Stuff Flashcards
Typical presentation of porphyria cutanea tarda?
Fragility and blisters
Other features: hyperpigmentation, hypertrichosis, solar urticaria, morphoea
Investigation for porphyria cutanea tarda?
Woods lamp
Causes of porphyria cutanea tarda?
Alcohol, viral hepatitis, oestrogens, haemochromatosis
Defect in uroporphyrinogen decarboxylase causes what?
Porphyria cutanea tarda
Cancer you might get if you have PCT?
Hepatoma (hepatocellular carcinoma)
Defect in ferrochelatase causes which condition?
Erythropoietic protoporphyria
Investigations for erythropoietic protoporphyria
Quantitative RBC porphyrins Fluorocytes (patient, relatives) Transaminases [Hb], red cell indices (Biliary tract USS) (Phototesting)
Management of EPP
6 monthly LFTs and RBC porphyrins
Visible light photoprotection measures (particular need for caution if surgery needed)
Prophylactic TL-01 phototherapy
Anti-oxidants (beta-carotene, cysteine, high dose vitamin C)
(Avoid iron)
Incipient liver failure: oral charcoal; cholestyramine; ? ALA synthase inhibition
Liver failure: transplant liver; ? Bone marrow
Acute intermittent porphyria differentials?
Acute abdomen
Mononeuritis multiplex
Guillain-Barré syndrome
Psychoses
(female) patient with abdominal pain +/- neurological +/- psychiatric +/- hyponatremia
Acute intermittent porphyria
Fluid and electrolyte balance
Sweat glands
Epidermis cells
Mainly squamous
Stratified keratinising
Where are the melanocytes found?
Dermo-epidermal junction
Ratio of melanocytes : basal cells
1:10
Which layer of epidermis are melanocytes found in?
The basal layer
Where are langerhans cells found?
Mid - upper epidermis
Dermis
The dermis: a thicker inner portion. This is the connective tissue layer of skin. It is important for sensation, protection and thermoregulation. It contains nerves, the blood supply, fibroblasts, etc, as well as sweat glands, which open out onto the surface of the skin, and in some regions, hair. The apical layers of the dermis are folded, to form dermal papillae, which are particularly prominent in thick skin
Acanthosis
Thickening of epithelium
Parakeratosis
Persistence of nuclei in the keratin layer
Hyperkeratosis
Increased thickening of keratin layer
Papillomatosis
Irregular epithelial thickening
Psoriasiform
Elongation of rete ridges e.g. psoriasis
Munro micro abcess
Associated with psoriasis
-an abcess (collection of neutrophils) in the stratum corneum
Which investigation should all patients presenting with erythema nodosum be given?
x-ray for chest involvement
Anti-endomysial antibodies
Dermatitis herpetiformis
Areas affected by dermatitis herpetiformis?
Elbows, knees, back and buttocks
Side effect of nifedipine?
Gingival hyperplasia
People with polymyalgia rheumatica may also develop what?
Giant cell arteritis
Lattisimus dorsi?
Adducts shoulder
Extends shoulder
Internally rotates shoulder
SLE biologics
Anti-CD20 (rituximab)
Anti-Blys (Belimumab)
Serum and synovial keratin sulphate
Levels of serum and synovial keratin sulphate increase with age and in OA
SLE is which kind of hypersensitivity?
Type III hypersensitivty
Nucleolar ANA
Scleroderma
Speckled antibody
SLE and sjogrens
What would you find in a type III hypersensitivity biopsy?
Immune complexes
Neutrophils and macrophages
Products of complement activation
Rheumatoid arthritis type of hypersensitivity
Type IV (delayed) Characterised by infiltration of synovium with CD4+ cells
TNF AND IL-1 PLAY CRITICAL ROLE IN WHICH DISEASE?!
RA
-Important mediators of joint damage!!
What is rheumatoid factor?
antibody directed against the Fc region of IgG
Major indications for Rheumatoid factor testing?
Good indicator of PROGNOSIS of RA (more severe etc, associated with extra-articular manifestations e.g. nodules and vasculitis)
May be helpful in diagnosis of Sjogrens and cryoglobulinemia
Best predictor of aggressive disease course in RA?
Anti-CCP
Systemic inflammatory response syndrome?
HB > 90
RR > 20
WBC >12,000 or <4.3 kPa
Salmonella may cause osteomyelitis in which specific set of patients?
Sickle cell anemia patients
Poorly controlled diabetics, immunocompromised and IV drug users are particularly at risk of osteomyelitis where?
In the spine!!
Risks of HRT
Endometrial and breast cancer
DVT
How to diagnose stress fracture
Ultrasound or bone scintigraphy
They are common in the pelvis
Show first radiological signs of healing 4-8 weeks post fracture
Galeazzi fracture?
Fracture of the distal third of the radius and dislocation of the ulna
Fracture just above the elbow with displacement of the distal fragment
Supracondylar fracture
Supracondylar fracture risks
Median nerve
Brachial artery
Compartment syndrome
SUFE associated with which endocrine disorder?
Hypothyroidism
Osgood schlatters disease
Osteochondritis of the tibial tuberosity
- Classically affects teenagers and those who enjoy sport
- local swelling or tender lump over tibial tuberosity
- symptoms exacerbated by exercise and relieved by rest
Pink/blue sweaty hand that is tender to touch and stiff
Injury occurred some weeks ago
Complex regional pain syndrome
Swelling an calf tenderness following THR?
Deep vein thrombosis
Chondromalacia
Damage to the cartilage at the back of the knee cap
-More commonly found in girls who exercise regularly
What commonly precedes tranient synovitis of the hip?
Upper respiratory tract infection
Where would you develop a chondroma?
Hands and feet
Distinct clinical picture of osteoid osteoma?
Worse at night, dull pain, characteristically relieved by NSAIDs or aspirin
Enchondroma histology?
Small round pyknotic nuclei, no atypia
Classical RADIOLOGICAL image of osteoid osteoma?
Radiolucent niddus surrounded by reactive sclerosis in cortex of bone
Chicken wire calcification
Chondroblastoma
Chondroblastoma histology
Closely packed polygonal cells plus areas of immature chondroid
-Mitotic activity low
Treatment for chondroblastoma?
Biopsy and curretage plus adjuvant liquid nitrogen
Multi-nucleated cells in a sea of round to oval cells
Giant cell tumour
Irregular spicules of mineralised bone and osteoid surrounded by osteoblasts
Vascular stroma with pleomorphic SPINDLE CELLS
Osteoid and woven bone are seen
Osteoblastoma
Lobules and fibrous septa
Chorda
Lobules and fibrous septa
The malignant cells have eosinophilic cytoplasm and vacuoles of mucus push the nuclei to the side
Separation of nodules by fibrous bands would be highly suggestive of what?
Malignancy
Malignant nodules infiltrate between the lamellar bone obliterating the marrow. Separation of the nodules by fibrous bands would be highly suggestive of malignancy
Peripheral primitive neuroectodermal tumour
Ewings sarcoma