Peds II- Exam 1 Flashcards
1
Q
Abdominal mass that CROSSES midline
A
Neuroblastoma
2
Q
Most common solid tumor of childhood
A
Brain tumor
3
Q
ALL/LBL
Acute Lymphoblastic Leukemia
A
Most common CA of childhood
circulating lymphoblasts
4
Q
AML
A
Auer rods with circulating myeloblasts
5
Q
CML
A
Philadelphia chromo
rare in childhood
6
Q
Hodkin lymphoma
A
painless cervial and supraclavicular lymphadenopathy
Reed sternberg cells
7
Q
Ewing Sarcoma
A
2nd most common primary bone tumor
Affecting children and young adults
Nighttime bone pain
8
Q
Retinoblastoma
A
Most common intraocular tumor in kids
White pupillary reflex
9
Q
Rhabdomyosarcoma
A
Painless, progressive, enlarging mass
10
Q
Most common nutritional deficiency in kids
A
Iron deficiency
11
Q
Most common bleeding disorder of kids
A
Acute ITP
Idiopathic Thrombocytopenic Purpura
12
Q
Anemia
A
2 SD below normal for age and sex
13
Q
Anemia in age 6 mo- 5 YO
A
Hgb <11
14
Q
Normocytic and Normochromic anemia
A
anemia of chronic dz
15
Q
Microcytic, Hypochromic Anemias
A
Iron def
Thalassemia
Lead intox
16
Q
Macrocytic
A
B12 and Folate def
17
Q
Fanconi Anemia
A
Inherited
Progressive pancytopenia- all cell lines
Skin pigment, short stature, skeletal malform
Inc incidence of CA
often misdx as ITP
18
Q
Tx for Fanconi
A
Supportive
Stem Cell transplant
19
Q
Fanconi prognosis
A
Succumb to infection, bleeding, or CA in teen or early adult
Pt at high risk of developing MDS or AML
20
Q
Acquired Aplastic Anemia
A
any age
Peripheral pancytopenia
Low WBC with marked Neutropenia
Thrombocytopenia
Low reticulocytes
21
Q
Acquired Aplastic Anemia
A
Complications: overwhelming infection and severe hemorrhage: leading causes of death
foot picture with lost of bleeding- petechaie, purpura
22
Q
Tx for Acquired Aplastic Anemia
A
Supportive, refer, stop offender, Abx, Transfusion, platelet transfusion (sparingly), Immunosuppressive agent, Stem Cell *** tx of choice
23
Q
Iron Def Anemia
A
most common nutritional def in kids
Afirican Amer, Hispanic, poor communities
1-2 YO and mothers of childbearing age
24
Q
Iron Def Anemia
A
hx of Pica
delayed motor development
fatigue, irritable
25
Screen for Iron Def Anemia
@ 12 months!!
determine hg concentration and risk factors
26
Labs for Iron Def Anemia
Microcytic (Small)
Hypochromic
Ferritin <12
27
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
28
B12
malabsorption!!
29
Folic acid
increased requirements, malabsorption, dietary (rare), meds
30
B12
NEURO sx
31
Megaloblastic sx (B12 and folate)
Pallor
| Glossitis
32
Labs of B12 and Folate
Neutrophils are large and hypersegmented
Macro-ovalcytes; large, oval RBC
33
B12 only
Neuro and
| MMA elevated lab
34
Homo lab elevated in
both Folic Acid and B12 def
35
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
36
Thalassemia
beta or alpha
Microcytic, hypochromic
RDW is normal
Hgb electrophoresis*
Suporrtive care maybe transfusion, iron chelation, splenectomy
severe beta- stem cell transplant
37
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
38
G6PD
enzyme defect that -> hemolytic anemia
African, Mediteranea, Asian
Episodic hemolyis
```
Signs: jaundice
HEINZ BODIES (bite cells)
```
Supportive and avoid triggers
39
Lead poisoning
Basophillic stippling on labs (little black dots)
| tx: chelation
40
Primary polycythemia
```
familial
only RBC affected
Hgb can be as high as 27
Plethora and splenomegaly
HA and lethargy
Tx: phlebotom
```
41
Secondary polycythemia
in response to hypoxemia (Cyanotic heart condition or CF)
Tx: correct underlying
phlebotomy prn
42
Normal platelet count
150-400K
43
Risk of spontaneous bleeding occurs when platelet levels are
<20K
44
PT
extrinsic factor
| 7
45
PTT/aPTT
intrinsic
| 8, 11, 12
46
INR
more accurate reflection of PT
| Warfarin monitoring
47
Bleeding time
test platelet fx
48
ITP
most common bleeding disorder of childhood
49
ITP
follows VIRAL infection
2-5 YO
immune mediated
50
ITP
Immune mediated attack on platelets- they are phagocytozed by splenic macrophages
SPLEEN eats the PLATELETS
51
Sx of ITP
multiple petechiae
bruising
nosebleeds
52
Lab ITP
Thrombocytopenia
| normal everything else
53
Tx for ITP
```
Observe is Asx
Avoid ASA, NSAIDs
Bleeding precautions
Maybe Prednisone
IVIG
Splenectomy
```
54
Von Wille
most common inherited bleeding disorder
decrease or impairment of VWF
can't clot correctly
VWF binds to factor 8!!!
55
Von Wille clinical
Prolonged bleeding from mucosal surface
| easy bruisin
56
What lab is abnormal with Von Wille?
factor 8
aPTT because this is intrinsic and measures factor 8
Also, prolonged bleeding time
57
Tx of Von Wille
Desmopressin
| VWF replacement therapy
58
Hemophilia A
factor 8 def
| most common
59
Hemophilia B
factor 9
60
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
61
Hemophilia bleeding sx
can occur anywhere, but most commonly into JOINTS and MUSCLES
Spontaneous hemarthrosis- severe dz, if recurrent can lead to destruction
62
Hemophilia labs
Everything normal except MAY have prolonged aPTT
63
Hemophilia tx
Desmopressin for type A
Factor replacement for both
64
DIC
acquired
| bleeding, clotting, bleeding, clotting
65
DIC
triggered by an event- sepsis, trauma, CA
66
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"
67
Labs of DIC
decreased platelet count
prolonged aPTT AND PT
decreased fibrinogen level
Elevated D-Dimer and FDP!!!
68
Liver makes:
prothrombin, fibrinogen
| factors 5,7,9,10,13
69
Vitamin K dependent factors
2,7,9,10
Newborn period: physiologically depressed activity of the vitamin K dependent factors
70
Platelet count with Liver dz
normal or low
71
Liver dz or Kidney dz
prolonged PT and aPTT
72
Protein C def
normal activated protein C turns off factors 5 and 8
These pts may develop "Warfarin induced Skin Necrosis"
73
Protein S def
S is a cofactor for protein C
74
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
75
Antithrombin def
CLOT
efficiency of Heparin may be diminished if they have this def
76
Hypercoag disorders
increased risk of CLOT
Tx proph with anticoags
UFH, LMWH, Warfarin
77
Vasculitis- HSP
most common type of small vessel vasculitis
boys 2-7 YO
URI often precedes dx
deposition of IgA immune complexes
78
HSP affects
small vessels of skin, GI tract, kidneys
79
Clinical presentation of HSP
Palpable purpura
Arthritis/Arthralgia
Abd pain
Renal dz
80
Labs of HSP
```
normal or ELEVATED platelet count
ASO elevated
Serum igA may be elevated
Hemoccult (+)
UA: hematuria, sometime protein
```
81
Benefits of circumcision
```
Decrease rate of:
UTI
penile CA
penile inflammation/dermatoses
some STD
```
82
Risk of circum
procedure related complications: inadequate skin removal, bleeding, infection, urethral comps
83
Contraindications to circum
Unstable infant
| Congenital penile abn
84
Phimosis
inability to retract foreskin
physiologic vs pathologic
85
Presentation of Pathologic Phimosis
```
Non-retractable after previousl being able to retract
Painful erection
Irritation/bleeding
Dysuria/ urinary retention
Recurrent infection
```
86
Tx of pathologic phimosis
Stretching exercises
Topical steroid
Circumcision (often not needed though)
87
Paraphimosis
MEDICAL EMERGENCY
retracted skin that cannot be returned to normal position- entrapment- cut off blood flow- engorgement - arterial compromise
88
Paraphimosis (!)
```
caused by:
forced retraction by caretaker
infection or inflammation
GU procedure
sexual activity, trauma
```
89
Paraphimosis Physical Exam
```
Color change (blue/black) if ischemia is present
Edema and tend of glans
tender swelling of distal retracted foreskin (constricting band)
```
90
Tx of Paraphimosis (!)
Pain control
Timely manual reduction in office or ED
Surgical intervention by urology (DORSAL SLIT PROCEDURE)
91
Epispadias
DORSAL
displacement of urethra
May happen with Bladder Exstrophy (exposed bladder)
92
Hypospadias
VENTRAL displacement of urethra
can be on glans, shaft, scrotum, perineum
May also involve chordee (curvature)
93
Pt has hypospadias and chordee, and cyptor.... consider
DSD Disorder of Sexual Development
94
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
95
Cryptor
testis do not descend by 4 months
96
Cryptor
most common GU congenital abn- 70% resolve spontaneously
97
Cryptor can increase risk of
Testicular torsion
Subfertility (risk improves if corrected b4 1 YO)
Testicular CA
98
Retractile
overactive cremasteric reflex pulls testis back inside
99
Cryptor
usually unilateral, if bilateral (10%) further testing is recommended!!
100
Most common location for Cryptor (hidden testis)
Suprascrotal
101
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
102
Orchiopexy surgery
Tx Cryptor: testicle is repositioned and attached into the scrotum
Allows for improved testicular growth and fertility potential
103
Testicular torsion (!)
twisting of spermatic cord d/t poorly anchored testicule
*Risk of vascular compromise
104
Two peak incidences of Testicular torsion
Neonatal period
| Puberty 12-18
105
Sx and PE of Testicular Torsion
Abrupt onset of severe pain- constant
N/V
Swollen, indurated, red sctorum
"High riding scrotum"
**Absent cremasteric reflex**
Negative Prehn sign (elevated it does not help pain)
106
Testicular torsion
"High riding"
* Absent cremasteric reflex
* Negative Prehn sign
107
Dx of Test Torsion
Hx and PE can be enough
| but DOPPLER US- confirmatory test of choice
108
Tx of Test Torsion (!)
Immediate consult with urologist
| Surgical detorsion and fixation (Orchiopexy) of both testes if viable
109
Test torsion (!) can it be saved?
Detorsion within:
4-6 hrs: 100% viable
12 hrs: 20% viable
after 24 hrs: 0% viable
110
Cystitis vs Pyelonephritis
Cys: bladder
Pyelo: kidney
111
Most common cause UTI
bacterial- E. COLI (80%)
112
Risk Factor for UTI
Highest in uncircum boys <3 months
Girls have 2-4x greater risk than circumsized boys
113
How to collect UA
potty trained: clean voided specimen
not potty trained: CATHETER SPECIMEN RECOMMENDED
bag collection NOT recommended!
114
UTI dx
obtain UA AND Culture
UA: show significant bacteria with Pyuria (usually)
Leukocytes
Nitrites
C&S: help direct tx
115
UTI tx
Febrile: 10 days
| Afebrile (and immune competent): 3-5 days
116
UTI tx
Cephalosporin is 1st line oral agent in kids
Start empiric and then adjust based on C&S
117
F/u UA or culture for UTI?
not recommended when using C&S as long as kid is improving
118
Order RBUS (Renal and Bladder US) IF:
Non invasive 1st line imaging study for pts with UTI if:
<2YO with first Febrile UTI
Any age with UTI and FH of Renal or urologic dz, poor growth, or HTN
Children who aren't responding to abx
119
VCUG
invasive, radiation, catheter
| TEST OF CHOICE to detect VUR
120
When to order VCUG?
-Children any age with 2 or more Febrile UTIs
-Children of any age with 1st Febrile UTI AND on of following:
any anomaly on RBUS, temp 102.2 and pathogen other than E.Coli, or Poor growth/HTN
121
Clinical presentation of VUR
Hydronephrosis (prenatal, seen on US, fluid around the kidneys)
Post natal febrile UTI
122
Risks of pts with VUR
Recurrent infections
Scarring and damage to kidney
Many cases spontaneously resolve
123
Controversy regarding management of VUR
Watchful waiting
Low dose abx to prevent UTI
Surgical correction
```
Monitor reflux
Aggressive screening (UA) in febrile/symptomatic pts
```
124
Renal Scintigraphy (not ever 1st time)
| DMSA
Nuclear medicine scan
Detect Pyelonephritis and Renal Scarring
areas of decreased uptake indicate scarring and inflammation
125
DMSA scans
expensive, invasive, exposure to radiation
Not recommended in routine eval of kids w/first UTI
126
When to refer kids w/ UTI
```
Recurrent UTI
Severe VUR (grade 3-4)
Renal abn
Impaired kidney fx
Elevated BP
Bowel/ bladder dysfx
```
127
Horseshoe kidney
most common type of Renal fusion- fusion of one pole of each kidney
caused by abnormal migration of kidneys 5-9th week gestation
128
Sx of Horseshoe kidney
Pts usually Asx
| May have pain and hematuria if infection or obstruction
129
Associated stuff w horseshoe kidney
1/2-1/3 have something else:
VUR
Hypospadias
Cryptor
May be assoc with other genetic factors
Small increased risk for Wilms tumor
130
Wilms tumor
most common Renal malignancy in kids
131
Horseshoe kidney eval and tx
US, VCUG (if UTI) and Serum creatinine
If creatinine normal and no hydronephrosis- no further eval needed
if either is abnormal, need to get additional renal scans
132
Horseshoe kidney
excellent prognosis in most pts w/o any intervention
133
Horseshoe kidney AND VUR
consider Prophlyactic Abx
134
Nocturnal Enuresis: kids 5 yr or older
spontaneous resolution 15%/year
Reassurance, Pharm: Desmopressin (synthetic ADH): works but high relapse rate after d/c
135
Hematuria
positive dipstick is confirmed by Microscopic Examination
Microscopic hematuria >5 RBCs per hpf
136
Micro hematuria, if pt has no sx and normal physical,
Repeat UA weekly for 2 wks
137
If hematuria persists
Urine culture if UTI suspected
Close watch BP
RBUS
Referral
138
Gross hematuria
UA w microscopy/ urine culture
Serum creatinine
Serum complement
Consider ASO, streptozyme, ANA
Imaging: RBUS/ CT Without contrast
May need to refer
139
Post strep Glomeruloneph
7-14 days after GAS
usually pharyngitis or impetigo
Acute onset sx
140
Sx of Post strep glomeruloneph
Edema (periorbital, peripheral)
bc kidneys arent removing waste and fluid efficiently
Cola colored urine, elevated BP, Some renal insuff
141
Labs of poststrep glomeruloneph
Gross hematuria
*RBC Casts* - diagnostic of glomerulonephritis, Tubular shaped RBC
Proteinuria, increased serum creatinine
Demonstration of recent strep infection
+ throat or skin culture
+ASO or streptzyme
142
Tx for post strep glomeruloneph
supportive
143
HSP (IgA vasculitis Henoch-Schonlein Purpura)
immune mediated IgA Vasculitis
cause unknown
Classic tetrad:
1. palpable purpura (usually on legs)
2. Arthritis/arthralgia
3. Abd pain
4. Renal dz
144
Tx of IgA HSP
supportive
| sx usually spontaneously resolve
145
HUS
One of main causes of AKI in kids
Classic triad:
-Hemolytic anemia
-Thrombocytopenia
AKI (hematurie, proteinuria --> severe renal failure)
146
HUS
can be genetic
but also SHIGATOXIN producing E Coli is most common cause
Prodromal illness (abd pain, n/v/d) precedes HUS by 5-10 days
147
Proteinuria
```
Nephrotic syndrome:
Renal dz (intrinsic of post infectious) causing massive protein loss
```
148
Four main sx of Nephrotic syndrome
Nephrotic range proteinuria
Hypoalbuminemia
Edema (O face)
Hyperlipidemia
149
Reflux is common in infants what age
< 6 months
150
Unhappy spitter "GERD"
complications arise
FTT, esophagitis, respiratory complications
Irritable dystonic neck
occult blood in stool
151
GERD sx usually resolve by
9-12 months
| no big workup needed unless there are signs of something worse or other complications
152
GERD tx
```
1st line: lifestyle mod
avoid tobacco smoke
upright position 30 min after feed
hypoallergenic diet
do not overfeed
```
153
Complicated dz GERD
underlying medical condition i.e. Cystic Fibrosis
Esophagitis on endoscopy
154
Pharm Med for GERD
PPI (Omeprazole) vs H2 blocker (Ranitidine)
155
Hypertrophic Pyloric Stenosis
hypertrophy of pylorus can progress to near or complete gastric obstruction
156
Pyloric Stenosis
Genetic preD and environmental factors
Assoc with MACROLIDE us during first few weeks of life
157
Sx of Pyloric Stenosis
3-6 wk old infant with FORCEFUL vomiting
Non bilious PROJECTILE emesis
immediately after eating (postprandial)
Hungry vomiter
FTT and dehydration
158
Physical exam of pyloric stenosis
Olive like mass in RUQ
159
Dx Pyloric Stenosis
US: test of choice
160
Tests for Pyloric stenosis
US (test of choice) shows elongation and thickening
Upper GI Barium contrast study: use if US is non-dx. shows STRING sign of narrowed lumen
161
Tx of Pyloric stenosis
SURGERY :pyloromyotomy
| cut through the thickened area to open it up
162
Intestinal atresia
blockage of lumen!
| Duodenum is most common site
163
Increased risk of intestinal atresia in pts with:
Cystic Fibrosis
Down Syndrome
Maternal cig smoking
164
Congenital atresia (blockage) sx
Bilious Vomiting within 48 hrs of life
Abdominal distension
Failure to pass meconium +/-
165
Dx of intestinal atresia
Abdominal X Ray
Duodenal: "Double bubble" d/t gas and dilation in both stomach and duod
Jujenoileal/colonic atresia: dilated loops of bowel with air fluid levels
Can also do UGI or Contrast enema for confirmation
166
Tx of Congenital Atresia
Surgery!
feedings withheld
fluids, correct electrolytes
Broad spectrum abx to prevent post op infection
good prognosis
167
Midgut malrotation +/- Volvulus
Volvulus: small bowel twists around Superior Mesenteric Artery
Vascular compromise can lead to Small bowel ischemia and Necrosis!!
168
Sx of Midgut rotation +/- Volvulus
Vomiting (bilious)
Abd pain
Hemodynamic instable
+/- Hematochezia (passage of fresh blood) - sign of bowel ischemia
169
Physical exam findings of Midgut rotation
Abd distension and tenderness
170
Midgut rotation workup
X Ray to r/o perforation
UGI: GOLD STANDARD to detect malrotation +/- volvulus
- displacement of duod
- duod obstruction
- "corkscrew appearance"
US helpful to use with UGI but not best for confirming malrotation
171
Tx for Midgut rotation
Surgical Ladd Procedure: untwist bowel and reposition in abdomen- adhesions keep it in place
great prognosis
172
Intussusception: telescoping of intestine
Most common abdominal emergency in kids <2 yO!!
173
Intuss
usually kids 6 mo-3 YO
| most common cause of obstruction in these kids
174
Intuss sx
sudden onset intermittent, severe, crampy, progressive abdominal PAIN
crying, drawing up legs, vomiting becomes bilious as obstruction progresses
175
PE of Intuss
Sausage shaped mass (swollen bowel)
| Currant jelly stools
176
Triad of intuss
Abd pain
Abd mass
Currant jelly stool
177
Etiology of Intuss
Idiopathic 75%
| Other causes: Lead point- one part of bowel messed up and drags folding of rest of bowel with peristalsis
178
Contributing factors to "Lead point theory" and Intuss
```
Meckel divert (most common)
Crohn, Celiac, CF, Rotavirus!!! vaccine removed from marker
```
179
Intuss dx
US: test of choice!!
| "Target sign, coiled spring"
180
Intuss tx
Hydrostatic/ pneumatic enema is BOTH diagnostic and therapeutic
Tx of choice if there is no perforation or shock
181
Labs for Appendicitis
WBC > 10K increases likelihood
182
Diarrhea
3 or more water stools /day
183
Flagyl is tx for what types of diarrhea:
C-Diff and
| Parasitic
184
Most common cause of diarrhea
Viral: noravirus, rotavirus, adenovirus
185
Diarrhea red flags
```
Fever
sever abd pain
blood in stool
recent abx
persistent sx
dehdyation
leukocytosis
growth/dev delays
```
186
Chronic diarrhea
>1 month
187
Celiac dz
Immune mediated inflammatory dz of small intesting caused by GLUTEN insensitivity
Dx: igA to TTG, small bowel biopsy
188
Functional/ toddler's diarrhea
6 months - 5 YO
| self limited
189
2 main types if IBD
Crohn and Ulcerative Colitis
190
IBD can affect
mouth, skin, liver, joints also
| may get these extraintestinal sx before traditional sx
191
Crohn dz
```
Transmural inflammation (deeper)
Mouth to anus
SKIP lesions
Cobblestone appearance
Endoscopy OR Colonoscopy diagnostic
```
192
Ulcerative Colitis
CONTINUOUS from rectum to colon
superficial
Diffuse/continuous edema, erythema, friability, ulcers
*Inc risk of Colon CA!!
Dx: Colonoscopy only bc problem is closer to rectum
193
IBD tx
"Step up" therapy for most
"Step down" for high risk pts
```
Meds
Surgery
Nutritional rehab
Behavioral health supp
Colorectal screening for CA (older pts)
```
194
Meds for IBD
```
Aminosalic
Immunomodulating
Steroids- primary for acute flare!
+/- Abx
Surgery- refractory cases
```
195
Meckels divertic
outpouching of small intestine
rule of 2s
problem is gastric acid is released from the gastric epithelium where its not supposed to be released!!
Bleeding from mucosal ulceration
196
Meckels divertic
most common congenital anomaly of GI tract
197
Mecksl divertic sx
PAINLESS rectal bleeding
obstruction
diverticulitis
198
Labs for Meckels
Technetium 99 scan- this shows ectopic gastric mucosa in the diverticulum (where it should not be)
199
Tx for Meckels
Surgical resection
200
Encopresis
leakage of retained stool
201
Fiber rec for <2 YO
5g/day
202
Hirchsprung dz
Aganglionic megacolon!!
absence of ganglion cells
Spasm and abnormal movement!!
colon fails to relax --> can lead to obstruction
203
Hirchsprung dz higher risk in
Down syndrome
204
Sx of Hirchsprung dz
Classic: failure to pass meconium in first 48 hrs of life
Bilious vomiting
Abdominal distention
205
Unclassic sx of Hirchsprung dz
Later, if newborn passes meconium but develop later sx:
Chronic constipation and FTT (the later the dz, the less severe)
206
Physical exam and dx of Hirchsprung
Abdominal distension
Squirt sign!! tight anal sphincter- relieves obstruction temporarily
Dx:
- Rectal biopsy: GOLD STANDARD for dx
- Contrast enema to localize "transition zone"
207
Tx of Hirchsprung
Surgical resection of aganglionic colon
overall: good prognosis
208
ALL
Most common malignancy of childhood OVERALL
209
ALL
Bone pain
Anemia
Neutropenia
Lymphoblasts on smear* or biopsy. Replacing normal marrow
210
Tx of ALL
may take 2-3 yrs
Chemo-multidrug
Stem Cell transplant- best if from matched sibling
211
Independent predictors of Tumor Lysis Synd
Age >10
Splenomegaly
Mediastinal Mass
Initial WBC >20,000
212
AML
Myeloid cells get stuck in that stage
CNS sx maybe
Anemia, thrombocytopenia, neutropenia
WBC >100,000
213
AML and hyperleukocytosis
Hyperleuko >100,000 associated with life threatening complications!
214
AML
Circulating myeloblasts >20% are diagnostic!
Auer Rods- pathognomonic o myeloblasts
Dx: requires BOTH
- 20% or more blasts on biopsy
- leukemic cells must be myeloid
215
AML tx
2 courses chemo
| Stem cell transplant may be helpful
216
CML
Myeloprolif disorder
| Uncontrolled dividing of MATURE granulocytes
217
CML
Philadelphia chromosome!!
Only risk factor known: exposure to Ionizing Radiation
218
Tx for CML
Cure: STEM CELL
Control: Tyrosine Kinase Inhibitor
219
Hodkin Lymphoma
Familial dz
Epstein Barr Virus assoc
Starts in lymph nodes--> surrounding areas
REED STERNBERG cells
(b cells)
220
Hodkin Lymphoma sx
Painless cervical or supraclavicular adenopathy
Mediastinal mass- caution SVC syndrome
221
Tx for Hodkin
Chemo- combo
Radiation-low dose
Autologous stem cell
222
Non Hodkin lymphoma
Can arise in ANY SITE of lymphoid tissue --> DISTANT nodes
Associated with Immunodeficiency syndromes!!! & Epstein Barr Virus again
223
Unlike Adult Non Hodkin Lymphoma.. most children's cases are:
Rapidly prolif
High grade
Diffuse
224
Clinical findings of Non- Hod Lymphoma
FAST, 1-3 wks
Enlarging non tender lymphadenopathy
may see other classic sx and Hepato/Splenomegaly in advanced stage
Dx: tissue biopsy
225
Non- Hod Lymph Tx
BEWARE of TLS
Chemo
Stem cell option for those that relapse
226
Brain tumor
most common Solid tumor of childhood
| Leading cause of death from CA
227
30% brain tumors have this triad
Morning HA
Vomiting
Papilledema (eye)
228
Brain tumor dx
MRI: PREFERRED diagnostic study
229
Two categories of brain tumor
Glial (usually benign)
Nonglial (usually CA)
-Medulloblastoma
230
Neuroblastoma
most common ABDOMINAL tumor, most common solid outside of CNS
231
Neuroblastoma
Abdominal mass- fixed, irregular, CROSSES MIDLINE
doesnt stay in ints lane
often mets to bone
232
Labs with Neuroblastoma (abdominal)
Anemia
| Urinary Catecholamines***
233
Neuroblastoma dx
Histology confirmation OR
| Evidence of mets to bone + Urine catecholamines
234
Tx for Neuroblastoma
Surgery + Chemo
| surgery alone if low grade
235
Nephroblastoma
Wilms tumor
Second most common abdominal
KIDNEYS
236
Nephroblastoma/Wilms tumor sx
Asx abdominal mass/swelling
Smooth, firm, well demarcated
DOES NOT cross midline
237
Dx of Wilms
Histology confirm from biopsy or surgical excision
238
Tx of Wilms/nephroblastoma
Surgical, chemo, radiation
239
Osteosarcoma
Most common primary bone CA in peds
peak incidence: 13-16
time of rapid bone growth
240
Osteosarcoma
occurs in long bones (Metaphysis)
241
Cardinal sx of bone tumor
Bone pain @ site
Mass formation
Fracture thru area of destruction
242
Evaluate for mets to lungs after dx with:
Osteosarcoma
243
Tx for Osteosarcoma
Surgery
| Chemo
244
Ewing Sarcoma
White Males: 20s
| Often in long bones, but in the DIAPHYSIS (long shaft part)
245
Ewing Sarcoma sx
Extremities & pelvis
Worsening localized pain
WORSE AT NIGHT
246
Tx of Ewing
Chemo, surgery, radiation, or combo
247
Retinoblastoma
Mets rare
| death within 1 year is typical
248
Retinoblastoma presentation
Leukocoria: white pupillary reflex (most common sign)
Others: strabismus, nystagmus, red inflamed eye
249
Dx of Retinoblastoma
Chalky, off white retinal mass with soft, friable consistency = diagnostic
250
Should you do biopsy of Retinoblastoma?
NO!!
| Contraindicated d/t risk of Tumor Seeding
251
Tx for Retinoblastoma
Variety of options
Several "Vision sparing" options
External beam radiation
Chemo if confined to globe
252
Rhabdomyoscaroma
```
RARE
Any body part can be affected
Most common places for-
Younger children: head and neck
Teen: extremities
Infant: GU
```
253
Rhabdomyoscaroma sx
Painless enlarging mass
Orbital: exopth
Bladder: hematuria, urinary obstruction, pelvic mass
254
Dx of Rhabdo
X Ray/CT/MRI of mass
| Chest CT and Skeletal survery to r/u mets to bone or lungs
255
Tx for Rhabdo
Surgery
Chemo
Radiation
256
Hepatic tumors (liver)
2/3 liver masses are malignant!
257
hepatic tumors
Serum a-fetoprotein
| excellent marker for response to tx
258
Hepatic tumors
Enlarging abdomen
259
Hepatic tumors Tx
Surgery and chemo
Complete resection is essential for survival!!
liver transplant when tumors are unresectable
