Risks associated with oral deferiprone in the treatment of infratentorial superficial siderosis

Abstract Objective Deferiprone is an iron chelator that has recently been used to treat patients with infratentorial superficial siderosis (iSS). It is considered to have a generally favourable safety profile but concerns have been raised due to the risk of agranulocytosis. We aimed to evaluate the safety and tolerability of oral deferiprone as a treatment for patients with iSS. Methods We present a case series of 10 consecutive patients presenting with classical iSS treated with deferiprone. Results Ten patients were followed up for a mean period of 2.3 years (range 0.5–5.5 years). Four patients (40%) were withdrawn from treatment because of treatment-related side effects. The reasons for treatment discontinuation were neutropenic sepsis (n = 3) and fatigue (n = 1). In 2 out of the 3 cases of neutropenic sepsis, patients initially developed neutropenia without sepsis. The mean time to neutropenic sepsis following deferiprone was 1.2 years (range 0.3–2.5) with mean neutrophil count of 0.4 (range 0.3–0.5). Six patients (60%) reported no change in neurological function while on treatment, and four patients (40%) reported that their condition deteriorated. Conclusions Deferiprone was poorly tolerated, with 40% of patients withdrawing from treatment, most commonly due to neutropenic sepsis, after an average of 2 years on treatment. This study increases the number of reported cases of agranulocytosis in patients with iSS treated with deferiprone. Clinicians treating iSS patients with deferiprone should be aware that this drug has a potentially life-threatening side effect of neutropenic sepsis, and should ensure that appropriate haematological monitoring is in place.

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Management of Vertical Sternal Fracture Nonunion in Elite-Level Athletes

Orthopaedic Journal of Sports Medicine ◽

10.1177/23259671211010804 ◽

2021 ◽

Vol 9 (6) ◽

pp. 232596712110108

Author(s):

Andrea Bardos ◽

Sanjeeve Sabhrawal ◽

Graham Tytherleigh-Strong

Keyword(s):

Ct Scan ◽

Constant Score ◽

Case Series ◽

Sternal Fracture ◽

Elite Level ◽

The Mean ◽

Mean Time ◽

Time From Injury ◽

Sternal Fractures

Background: Sternal fractures are rare, and they can be treated nonoperatively. Vertical sternal fractures have rarely been reported. Purpose: To describe the management and surgical treatment of a series of elite-level athletes who presented with symptomatic nonunions of a vertical sternal fracture. Study Design: Case series; Level of evidence, 4. Methods: Patients with an established symptomatic nonunion of a vertical sternal fracture, as diagnosed by computed tomography (CT) or magnetic resonance imaging (MRI), underwent open reduction and internal fixation using autologous bone graft and cannulated lag screws. The patients were assessed preoperatively and at the final follow-up using the Rockwood sternoclavicular joint (SCJ) score; Constant score; and shortened version of the Disabilities of the Arm, Shoulder and Hand (QuickDASH) scores. Bony union was confirmed on postoperative CT scan. Results: Five patients (4 men and 1 woman) were included; all were national- or international-level athletes (rugby, judo, show-jumping, and MotoGP). The mean age at surgery was 23.4 years (range, 19-27 years), the mean time from injury to referral was 13.6 months (range, 10-17 months), and the mean time from injury to surgery was 15.8 months (range, 11-20 months). The mean follow-up was 99.4 months (range, 25-168 months). There was a significant improvement after surgery in the mean Rockwood SCJ score (from 12.6 to 14.8 [ P < .05]), Constant score (from 84 to 96.4 [ P < .05]; 80% met the minimal clinically important difference [MCID] of 10.4 points), and QuickDASH (from 6.8 to 0.98 [ P < .05]; 0% met the MCID of 15.9 points). Four of the patients were able to return to sport at their preinjury level, and 1 patient retired for nonmedical reasons. All of the fractures had united on the postoperative CT scan. There were no postoperative complications. Conclusion: Vertical fractures of the sternum are very rare and tend to behave clinically like an avulsion fracture injury to the capsuloligamentous structure of the inferior SCJ. The requirement of advanced imaging to diagnose this injury means that the actual incidence and natural history are not known. For high-demand athletes, early identification, surgical reduction, and fixation are likely to achieve the best outcome.

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Plasmapheresis in the treatment of myasthenia gravis: retrospective study of 26 patients

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2004000300003 ◽

2004 ◽

Vol 62 (2b) ◽

pp. 391-395 ◽

Cited By ~ 9

Author(s):

Rosana Carandina-Maffeis ◽

Anamarli Nucci ◽

José F.C. Marques Jr ◽

Eduardo G. Roveri ◽

Beatriz H.M. Pfeilsticker ◽

...

Keyword(s):

Retrospective Study ◽

Side Effects ◽

Myasthenia Gravis ◽

Plasma Exchange ◽

Mortality Rates ◽

Motor Deficit ◽

Myasthenic Crisis ◽

Clinical Hospital ◽

The Mean ◽

Mean Time

We analyzed the experience of Unicamp Clinical Hospital with plasma exchange (PE) therapy in myasthenia gravis (MG). About 17.8 % of a totality of MG patients had PE performed: 26 cases, 19 women and seven men. The mean age-onset of MG was 28 years, extremes 11 and 69. Minimum deficit observed in the group was graded IIb (O & G) or IIIa (MGFA scale). One patient had prethymectomy PE. In seven the procedures were performed due to myasthenic crisis and in 18 patients due to severe myasthenic symptoms or exacerbation of previous motor deficit. Two patients were also submitted to chronic PE considering refractoriness to other treatments. Twenty-six patients had 44 cycles of PE and 171 sessions. The mean number of sessions was 3.9 (SD ± 1.4) each cycle; median 5, extremes 2 and 6. The mean time by session was 106,5 minutes (SD ± 35.2); median 100.5 (extremes of 55 and 215). The mean volume of plasma exchanged in each session was 2396 ml (SD ± 561); median 2225 (extremes 1512 and 4500). Side effects occurred: reversible hypotension (seven cases), mild tremor or paresthesias (seven cases). Infection and mortality rates due to PE were zero. All patients had immediate benefit of each PE cycle and usually they also received prednisone or other immunosuppressors. Good acceptance of the procedure was observed in 80.7% of patients.

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Our comprehensive experience with tracheostomy in tertiary multi-speciality hospital over four years

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20213900 ◽

2021 ◽

Vol 7 (10) ◽

pp. 1649

Author(s):

Parth B. Kapadia ◽

Manit M. Mandal ◽

Ajay J. Panchal ◽

Rakesh Kumar ◽

Neel Parmar ◽

...

Keyword(s):

Medical Education ◽

Retrospective Study ◽

Surgical Procedure ◽

Case Series ◽

Final Outcome ◽

Patient Age ◽

Significant Case ◽

Surgical Emphysema ◽

The Mean ◽

Mean Time

Background: Tracheostomy is a common surgical procedure used to achieve a secure airway in patients in elective as well as emergency conditions. Our study accounts for our first-hand experience with the procedure in our tertiary multi-speciality hospital.Methods: It is a four year (January 2017 to February 2021) retrospective study in which all the 246 cases of tracheostomies were taken into account which were performed during this study period in the department of otorhinolaryngology (ENT), Surat Municipal Institute of Medical Education and Research (SMIMER), Surat (India). The patients were thoroughly followed up until the final outcome of the tracheostomy was established. Results: A total of 246 tracheostomies were performed, 63.82% of which were in men. The mean patient age was 42 years. The majority (76%) were elective, performed for various indications, while the remaining 24% were emergency tracheostomies. All tracheostomies were performed by otorhinolaryngologists. Complications relating to tracheostomy were documented in 40 patients (16%) intra-operatively and/or post-operatively which included bleeding, surgical emphysema, granulations along the stomal site and rarely shock. The mean time to decannulation was 13.48 days.Conclusions: Our study takes into account and discusses the findings of all the 246 tracheostomies which were performed during the study period of 4 years. Elective cases form the majority although there is a significant case series of emergency tracheostomies performed for a range of pathologies.

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COVID-19 in HIV: a Review of Published Case Reports

SN Comprehensive Clinical Medicine ◽

10.1007/s42399-020-00593-6 ◽

2020 ◽

Vol 2 (12) ◽

pp. 2647-2657

Author(s):

Zoya Morani ◽

Saumil Patel ◽

Sudeshna Ghosh ◽

Falah Abu Hassan ◽

Shriya Doreswamy ◽

...

Keyword(s):

Morbid Obesity ◽

Web Of Science ◽

Case Reports ◽

Case Series ◽

Hiv Patients ◽

High Burden ◽

Cardiovascular Comorbidities ◽

Life Threatening ◽

The Mean ◽

Immunocompromised State

AbstractPatients with COVID-19 present with a myriad of comorbidities. An immunocompromised state like HIV in patients with COVID-19 can be life-threatening. We searched PubMed/Medline, Scopus, and Web of Science for case reports and case series about COVID-19 in HIV patients. We finally reviewed 20 case reports including cases of 43 patients with HIV and COVID-19. The mean age of 43 adult patients was 51.56 ± 27.56 years (range 24–76 years). Of these, 30 were male (69.77%), 11 were female (25.58%), and 2 were transgender (4.65%). A total of 25 patients (58.14%) were above 50 years of age. The most common cardiovascular comorbidities were hypertension and hyperlipidemia (48.8%), diabetes (20.93%), and morbid obesity (11.63%). Out of 43 HIV patients with COVID-19, 6 resulted in death (13.95%). All the patients who died were elderly above 50 years and required mechanical ventilation. HIV patients infected with COVID-19 had a high mortality rate. A high burden of pre-existing comorbidities and an advanced age in these patients make them prone to disease progression and worse outcomes.

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Epidemiological and clinical characteristics of 671 COVID-19 patients in Henan Province, China

International Journal of Epidemiology ◽

10.1093/ije/dyaa081 ◽

2020 ◽

Vol 49 (4) ◽

pp. 1085-1095

Author(s):

Yifei Nie ◽

Jitian Li ◽

Xueyong Huang ◽

Wanshen Guo ◽

Xiaobai Zhang ◽

...

Keyword(s):

Clinical Laboratory ◽

Case Series ◽

Henan Province ◽

X Rays ◽

Illness Onset ◽

Medical Disorders ◽

History Of ◽

The Mean ◽

Wet Market ◽

Mean Time

Abstract Background Despite many reports on the characteristics of coronavirus disease 2019 (COVID-19) in Wuhan, China, relatively little is known about the transmission features of COVID-19 outside Wuhan, especially at the provincial level. Methods We collected epidemiological, demographic, clinical, laboratory, radiological and occupation information, along with contact history, of 671 patients with laboratory-confirmed COVID-19 reported from January 23 to February 5, 2020, in Henan province, China. We described characteristics of these cases, compared the diagnostic accuracy and features of blood testing, computed tomography (CT) scans and X-rays, and analysed SARS-CoV-2 transmission sources and patients’ occupations in Henan province. Results The mean age of patients in this case series was 43 years, 56.2% were male and 22.4% had coexisting medical disorders. The death rate was 0.3%. Fourteen patients did not show any symptoms. Lymphocyte percentage was associated with disease severity (χ2 = 6.71, P = 0.035) but had a large variation in each sample group. The mean time from illness onset to diagnosis was 5.6 days. A total of 330 patients had ever lived in or visited Wuhan, 150 had contact with confirmed cases, 323 had been to a hospital and 119 had been to a wet market. There were 33 patients who did not have a traceable transmission source, with 21.2% of these being farmers and 15.2% being workmen. Conclusions Lymphocyte percentage was a sign of severe COVID-19 in general but was not a good diagnostic index. Longer time from illness onset to diagnosis was associated with higher COVID-19 severity, older age, higher likelihood of having coexisting cardiovascular diseases including hypertension, and being male. Farming was found to be a high-risk occupation in Henan province, China.

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The Safety and Effectiveness of the Percutaneous Flexor Tenotomy in Healing Neuropathic Apical Toe Ulcers in the Outpatient Setting

Foot & Ankle Specialist ◽

10.1177/1938640019843314 ◽

2019 ◽

Vol 13 (2) ◽

pp. 123-131

Author(s):

Simon E. Smith ◽

Julie Miller

Keyword(s):

Patient Satisfaction ◽

Ulcer Healing ◽

Case Series ◽

Outpatient Setting ◽

The Mean ◽

Prospective Case Series ◽

Protective Sensation ◽

Loss Of Protective Sensation ◽

Mean Time ◽

Flexor Tenotomy

Percutaneous flexor tenotomy is a minimally invasive procedure that involves cutting one or both flexor digitorum tendons underneath the toe. It is an alternative to open surgical procedures performed in an operating theatre. This project is a prospective case series investigating the utility, effectiveness, and participant satisfaction of the percutaneous flexor tenotomy when performed in the outpatient setting. People with diabetes, digital flexion deformity, and loss of protective sensation with an apical ulcer or preulcerative lesion presenting to the Austin Health Foot Ulcer Specialist Outpatient Clinic were enrolled in the study. The procedure was performed under sterile technique with an 18-gauge needle or fine scalpel blade. Participants were reviewed weekly until the incision and/ or ulcer healed. Participants were then followed up at 3 and 6 months. Time to ulcer healing, reulceration rate, frequency of complications, and patient satisfaction were recorded. There were 11 toe ulcers and 41 preulcerative lesions. A total of 76 tenotomy procedures were performed on the 23 participants across 35 episodes of care. The mean time to ulcer healing following tenotomy was 10.2 ± 4.3 days. There were no recurrences of ulceration. The infection rate per episode of care was 2.8%. There were 11 toes (14.5%) that sustained a transfer lesion post the index procedure. The mean time to development of a transfer lesion was 95.5 ± 98.1 days. All participants strongly agreed that they were satisfied with the outcome of the procedure. The flexor tenotomy is an effective procedure to expedite the healing of apical toe ulcers in people with loss of protective sensation and flexion digital deformity. It has a low infection rate and high patient satisfaction rate when performed in the outpatient setting. There is a moderate risk of transfer preulcerative lesions to an adjacent digit. Levels of Evidence: Level IV: Prospective case series

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Mycophenolate Mofetil Therapy Is a Steroid Sparing In Childhood and Young Adult Chronic ITP and Evans Syndrome.

Blood ◽

10.1182/blood.v116.21.1441.1441 ◽

2010 ◽

Vol 116 (21) ◽

pp. 1441-1441

Author(s):

Zaher Naji ◽

Madhvi Rajpurkar ◽

Sureyya Savasan ◽

Roland Chu ◽

Meera Chitlur ◽

...

Keyword(s):

Mycophenolate Mofetil ◽

Side Effects ◽

Bleeding Complications ◽

Severe Bleeding ◽

Evans Syndrome ◽

Corticosteroid Administration ◽

Chronic Itp ◽

Life Threatening ◽

The Mean ◽

Steroid Sparing

Abstract Abstract 1441 Chronic ITP and Evans syndrome are diseases characterized by autoantibody formation with resultant destruction of platelets or both platelets and red blood cells on neutrophils, respectively. Affected patients are at risk of life threatening bleeding complications and/or life threatening anemia. Conventional therapies are often ineffective or transiently effective and have significant toxicity. One of the most commonly used therapeutic strategies employs chronic corticosteroid administration with the attendant weight gain, hypertension, hyperglycemia, bone loss, infection risk, mood changes and protean other undesirable side effects. Mycophenolate Mofetil (MMF) is an immunosuppressive agent with a favorable side effect profile. It is converted to the active metabolite, mycophenolic acid, and interferes with purine metabolism in T-lymphocytes, effectively killing many of these cells and down-regulating autoimmune phenomena. With approval of our local IRB /HIC we retrospectively reviewed the charts of 11 chronic ITP/Evans syndrome patients who had received MMF, all such patients treated at a large urban Children's Hospital. Clinical variables included age, sex, duration of disease, steroid use, IVIG use, Anti-D use, platelet counts, hemoglobin concentrations and reticulocyte percentages. These data were analyzed using paired t-tests, one-sample t-test and descriptive statistics. The 11 patients ranged in age from 9–22 years old, with a mean age of 15 years. The mean time from diagnosis of disease was 41.8 months with a range of 6–95 months. There were 5 female subjects, 6 Evans syndrome patients and 5 with chronic ITP alone. The median platelet count over the 6 months prior to MMF was 70×109/L, (18-223), with a median of 90×109/L with (27-145) during the first 6 months of MMF therapy (p=0.4). In the Evans syndrome group, the mean hemoglobin prior to MMF was 10.9 g/dL, (9.1-14.6), with a mean of 12.1 g/dL, (6.9-16.7) on MMF (p=0.54). Similarly, the mean reticulocyte percentage was 2.7%, (0.5-14) prior to MMF, with a mean of 2.3%, (0.3-8.9) during MMF therapy. The mean total dose of steroids used in the 6 months prior to MMF was 84.2 mg/kg (prednisone equivalent), (11.2-170), compared to 62.2 mg/kg, (0-193.8) on MMF. 9 of 11 patients had reductions in steroid requirement by an average of 49.3% (p=0.0013). During the first 6 months of MMF both IVIG and anti-D usage decreased from total doses for the entire group of 17 and 3 doses to 8 and 1 dose, respectively. None of the patients experienced severe bleeding episodes or side effects more serious than a transient rash while on MMF. These data suggest that MMF may have a role as a steroid sparing therapy in the treatment of chronic ITP and Evans syndrome. Disclosures: Off Label Use: mycophenolate mofetil for ITP Evans syndrome.

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Spondylodiscitis Caused by Aspergillus Species

Diagnostics ◽

10.3390/diagnostics11101899 ◽

2021 ◽

Vol 11 (10) ◽

pp. 1899

Author(s):

Christos Koutserimpas ◽

Ifigeneia Chamakioti ◽

Symeon Naoum ◽

Konstantinos Raptis ◽

Kalliopi Alpantaki ◽

...

Keyword(s):

Surgical Treatment ◽

Multidisciplinary Approach ◽

Surgical Intervention ◽

Treatment Options ◽

Standard Of Care ◽

Clinical Challenge ◽

Life Threatening ◽

The Mean ◽

Magnetic Resonance Imaging Mri ◽

Mean Time

Background: Spondylodiscitis caused by Aspergillus spp. is a rare but life-threatening clinical entity. However, a consensus on diagnostic criteria and most effective medical management is still missing. The present study is a review of all published cases of spondylodiscitis caused by Aspergillus spp., in an effort to elucidate epidemiology, patients’ characteristics, andand the medical and surgical treatment options and their effectiveness. Methods: A thorough review of all existing spondylodiscitis cases caused by Aspergillus was performed. Data regarding demographics, responsible fungus, time between symptoms’ onset and firm diagnosis, antifungal treatment (AFT), surgical intervention, andand the infection’s outcome were investigated. Results: A total of 118 Aspergillus spondylodiscitis cases, yielding 119 Aspergillus spp. isolates, were identified in the literature. The patients’ mean age was 40.6 years. Magnetic resonance imaging (MRI) (after its introduction) indicated the diagnosis in most cases (66.7%), while definite diagnosis was established through cultures in the majority of cases (73.7%). Aspergillus fumigatus was isolated in most cases (73; 61.3%), followed by Aspergillus flavus (15; 12.6%) andand Aspergillus nidulans and terreus (7; 5.9%, each). The mean time between symptoms’ onset and diagnosis was 5.7 months. Amphotericin B was the preferred antifungal regiment (84 cases; 71.2%), followed by voriconazole (31; 26.3%), and the mean AFT duration was 6.1 months. The final outcome was successful in 93 cases (78.8%). Furthermore, 77 patients (65.3%) underwent surgery. Conclusions: Spondylodiscitis caused by Aspergillus spp. represents a clinical challenge, requiring a multidisciplinary approach. The present review has shown that prolonged AFT has been the standard of care of the studied cases, while surgical treatment seems to play an important role in selected patents.

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Efficiency of infiltration anesthesia with levobupivacaine and ropivacaine after the knee arthroplasty

N.N. Priorov Journal of Traumatology and Orthopedics ◽

10.17116/vto201803-04142 ◽

2018 ◽

Vol 25 (3-4) ◽

pp. 42-46

Author(s):

A. M Ageenko ◽

V. S Baitov ◽

S. A Pervukhin

Keyword(s):

Side Effects ◽

Knee Arthroplasty ◽

Randomized Study ◽

Pain Syndrome ◽

Narcotic Analgesics ◽

Total Knee ◽

The Mean ◽

Main Component ◽

Mean Time ◽

Infiltration Anesthesia

Purpose: to study the efficacy and safety of levobupivacaine as the main component of infiltration anesthesia after knee arthroplasty. Patients and methods. Open randomized study included 284 patients (20 - 81 years) after total knee arthroplasty. Patients were divided into 2 groups, 142 patients in each group, depending on the anesthetic used (levobupivacaine or ropivacaine). The severity of pain syndrome within the first 48 hours after the operation by VAS, the need for the use of narcotic analgesics, presence of side effects was evaluated. Results. The mean time of acute pain occurrence was 255±83 min in ropivacaine group and 238±87 min in levobupiavacaine group (p=108). In 4 hours after surgical intervention 30 and 43% of patients from ropivacaine and levobupiavacainegroups required narcotic analgetics, respectively. Neither serious side effects nor complications directly related to infiltration anesthesia were recorded. Conclusion. Infiltration anesthesia with both levobupivacaine and ropivacaine is simple, safe and effective method for pain arrest after surgical interventuions of the knee joint.

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The Use of Sixty Degree Rotation of the Acetabulum for Treatment of Dogs With Canine Hip Dysplasia. A Short Case Series

Frontiers in Veterinary Science ◽

10.3389/fvets.2021.669680 ◽

2021 ◽

Vol 8 ◽

Author(s):

Horia Elefterescu ◽

Ciprian Andrei Ober ◽

Andrei Timen ◽

Christos Yiapanis ◽

William McCartney ◽

...

Keyword(s):

Hip Dysplasia ◽

Case Series ◽

Pelvic Osteotomy ◽

Term Outcome ◽

Secondary Osteoarthritis ◽

Clinical Consequences ◽

The Mean ◽

Canine Hip Dysplasia ◽

Mean Time

Triple pelvic osteotomy (TPO) is a prophylactic surgical procedure performed on dogs with canine hip dysplasia. The procedure is indicated in skeletally immature dogs without secondary osteoarthritis (OA). It has been suggested that 60° of rotation is excessive and is associated with poor outcome. The objective of the study was to assess the medium term outcome in dogs having undergone triple pelvic osteotomy (TPO) using 60° dedicated plates. Nine TPOs were performed in seven dogs with hip dysplasia. Eight of nine hips had 72–100% osseous union at the time of revisit. The mean time to final radiographic recheck was 200 days (range, 185–229 days). The mean time to follow-up was 11.5 months (range 11–12 months). All 7 dogs had regained full function and did not require supplemental analgesia. Pelvic canal narrowing was noted in the two dogs with bilateral surgeries, but no clinical consequences were noted according to owner's statement.If more than 40 degrees reduction angles at Ortolani test, 60° of rotation of the acetabulum can be used successfully in dogs with hip dysplasia. At the time of mid-term follow-up, all dogs in this case series had full function.

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