Peds 10 Flashcards

1
Q

Pathogenesis of Duchenne muscular dystrophy

A

X-linked frameshift mutation causing a absent or truncated dystrophin gene

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2
Q

How do you diagnose Duchenne’s

A

DNA peripheral blood analysis and/or immunohistochemical detection of abnormal dystrophin on a muscle biopsy

Will also have elevated CK due to muscle breakdown

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3
Q

What is the most common cause of death on Duchennes

A

o Dilated cardiomyopathy is common cause of death

  • Respiratory failure is also a common cause
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4
Q

Inheritance of muscular dystrophy

A

X-linked recessive

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5
Q

What is a neuroblastoma

A

• Most common tumor of adrenal medulla in children

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6
Q

Describe metastatic spread of neuroblastoma

A

o Typically involves the long bone and skull, lymph nodes, liver, and skin
o Bluish skin discoloration represents subcutaneous infiltration
o Pulmonary involvement = respiratory distress
o Bone marrow involvement may cause bone pain and pancytopenia
o If orbital bones are involved, proptosis and bluish periorbital discoloration, described as “raccoon eyes”
o May present with opsoclonus-myoclonus syndrome (“dancing eyes-dancing feet”)

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7
Q

Describe lab markers of neuroblastoma

A

o Increased HVA and VMA in urine

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8
Q

Diagnostic test of choice for malrotation with volvulus

A

Upper GI - will show “corkscrew” pattern of the duodenum or “bird’s beak” of the 2nd or 3rd portion of the duodenum

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9
Q

Tx of malrotation with volvulus

A

Emergent surgery

Preceded by evaluation of fluid status

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10
Q

Describe posterior urethral valve

A

o An obstructing membrane in the posterior male urethra as a result of abnormal in utero development
o Most common cause of bladder outlet obstruction in male newborns

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11
Q

Describe management of male neonate with UTI

A

Evaluation of anatomy and function is necessary - renal US and voiding cystourethrogram should be performed

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12
Q

Most common infections caused by Klebsiella, Enterobacter, and Serratia

A

Pneumonia and UTI

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13
Q

Tx of Klebsiella, Enterobacter, and Serratia

A

Are often multi-drug resistant

Can treat with Carbapenem (Amy’s Hover car repair when others faiL)

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14
Q

Describe signature feature of Klebsiella

A

Currant jelly sputum with pneumonia

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15
Q

Describe infectivity strength of Salmonella vs. Shigella

A

Salmonella is acid labile - need high dose to cause infection

Shigella is acid stable - low dose can infect

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16
Q

What part of the body is chronic salmonella carried in

A

Gall bladder

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17
Q

Tx of Salmonella

A

Fluoroquinolones

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18
Q

What type of diarrhea is caused by Salmonella

A

S. Typhi = pea soup diarrhea

S. Enteritidis = inflammatory diarrhea

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19
Q

Describe HUS caused by E. Coli and Shiga toxin

A
  • Toxin causes endothelial damage in glomerulus
  • This activates platelets and causes their aggregation leading to a drop in platelet count
  • Aggregated platelets lyse RBCs
20
Q

Why is E. Coli so good at causing UTIs

A

Because of fimbriae

21
Q

Diseases caused by E. Coli

A

UTI, sepsis, meningitis, watery diarrhea (ETEC), bloody diarrhea (EHEC), HUS

22
Q

Mode of transmission of yersinia enterocolitica

A

Puppy feces and contaminated milk products

23
Q

Presentation of Yersinia enterocolitica

A
  • Bloody diarrhea
  • Abd pain may mimic appendicitis
  • Invasive systemic effects like fever, intestinal issues, leukocytosis, abscesses, bowel issues
24
Q

Mode of transmisson of Yersinia Pestis

A

Human as incidental host, usually through fleas bites from rats or prairie dogs

25
Q

Presentation of Yersinia Pestis

A
  • Bubonic plague = fever, chills, weakness, HA, followed by pain and swelling in lymph node
  • Septicemic plague = fever, GI sx, hypotension, DIC, multiorgan failure
  • Pneumonic plague = fever, dyspnea, pleuritic chest pain, cough, maybe bloody sputum
26
Q

Treatment of Yersinia

A

Aminoglycosides or Tetracyclines

27
Q

Mode of transmission of Campylobacter

A

Fecal oral, contaminated water supplies, ingestion of raw milk

28
Q

Diseases caused by Campylobacter

A
  • Bloody diarrhea
  • Invasive bacteremia
  • Reactive arthritis (Reiter’s syndrome)
  • Guillam barre (ascending paralysis)
29
Q

What are the 3 curved, or comma shaped, bacteria

A

Campylobacter, Vibrio, Helicobacter

30
Q

Describe how Vibrio bacteria attach in the gut

A

Vibrio attaches to the mucosa by fimbriae that attach to ganglioside receptors in the intestinal wall = NOT INVASIVE (vs. invasive Camplyobacter)

31
Q

Describe how Vibrio causes bacteria

A

o After attaching to mucosal wall, Vibrio then releases cholera toxin
♣ Toxin upregulates production of cAMP by binding to and constitutively activating adenylate cyclase in the Gs pathway (Gas canister)
• Activates GS = upregulates cAMP = Produces watery diarrhea through an efflux if Cl and H2O

32
Q

Tx of Vibrio Cholera

A

Oral rehydration therapy with electrolytes

33
Q

What is the main virulence factor of H. Pylori

A

Urease positive
o Urease splits urea into ammonia and CO2 = Allows to reduce the acidity of the stomach and allows Helicobacter to survive there

34
Q

What cancers are associated with H. Pylori

A

Gastric adenocarcinoma, MALToma

35
Q

Tx of H. Pylori

A

PPI + Amoxicillin + Macrolide

36
Q

What type of bug is Pseudomonas

A

Gram negative rod

37
Q

Diseases caused by Pseudomonas

A
  • Pneumonia and pulmonary infections in CF pts
  • Osteomyelitis (especially in diabetics and IV drug users)
  • UTI
  • Skin lesions (hot tub folliculitis, ecthyma grangrenosum)
  • Otitis externa (swimmer’s ear)
  • Especially affects burn patients
38
Q

Tx of Pseudomonas

A

Piperacillin + Tazobactam, Aminoglycosides, Fluoroquinolones

39
Q

Tx of neisseria

A

Ceftriaxone

40
Q

Describe pathogenesis of nursemaids elbow

A

♣ Subluxation (aka partial dislocation) of the radial head
♣ Annular ligament is torn at the attachment site to the radius bone when sudden traction is placed on the child’s arm
♣ The radial head slips through the tear, and when the pulling motion has ceased, the radial head recoils with a small portion of the annular ligament trapped between the radius and humerus

41
Q

How do you diagnose nursemaids elbow

A

♣ Clinical diagnosis – X-ray not necessary

42
Q

Tx of nursemaids elbow

A

♣ Supinate the forearm with the elbow in in flexed position while applying pressure over the radial head – a click may be felt when the annular ligament is freed from the joint

43
Q

What shape should kids be able to draw at the following ages:
2, 3, 4, 5, 6, 7

A
2 = line
3 = circle
4 = cross
5 = square
6 = triangle
7 = diamond
44
Q

At what age should kids be able to stack the following amount of blocks: 2, 3, 4, 5

A
2 = 15 months
4 = 18 months
6 = 24 months (think 2+4=6)
9 = 3 years
45
Q

What type of play should kids be doing at the following ages: 1, 2, 3, 4, 5

A
1 = alone
2 = parallel play
3 = group play
4 = stories/imaginary friends
5 = cards/board games
46
Q

Describe stepwise tx of persistent asthma

A

1) Low dose inhaled glucocorticoids
2) Low dose inhaled glucocorticoids + LABA
3) Medium dose inhaled glucocorticoids + LABA
4) High dose inhaled glucocorticoids + LABA
5) High dose inhaled glucocorticoids + LABA + oral systemic glucocorticoids