Session 26 Multi System Disorders 3 Bioscience Department · 2016-01-04 · Multi System Disorders 3 Bioscience Department ... damage: Blood vessels, central nervous system, heart,

BIOS222

Pathology and Clinical Science 2 & 3

www.endeavour.edu.au

Session 26

Multi System Disorders 3

Bioscience Department

© Endeavour College of Natural Health www.endeavour.edu.au 2

Session Learning Outcomes

At the end of the session, you should be able to:

o Outline the risk factors and aetiology of hypertension

o Detail the impact of hypertension on the target organs

o Describe the diagnostic measures, complications and management of hypertension.

o Understand the triggers, clinical presentation, diagnosis and management of chronic fatigue syndrome

o Understand the aetiology, clinical features, diagnosis and management of SLE

o Discuss the aetiology, clinical features, diagnosis and management of Marfan’s syndome


Session Plan

o Hypertension

o Chronic fatigue syndrome

o Systemic lupus erythematosus

o Marfan’s Disease


Hypertension

Chronic fatigue syndrome


Hypertension

o Definition: Hypertension is a condition in which arterial

BP is chronically elevated. BP occurs within a

continuous range, so cut-off levels are defined according

to their effect on patients' risk.

o Classification:

• Primary (essential) hypertension: It is the term applied

to 95% of cases in which no cause for hypertension

can be identified.

• Secondary hypertension: The elevation of blood

pressure results from an identifiable underlying

secondary cause, such as kidney or endocrine

disease.


Hypertension

o Risk factors for primary hypertension

• Family history

• Age

• Race

• Insulin resistance and hyperinsulinemia

• A high salt intake,

• Heavy consumption of alcohol

• Obesity,

• Lack of exercise

• Impaired intrauterine growth


Hypertensiono Aetiology for secondary hypertension:

• Pregnancy (pre-eclampsia)

• Renal disease

• Endocrine disease

• Drugs

• Coarctation of the aorta

o Clinical features:

• Asymptomatic initially

• Long term hypertension leads to target organ

damage: Blood vessels, central nervous system,

heart, kidney and retina


Hypertension

o Target organ damage:

• Blood vessels:

– Dilation of vessels due to smooth muscle atrophy

– Hyaline arteriosclerosis in the smaller arteries

• Heart:

– Left ventricular hypertrophy

– Angina or prior myocardial infarction

– Prior coronary revascularization

– Heart failure


Hypertension

o Target organ damage:

• CNS:

– Stroke, Carotid atheroma and TIA

– Subarachnoid haemorrhage, papilloedema

• Retina:

– ‘Cotton wool’ exudates with retinal ischaemia or

infarction

– Central retinal vein thrombosis

• Kidneys:

– Proteinuria and progressive renal failure


Hypertension

Hypertensive retinopathy showing

swollen optic disc, retinal

haemorrhages and multiple cotton

wool spots (infarcts)

Central retinal vein thrombosis

showing swollen optic disc and

widespread fundal

haemorrhage, commonly

associated with systemic

hypertension.


Hypertension

o Diagnosis:

• Urinalysis: for blood, protein and glucose

• Blood urea, electrolytes and creatinine

• Blood glucose

• Serum total and HDL cholesterol

• Thyroid function tests

• 12-lead ECG for left ventricular hypertrophy,

coronary artery disease


Hypertensiono Complications:

o Malignant’ or ‘accelerated’ phase hypertension:

• Accelerated microvascular damage

• Necrosis in the walls of small arteries and

arterioles

• Intravascular thrombosis

• Rapidly progressive end organ damage

• retinopathy (grade 3 or 4), renal dysfunction (especially

proteinuria) and/or hypertensive encephalopathy

• Left ventricular failure

• Death


Hypertensiono Management:

• Non-drug therapy

– Weight loss

– Reduction of alcohol and smoking

– Increase exercise

• Antihypertensive drugs

– Diuretics and Beta-blockers

– ACE inhibitors

– Angiotensin receptor blockers

– Calcium channel antagonists


Chronic Fatigue Syndrome

o Definition: It is characterised by excessive fatigue after

minimal physical or mental exertion, poor concentration,

dizziness, muscular aches and sleep disturbance.

o Triggering factors:

• Viral infection

• Immune disorders

• Stress

• Trauma

• Toxins


Chronic Fatigue Syndrome

o Clinical features: The fatigue of CFS is accompanied by

below characteristic illness symptoms lasting at least 6

months.

• Increased malaise following physical activity or

mental exertion

• Problems with sleep

• Difficulties with memory and concentration

• Persistent muscle pain

• Joint pain (without redness or swelling)

• Headache

• Tender lymph nodes in the neck or armpit

• Sore throat


Chronic Fatigue Syndromeo Diagnosis:

• Clinical symptoms

• A detailed medical history

• Physical and mental status examination

• Laboratory screening tests

o Management:

• Rest

• Education

• Emotional support

• Treatment of symptoms

• Overall management of general health


Systemic Lupus Erythematosus

Marfan’s Disease



(SLE)o Definition: SLE is a chronic inflammatory disease that

can affect virtually any organ system, including the

musculoskeletal system.

o Aetiology:

• Autoimmune attack

• Genetic factors

• Environmental factors



(SLE)o Clinical features:

• Musculoskeletal system: Arthralgias and polyarthritis,

tenosynovitis, rupture of the intrapatellar and Achilles

tendons, and avascular necrosis

• Skin: Malar or “butterfly” rash on the nose and

cheeks, hives, discoid rash, periungual erythema, nail

fold infarcts, and splinter hemorrhages, Hair loss,

Sun sensitivity

• Gastrointestinal: Mouth ulcers, Mesenteric vasculitis

with abdominal pain, bowel infarction or perforation



(SLE)o Clinical features: (cont.)

• Cardiovascular system: Pericarditis, myocarditis,

hypertension, Ischemic heart disease

• Lungs: pleural effusions or pleuritis

• Kidneys: Glomerulonephritis, Interstitial nephritis,

Nephrotic syndrome, proteinuria with resultant edema

in the legs and abdomen, and around the eyes



(SLE)o Clinical features: (cont.)

• Central nervous system: Strokes or hemorrhage,

seizures, depression and unnatural euphoria,

decreased cognitive functioning, confusion, and

altered consciousness

• Haematological system: hemolytic anaemia,

leukopenia, lymphopenia, or thrombocytopenia,

Lymphadenopathy, Raynaud’s phenomenon


Syste

mic

Lupus

Ery

them

ato

sus (

SLE

)



(SLE)

Severe secondary Raynaud’s phenomenon leading to

digital ulceration



(SLE)

Butterfly (malar) rash of systemic

lupus erythematosus, sparing the

nasolabial folds


SLE

o Diagnosis:

• Complete history, Physical examination

• Analysis of blood:

• RBC, WBC, Platelet count,

• ESR, CRP

• Autoantibodies: ANA

• Clinical criteria


SL

E


SLE

o Management:

• Avoid sun and UV light exposure and to employ sun

blocks

• Analgesics

• NSAIDs

• Hydroxychloroquine

• Corticosteroids

• Immunosuppressants


Marfan’s Syndrome

o Definition: This is a disorder of connective tissue

inherited as an autosomal dominant trait

o Aetiology:

• Genetic mutation: fibrillin gene on chromosome 15.


Marfan’s Syndrome


• Skeletal deformities: a long, thin body with

exceptionally long extremities spider fingers,

hyperextensible joints, kyphosis, and scoliosis

• Chest deformities: pectus excavatum, pigeon chest

deformity


Marfan’s Syndrome


• Eye disorders: Bilateral dislocation of the lens,

Myopia and predisposition to retinal detachment

• Cardiovascular defects: mitral valve prolapse,

progressive dilation of the aortic valve ring, and

weakness of the aorta and other arteries. Dissection

and rupture of the aorta, premature death.


Ma

rfa

n’s

Syn

dro

me

https://www.nlm.nih.gov/medlineplus/marfansyndrome.html

© Endeavour College of Natural Health www.endeavour.edu.au

32

Marfan’s Syndrome

o Arachnodactyly (“spider fingers”)

Image courtesy of Prof. Vardaxis.

Endeavour College


33

Marfan’s

Syndrome

Dissecting

aneurysm of the

aorta


Endeavour College


34

Marfan’s Syndrome

o CT scan of dissecting aneurysm of the aorta


Endeavour College


35

Marfan’s Syndrome

Subluxation of the lensImage courtesy of Prof. Vardaxis.

Endeavour College


Marfan’s Syndrome

o Diagnosis:

• Major and minor diagnostic criteria that include

skeletal, cardiovascular, and ocular deformities

• Chest X-ray,

• Echocardiography

• MRI or CT scan


Marfan’s Syndrome

o Management:

• No curative treatment

• β-blockers

• Elective replacement of the ascending aorta

• Pain medication and physiotherapy

• Genetic counseling


Reading and Resourceso Crowley LV, 2012, An Introduction to Human Diseases – Pathology and

Pathophysiology Correlations, 9th edn, Jones and Bartlett Learning

o Grossman SC & Porth CM 2014, Porth’s Pathophysiology- Concepts of

Altered Health States, 9th edn. Wolters Kluwer Health - Lippincott, Williams

& Wilkins

o Hinson, J, Raven, P & Chew, S 2010, The endocrine system: basic science

and clinical conditions, 2nd edn, Churchill Livingstone Elsevier, Edinburgh

o Jamison, JR 2006, Differential diagnosis for primary care: a handbook for

health care practitioners, 2nd edn, Churchill Livingstone Elsevier,

Edinburgh.

o Jarvis, C, 2012 Physical Examination & Health Assessment, 6th ed.,

Elsevier Saunders, Philadelphia.

o Kumar, P & Clark, M 2012, Kumar and Clark’s clinical medicine, 8th edn,

Saunders Elsevier, Edinburgh.

o Kumar, V, Abbas, AK & Aster, JC 2015, Robbins & Cotran pathologic basis

of disease, 9th edn, Elsevier Saunders, Philadelphia.


Reading and Resourceso Lee, G & Bishop, P 2009, Microbiology and infection control for health

professionals, 4th edn, Pearson Education, Frenchs Forest, NSW.

o McCance, KL, Heuther, SE, & Brashers, VL 2014, Pathophysiology: the

biologic basis for disease in adults and children, 7th edn, Elsevier.

o Michael-Titus, A, Revest, P & Shortland, P 2010, The nervous system: basic

science and clinical conditions, 2nd edn, Churchill Livingstone Elsevier,

Edinburgh

o Mosby’s dictionary of medicine, nursing and health professions 2013, 9th

edn, Elsevier, St. Louis, MO.

o Tortora, GJ & Derrickson, B 2014, Principles of anatomy and physiology,

14th edn, John Wiley & Sons, Hoboken, NJ.

o VanMeter, KC & Hubert, RJ 2014, Gould's pathophysiology for the health

professions, 5th edn, Elsevier, St Louis, MO.

o Walker, BR, Colledge, NR, Ralston, SH, & Penman, ID (eds) 2014,

Davidson’s principles and practice of medicine, 22nd edn, Churchill

Livingstone Elsevier, Edinburgh.


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Documents

Session 26 Multi System Disorders 3 Bioscience Department · 2016-01-04 · Multi System Disorders 3 Bioscience Department ... damage: Blood vessels, central nervous system, heart,