Design and implementation of electronic health record common data elements for pediatric epilepsy: Foundations for a learning health care system.

OBJECTIVE: Common data elements (CDEs) are standardized questions and answer choices that allow aggregation, analysis, and comparison of observations from multiple sources. Clinical CDEs are foundational for learning health care systems, a data-driven approach to health care focused on continuous improvement of outcomes. We aimed to create clinical CDEs for pediatric epilepsy. METHODS: A multiple stakeholder group (clinicians, researchers, parents, caregivers, advocates, and electronic health record [EHR] vendors) developed clinical CDEs for routine care of children with epilepsy. Initial drafts drew from clinical epilepsy note templates, CDEs created for clinical research, items in existing registries, consensus documents and guidelines, quality metrics, and outcomes needed for demonstration projects. The CDEs were refined through discussion and field testing. We describe the development process, rationale for CDE selection, findings from piloting, and the CDEs themselves. We also describe early implementation, including experience with EHR systems and compatibility with the International League Against Epilepsy classification of seizure types. RESULTS: Common data elements were drafted in August 2017 and finalized in January 2020. Prioritized outcomes included seizure control, seizure freedom, American Academy of Neurology quality measures, presence of common comorbidities, and quality of life. The CDEs were piloted at 224 visits at 10 centers. The final CDEs included 36 questions in nine sections (number of questions): diagnosis (1), seizure frequency (9), quality of life (2), epilepsy history (6), etiology (8), comorbidities (2), treatment (2), process measures (5), and longitudinal history notes (1). Seizures are categorized as generalized tonic-clonic (regardless of onset), motor, nonmotor, and epileptic spasms. Focality is collected as epilepsy type rather than seizure type. Seizure frequency is measured in nine levels (all used during piloting). The CDEs were implemented in three vendor systems. Early clinical adoption included 1294 encounters at one center. SIGNIFICANCE: We created, piloted, refined, finalized, and implemented a novel set of clinical CDEs for pediatric epilepsy.

Functional Connectivity as a Potential Mechanism for Language Plasticity.

BACKGROUND AND OBJECTIVES: Task fMRI is a clinical tool for language lateralization, but has limitations, and cannot provide information about network-level plasticity. Additional methods are needed to improve the precision of presurgical language mapping. We investigate language resting-state functional connectivity (RS fMRI; FC) in typically developing children (TD) and children with epilepsy. Our objectives were to (1) understand how FC components differ between TD children and those with epilepsy; (2) elucidate how the location of disease (frontal/temporal epilepsy foci) affects FC; and (3) investigate the relationship between age and FC. METHODS: Our sample included 55 TD children (mean age 12 years, range 7-18) and 31 patients with focal epilepsy (mean age 13 years, range 7-18). All participants underwent RS fMRI. Using a bilateral canonical language map as target, vertex-wise intrahemispheric FC map and interhemispheric FC map for each participant were computed and thresholded at top 10% to compute an FC laterality index (FCLI; [(L - R)/(L + R)]) of the frontal and temporal regions for both integration (intrahemispheric FC; FCLIi) and segregation (interhemispheric FC; FCLIs) maps. RESULTS: We found FC differences in the developing language network based on disease, seizure foci location, and age. Frontal and temporal FCLIi was different between groups (t[84] = 2.82, p < 0.01; t[84] = 4.68, p < 0.01, respectively). Frontal epilepsy foci had the largest differences from TD (Cohen d frontal FCLIi = 0.84, FCLIs = 0.51; temporal FCLIi = 1.29). Development and disease have opposing influences on the laterality of FC based on groups. In the frontal foci group, FCLIi decreased with age (r = -0.42), whereas in the temporal foci group, FCLIi increased with age (r = 0.40). Within the epilepsy group, increases in right frontal integration FCLI relates to increased right frontal task activation in our mostly left language dominant group (r = 0.52, p < 0.01). Language network connectivity is associated with higher verbal intelligence in children with epilepsy (r = 0.45, p < 0.05). DISCUSSION: These findings lend preliminary evidence that FC reflects network plasticity in the form of adaptation and compensation, or the ability to recruit support and reallocate resources within and outside of the traditional network to compensate for disease. FC expands on task-based fMRI and provides complementary and potentially useful information about the language network that is not captured using task-based fMRI alone.

Functional connectivity hemispheric contrast (FC-HC): A new metric for language mapping.

Development of a task-free method for presurgical mapping of language function is important for use in young or cognitively impaired patients. Resting state connectivity fMRI (RS-fMRI) is a task-free method that may be used to identify cognitive networks. We developed a voxelwise RS-fMRI metric, Functional Connectivity Hemispheric Contrast (FC-HC), to map the language network and determine language laterality through comparison of within-hemispheric language network connections (Integration) to cross-hemispheric connections (Segregation). For the first time, we demonstrated robustness and efficacy of a RS-fMRI metric to map language networks across five groups (total N = 243) that differed in MRI scanning parameters, fMRI scanning protocols, age, and development (typical vs pediatric epilepsy). The resting state FC-HC maps for the healthy pediatric and adult groups showed higher values in the left hemisphere, and had high agreement with standard task language fMRI; in contrast, the epilepsy patient group map was bilateral. FC-HC has strong but not perfect agreement with task fMRI and thus, may reflect related and complementary information about language plasticity and compensation.

Using EHRs to advance epilepsy care.

The improved use of Electronic Health Record (EHR) Systems provides an opportunity to improve the overall efficiency and quality of care of patients with epilepsy. Tools and strategies that may be incorporated into the use of EHRs include utilizing patient generated data, clinical decision support systems and natural language processing systems. Standardization of data from EHR systems may lead to improvement in clinical research through the creation of data collections and multi-center collaborations. Challenges to collaborative use of EHR Systems across centers include costs and the diversity of EHR systems.

Role of soy protein with normal or reduced isoflavone content in reversing bone loss induced by ovarian hormone deficiency in rats.

Soy protein, a rich source of isoflavones, fed immediately after an ovariectomy prevents bone loss in rats. Reports of the effectiveness of natural and synthetic isoflavones in preventing or treating osteoporosis led us to examine the effect of soy protein in reversing established bone loss. Seventy-two 95-d-old female Sprague-Dawley rats were assigned to 6 groups. The rats were either sham operated (SHAM; 2 groups) or ovariectomized (OVX; 4 groups) and then fed a casein-based, semipurified diet. Thirty-five days after surgery, 1 SHAM and 1 OVX group were killed to examine the occurrence of bone loss. Thereafter, the other SHAM and 1 OVX groups continued to receive the casein-based diet. Whereas the remaining 2 OVX groups received diets in which casein was replaced by soy protein with normal (OVX+SOY) or reduced (OVX+SOY-) isoflavone content for 65 days. The OVX control group had significantly lower femoral and fourth lumbar vertebral bone densities than the SHAM group. Femoral density of rats fed SOY or SOY- diets were not significantly different from SHAM or OVX controls. This suggests a slight reversal of cortical bone loss that may be partially due to higher femoral insulin-like growth factor I mRNA transcripts resulting from both the SOY and SOY- diets. The ovariectomy-induced increases in indexes of bone turnover were not ameliorated by either of the soy diets, suggesting that any positive effect of soy was achieved through enhanced bone formation rather than slowed bone resorption. Long-term consumption of soy or its isoflavones may be needed to produce small but continued increments in bone mass.

Bone-sparing effect of soy protein in ovarian hormone-deficient rats is related to its isoflavone content.

Our previous studies showed that a soy-protein diet prevents ovariectomy-induced bone loss. The purpose of this study was to determine whether isoflavones in soy protein are responsible for this bone-protective effect. Forty-eight 95-d-old Sprague-Dawley rats were divided into 4 groups: sham-operated fed a casein-based diet (SHAM), ovariectomized fed a casein-based diet (OVX+CASEIN), ovariectomized fed soy protein with normal isoflavone content (OVX+SOY), and ovariectomized fed soy protein with reduced isoflavone content (OVX+SOY-). The OVX+SOY group had significantly greater femoral bone density (in g/cm3 bone vol) than the OVX+CASEIN group, whereas OVX+SOY- was similar to OVX+CASEIN (mean +/- SD; SHAM, 1.522 +/- 0.041; OVX+CASEIN, 1.449 +/- 0.044; OVX+SOY, 1.497 +/- 0.030; OVX+SOY-, 1.452 +/- 0.030). Ovariectomy resulted in greater bone turnover as indicated by higher serum alkaline phosphatase activity, serum insulin-like growth factor I and insulin-like growth factor binding protein 3 concentrations, and urinary hydroxyproline. These increases were not affected by soy with either normal or reduced isoflavone content. Similarly, histomorphometry revealed a greater bone formation rate with ovariectomy, and this was not altered by the soy diets. The findings of this study suggest that isoflavones in soy protein are responsible for its bone-sparing effects. Further studies to evaluate the mechanism of action of isoflavones on bone are warranted.

Transurethral fulguration of the prostate with the roller ball.

OBJECTIVES: To assess the histologic changes in the prostate and the clinical outcome in men with symptomatic benign prostatic hyperplasia (BPH) following transurethral fulguration of the prostate (TUFP) with the roller ball. METHODS: The study was conducted in two phases. In the first phase of the study, histologic changes in the prostate following fulguration with the roller ball were studied in 10 men with BPH who were already scheduled for transurethral resection of the prostate (TURP). In the second phase of the study, 20 male patients with symptomatic BPH underwent TUFP with the roller ball. All patients had preoperative assessment with history and physical examination, urinalysis, uroflow, transrectal ultrasound of the prostate, serum prostate-specific antigen, serum hemoglobin and electrolytes, and cystoscopy. The procedure was performed under general or spinal anesthesia using standard equipment and 3-mm roller ball. The patients were seen in follow-up at 1, 3, 6, 9, and 12 months. RESULTS: The results of the first phase of the study indicate that the coagulating current results in thermal destruction of prostatic tissue in the form of coagulative necrosis with minimal if any vaporization. The cutting current results in tissue vaporization with minimal coagulative necrosis. Twenty men have enrolled in the second phase of the study. Their mean age is 63.2 +/- 7.5 years and their mean follow-up is 4.6 months (range, 1 to 12). Their mean American Urological Association (AUA) symptom score declined from 22.9 +/- 4.2 preoperatively to 6.4 +/- 4.9, 5.3 +/- 3.2, 4.3 +/- 1.9, 6 +/- 2.9, and 9 at 1, 3, 6, 9, and 12 months, respectively. The mean maximum flow rate (Qmax) increased from 8.9 +/- 3.4 mL/s preoperatively to 24.3 +/- 1.9 mL/s at 3 months, 22 +/- 4.4 mL/s at 6 months, 17.6 +/- 5 mL/s at 9 months, and 21 mL/s at 12 months postoperatively. The mean prostate volume was 31.9 +/- 10 mL and the mean operative time was 44.9 +/- 10 minutes. No significant changes were seen in serum hemoglobin and serum sodium. The mean hospital stay was 0.7 day (0 to 2). Nineteen patients had their catheters removed within 24 hours and 1 patient had his catheter removed 48 hours postoperatively. No patient experienced impotence, incontinence, urethral stricture, post-transurethral resection syndrome, or required blood transfusion. In 1 patient (5%) bladder neck stenosis developed at 9 months and 1 patient required TURP for residual apical adenoma at 3 months. CONCLUSIONS: The nature and the degree of tissue changes in the prostate following fulguration with the roller ball are well controlled and predictable. When the cutting current is used, prostatic tissue can be removed safely and effectively. The clinical outcome after TUFP with the roller ball demonstrates significant improvement in subjective (AUA symptom score) and objective (Qmax) parameters, with reduced morbidity and short hospital stay. These early results compare favorably with those seen after TURP and laser ablation of the prostate. Larger series with longer follow-up are necessary to establish the long-term efficacy of TUFP in the treatment of BPH.

Spontaneous subcapsular hematoma in an ectopic kidney.

An unusual case of spontaneous rupture of the kidney is presented. We believe this to be the first reported case of spontaneous subcapsular renal hematoma in a kidney that is crossed-ectopic (pelvic) and hydronephrotic.

Urodynamic studies in interstitial cystitis.

Urodynamics, although not diagnostic of IC, are useful in the differential diagnosis of painful voiding disorders and provide a convenient way to quantitate response to therapy. They may prove useful as prognostic indicators of response to treatment and are of value in those patients in whom surgery is considered. Our studies suggest that IC is a progressive disease if not treated and that early diagnosis may improve response to treatment. Although similar information can be gathered by other means, we find urodynamics reliable, reproducible, and quantifiable and a valuable tool in the diagnosis, treatment, and follow-up of patients with IC.

Vaginal wall sling: four years later.

Since December 1985, we have treated 65 patients with urinary stress incontinence due to intrinsic sphincter dysfunction with the vaginal wall sling procedure. Of the 54 patients who were available for follow-up, intrinsic sphincter dysfunction was related to multiple prior bladder neck suspension procedures in 48 patients. In the remaining 6 patients, 2 had pelvic trauma, 2 had neurogenic urethral dysfunction, 1 had urethral diverticulectomy, and 1 had pelvic radiation. The success rate of the vaginal wall sling procedure for correcting stress incontinence was 94.4 percent at a mean follow-up of 23.9 months. Postoperative complications were minimal. Although 83 percent were temporarily in urinary retention, in the absence of neurogenic bladder and augmentation cystoplasty, only 5.5 percent needed intermittent self-catheterization on a long-term basis. De novo detrusor instability developed postoperatively in 14.8 percent of the cases. In no patient did a vaginal inclusion cyst develop. The vaginal wall sling is a simple procedure with excellent success rate and minimal morbidity. We recommend it for patients with stress urinary incontinence due to intrinsic sphincter dysfunction.

Transrectal ultrasound urodynamics.

Seventy-seven male patients (34 with cervical spine injury, 32 with thoracic spine injury, 9 with lumbosacral spine injury, and 2 with multiple sclerosis) who were referred to our spinal cord injury unit for urologic evaluation had videourodynamic studies using transrectal ultrasound and fluoroscopy. All were studied twice in the same session: first with transrectal ultrasound urodynamics (TRUSU) and the second time with conventional fluoroscopic videourodynamics (VUD). The findings were compared. Efficacy of TRUSU: (1) clearly documented flow of fluid through bladder neck into posterior urethra before clinical leakage occurred per urethra; (2) allowed accurate placement of EMG needle electrodes in external urinary sphincter under visual guidance; (3) provided high-quality imaging of surrounding structures and allowed assessment of bladder neck, prostate, seminal vesicles, and posterior urethra; (4) eliminated radiation exposure to the patient and the examiner, with no time limit imposed on imaging so that repeat studies could be done to evaluate patients on new drug treatments or postoperatively at no increased risks; and (5) its availability to most urologists at their office at considerably less cost to set up than fluoroscopy. In our study TRUSU identified a hypoechoic lesion in the prostate of 1 patient and a tumor in the bladder neck of another patient. Fluoroscopic VUD identified 6 patients with bladder diverticula and 2 with grades 1 and 2 vesicoureteral reflux which TRUSU did not identify. Our experience indicates TRUSU is a valid and preferable alternative to fluoroscopic VUD for patients with spinal cord injury.

Prolongation of response to DMSO by heparin maintenance.

Dimethylsulfoxide (DMSO) is an effective treatment of symptomatic patients with detrusor mastocytosis but it is associated with frequent relapses. A group of patients (N = 25) followed for twelve months showed a relapse rate of 59 percent. Our experience with a combination of DMSO and heparin has suggested that the relapse rate may be lower. Heparin is a glycosaminoglycan that may afford protection to the urothelium and may reduce the relapse rate. It is better tolerated than DMSO or a combination of DMSO and heparin and does not produce garlic halitus. It is not associated with coagulation anomalies when administered intravesically. To determine whether or not maintenance therapy with intravesical heparin may reduce relapses we have treated a similar cohort of 25 patients with monthly instillations of 10,000 IU of heparin over a twelve-month period. Both groups were comparable in age, duration of symptoms, severity of symptoms, and response to DMSO. At twelve months only 20 percent of the heparin-treated group had relapsed versus 52 percent of the control group. Furthermore 6 patients (24%) in the follow-up group failed to respond to retreatment with DMSO while all of the heparin maintenance group continued to respond to one or more treatments with DMSO. Thus, it seems that heparin maintenance produces a significant reduction in the relapse rate of patients who respond to DMSO and reduces the number of patients requiring alternative therapy.

Prune suppresses ovariectomy-induced hypercholesterolemia in rats.

Elevated cholesterol among women who have experienced natural or surgical menopause has been linked to ovarian hormone deficiency. The purpose of this study was to investigate the efficacy of prune, a good source of dietary fiber and phytochemicals, on lowering cholesterol in an ovariectomized (ovx) rat model. Forty-eight 90-day-old female Sprague-Dawley rats were randomly assigned to four groups: sham-operated (sham) + control diet, ovx + control diet, ovx + low-dose (LD; 5%) prune, and ovx + high-dose (HD; 25%) prune. After 45 days of treatment, rats were euthanized and tissues were collected for analyses. Ovariectomy elevated serum total cholesterol by 22% compared with sham, and HD prune diet prevented this increase without affecting high density lipoprotein cholesterol concentrations. Animals fed the HD prune diet had 13% lower liver total lipids compared with ovx animals. The findings of this study showed that prune exhibits hypocholesterolemic properties in ovarian hormone deficiency. Dose-response studies should be conducted to establish the effectiveness of prune in prevention of hypercholesterolemia in postmenopausal women who are not on estrogen replacement therapy and seek dietary alternatives. Mechanistic studies also are needed to establish its mode of action.

Epidemiology of current treatment for sexual dysfunction in spinal cord injured men in the USA model spinal cord injury centers.

This study is a prospective multicenter cooperative survey of the evaluation and treatment of erectile dysfunction in men with spinal cord injury (SCI). Uniform database questionnaires were completed prospectively by patients seeking therapy for erectile dysfunction. Eighty-five SCI men aged 17-68 years (mean age = 26 +/- 17) were enrolled. Mean duration of traumatic SCI was 3 +/- 3.2 years (Range = 0.3-18 years). The level of injury was cervical in 20 patients, thoracic in 31, lumbar in 29 and sacral in five. Patients were fully evaluated and then counseled as to their therapeutic options. Twenty-eight chose to use a vacuum erection device (VED), 26 preferred pharmacological penile injection and five used both intracorporeal therapy and VED. The remainder were managed with marriage and sexual counseling in 10 patients, three underwent penile prosthesis placement and two used topical pharmacotherapy. Four patients used other forms of treatment and in nine no therapy was recommended. Of the patients that used pharmacologic injection only, 74 percent used papaverine as a single agent, 20 percent used papaverine with phentolamine, five percent used prostaglandin E (PGE1) alone and one percent used a mixture. Patients using injection therapy report sexual intercourse a mean of 3 +/- 3.4 times per month as compared with 5 +/- 3.2 times per month in those using VED. Five intracorporeal injection patients developed priapism while two patients using the VED developed subcutaneous bleeding and one developed penile ischemia. We conclude that although a spectrum of erectile dysfunction treatment is present among SCI centers, VED and pharmacological penile injection are by far the two most popular methods of treatment and papaverine is the most common drug. The incidence of complications is small in the model centers.

Diffuse bone metastases in a case of astrocytoma.

Presented is an unusual case of astrocytoma with diffuse multiple bony metastases in bone imaging. The classification, spread, and prognosis of astrocytoma are briefly reviewed.

Endourethral prosthesis for treatment of detrusor sphincter dyssynergia: impact on quality of life for persons with spinal cord injury.

Neurogenic bladders with detrusor sphincter dyssynergia (DSD) are common in persons with spinal cord injury and with multiple sclerosis. Treatment options such as sphincterotomy, urethral foley and suprapubic catheters are not without complications or failure; however, they are recommended for patients who are unable to do intermittent catheterization. An endourethral prosthesis for DSD has been developed for experimental use in the United States. We report one year results of the clinical trials with endourethral prostheses implanted in ten patients. Initial subjective and objective results show an 80% success rate. At present, preliminary data show that an endourethral prosthesis, which is simple to insert and has few complications, appears to offer an alternative to conventional treatment. A key to success is to prepare for changes in life style that impact the patient's quality of life. Both patient and staff education are necessary pre-operatively to achieve successful outcomes post-operatively.

Soy isoflavones may protect against orchidectomy-induced bone loss in aged male rats.

Evidence from several studies suggests that soy protein and/or its isoflavones may have beneficial effects on bone in postmenopausal women and animal models who have osteoporosis. The present study examined the dose-dependent effects of soy isoflavones in the context of soy protein or casein on the male skeleton. Thirteen-month-old male Fisher 344 rats were orchidectomized (ORX; 5 groups) or sham-operated (Sham; 1 group) and immediately placed on dietary treatments for 180 days. Diets were semi-purified and the protein source was either casein (Sham and ORX; controls), casein with two added doses of isoflavones (Iso1; 600 mg/kg diet and Iso2; 1200 mg/kg diet), soy protein with normal isoflavones content (Soy; 600 mg/kg diet), or soy protein with added isoflavones (Soy+; 1200 mg/kg diet). A 7% loss of whole body bone mineral density (BMD) was observed due to orchidectomy; however, the ORX induced BMD loss was significantly reduced to 4.3 and 4.7 % with the Soy and Soy+, respectively. Both doses of isoflavones in conjunction with casein also reduced the loss of whole body BMD, albeit not significantly different from ORX control animals. Trabecular bone histomorphometric analysis of the proximal tibia further supported the bone-sparing role of soy isoflavones as indicated by higher percent bone volume and trabecular number, and lower trabecular separation. We conclude that isoflavones exert modest beneficial effects on the male skeleton whether provided with casein or a soy protein.

Anterior vaginal suspension for vaginal vault prolapse.

Vaginal prolapse may occur following hysterectomy or may evolve with the uterus in place. Current treatment options for vaginal prolapse have a limited success rate or are associated with significant morbidity. In this retrospective review, we present our experience with a new procedure for repair of vaginal prolapse. This technique relies on anterior suspension of the vaginal vault to the anterior rectus sheath in a fashion similar to bladder neck suspension. Forty patients have undergone this procedure. All patients had vaginal prolapse, cystocele, and urinary incontinence. In addition, 34 patients had enterocele, six had uterine prolapse, 33 had rectocele, and two had urethral diverticula. All patients had anterior vaginal suspension (AVS), with cystocele repair and bladder neck suspension. Six patients had vaginal hysterectomy, 34 had enterocele repair, 33 had rectocele repair, and two had urethral diverticulectomy. The mean hospital stay was 2.5 days (range, 1-7 days), and their mean follow-up was 30 months (range, 12-54 months). Thirty-six (90%) patients have excellent support of the vagina with no evidence of recurrent cystocele, enterocele, or rectocele. Four (10%) patients have recurrent enterocele. Thirty-three (82.5%) patients are dry or have rare episodes of urinary incontinence (less than one episode/month), whereas four (10%) patients have recurrent stress incontinence and three (7.5%) have urge incontinence. Constipation and fecal incontinence were resolved in all patients. All patients who were sexually active preoperatively remained so postoperatively, and none reported dyspareunea. Vaginogram in 10 patients demonstrated that posterior angulation of the vaginal axis was retained in all patients. AVS is associated with an excellent success rate in terms of resolution of symptoms and correction of prolapse. Morbidity is minimal, and hospital stay is short. The technique is simple and relies on anatomy that is familiar to all urologists. In addition, the vaginal approach allows for simultaneous correction of all components of vaginal prolapse and any associated vaginal pathology.

Magnetic resonance imaging of the UroLume urethral stent.

We discuss the use of magnetic resonance imaging of the pelvis in a patient with the UroLume urethral stent. This case demonstrates that magnetic resonance imaging is a safe and effective modality for imaging tissue surrounding the stent.