bluuebunny/crossref_metadata_2025_split huggingface dataset:
Safe and Effective Use of the Ketogenic Diet in Children with Epilepsy and Mitochondrial Respiratory Chain Complex Defects
Authors: Kang Hoon‐Chul , Lee Young‐Mock , Kim Heung Dong , Lee Joon Soo , Slama Abdelhamid 
Published: December 2006

Summary:  Purpose: To evaluate the clinical efficacy and safety of the ketogenic diet (KD) for patients with intractable childhood epilepsy and mitochondrial respiratory chain (RC) complex defects. Methods: A retrospective analysis evaluated outcomes in 14 children with intractable epilepsy and RC complex defects who were treated with the classic KD involving a 4:1 lipid to nonlipid ratio (% by weight), but without an initial fast and fluid restriction. Outcome measures included seizure frequency, electroencephalography (EEG) findings, the number of antiepileptic drugs, and adverse reactions. Results: Of the 14 patients, 9 had Complex I defects, 1 had a Complex II defect, 3 had Complex IV defects, and 1 had combined Complex I and IV defects. Two patients with Complex IV defects showed clinical progress compatible with the Leigh disease. The epileptic diagnoses were as follows: 5 patients were diagnosed with infantile spasms, 4 with the Lennox–Gastaut syndrome, 1 with the Landau–Kleffner syndrome, 1 with nonspecific generalized seizure disorder, and 3 with partial seizure disorder. The study found that 7 patients became seizure‐free after commencing the KD, three of whom successfully completed the diet without relapse. One patient with a greater than 90% seizure reduction, and 2 patients with seizure reductions between 50% and 90%, remained on the diet. Four patients, including two diagnosed with the Leigh disease, did not show any favorable responses to the diet or ceased the diet due to complications. Conclusions: The KD was a safe and effective therapy for seizures in children with intractable epilepsy and RC complex defects.
The Epileptology of John Thompson Dickson (1841–1874)
Authors: Eadie Mervyn 
Published: December 2006

Summary:  Purpose: To document John Thompson Dickson's interpretation of epileptogenesis, published between 1869 and 1874, and to compare it with John Hughlings Jackson's contemporaneous thought. Method: Examination of the relevant contemporary English language medical literature. Results: Dickson probably built on Samuel Wilks's interpretation of epileptic seizure mechanisms to develop a reasonably comprehensive hypothesis of epileptogenesis that was in advance of Jackson's thought on the matter, and was in many respects consistent with present‐day concepts, at least in relation to the focal epilepsies. Conclusion: Dickson's untimely death probably prevented his concept of epileptogenesis from being more widely appreciated.
Use of a Modified Atkins Diet in Intractable Childhood Epilepsy
Authors: Kang Hoon‐Chul , Lee Hyun Sug , You Su Jeong , Kang Du Cheol , Ko Tae‐Sung , Kim Heung Dong 
Published: December 2006

Summary:  Purpose: To evaluate the efficacy, safety and tolerability of a modified Atkins diet in intractable childhood epilepsy. Methods: Fourteen children with epilepsy were treated prospectively with a modified Atkins diet. Outcome measures included seizure frequency, adverse reactions and tolerability to the diet; blood β‐hydroxybutyrate and urine ketones were also measured. Results: Six months after diet initiation, seven (50%) remained on the diet, five (36%) had >50% seizure reduction, and three (21%) were seizure free. The diet was well tolerated by 12 (86%) patients. Most complications were transient and were successfully managed by careful follow‐up and conservative strategies. A consistently strong ketosis (β‐hydroxybutyrate of >3 mmol/L) seemed to be important for maintaining the efficacy of the diet therapy. Conclusions: The modified Atkins diet was well tolerated and sometimes a modified Atkins diet can be substituted for the conventional ketogenic diet. Serious complications were rare, but long‐term complications remain to be determined.
Thrombocytopenia in association with splenomegaly during granulocyte–colony‐stimulating factor treatment in mice is not caused by hypersplenism and is resolved spontaneously
Authors: Takamatsu Yasushi , Jimi Shiro , Sato Tomohito , Hara Shuuji , Suzumiya Junji , Tamura Kazuo 
Published: December 2006

BACKGROUND: Granulocyte–colony‐stimulating factor (G‐CSF) is used for hematopoietic progenitor cell (HPC) mobilization. Platelet (PLT) counts decrease during G‐CSF administration. The mechanisms have not been determined, however. Because splenic pooling of PLTs caused thrombocytopenia in patients with splenomegaly and splenic enlargement was observed in G‐CSF–treated donors, it was hypothesized that hypersplenism might cause G‐CSF–induced thrombocytopenia.STUDY DESIGN AND METHODS: Mice were treated with several concentrations of G‐CSF, and PLT count was measured. Because transfused PLTs should be cleared rapidly from the blood stream under hypersplenic state, PLT life span was studied. To determine direct role of spleen on thrombocytopenia, G‐CSF was given to splenectomized mice. Because PLT count did not decrease in G‐CSF–expressing transgenic mice, G‐CSF was given to mice for a longer period of time and PLT count was investigated.RESULTS: PLT counts decreased while spleen weight increased in a dose‐dependent manner by G‐CSF treatment. No significant difference in PLT life span was found between G‐CSF–treated and control mice. Histologic analysis showed no significant increase in PLT numbers trapped in either spleen or other tissue after PLT transfusion in G‐CSF–treated mice. In splenectomized mice as well as in normal mice, G‐CSF caused thrombocytopenia. When G‐CSF was given to mice for a longer period of time, PLT counts decreased during the first 7 days and thereafter began to increase followed by returning to baseline on Day 15.CONCLUSION: Thrombocytopenia coincided with splenomegaly during G‐CSF treatment, but hypersplenism was not responsible for thrombocytopenia. G‐CSF–induced thrombocytopenia was a transient event and improved spontaneously despite continual G‐CSF treatment.
Platelet function in whole‐blood donors is impaired: the effects of painkillers
Authors: Curvers Joyce , Dielis Arne W.J.H. , Heeremans Judith , Van Wersch Jan W.J. 
Published: December 2006

BACKGROUND: Aspirin (ASA) or non–aspirin‐like nonsteroidal anti‐inflammatory drugs (NSAIDs) influence platelet (PLT) function by inhibiting cyclooxygenase enzymes. In this study, the aim was to address the use of ASA or NSAIDs before donation and the effect on PLT function.STUDY DESIGN AND METHODS: Donors were asked questions about recent use of ASA or NSAIDs. Furthermore, PLT function was evaluated by measurement of the closure time (CT) in a PLT function analyzer (PFA‐100, Dade Behring) and by aggregometry (response to ADP or arachidonic acid [AA]).RESULTS: Of 100 questioned donors, 22 percent had used ASA (n = 4), NSAIDs (n = 6), or paracetamol (n = 12) before donation. Upon assessment of the PLT function in the PFA‐100, 27 donors showed values of greater than 180 seconds, indicative of impaired PLT function. Of these, only 7 had used pain killers before donation. Furthermore, 15 of 22 users had normal CTs. Aggregation after stimulation with AA was absent in 33 PLT‐rich samples. Again only 8 had reported use of ASA (3), NSAIDs (1), or paracetamol (4). Of the 22 users, 14 had normal AA aggregation responses. All donor samples showed ADP‐induced aggregation, indicating PLT integrity. There was no difference between the group of donors who reported the intake of ASA or NSAIDs and the group of donors who did not with respect to the tested PLT function assays.CONCLUSION: It is concluded that there is a considerable group of donors that use PLT‐influencing medication before donation. A relation between the reported use and impaired PLT function in blood donors could not be established, however. Impaired PLT function as tested may have other causes than intake of ASA or NSAIDs.
Evaluation of a new enzyme‐linked immunosorbent assay for detection of Chagas antibody in US blood donors
Authors: Tobler Leslie H. , Contestable Paul , Pitina Lubor , Groth Holly , Shaffer Shirley , Blackburn Gary R. , Warren Harold , Lee Stephen R. , Busch Michael P. 
Published: December 2006

BACKGROUND: Chagas disease, caused by the parasite Trypanosoma cruzi, represents a serious blood safety problem due to increasing immigration from Latin America. The Food and Drug Administration recently recommended implementation of Chagas antibody screening for US donors as soon as a suitable assay is licensed. An anonymized preclinical study of a prototype T. cruzi lysate‐based enzyme‐linked immunosorbent assay (ELISA) developed by Ortho‐Clinical Diagnostics was conducted.STUDY DESIGN AND METHODS: Two populations of specimens were evaluated: 1) 10,192 sequential donations from blood donors residing in the El Paso, Texas, area and 2) 178 specimens from South America which were presumptively positive for antibodies to T. cruzi and purchased from commercial vendors.RESULTS: A total of 10,189 (99.97%) of the 10,192 screened donor specimens did not react, whereas 3 (0.03%) tested initially reactive. The 3 initially reactive specimens tested repeat reactive and were confirmed by radioimmunoprecipitation analysis (RIPA). Based on antibody profile analysis, 2 of the 3 Chagas‐positive specimens were from the same donor. Observed specificity of the test was therefore 100 percent. Of the specimens from South America, 173 of 178 were reactive by the prototype ELISA. Of the 5 nonreactive specimens, all did not react by indirect fluorescence assay, but 4 were positive by RIPA. Therefore, calculated sensitivity of the ELISA was 97.7 percent (173/177).CONCLUSIONS: These studies indicate that the prototype ELISA has excellent sensitivity and specificity for detection of antibodies to T. cruzi in donors. Moreover, among donations from a geographically selected collection region of the United States, observed seroprevalence was 0.03 percent.
Predictors of response and relapse in a cohort of adults with thrombotic thrombocytopenic purpura‐hemolytic uremic syndrome: a single‐institution experience
Authors: Tuncer Hande H. , Oster Robert A. , Huang Shu T. , Marques Marisa B. 
Published: December 2006

BACKGROUND: Thrombotic thrombocytopenic purpura‐hemolytic uremic syndrome (TTP‐HUS) is a diagnosis of exclusion when a patient presents with the sine qua non findings of thrombocytopenia and microangiopathic hemolytic anemia without an identifiable cause. Although most patients respond to therapeutic plasma exchange (TPE), a significant number of patients relapse. The aim was to determine if clinical, laboratory, and/or treatment features could predict response and/or relapse.STUDY DESIGN AND METHODS: This study was a retrospective review of adults with TTP‐HUS treated with TPE at our institution from January 1996 to February 2004.RESULTS: The study population consisted of 90 patients (69% female) with mean age of 45 years and mostly obese (65%). The majority of cases were considered idiopathic. Ten patients died (11%) from the disease before achieving a response, whereas 79 percent were considered responders. Obesity and severe anemia at presentation were predictors of response to TPE (p = 0.0126 and p = 0.0071, respectively). Among the responders, 28 percent relapsed in a median of 14 months. Male sex, severe thrombocytopenia (mean ± SD, 13 × 109 ± 8 × 109/L), and higher lactate dehydrogenase pre‐/posttreatment ratio were associated with relapse (p values of 0.0141, 0.0199, and 0.0407, respectively). ADAMTS‐13 values were not obtained on enough number of patients to provide important data.CONCLUSION: Although patient and laboratory characteristics associated with response and relapse were identified, there was significant overlap between patient groups. Thus, our findings offer preliminary evidence and do not yet justify short‐ or long‐term changes in the management of patients with TTP‐HUS.
Evaluation of platelet activation and cytokine release during storage of platelet concentrates processed from buffy coats either manually or by the automated OrbiSac system
Authors: Vetlesen Annette , Mirlashari Mohammad Reza , Ezligini Farshid , Kjeldsen‐Kragh Jens 
Published: December 2006

BACKGROUND: This study aimed to compare platelet (PLT) quality during storage of buffy coat (BC) PLT concentrates (PCs), prepared either manually or by the automated OrbiSac system (Gambro BCT).STUDY DESIGN AND METHODS: Following overnight storage at 20 to 22°C, five BCs were pooled with 300 mL of PLT additive solution. Twenty‐one PCs were produced manually (M‐PCs) and 21 by the automated OrbiSac system (A‐PCs). Swirling, PLT count, mean PLT volume, blood gas analyses, potassium, glucose, and lactate were assessed. Expression of the activation markers CD42a, CD62P, and PAC‐1 was analyzed by flow cytometry on resting PLTs and PLTs stimulated with thrombin receptor agonist peptide (TRAP). Levels of CCL5 and transforming growth factor‐β1 (TGF‐β1) were measured by enzyme‐linked immunosorbent assay.RESULTS: The A‐PCs had significantly larger volume and higher PLT yield, PLT recovery, and white blood cell concentration than the M‐PCs, whereas the red blood cell content was significantly highest in the M‐PCs. pH levels were between 6.9 and 7.2 in all PCs. Neither glucose consumption nor lactate production differed significantly over time. A‐PCs had, compared to M‐PCs, significantly higher expression of CD62P on resting PLTs, lower capacity for up regulating CD62P on TRAP‐stimulated PLTs, and higher levels of CCL5 during storage. TRAP‐stimulated A‐PCs had a significantly higher potential for down regulation of CD42a than M‐PCs. No difference was found in TGF‐β1 levels or TRAP‐induced up regulation of PAC‐1.CONCLUSION: The levels of CCL5 and the expression of CD62P in resting and stimulated PLTs indicate that PLTs in A‐PCs are slightly more activated than in M‐PCs, but the clinical importance of this finding is yet unknown.
Assessment of education and computerized decision support interventions for improving transfusion practice
Authors: Rothschild Jeffrey M. , McGurk Siobhan , Honour Melissa , Lu Linh , McClendon Aubre A. , Srivastava Priya , Churchill W. Hallowell , Kaufman Richard M. , Avorn Jerry , Cook E. Francis , Bates David W. 
Published: December 2006

BACKGROUND: Overuse of blood products is common, but prior efforts to improve transfusion decisions have met with limited success.STUDY DESIGN AND METHODS: This study examines transfusion practices before and after a conventional educational intervention followed by a randomized controlled trial of a decision support (DS) intervention with computerized physician order entry (CPOE) for red blood cell, platelet, and fresh‐frozen plasma orders. The study was conducted in an academic medical center between April 2003 and June 2004. Orders originating from units not using CPOE with DS (e.g., the emergency department) were excluded. Junior housestaff were randomly assigned into a control group and an intervention group who received DS for transfusion orders. Transfusion orders were initially classified according to guideline rules as DS‐agree or DS‐disagree. Chart reviews assessed inappropriateness for all DS‐disagree orders and a sample of DS‐agree orders. The total of inappropriate transfusion orders included chart review confirmed DS‐disagree orders and DS‐agree orders reclassified as inappropriate.RESULTS: The percentages of inappropriate nonemergent transfusion orders during the baseline phase for the entire staff and randomly assigned junior housestaff were 72.6 percent (2154/2967) and 71.9 percent (1259/1752) and improved after conventional education to 63.8 percent (1699/2663; p < 0.0001) and 63.3 percent (1263/1996; p < 0.0001), respectively. The percentage of inappropriate orders in the DS intervention group continued to improve (59.6%, 804/1350; p < 0.0001). Physicians accepted 14 percent (133/939) of new DS‐recommended orders, especially recommendations to increase transfusion doses (73%).CONCLUSIONS: Education and computerized DS both decreased the percentage of inappropriate transfusions, although the residual amount of inappropriate transfusions remained high.
Inactivation of Orientia tsutsugamushi in red blood cells, plasma, and platelets with riboflavin and light, as demonstrated in an animal model
Authors: Rentas Francisco , Harman Ronald , Gomez Charlotte , Salata Jeanne , Childs Joseph , Silva Tonya , Lippert Lloyd , Montgomery Joshua , Richards Allen , Chan Chye , Jiang Ju , Reddy Heather , Li John , Goodrich Raymond 
Published: December 2006

BACKGROUND: Treatment of blood products with riboflavin and light has been used to reduce the number of certain pathogens. Orientia (formerly Rickettsia) tsutsugamushi, the scrub typhus agent, is an obligate intracellular bacterium that grows free in the cytoplasm of infected cells. This study evaluated the capability of riboflavin and light to inactivate O. tsutsugamushi in red blood cells (RBCs), platelets (PLTs), and plasma, as measured by mouse infectivity.STUDY DESIGN AND METHODS: A total of 108 mice, equally divided into groups receiving RBCs, plasma, and PLTs, received untreated products infected with 100 to 105 organisms. Eighteen mice received products infected with 105 organisms and were subsequently treated with riboflavin and light. Mice were monitored daily for up to 17 days for signs and symptoms of infection (e.g., lethargy, labored breathing, rough coat) and killed upon appearance of symptoms or on Day 17 after infection. Real‐time polymerase chain reaction (PCR) on blood and Giemsa stains from peritoneal exudates were performed.RESULTS: A total of 102 of 108 mice receiving the untreated products developed signs and symptoms of infection and had positive PCR and Giemsa stain results. None of the 18 animals receiving riboflavin and light–treated blood products exhibited signs or symptoms of infection, nor was infection observed by PCR testing or Giemsa staining.CONCLUSIONS: Riboflavin and light are effective in reducing O. tsutsugamushi. Mice injected with blood products inoculated with 105 organisms and treated with riboflavin and light did not experience any signs or symptoms of infection, 17 days after inoculation. A 5‐log reduction of this organism in blood was achieved as assayed in an animal model.
Challenges and Solutions for Difficult Implantations of CRT Devices: The Role of New Technology and Techniques
Authors: SHEPARD RICHARD K. , ELLENBOGEN KENNETH A. 
Published: December 2006

Introduction: Cardiac resynchronization therapy (CRT) can markedly improve heart failure treatment in selected patients, but left ventricular (LV) lead implantation can be difficult or optimal lead position not obtainable. Methods: This review examines newer techniques and technologies to optimize implant success. Goals of implant include placement of the LV lead in a coronary vein near the midlateral left ventricle with good lead stability, adequate thresholds, and no phrenic nerve stimulation. We will examine methods to access difficult and tortuous veins, test multiple veins for the best pacing site, and avoid complications. Newer technologies that will be discussed include magnetic wire navigation using Stereotaxis™, endoscopic, and CT‐guided approaches, and nontraditional methods such as pericardial puncture for epicardial LV lead placement. Conclusion: The methods discussed here may help to make CRT implant safer, faster, and more successful.
Corneal flap thickness with the Moria M2 single‐use head 90 microkeratome
Authors: Huhtala Anne , Pietilä Juhani , Mäkinen Petri , Suominen Sakari , Seppänen Matti , Uusitalo Hannu 
Published: December 2006

Abstract.Purpose:  To analyse the accuracy of corneal flap thickness created in laser‐assisted in situ keratomileusis (LASIK) using the Moria Model 2 (M2) single‐use head 90 microkeratome.Methods:  The corneal thickness of 300 (266 myopic and 34 hyperopic) eyes of 150 patients was measured by ultrasonic pachymetry preoperationally and intraoperationally after flap cut. The Moria M2 single‐use head 90, intended to create a flap with a thickness of 120 µm, was used in all eyes. The right eye was always operated first and the left eye second, using the same blade.Results:  Mean corneal flap thickness was 115.4 µm (standard deviation [SD] 12.5) in the two eyes, 115.7 µm (SD 12.4, range 73–147 µm) in right eyes and 115.1 µm (SD 12.6, range 74–144 µm) in left eyes. Mean horizontal flap diameter was 9.1 mm (SD 0.2) and mean hinge length 4.1 mm (SD 0.1). There were no free flaps, incomplete flaps or flaps with buttonholes in the study. Occasional iron particles were observed in three (1.0%) eyes.Conclusions:  As with most microkeratomes, the single‐use head 90 microkeratome cut thinner flaps than were intended. The range of the cuts was relatively wide. However, thin flaps did not increase the rate of flap‐related complications. The difference between the first and second eyes was not significant.
Stroma remodelling during healing of corneal surface irregularities induced by PTK
Authors: Panagiotopoulos Marios , Gan Lisha , Fagerholm Per 
Published: December 2006

Abstract.Purpose:  To study the histopathology of the remodelling process in the stroma after excimer‐laser‐induced corneal irregular injuries.Methods:  Seven New Zealand white rabbits received in one eye a transepithelial plano photoablation (60 µm) and an additional plano ablation (25 µm). On the denuded stroma, an electron microscopy specimen grid was placed and another 25 µm ablation was applied to produce surface irregularities. Dichlorotriazinyl aminofluorescein (DTAF) was then applied for 45 seconds. Another seven right eyes of seven rabbits were ablated the same way but without using the grid, resulting in a plano ablation. All the rabbits were killed at weekly intervals after treatment. The harvested corneas from both eyes were further processed for haematoxylin‐eosin staining and were also stained with monoclonal antibodies directed against Ki‐67 antigen and α‐smooth muscle actin (α‐SMA). All specimens were examined under light and fluorescence microscope.Results:  The corneal wounds were covered by epithelium during the first week. The 25 µm × 25 µm × 25 µm stromal irregularities were clearly discernible up to 3 weeks after treatment, during which time they melted and disappeared. A homogeneous zone was formed in which stroma cells laid down an initially disorganized stroma. This was sharply visible under a fluorescence microscope as a dark area between the dichlorotriazinyl aminofluorescein (DTAF) fluorescent stroma and autofluorescent epithelium. Very little response was seen in the plano‐ablated wound microscopically and in terms of positive stained cells.Conclusion:  As the irregularities are flattened and the homogenous zone becomes repopulated with keratocytes forming extracellular matrix material (ECM), the cornea regains its previous architecture in both groups. The irregular wound surface promotes wound‐healing reactivity, a process that allows the cornea to compensate for the irregularities and heal to a functional state.