrombocytopenia sequela. acute ~ost-streptococcal ~lomerulonephritis.Antibiotic
Headache Hypnauemia the.& also redukes the ticidence of ;uppurativc Guelae of group A
Abdominal pain Hypoalbuminemia sveplococcal pharyngitis, such as peritonsillar abscess and cervic31
Vomiting Hyperbili~binemia(direct) adenitis. I n addition, ueatment produces a more rapid resolution of
Local extremity pain and Elevated AST. ALT, BUN signs and symptoms and terminates ~onla~iousnesswithin 24 hours.
swelling Renal sediment abnormalities For h s e reasons, antibiotics should be instituted as s w n as the
Hypsthesia Coagulopathy diagnosis is suppotled by laboratary studies.
Cellulitis Hypoxia There ue numerous antibiotics available for o r t i n e sueotococcal
Scarlatiniform rash (40%) pharyogitir (Table I . l o ) The drug of choice i
s penicilin. &spite the
Eythroderma (25%) wrdespiwd use of penicillin to lreat smptococcaland other infections.
Conjunctival injection penicillin resistance among group A tococ cocci has not developed.
Red pharynx Penicillin can be given by mouth for 10 days or inmuscularly as a
Pneumonia with or single injection of bermthine penicillin. Inmuscular be-thine
without empyema penicillin alleviates concern with patient compliance. A less painful
Osteomyelitis
Vaginitis
alternative
~- ~ ~
.
is bermthine oenicillin incombinalion with orocaine ocni-
~~

cillin. lnlramuscular procaine penicillin alone is inadequate for pre-

Plocdtis
Derquamation
Necrotizing fasciitis
Diarrhea
.Cau definition ofwrcpcaacal toxic shock radromcrcquirrr (l)irolation ofgrmpA
rmprococci horn (a) a normally rccrilc rice (blood. synovid or p - t o n e d fluid) or (b) a
nonrlilc lire ( h l . weund). (It) Severity ir defined by (a) hpxension a d (b) Nm OI
mom of mnal impairmcnL coaguloparhy. lit," involvcmcm. adult rcrpiralory dkuur
ryndmme. a gcnediud oylhcmalour mular rash (uirh or uiihaut lrlcr d q m a -
tion). and ran tisue ~ m r i(nccmtizing
r raiitir. myoritis, ganpnc).The definitive
diaporir rcquirvcrilcriaLA and IIA plus 8. Crilcria IB and I I A ptur B areconridd
probabtcif no orhcr idcntifiablc cavw ir pmres.
ALT. nl-s nminomrfr-; ASS. arpanacs uninomrfcrur: BUN, blood wra
nitrogen.
the sensitivities, specificities, and overall accuracy o f the office
culwre and the office rapid tesr are quite similar: the latter often
performs better than the culture.
The low sensitivity o f these tests, coupled with their excellent
specificity, has led to the recomrn~odationthat two swabs be
obtained from patients with suspected sueptococcal pharyngitis.
One swab i s used for a rapid test. When the radd antigen detection
test result ii pos~ttve,i t ic'hlghly Ijkcly that th; pa~len;has group A
streptococcal infcct!on. and the extra swab can be discarded When
the rapid test result is negative, group A streptococci may nonethe-
less be present; thus. the extra swab should be processed for culture.
Physician oftices that have demonstrated that their rapid test and
throat culture results u e cornpxablz may be able 13rely on thz rapid
tesr resull even when i t i s negative, without performing a backup
culture.
In general, patients with a negative result of the rapid test do not
.require treatment before culture verification unless there is a panic-
ularly high suspicion group A streptococcal infection (e.g.. scarlet
fever, pritonsillar abscess. or tonsillar exudates i n addition to tender
cervical adenopathy. palatal petechiae, fever, and recent exposure to
a person with group A streptococcal pharyngitis).
vention of acute rheumatic fever because adequate levels of penicillin
are not nrcsent in blood and tissues for a.rufficienl time. Other
placlams, including semisynthetic derivatives of penicillin and the
cepbalosprins, are at least as effective as penicillin for treating group
A sacptococcalpharyngitis. Their broader specrmm, their higher cost.
and the lack of formal data concerning prevention of acute rheumatic
fcver relegate them lo second-line status. The decreased frequency of
dose administration of some of these agents may impmve patienl
compliance and makes their use attractive in selected circumstances.
Patients who are allergic to penicillin should receive erythro-
mycin or another "on-p-lactam antibiotic, such as clatithromycin.
azithmmycin. or clindamycin. Resistance of group A streptococci to
erythmmycin has increased dramatically i n areas such as Japan.
France. Spain. Taiwan, and Finland, where erythromycin has been
widely used. This has not yet emerged as a major problem i n the
United Slates, where the rate of macmlide resistance is about 5%.
Sulfa dmgs (including sulfamethoxazole combined with uimetho-
prim), tetracyclines, and chloramphenicol should not be used for
treatmen1 of acute streptococcal pharyngitis because they do not
eradicate group A streptococci.
COMPLICATIONS
~u~~uraC
t iovmep l i c a t i o n s
Antibiotic therapy has greatly reduced the likelihood of developing
.. .. - .
suoourative comolicatians caused bv soread of m o u A ~ streotococci
from the phaqnx or middle ear to adjacent structures. Pdrifo!trillor
, . C L ~(.JI .., q ~.i ~ ~twan~fes~~
y") w ~ t hfcvcr. setere mroal pain, dyspna-
.
peritonsillar area with asymmetry of the tonsils and sometimes
-
-eia.. "hot wtato voice:'. oain referred to the ear. and bulainz- of the
displacement of the uvula (Fig. 1-4; see Table 14). On occasion.
there is ~eritonsillarcellulitis without a well-defined abscess CaVltY.
Tnsmus may be pressnl When an abscess i s found clin~cdllyor by
an lmaglng study such as a computed tomograph~cscan. surgical
drsinaec IS indtcated. Pentonr!llar abscess occurs most commonl) in
older ciildren and adolescents.