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Pediatrics Clerkship > Pediatrics > Flashcards

Pediatrics Flashcards

1
Q

Reye syndrome

A

Rare, often fatal childhood hepatic encephalopathy. Findings: mitochondrial abnormalities, fatty liver (microvesicular fatty changes), hypoglycemia, vomiting, hepatomegaly, coma. Associated w/ viral infection (especially VZV and influenza B) that has been treated with aspirin. Mechanism: a. mitochondrial damage b. disruption of urea cycle (normally used to metablize ammonia); causes increase in serum ammonia c. aspirin metabolites decrease beta-oxidation by reversible inhibition of mitochondrial enzymes; more FA become available to synthesize TG. *Avoid aspirin in children, except for those w/ Kawasaki disease (FA p 369, Goljan pp 533-34)

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2
Q

Kawasaki disease

A

Necrotizing medium-vessel vasculitis affecting Asian children <4 yo; likely has infectious etiology that precipitates immune rxn in genetically susceptible individuals. Mucocutaneous lymph node syndrome: Conjunctival injection, Rash (polymorphous –> desquamating), Adenopathy (cervical), Strawberry tongue (oral mucositis), Hand-foot changes (edema, erythema): CRASH and burn mnemonic. May develop coronary artery aneurysm; thrombosis or rupture can cause death. Treat w/ IVIG and aspirin. FA pp 302-303; Goljan 269

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3
Q

Bronchiolitis

A

Blockage of small airways in lungs due to infection. Affects children <2. Sx: wheezing, fever, cough, runny nose, breathing problems Tx: supportive care

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4
Q

RSV

A

Most common cause of pneumonia and bronchiolitis (wheezing) in infants. Infections commonly occur in winter Causes otitis media in older children Hand washing/gloves presents nosocomial outbreaks in nurseries Fusion protein causes cells to fuse, producing multinucleated giant cells. Rapid diagnosis by detection of antigen in nasopharyngeal wash. PCR also useful for dx Passive immunization (high risk children): Palivizumab (monoclonal ab) reduces hospitalization rates between Nov and April Rx = ribavirin (FA p 161, 163)

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5
Q

Paramyxoviruses

A

Paramyxovirus (enveloped, SS, (-) linear, nonsegmented helical capsid). Parainfluenza - croup RSV - bronchiolitis in babies Measles Mumps All contain surface F (fusion) protein, which causes respiratory epithelial cells to fuse and form multinucleated cells. Palivizumab (monoclonal ab against F protein) prevents pneumonia caused by RSV infection in premature infants. (FA p 161, 163)

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6
Q

Turner syndrome: genetic basis and clinical features

A

Genetic basis: Complete of partial loss of an X chromosome (45, X). - Most common sex-chromosome abnormality. Clinical features: - Narrow, high-arched palate - Low hairline - Webbed neck - Broad chest with widely spaced nipples - Cubitus vagus - Short stature - Coarctation of the aorta - Bicupsid aortic v alive - Horseshoe kidney - Streak ovaries, amenorrhea, infertility

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7
Q

Associations of the following congenital heart abnormalities: - Atrioventricular canal defects (ASD, VSD) - Mitral valve prolapse - Tetralogy of Fallot - bicuspid aortic valve/aortic coarctation/aortic root dilatation - Patent ductus arteriosus - Truncus arteriosus - Transposition of the great arteries

A

AV canal defects: Down syndrome (most common congenital heart defect in Down is complete atrioventricular septal defect (CAVSD) Mitral valve prolapse: connective tissue disorders (Marfan, Ehlers-Danlos) Tetralogy - DiGeorge syndrome (chromosome 22q11.2 deletion), Down syndrome bicuspid aortic valve/aortic coarctation/aortic root dilatation - Turner syndrome Patent ductus arteriosus - congenital rubella syndrome Truncus arteriosus - DiGeorge Transposition of the great vessels - DiGeorge

VSD, ASD, PDA -Edwards syndrome

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8
Q

Heart defects that cause cyanosis and their presentations

A

Tetralogy of Fallot - cyanosis and a single second heart sound. Primary sound is harsh crescendo-decrescendo murmur caused by RV outflow tract obstruction rather than a VSD murmur. Occurs sporadically w/o other anomalies; 15% of patients with TOF have an associated condition (Eg Down or DiGeorge)

Symptomatic Ebstein anomaly. (Presents with cyanosis and heart failure due to severe tricuspid regurgitation; Auscultation reveals a “triple or quadruple gallop” [widely split S1 and S2 sounds plus a loud S3 and/or S4) and a holosystolic or early systolic murmur at the left lower sterna border)

Truncus arteriosus - neonatal cyanosis, heart failure, and systolic ejection murmur w/ loud ejection at L sternal border

Transposition of the great arteries - severe neonatal cyanosis and tachypnea

Total anomalous pulmonary venous return with obstruction - severe cyanosis, respiratory distress. Pulmonary edema, “snowman” sign (enlarged supracardiac veins and SVC)

Tricuspid atresia: Single S2, VSD murmur. X ray shows minimal pulmonary blood flow.

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9
Q

Necrotizing enterocolitis: Risk factors Clinical features X ray findings Treatment

A

Risk factors: prematurity, very low birth weight (<1.5 kg [3.3 lb]), enteral feeding (formula > breast milk) Clinical features: vital sign instability, lethargy, bilious ekes is, bloody stools, abdominal distension X-ray findings: pneumatosis intestinalis, portal venous gas, pneumoperitoneum Treatment: Bowel rest; parenteral nutrition, Broad spectrum IV abx; +/- surgery

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10
Q

Newborn with cyanosis aggravated by feeding and relieved by crying?

A

Chantal atresia. Condition may be isolated or part of a syndrome (ie CHARGE: Coloboma, Heart Defects, Atresia choanae, Retardation of growth/development, Genito-urinary anomalies, Ear abnormalities/deafness)

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11
Q

SIRS criteria

A

/

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12
Q

Kawasaki disease (mucocutaneous lymph node syndrome)

A

Fever >/= 5d + >/= 4 of the following mucocutaneous findings: - conjunctivitis: b/l, nonexudative, spares the limbus - oral mucosal changes: erythema, fissured lips, “strawberry tongue” - rash -extremity changes: erythema, edema, desquamation of hands and feet - cervical lymphadenopathy: >1.5 cm node

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13
Q

Measles presentation

A

Fever Cough Coryza (rhinitis) Conjunctivitis Rash: spreads cephalocaudally

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14
Q

Liver pathology findings in Reye syndrome

A

Microvesicular fatty changes

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15
Q

Physiologic jaundice of the newborn: path o physiology

A

(See uworld question 12)

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16
Q

DiGeorge/velocardiofacial syndrome: pathogenesis and clinical features

A

Pathogenesis: Chromosome 22q11.2 deletion. Defective development of pharyngeal pouches.

Clinical features: CATCH-22: Conotruncal cardiac defects (tetralogy of Fallot, truncus arteriosus, interrupted aortic arch), Abnormal facies, Thymic hypoplasia/aplasia (T cell deficiency), Craniofacial deformities (cleft palate), Hypocalcemia/Hypoparathyroidism

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17
Q

VACTERL

A

Vertebral, Anal atresia, Cardiac, Tracheoesophageal fistula, Renal, Limb

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18
Q

Anomalous branches of the aortic arch: anatomy, presenting symptoms

A

Can cause stridor and dysphagia due to compression of trachea and esophagus.

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19
Q

Infantile hypertrophic pyloric stenosis: risk factors, presentation, diagnostic studies, treatment

A

Risk factors: first born boy, erythromycin, bottle feeding

Presentation: projectile nonbilious emesis, poor weight gain, dehydration, olive-shaped abdominal mass. “Hungry vomiter.”

Diagnostic studies: hypochloremic metabolic alkalosis, thickened pylorus on abdominal ultrasound

Tx: IV rehydration, pyloromyotomy

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20
Q

Presentation of milk protein-induced allertic proctocolitis (milk protein intolerance)

A

Vomiting, poor weight gain, bloody stools.

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21
Q

Treatment for long QT

A

Beta blockers (eg propranolol) are class II antiarrhythmics and the medication class of choice to blunt exertional heart rate and shorten the QT interval.

Symptomatic patients (lightheadedness, palpitations) or those w/ hx of syncope require a beta blocker therapy plus long-term pacemaker placement.

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22
Q

Fastest/most accurate method of diagnosing malrotation with midgut volvulus?

A

Upper GI series. The finding of the ligament of Treitz on R side of abdomen reflects malrotation while contrast in a “corkscrew” pattern indicates volvulus.

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23
Q

Diagnostic study to distinguish meconium ileus vs Hirschprung?

A

Contrast enema.

(Microcolon indicates meconium ileus; rectosigmoid transition zone indicates Hirschprung)

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24
Q

MEN syndromes

A
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25
Q

clinical features of ToRCHeS infections

A

All: IUGR (inflammation), hepatosplenomegaly (reticuloendothelial activation), jaundice, blueberry muffin spots (extramedullary hematopoiesis)

Toxoplasmosis - diffuse intracerebral calcifications, severe chorioretinits, hydrocephalus

Rubella - cataracts, heart defects (PDA, pulmonary artery hypoplasia), deafness

Cytomegalovirus - periventricular calcifications, hearing loss, seizures, petechial rash

HSV-2 - Encephalitis, herpetic lesions

Syphilis - copious rhinorrhea (snuffles), abnormal long-bone radiographs, desquamating or bullous rash (can be on palms/soles), facial abnormalities (notched teeth, saddle nose, short maxilla); often results in stillbirth, hydrops.

(FA p 175)

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26
Q

Causes of meningitis in children: <1 month vs >/= 1 month

A

< 1 month:

  • GBS
  • E. coli &gram negatives
  • Listeria
  • HSV

>/=1 month

  • S. pneumo
  • N. meningitidis
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27
Q

Most commonly implicated organisms in acute bacterial rhinosinusitis?

A

S. pneumoniae (~30%), nontypeable H. influenzae (~30%), . M. catarrhalis (~10%)

S. aureus may be seen in chronic sinusitis (>12 weeks of sinus inflammation)

Pseudomonas common in nosocomial sinusitis, esp in immunocompromised pts w/ nasal tubes or catheters.

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28
Q

Diagnostic features of acute bacterial rhinosinusitis

A
  • Persistent symptoms >/= 10 days w/o improvement

OR

  • Severe symptoms, fever >/= 30 C (102 F), purulent nasal discharge, or face pain >/= 3 d

OR

  • Worsening symptoms >/= 5d after initially improving viral URI
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29
Q

Potentially serious cause of infectious mononucleosis?

A

Acute airway obstruction. Sx include throat tightness and difficulty swallowing. Patients have severe tonsillar enlargement, difficulty swallowing, labored breathing. Tx includes corticosteroid injection.

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30
Q

Kawasaki diagnostic criteria

A
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31
Q

CENTOR criteria

A
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32
Q

Ludwig angina

A

Severe and potentially dangerous cellulitis of sublingual or submandibular space. Caused by bacterial spread from adjacent infection (dental abscesses or infections of tongue)

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33
Q

Acute Rheumatic Fever

A

Epidemiology: Peak incidence age 5-15

Twice as common in girls.

Clinical features: Major = JONES = joints (migratory arthritis), carditis, nodules (subcutaneous), erythema marginatum, Sydenham chorea

Minor = fever, arthralgias, elevated ESR/CRP, prolonged PR interval

Late sequelae = mitral regurg/stenosis

Prevention = penicillin for GAS pharyngitis

Dx is made if patient has 2 major, 1 major + 2 minor, or if either Sydenham chorea or carditis is present

Tx: prophylactic long-acting, IM benzathine penicillin G for several years to eradicate bacterial carriage and recurrent ARF

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34
Q

Diseases transmitted by Ixodes scapularis

A

Lyme

anaplasmosis

babesiosis

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35
Q

Vibrio vulnificans

A

Gram-neg, motile, curved rod shaped bacteria present in marine environments (estuaries, brackish ponds, coastal areas).

Leading cause of death related to seafood consumption in US

Causes cellulitis and if ingested, sepsis w/ hypotension and bullous skin lesions.

36
Q

Treatment of croup

A

Aims to reduce subglottic edema:

corticosteroids for mild cases; racemic epinephrine is added for patients with stridor at rest

37
Q

Amoxicillin triggering maculopapular rash in patient w/o penicillin allergy?

A

Infectious mononucleosis.

38
Q

Stages of pertussis

A
  1. Catarrhal (1-2 wks): mild cough, rhinitis
  2. Paroxysmal (2-6 wks): cough w/ inspiratory “whoop,” posttussive emesis (symptoms worsen at night or with laughing, exercise, or exposure to steam or smoke)
  3. Convalescent (weeks to months): symptoms resolve gradually
39
Q

Most common cause of community-acquired pneumonis in children?

A

Strep pneumo

40
Q

Contraindications to rotavirus vaccine

A
  1. anaphylaxis to vaccine ingredients
  2. hx of intussusception
  3. hx of uncorrected congenital malformation of GI tract (eg Meckel’s diverticulum)
  4. SCID
41
Q

Causes of meningitis in children

A

<1 month: GBS, E. coli and other gm negs, Listeria monocytogenes, HSV

>/= 1 month: strep pneumo, neisseria meningitidis

42
Q

Most common causes of acute unilateral lymphadenitis in children?

A

Staph/strep.

Treat with clindamycin (good coverage for MRSA as well as GAS)

43
Q

Glucose 6 phosphate deficiency

A

Type 1 glycogen storage disease, von Gierke disease. Deficient glucose-6-phosphatease in liver, kidneys, and intestinal mucosa. This results in impaired conversion of glycogen to glucose, leading to glycogen accumulation in affected organs.

Pt: 3-4 m w/ hypoglycemia (resulting in seizures) and lactic acidosis (due to buildup in the liver. Other labs: hyperuricemia, hyperlipidemia.

PE: doll-like face w/ rounded cheeks, thin extremities, short stature, hepatomegaly.

44
Q

Gaucher disease

A

due to glucocerebrocidase deficiency.

Glucocerebroside accumulates in macrophages of liver, spleen, marrow –> bone pain, cytopenias.

Hepatosplenomegaly.

45
Q

Medium chain acyl CoA dehydrogenase deficiency

A

Inability to break down fatty acid chains. Results in episodes of hypoketotic hypoglycemia during fasting states (eg illness).

46
Q

Most common cause of otitis externa

A

Pseudomonas seuruginosa

Tx: fluoroquinolone drops

Second most common: staphylococcus

47
Q

Most common causes of otitis media

A

H. influenzae, Moraxella catarrhalis, Strep pneumo

48
Q

Diagnostic criteria for acute bacterial rhinosinusitis, treatment, common bugs

A

1 of 3:

  1. persistent symptoms >/= 10 d without improvement
  2. severe onset (fever >/= 102.2) + drainage, >/=3d
  3. Worsening sx following initial improvement

Tx = amoxicillin +/- clavulanate

Clinical features: cough, nasal discharge, fever, face pain/headache

Bugs: S. pneumo, nontypeable Haemophilus influenzae

49
Q

Precocious puberty in patients with advanced bone age:

A

LH levels differentiate between peripheral and central. Peripheral (gonadotropin-independent) will have a low/normal LH level due to inhibition of hypothalamus by high circulating androgen levels from the adrenal glands or testes.

50
Q

When to do tympanocentesis and culture during myringotomy with tympanostomy tubes

A

Multiple episodes of acute otitis media (eg >/= 3 episodes in 6 months, >/= 4 in 12 months) despite appropriate abx.

51
Q

CSF analysis

A

Normal: WBC 0-5 cells/uL, Glucose 40-70 mg/dL, protein <40 mg/dL

Bacterial meningitis: WBC >1000, Glucose <40, protein >250

Tuberculous meningitis: WBC 5-1000, glucose <10, protein >250

Viral: WBC 10-500 (lymphocyte predominance), Glucose 40-70, protein <150

Cryptococcal: mildly elevated WBC w/ lymphocyte predominance, protein high, glucose low

Neurosyphilis: pleocytosis with lymphocyte predominance, elevated protein, low glucose

52
Q

Reactive arthritis is a/w?

A

Urethritis.

Preceded by enteric infection (eg campylobacter, shigella)

53
Q

Chelation therapy for lead poisoning?

A

Dimercaptosuccinic acid (succimer) - used when lead levels are 45-69

Dimercaprol (British anti-Lewisite) plus calcium EDTA should be administerd on emergency basis for levels >/=70 or acute encephalopathy

Pts w/ elevated blood lead levels should be screened for iron deficiency and prescribed oral ferrous sulfate if deficiency exists. Comorbid iron deficiency can increase GI abs of lead.

54
Q

Mumps

A

Viral infection causing fever, parotitis after nonspecific prodrome.

Self-limited. Can lead to aseptic meningitis and orchitis.

55
Q

Complications of acute otitis medica?

A

Facial nerve palsy, mastoiditis

56
Q

Organisms that chronic granulomatous disease patients are susceptible to?

A

Catalse-positive. (Staphylococcus aureus, Serratia, Burkholderia, Aspergillus, Nocardia). Recurrent, severe cutaneous and pulmonary infections. Other common infections include suppurative adenitis and osteomyelitis.

57
Q

Pertussis treatment and post-exposure prophylaxis

A

Age <1 month: azithromycin for 5 days

Age >/= 1 month: Azithromycin x 5days, OR clarithromycin x 7d, OR Erithromycin x 14d

All close contacts shuld be given a macrolide antibiotic regardless of age, immunization status, or sx.

58
Q

Infectious mono - clinical presentation

A

fever, fatigue, pharyngitis, cervical LAD, splenomegaly

59
Q

Clinical presentation: congenital hypothyroidism

A
  • Initially normal at birth
  • Sx develop after maternal T4 wanes: lethargy, enlarged fontanelle, protruding tongue, umbilical hernia, poor feeding, constipation, dry skin, jaundice.

Labs: increased TSH, decreased free T4. Newborn screen.

Tx: levothyroxine

Most common cause worldwide is thyroid dysgenesis

60
Q

Effects of fetal hyperglycemia: first trimester

A

Fetus cannot produce insulin/protect itself from hyperglycemia. Hyperglycemia can disrupt organogenesis, leading to malformations (eg congenital heart disease, neural tube defects, small left colon syndrome)

61
Q

Effects of fetal hyperglycemia: second and third trimester

A

Fetal pancreas has developed. Hyperglycemia triggers insulin release, resulting in increased glycogen and fat storage (organomegaly), increased growth factor production (macrosomia), increased oxygen consumption (polycythemia).

62
Q

Effects of fetal hyperglcyemia: delivery

A

Macrosomia makes infants more difficult to deliver, leading to higher rates of C section or device-assisted delivery (forceps, vacuum). Clavicle fracture, brachial plexus injuries, asphyxia are a/w macrosomia.

63
Q

Effects of fetal hyperglycemia - after deliery

A

Fetal insulin production takes time to decrease after delivery while maternal glucose exposure ends. This results in period of neonatal hypoglycemia until insulin levels normalze. *hypoglycemia is the most common complication among IDMs.

64
Q

Exam used to diagnose corneal abrasion?

A

Fluorescein exam

65
Q

Most common predisposing factor for acute bacterial sinusitis

A

upper respiratory infection

66
Q

Inactivated (killed) immunizations

A

Polio, Hep A

67
Q

Live attenuated

A

Rotavirus, Measles, Mumps, Varicella, Rubella

68
Q

Toxoid vaccines

A

Diphtheria, tetanus

69
Q

Subunit/conjugate vaccines

A

Hep B, Pertussis, HiB, Pneumococcal Meningococcal, HPV, Influenza (injection)

70
Q

Older children with history of periodontal disease with acute, unilateral lymphadenitis?

A

Peptostreptococcus

71
Q

Nontuberculous mycobacteria lymphadenopathy

A

Most commonly caused by Mycobacterium avium-intracellulare.

Unilateral, subacute-chronic LAD. <5 yo present w/ firm, nontender lymphadenopathy usually less than 4 cm. Skin over lymph node thins and develops violaceous color.

Usually no fever or tenderness

72
Q

Lyme disease treatment in kids <8 yo, pregnant patients

A

Amoxicillin or cfuroxime, NOT doxycycline.

Dox useful b/c it treats potential coexisting Anaplasma which is also transmitted by Ixodes tick.

IV ceftriaxone for Lyme meningitis and heart block (early disseminated disease).

73
Q
A
74
Q

Most common complication of patients with sickle cell trait

A

painless hematuria

75
Q

Major adverse effect of hydroxyurea for sickle cell disease?

A

Myelosuppression

76
Q

First-line treatment for enterobius vermicularis

A

albendazole, pyrantel pamoate

77
Q

Chaga disease

A

Trypanosoma cruzi infection.

Affects heart (cardiomyopathy, R bundle branch block)

GI tract (megacolon, megaesophagus)

Benznidazole is first-line antitrypanosomal tx

78
Q

First line tx for strongyloidiasis

A

ivermectin

79
Q

Life threatening complication of marfan syndrome?

A

Aortic root dilatation

80
Q

Side effect of hydroxyurea?

A

Myelosuppression

81
Q

Galactose-1-phosphate uridyl transferase deficiency

A

Causes galactosemia: pts have vomiting, poor weight gain, jaundice, hepatomegaly, convulsions, cataracts.

Other common manifestations include aminoaciduria, hepatic cirrhosis, hypoglycemia, mental retardation. Such patients are at increased risk for E coli neonatal sepsis.

Tx: no galactose in diet (eg stop breastfeeding)

82
Q

Galactokinase deficiency

A

Patients present with cataracts only, otherwise asymptomatic

83
Q

Uridyl diphosphate galactose-4-epimerase deficiency

A

Galactosemia manifestations + hypotonia, nerve deafness.

84
Q

Trisomy 18

A

Edwards syndrome.

A/w:

Micrognathia, prominent occiput, low-set ears, clenched hands with overlapping fingers, renal defects, limited hip abduction, heart defects, rocker bottom feet

85
Q

Cri du chat

A

cat-like cry, seen in 5p deletion syndrome.

Also may have microcephaly, protruding metpopic suture. Hypotonia, short stature, hypertelorism, wide and flat nasal bridge, ID

86
Q

Trisomy 13

A

Patau syndrome.

Cutis aplysia

Microcephaly/holoprosencephaly, microphthalmia, cleft lip/palate, umbilical hernia/omphalocele, renal defects, cardiac defects, polydactyly, rocker bottom feet

Pediatrics Clerkship (2 decks)

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