An evaluation of the SAFIRE grading scale as a predictor of long-term outcomes for patients in the Barrow Ruptured Aneurysm Trial

2021 ◽  
pp. 1-5
Author(s):  
Joshua S. Catapano ◽  
Mohamed A. Labib ◽  
Fabio A. Frisoli ◽  
Megan S. Cadigan ◽  
Jacob F. Baranoski ◽  
...  
Keyword(s):  
Univariate Analysis ◽  
Ruptured Aneurysm ◽  
Poor Outcome ◽  
Increased Risk ◽  
Grade Ii ◽  
Grading Scale ◽  
Poor Outcomes ◽  
Grade Iii

OBJECTIVEThe SAFIRE grading scale is a novel, computable scale that predicts the outcome of aneurysmal subarachnoid hemorrhage (aSAH) patients in acute follow-up. However, this scale also may have prognostic significance in long-term follow-up and help guide further management.METHODSThe records of all patients enrolled in the Barrow Ruptured Aneurysm Trial (BRAT) were retrospectively reviewed, and the patients were assigned SAFIRE grades. Outcomes at 1 year and 6 years post-aSAH were analyzed for each SAFIRE grade level, with a poor outcome defined as a modified Rankin Scale score > 2. Univariate analysis was performed for patients with a high SAFIRE grade (IV or V) for odds of poor outcome at the 1- and 6-year follow-ups.RESULTSA total of 405 patients with confirmed aSAH enrolled in the BRAT were analyzed; 357 patients had 1-year follow-up, and 333 patients had 6-year follow-up data available. Generally, as the SAFIRE grade increased, so did the proportion of patients with poor outcomes. At the 1-year follow-up, 18% (17/93) of grade I patients, 22% (20/92) of grade II patients, 32% (26/80) of grade III patients, 43% (38/88) of grade IV patients, and 75% (3/4) of grade V patients were found to have poor outcomes. At the 6-year follow-up, 29% (23/79) of grade I patients, 24% (21/89) of grade II patients, 38% (29/77) of grade III patients, 60% (50/84) of grade IV patients, and 100% (4/4) of grade V patients were found to have poor outcomes. Univariate analysis showed that a SAFIRE grade of IV or V was associated with a significantly increased risk of a poor outcome at both the 1-year (OR 2.5, 95% CI 1.5–4.2; p < 0.001) and 6-year (OR 3.7, 95% CI 2.2–6.2; p < 0.001) follow-ups.CONCLUSIONSHigh SAFIRE grades are associated with an increased risk of a poor recovery at late follow-up.

scholarly journals Long-term outcomes of pediatric arteriovenous malformations: the 30-year Pittsburgh experience

2020 ◽  
Vol 26 (3) ◽  
pp. 275-282
Author(s):  
Michael M. McDowell ◽  
Nitin Agarwal ◽  
Gordon Mao ◽  
Stephen Johnson ◽  
Hideyuki Kano ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Arteriovenous Malformations ◽  
Multimodality Treatment ◽  
Treatment Choice ◽  
University Of Pittsburgh ◽  
Poor Outcome ◽  
Poor Outcomes ◽  
The University

OBJECTIVEThe study of pediatric arteriovenous malformations (pAVMs) is complicated by the rarity of the entity. Treatment choice has often been affected by the availability of different modalities and the experience of the providers present. The University of Pittsburgh experience of multimodality treatment of pAVMs is presented.METHODSThe authors conducted a retrospective cohort study examining 212 patients with pAVM presenting to the University of Pittsburgh between 1988 and 2018, during which patients had access to surgical, endovascular, and radiosurgical options. Univariate analysis was performed comparing good and poor outcomes. A poor outcome was defined as a modified Rankin Scale (mRS) score of ≥ 3. Multivariate analysis via logistic regression was performed on appropriate variables with a p value of ≤ 0.2. Seventy-five percent of the cohort had at least 3 years of follow-up.RESULTSFive patients (2.4%) did not receive any intervention, 131 (61.8%) had GKRS alone, 14 (6.6%) had craniotomies alone, and 2 (0.9%) had embolization alone. Twenty-two (10.4%) had embolization and Gamma Knife radiosurgery (GKRS); 20 (9.4%) had craniotomies and GKRS; 8 (3.8%) had embolization and craniotomies; and 10 (4.7%) had embolization, craniotomies, and GKRS. Thirty-one patients (14.6%) were found to have poor outcome on follow-up. The multivariate analysis performed in patients with poor outcomes was notable for associations with no treatment (OR 18.9, p = 0.02), hemorrhage requiring craniotomy for decompression alone (OR 6, p = 0.03), preoperative mRS score (OR 2.1, p = 0.004), and Spetzler-Martin score (OR 1.8, p = 0.0005). The mean follow-up was 79.7 ± 62.1 months. The confirmed radiographic obliteration rate was 79.4% and there were 5 recurrences found on average 9.5 years after treatment.CONCLUSIONSHigh rates of long-term functional independence (mRS score of ≤ 2) can be achieved with comprehensive multimodality treatment of pAVMs. At this center there was no difference in outcome based on treatment choice when accounting for factors such as Spetzler-Martin grade and presenting morbidity. Recurrences are rare but frequently occur years after treatment, emphasizing the need for long-term screening after obliteration.

Outcomes after Double Umbilical Cord Blood Transplantation in Children: A Survey on Behalf of Eurocord and PDWP-EBMT

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 2570-2570
Author(s):  
Federica Giannotti ◽  
Annalisa Ruggeri ◽  
Gerard Michel ◽  
Jean-Hugues Dalle ◽  
Tracey O'Brien ◽  
...  
Keyword(s):  
Single Unit ◽  
Acute Gvhd ◽  
Chronic Gvhd ◽  
Univariate Analysis ◽  
Median Number ◽  
Cell Dose ◽  
Gvhd Prophylaxis ◽  
Grade Ii ◽  
Grade Iii

Abstract Double UCBT (dUCBT) has been used in adults to reach an acceptable cell dose. For most children a single unit with a total nucleated cell (TNC) dose >3x107/Kg can be easily identified, but that is not always the case for heavier patients (pts). Use of dUCBT might decrease relapse and increase graft-versus-host-disease (GvHD). Data on dUCBT in children are scarce in the literature. A recent randomized study in children has described similar outcomes after double compared to single UCBT. Our study provides an overview of the use of dUCBT in the pediatric population reported to Eurocord. We retrospectively analyzed the outcomes of unrelated dUCBT in 177 children transplanted between 2002 and 2012 in 61 EBMT centres. Analysis was performed separately for pts with malignant (n=139) and non-malignant (NM, n=38) diseases. Among pts with malignancies, 76 had ALL, 40 AML, 6 MDS, 2 CML, 11 NHL, 3 Hodgkin Lymphoma and 1 Multiple Myeloma. Median age at dUCBT was 15 years (1.3-17.9) and median weight was 55 kg (13-97). Disease status at dUCBT was 1st complete remission (CR) (36%), ≥2nd CR (34%) or advanced (25%), and missing in 5% of the pts. In this group, 117pts received a myeloablative conditioning (MAC) and 22 a reduced intensity regimen (RIC). Cyclophosphamide+fludarabine+TBI was administered to 41% of the pts; 55% received ATG in the conditioning. Median number of collected TNC was 5.7x107/kg (3,6-12,8). Considering the unit with the higher number of HLA incompatibilities with the recipient, 56% had 2 mismatches. GvHD prophylaxis was cyclosporine-A (CSA) based in 93% of the pts (58% received CSA + mycofenolate mofetil). Median follow-up was 31 months. Cumulative incidence (CI) of neutrophil (PMN) and platelet (PLT) engraftment was 88% at 60 days and 64% at 180 days after dUCBT, and it was achieved with a median time of 24 and 45 days, respectively. Among the 122 pts with PMN engraftment, 85/94 with available data on chimerism were full donor and, of these, 20% had dual chimerism. CI of acute GvHD grade II-IV and grade III-IV at 100 days was 51% and 26%, respectively; it was significantly higher in pts who did not receive ATG (grade II-IV: 35% vs 67%, p=0.004; grade III-IV: 12% vs 37%, p=0.0075). Chronic GvHD was observed in 24/104 pts at risk (60% extensive; 2-year (yr) CI: 18%). The 2-yr CI of relapse was 31%. In univariate analysis, RIC, advanced stage at transplantation and a collected TNC dose lower than the median, were significantly associated with higher rates of relapse.The 2-yr CI of transplant related mortality (TRM) was 27%. Overall, 73 pts died: 35 of relapse, 15 of infections, 9 of GvHD and 14 of other causes. The 2-yr disease free survival (DFS) and overall survival (OS) were 42% and 45%, respectively. Among pts with NM disorders, 24 had bone marrow failure syndrome (BMFS) (10 Fanconi Anemia, 13 Acquired Aplastic Anemia and 1 other inherited BMFS), 2 hemoglobinopathies, 7 immune deficiencies and 5 metabolic disorders. Median age at dUCBT was 11 years (0.7-17.9) and the median weight was 40 kg (13-70). In this group, 27 pts received a RIC (40% TBI based), 10 a MAC (90% busulfan based), and 1 no conditioning regimen. ATG was administered to 82% of the pts and GvHD prophylaxis was CSA-based in 77%. The median number of collected TNC was 8.4x107/kg (1,2-11,2) and 60% of the grafts had ≥2 HLA mismatches with the recipient. Median follow-up was 39 months. Overall, 28 pts achieved PMN engraftment and 16 PLT engraftment, with a median time of 23 and 61 days, respectively. In univariate analysis, pts with BMFS compared to others had a significantly lower CI of PMN engraftment (58% vs 100%, p=0.002). Among the 10 pts who did not engraft, 3 had autologous reconstitution and 3 had a subsequent allogeneic HSCT. Forteen pts developed acute GvHD grade II-IV and 10/25 pts at risk had chronic GvHD (3 extensive). Overall 21 pts died (17 with BMFS): 9 of infections, 5 of GvHD and 7 of other causes. The 2-yr OS was 42% and it was significantly lower in pts with BMFS compared to those affected by other NM disorders (28% vs 70%, p=0.03). In pts with malignancies, despite a higher incidence of acute GvHD, DFS and OS seem to be comparable to those reported in the literature for single UCBT or HSCT from other alternative stem cell sources. In the NM disorders group, despite the high cell dose, dUCBT did not seem to improve results in pts with BMFS. This survey suggests that dUCBT is feasible in children and should be considered when a single unit with an adequate cell dose is not available. Disclosures No relevant conflicts of interest to declare.

Surgery for cerebral arteriovenous malformation: risks related to lenticulostriate arterial supply

1997 ◽  
Vol 86 (5) ◽  
pp. 801-805 ◽  
Author(s):  
Michael K. Morgan ◽  
Katharine J. Drummond ◽  
Verity Grinnell ◽  
William Sorby
Keyword(s):  
Cerebral Arteriovenous Malformation ◽  
Arterial Supply ◽  
Content Type ◽  
Increased Risk ◽  
Complications Of Surgery ◽  
Grade Ii ◽  
Martin Grade ◽  
Grade Iii ◽  
Fine Print

✓ The aim of this study was to compare complications of surgery in arteriovenous malformations (AVMs) supplied by the middle cerebral artery (MCA) with and without a lenticulostriate arterial contribution. Ninety-two consecutive surgical resections of AVMs with an angiographically demonstrated MCA supply were performed between January 1989 and July 1996. Ten of these cases had a significant lenticulostriate arterial contribution. The cases were graded according to the Spetzler—Martin classification. There were no deaths and 4.3% of cases developed new major neurological deficit by the 3-month follow-up examination. All cases had angiographically confirmed obliteration of the AVM. There were no complications in 16 patients with Spetzler—Martin Grade I AVMs, one case of complications in 40 patients with Grade II AVMs, eight cases of complications in 26 patients with Grade III AVMs, and seven cases of complications in 10 patients with Grade IV and V AVMs. The supply of blood from lenticulostriate branches was associated with complications in eight of the 10 cases. The effect of the presence of a lenticulostriate arterial supply was most apparent in cases of Grade III AVMs: complications were experienced in three of 20 patients whose AVMs were not supplied by the arteries and in five of six patients whose AVMs were fed by the lenticulostriate arteries. This difference is significant (p < 0.0001). The conclusions drawn from this study are that for Grade III AVMs, the presence of a lenticulostriate arterial supply can be considered a factor predictive of an increased risk of surgical complications.

Multivariate Analysis of Risk Factors for Clinically Overt Hemorrhagic Cystitis after Allogeneic Hematopoietic Stem Cell Transplantation.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5295-5295
Author(s):  
Kanger Zhu ◽  
Chunhui Ma ◽  
Tao Zhang ◽  
Juan Zhong
Keyword(s):  
Risk Factors ◽  
Multivariate Analysis ◽  
Cox Regression ◽  
Hemorrhagic Cystitis ◽  
Univariate Analysis ◽  
Male Gender ◽  
Gvhd Prophylaxis ◽  
Increased Risk ◽  
Grade Ii ◽  
Grade Iii

Abstract Objective: To analyze the risk factors of clinically overt hemorrhagic cystitis (HC) (grade ≥II) in 114 patients undergoing allo-HSCT to predict the occurrence of HC. Methods: We retrospectively analyzed 29 cases of clinically overt HC from a series of 114 patients given allo-HSCT from April 1997 to December 2004. The time of follow-up began from the day of initiating conditioning to day 180 post-transplant. The 11 clinical parameters were selected for univariate analysis using a Cox regression: age, sex, underlying disease, conditioning regimen, disease status at transplant, aGVHD, donor type, use of ATG, GVHD prophylaxis, platelet and neutrophil engraftment. Factors that were significant at the 0.1 level on univariate analysis were evaluated by multivariate analysis using a Cox regression. The cumulative incidence of grade ≥ II HC within the day 180 after transplantation was calculated by the method of Kaplan and Meier. Results: 29 out of 114 patients (26%) developed HC with grade II in 12/29 cases (41.4%), grade III in 11/29 cases (37.9%) and grade IV in 6/29 cases (20.7%). The following factors were associated with an increased risk of HC by univariate analysis: male gender (RR=2.885, P=0.021),younger than 26 years (RR=3.265, P=0.007),grade III~IV aGVHD (RR=4.039, P=0.002),unrelated doner (RR=4.347, P=0),intense GVHD prophylaxis (RR=2.218, P=0.045),advanced disease (RR=2.668, P=0.009). These risk factors were entered into a multivariate model. Only male gender (RR=2.993, 95% CI 1.218–7.358; P=0.017) and unrelated donor (RR=4.478, 95% CI 2.049–9.786; P=0.000) were identified as being significantly associated with the occurrence of hemorrhagic cystitis. Conclusion: We found that in multivariate analysis, patients were at increased risk of HC if they were male or had received graft from unrelated donors.

HLA-DP Mismatches Increase the Risk of Acute GVHD after Unrelated Donor Hematopoietic Transplantation (UDT).

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3125-3125 ◽  
Author(s):  
Marcos de Lima ◽  
Simrit Parmar ◽  
Ping Liu ◽  
Poliana A. Patah ◽  
Pedro Cano ◽  
...  
Keyword(s):  
Acute Gvhd ◽  
Chronic Gvhd ◽  
Univariate Analysis ◽  
Unrelated Donor ◽  
Free Survival ◽  
Gvhd Prophylaxis ◽  
Grade Ii ◽  
Reduced Intensity ◽  
Grade Iii

Abstract The HLA class II DP locus encode for both subunits of DPB1 heterodimers, which have low levels of expression on the cell surface of antigen presenting cells. We hypothesized that donor-recipient HLA-DP mismatch would lead to an increased incidence of acute (a) graft-versus-host disease (GVHD), and that 2 mismatches would likely be even more significant. Methods: We studied 84 consecutive patients (pts) with myeloid leukemias in complete remission (CR) transplanted from 01/02 to 02/06. Preparative regimens were ablative IV Busulfan-based (n=58) or Cy/TBI (n=2), and reduced intensity (Fludarabine (Flu)/Bu 130 mg/m2/2 doses plus Gleevec (n=8), and Flu/Melphalan 140 mg/m2 (n=16). Stem cell (SC) source was bone marrow (n=70) or peripheral blood (n=14). ATG was given in 78 cases. GVHD prophylaxis was tacrolimus and mini-methotrexate in all cases, with additional pentostatin in 31 pts. High-resolution typing was sequence-based for HLA-A, B, DRB1; SSP was used for DRB3/4/5, DQB1 and DPB1, and SBT/SSOP for HLA-C. A Cox proportional hazards regression model was used to study aGVHD-free and relapse-free (RFS) survival. Variables with a p-value <0.25 by univariate analysis were included in the multiple regression analysis (MV). Variables were age, gender, weight, conditioning regimen, GVHD prophylaxis, diagnosis, cytogenetics, SC source, ABO group, infused CD34 and CD3 cell dose, and HLA matching. AGVHD-free survival was calculated from transplant date to date of development of grade II–IV GVHD or completion of 100 days of follow-up. Results: Median age was 48 yrs (range, 14–72). Diagnoses were MDS (n=5), AML (n=58), and CML (n=21). 54 pts (64%) were beyond 1st CR; all CML pts were in >1st chronic phase (CP). Sixty-one pts were 10/10 HLA match (A, B, C, DRB1, DQB1), and 23 had one or more mismatches. All but one pt engrafted neutrophils at a median of 13 days. 33 pts (39%) and 13 pts (15%) developed grade II–IV and III–IV aGVHD, respectively. Chronic GVHD incidence was 51%. With a median follow-up of 18 mo. (range,1.3–52) 60 pts are alive; 40 pts have relapsed or died. Median survival has not been reached. Number of DP mismatches and incidence of aGVHD is shown in the table. The following covariates influenced aGVHD-free survival by MV analysis: Flu-based regimen (P=0.005; HR 0.25 (95%CI 0.1–0.66), reduced intensity regimens (p=0.02; HR 0.35 (95%CI 0.15–0.83) and presence of 2 DPB1 mismatches (p=0.02; HR 3.07 (95%CI 1.19–7.95). Presence of 1 DPB1 mismatch was not significantly associated with aGVHD. There was no statistically significant correlation between presence of 2 DP mismatches and RFS (P=0.17;HR 0.3 (95%CI 0.06–1.65);HR 0.75 for 1 mismatch) or with cGVHD. Actuarial 2-yr survival for 10/10 matched pts without DP mismatches (12/12) versus those with DP mismatches is 82% versus 71%(P=0.6). In the 10/10 matched group, GVHD was the cause of death only among recipients of 2 DP mismatches transplants (n=4). Conclusion: Mismatching at HLA-DPB1 may increase the risk of aGVHD following UDT. The role of DP in the development of GVHD and GVL effects merits future study. Incidence of acute GVHD 10 of 10 matches number of DP mismatches grade II–IV grade III–IV 0 8% 0% 1 23% 8% 2 45% 18% < 10 of 10 matches number of DP mismatches grade II–IV grade III–IV 0 45% 15% 1 82% 36% 2 80% 40%

scholarly journals Clofarabine (Clo) Plus Busulfan (Bu) Is an Effective Conditioning Regimen for Allogeneic Hematopoietic Cell Transplantation (HCT) in Patients (pts) with Acute Lymphoblastic Leukemia (ALL): Long-Term Study Results

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4376-4376
Author(s):  
Partow Kebriaei ◽  
Roland L. Basset ◽  
Celina Ledesma ◽  
Gabriela Rondon ◽  
Betul Oran ◽  
...  
Keyword(s):  
Overall Survival ◽  
High Risk ◽  
Advanced Disease ◽  
Survival Rates ◽  
Unrelated Donor ◽  
Grade Ii ◽  
Disease Free ◽  
Grade Iii

Abstract Allogeneic HCT improves long-term disease control in pts with ALL, but the treatment-related mortality (TRM) associated with most myeloablative transplant conditioning regimens limits the benefits of HCT. Therefore, we investigated a novel regimen consisting of Clo combined with intravenous (i.v.) Bu in adult pts with ALL undergoing allogeneic HCT. Preliminary results were encouraging1, and we now report on long-term outcomes. Methods: Clo 40 mg/m2 was infused over 60 min, each dose followed by Bu 130 mg/m2 infused over 3 hours daily for 4 days followed by hematopoietic cell infusion 3 days later. Bu was infused either as a fixed dose per BSA, or to target an average daily AUC of 5,500 microMol-min for pts up to 60 years of age or 4000 microMol-min for pts greater than 60 years, determined by a test dose of Bu at 32 mg/m2 given 48 hours prior to the high dose regimen. Dilantin was administered for seizure prophylaxis. GVHD prophylaxis was based on tacrolimus andmini-MTX, with the addition of rabbit anti-thymocyte globulin (4 mg/kg total dose) for unrelated donor transplants. Results: 107 pts (91 B-lineage, 16 T-lineage) with median age 38 years (range 19-64 years) received an allogeneic matched sibling (n=52) or matched unrelated donor (n=55) HCT in CR1 (n=62), CR2 (n=28), or more advanced disease (CR3, n=2; incomplete recovery of counts, n=9; blasts >5%, n=6) .Complete remission was defined by <5% blasts in bone marrow and normal CBC. High-risk cytogenetic profile defined by the presence of t(9;22), t(4;11), or complex cytogenetics was noted in 41% of patients (n=45). The median time from diagnosis to HCT was 9.2 months (range 2.3-118.2 months). The most common grades II-III toxicities were mucositis (93%:70 grade II, 28 grade III) and reversible liver enzyme elevation (84%: 46 grade II, 44 grade III). The incidence of VOD was 6% (5 reversible grade III, 1 grade V); these 6 pts had extensive therapy prior to HCT with median time from diagnosis to HCT of 17.5 months. 104 pts are evaluable for response (early death within 30 days, n=2; recent HCT with less than 30 days follow-up, n=1). Median days to ANC > 0.5 x 109/L and platelet count > 20 x 109/L were 11 (range 10-25 days) and 13 (8-109 days; 8 pts without recovery), respectively. All pts with measurable disease prior to HCT achieved CR by day +30 after HCT. Full donor chimerism by day 30 was achieved in 70% pts; 84% of pts eventually achieved full donor chimerism defined as 100% donor T-cells and myeloid cells. The incidence of grades II-IV and III-IV acute GVHD were 35% and 10%, respectively; 18% pts developed extensive chronic GVHD. With a median follow-up of 2.5 years among surviving patients (0.1-4.9 years), the 2-year overall survival rates for pts transplanted in CR1, CR2, or more advanced disease were 68%, 58%, and 34%, respectively, as illustrated in figure below; 2-year disease-free survival rates were 60%, 40%, and 35%, respectively. Non-relapse mortality (NRM) rates at 100 days and 2 years were 6% and 18%, respectively. Among 9 pts older than 60 years treated with reduced dose Bu in CR1 (n=5), CR2 (n=3) or more advanced disease (n=1), 5 remain alive and disease-free. Conclusion: The CloBu combination is well-tolerated in this cohort of adult pts with high-risk ALL who received a median of 9.2 months of intensive (mainly HCVAD-based) chemotherapy prior to receiving transplant. Overall survival and NRM compare favorably with traditional TBI-based regimens. 1. Kebriaei et al. Biol Blood Marrow Transplant. 2012 Dec; 18(12): 1819-1826. Figure 1. Figure 1. Disclosures Alousi: Therakos, Inc: Research Funding. Andersson:Otsuka Research and Development, Inc.: Consultancy.

Erythrocytosis Post Allogeneic Stem Cell Transplantation, An Observational Study Of Hemoglobin Levels On Long-Term Follow Up Post Transplantation In Patients With Fanconi Or Acquired Aplastic Anemia

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4643-4643 ◽  
Author(s):  
Mouhab Ayas ◽  
Khawar Siddiqui ◽  
Abdulrahman Al-Musa ◽  
Hassan El-Solh ◽  
Abdullah Al-Jefri ◽  
...  
Keyword(s):  
Aplastic Anemia ◽  
Cell Transplantation ◽  
Median Time ◽  
Univariate Analysis ◽  
Thromboembolic Events ◽  
Allogeneic Hct ◽  
Increased Risk ◽  
Long Term Follow Up

Post-transplant erythrocytosis is an ominous complication of kidney transplantation, occurring in the first 8 to 24 months after surgery in 10% to 15% of transplant recipients; this is frequently associated with significant thromboembolic events and sometimes death. In patients undergoing allogeneic hematopoietic cell transplantation (HCT), erythrocytosis has not been previously well described. At our institution, we observed that some aplastic anemia (AA), and Fanconi anemia (FA) patients developed progressively increased hemoglobin (HB), hematocrit (HCT) and RBC readings on long term follow up. Thus, this study was conducted to assess the validity of this observation in AA/FA patients post HCT, and its impact on their health. Patients and Methods From January 1993 until December 2011, 144 pediatric patients underwent successful allogeneic HCT for AA or FA; median age at HCT 11.6 years (range, 6.6 -15). All patients included were alive at the time of the analysis, and had sustained engraftment; all have had a follow up time of ≥ 12 months. For those who underwent more than one HCT, only events after the last HCT were included. We retrospectively examined the HB levels as an indicator for erythrocytosis (Corresponding RBC, HCT, WBC, and platelet counts were also collected). HB values of 150, and 160 gm/l were considered the trigger value in females and males, respectively. Patients who reached this value were studied for higher values on follow up, and only those whose HB persisted for at least 3 months above trigger value were included in the analysis; 29 patients (15 females, 14 males) were identified after causes of secondary erythrocytosis were ruled out. Erythrocytosis was defined as HB ≥ 160 gm/l in females, a HB ≥ 170 gm/l in males. Results Median time to trigger HB was 51.4 months (range, 15-121) in females, and 65 months (range, 23.3-114) in males, and median age at trigger HB was 14.7 years (range, 8.6-21.4) in females, and 16.9 years (range, 13.4-20.6) in males. Median highest HB reached was 160 gm/l (range, 151-162) in females, and 172 gm/l (range, 164-189) in males, with a median time of 67 months (range, 17-164) in females, and 103 months (range, 23.3-206) in males; the median age at highest HB was 16 years (range, 9.7-24.8) in females, and 20.2 years (range, 13.4-27.4) in males. Upon follow up, the HB fell below the trigger level in 16 patients (9 females, 7 males) (55.2%), at a median time of 37.2 months from the trigger value (range, 3.6-104). Seventeen patients qualified for the diagnosis of erythrocytosis (12%); 8 females, and 9 males. In all 8 females and in 4 males, HB fell below the erythrocytosis value upon follow up. All HB values correlated positively with HCT and RBC, no correlation was detected with platelet count or WBC. On univariate analysis, patients with older age at HCT (≥ 10 years) appeared to be more likely to develop elevated HB (P=0.003); and those who had radiation in the conditioning regimen were less likely to develop elevated HB (P=0.008). Three of the males with persistent erythrocytosis were tested further and all 3 had normal erythropoietin levels and were negative for JAK-2 mutations. None of the 29 patients had any adverse clinical symptoms during the follow up visits, and no thromboembolic events were reported. Conclusion A proportion of patients with AA/FA who undergo HCT may experience elevated HB on long term follow up; 12% subsequently qualifying as erythrocytosis, with the highest reading requiring between 1.5-2 years to evolve. Unlike erythrocytosis post renal transplant, the phenomenon we are describing in our patient cohort does not appear to be associated with any adverse symptoms, or any increased risk of thrombosis. More in depth investigation to study the potential pathophysiology behind it is currently underway at our institution, together with further exploration of this observation in patients with other illnesses undergoing allogeneic HCT. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Predictors of poor outcome despite recanalization: a multiple regression analysis of the NASA registry

2015 ◽  
Vol 8 (3) ◽  
pp. 224-229 ◽  
Author(s):  
Italo Linfante ◽  
Amy K Starosciak ◽  
Gail R Walker ◽  
Guilherme Dabus ◽  
Alicia C Castonguay ◽  
...  
Keyword(s):  
Predictive Power ◽  
Rescue Therapy ◽  
Univariate Analysis ◽  
Multivariable Analysis ◽  
Nihss Score ◽  
Poor Outcome ◽  
The North ◽  
Intravenous Tissue Plasminogen Activator ◽  
Increased Risk ◽  
Poor Outcomes

BackgroundMechanical thrombectomy with stent-retrievers results in higher recanalization rates compared with previous devices. Despite successful recanalization rates (Thrombolysis in Cerebral Infarction (TICI) score ≥2b) of 70–83%, good outcomes by 90-day modified Rankin Scale (mRS) score ≤2 are achieved in only 40–55% of patients. We evaluated predictors of poor outcomes (mRS >2) despite successful recanalization (TICI ≥2b) in the North American Solitaire Stent Retriever Acute Stroke (NASA) registry.MethodsLogistic regression was used to evaluate baseline characteristics and recanalization outcomes for association with 90-day mRS score of 0–2 (good outcome) vs 3–6 (poor outcome). Univariate tests were carried out for all factors. A multivariable model was developed based on backwards selection from the factors with at least marginal significance (p≤0.10) on univariate analysis with the retention criterion set at p≤0.05. The model was refit to minimize the number of cases excluded because of missing covariate values; the c-statistic was a measure of predictive power.ResultsOf 354 patients, 256 (72.3%) were recanalized successfully. Based on 234 recanalized patients evaluated for 90-day mRS score, 116 (49.6%) had poor outcomes. Univariate analysis identified an increased risk of poor outcome for age ≥80 years, occlusion site of internal carotid artery (ICA)/basilar artery, National Institute of Health Stroke Scale (NIHSS) score ≥18, history of diabetes mellitus, TICI 2b, use of rescue therapy, not using a balloon-guided catheter or intravenous tissue plasminogen activator (IV t-PA), and >30 min to recanalization (p≤0.05). In multivariable analysis, age ≥80 years, occlusion site ICA/basilar, initial NIHSS score ≥18, diabetes, absence of IV t-PA, ≥3 passes, and use of rescue therapy were significant independent predictors of poor 90-day outcome in a model with good predictive power (c-index=0.80).ConclusionsAge, occlusion site, high NIHSS, diabetes, no IV t-PA, ≥3 passes, and use of rescue therapy are associated with poor 90-day outcome despite successful recanalization.

Neurosurgical treatment of oligodendroglial tumors in children and adolescents: a single-institution series of 35 consecutive patients

2013 ◽  
Vol 12 (3) ◽  
pp. 241-246 ◽  
Author(s):  
Tryggve Lundar ◽  
Bernt Johan Due-Tønnessen ◽  
Arild Egge ◽  
David Scheie ◽  
Einar Stensvold ◽  
...  
Keyword(s):  
Primary Resection ◽  
Single Institution ◽  
Who Grade ◽  
Fractionated Radiotherapy ◽  
Oligodendroglial Tumors ◽  
Grade Ii ◽  
Who Grade Iii ◽  
Grade Iii

Object The object of this study was to delineate long-term results of the surgical treatment of pediatric CNS tumors classified as oligodendroglioma (OD) or oligoastrocytoma (OA) WHO Grade II or III. Methods A cohort of 45 consecutive patients 19 years or younger who had undergone primary resection of CNS tumors originally described as oligodendroglial during the years 1970–2009 at a single institution were reviewed in this retrospective study of surgical morbidity, mortality, and academic achievement and/or work participation. Gross motor function and activities of daily living were scored using the Barthel Index (BI). Results Patient records for 35 consecutive children and adolescents who had undergone resection for an OA (17 patients) or OD (18 patients) were included in this study. Of the 35 patients, 12 were in the 1st decade of life at the first surgery, whereas 23 were in the 2nd decade. The male/female ratio was 1.19 (19/16). No patient was lost to follow-up. The tumor was localized to the supratentorial compartment in 33 patients, the posterior fossa in 1 patient, and the cervical medulla in 1 patient. Twenty-four tumors were considered to be WHO Grade II, and 11 were classified as WHO Grade III. Among these latter lesions were 2 tumors initially classified as WHO Grade II and later reclassified as WHO Grade III following repeat surgery. Fifty-four tumor resections were performed. Two patients underwent repeat tumor resection within 5 days of the initial procedure, after MRI confirmed residual tumor. Another 10 patients underwent a second resection because of clinical deterioration and progressive disease at time points ranging from 1 month to 10 years after the initial operation. Six patients underwent a third resection, and 1 patient underwent a fourth excision following tumor dissemination to the spinal canal. Sixteen (46%) of the 35 children received adjuvant therapy: 7, fractionated radiotherapy; 4, chemotherapy; and 5, both fractionated radiotherapy and chemotherapy. One patient with primary supratentorial disease experienced clinically malignant development with widespread intraspinal dissemination 9 years after initial treatment. Only 2 patients needed treatment for persistent hydrocephalus. In this series there was no surgical mortality, which was defined as death within 30 days of resection. However, 12 patients in the study, with follow-up times from 1 month to 33 years, died. Twenty-three patients, with follow-up times from 4 to 31 years, remained alive. Among these survivors, the BI was 100 (normal) in 22 patients and 80 in 1 patient. Nineteen patients had full- or part-time work or were in normal school programs. Conclusions Pediatric oligodendroglial tumors are mainly localized to the supratentorial compartment and more often occur in the 2nd decade of life rather than the 1st. Two-thirds of the patients remained alive after follow-ups from 4 to 31 years. Twelve children succumbed to their disease, 9 of them within 3 years of resection despite combined treatment with radio- and chemotherapy. Three of them remained alive from 9 to 33 years after primary resection. Among the 23 survivors, a stable, very long-term result was attainable in at least 20. Five-, 10-, 20-, and 30-year overall survival in patients with Grade II tumors was 92%, 92%, 92%, and 88%, respectively.

Correlation of a preoperative grading scale with progressive spinal deformity following surgery for intramedullary spinal cord tumors in children

2008 ◽  
Vol 2 (4) ◽  
pp. 277-281 ◽  
Author(s):  
Matthew J. McGirt ◽  
Shlomi Constantini ◽  
George I. Jallo
Keyword(s):  
Spinal Cord ◽  
Spinal Deformity ◽  
Spinal Cord Tumors ◽  
Intramedullary Spinal Cord ◽  
Surgical Decision ◽  
Scale Range ◽  
Grade Ii ◽  
Grading Scale ◽  
Grade Iii

Object Postoperative progressive spinal deformity often complicates functional outcome after resection of pediatric intramedullary spinal cord tumors (IMSCTs). The authors propose a preoperative grading scale that correlates with the postoperative development of progressive spinal deformity requiring subsequent fusion. Methods The data obtained in 164 patients who underwent resection of an IMSCT at a single institution were retrospectively collected and analyzed to determine the development of progressive spinal deformity requiring fusion. A grading scale (range of scores I–V) was created based on the presence or absence of 4 preoperative variables: preoperative scoliosis, involvement of the thorocolumbar junction, age < 13 years, and number of surgeries for an IMSCT. The grading scale was then retrospectively applied to this series of 164 children to assess the correlation of variables with subsequent spinal deformity. Results Nine patients presented with Grade I status, 41 patients with Grade II, 58 patients with Grade III, 44 patients with Grade IV, and 12 patients with Grade V. Overall, 44 patients (27%) developed progressive spinal deformity requiring fusion at a mean follow-up of 5 years after surgery. A higher preoperative grade was associated with an increasing need for subsequent fusion for progressive spinal deformity (Grade I [0%], Grade II [5%], Grade III [26%], Grade IV [40%], and Grade V status [75%]). Conclusions Application of this grading scheme to a series of resected pediatric IMSCTs has demonstrated its correlation with the incidence of postoperative progressive spinal deformity requiring fusion. The application of a standardized grading scheme will assist in the process of surgical decision making and postoperative evaluation.

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