title: Ulinastatin, a urinary trypsin inhibitor, for the initial treatment of patients with Kawasaki disease: a retrospective study.
abstract: BACKGROUND
  Markedly activated neutrophils or higher plasma levels of neutrophil elastase are involved in the poor response to intravenous immunoglobulin (IVIG) and the formation of coronary artery lesions (CAL) in patients with acute Kawasaki disease. We hypothesized that ulinastatin (UTI), by both direct and indirect suppression of neutrophils, would reduce the occurrence of CAL.
  
  
  RESULTS
  We retrospectively analyzed the clinical records of patients with Kawasaki disease between 1998 and 2009. Three hundred sixty-nine patients were treated with a combination of UTI, aspirin, and IVIG as an initial treatment (UTI group), and 1178 were treated with a conventional initial treatment, and IVIG with aspirin (control group). The baseline characteristics did not demonstrate notable differences between the two groups. The occurrence of CAL was significantly lower in the UTI group than in the control group (3% versus 7%; crude odds ratio [OR], 0.46; 95% confidence interval [CI], 0.25-0.86; P=0.01). The OR adjusted for sex, Gunma score (the predictive score for IVIG unresponsiveness), and dosage of initial IVIG (1 or 2 g/kg) was 0.32 (95% CI, 0.17-0.60; P<0.001). In addition, most CAL occurred in patients requiring additional rescue treatment and the proportion of those patients was significantly lower in the UTI group than in the control group (13% versus 22%; crude OR, 0.52; 95% CI, 0.38-0.73; P<0.001). The adjusted OR was 0.30 (95% CI, 0.20-0.44; P<0.001).
  
  
  CONCLUSIONS
  UTI was associated with fewer patients requiring additional rescue treatment and reduction of CAL in this retrospective study.
authors: The Department of Pediatrics, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama 359-8513, Japan. dr22040@ndmc.ac.jp
authors: Takashi
authors: T
authors: Kanai
authors: Takahiro
authors: T
authors: Ishiwata
authors: Tohru
authors: T
authors: Kobayashi
authors: Hiroki
authors: H
authors: Sato
authors: Mari
authors: M
authors: Takizawa
authors: Yoichi
authors: Y
authors: Kawamura
authors: Hiroshi
authors: H
authors: Tsujimoto
authors: Keigo
authors: K
authors: Nakatani
authors: Naoko
authors: N
authors: Ishibashi
authors: Mitsunori
authors: M
authors: Nishiyama
authors: Yoshiho
authors: Y
authors: Hatai
authors: Yuh
authors: Y
authors: Asano
authors: Tomio
authors: T
authors: Kobayashi
authors: Seiichiro
authors: S
authors: Takeshita
authors: Shigeaki
authors: S
authors: Nonoyama
mesh_terms: D000208:Acute Disease; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage; D001241:Aspirin / Q000008:administration & dosage; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003324:Coronary Artery Disease / Q000276:immunology / Q000517:prevention & control* / Q000628:therapy; D004359:Drug Therapy, Combination; D005260:Female; D006023:Glycoproteins / Q000008:administration & dosage* / Q000009:adverse effects; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D007223:Infant; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000188:drug therapy* / Q000276:immunology; D009504:Neutrophils / Q000187:drug effects* / Q000276:immunology / Q000378:metabolism; D012189:Retrospective Studies; D016896:Treatment Outcome; D014361:Trypsin Inhibitors / Q000008:administration & dosage* / Q000009:adverse effects
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D006023:Glycoproteins; D016756:Immunoglobulins, Intravenous; D014361:Trypsin Inhibitors; C028665:urinastatin; D001241:Aspirin