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
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.
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
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
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
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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