Chapter 48: Nursing Care of a Family

When a Child Has an Endocrine or a

Metabolic Disorder
A. Pituitary Gland
- is directed by the hypothalamus, an organ located in the center of the brain and which
serves as the regulator of the autonomic nervous system
- About 1 cm long, 1.0 to 1.5 cm wide, and 0.5 cm thick
- rests in the sella turcica, a depression of the sphenoid bone

PITUITARY GLAND DISORDERS

1. Growth hormone deficiency
 not able to grow to full size
 children may appear well proportioned but measure well below the
average on a standard growth chart
 may result from a nonmalignant cystic tumor of embryonic origin that
places pressure on the pituitary gland or from increased intracranial
pressure as a result of trauma

Assessment Treatment
 child begins to fall below  administration of intramuscular
the third percentile of recombinant human growth
height and weight on hormone (rhGH) usually given
growth charts daily at bedtime, the time of
 face appears infantile day at which GH normally
because the mandible is peak
 recessed and immature,  suppression of luteinizing
and the nose is usually hormone–releasing hormone
small (LHRH, or
 child’s teeth may be gonadotropinreleasing
crowded in a small jaw hormone [GnRH]) to delay
(and may erupt late) epiphyseal closure
 child’s voice may be high
pitched, and the onset of
pubic, facial, and axillary
hair and genital growth
will be delayed

2. Growth Hormone Excess

 overproduction of GH usually is caused by a benign tumor of the anterior
pituitary (an adenoma)

Assessment Treatment
 excessive or overgrowth  GH antagonist such as
 tongue can become so bromocriptine (Parlodel)
enlarged and thickened that taken orally or octreotide
it protrudes from the mouth, (Sandostatin) taken by
giving the child a dull, injection can slow the
apathetic appearance and production of GH
making it difficult to
 articulate words

3. Diabetes Insipidus
 a disease in which there is decreased release of ADH by the pituitary
gland
 causes less reabsorption of fluid in the kidney tubules.
 Urine becomes extremely dilute, and a great deal of fluid is lost from the
body
 may reflect an X-linked dominant trait, or it may be transmitted by an
autosomal recessive gene
 also result from a lesion, tumor, or injury to the posterior pituitary, or it
may have an unknown cause

Assessment Treatment
 excessive thirst (polydipsia)  Surgery is the treatment of
that is relieved only by choice if a tumor is present.
drinking large amounts of  If the cause is idiopathic, the
water condition can be controlled
 accompanying polyuria by the administration of
 specific gravity of the urine desmopressin (DDAVP), an
will be as low as 1.001 to arginine vasopressin
1.005 (normal values are
more often 1.010 to 1.030)
  Urine output may reach 4 to
10 L in a 24-hour period
(normal range, 1 to 2 L),
depending on age
 Urine output may reach 4 to
10 L in a 24-hour period
(normal range, 1 to 2 L),
depending on age

4. Syndrome of Inappropriate Antidiuretic Hormone (SIADH)

 a rare condition in which there is overproduction of ADH by the posterior
pituitary gland.
 results in a decrease in urine production, which leads to water
intoxication
 can be caused by central nervous system infections such as bacterial
meningitis, long-term positive pressure ventilation, or pituitary
compression such as could occur from edema or a tumor.

Assessment Treatment
 weight gain, concentrated  restriction of fluid and
urine (increased specific supplementation of sodium
gravity), nausea, and by IV fluid if needed.
vomiting  Demeclocycline
 (Declomycin), a tetracycline
antibiotic that has the side
effect of blocking the action
of ADH in renal tubules and
reducing resorption of water,
may be prescribed

B. Thyroid Gland
- located at the front of the neck, is responsible for controlling the rate of metabolism in
the body through the hormones thyroxine (T4 ) and triiodothyronine (T3 ), which are
produced by its follicular cells

THYROID GLAND DISORDERS

1. Congenital Hypothyroidism
 occurs as a result of an absent or nonfunctioning thyroid gland in a
newborn
 an indication that the infant’s thyroid is not functioning well may not be
noticeable at birth because the mother’s thyroid hormones
 (unless she ingested less than usual amounts of iodine) maintain adequate
levels in the fetus during pregnancy.

Assessment Treatment
 child sleeps excessively, but  oral administration of
 because the tongue is synthetic thyroid hormone
enlarged, they notice (sodium levothyroxine)
respiratory difficulty, noisy  Supplemental vitamin D may
respirations, or obstruction also be given to prevent the
 suck poorly because of development of rickets when,
sluggishness or choking with the administration of
from the enlarged tongue thyroid hormone, rapid bone
 skin of the extremities growth begins
usually feels cold, dry, and
perhaps scaly, and the child
does not perspire
 Pulse, respiratory rate, and
body temperature all
become subnormal
 Prolonged jaundice may be
present due to the immature
liver’s inability to conjugate
bilirubin

2. Acquired Hypothyroidism (Hashimoto Thyroiditis)

 most common form of acquired hypothyroidism in childhood; the age at
onset is most often 10 to 11 years.

Assessment Treatment
  increased level of TSH  administration of synthetic
 hypertrophy of the thyroid thyroid hormone (sodium
gland (goiter) can occur levothyroxine), the same as
 body growth is impaired by for congenital
a lack of T4 , with hypothyroidism
prominent symptoms of
obesity, lethargy, and
delayed sexual development

3. Hyperthyroidism (Graves Disease)

 oversecretion of thyroid hormones by the thyroid gland

Assessment Treatment
 overproduction of T3 and T4  first of a course of a β-
, children gradually adrenergic blocking agent,
experience nervousness, such as propranolol, to
tremors, loss of muscle decrease the antibody
strength, and easy fatigue. response
 basal metabolic rate, blood  After this, the child is placed
pressure, and pulse all on an antithyroid drug, such
increase as propylthiouracil (PTU) or
 skin feels moist, and they methimazole (Tapazole), to
perspire freely suppress the formation of T4
 always feel hungry and,
 although they eat constantly,
do not gain weight and may
even lose weight because of
the increased basal
metabolic rate

C. Adrenal Gland
- located retroperitoneally, just above the kidneys.
- They are made up of two distinct divisions; together, these divisions protect the body
against acute and chronic forms of stress.
- Three hormones—cortisol (a glucocorticoid responsible for glucose and protein
metabolism and preventing inflammation), androgen (a steroid hormone responsible for
muscle development), and aldosterone (a mineralocorticoid hormone necessary for
sodium and fluid balance)

ADRENAL GLAND DISORDERS

1. Acute Adrenocortical Insufficiency

Assessment Treatment
 child’s blood pressure  a medical emergency.
drops to extremely low  Treatment involves the
levels, the child appears immediate replacement of
ashen gray, and the pulse cortisol (with IV
 will be weak hydrocortisone sodium
 Temperature gradually succinate [SoluCortef]); the
becomes elevated; administration of
dehydration and deoxycorticosterone acetate
hypoglycemia (an (DOCA), the synthetic
abnormally low equivalent of aldosterone; and
concentration of blood IV 5% glucose in normal
glucose) become marked saline solution to restore
 the potassium level blood pressure, sodium, and
becomes elevated due to blood glucose levels. A
the usual inverse vasoconstrictor may be
relationship between necessary to elevate the blood
sodium and potassium pressure.
values.
 The child appears prostrate
and seizures may occur.

2. Congenital Adrenal Hyperplasia

 a syndrome that is inherited as an autosomal recessive trait and which
causes the adrenal glands to not be able to synthesize cortisol
 Because the adrenal gland is unable to produce cortisol, the level of
adrenocorticotropic hormone (ACTH) secreted by the pituitary increases
in an attempt to stimulate the gland to increase function.
 Assessment Treatment
 genital organs in a male  replace the cortisol that is
fetus to “overgrow,” or missing, thereby suppressing
increase in size; it ACTH concentrations and
masculinizes a female fetus normalizing adrenal size and
 usual adult height; pubic androgen production, a goal
and axillary hair, acne, and a that seems quite simple but is
deep masculine voice will actually very difficult to
appear precociously. achieve
 At puberty, there will be no  both male and female infants
breast development or are given a corticosteroid
menstruation. agent, such as oral
hydrocortisone, to replace
what they cannot produce
naturally

3. Salt-Losing Form of Congenital Adrenogenital Hyperplasia

 Without adequate aldosterone, salt is not retained by the body, so fluid is
not retained.
 Almost immediately after birth, affected infants begin to have vomiting,
diarrhea, anorexia, loss of weight, and extreme dehydration

Assessment Treatment
 detected before an infant  a need to be supplemented
 reaches an irreversible point with hydrocortisone, an
of salt depletion increased salt intake, and
DOCA, a synthetic
aldosterone, in order to
maintain a balance of fluid
and electrolytes

4. Cushing Syndrome
 caused by overproduction of the adrenal hormone cortisol; this usually
results from increased ACTH production due to either a pituitary or
adrenal cortex tumor.

Assessment Treatment
 increased glucose  Surgical removal of the
production; this causes fat to causative tumor.
accumulate on the cheeks,  The prognosis depends on
chin, and trunk, causing a whether the tumor is benign
moon-faced, stocky or malignant because a
appearance carcinoma of this type tends
 leads to muscle wasting, to metastasize rapidly.
making the extremities
 appear thin in contrast to the
trunk, and loss of calcium in
bones

D. Pancreas
- a unique organ in that it has both endocrine (ductless) and exocrine (with duct) types of
tissue
- islets of Langerhans form the endocrine portion, alpha islet cells have the responsibility
to secrete glucagon, and beta islet cells secrete insulin.
- Insulin is essential for carbohydrate metabolism and is also important in the metabolism
of both fats and protein

PANCREAS DISORDERS
1. Type I Diabetes Mellitus
 a disorder that involves an absolute or relative deficiency of insulin,
which is in contrast to type 2, where insulin production is only reduced

Assessment Treatment
 increased thirst and  involves five measures:
increased urination (which insulin, administration,
may be recognized first as regulation of nutrition and
bed-wetting [enuresis] in a exercise, stress management,
previously toilet-trained and blood glucose and urine
 child). ketone monitoring
 The dehydration may cause
constipation.

2. Type 2 Diabetes Mellitus

 characterized by diminished insulin secretion, is a separate disease from
type 1 diabetes because it is not caused by autoimmune factors
 children with T2D do not need daily insulin because their disease can be
managed with diet alone or with diet and an oral hypoglycemic agent

Assessment Treatment
 e increasing sex hormones  need good instruction in how
naturally increase insulin to manage their illness so,
resistance, creating a need whether they are home with
for more insulin production. their parents supervising their
Children’s urine will show care or away from home at
glucose but few ketones. camp or college, they can
Children experience prevent hyperglycemia and
lessened amounts of thirst the irritation to blood vessels
or increased urination which that cause
 dark shiny patches on the
skin (acanthosis nigricans),
which are most often found
between the fingers and
 between the toes, on the
back of the neck (“dirty
neck”), and in axillary
creases

E. Parathyroid Gland
 located posterior and adjacent to the thyroid gland, regulate serum levels of calcium
in the body by controlling the rate of bone metabolism through the secretion of
parathyroid hormone.
 This hormone is unique in that it is not under the control of the pituitary gland but
rather is controlled by a negative feedback from the circulating serum levels of
calcium and how much vitamin D is present to allow absorption of calcium from
the gastrointestinal tract into the bloodstream.

PARATHYROID GLAND DISORDER

1. Hypocalcemia
 is a lowered blood calcium level that occurs to some extent in all
newborns before they begin sucking well

Assessment Treatment
 neuromuscular irritability,  increasing the calcium level
referred to as latent tetany. in the blood above the point
 This occurs if the blood of latent tetany. This can be
calcium level falls below 7.5 administered orally as 10%
mg/dl. The newborn will calcium chloride if the
demonstrate jitteriness when infant can and will suck.
handled or if the infant has Otherwise, it will be given
been crying for an extended IV as a 10% solution of
period. calcium gluconate.

F. Metabolic Disorders (Inborn Errors of Metabolism)

1. Phenylketonuria
 a disease of metabolism, which is inherited as an autosomal
recessive trait. The infant lacks the liver enzyme phenylalanine
hydroxylase, which is necessary to convert phenylalanine, an
essential amino acid, into tyrosine (a precursor of epinephrine, T4 ,
and melanin).

Assessment Treatment
 causes urine to have a  Dietary restriction has been
typical musty or “mousy” the main treatment of PKU
odor that is so strong that it for over 50 years and still
often pervades not only the remains the main therapy.
 urine but the entire child.  Providing nutrition for a child
 child becomes blue-eyed with PKU becomes a difficult
with very fair skin and light task as the child grows older
blonde hair because there is no natural
protein with both a low
phenylalanine concentration
and normal levels of other
essential amino acids.
 Foods highest in
phenylalanine are those that
are rich in protein, such as
meats, eggs, and milk. Foods
low in phenylalanine include
orange juice, bananas,
potatoes, lettuce, spinach, and
peas. A formula such as
Lofenalac can be used to
prepare treat foods, such as
ice cream, milk shakes,
birthday cakes, and puddings

2. Maple Syrup Urine Disease

 a rare disorder, inherited as an autosomal recessive trait, in which
there is a defect in metabolism of the amino acids leucine,
 isoleucine, and valine, which leads to cerebral degeneration similar
to that observed in children with PKU.

Assessment Treatment
 Infants who have the  placed on a well-controlled
disorder appear well at birth diet that is high in thiamine
but quickly begin to show and low in the amino acids
signs of feeding difficulty, leucine, isoleucine, and
loss of the Moro reflex, and valine, cerebral degeneration
irregular respirations. The can be prevented, just as it
symptoms progress rapidly can be prevented in PKU.
to opisthotonos, generalized  Hemodialysis or peritoneal
muscular rigidity, and dialysis may be necessary to
seizures. If the condition temporarily reduce abnormal
remains untreated, an infant serum levels at birth or
may die of the disease as during a childhood infection,
early as 2 to 4 weeks of age when catabolism of cells
releases increased amino
acids into the bloodstream.

3. Galactosemia
 a disorder of carbohydrate metabolism that is characterized by
abnormal amounts of galactose in the blood (galactosemia) and in
the urine (galactosuria).
 Assessment Treatment
 lethargy, hypotonia, and  placing the infant on a diet
perhaps diarrhea and free of galactose or giving
vomiting. the child formula made with
 The liver enlarges as milk substitutes such as
cirrhosis develops. casein hydrolysates
 Jaundice is often present (Nutramigen)
and persistent, and bilateral
cataracts develop

4. Glycogen Storage Disease

 refers to a group of genetically transmitted disorders that involve
altered production and use of glycogen in the body.
 gen is normally stored in the liver to provide a reserve supply of
glucose.

Assessment Treatment
 child’s growth will be  need to eat a high-
stunted because there is carbohydrate diet with snacks
only glucose available for between meals to prevent
energy, not for growth. hypoglycemia.
 If hypoglycemic episodes  In addition, a continuous
have been severe, brain glucose nasogastric or
damage may result. gastrostomy feeding during
  Many children have a the night may be necessary to
tendency toward epistaxis prevent hypoglycemia while
or hemorrhage and are at sleeping.
risk when having surgery  Therapy with diazoxide
performed because of an (Proglycem), an
impaired clotting ability antihypoglycemic drug that
due to decreased platelet inhibits insulin release, may
adhesiveness. help regulate the glucose
level to provide additional
growth.
 Liver transplantation may be
a possibility,

5. Tay-Sachs Disease (Infantile GM2 Gangliosidosis)

 an autosomal recessively inherited disease in which the infant lacks
hexosaminidase A, an enzyme necessary for lipid metabolism

Assessment Treatment
 Children generally appear  No cure
well in the first few months of
life except for an extreme
Moro reflex and mild
 hypotonia. If left untreated, at
about 6 months of age, they
begin to lose head control and
are unable to sit up or roll
over without support
 By 1 year of age, children
will have developed
symptoms of spasticity and
are unable to perform even
simple motor tasks.
 By 2 years of age, generalized
seizures and blindness will
have occurred.