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Pediatrics > Emma Holiday Review II > Flashcards

Emma Holiday Review II Flashcards

1
Q

7 yo M w/ recurrent bruising, hematuria, and hemarthroses.

-prolonged PTT corrected w/ mixing studies

A

= Hemophilia = congenital clotting factor deficiency

-hemarthroses = characteristic of hemophilia, bleeding into joint space

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2
Q

Heart defect associated w/ DiGeorge Syndrome

A

Truncus arteriosis = single vessel coming out of both RV and LV instead of separate aorta and pulmonary artery

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3
Q

MR yet friendly and loquacious

A

Williams
= microdeletion of chr7

  • heart defects: supravalvular aortic stenosis
  • echolalia
  • developmental delay
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4
Q

Differentiate Blackfan-Diamond and Fanconi Anemia

A

Blackfan-Diamond = abnormal red count w/ normal white and platelet count

Fanconi Anemia = see pancytopenia (white and platelet counts are also low)

-both are genetic causes of anemia

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5
Q

4 yo w/ jerky mov’ts of eyes and legs, bluish skin nodules, and tender abdominal mass

A

Neuroblastoma

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6
Q

Hematuria + Deafness

A

Hematuria + Deafness- think Alport Syndrome

-X linked recessive mutation to collagen IV

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7
Q
  • severe eczema
  • petechiae
  • recurrent ear infxn
A

= Wisckott-Aldrich syndrome = X-linked d/o

  • immune deficiency
  • eczema
  • thrombocytopenia => petechiae, bloody diarrhea
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8
Q

What is the most common B-cell defect?

(a) Complication

A

Selective IgA deficiency

(a) Anaphylaxis if given blood containing IgA

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9
Q

2 yo w/ hypertension and asymptomatic abdominal mass

A

Wilm’s tumor

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10
Q

DiGeorge Triad

A

DiGeorge Syndrome

  • conotruncal cardiac anomalies
  • hypoplastic thymus => immune deficiency
  • hypocalcemia

Common to also have

  • palatal abnormalities
  • developmental delay
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11
Q

Which clotting factors may a CF pt be deficiency in?

A

CF pts often have malabsorption of fat-soluble vitamins => vitamin K deficiency => deficient in factors II, VII, IX, X (1972)

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12
Q

Describe the murmur seen in HOCM

Reaction to the following maneuvers

(a) squat
(b) valsalva
(c) exercise
(d) hand grip

A

HOCM = systolic ejection murmur

(a) better w/ increased preload => improves w/ squat or handgrip
(b) louder w/ decreased preload => louder w/ valsalva, standing, exercise

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13
Q

Describe a Still’s murmur

A

Systolic vibratory murmur

  • below II/VI
  • soft
  • heart best at lower mid-sternum
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14
Q

7 yo girl w/ non-productive cough and large anterior mediastinal mass on CXR

A

Non-Hodkin Lymphoma

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15
Q

14 yo boy w/ enlarged, painless, rubbery nodes

  • drenching fevers
  • 10% weght loss
A

Hodgkin Lymphoma

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16
Q

15 yo F w/ recurrent epistaxis, heavy menses, and petechiae

  • normal platelet count
  • increased bleeding time and PTT
A

= Von Willebrand disease = hereditary coagulation abnormality
-deficient vWF = factor needed for platelet adhesion

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17
Q

Hematuria + hemoptysis

A

Hematuria + hemoptysis- think Goodpasture’s syndrome

-antibodies

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18
Q

Tx of child w/ HOCM

A
  • Beta blockers or CCB
  • ablation or surgical myotomy
  • no sports or heavy exercise
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19
Q

W/ a simple test how can you distinguish Wilm’s tumor and neuroblastoma

A

Urine analysis
-increased HV (homovanillic) or VMA (vanillylmandelic acid) (catecholamine metabolites) in urine of Neuroblastoma
-

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20
Q

14 yo girl w/ no breast development, short stature, high FSH

A

Turner’s syndrome (XO)

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21
Q

Diagnostic criteria for diabetes

(a) fasting glucose
(b) 2 hr OGTT
(c) Any glucose

A

Diabetes criteria

(a) fasting glucose > 125 twice
(b) 2hr OGTT > 200
(c) Any glucose > 200 + symptoms

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22
Q

Meds to avoid in children w/ Wisckott-Aldrich syndrome

A

Wischkott-Aldrich characterized by thrombocytopenia => problems clotting => avoid NSAIDs/aspirin (interfere w/ paltelet fxn)

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23
Q

SCD treatment

(a) Stimulate HbF production
(b) Supportive
(c) Possible cure
(d) Prophylaxis

A

SCD treatment

(a) Stimulate HbF production w/ hydroxyurea
(b) Aggressively treat infections, manage pain appropriately
(c) Bone marrow transplant cures, but has 10% post op mortality
(d) Prophylactic penicillin to SCD pts 2-6 yoa to prevent infxn w/ encapsulated organisms

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24
Q

Cafe-au-lait spots
Macrocephaly
Seizures

A

Neurofibromatosis

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25
Q

What additional med to give to kids who get frequent blood transfusions

A

Deferoxamine = iron chelating agent

-remove excess iron from the body

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26
Q

Anomalies associated w/ Turner’s syndrome

(a) Renal
(b) Vascular
(c) Cardiac

A

Anomalies associated w/ Turner’s syndrome

(a) Horseshoe kidney
(b) Coarctation of the aortave
(c) Bicuspid aortic valve

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27
Q

-Broad, square face
-Short stature
-Self injurious behavior
Deletion on Chr 17

A

= Smith Magenis syndrome = complex genetic developmental d/o characterized by intellectual disability + distinct facial features

-due to microdeletion on chromosome 17

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28
Q

Associated murmur of

(a) Transposition of the Great Arteries
(b) Tetralogy of Fallot
(c) Ebstein anomaly
(d) VSD
(e) Endocardial cushion defect
(f) PDA

A

Murmurs

(a) TGA- none! (unless concurrent PDA or VSD)
(b) Tetralogy of Fallot- harsh SEM and single S2
(c) Ebstein anomaly- holosystolic murmur worse on inspiration
(d) VSD- harsh holosystolic murmur over LL sternal border, loud P2
(e) Endocardial cushion defect- fixed and split S2 + SEM w/ diastolic rumble
(f) PDA- continuous, machine-like murmur w/ bounding pulses (continuous flow murmur)

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29
Q

What is the most common microdeletion in humans?

A

DiGeorge syndrome

-microdeletion of chr22

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30
Q

Management if II/VI VSD detected in a 2 mo old

A

If not symptomatic (no FTT, no pHTN), watch and wait

-most close by 1-2 yr

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31
Q

Tetrad of Tetralogy of Fallot

A
  1. VSD
  2. RVH
  3. Overriding aorta: aorta connected to both LV and RV
    - degree of override = degree to which aorta is attached to RV
  4. Pulmonary stenosis: narrowing of RVOT
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32
Q

Infant w/ severe infxns, no thymus or tonsils, severe lymphophenia

A

SCID

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33
Q

VSD: is louder murmur better or worse

A

Louder murmur is better- means the defect is small and probably membraneous => more likely to spontaneously close

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34
Q

Tx for Turner’s syndrome

A

Estrogen replacement for secondary sex characteristics and to avoid osteoporosis

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35
Q

Endocardial cushion defect

(a) Why is surgery urgent
(b) Describe the murmur/heart sound pattern

A

Endocardial cushion defect

(a) Surgery before pulmonary HTN develops at 6-12 mo
(b) Fixed and split S2 + SEM w/ diastolic rumble

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36
Q

What kind of infections do children w/ DiGeorge syndrome get?

A

Candida, viruses, PCP pneumonia

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37
Q

15 yo F w/ recurrent epistaxis, heavy menses, and petechiae

-low platelets only on CBC

A

= ITP = idiopathic thrombocytopenic purpura = autoimmune destruction of platelets

  • F > M
  • low platelet count w/ normal BM
  • presents w/ purpuritic (non blanching) rash + increased tendency to bleed (epistaxis = nose bleeds)
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38
Q

Most common cause of proteinuria in children

A

Minimal change disease = fusion of the foot processes in the glomerulus

Tx = prednisone for 4-6 weeks

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39
Q

-hypotonia
-hypogonadism
-hyperphagia
-skin picking
-aggression
Deletion on paternal Chr15

A

= Prader-Willi syndrome = complex genetic syndrome

  • unrelenting hunger, never feel full => obesity
  • ID
  • short stature
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40
Q

SCD pt presenting w/ fatigue and megaloblastic anemia

A

Most likely folate deficiency

-SCD pts have higher folate needs due to high retic count

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41
Q

17 yo F w/ decreased IgG, IgM, IgE, and IgG but normal B cell count

A

Combined variable immune deficiency (acquired)

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42
Q

General population risk of Down’s syndrome

A

1 in 600-700 births

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43
Q

Poor prognosis congenital syndrome associated w/ midline defects

A

Midline defects: Trisomy 13

  • omphalocele
  • cleft lip/palate
  • holoprosencephaly
  • eyes far apart
  • heart defects
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44
Q

What are kids w/ Fanconi Anemia at an increased risk for

A

Increased risk for cancers, especially AML

-b/c the defect is a mutation in DNA repair enzymes

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45
Q

3 yo w/ limp and left leg pain

99.9 F, petechiae, pallor

A

= Acute Lymphocytic Leukemia

Dx: bone marrow biopsy

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46
Q

18 yo tall lanky boy w/ mild MR, gynecomastia, hypogonadism

A

Kleinfelter’s syndrome

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47
Q

Complication of combined variable immune deficiency

A

Increased lymphoid tissue => increased risk of lymphoma

48
Q

Most common congenital heart defect in Down Syndrome baby

A

Endocardial cushion defect

49
Q

Prognosis for Trisomy 18 and Trisomy 13

A

Prognosis: very poor

-most dont live past 1 yoa, and if do have severe MR

50
Q

Features of Turner’s

(a) Learning development
(b) Good prognostic indicator

A

Turner’s (XO)

  • hypogonadism => no ovaries
  • web neck
  • small stature
  • shield chest
  • heart defect: coarctation of aorta, bicuspid aortic valve
  • horseshoe kidney

(a) No delay in development, no learning problems
- so gonadal agenesis is similar to Kleinfelter’s, but not the learning difficulties
(b) Mosaicism = good prognostic features
- some may even have ovaries

51
Q

Cyanosis at birth w/ holosystolic murmur

-EKG shows LVH

A

Tricuspid atresia = no connection btwn RA and RV

52
Q

Most common congenital heart lesion

A

VSD

53
Q

Tx for

(a) ITP
(b) VWD

A

Treatments

(a) ITP: IVIG for 1-2 days, then prednisone. Splenectomy if refractory
- thought that spleen is sight of platelet auto-antibody production

(b) VWD: replace factor VIII (contains vWF) if bleeding continues
- DDAVP for bleeding or pre-op

54
Q

Describe behaviors of FAS kids

A

ADHD-like behavior

55
Q

8 mo w/ glossitis and FTT

  • picky eater
  • drinks lots of goat’s milk
A

Folate deficiency

  • glossitis = tongue swelling
  • goats milk lacks folate
56
Q

18 mo w/ lot plateletes, WBCs, and profound anemia

  • cafe au lait spots
  • microcephaly
  • absent thumbs
A

Fanconi anemia = rare genetic d/o, mutation in DNA repair enzyme => pancytopenia (from bone marrow hypoplasia)

57
Q

Bruton agammaglobulinemia

(a) Blood test results
(b) Mode of inheritance
(c) Common presentation
(d) Explain age of presentation

A

Bruton agammaglobulinemia = genetic (X-linked) inability to generate mature B-cells

(a) lack of antibodies in circulation
(b) X linked
(c) Presents w/ recurrent infections
ex: 2 yo M w/ multiple ear infxns, diarrheal episodes, and pneumonias
Especially extracellular, encapsulated bacteria
(d) 6-9 mo (when maternal antibodies are gone)

58
Q

3 yo w/ petechiae, abdominal pain, vomiting and lethargy

  • blood diarrhea 5 days ago
  • Labs: thrombocytopenia and increased creatinine
A

HUS = hemolytic uremic syndrome
-follows infxn (most likely E. Coli O157H7), usually w/ bloody diarrhea

  • hemolytic anemia
  • thrombocytopenia
  • acute renal failure
59
Q

Better prognostic factor for Trisomy 18 and 13

A

Mosaicism: not all cells have the trisomy feature

60
Q

Older child w/ exercise tolerance

Loud S1 w/ fixed and split S2

A

ASD

61
Q

18 mo old not pick eater w/ Italian mom

  • low Hct and Hgb
  • MCV 60

(a) Expected RDW
(b) Tx

A

Thalassemia

(a) Expect low RDS
(b) Tx w/ transfusion and deferoxamine
- deferoxamine = iron chelating agent, binds to free iron in blood stream and enhances it’s excretion

62
Q

One lab test that can distinguish iron vs. folate deficiency anemia

A

MCV

  • folate deficiency causes megaloblastic anemia
  • iron deficiency => microcytic anemia
63
Q

5 yo w/ purpura, abdominal pain, joint pain

-IgA and C3 deposited in skin

A

HSP = Henoch Schonlein Purpura

-would also see current jelly stool

64
Q

What congenital d/o may present w/ joint effusion?

A

Hemophilia
-X-linked recessive

Common presentation = bleeding into a joint

65
Q

Ig makeup of Wisckott-Aldrich syndrome

A
  • low IgM
  • high IgA and IgE
  • slightly low IgG
66
Q

2 yo child who gets cyanotic and hyperpnea while playing, improves when squats down

A

Tetralogy of Fallot = cyanotic and hyperpnea (labored breathing) while playing, improves w/ knee-chest position

  • VSD
  • RA hypertrophy
  • over-riding aorta
  • pulmonary stenosis
67
Q

3 week old M w/ seizure, trucus arteriosus, micrognathia

A

= DiGeorge Syndrome: genetic (deletion)

-heart defects, poor immune fxn, cleft palate, complications of low serum calcium, delayed development

68
Q

-Elfin-appearance
-friendly
-increased empathy and verbal reasoning ability
Del Chr 7

A

= Williams syndrome = neurodevelopmental d/o characterized by unusually cheerful children w/ developmental delay but strong language skills

  • strong association w/ heart defects
  • characteristic facial features: appearance
69
Q

Trisomy 18

-two most specific findings

A

Trisomy 18 almost pathognomonic when find SGA + hand finding

Hand finding = 2nd and 5th fingers over 3rd and 4th

70
Q

Tx for Blackfan-Diamond Anemia

A
  • corticosteroids
  • transfusions
  • stem cell transplant: thought to be defective stem cell early in RBC lineage
71
Q

Presentation of Transposition of the great arteries

(a) Increased risk in mothers w/ what
(b) Immediate tx

A

Cyanotic newborn
-O2 doesn’t improve

(a) Transposition of the great arteries most common in infants of diabetic murmurs
(b) Give PGE1 to keep PDA patent

72
Q

Physical characteristics of Fragile X syndrome

A
  • macrocephaly
  • macroorhidism: unusually large testicles
  • large ears
73
Q

Diagnostic test for ALL

A

Bone marrow biopsy w/ > 30% lymphoblasts

74
Q

Current jelly stool

A

Buzzword for HSP = Henoch Schonlein Purpura

75
Q

2 associations of PDA

A
  • prematurity

- congenital rubella syndrome

76
Q

At what age is Kleinfelter’s usually diagnosed

(a) Diagnostic features
(b) Other associated features

A

Kleinfelter’s (XXY) usually diagnosed at puberty (aka when its delayed)

(a) Diagnostic: features: hypogonadism + small testes
(b) ADHD, learning disability

77
Q

Flank pain radiating to groin + hematuria

A

Flank pain radiating to groin + hematuria- think kidney stones

-CT

78
Q

Serious complication of food poisoning w/ E. Coli O157H7

A

HUS = Hemolytic Uremic Syndrome

  • medical emergency: 5-10% mortality
  • hemolysis following blood diarrhea = give away clinical presentation

Triad of findings

  • microangiophatic hemolytic anemia
  • thrombocytopenia
  • acute renal failure
79
Q

18 mo old picky eater, drinks lots of cow’s milk

  • low Hct and Hgb
  • MCV 75 w/ low ferritin

(a) Expected TIBC
(b) Tx

A

Iron deficiency

(a) Expect high TIBC
- low iron around so lots of binding sits open
(b) Tx = oral ferrous salts

80
Q

Tx for Fanconi Anemia

A
  • corticosteroids
  • androgens
  • bone marrow transplant
81
Q
  • Mandibular hypoplasia = micrognathia/retrognathia
  • Glossoptosis
  • Cleft soft palate
A

Pierre Robin Sequence = developmental sequenced characterized by

-cleft palate
-retrognathia = abnormal jaw/maxilla positioning
micrognathia = unusually small mandible
-glossoptosis = tongue blocking airway

82
Q

What is a craniopharyngioma?

(a) Cell of origin

A

Craniopharyngioma = brain tumor from pituitary gland embryonic tissue

Develops from Rathke’s pouch = embryonic precursor to pituitary gland

83
Q

Tx for SCID

A

Pediatric emergency- need bone marrow by age 1 or death

84
Q

What is the cause of the most common type of MR in boys?

A

Fragile X syndrome

-CGG repeats on the X chrom

85
Q

Signs of lead poisoning

(a) Behavioral
(b) Location of pain
(c) GI complaints
(d) Constitutional
(e) Development

A

Lead poisoning

(a) Hyperactivity or irritability
(b) Pain in abdomen or joints
(c) GI: constipation, nausea, vomiting
(d) Fatigue
(e) Developmental delay, impaired growth

86
Q

SCD pt presents w/ fever, cough, CP, chills, SOB

A

Acute chest syndrome

  • pulmonary infarction
  • most common cause of death
87
Q

HA worse in morning + seizure, ataxia, vomiting

A

Brain tumor

-most likely infratentorial (kids more likely to get infratentorial tumors)

88
Q

Hematuria

(a) 1-2 days after runny nose, sore throat, cough
(b) 1-2 weeks after sore throat or skin infxn

A

Hematuria

(a) 1-2 days after runny nose, sore throat, cough- think Bergers disease (IgA nephropathy)

(b) 1-2 weeks after sore throat or skin infxn- think Post Strep glomerulonephritis
- test = ASO titer

89
Q

Explain the pathophysiology of a SCD kid coming in w/

(a) swollen, painful hands and feet
(b) excruciating pain in extremities, ulcers, hip pain
(c) Acute drop in hematocrit w/ very low retic count
(d) Proteinuria, high creatinine
(e) Point tenderness of femur, fever, and malaise

A

Sickle cel disease

(a) Swollen, painful hands and feet: dactylitis secondary to necrosis of small bones
(b) Excruciating pain: pain crisis, ischemic damage secondary to sickling
(c) Acute drop in Hct w/ low retic: aplastic crisis (parvo B19 virus)
(d) Kidney infarcts due to sickles RBC
(e) Point tenderness: osteomyelitis, most common bug is salmonella

90
Q

Most common heart defect in a Turner’s baby

A

Coarctation of the Aorta

91
Q

Definition of

(a) Nephritic syndrome
(b) Nephrotic

A

Definitions

(a) Nephritic syndrome- proteinuria (but 3.5g/24hr), hypoalbuminemia, edema, hyperlipidemia

92
Q

Describe the facial features of FAS

A

Fetal alcohol syndrome characteristic facies

  • microcephaly
  • smooth philtrum (vertical grove btwn nose and lips)
  • thin upper lip
93
Q

Common medical complications of Downs

(a) Heart
(b) GI
(c) Endocrine
(d) Msk
(e) Neuro
(f) Cancer

A

Medical complications associated w/ Downs syndrome

(a) Endocardial cushion defect, VSD
- 50% of Down’s pts have heart defect
(b) GI: Hirschsprung’s (lack of innervation to intestines), intestinal (specifically duodenal) atresia
(c) Hypothyroidism
(d) MSK: atlanto-axial instability: excessive mov’t btwn atlas (C1) and C2)l
(e) 30-35x increased risk of Alzheimer’s
(f) 10x increased risk of ALL

94
Q

Deletion on

(a) Paternal chr15
(b) Maternal ch15
(c) Ch17
(d) Chr7

A

Deletion on

(a) Paternal chr15 = Prader-Willi
(b) Maternal chr15 = Angelman
(c) Chr17 = Smith Magenis
(d) Chr7 = Williams

95
Q

What is the most common cause of

(a) sepsis in sickle cell pts
(b) death in sickle cell pts

A

SCD

(a) Strep pneumo = most common cause of sepsis
(b) Acute chest syndrome due to pulmonary infarction = most common cause of death

96
Q

Features of Downs syndrome

(a) Eyes
(b) Hands
(c) Tongue
(d) Iris
(e) Mental ability

A

Downs syndrome (trisomy 21)

(a) Slanted/oblique palpebrae fissures
(b) Single palmar crease = simean crease
(c) Large tongue
(d) White spots on iris
(e) MR, developmental delays

97
Q

What are the vitamin-K dependent clotting factors

A

1972

X, IX, VII, II

98
Q

3 organ systems affected in Marfan’s syndrome

A

Marfan’s Syndrome

(1) Cardiac: dilated aortic root in children => aneurysm/dissection in adults

(2) MSK: arachrodactyly- long thin tubular bones w/ prominent joints
- arm span > height

(3) Eyes: dislocated lenses

99
Q

Tetraology of Fallot

(a) What factor dictates degree of symptoms
(b) Mechanism by which knee-chest improves symptoms of hypercyanotic spells
(c) Is O2 therapy helpful?

A

Tetralogy of Fallot

(a) Degree of pulmonary stenosis dictates symptoms- b/c problem is really the lack of pulmonary blood flow
(b) Knee-chest increases SVR which increases blood flow from the RV into pulm circulation
(c) O2 won’t help b/c problem is lack of pulm blood flow, not oxygenation

100
Q

Tx for HUS

A

Treating hemolytic uremic syndrome:
-DONT GIVE PLATELETS

Tx w/ aggressive nutrition (TPN = total parenteral nutrition) + early peritoneal dialysis

101
Q

Complication of acute rheumatic fever

A

-mitral stenosis

102
Q

How to treat a CF pt w/ pneumonia

A

Most often pseudomonas or colonized w/ b. cepacia =>

Tx w/ piperacillin + tobramycin or ceftazidime

103
Q

Most common brain tumor in kids

(a) 2nd most common
(b) Which has a better prognosis

A

Pilocystic astrocytoma of cerebellum

(a) Medulloblastoma
(b) Pilocystic astrocytoma has better prognosis

104
Q

7 yo F w/ vague CP, pain in several joints, and rash

-EKG shows PR interval prolongation

A

Acute Rheumatic fever

105
Q

What are the following features of

(a) rocker-bottom feet
(b) severe MR
(c) microcephaly
(d) Cleft lip/palate
(e) holoprosencephaly
(f) omphalocele
(g) hammer toe

A

Trisomy syndromes: trisomy 13 (Patau’s) vs. trisomy 18 (Edward’s)

Patau’s: severe MR, microcephaly, cleft lip/palate, holoprosencephaly (cyclops)

Edward’s: rocker bottom feet, microcephaly (both), omphalocele, hammer toe

106
Q

4 yo w/ normal platelets and WBCs

  • Hgb 4
  • increased RBC ADA, low retics
  • triphalangeal thumbs
A

= Blackfan-Diamond Anemia = inherited pure red cell aplasia

  • associated w/ elevated ADA = adenosine deaminase activity
  • BM disease of defective RBC production, other cell lines remain normal
107
Q

Function of hydroxyurea in SCD

A

Hydroxyurea stimulants HbF production => diluting the number of sickled RBCs in circulation
-reduces vaso-occlusive episodes

108
Q

Tx for Tetralogy of Fallot

(a) Immediate
(b) Long term

A

Tx for Tetralogy of Fallot

(a) Immediate: O2 and knee-chest position
(b) Surgical correction

109
Q

Bipolar F gives birth to child w/ holosystolic murmur worse on inspiration

A

Ebstein anomaly

  • tricuspid insufficiency
  • TV displacement into RV
110
Q

Kleinfelter’s pts have an increased risk of what cancer?

A

Gonadal malignancy

111
Q

Which neonates should be worked up for DiGeorge syndrome?

A

Any neonate w/ conotruncal heart lesion, hypocalcemia, and/or cleft palate

112
Q

CF

(a) signs at birth
(b) signs during early childhood
(c) location of mutation
(d) diagnostic sweat test value

A

CF

(a) Meconium ileus = pathognomonic
- dilated loops, ‘ground glass’

(b) Early childhood: FTT, foul smelling bulky stools, recurrent respiratory infxns and nasal polyps
(c) Chromosome 7- CFTR protein
(d) > 60 mEq/L chloride is diagnostic

113
Q

Clinical features of selective IgA deficiency

A

Recurrent URIs, diarrhea

114
Q

14 yo- 5th percentile in height

  • bitemporal hemianopsia
  • calcifications in the sella turcica
A

= Craniopharyngioma = brain tumor from Rathke’s pouch (embryonic pituitary gland tissue)

-bitemporal hemianopsia = loss of peripheral vision due to tumor compressing on optic chiasm (where optic nerves cross near the pituitary)

115
Q

-Seizures
-Strabismus
-Sociable w/ episodic laughter
Deletion on maternal Chr15

A

Angelman syndrome = complex genetic syndrome affecting mainly the nervous system, charactrized by frequent smiling and laughter

  • epilepsy, ataxia
  • MR, severe speech impairment
  • strabismus = crossed eye
116
Q

Percent of kids w/ benign murmers

(a) What types of murmurs are never benign

A

30% of kids have benign murmurs
ex: stills murmur

(a) Diastolic, or > II/VI are never normal

117
Q

Tx for PDA

A

If not closed by week 1: give indomethacin or surgically close

Pediatrics (10 decks)

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