Adrenocortical insufficiency case study .pptx

This ppt contains the information of a patient with Adrenocortical Isnufficiency.

1. بسم الله الرحمن الرحیم

2. CASE PRESENTATION Internal Medicine By: Dr. Mahmoud Khan Zazai Supervised By: Dr. Riazullah omar 28-Nov-2021

3. BIO DATA Name: XY Age: 75-year-old Sex: Male Address: Kabul Date of visit. 18-11-2021

4. CHIEF COMPLAINT N/V  2-month Abdomen pain  2-month

5. HISTORY OF PRESENT ILLNESS A 75-year-old man present to CIH complaining of abdominal pain, nausea and vomiting since 2 months. He state the pain begin gradually in upper abdomen, was about 4/10 intense, sharp in character, worsen after food intake and relieve with N/V. He also developed nausea and vomiting, 5- 6 times in day, contents was food particle, no blood, initiated with food or water intake. He was also complaining of orthostatic dizziness along with burning micturition and increase in frequency of urination since 7-days. There was weight loss, loss of appetite, but no fever, no diarrhea, and no sweating.

6. PAST HISTORY Appendectomy long time

7. DRUG HISTORY URSO  1-week Pantoprazole  1-week Hepamerz  1-week Librax 1-week

8. SOCIAL HISTORY Engineer No Smoking

9. REVIEW OF SYSTEM General: Fatigue, weakness

10. VITAL SIGNS

11. PHYSICAL EXAM General: Looking ill + not in distress HEENT: Icteric sclera, Pale conjunctiva, Dry mouth NECK: Supple CHEST: Clear to auscultation bilaterally HEART: S1+S2+0 RRR ABDOMEN: Scaphoid in shape, Soft , upper abdomen tenderness on light palpation, and bowel sounds audible MS : Cold, no scar, no edema NEURO : Oriented x3

12. PROBLEM LIST Orthostatic Dizziness N/V Upper abdomen pain Fatigue Weak Anorexia Weight Loss Burning micturition Cold Extremities Icteric Sclera

13. ASSESSMENT ? Hypovolemic shock vs Cardiogenic Shock R/o ACS P/O Ascending Cholangitis PUD vs GI Malignancies Cystitis vs BPH R/O cholelithiasis R/O Prostate CA P/O Adrenal Insufficiency Lyte Imbalance Subclavian steel syndrome

14. MANAGEMENT PLAN ECG CBC ESR CRP Lytes Creatinine BUN Urine Analysis Total Bilirubin SGPT SGOT Abdominal U/S

15. CBC --------------------------- WBC: 12.5 LYM%: 14.1 GRA%: 79.6 HGB: 15.5 MCV: 80.9 PLT: 178 ESR : 10 CRP : <2.5 Na : 126.1 K : 4.6 Creatinine : 3.2 BUN : 149.5

16. Urine Analysis SUGAR : Trace PUS CELLS: 01-02 RBC CELLS : 00-01 EP CELLS : 02-03 Total Bilirubin : 1.1 SGPT : 25 SGOT : 20 Abdominal U/S : Cholelithiasis (single), Enlarged Prostate

17. Bolus IVF Keep NPO Record intake/output q6hr Pantoprazole Metoclopramide

18. 2 nd DOH Patient was doing well, upper abdomen pain decreased, two times N/V present. Vital Signs: stable General: Looking ill + not in distress HEENT: Pale conjunctiva, Dry mouth ABDOMEN : Scaphoid in shape, Soft, upper abdomen tenderness on light palpation, and bowel sounds audible Other System: No remarkable Findings Intake/Output: 2820/1500

19. CONT… ASSESSMENT: AKI (improving) Lyte Imbalance PUD vs GI Malignancy BPH Cholelithiasis P/O GI Prostate Malignancy P/O Adrenal Insufficiency PLAN: Creatinine: 2.0 Na: 131.1 K: 3.0 Upper GI Endoscopy Continue same management

20. 3 rd DOH Patient was doing well, upper abdomen pain improved, no N/V. Vital Signs: stable General: Looking ill + not in distress HEENT: MMM ABDOMEN : Scaphoid in shape, Soft, and bowel sounds audible Other System: No remarkable Findings Intake/Output: 2400/2200

21. CONT… ASSESSMENT : PUD vs GI Malignancy BPH Cholelithiasis P/O Adrenal Insufficiency P/O Prostate Malignancy PLAN: Creatinine: 0.9 Na: 131 K: 4 PSA: 11.5 Upper GI Endoscopy Continue same management

22. 4th DOH Patient was not doing well, he had 4-5 time vomiting, content food particles, yellow in color and no blood in it, also patient complains of constipation. Vital Signs: HR: 52, otherwise stable General: Looking good HEENT: MMM CVS: S1+S2+0, Bradycardia ABDOMEN : Scaphoid in shape, Soft, and bowel sounds audible Other System: No remarkable Findings

23. CONT… ASSESSMENT : PUD BPH Cholelithiasis P/O Adrenal Insufficiency P/O GI Malignancy P/O GI Prostate Malignancy PLAN: ECG: sinus Bradycardia Free Cortisol Level Upper GI Endoscopy Continue same management

24. 5 th & 6 th DOH Patient was doing well, only one time he had N/V, stool passed (dark in color). Vital Signs: HR: 50, otherwise stable General: Looking good HEENT: MMM CVS: S1+S2+0, Bradycardia ABDOMEN : Scaphoid in shape, Soft, and bowel sounds audible Other System: No remarkable Findings

25. CONT… ASSESSMENT : PUD BPH Cholelithiasis P/O Acute Pancreatitis P/O Adrenal Insufficiency P/O GI Malignancy P/O GI Prostate Malignancy

26. CONT… Free Cortisol Level: 1.30 Lipase: 20 Upper GI Endoscopy : Esophagus : More than 3 liner ulcer and breaks seen in distal esophagus. Stomach: Diffuse ulcerated erosions predominant in pyloric area. Duodenum: No Ulcer, No Mass Post Endoscopy inst : Esophagitis , some erosive gastritis, Biopsy taken PLAN: Sucralfat TID Domperidon TID Pantoprazole BD Normal Diet

27. 7 th DOH Patient was doing well, no N/V. Vital Signs: stable General: Looking good HEENT: MMM CVS: S1+S2+0 ABDOMEN : Scaphoid in shape, Soft, and bowel sounds audible Other System: No remarkable Findings

28. ASSESSMENT Esophagitis Gastritis Adrenal Insufficiency BPH P/O Colon Ca P/O Prostate Ca

29. PLAN: Discharge patient on: Hydrocortisone BD Esomeprazole BD Dompridon TID Sucralfat TID Dutasteride + Tamsulosin OD Follow Biopsy

30. CLINICAL CASE A 28-year-old man presents to the emergency room with headache, stiff neck, and fever . He is admitted to the intensive care unit after a lumbar puncture and is diagnosed with meningococcal meningitis . On hospital day 3, despite antibiotic treatment, he continues to be hypotensive and in shock , and complains of new abdominal pain when he is awake. On physical examination, he has multiple areas of purpura on his skin . Serum testing reveals hyponatremia, hyperkalemia, and hypoglycemia .

31. ADRENOCORTICAL INSUFFICIENCY Dr. MAHMOUD KHAN ZAZAI

32. LEARNING OBJECTIVES Learn the clinical manifestations and objective findings of chronic and acute adrenocortical insufficiency Understand the factors and illnesses that predispose to adrenocortical insufficiency Know the differential diagnosis of adrenocortical insufficiency Learn the treatments for chronic and acute adrenocortical insufficiency and how they differ Understand the follow-up and testing required after an episode of acute adrenocortical insufficiency Know how to alter treatment in patients with chronic adrenocortical insufficiency who become acutely ill

33. QUESTIONS 1 .  What are the salient features of this patient’s problem? 2.  How do you think through his problem? 3.  What are the key features, including essentials of diagnosis and general considerations, of adrenocortical insufficiency? 4.  What are the symptoms and signs of adrenocortical insufficiency? 5.  What is the differential diagnosis of adrenocortical insufficiency? 6.  What are laboratory, and imaging findings in adrenocortical insufficiency? 7.  What are the treatments for adrenocortical insufficiency? 8.  What is the outcome, including follow-up, complications, and prognosis, of adrenocortical insufficiency? 9.  When should patients with adrenocortical insufficiency be referred to a specialist or admitted to the hospital?

34. 1. Salient Features What are the salient features of this patient’s problem ? Meningococcal meningitis with purpura hypotension and shock abdominal pain Hyponatremia Hyperkalemia hypoglycemia

35. 2. How to Think Through How do you think through his problem? This case shows the importance of keeping the full differential diagnosis in mind when treating a patient in shock. This patient has a known infection, but what other causes of hypotension, beyond septic shock, could be at play? (Cardiogenic shock, anaphylactic shock, neurogenic shock, and adrenal insufficiency.) How should adrenal function be evaluated? (AM plasma cortisol level < 3 mg/ dL is diagnostic of adrenal insufficiency.)

36. Cont … If adrenal insufficiency is suspected, what are possible causes? (Previously undetected primary adrenal insufficiency [Addison disease]; indolent adrenal infection such as tuberculosis; secondary [pituitary level] or tertiary [hypothalamic] lesions are possible, but rare. ) What is the eponym for adrenal infarction in the setting of meningococcemia ? (Waterhouse– Friderichsen syndrome.)

37. Cont … How do hyponatremia and hyperkalemia help localize the problem ? (Only primary adrenal disease will impact production of aldosterone.) Is imaging indicated? (Yes. Computed tomography [CT] scan of the abdomen may show adrenal hemorrhage.) How should he be treated? (As soon as a plasma cortisol level is drawn, intravenous [IV] hydrocortisone for hypocortisolism and D50W for hypoglycemia should be started empirically.)

38. 3. Key Features What are the key features, including essentials of diagnosis and general considerations, of adrenocortical insufficiency? Essentials of Diagnosis Weakness Fatigue Anorexia Weight loss Nausea and vomiting Diarrhea Abdominal pain Muscle and joint pains Amenorrhea Sparse axillary hair Increased skin pigmentation Hypotension Dehydration Small heart. Serum sodium may be low potassium , calcium, and urea nitrogen may be elevated Cosyntropin (ACTH 1–24) administration is unable to stimulate an increase in serum cortisol to > 20 µg/ dL Plasma adrenocorticotropic hormone (ACTH) level elevated

39. General Considerations Adrenocortical insufficiency can be  chronic  or  acute

40. Chronic Adrenocortical Insufficiency Addison disease refers to primary chronic adrenocortical insufficiency caused by dysfunction or absence of the adrenal cortices ; it is distinct from secondary chronic adrenocortical insufficiency caused by deficient secretion of ACTH. The prevalence of chronic adrenocortical insufficiency is about 140 per million , with an annual incidence of about 4 per million in the United States. Characterized by chronic deficiency of cortisol, with consequent elevation of serum ACTH and α-melanocyte stimulating hormone (α-MSH), causing skin pigmentation that can be subtle or strikingly dark.

41. Causes of chronic adrenocortical insufficiency Autoimmune adrenal destruction of adrenal glands is most common cause in the United States May be associated with: Autoimmune thyroid disease Hypoparathyroidism Type 1 diabetes mellitus Vitiligo Alopecia areata Celiac disease (gluten enteropathy ) Primary ovarian failure Testicular failure Pernicious anemia

42. Cont … Combination of Addison disease and hypothyroidism is Schmidt syndrome Infections (Tuberculosis, Coccidioidomycosis and histoplasmosis ) Bilateral adrenal hemorrhage may occur spontaneously, or with Sepsis Heparin-associated thrombocytopenia or anticoagulation Antiphospholipid antibody syndrome Surgery (postoperatively) or trauma

43. Cont … Rare causes of chronic adrenocortical insufficiency Lymphoma Metastatic carcinoma Syphilitic gummas Scleroderma Amyloid disease Hemochromatosis

44. Acute Adrenocortical Insufficiency Acute adrenocortical insufficiency is an emergency  caused by insufficient cortisol Causes of acute adrenocortical insufficiency May occur in patients treated for chronic adrenal insufficiency or as its presenting manifestation More common in primary adrenal insufficiency ( adrenal gland disorder; Addison disease ) than secondary adrenocortical hypofunction ( pituitary gland disorder )

45. Cont … Stress Withdrawal of adrenocortical hormone Bilateral adrenalectomy Injury to both adrenals by trauma, hemorrhage, anticoagulant therapy, thrombosis, infection, or, rarely, metastatic carcinoma Pituitary necrosis , or when thyroid hormone replacement is given to a patient with adrenal insufficiency Meningococcemia may cause adrenal insufficiency secondary to adrenal infarction (Waterhouse– Friderichsen syndrome)

46. 4. Symptoms and Signs What are the symptoms and signs of adrenocortical insufficiency? Chronic Adrenal Insufficiency Symptom onset is usually gradual and the diagnosis is often delayed since many early symptoms are nonspecific. Muscle weakness and fatigue Fever Anorexia , nausea and vomiting, weight loss Anxiety , mental irritability, and emotional changes

47. Cont.. Patients usually have significant pain Arthralgias Myalgias Chest pain Abdominal pain Back pain Leg pain Headache

48. Cont … Skin Diffuse tanning over nonexposed and exposed skin or multiple freckles Hyperpigmentation , especially knuckles, elbows, knees, posterior neck, palmar creases, nail beds, pressure areas, and new scars. Vitiligo (10 %)

49. Cont … Hypoglycemia In diabetics, increased insulin sensitivity and hypoglycemic reactions Hypotension and orthostatic is typical Ninety percent have systolic blood pressure (SBP) < 110 mm Hg SBP > 130 mm Hg is rare Small heart Scant axillary and pubic hair (especially in women, if hypogonadism also present)

50. Acute Adrenal Insufficiency Headache Lassitude nausea and vomiting abdominal pain, and diarrhea Confusion or coma Fever, as high as 40.6°C or more Dehydration, hypotension, and shock

51. Cont … Recurrent hypoglycemia Reduced insulin requirements in patients with preexisting type 1 diabetes mellitus Meningococcemia is associated with purpura and adrenal insufficiency secondary to adrenal infarction ( Waterhouse– Friderichsen syndrome )

52. 5. Differential Diagnosis What is the differential diagnosis of adrenocortical insufficiency? Other cause of hypotension or shock Medications Sepsis Cardiogenic Hypovolemic Anaphylaxis Hyperkalemia due to other cause Chronic kidney disease Gastrointestinal bleeding Rhabdomyolysis Hyperkalemic paralysis Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers Spironolactone

53. Cont … Hyponatremia due to other cause Hypothyroidism Diuretic use Heart failure Cirrhosis Vomiting Diarrhea Severe illness Major surgery

54. Cont … Abdominal pain due to other cause Hyperpigmentation due to other cause, eg , hemochromatosis Isolated hypoaldosteronism Occult cancer Secondary adrenal insufficiency (hypopituitarism)

55. 6. Laboratory and Imaging Findings What are laboratory, and imaging findings in adrenocortical insufficiency? Laboratory Tests Moderate neutropenia Lymphocytosis , and total eosinophil count > 300/ μL Hyponatremia (90%), hyperkalemia (65%). (Patients with diarrhea may not be hyperkalemic ) Fasting blood glucose may be low Hypercalcemia may be present Blood , sputum, or urine culture may be positive if bacterial infection is precipitating acute cause

56. Plasma cortisol level < 3 mg/ dL (< 83 nmol /L) at 8 AM is diagnostic, especially if accompanied by simultaneous elevated ACTH (usually > 200 pg /mL [> 44 pmol /L]) Cosyntropin stimulation test: Synthetic ACTH 1 to 24 ( cosyntropin ), 0.25 mg, given intramuscularly Serum cortisol obtained 45 minutes later Normally, cortisol rises to ≥ 20 μg / dL For patients taking corticosteroids, hydrocortisone must not be given for at least 8 hours before the test Other corticosteroids (such as dexamethasone) do not interfere with specific assays for cortisol Plasma ACTH markedly elevated (generally > 200 pg /mL) if patient has primary adrenal disease.

57. Imaging Studies Chest radiograph for tuberculosis, fungal infection, or cancer Abdominal CT shows small noncalcified adrenals in autoimmune Addison disease Adrenals enlarged in about 85% of cases of metastatic or granulomatous disease Calcification noted in cases of tuberculosis (~50%), hemorrhage , fungal infection, pheochromocytoma , and melanoma

58. 7. Treatments What are the treatments for adrenocortical insufficiency? Chronic Adrenal Insufficiency Corticosteroid and mineralocorticoid replacement required in most cases hydrocortisone alone may be adequate in mild cases Hydrocortisone Drug of choice Usually 15 to 30 mg orally in two divided doses: two-thirds in morning and one-third in late night or early evening Prednisone 2 to 4 mg orally every morning and 1 to 2 mg orally every night or evening is an alternative

59. Corticosteroid dose raised in case of infection, trauma, surgery, diagnostic procedures, or other stress Fludrocortisone 0.05 to 0.3 mg once daily orally or once every other day, required by many patients Dosage increased for Postural hypotension Hyponatremia Hyperkalemia Fatigue Elevated plasma renin activity Dosage  decreased for Edema Hypokalemia Hypertension

60. Acute Adrenal Insufficiency If acute adrenocortical insufficiency is suspected Draw blood sample for electrolytes, cortisol, and ACTH determinations Without waiting for results, treat immediately with hydrocortisone 100 to 300 mg intravenously and saline Then, continue hydrocortisone 50 mg every 6 hours intravenously for first day every 8 hours the second day and taper as clinically appropriate Infections should be treated immediately and vigorously; broad-spectrum antibiotics given empirically while waiting for initial culture results.

61. Cont … D50W to treat hypoglycemia with careful monitoring of serum electrolytes, blood urea nitrogen, and creatinine When patient is able to take oral medication: Give hydrocortisone, 10 to 20 mg every 6 hours orally , and taper to maintenance levels Most require hydrocortisone twice daily : 10 to 20 mg every morning, 5 to 10 mg every night

62. Cont … Mineralocorticoid therapy Not needed when large amounts of hydrocortisone are being given However, as the dose is reduced , may need to add fludrocortisone , 0.05 to 0.2 mg once daily orally Some patients never require fludrocortisone or become edematous at doses > 0.05 mg once or twice weekly

63. 8. Outcomes What is the outcome, including follow-up, complications, and prognosis, of adrenocortical insufficiency? Follow-Up Follow clinically and adjust corticosteroid and (if required) mineralocorticoid doses Fatigue in treated patients may indicate Suboptimal dosing of medication Electrolyte imbalance Concurrent problems, such as hypothyroidism or diabetes mellitus Corticosteroid dose must be increased in case of physiologic stress

64. COMPLICATIONS Complications of underlying disease ( eg , tuberculosis) are more likely Adrenal crisis may be precipitated by intercurrent infections Fatigue often persists despite treatment Excessive corticosteroid replacement can cause Cushing syndrome Acute adrenocortical insufficiency can cause shock and death if untreated

65. PROGNOSIS Most patients able to live fully active lives Life expectancy is normal if adrenal insufficiency is diagnosed and treated with appropriate doses of corticosteroids and (if required) mineralocorticoids However, associated conditions can pose additional health risks, eg , patients with adrenal tuberculosis may have serious systemic infection Corticosteroid and mineralocorticoid replacement must not be stopped

66. Cont … Patients should wear medical alert bracelet or medal reading “Adrenal insufficiency—takes hydrocortisone” Higher doses of corticosteroids must be administered to patients with infection, trauma, or surgery to prevent adrenal crisis Acute adrenocortical insufficiency frequently unrecognized and untreated since manifestations mimic more common conditions Lack of treatment for acute adrenocortical insufficiency leads to shock that is unresponsive to volume replacement and vasopressors, resulting in death

67. 9. When to Refer and When to Admit When should patients with adrenocortical insufficiency be referred to a specialist or admitted to the hospital ? When to Refer If abnormal cosyntropin stimulation test to establish degree of permanent adrenal insufficiency and to establish its cause if possible When to Admit Patients presenting with shock suspected to be deriving from acute adrenal crisis Patients presenting with an acute abdomen to exclude acute adrenal insufficiency as its cause Patients threatened by acute adrenal crisis ( eg , those with severe bacterial infections) requiring immediate IV hydrocortisone administration

68. SUGGESTED REFERENCES Charmandari E et al. Adrenal insufficiency. Lancet. 2014 Jun 21;383(9935):2152–2167. [PMID: 24503135] Finkielstain GP et al. Clinical characteristics of a cohort of 244 patients with congenital adrenal hyperplasia. J Clin Endocrinol Metab . 2012 Dec;97(12):4429–4438. [PMID: 22990093] Husebye ES et al. Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med. 2014 Feb;275(2):104–115. [PMID: 24330030] Nilsson AG et al. Prospective evaluation of long-term safety of dual-release hydrocortisone replacement administered once daily in patients with adrenal insufficiency. Eur J Endocrinol . 2014 Sep;171(3):369–377. [PMID: 24944332] Quinkler M et al. What is the best long-term management strategy for patients with primary adrenal insufficiency? Clin Endocrinol ( Oxf ). 2012 Jan;76(1):21–25. [PMID: 21585418] Tiemensma J et al. Psychological morbidity and impaired quality of life in patients with stable treatment for primary adrenal insufficiency: cross-sectional study and review of the literature. Eur J Endocrinol . 2014 Aug;171(2):171–182. [PMID: 24801589] Yong SL et al. Supplemental perioperative steroids for surgical patients with adrenal insufficiency. Cochrane Database Syst Rev. 2012 Dec 12;12:CD005367. [PMID: 23235622] Yuen KC et al. Adrenal insufficiency in pregnancy: challenging issues in diagnosis and management. Endocrine. 2013 Oct;44(2):283–292. [PMID: 23377701 ]

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