Transcript Growth Hormone Deficiency

Endocrine Disorders
Jan Bazner-Chandler
CPNP, MSN, CNS, RN
BMI
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In recent years, BMI has received increased attention for
pediatric use. In 1994, an expert committee charged with
developing guidelines for overweight in adolescent
preventive services (ages 11-21 years) recommended that
BMI be used routinely to screen for overweight
adolescents. In addition, in 1997 an expert committee on
the assessment and treatment of childhood obesity
concluded that BMI should be used to screen for
overweight children, ages 2 years and older, using the BMI
curves from the revised growth charts.
BMI Calculation
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Can be calculated on-line at various sites including
www.cdc.gov
Good teaching site for parents:
http://www.cdc.gov/healthyweight/assessing/bmi/childrens
_bmi/about_childrens_bmi.html
Growth Charts
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The growth charts consist of a series of percentile curves
that illustrate the distribution of selected body
measurements in U.S. children. Pediatric growth charts
have been used by pediatricians, nurses, and parents to
track the growth of infants, children, and adolescents in
the United States since 1977. The 1977 growth charts
were developed by the National Center for Health
Statistics (NCHS) as a clinical tool for health professionals
to determine if the growth of a child is adequate. The
1977 charts were also adopted by the World Health
Organization for international use.
Tests
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Analysis of growth chart
Renal: BUN, Creatinine, urinalysis
Thyroid: TSH, T3 and T4
Infection: C-reactive protein (CRP) and Erythrocyte
sedimentation rate (ESR)
Anemia: Hgb and Hmct, iron levels if indicated
Growth hormone levels
Radiographs: bone age studies
Endocrine Disorders
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Growth hormone deficiencies
Hypo and hyper thyroid
Diabetes type I and type II
Diabetes Insipidus
Disorders of the Pituitary Gland
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Disorders of the pituitary gland depend on the location
of the lesion or physiologic abnormality.
Anterior Pituitary
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The anterior pituitary is made up of endocrine glandular
tissue and secretes growth hormone (GH),
adrenocorticotropic hormone (ACTH, TSH, FSH, LH, and
prolactin).
Growth Hormone Deficiency
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Failure of the pituitary to produce sufficient growth
hormone to sustain normal growth in children
80% are idiopathic
Familial patterns
Males are referred more often
Short at birth or preemie
Normal birth weight / height
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Birth weigh
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6 lb 2 oz to 9 lb 2 oz
Length
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19 to 21 inches
Assessment
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Cherub facial features, frontal bossing, large eyes, and
button nose
Males have small testes / micro-penis
Look much younger than chronological age
Delay of onset of puberty as a teenager
Emotional Difficulties
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Emotional difficulties related to small stature are common
Short child is often treated as if younger
Teased by peers
Child may dress as a younger child
Body image is altered
Hypopituitarism
Diagnostic Tests
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Renal and Liver function test
Thyroid function
Sedimentation rate / ESR
Done to rule out other causes of delayed growth
Definitive Diagnosis
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Deficiency in the Growth Hormone
Bone age by x-ray: delayed bone age
Slow growth rate: as documented on standard CDC
growth chart
Goals of Therapy
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The goal of therapy is to augment growth so that at the
time of epiphyseal close, a normal or normally expected
adult height is attained.
Child will attain a final adult height consistent with their
genetic potential
Growth Hormone Replacement
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GH products are currently labeled for use in “children
who have growth failure due to an inadequate secretion
of normal endogenous growth hormone”
Hormone Replacement Therapy
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Parents and child need to be educated on proper way to
reconstitute and administer the GH.
Videos available on line
Subcutaneous injection daily
Interdisciplinary Interventions
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Children should be managed by a pediatric
endocrinologist
Height and weight is obtained every 3 months and
plotted on the growth chart
Monitor thyroid
Bone age study yearly
Ethical Issues
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Social Justice Considerations
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Children must meet specific criteria to be eligible for
treatment
Parents must have access to health insurance coverage
Children who receive GH therapy will obtain the economic
and social benefits of growing taller
Long Term Effects
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Long term follow up needed:
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Long term risks unknown
Physiologic trauma of daily injection
Metabolic effects of the therapy: children on GH therapy are
usually lean and muscular
Therapy associated with increase risk of malignancies: leukemia,
lymphoma, and tumors especially when given in children who
have had childhood cancer
Precocious Puberty
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Development of sexual characteristics before the usual
age of onset of puberty.
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Girls
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Breast development before 7.5 years
Pubic hair before 8.5 years
Menses before 9.5 years
Boys
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Secondary sexual characteristics before age 9
Assessment
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Chart growth on growth chart.
Chronological timing of pubertal events.
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Tanner Scale: true precocious puberty is characterized by 2
signs of puberty
Family history
Management / Prognosis
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Treatment to halt or reverse sexual development.
Treatment needs to be started prior to closure of
epiphysis.
Good outcomes if treatment stared early
Puberty
Delayed Puberty
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Failure to develop sexually at an appropriate age.
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Girls
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No breast development by age 13 or lack of menses within 5 years.
Boys
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Secondary sexual characteristics not started by 14 years of age.
Rule out any Endocrine Abnormalities
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12% will have a pathologic reason for delayed puberty
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Congenital adrenal hyperplasia
Hypothyroidism
Growth hormone deficiency
Pharmacologic Interventions
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Low dose testosterone for the male.
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Oral ethinyl estradiol for the girl.
Hypothyroidism
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Most common endocrine disorder of childhood
Hypothyroidism can be congenital, acquired, or secondary
Congenital Hypothyroidism
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Results from absence or abnormal development of the
thyroid gland or abnormal synthesis of thyroid hormone.
Most common cause is incomplete development of the
thyroid gland
Importance of Thyroid Hormones
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Thyroid hormones promote normal myelination during
brain development in the first two to three years of life
and normal skeletal growth
Regulates metabolism
Assessment
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Dull appearance
Feeding difficulties
Inactivity
Constipation
Characteristic faces
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Flat nasal bridge
Puffy eyelids
Thick protruding tongue
Low hairline
Large posterior fontanel
Diagnosis
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Diagnosis
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Positive health history
Physical findings
Low levels of T3 and T4
High levels of TSH
Neonatal screening is mandatory
Interdisciplinary Interventions
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Replacement of sodium-l-thyroxine
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Daily administration based on weight (mg/kg)
Monitor TSH, T3 and T4
Monitor growth and development
Frequent visits with emphasis on importance of life long
therapy
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medication needs to be taken daily
Dosage needs to be adjusted to infant / child growth
CaREminder
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Early diagnosis and prompt treatment of congenital
hypothyroidism is essential for normal growth and
development.
The greater the delay treating congenital hypothyroidism,
the greater the degree of cognitive challenge.
With early diagnosis and treatment, children with
congenital hypothyroidism can develop normally.
Acquired Hypothyroidism
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15% of Down Syndrome children are hypothyroid
Auto-immune type of thyroiditis is most often the cause
High TSH levels as young as 2 years of age
Difficult to diagnose due to overlap of symptoms
Hyperthyroidism
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Excessive secretion of thyroid hormone
More common in females 7:1
Genetic and immunologic components
HLA-B8
Autoimmune disease of unknown cause
Assessment
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Cry easily
Emotionally labile
Nervous
Short attention span
Can’t sit still / Hyperactive
Fatigue but unable to sleep at night
Accelerated growth / tall for age
Physical Exam
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Enlarged thyroid gland
Asymmetric or lobular
Patient may present with neck swelling
Exophthalmos
Diagnosis
History and Physical
Levels of T3 and T4 are increased
Levels of TSH are decreased
Pharmacologic Interventions
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Antithyroid drugs to block T 4 synthesis
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Prophylthiouracil
Methimazole (Tapaxole)
Permanent Treatment
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Radioactive Iodine is given to kill off some of the thyroid
cells
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Most common negative outcome is giving too much iodine that
all thyroid producing cells are killed.
Surgical removal of gland or nodule – not always possible
since often it is the entire gland resulting in
overproduction of the hormone.
Disorders of the Pancrease
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Diabetes Mellitus – type 1 diabetes
Type 2 Diabetes
Diabetes Mellitus / Type 1
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Lack of insulin production in the pancreas.
Autoimmunity involved in destruction of beta cells.
15 new cases per 100,000 children under 20 years of age.
Peak incidence between 10 and 14 years.
Diabetes Type I
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Result of a genetic-environmental interaction
Seasonal variation – midwinter to spring
Family history
Illness or infection preceding the onset
Virus triggers the autoimmune response
Genetic Marker
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Genetic Markers:
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HLA –DR4 and HLA – DR3
20 to 40 % more susceptible
Natural History
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Exposure of genetically predisposed individuals to
environmental triggers
Leads to inflammation of beta cells of the pancreatic islets
(islitis) and subsequent beta-cell injury.
Beta Cell Function
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Hyperglycemia
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80 to 90% if beta cell function must be lost
before hyperglycemia develops
Pathophysiology
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Insulin deficiency causes physiologic and metabolic
changes in the body.
Glucose from dietary sources cannot be utilized by the
cells.
Renal tubules have difficulty reabsorbing the glucose.
Pathophysiology
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If the blood glucose level exceeds the renal threshold for
glucose osmotic diuresis ensues.
Renal threshold: when serum glucose levels approach 180
mg/dl the renal tubules have difficulty re-absorbing the
glucose
Hyperglycemia impairs leukocyte function – yeast
infection
Assessment
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Weight loss – as much as 30%
Elevated blood glucose leads to osmotic diuresis.
(polyuria and thirst)
Protein and fat breakdown lead to weight loss.
Accumulation of ketones causes a drop in pH.
(metabolic acidosis) and spilling of ketones in the
urine
Presenting Symptoms
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Hyperglycemia / glucose in blood stream
Glucosuria / sugar in urine
Polyuria / increased urine output
Electrolyte imbalance from dehydration
Polydipsia / attempt to relieve dehydration
Polyphagia / attempt to compensate for lost calories
Diagnostic Tests
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Blood glucose levels greater than 200 mg/dL
Urine sample reveals glucosuria and possible ketonuria.
Glucose tolerance test would reveal low insulin levels in
the face of elevated glucose levels.
Goals of Management
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Short term goals:
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Prevent the development of ketosis.
Prevent electrolyte abnormalities and volume depletion
secondary to osmotic diuresis.
Prevent impairment of leukocyte function
Prevent impairment of wound healing
Long term goal: prevention of microcirculatory and
neuropathic changes
Interdisciplinary Interventions
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Administration of insulin
Blood glucose levels
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Initially before every meal
Every am when diabetes under control
Dietary management / refer to nutritionist
Glycosylated hemoglobin / reflects average glucose
concentration for preceding 2 to 3 months. (A1C)
Blood Glucose Levels
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Target levels
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Toddler and preschool: 100 to 180 mg/dL
School-age: 90 to 180 mg/dL
Adolescents (13 to 19 years): 90 to 130 mg/dL
Urine Ketones
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Test urine for ketones only if blood sugar greater than
250 or during illness
Insulin
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Insulin
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Short acting – often used to cover extra carbohydrate
consumption
Combination of regular and intermediate-acting insulin
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Morning and evening dosing
Children on mixed insulin dosage schedules tend to experience
hypoglycemic episodes at 11:30 and 2:30 as peaking of insulin
occurs.
Hypoglycemia
Symptoms:
 Rapid onset
 Shaky feeling, hunger
 Headache
 Dizziness
 Vital signs
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Shallow respirations
tachycardia
Tremors
Lab Values:
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Glucose = low, below 60
Ketones = negative
Urine output
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 sugar negative
 negative ketones
Treatment of Hypoglycemia
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Day time hypoglycemia:
 Simple concentrated sugars such as honey by mouth, hard
candy, sugar cubes, or glucose tablets will elevate the blood
sugar immediately. Orange juice or sugar containing soda or
fruit drink. (Blood Glucose less than 70 mg/dL)
 Eat a snack if next meal is more than an hour away
 Identify reason for hypoglycemia. In children it is often
increase in activity without increase in food intake.
Hypoglycemia Prevention
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Using rapid-acting or Lispro insulin
Infusion pump (8 to 10 years of age)
Night time snack
Check blood glucose before bedtime
Do not skip snacks
Eat an extra snack on days of strenuous exercise
Night time hypoglycemia
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Eat 1 ½ snacks if blood glucose is less than 100 to 120
mg/dL before going to bed
Make sure the blood glucose is 100 – 120 mg/dL before
going to bed
Hyperglycemia or DKA
Symptoms:
Onset = gradual
Lethargic, confused, weak
Thirsty
Abdominal pain often with nausea and vomiting
Signs of dehydration
Vital signs: deep, rapid respirations, fruity acetone breath,
and weak pulses
DKA – Diabetic Ketoacidosis
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Presenting symptoms may include:
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Altered level of consciousness
Dehydration
Electrolyte disturbances
Dysrhythmias
Shock
Complete vascular collapse
Diabetic Ketoacidosis
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Mild
Moderate
Severe
Mild DKA
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Hyperglycemia and ketonuria with an ability to take in
and retain oral fluids.
Management: increased fluid intake
Diet drinks when blood glucose > / = 240 and
supplemental insulin administration
Check urine ketone levels
Moderate DKA
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Hyperglycemia, ketonuria, and acidosis (ph between 7.25
and 7.4) associated with an impaired ability to retain oral
fluids.
Need emergency care: IV fluids (normal saline),
supplementary insulin ( regular insulin IV)
Management of underlying medical condition: infections,
trauma
Severe DKA
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Characterized by severe acidosis (ph < 7.25), dehydration,
hyperglycemia, ketosis and a variety of other symptoms
including Kussmaul respirations, alteration in mental
status, and unconsciousness. Severe dehydration may lead
to shock.
Management of severe DKA
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3 phases of management
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Resuscitation
Correction of acid-base, glucose and electrolyte abnormalities
Transition to daily routine
Resuscitation
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ABC’s: securing an airway, ensuring adequate ventilation,
and correcting shock with IV volume expanders such as
normal saline.
Phase 2 & 3
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Correct acid-base:
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Intravenous fluids and insulin (regular insulin IV drip)
Administration of bicarbonate if acidosis is severe
Slowly bring down plasma glucose levels to avoid cerebral
edema
Restart child on regular routine with emphasis on
teaching and review of routine
Life Management
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Management by endocrinologist
Insulin
Blood sugar monitoring
Diet
Exercise
Screen for retinopathy: ophthalmologic exam annually
Nutritional Management
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Goals of nutritional therapy
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Maintaining near-normal blood glucose by balancing food intake
with insulin and activity.
Achieving optimal serum lipid levels.
Providing appropriate calories for normal growth and
development.
Preventing and treating acute and long-term complications.
Improving overall health through optimum nutrition
Exercise
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Vital component to management of child with diabetes.
May decrease the amount of insulin required.
Enhances insulin absorption.
Important for normal growth and development.
Management During Exercise
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Eat a snack before exercising.
Exercise lasting less than 1 hour usually requires a small
snack / complex carbohydrate or protein.
Longer exercising may require more frequent snacks /
complex carbohydrates or a protein.
Insulin adjustment may be needed if hypoglycemia occurs
during the activity.
Check blood glucose after activity and before bedtime to
prevent night time hypoglycemia
Diabetes Type 2
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Between 8 and 45 percent of newly diagnoses cases of
childhood diabetes are type 2
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Type 2 diabetes is caused by resistance to insulin as well
as the inability of the pancreas to keep up with the
increase demand of insulin.
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Insulin resistance + chronic hyperglycemia
Type 2 diabetes
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85% of children are obese
Age of onset is middle to late puberty around 13 years
Minority populations have an especially high rate of type 2
diabetes
Native American, Alaska Native, African American and
Mexican American
Strong family history
Pathophysiology
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Most often associated with obesity, hypertension, elevated
cholesterol.
Characterized by peripheral insulin resistance with a
defect of insulin secretion (hyperinsulinemia).
Assessment
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Obesity: BMI greater than 30 (normal range is 15 to 17 in
the pediatric population)
Waist to hip ratio: apple shape
Acanthosis nigricans: hyper-pigmentation and thickening of
the skin into velvety irregular folds in the neck and
flexural areas – reflects hyperinsulinemia
Hypertension
+ family history of type 2 diabetes
Ethnicity
Acanthosis nigricans
Assessment
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Chronic hyperglycemia
Often diagnosed during routine physical
Girls often present with vaginal monilial infection
Diagnostic tests
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Plasma insulin and C peptide are high - reflecting insulin
resistance
Auto-antibodies to the islet cell are negative in type 2
Interdisciplinary Interventions
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Comprehensive education on importance of regular
exercise and how to self-monitor for blood glucose
levels.
Dietary management
Glucose-lowering agent: drugs that improve insulin
sensitivity such as Glucophage (Metformin)
A few may need Insulin to initiate control
Diabetes Insipidus
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Disorder of the posterior pituitary
It results in deficiency in the secretion of ADH
ADH concentrates urine
Deficiency result in massive renal loss of fluid
Pathophysiology
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Antidiuretic hormone works directly on the renal
collection ducts and distal tubules to increase membrane
permeability for water and urea.
A deficiency in ADH will cause failure of kidneys to
reabsorb water.
This leads to massive water loss
Causes
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Hypothalamic lesion
¼ occur after craniotomy
Idiopathic or familial
CaREminder
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The first symptom of central DI seen in children,
especially in infants, are irritability and incessant crying
that can only be alleviated with feeding water. Formula or
breast milk does not quench the child’s thirst.
Assessment: clinical signs / symptoms
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Polyuria (excessive urination)
Polydipsia (excessive thirst)
Onset on symptoms abrupt
In the older child nocturia and enuresis are common
Urine
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Very low specific gravity: 1.005
Colorless
NO glucose or ketones
Interdisciplinary Interventions
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Careful monitoring of child to prevent fluid and
electrolyte imbalance
Administering Desmopressin (DDAVP): synthetic
analogue of ADH
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Comes as nasal spray, injection, tablet form
Parent education
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Administration of the medication
Signs and symptoms of fluid imbalance: dehydration and
over-hydration
Sign of hypernatremia (irritability or change in behavior)
Wear medi-alert tag
Nursing Diagnosis
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Fluid volume deficit
Desmopressin: medication used to treat DI…over use
may result in fluid volume excess
Activity intolerance: due to dehydration, excessive thirst
and frequent urination