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107 Cards in this Set
- Front
- Back
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ADHD
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syndrome of:
-hyperactivity -impulsitivity -inattention sx prior to age 7 |
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ADHD is more common in boys
T or F |
false
2-3 : 1 Girls: Boy (more common in girls and more likely inattentive, less likely to be identified) |
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Conduct disorder
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-repeated violation of important societal rules or the rights of others
-childhood onset prior to age 10 yrs -adolescent onset 13 yrs e.g stealing, animal cruelty, setting things on fire, bullying |
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Conduct disorder Criteria
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within the last 12 months
-aggression to people and animals -destruction of property -deceitfulness or theft -serious violation of rules (e.g. bringing a knife to school) |
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Oppositional defiant disorder
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-children who are argumentative, disobedient, defiant but do not seriously violate the rights of others.
-often take a self-defeating stand in arguments -may occur with ADHD |
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ODD may develop into CD.
what are risk factors? |
-poverty
-substance abuse -young birth mother -inconsistent discipline -child with low IQ, fights, resist discipline -no home structure |
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Mental Retardation
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-IQ <70
-deficits in adaptive funcitoning- ADLs -onset prior to 18 yrs -30-70% have a psych d.o. -co morbid ADHD, depression, and anxiety -Autism and PDDs 100x more likely -common symptoms-- impulsivity, irritability, hyperactivity, short attention span, and speech delay-- expressive and receptive |
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what is the most common inherited cause of mental retardation?
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Fragile X
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Autistic disorder: severe impairment prior to age 3 years in areas of:
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social interaction
communication and play interests and activities |
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examples of an impairment of social interactions in autism
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-impaired nonverbal behaviors, such as eye to eye gaze
-failure to develop peer relationships -lack of spontaneous sharing -lack of social reciprocity |
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Examples of communication impairments in autism:
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-delay in speech
-impairment in ability to initiate or maintain a conversation -stereotyped, repetitive, idiosyncratic use of language -lack of varied, spontaneous, or make believe play. parallel play |
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Examples of restricted repetitive and stereotyped behavior, interests, and activities in autism:
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-intense restricted interests
-inflexible nonfunctional routines -respective motor movements (hand flapping or twisting, complex whole body movements) -persistent occupation with parts of objects |
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Aspergers Disorder
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characterized by deficits in social interactions: repetitive, restricted and stereotyped behavior and interest, but no mental retardation or impaired speech
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Retts disorder
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-predominantly in females
-microcephaly -marked mental retardation -handwashing stereotypes -loss of purposeful motor skills -normal development until 6 mon then progressive encephalopathy with microcephaly -communication skills lost |
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most common primary immune deficiency
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selective IgA deficiency
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immune protection against common bacteria and viruses from mom starts about ____ wk gestation
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28-32 weeks
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Immunity in infants
-transfer of maternal antibodies (IgG) across the placenta -wanes with time, nadir at ____months |
5-7 months
Then infant must develop their own immunity (importance of vaccinations) |
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when does T cells being to function as efficiently as adults?
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1 yr
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risk factors of frequent infections
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day care, second-hand smoke, air pollution, allergic conditions, anatomic abnormalities
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for every child with a fever and/ or URI symptoms, ask about _____
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rashes
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s/s of potential severe bacterial infection
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-fever in newborn <2 months
-vascular instability (hypothermia, mottling, poor cap refill, tachycardia out of proportion to fever, low or unstable bp) -skin rashes (petechiae, ecchymosis) |
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Physiologic Anemia of Infancy
-erythropoietin levels drop to nadir at ___mo -RBC production drops off to nadir at ___ mo of age |
1-2 months
2 months |
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Normal CBC values
WBC Hgb Hct Plt ANC MCV MCHC |
WBC 4.5-13.5
Hgb 13-16 Hct 36-50 Plt 150-450 ANC 1800-3750 MCV 70-85 MCHC <35 |
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examples of Normocytic anemia
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-aplastic anemia
-anemia secondary to bone marrow inflitration -secondary anemia (hypothyroidism, renal disease, chronic illness) -transient erythroblastopenia of childhood (TEC) |
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examples of microcytic anemia
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-iron deficiency anemia
-thalassemias -sideroblastic anemia (congenital- mitochondiral disorders; Acquired- lead or INH toxicity) -anemia of chronic disease -hemoglobin E |
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examples of macrocytic anemia
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-folic acid or B12 deficiency
-primary bone marrow disorders (myeloproliferative disorders, aplastic anemia, diamond-blackfan anemia, pearson syndrome) |
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Mentzer index
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MCV/RBC
<13 more likely thalassemia (normal RBC, low MCV) >14 more likely iron deficiency (low RBC, low MCV) |
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Anemia with high Retic
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bone marrow response to hemolysis or acute blood loss (hemorrhage)
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Anemia with low Retic
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suboptimal bone marrow response
marrow aplasia, infiltration with malignant cells, depression from infection, nutritional deficiency |
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"hemoglobin barts" on newborn screen
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Alpha-thallasemia
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denatured hemoglobin seen as heinz bodies on smear
"blister cells" |
G-6-PD
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Meds to avoid if G6PD
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analgesics, ASA, nitrofurantoin, acetaminophen, methylene blue, acetophentidin, vitamin k, acetanilid, nalidixic acid, antimalaria, probenecid, primaquine, ascorbic acid, chloroquine, nitrates, petaquine, quinidine, sulfonamides, naphthlane, sulfones, chloramphenicol, quinine, quinidine
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treatment of G6PD
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-avoid agents that cause hemolysis (medications, fava beans)
-blood transfusion (hgb<7 or 9) -CHSNA transfuse to maintain hgb 8-10 -splenectomy |
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child presents with bruising and nosebleeds. CBC, PT/PTT all normal except there is NO PLATELETS.
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Idiopathic Thrombocytopenia Purpura
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ITP
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-more female than males
-precedes viral illness (60-70%) -hemorrhagic manifestations (purpura, petechiae, epistaxis, hematuria, intracranial hemorrhage) -Abrupt presentation -Antiplatelet antibodies (IgG against platelet membrane antigens-- usually GPIIb/IIIa |
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Management of ITP
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Observation
IVIG Oral prednisone IV Anti-Rho (WinRHo) *Treat peds d/t risk of intracranial hemorrhage |
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Management of Life threatening hemorrhage
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Platelet transfusion
IV methylprednisolone IVIG +/- emergency splenectomy |
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most common inherited bleeding disorder
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Von Willebrand Disease
deficiency of Von Willebrand Factor |
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Diagnosing vWD
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-Bleeding time or closure time (PFA-100)
-Von Willebrand Panel (vWF antigen assay, vWF activity, vWF multimers, Factor VIII activity) PT, PTT, CBC, platelet count are often normal |
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Treatment of vWD
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-DDAVP (desmopressin)
-Humate-P (plasma derived vWF/Facotr VIII products) adjuncts -antifibrinolytics -estrogen -topical agents -nasal saline |
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Sickle Cell Disease--clinical features
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-anemia-- normochromic, normocytic
-sickle dex positive -reticulocytosis -neutrophilia -thrombocytosis -HbS migrates slower than HbA on electrophoresis |
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SCD crises
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-swelling of hands and feet in young children
-bone crises (3-4 yo) -abdominal crises -vaso-occlusion of messenteric blood supply -infarction in liver, spleen, lymph nodes -differentiate from acute abdomen -priprism, painful lasting erection, impotence in many cases |
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SCD crises treatment
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-fluids
-ibuprophen around the clock -toradol if in ED or admitted -pain meds (codeine, percocet, morphine, pca) -simple transfusion as required -r/o concurrent infections (osteomyelitis, acute abdomen) |
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Sickle Cell disease-- Autosplectomy
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--Functional hypposplenism (precedes autosplenectomy)
--patients are at risk of overwhelming sepsis -require early intervention when febrile- cbc, blood cx, rocephin (<2 yo admit) -300-600x more likely to develop pneumococcal infection, Haemophilus influenzae sepsis -greatest risk if 0-5 yrs -young children should get PCN prophylaxis |
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Sickle Cell disease-- splenic sequestration
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-uncommon but rapidly fatal
-5-24 mo of age -enlarging spleen and worsening anemia -treat with immediate fluid/blood resuscitation -transfuse to Hgb 9-10 -watch for release of sequestered blood -surgery (1 major or 2 minor) |
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Sickle Cell Disease-- Aplastic crises
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-decreasing Hgb, increasing pallor, reticulocytopenia means aplastic crises
-PLT and WBC unaffected -persist for 10-14 days -profound drop in Hgb level (as low as 1g/dl) -almost always associated with infection (Parvovirus B19) -recovery after 10 days (reticulocytosis up to 50%) -folic acid supplementation necessary |
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Prophylaxis in children with sickle cell anemia
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Age 0-3 PCN VK 125 mg BID
Age 3+ PCN VK 250mg BID PCN allergy: erythromycin |
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Family testing and Counseling for Hgb S and C trait patients
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-laboratory testing of parents and siblings (CBC, Hgb electrophoresis)
-Provide educational information (genetics, what trait means) -consider confirmatory testing at 6-12 months with CBC and Hgb electrophoresis |
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Sickle Cell trait
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-2 fold increase risk of venous thromboembolism
-renal disease -2 fold increase in renal medullary carcinoma -increased rates of end stage renal disease -increased hematuria -impaired urinary concentration |
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Hemophilia
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-Factor VIII or Factor IX deficiency
-X-linked -Major bleed (head, abd or ocular trauma) -Correct to 100% (don't wait for labs or scans) -Factor VIII 1U/kg= 2% increase -Factor IX 1U/kg= 1% increase -Appropriate factor 50U/kg q8-12h -Fresh frozen plasma or cryoprecipitate if factor unavailable |
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Disseminated Intravascular Coagulopathy
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decreased platelets, factors II, V, VIII, XIII, and fibrinogen
increased fibrin split products associated with severe systemic illness/trauma |
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treatment of DIC
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-treat underlying cuase
-supportive care -transfuse plt to maintain >150 -FFP (10-15mg/kg) to replace clotting factors -cryoprecipitate to maintain fibrinogen >100 |
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calculate ANC
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WBC x neutrophils= ANC
*neutrophils= segs + bands *example: WBC = 4.3 Segs = 48% Bands = 2% 4300 x (0.48 + 0.02) = 4300 x 0.5 = ANC of 2,150 |
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Oncology
Infection support |
Education: Fever= emergency!
Fever is >100.4 in 2 separate occasions, or >101 Admit all neutropenic patients ANC<500 with IV broad spectrum coverage -3rd/4th gen cephalosporin with pseudomonas coverage (Cefepime +/- Vanc) -after 3-5 days persistent fever, add anti-fungal coverage (Amphotericin vs. Voriconazole) -Continue until neutropenia resolves, afebrile, and neg blood cx *NO rectal temp d/t risk for infection |
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Oncology
Infections are mainly from enteric organisms that migrate to blood or other body fluids or cause local infection. what are common organisms? and sites of entry? |
Staphylococcus epidermitis and aureus (MRSA), Enterococcus, alpha hemolytic strep, pseudomonas, fungus (aspergillosis, candida)
sites of entry- central line, GI tract, GU tract, peri-rectal area, mucositis |
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Oncology Infection support
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IV Broad spectrum antibiotics for febrile non-neutropenic patients (ANC>500) with central lines
-3rd gen Cephalosporin (ceftriaxone) -Treat aggressively for otitis media, sinusitis, pneumonia, superficial skin infections |
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Oncology
Management if pt has been exposed to varicella |
-any patient with an active exposure to varicella needs treatment regardless of history of disease or immunization
-risk for disseminated varicella -IVIG (intravenous gamma globulin) -Shingles may also become disseminated on reactivation -IV acyclovir -Valacyclovir daily prophylaxis until completion of therapy |
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Oncology
Pneumocytis carinii pneumonia prophylaxis |
Bactrim/Septra
Dapsone Pentamidine |
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Oncology
HSV prophylaxis |
acyclovir or valacyclovir
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Oncology
Fungal Prophylaxis |
fluconazole vs. voriconazole
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Neutropenic precautions
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-handwashing
-minimize ill contacts -mask for health care workers with mild URI symptoms -no sushi, raw oysters, or rare meat -fresh fruits and vegetables are fine if the family has prepared |
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Nutritional support of oncology patients
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-follow growth curves (most children will not grow during intense chemotherapy--goal is to maintain weight, catch-up growth after therapy)
-High calorie foods -taste will change with most chemotherapeutic agents |
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Nutritional intervention
Weight loss >5% |
-nutritional referral
-increase calories in foods, PO supplements -ensure effective anti-emetic treatments in place |
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Nutritional intervention
weight loss >10% |
-appetite stimulants: periactin, magace, marinol
-supplemental feeds (NGT vs Gastrostomy tube) *TPN last resort--- very hard on liver |
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Oncology
Lymphadenopathy |
70% of children <7 yo have enlarged nodes
size and location >0.5--- epitrochlear >1cm-- cervical, axillary >1.5 cm-- inguinal |
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_____ and _____ adenopathy are always abnormal
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epithrochlear
suprclavicular |
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most common cause of acute cervical adenopathy
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strep
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if a patient with lymphadenopathy has no response to abx in 7-14 days or firm non-flunctuant or >2cm, what labs should be ordered?
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CXR, CBC, LDH, Uric Acid, Phos, Ca, CMP, ESR
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malignant nodes
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firm, rubbery, and matted
nontender |
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Which diseases are associated with generalized lymphadenopathy?
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ALL
AML NHL (non-hodgkin's leukemia) Hodgkin's lymphoma Neuroblastoma |
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symptoms of mediastinal masses
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cough, dyspnea, orthopnea, sridor
superior vena cava syndrome -SOB upon lying down -MEDICAL EMERGENCY *DO NOT SEDATE THESE PATIENTS |
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Most common dx of mediastinal masses
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lymphoma, leukemia, neuroblastoma, rhabdomyosarcoma, PNET-- primitive neuroectodermal tumors
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Oncology
Headache |
-Few headaches are associated with tumor
-symptoms: -AM headaches, wake from sleep -incapacitating h/a, vomiting, changing pattern -abnormal neurological exam -vision loss, changes -less than 3 yrs old Brain tumors are the most frequently seen solid tumors--- CAT scan |
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Oncology
Bone pain Bad signs? |
-pain awakens child from sleep
-abnormal function with no trauma -no cause found in 2 weeks Bone cancer (Ewings and osteo) -intermittent at first, then worse Limp reported 1/3 of leukemia cases Neuroblastoma can present with bone pain (Abd U/a if suspected, urine VMA/HVA) |
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presenting signs of Acute Lymphoblastic Leukemia (ALL)
-most common childhood cancer -cure rate 88% -chemotherapy |
pancytopenia, anemia, thrombocytopenia, leukopenia OR leukocytosis, LAD, hepatosplenomegaly, bone pain, bruising, palness, fatigue
WBC are age are most impt prognostic factors --age >1 and <10 --WBC <50,000 |
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Non-Hodgkins lymphoma
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-usually 2nd decade of life
-rare <3 yo -most common malignancy in children with AIDS |
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presenting signs of non-hodgkins lymphoma
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-LAD without fever,
-fever -weight loss -bone pain -pancytopenia (low RBC, WBC, PLT) -headaches and vomiting -cough, dyspnea, pneumonia, dysphagia -SVC syndromes -abdominal masses -tumor lysis syndrome, renal failure |
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presenting signs of hodgkins lymphoma
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-painless lymph node enlargement (cervical and supraclavicular chains)
-subacute and prolonged onset -30% have systemic sx: fever, pruritus, weight loss, night sweats -B symptoms worse prognosis -2/3 with anterior mediastinal mass -high ESR and eosinophilia |
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presenting signs of neuroblastoma
<30% have long term survival rate--5 yrs |
depend on site
1) adrenal gland -abdominal mass or pain -firm, irregular and non-tender 2) cervical ganglia -horner syndrome, heterochromia 3) thoracic -dysphagia, dyspnea, infections, cough 4) pelvic -difficulty urinating or defecating 5) paraspinal -back pain, paraplegia, constipation |
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Wilm's Tumor
<90% survive to 5 yrs |
-asymptomatic abdominal mass
-1/3 will have pain, vomiting, listlessness -+/- hematuria -hypertension in 25% -anemia-- bleeding into tumor |
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germline mutations
Beckwith-Wiedemann 11p15 |
-macroglossia, hemihypetrophy, visceromegaly, omphalocele, facial nevus flammeus, ear lob crease
-increased risk for developing wilms tumor -US Q3 months until 7 yo |
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Left eye swelling
rapidly progressing vision changes |
rhabdomyosarcoma
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Rhabdomyosarcoma
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-soft-tissue maglignancy
-involves any skeletal muscle -most common presentation: painless, enlarging mass, sx related to mass, head, neck, orbit, gu, trunk |
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Osteosarcoma
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bone pain localized to one area
stage impt prognostic factor-- meatstases 30%, none 70% tumor site distal better prognosis; axial poor prognosis resectability is important b/c cancer is resistant to radiation |
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Retinoblastoma
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-leukocoria in one or both eyes (presents in 50% of pts, present when tumor is large or has detached retina)
-strabismus (present in 25% of pts) -bilateral 20-30% of the time -median age is 2 yrs old |
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Hyperleukocytosis
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-WBC >100,000
-commonly seen in ALL, AML, and T cell ALL with a mediastinal mass -myeloblasts are larger and stickier results in increased viscocity -intracranial and pulmonary hemorrhage -leukostasis -tumor lysis syndrome |
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Symptoms of Hyperleukocytosis
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CNS: mental status changes, headache, seizures, papilledema
Pulmonary: dyspnea, hypoxemia, RV heart vailure *CXR may be normal or show diffuse interstitial infiltrates |
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management of hyperleukocytosis
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-IV hydration (2-4x maintenance)
-Alkalinzation -allopurinol, -blood products (Plts <20k, PRBCs only if unstable, if Hgb >10 can cause sludging) -Exchange transfusion or leukopheresis |
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Tumor lysis syndrome
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occurs when large # of neoplastic cells are killed rapidly, leading to release of intracellular ions and metabolic byproducts into the systemic circulation
-Hyperuricemia----> renal failure -Hyperkalemia---> arrythmia -Hyperphosphatemia->hypocalcemia |
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signs/ symptoms of tumor lysis syndrome
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GI complaints, spasms, tetany, Loss of consciousness, seizures
PE: BP, mental status, cardiac rate/rhythm, abd masses, ascites, signs of superior vena cava syndrome |
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Treatment of Hyperuricemia in Tumor Lysis Syndrome
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Hyperuricemia
-uric acid crystals form in the collecting ducts -can lead to renal failure (levels >20) Txt: -hydration, alkalization -allopurinol vs rasburicase -monitor urine output, ph |
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Treatment of Hyperkalemia in Tumor Lysis syndrome
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Hyperkalemia
Severe if >6.5 or ECG changes (peaked T waves) Therapy -eliminate K intake -Calcium chloride -sodium bicarbonate -insulin -glucose -kayexelate -dailysis |
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Treatment of Hyperphosphatemia in Tumor Lysis Syndrome
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-lymphoblasts are rich in phosphate
associated with hypocalcemia -treat low Ca only if symptomatic -if Ca X PO4 reaches 60, calcium phosphate can precipitate adding to the risk of renal failure Txt: -dietary restriction, phosphate binders (amphogel), IV Ca gluconate for hypocalcemia |
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symptoms: SOB worse when supine, cough, and facial swelling
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Superior Vena Cava Syndrome
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SVCS
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venous and tracheal compression
progresses slowly associated with: lymphoma, T cell ALL, solid tumors, Central venous line occlusion |
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Clinical features of SVCS
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-swelling, plethora and cyanosis of face, engorged collateral
-wet brain syndrome -cough, hoarseness, dyspnea, orthopnea -lying on back makes sx worse *keep pt sitting up |
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Management of SVCS
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-Keep pt sitting up
-NO SEDATION -DX aids: AFP, B-HCG -treatment: radiation vs chemo, steroids, central lines |
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Spinal Cord compression
up to 4% of children with cancer |
--Compression of spinal cord (above T10)
-back pain in 80% -increased deep tendon reflex Compression of conus medullaris (T10-12) -DTR mixed pattern -sphincter disturbances Compression of the cauda equina (below L2) -depression DTRs -sphincter disturbance in 50% |
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Management of Spinal Cord Compression
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-- evalution: history/PE/plain films/ MRI with or w/o contrast
--Treat first!!!, then MRI, if sx are progressive -Dexamethasone, -Rapid decompression for epidural masses (radiation vs surgery vs chemo) |
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Outcome of spinal cord compression
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depends on duration of sx
1/2 who present non-ambulatory will NEVER regain function |
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most common CNS tumors increasing ICP
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astrocytomas
medulloblastomas |
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signs and symptoms of increased ICP
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-headache, emesis, diplopia, ataxia, speech disturbance
-cushing's triad, abnormal EOM movements, papilledema |
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Management of Increased ICP
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--stabilize pt before imaging
--dexamethasone, mannitol, elevate HOB, prophylactic seizure meds, maintain PLT count, --neuro intervention |
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leading cause of death in children with cancer is:
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infection
as high as 50% of pts have an established or occult infection Definition of fever >101 or >100.4 x2 in 24 hour period Levels of risk greatest with ANC <500 or ANC <1000 and expected to decline |
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Fever and Netropenia
Treatment? |
-empiric broad based abx
-monotherapy (cefipime, ceftazidime, imipenem -multiple drug therapy (+/- vanc) -antifungals (amphotericin, micafungin, voriconazole) -antivirals (acyclovir) -colony stimulating factors (G-CSF) |
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children with immune system defects typically present with
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1) severe, deep tissue or life threatening events (boils, abscesses)
2) recurrent or protracted infections 3) infections with unusual organisms |
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reassuring features of children with recurrent illnesses
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1) prior diagnoses are vague and lack clear documentation
2) lack of any deep tissue infections 3) presence of normal growth and development 4) general wellness (overall activity, absence of complaints between infectious episodes) |
