An endovascular repair of an abdominal aortic aneurysm (AAA) is technically less invasive than open surgery, but gives results as effective as open surgery if the anatomy is adequate. Unfortunately, 20-30% of AAA patients are not suitable for endovascular repair because they lack a sufficient proximal landing zone. In an effort to broaden the applicability of endovascular repair, the chimney technique has been introduced. This refers to deployment of a covered or bare-metal stent parallel to the main aortic endograft within the aneurysm, creating a conduit that runs outside the main aortic endograft into the aortic branches. We report the case of a 75-year-old male with a juxtarenal abdominal aortic aneurysm and multiple comorbidities who was treated successfully with an endovascular aneurysm repair using a chimney graft.
Aged ; Aneurysm ; Aortic Aneurysm, Abdominal* ; Comorbidity ; Endovascular Procedures ; Humans ; Male ; Stents ; Transplants*

An endovascular repair of an abdominal aortic aneurysm (AAA) is technically less invasive than open surgery, but gives results as effective as open surgery if the anatomy is adequate. Unfortunately, 20-30% of AAA patients are not suitable for endovascular repair because they lack a sufficient proximal landing zone. In an effort to broaden the applicability of endovascular repair, the chimney technique has been introduced. This refers to deployment of a covered or bare-metal stent parallel to the main aortic endograft within the aneurysm, creating a conduit that runs outside the main aortic endograft into the aortic branches. We report the case of a 75-year-old male with a juxtarenal abdominal aortic aneurysm and multiple comorbidities who was treated successfully with an endovascular aneurysm repair using a chimney graft.
Aged ; Aneurysm ; Aortic Aneurysm, Abdominal* ; Comorbidity ; Endovascular Procedures ; Humans ; Male ; Stents ; Transplants*

No abstract available.
Cardiomyopathy, Hypertrophic ; Mitral Valve Insufficiency ; Mitral Valve

Erdheim-Chester disease (ECD), also known as non-Langerhans cell histiocytosis, is a multi-systemic disease with unclear pathogenesis. Based on a small number of case studies, pegylated interferon-α (PEG-IFN-α) has been used as the front-line treatment option. However, there are limited data regarding administration of ropegylated-interferon α-2b (ROPEG-IFN-α 2b) for ECD patients. Herein, we report two cases of severe ECD treated with two types of PEG-IFN-α. One patient with heart and skeleton involvement and BRAF V600E mutation was treated with weekly PEG-IFN-α 2a. Another patient with bone involvement and no BRAF V600E mutation was administered monthly ROPEG-IFN-α 2b. The two types of PEG-IFN-α showed excellent disease control, excellent survival outcomes, and manageable toxicities in ECD patients. These results suggest that ROPEG-IFN-α 2b could be used equivalently to PEG-IFN-α 2a for management of advanced ECD.

Mowat-Wilson syndrome is an extremely rare genetic disease that is characterized by intellectual disability, facial dysmorphism, Hirschsprung's disease, and other congenital anomalies. This disorder is caused by heterozygous mutations or deletions in the zinc finger E-box-binding homeobox-2 gene (ZEB2). Thus far, approximately 200 cases of Mowat-Wilson syndrome have been reported worldwide. In Korea, only one case with a 2q22 deletion, which also affects ZEB2, has been previously reported. Here, we describe a patient with Mowat-Wilson syndrome who presented with developmental delays, typical facial dysmorphism, and Hirschsprung's disease. Molecular analysis of ZEB2 identified a novel heterozygous mutation at c.190dup (p.S64Kfs*6). To our knowledge, this is the second report of a Korean patient with Mowat-Wilson syndrome that has been confirmed genetically.
Hirschsprung Disease* ; Humans ; Intellectual Disability ; Korea ; Zinc Fingers