PACES Station 5 Flashcards

1
Q

Clinical Features of Acromegaly

A
Large, sweaty hands
Thickened skin
Prominent supraorbital ridges, enlarged nose and lips
Prognathism
Interdental separation
Macroglossia
Hypertrichosifs
Gynaecomastia
Bitemporal hemianopia (10%)
Carpel tunnel
HTN
Cardiomegaly
Diabetes
Arthropathy
Skin tags and colonic polyps
Headaches, change in ring size/show size
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2
Q

Pathophysiology of Acromegaly

A

Most commonly caused by a growth hormone secreting adenoma of the anterior pituitary. 75% are macro adenomas

Rarer causes include ectopic GH release from carcinoid or small cell tumours

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3
Q

Complications of Acromegaly

A
HTN
Cardiomegaly
Arthritis
IGGT/DM
Visual field defects
Cranial nerve palsies
Tumours: fibroids and colonic polyps with risk of developing into malignancy
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4
Q

Investigating possible Acromegaly

A

Oral glucose tolerance test - should suppress GH
Serum insulin-like growth factor 1
MRI pituitary

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5
Q

Management of Acromegaly

A

Surgical - transphenoidal hypophysectomy with attempt to leave remaining pituitary intact to preserve function

Medical - somatostatin analogues (ocreotide) inhibit GH release. Cause gallstones.
Bromocriptin (Dopamine)agonist

Radiotherapy if surgery contraindicated is an option but also effects other pituitary hormones and visual loss

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6
Q

Differential Diagnosis for lump in Neck

A

Congenital - thyroglossal cyst, branchial anomalies
LNs - bacterial, viral, granulomatous, malignancy
Vascular - aneurysm
Salivary - tumour
Neurogenic - schwannoma, neurofibroma
Thyroid…

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7
Q

Management of Thyroid Nodule

A
  1. Functioning or not?
    - serum TSH, if low suggests need scintigraphy to delineate hot nodule or not
    - if TSH normal or high then FNA

Histology may be indeterminate but generally still remove

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8
Q

Types of Thyroid Cancer

A

Papillary carcinoma - most common and most favourable prognosis. Radio iodine /surgical management

Follicular carcinoma

Anaplastic carcinoma - highly aggressive undifferentiated

Medullary thyroid carcinoma - associated with MEN2a (phaeo and primaryPTH), MEN2b (phaeo + marfanoid)

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9
Q

Complications of Large Goitre

A
Dyspnoea and airway obstruction
Dysphagia
Recurrent laryngeal nerve palsy
Horners
Jugular vein compression
Cerebrovascular steal syndrome
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10
Q

Clinical Features of Thyrotoxicosis

A

Goitre (usually non-tender), smooth diffuse in graves
Heat intolerance, sweating, agitation, stress
Weight loss, palpitations
AF
Brisk reflexes
Eyes - proptosis, ophthalmoplegia, lid lag
Specific to graves - proptosis, thyroid acropachy, pretibial myxoedema

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11
Q

Investigations of Thyrotoxicosis

A

TSH/T4
Thyroid autoantibodies
Radioisotope scanning

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12
Q

Management of Graves Thyrotoxicosis

A

Beta blocker - propranolol
Carbiomazole or propylthiouracil
Titrate dose
Stop at 18months (1/3 will remain euthyroid)

IF returns consider:

  • repeat course
  • radioiodine
  • subtotal thyroidectomy

High dose steroids/surgical decompression for severe ophthalmoplegia

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13
Q

Progression of Eye Signs in Graves

A
NOSPECS
No signs
Only lid lag
Soft tissue involvement
Proptosis
Extraoccular muscle involvement
Chemosis
Sight loss due to optic nerve compression/atrophy
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14
Q

Common causes of Hyperthyroidism

A
Graves
Hashimotos (thyrotoxicosis before hypothyroid)
Toxic adenoma
Iodine induced
TSH secreting tumour (rare)
Infective thyroiditis
Post-partum thyroiditis
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15
Q

Causes of Hypothyroidism

A

Primary

  • autoimmune (hashimotos)
  • iodpathic atrophy
  • previous radio iodine
  • antithyroid drugs
  • infiltrative: sarcoid, systemic sclerosis

Secondary
-hypothalamic, pituitary disease

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16
Q

Drugs causing Hypothyroidism

A

Iodine contrast
Lithium
Sunitinib (tyrosine kinase inhib) used in renal cell carcinoma

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17
Q

Features of Cushings

A
Cushingoid facies
Centripetal obesity
Inter scapular fat pad
Acne vulgaris
Purpuric and petechial rashes
Bruising
Violaceous striae
Proximal myopathy
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18
Q

Pathophysiology of Cushings Disease

A

Hyper secretion of ACTH from pituitary adenoma

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19
Q

Causes of Cushings Syndrome

A

ACTH dependent:

  • Cushings disease (pituitary adenoma)
  • Ectopic ACTH from SCLC or carcinoid

ACTH independent

  • exogenous steroid administration
  • adrenocortical adenoma/carcinoma
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20
Q

Investigations for cortisol excess

A

24hr urinary free cortisol (x3)
Midnight salivary/serum cortisol
Dexamethasone suppression test (no ACTH suppression)

Low dose dexamethasone suppression test can help distinguish between bushings and pseudocushings

Consider inferiors petrosal sinus sampling in ACTH dependent Cushings with no clear pituitary source

Check Calcium - hyperPTH in MEN1

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21
Q

Complications of Cushings

A

Diabetes
Osteoporosis
HTN
Cataracts

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22
Q

Management of Cushings Syndrome

A

If adenoma - surgical transphenoidal/adrenalectomy

If adjunct needed then ketoconazole used to inhibit steroid genesis

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23
Q

Nelsons Syndrome

A

Enlargement of a pituitary corticotroph adenoma due to absence of negative feedback from adrenal cortisol following removal of both adrenals.

Also get hyper pigmentation as high ACTM stimulated melanocyte stimulating hormone

May get bitemporal hemianopia

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24
Q

What is MEN1?

A

Autosomal dominant

  • Pituitary adenoma (cushings)
  • hyperparathyroidism
  • pancreatic gastrinoma
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25
Clinical Features of Addisons Disease
Widespread pigmentation - buccal mucosa, skin creases and pressure areas Calcification of auricular cartilage No adrenelectomy scars (would be present in Nelsons) Postural hypotension Other autoimmune conditions: vitiligo, T1DM, Pernicious anaemia, RA, SLE, Sj
26
Causes of Primary Adrenal Failure
``` Autoimmune (Addisons) TB Bilateral adrenelectomy Amyloidosis Haemochromatosis HIV Haemorrhagic infarcts - meningococcal Adrenal Mets Congenital Adrenal Hyperplasia ```
27
Diagnostic Tests for Adrenal Insufficiency
9 am serum cortisol | Synacthen test
28
Management of Adrenal Failure
Adrenal crisis - rapid steroid, fluid replacement and reversal of electrolyte abnormalities (low Na, high K)
29
Causes of Pituitary Failure
``` Pituitary mass Previous pituitary surgery/radiotherapy Infiltrative disease Pituitary infarct (Sheehans) Pituitary apoplexy Trauma/ SAH ```
30
Causes of Gynaecomastia
``` Hypogonadism Testicular tumour Chronic Liver Disease CKD Klinefelters Thyrotoxicosis Carcinoma of lung Drugs: digoxin, spironolactone Puberty ```
31
Klinefelters Syndrome
``` Tall Gynaecomastia Other features of hypogonadism including small, firm testes Autoimmune disease Mitral valve prolapse Varicose veins ``` Genetics: XXY or mosaicism XXY:XY
32
Turners Syndrome
XO ``` Short stature, square chest Widely spaced nipples Webbed neck Nail dysplasia High arch palate Short fourth metacarpals Numerous naevi Wide carrying angle ``` Associated: Coarctation, DM, renal malformations, cataracts, ptosis
33
Physiological Actions of Parathyroid Hormone
Stimulated by low Calcium Bone: increase bone resorption Kidneys: stimulates calcium reabsorption and inhibits phosphate (unlike vitD which promotes uptake of both) Stimulates vitD synthesis
34
Clinical Features of RA
``` Symmetrical deforming polyarthropathy Involves PIPs and MCPs Palmar subluxation and ulnar deviation Swan neck, boutonnieres and Z deformity Rheumatoid nodules (firm, non-tender) May be vasculitic nail fold infarcts/pupura Risk of nerve entrapments ``` Then: MSK, ears, eyes, c-spine, lungs, heart, spleen, kidneys
35
Diagnostic Criteria for RA
``` ACR - at least 4 of the following: -morning stiffness >1hr -symmetrical joint involvement -arthritis in >3 joints involves small joints of hand -postive RF -rheumatoid nodules -radiographic evidence ```
36
Rheumatoid Factor
IgM against Fc of IgG. Present in 75% of RA and occurs in other autoimmune connective tissue disease e.g. SLE. Suggests more aggressive joint disease than seronegative disease and extra-articular manifestations more common AntiCCP also a poor prognostic marker
37
Possible causes of anaemia in RA
``` Anaemia of chronic disease Iron deficiency -GI loss due to NSAIDs Folate deficiency - methotrexate Pernicious anaemia BM suppression - gold, methotrexate, sulphasalazine Feltys Anaemia of renal disease ```
38
Eye involvement in RA
``` Episcleritis (painless) Scleritis (painful) Cataracts - steroids Sjogrens Extraoccular muscle tenosynovitis ```
39
Renal disease in RA
Membranous glomerulonephritis - gold Mesangioproliferative glomerulonephritis Acute tubulointerstitial nephritis - NSAIDs Renal amyloidosis
40
Principles of Management of RA
MDT General: education, physio, OT, bone protection, pneumovax Analgesia DMARDS Biologicals - antiTNF (infliximab/adaluminab) Corticosteroids
41
DMARDS in RA
Sulphasalazine - risk of nausea, rash neutropenia - 3 monthly FBC, LFT Methotrexate - need baseline CXR and monitor LFT, FBC Leflunomide- risk of HTN, neutropenia Azathioprine - check TPMT prior to starting (predicts BM toxicity) Ciclosporin - causes HTN and renal impairment
42
Clinical Features of Psoriatic Arthropathy
Nails - pitting, onycholysis, transverse ridging Psoriatic plaques Koebners phenomenon - plaques over sites of trauma Other things to examine: - MSK association with gout - CVS: Aortitis and AR - Resp: apical lung fibrosis - Eyes: conjunctivitis, uveitis
43
Patterns of Psoriative Arthropathy
Asymmetrical, oligoarthritis involving DIP Symmetrical polyarthritis involving PIP and MCP (rheumatoid pattern) DIP arthritis Arthritis mutilans - aggressive deforming disease with bone resorption leading to telescoping Spondylitis/sacroilitis - asymmetrical unlike AnkSpond
44
Enthesitis
Inflammation of tendon insertion
45
Dactylitis
Inflammation of tendon sheath leading to sausage like swollen fingers
46
Management of Psoriatic arthropathy
MDT... Analgesia Corticosteroids DMARDs - methotrexate, sulfasalazine, cyclosporin
47
Adverse effects of antiTNF alpha
``` Serious infection e.g. TB Haematological malignancy Nausea Hypersensitivity Worsens heart failure ```
48
Contraindications for antiTNF alpha
``` Pregnancy/breastfeeding Active bacterial infection Active TB (need 2 month therapy before initiating) Septic arthritis CCF Demyelinating disease ```
49
Clinical Features of Ankylosing Spondylitis
``` Severe kyphosis with protuberant abdomen Decreased cervical movement Decreased spinal movement Reduced chest expansion Schobers test positive - 10 above, 5 below dimples of venus should increase by 5cm ``` Extra bits: anterior uveitis, aortic regard, apical fibrosis, AV block (check pacemaker)
50
Definition of Ankylosing Spondylitis
Symptomatic sacroilitis for > 3 months with morning stiffness and improvement with exercise, worsening with rest
51
Systemic Manifestations of Ankylosing Spondylitis
``` Eyes: anterior uveitis Aortic regurg AV block Apical fibrosis Atlantoaxial subluxation Achilles tendonitis Amyloidosis ```
52
Diagnosing Ank Spond
Clinical hx Raised inflammatory markers evidence of sacroilitis on XR/MRI
53
Management of Ank Spond
``` MDT... Education Physio/OT Analgesia Corticosteroids DMARDs - gold, penicillamine (methotrexate and sulphasalazine help peripheral disease but not spine) Biologicals such as adalimumab ```
54
Clinical Features of Gout
Asymmetrical joint deformity and swelling Tophi, hands, feet, ears Olecranon bursa
55
Differential Diagnosis for single inflamed joint
``` Gout/pseudogout Septic arthritis Spondyloarthritis OA Lyme disease/stills disease ```
56
Pathophysiology of Gout
Uric acid produced by breakdown of purines in liver by enzyme xanthine oxidase - eliminated by kidneys and GI tract Causes: - reduced excretion: HTN, CKD, drugs (ACEi, aspirin, thiazides) - Increased purine production: diet, lymphoproliferative disorders, secondary polycythaemia, glycogen storage disease
57
Management of Gout
Acute: rest, rehydrate, NSAIDs, colchicine Recurrent: lifestyle advise, allopurinol
58
What is pseudogout
Crystal arthropathy of calcium pyrophosphate deposition - postivitely bifringent crystals in polarised light Chondrocalcinosis on radiograph Occurs in haemochromatosis, hypothyroidism and CKD
59
Classification of Systemic Sclerosis
Diffuse: involvement of skin, trunk and extremities, early involvement of lung, kidney, gut and heart Limited: skin only at extremities and face, late lung involvement including pHTN, renal crisis is rare
60
Diagnostic Criteria for Systemic Sclerosis
1 major or 2+ minor Major - skin involvement Minor - sclerodactyl, finger pulp atrophy, bilateral pulmonary fibrosis
61
Organ involvement in systemic sclerosis
Skin - raynauds, sclerodactyly, pulp atrophy, calcinosis, nail dystrophy, ulceration, telangiectasia, vitiligo MSK - arthritis, myositis, myopathy, intraairticular calcification GI - dysphagia, GORD, intestinal hypo motility leading to bacterial overgrowth and malabsorption Renal - malignant HTN, glomerulonephritis, renal crises Resp - ILD, pleural effusions CVS - restrictive cardiomyopathy, pericarditis, pulmonary HTN
62
Antibodies in Systemic Sclerosis
Limited - ANA, RF, antiCentromere | Diffuse - anti Scl 70, anti RNA pol
63
Management of Systemic Sclerosis
General: education, PT/OT, analgesia, MDT Raynauds: smoking cessation, hand warmers, vasodilators (calcium antagonists, ACEi, prostacyclin), sympathectomy GI: low residue diet, nutritional supplements, Abx, PPI for reflux, pro kinetics Renal: ACEi Rs: vasodilator therapy for pHTN, antifibrotis therapy - D penicillamine, interferon gamma
64
Clinical features of psoriasis
Erythematous plaques with silvery white scales on extensor surfaces, behind ears and scalp Asymmetric arthropathy and nail changes Koebner phenomenon Auspitz sign - visible bleeding points over plaques from enlarged dermal capillaries and intense scratching CVS - aortic regard Rs - apical lung fibrosis Eye - uveitis
65
Pathophysiology of Psoriasis
Inflammatory skin condition Receive abnormal epidermal proliferation T cell mediated Associated with HLA-B13, B17 and Cw6, also susceptibility gene on chr 6 Genetics and environmental factors involved
66
Exacerbating factors in Psoriasis
``` Trauma Sunburn Stress Alcohol Smoking Beta haemolytic strep - guttate psoriasis Drugs - lithium, beta blockers, Acei, antimalarials Withdrawal of steroids HIV ```
67
Cardinal histopathological features of psoriasis
``` Acanthuses (skin thickening) Parakeraotisis Absence of granular cell layer Polymorphonuclear abscess of munro Dilated capillary loops in upper epidermis ```
68
Variants of Psorias
``` Chronic plaque psoriasis Pustular psoriasis Guttate psoriasis Palmoplantal psoriasis Erythrodermic psoriasis ```
69
What skin conditions demonstrate Koebners phenomenon?
``` Psoriasis Lichen planus Vitiligo Viral warts Pemphigus Pemphigoid Molloscum contagiosum ```
70
Management of psoriasis
General: patient education, avoid exacerbating factors Topical: emollients, steroids, UVB, tar, dithranol Systemic: methotrexate, azathioprine, cyclosporin, tacrolimus, retinoids
71
Clinical Features of Neurofibromatosis
Neurofibromas along course of peripheral nerves > 6 Axillary freckling Cafe au last spots >6 HTN - secondary to renal artery stenosis Acoustic neuroma ? hearing aid Eyes - cataracts, optic glioma, lisch nodules (hamartomata on iris) on slit lamp Pulmonary fibrosis, pneumothorax Heart - cardiomyopathy MSK: Kyphoscoliosis, bowing of legs, rib notching, charcot joints Kidney - RAS Phaeochromocytomas
72
Genetics of Neurofibromatosis
NF1 (peripheral) - AD, chr 17, tumour suppressor gene (neurofibromin normally binds and inhibits Ras) NF2 (central) AD chr 22 also tumour suppressor gene
73
Clinical features of HHT
Telangiectasis of face, mouth, lips, tongue, buccal mucosa NO features of Systemic sclerosis Conjunctival pallor (iron deficiency anaemia) Resp: AVMs, may be clubbed as a result CVS: high output cardiac failure GI: iron def anaemia, hepatosplenomegaly, portal HTN Eyes - retinal haemorrhage Neuro: SAH, central vascular lesions, ischaemic stroke - paradoxical emboli from pulmonary AVM
74
What are telangiectasia?
Enduring dilation of small blood vessels, capillary or venules (venules in HHT)
75
What is HHT
Autosomal dominant condition, multiple genes associated on different chromosomes Telangiectasia effect multiple organs
76
Management of HHT
``` Anaemia - iron sups, transfusion Skin - cosmetic/laser ablation Epistaxis - cauterise, laser ablation PAVM - embolisation, surgical resection/ligation GI - photocoag therapy, ```
77
Clinical Features of Dermatomyositis
Heliotrope rash around eyes Violaceous scaly papule over knuckles, elbows, knees - Gottron papule Erythema + Poikiloderma in photosensitive distribution Proximal muscle weakness and tenderness Associated ILD, dysphagia, arthritis
78
Drugs causing dermatomyositis like skin changes
Hydroxyurea Penicillamine Statin Quinidine
79
Investigations in Dermatomyositis
raised CK EMG - spontaneous fibrillation Muscle biopsy - necrosis, phagocytosis of muscle fibres
80
Causes of Purpura
Platelet defect - idiopathic, CLD Capillary defect - age, steroids, vasculitis, ehlers-danlos, HHT, scurvy Coag defect - warfarin, vWF, DIC, amyloidosis
81
Lichen Planus
Flat topped, violaceous polygonal papule on fleecers surfaces Fine white striae - wickhams - on surface of lesions Koebners phenomenon Scalp involvement with scarring alopecia Lace like white lines on buccal mucosa Dystrophic nails Associated drugs - ACEi, beta blockers, NSAIDs, antimalarials Hep C association
82
Underlying causes of Erythema nodosum
``` Streptococcal throat infection TB Sarcoidosis IBD Behcets Drugs - OCP, penicillin, tetracyclines, sulphonamides ```
83
Clinical Features of SLE
Erythematous, maculopapular rash in butterfly distribution, sparing nasolabial folds with scaling and follicular plugging May have vasculitis lesions, livedo, photosensitivity, alopecia Symmetrical non-erosive polyarthritis Hands - palmar erythema, arthritis, raynauds, dermatomyositis Nails - splinter haemorrhages, nail-fold capillaries, periungal infarcts Mouth - ulcers Scalp - scarring alopecia Rs - ILD, pleural effusions, CVS - pericarditis, pHTN CNS - hemiparesis, mononeuritis multiplex Renal - HTN, oedema, Haem - splenomegaly, lymphadenopathy Eyes - sjogrens, retinal infarcts
84
Diagnostic Criteria for SLE
``` 4 of the following: -malar rash -discoid rash -photosensitivity -oral ulcers -arthritis -serositis -renal involvement -neurological involvement -haematologial involvement ANA positive ```
85
Causes of Drug Induced Lupus
``` Procainamide Hydralazine Quinidine Chlorpromazine Methyldoxpa Phenytoin Carbamazepine OCP ```
86
Differentiating SLE with drug induced lupus
SLE - more females, renal and CNS involvement more common, positive antidsDNA Drug induced - equal male to female, rare renal and CNS involvement, positive anti-histone
87
Autoantibodies of SLE
``` ANA in 95% antihistone in drug induced antidsDNA - high specificity for SLE Anti-ro, antiLa antiphospholipid ```
88
Management of SLE
Non-pharm - avoid over exposure - avoid high dose OCP - remove offending drugs - smoking cessation Pharm - NSAIDs - antimalarials - hydroxychloroquine - corticosteroids - cytotoxics: azathioprine, mycophenalate, cyclosporin
89
Clinical Features of Tuberous Sclerosis
Salmon papule and nodules over face in butterfly distribution - adenoma sebaceous Periungal fibroma - smooth, firm nodules adjacent to nails Shagreen patches - irregular, green-brown patches over lumbosacral region Ash leaf macules - hypopigmented areas over trunk and buttocks Cafe au last spots Eyes - angiofibromata of eye lid, retinal hamartomata Oral - bifid uvula, high arch palate Resp - pneumothorax, cysts Abdo - hepatic and renal hamartoma Neural - focal deficit depending on location of tubers
90
Genetics of Tuberous sclerosis
Autosomal dominante - 2 loci identified Chr 9 and Chr 16 (hamertin and tuberin tumour suppressor genes) 65% spontaneous
91
Pemphigus vulgaris
``` Flaccid blisters with clear fluid Fragile Involved buccal mucosa\ Blisters just above basal cell layer IgG autoantibodies against desmogleins ``` Drugs implicated: penicillamine, benzylpenicillin, ACEi Treat with barrier nursing, corticosteroids, avoid dehydration
92
Pemphigoid
Tense blisters IgG against hemidesmosomes in basement membrane Mucosal involvement rare Treat with steroids/steroid sparing agents
93
Pseudoxanthoma elasticum
folds of loose skin in neck, popliteal fossa, axilla and groin Plucked chicken appearance Due to abnormal mineralisation and calcification of elastic fibres Associated with: - aortic regurg - mitral regurgitation - mitral valve prolapse - peripheral vascular disease - restrictive cardiomyopathy - renovascular disease - HTN - angioid streaks
94
Causes of Angioid Streaks
Irregular red-brown lines radiating from optic disc ``` Ehlers danlos Pseudoxanthoma elasticum marfans sickle cell disease lead poisoning ```
95
Ehlers Danlos Syndrome
11 different forms, some AD some AR Collage defect Features: skin - broad nasal bridge, wide eyes, thin hyper extensible skin, impaired wound healing MSK -kyphoscoliosis, hyper extensible joints, hallus valgus, pes planes GI: diverticula, diaphragmatic hernia, megacolon CVS - MVP, AR, aneurysms, dissections
96
Causes of Blue sclerae
``` Pseudoxanthoma Marfans OI Ehlers Danlos Alkaptonuria ```
97
Causes of Acanthosis Nigricans
``` Obesity Diabetes/insulin resistance Cushings Acromegaly Thyroid disease PCOS Familial Malignancy - adenocarcinoma of GI tract ```
98
Pretibila myxodema
Firm, non pitting pink/purply brown plaques on anterolateral aspect of lower legs peau d'orange texture
99
Diabetic Retinopathy
Background: blot haemorrhages, hard exudate Preprolifeative: large blot haemorrhages, cotton wool spots, venous dilatations/beading Proliferative: leashes of new vessels with evidence of photocoag scars
100
Eye problems in Diabetes
``` Cataracts Glaucoma Mononeuritis multiplex Infections Diabetic retinopathy (25%) ```
101
Pathophysiology of Diabetic Retinopathy
Hyperglycaemia's structural and physiological effect on retinal capillaries Increased sorbitol results in increased vessel permeability and weakness Retinal ischaemia (small vessel disease) triggers VEGF Production of vasoprolierative factors e.g. VEGF leads to new vessel formation
102
Indications to refer to Opthalmology
Non-proliferative with macular involvement Pre-proliferative Proliferative - urgent
103
Complications of laser photocoag
Vitreous haemorrhage Visual field constriction Headaches Retinal vein occlusions
104
Hypertensive retinopathy
1 - Arteriolar narrowing 2 - AV nipping 3 - micro aneurysms, blot haemorrhages, flame haemorrhage, exudates 4 - papilloedema
105
Retinitis Pigmentosa
Black-brown pigmentation resembling bone spicules Constricted visual fields Night blindness Photopsia - flashes of light Associated with alports syndrome, bardett-biedel (polydactyly)