BRS question notes Flashcards
First sign of puberty in males
testicular enlargement
First sign of puberty in females
breast development
Girl delayed puberty and older than 14 with high FSH and LH and normal genitalia think?
Think primary amenorrhea = turner syndrome or ovarian failure
turner more likely in short stature girl
Gradual testicular pain with blue coloration what do you do
rest and analgesics. Its a testicular appendage
Doppler would just show normal or increased uptake
Treatment options for uncomplicated cervicitis
Cover gonorrhea and chlamydia
Gonorrhea = IM ceftriaxone, oral ofloxacin, ciprofloxacin, cefixime
Chlamy = azithro, erythro, doxy
Teenager girl with irregular bleeding and anemia what do you do?
Perform complete pelvic exam and then put her on progestin:only pill since is anemic so the bleeding is causing symptoms
Teen pregnancy problem for mother health bc?
Increased risk of hypertension, anemia, and preterm labor
Don’t need parents for prenatal care
Infants are also at higher risk for having problems.
Homeless child and needs outpatient medication what do you do?
Warrant inpatient because of poor follow:up with outpatient therapy
Painful genital ulcers wtih irregular borders and purulent base =
chancaroid from Haemophilus ducreyi
Can also have lymphadenopathy
HSV vs chancroid
HSV have many shallow painful ulcers. Bases are nonpurulent
Newborn with increased oral secretions and respiratory difficulty =
esophageal atresia bc accumulated saliva in proximal esophageal pouch
Respiratory distress, scaphoid abdomen, bowel sounds in chest =
congenital diaphragmatic hernia
Most likely cause of abdominal newborn mass =
renal origin : hydronephrosis
What is hydrometrocolpos
retention of vaginal secretions presents as small cyst between laiba and during childhood as lower midline abdominal mass
Indirect hyperbilirubinemia in the first week of life and otherwise normal think
Breastfeeding jaundice due to inadequate fluid intake. Increase Feeds, monitor bili
Hyperbilirubinemia after first week of life in otherwise healthy infant think
breast milk jaundice from high lipase and beta:glucuronidase in breast milk
Where does omphalocele occur vs gastroschisis
Omphalocele is central through umbilical ring, has sack
Gastroschesis is lateral abdominal wall defect. : get inflammation of bowel and risk of bowel obstruction bc exposure to amniotic fluid
Omphalocele vs gastroschisis associated problems
Omphalocele associated with congenital defects heart, trisomy 13 and 18 NOT 21
Gastro has more damage to actual bowel bc it is free in amniotic fluid
By defintion persistent pulmonary hypertension excludes?
Anything caused by congenital heart disease
PPHN occurs most commonly in
near:term, full:ter and post term infants
What is most effectiv vasodilator for PPHN
oxygen therapy
Premature infant with diffuse ground glass opacities and air bronchograms =
respiratory distress syndrome
Two common causes of PPHN
perinatal asphyxia and meconium aspiration syndrome
Excess fluid in newborn appears on CXR as
normal or increased lung volumes with increased vascular markings
Breastfeeding vs formula and jaundice
breastfeeding have higher peak serum bilirubin values
When does benign physiologic jaundice peak in term and preterm infants
Term : 3:4 days, Preterm : 5:7 days
What is a derm lesion over face in trigeminal region and why is it important?
port wine stain or nevus flammeus : could indicate Sturge:webber sydrome which has intracranial vascular malformations and calcifications
small dry rash over dark acular base more commonly seen in african americans =
pustular melanosis
What is nevus simplex and does it have malginancy risk
vascular lesion (salmon colored) rash often on back of neck benign and often transient
whitish papules over nose, cheeks, forehead, chin=
milia which are bengin small cysts around pilosebaceous follices
What is erythema toxicum neonatorum
benign rash usually present at 72 hours of life in 50% of infants. Is maculopapular rash that are erythematous
CXR showing abdominal distension, air fluid levels, thickened bowel walls, and pneumatosis intestinalis =
Necrotizing enterocolitis esp if have bloody stools and abdominal pain
Double bubble on CXR think
duodenal atresia
CXR with soap bubble appearance =
meconium ileus, could be caused by cystic fibrosis
Down syndrome associated with what congenital GI problem
duodenal atresia
Choledochal cyst causes?
An elevated bilirubin with large direct component
5 areas of apgar score
appearance, pulse, grimace or reflex irritabiity, activity (flexion of extremities), repiratory effort
Tetralogy of fallot response to 100% oxygen
will not increase PaO2 by much because there is not much pulmonary flow in the first place
Truncus arteriosis response to 100% oxygen
Because there is a decent amount of pulmonary flow, they will have some response but not as much as in pulmonary diseases
VACTERL association group
Vertebral defects, anal atresia, cardiac anomalies, TracheoEsophageal fistula, renal and genital defects, limb defects
Newborn with vomiting after feeding w/ breast milk or cow’s milk, hypoglycemia and hepatomegaly think
galactosemia
Newborns with galactosemia susceptible to?
E coli sepsis
Neurodegeneration + splenomegaly + bony changes:often Erlenmyer flask distal femur =
Gaucher’s disease : sphingolipids accumulate in cells and organs. Deficiency of glucocerebrosidase
Most common lysosomal storage disease
Developmental delay, coarse facies, corneal clouding, dysostosis multiplex think
Hurler syndrome also called mucopolysaccharidosis : buildup of glycosaminoglycans
personality changes, ataxia, seizures in infant think
wilson’s disease
decreased height, coarctation of aorta, webbed neck think
turner syndrome
Noonan syndrome characteristic features…. vs turners?
Kind of like turner syndrome with webbed neck, pectus excavatum, short stature. Also have right sided heart lesions whereas turners have left sided heart lesions
Short stature with skeletal asymmetry, triangle shaped head,
Silver:Russel syndrome
Hyperactivity, developmental delay, mousy or musty odor think
phenylketonuria
Peripheral neuropathy, renal and liver disease, odor of rotten fish or cabbage =
tyrosinemia type 1
Hyperactive startle, loses eye contact, cherry red macula, enlarging head, neurodeneration/developmental delay, progressive blidness and seizures =
Tay:Sach’s disease a disorder of hexosaminidase A deficiency
Death by 4 years of age
Dysostosis multiplex found in
Hunter and hurler syndrome = when patients have mucopolysaccharides
Sodium benzoate useful for?
Helping to remove hyperammonemia
In maple syrup disease what should you avoid and go for in terms of feeding
give IV glucose as a basic energy source
don’t give parenteral nutrition with protein bc in disease can’t break down branched:chain amino acids. Also avoid enteral feeds until you confirm the diagnosis
large ears, macrocephaly, blue irides, and large testes, intellectual disability=
fragile X syndrome
Tall stature, gynecomastia, small penis and testes =
Klinefelter’s syndrome
infantile hypotonia, hypogonadism, short stature, obesity and hyperphagia =
Prader:willisyndrome
loquacious personality, supraclavicular aortic stenosis, hypercalcemia, elfin facial appearance=
Williams syndrome
atlantoaxial instability is a complication of what common syndrome
Down’s syndrome
ADHD symptoms, small head, short palpebral fissure, long smooth philtrum w/ a thin upper lip
fetal alcohol syndrome
upslanting palpebral fissure, epicanthal skin folds, protruding tongue =
Down syndrome
mental retardation, finger and nail abnormalities, cardiac defect =
fetal phenytoin syndrome
hypogonadism, almond shaped eyes, short stature, hyperphagia
prader:willi syndrome
mental retardation, small head, puppetlike gait =
angleman syndrome : problem with mothers 15
homocystinuria vs marfan syndrome
marfanoid habitus but without arachnodactyly aka long fingers = homocystinuria
homocystinuria has downward lens subluxation, marfan has upward
homocyst has mitral and aortic regurg but no aortic dilation like in marfarn
mental retardation in homocystinuria
Both have hypogonadism
scissoring lower extremity, clenched hands w/ overlapping digits, rocker bottom feet, delicate small facial features
trisomy 18
trisomy 13 associated w/
midline defects of brain and forebrain, microphthalmia, holopresencephaly, cleft lip and palate
Deletion on chromosome 7 causes?
william syndrome = elfin face and cocktail personality
corneal clouding, stiff joints, coarsened facial features =
hurler syndrome : mucopolysaccharidosis
catlike cry with slow growth, microcephaly, mental retardation, hypertelorism, =
Cri du chat syndrome : partial deletion on chromosome 5
defect in type 5 collagen in what syndrome
Ehler’s:Danlos syndrome
hyperextensible joints, fragile blood vessels, easily bruised skin, tissue:paper think scars, mitral valve prolapse, aortic root dilation =
Ehler’s danlos syndrome
decreased growth rate + early morning emesis think
mass lesion in CNS. craniopharyngioma
Which growth conditions have normal bone growth rate
genetic short stature and constitutional growth delay
Most common form of female pseudohermaphrodism is?
congenital adrenal hyperplasia from 21 hydroxylase deficiency
Stronger hereditary component in which type of DM
DM Type 2
What genetic markers do type 1 DM have
HLA haplotypes DR3 or DR4
What happens to bone age in growth hormone deficiency?
growth hormone deficiency causes slow growth velocity
Bone growth with hypothyroidism
delayed bone age aka bone age less than chrono age
bone growth in hypercortisolism
delayed bone age
Turner syndrome bone age?
normal bone age
skeletal dysplasia bone age
normal
intrauterine growth retardation bone age?
normal
When should dextrose be added while treating ketoacidosis
When sugar drops below 250 mg/dl
premature thelarce means
transient isolated early breast development in girls under 7
premature adrenarche
early onset pubic or axillary hair
If you have pubic hair and apocrine odor with no breast development or advanced bone age you have?
premature adrenarche
androgen and cortisol levels in peripheral precocious puberty
will have high androgens but low cortisol. Some
Differences in etiology of girls and boys with central precicious puberty
In girls its often idiopathic. In boys its almost always organic : get MRI of head
How is GnRH used in precocious puberty
administer and if dramatic increase in LH its central
If flat response its peripheral or any HPGA not activated
In peripheral precocious puberty how do boys present
feminization or premature pubic hair. NO testicular enlargement bc no increased FSH
In peripheral precoc puberty how do girls present?
Girls get virilization and breast development
Midline defect like cleft palate, hypoglycemia, and microphallus think
congenital hypopituitarism
What happens in nesidioblastosis
beta cell hyperplasia so you get hyperinsulinemia
large for gestational age, macroglossia, umbilical hernia, hyperinsulin think
Beckwith Wiedemann syndrome
Common bacterial infections in under 3 months caused by?
Group B strep, E. Coli, Listeria Monocytogenes
When does heterophile antibody measurement not work in mono
when kid is under 4
Most common cause of viral meningitis
Enteroviruses
What does CSF fluid cell count look like early in viral meningits
More poolymorphos that eventually shift to lymph predominance
Initial abx for bacterial meningitis
3rd gen cephalosporin and vanc
When are steroids useful for mengitis
Can reduce hearing loss in Haemophilus influenzae type b meningitis
What increases risk fo HIV transmission from mother to infant
high viral load, chorioamnionitis or genital tract infection, premature birth, prolonged rupture of membranes
HIV and breastmilk?
yes transmission can occur
Features of scarlet fever?
sandpaper rash, pharyngitis, fever
scarlet fever caused by
erythrogenic toxin producing strains of group A beta hemolytic strep
which complications of strep infection can be reduced by treatment
PANDAS and rheumatic fever
Therapy that is helpful with measles infection
supportive care and vitamin A
When do koplic spots occur? and what are they related to?
they are transient and occur before the rash happens
Fever, headache, conjunctivitis, diffuse macular rash on trunk and face think of
measles
What is common cause of death with measles
bacterial pneumonia
intracranial calcification, hydrocephalus, chorioretinitis consistent with?
Toxoplasmosis neonatal infection
wheezing, eosinophilia, pulmonary infiltrates think
noninvasive allergic disease secondary to aspergillus
When is PPD >10 positive?
when patient younger than 4yrs
What age are children not really contageous with TB and why?
When under 12 don’t have strong cough and significant pulmonary manifestation
Bloody stool sfrom? (6)
salmonella, shigella, campylobacter, EHEC, Cdiff
Shigella infection associated with?
seizures bc release of neurotoxin
salmonella associated with?
ingestion of contaminated poultry or exposure to turtles and lizards
Yersinia can unusually cause?
mesenteric adenities causing pain mimicking acute appendicitis
Acute appendicitis mimicker
Yersinia infection
Buccal cellulitis caused by
HiB
Impetigo caused by?
Staph aureus and group A Beta hemolytic strep
What is nikolsky sign
extension of bullae with lateral pressure applied to skin
Nikolsky sign indicates
staph scalded skin syndrome
Patient in Tet spell what do you do?
place patient in knee:chest position to increase SVR
What drugs are contraindicated in tetratolagy of fallot spell
dopamine and epinephrine
Preferred treatment after restenosis of coarctation?
Balloon angioplasty
What accompanies coarctation of aorta in half of patients
bicuspid aortic valve
How are VSD size and murmur related?
Smaller size VSD leads to higher pitched murmur
Newborn cyanosis and left axis deviation and LVH
Tricuspid atresia
What patients require prophylaxis against bacterial endocarditis
any patient with structural heart disease including patent ductus arteriosus. Structural but repaired in last 6 months. EXCEPT ostium secundum
Grade 1:3 systolic murmur heard best at mid:left systolic border that decreases on standing?
Stills murmur
grade 1:2 high pitched systolic murmur heard best at upper left sternal border
pulmonic systolic murmur
heart disease of infants born to lupus mothers?
congenital third degree AV block
What are two inherited forms of long QT?
Jervell:Lnage:Nielsen syndrome : when there is also congenital deafness and
Romano:Ward syndrome when there is no congenital deafness
What is inherited long QT sometimes associated with?
congenital deafness in Jervell:Lange:Nielsen syndrome
What causes snowman appearance on CXR?
unrepaired total anomalous pulmonary venous connection with supracardiac drainage
Most common cause of croup
parainfluenza
CXR thumbprint sign means
epiglottitis
Most effective management for bronchiolitis
supportive care
Most common cause of bronchiolitis
RSV
At what age does chlamydia trachomatis pneumonia generally occur
in young infants 1:3 months of age
Most common cause of pneumonia older children and adolscents
Mycoplasma pneumoniae and chlamydia pneumoniae
When does group B strep cause pneumonia
neonatal period
What colonizes patients with CF over time
first staph aureus and then pseudomonas
Class electrolytes of CF
hyponatremic, hypochloremic, hypokalemic, metabolic alkasosi
How do PFTs change in CF over time
first obstructive. Eventually become restrictive
GI symptoms of CF
Get pancreatic insufficiency. Replace with fat soluble vitamins
fever, muffled speech, tripoding, drooling, and neck hyperextension think
epiglottis. Used to be from Hflu but can also be from strep or staph
What should you avoid in epiglotittis
any stimulation of glottis like examining with tongue depressor
afebrile, staccato cough, hx of conjunctivitis, elevated eos on cbc, and neg blood cultures think
Chlamydia trachomatis : treat with azithro or erythro
Daily asthma symptoms with nighttime wheezing more than once per week =
moderate persistent asthma
What occurs in heterotaxy
intestines begin rotation from abnormal position and so end in one where they can get volvulus
Down’s syndrome patients often have what GI symptoms
Duodenal atresia
persistent vomiting with hypochloremic metabolic alkalsosi, may have hypokalemia =
hypertrophic pyloric stenosis
polyhydramnios, vomiting, epigastric sitension, scaphoid abdomen think
duodenal atresia
scaphoid abdomen can be what two things, differences?
congenital diaphragmatic hernia : severe resp problems
duodenal atresia : polyhydramnios
leading and second leading cause of pancreatitis in kids
trauma and then idiopathic
colicky abdominal pain, vomiting, lethargy, blood in stools think
intussuception
study to do when suspecting intussuception =
contrast enema that involves intestinal segment and often reduces
What can cause functional constipation in neonates?
Nay truama like diaper rash, painful diarrhea, abuse : baby doesn’t want to go
What do you do when child shows signs of protein intolerance
change to different protein. Don’t do studies
Rice and celiac disease?
Rice is ok
Conjugated bilirubinemia in newborn rule out
biliary atresia and choledochal cyst
When are kids with Hep A contagious
2:3 weeks before onset and 1 week after onset
Serology in Hep A
IgM and IgG rise early in infection
Infants with UTI have increased risk for?
underlying structural abnorm including vesicoureteral reflux
UTIs more common in boys or girls and when
before 6 months more in boys but after more common in women
How do you get urine in infant and neonate?
Must do cath or suprapubic aspiration
When can you get false negative on dipstick for blood?
Vitamin C ingestion
When is oral rehydration therapy acceptable?
Secretory diarrhea like in cholera, bc still maintain ability to absorb fluid and electrolytes
What is appropriate fluid tyep and amount for emergency phase of rehydration therapy?
20ml/kg of NS or LR
Where does strep infection have to occur for PSGN
skin or pharynx
When does nephrotic syndrome in kids occur?
Kids under 5
What are the two forms of HUS
shiga toxi:associated form and atypical form from medication or genetics
What are poor prognostic signs in HUS
elevated WBC and prolonged oliguria
When do you give antibiotics in HUS
Don’t give, actually could increase likelihood of developing HUS
Asian with recurrent gross hematuria after respiratory infections =
IgA nephropathy or Berger’s disease
Abnormalities ureterovescial junction inherited as?
Autosomal dominant
Treatment for VUR of any grade? When do you see a urologist?
All need low dose prophylactic abx. See urologist if grade 4 or 5
How much do you increase maintenance fluids for every degree over 38?
Increase by 12 %
Maintenance fluids using surface area method follow requirement of?
1500ml/m2/day
Infant with gross hematuria and unilateraly or bilateral flank mass, mother may have diabetes?
Renal vein thrombosis
Classic electrolyte presentation of RTA
hyperchloremic metabolic acidosis, with normal anion gap
Patient with acidosis, glucosuria, aminoaciduria, hyperphophaturia =
fanconi syndrome
severe htn, liver disease, enlarged cystic kidneys in kid think?
Infantile polycystic disease : Autosomal recessive
Most common inheritance of Alport syndrome
X linked dominant form
HTN, hematuria, renal failure in boys, hearing loss, ocular abnromalities think
alport syndrome
Patient has mother with difficulty releasing handgrip think
Congenital myotonic dystrophy : AD inheritance
infant with hypotonia, feeding problems, respiratory problems, facial weakness, hyporeflexia
congenital mytonic dystrophy
constipation+hypotonia, problems suck/swallow, progressive weakness w/ some paralysis =
Infant with botulism
Weakness and hypotonia but normal DTRs in infant think
Neonatal myasthenia from mother
Hypotonia and tongue fasciculations think
spinal muscular atrophy
Ascending symmetric paralaysi with absent DTRs and normal sensory exam think
Guillain:Barre syndrome
Most common agent in GBS is?
campylobacter jejuni
Ate honey, symmetric descending paralysis usually first have constipation. Hypotonia and hyporeflexia
Infantile botulism
generalised 3Hz spike? What is it and how is it inherited
absence seizure inherited as AD
treatment for absence seizure
ethosuximide
brief myclonic jerks lasting 1:2 seconds with a few happening over 3:5 minutes
extension of arm and flexion of neck think?
Infantile spasms or West syndrome.
Infantile spasm most commonly caused by?
Tuberous sclerosis. Can also be perinatal asphyxia, intravascular hemorrhage, and meningitis
Duchenne muscular dystrophy inherited as?
X linked
How do you make diagnosis of duchenne muscular dystrophy? and what would an EMG show?
DNA testing of dystrophin gene. EMG shows small polyphasic muscle potentials with normal nerve conduction veolocities
First thing you evaluate when suspecting acute cerebellar ataxia?
There is no actual physical evidence but do neuroimaging to rule out anything worse
bulging fontanelles and downward deviation of eyes =
hydrocephalus. Increased third ventricle pressure injures upward gaze center.
How do HgB levels change at birth?
High at birth and decrease until 2:3 months of age. (full:term)
In preterm its at lowest at 1:2 months
When does fetal hemaglobin disappear
Declines and disappears by 6:9 months
Normal growth and appearance, recurrent infxns, ANC and WBC low, below 4 =
chronic benign neutropenia
Oculocutaneous albinism and severe infections think
Chediak:Higashi syndrome
Frequent severe infections and extrememly low ANC think
Kostmann syndrome (severe agranulocytosis)
Poor growth, pancreatic insufficiency, metaphyseal chondrodysplasia =
Schwachman:Diamond syndrome
Treatments for ITP
supportive care, intravenous immune globulin, steroids, anti:D immunoglobulin
When is ITP more likely to become chronic?
Chronic ITP more likely if older than 10
What is earliest lab finding in iron deficiency anemia
low serum ferritin
Hemaglobin in sickle cell disease vs trait
Disease cannot have any Hgb A
Common problems in sickle cell trait
Inability to concentrate urine and hematuria
bruising, epistaxis, menorrhagia think
Von willebrand’s disease
Missing thumb and radius with pancytopenia, short stature
Fanconi Anemia
Can also have skin findings and kidney anomalies
What are some of the less obvious complications of B thalassemia
bone marrow hyperplasia esp in bones of face, delays in growth and puberty, hepatosplenomegaly. Hemochromatosis
Goats milk feeding leads to
folic acid deficiency.
Spoon shaped nails indicates
iron deficiency anemia
Thrombocytopenia and large hepatic hemangioma
Kasabach:Merritt Syndrome
Eczema, T and B cell defects, low platelet count =
Wiskott:Aldrich syndrome
Thrombocytopenia, absent radius, cardiac and renal disease
Thrombocytopenia:absent radius syndrome
TAR syndrome vs fanconi anemia
Thumb is present in TAR syndrome. Thumb AND radius missing in fanconi anemia
Diarrhea then prolonged PT, aPTT, normal bleeding time =
Vitamin K deficiency
Prolonged aPTT, normal PT, prolonged bleeding time, he bleeds after a procedure
von Willebrand’s disease
is common acute lymphocytic leukemia antigen negative a good predictive factor or positive?
negative is an unfavorable prognostic indicator
Is it better to be female or male with ALL
better to be a female and between 1:9yrs
Why do they give intrathecal methotrexate during induction treatment for ALL
give it to all patients regardless of prognosis as prophylaxis against CNS inovlement
Avoid brain radiation in which kids
Younger than 5 years
Hepatosplenomegaly, bursing, bone pain, gingival hypertrophy
AML
AML associated with what developmental disorder? And what does this mean about prognosis?
AML is associated with Down Syndrome. Has good prognosis
How is hodgkins staging done with A and B?
A means no systemic symptoms, B is many systemic symptoms like fever and weight loss
Male sterility common in what type of cancer?
Hodgkins disease
Most common tumors between 1:12 yrs
Infratentorial brain tumors
What is the most common infratentorial tumor? 2nd, 3rd
Medulloblastoma, astrocytoma, brainstem glioma
What imaging is good for looking in the infratentorial region?
MRI is preferred
How can you detect medulloblastoma
Homovanillic acid and vanillymandelic acid in the CSF
Most testicular tumors are benign or malignant?
2/3 are malignant
What are the most common testicular tumor?
Yolk sac tumors
What serum marker elevated in hepatocellular carcinoma
alpha fetoprotein
Down syndrome predisposes to what cancers?
AML and ALL
Beckwith:Wiedemann syndrome predisposes to what cancers?
Wilms tumor, rhabdomyoscaroma, hepatoblastoma
Neurofibromatosis type 1 vs 2 in terms of cancers
Type 1 to brain tumors and lymphomas
Type 2 to acoustic neuromas
Cancer: painless soft tissue mass
rhabdomyosarcoma
Osteogenic vs Ewing’s sarcoma in terms of area of bone where it affects
Osteogenic : metaphysis of tubular long bones, distal femur, proximal tibia
Ewing does flat bones and diaphysis like axial skeleton (pelvis)
sunburt =
osteogenic sarcoma
Onion skin =
Ewing’s sarcoma
How does langerhans cell histiocytosis normally present?
As a bony lesion.
Mass behind ear and chronic ear drainage
Langerhans cell histiocytosis
What is most common teratoma in infancy
Sacrococcygeal teratoma : soft tissue mass in area of cocyx. Mostly FEMALES
Anaphylaxis mediated by what cells?
IgE mediated
Most effective therapy for allergic rhinitis
intranasal steroids
Best method for testing food allergies are?
provocative food challenge : double blinded placebo controlled
Hypogammaglobulinemia, Tcell dysfunction with normal B and T cell numbers : later in childhood
cmmon variable immunodeficiency
chronic granulomatous disease due to what problem in neutrophils
defect in oxidative metabolism
Patient with repeat meningococcal sepsis
deficiencies in late complement C5,6,8
cerebellar ataxia, red spots on conjunctiva and skin, chronic sinopulmonary infections
ataxia telangiectasia
hallmark of immunoglobulin deficiency is?
increased susceptibility to sinopulmonary infections caused by encapsulated bacteria
What is the nitroblue tetrazolium test used for?
For diagnosing oxidative metabolism = chronic granulomatous disease
Infantile vs childhood eczema
Infantile involves extensor surfaces and cheeks
Early childhood involves flexural surfaces
Failure to thrive + recurrent soft tissue infections from neutropenia =
Schwachman:Diamond syndrome : because they have neutropenia and pancreatic exocrine insufficiency
Immunodeficiency with lots of abscess formation think about
Combined granulomatous disease : defect in neutro oxidative metabolism so cant do intracellular killing of catalase positive bacteria and fungal pathogens
Eczema + congenital thrombocytopenia + combined immunodeficiency (encapsulated organism infections) + small platelets =
Wiskott:Aldrich syndrome
adenosine deaminase deficiency is a subtype of?
severe combined immunodeficiency disease
adenosine deficiency given away by infection with?
both common and opportunistic stuff like candida or PCP
fatigue, muscle weakness, heliotrope rash
dermatomyositis
What happens to muscles in dermatomyositis
calcinosis = calcium deposition in muscle, fascia, and subcutaneous tissue
Difference between childhood and adult dermatomyositis
In childhood no increased risk of malignancy
IgA mediated vasculitis involving skin, joints, gi tract, kidneys
HSP
Why do Kawasaki disease patients get abdominal pain?
Hydrops of the gallbladder
Salmon:colored maculopapular, on trunk and proximal extremities
systemic onset JRA rash
What happens to systemic onset JRA joints?
Have 50% chage of severe erosive arthritis
Difference between systemic:onset JRA and early:onset pauciarticular JRA in terms of serology
ANA is negative in systemic onset
ANA is positive in early:onset and in 50% of patients with polyarticular JRA
How many hours for tic to give lyme
at least 36 hours
When does chorea occur in acute rheumatic fever?
Usually several months after
Why is late onset pauciarticular JRA different?
Male predominant, children older than 8, HLA:B27 positive
List different types of Juvenile Rheumatoid Arthritis
Early onset pauciarticular :
Late onset pauciarticular :
polyarticular :
systemic:onset
What occurs during subacute phase of Kawasaki disease
decreased ESR, marked thrombocytosis, coronary artery aneurysms
IBD can also cause?
HLA:B27 positive axial skeleton arthritis
violaceous rash around eyes, erythematous hypertrophic papules =
Dermatomyositis
Sexually active and arthritis think?
Reiter’s disease : can’t see, can’t pee, can’t climb a tree
Rash on buttocks or thigh with abd pain, arthritis, glomerulonephritis think
HSP : IgA mediated vasculitis
Spiral fracture of tibia with nothing on fibula
Toddler’s fracture : only need casting if its non:displaced
swelling and tenderness over tibial tuberosity
Osgood:Schlatter disease : overuse injury w/ inflammation or microfracture of tibial tuberosity : rest and stretch
When do you intervene in metatarsus adductus
When foot cannot be manipulated beyond straight
With septic joint important fact about treatment
Don’t start abx until cultures are obtained
When do you treat scoliosis
After growth spurt if cobb angle >50
brace during growth period to stop progression
When does growth stop in females
6 months after onset of menstrual cycle
supracondylar fracture is a?
orthopedic emergency bc risk of compartment syndrome and neurovascular injury
What should you move to check for compartment syndrome in supracondylar fracture
passive extensino of digits. Don’t move elbow bc can cause neurovascular injury
Who is at risk for slipped capital femoral epiphysis
male+obesity. Hypothyroidism makes you at risk for bilateral
complication of congenital torticollis
plagiocephaly
3 consecutive wedged vertebrae =
Scheuermann’s kyphosis : a painful hunched back
Down syndrome patient wants to do gymnastics what do you check?
Do lateral flexion:extension cervical spine to rule out instability bc predisposed to atlantoaxial instability
allergic conjunctivitis is what type of hypersensitivity reaction
type 1 allergic reaction
Hallmark of retinoblastoma
calcification within the tumor
conjunctivitis in first 24 hours
chemical irrigation
serous or purulent conjunctivitis with some lid swelling in first 4:10 days
chlamydia trachomatis
serous or purulent conjunctivitis with some lid swelling in 2:4 days
Gonorrhea
diagnosis of HSV
giant cells on tzanck prep
well:demarcated hair loss without scalp inflamm =
alopecia areata
cause of alopecia areata
autoimmune lymphocyte:mediated injuryt to hair follicle
alopecia with hairs borken off at scalp w/ lymphadenopathy think
tinea capitis
thickened white borken hairs, flouresce under woods light
M. Canis infection from dogs and cats
What is Koebner phenomenon in psoriasis
new lesions develop at sites of trauma
psoriasis inheritance
AD
Treatment for pityriasis rosea?
don’t need anything. can give antihistamines for itching
Why do you use only low dose corticosteroids in the groin
thinness of epidermis means absorption of steroid greater. Get growth suppression, cushing syndrome, adrenal suppression, pigmentation changes
What is the most effective method of moisturizing skin
Use ointments>creams>lotions
severe cystic or nodular acne treat with
isoretinoic acid
gianotti crosti syndrome rash =
red or flesh colored papules
HSV rash =
grouped vesicles on erythematous base
flesh colored papules w/ umbilication =
molluscum contagiosum
Lisch nodules or iris hamartomas =
Neurofibromatosis type 1
ash:leaf spots, angiofibromas =
Tuberous sclerosis
First sign of cerebral herniation in under 4 kids =
bradycardia
When does cushing’s triad actually happen in edema
late in the course of ICP
Modified GCS
Modified GCS
Why do children do better than adults in drowning?
They have primitive dive reflex shunting blood to necessary organs
What happens to PFTs after drowning
PFTs deteriorate during 12:24 hours after submersion injury
Why can’t you use charcoal, ipecac, NG tube in alkali ingestion
Burns esophagus so can’t use NG
charcoal messes up endoscopy
No Ipecac in caustic ingestion??
When do you get infection with pasteurella multocida
cat and dog bites
Brown recluse spider vs black widow spider bite?
Black widow has few local symptoms but then get muscle cramps and HTN
brown recluse get lots of local signs and symptoms
Iron ingestion toxicity management
gastric lavage and whole bowel irrigation. Also iron chelator with deferozamine. Don’t do activated charcoal, ipecac.
When do you see basophilic stippling?
lead intoxication
