Bondoc.Borela.Buenaventura.Buhat.Calaoagan.Carilo.Casi.Castano.Celino.Francisco.Garcia

Identifying data•M.I is 35 y/o •Female •Married•Filipino•Roman Catholic•Admitted for the first time at UERMMMC

Chief Complaint•Generalized body weakness of 12 hours

duration

History of Present IllnessHPI

0246810

0 12 8 4

Admission

days prior to admission

Inte

nsity

Past Medical History•Hypertension- diagnosed 7 years ago

Rx: Amlodipine 10 mg/tab 1 tab OD, Losartan 50 mg/tab 1 tab OD

•Surgery:▫1999: Right oophorectomy▫2008: total abdominal hysterectomy with left oophorectomy

•2 years PTA, admitted due to similar episode of weakness

•No history of DM, thyroid, kidney, lung, heart disorders

Family History•Father- DM type 2•Mother- hypertension•No family history of heart, lung, kidney

and connective tissue disorders

Personal and Social History•Unremarkable

Physical Exam•Awake, conscious, coherent, stretcher

bound•Vital signs: BP is 150/100, HR- 76 bpm,

RR-18 cpm, Temperature is 37.1 ˚C•Remarkable findings: grade 3/5 pulses,

Muscle strenght: 3/5 both upper extremities1/5 on both lower extremites

•Normotonic, normoreflexive, intact sensation, other neurological exams are normal.

<3.4 mmol/L >4.3 mmol/L

>50,000 WBC count<50,000 WBC count

Bilateral Weakness

Neurologic Neuromuscular Junction MyopathiesSerum K+

CBC

Recent insulin, B-adrenergic, theophyline use, or, more rarely, anabolic stimulus or family history suggestive of hypokalemic

periodic paralysis

Skin, GI or Renal K LossNo Yes

ABGAcidosis Alkalosis

Urine K+

<20 mEq K/24 hrs >20 mEq K/24 hrs

Redistribution or hypokalemic periodic paralysis

Pseudohypokalemia

Recent diuretic use?YesNo Probable diuretic-

induced hypokalemiaCHF, hepatic insufficiency, nephrotic syndrome or renal

artery stenosisYes Probable secondary

hyperaldosteronismNo

Hypomagnesemia?Yes

Hypomagnesemia induced hypokalemia

No Serum bicarbonate?

Low RTA, DKA or ureterosigmoidostomy

Normal or High

Random Urine K/C ratio

<1.5Thyrotoxic PP

>1.5Blood Pressure

Elevated

PRA and PAC

Both LowCAHCushing SyndromeLiddle Syndrome

Both High

RVH, COA, RST

High PACLow PRA

Primary Aldosteronism

LowHighTSH >4.25 uLU/mLFT4: >1.7 ng/L

TSH <0.34 uLU/mLFT4 <0.8 ng/L

Bilateral Weakness

Neurologic• Altered mental status• Signs of UMN/LMN• Sensory deficit

• Autonomic involvement

Neuromuscular Junction• Bulbar signs• Fatigability

Myopathies• All limbs involved

• No sensory involvement• No autonomic involvement

<3.4 mmol/L>4.3 mmol/L

Bilateral Weakness

Neurologic Neuromuscular Junction Myopathies

Serum K+

>50,000 WBC count<50,000 WBC countCBC

Recent insulin, B-adrenergic, theophyline use, or, more rarely, anabolic stimulus or family history suggestive of hypokalemic

periodic paralysis

Skin, GI or Renal K LossNo Yes

ABGAcidosis Alkalosis

Urine K+

<20 mEq K/24 hrs >20 mEq K/24 hrs

Redistribution or hypokalemic periodic paralysis

Pseudohypokalemia

Recent diuretic use?YesNo Probable diuretic-

induced hypokalemiaCHF, hepatic insufficiency, nephrotic syndrome or renal

artery stenosisYes Probable secondary

hyperaldosteronism

NoHypomagnesemia?

YesHypomagnesemia induced hypokalemia

No Serum bicarbonate?

Low RTA, DKA or ureterosigmoidostomy

Normal or High

Random Urine K/C ratio

<1.5Thyrotoxic PP

>1.5Blood Pressure

Elevated

PRA and PAC

Both LowCAHCushing SyndromeLiddle Syndrome

Both High

RVH, COA, RST

High PACLow PRA

Primary Aldosteronism

LowHighTSH >4.25 uLU/mLFT4: >1.7 ng/L

TSH <0.34 uLU/mLFT4 <0.8 ng/L

Differentials DiagnosesRule In Rule Out

LIDDELL’S SYNDROME

Presence of hypokalemiaPresence of metabolic alkalosisCommonly presents with hypertensionPossible family history of the disease

On the basis of imaging which showed a hypodense nodule on left lateral limb, which supports primary hyperaldosteronism↑ serum sodium↓ serum Aldosterone & renin

Rule In Rule OutTHYROTOXIC HYPOKALEMIC PERIODIC PARALYSIS

Presents with episodic generalized weakness

Presence of normal thyroid hormone levels during the episode of weakness↑ Thyroid function testLack of findings suggestive to support

MYASTHENIA GRAVIS

Presence of weakness in upper and lower limbs

Failure to regain movement/muscle strength after restLack of any ocular findings on neurological examLab findings are not suggestive to support this differential

Rule In Rule Out

Rule in Rule outDIABETES MELLITUS

•Weakness of lower extremities•Family history of diabetes •Presence of hypertension

•Inc FBS•No improvement of symptoms even after potassium administration•Lack of other DM indicators•Lack of associated findings such as abrupt onset of pain, tenderness and edema• There was also absent hardness and induration in the thighs

Primary Aldosteronism

Bilateral Adrenal Hyperplasia

Aldosterone Secreting Adenoma

CT scan

Primary Impression•Hypokalemia secondary to Primary

Aldosteronism (Conn’s Syndrome)

Why rule in Hypokalemia?•Acute Generalized weakness•Absence of UMN and LMN signs, Sensory

and Autonomic Involvement, Bulbar signs and Fatigability.

•Marked decrease in K+ level (1.5mmol/L)

Definition of Hypokalemia•Plasma K+ concentration <3.5 mmol/L•May be due to:

▫Decreased net intake▫Shift of K+ into cells▫Increased net loss

Clinical Manifestations of Hypokalemia•Usually asymptomatic

▫Unless plasma K+ concentration <3 mmol/L

•Fatigue, myalgia, muscular weakness of the lower extremities

•Severe Hypokalemia → progressive weakness, hypoventilation and complete paralysis

Occurrence of Metabolic Alkalosis•High pH (7.56)•Low pCO2 (32)•High HCO3 (28.7)

•Result of K+ redistribution + excessive renal K+ loss

•K+ depletion → intracellular acidification →increase HCO3 production

Primary Hyperaldosteronism (Conn’s Syndrome)

Why rule in Primary Hyperaldosteronism?•Triad of Hypertension, Hypokalemia, and

Metabolic Alkalosis•Elevated BP upon admission•Diagnosed with HTN 7 years ago•Poor compliance to maintenance

medications•Hypokalemia and Metabolic Acidosis on

lab tests

Primary Hyperaldosteronism•Syndrome associated with hypersecretion

of adrenal mineralocorticoid Aldosterone•Accounts for 5-10% of hypertension cases•Peak incidence → 30-60 years old

Pathophysiology•Cellular uptake of K+

▫Aldosterone → inc. Na+-K+ ATPase] → inc. transport of K+ into intracellular space

•Regulation of Renal K+ transport▫Aldosterone →inc. Apical Na conductance,

basolateral Na+-K+ ATPase activity, and electrogenic Na absorption in the collecting tubules → K+ movement from intracellular to luminal fluid

•Excessive aldosterone▫increased sodium retention▫decreased plasma renin ▫Increased renal potassium excretion

→hypokalemia

Clinical Manifestations•Hypokalemia•Muscular weakness

▫K+ depletion in the muscle cell membrane•Paresthesias•Headache•Polyuria•Polydipsia•Moderate hypertension (diastolic)

▫Due to inc. Na reabsorption

Plasma Renin Activity (PRA) Plasma Aldosterone Concentration (PAC)•Primary Hyperaldosteronism is consistent

with:▫↓PRA (baseline-12.69 ng/dL; post-12.36

ng/dL) ▫↑PAC (<0.1 ng/mL/hr),

Diagnostics•CT Scan

Course in the Ward• Day of Admission

▫BP: elevated at 150/100mmHg▫Inability to move both lower extremities▫CBC

↓ WBC count (13,100/L) with neutrophil predominance in absolute count

▫Serum electrolytes ↓ potassium (1.5mmol/L)

▫Albumin & BUN: normal

Course in the Ward•Day of admission

▫Uric acid: ↑ (22 umol/L)▫Creatinine: ↑ (100 umol/L)▫Urine electrolytes

potassium (9.2 mmol/l) ▫ABG

↑ pH (7.56) and HCO3 (28.7mmol/L) ↓ pCO2 (32mmHg)

Course in the Ward•Day of admission

▫FBS: normal▫Lipid profile: normal▫Urinalysis

Few bacterial and epithelial cells▫CXR: clear▫IV potassium chloride drip was started▫Thyroid function test was requested

Course in the Ward•Day 2-3

▫BP : 160/100mmHg▫Movement of both legs from side to side▫Gradual ↑ of potassium to 2.4mmol/L (day

3)▫Thyroid function test: normal TSH and fT4▫Plan: saline suppression test when

potassium level becomes normal

Course in the Ward•Day 4-5

▫Able to walk around the room without assist

▫Serum potassium: 4.7mmol/L IV potassium oral

▫Saline suppression test Baseline plasma renin activity and

aldosterone 2L IV saline infused over 4 hours plasma aldosterone

Course in the Ward•Day 4-5

▫BP : 200/100mmHg spironolactone 25mg/tab, 1 tablet BID and

felodipine10mg/tab, 1 tablet OD▫Dicharged with plans for follow-up▫Plasma renin and aldosterone results after

2 weeks

Follow-up•BP maintained at 100-120/70-80mmHg•No recurrent weakness•Saline suppression test

▫↑ baseline aldosterone: 12.69ng/dL▫↑ aldosterone post-infusion: 12.36ng/dL▫↓ plasma renin activitiy: <0.1ng/mL/hr

Follow-up•Abdominal CT scan requested

▫Hypodense enhancing nodule, measuring 9.9 x 7.6 x 11.7mm, at the lateral limb of the left adrenal gland

▫Right adrenal gland unremarkable▫No other abnormalities in the pre-contrast,

arterial, portal venous and wash-out phases▫Liver, pancreas and gallbladder are

unremarkable▫Referral to surgery

Management•Control of hypertension and aldosterone

level•Potassium supplementation•Abdominal CT scan•Adrenalectomy

▫Laparoscopic vs. open surgery•Complications•Prognosis

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