scholarly journals Attenuated androgen discontinuation in patients with hereditary angioedema: a commented case series

2022 ◽  
Vol 18 (1) ◽  
Author(s):  
Marcus Maurer ◽  
Markus Magerl ◽  
Emel Aygören-Pürsün ◽  
Konrad Bork ◽  
Henriette Farkas ◽  
...  
Keyword(s):  
Side Effects ◽  
Hereditary Angioedema ◽  
Treatment Strategies ◽  
Case Series ◽  
Additional Patient ◽  
Type I ◽  
Retrospective Case ◽  
Androgen Withdrawal ◽  
Life Threatening ◽  
Attenuated Androgen

Abstract Background Hereditary angioedema (HAE) is characterized by potentially severe and life-threatening attacks of localized swelling. Prophylactic therapies are available, including attenuated androgens. Efficacy of attenuated androgens has not been assessed in large, randomized, placebo-controlled trials and can be associated with frequent, and sometimes severe, side effects. As better tolerated targeted therapies become available, attenuated androgen withdrawal is increasingly considered by physicians and their patients with HAE. Attenuated androgens withdrawal has not been systematically studied in HAE, although examination of other disorders indicates that attenuated androgen withdrawal may result in mood disturbances and flu-like symptoms. Standardized protocols for attenuated androgen discontinuation that continue to provide control of attacks while limiting potential attenuated androgen withdrawal symptoms are not established as the outcomes of different withdrawal strategies have not been compared. We aim to describe the challenges of attenuated androgen discontinuation in patients with HAE and how these may continue into the post-androgen period. Case presentation We present a retrospective case series of 10 patients with confirmed type I HAE who have discontinued prophylactic treatment with attenuated androgens. The most common reason for attenuated androgen discontinuation was side effects. Attenuated androgens were either immediately withdrawn, tapered and/or overlapped with another treatment. The major challenge of discontinuation was the management of an increased frequency and severity of HAE attacks in some patients. Conclusions Healthcare teams need to undertake careful planning and monitoring after attenuated androgens discontinuation, and modify treatment strategies if HAE control is destabilized with an increased number of attacks. Discontinuation of attenuated androgens is definitively an option in an evolving HAE treatment landscape, and outcomes can be favourable with additional patient support and education.

scholarly journals Macrophage Activation Syndrome in Adults: A Retrospective Case Series

2021 ◽  
Vol 9 ◽  
pp. 232470962110264
Author(s):  
Taylor Warmoth ◽  
Malvika Ramesh ◽  
Kenneth Iwuji ◽  
John S. Pixley
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Pediatric Patients ◽  
Inflammatory Diseases ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Case Series ◽  
Adult Patients ◽  
Retrospective Case ◽  
Life Threatening ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a form of hemophagocytic lymphohistocytosis that occurs in patients with a variety of inflammatory rheumatologic conditions. Traditionally, it is noted in pediatric patients with systemic juvenile idiopathic arthritis and systemic lupus erythematous. It is a rapidly progressive and life-threatening syndrome of excess immune activation with an estimated mortality rate of 40% in children. It has become clear recently that MAS occurs in adult patients with underlying rheumatic inflammatory diseases. In this article, we describe 6 adult patients with likely underlying MAS. This case series will outline factors related to diagnosis, pathophysiology, and review present therapeutic strategies.

scholarly journals Deferiprone as adjunctive treatment for patients with invasive mucormycosis: A retrospective case series

2018 ◽  
Vol 10 (2) ◽  
Author(s):  
Maria N. Chitasombat ◽  
Pimjai Niparuck
Keyword(s):  
Mortality Rate ◽  
Treatment Strategies ◽  
Case Series ◽  
Hematologic Malignancies ◽  
Adjunctive Treatment ◽  
Combination Regimen ◽  
Retrospective Case ◽  
Day Range

Mucormycosis is a life-threatening disease requiring multimodal treatment with antifungals and surgery. The mortality rate remains high, prompting consideration of alternative treatment strategies. Deferiprone has in vitro activity against Mucorales, but its efficacy has never been evaluated in humans. Here, we retrospectively analyzed patients with confirmed mucormycosis who received deferiprone from 2011 to 2017. Five patients had hematologic malignancies and one was diabetic. The sites of infection included sinus-orbit-cerebral (67%), lung (17%), and disseminated infection (17%). Surgery was performed in 83% of cases and achieved local control for 33% of patients. A combination regimen of polyenes plus echinocandins was administered with stepdown treatment using posaconazole. The median duration of antifungal treatment was 86 days (range: 46-435 days) days. Deferiprone was given as adjunctive treatment with a median dose and duration of 100 mg/kd/day (range: 86.2-100 mg/kg/day) and 25 days (range: 15-215 days), respectively. Overall, deferiprone was well-tolerated. Successful outcomes were observed at 12-week follow-up for 67% of patients. The mortality rate at 180- day follow-up was 50%. Adjunctive therapy with deferiprone showed safety and tolerability.

scholarly journals Off-licence use of clozapine in patients with emotionally unstable personality disorder: a case series analysis

BJPsych Open ◽  
2021 ◽  
Vol 7 (S1) ◽  
pp. S116-S116
Author(s):  
Declan Hyland ◽  
Alex Walmsley ◽  
Victoria Simpson
Keyword(s):  
Side Effects ◽  
Personality Disorder ◽  
Suicide Attempts ◽  
Symptom Control ◽  
Case Series ◽  
Community Support ◽  
Retrospective Case ◽  
Psychotherapeutic Treatment ◽  
Self Harm ◽  
Emotionally Unstable Personality Disorder

ObjectiveThis retrospective case series followed emotionally unstable personality disorder (EUPD) patients following initiation of clozapine on an off-licence basis, aiming to examine tolerance by determining side effect prevalence and treatment cessation frequency, as well as examining efficacy, by investigating number of hospital re-admissions and symptom control.Case reportThis case series captured the experiences of 11 EUPD patients under the care of Mersey Care NHS Foundation Trust, all of whom had, at some time in the past five years, been initiated on clozapine. All patients were white British females, with a median age of 31. The median daily dose of clozapine was 300 mg. Most patients had significant psychiatric comorbidities, as well as illicit substance and / or alcohol misuse.Whilst prescribed clozapine, patients were only admitted to hospital once on average and this was commonly for clozapine re-titration. Whilst in hospital, rates of self-harm were low, but ligaturing and suicide attempts showed higher prevalence. Patients still demonstrated self-harming behaviour out of hospital leading to A and E presentations. In the community, contacts with the police were minimal, with only two patients undergoing Section 136 assessments or arrests.All patients reported side effects from clozapine - usually hypersalivation, over-sedation and constipation. All 11 patients experienced sinus tachycardia. Eight patients temporarily ceased taking clozapine at some point. In three patients, discontinuation of clozapine was as a result of intolerable side effects. Three patients experienced neutropenia, which subsequently resolved. Only two patients had a body mass index within healthy range.DiscussionDespite patients reporting clozapine to provide symptomatic benefit for their EUPD, and improved their engagement with mental health services, prevalence of self-harm and of A and E presentations remained high, indicating the importance of community support and concomitant psychotherapeutic treatment. Patients with more robust community support showed greater adherence to clozapine.High prevalence of side effects and obesity in these patients, in addition to risk of developing neutropenia, highlights the importance of rigorous monitoring after initiating clozapine. It is reassuring that, despite development of neutropenia in some patients, this recovered quickly, and clozapine treatment could resume.ConclusionClozapine may be an effective pharmacological treatment for enabling EUPD patients to engage more therapeutically with services. Clozapine may be of greater benefit to those with more stable, less chaotic lives. Although diminished, patients still show self-harming behaviour and need for A and E admissions and re-hospitalisation. Side effects of clozapine are common and regular monitoring is required.

Risk factors for corneal epithelial wound healing: Can sex play a role?

2021 ◽  
pp. 112067212110667
Author(s):  
Giulia Coco ◽  
Kevin J. Hamill ◽  
Lee D. Troughton ◽  
Stephen B. Kaye ◽  
Vito Romano
Keyword(s):  
Risk Factors ◽  
Wound Healing ◽  
Treatment Strategies ◽  
Case Series ◽  
Healing Time ◽  
Retrospective Case ◽  
Corneal Epithelial ◽  
Female Patients ◽  
Epithelial Wound Healing ◽  
Wound Healing Time

Purpose To determine if sex is associated with corneal epithelial wound healing time in patients with persistent corneal epithelial defects (PCEDs). Methods Retrospective case series on patients with PCED from November 2014 to January 2019. Records of 127 patients with diagnosis of PCED were reviewed. Patients with an epithelial defect that lasted more than two weeks in the absence of an active corneal infection were included. Main outcome was corneal epithelial wound healing time. Results 55 patients (29 males) with a mean age of 65.3 ± 16.5 years were included. No difference was found between female and male patients in terms of risk factors, age, treatment strategies or intervals between visits (median of 15 days in females and 12 days in males; p = 0.24). Median duration of the PCED was 51 days (IQR 32-130), with a median number of 5 clinical visits (IQR 4-8). Female patients had significantly longer healing times (p = 0.004) and a corresponding increase in the number of clinical visits (median of 7 visits vs. 5 clinical visits in males, p = 0.012). Conclusion Results from this study suggest female patients with PCED might have a longer corneal epithelial wound healing duration and may therefore require earlier intervention.

scholarly journals Nivolumab and Ipilimumab in the Treatment of Metastatic Uveal Melanoma: A Single-Center Experience

2019 ◽  
Vol 2019 ◽  
pp. 1-8 ◽  
Author(s):  
Vidhya Karivedu ◽  
Ihab Eldessouki ◽  
Ahmad Taftaf ◽  
Zheng Zhu ◽  
Abouelmagd Makramalla ◽  
...  
Keyword(s):  
Partial Response ◽  
Clinical Outcome ◽  
Uveal Melanoma ◽  
Stable Disease ◽  
Checkpoint Inhibitors ◽  
Evaluation Criteria ◽  
Treatment Strategies ◽  
Case Series ◽  
Retrospective Case ◽  
Study Results

Background. Metastatic uveal melanoma (MUM) is associated with a poor prognosis, with a median overall survival (OS) of 4–15 months. Despite new insights into the genetic and molecular background of MUM, satisfactory systemic treatment approaches are currently lacking. The study results of innovative treatment strategies are urgently needed. Patients and Methods. This was a retrospective case series of 8 patients with MUM managed at the University of Cincinnati between January 2015 and January 2018. The immune-related Response Evaluation Criteria in Solid Tumors (irRECIST) 1.1 criteria were used for patient evaluation, and magnetic resonance imaging was used for evaluation at treatment checkpoints. Objective. To assess the clinical outcome of patients with MUM treated with a combination of checkpoint inhibitors. Results. The series included eight patients, six men and two women, with MUM. Their median age at MUM diagnosis was 69 (range, 55–77) years. All patients were treated with ipilimumab and nivolumab combination along with transarterial chemoembolization (TACE), followed by nivolumab maintenance and monthly TACE procedures. The majority of patients had a partial response or stable disease. Two of the patients had partial response, while four others had stable disease. Two other patients experienced disease progression. Conclusion. We report the outcomes of eight patients with MUM treated with the combination of ipilimumab and nivolumab. We report the clinical outcome and toxicity associated with this treatment approach. Further studies are warranted to explore immunotherapy in MUM. These findings support the consideration of immunotherapy in MUM.

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

2019 ◽  
Vol 267 (1) ◽  
pp. 239-243 ◽  
Author(s):  
Y. Sammaraiee ◽  
G. Banerjee ◽  
S. Farmer ◽  
B. Hylton ◽  
P. Cowley ◽  
...  
Keyword(s):  
Side Effects ◽  
Case Series ◽  
Iron Chelator ◽  
Superficial Siderosis ◽  
Neutropenic Sepsis ◽  
Favourable Safety ◽  
Favourable Safety Profile ◽  
Life Threatening ◽  
The Mean ◽  
Mean Time

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.

CRPS of Lower Extremity– A Case Report and Review

2016 ◽  
Vol 27 (1) ◽  
pp. 22-28
Author(s):  
Mahima Agrawal
Keyword(s):  
Case Report ◽  
Lower Extremity ◽  
Lower Limb ◽  
Relaxation Training ◽  
Single Case ◽  
Case Series ◽  
Pain Syndrome ◽  
Type I ◽  
Retrospective Case ◽  
Single Case Report

Abstract Complex regional pain syndrome (CRPS) of the lower limb is a relatively uncommon entity as compared to CRPS of the upper extremity. Literature search has revealed only 2 retrospective case series and a single case report of lower extremity CRPS type I from 1975 to 2014 on Pubmed, isolated cases of CRPS type I of lower extremity have also been reported following knee surgeries and arthroscopies. This report presents a case of lower limb CRPS type I following blunt trauma to right foot, treatment of which was directed towards management of allodynia, vasomotor symptoms and surgical correction of deformity which had developed because of the disease, coping mechanisms were also reinforced through counselling and relaxation training. The individual responded well to treatment with a reported 75% reduction in the disabling symptoms and improvement in ambulatory status.

Idiopathic syringomyelia: retrospective case series, comprehensive review, and update on management

2011 ◽  
Vol 31 (6) ◽  
pp. E15 ◽  
Author(s):  
Anil K. Roy ◽  
Nicholas P. Slimack ◽  
Aruna Ganju
Keyword(s):  
Conservative Management ◽  
Incidental Finding ◽  
Imaging Techniques ◽  
Case Series ◽  
Neurological Dysfunction ◽  
Type I ◽  
Retrospective Case ◽  
Chiari Malformation Type I ◽  
Comprehensive Review ◽  
Idiopathic Syringomyelia

Object A syrinx is a fluid-filled cavity within the spinal cord that can be an incidental finding or it can be accompanied by symptoms of pain and temperature insensitivity. Although it is most commonly associated with Chiari malformation Type I, the advancement of imaging techniques has resulted in more incidental idiopathic syringes that are not associated with Chiari, tumor, trauma, or postinfectious causes. The authors present a comprehensive review and management strategies for the idiopathic variant of syringomyelia. Methods The authors retrospectively identified 8 idiopathic cases of syringomyelia at their institution during the last 6 years. A PubMed/Medline literature review yielded an additional 38 articles. Results Two of the authors' patients underwent surgical treatment that included a combination of laminectomy, lysis of adhesions, duraplasty, and syrinx fenestration. The remaining 6 patients were treated conservatively and had neurologically stable outcomes. Review of the literature suggests that an etiology-driven approach is essential in the diagnosis and management of syringomyelia, although conservative management suffices for most cases. In particular, it is important to look at disturbances in CSF flow, as well as structural abnormalities including arachnoid webs, cysts, scars, and a diminutive posterior fossa. Conclusions The precise etiology for idiopathic syringomyelia (IS) is still unclear, although conceptual advances have been made toward the overall understanding of the pathophysiology of IS. Various theories include the cerebellar piston theory, intramedullary pulse pressure theory, and increased spinal subarachnoid pressure. For most patients with IS, conservative management works well. Continued progression of symptoms, however, could be approached using decompressive strategies such as laminectomy, lysis of adhesions, and craniocervical decompression, depending on the level of pathology. Management for patients with progressive neurological dysfunction and the lack of flow disturbance is unclear, although syringosubarachnoid shunting can be considered.

Levetiracetam monotherapy for treatment of structural epilepsy in dogs: 19 cases (2010–2015)

2017 ◽  
Vol 181 (15) ◽  
pp. 401-401 ◽  
Author(s):  
Darren Kelly ◽  
Francesca Raimondi ◽  
Nadia Shihab
Keyword(s):  
Side Effects ◽  
Seizure Control ◽  
Case Series ◽  
Poor Response ◽  
Response To Treatment ◽  
Seizure Freedom ◽  
Retrospective Case ◽  
Cluster Seizures ◽  
Structural Epilepsy

To evaluate the efficacy and tolerability of levetiracetam monotherapy in dogs with structural epilepsy. Retrospective case series. Nineteen client-owned dogs with structural epilepsy. Seizure frequencies after initiation of treatment were used to evaluate the efficacy of levetiracetam monotherapy. Seizure control was considered good if no seizures occurred within three months of starting treatment or poor if seizures returned within one month of starting treatment. Tolerability was evaluated by considering the occurrence and severity of any reported side effects. Ten of the 19 dogs were considered to have a good response to treatment with 7 achieving complete seizure freedom. Nine dogs were considered to have poor response to treatment. There was a statistically significant reduction in the percentage of patients experiencing cluster seizures from 68.4% to 15.8% (p=0.002). Side effects were noted in 8 of the 19 dogs but were considered mild in all cases. Follow-up times ranged from 12 days to 426 days. When used in conjunction with other appropriate therapies, levetiracetam may be an efficacious option for monotherapy in dogs with structural epilepsy. Its tolerability makes it a suitable option for use in a wide variety of patients.

scholarly journals Clinical Characteristics of Severe COVID-19 in China: A Case Series and Meta-analysis

2021 ◽  
Author(s):  
Geling Teng ◽  
Hua Hu ◽  
Xia Wang ◽  
Junsheng Wang ◽  
Hongmei Zhang ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Coronary Heart Disease ◽  
Heart Disease ◽  
Bacterial Infections ◽  
Clinical Signs ◽  
Treatment Strategies ◽  
Case Series ◽  
Imaging Features ◽  
Chest Tightness ◽  
Retrospective Case

Abstract Background: There have been inconsistent reports regarding the unique manifestations of severe coronavirus disease 2019 (COVID-19) occurring in China. This study analyzed the clinical manifestation of 13 severe COVID-19 cases at a single institution and compared the data to previously reported characteristics of severe COVID-19 in China. Methods: This retrospective case study included patients with severe COVID-19 who were admitted to the isolation ward of the Shandong Chest Hospital from January 2020 to February 2020. The clinical signs and symptoms, laboratory examination results, imaging features, treatment strategies, and patient prognoses were summarized. A database search was then conducted for studies published through December 2020 documenting characteristics of severe COVID-19 cases in China. The pooled results for severe COVID-19 patients in China were calculated by using the random-effects model.Results: A total of 4 severe and 9 critical patients were included from Shandong Chest Hospital. The average patient age was 55.3 (range 23-88) years, and 61.5% of patients were male. Chest computed tomography for all patients showed multiple lesions as ground-glass shadows in both lungs. All patients presented bacterial infection and various degrees of liver and myocardial injury. The treatment strategies for patients included antibiotics, immunoglobulin, and glucocorticoids, and mechanical ventilation was used in all patients for respiratory failure. Two patients died, and 11 recovered. In the pooled data for severe COVID-19 patients, the most common comorbidities were hypertension, diabetes mellitus, and coronary heart disease. The common signs in these patients were fever, cough, fatigue, chest tightness, and a leukocyte count > 10. Conclusions: Older males with hypertension, diabetes mellitus, and coronary heart disease may be at higher risk of developing severe COVID-19. Patients should be assessed for concomitant bacterial infections. Cardiac and liver enzymes, fever, cough, fatigue, chest tightness, and leukocytosis should be monitored for signs of disease progression.

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