Peds Immuno

eesohbel's version from 2015-12-09 20:23


Question Answer
B cell deficienciestypically present after 6 months of age w/recurrent sinupulmonary, GI, UTIS w/encapsulated organisms
decrease of circulating B cells and decrease in all immunoglbulinsthink Bruton agammaglobulinemia
inheritance pattern of Bruton agammaglobulinemiaX-linked boys
absent tonsils and other lymphoid tissueBrutons
all Ig levels are low, normal B cellsCVID
most common B class defectIgA deficiency
anaphylactic reactions if given bloodIgA defciency
do not give IVIGIgA deficiency
Parathyroid and thymic hypoplasianeonatal hypocalcemic seizures; tetany in DiGeorge
absent thymic shadowDiGeorge
facial features DiGeorgedysmorphic features, mandibular hypoplasia
treatment for DiGeorge syndrometransplantation of thymic tissue of MHC compatible sibling or half matched parenteral bone marrow
SCIDabsence of all adaptive immune function
treatment for SCID this is a true pediatric emergency. need a bone marrow transplant or death by 1 year of age. Requires PCP prophylaxsis.
lab for Wiskott-Aldrichdecreased IgM, increased IgA and IgE, and thrombocytopenia
clinical findings of Wiskott-Aldrichatopic dermatitis, thrombocytopenia, susceptibility to infection
mnemonic for Wiskott-AldrichWIPE. Wiskott Aldrich, Infections, Purpura, Eczema
CGD diagnosisNBT replaced now by flow cytometry
what disease should be considered in a patient with recurrent infections with catalase positive organismsCGD
treatment for CGDtreat w/ daily TMP-SMX
patients with a history of delayed separation of the umbilical cordLAD
JObshyper IgE
giant lysosomes in neutrophilsChediak-Higashi
do not give IVIGIgA deficiency
all Ig levels are low, but normal B-cellsCVID
treat with daily TMP-SMXCGD
partial oculocutaneous albinism, peripheral neuropathy and neutropeniaChediak Higashi
symptoms of Job'sFATED (Coarse Facies, Abscesses, Retained Primary Teeth, Hyper-IgE, Dermatologic)
examples of catalase positive organismsS. aureus, serratia, klebsiella, aspergillus
recurrent episodes of angioedemaC1 esterase deficiency
recurrent neisseria infectionsterminal complement deficieny (C5-C9)
NBT test is diagnosticCGD
progressive cerebellar ataxia and oculocutaneous telangiectasiasataxia telangiectasia

Kawasaki and Scarlet fever

Question Answer
common features btwn Kawasaki and scarlet feverstrawberry tongue, rash, desquamation of the hands and feet, erythema of mucous membranes
difference btwn Kawasaki and scarlet feverchildren with scarlet fever have normal lips and no conjunctivits
treatment for Kawasakihigh dose ASA and IVIG


Question Answer
pauciarticular (oligoarthritis)most common. involves 4 or fewer joints. usually young females. uveitis is common and usually slit lamp. no systemic symptoms.
polyarthritis5 or more joints. symmetric.
Stills diseasesystemic symptms. salmon colored macular rash.

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