Random Facts from past Papers Flashcards
What is the inheritance pattern for all Multiple Endocrine Neoplasia (MEN) subgroups?
All are autosomal domiant
What gene is affected in MEN 1?
MEN1 gene on chromomsome 11
Leads to altered expression of Mening (usually TSG)
What malignancies are associated with MEN1?
MEN 1= 3 Ps
- Parathyroid (parathyroid adenoma)
- Pancreas (endocrine pancreatic tumours e.g. insulinoma, gastrinoma)
- Pituitary adenoma (most commonly prolactin)
What gene is affected in MEN 2?
Altered expression of the RET proto-oncogene → elevated tyrosine kinase activity
MEN 2 has 2 subtypes. Both share 2 of the 3 neoplasms, but one is different (they also have different body habitus)
What malignancies is MEN 2a associated with?
MEN 2a =2P and one M
For all MEN 2
- Medullary Thyroid carcinoma
- Phaeochromocytoma
For Men 2a
- above +
- Primary hyperparathyroisim
What malignancies are associated with MEN2b?
MEN 2b = 2M and one P
For all MEN 2
- Medullary Thyroid carcinoma
- Phaeochromocytoma
For MEN2b
- above +
- Multiple neurinomas (+ marfanoid features)
What sub-types of thyroid cancers are there?
- Derived from Thyrocytes
- Papillary
- Follicular
- Anaplasitc
- Derived from parafollicular C cells (calcitonin)
- Medullary
What is the most common sub-type of thyroid cancer?
What is the typical epidemiology and prognosis?
Papillary thyroid cancer
usually in women 20-40, associated with irradiation
Overall has excellent prognosis
What are the histological charactreistics of papillary thyroid cancer?
- Psamoma bodies
- empty appearing nuclei with central clearing (orphan annie eyes)
Where does papillary thyroid cancer usually metastasise?
Usually Lymph nodes + lungs (often presents as painless cervical lymphadenopathy)
What type of thyroid cancer is assocaited with MEN2?
What cells is it derived from?
Medullary thyroid cancer (5% of all thyroid cancers)
–> Derived from parafollicular C cells
20% of people with medullary thyroid cancer have MEN2
What tumour markers can be used in the diagnosis of medullary thyroid cancers?
- CEA
- calcitonin
What is the histological appearance of medullary thyroid cancer?
What other features might hint you towards a medullary thyroid cancer in an SBA?
Sheets of dark cells
With amyloid depositions (arrows pointing to fibrous bands ) with high vascularity within tumour
- Association with MEN2 (aka phaeos)
- high calcitonin
What is follicular thyroid cancer?
What is the epiodemiology?
Where does it usually metastasise?
What is the histopathological appearnace?
2nd most common thyroid cancer (10-20%), usually in 40-60 years and metastasises early
Usually metastasises into **Blood ** first+
Histology
- uniform cells forming small follicles and reminiscent of normal thyroid
What is the most agressive form of thyroid cancer?
What is the epidemiolgoy and histological appearance?
Anaplasic Thyroid cancer (only 1-2% of cases)
usually in elderly and prognosis <1 year
Histology: undifferentiated follicular, large pleomorphic giant cells, spindle cells
What is the most common type of pancreatic cancer?
What is the histological appearance
Ductal adenocarinoma (95%) with
- altered ductal structures
- cellular infiltration
Other subtypes (acinar adenocarcinoma, mucinous cystadenocarcinoma) are less common, but also exocrine
- There are also pancreatic endocrine tumours
What antiviral medicaiton is used for treatment of CMV?
Ganciclovir
What antiviral medication can be used in the managment of RSV?
Ribavirn
50 year old female presents to the ENT clinic following a recent admission of hypercalcaemia. Primary hyperparathyroidism secondary to adenoma is suspected.
What investigation is most useful in determining location and functional status of the adenomas?
Technetium 99 scan
What are the traunsfusion threshold for platelet transfusions?
<10 : Leukaemias
<20 : Sepsis
<50 : Surgery (prevent bleeding in surgery, might be higher depending on site)
<75: if big RBC transfusion to balance
NO transfusion if activel bledding or any cunsumption diorders (e.g. TTP; DIC; HIT)
What Breast cancer is also known as No specific type?
Invasice ductal carcinoma (NST) –> most common breast cancer
What breast cancer is associated with E cadherin loss?
Lobular carcinoma
Which common condition can be treated with drugs that target TNF, IL-17 and Il-12/23?
Psoriasis
What viral diseases do you use Ribavirin against?
RSV
Hepatitis C
What medication is used to treat Herpes infections (HSV, CMV, EBV) in immunocompromised patients or in patients with CMV infection pre-transplant?
Foscarnet
Foscarnet is principally used for the treatment of ganciclovir-resistant cytomegalovirus (CMV) infections in patients with the acquired immunodeficiency syndrome (AIDS) or in transplant recipients.
What antiviral treatment must be administered with probenecid?
Cidofovir
–> off-label use of probenecid (usually gout medication) to limit nephrotoxic effects of cidofovir
What is an important differential to Multtiple myeloma when you see overproduction of IgM?
Waldenstrom’s macroglobulinaemia
A type of non-Hodgekin lymphoma leading to monoconal overproduction of IgM
Often presents due to hyperviscocoity (IgM overproduction) + fatigue
What is the most common cause of hypercalcaemia in the community?
hyperparathyroidism –> primary? (parathyroid adenoma)
Venetoclax is a BCL2 inhibitor used in CLL. What cellular process does it affect?
Apoptosis
What is the most common cause of contrictive pericarditis in developing countries?
??????
TB?
Wha is the most common cause of nephrotic syndrome in aduls that is glomerular pathology?
Focal segmental glomerularnephritis
What is the most common cause of portal vein thrombosis?
Hypercoaguability –> Polycythemia vera
What is the microscopic appearnace of the pathogen causing hamoptysis in eldery patients and alcoholics?
-ve rod, enterobacter
Klebsiella
What is the commonest cause of myocarditis?
Viral: expecially coxacie B
Most commonly implicated: coxsackie B1-B5 (picornavirus), parvovirus B19, human herpesvirus 6 (HHV-6), adenovirus, HCV, HIV
Which virus characteristically causes encephalitis involving the temporal lobes?
Herpes simplex virus
Recall the Zones of the adrenal and in which zones
- Glococorticoids
- Mineralcorticoids
- Androgens
- Epineohrine are produced
Has Medulla (inside) and Cortex (outside)
Medulla = Cetecholamines (Epinephrine)
Then from inside out:
- Zone reticularis (androgens)
- Zona fasciculata: glucocorticoids (cortisone)
- Zone glomerulosa: Aldosterone (Mineralcorticoids)
What condition occurs in both MEN1 and MEN2a?
Hyperparathyroidism
What active enzyme in sarcoidosis patients causes hypercalcaemia
1-alpha hydroxylase
ctivated pulmonary alveolar macrophages → ↑ 1-alpha hydroxylase expression and activity → ↑ 1,25-dihydroxyvitamin D (calcitriol) → hypervitaminosis D → hyperphosphatemia, hypercalcemia, and, possibly, renal failure [6]
Deficiency of which plasma protein occurs in patients with liver disease and a movement disorder?
ceruloplasmin
–> Wilson’s disease
Doctors should measure the level/activity of which enzyme before prescribing azathioprine?
TPMT
Thiopurine methyltransferas
enzmyme needed in metabolism of azathioprine. If congenital deficiency of TPMT –> built up of toxic metabolit of azathioprine leading to severe BM failure
What gene is defected in X-linked severe combined immune deficiency?
What does it result in?
mutations in the gene encoding the common gamma chain → defective IL-2R gamma chain receptor linked to JAK3 (most common SCID mutation)
Mutation of CD40 ligand is associated with which form of primary immunodeficiency?
Hyper-IgM syndrome
A group of syndromes characterized by impaired interaction between Th cells and B cells that results in a B cell class-switching defect
CD40 ligand deficiency is most common form
Which class of antibiotics has concentration dependent killing (i.e. the goal of therapy is to maximise peak > MIC)
Aminoglycosides
What is the treatment of samonella typhi?
Cefotaxime (or ceftriaxone)
azithromycin may be an alternative in mild or moderate disease caused by multiple-antibacterial-resistant organisms.
Alternative if micro-organism sensitive, ciprofloxacin
What antifungalis used in the treatment of cryptococcal meningitis + invasive fungal infection?
Amphotericin B
What type of necrosis is seen after an acute Myocaridal infarction?
The pathological hallmark of acute MI is coagulative necrosis of the myocardiu