Pediatric

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IgA deficiency


Peds

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most common immunodefieciency
recurrent respiratory and GI infections. IgA low. Avoid immunoglbulins therapy (anaphylaxis)

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Recurrent respiratory and GI infections.

Peds

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IgA defiency

Front 3

Therapy to avoid in IgA deficiency

Peds

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Immunoglbulins may cause anaphylaxis.

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X-linked agammaglobulinemia
(Bruton agammaglobulinemia)

Peds

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X-llinked
Low or absent Bcells
begins after 6 months (material antibodyes disappear)
Look for recurrent lung or sinus infection with Streptoccus and Hemophilus

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6 month old male with recurrent lung/sinus infection. low or absent B cells. Culture Streptoccus. Might be?
Peds

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X-linked aggammaglobulinemia (Bruton agammaglobulinemia)

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Di George syndrome

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caused by hypoplasia of third and fourth haryngeal pouches.
Hypocalcemia or tetany (from absent parathyroids) in the first 24-48 hours of life, along with an absent or hypoplastic thymus. Congenital heart defects are common.

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A 2 day old infant presents with hypocalcemia and tetany

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DiGeorge syndrome is caused by hypoplasia for third and fourht pharyngial pouches. (absent parathyroids, absent or hypoplastic thymus) Congenital heart defects are common

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Severe combined immunodeficiency

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may be autosomal recessive or X-linked. Many cases are due to adenosine deaminase deficiency (autosomal recessive). Have B and T cell defects and sever infections in the first few months of life. cutaneous anergy usally is presnet. The thymus andlymph nodes may be absent or hypoplastic

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One month old with severe infections over the last month. Physical exam reveals absent of thymus and lymph nodes. Cutaneous anergy is present. B and T cell defects.

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Severe combines immunodeficiency

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Many cases are due to adenosine deaminase deficiency (autosomal recessive)

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Severe combined immunodeficiency

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Wiskott-Aldrich deficiency

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X linked recessive disorder that affects males. Looks for the classic triad: eczema, thombocytopenia and reccurrent infections usually respiratory

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Infant presents with eczema, thrombocytopenia and recurrent infection of the lung (classic triad)

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Wiskott-Aldrich deficiency

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Chronic granulomatous disease

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usually an x linked recessive disorder that affects males. Infections with catalase-positive organisms (ie S. aureus, Pseudomonas spp.) are common. The diagnos is clinched when deficient nitroblue tetrazolium dye reduction by granulocytes is present (a test that measures the resiratory burst, which these patient lack.

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You suppect Chronic granulomatous disease in a male infant infected with catalase positive organzism. What diagnostic test would clarify this?

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deficient nitroblue tetrazolium dye reduction by granulocytes

Front 15

Chediak-Higashi syndrome

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usually autosomal recessive. Look for giant grnaules in neutrophils and associated oculocutanious albinism. The syndrom is caused by a defect in micrtubule polymerization.

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Giant granules in neutrophils and asssociated oculocutaneous albinism. What do you suspect

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Chediak-Higashi syndrome

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Complement deficiencies

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(factors C5 though C9) cause recurrent neisserial infections. Specific complement components are low

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Cause recurrent neisserial infections. Specific complement components are low

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Complement deficiencies (factor C5 through C9)

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Chronic mucocutaneous candidiasis

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is a cellular immunodeficiency specific for Candida spp. Patients have thrush; candidal infections of the scalp, skin and nails and anergy to Candida spp. with skin testing. The condition often is associated with hypothyroidism; the rest of immun function is intact

Front 20

What might you suspect with a infant with chronic thrush, skin anergy positive for candia and the rest of the immune function intact

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Chronic mucocutaneous candidiasis

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Hyper-IgE syndrome (Joe-Buckley syndrome)

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is characterized by recurrent staphyloccoccal infections (especially of the skin). IgA levels are extremely high. Patients commonly has fair skin, red hair and eczema.

Front 22

A cute red headed, pale child present with recurrent staphyloccal infection of the skin and extremely high IgA levels.

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Hyper-IgE sydrome (Joe-Buckley syndrome)

Front 23

Otitis media is most commonly due to what organism?

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Steptococcus pneumonia, Haemophilus influenzea and Moraxella catarrhalis

Front 24

Patient presents with earache, fever, nausea/vomiting and erythermatous and bulging tympanic membrane (light reflec and landmarks are difficult to see). DX. Otitis media. What complication might occur if not treated appropriately?

Back 24

Tympanic membrane perforation (bloody or purulent discharge), mastoiditis (fluctuation and inflammation over mastoid process 2 week after otitis), labyrinthitis palsies of cranial nerves VII and VIII, meningitis/cerebral abscess, venous thomboisis and chronic otitis media which can lead to permanent perforation of eardrum, and patients may develop cholesteatomas with marginal perforation (treat with surgical excision)

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Patietn presents with cholesteatomas with marginal perforation of eardrum. How might this be treated

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surgical excision.

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What do you treat otitis with?

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amoxicillin, second generation cephalosporin such as cefuroxime, or trimethoprim-sulfamethoxazole.

recurrent: tubes controversial, adenoidectomy is some cases helpful.

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What are complications of recurrent otitis media

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hearing loss with resultant develpmental problems (speech, cognitive functi0ns)

Front 28

Presenting symptom of a urinary tract malformation in children less then 5

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UTI

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Most common examples of urinary tract malformation in kids
PEDS

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Vesicoureteral reflux (males and females) and posterior urethral valves (males)

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You suspect a urinary tract malformation in a boy under 6 years old with a UTI what test do you order

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renal untrasound and a voiding cystourethrogram
NOTE: same test should be order for a girl under six with UTI or pryelonephritis

Front 31

The highest incidence of meningitis is seen in_____; the majority of cases are seen in children younger than____years

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neonates
two

Front 32

Since young cheldren with meningitis do not have the classic physical findings (Kernig and Brudzinski signs) What should you look for to help you decide when to do a lumber puncture.

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Lethargy, fever or hypothermia, poor muscle tone, BULGINF FONTANELLE, ALTERED CONSCIOUSNESS,vomitng, photo hobia and signs of general sepsis. Seizure may also be seen, but remember simple febrile seizures if the patient is between 5 month and 6 years old and has a fever greater then 102 in the absence of other signs of meningitis.