The Joint Annual Scientific Meetings of the Endocrine Society of Australia and the Society for Reproductive Biology 2018

The prevalence and risk factors for renal calcific disease in Multiple Endocrine Neoplasia Type 1-related primary hyperparathyroidism (#200)

James Burgess 1 , Venkat Parameswaran 1 2 , John Burgess 1 2
  1. University of Tasmania, Sandy Bay, TAS, Australia
  2. Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart

Background

Renal calcific disease (calculi and nephrocalcinosis) and renal impairment are potential sequelae of primary hyperparathyroidism (PHPT).  Patients with Multiple Endocrine Neoplasia Type 1 (MEN 1) typically develop PHPT in the second decade of life.  Management strategies for MEN 1 aim to treat PHPT at an early age to prevent complications including renal calcific disease (RCD) however, the prevalence and calcaemic thresholds for RCD development are unclear.

Objective

To determine the age-related prevalence and calcaemic thresholds RCD in MEN 1. 

Methods

Patients with an MEN 1 genotype evaluated by the RHH were assessed in a retrospective longitudinal cohort study.  The relationship between serum calcium [ionised (ICa); albumin corrected (CCa)] and RCD (on abdominal CT and ultrasound) was examined.

Results

Of 94 patients studied 28.7% manifest RCD, with renal calcification in up-to one quarter by age 30 (Table).

RCD Prevalence

(All Patients)

RCD Prevalence

(Patients stratified by serum calcium)

Age Range(yr)

Total

n=

Sex(M:F)

n=

RCD

n=(%)

ICA<1.30;CCa<2.60

Total Pts n= (%RCD)

ICa1.30-1.40;CCa2.60-2.80

Total Pts n= (%RCD)

ICa>2.80;CCa>2.80

Total Pts n= (%RCD)

0 -9.99

0

0:0

0(0.0)

0(0.0)

0(0.0)

0(0.0)

10 -19.99

16

7:9

0(0)

2(0.0)

5(0.0)

9(0.0)

20 -29.99

43

14:29

7(16.3)

1(0.0)

19(5.3)

23(26.1)

30 -39.99

50

21:29

7(17.0)

1(0.0)

18(5.6)

31(19.4)

40 -49.99

53

19:34

6(11.3)

0(0.0)

17(0.0)

36(16.7)

50 -59.99

38

12:26

10(26.3)

1(0.0)

9(33.3)

28(25.0)

60 -69.99

22

6:16

2(9.1)

1(0.0)

2(0.0)

19(10.5)

>=70

10

2:8

0(0.0)

1(0.0)

0(0.0)

9(0.0)

Total

94

38:56

27(28.7)

4(0.0)

27(14.8)

63(36.5)

 

Discussion/Conclusions

Hypoparathyroidism is recognized as an important complication of the subtotal total parathyroidectomy used for treating PHPT in MEN 1.  Concern regarding hypoparathyroidism is particularly important when contemplating parathyroidectomy in adolescents with MEN 1.  Our findings suggest patient age and degree of hypercalcaemia do influence RCD risk and this information could be used to inform parathyroidectomy timing.