Describe the findings of each in 11β-hydroxylase deficiency: Mineralocorticoids, Cortisol, Sex hormones, Blood pressure, K+, Renin activity
Sex hormones: ⇧
Blood pressure: ⇧
Renin activity: ⇩
What is the function of 11β-hydroxylase?
Converts 11-deoxycorticosterone to Corticosterone
Describe the presentation/ findings of 11β-hydroxylase deficiency.
Hypertension(due to ⇧11-deoxycorticosterone)
Fluid & Salt retention
What bone is most common of the carpal bone fractures?
What frequently causes a scaphoid fractures?
They frequently result from falls onto an outstretched arm that cause axial compression or wrist hyperextension.
Describe the presentation of a scaphoid fracture.
Patients present with persistent wrist pain and tenderness in the anatomical snuffbox following a fall on outstretched hand
Scaphoid fractures are at risk for?
Avascular necrosis - retrograde blood supply
Describe the blood supply of the scaphoid bone.
Supplied by the dorsal scaphoid branch of the radial artery.
Blood supply to the proximal pole proceeds in a retrograde manner and can be easily interrupted by a fracture.
What is the pathology seen below?
Label the diagram below.
What other diseases/ conditions is carpal syndrome associated with?
Repetitive wrist movements
A fall on an outstretched hand that damages the hook of hamate can cause?
Ulnar nerve injury
Dislocation of the lunate may cause?
Acute carpal tunnel syndrome
What congential cardiac defect is Marfan syndrome associated with?
Mitral valve prolapse
Cystic medial necrosis of the aorta ⇨ dissecting aortic aneurysms, Aortic regurgitation
What is the most important prognostic indicator for patients with malignant melanoma?
Measurement of the depth of invasion (Breslow thickness)
Melanomas in the vertical growth phase indicate?
Increased risk of metastasis
What is the pathology seen below?
Porcelain gallbladderCalcified gallbladder
What is the pathology seen below?
Porcelain galbladderCalcified gallbladder
What is a Porcelain gallbladder? What causes it? How do we treat?
Calcified gallbladder due to chronic cholecystitis(inflammation)
Treatment: Cholecystectomy due to high rates of GALLBLADDER CARCINOMA
Rim of calcium deposits that outline the gallbladder is characteristic of which pathology?
What is the difference between Salk and Sabin polio vaccine?
Salk- Killed (INJECTED)
Sabin- Live attenuated (ORAL)
One patient is given a live attenuated polio vaccine and another a killed polio vaccine. The level of which poliovirus antibodies will differ the most between the patients?
Oropharyngeal/ Intestinal mucosal IgA would increase more with the live attenuated - Sabinwhich is ORAL vs the killed - Salk which is injected.
High peak in the gamma-globulin region on serum electrophoresis represents?
M spike- consists of an overproduced monoclonal immunoglobulin
Describe an M spike. Which diseases is it characteristic of?
M spike is an M proteinconsisting of an overproduced monoclonal immunoglobulun.
Serum protein electrophoresis shows a high peak in gamma-globulin region.
The absolute risk reduction of drug A versus standard therapy was 4%. The incidence of recurrent PE in the standard therapy group was 6%. There were 24 patients who developed recurrent PE in the drug A group. How many total subjects were there in the drug A group?
This drug is a fusion protein that works as a receptor for tumor necrosis factor α.
What causes the familial/ heritable form of Pulmonary arterial hypertension?
Mutation in BMPR2 gene which normally inhibits vascular smooth muscle proliferation.
What is the function of the BMPR2 gene?
Inhibits vascular smooth muscle proliferation.
Describe the pathogenesis of idiopathic pulmonary artery hypertension.
1. Muscularization of small arteries
2. Medial hypertrophy and intimal hyperplasia
3. Intimal fibrosis(onion skinning)
4. Formation of capillary turfts (plexiform lesion)
What is the function of pyruvate kinase?
Converts phosphoenolpyruvate to Pyruvate
One molecule of ATP is generated.
This enzyme converts phosphoenolpyruvate to pyruvate.
What stimulates the activity of pyruvate kinase?
Fructose 1, 6-bisphosphate
How does Pyruvate kinase deficiency cause hemolytic anemia?
Due to failure or glycolysis(pyruvate kinase stimulates glycolysis) and failure to regenerate sufficient ATP to maintain erythrocyte structure.
This type of anemia results from failure to generate sufficient ATP to maintain erythrocyte structure.
Pyruvate kinase deficiency
These drugs are analogues of pyrophosphate.
Bisphosphonates- Alendronate and other -dronates
What toxicity is associated with the use of Bisphosphonates? How can we avoid this?
Can cause CORROSIVE ESOPHAGITIS
Patients are advised to take with water and remain upright for 30 mins
Patient develops right-sided weakness and numbness, with his arm more severely affected than his leg. He understands everything that is said to him, but cannot speak. Which artery has been occluded?
LEFT Middle meningeal artery
Describe the symptoms of an occlusion to the Middle cerebral artery.
Contralateral loss of sensation/ paralysis of UPPER LIMB and FACE
APHASIA- loss of ability to understand/ express speech
A lesion to broca's area manifests as? What artery supplies this area?
Inability to speak or write, but preserved comprehension of the spoken and written word
Where is Broca's area located?
Inferior frontal gyrus in the dominant (left) hemisphere
This structure is located in the inferior frontal gyrus in the dominant (left) hemisphere.
Loss of ability to speak or write but preserved comprehension of the spoken and written word is associated with a lesion to?
Brocas area- inferior frontal gyrus in the dominant (left) hemisphere
A lesion of the left anterior cerebral artery leads to?
Right sided loss of sensory and motor functions in the lower limb
What causes hyperestrinism in alcoholic cirrhosis and what are the manifestations of that?
Hyperestrinism in alcoholic cirrhosis is caused by:
1. ⇩catabolism of estrogens(by nonfunctional liver)
2. ⇧sex hormone binding globulin which binds more testosterone than estrogen
This leads to:
Decreased body hair
What are the manifestations of hyperestrinism in alcoholic cirrhosis?
2. Testicular atrophy
3. Decreased body hair
4. Spider angiomata
What causes pedal/ ankle edema in cirrhosis?
Cirrhotic liver produces insufficient amounts of proteins, such as albumin.
Hypoalbuminema→ ⇩Intravascular oncotic pressure → Fluid moves into the extravascular space → Pitting edema
What is Fetor hepaticus and what is it characteristic of?
Malodorous breath- Alcoholic cirrhosis
What causes Fector hepaticus?
Breath smells musty
Caused by Hyperammonemia
What are the effects of portal hypertension in cirrhosis?
1. Esophageal varices hematemesis
2. Melena(due to peptic ulcer)
4. Caput medusae, ascites
5. Portal hypertensive gastropathy
6. Anorectal varices
What is Caput medusae?
Distended paraumbilical veins
Ppt presents with shortness of breath. Analysis of expiratory gases reveals:
Tracheal pO2: 150 mmHg
Alveolar pO2: 145 mmHg
Alveolar pCO2: 5 mm Hg
What causes the result of this patients pulmonary gas analysis?
Normal Tracheal pO2 = 150 mmHg
Normal alveolar pO2 = 104 mmHg
Normal alveolar pCO2 = 40 mmHg
Patient is suffering frrom a very poor alveolar perfusion evidenced by failure of the alveolar gas to reach its normal equilibrium point.
What is diffusion-limited gas exchange?
Gas does not equilibiriate by the time blood reaches the end of the capillary
In which situation can O2 equilibrium become diffusion-limited?
In states of ⇧pulmonary flow (e.g. exercise)
What is the clinical use of thiazide diuretics?
Hypercalciuria ⇨ Ca2+ oxalate kidney stones
How to we treat calcium kidney stones?
2. THIAZIDE diuretics
Which diuretic increases Ca2+ excretion?
Loops lose calcium
Furosemide, Bumetanide, Toresemide
What are the effects of loop diuretics on Ca+?
⇧Serum Ca2+ excretion
What is restless leg syndrome and how do we treat?
Uncomfortable urge to move the legs due to an uncomfortable sensation in the legs.
It is worst at rest or when falling alseep.
Tx: Dopamine agonsit - ROPINIROLE, PRAMIPEXOLE
In this disorder, there is an uncomfortable urge to move the legs due to an uncomfortable sensation in the legs. It occurs especially when one is about to go to sleep. How do we treat?
Restless leg syndrome
Dopamine agonist- Ropinirole, pramipexole
What is the MOA and use of Ropinirole?
MOA: Dopamine agonist(non-egot (preferred))
What is the MOA and use of Pramipexole?
MOA: Dopamine agonsit(non-ergot (preferred))
What are the dopamine agonists?
Pramipexole, Ropinirole- non-ergot
What is cataplexy and how can we treat?
A feature of Narcolepsy
Characterized by sudden loss of muscle tone, often triggered by strong emotional stimulus such as laughter.