Pediatrics Flashcards
(9 cards)
Idiopathic Thrombocytopenic Purpura (ITP)
Most common cause of thrombocytopenia in children
1:20000
Ages 1-4
Immune mediated destruction of platelets
Preceding viral infection in most cases, but rest of cases are unknown causes. Not chronic in kids
Antibody binds to platelets and Fc receptor splenic macrophages will destroy them.
Epstein-Barr Virus and HIV are viruses that can cause it.
Clinical: sudden onset of petechiae and purpura.
- “Was fine yesterday”
- Bleeding from the gums and mucous membranes. Less than 10 platelet count
- Normal physical exam except petechiae.
- Splenomegaly, bone pain, pallor are RARE
Treatment: Education, observation is recommended for those with mild bleeding and bruising.
-Single dose of intravenous immune globulin or short course of corticosteroids should be used first-line.
Hereditary Spherocytosis
1 in 2000-5000
Northern European origin Autosomal Dominant (25% no FH)
Defect in RBC skeletal Protein (ankyrin, spectrin)
Limits ability to move through capillaries and they are removed through spleen causing splenomegaly
Splenectomy greatly improves RBC life may be indicated in some patients. Some may need transfusions
Anemia, Hyperbilirubinemia, splenomegaly, jaundice, recurrent hemolytic crises with possible gallstones
Osmotic fragility test
Susceptible to aplastic rises from parvovirus B19 infection. Hematocrit drops lower than 10% with high-output heart failure
HIV and Hepatitis looked for with transfusions
Diagnosis: Hemolytic anemia with reticulocytosis and indirect hyperbilirubinemia.
Treatment: Visit the hematologists annually
- Growth and spleen sized should be monitored
- Keep Vaccines up to date
- Folic acid supplementation
- Screen for gallbladder disease at 4
Glucose 6-phosphate Dehydrogenase Deficiency
X-linked (more in males)
Malaria guarding
Episodic acute hemolytic anemia and chronic hemolytic anemia nonspherocytic
Manifestations: neonatal jaundice, episodic, acute hemolytic anemia
Caused by: infections, and rarely Fava beans, Sulfa drugs, aspirin, anti-malarial drugs
Risk factors: Central African and Mediterranean descent, Sardinians and Sephardic Jews. Defect occurs in 10% of black males
Oxidative stress causes: decreased hemoglobin, elevated bilirubin, splenomegaly, jaundice. Usually lasts 24-48 hrs
Diagnosis: Bite cells and RBC fragments, transient jaundice, dark urine, hemolytic anemia, acute kidney injury
Treatment: Supportive, transfusion as needed while avoidance of oxidative stressors. Really sever may need transfusions.
Clinical: Degree of hemolysis varies with inciting agent. (Favism). Beans have Divicine, isouramil, and convicine
Prevention: At risk Pop should be tested. Get great history
Congenital Hypoplastic Anemia
“Diamond-Blackfan Anemia”
Rare congenital bone marrow failure
Normochromic macrocytic reticulocytopenia.
Short stature, cardiac or urogenital abnormalities, cleft palate and microcephaly
Autosomal Dominant: 7 per million live births
Substantial phenotypic diversity
Profound Anemia by 2-6 months. 25% anemic at birth. 92% diagnosed by a year.
Only 40-50% have congenital anomalies. Craniofacial are most common.’’
-Other manifestations, skeletal analogies in 30%, thumb abnormalities, radial pulse absent, short stature.
Treatment: limited response to corticosteroids and transfusions. Corticosteroids first in 80%, but try to delay until after 1yr.
-Hematopoetic Stem Cell Transplant can be curative. Best donors are siblings. Give between 3 and 9 years.
Cancer predisposition. Cumulative incidence of solid tumor/leukemia.
Fanconi Anemia
Rare Multisystem hereditary disorder.
1 in 200000, but Jews, and afrikaners more
Predisposition to malignancy
Individuals have congenital malformations, bone marrow failure and elevated chromosome fragility. (Triad)
Symptoms: Short, pancytopenia, hearing loss, microcephaly, absent radii and thumbs, endocrine abnormatlities, hyperpigmentation
Diagnosis: bone marrow biopsy will show hypocellular bone marrow
Treatment: Supportive, but bone marrow transplantation is curative for hematopoetic problems.
Di George Syndrome
22q11.2 Microdeletion Syndrome
Hypoparathyroidism and cellular immune deficiency secondary to thymine hypoplasia
Triad of contour cal cardiac anomalies, hypoplastic thymus, and hypocalcemia.
-Hypoplastic thymus leads to T-cell immunodeficiency with normal B-cells
Signs of congenital heart disease and hypocalcemic tetany cause seizures in first few days. Have smooth philtrum and underdeveloped jaw.
Slight neurological delays. Walking 16-24 months.
Clinical features: cleft palate, conductive hearing loss, microcephaly, slender and hypotonic ands and fingers, ventricular septal defect
Kawasaki Disease
Most common Cause of acquired heart disease in children.
Cause remains unknown, but mostly in Asian descent.
Vasculitis of coronary arteries that can cause infarction.
Medium vasculitis with specific mucocutaneous features affecting younger children. Less than 5 yrs old 80% of time. Has effects on coronary arteries causing aneurysm.
Male to female 1.5:1
Sickness follows sick season: causes: viruses, bacteria, drug reactions, rheumatologic disease, heavy metal toxicity
Diagnosing: Fever for at least 5 day and four of the next five physical findings.
- Polymorphous exanthem
- Conjunctival injection without exudat (discharge)
- Oropharyngeal erythema, lip cracking, strawberry tongue
- Extremity changes, edema, redness, induration of hands and feet
- Cervical lymphadenopathy more than 1.5 cm unilaterally
*** If echo finds coronary a. Disease only need 3 criteria.
Clinical findings: coronary a. Aneurysms, myocarditis, aseptic meningitis, hepatitis, jaundice, urethritis, hydrocele, arthritis, erythema, anterior uveitis, radiographic pneumonitis. (Overall irritability)
Lab results: normocytic anemia, throbocytosis, elevated ESR and CRP, and low serum albumin.
Treatment: IVIG 1-2 doses.
- Aspirin for 1-2 months for anti-platelet effects, give low dose aspirin for 6-8 weeks after fever.
- Treatment needed within first 10 days of fever
- Will begin with criteria after 4 days of fever
- Cardiac follow-up needed. Aneurysms resolve in about 50%
Enoch-Schonlein Purpura
HSP
Unknown cause
Immune-mediated process induced by environmental factors
Aberrant Glucose Latino of IgA1 along with C3 deposition in small vessel walls that triggers inflammation.
Risk Factors: Age under 10
- Peak at 6 years
- 13-20 per 100000 in children under 17
- Male to female 1.5:1
Triggers: Infections: URI, streptococcus, staph, medications, insect bites, food, trauma, cold weather
Rash often first manifestation. Joint involvement may precede rash,
- glomerulonephritis can cause hematuria and proteinuria or HTN
- Low grade fever, and angioedema
- purpura, arthralgia, and abdominal pain classic triad*****
- can have GI manifestations, abdominal pain.
NO KNOW. HLA-DRB1 may be cause. Also HLA-A2, A11, or B35. Decrease risk with HLA-A1, B49, B50
Diagnosis: Off physical findings. Renal involvement. CBC for thrombocytopenia, Urinalysis, urine protein, serum albumin levels
Treatment: suppportive care, oral fluid intake, avoid strenuous exercise. Bed rest not required.
-First line, NSAIDs like Acetaminopehn. ACE inhibitors or angiotensin with proteinuria.
—Naproxen for the arthritis over 2
—Corticosteroids for acute pain phase. Prednisone
Monitoring: Weeks 1-4: weekly urinalysis and BP
5-12: urinalysis and BP every other week
6-12 months: Urinalysis and BP once a month.
If ranges are normal after a 1 year stop monitoring
Recurring disease. Will recur within 4 months in about 1/3 of children. Each manifestation is milder. More than 3 times consult a specialist.
InIntussusception
Complication of HSP
Ingush (within) suscipere (to receive)
One segment of bowel invaginates into another just distal to it leading to obstruction.
Bowel may simply “telescope” on itself. Gives colicky abdominal pain and vomiting. Can cause ischemia.
Happens ileocecal. Can be fixed with saline enema or in the OR
