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Ken Shared "Top 10 UGC cards by the ratings" - 6 Picmonics

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Top 10 UGC cards by the ratings

Familial Adenomatous Polyposis
Pathophysiology
Autosomal Dominant
APC
Chromosome 5
Management
Begin screening at 10-12 years old
Prophylactic removal of colon
Gardner Syndrome
Gardner syndrome
Osteomas
Dental Abnormalities
Soft Tissue Tumors
Congenital hypertrophy of retinal pigment epithelium
Turcot Syndrome
Turcot Syndrome
Medulloblastoma
CNS tumor
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2 mins
Langerhans cell histiocytosis
Collective group of proliferative disorders of dendritic (Langerhans) cells found predominantly in the skin.
Signs and Symptoms
Presents in a child as lytic bone lesions (IMAGE) and skin rash or as recurrent otitis media with a mass involving the mastoid bone.
Cells are functionally immature and do not effectively stimulate primary T cells via antigen presentation.
Birbeck granules (“tennis rackets” or rod shaped on EM) are characteristic (IMAGE)
Cells express CD1a
Cells express S-100 Tumor Marker (mesodermal origin)
LETTERER-SIWE DISEASE
Malignant proliferation of Langerhans cells
Classic presentation is skin rash and cystic skeletal defects in an infant ( < 2 years old).
Multiple organs may be involved; rapidly fatal
EOSINOPHILIC GRANULOMA
Benign proliferation of Langerhans cells in bone
Classic presentation is pathologic fracture in an adolescent; skin is not involved
Biopsy shows Langerhans cells with mixed inflammatory cells, including numerous eosinophils.
HAND-SCHULLER-CHRISTIAN DISEASE
Malignant proliferation of Langerhans cells
Classic presentation is scalp rash, lytic skull defects, diabetes insipidus, and exophthalmos in a child.
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Hepatocellular carcinoma
Hepatocellular carcinoma
Most common 1° malignant tumor of liver in adults
Increased alpha Fetoprotein
Increased Alkaline Phosphatase
Mildly increased LFT Values
Hematogenous spread
Associated with:
HBV
Hepatitis C
Alcoholic and non-alcoholic fatty liver disease
Hemochromatosis
Aflatoxin from Aspergillus
Alpha 1 Antitrypsin Deficiency
Symptoms
Jaundice
Ascites
Tender Hepatomegaly
Polycythemia
Anorexia
May lead to Budd Chiari syndrome
Dx: Ultrasound or contrast CT/MRI; biopsy if need confirmation
Tx: Partial hepatectomy (if single lesion <5cm or 3 lesions <3cm)
Tx: Sorafenib for advanced metastatic disease
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Albright Hereditary Osteodystrophy
Albright Hereditary Osteodystrophy (Pseudohypoparathyroidism type 1A)
Pseudohypoparathyroidism type 1A
Unresponsiveness of kidney to PTH
hypocalcemia despite increased PTH levels
Characterized by shortened 4th/5th digits
Short stature
Autosomal dominant
Defect must be inherited from mother due to imprinting
Due to defective Gs protein α-subunit causing end-organ resistance to PTH (from mother)
Tx: Calcium and Vitamin D (calcitriol)
Pseudopseudohypoparathyroidism
Pseudopseudohypoparathyroidism
Physical exam features of Albright hereditary osteodystrophy but without end-organ PTH resistance
Occurs when defective Gs protein α-subunit is inherited from father
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Autosomal Dominant Polycystic Kidney Disease
Cause
PKD1 Mutation
Symptoms
Bilateral Enlarged Cystic Kidneys
Flank Pain
Hematuria
Hypertension
Associated With
Berry Aneurysms
Subarachnoid Hemorrhage
Mitral Valve Prolapse (MVP)
Hepatic Cyst
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SGLT-2 Inhibitors
-flozin
indicated for T2DM
inhibits Glucose Reabsorption in PCT
promote glucosuria
SFx: DKA, UTI, Yeast Infection (Candida), Hypotension
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