Pediatric Board Review 2011
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| Hematopoiesis location; First 2-4 weeks of gestation | Yolk Sac
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| Hematopoiesis location; 5-8 weeks of gestation | Liver
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| Hematopoiesis location; 5 months of gestation and beyond | Bone Marrow
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| RBC lifespan in adult and newborn | 120 days;
45-60 days
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| Microcytic Hypochromic anemia; low MCV, low Fe, High TIBC, low Transferrin, low ferritin | Iron deficiency
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| Anemia - normocytic to microcytic; low FE, Low TIBC, Low to normal Tranferrin, normal to high ferritin; Low EPO | Anemia of Chronic disease
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| Anemia; Low MCV, High RDW; Chinese Toys; Mexican Candy; Neuropathy, dementia, abdominal pain, nausea, anorexia | Lead Toxicity
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| Anemia; All Hgb F at birth; Transfusion dependant; | B thalassemia major
(Cooleys anemia)
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| Impaired heme synthesis; Ringed Sideroblast; Pappenheimer bodies; 15-20% develop leukemia | Sideroblastic Anemia
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| Goats Milk; Watery diarrhea, flatulence, failure to thrive | Folate Deficiency
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| Megaloblastic anemia; Strict Vegan or Lack of intrinsic factor; Neurologic: decreased proprioception, paresthesia, spastic ataxia, dementia | B12 deficiency
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| Hypersegmented neutrophils | B12 deficiency
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| Defect in Ankyrin, spectrin, band 3 or protein 4.2; MCHC increased | Spherocytosis
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| Decreased glutathione; Fava beans, sulfonamides, anti-malarials, moth balls; X-linked Autosomal Recessive; | Glucose-6-Phosphate Dehydrogenase Deficiency
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| Reduced glycolysis; Very High Hemolysis; RBC enzyme deficiency; Autosomal recessive | Pyruvate Kinase Deficiencies
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| Acquired stem cell defect; PIG-A defect; CD 59: HRF - homologous restrictive factor; CD 55: DAF - decay acceleration factor; Symptoms: hematuria, thrombocytopenia, venous thrombosis, aplastic anemia at night | Paroxysmal Nocturnal Hemoglobinuria
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| Treatment for PNH | Glucocorticoids;
Monoclonal anti-C5;
Stem-cell Transplant
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| Chromosome 11 - point mutation resulting in substitution of adenine for thymine resulting in valine instead of glutamic acid | Sickle Cell Mechanism
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| Upregulation of surface adhesion molecules results in adherence to endothelium | Pathophysiology of Sickle Cell diease
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| Organism that causes aplastic crisis in sickle cell disease | Parvovirus B19
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| Therapy of choice for acute CVA from sickle cell crisis | exchange transfusion
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| Mutation in ELA2 gene; Cycles at 14 to 28 days; repeated fever, aphthous ulcers, adenopathy, skin infections, pneumonia; Nadir of peripheral neutrophils is <200 | Cyclic Neutropenia
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| Inheritance pattern of cyclic neutropenia | Autosomal Dominant
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| <10% neutrophils - chronically; Risk of MDS/AML <20%; Maturation arrest at promyelocyte stage; Autosomal recessive | Severe Congenital Neutropenia
(Kostmann's Syndrome)
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| SBDS gene; Neutropenia, exocrine pancreatic failure, short stature, metaphyseal dysostoses; Recurrent skin and sinopulmonary infections; SCT is curative; Autosomal Recessive | Shwachman-Diamond Syndrome
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| Short limb dysostsis; Defect in cell-mediated immunity associated with severe VZV infections; Autosomal recessive inheritance, primarily amish | Cartilage Hair Hypoplasia
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| Self limited isoimmuizations with foreign paternal neutrophil antigens; Wears off in 6-12 weeks | Neonatal Isoimmune Neutropenia
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| Diagnosis of exclusion; asymptomatic neutropenia | Benign Neutropenia of Childhood
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| Delayed umbilical Cord seperation 4-6 weeks; Defect CD18 locus on Chromosome 21; Elevated WBC >20K; Autosomal Recessive | Leukocyte Adherence Deficiency
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| Defective superoxide burst; 2/3 X linked; 1/3 autosomal recessive; Recurrent skin, osteomyelitisi, perirectal, pneumonia from catalase producers | Chronic Granulomatous Disease
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| Genetic defects: p22-phox, p47-phox, p67-phox, gp-91phox NBT test | Chronic Granulomatous Disease
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| Oculocutaneuous albinism, neutropenia, prolonged bleeding time, NK cell dysfunction, frequent infections; Large graunules in neutrophils and eosinophils; Autosomal Recessive | Chediak-Higashi Syndrome
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| Elevated IgE >1,000; Recurrent infections; Eczema and allergies with boils | Hyperimmunoglobulin E syndrome
(Job Syndrome)
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| MAHA, neurologic manifestations, renal dysfunction, thrombocytopenia, fever | TTP
Thrombotic Thrombocytopenia Purpura
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| Acquired autoantibody in TTP | ADAMSTS13
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| Treatment for ITP | IVIG, Anti-D, Corticosteroids
Splenectomy
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| Maternal IgG crosses the placenta and attaches to fetal platlets PLA1 | Neonatal Alloimmune Thrombocytopenia
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| Megakaryocytic bone marrow- thrombocytopenia; Absent radii with normal thumbs; Milk protein allergy; Autosomal recessive | Thrombocytopenia absent radii syndrome (TAR)
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| Small platelets; Eczema and immunodeficiency; SCT curative; X-linked defect in WAS protein | Wiskott-Aldrich Syndrome
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| Minor autosomal dominant defect on Chromosome 22; Thrombocytopenia with uniformly large platelets; Neutrophils have large blue Dohle-like bodies | May-Hegglin Anomaly
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| Deficiency of glycoprotein Ib; Giant platelets; Abnormal platelet agglutination; Autosomal Recessive | Bernard-Soulier Syndrome
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| Abnormally formed GPIIb/IIIa; mucocutaneous bleeding; Autosomal recessive | Glanzmann's Thrombocytopenia
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| Treatment for Glanzmanns thrombocytopenia | Platelet transfusion even if normal platelet count
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| Decreased granules in platelets; Oculocutaneous albinism; Puerto Rican; Autosomal recessive | Hermansky-Pudlak Syndrome
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| Immune response to stem cells - idiopathic in majority; drugs, toxins, radiation, CMV, EBV, hepatitis, HIV, parvovirus Anemia | Aplastic Anemia
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| Treatments for Aplastic Anemia | Matched sibling SCT 80% cure
cyclosporin A + anti-thymocyte globulin
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| defective DNA repair; Short stature, abnormal thumbs and radii, microcephaly, cafe-au-lait spots, renal abnormalitiies | Fanconi Anemia
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| Mutation sites in Fanconi Anemia | FANCA and FANCC
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| Mortality from fanconi anemia | head and neck cancers, squamous cell carcinoma, and hepatic malignany
Risk of AML is 15%
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| Hgb 6-8; Age 1-3 yo; insidious onset; normal MCV and Adenosine deaminase; self-limited | Transient Erythroblastopenia of Childhood (TEC)
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| Anemia and reticulocytopenia; Decreased RBC precursors; <1 yo but may be diagnosed later in life; Thumb abnormalities - bifid thumb, short stature, webbed neck, congenital heart disease, mental retardation | Diamond-Blackfan anemia
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| Immune dysregulation; Punched out lesion on plain radiography; Usually >5 yo; Treat with curettage, intralesional steroids, or focal radiation | Langerhans cell histiocytosis
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| Bone disease plus skin, DI, exopthalmos, floating teeth, gingiva, chronic otitis externa | Mutifocal Histiocytosis
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| Fever, HSM, lung, marrow, skin, and FTT; infantile presentation | Systemic Histiocytosis
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| Fever, HSM, CNS symptoms, cytopenias, coagulopathy, hyperlipidemia, hyperferritinemia >1000K; Acquired due to infection; Familial due to perforin gene mutation | Hemophagocytic Lymphohistiocytosis
(HLH)
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| Prolonged PTT, family history, normal PT, excluded vWD X-linked recessive | Factor VIII deficiency
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| Treatment of factor VIII def | Recombinant factor VIII;
DDAVP;
then consider FFP and cryo (discouraged)
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| Inheritance pattern of factor IX def | X-linked;
Same therapy except using factor IX replacement
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| Most common bleeding disorder; Autosomal dominant; Prolonged epistaxis, menorrhagia, mucosal bleeding, bruising, or post-surgical bleeding; prolonged PTT | von Willebrand disease
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| Hageman Factor; autosomal recessive; Normal PT; Markedly prolonged PTT; No clinical bleeding | Factor XII deficiency
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| Clot then start bleedin 12 - 24 hours later; Euglobulin lysis test - expose clot to 1M urea | Factor XIII deficiency
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| Prolonged PT, PTT; Thrombocytopenia; MAHA; Low fibrinogen; D-Dimer increased; Schistocytes | DIC
Disseminated intravascular coagulation
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| Decreased production in factors II, VII, IX, X, protein C and S Prolonged PT more than PTT | Vitamin K Deficiency
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| Most common childhood cancer | Acute lymphoblastic leukemia
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| Syndromes associated with ALL | Trisomy 21, ataxia telangiectasia, chromosomal breakage syndromes - Bloom and Fanconi
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| In ALL: intial WBC <50K; age >1 but less than 10; t(12,21), trisomy 4, 10, 17, t(4,11); pre-B; rapid early responder; minimal residual disease (MRD) | Good prognosis in ALL
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| In ALL: Initial WBC > 50K; Age <1 or >10; T-cell or mature b-cell; t(9,22); Slow response or persistent MRD | Poor prognosis in ALL
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| Induction therapy in ALL | vincristine, l-asparaginase, corticosteroids,(standard) +/- anthracyclines (high-risk)
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| Maintenance therapy in ALL | Methotrexate, 6-mercaptopurine, corticosteroids, vincristine
IT MTX, cytarabine, steroids depending on protocol;
CNS irradiation only for high risk
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| Associated syndromes with AML | Trisomy 21, fanconi, bloom, kostmann, NF-1, PNH, Etoposide( VP-16) exposure, ionizing radiation
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| FAB classification: M3 | Acute promyelocytic leukemia;
t(15,17);
DIC;
ATRA - all trans-retinoic acid;
90% survival
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| FAB classification: M2 | Acute myeloblastic leukemia with granulocyte maturation; t(8,21)
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| FAB classification: M7 | Acute megakayoblastic leukemia
Trisomy 21
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| FAB classification: M 5 | Acute monocytic leukemia;
del 11q2, t(9,11), t(11,19);
CNS disease, gingival hyperplasia
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| Induction therapy for AML | corticosteroids, cytarabine, anthracycline
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| Triphasic: chronic, accelerated, blast crisis; t(9,22) BCR/ABL gene fusion; Massive leukocytosis with rare blasts | Chronic Myelogenous Leukemia
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| Therapy for CML | tyrosine kinase inhibitor
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| Splenomegaly, lymphadenopathy, cutaneous lesions, hemorrhagic symptoms; NF-1, ras gene mutation; monosomy 7 maybe present in 30% | Juvenile Myelomonocytic leukemia
(JMML)
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| Therapy for Hodkins | Vincristine, bleomycin, cyclophosphamide, etoposide + XRT
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| Associated with Non-hodgkin lymphoma | Ataxia-telangiectasia, HIV, imminodeficiencies
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| mature b-cell tumor, often EBV related, often MALT-associated, fastest growing malignancy - doubling time of 18 hours; highest rate of tumor lysis | Burkitt's lymphoma
t(8,14)
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| Most common non-CNS solid tumor in children; neural crest cells; elevated HVA/VMA; raccoon eyes; opsoclonus/myoclonus | Neuroblastoma
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| Loss of function of tumor suppressor genes WT-1 and WT-2 from chromosome 11- | Wilms Tumor
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| Associated with wilm's tumor | WAGR: aniridia, GU abnormalities, MR;
Beckwith-Wiedemann: organomegaly, omphalocele, hemihypertrophy; Denys-Drash: pseudohermaphroditism, nephropathy; Perlman syndrome: macrocephaly, macrosomia, organomegaly, abnormal facies
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| Most common soft tissue tumor; associated with Li-fraumeni (p53) syndrome | Rhabdomyosarcoma
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| Therapy for Rhabdomyosarcoma | vincristine, actinomycin-D, cyclophosphamide
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| Associated with Osteosarcoma | Hereditary retinoblastoma; Li-Fraumeni syndrome, Rothmund-Thompson syndrome: short stature, telangiectasia, small hands/feet, hypoplastic thumbs; paget's disease, ionizing radiation, alkylating agents
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| Bone or soft tissue tumor derived from neural crest cells; lytic onion skinning, diaphysis more commonly affected | Ewing's sarcoma
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| Benign tumors of bone | Osteoblastoma;
Osteochondroma;
chondroblastoma;
Osteoid osteoma;
Enchondroma
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| Most common posterior fossa tumor; low grade; presenationwith ataxia, headache, comiting | Cerebellar astrocytoma
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| Vision changes, increased ICP, delayed puberty, neurobehavioral changes | Craniopharygioma
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| Leukocoria, strabismus, eye pain, headache, heterochromia, spontaneous hyphema, acute closed-angle glaucoma | Retinoblastoma
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| Endoderm, mesoderm, ectoderm | teratoma
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| Most common malignant germ cell tumor; gonadal, sacrococcygeal, or midline, B-hcg absent; alpha-FP reliable | Endodermal sinus tumor (yolk sac tumor)
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| Compnent of mixed germ cell tumor; B-HCG but no alpha-FP; aggressive | Choriocarcinoma
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| Most common liver malignancy in children; elevated alpha fetal protein | Hepatoblastoma
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