caa a22 4500 445824530 CHVBK 20180317145255.0 cr unu---uuuuu 170323e20111001xx s 000 0 eng 10.1007/s00246-011-0056-z doi (NATIONALLICENCE)springer-10.1007/s00246-011-0056-z Successful Treatment with Bosentan for Pulmonary Hypertension and Reduced Peripheral Circulation in Juvenile Systemic Sclerosis [Elektronische Daten] [Masaki Shimizu, Yoko Hashida, Kazuyuki Ueno, Tadafumi Yokoyama, Yuko Nakayama, Takekatsu Saito, Kunio Ohta, Kazuhiko Takehara, Akihiro Yachie] Pulmonary arterial hypertension (PAH) when associated with systemic sclerosis (SSc) (SSc-PAH) is one of the leading causes of mortality and is found in 10-15% of adult patients with SSc. The ET receptor antagonist bosentan has been shown to be effective in the treatment of adult patients with SSc-PAH. Furthermore, it has been shown that bosentan ameliorates decreased skin perfusion and digital ulceration secondary to SSc. However, the effectiveness and safety of bosentan for treatment of juvenile SSc still remains unclear. We describe a case of juvenile SSc-PAH successfully treated with bosentan. The present case shows that bosentan ameliorated PAH and peripheral circulation as evaluated by cold stress thermography. No bosentan-related adverse events such as liver dysfunction were observed. Prospective randomized trials are required to validate the effectiveness of bosentan for patients with juvenile SSc; however, bosentan might be useful for the management of patients with juvenile SSc. Springer Science+Business Media, LLC, 2011 Bosentan nationallicence Juvenile systemic sclerosis nationallicence Pulmonary hypertension nationallicence Shimizu Masaki Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut Hashida Yoko Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut Ueno Kazuyuki Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut Yokoyama Tadafumi Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut Nakayama Yuko Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut Saito Takekatsu Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut Ohta Kunio Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut Takehara Kazuhiko Department of Dermatology, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut Yachie Akihiro Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut Pediatric Cardiology Springer-Verlag 32/7(2011-10-01), 1040-1042 0172-0643 32:7<1040 2011 32 246 https://doi.org/10.1007/s00246-011-0056-z text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00246-011-0056-z text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Shimizu Masaki Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut NATIONALLICENCE 700 1- Hashida Yoko Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut NATIONALLICENCE 700 1- Ueno Kazuyuki Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut NATIONALLICENCE 700 1- Yokoyama Tadafumi Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut NATIONALLICENCE 700 1- Nakayama Yuko Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut NATIONALLICENCE 700 1- Saito Takekatsu Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut NATIONALLICENCE 700 1- Ohta Kunio Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut NATIONALLICENCE 700 1- Takehara Kazuhiko Department of Dermatology, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut NATIONALLICENCE 700 1- Yachie Akihiro Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan aut NATIONALLICENCE 773 0- Pediatric Cardiology Springer-Verlag 32/7(2011-10-01), 1040-1042 0172-0643 32:7<1040 2011 32 246 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer