The cumulative incidence and trends of rare diseases in South Korea: a nationwide study of the administrative data from the National Health Insurance Service database from 2011–2015

Lim, Sung-Shil; Lee, Wanhyung; Kim, Yeong-Kwang; Kim, Jihyun; Park, Jong Heon; Park, Bo Ram; Yoon, Jin-Ha

doi:10.1186/s13023-019-1032-6

Orphanet Journal of Rare Diseases

Table 1 Average annual cumulative incidence/10,000,000 and trend of annual cumulative incidence of rare diseases in the register of the co-payment assistance policy according to KCD codes from 2011 to 2015

From: The cumulative incidence and trends of rare diseases in South Korea: a nationwide study of the administrative data from the National Health Insurance Service database from 2011–2015

KCD	Average annual cumulative incidence^a	Annual trend (β)^b	p-value
A81	0.31	−0.02	0.898
A81.0	6.48	0.71	0.203
A81.1	0.04	0.04	0.182
A81.2	0.74	−0.16	0.063
A81.8	0.08	0.02	0.646
A81.9	0.12	0.02	0.654
B45	0.35	0.08	0.322
B45.0	7.46	0.37	0.353
B45.1	3.69	−0.10	0.736
B45.2	0.16	−0.08	0.138
B45.3	0.08	−0.02	0.629
B45.7	0.15	0.08	0.141
B45.8	0.15	0.08	0.142
B45.9	1.16	0.23	0.102
D35.2	562.81	12.94	0.366
D55.0	0.86	− 0.24	0.268
D55.2	0.19	0.02	0.818
D56	0.00	.	.
D56.0	0.08	0.02	0.639
D56.1	1.86	0.16	0.491
D56.3	0.19	−0.02	0.704
D56.4	0.08	0.02	0.639
D56.8	0.15	0.08	0.142
D56.9	1.24	0.19	0.430
D59.5	5.82	0.90	0.107
D60	0.35	0.09	0.366
D60.0	0.19	−0.06	0.211
D60.1	0.12	0.02	0.647
D60.8	0.62	0.09	0.701
D60.9	5.86	0.54	0.403
D61.0	3.74	−0.43	0.133
D61.2	0.58	0.10	0.487
D61.3	18.52	1.48	0.090
D61.8	2.53	−0.05	0.803
D61.9	123.60	1.69	0.612
D64.4	0.08	−0.02	0.629
D69.1	3.32	−1.17	0.143
D69.30	5.60	−0.21	0.777
D69.6	99.82	−8.77*	0.046
D70	41.74	−3.29*	0.044
D71	1.72	−0.48	0.236
D76.1	21.40	1.36*	0.012
D76.3	9.39	1.03	0.374
D80	0.16	−0.08	0.305
D80.0	1.01	0.33	0.465
D80.1	1.40	0.17	0.740
D80.2	1.64	−0.34	0.304
D80.3	5.11	1.42*	0.033
D80.4	0.04	0.04	0.182
D80.5	0.12	0.06	0.559
D80.6	0.04	−0.02	0.559
D80.7	0.08	0.08	0.182
D80.8	0.08	0.00	0.984
D80.9	0.23	0.04	0.647
D81	0.00	.	.
D81.1	0.08	0.04	0.311
D81.2	0.04	0.02	0.559
D81.8	0.04	−0.04	0.182
D81.9	0.23	0.00	0.984
D82	0.00	.	.
D82.0	0.31	0.04	0.660
D82.1	2.13	0.65	0.140
D82.3	0.08	0.00	0.984
D82.4	0.31	−0.08	0.322
D82.8	0.04	0.04	0.182
D83	0.00	.	.
D83.0	0.12	0.00	0.996
D83.9	0.31	0.06	0.499
D84	0.04	0.02	0.559
D84.0	0.00	.	.
D84.1	1.32	−0.07	0.752
D84.8	0.08	0.00	0.984
D84.9	0.78	−0.04	0.568
D86	3.81	−0.35	0.282
D86.0	22.89	3.72*	0.003
D86.1	12.46	1.64*	0.027
D86.2	8.96	1.35*	0.017
D86.3	4.61	0.87	0.219
D86.8	4.04	−0.06	0.885
D86.9	26.68	1.56	0.133
E22.0	28.45	3.00	0.318
E23.0	35.21	−0.14	0.958
E24.0	3.84	0.48	0.508
E24.1	0.08	0.00	0.984
E24.3	0.16	−0.04	0.692
E25	0.82	0.07	0.575
E25.0	12.88	1.95	0.413
E25.8	0.19	0.06	0.223
E25.9	1.01	0.27	0.482
E27.1	6.64	0.27	0.766
E27.2	1.86	0.12	0.389
E27.4	121.76	9.85	0.072
E34.8	1.09	−0.05	0.787
E55.0	11.05	−1.64	0.290
E70	0.00	.	.
E70.0	2.17	0.16	0.726
E70.1	2.29	0.08	0.601
E70.2	0.31	−0.22	0.076
E70.3	3.15	−0.39	0.345
E70.8	0.04	−0.04	0.182
E70.9	0.04	0.02	0.559
E71	0.23	0.12	0.559
E71.0	0.31	0.00	0.993
E71.1	2.02	0.18	0.274
E71.2	0.58	0.02	0.904
E71.3	4.00	0.33	0.437
E72	0.23	0.04	0.707
E72.0	1.79	−0.01	0.945
E72.1	1.75	0.26	0.450
E72.2	2.75	0.66	0.067
E72.3	0.31	0.00	0.964
E72.4	0.27	0.08	0.340
E72.5	0.51	−0.14	0.156
E72.8	0.50	0.09	0.379
E72.9	0.16	−0.02	0.539
E73.0	0.00	.	.
E73.1	0.04	0.02	0.559
E73.8	0.08	0.00	1.000
E73.9	0.23	−0.04	0.520
E74	0.12	0.08	0.183
E74.0	4.23	0.81	0.133
E74.1	0.00	.	.
E74.2	3.08	−0.50*	0.027
E74.3	0.16	0.04	0.698
E74.4	0.16	−0.06	0.314
E74.8	1.32	−0.24	0.076
E74.9	0.00	.	.
E75.0	0.19	0.00	0.985
E75.1	0.08	0.08	0.182
E75.2	5.16	1.14	0.161
E75.4	0.23	0.08	0.315
E75.5	2.13	0.46*	0.009
E76	0.00	.	.
E76.0	0.19	0.04	0.677
E76.1	1.13	−0.04	0.871
E76.2	0.62	−0.06	0.672
E76.3	0.43	0.21	0.071
E76.8	0.00	.	.
E76.9	0.00	.	.
E77	0.04	0.00	1.000
E77.0	0.27	0.12	0.547
E77.1	0.00	.	.
E77.9	0.00	.	.
E79.1	0.23	0.00	0.995
E80.2	1.28	−0.14	0.256
E83.0	16.61	−1.24	0.120
E83.3	5.98	0.86	0.282
E84	0.19	0.13	0.119
E84.0	0.23	−0.04	0.173
E84.1	0.08	−0.06	0.057
E84.9	0.19	−0.04	0.436
E85	1.98	0.12	0.685
E85.0	0.31	0.12	0.083
E85.2	0.19	0.15	0.161
E85.3	0.47	0.07	0.498
E85.4	5.00	0.77	0.084
E85.8	2.37	0.18	0.218
E85.9	16.11	1.49	0.112
F84.2	4.58	0.21	0.482
G10	5.74	1.14	0.065
G11	0.81	0.17	0.478
G11.0	0.66	−0.22	0.215
G11.1	10.17	1.43	0.266
G11.2	38.45	5.61	0.051
G11.3	0.66	−0.20	0.129
G11.4	10.45	0.60	0.494
G11.8	1.63	0.26*	0.030
G11.9	36.38	3.33	0.107
G12	0.97	0.19	0.189
G12.0	0.86	0.00	0.915
G12.1	9.38	2.32	0.208
G12.2	4.01	−0.77	0.367
G12.20	1.86	0.11	0.479
G12.21	55.69	7.32	0.287
G12.22	2.48	0.34	0.473
G12.23	5.83	0.09	0.905
G12.24	1.24	0.26	0.266
G12.8	3.03	−0.27	0.138
G12.9	6.06	0.46	0.662
G13	0.24	−0.20	0.162
G13.0	1.36	−0.30	0.242
G13.1	6.87	4.54*	0.013
G13.2	0.19	0.04	0.464
G13.8	2.88	−0.41	0.259
G20	2064.86	140.88*	0.029
G23.1	27.21	−0.79	0.850
G31.81	1.32	0.21	0.385
G35	45.14	−2.04	0.375
G40.4	24.10^c	.	.
G40.40	52.65^c	.	.
G40.41	33.90^c	.	.
G41	8.74	0.18	0.683
G41.0	8.89	0.91	0.372
G41.1	1.32	0.42*	0.043
G41.2	13.85	2.08*	0.013
G41.8	15.68	2.22*	0.037
G41.9	124.11	15.76*	0.001
G51.2	0.19	−0.04	0.436
G56.4	36.98	−5.16*	0.015
G60.0	30.34	−2.22	0.166
G61	2.29	−0.17	0.575
G61.0	123.72	12.43*	0.021
G61.1	0.08	−0.02	0.629
G61.8	15.62	0.30	0.549
G61.9	8.50	0.73	0.408
G63.0	1.94	0.03	0.882
G70.0	137.88	14.27	0.150
G70.1	0.08	−0.02	0.637
G70.2	0.08	0.00	0.996
G71	2.72	0.31	0.548
G71.0	40.04	2.65	0.567
G71.1	33.29	0.95	0.389
G71.2	6.44	0.82	0.431
G71.3	12.33	−1.25	0.141
G71.8	0.81	0.25	0.288
G71.9	2.32	0.88	0.084
G90.8	2.68	0.06	0.839
G95.0	46.81	−1.70*	0.030
H35.31	1359.61	103.62	0.121
H35.51	163.56	1.17	0.869
H35.58	6.33	0.78*	0.016
I27.0	40.80	−3.17	0.204
I27.8	10.09	1.13	0.437
I42.0	521.39	44.36*	0.037
I42.1	42.31	4.15	0.139
I42.2	322.48	34.75	0.058
I42.3	1.09	0.11	0.489
I42.4	3.26	0.39	0.373
I42.5	7.10	0.99*	0.007
I67.5	261.73	0.38	0.888
I73.1	52.76	−5.97	0.080
I78.0	1.40	−0.39	0.203
I82.0	7.32	−1.02	0.104
J84.0	4.79	−0.86	0.135
J84.18	311.03	19.79*	0.006
K50	32.85	2.30	0.312
K50.0	51.57	6.73	0.262
K50.1	53.85	8.29	0.223
K50.8	35.33	6.61	0.126
K50.9	183.98	31.23	0.065
K51	52.66	−0.32	0.944
K51.0	67.17	9.06	0.218
K51.2	181.53	27.31*	0.019
K51.3	54.01	7.81	0.110
K51.4	0.19	0.02	0.732
K51.5	8.14	2.15	0.099
K51.8	63.29	8.08	0.163
K51.9	373.46	79.92	0.093
K74.3	68.32	6.72*	0.008
K75.4	104.26	7.90*	0.036
L10.0	13.94	1.14	0.246
L10.2	7.18	0.43	0.286
L12.0	50.83	7.27*	0.026
L12.1	0.46	0.15	0.082
L12.3	1.52	−0.14	0.555
M07.20	2.06	0.38*	0.010
M07.28	0.39	0.13	0.192
M08.0	4.58	0.68	0.575
M08.1	0.47	0.11	0.085
M08.2	0.27	0.04	0.717
M08.3	0.39	0.02	0.881
M30.0	7.08	−0.66	0.159
M30.1	9.75	0.66	0.313
M30.2	0.00	.	.
M31.0	2.72	0.35	0.266
M31.1	7.26	0.29	0.370
M31.2	0.00	.	.
M31.3	8.89	1.02	0.114
M31.4	26.14	1.38	0.378
M31.7	9.26	2.61*	0.009
M32.1	13.13	3.76	0.506
M32.10	4.76	2.21	0.286
M32.12	2.48	0.59	0.333
M32.13	46.43	18.53	0.341
M32.15	20.27	5.24	0.306
M32.19	46.45	16.72	0.320
M32.8	11.48	1.77	0.506
M32.9	282.52	72.81	0.355
M33	0.66	−0.02	0.858
M33.0	2.83	0.26	0.301
M33.1	22.36	2.58	0.198
M33.2	19.88	1.95	0.265
M33.9	6.25	0.80	0.485
M34.0	18.01	1.96	0.355
M34.1	3.61	0.35	0.434
M34.8	10.21	1.22	0.332
M34.9	57.97	7.94	0.379
M35.0	241.86	16.04*	0.036
M35.1	25.04	2.21	0.406
M35.2	265.39	−14.30	0.221
M35.3	56.31	7.45*	0.022
M35.4	1.13	−0.03	0.872
M35.5	0.08	0.02	0.646
M35.6	0.23	− 0.14	0.068
M35.7	0.47	0.06	0.621
M45	75.30	1.51	0.869
M61.1	0.51	−0.16	0.228
M88	0.23	0.02	0.858
M88.0	0.19	−0.04	0.436
M88.8	0.04	0.04	0.182
M88.9	0.16	0.00	0.992
M89.0	13.45	−3.51	0.131
M94.1	1.09	0.05	0.695
N25.1	61.63	−23.97*	0.006
P22.0	519.43	85.09*	0.001
Q03.1	4.86	0.01	0.966
Q04.3	2.60	0.08	0.732
Q04.6	8.16	−0.14	0.649
Q05	1.32	0.07	0.807
Q05.0	0.12	−0.10*	0.047
Q05.1	0.04	0.00	1.000
Q05.2	0.66	0.23	0.059
Q05.3	0.23	−0.10	0.273
Q05.4	0.39	0.04	0.814
Q05.5	0.12	0.00	0.984
Q05.6	0.16	−0.06	0.314
Q05.7	1.75	−0.11	0.477
Q05.8	1.01	0.03	0.825
Q05.9	41.94	3.03	0.163
Q06.2	0.51	−0.08	0.472
Q07.0	21.11	−0.27	0.718
Q20.0	1.75	− 0.07	0.809
Q20.1	22.26	0.36	0.548
Q20.2	0.62	0.15	0.312
Q20.4	19.02	5.58	0.127
Q21.8	3.22	0.17	0.681
Q22.0	17.12	1.84	0.086
Q22.6	1.32	0.32*	0.007
Q23	0.43	0.00	0.988
Q23.0	14.37	1.09*	0.039
Q23.1	96.01	16.07*	0.011
Q23.2	1.79	0.21	0.411
Q23.3	18.17	1.97	0.097
Q23.4	2.10	0.12	0.542
Q23.8	1.94	0.17	0.538
Q23.9	4.80	1.98	0.117
Q24.5	71.33	1.77	0.748
Q25.5	9.59	0.56	0.583
Q26.0	0.31	−0.04	0.790
Q26.1	6.28	1.79*	0.001
Q26.2	12.06	−0.85	0.213
Q26.3	8.14	1.58*	0.025
Q26.4	0.54	−0.02	0.726
Q26.5	0.62	−0.02	0.901
Q26.6	0.16	−0.02	0.751
Q38.3	0.08	−0.04	0.305
Q44.2	13.31	−1.30	0.059
Q64.1	0.66	0.25	0.180
Q75.1	2.53	−0.40	0.103
Q75.4	1.94	0.09	0.283
Q77	0.12	0.02	0.777
Q77.0	0.39	−0.02	0.691
Q77.2	0.12	−0.06	0.354
Q77.3	1.52	−0.09	0.487
Q77.4	8.98	−0.50	0.107
Q77.5	0.16	0.00	0.975
Q77.6	0.04	−0.04	0.182
Q77.7	2.45	−0.19	0.424
Q77.8	3.19	0.00	0.995
Q77.9	0.50	0.07	0.745
Q78.0	7.54	−0.18	0.793
Q78.1	4.90	0.13	0.695
Q78.2	1.79	−0.34	0.158
Q78.4	5.44	−0.38*	0.004
Q78.5	0.04	0.04	0.182
Q78.6	4.98	−0.18	0.846
Q79	0.20	−0.10	0.109
Q79.0	8.82	−0.03	0.955
Q79.1	5.94	0.51	0.354
Q79.2	3.76	0.50	0.194
Q79.3	1.83	0.13	0.640
Q79.4	0.23	0.04	0.644
Q79.5	0.43	0.10	0.288
Q79.6	1.13	−0.14	0.616
Q79.8	7.07	0.19	0.609
Q79.9	5.17	−0.23	0.386
Q81.1	0.04	−0.04	0.182
Q81.2	1.05	−0.20	0.397
Q85.0	117.58	−12.67	0.080
Q85.1	17.35	−1.52*	0.050
Q85.8	14.18	0.50	0.684
Q86.0	0.04	0.04	0.182
Q87.0	8.67	−0.69	0.193
Q87.1	19.00	0.09	0.912
Q87.2	8.84	5.42*	0.050
Q87.3	2.06	−0.07	0.809
Q87.4	29.03	−0.11	0.910
Q90	7.10	0.89	0.293
Q90.0	5.32	0.59	0.347
Q90.1	0.23	0.17	0.058
Q90.2	0.23	0.11	0.167
Q90.9	48.67	6.03	0.278
Q91	0.00	.	.
Q91.0	0.12	−0.08	0.181
Q91.1	0.00	.	.
Q91.2	0.04	0.00	1.000
Q91.3	0.70	0.05	0.583
Q91.4	0.04	0.04	0.182
Q91.5	0.08	0.06	0.058
Q91.7	0.15	0.06	0.324
Q93.4	2.14	0.03	0.946
Q93.5	10.64	1.05	0.307
Q96	3.15	0.06	0.896
Q96.0	2.13	0.30	0.533
Q96.1	0.43	0.17	0.293
Q96.2	0.04	0.02	0.559
Q96.3	1.28	0.26	0.265
Q96.4	0.54	0.21	0.105
Q96.8	3.92	0.16	0.553
Q96.9	32.59	5.01	0.216
Q98.0	11.85	0.64	0.586
Q98.1	0.62	0.09*	0.017
Q98.2	0.35	0.10	0.288
Q98.4	4.19	0.23	0.632
Q99.2	1.24	0.44	0.197

Abbreviation: KCD Korean Standard Classification of Diseases
^aAverage of annual cumulative incidence per 10,000,000 of rare diseases from 2011 to 2015 according to the KCD-7 code
^blinear trend of annual cumulative incidence per 10,000,000 per year
^cAverage of annual cumulative incidence per 10,000,000 of rare diseases from 2014 to 2015 according to the KCD-7 code
*β was statistically significant (p value < 0.05)

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