Disease presentations and explanations Flashcards

1
Q

Cause of blood in the stool in HSP

A

These occur in approx 50% of patients, about 1 week after dx.
Edema of the GI tract can cause damage to the vasculature and result in colicky abdominal pain and bleeding. Ultimate cause: vasculitis causing enlarged mesenteric lymph nodes, bowel edema and hemorrhage

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2
Q

causes of secondary immunodeficiency

A

HIV infection, DM, malnutrition, hepatic disease, autoimmune disease, aging, stress

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3
Q

primary immunodeficiencies

A
  1. severe combined immunodeficiency
  2. chronic granulomatous disease (phagocytic cell def)
  3. complement deficiency
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4
Q

reduces vertical transmission of HIV

A

Zidovudine (antiretroviral) given in 2nd trimester then given to infant through 6 weeks reduces transmission to < 10%

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5
Q

preferred testing for HIV in neonate

A

HIV DNA PCR -> must be done this way due to maternal antibodies
** use ELISA followed by Western blot in 18 mos or older
Disease status eval: HIV RNA activity, CD4 count, clinical findings
“severe”: CD4 < 200
“normal”: CD4 >500

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6
Q

used to prevent PCP infection in HIV+ infants

A

TMP-SMX prophylaxis started at 6 weeks

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7
Q

Pediatric antiretroviral regimen for HIV+

A

2 nucleoside reverse transcriptase inhibitors and 1 protease inhibitor
Nucleoside reverse transcriptase inhibitors:
1. didanosine
2. stavudine
3. zidovudine
Nonnucleoside reverse transcriptase inhibitors:
1. efavirenz
2. nevirapine
Protease inhibitors:
1. indinavir
2. nelfinavir

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8
Q

erythematous macular rash with satellite lesions

A

cutaneous candidiasis

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9
Q

recurrent oropharyngeal and cutaneous infections with delayed wound healing
recurrent sinopulmonary infections

A

leukocyte adhesion deficiency (LAD)

be suspicious if have birth wounds that aren’t healing and attached umbilical cord at 6 weeks

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10
Q

diminished/absent T cells
diminished/absent serum immunoglobulins
thymic dysgenesis
recurrent cutaneous, GI or pulm infections (CMV, PCP)

A

SCID

AR or X-linked

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11
Q

Thymic aplasia
Boot-shaped heart on XR
atypical facies (wide-set eyes, prominent nose, small mandible)
cleft palate

A

DiGeorge Syndrome

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12
Q
hypogonadism
gynecomastia
long limbs
learning difficulties (spelling, reading, math)
often not dx until puberty
A

Klinefelter syndrome
(extra X in males) -> the more X’s the more MR -> check IQ
**Note increased breast cancer risk which approaches that for females

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13
Q

males display explosive temper and aggressive or defiant behavior
long asymmetrical ears

A

XYY

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14
Q

Child is exclusively fed goat’s milk. What are the complications?

A
megaloblastic anemia (from folate or B12 def); also is iron-poor
brucellosis (if not pasteurized)
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15
Q

congenital condition which the liver’s bile ducts are blocked and fibrotic, resulting in reduced bile flow into the bowel (since less bile salts have fat-soluble vitamin deficiency, ex: vit D)

A

biliary atresia
Surgical correction: Kasai procedure
Note: patient’s with liver failure are often treated with diuretics for ascites and this results in calcium loss in the urine -> higher risk for rickets)

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16
Q

Dental differences between rickets and familial hypophosphatemia

A

Rickets: enamel defects

Familial hypophosphatemia: intraglobular dentin deformities

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17
Q

lab value seen in all rickets cases

A

elevated serum alkaline phosphatase level

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18
Q

labs typical of low Vit D

A

decreased serum calcium and phosphorus

increased serum alkaline phosphatase and urine amino acids

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19
Q

caused by dystrophin gene deletion, resulting in proximal lower extremity weakness

A

Duchenne muscular dystrophy

-calf muscles show fat and fibrosis with absent dystrophin on immunochemistry staining

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20
Q
urinary tract anomaly
anuria/oliguria in utero 
oligohydramnios
pulmonary hypoplasia
flat facies
limb deformities
A

Potter sequence

decreased amniotic fluid can cause physical anomalies

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21
Q

most common cause of urinary tract obstruction in newborn boys

A

posterior urethral valves

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22
Q

Kawasaki disease vs Scarlet fever

A

Both have history of strep pharyngitis, but in scarlet fever it is “untreated strep pharyngitis”
Kawasaki has a blanching rash that includes the palms and soles. Swelling and erythema of the palms/soles.
Kawasaki has cervical lymphadenopathy of at least one lymph node.
Scarlet fever has a sandpaper rash that spares the palms and soles. It also has tonsillar exudates.

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23
Q

How much weight loss equals 1 L fluid loss?

A

1 kg

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24
Q
cool, clammy skin
capillary refill > 3s
cracked lips
dry mucous membranes
sunken eyes
sunken fontanelle
tachycardia
lethargy
A

severe dehydration (10-15% volume loss)

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25
Q
decreased skin turgor
dry mucous membranes
tachycardia
irritability
capillary refill 2-3 s
A

moderate dehydration (6-9%)

26
Q

sign on dehydration in diaper

A

brick-red urate crystals

27
Q

Blanching rash vs nonblanching rash

A

Blanching rashes: blanching suggests that the erythema is due to vasodilation and is seen with viral illnesses, drug eruptions, Kawasaki disease, roseola and scarlet fever.
Nonblanching rashes are from dermal hemorrhage: meningococcal disease and late in Rocky Mountain spotted fever

28
Q

starts with “pins and needles” then ascending weakness

flaccid paralysis with absent DTRs and nerve conduction abnormalities

A

Guillain-Barre

29
Q

edema, fatigue, abdominal pain and proteinuria

A

minimal change disease

30
Q

doll-like face, thin extremities, protuberant abdomen from enlarged liver and kidneys, short stature
seizures from hypoglycemia

A

glucose-6-phosphatase deficiency

31
Q

50,000 colony-forming units/mL in catheter-obtained urine specimen

A

bacteriuria

32
Q

greater than or equal to 5 WBCs in urine

A

pyuria

33
Q

stridor that improves with neck extension

associated with cardiac abnormalities

A

vascular ring

34
Q
metabolic acidosis with AG
hyperketonemia
hyponatremia
Elevated BUN & creatinine
may see elevated WBCs from infection causing exacerbation
A

DKA

35
Q

hemoglobin A1c goals for diabetics

A

6-9%

> 12% suggests poor control

36
Q

Other than DKA, what might cause glycosuria?

A

stress response to infection

37
Q

Special management of sickle cell disease (SCD)

A
  1. daily PCN by 2 months age
  2. daily folate by 6 months age
  3. pneumococcal and meningococcal vaccines by age 2
  4. watch for evidence of stroke -> treat with exchange transfusion to reduce the number of circulating sickle cells.
  5. routine CBC with retic count starting at diagnosis or 2 months
38
Q

complication of splenic sequestration

A

cardiovascular collapse

39
Q

coughing spells with quiet intervals, often heard with pertussis and chlamydial pnemonia

A

staccato cough

40
Q

causes of pleural effusion

A
  1. CHF
  2. mycobacterial pneumonia
  3. malignancy (lymphoma)
41
Q

most frequent cause of pediatric bacterial pneumonia

A

pneumococcus

42
Q

cause of “belly pain” in a child with pneumonia

A

irritation of the diaphragm by pulm infection

43
Q
diarrhea/defecation
urination
miosis
bradycardia
bronchorrhea
emesis/excitation of muscles
lacrimation
salivation
A
cholinergic excess (organophosphate poisoning)
Tx: atropine and pralidoxime
44
Q

symptoms of arsenic ingestion

A

nausea, vomiting, abdominal pain, diarrhea

45
Q

red or maroon-colored stools

A

hematochezia

46
Q

black tarry stools produced by oxidation of heme by intestinal flora

A

melena

47
Q

causes 50% of lower GI bleeds in children less than 2 yr

A

Meckel diverticulum

48
Q

most common cause of hematochezia in infants, children and adolescents

A

anal fissure

49
Q

treatment of AOM

A

amoxicillin 80-90 mg/kg/d for 7-10 days
if clinical failure after 3d, change to augmentin, cefuroxime axetil (Ceftin), azithromycin, cefixime, ceftriaxone, or tympanocentesis

50
Q

Wheezing vs stridor

A

wheezing is polyphonic, and stridor is monophonic

51
Q

drooling, preference to sit in a tripod position, muffled vocalizations, inspiratory stridor, absence of cough

A

epiglottitis

52
Q

vaccine associated with thrombocytopenia

A

MMR

give with caution to those with ITP

53
Q

most common infectious cause of congenital sensorineural hearing loss (SNHL)

A

prenatal CMV
Other infectious causes: toxoplasmosis, rubella, syphilis
Postnatal causes: GBS sepsis and S. pneumoniae meningitis

54
Q

partial albinism (white forelock), SNHL, lateral inner canthi displacement, eyebrow confluence, broad nasal bridge and mandible

A

Waardenburg syndrome

55
Q

absence of the lens

A

aphakia

56
Q

reflux with intermittent torticollis

A

Sandifer syndrome

These have abnormal head posturing associated with reflux

57
Q

Drugs that caused dystonia that may present like torticollis

A

phenothiazine, metaclopramide, haloperidol

58
Q

most common cause of severe urinary tract obstruction in boys

A

posterior urethral valves

Risk: end stage renal disease

59
Q

Conditions associated with undescended testis

A

Hernia, testicular torsion, testicular cancer, infertility

60
Q

elevated urinary vanillylmandelic acid and homovanillic acid levels
elevated enolase, ferritin, lactate dehydrogenase

A

Neuroblastoma
Presentation: abdominal mass, pallor, proptosis, “raccoon eyes”
Tumor is typically found in the adrenal gland
Poor prognosis: older children with bony mets, N-myc oncogene amplification

61
Q

Structures at risk with retropharyngeal abscess

A

Mediastinal