Fourth grand round case presentation to the staff.pptx

Grand round presentation

1. Personal history S.M.I., 46 years old female patient, mother of 6 children, the youngest is 7 years old, born in El-Fayoum, and living in El-Marg, worked as a worker, resigned 4 months ago due to her current illness, no special habits of medical importance.

2. Complaint Easy fatigue & asthenia

3. Present History Over the past year, the patient noticed that her face gradually became more full & rounded, with increase in the body hair especially at the face. Also the patient noticed gradual gaining of weight, central more than peripheral; (abdomen, supraclavicular, & back of the neck).

4. Also, the patient complained of gradually increasing easy fatigability & generalized body aches (for which she took NSAIDs) , up to quitting her job 4 months ago. There was no muscle weakness, difficulty in climbing stairs, or rising from sitting position.

5. 9 months ago, the patient had irregular menstrual cycles 4 months ago she developed amenorrhea There is no history of headache, blurring of vision, or projectile vomiting. There is no history of nipple discharge. There is no history of skin pigmentation. There is no history of stria, easy bruising, delayed wound healing or recurrent infections.

6. There is no history of palpitations, fever, or diaphoretic episodes. There is no history of dyspnea, orthopnea, PND, cough or chest pain. There is no history of other system affection.

7. The patient sought medical advice and many investigations were done for her, she was discovered to be hypertensive, & there was cortisol secreting left suprarenal mass. She was referred to NCI 4 months ago, she was candidate for surgery, but surgery was postponed due to elevated liver enzymes.

8. She was prescribed Amlodipine 5mg/valsartan 160mg for hypertension & she was referred to NHI for following liver condition. 1 week ago, liver enzymes were nearly normal, & she was referred to our endocrinology clinic for perioperative recommendations.

9. Past history No history of previous operations,TB, RHD, or drug allergy Family history Irrelevant .

10. Examination

11. General examination The patient was fully conscious, with cushingoid appearance Weight: 81 kg Height: 156 cm BMI: 33 kg/m2 Waist to hip ratio: 1.2

12. Vital signs Pulse: 80 beats/minute, regular, average pulse volume, equal on both sides, with intact peripheral pulsations. Blood pressure: 140/90 mmHg with no postural drop . Respiratory rate: 18 /minute. Temperature: 37.1 .

13. Head & Neck Plethoric, moon face, buffalo hump Increased lanugo facial hair, No acne or hair line recession No acanthosis nigricans or mucosal pigmentation No thyroid enlargement No malar rash, oral ulcers Neck veins are not congested . Carotid pulsations are equally felt Trachea is central

14. Extremities Thin extremities with thin skin fold No stria or excessive pigmentation No rash, petechiae or ecchymosis. No clubbing or flat nails. No palmer erythema. No flapping tremors. No Lower limb edema

15. Cardiac examination Inspection and palpation: No scars or dilated veins No precordial bulge The apex is localized in the left fifth space MCL, with no thrill . No palpable pulsations. Auscultation: Normal S1 & S2 No additional sounds

16. Chest examination Inspection: Normal shaped chest, symmetrical, no dilated veins or scars. No striae or excessive hair Palpation: Normal chest expansion, central trachea, no tenderness Normal TVF with no palpable rub or rhonchi. Percussion: Normal percussion note . The upper border of the liver is in the right 5 th space MCL, Auscultation: Normal vesicular breathing. No additional sounds.

17. Abdominal examination Inspection Abdomen moves freely with respiration Generalized abdominal distention, more on left side No striae, scars or dilated veins . Normal hair distribution.

18. Abdominal examination Superficial Palpation No tenderness or rigidity Deep Palpation Abdominal mass in left hypochondrial & left lumbar region, 15x13 cm oval in shape, with firm consistency & smooth surface. Percussion No Ascites detected clinically. Auscultation Normal intestinal sounds

19. Back : No tenderness or deformity Lymph node examination: N ormal Neurological examination: Speech: N ormal Cranial nerves: N ormal with normal confrontation test Motor examination: no wasting, normal tone, power & reflexes Normal superficial & deep sensations Normal gait

20. Investigations

21. HB 12 g/dl MCV 87 fL MCH 28 Pg Plt 238 10ˆ3/UL TLC 9.5 10ˆ3/UL CBC

22. 26/6 11/8 8/9 18/9 Creat 0.8 mg/dl UA 5.2 mg/dl Na 140 143 mmol/dl K 3.7 4 mmol/dl Ca corrected 9.5 mg/dl T.Bil 0.17 0.3 mg/dl Alb 3.5 g/dl ALT 121 340 250 100 U/L AST 88 202 171 65 U/L ALP 114 U/L Chemistry :

23. Chemistry : FBG 90 Mg/dl PPG 110 Mg/dl TGs 170 Mg/dl CHOL 195 Mg/dl LDL 136 Mg/dl HDL 45 Mg/dl TIBC 292 Mg/dl

24. PT 13.9 PC 90% INR 1.07 Coagulation profile Negative HBs Ag Negative HCV Ab

25. Cortisol 9 am 26.9 ug/dl 4.3-22.4 ACTH (am) 7.34 pg /mL Less than 65 Cortisol 9 pm 35.88 ug/dl 3.1-16.7 24 hr urinary Cortisol 342 ug/24hrs 21 - 292 Dexamethasone Suppression Test  Serum Cortisol 9 am 29.76 ug/dl 4.3 - 22.4 Serum Cortisol 9 pm 32.47 ug/dl 3.1 - 16.7 Serum Cortisol (9 am, after dose) 31.07 ug/dl

26. Metanephrine, Normetanephrine & VMA in Urine Normetanephrine 131.6 ug/24hrs Less than 527 Metanephrine 42.48 ug/24hrs Less than 302 VMA 4.02 mg/24hr Up to 13.6

27. CT chest, abdomen & chest with contrast A left suprarenal neoplastic lesion with macrolbulated outline contour measuring 12.5 x 12.3 x 16 cm is seen indenting abutting the left kidney upper pole . Few regional pre-/para-aortic LNs are noted, largest measuring 23x18 mm, no intranodal calcification nor cystic breakdown changes Patent and enhancing left renal vein and IVC

28. Left adrenalectomy & nephrectomy were done 1weak ago.

29. Perioperative preparation of Cushing syndrome who undergo resection of pituitary, adrenal, or ectopic tumors   On the day of surgery : premedication with 50 to 100 mg  hydrocortisone IV, and then continued either continuously (10 mg/h) or in boluses every 6 hours for 24–48 hours . Over the next two days , if the patient does wel l Dose is tapered rapidly to 50 and then 25 mg IV every 8 hours . OR If the patient can take oral therapy; Double replacement dose hydrocortisone (20, 10, and 10 mg orally) instituted until well.

30. Perioperative preparation of Cushing syndrome who undergo resection of pituitary, adrenal, or ectopic tumors   The rate of steroid taper may be slowed if severe preoperative hypercortisolism was present when the patient is recovered ( 3 rd postoperative day) Oral replacement doses of  hydrocortisone  (& mineralocorticoid replacement if bilateral adrenalectomy has been performed; 0.1 mg fludrocortisone daily). N.B. Mineralocorticoid replacement is not required with high-dose glucocorticoid - Hydrocortisone 40 mg and prednisone 50 mg provide a mineralocorticoid effect that is approximately equivalent to 0.1 mg of fludrocortisone. - Dexamethasone lacks mineralocorticoid effect.

31. Corticosteroid replacement therapy Hydrocortisone: 15–30 mg daily orally in 2-3 divided doses, largest dose in morning upon awakening Preferred because more potent synthetic glucocorticoids with a longer half-life may prolong HPA axis suppression. Plenadren MR (5- or 20-mg modified-release tablets) is a once-daily dual-release preparation of hydrocortisone administered in the morning Prednisone: 5 mg (range: 2.5 to 7.5 mg) orally; If pigmentation and markedly elevated morning ACTH levels are a problem , owing to its longer t. 1/2, it may lead to better suppression of ACTH. Occasionally, Dexamethasone 0.75 mg (range: 0.25 to 0.75 mg) orally daily in divided doses is required for this purpose.

32. Corticosteroid replacement therapy Adjustments in dosage According to Electrolytes, BP & manifestations of Cushing syndrome The corticosteroid dose should be kept at the lowest level at which the patient feels clinically well. Serum ACTH levels vary substantially and should not be used to determine dosing.

33. Corticosteroid replacement therapy/ Adjustments in dosage Increased corticosteroid dosing is required in Mild illness, minor surgery, stressful diagnostic procedures : double or triple the dose Drug interactions Rifampicin : Double usual dose of hydrocortisone (Increases the clearance of cortisol) Mitotane: Double usual dose of hydrocortisone (Increases cortisol binding globulin) Third trimester , corticosteroid requirements are higher, so usual corticosteroid doses are increased by 50%. For severe stress, major illness, surgery, or delivery : maximum stress dose of hydrocortisone is given No extra supplementation is needed for most uncomplicated, outpatient dental procedures under local anesthesia.

34. Corticosteroid replacement therapy/ Adjustments in dosage Decreased corticosteroid dosing is required with Medications that inhibit corticosteroid metabolism by blocking the isoenzyme CYP34A, particularly the antifungals ketoconazole or itraconazole , anti-HIV protease inhibitors 

35. Mineralocorticoid replacement therapy Fludrocortisone 0.1 mg (range: 0.05 to 0.2 mg) orally. Liberal salt intake. Monitor lying and standing BP and pulse, edema, serum K, and plasma renin activity .

36. Patient education Each patient should have an injectable glucocorticoid ( eg , 4 mg vials of dexamethasone or 100 mg vials of hydrocortisone ) and vials of sterile 0.9% normal saline and syringes. Instruct patient/caregivers on how to inject entire dose in event of severe stress or trauma and get medical help immediately. Emergency precautions Obtain medical alert bracelet /necklace, and prefilled syringes containing dexamethasone 4 mg in 1 mL saline.

37. Glucocorticoid withdrawal syndrome Patients should be informed that they may feel unwell for 6–9 months , sometimes > 1 year, despite the use of physiological glucocorticoid replacement , The syndrome may persist even after the HPA axis has recovered . Symptoms include anorexia, nausea, weight loss, fatigue, myalgias, depressive disorders, anxiety, or panic symptoms Patients may improve with a temporary increase in the glucocorticoid dose , & gradually reduce the dose as soon as possible. Administering SSRIs may help.

38. Postoperative initial remission generally defined as Morning serum cortisol values are generally < 1.8 μg /dL within 7 days of tumor resection.  After bilateral adrenalectomy, patients have undetectable serum cortisol values, and morning plasma ACTH levels are often between 200 and 500 pg / mL. Patients who are eucortisolemic after resection , may have residual tumors In these patients, measure late-night serum or salivary cortisol. If there is a normal diurnal rhythm , then it is likely that the patient is in remission. Conversely, the lack of a diurnal rhythm suggests persistent disease.

39. Approach for long-term follow-up Any etiology of hypercortisolism can cause functional central hypothyroidism and central hypogonadism . Although these may resolve after 6 postoperative months . Clinicians should repeat testing to establish when and if the patient has recovered. Encourage diet rich in calcium and vitamin D , regular exercise . Treating the specific comorbidities associated with CS ( eg , HTN, DM, osteoporosis). Detailed fracture assessment , BMD, lateral imaging of the spine, Testing for HPA axis recovery Testing for recurrence throughout life, ( except in patients who underwent resection of an adrenal adenoma with a CT density of < 10 Hounsfield units)

40. Approach for long-term follow-up 3-month assessments History of frequency of stress doses, and symptoms of adrenal insufficiency. Measure weight, and BP and look for signs of overtreatment. 3-6-month assessments: Testing for HPA axis recovery Morning cortisol level obtained (before that day's glucocorticoid dose), followed by an ACTH stimulation test starting when the level is 7.4 μg /dL or more . The axis has recovered once 30-minute cortisol > 18 mcg/d L, hydrocortisone can be weaned and stopped. Patients with cortisol levels below 5 μg /dL should remain on glucocorticoids until retested in 3–6 months.

41. Approach for long-term follow-up In Cushing disease treated by TSS Measuring serum sodium several times during the first 5–14 days We recommend assessing free T 4  and prolactin within 1–2 weeks of surgery, to evaluate for overt hypopituitarism. Obtaining a postoperative pituitary MRI within 1–3 months.

42. Approach for long-term follow-up Patients have had bilateral adrenalectomy NO ACTH stimulation test In addition, because of the potential risk of corticotroph tumor progression ( Nelson syndrome ), lifelong follow-up is important with clinical examinations for hyperpigmentation , MRI of the pituitary and plasma ACTH measurements , annually for seven years , and then intermittently if there has been no tumor progression

43. Approach for long-term follow-up Patients with Carney complex and primary pigmented nodular adrenocortical disease Lifelong follow-up Followed annually for the development of new or recurrent cardiac myxomas and other features of the syndrome (cardiac myxomas, acromegaly, thyroid lesions) Patients with ectopic ACTH production Every 3 months ensure that they have no signs of toxicity from ketoconazole (including checking liver function tests) or other steroid synthesis inhibitor . Reassessment at 6-month intervals to look for the origin of the tumor.

44. Approach for long-term follow-up After pituitary irradiation Use medications to normalize cortisol until radiation takes effect. Assessing serum cortisol or UFC off-medication at 6- to 12-month intervals and if patients develop new adrenal insufficiency symptoms while on stable medical therapy. Hypopituitarism is a risk with all forms of radiation. careful assessment of anterior pituitary function post-therapy, annually (at least) or sooner if hormone deficiency symptoms develop.

45. Recurrence/ when to think? Evaluate patients for possible recurrence when the HPA axis recovers , and then annually , or sooner if they have clinical symptoms . Early recovery (within 6 mo ) of HPA axis function may indicate an increased risk of recurrence  Elevated late-night serum/salivary cortisol is one of the earliest biochemically detectable signs of recurrence and almost always precedes elevated urine cortisol.