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What is a hemangioma?
A benign self-involuting tumor of endothelial cells with unknown cause
most common tumor in infancy
"blood vessel tumor"
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Bruton's Agammaglobunemia
- - B cell deficiency because failure to mature
- - Bacterial and fungal infections increased
- - Boys
- - mutation on X chromosome (mutation in B cell tyrosine kinase)
- - primary immunodeficiency disease that is congenital
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Wiskott-Aldrich Syndrome
- Increased IgA, IgE, low IgM
- - only in Boys
- - WIPE:
- 1) Wiskott
- 2) recurrent pyogenic Infections
- 3) thrombocytopenic Purpura
- 4) Eczema
- - also incrased risk of lymphoid cancers
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Ataxia-Telangiectasia
- - inherited disorder affects many tissues
- - defect in DNA repair enzymes results in IgA deficiency
- 1) telangiectasis- dilation of capillaries; on whites of eyes and skin areas
- 2) ataxia- uncoordinated movements in late childhood
- - increased risk of malignancies
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Job Syndrome aka Hyper IgE syndrome
- - immunodeficiency disorder
- - decrease activation of PMNs bc Th cells don't produce interferon-gamma (activates macrophages and CD8+ Tc cells)
- - The Jobs of PMN's depends on FATE
- 1) Facies
- 2) Abscesses from S. aureus
- 3) primary Teeth retained
- 4) high igE and Eczema
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Lambert-Eaton Syndrome
- - NMJ disease
- - autoantibodies to Ca2+ channels on presynaptic nerve--> decreases Ach release
- - Sx: proximal muscle weakness
- - symptoms improve w/ muscle use
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What is Osteochondrosis?
Characteristic Sites?
- - during rapid bone growth, blood supply to epipheses of bones insufficient
- - get necrotic bone near joints= avascular necrosis
- the degeneration and aseptic necrosis followed by regeneration and reossification of 1+ ossification centers in children
- 1) head of femur- Legg-Calve-Perthes disease
- 2) Tibial tuberosity- Osgood-Schlatter
- 3) Intervertebral joints- Scheuermann disease
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Gardner's Syndrome
- Autosomal dominant polyposis syndrome
- 1) adenomatous polyps
- 2) bone and skin tumors
- - risk of malignant transformation 100%
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Turcot's Syndrome
- Autosomal recessive
- 1) adenomatous polyps
- 2) brain tumors
- high risk of malignant transformation
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Peutz-Jeghers Syndrome
- Autosomal dominant
- 1. Polyps of colon and small intestine
- 2. melanotic macules on lips, hands, genitalia
- - no malignant potential of polyps themselves
- - increases risk for adenocarcinoma of colon, stomach, breast, lung, ovaries
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What is Histiocytosis X?
Three Types?
Which has the worse prognosis?
- - Langerhans cell histiocytosis
- - Langerhans cells increase in # and invade body tissues
- - unknown cause
mnemonic: Enduring Histiocytes Live
- 1. Eosinophilic Granuloma
- - mild/benign form
- - solitary lesion on bone--> pain and swelling
- - 80% of histiocytosis X
- - in children 5-10 yrs
- 2. Hand-Christian- Schuller Disease
- - children ages 1-3 w/ classic triad:
- 1) skull lesions
- 2) diabetes insipidis
- 3) exophthalmos
- 3. Letterer-Siwe Disease
- - very aggressive, fatal ending
- - multiple organ systems involved: bone, lungs, skin, liver, spleen, bone marrow
- - age 0-1 yr
- - worst prognosis- depends on age at diagnosis (<2 yrs worse) and how many organs involved
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Polyarteritis nodosa
- - collagen vascular disease
- - blood vessel disease w/ unknown cause
- - necrotizing immune complex inflammation of small and medium sized arteries
- - associated with HEP B infection
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Cause of dermatomyositis?
- unknown cause, but damage to myofibers by CD8+ T cells
- 1) proximal muscle weakness
- 2) malar rash (similar to SLE) heliotrope rash
- - increased risk of malignancy
- - finding: + ANA, anti-Jo1
- similar to polymyositis (but no rash)
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Most common tumor of salivary glands?
Where?
Most common malignancy of salivary glands?
- Pleomorphic (mixed) adenoma
- -Parotid Gland> submandibular > Sublingual
- - plasmacytoid cells are diagnostic
- Mucoepidermoid Carcinoma
- - mucous secreting cells and and epithelial cells
- - accompanied by pain
- - may show damage to CN VII
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Ludwig's angina
- - life-threatening cellulitis infection of floor of mouth; usually in dental infection
- - angina= severe pain
- - usually from infection of mandibular molars
- - mixed infection- usually Streptococci always present
- 1) brawny/muscular induration that doesn't pit on pressure
- 2) rapid onset
- 3) Dysphagia, dyspnea, fever
- - EMERGENCY when swelling blocks airway
- - Tx- get rid of infection w/ antibiotics (penicillin)
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1. Acute Appendicitis signs and symptoms?
2. Most common neoplasm of appendix
- - most common emergency surgical procedure
- - most common in 10-19 yr olds
- Clinical Signs:
- 1. child walks bent over, limping, holding right side
- 2. diffuse abdominal tenderness
- 3. McBurney's point
- - point tenderness
- - rebound tenderness- indicates inflammation spread to peritoneum
Tx- surgical removal
2. carcinoid tumor- rarely metastasizes
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1. Most common cause of meningitis is adults?
2. In children under 2?
3. Signs and Symptoms
4 What is Waterhouse Friederichsen syndrome?
1. N. Meningitidis and S. Pneumoniae
2. H. Influenzae
3. high fever, stiffness of neck, severe headache bc inflammation of meninges
- 4. rapid infection by N. meningitidis
- - diarrhea
- - vomiting
- - bilateral adrenal hemorrhage--> low BP, shock, seizures
- - death
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Amyloidosis
1. Primary
2. Secondary
3. Hereditary
proteinaceous material (amyloid) deposits and accumulates in tissues and organs
- 1. unknown cause
- - malignant plasma cells abnormally produce a lot of Ig's that accumulate and deposit onto tissues
- - systemic: HEART, lungs, skin, tongue, thyroid gland, intestines, liver, kidney, blood vessels
- - e.g. Multiple Myeloma (CRAB- hypercalcemia, renal disease, Anemia, Bone pain)
- 2. reactive; complication of other diseases (TB, rheumatoid arthritis, etc)
- - heart rarely involved
3. rare; genetic mutations
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Down Syndrome
- Trisomy 21 chromosomal abnormality
- - mental retardation
- - large protruding tongue
- - small ears, large forehead, wide space eyes
- - simean crease; short, broad hands
- Complications
- - congenital heart disease
- - increased infections
- - ALL (we ALL fall DOWN)
- - increased Alzheimers
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Cri Du Chat Syndrome
- Chromosome 5 short arm deletion
- 1. severe mental retardation
- 2. microcephaly
- 3. catlike cry
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Edward's Syndrome
- Trisomy 18
- 1. mental retardation
- 2. pinched face appearance
- 3. micrognathia (Small jaw)
- 4. small head w/ low set ears
- 5. congenital heart disease
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Patau Syndrome
-Trisomy 13
- 1. mental retardation
- 2. microcephaly
- 3. microphthalmia
- 4. Cleft lip and Palate
- 5. congenital heart disease
- 6. polydactyly (extra fingers)
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