peds30 Flashcards
treatment for anaphylaxis
epinephrine acutely; systemic antihistamines, steroids, beta ags are also used
allergic shiners
dark circles nder the eyes caused by venous congestion
dennie’s lines
creases under the eyes as a result of chronic edema; seen in allergic rhinitis
allergic salute
when patient uses palm of hand to elevate tip of nose to relieve itching
nasal smear in allergic rhinitis
more than 10% eosinophils suggests allergic rhinitis; lots of PMNs suggests infectious cause
most effective drugs for rhinitis
intranasal steroids; antihistamiens, intransala cromolyn sodium; decongestants
difference between first and second generatio antihistamines
second are safer and better tolerated but not any more effective
how does cromolyn sodium work?
prevents mast cell degranulation
side effects of decngestants
pseudoephedrine; insomnia, nervousness, and rebound rhinitis
immunotherapy
repeated injections of the allergen lead to better tolerance over time
atopic dermatitis
eczema; dry skin and lichenification; pruritis leads to scratching
when does eczema first present
usually in infancy; almost always before 5 yo
fam hx of eczema
yes
clinical features of atopic dermatiits
pruritis; erythema, weeping and crusting; lichenification; pigmentary changes; secondary infection is common
infantile eczema presentation
truncal and facial areas, along with the scalp; extensor surfaces more common than flexor
early childhood eczema
flexural surfaces are more severely affectd and lichenification; chronic itching
late childhood eczema
disease more localized or tendency towards remission
diagnosis of eczema
three of four critera: pruritis, personal or family hx of atopy, typical morpology and distribution, and relapsing or chronic dermatitis
atopy
predisposition toward allergies
treatment for eczema
steroids, antihistamines, baths, avoid known triggers
exclusive breastfeeding for the first 6 mos may decrease food allergies
and atopic dermatitis
radioallergosorbent tests (RAST)
identify serum IgE antibodies to spec food antigens
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
common causes of drug allergies
penicillin, aspirin and other NSAIDs, and narcotics
most common immune def
igA def
clinical symptoms of IgA def
both infections and autoimmune diseases amd allergies
treatment of IgA def
igA cannot be replaced, so tx by managing infections
common variable immunoef
group of disorders characterized by hypogammaglobulinemia;
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
treatment of common variable immunodef
monthly IVIG; chronic diarrhea management; aggressive management of infection
Severe combined immunodef Disease
SCID; profoundly defective T and B cell function
clinical features of SCID
incr susceptibility to infection int eh first year of life; chronic diarrhea and FTT
diagnosis of SCID
persistent lymphopenia; decr T cells; severe hypogammaglobinemia; T cell response to mitogens and antigens is depressed
management of SCID
monthly IVIG; blood products should be irradiated to prevent GVH disease; PCP prophylaxis; BMT can be curative
ataxia telangiectasia
aut recessive; combined immunodef, cerebellar ataxia, oculocutaneous telangiectasia, and predisposition to malignancy
genetics of ataxia telangiectasia
utation of long arm of chrom 11; aut recess;
immunoglobulins in ataxia telangiectasia
IgE def in 85% and igA def in 75%; diminshed T cell proliferation
management of ataxia telangiectasia
treat neuro complications, treat infectiosn, monitor for malignanciesl avoid ionizing radiation
DiGeorge syndrome
immunodef, cardiac defect, abnormal facies, thymic hypoplasia, cleft palate, and hypocalcemia because of deletion on chrom 22q11
CATCH-22
digeorge syndrome; cardiac defects, abnormal facies, thymic hypoplasic, cleft palate, hypocalcemia; also immunodef
wiskott-aldrich syndrome
x-linked disorder characterized by combined immunodef, eczema, and congenital thrombocytopenia with small platelets
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
management of wiskot-aldrich syndrome
bone marrow transplant is the therapy of choice; IVIG; splenectomy cures thrombocytopenia
x-linked (bruton’s) agammaglobulinemia
severe hypogammaglobulinemia and paucity of mature B cells with normal T cell number
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
clinical features of x-linked agammaglobinemia
increased susceptibility to infections with encapsulated bacteria
diagnosis of x linked agammaglobinemia
decr in all ig subtypes; B cells absent; T cells present; mutation in the BTK gene
treatment for x linked agammaglobinemia
monthly IVIG
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
diagnosis of chronic granulomatous disease
NBT test demonstrates defecetive neutrophil oxidative burst
management of chronic granulomatous disease
abscesses drained and antibiotics; prophylactic bactim, itraconazole, interferon gamma, BMT is curative
chronic granulomatous disease classic feature
abscess formation
schwachman-diamond syndrome
decr neutrophil chemotaxis, cyclic neutropenia, and panc exocrine insuf; recurrent soft tissue infection, chronic diarrhea, and FTT
chediak-higashi syndrome
variable neutropenia and thrombocytopenia; patients have partial oculocutaneous albinism
complement def
genetic; most aut recessive
deficiencies of the early components of the classical pway
associated with autoimmune disease, like SLE
def of the late components of the classic pway
associated with increased susceptibility to meningococcal and gonoccocal infectins
def of C1 esterase inhib causes
hereditary angioedema; swelling of body parts; hands, feet, bowel, airway
CH-50
total serum hemolytic complement; normal means all components of complement system are present and functional
management of complement disorders
management of autoimmune disease; therapy with fibrinolysis inhib and attenuated androgens (danazol) for hereditary angioedema
henoch-schonlein purpura
igA mediated vasculitis
who does H-S affect?
kids less than 10; median age is 5 yo; males more likely to be affected
clinical features of H-S
viral or URI infection; distinctive skin, GI, and joint manifestations
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
joint manifestations of H-S
arthalgia or arthritis; knees and ankels most commonly involved
GI manifestations of HS
colicky abdominal pain, GI bleeding, incr risk of intussusception
renal manifestations of H-S
from mild hematuria to gross hematuria, nephrotic syndrome, and ESSRD
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)
management of H-S purpura
pain control and hydration; steroids may be effective for relief of abdominal painadn arthritis
prognosis for H-S purpura
most patients recover within 4 weeks; recurs in 50% of patient; long term morbidity depends on severity of nephritis
kawasaki disease
acute febrile vasculitis of childhood of unknown origin; affects multiple organ systems
most common cause of acquired heart disease in kids in the US
kawasaki disease
mean age at presentation of kawasaki disease?
18-24 monhts
diagnostic criteria for kawasaki
fever greater than 102 lasting over 5 days; four of the five clinical signs of kawasaki
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
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
other clinical features of kawasaki
cardiovascular manifestations, urethritis, aseptic meningitis, hydrops of the gallbladder, arthritis, anterior uveitis,
cardiovascular manifestations in kawasaki
coronary artery aneurysm occur in 20% of untreated; myocarditis, CHF, arrythmias; aneurysms of the brachial arteries
hydrops of the gallbladder
seen in kawasaki; acute RUQ abdominal pain
time course of kawasaki disease
acute phase 1-2 weeks; subacute phase weeks to month; convalescent phase (weeks to years)
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
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
juvenile rheumatoid arthritis
chronic joint inflamm in kids; mean age 1-3 years; more common in females
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
three categories of JRA
pauciarticular, polyarticular, systemic-onset
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
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
rheumatoid factor
IgM molecule against IgG
polyarticular JRA- what joints?
both large and small
systemic onset JRA
aka Still’s disease; high spiking fevers, transient rash, hepatosplenomegaly, lymphadenopathy; systemic features may overshadow joint sx
descirbe the fevers of stills disease (systemic JRA)
occur in the late afternoon and subsequently return to baseline
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
lab findings in JRA
microcytic hypochromic anemia (consistent w anemia of chronic disease); rheum factor negative in most patients; ANA present in some;
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
management of JRA
NSAIDS, immunomodulators for more severe sx; physical therapy
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
age of onset SLE
adolescence
most frequent cardiac manifestation of lupus
pericarditis
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;
which antibodies can be used as a measure of disease progression in SLE
anti-DS DNA (not anti-smit)
