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Phase II Pediatrics > Pedeiatrics III > Flashcards

Pedeiatrics III Flashcards

1
Q

MC presenting symptom in cancer and transplant patients.

A

Fever (Due to Inc. infx w Cancer and Cancer Tx)

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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)

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3
Q

Leukemia lab findings

A

WBC > 50,000 Anemia Thrombocytopenia

Peripheral smear= immature blast cells

Lumbar puncture for CNS involvement.

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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

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5
Q

Tx-

A

Chemotherapy for ALL- 80% cure rate
(Not effective for AML- 50% cure rate)
Bone Marrow Transplant

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6
Q

Leukemia Poor prognosis indicators

A
  • < 1 YOA or > 10 YOA
  • > 50,000 WBC
  • CNS or testicular Dz Relapse AML= Very Poor
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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)

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8
Q

incidence Inc. w age. white>Black (M>F), almost always diffuse always malignant,

two forms and subtypes.

A

Non-Hodgkin’s Lymphoma

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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)

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10
Q

MC Lymphoma affecting adolescents/ young adults

and age >50 YOA

A

Hodgkin’s Lymphoma

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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

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12
Q

Lymphoma Dx

A
  • CBC (Usually normal)
  • Tissue Bx (Pleural/peritoneal) Reed Steinberg cells
    Hodgkin’s Lymphoma
  • Bone Marrow (>25% blasts = Acute Leukemia)
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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

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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

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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.

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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)

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17
Q

MC type of brain tumor: usually found in posterior fossa. Low Grade = good prognosis

A

Astrocytoma

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18
Q

2nd MC Brain Tumor most often found in cerebellar vermis. High grade= variable prognosis

A

Medulloblastomas

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19
Q

Dx of brain tumor work up

A

Neuro PE

CT and MRI

LP, X-ray and EEG (Never LP w/o imaging)

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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

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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)

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22
Q

Neuroblastoma Tx:

A

Sx, Chemo, Radiation

5 year survival depends on stage. Favorable =<1 YOA

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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

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24
Q

Beckwith-Wiedmann Syndrome clinical Present.

A

Macroglossia, Umbilical Hernia, Omphalocele.

–> Inc. risk of Nephroblastoma.

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25
Q

Pediatric types of Sarcomas

A

Rhabdomyosarcoma (Soft Tissue)

Osteosarcoma (Bone MC)
Ewing Sarcoma (Bone 2nd MC)
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26
Q

Syndromes Assoc. w Increased risk of Sarcomas?

A

Li-Fraumeni (Incr. Risk of Soft tissue)

Neurofibromatosis (Incr. soft tissue)

Hereditary retinoblastoma (Osteosarcoma)

Radiation therapy and Chemo alkylating agents

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27
Q

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

A

Osteosarcoma

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28
Q

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

A

Rhabdomyosarcoma (Skeletal muscle)

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29
Q

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)

A

Ewing Sarcoma

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30
Q

Immunohistochemical/cytogenic that differentiates Rhabdo from Ewing sarcoma

A

Ewing= t (11,22) (95% of tumors)

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31
Q

Rhabdomyosarcoma histological variants

A

Embryonal: young children Head/neck/GU

Alveolar: older pts, trunk, extremity tumors t (2;13)

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32
Q

Normal crying is defined as

A

1st 2 weeks = Little 10 episodes in 24 hrs
6 Weeks = 3 hrs/day
12 weeks = 1 hr /day

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33
Q

Defined as difficult or fussy child. Facia grimacing, leg flexion, flatus.

Wessel’s rule of 3’s ?

A

Colic

Crying: 3 hrs/day, at least 3/wk, > 3 wks

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34
Q

Colic Tx:

A

Increased carrying and swaddling

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35
Q

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

A

Temper tantrums

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36
Q

Special Needs goal of therapy?

A

Maximize child’s potential for adult functioning

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37
Q

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

A

Mental Retardation D/O

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38
Q

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

A

Autism Spectrum Disorder

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39
Q

Autism Spectrum Disorder Dx and Tx

A

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

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40
Q

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

A

Cerebral Palsy

Tx: Neurodevelopment and Multidisciplinary PE, Sx, Ed.
Botox and Muscle relax for spasticity
Sx varus osteotomy (Femur)

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41
Q

< 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

A

Attention Deficit Hyperactivity D/O

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42
Q

Fidgety or restless, leaving seat when expected to be seated, talks excessively, acts as if on the go, running

Climbing

A

Hyperactivity Predominant

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43
Q

Blurting out answers before question is complete. Difficulty waiting his/her turn.

Frequent interruptions or intrusion

A

Predominant Impulsivity.

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44
Q

Tx for ADHD

A

Methylphenidate or Amphetamine (1st Line)
(Sleep disturbance or Appetite Suppression

Atomoxetine (Strattera)- GI S/S

Guanfacine or Clonidine (sedation)

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45
Q

frequent/persistent pattern of anger and irritable mood, argumentative, vindictive

A

Oppositional Defiant d/o

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46
Q

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

A

Conduct Disorder

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47
Q

Sleep stages 1-3

A

Rapid Eye Movement Non-REM sleep

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48
Q

MC in preschool children: sleep walking, sleep terror, abrupt wakening w loud scream, agitation and unresponsiveness

resolves w time and developmental maturation

A

Parasomnias

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49
Q

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

A

Circadian Rhythm D/O

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50
Q

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

A

Anorexia Nervosa

Tx: Restore weight, Bradycardia, hypothermia, hypokalemia, dysrrythmia. Risk of SA

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51
Q

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

A

Bulimia Nervosa

Tx: Antidepressants, nutritional ed.

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52
Q

Female athlete triad consists of

Low Body fat–> hypothalamic-pituitary-Gonad system

A

Eating D/O- Anorexia or Bulimia

Osteoporosis- Low Bone Density

Menstrual Dysfunction- Delayed or absent

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53
Q

Female athlete triad treatment

A

Restore caloric intake Ca+,K, Vit. D supplement

OCP- restores menstruation

Decrease exercise

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54
Q

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

A

Major Depressive D/O

Fluoxetine- Only drug approved for children

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55
Q

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,

A

Panic D/O

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56
Q

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

A

Generalized Anxiety

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57
Q

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

A

Schizophrenia

Brief psychotic = remission < 1 month

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58
Q

5 Types of Schizophrenia

A

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

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59
Q

Substance abuse screening

A

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

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60
Q

hematopoiesis

A

Liver @ 2 mos

Bone Marrow @ 5-6 mos

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61
Q

Lab Test Confirms and indicates severity of anemia

A

CBC: HgB HcT

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62
Q

Lab test for average RBC size microcytic, Normocytic, or Macrocytic.

A

Mean Corpuscular Volume

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63
Q

Measures variation in RBC size–> indication of RBC destruction or heterogenous population

A

Red Cell Distribution Width

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64
Q

Microcytic Anemias

A

Low Mean corpuscular Volume <79fL

Thalassemia
Anemia of Chronic Dz
Iron Deficiency
Lead Poisoning
Sideroblastic
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65
Q

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.

A

Iron deficiency anemia

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66
Q

Iron deficiency anemia Tx:

A

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

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67
Q

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)

A

Lead poisoning

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68
Q

Lead Poisoning Tx:

A

Immediate removal from exposure
Chelation therapy

All children screening at 12 and 24 mos

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69
Q

Decreased RBC production the RDW test will be?

Red Cell Distribution Width

A

Normal

Transient erythroblastopenia, Anemia of chronic dz, bone marrow failure.

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70
Q

An increased rate of RBC destruction will show a ____ RDW

A

High

G6PD Def., Sickle Cell anemia, Spherocytosis.

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71
Q

Reticulocyte-

A

serves as index for RBC production by the bone marrow:

Retic = Incr.--> Incr. Destruction
Retic = Decr.--> Hypoproliferation
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72
Q

Macrocytic Anemias

A

High Mean corpuscular Volume = >100fL

Folate Deficiency
Alcohol
Thyroid
B12 deficiency
Cirrhosis
73
Q

Macrocytic anemias Dx and Tx

A

B12, Folate, TSH, LFTs

Tx: folate replacement (improves CBC in <1 wk

74
Q

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

A

Aplastic Anemia

75
Q

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

A

Franconi Anemia

76
Q

Fetal Hemoglobin (Intrauterine HgB)

A

HgB F (Alpha and Gamma)

77
Q

Alpha and Beta HgB

A

Adult HgB A= Normal HgB Formed at 3rd Trimester only traces of Gamma by 6MOS

78
Q

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

A

Alpha Thalassemia trait 2 gene Mutation

79
Q

Alpha Thalassemia that presents w/ Microcytic Anemia, and mild hemolysis. Not transfusion dependent

Folate supp. and w/ occasional transfusion during crisis. High HgB H

A

Alpha Thalassemia (3 Gene Mutation HgB H Disease)

80
Q

Alpha Thalassemia silent carrier. Completely asymptomatic. Normal CBC. Only detectable though genetic studies

A

Alpha Thalassemia (1 Gene Mutation)

81
Q

Alpha Thalassemia w severe anemia, intrauterine anasarca from CHF, death in utero or at birth.

A

Alpha Thalassemia Bart/Hydrops Fetalis

82
Q

Beta Thalassemia w moderate hemolysis, splenomegaly, moderately severe anemia: (Moderate Severity)

A

Beta Thalassemia Intermedia (B+/B+ or B+/Bo)

```
B+ = Reduced Beta Chain Synthesis
(Bo = Absent Beta Chain Synthesis)
~~~

83
Q

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

A

Beta Thalassemia Major “Cooley’s Anemia”

Bo/Bo

84
Q

Beta Thalassemia w mild microcytic anemia. With 1 gene mutation. Mutation results in 1 absent or reduced chain

with one normal chain.

A

Beta Thalassemia Minor Trait

85
Q

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

A

Sickle Cell anemia

86
Q

Sickle cell clinical presentation

A

Pain crisis- Vasoocclusive painful events
(Long bones Legs/Arms) femoral necrosis

Tx: fluids, analgesics (Narcotics/NSAIDS, O2,

87
Q

Sickle Cell Chest presentation

A

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
Q

Sickle cell Triggers.

A

Swim in cold water/ being in cold weather

Getting too hot or not enough rest.

ETOH consumption, dehydration, menstruation

89
Q

Sickle cell functional asplenia # 1 cause of death

A

Sepsis Strep Pneumoniae

90
Q

Sickle cell Aplastic crisis

A

Parvo virus B19 (fifth Dz) infects RBC precursor in bone marrow–> Anemia rapidly worsens

91
Q

Sickle cell Acute management

A

Tx: fluids, analgesics (Narcotics/NSAIDS, O2, transfusion

92
Q

Sickle cell Chronic management

A

Hydroxyurea- Inc. HgB F (Alpha2 Gamma2) Dec. event

Hematopoietic stem cell transplant- HLA matched

Prevent: PO PNC, vaccinations, Folate supp.

93
Q

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

A

G6PD deficiency

94
Q

G6PD Dx and Tx

A

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
Q

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

A

Pyruvate Kinase Deficiency

96
Q

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

A

Hemostatic Disease

97
Q

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

A

Immune Thrombocytopenic Purpura

98
Q

Immune Thrombocytopenic Purpura

A

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
Q

Cytoskeletal protein molecular defect in lymphocytes and platelets. small platelets Microscopy: X-linked

Hypogammaglobinemia, Eczema, Thrombocytopenia*
Tx Splenectomy improves thrombocytopenia

A

Wiskott-Aldrich Syndrome

100
Q

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.

A 
Hemophilia A (Factor 8 Def,)
Hemophilia B (Factor 9 Def.)
101
Q 
Hemophilia A (Factor 8 Def,)
Hemophilia B (Factor 9 Def.)
A

Factor replacement Recombinant factor VII or IX

Desmopressin (Hemophilia A Factor 8 Def ONLY
Inc. Factor 8 and Von-Willebrand F)

102
Q

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.

A

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
Q

Von Willebrand Disease (Def vW factor) Tx:

A

Desmopressin Type 1 and 2

vWF concentrate for Type 3

104
Q

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

A

Henoch-Schonlein Purpura

105
Q

DX Henoch-Schonlein Purpura

A

2 of 4

Palpable purpura, Diffuse abd. pain (Bowel angina), Bx of vessels= IgA deposits at walls, < age 20

106
Q

Henoch-Schonlein Purpura Tx:

A

Renal Fx Test

NSAIDS- Arthritis and Steroids (GI and Nephritis)

107
Q

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,

A

Kawasaki Disease

108
Q

Kawasaki Disease Tx:

A

IVIG coronary Aneurysm prevention
(Echo@ 2-3 wks then 6-8 wks for aneurysm)

Aspirin HD (Reye syndrome Low RF)

109
Q

Headache red flags.

A

Worse HA of my life.

Moring HA- Tumor

Pain awakens child at night

ABn. Focal Neuro exam

110
Q

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

A

Tension HA

111
Q

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

A

Migraine

112
Q

Dx and Tx for HA

A

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
Q

Arise from specific anatomic focus may not spread to surrounding brain regions.

tonic, clonic, myoclonic, sensory, psychic or autonomic abn. consciousness is preserved

A

Simple Partial Seizure

114
Q

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

A

Complex focal seizures

115
Q

Generalized seizures

A

Tonic-clonic

Absence (Petit-Mal)

116
Q

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

A

Absence (Petit-Mal)

117
Q

Sustained/rhythmic contractions. spastic symmetrical shaking. Gradual return of consciousness.

Post-ictal state. EEG normal when not seizing- Myoclonic-rapid shock like contraction

A

Tonic-Clonic Seizure (tonic=sustained contraction)

Clonic= rhythmic contractions

118
Q

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

A

Status Epilepticus

119
Q

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.

A

Febrile Seizures

120
Q

Disease/ Disorder Affects Anterior Horn Cells

A

Spinal Motor atrophy or Polio

121
Q

Disease/ Disorder Affects Peripheral nerves

A

Guillain-Barre syndrome Charcot-Marie-Tooth

or Tick Paralysis

122
Q

Disease/ Disorder Affects neuromuscular Junction

A

Myasthenia Gravis or Botulism

123
Q

Disease/ Disorder Affects muscles

A

Muscular Dystrophies

124
Q

Is this upper or lower motor neuron UMN or LMN

Tone-Decreased Reflexes Decreased
Babinski Reflex- absent Atrophy- possible
Fasciculations-Possible

A

Lower Motor Neuron

125
Q

Is this upper or lower motor neuron UMN or LMN

Tone-Increased Reflexes Increased
Babinski Reflex- present Atrophy- possible
Fasciculations- Absent

A

Upper Motor Neuron UMN

126
Q

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

A

Spinal Motor Atrophy

127
Q

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

A

Poliomyelitis

128
Q

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

A

Guillain-Barre Syndrome

Tx- Admit, resp. support, IVIG or plasmaphoresis

129
Q

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

A

Charcot-Marie Tooth

Tx- foot bracing and PT

130
Q

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

A

Tick Paralysis

131
Q

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

A

Myasthenia Gravis

132
Q

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

A

Botulism (No honey or canned foods in < 1 yoa)

133
Q

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

A

Duchenne Muscular Dystrophy (Becker’s Milder)

134
Q

Severe neonate hypotonia and feed problems–> FTT

small hands/feet, small penis/testes, cryptorchidism.
Hyperphagia and obesity develop.

A

Prader-Willi Syndrome

135
Q

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

A

Concussion (RF artificial Turf/Football/Rugby)

136
Q

Acute sometimes fatal brain swelling occurring after a second concussion is sustained prior to 1st recovery

Rapid Inc. ICP impossible to control.

A

Concussion 2nd Impact Syndrome

137
Q

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

A

Post- concussive Syndrome

138
Q

Onset of blee min. - hours. Lucid interval followed by progressive neurologic deficits. CT= Lens shape

Prognosis = good w prompt Tx, otherwise poor

A

Epidural Hemorrhage

139
Q

onset occurs over hours focal neurologic deficits.
CT= Crescent

extracranial hemorrhage compresses brain

A

Acute subdural Hemorrhage

140
Q

Onset over weeks to months. Anemia, macrocephaly, seizures, vomiting. CT= Low density Crescent

A

Chronic Subdural Hemorrhage

141
Q

Depressed consciousness, focal neurologic deficits.

+- additional contusions Tx supportive

A

Intraparenchymal

142
Q

Stiff neck, worst HA of life. Late hydrocephalus

A

Subarachnoid Hemorrhage

143
Q

Focal neuro deficits, brain swelling with trans-tentorial herniation, CT= low density w punctate hemorrhages

Tx for ICP

A

Brain contusion

144
Q

fracture of skull that spreads a suture. no treatment necessary. Nuero and CT normal = send home

or observe overnight in hospital.

A

Linear or Diastatic

145
Q

Fx: surgical elevation required if >5-10 mm deep

A

Depressed fx

146
Q

Fx Necessitates emergent Sx debridement and tetanus prohylaxis

A

Compound Fx

147
Q

Fx S/S Epistaxis, hemotympanum, Battle’s sign, Cranial Nerve palsies.

A

Basilar Skull Fx

148
Q

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

A

Neurofibromatosis type 1

149
Q

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.

A

Neurofibromatosis type 2 NF2

150
Q

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

A

Tuberous sclerosis

151
Q

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

A

Sturge Webber Syndrome

152
Q

Sturge Webber Syndrome Tx:

A

Laser sx (Cosmetic) anticonvulsants, Ophthalmology, Hemispherectomy

153
Q

Type I cerebellar tonsils protrude down through foramen magnum into spinal canal.

A-s/s @ 1s– ataxia and vertigo in children. Obstruction–> hydrocephalus

A

Arnold Chiari Malformation Type I

154
Q

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

A

Arnold Chiari Malformation Type III

155
Q

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

A

Arnold Chiari Malformation Type II

156
Q

Premature cranial suture closure Tx- Elective Sx @ 6 MOS

A

Craniosynostosis

157
Q

Inc volume in CSF. communicating (w Subarachnoid) non-communicating (Obstructed)

Macrocephaly, irritability, vomiting, anorexia, papilledema, setting sun gaze , bulging fontanel

A

Hydrocephalus

Tx: Ventriculoperitoneal shunt

158
Q

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

A

Neglect

159
Q

Caregiver reports factitious S/S in child

A

Munchausen Syndrome by proxy

160
Q

various stages of healing bruises, torso ears, neck. whipped items, hand/bite marks. restraints marks

immersion or circumferential or cigarette burns. Multiple concurrent fxs

A

Physical Abuse

161
Q

Fractures that suggest abuse

A

Spiral, bucket handle (Metaphyseal), scapular, rib, spinous process/vertebrae, skull , sternal

162
Q

shaken baby syndrome triad

A

Retinal Hemorrhaging
Brain swelling
Subdural Hematoma

163
Q

Recurrent UTIS, STDs, precocious sexual behavior (hypersexual) guilt and low self esteem.

Night terrors, nightmares, social isolation, promiscuity, sudden schoold difficulties. avoids physical contact.

A

Sexual Abuse (W/I 72 hrs Sex assault kit)

164
Q

Primary cause of Cardiopulmonary arrest in children

A

Respiratory arrest

165
Q

MC common type of shock in children

A

Hypovolemic (Hemorrhage, Diarrhea, renal fluid,)

166
Q

MC cause of distributive shock

A

Sepsis (venous pooling/Vasodilation) then Anaphylaxis- CNS

167
Q

Causes of obstructive shock

A

Cardiac Tamponade, massive PE, Tension pneumo, cardiac tumor

168
Q

Cardiogenic shock

A

Congenital HD, Arrythmia, Myocarditis drug intox, Kawasaki dz

169
Q

Dissociative shock

A

Carbon Monoxide poisoning, Methemoglobinemia

170
Q

MC types of trauma injuries in pediatrics

A

Head injuries and limbs

171
Q

if you suspect spinal cord injury w/o radiological abnormality (SCIWORA) then conduct a

A

MRI

172
Q

2nd leading cause of trauma death in pediatrics

A

Thoracic trauma (Post rib Fxs= abuse)

173
Q

3rd Leading cause of trauma death in peds

A

Abdominal ( CT)

174
Q

Most frequently injured abdominal organ in children

A

spleen (Hemodynamic stable = no Sx)

175
Q

LUQ direct pressure causes L shoulder pain suspicion for splenic injury w LUQ abrasion, ecchymosis, TTP

Is known as ____ sign

A

Kehr Sign

176
Q

NV, abd. pain, high amylase lipase, bicycle handle bar injury or other trauma

A

Pancreatic injury

177
Q

Epiphyseal Fxs MC sites

A

1 Distal Radius, #2 Distal Tibia, #3 distal Fibula

SALTR

178
Q

Force is insufficient to cause a complete fx. sustains a bend deformity on the compression side

A

Greenstick Fx

179
Q

Compression of the bone, occurs at Metaphysis. Bony cortex does not truly break. fall on to outstretch hand

A

Buckle or Torus Fx

Phase II Pediatrics (3 decks)

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