Nelson Pediatrics Review (MCQs) 19ed PDF
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Summary
This document appears to be a collection of multiple-choice questions (MCQs) and their explanations for a pediatrics review. The topics covered include foster care, vaginal bleeding in children, Munchausen syndrome by proxy, malnutrition, and reproductive technologies.
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Nelson Pediatrics Review(MCQs)19ed ﻭﻻ ﺘﻨﺴﻭﻨﺎ ﻤﻥ ﺼﺎﻟﺢ ﺍﻟﺩﻋﺎﺀ، ﺯﻫﻴﺭ ﺍﻟﺩﺠﺎﻨﻲ ﺒﺎﻟﺘﻭﻓﻴﻕ.ﻤﻊ ﺘﻤﻨﻴﺎﺕ ﺩ -------------------------------------------------------------- 1. Which of the following statements regarding foster care is true? □A per...
Nelson Pediatrics Review(MCQs)19ed ﻭﻻ ﺘﻨﺴﻭﻨﺎ ﻤﻥ ﺼﺎﻟﺢ ﺍﻟﺩﻋﺎﺀ، ﺯﻫﻴﺭ ﺍﻟﺩﺠﺎﻨﻲ ﺒﺎﻟﺘﻭﻓﻴﻕ.ﻤﻊ ﺘﻤﻨﻴﺎﺕ ﺩ -------------------------------------------------------------- 1. Which of the following statements regarding foster care is true? □A permanency plan must be made for a child in foster care no later than 12 mo from the child's entry into care □A minority of children in foster care have a history of abuse or neglect □The mission of foster care is to safely care for children while providing services to families to promote reunification □Most (>70%) of children in foster care are reunited with their families A and C description The mission of foster care is to provide for the health, safety, and well-being of children while assisting their families with services to promote reunification. Children entering foster care have frequently experienced early childhood trauma. More than 70% have a history of abuse, neglect, or both. Only about 50% of children achieve reunification. In the USA, the Adoption and Safe Families Act (P.L. 105-89) passed in 1997 requires that a permanency plan be made for each child no later than 12 mo after entry to foster care and that a petition to terminate parental rights typically must be filed when a child has been in foster care for at least 15 of the previous 22 mo. (See Chapter 35, page 134, and e35-1.) 2. A 4 yr old girl is admitted to the hospital for her third evaluation for vaginal bleeding. The mother noted bright red blood on the child's underwear. Previous examinations revealed a normal 4 yr old girl, Tanner stage 1, with normal external genitalia. Pelvic ultrasound results were normal, as was the serum estradiol level. The hemoglobin and platelet counts were normal, as were the bleeding time and coagulation studies. Findings on pelvic examination conducted under anesthesia also were normal. The next step in the examination is to: Determine the blood type of the blood on the underwear □Interrogate the father □Isolate the parents and child □Determine von Willebrand factor levels □Measure fibronectin in the vagina description Consideration of factitious disorder by proxy should be triggered when the reported symptoms are repeatedly noted by only one parent, appropriate testing fails to confirm a diagnosis, and seemingly appropriate treatment is ineffective. At times, the child's symptoms, their course, or the response to treatment may be incompatible with any recognized disease. Preverbal children are usually involved. Bleeding is a particularly common presentation. This may be caused by adding dyes to samples, adding blood (e.g., from the mother) to the child's sample, or giving the child an anticoagulant (e.g., warfarin). (See Chapter 37, page 146.) 3. Munchausen syndrome by proxy is characterized by all of the following EXCEPT: □Mother who appears devoted and wins over members of care team □Multiple hospitalizations and investigations without diagnosis □Symptoms on history but not witnessed by medical team Symptoms occurring in presence of different caregivers (e.g., while mother is out of town) □Use of medications or toxins description Symptoms in young children are mostly associated with proximity of the offending caregiver to the child. The mother may present as a devoted or even model parent who forms close relationships with members of the health care team. While appearing very interested in her child's condition, she may be relatively distant emotionally. (See Chapter 37, page 146.) 4. Which statement is false? Malnutrition is the second leading cause of acquired immune deficiency worldwide behind HIV infection □Zinc is important in immune function and linear growth □Kwashiorkor and marasmus are rare in developed countries □The Western diet is associated with increased noncommunicable disease description The significant global burden of malnutrition and undernutrition is the leading worldwide cause of acquired immunodeficiency and the major underlying factor for morbidity and mortality globally for children 40 years of age Long interpregnancy interval □Cigarette smoking description See Table 89-1 for Factors Associated with High-Risk Pregnancy. The lowest neonatal mortality rate occurs in infants of mothers who receive adequate prenatal care and who are 20-30 yr of age. Pregnancies in both teenagers and women older than 40 yr, particularly primiparous women, are at increased risk for intrauterine growth restriction, fetal distress, and intrauterine death. A short interpregnancy interval is associated with increased risk. (See e89-1.) 62. Reproductive technologies are associated with all of the following pregnancy-related risks EXCEPT: □Higher risk for multiple fetuses Higher risk for group B streptococcal sepsis □Higher risk for low and very low birthweight □Higher risk for prematurity □Higher risk for congenital anomalies description The use of assisted reproductive technology (in vitro fertilization, intracytoplasmic sperm injection) increases the risk of perinatal mortality, infant morbidity, prematurity, low and very low birthweight, and cerebral palsy, largely because of the increase in multiple-fetus pregnancies with such technology; the risks for birth defects are also increased, in part, because of epigenetic effects on gene expression. (See e89-1.) 63. Oligohydramnios is associated with all of the following EXCEPT: Esophageal atresia □Pulmonary hypoplasia □Potter syndrome □Posterior urethral valves □Skeletal contractures description Oligohydramnios is associated with congenital anomalies; intrauterine growth restriction; severe renal, bladder, or urethral anomalies; and drugs that interfere with fetal urination. Oligohydramnios becomes most evident after 20 wk of gestation, when fetal urination is the major source of amniotic fluid. Rupture of the membranes is the most common cause of oligohydramnios and must be ruled out if oligohydramnios is suspected, especially if a normal-sized bladder is seen on fetal ultrasound evaluation. Oligohydramnios causes fetal compression abnormalities such as fetal distress, clubfoot, spadelike hands, and a flattened nasal bridge. The most serious complication of chronic oligohydramnios is pulmonary hypoplasia. Polyhydramnios is associated with premature labor, abruptio placentae, multiple congenital anomalies, and fetal neuromuscular dysfunction or obstruction of the gastrointestinal tract that interferes with reabsorption of the amniotic fluid that is normally swallowed by the fetus. Increased fetal urination or edema formation is also associated with excessive amniotic fluid volume. (See Table 89-4 and e89-2.) 64. Amnion nodosum is associated with all of the following EXCEPT: □Oligohydramnios Fetal Candida infection □Renal agenesis □Pulmonary hypoplasia □Flattened facies description Examination of the fresh placenta, cord, and membranes may alert the physician to a newborn infant at high risk and may help confirm a diagnosis in a sick infant. Amnion nodosum (granules on the amnion) and oligohydramnios are associated with pulmonary hypoplasia and renal agenesis, whereas small whitish nodules on the cord suggest a candidal infection. (See Chapter 90, page 552.) 65. Which statement about twins is not true? Monovular twinning appears to be an inherited tendency □Most twins are born prematurely □Ninety percent of twins are detected before delivery □Monochorionic twins may be presumed to be monovular □The second born twin is at increased risk for respiratory distress syndrome and asphyxia compared with the first born description The occurrence of monovular twins appears to be independent of genetic influence. Polyovular pregnancies are more frequent beyond the 2nd pregnancy, in older women, and in families with a history of polyovular twins. (See Chapter 91, page 553.) 66. Fetal transfusion syndrome results in which of the following? □Donor twin large and plethoric □Recipient twin small and anemic □Donor twin small and anemic □Recipient twin large and plethoric □A and B C and D description In the fetal transfusion syndrome, an artery from one twin acutely or chronically delivers blood that is drained into the vein of the other. The latter becomes plethoric and large, and the former is anemic and small. Generally, with chronicity, 5 g/dL hemoglobin and 20% body weight differences can be noted in this syndrome. (See Table 91-2, Chapter 91, page 554.) 67. Very-low-birthweight (VLBW) infants are best described as: □Predominantly growth restricted Predominantly premature □Predominantly post dates □The result of maternal illness □The result of placental infarction description VLBW infants weigh 90% of those between 1250 and 1500 g surviving. The VLBW rate has remained unchanged for black Americans but has increased among whites, perhaps because of a rise in multiple births among whites. Perinatal care has improved the rate of survival of VLBW infants. When compared with term infants, VLBW neonates have a higher incidence of rehospitalization during the 1st yr of life for sequelae of prematurity, infections, neurologic complications, and psychosocial disorders. (See Chapter 91, page 556.) 68. Which of the following statements regarding the assessment of gestational age at birth is NOT true? □In the absence of asphyxia, neurologic maturity correlates with gestational age The Ballard scoring system is accurate to within 2 days □If a discrepancy exists between the estimation of gestational age by physical examination and fetal ultrasonographic evaluation, the infant is at high risk for morbidity and mortality □Physical features used to assess gestational age include breast buds, ear development, and presence of lanugo description Neurologic maturity (nerve conduction velocity), in the absence of asphyxia, correlates with gestational age despite reduced fetal weight. Physical signs may be useful in estimating gestational age at birth. Commonly used, the Ballard scoring system is accurate to ±2 wk. An infant should be presumed to be at high risk for mortality or morbidity if a discrepancy exists between the estimation of gestational age by physical examination, the mother's estimated date of her last menstrual period, and fetal ultrasonographic evaluation. (See Figure 91-6, Chapter 91, page 557.) 69. A term female is born by spontaneous vaginal delivery to a primiparous woman who received 2 doses of meperidine 30 min and 2 hr before an abrupt delivery. The baby is apneic and limp. The most important, immediate management is to: □Administer naloxone in the umbilical vein Perform bag-mask ventilation □Administer naloxone in the endotracheal tube □Begin chest compressions □Obtain a cord pH level description Narcosis results from administration of morphine, meperidine, fentanyl, barbiturates, or tranquilizers to the mother shortly before delivery or from maternal anesthesia given during the 2nd stage of labor. This sequela should be avoided by the use of appropriate analgesic and anesthetic practices. Treatment includes initial physical stimulation and securing of a patent airway. If effective ventilation is not initiated, artificial breathing with a bag and mask must be instituted. At the same time, if the respiratory depression is due to an opiate, naloxone hydrochloride (Narcan), 0.1 mg/kg, should be given intravenously or intramuscularly. Naloxone is contraindicated in infants born to mothers with opiate addiction because it precipitates acute neonatal withdrawal with severe seizures. (See Chapter 94, page 575.) 70. Successful ventilation is determined by all of the following EXCEPT: Zero reading of end-tidal CO2 measurement □Pink color □Rising heart rate □Symmetric breath sounds □Good chest rise description Successful ventilation is signified by adequate chest rise, symmetric breath sounds, improved pink color, heart rate > 100 beats/min, spontaneous respirations, presence of end-tidal CO2, and improved tone. (See Chapter 94, page 576.) 71. The biopsychosocial model of development, when applied to the child's height, includes all of the following EXCEPT: □Genetic endowment □Personal eating habits □Access to food □Parents' beliefs Differences between breast milk and formula description The biopsychosocial model of medicine recognizes biologic factors, social factors, and psychologic factors that contribute to health and disease. Genetics, environment, economics (cost/access), personal habits, and family beliefs all contribute in this model. (See e6-1.) 72. All of the following statements regarding a child's temperament are true EXCEPT: Temperament is absolute and stable throughout the life span □Biology influences temperament □It is a pattern of the child's responses □It is relatively resistant to parents' attempts to modify □It helps parents understand the child's behavior without guilt description Temperament describes the stable, early-appearing individual variations in behavioral dimensions, including emotionality (crying, laughing, sulking), activity level, attention, sociability, and persistence. Temperament has long been described as biologic or "inherited," largely based on parent reports of twins. We now know that genes are dynamic, changing in the quantity and quality of their effects as a child ages and thus, like environment, may continue to change. Longitudinal twin studies of adult personality indicate that personality changes largely result from non-shared environmental influences, whereas stability of temperament appears to result from genetic factors. The concept of temperament can help parents understand and accept the characteristics of their children without feeling responsible for having caused them. (see e6-1). 73. The parents of a 3 yr old girl report that "she ran before she walked," "she is never hungry at the same time," and "she goes from toy to toy." This child is best described as: □Autistic □Having a specific temperament □Having attention-deficit/hyperactivity disorder Having developmental pervasive disorder □Being deaf description The classic theory of Thomas and Chess proposes 9 dimensions of temperament (Table 6-1). These characteristics lead to 3 common constellations: (1) the easy, highly adaptable child, who has regular biologic cycles; (2) the difficult child, who withdraws from new stimuli and is easily frustrated; and (3) the slow-to-warm-up child, who needs extra time to adapt to new circumstances. Various combinations of these clusters also occur. (See e6-1, including Table 6-1.) 74. A term baby girl has 2 episodes of bile-stained emesis at 24 hr after birth. There is a history of excessive amniotic fluid volume. The most appropriate diagnostic test is: □Blood culture Barium upper gastrointestinal x-ray series with small bowel follow-through □Barium enema □CT scan □Head ultrasound study description Bile-stained emesis suggests intestinal obstruction beyond the duodenum but also may be idiopathic. Abdominal radiographs (kidney-ureter-bladder [KUB] and cross-table lateral views) should be performed in neonates with persistent emesis and in all infants with bile-stained emesis to detect air-fluid levels, distended bowel loops, characteristic patterns of obstruction (double bubble: duodenal atresia), and pneumoperitoneum (intestinal perforation). A contrast swallow radiograph with small bowel follow-through is indicated in the presence of bilious emesis. (See Chapter 96, page 600.) 75. Meconium plug is associated with all of the following EXCEPT: □Hypermagnesemia □Infants of diabetic mothers □Cystic fibrosis □Hirschsprung disease Prematurity description Meconium plugs are associated with small left colon syndrome in infants of diabetic mothers and with cystic fibrosis, rectal aganglionosis, maternal opiate use, and magnesium sulfate therapy for preeclampsia. (See Chapter 96, page 600.) 76. Which statement about hemolytic disease of the newborn is NOT true? □RhoGAM administration should be given to the mother within 72 hours of the delivery of an Rh-negative infant; it should also be given after abortion of amniocentesis □ABO incompatibility is the most common cause of hemolytic disease of the newborn Most cases of ABO incompatibility require transfusion □Rh and ABO incompatibility account for approximately 95% of cases of hemolytic disease of the newborn description Most cases of ABO incompatibility are mild, with jaundice being the only clinical manifestation. The infant is not generally affected at birth; pallor is not present, and hydrops fetalis is extremely rare. The liver and spleen are not greatly enlarged, if at all. Jaundice usually appears during the 1st 24 hr. Rarely, it may become severe, and symptoms and signs of kernicterus develop rapidly. Treatment of ABO incompatibility relies on phototherapy to lower serum bilirubin levels. In severe cases, IVIG administration can reduce the rate of hemolysis and the need for exchange transfusion. Exchange transfusions with type O blood of the same Rh type as the infant may be needed in some cases to correct dangerous degrees of anemia or hyperbilirubinemia. (See Chapter 97, page 619.) 77. The best approach to prevent congenital anomalies in infants of diabetic mothers is to: □Discontinue insulin and begin glyburide □Provide continuous insulin infusion during labor and delivery □Switch from an oral hypoglycemic agent to insulin until 36-wk of gestation □Do serial glucose tolerance tests Maintain periconceptional tight control of maternal blood glucose levels description Periconception glucose control reduces the risk of anomalies and other adverse outcomes, and glucose control during labor reduces the incidence of neonatal hypoglycemia. Women with type 1 diabetes who have tight glucose control during pregnancy (average daily glucose levels < 95 mg/dL) are delivered of infants with birthweights and anthropomorphic features similar to those of infants of nondiabetic mothers. (See Chapter 101, page 629.) 78. Which statement regarding adolescent development is NOT true? □In girls, the first sign of puberty is breast bud development □Adolescents experience asymmetric growth beginning with enlargement of the hands and feet, followed by the arms and legs, and finally, the trunk and chest □Some degree of breast hypertrophy, typically bilateral, occurs in ~50% of boys during Tanner stages 2-3 It is abnormal for early adolescents to be preoccupied with their body changes and feel that everyone is staring at them □Adolescents are able to make more complex decisions, but these decisions are highly susceptible to emotion description Self-consciousness increases exponentially in response to the somatic transformations of puberty. Self- awareness at this age centers on external characteristics, in contrast to the introspection of later adolescence. It is normal for early adolescents to be preoccupied with their body changes, scrutinize their appearance, and feel that everyone else is staring at them. (See Chapter 104, page 651.) 79. Papanicolaou smears are indicated in: □Women aged 16 and older □Women aged 21 and older All women beginning 3 years after onset of sexual activity or age 21 years, whichever is earlier □All sexually active teenage girls description For sexually active females, the guidelines for Pap smears for cervical cancer screening suggest that annual screening can be delayed safely up to 3 yr after the onset of sexual activity or age 21 yr, whichever is earlier; HPV DNA testing should not be used routinely for females 29 yr and younger since the results would not influence management. (See Chapter 106, page 667.) 80. All of the following may cause gynecomastia EXCEPT: □Klinefelter syndrome □Phenothiazines □Normal puberty □Heroin Albuterol □Anabolic steroids description Pubertal gynecomastia, occurring in up to 60% of normal adolescent males, has long been attributed to a transient imbalance of estrogen and androgen concentrations. With typical onset between chronological ages of 10 and 13 yr, it usually regresses over several months Nonpubertal gynecomastia with hypogonadism is associated with Klinefelter syndrome and places a patient at a higher risk of breast cancer. Other conditions associated with nonpubertal gynecomastia are secondary to endocrine disorders, neoplasms, chronic disease, trauma, and medications as well as drugs of abuse. See Table 109-1, Drugs causing Gynecomastia. (See e109-1.) 81. Common presenting complaints of pregnancy include all of the following EXCEPT: □Fatigue □Breast tenderness □Nausea □Irregular menses Dysuria description Adolescents may experience the traditional symptoms of pregnancy: morning sickness (vomiting, nausea that may also occur any time of the day), swollen tender breasts, weight gain, and amenorrhea. Often the presentation is less classic. Headache, fatigue, abdominal pain, dizziness, and scanty or irregular menses are common presenting complaints. (See Chapter 112, page 700.) 82. Teenage pregnancy carries increased risk of all of the following EXCEPT: □Prematurity □Low birth weight infant □Gastroschisis Multiple birth (twins) □Maternal eclampsia description As expected, teen mothers have low rates of age-related chronic disease (diabetes or hypertension) that might affect the outcomes of a pregnancy. They also have lower rates of twin pregnancies than older women. They tolerate childbirth well with few operative interventions. However, as compared with 20-39 yr old mothers, teens have higher incidences of low birthweight infants, preterm infants, neonatal deaths, passage of moderate to heavy fetal meconium during parturition, and infant deaths within 1 yr after birth. The highest rates of these poor outcomes occur in the youngest and most economically deprived mothers. Gastroschisis, although very rare, has a markedly higher incidence in infants of teen mothers for reasons that are not yet clear. Teen mothers also have higher rates of anemia, pregnancy-associated hypertension, and eclampsia, with the youngest teens having rates of pregnancy-associated hypertension 40% higher than the rates of women in their 20s and 30s. The youngest teens also have a higher incidence of poor weight gain (5,000 cells/μL) eosinophilia (see Table 123-1). Allergy is the most common cause of eosinophilia in children in the USA. Eosinophilia is often associated with infection with multicellular helminthic parasites, which are the most common cause in developing countries. Severe eosinophilia in children is most commonly due to visceral larva migrans. The level of eosinophilia tends to parallel the magnitude and extent of tissue invasion, especially by larvae. Eosinophilia often does not occur in established parasitic infections that are well contained within tissues or are solely intraluminal in the gastrointestinal tract, such as Giardia lamblia and Enterobius vermicularis infection. Eosinophilia is observed in many patients with primary immunodeficiency syndromes, especially hyper-IgE syndrome, Wiskott-Aldrich syndrome, and Omenn syndrome. (See Chapter 123, page 740.) 87. A 2 yr old boy refuses to hold his mother's hand when walking across the parking lot of a shopping mall. He attempts to run away from her, but she quickly grabs his hand before he darts out in front of a car. She is exasperated and scared by his actions. Of the following, which technique is most effective in addressing the behavior? □Verbal reprimand □Spanking □Scolding Time-out □Slapping his hand description Discipline should be immediate, specific to the behavior, and time limited. Time-out for approximately 1 min/yr of age is very effective. A kitchen timer allows the parent to step back from the situation; the child is free when the timer rings. (See Chapter 10, page 36.) 88. To be effective, time-outs should: □Be accompanied by verbal reprimands □Be accompanied by punishment □Last at least 5-10 min Last 1 min/yr of age □Be done at a point in time removed from the incident description Discipline should be immediate, specific to the behavior, and time limited. Time-out for approximately 1 min/yr of age is very effective. A kitchen timer allows the parent to step back from the situation; the child is free when the timer rings. (See Chapter 10 page 36.) 89. Handedness is usually determined by age: □2-4 mo □6-12 mo □15-18 mo □20-24 mo 36-48 mo description Handedness is usually established by the 3rd yr. Frustration may result from attempts to change children's hand preference. Variations in fine motor development reflect both individual proclivities and different opportunities for learning. Children who are seldom allowed to use crayons, for example, develop a mature pencil grasp later. (See Chapter 10, page 33.) 90. The best approach for parents to help a preschool child overcome monster fears is to: □Rationalize that monsters do not exist □Read books that do not have monsters in them □Have the pediatrician explain that monsters are make-believe Use "great power" like monster spray to keep monsters away □None of the above description The active imagination that fuels play and the magical, animist thinking characteristic of preoperational cognition can also generate intense fears. More than 80% of parents report at least 1 fear in their preschool children. Refusal to take baths or to sit on the toilet may arise from the fear of being washed or flushed away, reflecting a child's immature appreciation of relative size. Attempts to demonstrate rationally that there are no monsters in the closet often fail, inasmuch as the fear arises from prerational thinking. However, this same thinking allows parents to be endowed with magical powers that can banish the monsters with "monster spray" or a night light. Parents should acknowledge the fears, offer reassurance and a sense of security, and give the child some sense of control over the situation. (See Chapter 10, page 35.) 91. Between 2 and 5 yr of age, language increases; as a rule, the number of words in a sentence is: □Based on knowledge of numbers Equal to the age of the child in years □Independent of the environment □Independent of the number of questions asked the child by adults □Based on the ABCs description Language development occurs most rapidly between 2 and 5 yr of age. Vocabulary increases from 50-100 words to more than 2,000. Sentence structure advances from telegraphic phrases ("Baby cry") to sentences incorporating all of the major grammatical components. As a rule of thumb, between the ages of 2 and 5 yr, the number of words in a typical sentence equals the child's age (2 by age 2 yr, 3 by age 3 yr, and so on). By 21 mo to 2 yr, most children are using possessives ("My ball"), progressives (the "-ing" construction, as in "I playing"), questions, and negatives. (See Chapter 10, page 35.) 92. All of the following statements regarding language development are true EXCEPT: □Deaf children may create their own language □The basics for language may be "hard-wired" in the brain Environment and experience have minimal impact on the rate of language development □Delayed language may signify deafness □Delayed language may signify mental retardation description It is important to distinguish between speech (the production of intelligible sounds) and language, which refers to the underlying mental act. Language includes both expressive and receptive functions. Receptive language (understanding) varies less in its rate of acquisition than does expressive language; therefore, it has greater prognostic importance. (See Chapters 14 and 32.) Language acquisition depends critically on environmental input. Key determinants include the amount and variety of speech directed toward children and the frequency with which adults ask questions and encourage verbalization. Children raised in poverty typically perform lower on measures of language development compared with children from economically advantaged families. Although experience influences the rate of language development, many linguists believe that the basic mechanism for language learning is "hard-wired" in the brain. Children do not simply imitate adult speech; they abstract the complex rules of grammar from the ambient language, generating implicit hypotheses. Evidence for the existence of such implicit rules comes from analysis of grammatical errors, such as the overgeneralized use of "-s" to signify the plural and "-ed" to signify the past ("We seed lots of mouses."). Language is linked to both cognitive and emotional development. Language delays may be the first indication that a child has mental retardation, has an autism spectrum disorder, or has been maltreated. Language plays a critical part in the regulation of behavior through internalized "private speech" in which a child repeats adult prohibitions, first audibly and then mentally. Language also allows children to express feelings, such as anger or frustration, without acting them out; consequently, language-delayed children show higher rates of tantrums and other externalizing behaviors. (See Chapter 10, page 35.) 93. Repeated meningococcal infections suggest which of the following types of immune disorder? □B-cell defect □T-cell defect □Combined B- and T-cell defect □Phagocyte function defect Complement component deficiency description A defect of complement function should be considered in any patient with recurrent pyogenic infections, disseminated meningococcal or gonococcal infection, or a second episode of bacteremia at any age. A previously well adolescent or young adult with meningococcal meningitis due to an uncommon serotype (not A, B, or C) should undergo screening for a late-component or alternative pathway deficiency with CH50 and AP50 assays. (See Chapter 128, page 753.) 94. A complement defect should be suspected in which disorder? □Recurrent angioedema □Chronic nephritis □Recurrent hemolytic-uremic syndrome □A and B All of the above description A defect of complement function should be considered in any patient with recurrent angioedema, autoimmune disease, chronic nephritis, hemolytic-uremic syndrome (HUS), or partial lipodystrophy, or with recurrent pyogenic infections, disseminated meningococcal or gonococcal infection, or a second episode of bacteremia at any age. A previously well adolescent or young adult with meningococcal meningitis due to an uncommon serotype (not A, B, or C) should undergo screening for a late-component or alternative pathway deficiency with CH50 and AP50 assays. (See Chapter 128, page 753.) 95. Signs of allergy on physical examination include all of the following EXCEPT: □Dennie lines (Dennie-Morgan folds) □Purple discolorations beneath the lower eyelids Erythematous nasal mucosa with thick yellow secretions □Xerosis □Keratoconus description Parents of allergic children are often concerned about blue-gray to purple discolorations beneath the lower eyelids, attributed to venous stasis and referred to as allergic shiners. They are found in up to 60% of allergic patients and almost 40% of patients without allergic disease. They are often accompanied by Dennie lines (Dennie-Morgan folds), which are prominent symmetric skin folds that extend in an arc from the inner canthus beneath and parallel to the lower lid margin. The nasal mucosa in allergic rhinitis is classically described as pale to purple in comparison with the beefy red mucosa of patients with nonallergic rhinitis. Allergic nasal secretions are typically thin and clear. Purulent secretions suggest another cause of rhinitis. Keratoconus, or protrusion of the cornea, may occur in patients with atopic dermatitis as a result of repeated trauma produced by persistent rubbing of the eyes. (See Chapter 135, page 765.) 96. A 7 yr old boy with asthma has roughness over the extensor surfaces of the upper arms and thighs, which is caused by keratin plugs lodged in the openings of hair follicles. This physical finding is termed: Keratosis pilaris □Fibroepitheliosis □Hidradenitis □Xerosis □Acrochordon description Keratosis pilaris, often found on the extensor surfaces of the upper arms and thighs, is characterized by roughness of the skin caused by keratin plugs lodged in the openings of hair follicles. Examination of the skin of the palms and feet reveals exaggerated palmar and plantar creases in some allergic children. (See Chapter 135, page 765.) 97. The radioallergosorbent test (RAST) and related as-IgE assay determine: □Bronchial reactivity to subcutaneous serotonin □Bronchial reactivity after inhalation bronchial provocation test □The proportion of total allergic immunoglobulin Antigen-specific serum IgE concentrations □The overall allergic risk profile based on absolute eosinophil count, total IgE, and skin test results description The presence of IgE specific for a particular allergen can be documented in vivo by skin testing or in vitro by the measurement of allergen-specific IgE (as-IgE) levels in the serum. The first test for documenting the presence of as-IgE was called the radioallergosorbent test (RAST) because it used a radiolabeled anti-IgE antibody. The RAST has been replaced by an improved generation of as-IgE assays that use enzyme-conjugated rather than radiolabeled anti-IgE. These assays use solid-phase supports to which allergens of an individual allergen extract are bound. A small amount of the patient's serum is incubated with the allergen-coated support, resulting in binding of the patient's as-IgE to the allergens on the support. Next, the allergen-coated support to which the patient's as-IgE is bound is incubated with enzyme conjugated antihuman-IgE that then binds to the patient's as-IgE. Incubation of this complex with a fluorescent substrate of the conjugated enzyme results in the generation of fluorescence that is proportional to the amount of as-IgE in the serum sample. (See Chapter 135, page 766.) 98. All of the following statements regarding skin testing for allergic reactivity are true EXCEPT: □Antihistamines given prior to testing may inhibit the reaction □Intradermal tests are more sensitive than puncture tests Positive skin test results by intradermal testing correlate better than results by puncture tests with clinical symptoms □The reaction peaks within approximately 20 min and usually resolves over 20-30 min □Larger reactions have greater clinical relevance description Skin testing in children is usually first performed using the prick/puncture technique. With this technique, a small drop of allergen is applied to the skin surface and a tiny amount is introduced into the epidermis by lightly pricking or puncturing the skin through the drop of extract with a small needle. When the prick/puncture skin test result is negative and the history is suggestive, selective skin testing using the intradermal technique may be performed. This technique involves using a 26-gauge needle to inject 0.01-0.02 mL of a dilute allergen extract into the dermis of the arm. This technique is more sensitive than the prick/puncture technique, and the allergen extracts used are 1,000- to 100-fold less concentrated than extracts used for prick/puncture testing. Intradermal skin tests are not recommended for use with food allergens because of the risk of triggering anaphylaxis. Irritant rather than allergic reactions can occur with intradermal skin testing if higher concentrations of extracts, such as 1:100 weight:volume, are used. Although prick/puncture testing is less sensitive than intradermal skin testing, positive prick/puncture skin test results tend to correlate better with symptoms on natural exposure to the allergen. (See Chapter 135, page 767.) 99. Which of the following is an advantage of skin testing over serum assays such as RAST to determine specific IgE? □Skin testing is not affected by administration of antihistamines Skin testing has greater sensitivity than RAST □Skin testing is semiquantitative □Skin testing is associated with less risk of allergic reaction □Skin testing is not confounded by dermographism description The primary advantages of the serum assays (as-IgE and RAST) in comparison with allergen skin testing are their safety and that the results are not influenced by skin disease or medications. Overall, the results of these tests correlate well with those obtained by skin testing and provocation challenges. The as-IgE assays are not as sensitive as the skin test. In patients with histories of life-threatening reactions to foods, insect stings, drugs, or latex, skin testing is still required because of its higher sensitivity even if the as-IgE assay result is negative. (See Chapter 135, page 767.) 100. Features characteristically associated with atopic dermatitis include all of the following EXCEPT: □Allergic rhinitis or asthma □Elevated serum IgE level □Peripheral blood eosinophilia Lymphopenia description Infants with atopic dermatitis (AD) are predisposed to development of allergic rhinitis and/or asthma later in childhood, a process called "the atopic march." Two forms of atopic dermatitis have been identified. Atopic eczema is associated with IgE-mediated sensitization (at onset or during the course of eczema) and occurs in 70-80% of patients with AD. Nonatopic eczema is not associated with IgE-mediated sensitization and is seen in 20-30% of patients with AD. Both forms of AD are associated with eosinophilia. (See Chapter 139, page 801-802.) 101. Major features of atopic dermatitis in children include all of the following EXCEPT: □Pruritus □Facial and extensor eczema Angioedema □Chronic or relapsing course □Personal or family history of atopic disease description Atopic dermatitis (AD) is diagnosed on the basis of 3 major features: pruritus, an eczematous dermatitis that fits into a typical presentation, and a chronic or chronically relapsing course. Associated features, such as a family history of asthma, hay fever, elevated IgE, and immediate skin test reactivity, are variably present. Acute AD skin lesions are intensely pruritic with erythematous papules. Subacute dermatitis manifests as erythematous, excoriated, scaling papules. In contrast, chronic AD is characterized by lichenification, or thickening of the skin with accentuated surface markings, and fibrotic papules (prurigo nodularis). In chronic AD, all three types of skin reactions may coexist in the same individual. AD is generally more acute in infancy and involves the face, scalp, and extensor surfaces of the extremities. The diaper area is usually spared. Older children and children with chronic AD have lichenification and localization of the rash to the flexural folds of the extremities. AD often goes into remission as the patient grows older, leaving an adolescent or adult with skin prone to itching and inflammation when exposed to exogenous irritants. (See Chapter 139, pages 802-803.) 102. A 5 yr old boy with severe atopic dermatitis develops illness with dozens of vesicles primarily covering areas of skin previously affected by atopic dermatitis. The distribution crosses many dermatomes. Findings include fever and lymphadenopathy. The most likely diagnosis is: □Chickenpox □Zoster Eczema herpeticum □Eczema vaccinatum □Coxsackievirus infection description AD is associated with recurrent viral skin infections. The most serious viral infection is Kaposi varicelliform eruption, or eczema herpeticum, which is caused by HSV and affects patients of all ages. The incubation period of 5-12 days is followed by disseminated eruption of multiple, itchy, vesiculopustular lesions. The vesicular lesions are umbilicated, tend to crop, and often become hemorrhagic and crusted. (See Chapter 139, page 806.) 103. Which of the following is not a complication of atopic dermatitis? □Recurrent Staphylococcus aureus cellulitis □Eczema herpeticum □Cataracts □Increased susceptibility to ringworm Erythema nodosum description Patients with AD have increased susceptibility to bacterial, viral, and fungal skin infections. Antistaphylococcal antibiotics are very helpful for treating patients who are heavily colonized or infected with Staphylococcus aureus. S. aureus is found in > 90% of AD skin lesions. Antibiotic therapy is required if honey-colored crusting, folliculitis, impetigo, and pyoderma are present. AD is associated with recurrent viral skin infections. The most serious viral infection is Kaposi varicelliform eruption, or eczema herpeticum, which is caused by HSV and affects patients of all ages. Exfoliative dermatitis may develop in patients with extensive skin involvement. It is associated with generalized redness, scaling, weeping, crusting, systemic toxicity, lymphadenopathy, and fever and is usually caused by superinfection (e.g., with toxin-producing S. aureus or HSV infection) or inappropriate therapy. In some cases, the withdrawal of systemic glucocorticoids used to control severe AD precipitates exfoliative erythroderma. Cataracts may be a primary manifestation of AD or from extensive use of systemic and topical glucocorticoids, particularly around the eyes. (See Chapter 139, page 806.) 104. A 2 yr old is diagnosed with atopic dermatitis. Which of the following environmental modifications is recommended? □A bland diet, especially minimizing meats □Installation of wool carpeting instead of synthetic carpeting Use of a liquid rather than powder laundry detergent and adding a 2nd rinse cycle □Use of soaps that are especially effective in removing fatty substances □Bathing less often than daily description The treatment of atopic dermatitis (AD) requires a systematic, multifaceted approach that incorporates skin hydration, topical anti-inflammatory therapy, identification and elimination of flare factors, and, if necessary, systemic therapy. Lukewarm soaking baths for 15-20 min followed by the application of an occlusive emollient to retain moisture provide symptomatic relief. Hydrophilic ointments of varying degrees of viscosity can be used according to the patient's preference. Patients with AD have a low threshold response to irritants that trigger their itch-scratch cycle. Soaps or detergents, chemicals, smoke, abrasive clothing, and exposure to extremes of temperature and humidity are common triggers. Patients with AD should use soaps with minimal defatting properties and a neutral pH. New clothing should be laundered before wearing to decrease levels of formaldehyde and other chemicals. Residual laundry detergent in clothing may trigger the itch-scratch cycle; using a liquid rather than powder detergent and adding a 2nd rinse cycle facilitates removal of the detergent. Extensive elimination diets, which can be nutritionally deficient, are rarely required. Avoidance measures for dust mite–allergic patients include removing bedroom carpeting. (See Chapter 139, pages 804 and 806.) 105. The most common single cause of anaphylaxis outside of the hospital is: □Insect sting allergy □Drug allergy Food allergy □Latex allergy □Food-associated exercise-induced anaphylaxis description The most common causes of anaphylaxis in children are different for hospital and community settings. Anaphylaxis occurring in the hospital results primarily from allergic reactions to medications and latex. Food allergy is the most common cause of anaphylaxis occurring outside the hospital, accounting for about one half of the anaphylactic reactions reported in pediatric surveys from the USA, Italy, and South Australia. (See Chapter 143, page 816.) 106. A 12 yr old child with a history of allergy to yellow jackets is stung and immediately begins experiencing tightness in the chest and wheezing. The drug of first choice for management of this child is: □Inhaled albuterol □Subcutaneous epinephrine □Intramuscular diphenhydramine Intramuscular epinephrine □Oral corticosteroids description Anaphylaxis is a medical emergency requiring aggressive management with intramuscular or intravenous epinephrine, intramuscular or intravenous H1 and H2 antihistamine antagonists, oxygen, intravenous fluids, inhaled β- agonists, and corticosteroids. The initial assessment should ensure an adequate airway with effective respiration, circulation, and perfusion. Epinephrine is the most important medication, and there should be no delay in its administration. If an intravenous line is not available, epinephrine should be given by the intramuscular route (0.01 mg/kg; max 0.3-0.5 mg.) For children ≥ 12 yr, many recommend the 0.5-mg intramuscular dose. The intramuscular dose can be repeated 2 or 3 times at intervals of 5 to 15 min if an intravenous continuous epinephrine infusion has not yet been started and symptoms persist. Other drugs (antihistamines, glucocorticosteroids) have a secondary role in the management of anaphylaxis. (See Chapter 143, page 817.) 107. The mother of an 8-yr-old boy with acute streptococcal tonsillitis calls to report that now, within 15 min after the first dose of oral penicillin V that you prescribed, he is complaining of itching and has developed hives. Which of the following should you recommend? ` □A dose of oral Benadryl, with instructions to call again if he has not improved within 30 min Immediate return to your office or the nearest emergency department □Careful monitoring at home, with instructions to return to your office or the nearest emergency department if he becomes short of breath or loses consciousness □Schedule a visit for a laboratory test to determine serum trypticase level □Substitution of erythromycin for penicillin description In anaphylaxis, the onset of symptoms may vary somewhat depending on the cause of the reaction. Reactions from ingested allergens (foods, medications) are delayed in onset (minutes to 2 hr) compared with those from injected allergens (insect sting, medications) and tend to have more gastrointestinal symptoms. Initial symptoms vary with the etiology and may include any of the following constellation of symptoms: pruritus about the mouth and face; a sensation of warmth, weakness, and apprehension (sense of doom); flushing, urticaria and angioedema, oral or cutaneous pruritus, tightness in the throat, dry staccato cough and hoarseness, periocular pruritus, nasal congestion, sneezing, dyspnea, deep cough and wheezing; nausea, abdominal cramping, and vomiting, especially with ingested allergens; uterine contractions (manifesting as lower back pain; not uncommon); and faintness and loss of consciousness in severe cases. Some degree of obstructive laryngeal edema is typically encountered with severe reactions. Cutaneous symptoms may be absent in up to 20% of cases, and the acute onset of severe bronchospasm in a previously well asthmatic person should suggest the diagnosis of anaphylaxis. (See Chapter 143, page 816.) 108. A child is diagnosed with latex allergy in the hospital. He should be counseled that which of the following foods are likely to cause an allergic reaction due to homologous proteins? □Bananas □Kiwi □Avocado □Passion fruit □Chestnut All of the above description Patients with latex allergy may also experience food-allergic reactions from homologous proteins in foods such as bananas, kiwi, avocado, chestnut, and passion fruit. (See Chapter 143, page 816.) 109. Administration of which of the following drugs is the treatment of choice for anaphylaxis? □Diphenhydramine orally □Diphenhydramine by intravenous infusion □Aqueous epinephrine (1:1,000) by subcutaneous injection Aqueous epinephrine (1:1,000) by intramuscular injection □Aqueous epinephrine (1:1,000) by intravenous infusion description Anaphylaxis is a medical emergency. Epinephrine is the most important medication, and there should be no delay in its administration. If an intravenous line is not available, epinephrine should be given by the intramuscular route (0.01 mg/kg; max 0.3-0.5 mg). For children ≥ 12 yr, many recommend the 0.5-mg intramuscular dose. The intramuscular dose can be repeated 2 or 3 times at intervals of 5 to 15 min if an intravenous continuous epinephrine infusion has not yet been started and symptoms persist. Other drugs (antihistamines, glucocorticosteroids) have a secondary role in the management of anaphylaxis. (See Chapter 143, page 817.) 110. Common symptoms of rheumatologic disease include all of the following EXCEPT: □Fever Headache □Rash □Joint pain □Fatigue description There are no classic symptoms of a rheumatic disease, but common symptoms include joint pain, fever, fatigue, and rash. Presenting signs and symptoms help direct the evaluation and limit unnecessary testing. (See e147-1.) 111. Arthritis may be present in which disease? □Systemic lupus erythematosis □Acute rheumatic fever □Kawasaki disease □Parvovirus B-19 All of the above description Arthritis is defined by the presence of intra-articular swelling or 2 or more of the following findings on joint examination: pain on motion, loss of motion, erythema, and heat. Arthritis is present in all of the chronic childhood arthritis syndromes, along with SLE, juvenile dermatomyositis, vasculitis, Behçet disease, sarcoidosis, Kawasaki disease, and Henoch-Schönlein purpura. Nonrheumatic causes of arthritis include malignancy, infections such as septic arthritis, Lyme disease, osteomyelitis, viral infections (e.g., rubella, hepatitis B, parvovirus B19), and postinfectious causes such as Epstein- Barr virus, acute rheumatic fever, and reactive arthritis. (See e147-2.) 112. Which disease is incorrectly matched with the characteristic rash? □Systemic lupus erythematosis—malar rash sparing nasolabial folds □Discoid lupus—hyperkeratotic rash around the ears Kawasaki disease—vesicular rash □Juvenile dermatomyositis (JDM)—Gottron papules and heliotrope rash □Henoch-Schönlein purpura—palpable purpura on extensor surfaces of lower extremities description Presence of a photosensitive malar rash that spares the nasiolabial folds is suggestive of SLE, especially in an adolescent girl. Diffuse facial rash is more indicative of JDM. A hyperkeratotic rash on the face or around the ears of an adolescent African-American girl may represent discoid lupus. A palpable purpuric rash on the extensor surfaces of the lower extremities points to Henoch-Schönlein purpura. Less localized purpuric rashes and petechiae are present in systemic vasculitis or blood dyscrasias including coagulopathies. Nonblanching erythematous papules on the palms are seen in vasculitis and SLE. Gottron papules and heliotrope rashes along with erythematous rashes on the elbows and knees are pathognomonic of JDM. Dilated capillary loops in the nail beds (periungual telangectasias) are common in JDM, scleroderma, and secondary Raynaud phenomenon. An evanescent macular rash associated with fever is part of the diagnostic criteria for systemic onset arthritis. Sun sensitivity or photosensitive rashes are indicative of SLE or JDM but also can be caused by antibiotics. Kawasaki disease may have many presentations of rash, but vesicles point to another diagnosis. (See e147-1 and Table 147-2.) 113. Organisms associated with reactive arthritis include: □Shigella □Chlamydia trachomatis □Yersinia enterocolitica □Campylobacter jejuni All of the above description Reactive arthritis typically follows enteric infection with Salmonella, Shigella, Yersinia enterocolitica, Campylobacter jejuni, Cryptosporidium parvum, or Giardia intestinalis or genitourinary tract infection with Chlamydia trachomatis or Ureaplasma. (See Chapter 151, page 839.) 114. An 18 yr old boy has a swollen right wrist and left ankle with bilateral pain over both Achilles tendons. He was treated 2 wk ago with an antibiotic for a urethral discharge; his girlfriend also was treated. Physical examination reveals tenderness over both Achilles tendons, swollen painful joints (right wrist and left ankle), and limited forward bending at the waist. The most appropriate therapeutic agent would be: □Ceftriaxone □Doxycycline □Prednisone A nonsteroidal anti-inflammatory agent □Intravenous immunoglobulin description Specific treatment is unnecessary for most cases of reactive or postinfectious arthritis. Nonsteroidal anti- inflammatory agents are often needed for management of pain and functional limitation. Unless ongoing Chlamydia infection is suspected, attempts to treat the offending organism are not warranted. (See Chapter 151, page 841.) 115. Viruses associated with arthritis include all of the following EXCEPT: □Rubella vaccine Respiratory syncytial virus □Parvovirus □Hepatitis B □Cytomegalovirus description Familiarity with other causes of postinfectious arthritis is vital when a diagnosis of reactive arthritis is being considered. Numerous viruses are associated with postinfectious arthritis and may result in particular patterns of joint involvement. Rubella and hepatitis B virus typically affect the small joints, whereas mumps and varicella often involve large joints, especially the knees. The hepatitis B arthritis–dermatitis syndrome is characterized by urticarial rash and a symmetric migratory polyarthritis resembling that of serum sickness. Rubella-associated arthropathy may follow natural rubella infection and, infrequently, rubella immunization. It typically occurs in young women, with an increased frequency with advancing age, and is uncommon in preadolescent children and in males. Arthralgia of the knees and hands usually begins within 7 days of onset of the rash or 10-28 days after immunization. Parvovirus B19, which is responsible for erythema infectiosum (fifth disease), can cause arthralgia, symmetric joint swelling, and morning stiffness, particularly in adult women and less frequently in children. Arthritis occurs occasionally during cytomegalovirus infection and may occur during varicella infections but is rare after Epstein-Barr virus infection. Varicella also may be complicated by suppurative arthritis, usually secondary to group A streptococcus infection. HIV is associated with an arthritis that resembles psoriatic arthritis more than juvenile idiopathic arthritis (JIA). See Table 151-1, Viruses Associated with Arthritis. (See Chapter 151, page 840.) 116. On exposure to cold, a 14 yr old girl characteristically develops pallor, then cyanosis, and finally erythema of the fingers and toes. She is at increased risk of developing: □Systemic-onset juvenile rheumatoid arthritis Scleroderma □Leukemia □Histiocytosis □Diabetes mellitus description Raynaud phenomenon (RP) is the most frequent initial symptom in pediatric systemic sclerosis, present in 70% of affected children months to years before other manifestations. Raynaud phenomenon refers to the classic triphasic sequence of blanching, cyanosis, and erythema of the digits induced by cold exposure and/or emotional stress. Raynaud phenomenon is most commonly independent of an underlying rheumatic disease (Raynaud disease), but it can be a consequence of other diseases as well as scleroderma, such as systemic lupus erythematosus and mixed connective tissue disease (Table 154-2). (See Chapter 154, page 852.) 117. All of the following diseases are known to be complicated by reactive amyloidosis (AA type) EXCEPT: □Familial Mediterranean fever □Juvenile idiopathic arthritis Henoch-Schnlein purpura □Inflammatory bowel disease □Ankylosing spondylitis description Amyloidosis comprises a group of diseases characterized by extracellular deposition of insoluble, fibrous amyloid proteins in various body tissues. Amyloidosis in patients with the hereditary periodic fever syndromes (including familial Mediterranean fever [FMF], tumor necrosis factor receptor–associated periodic syndrome [TRAPS], CAPS, and hyperimmunoglobulinemia D syndrome [HIDS]), other autoinflammatory disorders, chronic infection, and chronic inflammatory diseases involving amyloid A protein is referred to as AA amyloidosis (formerly referred to as secondary or reactive amyloidosis). AA amyloidosis is the most common serious complication of FMF and TRAPS. Amyloid conditions associated with aging (Alzheimer disease) as well as several rare autosomal dominant forms of amyloidoses have fibril protein composed of variants of the transport protein transthyretin (TTR) and are now referred to as TTR amyloidosis. Only AA amyloidosis affects children in appreciable numbers, occurring in some individuals with hereditary periodic fever syndromes, chronic inflammatory diseases (e.g., juvenile idiopathic arthritis, ankylosing spondylitis, and inflammatory bowel disease), chronic infections (e.g., chronic osteomyelitis and tuberculosis), cystic fibrosis, and, less commonly, systemic lupus erythematosus and juvenile dermatomyositis. The factors that determine the risk for amyloidosis as a complication of inflammation are not clear, because many individuals with long-standing inflammatory disease do not demonstrate tissue amyloid deposition. (See e158-1.) 118. Which of the following is NOT true regarding polyarteritis nodosa (PAN)? □Boys and girls are equally affected □Biopsies reveal necrotizing vasculitis in a segmental pattern Large vessels are primarily involved □PAN often presents after an acute infection, and may be related to a postinfectious autoimmune response in susceptible individuals □Group A streptococcus is associated with the development of PAN description Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis affecting small and medium-sized arteries. Aneurysms and stenoses form at irregular intervals throughout affected arteries. Cutaneous PAN is limited to the skin. PAN is rare in childhood. Boys and girls are equally affected, and the mean age at presentation is 9 yr. The cause is unknown, but the development of PAN following infections, including group A streptococcus and chronic hepatitis B, suggests that PAN represents a postinfectious autoimmune response. Infections with other organisms, including Epstein-Barr virus, Mycobacterium tuberculosis, cytomegalovirus, parvovirus B19, and hepatitis C virus, also have been associated with PAN. Biopsies show necrotizing vasculitis with granulocytes and monocytes infiltrating the walls of small and medium-sized arteries. Involvement is usually segmental and tends to occur at vessel bifurcations. Granulomatous inflammation is not present, and deposition of complement and immune complexes is rarely observed. (See Chapter 161, page 872.) 119. A 3 yr old girl has fever. Urine is collected by catheterization for culture. What is the minimum concentration of bacterial growth in the culture that would generally be considered diagnostic of a urinary tract infection in this young girl? □1 bacterium/mL □10 bacteria/mL 103 bacteria/mL □105 bacteria/mL □None of the above description Urine for culture and colony count can be obtained by collecting clean-voided midstream specimens, by catheterization, or by suprapubic aspiration. Urine samples collected by placing bags on the perineum are unacceptable for culture because samples are often contaminated, rendering the results uninterpretable. Rapid transport of urine to the laboratory is imperative because gram-negative enteric pathogens have generation times of 20-30 min and any delay in transport or plating renders colony counts unreliable. Refrigeration may be used when delay is unavoidable. Culture systems using media-coated paddles permit prompt inoculation of the specimen, but confluent growth may be difficult to detect; moreover, accurate antibiotic susceptibility testing requires the presence of individual discrete colonies. Urine obtained by 3 suprapubic puncture is normally sterile. Urine collected by catheterization is likely to reflect infection if there are ≥ 10 5 organisms/mL. Clean-voided urine is considered abnormal if ≥ 10 organisms/mL are present and possibly abnormal if 4 5 10 -10 organisms/mL are present. However, lower counts are sometimes found in urinary tract infections in adolescent girls and young women, especially those with bacterial urethritis, or in patients with fungal infections. A Gram stain of 5 unspun urine with ≥ 1 bacterium per oil immersion field correlates well with the presence of ≥ 10 organisms/mL. (See e164-2.) 120. Which of the following defines the concept of bacterial tolerance? □MIC in vivo > 4 times MIC in vitro □MBC in vivo > MIC in vivo □MIC in vivo > 2 times MIC in vitro □MIC > 4 times MBC MBC > 4 times MIC description The other widely used technique for susceptibility testing is dilution testing. A standard concentration of a microorganism is inoculated into serially diluted concentrations of antibiotic, and the minimum inhibitory concentration (MIC) in μg/mL, the lowest concentration of antibiotic required to inhibit growth of the microorganism, is determined. Dilution testing also permits determination of the minimum bactericidal concentration (MBC), the lowest concentration of antibiotic required to kill the organism. The MBC is sometimes determined to exclude the possibility of bacterial tolerance (MBC > 4 times the MIC). (See e164-3.) 121. A 14 yr old boy in Connecticut has an illness characterized by fever and an erythematous rash. An initial Borrelia burgdorferi IgG antibody titer was 1:32. Repeat antibody titer 5 wk later is 1:64. The most likely interpretation of these serologic tests is: □Acute Lyme disease □Atypical Lyme disease □Relapsing disease □Syphilis None of the above description To confirm a new infection using IgG testing, it is essential to demonstrate either seroconversion or a rising IgG titer. A 4-fold increase in a convalescent titer obtained 2-3 wk after the acute titer is considered diagnostic in most situations. Serologic diagnosis of Lyme disease remains problematic because of lack of specificity of the commercial enzyme immunoassays. A confirmatory immunoblot (Western blot) is required for all positive and equivocal EIA results for Lyme disease. For early Lyme disease, both IgG and IgM antibody positivity (“bands”) are required for confirmation, whereas for late disease, only an IgG immunoblot assay should be performed, because false-positive reactions with IgM immunoblot assays are common. Ordering serologic tests for children with nonspecific complaints such as arthralgia or fatigue is unnecessary and creates needless alarm, because invariably these represent false-positive reactions. (See e164- 4.) 122. Amoxicillin was begun 4 days ago for treatment of otitis media in a 4 yr old boy. He now 3 presents with signs of meningitis and is found to have a pleocytosis with 126 leukocytes/mm. The diagnostic stain of the cerebrospinal fluid that would be most useful is: Gram stain □Methenamine silver stain □Kinyoun stain □Trichrome stain □Wright stain description In many cases the Gram stain can provide very rapid and useful results, such as in the examination of cerebrospinal fluid (CSF). The Gram stain is an insensitive technique, requiring 104-105 microorganisms per milliliter for detection. A trained observer may be able to reach a tentative conclusion that there are specific microorganisms in the specimen based on their morphology and staining properties (gram-positive cocci in clusters are likely to be staphylococci), but such preliminary interpretations should be made cautiously and must be confirmed by culture. (See e164-1.) 123. All of the following advice for parents of children traveling internationally is true EXCEPT: □Parents should schedule an appointment to discuss their child's travel with the pediatrician at least 4 weeks prior to travel □Insect spray should contain no more than 40% DEET □Malaria and yellow fever are caused by night-biting mosquitos whereas dengue is caused by day-biting mosquitos □Water should be boiled for > 1 min before use for drinking, washing dishes, or brushing teeth Clothes should be re-treated with permethrin after every wash description Parents of traveling children should seek medical consultation ≥ 4-6 wk before departure to obtain a realistic assessment of health risks, a schedule of vaccinations and list of medications, and instructions on dealing with disease during travel. Tap water should not be used when brushing teeth. Boiling water for ≥ 1 min (or 3 min at altitudes > 2000 m) is the most reliable method of disinfecting water. Malaria, yellow fever, Japanese encephalitis, and filariasis are typically caused by night-biting mosquitoes, whereas dengue is usually caused by day-biting mosquitoes. Rare instances of toxic encephalopathy have been reported in young children with exposure to high concentrations of DEET, but use of repellent with no more than 40% DEET and avoidance of repeated applications as well as avoiding application to mucous membranes minimize the risk of this complication. Spraying clothing with permethrin, a synthetic pyrethroid, is a safe and effective method of reducing insect bites in children. Permethrin-sprayed clothes remain effective for ≥ 2 wk, even with laundering. (See e168-1.) 124. Which of the following is not a valid reason to start a psychotropic medication? Child requests a medicine to feel better □A patient has not responded to psychosocial intervention □Patient's aggressive behavior is a threat to safety of others □Anxiety symptoms are interfering with school performance description To ensure safe and appropriate use of psychotropic medications, prescribers should follow best practice principles that underlie medication prescribing. Cognitive, emotional, and/or behavior symptoms are targets for medication intervention when there is no response to available evidence-based psychosocial interventions, there is a significant risk of harm, and/or there is significant functional impairment. Commonly encountered target symptom domains include agitation, anxiety, depression, hyperactivity, inattention, impulsivity, mania, and psychosis. (See Chapter 19, page 61.) 125. Neuroleptic antipsychotic agents produce all of the following unwanted side effects EXCEPT: □Weight gain □Leukopenia □Tardive dyskinesia Inappropriate secretion of ADH □Hyperthermia description The atypical antipsychotics have relatively strong antagonistic interactions with 5-HT2 receptors and perhaps more variable activity at central adrenergic, cholinergic, and histaminic sites, which might account for varying side effects noted among these agents. The atypical antipsychotics have significant side effects, including extrapyramidal symptoms (e.g., restlessness and dyskinesias), weight gain, metabolic syndrome, diabetes, hyperprolactinemia, hematologic adverse effects (e.g., leukopenia or neutropenia), seizures, hepatotoxicity, neuroleptic malignant syndrome, and cardiovascular effects. For all atypical antipsychotics, body mass index, blood pressure, fasting blood glucose, fasting lipid profiles, and abnormal movements should be closely monitored. If there is a family or personal history suggestive of cardiac disease, electrocardiograms should also be monitored. Haloperidol is a high-potency butyrophenone that is the typical antipsychotic most commonly used. This medication is useful in psychosis, Tourette disorder, and severe agitation. Side effects include anticholinergic effects, weight gain, drowsiness, and extrapyramidal symptoms (dystonia, rigidity, tremor, and akathisia.) There is a risk of tardive dyskinesia with chronic administration. (See Chapter 19, page 62.) 126. Which clinical situation does not pose a high risk for adverse medication effects? □Teenager on venlafaxine for anxiety is prescribed linezolid for MRSA pustules Patient with Wolff-Parkinson-White syndrome is prescribed fluoxetine for depression □Teenager with history of seizures is prescribed bupropion for smoking cessation and depressive symptoms □A patient with heart failure is prescribed trazodone for difficulty sleeping □A bipolar patient on lithium undergoes kidney transplant with subsequent cyclosporine treatment description There are special considerations in the use of psychotropic medications with physically ill children. Significant interactions are organized by organ system in Chapter 19, pages 64-65. Notable considerations include the following: (1) Drug-drug interactions that can cause serotonin syndrome include linezolid (an antibiotic that has MAOI properties) and antimigraine preparations used with an SSRI, as well as combinations of SSRI, trazodone, buspirone, and venlafaxine. (2) Patients with Wolff-Parkinson-White syndrome who have a short PR interval ( 50% present within the 1st month of life, with symptoms of acute or chronic obstruction. Vomiting is the most common symptom in this age group. Infants often present in the 1st week of life with bilious emesis and acute bowel obstruction. Older infants present with episodes of recurrent abdominal pain that can mimic colic and suggest intermittent volvulus. Malrotation in older children can manifest with recurrent episodes of vomiting and/or abdominal pain. Patients occasionally present with malabsorption or protein-losing enteropathy associated with bacterial overgrowth. Symptoms are caused by intermittent volvulus or duodenal compression by Ladd bands or other adhesive bands affecting the small and large bowel. Volvulus occurs when the small bowel twists around the superior mesenteric artery leading to vascular compromise of the bowel. The diagnosis may be suggested by ultrasound but is confirmed by contrast radiographic studies. The abdominal plain film is usually nonspecific but might demonstrate a gasless abdomen or evidence of duodenal obstruction with a "double bubble" sign. Barium enema usually demonstrates malposition of the cecum but is normal in up to 20% of patients. Upper gastrointestinal series is the imaging test of choice and the gold standard in the evaluation and diagnosis of malrotation and volvulus. It is the best exam to visualize the malposition of the ligament of Treitz and can also reveal a corkscrew appearance of the small bowel or a duodenal obstruction with a "bird's beak" appearance of the duodenum. Ultrasonography demonstrates inversion of the superior mesenteric artery and vein. A superior mesenteric vein located to the left of the superior mesenteric artery suggests malrotation. Malrotation with volvulus is suggested by duodenal obstruction, thickened bowel loops to the right of the spine, and free peritoneal fluid. (See Chapter 322, page 1281.) 203. A term neonate develops bilious emesis without associated abdominal distention on the 1st day of life. A plain film demonstrates a "double bubble" sign. After nasogastric decompression and IV hydration, the next step in management is: □Surgical repair of the anomaly □Echocardiogram □Renal ultrasound □Radiographs of the chest and spine □Blood culture B, C, and D description The hallmark of duodenal obstruction is bilious vomiting without abdominal distention, which is usually noted on the 1st day of life. Peristaltic waves may be visualized early in the disease process. A history of polyhydramnios is present in half the pregnancies and is caused by inadequate absorption of amniotic fluid in the distal intestine. This fluid may be bile stained due to intrauterine vomiting. Jaundice is present in one third of the infants. The diagnosis is suggested by the presence of a "double bubble" sign on a plain abdominal radiograph. The initial treatment of infants with duodenal atresia includes nasogastric or orogastric decompression and intravenous fluid replacement. Echocardiography, renal ultrasound, and radiology of the chest and spine should be performed to evaluate for associated anomalies. Definitive correction of the atresia is usually postponed until life-threatening anomalies are evaluated and treated. (See Chapter 322, page 1278.) 204. A 4 yr old child was playing with loose pocket change and swallowed a coin. Radiographs show a penny-sized coin in the stomach. The appropriate clinical management is: Continue a normal diet, and monitor stool for passage of the coin □Perform endoscopy to retrieve the coin □Admit to the hospital and monitor with serial radiographs for passage of the coin □Prescribe a puree diet to aid the passage of the coin and monitor stool for appearance of coin □Prescribe cathartics to aid the passage of the coin, monitor stool description Most patients who ingest foreign bodies are between the ages of 6 mo and 6 yr. Coins are the most commonly ingested foreign body in children. Conservative management is indicated for most foreign bodies that have passed through the esophagus and entered the stomach. Most objects pass though the intestine in 4-6 days, although some take as long as 3-4 wk. While waiting for the object to pass, parents are instructed to continue a regular diet and to observe the stools for the appearance of the ingested object. Cathartics should be avoided. Exceptionally long or sharp objects are usually monitored radiologically. Parents or patients should be instructed to report abdominal pain, vomiting, persistent fever, and hematemesis or melena immediately to their physicians. Failure of the object to progress within 3-4 wk seldom implies an impending perforation but may be associated with a congenital malformation or acquired bowel abnormality. (See Chapter 326, page 1290.) 205. An 18 mo old white boy manifests failure to thrive, poor appetite, abdominal distention, diarrhea, and irritability. He had been well until 9 mo of age. Thereafter, he was weaned from breast milk to regular foods. His growth curve is flattening. The most likely diagnosis is: □Giardiasis Celiac disease □Cystic fibrosis □Pancreatitis □Mitochondrial myopathy description Celiac disease is a common disorder (1% prevalence of biopsy-proven disease). It is thought to be rare in Central Africa and East Asia. Environmental factors might affect the risk of developing celiac disease or the timing of its presentation. Prolonged breast-feeding has been associated with a reduced incidence of symptomatic disease. Less clear is the effect of the time of gluten introduction in the infant diet; the ingestion of increased amounts of gluten in the 1st year of life can increase the incidence. Clinical features of celiac disease vary considerably. Intestinal symptoms are common in children whose disease is diagnosed within the 1st 2 years of life; failure to thrive, chronic diarrhea, vomiting, abdominal distention, muscle wasting, anorexia, and irritability are present in most cases. (See Chapter 330, page 1309.) 206. Extraintestinal manifestations of celiac disease include: □Anemia □Osteoporosis □Short stature □Alopecia All of the above description The most common extraintestinal manifestation of celiac disease is iron-deficiency anemia unresponsive to iron therapy. Osteoporosis may be present; in contrast to the situation in adults, it can be reversed by a gluten-free diet, with restoration of normal peak bone densitometric values. Other extraintestinal manifestations include short stature, endocrinopathies, arthritis and arthralgia, epilepsy with bilateral occipital calcifications, peripheral neuropathies, cardiomyopathy, chronic lung disease, isolated hypertransaminasemia, dental enamel hypoplasia, aphthous stomatitis, and alopecia. The mechanisms responsible for the severity and the variety of clinical presentations remain obscure. Nutritional deficiencies or abnormal immune responses have been advocated. (See Chapter 330, page 1309.) 207. Which co-morbid disease has a higher than normal incidence in patients with celiac disease? □Diabetes mellitus type 1 □Autoimmune cholangitis □Thyroid disease □IgA nephropathy All of the above description Some diseases, many with an autoimmune pathogenesis, are found with a higher than normal incidence in celiac patients. Among these are type 1 diabetes, autoimmune thyroid disease, Addison disease, Sjögren syndrome, autoimmune cholangitis, autoimmune hepatitis, primary biliary cirrhosis, IgA nephropathy, alopecia, and dilated cardiomyopathy. Such associations have been interpreted as a consequence of the sharing of identical HLA haplotypes. The relation between celiac disease and other autoimmune diseases is poorly defined; once those diseases are established, they are not influenced by a gluten-free diet. Other associated conditions include selective IgA deficiency, Down syndrome, Turner syndrome, and Williams syndrome. (See Chapter 330, page 1309.) 208. A 5 yr old in your practice underwent screening for celiac disease because his mother was recently diagnosed with this condition. His screening test is positive, however he is asymptomatic. Intestinal biopsy is negative. How would you classify this patient, and what are your recommendations to the mother? The child has potential celiac disease and should be monitored for the development of future symptoms; a gluten-free diet is not mandatory □The child does not have celiac disease and requires no further testing or dietary intervention □The child has latent celiac disease and should start a gluten-free diet □The child has celiac disease and should undergo testing for associated autoimmune diseases □The child has silent celiac disease and should initiate gluten-free diet description Potential celiac disease is defined when patients are identified by positive screening studies but without documented celiac disease on small bowel biopsy. It is important to follow these patients because they can develop established celiac disease in the future (see Table 330-6). There is a consensus that all celiac disease patients should be treated with a gluten-free diet regardless of the presence of symptoms. However, whereas it is relatively easy to assess the health improvement after treatment of celiac disease in patients with clinical symptoms of the disease, it proves difficult in persons with asymptomatic celiac disease. The nutritional risks, particularly osteopenia, are those mainly feared for subjects who have silent celiac disease and continue on a gluten-containing diet. Little is known about the health risks in untreated patients with minor enteropathy, which may be clinically silent. There are no guidelines concerning the need for a gluten-free diet in subjects with "potential" celiac disease (patients with positive celiac disease–associated serology but without enteropathy). (See Chapter 330, pages 1309-1311.) 209. An 11 yr old girl, Tanner stage 2, experiences intermittent periumbilical abdominal pain 2 days before an emergency visit to her pediatrician. Six hours later, she is nauseated and has one or two episodes of emesis. She also has had 2 soft bowel movements without blood or relief of symptoms. She walks cautiously into your office and lies still on your examining table. When you begin your examination, she is apprehensive and watches every move of your examining hand. You notice guarding and tenderness throughout her abdomen; the most tender area is the right lower quadrant. The most likely diagnosis is: □Pelvic inflammatory disease □Ruptured ectopic pregnancy □Crohn disease Appendicitis □Mesenteric adenitis description Whereas the classic presentation of acute appendicitis is well described, this represents less than half the cases; therefore, most cases of appendicitis have an "atypical" presentation. The illness typically begins insidiously with generalized malaise and anorexia; the child does not appear ill and the family is not likely to seek consultation assuming the child has "stomach flu" or a viral syndrome. Unfortunately, if the diagnosis is appendicitis, the illness escalates rapidly with abdominal pain followed by vomiting; appendiceal perforation is likely to occur within 48 hr of the onset of illness, and the opportunity for diagnosis before perforation is generally brief. Abdominal pain is consistently the primary and often the 1st symptom and begins shortly (hours) after the onset of illness. The pain is initially vague, unrelated to activity or position, often colicky, and periumbilical in location as a result of visceral inflammation from a distended appendix. Progression of the inflammatory process in the next 12-24 hr leads to involvement of the adjacent parietal peritoneal surfaces, resulting in somatic pain localized to the right lower quadrant. The pain becomes steady and more severe and is exacerbated by movement. The child often describes marked discomfort with the "bumpy" car ride to the hospital, moves cautiously, and has difficulty getting onto the examining room stretcher. Nausea and vomiting occur in more than half the patients and usually follow the onset of abdominal pain by several hours. Anorexia is a classic and consistent finding in acute appendicitis, but, occasionally, affected patients are hungry. Diarrhea and urinary symptoms are also common, particularly in cases of perforated appendicitis when there is likely inflammation near the rectum and possible abscess in the pelvis. Because enteric infections can cause appendicitis, diarrhea may be the initial manifestation and gastroenteritis may be the assumed diagnosis. In contrast to gastroenteritis, the abdominal pain in appendicitis is constant (not cramping or relieved by defecation), the emesis may become bile stained and persistent, and the clinical course worsens rather than improves over time. Fever is typically low grade unless perforation has occurred. Most patients demonstrate at least mild tachycardia. (Chapter 335, pages 1349-1350.) 210. A 4 yr old boy has had vomiting and diarrhea for 4 days. He now has had 24 hr of abdominal pain that appears maximal in the right lower abdomen. His temperature is 38.4°C; WBC count, 3 9,400/mm with 75 PMNs and 20 lymphocytes; hematocrit, 36; and platelet count, 3 160,000/mm. Urinalysis is normal, with the exception of 3+ ketonuria. He has moderate tenderness in the right lower quadrants, with muscular guarding. The most likely diagnosis is: □Intussusception □Viral gastroenteritis Appendicitis □Bacterial enteritis □Henoch-Schönlein purpura description Because enteric infections can cause appendicitis, diarrhea may be the initial manifestation and gastroenteritis may be the assumed diagnosis. In contrast to gastroenteritis, the abdominal pain in appendicitis is constant (not cramping or relieved by defecation), the emesis may become bile stained and persistent, and the clinical course worsens rather than improves over time. Fever is typically low grade unless perforation has occurred. Localized abdominal tenderness is the single most reliable finding in the diagnosis of acute appendicitis. A consistent finding in acute appendicitis is rigidity of the overlying rectus muscle. This rigidity may be voluntary, to protect the area of tenderness from the examiner's hand, or involuntary, secondary to peritonitis causing spasm of the overlying muscle. Physical examination findings must be interpreted relative to the temporal evolution of the illness. (See Chapter 335, page 1350.) 211. The most common developmental abnormality of the exocrine pancreas is: □Cystic fibrosis Pancreas divisum □Annular pancreas □Pancreatic pseudocyst □Choledochal cyst description Pancreas divisum, which occurs in 5-15% of the population, is the most common pancreatic developmental anomaly. As the result of failure of the dorsal and ventral pancreatic anlagen to fuse, the tail, body, and part of the head of the pancreas drain through the small accessory duct of Santorini rather than the main duct of Wirsung. This anomaly may be associated with recurrent pancreatitis if there is relative obstruction of the outflow of the ventral pancreas. (See e339-3.) 212. A 10 yr old boy develops pancreatitis after a blow to the abdomen. During a 5-day hospitalization he is treated with intravenous fluids and analgesics and is discharged home on a low-fat diet. Four weeks later he has recurrence of abdominal pain and vomiting. The most likely diagnosis is: □Functional abdominal pain □Duodenal ulcer due to Helicobacter pylori □Chronic pancreatitis □Hemorrhagic pancreatitis Pancreatic pseudocyst description A pancreatic pseudocyst is suggested when an episode of pancreatitis fails to resolve or when a mass develops after an episode of pancreatitis. Clinical features usually include pain, nausea, and vomiting, but many patients are asymptomatic. The most common signs are a palpable mass in 50% of patients and jaundice in 10%. Other findings include ascites and pleural effusions (usually left sided). It is recommended that the patient with acute pancreatitis undergo an ultrasonographic evaluation 2-4 wk after resolution of the acute episode for an evaluation of possible pseudocyst formation. (See Chapter 344, page 1373.) 213. Which statement regarding liver abscess is NOT true? □Amebic abscess is rare in the USA and is associated with travel from endemic areas □Symptoms include fever, nausea, and right upper quadrant pain Patients with hepatic abscess usually are jaundiced □Solitary liver abscesses in the right lobe of the liver are most common □Treatment requires drainage (percutaneous vs surgical) description Signs and symptoms of liver abscess are nonspecific and can include fever, chills, night sweats, malaise, fatigue, nausea, abdominal pain with right upper quadrant tenderness, and hepatomegaly; jaundice is uncommon. Diagnosis can be challenging and is often delayed; a high index of suspicion is necessary in children with risk factors. Serum aminotransferase and more often the alkaline phosphatase levels are elevated. The erythrocyte sedimentation rate is high, and leukocytosis is common. The results of blood cultures are positive in 50% of patients. Chest radiographs might show elevation of the right hemidiaphragm with decreased mobility or a right pleural effusion. Ultrasound or CT can confirm the diagnosis. Solitary liver abscesses (70% of cases) in the right lobe of the liver (75% of cases) are more common than multiple abscesses or solitary left lobe abscesses. Amebic disease is rare in the USA and is associated with immigrants from or travel to highly endemic areas. Treatment requires percutaneous ultrasound- or CT-guided needle aspiration and less often open surgical drainage, particularly if multiple or large abscesses are present. (See Chapter 351, page 1404.) 214. Hydrops of the gallbladder is associated with what disease? □Group A streptococcal pharyngitis □Kawasaki disease □Total parental nutrition dependence □Mesenteric adenitis All of the above description Acute noncalculous, noninflammatory distention of the gallbladder can occur in infants and children. It is defined by the absence of calculi, bacterial infection, or congenital anomalies of the biliary system. The disorder can complicate acute infections, but the cause is often not identified. Hydrops of the gallbladder can also develop in patients receiving long-term parenteral nutrition, presumably as a result of gallbladder stasis during the period of enteral fasting. Affected patients usually have right upper quadrant (RUQ) pain with a palpable mass. Fever, vomiting, and jaundice may be present and are usually associated with a systemic illness such as streptococcal infection. See Table 358-1, Conditions Associated with Hydrops of the Gallbladder. (See e358-1.) 215. A 4 yr old boy in good health presents with a 3-day history of a tender mass, 2 cm in diameter, midway between the umbilicus and the xiphoid process. He is afebrile, is eating normally, and has an otherwise normal physical examination. The most likely diagnosis is: □Metastatic neuroblastoma □Rhabdomyosarcoma of the abdominal wall Incarcerated epigastric hernia □Lipoma □Infected dermoid cyst description Epigastric hernias are ventral hernias in the midline of the abdominal wall between the xiphoid and the umbilicus. Epigastric hernias result from defects in the decussating fibers of the linea alba and are more likely congenital than acquired. Most epigastric hernias are small and asymptomatic; therefore, the true incidence is unknown, but the reported incidence in childhood varies from < 1% to as high as 5%. Epigastric hernias may be single or multiple and are 2-3 times more common in males than females. The defect typically contains only preperitoneal fat without a peritoneal sac or abdominal viscera. The mass may be tender to examination, but strangulation of the hernia contents is uncommon. Physical examination is almost always diagnostic and imaging studies are unnecessary. Epigastric hernias do not resolve spontaneously, and therefore operative repair is the recommended treatment. (See Chapter 364, page 1418 and e364-1.) 216. What is the most common congenital abnormality of the nose? □Arhinia □Nasal hypoplasia Choanal atresia □Perforated septum □Pyriform aperture stenosis description Choanal atresia is the most common congenital anomaly of the nose and has a frequency of 1/7,000 live births. It consists of a unilateral or bilateral bony (90%) or membranous (10%) septum between the nose and the pharynx; most cases are a combination of bony and membranous atresia. About 50-70% of affected infants have other congenital anomalies, with the anomalies occurring more often in bilateral cases. (See Chapter 368, page 1430.) 217. A parent of a 9 mo old complains that her child is "always" sick with a cold. She wonders if her child needs antibiotics to clear the infection. You review the child's chart and note normal growth and development and several phone calls for cold management. The most appropriate response is: □Otherwise healthy young children may have as many as 12 colds per year □Withdrawing the child from daycare may decrease the number of colds □Antibiotics are not effective in the treatment of cold viruses □Over-the-counter (OTC) cold remedies are not effective in decreasing symptoms in infants All of the above description Young children have an average of 6-8 colds per year, but 10-15% of children have at least 12 infections per year. The incidence of illness decreases with age, with 2 to 3 illnesses per year by adulthood. The incidence of infection is primarily a function of exposure to the virus. Children in out-of-home daycare centers during the 1st year of life have 50% more colds than children cared for only at home. The difference in the incidence of illness between these groups of children decreases as the length of time spent in daycare increases, although the incidence of illness remains higher in the daycare group through at least the 1st 3 yr of life. Studies have shown that OTC cough and cold products are ineffective in treating symptoms of children < 6 yr of age. (See Chapter 371, pages 1434-1435.) 218. All of the following statements are true EXCEPT: □Most episodes of acute pharyngotonsillitis are viral Rapid enlargement of one tonsil is typical of pharyngotonsillitis □With cryptic tonsillitis, a frequent clinical presentation is halitosis, chronic sore throat, or a history of expelling foul-tasting and foul-smelling cheesy lumps □In many children, the diagnosis of airway obstruction is made by history and physical examination □Tonsillectomy alone is usually performed for recurrent or chronic pharyngotonsillitis description Rapid enlargement of one tonsil is highly suggestive of a tonsillar malignancy, typically lymphoma in children. (See Chapter 375, page 1443.) 219. Which of the following is not an indication for adenoidectomy alone? □Chronic nasal infection (chronic adenoiditis) □Chronic sinus infections that have failed medical management □Recurrent bouts of acute otitis media □Recurrent otorrhea in children with tympanostomy tubes Recurrent pharyngotonsillitis description Adenoidectomy alone may be indicated for the treatment of chronic nasal infection (chronic adenoiditis), chronic sinus infections that have failed medical management, and recurrent bouts of acute otitis media, including those in children with tympanostomy tubes who suffer from recurrent otorrhea. Adenoidectomy may be helpful in children with chronic or recurrent otitis media with effusion. Adenoidectomy alone may be curative in the management of patients with nasal obstruction, chronic mouth breathing, and loud snoring suggesting sleep-disordered breathing. Adenoidectomy may also be indicated for children in whom upper airway obstruction is suspected of causing craniofacial or occlusive developmental abnormalities. (See Chapter 375, page 1444.) 220. Complications of tonsillectomy and adenoidectomy include all of the following EXCEPT? □Postoperative bleeding Immunologic defect with propensity for sinus infections □Velopharyngeal insufficiency □Dehydration □Acute airway obstruction description The risks and potential benefits of surgery must be considered (Table 375-1). Bleeding can occur in the immediate postoperative period or be delayed after separation of the eschar. Bleeding is more common after high-dose dexamethasone (0.5 mg/kg), although postoperative nausea and emesis is reduced. The risk of bleeding is lower with lower-dose dexamethasone (0.15 mg/kg), which also has a lowered risk of postoperative nausea and emesis. Swelling of the tongue and soft palate can lead to acute airway obstruction in the 1st few hours after surgery. Children with underlying hypotonia or craniofacial anomalies are at greater risk for suffering this complication. Dehydration from odynophagia is not uncommon in the 1st postoperative week. Rare complications include velopharyngeal insuffici