treatment of developmental dysplasia of the hip when dx made in first few weeks or months of life

dx made in first 4 months

dx made late

within first few weeks or months: pavlik harness

first 4 months: reduction by flexing and abducting hip

late cases: preoperative traction for 2 - 3 weeks, then an operative traction, closed reduction procedure, and a hip spica for 6 mo following reduction

treatment of septic arthritis for:

infants

> 5 yrs

infants: nafcillin/oxacillin + 3rd gen cephalosporin

> 5 yrs: antistaph x 3 wks

oral Abx started with clinical signs markedly improve

IV Abx started within 24 - 72 hrs

change to oral Abx when tenderness, fever, WBCs, and CRP start to decrease, and culutre is positive

splinting of limb

frank pus is aspirated from bone

infection shows no dramatic response within 24 hrs

limits bone damage

cast the fott, changing at intervals of 2 weeks to correct the deformity

corrective shoes must be used to maintain correction obtained by the casting

(clubfoot)

manipulate foot to stretch contracted tissue on medial and posterior aspects

splint to hold the correction

add night braces after full correction is obtained (necessary as long term maintenance)

surgical release if foot is rigid and resistant to casting

bisphosphonates

surgical tx to correct deformity of long bones with multiple intramedullary rods

non-weight bearing w/crutches

immediate referral to orthopedic surgeon - femure head internally fixated to neck and fracture line allowed to heal

apparent only in lateral view xray:

"frog leg" position - shows widening of the physis and epiphyseal slippage

protection of the joint - principal of treatment

surgical tx is controversial

typically resolves spontaneously

NSAIDs for pain control

stretch the hamstrings

ice after workouts

during what age period is genu varum physiologically normal

when is genu valgum physiologically 

genu varum is normal from infancy to 2 yrs

genu valgum is normal from 2 yrs to 8 yrs

persistent bowing/varum beyong age 2

bowing/varum that is increasing rather than decreasing

bowing/varum of one leg only

knock knee associated with short stature

bracing

osteotomy - rare

necessary for severe problem such as blount disease

shoes and braces show little benefit

active external rotation exercises - skating, bike riding

osteotomy for rotational correction - rare

if pt has no external rotation of hip in extension - refer to ortho

treatment for a pt with scoliosis that is between 20-40 degrees of curvature

for a pt that is >40 degrees and resistant to bracing

for a pt that is >60 degrees with poor pulmonary function is adult life

bracing in skeletally immature child

>40 degress: surgical correction and posterior spinal fusion

>60 degrees: surgical correction and posterior spinal fusion

grasp invloves the ulnar aspect at 3 - 4 months

thumb is added, movement shifts to radial side at about 5 months

thumb opposes the fingers just before 7 months

neat pincer grasp emerges at about 9 months

child typically develops handedness between 18 and 30 months

and should NOT have a significant preference before 1 year of age

2 months: melodic vowel sounds (cooing)

9 months:  word comprehension begins to increase

6 - 10 months: babbling and repetition (da, da, da, da), has added consonants

peaks at 12 months

2 years: 2 - 3 word phrases

completing puberty and somatic growth

developing socially, emotionally, cognitively, and moveing from concrete to abstract thinking

establishing an independent identity, separating from family

preparing for a career or vocation

skin on scrotum thins/enlarged, penis length unchanged

OR

breast bud forms, w/small area of surrounding glandular tissue, areola begins to widen

scrotum enlarges more and darkens, penis continues to increase

OR

increased breast size and elevation, areola and papilla form 2ndary mound

abnormal function in:

language development

social development

behavior

can be diagnosed by prenatal ultrasound

(renal U/S identifies cysts in about 80% of affected children by age 5)

at what age is it more common for boys to get a UTI vs girls;

and when is it more common for girls to get a UTI than boys

uncircumcised boys < 3 months are more likely to get a UTI

girls older than 6 months have UTIS much more commonly than boys

E coli (>85%)

klebsiella

indicated for:

select children with recurring UTI

children with high grade VUR - as an alternative to surgery or before surgery

prophy: trimethoprim-sulfamethoxazole and nitrofurantion

surgical emergency

must treat within 4 - 6 hours

if no GI abnormalities:

treat the constipation

tend to get better in time (1 - 5 yrs)

assess and treat comorbid findings and consider abx prophy

rash

arthralgias

ABD pain

renal disease

aggressive high dose pulse methylprednisone with cyclophosphamide

follow with oral prednisone to decrease inflammatory processes

ANA+ : slit lamp every 3 months for first 4 years of dz

ANA- : slit lamp every 6 months for the first 4 years of dz

early in the dz, MRI is the only way to detect the dz

(later findings will show up on x-ray)

NSAIDs

try for a month and on a regular basis

second line tx for juvenile rheum arthritis

and what must be monitored while on this medication

if pt fails NSAIDs - next line: DMARDs

methotrexate weekly

must monitor CBC and LFTs

immediate referral to ophthalmology 

ocular steroids - if refractory, may use methotrexate or biologics

PT/OT - important for mobility!

heat, water therapy

shoe lifts for people with leg length discrepancy

must have four of the following:

malar rash

discoid rash

photosensitivity

mucous membrane ulcers

arthritis

serositis

renal abnormalities

neurologic abnormalities

blood count abnormalities

+ANA

autoantibodies

treatment of SLE pt with skin manifestations, arthritis, and fatigue

treatment of SLE pt with pleuritic pain and arthritis

can treat with antimalarials: hydroxychloroquine

NSAIDs

pt presentation:

microcephaly, midface hypoplasia characterized by poorly developed long philtrum (midline groove in upper lip), narrow palpebral fissures, short nose with anteverted nares

suspicion of what?

pt (infant) presentation:

small head circumference, anteverted nares, cleft lip and palate (not always), distal digital hypoplasia, and pt has increased risk for spina bifida

suspicion of what?

pt/infant presentation:

flattened occiput, up slanting palpebral fissures, epicanthal folds, midface hypoplasia, and small dysplastic pinae

suspicion of what?

pt/infant presentation:

small for gestational age, overlapping fingers and rockerbottom feet, and VSD or PDA

suspicion of what?

Sx or medications for heart problems (endocardial cushion defect or septal defect)

Abx for infection

thyroid function tests

stimulation programs

special ed, PT/OT/speech therapy

support gorup for parents

female pt presentation:

triangular facies, short stature, wide-set nipples, amenorrhea, absence of 2ndary sex characteristics, webbed neck

suspicion of what?

estrogen replacement therapy - development of 2ndary sex characteristics, norm menstruation, prevent osteoporosis

growth hormone - for height

ID and tx of perceptual difficulties before the become problematic is crucial

male pt presentation:

microorchidism, lack of libido, minimal facial hair, tall, eunochoid build, gynecomastia

suspicion of what?

pt presentation of:

lisch nodules upon slit lamp examination, scoliosis, large head, with average IQ

suspicion of what?

Neurofibromatosis

(didn't give you the easy one: cafe au lait macules of 6 or more by age 1)

pt presentation:

astigmatism, mitral valve prolapse, Hx of spontaneous pneumothorax, joint laxity,  mild myopathy, and crowded dentition

suspicion of what?

Marfan Syndrome

(may have progressive aortic root dilation, valvular incompetency, dysrhythmias, down slanting palpebral fissues, tall and slender, high arched palate)

serial EKGs to follow aortic root enlargement

prophylactic BB to slow heart rate

(other tx's non-heart related: management of ophthalmologic

pt presentation:
shallow orbits, midface narrowing, hydrocephalus, normal IQ

suspicion of what? 

followed at a CF foundation-accredited CF care center

high calorie diet - high protein and high fat diet

pancreatic enzyme capsules with meals - PPI to decrease stomach acidity

multi vitamin (esp. A,D,E,K) and daily salt supplement

airway clearance tx - inhaled mucolytic agent, hypertonic saline and tobramycin, and chronic azithro PO

lung transplant for end stage lund dz

replacement of factor VIII - using variety of preparations derived from human plasma or recombinant techniques

10 - 15% of treated individuals develop neutralizing antibodies that decrease its effectiveness

infant with cleft lip - surgical lip closure typically occurs at what age

palate closure to facilitate normal speech w/o nasality typically occurs at what age

 surgical lip closure: occurs usually after 10 weeks of age

palate closure: occurs at 12 - 18 months of age

Vertebral anomalies

Anal atresia

CV anomalies

TracheoEsophageal fistula

Renal or radial anomalies

Limb defects

pt presentation:

macrocephaly, increased startle reaction, macular cherry red spot, ataxia and dementia

suspicion of what?

tay-sachs

(adult presentation would include: additional spincerebellar, lower motor neuron symptoms, and dysarthria)

at what age is anemia physiologic

and what level is the Hgb?

6 - 8 weeks - physiological anemia of infancy

Hgb: 10 g/dL

bone marrow transplant for severe aplastic anemia if pt has HLA-compatible sibling donor

comprehensive supportive care

Abx is fever is present

platelet transfusions - but sparingly

oral dose of elemental iron - 6 mg/kg/d in three divided daily doses

if mild: 2 mg/kg/d once daily before breakfast

avoid medications that bring on episodes (ex. antimalarials, and sulfonamide compounds)

most episodes are self-limiting, but red cell transfusion can be life saving when S&S indicate cardiovascular compromise

platelet transfusion should be avoided except in circumstances of life-threatening bleeding

if necessary - urgen splenectomy is indicated, and administration of corticosteroids and IVIG

first month: induction (at the end > 95% exhibit remission)

then consolidation: intrathecal chemo and systemic therapy

and then several months of intensive chemo

induction of remission (might be wrong terminology, but didn't want to list all the drugs for this part, since Julie said we didn't need to know the names)

after remission: if pt has a matching sibling, pt can undergo allogeneic hematopoietic stem cell transplantation (HSCT)

after remission: if pt does NOT have matched sibling - additional cycles of aggressive chemo for total of 6 - 9 months

control complications due to mass compression

control complications dur to tumor lysis syndrome (IV fluids, diuretics, allopurinol)

surgical resection is not indicated unless the entire tumor can be resected safely (rare)

high dose methotrexate is short intervals

monoclonal antibodies

permeative destruction of the normal bony trabecular pattern with indistinct margins

periosteal new bone formation and lifting of the bony cortex may create a codman triangle

en bloc resection of the tumor with a margin of uninvolved tissue

amputation, limb salvage, and rotationplasty are equally effective

follow the surgery with chemotherapy for about 1 year

treatment begins with surgical exploration of the ABD via an anterior surgical approach to allow for inspection and palpation of the contralateral kidney

liver, lymph, and suspicious nodes biopsied/excised

tumor resectioning and surgery tailored to tumor stage

presentation of eczema on child <2 years

on child > 2 years

< 2 years:begin on cheeks, scalp, then on trunk and extensor surfaces

> 2 years: moves to flexural surfaces, neck, wrists, hands and feet

what is the preferred drug for pts of all ages with asthma as their FIRST line treatment

if not enough, what is added next

if that is not enough, what else is added to the above two

all ages, first line: SABA as needed

if not enough, for all ages, add low dose ICS

third step:

0 -4 yrs: medium dose ICS

5 - 11 yrs: low dose ICS + LABA or LTRA or theophylline OR medium dose ICS

>12 yrs: low dose ICS + LABA OR medium dose ICS

PCN prophy, routine immunizations (esp. yearly flu shot), red cell transfusions during exacerbations

successful stem cell transplantation cures, but is a very risky procedure - so, rarely done