Interesting case 28 สงหาคม 2556

โดย ... นกศกษาแพทยชนปท 6 / อ.รงนภา ลออธนกล

ผปวยหญงไทยค อาย 61 ป Chief complaint: ไข 1 วนกอนมา รพ.

Present illness:

1 วนกอน - มไขต าๆ ไอ มเสมหะสขาวขน ไมมน ามก ไมมปสสาวะแสบขด ไมมคลนไสอาเจยน ไมมทองเสย ถายเหลว ตอมาไอเยอะขน รวมกบเหนอยมากขน จงไป รพช.

ทรพช. แรกรบพบ BP 100/60 mmHg, Temp. 38.6°C ตอมา ผ ปวยเหนอยมากขน หายใจเรวขน พบ HR 160/min. EKG เปน SVT แพทยจงให adenosine หลงจากนน EKG เปลยนเปน sinus rhythm rate 100/min. แตผ ปวยยงม BP 70/50 mmHg, pulse 120 /min แพทยได load NSS

3500 ml และให inotropic เปน Dopamine 1000 mg+ 5%DW 500

ml. IV drip และสงตวมา

Past history:

- Underlying COPD รบยาท รพช.เปนยาพน

- ปฏเสธประวตดมสรา และสบบหร

- ปฏเสธประวตการใช steroid

- ปฏเสธการแพยา แพอาหาร

- สขภาพกอนหนานแขงแรงด

Family history:

- ทกคนในครอบครว สขภาพแขงแรงด

Physical examination

• A Thai woman, Good consciousness, well co-operative • Vital Signs : BT 37.3 °C ,PR 60 /min, BP 100/60 mmHg, RR 18 /min • HEENT: not pale conjunctiva, no icteric sclerae, no lid lag, no lid

retraction, no thyroid gland enlargement, hyperpigmented gum • Heart : no active precordium, no heaving, no thrill, normal S1,S2 ,no

murmur • Lungs : Equal breath sound, crepitation both lungs • Abdomen : not distend, normoactive bowel sound, not tender, liver span

10 cm., spleen no dullness on percussion. • Extremities : no pitting edema, hyperpigmentation at both palmar crease

Hyperpigmented gum

Hyperpigmentation at both palmar crease

Physical examination

Neurological exam

– Good consciousness, follow to command

– Cranial nerve : pupil 3 mm. RTLBE, Full EOM, no facial palsy, normal gag reflex

– Motor normal tone, power gr. V all extremities

– Sensory pinprick sensation intact

– Reflex 2+ all extremities

– BBK absent

Problem List

• Shock

Differential Diagnosis

• Septic shock

• Hypovolumic shock

• Cardiogenic shock • Adrenal (crisis) shock

Investigation

• CBC

- WBC 17,100 /uL (N95.7%,LY3.2%,MO1.1%,EO0.0%,BA0.0%)

- Hb 9.9 ,Hct 30.8% MCV 76.4 RDW 16.1

- Platelet 227,000

Investigation

• Chemistry

– BUN 16.0

– Creatinine 1.27

– Sodium 134.9

– Potassium 3.38

– Chloride 106.2

– CO2 19.1

• Liver Function Test

– DB/TB 0.1/0.4

– AST/ALT 38/11

– ALP 55

– TP 6.0

– Albumin 3.2

– Globulin 2.8

– Cholesterol 103

• Serum Cortisol (ขณะท BP drop)

- Cortisol 1.9

• Serum L-Lactate

- L-lactate 4.8

• Thyroid Function Test – FT4 : 0.8 (0.6-1.6) – FT3 : 3.7 (2.39-6.79) – TSH : 0.22 (0.3-5.0)

• UA – Yellow ,cloudy

– Specific gravity 1.011

– pH 6.0

– Protein 1+

– Leukocyte 1+

– RBC 10-20 cell/HPF

– WBC 5-10 cell/HPF

– Squamous epithelium cell : negative

– Bacterial : Few cocci

– Mucous thread : Trace

Adrenal Insufficiency

Adrenal Insufficiency : Etiology Primary AI : Addison’s Disease

1. Autoimmune Disease

-APS -Isolated Autoimmune adrenalitis

2. Congenital - CAH

3. Infection - TB Histoplasma CMC

4. Vascular - Hemorrhage (meningococcemia) -1 Antiphospholipid synd. -Trauma

5. Metastatic

6. Drugs Ketoconazole Rifampicin

7. Infiltrative disease Hemochromatosis Amyloidosis

Adrenal Insufficiency : Etiology

Secondary AI HPA Axis dysfunction 1. Extrinsic -Exogenous Corticosteroid

2. Intrinsic

-Pituitary Tumor -Brian Tumor -Pituitary Apoplexy -Pituitary Irradiation -Pituitary Infiltration : TB, Actinomycosis, Sarcoidosis -Congenital isolated ACTH Deficiency

Clinical Manifestation

Primary adrenal insufficiency • hyperpigmentation, hyper K, vitiligo, adrenal

calcification, orthostatic hypotension, craving salt Secondary adrenal insufficiency • Pale skin not due to anemia, decrease libido and

impotence, DI, delayed puberty, loss of axillary or pubic hair

Both • Nausea, vomiting, diarrhea, hypoNa, orthostatic

hypotension, shock, anorexia, weight loss, hypoglycemia, lymphocytosis, normocytic anemia, tiredness, depression

www.medscape.com

Investigation

ACTH stimulation test

Laboratory Testing

Initial testing

• Measure early morning serum cortisol

If Cortisol ≥ 3 µg/dL

primary adrenal insufficiency less likely

However, if serious consideration

is given to this diagnosis, perform dynamic testing

Dynamic tests

• Insulin-induced hypoglycemia

• 250 μg ACTH stimulation test – 250 μg ACTH IV – Serum cortisol 0, 30 and 60 min – Peak cortisol ≥18 μg/dl exclude adrenal insufficiency

• 1 μg ACTH stimulation test – More sensitivity test in secondary adrenal insufficiency

Goal standard test

William Textbook of Endocrinology 11th edition

Primary or Secondary AI?

• ACTH level

If ACTH >100 pg/dL

Primary adrenal insufficiency

Imaging Studies

• MRI/CT based on stimulation testing

• If stimulation testing or absolute cortisol suggests adrenal failure – MRI/CT of adrenal glands

• If stimulation testing suggests pituitary failure – MRI/CT of pituitary

Management

Adrenal Crisis

• Required urgent diagnosis and management

• Blood for serum cortisol, renin, ACTH, chemistry

• Therapy initiated immediately

• Find precipitating cause and properly treated

Adrenal Crisis

• 1-3 liters of IV Fluid in 12-24 hr. based on volume status and Urine output – 0.9%NaCl

– 5%D/NSS – to correct possible hypoglycemia

• Glucocorticoid – Dexamethasone 4 mg IV bolus for no history of AI

– Hydrocortisone 100 mg IV bolus for 1° AI with high K

– Any preparation

• Taper over 1-3 days and change to an oral maintenance dose

Chronic AI

• 1˚ Chronic AI

– Replacement

• Glucocorticoid

• Mineralocorticoid

• Androgen

• 2˚ Chronic AI

– Replacement

• Glucocorticoid

Chronic AI

• Patient education

– The nature of hormonal deficit and the rationale for

treatment

– Maintenance med. And adjustment during minor illnesses

– When to consult a clinician

– When and how to injected a glucocorticoid for emergencies

Chronic AI

• Ideal glucocorticoid replacement therapy

– Mimic the endogenous cortisol rhythm

– Have little inter-individual variability in metabolism

– Be amenable to easy dose titration

– Minimize the risk of over treatment, resulting in iatrogenic Cushing’s syndrome

Chronic AI

• Prednisolone

– 5 mg oral OD – General dose

• Advantage

• OD dose

• Disadvantage

• More variable inter-individual metabolism

• Over-treatment

Chronic AI

• Monitoring dose

– Symptoms assessment

• Lowest dose that relieves sx.

• Avoid signs & symptoms of excess steroid : wt. gain, facial plethora, Cushing’s, osteoporosis

– Plasma ACTH

• Low normal or suppressed morning plasma ACTH from excessive glucocorticoid

Chronic AI

• Mineralocorticoid replacement

• In primary adrenal insuff.

• Fludrocortisone is drug of choice

• Usual dose 0.1 mg/day

• Monitor – orthostatic hypotension, blood pr. & pulse, serum K, plasma renin activity : upper normal range

Chronic AI

• Androgen replacement - DHEA

• Esp. Women : Mood & Quality of life

• Only women who have sig. impaired mood & sense of well-being despite optimal glucocorticoid & mineralocorticoid replacement

• Start dose : 25-50 mg/day 3-6 mo.

• Monitor S/S & adverse effect – If not sig. improve or have adverse eff. : discontinue

Chronic AI

• Consider in 2˚ AI

– Replacement of thyroid hormone without glucocorticoid can precipitate acute adrenal crisis