Table 1 Results of individual studies. This table gives an overview of the characteristics of each study included in the review, and summarizes the main endpoints
From: Pain management strategies for neuropathic pain in Fabry disease - a systematic review
Study (N, sex) | Medication | Dose | Effectiveness recorded chronic pain reduction after any treatment period | Adverse events | |
|---|---|---|---|---|---|
| Â | Â | Â | As reported in study | As rated for this review | Â |
Monotherapy – primary endpoint | |||||
Patil [52] (N = 1, male) | Carbamazepine | Not reported | Some response | Partial pain relief | Not reported |
Politei [67] (N = 2, 1 male) | Carbamazepine | 600 mg/day | Good response | Complete pain relief (N = 1) | Not reported |
|  |  | ? | No response | No response (N = 1) |  |
Lim [48] (N = 1, male) | Carbamazepine (+ERT) | 600 mg/day | Condition stationary | Partial pain relief | Not reported |
Tümer [58] (N = 1, female) | Carbamazepine | 10 mg/kg/day (23 kg) | Pain resolved completely | Complete pain relief | Not reported |
Chaudhuri [60] (N = 1, male) | Carbamazepine | Not reported | Pain reasonably controlled | Partial pain relief | Not reported |
Mills [65] (N = 3, males) | Carbamazepine (+ERT) | Patient 1: 200 mg eod | 60 % improvement on questionnaire on double dose of ERT | Partial pain relief | Not reported |
Patient 2: 200Â mg/day | Subjective improvement (25Â % improvement on questionnaire on double dose of Fabrazyme) | Partial pain relief | |||
Patient 3: 200Â mg/day | Absence of breakthrough pain, questionnaire results improved by over 90Â % | Partial pain relief | |||
Yang [59] (N = 1, male) | Carbamazepine | 200-400 mg/day | Pain controlled | Partial pain relief | Not reported |
Asahi [61] (N = 1, male) | Carbamazepine | Not reported | Complete pain relief | Complete pain relief | Not reported |
Slee [55] (N = 1, male) | Carbamazepine | 600 mg/day | Pain controlled | Partial pain relief | Not reported |
Brady [62] (N = 1, male) | Carbamazepine | Not reported | Considerable relief from painful acroparesthesias | Partial pain relief | Discontinued due to drowsiness |
Shelley [63] (N = 1, male) | Carbamazepine | Not reported | Modest relief | Partial pain relief | Not reported |
Inagaki [46] (N = 1, male) | Carbamazepine | 300-500 mg/day | Partially helpful in alleviating shooting pain | Partial pain relief | Not reported |
Filling-Katz [43] (N = 7, males) | Carbamazepine | 0.8-15.9 mg/kg/day | Partial amelioration in 3 patients (43 %), complete pain relief in 2 patients (29 %), no benefit in 2 patients (29 %) | Complete pain relief (N = 2) | Autonomic complications in 2/7 patients (27 %), discontinuation in 1 patient. |
Partial pain relief (N = 3) | |||||
No effect (N = 2) | |||||
Tomé [57]+ Lenoir [47, 57] (same patient) (N = 1, male) | Carbamazepine | 600 mg/day | Pain attacks almost disappeared | Partial pain relief | Not reported |
Shibasaki [54] (N = 1, male) | Carbamazepine | 200 mg/day | Pain suppressed | Partial pain relief | No side effects |
Gordon [44] (N = 1, male) | Phenytoin | 5 mg/kg/day | Ineffective | No effect | Discontinuation |
Paira [50] (N = 1, male) | Phenytoin | 300 mg/day | Pain controlled | Partial pain relief | Not reported |
Filling-Katz [43] (N = 5, males) | Phenytoin | Therapeutic dosage (mean 13 mg/dl) | Inadequate pain control (5/5 patients) | No effect (N = 5) | Not reported |
Sheth [64] (N = 2, 1 male) | Phenytoin | Not reported | Pain controlled in both patients | Partial pain relief (N = 2) | Not reported |
Duperrat [66] (N = 1, male) | Phenytoin | 200 mg/day | Pain completely disappeared | Complete pain relief | Not reported |
Lockman [49] (N = 8, 7 males) | Phenytoin | 300 mg/day or 4-6 mg/kg/day | Average pain relief score of 2,7 (complete pain relief, p < 0,001 when compared to ASA or placebo) | Partial pain relief (N = 8) | Dizziness, drowsiness and headache in 1 patient |
Shibasaki [54] (N = 1, male) | Phenytoin | 200 mg/day | Ameliorated pain | Partial pain relief | Not reported |
Park [51] (N = 1, male) | Gabapentin | Not reported | Pain persisted | No effect | Not reported |
Gabapentin | Average daily dose 917 mg (range 100-1200 mg) | Average pain scores decreased from 5.0 (range 4-6) to 3.7 (range 3-6) with an intraindividual reduction of 1.3 (range 0-3) (p = 0,22) | Partial pain relief (N = 6) | Generally well tolerated. Vertigo and blurred speech in 1 patient | |
Inagaki [46] + Inagaki [45] (same patients) (N = 2, males) | Neurotropin | 4 units (crisis) | Pain almost completely eliminated | Partial pain relief (N = 2) | Not reported |
Wise [21] (N = 2, males) | Pethidine | 500-700 mg/day i.m. | Pain reasonably controlled | Partial pain relief (N = 2) | Not reported |
Politei [67] (N = 2) | Lidocaine | 2 mg/kg i.v. | Quick pain relief in pain crisis | Partial pain relief (N = 2) |  |
Combination therapies – primary endpoint | |||||
Park [51] (N = 1, male) | Phenytoin + Carbamazepine | 200 mg/day + 400 mg/day | Mild-moderate pain persisted | Partial pain relief | Not reported |
Phenytoin + Carbamazepine (+ERT) | 100 mg/day + 200 mg/day | Pain decreased | Partial pain relief | ||
Gordon [44] (N = 1, male) | Morphine + Amitriptyline (crisis) | 0.06 mg/kg IV push, 0.02 mg/kg/hr IV; 0.25 mg/kg p.o. at bedtime | Pain control within hour, remaining pain free overnight | Complete pain relief | Not reported |
Inagaki [46] + Inagaki [45] (same patients) (N = 2, males) | Neurotropin + Carbamazepine | 12-16 units/day + 600 mg/day | Pain disappeared almost completely | Partial pain relief (N = 2) | Not reported |
Monotherapy - Secondary endpoints | |||||
Gordon [44] (N = 1, male) | Carbamazepine | ? | Reduced frequency and duration of crises (to 3-4 times annually) | Partial pain relief (N = 1) | Not reported |
Spence [56] (N = 8, males) | Phenytoin | 100-400 mg/day | Significant reduction in frequency of painful crises in 7/8 patients | Partial pain relief (N = 8) | Not reported |
Unknown (N = 1) | |||||