Peds II- Exam 1 Flashcards
Abdominal mass that CROSSES midline
Neuroblastoma
Most common solid tumor of childhood
Brain tumor
ALL/LBL
Acute Lymphoblastic Leukemia
Most common CA of childhood
circulating lymphoblasts
AML
Auer rods with circulating myeloblasts
CML
Philadelphia chromo
rare in childhood
Hodkin lymphoma
painless cervial and supraclavicular lymphadenopathy
Reed sternberg cells
Ewing Sarcoma
2nd most common primary bone tumor
Affecting children and young adults
Nighttime bone pain
Retinoblastoma
Most common intraocular tumor in kids
White pupillary reflex
Rhabdomyosarcoma
Painless, progressive, enlarging mass
Most common nutritional deficiency in kids
Iron deficiency
Most common bleeding disorder of kids
Acute ITP
Idiopathic Thrombocytopenic Purpura
Anemia
2 SD below normal for age and sex
Anemia in age 6 mo- 5 YO
Hgb <11
Normocytic and Normochromic anemia
anemia of chronic dz
Microcytic, Hypochromic Anemias
Iron def
Thalassemia
Lead intox
Macrocytic
B12 and Folate def
Fanconi Anemia
Inherited
Progressive pancytopenia- all cell lines
Skin pigment, short stature, skeletal malform
Inc incidence of CA
often misdx as ITP
Tx for Fanconi
Supportive
Stem Cell transplant
Fanconi prognosis
Succumb to infection, bleeding, or CA in teen or early adult
Pt at high risk of developing MDS or AML
Acquired Aplastic Anemia
any age
Peripheral pancytopenia
Low WBC with marked Neutropenia
Thrombocytopenia
Low reticulocytes
Acquired Aplastic Anemia
Complications: overwhelming infection and severe hemorrhage: leading causes of death
foot picture with lost of bleeding- petechaie, purpura
Tx for Acquired Aplastic Anemia
Supportive, refer, stop offender, Abx, Transfusion, platelet transfusion (sparingly), Immunosuppressive agent, Stem Cell *** tx of choice
Iron Def Anemia
most common nutritional def in kids
Afirican Amer, Hispanic, poor communities
1-2 YO and mothers of childbearing age
Iron Def Anemia
hx of Pica
delayed motor development
fatigue, irritable
Screen for Iron Def Anemia
@ 12 months!!
determine hg concentration and risk factors
Labs for Iron Def Anemia
Microcytic (Small)
Hypochromic
Ferritin <12
Tx for Iron Def Anemia
Hgb 10-11 at 12 month checkup- just watch closely or empirically treat
check again in 1 month
if replace: Iron 6 mg TID
B12
malabsorption!!
Folic acid
increased requirements, malabsorption, dietary (rare), meds
B12
NEURO sx
Megaloblastic sx (B12 and folate)
Pallor
Glossitis
Labs of B12 and Folate
Neutrophils are large and hypersegmented
Macro-ovalcytes; large, oval RBC
B12 only
Neuro and
MMA elevated lab
Homo lab elevated in
both Folic Acid and B12 def
Hereditary Spherocytosis HS
Red cells lyste and burst open
Jaundice, splenomeg, gallstones
Spherocytes o nlab
Increased osmotic fragility
supportive care and maybe transfusion
SPLENECTOMY maybe
Thalassemia
beta or alpha
Microcytic, hypochromic
RDW is normal
Hgb electrophoresis*
Suporrtive care maybe transfusion, iron chelation, splenectomy
severe beta- stem cell transplant
Sickle cell
vaso occlusive pain most common sign
chronic hemolysis
splenic infarcts -> functional splenia
Hgb electrophoresis needed
Tx: avoid triggers
med: Hydroxyurea
stem cell transplant
G6PD
enzyme defect that -> hemolytic anemia
African, Mediteranea, Asian
Episodic hemolyis
Signs: jaundice HEINZ BODIES (bite cells)
Supportive and avoid triggers
Lead poisoning
Basophillic stippling on labs (little black dots)
tx: chelation
Primary polycythemia
familial only RBC affected Hgb can be as high as 27 Plethora and splenomegaly HA and lethargy Tx: phlebotom
Secondary polycythemia
in response to hypoxemia (Cyanotic heart condition or CF)
Tx: correct underlying
phlebotomy prn
Normal platelet count
150-400K
Risk of spontaneous bleeding occurs when platelet levels are
<20K
PT
extrinsic factor
7
PTT/aPTT
intrinsic
8, 11, 12
INR
more accurate reflection of PT
Warfarin monitoring
Bleeding time
test platelet fx
ITP
most common bleeding disorder of childhood
ITP
follows VIRAL infection
2-5 YO
immune mediated
ITP
Immune mediated attack on platelets- they are phagocytozed by splenic macrophages
SPLEEN eats the PLATELETS
Sx of ITP
multiple petechiae
bruising
nosebleeds
Lab ITP
Thrombocytopenia
normal everything else
Tx for ITP
Observe is Asx Avoid ASA, NSAIDs Bleeding precautions Maybe Prednisone IVIG Splenectomy
Von Wille
most common inherited bleeding disorder
decrease or impairment of VWF
can’t clot correctly
VWF binds to factor 8!!!
Von Wille clinical
Prolonged bleeding from mucosal surface
easy bruisin
What lab is abnormal with Von Wille?
factor 8
aPTT because this is intrinsic and measures factor 8
Also, prolonged bleeding time
Tx of Von Wille
Desmopressin
VWF replacement therapy
Hemophilia A
factor 8 def
most common
Hemophilia B
factor 9
Hemophilia sx
varies w severity-
mild: bleed in response to trauma, may not be apparent til later in life
severe: severe and spontaneous bleeding, earlier age of first bleeding episode
onset of bleeding may be days
Hemophilia bleeding sx
can occur anywhere, but most commonly into JOINTS and MUSCLES
Spontaneous hemarthrosis- severe dz, if recurrent can lead to destruction
Hemophilia labs
Everything normal except MAY have prolonged aPTT
Hemophilia tx
Desmopressin for type A
Factor replacement for both
DIC
acquired
bleeding, clotting, bleeding, clotting
DIC
triggered by an event- sepsis, trauma, CA
Signs of DIC
Shock- end organ dysfx
Diffuse bleeding tendency- hematuria, melena, purpura, petechiae, oozing
Evidence of thrombotic lesions- major vessel clots, p”purpura fulminans”
Labs of DIC
decreased platelet count
prolonged aPTT AND PT
decreased fibrinogen level
Elevated D-Dimer and FDP!!!
Liver makes:
prothrombin, fibrinogen
factors 5,7,9,10,13
Vitamin K dependent factors
2,7,9,10
Newborn period: physiologically depressed activity of the vitamin K dependent factors
Platelet count with Liver dz
normal or low
Liver dz or Kidney dz
prolonged PT and aPTT
Protein C def
normal activated protein C turns off factors 5 and 8
These pts may develop “Warfarin induced Skin Necrosis”
Protein S def
S is a cofactor for protein C
Factor 5 Leiden
increased risk of CLOT bc resistant to inactivation by protein C
35 fold heterozygous and taking birth control
80 fold homozygous for factor 5
Antithrombin def
CLOT
efficiency of Heparin may be diminished if they have this def
Hypercoag disorders
increased risk of CLOT
Tx proph with anticoags
UFH, LMWH, Warfarin
Vasculitis- HSP
most common type of small vessel vasculitis
boys 2-7 YO
URI often precedes dx
deposition of IgA immune complexes
HSP affects
small vessels of skin, GI tract, kidneys
Clinical presentation of HSP
Palpable purpura
Arthritis/Arthralgia
Abd pain
Renal dz
Labs of HSP
normal or ELEVATED platelet count ASO elevated Serum igA may be elevated Hemoccult (+) UA: hematuria, sometime protein
Benefits of circumcision
Decrease rate of: UTI penile CA penile inflammation/dermatoses some STD
Risk of circum
procedure related complications: inadequate skin removal, bleeding, infection, urethral comps
Contraindications to circum
Unstable infant
Congenital penile abn
Phimosis
inability to retract foreskin
physiologic vs pathologic
Presentation of Pathologic Phimosis
Non-retractable after previousl being able to retract Painful erection Irritation/bleeding Dysuria/ urinary retention Recurrent infection
Tx of pathologic phimosis
Stretching exercises
Topical steroid
Circumcision (often not needed though)
Paraphimosis
MEDICAL EMERGENCY
retracted skin that cannot be returned to normal position- entrapment- cut off blood flow- engorgement - arterial compromise
Paraphimosis (!)
caused by: forced retraction by caretaker infection or inflammation GU procedure sexual activity, trauma
Paraphimosis Physical Exam
Color change (blue/black) if ischemia is present Edema and tend of glans tender swelling of distal retracted foreskin (constricting band)
Tx of Paraphimosis (!)
Pain control
Timely manual reduction in office or ED
Surgical intervention by urology (DORSAL SLIT PROCEDURE)
Epispadias
DORSAL
displacement of urethra
May happen with Bladder Exstrophy (exposed bladder)
Hypospadias
VENTRAL displacement of urethra
can be on glans, shaft, scrotum, perineum
May also involve chordee (curvature)
Pt has hypospadias and chordee, and cyptor…. consider
DSD Disorder of Sexual Development
Tx of Hypospadias +/- Chordee, and cyptor …
DO NOT DO CIRCUM during newborn period
need that foreskin to perform surgery at ~6 months of age to fix Hypo and Chordee
Cryptor
testis do not descend by 4 months
Cryptor
most common GU congenital abn- 70% resolve spontaneously
Cryptor can increase risk of
Testicular torsion
Subfertility (risk improves if corrected b4 1 YO)
Testicular CA
Retractile
overactive cremasteric reflex pulls testis back inside
Cryptor
usually unilateral, if bilateral (10%) further testing is recommended!!
Most common location for Cryptor (hidden testis)
Suprascrotal
Tx for Cryptor
Urology referral
If they haven’t descended by 4 mo, rare that they will on their own
Surgery recommended ASAP after 4 mo mark- ideally before 2 YO
Orchiopexy surgery
Tx Cryptor: testicle is repositioned and attached into the scrotum
Allows for improved testicular growth and fertility potential
Testicular torsion (!)
twisting of spermatic cord d/t poorly anchored testicule
*Risk of vascular compromise