CRETINISM

Case Study in Pediatric Ward

Submitted by: Morales, Monica Marie A. BSN ² III A2b

Submitted to: Mr. Romeo T. Papa, RN Clinical Instructor

August 4, 2010

I. INTRODUCTION

Cretinism or congenital hypothyroidism is inadequate thyroid hormone production in newborn infants. This can occur because of an anatomic defect in the gland, an inborn error of thyroid metabolism, or iodine deficiency. If left untreated, it results in irreversible damage to the central nervous system and developmental defects. The term endemic cretinism is used to describe clusters of infants with goiter and hypothyroidism in a defined geographic area. Such areas were discovered to be low in iodine, and the cause of endemic cretinism was determined to be iodine deficiency. The term sporadic cretinism was initially used to describe the random occurrence of cretinism in non-endemic areas. The cause of these abnormalities was identified as nonfunctioning or absent thyroid glands. This led to replacement of the descriptive term sporadic cretinism with the etiologic term congenital hypothyroidism. Treatment with thyroid replacement therapy was found to elicit some improvement in these infants, although many remained impaired. The chances of a child contracting this condition are very small. Data from most countries with well-established newborn screening programs indicate an incidence of congenital hypothyroidism of about 1 per 4000 births (United States) so this show just how remote this condition can be. Some of the highest incidences (1 in 1400-2000 births) have been reported from various locations in the Middle East. Most studies of congenital hypothyroidism suggest a female-to-male ratio of a 2:1. Early diagnosis and treatment of congenital hypothyroidism prevents severe mental retardation and other neurologic complications. As might be expected, infants with delayed bone age at diagnosis or a longer time to normalize thyroid hormone levels have poorer outcomes.

II. CAUSATIVE FACTORS         A missing or abnormally developed thyroid gland Insufficient production of thyroid hormone Pituitary gland's failure to stimulate the thyroid Defective or abnormal formation of thyroid hormones Abnormal iodide uptake autoimmune thyroiditis of the mother Thyroidectomy of the mother Uses of radioactive iodine

III. SIGNS AND SYMPTOMS                jaundice pallor blotchy, cool, and dry skin dry, brittle and dull dull facial expression large fontanels widely separated skull bone puffy wide set eyes (periorbital edema) open-mouthed thick protruding tongue macroglossia poor feeding choking episodes teeth erupt late and decay early enlargement of the thyroid gland                 short, thick neck dyspnea on exertion bradycardia protuberant abdomen umbilical hernia (rare) hypotonic abdominal muscle constipation sluggishness anasarca weight gain short extremities stunted growth decrease muscle tone sleepiness slow deep tendon reflex mentally retarded

IV. POSSIBLE COMPLICATIONS  Mental retardation  Growth retardation  Heart problems If this condition is left untreated then the child may not be able to do simple tasks as this condition can lead to retardation in the child. Growth will also not be at a steady rate and other problems will appear and also they will become more severe as the child ages.

V. LABORATORY AND DIAGNOSTICS PROCEDURE 1. Serum TSH  Pituitary production of TSH is measured by a method referred to as IRMA (immunoradiometric assay). Expected result: TSH becomes elevated.  This rise in TSH represents the pituitary gland's response to a drop in circulating thyroid hormone; it is usually the first indication of thyroid gland failure. 2. Serum T3 and T4 by RIA  The most used thyroid test of all. Thyroxine (T4) represents 80% of the thyroid hormone produced by the normal gland and generally represents the overall function of the gland. The other 20% is triiodothyronine measured as T3 by RIA. Expected result: Low level of T3 & T4  This means that the thyroid gland fails to produce enough thyroxine and T3 level for blood circulation 3. Serum Thyroid Binding Globulin  Most of the thyroid hormones in the blood are attached to a protein called thyroid binding globulin (TBG). Expected result: Increased TBG  Increased in TBG level shows decrease level of the thyroid hormones in the blood 4. Thyroid scan and a radioactive iodine uptake test (RAIU)  The thyroid scan is used to determine the size, shape and position of the thyroid gland.  The thyroid uptake is performed to evaluate the function of the gland. Expected result: Absence or small thyroid tissue and Decreased iodine uptake Preparation prior to procedure:  Verify if the client has history of allergic reaction to iodine. The radioactive tracer used for these tests may contain iodine. However, even if the patient is allergic to iodine, he will probably be able to have this test because the amount that may be used in the radioactive tracer is so small that the risk of an allergic reaction is very low. Before a radioactive iodine uptake (RAIU) test, secure a consent form that says the patient understands the risks of the test and agree to have it done. Before an RAIU test, blood tests may be done to measure the amount of thyroid hormones (TSH, T3, and T4) in blood. Instruct the patient or care provider that he must not eat for up to 2 hours before the test and not taking any anti-thyroid medication for 5 to 7 days before the test. Remove jewelry, dentures, or other metals, because they may interfere with the image. 

  

5. ECG  used to measure the rate and regularity of heartbeats as well as the size and position of the chambers, the presence of any damage to the heart, and the effects of drugs or devices used to regulate the heart (such as a pacemaker). Expected result: Sinus bradycardia and inverted T-wave Preparation prior to procedure:  Explain to the patient·s care provider that there are no restrictions for food or fluids. However, ingestion of cold water immediately before an ECG may produce changes in one of the waveforms recorded (the T wave). Exercise (such as climbing stairs) immediately before an ECG may significantly increase heart rate.  Instruct the patient to remove all jewelry and to wear a hospital gown. 6. Long Bone X-ray  Used to look for injury, infection, arthritis, abnormal bone growths, and bony changes seen in metabolic conditions.  Most bone x-rays require no special preparation.  The patient may be asked to remove some or all of his clothes and to wear a gown during the exam. Expected result: absence of femoral or tibial epiphysial line 7. CBC  used to evaluate the composition and concentration of the cellular components of blood Expected result: Low level of hemoglobin 8. Test for Electrolytes  used to determine if the client have electrolyte imbalances Expected result: increased Ca and decrease sodium 9. Cholesterol  Used to check the level of cholesterol-carrying proteins in the blood to determine impending risk for heart disease Expected result: increased level of LDL

VI. TREATMENT Cretinism is treatable however it is vitally important that the condition is caught at the very early stages of the child·s birth. If this condition is caught early then the effects that have taken place can be reversed. Replacement therapy with thyroxine is usually the standard treatment given to a child that has cretinism. Treatment with thyroid hormone promotes normal physical and mental development. It is essential that treatment be started during the first six weeks of life or irreversible changes may take place. Once medication starts, the blood levels of T3 and T4 are monitored to keep the values within a normal range. Endemic cretinism can be prevented by appropriate iodine supplementation. Iodization of salt is the usual method, but cooking oil, flour, and drinking water have also been iodinated for this purpose. Long-acting intramuscular injections of iodized oil (Lipiodol) have been used in some areas.

VIII. PRIORITIZED LIST OF NURSING PROBLEMS NURSING PROBLEMS IDENTIFIED Ineffective breathing pattern r/t weak diaphragm as evidenced by dyspnea on exertion CUES Subjective: ´Nahihirapan syang huminga.µ as verbalized by the patient·s mother. Objective:  bradypnea  dyspnea on exertion  alteration in depth of breathing  nasal flaring Hypothermia r/t decrease metabolic rate Subjective: ´Madali syang lamigin at lageng malamig ang kamay nya.µ at as verbalized by the patient·s mother. Objective:  cool skin  pallor  body temperature below normal range  slow capillary refill Subjective: ´Tatlong beses lang syang tumae sa isang lingo at madalas na nahihirapan syang ilabas.µ as verbalized by the patient·s mother. Objective:  hard, dry stool  hypoactive bowel sound  distended abdomen straining with defecation JUSTIFICATION According to Rule of ABC, breathing is the second priority. Ineffective breathing pattern will interfere with the respiration of the patient. This must be the nurse·s primary concern because it can be life threatening if not address immediately.

Hypothermia can also be life threatening if left untreated. This can affect blood flow and reduced oxygenation of cells in the body.

Constipation r/t decrease intestinal peristalsis as evidenced by hypoactive bowel sound

This problem is not currently health threatening, but it could be if it were to persist. Feces prolonged in intestine might produce toxin that could harm the body.

IX. RECOMMENDATION Prevention Pregnant women with diagnosed iodine deficiency or problem with thyroid gland is advised to comply with their medication. Iodine or thyroxine supplementation helps in giving the fetus adequate hormones needed for growth and development during gestation.

Early Diagnosis In pregnant women who take radioactive iodine for thyroid cancer, the thyroid gland may be destroyed in the developing fetus. Infants whose mothers have taken such medicines should be observed carefully after birth for signs of hypothyroidism. Also, mothers with a newborn infant are advised to submit their babies for newborn screening test for early detection of Cretinism.

Promoting Home-Based Care Nurse should instruct the patient·s care provider about the medications that are prescribed and their actions. It is also important to inform the family about symptoms that should be reported to the physician. Also, make sure that they understand that the thyroid hormone replacement therapy is life long and their compliance is very much needed.

Further Outpatient Care Laboratory measurements of T4 (total or free T4) and TSH should be repeated 4-6 weeks after initiation of therapy, then every 1-3 months during the first year of life and every 2-4 months during the second and third years. In children aged 3 years and older, the time interval between measurements may be increased, depending on the reliability of the patient's caretakers. As dosage changes are made, testing should be more frequent. Formal developmental and psycho neurological evaluations should be considered in all infants with congenital hypothyroidism. Such evaluations are especially important in children whose treatment was delayed or inadequate. As with any child, school progression should be monitored and parents is encourage to seek early evaluations and interventions as soon as problems are recognized.

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