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排序方式: 共有110条查询结果,搜索用时 15 毫秒
1.
Saman K. Hashmi Katie Bergstrom Alison A. Bertuch Jenny M. Despotovic Eyal Muscal Fan Xia Weimin Bi Andrea Marcogliese Rosa Diaz 《Pediatric blood & cancer》2019,66(1)
Neutropenia in pediatric patients can be due to a variety of disorders. We describe two patients who underwent extensive evaluation over many years for arthralgias and moderate neutropenia of unclear etiology. Genetic testing identified a pathogenic variant in PSTPIP1 (proline‐serine‐threonine phosphatase‐interacting protein 1) in both patients. Markedly elevated inflammatory markers and zinc levels confirmed the rare diagnosis of PSTPIP1‐associated myeloid‐related proteinemia inflammatory (PAMI) syndrome, tailoring treatment. Neutropenia is common in patients with PAMI syndrome. Unique mutations seen in PAMI syndrome may account for the specific phenotypic features of this disorder. 相似文献
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Thrombopoietin Receptor Agonist Use in Children: Data From the Pediatric ITP Consortium of North America ICON2 Study 下载免费PDF全文
Cindy Neunert MD MSCS Jenny Despotovic DO MS Kristina Haley DO MCR Michele P. Lambert MD Kerri Nottage MD Kristin Shimano MD Carolyn Bennett MD Robert Klaassen MD FRCP Kimo Stine MD Alexis Thompson MD Yves Pastore MD Travis Brown BS Peter W. Forbes MA Rachael F. Grace MD MMSc 《Pediatric blood & cancer》2016,63(8):1407-1413
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Paola Bianchi Elisa Fermo Kimberly Lezon-Geyda Eduard J. van Beers Holmes D. Morton Wilma Barcellini Bertil Glader Satheesh Chonat Yaddanapudi Ravindranath Peter E. Newburger Nina Kollmar Jenny M. Despotovic Madeleine Verhovsek Mukta Sharma Janet L. Kwiatkowski Kevin H. M. Kuo Marcin W. Wlodarski Hassan M. Yaish Susanne Holzhauer Heng Wang Joachim Kunz Kathryn Addonizio Hasan Al-Sayegh Wendy B. London Oliver Andres Richard van Wijk Patrick G. Gallagher Rachael F. F. Grace 《American journal of hematology》2020,95(5):472-482
Pyruvate kinase (PK) deficiency is a rare recessive congenital hemolytic anemia caused by mutations in the PKLR gene. This study reports the molecular features of 257 patients enrolled in the PKD Natural History Study. Of the 127 different pathogenic variants detected, 84 were missense and 43 non-missense, including 20 stop-gain, 11 affecting splicing, five large deletions, four in-frame indels, and three promoter variants. Within the 177 unrelated patients, 35 were homozygous and 142 compound heterozygous (77 for two missense, 48 for one missense and one non-missense, and 17 for two non-missense variants); the two most frequent mutations were p.R510Q in 23% and p.R486W in 9% of mutated alleles. Fifty-five (21%) patients were found to have at least one previously unreported variant with 45 newly described mutations. Patients with two non-missense mutations had lower hemoglobin levels, higher numbers of lifetime transfusions, and higher rates of complications including iron overload, extramedullary hematopoiesis, and pulmonary hypertension. Rare severe complications, including lower extremity ulcerations and hepatic failure, were seen more frequently in patients with non-missense mutations or with missense mutations characterized by severe protein instability. The PKLR genotype did not correlate with the frequency of complications in utero or in the newborn period. With ICCs ranging from 0.4 to 0.61, about the same degree of clinical similarity exists within siblings as it does between siblings, in terms of hemoglobin, total bilirubin, splenectomy status, and cholecystectomy status. Pregnancy outcomes were similar across genotypes in PK deficient women. This report confirms the wide genetic heterogeneity of PK deficiency. 相似文献
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Predrag Erceg Mladen Davidovic Zorana Vasiljevic Predrag Mitrovic Vladan Vukcevic Sanja Zdravkovic Gordana Mihajlovic Nebojsa Despotovic Dragoslav P Milosevic 《Circulation journal》2007,71(10):1530-1533
BACKGROUND: Most studies have shown that early post-infarction angina (EPA) implies an unfavorable long-term prognosis among patients with acute myocardial infarction (AMI). However, some studies have failed to establish a link between the occurrence of EPA and increased mortality and recurrent infarction rates. METHODS AND RESULTS: In order to evaluate a long-term prognosis in patients with EPA, we assessed the 5-year prognosis of 80 patients with AMI by the presence or absence of EPA. During the 5-year follow up, the occurrence of death, cardiac death, recurrent infarction, unstable angina, heart failure, revascularization and cardiac events were recorded. A cardiac event was defined as an occurrence of any of the following events: cardiac death, recurrent infarction, unstable angina, heart failure and revascularization. Survival analysis showed no differences between patients with and without EPA in the probability of death (p=NS), cardiac death (p=NS), recurrent myocardial infarction (p=NS) and unstable angina (p=NS). Patients with EPA had a higher probability of developing cardiac events (p=0.0285) and undergoing revascularization procedures (p=0.0188). CONCLUSIONS: EPA increases the risk of patients developing cardiac events and undergoing revascularization procedures, and thereby implies a poor long-term prognosis for patients with AMI. 相似文献
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Aleksa Marković DDS PhD José Luís Calvo‐Guirado DDS PhD MSc Zoran Lazić DDS PhD Gerardo Gómez‐Moreno DDS PhD MSc Dejan Ćalasan DDS MSc Javier Guardia DDS PhD Snježana Čolic DDS PhD Antonio Aguilar‐Salvatierra DDS Bojan Gačić DDS PhD Rafael Delgado‐Ruiz DDS PhD MSc Bojan Janjić DDS MSc Tijana Mišić DDS 《Clinical implant dentistry and related research》2013,15(3):341-349
Purpose: The aim of this study was to investigate the relationship between surgical techniques and implant macro‐design (self‐tapping/non‐self‐tapping) for the optimization of implant stability in the low‐density bone present in the posterior maxilla using resonance frequency analysis (RFA). Materials and Methods: A total of 102 implants were studied. Fifty‐six self‐tapping BlueSkyBredent® (Bredent GmbH&Co.Kg®, Senden, Germany) and 56 non‐self‐tapping Standard Plus Straumann® (Institut Straumann AG®, Waldenburg, Switzerland) were placed in the posterior segment of the maxilla. Implants of both types were placed in sites prepared with either lateral bone‐condensing or with bone‐drilling techniques. Implant stability measurements were performed using RFA immediately after implant placement and weekly during a 12‐week follow‐up period. Results: Both types of implants placed after bone condensing achieved significantly higher stability immediately after surgery, as well as during the entire 12‐week observation period compared with those placed following bone drilling. After bone condensation, there were no significant differences in primary stability or in implant stability after the first week between both implant types. From 2 to 12 postoperative weeks, significantly higher stability was shown by self‐tapping implants. After bone drilling, self‐tapping implants achieved significantly higher stability than non‐self‐tapping implants during the entire follow‐up period. Conclusions: The outcomes of the present study indicate that bone drilling is not an effective technique for improving implant stability and, following this technique, the use of self‐tapping implants is highly recommended. Implant stability optimization in the soft bone can be achieved by lateral bone‐condensing technique, regardless of implant macro‐design. 相似文献
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Guang‐Shing Cheng Katherine E. Selwa Charles Hatt Sundaresh Ram Aleksa B. Fortuna Margaret Guerriero Ben Himelhoch Daniel McAree Timothy C. Hoffman Joseph Brisson Ryan Nazareno Kiernan Bloye Timothy D. Johnson Mats Remberger Jonas Mattsson Dharshan Vummidi Ella E. Kazerooni Vibha N. Lama Stefanie Galban Michael Boeckh Gregory A. Yanik Craig J. Galban 《American journal of transplantation》2020,20(8):2198-2205
Parametric response mapping (PRM) is a novel computed tomography (CT) technology that has shown potential for assessment of bronchiolitis obliterans syndrome (BOS) after hematopoietic stem cell transplantation (HCT). The primary aim of this study was to evaluate whether variations in image acquisition under real‐world conditions affect the PRM measurements of clinically diagnosed BOS. CT scans were obtained retrospectively from 72 HCT recipients with BOS and graft‐versus‐host disease from Fred Hutchinson Cancer Research Center, Karolinska Institute, and the University of Michigan. Whole lung volumetric scans were performed at inspiration and expiration using site‐specific acquisition and reconstruction protocols. PRM and pulmonary function measurements were assessed. Patients with moderately severe BOS at diagnosis (median forced expiratory volume at 1 second [FEV1] 53.5% predicted) had similar characteristics between sites. Variations in site‐specific CT acquisition protocols had a negligible effect on the PRM‐derived small airways disease (SAD), that is, BOS measurements. PRM‐derived SAD was found to correlate with FEV1% predicted and FEV1/ forced vital capacity (R = ?0.236, P = .046; and R = ?0.689, P < .0001, respectively), which suggests that elevated levels in the PRM measurements are primarily affected by BOS airflow obstruction and not CT scan acquisition parameters. Based on these results, PRM may be applied broadly for post‐HCT diagnosis and monitoring of BOS. 相似文献
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