Hemophilia

is a bleeding disease thata occurs almost exclusively in males.In 85 percent of cases, it is caused by an abnormality or deficiency of Factor VIII; this type of hemophilia is called hemophillia A or classic hemophilia.

About 1 of every 10,000 males in the United States has classic hemophilia.In the other 15 percent of hemophilia patients , the bleeding tendency is caused by deficiency of Factor IX.Both of these factors are transmitted genetically by way of the female chromosome.Therefore, almost never will a woman have hemophilia because al least one of her two X chromosomes will have the appropriate genes.If one of her X chromosome is deficient, she will be a hemophilic carrier , transmitting the disease to half of her male offspring and transmitting the carrier state to half of her female offspring.

The bleeding trait in hemophilia can have various degree of severity, depending on the character of the genetic deficiency. Bleeding usually does not occur except after trauma, but in some patients, the degree of trauma required to cause severe aand prolonged bleeding maay be so mild that it is hardly noticeable.For instance, bleeding can often last days after extraction of a tooth.

Factor VIII hs two active components, a large component with a molecular weight in the millions and a smaller component with a molecular weight of about 230,000. The smaller component is most important in the intrinsic pathway for clotting and it is deficiency of this part of Factor VIII that cause classic hemophilia.Another bleeding disease with somewhat different characteristics, called von Willbrand's disease, results from loss of the large component.

When a person with classic hemophilia experiences severe prolonged bleeding, almost the only therapy that is truly effective is injection of purified Factor VIII. The cost of Factor VIII is high, and its availability is limited because it can be gathered only from human blood and only in extremely small quantities.

Treatment of hemophilia

ECG

What is ECG?

When impulses passes through heart the electrical current also spreads to the surrounding tissue and to surface of body.When electrodes are placed on the surface then it can record the electrical activity.This is known as ECG


ECG Paper:
ECG Paper: contains small and large squares.

Each small squares is 1mm and large square is 5mm

Time is measured along horizontal line and each small square is 0.04s and each large square is 0.2s.

Voltage is measured along vertical line and 10mm is equal to 1mV.

ECG paper moves at 25mm/s speed i.e. 1500sq/min



ECG leads
3 types of leads:

(HORIZONTAL PLANE LEADS)

1.Chest leads/precordial leads:V1,V2,V3,V4,V5, and V6.

(FRONTAL PLANE LEADS)

2.Bipolar leads/standard/Einthoven's Leads: I II III.

3.Augmented Unipolar leads: aVR, aVL ,aVF.


PLACEMENT OF LEADS

Augmented Leads:

aVR: Right arm

aVL: Left arm

aVF: Left foot


Chest leads:

V1: in 4th ICS at right sternal border .

V2:in 4th ICS at left sternal border .

V3:Mid way between V2 and V3

V4:5th ICS in left MCL (mid clavical line)

V5:Anterior axillary line in 5th ICS

V6:Mid axillary line in 5th ICS .....

ECG

What is ECG?

When impulses passes through heart the electrical current also spreads to the surrounding tissue and to surface of body.When electrodes are placed on the surface then it can record the electrical activity.This is known as ECG

ECG Paper:

ECG Paper: contains small and large squares.

Each small squares is 1mm and large square is 5mm

Time is measured along horizontal line and each small square is 0.04s and each large square is 0.2s.

Arthritis

What is arthritis? What causes arthritis?

Arthritis is a joint disorder featuring inflammation. A joint is an area of the body where two different bones meet. A joint functions to move the body parts connected by its bones. Arthritis literally means inflammation of one or more joints.

Arthritis is frequently accompanied by joint pain. Joint pain is referred to as arthralgia.

There are many types of arthritis (over 100 and growing). The types range from those related to wear and tear of cartilage (such as osteoarthritis) to those associated with inflammation resulting from an overactive immune system (such as rheumatoid arthritis). Together, the many types of arthritis make up the most common chronic illness in the United States.

The causes of arthritis depend on the form of arthritis. Causes include injury (leading to osteoarthritis), metabolic abnormalities (such as gout and pseudogout), hereditary factors, infections, and unclear reasons (such as rheumatoid arthritis and systemic lupus erythematosus).

Arthritis is classified as one of the rheumatic diseases. These are conditions that are different individual illnesses, with differing features, treatments, complications, and prognoses. They are similar in that they have a tendency to affect the joints, muscles, ligaments, cartilage, and tendons, and many have the potential to affect other internal body areas.

Symptoms of arthritis include pain and limited function of joints. Inflammation of the joints from arthritis is characterized by joint stiffness, swelling, redness, and warmth. Tenderness of the inflamed joint can be present.

Many of the forms of arthritis, because they are rheumatic diseases, can cause symptoms affecting various organs of the body that do not directly involve the joints. Therefore, symptoms in some patients with certain forms of arthritis can also include fever, gland swelling (lymph node), weight loss, fatigue, feeling unwell, and even symptoms from abnormalities of organs such as the lungs, heart, or kidneys.

Who is affected by arthritis?

Arthritis sufferers include men and women, children and adults. Approximately 350 million people worldwide have arthritis. Nearly 40 million people in the United States are affected by arthritis, including over a quarter million children!

More than 27 million Americans have osteoarthritis. Approximately 1.3 million Americans suffer from rheumatoid arthritis.

More than half of those with arthritis are under 65 years of age. Nearly 60% of Americans with arthritis are women.

regards by
Dr.M M ADNAN
contact id:adnan_dani12@yahoo.com

Parkinson's disease

(also known as Parkinson disease or PD) is a degenerative disorder of the central nervous system that often impairs the sufferer's motor skills, speech, and other functions.

Parkinson's disease belongs to a group of conditions called movement disorders. It is characterized by muscle rigidity, tremor, a slowing of physical movement (bradykinesia) and, in extreme cases, a loss of physical movement (akinesia). The primary symptoms are the results of decreased stimulation of the motor cortex by the basal ganglia, normally caused by the insufficient formation and action of dopamine, which is produced in the dopaminergic neurons of the brain. Secondary symptoms may include high level cognitive dysfunction and subtle language problems. PD is both chronic and progressive.

PD is the most common cause of chronic progressive parkinsonism, a term which refers to the syndrome of tremor, rigidity, bradykinesia and postural instability. PD is also called "primary parkinsonism" or "idiopathic PD" (classically meaning having no known cause although this term is not strictly true in light of the plethora of newly discovered genetic mutations). While many forms of parkinsonism are "idiopathic", "secondary" cases may result from toxicity most notably of drugs, head trauma, or other medical disorders. The disease is named after English physician James Parkinson, who made a detailed description of the disease in his essay: "An Essay on the Shaking Palsy

Signs and symptoms

Parkinson's Disease affects movement (motor symptoms). Other typical symptoms include disorders of mood, behaviour, thinking, and sensation (non-motor symptoms). Patients' individual symptoms may be quite dissimilar and progression of the disease is also distinctly individual

Motor symptoms:

The cardinal symptoms are:

• Tremor: normally 4-6 Hz tremor, maximal when the limb is at rest, and decreased with voluntary movement. It is typically unilateral at onset. This is the most apparent and well-known symptom, though an estimated 30% of patients have little perceptible tremor; these are classified as akinetic-rigid.
• Rigidity: stiffness; increased muscle tone. In combination with a resting tremor, this produces a ratchety, "cogwheel" rigidity when the limb is passively moved.
• bradykinesia/Akinesia: respectively, slowness or absence of movement. Rapid, repetitive movements produce a dysrhythmic and decremental loss of amplitude.
• Postural instability: failure of postural reflexes, which leads to impaired balance and falls.

Sleep:

• Excessive daytime somnolence
• Initial, intermediate, and terminal insomnia
• Disturbances in REM sleep: disturbingly vivid dreams, and rapid eye movement behavior disorder, characterized by acting out of dream content — can occur years prior to diagnosis.

Perception

• Impaired visual contrast sensitivity, spatial reasoning, colour discrimination, convergence insufficiency (characterized by double vision) and oculomotor control
• Dizziness and fainting; usually attributable orthostatic hypotension, a failure of the autonomic nervous system to adjust blood pressure in response to changes in body position
• Impaired proprioception (the awareness of bodily position in three-dimensional space)
• Reduction or loss of sense of smell (hyposmia or anosmia) - can occur years prior to diagnosis
• pain: neuropathic, muscle, joints, and tendons, attributable to tension, dystonia, rigidity, joint stiffness, and injuries associated with attempts at accommodation

Autonomic:

• Oily skin and seborrheic dermatitis^[10]
• Urinary incontinence, typically in later disease progression
• Nocturia (getting up in the night to pass urine) — up to 60% of cases
• Constipation and gastric dysmotility that is severe enough to endanger comfort and even health
• Altered sexual function: characterized by profound impairment of sexual arousal, behavior, orgasm, and drive is found in mid and late Parkinson disease. Current data addresses male sexual function almost exclusively.
• Weight loss, which is significant over a period of ten years.

Levodopa:

Stalevo for treatment of Parkinson's disease

The most widely used form of treatment is L-dopa in various forms. L-dopa is transformed into dopamine in the dopaminergic neurons by L-aromatic amino acid decarboxylase (often known by its former name dopa-decarboxylase). However, only 1-5% of L-DOPA enters the dopaminergic neurons. The remaining L-DOPA is often metabolised to dopamine elsewhere, causing a wide variety of side effects. Due to feedback inhibition, L-dopa results in a reduction in the endogenous formation of L-dopa, and so eventually becomes counterproductive.

Carbidopa and benserazide are dopa decarboxylase inhibitors. They help to prevent the metabolism of L-dopa before it reaches the dopaminergic neurons and are generally given as combination preparations of carbidopa/levodopa (co-careldopa) (e.g. Sinemet, Parcopa) and benserazide/levodopa (co-beneldopa) (e.g. Madopar). There are also controlled release versions of Sinemet and Madopar that spread out the effect of the L-dopa. Duodopa is a combination of levodopa and carbidopa, dispersed as a viscous gel. Using a patient-operated portable pump, the drug is continuously delivered via a tube directly into the upper small intestine, where it is rapidly absorbed. There is also Stalevo (Carbidopa, Levodopa and Entacapone).

Tolcapone inhibits the COMT enzyme, thereby prolonging the effects of L-dopa, and so has been used to complement L-dopa. However, due to its possible side effects such as liver failure, it's limited in its availability. A similar drug, entacapone has not been shown to cause significant alterations of liver function and maintains adequate inhibition of COMT over time.^[35]

Dopamine agonists:

The dopamine agonists bromocriptine, pergolide, pramipexole, ropinirole , piribedil, cabergoline, apomorphine, and lisuride are moderately effective. These have their own side effects including those listed above in addition to somnolence, hallucinations and/or insomnia. Several forms of dopamine agonism have been linked with a markedly increased risk of problem gambling. Dopamine agonists initially act by stimulating some of the dopamine receptors. However, they cause the dopamine receptors to become progressively less sensitive, thereby eventually increasing the symptoms.

Dopamine agonists can be useful for patients experiencing on-off fluctuations and dyskinesias as a result of high doses of L-dopa. Apomorphine can be administered via subcutaneous injection using a small pump which is carried by the patient. A low dose is automatically administered throughout the day, reducing the fluctuations of motor symptoms by providing a steady dose of dopaminergic stimulation. After an initial "apomorphine challenge" in hospital to test its effectiveness and brief patient and primary caregiver (often a spouse or partner), the latter of whom takes over maintenance of the pump. The injection site must be changed daily and rotated around the body to avoid the formation of nodules. Apomorphine is also available in a more acute dose as an autoinjector pen for emergency doses such as after a fall or first thing in the morning. Nausea and vomiting are common, and may require domperidone (an antiemetic).

regards by
Dr.M M ADNAN
contact id:adnan_dani12@yahoo.com