Pathology deck Flashcards
(260 cards)
Define amyloidosis
Improper collection of Beta pleated sheets of amyloid protein in the extracellular matrix. It is a life-threatening condition as humans lack the enzyme to break down this protein structure
How do you classify amyloidosis
Al - congenital and primary
This is caused by clonal proliferation of plasma cells producing this amyloid immunoglobulin
AA - secondary to chronic inflammation (in RA, IBD, TB, bronchiectasis, renal cell carcinoma)
Inflammation forces macrophages to secrete interleukin that then stimulates hepatocytes to produce amyloid protein A.
ATTR - autosomal dominant and most commonly affected protein is tranthyretin
What are the clinical features of AL amyloidosis
Kidneys: Proteinuria and nephrotic syndrome
Cardiac: Restrictive cardiac disease and arrythmias
Nervous system: Peripheral and central symptoms
GI: Malabsorbtion, bleeding, obstruction and perforation
Vascular: purpura
Clinical features of AA amyloid
Hepato and splenomegaly and proteinuria
How is amyloid diagnosed
Tissue biopsy
Subcut fat or rectal tissue is regarded as the best sample
Isotope scanning can also show hot spots of amyloid deposits
How is amyloidosis managed
AA can be controlled if underlying inflammation is controlled
AL needs chemo and eradicating abnormal plasma cells
Stem cellls
Supportive tx
It has poor survivial and the median survivial rate is 1-2 years
Which thyroid cancer causes amyloid deposition?
Meduallry as amyloid is composed of calcitonin
Where can isolated amyloid deposits be found
Larynx
Anwhere in the urinary tract
Aorta
Pituitary
Amyloid microsopy
Congo red stain shows apple gree birefingence under polarized light
What is the pathogenesis of calcification of the aortic valve in aortic stenosis
Lipid accumulation, inflammation and clacification
What are the symptoms of aortic stenosis
Syncope, angina and dyspnoea. The symptoms may not present until they are life-threatening and hence patients can also present with sudden cardiac death
How would you investigate for aortic stenosis
ECG, ECHO, CT angio, cardiac CT/MRI and exercise tolerance test
Aortic sclerosis vs aortic stenosis
Sclerosis is calcification of the valve without a significant gradient across the valve, this may progress into aortic stenosis
Do patients that undergo routine aortic valve replacement need prophylactic abx
No, I would consult the nice guidelines but the only exception to this would be if a patient was having a contaminated surgery, then abx to cover for those bacteria would be considered
What are organisms that commonly cause IE
HACEK organisms Strep Viridans and groups B and D, staph auerus (most common), Candida, enterococci, Pseudomonas aueriginosa
What is the definition of AKI
Abrupt reduction in GF resulting in the reduction in the ability of the kidneys to excrete nitrogenous waste. This is reflected by a rise in serum creatinine and urea
Biochemical cutoff is an increase in serum creatinine of 1.5-2x
Over what timescale does AKI normally develop
48 hours
What is a normal U and Cr
U 2.5-7.8 mmol/L
Cr 60-110 mcrmmol/L for men and 45-90mcrmmol/L for females
Emergency management of pulmonary oedema
ABCDE approach
Stop IV fluids
Sit the patient up
Oxygen
furosemide
Consider HF
CXR
What is normal urine output
0.5ml/kg/hr for adults and 1 for child
Define oliguria
<0.5ml/kg over a 6 hour period or <400mls over a 24 hour period
What is the most common cause of anuria in a surgical patient
Blocked catheter
In which decade is appendicitis most common
Second decade of life 10-20, slightly more common in males 1.4:1
In what positions can appendix be found
Retrocaecal 75%
Pre and post ileal 5%
Pelvic 20%