Integrated + HCOLL Flashcards

Question

Iron deficiency anaemia - causes, investigations, management

Answer 1

Diet, absorption (coeliac, PPIs), pregnancy, blood loss (menstruation, GI*) FBC ↓ ferritin, ↓ transferrin saturation, ↑ TIBC Blood film; anisopoikilocytosis (red blood cells of different sizes and shapes) , target cells, 'pencil' poikilocytes If no clear cause -> colonoscopy and OGD Oral iron supplements Best taken 1 hour before food with orange juice (vit C) ADR: N+V, constipation, diarrhoea, dark stools

Answer 2

Intrinsic factor antibodies -> B12 deficiency -> macrocytic anaemia, peripheral neuropathy, subacute degeneration of the spinal cord; Dorsal columns: impaired proprioception & vibrioception Lat. corticospinal tract: UMN signs in legs; brisk knee, absent ankle reflexes and upgoing plantars Spinocerebellar: positive rombergs sign, ataxia Management: No neuro signs; 3 injections/week for 2 weeks then 2 monthly (for life if pernicious is the cause) Neuro signs; injections every other day until improvement Oral cyanocobalamin can be used for maintenance IF Folate deficient too, treat B12 first to avoid SCDSC ^ Increased gastric cancer risk

Answer 3

↑ ferritin, ↓ transferrin saturation, ↓ TIBC

Answer 4

Anaemia, splenomegaly, jaundice Normocytic, schistocytes, (positive coombs in AIHA) Hereditary spherocytosis G6PD deficiency Thalassaemia Sickle cell Autoimmune microangiopathic haemolytic anaemia (MAHA): TTP/HUS, DIC, malignancy, pre-eclampsia prosthetic heart valves

Answer 5

Autosomal dominant Fragile sphere shaped RBCs, which are destroyed in the spleen -> FTT, jaundice, splenomegaly, aplastic crisis (from parvovirus infection), gallstones ↑ MCHC, ↑ reticulocytes, spherocytes on film EMA binding test if inconclusive Folate replacement, transfusions, splenectomy cholecystectomy if recurrent gall stones

Answer 6

X linked recessive Increased RBC susceptibility to stress -> acute haemolytic episodes; neonatal jaundice, intravascular haemolysis, splenomegaly, gallstones heinz bodies on film; bite and blister cells G6PD enzyme assay 3 months after acute episode Some drugs/foods causing haemolysis: anti-malarials: primaquine ciprofloxacin sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas. infections broad beans

Answer 7

Autosomal recessive Thalassaemias are a group of genetic disorders characterised by a reduced production rate of either alpha or beta chains. microcytic anaemia, jaundice, gallstones, splenomegaly, FTT Alpha thalassaemia 2 genes on each C16: Carrier : 1-2 genes affected Hb H disease - 3 genes - microcytic anaemia, splenomegaly Bart's hydrops - 4 genes - in utero death Beta thalassaemia: minor/trait - 1 abnormal gene, 1 normal - asymptomatic microcytic anaemia Intermedia - 1 abnormal, 1 deleted or 2 abnormal - symptomatic microcytic anaemia Major - 2 deleted - severe anaemia, FTT. Increased HbA2 and HbF Bone marrow expands; increased fractures, frontal bossing, enlarged maxilla, flat nose Requires repeated transfusions -> iron overload; liver cirrhosis, hypogonadism, hypothyroid, HF, DM, osetoporosis Iron chelation therapy - desferrioxamine

Answer 8

autosomal recessive for beta globin gene -> fragile, sickle shaped RBCs Sx from 6 months old when HbA takes over Anaemia Sickle cell crisis: triggered by dehydration, infection, stress, cold Vaso-occlusive crisis; clogging of capillaries -> distal ischaemia, priapism (emergency), fever Splenic sequestration crisis; blocked blood flow through spleen -> acutely enlarged and painful spleen, pooling of blood (severe anaemia and hypovolaemic shock) Splenic infarction, hyposplenism Aplastic crises; caused by infection with parvovirus sudden fall in haemoglobin bone marrow suppression causes a reduced reticulocyte count Acute chest syndrome; vaso-occlusion within the pulmonary microvasculature → infarction in the lung parenchyma SOB, chest pain, cough, hypoxia Pulmonary infiltrates on cxr Crisis management: iv fluids, analgesia, +-O2, +-Abx +- Blood transfusion +- Exchange transfusion Long term: 5 yearly pneumococcal transfusions Hydroxyurea to stimulate HbF Crizanlizumab -I P-selectin on EC ; decreases crisis

Answer 9

reticulocytes, ↓ haptoglobin, ↑ LDH, ↑ indirect bilirubin, +ve coombs test Warm AIHA; extravascular (spleen), IgG mediated haemolysis at normal body temp. Idiopathic, SLE, lymphoma, CLL, methyldopa Cold AIHA; intravascular, IgM mediated haemolysis when RBCs agglutinate at cold temps <10 degrees. Lymphoma, EBV, mycoplasma Management; steroids, rituximab (--I B cells), splenectomy

Answer 10

transfusion reactions or haemolytic disease of the newborn

Answer 11

Acquired condition; loss of GPI protein which regulates complement .'. increased cascade -> haemolysis Haemoglobinuria in the morning, increased thrombosis, smooth muscle dystonia, aplastic anaemia Stem cell transplantation Eculizimab -I C5

Answer 12

Blood cells churned up due to obstruction in small vessels -> schistocytes Associated with HUS, DIC, TTP, SLE, cancer

Answer 13

Most common in <5 year olds, Increased in down's syndrome anaemia: lethargy and pallor neutropenia: frequent or severe infections thrombocytopenia: easy bruising, petechiae (bone marrow failure ; lymphocytosis (increased lymphocytes)) -> FBC within 48 hours +- blood film -> bone marrow biopsy

Answer 14

60yo< Usually B lymphocytes Features: often none: may be picked up by an incidental finding of lymphocytosis constitutional: anorexia, weight loss bleeding, infections lymphadenopathy more marked than chronic myeloid leukaemia lymphocytosis anaemia: thrombocytopenia: Blood film; smudge cells Hypogammaglobulinaemia Richter's transformation: CLL -> high grade B cell lymphoma (B symptoms, lymph nodes)

Answer 15

anaemia: lethargy and pallor functional neutropenia (Raised WCC) : frequent or severe infections thrombocytopenia: easy bruising, petechiae Auer rods on blood film

Answer 16

Philadelphia chromosome; abnormal 9->22 translocation of the BCR-ABL gene (tyrosine kinase) I--- Imatinib Often asymptomatic in chronic phase Accelerated phase; as per ALL etc. Blast phase: severe sx, pancytopaenia, death

Answer 17

Painless lymph nodes (alcohol induced pain) B symptoms (worse prognosis if so) RF: HIV. EBV, autoimmune Excisional biopsy : Reed Sternberg Cells; multinucleated with nucleoli 'Owl like' CT for staging (I- 1 node, II 1< group of nodes, III - nodes either side of diaphragm, IV widespread) FBC for ddx ESR, LDH for prognosis Types: Nodular sclerosing - most common Mixed Lymphocyte predominant - Best prognosis Lymphocyte depleted - Worst prognosis Chemotherapy

Answer 18

All types that aren't hodgkins RF: EBV, HIV, autoimmune (-> Burkitts lymphoma (t8:14 c-myc translocation) H.pylori (->MALT lymphoma (H. pylori eradication treats low grade) Pesticides, Hep B/C, trichloroethylene Excisional lymph node biopsy CT for staging (I- 1 node, II 1< group of nodes, III - nodes either side of diaphragm, IV widespread) FBC for ddx ESR, LDH for prognosis Management Chemo Rituximab (screen for Hep B before starting)

Answer 19

Plasma cell proliferation CRABBI presentation: ↑ Calcium; ↑ osteoclast activity from cytokines released by myeloma -> Bone pain, kidney stones, constipation, N+V, confusion Renal; Monoclonal Ab production by myeloma cells -> light chain deposition in the tubules -> dehydration polydipsia Anaemia; bone marrow overcrowding -> ↓ erythropoiesis Bleeding; as above, -> thrombocytopaenia Bone pain; lytic bone lesions from osteoclast activity -> pain and pathological fractures Infections; ↓ Immunoglobulins Hyperviscosity syndrome (paraproteins released by myeloma cells -> bleeding, visual changes, neuro, HF), carpal tunnel, neuropathy, amyloidosis) Investigations: FBC; ↓Hb, ↓WCC peripheral blood film: rouleaux formation U+E: renal failure bone profile: hypercalcaemia a rise in immunoglobulins may be implied by an elevated (total protein - albumin) Protein electrophoresis; raised concentrations of monoclonal IgA/IgG proteins will be present in the serum in the urine, they are known as Bence Jones proteins MRI for bone lesions Bone marrow biopsy for definitive diagnosis

Answer 20

Proliferation of a single cell line (in primary this is haematopoietic stem cells), due to Jak2 mutation. -> cytokine release -> fibroblast proliferation -> scarring in the bone marrow => ↓Hb, ↓WCC, ↓ Platelets -> extramedullary haematopoiesis; splenomegaly, hepatomegaly, spinal cord compression .'. anaemia e.g. fatigue (the most common presenting symptom), massive splenomegaly, weight loss, night sweats etc Blood film; tear drop poikilocytes, blasts, anisocytosis Diagnosis: Unobtainable bone marrow biopsy - 'dry tap'; Trephine biopsy needed Genetic analysis

Answer 21

myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume; Jak2 mutation ↑ Hb, ↑Haematocrit, ↑neutrophils, ↑plts Jak2 mutation for diagnosis Pruritus after hot bath, splenomegaly, HTN, hyperviscosity, low ESR, ruddy complexion, conjunctival plethora Management: Aspirin - to reduce thrombotic events Venesection - to control Hb levels +- chemo; hydroxyurea Can progress to acute leukaemia

Answer 22

myeloproliferative disorder - ↑ Platelets -> ↑ thrombosis, burning sensation in hands Management: Aspirin hydroxyurea (hydroxycarbamide) is widely used to reduce the platelet count Ruxolitinib (Jak2 inhibitor)

Answer 23

Type II hypersensitivity reaction; Ab against platelets .'. isolated Thrombocytopenia may follow an infection or vaccination Non blanching rash, bruising, bleeding (if <10x10^9 plts, spontaneous bleeding e.g. GI, intracranial) Self resolving in children Oral pred in adults IVIG/Plt infusion in active bleeding +- rituximab --I B cells (which produce the Ab)

Answer 24

ADAMTS13 mutation (usually --I Von Willebrand Factor (which decreased plt adhesion and clot formation) .'. -> tiny thrombi in small vessels, using up plts Thrombocytopaenia, purpura, tissue ischaemia; fever, neuro signs, haemoptysis

Answer 25

Ab against platelet factor 4; approx. 5-10 days after starting treatment -> activates clotting and thrombosis, but also breaks down plts. Swap to a direct thrombin inhibitor e.g. argatroban

Answer 26

Most common inherited bleeding disorder; Autosomal dominant Easy and heavy bleeding (defective primary haemostasis; initial plt plug slow to form. Fibrin clot slow to form) Prolonged bleeding time; ↑APTT if factor VIII also reduced (vWF is a carrier molecule for factor VIII) Management: txa for mild bleeding Desmopressin - stimulates vWF release VIII & vWF infusion - for severe bleeding

Answer 27

A - ↓ VIII B - ↓ IX X linked recessive .'. ↑ in males -> Spontaneous bleeding; neonatal intracranial bleeding, haematomas, cord bleeding haemarthrosis epistaxis, GI, haematuria etc. ↑ APTT, normal otherwise Clotting factor infusions; can become resistant (Ab)

Answer 28

Likes to clot Factor V leiden* - activated protein C resistance prothrombin mutation protein C or S deficiency Antithrombin III deficiency Antiphospholipid syndrome

Answer 29

lymphoplasmacytoid malignancy characterised by the secretion of a monoclonal IgM paraprotein Systemic upset: weight loss, lethargy hyperviscosity syndrome e.g. visual disturbance the pentameric configuration of IgM increases serum viscosity hepatosplenomegaly lymphadenopathy cryoglobulinaemia e.g. Raynaud's

Answer 30

Major bleeding e.g. intracranial: Stop warfarin i.v. Vitamin K 5mg Prothrombin complex INR 8.0< with minor bleeding: Stop warfarin i.v. Vitamin K 1-3mg (repeat if INR still raised after 24 hours) Restart warfarin when INR <5 INR 8.0<, no bleeding: Stop warfarin Oral Vitamin K 1-5mg (repeat if INR still raised after 24 hours) Restart warfarin when INR <5 INR 5-8, minor bleeding: Stop warfarin i.v. Vitamin K 1-3mg (repeat if INR still raised after 24 hours) Restart warfarin when INR <5 INR 5-8 no bleeding: Withhold 1-2 doses Reduce subsequent dose

Answer 31

Seronegative Inflammatory condition affecting the axial spine; sacroiliac & vertebral joint inflammation & fusion HLA-B27 Pain, stiffness in the lumbar region; worse in morning, improves with exercise Associated with Anterior uveitis, Aortic regurgitation, AV block, Apical lung fibrosis, Achilles tendonitis Reduced lateral flexion, reduced forward flexion (Schober's test) Investigations: XR of sacroiliac joints; sacroilitis, squaring of lumbar vertebrae, syndesmophytes, bamboo spine Management: NSAIDs and physio/exercise first line Anti-TNF e.g. infliximab +- intraarticular steroid injections

Answer 32

Symmetrical or assymetrical arthritis +- psoriatic plaques, nail pitting, onycholysis, dactylitis, enthesitis PEST screening tool; If joint pain, swelling, history of arthritis, nail pitting -> refer Investigations: X ray of hands; erosions and new bone formation. Pencil in cup, telescoping digit (arthritis mutilans - most severe; destruction of bone) Seronegative Raised CRP/ESR Management; specialist NSAIDs first line Methotrexate for peripheral disease Biologics

Answer 33

Acute monoarthritis, following an infection (most commonly dysentery or Chlamydia after 4 weeks) where an organism cannot be recovered from the joint Associated with bilateral conjunctivitis and urethritis (cant see, cant pee, cant climb a tree) +- anterior uveitis, balanitis, keratoderma blenorrhagica Rule out septic arthritis or gout (so no growth or crystals) Manage with NSAIDs; self resolving around 6 months

Answer 34

Acute swollen red joint with fever Staph aureus most common, in young adults who are sexually active, Neisseria gonorrhoeae Synovial fluid sampling Blood cultures IV Flucloxacillin; clindamycin if allergic

Answer 35

symmetrical polyarthritis; Pain, stiffness, swelling MCP, PIP, wrist, MCP, Can involve large joints Systemic sx; fatigue, wt less, flu-like illness, myalgia Hand signs: Z shaped thumb, swan neck (hyperextended PIP, Flexed DIP), boutonniere deformity (opposite), ulnar deviation Atlantoaxial subluxation - damage to odontoid peg of C2 -> spinal cord compression MRI before GA and intubation Extraarticular: respiratory: pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans, methotrexate pneumonitis, pleurisy ocular: keratoconjunctivitis sicca (most common), episcleritis, scleritis, corneal ulceration, keratitis, steroid-induced cataracts, chloroquine retinopathy osteoporosis ischaemic heart disease: RA carries a similar risk to type 2 diabetes mellitus increased risk of infections depression Less common Felty's syndrome (RA + splenomegaly + low white cell count) amyloidosis carpal tunnel Investigations: RF, anti-CCP ab, CRP, ESR X rays of hands and feet; loss of joint space, juxta-articular osteoporosis, swelling, periarticular erosions, subluxation Management: Initial DMARD monotherapy +- short term prednisolone - Methotrexate* ; FBC and LFT monitoring for myelosuppression - Sulfalazine - oligospermia, rash ILD - Leflunamide - ILD, HTN, HTN - Hydroxychloroquine (if mild or palindromic disease) - ophthalmology screen before and yearly for bull's eye retinopathy, Safe in pregnancy Biologics if 2 of the above tried Anti-TNF e.g. infliximab; screen for latent TB Anti CD20 e.g. rituximab FLARES: steroids

Answer 36

Management: Initial DMARD monotherapy +- short term prednisolone - Methotrexate* ; FBC and LFT monitoring for myelosuppression - Sulfalazine - oligospermia, rash ILD - Leflunamide - ILD, HTN, HTN - Hydroxychloroquine (if mild or palindromic disease) - ophthalmology screen before and yearly for bull's eye retinopathy, Safe in pregnancy Biologics if 2 of the above tried Anti-TNF e.g. infliximab; screen for latent TB Anti CD20 e.g. rituximab FLARES: steroids

Answer 37

Inflammatory arthritis associated with IBD When stable; polyarticular, symmetrical, smaller joints When flares in IBD: 'reactive like' oligoarthritis

Answer 38

Pain & stiffness in shoulders, pelvic girdle, and neck But no weakness +- systemic sx. Typically in old white pts. Worse in morning, inactivity and sleep Investigations Raised ESR/CRP Creatine kinase and EMG normal (raised in myositis) FBC, U+E, LFT, TFT Serum protein electrophoresis (myeloma) RF Management: 15mg OD prednisolone - sx should improve within a week Taper dose down over a long period of time (15mg until the symptoms are fully controlled, then 12.5mg for 3 weeks, then 10mg for 4-6 weeks, then Reducing by 1mg every 4-8 weeks) Don't STOP If on steroids for 3 weeks<, and abruptly stopped -> adrenal crisis Sick day rules - Double the dose Treatment card to be carried at all times Osteoporosis prevention - bisphosphonates PPI cover Associated with Giant Cell Arteritis; headache over temple, jaw claudication, anterior ischaemic optic neuropathy (vision loss with swollen pale disc and blurred margins) Raised ESR. Biopsy or USS Management: if there is no visual loss then high-dose prednisolone is used if there is evolving visual loss IV methylprednisolone is usually given prior to starting high-dose prednisolone + same day ophthalmology review.

Answer 39

CTD autoimmune condition; Type III hypersensitivity Presentation: Malar rash sparing nasolabial folds fatigue, fever, lymph discoid rash, reynauds, livedo reticularis, alopecia arthralgia, arthritis pleurisy, fibrosing alveolitis Complications: CVD, infection, anaemia, pericarditis, libmann-sacks endocarditis, miscarriage, VTE Lupus nephritis; diffuse proliferative glomerulonephritis most common and severe type Treat with steroids and mycophenolate (then monotherapy) Investigations: ANA 99% +ve (very sensitive) Anti-dsDNA (very specific) biopsy for AKI Monitoring disease activity; If ↑: Raised ESE, normal CRP Low complement levels Management: Hydroxychloroquine* NSAIDs, sun cream

Answer 40

photosensitive erythematous, raised rash, sometimes scaly +- alopecia scarring Small % -> SLE Management; topical steroids Avoid sun exposure

Answer 41

arthralgia, myalgia Malar rash and pulmonary involvement (e.g. pleurisy) are common ANA positive in 100%, dsDNA negative anti-histone antibodies are found in 80-90% Most common causes procainamide hydralazine Less common causes isoniazid minocycline phenytoin It usually resolves on stopping the drug.

Answer 42

Limited cutaneous systemic sclerosis: Scleroderma of face and distal limbs Calcinosis, Reynaud's, oEsophageal dismobility, Sclerodactyly, Telangiectasia Anti-centromere antibodies ANA positive in 90% Diffuse cutaneous systemic sclerosis: Scleroderma of trunk and proximal limbs CREST + HTN, CVD, ILD, Pulmonary hypertension, scleroderma renal crisis, primary biliary cirrhosis (resp. involvement most common cause of death) Anti-Scl70 antibodies ANA positive in 90% Management: DMARDs/biologics Nifedipine for reynaud's PPI, metoclopramide for reflux Captopril for renal involvement Bosentan for pulmonary hypertension or sildenafil

Answer 43

Autoimmune inflamm. disorder with proximal muscle weakness - associated with underlying malignancy Presentation: gradual onset, symmetrical proximal muscle weakness +- myalgia Dermatomyositis: as above + heliotrope rash on face, gottron's papules on knuckles, 'mechanic's hands' (extremely dry and scaly hands with linear 'cracks' on the palmar and lateral aspects of the fingers) Investigations: Raised CK anti - Jo 1 antibodies Muscle biopsy Management: high-dose corticosteroids tapered as symptoms improve azathioprine may be used as a steroid-sparing agent Screen for underlying malignancy

Answer 44

Autoimmune disorder against exocrine glands -> dry eyes, dry mouth, vaginal dryness Arthralgia, parotitis, myalgia Investigations: Schirmer test - for dry eyes ANA +ve Anti-Ro antibodies Anti-La antibodies RF +ve Management: artificial tears and saliva Pilocarpine - stimulates saliva production Safety net for lymphoid malignancies as great increased risk

Answer 45

Small vessel vasculitis causing: Renal failure due to immune complex glomerulonephritis Diffuse alveolar haemorrhage systemic sx pANCA

Answer 46

Small vessel vasculitis causing: upper respiratory tract: epistaxis, sinusitis, nasal crusting, saddle-shape nose lower respiratory tract: dyspnoea, haemoptysis rapidly progressive glomerulonephritis cANCA

Answer 47

Small vessel vasculitis causing: Late onset asthma Sinusitis, allergic rhinitis Increased eosiniphils pANCA

Answer 48

Medium vessel vasculitis - idiopathic or secondary to Hep B -> systemic sx renal impairment, HTN, tender skin nodules livedo reticularis

Answer 49

Large vessel vasculitis Inflammation of the aorta -> occlusion; unequal blood pressure in the upper limbs absent or weak peripheral pulses and claudication angiography to confirm

Answer 50

Steroids Cyclophosphamide if resistant

Answer 51

complex multisystem disorder associated with presumed autoimmune-mediated inflammation of the arteries and veins classic triad of symptoms are oral ulcers, genital ulcers and anterior uveitis

Answer 52

small-vessel vasculitis: IgG deposits on BM pulmonary haemorrhage rapidly progressive glomerulonephritis

Answer 53

Hot, painful, swollen red joint +- gout tophi 1st metatarsophalangeal joint* Uric acid levels >360 umol/L (repeat in 2 weeks if lower but still ? gout) joint aspiration: needle shaped negatively birefringent monosodium urate crystals under polarised light X ray: no loss of joint space, lytic lesions, 'punched out' juxtaarticular erosions RF: diuretics, CKD, blood cancers, cytotoxic drugs, Lesch-Nyhan syndrome Management: ACUTE: NSAIDs*, or colchicine (diarrhoea ADR) if CKD/contraindicated e.g. PUD/elderly Prophylaxis: Offered to all pts 2 weeks after 1st attack; Allopurinol +- initial colchicine cover

Answer 54

Hot swollen stiff painful knee typically Can be more chronic Joint aspiration: rhomboid shaped positively birefringent monosodium calcium pyrophosphate crystals under polarised light X ray: Chondrocalcinosis (Ca deposits show as a white line in the joint space) +- "LOSS" RF: Haemochromatosis, hyperparathyroidism low magnesium, low phosphate acromegaly, Wilson's disease Management: Sx control; NSAIDs Self resolving

Answer 55

↓Vit D -> ↓Ca, ↓Phosphate -> defective bone mineralisation => bone pain, tenderness, proximal myopathy (waddling gait) Causes: Malabsorption, lack of sunlight, diet CKD Metabolic disorders, liver disease Investigations: ↓Vit D ↓Ca ↓Phosphate (↑ in CKD due to decreased secretion) ↑ALP ↑PTH Looser's zones/pseudofractures on x ray Management: Vit D supplements (colecalciferol) +- calcium if still low (Calcitriol in CKD (active form))

Answer 56

Anti-phospholipid antibodies -> bind and inflame cell surfaces => venous & arterial thrombosis Pregnancy complications; PET, recurrent miscarriage (3<) Livedo reticularis Can be 1 or 2 to SLE Investigations: anticardiolipin antibodies anti-beta2 glycoprotein I (anti-beta2GPI) Ab lupus anticoagulant Ab thrombocytopenia prolonged APTT (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.) Management: Primary thromboprophylaxis - low dose aspirin Secondary - after initial VTE event: lifelong warfarin INR 2-3 (recurrent venous events INR 3-4) In pregnancy: aspirin + LMWH

Answer 57

Excess bone turnover -> uncoordinated process -> sclerotic and lytic areas => bone pain, enlarged mishapen bones, pathological fractures; Skull (bossing), spine/pelvis, and leg bones (bowing) most commonly affected. Investigations -- Ca -- phosphate ↑ ALP -- PTH Xray: plain radiographs*: osteolysis in early disease → mixed lytic/sclerotic lesions later skull x-ray: cotton wool appearance, thickened vault, osteoporosis circumscripta Management: Bisphosphonates - ALP will normalise with treatment Complications: hearing loss high output HF (high vascularity of abnormal bone) osteosarcoma spinal stenosis

Answer 58

Loss of bone mass Assess pts: Men 75<, Women 65<, previous fragility fracture, 50> but have RF: post menopausal, low BMI, low Ca, low Vit D, alcohol, smoking, chronic disease, long term steroids (7.5mg< daily for 3 months<), FH Exclude secondary causes: hypogonadism, DM, cushings, hyperthyroid, malabsorption, RA Qfracture tool (10%<) or FRAX (amber or red) Or fragility fracture and <75yo (if 75< straight to management) -> DEXA: T score (compares against young reference population) -1< normal -1 <-> -2.5 osteopenia <-2.5 osteoporosis Management: Osteoporosis, long term steroids, symptomatic vertebral fracture, 75< fragility fracture: Oral bisphosphonate e.g. alendronic acid If hip fracture: yearly iv zoledronic acid Treat for 5 years and reassess Denosumab - human monoclonal antibody that inhibits RANK ligand, which in turn inhibits the maturation of osteoclasts - can be used 2nd line

Answer 59

Oral tablet once a week Taken with water on an empty stomach, sit up for 30 minutes after. 6 months for full effect ADRs: oesophageal reactions, GI upset Atypical fractures Osteonecrosis of the jaw (pain, swelling, ulcers) - requires regular dental check ups; increased risk if iv treatment or for sx control in cancer.

Answer 60

Non-caseating granulomatous disorder, typically affects black females 20-40yo. Multi system presentation: Lungs: Bilateral hilar lymphadenopathy, fibrosis, nodules, dyspnoea, non-productive cough Malaise, et loss erythema nodosum, lupus pernio (chronic violaceous indurated plaques on the nose/face) uveitis, conjunctivitis, neuritis CNS nodules, diabetes insipidus Investigations: CXR: BHL, upper zone fibrosis Raised ACE levels, raised Calcium levels Restrictive spirometry Syndromes: Lofgren's syndrome: hilar lymphadenopathy, erythema nodosum, polyarthralgia Heerfordt's syndrome: parotid enlargement, fever, uveitis Management: oral steroids for approx. 2 years Indications for steroids: patients with chest x-ray stage 2 or 3 disease who are symptomatic. hypercalcaemia eye, heart or neuro involvement

Answer 61

Borrelia burgdorferi, spread by ticks Within 30 days of bite: erythema migrans (bullseye rash), headache, lethargy, fever arthralgia -> heart block, carditis, CNVII palsy, radicular pain, meningitis Investigations: Clinical diagnosis if erythema migrans ELISA Ab test*, if +ve -> immunoblot test If -ve, but strong suspicion, repeat in 4 weeks. Management: Doxycycline* (amoxicillin if pregnant) ceftriaxone if disseminated disease Jarisch-Herxheimer reaction: fever, rash, tachycardia after first dose of antibiotic (more commonly seen in syphilis, another spirochaetal disease)

Answer 62

Central, lateral or foraminal stenosis Causes: congenital, degenerative, herniated discs, ligament thickening (flava, posterior longitudinal), fractures, tumours Presentation (central): intermittent neurogenic claudication; lower back pain, buttock and leg pain: leg weakness that is worse on standing and sitting, better leaning forwards Presentation (lateral and foraminal): radiculopathy Investigation: MRI ABPI to exclude PAD Specialist management: MDT, physio, analgesia, decompression surgery

Answer 63

the most common cause is a central disc prolapse this typically occurs at L4/5 or L5/S1 Presentation: Lower back pain, bilateral sciatica, saddle anaesthesia, decreased anal tone Urinary dysfunction - late sign Urgent MRI Surgical decompression

Answer 64

Rise in creatinine of 26µmol/L in 48 hours OR > 50% rise in creatinine over 7 days OR Fall in urine output to < 0.5ml/kg/hour for more than 6 hours in adults Stage 1: Increase in creatinine to 1.5-1.9 times baseline, or Reduction in urine output to <0.5 mL/kg/hour for ≥ 6 hours Stage 2: Increase in creatinine to 2.0 to 2.9 times baseline, Reduction in urine output to <0.5 mL/kg/hour for ≥12 hours Stage 3: Increase in creatinine to ≥ 3.0 times baseline, or Reduction in urine output to <0.3 mL/kg/hour for ≥24 hours, Causes: Prerenal - dehydration, shock, HF, renal artery stenosis Intrinsic - ATN, glomerulonephritis, rhabdo, acute interstitial nephritis, tumour lysis syndrome, HUS Postrenal - obstructive uropathy; stones, BPH, cancer RF: CKD, chronic disease, AKI history, iodinated contrast agents in last 7 days, age, nephrotoxic drugs; NSAIDs, aminoglycosides, ACE-Is, diuretics Presentation: if not asymptomatic; oliguria, oedema, arrhythmias, uraemia Investigations: U+E: In prerenal; ↑ Urine osmolality, ↑urea:creatinine ratio, ↓Na excretion urine dip USS if no clear cause or ?obstruction Management: careful fluid balance Stop nephrotoxic drugs Stop metformin, lithium and digoxin due to ↑ toxicity Treat underlying cause

Answer 65

Most common intrinsic cause of AKI Death of tubular epithelial cells; due to ischaemia (shock/sepsis) or nephrotoxins (aminoglycosides, myoglobin, contrast agents, lead) AKI Muddy brown casts in urine

Answer 66

Immune reaction to: Penicillins, rifampicin, NSAIDs, allopurinol, furosemide SLE, sarcoidosis, sjogren's staphylococci -> AKI, sterile pyuria, white cell casts fever, rash, arthralgia, HTN eosinophilia

Answer 67

Long lie, extreme exercise, crush injuries, seizures, statins -> muscle breakdown => myoglobin, K+, phosphate, CK all released Presentation: AKI; oliguria, red-brown urine Muscle pain, weakness, swelling N+V, fatigue, confusion Investigations: ↑CK (5x upper limit for dx) ↑ K+ ↓ Calcium (myoglobin bind calcium) ↑Phosphate Metabolic acidosis Management: iv fluids to maintain good urine output Manage complications; ECG monitoring for hyperkalaemia

Answer 68

Hyperchloremic Metabolic Acidosis - normal anion gap Type 1: Distal tube unable to secrete H+ Hypokalaemia (due to H+/K+ exchange failure) ↑ urinary pH, ↑ Calcium, ↑ Stones Presents with FTT, muscular weakness, UTIs, bone disease, arrythmias, stones, nephrocalcinosis Many causes: RA, SLE Treat with oral bicarbonate Type 2: Proximal tubule decreased HCO3- resorption Hypokalaemia, (due to the need for intracellular Cl- for K+/Cl- cotransporter, which cannot enter the cell as Bicarb needed for electrical gradient) ↑ urinary pH Presents as above Causes include fanconi syndrome (type 2 RTA + polyuria, aminoaciduria etc), wilsons disease Treat with oral bicarb Type 3: Mixed 1 and 2 caused by carbonic anhydrase II deficiency Type 4: Reduced aldosterone -> ↓Na, ↑K+, ↑H+ ↓ Ammonium production due to serum hyperkalaemia ↓Urinary pH, ↓Serum pH Causes include addisons, eplerenone Fludrocortisone treatment

Answer 69

eGFR: CKD stage GFR range 1 Greater than 90 ml/min *(if kidney tests are normal, i.e. normal U&Es and no proteinuria, there is no CKD) 2 60-90 ml/min* 3a 45-59 ml/min 3b 30-44 ml/min 4 15-29 ml/min 5 Less than 15 ml/min ACR categories (mg/mmol) A1 <3 A2 3-30 A3 30< Oedema, polyuria (anuria in severe CKD), lethargy, pruritus, N+V, HTN Causes: Diabetes, HTN, PCKD, glomerulonephritis -> ↑ Na+ ↑Phosphate ↑K+ Metabolic acidosis Uraemia ↓ Vit D .'. ↓Ca .'. Secondary hyperparathyroidism Management: 130/80 BP target - ACE-Is, SGLT-2 inhibs. Atorvastatin 20mg Check iron; EPO for anaemia Decrease dietary phosphate if bone disease present Vit D analogues

Answer 70

Acidosis (severe and not responding to treatment) Electrolyte abnormalities (particularly treatment-resistant hyperkalaemia) Intoxication (overdose of certain medications) Oedema (severe and unresponsive pulmonary oedema) Uraemia symptoms such as seizures, reduced consciousness, pericarditis Long term: CKD 5 Haemodialysis: Requires an AV fistula (central venous line used in emergency (inf., thrombosis ADRs)): Easy access high pressure system Complications: aneurysm, infection, thrombosis, stenosis, STEAL syndrome; ischaemia of distal limb, disequilibrium syndrome, high output HF Peritoneal dialysis: High conc. dextrose solution injected into abdomen, which draws waste products from blood over hours, then removed. Complications: bacterial peritonitis, peritoneal sclerosis, hyperglycaemia, weight gain

Answer 71

Donor matching; HLA DR, A and B most important Donor kidney plumbed into external iliacs in RIF, ureter to the bladder Post op complications: ATN of the graft, vascular thrombosis, urine leakage, UTI Hyperacute rejection (minutes to hours): Pre-existing antibodies against HLA or ABO antigens Type II hypersensitivity -> necrosis Graft must be removed Acute graft failure (<6months) Mismatched HLA Cytotoxic T cell mediated (CMV other cause) -> asymptomatic; ↑creatinine, pyuria, proteinuria Steroids/immunosuppressants may reverse damage Chronic graft failure (>6 months) Cell mediated & antibodies -> fibrosis Recurrence of original renal disease Basilixuimab - IL2 receptor mab; 2 doses given post op Then lifelong immunosuppression

Answer 72

Basilixuimab - IL2 receptor mab; 2 doses given post op Then lifelong immunosuppression Ciclosporin - Calcineurin inhibitor; prevents T cell activation ADRs; hepato and nephrotoxic, ↑fluid retention, ↑BP, ↑K+, ↑hair, ↑ gingivial hyperplasia, ↑glucose Tacrolimus ADRs; ↑glucose, DM, HTN, ↑lipids hyperkalaemia k Mycophenolate mofetil - blocks purine synthesis ADRs: GI and marrow suppression Sirolimus - Inhibits T cell prolif. via IL2 receptor Pts should be monitored for CVD, renal failure, malignancy (squamous cell carcinoma*)

Answer 73

Type 1* 85% of acses, chromosome 16, more severe, earlier renal failure Type 2 chromosome 4 ↑BP, recurrent UTIs, flank pain, haematuria, palpable kidney, stones, CKD -> liver cysts, berry aneurysms, mitral valve prolapse, aortic root dilation Diagnosis; USS 2 cysts if <30yo 2 cysts bilaterally if 30-59yo 4 cysts bilateral >60yo Management: Tolvaptan if rapid progression or CKD3/4 Manage BP, pain etc.

Answer 74

Deficiency in the action of ADH; Cranial: idiopathic, TBI, pituitary masses Nephrogenic: genetic, ↑Ca+, ↓K+, Lithium, haemochromatosis -> polydipsia, polyuria high plasma osmolality, low urine osmolality -> water deprivation test: then give desmopressin = cranial, urine osmolality increases (becomes more concentrated) nephrogenic no change Management: nephrogenic; low salt/protein diet then thiazides central; desmopressin

Answer 75

ECG changes: (in order of worsening ↑K+) Tall and tented T waves Flattened P waves Broad QRS Sinusoidal wave pattern VF Causes: AKI, CKD, rhabdo, Addison's, RTA ACE-Is, K+ sparing agents, heparin Management: If K+ 6.5< or ECG changes present -> emergency treatment; iv calcium gluconate - stabilise cardiac muscle Insulin + dextrose infusion - short term shift of K+ into ICF +- salbutamol nebs K+ binders; calcium resonium or zirconium (enema more effective) If persistent -> dialysis

Answer 76

Muscle weakness, hypotonia ECG changes: Flattened T waves ST depression U waves Prolonged PR Apparent long QT (u wave appears to be the t wave) Causes: Alkalosis; Vomiting, diuretics, cushings(↑BP too), conns (↑BP too) Acidosis; diarrhoea, RTA1/2, acetazolamide Management: admit of <2.5 or symptomatic For oral or iv K+ supplementation

Answer 77

Defective Na+K+2Cl- cotransporter (Loop diuretic action) Autosomal recessive Polyuria, polydipsia, ↓K+, ↑pH, FTT

Answer 78

Defective Na+ resorption in distal tubule (Thiazide action) ↓Mg, ↓k+, ↓Ca2+, polydipsia, polyuria, ↓BP

Answer 79

↑ Na reabsorption -> ↑BP, ↑pH, ↓k+

Answer 80

Muscle spasms, perioral parasthesia, Trosseau's sign, Chovstek sign (Facial twitch when tapping carotids) and trousseau sign (carpal spasm during BP reading), Prolonged QT interval Causes: ↓Vit D, CKD, hypoparathyroid, early rhabdo, acute pancreatitis Management: If sx, iv calcium gluconate 10mls 10% solution over 10 mins ECG monitoring Treat underlying causes

Answer 81

lethargy, irritable, seizures, coma Causes: dehydration, osmotic diuresis, diabetes insipidus, excess saline management: Oral or iv fluids to correct Correction rate of <0.5mmol/hour to reduce risk of cerebral oedema; (over days, brain adapts to ↑ ECF osmolality, .'. water moves out of the brain. When ↑Na is corrected, ECF becomes hypotonic .'. water moves back into brain cells, causing oedema.)

Answer 82

headache, fatigue, muscle cramps, confusion, seizures Causes: High urinary sodium: Sodium depletion/renal loss; diuretics, addison's (Hypovolaemia) SIADH, hypothyroid (euvolaemia) Low urinary sodium: Extra renal losses; D+V, sweating, burns (Hypovolaemia) Water excess; HF, liver cirrhosis, nephrotic syndrome, iv dextrose, psychogenic polydipsia Management: Chronic ↓ Na+; Hypovolaemic (diuretics, Addison's, end stage CKD) - 0.9% NaCl Euvolaemic (SIADH) - fluid restrict 500-1000mls/day, consider demeclocycline (reduces ADH action) and vaptans (ADH receptor antagonist) Hypervolaemic - fluid restrict 500-1000mls/day, consider loop diuretics and vaptans (ADH receptor antagonist) If acute/symptomatic ↑ Na+; hypertonic saline use to correct rapidly If too quick in chronic pts (chronic hyponatraemia → loss of osmotically active organic osmolytes from astrocytes. These provide protection against cerebral oedema. Once ECF osmolality increases with correction, water leaves the brain) -> Osmotic demyelination syndrome: 2 days after, dysarthria, dysphagia, paralysis, seizure, coma, locked in syndrome

Answer 83

Morphine*; start with 30mg with 5mg for breakthrough pain (1/6 of daily dose for breakthroughs) Oxycodone in mild-moderate renal impairment Buprenorphine or fentanyl in severe ADRs: initial nausea and drowsiness Persistent constipation; always prescribe laxative alongside Opioid conversions Codeine/tramadol (weak opioids) ÷ 10 -> morphine (e.g. 10mg codeine = 1mg morphine) Morphine ÷ 1.5 -> Oxycodone Oral morphine ÷ 2 -> SC morphine Oral morphine ÷ 3 -> SC diamorphine

Answer 84

Morphine - ↓ resp. drive Midazolam - reduces anxiety

Answer 85

If: Gastric stasis - Metoclopramide Chemical - Haloperidol* or Levopromazine Increased ICP - Cyclizine

Answer 86

Haloperidol or Levopromazine

Answer 87

Hyoscine Butylbromide (Buscopan) (Does not cross BBB .'. less sedation) or Hyoscine hydrobromide

Answer 88

Chlorpromazine

Answer 89

Progressive impairment in memory, cognition, etc -> affecting ADLs Differentials/reversible causes: ↓B12, ↓Thiamine Hypothyroid, Cushing's, Hypercalcaemia Depression Anti-cholinergics Investigations: FBC, U+E, LFT, Ca, glucose, ESR, CRP, TFTs, B12, Folate -> Memory Clinic for cognitive screening tests; ACE III <82-88 = ?dementia MMSE <24/30 = ?dementia

Answer 90

Most common form Widespread cerebral atrophy; cortex and hippocampus Beta amyloid plaques and intraneuronal neurofibrillary tangles of hyperphosphorylated Tau protein ↓ACh Gradual decline in memory, cognition, etc Management: Acetylcholinesterase inhibitors; Donezepil (can cause bradycardia), galantamine, rivastigmine Memantine - NMDA receptor antagonist - used in severe cases

Answer 91

Stroke related VD, Subcortical VD, Mixed Stepwise deterioration in memory, cognition, etc Management: Non-pharma MDT Manage CVD risk factors

Answer 92

Alpha synuclein cytoplasmic inclusions -> Progressive cognitive impairment, with fluctuations in cognition Then parkinsonism develops Visual hallucinations; children and animals Clinical diagnosis +- single photon emission computed tomography SPECT

Answer 93

Onset before 65yo, insidious onset, relatively preserved memory, visuospatial skills, personality change and social conduct problems 3 types: Pick's disease; focal gyral atrophy with knife blade appearance -> personality change Progressive non fluent aphasia Semantic dementia

Answer 94

<35 degrees Cold environment, GA, newborns, cardiopulmonary bypass RF: GA, substance abuse, hypothyroid, homelessness -> shivering, cold, pale, slurred speech, ↑RR, ↑HR, ↑BP (All ↓ id worse temp.) ECG; J waves (just after QRS) ↓K+ due to ICS shift Management: gentle warming +- warm iv fluids but can -> peripheral vasodilation and shock

Answer 95

Shortened and externally rotated leg, pain, non-weight bearing. Intracapsular; undisplaced - internal fixation displaced - arthroplasty (total hip replacement) or hemiarthroplasty if limited mobility/significant co-morbidities Extracapsular; intertrochanteric - dynamic hip screw subtrochanteric - IM nail AP and lateral X rays Complications: peri-operative; VTE, fracture, nerve injury, infection Posterior dislocation aseptic loosening

Answer 96

Non-contrast CT to exclude haemorrhage If haemorrhage Then 300mg aspirin Thrombolysis if: within 4.5 hours of symptoms onset or if between 4.5 and 9 hours (or within 9 hours of the midpoint of sleep) AND imaging shows salvageable brain tissue Thrombectomy if: Within 6 hours AND proximal anterior circulation on CTA Between 6 and 24 hours AND proximal anterior circulation on CTA AND imaging shows salvageable brain tissue or Within 24 hours AND proximal Posterior circulation on CTA AND imaging shows salvageable brain tissue Secondary prevention: Aspirin for 2 weeks then clopidogrel lifelong (DOAC if AF) Statin 80mg after 48 hours Carotid endarterectomy if 50%< stenosis

Answer 97

Exclude hypoglycaemia & CT head if on anticoags 300mg aspirin Assessment within 24 hours; MRI with diffusion weighted imaging Check bleed risk; Orbit score Hb low 2 75yo< 1 Bleed history 2 GFR <60 1 tx with antiplatelets 1 0-2 low risk 3 medium 4-7 high If low risk: -> Dual antiplatelet therapy for 21 days: Clopidogrel (300mg loading dose then 75mg OD) Aspirin (300mg loading dose then 75mg OD) Then clopidogrel monotherapy

Answer 98

Degeneration of dopaminergic neurones in substantia nigra: Bradykinesia; shuffling gait, reduced arm swinging, difficulty initiating illness Tremor; worse on rest: pill-rolling, unilateral, improves on intentional movement Rigidity; lead pipe, cogwheel +- mask-like facies, micrographia, depression, ↓olfaction, fatigue, postural hypotension Diagnosis is clinical SPECT scan can confirm if still ? Management: If motor sx: Levodopa (+ DOPA decarboxylase inhibitor); ADRs dry mouth, anorexia, palpitations, postural hypotension. If not: Dopamine agonists; ADRs risk of hallucinations and impulse control disorders

Integrated + HCOLL Flashcards

(122 cards)