Long Term Follow-Up of Pediatric Sickle Cell Disease Patients with Conditional Velocities on Transcranial Doppler (TCD).

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2338-2338
Author(s):  
Lena Coïc ◽  
Suzanne Verlhac ◽  
Emmanuelle Lesprit ◽  
Emmanuelle Fleurence ◽  
Francoise Bernaudin

Abstract Abnormal TCD defined as high mean maximum velocities > 200 cm/sec are highly predictive of stroke risk and justify long term transfusion program. Outcome and risk factors of conditional TCD defined as velocities 170–200 cm/sec remains to be described. Patients and methods Since 1992, 371 pediatric SCD patients (303 SS, 44 SC, 18 Sß+, 6 Sß0) were systematically explored once a year by TCD. The newborn screened cohort (n=174) had the first TCD exploration between 12 and 18 months of age. TCD was performed with a real-time imaging unit, using a 2 MHz sector transducer with color Doppler capabilities. Biological data were assessed at baseline, after the age of 1.5 years and remotely of transfusion or VOC. We report the characteristics and the outcome in patients (n=43) with an history of conditional TCD defined by mean maximum velocities ranging between 170 and 200 cm/s in the ACM, the ACA or the ICA. Results: The mean follow-up of TCD monitoring was 5,5 years (0 – 11,8 y). All patients with an history of conditional doppler were SS/Sb0 (n=43). Mean (SD) age of patients at the time of their first conditional TCD was 4.3 years (2.2) whereas in our series the mean age at abnormal TCD (> 200 cm/sec) occurrence was 6.6 years (3.2). Comparison of basal parameters showed highly significant differences between patients with conditional TCD and those with normal TCD: Hb 7g4 vs 8g5 (p<0.001), MCV 82.8 vs 79 (p=0.047). We also had found such differences between patients with normal and those with abnormal TCD (Hb and MCV p< 0.001). Two patients were lost of follow-up. Two patients died during a trip to Africa. Conditional TCD became abnormal in 11/43 patients and justified transfusion program. Mean (SD) conversion delay was 1.8 (2.0) years (range 0.5–7y). No stroke occurred. 16 patients required a treatment intensification for other indications (frequent VOC/ACS, splenic sequestrations): 6 were transplanted and 10 received HU or TP. Significant risk factors (Pearson) of conversion to abnormal were the age at time of conditional TCD occurrence < 3 y (p<0.001), baseline Hb < 7g/dl (p=0.02) and MCV > 80 (p=0.04). MRI/MRA was performed in 31/43 patients and showed ischemic lesions in 5 of them at the mean (SD) age of 7.1 y (1.8) (range 4.5–8.9): no significant difference was observed in the occurrence of lesions between the 2 groups. Conclusions This study confirms the importance of age as predictive factor of conditional to abnormal TCD conversion with a risk of 64% when first conditional TCD occured before the age of 3 years. TCD has to be frequently controled during the 5 first years of life.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e11616-e11616
Author(s):  
Barbara Pistilli ◽  
Andrea Marcellusi ◽  
Michele Valeri ◽  
Umberto Torresi ◽  
Dania Nacciarriti ◽  
...  

e11616 Background: Continuing T beyond progression has become a common strategy in the treatment of human epidermal growth receptor 2- overexpressing (HER2) MBC. However, T administered for several years with concomitant chemotherapy elicits concern about cardiac safety especially in patients (pts) with risk factors. Methods: Cardiac events (CEs) and survival of HER2 MBC pts treated with T +/- chemotherapy at our institution from Dec 2003 to Jun 2012 were evaluated. CEs were graded by NCI-CTCAE v 3.0. Risk factors assessed for cardiotoxicity were: age, body mass index, antihypertensive therapy, history of cardiac disease, diabetes, hypothyroidism, smoking, prior radiotherapy on the chest wall, prior cumulative dose of anthracycline(A), interval between last A dose and first T dose, baseline LVEF, continued/interrupted T exposure, concomitant chemotherapy. Chi-square test was used to compare distribution of CEs over different times of T exposure (p≤ 0.05). Univariate and multivariate Cox regression analysis were used to assess the effect of risk predictors. Results: Sixty-two pts assessable. Median age 52 years (range, 29 to 76), median cumulative time receiving T 29.5 months (range, 3 to 99 months); 40 pts (64.5%) received T without interruption and 19 pts (30.6%) were treated for more than 36 months. CEs occurred in 11 out of all pts (17.7%): grade 1 in 3 pts (4.8%), grade 2 in 5 (8.1%) and grade 3 in 3 (4.8%). The rate of CEs showed no statistically significant difference in pts receiving T for up to 36 months and over: 7/43 (16.3%) and 4/19 (21%), respectively, (p =0.724). In univariate Cox regression analysis significant risk factors were: history of cardiac disease (HR 6,814, 95% CI: 1,384-33,542) and smoking (HR 5,228, 95% CI: 1,403-19,491). In multivariate analysis smoking was the only independent predictor (HR 5,886, 95% CI: 1,479-23,247). Median survival from MBC diagnosis was 50 months (range, 6 to 101 months). Conclusions: Despite the limited sample size, our analysis suggests that cardiotoxicity does not hamper a long-term use of T, since the rate of CEs did not increase in pts treated over 36 months. Moreover, smoking appears to be a predictive factor of T cardiotoxicity.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3196-3196
Author(s):  
Francoise Bernaudin ◽  
Emmanuelle Lesprit ◽  
Lena Coïc ◽  
Cécile Arnaud ◽  
Emmanuelle Fleurence ◽  
...  

Abstract Treatment intensifications in SCD with HU, TP or SCT are applied in order to reduce SCD related complications but their comparative effects have still to be described. We report our experience concerning the annual check-up performed in SCD pediatric patients. Patients and Methods: Among our cohort of 397 SS/Sb0 pediatric SCD patients, 157 of them were intensified with HU (n= 86), TP (n=104) or SCT (n=36) and some of them received successively HU, TP and SCT. HU was proposed to patients > 3 years of age and having experienced more than 3 VOC/ACS/year or < 7g/dl severe anemia. TP defined as > 4 months program was applied in patients with cerebral vasculopathy defined by an history of stroke or abnormal TCD (> 200 cm/sec). TP was also proposed in patients with HU-failure and in patients with frequent VOC, less than 3 years old. SCT was proposed in patients with an indication of treatment intensification and an available HLA identical sibling donor. Annual check-up were performed in our day-care unit. We analysed 1261 check-ups performed and recorded since 1992 in 341 SS/Sb0 patients (sex: 164 F, 177 M). Median age was 8.8 ± 5.1 years. Mean number of annual check-ups per patient was 3.7 ± 2.8 (range 1 to 13): 816 were performed in non intensified patients, 196 in HU, 123 in TP and 126 in transplanted patients. Categories of age were distinguished: < 2 y of age (n=110), 2–5y (n=244), 5–10y (n=415), 10–15y (n=317) and 15–20y (n=175). Results: Respective follow-up were 4.4 y ± 3.3 in HU, 2.6 y ± 2.6 inTP and 5.8 y ± 4.7 in SCT patients. Comparison with non intensified patients showed that weight was significantly higher in SCT patients > 15 y of age (p=0.001), spleen size was significantly higher in (2–5y) young patients treated with HU (p=0.005) or TP (0.001) and in 5–10 y old patients on HU (p=0.046) but no difference was observed after the age of 10 y. O2 saturation was significantly improved after SCT (p<0.001) (98.8 ± 1.0 vs 97.1 ± 2.6) and was unchanged on HU and TP. Cardiac pulsations were significantly (p<0.001) decreased after all type of intensification. Biological data are shown (table1and 2). Conclusion : Treatment intensifications (TP, HU, SC) reduced the decrease of weight observed with aging in SCD patients and significantly reduced anemia using different mechanisms. SCT was the most effective to correct anemia, supress hemolysis and decrease leucocytosis. Intensif. n Follow-up HbF% Eryht Hb MCV Retic mean (SD) No 816 11.4 (9.2) 3.1 (0.9) 8.1 (1.2) 81.4 (8.9) 268.9 (105.2) HU 196 4.4 y. (3.3) 13.9 (7.0) 2.7 (0.6) 8.5 (1.2) 97.7 (13.7) 188 (83.8) TP 126 2.6 y.(2.7) 3.3 (3.1) 3.1 (0.6) 9.1 (1.4) 86.8 (4.8) 258.2 (126.0) SCT 123 5.8 y.(4.7) 4.6 (6.4) 4.3 (0.9) 11.4 (1.6) 81.5 (8.9) 89.4 (63.4) Intensif. n Tot Bili Conj Bili LDH Ferritin Leucocytes Platelets No 816 49.8 (34.4) 5.7 (3.4) 1016 (312) 192 (322) 13.2 (9.9) 385 (124) HU 196 47.5 (34.4) 5.0 (2.2) 943 (264) 399 (582) 9.7 (3.8) 352 (133) TP 126 58.8 (39.6) 5.6 (2.2) 973 (377) 2238 (6310) 13.1 (4.7) 365 (128) SCT 123 15.6 (13.9) 2.8 (4.2) 493 (200) 1099 (1386) 6.8 (3.3) 295 (109)


2020 ◽  
Vol 24 (6) ◽  
pp. 606-611
Author(s):  
Y. Li ◽  
Z. Jia ◽  
S. Li ◽  
Y. Huang ◽  
X. Yuan ◽  
...  

OBJECTIVE: To assess factors associated with long-term haemoptysis recurrence after transarterial embolisation (TAE) for haemoptysis due to bronchiectasis.METHODS: Patients with haemoptysis due to bronchiectasis who underwent TAE between May 2010 and May 2019 were included in this retrospective study. Long-term haemoptysis recurrence was defined as the expectoration of >10 mL/day of fresh blood (for at least 1 day) 1 month after TAE. Univariate and multivariate analyses were performed to identify risk factors for long-term haemoptysis recurrence after TAE.RESULTS: A total of 197 patients (108 women; mean age, 61.0 ± 12.2 years) were included in the study. TAE was performed successfully in all patients. Side effects occurred in 43 (21.8%) patients, and all patients recovered uneventfully. During 37.6 ± 11.6 months of follow-up, long-term haemoptysis recurrence occurred in 41 (20.8%) patients; the mean interval between the TAE and haemoptysis recurrence was 21.4 ± 16.3 months. Long-term haemoptysis recurrence after TAE was associated with a history of haemoptysis (OR 3.483, 95% CI 1.373–8.836; P = 0.009).CONCLUSIONS: Approximately one fifth patients with bronchiectasis had long-term haemoptysis recurrence after TAE. Risk factor for long-term haemoptysis recurrence after TAE was a history of haemoptysis.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 2200-2200
Author(s):  
Agnes Y. Lee ◽  
Carolyn Webb ◽  
Qing Guo ◽  
Lorrie Costantini ◽  
Greg Butler ◽  
...  

Abstract Long-term indwelling central venous catheters (CVCs) are used for delivering chemotherapy, parenteral nutrition, antibiotics, and blood products, as well as for facilitating blood drawing, in many patients with malignancy. Although the important supportive role of CVCs is unquestioned, there is uncertainty regarding the prevention and treatment of catheter-related thrombosis (CRT) because there is a lack of prospective and contemporary data on the natural history of this complication. As a first step towards improving CRT management, we conducted a prospective cohort study to examine the incidence, clinical risk factors, and the long-term sequelae of symptomatic CRT in adults with cancer. Consecutive patients undergoing insertion of a CVC at a tertiary care center were enrolled and followed for the duration of their catheter-dwell time plus 4 weeks or a maximum of 52 weeks, whichever comes first. Scheduled assessments were done at weeks 1, 2, 4, 8, 12, 24, 36 and 52 weeks after insertion. Patients with symptomatic CRT were treated with anticoagulants and were followed for an additional 52 weeks from the date of CRT diagnosis. Baseline information and follow-up data regarding catheter patency, thromboprophylaxis, clinical symptoms, and thrombotic events was collected. Standardized regional guidelines for catheter care were followed and symptomatic CRT was diagnosed based on objective testing and satisfaction of prespecified criteria. Between March 2002 and July 2003, 444 patients underwent 500 catheter insertions. The mean patient age was 56 y (range 18–91 y) and 55% of patients were female. Catheters inserted included PICCs (65%), ports (18%), pheresis (11%), and Hickman catheters (6%). As of July 22, 2004, 442 patients had completed follow-up. The total catheter-dwell time was 59,959 d (median 88 d), while the total follow-up was 73,654 pt-d (median 151 d). Colorectal was the most common tumor type in 18% of patients and 41% of all patients at enrolment had metastatic solid tumor. Overall, there were 19 episodes of symptomatic CRT, representing an incidence of 4.3% (95% CI 2.6–6.6%) of patients or 0.3 CRTs per 1000 catheter-dwell days (95% CI 0.2–0.5 per 1000 d). The mean time to CRT was 53 d (range 6–162 d). Development of CRT was not associated with age, ECOG performance status, cancer treatment, catheter type, side of insertion, thromboprophylaxis, infection, or previous history of thrombosis. The only significant risk factor was ovarian cancer (P=0.02). In patients with symptomatic CRT, 89% (17/19) of CRTs were treated with anticoagulant therapy alone, 5.3% (1/19) had the catheter removed, and 5.3% (1/19) were treated with both; none had symptomatic pulmonary embolism or post-thrombotic syndrome during follow-up. In summary, the incidence of symptomatic CRT in adults with cancer is low and treatment with anticoagulant therapy alone was not associated with any serious long-term sequelae. Due to the small number of CRTs observed, larger studies are required to further evaluate risk factors and identify the optimal therapeutic approach for CRTs.


2013 ◽  
Vol 118 (1) ◽  
pp. 58-62 ◽  
Author(s):  
William J. Kemp ◽  
Daniel H. Fulkerson ◽  
Troy D. Payner ◽  
Thomas J. Leipzig ◽  
Terry G. Horner ◽  
...  

Object A small percentage of patients will develop a completely new or de novo aneurysm after discovery of an initial aneurysm. The natural history of these lesions is unknown. The authors undertook this statistical evaluation a large cohort of patients with both ruptured and unruptured de novo aneurysms with the aim of analyzing risk factors for rupture and estimating a risk of subarachnoid hemorrhage (SAH). Methods A review of a prospectively maintained database of all aneurysm patients treated by the vascular neurosurgery service of Goodman Campbell Brain and Spine from 1976–2010 was performed. Of the 4718 patients, 611 (13%) had long-term follow-up imaging. The authors identified 27 patients (4.4%) with a total of 32 unruptured de novo aneurysms from routine surveillance imaging. They identified another 10 patients who presented with a new SAH from a de novo aneurysm after treatment of their original aneurysm. The total study group was thus 37 patients with a total of 42 de novo aneurysms. The authors then compared the 27 patients with incidentally discovered aneurysms with the 10 patients with SAH. A statistical analysis was performed, comparing the 2 groups with respect to patient and aneurysm characteristics and risk factors. Results Thirty-seven patients were identified as having true de novo aneurysms. This group had a female predominance and a high percentage of smokers. These 37 patients had a total of 42 de novo aneurysms. Ten of these 42 aneurysms hemorrhaged. De novo aneurysms in both the SAH and non-SAH group were anatomically small (< 10 mm). The estimated risk of hemorrhage over 5 years was 14.5%, higher than the expected SAH risk of small, unruptured aneurysms reported in the ISUIA (International Study of Unruptured Intracranial Aneurysms) trial. There was no statistically significant correlation between hemorrhage and any of the following risk factors: hypertension, diabetes, tobacco and alcohol use, polycystic kidney disease, or previous SAH. There was a statistically significant between-groups difference with respect to patient age, with the mean patient age being significantly older in the SAH aneurysm group than in the non-SAH group (p = 0.047). This is likely reflective of longer follow-up and discovery time, as the mean length of time between initial treatment and discovery of the de novo aneurysm was longer in the SAH group (p = 0.011). Conclusions While rare, de novo aneurysms may have a risk for SAH that is comparatively higher than the risk associated with similarly sized, small, initially discovered unruptured saccular aneurysms. The authors therefore recommend long-term follow-up for all patients with aneurysms, and they consider a more aggressive treatment strategy for de novo aneurysms than for incidentally discovered initial aneurysms.


2021 ◽  
pp. 088307382110531
Author(s):  
Cemal Karakas ◽  
Emin Fidan ◽  
Kapil Arya ◽  
Troy Webber ◽  
Joan B. Cracco

To determine the frequency, predictors, and outcomes of seizures in patients with myelomeningocele, we retrospectively analyzed the data from patients with myelomeningocele followed longitudinally at a single center from 1975 to 2013. We identified a total of 122 patients (61% female). The mean follow-up duration was 11.1 years (minimum-maximum = 0-34.5 years, SD = 8.8, median = 9.1 years). A total of 108 (88.5%) patients had hydrocephalus, and 98 (90.7%) of those patients required a ventriculoperitoneal shunt procedure. Twenty-four (19.7%) patients manifested with seizures, 23 of whom had hydrocephalus. The average age of seizure onset was 4.8 years (median 2 years of age). Falx dysgenesis ( P = .004), lumbar myelomeningocele ( P = .007), and cortical atrophy ( P = .028) were significantly associated with epileptic seizure development. The average seizure-free period at the last follow-up in patients with a history of myelomeningocele and seizures was 8.1 years. We conclude that myelomeningocele patients with seizures have an overall good prognosis with considerable long-term seizure freedom.


2019 ◽  
Vol 104 (2) ◽  
pp. 276-281 ◽  
Author(s):  
Sang Yeop Lee ◽  
Eun Woo Kim ◽  
Wungrak Choi ◽  
Chan Keum Park ◽  
Sangah Kim ◽  
...  

AimsIn this study, we tested the hypothesis that intraocular pressure (IOP) parameters measured by dynamic contour tonometry (DCT) would be more relevant in progression of glaucoma when there is a history of laser refractive surgery (LRS) than the IOP parameters measured by Goldmann applanation tonometry (GAT) or calculated by correction formulae.MethodsNinety-eight eyes in 54 patients with open-angle glaucoma and a history of LRS were included in this retrospective study. IOP was measured by both GAT and DCT during follow-up. Baseline, mean, and peak IOP, IOP fluctuation, and IOP reduction were measured by each tonometry method. Corrected IOP parameters using central corneal thickness and mean keratometry values were also analysed. Clustered logistic regression was used to identify variables correlated with progression of glaucoma. Areas under the curve (AUCs) for correlated variables were also compared.ResultsThe mean DCT value (OR 1.36, p=0.024), peak DCT value (OR 1.19, p=0.02) and pattern SD (OR 1.10, p=0.016) were significant risk factors for progression. There was a significant difference in the predictive ability of the mean DCT and GAT values (AUC 0.63 and 0.514, respectively; p=0.01) and of the peak DCT and GAT values (0.646 and 0.503, respectively, p=0.009). The AUCs for corrected IOP did not exceed those of DCT.ConclusionsIOP measurements were more associated with progression of glaucoma when measurements were obtained by DCT than by GAT or correction formulae in eyes with a history of LRS.


2020 ◽  
Vol 28 (1) ◽  
pp. 75-79
Author(s):  
Mark Savage ◽  
Ross Kung ◽  
Cameron Green ◽  
Brandon Thia ◽  
Dinushka Perera ◽  
...  

Objective: To describe the characteristics of patients presenting to an Emergency Department (ED) following overdoses; to identify risk factors for intensive care unit (ICU) admission among these patients; and to identify the rate of mortality and repeat overdose presentations over four years. Methods: Adult patients presenting to ED following drug overdose during 2014 were included. Data were collected from medical notes and hospital databases. Results: During the study period, 654 patients presented to ED 800 times following overdose. Seventy-eight (9.8%) resulted in ICU admission, and 59 (7.4%) required intubation; 57.2% had no history of overdose presentations, and 72.9% involved patients with known psychiatric illness. Overdose of atypical antipsychotics (AAP), age and history of prior overdose independently predicted ICU admission. A third of patients ( n = 196, 30%) had subsequent presentations to ED following overdose, in the four years from their index presentation, with an all-cause four-year mortality of 3.4% ( n = 22). Conclusion: A history of overdose, use of AAP and older age were risk factors for ICU admission following ED presentations. Over a third of patients had repeat overdose presentation in the four-year follow-up with a mortality of 3.4%.


Lupus ◽  
2019 ◽  
Vol 28 (4) ◽  
pp. 555-559 ◽  
Author(s):  
D Martín-Iglesias ◽  
J Artaraz ◽  
A Fonollosa ◽  
A Ugarte ◽  
A Arteagabeitia ◽  
...  

Objective The objective of this report is to analyse retinal changes over a five-year period, assessed by spectral domain-optical coherence tomography (SD-OCT), in patients from the Lupus-Cruces cohort treated with hydroxychloroquine (HCQ). Methods SD-OCT screening was performed annually between 2012 and 2017. Average macular thickness (AMT), ganglion cell layer thickness (GCLT) and qualitative data of retinal pigment epithelium (RPE) and external retina (ExtR) were collected prospectively. We compared data from 2012 (first) and 2017 (second) SD-OCT. Results We studied 110 patients and 195 eyes. No cases of HCQ toxicity were detected. At the time of the second SD-OCT, 99% patients had taken a daily dose of HCQ ≤5 mg/kg/day. The median time on HCQ was 133 months. The mean AMT and GCLT were significantly lower in both eyes at the second SD-OCT; however, all the differences were clinically insignificant at less than 1%. Qualitative analysis of RPE and ExtR showed no significant changes. Similar results were found among patients with risk factors for retinopathy. The comparison of patients with and without risk factors showed no differences. Conclusions This study shows clinically irrelevant retinal changes in an SLE cohort on HCQ treatment over a five-year follow-up. Our findings support the safety of long-term HCQ at doses ≤5 mg/kg/day.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4937-4937
Author(s):  
Franca Radaelli ◽  
Stefania Bramanti ◽  
Mariangela Colombi ◽  
Alessandra Iurlo ◽  
Alberto Zanella

Abstract Essential thrombocythemia (ET) is a chronic myeloproliferative disorder characterized by peripheral thrombocytosis and abnormal proliferation of megakariocytes in the bone marrow. Even thought thrombosis is frequently associated to ET, the risk factors of this clinical complication are still controversial. The aim of this retrospective, single institution study was to investigate clinical and laboratory characteristics associated with the occurrence of thrombotic events, with the purpose of identifying subgroups of patients who could benefit from antiaggregant and/or cytostatic treatment. 306 consecutive ET patients (109 men and 197 females, median age 58 yr) diagnosed between January 1979 and December 2002 were included in the study. At the time of analysis, 196 patients were still alive with a median follow up of 96 months. The following variables were investigated for the association with thrombotic complications: age, platelet count, previous history of thrombotic events, time from diagnosis, treatment with antiaggregant/cytostatic drugs, and cardiovascular risk factors such as arterial hypertension, obesity, hypercolesterolemia, diabetes, cigarette smoking. At the time of last follow up, 46 patients (15%) experienced at least one thrombotic event. The occurrence of thrombotic events was observed in 26/64 (40.6%) patients with previous history of thrombosis and in 20/242 (8.3%) patients with no previous history of thrombosis (p&lt;0.0001 Fisher’s exact test, odd ratio 7.6). A significant difference between the two groups of patients was also confirmed when Kaplan Meier estimates of thrombosis-free survival were compared by log-rank test (p&lt;0.0001). By logistic regression, platelet number at diagnosis did not associate with occurrence of thrombosis in the whole patient population. When patients without previous history of thrombosis were stratified according to the number of cardiovascular risk factors (none vs one vs more than one), a significant correlation with occurrence of thrombotic events was observed (Mantel-Haenszel Chi-square 5.47, p&lt;0.05). This study confirms that history of thrombosis is strongly related with risk of further thrombotic events in patients with ET, whereas platelet number at diagnosis does not seem to represent a prognostic factor. In patients with no previous history of thrombosis, the presence of other cardiovascular risk factors has to be taken into account when establishing the therapeutic approach.


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