Table 1.
Laboratory values before (A), at onset (B) and during manifest (C) protein-losing enteropathy (PLE)
| Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | Case 6 | Case 7 | Case 8 | Normal range | |||
| Age at Fontan surgery (years) | 4.5 | 5.0 | 6.5 | 4.0 | 6.0 | 6.5 | 5.0 | 5.5 | |||
| Time of PLE onset after surgery (days) | 260 | 2790 | 90 | 2070 | 720 | 1530 | 2160 | 660 | |||
| PLE-associated infection | Rotavirus, streptococcus | Staphylococcus aureus | No Infection | Candidosis | Influenza | Viral pneumonia | Local infection | Viral, fever | |||
| Sampling | Before PLE (A) | Onset of PLE (B) | Chronic PLE (C) | A/B/C | A/B/C | A/B/C | A/B/C | A/B/C | A/B/C | A/B/C | |
| Total protein | |||||||||||
| Mean (g/l) | 76.5 | 46.2 | 55.6 | 64/43/55 | 67/37/37 | 71/49/42 | 80/43/66 | -/50/39 | 65/42/66 | 73/42/36 | 60–80 |
| Range | 71–82 | 34–49 | 44.1–71.2 | ||||||||
| Albumin | |||||||||||
| Mean (g/l) | 45.1 | 29.3 | 46.5 | -/28/32 | 49/24/23 | 42/30/22 | 46/23/42 | -/29/18 | 44/-/38 | -/-/21 | 35–52 |
| Range | 42.1–48 | 20.8–39.0 | 35.5–50.6 | ||||||||
| α1-globulin | |||||||||||
| Mean (g/l) | 2.9 | 2.5 | 3.4 | -/5.6/4.7 | 3.1/5.1/6.3 | 2.2/6/8 | 2.7/7.9/3.9 | -/7.5/10.3 | 3.2/3.2/4.6 | -/-/6.1 | 1.3–3.9 |
| Range | 2.7–3.0 | - | 2.8–3.9 | ||||||||
| α2-globulin | |||||||||||
| Mean (g/l) | 6.2 | 6.7 | 6.4 | -/13.9/12.5 | 7.8/15.3/16 | 7.7/13.5/19.1 | 7.4/16.6/8.4 | -/14.6/16.5 | 7.7/-/12.1 | -/-/13 | 5.4–9.3 |
| Range | 6.0–6.4 | - | 5.9–6.9 | ||||||||
| β-globulin | |||||||||||
| Mean (g/l) | 7.2 | 5.2 | 5.3 | -/9.4/16.5 | 5.8/15.2/11 | 7.6/10.9/14.7 | 9/12.1/11.2 | -/14.6/16.5 | 8/8/13.9 | -/-/11.8 | 5.9–11.4 |
| Range | 7–7.3 | - | 4.5–6.0 | ||||||||
| γ-globulin | |||||||||||
| Mean (g/l) | 13.2 | 3.5 | 2.8 | -/5.9/7.6 | 8.9/2.1/5.9 | 9.6/8.6/6.5 | 22.5/9.9/13.5 | -/5.4/8.3 | 14.3/-/12.2 | -/7.9/11.7 | 5.8–15.2 |
| Range | 13–13.3 | - | 2.7–2.9 | ||||||||
| α1-antitrypsin stool | |||||||||||
| Mean (mg/g) | - | + | 20.2 | -/-/4.4 | -/-/27.7 | -/-/14.1 | -/0.6/- | -/-/45.7 | -/54.3/13.0 | -/-/9.4 | < 1 |
| Range | - | - | 4.8–34 | ||||||||
| α1-antitrypsin serum | |||||||||||
| Mean (mg/dl) | 180 | - | - | -/-/260 | -/-/160 | - | -/332/- | - | - | -/-/183 | 90–200 |
| Range | 176–187 | - | - | ||||||||
| IgG | |||||||||||
| Mean (g/l) | 12.7 | 3.4 | 2.9 | -/-/2.6 | -/-/2.4 | -/-/1.25 | - | - | - | - | 5.5–15 |
| Range | 9.6–13.7 | - | 1.4–6.6 | ||||||||
| IgM | |||||||||||
| Mean (g/l) | 1 | 0.4 | 0.5 | -/-/1.2 | -/-/0.6 | -/-/0.51 | - | - | - | - | 0.4–2.1 |
| Range | - | - | 0.2–0.6 | ||||||||
| IgA | |||||||||||
| Mean (g/l) | 1.7 | 0.8 | 0.4 | -/-/0.7 | -/-/32 | - | - | - | - | - | 0.3–2.3 |
| Range | - | - | 0.2–0.6 | ||||||||
| Leukocyte count | |||||||||||
| Mean (Gpt/l) | 11 | 19.3 | 17.3 | 9.4/13.2/6.3 | 7.8/7.3/7.7 | 14.3/6.6/6.1 | 6.3/7.7/6.3 | 8/5.3/7.5 | 8.9/9.3/5.7 | 6.1/11.3/5.6 | 3.8–13.5 |
| Range | 9.5–12.9 | 12.5–24.7 | 8.7–22.1 | ||||||||
| Lymphocyte count | |||||||||||
| Mean (Gpt/l) | 4.3 | 3.7 | 2.5 | -/0.8/1.0 | 2.6/-/0.7 | 2.8/1.6/0.6 | 1.6/1.3/1.3 | -/-/0.7 | 3.5/0.9/1.0 | 2.6/-/0.4 | 1.5–4.0 |
| Range | 3.1–5.8 | - | 1.7–4.3 |
Mean and respective data ranges. +, value above normal range, without quantitative data in the records; -, no data available. The date of PLE onset in cases 2–8 was not exactly recorded and is estimated.