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181.
The occurrence of a single median maxillary central incisor (SMMCI) is a very rare condition and might be a sign of a more severe midline defect, which could be a mild degree of holoprosencephaly. Absence of the internasal and partial absence of the intermaxillary suture has been observed in a fetus with holoprosencephaly. The purpose of this study was to evaluate the intermaxillary suture, the eruption pattern of the single central incisor in the SMMCI condition, and the growth of the maxilla in a group of patients with SMMCI. A similar study was not found in the scientific literature. The material included orthopantomographs, dental radiographs, and lateral cephalometric radiographs from 11 patients with an SMMCI. The orthopantomographs and dental radiographs showed that the intermaxillary suture was abnormal anterior to the incisive foramen; however, the SMMCI erupted within the expected time interval. Superimposition on stable structures on lateral cephalometric radiographs from two untreated patients, in which growth analysis was possible, showed that the horizontal and vertical growth of the maxilla was normal. Due to the sutural midline defect it is suggested that a transversal growth analysis is included in all treatment planning of SMMCI patients.  相似文献   
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It is presently accepted that the KIT D816V mutation is detectable in tissues with neoplastic mast cells in most patients with indolent systemic mastocytosis. In this study, neoplastic mast cells were detected in bone marrow, but not in peripheral blood, by flow cytometry in all 25 included cases of indolent systemic mastocytosis. However, the KIT D816V mutation was detected using mutation-specific qPCR in both bone marrow and peripheral blood in all 25 cases, demonstrating for the first time that the KIT D816V mutation is consistently present in non-mast cells in indolent systemic mastocytosis and that these cells are circulating in peripheral blood.  相似文献   
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The acronym POEMS syndrome stands for a rare multi-system disorder, comprised of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Here, we present a single-center report of a series of five POEMS patients treated with melphalan high-dose therapy (HDT) with subsequent autologous blood stem cell transplantation (ABSCT). After a median follow-up of 52 months from time of diagnosis (range, 15-192) and a median follow-up of 18 months after ABSCT (range, 11-120), all patients were alive. Overall, no severe transplantation-associated complications such as engraftment syndrome or peri- or post-transplant death were noted. In two cases, HDT followed by ABSCT resulted in a complete hematologic response; in the additional three cases, partial responses (PR) were achieved including one very good hematologic PR. Only one patient with initial PR developed progressive disease nearly 2.5 years after transplantation. Consequently, a second HDT with ABSCT was successfully applied resulting in clinical improvement and hematologic PR. In line with previous single-center reports, melphalan HDT followed by ABSCT proved to be a first-line treatment option with tolerable side effects in severely affected POEMS patients with progressing symptoms.  相似文献   
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Aims To assess correlations and agreement between timeline follow‐back (TLFB)‐assisted self‐report and blood samples for cannabis use. Design Secondary analysis of a randomized trial. Setting Copenhagen, Denmark. Participants One hundred and three patients from the CapOpus trial with cannabis use disorder and psychosis, providing 239 self‐reports of cannabis use and 88 valid blood samples. Measurements Delta‐9‐tetrahydrocannabinol (THC), 11‐hydroxy‐delta‐9‐tetrahydrocannabinol (11‐OH‐THC) and 11‐nor‐delta‐9‐tetrahydrocannabinol‐9‐carboxylic acid (THC‐COOH) detected in plasma using high‐performance liquid chromatography with tandem mass spectrometry detection. Self‐report of cannabis‐use last month by TLFB. Pearson's r, sensitivity and specificity calculated as measures of correlation or agreement. Findings Correlations were strong; r = 0.75 for number of days and r = 0.83 for number of standard joints in the preceding month when excluding outliers. Including outliers, coefficients were moderate to strong (r = 0.49). There were differences in subgroups, mainly inconsistent, depending on inclusion or exclusion of outliers. Sensitivity and specificity for TLFB detecting the presence or absence of cannabis use were 95.7% [95% confidence interval (CI) 88.0–99.1%) and 72.2% (95% CI 46.5–90.3%), respectively. Using 19 days as cut‐off on TLFB, they were 94.3% (95% CI 86.0–98.4%) and 94.4% (95% CI 72.2–99.9%), respectively. Area under the receiver operating characteristic (ROC) curve was 0.96. Conclusions Timeline follow‐back (TLFB)‐assisted self‐report of cannabis use correlates highly with plasma‐delta‐9‐tetrahydrocannabinol in patients with comorbid cannabis use disorder and psychosis. Sensitivity and specificity of timeline follow‐back appear to be optimized with 19 days as the cut‐off point. As such, timeline follow‐back may be superior to analysis of blood when going beyond 19 days of recall.  相似文献   
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Background  

HIV-associated general immune activation is a strong predictor for HIV disease progression, suggesting that chronic immune activation may drive HIV pathogenesis. Consequently, immunomodulating agents may decelerate HIV disease progression.  相似文献   
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Graefe's Archive for Clinical and Experimental Ophthalmology - The purpose of this article is to review the literature on nomenclature, natural history, clinical features, diagnosis,...  相似文献   
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