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Background: Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory disease of brain vessels. The small vessel subtype is diagnosed on brain biopsy and often presents with cognitive and behavioural changes, headaches and seizures. However, there are few reported cases of super-refractory status epilepticus. Methods: We present a case of small vessel cPACNS complicated by super-refractory status epilepticus and review the literature. Results: Our patient is a previously healthy 11-year-old boy who presented with new-onset seizures and encephalopathy in the context of fever. He developed super-refractory status epilepticus, requiring burst suppression for four weeks with various IV infusions. During this time, he was on the ketogenic diet and tried eight anti-seizure medications. Extensive investigations included brain biopsy confirming small vessel cPACNS. He was treated with IV methylprednisolone, oral steroids, IVIG, and cyclophosphamide. After prolonged rehabilitation, he recovered almost completely and has a normal neurological examination with no epileptiform activity on EEG. Conclusions: Small vessel cPACNS should be considered in the differential diagnosis of super-refractory status epilepticus. Despite being in SE for four weeks, symptomatic management of seizures and immunosuppression to treat the underlying pathology resulted in favourable neurological outcomes. This is one of the longest cases of SE in small vessel cPACNS in the literature.
A new lonchidiid genus, Pristrisodus, from the Upper Triassic Tiki Formation of India is described based on multiple, well-preserved, isolated teeth. Comparative analysis resulted in synonymizing Parvodus tikiensis and Lissodus duffini, which are known from the same horizon and resulted in a new taxon, Pristrisodus tikiensis n. comb. These teeth are elongated with mesiodistal length greater than or equal to twice the labiolingual width and have a high principal cusp, lateral cusplets, a distinct ridge near the crown-root junction labially and higher up on the crown lingually, weak ornamentation, and linear depression along the crown-root junction. Five morphotypes based on overall shape, robustness and crown height are determined. The teeth show a gradual monognathic heterodonty. The anterolateral teeth (morphotypes I−II) have high, pyramidal principal cusp with two or three small but pointed cusplets, and triangular labial and lingual protuberance. The posterolateral teeth (morphotypes III−IV) have four incipient cusplets, relatively low principal cusp, bilobed/rounded, hanging labial and incipient lingual protuberances. Morphotype V comprises anterior teeth that are broad, triangular and robust, and have rounded/blunt principal cusp, one cusplet, and low, hanging labial peg. Multivariate analyses corroborate the qualitative assessment of the Indian hybodonts. Dental histology of Pristrisodus n. gen., shows that it is distinctly different from other lonchidiid genera. The assemblage of freshwater sharks, along with other vertebrate microfossils of the Tiki Formation, shows similarity with that of the lower Tecovas Formation of the Chinle Group, USA. The euryhaline nature resulted in the adaptation of the hybodonts to freshwater systems in India during the Carnian.
Background: The KCNT1 gene encodes subunits of the Na+-activated K+ channel, widely expressed in the CNS. Mutations of this gene have been implicated in Malignant Migrating Partial Seizures of Infancy (MMPSI). This early-onset epileptic encephalopathy represents a challenge due to pharmacoresistance. The channel-specific mutation represents the potential for targeted pharmacotherapy. Quinidine is a partial antagonist of the KCNT1 encoded channel; patients with MMPSI have been reported to have responded to doses ranging 34.4/kg/d - 60mg/kg/d. We present a case of MMPSI with a KCNTI mutation (c.G1283A:p.R428Q) trialled on quinidine. Methods: Following ineffective trials of 6 anti-seizure medications, this patient was trialled on oral quinidine. This patient was titrated up to a dose of 52mg/kg/d. Twenty-four hour EEG monitoring prior to quinidine therapy, and at target dose were compared. Results: Prior to initiation of quinidine, this patient experienced 22 electrographic seizures over 24 hours. At target dose, this patient experienced greater than 70 seizures over 24 hours. Conclusions: Quinidine has previously been reported to be effective in patients with MMPSI with the same and different mutations. We report the second case of a patient with MMPSI and KCNT1 mutation R428Q with poor clinical response to quinidine.
Background: Hemimegalencephaly (HME) is a hamartomatous malformation of one cerebral hemisphere, resulting in refractory epilepsy, intellectual disability, and autistic features. Hemispherectomy is the definitive treatment, but there is risk of high morbidity and mortality, especially when done in early infancy. Various preclinical studies have shown that dysregulation of the mTOR pathway has an integral role in the development of various epilepsy syndromes, including tuberous sclerosis complex (TSC), focal cortical dysplasia and HME. Recently, mTOR inhibitors were proven to be effective in treating seizures in TSC. Methods: We present a case of a 6 day old female with refractory epilepsy despite the trial of 9 anti-seizure medications and the ketogenic diet. As the patient was awaiting epilepsy surgery, an mTOR inhibitor, rapamycin was initiated. Results: After 1 week of the initiation, she had over a 50% reduction in seizures. At two weeks, the parents felt that for the first time, she was making developmental gains. She also appeared brighter and more interactive. Due to her response to treatment, her hemispherectomy was deferred to when she is older, so there will be a decreased risk of complications from the surgery. Conclusions: This case exemplifies how mTOR inhibitors should be considered as a treatment option for patients with HME and refractory epilepsy.
Background: Perampanel (PER) is a new anti-seizure medication that inhibits the α-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid (AMPA) class of glutamate receptors. It is available in Canada for children since 2014. It is important for physicians to be aware of the efficacy and tolerability of drugs in the post-marketing phase. Methods: We did a retrospective review of our experience with PER at BC Children’s Hospital. Patients on PER were identified. Clinical data, including demographics, efficacy, tolerability, adverse effects (AE) and retention rates were obtained by review of clinical records. Results: Of 24 patients pediatric patients prescribed PER, 21 (87%) had focal and three had symptomatic generalized epilepsy. Ten (42%) had greater than 50% reduction in seizures. In fifteen patients, (63%) PER was discontinued due to AE or poor response. Twelve (50%) had behavioral AE and eight (33%) had non-behavioral AE. PER was effective, at lower doses than required for adults. One third experienced serious AE. One patient experienced oculogyric crisis, not previously reported with PER. AE were not associated with high doses and were reversible. Possible risk factors for behavioral AE include behavioral problems with other medications and pre-existing behavioral co-morbidities. Conclusions: It is important for clinicians to be aware of and counsel patients about serious AE, particularly behavioral, when prescribing PER.
Background: Epileptic encephalopathy (EE) is a severe condition in which epileptic activity itself may contribute to severe cognitive and behavioural impairments above and beyond what might be expected from the underlying pathology alone. Next generation sequencing technologies such as whole exome sequencing (WES) can detect underlying genetic causes of in EE. Methods: This report describes genotype-phenotype correlation of 29 subjects with unexplained epileptic encephalopathy, in whom WES, targeting a list of 557 epilepsy-associated genes was performed. Epilepsy phenotyping was done according to current ILAE recommendations. Results: Median age at seizure onset was 14 months (range 1-48). Electroclinical syndromes were applicable for 16/29, 8/16 had a definite/likely diagnosis. 6/8 subjects with West syndrome had variants in ALG13, STXBP1, PAFAH1B1, SLC35A2, CDKL5 and ADSL. 2 patients with Dravet syndrome had variants in SCN1A and PCDH19 respectively. 4/29 had unspecified EE and definite/likely diagnosis due to STXBP1, POLG, and KCNQ2 (2) variants. 4/29 had a possible diagnosis involving GABRB3, ARHGEF9, PCDH19 and SCN3A variants. Conclusions: The high diagnostic yield (definite/likely diagnosis in 11/29 = 38%), involving a broad variety of epilepsy-associated genes in different electroclinical syndromes justifies the diagnostic approach of early onset EE by next generation sequencing.
There are few data on excess direct and indirect costs of diabetes in India and limited data on rural costs of diabetes. We aimed to further explore these aspects of diabetes burdens using a clinic-based, comparative cost-of-illness study.
Persons with diabetes (n = 606) were recruited from government, private, and rural clinics and compared to persons without diabetes matched for age, sex, and socioeconomic status (n = 356). We used interviewer-administered questionnaires to estimate direct costs (outpatient, inpatient, medication, laboratory, and procedures) and indirect costs [absence from (absenteeism) or low productivity at (presenteeism) work]. Excess costs were calculated as the difference between costs reported by persons with and without diabetes and compared across settings. Regression analyses were used to separately identify factors associated with total direct and indirect costs.
Annual excess direct costs were highest amongst private clinic attendees (INR 19 552, US$425) and lowest amongst government clinic attendees (INR 1204, US$26.17). Private clinic attendees had the lowest excess absenteeism (2.36 work days/year) and highest presenteeism (0.06 work days/year) due to diabetes. Government clinic attendees reported the highest absenteeism (7.48 work days/year) and lowest presenteeism (−0.31 work days/year). Ten additional years of diabetes duration was associated with 11% higher direct costs (p < 0.001). Older age (p = 0.02) and longer duration of diabetes (p < 0.001) were associated with higher total lost work days.
Excess health expenditures and lost productivity amongst individuals with diabetes are substantial and different across care settings. Innovative solutions are needed to cope with diabetes and its associated cost burdens in India.
Listeria monocytogenes is a foodborne pathogen that can cause bacteraemia, meningitis, and complications during pregnancy. In July 2012, molecular subtyping identified indistinguishable L. monocytogenes isolates from six patients and two samples of different cut and repackaged cheeses. A multistate outbreak investigation was initiated. Initial analyses identified an association between eating soft cheese and outbreak-related illness (odds ratio 17·3, 95% confidence interval 2·0–825·7) but no common brand. Cheese inventory data from locations where patients bought cheese and an additional location where repackaged cheese yielded the outbreak strain were compared to identify cheeses for microbiological sampling. Intact packages of imported ricotta salata yielded the outbreak strain. Fourteen jurisdictions reported 22 cases from March–October 2012, including four deaths and a fetal loss. Six patients ultimately reported eating ricotta salata; another reported eating cheese likely cut with equipment also used for contaminated ricotta salata, and nine more reported eating other cheeses that might also have been cross-contaminated. An FDA import alert and US and international recalls followed. Epidemiology-directed microbiological testing of suspect cheeses helped identify the outbreak source. Cross-contamination of cheese highlights the importance of using validated disinfectant protocols and routine cleaning and sanitizing after cutting each block or wheel.
Background: For adolescents with epilepsy, there is often a poor system in place to meet their individualized transition needs. Our objectives were 1) to develop epilepsy-specific transition care management plans (TCMPs) to ensure access, and attachment to adult healthcare providers, and 2) to identify strategies for providing support during the transition period, including through the development of physician and patient (or caregiver) navigated web-based tools, resources and recommendations for health system improvements. Methods: Physicians and nurses with expertise in areas including adult and pediatric epilepsy, family medicine, psychiatry, and varied allied health professionals were engaged to generate epilepsy-related TCMPs. Results: Through an iterative process spanning the course of over a year, TCMPs were developed to cover areas including: treatment responsive and resistant epilepsy, ketogenic diet, epilepsy surgery, women’s issues, mental health, and psychosocial aspects of epilepsy. The TCMPs referenced established guidelines and best practices in the literature wherever possible. Caregiver roles and responsibilities were outlined, remaining cognoscent of available provincial resources. Conclusions: Epilepsy specific TCMPs can be developed through a collaborative approach between pediatric and adult healthcare providers, easing the patient experience, creating educated accountability, and providing a forum to identify and address gaps of care in adolescents with epilepsy.
This study aimed to determine the prevalence of hearing impairment in Bangladeshi people of all ages.
A nationally representative cross-sectional survey was carried out in 2013. A total of 4260 subjects (1774 males and 2486 females), with a mean age of 32 years, participated. Hearing impairment was determined by pure tone audiometry and otoacoustic emissions testing.
Disabling hearing loss (greater than 40 dB loss in adults, and greater than 30 dB loss in children younger than 15 years, in their better hearing ears) was present in 9.6 per cent (95 per cent confidence interval, 8.5–10.8 per cent) of the respondents. Hearing loss was more prevalent in socio-economically deprived people and in those older than 60 years. Multiple logistic regression analysis identified age, socio-economic deprivation, family history, impacted ear wax, chronic suppurative otitis media, otitis media with effusion, and otitis externa as the significant predictors of disabling hearing loss.
Deafness prevention should focus mainly on chronic suppurative otitis media, otitis media with effusion, and impacted ear wax prevention, integrated within the primary healthcare system and addressing the equity issue.
States have established public reporting of hospital-associated (HA) infections—including those of methicillin-resistant Staphylococcus aureus (MRSA)—but do not account for hospital case mix or postdischarge events
Identify facility-level characteristics associated with HA-MRSA infection admissions and create adjusted hospital rankings.
A retrospective cohort study of 2009–2010 California acute care hospitals. We defined HA-MRSA admissions as involving MRSA pneumonia or septicemia events arising during hospitalization or within 30 days after discharge. We used mandatory hospitalization and US Census data sets to generate hospital population characteristics by summarizing across admissions. Facility-level factors associated with hospitals’ proportions of HA-MRSA infection admissions were identified using generalized linear models. Using state methodology, hospitals were categorized into 3 tiers of HA-MRSA infection prevention performance, using raw and adjusted values.
Among 323 hospitals, a median of 16 HA-MRSA infections (range, 0–102) per 10,000 admissions was found. Hospitals serving a greater proportion of patients who had serious comorbidities, were from low-education zip codes, and were discharged to locations other than home were associated with higher HA-MRSA infection risk. Total concordance between all raw and adjusted hospital rankings was 0.45 (95% confidence interval, 0.40–0.51). Among 53 community hospitals in the poor-performance category, more than 20% moved into the average-performance category after adjustment. Similarly, among 71 hospitals in the superior-performance category, half moved into the average-performance category after adjustment.
When adjusting for nonmodifiable facility characteristics and case mix, hospital rankings based on HA-MRSA infections substantially changed. Quality indicators for hospitals require adequate adjustment for patient population characteristics for valid interhospital performance comparisons.
Infect Control Hosp Epidemiol 2014;35(10):1263–1270
To evaluate whether an ecologic inverse association exists between methicillin-susceptible Staphylococcus aureus (MSSA) prevalence and methicillin-resistant S. aureus (MRSA) prevalence in nursing homes.
We conducted a secondary analysis of a prospective cross-sectional study of S. aureus prevalence in 26 nursing homes across Orange County, California, from 2008–2011. Admission prevalence was assessed using bilateral nares swabs collected from all new residents within 3 days of admission until 100 swabs were obtained. Point prevalence was assessed from a representative sample of 100 residents. Swab samples were plated on 5% sheep blood agar and Spectra MRSA chromogenic agar. If MRSA was detected, no further tests were performed. If MRSA was not detected, blood agar was evaluated for MSSA growth. We evaluated the association between MRSA and MSSA admission and point prevalence using correlation and linear regression testing.
We collected 3,806 total swabs. MRSA and MSSA admission prevalence were not correlated (r = −0.40, P = .09). However, MRSA and MSSA point prevalence were negatively correlated regardless of whether MSSA prevalence was measured among all residents sampled (r = −0.67, P = .0002) or among those who did not harbor MRSA (r = −0.41, P = .04). This effect persisted in regression models adjusted for the percentage of residents with diabetes (β = −0.73, P = .04), skin lesions (β = −1.17, P = .002), or invasive devices (β = −1.4, P = .0006).
The inverse association between MRSA and MSSA point prevalence and minimal association on admission prevalence suggest MSSA carriage may protect against MRSA acquisition in nursing homes. The minimal association on admission prevalence further suggests competition may occur during nursing home stays.
Infect Control Hosp Epidemiol 2014;35(10):1257–1262
The loss of tropical forests and associated biodiversity is a global concern. Conservation efforts in tropical countries such as India have mostly focused on state-administered protected areas despite the existence of vast tracts of forest outside these areas. We studied hornbills (Bucerotidae), an ecologically important vertebrate group and a flagship for tropical forest conservation, to assess the importance of forests outside protected areas in Arunachal Pradesh, north-east India. We conducted a state-wide survey to record encounters with hornbills in seven protected areas, six state-managed reserved forests and six community-managed unclassed forests. We estimated the density of hornbills in one protected area, four reserved forests and two unclassed forests in eastern Arunachal Pradesh. The state-wide survey showed that the mean rate of encounter of rufous-necked hornbills Aceros nipalensis was four times higher in protected areas than in reserved forests and 22 times higher in protected areas than in unclassed forests. The mean rate of encounter of wreathed hornbills Rhyticeros undulatus was twice as high in protected areas as in reserved forests and eight times higher in protected areas than in unclassed forests. The densities of rufous-necked hornbill were higher inside protected areas, whereas the densities of great hornbill Buceros bicornis and wreathed hornbill were similar inside and outside protected areas. Key informant surveys revealed possible extirpation of some hornbill species at sites in two protected areas and three unclassed forests. These results highlight a paradoxical situation where individual populations of hornbills are being lost even in some legally protected habitat, whereas they continue to persist over most of the landscape. Better protection within protected areas and creative community-based conservation efforts elsewhere are necessary to maintain hornbill populations in this biodiversity-rich region.
We have prepared stable ultrafine narrow dispersed copper nanoparticles (Cu-NPs) using a facile chemical reduction technique below room temperature (300 K), without any template. X-ray diffraction and high-resolution transmission electron microscopy studies reveal the growth of highly crystalline Cu-NPs with an average diameter of 2.2 nm. Interestingly, these Cu-NPs demonstrate both interband electronic transitions along with usual surface plasmon resonance, a unique phenomenon previously unobserved in any noble metal nanoparticles. These Cu-NPs do not get oxidized easily and could be suitable candidates for different optical devices, heat transfer liquids, and biological applications.
Central line-associated bloodstream infection (CLABSI) is a national target for mandatory reporting and a Centers for Medicare and Medicaid Services target for value-based purchasing. Differences in chart review versus claims-based metrics used by national agencies and groups raise concerns about the validity of these measures.
Evaluate consistency and reasons for discordance among chart review and claims-based CLABSI events.
We conducted 2 multicenter retrospective cohort studies within 6 academic institutions. A total of 150 consecutive patients were identified with CLABSI on the basis of National Healthcare Safety Network (NHSN) criteria (NHSN cohort), and an additional 150 consecutive patients were identified with CLABSI on the basis of claims codes (claims cohort). Ail events had full-text medical record reviews and were identified as concordant or discordant with the other metric.
In the NHSN cohort, there were 152 CLABSIs among 150 patients, and 73.0% of these cases were discordant with claims data. Common reasons for the lack of associated claims codes included coding omission and lack of physician documentation of bacteremia cause. In the claims cohort, there were 150 CLABSIs among 150 patients, and 65.3% of these cases were discordant with NHSN criteria. Common reasons for the lack of NHSN reporting were identification of non-CLABSI with bacteremia meeting Centers for Disease Control and Prevention (CDC) criteria for an alternative infection source.
Substantial discordance between NHSN and claims-based CLABSI indicators persists. Compared with standardized CDC chart review criteria, claims data often had both coding omissions and misclassification of non-CLABSI infections as CLABSI. Additionally, claims did not identify any additional CLABSIs for CDC reporting. NHSN criteria are a more consistent interhospital standard for CLABSI reporting.
Asymmetric (10L) XRD peaks have been employed as a measure of epitaxial quality for aluminum nitride (AlN) nucleation layers (NL) deposited on sapphire substrate. Epitaxial AlN films have been deposited on 2-6” sapphire substrate by reactive sputtering. FWHM of AlN (103) and (105) were found to be an excellent indicator of quality of AlN films for GaN growth. AlN films produced nucleation layers with highly reproducible microstructure and GaN film growth. NLs had in-plane and out-of-plane texture as evident by the pole-figure results and selected area diffraction pattern. Based on electron microscopy results, AlN film thickness for complete atomic ordering was estimated to be 6-7 nm and most of the edge dislocations were seen in the first 20 nm of the film. Excellent thickness and texture uniformity were seen on planar and patterned sapphire substrates. A compressive stress of 2.9±0.2 GPa was seen in our BKM films. The maximum screw and edge dislocation densities of films were found to be ∼3 x 108 cm−2 and ∼9 x 109 cm−2 respectively. The root mean square roughnesses of A-polar films were found to be < 0.3 nm.
A nonlinear optical crystal of L-valine was grown from an aqueous solution containing a small amount of phosphoric acid by the slow evaporation method. The grown crystal was characterized by a single crystal X-ray diffraction to determine the unit cell parameters. The powder X-ray diffraction analysis also confirmed the lattice parameters to be a = 9.6687(7) Å, b = 5.2709(4) Å, c = 12.0371(10) Å and β = 90.805(4)°. The results of the Inductively Coupled Plasma Optical Emission Spectrometry (ICPOES) indicate the presence of a small amount of phosphorus in the grown crystal. The Vickers micro hardness test was performed to study the mechanical strength of the crystals. Chemical etching studies were carried out to analyze the dislocation structure. The laser damaged threshold of the grown crystal was measured to be 11.11 GW/cm2 for 10 ns pulse at 1064 nm, which is higher than that of the standard nonlinear optical crystals like KDP. Second harmonic generation of the grown crystals was also 1.44 times that of KDP.