Table 2.
Characteristics of enteral tube feeding regimens in the study population (N = 190).
| Inherited metabolic disease | Enteral tube feeding |
Gastrostomy |
|||
|---|---|---|---|---|---|
| n (%) | Median [P25-P75] age (in years) | n (%) | Median [P25-P75] age (in years) | ||
| Organic aciduria | Disease | ||||
| All OAs (n = 60) | 40 (66.7) | 0.2 [0.1–1.0] | 14 (23.3) | 2.3 [1.4–4.5] | |
| MA (n = 29) | 23 (79.3) | 0.1 [0.1–0.5] | 7 (24.1) | 2.6 [1.5–4.5] | |
| PA (n = 17) | 16 (94.1) | 0.3 [0.1–1.6] | 6 (35.3) | 2.3 [1.3–4.6] | |
| IVA (n = 14) | 1 (7.1) | 0.3 [0.3–0.3] | 1 (7.1) | 1.9 [1.9–1.9] | |
| Age at diagnosis | |||||
| <1 month (n = 44) | 28 (63.6) | 0.1 [0.1–0.3] | 10 (23) | 2.1 [1.5–4.5] | |
| >1 month (n = 16) | 12 (75.0) | 1.0 [0.3–3.7] | 4 (25) | 2.6 [1.3–6.6] | |
| Urea cycle disorder | Disease | ||||
| All UCDs (n = 55) | 12 (21.8) | 1.1 [0.7–2.0] | 6 (10.9) | 2.4 [2.3–3.1] | |
| NAGS (n = 3) | 0 (0) | – | – | – | |
| CPS (n = 5) | 1 (20.0) | 1.0 [1.0–1.0] | 1 (20.0) | 2.4 [2.4–2.4] | |
| OTC (n = 14) | 6 (42.9) | 1.6 [0.6–2.1] | 2 (14.3) | 2.5 [1.9–3.1] | |
| ASS (n = 9) | 3 (33.3) | 0.7 [0.7–1.2] | 1 (11.1) | 2.3 [2.3–2.3] | |
| ASL (n = 19) | 2 (11) | 1.7 [0.7–2.6] | 2 (10.5) | 3.1 [2.5–3.8] | |
| Arginase (n = 3) | 0 (0) | – | – | – | |
| CA (n = 2) | 0 (0) | – | – | – | |
| Age at diagnosis | |||||
| <1 month (n = 41) | 8 (19.5) | 0.9 [0.7–1.6] | 5 (12) | 2.5 [2.4–3.1] | |
| >1 month (n = 14) | 4 (28.6) | 1.7 [0.9–5.2] | 1 (7) | 2.3 [2.3–2.3] | |
| Maple syrup urine disease | Disease | ||||
| All MSUDs (n = 32) | 9 (28.1) | 1.1 [0.4–1.7] | 2 (6.3) | 2.7 [0.8–4.6] | |
| Age at diagnosis | |||||
| <1 month (n = 30) | 8 (26.7) | 0.8 [0.3–1.5] | 1 (3.3) | 0.8 [0.8–0.8] | |
| >1 month (n = 2) | 1 (50.0) | 3.5 [3.5–3.5] | 1 (50.0) | 4.6 [4.6–4.6] | |
| Glycogen storage disease | Disease | ||||
| All GSDs (n = 26) | 23 (88.5) | 0.3 [0.1–0.9] | 8 (30.8) | 1.4 [0.4–2.3] | |
| 0 (n = 3) | 3 (100) | 0.9 [0.6–0.9] | 0 (0) | – | |
| Ia (n = 11) | 10 (90.9) | 0.2 [0.1–0.4] | 7 (63.6) | 1.5 [0.5–3.2] | |
| Ib (n = 8) | 8 (100) | 0.2 [0.1–1.2] | 1 (12.5) | 0.3 [0.3–0.3] | |
| III (n = 4) | 2 (50.0) | 0.6 [0.2–1.1] | 0 (0) | – | |
| Age at diagnosis | |||||
| <1 month (n = 10) | 10 (100) | 0.1 [0.1–0.1] | 4 (40) | 0.4 [0.3–1.8] | |
| >1 month (n = 16) | 13 (81.3) | 0.9 [0.4–1.0] | 4 (25) | 1.5 [1.4–3.4] | |
| Fatty acid oxidation deficiency | Disease | ||||
| All FAODs (n = 17) | 14 (82.4) | 0.4 [0.1–0.9] | 5 (29.4) | 1.5 [1.4–1.5] | |
| CPT2 (n = 2) | 1 (50.0) | 0.1 [0.1–0.1] | 1 (50.0) | 1.3 [1.3–1.3] | |
| VLCAD (n = 4) | 4 (100) | 0.3 [0.1–0.9] | 2 (50.0) | 1.4 [1.4–1.5] | |
| LCHAD (n = 8) | 7 (87.5) | 0.4 [0.1–0.8] | 2 (25.0) | 2.3 [1.5–3.0] | |
| MCAD (n = 2) | 2 (100) | 0.9 [0.9–1.0] | 0 (0) | – | |
| SCAD (n = 1) | 0 (0) | – | – | – | |
| Age at diagnosis | |||||
| <1 month (n = 7) | 6 (85.7) | 0.1 [0.1–0.1] | 3 (43) | 1.4 [1.3–1.5] | |
| >1 month (n = 10) | 8 (80.0) | 0.7 [0.4–0.9] | 2 (20) | 2.3 [1.5–3.0] | |
| ALL PATIENTS (N = 190) | 98 (51.6) | 0.4 [0.1–1.1] | 35 (18.4) | 1.9 [1.4–3.1] | |
Abbreviations: ASL, argininosuccinate lyase deficiency; ASS, argininosuccinate synthetase deficiency; CA, carbonic anhydrase deficiency; CPS, carbamylphosphate synthetase deficiency; FAOD, fatty acid oxidation deficiency; GSD, glycogen storage disease; IVA, isovaleric acidemia; MA, methylmalonic acidemia; MSUD, maple syrup urine disease; NAGS, N-acetylglutamate synthase deficiency; OA, organic aciduria; OTC, ornithine transcarbamylase deficiency; P25-P75, 25th and 75th percentiles; PA, propionic acidemia; UCD, urea cycle disorder.
Gastrostomy feeding was always preceded by NGT feeding, thus gastrostomy patients are also included in ETF patients. For example, among 26 GSD patients, 23 had ETF and 8 of them had gastrostomy.