Case 2: 2mo, 6mo, 9mo WCC, Neuroblastoma Flashcards
appropriate growth patterns in infants up to 9mo of age using standard growth charts
“1) right after birth - 10% weight los
2) within 1-2w - regain birth weight”
“nutritional requirements for infants at ages 1-2mo
caloric requirements?
Dietary recommendations”
“1) term –> 100-120cal/kg/d. 20-30g daily weight gain
2) preterm –> 115-130 cal/kg/d
3) very low birth weight (VLBW) –> 150cal/kg/d
VitD - 400U / d (or 32oz of formula or milk /d
- supplement for exclusive/mostly breastfed”
“nutritional requirements for infants at age: 9mo
caloric requirements?”
“100cal/kg/d –> 75% from breast mild / formula (24oz/d)
- very small chunks of food (if have teeth)
- self-feed finger foods (toast, crackers, pasta, banana)
- meats
NEVER - popcorn, grapes, hard candies, hot dogs –> d/t risk of choking (b/c infant often swallow w/out chewing)”
“diffdx: abdominal mass in RUQ + pallor in 9mo (with no jaundice, splenomegaly, or lymphadenopathy). otherwise healthy
-plan for evaluation”
1 - Neuroblastoma (peak <2y)–> most common neoplasm in infants; usually with fever, pallor, NO JAUNDICE, wt loss/ retarded growth, lymphadenopathy, painless mass in neck / chest, abd +/- mets to bone marrow
- Hepatic neoplasm –> +/- jaundice; rare in so young
- Hydronephrosis –> usually @ this age; +/- UTI
(1) obstruction at uretero-pelvic junction = hydronephrosis; palpable kidney / flank mass
(2) multicystic kidney
- smooth, rarely cross midline
- 50% abd pain, vomiting, +/- HTN
- Teratoma –> rare malignant tumor; can cause mass effect / compressive sxs in surrounding areas –> abd / back pain; N/V, constipation, UTI
difference between expected developmental milestones (surveillance) and standardized tools
“Developmental surveillance = checking milestones in 4 domains: gross motor, fine motor; communication / social, cognitive/adaptive
==> helps to determine areas of concern - “mental snapshot”
validated developmental screening test (more sensitive/specific v. developmental surveillance) = pick up true developmental / behavioral abnormalities –> done at 9, 18, 24mo”
WCC: maternal / infant factors
“Maternal factors
- complications / infections during pregnancy; meds; drugs / EtOH
- concerns about prenatal labs: HIV, RPR, Hep B, Group B strep
Infant
- delivery date (wrt due date)
- birth weight
- perinatal course (complications in nursery; received newborn meds / vaccines); jaundice; when discharged
- hearing test”
WCC: social hx and diet
“Social hx
- who lives at home; ? Daycare
- IT HELLPS
Income
Transportation
Housing Education Legal Status/immigration Literacy Personal Safety
Diet
- ? Breast / bottle feeding (how formulated)
- how many diapers does she change.”
“what special physical exam tests do you do for a:
2mo”
2mo –> fontanelles; red reflex; ortolani, barlow, moro & babinski reflexes; rashes; back: sacral dimple / hair tuft
immunizations for 2mo
"DTaP (A) Hib (D) IPV (E) PCV13 (F) RotaV (G) and HepB #2 (C) --> +/- 1st HepB if not given in nursery prior to discharge"
immunizations for 6mo
"DTaP #3 Hib #3 HepB #3 RotaV #3 PCV13 #3 \+/- IPV #3"
Does the IPV #3 need to be given in the 6mo visit?
“No
Can give between 6-18mo to reduce # of shots in 6mo visit”
Imaging & pathology of a neuroblatoma
“Abd U/S - retroperitoneal mass arising from the adrenal gland that does not cross the midline. Tumor has heterogeneous consistency with both solid & cystic elemenents (== hemorrhage / necrotic tumor)
AXR - large mass in R/L UQ
Abd CT - same as U/S + diffuse stippled calcification with invasion of renal parenchyma. +/- LN enlargement
Bone marrow aspiration / biopsy - ““small round blue cells”” or small, uniform cells containing dense, hyperchromatic nuclei and scant cytoplasm, forming small cell rosettes”
Neuroblastoma genetics
- assoc.with MYC-N gene
FAMILIAL (1%) - AD, low penetrance
NON-FAMILIAL- somatic mutations
Neuroblastoma labs / studies
1) CBC - Anemia / other cytopenia (2/2 bone marrow infiltration)”
2) Elevated urinary HVA/VMA
IMAGING
1) Abd US –> identification of mass, organ of origin, determine if mass is solid / cystic / combined
2) AXR - identify presence of mass, ?
calcifications, ? bowel obstruction from mass
3) CXR - assessment for mets to lung
4) Skeletal survey - mets to bone
5) Abd CT - reveals calcifications; surrounding anatomy for surgery; consistence of tumor (homo/heterogeneous); evaluate lungs
6) MRI if near spine
7) bone scan
8) BM aspirates
Neuroblastoma metastases and imaging
CXR
regional LN liver bone marrrow skeleton posterior mediastinum
diffdx infant rashes
“Benign; resolve over time:
- neonatal acne = papules & pustules over the face; d/t hormonal stimulation of sebaceous glands
- seborrheic dermatitis = ““cradle cap””; yellowish, oily scales over the scalp”
parental evaluation of developmental status
“Child between 0-8yo –> to help identify children who may have developmental delay
- parental concerns (10 q on perspective on child for each developmental domain)
- direct toward one of 5 decisions: refer, screen further, watch carefully, counsel parents, reassurence”
“nutritional requirements for infants at age: 4mo
caloric requirements?”
Start on solid foods - rice cereal with a spoon
car seat recommendations: <2yo,
rear-facing car seat; restained in rear seat
car seat recommendations: 2-4yo,
forward-facing car seat; restained in rear seat
car seat recommendations: 4-8yo,
belt-positioning booster seat, restained in rear seat
car seat recommendations: >8yo
nml; restarined in rear seat
car seat recommendations: >13yo
nml; restarined in rear/front seat
Imaging and pathology of a Wilm’s tumor
- U/S renal vein - intrarenal mass
- CT chest and abd - heteroeneous with areas of low density (== necrosis). Pseudocapsule (== d/t sharp demarcation b/w tumor and normal renal parenchyma) = “claw sign” as tumor grows
- pathology == tubules (as though it’s trying to grow into a kidney itself)
Wilm’s tumor labs
Hematuria
Wilm’s tumor metastases
“Imaging: CXR, CT chest (areas of lung below level of diaphragm dome)
- Pulmonary”
Imaging & pathology of a hepatic tumor
“AXR - hepatic enlargement + hepatic tuomors
CT scan - hepatic tumor”
hepatic tumor labs
”.- nml LFTs (in 20%, elevated LFTs, bilirubin)
- increased AFP”
hepatic tumor metastases
“chest CT
- pulmonary”
Imaging & pathology of a teratoma
“CT - identification of teratomas / germ cell tumors. Well-defined mass with both solid & ystic components
X-ray - ONLY if have calcified components (teeth, bony fragments)”
what is the best imaging modality for identifying teratomas & germ-cell tumors?
CT - Well-defined mass with both solid & ystic components
Imaging & pathology of a benign renal mass
“U/S - hydronephrosis
voiding cystourethrogram - for ureteral obstruction; vesicoureteral reflux”
“staging of neuroblastoma
Stage 1, 2A, 2B, 3, 4, 4s
- identify and define treatment and prognosis”
“Stage 1 - tumor localized to area of origin
- tx = surgical resection (chemotherapy if recurrence)
- 90% disease free survival
Stage 2A - extends beyond area of origin; does not cross midline
- tx = surgical resection + low dose chemotherapy if recurrence (radiation for failure to respond)
- 85% disease free 2y survival
Stage 2B - extends beyond area of origin; does not cross midline + ipsilateral LN
- tx:
== [INFANT] surgical resection + low dose chemotherapy if recurrence (radiation for failure to respond)
- 85% disease free 2y survival
== [CHILD] chemo and/or radiation
- 60-70% survival
Stage 3 - extends beyond midline / regional LN involvement
- tx & survival same as 2B
Stage 4 - disseminated to distant LN, bone, bone marrow, liver or other origins (lungs) - tx: == [INFANT] chemo and/or radiation - 60-70% survival == [CHILD] chemo + radiation + BMT - <15% survival
Stage 4S - localized primary tumor + dissemination limited to liver, skin, or bone marrow
- tx:
== [INFANT <6w (-) skin involvement] - 38% survival
== [INFANT > 6w] = observation; chemo / local radiation for enlarging abd mass causing respiratory compromise
- 86% survival”
favorable prognostic factors for neuroblastoma
”.- non-amplification fo n-myc oncogene
- DNA index > 1
- well-differentiated histology (Shimada classification)
- age < 1y”
Components of WCC
“1) interval hx (any illnesses, problems; detailed birth hx for first visit)
2) development - PEDS / ASQ”
developmental screening - when is it mandated to be done by AAP
9mo, 18mo, 30mo
when is specific autism screening recommended
18mo, 2y
infant sleeping recommendations
“sleep through the night by 4-6mo
Sleep in supine (on back)”
Safety concerns for a baby
“NO SMOKING around the baby
NO small objects / plastic bags
NO hot liquids aroud baby
NO leaving baby alone on high places”
car safety: at what age can a child sit in the front seat
“>13yo
the back seat is the safest place in a vehicle for children”
for how long should a child sit in car seat rear-facing
2y