N-Trivia

Muscular Dystrophy (MD)
Principles of Sterility – Principles 1 & 2
Diabetes Insipidus

Posted: 20 Nov 2010 09:00 PM PST

Definition

Muscular dystrophy is a collective term denoting a group of genetic disorders that results to the progressive degeneration of the skeletal muscles. Individuals affected with this disorder apparently lack information on their genes that prevents their body from making a protein (merosin) which is necessary for muscle contractions.

Types

Congenital Myotonic Dystrophy (CMD)

This type of muscular dystrophy is inherited through an autosomal dominant trait pattern. The disease begins in the utero and the newborn may have severe muscle weakness (myotonia) at birth. The degeneration of the muscle progresses until respiratory movement becomes very hard and difficult. Many of these infants die before 1 year of age because they cannot sustain respiratory function. In rare cases, congenital myotonic muscular dystrophy can cause learning and intellectual disabilities in affected children.

Fascioscapulohumeral Muscular Dystrophy (FSMD)f
fascioscapulohumeral muscular dystrophy

Fascioscapulohumeral muscular dystrophy is inherited as dominant trait, where the defect is carried in the chromosome 4. Symptoms are apparent during late school-age or adolescent period, about 10 years of age. It affects both sexes and this type of muscular dystrophy usually affects the muscles of the face and shoulder, giving its name. The predominant manifestation of FSMD is facial weakness making a child unable to whistle and wrinkle his or her forehead, close eyes tightly and raise arms. The progression of the disorder is slow, hence allowing a normal-life span.

Pseudohypertrophic Muscular Dystrophy (Duchenne's Muscular Dystrophy)

This is the most common form of muscular dystrophy and is inherited as a sex-linked recessive trait. Duchenne's muscular dystrophy affects only boys where the symptoms usually become apparent by 3 years of age. A problem with the gene that makes a protein called dystrophin causes this type of MD. Without dystrophin muscles cannot keep their shpe and strength. At about 10-12 years of age children with this disease needs a wheelchair. Later on, respiratory muscles become weak necessitating the help of a ventilator to breathe. Affected individuals usually die in their late teens or early adulthood.

Clinical Manifestations

Progressive muscular weakness
Delayed meeting of motor milestones
Waddling gait
Walking on toes
Frequent falls
Gower's sign (to stand, affected children press their hands against their ankles, knees and thighs)
Hyperthrophied calf muscles
Poor balance
Scoliosis of the spine
Fracture of long bones
Inability to walk (late stages)

Diagnostic tests

Muscle biopsy
History and physical findings
Electromyography
Enzyme analysis (increased concentration of serum creatine phosphokinase)

Management

There is no cure for muscular dystrophy. Treatments include physical and speech therapy, orthopedic devices, surgery and medications.

Encourage to remain ambulatory for as long as possible.
gower's sign
Active and passive daily ROM exercise program.
Splinting and bracing ( to maintain lower extremity stability and avoid contractures)
Low-calorie, high-protein diet to avoid excessive weight gain (ambulation becomes more difficult if the child is overweight)
Increase fiber and fluids in diet (to prevent constipation.)
Stool softeners if needed.
Support groups for parents to assist them with coping (Muscular Dystrophy Association)

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Principles of Sterility – Principles 1 & 2

Posted: 20 Nov 2010 08:57 PM PST

Definition of terms

Sterile means free of microorganisms including the pores while asepsis means absence of microorganisms that cause disease. Sterile techniques are methods employed inside the operating room to prevent contamination of organisms throughout the surgical procedure. It is very important for nurses to know and understand the principles governing sterility to promote safety of the patient during operation.

When are sterile techniques used or applied?

Preparation for an invasive procedure
In preparation of the sterile team to handle sterile supplies and contact to the surgical site (gowning, gloving and scrubbing)
Skin preparation and draping of the patient
Sterility maintenance throughout the operation

Principles of Sterility

Principle Number 1: Only sterile items are used within the sterile field.

Drapes, basins, sponges are obtained from a stock room with sterile packages. The instruments used are sterilized and are placed in a sterile table. Any person who holds the sterile equipments should be very cautious to maintain sterility. One important consideration in implementing sterility is this: IF YOU ARE IN DOUBT ABOUT THE STERILITY OF A CERTAIN OBJECT, CONSIDER IT UNSTERILE. Any suspected or known unsterile items should not be placed the sterile field.

Any sterile package found in an unsterile or contaminated area is considered unsterile.
If the actual timing or sterilization procedure is undetermined and the nurse is unsure about the sterilization process, the equipments sterilized with the suspected procedure are considered contaminated.
A sterile table which has been touch or rubbed accidentally by an unsterile person or vice versa is no longer considered sterile.
If the packaging material is broken or has missing pieces it is no longer sterile.
Microorganisms can enter a packed sterile package when it is damp or wet. Thus, damp packages are unsterile.
A sterile package dropped on a floor is considered contaminated.

Principle Number 2: Sterile persons are gown and gloved.

When wearing a gown, the considered sterile area is the part where you can see in front down to the level of the sterile field. Thus, gowns are only considered sterile in front of the chest, sleeves above the elbow to the cuffs down to the level of the sterile field. Certain methods should be employed in the OR:

Gowning is not done on the sterile table to avoid dripping water onto the sterile equipments. Gloving and self-gowning should be done in a distinct sterile surface.
Stockinette cuffs of the gowns are absorbent and may retain moisture, thus making it a suitable area for bacteria or microorganisms to thrive in. because of the said principle, stockinette cuffs should be inserted beneath the sterile gloves.

Diabetes Insipidus

Posted: 20 Nov 2010 08:45 PM PST

Definition

Diabetes insipidusis an endocrine disorder characterized by the inability of the kidney to conserve water. This leads to excessive urination and thirst of the person affected. The word diabetes comes from the Greek word diabainein, which means to stand with legs apart (as in urination) or to go through. Insipidus is originated from a Latin word meaning without taste. The urine excreted in diabetes insipidus is tasteless (because it has relatively low sodium content) whilst in Diabetes Miletus a sweet urine (glucose content) is excreted.

Types

There are different types of Diabetes Insipidus, each has a different cause. The most common types are the following:

Central Diabetes Insipidus – caused by the deficiency of antidiuretic hormone (ADH). This type of diabetes insipidus is also called pituitary diabetes insipid or neurogenic diabetes insipidus.
Nephrogenic Diabetes Insipidus – caused an insensitivity of the kidneys to the ADH.

Review of Related Anatomy and Physiology

The antidiuretic hormone (ADH) or vasopressin is secreted by the posterior pituitary or neurohypophysis. It targets the kidney to conserve or retain water in the body. This hormone is very helpful in regulating the fluid volume and urine output by decreasing the amount of urine excreted and increasing water reabsorption. Through the said mechanism, the extracellular fluid volume rises resulting in a vasoconstrictor effect (increased BP). In cases where the vascular volume drops more ADH is released.

Anxiety, trauma and pain all contributes to the release of ADH from the neurohypophysis. When a person changes position (from lying to standing) and when the body is exposed to high temperatures, ADH secretion is also augmented. When the level of ADH falls, minimal or no water reabsorption will occur thereby, increasing the urine output and might lead to dehydration.

Pathophysiology

Causes

Pituitary or Neurogenic or Central Diabetes Insipidus

Head trauma
Brain tumor
Surgical ablation of the pituitary gland
Irradiation of the pituitary gland
Infection to the CNS (meningitis, encephalitis)
Tumors (metastatic disease)

Nephrogenic Diabetes Insipidus

Hypokalemia
hypercalcemia
Medications (lithium, demecleocyclin, amphotericin B)

Clinical Manifestations

Polyuria
Polydipsia
Diluted urine with a specific gravity of 1.001-1.005
Signs of dehydration

Diagnostic Evaluation

Radiography, CT scan or ultrasound of the skull
Urinalysis

Treatment

Pituitary or Neurogenic or Central Diabetes Insipidus

The goals of the treatment are focused on the following:

Fluid replacement
Vasopressin replacement with Desmopressin (DDVAP), which is a synthetic vasopressin without the vascular effects of the natural ADH.
Correct the underlying disease process

Nephrogenic Diabetes Insipidus