. 2022 Sep 1;57(5):551–557. doi: 10.5152/TurkArchPediatr.2022.22106
Content of this journal is licensed under a Table 1.
Comparative Data of FMF Patients of Turkish and Crimean Tatar Origin
|
FMF Features
Median (IQR) or n (%) |
Turkish FMF Patients (n = 40) | Crimean Tatars FMF Patients (n = 18) | P |
|---|---|---|---|
| Gender, males, n (%) | 23 (57.5) | 6 (33.3) | .089* |
| Age of inclusion, years | 12.7 (8.5; 16.4) | 11.3 (6.8; 14.9) | .326** |
| The age of onset, years, (IQR) | 3.3 (1.9; 5.0) | 1.3 (0.2; 3.9) | .040** |
| Age of FMF diagnosis, years, (IQR) | 4.7 (2.5; 8.2) | 9.6 (4.1; 14.3) | .005** |
| Diagnosis delay, years, (IQR) | 0.9 (0.2; 2.1) | 5.5 (2.1; 9.5) | <.001 |
| Family history of FMF, n (%) | 16 (40.0) | 9 (50.0) | .477* |
| Familial consanguinity, n (%) | 8 (20.0) | 6 (33.3) | .272* |
| Initial features | |||
| Fever, n (%) | 33 (82.5) | 18 (100.0) | .058* |
| Episode duration, days, (IQR) | 2.0 (2.0; 3.0) | 3.0 (3.0; 6.0) | <.001** |
| Fever duration, hours, (IQR) | 48.0 (48.0; 72.0) | 72.0 (72.0; 120.0) | <.001** |
| Chest pain, n (%) | 12 (30.0) | 1 (5.6) | .039* |
| Chest pain duration, hours, (IQR) | 48.0 (24.0; 72.0) | 0.0 (0.0; 0.0) | <.001** |
| Abdominal pain, n (%) | 30 (75.0) | 9 (50.0) | .061* |
| Abdominal pain duration, hours, (IQR) | 48.0 (24.0; 48.0) | 24.0 (24.0; 24.0) | .043** |
| Arthritis, n (%) | 10 (25.0) | 16 (88.9) | <.001* |
| Joints with arthritis, n (%) | |||
| Knee | 8 (80.0) | 15 (68.2) | .082* |
| Ankle | 2 (20.0) | 0 (0.0) | |
| Hip | 0 (0.0) | 5 (22.7) | |
| Pubic symphysis | 0 (0.0) | 2 (9,1) | |
| Wrist | 0 (0.0) | 1 (5.5) | |
| Monoarthritis, n (%) Two joints with arthritis, n (%) |
9 (90.0) 1 (10.0) |
11 (68.8) 5 (31.2) |
.033* |
| Arthralgia, n (%) | 19 (47.5) | 17 (94.4) | <.001* |
| Erysipeloid rash, n (%) | 0 (0.0) | 9 (50.0) | <.001* |
| Genotype distribution | |||
| MEFV genotypes, n (%) | |||
| M694V/M694V | 17 (42.5) | 2 (11.0) | .01* |
| M694V/M680I | 2 (5.0) | 0 (0) | |
| M694V/V726A | 3 (7.5) | 0 (0) | |
| M694V/R202Q | 1 (2.5) | 0 (0) | |
| M694V/- | 7 (17.5) | 13 (72.2) | |
| M694V/R761H | 1 (2.5) | 0 (0) | |
| M680I/M680I | 1 (2.5) | 0 (0) | |
| M680I/V726A | 5 (12.5) | 1 (5.6) | |
| M680I/- | 1 (2.5) | 1 (5.6) | |
| V726A/- | 1 (2.5) | 1 (5.6) | |
| R761H/- | 1 (2.5) | 0 (0) | |
| Homozygous, n (%) | 18 (45.0) | 2 (11.1) | .001* |
| Compound-heterozygous, n (%) | 12 (30.0) | 1 (5.6) | |
| Heterozygous, n (%) | 10 (25.0) | 15 (83.3) | |
*χ2 test/Fisher’s exact test, **Mann–Whitney U test.
ESR, erythrocyte sedimentation rate; IQR, interquartile range; FMF, familial Mediterranean fever; IBD, inflammatory bowel disease; JIA, juvenile idiopathic arthritis.