Flashcards in UWorld Deck (93):
1
What are light microscopy and immunofluorsence staining of glomeruli of someone with minimal change diease show?
light microscopy: normal renal architecture
Immunofluorescence staining: diffuse effacement of foot processes of podocytes on EM
Renal biospy shows normal kidney architecture; usu not obtained in pts
2
Turner syndrome (short stature, amenorrheic, coarctation if aorta) -what are lab values for FSH, LH, estrogen
Due to ovarian dysgenesis, estrogen is low which will feedback positively to FSH. FSH is high so is LH
3
Inhibin is a marker of ovarian function. High or low in Turner?
Low b/c of ovarian dysgenesis
4
Describe symptoms of neonatal tetanus
-seen in infants born to unimmunized mothers
-frequently follows UMBILICAL STUMP infection (PE: swollen and erythematous umbilical cord)
-first two weeks of life with poor suckling, fatigue, followed by rigidity, spasms, and opisthotonus
5
How does vomiting assoc with pyloric stenosis results in a hypochloremic, hypokalemic metabolic alkalosis
-vomiting --> loss of gastric HCl --> initiation of metabolic alkalosis, hypochloremia
-vomiting --> volume depletion of loss of NaCl and H2O --> Renin-angiotensin system is activated --> angiotensin II (increases HCO3 reabsorption) --> aldosterone --> 1) Na+ reabsorption, 2) K+ secretion and 3) H+ secretion
6
What to watch out for in an infant with meningococcemia?
Waterhouse-Friderichsen syndrome: sudden vasomotor collapse and skin rash due to adrenal hemorrhage. Mortality is high
7
Suspect henoch schonlein purpura when child has palpable purpura on the lower extremities, arthralgias, abdominal pain, and renal disease. What will immunofluorescence show?
IgA deposition in mesangium
Treat supportively with hydration and NSAIDs for most; If severe, hospitalize and do systemic steroids
8
Most common causes of viral meningitis in peds
non-polioenteroviruses like echoviruses and coxsackievirus
9
Treatment of acute otitis media
1) amox for 10 days
If not improved, then do augmentin
10
When to suspect acute bacterial rhinosinusitis that requires abx vs viral rhinosinusitis which happens to be the most common predisposing factor?
often viral symptoms occur first and when they become persistent or worsen, then suspect progression to bacterial. Treat with augmentin
11
Unilateral cervical adenitis with rapidly enlarging, fluctuant cervical LNs in children are most commonly due to staph or strep infections. How to treat?
incision and drainage plus abx (clindamycin is a great choice)
12
How to treat non-bullous impetigo (painful non-itchy pusutles and honey-rusted lesions)?
Usu due to staph or strep
Can use topical abx like mupirocin
13
Prematurity and maternal diabetes are the two most impt risk factors for causing respiratory distress syndrome. How does maternal diabetes do that?
Maternal hyperglycemia --> fetal hyperglycemia --> triggers hyperinsulinemia --> high levels of insulin antagonizes cortisol that is impt in maturation of sphingomyelin
so low cortisol bc blocked by high insulin leads to low sphingomyelin --> RDS due to surfactant deficiency
14
What are steps involved in you suspect Wilms tumor?
1) US of abdomen
2) CT of abdomen w/ contrast to evaluate nature and extent of the mass and of chest to identify any pulmonary metastases as this is the most common site
3) treatment includes surgery, chemo, with radiation if high-stage
15
A child with macrocytic anemia, low retic count and congenital anomalies (short stature, webbed neck, cleft lip, shielded chest, triphalganeal thumbs)-suspect? Over 90% diagnosed within first year of life.
Diamond-Blackfan Syndrome aka congenital hypoplastic anemia
16
Fanconi's anemia is AR d/o characterized by progressive pancytopenia and macrocytic aenmia. Avg age of diagnsois is 8 yr old. What are some assoc deformities?
-cafe au lait spots
-microcephaly
-short stature
-horseshoe kidneys
-absent thumbs
17
Iron poisoning when ingested in large amts can be corrosive to the GI mucosa. Symptoms can occur within 30 minutes to 4 days including abdominal pain, vomiting (hematemesis), diarrhea (melena), hypotensive shock. It can present as HAGMA. What will you see on x-ray?
X-ray will show radioopaque pills in GI tract
Can treat with whole bowel irrigation, deferoxime and supportive care
May get upper endoscopy to assess complications like gastric scarring and pyloric stenosis from the toxicity
18
ALL is the most common leukemia in children. Can present with petechiae, weight loss, fever, bone pain, anemia, thrombocytopenia, either leukopenia or leukocytosis. How to diagnose?
BM biopsy with at least 20% lymphoblasts (PAS+, terminal deoxynucleotidyltransferase (TdT) +
TdT is only expressed in pre B and T lymphoblasts
19
A child with recurrent sinopulmonary infections develops Giardia. What underlying immunodeficiency explains all this
lack of B cells b/c no IgA which is what is needed to fight the Giardia infection. The recurrent sinopulmonary infections are from H. influenzae and S. pneumoniae
20
Patients who are more prone to abscesses 2/2 to catalase producing organisms like staph, serratia, burkholderia. and aspergillus. Treatment?
Chronic granulomatous disease due to defect in NADPH oxidase system
Treatment includes prevention of infection with daily TMP-SMX and gamma-interferon 3x/week
21
T/F: All children age
True
22
Infant with microcolon and does not pass meconium within first 48 hrs of life... suspect
meconium ileus assoc with CF
microcolon b/c obstruction is at the ileum so the large bowel has not been used --> small in size
no squirt sign (rectal exam doesn't cause passage of stool unlike in hirschsprung when it does)
23
Refeeding syndrome is a potentially fatal complication of nutritional rehabilitation in anorexia nervosa. Why?
Carb intake stimulates INSULIN activity, which promotes cellular intake of phosphorous, potassium and magnesium and thiamine.
Clinical manifestations: arrhythmia, CHF, seizures, wernicke
24
Triple bubble sign on X-ray with bilious vomiting
jejunal atresia (likely due to a vasoconstrictive process, mom could have taken cocaine during pregnancy)
25
What's the most common cause of bacterial pneumonia in children with CF? Make sure to give what types of abx
Staph aureus
start on iv vancomycin
*pseudomona is more common in adult patients with CF
26
Hereditary spherocytosis is AD disorder -a defect in RBC membrane leading to hemolytic anemia (increased UCB), jaundice and splenomegaly. How to test for it? What are some common complications.
-spherocytes on peripheral smear
-increase osmotic fragility on acidified glycerol lysis test
-abnormal eosin-5-maleimide binding test
complications: pigment gallstones and aplastic crises from parvovirus B19 infection
27
Neonatal toxicum neonatorum is a benign neonatal rash characterized by blanching erythematous papules and or pustules. How long do they take to go away? Any treatment?
Just reassurance; should resolve spontaneously within 2 weeks after birth
28
both non-immune but exposed to VZV, how does treatment differ for someone who is immunocompetent vs immunocompromised
immunocompetent: can get vaccine within 3-5 d of exposure
immunocompromised: get VZIG
29
Congenital cyanotic heart lesion
X-ray: "egg on a string" heart (narrow mediastinum)
Single S2 on heart exam (maybe VSD murmur as well)
transposition of the great vessels
30
Congenital cyanotic heart lesion
Boot-shaped heart on X-ray
Harsh pulmonic stenosis murmur, VSD murmur
Tetralogy of Fallot
31
Congenital cyanotic heart lesion
Minimal pulmonary blood flow on x-ray
single S2, VSD murmur
Tricuspid atresia
32
Congenital cyanotic heart lesion
Xray showing "snowman sign", pulmonary edema, enlarged supracardiac veins and SVC
Total anomalous pulmonary venous return with obstruction
33
Osgood schlatter disease aka
traction of the apophysitis of the tibial tubercle
34
Relationship btw constipation and recurrent cystitis
chronic constipation is a risk factor for recurrent cystitis in toddlers b/c impacted stool can cause rectal distension which in turn compresses the bladder, prevents complete voiding and leads to urinary stasis.
35
Viruses (adeno, coxsackie) are the most common cause of pediatric myocarditis, which presents with fever, lethargy, and signs of heart failure after a viral prodrome. Pt should be monitored in ICU for risk of decompensation and fatal arryhtmias. What will CXR, ECHO, EKG, show? What's the gold std for diagnosis?
CXR: cardiomegaly, pulmonary edema
EKG: sinus tachy
ECHO: decreased EF, diffuse hypokinesis
Endomyocardial biopsy is gold std which will show inflammatory infiltrate of the myocardum with myocyte necrosis
Treatment: supportive with diuretics and inotropes
36
How come LAD is never normal on EKG of newborns?
b/c in newborns, the Right heart is bigger b/c in the fetus, blood is shunted away from the lungs by the PDA and the systemic circulation relies primarily on the right heart. So infants are born with bigger r heart. So EKG will show RAD
37
strawberry hemangiomas aka superficial infantile hemangiomas are?
most benign vascular tumor in children that blanch with pressure that grows and then spontaneously regresses
38
someone with subcutaneous emphysema palpated on anterior chest should get what next
chest x ray to rule out pneumothorax
39
Clinical signs of Pineal gland mass
-parinaud syndrome: limited upward gaze, ptosis, upper eyelid retraction, pupillary abnormalities
-obstructive hydrocephalus: papilledema, headache, vomiting, ataxia
-central precocious puberty
workup includes MRI of brain and serum and CSF a-fetoprotein and b-hcG
40
Kallman syndrome is an X linked recessive disease where theres a problem with migration of fetal GnRH and olfactory neurons --> hypogonadotropin hypogonadism. HOw do affected boys and girls look?
affected boys and girls have normal GENOTYPE
But both with short stature and delayed or absent puberty
most distinguishing feature is anosmia/hyposmia
41
Prenatal exposure to phenytoin can lead to fetal hydantoin syndrome: symptoms include
nail and digit hypoplasia
dysmorphic facies
42
The edema in Turner syndrome is most likely due to
congenital lymphedema from abnormal development of of the lymphatic network. The dysfunctional lymphatic system causes accumulation of protein-rich intersitital fluid in the hands, feet and neck (webbed- cystic hygroma)
43
Chediak higashi syndrome is characterized by decreased degranulation, chemotaxis and granulopoiesis. leading to mild coagulopathy, peripheral and cranial neuropathy, HSM, pancytopenia, partial oculocutaneous albinism, frequent bacterial infections. How to confirm diagnosis and treat?
-neutropenia + giant lysosomes in neutrophils confirm the dx
-treat with daily TMP-SMX, daily ascorbic acid
44
Pericardial effusion can occur within days or months after cardiac surgery and is referred to as ____ syndrome?
postpericardiotomy syndrome
life threatening fluid accumulation is characterized by distant heart sounds, hypotension, distended jugular veins and requires drainage
45
Ewing's sarcoma is highly malignant tumor that is found in the LE more than the UE. Most common sites are metaphysis and diaphysis of the femur. Pain and swelling for weeks or months with erythema and warmth. X-ray shows?
lamellated appearance or "onion" skin" periosteal reaction which is then followed with a "moth eaten" or mottled appearance
46
Paroxysmal nocturnal hemoglobinuria has to do with a defect in RBC anchoring protein that prevents complement from attacking self presents as hemolytic anemia, cytopenias, hypercoagulability. How to diagnose
erythrocyte CD55and CD59 testing
47
Arching of the back during or after feeding is a sign of GERD. what is this called
sandifer syndrome
48
What test to order if child has a breath holding spell either cyanotic (crying followed by breath holding in forced expiration) or pallid (minor trauma followed by loss of consciousness? Why
CBC b/c iron def anemia is often assoc with breath holding
49
Macrolides are the abx of choice for pertussis. Who should get macrolides if living with someone with pertussis
everyone should be given macrolide abx regardless of age, immunization or symptoms as post-exposure ppx
50
Some clinical findings in an infant with vit D deficiency
-craniotabes ("ping pong ball" skull")
-delayed fontanel closure
-enlarged skull (frontal bossing), costochondrol joints ("rachitic rosary"), long-bone joints (wrist widening)
-genu varum
51
Suspect what in children who present with fever, dysphagia, inability to extend the neck, muffled voice and lateral x ray showing a widened prevertebral space?
Retropharyngeal abscess
Not epiglottitis caused by h influenzae bc that on x ray has a thumbprint sign
52
What happens when an infant needs emergent resuscitation but IV access cannot be obtained?
Intraosseus access should be attempted immediately. Intraosseus access requires less skill and practice than central line and clinical trials have shown that intraosseus lines are safer and faster than central lines. Most common site is proximal tibia
53
Friedrich ataxia is the most common type of spinocerebellar ataxias with neurologic (ataxia, dysarthria), skeletal (scoliosis, feet deformities), and cardiac symptoms. It is AR d/o and symp begin before 22 y.o. Most common cause of death is
cardiomyopathy
54
Acute setting treatment of stroke in someone with sickle cell
exchange transfusion to decrease the percentage of sickle cells and prevent a second infarct. Does nothing to help with current stroke but prevents likelihood of second one in acute setting
55
Biliary cysts aka choledochal cysts are congenital dilation of the biliary tree. The most common type of biliary cyst is a single, extrahepatic syst. What are classic signs? How to diagnose? Treatment?
Classic signs: pain, jaundice, palpable mass presenting before 10 y.o
Dx: US or ERCP
Treatment: surgical resection to relieve obstruction and prevent malignant transformation
56
A soft click, leg-length discrepancy or asymmetric inguinal skin folds requires what type of diagnostic imaging to assess for congenital hip dysplasia aka developmental dysplasia of the hips due to dislocation of femoral head from acetabulum
if - 6 months --> ultrasound
if >/- 4-6months --> x-ray
treatment of choice for age
57
How to differentiate btw herpangina caused by coxsackie virus vs herpetic gingivostomatits caused by herpes simplex virus type 1 if both can present with fever, pharyngitis, and oral lesions.
unlike HSV gingiviostomatitis, herpangina has a seasonal pattern more commonduring summer/early fall
location! Herpangina typically presents 1mm gray vesicles on the tonsillar pillars and psoterior oropharynx that progress to fibrin-coated ulcerations vs vesciles of HSV usu localizing to the anterior oropharynx and lips
58
How to prevent pneumococcal sepsis in pts with sickle cell anemia?
b/c they become asplenic, they are more prone to infections by encapsulated organisms. Pneumococcal vaccination with the conjugate capsular polysarcch vaccine and penicillin ppx can prevent almost all cases of pneumococcal sepsis.
59
McCune-Albright syndrome is rare. What are the 3 Ps associated with this condition
precocious puberty
pigmentation (cafe au lait spots)
polyostotic fibrous dysplasia (multiple bone defects)
60
Atlantoaxial instability is a malformation seen in 10-15% of patients with DS that should be suspectedin DS patients coming with UMN signs. Most commonly occurs due to? Symptoms?
most commonly due to excessive laxity in the posterior transverse ligament which causes increased mobility btw the atlas (C1) and axis (C2)
Symptoms: behavioral changes, torticollis, urinary incontience, vertebrobasilar symptoms such as dizziness, vertigo and diplopia, UMNsigns
61
Acute u/l cervical lymphadenitis in children is usu caused by?
bacterial infection -staph aureus
62
Beckwidth-Wiedemann syndrome is characterized by macrosomia, macroglossia, umbilical hernia/omphalocele, hemihyperplasia. Children should be monitored for
US for wilms tumor or hepatoblastoma
screening abd US should occur every 3 months from 4-8 y.o and then renal US from 8 thru adolescence
63
Abnormal uterine bleeding in adolescents is most likely due to ovulation dysfunction and requires clinical intervention, most commonly due to immature hypothalamic-pituitary-ovarian axis --> anovulatory cycles. Without ovulation, there's persistent endometrial proliferation followed by heavy menses when ovulation does occur. What's the work-up?
1st line: hormone medication like estrogen or progesterone or mixed estrogen-progesterone as treatment for abnormal uterine bleeding
64
HOw does squatting help with tet spells?
increases systemic vascular resistance, increases pulmonary blood flow so more blood can go thru the stenotic pulmonic valve to the lungs for oxygenation
65
Laryngomalacia is the most common cause of inspiratory stridor that worsens when supine, crying or feeding. It improves in prone position and begins in the neonatal period, peaks at age 4-8 months,resolves by age 12-18 months. What's the diagnostic tool?
laryngoscopy which will show collapse of supraglottic structures with inspiration and omega-shaped epiglottis
66
Stress test to confirm CF is done how?
quantitative pilocarpine inotophoresis.
pilocarpine is a cholinergic drug that is applied to the pt's extremity to induce sweating. A Cl level of >/- 60 mmol/L on 2 occasions confirms diagnosis
67
How to test for primary ciliary dyskinesia
exhaled nasal nitric oxide
68
Capillary lead level is good for iniital lead screening, but what should be done after?
cap lead can have false positives
impt to follow up with venous lead
69
Sigmoid volvulus presents as abd pain,distension, and constipation due to torsion of sigmoid colon. What do you see on xray? (very rare in children; more common in adults)
inverted U-shaped appearance of the distended sigmoid loop ("coffee bean sign")
70
Vascular ring presents before 1 yr of age. It is assoc with cardiac abnormalities and improves with
neck extension
71
What's the preferred modality for long-term evaluation for renal scarring?
Renal scintigraphy with dimercaptosuccinic acid
72
Define "aplastic crisis" assoc with sickle cell disease
"aplastic crisis" is not the same as aplastic anemia
aplastic crisis is a transient arrest of erythropoiesis that results in a severe drop in Hb and virtual absence of reticulocytes on peripheral smear
treat with blood tx
73
Treatment of long QT syndrome
beta blockers and/or pacemaker placement
74
To diagnose CGD, nitroblue tetrazolium test is negative. What's a more sensitive test that can quantify severity of illness
dihydrorhodamine 123 test
75
Suspect fibromuscular dysplasia as a cause of secondary HTN in children. Right renal is more affected than left. PE reveals a hum or bruit in costovertebral angle. What does angiography show?
"string of beads" pattern to the renal artery
76
Spondylolisthesis is a developmental disorder characterized by
forward slip of vertebrae (usu L5 over S1) that usu manifests in preadolescent children.
Clinical: back pain, neuro dysfunction, palpable step-off at the lumbosacral area are present if the disease is severe
77
Precocious puberty has both central and peripheral causes. Central precocious puberty presents with increased FSH and LH while peripheral causes have low FSH and LH. How to manage?
all pts with central precocious puberty should receive brain imaging with CT or MRI. Treatment is with GnRH analog therapy.
78
What's the gold std for diagnosing malrotation?
upper GI contrast study
79
after behavioral therapy fails to correct enuresis, whats the first line med?
desmopressin is ADH analog
if that doesn't work, can try TCAs
80
Biliary atresia can occur in newborns that are initially well-appearing and then over 1-8 wks, develop jaundice, pale stools or dark urine, hepatomegaly, conjugated hyperbilirubinemia, mild elevations in transaminases. What's the initial eval? What's the gold std? What's treatment?
Initial eval: abdominal ultrasound which can show absent or abnormal gallbladder
a hepatobiliary scintigraphy can be done which can show failure of tracer excretion
gold standard is intraoperative cholangiogram showing the actual obstruction
treatment is hepatoportoenterosomy (kasai procedure) or liver transplant
81
SGA infants have complications like hypoxia, polycythemia, hypoglycemia, hypothermia, and hypocalcemia. How come small for gestational age infants (weight under 10th percentile) have polycythemia? Why do they have hypoglycemia? Why hypothermia? Why hypocalcemia?
hypoxia causing EPO secretion causing polycythemia (increased risk of renal vein thrombosis)
hypoglycemia due to low glycogen stores
hypothermic bc low fat stores
hypocalcemia bc of decreased transfer of calcium
82
Classic triad for which TORCH infection is this: leukocoria (white pupillary reflex) from cataracts, murmur of PDA, and hearing loss
congenital rubella
83
Contraindications to rotavirus vaccine
-anaphylasix to vaccine ingredients
-history of intussusception
-history of uncorrected congenital malformation of GI (meckel's diverticulum)
-SCID
84
Intussusception: most common causes before age 2 serving as lead point is? After, age 2, suspect what?
before age 2: most cases of intussusception follows a viral illness that causes hypertrophy of peyers patches which act as the nidus, but often times, no lead point is identified
after age 2: recurrent intussuscept --> suspect meckels diverticulum
85
3-4 month old with hypoglycemia, lactic acidosis, hyperuricemia, hyperlipidemia with a doll-like face, thin extremities, short stature and a protuberant belly from enlarged liver and kidneys with normal spleen and heart.
Von gierke disease with glucose 6 phosphatase deficiency. Hypoglycemic seizures can occur
86
Hepatomegaly, first few weeks of life with floppy baby with feeding difficulties, macroglossia, and heart failure
Pompes disease aka acid Maltase deficiency
87
Mesenteric adenitis
Abdominal pain of right lower quadrant, abdominal guarding, tenderness. Can be mistaken for appendicitis. Do CT
88
Hyper IgM syndrome where's the defect
x linked genetic defect in CD 40 ligand on T cells that need to bind CD 40 on bcells for class switching
89
Infant botulism vs food borne
Infant botulism: from ingestion of c botulinum SPOREs from environment; descending paralysis, treat with human derived botulism immune globulin
Food borne botulism: ingestion of c botulinum toxin (honey); descending paralysis with prodrome of vomiting, nausea, diarrhea. Treat with equine-derived botulism antitoxin
90
Cyanotic infant with left axis deviation and small or absent R waves in pre cordial leads
Tricuspid atresia --> hypo plastic right ventricle so underdeveloped pulmonary artery --> decreased pulmonary markings on chest x Ray. ASD is present for survival --> right atrial hyper trophy peaked P waves
VSD is present for survival
91
To treat ITP, signs of bleeding or platelet count below 30,000 with?
IVIG or glucocorticoid
92
Hereditary fructose intolerance
Aldolase deficiency when introduction of fruits and vegetables into the diet causes accumulation of fructose 1 phosphate --> vomiting, poor feeding, lethargy, seizures and encephalopathy
93