Step 2 Peds Flashcards
This type of newborn hemorrhage is limited by the cranial bones. Where is the hemorrhage located? What is the treatment?
What is the “sister” component to this and how does it present differently?
Cephalohematoma. It is a subperiosteal hemorrhage - monitoring is sufficient b/c it resolves on it’s own mostly.
Caput Succedaneum is a diffuse swelling over suture lines, can be ecchymotic, and usually appears over the presenting vertex part of the baby’s scalp.
What would you be worried about with a child presenting with episodic apnea, pallor or cyanosis, poor suckling, abnormal eye movements, high-pitched cry, muscle twitching, convulsions, decreased hematocrit, metabolic acidosis, and shock? What might you find on PE?
Intracranial hemorrhage. Bulging or pulsatile fontanels.
What would you suspect the etiology to be of a depressed calvarium, resembling a “dented ping pong ball”?
Forceps delivery or fetal head compression. This is a depressed skull fracture.
Why do up to 2/3 boys experience transit gynecomastia during puberty? What’s the normal size of testicular volume in a prepubescent boy? *BONUS - what’s the name of the measuring device?
The testicles make a transient elevated level of estrogen and there’s an increase in peripheral prohormones into estrogen. < 3 mL for prepubescent testicular size measured via a Prader orchidometer.
How would IgA deficiency present? What are common infectious agents seen?
Recurrent sinopulmonary (Hemophilus & Streptococcus) and GI (Giardia) infections. They also can form antibodies against IgA so transfusions can cause an anaphylactic reaction.
What problem do you think of with deficient T cell synthesis, thymic aplasia, fungal infections, hypoparathyroidism with hypocalcemia, cleft palate, and congenital heart disease?
DiGeorge Syndrome - remember CATCH-22: 1) Cardiac anomaly (Tet Fallot = MC or Truncus Arteriosus) 2) Abnormal Facies 3) Thymic Aplasia 4) Cleft Palate 5) Hypoparathyroidism / Hypocalcemia Problem with Chromosome 22
What is deficient in X-linked Agammaglobulinemia? What is the common respiratory pathogen? How is this different from IgA Def?
B Cell Deficiency and all immunoglobulins. Pseudomonas. You won’t have an anaphylactoid reaction because you can’t mount any antibodies.
What do you think of in a child with thrombocytopenia, recurrent otitis media, and eczema? What is deficient in these kids?
Wiskott-Aldrich Syndrome. Combined deficiency in B and T cells.
What is the difference in kids with Hyper-IgM Syndrome and IgA Def?
The IgM levels are elevated in relation to IgA and IgG. But lymphoid hyperplasia is key to distinguishing this syndrome b/c both sinopulmonary and GI infections can present.
What are Cystic Fibrosis patients prone to getting?
Sinopulmonary infections (Pseudo), pancreatic insufficiency, infertility, and meconium ileus.
What do you think of in a child with thrombocytopenia, recurrent otitis media, and eczema? What is deficient in these kids?
Wiskott-Aldrich Syndrome. Combined deficiency in B and T cells.
What causes the arthropathy seen in hemophilic patients? What complications can occur do to this? What imaging is used to best see this? What prophylactic treatment helps prevent these?
Hemosiderin and Fe deposition in the joint causing synovitis and fibrosis. Complications include chronic worsening joint pain, swelling of the joint, contractures of the joint, and limited ROM. MRI is best. Early factor concentrates are used for prophylaxis.
How does each anti-epileptic work and what is it used for? Ethosuximide, Phenytoin, Carbamazepine, Phenobarbital.
BONUS - What two serious side effects do you look out for in the first several weeks of treatment with Phenytoin and Carbamazepine?
Ethosuximide - (childhood absence seizures) works on the thalamic neurons providing Ca-channel blockade
Phenytoin / Carbamazepine - (primary generalized tonic-clonic seizures or partial seizures) blocks neuron’s sodium channels. SEfx’s of Phenytoin = hirsutism, lymphadenopathy, gingival hypertrophy, and rash.
Phenobarbital - (second line) extends Chloride channel opening of GABA receptors
BONUS = Steven’s Johnson Syndrome or Toxic Epidermal Necrosis
How much do you expect a child’s weight and height to change in one year?
Increase height by 50% and weight should triple.
1) What congenital syndrome is associated with William’s Syndrome? (Happy elves)
2) What congenital syndrome is associated with PDA?
3) What congenital syndrome is associated with coronary artery aneurysms?
4) What congenital syndrome is associated with VSD?
5) What congenital syndrome is associated with MVP?
1) Supravalvular stenosis
2) Rubella and Char Syndrome
3) Kawasaki’s Disease
4) Aneuploidy (Trisomy 13, 18, 21)
5) Connective tissue d/o’s like Ehlers-Danlos, Osteogenesis Imperfecta, and Marfan’s
What abnormalities are common with 45 X karyotype? What should you order with this patient to exam for the above findings?
Bicuspid aortic valve, coarctation of the aorta, dilation of the aortic root (increased risk of aortic dissection), streak ovaries, broad chest with wide spaced nipples, amenorrhea, infertility, low-set ears, webbed neck, low hairline, narrowed high arch palate, horseshoe kidney. Order Echo and/or 4-extremity BP readings.
What congenital problem does a child with micrognathia, microcephaly, overlapping fingers, absent palmar crease, VSD, and rocker bottom feet?
Edward’s Syndrome = Trisomy 18
1) What congenital syndrome is associated with William’s Syndrome? (Happy elves)
2) What congenital syndrome is associated with PDA?
3) What congenital syndrome is associated with coronary artery aneurysms?
1) Supravalvular stenosis
2) Rubella / Char Syndrome
3) Kawasaki’s Disease
What abnormalities are common with 45 X karyotype? What should you order in all of these patients?
Bicuspid aortic valve, coarctation of the aorta, dilation of the aortic root (increased risk of aortic dissection), streak ovaries, broad chest with wide spaced nipples, webbed neck, low hairline, narrow high arched palate, low set ears, cubits valgus (angle of the arm is angled away from the body more than normal), horseshoe kidney.
Order an echo and BP measurement in all 4 extremities.
What do you suspect in a child who recently had a cold and now presents with symmetric palpable purpura on his lower extremities, colicky abdominal pain, hematuria on labs, and arthralgias with normal platelets? How do you treat this? What would renal bx show (not needed for dx)?
Henoch-Schonlen Purpura = IgA mediated vasculitis = leukocytoclastic (damage from the nuclear material in neutrophils).
Supportive care and NSAIDs for trmt.
Renal bx would show IgA deposition in the mesangium.
What causative organisms would come to mind in a kid with blue/black hemorrhagic, purpuric lesions?
Neisseria Meningitidis or Strep PNA. This is Purpura Fulminans = life threatening
What is the most common cause of poikilocytosis, anisocytosis, and elevated TIBC in a child?
Fe-def anemia 2/2 poor intake of Fe-fortified foods in the early years of life. Other causes = bleeding or parasitic infxns (elevated bili and eosinophelia would help distinguish the latter respectively)
- Anisocytosis = varying size of RBC’s
- Poikilocytosis = varying shape of RBC’s
What disease do you think of in a kid taking vitamins A, D, E, and K with pancrelipase? What’s the pathology behind this d/o? What are they at risk for?
This is Cystic Fibrosis - a mutation of the transmembrane regulator involving Chloride channels. They are at risk for recurrent PNA, chronic rhino sinusitis, obstructive lung disease leading to bronchiectasis, GI malignancy, obstructive azospermia leading to infertility in males, meconium ileum, clubbing, osteopenia leading to fractures, and kyphoscoliosis.
What are the most common pathogens causing PNA in a patient with CF as it relates to age?
S. Aureus (Gram + cocci in clusters) in infants and young children, Pseudomonas A. (Gram - Rods in adolescents and young adults)
What has occurred in a child with their arm held in a pronated position without any PE evidence to suggest trauma? How is it reduced?
Radial Head Subluxation = Nursemaid’s Elbow. The radial head has slipped from the proper placement in the annular ligament. Holding the child’s arm supinated with pressure on the radial head will most likely bring the radial head back into proper alignment with an audible click.
What would you suspect in a child who is an active sports, involved with throwing, player that complains of pain, “popping sounds”, and inability to rotate his forearm?
Panner’s Disease = Osteochondrosis of the capitellum (distal end of humerus) 2/2 to overuse causing decreased vascular supply to the area
What would you suspect in an older adolescent who has pain on palpation of the lateral epicondyl, pain with passive flexion of the wrist and with resisted extension of the wrist?
Tennis Elbow = Lateral Epiocondylitis 2/2 to overuse of the extensor muscles and their attachment point on the lateral epicondyl.
What are you concerned of in a child that presents with poor suckling, fatigue, rigidity, spasms, from an unimmunized mother with umbilical stump infection? What are your main concerns in the first and second weeks of life for the infant?
Clostridium Tettani causing tetanus. First week risk = apnea and second week risk = septicemia.
What kind of RBC d/o do you suspect in a patient with Coombs negative, jaundice, hemolytic anemia, elevated MCV, splenomegaly and + FM history?
What 2 tests are the gold standard for dx?
Hereditary Spherocytosis.
Acidified Glycerol Lysis Test and Eosin-5-Maleimide Binding Test (flow cytometry)
What d/o presents with a “baby-doll” (chubby face), short stature, thin extremities, protuberant abdomen, occasional hypoglycemic seizures, hypoglycemia, hyperlipidemia, hyperuricemia in a 3-4 month old child?
Type I Glycogen Storage Disease = Von Gierkes’ = G6PD Def. Affects the liver, kidneys, and gastric mucosa (hepatic labs are normal). Heart and spleen are normal.
What would you suspect with hepatomegaly, “floppy baby”, feeding difficulties, macroglossia, and heart failure in children within the first few weeks of birth?
Type II Glycogen Storage Disease = Acid Maltase Deficiency = Pompe’s. HF is 2/2 to progressive hypertrophic cardiomyopathy.
How would you distinguish Type III Glycogen Storage Disease from Type I?
Type III = Glycogen Debranching Enzyme deficiency. Common presenting factors = hepatomegaly, hypoglycemia, hyperlipidemia, and growth retardation. Distinguish based on NORMAL uric acid and lactate levels with ABNORMAL elevated liver enzymes and fasting ketosis. Also, spenomegaly is present with normal kidneys.
Type IV Glycogen Storage Disease = Amylopectinosis. What does the presenting patient have?
Progressive cirrhotic liver.
What could a patient have 2-4 weeks after having a Group A Streptococcus pharyngitis with migratory joint pain, carditis, subcutaneous nodules, erythema migrans, and Sydenhams chorea? What antibody level could you check?
Rheumatic Fever. Antistreptolysin-O titers
A child presents within 48 hrs of delivery with a high-pitched cry, poor feeding, irritability, poor sleeping, sweating, sneezing, seizures, diarrhea, vomiting, and tremors - what two drugs are you considering have caused this? Why might a mother been given one of these aforementioned suspected caustic agents? How is this treated?
Methadone or Heroin b/c opiates = MC cause withdrawal symptoms in newborns (t cause withdrawal
What are the MCC of hematochezia in a child? What do you suspect in a child with painless rectal bleeding and no abdominal tenderness or palpable findings? What should you order for this suspected dx?
Hemorrhoids, anal fissure, Meckel’s Diverticulum, Infectious Collitis, IBD, and intussusception.
Meckel’s Diverticulum = heterotopic gastric mucosa can be found by technetium-99m pertechenate scan.
What is the most common brachial plexus abnormality seen in delivery of a macrosomic baby? What nerve roots does this involve? How does it present? How’s the prognosis?
Erb-Duchenne Palsy - C5, C6, and C7 causing infraspinatous, deltoid, and wrist/finger extensors to be affected with compensation by opposing muscle groups - results in “waiter’s tip” appearance = extended elbow, pronated forearm, and flexed wrist. 80% spontaneously resolve with PT and massage by 3 months. If refractory, surgery can be attempted.
What are the common characteristics of Down Syndrome? What associated finding would cause bilious vomiting within the first 2 days of life? What clue would be seen on prenatal U/S? What further work-up should be done?
Flat facies, hypotonic child, curved fifth digit, increased space between first and second toe, macroglossia, slanted palpebral fissures, and low-set ears. Duodenal atresia. Polyhydramnios. Cardiac workup b/c of associated VSD/ASD problems.
When is meconium said to be “delayed”?
> 48 hrs
MCC of conductive hearing loss in children? What would distinguish an auditory problem vs. autism?
Recurrent ear infections. Autism would more likely have little affection, repetitive movements, and poor eye contact.
What is the only possible complication resulting from Hydroxyurea use?
Myelosuppresion causing thrombocytopenia, anemia, neutropenia.
What findings are associated with Celiac’s Disease? What would you order?
DM Type I, dermatitis herpetiformis, diarrhea, and anemia. IgA anti-tissue transglutaminase antibody assay - endoscopy with bx can also confirm
What is the MC piggy-back pathogen s/p influenza infection? What type of treatment would you begin w/ a hx of recurrent plum disease?
Staph A. - consider starting Vanco!
What is the spirochete responsible for Lyme Disease? What is the name of it’s vector?
Borrelia Burgdorferi carried by the Ixodes Tick
What are the indication for further w/u of neonatal jaundice?
1) Conjugated Hyperbilirubinemia >2mg/dL
2) Jaundice that appears in the first 24-36 hrs
3) Serum Bili that rises at a rate > 5mg/dL/24 hrs
4) Bili that is > 12 mg/dL (term) or >10-14 mg/dL (preterm)
5) Persistence of bilirubin for > 2 weeks
6) Signs of symptoms
What d/o do you think of with an elevated conjugated bilirubin?
Neonatal Cholestasis 2/2 to Biliary Atresia or from external compression of the biliary ducts.
What two d/o do you think of with elevated unconjugated bili?
1) Gilbert’s Syndrome = mild def of UDP glucuronyl transferase
2) Crigler-Najjar = absent UDP glucuronyl transferase
When does Breast Milk jaundice present and how is it managed?
Breast milk jaundice normally occurs in the 2nd week of life with elevated unconjugated bili levels reaching 30 mg/dL. Cessation of breast milk intake with replacement of milk-formula resolves symptoms in a day - return to breast milk after 2-3 days usually does not cause the hyperbilirubinemia to return.
When does physiologic jaundice present and resolve?
This jaundice (< 12 - 14 mg/dL) appears in the first days of life and resolves by the first week.
What is the MCC of nephrotic range proteinuria, edema, hypoalbuminemia in a child and adult respectively? What if the child has Hep B?
1) MCC of Nephrotic Syndrome in adults = Membranous and Focal Segmental Glomerulonephritis
2) MCC of Nephrotic Syndrome in children = Minimal change disease
Child with nephrotic syndrome + Hep B = Membranous type
Nephrotic Syndrome protein = 3g/day
What’s the MCC of…
1) Crescentic Glomerulosclerosis
2) Focal Segmental Glomerulosclerosis
1) Presents as rapidly progressive nephritic syndrome with renal insufficiency
2) African Americans & HIV
What stages of sleep do the following occur…
1) Night terrors
2) Somnambulism
3) Nightmares
1) Non-REM
2) Non-REM
3) REM
What RNA Togavirus do you suspect with a child who presents with a cephalocaudal proceeding rash lasting
Rubella (German Measles) - treat with acetaminophen and symptomatic care.
BONUS = Forschheimer Spots
When do vaccines get administered for preterm infants - according to what age scale?
Chronologic, NOT gestational. Exception is Hep B, child should be more than 2kg.
What is Eisenmenger Syndrome?
Cyanosis and dyspnea 2/2 to R-2-L shunting
What spinocerebellar atxia is most common and presents with ataxia, dysarthria, concentric hypertrophy causing cardiomyopathy, hammer toes, scoliosis, and diabetes?
Friederich’s Ataxia = MCC of death is cardiomyopathy or respiratory causes
What type of rash causes sterile, eosinophil filled, pustules in a child that is otherwise asymptomatic?
Erythema Toxicum Neonatorum
What would you expect to find with a child having eczema, bloody stools, and weight loss?
Milk Allergy - switch to hydrolyzed formula
If a child’s birth weight and height has been consistent since birth in the setting of “spitting up”, are you concerned?
No, this is most likely physiologic spit up 2/2 shorter esophagus, more laying down, and loose LES.
What is wrong with a child who has blue sclera, multiple intrauterine fractures, and growth retardation?
Osteogenesis Imperfecta - a type I collagen def
What is the trmt for a toxic child with circumoral pallor, erythematous tongue, sandpaper rash, pharyngitis, and a fever?
Penicillen V - this is Scarlett Fever
Hepangina presents as…
Ulcers on the soft palate, tonsils, and throat. Sometimes they appear of the hands and feet too! IV Hydration may be needed 2/2 to difficulty swallowing. MCC = Enterovirus like Coxsackie A
MCC of meningitis in children and young adults?
Presents with…
In contrast to HSV because it would cause…
Neisseria Meningititis - 75% present with petechial rash on either axilla, flanks, wrists, and ankles. Appears 24 hrs s/p infxn. Meningeal signs = N/V, lethargy, hip flexion with neck flexion
HSV - temporal encephalitis with seizures
Why do you give morphine, Oxygen, and have the kid assume a knee-to-chest position for a child with Tet Fallot? What are the findings?
Findings:
1) Right Obstructive Outflow (pulmonary artery atresia or stenosis)
2) Overriding Aorta
3) VSD
4) Right Ventricular Hypertrophy
Oxygen can help vasodilator the lungs causing a decrease in pulm. resistance. Morphine can calm them. Goal = increase SVR and decrease Pulm. Res. to promote L to R shunting)
What do you suspect to see with a child having strabismus? What age is strabismus abnormal after? How is it treated?
Expect to see deviating eye with cover test (when “good” eye is covered), asymmetric CORNEAL light reflexes.
Abnormal > 4months
Patch the good eye
Child with projectile non-bilious vomiting, even w/o palpable olive in the abdomen, w/ dehydration, can be at risk of?
Pyloric Stenosis: hypothesized to be 2/2 to formula fed male infants being able to drink more in shorter time causing pylorus to hypertrophy to hold it all in. Difference b/w normal GERD from infantile LES is the dehydration and or poor wight gain.
At what age is bedwetting normal in kids?
Up to age 5, boys take longer.
What do you suspect with the sudden onset of abdominal pain, bloody diarrhea, and the triad of renal failure, hemolytic anemia (jaundice), thrombocytopenia? What’s the causative organism?
HUS via E. Coli. The verotoxin released causes GI mucosal damage leading to bloody diarrhea with the activation of the clotting cascade
How does Staphylococcal Scalded Skin Syndrome present? Trmt?
Fever, irritability, diffuse erythema followed by blistering, exfoliation and positive Nilkolsky sign.
Oxacillin, Naficillin or Vanco
When would you use a flexible endoscope vs. rigid bronchoscopy vs. rigid endoscopy vs. laparotomy for an ingested foreign body?
Use a flexible endoscope for a non-dangerous object that has been ingested for an unknown amount of time or if the child is symptomatic; rigid endoscopy for sharp objects in the proximal esophagus; rigid bronchoscopy for aspirated objects (associated wheezing, stridor, etc); and laparotomy for dangerous objects (sharp items, multiple magnets, or batteries). If non-symptomatic and non-dangerous, can observe to see if it passes through GI system.
What do you think of with a pt presenting with recurrent bacterial respiratory track infections from 6-18 months like otitis media, PNA, sinusitis, and absent lymphoid tissues (tonsils, etc)? Labs? Trmt?
Bruton’s Agammaglobulinemia = X-linked Agammaglobulinemia = defect in Bruton’s tyrosine kinase which is a signaling molecule on all developing B-cells (CD19). T cells are fine (CD3). Give lifelong IVIG.
Cyanotic congenital heart disease + recurrent sinusitis places someone with a fever, seizures, morning/nocturnal headaches, focal neurologic deficits at risk of?
Brain abscess - the R-2-L shunting in Tet Fallot has blood bypass the immunologic filter in the lungs via phagocytosis. Bacterial seeding tends to occur in the gray white matter junction
Acquired torticolis looks like…? What do you order first? What are potential causes?
Child who resists flexion of their neck to one side and favors the other in the setting of neck swelling. Order Xray first to r/o cervical spine fracture or dislocation. MCC= URI, minor trauma, and cervical lymphadenitis. More serious cases = retropharyngeal abscess and atlantoaxial subluxation.
What is the MC predisposing factor for acute bacterial sinusitis?
Viral URI - mucosal inflammation causes offending bacteria to be inefficiently cleared away via mucociliary clearance
What does it mean if the null value is included in the given Confidence Interval?
The statistical significance is greater than the associated P-value for the respective CI. For example, A 95% CI ranging from 0.9 - 2.0 (includes null, 1.0) therefore, the real P-value is greater than 0.05 (since 0.05 is associated with 95% CI)
Sandifer Syndrome is associated with what type of feeding problem in infants?
GERD - it’s the arching of the back positioning
Toxic Epidermal Necrolysis (TEN) is a cutaneous form of hypersensitivity MCC by what the types of medications?
Sulfa drugs, anticonvulsants, and NSAIDS - mucous membranes are severely affected and shedding of the nails may occur
Stevens-Johnsons is a severe variant of this skin finding which later becomes inflammatory bullae of two or more mucous membranes (GI, Resp, GU…)
Erythema Multiforme
Drug of choice for dx w/ fever, toxicity, pharyngitis, sand-paper rash, circumoral pallor and strawberry tongue? Differentiating factor b/w this and Kawazaki?
Treat w/ Penicillin G for Scarlett Fever 2/2 to Group A Strep.
Bilateral conjunctival injection or peripheral extremity changes.
Medulloblastoma is the 2nd MCC posterior fossa tumor after _____ and is commonly located in ______.
Cerebellar Astrocytoma
Cerebellar Vermis
A palpable clunk on Barlow or Ortolani maneuvers warrants referral to orthopedic surgeon. What is it’s a soft click, leg length asymmetry, or asymmetric inguinal skin folds…what’s the w/u? What are we worried about - dx and complications?
Developmental Dysplasia of the Hip (DDH) warrants concerns for complications like Trendelenburg gait, scoliosis, arthritis, and avascular necrosis.
Get an U/S AFTER 2 wks ( 6 months get an X-ray b/c the hips are ossifying.
*Trmt
First initial step in child with speech delay?
Audiology eval - especially in the setting of past/recurrent ear infections
meconioum ileus is associated with what d/o? What level do you tend to see the obstruction?
Cystic Fibrosis - it tends to be inspissated ( thickened) and obstructed at the ileus vs rectosigmoid in Hirschsprungs
Winters Formula
Assess the PCO2 response for primary metabolic acidosis:
PCO2 = (1.5 x HCO3-) +8 and then +/- 2 for range
If it’s above the range = additional resp. acidosis and if below = prim resp alkalosis
Describe the findings in Fanconi Anemia…”BASE”
B- bone marrow problems leading to aplastic anemia and BM failure
A- appearance is short, small head, abnormal thumbs, and hypogonadism
S- skin has hypo/hyperpigmented areas, cafe au laid spots and large freckles
E- ears and eyes are affected, strabismus, low-set ears, deafness, infections
*autosomal recessive
When do communicating hydroceles start becoming a risk for indirect inguinal hernia? What would you do next?
Communicating hydrocele beyond 12 months of age is unlikely to resolve spontaneously - surgical intervention warranted.
The most common manifestations of polycythemia in newborns are?
Resp. distress, poor feeding, and neuro manifestations
Iron deficiency in infants is most commonly caused by?
Maternal iron def, prematurity, early introduction of cows milk before 12 months; full term kids normally are iron def as of 6 months but are subsequently started on pureed foods
What do you supplement in a breastfed infant of a veggie mother?
Vit B12 and Vit D- Vit D of 400 units for all breastfed infants
Infant/newborn with failure to thrive, bilateral cataracts, HSM, weight loss, convulsions, jaundice and hypoglycemia = ? Caused by defect in?
Galactosemia 2/2 galactose-1-phosphate uridyl transferase def = elevated galactose levels
*Risk of neonatal sepsis 2/2 E. Coli
** Galactokinase def = cataracts only
Parents with migraines with children who have recurrent vomiting of unknown origin = ?
Cyclic vomiting - reassurance
Transient Tachypnea of the Newborn = ?
Tachypnea that begins after birth but resolves by day 2 - see bilateral perihilar linear streaking 2/2 inadequate alveolar fluid clearance at birth
Persistent pulmonary hypertension of the newborn should be suspected in ?
All terma nd post-term infants - can see R-L shunting through foramen oval and ductus arteriosus - decreased pulmonary vascular markings with clear lungs
What analgesic is preferred in alcoholic pancreatitis?
Meperidine or Fentanyl is favored over morphine
MC organ to be affected with Mumps =
Testes
Two common causes of “floppy baby”
Botulism (canned/jarred foods, honey); Werdnig-Hoffman Syndrome
Congenital Hypothyroidism can occur 2/2 what three mech? How would the child present?
Can be 2/2 to congenital thyroid aplasia (MC), poor production of hormone, or transmitted Ab’s transplacentally. Child is fine at birth but then becomes: weak, hypotonia, large tongue, sluggish movement, refractory macrocytic anemia, hypothermia, bloating and UMBILICAL hernia!
Risk factors for constipation in young kids?
Lots of cow’s milk intake, switching to solids, starting new school, potty training.
Trmt = more fiber, more water, < 24 oz cows milk, sit on the toilet after each meal. Polyethylene glycol or Mineral Oil = oral treatment options
newborns with elevated palatal arch, 4 metacarpal, nail dysplasia + webbed neck?
Turners - SCREEN FOR HORSESHOE KIDNEY! Edema is 2/2 poor lymphatic system
Duchenne muscular dystrophy has what two characteristic PE signs/movement? Dx test?
Bilateral calf pseudohypertrophy 2/2 to Gower’s sign (walking themselves up to stand). GENETIC testing = gold std to show deletion of Dystrophin Gene on Xp21. Muscle biopsy would should fibrosis and fatty infiltration of the muscle as it’s slowly replaced (absent dystrophin also noted).
Type 1 RTA, Type 2, Type 4?
These are normal anion gap metabolic acidoses.
T1 = Defect in hydrogen secretion = acidotic, hypokalemic, with elevated urinary pH; pt commonly has nephrolithiasis
T2 = decreased bicard reabsoprtion in prox tubule. Fanconi = CC
T4 = sodium/potassium exchange problem in distal tubule = hyperkalemic, hyperchloremic acidosis
CAN PRESENT AS GROWTH FAILURE IN KIDS!!!
Paget’s Disease has what lab values?
Normal Ca, Normal Phosphorus, Elevated Alk Phos and elevated urine byproducts of bone breakdown
Two diseases aspirin is not contraindicated for?
Kawasaki and Juvenille Idiopathic Arthritis
NSAIDS can cause SIADH? T/F
True
Whats the treatment with a foot deformity with adducted anterior aspect of the foot with convex lateral border and concave medial border?
If the Metatarsus Adductus can be passively moved, reassurance…if not, cast
Myotonic dystrophy (extension of CTG on chromosome 19q) has what muscle weakness distinguishing it from Duchenne and Becker?
Facial weakness, hand grip, myotonia, dysphagia. Also would see cataracts, balding, and testicular atrophy in these kids (occurs later 12 - 30 yrs). Duchenne is most severe with absent dystrophin and Becker is less severe with mild dystrophin
What would you find on ECG and Xray with Tricuspid atresia?
Hypoplastic Right ventricle (unused), a VSD/ASD are necessary, Left axis deviation seen with peaked p waves, poorly developed pulm vasculature so decreased lung markings
vs.
Ebsteins anomaly (associated with Lithium) that has the hypo plastic Right Ventricle too but the TV is still patent thus allowing for TR to cause RAD and peaked P waves too with RIGHT axis deviation.
Chromosome 11p15, fetal macrosomia, omphalocele OR umbilical hernia, macroglossia, and HEMIHYPERPLASIA = ?
Beckwith-Wiedemann Syndrome. Complications = Wilms tumor and hepatoblastoma. Survey with serum alpha fetoprotein and abdominal/renal U/S
Erythematous. scaly lesions around eyebrows, sides of nose and scaly scalp?
Seborrheic Dermatitis - treat with moisturizers, topical antifungals, and anti-dandruff shampoo, and steroids
MCC of urinary tract obstruction in newborn boys with bladder distention, bilateral hydronephrosis, and bilateral hydrometers = ?
Posterior Urethral Valves causing Potter Sequence = flat facies, feet, oligo hydramnions and poor pulm development
Confirm with voiding cystourethrogram and cystoscopy. Risk of renal failure.
Where in the bone is Ewing’s Sarcoma MC found? Xray shows>? Trmt>?
Metaphysis and diaphysis of the femur followed by the tibia and humerus. See pain and swelling
Lamellated appearance or onion skin periosteal reaction. Lytic, central, and has endosteal scalloping with extension into the soft tissue.
Trmt = Surgery, Radiation, and Multi-Drug Chemo (latter two used preoperatively)
*mets to the lung and lymph nodes early b/c aggressive
Gold std for dx of myocarditis (MCC?)?
Myocardial biopsy - Coxsackie B and Adenovirus. But Echop is best at viewing hearts function.
Give diuretics, inotropes and close monitoring!
Bronchiolitis is a common winter respiratory tract infection caused by ? In kids > 2 it’s usually self limiting but in < 2 it can….
RSV - wheezing and crackles and respiratory distress. < 2 months are especially high risk for apnea and respiratory failure!
If the child has bronchopulmonary dysplasia, prematurity or severe congenital heart disease, prophylais with Palivizumab
Constitutional growth delay is characterized by what three things?
Delayed growth spurt, delayed puberty, and delayed bone age
Classic cause of diastolic dysfunction?
Systemic Hypertension
Those in close contact with Pertusis should get what prophylaxis?
If < 1 month, Azithromycin for 5 days, > 1 month of age = Azithromycin or Clarithromycin or Erythromycin ….everyone should get antibiotic prophylaxis AND immunized if not already done so
Young patient with cafe au laid spots and problems hearing?
Neurofibromatosis with possible acoustic neruomas - get MRI with Gadolinium
ALL = >25% lymphoblasts = diagnostic; would see what kind of immunostaining?
Strong periodic acid Schoff reaction and terminal TdT positivity.
Hernia repair advisable for: ?
Most disappear by 1 year of age. Surgery si advised if persists beyond 3-4 yrs of age, > 2cm, causes symptoms, becomes strangulated, or gets largers after the age of 1-2 yrs.
Micrognathia, microcephaly, rocker bottom feet, overlapping fingers and absent palmar crease? Cardiac abnormality?
Edwards = Trisomy 18
VSD = MC
- vs. ASD and endocardial cushion defects in tri 21
- PDA in Cong Rubella
In kids, look for what distinguishing features of bacterial vs. viral pharyngitis?
Viral = cough, rhinorrhea, conjunctivits, oral ulcers
Bacteria = exudates, edema, palatal petechia
Need a neg throat culture even with negative rapid antigen test
Trmt = PCN or amox
Triple bubble can be caused by maternal history of?
Vasoconstrictive meds or drugs like coccaine and tobacco. See atresia of the jejunum or ileum
Acute unilateral cervical adenitis in children = caused by?
Strp or Staph - I/D + Clindamycin
Laryngomalace present with?
Inspiratory stridor that worsens in the supine position and with crying and peaks at age 4-8months.
Laryngoscopy shows collapse of the supraglottic structures during inspiration - usually self resolves by 18 months
Stridor is a squeaky, whistelike
Supraglottoplasty can improve breathing and is indicated with dangerous situations like apnea, cyanosis, poor weight gain or worsening stridor
- Vascular rings = biphasic stridor
Pneumococcal vaccine boosters are required every ___ yrs s/p splenectomy?
5 yrs
Waterhouse-Friderichsen Syndrome
Sudden vasomotor collapse and skin rash in kids 2/2 adrenal hemorrhage and fulminant meningococcemia
All kids with night exacerbated episodes of pruritus should get what test and trmt?
Scoth tape test for pinworms - treat with mebendazole
Preventing sickle cell pneumococcal sepsis =
Pneumococcal vaccination; and twice daily penicillin until age of 5
Whooping cough has three phases ? Trmt?
Catarrhal, paroxysmal, and convalescent. Give macrocodes as first line in catarrhal stage! Can have long coughing spells, worse at night, staccato cough, pottussive emesis. Lymphocyte predominate leukocytosis
What’s common cause of laryngotracheobronchitis (croup) in children?
Parainfluenza = barky cough with inspiratory stridor
RSV = MCC?
Bronchiolitis in children < 2 = fever rhinorrhea, cough and mild respiratory distress.
Transposition of great vessels is a cyanotic heart disease commonly seen in what mothers?
Diabetic mothers
This tumor is most popular in the metaphases of long bones and has soft-tissue tender mass, X-ray is speculated with sunburst appearance and there is periosteal elevation = ?
Osteosarcoma = MC primary bone tumor in adults and kids.
Young 2?
2 and recurring = anatomical thing - look for Meckels!
Genotype is what with short stature, delayed or absent puberty, low FSH/LH, absent GnRH, anosmia ?
XX For Kallman syndrome = normal genotype
Kid with transient synovitis is the MCC of hip pain in children - work up?
If they look sick and can’t bear weight, then tap the joint, if not can give NSAIDS and follow up in one week + X-rays to r/o LCP
Viral infection + isolated thrombocytopenia and NO bleeding just petechiae>?
Observe - most resolve by 6 months - if bleeding then give IVIG or steroids
In adults, if Plt < 30,000 treat if over this and NO bleeding, observe
What vitamin reduces mortality in kids with measles?
Vitamin A
