Table 8 Summary of included larger case series studies for SLC19A2-diabetes (TRMA syndrome)

From: Precision treatment of beta-cell monogenic diabetes: a systematic review

SLC19A2-diabetes, case series

Number of patients, n (males, %)

Age at diabetes diagnosis, median (years)

Age at Thiamine start, median (years)

Thiamine dose (mg/day), median

Current age, median (years)

HbA1c (%), median

Outcome, if specified

Clear response to Thiamine Yes, n (%)

Pre

Post, latest

Habeb et al.118

15 (NR)a

1.4 (Range, 0.1–3.5)

2.0 (IQR, 1.0–7.4)

NR (Range Max, 25–300)

14 (Range, 3.7–31.5)

9.0 (Range, 6.8–11.9)

7.8 (Range, 5.2–9.1)

27 % insulin-independent, n = 4

11 (73%)b

47% Reduced dose or HbA1c, n = 7

27% No response, n = 4

Ricketts et al.119

13 (38%)c

2.2 (Range, 0.5–12)

NR

50 (Range, 25–150)

9 (Range, 2–30)

NR

NR

15 % insulin-independent, n = 2

NR

Warncke et al.120

23 (52%)d

1.4 (IQR, 0.8–3.6)

5.9 (IQR, 2.4–12.4)

Initial, 100 (IQR 80–200)

14.3 (IQR, 8.1‒17.5)

NR

6.9 (IQR, 6.1‒7.9)

13% Insulin-independent, n = 3

NR

Current, 200 (IQR, 100–300)

  1. aIndividual follow-up data of the total 32 patients of the study. Includes follow-up data of seven patients previously reported. .
  2. bClear response was defined when there was a decrease in daily insulin dose of ≥10% or in HbA1c of ≥1%.
  3. cIncludes follow-up data of six patients previously reported. .8105295).
  4. dIndividual data not available. n = 18 (78%) of the patients had genetic confirmation of TRMA.
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