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.