Endo

Question 1

puberty brings on many changes

Answer 1

increase in GH released, increased production of LH and FSH in girls, development is sexual characteristics, feedback mechanism in place

Question 2

data to collect for endocrine assessment

Answer 2

% in height/ weight, distinguishing facial features and abdominal fat, onset of puberty, routine NB screening, blood glucose levels, detection of chromosomal disorders

Question 3

new onset DM- baseline labs

Answer 3

glucose, urea, creat, lytes, gas, urine for glucose and ketones, TSH, thyroid antibodies

Question 4

new onset DM: diet

Answer 4

1000 kcal + 100kcal/ yr of age. ex 8 year old= 1,800kcal/ day

Question 5

insulin therapy

Answer 5

monitored q3 months by A1c- represents amt of glucose attached to hemoglobin over period of time, roughly 120 days, good predictor of levels over 6- 8 wks

Question 6

insulin adjustment: basic principles

Answer 6

new onset- making daily changes until stabilized
established pts- high BG same time of day for 3 consecutive days, increase 10% at a time. If unexplained lows 2x/ week same time of day, decrease 10- 20% at a time

Question 7

factors that may affect insulin dosage in kids

Answer 7

stress, infection, illness, growth spurts (puberty), meal coverage for finicky toddlers, adolescents concerned about not wanting to gain weight/ eat in AM

Question 8

insulin pumps

Answer 8

replace the need for periodic injections by delivering rapid- acting insulin continuously throughout the day using a catheter

Question 9

advantages of insulin pumps in kids

Answer 9

delivers cont infusion, maintains better control of BS, decrease number of injection sites, decrease hypo/ hyper episodes, more flexible lifestyle, eat with more flexibility, improves growth in child

Question 10

disadvantages of insulin pumps in kids

Answer 10

requires motivation and willingness to be connected to device, have to change the site every 2- 4 days, more time/ energy to monitor BS, syringe/ cath changes every 2- 3 days, infection may occur at site, weight gain common when BS is controlled

Question 11

“sick day guidelines”

Answer 11

day the child is ill, pay close attention to glycemic control, should take BS levels more often than routine, DO NOT SKIP INSULIN, factors key to preventing DKA

Question 12

DKA how common is it?

Answer 12

at diagnosis of diabetes- 15-67%

established pts- 1- 10% of pts/ year

Question 13

hypoglycemia causes

Answer 13

too much exercise which you didn’t plan, not enough food and/ or delay in getting the meal/ snack

Question 14

tx for severe hypoglycemia

Answer 14

glucagon-

5 yrs 1 mg

Question 15

mini dose glucagon protocol

Answer 15

persistently low but alert and unable to manage orally (e.g. during illness or inadvertent insulin error)

Question 16

risk factors for type II dm

Answer 16

ethnicity, female gender, family hx, intrauterine factors (large >4kg or small

Question 17

common physical sign on insulin resistance

Answer 17

acanthosis nigricans- looks like dirt on skin that won’t come off

Question 18

tx of type II dm in youth

Answer 18

education for family, set glycemic targets (A1c or equal to 9%, sx of severe hyperglycemia, ketonuria

Question 19

maturity- onset diabetes of the young MODY

Answer 19

transmitted as autosomal dominant disorder with formation of structurally abnormal insulin with decreased biologic activity, similar to type II but not, usually before age 25, may be seen in obese teens, may be controlled by oral hypoglycemic agents and diet, more benign dx but increasingly common in peds

Question 20

juvenile hypothyroidism (she said to read more about this in book)

Answer 20

congenital- congenital hypoplastic thyroid gland
acquired- partial or complete thyroidectomy from ca or thyrotoxicosis (following radiation from Hodgkins or other malignancy)
- rarely occurs from dietary insufficiency in US

Question 21

clinical manifestations of juvenile hypothyroidism

Answer 21

decelerated growth, constipation, sleepiness

myxedematous skin changes (dry skin, sparse hair, periorbital edema)

Question 22

therapeutic management of juvenile hypothyroidism

Answer 22

oral thyroid replacement therapy, prompt tx needed for brain growth in infants, may administer in increasing amounts over 4- 8 wks to reach euthyroidism
- compliance w/ meds is crucial

Question 23

tx/ meds for juvenile hypothyroidism

Answer 23

thyroxine 75- 100mcg/ m2/ day; monitor q6 months until growth complete, and then annually.

monitor: s/s hypo/ hyper thyroid, growth, sexual maturation, TSH (want 0.25- 5mU/L), +/- FT4
- recheck TSH 4- 6 wks after dose adjustments

Question 24

goiter

Answer 24

hypertrophy of the thyroid gland

• congenital- usually results from maternal ingestion of antithyroid during pregnancy
• acquired- result of neoplasm, inflammatory dx, dietary deficiency (but rarely in kids) or increased secretion of pituitary thyrotropic hormone

Question 25

nursing considerations for goiter

Answer 25

thyroid enlargement at birth my compromise newborn airway, may become noticeable during periods of rapid growth, large goiters may be obvious but smaller ones only evident w/ palpation -TH replacement is needed for tx of hypothyroidism

Question 26

lymphocytic thyroiditis

Answer 26

Hashimoto's or juvenile autoimmune thyroiditis, most common cause of thyroid dx in kids and teens, accounts for largest % of juvenile hypothyroidism

Question 27

pathophysiology of lymphocytic thyroiditis

Answer 27

genetic predisposition but specifics unclear; characterized by lymphocytic infiltration of the gland, inflammation, hyperplasia- which may be replaced with fibrous tissue - child usually euthyroid, with some sx of hyperthyroidism

Question 28

therapeutic management of lymphocytic thyroiditis

Answer 28

goiter may be transient, asymptomatic may resolve spontaneously within 1- 2 yrs, oral TH often decreases the goiter significantly, surgery contraindicated for this disorder

Question 29

sx of graves disease

Answer 29

manifestations develop gradually, often 6- 12 months - eyes- exopthalmous - excessive motion, irritability, hyperactivity, short attention span, tremors, insomnia and emotional lability - GI hyperactivity - cardiac- rapid pounding pulse even during sleep, widened pulse pressure, systolic murmurs and cardiomegaly - dyspnea occurs during slight exertion - skin wamr, flushed and moist- heat intolerance may be severe and accompanied by diaphoresis

Question 30

graves dx management

Answer 30

- diagnosis based on increased levels of t3 and t4 with suppressed TSH - therapy controversial - goal of therapy- retard rate of hormone secretion treatments- antithyroid drugs (PTU and methiamazole), subtotal thyroidectomy, ablation and radioiodine

Question 31

hyperthyroidism- management

Answer 31

antithyroid meds-methiamazole (MMI, TapazoleTM) or Propylthiouracil (PTU), propranolol, iodine, surgery

Question 32

s/e of antithyroids meds

Answer 32

mild- pruritis, rash, abdominal pain, neutropenia | serious- agranulocytosis, arthropathy, lupus- like syndrome, hepatits

Question 33

further management of hyperthyroidism

Answer 33

monitor- initially q 4- 6 wks until T4 stabilized on maintenance doses of MMI/ PTU, then 3- 4 months. - generally continue tx for 2 yrs then try off tx and monitor closely for relapse

Question 34

summary of thyroid disorders

Answer 34

- common in children and teens - TSH and thyroid antibodies is usually all that is required to establish dx - US should be limited to those w/ a palpable nodule, like you Ave! - normal range of TSH may be higher in peds leading to over- investigation/ dx and tx of thyroid disorders - mild elevations of TS should be verified on repeat testing- TSH

Question 35

cushing syndome

Answer 35

characteristic group of manifestations by excessive circulating free cortisol - may be caused by prolonged steroid therapy- reversible once steroids d/c'ed - abrupt withdrawal of steroids may precipitate acute adrenal insufficiency

Question 36

etiologies of cushings

Answer 36

pituitary- excess ACTH adrenal- hypersecretion of glucocorticoids ectopic- extrapituitary neoplasm iatrogenic- administration of excessive steroids food- dependent- inappropriate response to secretion of polypeptide

Question 37

cushingoid appearance

Answer 37

``` excessive hair growth moon face with red cheeks weight gain prendulous abdomen with red striae poor wound healing exxhymoses ```

Question 38

diagnostic eval for cushings

Answer 38

- confirm excess cortisol levels - xrays to evaluate for osteoporosis and skull films to look for enlargement of sella turcica - labs- fasting blood glucose, serum electrolytes, 24 hr urine

Question 39

therapeutic management for cushings

Answer 39

- surgery | - replacement of growth hormone, ADH, TH, gonadotropins and steroids

Question 40

precocious puberty

Answer 40

presence of secondary sexual development by age: 8 in a girl 9 in a boy occurs more in girs

Question 41

potential causes of precocious puberty

Answer 41

disorder of the gonads, adrenal glands or hypothalmic- pituitary gonadal axis - no causative factor in 80- 90% of girls and 50% of boys

Question 42

spectrum of precocious puberty- benign mild incomplete precocious puberty

Answer 42

- premature thelarche (breasts) - premature adrenarche (pubic hair) - minimal impact on adult height or timing of menstruation, minimal social/ emotional impact

Question 43

spectrum of precocious puberty- pathological precocious puberty

Answer 43

- central precocious puberty - peripheral precocious puberty - negative impact on adult height - early menstruation - social/ emotional distress Refer to endo for this

Question 44

therapeutic management of precocious puberty

Answer 44

- specific to cause if known - may be treated with Lupron- slows prepubertal growth to normal rates, discontinued at age for normal pubertal changes to resume (i bet those asian gymnasts take this shit forever) - psychological support for child/ family

Question 45

Delayed puberty

Answer 45

look at growth records, bone age, LH, FSH, sex hormone levels not needed if indicated check T4, TSH, GH, prolactin, cortisol

Question 46

Delayed puberty tx constitutional delay of growth and puberty- for boys

Answer 46

tx if psychologically distressed w/ | Depot testosterone 75- 100 mg IM X3

Question 47

Delayed puberty tx constitutional delay of growth and puberty- for girls

Answer 47

usually don't treat ( even low dose estrogen cause accelerated skeletal maturation)

Question 48

Delayed puberty tx for hyper/ hypogonadotropic hypogonadism- for boys

Answer 48

testosterone IM injection, transdermal patch/ gel or orally, gradually increasing to adult doses

Question 49

Delayed puberty tx for hyper/ hypogonadotropic hypogonadism- for girls

Answer 49

start with low dose estrogen, increasing over 1- 2 years, then begin cycling with estrogen and progesterone

Question 50

hypopituitarism- Growth hormone (GH) deficiency

Answer 50

inhibits somatic growth, primary site of dysfuntion appears to be in the hypothalamus

Question 51

diagnostic eval of GH deficiency

Answer 51

family hx, growth patterns and health history - definitive dx based on radioimmunoassay of plasma GH levels - hand xrays to evaluate growth potential vs. ossification - endocrine studies to detect deficiencies

Question 52

therapeutic management of GH definiciency

Answer 52

biosynthetic growth hormon injections, other hormone replacements as needed- thyroid extract, cortisone, testosterone, estrogen, progesterone

Question 53

prognosis of GH deficiency

Answer 53

replacement therapy successful in 80% of affected children, response varies based on age, length of tx, frequency of doses, doasge weight and GH amount - growth rate of 3.5 to 4 cm/ yr before tx and increase to 8- 9 cm/ yr after tx

Question 54

nursing considerations for GH replacement

Answer 54

fam needs support, child's body image, preparing child for daily injections, injections given at bedtime for best results, tx very expensive ($20,000- 30,000 per year)

Question 55

pituitary hyperfunction- acromegaly

Answer 55

aka Gigantism- excess GH before closure of the epiphyseal shafts results in overgrowth of long bones - reach heights of 8 feet + - vertical growth and increased muscle - weight is generally proportional to height

Question 56

nursing considerations for pituitary hyperfunction

Answer 56

early identification of kids with excessive growth rates, early tx for improved outcomes, emotional support, body image concerns

Question 57

nursing measures for metabolic disorders

Answer 57

genetic counseling, dietary teaching, compliance, mixing special preparations, mainly supportive.

Question 58

inborn errors of metabolism

Answer 58

phenylketonuria, galactosemia, defects in fatty acid oxidation, maple syrup urine disease

Question 59

phenylketonuria (PKU)

Answer 59

autosomal recessive | - deficiency of liver causing phenylalanine accumulation in the blood- causing a musty or mousy body and urine odor

Question 60

phenylketonuria (PKU) s/s

Answer 60

irritability, vomiting, hyperactivity, hypertonia, hyperreflexivity, seizures and may lead to mental retardation. Screening for it required in all 50 states, needs to be done within 48 hours of birth and repeated 1- 2 weeks after birth

Question 61

phenylketonuria (PKU) tx

Answer 61

directed towards the use of a special formula (Lofenalax, Minafen, and Albumaid XP) and a diet low in phenylalanine - foods high in phenylalanine to avoid: high protein foods like milk, dairy products, meat, fish, chicken, eggs, beans and nuts - breast feeding possible if PKU levels monitored

Question 62

Galactosemia

Answer 62

- carbohydrate metabolic dysfunction, autosomal recessive. - liver is deficient in GALT (one of the 3 enzumes needed to convert galactose to glucose), this leads to accumulation of galactose metabolites in the eyes, liver, kidney and brain - children become susceptible to gram negative sepsis- can die within a month w/o treatment, usually from sepsis

Question 63

Galactosemia s/s

Answer 63

poor sucking, failure to gain weight d/t vomiting followed by diarrhea, hypoglycemia and an enlarged liver. Later signs include mental retardation, sepsis, sz, cataracts and coma.

Question 64

diet for Galactosemia

Answer 64

all sources of galactose and lactose must be eliminated from diet- primarily means that all dairy products must be strictly avoided. Also need to restrict other foods like legumes, some fruits because of high galactose content. Kids with this need the same kinds and amounts of nutrients as other kids to grow and develop normally- just no galactose

Question 65

defects in fatty acid oxidation

Answer 65

most common of inborn errors

Question 66

maple syrup urine disease

Answer 66

MSUD- 3 amino acids affected- leucine, isoleucine and valine. They can't break down which causes abnormal structures such as hair, skin and muscle. - Leucine can build up in the brain and cause cerebral edema, progressive neuro impairment and death

Question 67

MSUD s/s

Answer 67

within 3- 7 days of life, newborn exhibits poor appetite, lethargy, vomiting, variable muscle tone, seizures, high- pitched crying and sweet odor of maple syrup in the body fluids

Question 68

MSUD tx

Answer 68

initially involves the removal of the amino acids and their metabolites from the tissues and body fluids- some require dialysis

Question 69

MSUD tx cont

Answer 69

in most cases, sx of MSUD can be prevented by a diet very low in BCAA's- but depends on type of MSUD and the gene involved. - This diet should begin ASAP following dx, nutritionist will recommend a special diet that includes certain vegetables, fruits, grains and a metabolic formula that provides protein w/o BCAA (branch chain amino acids) - some rare forms may be difficult to treat and may require thiamine - need regular blood tests to monitor BCAA levels