UWorld Flashcards
S/sx of tetralogy of fallot
Cyanosis
Harsh systolic ejection murmur left sternal border due to RV outflow obstruction
Squatting increases after load and decreases right-to-left shunting, helping end tet spells
Squatting increases preload and thus murmur due to inc flow across RVOT obstruction
How does blood flow in a VSD? And in TOF?
Normal VSD is a left to right shunt due to lower pressure on right side, and are usually not cyanotic.
VSD in Tet becomes right to left due to RVOT obstruction, and RV hypertrophy.
Squatting in VSD?
Increased SVR increases murmur by directing blood through to right side
How does hypertrophic cardiomyopathy murmur change with squatting? Handgrip?
Squatting and handgrip increase SVR = decreased blood flow through LVOT, and decreased murmur.
Sickle cell anemia - 3 signs, 1 consequence, 2 prophylaxis
Anemia
High reticulocyte count
History of pain crises
Functional hyposplenia - increased susceptibility to encapsulated organisms
Twice daily penicillin prophylaxis until age 5
Vaccination with conjugate capsular polysaccharide
What are nevus simplex lesions? What should be done with them?
Stork bites or angel kisses
Normally resolve by 12 months of age
What should be done with superficial hemangiomas?
May appear in first two weeks of life and grow rapidly for two years. Most are harmless and regress spontaneously during childhood. If they are in a problematic location or ulcerating, they may be treated with beta blockers
What should be done if a child swallows a battery?
X-ray to determine location of the battery. If the battery is in the esophagus, immediate endoscopic removal is indicated. If it has passed beyond the esophagus, 90% will pass uneventfully. Observation to confirm passage by stool examination or radiographic follow up.
How does sickle cell disease differ from sickle cell trait on hemoglobin electrophoresis?
Disease = Hemoglobin S 85-95% Hemoglobin A 0% Hemoglobin F 5-15% Trait = Hemoglobin S 35-45% Hemoglobin A 50-60% Hemoglobin F less than 2%
What is the most common complication of sickle cell trait?
Painless Hematuria, either microscopic or gross
Isothenuria (impairment in concentrating ability) is also common and may cause nocturia or polyuria.
Path and Clinical findings in Henoch-Schonlein purpura
Path: IgA mediated leukocytoclastic vasculitis Palpable purpura Arthritis/Arthralgia Abdominal pain, intussusception Renal disease similar to IgA nephropathy
Lab and kidney findings of Henoch-Schonlein purpura
Normal platelet count and coag studies
Normal to increased creatinine
Hematuria w/wo RBC casts w/wo proteinuria
Kidneys: Mesangial deposition of IgA
Treatment for Henoch-Scholein purpura
Supportive (Hydration and NSAIDS) for most patient
Hospitalization and systemic steroids in patients with severe symptoms
What is the most common type of idiopathic nephrotic syndrome in children, what does it present with, and what are the EM findings?
Minimal change disease
Presents with edema and hematuria
EM shows fusion or flattening of the podocytes
Crigler-Najjar vs Gilberts
Both inherited deficiencies of UDP-glucuronyl transferase
Both result in unconjugated hyperbilirubinemia
Gilberts is mild with period jaundice is times of stress
C-N is total absence of the enzyme and requires liver transplant
Rash in lyme disease
Erythematous patch with central area of pallor
May be expanding slowly
No itch or pain
No associated symptoms
What features do homocystinuria and marfan syndrome share?
Pectus deformity Tall stature (inc arm:height ratio, dec upper:lower segment ratio) Arachnodactyly Joint hyper laxity Skin hyper elasticity Scoliosis
What features does Marfan’s syndrome have that homocystinuria does not?
Marfan: Aortic root dilation, normal intellect, upward lens dislocation, autosomal dominant
What features does homocystinuria have that Marfan’s does not?
Intellectual disability Thrombosis (strokes, etc.) Downward lens dislocation Megaloblastic anemia Fair complexion Autosomal recessive
How is Ehler’s-Danlos syndrome different from homocystinuria?
ED does not have tall stature, lens dislocation, or hyper coagulability.
ED does have scoliosis, hyper extensible joints and hyper elastic skin, like homocystinuria and Marfan’s
Clinical signs of iron poisoning?
30 min to 4 days: Abdominal pain, vomiting (hematemesis, corrosive), diarrhea (melena), anion-gap metabolic acidosis, hypotensive shock
2 days: Hepatic necrosis
2-8 weeks: Pyloric stenosis
Dx and treatment of iron poisoning?
Dx: Anion gap acidosis, Radiopaque pills on x-ray
Tx: Whole bowel irrigation, deferoxamine, Supportive care for A, B, Cs
S/sx of Vitamin A overdose?
Nausea, vomiting, blurry vision. Chronically leads to pseudo tumor cerebri.
S/sx of Vit D overdose?
Related to hypercalcemia including nausea, vomiting, confusion, polyuria, polydipsia.
Why should SS patients take penicillin prophylaxis?
Patients are still susceptible to infection by S. pneumoniae serotypes that are not covered by the vaccine
What is the pathology and clinical findings of Beckwith-Weideman syndrome?
Path: dysregulation of imprinted gene expression in chromosome 11p15
Clinical findings: Fetal macrosomia, rapid growth until late childhood (big kids), Omphalocele or umbilical hernia, Macroglossia, Hemihyperplasia
What are the complications and proper surveillance of Beckwith-Weideman syndrome?
Complications: Wilms tumor, hepatoblastoma
Surveillance: Serum alpha fetoprotein, Abdominal ultrasounds every 3 months until 8 years of age
Complications found in infants who are small for gestational age
hypoxia,
perinatal asphyxia,
meconium aspirate,
hypothermia, decreased fat
hypoglycemia. decreased glycogen stores
hypocalcemia, decreased Ca+ transfer across placenta
polycythemia. Polycythemia develops due to increase erythropoietin production 2/2 hypoxia
Age group, cause and S/sx of croup
Age 6mo to 3 years
Parainfluenza virus
Barky cough, stridor, hoarse voice
Patients, cause and s/sx of epiglotitis
Unvaccinated children
Haemophilus influenza
Sore throat, dysphagia, drooling, and tripod posturing
Age, cause, and s/sx of bronchiolitis?
Age under 2 years
RSV virus
Wheezing, coughing
Description of club foot
Club foot (talipes equinovarus) equinus and varus of the calcaneous and talus, various of the mid foot, and adduction of the fore foot.
Treat with stretching, manipulation, and serial casting. Surgery may eventually be required before 12 months.
Clinical features of pineal gland mass?
Parinaud syndrome: Limited upward gaze, upper eyelid retraction, pupils non-reactive to light
Obstructive hydrocephalus: Papilledema, headache, vomiting, ataxia
Clinical features of medulloblastoma?
arise from the cerebellar vermis
Ataxia, truncal instability, and hydrocephalus
Clinical features of craniopharyingiomas?
Supra cellar masses that compress the optic chasm
Visual field defects
organisms causing meningitis in infants less than 3 months
#1 Group B Strep #2 E coli and gram negatives #3 Listeria monocytogenes #4 Herpes simplex virus
Organisms causing meningitis in children age 3 months to 10 years old?
#1 Strep Pneumoniae #2 Neisseria meningitidis
Organisms causing meningitis in children 11 or older?
neisseria meningitidis
Inheritance, signs of Fredrich’s ataxia and systemic complications?
Autosomal recessive
Gait ataxia
Dysarthria
Nystagmus
Loss of deep plantar reflexes
Complications: Diabetes mellitus, Concentric hypertrophic cardiomyopathy, skeletal deformities
Death is from Cardiomyopathy (90%) or pulmonary disorders
Common etiologies of pediatric stroke
#1 Sickle cell disease Prethrombotic disorders Congenital cardiac disease Bacterial meningitis Vasculitis Focal cerebral arteriopathy Head/neck trauma
Respiratory features of cystic fibrosis
Obstructive lung disease - bronchiectasis
Recurrent pneumonia
chronic rhinosinusitis
GI features of cystic fibrosis
Obstruction - meconium ileus, distal intestinal obstruction syndrome
Pancreatic disease - Exocrine pancreas insufficiency, CF-related diabetes
Biliary cirrhosis
Reproductive features of cystic fibrosis
Infertility 95%+ in men, about 20% in women
MSK features of cystic fibrosis
Osteopenia and fractures
Kyphoscoliosis
Digital clubbing
What is oral succimer used for?
Chelating lead in mild to moderate poisonings
What is calcium EDTA used for?
Chelation in moderate to severe lead poisonings
Children with irritability, poor appetite, headaches, abdominal pain, and anemia
Sodium bicarb is used in which overdoses or poisonings?
Tricyclic antidepressants
Aspirin
Rotavirus vaccine: Live or dead? Contraindications?
Live vaccine
Contraindications:
- Anaphylaxis to vaccine ingredients
- History of intussusception (may cause intus.)
- History of uncorrected congenital malformation of the GI tract (i.e. Meckel’s)
- SCID
Imaging after a positive Ortolani or Barlow?
Age dependent:
2 weeks to 6 months: Hip ultrasound
Greater than 4-6 months: Hip X-ray
Physical features of fetal alcohol syndrome?
Smooth (or absent) philtrum
Thin vermillion border (upper lip)
Small palpebral fissures
Microcephaly
Physical features of down syndrome?
Face: Flat facial profile Slanted palpebral fissures Small, low set ears Body: Excessive skin at nape of neck Single transverse palmar crease Clindodactyly (bent fingers) Large space between first two toes
Physical features of fragile X syndrome?
Face: Long narrow face Prominent forehead and chin Large ears Macrocephaly Body: Macroorchidism
How does congenital rubella present?
Developmental delay Sensorineural deafness Cataracts Hepatosplenomegally Purpura
Kallman syndrome: Path, presentation, and treatment?
Path: failure of GnRH and olfactory cells to migrate, normal genetics, hypogonadotropic hypogonadism
Pt: Anosmia and absent puberty, low LH and FSH
Treatment: Hormone supplementation
Rene syndrome: Etiology, clinical features, lab findings, pathology and treatment?
Etiology: Pediatric aspirin use during influenza or varicella infection
Features: Acute liver failure, encephalopathy
Lab: Elevated AST, ALT, PT, INR, PTT, High NH3
Pathology: Micro vesicular steatosis (fatty accumulation) on liver biopsy
Treatment: Supportive
What is the differential for flaccid paralysis?
Infant botulism
Food borne botulism
Guillain-Barre syndrome
What is the pathogenesis, presentation, and Treatment of infant botulism?
Path: Ingestion of clostridium botulinum spores from environmental dust
Presentation: Descending flaccid paralysis
Treatment: Human-derived botulism immune globulin
What is the path, presentation, and treatment of food borne botulism?
Path: ingestion of preformed C botulinum toxin.
Presentation: Descending flaccid paralysis
Treatment: Equine-derived botulism antitoxin
What is the path, presentation, and treatment of Guillain-Barre?
Path: Autoimmune peripheral nerve demyelination
Presentation: Ascending flaccid paralysis
Treatment: Pooled human immune globulin
Serum sickness-like reaction: Etiology, clinical features, labs, treatment?
Etiology: Antibiotics (B-lactam, sulfa) are most common
Clinical features: Fever, urticaria, polyarthralgia 1-2 weeks after first exposure. Headache, edema, lymphadenopathy, and splenomegaly less common
Labs: Elevated CRP, ESR, hypocomplementemia (Type III hypersensitivity reaction)
Treatment: Remove/avoid offending agent, Steroids for severe cases.
When is it ok to pursue a court order against parents wishes for their child’s treatment?
Parents are not allowed to refuse life-saving treatment for their child. A court order may be obtained to permit necessary treatment.
What features distinguish acute bacterial rhino sinusitis from viral?
Persistent symptoms more than 10 days without improvement
Severe symptoms like fever above 39/102, purulent nasal discharge, or facial pain for 3+ days
Worsening symptoms 5+ days after viral URI began improving (double dip)
Contraindications and precautions for diphtheria/tetanus vaccine (not full TDaP)?
Contraindications: Anaphylaxis to vaccine
Precautions: Moderate or severe acute illness +/- fever
Guillain-Barre within 6 wks of tetanus toxoid-containing vaccine
Arthus-type hypersensitivity reaction following previous dose of D/T vaccine
Contraindications and precautions for acellular Pertussis vaccine?
Contraindications: Anaphylaxis to vaccine
Progressive neurological disorder (uncontrolled epilepsy, infantile spasms)
Encephalopathy within a wk of previous dose
Precautions:
Moderate or severe acute illness +/- fever
Reactions to previous doses:
- Seizure within 3 days,
- Temp above 40.5/105 within 2 days,
- Hypotonic/hyporesponsive episode within 2 days,
- Inconsolable persistent crying within 2 days
What is the differential for regurgitation and vomiting in infants?
Gastroesophageal reflux
Milk protein allergy
Pyloric Stenosis
What are the two types of gastroesophageal refluxes in infants, what are their clinical features, and what is the management of each?
Physiologic:
Features: asymptomatic, “Happy spitter”
Management: Reassurance, Positioning therapy
Pathologic (GERD):
Features: Failure to thrive, significant irritability, Sandifer syndrome
Management: Thickened feeds, Antacid therapy, If severe - esophageal pH probe monitoring and upper endoscopy
Milk protein allergy: Clinical features and management?
Features: Regurgitation, vomiting, eczema, bloody stools
Management: Elimination of dairy and soy protein from diet
