general path Flashcards
vaccinations in pregnancy
Vaccinations:
Flu vaccine straightaway, pertussis vaccine book for @16 weeks.
most common cause of lobar pnuemonia
strep pneumonia
which infections are checking in routine antenatal screening ?
hepatitis B virus, HIV, syphilis
which psych drug can cause hyponatraemia ?
sertraline
baby can be born with these if mothers gets this in pregnancy:
sensorineural deafness
congenital cataracts
congenital heart disease (e.g. patent ductus arteriosus)
growth retardation
hepatosplenomegaly
purpuric skin lesions
‘salt and pepper’ chorioretinitis
microphthalmia
cerebral palsy
congenital rubella
pituitary adenoma with slightly raised prolactin ?
A non-functioning adenoma may crush the stalk, leading to increase prolactin levels (lower dopamine inhibition as reduced blood flow). However, the increased prolactin will be relatively small (but will be massively raised in prolactinoma)
which hepattitis virus is very dangerous for women ?
hepatitis E
what is the most specific antibody for Rheumatoid arthritis ?
anti cyclic citrullinated peptide
what do you see on biopsy in Hirschsprung’s disease –
Absence of ganglion cells in myenteric plexus (80% males).
hypertrophied nerve fibres
A person has MEN2, underwent thyroidectomy for medullary thyroid cancer, develops palpitations and hypertension. Dx?
Phaeochromocytoma
different congenital adrenal hyperplasia and what you get in them?
21-hydroxylase deficiency (90%)
impairs the conversion of 17-hydroxyprogesterone to 11-deoxycortisol, leading to cortisol deficiency and excess androgen production. you get salt wasting crisis
11-beta hydroxylase deficiency (5%)
results in hypertension due to excess deoxycorticosterone
17-hydroxylase deficiency (very rare)
leads to mineralocorticoid excess with low androgen and estrogen levels
- A child with sickle cell comes in SOB, listless. Lo rbc, lo reticulocytes
parvovirus B19 infection causing aplastic crisis
which resp infection in CF and why ? and how to manage ?
immunocompromsied
Pseudomonas aeruginosa. Assoc with worse prognosis, treat with ceftazidine
what is Rituximab and when do you use it ?
Anti-CD20, depletes mature B cells (not plasma cells)
Lymphoma, rheumatoid arthritis, SLE
first step in clincial assessment of someone with low sodium
clinical assessment of volume statuts
most frequent route of pathogen entry into CNS ?
haematagenous spread
what are the Myeloproliferative Disorders
A group of conditions characterized by clonal proliferation of one or more haemopoietic component i.e. increased production of mature cells.
“Philadelphia Chromosome positive”
Chronic Myeloid Leukaemia (CML)
“Philadelphia Chromosome negative”
- Polycythaemia vera (PV)
- Myelofibrosis (MF)
- Essential thrombocytosis (ET)
why do you get tetany when you hyperventilate ?
Hyperventilation due to anxiety can result in tetany
Respiratory alkalosis happens when you’re breathing too fast or too deeply, causing your lungs to get rid of too much carbon dioxide. This causes the carbon dioxide levels in your blood to decrease, and your blood then becomes alkaline.
put these in order of broad spectrum
pip taz
benzyl penicillin
meropenem
ceftriaxone
amoxicclin
benzyle pen
amoxicilin
ceftriaxone
pip taz
meropenem
what happens to platelets in pregancy ?
dilutional thrombocytopaenia
peripheral pancytopenia + qualitative abnormalities of cell maturation
myelodysplastic syndrome
outline myelodysplastic syndrome
Heterogeneous group of progressive disorders featuring ineffective proliferation and differentiation of abnormally maturing myeloid stem cells.
* Characterised by: peripheral cytopenia; qualitative abnormalities of cell maturation; risk of AML transformation
* Typically seen in the elderly; symptoms usually develop over weeks/months (incidental)
* By definition all patients have <20% blasts (>20% blasts = acute leukaemia)
Clinical Features
* BM failure and cytopenias infection, bleeding, fatigue
* Hypercellular BM
* Defective cells:
o RBCs e.g. ring sideroblasts (abn nucleated blast surrounded by iron granule ring)
o WBCs – hypogranulation, Pseudo-Pelger-huet anomaly (hyposegmented neutro)
o Platelets – micromegakaryocytes, hypolobated nuclei
tx for beta thalassaemia ? and what do you need to be careful of ?
- Minor and some intermedia forms may not need regular treatment
- Blood transfusions with iron chelation (desferrioxamine) to stop iron overload, plus folic acid
- Regular screening for iron overload in heart and liver
outline hypoglycaemia management for all cases
if they’re conscious
1) Glucotabs – short acting simple CHO
2) Sandwich – long acting complex CHO
if not conscipus and you have IV access
- Glucose 10% 100ml IV over 10 mins
if unconscious and no IV access
IM glucagon
name the 3 eznymes deficient in congenital adrenal hyperplasia
21-hydroxylase deficiency (90%)
impairs the conversion of 17-hydroxyprogesterone to 11-deoxycortisol, leading to cortisol deficiency and excess androgen production
11-beta hydroxylase deficiency (5%)
results in hypertension due to excess deoxycorticosterone
17-hydroxylase deficiency (very rare)
leads to mineralocorticoid excess with low androgen and estrogen levels
what is HHS and who gets it ?
Hyperosmolar hyperglycaemic state (HHS) is a medical emergency that can be difficult to manage and has a significant associated mortality (up to 20%). Hyperglycaemia results in osmotic diuresis, severe dehydration, and electrolyte deficiencies. HHS typically presents in the elderly with type 2 diabetes mellitus (T2DM).
Some people who don’t realise they have type 2 diabetes don’t get diagnosed until they are very unwell with HHS
Hyper Igm due to a mutation in what gene ?
CD40 ligand gene
which cytokine for eosinophils ?
IL-5
Investigations: confirm a mutation of the MEFV gene encoding pyrin. whats going on… what drug …. what do you prevent ?
Familial Meditaranean Fever
Prevent amyloidosis
tx = colchicine
A 6 month old baby has presented with recurrent ix is being investigated for immunodeficiency. Analysis of lymphocyte subsets shows presence of B cells and CD8+ T cells but very low numbers of CD4+ T cells.
presence of IgM but low levels of IgG
Bare lymphocyte syndrome
Defect in one of the regulatory proteins involved in Class II gene expression
* Regulatory factor X or Class II transactivator
Absent expression of MHC Class II molecules
Profound deficiency of CD4+ cells
* Usually have normal number of CD8+ cells
* Normal number of B cells
* Failure to make IgG or IgA antibody (no class switching)
which cytokine in ankylosing spondyltitis and what is teh name of the drug?
Secukinumab
anti-IL-17A
psoriasis, psoriatic arthritis, ankylosing spondylitis
A 32 year old man presents with fever, abdominal pain and constipation. He has recently returned from Pakistan. He undertook no vaccinations prior to travel. Blood cultures taken on admission have grown non-lactose fermenting Gram negative bacilli in both bottles. What is the most likely causative organism? + mx?
Salmonella Typhi + Paratyphi
(Typhoid- see fever in returning traveller)
Enteric fever: constipation, fever, rose spots, splenomegaly
mx = IV ceftriaxone then PO azithromycin
HIV patients develops fever, confusion, headache - whats going on
immunosupression –> toxoplasmosis gondii causing encephalitis sx
PML/RARA fusion gene
Acute promyelocytic leukaemia (APML)
15;17– prone to DIC & bleeding
Chromosomal translocation involving c-myc proto oncogene
birkitt’s leukaemia
presentation of nephortic with myeloma ?
myeloma kidney (cast nephropathy)
Hb fall in patient with CLL (spherocytes )
Associated with autoimmunity (Evan’s Syndrome) – AIHA, ITP
mother with ITP - how can you check baby?
check cord FBC
what monitoring for doAC
none
Renal transplant recipients on long term immunosuppression have an increased risk of which lymphoid cancer?
EBV associated Diffuse large B cell lymphoma (DLBCL)
Children with Down syndrome have an increased incidence of which type of blood cancer?
ALL
outline Antiphospholipid syndrome
Antiphospholipid syndrome is an acquired disorder characterised by a predisposition to both venous and arterial thromboses, recurrent fetal loss and thrombocytopenia. It may occur as a primary disorder or secondary to other conditions, most commonly systemic lupus erythematosus (SLE). Around 30% of patients with SLE have positive antiphospholipid antibodies.
A key point for the exam is to appreciate that antiphospholipid syndrome causes a paradoxical rise in the APTT. This is due to an ex-vivo reaction of the lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade.
Associations other than SLE
other autoimmune disorders
lymphoproliferative disorders
phenothiazines (rare)
Features
venous/arterial thrombosis
recurrent miscarriages
livedo reticularis
other features: pre-eclampsia, pulmonary hypertension
Investigations
antibodies
anticardiolipin antibodies
anti-beta2 glycoprotein I (anti-beta2GPI) antibodies
lupus anticoagulant
thrombocytopenia
prolonged APTT
Management - based on EULAR guidelines
primary thromboprophylaxis
low-dose aspirin
secondary thromboprophylaxis
initial venous thromboembolic events: lifelong warfarin with a target INR of 2-3
recurrent venous thromboembolic events: lifelong warfarin; if occurred whilst taking warfarin then consider adding low-dose aspirin, increase target INR to 3-4
arterial thrombosis should be treated with lifelong warfarin with target INR 2-3
Which of these tumours is most likely to be associated with Pseudomyxoma peritonei?
Mucinous tumour of the ovary
A 21 year old Syrian woman who has recently moved to the UK from a refugee camp attends a haematology clinic. She has Beta Thalassaemia major and for the last six years has received regular blood transfusions as treatment for anaemia. On examination she has signs of congestive cardiac failure and gonadal failure
iron overload (due to blood transfusions)
What species of Coagulase Negative Staphylococcus is a common cause of lower urinary tract infection in young women?
- Staphylococcus saprophyticus
What double stranded DNA virus is associated with nephritis in renal transplant recipients on immunosuppresive therapy?
BK polyomovirus
Which vaccine preventable disease presents initially with mild fever, swollen neck glands, anorexia, malaise. and cough. After 2-3 days a membrane of dead cells forms in the throat, tonsils, larynx or nose which may narrow or occlude the airway leading to respiratory distress?
diphtheria
Diphtheria is caused by the Gram positive bacterium Corynebacterium diphtheriae
Possible presentations
recent visitors to Eastern Europe/Russia/Asia
sore throat with a ‘diphtheric membrane’ - grey, pseudomembrane on the posterior pharyngeal wall
bulky cervical lymphadenopathy
may result in a ‘bull neck’ appearanace
neuritis e.g. cranial nerves
heart block
Investigations
culture of throat swab: uses tellurite agar or Loeffler’s media
Management
intramuscular penicillin
diphtheria antitoxin
Which of these tumours is most likely to be associated with Pseudomyxoma peritonei
mucinous tumour of the ovary
complete mole vs partial mole pregancy
complete = diploid
partial = triploid
The presentation of a partial hydatiform mole is usually less dramatic than that of a complete mole. These patients typically present as described before with symptoms similar to a threatened or spontaneous abortion, including vaginal bleeding. Since partial hydatiform moles have fetal tissue, on examination, these patients may have fetal heart tones evident on Doppler.
On physical exam, in more than 50% of cases, uterine size discrepancy and date discrepancy usually takes place.
In a complete mole, the uterus is usually larger than the expected gestational date of the pregnancy, whereas, in partial moles, the uterus can be smaller than the suggested date.
what is this descirbing
cytoplasmic hyaline inclusions of hepatocytes
Pre-keratin intermediate filaments
Mallory bodies (MBs), also known as Mallory-Denk bodies (MDBs), are cytoplasmic hyaline inclusions of hepatocytes, once thought to be specific for alcohol-related steatohepatitis (ASH)
A 42 year old male cigarette smoker presents with urinary frequency and haematuria. Cystoscopy finds an ‘exophytic fronded lesion’ which is biopsied. Histologic examination shows fibrovascular cores covered by atypical cells
papillary urothelial carcinoma
what molecule transports cholesterol to the liver?
The main lipoprotein involved in this process is the HDL-c
what is caused by niacin deficiency ?
pellagra
polymyositis/dermatomyositis what enzyme would be raised ?
Creatine kinase is the muscle enzyme most widely used to diagnose polymyositis/dermatomyositis and to follow therapeutic response
what are the hormones released from the hypothalamus
The thyrotropin-releasing hormone (TRH), gonadotropin-releasing hormone (GnRH), growth hormone-releasing hormone (GHRH), corticotropin-releasing hormone (CRH), somatostatin, and dopamine are released from the hypothalamus into the blood and travel to the anterior pituitary.
Multiple Endocrine Neoplasia TYPE 1 AND TYPE 2 - FEATURES
- MEN 1= ‘PPP’ - Parathyroid hyperplasia/adenoma, Pancreatic endocrine tumour (often phaeochromocytoma), Pituitary adenoma.
- MEN 2A- Parathyroid, Thyroid, Phaeochromocytoma
- MEN 2B- Medullary Thyroid, Phaeochromocytoma, Acoustic Neuroma. Marfanoid phenotype
Lymphadenopathy and hepatosplenomegaly in immune deficiency question - what is it ?
chronic granulomatous disease
- Very low or absent T cell and NK cell numbers
- Normal or increased B cell numbers
in immune deficiencies what is this ?
X linked SCID
A 29 year old female has experienced three second trimester miscarriages. She has a mild livedo. reticularis rash affecting her forearms and lower legs. Which auto-antibody is most likely to be present?
anti cardiolipin
Anti phospholipid syndrome (APS) also known as Hughes syndrome presents with diverse clinical symptoms including thrombosis (arterial and venous) and recurrent miscarriage. Anti-cardiolipin is one of the many antibodies to a variety of phospholipid molecules in APS
anti virals for influenza
- Oral Oseltamivir (Tamiflu)
- Inhaled Zanamivir (Relenza – used in patients with underlying respiratory disease)
What is the correct pathological term for a discontinuation in the epithelial surface?
ulcer
What is the technical term for a connection between two epithelial surfaces, as occurs in Crohns disease where for example there might be a connection between the bladder and the bowel?
fissure
A 46-year old Japanese female is found to have bilateral ovarian masses on imaging of the abdomen and had both ovaries removed. Histology revealed malignant signet ring cells containing mucin in both ovaries. What is the diagnosis?
Krukenberg tumour. Signet cells are present in gastric carcinoma. This is a gastric carcinoma that has metastasised to both ovaries. If both ovaries are affected, a primary elsewhere spreading to both ovaries is more likely.
Gastric carcinoma is far more prevalent in Japan that anywhere else
A 50 year-old female was found to have an ovarian mass. On its removal, histology revealed several cell types, including thyroid follicles, hair and teeth. What is the likely diagnosis?
Correct answer: Dermoid cyst or Cystic Teratoma
