Table 1.
Clinical observations and laboratory data before and after empagliflozin administration.
| Sex (M/F) | Current age (years) | Follow-up (months) | Average dose of empagliflozin (mg/kg/day) | |
|---|---|---|---|---|
| Patient 1 Patient 2 Patient 3 |
M M F |
7.5 5 8.5 |
36 34 31 |
0.38 0.48 0.57 |
| Equal time frames | BEFORE TREATMENT | AFTER TREATMENT | ||
| NEUTROPHYL DYSFUNCTION | ||||
| Absolute neutrophil count—median, range (/µL) | ||||
| Patient 1 Patient 2 Patient 3 |
1200 (300–2500) 1260 (0–2300) 550 (100–970) |
1750 (800–4100) 1850 (1120–3370) 1600 (1200–3100) |
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| Infections (No, description) * ABTh—antibiotic therapy, H—hospitalization | ||||
| Patient 1 Patient 2 Patient 3 |
8 ABTh, 7 H; chronic cough, septic complication following gastrostomy insertion 6 ABTh, 9 H; inflammatory complications following gastrostomy insertion, slow postoperative wound healing, sepsis due to abdominal infection 7 ABTh, 5 H; frequent bacterial infections of the respiratory and GIT |
3 ABTh, 0 H; predominantly viral infections, chronic cough ceased 2 ABTh, 1 H 0 ABTh, 2 H; fewer viral infections |
||
| G-CSF dose (µg/kg/day) | ||||
| Patient 1 Patient 2 Patient 3 |
1.39 0.80 1.64 |
Discontinued (17th month) Discontinued (13th month) Discontinued (17th month) |
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| Neutrophil burst test | ||||
| Patient 1 Patient 2 Patient 3 |
Abnormal, 0.11 Not performed Normal |
Normal Normal Normal |
||
| INFLAMMATION | ||||
| Sedimentation rate median value (mm/h) | ||||
| Patient 1 Patient 2 Patient 3 |
120 55 120 |
23 18 15.5 |
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| Ig levels * normal values: IgG (3.86–14.7); IgA (0.29–2.56); IgM 2 (0.37–2.24) (g/L) | ||||
| Patient 1 Patient 2 Patient 3 |
IgG 29; IgA 3.48; IgM 1.85 IgG 13.5; IgA 1.14; IgM 0.62 IgG 25.35; IgA 6.82; IgM 2.55 |
IgG=19.3; IgA=2.69; IgM=1.73 IgG=19.3; IgA=2.69; IgM=1.73 IgG=13.65; IgA=3.30; IgM=2.18 |
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| Platelets median value | ||||
| Patient 1 Patient 2 Patient 3 |
450x109/L 477x109/L 649x109/L |
345x109/L 418x109/L 443x109/L |
||
| ANEMIA | ||||
| Hemoglobin values (g/L), MCV values (fL) | ||||
| Patient 1 Patient 2 Patient 3 |
Hb: 113, MCV: 72.2 Hb: 108, MCV: 73.5 Hb: 106, MCV: 72.7 |
Hb: 132, MCV: 75.0 Hb: 143, MCV: 77.9 Hb: 145, MCV: 81.0 |
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| Iron supplementation | ||||
| Patient 1 Patient 2 Patient 3 |
Often required Regularly required Regularly required |
Not needed Not needed Not needed |
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| CHRONIC INFLAMMATORY BOWEL DISEASE | ||||
| Symptoms, histological status; * IBD—Inflammatory bowel disease | ||||
| Patient 1 Patient 2 Patient 3 |
Cramping abdominal pain, bloody stools Intestinal colics, poorly formed stools up to 8 times a day; no histological signs of IBD Asymptomatic, no histological signs of IBD |
The pain subsided in 1 week, and stool consistency improved, mucus and blood vanished, IBD remission on histology Improved stool, occasional constipation; no histological signs of IBD Asymptomatic, no histological signs of IBD |
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| Aphthous ulcers | ||||
| Patient 1 Patient 2 Patient 3 |
Recurrent Recurrent Constant, requires pain medications and refuses oral feeding |
2 episodes after viral infection None None |
||
| SEVERE HYPOGLYCEMIA EPISODES | ||||
| Patient 1 Patient 2 Patient 3 |
6 (technical problems with the enteral pump, insufficient food intake) 0 1 (skipped meal) |
7 (enteral feeding pump dysfunction, physical activity, skipped meal) 0 1 (acute gastroenteritis) |
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| OBESITY | ||||
| BMI (kg/m2) | ||||
| Patient 1 Patient 2 Patient 3 |
17.9 (95thp) 20.8 (100thp) 15.8 (58thp) |
23.2 (100thp) 21.2 (100thp) 16.5 (60thp) |
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| GENERAL WELLBEING | ||||
| Patient 1 Patient 2 Patient 3 |
Lack of energy for physical activity Sleep problems Diminished appetite, overly sensitive |
Improved, playful, energized Improved quality of sleep, improved appetite Improved, more active, improved appetite |
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