New Insights in Abdominal Pain in Paroxysmal Nocturnal Hemoglobinuria (PNH): A MRI Study

• Published in: PloS one 2015-04, Vol.10 (4), p.e0122832-e0122832
• Main Authors: De Cobelli, Francesco, Pezzetti, Giulio, Margari, Sergio, Esposito, Antonio, Giganti, Francesco, Agostini, Giulia, Del Maschio, Alessandro
• Format: Article
• Language: English
• Subjects: Abdomen; Abdominal Pain - diagnosis; Abdominal Pain - etiology; Abdominal Pain - physiopathology; Adult; Angiogenesis; Area Under Curve; Blood; Blood vessels; Contraction; Duodenum; Duodenum - blood supply; Female; Flow; Flow cytometry; Flow velocity; Heart; Hemoglobinuria, Paroxysmal - complications; Hemoglobinuria, Paroxysmal - pathology; Hemoglobinuria, Paroxysmal - physiopathology; Humans; Ileum; Ileum - blood supply; Ischemia; Ischemia - diagnosis; Jejunum; Jejunum - blood supply; Magnetic Resonance Imaging; Male; Middle Aged; NMR; Nuclear magnetic resonance; Pain; Paroxysmal nocturnal hemoglobinuria; Perfusion; Polyethylene glycol; Prospective Studies; Regional Blood Flow; Small intestine; Splanchnic Circulation; Stroke; Stroke Volume; Studies; Thromboembolism; Veins & arteries; Young Adult
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0122832

Abdominal pain in PNH has never been investigated by in-vivo imaging studies. With MRI, we aimed to assess mesenteric vessels flow and small bowel wall perfusion to investigate the ischemic origin of abdominal pain. Six PNH patients with (AP) and six without (NOP) abdominal pain underwent MRI. In a blinded fashion, mean flow (MF, quantity of blood moving through a vessel within a second, in mL·s-1) and stroke volume (SV, volume of blood pumped out at each heart contraction, in mL) of Superior Mesenteric Vein (SMV) and Artery (SMA), areas under the curve at 60 (AUC60) and 90 seconds (AUC90) and Ktrans were assessed by two operators. Mean total perfusion and flow parameters were lower in AP than in NOP group. AUC60: 84.81 ± 11.75 vs. 131.73 ± 18.89 (P < 0.001); AUC90: 102.33 ± 14.16 vs. 152.58 ± 22.70 (P < 0.001); Ktrans: 0.0346 min-1 ± 0.0019 vs. 0.0521 ± 0.0015 (P = 0.093 duodenum, 0.009 jejunum/ileum). SMV: MF 4.67 ml/s ± 0.85 vs. 8.32 ± 2.14 (P = 0.002); SV 3.85 ml ± 0.76 vs. 6.55 ± 1.57 (P = 0.02). SMA: MF 6.95 ± 2.61 vs. 11.2 ± 2.32 (P = 0.07); SV 6.52 ± 2.19 vs. 8.78 ± 1.63 (P = 0.07). We found a significant correlation between MF and SV of SMV and AUC60 (MF:ρ = 0.88, P < 0.001; SV: ρ = 0.644, P = 0.024), AUC90 (MF: ρ = 0.874, P < 0.001; SV:ρ = 0.774, P = 0.003) and Ktrans (MF:ρ = 0.734, P = 0.007; SV:ρ = 0.581, P = 0.047). Perfusion and flow MRI findings suggest that the impairment of small bowel blood supply is significantly associated with abdominal pain in PNH.