MCN Finals Lecture: Endocrinology and Metabolic Disorders PDF

MCN FINALS (SECOND SET) LECTURE M6L3: ENDOCRINE/ METABOLIC DISORDERS elimination habits. Extreme thirst or appetite may occur with an endocrine disorder such as diabetes insipid...

MCN FINALS (SECOND SET) LECTURE M6L3: ENDOCRINE/ METABOLIC DISORDERS elimination habits. Extreme thirst or appetite may occur with an endocrine disorder such as diabetes insipidus The endocrine system keeps our bodies in balance, or type 1 diabetes mellitus. Frequent voiding in children maintaining homeostasis and guiding proper growth most often reflects a urinary tract infection, but it may and development. A single hormone may affect more be evidence of excessive urine excretion (polyuria), than one of these functions and each function may be possibly from pituitary dysfunction or diabetes mellitus. controlled by several hormones. A child’s general appearance may reveal early or late puberty changes, scaling or dry or darkening skin, Although the endocrine glands are the body's main drooping eyelids, protrusion of the eyeballs hormone producers, some non-endocrine organs— (exophthalmos), or poor muscle tone, all indications of such as the brain, heart, lungs, kidneys, liver, thymus, endocrine disorders. pancreas, skin, and placenta — also produce and release hormones. Hormones communicate this effect by their unique chemical structures recognized by specific receptors on their target cells, by their patterns of secretion and their concentrations in the general or localized circulation. Hormones functions as reproduction and sexual differentiation, for development and growth, for maintenance of the internal environment and regulation of metabolism and nutrient supply. Any abnormality in our endocrine system disrupts the normal growth and development of the children. M6L3.1: ENDOCRINE/ METABOLIC ASSESSMENT Endocrine and metabolic disorders, as a group, commonly cause changes in growth and a child’s social and physical development and may impair their cognitive development. These disorders are often manifested in growth and development delays, especially during puberty or adolescence. If not identified at birth, the disorder is usually detected when a child’s height and weight are measured at a healthcare visit and found to be above or below a typical measurement for that age. An acute loss in weight is often the first symptom of type 1 diabetes mellitus in children. Thyroid deficiencies or type 2 diabetes mellitus (T2D) may be revealed by being overweight. Pituitary difficulties may be revealed by unusually short or tall stature. To obtain information on activity in the child, take a day history by asking a parent or child to describe all of the child’s actions on a typical day because this type of information yields clues that are helpful in distinguishing between a child with a more introverted nature (a “quiet” child) and one who is experiencing inactivity and chronic fatigue as a result of decreased endocrine function. For example, the quiet child lies down after school and reads, whereas the ill child lies down and sleeps. The healthy child appears to “go constantly” but can sit through a favorite television program or a meal. The child with increased thyroid hormone production may be unable to sit quietly at all. Also assess dietary and TMNRY 1  M6L3.1: ENDOCRINE/ METABOLIC: injured the pituitary gland or chronic illness, such as a COMMON DISORDER heart, kidney, or intestinal disorder that could have contributed to the decreased level of growth. Take a 24-hour nutrition history to see if “picky eating habits” 1. GROWTH HORMONE DIFICIENCY are extensive enough to halt growth. Be certain to If production of human growth hormone (GH, or assess not only the child’s actual height but also his or somatotropin) is deficient, children are not able to grow her feelings about being short. to full size (Dörr, Boguszewski, Dahlgren, et al., 2015). As a result, children may appear well proportioned but A physical assessment, including a funduscopic measure well below the average on a standard growth examination, neurologic testing, and blood analysis for chart. Deficient production of GH may result from a hypothyroidism, hypoadrenalism, hypoaldosteronism, nonmalignant cystic tumor of embryonic origin that and growth factor–binding proteins are also helpful in places pressure on the pituitary gland or from ruling out a lesion or tumor. Bone age is established by increased intracranial pressure as a result of trauma. a wrist X-ray (epiphyseal closure of long bones is In most children with hypopituitarism, however, the delayed with GH deficiency but is proportional to the cause of the defect is unknown; it may have a genetic height delay). A skull series, computed tomography origin. If hypopituitarism is not treated, predicting (CT) scanning, magnetic resonance imaging (MRI), or exactly what height a child will reach is difficult because ultrasound will be prescribed to detect possible height varies with each individual. Without treatment, enlargement of the sella turcica, which would suggest however, most children will not reach more than 3 or 4 a pituitary tumor. ft in height. THERAPEUTIC MANAGEMENT ASSESSMENT ▪ GH deficiency is treated by the administration o The child with deficient production of GH is of intramuscular recombinant human growth usually normal in size and weight at birth. hormone (rhGH) usually given daily at bedtime, o Within the first few years of life, however, the the time of day at which GH normally peaks child begins to fall below the third percentile of (Graber & Rapaport, 2012) height and weight on growth charts. ▪ In addition, some children may need o The face appears infantile because the suppression of luteinizing hormone–releasing mandible is recessed and immature, and the hormone (LHRH, or gonadotropin releasing nose is usually small. hormone [GnRH]) to delay epiphyseal closure. o The child’s teeth may be crowded in a small jaw ▪ Other children may need supplements of (and may erupt late). gonadotropin or other pituitary hormones if o The child’s voice may be high pitched, and the these are determined to be deficient as well. onset of pubic, facial, and axillary hair and genital growth will be delayed. GH has been used irresponsibly by athletes in the hope o The history, physical findings, and a decreased that it will improve muscle growth and overall stamina. level of circulating GH contribute to the Caution children that the use of the drug when there is diagnosis. no medical reason for it is potentially dangerous and so o A pituitary tumor must be ruled out as the they should not share the drug with friends or take cause of decreased GH production. excessive doses themselves (Baumann, 2012). o Sudden halted growth suggests a tumor; Because they have delayed epiphyseal closure, if gradual failure suggests an idiopathic treatment is begun early, children can expect to reach involvement. a height individually targeted for them. Once o A history of vision loss, headache, an increase epiphyseal lines of long bones close (with in head circumference, nausea, and vomiting adolescence), GH will be tapered and stopped. (signs of increased intracranial pressure) also suggest a pituitary tumor. 2. GROWTH HORMONE EXCESS o Growth failure may be so marked in some An overproduction of GH usually is caused by a benign children that the parents may be tumor of the anterior pituitary (an adenoma). If the suspected of child neglect. overproduction occurs before the epiphyseal lines of the long bones have closed, excessive or overgrowth As part of the history taking, evaluate the family history will result. for traits of short stature or constitutional delay (familial late development). If at all possible, obtain estimates of ASSESSMENT the parents’ height and siblings’ height and weight o Weight will become excessive also, but it is during their periods of growth. proportional to height. o The skull circumference typically exceeds Assess the child’s prenatal and birth history for any usual, and the fontanels may close late or not suggestion of intrauterine growth restriction. Assess at all. also for any severe head trauma that could have TMNRY 2  o After epiphyseal lines close, acromegaly o Because so much fluid is lost, sodium becomes (enlargement of the bones of the head and soft concentrated or hypernatremia occurs with parts of the hands and feet) begins to be symptoms of irritability, weakness, lethargy, evident. fever, headache, and seizures. o The tongue can become so enlarged and o The signs and symptoms usually appear thickened that it protrudes from the mouth, gradually. Parents may notice the polyuria first giving the child a dull, apathetic appearance as bed-wetting in a toilet-trained child or weight and making it difficult to articulate words. loss because of the large loss of fluid. If the o If the condition remains untreated, a child may condition remains untreated, the child is in reach a height of more than 8 ft. danger of losing such a large quantity of water that dehydration and death can result. THERAPEUTIC MANAGEMENT o MRI, CT scanning, or an ultrasound study of ▪ If X-rays or ultrasounds of the skull reveal that the skull reveals whether a lesion or tumor is the sell a turcica is enlarged or that a tumor is present. present, laser surgery to remove the tumor or o A further test is the administration of cryosurgery (freezing of tissue) is the primary vasopressin (Pitressin) to rule out kidney treatment. disease. For this, after the child’s urine output ▪ If no tumor is present, a GH antagonist such as has been measured to establish a baseline, bromocriptine (Parlodel) taken orally or vasopressin is administered. The drug octreotide (Sandostatin) taken by injection can decreases the blood pressure, alerting the slow the production of GH. When GH secretion kidney to retain more fluid in order to maintain is halted in this way, other hormones may also vascular pressure. be affected; therefore, the child may need to o The drug decreases the blood pressure, receive supplemental thyroid extract, cortisol, alerting the kidney to retain more fluid in order and gonadotropin hormones in later life. to maintain vascular pressure. If the fault that ▪ A more permanent therapy is irradiation or is causing the dilute urine is with the pituitary radioactive implants of the pituitary gland, gland, not the kidneys, the child’s urine output again to halt GH production. will decrease; if the fault is with the kidneys, ▪ It is difficult for a child always to be bigger and urine will remain dilute and excessive in taller than playmates, and problems such as amount because the diseased kidneys cannot buying clothes or fitting into airline seats concentrate fluid. continue to be very real and distressing in adulthood. Counseling them about maintaining THERAPEUTIC MANAGEMENT self-esteem and making the adjustments ▪ Surgery is the treatment of choice if a tumor is necessary to accommodate their larger-than- present. usual size is a nursing responsibility. ▪ If the cause is idiopathic, the condition can be controlled by the administration of 3. DIABETES INSIPIDUS desmopressin (DDAVP), an arginine Diabetes insipidus is a disease in which there is vasopressin. In an emergency, this drug can be decreased release of ADH by the pituitary gland. This given intravenously (IV). For long-term use, it causes less reabsorption of fluid in the kidney tubules. is given intranasally or orally Urine becomes extremely dilute, and a great deal of ▪ If desmopressin is given as an intranasal fluid is lost from the body. Diabetes insipidus may spray, this may cause nasal irritation; the route reflect an X-linked dominant trait, or it may be will not be effective if the child develops an transmitted by an autosomal recessive gene. It may upper respiratory tract infection with swollen also result from a lesion, tumor, or injury to the mucous membranes. posterior pituitary, or it may have an unknown cause. ▪ Caution children that they will notice an In a rare type of diabetes insipidus, pituitary function is increasing urine output just before the next adequate, but the kidneys’ nephrons are not sensitive dose is due so they can arrange their day to ADH (a kidney-related etiology). according to where bathrooms are located. ASSESSMENT 4. CONGENITAL HYPOTHYROIDISM o Experiences excessive thirst (polydipsia) that Thyroid hypofunction causes reduced production of is relieved only by drinking large amounts of both T4 and T3. Congenital hypofunction (reduced or water; there is accompanying polyuria. absent function) occurs as a result of an absent or o The specific gravity of the urine will be as low nonfunctioning thyroid gland in a newborn. Congenital as 1.001 to 1.005 (normal values are more hypothyroidism or an indication that the infant’s thyroid often 1.010 to 1.030). Urine output may reach is not functioning well may not be noticeable at birth 4 to 10 L in a 24-hour period (normal range, 1 because the mother’s thyroid hormones (unless she to 2 L), depending on age. ingested less than usual amounts of iodine) maintain adequate levels in the fetus during pregnancy. The TMNRY 3 symptoms of the disorder become apparent during the ▪ The treatment for true hypothyroidism is the first 3 months of life in a formula-fed infant and at about oral administration of synthetic thyroid 6 months in a breastfed infant. Because congenital hormone (sodium levothyroxine). A small dose hypothyroidism leads to both severe, progressive is given at first, and then the dose is gradually physical and cognitive challenges, early diagnosis is increased to therapeutic levels. crucial. ▪ The child needs to continue taking the synthetic thyroid hormone indefinitely to ASSESSMENT supplement that which the thyroid does not o A screening test for hypothyroidism is make. mandatory at birth in the United States in all 50 ▪ Supplemental vitamin D may also be given to states (using the same few drops of blood prevent the development of rickets when, with obtained for a phenylketonuria [PKU] blood the administration of thyroid hormone, rapid spot test) bone growth begins o If an infant should miss this screening ▪ Further cognitive challenges can be prevented procedure, an early sign that parents report is as soon as therapy is started, but any degree that their child sleeps excessively, but because of impairment that was already present cannot the tongue is enlarged, they notice respiratory be reversed, making the disorder one of the difficulty, noisy respirations, or obstruction. most preventable causes of mental o The child may also suck poorly because of development delay known sluggishness or choking from the enlarged ▪ Be certain the parents know the rules for long- tongue. term medication administration with children, o The skin of the extremities usually feels cold, particularly the rule about not putting medicine dry, and perhaps scaly, and the child does not in a large amount of food (T4 tablets must be perspire. crushed and added to food or a small amount o Pulse, respiratory rate, and body temperature of formula or breast milk) and being certain all become subnormal. they have medicine during holidays or o Prolonged jaundice may be present due to the vacations. immature liver’s inability to conjugate bilirubin ▪ Periodic monitoring of T4 and T3 helps to o Anemia may increase the child’s lethargy and ensure an appropriate medication dosage. If fatigue. the dose of thyroid hormone is not adequate, o On a physical exam, the hair is brittle and dry, the T4 level will remain low and there will be and the child’s neck appears short and thick. few signs of clinical improvement. If the dose is o The facial expression is dull and open mouthed too high, the T4 level will rise and the child will because of the infant’s attempts to breathe show signs of hyperthyroidism: irritability; around the enlarged tongue. fever; rapid pulse; and perhaps vomiting, o The extremities appear short and fat; as diarrhea, and weight loss. muscles become hypotonic, deep tendon reflexes decrease and the infant develops a 5. ACQUIRED HYPOTHYROIDISM floppy, rag-doll appearance. (HASHIMOTO THYROIDITIS) o Generalized obesity usually occurs Hashimoto disease is the most common form of o Dentition will be delayed, or teeth may be acquired hypothyroidism in childhood. The age at defective when they do erupt. onset is most often 10 to 11 years. There may be a o The hypotonia affects the intestinal tract as family history of thyroid disease and it occurs more well, so the infant develops chronic often in girls than in boys. The decrease in thyroid constipation; the abdomen enlarges because secretion is caused by the development of an of intestinal distention and poor muscle tone. autoimmune phenomenon that interferes with thyroid o Many infants have an umbilical hernia. Infants production. have low radioactive iodine uptake levels, low serum T4 and T3 levels, and elevated thyroid- ASSESSMENT stimulating factor. o The excretion of thyroid-stimulating hormone o Blood lipids are increased. (TSH) from the pituitary increases when thyroid o An X-ray may reveal delayed bone growth hormone production decreases in an attempt o An ultrasound reveals a small or absent thyroid by the pituitary gland to increase thyroid gland. function. o Untreated, the condition will result in severe o In response to the increased level of TSH, irreversible cognitive deterioration or delay hypertrophy of the thyroid gland (goiter) can occur, and body growth is impaired by a lack of THERAPEUTIC MANAGEMENT T4, with prominent symptoms of obesity, ▪ Transient hypothyroidism usually fades by 3 lethargy, and delayed sexual development. months’ time. TMNRY 4  o Antithyroid antibodies will be present in serum although Graves disease often follows a viral if the illness was caused by an autoimmune illness or a period of stress. process. o With overproduction of T3 and T4, children o If the thyroid enlarges, it may become nodular gradually experience nervousness, tremors, as well. loss of muscle strength, and easy fatigue. o Although in childhood, a nodular thyroid is o Their basal metabolic rate, blood pressure, and usually benign, an investigation into the pulse all increase. possibility this could be a thyroid malignancy o Their skin feels moist, and they perspire freely must be considered. o They always feel hungry and, although they eat o For diagnosis, children are administered constantly, do not gain weight and may even radioactive iodine lose weight because of the increased basal o If the nodes are benign, there is generally a metabolic rate. rapid uptake of radioactive iodine (“hot o On X-ray, bone age will appear advanced nodes”). beyond the chronologic age of the child. Unless o If there is no uptake (“cold nodes”), carcinoma the condition is treated, the child is not likely to is a much more likely diagnosis (which is rare reach usual adult height because epiphyseal at this age). lines of long bones will close before full height can be attained. THERAPEUTIC MANAGEMENT o The thyroid gland, which usually is not ▪ Treatment for acquired hypothyroidism is the prominent in children, appears as a swelling on administration of synthetic thyroid hormone the anterior neck as goiter develops. (sodium levothyroxine), the same as for o In a few children, the eye globes become congenital hypothyroidism. prominent (exophthalmia), giving the child a ▪ With adequate dosage, the obesity diminishes wide-eyed, staring appearance. and growth begins again. o Laboratory tests show elevated T4 and ▪ It is important that the disease be recognized T3 levels and increased radioactive iodine as early as possible so there is time to uptake. stimulate growth before the epiphyseal lines o TSH is close at puberty. low or absent because the thyroid is being ▪ If acquired hypothyroidism exists in a woman stimulated by antibodies, not by the pituitary during pregnancy, her infant can be born gland. cognitively challenged because there was not o Ultrasound will reveal the enlarged thyroid. enough iodine present for fetal growth ▪ It is important, therefore, that girls with this THERAPEUTIC MANAGEMENT syndrome be identified before they each ▪ Therapy consists first of a course of a β- childbearing age adrenergic blocking agent, such as propranolol, to decrease the antibody 6. HYPERTHYROIDISM (GRAVES DISEASE) response. Hyperthyroidism is over secretion of thyroid hormones ▪ After this, the child is placed on an antithyroid by the thyroid gland. Neonatal Graves disease drug, such as propylthiouracil (PTU) or develops in the newborns of 1% to 2% of pregnant methimazole (Tapazole), to suppress the women who have the disease. Like transient formation of T4. hypothyroidism, this usually resolves between 3 to 12 ▪ While the child is taking these drugs, the blood weeks of age with no long-term results as the maternal is monitored for leukopenia (decreased white antibodies are cleared blood cell count) and thrombocytopenia (decreased platelet count)— side effects of In older children, overactivity of the thyroid gland can these drugs. occur from the glands being overstimulated by TSH ▪ If either of these results, the drug is from the pituitary gland due to a pituitary tumor. More discontinued until the white blood cell or frequently, however, hyperthyroidism in children is platelet count returns to normal, so the child caused by an autoimmune reaction that results in does not develop an infection or experience overproduction of immunoglobulin G (IgG), which spontaneous bleeding. Because the thyroid stimulates the thyroid gland to overproduce T4. An stores considerable thyroid hormone that must exophthalmos-producing pituitary substance causes be used up first before T4 levels decline, it the prominent-appearing eyes that accompany takes about 2 weeks for these drugs to have an hyperthyroidism in some children. effect. ▪ The child needs to continue to take the drug for ASSESSMENT 2 to 3 years before the condition “burns itself o Some children may have a genetic out.” predisposition to development of the disorder, ▪ The exophthalmos may not recede, but it will not become worse once therapy is instituted. TMNRY 5  ▪ If the child has a toxic reaction to medical ▪ A vasoconstrictor may be necessary to elevate management (severely lowered white blood the blood pressure. cell count or platelet count) or is noncompliant ▪ Although acute adrenal insufficiency is seen about taking the medicine, radioiodine ablative less often now than in the past because of the therapy with 131I or thyroid surgery to reduce availability of antibiotics that quickly halt the the size of the thyroid gland can be course of infectious disease because more accomplished. conditions are being treated with ▪ This has long-term effects, however, because corticosteroids than ever before, the chance after both radioiodine ablative therapy and the syndrome will occur from sudden thyroidectomy, supplemental thyroid hormone withdrawal of high-dose steroids is actually therapy may need to be taken indefinitely increasing. because the gland is no longer able to produce an adequate amount 8. PHENYLKETONURIA ▪ It is important that adolescent girls be carefully Condition inherited through an autosomnal recessive regulated before they consider childbearing gene. It takes two parent-carriers before one out of four because hyperthyroidism during pregnancy children (25%) can have the disease. can lead to neonatal hyperthyroidism in a fetus. An inborn error of metabolism transferred as an autosomnal recessive gene in which there is 7. ACUTE ADRENOCORTICAL INSUFFICIENCY decreased liver enzyme phenylalanine hydroxylase, Insufficiency (hypofunction) of the adrenal gland can resulting in the absence of decreased metabolism of occur in either an acute or chronic form. In either type, the amino acid phenylalanine to tyrosine needed to the function of the entire gland suddenly becomes form melanin: nonproductive. Usually, this occurs following a severe 1. Phenylalanine rises in the blood, causing brain overwhelming body infection such as damage and mental retardation. meningococcemia. It also can occur when 2. The absence of melanin causes blond hair, corticosteroid therapy such as prednisone, which has blue eyes, and fair skin. been maintained at high levels for a long period, is 3. The dietary management of ‘no phenylalanine’ abruptly stopped and the gland does not return to usual for six to eight years and a low-protein diet function. results in phenylalanine deficiency. ASSESSMENT ASSESSMENT o With acute adrenocortical insufficiency, the Signs/ Symptoms child’s blood pressure drops to extremely low 1. Signs of phenylalanine deficiency: diarrhea, levels, the child appears ashen gray, and the anorexia, lethargy, anemia, and skin rashes pulse will be weak. (D-A-L-A-S) o Temperature gradually becomes elevated; 2. Melanin deficiency: blond hair, blue eyes, and dehydration and hypoglycemia (an abnormally fair skin. low concentration of blood glucose) become 3. Abnormal neurologic development: mental marked because cortisol is no longer present retardation. to regulate this. 4. Musty odor of urine o As sodium and chloride blood levels fall from a lack of aldosterone production, the potassium Diagnostic Procedures level becomes elevated due to the usual 1. Guthrie capillary blood test. A screening test inverse relationship between sodium and done for two to four days after birth: potassium values. a. The screening test may result in a false o The child appears prostrate and seizures may negative because the newborn had no occur. adequate protein intake during testing. o Without treatment, death can occur abruptly b. To prevent a false negative result, there should be adequate preparation: high- THERAPEUTIC MANAGEMENT protein diet (milk-feeding) for 24-48 hours ▪ Acute adrenocortical insufficiency is a medical prior to screening test. emergency. 2. Ferric chloride urine test. An out-patient ▪ Treatment involves the immediate replacement follow-up screening test done in the community of cortisol (with IV hydrocortisone sodium by the community health nurse: succinate [SoluCortef]); the administration of a. effective only when the infant is over two deoxycorticosterone acetate (DOCA), the weeks old, when brain damage may have syntheticequivalent of aldosterone; and IV 5% occurred; glucose in normal saline solution to restore b. Ferric chloride (10%) is dropped onto the blood pressure, sodium, and blood glucose infant’s diaper wet with urine. If PKU is levels. present, the spot on the diaper will turn green TMNRY 6 THERAPEUTIC MANAGEMENT Treatment 1. Dietary treatment: phenylalanine-free formula; lofenalac – a milk-free formula - for six to eight years to prevent mental retardation. 2. Low-protein, low-phenylalanine diet after full brain development Nursing Care 1. Implement dietary management: a. Lofenalac to prevent high levels of phenylalanine, which can cause mental ETIOLOGY retardation. The disease apparently results from b. Restrict foods high in phenylalanine and immunologic damage to islet cells in protein: meat, eggs, green vegetables, and susceptible individuals. fruits. Apple may be given, as it is low in Why autoimmune destruction of islet cells phenylalanine occurs is unknown, but children with the c. Dietary management in PKU is to maintain disorder have a high frequency of certain phenylalanine at a safe level: between 3-7 human leukocyte antigens (HLAs), particularly mg/100mL; high levels can cause mental HLA-DR3 and HLA-DR4, located on retardation. chromosome 6, that may lead to susceptibility. d. Nutrition referrals as needed. If one child in a family has diabetes, the chance that a sibling will also develop the illness is 2. Provide health teaching higher than in other families because siblings a. The need for genetic counseling and also tend to have one of the specific HLA that screening of future children. Early are associated with the disease. screening is a MUST to prevent mental retardation. DISEASE PROCESS b. Instruct on indicated and contraindicated Insulin can be thought of as a compound that foods and the need for compliance with opens the doors to body cells, allowing them to dietary regimen. admit glucose, which is needed for functioning. 3. Provide psychological support: explain that It does not play a major role in glucose mental retardation is prevented with early transport into the brain, erythrocytes, implementation of dietary regimen. leukocytes, intestinal mucosa, or kidney epithelium. These cells, therefore, can survive insulin TYPE 1 DIABETES MELLITUS deficiency but not glucose deficiency. If Type 1 diabetes mellitus is a disorder that involves an glucose is unable to enter body cells because absolute or relative deficiency of insulin, which is in of a lack of insulin, it builds up in the contrast to type 2, where insulin production is only bloodstream (hyperglycemia) reduced Type 1 diabetes is equal in incidence in boys As soon as the kidneys detect hyperglycemia and girls and affects approximately 1 of every 500 (greater than the renal threshold of about 160 children and adolescents in the United States mg/dl), the kidneys attempt to lower it to normal levels by excreting excess glucose into the urine, causing glycosuria, accompanied by a large loss of body fluid (polyuria). Excess fluid loss, in turn, triggers the thirst response (polydipsia), producing the three cardinal symptoms of diabetes: polyuria, polydipsia, and hyperglycemia. Because body cells are unable to use glucose but still need a source of energy, the body begins to break down protein and fat. If large amounts of fat are metabolized this way, weight loss occurs and ketone bodies, the acid end product of fat breakdown, begin to accumulate in the bloodstream (creating high serum cholesterol levels and ketoacidosis) and spill into the urine as ketones. TMNRY 7  Potassium and phosphate, attempting to serve application of lidocaine/prilocaine [EMLA] as buffers, pass from body cells into the cream to finger stick or venipuncture sites and bloodstream. From there, they are evacuated, use of intermittent infusion devices greatly causing a loss of these important electrolytes. reduces this problem). Untreated diabetic children, therefore, lose Do not take blood for glucose analysis from weight, are acidotic due to the buildup of functioning IV tubing to try to ketone bodies in their blood, are dehydrated help with pain because the glucose in the IV because of the loss of water, and experience solution will cause the serum reading to be an electrolyte imbalance because of the loss of abnormally high. potassium and phosphate in urine. Because large amounts of protein and fat are Other Diagnostic Test being used for energy instead of glucose, - If diabetes is detected, the diagnostic workup children lack the necessary components for also usually includes an analysis of blood growth; they therefore remain short in stature samples for pH, partial pressure of carbon and underweight. dioxide (PCO2), sodium, and potassium levels; a white blood cell count; and a glycosylated ASSESSMENT hemoglobin (HbA1c) evaluation. o Although children may be prediabetic for some - Normally, the hemoglobin in red blood cells time, the onset of symptoms in childhood is carry only a trace of glucose. If serum glucose usually abrupt. is excessive, however, excess glucose o Parents notice increased thirst and increased attaches itself to hemoglobin molecules, urination (which may be recognized first as creating HbA1c. In nondiabetic children, the bed-wetting [enuresis] in a previously toilet- usual HbA1c value is 1.8 to 4.0. A value greater trained child). than 6.0 reflects an excessive level of serum o The dehydration may cause constipation. glucose. - Measuring HbA1c has advantages because it Laboratory Studies not only provides information on what is the In some children, diabetes is detected at a child’s present serum glucose level but what routine health screening. the serum glucose levels have been during the For others, although the disease has been preceding 3 to 4 months (red blood cells have progressing internally for some time, outward a life span of 120 days). If the potassium level symptoms have such an abrupt onset that the of the blood is low, a child may need an child is in a coma from acidosis and electrocardiogram to observe for T-wave hyperglycemia by the time it is detected. abnormalities, the mark of potassium Laboratory studies usually show a random deficiency. plasma glucose level greater than 200 mg/dl - The white blood cell count of a child with (normal range, 70 to 110 mg/dl fasting; 90 to diabetes may be elevated even though no 180 mg/dl not fasting) and significant infection is present, apparently as a response glycosuria Two diagnostic tests, the fasting to the ketoacidosis. blood glucose test and the random blood - The presence of infection must always be glucose test, are used to confirm diabetes. suspected, however, because it is often a precipitant to a diabetic crisis. For this reason, A diagnosis of diabetes is established if one of the nose and throat cultures may be obtained as following three criteria is present on two separate well. occasions: Symptoms of diabetes plus a random blood THERAPEUTIC MANAGEMENT glucose level greater than 200 mg/dl ▪ Therapy for children with type 1 diabetes A fasting blood glucose level greater than 126 involves five measures: insulin administration, mg/dl regulation of nutrition and exercise, stress A 2-hour plasma glucose level greater than 200 management, and blood glucose and urine mg/dl during a 75-g oral glucose tolerance test ketone monitoring. (GTT) The Initial Regulation of Insulin Typically, a GTT involves the oral ingestion of When children are first diagnosed with a concentrated glucose solution followed by diabetes, they are usually hyperglycemic and blood glucose levels drawn at fasting perhaps ketoacidotic. (baseline), after 1 hour, and after 2 hours. To correct the metabolic imbalance, they are The test is difficult for children to undergo given insulin administered IV at a dose of 0.1 because it requires them to fast for 8 hours, to 0.2 units per kilogram of body weight per drink an overly sweet solution, and submit to hour. painful, intrusive procedures (routine TMNRY 8  This initial IV infusion of insulin is then gradually reduced once the blood glucose level is lower than 200 mg/dl. Ideally, within 12 hours, the acidosis is considerably less than when a child was admitted to the hospital, and the serum glucose level is near the normal range. The insulin given for emergency replacement this way is regular (short-acting) insulin such as Humulin-R because this is the form that takes effect most quickly. MICROCEPHALY It may seem that in a child with diabetes in a The brain fails to grow state of acidosis, the administration of glucose May be due to chromosomal defect or from would not be warranted. Because the child is drugs, toxins or radiation MR being given insulin, however, body cells soon become ready to use glucose, so they require, incorporate, and use available glucose quickly. If more glucose is not provided, cells are forced to continue to break down fats and protein, and the acidosis can increase, not decrease. Glucose, therefore, may be added to the infusion After 24 hours, as the child’s serum glucose HYDROCEPHALUS returns to normal, oral feedings may replace Imbalance of CSF absorption or production the IV route. caused by malformations, tumors hemorrhage, Further management in the days after this first infection, trauma crucial 24-hour period is based on serum glucose determinations. Types: A child may remain on regular insulin Communicating – impaired absorption within given SC alone (given three or four times a arachnoid space day) for the first 1 or 2 days. Non-communicating – obstruction of CSF Typically, intermediate-acting insulin is then flow within the ventricular system added as soon as oral fluids are taken, usually on the second day of therapy. ASSESSMENT Infant – increased HC Macewen’s sign – cracked-pot soound on percussion of bones of head Anterior fontanel tense, bulging Scalp veins dilated Frontal bossing, sunsetting eyes M7L1: NEUROLOGIC DISORDERS Child – behavior changes Headache, nausea and vomiting Ataxia, nystagmus CRANIAL DEFECTS CRANIOSYNOSTOSIS SURGICAL IMPLEMENTATION One or more of the sutures will close too soon Goal: to prevent further CSF accumulation by ICP increases; interferes with normal brain byppassing the blockage and draining the fluid from the growth MR ventricles to a location where it may be reabsorbed ASSESSMENT 1. VP SHUNT – CSF drains into the peritoneal Suture lines of the skull manually palpated cavity from the lateral ventricle Radiographs are made to confirm 2. AV SHUNT – CSF drains into the right atrium TMNRY 9 Post OP Care IMPLEMENTATION Otitis media Evaluate sac; measure lesion Observe increase ICP – if present, elevate Neuro check HOB 15-30 deg Monitor for increase ICP Monitor for infection Measure HC; assess fontanelles Measure HC Protect the sac Monitor I and O 1. Cover with sterile, moist (normal saline) Provide comfort measures; administer non-adherent dressing medications (diuretucs, antibiotics, or 2. Change dressing every 2-4 hours anticonvulsants) Place prone position Toddler – headache and anorexia – earliest Head is turned to one side for feeding common signs of shunt malfunction Diapering may be C/I until defect repaired Aseptic technique Watch for early signs of infection SPINA BIFIDA Administer antibiotics CNS defect that occurs as a result of neural Administer anticholinergics – improve urinary tube failure to close during embryonic continence development Administer laxatives, antispasmodics Defect closure usually done during infancy MENINGITIS Types: Infectious process of the CNS caused by 1. SPINA BIFIDA OCCULTA bacteria and viruses Posterior vertebral arches fail to close Acquired as a primary or as a result of in the lumbosacral area complications Spinal cord intact; not visible Diagnosis – CSF analysis (increase pressure, Meninges not exposed on the skin cloudy CSF, high protein, low glucose surfaces Bacterial or viral 2. MENINGOCOELE Protrusion involves meninges and a ASSESSMENT sac-like cyst Signs and symptoms vary depending of age Lumbosacral area group 3. MYELOMENINGOCOELE Fever, chills Protrusion of meninges, CSF, nerve Vomiting, diarrhea roots, portion of spinal cord Poor feeding or anorexia Sac covered by a thin membrane, may rupture or leak Altered LOC Neuro deficit evident Bulging anterior fontanel Nuchal rigidity ASSESSMENT Depends on spinal cord involvement Visible spinal defect Flaccid paralysis of legs Altered bladder and bowel function TMNRY 10 IMPLEMENTATION IMPLEMENTATION Isolation; maintain for at least 24 hours after Early recognition antibiotics are initiated PT, OT, speech therapy, education and Administer antibiotics as prescribed recreation Monitor VS and neuro status Assess the child’s developmental level and Monitor I and O intelligence Assess nutritional status Early intervention Determine close contacts of the child with Encourage communication and interaction with meningitis the child on a functional level provide safe environment SEIZURE DISORDERS position upright after meals Sudden transient alterations in brain function provide safe, appropriate toys for age and resulting from excessive levels of electrical developmental level. activity in the brain ASSESSMENT M7L2: EENT DISORDERS Obtain information from patents about the time of onset, precipitating events and behavior before and after the seizure. DIPLOPIA DOUBLE VISION SEIZURE PRECAUTIONS: Vision occurs because light rays reflect from an 1. Raise side rails object through the cornea, aqueous humors, 2. Pad side rails lenses, and vitreous humors to the retinas 3. Place waterproof mattress on bed If any of these structures have defects, light 4. Instruct child to swim with companion rays, may not be able to reach the retinas or 5. Alert caregivers to the need for special focus correctly there, resulting in vision precautions disturbance Each eye globe must develop good central and Emergency Treatment for Seizures: peripheral vision, However, in addition, fusion Ensure patency of airways must occur- that is, both eyes together must If the child is standing or sitting, ease the child interpret a visual image as one image fusing down to the floor visual perception into a single image as one Place pillow or folded blanket under the child’s image, fusing visual perception into a single head image Loosen restrictive clothing Infants with poor eye alignment cannot establish binocular vision Clear area of any hazards If vomiting occurs, turn child to one side ETIOLOGY Do not restrain child; do not place anything in It is usually the result of impaired function of the the child’s mouth extraocular muscles (EOMs), where both eyes Prepare to administer medications are still functional but they cannot converge to target the desired object CEREBRAL PALSY Problems with EOMs may be due to Disorder characterized by impaired movement mechanical problems, disorders of the and posture resulting from an abnormality in neuromuscular junction, disorders of the the extrapyramidal motor system cranial nerves (III, IV, and VI) that stimulate the Spastic type – most common muscles, and occasionally disorders involving the supranuclear oculomotor pathways or ASSESSMENT ingestion of toxins. Extreme irritability and crying Diplopia is often one of the first signs of a Feeding difficulties systemic disease, particularly to a muscular or Stiff and rigid arms and legs neurological process, and it may disrupt a Delayed gross development person’s balance, movement, and/or reading Abnormal motor performance abilities Alterations of muscle tone Abnormal posturing CLASSIFICATION Persistence of primitive ref lexes BINOCULAR Binocular diplopia is double vision arising as a result of strabismus (cross-eyed) TMNRY 11 MONOCULAR INTERVENTION Diplopia can also occur when viewing with only Encourage fluids one eye Upright position when feeding Avoid chewing – increases pain TEMPORARY Have the child lie with the affected ear down Temporary diplopia can be caused by alcohol Instruct on appropriate technique to clean intoxication or head injuries, such as drainage from the ear with sterile cotton swabs concussion Administer analgesics and antibiotics (10-14 days) VOLUNTARY Screening for hearing loss Some people are able to consciously uncouple Otic medications their eyes, either by over focusing closely (i.e. 1. If younger than age 3 – auditory canal is going cross eyed) or unfocusing. straightened by pulling the pinna down and back MEDICAL MANAGEMENT 2. If older than 3 years – pull pinna up and The appropriate treatment for binocular back diplopia will depend upon the cause of the condition producing the symptoms. Efforts MYRINGOTOMY must first be made to identify and treat the Insertion of tympanoplasty tubes into the underlying cause of the problem middle ear to equalize pressure and keep ear Treatment options include: aerated Eye exercises Keep ears dry Wearing an eye patch on alternative eyes Earplugs should be worn during bathing, Prism correction shampooing, swimming Surgery NURSING MANAGEMENT Pre and post operative management M7L3: MUSCULOSKELETAL DISORDERS Safety Assistance in daily activities CONGENITAL CLUBFOOT From the latin word talus (ankle) and pes (foot) DISORDER OF THE EAR – OTITIS MEDIA a. Talipes Equinovarus Infection of the middle ear which occurs as a Characterized by internal tibial result of a blocked Eustachian tube that torsion, plantarflexion, inversion prevents normal drainage and adduction of the forefoot A common complication of an Acute b. Calcaneovalgus Respiratory Infection (ARI) Foot turns out and the heel is held Infants and children more prone – ET shorter, lower than the anterior foot wider, straighter Both are managed through serial Usually caused by Streptococcus pneumoniae casting, corrective surgery or shoe and Haemophilus influenzae (esp. children