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Flashcards in peds30 Deck (100):
1

treatment for anaphylaxis

epinephrine acutely; systemic antihistamines, steroids, beta ags are also used

2

allergic shiners

dark circles nder the eyes caused by venous congestion

3

dennie's lines

creases under the eyes as a result of chronic edema; seen in allergic rhinitis

4

allergic salute

when patient uses palm of hand to elevate tip of nose to relieve itching

5

nasal smear in allergic rhinitis

more than 10% eosinophils suggests allergic rhinitis; lots of PMNs suggests infectious cause

6

most effective drugs for rhinitis

intranasal steroids; antihistamiens, intransala cromolyn sodium; decongestants

7

difference between first and second generatio antihistamines

second are safer and better tolerated but not any more effective

8

how does cromolyn sodium work?

prevents mast cell degranulation

9

side effects of decngestants

pseudoephedrine; insomnia, nervousness, and rebound rhinitis

10

immunotherapy

repeated injections of the allergen lead to better tolerance over time

11

atopic dermatitis

eczema; dry skin and lichenification; pruritis leads to scratching

12

when does eczema first present

usually in infancy; almost always before 5 yo

13

fam hx of eczema

yes

14

clinical features of atopic dermatiits

pruritis; erythema, weeping and crusting; lichenification; pigmentary changes; secondary infection is common

15

infantile eczema presentation

truncal and facial areas, along with the scalp; extensor surfaces more common than flexor

16

early childhood eczema

flexural surfaces are more severely affectd and lichenification; chronic itching

17

late childhood eczema

disease more localized or tendency towards remission

18

diagnosis of eczema

three of four critera: pruritis, personal or family hx of atopy, typical morpology and distribution, and relapsing or chronic dermatitis

19

atopy

predisposition toward allergies

20

treatment for eczema

steroids, antihistamines, baths, avoid known triggers

21

exclusive breastfeeding for the first 6 mos may decrease food allergies

and atopic dermatitis

22

radioallergosorbent tests (RAST)

identify serum IgE antibodies to spec food antigens

23

acute vs chronic urticaria

acute is ppt by something; chronic is greater than 6 mos and may be associated with underlying condition like malignancy or thyroid disease

24

common causes of drug allergies

penicillin, aspirin and other NSAIDs, and narcotics

25

most common immune def

igA def

26

clinical symptoms of IgA def

both infections and autoimmune diseases amd allergies

27

treatment of IgA def

igA cannot be replaced, so tx by managing infections

28

common variable immunoef

group of disorders characterized by hypogammaglobulinemia;

29

clinical features of common variable immunodef

infections and autoimmune disorders and incr risk of malignancy; normal numbers of B and T cells but T cell dysfunction

30

treatment of common variable immunodef

monthly IVIG; chronic diarrhea management; aggressive management of infection

31

Severe combined immunodef Disease

SCID; profoundly defective T and B cell function

32

clinical features of SCID

incr susceptibility to infection int eh first year of life; chronic diarrhea and FTT

33

diagnosis of SCID

persistent lymphopenia; decr T cells; severe hypogammaglobinemia; T cell response to mitogens and antigens is depressed

34

management of SCID

monthly IVIG; blood products should be irradiated to prevent GVH disease; PCP prophylaxis; BMT can be curative

35

ataxia telangiectasia

aut recessive; combined immunodef, cerebellar ataxia, oculocutaneous telangiectasia, and predisposition to malignancy

36

genetics of ataxia telangiectasia

utation of long arm of chrom 11; aut recess;

37

immunoglobulins in ataxia telangiectasia

IgE def in 85% and igA def in 75%; diminshed T cell proliferation

38

management of ataxia telangiectasia

treat neuro complications, treat infectiosn, monitor for malignanciesl avoid ionizing radiation

39

DiGeorge syndrome

immunodef, cardiac defect, abnormal facies, thymic hypoplasia, cleft palate, and hypocalcemia because of deletion on chrom 22q11

40

CATCH-22

digeorge syndrome; cardiac defects, abnormal facies, thymic hypoplasic, cleft palate, hypocalcemia; also immunodef

41

wiskott-aldrich syndrome

x-linked disorder characterized by combined immunodef, eczema, and congenital thrombocytopenia with small platelets

42

immune system in wiskott-aldrich

decr igM; antibody response to polysach antigens is defective; cellar immune function is defective and anergy is present; no antigen-spec cytotoxic T cells; but normal number of T cells

43

management of wiskot-aldrich syndrome

bone marrow transplant is the therapy of choice; IVIG; splenectomy cures thrombocytopenia

44

x-linked (bruton's) agammaglobulinemia

severe hypogammaglobulinemia and paucity of mature B cells with normal T cell number

45

genetics of x-linked (bruton's) agammaglobulinemia

mutation in the bruton's tyrosine kinase gene on the x chrom; it is critical to normal B cell ontogeny

46

clinical features of x-linked agammaglobinemia

increased susceptibility to infections with encapsulated bacteria

47

diagnosis of x linked agammaglobinemia

decr in all ig subtypes; B cells absent; T cells present; mutation in the BTK gene

48

treatment for x linked agammaglobinemia

monthly IVIG

49

chronic granulomatous disease

defective neutrophil oxidative metabolism as a result of defects in NADPH system; severely impaired intracellular killing of catalase pos bacteria and some fungal pathogens

50

diagnosis of chronic granulomatous disease

NBT test demonstrates defecetive neutrophil oxidative burst

51

management of chronic granulomatous disease

abscesses drained and antibiotics; prophylactic bactim, itraconazole, interferon gamma, BMT is curative

52

chronic granulomatous disease classic feature

abscess formation

53

schwachman-diamond syndrome

decr neutrophil chemotaxis, cyclic neutropenia, and panc exocrine insuf; recurrent soft tissue infection, chronic diarrhea, and FTT

54

chediak-higashi syndrome

variable neutropenia and thrombocytopenia; patients have partial oculocutaneous albinism

55

complement def

genetic; most aut recessive

56

deficiencies of the early components of the classical pway

associated with autoimmune disease, like SLE

57

def of the late components of the classic pway

associated with increased susceptibility to meningococcal and gonoccocal infectins

58

def of C1 esterase inhib causes

hereditary angioedema; swelling of body parts; hands, feet, bowel, airway

59

CH-50

total serum hemolytic complement; normal means all components of complement system are present and functional

60

management of complement disorders

management of autoimmune disease; therapy with fibrinolysis inhib and attenuated androgens (danazol) for hereditary angioedema

61

henoch-schonlein purpura

igA mediated vasculitis

62

who does H-S affect?

kids less than 10; median age is 5 yo; males more likely to be affected

63

clinical features of H-S

viral or URI infection; distinctive skin, GI, and joint manifestations

64

skin manifestations of H-S

petechiae and palpable purpuric lesions on the butt and lower exremities; edema of hands, feet, scrotum; Gi or joint symptoms may precede the diagnostic rash in 30% of patients

65

joint manifestations of H-S

arthalgia or arthritis; knees and ankels most commonly involved

66

GI manifestations of HS

colicky abdominal pain, GI bleeding, incr risk of intussusception

67

renal manifestations of H-S

from mild hematuria to gross hematuria, nephrotic syndrome, and ESSRD

68

H-S what do you see in lab

increased igA; platelet counts are normal despite the presence of petechiae and purpura (i.e. the skin rash is nonthrombocytopenia purpura)

69

management of H-S purpura

pain control and hydration; steroids may be effective for relief of abdominal painadn arthritis

70

prognosis for H-S purpura

most patients recover within 4 weeks; recurs in 50% of patient; long term morbidity depends on severity of nephritis

71

kawasaki disease

acute febrile vasculitis of childhood of unknown origin; affects multiple organ systems

72

most common cause of acquired heart disease in kids in the US

kawasaki disease

73

mean age at presentation of kawasaki disease?

18-24 monhts

74

diagnostic criteria for kawasaki

fever greater than 102 lasting over 5 days; four of the five clinical signs of kawasaki

75

what are the five clinical signs of kawasaki

bilateral conjunctivitis without exudate; oropharyngeal changes (red, cracked, swollen lips); cervical adenopathy; rash; changes in distal extremities

76

what kinds of changes in distal extremities do you see in kawasaki?

early (first 7-10 days) you see brawny edema and induration of the hands and feet with red plams and soles; later (7-10 days after fever) peeling around nails or of the distal extremitis

77

other clinical features of kawasaki

cardiovascular manifestations, urethritis, aseptic meningitis, hydrops of the gallbladder, arthritis, anterior uveitis,

78

cardiovascular manifestations in kawasaki

coronary artery aneurysm occur in 20% of untreated; myocarditis, CHF, arrythmias; aneurysms of the brachial arteries

79

hydrops of the gallbladder

seen in kawasaki; acute RUQ abdominal pain

80

time course of kawasaki disease

acute phase 1-2 weeks; subacute phase weeks to month; convalescent phase (weeks to years)

81

management of kawasaki

IVIG wit aspirin within 10 days of onset of fever; acute phase- high dose ASA for anti-inflamm; subactute ohase- low dose ASA for antiplatele t effect; steroids if unrespons to IVIG

82

prognosis for kawasaki

if no coronary artery disease, no long term sequealae; mortality less than 1% even with CAD; risk of atherosclerotic disease in adulthood

83

juvenile rheumatoid arthritis

chronic joint inflamm in kids; mean age 1-3 years; more common in females

84

male v female predom in JRA

females are more likely to have JRA EXCEPT equally likely to have systemic-onset JRA and males more likely to have late onset pauciarticular JRA

85

three categories of JRA

pauciarticular, polyarticular, systemic-onset

86

pauciarticular JRA

less than 4 joints involved; early onset (female predom) present at 1-5 yo; pos ANA, high risk from chronic uveitis; late-onset (male predom) present over 8 yo; HLA-B27 pos with invovlement of sacral and iliac joint s

87

polyarticular

greater than 4 joints involved; rheum factor pos presents in kids over 8 and is more severe; rheum fact neg presents both early and late

88

rheumatoid factor

IgM molecule against IgG

89

polyarticular JRA- what joints?

both large and small

90

systemic onset JRA

aka Still's disease; high spiking fevers, transient rash, hepatosplenomegaly, lymphadenopathy; systemic features may overshadow joint sx

91

descirbe the fevers of stills disease (systemic JRA)

occur in the late afternoon and subsequently return to baseline

92

rash of stills disease

transient salmon colored; found on trunk and prox extremities, esp during febrile episodes; rash is evanescent (occurs with fever and then fades) and is nonpruritic

93

lab findings in JRA

microcytic hypochromic anemia (consistent w anemia of chronic disease); rheum factor negative in most patients; ANA present in some;

94

ANA in JRA

ANA is present in most with early onset pauci JRA; half of patients with polyart JRA; not present in kids with systemic onset JRA and late pauci

95

management of JRA

NSAIDS, immunomodulators for more severe sx; physical therapy

96

diagnostic criteria for JRA

age of onset less than 16 years; arthritis in at least one joint; duration of disease greater than 6 weeks; exclusion of other causes of arthritis

97

age of onset SLE

adolescence

98

most frequent cardiac manifestation of lupus

pericarditis

99

lab findins in SLE

elevated ESR, anemia of chronic disease, leukopenia, thrombocytopenia, proteinuria, ANA pos; RF may be pos; anti-DS DNA antibodies; anti-smith antibodies;

100

which antibodies can be used as a measure of disease progression in SLE

anti-DS DNA (not anti-smit)