Table 1 Summary of Clinical Characteristics of Reported Pediatric Cases
Patient [Ref] | Gender/ age | Presentations | BP (mmHg) | Renin (range) | Aldosterone | SNa (mEq/L) | SK (mEq/L) | SHCO3- (mEq/L) | Proteinuria | Organs involvement | Treatment | Outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 [2] | F/ 2y9m | Polydipsia, polyuria Presyncope | 215/156 | Low | Elevated | 129 | 3 | 27 | 700 mg/day | CNSa Kidney | IV β blocker, oral β blocker, CCBb, ACEIc dPTA | Recovery |
2 [2] | M/ 2y3m | Polydipsia | 142/92 | NA | Elevated | 122 | 3.9 | 25.9 | 3200 mg/day | Heart Kidney | IV β blocker, oral β blocker, CCB, ACEI PTA | Recovery |
3 [2] | M/ 2y | Polydipsia, polyuria Restlessness | 220/150 | Elevated | Elevated | 125 | 3.2 | 27.2 | 5300 mg/day | Heart Kidney | IV β blocker, oral β blocker, CCB, ACEI PTA | Recovery |
5 [9] | M/ 1y6m | Seizure, hemorrhagic and ischemic stroke | 210/160 | 172 ng/ml/min (3~11) | 91 ng/dl (4~16) | 120 | 2.1 | NAe | NA | CNS Heart Kidney | Nitroprusside, IV β blocker, oral CCB Aorto-renal bypass | Hypertension |
4 [14] | F/ 1y3m | Polyuria, polydipsia | 190/120 | 24 ng/ml/hr. (1~4.5) | 8 nmol/l (0.1~0.8) | 122 | 2.4 | 29.5 | 1800 mg/day | Heart Kidney | ACEI, β blocker, CCB, spironolactone PTA | Recovery |
6 [15] | M/ 7y | Polydipsia, polyuria | 210/120 | NA | NA | 114 | 2.4 | NA | NA | Retina Kidney | CCB, α1 blocker Nephrectomy | Recovery |
7 [16] | M/ 2y9m | Polydipsia, polyuria Seizure | 160/120 | 80.44 ng/ml/hr. (0.2~2.8) | 6861 pg/ml (10~160) | 118 | 1.9 | NA | NA | CNS Kidney | CCB, desmopressin ACEI Nephrectomy | Recovery |
8 [16] | F/ 1y4m | Polyuria, polydipsia | 140/90 | NA | NA | 131 | 2.6 | NA | NA | Kidney | CCB ACEI Nephrectomy | Hypertension |
9 [17] | M/ 9y | Polyuria, polydipsia Seizure | 156/120 | NA | NA | 124 | 3.2 | 34 | NA | CNS Retina Kidney | Nitroprusside ACEI, CCB PTA with stenting | Hypertension |
10 [18] | M/ 1y7m | Polyuria Polydipsia Seizure | 248/150 | 137 ng/ml/min (3~11) | 743 ng/dl (7~93) | 128 | 3.2 | 24 | NA | CNS Heart Kidney | Nitroprusside, ACEI PTA with stent | Hypertension |
11 [19] | M/ 5y | Seizure | 236/132 | 21.06 ng/ml/hr. (1.3~3.9) | 1172 ng/dl (1~16) | 112 | 3.2 | 33.4 | fUP/UCr 6.84 | CNS Kidney | β blocker, CCB, hydralazine | Proteinuria Hypertension |
12 [19] | M/ 8y | Polydipsia, polyuria Seizure | 184/110 | 32.8 ng/ml/hr. (1.3~3.9) | 1436 ng/dl (1~16) | 127 | 3.1 | 27.2 | UP/UCr 3.91 | CNS Retina Kidney | β blocker, CCB, hydralazine PTA | Recovery |
13 [19] | M/ 12y | Polydipsia, polyuria Seizure | 244/166 | 25.04 ng/ml/hr. (1.3~3.9) | 1358 ng/dl (1~16) | 126 | 3.2 | 32.2 | UP/UCr 4.36 | CNS Retina Kidney | β blocker, CCB, hydralazine PTA with stenting | Hypertension |
14 [20] | M/ 2y | Polydipsia, polyuria | NA | 2537 ng/dl | 31.6 ng/dl | 124 | 2.8 | NA | 1230 mg/day | Heart Kidney | Hydralazine Angioplasty with patch | Recovery |
15 [20] | F/ 2y | Polydipsia, polyuria | NA | 76.5 ng/dl | 48.1 ng/dl | 128 | 2.7 | NA | 2400 mg/day | Heart Retina Kidney | CCB, β blockers, hydralazine Angioplasty with patch | Recovery |
Our case | M/ 4y | Polyuria, polydipsia | 230/120 | 174.5 ng/dl | 9.26 ng/dl | 124 | 2.4 | 34.5 | 55 mg/m2/hr | Kidney | IV CCB, Oral ACEI Nephrectomy | Recovery |