title: Intravenous ulinastatin therapy for Stevens-Johnson syndrome and toxic epidermal necrolysis in pediatric patients. Three case reports.
abstract: BACKGROUND
  More effective therapy is needed for the treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). The clinical efficacy of intravenous ulinastatin therapy was investigated in 3 Japanese pediatric patients with SJS or TEN.
  
  
  METHODS
  Ulinastatin was given to 1 pediatric SJS patient and 2 pediatric TEN patients within 7 days (patient 1; SJS), 6 days (patient 2; TEN), or 4 days (patient 3; TEN) after the onset of the skin rash. Ulinastatin was administered intravenously at a dose of 7,500 U/kg/day (maximum dose: 300,000 U/day). No corticosteroids were given. After the skin lesions resolved, the ulinastatin dose was reduced to between 2,500 and 5,000 U/kg/day as maintenance therapy and then the drug was withdrawn.
  
  
  RESULTS
  Erythema, fatigue, and fever improved within 12-36 h of starting the ulinastatin infusion, and the skin lesions resolved completely after 4-7 days of ulinastatin therapy. None of the patients had cutaneous or ocular sequelae. No patient developed secondary infection or relapse and ulinastatin therapy caused no side effects.
  
  
  CONCLUSIONS
  Ulinastatin dramatically reduced the febrile period with no adverse effects and was very safe in this study. Ulinastatin appears to be a useful and effective therapy for controlling SJS and TEN without sequelae.
authors.affiliation: Department of General Pediatrics, Nihon University Nerima-Hikarigaoka Hospital, Nihon University School of Medicine, Tokyo, Japan. y-inamo@pb3.so-net.ne.jp
authors.forename: Yasuji
authors.initials: Y
authors.lastname: Inamo
authors.forename: Takahiro
authors.initials: T
authors.lastname: Okubo
authors.forename: Mika
authors.initials: M
authors.lastname: Wada
authors.forename: Sachiko
authors.initials: S
authors.lastname: Fuchigami
authors.forename: Kouji
authors.initials: K
authors.lastname: Hashimoto
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Fuchigami
authors.forename: Shigeru
authors.initials: S
authors.lastname: Takahashi
authors.forename: Shigemasa
authors.initials: S
authors.lastname: Sawada
authors.forename: Kensuke
authors.initials: K
authors.lastname: Harada
mesh_terms: D000818:Animals; D002648:Child; D006023:Glycoproteins / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D007275:Injections, Intravenous; D008297:Male; D013262:Stevens-Johnson Syndrome / Q000188:drug therapy*; D016896:Treatment Outcome; D014361:Trypsin Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use*
chemical_list: D006023:Glycoproteins; D014361:Trypsin Inhibitors; C028665:urinastatin