peds102 Flashcards
ITP blood labs
isolated thrombocytopenia
what is bleeding in renal failure patients due to?
dysfunctional platelets
chronic granulomatous disease puts you at risk for what infections?
catalase-producing organisms like staph aureus; neutrophils filled with bacteria are classic finding because they can still chemotax and eat the bacteria but just not do intracellular killing
chronic granulomatous disease- what diseases are you susceptible to?
pneumonias and adenitis are common because theses are caused by staph
adenosine deaminase def classic finding on CBC
lymphopenia because this leads to lack of maturation of B and T cells
common organisms that you are at risk for with adenosine deaminase activity
PCP, candida, paraflu, and herpes virus
when does complement consumption happen (and lead to immunodef)
diseases like vasculitis, glomerulonephritis, and certain connective tissue diseases. SLE
C3 def exposes you to risk of what kinds of infections
encapsulated bacteria, begins shortly after birth
most common primary immunodef
B cell maturation arrest; puts you at high risk for infections with encapsulated bacteria;
types of infections diGeorge syndrome patiens get
fungi and viral since T cell dysfunction is the hallmark
tetrad of HSP
palpable purpura, arthralgias, abdom pain, renal disease
platelets in HSP
normal
meabolic acidosis
think ischemia
causes of orbital cellulitis
staph and streps
IUGR, diaphoretic, and high pitched cry. Fetus was exposed to what?
heroin
fetal hydrantoin syndrome
phenytoin
nail and digit hypoplasia, dysmorphic facies and mental retardation
fetal hydantoin syndrome
NT defects, cardiac abnormalities, dysmorphic facies like cleft lip, narrow bifrontal diameter, midface hypoplasia, broad and depressed nasal bridge, long philtrum
valproic acid
growth deficiency, long smooth philtrum, small palpebral fissures, thin upper lip, in addition to CNS abnormalities like mental retard.
fetal alcohol syndrome
jitteriness, excessive sucking, and hyperactive moro reflex
cocaine in utero
neonatal abstinence syndrome
neonate withdrawal from opiates in the first few days of life; high pitched cry, tremors, seizures, sweating, sneezing, vomitting, diarrhea
Lesch-Nyan syndrome
self mutilation in the presence of dystonia; X-linked recessive; def in HPRT enzyme, leads to increased uric acid
clinical features of Lesch-Nyan syndrome
all due to build up of uric acid; hypotonia, persistent vomitting, progressive mental retard, choreoathetosis, spasticity, dysarthric speech, dystonia and compulsive self-injury; gouty arthritis, tophus formation, and obstructive nephropathy
treatemnt for Lesch-nyan
allopurinol to reduce uric acid levels; patients should also drink lots of fluids
why are SGA infants at risk for polycythemia
increased EPO in response to hypoxia
myotonia
delayed muscle relaxation
autoimmune myositis with symmetrical proximal muscle weakness, cutaneous findings, arthralgias, and dysphagia
juvenile dermatomyositis
mental retard, global developmental delay, hyperactivity, and long narrow face, large ears and macroorchidism
fragile x syndrome
contraindic to breast feeding
active untreated TB, maternal HIV, herpetic breast lesions, varicells infection less than 5 days before; certain meds; chemo or XRT; street drugs or alcohol
hand-foot syndrome (aka dactylitis)
earliest manifestation of vaso-occlusion in sickle cell anemia; acute onset of pain and swelling of hands and feet; fever sometimes
pathophys of hand-foot syndrome in sickle cell disease
vascular necrosis of the metacarpals and metatarsals, which may be seen on xray as osteolytic lesions
when giving IV fluid boluses
only isotonic solutions such as nromal saline or lactated Ringer’s should be used
hypernatremic hypovolemia bolus?
normal salie; later on you will deliver (non-bolus) hypotonic saline
signs of hypernatremia
lethargy, altered mental status, irritability, and seizures
organisms that cause septic arthritis in birth to 3 month
staph, GBS, and gram neg bacilli
antibiotics for septic arthritis birth to 3 mos
antistaph (nafcillin or vanc) PLUS gent or cef
organisms that cause septic arthritis in older than 3 mos
staph, GABHS and strep pneumo
antibiotics for septic arthritis in greater than 3 mos
nafcillin, clinda, cef, or vanc
causes of anemia of prematurity
transitions in the erythropoeisis site of the neonate; shorter life span of RBCs in neonates; diminished fetoplacental transfusion
metoclopramide
for gastric motility
4 neonatal rashes
erythema toxicum neonatorium, neonatal HSV, neonatal varicella, staph scalded skin syndrome
scttered erythematous macules, papules and pustules throughout the body of a neonate but spares palms and soles
erythema toxicum neonatorium; no treatment
vesicular clusters on skin, eyes, mucous membrnaes, CNS infection, fulminent dissem multiorgan disease
neonatal HSV
fever, rash ranges from vesicular clusters on skin to fulminant dissem disease
neonatal varicella
fever, irritability, and diffuse erythema followed by blishtering and exfoliation, pos Nikolsky sign
staph scalded skin syndrom
treatment for SSSS
oxacillin, nafcillin, or vanc
treatment for neonatal varicella
acyclovir
diagnosis of ALL
bone marrow biopsy (not lymph node biopsy) showing greater than 25% lymphoblasts
herpangina
throat infection caused by enteroviruses, like coxsackie A; ulcerative lesions on palate, tonsils, and pharynx
when herpangina is accompanied by lesions on the palms and soles
hand-foot-mouth disease
GB affects which nerves
primarily peripheral motor but sensory can also be affected
initial sx of tingling in the foot, followed by ascening weakness; polyneuropathy; flaccid paralysis in final stage
G-B
CSF analysis in G-B
elevated protein levels and mildly elevated cells
treatment for G-B
plasmapheresis or human immunoglobulin
fanconi anemia
chromosomal breaks on genetic analysis
treatment of fanconi anemia
hematopoeitic stem cell transplant
aplastic anemia, abnormal thumbs, hypogonadism, hypo or hyperpigmented skin, ear findings
fanconi anemia
total anomalous pulm venous connection
cyanotic disease; blood from lungs goes into the RIGHT atrium and there is ALSO an ASD which means this partially oxygenated blood goes from RA to LA
decreased pulmonary markings on CXR
pulmonary undercirculation
holosystolic murmur loudest at LLSB
VSD
complete AV canal defect
assoc with down syndrome; ASD and VSD
ebstein’s anomaly
assoc with maternal lithium use during pregnancy; displacement of tricuspid valve into the RV; results in severe tricuspid regurg and RA enlargement
paucity of vascular markings on CXR
tet of fallot and tricuspid valve atresia
boot shaped heart
RV hypertrophy, from tet of fallot
truncus is assoc with what syndrome
digeorge
treatment of pinworm (enterobius vermicularis)
albendazole or mebendazole is first line treatment; pyrantel pamoate is an alternative; NOT metronidazole, thiabendazole, or ivermectin
acute drop in hemoglobin in a sickle cell patient can be caused by what?
hyperhemolytic crisis, splenic sequestration, or an aplastic crisis
aplastic crisis in sickle cell disease is not the same as aplastic anemia
aplastic crisis in SS does not have drops in other blood cells
splenic sequestraton can lead to
severe hypotensive shock; accompanied by persistent reticulocytosis; rapidly enlarging spleen; high mortality; splenectomy recommended
vaso-oclusive crisis
acute onset of pain; severe anemia and low retics are not seen in vasoocclusive crises
rash, leukopenia, and thrombocytopenia
measles
vitamin A reduces the morbidity and mortality rates or patients with measles through immune enhancement
measles
calyceal clubbin (blunting of calices) and focal parenchymal scarring
chronic pyleonephritis
HBeAg
protein marker of viral replication and infectivity
encapsulated bacteria
Hib, strep pneumo, neisseria meningitidis, GBS, klebsiella, salmonella (HSKNGS)
nitroblue tetrazolium (NBT) slide test abnormal
chronic granulomatous disease
treatment of chronic granulomatous disease
prevention of infection with daily bactrim and gamma interferon three times a week
wiskott-aldrich syndrome
x linked recess; eczema, thrombocytopenia, and recurrent infections with encapsulated germs; presents at birth;
blood findings in wiskott-aldrich syndrome
thrombocytopenia, low IgM, high IgA and IgE, moderateley reduced T cells and platelets
chediak-higashi syndrome
decreased degranulation, chemotaxis and granilopoeisis; neutropenia and giant lysosomes in neutrophils; treatm with daily bactrim and ascorbic acid
mild coagulopathy, peripheral and cranual neuropathy, hepatosplenomegaly, pancytopenia, partial oculocutaneous albinism, freq bacterial infections and progressive lymphoproliferative syndrome
chediak-higashi syndrome
Hyper-IgM syndrome
poor response to vaccines; less than 2% CD19+ B cells in the peripheral circulation
parinaud’s syndrome
paralysis of vertical gaze that may be assoc with pupillary disturbances and eyelid retracton (collier’s sign); commonly caused by germinomas and pinealomas; endocrine syndrome due to interruption of the hypothal inhib pways
treatment of lyme disease in kids less than 8
oral amoxicillin; it’s oral doxy in greater than 8 and not preg
hypotonia, flat face, upward slanted palpebral fissures, epicanthal folds, Brushfield spots, dysplasia of the pelvis, cardiac malformations, simian crease, hypoplasia of middle phalynx of 5th finger
downs syndrome
cleft lip, flexed fingers with polydactyly, ocular hypotelorism, bulbous nose, low-set malfromed ears, small abnormal skull, cerebral malform, micropthalmia
Patau’s syndrome (trisomy 13)
microcephaly, protruding metopic suture, cat-like cry, hypotonia, short stature, moonlike face, hypertelorism, bilateral epicanthic folds, mental retard
cri-du-chat syndrome, 5p deletion
microcephaly, prominent occiput, micrognathia, closed fists with index finger overlapping the 3rd digir and the 5th digit overlapping the 4th, and rocker bottom feet
edwards syndrome
when does chlamydia conjnctivitis present? Chlamydia pneumonia?
conjunctivitis days 5-14 of life; pneumonia age 4-12 weeks
treatmen of BOTH chlamydial conjunctivitis and pneumonia
14 day course or oral erythromycin, though there is a risk of hypertrophic pyloric stenosis with oral erythromycin
cause of solitary lytic bone lesions
langerhans cell histiocytosis
langerhans cell histiocytosis
clonal proliferation of langerhans cells (abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes); isolated bone lesions or multisystem disease
histiocytoses
abnromal proliferation of histiocytes (activated dendritic cells and macrophages); related to abnormal prolif of WBCs like leukemias and lymphomas
most common cause of hypertension in kids
fibromuscular dysplasia
polyuria and diaper candidiasis
red flags for DM type 1
sideroblastic anemia
body has iron available but cannot incorporate it into hemoglobin. Sideroblasts are atypical erythroblasts that have accum of iron in them
dysmorphic anemia
two pops or RBCs; commonly seen in transfusion patients
treatment of impetigo
topical mupyricin; oral erythromycin is an alternative (not topical erythro)
mupyricin
used for topical skin infections, including MRSA
imipramine, amitryptaline, desipramine
TCAs; effective for nocturnal anuresis
oxybutinin
anticholinergic that causes urinary retention; it can be used for use in combo with desmopressin to increae bladder capacity in kids with daytme incontinence
meningococcus meningitis can cause a petechial or purpruic rash
in 75% of patients
when do kids get the meningococcal vaccine
11-12 years old and then booster at age 16; at risk kids (asplenic) can get it as young as 2
when do kids get the rotavirus vaccine?
between 2 and 8 mos; first dose between 6 and 14 weeks; final dose not beyond 8 months
what kids get vision exam?
all kids 0-5 yo
testicular volume in prepubertal boys
less than 3 mL
increaed risk of male breast cancer
klinefelters
small firm testes
klinefelters
p24 antigen testing
detects the presence of the p24 protein of HIV
most common primary bone tumor in kids
osteosarcoma
second most common bone tumor in kids
ewing’s sarcoma
spiculated sunburst pattern of bone on xray
osteosarcoma
periosteal elvation known as Codman triangle
osteosarcoma
alk phos and LDH elevated in osteosarcoma
from turnover of damaged osteocytes
treatment of osteosarcoma
tumor excision and chemotherapy
osteolytic lesion with a periosteal reaction that produces layers of reactive bone giving onion skin appearance
ewing’s sarcoma
sclerotic cortical lesion on bone xray with central nidus of lucency
osteoid osteoma
how does hydroxylurea work to manage sickle cell crises
causes the bone marrow to make new blood cells with hemoglobin F
oligohydramnios can lead to flat facies and limb deformities
interesting
excessive consumption f cows milk
can lead to iron def anemia
beta thal
microcytic anemia
seborrheic dermatitis
cradle cap’ cause unknown
atopic dermatitis distribution in kids
face, scalp and extensor sufaces (different from distribution seen in adults)
first line treatement for pertussis
macrolide antibiotic like erythromicin, azithromycin, or clarithromycin
pansystolic murmur over left lower sternal border and diastolic murmur at the apex
VSD; the diastolic murmur is due to increased flow across the mitral valve
wide split and fixed S2, systolic ejection murmur in the left upper sternal border, and sometimes if it is large, diastolic murmur at LLSB
ASD
elevated 17-oh progesterone
CAH
loud P2
pulmonary hypertension
parotitis
think mumps
orchitis
think mumps
sore throat, hyperemic and edematous mucous membranes, chilitis, stomatitis, glossitis, normocytic normochrmic anemia, seborrheic dermatitis, and photophobia
riboflavin (vit B2) def
cheilitis
inflamm of the lips
stomatitis
inflamm of the mouth
impaired collagen synthesis and damaged connective tissue; ecchymosies, petechiae, bleeding gums, hyperkeratosis, Sjogren’s, arthralgias, and impaired wound healing
ascorbic acid def (vit C)
pellagra
niacin def (vit b3)
symmetric reddish rash in exposed areas of skin, red tongue, nonspec symptoms
pellagra
infantile and adult beriberi
thiamine (b1) def
fulminant cardiac syndrome with cardiomegaly, tachycardia, cyanosis, dyspnea, and vomiting
thiamine def (infantile beriberi)
photophobia, drya scaly skin, dry conjunctiva, dry cornea, and a wrinkled cloudy cornea; bitot spots and follicular hyperkeratosis of the shoulders, butt and extensor surfaces are less common findings
vit A def
anorexia, pruritis, lack of weight gain, incr irritability, limitation of motion, tender swelling of bones, alopecia, seborrheic cutaneous lesions, incr ICP, hepatomegaly
hypervitaminosis A (ingestion of excessive vit A)
crigler-najjar
inherited def of UDP-glucuronyl transferase that results in unconj hyperbilirubinemia; patients die in the first year
gilbert’s syndrome
inherited def in UDP glucouronyl transferase and leads to unconj hyperbilirubinemia
ankylosing spondylitis
inflamm of the spine that can lead to fusion
spondylolisthesis
forward displacement of a vertebra, commonly in lumbar spine; results in back pain and neuro disfunction
kids must be at least 2 kg to get vaccines
remember in premies
sinopulm infections and intermittent episodes of diarrhea since childhood
igA deficiency, since IgA is involved in mucosal barrier protection
why are patients with iga def at risk for anaphylactic transfusion reactions?
they form antibodies to iga that they get from the transfused blood
hypoparathyroidism, thymic aplasia, hypocalcemia, cleft palat, and congenital heart disease
digeorge syndrome
predisposes to fungal infections
digeorge because T cell def
sinopulm infections and pseudomonas
x linked agammaglobulinemia (bruton’s)
x linked combined B and T cell disorder
wiskott-aldrich
recurrent otitis media, eczema, and thrombocytopenia
wiskott aldrich
recurrent resp infections, sinus disease, pancreatic insuff, and infertility
CF
elevated IgM and depressed IgG and IgA
hyper-IgM syndrome
susceptible to Giardia and recurrent sinopulm infection, lymphoid hyperplasia common
hyper-igM
first line drugs for primary generalized tonic clonic seizures or partial seizures with ot without secondary generalization
phenytoin and carbamazepine
first line agent for mixed seizures
lamotrigine
drugs for absence seizures
ethosuximide and valproic acid
side effects of phenytoin
gingival hypertrophy, lymphadenopathy, hirsutism, and rash
side effect of phenytoin and carbamazepine in the first several weeks of therapy
stevens johnson syndrome and toxic epidermal necrolysis
aut dom mutation of COL1A1
osteogenesis imperfecta
osteopenia, with blue sclerae, recurrent fractures, easy bruisability, hypotonia, and hearing loss
OI
dentinogenesis imperfecta
blue gray to yellow brown discoloration caused by dentin shining through the weak enamel in OI
aortic root dilation
marfan syndrome
retinal hemorrhages and subdural hematomas
child abuse
most common affected sites in NEC
terminal ileum and colon
feeding intolerance, incr abdominal girth, bloody stools, and vomitting
classic features of NEC
pneumatosis intestinalis on abdominal xray
NEC
bloody stools, uncommon in the neonate
intussception
“target sign” on xray or US of abdomen
intussusception
vomitting and abdominal pain n neonates less than 1 month
malrotation with midgut volvulus
gasless abdomen on xray
malrotation, due to obstruction in the duodenum
toga virus
rubella
rubella transmission in the first 4 weeks of pregnancy
risk of CRS is 50%, but drops to % if transmission in the third trimester
IUGR, deafness, PDA or ASD, micropthalmia, cataract, retinopathy, hepatosplenomegaly, thrombocytopenia and CNS involvement
congenital rubella syndrome
purple skin lesions
blueberry muffin baby rubella
port wine stain on the trigeminal nerve distribution, mental retard, seizures and glaucoma
sturge-weber
hydrocephalus, cerebral calcification, chorioretinitis, microcephaly, and micropthalmis
CMV and toxo
lymphedema, high-arched palate, congenitally bicuspid aortic valves, coarctatin of the aorta
turners syndrome
anaphylaxis
ige- mediated type 1hypersensitivity reaction
post-exposure prophylaxis for varicella for patients greater than 1 yr who are immunocomp
varicella vaccine
post-exposure prophylaxis for immunocomp hosts
varicella zoster immunoglobulin within 10 days of exposure; can reduce disease severity
how can H-S lead to intussuscepton
causes a hematoma that acts as a lead point
periodic abdominal pain assoc with drawing the legs up toward the abdomen, emesis may follow these episodes
intusussception
currant jelly stools
intussception that is not treated and causes ischemia; currant jelly is grossly bloody
tubular sausage shaped mass in the RUQ
intussusception
what imaging to detect intussusception
ultrasound
most common organism in osteomyelitis
staph aureus; also GBS and e coli in infants and strep pyogenes in children
osteomyelitis assoc with prosthetic devices. What organism?
staph epidermis
osteomyelitis when patient has a history of UTI. What organisms
klebsiella and pseudomonas
air xray for bronchiolitis
air trapping or atelectasis may be seen
ribavirin
administer to kids at high risk for RSV
contraindic to rotavirus vaccine
history of intussusception, history of uncorrected congenital malform of the GI tract like meckel’s; severe combined immunodef
at what age is the rotavirus vaccine series given
age 2-6 months; there is a risk of intussusception with this vaccine
anemia, heat intolerance, steatorrhea, wheezing, hemoptysis, clubbing, rectal prolapse, hx of neonatal jaundice
CF
complications of prolonged CF
gallstones, cirrhosis with portal htn, and pancreatic fibrosis
pos Coomb’s test
autoimmunue hemolytic anemia (warm antibodies directed against RBCs). Typical patient is 2 to 12 yo and the anemia is usually acute and transiet lasting 3-6 mos; freq preceded by infection
