newman- all the SBL pediatric clin

Question 1

define lymphadenopathy

Answer 1

LNs that are abnormal in size, number, or consistency

Question 2

the most important part of the work up in a child w lymphadenopathy

Answer 2

H&PE

Question 3

quality of LNs: what is good, what is more concerning

Answer 3

good: they’re where you expect, soft, mobile, NOT warm, tender, or red: pt feels okay

more concerning: multiple locations, very large, matted and stuck down, fluctuant, tender, associated w big liver/spleen

Question 4

when do you biopsy an unusual LN: if watchful waiting is the plan, what do you have to make sure to do

Answer 4

biopsy if the nodes get bigger, more developed, or don’t go away after 4-6 weeks of watching and waiting

if watching and waiting, ALWAYS lock your patient into follow up with instructions to return sooner if things get worse

Question 5

what do you have to make sure to do when ordering labs on a patient who’s systemically ill/has respiratory sx, or B-signs

Answer 5

actively look for the results

communicate with the patient and their family as soon as the results are available

Question 6

what is the best test for enlarged/unusual LN

Answer 6

excisional biospy

Question 7

common viral causes of lymhadenopathy in children

Answer 7

EBV –> mono (severe pharyngitis, cervical LAD, splenomegaly)

HIV– wasting away, diffused LAD

Question 8

common bacterial causes of lymhadenopathy in children

Answer 8

cat scratch ds: bartonella henselae (ipsi axillary LAD)

tularemia (skinning rabbits)

staph/strep: super common: look for lesion on skin in region of body that drains into the enlarged node

Question 9

most common tumor of infancy

Answer 9

hemangioma (tumor)

Question 10

hemangioma:
clinical manifestations

what finding requires specific investigation

Answer 10

cavernous (big, dilated, deformed vessels)
skin of infants, usually raised and usually get a little bigger before regressing

ANY growth or deep dimple over a baby’s lumbosacral area –> MRI or US to check for spinal cord or vertebral abn

Question 11

what are the malignant tumors of childhoods

how do they present

Answer 11

NEUROBLASTOMA: of sympa ganglia and adrenal medulla
elevated urine VMA and HVA
if cut: blueberry muffin baby

WILMS TUMOR: most common primary renal tumor of kids, peak 2-5 yo

Question 12

what syndrome has an increased risk of wilms tumor

Answer 12

beckwith-weidmann syndrome

=macroglossia, organ enlargement, hemi-hypertrophy, omphalocele

Question 13

most common malignancy in kids <10 yo

Answer 13

leukemia (AML or ALL)

Question 14

most common malignancy in adolescents 15-19 yo

Answer 14

hodgkin lymphoma

EBV, Reed Sternberg Cells, B sx: fever, weight loss, night sweats

Question 15

level of thrombocytopenia

~primary hemostasis impaired
~spontaneous bleeding
~clin sign bleeding
~life threatening bleeding

Answer 15

~ <75k
~< 50k
~ <20 k
~ <10 k

Question 16

causes of thrombocytopenia in..

fetal
early onset neonatal (<72 hrs)
late onset neonatal (>72 hrs)

Answer 16

fetal = alloimmune, autoimmune, chr abn, congenital infection

early onset neonatal (<72 hrs): placental abn, perinatal asphyxia or infection, DIC, alloimmune/autoimmune

late onset neonatal (>72 hrs) = late onset sepsis

Question 17

HUS present in kids

Answer 17

present after acute gastroenteritis w classic triad:

1. microangiopathic hemolytic anemia
2. thrombocytopenia
3. acute renal damage/failure

Question 18

young M w eczema and recurrent infection and small platelets

dx?

Answer 18

wiscott-aldrich syndrome (aka x-linked thrombocytopenia)

= x-lined caused for thrombocytopenia and immune deficiency

Question 19

ITP in kids
ACUTE vs CHRONIC

labs

Answer 19

peak age: (A) 2-6 yrs (C) 20-40yo
sex: (C) F
antecedent infection: (A) common, 1-3 wks
onset of bleeding: (A) abrupt (C) insidious
plt count: (A) <20k (C) 30-80k
eosinophilia/lymph: (A) y (C) rare
duration: (A) 2-6 wks (C) months-yrs
spont remission: (A) ~80% (C) rare

(ACUTE): suddent petechiate and bruising, 30% mucosal hemorrhage.
have anti-plt Ab, n PT/PTT,

(CHRONIC) at 12 months, eval for other ds too (SLE, HIV, H pylori, Xlinked TP)

LABS:

CBC- isolated thrombocytopenia
WBC, reticulotyte, RBC indices, peripheral blood smear, quantitative Igs= normal
DAT= (-)

Question 20

usual trx from ITP

trx in severe/life threatening hemorrhage w ITP

Answer 20

usually= supportive, keep plts >20k

severe: IVIG, prednisone, anti-D Ig

Question 21

kasabach-merrit syndrome = who he?

Answer 21

thrombocytopenia and hypo-fibrinogemia secondary to giant hemangioma and associated intravascular coagulation

Question 22

congenital/hereditary ds that cause thrombocytopenia in children VIA impaired thrombopoiesis

Answer 22

TAR= thrombocytopenia with absent radii syndrome

fanconi syndrome (macrocyti anemia, reticulocyto+thrombocyto+leukocyto -penia) ~ progress to pancytopenia, may not see sx until 10yo

Schwachman-Diamond syndrome= congenital pure red cell aplasia

Wiskott Aldrich

Question 23

physiologic response to anemia

Answer 23

concentration (2,3-DPG) increases w/i the RBC

O2 dissociation curve shifts to the right

transfer of oxygen to the tissues is more complete

Question 24

clinical features of anemia present once Hgb falls below what??

sx=

Answer 24

Hgb < 7-8 g/dL

pallor, sleepiness, flow murmur, SOB exertion, high output HF

Question 25

the ___ anemia develops, the better the body can compensate

Answer 25

slower

Question 26

lab tests to order in an anemic pt

Answer 26

H/H = Hgb and Hct

CBC and RBC indicies==> 
MCV=mean V of individual RBC
MCH: avg mass of Hgb per RBC
MCHC= grams of Hgb per 100mLs RBC
RDW= variability in RBC sizes in sample

peripheral smear= size, shape, presence of fragments

Question 27

microcytic vs macrocytic RBCs

hypochromic vs hyperchromic RBCs

Answer 27

MCV <2.5th percentile= microcytic

MCV>97.5th percentile = macrocytic

MCHC< 32g/dL= hypochromic
MCHC>35g/dL= hyperchromic

Question 28

in anemic patients, a low/low-normal number of reticulocytes suggests what

Answer 28

inadequate BM response, ineffective erythropoeisis

Question 29

the normal response to anemia is ___ reticulocytes

Answer 29

increased

Question 30

what are the normal changes in Hgb and Hct in the first year of life

Answer 30

born w v high Hgb and Hct

@ 2 months, they both drop down to normal (Hct slightly undershoot, increase a lil at 4 months)
will normalize over the rest of the first year

final: Hct ~38%, Hgb~12

Question 31

age specific causes of anemia

neonatal

Answer 31

blood loss
immune hemolytic anemia (ABO, Rh), congenital hemolytic anemia
congenital infection with parvovirus B19, HIV, syphilis, rubella, sepsis, (normocytic, low reticulocyte)

diamond blackfan sybdrome= MACRocytic anemia, low reticulocyte count= congenital pure red cell aplasia

fanconi syndrome (macrocyti anemia, reticulocyto+thrombocyto+leukocyto -penia) ~ progress to pancytopenia, may not see sx until 10yo

Question 32

age specific causes of anemia

infancy to toddlerhood

risk factor

Answer 32

iron deficiency anemia (microcytic, hypochromic anemia, target cells)
chronic infection
blood loss
thalassemia (basophilic stippling),
sickle cell ds
leukemia, myelofibrosis
Pb poisening (microcytic hypochromic, basophilic stippling) RISK = young, house built before 1970, areas w contaminated soil, pica, Pb level >5

Question 33

age specific causes of anemia

late childhood and adolescence

Answer 33

iron deficiency
chronic ds
blood loss
ds of Hgb synthesis or RBC membrane defects
acquired hemolytic anemia
leukemia, other BM ds