"MY TEACHING HOURS " FOR PCL NURSING

UNIT 6.NURSING CARE OF THE CHILDREN

6.5ENDOCRINE DISORDERS

ENDOCRINE GLANDS

Endocrine glands are the ductless glands, which consists of group of secretory cells, which secrete several hormones and diffuse them directly to the blood stream.

The endocrine system controls and regulates metabolic process, governing energy production, growth, fluid and electrolyte balance, response to stress and sexual reproduction.

The endocrine glands are:

1) 1 pituatory Gland

2) 1 Thyroid Gland

3) 4 Para thyroid Gland

4) 2 Adrenal Gland

5) The Pancreatic Islets(Islet of Langerhans)

6) 1 Pineal Gland

7) 1 Thymus Gland

8) 2 Ovaries in Females

9) 3 Testes in Male

1. Pituatory Gland:

Roughly oval shaped gland, situated in the hypophyssal fosa of the sphenoid bone below the hypothalamus.

Functions of Anterior Pituatory Gland:

i. Growth hormone or somatotrophin hormones(GH):

ii. Adrenocorticotrophic hormone(ACTH)

iii. Thyrotrophin or thyroid stimulating hormone(TSH)

iv. Gonadotrophin Hormone:

a) Follicle stimulating hormone(FSH)

b) Lutenizing hormone(LH)

v. Prolactin Hormone

Functions of Posterior Pituatory Gland:

It produces 2 hormones.

i. Oxytocin

ii. Antidiuretic hormone(ADH) or vasopressin

ANTERIOR PITUATORY GLAND DISORDERS

Growth hormone:

This is the most abundant hormone synthesized by the anterior pituatory gland.It stimulates growth and division of the most body cells but especially those in the bones and skeletal muscles.It also regulates aspects of the metabolism in many organs e.g.live, intestines and pancreas;stimulates protein synthesis promotes breakdown of the fats and increase blood glucose level.

GIGANTISM and ACROMEGALY:

GIGANTISM:

Gigantism is the endocrine disorder resulting when there is excess Growth Hormones while epiphyseal cartilage of the long bone are still growing i.e. before the ossification of bone is complete or epiphyseal closure.

Clinical features of Gigantism:

i. Excessive growth of long bones (2.1m/7feet-2.7m/9feet) and enlargement of soft tissue including muscles.

ii. Increase in weight,usually not excessive but remains in proportion to child height

iii. Enlargement of organs(heart,spleen)

iv. Increased metabolic rate

v. Vertical growth is accompanied by rapid and increased development

vi. In case of early childhood and infancy, increased head circumference and delayed closure of fontanelles may b observed.

vii. Incomplete or slow development of secondary sex charateristics in adolesence

viii. Headache and visual disturbance(Increased intracranial pressure creates the pressue in the optic chiasma)

ACROMEGALY:

The endocrine disorder occurs due to excessive growth hormone after the ossification is complete; there is excessive growth of children in transverse direction and common in children

Clinical features of Acromegaly:

i. Overgrowth of head

ii. Lips are large

iii. Board nose, tongue,jaw and mastoid sinuses

iv. Facial hair increase

v. Mandible enlarge and teeth may separate

vi. Headache and visual distrubance due to increased intracranial pressure

vii. Dorsal kyphosis may be present

viii. High arched palate

ix. Mental retardation may be present in about 85% of the cases

x. Increased sweating ,tachycardia

xi. Amenorrhoea in females

xii. Impotence in males

Diagnosis of Gigantism and Acromegaly:

a) Clinical examination and periodic growth assessment

b) Investigations shows elevated growth hormone level; above normal

c) X –Ray of the skull may show the enlargement of the sella tunica and para nasal sinuses in case of any tumours

d) CT Scan and MRI to rule out tumours

Treatment of Gigantism and Acromegaly:

a) Sometimes in case of pituatory insufficiency,hormone replacement with thyroid extract,cortisone and sex hormone may be necessary

b) Dopamine and dopamine agonist paradoxically lower the circulatory growth hormone level on case of Acromeagly(in contrast it elevates the growth hormone level in normal individuals)

Bromocriptine(2 Bromo-alpha ergokryptine):dopamine like long acting drug.10-20mg/kg in case of acromegaly.the treatment is started in low doses and should be given along with the meals. It may cause side efffects like dyspepsia, vomitting, dizziness and constipation

Octreotide is also seen benificial to acromegaly.

c) Surgical treatment:

In case of micro-adenoma, hypophysectomy is required to remove the micro adenoma with the functional tissue intact.After surgey, external irradiation therapy and hormonal repalcement may be required.

Nursing management of Gigantism and Acromegaly:

Nursing care:

a) Early identification of the child with excessive growth rate by careful growth monitoring and recording with the use of appropiate growth chart

b) Clear explanation to the child and parents about the need of different types of possible therapies and the rationale of the therapies

c) Monitor the signs of the increased intracranial pressure such as headache,poor vision and timely management of it

d) Provide pre and post operative nursing care to the child if surgery is needed

e) Prepare the child for the irradiation therapy if needed

f) Post operative care of craniotomy:

· Observation of bleeding from the operated site

· Watch for signs of increased intracranial pressure

· Looking for any indication of endocrine dysfunctions

· Close assessment for sign and symptoms of diabetes insipidus and/or ACTH deficiency

g) Instruct the parents on:

· Medication(types of drugs used,doses and possible side effects)

· Homecare

· Follow up visits

· General care of children at home

· Explain that the medical supervision will be required regular for life

· Protect stress situation and provide emotional support and motivation

DWARFISM/SHORT STRATURE

It is the condition associated with growth hormone deficiency.It may be congenital or acquired.

It is defined as length /height below 3^rd percentile for age according to international standard or 3 standard deviation of mean for age.

Aetiology:

A. Genetic Defects:

i. Autosomal recessive and dominant

ii. Panhypopituitory disorder dwarfism(Total absence of pituatoryhormone:panhypopituituarism)

B. Acquired :

i. Tumours:hypothalamus,pituitary (carniopharyngioma)

ii. Irradiation

iii. Post infection(enchepalitis,meningitis)

iv. Prolong malnutrition

v. Primodial –intrauterine growth retardation

Note:The infants with congenital deficiency of growth hormone may appear normal at birth .The growth impairment starts within a few month after birth,but a clinical diagnosis maynot be easily made till the child is about 1-2 years unless very accurate records are available.

Clinical manifestations:

i. Short stature

ii. Overweight for height with marked increased subcutaneous fat

iii. Bone age may be delayed(height age less than skeletal age)

iv. Skeletal maturation may be delayed

v. Delayed teeth developed

vi. Crowding of mid facial structure

vii. High pitched voice

viii. Increased skin fold thickness and obesity

ix. Small penis and scrotum

x. New born with congenital growth hormone deficiency may present with severe hypoglycemic convulsion and adrenocorticotrophic deficiency.

Diagnosis:

The latest criteria used for diagnosis are

i. Height below 3^rd percentile

ii. Prepubertal growth velocity less than 4cm per year

iii. Bone age below chronological age

iv. Abnormal 24 hours growth hormone secretory pattern

v. Peak growth hormone levels <10 mg/ml during provocative stimulation tests

vi. Resumption of growth following growth hormone administration

In addition, following diagnosis are done

i. Family history of short stature and child health history

ii. Child’s presentation and physical examination findings

iii. X-Ray of skull: to detect carniopharyngioma where the sella turcica will be enlarged

X-Ray of wrist: for center of ossification to evaluate the growth of the child

iv. CT Scan and MRI to rule out pituatary neoplasm

Treatment:

a) Keep the child under surviellence for regular growth measuremetn for 6 months to 1 year before starting investigation on the basis of physical examination

· A child who gains more than 4 cm a year is less likely to have hypopituitarism

b) Surgical removal or irradiation of the tumour is needed in case of organic lesions

Nursing care:

a) Nurses should have knowledge of normal growth and development of children,regular monitoring of physical parameters such as height ,weight of the child and maintain record properly

b) Assist in estabilishing diagnosis

c) Provide emotional support to parent and child

d) Provide health education

e) Provide oppurtunity to express their feelings about body appearance and sexual function

f) Medications and doses must be calculated according to size

g) Clothes ,furniture and equipment to be designed according to child’s size for their comfort

DIABETES INSIPIDUS

This is the disorder of posterior pituatory gland caused by hyposecretion of antidiuretic hormone (ADH).It is relatively rare in condition.

It is characterised by persistant excessive quantity of urine, which of low specific gravity, causing dehydration, and excessive thirst and poly dipsia.

Types:

1. Cranial/Neurogenic Diabetes Insipidus:

This is caused due to deficient production of anti diuretic hormone; may be due to lesions in the neurohypophysis.

2. Nephrogenic Diabetes Insipidus

Renal tubules become irresponsive to the anti diuretic hormone/vasopressin

Aetiology:

A. Idiopathic

B. Congenital:

Herediatory and nervous system defect at birth

C. Acquired:

Central nervous system trauma, tumour, infection, head injury, vascular lesions and chronic renal disease, hypokalemia, hypercalcemia

D. Neurogenic Diabetes Insipidus:

It can occur in new born follwing birth asphyxia, intracranial bleeding and meningitis due to beta haemolytic streptococcus

Pathogenesis:

In normally functioning body,while there is loss of water;thirst center (hypothalamus) intiates thirst and synthesized anti diuretic hormone.Thirst help to maintaine body water level by adequate intake of water whilst antidiuretic hormones acts on the kidney for reabsorption of water and prevents further water loss from the body.

In case of deficiency of antidiuretic hormone, the mechanism of water reabsorption from kidney is altered resulting in fluid volume deficit in body.

Clinical manifestation:

a) Polyuria,polydipsia,nocturnal enuresis

b) Thirst and passess of large quantities of urine

c) Poor appetite

d) Dehydration

e) Weight loss

f) Sleep distrubances,visual distrubances and emotional disorders,hyperthermia

g) Infants with diabetes insipidus present with excessive crying,quited with water than milk,rapid weight loss,sunken eye,dehydration and growth failure

Diagnosis:

a) 24 hour intake output record i.e.10 liter per 24 hours or more

b) Urine specific gravity(1.001 to 1.005)

c) Urine osmolarity (<300mos/lit remains below plasma level)

d) Water deprivation test:

Water deprivation for 6 hours, children with diabetes insipidus fails to concerntrate urine

e) Renal funtion test

f) Radiological test

X -Ray of skull, CT and MRI

g) History of illness and physical examination

Treatment:

a) Demopressin ,an analog of antidiuretic hormone throuh nasal spray with dose 5-10 ug daily in single or divided dose

b) Nephrogenic diabetes is treated with:

· Sufficient water intake to prevent dehydration

· Drug-Hydrochlorothiazide (0.5-1.5mg/kg daily).It reduces urinary volume as a paradoxical effect

· Dietary restriction of salt and protein

Nursing care:

a) Assesment of the child’s general health status including clinical presentation

b) Monitor intake and output strictly

c) Take weight measurement daily

d) Parents and child teaching on

· Disease process along with its consequences

· About drug and hormonal therapy

· Carry medical identity card

· School teachers should realize the need of unrestricted use of toilet and drinking water facilities

· Home mangement

THYROID GLAND:

The thyroid gland is composed of two lobes (right and left) that are connected by an isthmus, is located immediately below the larynx on the either side and anterior of the trachea.

Regulation of the thyroid hormones

• TSH is released from the anterior pituitary gland in response to thyroid releasing hormone from the hypothalamus and causes the synthesis and secretion of triiodothyronine (T3) and thyroxine (T4) by the thyroid gland.

• This is acheived by stimulation of the thyroid follicular cells by binding of TSH to the receptor on the basal surface of the cell and activation of adenylate cyclase.
• This leads to iodide uptake. T3 and T4 exert negative feedback on both the pituitary production of TSH and the hypothalamic production of TRH.
• Other factors affecting release of TRH from the hypothalamus incluse blood levels glucose and the body's metabolic rate.
• Somatostatin inhibits TSH secretion and oestrogen has been shown, in rats, to reverse the negative feedback affect of T3 and T4 on the TSH response to TRH.

FUNTIONS OF THYROID HORMONE:

1. Growth and development

2. General metabollic rate

3. Metabolism

4. Respiratory effect

5. Cardiovascular effect

6. Gastrointestinal effect

7. Effects on nervous system

8. Reproductive system

Thyroid Hormones levels in body:

Generally, a normal range for TSH for adults is between 0.4 and 5.0 uIU/mL (equivalent to mIU/L), but values vary slightly among labs. The optimal goal TSH level for patients on treatment ranges between 0.3 to 3.0 mIU/L.[4]. The interpretation depends also on what the blood levels of thyroid hormones (T3 and T4) are. The National Health Service in the UK considers a "normal" range to be more like 0.1 to 5.0 uIU/mL
For full term newborns, the range of normal TSH levels is quite large. TSH can vary between 1.3 and 16 microunits per milliliter of blood. After about a month, this range narrows to 0.9 to 7.7 microunits per milliliter, and by school age it decreases to 0.6 to 5.5 microunits per milliliter. This gradual decrease in TSH levels is normal, though levels of free thyroid hormone (T4) in the blood will remain relatively stable over the same time period.

Thyroid hormone tests

Total thyroxine (T4): 9.8–22.6 micrograms per deciliter (mcg/dL) in newborns (1 to 14 days of age)

5.6–16.6 mcg/dL in babies and older children

5–14 mcg/dL in adults

Free thyroxine (FT4): 0.8–2.4 nanograms per deciliter (ng/dL)

Total triiodothyronine (T3): 32–250 ng/dL in newborns (1 to 14 days of age)

83–280 ng/dL in babies and older children

80–230 ng/dL in adults

Free triiodothyronine (FT3): 0.2–0.6 ng/dL

Free Thyroxine Index (FTI): 7.5–17.5 in newborns (1 to 14 days of age)

5.0–12.8 in babies and older children

4.2–13.0 in adults

Read more: http://www.livestrong.com/article/224892-normal-tsh-levels-in-children/#ixzz1q2ZiHVji

http://in.answers.yahoo.com/question/index?qid=20080817230852AA1Cspi

TSH-

· Normal adult range:5-6 milliunit/l

For full term newborns, the range of normal TSH levels is quite large. TSH can vary between 1.3 and 16 microunits per milliliter of blood. After about a month, this range narrows to 0.9 to 7.7 microunits per milliliter, and by school age it decreases to 0.6 to 5.5 microunits per milliliter. This gradual decrease in TSH levels is normal, though levels of free thyroid hormone (T4) in the blood will remain relatively stable over the same time period.

HYPOTHYROIDDISM:

Clinical syndrome caused by low level circulating thyroid hormone known as hypothyroidism.It generally means the hyposecretion of thyroid hormone.

This is one of the common endocrine problem of childhood, resulting due to deficient production of thyroid hormone.

Types:

1. Congenital e.g.cretinism

2. Acquirede.g.hashimoto’s thyroiditis

3. Dietary insufficiency of iodine e.g goiter

4. Secondary hypothyroidism e.g. due to deficiency of thyroid stimulating hormone and hypo pituitarism.

CONGENITAL HYPO THYROIDISM:

Congenital hypo thyroidism is the condition caused by the failure of embryonic development of thyroid gland but it may also be as a result of enzymatic defect in the synthesis of thyroxine.

Incidence:

85% of cases are due to dysgenesis (manifest at birth and evidence late as in ectopic thyroid)

Around 10-15%cases are due to errors of thyroid hormone synthesis

*10% of cases are due to heridiatrory defect

5% of cases are due to secondary causes

Causes of congenital hypothyroidism

i. Dysgenesis:

Agenesis, hypoplasia of thyroid gland or ectopic thyroid

ii. Inborn errorsof thyroid hormone synthesis

iii. Insensitivity or resistance of thyroid hormone

Inherited defect of thyroid hormone receptors

iv. Maternal conditons:

Radoiiodine, Prophythiouracil, carbimazole

Clinical features of congenital hypothyroidism:

It is difficult to diagnose in the early neonatal period.Clinical features may be apparent after several weeks of birth .The usual clinical symptoms includes

a. Open posterior fontanelle ,wide open cranial sutures,thick and dry coarse and cool skin,birth cot sleepy

b. By the age of 8-10 weeks,coarse facial features i.e.puffy face,swollen eyelids,wide seperated eyes,board nose,with large tongue,delayed social smile

c. Hypothermia poor feeding,umbilical hernia, marked physical and mental retardation may present

d. Long standing untreated cases:hypertropied muscles with an atheletic appearance

e. Hypotonia

Causes of acquired hypothyroidism:

i. Iodine deficeiency

ii. Autoimmune (chronic lymphocytic thyroiditis e.g.Hashimoto’s thyroiditis)

iii. Irradiation(after treatment of lymphomas)

iv. Surgical ablation

v. Ingestion of goiterogens(these includes iodides found in asthmatic medicines like amidarone;neonatal exposure to iodide containing antiseptic,prophylthiouracil and methimazole)

vi. Drug induced (iodide,carbimazole,and lithium)

vii. Secondary hypothyroidism due to deficiency of thyroid stimulating hormone or thyroid releasing hormone or hypopituitarism

Sign and symptoms of acquired hypothyroidism:

a. Growth retardation

b. Delayed dental development

c. Delayed puberty

d. Myopathy

e. Delayed skeletal maturation

f. Enlarged sella turcica

g. Pseudotumour cerebri(a condition in which there is increased intracranial pressure without any biochemical and cellular abnormalities)

Diagnosis:

i. History taking and physical examination

ii. Serum analysis of T3,T4 and TSH(thyroid function test):

The level of T3, T4 may be low or boarderline but hthe TSH is usually elevated if the defect is in the thyroid gland; TSH is usually more than 100 mu/ml.

iii. Fine needle aspiration cytology(FNAC) of the thyroid gland

iv. Thyrotrophin releasing hormone(TRH)test(to detect hypothyroidism secondary to hypothalamic or pituitory damage)

v. X-ray of bone shows related growth

vi. Skull X-ray shows large fontanelle,wide sutures

vii. Serum cholesterol level is usually elevated

viii. Urine iodide excretion test shows low level iodine

ix. Estimation of thyroid antibiotics to detect chronic lymphocytic thyroiditis

x. ECG to asses cardiac conditons

Treatment:

a. Treatment of choice is synthethic sodium levothyroxine:

Dose: neonate>10-15 ug/kg /day, until normal metabollic rate is maintained

Older children>4-8 ug/kg/day, until normal metabollic rate is maintained

*children less than 1 year should receive treatment immediately and shouldn't wait till investigation completed

b. For secondary hypothyroidism:

Cortisol replacement should be initiated before thyroid replacement

c. Therapy should be monitored by assessment of clinical symptoms,mental performance,periodic TFT,and radiological evaluation for bone age annually

Nursing Mangement:

i. Observe the child physical and mental activity level

ii. Maintain intake and output chart

iii. Avoid sedatives or narcotics(as hypothyroidism delays metabolism)

iv. Provide patient with comfortable and warm environment

v. Provide high fibre diet and encourage plenty of food

vi. Advice patient to report aggravated conditions such aschest pain and tachy cardia

vii. Well instruct patient for sign of over dose of medication like weight loss, tachycardia, sweating and diarrhoea.

Goiter formation:

The enlargement of thyroid gland generally refers as goiter; although the enlargement is difused but not symmetrically.

This may occur secondary to the effect of TSH receptor stimulatory antibodies inflammatory lymphocytic infiltration or compensatory hyperplasia because of decreased serum T4 and increased TSH concerntration.

Upon palpation, the thyroid gland may initally be soft but then takes on a firm feeling with rubbery consistency and a seed like surface secondary to hyperplasia of normal lobular architecture

ADRENAL GLANDS

There are two adrenal glands situated on the upper pole of the each kidney enclosed with renal fascia.

Hormones secreted by

A. Adrenal cortex:

i. Glucocorticoids:

(Cortisol/hydrocortisone, corticosterone and cortisoneare main glucocorticoids)

The chief function of the glucocorticoids includes regulating metabolism (by gluconeogenesis, lipolysis, absorption of sodium and water from renal tubules)

And response to stress via anti inflammatory action supresseing immune response of tissue to injury and delay wound healing

ii. Mineralocorticoids(aldosterone):

It helps in maintenance of water and electrolyte

iii. Sex hormones(androgens):

It contributes to the onset of puberty

B. Adrenal medulla:

a. Adrenaline and Non adrenaline:

They potentiate the flight and fight response after initial sympathethic stimulation (increasing heart rate, blood pressure, increasing metabollic rate, peripheral vasoconstriction and high blood flow to essential organs, dilating pupils)

ADRENO-CORTICAL DISORDER:

Hypersecretion of glucocorticoids (Cushing’s syndrome):

Cushing’s syndrome is a contition when body experience excessive circulating hormone cortisol and to less extent of androgens and aldosterone. It is sometimes called “hypercorticolism” and usually uncommon in children.

Causes:

1. Pituitary:

An excess production of ACTH, predisposing to adrenal hyperplasia presenting Cushing’s syndrome

2. Adrenal causes:

Incase of adrenal neoplasm, hypersecretion of glucocortiods may occur

3. Administration of large amount of exogenous corticosteriods

4. Food dependent:

Inappropriate sensitivity of adrenal glands to normal, postprandial increases in secretion of gastric inhibitory, polypeptide

Clinical manifestations:

i. Weight gain(fat pads on neck and back-buffalo hump)

ii. Hair thinning

iii. Psychosis (depression anxiety and irritation)

iv. Moon face

v. Cataract

vi. Acne

vii. Muscle wasting and weakness

viii. Poor wound healing

ix. Hypertension

x. Peptic ulcer

xi. Kidney stone

xii. Hyperglycaemia

xiii. Vertebral collapse

xiv. Peptic ulcer

xv. Menstrual distrubance in female:impotence in male

xvi. Osteoporesis

xvii. Tendency to infection

Diagnosis:

a. Fasting blood sugar

b. Serum electrolyte for hypokalaemia

c. Twenty four hour urine test for 17-keto steriods and hydroxycorticoids as cortisone is excreted in urine

d. Intravenous pyleogram(it outlines the shadow of the kidney and adrenal gland)

e. CT scan,MRI,and ultrasound may be preformed to localised adrenal tissue and to detect tumours of adrenal and pituitary gland

f. Cortisone supression therapy(administration of exogenous supply of cortisone normally supress adrenocorticotrophic hormone production)

Treatment:

The treatment of Cushing’s syndrome depends on the cause of Cushing’s syndrome:

i. Bilateral adrenalectomy for removal of adrenal tumours followed by post operative replacement of cortisol hormone

ii. Radiation therapy

iii. Adrenolytic agents(e.g mitotone or aminoglutethimide)are administered with an inoperatable patient

iv. Antidiuretics,gonadotrophins,and steroidsmay be necessary for an indefinite periods

Nursing Manangement:

Nursing diagnosis:

v Anxiety relaterd to altered body function

v Altered physiological coping related to situational crisis

v Fluid volume alteration related to increased metaboliic rate of body;hyperactiveness

v Injury , potential for, related to immunosupressive conditions

v Knowledge defecit related to complex medication pattern

Nursing care:

a. Decrease controllable stressors:

· Provide continuity care

· Explain all procedure slowly and carefully

· Spend time with patient and listen carefully

· Avoid sudden noise temperature changes,draft and unnecessary invasion of privacy

b. Controlling fluid and electrolyte excess:

· Restrict fluid

· Low sodium and potassium replacement

· Daily weight and intake out put monitoring

c. Monitoring physiological coping:

· Regular monitoring of vital signs especially bloodpressure

d. Prevention from infection and fall:

· Daily assesment(lungs, mouth, ear etc) for signs of infection

· Institute preventive care,oral hygiene,maintaining aseptic technique for related procedure

· Limiting physical activity

e. For person undergoing surgery:

i. Pre-operative care:

· Provide supportive and preoperative care

· Assist with measures used to prevent and treat the adrenal crisis

· Maintain nutritional status

ii. Post operative care:

· Assesment of complication of surgery and adrenal crisis

· Provide measures to minimize the postural hypotension by ambulation,elastic stockings

· Provide measures to reduce the risk of infections in immunosupressed patient

· Administer cortisol replacement

· Assist patient and patient party in learning about required hormonal replacement and its effects,

6.7 ORTHOPAEDIC DISORDERS AND COLLAGEN DISODERS:

RHEUMATIC FEVER:

Rhuematic fever appears to be an immunological disordes initiated by Group “A” beta haemolytical streptococcus.

Antibiotics produced against some streptococcal wall protein and sugar reacts with the connective tissues of the body as well as the heart and result in the rheumatic fever

The child with 5-15 years is the commonest age group and both sexes are normally equally affected

Predisposing factors:

· Poor economic status

· Unhygenic living conditions

· Overcrowding communities

· Poor nutritional status of the children

· Low immunological status

Aeitology:

The exact aeitology of the rheumatic fever is not known.But a strong association with beta haemolytic streptococci of Group “A”is indicated by a number of observations

i. A history of preceeding sore throat is available in approximately 50% of the patients

ii. Epidemic of streptococci infection is followed by a higher incidence of rheumatic fever

iii. The seasonal variation of rheumatic fever and streptoccocal infections are identical

iv. The patient with estabilished rheumatic heart disease,streptococci infection is followed by recurrence of acute rheumatic fever

v. Penicillin prophylaxis for streptococcal infection prevents recurrence of rheumatic fever is those patients who had it earlier

vi. More than 85% of the patients with rheumatic fever consistently show elevated level of anti streptococcal anti body titre.

Pathophysiology:

when there is Streptococal sore throat infection,the Body will produce antibody against streptococcal cell wall, cell membrane protein and sugar which consist streptococal wall carbohydrate called

N-acetyl –glucasamine

In human connective tissue (cardiac muscle, striated muscle and vascular smooth muscle)there is Presence of N-acetyle-glucosamine; the antibody produces while infection reacts in such tissues

Result inflammation with edema, hyperemia and leukocyte inflitration

Clinical features:

Streptococcal sore throat with fever followed by 10 days to a few weeks later by recurrence fever and various manifestation of acute rheumatic fever

Guidelines for the clinical diagnosis of acute rheumatic fever, originally suggested by Dr.TD Jones, have been subsequently revised by the American Heart Association in 1965.

Criteria for diagnosis of rheumatic fever:

Major criteria:

v Carditis:

The rheumatic carditis is a pancarditis involving the pericardium, myocardium and endocardium.It accours in 50-60% of the patients with acute rheumatic fever.

v Arthritis:

Polyarthritis involving large joints like knee, ankles and elbow

v Subcutaneous nodules:

Nodules are seen around six weeks after the onset of rheumatic fever on bony prominence area like elbows, chin, occipital and spine.

v Chorea:

Chorea is the late manifestation and most common in female children.Chorea consist of purposeless jerky movements resulting in derranged speech, muscular inco-ordination, awkard gait and weakness.

v Erythema Marginatum:

Faintly reddish rash not raised above the skin and absent itching, commonly seen with a pale center

Minor criteria:

v Clinical:

· Fever

· Arthalgia(joint pain)

· Previous history of rheumatic fever

v Laboratory:

· Acute phase:leucocytes,elevated ESR and C-Reactive Protein,Prolong PR interval in ECG

Essential criteria:

Evident for recent streptococcal infection as indicated by

· Increased antistreptolysin “O”(ASO) titre

· Positive throat culture

· Recent scarlet fever

Two major or one major or two minor criteria are required in the presence of essential criteria to diagnose acute rheumatic fever.

Investigations:

i. Laboratory:

· Increased sedimentation rate

· C-Reactive Protein-positive

· Antistreptolysin “O” titre(ASO titre)-positive

· Throat culture-positive(But it only means streptococci present in throat not rheumatic fever)

ii. ECG

iii. Echocardioraphy

Prevention of rheumatic fever:

Primary prevention:

For primary prevention, it is necessary to educate the community regarding the consequences of streptococcal sore throat.Logistically, it is difficult since it requires

i. Identification of sore throat

ii. Rapidly confirming that sore throat is streptococcal

iii. Provision of appropriate treatment i.e. oral penicillin for 10 days or I/M benzathine penicillin 1 dose

Secondary prevention:

It consist in giving long-active benzathine penicillin.The dose is 1.2 million units once every 3 weeks or 0.6 million every 15 days,depending on patient’s age and muscle mass.The injection is painful and some patient get fever for 24-36 hours following the injection.

· <30 kg body weight=600,000 unit

· >30 kg body weight=1,200,000 Unit

If allergic to penicillin,

a. Erythromycin 40 mg/kg/day,once a day

b. Sulphadiazine 0.5gm once a day for & lt;30 kg

1mg once a day for>30 kg

Ideally penicillin prophylaxis should continue life long.

Less ideal would be to continue till the age of 35 years

The least satisfactory approach is to give it for 5 years from the most recent attack of rheumatic fever or when they reach their 21^st birthday, whichever comes last.

Treatment:

There is no specific treatment for rheumatic fever.

Management is symptomatic combined with supressive therapy.Management includes

i. Bed rest:

During acute phase of attack, all patient should be kept on bed rest.For carditis, with or without cardiomegaly or with or with out failure from 4 weeks to 3 month and without cardiac involvement for2 weeks and gradual ambulation for 2 weeks

ii. Diet:

Normal light diet but if involvement of cardiac, salt intake should be restricted

iii. Medicine:

After obtaining throat culture, the patient should be kept on penicillin with initial dose of 4 lakh units of procaine penicillin deep intramuscular two times a day for 10 days

This is followed by prophylactic penicillin using benzathine penicillin 1.2 mega units every 21 days cycle.

iv. Supressive therapy:

Prednisolone:60 mg/day for patient weighing more than 20 kg and 40 mg /day for patient weighing less than 20 kg. This is continued for 3 weeks and then reduced to 50 mg/day for 1 week and 40 mg/day for another week

Aspirin: Preferred in patient who doesn’t have cardiac involvement

90-120 mg/kg in 4 divided doses for 4 weeks then tapered after next 2 weeks

v. Others:

· Reassure and told about the self limiting course of disease to client and parents

· Complete physical and mental rest

· Advise phenobarbitone 30 mg thrice a day in case of chorea

Nursing consideration:

The objectives of nursing care includes

a. Encourage compliance with drug regimens

b. Facilitate recovery from the illness

c. Provide the emotional support

d. Prevent the recurrence of the disease

Assessment:

i. History of current attacks

ii. Vital signs

iii. Obtaining history for throat infection

iv. Observe for pallor , anxiety

v. Blood investiations

vi. Knowledge of the drug therapy

Nursing dianosis:

i. Risk of infection related lower body defense

ii. High risk for injuries related to lowered immune response;physical condition

iii. Interrupted family process related to situational crisis

iv. Impaired physical mobility related to decreased strength and endurance;pain and discomfort

v. Alteration in body temperature related to group “A”beta haemolytic infection

Nursing care:

i. Promoting rest:

Provide rest during acute phase of disease

ii. Preventing injuries and falls:

· Limit heavy physical activity

· Provide indoor play environment

· Provide proper explanation regarding chorea that it is transitory and that all manifestaiton eventually disappear, Use side rails to prevent injuries.

iii. Preventing infection:

Regular dose of penicillin 1.2 millon every 21 days

iv. Physiologic assessment:

· Monitor vital signs 4 hourly

· Monitor intake and output if child had cardiac problems

v. Provide adequate nutrition

vi. Health education about prevention of disease i.e.screening school age, children for sore throat caused by group A streptococci and proper treatment.

Complications:

· Significant congestive heart failure

· Pericarditis

· Pericardial effusion

· Artial/mitral valve regurgitation

· Permanent cardiac damage

RHEUMATIC HEART DISEASE:

Rheumatic heart disease refers to the cardiac manifestation of rheumatic fever;manifested by pericarditis,myocarditis,endocarditisand valvular disease.

It is one of the major cause of heart disease in children.

It may involve

a. Lining of the heart(endocardium) including mitral,aortic and tricupsid valve.The stenosis of valve may occur due to tear or distrotion

b. Heart muscles(myocardium)

c. Outer covering of the heart(pericardium)

It is the inflammatory condition which has both acute and chronic stages.

1. Acute rheumatic heart disease:

Usually occurs after the course of acute rheumatic fever .Mostly occurs in the young children.

a. Acute Rheumatic endocarditis:

This is the acute inflamamtion of the endocardium;particularly the valves of the heart

b. Acute rheumatic myocarditis:

In this case ,myocardium is inflammed. It is quite dangerous,because in acute stae,myocardium might fails to act normally and death may occur.

c. Acute Rheumatic pericarditis:

It is the inflammation of pericardium,usually serofibrious pericarditis may persist.But it may be dry or wet(Pericardial effusion)too.Pericarditis may occur in case of severe acute rheumatic fever.Pericardial effusion with large amount of fluid may press the heart causing severe disturbances to an already damaged heart.

Clinical features:

i. High fever with chills

ii. Sore throat with high temperature

iii. Profuse sweating with rashes

iv. Involvement of joints is very common

v. Rapid pulse with irregular rhythm

vi. Presence of heart murmur

vii. Fibrous nodes may present in joint and tendon ,redness and polyarthritis may present

2. Chronic rheumatic heart disease:

In chronic rheumatic heart disease,valves are particularly damaged due to chronic inflammation.

The valve become thick,distorted and lose their normal elasticity,which result in loss of its normal function.

Two physiological changes in heart valves are:

i. The valves may adhere together causing narrowing of valve opening causing obstruction of flow of blood. This is known as stenosis of valve.

ii. Secondly,because of loss of elasticity and distortion of valve,the valve may not close properly,causing leakage and regurgitation of blood.This is known as incompetency of valve.

Clinical features of chronic rheumatic heart disease:

i. Less elasticity of valves and it becomes scarrified and leaflet adhere together

ii. Papillary muscles and chordea tendinae thicker and are plastered with exudates.

iii. Fresh vegetation occurs with recurrence

iv. Mitral valve stenosis occurs with in 2 to 8 years

v. Valvular incompetence because of shortening of chordae and papillary muscles occurs within a few weeks giving rise to mitral incompetence

vi. Scaring small occurs round the conducting system giving conduction distrubances

vii. Cardiac dialtion

viii. Pericarditis usually heals sequelae

Treatment of the Rheumatic Heart disease:

a. 1.2 million units of benzathine penicillinG I/M or 6 lakh units of Procaine Penicillin g for 10 days.Erythromycin 1 gram orally for 10 days in those within penicillin allergy.For prophylaxis,1.2 million units of benzathin penicillin G in every 3 weeks,Optimal duration of prophylaxis is not certain.It is recommended for life for those with heart disease and to age 21 in patient without heart disease

b. Aspirin 100 mg/kg/day for 4 weeks.Optimum serum level is 20-25 ml/100ml.The dose should be radually reduced

c. Use of steroids is controversial.Prednisolone 2mg/kg for 4 to 6 weeks with tapering over the next 2 weeks is recommended.these promptly reduce inflammation but no conclusive evidence exists indicating that they prevent residual valvular damage.They are definitely indicated with severe carditis

A. Assessment

The objective assessment is to collect data on :

• Cardiac function
• Tolerance to client activity and attitudes toward restrictions on activities
• Nutritional Status
• Discomfort level
• Sleep disruption
• Client's ability to overcome problems
• The things that can help clients
• Knowledge of parents and patients (according to the patient's age) of patient understanding.

Assessment :

• Hospital chart
• Monitor cardiac complications
• Cardiac auscultation, heart sounds with the rhythm of marching weakened diastole
• Vital signs
• The presence of pain
• The existence of joint inflammation
• The presence of skin lesions

Nursing Diagnosis :

1. Reduced cardiac output associated with valve stenosis
   
   Destination : COP increases
   
   Results Criteria :
• Clients shows a decrease dyspnea
• Participating in activities and demonstrate increased tolerance

Intervention :

• Monitor blood pressure, pulse apical and peripheral pulse
• Monitor cardiac rhythm and the frequency
• Sleeping position 450 semifowler
• Instruct the client to do stress management techniques (quiet environment, meditation)
• Aids client activity as indicated when the client is able
• Collaboration and providing oxygenation therapy

2. Activity intolerance related to decreased cardiac output, oxygenation supply and demand imbalance
   
   Objective: Clients can tolerate optimal activity against
   
   Result Criteria :
• Verbal Response fatigue is reduced
• Carrying out activities within the limits of his ability (pulse activity can not be greater than 90 X / minute, no chest pain)

Intervention :

• Energy saving clients during the acute
• Maintain a sleep until the results of laboratory and clinical status improved
• In line with the better situation, monitor the gradual increase in the level of activity
• Create a schedule of activity and rest
• Teach to participate in activities.
• Teach the children / parents who do not realize that the movement is connected with the Korean and temporary.
• In case of chorea, protect from accidents, bedrest and provide appropriate sedation program.

3. Anxiety related to complex medication pattern and pain
   
   Objective: Clients can tolerate anxiety and feeled relaxed
   
   Result Criteria :
• Verbal expression of feelings
• Carrying out regular medication mattern

Intervention :

• Give opportunity to patient to express feeling
• Give medication for relaxation
• Create a comfortable atmosphere for the family visitors

4. Aleration in nutrition related to complex medication pattern and pain
   
   Objective: Clients can have nutritional body state
   
   Result Criteria :
• Regular body metabolism estabilished
• Carrying out normal high nutritious diet

Intervention :

• Provide good and nutritional diet according to patient desire and condition
• Serve food in attractive manner

5. Tendency to constipation related to long term resting

Objective: Clients can have regular bowel habit without constipation

Result Criteria :

• Relieved of constipation

Intervention :

• Encourage patient to take sufficient fliud
• Encouraged activities as prescribed

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