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Project - USMLE Step 3 > Pediatrics: Case Files > Flashcards

Pediatrics: Case Files Flashcards

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USMLE® Step 1 flashcards
1
Q

The following findings are consistent with what diagnosis?

  1. Mild coagulopathy
  2. Peripheral and cranial neuropathy
  3. Hepatosplenomegaly
  4. Pancytopenia
  5. Partial oculocutaneous albinism
  6. Frequent bacterial infections (usually Staph aureus)
  7. Progressive lymphoproliferative syndrome
A

Chediak-Higashi syndrome

The findings of neutropenia and giant lysosomes in neutrophils will confirm the diagnosis. Treatment includes prevention of infection with daily trimethoprim-Sulfamethoxazole and daily ascorbic acid.

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2
Q

Which heart condition is characterized by dyspnea that improves with squating?

A

Tetralogy of fallot

A “boot-shaped heart” is a characteristic radiographic finding.

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3
Q

What 2 vitamin/nutrient deficiencies cause muscle fasciculations?

A
  1. Vitamin D
  2. Calcium
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4
Q

What vitamin/nutrient deficiency causes hair loss?

A

Zinc

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5
Q

The following signs/symptoms are characteristic of what diagnosis?

  1. Lethargy
  2. Poor feeding
  3. Gravely ill appearance
  4. Respiratory distress
  5. Diminished tendon reflexes
  6. Jaundice
A

Kernicterus

This is a neurologic syndrome resulting from the deposition of unconjugated bilirubin in brain cells, especially basal ganglia, globus pallidus, putamen, and caudate nuclei. Less mature or sick infants have greater susceptibility.

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6
Q

What parasite can cause pernicious (B12 deficiency) anemia?

A

Diphyllobothrium latum

This fish tapeworm uses vitamin B12 for growth and egg production; as many as one million eggs per day may be produced. The parasite also inactivates the vitamin B12-intrinsic factor complex, inhibiting absorption fro the terminal ileum. The fish tapeworm is the longest tapeworm to infect humans, sometimes growing to more than 10 meters in length. Most infestations are asymptomatic, with megaloblastic anemia occuring in 2% to 9% of tapeworm infections. Risk factors include eating raw or undercooked fish. In North America, it is most commonly seen in the northern United States, Alaska, and Canada.

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7
Q

At what age is the diagnosis of delayed puberty made for

  1. Girls
  2. Boys
A
  1. Girls > 13 years of age
  2. Boys > 14 years of age

Possible causes include gonadal failure, chromosomal abnormalities, hypopituitarism, chronic disease, or malnutrition.

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8
Q

What changes on screening labs will be seen in renal tubular acidosis?

A

Low bicarbonate and Increase in chloride (normal anion gap metabolic acidosis)

Low pH (acidosis) will be present but basic screening labs will not assess pH.

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9
Q

The following findings are consistent with what diagnosis?

  1. Chronic pruritic dermatitis
  2. Recurrent staphylococcal infections (skin and respiratory tract)
  3. Markedly elevated serum IgE levels
  4. Eosinophilia
  5. Coarse facial features
A

Hyper-IgE (Job’s ) syndrome

Recurrent staphylococcal infections involving the skin, lungs, and joints, with other features such as distinctive facial appearance, dental abnormalities and bone fractures are essential for the diagnosis.

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10
Q

What is the average daily weight gain for a term infant in the first couple months of life?

A

20 to 30 grams

Adequate growth for a term infant requires approximately 100 to 120 cal/kg/day. Preterm infants require 115 to 130 cal/kg/day. VLBW (very low birth weight) infants require up to 150 cal/kg/day.

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11
Q

What is the treatment for juvenile idiopathic arthritis (JIA)

A
  1. Nonsteroidal anti-inflammatory drugs (NSAIDs)
  2. Methotrexate
  3. Glucocorticoids
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12
Q

What is the most common cardiac defect seen in Down Syndrome?

A

Endocardial cushion defect

50% of patient with Down Syndrome have an associated heart defect: 60% are endocardial cushion defects, 32% are ventricular septal defects, and 6% are tetrology of Fallot.

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13
Q

The following findings in a child under 4 years of age are consistent with what diagnosis?

  1. Acute onset pallor
  2. Acute onset irritability
  3. Decreased or absent urine output
  4. Stroke (may occur)
  5. Petechia (may occur)
  6. Edema (may occur)
  7. Diarrheal illness 1-2 weeks prior to above symptoms
A

Hemolytic-uremic syndrome

This is the most common cause of acute childhood renal failure. It develops in 5% to 8% of children with diarrhea caused by enterohemorrhagic E. coli (O157:H7). It is less commonly seen after Shigella, Salmonella, and Yersinia infections. The underlying process may be microthrombi, microvascular endothelial cell injury causing microangiopathic hemolytic anemia, and consumptive thrombocytopenia. Renal glomerular deposition of an unidentified material leads to capillary wall thickening and subsequent lumen narrowing.

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14
Q

The following signs/symptoms in a neonate between 2 and 5 days of birth is characteristic of what diagnosis?

  1. Puffy, tense eyelids
  2. Red conjunctivae
  3. Copious purulent ocular discharge
  4. Chemosis (swelling or edema of the conjunctivae)
A

Gonococcal ophthalmia

Gonococcal conjunctivitis usually occurs within 2 to 5 days of birth and is the most serious of the bacterial infections. Prompt aggressive topical and systemic treatment can prevent serious complications such as corneal ulceration, perforation, and blindness. In contrast, chlamydial conjunctivitis often presents 5 to 14 days after birth and the discharge is usually more watery in nature (mucopurulent) and less inflamed.

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15
Q

When is the risk of dangerous defects from maternal-fetal transmission of rubella the highest?

A

First trimester

Birth defects are less likely if the mother is infected later in pregnancy. The best way to protect infants is by maternal vaccination (with the live attenuated rubella vaccine) prior to conception.

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16
Q

The following are clinical features of abuse of which substance?

  1. Euphoria
  2. Decreased pain sensation
  3. Pinpoint pupils
  4. Hypothermia
  5. Vasodilation
  6. Possible respiratory depression
A

Opiates (heroin, morphine, codeine)

Retention time for Urine screening purposes

2 days

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17
Q

What is the treatment for organophosphate poisoning?

A
  1. Rapid decontamination by removing all clothing and washing all skin surfaces
  2. Atropine and pralidoxime both counter the effects of organophosphates
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18
Q

Moderate to severe dehydration in children should be treated with what?

A

Intravenous bolus of isotonic fluid

Oral rehydration is the initial treatment of choice in mild (3-5%) to moderate (6-9%) dehydration, but acts too slowly to adequately restore perfusion in patients with severe (10-15%) dehydration. Dextrose containing fluids are not used for initial fluid resuscitation.

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19
Q

The following are clinical features of abuse of which substance?

  1. Euphoria
  2. Grogginess
  3. Impaired short-term memory
  4. Talkativeness
  5. Vasodilation
  6. Respiratory depression (severe intoxication)
A

Alcohol

Retention time for screening purposes

  1. Blood: 7-10 hours
  2. Urine: 10-13 hours
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20
Q

When are pregnant women screened for gestational diabetes?

A

Between 24 and 28 weeks gestation

Earlier screening may be required in higher risk patients. Many of the congenital malformations associated with gestational diabetes are thought to result from hyperglycemia early in the pregnancy.

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21
Q

What changes in genitalia occur in boys during

  1. Tanner stage 2
  2. Tanner stage 3
  3. Tanner stage 4
A
  1. Testes increase in size and skin of scrotum reddens
  2. Penis grows in length
  3. Penis grows in width
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22
Q

The following characteristics are consistent with which diagnosis?

  1. Wide-set eyes
  2. Prominent nose
  3. Micrognathia
  4. Cleft palate
  5. Holosystolic murmur
  6. Immunodeficiency
A

DiGeorge syndrome (22q11 microdeletion)

This is characterized by decreased T-cell production and recurring infection. Findings include characteristic facies, and velocardiofacial defects suchs as VSD and tetrology of fallot. Thymic or parathyroid dysgenesis can occur, accompanied by hypocalcemia and seizures. Developmental speech delay are common in older patients.

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23
Q

Healthy infants in the first year of life require approximately how many kcals/kg/day?

A

120 kcal/kg/day

After the first year, they require roughly 100 kcal/kg/day. Failure to thrive children require an additional 50-100% to ensure adequate catch-up growth.

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24
Q

At what age is the diagnosis of precocious puberty made for

  1. Girls
  2. Boys
A
  1. Girls with secondary sexual characteristics before age 6-8 years of age (African American girls typically undergo puberty prior to Caucasian girls)
  2. Boys with secondary sexual characteristics before age 9 years of age
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25
Q

What usually presents in children under the age of 2 years and is characterized by severe colicky intermittent pain that on subsidence leave the patient calm or lethargic?

A

Intussusception

Peristalsis is still active and attempts to propel contents past the obstruction; this creates the episodes of severe colicky intermittent pain. If the bowel becomes ischemic with resultant areas of necrosis, blood may appear, and this will usually be in the first 12 hours of the obstruction. Only 60% of infants will have the classic “currant jelly” stool composed of blood and mucus. An air-contrast enema can be diagnostic as well as therapeutic, but a pediatric surgeon should be available in case perforation occurs or if reduction is unsuccessful.

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26
Q

What are 4 complications associated with Prader-Willi syndrome?

A
  1. Sleep apnea (70%)
  2. Type II diabetes mellitus (25%)
  3. Gastric distension/rupture
  4. Death by choking (8%)
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27
Q

Why must Goat’s milk be boiled before being fed to infants?

A

To prevent brucellosis

Goats are particularly susceptible to brucellosis.

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28
Q

What diagnosis is characterized by an infant with respiratory distress when not crying and no distress while crying?

A

Choanal atresia

Infants are obligate nose breathers until 4 months of age, but they can breathe through their mouths while crying.

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29
Q

What therapies are initiated by 2 months of age, 6 months of age, and at 2 years of age for sickle cell disease patients.

A
  • 2 months: Daily penicillin therapy
  • 6 months: Daily folate supplementation
  • 2 years: Meningococcal and 23-valent polysaccharide pneumococcal vaccines
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30
Q

Pneumonia in an intubated intensive care patient with central lines is more likely to be related to what?

A

Pseudomonas aeruginosa or fungal species

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31
Q

Besides administering the appropriate treatment to a patient with juvenile idiopathic arthritis (JIA), what complication should be looked for on physical exam?

A

Uveitis

Routine slit-lamp ophthalmic examination to monitor for uveitis is indicated, and findings include band keratopathy, posterior synechiae, and cataracts. Uveitis onset may be insidious, and may be the only initial manifestation of JIA. The disease is more common in young girls.

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32
Q

What presents in a similar fashion to acute lymphoblastic leukemia (with fever, arthralgias, arthritis, and a limp)) and may require bone marrow examination to differentiate it?

A

Juvenil rheumatiod arthritis (JRA)

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33
Q

During what time period was asbestos commonly used as a fire retardant on school ceilings?

A

1940’s-1970’s

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34
Q

What presents in a similar fashion to acute lymphoblastic leukemia (with fever, malaise, adenopathy, splenomegaly, and lymphocytosis) but is differentiated by atypical lymphocytes resembling leukemic lymphoblasts?

A

Infectious mononucleosis (Epstein-Barr virus)

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35
Q

What disorder results from a deficiency of the hypoxanthine-guanine phophoribosyl transferase enzyme?

A

Lesch-Nyhan syndrome

This deficiency results in marked hyperuricemia. It is characterized by self-mutilation, mental retardation, and extrapyramidal symptoms (eg, dystonia, choreoathetosis).

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36
Q

Failure to thrive in the setting of elevated chloride levels, low bicarbonate, and low potassium levels is indicative of what?

A

Renal tubular Acidosis

  • Patients with renal tubular acidosis, a common cause of organic failure to thrive, can have proximal tubule defects (type 2) caused by impaired tubular bicarbonate reabsorption or distal tubule defects (type 1) caused by impaired hydrogen ion secretion. Type 4 is also a distal tubule problem associated with impaired ammoniagenesis.*
  • Oral bicarbonate supplementation would be expected to correct the elevated chloride level, the low bicarbonate and potassium levels (although potassium supplements may be required), and poor growth.*
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37
Q

Isolated intestinal Salmonella infections do not require antibiotic treatment in children after what age?

A

3 months

After 3 months of age, treatment is not indicated. Antibiotics do not shorten the course of illness.

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38
Q

The following characteristics are consistent with which diagnosis?

  1. Severe nodulocystic acne
  2. Pectus excavatum
  3. Large teeth
  4. Prominent glabella
  5. Long face and fingers
  6. Poor fine motor skills
  7. Low-normal IQ
  8. Juvenile delinquent with explosive temper
A

XYY syndrome

Other findings include long and asymmetrical ears, and increased length vs. breadth for the hands, feet, and cranium. By age 5-6, they tend to be taller than their peers and begin displaying aggressive or defiant behavior.

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39
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Xerophalmia (eye fails to produce tears)
  2. Keratomalacia (softening and necrosis of the cornea)
  3. Conjunctivitis
  4. Poor growth
  5. Impaired resistance to infection
  6. Abnormal tooth enamel
  7. Night blindness
A

Vitamin A

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40
Q

The following findings are consistent with what diagnosis?

  1. Developmental delay
  2. Intrauterine growth retardation (including microcephaly)
  3. Cataracts
  4. Seizures
  5. Hepatosplenomegaly
  6. Prolonged neonatal jaundice
  7. Purpura at birth
A

Congenital cytomegalovirus or toxoplasmosis infection

Calcified brain densities of CMV typically are found in a periventricular pattern, whereas in toxoplasmosis, they are found scattered throughout the cortex.

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41
Q

The following characteristics are consistent with which diagnosis?

  1. Jaundice
  2. Hepatosplenomegaly
  3. Vomiting
  4. Hypoglycemia
  5. Seizures
  6. Lethargy
  7. Irritability
  8. Poor feeding and failure to thrive
  9. Aminoaciduria
  10. Liver failure
  11. Mental retardation
  12. And an increased risk of E. Coli sepsis
A

Uridyl transferase deficiency

This condition causes galactosemia and is managed with a lactose-free formula.

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42
Q

A systolic murmur at the left upper and midsternal borders, and a widely split second heart sound that does not vary with respiration are both suggestive of what heart lesion?

A

Atrial septal defect (ASD)

The murmur is caused by high-volume bllod flow from the right ventricl into the normal pulmonary artery; the murmur is not due to blood flowing across the ASD. A lower left sternal border diastolic murmur produced by increased flow across the tricuspid valve may be present. ASDs are well tolerated during childhood but can lead to pulmonary hypertension in adulthood or atrial arrhythmias from atrial enlargement.

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43
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Glossitis
  2. Pharyngeal ulcers
  3. Impaired cellular immunity
A

Vitamin B9 (Folate)

This is also associated with megaloblastic anemia.

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44
Q

Angelman syndrome results from what chromosomal abnormality?

A

Deletion of the maternal copy of chromosome 15q11-q13

These patients may have short stature and intellectual disability. However, other unique features include frequent smiling/laughter, hand-flapping, ataxia, and seizures.

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45
Q

The following characteristics are consistent with which diagnosis?

  1. Low-set and malformed ears
  2. Microcephaly
  3. Rocker- bottom feet
  4. Inguinal hernias
  5. Cleft lip and palate
  6. Micrognathia
A

Edward Syndrome (Trisomy 18)

Other features include:

  1. Clenched hands with overlapping digits
  2. Small palpebral fissures
  3. Prominent occiput
  4. Short sternum
  5. Cardiac defects (VSD, ASD, PDS, and coarctation of the aorta)
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46
Q

All female children with recurrent episodes of nocturnal vulvar itching should be examined for what?

A

Pinworms

All children with recurrent episodes of ianal or vulvar tching that is severe at night should be examined for pinworms. Diagnosis is made using the scotch-tape test. Treatment is empirical administration of mebendazole.

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47
Q

What defect causes Type 1 renal tubular acidosis (RTA)?

A

A defect in hydrogen ion secretion

These patients are acidotic, hypokalemic, and have an elevated urinary pH. In children, type 1 RTA is often a genetic disorder. Patients commonly develop nephrolithiasis.

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48
Q

What is the first step in investigating a neonate’s failure to pass meconium within 24 hours of birth?

A

Abdominal x-ray

This is always the first step as it can identify pneumoperitoneum from perforated bowel that would require emergency surgery. After pneumoperitoneum has been excluded, water-soluble contrast enema is the best next step in management.

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49
Q

What is the temporary management of choanal atresia employed while preparing for surgical intervention?

A

Endotracheal intubation

This bypasses the obstruction until surgery can be performed.

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50
Q

In an child, exercise-induced cyanosis and a systolic murmur that radiates to the back are suggestive of what diagnosis?

A

Pulmonary stenosis

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51
Q

What is the treatment for mild or moderate lead poisoning?

A

Calcium EDTA or Succimer

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52
Q

By what age should an infant double his/her birth length?

A

4 years

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53
Q

What is the treatment for a child with symptomatic lead toxicity?

A
  1. Admission to the hospital (removal from lead source)
  2. Stabilization
  3. Chelation with 2,3-dimercaptopropanol and calcium disodium ethylenediaminetetraacetic acid (CaEDTA)

Treatment for asymptomatic patients could involve simple investigation of the child’s environment, outpatient chelation, or immediate hospitalization.

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54
Q

The following findings in a young boy are consistent with what diagnosis?

  1. Eczema
  2. Thrombocytopenia
  3. Recurrent infections with encapsulated organisms
A

Wiskott-Aldrich syndrome

The initial manifestations often present at birth and consist of petechiae, bruises, bleeding from circumcision, or bloody stools. Immunologic findings include low IgM levels, high IgA and IgE levels, poor antibody responses to polysaccharide antigens, and moderately reduced number of T cells and platelets.

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55
Q

What are the first 3 tests that should be ordered when juvenile idiopathic arthritis (JIA) is suspected?

A
  1. Complete blood count (CBC)
  2. Erythrocyte sedimentation rate (ESR)
  3. Blood cultures

Leukocytosis, thrombocytosis, and anemia support the diagnosis of systemic JIA. The ESR should be elevated but the blood cultures should be negative. Evaluation of the synovial fluid may be necessary to rule out septic arthritis, particularly in the presence of exquisitely tender joints or when only a single joint is involved. Rheumatoid factor and ANA are usually negative in systemic JIA.

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56
Q

In addition to cardiac and GI defects, what 5 other conditions are associated with Down Syndrome?

A
  1. Hearing loss
  2. Strabismus
  3. Cataracts
  4. Nystagmus
  5. Congenital Hypothyroidism

Longer-term consequences of DS include obesity, increased risk of leukemia, atlantoaxial (cervical spine) instability, premature aging with an increased risk of Alzheimer disease.

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57
Q

What are the following developmental milestones for a 6 month old?

  1. Gross motor
  2. Fine motor
  3. Cognitive/linguistic/communication
  4. Social/Emotional
A
  1. Sit with no support/Roll over
  2. Look for dropped yarn/reach
  3. Turn to voice/Turn to rattling sound
  4. Feed self/Work for toy that is out of reach

50-90% of infants of this age pass this item/90% of infants this age pass this item

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58
Q

Why does physiologic jaundice (unconjugated hyperbilirubinemia) occur in approximately 60% of full-term infants and 80% of preterm infants?

A

Newborn infants have a limited ability to conjugate bilirubin and cannot readily excrete unconjugated bilirubin

Jaundice usually begins on the face and then progresses to the chest, abdomen, and feet. Full-term newborns usually have peak bilirubin concentrations of 5 to 6 mg/dL between the 2nd and 4th days of life.

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59
Q

The following are clinical features of abuse of which substance?

  1. Euphoria
  2. Increased motor activity
  3. Decreased fatigability
  4. Dilated pupils
  5. Tachycardia
  6. Hypertension and hyperthermia
  7. Sometimes associated with paranoid ideation
A

Cocaine

Retention time for Urine screening purposes

2-4 days

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60
Q

A deficiency in galactocerebrosidase characteristic of what diagnosis?

A

Krabbe’s disease

This is characterized by hyperacusis (intolerance of everyday sounds), irritability and seizures.

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61
Q

Beyond the newborn period and through approximately 5 years of age, lower respiratory tract infections of what etiology are common?

A

Viral pneumonia is common

Common viruses include respiratory syncitial virus (RSV), adenovirus, rhinovirus, influenza, and parainfluenza virus. Bacterial etiologies include pneumococcus and nontypeable Haemophilus influenzae.

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62
Q

What is the cause of 50% of all lower GI bleeds in children under the age of 2 years?

A

Meckel diverticulum

Meckel radionuclide scan is needed to confirm the diagnosis, but it has a high false-negative rate, so a diagnostic laparoscopy may be needed. Even if the bleeding stops, surgical excision of the mucosa is often done to prevent re-bleeding, obstruction, or diverticulitis.

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63
Q

What 4 active or historical heart conditions require antibiotic prophylaxis?

A
  1. History of endocarditis
  2. Prosthetic valve
  3. History of heart transplant
  4. Severe or partially repaired cyanotic congenital heart defect
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64
Q

Infants of diabetic mothers are at increased risk for what 4 congenital malformations?

A
  1. Congenital heart disease
  2. Neural tube defects
  3. Small left colon syndrome
  4. Caudal regression syndrome

Caudal regression syndrome is a rare congenital malformation found almost exclusively in the infant of a diabetic mother. It is characterized by hypoplasia of the sacrum and lower extremities.

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65
Q

Pneumonia in a patient with skin findings is more likely to be related to what?

A

Varicella-Zoster

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66
Q

What defect causes type 2 renal tubular acidosis (RTA)?

A

A defect in bicarbonate reabsorption in the proximal tubule

Fanconi syndrome is a common cause in children.

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67
Q

What is the most common cause of hematochezia in infants, children, and adolescents?

A

Anal fissure

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68
Q

What is the treatment for acetaminophen toxicity?

A

N-Acetylcysteine

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69
Q

As part of the routine well-child care of a sickle cell disease patient, what is often recommended to identify those individuals at high risk of having a stroke?

A

Transcranial doppler (TCD) ultrasonography

This identifies individuals with increased flow velocity in the large cerebral blood vessels and thus at high risk for developing a first stroke. Chronic transfusions amonge these high-risk children has resulted in reduced risk of first stroke.

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70
Q

What complications of sickle cell disease are most closely associated with each of the following?

  1. Fever
  2. Palor
  3. Abdominal pain and distension
  4. Lower respiratory disease
  5. Neurologic findings
A
  1. Sepsis
  2. Aplastic crisis
  3. Splenic sequestration
  4. Acute chest
  5. Stroke
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71
Q

The following characteristics are consistent with which diagnosis?

  1. Decreased tone at birth
  2. Upslanting palpebral fissures
  3. Epicanthal folds
  4. Redundant nuchal skin
  5. Fifth finger clinodactyly and brachydactyly
A

Down Syndrome (Trisomy 21)

A single transverse palmar crease is also characteristic of Down Syndrome. Confirmation of the diagnosis is made with a chromosome evaluation. After confirming the diagnosis, identification of potentially life-threatening features, including cardiac and GI anomalies is appropriate.

  1. Clinodactyly: Incurving of one of the digits (in DS, the fifth digit curves toward the fourth digit due to midphalanx dysplasia)
  2. Brachydactyly: Excessive shortening of hand and foot tubular bones resulting in a boxlike appearance
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72
Q

Infants receiving goat’s milk as a primary source of nutrition should be supplemented with what?

A
  1. Folate and vitamin B12 to prevent megaloblastic anemia
  2. Iron to prevent microcytic anemia
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73
Q

The following characteristics are consistent with which diagnosis?

  1. Wide-set eyes
  2. Flattened palpebral fissures
  3. Prominent epicanthus
  4. Flattened nasal bridge
  5. Mandibular micrognathia
  6. Large, low-set, cartilage-deficient ears
  7. White/grey spots in the periphery of the iris (Brushfield spots)
  8. Hypotonia at birth with a poor moro reflex
A

Potter’s disease

This condition is characterized by a lack of normal infant kidney, causing reduced urine output, oligohydramnios, and constraint deformities.

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74
Q

Why do lead levels tend to rise again following chelation therapy?

A

Lead deposits in bone, and chelation does not remove all lead from the body

A rising lead level following chelation therapy is thought to be a redistribution of lead stored in bone. Repeat chelation is only recommended if the blood lead level rebounds to 45 ug/dL or higher.

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75
Q

Approximately 12% of Down Syndrome infants have which GI anomaly?

A

Intestinal atresia

This is usually duodenal atresia.

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76
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Neuritis
  2. Edema
  3. Cardiac failure
  4. Hoarseness
  5. Anorexia
  6. Restlessness
  7. Aphonia (inability to produce voice)
A

Vitamin B1 (Thiamine)

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77
Q

What are 4 acyanotic heart lesions?

A
  1. Ventricular septal defect
  2. Atrial septal defect
  3. Atrioventricular septal defect
  4. Patent ductus arteriosus
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78
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Hemorrhagic manifestations
A

Vitamin K

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79
Q

Pneumonia in a patient with concomitant retinitis is more likely to be related to what?

A

Cytomegalovirus (CMV)

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80
Q

The following signs/symptoms in a neonate during the first few days of life are characteristic of what diagnosis?

  1. Irritability
  2. High-pitched cry
  3. Poor sleeping
  4. Tremors
  5. Seizures
  6. Sweating
  7. Sneezing
  8. Tachypnea
  9. Poor feeding
  10. Vomiting
  11. Diarrhea
A

Neonatal abstinence syndrome (NAS)

This is caused by infant withdrawal to opiates and usually presents in the first few days of life. Treatment for NAS includes symptomatic care to calm the infant and help the infant sleep, such as swaddling, providing small frequent feeds, and keeping the infant in a low stimulation environment. Morphine can be administered and systematically weaned to help control opiate withdrawal.

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81
Q

There is increased risk for early-onset neonatal group streptococcus (GBS) disease when delivery occurs how many hours after rupture of membranes?

A

>18 hours

Other factors associated with early-onset GBS infection include chorioamnionitis, intrapartum temperature >100.4F, mother with a previous infant with GBS infection, maternal age

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82
Q

Which vaccines should an infant recieve at the age of 4 months?

A

The second doses of the vaccinations recieved at 2 months, with the exception of the Hepatitis B vaccine

This includes Rotavirus (RV), Dtap, Haemophilus Influenzae B (Hib), Pneumococcal conjugate (PCV13), Inactivated poliovirus (IPV)

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83
Q

The following characteristics are consistent with which diagnosis?

  1. Diarrhea/defecation
  2. Urination
  3. Miosis
  4. Brachycardia
  5. Bronchorrhea
  6. Emesis/excitation of muscles
  7. Lacrimation
  8. Salivation
A

Organophosphate poisoning

Organophosphates are commonly found in pesticides and bind irreversibly to cholinesterase of neurons and erythrocytes, as well as to liver pseudocholinesterase. The common finding is failure to terminate the effects of acetylcholine at the receptor sites. Signs and symptoms of cholinergic excess are often remembered with the mneumonic “dumbbells”.

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84
Q

How is the diagnosis of chronic granulomatous disease made?

A

Nitroblue tetrazolium (NBT) slide test, flow cytometry, or cytochrome C reduction

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85
Q

A deficiency in glucocerebrosidase is characteristic of what diagnosis?

A

Gaucher’s disease

It is characterized by hepatosplenomegaly, anemia, leucopenia, and thrombocytopenia. It is not characterized by a cherry red macula (as seen in Tay-Sach’s disease and Niemann-Pick’s disease).

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86
Q

Deficiency of sphingomyelinase is characteristic of what diagnosis?

A

Niemann-Pick’s disease

This is characterized by a cherry red macula, protruding abdomen, hepatosplenomegaly, lymphadenopathy, and regression of developmental milestones.

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87
Q

Recurrent infections with the following organisms is characteristic of what diagnosis?

  1. Staph Aureus
  2. Serratia marcescens
  3. Burkholderia cepacia
  4. Klebsiella
  5. Aspergillus
A

Chronic granulomatous disease

Patients are susceptible to catalase-positive bacteria.

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88
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Depression (Rare)
  2. Hypotension
  3. Muscle weakness
  4. Abdominal pain
A

Vitamin B5 (Pantothenic acid)

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89
Q

What comprises approximately 40% of all pediatric malignancies?

A

Leukemia

75% are acute lymphoblastic leukemia (ALL), 20% are myeloblastic leukemia (AML). The clinical manifestations of AML and ALL are similar.

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90
Q

What class of antiretroviral do the following drugs belong to?

  1. Indinavir
  2. Nelfinavir
  3. Ritonavir
A

Protease inhibitors

91
Q

What are the following developmental milestones for a 2 month old?

  1. Gross motor
  2. Fine motor
  3. Cognitive/linguistic/communication
  4. Social/Emotional
A
  1. Head up 45 degrees/Lift head
  2. Follow past midline/Follow to midline
  3. Laugh/Vocalize
  4. Smile spontaneously/Smile responsively

50-90% of infants of this age pass this item/90% of infants this age pass this item

92
Q

The following are clinical features of abuse of which substance?

  1. Euphoria
  2. Increased sensual awareness
  3. Increased psychic and emotional energy
  4. Nausea
  5. Teeth grinding
  6. Tachycardia
  7. Blurred vision
  8. Jaw clenching
  9. Anxiety
  10. Panic attacks
  11. Psychosis
A

Methamphetamines and Ecstasy

Retention time for Urine screening purposes

2 days

93
Q

What 3 findings may be present in an infant exposed to methyl mercury in utero?

A
  1. Low birth weight
  2. Microcephaly
  3. Seizures

They also dysplay significant developmental delay and can have vision and hearing impairments.

94
Q

What vitamins/nutrients are higher in cow’s milk than goat’s milk?

A
  1. Sodium
  2. Vitamin D
  3. Iron
  4. Folate
  5. Vitamin B12
95
Q

What is the likely diagnosis in an infant younger than a year of age who has an asymptomatic right upper quadrant abdominal mass and pallor, but no jaundice?

A

Neuroblastoma

96
Q

At what age do children often become picky eaters, often causing their growth rates to plateau?

A

Between 18-30 months of age

Effective measures include calm counseling of parents to provide nutrition, avoid force-feeding, and avoid providing snacks; and close follow-up.

97
Q

How long after initial group A strep pharyngitis does acute post-streptococcal glomerulonephritis occur?

A

1-2 weeks

The interval between group A strep impetigo and acute post-streptococcal glomerulonephritis is 3-6 weeks. Symptom onset is abrupt. Although almost all patients have microscopic hematuria, only 30%-50% develop gross hematuria. In addition, 85% present with edema and 60%-80% develop hypertension.

98
Q

Breast milk is considered the ideal human infant food because it contains complete nutrition with the exception of what?

A

Vitamin D and fluoride

In addition it has antimicrobial properties and offers psychological advantages to mothers and infants.

99
Q

What is the treatment of iron poisoning?

A

Intavenous deferoxamine

Deferoxamine binds ferric iron and allows it to be excreted in the urine.

100
Q

What are 4 positive prognostic factors for acute lymphoblastic leukemia?

A
  1. Female gender
  2. Age between 1 and 10 years
  3. Lower leukocyte counts (especially below 50,000/mm3)
  4. B-cell immunophenotype (T-cell immunophenotype has worse prognosis)
101
Q

Pneumonia in a patient with exposure to sheep or cattle is more likely to be related to what?

A

Coxiella brunetti

102
Q

What is the likely heart lesion in an asymptomatic child with a harsh, left lower sternal border holosystolic murmur?

A

A small ventricular septal defect (VSD)

The murmur of a large VSD may be less harsh because of the absence of significant pressure gradient across the defect.

103
Q

After X-rays are taken, what is the next step in investigating a newborn’s failure to pass meconium in the first 24 hours of birth?

A

Water-soluble contrast enema

The appearance of a microcolon on contrast enema should raise concern for meconium ileus (virtually pathognomonic for cystic fibrosis). If contrast enema demonstrates a transition zone between a narrow rectosigmoid and a dilated megacolon, workup for congenital aganglionic megacolon (Hirschsprung disease) should be pursued.

104
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Osteomalacia
  2. Infantile tetany
  3. Rickets (with elevated serum phosphatase levels appearing before bone deformities)
A

Vitamin D

105
Q

What is the most frequently diagnosed neoplasm in infants?

A

Neuroblastoma

More than half of patients present before 2 years of age. It may present as a painless mass in the neck, chest, or abdomen. Fever, pallor, and weight loss are frequent presenting symptoms.

106
Q

The following characteristics are consistent with which diagnosis?

  1. Short stature
  2. Hypertension
  3. Low posterior hairline
  4. Prominent and low-set ears
  5. Excessive nuchal skin
  6. Primary amenorrhea
A

Turner Syndrome (XO)

Other features include widely spaced nipples and a broad chest, cubitus valgus (increased carrying angle of arms), edema of the hands and feet in the newborn period, congenital heart disease (coarctation of the aorta or bicuspid aortic valve), horseshoe kidney, short 4th metacarpal/metatarsal, hypothyroidism, decreased hearing, and normal mental development.

107
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

Younger patients

  1. Irritability
  2. Convulsions
  3. Anemia

Older patients

  1. Dermatitis
  2. Glossitis
  3. Cheilosis
  4. Peripheral neuritis
A

Vitamin B6 (Pyridoxine)

108
Q

What is the treatment for Hemolytic-uremic syndrome?

A

Supportive measures

Some children require dialysis. Most children recover and regain normal renal function; all are followed after infection for hypertension and chronic renal failure.

109
Q

What are the following developmental milestones for a 9 month old?

  1. Gross motor
  2. Fine motor
  3. Cognitive/linguistic/communication
  4. Social/Emotional
A
  1. Pull to a stand/Stand holding on to something
  2. Take 2 cubes/Pass cube
  3. Dada and mama, nonspecifically/Single syllables
  4. Wave bye-bye/Feed self

50-90% of infants of this age pass this item/90% of infants this age pass this item

110
Q

Recurrent painless gross hematuria, frequently associated with an upper respiratory tract infection, is typical of what diagnosis?

A

IgA nephropathy

These patients may develop chronic renal disease over decades. If proteinuria, hypertension, or impaired renal function were found, a biopsy would be necessary.

111
Q

The following are clinical features of abuse of which substance?

  1. Sedation
  2. Pinpoint pupils
  3. Hypotension
  4. Bradycardia
  5. Hypothermia
  6. Hyporeflexia
  7. CNS and respiratory depression
A

Barbiturates

Retention time for Urine screening purposes

  1. 1 day for short-acting agents
  2. 2-3 weeks for long-acting agents
112
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Easy bruising w/ petechiae
  2. Gum disease
  3. Loosening of teeth
  4. Poor wound healing
  5. Emotional changes
A

Vitamin C (Ascorbic acid)

113
Q

Airway inflammation in asthma is a result of what?

A

Mast cell activation

An immediate immunoglobulin E response to environmental triggers occurs within 15 to 30 minutes and includes vasodilation, increased vascular permeability, smooth-muscle constriction, and mucus secretion. 2-4 hours after this acute response, a late-phase reaction (LPR) begins. This is characterized by infiltration of inflammatory cells into the airway parenchyma, and is responsible for the chronic inflammation seen in asthma. Airway hyperresponsiveness may persist for weeks after the LPR.

114
Q

Which sequela of Group A strep infection is not prevented by antibiotic administration?

A

Acute post-streptococcal glomerulonephritis (APSGN)

Antibiotic use during the initial group A strep infection may reduce the subsequent rheumatic fever risk, yet has not been shown to prevent APSGN. The nephritis risk after infection with a nephritogenic strain of Group A strep remains 10%-15%.

115
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Hemolytic anemia in premature infants
A

Vitamin E

116
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Dermatitis
  2. Seborrhea
  3. Anorexia
  4. Muscle pain
  5. Pallor
  6. Alopecia
A

Vitamin B7 (Biotin)

117
Q

The following characteristics are consistent with which diagnosis?

  1. Macrocephaly
  2. Long face
  3. High arched palate
  4. Large ears
  5. Macroorchidisim (after puberty)
  6. Mental retardation
A

Fragile X syndrome

This is the most common form of inherited mental retardation. It usually occurs in boys.

118
Q

The following signs/symptoms are characteristic of what diagnosis?

  1. Leg/joint pain
  2. Fever
  3. Petechia
  4. Hepatosplenomegaly
  5. Anorexia
  6. Irritability
  7. Lethargy
  8. Pallor
  9. Bleeding
A

Acute lymphoblastic leukemia (ALL)

Most of the signs and symptoms of ALL result from either replacement of normal bone marrow components with clonal proliferation of a single lymphoblast that has undergone malignant transformation, or from infiltrates of extramedullary sites by these malignant lymphoid cells. Rapid diagnosis and referral to a pediatric cancer center can increase survival.

119
Q

What class of antiretroviral do the following drugs belong to?

  1. Efavirenz
  2. Nevirapine
  3. Delavirdine
A

Non-Nucleoside reverse transcriptase inhibitors (NNRTI)

120
Q

An adolescent with complaints of a dry cough, low-grade fever, and fatigue over the course of 2 weeks is consistent with what diagnosis?

A

Mycoplasm pneumoniae infection

This is also known as walking pneumonia. The incubation period for Mycoplasma is 5-7 days, and most symptoms are noted during the second to third week of infection. Hemolysis occurs as antibodies attach to red blood cells, prompting reticulocyte production. Chest x-ray could be normal or show an interstitial pattern, effusion, or atelectasis.

121
Q

What is the most common rheumatologic disorder in children?

A

Juvenile idiopathic arthritis (JIA)

122
Q

Breast-fed infants of mothers who adhere to a strict vegan diet are at risk for what vitamin deficiency?

A

Vitamin B12

Vitamin B12 deficiency can also occur when the terminal ileum is absent or when infectious or inflammatory conditions compromise intestinal function.

123
Q

What are the common causative organisms of otitis externa?

A
  1. Pseudomonas species
  2. Staph aureus
  3. Candida or aspergillus (occaisionally)
124
Q

What vitamins/nutrients are higher in goat’s milk than cow’s milk?

A
  1. Potassium
  2. Chloride
  3. Linoleic acid
  4. Arachidonic acid
125
Q

The following signs/symptoms in a child are characteristic of what diagnosis?

  1. Anorexia
  2. Hyperirritability
  3. Altered sleep pattern
  4. Decreased play
  5. Developmental regression (especially with speech)
  6. Abdominal complaints (vomiting, intermittent pain, constipation)
A

Lead toxicity

Permanent, long-term consequences include learning and cognitive defecits and aggressive behavior.

126
Q

The following characteristics are consistent with which diagnosis?

  1. Small for gestational age
  2. Cutis aplasia of the scalp
  3. Polydactyly
  4. Micropthalmia
  5. Omphalocele
  6. Microcephaly with sloping forehead
A

Patau Syndrome (Trisomy 13)

Other common features include:

  1. Holoprosencephaly (failure of growth of the forebrain)
  2. Cleft lip or palate
  3. Postaxial polydactyly
  4. Flexed and overlapping fingers
  5. Cardiac defects (VSD, ASD, PDA, and dextrocardia)

Cutis aplasia: Missing portion of skin and hair

127
Q

Absence of a red reflex may indicate which underlying abnormalities?

A
  1. Cataracts
  2. Glaucoma
  3. Retinoblastoma
  4. Chorioretinitis
128
Q

At what tanner stage in girls do the nipple and areola form a separate mound, protruding from the breast?

A

Breast tanner stage 4

The areola rejoins the breast contour and development is completed around age 12.5-18.5 years.

129
Q

What presents in a similar fashion to acute lymphoblastic leukemia (with bruising and petechia) but is differentiated by the absence of anemia, leukocyte disturbances, and hepatosplenomegaly?

A

Idiopathic thrombocytopenic purpura (ITP)

130
Q

The sudden onset of respiratory symptoms in a previously healthy infant, particularly in association with fever, is most consistent with what diagnosis?

A

Bronchiolitis

Initial treatment is with oxygen and a trial of nebulized alvuterol or epinephrine. A bllod gass measurement should be obtained immediately for any patient who presents in severe respiratory distress. Chest radiographs in infants with bronchiolitis typically show hyperinflated lungs with areas of atelectasis. Respiratory syncytial virus and influenza A are common causes of bronchiolitis in infants in the wintertime.

131
Q

What should be given to a term neonate with known coarctation of the aorta who develops sudden respiratory distress sometime in the first 3 weeks of life?

A

Prostaglandins

The symptoms are likely due to the ductus arteriosus begining to close. The ductus usually begins to close during the first or second day of life in term infants and is fully closed by 3 weeks. Prostaglandin therapy can reverse this process in the short-term. Surgery or catheterization techniques provide definitive repair.

132
Q

By what age should an infant double and triple his/her birth weight?

A
  1. Double: 4-5 months
  2. Triple: 12 months

Infants should regain their birth weight by 2 weeks.

133
Q

What is the treatment for otitis externa?

A

Topical mixture of polymyxin and corticosteroids

134
Q

When does PCP prophylaxis in the form of TMP-SMX begin for HIV-positive infants?

A

Approximately 6 weeks of age

135
Q

Prader-Willi syndrome results from what chromosomal abnormality?

A

Deletion of the paternal copy of chromosome 15q11-q13

This results in poor suck and feeding problems in infancy followed by a life of compulsive binge-eating and obesity-related problems.

136
Q

Respiratory distress syndrome, polycythemia with hyperviscosity syndrome, hypocalcemia, hypomagnesemia, and hyperbilirubinemia are all possible neonatal sequelae of what?

A

Gestational diabetes

Fetal hyperinsulinism is a response to poorly controlled maternal hyperglycemia resulting in fetal macrosomia and increased fetal oxygen requirements. These two factors can make the birth process difficult and result in neonatal distress. Many of the congenital malformations associated with gestational diabetes are thought to result from hyperglycemia early in the pregnancy.

137
Q

The following signs/symptoms are characteristic of what diagnosis?

  1. Ataxia
  2. Tremor
  3. Dysarthria
  4. Memory loss
  5. Altered sensorium (Including vision, hearing, smell, and taste)
  6. Dementia
A

Methyl mercury toxicity

This ultimately will cause death.

138
Q

What is the most common heart lesion in children?

A

Ventricular septal defect (VSD)

It occurs in 3-6 of every 1000 live term births. The majority of VSDs occur in the membranous portion of the septum, and small VSDs with minimal left-to-right shunts are the most common.

139
Q

What must breast milk be supplemented with when an infant is 4-6 months of age?

A

Iron

Iron levels are low in breast milk, but highly bioavailable and do not require supplementation until 4-6 months of age.

140
Q
  1. It is important to never give regular cow’s milk to an individual under what age?
  2. Why?
A
  1. 12 months of age
  2. Infants do not tolerate the protein mixture of unaltered cow’s milk and may develop colitis, causing microscopic bleeding and gradually worsening anemia.
141
Q

What is the first step in management of a neonate with a heart rate < 60bpm immediately after birth?

A

Endotracheal intubation with positive pressure ventilation of 100% O2

142
Q
  1. Where is intrisic factor produced?
  2. Where is it absorbed?
A
  1. Gastirc antrum
  2. Terminal ileum
143
Q

What differentiates a benign pulmonary flow murmur from a pathologic pulmonary murmur?

A

A murmur that radiates and/or has an audible click is more likely pathologic

Signs/symptoms of cardiac disease (digital clubbing, cyanosis, and exercise intolerance) are all suggestive of pathology.

144
Q

What is characterized by nocturnal hypoglycemic episodes that are manifested as night terrors, headaches, or early morning sweating and then presents a few hours later with hyperglycemia, ketonuria, and glucosuria?

A

Somogyi phenomenon

The counter-regulatory hormones cause hyperglycemia in response to hypoglycemia. The causes of Somogyi phenomenon include excess or ill-timed insulin, missed meals or snacks, and inadvertent insulin administration. It occurs in diabetes mellitus type 1 and is less common in diabetes mellitus type 2.

145
Q

What condition is characterized as a disorder of movement or posture resulting from an insult to, or an anomaly of, the central nervous system.

A

Cerebral palsy

Most children with cerebral palsy have no identifiable risk factors for the disorder.

146
Q

What is the treatment for an infant with chlamydial conjunctivitis or pneumonia presenting after the first 2 months of life?

A

Oral Sulfisoxazole for 2 weeks

147
Q

What are two significant risk factors for sudden infant death syndrome (SIDS)?

A
  1. Prone sleep position (Babies should sleep on their backs)
  2. Cigarette smoke exposure
148
Q

What presents in a similar fashion to acute lymphoblastic leukemia (with pancytopenia and fever) but is differentiated by the absence of lymphadenopathy, arthralgias, bone pain, and hepatosplenomegaly?

A

Aplastic anemia

149
Q

Which 6 vaccines are given at 2 months of age?

A
  1. Diptheria, tetanus, and acellular pertussis (Dtap)
  2. Haemophilus Influenzae type B (Hib)
  3. Inactivated Poliovirus (IPV)
  4. Pneumococcal conjugate (PCV13)
  5. Rotavirus (RV)
  6. 2nd dose of Hepatitis B (HepB)
150
Q

Which disorder is characterized by recurrent cutaneous, gastrointestinal, or pulmonary infections that occur with opportunistic organisms such as cytomegalovirus (CMV) and pneumocystis pneumonia (PCP)?

A

Severe combined immunodeficiency (SCID)

Serum immunoglobulins and T cells are often markedly diminished or absent in SCID. Thymic dysgenesis is also seen. Death typically occurs in the first 12-24 months of life unless bone marrow transplantation is performed.

151
Q

How many weeks of cromolyn or nedocromil therapy does it take before the drugs become effective?

A

2-4 weeks

These drugs function by reducing the immune response to allergen exposures. They are only successful in 75% of patients.

152
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Photophobia
  2. Cheilosis (inflammation of the corners of the mouth)
  3. Glossitis
  4. Corneal Vascularization
  5. Poor growth
A

Vitamin B2 (Riboflavin)

153
Q

What are the following developmental milestones for a 4 month old?

  1. Gross motor
  2. Fine motor
  3. Cognitive/linguistic/communication
  4. Social/Emotional
A
  1. Roll over/Sit with head steady
  2. Follow to 180 degrees/Grasp rattle
  3. Turn to rattling sound/Laugh
  4. -/Regard own hand

50-90% of infants of this age pass this item/90% of infants this age pass this item

154
Q

DNA testing of someone with Cystic fibrosis will show what?

A

Mutations in the long arm of chromosome 7

The most common such mutation is a single phenylalanine deletion at amino acid 508 (Delta508). The disease shows autosomal recessive inheritance.

155
Q

Pneumonia in a patient with recent exposure to stagnant water is more likely to be related to what?

A

Legionella pneumophila

156
Q

Cough and increased respiratory effort in an afebrile infant with eye discharge are findings consistent with what?

A

Chlamydia trachomatis

Transmission typically occurs during vaginal delivery and infants typically present with respiratory infection in the second month of life (but symptoms can be seen as early as the second week).

157
Q

Which age group is the cause of hematochezia most often a life-threatening condition?

A

Newborns

In infants and children the cause of hematochezia is more often benign.

158
Q

Pneumonia in a patient with chronic lung disease is more likely to be related to what?

A

Pseudomonas aeruginosa or Aspergillus

159
Q

What is the appropriate approach to mammary gland enlargement and non-purulent vaginal discharge in a newborn?

A

Observation and routine care

These are common findings in newborn infants. They are benign, transitory events which represent the infant’s physiologic target-organ response to transplacentally acquired maternal hormones (estrogens). No work-up is necessary, and routine care should be provided to the infant.

160
Q

Pneumonia in a patient with recent travel to the southwestern United States is more likely to be related to what?

A

Coccidioides immitis

161
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Pernicious anemia
  2. Neurologic deterioration
  3. Methylmalonic acidemia
A

Vitamin B12 (Cobalamin)

162
Q

Lower respiratory tract infections in the first few days of life are more commonly caused by what?

A

Group B streptococcus (GBS) and Enterobacteriaceae

Other possibilities include staphylococcus aureus, streptococcus pneumoniae (pneumococcus), and Listeria monocytogenes. In the newborn with pneumonea, broad-spectrum antimicrobials (ampicillin + gentamicin or cefotaxime) are customarily prescribed.

163
Q

What is the management of transposition of the great arteries (TGA)?

A
  1. Prostaglandins to maintain a patent ductus arteriosus
  2. Creation of an atrial septum via cardiac catheterization, which provides immediate symptom palliation
  3. Definitive surgical care often occurs in the first 2 weeks of life

Postoperative stenosis at the repair sites is a potential long-term complication.

164
Q

The following characteristics are consistent with which diagnosis?

  1. Developmental delay
  2. Immature/insecure behavior
  3. Gynecomastia
  4. Hypogonadism
  5. Long limbs
A

Klinefelter Syndrome (XXY)

165
Q

What usually presents in boys prior to 3 months of age and ischaracterized by more frequent stools and blood streaked stools?

A

Allergic proctocolitis

This is induced by allergy to the protein in cow’s milk. Standard infant formulas are composed of this protein. Soy protein is similar in structure so cross-allergy often exists. The protein can cross over into breast milk so the breast feeding mother must either remove cow’s milk from her diet or the infant must be switched to an elemental formula (containing amino acids rather than proteins). If the inciting protein is not removed from the diet, the infant can progress to enterocolitis with resulting severe diarrhea, malabsorption, vomiting, and dehydration. There may be a family history of atopy.

166
Q

What would present in a neonate with a negative coombs test, a normal (or low) hemoglobin, a normal (or mildly elevated) reticulocyte cound, and prolonged hyperbilirubinemia?

A

Gilbert syndrome

Gilbert syndrome is a benign condition that does not require treatment; it has a prevalence rate of 2% to 10%. It is a genetic disorder of bilirubin metabolism, involving a decrease in the activity level of uridine diphosphate (UDP)-glucuronosyltransferase 1A1.

167
Q

Why is there often increased wheezing on auscultation of an asthmatic following treatment with albuterol?

A

Lung areas that were previously closed are no opening, allowing additional airflow

168
Q

How is the diagnosis of leukemia made?

A

Examination of the bone marrow

Leukemic blasts may not be seen in the peripheral blood smear, so leukemia is established by examination of bone marrow, most commonly aspirated from the posterior iliac crest. Normal marrow contains less than 5% blasts. A minimum of 25% blasts confirms the diagnosis of leukemia.

169
Q

What should be considered in any patient with recurrent or unusual lymphadenitis, hepatic abscesses, osteomyelitis at multiple sites, or unusual infections with catalase-positive organisms?

A

Chronic granulomatous disease

Catalase-positive organisms include Staph Aureus, Klebsiella, Aspergillus, Serratia marcescens, and Burkholderia cepacia.

170
Q

In a neonate, O2 saturation measured from the lower extremity that is >3-5% higher than O2 saturations measured from the upper extremity or from the earlobe are suggestive of what diagnosis?

A

Ductus dependent heart lesion

This occurs because oxygenated blood from a patent ductus arteriosus is often down stream of the outflow tract to the head or upper extremity.

171
Q

What is the treatment of choice for idiopathic central precocious puberty?

A

Gonadotropin releasing hormone (GnRH) agonists

These analogues desensitize the gonadotropic cells of the pituitary to the stimulatory effect of GnRH produced by the hypothalamus. Nearly all boys and most girls with rapidly progressive precocious puberty are candidates for treatment. Girls with slowly progressive puberty do not seem to benefit from GnRH agonist therapy in adult height prognosis.

172
Q

What presents with a delay in passing meconium after birth and can progress to bloody stools and even diarrhea?

A

Hirschsprung disease

It can progress to toxic megacolon and enterocolitis with is when it will present with bloody stools and diarrhea.

173
Q

What are 3 risk factors for developing acute lymphoblastic leukemia (ALL)?

A
  1. Age 2-4 yrs
  2. Male gender
  3. Chromosomal abnormalities such as Down syndrome and fanconi anemia
174
Q

A breast-feeding vegan should supplement her infant’s or toddler’s diet with what?

A

Vitamin B12

This is to prevent methylmalonic acidemia and trace minerals.

175
Q

What is the treatment for chronic granulomatous disease?

A

Daily Trimethoprim-Sulfamethoxazole and gamma-interferon 3 times per week.

Bone marrow transplantation is experimental but curative.

176
Q

Pneumonia in a patient with a history of spelunking or working on a farm east of the Rocky Mountains is more likely to be related to what?

A

Histoplasma capsulatum

177
Q

Meconium ileus in the newborn period is nearly pathognomonic for what?

A

Cystic fibrosis

178
Q

The pediatric patient older than approximately 5 years of age with lower respiratory tract infection is typically infected with what?

A

Mycoplasma

Antibiotics in this age group are directed toward mycoplasma and typical bacteria (pneumococcus). Treatment options include macrolides (azithromycin), or cephalosporins (ceftriaxone or cefuroxime).

179
Q

What test can be done to differentiate between maternal and fetal hemoglobin?

A

APT test

This test works because infant’s blood is alkali-resistant.

180
Q

Extremely elevated WBC counts (~50,000 cells/mm3) in an immunocompromised individual is consistent with which diagnosis?

A

Leukocyte adhesion deficiency (LAD)

LAD is an inheritable disorder of leukocyte chemotaxis and adherence characterized by recurrent sinopulmonary, oropharyngeal, and cutaneous infections with delayed wound healing.

181
Q

Pneumonia in a patient with a fungal ball on chest radiograph is more likely to be related to what?

A

Aspergillus

182
Q

When does sudden infant death syndrome (SIDS) typically occur?

A

Between 1 and 5 months

Peak incidence is between 2 and 4 months and it occurs more frequently in winter. It occurs more frequently in African-American and Native-American infants.

183
Q

The following findings in a neonate are consistent with what diagnosis?

  1. Bilious emesis
  2. Abdominal distension
  3. “Triple bubble” sign and gasless colong on x-ray
A

Jejunal atresia

Risk factors include prenatal exposure to cocaine and other vasoconstrictive drugs.

184
Q

What potentially life-threatening complication of diabetic ketoacidosis treatment presents as headache, personality changes, vomiting, and decreased reflexes?

A

Cerebral edema

185
Q

The following signs in a newborn are suggestive of what diagnosis?

  1. Immediate respiratory distress
  2. Scaphoid (concave) abdomen
  3. Cyanosis
  4. Heart sounds displaced to the right of the chest
A

Diaphragmatic hernia

The first step in management is endotracheal intubation.

186
Q

The following findings in a newborn are consistent with what diagnosis?

  1. Deafness
  2. Cataracts
  3. Cardiac defects
A

Congenital rubella syndrome

These newborns may also have microcephaly, microphthalmia and miningoencephalitis; however, these symptoms are characteristically accompanied by sensorineural deafness, cardiac anomalies (Persistant ductus arteriosus, atrial septal defects), congenital glaucoma and cataracts.

187
Q

What class of antiretroviral do the following drugs belong to?

  1. Didanosine
  2. Stavudine
  3. Zidovudine
A

Nucleoside reverse transcriptase inhibitors (NRTI)

188
Q

In addition to a bone marrow examination, what is included in the workup of leukemia?

A
  1. Lumbar puncture to examine the central nervous system for early leukemic involvement (higher number of blasts in the CSF is associated with a worse prognosis)
  2. Chest x-ray to detect a mediastinal mass
189
Q

Which disorder is characterized by recurring sinopulmonary, oropharyngeal, and cutaneous infections with delayed wound healing?

A

Leukocyte adhesion deficiency (LAD)

Neutrophilia is a common finding of LAD with WBC counts typically more than 50,000 cells/mm3. Severe, life-threatening infection is possible with staphylococcus species, enterobacteriaceae, and candida species. Good skin and oral hygiene are important. Broad-spectrum antimicrobials and surgical debridement are early considerations with infection.

190
Q

What agent is used to treat tricyclic antidepressant or aspirin overdoses?

A

Sodium bicarbonate

191
Q

Jaundice presenting within the first 24 hours of life requires immediate attention and might be caused by what 6 conditions?

A
  1. Erythroblastosis fetalis
  2. Hemorrhage
  3. Sepsis
  4. Cytomegalic inclusion disease
  5. Rubella
  6. Congenital toxoplasmosis
192
Q

What is the treatment for an infant with chlamydial conjunctivitis or pneumonia presenting within the first 2 months of life?

A

Oral Erythromycin for 2 weeks

193
Q

The following characteristics are associated with what vitamin/nutrient deficiency?

  1. Dementia
  2. Dermatitis
  3. Diarrhea
A

Vitamin B3 (Niacin)

Niacin deficiency is known as pellagra.

194
Q

What is the inheritance pattern of cystic fibrosis?

A

Autosomal recessive

195
Q

What is the most common cause of neonatal sepsis from birth to 3 months?

A

Group B streptococcus

Approximately 80% of cases occur as early-onset disease resulting from vertical transmission from mother to infant during labor and delivery. Respiratory signs (apnea, grunting respirations, tachypnea, or cyanosis) are the initial findings in more than 80% of neonates, regardless of the site of involvement.

196
Q

The following findings in a newborn are consistent with what diagnosis?

  1. Chorioretinitis (inflammation of the choroid and retina of the eye)
  2. Hydrocephalus
  3. Intracranial calcifications
A

Congenital toxoplasmosis

The risk of toxoplasmosis transmission during pregnancy increases as the pregnancy progresses, but the severity of the neonatal disease decreases. Microphthalmia, microcephaly, hepatomegaly, diffuse lymphadenopathy, jaundice and diffuse petechiae may be seen. Laboratory reports show hyperbilirubinemia and thrombocytopenia.

197
Q

The following characteristics are consistent with which diagnosis?

  1. Dehydration
  2. Polyuria
  3. Nausea
  4. Vomiting
  5. Abdominal complaints
  6. Hypothermia
  7. Hypotension
A

Diabetic ketoacidosis

Kussmaul respirations and acetone breath are also characteristic. A high index of suspicion is required to make the diagnosis, especially in younger children because of the nonspecific presentation.

198
Q

The following are clinical features of abuse of which substance?

  1. Euphoria
  2. Nystagmus
  3. Ataxia
  4. Emotional lability
  5. Hallucinations affecting body image that can result in panic reactions
  6. Disorientation
  7. Hypersalivation
  8. Abusive language
A

Phencyclidine (PCP)

Retention time for Urine screening purposes

8 days

199
Q

The most common form of nonnutritional rickets is what?

A

Familial primary hypophosphatemia

This is an X-linked dominant condition in which phosphate reabsorption is defective, and conversion of 25(OH)D to 1,25(OH)2D in the proximal tubules of the kidneys is abnormal. This results in low serum 1,25(OH)2D, low-normal serum calcium, moderately low serum phosphate, and elevated serum alkaline phosphatase levels. Additionally hyperphosphaturia without evidence of hyperparathyroidism is present.

200
Q

Approximately 60% of patients with cerebral palsy are affected by what?

A

Mental retardation

201
Q

After intubation, what is the next step in management of a neonate with a heart rate < 60bpm immediately after birth?

A

Chest compressions for 30 seconds

If this fails to bring the heart rate above 60bpm, epinephrine is usually administered.

202
Q

What is measured in serum trisomy screening and when is screening performed?

A
  1. Alpha-fetoprotein: Decreased in DS
  2. Human chorionic gonadotropin (hCG): Increased in DS
  3. Inhibin A: Increased in DS
  4. Estriol: Decreased in DS

Screening is performed between 15 and 20 weeks

203
Q

What is the most common childhood movement disorder?

A

Cerebral Palsy

It has a prevalence of 3-4 cases per 1000 live births.

204
Q

What is the inheritence pattern of severe combined immunodeficiency (SCID)?

A

Autosomal recessive or X-linked

205
Q

What treatment is used for severe lithium toxicity?

A

Hemodialysis

Lithium is the most dialyzable toxin.

206
Q

Approximately 1/3 of cerebral palsy patients are affected by what?

A

Seizures

Other comorbidities of cerebral palsy include: Deafness, visual impairments, swallowing difficulty w/ concomitant aspiration, limb and sensory impairments, and behavioural disturbances are common comorbidities.

207
Q

The following findings are suggestive of what diagnosis?

  1. Stepping response after the age of 3 months
  2. Moro reflex after the age of 6 months
  3. Asymmetrical tonic neck reflex beyond 6 months
A

Cerebral palsy

208
Q

The following signs/symptoms are suggestive of what diagnosis?

  1. Digital clubbing
  2. Recurrent sinusitis
  3. Growth retardation
  4. Fat malabsorption
A

Cystic fibrosis

209
Q

What is the most common cardiac cause of cyanosis in neonates?

A

Transposition of the great arteries (TGA)

It occurs in approximately 5% of children with congenital heart disease. TGA typically causes an “egg-on-a-string” appearance on chest radiography.

210
Q

what is the 5-year survival rate for childhood acute lymphoblastic leukemia (ALL)?

A

Greater than 80%

Treatment is with combination chemotherapy. Induction therapy with prednisone, vincristine, and asparginase, produces remission within 4 weeks in approximately 98% of children with non-high-risk ALL. Maintenance therapy with methotrexate, 6-mercaptopurine, vincristine, and prednisone is given for 2-3 years to prevent relapse.

211
Q

The following characteristics are consistent with which diagnosis?

  1. Nausea
  2. Vomiting
  3. Abdominal pain
  4. Diarrhea
  5. Third spacing and hemorrhage in the gut (can lead to hypovolemic shock)
  6. Ventricular tachycardia (QT prolongation)
  7. Congestive heart failure
  8. Seizures
  9. Cerebral edema
  10. Encephalopathy
  11. Coma
A

Acute arsenic ingestions

Early on, patients develop loss of deep tendon reflexes, paralysis, painful dysthesias, and respiratory failure similar to Guillain-Barre syndrome. Fever, anemia, alopecia, hepatitis, and renal failure also can be seen.

212
Q

What is the treatment regimen an individual who tests positive for tuberculosis?

A

Two months of a 3 or 4 medication regimen followed by four to ten months of isoniazid and rifampin

Therapy for 9-12 months is recommended for CNS or disseminated TB.

213
Q

The following are clinical features of abuse of which substance?

  1. Elation and euphoria
  2. Impaired short-term memory
  3. Distortion of time perception
  4. Poor performance of tasks requiring concentration (IE driving
  5. Loss of judgement
A

Marijuana

Retention time for Urine screening purposes

  1. Occaisional users: 3-10 days
  2. Chronic users: Up to 2 months
214
Q

Failure to thrive and multiple vitamin deficiencies can be caused by what inheritable disease?

A

Cystic fibrosis

The vitamin deficiencies are due malabsorption.

215
Q

What differes between the calcified brain densities of congenital CMV and congenital toxoplasmosis?

A

CMV: Calcified brain densities typically are found in a periventricular pattern

Toxoplasmosis: Calcified brain densities typically are found scattered throughout the cortex

216
Q

Parinaud’s syndrome (paralysis of vertical gaze that may be associated with pupillary disturbances) and Collier’s sign (eyelid retraction) are indicative of what?

A

A lesion in the rostral midbrain

The lesion is specifically at the level of the superior colliculus and cranial nerve III. This is most likely caused by a pinealoma or germinoma at this region.

217
Q

The following signs/symptoms in a newborn are characteristic of what diagnosis?

  1. Rapid onset (within the first 6 hours of birth) tachypnea, hypothermia, and poor perfusion
  2. Low WBC count with left shift
  3. Poor feeding
A

Group B streptococcal infection

Management would include rapid application of the ABC’s of resuscitation, followed by institution of appropriate antibiotics once cultures are obtained. Despite these measures, mortality from this infection is high.

218
Q

What defect causes type 4 renal tubular acidosis (RTA)?

A

a defect in sodium/potassium exchange in the distal tubule

This results in hyperkalemic, hyperchloremic acidosis. In children, obstructive uropathy, renal disease, or multicystic dysplastic kidneys are common causes.

219
Q

Infants drinking goat’s milk must have nutritional supplementation with what?

A
  1. Vitamin B12
  2. Folate
  3. Iron

Unpasteurized goat’s milk can contain Brucella ovis and cause brucellosis.

220
Q

A deficiency in hexosaminidase A is characteristic of what diagnosis?

A

Tay-Sach’s disease

This is characterized by hyperacusis (difficulty tolerating everyday sounds), mental retardation, seizures, cherry red macula. It is not characterized by hepatosplenomegaly or cervical lymphadenopathy (as seen in Niemann-Pick’s disease).

221
Q

What is the most important laboratory test in patients with suspected acute post-streptococcal glomerulonephritis (APSGN)?

A

Serum C3 and C4 levels

C3 is low in 90% of APSGN cases, whereas C4 usually is normal. If both levels are low, an alternate diagnosis is considered. Urinalysis typically reveals high specific gravity, low pH, hematuria, proteinuria, and red cell casts. Documentation of a recent streptococcal infection is helpful; serum markers include the presence of ASO enzyme antibodies and anti-DNase B antibodies. Anti-DNase B antibody assays are more reliable.

222
Q

How does diabetes contribute to immunodeficiency?

A

Neutrophil dysfunction and ineffective neutrophil chemotaxis

Hyperglycemia promotes neutrophil dysfunction, and circulatory insufficiency contributes to ineffective neutrophil chemotaxis during infections.

223
Q

When are chest radiographs obtained for patients with sickle cell disease?

A

At 2 years of age and periodically thereafter

Periodic chest radiographs are for screening purposes, for recent acute chest syndrome, or if the child has chronic cardiac or pulmonary disease.

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