Encephaloduroarteriosynangiosis for Pediatric Moyamoya Disease: A Single-Center Experience With 67 Cases in China

2018 ◽  
Vol 33 (14) ◽  
pp. 901-908 ◽  
Author(s):  
Chengjun Wang ◽  
Meng Zhao ◽  
Jia Wang ◽  
Shuo Wang ◽  
Dong Zhang ◽  
...  
Keyword(s):  
Moyamoya Disease ◽  
Pediatric Patients ◽  
Positive Impact ◽  
Case Series ◽  
Stroke Recurrence ◽  
Long Term Outcomes ◽  
Single Center Experience ◽  
The Mean

The purpose of this study is to investigate the surgical results and long-term outcomes of encephaloduroarteriosynangiosis for moyamoya disease in pediatric patients. We performed a retrospective analysis of 67 pediatric patients with moyamoya disease who underwent encephaloduroarteriosynangiosis in Beijing Tiantan Hospital. The case series included 36 boys and 31 girls. All the patients underwent surgical revascularization, and a total of 93 encephaloduroarteriosynangiosis procedures were performed (41 unilateral, 26 bilateral). The mean follow-up period after surgery was 30 months. During follow-up, ischemic stroke events were detected in 5 patients and the stroke rate for pediatric patients who underwent encephaloduroarteriosynangiosis procedure was 7.1% per patient-years. At the last follow-up, favorable outcomes (modified Rankin Scale score ≤ 2) were observed in 65 cases (97.0%). Our study suggests that long-term surgical outcome of encephaloduroarteriosynangiosis in pediatric moyamoya disease patients is satisfactory, and this technique has a positive impact on the prevention of stroke recurrence.

Long-term outcomes of lumbar microdiscectomy in the pediatric population: a large single-institution case series

2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  
Keyword(s):  
Conservative Management ◽  
Functional Outcomes ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Case Series ◽  
Csf Leak ◽  
Single Institution ◽  
The Mean

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.

MO291RITUXIMAB IS EFFECTIVE AND SAFE IN ADULTS WITH STEROID-DEPENDENT NEPHROTIC SYNDROME: A LONG-TERM, SINGLE-CENTER EXPERIENCE

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Gemma Patella ◽  
Alessandro Comi ◽  
Giuseppe Coppolino ◽  
Nicolino Comi ◽  
Giorgio Fuiano ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Case Series ◽  
Adult Patients ◽  
Single Center ◽  
Single Center Experience ◽  
Steroid Dependent ◽  
The Mean ◽  
Steroid Dependent Nephrotic Syndrome

Abstract Background and Aims Steroid-dependent nephrotic syndrome (SDNS) may require a prolonged multi-drug therapy with risk of drug toxicity and renal failure. Rituximab (RTX) treatment has been found to be helpful in reducing the steroid dosage and the need for immunosuppressants (ISs), but little data are currently available regarding very long-term outcomes in adults. We herein describe a long-term, single-center experience of RTX use in a large series of adults with SDNS. Method We studied 23 adult patients with SDNS (mean age 54.2±17.1 y; 65% male; BMI 28.5±4.7), mostly consequent to membranous (47.8%) or focal glomerulonephritis (30.2 %) who were eligible to start a RTX regimen. Before entering the RTX protocol, proteinuria and eGFR were 7.06±3.87 g/24h and 65.9±28.2 ml/min/1.73 m2, respectively; albumin and CD19/CD20 ratio were 2.9±0.9 g/L and 0.99±0.01 respectively; the mean number of ISs was 2.39±0.89 and the mean annual rate of relapses was 2.2±0.9. Results Patients were followed over a mean follow-up of 64 months (range: 12-144). After RTX (mean dose: 1202.1±372.4 mg) the rate of relapses was virtually nullified (p<0.001). eGFR remained roughly stable (62.1±19.8 ml/min/1.73 m2, p=NS), while proteinuria, albumin, CD19/CD20 and BMI all significantly improved (p ranging from 0.01 to 0.001). The mean number of additional ISs was also reduced (0.44±0.12; p<0.001) and RTX enabled discontinuation of steroids in 13/23 (56.5%) patients. No major adverse events related to therapy were recorded. Conclusion Findings from this large case-series with a remarkable very long follow-up reinforce the role of RTX as an efficient and safe weapon to improve outcomes in adult patients suffering from SDNS.

Long-term Outcomes of Microfracture for Treatment of Osteochondral Lesions of the Talus

2021 ◽  
pp. 107110072199542
Author(s):  
Daniel Corr ◽  
Jared Raikin ◽  
Joseph O’Neil ◽  
Steven Raikin
Keyword(s):  
Survival Rate ◽  
Case Series ◽  
Return To Sport ◽  
Osteochondral Lesions ◽  
Long Term Outcomes ◽  
Level Of Evidence ◽  
Additional Surgery ◽  
Case Series Study

Background: Microfracture is the most common reparative surgery for osteochondral lesions of the talus (OLTs). While shown to be effective in short- to midterm outcomes, the fibrocartilage that microfracture produces is both biomechanically and biologically inferior to that of native hyaline cartilage and is susceptible to possible deterioration over time following repair. With orthobiologics being proposed to augment repair, there exists a clear gap in the study of long-term clinical outcomes of microfracture to determine if this added expense is necessary. Methods: A retrospective review of patients undergoing microfracture of an OLT with a single fellowship-trained orthopedic surgeon from 2007 to 2009 was performed. Patients meeting the inclusion criteria were contacted to complete the Foot and Ankle Ability Measure (FAAM) Activities of Daily Living (ADL) and Sports subscales and visual analog scale (VAS) for pain, as well as surveyed regarding their satisfaction with the outcome of the procedure and their likelihood to recommend the procedure to a friend with the same problem using 5-point Likert scales. Patient demographics were reviewed and included for statistical analysis. Results: Of 45 respondents, 3 patients required additional surgery on their ankle for the osteochondral defect, yielding a 10-year survival rate of 93.3%. Of surviving cases, 90.4% (38/42) reported being “extremely satisfied” or “satisfied” with the outcome of the procedure. The VAS score at follow-up averaged 14 out of 100 (range, 0-75), while the FAAM-ADL and FAAM-Sports scores averaged 90.29 out of 100 and 82 out of 100, respectively. Thirty-six patients (85.7%) stated that their ankle did not prevent them from participating in the sports of their choice. Conclusion: The current study represents a minimum 10-year follow-up of patients undergoing isolated arthroscopic microfracture for talar osteochondral defects, with a 93.3% survival rate and 85.7% return to sport. While biological adjuvants may play a role in improving the long-term outcomes of microfracture procedures, larger and longer-term follow-up studies are required for procedures using orthobiologics before their cost can be justified for routine use. Level of Evidence: Level IV, retrospective cohort case series study.

Immunoadsorption for Recurrent Primary Focal Segmental Glomerulosclerosis on Kidney Allografts: A Single-Center Experience and Literature Review

2020 ◽  
Vol 49 (3) ◽  
pp. 322-333 ◽  
Author(s):  
Hamza Naciri Bennani ◽  
Juste Yérémandé Bonzi ◽  
Johan Noble ◽  
Florian Terrec ◽  
Lionel Motte ◽  
...  
Keyword(s):  
Primary Objective ◽  
Allograft Survival ◽  
Frequent Adverse Event ◽  
Single Center Experience ◽  
Cytomegalovirus Reactivation ◽  
Segmental Glomerulosclerosis ◽  
The Mean ◽  
Allograft Loss

Introduction: Primary focal and segmental glomerulosclerosis (FSGS) frequently reoccurs on kidney transplants and may lead to premature allograft loss. There are no guidelines for treating FSGS recurrence on allografts; treatment is based on apheresis (plasma exchange plasmapheresis [PP], semi-specific immunoadsorption [IA] with reusable columns) plus rituximab. Objective: We aimed to assess the efficacy of IA to treat recurrent FSGS. Methods: We report on 7 patients with recurrent FSGS on kidney allograft (proteinuria ≥3 g/g of urinary creatinine or ≥3 g/day); they all received IA. Our primary objective was to reduce proteinuria by >50%. Patients’ mean age was 45 ± 10 years. Postoperative immunosuppression relied on steroids, mycophenolate mofetil, tacrolimus, with an induction therapy of basiliximab or antithymocyte globulins. Prophylaxis to prevent FSGS recurrence was either rituximab alone (n = 3), rituximab plus either PP or IA (n = 3), or no treatment (n = 1). Mean follow-up was 20 ± 13 months. There was a median of 72 (14–101) IA sessions per patient, that is, a mean of 14 ± 1 sessions per IA column. Results: At 12 months after starting IA, all patients had partial (n = 6) or complete (n = 1) remission, and allograft survival was 100%. The mean reduction in proteinuria within an IA session was 45 ± 15%. At last follow-up, 2 patients are in remission without IA, 3 patients are in partial remission that is IA dependent, and 2 patients lost their allograft due to FSGS recurrence. The most frequent adverse event was cytomegalovirus reactivation (n = 13), which subsided after valganciclovir therapy. Conclusions: We show that recurrence of FSGS can be controlled long term with IA plus rituximab. However, some patients remained dependent on IA.

Snake envenomation-induced acute kidney injury: prognosis and long-term renal outcomes

2021 ◽  
pp. postgradmedj-2020-139021
Author(s):  
Manoj Kumar ◽  
Maasila Arcot Thanjan ◽  
Natarajan Gopalakrishnan ◽  
Dhanapriya Jeyachandran ◽  
Dineshkumar Thanigachalam ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Kidney Injury ◽  
Long Term Outcomes ◽  
Urine Protein ◽  
Renal Outcomes ◽  
Snake Envenomation ◽  
The Mean ◽  
New Onset

BackgroundSnake bite continues to be a significant cause of acute kidney injury (AKI) in India. There is paucity of data regarding long-term outcomes of such patients. In this study, we aim to assess the prognosis and long-term renal outcomes of such patients.MethodsWe analysed the hospital records of snake envenomation-induced AKI from January 2015 to December 2018. Predictors of in-hospital mortality were assessed. Survivors were advised to visit follow-up clinic to assess their kidney function.ResultsThere were 769 patients with evidence of envenomation and of them, 159 (20.7%) had AKI. There were 112 (70.4%) males. Mortality occurred in 9.4% of patients. Logistic regression analysis identified shock (OR 51.949, 95% CI 4.297 to 628.072) and thrombocytopenia (OR 27.248, 95% CI 3.276 to 226.609) as predictors of mortality. Forty-three patients attended the follow-up. The mean follow-up duration was 30.4±15.23 months. Adverse renal outcomes (eGFR <60 mL/min/1.73 m2 or new-onset hypertension (HTN) or pre-HTN or urine protein creatinine ratio >0.3) occurred in 48.8% of patients. Older age (mean age (years) 53.3 vs 42.8, p=0.004) and longer duration on dialysis (median duration (days) 11.5 vs 5, p=0.024) were significantly associated with adverse renal outcomes.ConclusionsThe incidence of AKI in snake envenomation was 20.7%. The presence of shock and thrombocytopenia were associated with mortality. Adverse renal outcomes occurred in 48.8% of patients in the long term.

Abstract TP397: Clinical And Functional Outcome Of Adult Patients Diagnosed With Moyamoya Disease In Childhood

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
David Fam ◽  
Nomazulu Dlamini ◽  
Cheryl Jaigobin
Keyword(s):  
Ischemic Stroke ◽  
North America ◽  
Natural History ◽  
Functional Outcome ◽  
Moyamoya Disease ◽  
Vascular Event ◽  
Moyamoya Syndrome ◽  
The Mean

Background: Moyamoya disease is a progressive occlusive arteriopathy of the terminal ICA and its branches, leading to the formation of unstable collateral vessels. The disease is found worldwide, and is associated with a number of predisposing conditions, termed moyamoya syndrome. Currently there is a paucity of data on the natural history of moyamoya disease in North America, especially the long-term outcome of pediatric moyamoya. Objective: Our objectives were to determine the rate of recurrent TIA, ischemic stroke and intracerebral hemorrhage in patients presenting with pediatric Moyamoya disease and to assess long-term functional outcome. Methods: This study was a retrospective chart review of 52 patients presenting with pediatric moyamoya disease. We included patients aged 18 or older with a diagnosis of moyamoya disease or syndrome. All patients had confirmatory baseline vascular imaging (MRA or CTA +/- cerebral angiography). Baseline demographic variables and annual vascular event recurrence risk were obtained from the records. Modified Rankin Score (MRS) at presentation and last follow-up were determined from clinical records. Results: Of the original cohort, 34 patients were included for analysis. The mean age of the patients was 23.9 years. The mean age at presentation was 9 years (SD=4) with an average follow-up of 11.3 years (SD=5.1) for a total of 383 patient years. There was slight female predominance (1.4:1). Fifty percent of patients (17) presented with ischemic stroke. A total of 26 patients (76%) underwent surgery, with 7 (21%) requiring repeat surgery. The annual vascular recurrent event rate was 5.0% (19/383), which was not significantly different between surgical and non-surgical groups or between different moyamoya syndrome subtypes. Most of the recurrent events were TIA (annual recurrence 3.7%). There was no significant difference between initial MRS, MRS at last follow-up and mean change in MRS between surgical and non-surgical groups. Conclusion: Our study is the largest North American natural history study of pediatric moyamoya. Our observations indicate that pediatric moyamoya in North America have low recurrent vascular event rates and long-term functional outcomes are good, even in conservatively managed patients.

scholarly journals Transscleral Fixation of Black Diaphragm Intraocular Lens in Complete Aniridia and Aphakia Due to Posttraumatic Eye Rupture: A Pilot Study

2019 ◽  
Vol 8 (1) ◽  
pp. 46
Author(s):  
Tomasz Chorągiewicz ◽  
Katarzyna Nowomiejska ◽  
Dariusz Haszcz ◽  
Dominika Nowakowska ◽  
Teresio Avitabile ◽  
...  
Keyword(s):  
Intraocular Lens ◽  
Anterior Segment ◽  
Case Series ◽  
Functional Restoration ◽  
Long Term Outcomes ◽  
Ahmed Valve ◽  
Post Traumatic ◽  
Valve Implantation

Introduction: To assess long-term outcomes of implantation of black diaphragm intraocular lens (BD IOL) in post-traumatic aniridia and aphakia due to eye rupture. Methods: This is a retrospective consecutive case series of 14 eyes with post-traumatic complete aniridia and aphakia treated with scleral fixation BD IOL. Measurements included ophthalmological comorbidities, best corrected visual acuity (BCVA), complications, and postoperative interventions. The average postoperative follow-up period was 36 months. Results: BCVA improved in 6 cases, was stable in 6 cases and worsened in 2 cases. The lens was well centered in 13 cases. Glaucoma was diagnosed in six cases developed, and three of them required Ahmed valve implantation. One lens developed opacity. The cornea was decompensated in 6 cases, while two of them required penetrating keratoplasty. Conclusion: Implantation of BD IOL in eyes with severely traumatized eyes enables reconstruction of the anterior segment and some functional restoration, although many complications may arise during the longitudinal follow-up.

scholarly journals Clinical features and long-term outcomes of moyamoya disease: a single-center experience with 528 cases in China

2015 ◽  
Vol 122 (2) ◽  
pp. 392-399 ◽  
Author(s):  
Xing-ju Liu ◽  
Dong Zhang ◽  
Shuo Wang ◽  
Yuan-li Zhao ◽  
Mario Teo ◽  
...  
Keyword(s):  
Clinical Features ◽  
Moyamoya Disease ◽  
Positive Impact ◽  
Chinese Patients ◽  
Single Center ◽  
Long Term Outcomes ◽  
Kaplan Meier ◽  
Significant Difference ◽  
Revascularization Procedures

OBJECT The aim of this study was to describe the baseline clinical features and long-term outcomes of patients with moyamoya disease (MMD) based on a 25-year period at a single center in China. METHODS  Data obtained in 528 consecutive patients with MMD treated at the authors' hospital from 1984 to 2010 were reviewed retrospectively. Events of transient ischemic attack, new infarction, and hemorrhage were included. The Kaplan-Meier risk of stroke was calculated. RESULTS  The mean (± SD) patient age was 26 ± 13 years (range 2–67 years), and the female/male ratio was 0.9:1. There were 332 cases of ischemia and 196 hemorrhages. Adults had a higher rate of bleeding than children (50.7% vs 14.0%, respectively; p < 0.001). One hundred twenty-two patients were treated conservatively, and 406 patients underwent revascularization procedures. Of 528 patients, 331 (62.7%) had at least 1 year of follow-up (median 39.5 months) and data from these patients were analyzed. Rebleeding and mortality rates in patients with hemorrhagic MMD (n = 104) were higher than in those with ischemic MMD (n = 227) (26.9% vs 2.2% [p < 0.001] and 4.8% vs 0.4% [p < 0.05], respectively). Twenty-five of 60 (41.7%) conservatively treated patients and 8 of 271 (2.9%) surgically treated patients experienced rebleeding events, a difference that was significant in the Kaplan-Meier curve of rebleeding (p < 0.01). An improvement in perfusion was found in 164 of 224 (73.2%) surgically treated patients 1 month after discharge. However, there was no significant difference in the rate of ischemic events in the surgical and conservative groups (18.8% and 28.3%, respectively; p = 0.09). Among the 104 hemorrhagic cases, rebleeding attacks were observed in 25 patients in the nonsurgical group (n = 60) and 3 patients in the surgical group (n = 44) (41.7% and 6.8%, respectively; OR 9.7 [95% CI 2.7–35.0]; p < 0.01). CONCLUSIONS  There was no difference in the sex distribution of Chinese patients with MMD. Patients with hemorrhagic MMD had a much higher rate of rebleeding and poorer prognosis than those with the ischemic type. Surgical revascularization procedures can improve cerebral perfusion and have a positive impact in preventing rebleeding in patients with hemorrhagic MMD.

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