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M3 - Clerkships > Peds Clerkship > Flashcards

Peds Clerkship Flashcards

1
Q

Apnea of prematurity
- cessation of respiration for >20 seconds starting at age 2-3 days
- What is the treatment?

A
  1. Caffeine
  2. Noninvasive ventilation
    –> This is caused by immature central respiratory centers in the brainstem due to prematurity.
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2
Q

A pathologic cause (e.g. tumor, infection, trauma) should be suspected in a patient with scoliosis if they have any of the following red flags.
1. through 4.

A
  1. Back pain that causes nocturnal awakening, interferes with activities, or requires frequent analgesics
  2. Neurologic symptoms
  3. Rapidly progressing curve (>= 10 degrees per year)
  4. Vertebral anomalies on x-ray
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3
Q

What is the most common risk factor for orbital cellulitis?

A
  1. Sinusitis, particularly of the ethmoid or maxillary sinuses. Proximity to orbital space allows for spread of bacteria.

–> after this its dental infection, skin infection, orbital trauma

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4
Q

Early clinical features of what?
- snuffles, copious clear/puruulent/serosanguinous rhinorrhea
- Maculopapular rash (palms, soles, buttocks, legs usually + desquamation and hyperpigmentation can occur)
- long bone abnormalities
- skin fissures adjacent to orifices
- hepatomegaly

A

Congenital syphilis

–> hepatomegaly due to spirochetes invading the liver

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5
Q

Late clinical features of what?
- saddle nose
- notched (hutchinson) teeth
- saber shins
- sensorineural hearing loss

A

Congenital syphilis

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6
Q

Epidemiology: Most commonly transmitted via undercooked poultry

Clinical features: fever, abdominal pain, diarrhea (mucoid +/- blood), pseudoappendicitis (RLQ pain)

—what is this?

A

Campylobacter gastroenteritis

-can also present with sick contacts

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7
Q

Campylobacter gastroenteritis
- Treatment?
- Complications?

A
  1. supportive care (self limited <7 days).
  2. Guillain-Barre syndrome, reactive arthritis
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8
Q

Medications that can cause idiopathic intracranial hypertension?

A
  1. isoretinoin (vitamin A metabolite) - believed to impair CSF reabsorption
  2. Also tetracyclines
  3. Growth hormone
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9
Q

What pediatric neck masses are midline?
- what is the difference?

A
  1. Thyroglossal duct cyst - from a embryological cause
  2. Dermoid cyst - cystic mass trapped with epithelial debris, no displacement with tongue protrusion
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10
Q

Lateral pediatric neck masses
1. Branchial cleft cyst
2. Reactive adenopathy
3. Mycobacterium avium lymphadenitis

  • what is the difference
A
  1. anterior to the SCM
  2. firm, often tender and multiple nodules
  3. necrotic lymph node, violaceous discoloration of skin, frequent fistula formation
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11
Q

What is a cystic hygroma?

A
  • posterior pediatric neck mass
  • dilated lymphatic vessels
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12
Q

X linked agammaglobulinemia
- What is the pathophysiology?
- Laboratory findings?
- Age of showing symptoms?

A
  • BTK gene mutation resulting in defective Bruton tyrosine kinase. This causes impaired B cell maturation and impaired immunoglobulin production
  • flow cytometry shows decreased CD19+ B cells. Labs show normal T cells. Decreased immunoglobulins and response to vaccines
  • Recurrent sinopulm and GI infections at age >3-6 months
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13
Q

Labial adhesion
1. What is this?
2. What causes this?
3. How to treat
4. What complications can occur?

A
  1. Fusing of the posterior labia minora which typically occurs in prepubertal girls (age 2-3)
  2. due to low estrogen production (but can also be due to chronic inflammation, poor hygiene, skin irritation, trauma)
  3. estrogen cream
  4. pain, pruritus, abnormal urinary stream, increased risk/recurrence of UTIs

—> mild/asymptomatic ones require no tx bc 80% of them resolve spontaneously

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14
Q

Meningococcemia (due to neisseria meningitidis)
1. Dx test:
2. Tx: for patient and close contacts

A
  1. Lumbar puncture , also blood culture
  2. Ceftriaxone (started even before getting lab results back) — Rifampin for close contacts
    –Get CT before lumbar puncture if incident of seizure, obtundation, FND, papilledema, and immunocompromised state
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15
Q

Facial dysmorphisms (midfase hypoplasia)
- smooth philtrum/ indistinct nasal philthrum
- thin vermillion border (transition form lip to skin around it on upper lip)
- small palpebral fissures (a line from inner out to outer eye)

  • <10th percentile growth

-what is this syndrome?

A

Fetal alcohol syndrome

Others: microcephaly, poor growth
–neuro: developmental delay, intellectual disability, memory issues, and poor adaptive functioning. Hyperactivity, inattention, and poor social skills

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16
Q

Autosomal recessive polycystic kidney disease
vs
Autosomal dominant polycystic kidney disease

– how to differentiate

A
  1. manifests in infancy as large flank masses BUT also with pulmonary hypoplasia, Potter faces
  2. asymptomatic in childhood
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17
Q

Vesicoureteral reflux is definitive diagnosis after what procedure?

A

Voiding cystourethrogram
- renal ultrasound is performed to screen for hydronephrosis
- Renal scintigraphy with dimercaptosuccinic acid is modality for long term eval for renal scarring

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18
Q

Neurofibromatosis Type I vs II
1. Gene mutation
2. location of mutated gene
3. Main clinical features

A

Type I
1. NF1 - codes for neurofibromin
2. Chrom 17
3. Cafe au lait spots, multiple neurofibromas (includes optic gliomas), lisch nodules

Type II
1. NF2 - codes for merlin
2. Chrom 22
3. Bilateral acoustic neuromas

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19
Q
  1. What is the greatest risk factor for intraventricular hemorrhage (IVH) ?
  2. when does IVH present if it does
  3. what is the screening test used?
  4. what is tx?
A
  1. prematurity- ruptured germinal matrix vessels. Neonates born <32 weeks are at highest risk bc germinal matrix involutes by week 32
  2. first 3-4 days of life, up to 50% are asymptomatic
  3. all neonates born <32 weeks gestation should undergo screening head ultrasound at age 1-2 weeks
  4. Tx is symptomatic
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20
Q

What is the pathophysiology of acute poststrep glomerulonephritis (APSGN)

A
  1. immune complexes (strep antigens + Ab) deposit between glomerular basement membrane and the mesangium/subepithelial – leads to complement system activation and accumulation of C3 within the deposits. You get low C3 but normal C4
  2. complement activation causes leukocytic infiltration and inflammation –> thickened GBM, decreased GFR (elevated Cr), and subsequent fluid retention (HTN, edema)
  3. damage to glomerular podocytes causes increased glomerular proliferation and permeability resulting in proteinuria, hematuria, and RBC casts (nephritic syndrome)
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21
Q
  1. What is the most common cause of early onset (age < 7 days) neonatal pneumonia?
A
  1. strep agalactiae/ Group B strep (commonly causes neonatal sepsis but can present with pneumonia in minority of cases)
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22
Q

GBS pneumonia
1. onset of symptoms
2. symptoms
3. x-ray findings
4. tx

A
  1. hours to days of birth
  2. temp instability, irritability, lethargy, poor feeding, tachycardia, hypotension, tachypnea, grunting
  3. diffuse alveolar densities with pleural effusions
  4. ampicillin and gentamicin
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23
Q

Until what age should parents be reassured that bedwetting is normal?

A

until age 5

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24
Q

What does CATCH 22 mean for digeorge syndrome

A

C= cardiac outflow tract anomalies (e.g. tet of fallot, persistent truncus arteriosus)
A = Anomalous face (e.g. prominent nasal bridge, low-set ears, micro/retrognathia)
T = thymic hypoplasia/aplasia –> decreased T cell immunity
C = cleft palate
H = hypoparathyroidism –> Hypocalcemia

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25
Q

Pathophysiology - proliferation of abnormal dendritic cells

Clinical features
-> Bone: localized pain (e.g. skull, pelvis, femur)
-> Skin: variable rash (e.g. purple papules, eczematous rash, petechiae)
-> CNS: polyuria/polydipsia (ie, central diabetes insipidus)
-> Pulmonary: cough/dyspnea

–What is this?
–Diagnosis?

A
  1. Langerhans cell histiocytosis - dendritic cells proliferate and infiltrate one or more organ systems
  2. skin/bone biopsy: langerhans cells
    –> lytic, “punched out” lesions on x-ray
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26
Q

What are some symptoms that occur with congenital hypothyroidism?

A
  1. infants initially appear normal at birth
  2. Gradually develop apathy, weakness, hypotonia, large tongue, sluggish movement, abdominal bloating, and an umbilical hernia

–> pathologic jaundice, difficult breathing, hypothermia, and refractory macrocytic anemia

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27
Q

Legg-Calve-Perthes (LCP) disease - idiopathic osteonecrosis (avascular necrosis)
1. ages affected
2. symptoms
3. physical exam

A
  1. 3-12 with peak between 5-7
  2. Chronic progressive leg pain and/or limp. Pain is worse with activity and may be localized to the hip or referred to the groin, thigh, or knee
  3. Limited range of motion (internal rotation and abduction), atrophy of proximal thigh muscle
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28
Q

Respiratory distress syndrome
1. What is this?

A
  1. Deficiency in surfactant –> leads to increased alveolar surface tension and diffuse atelectasis.
    –> leads to right to left shunting because areas of collapse are being perfused by not ventilated (blood is not oxygenated so as if going straight from right to left side of heart with no change)
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29
Q

Harsh crescendo-decrescendo systolic murmur best heard at the apex and left sternal border with change in intensity on physiologic maneuvers (e.g. standing from supine)
–what is this?

A

Hypertrophic cardiomyopathy - with less blood in LV you get motion of mitral leaflet toward the interventricular septum

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30
Q

Hemophilia A is deficiency in (Blank A)
Hemophilia B is deficiency in (Blank B)

A
  1. Factor VIII
  2. Factor IX

You get prolonged PTT but normal PT, normal bleeding time

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31
Q

GBS - what does it look like on culture?
–> how to treat

A

Gram-positive cocci in pairs and chains
–> IV penicillin G

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32
Q

Staph aureus - what does it look like on culture?

A

gram positive cocci in clusters

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33
Q

What are risk factors to developing celiac disease?

A
  1. first degree relative with celiac disease
  2. down syndrome
  3. autoimmune disorders (e.g. type 1 diabetes, autoimmune thyroiditis)
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34
Q

General: failure to thrive/weight loss, short stature,* delayed puberty/menarche*

Oral: enamel hypoplasia, atrophic glossitis

Dermatologic: dermatitis herpetiformis

Hematologic: iron deficiency anemia (due to malabsorption)

Neuropsychiatric: peripheral neuropathy, mood disorders (eg, anxiety, depression)

Musculoskeletal: arthritis, osteomalacia/rickets* (due to vitamin D malabsorption)

+classic symptoms: abdominal pain, distension, bloating, diarrhea

–> what is this most likely?

A

Celiac disease

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35
Q

How to diagnose celiac disease?

A

serology - showing elevated anti-tissue transglutaminase antibody

–> or duodenal biopsy (intraepithelial lymphocytosis, villous blunting)

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36
Q

Bullous impetigo vs staph scalded skin syndrome
1. blisters

A
  1. bullous impetigo is localized form of SSSS
    –> when ruptured the blisters have distinct honey colored crust
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37
Q

(Blank) is extensive >30% of body SA skin blistering and erosion that is typically triggered by a medication. Nikolsky sign positive. Mucous membrane involvement is expected!

A

Toxic epidermal necrolysis

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38
Q

Injury to 8th cervical and 1st thoracic nerve can result in what outcomes?

A
  1. Klumpke palsy - clawhand (extended wirst, hyperextended MCP joints, flexed interphalangeal joints, absent grasp reflex)
  2. Horner syndrome (ptosis, miosis)
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39
Q

Injury to 5th and 6th cervical nerves can result in what outcomes?

A
  1. “waiters tip” - Erb-duchenne palsy (extended elbow, pronated forearm, flexed wrist and fingers)
    –> decreased moro and biceps reflexes
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40
Q

Differentiate between preseptal and orbital cellulitis
1. clinical features
2. treatment

A

Preseptal cellulitis
1. eyelid erythema and swelling. Chemosis (swelling of bulbar conjunctiva)
2. oral antibiotics

Orbital cellulitis
1. sx of preseptal cellulitis AND pain with EOM, proptosis and/or ophthalmoplegia with diplopia
2. IV antibiotics +/- surgery

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41
Q

What is pathophysiology of wiskott-aldrich syndrome?

A
  1. x linked recessive defect in WAS protein gene
    — this gene encodes for a protein essential for actin cytoskeleton rearrangement that occurs during interactions between T lymphocytes, antigen-presenting cells, and B lymphocytes leading to impaired innate and adaptive immune system

–>can be seen with frequent infections and eczema
–> thrombocytopenia
-> infections with encapsulated bacteria and opportunistic pathogens

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42
Q

What electrolyte disturbances occur with pyloric stenosis?
–> how is it initially managed and then treated?

A
  1. Hypochloremic
  2. Hyokalemic
  3. Metabolic alkalosis

–> IV hydration and electrolyte correction
–> then surgical pylorectomy

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43
Q

clinical findings
1. jaundice and hepatomegaly
2. vomiting and poor feeding/failure to thrive
3. Cataracts
4. Increased risk for E coli sepsis

what is this most likely?

A

Galactosemia
–> absent red blood cell GALT activity leads to galactose accumulation after lactose or galactose ingestion.

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44
Q
  1. What is the rash pattern of measles?
  2. treatment
A
  1. Maculopapular rash –> cephalocaudal (head first then goes down) and centrifugal spread (trunk first then extremities), spares plams/soles
  2. supportive and vitamin A in hospitalized patients
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45
Q

Clinical features:
- preceding bloody diarrhea
- fatigue, pallor
- bruising, petechiae
- oliguria, edema

Lab findings
- Hemolytic anemia (shistocytes and increased bilirubin)
- thrombocytopenia
- acute kidney injury (increased BUN, increased Cr)

–> what is this?

A

Hemolytic Uremic Syndrome
(E coli O157:H7 or shigella dysenteriae toxin causes vascular damage and microthrombi formation)
—> leads to shearing and hemolysis of RBC. Why you see anemia and shistocytes
–> vascular damage uses up platelets leading to thrombocytopenia
–> microthrombi cause increased BUN, increased Cr (AKI)

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46
Q

Physical exam
- new regurgitant murmur
- skin: janeway lesions (hemorrhagic macules on palms and soles, etc), osler nodes
- roth spots (eyes), splinter hemorrhages (nails)
- splenomegaly

—> what is this ?

A

infective endocarditis

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47
Q

Marfan vs homocystinuria - which is associated with intellectual disability?

A

Homocystinuria

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48
Q

What are some negative side effects of hydroxyurea that can occur?

A
  1. macrocytosis
  2. myelosuppresion
  • hydroxyura inhibits ribonucleotide reductase (generates deoxyribonucleoside triphosphates for DNA synthesis and repair) which leads to inhibition of RBC maturation and leaves RBC with increased MCV (since reticulocytes have increased MCV).
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49
Q

What is mullerian agenesis?

A
  • Muller duct system defect which causes abnormal development of uterus, cervix, and upper third of vagina
  • this is failure of paramesonephric (muller) duct to develop wich is needed for uterus, etc

in the female the paramesonephric (Müllerian) duct continues to develop and the mesonephric (Wolffian) one to degenerate; in the male the opposite occurs

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50
Q

Clinical features
- median age <2
- abdominal mass
- periorbital ecchymosis
- spinal cord compression from epidural invasion (dumbbell tumor)
- Opsoclonus-myoclonus syndrome

what is this?
what are dx findings

A

Neuroblastoma
- elevated catecholamine metabolites
- small round blue cells on histology
- N-myc gene amplication

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51
Q
  • anosmia/hyposmia
  • no body hair
  • no breast development (female)
  • primary amenorrhea (female)
  • small phallus and testicles (male)

—due to disorder of migration of fetal olfactory and GnRh producing neuron (hypogonadotropic hypogonadisim)

what is this?

A

Kallmann syndrome (karyotype consistent with phenotype)
- low FSH and LH levels –> delayed puberty

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52
Q

Patients with intravascular volume depletion (eg, vomiting, diarrhea) normally have increased renal prostaglandin production to dilate the afferent arteriole and maintain the glomerular filtration rate.

(BLANK) inhibit prostaglandin synthesis, which can cause prerenal azotemia.

A

Nonsteroidal anti-inflammatory drugs

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53
Q

(BLANK), which occurs due to in utero exposure to an antiepileptic (eg, phenytoin, carbamazepine, valproate).

associated cleft lip and palate, wide anterior fontanelle, distal phalange hypoplasia, and cardiac anomalies (eg, pulmonary stenosis, aortic stenosis). The associated neural tube defects and microcephaly can also result in developmental delay and poor cognitive outcomes.

A

fetal hydantoin syndrome

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54
Q

Classic triad in children/adults
1. abdominal pain
2. RUQ mass
3. Jaundice
+/- nausea, vomiting

A

biliary cyst

Quick note: - pancreatic pseudocysts develop slowly after several months in pts with history of acute or chronic pancreatitis. Biliary cysts can be acute onset.

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55
Q

Congenital infections
1. chorioretinitis
2. hydrocephalus
3. diffuse intracranial calcifications

  • what is this?
  • how is it transmitted?
A

Toxoplasmosis
- exposure to cat feces or kitty litter or indirectly via unwashed fruits,veggies
- or consumption of undercooked meat from infected animals

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56
Q

Congenital infections
1. rhinorrhea
2. skeletal anomalies
3. desquamating rash (palms/soles)

  • what is this?
A

syphilis

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57
Q

Congenital infections
1. cataracts
2. heart defects (PDA)
3. sensorineural hearing loss

  • what is this?
A

Rubella

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58
Q

Congenital infections
1. Periventricular calcifications
2. Microcephaly
3. Sensorineural hearing loss

  • what is this?
  • how is it transmitted?
A

CMV
- bodily fluids (e.g. urine, saliva)

– also can have: intrauterine growth restriction, seizures, hepatosplenomegaly, jaundice, thrombocytopenia, and petechial rash

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59
Q

Herpangina
1. What is this?
2. What causes this?

A
  1. vesicular oral infection in children (3-10 years old). Present with fever and pharyngitis and typically in late summer/early fall. Shows gray vesicles that progress to fibrin coated ulcers - located in posterior soft palate, anterior palatine pillars, tonsils, and uvula.
  2. Coxsackie virus
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60
Q

How to differentiate between herpangina and herpetic gingivostomatitis
1. age groups in kids
2. Clincal features
3. treatment

A

Herpangina
1. 3-10 years
2. gray vesicles that progress to fibrin coated ulcers in posterior soft palate, tonsils, uvula. Caused by coxsackie virus
3. supportive

Herpetic Gingivostomatitis
1. 6 months to 5 years
2. HSV-1 causes cluster of vesicles that generally localize to anterior oral cavity (buccal mucosa, tongue, gingiva, hard palate ) and lips
3. Acyclovir

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61
Q

Why is there
-decrease in MCW (mean corpuscular volume)
- elevations in red blood cell distribution width (RDW) in people with iron deficiency anemia?

A
  1. Inadequate iron stores in the bone marrow lead to a low erythrocyte count and the production of hypochromic RBCs with a low mean corpuscular volume.
  2. RBC distribution width (size variability) is elevated because the amount of iron available for RBC synthesis varies throughout the day.
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62
Q

What is a common complication from surgical correction of tetralogy of fallot?

A

When correcting the pulmonic stenosis or RVOT obstruction –> you can get pulmonic regurgitation which sounds like
—decrescendo diastolic murmur at the left sternal border that INCREASES with inspiration (bc youre increasing preload)

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63
Q

Clinical presentation
1. hemolytic anemia
2. jaundice
3. splenomegaly

Lab findings
1. increased MCHC
2. Neg coombs test
3. spherocytes on peripheral smear
4. TESTS: increased osmotic fragility on ACIDIFIED GLYCEROLD LYSIS TEST and abnormal EOSIN-5-MALEIMIDE BINDING TEST

  • what is this?
  • how to treat?
  • Is this micro,normo,macro - cytic
A

Hereditary spherocytosis

  • tx with folic acid, blood transfusion, and splenectomy

—This is normocytic (normal MCV), normochromic (normal red color)
—The increase in MCHC is the result of a decrease in RBC cell volume (caused by a decrease in RBC water content), whereas the hemoglobin content remains constant. The exact mechanism of this cellular dehydration remains unclear, but it is a fairly HS-specific finding.

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64
Q

1 What are the 4 main organisms that cause sepsis in children <1 month

  1. what about >1 month (2)
A
  1. GBS** (most common), E coli (+ other gram neg), Listeria, Herpes simplex
  2. Neisseria, strep pneumo

– although intrapartum antibiotic prophylaxis prevents against early onset infection it does not decrease risk for late onset (>1 week old)

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65
Q

harsh crescendo decrescendo systolic ejection murmur over left upper sternal border (with radiation to axillae or back) is due to what issue in heart?

A

Pulmonary stenosis (can be see in various cases but one example is tet of fallot)

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66
Q

Clinical features and diagnosis
1. Difficult to control asthma, thick sputum
2. Chest imaging shows fleeting infiltrates, bronchiectasis, bronchial mucoid impaction
3. elevated serum IgE (>1000 IU/mL)

-what is this?
- how to treat

A
  1. allergic bronchopulmonary aspergillosis
  2. systemic glucocorticoids
    - antifungal drugs (voriconazole)
    - treatment of underlying asthma
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67
Q

In pediatric patient with suspected AIS (acute ischemic stroke) what is the most urgent test to do?

A
  1. MRI with MR angiography
  2. young children have more nonlocalizing symptoms like headache, generalized or focal seizures, AMS (lethargy), and FND (weakness, aphasia)
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68
Q

Non-bullous vs bullous impetigo

A

Non-bullous is crusted (begins with tiny blisters that eventually burst and leave small wet patches of red skin that may weep fluid)

Bullous is large blisters (contain clear or yellow fluid that eventually become purulent or dark) - does not form honey colored crust!

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69
Q

How does nonbullous impetigo get treated?

A
  1. topical antibiotics (eg mupirocin) if localized
  2. oral antibiotics (eg cephalexin) if extensive
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70
Q

Duchenne muscular dystrophy (DMD)
- other than proximal muscle weakness that causes delayed motor milestones, toe walking, waddling gait, gowers sign, calf pseudohypertrophy

  • what other issues can occur?
  • what lab value is elevated
  • what is inheritance pattern?
A
  1. scoliosis, lumbar lordosis
    – dilated cardiomyopathy (get an ecg, echo) and restrictive lung disease (PFT)
  2. increased serum creatinine kinase. This is the initial screening to see if pt has DMD

DMD - absent dystrophin in muscle cytoskeleton. Muscle atrophy –> replacement with connective tissue/fat

  1. x linked recessive
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71
Q

What features differentiate Marfan from Homocystinuria?

A

BOTH:
- pectus deformity, tall stature, arachnodactyly, joint hyperlaxity, skin hyperelasticity, scoliosis

Marfan:
- AD
- Normal intellect
- aortic root dilation
- upward lens dislocation

Homocystinuria
- AR
- Intellectual disability
- Thrombosis
- Downward lens dislocation
- Megaloblastic anemia
- Fair complexion

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72
Q

Clinical symptoms
– Constitutional: fever, fatigue & weight loss
—Symmetric, migratory arthritis
—Skin: butterfly rash & photosensitivity
—Serositis: pleurisy, pericarditis & peritonitis
—Thromboembolic events (due to vasculitis & antiphospholipid antibodies)
—Neurologic: cognitive dysfunction & seizures

Lab findings
—-Hemolytic anemia, thrombocytopenia & leukopenia
—-Hypocomplementemia (C3 & C4)
—-Antibodies:
**Antinuclear antibodies (sensitive)
**Anti-dsDNA & anti-Smith (specific)
—–Renal involvement: proteinuria & elevated creatinine

what is this?

A

Systemic lupus erythematosus (SLE)
- classic malar rash is often absent and should not be relied on for dx
- SLE can affect CNS, psychiatric symptoms (psychosis, depression, mania, and anxiety)

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73
Q
  1. neonatal polycythemia is defined as…
  2. what causes this in neonates?
A
  1. hematocrit level >65%
  2. intrauterine hypoxia (maternal diabetes, HTN, or smoking), erythrocyte transfusion, genetic/metabolic disease
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74
Q

What procedure does androgen insensitivity syndrome usually require/have need for?

  • when patient is genotypically male but phenotypically female due to receptors being insensitive to androgens
A

Gonadectomy - because these patients have undescended testes and if left like this can have increased risk of testicular cancer

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75
Q
  • Pigmented macules (lips, buccal mucosa, palms/soles)
  • > = 2 GI hamartomatous polyps (abdominal pain due to obstruction or intussusception, anemia, rectal prolapse)
  • increase cancer risk
A

Peutz-Jeghers Syndrome
- Autosomal dominant
- Tumor suppressor gene mutation causes unregulated tissue growth

– can get upper and lower endoscopy to identify bleeding in someone who is appearing anemic

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76
Q

prolonged QT is identified as how many seconds?

A

> 460 ms on ECG

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77
Q

Neuroblastoma vs nephroblastoma (wilms tumor)

A

Neuroblastoma
- age <2
- abdominal mass
- arises on the adrenal glands
- does not cause hematuria

Nephroblastoma (wilms tumor)
- Age 2-5
- abdominal mass
- usually asymptomatic but can be associated with WAGR (wilms tumor, aniridia, GU abnormalities, mental retardation)
- hematuria can occur in up to 25%

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78
Q
  1. patient with primary amenorrhea, no secondary sexual characteristics, and short stature has a uterus andelevated FSH and LH. This presentation is consistent with(BLANK)
A

Turner syndrome (TS) - , a sporadic chromosomal disorder caused by complete or partial deletion of an X chromosome. Absence of the second X chromosome results in gonadal dysgenesis (ie, poorly developed ovaries), which presents as streak ovaries (ie, small ovaries with minimal or no follicles) on pelvic ultrasonography.

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79
Q
  • Fever for >= 5 days
  • Cervical lymph nodes > 1.5 cm
  • Rash
  • Bilateral nonexudative conjunctivitis
  • Mucositis
  • Swelling and/or erythema of palms/soles
  • Coronary artery aneurysm risk

what is this?

A

Kawasaki disease

CRASH and BURN
C- conjunctivitis, mucositis
R- rash
A - adenopathy, cervical
S - strawberry tongue
H - hands and feet swelling or erythema of palms/soles
Burn: fever

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80
Q

What is treatment of splenic sequestration in people with sickle cell disease?

(symptoms: acute drop in hemoglobin, reticulocytosis, thrombocytopenia)

A

isotonic fluid resuscitation
red blood cell transfusion (to help restore circulatory volume and correct anemia)
+/- splenectomy (especially if splenic sequestration is recurrent)

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81
Q

What chromosomal alteration leads to
1. prominent occiput
2. low set ears
3. clenched hands w/overlapping fingers
4. Micrognathia (small chin)
5. heart defects (VSD most common)
6. Kidney defects
7. Limited hip abduction
8. Rocker bottom feet

A

Trisomy 18 (Edwards syndrome)

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82
Q

Clinical features
1. severe, chronic eczema
2. Noninflammatory (cold) abscesses (e.g. staph, candida)
3. Recurrent sinopulm infections
4. Dysmorphic facies
5. Retained primary teeth

Lab
1. Increased Ige
2. Eosinophilia
3. Normal leukocyte count with decreased Th17 (helper T cell)

  • What is this?
  • Treatment
A
  • Hyper IgE syndorme
  • supportive skin care and antibiotic prophylaxis and treatment
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83
Q

clinical findings
1. nephropathy (hematuria, progressive renal insufficiency, proteinuria, HTN)
2. Bilateral sensorineural hearing loss
3. Anterior lenticonus (lens protrusion)

–what is this + pathophysiology?
– what findings are found in renal biopsy

A
  1. Alport syndrome - mutation of type IV collagen, inherited (X linked)
  2. Longitudinal splitting of GBM (basket weaving)
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84
Q
  1. Signs of infantile Vitamin K deficient bleeding include?
  2. PT changes?
  3. PTT changes?
A
  1. Classically presents on days 2-7 of life. Easy bruising, umbilical/mucosal/GI bleeding, intracranial hemorrhage
  2. increased PT
  3. increased PTT if severe

*vitamin K affects carboxylation of coagulation factors II (prothrombin), VII, IX, and X

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85
Q
  1. What are the three phases of pertussis
  2. What bacteria causes pertussis
  3. How to treat?
A
  1. Catarrhal (wks 1-2) ; mild cough, rhinitis
    –> Paroxysmal (wks 2-8), severe coughing spells, inspiratory whoop, postussive emesis, apnea/cyanosis (infants), posttussive syncope (older pts)
    —> convalescent (wks 8+): sx resolve gradually
    (Also recognize: pts have low grade/no fever, leukocytosis with lymphocytosis, normal chest x ray)
  2. bordatella pertussis
  3. empirically with macrolide (e.g. azithromycin)
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86
Q

Out of all the major causes of neonatal sepsis which one is most likely to cause temporal lobe hemorrhage

A

HSV

  • GBS and Listeria can cause cerebral edema, hydrocephalus, and abscess
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87
Q

X ray findings for neonatal respiratory distress syndomre?

A

Ground glass opacities –> tx with respiratory support and exogenous surfactant

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88
Q

Hematuria
- what can differentiate between non-glomerular and glomerular cause?

A

Glomerular will show
- RBC casts (pathognomonic)
- Proteinuria
- HTN
- Edema
- Brown, cola-colored urine

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89
Q

When it is likely glomerular disease causing hematuria… what should be assessed?

A
  1. Serum complement (C3, C4) levels –> low C3 is suggestive of PSGN (most common glomerulonephritis in children, or lupus nephritis)
  2. CBC
  3. Albumin level

–> if these labs are unrevealing then renal biopsy may be indicated

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90
Q

how to differentiate between central and peripheral precocious puberty?

A
  1. CPP = results from premature activation of the hypothalamic pituitary gonadal (HPG) axis. This is seen with elevated LH at baseline
  2. PPP = Elevated sex hormones in patients with peripheral PP suppress LH levels via negative feedback
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91
Q

In strabismus which eye do you patch up to fix eye deviation?

A

patch the unaffected eye to strengthen the deviated eye.

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92
Q

For those with penicillin allergy and who need to be treated for acute otitis media what drug is given?

A

Azithromycin

  1. 1st line amoxicillin
  2. 2nd line amoxicillin-clavulanate
  3. Azithromycin (penicillin allergy)
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93
Q

X- linked recessive mutation of NADPH oxidase
(impaired respiratory burst and decreased ROS leads to inhibition of phagocytic intracellular killing)

Clinical findings
- recurrent infections with catalase positive bacteria and fungi
- Lungs, skin, liver, LN involvement
- Diffuse granulomas (e.g. GI, GU – can lead to IBD)

  • what is this?
  • How to dx
  • tx?
A
  1. Chronic granulomatous disease
  2. Measure of neutrophil superoxide production via
    —> DHR flow cytometry* – an oxidative burst test
    —> NBT (nitroblue tetrazolin test) testing
  3. Prophylaxis: TMP-SMX, itraconazole, interferon gamma
    —> active infection: culture based
    —> cure: hematopoietic cell transplant
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94
Q
  1. What causes physiologic jaundice of the newborn
  2. When does this happen?
A
  1. Mechanisms include increased bilirubin production, decreased bilirubin clearance, and increased enterohepatic recycling.
  2. days 2-4 of life
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95
Q

Bile duct destruction leads to what changes in GGT and alkaline phosphatase?

A
  1. Elevated GGT
  2. Elevated alkaline phosphatase

–conjugated bilirubin is produced by hepatocytes by cannot be transported through the fibrotic duct, leading to cholestasis
—> develop jaundice and hepatomegaly at age 2-8 weeks
—> Reticulocyte count is normal due to the absence of hemolysis. It is mainly just a backing up issue

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96
Q

Epidemiology:
- transmission via wild animal (eg hare, rabbit) hunting/skinning
- tick or mosquito bite
-bioterrorism agent

Manifestation
- fever, malaise
- ulceroglandular disease (single/papuloulcerative lesion)
- tender, suppurative regional LN
- pneumonia

  • what is this? (shape of bacteria?)
A
  • Francisella tularensis (gram-negative coccobacillus)

***Note: Yersinia pestis is transmitted by fleas from rodents and wild/domestic animals. Therefore, exposure can occur during hunting/trapping. Although this organism frequently causes very painful suppurative lymphadenitis, ocular manifestations are uncommon.

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97
Q

Pertussis
1. first line treatment
2. Treatment for close contacts

A
  1. those sick… macrolide antibiotic (e.g. azithromycin, clarithromycin) - within first 3 weeks of illness can shorten course of illness and decrease transmission risk
  2. post exposure prophylaxis –> macrolide antibiotic for all household contacts regardless of vax status

*vaccination prevents most pertussis cases

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98
Q

Howell-Jolly bodies -
1. what do these look like?
2. What causes this?

A
  1. single, round, blue inclusions within the RBC
  2. This is retained RBC nuclear remnants that are typically removed by the spleen
    –> typically indicates physical absence of spleen
    –> Could also be functional hyposplenism due to splenic autoinfarction
    –> infiltrative disorders of the spleen
    –> splenic congestion (thrombosis)

**spleen is normally responsible for clearing bacteria, particularly encapsulated organisms

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99
Q

Once guillain-barre syndrome is suspected in hemodynamically stable patient, next step is (BLANK)

A
  1. assess pulmonary function with spirometry

–> a decline in Forced vital capacity (<= 20 mL/kg) indicates impending respiratory failure warranting intubation

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100
Q

How is congenital diaphragmatic hernia managed?

A
  1. endotracheal intubation
  2. gastric decompression with NG tube
  3. surgical correction
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101
Q

Transfusion associated circulatory overload
1. symptoms
2. treatment

A
  1. within 6 hours - respiratory distress, HTN, tachycardia, and/or pulmonary edema (bilateral crackles)
    –signs of heart failure including S3 gallop and JVD
  2. Respiratory support and diuresis with furosemide
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102
Q

Clinical features
- hypotonia
- weak suck/feeding problems in infancy
- hyperphagia/obseity
- short stature
- hypogonadism
- intellectual disability
- Dysmorphic facies (narrow forehead, almond-shaped eyes, downturned mouth)

  1. what is this (chrom abnormality)
A
  1. prader-willi syndrome (loss of expression on paternal chromosome 15 q11-13) - mothers chromosome is abnormal
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103
Q

What are risk factors for acute otitis media?

A
  1. age 6-18 months
  2. lack of breastfeeding
  3. day care attendance
  4. smoke exposure
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104
Q

What risk factors at diagnosis of scoliosis is associated with curve progression? (5)
1. sex
2. age
3. puberty status
4. skeletal…
5. curve angle

A
  1. female sex
  2. age <12
  3. early pubertal status (e.g. premarchal)
  4. skeletal immaturity
  5. severe curvature (cobb angle >= 25 degrees)
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105
Q
  1. In young children with CF what is the most common pathogenic organism is…
  2. In order patients with CF what is the most common pathogenic organism?

for each how to treat

A
  1. staph aureus - treat with vancomycin for those who have had recurrent hospitalizations and skin abscesses since they have increased risk of having MRSA
  2. psuedomonas aeruginosa - treat with cefepime
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106
Q

What is cyclic neutropenia?

how to treat?

A
  1. predictable pattern of neutropenia due to genetic mutation in neutrophil elastase (which helps degrade virulence factors on bacterial pathogens)
  • cyclic neutropenia correlate with neutrophil nadirs (typically q 3 weeks) and include recurrent episodes of fever, fatigue, and mucositis
  1. Treat with G-CSF
    –>Misfolded neutrophil elastase leads to intracellular stress signaling that causes apoptosis of some neutrophil precursors in the bone marrow like ANC
    —> The ANC decreases, which transiently upregulates granulocyte colony-stimulating factor (G-CSF) to stimulate neutrophil production.
    —> As neutrophil counts normalize, G-CSF production decreases, allowing again for a decline in ANC and cycle cont.
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107
Q

Neonates with RDS who have adequate respirations and heart rate need (BLANK)

A

noninvasive positive airway pressure to maintain alveolar patency.

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108
Q
  1. Why are premature infants at greater risk for cerebral palsy?
  2. What are other risks for cerebral palsy?
A

Premature infants are at greatest risk for CP due to ischemia and necrosis in the poorly perfused area adjacent to the lateral ventricles, which is seen as white matter injury (ie, periventricular leukomalacia) on brain MRI. These patients may also have friable germinal matrix vessels that predispose to intraventricular hemorrhage.

  1. Hypoxic-ischemic injury, perinatal stroke, intrauterine infection (calcification d/t CMV, toxo)
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109
Q

Age: 3-8 years
- preceding viral illness

Clinical features
- well-appearing, afebrile or low grade fever
- Limp but able to bear weight
- Hip pain (mildly restricted ROM)

Labs:
- normal WBC count, ESR, CRP
- small effusions on US

  • What is it?
    -How is it managed?
A
  1. Transient synovitis
  2. Conservative and NSAIDs
    —full recovery within 1-2 weeks, recurrence uncommon but can happen in <15%
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110
Q

What prophylaxis is given to chronic granulomatous disease?

A

–> since this is recurrent infections with catalase positive bacteria and fungi need

  • TMP-SMX, itraconazole, interferon gamma (for severe phenotypes that need immunomodulation)
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111
Q

Neonate has bilious emesis
-> stable condition get abdominal x-ray
–> x-ray shows dilated loops of bowel
—> patient sx: Increased rectal tone and/or delayed passage of meconium (YES OR NO) - what happens if yes or no

A

YES - get contrast enema and results will indicated meconium ileus (microcolon) or Hirschsprung disease (rectosigmoid transition zone)

NO - get upper GI series –> if shows right sided ligament of treitz then this indicates malrotation

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112
Q
  1. What are the most common causes of microcytic anemia ? (2)
  2. How does MCV, RDW, and RBC change with each
A
  1. iron deficiency - decreased MCV, increased RDW, decreased RBC
  2. thalassemia - abnormally small RBC reflected with low mean corpuscular volume (MCV), a normal red cell distribution width (RDW), and normal to elevated total RBCs, reticulocyte count may be slightly elevated
113
Q

What is the classic triad of congenital toxoplasmosis?

A
  1. Chorioretinitis - inflammation and scarring of the retina and choroid
  2. Diffuse intracranial calcifications (ring enhancing appearance)
  3. Hydrocephalus
  • can also have microcephaly or macrocephaly, seizures, nonspecific findings (jaundice, hepatosplenomegaly, rash, growth restriction)
114
Q

Treatment of congenital toxoplasmosis

A

Pyrimethamine, sulfadiazine, folinic acid

115
Q

How to treat hypertrophic cardiomyopathy in infants of diabetic mothers?

A

IV fluids
Beta blockers to increase LV blood volume (increase volume diminishes the stenosis coming out of aorta)

116
Q

Normal CSF (0-5 WBC), (40-70 glucose), (<40 protein)

–> what changes for bacterial meningitis?

A
  1. > 1000 WBC
  2. < 40 glucose
  3. > 250 protein
117
Q

Normal CSF (0-5 WBC), (40-70 glucose), (<40 protein)

–> what changes for TB meningitis?

A
  1. 100-500 WBC
  2. < 45 glucose
  3. 100-500 protein
118
Q

Normal CSF (0-5 WBC), (40-70 glucose), (<40 protein)

–> what changes for viral meningitis?

A
  1. 10-500 WBC
  2. normal glucose
  3. <150 protein
119
Q

Normal CSF (0-5 WBC), (40-70 glucose), (<40 protein)

–> what changes for Guillain-Barre Syndrome?

A
  1. normal WBC
  2. normal glucose
  3. 45-1000 protein
120
Q
  • syndrome typically occurs 2-4 weeks after transmission
  • includes transient unexplained fever
  • generalized lymphadenopathy
  • maculopapular rash
  • 25% of pts have aseptic meningitis

what is this?

A

acute HIV infection

121
Q
  1. Minimal change disease - nephritic or nephrotic?
  2. pathophysiology?
  3. clinical features?
  4. treatments
A
  1. Nephrotic
  2. T cell mediated injury (cytokine induced) to podocytes – leads to increased molecular permeability to albumin
  3. Edema (periorbital, scrotal, general), fatigue, abdominal pain
  4. corticosteroids
122
Q

When there are overlapping clinical features that may indicate either transient synovitis or septic arthritis what is the best dx step?

A
  1. ultrasound (bilateral hip ultrasound - for atraumatic hip pain and limp )
    - if US shows unilateral effusion, arthrocentesis is required to evaluated for septic arthritis
123
Q

– Type I collagen gene defect
– frequent fractures
– blue sclera
– conductive hearing loss
– short to normal stature
– joint hypermobility

what is this?

A

osteogenesis imperfecta

124
Q
  • Defective type V collagen
  • joint hypermobility (subluxation, dislocation)
  • no recurrent fractures
A

Ehlers Danlos syndrome
–connective tissue disorder

125
Q
  • Fever, myalgias, fatigue
  • parotitis (unilateral or bilateral)
  • orchitis
  • pancreatitis
  • can cause aseptic meningitis (generally benign)
  • sensorineural hearing loss (often transient but can lead to deafness)

-what is this?

A

MUMPS

126
Q
  • what are some negative effects of frequent loperamide use?
A
  1. Loperamide is an over the counter antidiarrheal med that stimulates mu opioid receptions in the GI tract and reduces peristalsis
    –> in high doses it can cross the BBB, producing opioid like symptoms such as respiratory depression and AMS
127
Q

–> dx that has glucocerebrosidase deficiency
Clinical features
- at any age
- visceral organ involvement: splenomegaly (severe), hepatomegaly
- bone/bone marrow infiltration (anemia, thrombocytopenia, bone pain)
- failure to thrive, delayed pubert

  • what is this?
A

Gaucher disease (lysosomal storage disease)
—glucocerebrosidase deficiency results in accumulation of glucocerebroside in lipid laden macrophages of different tissues.

128
Q

What type of infections are common with lack of opsonizing IgG and mucosal IgA?

A

Sinopulmonary infections caused by encapsulated bacteria
- acute otitis media
- pneumonia

129
Q

liver edge is normally palpating up to how many cm below the right costal margin?

A

3 cm is normal in newborns

130
Q

-Early or midsystolic
-holosystolic or diastolic

–>which one is pathology

A

Benign: early or midsystolic (also musical or vibratory)
Pathologic: holosystolic or diastolic (also harsh)

131
Q

sepsis in neonates (<=28 days) are often subtle and non specific
1. behaviors
2. temp
3. leukocyte count

A
  1. CNS signs (lethargy, irritability, apnea), poor feeding, respiratory distress (tachypnea, grunting)
  2. temp instability (fever or hypothermia)
  3. increased or decreased leukocyte count or elevated immature neutrophil count (bands)
132
Q

IgA vasculitis (henoch-schonlein purpura)
1. pathophysiology
2. symptoms (and whats necessary for clinical diagnosis)

A
  1. deposition of IgA, C3 and fibrin occurs in small vessels
  2. palpable purpura/petechiae on lower extremities (ankles, buttocks, and thighs)*
    –> plus >=2 of the following
    - arthritis/arthralgia
    - abdominal pain, intussusception
    - renal disease (similar to IgA nephropathy)

this is other…
- other: scrotal pain and swelling

133
Q

IgA vasculitis (henoch-schonlein purpura)
1. lab findings (platelet count, coag, kidney findigns)
2. tx

A
  1. Normal platelet count and coag studies. Hematuria +/- RBC casts and/or proteinuria
  2. supportive care (hydration and NSAIDs) for most
    –hospital and systematic glucocorticoids for severe sx
134
Q

Juvenile Idiopathic Arthritis
- what are the different types?
- how to treat

A
  1. systemic, polyarticular, oligoarticular
  2. NSAIDs, disease modifying antirheumatic drugs such as methotrexate are also often required
135
Q

Juvenile Idiopathic Arthritis
Differentiate between systemic, polyarticular, and oligoarticular
-age
-clinical features

A

Systemic
1. age <18
2. arthritis in >= 1 joint for >= 6 wks
- quotidian fever for >= 2 wks
- evanescent rash
- hepatosplenomegaly
- lymphadenopathy

Polyarticular
1. age 2-5 or 10-14
2. arthritis >= 5 joints, may be complicated by uveitis

Oligoarticular
1. age 2-4
2. arthritis < 5 joints, may be complicated by uveitis

136
Q

Midsystolic or crescendo-decrescendo systolic murmur best heard at the left sternal border or cardiac apex

  • with dyspnea, fatigue, chest pain, light headedness, and syncope, sudden cardiac arrest can happen

–> what is this?

A

Hypertrophic cardiomyopathy (HCM)

137
Q

Clinical features
- usually asymptomatic
- recurrent sinopulmonary and GI infections
- associated with autoimmune disease (e.g. celiac)
- atopy (e.g. asthma, eczema)
- anaphylaxis during transfusion

-what is this?

A

Selective IgA deficiency
- low or absent IgA (which is a mucosal barrier), normal IgG, IgM, B cells
- supportive care is tx

138
Q

What type of rash does roseola have?

A

High fever followed by diffuse, maculopapular rash (not vesicular!)
-caused by HSV-6

139
Q

Hand food and mouth disease - what kind of rash comes with this?

A
  1. rash may be vesicular but mainly involve hands and feet. Oral ulcers are typical
  2. caused by coxsackievirus
140
Q
  1. Those with sickle cell disease are more susceptible to what type of organisms?
  2. What vaccinations is important for them
  3. What medication is important for them
A
  1. encapsulated organism (step pneumo, H. influenzae, N. meningitidis)
  2. vaccination with a conjugate capsular polysaccharide (conjugated s pneumoniae vaccine)
  3. twice daily administration of prophylactic penicillin - until 5 years of age
141
Q

What does the sail sign mean on chest x ray?

A

The thymic shadow
- thymus is normally visible on chest x-rays in children age <3

142
Q

commonly associated conditions include recurrent sinopulmonary infections, chronic enteroviral meningoencephalitis, and prolonged diarrhea illness

–> what immunodeficiency likely causes this?

A

X-linked agammaglobulinemia - failure of B cell maturation in the bone marrow due to defective bruton tyrosine kinase signaling. B lymphocytes cannot enter circulation, resulting in decreased antibody production and impaired humor immunity

–> usually starts showing symptoms at age >3-6 months bc maternal IgG is waning
–> sinopulmonary infections typically caused by encapsulated bacteria which require antibody opsonization for clearance
–> predisposed to chronic enteroviral infection due to lack of antibodies to neutralize disease
–> diarrheal illness (Giardia) due to low IgA

143
Q

HCM in infants of diabetic mothers
–> how does this affect child growth?
–> pathophysiology

A
  1. HCM in these patients is transient as insulin levels normalize in first few weeks of life - medical management is rarely needed. Spontaneous regression by age 1
  2. increased glycogen and fat deposition in myocardial cells leads to interventricular septum thickening and a small left ventricular cavity
144
Q

Clinical features
- Derm (ash-leaf spots, angiofibromas of the malar region, shagreen patches)
- Neuro (CNS lesions like subependymal tumors, epilepsy, intellectual disability, autism and behavioral disorders like hyper activity)
- Cardio –> rhabdomyomas
- Renal –> angiomyolipomas (benign tumors form in your kidney)

What is this?

A
  1. Tuberous sclerosis complex (TSC1 or TSC2 gene mutations) - Autosomal dominant
145
Q

UTI in children
- management during UTI is with antibiotics but other steps
1. First febrile UTI for pt
—> Age <2 - what do you do? (BLANK A)
–> Age >=2 - what do you do? (BLANK B)

  1. recurrent febrile UTIs - what do you do? (BLANK C)
A
  1. renal and bladder US, followed by VCUG if abnormal
  2. observation alone
  3. renal and bladder US AND VCUG
146
Q
  1. Shigella sonnei presentation
  2. Norovirus presentation
  3. Bacillus cereus and staph aureus GI illness presentation
  4. Rotavirus presentation
A
  1. abrupt onset of HIGH FEVER, abdominal cramping, stools with mucus and/or blood
  2. vomiting, watery diarrhea, abdominal pain 1-2 days after exposure
  3. vomiting that typically begins within 6 hours of eating contaminated food
  4. severe vomiting and watery diarrhea in unvaccinated children age <=2. Infection in adolescent and adults is typically mild or asymptomatic due to previous vaccination.
147
Q

*Nonspecific - apnea, lethargy, vital sign instability
*GI - abdominal distention, feeding intolerance, bilious emesis, blood stools
–usually in premature, very low birth weight, and babies having enteral feeding

A

Necrotizing enterocolitis

148
Q
  1. What are the two main risk factors of intraventricular hemorrhage?
  2. What are some clinical findings
  3. diagnosis?
A
  1. prematurity, very low birth weight
  2. often asymptomatic, seizures or apnea, full fontanel, acute anemia
  3. Cranial ultrasonography
149
Q

At what age should patients be referred to orchipexy surgery if patient has cryptorchidism (undescended testes)?

A
  1. > = 6 months
150
Q

all neonates with oliguria require further evaluation with the following
1. A
2. B
3. C

A
  1. History and physical exam to evaluate for risk factors (nephrotoxins, family history, renal anomaly)
  2. volume status (volume overload vs hypovolemia)
  3. Renal and bladder ultrasound (RBUS)
151
Q

what sinuses are present at birth?

A

ethmoid and maxillary
- paranasal sinuses are not fully developed in children

152
Q

What are the most commonly implicated bacteria in acute bacterial rhinosinusitis in children? (3)

A
  1. Nontypeable H influenzae (~40-50%)
  2. Strep pneumo (~20-25%)
  3. Moraxella catarrhalis (~25%)
  • bc of the 13 valent pneumococcal vaccine –> S. pneumo is less prevalent
153
Q
  1. nonspecific systemic symptoms: fever, weight loss
  2. pallor/fatigue, easy brusing/petechiae (thrombocytopenia), bone pain
  3. extramedullary spread (lymphadenopathy, hepatosplenomegaly, testicular enlargement)
  4. Mediastinal mass (T cell lineage); airway compression and/or superior vena cava syndrome
  5. Leptomeningeal spread–> neurological symptoms

—> >20% blasts is diagnostic in bone marrow bx

A

acute lymphoblastic leukemia

154
Q
  1. How is turner sydnrome (45, XO) treated?
  2. What comorbidity screening is done?
A
  1. growth hormone and estrogen therapy
  2. cardiac and renal imaging
155
Q

Main characteristics of turner syndrome
1. Infancy
2. childhood
3. adolescence

A
  1. lymphedema, cystic hygroma
    –Renal and heart defects (horseshoe kidney, aortic coarctation, bicuspid aortic valve)
  2. short stature, dysmorphic features (webbed neck, broad chest, high arched palate, cubitus valgus, short 4th metacarpals)
  3. Delayed thelarche, amenorrhea (primary or secondary)
156
Q

Types of fractures that indicate possible child abuse:
1. multiple and in various stages of healing
2. femur fracture in nonambulatory child
3. (BLANK A) rib fractures
4. (BLANK B) fractures

A

A: posterior
B: metaphyseal corner fractures

157
Q

large VSD- explain these sounds
1. Prominent S2
2. Diastolic flow murmur/diastolic rumble over the cardiac apex
2. murmur over left lower sternal border

A
  1. With so much blood going to lungs due to L to R shunt then you get pulm HTN. This HTN causes pulmonic valve to close shut loudly giving prominent S2
  2. Diastolic flow murmur - again is large volume blood flow across the mitral valve since so much blood is going to lungs, so much has to come out
  3. normal VSD sound
158
Q

Clinical features:
- Chronic, localized pain and swelling
- usually affects long bone diaphyses & axial skeleton (e.g. pelvis)
- +/- systemic findings (fever, leukocytosis)

X-ray findings
- central lytic lesion
- onion skinning
- moth eaten appearance
- periosteal elevation (codman triangle)

what is this?

A

Ewing sarcoma (2nd most common pediatric bony malignancy after osteosarcoma)
–usually white, adolescent boys

159
Q

Nontyphoidal vs Typhoidal salmonella
1. which is most common
2. How is this usually contracted
3. differences in clinical symptoms
4. diagnosis
5. tx

A

Nontyphoidal
1. most common
2. undercooked poultry/eggs
3. vomiting, diarrhea (+/- blood), fever
4. stool culture
5. self limited

Typhoidal
1. usually in resource limited regions with poor sanitation
2. contaminated food or water
3. fever & bacteremia, abdominal pain and ROSE SPOTS
4. Blood culture
5. potentially fatal, give antibiotics (ceftriaxone)

160
Q

Oligohydramnios leads to
1. flat facies
2. pulmonary hypoplasia
3. limb deformities (club feet, hip dislocation)
4. Suprapubic mass indicating bladder distension resulting from urinary tract obstruction –> in boys commonly due to posterior urethral valves

what is this?

A

Potter sequence

  • may also have weak urinary stream and renal failure postnatally due to obstructive uropathy
161
Q

How is toxoplasmosis contracted?

A
  1. undercooked meat or inadvertent ingestion of cat feces
162
Q

what differentiates exertional heat stroke from heat exhaustion?

A
  1. EHS has core body temp >104 (40 C) and also has CNS dysfunction
163
Q

What are complications of shigella infection (ingestion of contaminated food/water leading to bacterial gastroenteritis)? (2)

A
  • less commonly HUS
  • Seizure (in children)

*shigella preferentially involves rectosigmoid region causing frequent, small volume but bloody/mucoid diarrhea
*tenesmus is also common (persistent and painful desire to evacuate)

164
Q

What are the two ways that PKU can be diagnosied?

A
  1. universal newborn screening
  2. serum amino acid analysis
    check for increased phenylalanine levels
165
Q

What are some neurologic issues that occur with PKU? (4)

A
  1. microcephaly
  2. developmental delay
  3. seizures
  4. severe intellectual disability
166
Q

Osgood-Schlatter Disease
1. What causes this?
2. what is this?
3. Dx
4. how to treat

A
  1. benign cause of knee pain caused by overuse in young adolescents
  2. During periods of rapid growth, the quadriceps muscles pull on the insertion site of the patellar tendon. Pain occurs at this site (apophysis of the tibial tubercle). Traction apophysitis may lead to elevation and chronic avulsion of the tibial tubercle. Patients get progressive pain with activities (running, jumping, kneeling).
  3. clinical dx
  4. Supportive tx like pain control, activity as tolerated, PT
167
Q
  • Associated with atopic dermatitis
  • eruption of overlying painful vesicles
  • erythematous base
  • punch out erosions & hemorrhagic crusting
  • what virus causes this?
  • how to treat?
A

HSV-1
- can also have fever and lymphadenopathy
- acyclovir therapy

168
Q
  1. What are common organisms involved in pediatric empyema?
  2. Best way to treat?
A
  1. strep pneumo
  2. Staph aureus (eg. MRSA)

–> Empiric antibiotics + drainage (Ceftriaxone or cefotaxime plus MRSA coverage with vancomycin or clindamycin)
-ceftriaxone
- cefotaxime
- vancomycin
- clindamycin

169
Q

What are associated conditions that lead to membranous nephropathy?

A
  1. Malignancy
  2. Infection (e.g. Hep B)
  3. Autoimmune (e.g. Lupus, thyroiditis)
  4. Drugs (e.g. NSAIDs, penicillamine)
170
Q
  1. HUS is seen with what two illnesses/pathogens?
  2. What is the pathophysiology to kidneys
  3. Treatment
A
  1. Shiga producing E coli. O157:H7 and Shigella (less commonly)
  2. Shiga toxin causes injury to small blood vessel endothelium leading to thrombotic microangiopathy and resultant renal vascular occlusion (afferent arteriole, glomerular capillaries) –> intrinsic acute kidney injury BUN/creatinine ratio <20:1, oliguria, HTN, hematuria, proteinuria PLUS RBC shearing through blood vessels occluded by systemic microthrombi (hemolytic anemia)
  3. supportive (fluid management, transfusion)
171
Q

Acute rheumatic fever is a complication that occurs 2-4 wks after an episode of untreated GAS pharyngitis –> dx is based on 2 major or 1 major + 2 minor criteria

  1. what is 5 major criteria
  2. 4 minor criteria
  3. elevated labs?
A
  1. JONES:
    –Joints (migratory arthritis),
    –<3 (O) carditis;
    –Nodules (subcutaneous);
    –Erythema marginatum (annular pink rash with sharp, raised edges and central clearing);
    —Sydenham chorea
  2. Fever, arthrlagias, Elevated ESR/CRP, Prolonged PR interval
  3. Elevated ESR and CRP
172
Q
  1. What is the anion gap equation
  2. What is normal anion gap value
A
  1. Na - (Cl + HCO3)
  2. 10-14
173
Q

Renal tubular acidosis Type 2
1. pathophysiology
2. urine pH range?

A
  1. inherited or acquired defect in the ability of the renal tubules to reabsorb HCO3- so HCO3- in labs is decreased and makes this metabolic acidosis
  2. urine pH > 7 if plasma bicarbonate concentration is normal, and a urine pH < 5.5 if plasma bicarbonate concentration is already depleted as a result of ongoing losses
174
Q

Renal tubular acidosis Type 1 (Fanconi)
1. pathophysiology
2. urine pH range?

A

1.inherited or acquired defect in the ability of the renal tubules to excrete H+ so you get metabolic acidosis
–comes with growth failure due to chronic acidosis
2. urine ph >5.5

175
Q

Spondylosis
1. what is this?
2. who typically gets this?
3. symptoms
4. what confirms dx

A
  1. unilateral or b/l defect (fatigue fracture) in the pars interarticularis that is commonly caused by overuse injury in athletes who perform repetitive back extension and rotation
  2. gymnasts
  3. pain with lumbar extension, occasionally radicular pain if compresses nerve roots, worsens with activity and improves with rest
  4. plain x ray films
176
Q

diabetic mothers vs gestational diabetes affect infants differently?
1. Changes in trimesters
2. effects after birth

A
  1. First trimester fetus cannot produce insulin so cannot protect from hyperglycemia that mother with DM causes - this can cause malformations (congenital heart disease). Infants with moms with gestational diabetes are not at increased risk for this bc hyperglycemia does not occur until after organogenesis is complete.

–2nd/3rd trimesters: Hyperglycemia can now trigger insulin which results in increased glycogen and fat storage (organomegaly), increased growth factor production, increased oxygen consumption (polycythemia)

  1. After delivery fetal insulin production takes time to decrease and this leads to neonatal hypoglycemia until insulin normalizes.
177
Q

Bronchopulmonary dysplasia
1. pathogenesis
2. dx
3. tx

A
  1. premature arrest of pulmonary development leads to alveolar hypoplasia with decreased septation and impaired vasculogenesis
  2. premature infant with continued supplemental oxygen requirement >= 28 days from birth
  3. supportive
178
Q

Multiple endocrine neoplasia
1. type 1

A
  1. primary hyperparathyroidism (parathyroid adenomas or hyperplasia)
  2. pituitary tumors (prolactin, visual defects)
  3. pancreatic tumors (esp gastrinomas)
179
Q

Multiple endocrine neoplasia
1. type 2

A
  1. medullary thyroid cancer
  2. pheochromocytoma
  3. Primary hyperparathyroidism (parathyroid hyperplasia)
180
Q

Multiple endocrine neoplasia
1. type 3

A
  1. medullary thyroid cancer
  2. pheochromocytoma
  3. mucosal neuromas/marfanoid habitus

-with risk of MTC prophylactic thyroidectomy is often done (age<1 yr)

181
Q

When a UTI is suspected (usually E coli.) based on clinical presentation and urinalysis, empiric antibiotics are recommended because a delay in therapy increases the risk of renal complications (eg, scarring, hypertension).

To cover the most common pathogens, children are typically treated with (BLANK)

A

a third-generation cephalosporin, such as cefixime

–> there are increasing rates of resistance amongE colito trimethoprim-sulfamethoxazole, first-generation cephlosporins, and amoxicillin-clavulanate.

182
Q
  1. painless lymphadenopathy that is typically nontender, rubbery, and firm; most often occurs in the cervical and axillary areas
  2. mediastinal mass that is often asymptomatic at dx but may cause chest pain, cough, or SOB
  3. B symptoms: fever, night sweats, weight loss
  4. Reed-sternberg cell on biopsy of lymph node

-what is this?

A
  1. Hodgkin Lymphoma
  • hepatomegaly and/or splenomegaly
  • other: leukocytosis, eosinophilia and/or >=1 cytopenia
  • LDH and inflammatory markers are often elevated
  • PET scan is required for staging
183
Q

Clinical features
- 3-4 days of nonspecific fever, headache, myalgia, arthralgia
- followed by macular & petechial rash on wrists/ankles that spreads toward the center of body
- can develop encephalitis, pulmonary edema, bleeding, shock

Lab findings
- decreased platelets
- decreased sodium
- increased AST and ALT

  • What is it?
  • What is treatment
A
  1. Rocky mountain spotted fever
  2. Doxycycline
184
Q

Why is serum fibrinogen good to check in with meningococcal meningitis?

A
  • when potentially fatal it can lead DIC
  • DIC (overwhelming activation of coagulation and fibrinolysis, leading to clotting factor and platelet consumption) – manifestations include oozing at IV site, GI hemorrhage, and purpura.
    –Labs would show thrombocytopenia, elevated PT and PTT, and low fibrinogen
    –management is supportive
185
Q
  1. premature adrenarche
  2. premature thelarche
A
  1. isolated pubic hair development
  2. isolated breast development

*both have normal bone age

186
Q

What is premature adrenarche is caused by?

A
  1. early activation of adrenal androgens
    – manifestations include body odor, oily skin, acne, and pubic and axillary hair
    –Estrogen and Testosterone levels remain normal
    –common in obese children because adiposity can trigger excess insulin production, which then stimulates the adrenal glands to produce sex hormones
187
Q

At what level of unconjugated bilirubin should exchange transfusion be started?

A

> 25 mg/dL total bilirubin

+evaluate for pathologic causes

188
Q

Drug-induced, immune mediated hemolytic anemia
–> acute anemia, dark colored urine, and normal coagulation studies

  1. most common triggers include NSAIDs, (BLANK A), (BLANK B)
A

Blank A: cephalosporins (ceftriaxone)
Blank B: penicillins (piperacillin-tazobactam)

189
Q

Although drugs can cause hemolysis through a variety of mechanisms (eg, oxidative injury, complement-mediated lysis), penicillins and cephalosporins trigger hemolysis when the drug binds directly to the surface of erythrocytes and creates a (BLANK A) for IgG attachment.

–> IgG-coated erythrocytes are then identified by the Fc receptor on splenic macrophages, leading to partial or whole erythrocyte phagocytosis (extravascular hemolysis).

A

–> hapten

190
Q

what repeat is in myotonic dystrophy?

A

CTG trinucleotide repeat

191
Q

Most cases of FGR are a result of uteroplacental insufficiency, which is impaired blood flow to the fetus with subsequent decreased oxygen and nutrient delivery. Fetuses adapt to a hypoxic, nutrient-depleted in utero environment by shunting blood from nonvital organs (eg, liver, subcutaneous fat) to preserve blood flow to vital organs (eg, brain), which can lead to the following complications at birth
1. (BLANK A)
2. Hypoglycemia
3. (BLANK B)
4. Hypocalcemia
5. Perinatal asphyxia and meconium aspiration

A

Polycythemia: chronic hypoxia induces increased erythropoietin and red blood cell expansion

Hypoglycemia: chronic nutrient deprivation results in lower plasma glucose and insulin concentrations, leading to decreased liver and muscle glycogen stores

Hypothermia: shunting blood to vital organs causes a decrease in subcutaneous fat and increased heat loss

Hypocalcemia: placental dysfunction decreases calcium transfer to the fetus

Perinatal asphyxia and meconium aspiration: growth-restricted infants have a difficult respiratory transition following birth and may experience hypoxic events leading to organ damage; placental dysfunction can cause intrauterine stress during labor and increase the likelihood of meconium-stained fluid

192
Q
  1. Fetal growth restriction is defined as estimated fetal weight (blank) percentile

OR
2. birth weight (BLANK) percentile for gestational age

A
  1. Fetal growth restriction is defined as estimated fetal weight <10th percentile

OR
2. birth weight <3rd percentile for gestational age

193
Q

Rett syndrome has microcephaly: TRUE or FALSE

A

True - it is an additional finding

194
Q

Symmetric vs asymmetric fetal growth restriction
1. onset
2. etiology
3. clinical features
4. management for
—Glucose levels
—temp control
—RBC levels
–Calcium levels

A

Symmetric
1. 1st trimester
2. chromosomal abnormalities or congenital infection
3. global growth lag

Asymmetric
1. 2nd/3rd trimester
2. Uteroplacental insufficiency or maternal malnutrition
3. Head sparing growth lag

4 for both
–Hypoglycemia: frequent screen and feedings
–Hypothermia: skin to skin with mom, exam in incubator
–Polycythemia and hypocalcemia: screen if poor feeding, vomiting, jitteriness

195
Q
  1. loss of function, repeat of GAA
  2. neurologic deficits (cerebellar ataxia, dysarthria/difficulty speaking, loss of vibration and/or position sense, absent deep tendon reflexes)
  3. Hypertrophic cardiomyopathy
  4. skeletal deformities (e.g. scoliosis)
  5. Diabetes mellitus
  • what is this?
A
  1. Friedreich ataxia - GAA repeat in frataxin gene
    –mean survival age 30-40
    - mortality due to cardiac dysfunction (e.g. arrhythmia, congestive heart failure)
196
Q
  1. what are the two serologic testing for syphilis?
  2. What else can be used to test?
A
  1. Venereal Disease Research Laboratory (VDRL) and RPR (rapid plasma reagin) test
  2. Darkfield microscopy: treponema pallidum in infectious material
197
Q
  1. erythematous papules, plaques. Spares skinfolds
  2. Beefy-red, confluent plaques. Involves skinfolds. Satellite lesions.
  3. Bright, sharply demarcated erythema of perianal/perineal area

–which one is candida diaper dermatitis, perianal streptococcus, irritant contact diaper dermatitis
–how is each treated?

A
  1. Irritant contact diaper dermatitis — Topical barrier (e.g. petrolatum, zinc oxide)
  2. Candida diaper dermatitis - Topical antifungal (e.g. nystatin)
  3. Perianal streptococcus - Oral antibiotics (e.g. amoxicillin). (this is mostly infants through school aged children)
198
Q

lab findings in diabetic ketoacidosis in children
1. glucose levels
2. bicarb
3. pH
4. anion gap
4. serum/urine ketones

–how to manage?

A
  1. glucose >200 mg/dL
  2. bicarb <15 mEq/L
  3. pH <7.3
  4. Anion gap >14

–manage
- 10 mL/kg isotonic fluid bolus over 1 hour
- insulin infusion + isotonic fluids with potassium

199
Q

normal hemoglobin is a heterotetramer composed of what?

A
  1. 2 alpha and 2 beta chains

– fetal hemoglobin is 2 alpha and 2 gamma chains

200
Q

Alpha thalassemia
- various types depending how many of the 4 alpha genes are deleted
1. What is 1 gene loss called?
2. What is 2 gene loss called?
3. What is 3 gene loss called
4. what is 4 gene loss called?

*and clinical features of each

A
  1. Alpha thalassemia minima - asymptomatic
  2. Alpha thalassemia minor - mild MICROCYTIC anemia
  3. Hemoglobin H disease - chronic hemolytic anemia
  4. Alpha thalassemia major, Hemoglobin Barts disease - hydrops fetalis/high output cardiac failure/death in utero
201
Q

Hemoglobin H disease (when 3 genes are loss –> alpha thalassemia)
1. Nonalpha chains accumulate into homotetramers made up of…
—> in adults
—-> in infants

A
  1. in adults these homotetramers are made up of beta chaiins
  2. in infants/fetuses these homotetramers are made up of gamma chians (also called hemoglobin barts)
202
Q

Hemoglboin H disease
– chronic hemolysis due to shorter RBC lifespan and increased splenic sequestration
1. Erythrocyte levels?
2. Micro or macrocytic or normocytic?
3. Shape of RBC in peripheral smear

A
  1. Elevated erythrocyte and reticulocyte levels to upkeep with constant hemolysis
  2. Microcytic (<80) and hypochromic because hemoglobin accounts for majority of RBC volume
  3. Target cells - result of reduced RBC volume
203
Q
  • Recurrent severe viral, fungal, or opportunistic infections
  • failure to thrive
  • chronic diarrhea

+ failure of T cell develop and B cell dysfunction due to absent T cells

  • what is this?
A
  1. Severe combined immunodeficiency
  • treated with stem cell transplant
204
Q

Scarlet fever
- what causes this
- what are clinical features
- what is treatment

A
  1. strep pyogenes
  2. Fever, pharyngitis
    - tonsillar erythema and exudates
    - strawberry tongue
    - tender anterior cervical nodes
    - sandpaper rash
  3. Penicillin
205
Q

what are the x-ray findings for a baby with respiratory distress syndrome?

A
  1. diffuse, reticular, ground glass opacities and air bronchograms
206
Q
  1. What is a myelomeningocele?
  2. What are some associated abnormalities?
A
  1. failure of neural tube closure which leads to protrusion of meninges, CSF And spinal cord through skin.
  2. Chiari II malformation (inferior displament of medulla and cerebellum through foramen magnum, obstructive hydrocephalus)
207
Q

Rheumatic heart disease is chronic valvular damage (e.g. mitral regurg/stenosis) resulting from one or more episodes of acute rheumatic fever

– these patients are increased risk of recurrence and progression of RHD with repeated episodes of GAS phayrngitis
–what prophylaxis should be given?

A
  1. Intramuscular benzathine penicillin G q 4 weeks to prevent recurrence
  2. Also treat at time of illness to eradicate GAS
208
Q
  • situs inversus
  • recurrent sinusitis
  • bronchiectasis

–> what is this?

A
  1. Kartagener syndrome - subgroup of primary ciliary dyskinesia. Autosomal recessive - dysmotile cilia that results from the aberrant production or attachmetn of ciliary dynein arms. This mutation cause impaired cilial function, poor mucociliary clearance of secretions, and chronic infections
209
Q

What vaccinations are necessary at birth?

A

Hep B

210
Q

What vaccinations are necessary at two months

A

B DR HIP

  1. Hep B (#2/3)
  2. DTaP (diptheria, tetanus, pertussis) (#1/5)
  3. Rotavirus (#1/3)
  4. HIB (H. influenzae type B) (#1/4)
  5. IPV (polio) (#1/4)
  6. PCV (pneumococcal - strep pneumo) (#1/4)
211
Q

What vaccinations are necessary at 4 months?

A

DR HIP

  1. DTaP (diptheria, tetanus, pertussis) (#2/5)
  2. Rotavirus (#2/3)
  3. HIB (H. influenzae type B) (#2/4)
  4. IPV (polio) (#2/4)
  5. PCV (pneumococcal - strep pneumo) (#2/4)
212
Q

What vaccinations are necessary at 6 months?

A

B DR HIP

  1. Hep B (#3/3)
  2. DTaP (diptheria, tetanus, pertussis) (#3/5)
  3. Rotavirus (#3/3)
  4. HIB (H. influenzae type B) (#3/4)
  5. IPV (polio) (#3/4)
  6. PCV (pneumococcal - strep pneumo) (#3/4)

*same as two months!
* can now get first flu shot

213
Q

What vaccines are necessary for 1 - 1.5 years old?

A

MAD HPV

  1. MMR (#1/2)
  2. A (hep A)
  3. DTaP (#4/5)
  4. H (HIB) (#4/4)
  5. P (pneumococcal) (#4/4)
  6. V (varicella) (#1/2)
214
Q

What vaccines are necessary for 4-6 years old?

A

VERY DIM
1. VERY –> Varicella (#2/2)
2. DTaP (#5/5)
3. I (IPV/polio) (#4/4)
4. MMR (#2/2)

215
Q

What vaccines are necessary at 11-12 years old?

A

Tada!! Human Men!!

  1. TDaP (tetanus, dip, acellular pertussis) - this is like DTap but for older chlidren
  2. HPV
  3. Meningococcal
216
Q

What vaccines are given at 16-18 years old?

A

Men
1. meningococcal booster

217
Q

APGAR Score
1. A =
2. P =
3. G =
4. A =
5. R =

and include how many points for each?

A

APGAR Score
1. A = appearance/skin color
—2: pink
–1: peripheral cyanosis only
-0: cyanotic/pale all over/central cyanosis

  1. P = pulse
    —2: over 100 bpm
    –1: <100
    -0: no pulse
  2. G = grimace (reflex irritability)
    —2: cry when stimulated
    –1: grimace or weak cry when stimulated
    -0: no response to stimulation
  3. A = activity/tone
    —2: well flexed and resisting extension
    –1: some flexion
    -0: floppy
  4. R = respiration
    —2: strong cry
    –1: slow, irregular breathing
    -0: apneic
218
Q

Primary vesicoureteral reflux in children
1. Renal US shows hydronephrosis
2. VCUG shows retrograde urine flow into the ureter and collecting system with blunting of renal calyces
3. what is the next step in management?

A
  1. Mild: Observation and prophylactic antibiotics
    —spontaneous resolution with growth
  2. severe: prophylactic antibiotics and surgery
    —without intervention pt will get scarring and chronic kidney disease
219
Q

Hypoxic ischemic brain injury can cause neuronal cell death within minutes
–> this can lead to what changes in pt presentation, vitals?

A
  1. leads to cerebral edema which increases ICP and compresses small arterioles, decreasing cerebral blood flow
  2. Consists of the HTN: body trying to maintain BP high enough to perfuse brain
    —bradycardia – hypertension stimulates baroreceptors to slow down heart (Parasympathetic resposne)
220
Q

immune thrombocytopenic purpura
1. pathophysiology
2. clinical features
3. treatment in children vs adults

A
  1. platelet autoantibodies, preceding viral infection
  2. petechiae, ecchymosis, mucosal bleeding
  3. Children: observation if cutaneous symptoms only, Glucocorticoids/IVIG/or anti-D if bleeding
    —-ADULTS
    –observation if cutaneous symptoms and platelets >30,000 then Glucocorticoids/IVIG/or anti-D if <30,000 platelets
221
Q

slipped capital femoral epiphysis (SCFE)
– most often seen in what population (2)
–definitive treatment?

A
  1. obese, adolescent (10-14) boys
    - patients with hypothyroidism (<10 years) but often bilateral
  2. surgical screw fixation to stabilize the physis and prevent further slippage
222
Q

Treatment for acute, unilateral cervical lymphadenitis?

A
  1. antibiotic therapy like clindamycin or amoxicillin-clavulanate. I&D if abscesses present
    — most commonly due to staph aureus and strep pyogenes
223
Q

How does FSH help distinguish between central and peripheral causes of amenorrhea?

A
  1. Typically, in central causes, FSH levels are low or normal because the pituitary gland fails to release enough FSH to stimulate ovarian follicles. Common central causes include hypothalamic dysfunction (e.g., due to stress, excessive exercise, or eating disorders) or pituitary tumors affecting FSH secretion.
  2. Peripheral causes of amenorrhea involve issues within the ovaries themselves, disrupting the production or response to sex hormones. In these cases, FSH levels may be elevated as the pituitary gland tries to stimulate the ovaries to produce estrogen. You still don’t get menstruation though because you need estrogen to help with these changes.
224
Q

absent breast development indicates lack of what hormone?

A

estrogen

225
Q
  • Th2- mediated inflammatory response triggered by food antigen exposure. Often comorbid atopy occurs
  1. Presentation
    — toddler: feeding difficulties (solid food refusal), weight loss
    —school aged child: abdominal pain, vomit
    —adolescent: dysphagia, heartburn, food impaction
  2. What is this?
  3. diagnosis steps?
  4. Treatment?
A
  1. Eosinophilic esophagitis in children
  2. endoscopy with esophageal biopsy (eosinophils >15/hpf)
    —exclusion of alternate diagnosis
  3. elimination diet, pharm therapy (PPI, topical glucocorticoid)
    –feeding therapy
226
Q

Risk factors for lead poisoning
1. house built before (BLANK A)
2. PICA or mouthing behaviors
2. siblings with lead poisoning
3. Low SES
4. Immigrant or international adoptee

  • When is lead levels check in children?
A
  1. 1978
  2. 6 months, 9 months, 12 months, 18 months, 24 months, and at 3, 4, 5 and 6 years of age - earlier months are more often done
227
Q
  1. capillary (fingerstick) blood specimens are widely used in screening for lead poisoning but what should be tested for confirmation?
  2. what levels should chelation be done?
  3. what is used for chelation?
A
  1. venous lead measurement should be done if screening lead level is >=5 ug/dL
  2. > = 45 ug/dL
  3. Dimercaptosuccinic acid (succimer) is typically used when lead levels are 45-69 µg/dL.

— Dimercaprol (British anti-Lewisite) plus calcium disodium edetate (EDTA) should be administered on an emergency basis for levels ≥70 µg/dL or acute encephalopathy.

228
Q

What is involved in the WAGR syndrome?
1. gene deletion on chromosome (BLANK)
2. W, A, G, R stand for?

A
  1. chromosome 11p13
  2. w - wilms tumor (tumor on kidney- nephroblastoma, often bilateral and present in age 1-3)
    a- aniridia (no irises)
    g - genitourinary abnormalities
    r - mental retardation/intellectual disability
229
Q

What are the common 3 innocent murmurs?
1. Still murmur:
2. pulmonic flow murmur:
3. venous hum:

–describe them?

A
  1. Still: systolic, vibratory, best heard over the left lower sternal border, increases intensity when supine because blood flow increases to heart
  2. Pulmonic flow murmur: systolic ejection best heard over left upper sternal border, may radiate to the axilla
  3. venous hum: continuous, best heard over supraclavicular or infraclavicular areas, decreased intensity when supine or with ipsilateral head rotation. —murmur is causes because blood is going through a physiologic curvature of the jugular or subclavian veins. Decreases with intensity as head turns toward “bent” vein because of jugular vein compression + with supine
230
Q

meconium ileus has a strong association with what disorder?

A

Cystic fibrosis
–patient with meconium ileus and CF dx can be at increased risk of chronic rhinosinusitis

231
Q
  1. What is treatment for gonorrhea
  2. what is treatment for chlamydia
A
  1. ceftriaxone
  2. doxycycline
  • give both because often occur together
232
Q

How is torsion of appendix testis different from torsion of testis?
1. symptoms
2. tx

A
  1. groin pain, nontender testicle, tender/palpable localized mass adjacent to one pole of the testicle and an intact cremasteric reflex. May appear as small blue/black mass in scrotum.
  2. management is supportive
233
Q

What testicle abnormality reduces pain with testicle elevation?

A

epididymitis - usually caused by Chlamydia trachomatis or N. gonorrhea

234
Q

difference in pupils between opioid vs barbiturate intoxication?

A
  1. Barbiturate - pupils normal
  2. opiates - constricted (miosis)
235
Q

nasal decongestants if taken in large quantities can cause negative side effects because of what reason?

A
  1. Some use pseudoephedrine which is a sympathomimetic drug –> tachycardia, palpitations, anxiety
236
Q
  1. Differentiate between SVT and Vtach
A
  1. SVT shows narrow QRS complexes (220-280/min) - can be addressed with cardioversion for hemodynamically unstable patients or vasovagal tactics in hemodynamically stable patients
  2. Vtach has wide QRS complexes -
237
Q

What is leg-calves-perthes disease?

A
  1. idiopathic avascular necrosis of the femoral head that most commonly occurs in younger boys aged 4-9 years
    –slow onset of hip pain which is worsened by activity and associated with a limp
    - X rays show no abnormalities
    - Bone scan shows decreased perfusion of the femoral head

Tx: prevent weight bearing on affected leg + may require operative intervention or hip arthroplasty

238
Q

Aplastic anemia
1. micro, normo, or macrocytic?
2. What viruses can cause this?
3. treatment
4. what other important lab values?

A
  1. normocytic anemia
  2. Hepatitis, EBV, HIV, parvovirus
  3. immunosuppressive meds or hematopoietic stem cell transplant from a matched sibling if available?
  4. this causes pancytopenia - so leukopenia, anemia, thrombocytopenia
239
Q

Why do VSD murmurs get louder with age?

A
  1. Because the pulmonary vascular resistance decreases …so with less resistance from lungs then LV can push more blood into RV(right side of heart).
    —Why when babies are in utero…U/S can’t find VSDs that well because PVR is high and LV has more resistance and able to push less blood through VSD into RV
240
Q
  1. antithyroid peroxidase antibodies
  2. antithryoglobulin antibodies
  • these antibodies are seen typically in what disease?
  • sx?
A
  1. Hashimotos (hypothyroidism) thyroiditis
  2. fatigue, cold intolerance, weight gain, hyporeflexia, and dry/cool skin. May have neck discomfort but thyroid is not usually tender
241
Q
  1. Avg weight for newborns
  2. Avg length
  3. Average head circumference
A
  1. between 5 pounds, 8 ounces (2,500 grams) and 8 pounds, 13 ounces (4,000 grams)
  2. 49-50 centimeters
  3. 34.5 cm (13.5 in.)
242
Q
  1. Needs 4/11 criteria to meet dx
    which are…
  2. malar rash
  3. Photosensitivity
  4. Discoid rash
  5. painless oral ulcers
  6. Arthritis
  7. serositis (pleuritis or pericarditis)
  8. neurologic disorder (seizures or psychosis)
  9. renal impairment
  10. Hematologic abnormalities (hemolytic anemia, leukopenia, lymphopenia, or thrombocytopenia)
  11. lupus specific autoantibodies: Anti DND, anti-smith, antiphospholipid antibody
  12. Abnormal antinuclear antibody

what is this?

A

SLE (systemic lupus erythematosus)

243
Q

(Blank) play an important role in the control of fungal organisms, thus dysfunction in this causes increase risk for both mucocutaneous and invasive fungal infections

A
  1. T lymphocytes
    –cytotoxic CD8+ T lymphocytes, Th1 cells, and Th17 cells play a role
244
Q

Heritable disease of peripheral neuropathy - Autosomal dominant mutations in genes expressed in Schwann cells.
- Motor weakness, decreased sensation to vibration and proprioception most often in lower extremities
- Hyporeflexia/areflexia
- Clumsy walking and MSK deformities like pes cavus and hammer toes

  • What is this?
  • How is it dx?
  • How is it often tx?
A

Charcot-Marie-Tooth disease
- dx via nerve conduction studies

  • Physical therapy is treatment
245
Q

Progressive infantile spinal muscular atrophy (SMA)

  • autosomal recessive mutation of the SMN protein, resulting in defective mRNA synthesis in motor
    neurons.
  • Age: Blank
  • Symptoms: Blank
  • Treatment is supportive and includes promoting nutrition, motor, and respiratory function.
A
  • Age: The infantile form of SMA typically affects normally developing infants who are several months old.
  • Symptoms: progressive, symmetric muscular weakness and a lower motor neuron-pattern of dysfunction (eg, decreased tone, hyporeflexia, fasciculations, early-onset atrophy). The lower extremities are typically more
    affected than the upper extremities. Dysfunction progresses over the course of months. In severe cases, progressive respiratory insufficiency may develop.
246
Q

Myasthenia gravis

— is an autoimmune disorder of neuromuscular transmission that may result in focal or generalized muscle weakness and fatigability.

  • Age: Blank
A

Age: Myasthenia gravis demonstrates a bimodal age of
onset in both young adults (teens to 20s) and older adults (50s to 60s).

247
Q

X-ray findings of small
bowel obstruction include dilated loops of bowel with air-fluid levels indicate (BLANK)

A

Volvulus

248
Q

Histoplasmosis is an endemic mycosis secondary to infection with Histoplasma capsulatum.

  1. Pulmonary histoplasmosis can present in acute, subacute, or chronic phases, generally characterized by (Blank)
  2. Transmission occurs through inhalation of the organism’s fungal spores.
  3. It is endemic to the (BLANK) River valleys in the United States, as well as parts of Central and South America, sub-Saharan Africa, and Southeast Asia.
  4. Risk factors for histoplasmosis include exposure to (BLANK).
  5. Diagnosis is typically made via the detection of histoplasma antigen in the serum and/or urine. Histology will disclose oval yeast cells within macrophages and caseating, or necrotizing, granulomas.
A
  1. fevers, malaise, myalgia, cough, and hemoptysis with the associated development of small calcified nodules and mediastinal or hilar lymphadenopathy.
  2. Mississippi and Ohio
  3. bird or bat droppings
249
Q

Q fever is a vector-borne illness caused by infection with Coxiella burnetii.

  1. Coxiella burnetii is acquired through the inhalation of spores from (BLANK)
  2. Q fever commonly presents as pneumonia or (BLANK).
A
  1. cattle or sheep amniotic fluid
  2. pneumonia or endocarditis.
250
Q

(BLANK) is hypothesized to decrease the rate of food allergy in infants.

A

Exclusive breast-feeding for at least 6 months is hypothesized to decrease the rate of food allergy in infants.

251
Q

Treatment for CO poisoning begins with (BLANK)

Sx?

A

the administration of 100% oxygen.
–sx– headache, loss of consciousness, dizziness, vomiting, and fatigue; in severe cases, seizures, dysrhythmias, coma, and death may occur. Examination may show red skin.

252
Q

thyroid-stimulating antibodies
–what is this found in ?

A

Graves disease

253
Q
  1. child with mild anemia, microcytosis, and low red cell distribution width (RDW) who is of southeast Asian heritage most likely (BLANK)
  2. Supportive features include the presence of target cells on peripheral blood smear, a low RDW (which indicates that most erythrocytes are of the same size with little variation), and persistence of microcytosis.
A
  1. either a- or b -thalassemia minor. Thalassemia trait is common in southeast Asia and commonly presents with
    mild microcytic anemia.

–Iron concentrations can be measured to rule out iron deficiency.
–Other cell lines including platelets and white blood cells should be within normal ranges.
–There is no treatment required for thalassemia minor as the resultant anemia is usually not severe.

254
Q

Patient with SCD
– Peripheral oxygen delivery is based on the arterial concentration of hemoglobin and (BLANK).

A
  1. the cardiac output

– In states of severe anemia, where the hemoglobin concentration is low, cardiac output
must increase in order to compensate, resulting in a high-output cardiac state, as seen in this patient.

255
Q

A luteal phase progesterone concentration that is below the cutoff value is indicative of (BLANK).

A

Anovulation
—this can lead to abnormal and heavy uterine bleeding. —A luteal phase progesterone concentration that is below the cutoff value is indicative of anovulation.

256
Q

Aspirin toxicity
1. Aspirin is an antiplatelet agent that irreversibly inactivates cyclooxygenase, which prevents the conversion of arachidonic acid to thromboxane A2: Thromboxane A2 is involved in platelet activation and its decreased synthesis results in diminished platelet activation.

  1. salicylate poisoning in large doses results in a (BLANK) from the stimulation of respiratory centers causing hyperventilation.
  2. Subsequently, it results in the development of a delayed (BLANK)
  3. It does not typically affect sodium and potassium serum concentrations.
  4. Sx:
  5. Management of salicylate toxicity involves systemic alkalinization with a sodium bicarbonate infusion. Patients may need dialysis in the setting of severe altered mental status, end-stage kidney disease, pulmonary edema, or clinical deterioration.
A
  1. respiratory alkalosis
  2. anion-gap metabolic acidosis d/t decoupling effects on the electron transport chain, resulting in increased anaerobic metabolism and the production of lactate and ketoacids beyond the acidic properties of salicylic acid itself.
  3. Patients with salicylate toxicity will present with hyperventilation, gastrointestinal upset, hyperthermia, tinnitus, agitation, delirium, and hallucinations.
257
Q

Urethral injuries can occur from straddle injuries (anterior urethra) or pelvic fractures (posterior urethra). 1. (BLANK) permits evaluation of the urethra as a source of hematuria.
2. What is contraindicated

A
  1. Retrograde urethrography
  2. Insertion of a urinary catheter would be contraindicated as the first step in management in a patient with blood at the urethral meatus, portending a likely urethral injury. It may cause further trauma and
    injury to the urethra and internal pelvic structures without the injury first being delineated on retrograde urethrography.
258
Q

Anion gap metabolic acidosis (AGMA) is a common scenario in critically ill patients.
Common causes are often implied by the acronym, MUDPILES

A

(methanol, uremia, diabetic ketoacidosis, paraldehyde, iron overdose, lactic acid, ethylene glycol and salicylate)

259
Q
  1. What is the winters formula
  2. What is it for?
A
  1. The Winters formula is used to determine the range of pCO2 if compensation is adequate: 1.5(HCO3 ·) + 8 +/- 2
  2. when someone is in metabolic acidosis you would assume your body tries to fix this by respirations (breathing out CO2) - winters formula gives you what pCO2 in ABG should be if body is doing this adequately to treat metabolic acidosis. If pCO2 is not appropriate then likely you have a respiratory acidosis on top of alkalosis
260
Q

Severe hyperkalemia leads to
decreased myocardial excitability resulting in repolarization abnormalities, conduction dysfunction, heart block, and bradycardia.

–> These abnormalities can manifest on an ECG with peaked T waves, PR interval
prolongation, loss of P waves, and, if severe, widened QRS complex and bradycardia.

–> Hyperkalemia resulting in ECG changes requires the emergent administration of (BLANK)

A
  1. calcium gluconate or calcium chloride

*Acute renal failure can lead to hypertension and hyperkalemia

261
Q

What are parts of HEADSS exam?

A

home, education (ie, school), activities/employment, drugs, suicidality, and sex

262
Q

Von willebrand multimer acts as a serum transport protein for what coagulation factor?

A

Factor VIII - which can cause isolated prolonged PTT in someone bleeding profusely/too long but originally this issue is with von willebrand factor

263
Q
  1. How does vitamin D play a role in calcium and phosphate levels?
  2. what about PTH?
A
  1. Vitamin D promotes intestinal absorption of calcium and phosphate
  2. PTH stimulates osteoclastic bone reabsorption/breakdown and calcium reabsorption+phosphate excretion in kidney
263
Q
  1. what do levels of alkaline phsophatase indicate in bone changes?
A
  1. Increased osteoclast activity leads to increased bone turnover –> which gets you release of calcium and alkaline phosphatase into bloodstream
264
Q

What happens to LH and FSH in turner syndrome (45, X)

A
  1. LH and FSH are increased because there is lack of negative feedback from estrogen

—gonadal dysgenesis in turner syndrome is what leads to primary amenorrhea (never having a period after 15)

265
Q

Adrenal cortex
—glomerulosa
—fasciculata
–reticularis

-what does each section make?

A
  1. aldosterone
  2. cortisol
  3. dehydroepiandrosterone sulfate (17 alpha hydroxylase makes this)
266
Q

What is addison disease?

A

Primary adrenal insufficiency
–commonly due to autoimmune destruction

–> presents with hypotension, hyperkalemia, metabolic acidosis, and skin hyperpigmentation secondary to deficiency in cortisol and aldosterone

267
Q

What changes to potassium happen with renal tubular acidosis?

A

Kidney potassium wasting and hypokalemia

267
Q

Alpha 1 antitrypsin deficiency

  1. what is this?
  2. sx?
A
  1. alpha 1 antitrypsin regulates neutrophil elastase (a serine protease secreted by neutrophils and macrophages as a part of a localized inflammatory response)
  2. with less alpha 1 antitrypsin —–> neutrophil elastase is not regulated and leads to host tissue damage, especially in lungs

sx: panlobar emphysema, chronic lung disease with bibasilar emphysematous changes

268
Q
  1. What is lung sequestration?
  2. sx
A

Congenital lung malformation in which a segment of dysplastic, nonfunctional lung tissue is formed that does not communicate with rest of the airway

–present with recurrent pneumonia in same localized region
-persistent cough, hemoptysis, exertional dyspnea, and back pain

269
Q

–> Abnormal leukocyte degranulation (on histology it looks like large granules inside leukocyte)
– frequent bacterial infections
– oculocutaneous albinism
–peripheral neuropathy
–progressive neurologic dysfunction

what is this?

A

chediak-higashi syndrome

270
Q
  1. How to test for CMV infection?
  2. How to treat for CMV?
A
  1. viral culture or PCR from urine or blood within first week of life
  2. ganciclovir or valganciclovir
271
Q

human bites are associated with flora from where?

A
  1. mixed flora - from oral and skin surfaces

-staph aureus
-strep pyogenes
-eikenella corrodens
-various anaerobes

-aerobic + anaerobes

272
Q

endogenous (in the body) insulin release will be associated with increased insulin and (BLANK A)

A
  1. Blank - C peptide
273
Q

mutations in FGF3 gene leads to what diagnosis?

A
  1. achondroplasia

–failure of long bone growth leads to short stature and is seen with macrocephaly, frontal bossing, varus deformity, and hypotonia in infancy

274
Q

Germ cell tumors include seminoma, choriocarcinoma, teratoma, yolk sac tumor, and embryonal carcinoma

What will be increased in lab values ?

A
  1. alpha fetoprotein
  2. Beta hCG (this can lead to gynecomastia)

Note: Non-germ cell tumors are often sex cord-gonadal stromal tumors originating from Sertoli or Leydig cells.

275
Q

Gross motor: hop on one foot, throwing a ball overhand
Fine motor: draw a stick figure and square
Language: speak in full sentences that can be understood by others
Social: play cooperatively with others, having imaginary friends, telling complex stories, ID colors and numbers

A

4 years old

276
Q
A

M3 - Clerkships (8 decks)

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