Growth Charts

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.

Endocrine Disorders

Growth hormone deficiencies Hypo and hyper thyroid Diabetes type I and type II Diabetes Insipidus PKU

Growth Charts

Disorders of the Pituitary Gland

Disorders of the pituitary gland depend on the location of the lesion or physiologic abnormality.

Posterior Pituitary

Secretes antidiuretic hormone (ADH or vasopressin) and oxytocin.

Anterior Pituitary

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

Hypopituitarism 80% are idiopathic Familial patterns 1 in 10,000 children Males are referred more often Short at birth or premie

Causes

Growth hormone produced by the pituitary gland If the pituitary gland doesnt produce enough hormones for normal growth, growth slows down or stops. Underdeveloped, damaged or malfunctioning pituitary gland.

Growth Hormone

GH stimulates the growth of all organs and tissues in the body, particularly the long bones.

Clinical Manifestations

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

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

Renal and Liver function test Thyroid function Sedimentation rate / ESR

Done to rule out other causes of delayed growth

Definitive Diagnosis

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

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

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

Children should receive GH injections daily and at a minimum of three times a week Treatment costs $10,000 to 50,000 dollars annually Therapy can last for 2-4 years or until the epiphyses close

Management

Children should be managed by a pediatric endocrinologist

Ethical Issues

Social Justice Considerations

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

Outcomes of Treatment

The child will verbalize positive feelings about his or her body image. The child will demonstrate an increase in age-appropriate activities with peers. Child will be able to participate in age related activities of daily living

Long Term Effects

Long term follow up needed:

Long term risks unknown Physiologic trauma of daily injection Metabolic effects of the therapy: children on GH therapy usually lean muscular Therapy associated with increase risk of malignancies: leukemia, lymphoma, and tumors

Hypersecretion of Growth Hormone

In children called gigantism Uncommon disease 15% due to pituitary tumors causing increase release of GH. Goal of treatment is surgical removal of GHsecreting adenoma.

Gigantism

Acromegaly
Anatomy & Physiology: Mosby

Precocious Puberty

Development of sexual characteristics before the usual age of onset of puberty.

Girls

Breast development before 7.5 years Pubic hair before 8.5 years Menses before 9.5 years Secondary sexual characteristics before age 9

Boys

Assessment

Chart growth on growth chart. Chronological timing of pubertal events.

Tanner Scale: true precocious puberty is characterized by 2 signs of puberty

Family history

Management / Prognosis

Treatment to halt or reverse sexual development. Treatment needs to be started prior to closure of epiphysis. Good outcomes if treatment stared early

Delayed Puberty

Failure to develop sexually at an appropriate age.

Girls

No breast development by age 13 or lack on menses within 5 years. Secondary sexual characteristics not started by 14 years of age.

Boys

Etiology and Incidence

2 to 3% of all adolescents. Bone age moderately delayed. History of small stature during infancy and early childhood. Familial history

Rule out any Endocrine Abnormalities

12% will have a pathologic reason for delayed puberty

Congenital adrenal hyperplasia Hypothyroidism Growth hormone deficiency

Management

Low dose testosterone for the male. Oral ethinyl estradiol for the girl.

Hypothyroidism

Most common endocrine disorder of childhood Hypothyroidism can be congenital, acquired, or secondary

Congenital Hypothyroidism

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

Thyroid hormones promote normal myelination during brain development in the first two to three years of life and normal skeletal growth Regulates metabolism

Clinical Manifestations

Dull appearance Feeding difficulties Inactivity Constipation Characteristic faces

Flat nasal bridge Puffy eyelids Thick protruding tongue Low hairline Large posterior fontanel

Diagnosis

Diagnosis

Positive health history Physical findings Low levels of T3 and T4 High levels of TSH Neonatal screening is mandatory

Management

Replacement of sodium-l-thyroxine Monitor TSH, T3 and T4 Monitor growth and development Frequent visits with emphasis on importance of therapy

Acquired Hypothyroidism

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

Excessive secretion of thyroid hormone More common in females 7:1 Genetic and immunologic components HLA-B8 Autoimmune disease of unknown cause

Clinical Manifestations

Cry easily Emotionally labile Nervous Short attention span Cant sit still / Hyperactive Fatigue but unable to sleep at night Accelerated growth / tall for age

Physical Exam

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

Treatment

Antithyroid drugs to block T 4 synthesis

Prophylthiouracil Methimazole (Tapaxole)

Permanent Treatment

Radioactive Iodine is given to kill off some of the thyroid cells

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.

Diabetes Mellitus / Type 1

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

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

Genetic Markers:

HLA DR4 and HLA DR3 20 to 40 % more susceptible

Natural History

Exposure of genetically predisposed individuals to environmental triggers Leads to inflammation of beta cells of the pancreatic islets (islitis) and subsequent betacell injury.

Beta Cell Function

Hyperglycemia 80 to 90% if beta cell function must be lost before hyperglycemia develops

Pathophysiology

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

If the blood glucose level exceeds the renal threshold for glucose osmotic diuresis ensues. Renal threshold: when serum glucose levels approach 200mg/dl the renal tubules have difficulty re-absorbing the glucose Hyperglycemia impairs leukocyte function yeast infection

Clinical Manifestations

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

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

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

Short term goals:

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

Interventions

Administration of insulin Blood glucose levels

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.

Blood Glucose Levels

Target levels

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

Test urine for ketones only if blood sugar greater than 250 or during illness

Insulin

Insulin

Short acting often used to cover extra carbohydrate consumption Combination of regular and intermediate-acting insulin

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

Lab Values:

Glucose = low, below 60 Ketones = negative Urine output

Shallow respirations tachycardia

Normal sugar negative negative ketones

Tremors

Treatment of Hypoglycemia

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

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

Eat 1 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 Check blood glucose at midnight and 3 am

Hyperglycemia
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

Presenting symptoms may include:

Altered level of consciousness Dehydration Electrolyte disturbances Dysrhythmias Shock Complete vascular collapse

Diabetic Ketoacidosis

Mild Moderate Severe

Mild DKA

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

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

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

3 phases of management

Resuscitation Correction of acid-base, glucose and electrolyte abnormalities Transition to daily routine

Resuscitation

ABCs: securing an airway, ensuring adequate ventilation, and correcting shock with IV volume expanders such as normal saline.

Phase 2 & 3

Correct acid-base:

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

Management by endocrinologist Insulin Blood sugar monitoring Diet Exercise Screen for retinopathy: ophthalmologic exam annually

Nutritional Management

Goals of nutritional therapy

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

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

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

Between 8 and 45 percent of newly diagnoses cases of childhood diabetes are type 2 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. Insulin resistance + chronic hyperglycemia

Type 2 diabetes

85% of children are obese Age of onset is middle to late puberty Minority populations have an especially high rate of type 2 diabetes Native American, Alaska Native, African American and Mexican American

Who is at risk?

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

Presenting Symptoms

Chronic hyperglycemia Often diagnosed during routine physical Girls often present with vaginal monilial infection Severe infections: pharyngitis or osteomyelitis:

Diagnostic tests

Plasma insulin and C peptide are high reflecting insulin resistance Autoantibodies to the islet cell are negative in type 2

Management

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

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

Causes

Hypothalamic lesion occur after craniotomy Idiopathic or familial

Pathophysiology

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

Assessment

Polyuria (excessive urination) Polydipsia (excessive thirst) Onset on symptoms abrupt In the older child nocturia and enuresis are common

CaReminder

The first symptoms of diabetes insipidus seen in children, especially in infants, are irritability and incessant crying that can only be alleviated with feedings of water and not formula or breast milk.

Urine

Very low specific gravity: 1.005 Dilute Colorless NO glucose or ketones

Management

Desmopressin (DDAVP): synthetic analogue of ADH Administered by nasal insufflation once or twice a day Aqueous pitressin may be given IV, IM or sub-q

Parent education

Administration of the medication Signs and symptoms of fluid imbalance: dehydration and over-hydration Sign of hypernatremia Wear medi-alert tag

Nursing Diagnosis

Fluid volume deficit Desmopressin: medication used to treat DIover use may result in Fluid volume excess Activity intolerance: due to dehydration, excessive thirst and frequent urination

Phenylketonuria

PKU First discovered in 1934 PKU is an autosomal recessive genetic defect found on chromosome 12 Child must receive the defective gene from both parents 1 in 60 people is an asymptomatic carrier Symptoms 1 in 10,000 births In turkey 23 in 10,000

Pathophysiology

Phenylalanine is an essential amino acid found in all protein food. The accumulation of phenylalanine leads to severe retardation. With early identification of the defective gene intervention can prevent retardation.

Diagnosis

Heel stick done 24 to 48 hours after birth. Infant must have an adequate intake of breast milk or formula. (protein) The drop of blood must be large enough to fill the imprinted space on the filter paper. Squeezing out more blood onto the paper creates a layered effect that can produce a false-positive test result.

Treatment

Focuses on preventing excessive accumulation of phenylalanine by restricting protein intake. Maintain levels below 0.9 mmol/L but maintain at0.2 to allow for normal growth and tissue repair. Aspartame or NutraSweet need to be avoided in diet.

Prognosis

Teaching that reinforces the dietary regimen is critical to the successful management of PKU Family cohesion and adherence to the restricted diet positively correlates with higher IQ levels. Children at high risk for learning difficulties. Diet generally discontinued around 10 years with full brain development Pregnant women with PKU deficiency at high risk for having a fetus with mental retardation.