PACES Station 5 Flashcards
Clinical Features of Acromegaly
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
Pathophysiology of Acromegaly
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
Complications of Acromegaly
HTN Cardiomegaly Arthritis IGGT/DM Visual field defects Cranial nerve palsies Tumours: fibroids and colonic polyps with risk of developing into malignancy
Investigating possible Acromegaly
Oral glucose tolerance test - should suppress GH
Serum insulin-like growth factor 1
MRI pituitary
Management of Acromegaly
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
Differential Diagnosis for lump in Neck
Congenital - thyroglossal cyst, branchial anomalies
LNs - bacterial, viral, granulomatous, malignancy
Vascular - aneurysm
Salivary - tumour
Neurogenic - schwannoma, neurofibroma
Thyroid…
Management of Thyroid Nodule
- 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
Types of Thyroid Cancer
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)
Complications of Large Goitre
Dyspnoea and airway obstruction Dysphagia Recurrent laryngeal nerve palsy Horners Jugular vein compression Cerebrovascular steal syndrome
Clinical Features of Thyrotoxicosis
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
Investigations of Thyrotoxicosis
TSH/T4
Thyroid autoantibodies
Radioisotope scanning
Management of Graves Thyrotoxicosis
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
Progression of Eye Signs in Graves
NOSPECS No signs Only lid lag Soft tissue involvement Proptosis Extraoccular muscle involvement Chemosis Sight loss due to optic nerve compression/atrophy
Common causes of Hyperthyroidism
Graves Hashimotos (thyrotoxicosis before hypothyroid) Toxic adenoma Iodine induced TSH secreting tumour (rare) Infective thyroiditis Post-partum thyroiditis
Causes of Hypothyroidism
Primary
- autoimmune (hashimotos)
- iodpathic atrophy
- previous radio iodine
- antithyroid drugs
- infiltrative: sarcoid, systemic sclerosis
Secondary
-hypothalamic, pituitary disease
Drugs causing Hypothyroidism
Iodine contrast
Lithium
Sunitinib (tyrosine kinase inhib) used in renal cell carcinoma
Features of Cushings
Cushingoid facies Centripetal obesity Inter scapular fat pad Acne vulgaris Purpuric and petechial rashes Bruising Violaceous striae Proximal myopathy
Pathophysiology of Cushings Disease
Hyper secretion of ACTH from pituitary adenoma
Causes of Cushings Syndrome
ACTH dependent:
- Cushings disease (pituitary adenoma)
- Ectopic ACTH from SCLC or carcinoid
ACTH independent
- exogenous steroid administration
- adrenocortical adenoma/carcinoma
Investigations for cortisol excess
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
Complications of Cushings
Diabetes
Osteoporosis
HTN
Cataracts
Management of Cushings Syndrome
If adenoma - surgical transphenoidal/adrenalectomy
If adjunct needed then ketoconazole used to inhibit steroid genesis
Nelsons Syndrome
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
What is MEN1?
Autosomal dominant
- Pituitary adenoma (cushings)
- hyperparathyroidism
- pancreatic gastrinoma
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
Causes of Primary Adrenal Failure
Autoimmune (Addisons) TB Bilateral adrenelectomy Amyloidosis Haemochromatosis HIV Haemorrhagic infarcts - meningococcal Adrenal Mets Congenital Adrenal Hyperplasia
Diagnostic Tests for Adrenal Insufficiency
9 am serum cortisol
Synacthen test
Management of Adrenal Failure
Adrenal crisis - rapid steroid, fluid replacement and reversal of electrolyte abnormalities (low Na, high K)
Causes of Pituitary Failure
Pituitary mass Previous pituitary surgery/radiotherapy Infiltrative disease Pituitary infarct (Sheehans) Pituitary apoplexy Trauma/ SAH
Causes of Gynaecomastia
Hypogonadism Testicular tumour Chronic Liver Disease CKD Klinefelters Thyrotoxicosis Carcinoma of lung Drugs: digoxin, spironolactone Puberty
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
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
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
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
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
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
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
Eye involvement in RA
Episcleritis (painless) Scleritis (painful) Cataracts - steroids Sjogrens Extraoccular muscle tenosynovitis
Renal disease in RA
Membranous glomerulonephritis - gold
Mesangioproliferative glomerulonephritis
Acute tubulointerstitial nephritis - NSAIDs
Renal amyloidosis
Principles of Management of RA
MDT
General: education, physio, OT, bone protection, pneumovax
Analgesia
DMARDS
Biologicals - antiTNF (infliximab/adaluminab)
Corticosteroids
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
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