Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder with characteristic vascular malformations of the skin, gastrointestinal system, and, less often, other organ systems. The characteristic cutaneous lesions consist of deep-blue, soft, rubbery blebs, which are easily compressible. The most serious complication is abundant gastrointestinal bleeding. We describe the case of an 8-year-old girl with diagnosed BRBNS who had multiple venous malformations all over her body, importantly, throughout the gastrointestinal tract, mouth, esophagus, stomach, small bowel, and colon. She presented with recurrent massive gastrointestinal bleeding and soft tissue hematoma despite prednisolone and α-interferon therapy. We started low-dose sirolimus as an antiangiogenic agent. The vascular masses were reduced rapidly and there was no gastrointestinal bleeding and muscular hematoma after sirolimus therapy. There was no drug adverse reaction at 20-month follow-up. To the best of our knowledge, this is the first report related to the use of sirolimus in a patient with BRBNS.

Purpose: Pulmonary fibrosis is a devastating disease with a poor prognosis. Although the diagnosis and pathophysiology of this disease have been better characterized over the past few years, there is no effective therapy for the disease. The aim of this study was to evaluate the anti-inflammatory and anti-fibrotic effects of sirolimus (SRL), which is a potential anti-fibrotic agent, by using bleomycin (BLM)-induced pulmonary fibrosis model in rats. Methods: A single intra-tracheal injection of BLM (2.5 U/kg) was administered and sirolimus (2.5 mg/kg/day) was given orally, beginning either one day before (early SRL) or nine days after (late SRL) the BLM administration. The effect of SRL on fibrosis was studied by analysis of cytokine levels in BAL fluid, measurement of lung tissue hydroxyproline (HPL) content and histopathological examination. Results: Both early and late SRL administrations caused a decrease in the levels of IL-13, PDGF-A and TGF-β1 (p=0.001) and an increase in IFN-γ levels (p=0.001) in BAL fluid. Early and late SRL also caused a decrease in HPL content (p=0.001). Early sirolimus caused a significant decrease in fibrosis score (p=0.001), while late SRL did not. Conclusion: Sirolimus was effective in BLM-induced pulmonary fibrosis model, especially in the early phases of the disease.

No abstract

AMAÇAkut mezenter iskemi (AMİ) modeli kullanılarak yapılan bu çalışmada, serum prokalsitonin ve fosfor düzeylerinin AMİ erken tanısında kullanılabilirliği araştırıldı. GEREÇ VE YÖNTEMÇalışmada 21 adet Yeni Zelanda tavşanı kullanıldı. Denekler Kontrol, Sham ve İskemi grubu olarak adlandırıl-dı. Kontrol grubundaki deneklere herhangi bir girişim yapılmadı. Sham ve İskemi grubundaki deneklere orta hat insizyonu ile laparotomi yapıldı. İskemi grubundaki deneklere ise laparatomi yapıldıktan sonra süperior mezenterik arter bulunarak bağlandı. Her üç gruptaki hayvanlardan 0., 1., 3. ve 6. saatlerde kan alındı, bu numunelerden prokalsitonin ve fosfor çalışıldı. BULGULARİskemi grubunda, serum fosfor ve prokalsitonin düzeyle-rindeki yükselme kontrol ve sham gruplarına göre istatistiksel olarak anlamlı bulundu (p<0,05). Fosfor ve prokalsitonin düzeylerinin, iskemi oluşturulduktan sonra 1. saatten itibaren arttığı ve bu yüksekliğin 6 saat boyunca devam ettiği saptandı (p<0,05). SONUÇFosfor ve prokalsitonin'in AMİ'nin erken tanısında ve prognozunda kullanılabilecek önemli parametreler olabileceğini düşünüyoruz.Anahtar Sözcükler: Akut mezenter iskemi; fosfor; prokalsitonin.

The best treatment modality for astroblastoma is surgical resection if possible, whereas adjuvant therapy (radiotherapy and/or chemotherapy) can be considered in high-grade astroblastomas, with a close follow-up for all cases.