Peds 11 Flashcards
What are names of inhaled glucocorticoids used for asthma
Budesonide, Fluticasone, Beclomethasone
What are names of systemic glucocorticoids used for asthms
Oral prednisone/prednisolone or dexamethasone
IV methylprednisolone
Describe clinical presentation of HUS
♣ Findings (triad):
• Microangiopathic hemolytic anemia
• Renal insufficiency (thrombi involve vessels of the kidney)
• Thrombocytopenia
Often preceded by diarrhea (caused by E. Coli or Shigella)
Describe pathogenesis of thrombotic thrombocytopenic purpura (TTP)
♣ Platelets used up in pathologic formation of microthrombi in small vessels
♣ Due to decreased ADAMTS13, enzyme that normally cleaves vWF for degradation
• No vWF degradation = abnormal platelet adhesion = microthrombi
Describe clinical presentation of TTP
♣ Findings (Pentad):
• Thrombocytopenia = platelets being used up
• Microangiopathic hemolytic anemia = RBCs sheared by microthrombi
• Renal insufficiency (thrombi involve vessels of the kidney)
• Neurological symptoms (confusion, HA, seizures, coma) – thrombi involve vessels of CNS
• Fever
Describe pathogenesis of immune thrombocytopenic purpura (ITP)
♣ IgG autoantibodies to GP2b3a
♣ Antibodies produced by plasma cells of spleen and antibody-bound platelets consumed by macrophages of spleen
Clinical presentation of ITP
- Decreased platelet count
- Increased megakaryocytes on bone marrow biopsy
♣ Often triggered by preceding viral illness
o WBC and hemoglobin levels will be normal (unless excessive bleeding has occurred) because bone marrow infiltration does not occur
Tx of ITP
- Usually a self-limited disease
- Steroids and IVIG (autoimmune treatment)
- Platelet transfusion for life-threatening bleeding
- Splenectomy for serious complications without response to other therapies
What platelet count is considered thrombocytopenia
< 150,000 (nl = 150-400)
What is a normal WBC
4,500-11,000
Describe presentation of Henoch Schonlein purpura
o Most common vasculitis in children o Follows URI infections o Findings: ♣ Palpable purpura on butt and legs ♣ Arthralgias ♣ Abdominal pain ♣ Renal disease – IgA nephropathy (IgA immune complexes deposited in mesangium) aka Berger disease
Treatment of Henoch Scholnein purpura
♣ Disease is self-limited
♣ Steroids if severe
Necessary testing/tx of Kawasaki
♣ Echocardiography to identify coronary artery abnormalities, pericarditis, CHF, and valvular regurgitation
♣ Aspirin (to prevent thrombosis of coronary arteries)
♣ IVIG
Diagnostic criteria for Kawasaki
♣ Fever lasting > 5 days with at least 4 out of five of the following:
• Bilateral bulbar conjunctival injection
• Oral mucous membrane changes, including injected or fissured lips, injected pharynx, or strawberry tongue
• Peripheral extremity changes, including erythema or palms or soles, edema of hands or feet, and periungual desquamation
• Polymorphous rash
• Cervical lymphadenopathy
Describe presentation of Scarlet fever
o 3 main symptoms
♣ 1) redness and swelling of tongue (strawberry tongue)
♣ 2) pharyngitis
♣ 3) widespread rash that spares the face
• Rash has a “sandpaper” texture with accentuation in flexural creases known as Pastia lines
Diseases caused by H. Flu
- pneumonia
- epiglottitis
- otitis media
- meningitis
- sepsis
- septic arthritis
Two general names of diseases caused by Legionella
Pontiac fever and Legionnaire’s disease
Describe presentation of Pontiac disease
fever and malaise - usually self-limiting
Describe presentation of Legionnaire’s disease
Atypical pneumonia Unique characteristics: - hyponatremia - neuro sx (HA and confusion) - diarrhea - high fever
Tx of Legionairre disease
Macrolides, Fluoroquinolones
Describe clinical findings of Henoch Schonlein purpura
♣ Palpable purpura on butt and legs ♣ Arthralgias ♣ Abdominal pain • Intussusception is most common GI complication – intestinal edema and bleeding can act as a lead point Renal disease – IgA nephropathy
Tx of Henoch Schonlein
♣ Disease is self-limited
♣ Steroids if severe
Presentation and tx of Reye syndrome
o Clinical features
♣ Acute liver failure
♣ Enceophalopathy
o Treatment
♣ Supportive
Describe difference in CSF between bacterial and viral meningitis
o Bacterial meningitis
♣ WBC: > 1000
♣ Glucose: <40
♣ Protein: >250
o Viral meningitis
♣ WBC: 100-1000
♣ Glucose: 40-70
♣ Protein: >250
Describe CSF of TB
o Tuberculosis meningitis
♣ WBC: 5-1000
♣ Glucose: <10
♣ Protein: >250
Describe CSF of Guillan Barre
o Guillain-Barre
♣ WBC: 0-5
♣ Glucose: 40-70
Protein: 45-1000
Diagnose: 15 y/o male presenting with ataxia, dyarthria, and scoliosis
Friedreich ataxia
What is the most common cause of death in Friedriech ataxia
Hypertrophic cardiomyopathy
Diagnosis: progressive hip pain in a sickle cell patient
Avascular necrosis due to red blood sickling
What type of liver damage is seen in Reye syndorme
Microvascular steatosis
What bug causes perianal itching with positive tape test
Enterobius vermicularis (aka pinworm)
Tx of pinworm
Albendazole or Pyrantel pamote
Describe the etiology of SCID
o Defect in early stem cell differentiation
♣ Gene defect leading to failure of T cell development
♣ B cell dysfunction due to absent T cells
Presentation of SCID
♣ Failure to thrive
♣ Chronic diarrhea
♣ Recurrent infections
♣ Absence of thymic shadow
Tx of SCID
Stem cell transplant
What are the causes of microcytic anemia
♣ Iron deficiency ♣ Anemia of chronic disease (late) ♣ Thalassemia ♣ Lead poisoning ♣ Sideroblastic anemia
Describe clinical features of necrotizing enterocolitis
♣ Systemic: Vital sign instability, lethargy
♣ GI: Vomiting, bloody stools, abd distension/tenderness
X-ray findings of nec enterocolitis
♣ Pneumatosis intestinalis
♣ Portal venous gas
♣ Pneumoperitoneum
Tx of nec enterocolitis
• Bowel rest
• Paraenteral hydration/nutrition
♣ Broad spectrum IV abx
♣ +/- surgery
Describe cause and presentation of laryngomalacia
o Pathophysiology
♣ Caused by “floppy” supraglottic structures that collapse during inspiration
o Clinical presentation
♣ Inspiratory stridor worsens when supine
♣ Peaks at age 4-8 months
Diagnosis and management of laryngomalacia
o Diagnosis
♣ Usually clinical
♣ Confirmation by flexible laryngoscopy for moderate/severe cases
o Management
♣ Reassurance for most cases
Supraglottoplasty for severe symptoms.
Preferred imaging modality of midgut volvulus
Upper GI series
Diagnose: long limbs, gynecomastia, low IQ
Klinefelter
Tx of DKA
- IV fluids + Potassium
- Insulin
- Add dextrose to IV fluids once serum glucose reaches 250-300
What will you see on bone age in GH deficiency vs familial short stature
GH def: Bone ages are delayed, indicating catch-up growth potential
Familial short stature: Bone age equals chronological age, indicating no “extra” growth potential
Tests used to diagnose GH deficiency
- GH level is of little value because secretion is pulsatile and always chagneing
- Screening tests include: IGF-1 (insulin-like growth factor)
- Confirmation often requires GH stimulation testing
Describe the cause of Central precocious puberty
o Gonadotropin dependent
o Hypothalamic-pituitary-gonadal activation leading to secondary sex characteristics
Describe the cause of Noncentral precocious puberty
o Gonadotropin independent
o No hypothalamic-pituitary-gonadal activation
o Hormones usually are either exogenous (birth control pills, estrogen, testosterone cream) or from adrenal/ovarian tumors
What is premature thelarche
♣ Early breast development (typically in girls ages 1-4 years)
♣ No pubic/axillary hair development or linear growth acceleration
What is premature adrenarche
Early activation of adrenal androgens (typically in girls ages 6-8 years), with gradually increasing pubic/axillary hair development and body odor
Causes of premature thelarchy
♣ Causes include ovarian cysts and transient gonadotropin secretion
Tx of premature thelarchy
No tx necessary
