Pedeiatrics III Flashcards
1
Q
MC presenting symptom in cancer and transplant patients.
A
Fever (Due to Inc. infx w Cancer and Cancer Tx)
2
Q
Malignant proliferation of leukocytes. MC Childhood cancer.
Fever, pallor, petechiae, lethargy, malaise, anorexia, bone or joint pain, LAD, Hepatosplenomegaly
A
Leukemia
Acute Lymphocytic Leukemia (ALL)
Acute Myelogenous Leukemia (AML)
3
Q
Leukemia lab findings
A
WBC > 50,000 Anemia Thrombocytopenia
Peripheral smear= immature blast cells
Lumbar puncture for CNS involvement.
4
Q
Leukemia Dx
A
Cell surface markers
t(12;21)- Translocation = MC favorable prognosis
t (9;22)- Translocation= Less common poor prognosis
4;11
5
Q
Tx-
A
Chemotherapy for ALL- 80% cure rate
(Not effective for AML- 50% cure rate)
Bone Marrow Transplant
6
Q
Leukemia Poor prognosis indicators
A
- < 1 YOA or > 10 YOA
- > 50,000 WBC
- CNS or testicular Dz Relapse AML= Very Poor
7
Q
3rd MC childhood malignancy (2nd CNS Tumors)
Epstein Barr Virus may play a role.
Fatigue, anorexia, pruritus, painless LAD (cervical Supra clavicular) pleural effusion
A
Lymphoma (Hodgkin’s or Non-Hodgkin’s)
8
Q
incidence Inc. w age. white>Black (M>F), almost always diffuse always malignant,
two forms and subtypes.
A
Non-Hodgkin’s Lymphoma
9
Q
Non-Hodgkin’s Lymphoma subtypes
A
B Cell (Burkitt non small non-cleaved cell)
Sporadic= North America Endemic= Africa (EBV*)
T Cell (Lymphoblastic)
Large Cell (B or T Cell origin)
10
Q
MC Lymphoma affecting adolescents/ young adults
and age >50 YOA
A
Hodgkin’s Lymphoma
11
Q
Specific symptoms of drenching night-sweats, weight-loss, >10% w/I 6 mos SOB mediastinal LAD
Poor prognosis
A
B Cell (Burkitt) NHL
12
Q
Lymphoma Dx
A
- CBC (Usually normal)
- Tissue Bx (Pleural/peritoneal) Reed Steinberg cells
Hodgkin’s Lymphoma - Bone Marrow (>25% blasts = Acute Leukemia)
13
Q
Lymphoma Tx
A
Hodgkin’s = Chemo (LD) and Radio Therapy
90% 5 year survival (Stage I and II)
NHL = Aggressive/Persistent Chemo
(Rare Sx and Radiation) 70-90% 3 yr survival
14
Q
MC solid tumor in children 2nd MC childhood malignancy. Loss of of dev. milestones. irritability, anorexia, poor school performance. ICP,
CN deficits except 6th CN = brainstem involvement
Pituitary-Prolactinoma= Galactorrhea
GH secreting= Precocious Puberty
A
CNS Tumors
15
Q
<2 YOA rapidly growing tumor, bad locale. vomiting, lethargy, irritability and ataxia.
Can also be slow growing w/ Macrocephaly, hyper reflexia, cranial nerve palsies, weight loss.
A
Acute or Chronic Infratentorial (65%)- Cerebellum and brainstem (35% cerebrum)
Chronic= slow growing etc.
16
Q
> 2 YOA Visual S/S, seizures (General or partial) focal neural deficits, personality changes.
May also be hydrocephalus and increased ICP (Papilledema) cranial nerve palsies and ataxia
A
Supratentorial (visual S/S and focal Deficits)
Infratentorial (ICP Papilledema)
17
Q
MC type of brain tumor: usually found in posterior fossa. Low Grade = good prognosis
A
Astrocytoma
18
Q
2nd MC Brain Tumor most often found in cerebellar vermis. High grade= variable prognosis
A
Medulloblastomas
19
Q
Dx of brain tumor work up
A
Neuro PE
CT and MRI
LP, X-ray and EEG (Never LP w/o imaging)
20
Q
MC childhood solid neoplasm outside CNS and MC infant malignancy @ 20 mos. From neuro crest cells
in adrenal medulla/symp. system (anywhere)
MC abd. mass (flank, hard, smooth, nontender) Abd. pain* w Horner’s syndrome neck apical masses. profuse sweating, secretory diarrhea, Opsomyoclonus (dancing eye/feet)
A
Neuroblastoma
21
Q
Neuroblastoma Dx
A
Tissue Bx and Bone marrow aspiration
90% catecholamines (Urine) (Vanillymandelic/homovanillic acid)
CT (chest, abd, Pelvis) Bone scan and X-ray (Calcifi)
22
Q
Neuroblastoma Tx:
A
Sx, Chemo, Radiation
5 year survival depends on stage. Favorable =<1 YOA
23
Q
MC Malignant renal tumor of childhood. assoc w/ Wilm’s tumor, Aniridia (Absent Iris), GU malformation, MR, Beckwith-Weidman Syndrome–> Increased risk.
Abdominal Mass, fever, HTN, Hematuria. Mean age 3-3.5 YOA: prognosis = Good Tx = Sx mainstay, chemo/rad
A
Nephroblastoma
24
Q
Beckwith-Wiedmann Syndrome clinical Present.
A
Macroglossia, Umbilical Hernia, Omphalocele.
–> Inc. risk of Nephroblastoma.
25
Pediatric types of Sarcomas
Rhabdomyosarcoma (Soft Tissue)
```
Osteosarcoma (Bone MC)
Ewing Sarcoma (Bone 2nd MC)
```
26
Syndromes Assoc. w Increased risk of Sarcomas?
Li-Fraumeni (Incr. Risk of Soft tissue)
Neurofibromatosis (Incr. soft tissue)
Hereditary retinoblastoma (Osteosarcoma)
Radiation therapy and Chemo alkylating agents
27
Presence of osteoid substance on w pain and mass at site (Epiphysis or metaphysis).
Distal Femur, proximal, tibia, proximal humerus) S/ initially associated to trauma, *Starburst pattern*
Tx: Neoadjuvant then Sx and adjuvant therapy
Osteosarcoma
28
MC soft tissue malignancy in children. Microscope- small, round, blue cell, tumors (Like Ewing)
S/S depend on GU, head and neck, Extremities.
Tx: Sx resection, postop chemo/radiation
Rhabdomyosarcoma (Skeletal muscle)
29
Under microscopy: small round cell tumors. Multi centric tumor causing pain and fever. In any bon
Femur and pelvis MC. *Starburst Pattern*, onion skin or moth eaten*
Tx: Sx resection, postop chemo/radiation (sensitive to)
Ewing Sarcoma
30
Immunohistochemical/cytogenic that differentiates Rhabdo from Ewing sarcoma
Ewing= t (11,22) (95% of tumors)
31
Rhabdomyosarcoma histological variants
Embryonal: young children Head/neck/GU
Alveolar: older pts, trunk, extremity tumors t (2;13)
32
Normal crying is defined as
1st 2 weeks = Little 10 episodes in 24 hrs
6 Weeks = 3 hrs/day
12 weeks = 1 hr /day
33
Defined as difficult or fussy child. Facia grimacing, leg flexion, flatus.
Wessel's rule of 3's ?
Colic
Crying: 3 hrs/day, at least 3/wk, > 3 wks
34
Colic Tx:
Increased carrying and swaddling
35
MC reported Behavioral problem. Screaming, stomping, hitting, head banging , falling down, breath holding, vomiting, biting. 1-3 yoa
short duration 2-5 min. non-manipulative
Tx; Parent reassurance, eliminate triggers, pos. reinforc
Temper tantrums
36
Special Needs goal of therapy?
Maximize child's potential for adult functioning
37
Cognitive performance (Intel testing) 2 standard deviations below the meand (<3rd %tile) IQ< 70
TORCH infx, ETOH/Rec. Rxs, hypoxia, bleeding.
DX: UA-aminoacids, Hyptothyroid, EEG, CHromosome
Mental Retardation D/O
38
Persistent deficit in social comms/Interaction. Restricted repetitive pattern of behavior, interest or activities. (Lining toys, flipping objects, echolali, idiosynchratic phases.
Inflexible to routines/ritual patterns (Likes sameness). Inc. risk w advanced parental age, LBW, Valproate fetal exposure. Hyper/hyporeactivity to sensory input
Autism Spectrum Disorder
39
Autism Spectrum Disorder Dx and Tx
Dx: Modified Checklist for Autism (MCHAT) 18-24 mos
Tx; Antipsychotics (aggression or agitation, self injury)
SSRIs Depression or anxiety
Special Ed and and Ped psych, Occupational Ther
40
A group of non progressive motor impairment synd. D2 Prema/Twins, LBW, asphyxia, Kernicterus
6 Types- Spastic=80% MC: Dev. delay w brisk DTRs
persistent infant reflexes Moro> 6 mos, Assymetric motor Activity dominant hand prior to 1 yoa, MR assoc.
ADHD assoc., seizures, Hearing/speech, GERD
Cerebral Palsy
Tx: Neurodevelopment and Multidisciplinary PE, Sx, Ed.
Botox and Muscle relax for spasticity
Sx varus osteotomy (Femur)
41
< 16 yoa w at least 6 S/S of inattention or hyperactivity/ impulsivity for at least 6 mos in 2 or more environments
60-80% S/S impulsivity inattention persist into adolescence and adulthood. Fails attention to detail, not listening, fail to follow through, loses things, easily distracted, forgetful
Attention Deficit Hyperactivity D/O
42
Fidgety or restless, leaving seat when expected to be seated, talks excessively, acts as if on the go, running
Climbing
Hyperactivity Predominant
43
Blurting out answers before question is complete. Difficulty waiting his/her turn.
Frequent interruptions or intrusion
Predominant Impulsivity.
44
Tx for ADHD
Methylphenidate or Amphetamine (1st Line)
(Sleep disturbance or Appetite Suppression
Atomoxetine (Strattera)- GI S/S
Guanfacine or Clonidine (sedation)
45
frequent/persistent pattern of anger and irritable mood, argumentative, vindictive
Oppositional Defiant d/o
46
Repetitive/persistent pattern of behavior in which basic rights of others or major rules violated.
Aggression to people or animals, property destruction, deceitfulness, serious violations of rules
Conduct Disorder
47
Sleep stages 1-3
Rapid Eye Movement Non-REM sleep
48
MC in preschool children: sleep walking, sleep terror, abrupt wakening w loud scream, agitation and unresponsiveness
resolves w time and developmental maturation
Parasomnias
49
Exaggerated delayed sleep phase--> inability to arouse in a.m. Does not meet sleep requirements.
problems w cognition and emotional regulations.
MC in adolescence: Tx- Hygiene and Melatonin/Clonidine
Circadian Rhythm D/O
50
MC in teenage girls. Weight loss, Lanugo, Bradycardia, Osteopenia, Amenorrhea, alopecia. Purging, dieting
Restricts caloric intake --> sig. weight loss. Refusal to maintain body weight or be at normal weight
Anorexia Nervosa
Tx: Restore weight, Bradycardia, hypothermia, hypokalemia, dysrrythmia. Risk of SA
51
Binge eating episodes large qty w rapid consumption. Normal to overweight. Inc. vomiting or laxatives.
Concerned w body image, enamel erosion at lingual aspect of teeth, incisors, scrapes on dorsum of hand
Bulimia Nervosa
Tx: Antidepressants, nutritional ed.
52
Female athlete triad consists of
Low Body fat--> hypothalamic-pituitary-Gonad system
Eating D/O- Anorexia or Bulimia
Osteoporosis- Low Bone Density
Menstrual Dysfunction- Delayed or absent
53
Female athlete triad treatment
Restore caloric intake Ca+,K, Vit. D supplement
OCP- restores menstruation
Decrease exercise
54
Minimum 2 weeks of depressed mood or loss of interest/ pleasure in nearly all activities.
5 or more s/s during the same 2 week period. Auicide surpasses MVAs cause of death in adolescents
Major Depressive D/O
Fluoxetine- Only drug approved for children
55
Recurrent episodes of persistent concern about having additional episodes >= 1 month.
Agoraphobia present. worry about the implications of the attack. Sweating, trembling, choking, palpitations, chest pain, nausea, dizzy,
Panic D/O
56
Excessive worry occurring more days than not for at least 6 months. X1 S/S required for children
Easily fatigued, blank mind, muscle tension, sleep disturbance. cause social or occupational dysfunx
Generalized Anxiety
57
1st degrees relatives 10X higher risk. Hallucinations or delusions (Pos. S/S) lack motivation or social interaction
>2 S/S > 6mosw 1 moth of acute S/S must include at least 1 of the following; hallucination, delusion or disorganized speech
Schizophrenia
| Brief psychotic = remission < 1 month
58
5 Types of Schizophrenia
Paranoid- Persecutory delusion or hallucination
Disorganized- in speech, behavior, flat or inappropriate
Catatonic- Extreme inactivity or excessive motor activity
undifferentiated- does not fit other
Residual- fits dx criteria but no current positive s/s
59
Substance abuse screening
Car- Driving under the indluence
Relax-substance use to relax, fit in, feel better
Alone-substance abuse while alone
Forgetting-as result of substance
Family/Friends- tell teen to cutdown
Trouble- getting in trouble due to substances
60
hematopoiesis
Liver @ 2 mos
Bone Marrow @ 5-6 mos
61
Lab Test Confirms and indicates severity of anemia
CBC: HgB HcT
62
Lab test for average RBC size microcytic, Normocytic, or Macrocytic.
Mean Corpuscular Volume
63
Measures variation in RBC size--> indication of RBC destruction or heterogenous population
Red Cell Distribution Width
64
Microcytic Anemias
Low Mean corpuscular Volume <79fL
```
Thalassemia
Anemia of Chronic Dz
Iron Deficiency
Lead Poisoning
Sideroblastic
```
65
MC cause of anemia WW. RF Cow's milk< 1 yoa. Menstruating teenagers. Lead poisoning, poor diet.
> 1 yoa, increased amounts of cow's milk.
Iron deficiency anemia
66
Iron deficiency anemia Tx:
otherwise healthy = Oral Iron (>6 mos Iron Supp.)
4-6 mg/day (HcT Inc. 1%/day: HgB Inc. 0.25-0.40 g/dL/day) Reticulocytosis improves 48-72 hrs
Re-eval if no response in 2 weeks
67
Hypochromic microcytic anemia and Basophilic stippling. concomitant Iron deficiency.
Child w pica home older than 1980. 20 Micrograms/dL on single visit or persistent 15 Micrograms/dL 3 mo period. (5-10 Micrograms may cause learning problems)
Lead poisoning
68
Lead Poisoning Tx:
Immediate removal from exposure
Chelation therapy
All children screening at 12 and 24 mos
69
Decreased RBC production the RDW test will be?
| Red Cell Distribution Width
Normal
Transient erythroblastopenia, Anemia of chronic dz, bone marrow failure.
70
An increased rate of RBC destruction will show a ____ RDW
High
G6PD Def., Sickle Cell anemia, Spherocytosis.
71
Reticulocyte-
serves as index for RBC production by the bone marrow:
```
Retic = Incr.--> Incr. Destruction
Retic = Decr.--> Hypoproliferation
```
72
Macrocytic Anemias
High Mean corpuscular Volume = >100fL
```
Folate Deficiency
Alcohol
Thyroid
B12 deficiency
Cirrhosis
```
73
Macrocytic anemias Dx and Tx
B12, Folate, TSH, LFTs
Tx: folate replacement (improves CBC in <1 wk
74
MC is idiopathic but caused by Felbamate and chloramphenicol. Toxins-Benzene
Platelets <20,000, Neutrophil count < 500/mm3, Bone marrow cellularity <25% of normal. Dx Bone marrow Bx
Tx: Hematopoietic stem cell transplantation
Immunosuppressive therapy
Aplastic Anemia
75
Microcephaly, absent thumbs, café au lait spots, horseshoe kidney, cutaneous hyperpigmentation
Before 10 yoa, form of Aplastic anemia. High CMV and Hgb F, all cell lines affected
Franconi Anemia
76
Fetal Hemoglobin (Intrauterine HgB)
HgB F (Alpha and Gamma)
77
Alpha and Beta HgB
Adult HgB A= Normal HgB Formed at 3rd Trimester only traces of Gamma by 6MOS
78
Alpha Thalassemia that leads to mild microcytic anemia. Mild dec. HgB and HcT, Mild Microcytosis
Incr. RBC number w normal reticulocyte count. R/O Iron deficiency. HgB electrophoresis usually normal
Alpha Thalassemia trait 2 gene Mutation
79
Alpha Thalassemia that presents w/ Microcytic Anemia, and mild hemolysis. Not transfusion dependent
Folate supp. and w/ occasional transfusion during crisis. High HgB H
Alpha Thalassemia (3 Gene Mutation HgB H Disease)
80
Alpha Thalassemia silent carrier. Completely asymptomatic. Normal CBC. Only detectable though genetic studies
Alpha Thalassemia (1 Gene Mutation)
81
Alpha Thalassemia w severe anemia, intrauterine anasarca from CHF, death in utero or at birth.
Alpha Thalassemia Bart/Hydrops Fetalis
82
Beta Thalassemia w moderate hemolysis, splenomegaly, moderately severe anemia: (Moderate Severity)
Beta Thalassemia Intermedia (B+/B+ or B+/Bo)
| ```
B+ = Reduced Beta Chain Synthesis
(Bo = Absent Beta Chain Synthesis)
```
83
Beta Thalassemia w severe hemolysis, ineffective erythropoiesis, transfusion dependent, hepatosplenomegaly
Frequent anemic crises requires regular transfusions by 2 MOS: HgB Alpha Gamma lack normal HgB A
Iron overload common (Hemochromatosis) 2 Gene mutations
Beta Thalassemia Major "Cooley's Anemia"
Bo/Bo
84
Beta Thalassemia w mild microcytic anemia. With 1 gene mutation. Mutation results in 1 absent or reduced chain
with one normal chain.
Beta Thalassemia Minor Trait
85
HgB S polymerizes--> RBC distortion. the distortion is predisposed by hypoxia, acidosis, Fever, hypothermia
Cannot move efficiently through capillaries--> thrombosis, sequestration, infarction. by 4 mos= fatal infx, spleen dysfx/infarction 2-4 yoa, Dactylitis foot swelling and priapism 6-20 y/o
Sickle Cell anemia
86
Sickle cell clinical presentation
Pain crisis- Vasoocclusive painful events
(Long bones Legs/Arms) femoral necrosis
Tx: fluids, analgesics (Narcotics/NSAIDS, O2,
87
Sickle Cell Chest presentation
Acute Chest Syndrome- crisis w/I lungs. CXR- infiltrate
Chest pain w/I hrs cough, hypoxia, Tachypnea, Resp. Distress. Dec. BS BL w dullness to percussion.
Tx: 02, fluids, analgesics, Abx, Bronchodilators, Transfusion, spirometry
88
Sickle cell Triggers.
Swim in cold water/ being in cold weather
Getting too hot or not enough rest.
ETOH consumption, dehydration, menstruation
89
Sickle cell functional asplenia # 1 cause of death
Sepsis Strep Pneumoniae
90
Sickle cell Aplastic crisis
Parvo virus B19 (fifth Dz) infects RBC precursor in bone marrow--> Anemia rapidly worsens
91
Sickle cell Acute management
Tx: fluids, analgesics (Narcotics/NSAIDS, O2, transfusion
92
Sickle cell Chronic management
Hydroxyurea- Inc. HgB F (Alpha2 Gamma2) Dec. event
Hematopoietic stem cell transplant- HLA matched
Prevent: PO PNC, vaccinations, Folate supp.
93
Hemoglobinuria, anemia, and jaundice 24-48 hr after ingestion of oxidizing compound. Fava beans, ASA, sulfa drugs, primaquine, nitrofurantoin, mothballs
Keeps Glutathione reduced--> free radicals --> damge RBC. Causes Heinz Bodies (RBC membrane fragile) bite cells
G6PD deficiency
94
G6PD Dx and Tx
Dx: Measurement of G6PD Enzyme activity and NADPH production. G6PD generates NADPH
NAPDH keeps Glutathione reduced (Nonoxidized)
Tx: Avoid exposure to triggers and supportive
95
caused by functional deficiency ATP depletion--> impairs RBC survival. (Na- K+ pump fails-> unstable)
Hemolysis through spleen removal. Jaundice and anemia, Tx: Neonates- transfusion, splenectomy
Pyruvate Kinase Deficiency
96
Thrombocytopenia <150,000, platelets >80,000 no bleeding risk, platelets < 20,000 = Bleeding risk
Nonblanching petechiae, purpura raised palpable, ecchymoses, Aberrant clotting --> DVT (warm swollen tender extremity) Arterial clots, Inc. PT PTT, Platelet fx abnormality
Hemostatic Disease
97
MC childhood bleeding D/O. 1-4 wks post viral infx. w abrupt petechiae, purpura, and epistaxis. Autoimmune
Splenic destruction of Ab coated platelets. Buccal mucosa petechiae, multiple bruises, bleeding gums, hematuria. Severe thrombocytopenia WBC RBC normal
PT and PTT normal
Immune Thrombocytopenic Purpura
98
Immune Thrombocytopenic Purpura
IVIG 1 G/Kg/d 1-2 ds
Prednisone 2-4 mg/kg/d X2 wks
IV Anti-D Rh + patients (R/O SLE or HIV)
Splenectomy= Definitive Tx
99
Cytoskeletal protein molecular defect in lymphocytes and platelets. small platelets Microscopy: X-linked
Hypogammaglobinemia, Eczema, Thrombocytopenia*
Tx Splenectomy improves thrombocytopenia
Wiskott-Aldrich Syndrome
100
Bleeding w minor trauma/ or into joints spontaneous. PTT prolonged, PT and bleeding time normal.
Factor VIII Low = ________ Factor IX Low= ________
*X-Linked* , Platelet aggregation normal.
```
Hemophilia A (Factor 8 Def,)
Hemophilia B (Factor 9 Def.)
```
101
```
Hemophilia A (Factor 8 Def,)
Hemophilia B (Factor 9 Def.)
```
Factor replacement Recombinant factor VII or IX
| Desmopressin (Hemophilia A Factor 8 Def ONLY
Inc. Factor 8 and Von-Willebrand F)
102
MC Congenital bleeding D/O Deficiency in factor causes bleeding gums, epistaxis, heavy menses, prolonged bleeding, mucocutaneous bleeding
Dx: Measure factors and function w Ristocetin*
X3 Types.
Von Willebrand Disease (Def vW factor)
Type 1- AD Dec. in vWF production
Type 2- Normal Prod. but defective vWF
Type 3- No production (Rare)
103
Von Willebrand Disease (Def vW factor) Tx:
Desmopressin Type 1 and 2
vWF concentrate for Type 3
104
MC childhood systemic vasculitis: before age 6 MC in winter, 50% follow URI. inflamed blood vessels.
Immune complexes predominantly IgA--> GNS: Palpable purpura buttocks, LE, urticaria--> purpura* lesions w ecchymosis. 80% Arthralgia*, Abd pain *, renal involved hematuria GNS, Calf edema, orchitis
Henoch-Schonlein Purpura
105
DX Henoch-Schonlein Purpura
2 of 4
Palpable purpura, Diffuse abd. pain (Bowel angina), Bx of vessels= IgA deposits at walls, < age 20
106
Henoch-Schonlein Purpura Tx:
Renal Fx Test
NSAIDS- Arthritis and Steroids (GI and Nephritis)
107
2nd MC childhood syst vasculitis. small-med artery size infl. w aneurysm. Prolonged acute febrile illness.
Conjunctivitis, cracked lips, strawberry tongue, scarlatina rash <24hrs post fever, skin desquamation, Cervical LAD, high fever, lasts 1-2 weeks,
Kawasaki Disease
108
Kawasaki Disease Tx:
IVIG coronary Aneurysm prevention
(Echo@ 2-3 wks then 6-8 wks for aneurysm)
Aspirin HD (Reye syndrome Low RF)
109
Headache red flags.
Worse HA of my life.
Moring HA- Tumor
Pain awakens child at night
ABn. Focal Neuro exam
110
MC recurrent pattern of primary HA in children/Adol.
global squeezing band like. hrs-days, no visual/Neuro
Children continue to function well. Normal neuro
Tension HA
111
Frontal, bitemporal, UniLat HA. Mod-Severe. Punding or throbbing. Aggravated by activity. Duration 72 hrs
N/V, Pallor, dizziness, photo/phono phobia. Aura lasts 15-30 min. seek dark quiet room, worse w movement
Migraine
112
Dx and Tx for HA
CT= Bleeding MRI = subtle abn.
Tx- Lifestyle mods, avoid triggers, NSAIDS/Tylenol 1st, Sumatriptan, Zofran, Phenergan, Reglan
Migraines prophylaxis- propranolol, TCAs, Valproate, CCBs SSRIs (Fluoxetine)
113
Arise from specific anatomic focus may not spread to surrounding brain regions.
tonic, clonic, myoclonic, sensory, psychic or autonomic abn. consciousness is preserved
Simple Partial Seizure
114
Arise from specific anatomic focus may not spread to surrounding brain regions.
tonic, clonic, myoclonic, sensory, psychic or autonomic abn. consciousness is altered. Staring/automatisms
Complex focal seizures
115
Generalized seizures
Tonic-clonic
Absence (Petit-Mal)
116
Age 5-15 yrs Develop short lived, blank stare. May involve eye movements or lip smacking.
No-post-ictal state. EEG 3Hz spike/dome wave
Tx: Ethosuximide or valproic acid
Absence (Petit-Mal)
117
Sustained/rhythmic contractions. spastic symmetrical shaking. Gradual return of consciousness.
Post-ictal state. EEG normal when not seizing- Myoclonic-rapid shock like contraction
Tonic-Clonic Seizure (tonic=sustained contraction)
| Clonic= rhythmic contractions
118
Neurologic emergency ongoing seizure activity or repetitive seizures w/o return of conscious for >30 min
Tx Airway, ECG, IV, Pulse ox, Glucose, BMP, Tox screen, Lorazepam/Diazepam/Mida. R/O Menin/Enceph-itis if febrile
Status Epilepticus
119
MC cause of seizures ages 6 mos- 6 yrs. Fever= 102+
recurring temperature w incr. rate of rise. last <15 min.
No inc. risk of epilepsy. Typically does not recur in 24 hrs (neurologic= occurs>1) Tx- ABCs, Antipyretics.
Febrile Seizures
120
Disease/ Disorder Affects Anterior Horn Cells
Spinal Motor atrophy or Polio
121
Disease/ Disorder Affects Peripheral nerves
Guillain-Barre syndrome Charcot-Marie-Tooth
or Tick Paralysis
122
Disease/ Disorder Affects neuromuscular Junction
Myasthenia Gravis or Botulism
123
Disease/ Disorder Affects muscles
Muscular Dystrophies
124
Is this upper or lower motor neuron UMN or LMN
Tone-Decreased Reflexes Decreased
Babinski Reflex- absent Atrophy- possible
Fasciculations-Possible
Lower Motor Neuron
125
Is this upper or lower motor neuron UMN or LMN
Tone-Increased Reflexes Increased
Babinski Reflex- present Atrophy- possible
Fasciculations- Absent
Upper Motor Neuron UMN
126
autosomal recessive, progressive proximal weakness/atrophy. normal cognitive and sensation.
Dec. spontaneous movements, floppiness (reflex-absent) fasciculation of tongue (Obs-sleep)
Wernig Hoffman- die in 1st 2 yoa Kugleberg-welander 50% survive to adulthood
Spinal Motor Atrophy
127
Viral infections destroys anterior horn cell. Fecal oral transmission. fever, malaise, HA, URI, myalgias, NVD,
followed by asymmetric flaccid paralysis after >50% neurons destroyed for that muscle. Vaccine
Poliomyelitis
128
post infectious Acute inflammatory demyelinating polyradiculoneuropathy. after URI or GI Infx
Mycoplasma Pneumo or campylobacter Jejuni. Areflex flaccidity, symmetrical ascending weakness hrs or wks
paresthesia to hand/feet--> heaviness leg weakness
Guillain-Barre Syndrome
Tx- Admit, resp. support, IVIG or plasmaphoresis
129
Genetic chronic progressive peripheral polyneuropathy. Weakness begins distal legs-->hands --->forearms. pre-school years. reduced or absent DTR
Pes cavus (High arch), Bilat weakness@ dorsiflex, normal sensation c/o paresthesia occasionally
Charcot-Marie Tooth
Tx- foot bracing and PT
130
Causes interference w nerve impulse transx in the peripheral nerves.
Rapid ascending flaccid general paralysis over 1-2 days
Tx- complete resolution post removal of trigger bug
Tick Paralysis
131
Autoimmune Ab blocking acetylcholine Rec. @ NMJ adolescent or in neonates if mother carrier.
Ptosis, diplopia, weakness to extremities, neck or jaw. minimal s/s in a.m progressing through day
Dx confirmed by Acetylcholinesterase Tensilon edrophonium test. Tx pyridogstigmine
Myasthenia Gravis
132
seen MC in 2-3 month old, constipation, poor suck, lethargy, eyelid lag, facial weakness, dysphagia
nasal speech, difficulty respirations and limb weakness (late findings) Tx: Antitoxin and supportive care
Botulism (No honey or canned foods in < 1 yoa)
133
Muscle weakness due to D2 dystrophin mutation Delay in motor milestones onset 2-6 yoa, awkward gait. Wheel chair bound by 16yoa. X-linked
Proximal muscles of pelvic then shoulder girdle. clumsiness and easy fatigued--> muscle weakness (Gowers sign) Pseudohypertrophy of calves
Duchenne Muscular Dystrophy (Becker's Milder)
134
Severe neonate hypotonia and feed problems--> FTT
small hands/feet, small penis/testes, cryptorchidism.
Hyperphagia and obesity develop.
Prader-Willi Syndrome
135
rapid onset TBI w transient neuro impairment that resolves spontaneously. HA MC S/S 72 hrs (7 day= benign)
Slow reaction times, confusion, loss of concentration, disoriented, foggy thinking. Anxiety/depression irritable, sleep disturbances, amnesia
Concussion (RF artificial Turf/Football/Rugby)
136
Acute sometimes fatal brain swelling occurring after a second concussion is sustained prior to 1st recovery
Rapid Inc. ICP impossible to control.
Concussion 2nd Impact Syndrome
137
RF to develop include young age, level of play, ongoing s/s, chronic neurobehavior--> subdural hema.
hallucinations, dizziness, HA, Unclear thinking, sleep disturbance, lasts days - weeks, affects school/capab
Post- concussive Syndrome
138
Onset of blee min. - hours. Lucid interval followed by progressive neurologic deficits. CT= Lens shape
Prognosis = good w prompt Tx, otherwise poor
Epidural Hemorrhage
139
onset occurs over hours focal neurologic deficits.
CT= Crescent
extracranial hemorrhage compresses brain
Acute subdural Hemorrhage
140
Onset over weeks to months. Anemia, macrocephaly, seizures, vomiting. CT= Low density Crescent
Chronic Subdural Hemorrhage
141
Depressed consciousness, focal neurologic deficits.
+- additional contusions Tx supportive
Intraparenchymal
142
Stiff neck, worst HA of life. Late hydrocephalus
Subarachnoid Hemorrhage
143
Focal neuro deficits, brain swelling with trans-tentorial herniation, CT= low density w punctate hemorrhages
Tx for ICP
Brain contusion
144
fracture of skull that spreads a suture. no treatment necessary. Nuero and CT normal = send home
or observe overnight in hospital.
Linear or Diastatic
145
Fx: surgical elevation required if >5-10 mm deep
Depressed fx
146
Fx Necessitates emergent Sx debridement and tetanus prohylaxis
Compound Fx
147
Fx S/S Epistaxis, hemotympanum, Battle's sign, Cranial Nerve palsies.
Basilar Skull Fx
148
AD mutation on chromo 17 (50% maybe spontaneous)
café au lait spots, axillary or inguinal freckling. >5mm >x6, 2+ Lisch nodules- Iris hemrtomas 90% of type D/O
FHX of D/O, osseus lesions, 2+ Neurofibromas, opticglioma: Life span dec. X 15 yrs-Malignancy MC
Neurofibromatosis type 1
149
AD mutation on chromo 22-Tumor suppressant gene. 1/2 w no FHX. Intracranial/spinal tumors, CN gliomas schwannomas.
Posterior cortical cataracts, plaque like lesions. Sub-q nodules and cutaneous schwannomas.
Neurofibromatosis type 2 NF2
150
AD characterized by hamartomas in brain, eye, skin, kidney and heart. mutation of chromo 9 hamartin: 21 tuberin.
Possible MR w seizures. cardinal feature- facial angiofibroma, MR, epilepsy. *Hypomelanotic macules (Ash-leaf spots), Lumbosacral plaque- Shagreen patch
* Renal angiomyolipomas- hematuria--> death
Tuberous sclerosis
151
Comprised of abnormal blood vessels. Port Wine stain of upper face must include forehead and upper eye
Choroidal/Venous angioma. MC s/s seizure 75% ischemia to brain, hemiparesis, stroke like episodes, HA, MR and learning disabilities
Sturge Webber Syndrome
152
Sturge Webber Syndrome Tx:
Laser sx (Cosmetic) anticonvulsants, Ophthalmology, Hemispherectomy
153
Type I cerebellar tonsils protrude down through foramen magnum into spinal canal.
A-s/s @ 1s-- ataxia and vertigo in children. Obstruction--> hydrocephalus
Arnold Chiari Malformation Type I
154
Type I cerebellar tonsils protrude down through foramen magnum into spinal canal.
A-s/s @ 1s-- ataxia and vertigo in children. Obstruction--> hydrocephalus + occipital encephalocele
Arnold Chiari Malformation Type III
155
Type I cerebellar tonsils protrude down through foramen magnum into spinal canal.
A-s/s @ 1s-- ataxia and vertigo in children. Obstruction--> hydrocephalus + lumbar meningomyocele
Arnold Chiari Malformation Type II
156
Premature cranial suture closure Tx- Elective Sx @ 6 MOS
Craniosynostosis
157
Inc volume in CSF. communicating (w Subarachnoid) non-communicating (Obstructed)
Macrocephaly, irritability, vomiting, anorexia, papilledema, setting sun gaze , bulging fontanel
Hydrocephalus
Tx: Ventriculoperitoneal shunt
158
MC form of child abuse which fails to provide for the needs of the child - hardest to document
Clothing, food, housing, love, compassion, bonding or medical care
Neglect
159
Caregiver reports factitious S/S in child
Munchausen Syndrome by proxy
160
various stages of healing bruises, torso ears, neck. whipped items, hand/bite marks. restraints marks
immersion or circumferential or cigarette burns. Multiple concurrent fxs
Physical Abuse
161
Fractures that suggest abuse
Spiral, bucket handle (Metaphyseal), scapular, rib, spinous process/vertebrae, skull , sternal
162
shaken baby syndrome triad
Retinal Hemorrhaging
Brain swelling
Subdural Hematoma
163
Recurrent UTIS, STDs, precocious sexual behavior (hypersexual) guilt and low self esteem.
Night terrors, nightmares, social isolation, promiscuity, sudden schoold difficulties. avoids physical contact.
Sexual Abuse (W/I 72 hrs Sex assault kit)
164
Primary cause of Cardiopulmonary arrest in children
Respiratory arrest
165
MC common type of shock in children
Hypovolemic (Hemorrhage, Diarrhea, renal fluid,)
166
MC cause of distributive shock
Sepsis (venous pooling/Vasodilation) then Anaphylaxis- CNS
167
Causes of obstructive shock
Cardiac Tamponade, massive PE, Tension pneumo, cardiac tumor
168
Cardiogenic shock
Congenital HD, Arrythmia, Myocarditis drug intox, Kawasaki dz
169
Dissociative shock
Carbon Monoxide poisoning, Methemoglobinemia
170
MC types of trauma injuries in pediatrics
Head injuries and limbs
171
if you suspect spinal cord injury w/o radiological abnormality (SCIWORA) then conduct a
MRI
172
2nd leading cause of trauma death in pediatrics
Thoracic trauma (Post rib Fxs= abuse)
173
3rd Leading cause of trauma death in peds
Abdominal ( CT)
174
Most frequently injured abdominal organ in children
spleen (Hemodynamic stable = no Sx)
175
LUQ direct pressure causes L shoulder pain suspicion for splenic injury w LUQ abrasion, ecchymosis, TTP
Is known as ____ sign
Kehr Sign
176
NV, abd. pain, high amylase lipase, bicycle handle bar injury or other trauma
Pancreatic injury
177
Epiphyseal Fxs MC sites
#1 Distal Radius, #2 Distal Tibia, #3 distal Fibula
SALTR
178
Force is insufficient to cause a complete fx. sustains a bend deformity on the compression side
Greenstick Fx
179
Compression of the bone, occurs at Metaphysis. Bony cortex does not truly break. fall on to outstretch hand
Buckle or Torus Fx
