Article Figures & Data
Figures
- Figure 1.
Biopsy specimen of striated muscle shows destruction of muscle fascicles and focal necrosis of muscle fibers, with infiltration of many histiocytes, lymphocytes, and neutrophils between striated muscle fascicles; and no vacuolar changes in the muscle fibers (a: H&E, original magnification ×100; b: H&E, original magnification ×400).
Tables
- Table 1.
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.
-
