Efficient Stroke Prevention Based on Early Detection of Cerebral Vasculopathy by Transcranial Doppler: The Creteil Newborn-Screened Sickle Cell Cohort.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 1212-1212
Author(s):  
Francoise Bernaudin ◽  
Suzanne Verlhac ◽  
Lena Coic ◽  
Emmanuelle Lesprit ◽  
Cecile Arnaud ◽  
...  

Abstract About 11% of SS patients will have a first stroke by age 18–20 years. Abnormal high velocities are associated with a high risk of stroke, which can be significantly reduced by a long-term transfusion program. The stroke rate in Californian SCD children has declined since the advent of transcranial doppler (TCD), but there are no reports on the outcome of patients screened from birth by TCD. Since 1992, TCD has been systematically performed from the age of 12–18 months in the Creteil cohort. MRI/MRA was performed annually after the age of 5 years, or earlier in case of abnormal TCD. Patients with abn. high velocities (TAMMX >= 200 cm/s) were offered transfusion regimens for stroke prevention, but hydroxyurea was proposed secondarily to patients who had normal MRA findings and whose velocities normalized (< 170 cm/s). TCD was performed every three months in these patients, and a transfusion program was resumed if the findings were again abnormal. We report the cerebrovascular outcome of patients born after 1988 who were regularly followed in our center and screened by TCD. This cohort consisted of 242 SCD patients (178 SS, 9 Sb0, 41 SC, and 14 Sb+). Mean follow-up was 6.2 y (range 0.1–17.8), representing 1498 patient-years overall and 1139 patient-years in the SS/Sb0 population. Alpha gene deletion was present in 41% of patients (71/175). The beta genotype was available in 165 patients, and was homozygous Bantou (n=56, 34%), Senegal (n=9, 5.5%), Benin (n=35, 21.2%), or "other" (n=65, 39.4%). Four deaths occurred, all involving SS patients, at 1.2 y (enteritis in 1995), 1.8 y (trip to Africa in 2003), 1.9 y (pneumo. meningitis in 1997) and 3.7 y (ACS and curare allergy in 1998). The mortality rate was 0.27 per 100 patient-years overall and 0.35 in SS/Sb0 patients. The Kaplan-Meier (KM) estimate of the risk of abn. velocities (> 200 cm/s) was 15.7% at 5 y and 22.7% at 10 y of age in SS/Sb0 patients. No SC/Sb+ patients had abn. velocities. Strokes occurred in only two SS patients: the first patient had abn. high velocities detected at the age of 1.5 years and had a stroke at the age of 1.6 years, just before the TCD control and initiation of the transfusion program. The second patient had normal left-sided velocities but no available temporal window on the right side after the age of 1.4 y; this patient had a stroke at age 4.4 y. These observations prompted us to begin TP after the first abn. TCD and to perform MRI/MRA when no window was available. The rates of stroke were 0.13 per 100 patient-years overall, and and 0.18 per 100 patients-years in SS/Sb0 patients. The KM estimate of the risk of stroke was 1.02% at 5 y and 18 y of age in SS/Sb0 patients. KM estimates of the risk of ischemic lesions at 5 and 10 y were respectively 6.4% and 17.7% in SS/Sb0 patients with normal TCD and 20.9% and 43.5% in patients with a history of abn. TCD (Log Rank test 0.0035). This single-center study shows that a strategy based on early and regular TCD can efficiently prevent stroke in a newborn screened cohort: the rate of stroke and the KM risk of stroke were far lower than recently reported (Quinn CT Blood 2004). However, the risk of ischemic lesions remained high particularly in patients with a history of abn. velocities. Abn. TCD velocities identify a high-risk group who may qualify for matched related donor stem cell transplantation.

2013 ◽  
Vol 31 (6_suppl) ◽  
pp. 318-318 ◽  
Author(s):  
Daniel Levi Willis ◽  
Mario Fernandez ◽  
Rian J. Dickstein ◽  
Sahil Parikh ◽  
Arlene O. Siefker-Radtke ◽  
...  

318 Background: Micropapillary bladder cancer (MPBC) is an uncommon and aggressive variant of urothelial carcinoma for which the role of neoadjuvant chemotherapy (NAC) is not well-defined. Here we report a retrospective analysis of patients with MPBC undergoing radical cystectomy (RC) with and without NAC. Methods: An IRB-approved review of our radical cystectomy database demonstrated 159 patients with a preoperative diagnosis of MPBC. Of these, 131 patients presented with surgically resectable (≤cT4aN0M0) disease and form the basis of this report. Disease-specific (DSS) and overall survival (OS) were estimated using the Kaplan-Meier estimator and compared by log-rank test. Results: The clinical stage breakdown was cT1:50; cT2:66; cT3:15; and cT4a:0. NAC was administered to 61 patients (47%) with 78% receiving cisplatin-based regimens. Patients were more likely to receive NAC if they presented with cT3 disease, hydronephrosis, or lymphovascular invasion (LVI) in the transurethral resection (TUR) specimen. After a median follow-up of 44 mo., 50% of patients recurred and 41% died of disease, resulting in a 5-year estimated DSS of 58.4% for all 131 patients. Survival analysis was performed for comparable groups according to our established risk factors. For the low risk patients (i.e. cT1-T2, no hydronephrosis, and no LVI) RC upfront (n=47) resulted in a 73% 5-yr DSS compared to 83% with NAC (n=19) (p=0.47). In the high-risk group (cT3 or cT2 with LVI and/or hydronephrosis), 41 patients were treated initially with NAC and 5-yr DSS was 40%, which was similar (p=0.74) to the 31% 5-yr DSS for those treated with RC upfront (n=20). The most important overall prognostic factor was pathologic downstaging to pT0, pTa, or pTis at cystectomy (seen in 52% after NAC and 19% after TUR alone) which conferred a significant survival advantage (5-yr DSS 93% vs. 40% in those not downstaged; p<0.001). Conclusions: Despite incorporation of a multimodal treatment strategy, patients with MPBC and high risk features (LVI, hydronephrosis and/or cT3 stage) have poor outcomes. Further studies are necessary to define the optimal treatment strategy in this challenging subset of patients with urothelial cancer.


2021 ◽  
Vol 122 (4) ◽  
pp. 294-299
Author(s):  
Yavuz Onur Danacıoğlu ◽  
Yusuf Arıkan ◽  
Fatih Akkaş ◽  
Emre Şam ◽  
Deniz Noyan Özlü ◽  
...  

Percutaneous nephrolithotomy (PNL) surgeries are performed with different patient positions, anesthesia methods and different-sized access sheaths in order to reduce the complication rates. Supine positioned PNL can be performed safely in the high-risk group patients with comorbidities. Herein, we present a patient who had a past surgical history of right pneumonectomy and underwent a supine PNL procedure under regional anesthesia for a staghorn renal stone in the right kidney.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Annette Bley ◽  
Jonas Denecke ◽  
Alfried Kohlschütter ◽  
Gerhard Schön ◽  
Sandra Hischke ◽  
...  

Abstract Background Canavan disease (CD, MIM # 271900) is a rare and devastating leukodystrophy of early childhood. To identify clinical features that could serve as endpoints for treatment trials, the clinical course of CD was studied retrospectively and prospectively in 23 CD patients. Results were compared with data of CD patients reported in three prior large series. Kaplan Meier survival analysis including log rank test was performed for pooled data of 82 CD patients (study cohort and literature patients). Results Onset of symptoms was between 0 and 6 months. Psychomotor development of patients was limited to abilities that are usually gained within the first year of life. Macrocephaly became apparent between 4 and 18 months of age. Seizure frequency was highest towards the end of the first decade. Ethnic background was more diverse than in studies previously reported. A CD severity score with assessment of 11 symptoms and abilities was developed. Conclusions Early hallmarks of CD are severe psychomotor disability and macrocephaly that develop within the first 18 months of life. While rare in the first year of life, seizures increase in frequency over time in most patients. CD occurs more frequently outside Ashkenazi Jewish communities than previously reported. Concordance of phenotypes between siblings but not patients with identical ASPA mutations suggest the influence of yet unknown modifiers. A CD severity score may allow for assessment of CD disease severity both retrospectively and prospectively.


BMC Urology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Zoë G. Baker ◽  
Arthi Hannallah ◽  
Melissa Trabold ◽  
Danielle Estell ◽  
Cherry Deng ◽  
...  

Abstract Background Hydronephrosis (HN) is the most common abnormality detected on prenatal ultrasound. This study sought to stratify outcomes of patients by severity of prenatal HN with postnatal outcomes. Methods This was a retrospective review of patients referred to a tertiary care fetal-maternal clinic with diagnosis of prenatal HN from 2004 to 2019. HN severity was categorized as mild, moderate, or severe. Data were analyzed to determine the association between HN severity and surgical intervention. Decision for surgery was based on factors including history of multiple urinary tract infections, evidence of renal scarring, and/or reduced renal function. Surgery-free survival time was represented by the Kaplan–Meier method, and hazard ratios were calculated using the log-rank test. Results 131 kidneys among 101 infants were prenatally diagnosed with hydronephrosis; 35.9% had mild HN, 29.0% had moderate HN, and 35.1% had severe HN. 8.5% of patients with mild HN, 26.3% of patients with moderate HN, and 65.2% of patients with severe HN required surgery. Patients with severe HN were 12.2 (95% CI 6.1–24.4; p < 0.001) times more likely to undergo surgery for HN than patients with mild HN and 2.9 (95% CI 1.5–5.3; p = 0.003) times more likely to undergo surgery than patients with moderate HN. Patients with moderate HN were 4.3 times more likely to require surgery than patients with mild HN (95% CI 1.5–12.9; p = 0.01). Median age at surgery was 11.8 months among patients with mild HN (IQR 11.7–14.1 months), 6.6 months among patients with moderate HN (IQR 4.2–16.4 months), and 5.4 months among patients with severe HN (3.7–12.4 months). Conclusion Among this cohort of referrals from a fetal-maternal clinic, severity of HN correlated with increased likelihood of surgical intervention. Continued assessment of patients with prenatal HN should be evaluated to best determine the role of the pediatric urologist in cases of prenatal HN.


2021 ◽  
Author(s):  
Yuichi Kojima ◽  
Sho Nakakubo ◽  
Keisuke Kamada ◽  
Yu Yamashita ◽  
Nozomu Takei ◽  
...  

SummaryBackgroundAlthough biological agents, tocilizumab and baricitinib, have been shown to improve the outcomes of patients with COVID-19, a comparative evaluation has not been performed.MethodsA retrospective, single-center study was conducted using the data of patients with COVID-19 admitted to the Hokkaido University hospital between April 2020 and September 2021, who were treated with tocilizumab or baricitinib. The clinical characteristics of patients who received each drug were compared. Univariate and multivariate logistic regression models were performed against the outcomes of all-cause mortality and the improvement in respiratory status. The development of secondary infection events was analyzed using the Kaplan–Meier analysis and the log-rank test.ResultsThe use of tocilizumab or baricitinib was not associated with all-cause mortality and the improvement in respiratory status within 28 days of drug administration. Age, chronic renal disease, and comorbid respiratory disease were independent prognostic factors for all-cause mortality, while anti-viral drug use and severity of COVID-19 at baseline were associated with the improvement in respiratory status. There was no significant difference in the infection-free survival between patients treated with tocilizumab and those with baricitinib.ConclusionThere were no differences in efficacy and safety between tocilizumab and baricitinib for the treatment of COVID-19.


2021 ◽  
Author(s):  
juanjuan Qiu ◽  
Li Xu ◽  
Yu Wang ◽  
Jia Zhang ◽  
Jiqiao Yang ◽  
...  

Abstract Background Although the results of gene testing can guide early breast cancer patients with HR+, HER2- to decide whether they need chemotherapy, there are still many patients worldwide whose problems cannot be solved well by genetic testing. Methods 144 735 patients with HR+, HER2-, pT1-3N0-1 breast cancer from the Surveillance, Epidemiology, and End Results database were included from 2010 to 2015. They were divided into chemotherapy (n = 38 392) and no chemotherapy (n = 106 343) group, and after propensity score matching, 23 297 pairs of patients were left. Overall survival (OS) and breast cancer-specific survival (BCSS) were tested by Kaplan–Meier plot and log-rank test and Cox proportional hazards regression model was used to identify independent prognostic factors. A nomogram was constructed and validated by C-index and calibrate curves. Patients were divided into high- or low-risk group according to their nomogram score using X-tile. Results Patients receiving chemotherapy had better OS before and after matching (p < 0.05) but BCSS was not significantly different between patients with and without chemotherapy after matching: hazard ratio (HR) 1.005 (95%CI 0.897, 1.126). Independent prognostic factors were included to construct the nomogram to predict BCSS of patients without chemotherapy. Patients in the high-risk group (score > 238) can get better OS HR 0.583 (0.507, 0.671) and BCSS HR 0.791 (0.663, 0.944) from chemotherapy but the low-risk group (score ≤ 238) cannot. Conclusion The well-validated nomogram and a risk stratification model was built. Patients in the high-risk group should receive chemotherapy while patients in low-risk group may be exempt from chemotherapy.


Stroke ◽  
2013 ◽  
Vol 44 (suppl_1) ◽  
Author(s):  
Yasuhiro Kumai ◽  
Takuya Kiyohara ◽  
Masahiro Kamouchi ◽  
Sohei Yoshimura ◽  
Hiroshi Sugimori ◽  
...  

Background and Purpose— ABCD 2 score has been developed to predict the early risk of stroke after transient ischemic attack (TIA). The aim of this study was to clarify whether ABCD 2 score predicts the occurrence of stroke in the long term after TIA. Methods— Fukuoka Stroke Registry (FSR) is a multicenter epidemiological study database on acute stoke. From June 2007 to June 2011, 496 (305 males, 70 ± 13 years of age) patients who had suffered from TIA and were hospitalized in the 7 stroke centers within 7 days after the onset of TIA were enrolled in this study. The patients were divided into three groups according to the risk: low-risk (ABCD 2 score 0-3; n=72), moderate-risk (4-5; n=229) and high-risk group (6-7; n=195). They were followed up prospectively for up to 3 years. Cox proportional hazard regression model was used to elucidate whether ABCD 2 score was a predictor for stroke after TIA after adjusting for confounding factors. Results— Among three groups, there were significant differences in age, hypertension, diabetes mellitus and the decrease in estimated glomerular filtration rate (P<0.01, significantly). During a mean follow-up of 1.3 years, Kaplan-Meier analysis demonstrated that the stroke rate in TIA patients was significantly lower in low-risk group than in moderate-risk or high-risk group (log rank test, p<0.001). The adjusted hazard ratios for stroke in patients with TIA increased with moderate-risk group (Hazard ratio [HR]: 3.47, 95% CI: 1.03-21.66, P<0.05) and high-risk group (HR: 4.46, 95% CI: 1.31-27.85, P<0.05), compared to low-risk group. Conclusions— The ABCD 2 score is able to predict the long-term risk of stroke after TIA.


2020 ◽  
Author(s):  
FengLing Shao ◽  
Zhenni Wang ◽  
Shan Wang

Abstract BackgroundDue to the extremely high mortality rate of children with high-risk Neuroblastoma (NB), there is an urgent need for new indicators to further classify children in the high-risk group for more precise treatment. The purpose of our research is to explore the immune-related genes in NB in the high-risk group, and to further identify and develop a prognostic nomogram based on immune IRG signatures. MethodsThrough bioinformatics analysis to explore the abnormal expression of immune-related genes in the high-risk group. Cox regression and the least absolute shrinkage and selection operator (LASSO) analysis were conducted to identify the immune and overall survival (OS) related mRNA. The accuracy of the risk score is evaluated by Kaplan-Meier method and receiver operating characteristics (ROC) analysis, which is used to build a nomogram in combination with other clinical characteristics.. Quantitative real-time polymerase chain reaction (qRT-PCR) was conducted to detect the accuracy of our results. ResultsA total of 127 common differentially expressed immune genes were found between the high-risk group and the non-high-risk group of the two data sets. Four immune-related genes (IRG) related to prognosis were identified and a risk score was established. Kaplan–Meier survival analysis and time-dependent ROC analysis showed that the 4-IRG risk score has satisfactory predictive potential and achieved consistency in the verification of external data sets. Subsequently, the risk score combined with clinical characteristics draws a nomogram. The reliability of the results was verified on 29 cases of NB tissues by qRT-PCR. ConclusionsOverall, we have developed a powerful multi-gene classifier that can effectively classify NB patients into low- and high-risk groups with poor prognosis, and draw a nomogram for children in the high-risk group. This feature can help select high-risk patients who need more aggressive adjuvant target therapy or immunotherapy.


2012 ◽  
Vol 28 (3) ◽  
pp. 168-173 ◽  
Author(s):  
F. Bat-Pitault ◽  
D. Da Fonseca ◽  
S. Cortese ◽  
Y. Le Strat ◽  
L. Kocher ◽  
...  

AbstractObjectiveThe primary aim of this study was to compare the sleep macroarchitecture of children and adolescents whose mothers have a history of depression with children and adolescents whose mothers do not.MethodPolysomnography (PSG) and Holter electroencephalogram (EEG) were used to compare the sleep architecture of 35 children whose mothers had at least one previous depressive episode (19 boys, aged 4–18 years, “high-risk” group) and 25 controls (13 males, aged 4–18 years, “low-risk” group) whose mothers had never had a depressive episode. The total sleep time, wakefulness after sleep onset (WASO), sleep latency, sleep efficiency, number of awakenings per hour of sleep, percentages of time spent in each sleep stage, rapid eye movement (REM) latency and the depressive symptoms of participants were measured.ResultsIn children (4–12 years old), the high-risk group exhibited significantly more depressive symptoms than controls (P = 0.02). However, PSG parameters were not significantly different between high-risk children and controls. In adolescents (13–18 years old), the high-risk subjects presented with significantly more depressive symptoms (P = 0.003), a significant increase in WASO (P = 0.019) and a significant decrease in sleep efficiency compared to controls (P = 0.009).ConclusionThis study shows that children and adolescents born from mothers with a history of at least one depressive episode had significantly more depressive symptoms than controls. However, only high-risk adolescents presented with concurrent alterations of sleep macroarchitecture.


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