general 4 Flashcards
what level of dehydration is suggested by the following clinical exam finding:
skin turgor good
mild
what level of dehydration is suggested by the following clinical exam finding:
alert, irritable child
moderate
what level of dehydration is suggested by the following clinical exam finding:
parched/cracked buccal mucosa/lips
severe
what level of dehydration is suggested by the following clinical exam finding:
dry/sunken eyes
severe
what level of dehydration is suggested by the following clinical exam finding:
tears present when crying
mild
what level of dehydration is suggested by the following clinical exam finding:
urine output significant diminished
moderate
what level of dehydration is suggested by the following clinical exam finding:
urine output oliguric
severe
what level of dehydration is suggested by the following clinical exam finding:
urine output diminished
mild
what level of dehydration is suggested by the following clinical exam finding:
flat fontanel
mild
what level of dehydration is suggested by the following clinical exam finding:
mild skin tenting
moderate
what level of dehydration is suggested by the following clinical exam finding:
cap refill above 3seconds
severe
what level of dehydration is suggested by the following clinical exam finding:
fontanel sunken
severe
what level of dehydration is suggested by the following clinical exam finding:
buccal musoca/lips moist or tacky
mild
what level of dehydration is suggested by the following clinical exam finding:
present tears, but reduced on crying
moderate
what level of dehydration is suggested by the following clinical exam finding:
dry skin to touch
moderate
what level of dehydration is suggested by the following clinical exam finding:
unremarkable skin to touch
mild
what level of dehydration is suggested by the following clinical exam finding:
soft, slightly depressed fontanelle
moderate
what level of dehydration is suggested by the following clinical exam finding:
slightly dry, deep set eyes
moderate
what level of dehydration is suggested by the following clinical exam finding:
dry, sunken eyes
severe
what level of dehydration is suggested by the following clinical exam finding:
increased pulse
severe
what level of dehydration is suggested by the following clinical exam finding:
age appropriate or slightly increased pulse
mild…mild/moderate
what level of dehydration is suggested by the following clinical exam finding:
cap refill 2-3 seconds
moderate
what level of dehydration is suggested by the following clinical exam finding:
lethargic
severe
what level of dehydration is suggested by the following clinical exam finding:
alert, consolable or irritable
mild
why do kids have a higher risk for dehydration
have higher surface area:body mass ration–greater relative area for evaporation
higher basal metabolic rates than adults which generates heat and expends water
higher % body weight that is water (infants are 70%, kids 65%, adults 60%)
why is diffuse abdo pain characteristic of DKA
ongoing acidosis
how does toxic ingestion often present
vomiting
altered mental status
obtundation
- aspirin overdoses may present with tachypnea
- may have abdo pain i.e with iron ingestion
ddx of vomiting and altered mental status
- DKA
- toxic ingestion
- GI obstruction
- increased ICP
- gastroenteritis
- appendicitis
- bacteria pna
- pyelonephritis
what is cushings triad
sign of increased ICP
- hypertension (progressive), widening pulse pressure
- bradycardia
- irregular breathing
what are the diagnostic criteria for DKA
- random blood glucose of over 11.1 mmol/L
- a venous pH below 7.3 or serum bicarb less than 15mmol/L
- moderate or large ketouria or ketonemia
how do you manage insulin in DKA
insulin drip 0.1 unite/kg/hour started after patient received initial volume expansion
should you give bicarb to manage DKA
no
what is the most common cause of diabetes associated death in kids
cerebral edema
should you give an insulin bolus in kids? why or why not?
no
increased risk of cerebral edema from rapid correction
why might you have a white count in DKAq
left shift from stress of DKA alone or from precipitating infection
do you get DKA only in T1DM
most commonly but can get it also in T2DM
what symptoms might make you think DKA
vomiting weight loss dehydration shortness of breath abdominal pain change in level of consciousness
how would the following be affected in DKA:
pH on venous blood gas
decreased
due to metabolic acidosis due to ketoacids
how would the following be affected in DKA:
serum sodium
decreased
hyponatremia from osmotic movement of water into the extracellular space in response to hyperglycemia and hyperosmolarity (dilutional hyponatremia) as well as from increased renal sodium losses
calculate corrected sodium and monitor with therapy
how would the following be affected in DKA:
potassium
lab value–often normal
total body sodium–depleted
acidosis and low insulin drive K out of cells into blood–as you correct the acidosis her serum K will drop leading to hypokalemia
must anticipate this drop and monitor K
usually add K to IVF after initial volume expansion and after urine output is established unless there are ECG changes suggestive of hyperkalemia
how would the following be affected in DKA:
bicarbonate
decreased
due to metabolic acidosis
how would the following be affected in DKA:
creatinine
elevated
even in context of normal renal function, can be elevated due to severe dehydration (prerenal azotemia)
how would the following be affected in DKA:
serum ketones
elevated
how would the following be affected in DKA:
urine ketones
elevated
what are the three types of dehydration
isotonic/isonatremic
hypotonic/hyponatremic
hypertonic/hypernatremic
what is isotonic/isonatremia dehydration
most common type in kids
occurs when sodium and water losses are balanced (including those with acute gastroenteritis and diarrhea)
typically deficit can be replaced within 12 hours
what is hypotonic/hyponatremic dehydration
occurs when sodium losses exceed those of water
usually when patients consume diluted fluids or water in the face of dehydration
hyponatremia may be the result of adrenal insufficiency
typically replaced over 12 hours
*rapid correcttion of hyponatremia is associated with central pontine myelinosis
what happens is you correct hyponatremia too quickly
central pontine myelinosis
–> damage to myelin sheath of CNS nerve cells in brainstem
what is hypertonic/hypernatremic dehydration
when water losses exceed that of sodium
associated with the highest mortality
can be due to breastfeeding failure, use of inappropriate rehydration solutions (boiled milk) and diabetes insipidus
typically replaced over 24 hours
what happens if you correct hypernatremia too fast
cerebral edema
what are the steps of fluid management
- provide bolus to restore intravascular volume
- correct dehydration
- provide maintenance fluids
- replace ongoing losses
how do you bolus to restore intravascular volume in a dehydrated patient
10-20 mL/kg bolus of 0.9% saline (NS)
NOT DKA–> serial boluses until patient urinates
IN DKA–> urinate very quickly due to osmotic diuresis, therefore monitor vital signs (normalized HR and BP) and mental status
how do you correct dehydration in a dehydrated patient
dictated by your assessment of severity of dehydration
how do you provide maintenance fluids in a dehydrated patient
replace daily insensible losses
1/4 normal saline or 1/2 normal saline with 5-10% dextrose usually used
approx 2 mL/kg/hr for kids under 15 kg and 1mL/kg/hr for kids above 15kg
list signs/symptoms of cerebral edema
- headache
- recurrence of vomiting
- bradycarida
- rising BP
- hypoxia
- restlessness, irritability
- increased drowsiness (lethargy)
- CN palsies–> CN VI
- abnormal pupillary response–> unequal, fixed dilated, absent responses unilaterally or bilaterally
what should admit orders include for DKA
- continuous monitoring of vitals
- hourly neuro checks
- monitor ins and outs
- insulin orders
- serum glucose every 60 minutes
- serum calcium, magnesium, and phosphorous now
- check serum pH (VBG) every 60 minutes
- urine dipstick for ketones
patients with T1DM are at risk for what other diseases?
other autoimmune related diseases with autoimmune thyroid disease and celiac disease being the most common
should have annual thyriod fxn test, screen for celiac at diagnosis
what do all insulin regimens consist of
- basal insulin–intermediate to long acting to suppress hepatic glucose production and maintain normoglycemia in fasting state
- prandial insulin–short acting to take before meals to cover carbohydrate load from food intake
peak age of T2DM dx
ages 12-16
what bruises raise the suspicion of non accidental bruising
bruising over well-cushioned areas of the body–buttocks, back, genitals
can bruises be aged based on color
no
what causes lyme disease
tick borne Borrelia burgdorferi
what is the characteristic rash associated with lyme disease
erythema migrans –> looks like a bullseye
symptoms of lyme disease
erythema migrans plus fever, malaise, fatigue, headache
how do you dx lyme disease
clinically, with rash etc…
what are the complications of lyme disease
arthritis is second most common manifestation of lyme disease–begins about 4 weeks after skin lesion
large joints closest to rash are most commonly affected
may relapse or recur only once
risk factors for DDH
female
breech delivery
family history of DDH
list 4 predictors of septic arthritis of the hip
fever
non weight bearing
ESR above 40
WBC above 12
1 predictor is 10% probability
4 predictors is 93% probability
fever was the best predictor
CRP above 20 was a strong risk factor
how do you dx septic arthritis of the hip
US of hip useful for ID of effusion and for guiding needle aspiration
most common causative organisms for septic arthritis
staph aureus
strep
Hib in unimmunized kids
N. gonorrhea
kingella kingae
tx of septic arthritis
empiric IV abx coverage should begin right after joint aspiration
may require surgical incision and drainage to remove debris and reduce pressure
requires prolonged abx and may require repeat arthrocentesis or surgical drainage
what is transient synovitis of the hip
relatively common condition resulting from inflammation and swelling of the tissues around the hip joint and can often be seen in kids who have recently had a cold
what causes transient synovitis of the hip
unknown
most have an URTI with it–?post infectious viral syndrome
tx for transient synovitis of the hip
rest and ibuprofen
duration of symptoms of transient synovitis of the hip
3-10 days
ddx of acute refusal to walk
more likely-- leukemia osteomyelitis reactive arthritis septic arthritis transient synovitis trauma
less likely-- JIA SCFE legg calve perthes avascular necrosis of the femoral head
what should always be considered in a child refusing to walk
leukemia
replacement of bone marrow by leukemia cells can cause bone pain that presents as limp, refusal to walk or localized discomfort of the jaw/long bones/vertebral column/hip/scapula or ribs
may preceed systemic signs such as fever and weight loss
most common cause of OM
staph aureus
what is reactive arthritis
inflammatory process assoc with infection outside of the joint–most often GI or GU
what are the infections outside the joint most commonly assoc with reactive arthritis
GI
GU
what is the typical presentation of transient synovitis
acute onset of hip pain with prehaps no other constitutional symptoms
what is JIA
group of disorders characterized by chronic inflammation of the joints
what are the diagnostic criteria for JIA
- less than 16 years old
2. arthritis in at least one joint for more than 6 weeks
what are the subtypes of JIA
- systemic–> constitutional sx such as fever and rash
- oligoarthritis–> usually knee
- polyarthritis (Rh positive and Rh negative)
- psoriatic arthritis
- enthesitis related arthritis
other arthritis
what is the most common hip disorder in adolescents
SCFE
what is the most common presentation for SCFE
months of vague hip or knee symptoms and limp with or without an acute exacerbation
what is legg calve perthes
avascular necrosis of the capital femoral epiphysis
most commonly affects boys between ages of 4 and 10
typical presentation of legg calve perthes
indolent or chronic pain rather than acute
natural history of legg calve perthes
typically self resolving but may lead to complications includng femoral head deformity and degenerative arthritis
treatment for legg calve perthes
refer to ortho
prevent damage to hip by containing femoral head within acetabulum
what avascular necrosis of the femoral head
necrosis of the bone due to loss of blood supply
traumatic or non traumatic
ibuprofen pediatric dose
10mg/kg q6-8h max dose 40mg/kg/24 hour PO
what type of headache presents as: episodic worsens thru day mild to moderate intensity band around the head or occipital area with accompanying tenderness to posterior muscle of the neck
tension headache
what are the most common cause of recurrent headache in kids
migraine
what should be considered in a child with
cyclical vomiting
benign parxysmal vertigo
migrains
can also have abdominal migraines
common migraine precipitants
stress
bright lights
odors like perfumes
foods
are migraines relieve by sleep
yes commonly
what should you be concerned about in a patient with headaches that occur agyer a period of recumbency (i.e early morning) and may be accompanied by and relieved after forceful vomiting?
increased ICP
what should you be concerned about in a patient with headaches that awaken them from sleep
increased ICP
what should you be concerned about in a patient with headaches, photophobia and fever
infectious etiology like meningitis or encephalitis
what should you be concerned about in a patient with headaches that worsen with cough or valsalva
increased ICP
what should you be concerned about in a patient with headaches that progressively worsen
red flag for serious pathology
list findings associated with allergies
- allergic shiners
- allergic salute
- dennie’s lines
- cobblestoning
define allergic shiners
darkness and swelling beneath the eyes due to sinus congestion
define allergic salute
frequent upward rubbing of the nose to alleviate itching leads to transverse nasal crease along lower third of nose
define dennie’s lines
infraorbital transverse creases–> assoc with mild chronic inflammation of the conjunctivae
define cobblestoning
fine granular appearance of the palpebral conjunctivae resulting from edema and hyperplasia of the papillae
cobblestoning of the pharynx occurs with chronic nasopharyngeal drainage of allergic rhinitis –> lymphocytic hyperplasia
ddx acquired ataxia
- post infectious cerebellitis
- infectious cerebellitis
- meds or toxins
- intracranial mass
- opsoclonus-myoclonus syndrome
- migraine headache
- hydrocephalus
- metabolic disease
- neurodegenerative disease
- psychiatric illness
what is the most common cause of acute ataxia in kids
post infectious cerebllitis
what is post infectious cerebellitis
causes ataxia
diagnosis of exclusion in kids 1-3
autoimmune response leading to cerebellar demyelination
several weeks after viral infection i.e varicella
onset sudden–> ataxia, vomiting, nystagmus in about half of patients, dysarthria in some
CSF may be normal or have pleocytosis… protein may be elevated
majority recover within a few months
most typically assoc with fevers or other systemic manifestation
what is infectious cerebellitis
causes ataxia
viral or bacterial
fever often
mental status changes seen
i.e pumps, enteroviruses, EBV
where might a tumour be located causing ataxia
cerebellum or frontal lobe
what is opsoclonus-myoclonus syndrome
paraneoplastic syndrome that occurs most often with neuroblastoma–> usually in younger kid 6mo - 3 years
ataxia accompanied by intermittent jerking movements and erratic, jerky conjugate movements of the eyes (opsoclonus)
what migraines can cause ataxia
basilar artery or hemiplegic migraines –> intermittent episodes of acute ataxia
may also have intermittent loss of vision, speech change, headache, vomiting
how does hydrocephalus present
ataxia that is generally insidious and chronic with increasing loss of coordination over weeks to months
often with headache and vomiting
what metabolic disturbances can cause ataxia
PKU
maple syrup urine disease
what neurodegenerative diseases of childhood cause ataxia
ataxia-telangiectasis
friedrich ataxia
most kids are younger than 10 and sx are loss od devel milestones, ataxia and other neuro sx
what do lesions in the vermis of the cerebellum cause?
vermis is midline
causes truncal ataxia, dysarthria, gait abnormalities
what do lesions in the cerebellar hemispheres cause?
ipsilateral limb abnormalities, nystagmus, tremor/dysmetria and tend to spare speech
what do lesions in the deep nuclei of the cerebellum cause?
resting tremor, myoclonis, opsoclonus (similar to neuroblastoma)
what is the most common childhood cancer
leukemia
what is the second most common childhood cancer
brain tumour –> highest among childhood cancer deaths
in what age range are brain tumours most common
younger age group–less than 7
when are the peaks of incidence for brain tumours
first decade of life and 8th decade
what are the risk factors for childhood brain tumours
exposure to ionizing radiation
certain genetic syndrome–tuberous sclerosis, neurofibromatosis, li-fraumeni syndrome
what are the most common brain tumours
- medulloblastoma–20%
- juvenile pilocytic astrocytoma–20%
- low grade astrocytoma–15%
- high grade astrocytoma–7%
what is a medulloblastoma
most common pediatric brain tumous
malignant tumour that may spread throughout the CNS and is capable to metastasizing to extracranial sites
tx for medulloblastoma
treatment generally includes surgical resection, radiation and chemo
prognosis for medulloblastoma
dependent on size and dissemination of the tumour
what is an astrocytoma of the cerebellum
best prognosis of all intratentorial tumours in kids
often have cystic component
tx for astrocytoma of the cerebellum
surgical resection
radiation reserved for patients with high grade astrocytomas, partial resections or with post op progression
prognosis for astrocytoma of the cerebellum
5 year survival is 90% with complete resection
drop to 50-70% with partial resection
what is an ependymoma
arise from within fourth ventricle (ependymal lining) and cause symptoms related to hydrocephalus
treatment for ependymoma
surgical resection, then radiation
prognosis ependymoma
5 year survival approx 50%
what is a brain stem glioma
may be either quite aggressive–> diffuse infiltration of the pons//or low grade–> focal tumour in midbrain or medulla
treament brain stem glioma
surgical resection alone for low grade gliomas
prognosis brain stem glioma
quite grave to quite good–range
most common complications of brain tumours
neurocognitive defects ADD learning disabilities endocrine abnormalities stroke
best imaging for brain tumour
MRI
best imagine for cranial hemorrhage
CT
why do we care about testicular torsion
urologic emergency in which the goal is to save the affected testes
when does testicular torsion usually occur
early adolescence
how does testicular torsion present
acute onset of severe hemi scrotal pain with N/V
physical exam findings in testicular torsion
enlarged tender testis, scrotal edema, absence of cremasteric reflex
treatment of testicular torsion
surgical exploration and detorsion promptly
irreversible changes to the testis can occur within hours
what is the most common condition requiring emergency surgery in the pediatric population
appendicitis
is appendicitis common in children under 2
no
what fraction of paeds patients have atypical presentations of appendicitis
1/3–therefore high incidence of perforation in this pop
who is at highest risk for PID
sexually active females between 15-19
why are sexually active females between 15-19 at highest risk for PID
fewer protective antibodies in the vagina compared to older women
cervical ectropion (transition zone between columnar and squamous epithelium) not fully matured–> cells here are particularly prone to STDs and thus cervix easier to infect
behavioural–multiple sexual partners, intercourse during menses, infrequent or no condom use
which organisms most commonly cause PID
N. gonorrhea and C. trachomatis (more than 50% of all cases)–lower tract infection with these leads to alteration in the normal vaginal flora and allows bacteria like ecoli, bacteroides and other anaeobes, mycoplasma or ureaplasma access to the uterus and fallopian tubes
how do you dx PID
cervical motion tenderness
abdo pain
cervical discharge
cx for bacteria
molecular dx testing on urine or cervical discharge for chlamydia and gonorrhea
complications from PID
increased rates of infertility
sepsis
tubo-ovarian abscess
intra-abdominal abscesses
when should you think about doing a rectal exam
if there is an abdo complaint
part of an in depth neuro exam
ddx of abdo pain and vomiting
- appendicitis
- cholecystitis
- ectopic pregnancy
- hepatitis
- pancreatitis
- PID
- UTI
- acute gastroenteritis
- incarcerated hernia
- mesenteric adenitis
- ovarian torsion
- pneumonia
classic pattern of appendicitis
periumbilical pain followed by generalized RLQ pain
vomiting very common, diarrhea uncommon
descrieb cholecystitis pain
RUQ, steady, may radiate to shoulder
usually worse after eating, especially fatty foods
may be intermittent/colicky and accompanied by low appetite and N/V
less common in kids than adults
what should you always consider in a sexually active female with abdo pain
ectopic pregnancy because it is an emergency
how does ectopic pregnancy present
lower abdo pain, vaginal bleeding, abnormal menstual hx
fever and diffuse abdo pain are uncommon
vomiting without diarrhea suggests extra-intestinal pathology
what does band-like abdo pain radiating to the back suggest
pancreatitis
N/V almost always present
what is an incarcerated hernia
hernia that can no longer be reduced to its usual position with manipulation
most present before 1 year of age, slightly more in girls
what is mesenteric adenitis
inflamm of mesenteric lymph nodes
often presents like appendicitis
typical presentation of mesenteric adenitis
RLQ pain with fever, vomiting and diarrhea
when would you hospitalize a patient with PID
pregnant
previous noncompliance
high fever
intractable vomiting
inability to exclude a surgical emergency
when is fever an emergency
infants younger than 6-8 weeks
list the exam findings of bacterial meningitis
nuchal rigidity–57-92%
fever–66-100%
altered mental status–44-96%
kernigs or brudzinskis–61%
do you diagnose shock in kids based on BP?
no–kids can maintain normal BP until in profound shock
how do kids compensate for shock
- increased HR
- increased systemic vascular resistance–vasoconstriction
- increased heart contractility, with more complete emptying of ventricles
- increased venous tone–> more blood return to heart
- increased resp rate–compensate for metabolic acidosis from decreased oxygen perfrusion of cells and tissues
signs of early shock in kids
elevated HR and RR
peripheral vasoconstriction (cool, clammy extremities and delayed cap refill)
decreased peripheral pulses
**hypotension is sign of late shock
name 2 types of distributive shock
neurogenic
anaphylactic
characteristics of distributive shock
intravascular hypovolemia due to vasodilation, increased capillary permeability and third space fluid loss
what is the most common type of shock
hypovolemic
what causes hypovolemic shock
inadequate fluid intake to compensate for fluid output
signs of hypovolemic shock
mental status change
tachypnea
tachycardia
hypotension
cool extremities
oliguria
what conditions are associated with cardiogenic shock
severe CHD
dysrhythmias
cardiomyopathy
tamponade
signs of cardiogenic shock
cool extremities
delayed cap refill
hypotension
tachypnea
increasing obtundation
decreased urine output
what causes septic shock
infectious organisms release toxins that affect fluid distribution and cardiac output
can be bacterial, viral or fungal in immunocompromised patient
how do you treat septic shock
require repeat fluid bolusing
may also need pressors and dopamine
symptoms of warm shock
warm extremities
bounding pulses
tachycardia
tachypnea
adequate urination
mild metabolic acidosis
complications of meningococcal disease
hearing loss
neurologic disability
digit or limb amputations
skin scarring
what is a sensitive measure of volume status
HR
tachycardia is the first and most subtle sign of possible inadequate perfusion
what is a sensitive sign of hypovolemia
cap refill
ddx lethargy
- sepsis
- meningitis
- encephalitis
- toxic ingestion
- pneumonia
- CNS tumour
- DKA
- hypoglycemia
- renal failure
what often causes encephalitis in kids
virus
what diagnosis must be at the top of the list in a patient with fever and petechiae
meningococcal sepsis
blood cx must always be done until disease ruled out
describe the mucocutaneous leasions of kawasaki disease
associated with fever and rash
- “strawberry” tongue
- dry, red, cracked lips
- diffuse erythema of the oral cavity
- erythema and/or edema of the hands/feet
- polymorphic truncal rash
what disease is associated with strawberry tongue
kawasaki
presentation of toxic shock syndrome
fever with sun-burn looking rash that is rough to touch like sandpaper
where are the petechiae of rocky mountain spotted fever
palms and soles
how does scarlet fever start
finely punctate pink-scarlet exanthem
rash appears on upper trunk 12-48 hours after onset of fever
as exanthem spreads to extremities, becomes confluent and feels like sandpaper
fades in 4-5 days followed by desquamation of the skin
linear petechiae evident in body folds–> pastia’s sign
what are the linear petechiae found in body folds in scarlet fever called
pastia’s sign
describe the appearance of the pharynx in scarlet fever
beefy red and tongue is initially white and rough (strawberry tongue also)–> white coating sloughs in 4-5 days and tongue is bright red
what is the priority in the management of shock
intravascular fluid replacement with ISOTONIC (NS) solutions
when should you place an IO line
if you cant get IV access when needed within 90 sec
apart from repeat fluid boluses, how can you manage shock
with inotropes to increase contractility and pressors to increase BP
list common procedures for kids with sickle cell
- tonsillectomy
2. cholecystectomy
why do kids with SCD get tonsillectomies
lymphoidal tissue hypertrophy involving waldeyer’s ring is common in kids with SCD
may see excessive snoring or OSA
tonsillectomy with adenoidectomy will improve the obstructive apnea for most patients
why do kids with SCD get cholecystectomies
bilirubin gallstones form frequently in all patients with hemolytic anemias because of increased Hb breakdown
more common in adolescents
only for those who are symptomatic
preferably elective
why are infants and young kids with SCD at increased risk of sepsis? what do you do about it?
decreased splenic function leads to decreased resistance to infection with encapsulated organisms (strep pneumo, Hib, neisseria meningitidis)
given penicillin as decreases risk of death from overwhelming sepsis in kids with SCD/sickling disorders
list expected complications for kids with SCD
- jaundice
- anemia
- stroke
- resp problems
what is the risk of stroke with SCD
10% by age 15
what is acute chest syndrome
vaso-occlusion in the lung parenchyma from SCD
med emergency
give supplemental O2 and transfusion therapy
why might growth be impaired in a kids with sickle cell
chronic anemia poor nutrition painful crises endocrine dysfunction poor pulmonary function
what should you look for in particular in the exam for a kid with SCD
- splenic enlargment
- sclera (icterus from hemolysis)
- neuro exam (stroke)
what is splenic sequestration crisis
in kids with SCD
indicated by a massive enlargement or rapid change in size of spleen
accompanied by increased pallor, fatigue, irritability
life threatening
occurs when blood pools in spleen and leads to severe anemia and shock
what happens to the spleen over time in SCD
progressively fibrotic
by time 4-6 years old, no longer palpable (kids with hemoglobin SC or Sbeta thalassemia can have splenic enlargment into teens)
what is a typical baseline Hb in SCD
60-90
what should you think in a kid with SCD and cough/breathing difficulty
pneumonia
intrapulmonary sickling
pulm fat embolism
–all of these are covered by the term acute chest syndrome
must address possibility of infection, pulm and resp complaints and the pain
acute chest syndrome on xray
multilobal infiltrates
effusions
atelectasis
pericarditis on xray
effusion and infiltration
what is the only way to directly reduce or reverse the sickling process that is the undelrying cause of acute chest syndrome
rbc transfusion
when do you transfuse in acute chest syndrome in SCD
consider when…
- fall in hb from baseline
- increasing resp rate
- worsening chest symptoms
- declining O2 sat
- progressive infiltrates on CXR
when do you seek emergency care for kids with SCD
- fever–medical emergency in kid with SCD because sometimes only sign of serious infection
- splenic enlargment
- slurred speech–stroke?
- chest pain
- rapid breathing
- increased pallor
- increased jaundice
- priapism
how does seasonal allergic conjunctivitis present
periorbital swelling
may be associated with pruritis and mucoid discharge
often assoc with sneazing, itchy nose, clear rhinorrhea
look for allergic shiners, dennie’s lines, cobblestoneing of conjunctiva
what is the most prominent symptom of nephrotic syndrome
edema
what is a common presenting finding of nephrotic syndrome
periorbital edema that improves when upright
edema then becomes more generalized–leads to pitting edema, ascites, weight gain
what causes the edema in nephrotic syndrome
more due to interstitial fluid accumulation rather than intravascular fluid overload (which happens in acute glomerulomephritis)
hypertension thus does NOT usually occur
do you expect hypertension in nephrotic syndrome
no
do you expect hypertension in acute glomerulonehpritis
yes
what is the most common form of nephrotic syndrome in kids
minimal change disease –90% of cases under age 10
what is the characteristic histologic finding in kids with minimal change disease
fusion and diffuse effacement of the epithelial cell foot processes on electron microscopy
nephron appears relatively normal on light microscopy
what are benign causes of proteinuria
fever
significant exertion
orthostatic proteinuria can be found in teens and doesnt mean renal disease–protein excretion only when standing, not recumbent (first morning urine is negative for protein)
what are the mechanisms for proteinuria in nephrotic syndrome
- loss of polyanion charge characteristic of a normal capillary wall–> loss of charge permits large negatively charged proteins like albumin (previously repelled) to leak through the membrane of the glomerulus
- shift in capillary wall pore size in a direction permitting increased leaking of large molecules
- change in hemodynamic characteristics of capillary flow so greater filtration of larger molecules
what diseases can cause proteinuria
any disease causing inflammation of the renal parenchyma (nephritis) can cause proteinuria
pyelonephritis
acute glomerulonephritis
interstitial nephritis
if you saw tea coloured urine what might you think if the cause of renal disease in that patient
acute glomerulonephritis (i.e post strep)due to RBCs and red blood cell casts
what lab test abnormalities would you expect in nephrotic syndrome
hypoalbuminemia (major loss of albumin in urine)
hyperlipidemia–> in reaction to decreased albumin, liver stimulated to make lipoproteins as well as albimun–> hyperlipidemia and hypercholesterolemia (also decreased clearance of lipids from blood)
how do you classify minimal change disease
steroid responsive or relapsing/steroid dependent or
steroid resistant
95% are in first two groups
how do you manage nephrotic syndrome in kids
kids between ages of 1-10 without gross hematuria and normal serum complement get trial of corticosteroids
most of them will be minimal change disease
where can generalized edema best be seen in the male and female patients
male-scrotum
female-labia
common features of down syndrome
- upslanting palpebral fissures
- small ears
- flattened midface
- epicanthal folds
- redundant skin on back of neck (nuchal skin)
- hypotonia (most consistent finding)
- predisposition to leukemia
- heart defects
- intestinal stenosis
- umbilical hernia
which form of down syndrome geentics is most likely
trisomy (versus translocation and mosaic)
how do you TEST for downs prenatally
chromosome analysis of amniotic fluid and chorionic villus sampling are both direct and diagnostic tests for downs because cells are of fetal origin
how do you SCREEN for downs prenatally
maternal serum screening and fetal US can potentially screen for them but not definitive
what is fragile X disease
caused by inheritance of abnormal number of CGG repeats in FMR1 gene
physical features of fragile X
long face with large mandible
large prominent ears
large testicles after puberty
what are the two most common genetic causes of intellectual disability
downs then fragile x
what is the sex chromosomal disorder most likely to be assoc with physical differences at birth
turners
what process happens in utero with turners girls
lymphedema
features of trisomy 13/patau
microcephaly microphthalmia severe intellectual disability polydactyly cleft lip and palate cardiac and renal defects umbilical hernia cutis aplasia
features of trisomy 18/edwards
severe intellectual disability prominent occiput micrognathia low set ears short neck overlapping fingers heart defects renal malformations limited hip adbuction rocker-bottom feet
what endocrine abnormality is common in downs
hypothyroid
ddx hypotonia in a newborn
- downs
- benign neonatal hypotonia
- zellweger syndrome
- family resemblance
what is zellweger syndrome
peroxisomal disorder
generally hypotonic and poorly responsive
test for downs
lymphocyte karyotype
define macule
flat, circumscribed discoloration less than 1cm
ie freckle
define patch
larger flat lesion of color change of the skin greater than 1cm
define papule
elevated, circumscribed solid lesion
less than 1cm
ie mole
define plaque
broad, elevated lesion (or confluence of papules)
greater than 1cm
define vesciel
circumscribed, elevated lesion containing clear colored fluid
less than 1cm
define bulla
larger, circumscribed, elevated fluid containing lesion greater than 1cm
define pustule
exudate containing lesion
variable size
define nodule
circumscribed elevated lesion that involves the dermis and may extend into the subcutaneous tissue
majority of nodules is below skin
define wheal
a blanching, edematous, thin, erythematous papule or plaque, often with a ring of hypopigmentation
may be white to ale red and often appears and disappears over a period of hours
define telangiectasia
dilation of superficial venules, arterioles or capillaries visible under the skin or mucous membrane
define petechiae
tiny, red or purple macules caused by capillary hemorrhage under the skin or mucous membrane
petechiae do not blanch with pressure
define purpura
larger, purple lesion caused by bleeding under the skin
may be palpable and do not blanch
what is kawasaki disease
fever of at least 5 days duration–> high, spiking, not responsive to antimicrobial or antipyretic agents
presence of at least 4 of the following principal clinical factors:
- polymorphous exanthem (face, trunk, extremities, perineal region)
- bilateral conjunctivitis (nonexudative, bulbar)
- changes in lips and oral cavity (dry, cracked lips and strawberry tongue)
- changes in extremities (erythema of palms and soles, edema of hands and feet, followed by 1-3 weeks later of desquamation of fingers, toes and perineal region in infants)
- cervical lymphadenopathy
in what kids should you consider kawasaki
child with persistent and unabating fever and
- no focus of infection
- no response to antimicrobial tx-or-
- other clinical and lab findings of kawasaki
what is erythema toxicum
idiopathic, asymptomatic, benign self limiting cutaneous eruption of full term newborns
blotchy, macular erythema
progresses to 1-3mm yellow/white papules and pustules
present from day 2-10 of life
no treatment needed
what is neonatal acne
assoc with saprophytic Malassezia species
not true acne
located on cheeks, chin, forehead
no tx necessary (topical antifungals can help)
what is milia
clustered small white papules over the nose
small retention cysts resulting from keratin within dermis
seen in 40-50% of infants
cheeks, nose, chin, forehead
newborn milia resolves in 3-4 weeks
what is neonatal herpes simplex
ranges from mild, self limiting illness to devastating neuro consequences
3 patterns:
SEM (skin eyes mucous membranes)
neuro disease
disseminates herpes
what is transient neonatal pustular melanosis
benign self limiting disorder of unknown etiology
superficial vesciculopustular lesions
rupture easily and evolve into hyperpigmented macules
most common in infants with black skin
inferior chin, forehead, neck, lower back, shins
pustular lesions disappear within 24-48 hours
hyperpigmented macules fade gradually over weeks to months
what is roseola
common childhood disease caused by HHV type 6 or 7
what is the classic presentation of roseola
high fever last 3-5 days
otherwise well infant
with reduced fever–> classic exanthem appears–> initially on trunk, spreads to extremities, neck, face
what infection has a slapped cheek appearance
erythema infectiosum
caused by parvovirus B19
how does erythema infectiousum present
prodrome of 5-7 days of headaches, fever, chills
rash
what disease causes a “dewdrops on a rosepetal” rash
varicella
how does varicella present
prodrome of fever, chills, malaise, headache, arthralgia, myalgia
skin lesions occur 24-48 hours after
red macules/papules that rapidly evolve into vesicles
older lesions crust over
v
various stages present at same time
how does the measles rash present
intensely erythematous patched of the face with cephalocauded spread onto the trunk and extremities
what causes measles
single stranded RNA virus in family paramyxoviridae
what is the measles prodrome
fever and the 3Cs: cough, coryza, conjunctivitis
koplik spots
what are koplik spots
punctuate, gray-white to erythematous papules distributed on the buccal mucosa
how does measles spread
cephalocaudad
what are some complications of measles
pneumonia bronchitis otitis gastroenteritis myocarditis encephalitis
what might you think in a patient with nonspecific “rose pink” macules and papules on the trunk of an adolescent male associated with lymphadenopathy and arthralgia
rubella
what causes rubella
RNA virus in the togaviridae family
what is the rubella prodrome
low grade fever headache malaise eye pain myalgias sore throat rhinorrhea cough
progresses to…
rash
generalized lymphadenopathy (suboccipital, postauricular, cervical regions)
arthralgias and arthritis common
describe the rubella rash
rose pink macules and papules becoming confluent
cephalocaudal spread and resolution
why do we vaccinate against rubella
prevent fetal infection
what is sturge-weber syndrome
facial bright red rash with glaucoma and seizures
neuroectodermal syndrome
triad:
- port wine stain in V1 distribution
- seizures
- glaucoma
requires multidisciplinary tx
what are the 7 diagnostic features of NF1
- six cafe au lait macules that measure 0.5cm or more before puberty or 1.5 cm or more in diameter in adults
- freckling of the axillary and/or inguinal areas
- plexiform neurofibroma or two or more dermal neurofibromas
- two or more lisch nodules
- optic nerve glioma
- pathognomonic skeletal dysplasia (tibial or sphenoid wing dysplasia)
- affected first degree relative
what is tuberous sclerosis
autosomal dominant disorder
characterized by development of hamartomas of the skin, brain, eye, heart, kidneys, lungs and bone
list the major criteria for tuberous sclerosis dx
facial angiofibroma ungual fibroma shagreen patch hypomelanotic macule cortical tuber subependymal nodule subependymal giant cell tumour retinal hamartoma cardia rhambomyoma renal angiomyolipoma lymphangiomyomatosis
what is ataxia telangiectasia
can present with dilated vessels over the conjunctiva associated with progressive ataxia and immunodeficiency
what is ataxia telangiectasia
- ataxia
- -begins in infancy
- -truncal ataxia progresses to axial ataxia - telangiectasias
- -characteristically conjunctival - other features
- -immunodeficiency
- -predisposition to malignancies
what is incontinentia pigmenti
x linked disorder that affects the skin, CNS, eyes and skeletal system
affects only women
what are the 4 distinct phases of incontinentia pigmenti
- inflammatory vesicles or bullae
- verrucous lesions
- streaks of hyperpigmentation
- streaks of atrophy/hypopigmentation
may have cicatricial alopecia and nail dystrophy
also dental, ocular and CNS manifestations
peripheral eosinophilia in 70% of patients
lasts 4-5 months
what is the most common form of allergic contact to metal
nickel dermatitis
where do you often see nickel dermatitis
subumbilical eruption; earring or other piercing
tx of nickel dermatitis
contact avoidance
text solution of dimethylglyoxime in a 10% aqueous solution of ammonia
what is eczema herpeticum
a severe, disseminated HSV infection that occurs in individuals with atopic dermatitis
complications of eczema herpeticum
keratoconjunctivitis
sercondary bacterial superinfection
fluid loss
viremia
how do you treat eczema herpeticum
recognition and systemic acyclovir
what is pityriasis alba
circumscribed scaly hypopigmented lesions on the cheek associated with atopic dermatitis
asymptomatic
face, neck, upper trunk, proximal extremities
non specific dermatitis with residual post inflammatory hypopigmentation
commonly seen in atopic dermatitis patients
what is wiskott-aldrich syndrome
rare x linked recessive disorder
classic features:
recurrent pyogenic lesions
bleeding from thrombocytopenia and platelet dysfunction
recalcitrant dermatitis
most patients die by 3 years of age without transplantation…low levels of IgM
what two hormones control the release of growth hormone
GHRH and somatostatin
how is GH secreted
in a pulsatile manner, occurring at stage 4 sleep
where does GH act
growth hormone receptor and causes some direct metabolic effects as well as the transcription of the IGF1 gene
where is IGF1 produced
liver and target tissues
what is IGF1 bound to in circulation
IGFBP
how does IGF1 act
binds to IGF receptors, activating tyrosine kinases and specific cell pathway
leads to cell growth by inducing the cell cycle machinery, inhibiting apoptosis and inducing cellular differentiation
list the growth hormone target tissues
liver growth plate adipose tissue muscle lymphocytes gonads
what does GH do in the muscle and liver
insulin antagonism
what does GH do in adipocytes
lipolysis
how does thyroid hormone affect growth
direct effect on epiphyseal cartilage
TH needed in order to release growth hormone from the pituitary
TH needed for POST natal growth only
how do sex steroids affect growth
act by augmenting GH pulses
do not play major role prior to puberty
early exposure to sex steroids results in rapid linear growth, skeletal maturation and premature fusion of the growth plates
define familial short stature
childs height falls on percentil appropriate for his mid parental height
normal growth velocity
bone age equal to the chronological age
define constitutional growth delay
children have growth deceleration between 18 months and 3 years
at 3 years they grow at normal rate
puberty is delayed as is their bone age
often history of later puberty or prolonged growth in a family member
name the common bone/cartilage disorders that affect growth
achondroplasia
hypochondroplasia
spondylo-epiphyseal dysplasia
name the chromosomal disorders associated with short stature
down syndrome turner syndrome noonan syndrome russel silver syndrome chromosomal deletion syndromes
define IUGR
height and weight less than 2 SD for the mean for gestational age
15% of these kids fail to achieve catch up growth
normally do not have abnormalities of the growth hormone axis
are IGF1 levels high, normal or low in malnutrition
low
how does hypopituitarism present in neonates
- hypoglycemia that requires 6-8 mg/kg/min of IV glucose to maintain euglycemia
- micropenis with or without undescended testes
- liver dysfunction secondary to giant cell hepatitis
- temperature instability
- associated midline defects (i.e cleft lip palate)
how does hypopituitarism present in childhood
- growth failure
- difficulty handling illnesses necessitating hospitalization for dehydration
- hypoglycemia
- polyuria and polydipsia
- symptoms of increased intracranial pressure if tumour is present
- visual disturbance
how do you assess for possible GH deficiency
stimulation tests to assess GH reserve: hypoglycemia arginine glucagon dopamine clonidine
how do you test ACTH reserve
stimulation tests:
glucagon stimulation
hypoglycemia
low dose ACTH stimulation
how do you test TSH reserve
free T4 or TRH test
how do you test ADH reserve
water deprivation
what are suprasellar tumours
cause hypopituitarism by invading the pituitary or stalk
craniopharyngiomals, gliomas of the optic nerve or hypothalamus and germinomas are most frequently seen
how do germ cell tumours often present
with diabetes insipidus in both sexes, or with precocious puberty in males
how can a UTI present in infants
N/V
irritability
poor feeding
sepsis
what bug usually causes UTI
ecoli
what other bugs can cause UTI
KEEPPSS
klebsiella ecoli enterococcus proteus pseudomonas strep staph saprophyticus
how does glomerular hematuria present
brown or “tea colored” urine
RBC casts, cellular casts
tubular casts
proteinuria on dipstick if no gross hematuria
dysmorphic erythrocytes
how does non glomerular hematuria present
red/pink urine
blood clots
no proteinuria on dipstick if no gross hematuria
normal erythrocytes
definition of hematuria
more than 5 RBC/hpf on urine microscopy
what are the 1. non renal 2. lower urinary tract 3. renal, non glomerular 4. renal, gomerular etiologies of hematuria
- non renal–fever, drugs, exercise, menses, coagulopathy
- lower urinary tract–infection, trauma, stones
- renal, non glomerular–hypercalciuria, stones, sickle cell, cysts
- renal, glomerular–post infectious glomerulonephritis, IgA nephropathy, HSP, HUS, SLE, familial thin basement membrane disease, alports
define proteinuria
more than 100mg/m2/day
what causes proteinuria generally
- increased glomerular permeability
- inadequate tubular reabsorption
- overflow proteinuria
what things can cause transient proteinuria
fever, exercise, seizures, stress
what things can cause glomerular proteinuria
minimal change disease FSGS membranous membranoproliferative SLE drugs diabetes
definition of nephrotic syndrome
proteinuria above 50mg/kg/day
hypoalbuminemia
hyperlipidemia
edema
list the examples of end organ damage from hypertension
- hypertensive encephalopathy
- cerebral infarction or hemorrhage
- retinal infarction or hemorrhage
- CHF with pulm edema
- acute renal failure
renal causes of HTN
glomerulonephritis
renovascular
acute and chronic renal failure
fluid volume overload
endocrine causes of HTN
CAH, cushings
hyperaldosteronism
pheochromocytoma
hypo/hyperthyroid
CV causes of HTN
coarctation
CNS causes of HTN
infections
neoplasm
drug causes of HTN
steroids
stimulants
what is acute urticaria
a rash that comes and goes changing almost as one watches
“hives”
often caused by a histamine release triggered by allergens like drugs, foods or pollen
can also be caused by viruses, or temperature
often dont know trigger
what is the atopic triad
family history of any of all three of the following
atopic dermatitis (eczema) asthma allergic rhinitis (hayfever)
what is seborrheic dermatitis
very common skin condition for infants
little to no harm from it and most grow out of it
in older patients is caused by fungus (malassezia)
treatment for seborrheic dermatitis in infants
baby oil and small brush to remove scales
frequency (daily) shampooing with gentle baby shampoo or prescription shampoo with hetoconazole or pyrithione zinc
low potency topical steroid cream or ketoconazole cream
what are the stages of acne
usually starts as comedones (open–blackheads, closed–whiteheads)
then become inflamed, which may lead to larger, erythematous lesions called papules and pustules
can continue to progress in severe cases and become nodulo-cystic acne
list 4 acne triggers
- make up (unless noncomedogenic)
- mechanical factors such as manipulation
- occlusion (i.e with some sports gear)
- overzealous cleaning
?high glycemia diets and acne
what type of hypersensitivity reaction is chronic nickel contact dermatitis
delayed type IV hypersensitivity
where is the most common site for impetigo
right below the nates (because of rubbing and colonization) but it can be anywhere
red sores that pop easily and leave a yellow crust
what bug causes impetigo
most common is staph aureus and staph pyogenes (GAS)
tx of impetigo
mild, localized–> treat with topical abx like mupirocin
watch out for abscess formation from MRSA
what genus of plant often causes acute contact dermatitis
toxicodendron genus–poison ivy, poison oak and poison sumac
list 4 side effects of topical steroid use
skin atrophy
telangiectasias
hypopigmentation
suppression of the hypothalamic pituitary axis
first line tx for head lice
1% permethrin lotion
what causes scabies
mmite–>sarcoptes scabiei
when is the most intense time of itching for scabies
at night
what is the common distribution pattern for scabies
wrists, elbows, fingers, toes
describe the common scabies lesion
5-10 mm curvilinear thread-like lesion (burrow) –infants often do not have burrows on presentation
tx for scabies
two applications of permethrin 5% cream one full week apart for all affected household members
cream applied at night before bed and washed off in morning
adults apply from neck down, infants from hairline down including behind the ears
what bug causes ringworm
fungus tinea corporis
describe the classic lesion of ringworm/tinea corporis
annular, well circumscribed, scaly plaque with raised border and a center that is brown or hypopigmented
lesions gradually enlarge and may coalesce with surrounding lesions
may be mildly pruritic or asymptomatic
dx of tinea corporis
clinical or with KOH wet mount exam of skin scrapings –> classic branches and rod-shaped septated hyphae
what causes tinea versicolor
yeast form of fungus malassezia globosa and others
what does tinea versicolor look like
rash of pink, brown or white lesions depending on the background color of the skin
fine scale
changes color
can be contagious –> some people are more susceptible than others
excess heat and humidity predispose to the infection
treatment of tinea versicolor
selenium sulfide lotion
tx for tinea capitis
griseofulvin over 6-8 week
what should you watch out for when treating tinea capitis?
significant allergic reaction called KERION–inflamed, weeping, boggy lesion that often requires treatment with oral steroids
what are the common causes of diaper rash
- irritant dermatitis
- diaper candidiasis
- bacterial infection
what is the most common cause of diaper rash
irritant dermatitis –> irregular areas of erythema with skin maceration on the convex surfaces of skin
treatment for irritant dermatitis diaper rash
keep diaper area clean and dry and use zinc oxide creams or ointments (barrier)
tx for diaper candidiasis
nystatin
name 3 conditions that can present as diaper rash
- seborrhea and other common inflammatory conditions
- zinc deficiency
- langerhans cell histiocytosis
focused hx for a rash
duration rate of onset location associated sx family history of similar sx whether the patients has any allergies any new exposures any previous treatments
on what skin surfaces does eczema usually present
extensor surfaces
ddx for pustular rashes
- staph folliculitis
- acne vulgaris
- hidradenitis suppurativa
- rosacea
- perioral dermatitis
where do you often find staph folliculitis
below waist or in groin area
which form of hyperbilirubinemia causes the most problems
unconjugated
define kernicterus
pathological term used to describe staining of the basal ganglia and cranial nerve nuclei by bilirubin
also described the clinical condition that results from the toxic effects of high levels of unconjugated bilirubin
what may happen to kids with kernicterus
lose suck reflex
become lethargic
develop hyperirritability and seizures
ultimately die
can also develop…
opisthotonus
rigidity
oculomotor paralysis
temors
hearing loss
ataxia
what is opisthotonus
abnormal posturing that involves rigidity and severe arching of the back with the head thrown backward
assoc with kernicterus
where does most of the bilirubin formed by the healthy newborn come from
75% from physiological breakdown of RBCs
hemoglobin from RBCs–> unconjugated bilirubin–> INsoluble in aqueous solutions–> binds to albumin on bloodstream
in liver–> bilirubin extracted by hepatocytes–> binds to cytosolic proteins and is then conjugated with glucoronide by uridine diphosphate glucoronyl transferase
conjugated bilirubin is soluble in aqueous solution and excreted into bile
**newborn infant lacks GI flora to metabolize bile which allows B-glucoronidase present in meconium to hydrolyze the conjugated bili back to unconjugated –> reabsorbed into blood stream –> binds to albumin (enterohepatic recirc)
define physiologic jaundice
total bili below 257 in full term infants who are otherwise healthy and have no other demonstratable cause for elevated bili
*almost all newborns have hyperbili but it is benign and self limited
usually noticed on day 2 or 3–> bili levels reach peak at day 3/4
what factors can promote increased enterohepatic circulation that results in physiologic jaundice
- increased bili production from breakdown of short lived fetal cells
- relative deficiency of hepatocyte proterins and UDPGT
- lack of intestinal flora to betabolize bile
- high levels of B-glucuronidase in meconium
- minimal oral intake in first 2-4 days of life–> low meconium excretion (especially with breastfed kids)
what is breastfeeding jaundice
happens early in first week of life and occurs when milk supply is relatively or absolutely low–> limited PO intake
“lack of breast milk jaundice” / “breastfeeding assoc jaundice”
low intake–> decreased GI motility–> promotes meconium retention
B-glucuronidase in meconium deconjugates bili –> unconj bili is reabsorbed via enterohepatic recirculation
breast milk production increases eventually
what is breast-milk jaundice
occurs in first 407 days but may not peak until day 10-14
NOT result of low breast milk volume
cause not completely understood–> ?B-glucuronidase present in breast milk deconjugates bili in intestinal tract
can persist for up to 12 weeks but total bili rarely concerning levels
what are the most common DAT (antibody) positive hemolysis conditions
Rh incompatibility
ABO incompatibility
incompatibilities with minor blood group antigens (less common)
when do infants get antibody negative hemolysis?
infants who have RBC membrane defects (spherocytosis) or RBC enzyme defects (G6PD or pyruvate kinase deficiency)
how does non-hemolytic red cell breakdown cause jaundice
same way as hemolysis but not hemolysis
occurs in extensive bruising from birth trauma, large cephalohematomas or other hemorrhage, polycythemia or swallowed blood during delivery
name metabolic errors that can cause neonatal jaundice
- Crigler-Najjar syndrome–> hyperbilirubinemia from decreased bili clearance from deficienct or absent UDPGT
- galactosemia
- hypothyroidism
in what populations is hyperbili most ocmmon
asian newborns then white then black
what other factors can contribute to hyperbili
prematurity
bowel obstructon
birth at high altitude
main carb in breast milk and formula
lactose
what % of calories in human milk is lipids
50%
what types of protein are in breast milk
whey and casein
what should you suspect in a healthy-appearing infant who develops jaundice, dark urine, and pale (acholic) stools between 3-6 weeks of age
biliary atresia
eval with fractionated bili (total and direct)
tx for biliary atresia
surgically when caught early–> Kasai procedure (anastamosis of the intrahepatic bile ducts to a loop of intestine to allow bile to drain directly into intestine)
normal urination on
- day 3
- day 6
- 3-4 times a day
2. 6-8 times a day
by what day should meconium have passed
day 3–> stools begin to appear yellow once meconium passed
normal stooling for day 6/7
3-4 stools per day tho some have more
signs of untreated congenital hypothyroid
prolonged jaundice lethargy large fontanelles macroglossia umbilical hermia constipation abdominal distention severe devel delay
what is gilbert’s syndrome and what symptom does it cause
reduced activity of the enzyme glucuronyltransferase
relatively common cause of harmless jaundice
enterovirus rash description
erythematous and maculopapular
palms and soles
can be petechial
late summer early fall
infectious causes of generalized LAD
- measles (marked generalized LAD and splenomegaly that may last several weeks)
- infectious mononucleosis–EBV or CMV
- HIV
- histoplasmosis
- toxoplasmosis
- mycobacteria (can also be localized)
noninfectious causes of generalized LAD
- lymphomas
- leukemia
- histiocytosis
- metastatic neuroblastoma
- rhabdomyosarcoma
what can cause unilateral cervical LAD
- kawasaki
- reactive node
- bacterial cervical adenitis
- cat scratch disease
- mycobacteria
in what diseases do you see strawberry tongue
strep pharygnitis
kawasaki
toxic shock
