UWorld II Flashcards
Clinical features and lab findings of Vit K deficiency?
Clinical features: Easy bruising, mucosal bleeding, GI bleeding (Superficial bleeds characteristic of clotting factor deficiencies)
Lab findings:
Inc PT/INR - Predominant deficiency is Factor VII
Nl aPTT - Decreased in severe Vit K def only
Presentation of scarlet fever - prodrome, rash, species, and tx?
Prodrome - Fever, chills, toxicity, abdominal pain, pharyngitis +/- exudates
Rash - After 1-7 days, starts on neck, groin, and axilla, then generalizes, “Sandpaper-like”
Species - Group A Strep
Treatment - Penicillin V or Erythromycin, Clindamycin, or 1st gen Ceph if allergy to Pen
Presentation of Kawasaki disease?
Lymphadenopathy
Buccal mucosa changes - Pharyngitis, strawberry tongue
Rash
Bilateral conjunctival injection or changes in peripheral extremities
Very similar to scarlet fever, but does not respond to Penicillin therapy
Scalded skin syndrome - species and presentation?
S. aureus
Superficial flaccid bullae
Extensive exfoliation of the skin
Herpangina - species, presentation?
Enterovirus - esp Coxsackie A
High fever
Severe sore throat (poss difficulty swallowing)
Ulcerative lesions on palate, tonsils, pharynx
May require IV fluids
Stevens-Johnson syndrome
Variant of erythema multiforme
Skin rash with target lesions (erythema multiforme)
Inflammatory bullae of two or more mucous membranes
Manifestations of refeeding syndrome
Arrhythmias
Congestive heart failure (pulmonary and peripheral edema)
Seizures
Wernicke encephalopathy
Hormone responsible for refeeding syndrome?
Insulin
New feeds cause increased insulin, glycogen and protein synthesis, cellular uptake of phosphorus, potassium, magnesium, and thiamine and serum decreases in the same.
Also, sodium and water retention.
Sturge-Weber syndrome - presentation, associated symptoms, and tx?
Focal or generalized seizures
Mental retardation
Port-wine stain
Intracranial calcifications along trigeminal nerve
May also show hemianopia, hemiparesis, hemisensory disturbance, ipsilateral glaucoma.
Tx - control seizures and reduce intracranial pressure, laser therapy for skin lesion
Red or pink stains in neonate diaper?
Uric acid crystals
Normal and do not require workup in properly progressing children. Uric acid secretion is high at birth and gradually decreases until adolescence when adult levels are found.
Triggers and clinical features of generalized seizures?
Trigger: Fever, hypoglycemia, sleep deprivation
Clinical features: +/- preceding aura
Los of consciousness and tone, convulsions
Postictal state
Triggers of vasovagal syncope vs cardiogenic syncope
Vasovagal: Prolonged standing, physical/emotional stress
Will have prodrome (lightheadedness, diaphoresis, pallor)
Cardiogenic: Exertion, Dehydration
Treatment for histiocytosis?
Conservative treatment,
May cause lytic bone lesions that are locally destructive, but which usually resolve spontaneously.
Often associated with hypercalcemia
Common etiologies for avascular necrosis
Steroid use Alcohol abuse SLE Antiphospholipid syndrome Hemoglobinopathies (Sickle Cell) Infections (Osteomyelitis, HIV) Renal transplantation Decompression sickness
Clinical manifestations of avascular necrosis
Groin pain on weight bearing
Pain on hip abduction and internal rotation
No erythema, swelling or point tenderness
Lab findings and imaging in avascular necrosis
Lab: normal white blood cell count
Normal ESR and CRP
Imaging: MRI most sensitive modality
Crescent sign in advanced stages
Complications of meningococcemia
Meningococcemia is associated with meningitis
May lead to Waterhouse-Friderichsen syndrome which is adrenal hemorrhage leading to adrenal failure. Presents as vasomotor collapse with large petechiae and purpuric lesions
Symptoms and most common cause of infectious mononucleosis?
Long lasting illness (weeks) Fever Lymphadenopathy Fatigue Pharyngitis Splenomegaly
Epstein-Barr virus is most common cause
Confirmatory test, complications, and treatment for infectious mononucleosis?
Test: Heterophile antibody (monospot) test
Complication: Acute airway obstruction
Treatment: Generall resolves without sequelae. IV corticosteroid may be used
Inheritance and S/Sx of Freidrich’s Ataxia
Autosomal recessive
Unsteady, wide-spaced gait, shifting to maintain balance
Weakness in lower extremities
Decreased vibratory and position sense in lower limbs and diminished to absent reflexes.
Lifespan and associated complications of Friedrich’s ataxia?
Wheelchair bound by 25
Death by 30-35
Necrosis and degeneration of cardiac muscle fibers, myocarditis, myocardial fibrosis, cardiomyopathy. Cardiac arrhythmia and congestive heart failure lead to a significant number of deaths
Measles virus: Transmission, presentation, and treatment?
Airborne transmission
Cough, coryza (rinorrhea), conjunctivitis, fever, koplic spots) before rash that starts on head and moves downward
Supportive treatment and Vit A if hospitalized
Differentiating central and peripheral precocious puberty
Central: Early activation of the hypothalamic-pituitary-ovarian axis, GnRGH activation leads to High FSH and LH
Peripheral: Caused by gonadal or adrenal release of sex hormones, No activation of HPO axis leads to low FSH and LH levels
S/sx and diagnostic steps for hydrocephalus
Sx: Poor feeding
Irritability
Decreased activity
Voting
Signs: Tense and bulging fontanelle
Prominent scalp veins
Widely spaced cranial sutures
Rapidly increasing head circumference
Dx: CT scan for acutely symptomatic infant
Sedated MRI is an option if infant is stable and asymptomatic
Four characteristics of absence seizures
Occur during all activities
Last less than 20 seconds
Lack of response to vocal or tactile stimulation
Presence of automatisms (blinking eyes, smacking lips etc.)
Ddx for prepubertal vaginal bleeding
Withdrawal of estrogen
- Presents in neonatal period
- Lasts less than 1 week
- Exam is otherwise normal
Trauma
- Usually unintentional from fall
- Can be a sign of sexual abuse
- Genital exam may show laceration/abrasion
Malignancy (rhabdomyosarcoma)
- Rare
- Ages under 3
May visualize protruding vaginal nodules
What are the most common causative organisms for osteomyelitis in children aged less than 2 months? 2mo - 4 years? Over 4 years? With sickle cell?
Under 2 mo: Group B Strep, E coli
2m - 4y: Kingella kingae
Over 4yr: Staph aureus
Sickle Cell: Salmonella, Staph aureus
Risk factors for and complications from constipation in young children?
Risk factors: Initiation of solid food and cow milk, toilet training, school entry
Complications: Anal fissures, hemorrhoids, encopresis, enuresis or UTI, vomiting
Presentation of Kawasaki syndrome?
Fever for 5+ consecutive days
Conjunctivitis: bilateral
Oral mucosa change: erythema, fissured lips, “strawberry tongue”
Rash
Extremity change: erythema, edema, desquamation of hands and feet (late sign)
+/- Cervical lymphadenopathy: usually unilateral, but infrequent finding
Labs of Kawasaki disease?
Elevated CRP and ESR
Leukocytosis with neutrophilia (as opposed to lymphocytosis in viral infections)
Reactive thrombocytosis
Sterile pyuria on urinalysis
How is Kawasakis different from hand foot and mouth?
HFM is self-limiting and usually resolves in 2-3 days
Treatment and complications of Kawasaki dz?
Tx: aspirin and IVIG
Complications: Coronary artery aneurysms (Aspirin prevents this)
Myocardial infarction and ischemia (Yes in KIDS!!)
Two conditions that increase the risk of intussusception
Henoch-Schonlein Purpura
Mickey’s diverticulum
Small degree of breast development in a teenage boy
Increased estrogen production or peripheral conversion: Testosterone can be converted to estrogen
Also, hormone secreting tumors, cirrhosis or malnutrition
Thyrotoxicosis, excessive aromatase activity, androgen use, or drugs (spironolactone, cimetidine)
Also androgen deficiency: 1 or 2 male hypogonadism (klinefelters, testicular trauma), hyperprolactinemia (via gonadal suppression), renal failure
Three muscular dystrophies and their causes?
Becker, Duchenne, Myotonic
Backer and Duchenne: X-linked recessive deletion of dystrophin gene on Xp21
Myotonic: Most common MD disease (1:8000) Thus autosomal dominant, expansion of CTG trinucleotide repeats in DMPK gene on 19q13.3
Comorbidities and prognosis of Beckers and Duchenne MD?
Becker: Cardiomyopathy, Death by age 40-50 from heart failure
Duchenne: Scoliosis, cardiomyopathy, Wheelchair dependent by adolescence, death by age 20-30 from respiratory or heart failure
Clinical presentation, comorbidities, and prognosis of myotonic muscular dystrophy?
Clinical presentation: Age of onset 12-30, Facial weakness, hand grip myotonia (delayed relaxation of muscles), dysphagia
Comorbidities: Arrhythmias, cataracts, balding, Testicular atrophy/infertility
Prognosis: Death from respiratory or heart failure depending on age of onset
What are the common features of herpangina and herpetic gingivostomatosis? What distinguishes them?
Common: Fever, pharyngitis, vesicles on oropharynx.
Distinguishing feature: Herpangitis (Coxsackie A) has vesicles and ulcers on the posterior oropharynx while herpetic gingivostomatosis (HSV) has vesicles only on the anterior oropharynx (herpes is close to the lips)
What are the clinical features of chronic granulomatous disease and how is it diagnosed?
Recurrent pulmonary and cutaneous infections
Catalase positive infections (S. aureus, Serratia, Burkholderia, Aspergillus)
Dx is neutrophil function testing:
Dihydrorhodamine 123 test
Nitroblue tetrazolium test
Dysfunction and treatment for chronic granulomatous disease?
NADPH oxidization complex gene dysfunction
Majority of cases are X-linked recessive
Tx is antibiotic prophylaxis with trimethoprim-sulfamethoxazole and itraconazole
Interferon gamma in severe cases.
Presentation, dx, and tx of Trachoma
Presentation: follicular conjunctivitis (unilateral), panes formation (neovascularization) of the cornea leading to blindness
Dx: Giemsa staining
Tx: topical tetracycline or oral azythromycin
Differential for secondary enuresis?
Secondary enuresis is the return of bed or underwear wetting after being toilet trained.
Psychological stress - behavior regression, mood lability
UTI - Dysuria, hesitancy, urgency, abdominal pain
DM - Polyuria, polydipsia, polyphagia, weight loss, lethargy, and candidiasis
DI - Polyuria, polydipsia with out other s/sx
Obstructive sleep apnea - Snoring, dry mouth, fatigue, irritability, hyperactivity
Prevention and treatment of measles?
Rubeola virus
Prev: Live attenuated vaccine
Tx: supportive, Vitamin A for hospitalized patients
Inheritance pattern and clinical features of hemophilia A and B?
X-linked recessive
Delayed or prolonged bleeding after minor procedures or trauma
- Hemarthrosis, hemophilic arthropathy (Deposition of hemosiderin and fibrosis in joints)
- Intramuscular hematomas
- GI or GU tract bleeding
Lab findings and treatment of hemophilia A and B?
Labs: Prolonged aPTT
Normal platelet count, bleeding time, prothrombin time
Decreased or absent Factor VIII (Hem A) or Factor IX (Hem B)
Treatment: administration of Factor VIII or IX
Desmopressin for mild Hemophilia A
