scholarly journals Treated hydrocephalus in individuals with myelomeningocele in the National Spina Bifida Patient Registry

2018 ◽  
Vol 22 (6) ◽  
pp. 646-651 ◽  
Author(s):  
Irene Kim ◽  
Betsy Hopson ◽  
Inmaculada Aban ◽  
Elias B. Rizk ◽  
Mark S. Dias ◽  
...  

OBJECTIVEAlthough the majority of patients with myelomeningocele have hydrocephalus, reported rates of hydrocephalus treatment vary widely. The purpose of this study was to determine the rate of surgical treatment for hydrocephalus in patients with myelomeningocele in the National Spina Bifida Patient Registry (NSBPR). In addition, the authors explored the variation in shunting rates across NSBPR institutions, examined the relationship between hydrocephalus, and the functional lesion level of the myelomeningocele, and evaluated for temporal trends in rates of treated hydrocephalus.METHODSThe authors queried the NSBPR to identify all patients with myelomeningoceles. Individuals were identified as having been treated for hydrocephalus if they had undergone at least 1 hydrocephalus-related operation. For each participating NSBPR institution, the authors calculated the proportion of patients with treated hydrocephalus who were enrolled at that site. Logistic regression was performed to analyze the relationship between hydrocephalus and the functional lesion level of the myelomeningocele and to compare the rate of treated hydrocephalus in children born before 2005 with those born in 2005 or later.RESULTSA total of 4448 patients with myelomeningocele were identified from 26 institutions, of whom 3558 patients (79.99%) had undergone at least 1 hydrocephalus-related operation. The rate of treated hydrocephalus ranged from 72% to 96% among institutions enrolling more than 10 patients. This difference in treatment rates between centers was statistically significant (p < 0.001). Insufficient data were available in the NSBPR to analyze reasons for the different rates of hydrocephalus treatment between sites. Multivariate logistic regression demonstrated that more rostral functional lesion levels were associated with higher rates of treated hydrocephalus (p < 0.001) but demonstrated no significant difference in hydrocephalus treatment rates between children born before versus after 2005.CONCLUSIONSThe rate of hydrocephalus treatment in patients with myelomeningocele in the NSBPR is 79.99%, which is consistent with the rates in previously published literature. The authors’ data demonstrate a clear association between functional lesion level of the myelomeningocele and the need for hydrocephalus treatment.

2018 ◽  
Vol 22 (6) ◽  
pp. 652-658 ◽  
Author(s):  
Irene Kim ◽  
Betsy Hopson ◽  
Inmaculada Aban ◽  
Elias B. Rizk ◽  
Mark S. Dias ◽  
...  

OBJECTIVEThe purpose of this study was to determine the rate of decompression for Chiari malformation type II in individuals with myelomeningocele in the National Spina Bifida Patient Registry (NSBPR). In addition, the authors explored the variation in rates of Chiari II decompression across NSBPR institutions, examined the relationship between Chiari II decompression and functional lesion level of the myelomeningocele, age, and need for tracheostomy, and they evaluated for temporal trends in rates of Chiari II decompression.METHODSThe authors queried the NSBPR to identify all individuals with myelomeningocele between 2009 and 2015. Among these patients, they identified individuals who had undergone at least 1 Chiari II decompression as well as those who had undergone tracheostomy. For each participating NSBPR institution, the authors calculated the proportion of patients enrolled at that site who underwent Chiari II decompression. Logistic regression was performed to analyze the relationship between Chiari II decompression, functional lesion level, age at decompression, and history of tracheostomy.RESULTSOf 4448 individuals with myelomeningocele identified from 26 institutions, 407 (9.15%) had undergone at least 1 Chiari II decompression. Fifty-one patients had undergone tracheostomy. Logistic regression demonstrated a statistically significant relationship between Chiari II decompression and functional lesion level of the myelomeningocele, with a more rostral lesion level associated with a higher likelihood of posterior fossa decompression. Similarly, children born before 2005 and those with history of tracheostomy had a significantly higher likelihood of Chiari II decompression. There was no association between functional lesion level and need for tracheostomy. However, among those children who underwent Chiari II decompression, the likelihood of also undergoing tracheostomy increased significantly with younger age at decompression.CONCLUSIONSThe rate of Chiari II decompression in patients with myelomeningocele in the NSBPR is consistent with that in previously published literature. There is a significant relationship between Chiari II decompression and functional lesion level of the myelomeningocele, which has not previously been reported. Younger children who undergo Chiari II decompression are more likely to have undergone tracheostomy. There appears to be a shift away from Chiari II decompression, as children born before 2005 were more likely to undergo Chiari II decompression than those born in 2005 or later.


2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S287-S287
Author(s):  
Jessica Penney ◽  
Amanda Jung ◽  
Benjamin Koethe ◽  
Shira Doron

Abstract Background Real-time reverse transcriptase PCR (rRT-PCR) has become the primary method for detection of SARS-CoV-2. Specific measurements of cycle threshold (Ct) values can give an estimate of viral load. Previous studies have shown temporal trends in Ct values, which could be used to predict the phase of the pandemic. This study’s goal was to examine the relationship between Ct and disease severity, as well as Ct trends. Methods Testing was performed using the Abbott M2000 SARS-CoV-2 assay. Data was collected for 262 SARS-CoV-2 positive patients from March-May 2020. Kruskal-Wallis testing was performed to determine differences in median Ct based on age, gender, race and ethnicity. To determine relationship between symptom onset and clinical severity with Ct, linear and logistic regression were performed. Results The majority of the patients had mild to moderate disease. Average time since symptom onset was 5.9 days, and 92% were symptomatic. Figure 1 demonstrates the distribution of Ct by disease severity at time of testing. There was no significant difference in cycle threshold by sex, age, race or ethnicity. Figure 2 shows weekly mean cycle threshold by total new cases in Massachusetts to reflect temporal trend of Ct and cases. In the multivariable linear regression model, Ct increased with days since symptom onset (P&lt; 0.001). Cycle threshold was inversely associated with disease severity in multivariable logistic regression though (OR 1.06, 95%CI 1.01-1.11, p=0.03), even when controlling for time since symptom onset. Figure 1. Distribution of Ct by disease severity at time of SARS-CoV-2 testing Boxplot demonstrating distribution of Ct by disease severity at time of testing. There was no significant difference between groups. Figure 2. Weekly Mean Cycle Threshold by Total New MA Cases Line represents mean Ct over time period included in this study overlaid on total new cases in Massachusetts. Lower Ct were seen in the course as cases were increasing which peaked as cases stabilized. Conclusion Cycle threshold increased with time since symptom onset, consistent with prior data showing increasing Ct from time since infection due to decreasing viral replication. This study showed an inverse relationship between cycle threshold and disease severity, which differs from previous studies which demonstrated higher odds of progression to severe disease and mortality with lower Ct. This finding may reflect the disease severity associated with the secondary inflammatory phase of SARS-CoV-2 seen later in the disease course, although there was only moderate correlation between Ct and time since symptom onset. Further research is needed to better understand the role of Ct in predicting clinical severity of SARS-CoV-2 infections. Disclosures All Authors: No reported disclosures


2021 ◽  
Vol 80 (2) ◽  
pp. 673-681
Author(s):  
Jin Wang ◽  
Xiaojuan Guo ◽  
Wenhui Lu ◽  
Jie Liu ◽  
Hong Zhang ◽  
...  

Background: Vascular factors and mitochondria dysfunction contribute to the pathogenesis of Alzheimer’s disease (AD). DL-3-n-butylphthalide (NBP) has an effect in protecting mitochondria and improving microcirculation. Objective: The aim was to investigate the effect of donepezil combined NBP therapy in patients with mild-moderate AD. Methods: It was a prospective cohort study. 92 mild-moderate AD patients were classified into the donepezil alone group (n = 43) or the donepezil combined NBP group (n = 49) for 48 weeks. All patients were evaluated with Alzheimer’s Disease Assessment Scale-Cognitive subscale (ADAS-cog), Clinician’s Interview-Based Impression of Change plus caregiver input (CIBIC-plus), Alzheimer’s Disease Cooperative Study-Activities of Daily Living (ADCS-ADL), and Neuropsychiatric Inventory (NPI) every 12 weeks. All patients were monitored for adverse events (AEs). The efficacy was analyzed using multivariate logistic regression analysis. Results: The multivariate logistic regression analysis showed that the changes of ADAS-cog score (OR = 2.778, 95% CI: [1.087, 7. 100], p = 0.033) and ADCS-ADL score (OR = 2.733, 95% CI: [1.002, 7.459], p = 0.049) had significant difference between donepezil alone group and donepezil combined NBP group, while the changes of NPI (OR = 1.145, 95% CI: [0.463, 2.829], p = 0.769), MMSE (OR = 1.563, 95% CI: [0.615, 3.971], p = 0.348) and CIBIC-plus (OR = 2.593, 95% CI: [0.696, 9.685], p = 0.156) had no significant difference. The occurrence of AEs was similar in the two groups. Conclusion: Over the 48-week treatment period, donepezil combined NBP group had slower cognitive decline and better activities of daily living in patients with mild to moderate AD. These indicated that the multi-target therapeutic effect of NBP may be a new choice for AD treatment.


2021 ◽  
Vol 40 (1) ◽  
Author(s):  
Li Luo ◽  
Huan Zeng ◽  
Mao Zeng ◽  
Xueqing Liu ◽  
Xianglong Xu ◽  
...  

Abstract Background After the implementation of the universal two-child policy in China, the increase in parity has led to an increase in adverse pregnancy outcomes. The impact of one and two fetuses on the incidence of fetal macrosomia has not been fully confirmed in China. This study aimed to explore the differences in the incidence of fetal macrosomia in first and second pregnancies in Western China after the implementation of the universal two-child policy. Methods A total of 1598 pregnant women from three hospitals were investigated by means of a cross-sectional study from August 2017 to January 2018. Participants were recruited by convenience and divided into first and second pregnancy groups. These groups included 1094 primiparas and 504 women giving birth to their second child. Univariate and multivariate logistic regression analyses were performed to discuss the differences in the incidence of fetal macrosomia in first and second pregnancies. Results No significant difference was found in the incidence of macrosomia in the first pregnancy group (7.2%) and the second pregnancy group (7.1%). In the second-time pregnant mothers, no significant association was found between the macrosomia of the second child (5.5%) and that of the first child (4.7%). The multivariate logistic regression model showed that mothers older than 30 years are not likely to give birth to children with macrosomia (odds ratio (OR) 0.6, 95% confidence interval (CI) 0.4,0.9). Conclusions The incidence of macrosomia in Western China is might not be affected by the birth of the second child and is not increased by low parity.


Hypertension ◽  
2013 ◽  
Vol 62 (suppl_1) ◽  
Author(s):  
Cheng Wang ◽  
Jun Zhang ◽  
Cuicui Li ◽  
Wenyu Gong ◽  
Tanqi Lou

Background: Neural precursor cell expressed developmentally down-regulated 4-like (NEDD4L) is a candidate gene for hypertension, and carriers of an intact NEDD4L C2-domain,encoded by the NEDD4L rs4149601 (G/A) GG genotype, together with the C-allele of the NEDD4L rs2288774 (C/T) polymorphism have been found be associated with hypertension both in African Americans and whites. However, there is no data on the relationship between polymorphism of NEDD4L rs4149601 and rs2288774 and hypertension in Chinese chronic kidney disease (CKD) patients. The purpose of the current study was to investigate the relationship between the variation of NEDD4L rs4149601, rs2288774 and hypertension in CKD patients. Methods: A total of 546 Chines Hans CKD patients were enrolled in our study. The SNPs were genotyped using PCR-based techniques. All patients underwent ambulatory blood pressure monitoring, and clinical data were also collected. Multivariate logistic regression analysis was used to identify the relationship between polymorphisms and hypertension. Results: 506 patients carried GG/GA genotype and 30 carried AA genotype. Rs4149601 AA genotype carriers had significantly higher rate of hypertension (68.3% vs 46.2%, P = 0.022) than GG/GA genotype carriers by Chi-squared test. AA genotype carriers also had a higher day-time and bedtime systolic blood pressure (142±16 vs 135±23, P=0.036; 137±18 vs 127±13, P=0.022, respectively) when compared with GG/GA genotype carriers. AA genotype [OR= 3.08, 95% CI (1.06-9.80)], lowever eGFR [OR=0.98, 95% CI (0.97-0.99)], older age [OR=1.03, 95% CI (1.01-1.05)] were independently associated with hypertension in CKD patients by multivariate logistic regression. However, No difference was found in blood pressure with rs2288774 TT/TC/CC genotypes, and no difference was found in the incidence of hypertension among patients with three genotypes. Conclusions: Our results suggested 4149601AA genotype of NEDD4L may be associated with hypertension in CKD patients, and further genetic and functional studies are required to understand its role in the manifestation of hypertension in Chinese CKD patients.


2018 ◽  
Vol 26 (9) ◽  
pp. 1210-1217 ◽  
Author(s):  
Mathilde Bourdon ◽  
Pietro Santulli ◽  
Yulian Chen ◽  
Catherine Patrat ◽  
Khaled Pocate-Cheriet ◽  
...  

Objective: The aim of this study was to assess whether a deferred frozen–thawed embryo transfer (Def-ET) offers any benefits compared to a fresh ET strategy in women who have had 2 or more consecutive in vitro fertilization (IVF)/intracytoplasmic injection (ICSI) cycle failures. Design: An observational cohort study in a tertiary referral care center including 416 cycles from women with a previous history of 2 or more consecutive IVF/ICSI failures cycles. Both Def-ET and fresh ET strategies were compared using univariate and multivariate logistic regression models. The main outcome measured was the cumulative live birth rate (CLBR). Results: A total of 416 cycles were included in the analysis: 197 in the fresh ET group and 219 in the Def-ET group. The CLBR was not significantly different between the fresh and Def-ET groups (58/197 [29.4%] and 57/219 [26.0%], respectively, P = .437). In addition, after the first ET, there was no significant difference in the live birth rate between the fresh ET and Def-ET groups (50/197 [25.4%] vs 44/219 [20.1%], respectively). Multivariate logistic regression analysis indicated that compared to the fresh strategy, the Def-ET strategy was not associated with a higher probability of live birth. Conclusions: In cases with 2 or more consecutive prior IVF/ICSI cycle failures, a Def-ET strategy did not result in better ART outcomes than a fresh ET strategy.


2021 ◽  
Vol 28 (1) ◽  
pp. 21-27
Author(s):  
Mark S. Dias ◽  
Ming Wang ◽  
Elias B. Rizk ◽  
Robin Bowman ◽  
Michael D. Partington ◽  
...  

OBJECTIVE The aims of this study were to review the National Spina Bifida Patient Registry (NSBPR) data set to study the rates of tethered spinal cord release (TCR) among patients with myelomeningocele and variability between centers, to compare TCR rates between males and females, and to study the relationships between TCR rates and other condition-specific characteristics. METHODS The NSBPR registry was queried to identify all patients with myelomeningocele. TCR rates were calculated over time using survival analyses; rates between centers and between males and females were compared. Cox proportional hazards models were constructed to identify relationships between TCR rates and sex, functional lesion level, ambulation status, treated hydrocephalus, and prior Chiari decompression. RESULTS Of 6339 patients with information about their operations, 1366 (21.5%) underwent TCR, with significant variability between centers. The majority (75.8%) underwent a single TCR. The annual TCR rate was linear between birth and 13 years (1.8%/year) but declined sharply from 14 to 21 years (0.7%/year). There was no period of time at which the TCR rate accelerated. There were no significant differences in TCR rates between males and females. TCR rate was not related to functional lesion level but was lower among nonambulators compared with community ambulators (p = 0.005) and among those with treated hydrocephalus (HR 0.30, p < 0.001), and higher among those having prior Chiari decompression (HR 1.71, p < 0.001). CONCLUSIONS These results extend the results of prior single-institution studies, demonstrate significant treatment variability between institutions, and challenge the traditional concept that tethering is related to spinal cord stretching due to spinal growth.


2019 ◽  
Vol 32 (7-8) ◽  
pp. 554-563 ◽  
Author(s):  
Zachary A. Morris

Objective: The objective of this study is to examine the relationship between loneliness and work disability and whether depression mediates the hypothesized relationship. Method: We draw on data from the 2013 and 2015 waves of the Survey of Health, Aging, and Retirement in Europe. We limited the sample to adults between the ages of 50 and 65 who were working and without work limitations in 2013, which consisted of 10,154 adults from 14 countries. We apply multivariate logistic regression and a binary mediation analysis with logistic regression to examine predictors of the onset of work disability in 2015. Results: Loneliness was predictive of future work disability onset when adjusting for other factors in the disablement process. Depression partly mediated the longitudinal relationship between loneliness and work disability. Discussion: The results indicate that addressing loneliness could mitigate the risk of depression and, in turn, work disability onset.


2019 ◽  
Vol 24 (01) ◽  
pp. 6-12
Author(s):  
H.R. Smith ◽  
C. Conyard ◽  
J. Loveridge ◽  
R. Gunnarsson

Background: Tooth knuckle injuries can be expensive to treat and may necessitate amputation in some cases. Several limitations exist in the literature regarding our knowledge around the factors predicting amputation and the need for multiple debridements in treating this injury.Methods: A historic cohort study of 321 patients treated for tooth knuckle injuries was undertaken. Twenty-one demographic, clinical and laboratory variables were collected. Two outcome measurements were collected - the need for amputation and the need for more than one surgical debridement. A multivariate logistic regression was performed to determine the relationship between the predictor variables and the outcome measurements.Results: Of the 321 patients examined, 1.6% required amputations and 25% required multiple debridements. Osteomyelitis was found to be a major predictor for amputation in these patients (OR = 35). Delayed presentation (OR = 1.1) and diabetes (OR = 2.6) were found to significantly increase the risk of requiring multiple debridements.Conclusions: Our models were able to predict what patients were at the greatest risk for amputation and multiple debridement. Reducing rates of osteomyelitis and delays in presentation may help reduce the incidence of amputation and reoperation in this injury.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2209-2209
Author(s):  
Dick Chung ◽  
Lily Ding ◽  
Isabelle Amigues ◽  
Katuna Kadeishvili ◽  
Theresa Lo ◽  
...  

Abstract Background: Low MBL2 concentration and MBL2 genotype variants have been associated with an increased risk of infection in various clinical settings. Pulmonary infection is a major complication of HSCT. We examined the relationship of MBL genotypes with post-engraftment bacterial (B-PNA) and fungal (F-PNA) pneumonia Methods: Retrospective review of 236 non-consecutive, non-selected patients who underwent HSCT at MSKCC from 1/1/2000–4/30/2007. Microbiologically confirmed infections and pneumonias were recorded. Antifungal prophylaxis consisted of fluconazole 400 mg daily. Patients at high risk for mold infection received mold-active prophylaxis. After 1/1/2006 voriconazole was the first line anti-mold prophylaxis. Genotype was determined by PR-Melting Curve Analysis on blood or buccal swab specimens. MBL genotype was classified as wild-type: A/A (MBL-sufficient, MBL-S) or variant-type: A/O, O/O (MBL deficient, MBL-D). Patients were followed for up to 2 years. Statistical analysis: Fisher’s Exact test was used to compare the incidence rate between MBL-S and MBL-D patients. Multivariate logistical regression models were used to investigate the relationship between bacterial or fungal pneumonia and MBL genotype, matched related donor (MRD), myeloablative conditioning (MC) peripheral blood as stem cell source (PBSC), acute GVHD grade 2–4 (aGVHD). The results from Maximum Likelihood Estimates were summarized. Results: Transplant characteristics: 80% MC, 76% PBSC, 48.6% MRD. Incidence of aGVHD: 22.4%. MBL genotypes: One-hundred and forty-two (60%) patients were homozygous for wild-type MBL2 (AA), 85(36%) were heterozygous (A/0) and 9 (3.8%) were homozygous for variant genotypes (OO). Transplant characteristics, rates of GVHD, relapse-free and overall survival were similar between MBL-D and MBL-S. There was higher incidence of overall bacterial infections in MBL-D compared to MBL-S pts (47.87% vs 36.62%, p=0.1049). MBL-D had a higher incidence of B-PNA (12.7% vs 4.9%, p=0.048). In multivariate logistic regression analysis, MBL-D(p=0.04) and aGVHD(p=0.06) were likely associated with B-PNA. Rates of overall fungal infections and F-PNA were similar [among MBL-D and MBL-S pts (12.77% vs 9.86%, p=0.5277) and (8.51% vs 7.75%, p=0.1049) respectively]. In multivariate logistic regression analysis only aGVHD was statistically significantly associated with F-PNA p=0.0002. Conclusions: 1) MBL-D genotype was likely associated with increased risk of bacterial pneumonia. 2) MBL-D and aGVHD were risk factors for B-PNA. 3) Further analyses are in progress to evaluate the effect of MBL-D on fungal pneumonia in patients who received mold prophylaxis versus patients who did not receive mold prophylaxis. 4) Prospective studies are needed to assess the relative contribution of MBL-D genotype on the risk of pulmonary infection in HSCT.


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