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Disorders MBBS Students Club肾上腺疾病 MBBS学生俱乐部课件.ppt

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1、Question,Scenario 1,Make a scenario of an MCQ. Outline the signs and symptoms that you would expect in a patient with hyposecretion of adrenocortical hormones. Give 4 options for the MCQ question.,Scenario 2,Make a scenario of an MCQ. Outline the signs and symptoms that you would expect in a patient

2、 with hypersecretion of adrenocortical hormones. Give 4 options for the MCQ question.,Scenario 1 (Mian Seher Munir),An obese 50-year old man presents with high blood glucose levels. He shows buffalo torso and moon face and is complaining of purple striae after injury. The laboratory blood tests show

3、 high blood RBCs and relative polycythemia, hypokalemia and hyponatremia. He is most probably suffering from: Tumour of adrenal cortex Tumour of adrenal medulla Pituitary adenoma Increased oral steroid intake,Scenario 2 (Adil Maqbool),A 40-year old woman presents to the physician with symptoms of: S

4、welling in the face Hypertension Excessive facial hair growth Abdominal fat deposition Polydisia The lab results show: levels of ADH Na in urine High plasma levels of ACTH & cortisol levels of blood glucose upto 200 mg/dl The most appropriate diagnosis is: Cushing syndrome Diabetes mellitus Tumor of

5、 Pituitary gland (corticotropes) Conns syndrome,Scenario 3 & 4,Scenario 3 ( M. Halim),A patient was presented to the hospital having high blood pressure. His lab tests show: Hypernatremia Hypokalemia High blood pH and urinary pH Which of the following disorder is he most probably suffering from: Met

6、abolic alkalosis Diabetes insipidus Hypersecretion of Aldosterone Hypersecretion of Cortisol Tumour of Adrenal Medulla,Scenario 4 (Mahnoor),A woman comes into the clinic and complains to the doctor about her lethargicness. The doctor observes blotches on her face along with hypertrichosis. She compl

7、ains about being sick more than usual and irregular menstrual cycles. Her blood report showed glucose levels and hypokalemia was also observed. What do you think this patient is suffering from: Diabetes mellitus Hyposecretion of ACTH Hypersecretion of ACTH Hypothyroidism,Scenario 5 & 6,Scenario 5 (W

8、adia Imtiaz),A female of about 45 years presents to the hospital with polyuria, dehrdration, sluggishness, muscle wasting and skin pigmentation in blotches. Clinical examination reveals: HypotensionK levels of 7 mmol/L (Hyperkalemia) Mild acidosis What is the appropriate diagnosis: Addisons disease

9、Primary atrophy of adrenal cortices Primary aldosteronism Cushings syndrome,Scenario 6 (Khizra),A person reported to the physician with high blood pressure, muscle weakness, nervousness, lightheadedness, numbness and hair on the face. His lab reports show decrease in K levels, decreased eosinophils

10、and basophils, increased RBCs and platelet count.What can be the most likely diagnosis? Leukopenia Hyposecretion of adrenocortical hormones Hypersecretion of adrenocortical hormones Metabolic acidosis Polycythemia,Scenario 7 (Qasam),A patient reports to the doctor of lethargic condition with polypha

11、gia. Upon his vital examination his/her pulse and blood pressure were low. Sexually, he/she has slow/ decreased libido and hyposecretion of sex hormones. Upon his urinary and blood glucose test, following are the values: Urine: hyperkalemiahyervolemiahypernatremia Blood sugar (fasting level): lower

12、than 90 mg/ dl Blood pH: less than 7.34 (acidosis) which hormone deficiency is possible: Glucocorticoid Mineralocorticoid Catecholamine Adrenocortical hormones None of the above,Scenario 8 (Nida),A patient reports to the doctor with edematous appearance of his face apparently moon face with excessiv

13、e deposition of fats in thoracic and upper abdominal region. His blood pressure is also high. On examination doctor comes to know that the patient has excessive growth of facial hair and acne problems. Lab reports show that his plasma ACTH & cortisol levels are high. He also has: Low WBCs Hyperglyce

14、mia and hyperkalemia What would you suspect that patient will be suffering from: Hypocortisolism Hypoadrenalism Hyperadrenalism Hyperthyroidism,DISORDERS OF THE ADRENAL GLAND,Dr. Ayisha Qureshi Assistant Professor, MBBS, MPhil,HYPo & hypersecretion of aldosterone,Zona glomerulosa:,Primary aldosteron

15、ism (conns syndrome),DEFINITION: Conns Syndrome is the disorder caused by the hypersecretion of Aldosterone due to primary cause or defect in the zona glomerulosa itself.,DIAGNOSIS,Primary adrenocortical failure,Causes of Primary Adrenocortical failure:,It may occur due to: Addisons disease Congenit

16、al Adrenal Hypoplasia Secondary Adrenocortical failure Pituitary destruction,Hypoadrenalism- ADDISONS DISEASE,DEFINITION: It is a disorder caused by the inability of the adrenal cortex to produce the cortical hormones. This is frequently caused by the primary atrophy of the adrenal cortex. The patie

17、nt presents with mineralocorticoid and glucocorticoid deficiency.,CAUSE:,The causes can be: Autoimmune disorder Tuberculous destruction of the glands Cancer Iron overload,Signs & Symptoms,Mineralocorticoid deficiency,Greatly decreased renal sodium reabsorption. Increased loss of Na, Cl & water in th

18、e urine. Decreased ECF leading to hypovolemia leading to dehydration. Hyponatremia leading to cramps. Hyperkalemia. Mild Acidosis. Hypotension. Decreased Cardiac output. Shock. Death within 4 days to 2 weeks after complete cessation of the aldosterone production.,Glucocorticoid deficiency,Patient ca

19、nnot maintain normal blood glucose levels as no gluconeogenesis b/w meals. Patient cannot Fast. Nausea, vomiting, fever, diorrheoa and anemia. All metabolisms effected leading to fatigue and sluggishness. Patient highly susceptible to the deteriorating effects of stress. Even mild infections can lea

20、d to death. Pigmentation of the skin in the form of blotches b/c of increased secretion of ACTH as feedback. These brownish-black areas appear in axilla, genital areas, lips, buccal mucosa.,TREATMENT,A person with complete destruction of the adrenal may die within a few days b/c of weakness & circul

21、atory shock. However, if small quantities of mineralo & glucocorticoids are administered daily, they can live for years.,ADDISONIAN CRISIS,In a person with Addisons disease, the output of glucocorticoids does not increase during stress. Yet whenever different types of trauma, disease, or other stres

22、ses, such as surgical operations, supervene, a person is likely to have an acute need for excessive amounts of glucocorticoids and often must be given 10 or more times the normal quantities of glucocorticoids to prevent death. This critical need for extra glucocorticoids and the associated severe di

23、sease state in times of stress is called an addisonian crisis.,Hyperadrenalism- Cushings Syndrome,DEFINITION: Hypersecretion by the adrenal cortex causes a complex cascade of hormone effects called Cushings syndrome. Many of the abnormalities are due to abnormal amounts of cortisol but increased amo

24、unts of aldosterone and androgens are also responsible.,CAUSES:,Exogenous ACTH-Dependant- Pituitary adenoma- Ectopic ACTH syndrome ACTH-Independant- Adrenal adenoma- Adrenal carcinoma,When Cushings syndrome is secondary to excess secretion of ACTH by the anterior pituitary, it is called CUSHINGS DIS

25、EASE.,Signs & Symptoms,Truncal Obesity Supraclavicular & dorsal fat pad Buffalo hump (diagnostic) Moon Facies due to deposition in the cheeks and temporal area of the face, with a fish-like mouth. Purple striae due to decreased proteins in the collagen fibres. This causes the collagen fibres to tear

26、 easily and leads to the formation of purple striae. Thin skin. Capillary fragility & easy bruising. Hirsutism (excess facial hair growth) and acne vulgarisdue to increased androgens.,Hb percentage is raised. Depression and psychosis. Diabetes Mellitus also called Adrenal Diabetes. Severe muscular w

27、eakness due to increased protein catabolism. Thin extremities due to muscle wasting and protein catabolism. This leads to myopathy and inability of the patient to stand up from a sitting position. Infections due to suppressed immune system with impaired wound healing. Osteoporosis leading to frequen

28、t fractures. Hypertension and hypokalemia - due to increased aldosterone. Amenorrhoea.,Purple striae due to collagen breakdown in the subcutaneous tissue:,Diagnosis,Very challenging. Diagnose excess Cortisol. Dexamethasone suppression test: this test helps differentiate between ACTH-dependant and AC

29、TH-independent Cushings syndrome. Confirmation of cause.,TREATMENT,Removal of the cause: Surgery of the adrenal tumour OR pituitary tumour. Drugs that block steroidogenesis.e.g. Ketaconazole. Drugs that inhibit ACTH secretion.e.g. Serotonin antagonists. Partial or total adrenalectomy. The treatment

30、is followed by administration of adrenal steroids to make up for any insufficiency that may arise.,Adrenogenital syndrome,DEFINITIONS: It is a disorder caused by an occasional adrenocortical tumor secreting excessive quantities of androgens that cause intense masculinizing effects throughout the bod

31、y.,SIGNS & SYMPTOMS,IN FEMALES,Virile characteristics in women & girls. Hirsutism: hair on the face, chin and upper lips. Deeper voice. Occasionally baldness. Masculine distribution of hair on the body. Deposition of proteins in a masculine manner.,IN MALES,Precocious development in boys. In prepube

32、rtal male, a virilizing adrenal tumour causes same characteristics as in the female plus rapid development of the male sex organs. In adult male, the virilising characteristics are often masked by the virilising effects of Testosterone. Diagnosis is made by the presence of excess 17-ketosteroids in the urine.,

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