King saud university nursing collage master degree 1431-1432H

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King saud university nursing collage master
degree1431-1432H

• Nursing management for acute disease in
• Respiratory system
• Endocrine system
• Hematology system
• Immunology system
• Done by maha alonazi

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Objective

• At the end of the lecture the master student well
  be able to
• Know the most common of acute respiratory disease
  .
• Identify nursing management for RD .
• Know the most common of acute endocrine disease .
• Identify nursing management for ED .
• Know the most common of acute hematology
  immunology disease .
• Identify nursing management for HD ID .

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Outline

• RESPIRATORY DISEASE
• Anatomy of respiratory system .
• Croup.
• Acute epiglottitis
• Broncholitis .
• Pneumonia.
• Aspiration pneumonia .
• ARDS
• Asthma.
• ENDOCRINE DISEASE
• Anatomy of endocrine system .

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• Diabetes Mellitus
• Diabetic ketoacidosis .
• Diabetes Insipidus.
• SIADH .
• Hyperthyroidism.
• Hypothyroidism.
• Gigantism
• HEMATOLOGY IMMUNOLOGY DISEASE
• Anatomy of immune hemato system .

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• Anemia
• Sickle cell disease
• DIC
• Hemophilia
• Leuckemia
• Thalasmia

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Pulmonary System
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Anatomy of respiratory system

• Upper airway .
• Lower airway .
• Thoracic cavity .

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Upper Airway

• Ideally above larynx is the upper airway
• Practically speaking Nose, Larynx (Glottic
  opening and vocal
• cords) and trachea (Up to Carina).
• The upper airway is responsible for
• warming, humidifying and filtering air before
• it reaches the trachea.
• Nose
• Pharynx
• Larynx

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Lower Airway (Lung)

• Trachea
• Lung
• Conducting Airways
• Gas exchange units (Alveoli)

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Thoracic Cavity

• Diaphragm
• The chest wall

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Spectrum of Resp. diseases

• Upper AW - Stridor, croup, laryngomalacia
• Lower AW - Asthma, bronchiolitis
• Lung Parenchymal - Pneumonia, Pulmonary edema
• Pleural - Empyema, Pneumothorax

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When will you suspect UAW dis.?

• Stridor
• Neck retraction
• Suprasternal retraction
• Sniffing position

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Acute Laryngotracheobronchitis (Croup)

• Definition inflammatory swelling of the
  submucosa in the subglottic area trachea
  ,bronchi ,bronchioles
• Commonest cause of acute stridor usually occure
  for child from age 3 month to 5 yrs .
• causes Viruses Parainfluenza. RSV .
• Sing symptoms
• barking cough.
• Hoarseness.
• stridor.
• Low fever.

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(Croup) management

• Mild Stridor at rest, cough
  - Minimal handling
• Moderate to severe Stridor at rest.
  - Racemic epinephrine nebulization
  Dexamethazone IV or IM, also oral or Nebulized
  Budesonide
• Severe Impending respiratory failure
  Intubation for 2-3 days. Improves in a week.
• Majority may not need intubation

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X-ray Neck in croup Sharpened pencil appearance
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Nursing management

• provide medication as order .
• Provide cool humidified oxygen as needed
• Check vital sign .
• Monitor pulse oximetry.
• Minimal handling.
• Provide quit environment .
• Enteral feeding may be considered in pt. with
  respiratory distress .

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• Definition Is severe life threatening rapidly
  progressive infection of the epiglottis
  aryepiglottic folds surrounding tissue .
• Usually occurs in children aged 2-6 yrs but can
  occurs any any age.
• Caused before the use of the HiB vaccine
• ( haemophilus influanzae type B) was the moust
  commonly identified cause of acute epiglotittis
  ,the usual cause in the vaccinated child is now
  streptococcus pyogenes, S.pneumoniae ,or
  staphylococcus aureus .

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• Sing symptom
• Sudden onset of high fever.
• dysphagia.
• drooling,.
• muffled voice.
• soft stridor.
• inspiratory retractions.

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Normal Lateral neck
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Acute epiglottitis - Management

• Intubation by a skilled person
• IV Ceftriaxone
• O2 and other supportive management
• Usually extubatable in 3-4 days
• Antibiotics for 10 days.

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Nursing management

• Close monitoring .
• Arm restraint to prevent self extubation .
• Decreased movement of the ETT in the larynx .
• Deliver oxygen as necessary .

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Croup Epiglottitis

• Age Younger infants
• No fever
• Harsh stridor
• Parainfuenza virus
• Usually no antibiotics, no intubation

• Age older children
• High fever, toxic
• Soft stridor, Drooling, muffled
• H influenza(Bacteria)
• Needs antibiotics and intubation

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DO and DONT in UAW dis.

• Allow the patient in his position of comfort
• Do not separate the child from mother
• Do not force the child to lie down
• Do not make the child cry?
• Do not send the child for X-ray without
  accompanied by medical team
• Continuously monitor for the need for intubation.

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Broncholitis

• Definition acute inflammatory disease of the
  lower tract that result in obstruction of small
  airways .
• Causeas viral infection .
• Sing symptom
• Cough.
• Wheezing .
• Prolong expiration.
• Irritability .
• Low grade fever .
• Tachypnea .
• Retraction .

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Nursing management

• Maintained fluid nutritional requirement
• Monitor child closely for respiratory failure.
• provide medication as order .
• Provide oxygen as needed or M.V if respiratory
  failure ,hypoxemia or apnea are developed .
• Check vital sign .
• Monitor pulse oximetry
• Prevent nosocomial infection by good HW.

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Pneumonia

• Pneumonia is infection of the lung caused most
  often by bacteria or viruses .
• Mode of transmission
• 1-Inspiration.
• 2-Aspiration.
• 3-Circulation.
• Bypass of nasal defense
• Pulmonary aspiration (CNS, GER, TEF)
• Abnormal secretions or mucociliary clearance
• Underling chronic disease/nutrition
• Defect in the immune system

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• Sign symptom
• Chills, fever and cough
• Stuffy nose
• Irritability
• Resp distress expiratory grunting, nasoflaring,
  retraction, tachypnea, tachycardia.
• Cyanosis, air hunger, and occasionally apnea

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Nursing management

• Monitor V\S ,ABG level ,oxygen saturation .
• Administered nebulization oxygen at
  concentration as prescribed .
• Placed in high fowler position .
• Rendered chest physiotherapy .
• Encourage pt. to deep breathing cough .
• Encourage pt. to increased fluid intake to
  liquefy secretion .
• Assess lung sound .
• Administered antibiotic as ordered .
• Avoid contact with people with URTI .

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Aspiration pneumonia

• Definition inflammation caused by pulmonary
  aspiration of gastric fluid produced direct
  injury to the mucosal surface of the respiratory
  tract .
• Causes see table
• Sing symptom
• Cough ,fever .
• acute dyspnea.
• wheezing , crackles or absent breathing sound in
  the effected lobes ,
• cyanosis, retraction , tachypnea .

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Causes of Aspiration Pneumonia
Drugs, alcohol, anesthesia, seizures, CNS disorders Altered level of consciousness
Traceal or esophageal abnormalities, ETT, tracheostomy Altered anatomy or function of trachea or esophagus
Loss of normal reflexes which prevent aspiration of stomach contents, GER (worse with neuro or anatomical impairment) Altered function of swallow or esophageal motility
Inhalation of toxic substances Inhalation injury
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Nursing management

• Monitor V\S ,ABG level ,oxygen saturation .
• Administered nebulization oxygen at
  concentration as prescribed .
• Placed in high fowler position .
• Rendered chest physiotherapy .
• Encourage pt. to deep breathing cough .
• Encourage pt. to increased fluid intake to
  liquefy secretion .
• Assess lung sound .
• Administered antibiotic as ordered .

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ARDS

• Definition acute lung injury noncardiogenic
  pulmonary edema .
• Causes
• Gastric aspiration.
• Toxic inhalation .
• Pulmonary infection .
• FBA .

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• Sing symptom
• Stage 1 (1-2 day) mild tachypnea,
  hypoxemia,anxiety or restlessness.
• Stage2 (2-3 day) cyanosis in room air
  ,tachycardia,retraction .
• Stage 3(3-10 day )symptom change from distress
  to failure (inability to oxygenate
  ventilate,alveolar collapse desaturation ,high
  oxygen requirement .
• Stage 4 (after 10 day )development of
  pulmonary fibrosis progressive impairment of
  oxygenation are observed .

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Nursing management

• Encourage coughing deep breathing in awake pt.
• Frequent position change with CPT as needed .
• Reduce abdominal distention by NGT if necessary .
• Check vital sign .
• Monitor pulse oximetry .
• Do VBG ABG analysis .
• Provide supplemental oxygen for pt. with adequate
  ventilation .
• Provide medication such as sedative ,analgesic
  ,neuromuscular blocking agents ,bronchodilator as
  ordered .
• Provide high calories high protein diet.

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Asthma (chronic disease )

• Definition obstructive pulmonary disease
  characterized by airway inflammation with mucosal
  edema,thick secretion that cause plugging
  hyperreactivity of the tracheobronchial tree that
  result in bronchospasm of the smooth muscle .
• Causes inflamatory mediators are thought to
  stimulate the vagus nerve ( cholinergic
  stimulation )causing smooth muscle constriction
  increased production of mucus.

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• Sing symptom
• Tachycardia.
• Tachypnea.
• Cynosis.
• Expiratory wheezing in severe case.
• Inspiratory expiratory wheezing .
• Hypoxemia .

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Nursing management

• Monitor pattern of breathing V\S at regular
  interval .
• Assess S S of respiratory distress .
• Administer oxygen nebulization as indicated .
• Assess lung sound .
• Monitor pulse oximetry ABG level as indicated .
• Encourage rest in between activity to prevent
  fatigue encourage deep breathing .

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Endocrine System
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• 1- Endocrine glands secrete hormones directly
  into the bloodstream ( adrenal , pancreas
  ,thyroid glands)
• 2-Exocrine glands secrete biochemical substances
  that are released into ducts to be delivered to
  target organs ( salivary ,sweat glands )

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• Major glands
• 1-Hypothalamus-pituitary complex
  .(ACTH,TSH,ADH,Oxytocin )
• 2-Thyroid gland.(T3,T4)
• 3-Parathyroid gland.(PTH)
• 4-Adrenal gland.(sex hor. Cortisol,epinephrine,nor
  epinephrine)
• 5-Islets of langerhans in the pancreas.insullin-be
  ta cell, glucagon-alpha cell, somatostatin-delta
  cells
• 6-Gonads.estrogen,progesterone,testosterone .

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Fig 1. Pituitary Hormones and their target organs.
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Diabetes Mellitus

• Definitions
• Type I Insulin-dependent mellitus (IDDM)
• It is autoimmune disease that result in the
  T-cell-mediated destruction of the beta
  pancreatic cells .it is the most common form of
  diabetes in infants children requires insulin
  replacement therapy .
• Type II Non-insulin-dependent diabetes mellitus
  (NIDDM)
• Is associated with obesity ,strong family history
  older age .it is not autoimmune process but
  instead due to insulin resistance enough insulin
  is produced to prevent ketoacidosis. it can
  treated by oral hypoglycemia agents ,
  diet.exercise .

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Diabetes Mellitus

• Diabetic ketoacidosis
• Is the absence of insulin cellular uptake of
  glucose is inhibited glucose production by the
  liver is increased glucose utilization
  decreased resulting in hyperglycemia .
• Etiology
• is related to inadequate endogenous insulin
  secretion . Acute stress, infection ,trauma ,
  high dose of steroide .

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Table 1. Signs and symptoms
Symptoms
Hyperglycemia Metabolic acidosis (gap acidosis)
Dehydration, shock Kussmaul breathing
Cardiac arrhythmia Sodium imbalance
Potassium imbalance
Mental status changes Hyperosmolality Ketonuria Glucosuria
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Management

• Fluid
• Electrolytes
• Insulin
• Monitoring

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Nursing management

• Monitor V\S blood sugar level .
• Provide 3 regular diabetic meals with in between
  snacks as tolerated .
• Check weight daily \weekly as indicated .
• Observed for circulation on feet .
• Monitor intake output .
• Check urine for protein ketones .
• Monitor S\S of hypo hyperglycemia .
• Administered insulin regimen as prescribed .

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Table 3. Clinical observation in dehydration
9 (90mL/kg) 6 (60mL/kg) Older Child 3 (30mL/kg)
15 (150mL/kg) 10 (10mL/kg) Infant 5 (50mL/kg) Examination
Severe None Clammy Parched/cracked Sunken None Sunken Lethargic/obtunded Increased Feeble/impalpable gt3 sec Anuric Moderate Tenting Dry Dry Deep set Reduced Soft Irritable Slightly increased Weak 2 sec Decreased Mild Normal Normal Moist Normal Present Flat Consolable Normal Normal Normal Normal Dehydration Skin turgor Skin (touch) Buccal mucosa/lips Eyes Tears Fontanelle CNS Pulse rate Pulse quality Capillary refill Urine output
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Table 4. Insulin preparation
Duration (hr) Peak (hr) Onset (hr) Insulin
3-5 6-8 1-3 0.5-1.5 0.17-0.33 0.25-0.5 Rapid acting NovoLog Humalog
3-10 1-5 0.5-1 Short acting Regular
10-24 12-24 4-14 4-14 1-4 1-4 Intermediate acting NPH Lente
18-36 8-30 4-10 Long acting Ultralente
20-24 No pronounced peak 5 Basal Lantus
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Syndrome of Inappropriate antidiuretic hormone

• Characterized by inappropriate, excessive
  secretion of ADH
• Occurs in the face of low serum Na and low serum
  osmolality
• Clinical signs and symptoms are secondary to
  increased blood volume and hyponatremia.

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Pathophysiology of SIADH
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Etiology and risk factors

• Conditions associated with SIADH
• Meningitis, Head trauma, Cerebral tumors,
  Cerebral hemorrhage, Chornically ill or
  malnourished, Spinal surgery

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Signs and symptoms

• Low urine output and the absence of hypovolemia
• Headache, confusion, lethargy, altered LOC, coma
  or seizures
• Nausea and vomiting
• Weight gain
• Filling pressures (normal or increase)
• Hypertension and tachycardia

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Management

• Serum sodium normalized over 24 to 48 hours
• Monitoring intake output , specific gravity,
  serum electrolytes and osmolality, daily
  weights.

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Nursing management

• Monitor intake output
• Monitor specific gravity.
• Monitor serum electrolytes and osmolality
• daily weights.

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Diabetes Insipidus

• Characterized by a decrease in both urine
  concentrating ability and water conservation.
• Types of DI
• Central
• Common form in children and critical care
  environment, deficiency of ADH due to failure of
  the hypothalamus to synthesize or failure of the
  posterior pituitary to secrete or both .

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• Nephrogenic
• Characterized by normal secretion of ADH by the
  posterior pituitary but the distal tubule
  collecting ducts in the kidney are resistant to
  the effects of ADH .

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Diabetes Insipidus

• Etiologyy
• Central
• Pituitary and head trauma, CNS infections,
  cerebral edema, cerebral hemorrhage or infarct,
  congenital, familial, idiopathic
• Nephrogenic
• Chronic renal disease, polycystic kidney disease,
  pregnancy, starvation

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Signs and symptoms

• Large quantity of dilute urine
• Hypernatremia
• Serum hyperosmolality
• Polyuria
• Urine osmolality
• Polydipsia
• Signs of dehydration
• Altered mental status

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Diabetes Insipidus

• Management
• Central
• Use fluid or ADH replacement therapy
• Nephrogenic
• Difficult to treat, Sodium-restricted diet

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Nursing management

• Monitor intake and output.
• Daily weights.
• Frequent hemodynamic.
• Neurologic assessments.
• Assess signs of dehydration.
• Assess urine electrolytes result .
• Monitor specific gravity.

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Differentiation between DI and SIADH
DI SIADH
Serum sodium gt145 mEq/L lt135 mEq/L
Urine sodium Low High
Serum osmolality gt300 mOsm/kg lt275 mOsm/kg
Urine osmolality lt300 mOsm/L gt800mOsm/L
Urine specific gravity lt1.005 gt1.020
Urine output High Low
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Thyroid gland

• It is the largest endocrine gland. It lies in
  front of the neck it is formed two lobes
  connected by an isthmus which crosses the
  trachea.
•  

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Thyroid gland

• The thyroid gland secretes the following
  hormones
• 1-Thyroxine (tetraiodothyronine T4) and
  (triiodothyronine, T3) both hormones are iodine
  containing amino acid.
• 2-Calcitonin it is a calcium lowering hormone,
  polyeptide in nature, secreted from
  parafollicular cells of thyroid gland. It lowers
  the plasma calcium level.

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• Actions of T4 and T3
•  
• Increase oxygen consumption and enhances the
  metabolism of all the body tissues.
• Increase protein synthesis in the kidney, muscle
  and liver. They stimulate growth. They increase
  activity of most of enzymes in the cells.
• Increase the rate of glucose absorption, uptake,
  and utilization by tissues.

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• Increase the metabolism of fat from its stores
  and use its fat for production of energy and it
  decreases the level of plasma cholesterol.
• Accelerates the heart rate and it is an
  erythropoietic factor.
• It increases appetite, the motility and
  secretions of gastro intestinal tract.
• Are important for normal development and
  maturation of Central Nervous System.

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Hyperthyroidism

• Hyperthyroidism(Graves disease) Overactivity of
  the Thyroid Gland, In healthy people, the thyroid
  makes just the right amounts of two hormones, T4
  and T3.
• Hyperthyroidism is a condition caused by the
  effects of too much thyroid hormone on tissues of
  the body.

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Causes

• There are several causes of hyperthyroidism. Most
  often, the entire gland is overproducing thyroid
  hormone. This is called Graves' disease.
• Less commonly, a single nodule is responsible for
  the excess hormone secretion. We call this a
  "hot" nodule. 
• Thyroiditis (inflammation of the thyroid) can
  also cause hyperthyroidism.

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symptoms and signs

• Palpitations
• Heat intolerance
• Nervousness
• Insomnia
• Breathlessness
• Increased bowel movements
• Light or absent menstrual periods
• Fatigue
• Fast heart rate
• Trembling hands
• Weight loss
• Muscle weakness
• Warm moist skin
• Hair loss
• Enlargement of the thyroid gland (goiter).
• Protrusion of the eye balls due to excessive
  growth and degeneration of tissues behind the
  eye.

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Treatment

• Anti-thyroid Drugs
• Radioactive Iodine Treatment
• Surgical Removal of the Gland or Nodule

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Nursing management

• Monitor V\S .
• Assess skin turgor mucous membrane for signs of
  fluid deficit .
• Monitor intake output .
• Monitor weight daily .
• Provide high calorie food .
• Provide quiet ,calm ,cool environment
• Inspected skin frequently

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hypothyroidism

• condition leading to the deficiency in the
  production of thyroid hormone.
• Iodine deficiency is the most common cause of
  hypothyroidism worldwide but it can be caused by
  any number of other causes such as several
  conditions of the the thyroid gland, or less
  commonly, the pituitary gland or hypothalamus.

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• Before Puberty (cretinism)
• Mal development or complete absence of gland in
  a new born gives the signs and symptoms of
  cretinism at the age of about 6 months when the
  mothers milk becomes deficient in thyroxin, it
  is characterized by
• Mental retardation due to defective of nerve
  fibers, which lead to delayed mile stones as
  (sitting walking and speech).
• Retarded growth, the height is less than one
  meter.
• Intolerance to cold weather and decrease BMR.
• Abdominal muscle weakness, with protruded viscera
  and protruded enlarged tongue.
• Infantile sexual organs.
• Thick skin.
• Raised serum cholesterol level.

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• After Puberty Myxoedema
• This due to atrophy of the thyroid glands or due
  to thyroidectomy for any reason. It is
  characterized by
• Slow mental activity.
• Increase sensitivity to cold, the BMR decreased
  (30 to 40).
• The hair is coarse and sparse, dry skin, firm non
  pitting oedema.
• Sexual hypo function in female, there is
  irregular cycle, in male there is impotence.
• Loss of appetite and decrease motility of the
  gastrointestinal tract which leads to
  constipation.
• Deficient erythropoiesis leading to anaemia.

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Sing symptom

• Fatigue.
• Cold intolerance increased sensitivity to cold
• Constipation
• Depression.
• muscle cramps and joint pain
• Thin, brittle fingernails
• Coarse hair
• Decreased sweating
• Dry, itchy skin
• Weight gain and water retention
• Bradycardia.
• Thinning of the outer third of the eyebrows (sign
  of Hertoghe).
• Abnormal menstrual cycles.

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Causes

• Iodine deficiency is the most common cause of
  hypothyroidism worldwideor a deficiency in
  stimulating hormones from the hypothalamus or
  pituitary.
• Congenital hypothyroidism is very rare accounting
  for approximately 0.2
• Hypothyroidism can result from postpartum
  thyroiditis, a condition that affects about 5 of
  all women within a year of giving birth.
• Hypothyroidism can also result from sporadic
  inheritance, sometimes autosomal recessive.

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classification

• Primary Thyroid gland The most common forms
  include Hashimoto's thyroiditis (an autoimmune
  disease) and radioiodine therapy for
  hyperthyroidism.
• Secondary Pituitary gland Occurs if the
  pituitary gland does not create enough
  thyroid-stimulating hormone (TSH) to induce the
  thyroid gland to produce enough thyroxine and
  triiodothyronine. Although not every case of
  secondary hypothyroidism has a clear-cut cause,
  it is usually caused by damage to the pituitary
  gland, as by a tumor, radiation, or
  surgery.Secondary hypothyroidism accounts for
  less than 5 or 10 of hypothyroidism cases.

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• Tertiary Hypothalamus Results when the
  hypothalamus fails to produce sufficient
  thyrotropin-releasing hormone (TRH). TRH prompts
  the pituitary gland to produce thyroid-stimulating
  hormone (TSH). Hence may also be termed
  hypothalamic-pituitary-axis hypothyroidism. It
  accounts for less than 5 of hypothyroidism
  cases.

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Treatment

• Hypothyroidism is treated with the levorotatory
  forms of thyroxine (levothyroxine) (L-T4) and
  triiodothyronine (liothyronine) (L-T3).

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Nursing management

• Monitor vital signs .
• Monitor ECG tracing to detect arrhythmias .
• Provide warm environment.
• Provide high fiber diet .
• Administered prescribed stool softener .
• Administered medication as prescribed .
• Encourage increase intake of fluids .
• Provided warm drink .

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Growth Hormone (Somatotropin)

• HGH is synthesized and secreted from the anterior
  pituitary gland Effects of growth hormone on the
  tissues of the body can generally be described as
  anabolic (building up).
• In addition to increasing height in children and
  adolescents, growth hormone has many other
  effects on the body
• Increases calcium retention, and strengthens and
  increases the mineralization of bone
• Increases muscle mass through sarcomere
  hyperplasia
• Promotes lipolysis

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• Increases protein synthesis
• Stimulates the growth of all internal organs
  excluding the brain
• Plays a role in homeostasis
• Reduces liver uptake of glucose
• Promotes gluconeogenesis in the liver
• Contributes to the maintenance and function of
  pancreatic islets
• Stimulates the immune system

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• High levels of growth hormone may indicate
• Acromegaly
• Gigantism
• Growth hormone resistance
• Pituitary tumor.
• Low levels of growth hormone may indicate
• Growth hormone deficiency.
• Hypopituitarism.

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excessive growth hormone secretion

• Gigantism is the result of excessive growth
  hormone secretion that begins in young children
  or adolescents. It is a very rare disorder,
  usually resulting from a tumor of somatotropes.
  One of the most famous giants was a man named
  Robert Wadlow. He weighed 8.5 pounds at birth,
  but by 5 years of age was 105 pounds and 5 feet 4
  inches tall. Robert reached an adult weight of
  490 pounds and 8 feet 11 inches in height. He
  died at age 22.
• Acromegaly results from excessive secretion of
  growth hormone in adults.

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growth hormone deficiency

• The effects of growth hormone deficiency vary
  depending on the age at which they occur. In
  children, growth failure and short stature are
  the major manifestations of GH deficiency, with
  common causes including genetic conditions and
  congenital malformations. It can also cause
  delayed sexual maturity.

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• Dwarfism
• It is deficiency of growth hormone in young age.
  The rate of development is decreased so the child
  who has reached the age of 10 years has the body
  of 4 years. The dwarf is short and mentally
  normal.
•  

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Treatment

• For excessive secration drugs like octreotide
  (somatostatin agonist) and bromocriptine
  (dopamine agonist) can be used to block GH
  secretion because both somatostatin and dopamine
  negatively inhibit GHRH-mediated GH release from
  the anterior pituitary.
• For deficiency injection of GH.

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Nursing management

• provide medication as order .
• Measure weight height .
• Check weight daily \weekly as indicated .
• Assess GH level .
• Educate pt. family about the important of
  follow up .

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Adrenal gland Dysfunction

• Hypo function of adrenal cortex
• (Addison disease)
• It is due to adrenal cortical insufficiency. It
  is characterized by
• Loss of appetite, vomiting, and diarrhea i.e.
  gastro intestinal disturbances.
• Muscle weakness due to decrease of anabolic
  effect of androgens.

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• Decrease sodium and increase potassium in the
  blood. (Water moves intracellular, ----- the
  blood volume decreases and hypotension occur.
• Hypoglycemia.
• Decrease sexual function.
• The Bronze hyper pigmentation of the skin due to
  high level of ACTH secondary to low level of
  glucocorticoids. ACTH has melanocytic stimulating
  activity.

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Cushing's syndrome

• Cushing's syndrome is a hormone disorder caused
  by high levels of cortisol in the blood.
• This pathology was described by Harvey Cushing
  in 1932.The syndrome is also called
  Itsenko-Cushing syndrome, hyperadrenocorticism or
  hypercorticism)

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• Hyper function of Adrenal Cortex
• (Cushing's Syndrome)
•  It is characterized by
• Wasting and weakness of muscle, (catabolic
  effect).
• Increase sodium and water retention in the blood
  (leading to oedema and hypertension with decrease
  level of plasma potassium).
• Redistribution of fat, the extremities are thin,
  there are accumulation of fat in the abdominal
  wall, face (MOON) and the upper back (buffalo
  trunk).
• Hyperglycemia.
• Gonadal disturbances.
• The skin is thin.

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• the hypothalamus releases corticotropin-releasing
  hormone (CRH), which stimulates the pituitary
  gland to release adrenocorticotropin (ACTH). ACTH
  travels via the blood to the adrenal gland, where
  it stimulates the release of cortisol. Cortisol
  is secreted by the cortex of the adrenal gland
  from a region called the zona fasciculata in
  response to ACTH. Elevated levels of cortisol
  exert negative feedback on the pituitary, which
  decreases the amount of ACTH released from the
  pituitary gland. Strictly, Cushing's syndrome
  refers to excess cortisol of any etiology.

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• One of the causes of Cushing's syndrome is a
  cortisol secreting adenoma in the cortex of the
  adrenal gland. The adenoma causes cortisol levels
  in the blood to be very high, and negative
  feedback on the pituitary from the high cortisol
  levels causes ACTH levels to be very low.
  Cushing's disease refers only to hypercortisolism
  secondary to excess production of ACTH from a
  corticotrophic pituitary adenoma. This causes the
  blood ACTH levels to be elevated along with
  cortisol from the adrenal gland. The ACTH levels
  remain high because a tumor causes the pituitary
  to be unresponsive to negative feedback from high
  cortisol levels.
• Cushing's Syndrome was also the first autoimmune
  disease identified in humans.

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causes

• Exogenous vs. endogenous
• Hormones that come from outside the body are
  called exogenous.
• hormones that come from within the body are
  called endogenous.

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• The most common cause of Cushing's syndrome is
  exogenous administration of glucocorticoids
  prescribed by a health care practitioner to treat
  other diseases (called iatrogenic Cushing's
  syndrome). This can be an effect of steroid
  treatment of a variety of disorders such as
  asthma and rheumatoid arthritis, or in
  immunosuppression after an organ transplant.

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• Administration of synthetic ACTH is also
  possible, but ACTH is less often prescribed due
  to cost and lesser utility.

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• Endogenous Cushing's syndrome results from some
  derangement of the body's own system of secreting
  cortisol. Normally, ACTH is released from the
  pituitary gland when necessary to stimulate the
  release of cortisol from the adrenal glands.
• In pituitary Cushing's, a benign pituitary
  adenoma secretes ACTH. This is also known as
  Cushing's disease and is responsible for 70 of
  endogenous Cushing's syndrome.

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• In adrenal Cushing's, excess cortisol is produced
  by adrenal gland tumors, hyperplastic adrenal
  glands, or adrenal glands with nodular adrenal
  hyperplasia.
• Finally, tumors outside the normal
  pituitary-adrenal system can produce ACTH that
  affects the adrenal glands. This final etiology
  is called ectopic or paraneoplastic Cushing's
  syndrome and is seen in diseases like small cell
  lung cancer .

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Sing symptom

• rapid weight gain particularly of the trunk and
  face "moon face with sparing of the limbs
  (central obesity ).
• A common sign is the growth of fat pads along the
  collar bone and on the back of the neck (buffalo
  hump) .
• Other symptoms include hyperhidrosis (excess
  sweating),.
• Telangiectasia (dilation of capillaries).

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• Thinning of the skin (which causes easy bruising
  and dryness particularly the hands and other
  mucous membranes, purple or red striae (the
  weight gain in Cushing's syndrome stretches the
  skin, which is thin and weakened, causing it to
  hemorrhage) on the trunk, buttocks, arms, legs or
  breasts, proximal muscle weakness (hips,
  shoulders).
• Hirsutism (facial male-pattern hair growth).
• Baldness and/or cause hair to become extremely
  dry and brittle.

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• In rare cases, Cushing's can cause hypercalcemia
  which can lead to skin necrosis (osteoporosis ).
  The excess cortisol may also affect other
  endocrine systems and cause, for example,
  diabetes mellitus
• menstrual disorders such as amenorrhea in women
  and decreased fertility in men infertility due to
  elevations in androgens.
• signs include polyuria (and accompanying
  polydipsia), persistent hypertension (due to
  cortisol's enhancement of epinephrine's
  vasoconstrictive effect) and insulin resistance
  (especially common in ACTH production), leading
  to hyperglycemia (high blood sugar) and insulin
  resistance which can lead to diabetes mellitus.

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• Patients frequently suffer various psychological
  disturbances, for example, euphoria , depression
  and anxiety.

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Haematology and Immunology
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Cell differentiation from stem cells
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Anatomy and Physiology

• 1- Red blood cells (RBCs, erythrocytes)
• Develop from erythroid precursor cells
• Reticulocytes represent the stage of maturation
• Mature RBCs have a life span of 120 days
• Hemoglobin large, complex, iron-containing
  protein. Responsible for the RBCs oxygen-carrying
  abilities.
• RBC transport oxygen from the lungs to the
  tissues

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• Hgb A, normal adult hemoglobin
• Hemoglobin F ( fetal Hgb) present in large
  concentrations in the fetus, decrease after birth
  and minimally present in children and adults.
• Normal RBC production requires iron, folic acid
  and Vit B12.

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The immune system

• 2-Leukocytes (white blood cells)
• group that serve to protect the organism.
• Neutrophils the largest component of total
  circulating WBCs, 60 70.
• Originate and mature in bone marrow
• Polymorphonucleated (PMNs or poly) mature form
  of neutrophil
• Band unsegmented appearing nucleus
• Neutrophilia increased number of circulating
  neutrophils

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Anatomy

• Neutropenia decreased number of circulating
  neutrophils (lt1500/mm3)
• WBCs mature in bone marrow for 10 days
• Eosinophils 2 5 of the circulating WBC.
• Eosinophilia increased number of eosinophils
• Eosinopenia decreased number of eosinophils
• Basophils smallest portion of granulocytes, 1
  of WBC
• Basophilia increased number of circulating
  basophils
• Basopenia decreased number of circulating
  basophils

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• Mononuclear phagocytes
• Monocytes 3 8 of circulating WBCs
• Monocytosis increase in the number of circulating
  monocytes
• Monocytopenia decrease in the number of
  circulating monocytes
• Macrophages have long life span.

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• Lymphocytes (lymphoid lineage) primary immune
  cells
• Lymphocytes 10 40 of the ciculating WBCs
• Lymphocytosis increase number of circulating
  lymphocytes
• Lymphopenia decrease in number of circulating
  lymphocytes
• T lymphocytes or T cells 65 85 of all
  lymphocytes
• Clusters of differentiation (CD)
• Helper T cells (CD4)
• Suppressor T cells (CD8)
• Cytotoxic or killer T cells (CD8)

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Lymph nodes of the body
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Anatomy

• B Lymphocytes or B cells constitute up to 35
• NK cells constitute 5 8 of the total
  lymphocyte count
• Memory cells have CD. Programmed to recognize
  the original invading microorganism on subsequent
  invasions.
• WBC
• Functions
• Defense protect the bodys internal environment
  from nonself antigens or microorganisms
  invasion.
• Assists the immune system in discriminating
  self from nonself or altered self
• Second line of defense involves the inflammatory
  response, phagocytosis .

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• 3-Platelets (thrombocytes)
• Megakaryoblasts mature into megakaryocytes
• Characteristics minute, round or oval discs.
• Platelet count 150, 000 to 400, 000/mm3.
• Maintain normal hemostasis vascular integrity
  when a blood vessel wall is injured.

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Anatomy

• 4-Plasma factors
• Procoagulants aka plasma clotting factors.
  Promotes coagulation.
• Anticoagulants inhibit coagulation
• Circulating anticoagulants antithrombin III,
  protein C, protein S
• Coagulation depends on the balance between the
  procoagulants and the anticoagulants

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Nomenclature for coagulation factors
Factor Synonym
I Fibrinogen
II Prothrombin
III Tissue thromboplastin
IV Calcium
V Proaccelerin
VI Not assigned
VII Proconvertin
VIII Antihemophilic factor (AHF)
IX Plasma thromboplastin component (Christmas factor
X Stuart factor (Stuart-Prower factor)
XI Plasma thromboplastin antecedent (PTA)
XII Hageman factor
XIII Fibrin-stabilizing factor (FSF)
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Anemia

• Anemia is defined as a reduction below normal in
  the volume of red blood cells volume or
  hemoglobin concentration.
•  
• Classification of Anemias
• Anemias can be classified by two
  approaches
• Etiologic factors.
• Morphologic factors.

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• I. Etiologic Factors
• Anemias resulting from impaired or decreased
  production
• The production of the RBCs and Hb can be
  impaired by
• Nutritional deficiency (e.g.folate, iron, B12).
• Bone marrow failure (e.g. irradiation,
  malignancy).
• Interference with bone marrow activity (e.g.
  infection, chronic diseases).

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• Anemias resulting from accelerated destruction of
  RBCs (Hemolytic)
• There are causes that can shorten the
  life span of the cell, which can be
• Corpuscular Causes
• Defect in cell membrane Hereditary
  erythrocytosis.
• Defect in cell enzyme G6PD deficiency.
• Defect in cell hemoglobin Sickle cell anemia,
  Thalassemia.
• Extracorpuscular causes
• Immune blood transfusion.
• Non- immune - burns. - Snake venoms.
• Anemias resulting from excessive blood loss
• Acute blood loss (bleeding)
• Alteration in vital signs doesnt occur unless
  20 or more of blood lost occur. Shock occurs
  with losses of 40 of blood volume.
• Chronic blood loss (occult bleeding,
  Ancylostoma)

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• II. Morphologic Factors
• The characteristic changes in RBCs
  size, shape, color is described as below
• Cell size for example, normocytes (normal),
  microcytes (smaller than normal), or macrocytes
  (larger than normal).
• Shape- irregularly shape RBCs for example,
  poikilocytes (irregularly shaped cell),
  spherocytes (globular cells), and drepanocytes
  (sickle cells).
• Staining characteristics or color Reflects the
  hemoglobin concentration for example,
  normochromic (Sufficient or normal amount) or
  hypochromic (reduced amount).

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Sing symptom

• General Manifestation
• Dyspnea on exersion.
• Muscle weakness.
• Easy fatigability.
• Frequent resting.
• Shortness of breath.
• Poor sucking (infants).
• Pale skin waxy pallor seen in severe anemia.
• Tachycardia.
• Cardiac dilatation gradually develops.

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• Central nervous system manifestations
• Headache.
• Dizziness.
• Night headache.
• Irritability.
• Slowed thought processes.
• Decreased attention span.
• Apathy.
• Depression.
• Shock blood loss anemia
• Poor peripheral perfusion.
• Skin is moist and cool.
• Low blood pressure.
• Increased heart rate.

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• Anemias Caused by Defect in
• Hemoglobin Formation
• Sickle cell anemia (SCA).
• Thalassemia.

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Sickle Cell Anemia

• This disorder is characterized by severe chronic
  hemolytic disease resulting from premature
  destruction of the brittle poorly deformable
  erythrocytes. In which normal adult hemoglobin A
  (Hgb A) is partly or completely replaced by
  abnormal sickle hemoglobin (Hgb S).
• It is a genetically determined (autosomal
  reccesive) disease.

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Sickle cell Anemia

• sickle cell anemia, the number of red blood cells
  is low because sickle cells don't last very long.
  Sickle cells usually die after only about 10 to
  20 days. The bone marrow can't make new red blood
  cells fast enough to replace the dying ones.

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• Normal red blood cells are disc-shaped and look
  like doughnuts without holes in the center. They
  move easily through your blood vessels. Red blood
  cells contain an iron-rich protein called
  hemoglobin. This protein carries oxygen from the
  lungs to the rest of the body.
• Sickle cells contain abnormal hemoglobin called
  sickle hemoglobin or hemoglobin S. Sickle
  hemoglobin causes the cells to develop a sickle,
  or crescent shape.
• Sickle cells are stiff and sticky. They tend to
  block blood flow in the blood vessels of the
  limbs and organs. Blocked blood flow can cause
  pain, serious infections, and organ damage.

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Causes

• Sickle cell anemia is inherited from both
  parents. Sickle cell disease is much more common
  in people of African and Mediterranean descent.
  It is also seen in people from South and Central
  America, the Caribbean, and the Middle East.
• Someone who inherits the hemoglobin S gene from
  one parent and normal hemoglobin (A) from the
  other parent will have sickle cell trait. People
  with sickle cell trait do not have the symptoms
  of true sickle cell anemia.

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Sing symptom

• Severe sudden chest pain .
• Splenomegaly in the young children .
• Impaied growth development .
• Attacks of abdominal pain.
• Bone pain.
• Breathlessness.
• Delayed puberty.
• Fatigue.
• Fever.
• Paleness.
• Rapid heart rate.
• Ulcers. on the lower legs (in adolescents and
  adults).
• Yellowing of the eyes and skin (jaundice).

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• The main objectives of medical treatment are
• Bed rest to minimize energy expenditure and
  oxygen use.
• Hydration through oral and intravenous therapy.
• Electrolyte replacement.
• Analgesics for severe abdominal and joint pain.
• Blood replacement to treat anemia.
• Antibiotics to treat any existing infection.

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Nursing management

• Administer blood products as ordered .
• Improve oxygenation .
• Prevent iatrogenic anemia .
• Provide adequate nutrition with high iron .
• Administered iron replacement as ordered .
• Monitor vital signs .
• Assess pt. for palpitation , SOB, dizziness.
• Administered parenteral vitamin B12 as
  prescribed .
• Assess knowledge of present condition .reinforced
  importance of therapeutic home management OPD
  follow up .
• Assessed dietary intake . Provided diet high in
  iron .
• Advised to rest in between activities .
• Assessed level of fatigue , sleeping pattern ,
  and activity that causes fatigue

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• To reduce sickle cell crises, take the following
  precautions
• To prevent oxygen loss, avoid
• Demanding physical activity (especially if the
  spleen is enlarged)
• Emotional stress
• Environments with low oxygen (high altitudes,
  nonpressurized airplane flights)
• Smoking
• Known sources of infection

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• To make sure you're getting enough fluids
• Avoid too much exposure to the sun
• Have fluids on hand, both at home and away
• Recognize signs of dehydration.
• To avoid infection
• Consider having the child wear a Medic Alert
  bracelet
• Have the child vaccinated as recommended by the
  health care provider
• Share the above information with teachers and
  other caretakers, when necessary

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• They should take supplements of folic acid
  (essential for producing red blood cells) because
  red blood cells are turned over so quickly.
• Antibiotics and vaccines are given to prevent
  bacterial infections, which are common in
  children with sickle cell disease.
• Blood transfusions are used to treat a sickle
  cell crisis.

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• Nursing Diagnosis
• 1. Altered nutrition, less than body requirements
  related to reported inadequate iron intake,
  knowledge deficit regarding iron reach foods.
• Goal
• Patient will receive adequate supply of
  iron.
• Intervention
• Provide diet counseling to care giver, especially
  in regard to food source of iron (e.g. meat,
  liver, fish, egg yolks, green leafy vegetable,
  nuts, whole grains, infants cereals and dry
  cereals).
• Expected Outcomes
• Child receive at least minimum dailyrequirement
  of iron.

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• Nursing Diagnosis
• 2-Risk for injury related to abnormal
  hemoglobin, decreased ambient oxygen, and
  dehydration.
• Patient Goal 1
• Will maintain adequate tissue
  oxygenation.
• Nursing Intervention
• Explain measures to minimize complications
  related to physical exertion and emotional stress
  to avoid additional tissue oxygen needs.
• Prevent infection.
• Avoid low-oxygen environment.
• Expected Outcome
• Child avoids situations that reduce
  tissue oxygenation.
•  
• Patient Goal 2
• Will maintain adequate hydration.

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• Nursing Intervention
• Calculate recommended daily fluid intake (1600
  ml/m3/day) and base childs fluid requirements on
  this minimum amount (specify) to ensure adequate
  hydration.
• Increase fluid intake above minimum requirements
  during physical exercise/emotional stress and
  during crisis to compensate for additional fluid
  needs.
• Give parents written instructions regarding
  specific quantity of fluid required to encourage
  compliance.
• Encourage child to drink to encourage compliance.
• Teach family signs of dehydration to avoid delay
  in rehydration therapy.
• Stress importance of avoiding overheating as
  source of fluid loss.
• Expected Outcome
• Child drinks an adequate amount of fluid
  and shows no signs of dehydration.

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• Nursing Diagnosis
• 3. Anxiety /fear related to diagnostic
  procedures, transfusion.
• Goal
• Patient, family will become
  knowledgeable about the disorder.
• Intervention
• Prepare the child for tests.
• Remain with the child during tests and initiation
  of transfusion.
• Explain purpose of blood components.
• Expected Outcomes
• Child and family display minimal anxiety.
• Child and family demonstrate an understanding of
  the disorders, diagnostic tests and treatment.
•  

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• Nursing Diagnosis
• 4. Activity intolerance related to generalized
  weakness, diminished oxygen delivery to tissues.
• Goal
• Patient will receive adequate rest.
• Intervention
• Provide diversional play activities that promote
  rest and quite but prevent boredom and
  withdrawal.
• Choose appropriate roommate of similar age and
  interests who requires restricted activities.
• Plan nursing activities to provide sufficient
  rest.
• Assist with activities requiring exertion.
• Expected Outcomes
• Child plays and rests quietly and engages in
  activities appropriate to capabilities.

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Thalassemia

• Thalassemia is a blood disorder passed down
  through families an inherited autosomal recessive
  blood disease in which the body makes an abnormal
  form of hemoglobin, the protein in red blood
  cells that carries oxygen.
• The disorder results in excessive destruction of
  red blood cells, which leads to anemia.

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Causes

• Hemoglobin is made of two proteins Alpha globin
  and beta globin. Thalassemia occurs when there is
  a defect in a gene that helps control production
  of one of these proteins.
• There are two main types of thalassemia
• 1-Alpha thalassemia occurs when a gene or genes
  related to the alpha globin protein are missing
  or changed (mutated).
• 2-Beta thalassemia occurs when similar gene
  defects affect production of the beta globin
  protein.

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• Alpha thalassemias occur most commonly in persons
  from southeast Asia, the Middle East, China, and
  in those of African descent.
• Beta thalassemias occur in persons of
  Mediterranean origin, and to a lesser extent,
  Chinese, other Asians, and African Americans

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• There are many forms of thalassemia. Both alpha
  and beta thalassemia include the following two
  forms
• Thalassemia major
• Thalassemia minor
• Thalassemia major occurs if the baby receive
  the defective gene from both parents.
• Thalassemia minor occurs if the baby receive the
  defective gene from only one parent.
• Persons with this form of the disorder are
  carriers of the disease and usually do not have
  symptoms.

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• ß-Thalassemia Major(Cooleys Anemia)
• It is most common form of thalassemia in the
  world. More common in countries around
  Mediterranean Sea. It is inherited as autosomal
  genetic disorder of both genes controlling ß-
  chain synthesis (homozygous).
• Normal postnatal hemoglobin (Hgb A) is composed
  of two a and two ß- polypeptide chains, in
  thalassemia major there is a partial or complete
  deficiency in the synthesis of ß- chain of Hgb
  molecule.
• Consequently, there is a tremendous increase in
  the synthesis of a- chains and ? chains.

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Sign symptom

• The most severe form of alpha thalassemia major
  causes stillbirth (death of the unborn baby
  during birth or the late stages of pregnancy).
• Children born with thalessemia major (Cooley's
  anemia) are normal at birth, but develop severe
  anemia during the first year of life.

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Sings symptom

• Unexplained fever.
• Poor feeding.
• Markedly enlarged spleen.
• Headache.
• Bone pain.
• Decreased exercise tolerance.
• Anorexia.
• Frequent epistaxis.
• Hemochromatosis.
• Hemosiderosis.

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• Other Features
• Small stature.
• Delayed sexual maturation.
• Protrusion of the abdomen (hepato- spleno-
  megaly).
• Bone Change (older children if untreated)
• Enlarged head.
• Prominent frontal and parietal bosses.
• Flat or depressed bridge of the nose.
• Enlarged maxilla.
• Protrusion of the lip and upper central incisors
  and eventual malocclusion.

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Treatment

• Patients with thalassemia minor usually do not
  require any specific treatment unless they have
  very low HB.
• Treatment for patients with thalassemia major
  includes chronic blood transfusion therapy.
• iron chelation therapy to remove excess iron
  from the body.
• splenectomy .
• Bone marrow transplant may help treat the disease
  in some patients especially children.

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Therapeutic Management

• Blood Transfusion.
• Chelation Therapy

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• Nursing Diagnosis
• Anxiety / fear related to diagnostic procedures,
  transfusion.
• Activity intolerance and comfort altered related
  to hematomas in tissues and joints.
• High risk for bleeding after minor hematomas
  related to deficiency of coagulation factor.
• Pain related to bleeding in joints.
• Impaired physical mobility related to hemorrhage
  in joints and tissues.
• Altered family process related to child with
  chronic illness.
•  

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• Planning
• The objectives of nursing care can be divided
  into immediate needs and long-term goals
• Prevent bleeding.
• Recognize and control bleeding.
• Prevent crippling effect of bleeding.
• Support family and prepare for home care.
• Identify persons at risk.

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• Implementation
• 1. Prevent Bleeding
• Prophylactic administration of AHF.
• Appropriate exercises to strengthen muscles and
  joints and to allow age appropriate activity.
• To prevent oral bleeding, some readjustment in
  terms of dental hygiene may be related to,
  minimize trauma to the gums such as use of a
  water-irrigating device, softening the toothbrush
  in warm water before brushing.
• A peripheral fingerstick is better for blood
  samples and subcutaneous route is substituted for
  IM whenever possible.
• Neither aspirin nor any aspirin- containing
  compound should be used. Acetaminophen (Tylenol)
  is a suitable aspirin substitute, especially for
  use during control of pain at home.

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Disseminated Intravascular C oagulation DIC

• DIC is aserious bleeding disorder resulting from
  accelerated normal clotting with a subsequent
  decrease in clotting factors platelets leading
  to uncontrolled bleeding .
• Etiology it is always a result of another
  disease or condition ,it is often associated with
  shock ,infection ,hemolytic processes such as
  transfusion of mismatched blood .

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• Sing symptom
• 1- bleeding can occur any where in the body
  following trauma or normal activity or
  spontaneously .
• 2- specific sing symptom
• Slow,persistent ,prolonged bleeding from minor
  injuries .
• Uncontrollable hemorrhage subsequent to dental
  extraction or irritation of the gums .
• Epistaxis especially after facial injury .
• Hematuria.
• Ecchymosis ( petechiae are rare ).
• Bleeding into joints (hemarthrosis )which may
  lead to severe joint deformity .

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Hemophilia

• Is a disorder of hemostasis of one or more
  clotting factor.
• Type
• 1-hemophilia A deficient in factor VIII,called
  classic hemoph