GI/GU, Heme, Onco Flashcards

1
Q

inability to retract foreskin

A

phimosis

physiologic (no tx) vs pathologic (tx required)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

painful erection
irritation/bleeding
dysuria

A

phimosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Phimosis treatment

A

stretching exercises wth moisturizer
topical corticosteroid (ie betamethasone)
circumcision

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

retracted skin in uncircumcised male that cannot be returned to natural position

A

paraphimosis

impaired venous flow - engorgement - arterial compromise

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q
swelling of penis
penile pain
irritability in non-verbal infant
edema and tenderness of glans
penile shaft flaccid and unaffected
color change if ischemia
A

paraphimosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Paraphimosis treatment

A

medical emergency

manual reduction in office or ED for dorsal slit procedure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

congenital anomaly that results in abnormal ventral displacement of urethra

A

Hypospadias and chordee (abnormal penile curvature)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

abnormal foreskin
abnormal penile curvature
2nd opening (1 false)

A

Hypospadias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Hypospadias treatment

A

check normal penile length and curvature
refer to urology
circumcision NOT to be done during newborn period
surgery 6mo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

testis not within the scrotum and does not spontaneously descend into scrotum by 4mo
suprascrotal most common

A

Cryptochidism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

absent testicle UL or BL with flat underdeveloped scrotum

absent, undescended, retractile or ascending/ectopic

A

Cryptochidism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Cryptorchidism treatment

A

70% spontaneous descent (rare after 6mo)

surgery before 2yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

abrupt onset sever testicular or scrotal pain (may radiate)
n/v
edematous and erythematous scrotum
tender, swollen, slightly elevated testis
absent cremasteric reflex
- phren’s sign (+ = relief of pain when scrotum is elevated epididymitis)

A

Testicular torsion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Testicular torsion diagnostics and treatment

A

doppler US confirms
*medical emergency - immediate consult and surgical detorsion and fixation of both testes
within 4-6 hours = 100% viable
after 24 hours = 0% viable

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q
younger children:
fever
vomiting
irritability
poor appetite

older children:
dysuria and/or frequency
new onset urinary incontinence
abdominal and back pain

A

Urinary Tract Infection (UTI)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Urinary Tract Infection (UTI) risk factors

A
female
urinary tract anomalies
sexual activity
catheterization
VUR

girls and uncircumcised boys under 2yo with at least 1 risk factor for UTI are most at risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Urinary Tract Infection (UTI) diagnostics and treatment

A

UA and culture and sensitivity (C&S)
+ leukocyte esterase (produced when WBC dying)
+ nitrites (G- rods like E.coli)
full septic workup for neonates esp if febrile
*RBUS = 1st line imaging study

abx PO/IV empirically until sensitivity results - 7-14days
amoxicillin, augmente, cephalosporin, Bactrim
f/u UA/C&S not required

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

When to do RBUS

A

under 2yo with first febrile UTI
any age with recurrent UTI
UTI and +FH renal/urologic disease, poor growth, HTN
don’t respond to abx

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

retrograde flow of urine from bladder into upper urinary tract

A

Vesicoureteral Reflux (VUR)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

hydronephrosis (swelling of kidney) prenatally

febrile UTI in odler child

A

Vesicoureteral Reflux (VUR)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Vesicoureteral Reflux (VUR) diagnostics and treatment

A

*voiding cystourethrogram (VCUG)

low dose prophylactic abx (i.e. Bactrim)
surgical options
aggressive screening with UA if UTI sxs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

urinary incontinence during the day after 4yo or at night after 5yo (girls) or 6yo (boys)

A

Enuresis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Enuresis diagnostics and treatment

A

UA with specific gravity (can child concentrate urine? DM)
KUB

behavioral modifications vs pharmacology
timed voiding, increase hydration, no fluids 90min before bed
desmopressin (DDAVP - synthetic ADH) over 6yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

painful or painless urinating
parent complaining child’s urine is red or brown

check BP, edema, skin for purport, CVA and abd tenderness

A

Hematuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Hematuria diagnostics

A
microscopic = 3+ RBCs per hpf
urine cx if UTI suspected
Ca/Cr ratio (over 2.0 abnormal)
RBUS
f/u yearly
gross = COLORED
UA and cx
BUN/Cr and Ca/Cr with GFR
ASO and ANA titers (infection)
RBUS
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q
gross hematuria - dark colored urine
elevated serum Cr
edema
HTN
urine microscopy with elevated RBCs and *CASTS
A

Glomerular Disease (glomerulonephritis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

acute onset cola-colored urine
renal insufficiency
+ASO titer
recent infection with or without culture (1-2wk post infection)

A

Post-infectious glomerulonephritis (GN)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q
abd pain
\+/- bloody diarrhea
maculopapular purpuric rash
renal involvement varies (often microhematuria)
arthritis/arthralgia
A

Henoch-Schonlein Purpura (HSP) - GN

IgA autoimmune response to infectious process that may occur post-URI

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

bloody diarrhea
hemolysis
renal fault (hematuria)
electrolyte problems –> seizures

*associated with E.coli O157:H7 infection

A

Hemolytic-Uremic Syndrome (HUS)

*most common glomerular vascular cause of acute renal failure in childhood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q
microhematuria
proteinuria
HTN
deafness
visual disturbances
A

Alport syndrome

hereditary GN - autosomal dominant X-linked

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Nephrotic Syndrome characteristics

A

nephrotic range proteinuria (over 1000mg/24hr)
hypoalbuminemia
edema (esp. of the face)
hyperlipidemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q
2 presentations:
"happy spitter" - growing well, healthy clinically
"unhappy spitter"
FTT
fussy/dystonic neck posturing
resp. complications
feeding refusal
occult blood in stool
A
Gastroesophageal Reflux (GER)
GERD if sxs present

common under 6mo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Gastroesophageal Reflux (GER) treatment

A

positional therapy
elimination diet or change formula
thickened, small, frequent feeds

meds:
H2 blocker (ranitidine) under 1yo
PPI (lansoprazole) over 1yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

projectile, non-bilious vomiting after feeding
“hungry vomiter”
FTT and dehydration

distended upper and after feeding
prominent peristaltic waves L –> R
“olive” sized mass in RUQ

A

Pyloric stenosis

3-12wks old

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Pyloric stenosis diagnostics and treatment

A

CBC, CMP
US test of choice
UGI if US non-diagnostic –> string sign

IV fluids and electrolytes
pyloromyotomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

bile-stained vomiting first 24-48hrs of life
mild abd distention
*failure to pass meconium

A

Congenital Atresia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Congenital Atresia types

A

duodenal (8-10wk gestation) - associated with trisomy 21
embryonic development
jejunoileal (over 11-12wk gestation) - associated with CF
uterovascular accident
colonic (least common)
unknown

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Congenital Atresia diagnostics and treatment

A

CMP
KUB - double bubble sign for duodenal atresia
dilated loops of bowel and air fluid levels for J/C
UGI/contrast enema confirm dx and ID area of obstruction

IV fluids, electrolytes, possibly TPN
surgery
broad spec abx to prevent infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

bilious vomiting
abd pain, tenderness and distention
+/- hematochezia
visible peristalsis

A

Midgut malrotation +/- volvulus

under 1mo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Midgut malrotation +/- volvulus diagnostics and treatment

A

KUB
UGI - corkscrew appearance
barium enema to confirm (only as adjunct)

surgical intervention

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q
intermittent, severe cramps abd pain
cries and draws legs to chest
vomiting - does not feed
*currant-jelly* stools
palpable sausage shape in RUQ
A

Intussusception

most frequent cause of intestinal obstruction in first 2yr of life

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Intussusception diagnostics and treatment

A

CBC, CMP
abd US

IV fluids
surgical consult
air enema US-guided = reduces with 74% success

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

painless rectal bleeding
obstruction
diverticulitis
*can mimic appendicitis

A

Meckel’s Diverticulum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Meckel’s Diverticulum Rule of 2’s

A

2% population
2:1 M:F ratio
2% symptomatic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Meckel’s Diverticulum diagnostics and treatment

A

technetium-99 scan (Meckel’s scan)

surgical resection of diverticula and remnant (vitelline duct)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q
anorexia
migrating abd pain (periumbilical to RLQ)
vomiting after onset pain
fever
guarding
rebound tenderness
\+Rovsing, obturator and ileopsoas signs
A

Appendicitis

common in 2nd decade of life
rare under 5yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Appendicitis diagnostics and treatment

A

H&P
US
elevated WBC

fluids, electrolytes and abx pre-op
appendectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

failure to pass meconium in first 48-72hr of life
bilious vomiting
abd distention
reluctance to feed/irritable
older children: FTT and chronic constipation

“squirt” or “blast” sign with releasing finger from anal canal

A

Hirschsprung’s Disease

first 6wk of life
more than 20% develop enterocolitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

occurs secondary to absence of ganglion cells in the mucosal and muscular layers of the colon

peristaltic waves cannot extend beyond this zone of de-nervation

A

Hirschsprung’s Disease

most common cause of lower bowel obstruction in neonates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Hirschsprung’s Disease diagnostics and treatment

A

rectal biopsy is GOLD STANDARD
contrast enema

resection of aganglionic segment
colostomy
colorectal anastomosis after bowel has rested

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q
diarrhea/abd pain
\+/- hematochezia
weight loss and growth failure
arthritis
uveitis
nutrient deficiency and anemia
A

Inflammatory Bowel Disease
Crohn’s and Ulcerative colitis

20-30% present under20yo
more prevalent in whites

52
Q

Inflammatory Bowel Disease diagnostics and treatment

A

colonoscopy
Crohn’s = skip lesions, cobblestone appearance
Ulcerative colitis = continuous involvement, erythematous
and friable mucosa with small erosions

disease maintenance (anti-inflammatories)
biologics and immunomodulating agents
steroids used for flare-ups ONLY

53
Q
fever
severe abd pain
bloody stool/vomiting
dehydration
leukocytosis
growth/development issues
A

Diarrhea
>3 watery stools per day

infectious vs diet related

54
Q

Diarrhea treatment

A

hydration
abx sometimes
anti-motility agents rarely

55
Q
\+/- fecal leakage (encopresis)
abd discomfort
decreased bowel sounds and distention
stool mass may be palpated in LLQ
anal/rectal exam --> fissures, anatomic placement, check for fecal impaction
A

Constipation

increased prevalence 5-6yo

56
Q

Constipation red flags

A
weight loss/poor growth
anorexia, fever, hematochezia, vomiting
hx delayed passing of meconium
acute onset
failure to respond to conservative measures
57
Q

Constipation diagnostics and treatment

A
CBC, CMP, TTG, IgA, TSH
abd xray r/o impaction
MRI spine (neuro)
rectal biopsy (Hirschsprung's)
barium enema (anatomical malformations)
decrease dairy intake
polyethylene glycol to relieve impaction
laxative therapy
counseling
GI referral if worse or alarming sxs
58
Q

Neonatal and Breast Milk Jaundice

A

hyperbilirubinemia (indirect bili most commonly)
benign - resolves spontaneously

breast milk = appears in 1st wk of life

59
Q

Pathological Jaundice

A

hyperbilirubinemia that requires intervention

diagnostics:
serum bili
maternal blood group and Ab titers
baby blood group
Coombs test (hemolytic anemia)
CBC, retic count, peripheral smear
septic/infectious workup
60
Q

jaundice
dark urine
light stools

increase in direct bilirubin
treat with liver transplant

A

Post-Hepatic Jaundice (Biliary Atresia)

rare disease that destroys the bile ducts

61
Q

lethargy
tachycardia
pallor
irritability and poor oral intake (infants)

A

Anemia (acute)

62
Q

abnormal skin pigmentation
short stature
skeletal malformations

progressive pancytopenia

A

Fanconi anemia
autosomal recessive

up to 90% develop bone marrow failure in first 10yr of life

63
Q

Fanconi anemia diagnostics and treatment

A

pancytopenia
bone marrow hypoplasia or aplasia (hypocellular)
*often misdiagnosed as ITP

supportive
hematopeoitic stem cell transplant (HSCT) is DEFINITIVE tx

64
Q

weakness/fatigue
pillow
frequent, severe infections
purpura, petechiae, bleeding

A

Acquired Aplastic Anemia

65
Q

Acquired Aplastic Anemia diagnostics and treatment

A

normocytic anemia
pancytopenia
low relic count
*overwhelming infection and severe hemorrhage are leading COD

supportive (transfusions)
stop offending agent
abx if infection
HSCT if absolutely needed

66
Q
sometimes asymptomatic
pallor
fatigue/irritability
played motor development
hx of Pica (non-nutritive cravings)
A

Iron Deficiency Anemia

1-2yo and females of child-bearing age
AA and Hispanics more than whites

67
Q

Iron Deficiency Anemia diagnostics and treatment

A

microcytic, hypochromic anemia
low Hgb and ferritin

close monitor
iron supplement (6mg/kg/d)
68
Q

pallor
glossitis

specific:
paresthesia, weakens, unsteady gait
decreased vibratory and proprioception

A

Megaloblastic Anemia
vitamin B12 deficiency (neuro deficits)
folic acid deficiency

69
Q

Megaloblastic Anemia diagnostics and treatment

A

macrocytic (elevated MCV and MCH)
decreased folic acid and/or vitamin B12
*hypersegmented nuclei in large neutrophils

Vitamin B12 and/or folic acid supplementation
–> vit B12 shows methylmalonic acid

70
Q

Congenital Hemolytic Anemias

A
Hereditary Spherocytosis (HS)
Thalassemia
Sickle Cell disease
G6PD Deficiency
lead poisoning
71
Q

Hereditary Spherocytosis (HS)

A

hemolytic anemia
red cell membrane defect = trapped in spleen
jaundice, gallstones, splenomegaly

spherocytes on peripheral smear
*increased osmotic fragility

supportive, +/- RBC transfusion
splenectomy

72
Q

Thalassemia (alpha or beta)

A

hemolytic, microcytic, hypochromic anemia
*Hbg electrophoresis for dx

supportive, +/- RBC transfusion
iron monitoring
splenectomy (wait intel at least 6yo)
HSCT for severe beta thalassemia

73
Q

Sickle Cell disease

A
hemolytic anemia
homozygous HbSS
vaso-occlusion = PAIN (abd esp.)
gallstones, jaundice, splenomegaly and functional asplenia
growth failure and delayed puberty

Hgb electrophoresis
*Jolly bodies in asplenic pts.

avoid precipitating factors
*hydroxyurea helps painful episodes
stem cell transplant

74
Q

G6PD deficiency

A

hemolytic anemia
red cell ENZYME defect with X-linked recessive inheritance

episodic hemolysis at times of exposure to oxidative stress or certain drugs/food substances
*Heinz bodies on peripheral smear (denatured Hgb)

supportive and avoid triggers

75
Q

Lead Poisoning

A

mild hemolytic normocytic anemia
*basophilic stippling on peripheral smear

chelation therapy

76
Q

splenomegaly and plethora (deep red color)
h/a and lethargy (increased blood viscosity = thrombosis)
elevated Hgb

tx: phlebotomy

A

Congenital Erythrocytosis (inherited polycythemia)

77
Q

occurs in response to hypoxemia

tx: correction of underlying disorder (i.e. chronic pulmonary disease or cyanotic congenital heart disease)
phlebotomy when indicated

A

Secondary Polycythemia (acquired - more common)

78
Q

Bleeding disorder screening

A

CBC
peripheral smear
PT/INR (extrinsic pathway - 7 and TF) - prothrombin time
monitor Warfarin tx
aPTT (intrinsic pathway - 8, 9, 11, 12) - activated partial thromboplastin time
+/- bleeding time (prolonged in platelet disorders)
differentiate von Willebrand disease vs hemophilia

79
Q

multiple petechiae
ecchymosis
epistaxis

2-5yo most common and often following a viral infection

A

Idiopathic Thrombocytopenia Purpura (ITP)

most common bleeding disorder of childhood
immune-mediated against own platelets

80
Q

Idiopathic Thrombocytopenia Purpura (ITP) diagnostics and treatment

A

thrombocytopenia
normal WBC and Hgb (unless hemorrhage)
normal PT and aPTT
*diagnosis of exclusion

90% have spontaneous remission
*prednisone
avoid NSAIDs/ASA (compromise platelet function)
bleeding precautions
IVIG for more severe bleeding
splenectomy for emergent scenarios
81
Q

prolonged bleeding from mucosal surfaces
epistaxis, menorrhagia, GI
easy bruising

A

von Willebrand Disease

most common INHERITED bleeding disorder
autosomal dominant

82
Q

von Willebrand Disease diagnostics

A
normal PT
prolonged or normal aPTT (vWF is a co-protein for factor 8)
normal or decreased factor 8
normal or deceased vWF
*prolonged bleeding time
83
Q

von Willebrand Disease treatment

A

desmopressin (synthetic ADH) causes release of vWF and factor 8 from endothelium
vWF replacement therapy

84
Q

bleed in response to injury/trauma or surgery
severe, spontaneous bleeding

onset of bleeding may be delayed by several days
commonly occurs INTO joints and muscles
–> spontaneous hemarthrosis is very severe and can lead to joint destruction and deformity

A

Hemophilia A and B
type A = factor 8 deficiency (rare)
type B = factor 9 deficiency (Christmas disease)

X-linked inheritance

85
Q

Hemophilia diagnostics

A

normal platelet count, PT and bleeding time
prolonged aPTT (intrinsic pathway)
may be normal in mild disease
normal vWF

86
Q

Hemophilia treatment

A
Desmopressin (type A)
factor replacement (more effective)
87
Q

shock (end organ dysfunction)
hematuria, petechiae, purpura
persistent oozing from needle puncture
evidence of thrombotic lesions

A

Disseminated Intravascular Coagulation (DIC)

hemorrhage and microvascular thrombosis
*clotting AND bleeding problem

88
Q

Disseminated Intravascular Coagulation (DIC) diagnostics

A

decreased platelets
prolonged aPTT and PT
decreased fibrinogen level
increased D-dimer and fibrin degradation products

*everything is abnormal

89
Q

Disseminated Intravascular Coagulation (DIC) treatment

A

ID and treat triggering event (sepsis, trauma or malignancy)
replacement therapy
anticoagulation therapy when indicated

90
Q

Liver disease and vitamin K deficiency

A

Liver is major site of synthesis for:
prothrombin, fibrinogen, factors 5, 7, 9, 10, 12, 13
normal to low platelet count
prolonged PT and aPTT

Vitamin-K dependent factors include:
2, 7, 9, 10
normal platelet count
prolonged PT and aPTT

treat underlying condition and give vitamin K at birth

91
Q

Inherited Thrombotic Disorders

A

Protein C, Protein S and Antithrombin deficiencies
Factor V Leiden Mutation

hypercoaguable
increased risk for thrombosis
may see DVT or PE
+ family hx thrombosis

92
Q

Protein C deficiency

A

activated protein C inactivates activated factors 5 and 8
stops the clotting process when you don’t need it

pts may develop Warfarin-induced skin necrosis

93
Q

Protein S deficiency

A

protein S is a cofactor that activated protein C to help stop the clotting process when you don’t need it

94
Q

Factor V Leiden Mutation

A

more commonly seen – point mutation
factor 5 resistant to inactivation by activated protein C

35-fold increased risk of indecent VTE if taking oral contraceptives

95
Q

Antithrombin deficiency

A

major physiologic inhibitor of thrombin
clinically presents as VTE

*efficiency of heparin may be significantly diminished

96
Q

Inherited Thrombotic Disorders treatment

A
anticoagulation prophylaxis (UFH, LMWH, Warfarin)
1st episode VTE = anticoag for min 3mo
97
Q

Henoch-Schonlein Purpura (HSP) diagnostics and treatment

most common small vessel vasculitis
boy 2-7yo

A

normal platelet (despite purpura)
elevated ASO titer
elevated serum IgA

supportive

98
Q

uncontrolled proliferation of malignant lymphoid precursor clones
most common pediatric CA (3-7yo)
stain with TdT

pancytopenia with circulating blasts
bone pain
fatigue

A

Acute Lymphoblastic Leukemia (ALL)

99
Q

uncontrolled proliferation of malignant myeloid precursor clones
stain with myeloperoxidase
*Auer rods on peripheral smear

pancytopenia with circulating blasts
bone pain
fatigue

A

Acute Myeloid Leukemia (AML)

100
Q

Acute Leukemia (ALL/AML)

A

pancytopenia - pillow, tachycardia, SOB, ecchymosis, fever
infiltration of organs
skin
gum hypertrophy, CNS and thymic mass (ALL)
liver, spleen and nodes

101
Q

Acute Leukemia (ALL/AML) diagnostics

A
*bone marrow biopsy confirms
CBCD (blasts) and CMP
flow cytometry (looks for abnormal WBC)
coag tests (DIC)
lumbar puncture if neuro sxs (ALL)
uric acid
102
Q

Acute Leukemia (ALL/AML) treatment

A
hydration
transfusions PRN
abx
allopurinol or rasburicase to decrease uric acid
\+/- allogenic transplant with chemo
103
Q
may be asxs
fever, dyspnea
anorexia and early satiety (splenomegaly)
weight loss
night sweats

strikingly elevated WBC found on routine CBC

A

Chronic Myeloid Leukemia (CML)

translocation of philadelphia chromosome

104
Q

Chronic Myeloid Leukemia (CML) treatment

A

*Gleevec (TKI) shuts down transcription/translation
hydrea (brings down WBC if too high)
allogenic transplant
monitor

105
Q
painless, persistent LAD
hepatosplenomegaly
night sweats
pruritic
mediastinal bulky mass
A

Lymphoma (Non-Hodgkin’s or Hodgkin’s)

106
Q

Non-Hodgkin’s Lymphoma (NHL)

A

slow professing to very aggressive in adults
high grade in children (requires treatment)

5th most common peds CA under 15yo

107
Q

Hodgkin’s Lymphoma

A

bimodal (teens-30s, 50s)
EBV exposure
*Reed Sternberg cells on cytology

painful LAD after EtOH
supraclavicular nodes

108
Q

Lymphoma (Non-Hodgkin’s or Hodgkin’s) diagnostics

A
*excisional node biopsy differentiates
CBC, CMP, LDH (lactic dehydrogenase = cell turnover)
uric acid
CT neck, chest, abd, pelvis
bone marrow biopsy for staging

2D ECHO if on Adriamycin (can cause DCM or leukemia)
pulmonary testing if on Bleomycin

109
Q

Non-Hodgkin’s Lymphoma (NHL) treatment

A

chemo
autologous stem cell transplant
supportive (allopurinol/rasburicase)

steroids are toxic for lymphocytes
good prognosis

110
Q

Hodgkin’s Lymphoma treatment

A

chemo +/- radiation (even in children)
stem cell transplant for relapse
increased risk for leukemia and other lymphomas

steroids are toxic for lymphocytes

111
Q

Lymphoma staging

A

I - 1 area of nodes
II - 2 areas of nodes on 1 side of diaphragm
III - nodal sites on BOTH sides of diaphragm
IV - involvement of extranodal sites (i.e. bone marrow)

B sxs = night sweats, weight loss and bulky nodes
A = none of the above
E = extra nodal site contiguous with nearby node

112
Q

pain and swelling
commonly femur or tibia
fever, malaise
weight loss

corresponds with growth spurts

A

Osteosarcoma

most common primary bone malignancy in children and adolescents

113
Q

Osteosarcoma diagnostics and treatment

A

increased alk phos and LDH
MRI
CT for mets
*biopsy to confirm

surgery + chemo + radiation

114
Q

pain and swelling
+/- fever
12% have urogenital anomalies
high chance of relapse

A

Ewing’s Sarcoma

peak 10-15yo
males

115
Q

Ewing’s Sarcoma diagnostics and treatment

A

CBC, CMP, LDH
CT/MRI
PET if you think metastatic (commonly to lungs)
*biopsy to confirm

surgery
chemo (cytoxan and doxyrubicin) +/- radiation
116
Q
n/v
seizures, syncope, hemiparesis
papilledema
dysphagia
personality changes
CN palsies
FTT
A

Brain Tumor (metastatic vs primary)

glial more common than nonglial

2nd COD for neuro

117
Q

Brain Tumor diagnostics and treatment

A

MRI confirms
f/u MRI indicated (progression vs. pseudo)

surgery +/- radiation (temodar) and chemo

118
Q
abd mass/pain
fever, weight loss
Horner's syndrome
bone pain
anemia

can synthesize and secrete catecholamines

A

Neuroblastoma

most common solid neoplasm in children
40% adrenal gland

119
Q

Neuroblastoma diagnostics and treatment

A

bone marrow biopsy
abd imaging
urine catecholamines

watch and wait if low risk
surgery (+ chemo if mets to spine)

under 1yo better prognosis

120
Q

abd swelling
fever
hematuria
HTN

BL involvement in younger children

A

Nephroblastoma (Wilms tumor)

most common renal malignancy

under 15yo vs renal cell CA 15-19yo

121
Q

Nephroblastoma (Wilms tumor) diagnostics and treatment

A

CT or MRI

chemo + surgery or surgery + chemo

122
Q

leukocoria (white reflection from retina)

sporadic or genetic cause
deadly

A

Retinoblastoma

most common intraocular CA of childhood

123
Q

Retinoblastoma diagnostics and treatment

A

opthal exam
intraocular US or CT
MRI

enucleation (removal of eye)
external radiation
chemo

124
Q
affects soft tissue
can resemble fat, muscle or fibrous tissue
often in head/neck in younger children
   also orbit, GU, extremities
very rare
A

Rhabdomyosarcoma

arises from primitive mesenchymal cells

125
Q

Rhabdomyosarcoma diagnostics and treatment

A

MRI and CT or PET

surgery, chemo, radiation

126
Q

Hepatic Tumors

A

commonly a primary tumor found by a family member
could be mets from Wilms tumor or rhabdomyosarcoma

hepatocellular CA 0-4yo and 10-14yo
hepatoblastoma under 5yo - R side of liver
both require additional imaging to ensure it is not a benign tumor (i.e. adenoma or hemangioma)

tx: surgical resection