TABLE 1.
References | Gender | Case (s) | POD to peak | Peak NH3 | Bowel decontamination | N2 scavengers | RRT | Outcome | Hypothesized etiology |
---|---|---|---|---|---|---|---|---|---|
(31) | F | 1 | 7 | 3,207 | L | NR | CAVHD | Died | Glutamine synthetase deficiency |
(19) | F | 1 | 35 | 535 | L, N | SB | IHD | Died | Glutamine synthetase deficiency |
(32) | M | 1 | 1 | 269 | L, N | AR,SB, SP | Hemoperfusion with charcoal + IHD | Survived | NR |
(1) | 4 | ||||||||
Patient 1 | NR | — | 29 | 5000 | L +/− N | NR | None | Died | Idiopathic |
Patient 2 | NR | — | 27 | 900 | L +/− N | SB, SP | None | Died | Idiopathic |
Patient 3 | NR | — | 5 | 3136 | None | NR | None | Died | Idiopathic |
Patient 4 | NR | — | 20 | 1800 | L +/− N | SB, SP | IHD | Survived | Idiopathic |
(33) | M | 1 | 22 | 338 | L, ME, R | AR, LC, SB, SP | CVVHD | Survived | Idiopathic |
(14) | F | 1 | 5 | >1,200 | L, R | NR | CVVHD | Died | Mycoplasma hominis |
(34) | F | 1 | 7 | 704 | L, R | NR | CVVHD | Died | Mycoplasma hominis |
(35) | 4 | ||||||||
Patient 1 | NR | — | NR | NR | L | SB, SP | CVVHD | Died | Idiopathic |
Patient 2 | NR | — | NR | NR | L | SB, SP | CVVHD | Died | Idiopathic |
Patient 3 | NR | — | NR | NR | L | SB, SP | CVVHD | Died | Idiopathic |
Patient 4 | NR | — | NR | NR | L | SB, SP | CVVHD | Survived | Idiopathic |
(27) | 3 | ||||||||
Patient 1 | M | — | 9 | 269 | L, N | AA, AR, SB, SP | CVVHD+IHD | Survived | Idiopathic |
Patient 2 | M | — | 12 | 330 | R | AR, LC | CVVHD | Died | Idiopathic |
Patient 3 | M | — | 9 | 475 | L, R, ME | AR, LC, SP | CVVHD, IHD | Survived | Idiopathic |
(11) | 5 | ||||||||
Patient 1 | M | — | 10 | 291 | L, R | LC, SB, SP | CVVHD | Died | Ureaplasma urealyticum infection |
Patient 2 | M | — | 10 | NR | NR | NR | NR | Died | Ureaplasma urealyticum infection |
Patient 3 | M | — | 12 | 549 | NR | NR | NR | Died | Ureaplasma urealyticum infection |
Patient 4 | M | — | 9 | NR | NR | NR | NR | Survived | Ureaplasma parvum infection |
Patient 5 | F | — | 10 | >200 | L, R | AR, LC, SB | IHD | Survived | Ureaplasma parvum infection |
(2) | 6 | — | |||||||
Patient 1 | M | — | — | 1,597 | L | NR | IHD | Died | Idiopathic |
Patient 2 | F | — | — | 479 | L, N | NR | CVVHD | Died | Idiopathic |
Patient 3 | M | — | — | 366 | L, N | AR, LC | IHD | Died | Idiopathic |
Patient 4 | M | — | — | 289 | L, ME, R | AR, LV, SP | IHD | Died | Idiopathic |
Patient 5 | F | — | — | 304 | L, ME, R | AR, LV, SP | IHD | Survived | Idiopathic |
Patient 6 | M | — | 11 | 374 | NR | NR | CVVHD | Died | Idiopathic |
(12) | F | 1 | 5 | 80 | NR | NR | NR | Survived | Ureaplasma |
(36) | M | 1 | 29 | 246 | L, ME, R | SP | NR | Survived | Ureaplasma |
(37) | F | 1 | 8 | 399 | L, R | SB | CVVHD/HD | Died | Idiopathic |
(18) | — | 6 | — | ||||||
Patient 1 | M | — | 18 | 312 | L, ME, R | AR, LC, SB | CVVHD | Survived | Idiopathic |
Patient 2 | M | — | 8 | 341 | L, ME, R | AR, LC, SB, SP | CVVHD + IHD | Survived | Idiopathic |
Patient 3 | M | — | 11 | 55 | L, ME, R | AR, LC, SB, SP | CVVHD | Died | Idiopathic |
Patient 4 | M | — | 45 | 189 | L, ME, R | AR, LC, SB, SP | CVVHD | Died | Idiopathic |
Patient 5 | M | — | 6 | 198 | L, ME, R | AR, LC, SB | IHD | Survived | Idiopathic |
(38) | M | 1 | 8 | 830 | L, R | NR | CRRT + HD | Died | CPS I inhibition by VPA |
(39) | M | 1 | 32 | 506 | L, R | SB, SP | CVVHD | Survived | NR |
(40) | — | 2 | — | ||||||
Patient 1 | F | — | 3 | 144 | L, R, ME | LC, SB | MARS | Survived | Idiopathic |
Patient 2 | F | — | 9 | 95 | L, R, ME | NR | MARS/ECMO/PP/RRT | Survived | Idiopathic |
AA, acetohydroxamic Acid; AR, arginine; CA, carbaglumic acid; CAVHD, continuous arteriovenous hemodialysis; CVVHD, continuous veno-venous hemodialysis; CPS I, carbamoyl phosphate synthase I; ECMO, extracorporeal membrane oxygenation; F, female; IHD, intermittent hemodialysis; L, lactulose; LC, levocarnitine; M, male; MARS, molecular adsorbent recirculating system; ME, metronidozole; N, neomycin; NR, not reported; PP, plasmapheresis; POD, Post-operative day; R, rifaximin; RRT, renal replacement therapy; SB, sodium benzoate; SP, sodium phenylacetate; VPA, valproic acid.