Cushing syndrome in child .. for bachelor of nursing student

1. CUSHING SYNDROME
Prepared by: Tulasha lakandri
PBNS student

2. CONTENT
 Anatomy and Physiology
 Introduction
 Incidence
 Risk Factor
 Causes
 Pathophysiology
 Sign and Symptoms
 Diagnosis
 Medical management
 Surgical management
 Nursing management
 Discharge teaching
 Prognosis
 Complication
 Prevention

3. Anatomy and Physiology of Adrenal
gland
 Adrenal Gland:
Adrenal gland are the pair of important endocrine
glands situated on the upper pole of each Kidney on the posterior
abdominal wall.
Its has two distant parts:
1)Adrenal Cortex (Outer portion)
2)Adrenal Medulla (Central portion)
* Adrenal cortex has 3 layers:
a)Outer zona glomerulosa
b)Middle zona fasciculata
c)Inner zona reticulosa
*Hormone of Adrenal gland:
a)Mineralocorticoids ( aldosterone,11-aldeoxycorticosterone )
b)Glucocorticoids (cortisol, corticosterone , cortisone )
c)Sex Corticoids
(dihydroepipandrosterone,androsteredione,testerone)

5.  Function :
a) Effects on carbohydrate metabolism.
b) Effects on lipid metabolism.
c) Effects on protein and nucleic acid metabolism.
d) Effects on water and electrolytes metabolism.
e) Increase production of gastric acid and some enzymes.
f) Inhibits immune system
g) Stress resonce

6.  Introduction:
- Firstly described by Harvey Cushing.
- Cushing syndrome is a group of clinical problems
caused by an excessive secretion of one or all of the
adrenocorticoid hormone.
 Incidence:
-Rare in children.
- All over incidence is approximately 2 to 5 new cases per
million people per year.
- 10% of total new cases occur in children.
- below 7years children cushing syndrome disease is less
frequent.
 Risk factors:
- Tumors ( puitituary tumor , adrenal tumor)
CUSHING SYNDROME

7.  Causes:
(a) Exogenous cause :
-Taking long terms medicine
containing corticosteriod.
(b) Endogenous causes:
-Pituitary: (pituitary
adenoma)
-Adrenal:(carcinoma of
adrenal cortex)
-Ectopic:(Extrapituitary
neoplasma )
Fig: small cell lung
cancer

8. SCLC- they secrete
more
adenocorticotropic
hormone
Pathophysiology
Causes (Pituitary tumor, Adrenal cortex carcenoma ,
etc)
Ineffectiveness of normal
feedback mechanism
that control
adrenocortical function
Excessive secretion of cortisol from adrenal cortex
Excessive cortisol level in blood
Clinical features (blood pressure increased,
osteoporosis,muscle weakness, diabetes,edema etc)

10.  Sign and symptoms
-Buffalo hump,edema,weight gain
-Rounded moon like face with reddened oily skin
-Muscle wasting, thin extremities and pendulous
abdomen
-fragile thin skin, excessive bruishing and patachiae
-Reddish purple abdominal striae
-Increase susceptibility to infection,decrease
inflammatory response and poor wound healing
- Hyperglycemia , Hypertension
-Compression fracture of vetebrae , kyphosis and
osteoporosis
- Retarded linear growth , loss of libido in male
- In female: virilism,hirsutism, breast atropy
Fig: thin fragile skin,
striae
Fig: abdominal
distension

12.  Diagnosis:
a) History Taking: ( history of cancer, tumor ,using of medicine
like corticosteriod , baby daily activity felling of irritate ,sleep
pattern etc)
b) Physical Examination:(weight ,height,skin ,trunk neck , moon
face etc)
c) Blood Test:
-blood sugar increased (glucose random:125 mg/dl )
-Serum calcium and potassium ( Ca: 8.8mg/dl , k: 3.4-
4.7mmol/L )
-Overnight dexamethasone supression test :( administration of
0.3mg/m2 of dexamethasone at 12MN and plasma cortisol level
is checked after 8 hours . Cotisol should be less than 5mg/dl)
Fig: physical
Fig: blood sugar test
Fig: paediatric urine
collection bag

13. d) Urine test:
-24 hour urinary free cortisol level ( normal :10-100mcg/24hr)
e) Midnight plasma cortisol plasma measurement : (normal level
less then 50nnmol/l)
f) Serum ACTH level: (at 9am- 11 to 82pg/ml, at midnight- less
than 10pg/ml )
g) X-ray
h) Ultrasonography
I) CT- Scan
Fig: ultrasound
Fig: CT- Scan

14.  Management:
- Management is done according to underlying cause
Medical
surgery
Radiation

15.  Medical management:
- If the cause is exogeneous firstly discontinue or reduce the dose
of steriods.
or
Alternate day therapy
- Ketoconazole :
- decrease the steroidgenesis
- dose children 2years and older is 3.3mg to 6.6 per kg of
body wt.
- dose child below 2 years is
- Mifeprex ( cushing syndrome with diabetes )
- Antibiotic for infection
- Frusemide for edema ( dose1-2 mg/kg)

16.  Radiation therapy:
- Upto 80% of children improve by radiation therapy.
- It may take several months or years.
- It is special used to treat pituitary or adrenal tumors.
 Surgical Management:
According to causes surgery is indicated:
(a) Transpenoid Hypophysectomy : Pituitary adenoma
(b) Transfrontal craniotomy :Pituitary adenoma
(c) Adrenalectomy : Adrenal cortex tumor or carcinoma
(a) Transpenoidal hypophysectomy:
-Surgical removing of pituitary tumor
through sphenoid sinus there is air sinus
cavity at the back of nose.
-with the help of endoscope and pituitary
rongeurs tumor is removed.
Note: don’t bend, blow, swim, put you head
down.
:observe for CSF leakage.

17. (b)Transfrontal craniotomy:
- If the tumor enlarged
beyond the sella turnica and
patient complain of vision lost
then surgery is formed.
(c) Adrenalectomy:
- Laproscopiocally as a
minimally invasive procedure
through the abdomen, as an open
procedure can also formed for
removal of adrenal tumor or
carcinoma.
*Note: observe for haemorrage
- If unilateral adrenalectomy
done then temporary
Fig: laproscopical
adrenalectomy
Fig: transfrontal craniotomy

18.  Nursing management:
*Assessment:
-Assess the vital signs
-Assess the intake and output strictly
- weight the patient daily with same weighing machine to
monitor fliud retention
- Assess skin fragility, to detect redness, edema , tear of skin ,
infection
-Assess the strength of muscle
- Assess and ask about fatigue , nausea or seizure activity
- Assess the abdominal grith daily with measuring tape
- Assess the sign of infection on incision sign ( redness,
discharge, swelling)

19.  Pre-operative nursing diagnosis:
(1) Impaired skin integrity related to altered healing, thin and
fragile skin
(2) Dressing ,toileting self care deficit related to muscle wasting ,
weakness
(3) Disturbed body image related to altered physical appearance
and emotional instability
(4) Risk for injury related to surgical procedure
(5) Anxiety realted to outcome , procedure of surgery
 Post-operative nursing diagnosis:
(1) Acute pain realted to surgicial incision
(2) Risk for infection related to incision, leakage of cerebro spinal
fluid
(3) Risk for injury related to edema, Immobilization
(4) Risk for altered vision related to transpenoid hypophysectomy
procedure

20.  Nursing Intervention:
General:
(1) Monitor vital signs
(2) Weight patient every morning using same weighing machine
(3) Monitor glucose and electrolytes
(a) Improve skin intregity:
- Avoid risk of injury .( sharp instrument, toys )
- Put lotion on body
- Avoid using adhesive tape that acuse skin irritation
- Use light clothing
-Use humidifier in dry , winter month
-Foot care, bath with warm water
- Turning and reposition the patient timely
- cut the nails timely and caretaker / family member also
- High protein diet

21. (b) Prevent Infection:
-Hand washing during handling or taking care of patient
- Use aseptic teachnique while dressing cleaning the wound
- Use clean bedsheet or clothing
- Maintaing presonal hygiene by bathing, washing hand
frequently
- Toys of child or necessary equipment should also be clean
(c) Prevent Injury:
- Use siderails
- Small pins , scissors or sharp instrument should be in proper
place
- Care taker should be on bedside with child
- Floor should not be slippery and slipper too
- Well ventilated room

22. (d) Diet :
- Low sodium, high potassium (banana , orange )
- High calcium (milk produt )
-low calorie, low carbohydrate( to prevent hyperglycemia ,
obesity )
(e) Activity:
- Encourage for ambulation (child may have excessive play so
excessive play also prohibited to decrease risk of injury ,
fatigue )
- Re- strict activity like heavy lifting
- Re- strictdown after surgery ( transphenoid
hypophysectomy)
- Mobilize and reposition patient timely
(f) Play therapy:
- Make favourable environment for child
- educational play , bulding blocks should be given
- Sharp toys shouldnot be given
Fig: play therapy

23.  Discharge teaching:
(a) Take Medicine on time .
(b) Monitor Blood sugar level timely
(c)Weight yourself body weight daily every morning at same time
before meal
(d) eat plenty of fruits vegetables, nuts and source of fibers.
- low sodim , high pootasium , high protein
(e) avoid stress , Inform to school about the disease and
condition of patient
 When to seek medical help:
(a) You gain more weight
(b) Pain is worsen
(c) trouble staying awake or confused
(d) Severe headache or feel dizzy
(e) blurred vision , chest pain
(f) trouble breathing or shallow breathing

24.  Prognosis:
- If untreated , cushing’s syndrome can be life threatening.
If the cause can be treated , whether it means weaning the
patient off corticosteriods or surgically removing a tumour, the
patient will fully recover .
 Complication:
(a) Infection
(b) Hypokalaemia
(c) Hypertension
(d) Peptic ulcer
(e) Osteoporosis
(f) Retarded linear growth
(g) Vilirisation
Fig: retarded linear
growth

25.  Prevention:
-If the cause of cushing sydnrome is long- term use of
corticosteriod medications, your doctor may able to keep your
cushing syndrome sign and symptoms under control by
reducing the dosage of the drug over a period of time , while
still adequately managing your asthma, arthritis or other
condition.
ANY QUESTION ?

27. Reference:
1. Datta P, Padiatric Nursing(2nd edition) 2014, Jaypee
Brothers medical publishers pvt.Ltd
2. Shrestha T, Essential Child Health Care(2nd
edition)2018,Mahadhavi Publication , page no:552
to 554
3. https://www.cushingsyndrome.padietric.com
4. https://www.google.com/search?q=cushing+syndro
me+myoclinic-enciov=utg-8
5. youtube