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Common is common,
but rare is present !.
Case Report by
Mohammad G. Khalifa (MSc.)
Assist.Lecturer of Internal medicine,
Diabetes and Endocrinology
Faculty of medicine , Zagazig University.
(March,6th , 2019)
Personal History :
M.A. 33 years old , male patient, from zagazig ,
Married and has 2 children the youngest is 2
years old , he is right handed , cigarette
smoker “ 10 per day for 15 ys ”with no other
special habits of medical importance.
Chief Complaint:
multiple erythematous skin lesions
and chest pain 4 ms ago.
The condition started 4 ms ago , when the
patient noticed multiple superfacial skin
lesions scattered on the upper trunk , arms
and the back.
such dermatological lesions are maculo-
erythamtous in nature, purple , not raised ,
not itchy , size ranged from 3-4cm width.
There is no past or present history of any
drug intake , no past history of any medical
diseases.
The patient sought medical advice from a
dermatologist , who reassured him and
explained these lesions as a type of skin
allergy and prescribed him some medications
in form of systemic and local anti allergic
drugs .
2 weeks later , there was no any improvement
of the conditon inspite adherence to the
prescribed medications .and he began to
complaint from easay fatigability and
a typical chest pain , all over the chest ,
shoulders and upper arms , increase with
movement and exercise and decrease with
rest with inadequate response to analgesics .
Such chest pain was not associated with cough
, expectoration , dyspnea or heamoptysis or
any cardoipulmonary manifestation , also not
related to meals .
The patient sought medical advice of
pulmonologist who examined the patient and
noticed that patient is hypertensive , bl. Pr.
Was 150 / 105 mmHg.
and asked for some investiagtion in form of
CBC , Chest x ray , ECG… All are normal.
The doctor reassured the patient and advised
him to follow healthy life style “ decrease
weight , low salt intake , stop smoking “ and
follow up his bl. Pr. for further 2 measures to
confirm or exclude Hypertension.
HPN is established and patient was
adviced to take an antihypertensive
drug “ amlodipine 10 mg once daily “
beside life style modification and
reconsult the docotor later on .
After a week , normalization of bl. Pr. Occurred
but there was no improvement of the condition
neither skin lesions nor his chest problem. Beside
this , patient felt that he is generally unwell with
easy fatigability persist.
the pulmonologist asked further investigation in
form of CT scan of the chest.
CT scan of the chest revealed that :
Chest is normal but the upper abdomen shew a
small mass in Rt. Suprarenal area measuring
about 27 mm x 30 mm for further evaluation.
After that , patient was referred to Internal
medicine specialist who re-evaluted the case
and asked further investigations :
CBC : Normal
Serum Cortisol level A.M : High “50 mg/dl “ ( N : up to 22 )
Serum Na+ 140 , K+ 3.5 , Ca++ 8.4
24 hs urinary cortisol level : high
201 nmol/ 24hs ( normal : 0 – 146/ 24 hs)
ACTH : Not done till now
CT abdomen: the same as mentioned before .
Diagnosis of cushing’s syndrome is present .
“Adrenal cushing’s “
patient was referred to General surgery
Department for surgical treatment .
The patient referred to our Endocrinology
outpatient clinic for pre operative preparation.
Re evalution of the the case in our out patient
clinic revealed the following :
Fully conscious, heamodynamically stable ,
oriented to time , place and persons of average
mood and memory .
Body built : obese ” mainly Truncal”
length : 170 Weight : 95 BMI: 33
Bl. Pr. 140 / 90 “ on ttt “
pulse: 85 /min regular.
Chest , heart , abdomen ex. : Is normal
Face : plethoric , rounded , acne
Skin: maculo-erythamtous skin lesions not
raised not itchy scatrerd all over the
back ,upper arm.(superfacial infection
?!!)
No stria rubera , no Sc hge , no hump ,,,,,…..
L.L. : Mild bilateral pitting l.l. edema .
Some ivestigation are required to
confirm diagnosis and for pre operative
preparation:
FBS: 105 mg / dl “ normal”.
PPBS: 188 mg/dl .
HBA1C: 6 %.
ACTH: suppressed
S.Cortisol A.M.: high 53 mg/dl ( N: up to 22)
CBC: mild leukocytosis , lymphopenia.
LFTs: normal.
KFTs: normal.
INR: normal.
Acase of Adrenal cushing’s
Syndrome
Discussion
Cushing’s syndrome reflects many clinical features
that result from chronic exposure to excess
glucocorticoids of any etiology.
The disorder can be ACTH-dependent (80-90 %) :
(e.g. pituitary corticotrope adenoma, ectopic secretion
of ACTH by nonpituitary tumor) or
ACTH-independent (10 - 20%) : (e.g. adrenocortical
adenoma, adrenocortical carcinoma, nodular adrenal
hyperplasia), as well as iatrogenic
(e.g. administration of exogenous glucocorticoids )
Cushing’s syndrome is generally considered a
rare disease.
It occurs with an incidence of 1-2 per 100,000
populations per year.
In the overwhelming majority of patients,
Cushing’s syndrome is caused by an ACTH-
producing corticotrope adenoma of the
pituitary, as initially described by Harvey
Cushing in 1912 .
Only 10 % of patients with Cushing’s
syndrome have a primary adrenal cause of
their disease (e.g. autonomous cortisol excess
independent of ACTH).
Cushing’s disease more frequently affects
women, with the exception of prepubertal
cases, where it is more common in boys.
Glucocorticoids affect almost all cells of the
body, and thus signs of cortisol excess impact
multiple physiologic systems, with
upregulation of gluconeogenesis, lipolysis,
and protein catabolism causing the most
prominent features.
In addition, excess glucocorticoid secretion
overcomes the ability of inactivation of
cortisol to cortisone in the kidney, thereby
exerting mineralocorticoid actions, manifest as
diastolic hypertension, hypokalemia, and
edema.
Excess glucocorticoids also interfere
with central regulatory systems,
leading to suppression of
gonadotropins with subsequent
hypogonadism and amenorrhea.
Careful clinical assessment is an important
aspect of evaluating suspected cases , as The
majority of clinical signs and symptoms
observed in Cushing’s syndrome are relatively
nonspecific and include features such as
obesity, diabetes, diastolic hypertension,
hirsutism, and depression that are commonly
found in patients who do not have Cushing’s.
A diagnosis of Cushing’s should be considered
when several clinical features are found in the
same patient.
The most important first step in the
management of patients with suspected
Cushing’s syndrome is to establish the
correct diagnosis.
Most mistakes in clinical management,
leading to unnecessary imaging or surgery,
are made because the diagnostic protocol is
not followed
Investigations :
1- Non specific : suggest High cortisol level
High blood sugar ,Hypokalemia , hypernatremia
CBC… lymphopenia , neutrophilia , polycythemia.
2- 24 hs urinary free cortisol level …. High
3- plasma cortisol level … high and loss of
circadian rhytm .
4 – Screening with Standard Low
Dexamethasone suppression test “ LDST”
5- Overnight high dose dexamethasone
suppression test …… differentiate between
adrenal , pituitary and ectopic ACTH
6 – ACTH level : high in pituitary adenoma and
ectopic ACTH and low in adrenal cushing .
NB : ACTH can be measured in venous blood obtained
by catheterization of inf. Petrosal sinus… high level
compared with that of peripheral blood indicate
pituitary cause…
7-MRI pituitary ….. Pit. adenoma .
8- Chest x ray anf CT scan ….. Bronchogenic
carcinoma .
Treatment:
Expectations (prognosis):
Patients with adrenal adenoma who undergo surgery
have an excellent prognosis.
Surgery success rates are very high with this type of
tumor.
For adrenal carcinoma, surgery is sometimes not
possible. When surgery is performed, it does not
always cure the cancer. Survival time ranges from 14
to 36 months after diagnosis of adrenal carcinoma.
 Thank you

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Adrenal cushings

  • 1. Common is common, but rare is present !. Case Report by Mohammad G. Khalifa (MSc.) Assist.Lecturer of Internal medicine, Diabetes and Endocrinology Faculty of medicine , Zagazig University. (March,6th , 2019)
  • 2. Personal History : M.A. 33 years old , male patient, from zagazig , Married and has 2 children the youngest is 2 years old , he is right handed , cigarette smoker “ 10 per day for 15 ys ”with no other special habits of medical importance.
  • 3. Chief Complaint: multiple erythematous skin lesions and chest pain 4 ms ago.
  • 4. The condition started 4 ms ago , when the patient noticed multiple superfacial skin lesions scattered on the upper trunk , arms and the back. such dermatological lesions are maculo- erythamtous in nature, purple , not raised , not itchy , size ranged from 3-4cm width. There is no past or present history of any drug intake , no past history of any medical diseases.
  • 5. The patient sought medical advice from a dermatologist , who reassured him and explained these lesions as a type of skin allergy and prescribed him some medications in form of systemic and local anti allergic drugs .
  • 6. 2 weeks later , there was no any improvement of the conditon inspite adherence to the prescribed medications .and he began to complaint from easay fatigability and a typical chest pain , all over the chest , shoulders and upper arms , increase with movement and exercise and decrease with rest with inadequate response to analgesics .
  • 7. Such chest pain was not associated with cough , expectoration , dyspnea or heamoptysis or any cardoipulmonary manifestation , also not related to meals . The patient sought medical advice of pulmonologist who examined the patient and noticed that patient is hypertensive , bl. Pr. Was 150 / 105 mmHg.
  • 8. and asked for some investiagtion in form of CBC , Chest x ray , ECG… All are normal. The doctor reassured the patient and advised him to follow healthy life style “ decrease weight , low salt intake , stop smoking “ and follow up his bl. Pr. for further 2 measures to confirm or exclude Hypertension.
  • 9. HPN is established and patient was adviced to take an antihypertensive drug “ amlodipine 10 mg once daily “ beside life style modification and reconsult the docotor later on .
  • 10. After a week , normalization of bl. Pr. Occurred but there was no improvement of the condition neither skin lesions nor his chest problem. Beside this , patient felt that he is generally unwell with easy fatigability persist. the pulmonologist asked further investigation in form of CT scan of the chest. CT scan of the chest revealed that : Chest is normal but the upper abdomen shew a small mass in Rt. Suprarenal area measuring about 27 mm x 30 mm for further evaluation.
  • 11. After that , patient was referred to Internal medicine specialist who re-evaluted the case and asked further investigations : CBC : Normal Serum Cortisol level A.M : High “50 mg/dl “ ( N : up to 22 ) Serum Na+ 140 , K+ 3.5 , Ca++ 8.4 24 hs urinary cortisol level : high 201 nmol/ 24hs ( normal : 0 – 146/ 24 hs) ACTH : Not done till now CT abdomen: the same as mentioned before . Diagnosis of cushing’s syndrome is present . “Adrenal cushing’s “
  • 12. patient was referred to General surgery Department for surgical treatment . The patient referred to our Endocrinology outpatient clinic for pre operative preparation. Re evalution of the the case in our out patient clinic revealed the following : Fully conscious, heamodynamically stable , oriented to time , place and persons of average mood and memory .
  • 13. Body built : obese ” mainly Truncal” length : 170 Weight : 95 BMI: 33 Bl. Pr. 140 / 90 “ on ttt “ pulse: 85 /min regular. Chest , heart , abdomen ex. : Is normal Face : plethoric , rounded , acne Skin: maculo-erythamtous skin lesions not raised not itchy scatrerd all over the back ,upper arm.(superfacial infection ?!!) No stria rubera , no Sc hge , no hump ,,,,,….. L.L. : Mild bilateral pitting l.l. edema .
  • 14. Some ivestigation are required to confirm diagnosis and for pre operative preparation: FBS: 105 mg / dl “ normal”. PPBS: 188 mg/dl . HBA1C: 6 %. ACTH: suppressed S.Cortisol A.M.: high 53 mg/dl ( N: up to 22) CBC: mild leukocytosis , lymphopenia. LFTs: normal. KFTs: normal. INR: normal.
  • 15. Acase of Adrenal cushing’s Syndrome
  • 17. Cushing’s syndrome reflects many clinical features that result from chronic exposure to excess glucocorticoids of any etiology. The disorder can be ACTH-dependent (80-90 %) : (e.g. pituitary corticotrope adenoma, ectopic secretion of ACTH by nonpituitary tumor) or ACTH-independent (10 - 20%) : (e.g. adrenocortical adenoma, adrenocortical carcinoma, nodular adrenal hyperplasia), as well as iatrogenic (e.g. administration of exogenous glucocorticoids )
  • 18. Cushing’s syndrome is generally considered a rare disease. It occurs with an incidence of 1-2 per 100,000 populations per year. In the overwhelming majority of patients, Cushing’s syndrome is caused by an ACTH- producing corticotrope adenoma of the pituitary, as initially described by Harvey Cushing in 1912 .
  • 19. Only 10 % of patients with Cushing’s syndrome have a primary adrenal cause of their disease (e.g. autonomous cortisol excess independent of ACTH). Cushing’s disease more frequently affects women, with the exception of prepubertal cases, where it is more common in boys.
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  • 21. Glucocorticoids affect almost all cells of the body, and thus signs of cortisol excess impact multiple physiologic systems, with upregulation of gluconeogenesis, lipolysis, and protein catabolism causing the most prominent features. In addition, excess glucocorticoid secretion overcomes the ability of inactivation of cortisol to cortisone in the kidney, thereby exerting mineralocorticoid actions, manifest as diastolic hypertension, hypokalemia, and edema.
  • 22. Excess glucocorticoids also interfere with central regulatory systems, leading to suppression of gonadotropins with subsequent hypogonadism and amenorrhea.
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  • 24. Careful clinical assessment is an important aspect of evaluating suspected cases , as The majority of clinical signs and symptoms observed in Cushing’s syndrome are relatively nonspecific and include features such as obesity, diabetes, diastolic hypertension, hirsutism, and depression that are commonly found in patients who do not have Cushing’s. A diagnosis of Cushing’s should be considered when several clinical features are found in the same patient.
  • 25. The most important first step in the management of patients with suspected Cushing’s syndrome is to establish the correct diagnosis. Most mistakes in clinical management, leading to unnecessary imaging or surgery, are made because the diagnostic protocol is not followed
  • 26. Investigations : 1- Non specific : suggest High cortisol level High blood sugar ,Hypokalemia , hypernatremia CBC… lymphopenia , neutrophilia , polycythemia. 2- 24 hs urinary free cortisol level …. High 3- plasma cortisol level … high and loss of circadian rhytm . 4 – Screening with Standard Low Dexamethasone suppression test “ LDST” 5- Overnight high dose dexamethasone suppression test …… differentiate between adrenal , pituitary and ectopic ACTH
  • 27. 6 – ACTH level : high in pituitary adenoma and ectopic ACTH and low in adrenal cushing . NB : ACTH can be measured in venous blood obtained by catheterization of inf. Petrosal sinus… high level compared with that of peripheral blood indicate pituitary cause… 7-MRI pituitary ….. Pit. adenoma . 8- Chest x ray anf CT scan ….. Bronchogenic carcinoma .
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  • 36. Expectations (prognosis): Patients with adrenal adenoma who undergo surgery have an excellent prognosis. Surgery success rates are very high with this type of tumor. For adrenal carcinoma, surgery is sometimes not possible. When surgery is performed, it does not always cure the cancer. Survival time ranges from 14 to 36 months after diagnosis of adrenal carcinoma.
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