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 (ātennis racketsā 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 1Ā° 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 despiteĀ 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 Ī±-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 Ī±-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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