Peds UWorld Flashcards
Macrocytic anemia, low retic count, and congenital anomalies?
- think: diamond-blackfan syndrome
- AKA: congenital hypo plastic anemia
5 Early Ssx of congenital syphilis?
- Hepatosplenomegaly
- Cutaneous lesions
- Jaundice
- Anemia
- Rhinorrhea
Metatarsus adductus: what is it? Types? Tx?
- congenital foot deformity
- more commonly seen in first born infants
- 3 types & tx:
1. Type I - mild, feet over correct with passive and active mvmnt - tx = reassurance
2. Type II- feet correct to mid line with passive and active mvmnt - tx = orthosis (corrective shoes)
3. Type III - rigid feet that do not correct at all - tx = serial casts
What cancers does breast feeding reduce the mothers risks for?
- Breast
2. Ovarian
Patient with Down syndrome who presents with upper motor neuron findings?
-think: alantoaxial instability
Genetics of fragile X?
-mutation in FMR1 gene, caused by an increased number of CGG trinucleotide repeats
What is Reye syndrome AKA?
-fatty liver encephalopathy
What is given to close a PDA?
- indomethacin
- potent prostaglandin inhibitor
What is given to keep PDA open?
-prostaglandin E1
Alantoaxaial instability: what is it? Who is it commonly seen in? Ssx?
- presents over a few weeks
- commonly seen in Down syndrome
- due to laxity of transverse ligament that causes spinal cord compression btwn the atlas and axis
- upper motor neuron ax present
- Ssx: behavioral changes, torticollis, urinary incontinence, & vertebrobasilar Ssx (dizziness, vertigo, diploplia)
Asymmetry of inguinal folds in an infant or neonate?
- worry about developmental dysphasia of the hip
- get ultrasound (if < 6 mnths old) or xray ( > 6 mnths)
What should be done with an anyphylacyic rxn, encephalopathy, seizure, or any other CNS complication occurs within 7 days after getting DTaP?
- give DT should be given instead
- adverse rxns are assumed to be from the pertussis portion
What is the most common cause of communicating hydrocephalus?
-subarachnoid hemorrhage
Marfan features + mental retardation + thromboembolic events + downward dislocation of lenses?
-homocystinuria
Bruton’s agammaglobulinemia: what immunoglobulin concentrations are seen?
- igA, igM, igD, and igG are decreased (ALL)
- absence of B cells too!
Homocystinuria: what is it? Cause? Genetics? Tx?
- autosomal recessive
- caused by cystathionine synthase deficiency
- tx: high doses of bit B6
4 Ssx of homocystinuria?
- Marfan’s features
- Mental retardation
- Thromboembolic events
- Downward dislocation of lenses
Tx for tricyclic antidepressant intoxication?
-sodium bicarbonate
How is chlamydial conjunctivitis prevented?
- maternal prenatal testing and tx of mothers chlamydia
- ophthalmic prophylaxis given at birth does not prevent chlamydial conjunctivitis, only gonorrheal
5 Reasons an umbilical hernias should be referred to surgery?
- If they persist to age 3-4yrs
- Larger than 2 cm in diameter
- Causes symptoms
- Becomes strangulated
- Enlarges progressively from ages 1-2
Caput succedaneum?
- diffuse swelling of the scalp
- can be ecchymotic (bruised/discolored)
- can extend across the midline and across suture lines
3 Complications of orbital cellulitis?
- Orbital abscess
- Intracranial infection
- Cavernous sinus venous thrombosis
Metaphysis?
-wide portion of a long bone
Hemarthrosis after minor trauma in a young child?
- suggests bleeding disorder
- ex hemophilia
Tx for infants when ruling out sepsis: in a < 28 day old? In an infant > 1 mnth?
- < 28 days = ampicillin & gentamicin
- > 1 mnth = vancomycin & ceftriaxone
Triad of HUS?
- Thrombocytopenia
- Anemia
- Renal failure
3 Ssx of necrotizing enterocolitis? 2 findings on xray, why?
- Increased gastric residual volume
- Vomiting
- Abdominal distention
* *in a preterm neonate!!!!!
- xray: pneumatosis intestinalis + portal venous air! –> air is from gas produced by bacterial overgrowth
3 Most common causes of acute bacterial rhinosinusitis?
- Strep pneumo
- Nontypeable H. Influenza
- Moraxella catarrhalis
Tx for strawberry hemangiomas?
- none!
- usually regress by ages 5-8 yrs
Most common cause of osteomyelitis in infants and kids?
-staph aureus
What is the most common cause of cerebral palsy?
-cerebral anoxia
“Target sign” on abdominal ultrasound?
-intussusception
Classic triad of congenital toxoplasmosis?
- Chorioretinitis
- Hydrocephalus
- Intracranial calcifications
Tx for impetigo?
- Topical mupirocin
2. Oral erythromycin
What is thought to be the best way to decrease the rates of necrotizing enterocolitis in premature infants? Why?
- breast feeding all premies if possible!
- why: premies intestines may have increased permeability due to immaturity, breast milk may help counteract this
What is the most common cause of secondary HTN in kids?
-fibromuscular dysplasia
Wiskott-Aldrich syndrome triad? Most common of the 3?
- Thrombocytopenia (most common!) –> due to decreased platelet production
- Eczema
- Recurrent bacterial infections
What are the 3 causes if anemia of prematurity?
- Transitions in erythropoiesis sites of the neonate
- Shorter lifespan of RBCs in neonates
- Low fetoplacental transfusion –> occurs when the baby is held above the level of the placenta after delivery
Iron deficiency anemia in infants: common? Presentation? 3 things that increase risk?
- most common nutritional deficiency in infants and children
- often asymptomatic
- full term infants are born with enough iron stores to prevent them from developing deficiency until age 4-6 mnths, regardless of dietary intake
- increase risk:
1. Maternal iron deficiency (w/ breast feeding)
2. Prematurity
3. Cow’s milk before age 12 mnths
Describe the murmur of VSD?
-pansystolic murmur that is loudest at the left lower sternal border + diastolic flow murmur at apex
Tx for OCD?
- high-dose selective serotonin reuptake inhibitors (ex. Fluoxetine)
Cyclic vomiting syndrome: what is it? Etiology? Possible complications? Tx?
- recurrent, self-limiting episodes of vomiting and nausea in children in the absence of any apparent cause
- etiology: unclear, but more common in pts who have parents with migranes
- complications: dehydration and anemia
- tx: anti-emetics & reassurance
4 Common ssx of necrotizing enterocolitis? What is seen on xray?
- Feeding intollerance
- Increasing abdominal girth
- Bloody stools
- Vomiting
- see pneumatosis intestinalis on xray
3 Ssx of niacin deficiency?
- Diarrhea
- Dementia
- Dermatitis
* *vitamin B3 deficiency = pellagra
For how long is bed wetting normal?
- until age 5
- boys usually do it longer than girls
Todd’s paralysis?
- postictal paralysis that can follow generalized or focal seizures
- motor deficit rapidly improves and is back to normal within 24 hrs
- usually indicates a structural abnormality underlying the seizures
Absent or hypoplastic thumbs + depression of all cell lines? Pathophysiology?
- think: fanconi anemia
- pathophysiology: spontaneous chromosomal breaks
Breastfeeding failure jaundice v. Breast milk jaundice: what is it? When does it present? Tx?
failure jaundice:
-feeding insufficiency results in insufficient caloric intake
-occurs in 1st week of life
-tx: promote and support successful breast feeding
Milk jaundice:
-factor of human milk increases bilirubin enterohepatic circulation
-unconjugated hyperbilirubinemia from third week of life on
-tx: stop breast feeding temporarily if the bilirubin level is markedly increased, otherwise no tx
Most common cyanotic congenital heart defect at birth?
-transposition of great vessels
4 Common facial features of fetal alcohol syndrome?
- Smooth philtrum
- Thin vermillion border
- Small palpebral fissures
- Microcephaly
Legg-Calve-Perthes disease: what is it? When is it commonly seen? How does it usually present? Dx?
- what: idiopathic osteonecrosis (avascular necrosis) of the femoral head
- when: boys 4-10 yrs, most commonly 7 yrs
- how present: pain of insidious onset and antalgic gait
- Dx: may take a while for changes to be shown on xray, clinical suspicion needed, and repeat xray
Reye syndrome: what is it? Common presentation? Tx?
- rare syndrome that is seen only in children <15 who are treated with aspirin for a viral illness
- common presentations: vomiting, agitation, and irrational behavior, progresses to lethargy, stupor, and restlessness
What is the most common congenital heart disease associated with Edward’s syndrome?
-VSD
What is the most common cause of pulmonary infections in CF pts? Tx?
- pseudomonas aeruginosa
- tx: aminoglycoside (ex tobramycin) + antipseudomonal penicillin (ex piperacillin)
4 Red flags for orbital cellullitis?
- Decreased visual acuity
- Diplopia
- Opthalmoplegia
- Proptosis
Syncopal episode (w/out post-episode confusion) + hearing impairment + family hx of sudden cardiac death?
-think: congenital long QT syndrome
4 Ssx of intussusception?
- Sudden onset of abdominal pain
- Vomiting
- Red currant jelly stools that contain blood and mucus
- Abdominal mass
Tx of intussusception?
-air or water-soluble contrast enema
What are 3 classical signs of posterior urethral valves that can be seen on prenatal ultrasounds?
- Bladder distension
- Bilateral hydroureters
- Bilateral hydronephrosis
What embryonic structure do neuroblastoma masses arise from?
-neural crest cells
4 Ssx of cri-du-chat syndrome? Genetics?
- Microcephaly
- Hypotonia
- Short stature
- Cat-like cry
- genetics = deletion of 5p!
Describe chronic pyelonephritis findings on IV pyelogram?
- Blunting of calices
2. Focal parenchymal scarring
What should be done about a swallowed battery?
- if in esophagus = remove
- if distal to the esophagus = most pass uneventfully, so they only need to be observed
Tx for lyme dz in kids < 8 yrs old?
-amoxicillin
Tx of necrotizing enterocolitis?
- Empiric broad-spectrum antibiotics bc of the serious risk of lethal septic shock if perforation occurs
- Surgical intervention
5 Ssx of Beckwith-Wiedermann syndrome? What must they be monitored closely for?
- Macrosomia
- Macroglossia
- Umbilical hernia/omphalocele
- Hemihyperplasia
- Hypoglycemia
- monitor closely for Wilms tumor or hepatoblastoma!!
What os the cause of meningitis that is accompanied by a petechial or purpuric rash?
-Neisseria meningitis!!!
What congenital heart defect is associated with William’s syndrome?
-supravalvular aortic stenosis
Myotonic congenital myopathy?
- muscle weakness and atrophy (esp in the distal muscles of the upper and lower extremities)
- myotonia
- testicular atrophy
- baldness
- autosomal dominant
What is the most common cause of hip pain in children? Tx?
- transient synovitis
- tx: NSAIDs + rest
What is terminal deoxynucleotidyltransferase? What is it expressed in?
- TdT
- only expresses by preB and preT lymphoblasts
- positive in 95% of ALL pts
Failure to thrive, bilateral cataracts, jaundice, and hypoglycemia in newborn?
-think: galactosemia from galactose-1-phosphate uridyl transferase deficiency
5 Ssx of measles?
- Coryza
- Conjunctivitis
- Cough
- Koplik’s spots
- Maculopapular rash that begins on face
* *5 “C” sounds!
3 most common manifestations of polycythemia in newborns?
- Respiratory distress
- Poor feeding
- Neurologic manifestations
3 Most common causes of neonatal jaundice?
- Breast milk jaundice
- Hematologic
- Sepsis –> so work up all jaundice for sepsis if you dont know the underlying cause!!
Drug of choice for congenital long QT syndrome?
-beta-blockers!!!
What occurs in partial seizures with secondary generalizations?
-tonic-clonic manifestations –> diffuse muscle aches & elevated CPKs
Max oz of cows milk that 1-5yr olds should consume a day?
-20oz!
5 Ssx of HSP?
- Palpable purpura
- Hematuria
- Abdominal pain
- Arthralgias
- Scrotal swelling –> occasionally
Triad for congenital rubella syndrome?
- Sensorineurial deafness
- Cardiac malformations
- Cataracts
What is often the earliest manifestation of sickle cell anemia in pts?
- dactylitis
- vaso-occlusion of hands and feet
What to do for a unimmunized pt exposed to varicella zoster?
- normal = give vaccine
- immunocompromised = give varicella Ig within 10 days of exposure
Pertussis prevention of close contacts of an infected pt?
-14 days of erythromycin, no matter how old they are, their immunizations, or sx!
When does VSD usually present? How?
- usually at 6 wks
- presents w/: tachypnea & failure to thrive
Parinaud’s syndrome? Where is the lesion? What is probably the cause?
- syndrome = paralysis of vertical gaze, can be associated with pupillary disturbances and eyelid retraction (Collier’s sign)
- means there is a lesion in the rostral midbrain at the lever of the superior colliculus and CN III
- usually caused by germinomas and pinealomas
Which peds patients is orthostatic proteinuria common in?
-adolescent boys
Top 3 bacterial causes of otitis media (in order)?
- Strep pneumo
- H. Influenzae
- Moraxella catarrhalis
3 Common ssx of trachoma? Caused by? Tx?
- Follicular conjunctivitis
- Pannus of the cornea = neovascularization
- Concurrent infection of nasopharynx with nasal discharge
- caused by chlamydia trachomatis
- tx: topical tetracycline or oral azithromycin
Presentation of choledochal cysts in infants?
- Jaundice
2. Acholic stools
2 tx of absence seizures?
- Ethosuximide
2. Valproic acid
Tx of galactosemia?
-galactose-free diet!
What 3 types of seizures have loss of conciousness?
- Generalized seizures
- Partial seizures with generalization
- Complex partial seizures
Orbital cellulitis: what is it? 3 Ssx?
- serious bacterial infection
- Ssx:
1. Pain with extraocular movements
2. Visual impairments
3. Opthamoplegia
Serum sickness-like rxn: what is it? When is it commonly seen?
- hypersensitivity rxn that occurs 1-2 wks after administration of certain drugs
- NOT a true drug allergy!
- common drugs: (if given for a viral illness)
1. Penicillin
2. Amoxicillin
3. Cefaclor
6 Ssx of compartment syndrome?
- Sever pain
- Pallor
- Poikilothermia
- Paresthesias
- Pulselessness –> late finding
- Paralysis –> late finding
Confirmatory test for SLE?
-anti-smith antibody test
Tx of clubfoot?
- initially: stretching and manipulation of the foot (starting immediately!)
- then: serial plaster casts, malleable splints, or taping
- if unresponsive: surgery
What happens to benign murmurs of kids when they stand up or perform the valsalva manuver?
-decreases in intensity!
What is the most common cause of chronic inspiratory noise in infants? Tx? Prognosis?
- laryngomalacia
- tx: hold child in upright position for half an hour after feeding, and never feed child when he is lying down
- usually resolves by 18mnths
What is the most common cyanotic congenital disease in kids less than 4 yrs old?
-tetralogy of fallot
Traction apophysitis of the tibial tubercle: AKA?
-Osgood-Schlatters Disease
Osgood-Schlatters Disease: AKA?
-traction apophysitis of the tibial tubercle
What are the most common fractures in pediatrics? Most common complication of this fracture?
- supracondylar fractures
- complication: entrapment of the brachial artery = loss of radial a. Pulse
Neonate with non-bilious, projectile and persistent vomiting? What to do next?
- think pyloric stenosis
- do abdominal ultrasound next
Common presentation for neonatal enterocolitis (3)?
- Abdominal distention
- Vomiting
- Frank blood in stools
What is mitral valve stenosis almost always due to?
-rheumatic fever as the result of a group A strep infection
Thymus in CXR?
- thymus can be very lg on CXR of kids < 2 yrs old
- located in the anterior, superior mediastinum, behind the sternum
- thymus can overlap the heart un infants = “sail sign”
- thymus can be lg and can be mistaken for: cardiomegaly, lung infiltrates, atelectasis, or a mediastinal mass
3 Ssx of androgen excess in males with precocious pubarche? Cause?
- Coarse axillary hair
- Coarse pubic hair
- Severe cystic acne that is resistant to tx
- caused by late-onset (AKA: nonclassical) adrenal hyperplasia caused by 21-hydroxylase deficiency
6 Indications for the evaluation of neonatal jaundice?
- Hyperbilirubinemia of > 2 mg/dL
- Jaundice that appears in first 24-36 hrs of life
- Serum bili that is rising faster than 5mg/dL/24 hrs
- Serum bili > 12 mg/dL in full-term (esp in absence of risk factors) or of 10-14 in premeies
- Jaundice persists after 10-14 days of life
- Signs or symptoms
What congenital GI issue can be seen in newborns of moms who took cocaine during pregnancy?
- jejunal or ileal atresia
- bc cocaine is vasoconstrictor & causes a vascular accident in utero
What 2 things can be seen on plain xrays and CT scans in neuroblastoma?
- Calcifications
- Hemorrhages
* * most commonly in the abdomen
“Squirt sign”
- rectal examination causes explosive expulsion of gas and stool from temporary relief of an obstruction
- positive in hirschsprung
- negative in meconium ileus
Scarlet fever v kawaski, whats the big feature that can help separate them?
- scarlet fever: NO mucous membrane involvement = NO conjunctivitis
- Kawasaki dz: mucus membrane involvement = conjunctivitis!
Tx/workup of simple febrile seizures?
- no work up needed
- tx: reassurance
3 Ssx of cholesteatoma?
- New-onset of hearing loss
- Chronic ear drainage despite antibiotic tx
- Granulation and skin debris seen on otoscopy
Cholesteatoma?
- destructive and expanding cystic growth in the middle ear and/or mastoid proccess
- consists of keratinizing squamous epithelium
- can be acquired (secondary to chronic middle ear infection) or congenital
3 Ssx of McCune Albright syndrome?
- Precocious puberty
- Cafe au lait spots (Pigment) = they are lgr than in NF, have irregular borders, and not in axilla or groin
- Multiple bone defects = Polyostotic fibrous dysplasia
* * 3 Ps
5 Possible long term sequelae associated with bacterial meningitis?
- Hearing loss
- Loss of certain cognitive functions
- Seizures
- Mental retardation
- Spasticity or paresis
Pancytopenia following drug intake, exposure to toxins, or viral infection?
-Think: aplastic anemia!
When is chelation tx used in lead posioning?
-when the serum lead levels are 45 or greater
What is the cut off for small for gestational age?
-Birth weight less than the 10th percentile
What is PANDAS?
- Pediatric Autoimmune Neuropsychic Disorders Associated with strep infections
- onset of OCD ssx after a group A strep
3 Common features of IgA deficiency?
- Recurrent sinopulmonary infections (w/ strep & hemophilus)
- Recurrent GI infections (w/ giardia)
- Anaphylactic reactions to transfusions
Honey-colored crusted exudates?
- impetigo!
- ALWAYS when you see those words!!!!
Tx for immune thrombocytopenia?
-corticosteroids are the drug of choice, but only if the platelet count is <30,000
What should be obtained when a hemophilia is suspected?
-factor VIII and IX levels
Growing pains?
- bilateral lower extremity pains that occur at night in kids aged 2-12 yrs
- no systemic ssx, normal activity levels, normal physical exam!
4 abnormalities in tetrology of fallot?
- VSD
- Overriding aorta
- Pulmonary stenosis
- RVH
What causes measles?
-paramyxovirus
Presentation of Late-onset CAH in females?
- presents in adolescence with:
1. Acne
2. Irregular menses
3. Mild hirsuitism - hard to distinguish from PCOS sometimes
Constitutional growth delays?
- Delayed growth spurt
- Delayed puberty
- Delayed bone age w
What type of seizure is an aura indictive of?
-partial seizure from a specific focus of the brain
What does RSV increase the risk for?
-asthma later in life
5 Ssx of henoch-schonlein purpura?
- Palpable purpura on the lower extremities
- Arthralgias
- Abdominal pain
- Renal dz
- IgA depsition in the kidney on immunoflorescence
* *vasculitis of small vessels
2 Causes of “floppy baby” syndrome?
- Infant botulism
2. Werdnig-hoffman syndrome
Tx for tinea corporis?
- antifungal
- ex terbinafine
What is a “Sunburst” on x-ray classical for?
- osteosarcoma
- most common primary bone tumor in children and young adults
- usually seen at the metaphysis of long bones
Preterm infant with respiratory distress that is not responding to oxygen tx? Tx?
- think: hyaline membrane dz!
- tx: mechanical ventilation + surfactant
Friedreich ataxia: what is it? What 3 organ systems are involved? 2 Most common causes of death?
- most common type of spinocerebellar ataxia
- 3 systems:
1. Neurologic = ataxia & dysarthria
2. Skeletal = scoliosis & foot deformities
3. Cardiac = concentric hypertrophic cardiomyopathy - 2 Most common causes of death:
1. Cardiomyopathy
2. Respiratory complications
What should be done in a child < 24 mnths with their first febrile UTI?
- tx with 7-14 days of oral antibiotics
- renal and bladder ultrasound for any anatomical abnormalities
Wilms tumor tx and prognosis?
- lungs are the most common site metastatic spread, but children rarely present with pulmonary sx
- tx: surgery + chemo
- survival rates are excellent, esp in earlier stages of dz
Tx of migraines in peds?
- acetominophen or other NSAIDs
- supportive management
Toddler with firm, smooth, unilateral mass and hematuria?
-think: Wilms tumor
Most common cardio malformation in marfan’s syndrome?
-aortic root dilation
What is the most common cause of polycythemia in term infants?
- delayed clamping of the umbilical chord –> excess transfer of placental blood to infant
- hematocrit > 65%
Decreased pulmonary markings on CXR?
-means there is pulmonary undercirculation
Spondylolisthesis?
- developmental disorder with a forward slip of the vertebrae that mainfests in preadolescent kids
- ssx:
1. Back pain
2. Neurologic dysfunction (ex urinary incontinence)
3. Palpable “step-off” at lumbosacral area
Failure to pass meconium within first 24 hrs?
-think: hirschsprung disease
Premature pubarche: what is it? Clinical significance?
- pubic hair growth < 8 yrs old
- 50% of the time associated with a CNS disorder
Whey v. Casein in breast milk?
- 70% whey
- 30% cassein
- whey is more easily digested & it helps to improve gastric emptying
Chronic granulomatous disease?
- defect in NADPH-oxidase system of phagocytic cells
- prone to abcesses secondary to catalase-producing organisms, like aspergillius and staphylococcus
What is the fluid of choice when resuscitating in severe hypovolemic hypernatremia?
-isotonic, normal saline = 0.9% saline
What are the most common causes of pediatric myocarditis? How do they typically present (4)?
- viruses: 1. Coxsackie 2. Adenovirus
- presentation:
1. Lethargy
2. Fever
3. Signs of heart failure
4. Viral prodrome
Renal tubular acidosis: in general what is it?
- normal anion gap metabolic acidosis
- caused by a defect in the ability of the renal tubules to resorb bicarb or excrete hydrogen
- there are 3 types
Werdnig-Hoffman syndrome?
- degeneration of anterior horn cells and cranial nerve motor nuclei
- causes floppy baby syndrome
- autosomal recessive
2 Ssx of aplastic crisis in sickle cell anemia? What is it? What can cause it? Tx?
- Acute severe anemia
- Low or absent reticulocytes
- arrest of erthropoiesis
- can be caused by infections, ex. Parvovirus B19
- tx: blood transfusion
Describe the ventricular septal defect ,urmur?
-holosystolic murmur that is the loudest at the left lower sternal border
Tx of pertussis?
- macrolides (ex amoxicillin)
- tx should be based on clinical suspicion, dont wait for the confirmatory dx
Pediatric CNS tumors: what is more common supratentorial or infratentorial? What is the most common type?
- infratentorial is more common
- astrocytomas are the most common histologic type
What is the nursemaid’s elbow? Ssx?
- subluxed radial head that results from a pulling injury
- ssx: child keeps hand in pronated position and cries/refuses attempted forearm supination
Serum iron and TIBC in iron deficiency anemia?
- serum iron is decreased
- TIBC is increased
Glucose in CSF for bacterial v viral meningitis?
- bacterial = low glucose
- viral = normal glucose
Olive-sized mass palpable in the abdomen?
-pyloric stenosis!!
What does posterior vermis syndrome cause?
-truncal dystaxia
Juvenile angiofibroma: tx?
- some dont need tx
- bc can bleed easily, should only be touched by specialists
- tx is required if its:
1. Enlarging
2. Obstructing airway
3. Causing chronic nosebleeds
Niemann-Pick’s disease?
- sphingolipidosis due to a deficiency in sphingomyelinase
- sphingomyelin accumulates in the reticuloendothelial cells of the liver, spleen, bone marrow, and brain
Most common heart defect in infant of GDM?
-TGA
Most common cause of sepsis in a sickle cell anemia pt? Why?
- strep pneumo
- its encapsulated and they are functionally asplenic
What are 2 important predisposing factors of brain abcesses?
- Congenital heart disease
2. Recurrent sinusitis
Ssx of Riboflavin deficiency?
- non-specific and usually mild
- rarely seen in industrialized countries!
- B2
- Ssx: sore throat, hyperremic and edematous oropharyngeal membranes, cheilitis, stomatitis, glossitits, normocytic-normochromic anemia, seborrheic dermatitis, and photophobia
Precocious puberty, cafe au lait spots, and multiple bone defect?
-McCune-Albright syndrome
Cradle cap?
-seborrheic dermatitis
Inspiratory “whoop”?
- pertussis
- AKA: “whooping cough”
If an infant is infected with Hep B via vertical transmission, what is the chance that they will progress to chronic hepatitis?
-90% chance
What is commonly seen with complex partial seizures?
-automatisms during loss of conciousness
Normal anion gap metabolic acidosis in infants that causes a FTT?
-renal tubular acidosis
What does it mean if a murmur changes with position?
-its probably an innocent murmur
What is the deficiency in Lesch-Nyhan syndrome?
- hypoxanthine-guanine phosphoribosyl transferase (HgPRT)
- think: “he’s got purine recovery trouble”
First-line tx of bacterial OM?
-10 day course of amoxicillin
Most common cardio congenital defect in down syndrome?
-endocardial cushion defect
5 Possible complications of small for gestational age?
- Hypoxia
- Polycythemia
- Hypoglycemia
- Hypothermia
- Hypocalcemia
Genes coding for what are mutated in osteogenesis imperfecta?
-type I collagen
What is the most common presentation for meckle’s diverticulum? In what ages?
- painless melena
- 2-3 yr old kids
Tx of preseptal cellulitis v orbital cellulitis?
- preseptal cellulitis = outpatient oral antibiotics
- orbital cellulitis = inpatient IV antibiotics
Most common early symptom of Cavernous sinus thrombosis?
-headache that eventually becomes unbearable
Hemophilic arthropathy?
- consequence of recurrent hemarthrosis
- seen in hemophilia A & B
- associated with hemosiderin deposition in the joint space –> synovitis and fibrosis
Typical features of glucose-6-phosphatase deficiency?
- 3-4mnth child presenting with:
1. Hypoglycemia
2. Lactic acidosis
3. Hyperuricemia
4. Hyperlipidemia
5. Characteristic doll-like face (fat cheeks)
6. Thin extremities
7. Short stature
8. Protuberant abdomen (from enlarged liver)
Left axis deviation on EKG and decreased pulmonary marking on CXR in a neonate?
- think: tricuspid valve atresia
- will see cyanosis in child
- normally infants are born with a large right ventricle, which will be seen as right axis deviations on EKG
Ewing sarcoma: what is it? Who is it usually seen in? Common x-ray findings?
- What: second most common primary bone malignancy in kids
- who: most commonly seen in adolescent boys
- xray: osteolytic lesions + periosteal rxn that looks like “onion skin”
What portion of the bowel is usually affected in hirschsprung disease?
-rectosigmoid
Acute fever, joint pain, turbid synovial fluid, neutrophil-predominant leukocytosis? Tx?
- think: septic arthritis
- tx: emergency surgical drainage + IV antibiotics
Tx for an infant born to a mom with active hep B?
- passive immunization at birth with hep B immune globulin
- plus recombinant HBV vaccine
Most common predisposing factor for orbital cellulitis? 2 Reasons why?
- bacterial sinusitis
- why:
1. Proximity
2. The orbital venous system is valveless!!
Tx of cat bites?
-should be tx prophylactically with a five-day course of ammoxicillin/clavulante (augmentin)
5 Major criteria for rheumatic fever?
- use Jones criteria (think “J<3 - carditis
3. subcutaneous Nodules
4. Erythema marginatum
5. Sydenham chorea
Treatment of TET spell?
- knee-chest position
- beta-blocker to decrease afterload
Strawberry v. Cherry hemangioma?
- strawberry in infants
- cherry in adults
5 Common physical features of Edward’s syndrome?
- Micrognathia
- Microcephaly
- Rocker bottom feet
- Overlapping fingers
- Absent palmar creases
3 Main Ssx of prader-willi syndrome?
- Hypotonia
- Hyperphagia
- Obesity
What is the best thing to do with a child who is reluctant to potty training?
-take a break from potty training for a couple months and let him/her get interested in potty training on their own
What is the most common cause of intestinal obstruction during the first two years of life?
-intussusception
Collier’s sign?
- eyelid retraction
- usually due to a lesion in the rostral midbrain
Tx for kawasaki dz?
- Aspirin
2. IV Ig
Gastroschisis: what is it? How does it present? Initial tx?
- congenital abdominal wall defect
- bowel protrudes through defect
- bowel is not covered by membrane
- bowel looks “angry” & matted
- usually not associated with any other abnormalities
- tx: cover bowel with sterile dressings ASAP!
What leukemia has a strong PAS rxn?
-ALL
2 most common organisms that cause orbital cellulitis?
- Strep pneumo
2. Staph aureus
What is the most common cause of anemia in sickle cell patients?
-chronic hemolysis
Fibromuscular dysplasia: physical exam? Which side is more often effected? What is seen on angiogram?
- PE: hum or bruit in costovertebral angle due to well-developed collaterals
- right renal a. is often more affected than left
- angiography: see “string of beads” sign
7 Ssx of congenital hypothyroidism?
Normal at birth & gradually develop:
- Apathy
- Weakness
- Hypotonia
- Large tongue
- Sluggish movements
- Abdominal bloating
- Umbilical hernia
Diagnostic study of choice for an acute alkali ingestion?
-upper gastrointestinal endoscopy
Common Wilms tumor presentation?
- age 2-5 yrs old
- asymptomatic abdominal mass found incidentally
- most often unilateral
2 Complications of HSP?
- Intussusception –> bc of bowel wall edema
2. Gastrointestinal hemorrhage
What type of seizures has automatisms?
-complex partial seizures
What is the most common cause of acute and recurrent headaches in kids?
-migraines
What should be done when intravenous access cannot be obtained in an emergent peds case?
-intraosseous access should be tried next!
Sfx of desmopressin?
- if too much water is consumed it can cause hyponatremia and seizures!
- used for enuresis
Inflamed, fluctuant cervical lymph nodes are cause by which 2 bacterial infections usually? Tx?
- Strep
- Staph
- tx: dicloxacillin
Langerhans histiocytosis: how does it present? Prognosis? Tx?
- solitary, lytic, long bone lesion
- lesions can be painful, have overlying tender swelling, and can cause pathological fractures
- prognosis: can be locally destructive, but typically resolve spontaneously
- tx: conservative
Tx of choice for strep pharyngitis?
-penicillin
Dx of pertussis?
-Cultures and/or PCR of nasopharyngeal secretions
Osteosarcoma: what is it? Who is it typically seen in? Where? How does it typically present? What does it look like on x-ray?
- What: most common primary bone tumor in children and young adults
- who: boys ages 13-16 are most at risk
- where: usually involves the metaphyses of long bones
- presentation: large tender mass on PE, may also have elevated alk phos and lactate dehydrase (from turnover of damaged osteocytes –> worse prognosis)
- xray: classically see “sunburst” pattern
4 Most common causes of aquired torticollis? First thing for dx?
- URIs
- Minor trauma
- Cervical lymphadenitis
- Retropharyngeal abscess
- dx: cervical spine radiograph to make sure there is no fracture or dislocation!
What to do with a child who has a serum lead level of less than 20?
- recheck the level in one month
- council the parents on lead safety
Most common CAH?
-21 hydroxylase deficiency
What can be a consequence of trauma to the soft palate by a foreign body?
-internal carotid artery dissection that causes a stroke in children
Describe the murmur of mitral stenosis?
-loud first heart sound and mid-diastolic rumble
What congenital infection is the “blueberry muffin” rash seen in?
-congenital rubella
Post-tussive emesis?
- pertussis
- AKA: whooping cough
6 Ssx of cavernous sinus thrombosis (CST)?
- Headache –> early sign
- Periorbital edema –> usually starts unilaterally, but then becomes bilateral
- Exopthalmos
- Chemosis (= edema of conjunctiva)
- Papilledema
- Dilated tortuous retinal veins
Abnormal B-lymphocyte maturation?
- present after 6 mnths of age (when the levels of maternal antibodies drop)
- predisposed to recurrent sinopulmonary infections with encapsulated organisms, like H. Influenza & strep. Pneumo
- also have a deficiency in IgA = predisposes to giardia GI infections
Chemosis?
-swelling of the conjunctiva
Where is Lyme disease most common in the US?
-northeastern or upper midwestern United States
What is the expected weight and height of a child at 12 months?
- weight should triple from birth
- height should increase by 50% from birth
2 most common causes of microcytic anemia in children?
- Iron deficiency anemia
2. Thalasemmia minor
What PCO2 level is suggestive of impending respiratory failure?
-PCO2 > 40
What is a thrill? Grade?
- its a palpable murmur
- at least grade 4
What 2 ssx does Niemann-Pick’s disease have that Tay-Sach’s doesnt?
- Hepatosplenomegaly
2. Cervical lymphadenopathy
When does vascular ring stridor present? What improves it? What else can it be associated with?
- presents before age 1
- persistent stridor that improves with neck extension
- associated with cardiac abnormalities
Most common cardiovascular abnormalities in Turner’s syndrome?
- coarctation of the aorta
- aortic valve abnormalities
Transient proteinuria: what is it? Dx?
- Proteinuria that is intermittent, common in school-aged kids
- can be caused by fever, exercise, seizures, stress, or volume depletion
- dipstick test shows protein in urine –> check for hematuria –> if negative, repeat dipstick 2 other times
What does it mean to have a leukocyte adhesion deficiency?
- the leukocytes are unable to exit the vasculature and migrate to areas of infection or inflammation
Blood-stained eye discharge in a newborn?
-highly characteristic of chlamydial comjunctivits!
Cyanosis, single loud second heart sound, and no murmur in the first few hours of life? Explain why?
- transposition of the great vessels
- cyanosis: deoxygenated blood comes from the body and goes into the RA and goes back into the aorta = 2 separate, unconnected circuits
- loud single S2: aorta is closer to the anterior chest wall, so its better heard than the soft s2 of the pulmonic valave
- no murmur: foramen ovale and ductus arteriosus have closed
Jervell-Lange-Nielson syndrome?
- congenital long QT syndrome
1. Congenital deaftness
2. Syncopal episodes
3. Family Hx = autosomal recessive - molecular defects in ion channels
Seborrheic dermatitis: common locations? Tx? What can severe cases suggest?
- common locations:
1. Eyebrows
2. Nasolabial folds
3. Bases of eyelashes
4. Paranasal skin - tx: moisturizers, topical antifungals, anti-dandruff shampoos, and topical steroids
- severe cases can = immunodeficiency
Presentation of jejunial atresia? What is seen on Xray? What is one main risk factor?
- presentation:
1. Bilious vomiting
2. Abdominal distention - Xray = triple bubble sign
- risk = maternal use of cocaine and other vasoconstrictive drugs
The rash of what disease is described as “sandpaper-like”?
-scarlet fever
When is Guillain-Barre syndrome seen? Pathology?
- Ascending polyneuropathy typically seen one week after a viral infection
- pathology: involves mainly the peripheral motor nerves, but sensory and automatic can also be affected
X-linked agammaglobinemia: what is it? What is commonly seen in these pts? What is typically seen in the labs? Tx?
- what: inherited immune deficiency
- recurrent bacterial infections early in life
- labs: normal amnt if T lymphocytes, but low or absent amnt of B lymphocytes
- tx: regular infusions of IVIg
7 Contraindications to breast feeding?
Maternal: 1. Active, untreated TB 2. HIV infection 3. Herpetic breast lesion 4. Varicella infection 5. Specific meds/chemo tx 6. Street or alcohol abuse Infant: 7. Has galactosemia
2 Ssx of Waterhouse-Friderichsen syndrome?
- Sudden vasomotor collapse
- Large purpuric lesions on flanks p
* *both due to adrenal hemorrhage
What has been shown to reduce the morbidity and mortality with measles?
-vitamin A
Cause of transient synovitis?
-unknown, but usually follows a viral infection or mild trauma
When is croup most common? What are 4 common symptoms?
- most common btwn 6 mnths - 6 yrs
- ssx:
1. “Barky” cough
2. Fever
3. Rhinorrhea
4. Congestion
What is not required in neonates before an LP that is required in older kids? Why?
- head CT is not needed to look for ICP
- bc the fontanelles are still open, it is very rare that a neonate will herniate after an LP!
Choledochal cysts? How many types? Most common tupe?
- congenital abnormality of biliary ducts
- dilation of intra or extra-hepatic biliary ducts, or both
- 5 types, most common = type 1–> dilation of entire common hepatic and common bile ducts or segments of each
What acid/base abnormality and electrolyte disturbances are usually seen in pyloric stenosis?
-hypochloremic, hypokalemic, metabolic alkalosis
Neonatal abstinence syndrome: what is it? When does it usually present? Ssx? Tx?
- NAS = infant withdrawal to opiates
- usually presents in the first few days of life
- ssx:
1. Iritability
2. High-pitched cry
3. Poor sleeping
4. Tremors
5. Seizures
6. Sweating
7. Sneezing
8. Tachypnea
9. Poor feeding
10. Vomiting
11. Diarrhea
4 Common ssx of orbital cellulitis?
- Pain with eye mvmnts
- Proptosis
- Opthalmoplegia
- Diplopia
What is the most common cause of anemia in kids with sickle cell dz?
-hemolytic anemia
What fluid should be given for moderate to severe dehydration in children?
- iV bolus of isotonic fluid
Other than new born screening, how can you test for PKU?
-Guthrie test of urine = coloration test that detects the presence of metabolic products of phenylalanine in urine
Diastolic murmurs are always what?
-pathologic!
Recurrent sinopulmonary infections, recurrent GI infections, & anaphylactic rxn to transfusion?
-think: IgA deficiency!
Postpericardiotomy syndrome?
- pleuropericardial disease that occurs days to months after cardiac surgery or injury
- inflammation from surgical site can lead to reactive pericarditis, pericardial effusion, or even cardiac tamponade
- difficulty feeding can be a sx in infants
What are the three phases of pertussis? How long do they last if untreated?
- Catarrhal phase = nonspecific sx of malaise, mild fever, cough, rhinorrhea, etc –> lasts 1-2 wks
- Paroxysmal phase = severe paroxysms of coughing spells that can lead to post-tussive emesis + can have “inspiratory whoop”
- Convalescent phase = gradually decreasing frequency and severity of cough
* *can last for 3 mnths total if untreated!
Cystic calcified parasellar lesion on MRI is almost diagnostic of what?
-craniopharyngioma
Most common viral cause of myocarditis?
-coasacki
Two top viral causes of otitis media?
- RSV
2. Rhinovirus
What type of hypersensitivity rxn is allergic contact dermatitis?
- cell-mediated hypersensitivity
- type IV
- delayed
Tx for enuresis?
- desmopressin
- used only after behavior modifications and bed alarms havent worked!
When should chronic granulomatous disease be considered?
-with recurrent or unusual lymphadenitis, hepatic abcesses, osteomyelitis at multiple sites, or unusual infections with catalase-positive organisms
Abnormality in Marfan’s?
-mutation in fibrillin-1 gene
2 tx for enterobiasis?
- AKA: pinworm
1. Albendazole
2. Mebendazole
What is the next step when meconium ileus is suspected in a newborn?
- barium enema –> can break up the obstruction
- surgery required if the enema is unsuccessful
Most common cause of congenital hypothyroidism in the US?
-thyroid dysgenesis
How does infantile corarctation usually present?
- after child goes home the PDA closes and causes a left sided obstructed lesion in the heart
- child presents with heart failure and shock!
What does it mean when there is delayed umbilical cord separation?
-leukocyte adhesion deficiency type I
Adult botulism v infantile botulism?
- adult = ingestion of preformed toxin
- infantile = ingestion of the organism via food (ex honey) and produces the toxin in the intestinal tract
Presentation of choledochal cysts in children?
- Abdominal pain
- Jaundice
- Attacks of recurrent pancreatitis w/ increased amylase and lipase levels
4 Manifestations of osteogenesis imperfecta?
- Blue sclera
- Hearing loss
- Recurrent fractures
- Opalescent teeth
What 3 conditions are early childhood vision screens looking for? What are the recommended ages to do theses screens?
- Strabismus
- Amblyopia
- Refractive errors
- recommended to screen ages 0-5yrs
What is the most common pathogen that causes unilateral cervical lymphadenopathy?
-staph aureus
Dx of meckle’s diverticulum?
-technetium-99m pertechnetate scanning –> taken up by the heterotrophic gastric mucosa
Cephalohematoma: what is it? Presentation? Tx?
- subperiosteal hemorrhage
- presents a few hours after birth
- scalp swelling limited to one cranial bone
- tx: none, usually resolves in 2 wks - 3 mnths depending on the size
What 2 types of seizures can have an aura?
- Simple partial seizures
2. Complex partial seizures
What is the most common nutritional deficiency in infants?
-iron deficiency anemia
What should be done if a finger stick blood test for lead level is positive?
-should check the serum lead level, since the capillary test can give false positives
What screening test should be done in all children with behavioral concerns?
- hearing test
- undetected hearing impairment can often get confused with a behavioral disorder
5 Ssx of Niemann-Pick’s disease?
- Cherry red macula
- Protruding abdomen
- Hepatosplenomegaly
- Lymphadenopathy
- Regression of developmental milestones
Triple bubble sign and has less colon on abdominal X-ray?
-think: jejunal atresia!
Paroxysmal cough?
- pertussis
- AKA: whooping cough
When is laryngomalacia stridor most common? What worsens it? Improves it?
- most severe at ages 4-8 mnths
- persistent stridor that WORSENS in SUPINE and IMPROVES in PRONE position
Osteonecrosis?
-AKA avascular necrosis!
Cause of 80% of nephrotic syndrome in kids < 16 yrs old? Tx?
- minimal change dz
- steroids
What is the key feature in myotonic muscular dystrophy?
- Delayed muscle relaxation
- ex unable to release hand after a handshake
Beck’s triad?
- for tamponade
1. Distant heart sounds
2. Distended jugular veins (or scalp veins in infants)
3. Hypotension
What is the most common complication of HUS?
-renal damage
What conditions are pts with sickle cell trait at an increased risk for?
- Increased risk for renal issues –> most common = painless microscopic or gross hematuria –> caused by sickling in the renal medulla
- isosthenuria can also occur = inability to concentrate urine = nocturia or polyuria
Who can give consent in a child with divorced parents who have joint custody?
- only one parent is needed
- this is especially true when the decision is clearly in the child’s best interest
4 Ssx of serum sickness-like rxn to medications?
- Fever
- Urticarial rash
- Polyarthralgia
- Lymphadenopathy
Adult presentation of choledochal cysts?
- Vague epigastric or right upper quadrant abdominal pain
2. Cholangitis
What can be seen on a lateral neck Xray with retropharyngeal abcess?
- widened prevertebral space
- normally the prevertebral soft-tissue space should be narrower than the vertebral bodies
Infantile colic?
- presents by 3 wks of age
- excessive crying for more than 3 hrs a day
- more than 3 days a week
- more than 3 times a month
- usually resolves by 4 mnths
Triad of ssx of brain abcess?
- Fever
- Severe headaches (nocturnal and morning)
- Focal neurologic changes
Premature adrenarche: what is it? Organ involved? Clinical significance?
- isolated apperance of axillary hair < 6 yrs old
- generally benign, with no clinical significance
- adrenal glands are involved
What nutrient suppliment(s) should breast feed infants be given?
-vitamin D!
Most common cause of nephrotic syndrome in kids? Tx?
- minimal change dz
- steroids! Highly responsive!
When does infantile colic usually resolve by?
-usually resolves by four months
2 first line Tx for pinworm?
- Albendazole
2. Mebendazole
What are the 2 most common bacterial causes of osteomyelitis in sickle cell patients?
- Salmonella
2. Staph aureus
What age is it recommended to do meningicoccal vaccination?
-btwn ages 11-12 yrs
Purulent, foul-smelling vaginal discharge, and bleeding?
-think: vaginal foreign body
Juvenille angiofibroma: what is it? 3 Common ssx? Who is it more commonly seen in?
- benign growth that is capable of eroding and locally invading
- ssx:
1. Nasal obstruction
2. Visible nasal mass
3. Frequent nose bleeds - more common in adolescent males
Presentation of a milk protein intolerance in a neonate?
- vomiting and bloody diarrhea
- stools may have RBCs and eosinophils
- can have family hx of atopic disorder
What 2 types of newborns is respiratory distress syndrome common in?
- Premature infants
2. Infants of DM mothers
Active hep B & subsequent nephrotic syndrome?
- membranous glomerulonephritis
- usually secondary to deposition of HBeAg in the glomeruli
3 Ssx of acute iron poisoning?
- Abdominal pain
- Hematemesis
- Metabolic acidosis
2 Most common causes of viral meningitis?
- Echovirus
- Coxsackievirus
* *bot non-polio enteroviruses
What tx should precede intubation in a pt with croup?
- racemic epi
- often decreases need for intubation
What 2 types of infections are typically seen in pts with complement deficiency?
- Gonococcal
2. Meningococcal
Tx for rheumatic fever? Why?
- antibiotics (usually penicillin) regardless of whether or not pharyngitis is present at the time of exam –> prevents recurrent GAS infections
- bc of risk for developing and the progression of rheumatic heart dz
How to dx 21-hydroxylase deficiency?
-measure 17-hydroxyprogesterone –> will be increased!!
Tx of acute bacterial rhinitis?
-amoxicillin-clavulanic acid
When does separation anxiety usually occur?
-btwn 9-18 mnths