Table 6 Summary of included studies for HNF1B-diabetes

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

HNF1B-diabetes Cohort studies or case series (N  = 293 (132 men, 161 women)

Study ID

Number of participants

Sex (M/F)

Variant (intra-genic/ deletion)

Age (years)

Duration of diabetes (years)

Treatment before/after the genetic diagnosis

Intervention

Response

    

Diagnosis

Last appraisal

 

Before

After

  

Dubois- Laforgue et al.47

Adults

159

73/86

75/84

28

[20–37]

45

[3–56]

12

[5.5–22.5]

68 INS

47 OAD

25 LSa

111 INS

22 OAD

7 LS

SU or repaglinide

(n = 51b)

29 responders (57%):

HbA1c 7.1% [5.5– 12.1]

→ 6.1% [4.4–7.0], p < 10−4,

during treatment for 5 [3–9] years

         

INS replaced with SU (n = 10)

Three could convert

cColclough et al.46

50

26/24

26/24

18 [13–27]

 

3 [1–8]

23 INS

4 INS + OAD

   
 

18

7/11

4/14

17

[12–25]

 

3.5 [1–9]

10 INS

4 INS + OAD

   

Warncke et al.48

Minors

35

16/19

NA

mostly deletions

13.5

[11.2–15.7]

13.8

[12.4–16.3]

<1

20 INS

3 INS + OAD

4 OAD, 8 LS

   

Ng et al.49

10

6/4

9/1

31.5

[16–39]

55

(18–62)

18.5

(4–47)

4 INS,

2 INS + OAD

2 OAD

1 LS

6 INS

2 INS + OAD

1 OAD (MET)

Trial of SU for 5 patients on INS

none weaned off INS

Kettunen et al.50

11

2/9

4/7

    

9 INS

2 LS

  

Case reports combined

 d(see foot-notes)

10

2/8

5/5

16.5

[15.8–18.3]

(N = 8)

22.5

[18.3–33.5]

3 (0–22)

5 INS

1 OAD (MET)

3 INS

1 INS + OAD

2 OAD (MET)

1 GLPRA

1 LS

Duration of non-INS response

(HbA1c < 6.5–7%):

at least 1 year on GLP1RA (Terakawa)

6 years on SU+DPP4i (Carrillo)

at least 1 year on MET (Tao, Thirumalai)

 
  1. Data are shown as median (range) or [interquartile range] or mean ± SD; M/F number of men/women, NA not available, INS insulin, OAD oral antidiabetic agent, SU sulphonylurea, MET metformin, GLP1RA GLP1-receptor agonist, DPP4i DPP4 inhibitor, TZD thiazolidinedione, AGI Alpha-glucosidase inhibitor.
  2. aAt diagnosis.
  3. bThese were included among the cohort of 159 patients (diabetes duration 0.75 [0–5.25] years), with response defined as HbA1c < 7%.
  4. cGenetic testing laboratory serving clients globally; two subcohorts based on clinical suspicion of HNF1B disease (N = 50) or MODY (N = 18).
  5. dRoehlen (2018), Terakawa (2020), Carrillo (2017), Tao (2020), Aydın (2022), Ren (2021), Thirumalai (2013), Mateus (2020), with PMIDs: 30032214, 32871938, 28680642, 32756155, 35899569, NA (https://doi.org/10.1007/s13410-020-00904-6), 23480312, 32864159.
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