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Selected AbstractsLong-term use of the ketogenic diet in the treatment of epilepsyDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 12 2006Darcy K Groesbeck BS Long-term outcomes of the ketogenic diet in the treatment of epilepsy have not previously been reported. A retrospective chart review of children treated with the ketogenic diet for more than 6 years at the Johns Hopkins Hospital was performed. The response was documented at clinic visits and by telephone contacts; laboratory studies were obtained approximately every 6 to 12 months. Satisfaction and tolerability were assessed by means of a brief parental telephone questionnaire. In all, 28 patients (15 males, 13 females), currently aged 7 to 23 years, were identified. The median baseline seizure frequency per week at diet onset was 630 (range 1,1400). Diet duration ranged from 6 to 12 years; 19 remain on the diet currently. After 6 years or more, 24 children experienced a more than 90% decrease in seizures, and 22 parents reported satisfaction with the diet's efficacy. Ten children were at less than the 10th centile for height at diet initiation; this number increased to 23 at the most recent follow-up (p=0.001). Kidney stones occurred in seven children and skeletal fractures in six. After 6 years or more the mean cholesterol level was 201mg/dl, high-density lipoprotein was 54mg/dl, low-density lipoprotein was 129mg/dl, and triglycerides were 97mg/dl. Efficacy and overall tolerability for children are maintained after prolonged use of the ketogenic diet. However, side effects, such as slowed growth, kidney stones, and fractures, should be monitored closely. [source] Long-term outcomes of children treated with the ketogenic diet in the pastEPILEPSIA, Issue 7 2010Amisha Patel Summary Purpose:, The ketogenic diet has well-established short- and long-term outcomes for children with intractable epilepsy, but only for those actively receiving it. However, no information exists about its long-term effects years after it has been discontinued. Methods:, Living subjects were identified who were treated at the Johns Hopkins Hospital with the ketogenic diet from November 1993 to December 2008 for ,1 month, and had discontinued it ,6 months prior to this study. Of 530 patients who were eligible, 254 were successfully contacted by phone or e-mail with a survey and request for laboratory studies. Results:, Questionnaires were completed by 101 patients, with a median current age of 13 years (range 2,26 years). Median time since discontinuing the ketogenic diet was 6 years (range 0.8,14 years). Few (8%) still preferred to eat high fat foods. In comparison to the 52% responder rate (>50% seizure reduction) at ketogenic diet discontinuation, 79% were now similarly improved (p = 0.0001). Ninety-six percent would recommend the ketogenic diet to others, yet only 54% would have started it before trying anticonvulsants. Lipids were normal (mean total cholesterol 158 mg/dl), despite most being abnormal while on the ketogenic diet. The mean Z scores for those younger than age 18 years were ,1.28 for height and ,0.79 for weight. In those 18 years of age or older, the mean body mass index (BMI) was 22.2. Discussion:, This is the first study to report on the long-term effects of the ketogenic diet after discontinuation. The majority of subjects are currently doing well with regard to health and seizure control. [source] Solifenacin-induced small bowel pseudo-obstructionJOURNAL OF HOSPITAL MEDICINE, Issue 2 2008Naveen Pemmaraju MD Abstract An 89-year-old woman was admitted to Johns Hopkins Hospital with a small bowel obstruction and symptoms of urinary retention. She had been started on solfenacin for bladder overactivity 10 days prior to her presentation. Withdrawal of the solfenacin resulted in a full recovery, which has persisted for greater than 6 months without surgical intervention. This is the first reported case report of small bowel pseudo-obstruction due to solifenacin. Journal of Hospital Medicine 2008;3:176,178. © 2008 Society of Hospital Medicine. [source] Chromosome 8q24 risk variants in hereditary and non-hereditary prostate cancer patients,THE PROSTATE, Issue 5 2008Jielin Sun Abstract Background Multiple variants in three regions at 8q24 are consistently found to be associated with prostate cancer (PCa) risk in population-based association studies. The role that these variants may play in familial prostate cancer risk has not been extensively investigated. Methods We evaluated 12 SNPs at three 8q24 regions using population-based association and family-based linkage and association methods in hereditary PCa (HPC) probands and their families, non-HPC patients, and unaffected screened controls, all recruited at Johns Hopkins Hospital. Results For multiple variants in Region 1 (e.g., rs1447295) and Region 2 (e.g., rs16901979), we found statistically significantly higher frequencies of previously identified risk alleles and genotypes in HPC probands than in unaffected controls. Furthermore, in Region 2 the risk alleles were statistically significantly more frequent in HPC probands than in non-HPC patients. Family-based transmission tests found risk alleles of SNPs in Region 2, but not in Regions 1 and 3, were significantly over-transmitted to affected men in these families. We found little evidence supporting PCa linkage at 8q24 in 168 HPC families, in part explained by the observation of multiple, different risk allele-containing haplotypes segregating in the vast majority of these families. Conclusions Our study further supports the presence of PCa susceptibility loci at 8q24, particular at Region 2, and also provides evidence that these SNPs play an important role in familial prostate cancer. Large family-based studies are needed to confirm our novel findings. Prostate 68: 489,497, 2008. © 2008 Wiley-Liss, Inc. [source] Use of Quantitative Broad-based Polymerase Chain Reaction for Detection and Identification of Common Bacterial Pathogens in Cerebrospinal FluidACADEMIC EMERGENCY MEDICINE, Issue 7 2010Richard Rothman MD ACADEMIC EMERGENCY MEDICINE 2010; 17:741,747 © 2010 by the Society for Academic Emergency Medicine Abstract Background:, Conventional laboratory diagnosis of bacterial meningitis based on microscopy followed by culture is time-consuming and has only moderate sensitivity. Objectives:, The objective was to define the limit of detection (LOD), analytic specificity, and performance characteristics of a broad-based quantitative multiprobe polymerase chain reaction (PCR) assay for rapid bacterial detection and simultaneous pathogen-specific identification in patients with suspected meningitis. Methods:, A PCR algorithm consisting of initial broad-based detection of Eubacteriales by a universal probe, followed by pathogen identification using either pathogen-specific probes or Gram-typing probes, was employed to detect pathogens. The 16S rRNA gene, which contains both conserved and variable regions, was chosen as the target. Pathogen-specific probes were designed for Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae, Staphylococcus epidermidis, Staphylococcus aureus, Escherichia coli, and Listeria monocytogenes. Gram-positive and -negative typing probes were designed based on conserved regions across all eubacteria. The LOD and time to detection were assessed by dilutional mocked-up samples. A total of 108 convenience cerebrospinal fluid (CSF) clinical samples obtained from the Johns Hopkins Hospital (JHH) microbiology laboratory were tested, and results were compared with hospital microbiologic culture reports. Results:, The LOD of the assay ranged from 101 to 102 colony-forming units (CFU)/mL. Pathogen-specific probes showed no cross-reactivity with other organisms. Time to detection was 3 hours. In clinical specimens, the universal probe correctly detected 16 of 22 culture-positive clinical specimens (sensitivity = 72.7%; 95% confidence interval [CI] = 49.8% to 89.3%), which were all correctly characterized by either pathogen-specific or Gram-typing probes. Adjusted sensitivity after removing probable microbiologic laboratory contaminants was 88.9% (95% CI = 65.3% to 98.6%). The universal probe was negative for 86 of 86 culture-negative specimens. Conclusions:, A broad-based multiprobe PCR assay demonstrated strong analytic performance characteristics. Findings from a pilot clinical study showed promise in translation to human subjects, supporting potential utility of the assay as an adjunct to traditional diagnostics for early identification of bacterial meningitis. [source] The use of three-dimensional computed tomography for assessing patients before laparoscopic adrenal-sparing surgeryBJU INTERNATIONAL, Issue 5 2006Michael Mitterberger OBJECTIVE To evaluate the efficacy of three-dimensional computed tomography (3D-CT) in delineating the relationship of the adrenal mass to adjacent normal structures in preparation for laparoscopic partial adrenalectomy. PATIENTS AND METHODS Multislice CT (1 mm slices, 0.5 s rotation time) was used to evaluate 12 patients before adrenal-sparing surgery for aldosterone-producing adenoma or phaeochromocytoma. The CT data were reconstructed using two rendering techniques; (i) volume rendering with the modified VOLREN software (Johns Hopkins Hospital, Baltimore, MD, USA) which allowed interactive 3D examination of the whole data volume within a few minutes; (ii) surface representations only of the interesting structures (kidney, adrenal tumour, vessels) represented in different colours and depicted together in a 3D scene using the software package 3DVIEWNIX. RESULTS In all, 14 adrenal masses in 12 patients were evaluated with 3D-CT; the number and location of lesions was accurate in all cases with both rendering techniques. The coloured surface-rendered images showed a consistently better delineation of the adrenal tumour from the normal tissue than did the volume-rendering technique. From this information all laparoscopic partial adrenalectomies could be completed as planned. CONCLUSIONS Interactive visualization of volume-rendered CT images was helpful for the planning and successful performance of the procedure, but coloured surface-rendered CT provided more convenient, immediate and accurate intraoperative information. [source] |