Endocrinology : Addison's disease: Etiology, Incidence, Signs & Symptoms & Acute Addisonian Crisis:

 Addison's disease: Etiology, Incidence, Signs & Symptoms & Acute Addisonian Crisis:

Addison's disease, also called adrenal insufficiency, is an uncommon disorder that occurs when the body doesn't produce enough of certain hormones.

Eiology :

In Addison's disease, the adrenal glands, located just above kidneys, produce too little cortisol and, often, too little aldosterone.

Incidence

Addison's disease occurs in all age groups and both sexes, and can be life-threatening.

Treatment involves taking hormones to replace those that are missing.

Symptoms

Addison's disease symptoms usually develop slowly, often over several months.

Often, the disease progresses so slowly that symptoms are ignored until a stress, such as illness or injury, occurs and makes symptoms worse.

Signs and symptoms may include:

Extreme fatigue.

Weight loss and decreased appetite.

Darkening of skin (hyperpigmentation).

Low blood pressure, even fainting

Salt craving

Low blood sugar (hypoglycemia)

Nausea, diarrhea or vomiting (gastrointestinal symptoms)

Abdominal pain

Muscle or joint pains

Irritability

Depression or other behavioral symptoms

Body hair loss or sexual dysfunction in women

Acute adrenal failure (addisonian crisis)

Sometimes the signs and symptoms of Addison's disease may appear suddenly.

Acute adrenal failure (addisonian crisis) can lead to life-threatening shock.

Emergency medical treatment should be sought if the patient if you experiences the following signs and symptoms:

Severe weakness

Confusion

Pain in lower back or legs

Severe abdominal pain, vomiting and diarrhea, leading to dehydration

Reduced consciousness or delirium

Low blood pressure

High potassium (hyperkalemia) and low sodium (hyponatremia)

Geriatrics : Alzheimer"s Disease

 Alzheimer's disease

Alzheimer's disease is an irreversible, progressive brain disorder that slowly destroys memory and thinking skills and, eventually, the ability to carry out the simplest tasks. 

In most people with the disease—those with the late-onset type—symptoms first appear in their mid-60s. 

Early-onset Alzheimer’s occurs between a person’s 30s and mid-60s and is very rare. 

Alzheimer’s disease is the most common cause of dementia among older adults.

Origins and history:

The disease is named after Dr. Alois Alzheimer. In 1906, 

Dr. Alzheimer noticed changes in the brain tissue of a woman who had died of an unusual mental illness. Her symptoms included memory loss, language problems, and unpredictable behavior. 

After she died, he examined her brain and found many abnormal clumps (now called amyloid plaques) and tangled bundles of fibers (now called neurofibrillary, or tau, tangles).

These plaques and tangles in the brain are still considered some of the main features of Alzheimer’s disease. 

Another feature is the loss of connections between nerve cells (neurons) in the brain.

 Neurons transmit messages between different parts of the brain, and from the brain to muscles and organs in the body. 

Many other complex brain changes are thought to play a role in Alzheimer’s, too.

This damage initially appears to take place in the hippocampus, the part of the brain essential in forming memories. 

As neurons die, additional parts of the brain are affected. By the final stage of Alzheimer’s, damage is widespread, and brain tissue has shrunk significantly.

Clinical Features:

Memory problems are typically one of the first signs of Alzheimer’s, though initial symptoms may vary from person to person. 

A decline in other aspects of thinking, such as finding the right words, vision/spatial issues, and impaired reasoning or judgment, may also signal the very early stages of Alzheimer’s disease. 

Mild cognitive impairment (MCI) is a condition that can be an early sign of Alzheimer’s, but not everyone with MCI will develop the disease.

People with Alzheimer’s have trouble doing everyday things like driving a car, cooking a meal, or paying bills. 

They may ask the same questions over and over, get lost easily, lose things or put them in odd places, and find even simple things confusing. 

As the disease progresses, some people become worried, angry, or violent.

How Long Can a Person Live with Alzheimer’s Disease?

The time from diagnosis to death varies—as little as 3 or 4 years if the person is older than 80 when diagnosed, to as long as 10 or more years if the person is younger.

Although treatment can help manage symptoms in some people, currently there is no cure for this devastating disease.

How Is Alzheimer's Disease Treated?

Alzheimer’s disease is complex, and it is unlikely that any one drug or other intervention will successfully treat it. 

Current approaches focus on helping people maintain mental function, manage behavioral symptoms, and slow down the symptoms of disease.

Treatment for Mild to Moderate Alzheimer’s

Medications called cholinesterase inhibitors are prescribed for mild to moderate Alzheimer’s disease. 

These drugs may help reduce some symptoms and help control some behavioral symptoms. 

The medications are Razadyne® (galantamine), Exelon® (rivastigmine), and Aricept® (donepezil).

Scientists do not yet fully understand how cholinesterase inhibitors work to treat Alzheimer’s disease, but research indicates that they prevent the breakdown of acetylcholine, a brain chemical believed to be important for memory and thinking. As Alzheimer’s progresses, the brain produces less and less acetylcholine; therefore, cholinesterase inhibitors may eventually lose their effect.

Treatment for Moderate to Severe Alzheimer’s

A medication known as Namenda® (memantine), an N-methyl D-aspartate (NMDA) antagonist, is prescribed to treat moderate to severe Alzheimer’s disease.

 This drug’s main effect is to decrease symptoms, which could allow some people to maintain certain daily functions a little longer than they would without the medication. 

For example, Namenda® may help a person in the later stages of the disease maintain his or her ability to use the bathroom independently for several more months, a benefit for both the person with Alzheimer's and caregivers.

The FDA has also approved Aricept®, the Exelon® patch, and Namzaric®, a combination of Namenda® and Aricept®, for the treatment of moderate to severe Alzheimer’s disease.

Namenda® is believed to work by regulating glutamate, an important brain chemical. When produced in excessive amounts, glutamate may lead to brain cell death. Because NMDA antagonists work differently from cholinesterase inhibitors, the two types of drugs can be prescribed in combination.

Geriatrics : FRAILTY

 FRAILTY

Frailty is a common geriatric syndrome that embodies an elevated risk of catastrophic declines in health and function among older adults. 

Frailty is a condition associated with ageing, and it has been recognized for centuries.

Causes

Sarcopenia

Sarcopenia is the degenerative loss of skeletal muscle mass, quality, and strength associated with aging.

The rate of muscle loss is dependent on exercise level, co-morbidities, nutrition and other factors. 

Sarcopenia can lead to reduction in functional status and cause significant disability from increased weakness. 

Osteoporosis

Osteoporosis is an age-related disease of bone that leads to an increased risk of fracture.

 In osteoporosis the bone mineral density (BMD) is reduced, bone microarchitecture is disrupted, and the amount and variety of proteins in bone is altered. 

Osteoporosis is defined by the World Health Organization (WHO) in women as a bone mineral density 2.5 standard deviations below peak bone mass (20-year-old healthy female average) as measured by DXA; the term "established osteoporosis" includes the presence of a fragility fracture.

Muscle weakness

Muscle weakness, also known as muscle fatigue, (or "lack of strength") refers to the inability to exert force with one's skeletal muscles. 

Weakness often follows muscle atrophy and a decrease in activity, such as after a long bout of bedrest as a result of an illness. 

There is also a gradual onset of muscle weakness as a result of sarcopenia – the age-related loss of skeletal muscle.

Mechanism

It has been suggested that the biological underpinnings of frailty are multifactorial, involving dysregulation across many physiological systems.

A proinflammatory state, sarcopenia,  anemia, relative deficiencies in anabolic hormones (androgens and growth hormone and excess exposure to catabolic hormones (cortisol), insulin resistance,glucose levels,compromised altered immune function, micronutrient deficiencies and oxidative stress are each individually associated with a higher likelihood of frailty. 

Diagnosis

The most widely used frailty scale consists of five items:

Unintentional weight loss >4.5 kg in the past year

Self-reported exhaustion

Less than 20th population percentile for grip strength

Slowed walking speed, defined as lowest population quartile on 4-minute walking test

Low physical activity such that persons would only rarely undertake a short walk

A healthy person scores 0; a very frail person scores 5. 

Compared to non-frail elderly people, people with intermediate frailty scores (2 or 3) are twice as likely to have post-surgical complications, spend 50% more time in the hospital, and are three times as likely to be discharged to a skilled nursing facility instead of to their own homes.

 Frail elderly patients (score of 4 or 5) have even worse outcomes, with the risk of being discharged to a nursing home rising to twenty times the rate for non-frail elderly people.

Linda Fried / Johns Hopkins Frailty Criteria

A popular approach to the assessment of geriatric frailty encompasses the assessment of five dimensions that are hypothesized to reflect systems whose impaired regulation underlies the syndrome. These five dimensions are:

Unintentional weight loss,

Exhaustion,

Muscle weakness,

Slowness while walking, and

Low levels of activity.

Rockwood Frailty Index

Another notable approach to the assessment of geriatric frailty  is Rockwood Frailty Index ,in which frailty is viewed in terms of the number of health "deficits" that are manifest in the individual, leading to a continuous measure of frailty. This approach was developed by Dr. Rockwood and colleagues at Dalhousie University.

Surgical outcomes

Frail elderly people are at significant risk of post-surgical complications and the need for extended care. Frailty more than doubles the risk of morbidity and mortality from surgery and cardiovascular conditions.

Assessment of older patients before elective surgeries can accurately predict the patients' recovery outcomes  

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Thyrotoxic crisis : Investigations & Management :

Investigations :

ECG will show Sinus Tachycardia but Atrial fibrillation or other arrhythmias may be present.

Thyroid function tests would show Hyperthyroidism.

Thyrotoxic crisis is mainly a clinical diagnosis.

There are no specific lab investigations to diagnose thyrotoxic crisis.

The levels of thyroid hormones maybe the same as in uncomplicated hyperthyroidism.

Treatment should be started immediately as soon as clinical  diagnosis of thyrotoxic crisis has been made.

And time should not be wasted in confirming the diagnosis by lab investigations.

Management:

Thyrotoxic crisis is a life threatening condition with mortality of upto 20 – 30 % reported.

Aside from basic resuscitation give specific treatment for thyrotoxic crisis on clinical suspicion.

Hyperthyroidism :

The patient in thyrotoxic crisis requires both of the following :

Propylthiouracil or carbimazole :

Propylthiouracil is the preferred drug as it both blocks the further synthesis of thyroid hormones and inhibits peripheral conversion of T4 to T3.

However it is often not immediately available on the wards whereas carbimazole usually is.

If propylthiouracil is available ,give a loading dose of 600 mg to 1 Gm orally or via nasogastric tube and then 200 mg every 6 hours.

If PTU is not available, carbimazole 20 mg shoul be given followed by 20 mg three times a day.

Lugol”s iodine : Saturated solution of potassium iodide :

5 drops every 5 hours beginning 4 hours after starting PTU / Propylthiouracil / carbimazole not before , as thyroid hormone stores may be increased) to inhibit further rrelease of thyroxine.

Supportive measures particular to Thyrotoxic crisis :

Hyperpyrexia:

Peripheral cooling measures and Paracetamol

Aspirin should not be used as it can displace thyroid hormones from its binding sites.

Tachycardia :

Give propranolol 1 mg iv repeated every 20 minutes as necessary upto total of 5 mg or give 40 – 80 mg PO four times a day.

Be cautious in case of patient having Cardiac failure.

Esmolol is a short acting beta blocker and can be used as an infusion for immediate management of sympathetic overactivity.

Atrial fibrillation :

Consider digitalization but note that higher doses of digoxin than usual may be needed due to relative resistance to the drug.

Steroids : hydrocortisone 200 mg iv then 100 mg every 6 hours or dexamethasone 2 mg po four times daily .

Treat possible precipitating causes :

Start broad spectrum antibiotics if there is any suggestion of infection.

Endocrinology : Thyrotoxic crisis

Thyrotoxic crisis :

Thyroid storm, also referred to as thyrotoxic crisis, is an acute, life-threatening, hypermetabolic state induced by excessive release of thyroid hormones (THs) in individuals with thyrotoxicosis.

Clinical features of thyrotoxic crisis / Storm:

Weight loss

Heat intolerance

Sweating

Palpitations

Tremor

Diarrhoea

Anxiety

Agitation

Irritability

Precipitating causes of Thyrotoxic crisis:

Withdrawal of Antithyroid drug therapy

Infection

Radio iodine treatment

Iodinated contrast dyes

Thyroid surgery

Child birth

Clinical Examination :

Following features would be observed :

General :

Agitation

Anxiety

Restlessness

Tremor

Warm skin and moist

Hyperpyrexia

Cardiovascular compromise :

Sinus Tachycardia : Hewart beat greater than 140 bpm in thyroid crisis.

Fast Atrial fibrillation or supraventricular tachy cardia is also common.

Neurological features :

Altered consciousness

Frank psychosis

Delirium

Seizures and

Coma may be present.

Findings suggestive of Thyroid pathology include the following :

Signs of Graves disease :

Exophthalmos

Lid retraction

Lid Lag

Goitre :

Smooth Vs nodular

Any associated bruit

Vitiligo associated with Auto immune Thyroid disease.

Geriatrics : Capacity Assessment

What is capacity?

Capacity means the ability to use and understand information to make a decision, and communicate any decision made.

A person lacks capacity if their mind is impaired or disturbed in some way, which means they're unable to make a decision at that time.

All adults are presumed to have sufficient capacity to decide on their own medical treatment, unless there's significant evidence to suggest otherwise.

Examples of how a person's brain or mind may be impaired include:

Mental health conditions – such as schizophrenia or bipolar disorder

Dementia

Severe learning disabilities

Brain damage – for example, from a stroke or other brain injury

Physical or mental conditions that cause confusion, drowsiness or a loss of consciousness

Intoxication caused by drugs or alcohol misuse

Someone with such an impairment is thought to be unable to make a decision if they cannot:

Understand information about the decision

Remember that information

Use that information to make a decision

Communicate their decision by talking, using sign language or any other means.

Geriatric Assessments : Abbreviated Mental Test Score / AMTS

 Geriatric Assessments : Abbreviated Mental Test Score / AMTS

This is a set of ten questions designed to give the examiner a rough idea of the mental state of the patient.

Total score : 10

The questions, and their conditions for one point each, are:

1) Age - must be correct.

2) Time, without looking at a timepiece, correct to the nearest hour.

3) 42, West Street - given as at test of immediate memory and retested at the end.

4) Month - must be exact.

5) Year - exact, except in Jan or Feb when last year is OK.

6) Name of place, or type of place or town ("in hospital" is insufficient .

7) Date of birth - exact

8) Start of WWI, exact - 1914

9) Name of the present monarch.

10) Counting backwards from 20 to 0, can prompt to 18 and patient may self-correct or hesitate.

Check the address recall from point No 3.'

Assessing / Interpretation of the AMTS :

The patient scores one point for each correct answer in the mental test score as long as the criteria are fulfilled, e.g. the time is given correct to the nearest hour.

A score of 8 to 10 is normal - note that it is very easy for a patient not to know the time.

A score of 7 is probably abnormal, and any less is definitely abnormal.

The score is invalid if the patient:

is delirious

has an affective disorder.

Notes:

There is evidence that clinicians use variants on these questions when undertaking a 10-point mini-mental test score.

The above AMTS is based on the format taught to Oxford University medical students .

The stated AMTS varies from the original by questioning about month when the original mini-mental test asked about recognition of two people - score if roles of two people correctly recognised - for example, doctor and nurse