newman- all the SBL pediatric clin Flashcards
define lymphadenopathy
LNs that are abnormal in size, number, or consistency
the most important part of the work up in a child w lymphadenopathy
H&PE
quality of LNs: what is good, what is more concerning
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
when do you biopsy an unusual LN: if watchful waiting is the plan, what do you have to make sure to do
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
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
actively look for the results
communicate with the patient and their family as soon as the results are available
what is the best test for enlarged/unusual LN
excisional biospy
common viral causes of lymhadenopathy in children
EBV –> mono (severe pharyngitis, cervical LAD, splenomegaly)
HIV– wasting away, diffused LAD
common bacterial causes of lymhadenopathy in children
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
most common tumor of infancy
hemangioma (tumor)
hemangioma:
clinical manifestations
what finding requires specific investigation
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
what are the malignant tumors of childhoods
how do they present
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
what syndrome has an increased risk of wilms tumor
beckwith-weidmann syndrome
=macroglossia, organ enlargement, hemi-hypertrophy, omphalocele
most common malignancy in kids <10 yo
leukemia (AML or ALL)
most common malignancy in adolescents 15-19 yo
hodgkin lymphoma
EBV, Reed Sternberg Cells, B sx: fever, weight loss, night sweats
level of thrombocytopenia
~primary hemostasis impaired
~spontaneous bleeding
~clin sign bleeding
~life threatening bleeding
~ <75k
~< 50k
~ <20 k
~ <10 k
causes of thrombocytopenia in..
fetal
early onset neonatal (<72 hrs)
late onset neonatal (>72 hrs)
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
HUS present in kids
present after acute gastroenteritis w classic triad:
- microangiopathic hemolytic anemia
- thrombocytopenia
- acute renal damage/failure
young M w eczema and recurrent infection and small platelets
dx?
wiscott-aldrich syndrome (aka x-linked thrombocytopenia)
= x-lined caused for thrombocytopenia and immune deficiency
ITP in kids
ACUTE vs CHRONIC
labs
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= (-)
usual trx from ITP
trx in severe/life threatening hemorrhage w ITP
usually= supportive, keep plts >20k
severe: IVIG, prednisone, anti-D Ig
kasabach-merrit syndrome = who he?
thrombocytopenia and hypo-fibrinogemia secondary to giant hemangioma and associated intravascular coagulation
congenital/hereditary ds that cause thrombocytopenia in children VIA impaired thrombopoiesis
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
physiologic response to anemia
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
clinical features of anemia present once Hgb falls below what??
sx=
Hgb < 7-8 g/dL
pallor, sleepiness, flow murmur, SOB exertion, high output HF
the ___ anemia develops, the better the body can compensate
slower
lab tests to order in an anemic pt
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
microcytic vs macrocytic RBCs
hypochromic vs hyperchromic RBCs
MCV <2.5th percentile= microcytic
MCV>97.5th percentile = macrocytic
MCHC< 32g/dL= hypochromic
MCHC>35g/dL= hyperchromic
in anemic patients, a low/low-normal number of reticulocytes suggests what
inadequate BM response, ineffective erythropoeisis
the normal response to anemia is ___ reticulocytes
increased
what are the normal changes in Hgb and Hct in the first year of life
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
age specific causes of anemia
neonatal
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
age specific causes of anemia
infancy to toddlerhood
risk factor
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
age specific causes of anemia
late childhood and adolescence
iron deficiency chronic ds blood loss ds of Hgb synthesis or RBC membrane defects acquired hemolytic anemia leukemia, other BM ds
