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Absence epilepsy is a form of generalized epilepsy commonly seen in children. The clinician is often presented with a patient whose electroencephalogram does not fit the typical absence pattern. The purpose of this study is to more closely examine both typical and atypical absence variants and their outcome. A retrospective chart review was performed on children diagnosed with absence epilepsy over the past 5 years at the University of Alberta. A total of 119 patients were reviewed. Patients were classified with typical or atypical absence seizures following International League Against Epilepsy criteria and electroencephalography (EEG) characteristics. Clinical seizure characteristics, magnetic resonance imaging (MRI), initial response to treatment, and outcome were examined. Seizure characteristics were similar in both the typical and atypical absence groups. Aura, complex automatisms, changes in tone, and incontinence were seen in both groups, although status epilepticus was found only in the atypical group. Associated comorbid conditions such as attention-deficit hyperactivity disorder (ADHD), learning disorders, and enuresis were found equally in both groups. Developmental delay was found more often in the atypical group. Of the typical group, 83% responded to an initial antiepileptic drug (either valproic acid or ethosuximide), whereas only 51% of the atypical group came under control. Remission at 2 years however, was similar between groups, with 76% of the typical group and 71% of the atypical group completely seizure free. Absence seizures in childhood, both typical and atypical, share similar clinical and electroencephalographic features and appear to be part of a continuum. Associated comorbid features such as ADHD, learning disorders, and developmental delay are also seen in both groups. The outcome for both types is excellent, although the atypical variants may be initially more difficult to control. 相似文献
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Rawad Rihani MD Isam Lataifeh MD Hadeel Halalsheh MD Ayad Ahmed Hussein MD Abdulhadi Al‐Zaben MD Fawzi Abdel‐Rahman MD Mahmoud Sarhan MD 《Pediatric blood & cancer》2010,55(3):580-582
A 7‐year‐old male with Fanconi Anemia who developed primary graft failure following one antigen‐mismatched unrelated cord blood transplantation and a nonradiation‐based conditioning, underwent a second hematopoietic stem cell transplantation (HSCT) from his 2‐loci mismatched haploidentical father, using a nonradiation‐based regimen, 79 days after the first HSCT. A sustained hematological engraftment was achieved at 9 days post‐second HSCT. At 15 months post‐second HSCT; the patient demonstrated normal blood counts, sustained donor chimerism, and no evidence of GVHD. Haploidentical HSCTs as primary or secondary sources of stem cells, with appropriate T‐cell depletion, may be a readily available option in the absence of HLA‐matched related or unrelated donors. Pediatr Blood Cancer. 2010;55:580–582. © 2010 Wiley‐Liss, Inc. 相似文献
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Complications of systemic rheumatic diseases frequently have protean manifestations and may present a diagnostic problem. Patients with connective tissue diseases and vasculitides may have dangerous or life threatening conditions, which must be recognised and treated promptly to prevent rapidly evolving morbidity and mortality. Knowledge of possible emergencies in the context of a defined rheumatic disease may aid in promoting a high index of suspicion and contribute significantly to the timely diagnosis of many potentially dangerous conditions. This review is written for the emergency room physician and discusses the early recognition of selected emergencies in the context of a defined rheumatic disease. 相似文献
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Tahan HA Huber DL Downey WT 《Lippincott's case management : managing the process of patient care》2006,11(1):4-22; quiz 23-4
The Commission for Case Manager Certification (CCMC) conducted its third case managers' role and functions study in 2004. The purpose of this research was to validate the currency and relevancy of the certified case manager examination. The results of this study are shared in 2 parts of an article. Part I discusses the process the CCMC used for the development of the Case Manager's Role and Functions Survey Instrument (CMRFSI). The research leads to the identification of 6 new essential functions and 6 new knowledge areas, which describe case management practice. These findings were based on the survey of a large national sample of practicing case managers. Part II continues the analysis of the survey results and focuses on identifying the empirical activity and knowledge domains of case management practice, using factor analysis. It also discusses the similarities and differences found among various subgroups of case managers who were compared on the basis of certain demographic variables. In addition, it summarizes future changes in the field of case management as perceived by those who participated in the study. 相似文献
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Mohamed Hamed Hussein Ghada A. Daoud Hiroki Kakita Shin Kato Tatenobu Goto Michi Kamei Kenji Goto Yasuhiko Ozaki Tetsuya Ito Sumio Fukuda Ineko Kato Satoshi Suzuki Takashi Hashimoto Hajime Togari 《Pediatric surgery international》2010,26(2):187-193