peds30 Flashcards
(100 cards)
1
Q
treatment for anaphylaxis
A
epinephrine acutely; systemic antihistamines, steroids, beta ags are also used
2
Q
allergic shiners
A
dark circles nder the eyes caused by venous congestion
3
Q
dennie’s lines
A
creases under the eyes as a result of chronic edema; seen in allergic rhinitis
4
Q
allergic salute
A
when patient uses palm of hand to elevate tip of nose to relieve itching
5
Q
nasal smear in allergic rhinitis
A
more than 10% eosinophils suggests allergic rhinitis; lots of PMNs suggests infectious cause
6
Q
most effective drugs for rhinitis
A
intranasal steroids; antihistamiens, intransala cromolyn sodium; decongestants
7
Q
difference between first and second generatio antihistamines
A
second are safer and better tolerated but not any more effective
8
Q
how does cromolyn sodium work?
A
prevents mast cell degranulation
9
Q
side effects of decngestants
A
pseudoephedrine; insomnia, nervousness, and rebound rhinitis
10
Q
immunotherapy
A
repeated injections of the allergen lead to better tolerance over time
11
Q
atopic dermatitis
A
eczema; dry skin and lichenification; pruritis leads to scratching
12
Q
when does eczema first present
A
usually in infancy; almost always before 5 yo
13
Q
fam hx of eczema
A
yes
14
Q
clinical features of atopic dermatiits
A
pruritis; erythema, weeping and crusting; lichenification; pigmentary changes; secondary infection is common
15
Q
infantile eczema presentation
A
truncal and facial areas, along with the scalp; extensor surfaces more common than flexor
16
Q
early childhood eczema
A
flexural surfaces are more severely affectd and lichenification; chronic itching
17
Q
late childhood eczema
A
disease more localized or tendency towards remission
18
Q
diagnosis of eczema
A
three of four critera: pruritis, personal or family hx of atopy, typical morpology and distribution, and relapsing or chronic dermatitis
19
Q
atopy
A
predisposition toward allergies
20
Q
treatment for eczema
A
steroids, antihistamines, baths, avoid known triggers
21
Q
exclusive breastfeeding for the first 6 mos may decrease food allergies
A
and atopic dermatitis
22
Q
radioallergosorbent tests (RAST)
A
identify serum IgE antibodies to spec food antigens
23
Q
acute vs chronic urticaria
A
acute is ppt by something; chronic is greater than 6 mos and may be associated with underlying condition like malignancy or thyroid disease
24
Q
common causes of drug allergies
A
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)
