Clinical information and treatment of 10 cases of primary aldosteronism (PA) prominently characterized by hypokalemic myopathy (HM); 3 from our hospital and 7 reported by others.
| Case | Sex/Age, yrs | Duration of Disease* | Myalgia | Muscle Tenderness | Muscle Weakness | Power | Patellar Reflex | |||
|---|---|---|---|---|---|---|---|---|---|---|
| Proximal Muscle Groups of Upper Limbs | Distal Muscle Groups of Upper Limbs | Proximal Muscle Groups of Lower Limbs | Distal Muscle Groups of Lower Limbs | |||||||
| Our hospital | ||||||||||
| Case 1 | M 45 | 10 d | + | − | + | 2/5 | 2/5 | 2/5 | 2/5 | Diminished |
| Case 2 | F 36 | 3 ma | + | + | + | 3/5 | 4/5 | 3/5 | 4/5 | Diminished |
| Case 3 | F 41 | 7 d | + | − | + | 4/5 | 5/5 | 4/5 | 5/5 | Diminished |
| Hou2, n = 1 | F 27 | 6 d | + | + | + | 3/5 | 4/5 | 4/5 | 4/5 | Normal |
| Wang3, n = 1 | F 40 | 7 ma | − | − | Lower limbsb | 5/5 | 5/5 | 4/5 | 3/5 | Diminished |
| Lu4, n = 2 | ||||||||||
| Case 1 | M 50 | 6 d | Lower limbs | − | + | 4/5 | 4/5 | 3/5 | 4/5 | Diminished |
| Case 2 | F 38 | 1 mc | + | − | + | 5/5 | 5/5 | 5/5 | 5/5 | Diminished |
| Li5, n = 1 | M 35 | 1 ya | + | − | + | 3/5 | 3/5 | 3/5 | 3/5 | Diminished |
| Chen6, n = 1 | F 50 | 3 ya | + | + | + | 2/5 | 2/5 | 2/5 | 2/5 | Diminished |
| Zhang7, n =1 | F 46 | 3 da | − | − | + | 4/5 | 4/5 | 3/5 | 3/5 | Normal |
| Hypertension | Muscle Enzymes | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Case | Duration* | Highest BP, mm Hg | Serum K+, mmol/l | 24-h Urinary K+, mmol/l | CK, IU/l | CK-MB, IU/l | ALT, IU/l | AST, IU/l | LDH, IU/l | PRAd, μg/l/h | ALD+, pmol/l | CO2-CP, mmol/l | Blood pH | Urine pH |
| Our hospital | ||||||||||||||
| Case 1 | NS | 185/115 | 1.24 | 69.50 | 18561 | 82.6 | 385 | 825 | 1158 | 0.04 | 554.2 | 41.8 | NS | 8.0f |
| Case 2 | NS | 165/105 | 1.9 | 57.69 | 6954 | 45 | 255 | 687 | 948 | 0.05 | 495.3e | 34.8 | NS | 7.0 |
| Case 3 | 1 y | 185/110 | 2.0 | 49.55 | 3565 | 33 | 95 | 109 | 335 | 0.12 | 510.8e | 32.5 | NS | 7.0 |
| Hou2, n = 1 | 1 y | 178/110 | 1.89 | 181.50 | 9590 | 246.2 | 285 | 822 | 1172 | NS | 283.4e | NS | Normal | 8.0 |
| Wang3, n = 1 | 5 m | 180/110 | 1.89 | 216 | 11307 | NS | 674 | 394 | 1490 | 0.08 | 587.24 | NS | 7.503 | NS |
| Lu4, n = 2 | ||||||||||||||
| Case 1 | NS | 165/90 | 2.1 | 21.84 | 5369 | 150.2 | NS | 260 | 362 | NS | NS | 42 | NS | NS |
| Case 2 | 5 m | 166/100 | 2.7 | 28.24 | 4153 | 51 | NS | 132 | 367 | NS | 692.5e | 38 | NS | NS |
| Li5, n = 1 | NS | 180/100 | 1.45 | 65 | 8856 | 19 | NS | NS | 768 | NS | NS | 35.6 | NS | NS |
| Chen6, n = 1 | 3 y | 210/130 | 1.25 | NS | 5031 | 53 | NS | 392 | 516 | NS | Increased | 30.1 | NS | NS |
| Zhang7, n = 1 | 8 y | 201/120 | 1.76 | 52.74 | 14536 | NS | NS | 682 | 1015 | 0.10 | 475.5 | NS | NS | NS |
| Case | ECG | EMG | Muscle Biopsy | Adrenal Ultrasonography | Adrenal CT or MRI |
|---|---|---|---|---|---|
| Our hospital | |||||
| Case 1 | Flat T waves, obvious U waves, ST segment depression | Myo | Focal degeneration and necrosis of muscle fibers, inflammatory cell infiltration, with absence of vacuolar changes | No | Left adrenal adenoma 1.1 cm × 1.2 cm |
| Case 2 | ST segment depression | Myo | Not done | No | Right adrenal adenoma 1.0 cm × 1.1 cm |
| Case 3 | Flat T waves, visible U waves, ST segment depression | No | Not done | Left adrenal hypoechoic mass 1.5 cm | Right adrenal adenoma 1.1 cm × 2.0 cm |
| Hou2, n = 1 | No abnormality | Myo | Partial degeneration, necrosis and regeneration of muscle fibers, and macrophage response, interstitial edema, a small number of lymphocytes, mononuclear cell infiltration | Right adrenal hypoechoic mass 1.4 cm | Right adrenal mass 1 cm × 2 cm |
| Wang3, n = 1 | Flat T waves, obvious U waves, ST segment depression | No | Focal degeneration, necrosis and disintegration of muscle fibers, mononuclear cell infiltration, with absence of muscle regeneration | Not done | Left adrenal nodule 0.8 cm × 0.8 cm |
| Lu4, n = 2 | |||||
| Case 1 | Flat and inverted T waves, minor U waves, ST segment depression | Myo | Not done | No | Left adrenal adenoma 1.1 cm × 1.2 cm |
| Case 2 | ST depression | No | Not done | No | Left adrenal adenoma 1.1 cm × 1.2 cm |
| Li5, n = 1 | Flat T waves, obvious U waves, ST segment depression | Myo | Not done | No | Right adrenal mass 1.5 cm × 2.5 cm |
| Chen6, n = 1 | Flat T waves, obvious U waves, ST segment depression | Not done | Not done | Not done | Right adrenal adenoma 2.5 cm × 2.5 cm, mild hyperplasia of left adrenal gland |
| Zhang7, n = 1 | Left ventricular hypertrophy, prolonged Q-T interval | No | Not done | No | Short T1 and long T2 in right adrenal area 1.34 cm × 1.40 cm × 1.94 cm; MRI findings |
| Case | Postoperative Pathology | Main Pharmacological Treatments | Status After Pharmacological Therapy | Postoperative Status | Recurrence | ||||
|---|---|---|---|---|---|---|---|---|---|
| Time to Re-test* | CK, IU/l | Serum K+, mmol/l | CK, IU/l | Serum K+, mmol/l | Duration of Followup* | ||||
| Our hospital | |||||||||
| Case 1 | Adrenocortical adenoma | Steroidsg, potassium supplementation, antihypertensive therapy | 5 d | 762 | 2.9 | 65 | 4.5 | 2 y | Absent |
| Case 2 | Adrenocortical adenoma | Potassium supplementation, antihypertensive therapy | 7 d | 138 | 3.1 | 61 | 4.6 | 1 y | Absent |
| Case 3 | Adrenocortical adenoma | Potassium supplementation, antihypertensive therapy | 5 d | 152 | 2.9 | 44 | 4.1 | 6 m | Absent |
| Hou2, n = 1 | Adrenocortical adenoma | Potassium supplementation, antihypertensive therapy | 3 d | 597 | 3.0 | Normal | 4.0 | 3 y | Absent |
| Wang3, n = 1 | Adrenocortical adenoma | Potassium supplementation, antihypertensive therapy | 7 d | 127 | Normal | Normal | Normal | 6 m | Absent |
| Lu4, n = 2 | |||||||||
| Case 1 | Adrenocortical adenoma | Potassium supplementation, antihypertensive therapy | Not specified | Normal | 2.5 | 73 | 4.6 | 5 y | Absent |
| Case 2 | Adrenocortical adenoma | Not specified | Not specified | Not specified | Not specified | 56 | 4.3 | 6 m | Absent |
| Li5, n = 1 | Adrenocortical hyperplasia | Steroidsg, potassium supplementation, antihypertensive therapy | Not specified | Normal | > 3.0 | Not specified | Not specified | Not specified | Not specified |
| Chen6, n = 1 | Not doneh | Potassium supplementation, antihypertensive therapy | 7 d | Normal | Normal | Normal | Normal | 6 m | Absent |
| Zhang7, n = 1 | Adrenocortical adenoma | Potassium supplementation, antihypertensive therapy | Not specified | Normal | Normal | Normal | Normal | Not specified | Not specified |
↵* d: days; m: months; y: years.
↵a History of polydipsia and increased nocturia 6 months to 3 years prior to presentation;
↵b limb numbness at presentation;
↵c history of hypokalemia 2 years prior to presentation;
↵d supine position;
↵e normal cortisol levels;
↵f myoglobinuria by routine urinalysis;
↵g short-term use of methylprednisolone 80–120 mg/day;
↵h ablative therapy of adrenal tumors was performed.
NS: not specified; Myo: myogenic damage; No: no abnormalities. CK: creatine phosphokinase; CK-MB: creatine kinase isoenzyme; ALT: alanine aminotransferase; AST: aspartate aminotransferase; LDH: lactate dehydrogenase; PRA: plasma rennin activity.