Patient characteristics for all patients, all controls, and the respective controls cohorts
| . | Children with FHL (N = 366) . | Controls (N = 1064) . | Inf-controls-1 (n = 329) . | Inf-controls-2 (ICU) (n = 361) . | Rheuma controls (n = 374) . |
|---|---|---|---|---|---|
| Yes, N/evaluated, n/yes of total, % | |||||
| Male sex | 189/366/51.6% | 554/1064/52.1% | 166/329/50.5% | 204/361/56.5% | 184/374/49.2% |
| Fever | 331/363/90.4% | 836/1063/78.6% | 329/329/100% | 152/360/42.1% | 355/374/94.9% |
| Splenomegaly | 349/366/95.4% | 131/1059/12.3% | 20/328/6.1% | 25/361/6.9% | 86/370/23% |
| Hepatomegaly | 308/342/84.2% | 225/1060/21.1% | 32/328/9.7% | 77/361/21.3% | 116/371/31% |
| Median (mean; IQR)/evaluated, n | |||||
| Age at start of HLH-2004, y | 0.294 (1.88; 0.162-1.37)/366 | 4.48 (5.70; 1.68-9.16)/1064 | 3.33 (4.92; 1.45-7.92)/329 | 4.85 (5.92; 1.09-10.4)/361 | 5.49 (6.18; 2.58-9.56)/374 |
| Hemoglobin, g/L | 75.0 (75.8; 65.0-84.8)/342 | 105 (105; 92.0-118)/1044 | 118 (116; 108-127)/326 | 96.0 (97.9; 86.0-108)/355 | 101 (102; 90.0-113)/363 |
| Neutrophils, ×109/L | 0.60 (1.25; 0.295-1.20)/311 | 8.62 (10.2; 4.06-14.0)/910 | 6.84 (8.93; 3.73-11.8)/323 | 6.84 (8.97; 2.69-12.8)/330 | 11.6 (13.3; 7.65-17.4)/257 |
| Platelets, ×109/L | 31.0 (48.6; 17.8-56.0)/340 | 325 (348; 194-480)/1042 | 342 (361; 260-443)/327 | 149 (172; 72.3-235)/354 | 498 (510; 375-614)/361 |
| Triglycerides, mmol/L | 3.67 (4.18; 2.49-5.46)/338 | 1.40 (1.79; 1.09-1.86)/366 | 1.50 (1.75; 1.21-1.84)/122 | 1.52 (2.22; 1.01-2.99)/116 | 1.36 (1.45; 1.00-1.65)/128 |
| Fibrinogen, g/L | 1.00 (1.39; 0.690-1.80)/324 | 5.13 (5.32; 3.81-6.59)/330 | 4.01 (4.43; 2.90-5.60)/132 | 5.60 (5.91; 4.63-7.20)/198 | |
| Ferritin, μg/L | 2 590 (7 940; 1 200-7 640)/327 | 188 (1340; 76.0-624)/810 | 67.0 (156; 32.0-126)/201 | 212 (1790; 95.4-605)/361 | 462 (1660; 157-1600)/248 |
| sCD25, U/mL | 20 700 (23 000; 9 750-29 800)/113 | 2050 (2810; 1370-3080)/361 | 2050 (2810; 1370-3080)/361 | ||
| AST, U/L | 162 (317; 75.0-352)/288 | 33.0 (49.7; 24.0-44.0)/661 | 34.0 (61.7; 25.0-46.0)/311 | 32.0 (39.0; 22.0-42.0)/350 | |
| ALT, U/L | 125 (205; 56.3-266) /320 | 27.0 (87.1; 15.0-41.0)/938 | 22.0 (48.8; 14.0-36.0)/314 | 35.0 (190; 21.0-85.0)/297 | 25.0 (30.5; 13.0-35.0)/327 |
| LDH, U/L | 704 (1 070; 493-1 170)/286 | 442 (517; 314-608)/488 | 470 (529; 338-615)/236 | 427 (507; 302-598)/252 | |
| Bilirubin, μmol/L | 23.1 (58.2; 13.3-78.1)/281 | 8.55 (17.8; 5.13-15.4)/722 | 7.52 (12.8; 4.28-13.7)/215 | 10.3 (27.8; 5.13-22.2)/288 | 6.84 (9.55; 5.00-11.3)/219 |
| Creatinine, μmol/L | 27.0 (37.6; 19.1-44.2)/266 | 36.3 (50.1; 26.5-54.8)/999 | 35.4 (41.5; 25.7-52.2)/317 | 35.4 (59.0; 22.1-55.7)/361 | 44.2 (48.5; 32.7-61.9)/321 |
| Albumin, g/L | 28.0 (28.2; 24.0-32.0)/265 | 38.0 (37.4; 33.0-42.0)/521 | 39.6 (39.1; 35.4-43.0)/264 | 35.6 (35.7; 30.8-40.0)/257 | |
| . | Children with FHL (N = 366) . | Controls (N = 1064) . | Inf-controls-1 (n = 329) . | Inf-controls-2 (ICU) (n = 361) . | Rheuma controls (n = 374) . |
|---|---|---|---|---|---|
| Yes, N/evaluated, n/yes of total, % | |||||
| Male sex | 189/366/51.6% | 554/1064/52.1% | 166/329/50.5% | 204/361/56.5% | 184/374/49.2% |
| Fever | 331/363/90.4% | 836/1063/78.6% | 329/329/100% | 152/360/42.1% | 355/374/94.9% |
| Splenomegaly | 349/366/95.4% | 131/1059/12.3% | 20/328/6.1% | 25/361/6.9% | 86/370/23% |
| Hepatomegaly | 308/342/84.2% | 225/1060/21.1% | 32/328/9.7% | 77/361/21.3% | 116/371/31% |
| Median (mean; IQR)/evaluated, n | |||||
| Age at start of HLH-2004, y | 0.294 (1.88; 0.162-1.37)/366 | 4.48 (5.70; 1.68-9.16)/1064 | 3.33 (4.92; 1.45-7.92)/329 | 4.85 (5.92; 1.09-10.4)/361 | 5.49 (6.18; 2.58-9.56)/374 |
| Hemoglobin, g/L | 75.0 (75.8; 65.0-84.8)/342 | 105 (105; 92.0-118)/1044 | 118 (116; 108-127)/326 | 96.0 (97.9; 86.0-108)/355 | 101 (102; 90.0-113)/363 |
| Neutrophils, ×109/L | 0.60 (1.25; 0.295-1.20)/311 | 8.62 (10.2; 4.06-14.0)/910 | 6.84 (8.93; 3.73-11.8)/323 | 6.84 (8.97; 2.69-12.8)/330 | 11.6 (13.3; 7.65-17.4)/257 |
| Platelets, ×109/L | 31.0 (48.6; 17.8-56.0)/340 | 325 (348; 194-480)/1042 | 342 (361; 260-443)/327 | 149 (172; 72.3-235)/354 | 498 (510; 375-614)/361 |
| Triglycerides, mmol/L | 3.67 (4.18; 2.49-5.46)/338 | 1.40 (1.79; 1.09-1.86)/366 | 1.50 (1.75; 1.21-1.84)/122 | 1.52 (2.22; 1.01-2.99)/116 | 1.36 (1.45; 1.00-1.65)/128 |
| Fibrinogen, g/L | 1.00 (1.39; 0.690-1.80)/324 | 5.13 (5.32; 3.81-6.59)/330 | 4.01 (4.43; 2.90-5.60)/132 | 5.60 (5.91; 4.63-7.20)/198 | |
| Ferritin, μg/L | 2 590 (7 940; 1 200-7 640)/327 | 188 (1340; 76.0-624)/810 | 67.0 (156; 32.0-126)/201 | 212 (1790; 95.4-605)/361 | 462 (1660; 157-1600)/248 |
| sCD25, U/mL | 20 700 (23 000; 9 750-29 800)/113 | 2050 (2810; 1370-3080)/361 | 2050 (2810; 1370-3080)/361 | ||
| AST, U/L | 162 (317; 75.0-352)/288 | 33.0 (49.7; 24.0-44.0)/661 | 34.0 (61.7; 25.0-46.0)/311 | 32.0 (39.0; 22.0-42.0)/350 | |
| ALT, U/L | 125 (205; 56.3-266) /320 | 27.0 (87.1; 15.0-41.0)/938 | 22.0 (48.8; 14.0-36.0)/314 | 35.0 (190; 21.0-85.0)/297 | 25.0 (30.5; 13.0-35.0)/327 |
| LDH, U/L | 704 (1 070; 493-1 170)/286 | 442 (517; 314-608)/488 | 470 (529; 338-615)/236 | 427 (507; 302-598)/252 | |
| Bilirubin, μmol/L | 23.1 (58.2; 13.3-78.1)/281 | 8.55 (17.8; 5.13-15.4)/722 | 7.52 (12.8; 4.28-13.7)/215 | 10.3 (27.8; 5.13-22.2)/288 | 6.84 (9.55; 5.00-11.3)/219 |
| Creatinine, μmol/L | 27.0 (37.6; 19.1-44.2)/266 | 36.3 (50.1; 26.5-54.8)/999 | 35.4 (41.5; 25.7-52.2)/317 | 35.4 (59.0; 22.1-55.7)/361 | 44.2 (48.5; 32.7-61.9)/321 |
| Albumin, g/L | 28.0 (28.2; 24.0-32.0)/265 | 38.0 (37.4; 33.0-42.0)/521 | 39.6 (39.1; 35.4-43.0)/264 | 35.6 (35.7; 30.8-40.0)/257 | |
IQR, interquartile range.