Full Download Practical Strategies in Pediatric Diagnosis and Therapy 2nd Edition Robert Kliegman PDF
Full Download Practical Strategies in Pediatric Diagnosis and Therapy 2nd Edition Robert Kliegman PDF
https://ebookgate.com
https://ebookgate.com/product/practical-
strategies-in-pediatric-diagnosis-and-therapy-2nd-
edition-robert-kliegman/
https://ebookgate.com/product/pediatric-epilepsy-diagnosis-and-
therapy-3rd-edition-john-m/
ebookgate.com
https://ebookgate.com/product/pediatric-epilepsy-diagnosis-and-
therapy-3rd-ed-edition-john-m-pellock/
ebookgate.com
https://ebookgate.com/product/pediatric-anaerobic-infections-
diagnosis-and-management-infectious-disease-and-therapy-3rd-edition-
itzhak-brook/
ebookgate.com
https://ebookgate.com/product/lipid-nanocarriers-in-cancer-diagnosis-
and-therapy-1st-edition-souto/
ebookgate.com
Handbook of Pediatric Physical and Clinical Diagnosis
Eighth Edition Barness
https://ebookgate.com/product/handbook-of-pediatric-physical-and-
clinical-diagnosis-eighth-edition-barness/
ebookgate.com
https://ebookgate.com/product/advances-in-nuclear-oncology-diagnosis-
and-therapy-1st-edition-emilio-bombardieri/
ebookgate.com
https://ebookgate.com/product/advances-in-nuclear-oncology-diagnosis-
and-therapy-1st-edition-emilio-bombardieri-2/
ebookgate.com
https://ebookgate.com/product/pediatric-trauma-pathophysiology-
diagnosis-and-treatment-1st-edition-denis-d-bensard/
ebookgate.com
https://ebookgate.com/product/gene-therapy-protocols-2nd-ed-edition-
jeffrey-robert-morgan/
ebookgate.com
The Curtis Center
170 S Independence Mall W 300E
Philadelphia, Pennsylvania 19106
No part of this publication may be reproduced or transmitted in any form or by any means, electronic or
mechanical, including photocopying, recording, or any information storage and retrieval system, without
permission in writing from the publisher. Permissions may be sought directly from Elsevier’s Health Sciences
Rights Department in Philadelphia, PA, USA: phone: (+1) 215 238 7869; fax: (+1) 215 238 2239;
e-mail: [email protected]. You may also complete your request online via the Elsevier
homepage (http://www.elsevier.com) by selecting “Customer Support” and then “Obtaining Permissions.”
NOTICE
Medicine is an ever-changing field. Standard safety precautions must be followed, but as new research and
clinical experience broaden our knowledge, changes in treatment and drug therapy may become necessary or
appropriate. Readers are advised to check the most current product information provided by the manufacturer
of each drug to be administered to verify the recommended dose, the method and duration of administration,
and contraindications. It is the responsibility of the licensed prescriber, relying on experience and knowledge
of the patient, to determine dosages and the best treatment for each individual patient. Neither the publisher
nor the authors assumes any liability for any injury and/or damage to persons or property arising from this
publication.
Marjorie Greenfield, MD
Jack S. Elder, MD Associate Professor of Reproductive Biology, Case Western Reserve
Professor of Pediatrics and Carter Kissell Professor of Urology, Case University School of Medicine; Associate Professor of Obstetrics
Western Reserve University School of Medicine; Director, Pediatric and Gynecology and Pediatrics, University Hospitals of Cleveland,
Urology, Rainbow Babies and Children’s Hospital, Cleveland, Ohio Cleveland, Ohio
Acute and Chronic Scrotal Swelling; Ambiguous Genitalia Menstrual Problems and Vaginal Bleeding
Mitchell E. Geffner, MD
Professor, University of Southern California Keck School of Hugh F. Johnston, MD
Medicine; Physician and Director of Fellowship Training, Division Professor, Departments of Psychiatry and Educational Psychology,
of Endocrinology, Diabetes, and Metabolism, Childrens Hospital University of Wisconsin Medical School; University of Wisconsin
Los Angeles, Los Angeles, California Hospital and Clinics, Madison, Wisconsin
Disorders of Puberty Unusual Behaviors
Subra Kugathasan, MD
Associate Professor of Pediatrics, Medical College of Wisconsin; Susan R. Orenstein, MD
Children’s Hospital of Wisconsin, Milwaukee, Wisconsin Professor of Pediatrics, Division of Pediatric Gastroenterology,
Diarrhea University of Pittsburgh School of Medicine; Children’s Hospital of
Pittsburgh, Pittsburgh, Pennsylvania
Vomiting and Regurgitation
Robert M. Lembo, MD
Associate Professor of Clinical Pediatrics and Director, Medical
Education, Department of Pediatrics, New York University School of Michael J. Painter, MD
Medicine; Attending Physician, Bellevue Hospital Center, New York, Professor of Neurology and Pediatrics, Division of Child Neurology,
New York University of Pittsburgh School of Medicine; Children’s Hospital of
Fever and Rash Pittsburgh, Pittsburgh, Pennsylvania
Hypotonia and Weakness
David A. Lewis, MD
Associate Professor of Pediatrics, Division of Pediatric Cardiology; Cynthia G. Pan, MD
Director, Graduate Medical Education Program, Medical College of Associate Professor of Pediatrics, Medical College of Wisconsin;
Wisconsin, Milwaukee, Wisconsin (Deceased) Medical Director, Dialysis Unit and Nephrology, Children’s Hospital
Syncope and Dizziness of Wisconsin, Milwaukee, Wisconsin
Polyuria and Urinary Incontinence
Elaine Wyllie, MD
Frederick J. Suchy, MD
Head, Section of Pediatric Neurology and Pediatric Epilepsy,
Professor and Chair, Department of Pediatrics, Mount Sinai School of
Cleveland Clinic Foundation, Cleveland, Ohio
Medicine; Pediatrician-in-Chief, Mount Sinai Hospital, New York, Paroxysmal Disorders
New York
Hepatomegaly
Robert Wyllie, MD
Chair, Department of Pediatric Gastroenterology, Cleveland Clinic
William J. Swift, MD Foundation, Cleveland, Ohio
Professor Emeritus of Child and Adolescent Psychiatry, University Constipation
of Wisconsin Medical School and Wisconsin Psychiatric Institute
and Clinic, Madison, Wisconsin; Regional Medical Officer and
Psychiatrist, U.S. Department of State, Pretoria, South Africa
Unusual Behaviors
Preface
Most children’s hospitals and pediatric residency training programs This text is intended to help the reader begin with a specific chief
have multiple educational conferences, such as professor rounds, complaint that may encompass many disease entities. In a user-
patient management conference, clinicopathologic conference, and friendly, well-tabulated, and illustrated approach, the text will help
senior resident intake rounds. In these high-quality learning activi- the reader differentiate between the many disease states causing a
ties, experienced master clinician-educators lead a discussion of a common chief complaint. The inclusion of many original tables and
particular patient-based issue, permitting the trainees to see how a figures should help the reader identify distinguishing features of
master clinician thinks through diagnostic or therapeutic challenges. diseases and work through a diagnostic and/or therapeutic approach
The advice given is derived from the knowledge accumulated over to the problem using decision trees. Modified, adapted, and borrowed
many years of clinical experience and careful analysis of the medical artwork and tables from other outstanding sources have been added
literature. The synthesis of the facts of the case with the clinician’s as well. The combination of all of these illustrations and tables will
practical experience and knowledge of the literature often results in help provide a quick visual guide to the differential diagnosis or
the diagnosis and the appropriate treatment strategy. These master treatment of the various diseases under discussion.
clinician-educators provide wisdom that gives clarity to confusing
clinical cases and helps to reconcile discrepancies between practice We greatly appreciate the hard work of our contributing authors.
and theory. Writing a chapter in this type of format is quite different from
In addition, master clinician-educators focus on the importance writing in the format of a disease-based book. In addition, we greatly
of a detailed history and a complete physical examination. The chief appreciate the efforts of Judy Fletcher of Elsevier, whose patience
complaint directs the questioning during the history, whereas the and expertise contributed to the publication of this book. We are all
physical examination focuses on clues obtained by the history. also greatly appreciative of Carolyn Redman of the Department of
Laboratory and other studies are then employed to support the Pediatrics at the Medical College of Wisconsin, whose editorial
diagnosis, not to make the diagnosis. assistance and organization has made this edition a reality. The
The goal of this book is to put into a written text the oral teach- authors also wish to make a special acknowledgment to Dr. Brendan
ing rounds–based approaches toward clinical problem solving of the M. Reilly, for his courtesy and assistance. Finally, we acknowledge
many expert clinician-educators who present at teaching confer- the support and, at times, sacrifice of our families: Sharon, Jonathan,
ences. The combination of clinical experience and evidenced-based Rachel, Alison, and Matthew Kliegman; Jordan, Harry, and Irene
strategies will provide guidance in developing a differential diagno- Greenbaum; and Dale, Erin, John, and Therese Lye, whose
sis, then a specific diagnosis, and finally the appropriate therapy of understanding helped make the time and effort put into this book
common pediatric problems. This book is arranged in chapters that meaningful.
cover specific chief complaints, mirroring clinical practice. Patients
do not usually present with a chief complaint of cystic fibrosis; ROBERT M. KLIEGMAN
rather, they may present with a cough, respiratory distress, or chronic LARRY A. GREENBAUM
diarrhea.
PATRICIA S. LYE
1 Sore Throat
Sore throat is a common chief complaint. Each year approximately The enteroviruses (coxsackievirus and echovirus) 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
throat complaints. The majority of these illnesses are nonbacterial throat is slightly red; tonsillar exudate and cervical adenopathy are
and neither necessitate nor are alleviated by antibiotic therapy unusual. Symptoms resolve within a few days. Enteroviruses can
(Tables 1-1 to 1-3). Acute streptococcal pharyngitis, however, warrants
accurate diagnosis and therapy to prevent serious suppurative
and nonsuppurative complications. Furthermore, life-threatening
Table 1-1. Etiology of Sore Throat
infectious complications of streptococcal and nonstreptococcal
oropharyngeal infections may manifest with mouth pain, pharyngitis, Infection
parapharyngeal space infectious extension, and airway obstruction Bacterial (see Tables 1-2, 1-3)
(Tables 1-4 and 1-5). Viral (see Tables 1-2, 1-3)
Fungal (see Table 1-3)
Neutropenic mucositis (invasive anaerobic mouth flora)
VIRAL PHARYNGITIS Tonsillitis
Epiglottitis
Most episodes of pharyngitis are caused by viruses (see Tables 1-2 Uvulitis
and 1-3). It is difficult to clinically distinguish between viral and Peritonsillar abscess (quinsy sore throat)
bacterial pharyngitis with a very high degree of precision, but certain Retropharyngeal abscess (prevertebral space)
clues may help the physician. Accompanying symptoms of conjunc- Ludwig angina (submandibular space)
tivitis, rhinitis, croup, or laryngitis are common with viral infection Lateral pharyngeal space cellulitis-abscess
but rare in bacterial pharyngitis. Buccal space cellulitis
Many viral agents can produce pharyngitis (see Tables 1-2 Suppurative thyroiditis
and 1-3). Some cause distinct clinical syndromes that are readily Lemierre disease (septic jugular thrombophlebitis)
diagnosed without laboratory testing (see Tables 1-1, 1-4, and 1-6). Vincent angina (mixed anaerobic
In pharyngitis caused by parainfluenza and influenza viruses, bacteria–gingivitis–pharyngitis)
rhinoviruses, coronaviruses, and respiratory syncytial virus (RSV), Irritation
the symptoms of coryza and cough often overshadow sore throat,
which is generally mild. Influenza virus may cause high fever, Cigarette smoking
cough, headache, malaise, myalgias, and cervical adenopathy in addi- Inhaled irritants
tion to pharyngitis. In young children, croup or bronchiolitis may Reflux esophagitis
develop. RSV is associated with bronchiolitis, pneumonia, and croup Chemical toxins (caustic agents)
in young children. RSV infection in older children is usually indis- Paraquat ingestion
tinguishable from a simple upper respiratory tract infection. Smog
Pharyngitis is not a prominent finding of RSV infection in either age Dry hot air
group. Parainfluenza viruses are associated with croup and bronchi- Hot foods, liquids
olitis; minor sore throat and signs of pharyngitis are common at the Other
outset but rapidly resolve. Infections caused by parainfluenza, Tumor, including Kaposi sarcoma, leukemia
influenza, and RSV are often seen in seasonal (winter) epidemics. Wegener granulomatosis
Adenoviruses can cause upper and lower respiratory tract disease, Sarcoidosis
ranging from ordinary colds to severe pneumonia. The incubation Glossopharyngeal neuralgia
period of adenovirus infection is 2 to 4 days. Upper respiratory tract Foreign body
infection typically produces fever, erythema of the pharynx, and fol- Stylohyoid syndrome
licular hyperplasia of the tonsils, together with exudate. Enlargement Behçet disease
of the cervical lymph nodes occurs frequently. When conjunctivitis Kawasaki syndrome
occurs in association with adenoviral pharyngitis, the resulting Posterior pharyngeal trauma—pseudodiverticulum
syndrome is called pharyngoconjunctival fever. Pharyngitis may last Pneumomediastinum
as long as 7 days and does not respond to antibiotics. There are many Hematoma
adenovirus serotypes; adenovirus infections may therefore develop Systemic lupus erythematosus
in children more than once. Laboratory studies may reveal a leuko- Bullous pemphigoid
cytosis and an elevated erythrocyte 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.
3
4 Section One Respiratory Disorders
lasts less than 7 days, but severe pain may impair fluid intake
Table 1-2. Infectious Etiology of Pharyngitis and necessitate medical support.
Definite Causes Coxsackievirus A16 causes hand-foot-mouth disease. Vesicles
can occur throughout the oropharynx; they are painful, and
Streptococcus pyogenes (Group A streptococci) they ulcerate. Vesicles also develop on the palms, soles, and,
Corynebacterium diphtheriae less often, on the trunk or extremities. Fever is present in most
Arcanobacterium haemolyticum cases, but many children do not appear seriously ill. This
Neisseria gonorrhoeae disease lasts less than 7 days.
Epstein-Barr virus
Parainfluenza viruses (types 1–4) Primary infection caused by herpes simplex virus (HSV) usually
Influenza viruses produces high fever with acute gingivostomatitis, involving vesicles
Rhinoviruses (which become ulcers) throughout the anterior portion of the mouth,
Coronavirus including the lips. There is sparing of the posterior pharynx in herpes
Adenovirus (types 3, 4, 7, 14, 21, others) gingivostomatitis; the infection usually occurs in young children.
Respiratory syncytial virus High fever is common, pain is intense, and intake of oral fluids is
Herpes simplex virus (types 1, 2) often impaired, which may lead to dehydration. In addition, HSV
may manifest in adolescents with pharyngitis. Approximately 35%
Probable Causes
of new-onset HSV-positive adolescent patients have herpetic lesions;
Group C streptococci most patients with HSV pharyngitis cannot be distinguished from
Group G streptococci patients with other causes of pharyngitis. The classic syndrome of
Chlamydia pneumoniae herpetic gingivostomatitis in infants and toddlers lasts up to 2 weeks;
Chlamydia trachomatis data on the course of more benign HSV pharyngitis are lacking. The
Mycoplasma pneumoniae differential diagnosis of vesicular-ulcerating oral lesions is noted in
Table 1-6. A common cause of a local and large lesion of unknown
etiology is aphthous stomatitis (Fig. 1-1). Some children have a
cause meningitis, rash, and two specific syndromes that involve the combination of periodic fever (recurrent at predictable fixed times),
oropharynx: aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA); this
syndrome is idiopathic and may respond to oral prednisone or
Herpangina is characterized by distinctive discrete, painful, gray- cimetidine. PFAPA usually begins before the age of 5 years and is
white papulovesicular lesions distributed over the posterior characterized by high fever lasting 4 to 6 days, occurring every 2 to
oropharynx (Table 1-6). The vesicles are 1 to 2 mm in 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 39.5°C. The illness generally findings early in the course of the disease. In addition to high fever,
cough, coryza, and conjunctivitis, the pharynx may be intensely and
diffusely erythematous, without tonsillar enlargement or exudate.
The presence of Koplik spots, the pathognomonic white or blue-
Table 1-3. Additional Potential Pathogens Associated
white enanthem of measles, on the buccal mucosa near the mandibu-
with Sore Throat
lar molars provides evidence of the correct diagnosis before the rash
Bacteria develops.
Fusobacterium necrophorum (Lemierre disease)
Neisseria meningitidis
Yersinia enterocolitica INFECTIOUS MONONUCLEOSIS
Tularemia (orpharyngeal)
Yersinia pestis PATHOGENESIS
Bacillus anthracis
Chlamydia psittaci Acute exudative pharyngitis commonly occurs with infectious
Secondary syphilis mononucleosis caused by primary infection with Epstein-Barr virus
Mycobacterium tuberculosis (EBV) (Table 1-7). Mononucleosis is a febrile, systemic, self-limited
Lyme disease lymphoproliferative disorder that is usually associated with
Corynebacterium ulcerans hepatosplenomegaly and generalized lymphadenopathy. The pharyn-
Leptospira species gitis may be mild or severe, with significant tonsillar hypertrophy
Mycoplasma hominis (possibly producing airway 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
Cytomegalovirus young adults; EBV infection is generally milder or subclinical in
Viral hemorrhagic fevers preadolescent children. In United States high school and college
Human immunodeficiency virus students, attack rates are 200 to 800 per 100,000 population per year.
(HIV) (primary infection) EBV is transmitted primarily by saliva.
Human herpesvirus 6
Measles
Varicella CLINICAL FEATURES
Rubella After a 2- to 4-week incubation period, patients with infectious
Fungus mononucleosis usually experience an abrupt onset of malaise, fatigue,
Candida species fever, and headache, followed closely by pharyngitis. The tonsils are
Histoplasmosis enlarged with exudates and cervical adenopathy. More generalized
Cryptococcosis adenopathy with hepatosplenomegaly often follows. Fever and
pharyngitis typically last 1 to 3 weeks, while lymphadenopathy and
Table 1-4. Distinguishing Features of Parapharyngeal–Upper Respiratory Tract Infections
Submandibular Postanginal
Retropharyngeal Space Lateral Laryngotracheo- Sepsis*
Peritonsillar Abscess (Ludwig Pharyngeal Masticator bronchitis Bacterial (Lemierre
Abscess (Cellulitis) Angina)* Space* Space* Epiglottitis (Croup) Tracheitis Disease)
Etiology Group A Staphylococcus Oral anaerobes† Oral anaerobes† Oral anaerobes† Haemophilus Parainfluenza Moraxella Fusobacterium
streptococci, oral aureus, oral influenzae virus; influenza catarrhalis, necrophorum
anaerobes† anaerobes,† group A type b adeno-virus S. aureus,
streptococci, and respiratory H. influenzae
“suppurative syncytial virus type b or
adenitis” less common nontypable
Age Teens Infancy, preteens, Teens Teens Teens 2–5 yr 3 mo–3 yr 3–10 yr Teens
occasionally teens
Manifestations Initial episode of Fever, dyspnea, Fever, dysphagia, Severe pain, Pain, prominent Sudden-onset Low-grade fever, Prior history of Prior
pharyngitis, stridor, dysphagia, odynophagia, fever, trismus, trismus, fever high fever, barking cough, croup with pharyngitis
followed by drooling, stiff stiff neck, dysphagia, Swelling not “toxic ” hoarseness- sudden onset with sudden-
sudden worsening neck, pain, dyspnea; airway edematous always evident appearance, aphonia, of respiratory onset fever,
of unilateral cervical obstruction, appearing, muffled voice, stridor; mild distress, high chills,
odynophagia, adenopathy, swollen tongue painful lateral anxiety, pain, retractions; fever, “toxic” odynophagia,
trismus, hot swelling of and floor of facial (jaw) or retractions, radiograph appearance, neck pain,
potato (muffled) posterior mouth (tender) neck swelling dysphagia, shows hoarseness, septic
voice, drooling, pharyngeal space Muffled voice (induration) drooling, “steeple sign” stridor, thrombo-
displacement Descending May lead to stridor, sitting of subglottic barking phlebitis
of uvula mediastinitis (rare) Lemierre up, leaning narrowing on cough, tripod of internal
Lateral neck disease forward tripod anteroposterior sitting jugular vein
radiograph position, neck view position; with septic
reveals swollen cherry-red radiograph emboli
retropharyngeal swollen as per croup (e.g., lungs,
prevertebral space: epiglottis plus ragged joints),
Chapter 1 Sore Throat
†
Peptostreptococcus, Fusobacterium, Bacteroides (usually melaninogenicus).
*Often odontogenic; check for tooth abscess, caries, tender teeth.
CT, computed tomography.
5
6 Section One Respiratory Disorders
Recurrent
Scarifying
Systemic Inflammatory Ulcerative
Lupus Bowel Stomatitis
Hand-Foot- Erythematosus Disease Aphthous Behçet Vincent (Sutton
Gingivostomatitis Herpangina Mouth Disease Chickenpox (SLE) (IBD) Stomatitis Disease Stomatitis Disease)
Etiology Herpes simplex Coxsackievirus Coxsackievirus Varicella- Unknown; Unknown; Unknown Unknown; Unknown; Unknown
virus (HSV) I A, B; echovirus A, coxsackie- zoster virus autoimmune autoimmune vasculitis or
or HSV virus B anaerobic
(rarely) (rarely) bacteria
Location Ulcerative vesicles Anterior fauces Tongue, buccal Tongue, Oral, nasal Lips, tongue, As in IBD Oral (similar Gingiva; Tongue;
of pharynx, (tonsils), soft mucosa, gingiva, mucosa; buccal to IBD); ulceration buccal
tongue, and palate (uvula), palate, palms, buccal palate, mucosa, genital at base of mucosa
palate less often soles, mucosa, pharynx, oropharynx ulcers teeth
plus lesions of pharynx anterior marked buccal
mucocutaneous oral cavity cutaneous mucosa
(perioral) lesions;
margin trunk > face
Age Less than 5 yr 3–10 yr 1 yr–teens Any age Any age Any age Teens and Teens, adult- Teens; if Teens
adulthood hood, younger,
occasionally consider
<10 yr immuno-
deficiency
and blood
dyscrasia
Manifestations Fever, mouth pain, Fever, sore Painful bilateral Fever, pruritic Renal, central Multiple Similar to Painful Fever, Deep,
toxic, fetid throat, vesicles, cutaneous nervous recurrences; IBD ulcerations bleeding large,
breath, drooling, odynophagia; fever vesicles, system, painful (heal gums; painful
anorexia, cervical summer out- painful oral arthritis, ulcerations without gray ulcera-
lymphadenopathy; breaks; 6–12 lesions cutaneous, 1–2 mm, but scarring); membrane tions;
Chapter 1 Sore Throat
disease
8 Section One Respiratory Disorders
DIAGNOSIS
Although signs and symptoms may strongly suggest acute strepto-
coccal pharyngitis, laboratory diagnosis is highly recommended,
even for patients with scarlet fever (Fig. 1-3). Scoring systems for
diagnosing acute group A streptococcal pharyngitis on clinical
grounds have not proved very useful. Using clinical criteria alone,
physicians overestimate the likelihood that patients have streptococ-
cal infection. The throat culture has traditionally been used to
Figure 1-2. Typical human serologic response to Epstein-Barr virus infec- diagnose streptococcal pharyngitis. Plating a swab of the posterior
tion. At time of clinical presentation (usually 2 to 7 weeks after exposure), pharynx and tonsils on sheep blood agar, identifying β−hemolytic
anti–viral capsid antigen (VCA) response may consist of IgM and IgG colonies, and testing them for the presence of sensitivity to a bacitracin-
antibodies; anti–early antigen (EA) response is often present; and impregnated disk is the “gold standard” diagnostic test, but it takes
anti–nuclear antigen (EBNA) is usually negative. The IgM anti-VCA 24 to 48 hours to obtain results. There are a number of rapid
response usually subsides within 2 to 4 months, and the anti-EA response diagnostic tests that take less than 15 minutes. These “rapid strep”
usually disappears within 2 to 6 months. (Data from Andiman WA, tests detect the presence of the cell wall group A carbohydrate anti-
McCarthy P, Markowitz RI, et al: Clinical, virologic, and serologic evi- gen after acid extraction of organisms obtained by throat swab.
dence of Epstein-Barr virus infection in association with childhood pneu- Rapid strep tests are highly specific (generally >95%), with the
monia. J Pediatr 1981;99:880-886; Fleisher G, Henle W, Henle G, et al: throat culture used as the standard. Unfortunately, the sensitivity of
Primary infection with Ebstein-Barr virus in infants in the United States: most of these rapid tests can be considerably lower. In comparison to
Clinical and serological observations. J Infect Dis 1979;139:553-558; hospital or reference laboratory throat culture results, the sensitivities
Brown NA: The Epstein-Barr virus (infectious mononucleosis, B-lymphopro- of these tests are generally 80% to 85% and can be lower. However,
liferative disorders). In Feigin RD, Cherry JD [eds]: Textbook of Pediatric when both throat cultures and rapid tests performed in physicians’
Infectious Diseases, 2nd ed. Philadelphia, WB Saunders, 1987.) offices are compared with cultures performed in reference laboratories,
Chapter 1 Sore Throat 9
Testing patients for serologic evidence of an antibody response to
Table 1-8. Characteristics of Severe Invasive and/or extracellular products of group A streptococci is not useful for
Toxigenic Group A Streptococcal Infection diagnosing acute pharyngitis. Because it generally takes several
Positive Culture Sites weeks for antibody levels to rise, streptococcal antibody tests are
valid only for determining past infection. Specific antibodies include
Blood antistreptolysin O (ASO), anti-DNase B, and antihyaluronidase
Soft tissue abscess (AHT). When antibody testing is desired in order to evaluate a pos-
Synovial fluid sible post-streptococcal illness, more than one of these tests should
Throat be performed to improve sensitivity.
Peritoneal fluid
Surgical wound
Cellulitis aspirate TREATMENT
Clinical Manifestations Laboratory Manifestations Treatment begun within 9 days of the onset of group A streptococcal
Fever Leukocytosis pharyngitis is effective in preventing acute rheumatic fever. Therapy
Toxic Shock* Lymphopenia does not appear to affect the risk of the other nonsuppurative
Confusion Thrombocytopenia sequela, acute post-streptococcal glomerulonephritis. Antibiotic
Headache Hyponatremia therapy also reduces the incidence of suppurative sequelae of group A
Abdominal pain Hypoalbuminemia streptococcal pharyngitis, such as peritonsillar abscess and cervical
Vomiting Hyperbilirubinemia (direct) adenitis. In addition, treatment produces a more rapid resolution of
Local extremity pain and Elevated AST, ALT, BUN signs and symptoms and terminates contagiousness within 24 hours.
swelling Renal sediment abnormalities For these reasons, antibiotics should be instituted as soon as the
Hypesthesia Coagulopathy diagnosis is supported by laboratory studies.
Cellulitis Hypoxia There are numerous antibiotics available for treating streptococcal
Scarlatiniform rash (40%) pharyngitis (Table 1-10). The drug of choice is penicillin. Despite the
Erythroderma (25%) widespread use of penicillin to treat streptococcal and other infections,
Conjunctival injection penicillin resistance among group A streptococci has not developed.
Red pharynx Penicillin can be given by mouth for 10 days or intramuscularly as a
Pneumonia with or single injection of benzathine penicillin. Intramuscular benzathine
without empyema penicillin alleviates concern with patient compliance. A less painful
Osteomyelitis alternative is benzathine penicillin in combination with procaine peni-
Vaginitis cillin. Intramuscular procaine penicillin alone is inadequate for pre-
Proctitis vention of acute rheumatic fever because adequate levels of penicillin
Desquamation are not present in blood and tissues for a sufficient time. Other
Necrotizing fasciitis β-lactams, including semisynthetic derivatives of penicillin and the
Diarrhea cephalosporins, are at least as effective as penicillin for treating group
A streptococcal pharyngitis. Their broader spectrum, their higher cost,
*Case definition of streptococcal toxic shock syndrome requires (I) isolation of group A and the lack of formal data concerning prevention of acute rheumatic
streptococci from (a) a normally sterile site (blood, synovial or peritoneal fluid) or (b) a fever relegate them to second-line status. The decreased frequency of
nonsterile site (throat, wound). (II) Severity is defined by (a) hypotension and (b) two or
more of renal impairment, coagulopathy, liver involvement, adult respiratory distress
dose administration of some of these agents may improve patient
syndrome, a generalized erythematous macular rash (with or without later desquama- compliance and makes their use attractive in selected circumstances.
tion), and soft tissue necrosis (necrotizing fasciitis, myositis, gangrene). The definitive Patients who are allergic to penicillin should receive erythro-
diagnosis requires criteria IA and IIA plus B. Criteria IB and IIA plus B are considered mycin or another non–β-lactam antibiotic, such as clarithromycin,
probable if no other identifiable cause is present.
azithromycin, or clindamycin. Resistance of group A streptococci to
ALT, alanine aminotransferase; AST, aspartate aminotransferase; BUN, blood urea erythromycin has increased dramatically in areas such as Japan,
nitrogen.
France, Spain, Taiwan, and Finland, where erythromycin has been
widely used. This has not yet emerged as a major problem in the
United States, where the rate of macrolide resistance is about 5%.
the sensitivities, specificities, and overall accuracy of the office Sulfa drugs (including sulfamethoxazole combined with trimetho-
culture and the office rapid test are quite similar; the latter often prim), tetracyclines, and chloramphenicol should not be used for
performs better than the culture. treatment of acute streptococcal pharyngitis because they do not
The low sensitivity of these tests, coupled with their excellent eradicate group A streptococci.
specificity, has led to the recommendation that two swabs be
obtained from patients with suspected streptococcal pharyngitis. COMPLICATIONS
One swab is used for a rapid test. When the rapid antigen detection
test result is positive, it is highly likely that the patient has group A Suppurative Complications
streptococcal infection, and the extra swab can be discarded. When
the rapid test result is negative, group A streptococci may nonethe- Antibiotic therapy has greatly reduced the likelihood of developing
less be present; thus, the extra swab should be processed for culture. suppurative complications caused by spread of group A streptococci
Physician offices that have demonstrated that their rapid test and from the pharynx or middle ear to adjacent structures. Peritonsillar
throat culture results are comparable may be able to rely on the rapid abscess (“quinsy”) manifests with fever, severe throat pain, dyspha-
test result even when it is negative, without performing a backup gia, “hot potato voice,” pain referred to the ear, and bulging of the
culture. peritonsillar area with asymmetry of the tonsils and sometimes
In general, patients with a negative result of the rapid test do not displacement of the uvula (Fig. 1-4; see Table 1-4). On occasion,
require treatment before culture verification unless there is a partic- there is peritonsillar cellulitis without a well-defined abscess cavity.
ularly high suspicion group A streptococcal infection (e.g., scarlet Trismus may be present. When an abscess is found clinically or by
fever, peritonsillar abscess, or tonsillar exudates in addition to tender an imaging study such as a computed tomographic scan, surgical
cervical adenopathy, palatal petechiae, fever, and recent exposure to drainage is indicated. Peritonsillar abscess occurs most commonly in
a person with group A streptococcal pharyngitis). older children and adolescents.
10 Section One Respiratory Disorders
Retropharyngeal abscess represents extension of infection preventing rheumatic fever, but acute glomerulonephritis is not
from the pharynx or peritonsillar region into the retropharyngeal prevented by treatment of the antecedent streptococcal infection.
(prevertebral) space, which is rich in lymphoid structures (Figs. 1-5 Pharyngitis caused by one of the nephritogenic strains of group A
and 1-6; see Table 1-4). Children younger than 4 years old are most streptococci precedes the glomerulonephritis by about 10 days.
often affected. Fever, dysphagia, drooling, stridor, extension of the Unlike acute rheumatic fever, which occurs only after group A strep-
neck, and a mass in the posterior pharyngeal wall may be noted. tococcal pharyngitis, acute glomerulonephritis also can follow group
Surgical drainage is often required if frank suppuration has occurred. A streptococcal skin infection.
Spread of group A streptococci via pharyngeal lymphatic vessels to
regional nodes can cause cervical lymphadenitis. The markedly TREATMENT FAILURE AND CHRONIC CARRIAGE
swollen and tender anterior cervical nodes that result can suppurate.
Otitis media, mastoiditis, and sinusitis also may occur as compli- Treatment with penicillin cures group A streptococcal pharyngitis
cations of group A streptococcal pharyngitis. Additional parapharyn- but is unable to eradicate group A streptococci from the pharynx in
geal suppurative infections that may mimic streptococcal disease are approximately 25% of patients (Fig. 1-7). This causes considerable
noted in Table 1-4. Furthermore, any pharyngeal infectious process consternation among such patients and their families. Penicillin
may produce torticollis if there is inflammation that extends to the resistance is not the cause of treatment failure. A small proportion of
paraspinal muscles and ligaments, producing pain, spasm, and, on these patients are symptomatic and are thus characterized as having
occasion, rotary subluxation of the cervical spine. clinical treatment failure. Reinfection with the same strain or a dif-
The differential diagnosis of torticollis is presented in Table 1-11. ferent strain is possible, as is intercurrent viral pharyngitis. Some of
Oropharyngeal torticollis lasts less than 2 weeks and is not associated these patients may be chronic pharyngeal carriers of group A strep-
with abnormal neurologic signs or pain over the spinous process. tococci who are suffering from a new superimposed viral infection;
others may be noncompliant with regard to therapy.
Nonsuppurative Sequelae Many patients who do not respond to antimicrobial treatment are
asymptomatic and are identified when follow-up culture specimens
Nonsuppurative complications include acute rheumatic fever (see are obtained, a practice that is usually unnecessary. Patients who are
Chapters 11 and 44), acute post-streptococcal glomerulonephritis compliant with regard to therapy are at minimal risk for acute rheu-
(see Chapter 25), and possibly reactive arthritis/synovitis. In addi- matic fever. One explanation for asymptomatic persistence of group A
tion, an association between streptococcal infection and neuro- streptococci after treatment is that these patients were chronic
psychiatric disorders such as obsessive-compulsive disorder and carriers of group A streptococci who were initially symptomatic
Tourette syndrome has been postulated. This possible association because of a concurrent viral pharyngitis and who did not truly have
has been called PANDAS (pediatric autoimmune neuropsychiatric acute streptococcal pharyngitis.
disorders associated with streptococci). Therapy with an appropriate Patients who are chronically colonized with group A streptococci
antibiotic within 9 days of onset of symptoms is highly effective in are called chronic carriers. Carriers do not appear to be at risk for acute
Chapter 1 Sore Throat 11
Signs and Symptoms Compatible with GAS Available treatment options for the physician faced with a
chronic streptococcal carrier include the following:
Yes No Viral 1. Obtaining a rapid test, throat culture, or both each time the patient
pharyngitis has pharyngitis with features that suggest streptococcal pharyngitis,
Symptomatic and treating with penicillin each time a test is positive.
therapy 2. Treating with one of the regimens effective for terminating
Scarlet fever? (No antibiotics) chronic carriage.
Yes No Rapid
The first option is simple, as safe as penicillin, and appropriate for
GAS pharyngitis test many patients. The second option should be reserved for particularly
anxious patients; those with a history of acute rheumatic fever or
Positive living with someone who had it; or those living or working in nursing
Negative homes, chronic care facilities, hospitals, and perhaps schools.
Confirm with rapid test The two antibiotic treatment regimens that have been effective for
or throat culture; eradication of the carrier state are:
Treat with antibiotics Throat
culture ● Intramuscular benzathine penicillin plus oral rifampin (10 mg/kg/
dose up to 300 mg, given twice daily for 4 days beginning on the
Not GAS Negative Positive GAS day of the penicillin injection)
pharyngitis pharyngitis ● Oral clindamycin, given for 10 days (20 mg/kg/day up to 450 mg,
Standard
Penicillin V 250 mg 500 mg bid-tid Oral 10 days
Benzathine penicillin G 600,000 U (weight < 27 kg) 1.2 million U (weight ≥ 27 kg) Once IM Once
Amoxicillin 125 mg (weight < 15 kg) 250 mg (weight ≥ 15 kg) tid Oral 10 days
Penicillin-Allergic Patients
Oral Dose Frequency Duration
Erythromycin
Ethylsuccinate 40 mg/kg/day up to 1000 mg/day bid 10 days
Estolate 20-40 mg/kg/day up to 1000 mg/day bid 10 days
Clarithromycin 15 mg/kg/day up to 500 mg/day bid 10 days
Azithromycin* 12 mg/kg/day Once daily 5 days
Clindamycin 10-25 mg/kg/day up to 450 mg/day tid 10 days
Cephalosporins† Varies with agent chosen 10 days
Once Daily and Short Duration Treatment Schedules
Oral Dose Frequency Duration
However, the exact role of these agents, most of which are carried
asymptomatically in the pharynx of some children and young adults,
remains to be fully characterized.
When they are implicated as agents of acute pharyngitis, groups
C and G organisms do not appear to necessitate treatment, inasmuch
as they cause self-limited infections. Acute rheumatic fever is not a
sequela to these infections, although post-streptococcal acute
glomerulonephritis has been documented in rare cases after epidemic
group C and group G streptococcal pharyngitis.
ARCANOBACTERIUM INFECTION
A B
Figure 1-5. Retropharyngeal abscess. A, Lateral neck radiograph shows marked increased soft tissue (arrow) between
the upper airway and cervical spine. B, Axial computed tomographic scan shows the lower attenuation center of the abscess
(A), the anterior and leftward shift of the trachea (T), and the soft tissue mass (M) of abscess and surrounding edema. (Courtesy
of A. Oestreich, M.D., Cincinnati, Ohio.)
DIPHTHERIA of diphtheria in the former Soviet Union has been recorded (1990 to
1995), and infection has been documented in several travelers from
Western Europe.
Diphtheria is a very serious disease that is caused by pharyngeal
infection by toxigenic strains of Corynebacterium diphtheriae. It has
become very rare in the United States and other developed countries PATHOGENESIS
as a result of immunization. The handful of diphtheria cases recog-
nized annually in the United States usually occur in unimmunized The pathogenesis of diphtheria involves nasopharyngeal mucosal col-
individuals, and the fatality rate is about 5%. A relatively large outbreak onization by C. diphtheriae and toxin elaboration after an incubation
period of 1 to 5 days. Toxin leads to local tissue inflammation and
necrosis (producing an adherent grayish membrane made up of fibrin,
blood, inflammatory cells, and epithelial cells) and it is absorbed into
the blood stream. Fragment B of the polypeptide toxin binds particu-
larly well to cardiac, neural, and renal cells, and the smaller fragment
A enters cells and interferes with protein synthesis. Toxin fixation by
tissues may lead to fatal myocarditis (with arrhythmias) within 10 to
14 days and to peripheral neuritis within 3 to 7 weeks.
CLINICAL FEATURES
Imports. Exports.
1846. January 59 ships 11,564 tons. 59 ships 11,875 tons.
” February 60 ” 11,251 ” 62 ” 11,208 ”
” March 72 ” 11,252 ” 70 ” 11,289 ”
” April 63 ” 10,971 ” 66 ” 11,098 ”
” May 61 ” 11,539 ” 121 ” 11,790 ”
” June 61 ” 10,637 ” 97 ” 14,715 ”
” July 81 ” 13,413 ” 94 ” 14,274 ”
” August 80 ” 13,194 ” 93 ” 16,042 ”
” September 94 ” 13,515 ” 65 ” 11,609 ”
” October 64 ” 11,472 ” 71 ” 13,158 ”
” November 63 ” 11,094 ” 51 ” 8,619 ”
” December 41 ” 7,785 ” not obtained.
799 ships 137,687 tons. 849 ships 135,677 tons.
Foreign 24 ” 6,935 ” 13 ” 2,703 ”
Total 823 ships 144,622 tons. 862 ships 138,380 tons.
Now that the dock is no longer a mere word and promise, but has
at length a definite signification and a material existence, there is
every appearance that those into whose hands the fortunes of the
port may be said to have been entrusted have no intention of any
dilatory action in furthering the interests of their charge. Already, in
1875, a powerful steam dredger has been purchased at a cost of
£12,000 and set to its labours in the channel and harbour. This
dredger, which has superseded the older and much smaller one,
launched in 1840 and used until recently, was built by Simonds and
Company, of Renfrew, on the Clyde, and is of 100-horse power,
being capable of raising 250 tons of sand, shingle, etc., in an hour.
In addition it is able to work in twenty-six feet of water, whereas the
original one was obliged to wait until the tide had ebbed to fourteen
feet before operations could be commenced, so that really the work
which can be accomplished by the new machine is out of all
proportion to that which its predecessor could effect. Several iron
pontoons, or lighters, furnished with false bottoms to expedite the
business of discharging them, formerly performed by hand and
spade, have also been obtained; and the bed of the river seaward
from Fleetwood is rapidly being relieved of its superabundance of
tidal deposits and scourings, which is carried by the lighters beyond
the marine lighthouse at the foot of the Wyre and deposited in the
Lune.
Steamboat traffic was, and is, the most important branch of
shipping connected with the port, but notwithstanding the support
and encouragement which has been so freely extended to the
Belfast line, sundry attempts by the same company to establish sea-
communications between Fleetwood and other places have
invariably ended in complete failures. In the context we have
endeavoured to trace a brief outline of the steamship trade of the
harbour from its earliest days up to our time. The North Lancashire
Steam Navigation Company was established in 1843, and
commenced operations by running the “Prince of Wales” and the
“Princess Alice,” two large and fast iron steamships for that date,
between this port and Belfast on each Wednesday and Saturday
evening, the return trips being made on the Monday and Friday. In
that year, however, the number of trips was increased to three per
week, the fares for the single journey being, saloon, 15s.; and deck,
3s. Another steamship the “Robert Napier,” of 220 horse-power,
sailed also from Fleetwood in 1843, every Friday morning, at 10 a.m.
for Londonderry, calling at Portrush, and returned on Tuesday, the
fares being, cabin, 20s.; and deck, 5s. In 1844 we find that
communications, through the exertion and enterprise of the above
company, were open between Fleetwood and Belfast, Londonderry,
Ardrossan, and Dublin, respectively. The Ardrossan line consisted of
two new iron steamboats, “Her Majesty,” and the “Royal Consort,”
each of which was 300 tons register, and 350 horse-power, the fares
being, cabin, 17s.; and deck, 4s. The Dublin trip was performed
once, and afterwards twice, a week each way, by the iron steamship
“Hibernia,” which called off Douglas, Isle of Man, to land passengers,
but after a year’s trial this communication was closed. In the
summer of 1845, an Isle of Man line was opened by the steamship
“Orion,” which ran daily, except Sundays; and at the same season
the Belfast boats commenced to make the double journey four days
a week, whilst the Londonderry route was abandoned. As early as
1840, on the completion of the Preston and Wyre Railway, a daily
steam communication had been established to Bardsea, as the
nearest point to Ulverston and the Lakes; and in the month of
September, 1846, on the completion of Piel Pier, it was transferred to
that harbour, and continued by the steamship “Ayrshire Lassie,” of
100 horse-power, the fares being, saloon, 2s.; and deck, 1s. In the
following year this boat was superseded by a new steamer, the
“Helvellyn,” of 50 tons register and 75 horse-power, which continued
to ply for many years, in fact, almost until this summer line was
closed, at a comparatively recent date, about eight or ten years ago.
The Fleetwood and Ardrossan steamers discontinued running in
1847, and at the same time an extra boat, the “Fenella,” was placed
on the Isle of Man route, whilst the Belfast trips were reduced to
three double journeys per week. After a few years experience the
Isle of Man line, a season one only, was given up; but the Belfast
trade, continually growing, soon obliged the company to increase
the number of trips, and step by step to enlarge and improve the
boat accommodation. We need not trace through its different stages
the gradual and satisfactory progress of this line, but our object will
be sufficiently attained by stating that the two steamships were
shortly increased to three. Afterwards larger and finer boats, having
greater power, took the places of the original ones, and at the
present day the fleet consists of four fine steamers of fully double
the capacity of the original ones, which cross the channel from each
port every evening except Sunday.
In the year 1874 the whole of the interests of Frederick Kemp,
esq., J.P., of Bispham Lodge, in the Fleetwood and Belfast steam line
were acquired by the Lancashire and Yorkshire and London and
North Western Railway Companies, at that time owners of the larger
share, and now practically sole proprietors. Up to the date of this
transaction the vendor had been intimately and personally
associated with the traffic as managing-owner from its first
institution, in addition to which he was the chief promoter of the
Ardrossan and Isle of Man routes.
With the solitary exception of the service whose progress has just
been briefly traced out, there is perhaps no single branch of industry
which has assisted so ably in maintaining and stimulating such
prosperity as the town of Fleetwood has enjoyed, throughout its
chequered career, as the fishing traffic. In the earliest years of the
seaport, shortly before the Belfast steamer communication was
established, a second pilot boat, named the “Pursuit,” arrived in the
river from Cowes, but finding little occupation the crew provided
themselves with a trawl-net and turned their long periods of vigil to
profitable account by its use. This sensible plan of launching out into
another field of labour when opportunities of prosecuting their more
legitimate avocation failed them was not of long duration, probably
no more than a few months, for on the Irish line of steamships
commencing to ply the pilots secured berths as second officers, and
their boat was laid up. The “Pursuit” soon became a tender to a
government ship engaged in surveying; and about ten or twelve
months later was purchased by some gentlemen, denominated the
Fleetwood Fishing Company, and, together with four more boats,
hired from North Meols, Southport, sent out on fishing excursions. At
the end of one year the hired sloops were discharged, and five
similar craft bought by the company, thus making a fleet of six
smacks belonging to the place, connected with the trawling trade. In
the course of three or four years the whole of the boats were sold,
as the traffic had not proved so remunerative a venture as at first
anticipated; and one only remained in the harbour, being purchased
by Mr. Robert Roskell, of this place. Shortly afterwards a Scotch
smack arrived from Kirkcudbright, and in about twelve months the
two boats were joined by three or four from North Meols, owned for
the most part by a family named Leadbetter, which settled here.
Almost simultaneously another batch of fishing craft made its
appearance from the east coast and took up a permanent station at
Fleetwood. The success which attended the expeditions of the deep-
sea trawlers was not long in being rumoured abroad and attracting
others, who were anxious to participate in an undertaking capable of
producing such satisfactory results. Year by year the dimensions of
the originally small fleet were developed as new-comers appeared
upon the scene, and added their boats to those already actively
prosecuting the trade. To trace minutely each gradation in the
prosperous progress of this line of commerce would be wearisome to
the reader, and is in no way necessary to the object we have in view.
It will be sufficient for the purpose to state that in 1860 the number
of fishing smacks on the Fleetwood station amounted to thirty-two,
varying in tonnage from 25 to 50 tons each and built at an average
cost of £500 each, the lowest being £400 and the highest £1,000.
The following will illustrate the plan by which men in the humble
sphere of fishermen were enabled to become the proprietors of their
own craft: A shipmaster supplied the vessel on the understanding
that £100 was deposited at once, and the remainder paid by
quarterly instalments, no insurance being asked for or proffered
regarding risk. The arrangement entered into by the smack-owners
for the conveyance of fish to shore, when they were engaged out at
sea in their calling was most simple and business-like. The boats
kept company during fishing, and on a certain signal being given one
of the number, according to a previous agreement, received the
whole of the fish so far caught by her fellow craft and returned
home, for which service her men were paid 2s. each by the other
crews, who continued their occupation and arrived in harbour
generally on Friday. For the next week another smack was selected,
and thus all in turn performed the mid-week journey. At present
there are no less than eighty-four sloops belonging to this port,
pursuing the business of fishing, and the arrangements both for their
purchase and the landing of the captured fish have undergone a
revolution. All boats are now paid for when they leave the
shipbuilder’s yard, and the former custom of a mid-week relief, has
been relinquished, each sloop returning and discharging as occasion
requires. A fishing boat’s crew usually consists of four men and a