2017 WISE with Rationale Flashcards

Question

``` "A 16-year old female had frequent irritable moods & insomnia for the past 3 weeks. She recently transferred from a provincial high school to a large private school for girls in Manila. She missed her friends from the province & cried frequently. She eventually lost interest in making friends & school activities, & got low grades. She hardly ate & lost weight. She said she can disappear in this world because no one will notice her absence. What is the most probable diagnosis? A. Major depressive disorder B. Social anxiety disorder C. Severe adjustment disorder D. Panic disorder" ```

Answer 1

A "Nelsons 21st p218 Table 39.1 DSM-5 diagnostic criteria for major depressive episode A. Five or more of the following symptoms have been present during the same 2wk period and represent a change from previous functioning; at least 1 of the symptoms is either depressed mood or loss of interest or pleasure 1. Depressed mood most of the day, nearly every day 2. Markedly diminished interest or pleasure in all, or almost all activities most of the day, nearly every day 3. Significant weight loss when not dieting or weight gain, or decrease or increase in appetite nearly every day 4. Insomnia or hypersomnia nearly every day 5. Psychomotor agitation or retardation nearly every day 6. Fatigue or loss of energy nearly every day 7. Feelings of worthlessness or excessive or inappropriate guilt nearly every day 8. Diminished ability to think or concentrate, or indecisiveness, nearly every day 9. Recurrent thoughts of death, recurrent suicidal ideation without a specific plan, or a suicide attempt or a specific plan for committing suicide. B. The symptoms cause clinically significant distress or impairment in social, emotional, or other important areas of functioning C. The episode is not attributable to the physiologic effects of a substance or to another medical condition D. The occurence of a major depressive episode is not better explained by schizoaffective disorder, schizophrenia, schizophreniform disorder, delusional disorder, or other specified and other unspecified schizophrenia spectrum and other psychotic disorders E. There has never been a manic or hypomanic episode. DSM 5 p286-287 Adjustment disorder A. The development of emotional or behavioral symptoms in response to an identifiable stressor occuring within 3 months of the onset of the stressor B. The symptoms or behaviors are clinically significant C. The stress-related disturbance does not meet the criteria for another mental disorder and is not merely an exacerbation of a preexisting disorder D. The symptoms do not represent normal bereavement E. Once the stressor or its consequences have terminated, the symptoms do not persist for more than an additional 6 months Specify whether: 1. With depressed mood: Low mood, tearfulness, feelings of hopelessness are predominant 2. With anxiety: Nervousness, worry, jitteriness, or separation anxiety is predominant 3. With mixed anxiety and depressed mood: A combination of depression and anxiety is preodminant 4. With disturbance of conduct 5. With mixed disturbance of emotions and conduct: Both emotional symptoms (depression, anxiety), and a disturbance in conduct are predominant 6. Unspecified: For maladaptive reactions that are not classifiable as one of the specific subtypes of adjustment disorder DSM 5 p288 If an individual has symptoms that meet criteria for a major depressive disorder in response to a stressor, the diagnosis of an adjustment disorder is not applicable. The symptom profile of major depressive disorder differentiates it from adjustment disorders. "

Answer 2

C "Nelson 21st p1014 In males the first visible sign of puberty and the hallmark of SMR 2 is testicular enlargement, beginning as early as 9.5yr, followed by the development of pubic hair. This is followed by penile growth during SMR 3. Peak growth occurs when testes volumes reach approximately 9-10cm3 during SMR 4. In females, typically the first visible sign of puberty and the hallmark of SMR 2 is the appearance of breast buds (thelarche) between 7 and 12 yr of age. A significant minority of females develops pubic hair (pubarche) prior to thelarche. Menses typically begin within 3 yr of thelarche during SMR 3-4 (average age 12.5yr, normal range 9-15yr). Females attain a peak height velocity of 8-9cm/yr at SMR 2-3, approximately 6mo before menarche. Males typically begin their growth acceleration at a later SMR stage, achieve a peak height velocity of 9-10cm later in the course of puberty (SMR 3-4), and continue their linear growth for approximately 2-3yr after females have stopped growing."

Answer 3

B "Nelson 21st p1015 Table 132.3 Sexual maturity rating stages in males SMR 1 - pubic hair: none - penis: preadolescent - testes: preadolescent SMR 2 - pubic hair: scant, long, slightly pigmented - penis: minimal change/enlargement - testes: enlarged scrotum, pink, texture altered SMR 3 - pubic hair: darker, starting to curl, small amount - penis: lengthens - testes: larger SMR 4 - pubic hair: resembles adult type byt less quantity, coarse, curly - penis: larger, glans and breadth increase in size - testes: larger, scrotum dark SMR 5 - pubic hair: adult distribution, spread to medial surface of thighs - penis: adult size - testes: adult size "

Answer 4

C "Choice A: Late adolescence Choice B: Middle adolesence Choice D: Middle adolescence Nelson 21st p1015 Table 132.1 Milestones in early, middle, and late adolescent development Early adolescence (10-13 yr) Cognitive and moral - concrete operations - egocentricity - unable to percieve long-term outcome of current decisions - follow rules to avoid punishment Self-concept/identity formation - preoccupied with changing body - self-consciousness about appearance and attractiveeness Family - increased need for privacy - exploration of boundaries of dependence vs independence Peers - same-sex peer affiliations Sexual - increased interest in sexual anatomy - anxieties and questions about pubertal changes - limited capacity for intimacy "

Answer 5

A "Nelson 21st p1075 Although pregnant teens are at a higher-than-average risk for some complications of pregnancy, most teenagers have pregnancies that are without major complications, delivering healthy infants. Teen mothers have low rates of age-related chronic disease (diabetes or hypertension) that may affect the outcomes of pregnancy. They also have lower rates of twin pregnancies than older women. They tolerate childbirth well with few operative interventions. However, compared with 20-39 year 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 one year after birth. The higher rates of poor outcomes occur in the youngest and most economically disadvantaged mothers. Gastroschisis, although rare, has a much higher incidence in infants of teen mothers, for reason that are unclear. Teen mothers also have higher rates of anemia, pregnancy-associated hypertension, and eclampsia. The youngest teens also have a higher incidence of poor weight gain during their pregnancy. This correlates with a decrease in birthweights of their infants. Poor maternal weight gain also has correlated strongly with teens' late entrance into prenatal care and with inadequate utilization of prenatal care. Sexually active teens have higher rates of STIs than older sexually active women. "

Answer 6

C "Nelson 21st p620 Inhalation injury should be sustpected in a patient confined to a closed space, with a history of an explosion or decreased level of consciousness, or with evidence of carbon deposits in the oropharynx or nose, singed facial hair, and carbonaceous sputum Evaluation aims at early identification of inhalation airway injuries, which may result from: 1. Direct heat (greater problems with steam burns) 2. Acute asphyxia 3. CO poisoning 4. Toxic fumes, including cyanides from combustible plastics. Sulfur and nitrogen oxides and alkalis formed during the combustion of synthetic fabrics produce corrosive chemicals that may erode mucosa and cause significant tissue sloughing. Signs of CNS toxicity caused by asphyxia or carbon monoxide poisoning vary from irritability to depression: 1. Mild CO poisoning (<20% HbCO) - slight dyspnea, headache, nausea, decreased visual acuity and higher cerbral functions 2. Moderate CO poisoning (20-40% HbCO) - irritability, agitation, nausea, dimness of vision, impaired judgment, rapid fatigue 3. Severe (40-60% HbCO) - confusion, hallucination, ataxia, collapse, acidosis, coma CO poisoning should be assumed until the tests are performed, and is treated for 100% oxygen. Significant CO poisoning requires hyperbaric O2 therapy. Cyanide poisoning should be suspected if a metabolic acidosis persists despite adequate fluid resuscitation, or in environments containing synthetic polymers. "

Answer 7

A """Nelson 21st p1943 Treatment of esophageal foreign bodies usually merits endoscopic visualization of the object and underlying mucosa and removal of the object using an appropriately designed foreign body-retrieving accessory instrument through the endoscope and with an endotracheal tube protecting the airway. Sharp objects in the esophagus, multiple magnets or single magnet with a metallic object, or foreign bodies associated with respiratory symptoms mandate urgent removal within 12hr of presentation. Button batteries, in particular, must be emergently removed within 2hr of presentation regardless of the timing of the patient's last oral intake because they induce mucosal injury in as little as 1hr of contact time and involve all esophageal layers within 4hr Asymptomatic blunut objects and coins lodged in the esophagus can be observed for up to 24hr in anticipation of passage into the stomach. """

Answer 8

D """Nelson 21st p322 Nipple pain is one of the most common complaints of breastfeeding mothers in the immediate postpartum period. Poor infant positioning and improper latch are the most common reasons for nipple pain beyond the mild discomfort felt early in breastfeeding. """

Answer 9

A """Nelson 21st p326 Table 56.7. Important principles for weaning - Begin at 6 mo of age. - At the proper age, encourage a cup rather than a bottle. - Introduce 1 new food at a time. - Energy density should exceed that of breast milk. - Iron-containing foods (meat, iron-supplemented cereals) are required. - Zinc intake should be encouraged with foods such as meat, dairy products, wheat, and rice. - Phytate intake should be low to enhance mineral absorption. - Breast milk should continue to 12 mo of age; formula or cow’s milk is then substituted. - Give no more than 24 oz/day of cow’s milk. - Fluids other than breast milk, formula, and water should be discouraged. - Give no more than 4-6 oz/day of 100% fruit juice; no sugarsweetened beverages."""

Answer 10

B """Nelson 21st p326 Bottle weaning should begin around 12-15 mo, and bedtime bottles should be discouraged because of the association with dental carries. Unless being used at mealtime, the sippy cup should only contain water to prevent caries. Sugar-sweetened beverages and 100% fruit juice should also be discouraged from being used in bottles in all infants at all times."""

Answer 11

B """5 feet = 152.4cm = 1.52m BMI = 21.64 Patient is 17 years old, so we can use adult criteria. BMI is normal Nelson 21st p346 Adults with a BMI >=30 meet the criterion for obesity, and those with a BMI 25--30 fall in the overweight range. Obesity and overweight are defined using BMI percentiles for children >=2 yr old and weight/length percentiles for infants <2 yr old. The criterion for obesity is BMI >=95th percentile and for overweight si BMI between 85th and 95th percentiles. """

Answer 12

A """Nelson 21st p336 Severe acute malnutrition is defined as severe wasting and/or bilateral edema. Other terms are marasmus (severe wasting), kwashiorkor (characterized by edema), and marasmic-kwashiorkor (severe wasting and edema). Severe wasting is most visible on the thighs, buttocks, and upper arms, as well as over the ribs and scapulae, where loss of fat and skeletal muscle is greatest. Wasting is preceded by failure to gain weight and then by weight loss. The skin loses turgor and becomes loose as subcutaneous tissues are broken down to provide energy. The face may retain a relatively normal appearance, but eventually becomes wasted and wizened. The eyes may be sunken from loss of retroorbital fat, and lacrimal and salivary glands may atrophy, leading to lack of tears and a dry mouth. Weakened abdominal muscles and gas from bacterial overgrowth of the upper gut may lead to a distended abdomen. Severely wasted children are often fretful and irritable."""

Answer 13

B "Nelson 21st p368-369 After a long period of deficiency, the classic triad of dermatitis, diarrhea, and dementia appears. Dermatitis, the most characteristic manifestation of pellagra, can develop suddenly or insidiously and may be initiated by irritants, including intense sunlight."

Answer 14

B """Nelson 21st p365-366 Death from thiamine deficiency usually is secondary to cardiac involvement. The initial signs are cyanosis and dyspnea, but tachycardia, enlargement of the liver, loss of consciousness, and convulsions can develop rapidly."""

Answer 15

D """Nelson 21st p1954-1955 The majority of symptomatic Meckel diverticula are lined by an ectopic mucosa, including an acid-secreting mucosa that causes intermittent painless rectal bleeding by ulceration of the adjacent normal ileal mucosa. Less often, a Meckel diverticulum is associated with partial or complete bowel obstruction. The most common mechanism of obstruction occurs when the diverticulum acts as the lead point of an intussusception. The mean age of onset of obstruction is younger than that for patients presenting with bleeding."""

Answer 16

B "Moderate dehydration. Treat with IMCI plan B Choice A is plan A Choice B is plan B (although recommended is to give 450-600ml ORS) Choice C and D is plan C Nelsons 21st p429 Table 70.1. Clinical evaluation of dehydration 1. Mild dehydration (<5% in an infant, <3% in an older child) - normal or increased pulse, decreased urine output, thirsty, normal physical findings 2. Moderate dehydration (5-10% in an infant, 3-6% in an older child) - tachycardia, little or no urine output, irritable/lethargic, sunken eyes and fontanel, decreased tears, dry mucus membranes, mild delay in elasticicty (skin turgor), delayed capillary refill (>1.5 sec), cool and pale 3. Severe dehydration (>10% in an infant, >6% in an older child) - peripheral pulses either rapid and weak or absent, decreased BP, no urine output, very sunken eyes and fontanel, no tears, parched mucuous membranes, delayed elasticity (poor skin turgor), very delayed capillary refil (>3 sec), cool and mottled, depressed consciousness IMCI 2014 Chart Booklet p19 Plan B: Treat some dehydration with ORS In the clinic, give recommended amount of ORS over a 4 hour period ``` Determine the amount of ORS to give during the first 4hrs Weight <6kg, up to 4mos: 200-450ml Weight <10kg, 4-12mos: 450-800ml Weight <12kg, 12mos-2yrs: 800-960ml Weight 12-19kg, 2-5yrs: 960-1000ml " ```

Answer 17

B """Nelsons 21st p1947 Pyloric stenosis The diagnosis has traditionally been established by palpating the pyloric mass. The mass is firm, movable, approximately 2cm in length, olive-shaped, hard, best palpated from the left side, and located above and to the right of the umbilicus in the mid-epigastrium beneath the liver edge. Two imaging studies are commonly used to establish the diagnosis. Ultrasound examination confirms the diagnosis in the majority of cases. Criteria for diagnosis include pyloric thickness 3-4mm, an overall pyloric length 15-19mm, and a pyloric diameter of 10-14mm. Ultrasonography has a sensitivity of approximately 95%. When contrast studies are performed, they demonstrate an elongated pyloric channel (string sign), a bulge of pyloric muscle into the antrum (shoulder sign) and parallel streaks of barium seen in the narrowed channel, producing a double tract sign. """

Answer 18

C "Nelson 21st p1962 Table 358.9 Distinguishing features of Hirschsprung disease and functional constipation FUNCTIONAL CONSTIPATION ``` Onset after 2 yrs Encopresis common Failure to thrive uncommmon Enterocolitis none Forced bowel traning usual ``` ``` Abdominal distension uncommon Poor weight gain rare Rectum filled with stool Rectal examination: stool in rectum No malnutrition ``` Anorectal manometry reveals relaxation of internal anal sphincter Rectal biopsy is normal Barium enema shows massive amounts of stool with no transition zone HIRSCHSPRUNG DISEASE ``` Onset at birth Encopresis very rare Failure to thrive possible Enterocolitis possible Forced bowel training: none ``` ``` Abdominal distension common Poor weight gain common Rectum empty Rectal examination: explosive passage of stool Malnutrition: possible ``` Anorectal manometry reveals failure of internal anal sphincter relaxation Rectal biopsy shows no ganglion cells Barium enema shows transition zone with delayed evacuation of barium"

Answer 19

B "Choice A is intussussception: No mention of bloody stools in the question Choice C is meconium ileus, which presents with ground-glass appearance on abdominal radiography Nelson 21st p1962-1963 Hirschsprung disease is usually diagnosed in the neonatal period secondary to a distended abdomen, failure to pass meconium, and/or bilious emesis or aspirates with feeding intolerance. In 99% of healthy full-term infants, meconium is passed within 48hr of birth. Hirschsprung disease should be suspected in any full-term infant (the disease is unusual in preterm infants) with delayed passage of stool. Hirschsprung disease is the result of an absence of ganglion cells in the bowel wall, extending proximally and continuously from the anus for a variable distance. The absence of neural innervation is a consequence of an arrest of neuroblast migration from the proximal to distal bowel. Without the myenteric and submucosal plexus, there is inadequate relaxation of the bowel wall and bowel wall hypertonicity, which can lead to intestinal obstruction. Rectal suction biopsy is the gold standard for diagnosing Hirschsprung disease. An unprepared contrast enema is most likely to aid in the diagnosis in children older than 1mo of age because the proximal ganglioonic segment might not be significantly dilated in the first few wk of life. "

Answer 20

D "Nelson 21st p2098-2099 Abdominal ultrasound is a helpful diagnostic tool in evaluating neonatal cholestatis because it identifies choledocholithiasis, perforation of the bile duct, or other structural abnormalities of the biliary tree such as choledochal cyst. In patients with biliary atresia, ultrasound can detect associated anomalies such as abdominal polysplenia and vascular malformations. Percutanous liver biopsy is the most valuable procedure for evaluation of neonatal hepatobiliary diseases and provides the most reliable discriminatory evidence. All patients with biliary atresia should undergo exploratory laparotomy and direct cholangiography to determine the presence and site of obstruction. "

Answer 21

C "Nelson 21st p1935 Infantile reflux manifests more often with regurgitation (typically postprandially), signs of esophagitis (irritability, arching, choking, gagging, feeding aversion) and resulting failure to thrive. Symptoms resolve spontaneously in the majority of infants by 12-24mo. Olderchildren can have regurgitation during the preschool years; this complaint diminishes somewhat as children age, and complaints of abdominal and chest pain supervene in later childhood and adolescents. Occasional children present with food refusal or neck contortions (arching, turning of head) designated sandifer syndrome. "

Answer 22

B """CBC not necessary since the patient has been your regular patient since school-age and presumably has had his CBC for middle adolescence at his annual checkup at 14 years old. Prev Ped 2018 p16 During the annual visit, the adolescent should undergo the following: 1. Complete history-taking to screen for risks and protective factors using the tool HEEADSSS 2. Physical examination. In addition to the routine physical examinatinon, the following should be done: - Tanner staging/SMR - breast examination - examination of the spine and shoulders, check for scoliosis and kyphosis - inspection of the genitals and anus 3. Screening procedures - CBC at every stage of adolesence - UA on first encounter - Vaginal wet mount, PAP smear for sexually active females - Serologic test for syphilis for sexually active males, nonculture test for gonorrhea and chlamydia for sexually active males and females 4. Immunization update 5. Anticipatory guidance and counseling - Self breast examination for females - healthy lifestyle: physical activity, diet, avoidance of alcohol, smoking, drug abuse - sexual behavior and risk of acquiring STIs - injury and accident prevention: use of sports protetive gear, seat belts, no driving under the influence of alcohol, no smoking in bed, no hand gun use """

Answer 23

D """Prev Ped 2018 p10-11 The WHO and DOH both recommend the use of either albendazole or mebendazole in the following doses and schedule Albendazole 12-23 months: 200mg SD every 6 months 24 months and above: 400mg SD every 6 months Mebendazole 12 months and above: 500mg SD every 6 months Deworming must not be done in children with the following: - severe malnutrition - high grade fever - profuse diarrhea - abdominal pain - serious illness - previous hypersensitivity to antihelminthic drug """

Answer 24

C """Gross motor red flag for not walking is 15 months (Prev Ped 2018 p19) or 18 months (Nelsons 21st p 158 Table 28.2) Prev Ped 2018 p19 As children grow and develop, one needs to be mindful that each child develops at his/her own pace and the range of what is considered to be """"normal"""" is quite wide. However, there are absolute indicators often referred to as red flags that identify developmental markers suggesting the need for further neurodevelopmental evaluation"""

Answer 25

B """Prev Ped 2018 p7 The Pediatric Nephrology Society of the Philippines recommends routine BP measurement annualy for all children >3 years of age and adolescents. BP however should also be checked in every encounter on all ill patients and all patients at risk such as those with obesity, those taking medications known to increase BP, those with renal disease, history of aortic arch obstruction, diabetes. """

Answer 26

D "Prev Ped 2018 p26 Breastmilk storage period 1. Room temperature (<25C) - 4 hours 2. Room temperature (>25C) - 1 hour 3. Referigerator (4C) - 8 days 4. Freezer compartment of a 1 door refrigerator - 2 weeks 5. Freezer compartment of a 2 door refrigerator - 3 months 6. Deep freezer with a constant temperature -20C - 6 months"

Answer 27

C "Preseptal cellulitis - no proptosis, normal ocular movements, normal pupil function Orbital cellulitis - proptosis, limitation of eye movement, edema of the conjunctiva, inflammation and swelling of the eyelids with potentially decreased visual acuity Nelson 21st p3392 Orbital cellulitis Inflammation of the tissues of the orbit, characterized by the triad of proptosis, painful limitation of movement of the eye, and potentially decreased visual acuity, is termed orbital cellulitis. Edema of the conjunctiva (chemosis) and inflammation and swelling of the eyelids may be seen. Ortbital cellulitis must be recognized promptly and treated aggressively. Hospitalization and systemic antibiotic therapy are indicated. Antimicrobial agents should begin with IV ampicillin-sulbactam OR IV clindamycin plus ceftriaxone, cefepine or cefotaxime. In cases where there is suspicion for intracranial extension, vancomycin plus cefotaxime/ceftriaxone plue metronidazole should be given. "

Answer 28

D "Nelson 21st p3377 Beginning at 16wk of gestation, retinal angiogenesis normally proceeds from the optic disk to the periphery, reaching the outer rim of the retina nasally at about 36wk and extending temporally by approximately 40wk. Injury to this process results in various pathologic and clinical changes. The risk factors associated with ROP are not fully known, but prematurity and associated retinal immaturity at birth represent the major factors. Oxygenation, respiratory distress, apnea, bradycardia, heart disease, infection, hypercarbia, acidosis, anemia, and the need for transfusion are thought by some to be contributory factors. Generally the lower the gestational age, the lower the birthweight, and the sicker the infant, the greater the risk for ROP. The basic pathogenesis of ROP is still unknown. Exposure to the extrauterine environment, including the necessarily high inspired oxygen concentrations, produces cellular damage, perhaps mediated by free radicals. Later in the course of the disease, peripheral hypoxia develops and vascular endothelial growth factors (VEGFs) are produced in the nonvascularized retina. These growth factors stimulate abnormal vasculogenesis, causing neovascularization to occur. Because of poor pulmonary function, a state of relative retinal hypoxia occurs. This causes upregulation VEGF, which, in susceptible infants, can cause abnormal fibrovascular growth. This neovascularization may then lead to scarring and loss of vision. "

Answer 29

D """Nelson 21st p3421 A diagnosis of AOM according to the 2013 American Academy of Pediatrics guidelines should be made in children who present with: 1. Moderate to severe bulging of the TM or new onset otorrhea not caused by otitis externa 2. Mild bulging of the TM and recent (<48hr) onset of ear pain or intense TM erythema A diagnosis of AOM should not be made in children without middle ear effusion. Both AOM and OME are accompanied by physical signs of middle ear effusion, namely the presence of a least 2 of 3 TM abnormalities: white, yellow, amber, or blue discoloration; opacification other than that cause by scarring; decreased or absent mobility. To support a diagnosis of AOM instead of OME in a child with MEE, distinct fullness or bulging of the TM may be present, with or without accompanying erythema, or, at a minimum, MEE should be accompanied by ear pain that appears clinically important. In otitis media with effusion (OME), bulging of the TM is absent or slight or the membrane may be retracted; erythema is also absent or slight but may increase with crying or with superficial trauma to the external auditory canal incurred in clearing the canal of cerumen. """

Answer 30

A "Nelson 21st p2595 The genes for factors VIII and IX are carried near the terminus of the long arm of the X chromosome and are therefore X linked traits Nelson 21st p645 In X linked disorders, males are more commonly affected than females. Female carriers of these disorders are generally unaffected, or if affected, they are affected more mildly than males. In each pregnancy, female carriers have a 25% chance of having an affected son, a 25% chance of having a carrier daughter, and a 50% chance of having a child that does not inherit the mutated X linked gene. Since affected males pass the X chromosome to all their daughters and their Y chromosomes to their sons, they have a 50% chance of having an unaffected son dthat does not carry the disease and a 50% chance of having a daughter who is a carrier. Male-to-male transmission excludes X linked inheritanec but is seen with autosomal dominant and Y-linked inheritance. "

Answer 31

A """Trisomy 13, Patau syndrome - Cleft lip often midline; flexed fingers with postaxial polydactyly; ocular hypotelorism, bulbous nose; low-set, malformed ears; microcephaly; cerebral malformation, especially holoprosencephaly; microphthalmia, cardiac malformations; scalp defects; hypoplastic or absent ribs; visceral and genital anomalies - Early lethality in most cases, with a median survival of 12 days; ~80% die by 1 year; 10-year survival ~13%. - Survivors have significant neurodevelopmental delay. Trisomy 18, Edwards syndrome - Low birthweight, closed fists with index finger overlapping the 3rd digit and the 5th digit overlapping the 4th, narrow hips with limited abduction, short sternum, rocker-bottom feet, microcephaly, prominent occiput, micrognathia, cardiac and renal malformations, intellectual disability - ~88% of children die in the 1st year; 10-year survival ~10%. - Survivors have significant neurodevelopmental delay."""

Answer 32

C """Nelson 21st p2907 Delayed puberty is a failure of development of any pubertal feature by 13 yr of age in females and 14 yr of age in males Delay or absence of puberty is caused by 1. Constitutional delay: a variant of normal 2. Hypogonadotropic hypogonadism: low gonadotropin levels as a result of a defect of the hypothalamus/pituitary gland 3. Hypergonadotropic hypogonadism: high gonadotropic levels as a result of a lack of negative feedback because of a gonadal problem. Females may have isolated absence of adrenarche with normal breast development """

Answer 33

A "Nelson 21st p2930 Most pronounced differences in children may be related to growth and neuropsychologic systems. Tremulousness, headache, mood disturbances, behavioral swings, difficulties in sleep, decrease in attention span, hyperactivity, and a decline in school performance are common findings in childhood. Stare and lid lag are common eye findings caused by increased sympathetic activity and can be seen in thyrotoxicosis of any cause, not just Graves diesease. In general, ocular symptoms in children with Graves tend to be milder than in adults, and they improve with the restoration of euthyroidism. Childern with hyperthyroidism have an increase in cardiac output. Tachycardia, palpitations, increased systolic BP, and a widened pulse pressure are common cardiac manifestation. The skin is smooth and flushed, with excess sweating. Proximal muscle weakness is common. Thyroid hormone stimulates bone resorption, leading to low bone density and increased fracture risk in patients with chronic hyperthyroidism. "

Answer 34

C "Nelson 21st p155 In congenital pathologic short stature, an infant may or many not be born small, but growth graudally tapers throughout infancy. Causes include chromosome or genetic abnormalities, perinatal infection, extreme prematurity, and teratogens. Linear growth deceleration with or without changes in weight can occur at the onset or as a result of systemic illness or chronic inflammation. Medications such as high dose glucocorticoids may also impact growth. Analysis of growth patterns requires consideration of weight status. Poor linear growth in the setting of decreasing BMI suggests a nutritional or gastrointestinal issue, whereas poor linear growth in the context of good or robust BMI suggests a hormonal condition. In constitutional gworth delay, weight and height decrease near the end of infancy, parallel the norm through middle childhood, and accelerate toward the end of adolescence with achievement of normal adult height. In familial short stature, both the infant/child and the parents are small; growth runs parallel to and just below the normal curves. Nelson 21st p156 In familial short stature the bone age is normal (comparable to chronologic age), whereas constitutional delay, endocrinologic short stature, and undernutrition may be associated with delay in bone age comparable to height age. "

Answer 35

B "Nelson 21st p1435-1436 There are approximately 30 species of coagulase-negative staphylococci (CONS) affecting or colonizing humans: S. epidermidis, or less often, S. hominis, S. hemolyticus, and others, are widely distributed on the skin and are significant causes of nosocomial or device-associated infection. CONS, introduced at surgery, is the most common pathogen associated with CSF shunt meningitis. Most infections (70-80%) occur within 2 mo of the operation and maniest as signs of meningeal irritation, fever, increased ICP, or peritonitis from the intraabdominal position of the distal end of the shunt tubing. "

Answer 36

C "Nelson 21st p1602 Leptospirosis The septicemic phase of anicteric leptospirosis has an abrupt onset with flulike signs of fever, shaking chills, lethargy, severe headache, malaise, nausea, vomiting, and severe debilitating myalgia most prominent in the lower extremities, lumbosacral spine, and abdomen. Conjunctival suffusion with photophobia and orbital pain (in the absence of chemosis and purulent exudate), generalized lymphadenopathy, and hepatosplenomegaly may also be present. A transient erythematous maculopapular, urticarial, petechieal, purpuric, or desquamating rash occurs in 10% of cases. The second or immune phase can follow a brief asymptomatic interlude and is characterized by recurrence of fever and aseptic meningitis. Weil syndrome is a severe form of leptospirosis seen more commonly in adults (>30yr) than in children. The initial manifestations are similar to those described for anicteric leptospirosis. The immune phase, however, is characterized by jaundice, acute renal dysfunction, thrombocytopenia, and in fulminant cases, pulmonary hemorrhage and cardiovascular collapse. "

Answer 37

B "Nelson 21st p1441-1442 Scarlet fever is GAS pharyngitis associated with a characteristic rash, which is caused by an infection with pyrogenic exotoxin-producing GAS in individuals who do not have antitoxin antibodies. The rash appears within 24-48 hr after onset of symptoms, although it may appear with the first signs of illness. It often begins around the neck and spreads over the trunk and extremities. The rash is a diffuse, finely papular, erythematous eruption producing bright red discoloration of the skin, which blanches on pressure. It is often accenuated in the creases of the elbows, axilla, and groin. The skin has a goose-pimple appearance and feels rough. The cheeks are often erythematous with pallor around the mouth. After 3-4 days, the rash begins to fade and is followed by desquamation, initially on the face, progressing downward, often resembling a mild sunburn. Occasionally, sheetlike desquamation may occur around the free margins of the fingernails, palms, and soles. Examination of the pharynx of a patient reveals essentially the same findings as with GAS pharyngitis. In addition, the tongue is usually coated and the papillae are swollen. After desquamation, the reddened papillae are prominent, giving the tongue a strawberry appearance. "

Answer 38

A "Nelson 21st p1474 Empirical antimicrobial therapy should be initiated immediately after the diagnosis of invasive meningococcal infection is suspected and cultures are obtained, using a third-generation cephalosporin to cover the most likely bacterial pathogens until the diagnosis is confirmed. Once the diagnosis of B lactam sensitive meningococcal disease is confirmed in the laboratory, some authorities recommend a switch to penicillin. Nelson 21st p1474 Table 218.2 Treatment of Neisseria meningitides invasive infections 1. Penicillin G 300,000 ukday - does not clear carriage, and prophylaxis is required at the end of treatment 2. Ampicillin 200-400 mkday - does not clear carriage, and prophylaxis is required at the end of treatment 3. Cefotaxime 200-300 mkday - recommended in the neonate 4. Ceftriaxone 100mkday - preferred treatment as only given BID to OD, may reduce skin complications *Alternative therapy in patient's with allergy: Chloramphenicol, meropenem"

Answer 39

A "Nelson 21st p1510 Bacillary dysentery/shigella Presumptive data supporting a diagnosis of bacillary dysentery include the finding of fecal leukocytes (usually >50 or 100 PMNs per HPF), fecal blood, and demonstration in peripheral blood of leukocytosis with a dramatic left shift. The total peripheral WBC count is usually 5000-15000 cells/uL, although leukopenia and leukemoid reactions occur. Nelson 21st p1521 Campylobacter The clinical presentation of Campylobacter enteritis can be similar to that of enteritis caused by other bacterial pathogens. Fecal leukocytes are found in as many as 75% of cases, and fecal blood is present in 50% of cases. Campylobacter should be considered in patients with bloody stools, fever, and abdominal pain. Nelson 21st p1500 Salmonellosis The most common clinical presentation of salmonellosis is acute enteritis. The stool typically contains a moderate number of PMNs and occult blood. Mild leukocytosis may be detected. Nelson 21st p1515 E. coli Other laboratory data are at best nonspecific indicators of etiology. Fecal leukocyte examination of the stool is often positive with EIEC or occasionally positive with other diarrheagenic E. coli. "

Answer 40

B "Nelson 21st p1459 In tonsillar and pharyngeal diphtheria, sore throat is the universal early symptom. Only half of patients have fever, and fewer with dysphagia, hoarseness, malaise, or headache. Mild pharyngeal injection is followed by unilateral or bilateral tonsillar membrane formation, which can extend to involve the uvula, soft palate, posterior oropharynx, hypopharynx, or glottic areas. Underlying soft tissue edema and enlarged lymph nodes can cause a bull neck appearance. The degree of local extension correlated directly with profound prostration, bull neck appearance, and fatality due to airway compromise or toxin-mediated complications. Within a few days of respiratory tract infection (usually in the pharynx), a dense necrotic coagulum of organisms, epithelial cells, fibrin, leukocytes, and erythrocytes forms, initially white and advancing to become a gray-brown, leather like adherent pseudomembrane. Removal is difficult and reveals a bleeding edematous submucosa. "

Answer 41

A "Nelson 21st p1550 Neonatal tetanus, the infantile form of generalized tetanus, typically manifests within 3-12 days of birth. It presents as progressive difficulty in feeding (sucking and swallowing), associated hunger, and crying. Paralysis and diminished movement, stiffness and rigidity to the touch, and spasms, with or without ophistotonos, are characteristic. The umbilical stump, which is typically the portal of entry for the microorganism, may retain remnants of dirt, dung, clotted blood, or serum, or may appear relatively benign. "

Answer 42

C "Nelson 21st p1504 Typhoid fever usually manifests as high grade fever with a wide variety of associated features, such as generalized myalgia, abdominal pain, hepatosplenomegaly, and anorexia. In children, it may occur in the earlier stages of the illness and may be followed by constipation. In approximately 25% of cases, a macular or maculopapular rash (rose spots) may be visible around 7th-10th day of the illness, and lesions may appear in crops of 10-15 on the lower chest and abdomen and last 2-3 days. Patients managed as outpatients present with fever, but have less emesis, diarrhea, hepatomegaly, splenomegaly, and myalgias than patients who require hospital admission. "

Answer 43

D "Nelson 21st p3553 Staphylococcal scalded skin syndrome, which occurs predominantly in infants and children younger than 5yr of age, includes a range of disease from localized bullous impetigo to generalized cutaneous involvement with systemic illness. Onset of the rash may be preceded by malaise, fever, irritability, and exquisite tenderness of the skin. Scarlatiniform erythema develops diffusely and is accentuated in flexural and periorificial areas. The conjunctivae are inflamed and occasionally become purulent. The brightly erythematous skin may rapidly acquire a wrinkled appearance, and in severe cases, sterile, flaccid blisters and erosions develop diffusely. Circumoral erythema is characteristically prominent, as is radial crusting and fussuring around the eyes, mouth, and nose. At this stage, areas of epidermis may separate in response to gentle shear force (Nikolsky sign). As large sheets of epidermis peel away, moist, glistening, denuded areas become apparent, initially in the flexures and subsequently over much of the body surface. "

Answer 44

B "Nelson 21st p1529 The characteristic skin lesions of P. aeruginosa, ecthyma gangrenosum, whether caused by direct inoculation or a metastatic focus secondary to septicemia, begin as pink macules and progress to hemorrhagic nodules nd eventually to ulcers with ecchymotic and gangrenous centers with eschar formation, surrounded by an intendse red areola. "

Answer 45

D "Nelson 21st p1494 Azithromycin is the drug of choice in all age groups, for treatment or post eposure prophylaxis. TMP-SMX is an alternative to azithromycin for infants >2mo old and children unable to recieve azithromycin. Infantile hypertrophic pyloric stenosis is associated with macrolide use in young infants, especially in those <14 days old, with highest risk in those recieving erythromycin. "

Answer 46

C "Nelson 21st p1673 Table 273.3 Suggested intervals between Ig administration and measles vaccination 3 months - tetanus Ig, Hep A Ig, Hep B Ig 4 months - rabies Ig 5 months - varicella Ig 6 months - measles Ig in immunocompetent, CMV Ig 8 months - measles Ig in immunosuppressed, IVIG for immmune deficiencies, IVIG for ITP (400mg/kg) 10 months - IVIG for ITP (1000mg/kg) 11 months - IVIG for Kawasaki ``` Blood transfusion 0 months - washed RBCs 3 months - RBCs, adrenaline saline added 6 months - pRBC, whole blood 7 months - plasma or platelet products " ```

Answer 47

D "WRONG QUESTION. QUESTION SHOULD BE ""EXCEPT"" Answer is D if question is ""EXCEPT"" Nelson 21st p1713 Oral therapy wih acyclovir (20mg/kg/dose) given as 4 doses pe day for 5 days can be used to treat uncomplicated varicella in individuals at increased risk for moderate to severe varicella: 1. Nonpregnant individuals older than 12 yr of age 2. Individuals older than 12mo of age with chronic cutaneous or pulmonary disorders 3. Individuals recieving short term, intermittent, or aerosolized corticosteroid therapy 4. Individuals recieving long term salicylate therapy 5. Possible secondary cases among househould contacts. To be most effective, treatment should be initiated as early as possible, preferably within 24hr of the onset of the exanthem. There is less clinical benefit if treatment is initiated more than 72hr after the onset of the exanthem. "

Answer 48

C "Nelson 21st p1800-1801. Also see Fig 302.5 All HIV-exposed and HIV-infected children should recieve standard pediatric immunizations. Live oral polio vaccine should not be given due to poor immunologic response in HIV+ children as well as concern for live vaccination in potentially immunocompromised children. The risk and benefits of rotavirus vaccination should be considered in infants born to HIV-infected mothers. In other situations, the considerable attenuation of the vaccine's strains should be considered, and unless the infant has clinical symptoms of AIDS or a CD4 percentage of <15%, vaccination is likely appropriate. Other live bacterial vaccines (e.g. BCG) should be avoided because of the high incidence of BCG related disease in HIV-infected infants. Varicella and MMR vaccines are recommended for children who are not severely immunosuppressed (CD4 >=15%, CD4 count >500), but these vaccines should not be given to severely immunocompromised children. It is recommended that children with HIV recieve quadrivalent meningococcal conjugate vaccine at a younger age than the routine schedule. Adolescent vaccines are also important, including the Tdap booster and HPV vaccine. "

Answer 49

A """Nelson 21st p1764 Dengue shock syndrome is a medical emergency that may occur in any child who lives in or has a recent travel history to a tropical destination. Management begins with diagnostic suspicion and the understanding that shock often accompanies defervescence. Management of dengue hemorrhagic fever and dengue shock syndrome includes immediate evaluation of vital signs and degrees of hemoconcentration, dehydration, and electrolyte imbalance. Close monitoring is essential for at least 48hr because shock may occur or recur precipitously, usually several days after the eonet of fever. Patients who are cyanotic or have labored breathing should be given oxygen. Rapid intravenous replacement of fluids and electrolytes can frequently sustain patients until spontanous recovery occurs. Normal saline is more effective than Ringer lactated saline in treating shock. When the pulse pressure is <=10mmHg or when elevation of hematocrit persists after the replacement of fluids, Plasma or colloid preparations are indicated. Oral rehydration of children who are being monitored is useful. Prophylactic platelet transfusions have no been shown to reduce the risk of hemorrhaging or improve low platelet counts. Use of vasopressors has not resulted in significant reduction of mortality rates opver that observed with simple supportive therapy. Corticosteroids do not shorten the duration of dease or improve the prognosis in children recieving careful supportive therapy. """

Answer 50

D """Nelson 21st p1746-1747 Avoiding and treating dehydration are the main goals of the treatment of viral enteritis. A secondary goal is maintenance of the nutritional status of the patient. There is no routine role for antiviral drug treatment of viral gastroenteritis. Controlled studies show limited benefits for antidiarrheal drugs. Ig have been administered orally to both normal and immunodeficient patients with severe rotavirus gastroenteritis, but this treatment is currently considered experimental. Therapy with probiotic organisms such as Lactobacillus species has been shown to be helpful only in mild cases. Rehydration via the oral route can be accomplished in most patients with mild to moderate dehydration. Severe dehydration requires immediated IV therapy followed by oral rehydration. Modern oral rehydration solutions containing appropriate quantities of sodium and glucose promote the optimum absorption of fluid from the intestine. Other clear liquds, such as flat soda, fruit juice, and sports drinks, are inappropriate for the rehydration of young children with significant stool loss. Prolonged (>12hr) administration of exclusive clear liquids or dilute formula is without clinical benefit and actually prolongs the duration of diarrhea. Breastfeeding should be continued during hydration. Hypocaloric diets low in protein and fat such as BRAT (bananas, rice, cereal, applesauce, toast) have not been shown to be superior to a regular diet. Vaccine-associated disease has been reported in vaccine recipients who have severe combined immunodeficiency disease (a contraindication) """

Answer 51

A """Nelson 21st p1774-1775 Theoretically, rabies virus can infect any mammal, but true animal reservoirs that maintain the presence of rabies virus in the population are limited to terrestrial carnivores and bats. Rabies is rare in small mammals, including mice, squirrels, and rabbits. To date no animal-to-human transmission from these animals has been documented. Worldwide, transmission from dogs accounts for >90% of cases. In Africa and Asia, other animals serrve as prominent reservoirs, such as jackals, mongooses, and raccoon dogs. Among American domesticated pets, infected cats outnumber infected dogs probably because cats frequently prowl unsupervised and are not uniformly subject to vaccine laws. """

Answer 52

A "Nelson 21st p2375 ASD Sometimes a pulmonic ejection click may be heard. In most patients with ASD, the characteristic finding is that the second heart sound is widely split and fixed in all its splitting during all phases of respiration Nelson 21st p2379-2380 VSD The clinical findings of patients with VSD vary according to the size of the defect and pukmonary blood flow and pressure. Small VSDs with trivial left-to-right shunts and normal pulmonary arterial pressure are the most common. These patients are asymptomatic. Characteristically, a loud, harsh, or blowing holosystolic murmur is present and heard best over the lower left sternal border, and is frequently accompanied by a thrill. The holosystolic murmur of a large VSD is generally less harse than that of a small VSD and more blowing in nature because of the absence of a significant pressure gradient across the defect. Nelson 21st p2382 PDA A small PDA is usually asymptomatic and is usually diagnosed by the presence of a heart murmur. A large PDA results in bounding peripheral arterial pulses and wide pulse pressure, caused by a runoff of blood into the pulmonary artery during diastole. The classic continuous murmur is described as machinery-like in quality. It begins soon after onset of S1, reaches maximal intensity at the end of systole, and wanes in late diastole. It may be localized to the 2nd left intercostal space or radiate down the left sternal border or to the left clavicle. When PVR is increased, the diastolic component of the murmur may be less prominent or absent. In patients with a large left-to-right shunt, a low pitched mitral mid-diastolic murmur may be audible at the apex as a result of the increased volume of blood flow across the mitral valve. Nelson 21st p2373 PFO An isolated valve-incompetent patent foramen ovale is a common echocardiographic finding during infancy. It is usually of no hemodynamic significance and is not considered an ASD. A PFO may play an important role if other structural heart defects are present. "

Answer 53

ALL ARE CORRECT """Nelson 21st p1854 Table 314.1 WHO criteria for severe malaria, 2000 - impaired consciousness - prostration - respiratory distress - multiple seizures - jaundice - hemoglobinuria - abnormal bleeding - severe anemia - circuatory collapse - pulmonary edema Nelson 21st p1854 P. falciparum is the most severe form of malaria and is associated with higher density parasitemia and a number of complications. The most common complication is severe anemia, which also is associated with other malaria species. Serious complications that appear unique to P. falciparum include cerebral malaria, respiratory distress from metabolic acidosis, acute renal failure, hypotension, and bleeding diathesis. """

Answer 54

A """Nelson 21st p1641-1642 Signs of invasive candidiasis in premature infants are often nonspecific and include temperature instability, lethargy, apena, hypotension, respiratory distress, abdominal distension and thrombocytopenia In the absence of systemic manifestations, topical antifungal therapy is the treatment of choice for congenital cutaneous candidasis in full term infants. Congenital cutaneous candidiasis in preterm infants can progress to systemic disease, and therefore systemic therapy is warranted. Amphotericin B deoxycholate has been the mainstay of therapy for systemic candidiasis and is active against both yeast and myelial forms. Nephrotoxicity, hypokalemia, and hypomagnesemia are common, but amphotericin B deoxycholate is better tolerated in infants that in adult patients. Lipsosomal amphotericin is often associated with worse outcomes in infants and should only be used when urinary tract involvement can reliably be excluded. Fluconazole is often used instead of amphotericin B deoxycholate for treatment of invasive neonatal Candida infections because of its effectiveness and low incidence of side effects. It is particularly useful for urinary tract infections, obtaining high concentrations in urine. """

Answer 55

C """Nelson 21st p1718 Infection with EBV, the first human virus to ba associated with malignancy, accounts for up to 2% of cancers worldwide. Manipulation of infected cells by EBV to establish and maintain latency can lead to transformation and oncogenesis. EBV is associated with lymphoid malignancies, such as Burkitt lymphoma, Hodgkin lymphoma, aggressive NK cell leukemia, T- and NK cell lymphoproliferative disorder, and epithelial cell malignancies such as nasopharyngeal carcinoma and gastric carcinoma. """

Answer 56

B """Nelson 21st p1671-1672 Measles is a serious infection characterized by high fever, an enanthem, cough, coryza, conjunctivitis, and a prominent exanthem. After an incubation period of 8-12 days, the prodromal phase begins with a mild fever followed by onset of conjunctivitis with photophobia, coryza, a prominent cough, and increasing fever. Koplik spots represent the enanthem and are the pathognomonic sign of measles, appearing 1-4 days prior to the onset of the rash. They first appear as discrete red lesions with bluish white spots on the center on the inner aspects of the cheeks at the level of the premolars. Symptoms increase in intensity for 2-4 days until the 1st day of the rash. The rash begins on the forehead, behind the ears, and on the upper neck as a red maculopapular eruption. It then spreads downwards to the torso and extremities, reaching the palms and soles in 50% of cases. The exanthem frequently becomes confluent on the face and upper trunk. With the onset of the rash, symptoms begin to subside. The rash fades over about 7 days in the same progression as it eveolved, often leaving a fine desquamation of skin in its wake. Of the major symptoms, the cough last the longest, often up to 10 days. In more severe cases, generalized lymphadenopathy may be present, with cervical and occipital lymph nodes especially prominent. """

Answer 57

C TBIC 2016 p153. Refer to algorithm for approach to prophylaxis

Answer 58

A """ Only with radiologic evidence. Do TST as first line diagnostic. TBIC 2016 p73 Criteria for the diagnosis of TB (three or more of the following criteria) 1. EPIDEMIOLOGIC - exposure to an adult/adolescent with active TB disease 2. CLINICAL - signs and symptoms suggestive of TB 3. IMMUNOLOGIC - positive tuberculin skin test 4. RADIOLOGIC - abnormal chest radiograph suggestive of TB 5. LABORATORY - laboratory findings suggestive of TB (histological, cytological, biochemical, immmunological and/or molecular) """

Answer 59

B """Since patient is past the period of reading of TST, repeat the skin test ASAP TBIC 2016 p84 The TST should be read between 48 to 72 hours after administration. Positive TST reactions can be measured accurately for up to 7 days, while negatvie TST reactions can be read accurately up to 72 hours only. """

Answer 60

C "TBIC 2016 p86 Live virus vaccines against polio, varicella, MMR, rotavirus, or typhoid may cause suppression of the tuberculin reaction. For this reason, the TST should be postponed at least 4 to 6 weeks from a live-vaccine administration. The TST however may be administered at the same time with a live vaccine provided they are injected on different anatomic sites. It is recommended that the TST be delayed for 2 months after a bout of measles, mumps, varicella, or pertussis. In cases of generalized skin lesions (scabies, impetigo, atopic dermatitis), the TST shoould be delayed until lesions are completely healed. TBIC 2016 p152 Isoniazid preventive therapy (isoniazid (10) x6mos) is recommended for: 1. All HIV positive individuals 2. Children < 5 years old who are household contacts of a bacteriologically confirmed TB case, regardless of the TST results 3. Children < 5 years old who are household contacts of a clinically diganosed TB case, if the TST result is positive TBIC 2016 p153. Refer to algorithm for approach to prophylaxis "

Answer 61

C "TBIC p71 Table 7.1. Spectrum of TB exposure, infection, and pulmonary disease ``` TB exposure (+) exposure (-) signs and symptoms (-) TST/IGRA (-) chext xray (-) direct sputum smear microscopy (AFB) (-) other diagnostics ``` ``` TB infection (+) exposure (-) signs and symptoms (+) TST/IGRA (-) chext xray (-) direct sputum smear microscopy (AFB) (-/+) other diagnostics ``` ``` TB disease (+) exposure (+) signs and symptoms (+) TST/IGRA (-/+) chext xray (-/+) direct sputum smear microscopy (AFB) (-/+) other diagnostics" ```

Answer 62

A "TBIC 2016 p74-75 For patients at least 15 years old and above, presumptive TB has any of the following: 1. Cough of at least 2 weeks duration with or without the following symptoms: - significant and unintentional weight loss - fever - hemoptysis - chest/back paints not referrable to any musculoskeletal disorders - easy fatigability or malaise - night sweats - shortness of breath or difficulty of breathing 2. Unexplained cough of any duration in: - close contact of a known active TB case - high risk clinical groups (ICC/HIV, immunosuppressed, etc) - high risk populations (elderly, urban poor, inmates, etc)"

Answer 63

D """TBIC 2016 p178-179 If the newborn is well, do not give BCG first. Instead give isoniazid preventive therapy for 3 months. After 3 months, do TST. If TST is negative, stop IPT and give BCG If TST is positive and the baby remains well, continue IPT for another 3 months. After 6 months of IPT and the baby remains well, give BCG If TST is not available and the newborn is well, the newborn should recieve 6 months of IPT followed by BCG immunization. The mother who has current TB disease but has undergone treatment for at least 2 weeks is presiumed to be no longer contagious at the time of delivery. Separation is recommended for a mother who has active TB disease and has not recieved treatment. """

Answer 64

D "Adverse effects of TB drugs 1. Isoniazid - hepatitis, peripheral neuropathy, allergic skin reactions, possible hemolysis among G6PD patients - inhibits drug metabolizing enzymes, leading to increased risk of phenytoin, ethosuximide, carbamazepine toxicity 2. Rifampicin - hepatitis, hypersensitivity reactions (including a systemic flu-like syndrome +/- thromocytopenia in patients given high dose intermittent therapy), orange discoloration of body fluids - induces drug metabolizing enzymes, resulting in decreased plasma levels of some drugs (AEDS, anti-infectives, hormonal therapy agents, corticosteroids, etc) 3. Pyrazinamide - nausea, vomiting, most common cause of hepatotoxicity in regimens also containing isoniazid and rifampicin, hypersensitivity reactions, polyarthralgia 4. Ethambutol - peripheral neuropathy and retrobulbar optic neuritis (impairment of visual acuity and red-green color vision) 5. Streptomycin (second line TB drug) - nephrotoxicity and consequent electrolyte disorders, ototoxicity from CN 8 damage, neuromuscular blockade"

Answer 65

C ``` "TBIC 2016 p151 Cat I - 2HRZE / 4HR Cat Ia - 2HRZE / 10HR Cat II - 2HRZES / 1HRZE / 5HRE Cat IIa - 2HRZES / 1HRZE / 9HRE" ```

Answer 66

D "Child is treated for TB Cat I with good compliance, noted, weight gain, no progression of symptoms, no chronic cough mentioned. Ideally discontinue treatment. Best answer is D TBIC 2016 p100 In the small proportion of chidlren with radiological evidence of the disease, clearing usually occurs within 6 months to 2 years after the institution of therapy. Calcifications, although not common, may also be found in TB. Calcifications on chest xray may be due to healed, healing, or quiescent infection, thus it should be correlated with the history of treatment"

Answer 67

A "TBIC 2016 p40 Age is the most important factor that determines the risk of progression to disease following primary infection among immunocompetent children. Infants are at greatest risk. The risk decreases but remains significant in the second year of life and is lowest in children 5-10 years old. "

Answer 68

A "GINA 2021 p147 It may be challenging to make a confident diagnosis of asthma in children 5 years and younger, because episodic respiratory symptoms such as wheezing and cough are also common in children without asthma, particularly in those 0-2 years old, and it is not possible to routinely assess airflow limitation or bronchodilator responsiveness in this age grou. A probability-based approach, based on the pattern of symptoms during and between viral respiratory infections may be helpful for discussion with parents/carers. This allows individual decisions to be made about whether to give a trial of controller treatment. GINA 2021 p148 A diagnosis of asthma in young children is therefore based largely on recurrent symptom patterns combined with a careful clinical assessment of family history and physical findings with careful consideration of the differential diagnostic possibilities. A positive family history of allergic disorders, or the presence of atopy or allergic sensitization provide additional predictive support, as early allergic sensitization increases the likelihood that a wheezing child will develop persistent asthma."

Answer 69

D "GINA 2021 p166 Children with a severe asthma exacerbation must be observed for at least 1 hour after initiation of treatment, at which time further treatment can be planned. If symptoms persist after initial bronchodilator, a further 2-6 puffs of salbutamol (depending on severity) may be given 20 minutes after the first dose and repeated at 20 minute intervals for an hour. Consider adding 1-2 puffs of ipratropium. Failure to respond at 1 hour, or earlier deterioration, should prompt urgent admission to hospital, addition of nebulized ipratropium, and a short course of oral corticosteroids. "

Answer 70

D "Patient is currently on LOW DOSE maintenance ICS (Fluticasone 50ug) - STEP 2 If with poor control, increase to STEP 3 - low dose maintenance ICS-LABA (Choice D) GINA 2021 p54 RELIEVER: As-needed SABA Step 1: Take ICS whenever SABA taken Step 2: Low dose maintenance ICS Step 3: Low dose maintenance ICS-LABA Stap 4: Medium/high dose maintenance ICS-LABA Step 5: Add on LAMA, refer for phenotypic assessment, consider high dose ICS-LABA "

Answer 71

C "Nelsons 21st p1208. Emergency department management of asthma exacerbations Initial treatment includes supplemental oxygen, inhaled B-agonist therapy every 20 min for 1 hr, and, if necessary, oral or IV systemic corticosteroids. Inhaled ipratropium may be added to the B-agonist treatment if no significant rsponse is seen with the first inhaled B-agonist treatment. An IM injection of epinephrine or other B-agonist may be administered in severe cases. Oxygen should be administered and continued for at least 20 min after SABA administration to compensate for ventilation/perfusion abnormalities caused by SABA "

Answer 72

B All other choices can be diagnosed clinically. Atelectasis is a radiographic finding.

Answer 73

C "Enlarged cervical lymph nodes suggestive of tuberculous etiology. TBIC 2016 p75 For patients below 15 years old, a presumptive PTB has any of the following A. At least 3 of the following clinical criteria: 1. Coughing/wheezing of 2 weeks or more, especially if unexplained 2. Unexplained fever of 2 weeks or more after common causes such as pneumonia and malaria have been excluded 3. Loss of weight/failure to gain weight/weight faltering/loss of appetite 4. Failure to respond to 2 weeks of appropriate antibiotic therapy for lower respiratory tract infection 5. Failure to regain previous state of health 2 weeks after a viral infection or exanthema 6. Fatigue, reduced playfulness, or letheargy B. Any 1 of the above signs and symptoms in a child who is a close contact of a known active TB case TBIC 2016 p73 In the absence of baceteriologic evidence, a child is presumed to have active TB if 3 or more of the following criteria are present: 1. Epidemiologic - exposure to an adult/adolescent with active TB disease 2. Clinical - signs and symptoms suggestive of PTB 3. Immunologic - positive TST 4. Radiologic - abnormal chest radiograph suggestive of TB 5. Laboratory - laboratory findings suggestive of PTB (histological, cytological, biochemical, immunological, or molecular) GINA 2021 p148 Box 6-2 Features suggesting a diagnosis of asthma in children 5 years or younger 1. Cough - recurrent or persistent nonproductive cough that may be worse at night or accompanied by wheezing and breathing difficulties; cough occuring with exercise, laughing, crying, or exposure to tobacco smoke, particularly in the absence of an apparent respiratory infection 2. Wheezing - recurrent wheezing, including during sleep or with triggers such as activity, laughing, crying or exposure to tobacco smoke or air pollution 3. Difficult or heavy breathing or shortness of breath occuring with exercise, laughing or crying 4. Reduced activity - not running, playing, or laughing at the same intensity as other children, tires earlier during walks 5. Past or family history - other allergic disease (atopic dermatitis, allergic rhinitis, food allergy); asthma in first degree relatives 6. Therapeutic trial with low dose ICS and as needed SABA - clinical improvement during 2-3 months of controller treatment and worsening when treatment is stopped. "

Answer 74

A ``` "Nelson 21st p2276 Exudates usually have at least 1 of the following features: 1. Protein level >3.0 g/dl 2. PF:serum protein ratio >0.5 3. PF LDH >200 IU/L 4. PF:serum LDH ratio >0.6 ``` Although systemic acidosis reduces the usefulness of pleural fluid pH measurements, pH <7.20 suggests an exudate. Glucose is usually <60 mg/dl in malignancy, rheumatoid disease, and tuberculosis; the finding of many small lymphocytes and a pH < 7.20 suggests tuberculosis. The fluid of serofibrinous pleurisy is clear or slightly cloudy and contains relatively few leukocytes, and, occasionally, some erythrocytes. In older childen with suspected parapneumonic effusion, tube thoracostomy is considered necessary if the pleural fluid pH is <7.20 or the pleural fluid glucose level is <50mg/dl. If the fluid is thick, loculated, or clearly purulent, tube drainage with fibrinolytic therapy or less often VATS is indicated. Lights criteria 1. Transudate ``` Serous WBC <10,000 pH >7.2 (alkaline) Protein < 3.0 (low) PF:serum protein ratio <0.5 LDH <200 (low) PF: serum LDH ratio <0.6 Glucose >60 (high) ``` 2. Exudate ``` Cloudy WBC >50,000 pH <7.2 (acidic) Protein > 3.0 (high) PF:serum protein ratio > 0.5 LDH > 200 (high) PF: serum LDH ratio >0.6 Glucose < 60 (low) ``` TRANSUDATE: Alkaline, low protein, low LDH, high glucose EXUDATE: Acidic, high protein, high LDH, low glucose (Nelson 21st p2274)"

Answer 75

A Diagnosis is viral pnuemonia. Treatment is supportive and symptomatic treatment

Answer 76

A """Nelson 21st p2206 Tracheitis Typically the child has a brassy cough, apparently as part of a viral laryngotracheobronchitis. High fever and toxicity with respiratory distress can occur immediately or after a few days of apparent improvement. The patient can lie flat, does not drool, and does not hav the dysphagia assocaited with epiglottitis. The major pathologic feature appears to be mucosal swelling at the level of the cricoid cartilage, complicated by copious thick purulent secretions, sometimes causing pseudomembranes. The diagnosis is based on evidence of bacterial upper airway disease, which includes high fever, purulent airway secretions, and an absence of the classical findings of epiglottitis. Xrays are not needed but can show the classic findings (e.g. pseudomembranes, ragged air column). Purulent maternal is noted below the cords during endotracheal intubation. """

Answer 77

D "Diagnosis is croup Nelson 21st p2204-2205 The mainstay of treatment for children with croup is airway management and treatment of hypoxia. Treatment of the respiratory distress should take priority over any testing. Dspite the observation that cold night air is beneficial, a Cochrane review has found no evidence supporting the use of cool mist in the emergency department for treatment of croup Nebulized racemic epinephrine is the established treatment for moderate or severe croup. The mechanism of action is believed to be constriction of the precapillary arterioles through the B-adrenergic receptors, causing fluid resorption from the interstitial space and a decrease in the laryngeal mucosal edema. A dose of 0.25-0.5ml of 2.25% racemic epinephrine in 3ml of normal saline can be used as often as every 20 min. The effectiveness of corticosteroids in viral croup is well established. Corticosteroids decrease the edema in the laryngeal mucosa through their anti inflammatory action. Most studies have demonstrated the efficacy of oral dexamethasone used as a single dose of 0.6mg/kg Antibiotics are not indicated in croup. Nonprescription cough or cold medications should not be used in children younger than 6 yr of age. "

Answer 78

B "PPS Policy Statements 2009 vol 1 no 6 p27-30 Preoperative evaluation in pediatric patients undergoing surgery and other major therapeutic and diagnostic procedures Preoperative evaluation is required in all patients undergoing diagnostic or therapeutic procedures except: 1. Healthy patients requiring nerve blocks, local or topic anesthesia and or no more than 50% Nnitrogen oxide, oxygen, and no other sedative or analgesic agents 2. Patients recieving sedation analgesia or conscious sedation For the pediatric population, the greatest risks are plmonary and airway complications. Most laboratory examinations, including hemoglobin, potassium, coagulation studies, CXR, and ECG are not necessary with routine procedures unless a specific indication is present. Routine hematocrit is of importance in infants <6mo old who are undergoing surgery due to an increased incidence of unrecognized anemia which is a risk factor for perioperative apnea and cardiac arrest. The working group for this policy statement recommends the following laboratory examinations to be done routinely when obtaining preoperative clearance in the pediatric population: CBC, CXR Appendix B - indications for common lab tests 1. ECG - if with no ECG within last year with history of DM, HTN, smoking, peripheral vascular disease, inability to exercise, or morbid obesity 2. Coagulation studies - patient has a known history of coagulation abnormalities or recent history suggesting coagulation problems or is on anticoagulants; or if patient needs anticoagulation post surgery 3. Hgb - patient has a history suggesting recent blood loss or anemia 4. Potassium - patient is taking digoxin or diuretics 5. CXR - patient has signs and symptoms suggesting new or unstable cardiopulmonary disease"

Answer 79

C """Nelson 21st p2185-2186 Young children have an average of 6-8 colds per yr, but 10-15% of children have at least 12 infections per yr. The incidence of illness decreases with increasing age, with 2-3 illnesses per yr of by adulthood. Viruses that cause the common cold are spread by 3 mechanisms: 1. Direct hand contact - self-inoculation of one's own nasa mucosa or conjunctivae after touching a contaminated person or object 2. Inhalation of small-particle aerosols that are airborne from coughing 3. Deposition of large particle aerosols that are expelled during a sneeze and land on nasal or conjunctival mucosa Symptoms of the common cold vary by age and virus. In infants, fever and nasal discharge may predominate. Fever is uncommon in older children and adults. The onset of common cold symptoms typically occurs 1-3 days after viral infection. The first symptom noted is often sore or scratchy throat, followed closely by nasal obstruction and rhinorrhea. The sore throat usually resolves quickly and by the 2nd and 3rd day of illness, nasal symptoms predominate. Cough is associated with 2/3 of colds in children and usually begins after the onset of the nasal symptoms. Cough may persist for an additional 1-2 wk after resolution of other symptoms. """

Answer 80

C "PAPP 2021 PCAP CPG p15-16 Criteria for severe pneumonia (requiring admission) 1. Respiratory signs - cyanosis/hypoxemia, head bobbing, retractions, apena, grunting 2. CNS signs - lethargic/stuporous/comatose/GCS <13, seizures 3. Circulatory signs - capillary refill >3s or shock, pallor 4. General considerations - moderate to severe malnutrition, refusal or inability to take food/drink per orem, some to severe dehydration, age <6 months 5. Ancillary parameters - CXR or UTZ findings of consolidation, multifocal disease, moderate to large effusion, abscess, air leak; sustained O2 sat at room air <=93% "

Answer 81

A "Nelson 21st p2459 Mitral insufficiency With mild disease, signs of heart failure are not present, the precordium is quiet, and auscultation reveals a high pitched holosystolic murmur at the apex that radiates to the axilla. With severe mitral insufficiency, signs of acute or chronic heart failure may be noted. A holosystolic murmur is heard at the apex with radiation to the axilla. A short mid-diastolic rumbling murmur is caused by increased blood flow across the mitral valve as a result of the significant insufficiency. Therefore, auscultation of a diastolic murmur, often referred to as relative mitral stenosis (Carey-Coombs murmur) does not necessarily mean that true mitral stenosis is present. Nelson 21st p2393 Mitral stenosis Most patients have rumbling apical diastolic murmurs, but the ascultatory findings may be relatively obscure. S2 is loud and split Nelson 21st p2406 Tricuspid regurgitation A holosystolic murmur caused by tricuspid regurgitation is audible over most of the anterior left side of the chest. "

Answer 82

C "Nelson 21st p2396 Tetralogy of Fallot is one of the conotruncal family of heart lesions in which the primary defect is an anterior deviation of the infundibular septum. The consequences of this deviation are: 1. Obstruction to right ventricular outflow (pulmonary stenosis) 2. Malalignment type of VSD 3. Dextroposition of the aorta so it overrides the ventricular septum 4. Right ventricular hypertrophy The degree of pulmonary outflow obstruction and whether the ductus arteriosus is open or closed determine the degree of the patient's cyanosis and the age at first presentation. "

Answer 83

A "Nelson 21st p669 Turner syndrome is a condition characterized by complete or partial monosomy of the X chromosome and defined by a combination of phenotypic features. Half the patients with Turner syndrome have a 45, X chromosome complement. The other half exhibit moasicism and varied structural abnormalities of the X or Y chromosome. Maternal age is not a predisposing factor for children with 45, X. Clinical findings in the newborns can include small size for gestational age, webbing of the neck, protruding ears, and lymphedema of the hands and feet. Congenital heart defects (40%) and structural renal anomalies (60%) are common. The most common heart defects are bicuspid aortic valves, coarctation of the aorta, aortic stenosis, and mitral valve prolapse. In contrast to Turner syndrome, Noonan syndrome affects both sexes and has a different pattern of congenital heart disease, typcically involving right sided lesions. Klinefelter syndrome (47, XXY) is not associated with specific heart defects. Persons with Klinefelter syndrome are phenotypically male; this syndrome is the most common cause of hypogonadism and infertility in males and is the most common sex chromosome aneuploidy in humans. "

Answer 84

C "Nelson 21st p1445 As many as 2/3 of patients with an acute episode of RF have a history of URTI several weeks before, and the peak age and seasonal incidence of acute RF closely parallel that of GAS pharyngitis. Patients with acute RF almost always have serologic evidence of a recent GAS infection. Nelson 21st p1446 An immune-mediated pathogenesis for acute RF and rheumatic heart disease has been suggested by its clinical similarity to other diseases with an immunopathogenesis and by the latent perior between GAS infection and acute RF. Common epitopes are shared between certain GAS components and specific mammalian tissues (heart valve, sarcolemma, brain, joint). "

Answer 85

A "Nelson 21st p2375 ASD Sometimes a pulmonic ejection click may be heard. In most patients with ASD, the characteristic finding is that the second heart sound is widely split and fixed in all its splitting during all phases of respiration Nelson 21st p2379-2380 VSD The clinical findings of patients with VSD vary according to the size of the defect and pukmonary blood flow and pressure. Small VSDs with trivial left-to-right shunts and normal pulmonary arterial pressure are the most common. These patients are asymptomatic. Characteristically, a loud, harsh, or blowing holosystolic murmur is present and heard best over the lower left sternal border, and is frequently accompanied by a thrill. The holosystolic murmur of a large VSD is generally less harse than that of a small VSD and more blowing in nature because of the absence of a significant pressure gradient across the defect. Nelson 21st p2382 PDA A small PDA is usually asymptomatic and is usually diagnosed by the presence of a heart murmur. A large PDA results in bounding peripheral arterial pulses and wide pulse pressure, caused by a runoff of blood into the pulmonary artery during diastole. The classic continuous murmur is described as machinery-like in quality. It begins soon after onset of S1, reaches maximal intensity at the end of systole, and wanes in late diastole. It may be localized to the 2nd left intercostal space or radiate down the left sternal border or to the left clavicle. When PVR is increased, the diastolic component of the murmur may be less prominent or absent. In patients with a large left-to-right shunt, a low pitched mitral mid-diastolic murmur may be audible at the apex as a result of the increased volume of blood flow across the mitral valve. Nelson 21st p2373 PFO An isolated valve-incompetent patent foramen ovale is a common echocardiographic finding during infancy. It is usually of no hemodynamic significance and is not considered an ASD. A PFO may play an important role if other structural heart defects are present. "

Answer 86

B "Nelson 21st p2477 In infants heart failure may be difficult to distinguish from other causes of respiratory distress. Prominent manifestations of heart failure include tachypnea, feeding difficulties, poor weight gain, excessive perspiration, irritability, weak cry, and noisy labored respirations with intercostal and subcostal retractions with alar flaring. The signs of cardiac-induced pulmonary congestion may be indistinguishable from those of bronchiolitis; wheezing is often a more prominent finding in young infants with heart failure than rales. Pneumonitis with or without atelectasis is common as a result of bronchial compression by an enlarged heart. Hepatomegaly usually occurs, and cardiomegaly is invariably present. Despite pronounced tachycardia, a gallop rhythm can frequently be recognized. The other auscultatory signs are those produced by the underlying cardiac lesion. Clinical assessment of jugular venous pressure in infants may be difficult because of the shortness of the neck and the difficulty of observing a relaxed state; palpation of an enlarged liver is a more reliable sign. "

Answer 87

D "Nelson 21st p1277-1278 Hydroxychloroquine is recommended for all individuals with SLE when tolerated. In addition to treating mild SLE manifestations such as rash and mild arthritis, hydroxychloroquine prevents SLE flares, improves lipid profiles, and may improve mortality and renal outcomes. Potential toxicities include retinal deposition and subsequent visual impairment. Corticosteroids are a treatment mainstay for significant manifestations of SLE and work quickly to improve acute deterioration; side effects often limit patient adherence, especially in adolescence, and potential toxicities are worrisome. It is important to limit dose and length of exposure to corticosteroids whenever possible. Steroid-sparing immunosuppresive agents for the treatment for pediatric SLE include methotrexate, leflunomide, azathioprine, myophenolate mofetil, tacrolimus, cyclophosphamide, rituximab, and benilimumab. 1. Methotrexate, leflunomide, azathioprine - persistent moderate disease, including arthritis, significant cutaneous or hematologic involvement, and pleural disease. 2. Cyclophosphamide, MMF, azathioprine - lupus nephritis. 3. MMF, rituximab - significant hematologic manifestations, including severe leukopenia, hemolytic anemia, thrombocytopenia "

Answer 88

D "Nelson 21st p1310 Kawasaki In addition to fever, the 5 principal criteria of KD are: 1. Bilateral nonexudative conjunctival injection with limbal sparing 2. Erythema of the oral and pharyngeal mucosa with strawberry tongue and red, cracked lips 3. Edema and erythema of the hands and feet 4. Rash of various forms (maculopapular, erythema multiforme, scarletiniform or less often psoriatic-like, urticarial, or micropustular) 5. Nonsuppurative unilateral cervical lymphadenopathy (>1.5cm)"

Answer 89

B "Nelson 21st p1263-1265 JIA is a clinical diagnosis without any diagnostic laboratory tests. The meticulous clinical exclusion of other diseases and many mimics therfore essential. Laboratory studies, including tests for ANA and RF are only supportive or prognostic, and their results may be normal in patients with JIA. Hematologic abnormalities often reflect the degree of systemic or articular inflammation, with elevated WBC and platelet counts and microcytic anemia. Inflammation may also cause elevations in ESR and CRP, although it is not unusual for both to be normal in children with JIA Elevated ANA titers are present in 40-85% of children with oligoarticular or polyarticular JIA, but are rare with sJIA. Approximately 5-15% of patients with polyarticular JIA are seropositive for RF. Both ANA and RF seropositivity can occur in association with transient events, such as viral infections. Children with sJIA usually have striking elevations in inflammatory markers and WBC and platelet counts. Hgb levels are low, typically 7-10 g/dL, with indices consistent with anemia of chronic diease. The ESR is usually high, except in MAS. Although immunoglobulin levels tend to be high, ANA and RF are uncommon. Ferritin values are typically elevated and can be markedly increased in MAS. "

Answer 90

A "Nelson 21st p400-401 The plasma K level, the ECG, and the risk of the problem worsening determine the aggressiveness of the therapeutic approach. Peak T waves are the first sign of hyperkalemia, followed by a prolonged PR interval, and when most severe, prolonged QRS complex. Life threatening ventricular arrythmia can also develop. Calcium stablizes the cell membrane in heart cells, preventing arrhythmias; it is given intravenously over a few minutes, and its action is almost immediate. Bicarbonate causes K to move intracellularly, reducing the plasma K; it is most efficacious in a patient with metabolic acidosis. Insulin causes K to move intracellularly but must be given with glucose to avoid hyperglycemia. Nebulized salbutamol, by stimulation of B-adrenergic receptors, leads to a rapid intracellular movement of K. "

Answer 91

A "Diagnosis is hypernatremic dehydration Nelson 21st p394 Probably because of intracellular water loss, the pinched abdominal skin of a dehydrated, hypernatremic infant has a ""doughy"" feel. Patients are irritable, restless, weak, and lethargic. Some infants have a high-pitched cry and hyperpnea. Alert patients are very thirsty, even though nausea may be present. Brain hemorrhage is the most devastating consequence of untreated hypernatremia. As the cellular osmolality increases, water moves out of brain cells, leading to a decrease in brain volume. This decrease can result in tearing of intracerebral veins and bridging blood vessels as the brain moves away from the skull and the meninges. "

Answer 92

D """Urine Culture Interpretation – Suprapubic aspiration Growth of urinary pathogen in any number (except 2-3 x 1000 CFU of coagulase-negative staphylococci) Catheterization Febrile infants or children usually having 50,000 CFU of a single urinary pathogen, but infection may be present with counts >1000 CFU Midstream clean-void Symptomatic patients usually having 10,000 CFU of a single urinary pathogen Midstream clean-void Asymptomatic patients having at least two specimens on different days with 10,000 of same pathogen. *Routine reculturing of urine after 2 days of antibiotics generally not necessary."""

Answer 93

C "Nelson 21st p2732 Hemolytic-uremic syndrome is a common cause of community-acquired acute kidney injury in young children. It is the most common form of thrombotic microangiopathy in children. HUS is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency. The most common form of HUS is caused by Shiga-toxin producing E. coli (STEC) which causes prodromal acute enteritis and is commmonly termed STEC-HUS or diarrhea-associated HUS. Several serotypes of E. coli can produce the toxin; O157:H7 is the most common in Europe and the Americas. Nelson 21st p2734 The diagnosis is made by the combination of microangiopathic hemolytic anemia with schistocytes, thrombocytopenia, and some degree of kidney involvement. Thrombocytopenia is an invariable finding in the acute phase, with platelet counts usually 20,000 - 100,000/mm3. Urinalysis typically shows microscopic hematuria and low grade proteinuria With early recognition and intensive supportive care, the mortality rate for diarrhea-associated HUS is <5% in most major medical centers. Up to half of patients may require dialysis support during the acute phase of the illness. Recovery of platelet counts usually occurs first, followed by renal recovery about 5 days later, and finally by resolution of anemia. Most recover renal function completely, but of surviving patients, 5% remain dependent on dialysis, and up to 30% are left with some degree of chronic renal insufficiency. "

Answer 94

D "Nelson 21st p2723-2724 Poststreptococcal GN is most common in children ages 5-12yr and uncommon before the age of 3 yr. The typical patient develops an acute nephritic syndrome 1-2 week after an antecedent streptococcal pharyngitis or 3-6 wk after a streptococcal pyoderma. The acute phase generally resolves within 6-8wk. Although urinary protein excretion and hypertension usually normalize 4-6wk after onset, persistent microscopic hematuria can persist for 1-2 yr after the initial presentation. Urinalysis demonstrates RBCs, often in association with RBC casts, proteinuria and PMNs. The serum C3 level is significantly reduced in >90% of patients in the acute phase, and returns to normal 6-8wk after the onset. A mild normochromic anemia may be present from hemodilution and low-grade hemolysis. Confirmation of the diagnosis requires clear evidence of a prior streptococcal infection. The ASO titer is commonly elevated after a pharyngeal infection but rarely increases after streptococcal skin infections. The best single antibody titer to document cutaneous streptococcal infection is the anti-DNAse B level. The clinical diagnosis of PSGN is quite likely in a child presenting with an acute nephritic syndrome, evidence of recent streptococcal infection, and low C3 level"

Answer 95

D "Nelson 21st p2732 Goodpasture disease Goodpasture disease is an autoimmmune disease characterized by pulmonary hemorrhage, rapidly progressive glomerulonephritis, and elevated anti-glomerular basement membrane titers. Nelson 21st p2757 FSGS In FSGS, glomeruli show lesions that are both focal (present in only a proportion of glomeruli) and segmental (localized to >=1 intraglomerular tufts). The lesions consist of mesangial cell proliferation and segmental scarring on light microscopy. Lesions consistent with FSGS may be seen secondary to HIV infection, vesicoureteral reflux, and IV use of heroin. Only 20% of patients with FSGS respond to prednisone. The disease is often progressive, ultimately involving all glomeruli and end-stage renal disease in most patients. Nelson 21st p2727 CGN-RPGN Patients with systemic vasculitis appear to be particularly pronte to develop CGN. Patients with HSP, ANCA mediated GN (microscopic polyangitis and granulomatosis with polyangitis) and systemic lupus erythematosus account for the majority of patients with CGN. IgA nephropathy, a common GN, only rarely is rapidly progressive. Goodpasture disease often has rapidly progressive GN as a component of the syndrome. Nelson 21st p1322 PAN The clincial presentation of PAN is variable but generally reflects the distribution of inflamed vessels. Renovascular arteritis can cause hypertension, hematuria, or proteinuria, although glomerulonephritis is not typical. "

Answer 96

D "Nelson 21st p1099 Table 148.4 Characteristic features of primary immunodeficiency Predominant T cell defect > Age at onset of infection: 2-6mo > Pathogens - bacteria: common gram-positive, gram negative bacteria, mycobacteria - viruses: CMV, EBV, adenovirus, enterovirus, varicella - fungi: Candida, Pneumocystis > Affected organs: extensive mucocutaneous candidasis, lungs, failure to thrive, protracted diarrhea > Special features - graft vs host disease - postvaccination disseminated BCG, varicella - autoimmunity common Predominant B cell defect > Age at onset: after maternal antibodies diminish, 5-7mo > Pathogens - bacteria: encapsulated bacteria (pneumococci, Hib), strep, staph, Campylobacter, Mycoplasma - viruses: enterovirus - fungi and parasites: giardia, cryptosporidium > Affected organs: recurrent sinopulmonary infections, chronic GI symptoms, malabsorption, arthritis, enteroviral meningitis > Special features - Autoimmunity - Lymphoreticular malignancy: lymphoma, thymoma Granulocyte defect > Age at onset: early onset > Pathogens - bacteria: staph, Serratia, Salmonella, mycobacteria - fungi and parasites: Candida, Nocardia, Aspergillus > Affected organs - Skin: abscesses, impetigo, cellulitis - Lymph nodes: suppurative adenitis - Oral cavity: gingivitis, mouth ulcers - Viscera: abscess, osteomyelitis > Special features: prolonged attachment of umbilical cord, poor wound healing ``` Cytologic defect > Age at onset: childhood > Pathogens - viruses: CMV, EBV > Affected organs: hemophagocytic syndrome can affect any organ ``` Complement defect > Age at onset: any age > Pathogens - bacteria: encapsulated organisms, Neisseria > Affected organs: deep or systemic infections > Special features: SLE, GN, atypical HUS "

Answer 97

C """Nelson 21st p1228 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."""

Answer 98

D """Nelson 21st p1210. Table 170.1 Clinical features of atopic dermatitis MAJOR FEATURES - Pruritus - Facial and extensor eczema in infants and children - Flexural eczema in adolescents - Chronic or relapsing dermatitis - Personal or family history of atopic disease ASSOCIATED FEATURES - Xerosis - Cutaneous infections (Staphylococcus aureus, group A streptococcus, herpes simplex, coxsackievirus, vaccinia, molluscum, warts) - Nonspecific dermatitis of the hands or feet - Ichthyosis, palmar hyperlinearity, keratosis pilaris - Nipple eczema - White dermatographism and delayed blanch response - Anterior subcapsular cataracts, keratoconus - Elevated serum IgE levels - Positive results of immediate-type allergy skin tests - Early age at onset - Dennie lines (Dennie-Morgan infraorbital folds) - Facial erythema or pallor - Course influenced by environmental and/or emotional factors"""

Answer 99

B """Nelson 21st p3092 A simple febrile seizure is a primary generalized, usually tonic-clonic attack associated with fever, lasting for a maximum of 15min, and not recurrent within a 24hr period. A complex febrile seizure is more prolonged (>15min), and/or is focal, and/or recurs within 24hrs. Febrile status epilepticus is a febrile seizure lasting longer than 30 min. Most patients with simple febrile seizures have a very short postictal state and usually return to their baseline normal behavior and consciousness within minutes of the seizure. """

Answer 100

B "TBIC 2016 p52 Onset of TB meningitis is usually gradual occuring over a period of approximately 3 weeks. The clinical course is divided into three stages 1. Stage 1 - personality changes, irritability, anorexia, listlessness, and some fever 2. Stage 2 - signs of increased intracranial pressure and cerebral damage appearing; drowsiness, stiff neck, cranial nerve palsies, inequality of the pupils, vomiting, tache cerebrale, absence of abdominal reflexes, and seizures 3. Stage 3 - coma, irregular pulse and respirations, rising fever. "

Answer 101

C "Nelson 21st p1674 Clinical manifestations of SSPE begin insidiously 7-13yr after primary measles infection. Stage I: Subtle changes in behavior appear, including irritability, reduced attention span, and temper outbursts Stage II: The hallmark of the second stage is massive myoclonus, which coincides with extension of the inflammatory process site to deeper structures in the brain, including the basal ganglia. Involuntary movements and repetitive myclonic jerks begin in single muscle groups but give way to massive spasms and jerks involving both axial and appendicular muscles Stage III: Involuntary movements disappear and are replaced by choreoathetosis, immobility, dystonia, and lead pipe rigidity that result from destruction of deeper centers in the basal ganglia. The sensorium deteriorates into dementia, stupor, and then coma. Stage IV: The 4th stage is characterized by loss of critical centers that support breathing, heart rate, and blood pressure. Death soon ensues. Nelson 21st p1673-1674 Subacute sclerosing panencephalitis (SSPE) is a chronic complication of measles with a delayed onset and an outcome that is nearly always fatal. The diagnosis of SSPE can be eastablished through documentation of a complatible clinical course and at least one of the following supporting findings: 1. Measles antibody detected in CSF 2. Characteristic EEG findings 3. Typical histologic findings in and/or isolation of virus or viral antigen from brain tissue obtained by biopsy or postmortem examination"

Answer 102

B "Nelson 21st p3222 Table 621.1 CSF fluid findings in CNS disorders Normal - pressure <28 - WBC: <5, >=75% lymphocytes; in neonates <20 - protein: 20-45 - glucose >50 or 75% serum glucose Acute bacterial meningitis - elevated pressure - WBC: 100-10,000, PMN predominance - protein: 100-500 - glucose: decreased, usually <40 or <50% of serum glucose Partially treated bacterial meningitis - normal or elevated pressure - WBC 5-10,000, PMN usual but mononuclear cells may predominate if with extended pretreatment - protein: 100-500 - glucose: normal or decreased Viral meningitis - normal or slightly elevated pressure - WBC rarely >1000, PMN early, but mononuclear cells predominate through most of the course - protein: 50-200 - glucose: generally normal, may be decreased <40 in some viral diseases, particularly mumps TB meningitis - usually elevated pressure - WBC 10-500, PMN early, but lymphocytes predominate through most of the course - protein: 100-3000, may be higher in presence of block - glucose <50 in most cases, decreases with time if treatment is not provided Fungal meningitis - usually elevated pressure - WBC 5-500, PMn early but mononuclear cells predominate for most of the course - cryptococcal meningitis may lack pleiocytosis, coccidioidal meningitis may have eosinophilia - protein: 25-500 - glucose: <50, decreases with time if treatment is not provided Syphilis and leptospirosis - usually elevated pressure - WBC 50-500, lymphocytes predominate - protein: 50-200 - glucose usually normal "

Answer 103

B """Nelson 21st p2666 In children 0-14 yr old, the most common tumors are pilocytic astrocytomas and medulloblastoma/primitive neuroectodermal tumors (PNETS) In adolescents (15-19 yr), the most common tumors are pituitary/craniopharyngeal tumors, and pilocytic astrocytomas. During the first yr of life, supratentorial tumors predominate and most often include choroid plexus complex tumors and teratomas. In children 1-10 yr old, infratentorial tumors predominate because of the high incidence of juvenile pilocytic astrocytoma and medulloblastoma. After 10yr ofage, supratentorial tumors again predominate, with diffuse astrocytomas most common. Nelson 21st p2668 Table 524.3 Posterior fossa tumors of childhood 1. Medulloblastoma (35-40%) - heterogenously or homogenously enhancing 4th ventricular mass, may be disseminated; 65-85% survival, dependent on stage 2. Cerebellar astrocytoma (35-40%) - cerebrellar hemisphere mass, usually with cystic and solid components, 90-100% survival in totally resected pilocytic type 3. Brainstem glioma (10-15%) - diffusely expanded, minimally or partially enhancing mass in 80%, 20% more focal tectal or cervicomedullary lesion, >90% mortality in diffuse tumors 4. Ependymoma (10-15%) - usually enhancing 4th ventricular mass with cerebellopontine predeliction, >75% survival if totally resected 5. Atypical teratoid/rhabdoid (>5%, 10-15% of infantile malignant tumors) - as in medulloblastoma but more laterally extended; <20% survival"""

Answer 104

C """Nelsons 21st p2612 The most common cause of acute onset of thrombocytopenia in an otherwise well child is idiopathic thrombocytopenic purpura (ITP) The classic presentation of ITP is a previously healthy 1-4yr old child who has sudden onset generalized petechiae and purpura. There is a history of preceding viral infection 1-4 wk before the onset of thrombocytopenia. Findings on PE are normal, other than petechiae and purpura. Severe thrombocytopenia (PC <20 x 10^9/L) is common, and platelet size is normal or increased, reflective of increased platelet turnover. In acute ITP, Hgb, WBC and differential count should be normal. Bone marrow examination shows normal granulocytic and erythrocytic series, with characteristically normal or increased numbers of megakaryocytes. Some of the megakaryocytes may appear immature and reflect increased platelet turnover. Indications for bone marrow biopsy include an abnormal WBC count or differential or unexplained anemia, as well as history and PE findings suggestive of a bone marrow failure syndrome or malignancy. """

Answer 105

C """ Nelson 21st p2524 Table 482/2 Laboratory studies differentiating the most common microcytic anemias ``` IDA Decreased Hgb Decreased MCV Increased RDW Decreased RBC Decreased serum ferritin Increased total Fe binding capacity Decreased transferrin saturation Increased FEP Increased soluble transferrin receptor Decreased reticulocyte Hgb concentration ``` ``` ACD Decreased Hgb Normal-Decreased MCV Normal-Increased RDW Normal-Decreased RBC Increased serum ferritin Decreased total Fe binding capacity Decreased transferrin saturation Increased FEP Normal soluble transferrin receptor Normal-Decreased reticulocyte Hgb concentration ``` ``` Thalassemia Decreased Hgb Decreased MCV Normal or minimally increased RDW Normal-increased RBC Normal serum ferritin Normal total Fe binding capccity Normal transferrin saturation Normal FEP Normal soluble transferrin receptor Normal reticulocyte Hgb concentration""" ```

Answer 106

C """Nelson 21st p2561 G6PD deficiency Typically, hemolysis ensues in about 24-48hr after a patient has ingested a substance with oxidant properties. In severe cases, hemoglobinuria and jaundice result, the the Hgb concentration may fall precipitously. The onset of acute hemolysis usually results in a precipitous fall in hemoglobin or hematocrit. If the episode is severe, the Hgb binding globulins such as haptoglobin are saturated, and free hemoglobin may appear in the plasma and subsequently in the urine. Unstained or supravital preparations of RBCs reveal precipitated hemoglobin, or Heinz bodies. Also, the blood film may contain red cells with what appears to be a bite taken from their periphery (bite cells) and polychromasia (blueish larger RBCs) representing reticulocytosis. """

Answer 107

B "Nelson 21st p2712 Familial hemophagogytic lymphohistiocytosis (FHLH) and secondary hematophagic lymphohistiocytosis (HLH) have remarkably similar presentations, consisting of a generalized disease process, most often with fever (90-100%), maculopapular and/or petechial rash (10-60%), weight loss, and irritability. The initial clinical presentation can vary but is almost always very severe. Acute presentations include septic shock, acute respiratory distress and coma due to CNS infiltration. Other features that are frequently present result from bone marrow involvement and pancytopenia or hepatic dysfunction. "

Answer 108

A "Nelson 21st p2679 Table 525.1 Syndromes associated with neuroblastoma Horner syndrome - unilateral ptosis, myosis, and anhidrosis associated with a thoracic or cervical primary tumor; symptoms do not resolve with tumor resection Pepper syndrome - massive involvement of the liver with metastatic disease, with or without respiratory distress Hutchinson syndrome - limping and irritability in young child associated with bone and bone marrow metastasis Opsoclonus-myoclonus-ataxia syndrome - myoclonic jerking and random conjugate eye movements with or without cerebellar ataxia; likely immune mediated, may not resolve with tumor removal, and often exhibits progressive neuropsychologic sequelae; assocaited with biologically favorable and differentiated tumor Kerner-Morrison syndrome - intractable secretory diarrhea caused by tumor secretion of vasointestinal peptides Neurocristopathy syndrome - neuroblastoma associated with other neural crest disorders, including congenital hypoventilation syndrome or Hirschsprung disease ROHHAD - rapid onset obseity, hypothylamic dysfunction, hypoventilation, and autonomic dysregulation"

Answer 109

C "Nelson 21st p2653 Relapse occurs in the bone marrow in 15-20% of patients with ALL and carries the most serious implications, especially if it occurs during or shortly after completion of therapy. Intensive chemotherapy with agents not previously used in the patient followed by allogenic stem cell transplantation can result in long-term survival for some patients with bone marrow relapse. The incidence of CNS relapse has decreased to <5% since introduction of preventive therapy. CNS relapse may be discovered at a routine LP in an asymptomatic patient. Symptomatic patients with CNS relapse usually present with signs and symptoms of increased intracranial pressure and can present with isolated CN palsies. Treatment includes intrathecal medication and cranial or craniospinal irradiation. Testicular relapse occurs in <2% of boys with ALL, usually after completion of therapy. Such relapse occurs as painless swelling of 1 or both testes. Treatment includes systemic chemotherapy and possibly local irradiation. "

Answer 110

D """Nelson 21st p3140 Neurofibromatosis The neurofibromatoses are autosomal dominant disorders that cause tumors to grow on nerves and other systemic abnormalities. The disease is clinically diganosed when any two of the following 7 features are present 1. Six of more cafe au lait macules >5mm in greatest diameter in prepubertal individuals and >15mm in greatest diameter in postpubertal individuals. Cafe au lait macules are the hallmark of neurofibromatosis and are present in almost 100% of patients 2. Axillary or inguinal freckling consisting of multiple hyperpigmented areas 2-3mm in diameter 3. Two or more iris Lisch nodules, which are hamartomas located within the iris and are best identified by slit lamp examination 4. Two or more neurofibromas or one plexiform neurofibroma 5. Distinctive osseous lesion such as sphenoid dysplasia or cortical thinning of long bones with or without pseudoarthrosis 6. Optic gliomas Nelson 21st p3144 Table 614.4 Major features of tuberous sclerosis complex, Table 614.5 Minor features of tuberous sclerosis complex Major - cortical dysplasias (including tubers and cerebral white matter migration lines) - subependymal nodules - subependymal giant cell astrocytoma - facial angiofibromas or forehead plaque - ungual fibromas - hypomelanotic macules - Shagreen patch - multiple retinal nodule hamartomas - cardiac rhabdomyoma - renal angiomyolipoma - pulmonary lymphangioleiomyomatosis Minor - dental enamel pits - intraoral fibromas - retinal achromic patch - confetti skin lesions - nonrenal hamartoma - multiple renal cysts *The hallmark of TSC is the involvement of the CNS. Retinal lesions consist of hamartomas (elevated mulberry lesions or plaque like lesions) and white depigmented patches (similar to hypopigmented skin lesions). The characteristic brain lesion is a cortical tuber Nelson 21st p3146 Sturge-Weber Sturge-Weber syndrome is a segmental vascular neurocutaneous disorder with a constellation of symptoms and signs characterized by capillary malformation on the face (port-wine birthmark) and brain (leptomeninges) as well as abnormal blood vessels of the eye leading to glaucoma. Patients present with seizures, hemiparesis, stroke-like symptoms, headaches, and developmental delay. """

Answer 111

D "Nelson 21st p3495 Seborrheic dermatitis is a chronic inflammatory disease most common in infancy and adolescence that parallels the distribution, size, and activity of the sebaceous glands. The disorder may begin in the 1st month of life, and typically resolves by 1 yr. Diffuse or focal scaling and crusting of the scalp, sometimes called cradle cap, may be the initial, and at times, the only manifestation. A greasy, scaly, erythematous papular dermatitis, which is usually nonpruritic in infants, may involve the face, neck, retroauricular areas, axillae, umbilicus, and diaper area. "

Answer 112

A """Nelson 21st p3553 Staphylococcal scalded skin syndrome, also called Ritter disease, which occurs predominantly in infants and children younger than 5yr of age, includes a range of disease from localized bullous impetigo to generalized cutaneous involvement with systemic illness. Onset of the rash may be preceded by malaise, fever, irritability, and exquisite tenderness of the skin. Scarlatiniform erythema develops diffusely and is accentuated in flexural and periorificial areas. The conjunctivae are inflamed and occasionally become purulent. The brightly erythematous skin may rapidly acquire a wrinkled appearance, and in severe cases, sterile, flaccid blisters and erosions develop diffusely. Circumoral erythema is characteristically prominent, as is radial crusting and fussuring around the eyes, mouth, and nose. At this stage, areas of epidermis may separate in response to gentle shear force (Nikolsky sign). As large sheets of epidermis peel away, moist, glistening, denuded areas become apparent, initially in the flexures and subsequently over much of the body surface. """

Answer 113

B "Nelson 21st p3568 Molluscum contagiosum Discrete, pearly, skin-colored, smooth, dome-shaped papules vary in size from 1 to 5mm. They typically have a central umbilication from which a plug of cheesy material can be expressed. Molluscum contagiosum is a self-limited disease. The average attack lasts 6-9mos. However, lesions can persist for years, can spread to distant sites, and may be transmitted to others. Affected patients should be advised to avoid shared baths and towels until the infection is clear. Infection may spread rapidly and produce hundreds of lesions in children with atopic dermatitis or immunodeficiency. Immunotherapy with either candida or trichophyton antigen is the most coommonly used treatment. If lesions are limited in number, individual lesions can be treated with liquid nitrogen cryotherapy. For younger children, cantharidin may be applied to the lesions and covered with adhesive bandages to prevent unwanted spreading of the blistering agent. A blister forms at the site of the application, and the molluscum is removed with the blister. Cantharidin should not be used on the face. Imiquimod has not been proven more effective than placebo in RCTs. Molluscum is an epidermal disease and should not be overtreated such that scarring results. "

Answer 114

C """Nelson 21st p3577 With the exception of isotretinoin therapy, no evidence shows that early treatment alters the course of acne Little evidence shows that the ingestion of particular foods can trigger acne flares No evidence shows that preparations containing alcohol or hexachlorophene decrease acne, because surface bacteria are not involved in the pathogenesis. All topical preparations must be used for 6-8 wk before their effectiveness can be assessed. Retinoids may be used alone for mild acne, but combination therapy is frequently more effective. A popular and effective combination is use of benzoyl peroxide gel in the morning and a retinoid at night"""

Answer 115

A "Nelson 21st p3620 Osgood-Schlatter disease is an irritation of the patellar tendon at its insertion into the tibial tubercle or a traction apophysitis of the tibal tubercle growth plate. OS disease presents in slightly older patients with most symptomatic between the ages of 10 and 15 yr. This condition is most common in very physically active children. Anterior knee pain localized over the tibial tubercle is the most common patient complaint. Localized soft tissue swelling, along with an eventual firm and fixed increased prominence at the tibial tubercle, may occur with OS disease and may also be part of the initial complaint. There is typically no acute traumatic inciting event, and the history of an acute traumatic onset of symptoms should raise the possibility of a tibial tubercle fracture or patellar sleeve fracture. The pain is aggravated by sports activities and may often persist with regular daily activities and even at rest. Physical examination reveals point tenderness over the tibial tubercle and distal portion of the patellar tendon. Diagnosis is usually made clinically, but radiographs may reveal fragmentation of the tibial tubercle and soft tissue swelling. In most patients OS disease is a self limited process that resolves with rest. Patients are treated with increasing levels of activity restriction or immobilization to get them to a pain-free state before advancing their activities. "

Answer 116

A "Nelson 21st p3622 The management of ACL injury in this patient population can be challenging, and the severity of the ACL tear and degree of knee instability are important in directing treatment. Incomplete or partial ACL tears that still maintain a firm endpoint on examination can be treated nonperatively, and the patient and family's understanding and willingness to adhere to a protocol of braching and activity restriction are important factors in optimizing outcomes. For complete tears of the ACL, due to the risk of ongoing knee damage is stabilization of the knee is delayed, surgical reconstruction is usually the preferred treatment for patients who are physically, mentally, and emotionally capable of maintaing precautions and complying with the long rehabilitation course after the procedure. "

Answer 117

D "Nelson 21st p610 Once a submersion has occured, immediate institution of CPR efforts at the scene is imperative. The goal is to reverse the anoxia from submersion and limit secondary hypoxic injury after submersion. Common themes in children who have good recovery are a short duration of event and initiation of CPR as soon as possible, before arrival of emergency medical services. Initial resuscitation must focus on rapidly restoring oxygenation, ventilation, and adequate circulation. The airway should be clear of vomitus and foreign material, which may cause obstruction or aspiration. Abdominal thrusts should not be used for fluid removal, because many victims have a distended abdomen from swallowed water; abdominal thrusts may increase the risk of regurgitation and aspiration. In cases of suspected airway foreign body, chest compressions or back blows are preferable maneuvers. Recognition and treatement of hypothermia are the unique aspects of cardiac resuscitation in a drowning victim. Core temperature must be evaluated because moderate to severe hypothermia can depress myocardial function and cause arrhythmias. Wet clothing should be removed to prevent ongoing heat losses, although in the hemodynamically stable, rewarming should be initated in the controlled environment of the recieving ED or PICU. Unstable patients should be warmed to 34C, taking care not to overheat. "

Answer 118

C "Nelson 21st p2965 Hyperkalemia, hyponatremia, and hypoglycemia are prominent presenting signs of adrenal insufficiency in infants. Ketosis is not consistently present because infants generate ketones less well than older children. Hyperpigmentation is not usually seen because this takes weeks or months to develop, and orthostatic hypotension is obviously difficult to demonstrate in infants. Infants can become ill very quickly. There may only be a few days of decreased activity, anorexia, and vomiting before critical electrolyte imbalances develop. Nelson 21st p2973-2974 Affected females who are exposed in utero to high levels of androgens of adrenal origin have masculinized external genitalia. This is manifested by enlargement of the clitory and by partial or complete labial fusion. The vagina usually has a common opening with the urethra. The clitoris may be so enlarged that it resembles a penis; because the urethra opens below this organ, some affected females may be mistakenly presumed to be males with hypospadias and cryptorchidism. The severity of virilization is usually greatest in females with the salt-losing form of 21-hydroxylase deficiency. Nelson 21st p2975 Cortisol deficiency is treated with glucocorticoids. Treatment also suppresses excessive production of androgens by the adrenal cortex and thus minimizes problems such as excessive growth and skeletal maturation and virilization. Glucocorticoid treatment must be continued indefinitely in all patients with classic 21-hydroxylase deficiency. "

Answer 119

C "Severe dehydration, resuscitate via IV boluses of 20ml/kg pNSS Nelsons 21st p429 Table 70.1. Clinical evaluation of dehydration 1. Mild dehydration (<5% in an infant, <3% in an older child) - normal or increased pulse, decreased urine output, thirsty, normal physical findings 2. Moderate dehydration (5-10% in an infant, 3-6% in an older child) - tachycardia, little or no urine output, irritable/lethargic, sunken eyes and fontanel, decreased tears, dry mucus membranes, mild delay in elasticicty (skin turgor), delayed capillary refill (>1.5 sec), cool and pale 3. Severe dehydration (>10% in an infant, >6% in an older child) - peripheral pulses either rapid and weak or absent, decreased BP, no urine output, very sunken eyes and fontanel, no tears, parched mucuous membranes, delayed elasticity (poor skin turgor), very delayed capillary refil (>3 sec), cool and mottled, depressed consciousness"

Answer 120

A """Nelson 21st p543 If venous access is not available within approximately 1 min in a child with cardiopulmonary arrest, an IO needle should be placed in the anterior proximal tibia (with care taken to avoid traversing the epiphyseal plate). """

Answer 121

B """Nelson 21st p537 The definition of shock does not include hypotension, and it is important for care providers to understand that shock does not begin when blood pressure drops; it merely worsens and becomes more difficult (refractory) to treat once blood pressure is abnormal."""

2017 WISE with Rationale Flashcards

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