Conservative Management of Spontaneous Isolated Dissection of the Superior Mesenteric Artery

Purpose. We report the clinical outcomes of patients with spontaneous isolated dissection of the superior mesenteric artery (SIDSMA) who were treated conservatively. Materials and Methods. A retrospective review was performed in 14 patients from 2006 to 2016 with SIDSMA. Their clinical features and computed tomographic angiography (CTA) characteristics, treatment methods, and clinical outcomes were analyzed. The mean age was 53.6 (range, 41–73) years, and the mean follow-up duration was 20.6 (range, 1–54) months. Conservative management was the primary treatment if no bowel ischemia or arterial rupture was noted. Results. The mean initial abdominal visual analog pain score was 7 (range, 5–9) in seven patients. The mean total duration of abdominal pain was 10.2 days (range, 2–42 days) in 10 patients. The mean percentage stenosis of the dissected SMA at the initial presentation was 78.8% in 14 patients. Complete obstruction of the SMA at the initial presentation was evident in 4 of the 14 patients (28.6%). Conservative management was successful in all 14 patients. None of the 14 patients developed bowel ischemia or an infarction. Abdominal pain did not recur in any patient during follow-up (mean, 20.6 months; range, 1–54 months). Conclusion. Conservative management was successful for all SIDSMA patients, even those with severe compression of the true lumen or complete obstruction of the dissected SMA

Role of Staphylococcal Superantigen in Atopic Dermatitis: Influence on Keratinocytes

Staphylococcus aureus may perform an crucial function in atopic dermatitis (AD), via the secretion of superantigens, including staphylococcal enterotoxins (SE) A or B, and toxic shock syndrome toxin-1 (TSST-1). Dysregulated cytokine production by keratinocytes (KCs) upon exposure to staphylococcal superantigens (SsAgs) may be principally involved in the pathophysiology of AD. We hypothesized that lesional KCs from AD may react differently to SsAgs compared to nonlesional skin or normal skin from nonatopics. We conducted a comparison of HLA-DR or CD1a expression in lesional skin as opposed to that in nonlesional or normal skin by immunohistochemistry (IHC). We also compared, using ELISA, the levels of IL-1α, IL-1β, and TNF-α secreted by cultured KCs from lesional, nonlesional, and normal skin, after the addition of SEA, SEB and TSST-1. IHC revealed that both HLA-DR and CD1a expression increased significantly in the epidermis of lesional skin versus nonlesional or normal skin in quite a similar manner. IL-1α, IL-1β, and TNF-α secretion was also significantly elevated in the cultured KCs from lesional skin after the addition of SsAgs. Our results indicated that KCs from lesional skin appear to react differently to SsAgs and increased proinflammatory cytokine production in response to SsAgs may contribute to the pathogenesis of AD

Radiologic and Clinical Features of Idiopathic Granulomatous Lobular Mastitis Mimicking Advanced Breast Cancer

Idiopathic granulomatous lobular mastitis (IGLM), also known as idiopathic granulomatous mastitis, is a rare chronic inflammatory lesion of the breast that can clinically and radiographically mimic breast carcinoma. The aim of this study was to describe the radiological imaging and clinical features of IGLM in order to better differentiate this disorder from breast cancer. We performed a retrospective analysis of the clinical and radiographic features of 11 women with a total of 12 IGLM lesions. The ages of these women ranged between 29 and 42 years, with a mean age of 34.8 years. Ten patients were examined by both mammography and sonography and one by sonography alone. The sites that were the most frequently involved were the peripheral (6/12), diffuse, (3/12), and subareolar (3/12) regions of the breast. The patient mammograms showed irregular ill-defined masses (7/11), diffuse increased densities (3/11), and one oval obscured mass. In addition, patient sonograms showed irregular tubular lesions (7/12) or lobulated masses with minimal parenchymal distortion (2/12), parenchymal distortion without definite mass lesions (2/12), and one oval mass. Subcutaneous fat obliteration (12/12) and skin thickening (11/12) were also observed in these patients. Contrary to previous reports, skin changes and subareolar involvement were not rare occurrences in IGLM. In conclusion, the sonographic features of IGLM show irregular or tubular hypoechoic masses with minimal parenchymal distortion. Both clinical information and the description of radiographic features of IGLM may aid in the differentiation between IGLM and breast cancer, however histological confirmation is still required for the proper diagnosis and treatment of the disorder

Large Cell Neuroendocrine Carcinoma of the Thymus: A Two-Case Report

A large cell neuroendocrine carcinoma (LCNEC) of the thymus is a very rare malignant tumor that has a very poor prognosis. The detailed clinical features of LCNEC are still unknown, including the long term prognoses and the definitive modalities of the treatment for LCNEC of the thymus. We are reporting 2 cases of an enlarged LCNEC of the thymus, both of which were diagnosed and treated by surgical resection followed by postoperative adjuvant chemoradiation therapy. Although recurrences and metastases of the LCNEC were noticed 1 and 4 years postoperatively for each case, aggressive surgical resection and adjuvant chemoradiation therapy may be helpful for a patient's long term survival

Eccrine Spiradenoma Arising in the Breast Misdiagnosed as an Epidermal Inclusion Cyst

Eccrine spiradenomas are rare, benign, cutaneous tumors that originate in the sweat glands. Eccrine spiradenomas in the breast are very rare and only a few cases have been reported. We report here on the case of a 47-year-old woman with superficial masses in the breast and these masses had gradually increased in size during follow-up. They were confirmed to be an eccrine spiradenoma on pathologic examination. There have been a few reports about the radiologic findings of eccrine spiradenomas of the breast. This is the first case of an eccrine spiradenoma in the breast that was characterized by multiple imaging modalities, including mammography, ultrasonography and MRI. The lesion in our patient was first diagnosed as an epidermal inclusion cyst based on the imaging findings and the mass's superficial location. Although the mammographic and ultrasonographic imaging findings of eccrine spiradenomas and epidermal inclusion cysts are similar, the MRI findings are different between epidermal inclusion cysts and eccrine spiradenomas. Eccrine spiradenomas should be considered in the differential diagnosis of cutaneous and subcutaneous lesions of the breast

Multiphasic analysis of whole exome sequencing data identifies a novel mutation of ACTG1 in a nonsyndromic hearing loss family

BACKGROUND: The genetic heterogeneity of sensorineural hearing loss is a major hurdle to the efficient discovery of disease-causing genes. We designed a multiphasic analysis of copy number variation (CNV), linkage, and single nucleotide variation (SNV) of whole exome sequencing (WES) data for the efficient discovery of mutations causing nonsyndromic hearing loss (NSHL). RESULTS: From WES data, we identified five distinct CNV loci from a NSHL family, but they were not co-segregated among patients. Linkage analysis based on SNVs identified six candidate loci (logarithm of odds [LOD] >1.5). We selected 15 SNVs that co-segregated with NSHL in the family, which were located in six linkage candidate loci. Finally, the novel variant p.M305T in ACTG1 (DFNA20/26) was selected as a disease-causing variant. CONCLUSIONS: Here, we present a multiphasic CNV, linkage, and SNV analysis of WES data for the identification of a candidate mutation causing NSHL. Our stepwise, multiphasic approach enabled us to expedite the discovery of disease-causing variants from a large number of patient variants