Flashcards in UWorld - Peds Deck (272):
- X-linked recessive defect in WAS protein, causing impaired cytoskeleton in leukocytes and platelets
- Eczema, thrombocytopenia, recurrent infections
Juvenile idiopathic arthritis:
- Anemia (Low HgB)
- High Ferritin
- Thrombocytosis (High platelets)
- High gammaglobulin
- High inflammatory markers (ESR, CRP)
Corkscrew pattern on barium swallow
Most common cause of cyanosis in neonatal period:
Dx = Transposition of Great Vessels
Tx = Prostaglandins to keep ductus arteriosus patent
Most common cause of viral meningitis.
Enteroviruses (e.g. group B coxsackievirus)
Complete atrioventricular septal defect is most commonly associated with _________
Patent ductus arteriosus is strongly associated with _____
Congenital Rubella Syndrome
DiGeorge is associated with what heart defect?
Tetralogy of Fallot
Acute unilateral lymphadenitis in a child
Dx: Child with cervical paravertebral mass and Horner's syndrome (ptosis, anhydrosis, miosis)
Neuroblastoma of cervical paravertebral sympathetic chain
Dx: Positive acidified glycerol lysis test
The glycerol lysis test demonstrates cellular osmotic fragility. It is used to diagnose hereditary spherocytosis.
Dx: Newborn with X-ray showing fluid in the interlobar fissures
Transient tachypnea of the newborn
Tx: self resolution in 1-3 days
Dx: genu varum
= bowlegged = rickets = vitD defi
Most common cause of acute bacterial rhinosinusitis and the treatment.
Cause: Streptococcus pneumoniae OR Haemophilus influenzae.
Tx: amoxicillin-clavulanic acid
Dx: Inspiratory stridor that worsens in the supine position and improves in the prone position
Laryngomalacia (Increased laxity of supraglottic structures)
When to use EDTA + Dimercaprol
When Pb levels are above 70. OR in lead poisoning accompanied by acute encephalopathy.
When to use succimer.
Chelation in Pb toxicity when Pb levels are between 45-69
When to use ferrous sulfate
Tx of iron defi
Henoch-Schonlein Purpura pathogenesis and symtpoms
Pathogenesis = IgA vasculitis
Symptoms = Lower extremity purpura, lower extremity arthralgia, abdominal pain / intrussusception, renal disease (hematuria)
Acute bacterial rhinosinusitis:
definition and Tx`
Definition = more than 10 days of symptoms
Tx = amoxiciliin +/- clavulinate
Medical abortion medication
Mifepristone and Misoprostol
Medication that prevents pregnancy by delaying ovulation. Effective within 72 hours after intercourse.
Genotypical male (46 X,Y), but phenotypical female
Androgen insensitivity syndrome
Live attenuated vaccines
Haemophilus influenzae type B
Dx: Periosteal elevation of femur
Codman triangle, demonstrates osteosarcoma
Lytic lesion in bone of pediatric patient
Langerhans cell histiocytosis
Location of Osteosarcoma in bone
Location of Langerhans cell histocytosis in bone
Skull, jaw, femur
Dx: Bone pain with hypernatremia
Central Diabetes insipidus caused by langerhans cell histocytosis
Dx: Blue sclerae
What type of vaccine is the rubella vaccine?
Congenital Rubella 3 clinical findings
- Sensorineural hearing loss
- Patent ductus arteriosus
Congenital Toxoplasmosis 3 clinical findings
- Diffuse intracranial calcifications
Congenital Cytomegalovirus 2 clinical findings
- Periventricular calcifications
Congenital varicella syndrome 3 clinical findings
- Limb hypoplasia
- Distinctive skin lesions (scarring)
Congenital syphilis 3 clinical findings
- Nasal discharge
- Osteoarticular destruction
Treatment of pertussis if individual is not vaccinated
Treatment for iron poisoning
Whole bowel irrigation
Deferoxamine (chelation therapy)
Symptoms of iron poisoning within 30 minutes to 4 days
- Abdominal pain
- Hypotensive shock
- Metabolic acidosis
Dx when symptoms are dysphagia, drooling and respiratory distress
Epiglottitis associated with Haemophilus influenzae type b
Dx: Tympanic membrane with peripheral granulation and some skin debris
How does Rubella present in unvaccinated adolescents (3 symptoms)?
2) Rash (cephalocaudal spread of maculopapular rash)
How does congenital Rubella (German Measles) present (3 findings)?
1) Sensorineural hearing loss
3) Paten ductus arteriosus
What is the difference between preseptal cellulitis and orbital cellulitis?
Orbital cellulitis is essentially a more severe form of preseptal cellulitis. Both preseptal cellulitis and orbital cellulitis involve eyelid erythema and swelling. However, patients with orbital cellulitis also present with pain with extraocular movements and diplopia. Preseptal cellulitis is treated with oral antibiotics. However, orbital cellulitis is treated with intravenous antibiotics and sometimes surgery.
Sandpaper rash. Dx?
Scarlet fever from streptococcus pyogenes.
Cause = Strep pyogenes
Scarlet fever: Symptoms
Symptoms = fever, pharyngitis, tonsillary erythema/exudates, strawberry tongue, tender anterior cervical lymph nodes, sandpaper rash
Scarlet fever: Lab test
- rapid strep antigen test
- throat culture
Scarlet fever: Tx
Tx = Penicillin
"slapped cheek." Dx and cause?
Dx = erythema infectiosum
Cause = parvovirus B19
Hand foot and moth disease cause.
Criteria for diagnosis of Kawasaki
5 days of fever and more than 4 clinical criteria:
- >1.5 cm cervical node
- polymorphous rash
- edema of the hands/feet
- mucosal changes (eg strawberry tongue, dry/cracked lips)
Superficial flaccid bullae on the skin of an infant. Dx?
Staphylococcal scalded skin, caused by Staph aureus
Symptoms of rubeola (measles) infection
Prodrom of cough, coryza, conjunctivitis, fever, Koplic spots)
Followed by maculopapular rash: cephalocaudal, sparing the palms and soles.
Treatment of rubeola infection for hospitalized patients
When and how to treat nasal discharge with antibiotics?
Acute bacterial rhinosinusiitis is treated with amoxicillin and clavulanate. Acute bacterial rhinosinusitis is differentiated from viral upper respiratory infection by worsening of symptoms with more than 10 days without improvement.
Treatment of acute otitis media.
2nd-line: amoxicillin-clavulanic acid
Otitis externa. Tx?
Otic antibiotic drops
Most common cause of acute otitis media
Criteria for tympanostomy tube placement
More than 3 episodes in 6 months.
More than 4 episodes in 12 months.
Sticky stools with streaks of blood. Dx?
What do you see on ultrasound for instrussusception?
What is the purpose of the Technetium-99m scan?
To diagnose Meckel diverticulum
What is the purpose of Anorectal Manometry?
To diagnose Hirschsprung disease
Best test for pyloric stenosis
Triple bubble sign. Dx?
Treatment of children with severe dehydration
IV normal saline
1 side of body is larger than the other in a baby. Dx? Complications of this Dx? How to monitor for complications?
Dx = Beckwith-Wiedemenn syndrome
Complications = Wilms tumor, Hepatoblastoma
Monitor = Serum alpha fetoprotein (hepatoblastoma) and Abdominal ultrasound (Wilms tumor)
Rare but potentially fatal complication of infectious mononucleosis. Tx?
Acute airway obstruction.
Tx = corticosteroids
Acute rheumatic fever is a complication of ______
Group A streptococcal (GAS) pharyngitis
(aka Strep pyogenes)
Complication of Kawasaki disease.
Coronary artery aneurysm
Low B lymphocyte count. Dx?
X-linked (Bruton) agammaglobulinemia (XLA)
Defect in dynein. Dx?
Primary ciliary dyskinesia
When is varicella zoster vaccine given?
2 doses at ages 1 and 4
When is postexposure prophylaxis with VZV vaccine indicated?
For incompletely immunized child age >1 year who was exposed to chicken pox within the last 5 days.
When do you administer varicella immunoglobulin?
Varicella vaccine is made from a live virus, so it is contraindicated in pregnant women, immunocompromised hosts and neonates. In these populations varicella immunoglobulin is used instead (within 10 days of exposure to chicken pox).
Lateral neck X-ray demonstrates widened prevertebral space. Dx?
Retropharyngeal abcess (RPA)
What coagulation problem do patients with cystic fibrosis develop?
Vitamin K deficiency, due to CF patient's exocrine pancreas insufficiency, they cannot absorb the fat-soluble KADE vitamins.
What clotting factors are associated with Vitamin K?
Factors II, VII, IX, X, proteins C and S
Tinea corporis (ringworm). Tx?
Clotrimazole (topical antifungal)
Diaper dermatitis. Cause? Tx?
Cause = candida
Tx = topical nystatin
Teeth appear translucent and gray. Dx?
Dentinogenesis imperfecta, due to osteogenesis imperfecta
CBC in idiopathic juvenile arthritis
Limited upward gaze. Dx?
Parinaud (dorsal midbrain) syndrome, due to Pinealoma
Respiratory distress syndrome (RDS) in neonate.
Cause: immature lungs & lack of surfactant
Risk factors: Prematurity & Gestational Diabetes
Low platelets after a viral infections. Dx?
Immune Thrombocytopenia (ITP)
Cough with vomiting. Dx? Tx?
Dx = Bordetella pertussis
Tx = Macrolide (azithromycin, clarithromycin)
Dx? Newborn with left axis deviation on EKG and decreased pulmonary markings on chest radiograph.
Tricuspid valve atresia
What heart defect is diGeorge syndrome associated with?
Truncus arteriosus, where the aorta and pulmonary artery have a common outflow.
CD3+ is what cell type?
Child with abscess that is filled with neutrophils
Chronic granulomatous disease
Organisms associated with chronic granulomatous disease
Catalase Positive organisms:
What is the defect and chronic granulomatous disease?
Deficiency of oxidative burst (Hydrogen peroxide formation)
What lab testing is used for the diagnosis of chronic granulomatous disease?
Neutrophil function testing:
1) Nitroblue tetrazolium test
2) Dihydrorhodamine 123 test
Age of three finger grasp and age of two finger grasp.
Three finger grasp at nine months.
Two finger grasp at 12 months.
At what age should a baby be able to turn head to its name.
Yellow, oily scaling on face and scalp. Dx? Tx?
Dx = Seborrheic dermatitis, associated with colonization by Malassezia species
Tx primary = Emolient, non-medicated shampoo
Tx secondary = Topical antifungal or glucocorticoids
Recurrent severely pruritic rash not related to any contact, associated with family history of asthma or allergies. Dx?
Recurrent severely pruritic rash that is related to some contact and may be associated with family history of asthma or allergies. Dx?
Scaly lesions that form round or oval plaques typically on elbows and knees. Dx?
Fine white scales on scalp only. Dx?
Painful, non-pruritic pustules with honey-crusted adherent coating. Dx? Organism?
- Staph aureus
- Strep pyogenes (Group A Strep)
Painful vescicular rash with "punched-out" erosions and hemorrhagic crusting. Dx? Organism?
- Herpes simplex type 1
Flesh-colored papules with central umbilication. Dx? Organism?
(NOT chicken pox, which is caused by varicella zoster)
Pruritic circular patch on body with central clearing and raised, scaly border. Dx? Organism?
- Trichophyton rubrum
Fever, blisters, epidermal shedding with light pressure. But NO involvement of mucous membranes. Dx? Organism?
Staphylococcal Scalded Skin Syndrome
- Staph aureus
Pt with fever and well demarcated area of erythema, but NOT epidermal shedding. Dx? Organism?
- Strep pyogenes (group A strep)
Pink sand-paper rash in a child that has recently had pharyngitis. Dx? Cause?
- Strep pyogenes (Group A Strep)
Skin blistering that covers >30% of body with involvement of mucous membranes, after starting a new medication. Dx?
Toxic epidermal necrolysis
Sharply defined perianal rash. Dx? Organism?
Streptococcal perianal dermatitis
- Group A Strep (Strep Pyogenes)
Perianal rash with satellite lesions. Dx?
Treatment of Lyme in child vs. adult
Child under 8 = amoxicillin
Over 8 = doxycycline
Child with maculopapular rash that spares the palms and soles. Dx? Tx?
Dx = measles (rubeola)
Tx= Vit A + Supportive
Koplik spots. Dx?
Dx = measles (rubeola)
What test to perform if child has psychosis but also proteinuria and hematuria?
Test for antinuclear antibodies
BECAUSE child may have
Systemic Lupus Erythematosus
Mnemonic and list of findings in SLE
RASH OR PAIN:
Rash (malar or discoid)
Serositis (pleuritis, pericarditis)
Hematologic disorders (cytopenias)
Immunologic disorder (anti-dsDNA, anti-Sm, antiphospholipid)
Neurologic disorder (seizures, psychosis)
What type of kidney injury is associated with Sickle Cell Trait?
Acute Papillary Necrosis
Children aged ________ must receive a renal and bladder ultrasound after a UTI to assess for anatomic abnormalities.
< 2 yrs
The clinical manifestations of Henoch-Schonlein purpura.
- Palpable purpura
- Abdominal pain - intrussusception
- IgA nephropathy
At what age do symptoms of pyloric stenosis usually present?
Keratomalacia = wrinkling, cloudiness of cornea
Dx = Vit A deficiency
Pancreatic pseudocyst is a complication of _________.
Biliary cysts present at what age?
< 10 yrs old
Classic Triad of symptoms for Biliary cysts.
1) RUQ pain
3) Palpable mass
Anti-tissue tranglutaminase antibody assay.
- Used for diagnosis of what?
How to diagnose baby colic.
Baby colic is a type of pain that starts and stops abruptly, often occurs at the same time every day. Diagnostic features: Crying for more than three hours per day, more than three days per week, more than three months.
Hematochezia and eczema in a one month old infant. Diagnosis?
Milk or soy protein induced enterocolitis.
Three reasons for physiological jaundice in a newborn.
1) Low hepatic UGT activity
2) Low red blood cell lifespan
3) high enterohepatic recycling of bilirubin
If an infant has tracheoesophageal fistula, what other conditions are associated with this?
Next step in management, if you suspect UTI in an infant that wears diapers.
4) Urine culture
Newborn with flattened facies, abdominal distension, clubfeet and decreased aeration of lungs. Dx? Mechanism of disease?
Urinary tract anomaly (most often posterior urethral valves) cause oliguria (small urine production) in utero, which causes oligohydramnios (deficiency of amniotic fluid), which causes the 3 findings:
1) Pulmonary hypoplasia
2) Flat facies
3) Limb deformities
A congenital abnormality where the penile urethra opens on the ventral side of the penis rather than the tip.
Most common cause of urinary tract obstruction in newborn boys
Posterior urethral valves
Normal Urine pH
4.5 - 8
Normal specific gravity of urine
1.01 - 1.03
Inability of the kidneys to concentrate urine, causing low urine specific gravity.
Normal Serum Sodium
Low Urine Specific Gravity
Sickle cell trait
- response to poor renal perfusion is hyposthenuria, but intact ADH results in normal serum sodium.
High Serum Sodium
Low Urine Specific Gravity
- due to insufficient ADH production (central) or poor ADH response (nephrogenic).
How to diagnose the type of Diabetes Insipidus. Central vs. Nephrogenic?
Water deprivation test
- hold water, administer ADH
- measure urine osmolarity in response to ADH
After pediatric UTI -- what are the indications to perform a renal and bladder ultrasound?
If the first UTI is at age 2-24 months
If this is the second or more -- UTI
Urinary test for Cystinuria
Severe periodontal infections. What is the immunodeficiecy?
Leukocyte Adhesion Deficiency
What conditions are associated with cafe-au-lait macules?
Neurofibromatosis Type 1
Symptoms of McCune-Albright syndrome
Cafe-au-lait spots (unilateral)
Polyostotic fibrous dysplasia
Multiple endocrine abnormalities
Solitary hyperpigmented lesion with density of overlying dark, coarse hairs. Dx?
Congenital Melanocytic Nevus
Newborn with gray-blue patches that are poorly circumscribed. Dx?
Congenital dermal melanocytosis (Mongolian spots)
- these fade with time
What is nevus flammeus?
Scaly erythematous patch with hair loss on scalp. Also pt has tender lymphadenopathy. Dx? Tx?
Dx = Tinea capitis (dermatophyte infection)
Tx = Griseofulvin or Terbinafine
What kind of rash presents with acute rheumatic fever?
Describe the rash in that occurs in Scarlet Fever.
What kind of imaging is performed to diagnose hip dysplasia?
Age < 4 mo = Hip ultrasound
Age > 4 mo = Hip radiograph
X-ray of femur demonstrates cortical layering and a "moth eaten" appearance. Dx?
"onion skinning" and "moth eaten" bone = Ewing Sarcoma
Where does Ewing Sarcoma present?
Diaphyses of long bone
A lytic lesion in bone surrounded by concentric layers of new bone. Dx?
"Onion Skinning" = Ewing Sarcoma
Tumor in the epiphysis with "soap bubble" appearance. Dx?
Giant Cell Tumor
Most common cause of pneumonia in otherwise healthy patients.
Nocturnal perianal pruritis. Dx? Test? Tx?
Dx = Helminth infection (Enterobius vermicularis)
Test = Tape test
Tx = Albendazole or Pyrantel Pamoate
Suspect this diagnosis if individual develops a maculopapular rash after amoxicillin.
Infectious mononucleosis (Epstein-Barr virus)
What type of rash occurs in acute rheumatic fever?
Describe the rash of Erythema Infectiosum
Erythema infectiosum = rash initially involving the face (slapped cheek) progressing to entire body
How to diagnose Acute Lymphoblastic Leukemia.
Bone marrow biopsy with >25% lymphoblasts
Pediatric patient with fever and mediastinal mass on X-ray. Dx?
Rouleaux formation on blood smear. Dx?
Lymphadenopathy, tenderness of bones, splenomegaly, petichiae. Dx?
Acute Lymphoblastic Leukemia
Franconi Anemia. Mechanism? Symptoms?
Mechanism = Inherited DNA repair defect
- Short stature
- Hypo/hyper-pigmented macules
- Abnormal thumbs
- Genitourinary malformations
Lab findings for Fanconi Anemia
- Diagnosis of Fancioni Anemia is made by exposing DNA to interstrand crosslinking agents, demonstrating chromosomal breakage
Until what age is it normal to see the thymus on X-Ray?
< 3 yrs old
When to use Palivizumab?
Palivizumab is a monoclonal antibody against RSV, used for prophylaxis of children under 2 years old who are at exceptionally high risk. High risk individuals:
- Preterm <29 wks gestation
- Chronic lung disease of prematurity
- Hemodynamically significant congenital heart disease
If an infant < 2 months has bronchiolitis -- then what complication is the infant at greatest risk for?
Apnea (temporary cessation of breathing)
First step in management of a newborn with suspected congenital diaphragmatic hernia.
Endotracheal intubation to stabilize breathing.
Congenital absence of bilateral vas deferens is associated with what condition?
Opacification of ocular lens is associated with what condition?
Intrauterine rubella infection
Which pediatric tumor crosses the midline and which one does NOT?
Neuroblastoma may cross the midline
Wilms tumor does not
Abdominal mass that may cross the midline
"Owl eyes" on lymph node biopsy.
Bone pain and pancytopenia in Pediatric pt. Dx?
Acute Lymphoblastic Leukemia
What drug to avoid in G6PD deficiency if trying to treat UTI.
Ear canal is red and swollen. Diagnosis? Mechanism?
Diagnosis = Otitis externa
Mechanism = infection with Pseudomonas aeruginos OR Staph aureus, most commonly due to water exposure ("swimmer's ear")
Treatment of Epiglottitis
Infection with Haemophilus influenzae type b.
Tx = ceftriaxone (against haemophilius influenzae type b and streptococcus species) and vancomycin (against MRSA)
What antibiotic to use to treat whooping cough?
To treat Bordetella pertussis, use Azithromycin
Empiric therapy for neonatal sepsis
Ampicillin and gentamycin
Tx for atypical pneumonia.
Organisms of atypical pneumonia = Mycoplasma pneumoniae, Clamydia pneumoniae)
Tx = Azithromycin
How to treat pulmonary infection in cystic fibrosis.
CF patients are susceptable to Pseudomona aerguinosa.
Tx = Piperacillin-tazobactam
Osteosarcoma = "sunburst" periosteal reaction and Codman triangle
Ewing sarcoma = "onion-skin" appearance
Newborn patient demonstrates a preference to tilt hear head to the left while rotating the chin to the right. Dx?
Dx = Congenital torticolis
This is a postural deformity of the neck in which the sternecleidomastoid muscle is tight and contracted, likely due to intrauterine crowding.
A congenital lymphatic malformation located in the posterior triangle of the neck
Premature closure of cranial sutures, which results in skull deformity
Treatment of child with UTI
3rd gen cephalosporin
Age milestone: Babbling
6 months (language development)
Age milestone: Pincer grasp
12 months (motor development)
Age milestone: hops and balance on one foot
Age milestone: jumps with 2 feet
How to treat E Coli O157:H7?
What medications to avoid? Why?
Treat with supportive care.
Avoid: Antibiotics and Antimotility meds (loperamide), because they increase the chance of developing Hemolytic Uremic Syndrome
Complication of giardia infection.
Tx for beta thalassemia major.
Transfusion + Chelation
What are the key unique features of Rubella infection in children and what are the key features of Measles infection in children?
Rubella (German Measles) = Lymphadenopathy, specifically suboccipital, posterior auricular and posterior cervical.
Measles (Rubeola) = 3 Cs = Cough, Coryza, Conjunctivitis. Higher fever (40 C).
Which one is a Torches infection -- Measles OR Rubella? What are the manifestations?
Manifestations = Cataracts, Deafness, Patent Ductus Aretriosus
If pt has UTI. What is the indication for an ultrasound? What is the indication for a voiding cystourethrogram?
Ultrasound = IF fever does not improve with 2 days of antibiotics
Voiding Cystourethrogram = IF 1 of the following is met:
(1) Ultrasound shows abnormality (e.g. hydronephrosis)
(2) Unusual pathogen (Not E. coli)
(3) Chronic kidney injury (Hypertension, Poor growth)
(4) 2 or more febrile UTIs
Abdominal pain, diarrhea, seizures in a child.
What are the values of the pleural fluid analysis that tells you whether the parapneumonic effusion is uncomplicated or complicated? What is the Tx of uncomplicated vs complicated?
Uncomplicated = pH >7.2, Glucose>60, WBC<50k
Complicated = pH<7.2, Glucose<60, WBC>50k
Uncomplicated Tx = Antibiotics
Complicated Tx = Antibiotics + drainage
Criteria to place ear tubes for patients with Acute Otitis Media.
- 3 or more AOM episodes in 4 months
- 4 or more AOM episodes in 12 months
Criteria to give antibiotics to patients with Acute Otitis Media.
- Bilateral AOM
- High Fever
- Severe pain
Compare the skin findings of Neurofibromatosis type 1, Tuberous Sclerosis and Sturge-Weber
Neurofibromatosis type 1 = Neurofibromas, cafe-au-lait spots, axillary freckling
Tuberous Sclerosis = Angiofibromas, Shagreen patches, Ash-leaf spots
Sturge-Weber = port wine stain (along trigeminal nerve distribution)
Methanol or ethylyne glycol poisoning
Beta blocker or Ca2+ channel blocker poisoning
Methylene blue treats...
Methemoglobinemia (exposure to dapsone, nitrates, topical anesthetics)
(use pralidoxime together w/ atropine)
Perianal pruritis at night. Dx?
Pinworm = Enterobius vermicularis
- Strongyloides (intestine and lung)
- Onchocerca (river blindness)
- Trichomoniasis and bacterial vagenosis (Vagina)
- Giardia (GI)
- Entamoeba (GI --> liver abscess)
- Cestodes (tapeworms) like Taenia solium from pork or Diphyllobothrium from fresh fish
- Tematodes (flukes) like Schistosoma (which can lead to bladder cancer) from fresh water or Chlonorchis sinesis (which can lead to gallbladder cancer)
Pyrantel pamoate treats...
Enterobius vermicularis (pinworm) that causes anal pruritis
When is MMR vaccine given?
4 years old
What rheumatological comorbidity is associated with Down's syndrome?
Atlantoaxial instability (laxity between C1 and C2)
Treatment for lead poisoning depending on lead level.
Less than 45 = remove from environment
45 - 70 = Succimer (Dimercaptosuccinic acid
> 70 or sings of encephalopathy = EDTA + Dimercaprol (British anti-Lewisite)
Hamartomas on the iris --> what is the genetic condition?
Lisch nodules = NF1
White-grey spots on periphery of the iris - what is the genetic condition?
Brushfield spots = Down's
Renal angiomyolipoma - what is the genetic condition?
Retinal hemangioblastoma - what is the genetic condition?
What are the 3 conditions that have cafe-au-lait spots? What symptoms help to differentiate between the two?
NF1 (iris hamartoma - Lisch nodules, cutaneous neurofibromas)
McCune-Albright (multiple endocrine abnormalities, precocious puberty)
Fanconi anemia (thumb defect, anemia)
Skin condition associated with Celiac disease
What is the difference between the location,symptoms and treatment of preseptal cellulitis and orbital cellulitis?
Preseptal cellulitis is anterior to the orbital septum, while orbital cellulitis is posterior.
Symptoms that point to orbital cellulitis are extraocularr muscle defect like painful or weak eye movements AND proptosis (bulging eyes).
Treat preseptal with oral antibiotics, treat orbital with IV antibiotics and possible surgery.
What can cause megaloblastic anemia other than B12/Folate deficiency? What other symptoms to look for and mechanism of disease.
Fanconi anemia (DNA repair defect leads to PANcytopenia, not just anemia; Also look for thumb defect, cafe-au-lait spots)
Orotic aciduria (UMP synthase defect, which leads to orotic acid in urine)
Anal fissure in a toddler. Dx? Tx?
Dx = pediatric constipation
Tx = laxative (e.g. polyethylene glycol, mineral oil)
Most common complication for patients with Sickle Cell Trait.
Hyposthenuria (impairment in urine concentrating ability, which can present as nocturia and polyuria)
Glomerular basement membrane thickening. Dx? Symptoms?
Dx = Membranous nephropathy
Symptoms = Edema, proteinuria
Glomerular basement membrane thining. Dx? Symptoms?
Dx = Alport Syndrome
Symptoms = Hearing, Vision problems
Linear deposition of IgG on basement membrane. Dx? Symptoms?
Dx = Goodpasture syndrome
Symptoms = Pulmonary hemorrhage, glomerulonephritis
Localized areas of mesangial sclerosis. Dx? Symptoms?
Dx = Focal Segmental Glomerulosclerosis (FSGS)
Symptoms = Edema, proteinuria
Mesangial deposition of IgA. Dx? Symptoms?
Dx = Henoch-Schonlein Purpura
Symptoms = Abdominal pain (intrususeption), Renal disease, Arthralgias, Lower extremity rash
Podocyte fusion. Dx? Symptoms?
Dx = Minimal Change Disease
Symptoms = Edema, proteinuria
Limited upward gaze. Where is the tumor?
Special organisms associated with Chronic Granulomatous disease.
Tests used to diagnose Chronic Granulomatous Disease
Dihydrhodamine 123 Test
Nitbroblue Tetrazolium Test
(These are tests for neutrophil function)
Medications for Tourette Disorder
Alpha-2 agonists (Guanafacine, Clonidine)
Dopamine depleter (Tetrabenazine)
Antipsychotic (Risperidone, Haloperidol)
Common causes of anion gap metabolic acidosis
Isoniazid / Iron
Ethylene glycol (antifreeze)
Most common cause of pneumonia in healthy patients vs Cystic Fibrosis patients.
Healthy patients = Strep pneumoniae
CF before 20 months of age = Staph aureus
CF after 20 months of age = Pseudomonas
Until what age is bed wetting OK?
5 years. After 5 years old start enuresis alarm therapy.
Until what age is genu varum normal?
Percent Fetal Hemoglobin in Sickle Cell Disease.
SCD pts = 5-15% HgF
SCD pts on Hydroxyurea = > 15%
What therapy should be used in strabismus and at what age?
Until 4 months strabismus is OK. Afterwards need therapy such as eye patch on the good eye
What medications are used as emergency contraceptives to prevent pregnancy by delaying ovulation? Timeline to take.
Levonorgestrel (Plan B) - within 72 hrs of unprotected intercourse
Ulipristal - within 5 days
Most common cause of meningitis depending on age.
Before 6 months = Group B Strep
After 6 months - to old age/death = Strep pneumoniae
Longitudinal splitting of glomerular basement membrane. Dx?
Why does bruising and epistaxis occur in cystic fibrosis patients?
Cystic fibrosis patients have pancreatic insufficiency. This leads to decrease absorption of fat soluble vitamins K A D E. Lack of vit K increases PTT, which increases bruising and epistaxis.
Retinal hemorrhages on fundoscopy. Dx?
Dx = shaken baby syndrome (Subdural venous shearing)
Cystinuria. What type of crystal in nephrolythiasis? What is the diagnostic test?
Crystals = hexagonal
Diagnostic test = Cyanide-nitroprusside
Tourette syndrome often presents with what 2 other comorbid conditions?
2) Obsessive-compulsive disorder
Treatment of UTI in pediatric patient.
Third gen cephalosporin.
(Cefixime, Ceftriaxone, Cefotaxime, Cefpodoxime, Ceftazidime)
What are the two conditions that cause cherry-red macula? What is their mechanism? What symptoms help you to differentiate between the 2 conditions?
Niemann-Pick and Tay-Sachs have cherry-red macula.
Niemann Pick is Sphingomyelinase deficiency.
Tay-Sachs is beta-hexosaminidase A deficiency.
Niemann Pick symptoms = Heptosplenomegaly and Arreflexia
Tay-Sachs symptoms = NO hepatosplenomagaly and Hyperreflexia.
Patient with hip pain after a mild viral illness. CBC is normal. Patient can bear weight on the affected leg. Dx? Prognosis?
Prognosis = Full recovery in up to 4 weeks
Timing of IgA nephropathy vs Poststreptococcal glomerulonephritis.
IgA nephropathy = 1-2 days after URI
Poststrep Glomerulonephritis = 1-4 wks after URI
Another name for "Eczema"
An antibiotic that will cover both pseudomonas and MSSA.
Cefipime (4th gen cephalosporin)
Mechanism of Reye syndrome
Aspirin is a mitochondrial toxin. Causes liver damage. Results in microvesicular steatosis on liver biopsy.
Aspirin is usually contracindicated in children except in the treatment of what?
Rheumatologic disease (e.g. juvenile idiopathic arthritis)
Complications of infectious mononucleosis
Acute airway obstruction
Autoimmune hemolytic anemia and thrombocytopenia
Criteria to diagnose Acute Rheumatic Fever
JONES (major) + (minor)
Joints (mygratory arthritis)
+ High CRP/ESR
+ High PR interval
Turner syndrome: Estrogen, FSH and LH (high or low?)
Low Estrogen, High FSH, High LH
Kallman syndrome: Estrogen, FSH and LH (high or low?)
Normal Estrogen, Low FSH, Low LH (due to Low GnRH)
Androgen Insensitivity syndrome: FSH and LH (high or low?)
Normal to high FSH and LH
5 alphar reductase insufficiency: FSH and LH (high or low)
Normal to high FSH and LH
How to tel Androgen Insensitivity Syndrome from 5 alpha reductase, since they are both 46XY?
Androgen Insensitivity Syndrome will have breast development. 5-alpha reductase deficiency will NOT have breast development. 5-alpha reductase deficiency will have Virilization.
APGAR scoring system
Appearance: - +2 pink; +1 blue extremities; 0 all blue
Pulse: +2 >100; +1 <100; 0 absent
Grimace: +2 crying; +1 to grimace; 0 absent
Activity: +2 active; +1 some flexion; 0 limp
Respiratory: +2 good dry; +1 weak cry; 0 absent
When infant is crying or eating cyanosis appears. Dx?
This is a "tet spell" of Tetralogy of Fallot