Rapadilino syndrome

We report on a boy with severe radial hypoplasia, absent thumbs and patellae, short stature, persistent diarrhea, slender nose and normal intelligence as another example of the RAPADILINO syndrome. © 1992 Wiley-Liss, Inc.     

Is Preservation of the Adrenal Vein Mandatory in Laparoscopic Adrenal-Sparing Surgery?

BACKGROUND AND OBJECTIVE: Adrenal tissue-sparing or partial adrenalectomy evolved initially for patients with bilateral synchronous adrenal surgical pathology to preserve vital adrenal volume. In the laparoscopic era, the exact criteria for performing such procedures laparoscopically have yet to be defined. Controversy exists regarding the importance of preserving the adrenal vein, main or accessory. The aim of this retrospective study was to present our short series of laparoscopic tissue-sparing adrenalectomies with vein preservation. Our main goal is not to support partial adrenalectomy as an alternative to total (this is already advocated by many surgeons) but to emphasize the vein-preserving technique. METHODS: Seven patients with peripherally located either aldosterone-producing adenomas (4 cases) or myelolipomas (4 cases) underwent laparoscopic lateral partial adrenalectomy. One patient harbored an aldosterone-producing adenoma and a myelolipoma as well. The main adrenal vein was identified and preserved in 6 patients and the accessory vein in one. RESULTS: No conversion to open adrenalectomy was necessary, and no perioperative morbidity or mortality occurred. Three adenoma patients are normotensive 44, 23, and 20 months postoperatively, while the fourth one's pressure is refractory. CONCLUSIONS: Surprisingly, total adrenalectomies preceded the partial ones, which is controversial compared with other procedures. Laparoscopic lateral partial adrenalectomy is a technically challenging tissue-sparing operation. Meticulous dissection allows preservation of the middle artery and main or accessory vein resulting in a functioning adrenal stump.     

Lymphoepithelioma-like carcinoma of the bladder: three cases with clinicopathological and p53 protein expression study

Lymphoepithelioma-like carcinoma of the bladder is an uncommon neoplasm, of which 49 cases have been described in the English literature, none of which has been studied for p53 protein expression. We studied three muscle-infiltrating cases of this tumor using immunohistochemical, in situ hybridization and polymerase chain reaction (PCR) methods. The three cases were positive for epithelial markers and negative for lymphoid antigens in the tumoral syncytial areas. The intensive infiltrate of small cells was negative for epithelial and positive for lymphoid markers. This population was mainly made up of cytotoxic T-lymphocytes, positive for TIA-1. p53 protein was intensely positive in more than 90% of the epithelial component nuclei, being negative in the lymphoid cells. PCR study did not show mutations on p53. Both lymphocytes and epithelium were negative for Epstein–Barr virus markers, such as the latent membrane protein and EBER (Epstein–Barr-encoded RNA). The prognosis was very good after radiotherapy and chemotherapy treatment, preserving the bladder despite the muscle infiltration. The presence of an intense cytotoxic T-lymphocyte population may be related to this good prognosis. Both aspects, p53 protein status and T-lymphoid population, had never been studied before in bladder lymphoepithelioma-like carcinoma.     

Hydrophobicity of Lipid-Conjugated siRNAs Predicts Productive Loading to Small Extracellular Vesicles

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Optimized Cholesterol-siRNA Chemistry Improves Productive Loading onto Extracellular Vesicles

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Candida esophagitis: species distribution and risk factors for infection

Although Candida albicans is the main cause of fungal esophagitis, other species such as C. tropicalis, C. krusei and C. stellatoidea have also been implicated. Several studies have identified risk factors for C. albicans esophagitis. However, data for non-C. albicans species is still sparse. The aim of this study was to determine the etiology of Candida esophagitis in our medical centre over an 18-month period. Additionally, we aimed to investigate predisposing conditions for esophageal candidosis caused by different Candida species. A total of 21,248 upper gastroscopies were performed in Santa Casa Complexo Hospitalar between January 2005 and July 2006. The prevalence of Candida esophagitis was 0.74% (n = 158). C. albicans caused the vast majority of infections (96.2%), followed by C. tropicalis (2.5%), C. lusitaniae (0.6%) and C. glabrata (0.6%). There were 81 women (51.3%) and 77 men (48.7%). No case of mixed infection occurred. Concomitant oral candidosis was documented for 10.8% (n = 17). Most of cases (55.1%) involved outpatients. Around one fifth of patients in our cohort had no identifiable risk factors for esophageal candidosis (20.8%). Since nearly all infections were caused by C. albicans we were not able to determine risk factors for esophagitis caused by other Candida species.