scholarly journals Liver Fibrosis Associated with Iron Accumulation Due to Long-Term Heme-Arginate Treatment in Acute Intermittent Porphyria: A Case Series

2015 ◽  
pp. 77-81 ◽  
Author(s):  
Barbara Willandt ◽  
Janneke G. Langendonk ◽  
Katharina Biermann ◽  
Wouter Meersseman ◽  
François D’Heygere ◽  
...  
Keyword(s):  
Liver Fibrosis ◽  
Case Series ◽  
Acute Intermittent Porphyria ◽  
Iron Accumulation ◽  
Heme Arginate

scholarly journals Prophylactic Heme Arginate Infusion for Acute Intermittent Porphyria

2021 ◽  
Vol 12 ◽  
Author(s):  
Hung-Chou Kuo ◽  
Chia-Ni Lin ◽  
Yi-Fen Tang
Keyword(s):  
Renal Function ◽  
Body Weight ◽  
Acute Intermittent Porphyria ◽  
Porphobilinogen Deaminase ◽  
Attack Frequency ◽  
Weekly Administration ◽  
Clinical Counseling ◽  
Heme Arginate ◽  
Before And After

Objectives: This study aimed to evaluate the efficacy of long-term weekly prophylactic heme arginate (HA) infusions in reducing attack frequency and severity in female AIP patients.Methods: We report the results of five female AIP patients with frequent recurrent attacks (>9/year) before and after institution of weekly prophylaxis with heme arginate (3 mg/kg body weight). All five cases had confirmed disease-associated mutations in the porphobilinogen deaminase gene, and all had received genetic and clinical counseling about AIP.Results: In the five included patients, average annual attack rate (AAR) in the year prior to HA prophylaxis was 11.82 (range 9.03–17.06), and average total HA usage was 32.60 doses (range: 13.71–53.13). After 2.58–14.64 years of HA prophylaxis, average AAR was reduced to 2.23 (range 0.00–5.58), and attack severity (i.e., doses required per attack) was reduced from 2.81 to 1.39 doses/attack. Liver and renal function remained stable during weekly administration of HA prophylaxis. The most common complications were port-A catheter-related events. No other complications or safety concerns occurred with long-term use of HA prophylaxis.Conclusion: Our study demonstrated women with AIP receiving weekly prophylactic HA infusions resulted in fewer episodes that required acute HA treatment while maintaining stable renal and liver function. Weekly prophylactic HA infusions effectively prevent frequent porphyric attacks and reduce attack severity.

Force Plate Analysis of Dogs with Bilateral Hip Dysplasia Treated with a Unilateral Triple Pelvic Osteotomy: A Long-term Review of Cases

1998 ◽  
Vol 11 (02) ◽  
pp. 85-93 ◽  
Author(s):  
Joanne R. Cockshutt ◽  
H. Dobson ◽  
C. W. Miller ◽  
D. L. Holmberg ◽  
Connie L. Taves ◽  
...  
Keyword(s):  
Gait Analysis ◽  
Hip Dysplasia ◽  
Weight Bearing ◽  
Force Plate ◽  
Case Series ◽  
Pelvic Osteotomy ◽  
Vertical Force ◽  
Long Term Outcome ◽  
Case Series Study

SummaryA retrospective case series study was done to determine the long-term outcome of operations upon dogs treated for canine hip dysplasia by means of a triple pelvic osteotomy (TPO). Twentyfour dogs with bilateral hip dysplasia, that received a unilateral TPO between January 1988 and June 1995, were re-examined at the Ontario Veterinary College. The assessment included physical, orthopedic and lameness examinations, standard blood work, pelvic radiographs and force plate gait analysis. They were compared to bilaterally dysplastic dogs that had not been treated, and also to normal dogs. Force plate data analysis demonstrated a significant increase in peak vertical force (PVF) and mean vertical force over stance (MVF) in the limb that underwent surgical correction by means of a TPO, when compared to the unoperated hip. It was determined that performing a unilateral TPO on a young dysplastic dog resulted in greater forces and weight bearing being projected through the TPO corrected limb when compared to the unoperated limb.Dogs with bilateral hip dysplasia treated with a unilateral triple pelvic osteotomy (TPO) were assessed by force plate gait analysis, radiographs and orthopedic examination. There was a significant increase in hip Norberg angles over time, although degenerative changes did progress. Limbs that had been operated upon had significantly greater peak and mean ground reaction forces than limbs that had not received an operation.

Mechanical failure of the Mobi-C implant for artificial cervical disc replacement: report of 4 cases

2020 ◽  
Vol 33 (6) ◽  
pp. 727-733
Author(s):  
Jasmine A. T. DiCesare ◽  
Alexander M. Tucker ◽  
Irene Say ◽  
Kunal Patel ◽  
Todd H. Lanman ◽  
...  
Keyword(s):  
Cervical Spondylosis ◽  
Case Series ◽  
Dynamic Imaging ◽  
Disc Replacement ◽  
Cervical Disc ◽  
Artificial Disc ◽  
Younger Patients ◽  
Cervical Disc Replacement ◽  
Short And Long Term

Cervical spondylosis is one of the most commonly treated conditions in neurosurgery. Increasingly, cervical disc replacement (CDR) has become an alternative to traditional arthrodesis, particularly when treating younger patients. Thus, surgeons continue to gain a greater understanding of short- and long-term complications of arthroplasty. Here, the authors present a series of 4 patients initially treated with Mobi-C artificial disc implants who developed postoperative neck pain. Dynamic imaging revealed segmental kyphosis at the level of the implant. All implants were locked in the flexion position, and all patients required reoperation. This is the first reported case series of symptomatic segmental kyphosis after CDR.

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.

Mechanical thrombectomy in pediatric stroke: systematic review, individual patient data meta-analysis, and case series

2019 ◽  
Vol 24 (5) ◽  
pp. 558-571 ◽  
Author(s):  
Kartik Bhatia ◽  
Hans Kortman ◽  
Christopher Blair ◽  
Geoffrey Parker ◽  
David Brunacci ◽  
...  
Keyword(s):  
Systematic Review ◽  
Ischemic Stroke ◽  
Acute Ischemic Stroke ◽  
Mechanical Thrombectomy ◽  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Neurological Outcomes

OBJECTIVEThe role of mechanical thrombectomy in pediatric acute ischemic stroke is uncertain, despite extensive evidence of benefit in adults. The existing literature consists of several recent small single-arm cohort studies, as well as multiple prior small case series and case reports. Published reports of pediatric cases have increased markedly since 2015, after the publication of the positive trials in adults. The recent AHA/ASA Scientific Statement on this issue was informed predominantly by pre-2015 case reports and identified several knowledge gaps, including how young a child may undergo thrombectomy. A repeat systematic review and meta-analysis is warranted to help guide therapeutic decisions and address gaps in knowledge.METHODSUsing PRISMA-IPD guidelines, the authors performed a systematic review of the literature from 1999 to April 2019 and individual patient data meta-analysis, with 2 independent reviewers. An additional series of 3 cases in adolescent males from one of the authors’ centers was also included. The primary outcomes were the rate of good long-term (mRS score 0–2 at final follow-up) and short-term (reduction in NIHSS score by ≥ 8 points or NIHSS score 0–1 at up to 24 hours post-thrombectomy) neurological outcomes following mechanical thrombectomy for acute ischemic stroke in patients < 18 years of age. The secondary outcome was the rate of successful angiographic recanalization (mTICI score 2b/3).RESULTSThe authors’ review yielded 113 cases of mechanical thrombectomy in 110 pediatric patients. Although complete follow-up data are not available for all patients, 87 of 96 (90.6%) had good long-term neurological outcomes (mRS score 0–2), 55 of 79 (69.6%) had good short-term neurological outcomes, and 86 of 98 (87.8%) had successful angiographic recanalization (mTICI score 2b/3). Death occurred in 2 patients and symptomatic intracranial hemorrhage in 1 patient. Sixteen published thrombectomy cases were identified in children < 5 years of age.CONCLUSIONSMechanical thrombectomy may be considered for acute ischemic stroke due to large vessel occlusion (ICA terminus, M1, basilar artery) in patients aged 1–18 years (Level C evidence; Class IIb recommendation). The existing evidence base is likely affected by selection and publication bias. A prospective multinational registry is recommended as the next investigative step.

Primary Calvarial Ewing Sarcoma: A Case Series

2021 ◽  
Author(s):  
Sandeep Mohindra ◽  
Manjul Tripathi ◽  
Aman Batish ◽  
Ankur Kapoor ◽  
Ninad Ramesh Patil ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Tumor Resection ◽  
Case Series ◽  
Radical Excision ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Intradural Tumor ◽  
Age Range

Abstract Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting their clinical and radiological findings. Method In a retrospective analysis, we evaluated our 12-year database for pathologically proven EWS. A literature search was conducted for the comparative presentation and update on the management and outcome. Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3; age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS. All cases underwent wide local excision; two patients required intradural tumor resection, while one required rotation flap for scalp reconstruction. Mean hospital stay was 8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained asymptomatic at 5 years of follow-up, while two patients died. Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first two decades of life. These tumors can be purely intracranial, causing raised intracranial pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy may offer a favorable long-term outcome.

scholarly journals Minimum 10-Year Clinical Outcome of Lateral Collagen Meniscal Implants for the Replacement of Partial Lateral Meniscal Defects: Further Results From a Prospective Multicenter Study

2021 ◽  
Vol 9 (5) ◽  
pp. 232596712199491
Author(s):  
Alberto Grassi ◽  
Gian Andrea Lucidi ◽  
Giuseppe Filardo ◽  
Piero Agostinone ◽  
Luca Macchiarola ◽  
...  
Keyword(s):  
Survival Rate ◽  
Case Series ◽  
Final Analysis ◽  
Activity Level ◽  
Long Term Results ◽  
Level Of Evidence ◽  
Unicompartmental Knee Arthroplasties ◽  
Tegner Score

Background: The collagen meniscal implant (CMI) is a biologic scaffold aimed at replacing partial meniscal defects. The long-term results of lateral meniscal replacement have never been investigated. Purpose: To document the clinical outcomes and failures of lateral CMI implantation for partial lateral meniscal defect at a minimum 10-year follow-up. Study Design: Case series; Level of evidence, 4, Methods: This study included 24 consecutive patients who underwent lateral CMI implantation for partial lateral meniscal defects between April 2006 and September 2009 and who were part of a previous study with a 2-year follow-up. Outcome measures at the latest follow-up included the Lysholm score, Knee injury and Osteoarthritis Outcome Score, visual analog scale (VAS) for pain, Tegner activity level, and EuroQol 5-Dimensions score. Data regarding complications and failures were collected, and patients were asked about their satisfaction with the procedure. Results: Included in the final analysis were 19 patients (16 male, 3 female) with a mean age at surgery of 37.1 ± 12.6 years and a mean follow-up of 12.4 ± 1.5 years (range, 10-14 years). Five failures (26%) were reported: 1 CMI removal because of implant breakage and 4 joint replacements (2 unicompartmental knee arthroplasties and 2 total knee arthroplasties). The implant survival rate was 96% at 2 years, 85% at 5 years, 85% at 10 years, 77% at 12 years, and 64% at 14 years. Lysholm scores at the final follow-up were rated as “excellent” in 36% (5 of 14 nonfailures), “good” in 43% (6 of 14), and “fair” in 21% (3 of 14). The VAS score was 3.1 ± 3.1, with only 16% (3 of 19 patients) reporting that they were pain-free; the median Tegner score was 3 (interquartile range, 2-5). All clinical scores decreased from the 2-year follow-up; however, with the exception of the Tegner score, they remained significantly higher compared with the preoperative status. Overall, 79% of patients were willing to undergo the same procedure. Conclusion: Lateral CMI implantation for partial lateral meniscal defects provided good long-term results, with a 10-year survival rate of 85% and a 14-year survival rate of 64%. At the final follow-up, 58% of the patients had “good” or “excellent” Lysholm scores. However, there was a general decrease in outcome scores between the short- and the long-term follow-up.

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.

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