Addendum: Pediatrics Review - Topnotch Medical Board Prep

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Transcript Addendum: Pediatrics Review - Topnotch Medical Board Prep

Addendum: Pediatrics Review
WHO 2009 Classification
• Dengue without warning signs:
 Live in/travel to endemic areas
 Fever and 2 of the ff criteria:
1. Nausea, vomiting
2. Rash
3. Aches & pains
4. Tourniquet test positive
5. Leukopenia
WHO 2009 Classification
• Dengue with warning signs:
1. Abdominal pain or tenderness
2. Persistent vomiting
3. Clinical fluid accumulation
4. Mucosal bleed
5. Lethargy, restlessness
6. Liver enlargement > 2 cms
7. Increase in hct with decrease in platelet
WHO 2009 Classification
• Severe dengue:
1. Severe plasma leakage
 Shock (DSS)
 Fluid accumulation with respiratory distress
2. Severe bleeding
 As evaluated by clinician
WHO 2009 Classification
3. Severe organ involvement
liver: AST or ALT >1,000
CNS: impaired consciousness
Heart and other organs
Dengue Fever
• Dengue NS-1 Ag – Day 1 and Day 4 of
the illness
Rickets
• Disease of growing bone which occurs in
children only before fusion of the epiphyses
• Due to unmineralized matrix at the growth
• Increase in the circumference of the growth
plate and the metaphyses --> widening of the
wrists & ankles
• General softening of the bones
Rickets
• Craniotabes or softening of the cranial
bones
• Widening of the costochondral junctions
leads to “rachitic rosary”
• “Harrison groove” is a horizontal
depression along the lower anterior
chest due to pulling of the softened ribs
by the diaphragm during inspiration
La Leche League International
• All milk should be dated before storing.
• Preferably, human milk should be refrigerated
or chilled right after it is expressed.
• Guidelines for storing human milk:
1. At room temperature (19-26 C) for 4 hours
(ideal), up to 6 hours (acceptable)
2. In a ref < 4 C for 72 hrs (ideal), up to 8 days
(acceptable)
LLLI
3. In a freezer (-18- -20 C) for 6 months (ideal),
up to 12 months (acceptable)
. What type of container should be used?
1. Glass or hard-sided plastic containers with
well-fitting tops
2. Containers not made with the controversial
chemical bisphenol A (BPA)
3. Containers which have been washed in hot,
soapy water, rinsed well, and air-dried
LLLI
4. Containers should not be filled to the top leave one inch of space to allow for
expansion
5. Put only 2-4 ozs of milk in the container to
reduce waste.
6. Disposable plastic bags are not
recommended as it leads to greater risk of
contamination.
How to warm the milk:
1. Do not refreeze thawed milk.
2. Previously frozen milk that has been thawed
can be kept in the refrigerator for up to 24
hrs.
3. Frozen milk: thaw in the ref overnight or
under cool running water
4. Refrigerated milk: under warm running
water for several minutes. Do not heat the
milk directly on the stove. Do not use
microwave.
Guidelines on
Infant & Young Child Feeding
• UNICEF & WHO recommend that infants be
exclusively breastfed on demand for the 1st 6
months of life
• Early introduction of food & other liquids?
1. Reduces breast milk intake
2. Decreases full absorption of nutrients from
breast milk
3. Increases the risk of diarrhea and URI
Febrile Seizures
Most common seizure disorder in childhood
Rare before 9 months and after 5 yrs old
Peak age of onset: 14-18 months old
Normal neurologic exam
Normal EEG
(+) family history
Comparison:
• Simple:
 Lasts a few secs &
rarely >15 mins.
 Initially generalized and
tonic-clonic
 Followed by a brief
period of post-ictal
drowsiness
 Occurs only once in 24
hrs
• Complex:
 Duration is >15 mins.
 Repeated convulsions
occur within 24 hrs
 Focal seizure activity
Status Epilepticus
• One seizure lasting 30 mins or multiple
seizures during 30 mins without
regaining consciousness
• Usual cause: breakthrough seizures missed doses of anti-epileptic drug/s
• May be due to CNS infection
Persistent Pulmonary
Hypertension of the Newborn
• Failure of the normal circulatory transition that
occurs after birth
• Syndrome: marked pulmonary hypertension
that causes hypoxemia and right-to-left
extrapulmonary shunting of blood
• With inadequate pulmonary perfusion,
neonates are at risk for developing refractory
hypoxemia, respiratory distress, and acidosis.
PPHN
• Most common cause is meconium aspiration
syndrome
• about 13% of all live births are complicated
by meconium-stained fluid but only 5% who
had this complication subsequently develop
MAS
• Coarse streaking granular pattern in both
lung fields
• Irregularly aerated lungs
• Flattened diaphragm, increased AP diameter
PPHN
• 2nd most common cause is idiopathic
• “black-lung”
• Significant remodeling of pulmonary
vasculature with vascular wall thickening and
smooth muscle hyperplasia
• Contributory factor: use of NSAIDs during 3rd
trimester leading to constriction of the fetal
ductus arteriosus in utero
SMR in Boys
SMR
Stage PUBIC HAIR
1
None
2
Scanty, long,
slightly pigmented
PENIS
Preadolescent
TESTES
Preadolescent
Slight enlargement
Enlarged
scrotum, pink,
texture altered
3
Darker, starts to
curl, small amount
Longer
4
Resembles adult
type but less in quantity;
coarse, curly
Larger; glans and
breadth increase in size
5
Adult distribution,
spread to medial surface of thighs Adult size
Larger
Larger, scrotum
dark
Adult size
SMR in Girls
SMR
STAGE
PUBIC HAIR
BREASTS
1
Preadolescent
Preadolescent
2
Sparse, lightly pigmented,
straight, medial border of
labia
Breast and papilla elevated
as small mound; areolar
diameter increased
3
Darker, beginning to curl,
increased amount
Breast and areola enlarged,
no contour separation
4
Coarse, curly, abundant but
amount less than in adult
Areola and papilla form
secondary mound
5
Adult feminine triangle,
spread to medial surface of
thighs
Mature, nipple projects,
areola part of general breast
contour
Gross motor skills
• 6 years old – skip
• 8 years old – hop on one foot twice, then the
other
Fine motor skills
• 6 years old- tie shoe laces
• 7 years old- print letters, letter reversal
• 8-10 years old– rapid alternating
movement of the hand, cursive writing
• 10-12 years old – manipulative abilities
similar to adult
Social development
• Expanding social world
• Identification and reliance on peer groups
7 years – attachment to parents decrease
and to peers increase
9 years – tightly knit groups are formed;
group loyalty and commitment to
best friends
Social development 4-5 y/o
•
•
•
•
Toilet-trained
Plays imaginary games
Helps in tasks in house
Cooperative group play: takes turns and
shares
• Tender and protective
• Cooperative most of the time
• Chooses own friends
Emotional development
4-5 yrs old
Make-believe games
Toy guns are simply an innocent and
entertaining way to be competitive and to boost
their self-esteem (Shelov, 1994).
Interest in basic sexuality
May play with their genitals ---- signs of
normal curiosity!
Do not scold or punish! Be straightforward
Emotional development:
4-5 yrs old
• Parents should answer in simple and correct
terms.
• Parents should tell their child not to let other
person touch the “private parts”.
• Teach your child not to talk to strangers.
• Teach child’s name, address, phone if lost.
• Normal for a 4 year old to make up stories.
• Encourage child to sleep in own bed.
APGAR Score
• What is the order of disappearance in a
sick baby?
Color
Respiration
Muscle tone
Reflex
Cardiac rate
APGAR Score
• What is the order of appearance in a
resuscitated baby?
Cardiac rate
Color
Respiration
Reflex
Muscle tone
Essential Intrapartum
Newborn Care
• Immediately after birth: dry the baby to stimulate
breathing & to avoid hypothermia
• Delay cord clamping 2-3 minutes after birth or until
the cord has stopped pulsating (less occurrence of
IVH and anemia in terms & preterms)
***clamp the cord without milking it 2 cms from the base & put the
2nd clamp 5 cms from the base and cut the cord
Essential Intrapartum
Newborn Care
•
Early skin-to-skin contact: place the
baby on mother’s chest or abdomen
1. to provide warmth
2. to increase the duration of
breastfeeding
3. to allow the “good bacteria” from the
mother’s skin to infiltrate the newborn
(prevents hypoglycemia)
Essential Intrapartum
Newborn Care
• Washing should be delayed until after 6
hours of life because this removes the
vernix, thus exposing the newborn to
hypothermia.
• Non-separation of mother and newborn
for breastfeeding: 20-60 minutes after
birth
Newborn Care
• Eye prophylaxis
Erythromycin ointment 0.5% or
tetracycline ointment 1%
• Vitamin K: 1 mg IM
• Vaccine: Hepatitis B and BCG
Newborn Screening Test
•
•
•
•
Congenital hypothyroidism
Congenital adrenal hyperplasia
Galactosemia
Glucose 6-phosphate dehydrogenase
deficiency
• Phenylketonuria
Newborn Screening Test
• RA #9288
• Done at 48 hours old
• If blood was collected <24 hours old,
repeat at 2 weeks old.
Congenital Hypothyroidism
• Normal birth weight & length
• Delayed physical, mental &
sexual development
• Sluggish, feeding difficulties,
hypothermia
• Edema of scrotum / genitals
• Prolonged physiologic
jaundice
Congenital Adrenal
Hyperplasia
• Deficiency of 21-hydroxylase
enzyme: deficiency of
cortisol
• Normal at birth but signs of
sexual & somatic precocity
appear within the 1st 6
months of life
• Vomiting, failure to thrive
Galactosemia
• 3 distinct enzyme deficiencies:
1. galactose-1-phosphate uridyltransferase deficiency
(GALT) - classic form
2. Galactokinase deficiency (GALK)
3. Galactose-4-epimerase deficiency (GALE)
 Injury to parenychymal cells of the kidneys, liver &
brain
 Feeding intolerance, vomiting, jaundice, convulsions,
lethargy, hypotonia, mental retardation
G6PD deficiency
• Episodic or chronic hemolytic anemia
• Episodic: symptoms develop 1-2 days after
exposure to a substance with oxidant
properties:
 sulfonamides, nalidixic acid, nitrofurantoin,
chloramphenicol, antimalarials, vitamin K
analogs, ASA, benzene, naphthalene
G6PD deficiency
• Onset of hemolysis
results in precipitous
fall in Hgb & Hct
• Heinz bodies
• Reticulocytosis
• Jaundice, anemia,
hemolysis, acute
renal failure
Phenylketonuria
• Deficiency of the enzyme phenylalanine hydroxylase
causes accumulation of pheynylalanine in body fluids
(hyperphenylalaninemia)
• Excess phenylalanine is transaminated to
phenylpyruvic acid or decarboxylated to
phenylethylamine & disrupts normal metabolism &
cause brain damage
Phenylketonuria
• Affected infant is normal at birth
• Most common manifestation without treatment is
developmental delay
• MR develop gradually
• Infant: severe vomiting, hypertonic, hyperactive DTRs,
seizures; older: hyperactive with purposeless movements,
rhythmic rocking & athetosis
• unpleasant musty odor
Neonatal Jaundice
Physiologic
Pathologic
presents after the 48th hour of
life
presents in the 1st 24 hours of
life
TB increases not > 5
mg/dL/day
TB increases by > 0.5
mg/dL/hr
TB peaks at 14-15 mg/dL
TB increases to > 15 mg/dL
DB < 10% of TB
DB > 10% of TB
resolves in 1 week (term), 2
weeks (preterm)
persists beyond 1 week
(term), 2 weeks (preterm)
Jaundice related to
breastfeeding
• Breastfeeding jaundice:
 Onset at 3-4 DOL; 13% of
breastfed infants
 Accentuated unconjugated
hyperbilirubinemia
 Factors: decreased milk
intake with dehydration;
reduced caloric intake
 Duration is a few days
• Breast milk jaundice
 Onset after 7th DOL
 Increased B1 in 2% of
breastfed term infants
 As high as 10-30 mg/dL
during the 2nd-3rd week
 Factors: presence of
glucuronidase in some
breast milk
 Duration: 3 weeks to 3
months
Jaundice related to
breastfeeding
• To reduce incidence of
breastfeeding jaundice:
 Frequent breastfeeding
(>10/24 hrs)
 Rooming-in with night
feeding
 Discouraging 5% dextrose or
water supplementation
 Ongoing lactation support
• Breast milk jaundice:
 If breastfeeding is
continued, bilirubin
gradually decreases but
may persist for 3-10
weeks at lower levels.
 If discontinued, serum
bilirubin level falls
rapidly.
 Phototherapy may be of
benefit.
Small for Gestational Age
• also known as intra-uterine growth retardation
(IUGR)
• BW is < 3rd percentile for calculated gestational age
• growth of the fetus affected by fetal, uterine,
placental and maternal factors
• increased morbidity and mortality
IUGR
• Symmetric - earlier onset & associated with
diseases that seriously affect fetal cell number like
chromosomal, genetic, malformation, teratogenic,
infectious, or severe maternal hypertensive etiologies
• Asymmetric - late onset & associated with poor
maternal nutrition or with late onset or exacerbation
of maternal vascular disease
Large for gestational age
• maternal diabetes & obesity are predisposing factors
• maternal hyperglycemia leads to fetal
•
hyperglycemia & hyperinsulinism
• SSx: hypoglycemia, plethora
• increased risk of:
•
respiratory distress syndrome
•
•
•
congenital cardiac defects
lumbosacral agenesis
hyperbilirubinemia
Post-term infants
• Born after 42 wks of gestation regardless of birth
weight
• Unknown cause
• Their appearance & behavior suggest those of an
infant 1-3 wks of age
• Absence of lanugo & vernix caseosa, long nails,
abundant scalp hair, desquamating skin & increased
alertness
• CS may be indicated for older primigravidas who go
more than 2-4 wks beyond term
Respiratory Distress
Syndrome
• due to deficiency or immaturity of surfactant
– increased surface tension causes alveolar collapse & V/Q
mismatch & hypoxia
• seen in preterms – incidence is inversely
proportional to gestational age (60-80% in <28 wks of
gestation)
• SSx: respiratory distress soon after birth
• CXR: “ground-glass” pattern, air bronchograms
• prevention: reach term, maternal steroids at least
48 hrs prior to delivery
RDS (Hyaline
Membrane Disease)
•







Risk factors:
Maternal diabetes
Multiple births
Cesarean section delivery
Precipitous delivery
Asphyxia
Cold stress
History of previously affected infants
RDS
• Tx: supportive (most are self-limited), mechanical
ventilation for severe RDS & persistent apnea (to
improve oxygenation & elimination of CO2 without
causing pulmonary barotrauma or 02 toxicity),
exogenous surfactant
• complications:
•
pneumothorax
•
•
•
IVH
persistent PDA
bronchopulmonary dysplasia (“bubbly lungs” or cystic
lucencies, irregularly aerated lung)
ABO incompatibility
• Most common cause of hemolytic disease of the
newborn
• Occurs in 20-25% of pregnancies but hemolysis
develops in only 10% of such offspring
• Mother is type O and baby is either A or B
• Most cases are mild; jaundice
• Mild hepatosplenomegaly
• Phototherapy
RH incompatibility
• Rh antigenic determinants are genetically transmitted
from each parent & direct the production of blood
group factors (C, c, D, d, E, e).
• Each factor can elicit a specific antibody response
where 90% are due to D antigen.
• Rarely occurs during the 1st pregnancy because
transfusion of Rh+ fetal blood into an Rh- mother
occurs near the time of delivery, too late for the
mother to become sensitized & transmit antibody to
her infant before delivery.
Mnemonics:
• For infants < 6 months old:
Wt in grams = age in months x 600 + birth
weight
• For infants 6-12 months old:
Wt in grams = age in months x 500 + birth
weight
• For 1-6 years old:
 Wt in kilos = age in years x 2 + 8
Length / Height:
•
Birth to 3 months
4-6 months
7-9 months
10-12 months
9 cm
8 cm
5 cm
3 cm
•
Height in cm = Age in years X 5 + 80
Height in inches = Age in years X 2 + 32
Length / Height:
• At 1 year
30 inches
2 years
1/2 of mature height
3 years
3 feet tall
4 years
40 inches or 2x the
birth length
13 years
3x the birth length
Head circumference:
•
at birth = 14 inches (13.5-14.5)
33-35 cm
- routinely taken up to 3 years old
- approximates adult head circumference at
6 years old
- measured over the glabella and supraorbital
ridges anteriorly and part of the occiput
which gives the maximal circumference
posteriorly
Average increase
in head circumference:
• Age
Increase
Total
First Year
1st 4 mos. 1/2 in/mo
2 inches
Next 8 mos. 1/4 in/mo
2 inches
______________________________
Second Year 1 in
1 in
______________________________
3-5 Years
1/2 in/year
1.5 inches
______________________________
6-20 Years 1/2 in/5 yrs
1.5 inches
Chest circumference:
- measured at mid-respiration at the
level of the xiphoid cartilage or
substernal notch
- measured in recumbent position for
infants
More mnemonics:
•
1st part of infancy
Chest circumference < Head circumference
- Middle part of infancy
CC = HC
- Latter part
CC > HC
•
Number of teeth = Age in months - 6
Growth characteristics:
•
First Year
- 10% decrease in birth weight in the
1st week
- Birth weight regained or exceeded by
2 weeks
- Gain of 30 g/(1 oz) day in the 1st
month
- Gain of 20 g/day beginning the
3rd or 4th month
- Eruption of 1st tooth - mandibular
central incisors
More changes in 2-5 years
old:
• physical energy peaks, need for
sleep
declines (11-13 hr/24 hr)
• VA of 20/20 at 4 years old
• all primary teeth have erupted by
3 years old
School age (6-12 years old):
• gain of 3-3.5 kg (7 lb) and 6 cm
(2.5 in) per year
• head increases by 2-3 cm in the
entire period = slowed brain
growth
• myelinization is complete by 7
years old
• stable body habitus
School age (6-12 years old):
• growth of mid-face and lower face
occurs gradually
• loss of deciduous teeth beginning at
age 6 years (replaced with adult
teeth at about 4/year)
• lymphoid tissues hypertrophy =
impressive tonsils and adenoids
• muscular strength, stamina and
coordination increase progressively
Water requirements
• Normal infant’s absolute water
requirement is 75-100 ml/kg/24 hrs
• 0-6 mo: 700 ml/24 hrs
• 7-12 mo: 800 ml/24 hrs
• 1-3 yrs old: 1,300 ml/24 hrs
• 4-8 yrs old: 1,700 ml/24 hrs
Scurvy
• Vitamin C deficiency early symptoms: lowgrade fever, irritability, tachypnea, anorexia,
generalized tenderness esp. in the legs
• Pseudoparalysis with hips & knees semiflexed & the feet rotated outward
• “scorbutic rosary” at the costochondral
junction & depression of the sternum
• Angulation of the scorbutic beads is sharper
than the rachitic rosary
Scurvy
• Bluish, purple spongy swellings of the
mucous membranes esp. over the upper
incisors
• Other symptoms: swollen joints, purpura and
ecchymoses, poor wound & fracture healing,
perifollicular hemorrhages
• X ray changes: distal ends of long bones with
a ground-glass appearance
• Plasma ascorbate of <0.2 mg/dL is deficient
Treatment for scurvy
• Daily intake of 3-4 oz of orange or tomato
juice
• Vitamin C supplements of 100-200 mg orally
or parenterally are preferable to ensure more
rapid and complete cure.
• Larger doses (>2 grams) may produce
abdominal pain and osmotic diarrhea
Contraindications to
Breastfeeding
•
•
1.
2.
3.
4.
5.
6.
7.
Infants with galactosemia
Mothers with:
Septicemia
Active TB
Breast cancer
Malaria
Typhoid fever
Substance abuse
Severe neurosis or psychosis
Breastfeeding
• Acute maternal infection may contraindicate
breastfeeding IF the infant does not have the
same infection.
• When the infant is unaffected, the breast may
be emptied and the milk be given by cup or
bottle.
• Mastitis usually can be alleviated by
continued and frequent nursing on the
affected breast to keep it from becoming
engorged (warm compress).
Breastfeeding
• Transmission of HIV by breastfeeding is well
documented.
• If safe alternatives are available,
breastfeeding by HIV-infected mothers is not
recommended.
• In many developing countries, breastfeeding
may be crucial for infant survival.
• WHO recommends that breastfeeding be
continued unless safe infant formula is readily
available.
Breastfeeding
• CMV, rubella virus, hepatitis B virus, human
T-cell lymphotropic virus type 1, & herpes
simplex virus have been demonstrated in
breast milk.
• Evidence of breast milk transmission of other
viruses is rare.
• Although hep B virus has been isolated from
breast milk, the predominant means of
mother-infant transmission appears to be
through delivery.
Leukemias
• Most common malignant neoplasm in
childhood (41% of all malignancies that
occur in <15 years old)
• ALL (77%)
• AML (11%)
• CML (2-3%) - Philadelphia
chromosomal translocation
• JCML (1-2%)
Leukemias
• Group of malignant diseases in which genetic
abnormalities in a hematopoietic cell give rise
to an unregulated clonal proliferation of cells
• Increased rate of proliferation & decreased
rate of spontaneous apoptosis
• Result: disruption of normal marrow function
Acute Lymphoblastic
Leukemia
• malignant proliferation of lymphoblasts
• most common childhood malignancy
• peak incidence 2-6 yrs old; M>F
• unknown etiology
• increased incidence in:
– Down Syndrome, ataxia-telangiectasia,
– Fanconi syndrome, neurofibromatosis type 1
– risk is >70% if the first twin is diagnosed during the 1st year
of life and they shared the same placenta
– patients who have undergone intense Tx for a solid tumor
Other ALL risk factors:
• Exposure to medical diagnostic
radiation during in utero and in
childhood
• Drugs, alkylating agents
• Nitrosurea
• Benzene exposure
• Advanced maternal age
ALL
• SSx: (acute onset < 4 wks duration of Sx)
 non-specific (anorexia, irritability, lethargy)
 signs of marrow failure (anemia, bleeding,
purpuric/petechial lesions, low-grade fever)
 signs of infiltration (bone pain, lymphadenopathy,
–
splenomegaly > hepatomegaly)
ALL
• Dx: bone marrow aspiration
– anemia, thrombocytopenia (75%),
lymphoblasts in the PBS & bone marrow,
median WBC count is 33,000
– CXR may show a mediastinal mass
– CSF may contain lymphoblasts (10-20%)
Diagnosis
• ALL is diagnosed by a bone marrow evaluation that
shows >25% of the BM cells as a homogenous
population of lymphoblasts
• Differential dx: infectious mononucleosis (acute onset
of fever and lymphadenopathy) and JRA (fever and
joint swelling)
Prognostic / predictive factors:
•
The single most important prognostic factor in ALL
is the treatment.
•
1.
2.
3.

3 of the most important predictive factors:
Age of the patient at the time of diagnosis
Initial leukocyte count
Speed of response to treatment
Average risk: age between 1-10 yrs old and
leukocyte count of <50,000/uL
ALL
• no anatomic staging system because usually
disseminated at the time of Dx
• Tx:
– remission induction (vincristine, prednisone, Lasparaginase x 4 wks + CNS prophylactic irradiation or
chemotherapy) to eradicate leukemic cells from BM
– consolidation phase: 14-28 wks to prevent later CNS
relapse
– maintenance phase x 2-3 years (Mercaptopurine daily and
Methotrexate weekly)
•
complication of treatment: tumor lysis syndrome
Treatment
• Remission defined as <5% blasts in the BM
and a return of neutrophil and platelet count
to near-normal levels after 4-5 wks of tx
• RELAPSE occurs in the BM in 15-20% of
patients with ALL -- BAD! esp. if it occurs
during or shortly after the tx
• Options: intensive tx with agents not
previously used followed by allogenic stem
cell transplantation results in long-term
survival rate for a few patients
ALL
• sites of relapse: bone marrow, CNS (increased ICP
and isolated cranial nerve palsies), testes (painless
swelling of one or both testes in 1-2% of males)
• poor prognostic factors:
–
–
–
–
–
< 2 yrs or > 10 yrs
male
WBC > 100,000 u/L on presentation
presence of CNS leukemia
presence of a mediastinal mass
Comparison:
•








Osteosarcoma:
2nd decade; M>F
All races
Spindle-cell producing
osteoid
Metaphysis of long bones
Local pain & swelling, often
history of injury
Sunburst pattern
Spreads to lungs, bones
Chemotx and ablative
surgery of primary tumor
•








Ewing tumor:
2nd decade: M>F
Primarily whites
Undifferentiated small round
cell of neural origin
Diaphysis of long bones
Local pain & swelling with
fever
Onion-skinning
Spreads to lungs, bones
Chemotx, radiation and/or
surgery of primary tumor
Type
Description
Cause
Obstructive or Obstruction
Abnormality of the
noncommuni- within the ventri- aqueduct or a lesion in
cating
cular sytem
the 4th ventricle (aqueductal stenosis)
Nonobstruc- Obliteration of Follows a
tive or
the subarachsubarachnoid hemorrcommunica- noid cisterns
hage
ting
Malfunction of
the arachnoid
villi
Leukemic infiltrates
Febrile Seizure
•
•
•
•
•
•
Most common seizure disorder in childhood
Rare before 9 months and after 5 yrs old
Peak age of onset: 14-18 months old
Normal neurologic exam
Normal EEG
(+) family history
Comparison:
• Simple:
 Lasts a few secs &
rarely >15 mins.
 Initially generalized and
tonic-clonic
 Followed by a brief
period of post-ictal
drowsiness
 Occurs only once in 24
hrs
• Complex:
 Duration is >15
mins.
 Repeated
convulsions occur
within 24 hrs
 Focal seizure
activity
Summary of
Neurocutaneous syndromes
Syndrome Manifestations
PE
finding
Diagnos- Treattics
ment
Neurofibro
-matosis
(Von
Recklinghausen)
Café au lait
Axillary or
CT scan or
macules that
inguinal
MRI
spare the face freckling,
Lisch
nodules,
optic glioma
Genetic
counseling
& early
detection of
treatable
complications
Tuberous
Sclerosis
Multisystemic;
Seizures,
mental retardation
Seizure
control;
multi-disciplinary
approach
Tubers in
cerebrum
(candledripping);
ash leaf
shagreen
CT scan or
MRI of the
brain, heart,
abdomen;
2D echo;
renal UTZ
PEDIATRIC
PULMONOLOGY
Bronchial Asthma
• a reversible obstructive airway disease
involving both the small & large airways
– increased residual lung volumes
– decreased FEV1/FVC ratio
• 3 components of an asthma attack:
– bronchospasm
– mucus production
– airway edema
Bronchial Asthma
• SSx: family Hx of asthma or atopy, recurrent cough &
wheezing with exposure to certain “triggers” (viral
infection, weather changes, exercise, allergens,
emotions), responds to Tx with bronchodilators
• CXR: hyperinflation; helps to exclude structural
abnormalities of the airway or chronic infection
Bronchial Asthma
• Pulmonary function tests: increased
residual lung volumes, decreased
FEV1/FVC ratio
Not routinely done esp. for <5 yrs old
due to inability of these children to
perform reproducible expiratory
maneuvers
Exacerbation of asthma:
• Acute or subacute deterioration in symptom control
that is sufficient to cause distress or risk to health
• Any of the following:
 Increase in wheeze or shortness of breath
 Increase in coughing, esp. at night
 Lethargy or reduced exercise tolerance
 Impairment of daily activities
 Poor response to reliever medication
Management:
• Management of acute attacks:
–
–
–
–
•
short-acting inhaled beta2-agonist
oral or IV steroids (Prednisolone)
anticholinergics (ipratropium bromide) – never used alone
methylxanthines (theophylline, aminophylline) - NOT first
line
Management in between attacks:
– inhaled corticosteroids
– long-acting inhaled beta2-agonist
– leukotriene modifiers (Montelukast)
Levels of asthma control
Characteristic
Controlled (all Partly
of the following)
controlled (any
measure present in
any week)
Uncontrolled (3 or more
of features of partly controlled
asthma in any week)
Daytime
symptoms
None (less
than 2x/ wk)
More than
2x/wk
More than 2x/wk (last
minutes or hours or
recur)
Limitation of
activities
None (fully
active)
Any (may
cough, wheeze
during exercise,
vigorous play,
or laughing)
Any (may cough,
wheeze during exercise,
vigorous play, or
laughing)
Levels of asthma control
Characteristic
Controlled (all Partly
of the following)
controlled (any
measure present in
any week)
Uncontrolled (3 or more
of features of partly controlled
asthma in any week)
Nocturnal
symptoms or
awakening
None (no
nocturnal
coughing
during sleep)
Any (typically
coughs during
sleep or wakes
with cough,
wheezing,
and/or
dyspnea)
Any (typically coughs
during sleep or wakes
with cough, wheezing,
and/or dyspnea)
Need for
reliever /
rescue
treatment
2 days/week
> 2 days/week
> 2 days/week
Initial Assessment of Acute Asthma
Symptoms
Mild
Severe
Altered consciousness
No
Agitated, confused, or
drowsy
Oximetry on
presentation (Sa02)
Equal or more than
94%
Less than 90%
Talks in sentences/words sentences
words
Initial Assessment of Acute Asthma
Symptoms
Mild
Severe
Pulse rate
< 100 bpm
>200 bpm (0-3 yrs)
>180 bpm (4-5 yrs)
Central cyanosis
absent
Likely to be present
Wheeze intensity
variable
May be quiet
How to use table
• Any of the “severe” symptoms indicate a
severe asthma exacerbation
• Oximetry performed before
administration of oxygen or
bronchodilator
Pulmonary Tuberculosis
• Mycobacterium tuberculosis
• Most specific confirmation is isolation of the
organism!
• Sputum specimens for culture for those who can
expectorate
• Induce sputum with a jet nebulizer & chest
percussion followed by nasopharyngeal suctioning:
for culture and smear staining
PTB
• Gastric aspirates: cultured
• Young children: early AM gastric acid
obtained before the child has arisen &
peristalsis has emptied the stomach of
the pooled secretions
• 3 consecutive AM gastric aspirates yield
the organism in <50% of cases
Pulmonary Tuberculosis
•
1.
2.
3.
4.
Primary complex (Ghon complex):
Primary pulmonary focus
Regional lymph nodes
Peritracheal lymph nodes
Localized pleurisy between the middle
& lower lobes
Diagnostic Criteria for PTB:
•
•
•
•
•
Exposure to TB sputum (+) adults
(+)PPD test
Signs & symptoms (any 2 or more)
Chest X ray findings
Isolation of the organism
Signs and symptoms:
•
•
•
•
•
•
Cough w/ or w/o wheezing for > 2 wks
Unexplained fever for > 2 wks
Failure to gain weight; weight loss
Unexplained poor appetite
Painless cervical lymphadenopathy
Failure to respond to 2 wks appropriate
antibiotic therapy for LRTI
Classification:
• Class I: TB exposure: (+) exposure to
and adult/adolescent with active
disease, (-) PPD, no signs/symptoms,
negative chest x ray findings
• Class II: TB infection: +/- exposure, (+)
PPD, no signs/symptoms, negative
chest x ray findings
Classification:
•
1.
2.
3.
4.
5.
Class III: TB disease: 3 or more of the
ff criteria:
Exposure to an adult/adolescent with
active TB disease
(+)PPD
Signs/symptoms suggestive of TB
Abnormal chest x ray findings
Laboratory findings
Classification:
•
1.
2.
3.
4.
5.
6.
Class IV: TB inactive:
w/ or w/o history of previous TB
w/ or w/o previous chemotherapy
has radiographic evidence of healed/calcified TB
(+)PPD
no signs & symptoms
(-) smear or culture for TB
PPD interpretation:
 Equal or >10 mm is (+)
 Equal or >5 mm is (+) in the presence of any or all
of the ff:
1. history of close contact with a known or suspected
case of TB
2. clinical findings suggestive of TB
3. chest X ray findings suggestive of TB
4. immunocompromised condition
Management:
•
•
•
•
•
INH 10 mg/kg/day (6 months)
Rif 15 mg/kg/day (6 months)
PZA 25 mg/kg/day (2 months)
Ethambutol 15-25 mg/kg/day (>6 y/o)
Streptomycin 20-30 mg/kg/day