scholarly journals Thrombotic Microangiopathy in a Severe Pediatric Case of COVID-19

2021 ◽  
Vol 15 ◽  
pp. 117955652110498
Author(s):  
Tahir Dalkıran ◽  
Yaşar Kandur ◽  
Emine Manolya Kara ◽  
Besra Dağoğlu ◽  
Sevgin Taner ◽  
...  
Keyword(s):  
Respiratory Distress ◽  
Thrombotic Microangiopathy ◽  
Treatment Options ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Respiratory Acidosis ◽  
Pediatric Intensive Care ◽  
Peripheral Blood Smear ◽  
Fresh Frozen Plasma ◽  
Activity Level

In this case report, we report a pediatric patient with COVID-19 and atypical hemolytic uremic syndrome (aHUS). A 3-year-old girl with fever and respiratory distress was admitted to the hospital. The patient tested positive for COVID-19 by a PCR test. As her respiratory distress increased and blood gas indicated deep respiratory acidosis on the third day of the pediatric intensive care unit follow-up, the patient was intubated and ventilated. Thorax computerized tomography (CT) showed bilateral effusion and atelectasis. During her follow-up, the pleural effusion resolved but there were marked consolidation areas and ground glass opacities compatible with COVID-19 on the follow-up CT. On the 10th day, she became anuric and developed progressive thrombocytopenia and persistent microangiopathic hemolytic anemia, which were suggestive of HUS given a high creatinine level (1.9 mg/dl), an undetectable haptoglobin level, reticulocytosis (8%), and an LDH level of 2540 U/l. Direct Coombs test returned negative. Examination of a peripheral blood smear revealed schistocytes. Disseminated intravascular coagulation was ruled out by normal INR and PTT. In the light of the available findings, we considered the patient to have thrombotic microangiopathy (TMA) triggered by COVID-19. It was not a TTP-form of TMA but rather an aHUS type, based on an ADAMTS13 activity level >5%. Hence, plasmapheresis was performed with fresh frozen plasma (FFP). After 4 weeks, she became completely asymptomatic, and her hemodynamic parameters normalized. COVID-19 induced uncontrolled complement activation leading to the development of aHUS. Early diagnosis and treatment may reduce morbidity and mortality since its treatment options.

scholarly journals Therapeutic Plasma Exchange for Thrombotic Microangiopathy

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4939-4939
Author(s):  
Osman Ilhan ◽  
Erden Atilla ◽  
Pinar Ataca Atilla ◽  
Sinem Civriz Bozdag ◽  
Meltem Kurt Yuksel ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Thrombotic Microangiopathy ◽  
Conflicts Of Interest ◽  
Complete Response ◽  
Fresh Frozen Plasma ◽  
Therapeutic Plasma Exchange ◽  
Time Period ◽  
Fresh Frozen ◽  
Neurological Disturbance

Abstract Background: Thrombotic microangiopathy (TMA) is a syndrome characterized by thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, fever and renal dysfunction. Therapeutic Plasma Exchange (TPE) is a standart treatment approach for the patients. Our aim is to determine the clinical characteristics and outcome of patients with TMA treated with TPE in our center. Patients and Methods: We retrospectively evaluated 46 patients who have been diagnosed as TMA at Ankara University Department of Hematology between 2007 and 2015. Patients were treated with TPE (Frensenius Kabi AG, Homburg, Germany) until the normalization of laboratory parameters. The plasma exchanged was 1.5 times the predicted plasma volume with fresh frozen plasma for the first procedure, and usually 1.0 time the predicted volume thereafter until remission. Results: 22M/24F was included in the study with a median age of 55 (range, 18-8310 of 46 patients (22%) were consultated from intensive care units, 7/46 (15%) from emergency unit and 6/46 (13%) from nephrology unit. The most common presenting symptom was purpura in 52%, followed by neurological disturbance 48%, renal function abnormality in 43% and fever in 28% of patients. At diagnosis the median hemoglobin (g/dl), leucocyte count (10^9/L) and thrombocyte count (10^9/L) were as follows: 9.4, 11.4 and 58.6. Median time period of procedure was 99 minutes (range, 64-313). 5/46 (11%) patients had femoral catheters and central venous catheters were the access for the rest of patients. None of the patients had severe adverse events during procedures. 21 patients achieved complete response (46%) after 2-40 sessions and 2 of them were died during follow-up. Responders were diagnosed mostly with infectious related TMA. 21 patients (46%) who had progressive disease died within 30 days after diagnosis. 2 non-responder patients had diagnosis of TTP and treated with succesfully with Rituximab (Table 1). Conclusions: TPE is safe treatement modality in patients with TMA however there is still high mortality rate. Disclosures No relevant conflicts of interest to declare.

scholarly journals Varied Clinical Presentation and Management of Pediatric Vallecular Cyst

2021 ◽  
Author(s):  
Jyoti Singh ◽  
Neha Jain ◽  
Mamta Jajoo ◽  
Suparna Roy ◽  
Ekta Narang ◽  
...  
Keyword(s):  
Respiratory Distress ◽  
Reliable Method ◽  
Clinical Presentation ◽  
Treatment Options ◽  
Transoral Approach ◽  
Diagnostic Tools ◽  
Long Term Follow Up ◽  
Feeding Difficulty

The aim of this article is to highlight the varied presentation and management of vallecular cysts. Three children with diagnosis of vallecular cyst presented in our department were reviewed retrospectively for clinical presentation, diagnostic tools and treatment options. All three cases presented with respiratory and feeding difficulty. Diagnosis in all the three cases was made with laryngoscopy (flexible/direct) and imaging. All three patients were treated by transoral approach aimed for excision of cyst using cold instruments. Postoperative period was uneventful and no recurrences were observed on the long-term follow up. Vallecular cysts should be considered as one of the differentials in children with respiratory distress and dysphagia in spite of being a rare anomaly. Direct transoral approach for excision of the vallecular cyst is recommended as a safe and reliable method with no recurrences till date.    Keywords: Vallecular Cyst; Respiratory Distress; Dysphagia; Laryngoscopy

New Therapeutic Targets and Treatment Options for Thrombotic Microangiopathy: Caplacizumab and Ravulizumab

2020 ◽  
pp. 106002802094185
Author(s):  
Clement Chung
Keyword(s):  
Thrombotic Microangiopathy ◽  
English Language ◽  
Data Extraction ◽  
Multiorgan Failure ◽  
Treatment Options ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Organ Damage ◽  
Disease Recurrence ◽  
Current Standard ◽  
Complement Components

Objective: To review the efficacy and safety of caplacizumab and ravulizumab for thrombotic microangiopathy. Data Sources: A literature search from January 2011 to May 2020 was performed using the key terms caplacizumab (or ALX-0681), ravulizumab (or ALXN1210), atypical hemolytic uremic syndrome (aHUS), acquired thrombotic thrombocytopenic purpura (aTTP), and thrombotic microangiopathy. Study Selection and Data Extraction: Relevant clinical trials and articles in the English language were identified and reviewed. Data Synthesis: aTTP and aHUS are syndromes of thrombotic microangiopathy manifested by excessive platelet aggregation and endothelial cell destruction, with subsequent thrombocytopenia, hemolysis, and multiorgan failure. Current standard therapy for aTTP is therapeutic plasma exchange (TPE) to remove von Willebrand factor (vWF) multimers and anti-ADAMTS13 autoantibodies. As an adjunctive therapy to TPE, caplacizumab inhibits binding of vWF to platelets and prevents new microthrombi formation. It reduces thromboembolic event rate and days of TPE and delays relapse. Headache and epistaxis were the most common adverse events. aHUS develops because of dysregulation of the alternative complement pathway, followed by constitutive activation of complement components that causes thrombosis and end-organ damage. Short-term initial evaluation with ravulizumab, a long-acting complement inhibitor, demonstrates rapid hematological and renal improvement, with sustained complement inhibition and tolerable adverse effects. Relevance to Patient Care and Clinical Practice: This review describes the pharmacology, pharmacokinetics, cost consideration, and clinical studies for caplacizumab and ravulizumab for thrombotic microangiopathy. Place of therapy is also discussed. Conclusion: Targeted therapies with caplacizumab and ravulizumab are expected to reduce the burden of exacerbation, refractory disease, recurrence, and possibly death for thrombotic microangiopathy.

Antiretroviral Treatment-Associated Labia Majora Lipohypertrophy: A Rare Case and Plastic Surgical Treatment Options

2017 ◽  
Vol 2 (1) ◽  
pp. 43
Author(s):  
Akmal Hisham ◽  
Devananthan Ilenghoven ◽  
Wan Syazli Wan Ahmad Kamal ◽  
Salina Ibrahim ◽  
Shah Jumaat Mohd Yussof
Keyword(s):  
Highly Active Antiretroviral Therapy ◽  
Daily Living ◽  
Treatment Options ◽  
Hiv Positive ◽  
Highly Active ◽  
Labia Majora ◽  
The Face ◽  
Central Fat

The emergence of highly active antiretroviral therapy (HAART) has revolutionized the prognosis of HIV-infected patients. However, the extended use of HAART is associated with a disfiguring complication termed lipodystrophy, a disorder of body fat maldistribution causing peripheral fat loss (lipoatrophy) and central fat accumulation (lipohypertrophy). Lipoatrophy commonly affects the face, legs, buttocks and arm, whilst lipohypertrophy frequently favours the abdomen, breast and dorsocervical region. To our knowledge, we present only the second documented case in the literature of a labia majora lipohypertrophy in a HIV-positive patient receiving long-term HAART. The severity of labial abnormality caused significant physical and functional morbidities. Labiaplasty with dermolipectomy of the labia majora and excisional lipectomy of the mons pubis was successfully performed. At a 6-month follow-up, patient had no recurrence with resolution of symptoms and resumption of normal activities of daily living (ADL).

Efficacy and safety of oral dapsone in acne vulgaris – experience of a tertiary care teaching hospital in central Lahore

2020 ◽  
Vol 14 (2) ◽  
pp. 87-90
Author(s):  
Sadaf Amin Chaudhry ◽  
Nadia Ali Zafar ◽  
Rabia Hayat ◽  
Ayesha Noreen ◽  
Gulnaz Ali ◽  
...  
Keyword(s):  
Side Effects ◽  
Therapeutic Option ◽  
Acne Vulgaris ◽  
Treatment Options ◽  
Tertiary Care ◽  
Poor Response ◽  
Global Assessment Scale ◽  
Severe Acne ◽  
Hematologic Profile

Background: Acne is the eighth most prevalent disease affecting 9.4% of the population worldwide and its prevalence in our country is estimated to be around 5%. Severe inflammatory acne is most likely to leave scars and in order to prevent facial disfigurement due to acne scarring, early treatment is desirable. Various treatment options have been formulated for acne, and are tailored according to the severity of the disease. Numerous clinical trials have been conducted till now, to determine the usefulness and side effect profile of such therapies, making acne treatment a highly studied area in dermatology. Objective of this study is to highlight the fact that oral Dapsone could be used as a cheaper alternate to isotretinoin in recalcitrant severe acne, especially in females where retinoids are sometimes contraindicated. Patients and methods: 51 patients, suffering from severe nodulocystic acne, fulfilling the criteria, were enrolled from the Department of Dermatology, Sir Ganga Ram Hospital, Lahore. All the study patients were given oral Dapsone 50mg for initial two weeks and then 100mg daily for the next 10 weeks along with oral cimetidine and topical clindamycin application twice daily. Investigator Global Assessment Scale (IGAS) was employed to measure effectiveness. The treatment was considered ʽeffectiveʹ if the patient achieves 2 or more than 2-grade improvement or almost clear or clear skin at the end of 12 weeks according to IGAS scale. The lesion counts were also done before the start of therapy (day 1) and at every two weeks follow up for 12 weeks. The change in lesion count observed between the baseline number and that seen at follow up visits was also used to evaluate the effectiveness of oral Dapsone. Safety was analyzed by fortnightly visits of the patients to look for any undesirable side effects and monitoring of the hematologic profile of the patients. Final follow up was done at the end of 16 weeks. Results: The study was conducted on 51 patients, with a ratio of 1:3 for males and females and a mean age of 25.2 years (SD ±5.81). At 12th week, patients had significant reduction in their acne lesions; with 7 patients (13.7%) showing completely clear skin, 17 patients (33.3%) had almost clear skin, 5 patients (9.8%) had 3-grade improvement. Twelve patients (23.5%) had 2-grade improvement from baseline score and only 2 patients (3.9%) had 1-grade improvement from baseline. Based on percentage reduction of lesions, excellent response was seen in 32 patients (62.7%), good response in 9 patients (17.6%), moderate response in 2 patients (3.9%), while no patient showed poor response. Dapsone was discontinued in 8 patients due to derangement of hematologic profile. Conclusion: Oral Dapsone, when given carefully, is a very effective therapeutic option in severe recalcitrant acne, with limited side effects.

scholarly journals Data Driven Mathematical Model of FOLFIRI Treatment for Colon Cancer

Cancers ◽  
2021 ◽  
Vol 13 (11) ◽  
pp. 2632
Author(s):  
Aparajita Budithi ◽  
Sumeyye Su ◽  
Arkadz Kirshtein ◽  
Leili Shahriyari
Keyword(s):  
Mathematical Model ◽  
Colon Cancer ◽  
Cancer Patients ◽  
Immune Cell ◽  
Treatment Options ◽  
Data Driven ◽  
T Helper ◽  
Primary Tumors ◽  
Cell Fractions

Many colon cancer patients show resistance to their treatments. Therefore, it is important to consider unique characteristic of each tumor to find the best treatment options for each patient. In this study, we develop a data driven mathematical model for interaction between the tumor microenvironment and FOLFIRI drug agents in colon cancer. Patients are divided into five distinct clusters based on their estimated immune cell fractions obtained from their primary tumors’ gene expression data. We then analyze the effects of drugs on cancer cells and immune cells in each group, and we observe different responses to the FOLFIRI drugs between patients in different immune groups. For instance, patients in cluster 3 with the highest T-reg/T-helper ratio respond better to the FOLFIRI treatment, while patients in cluster 2 with the lowest T-reg/T-helper ratio resist the treatment. Moreover, we use ROC curve to validate the model using the tumor status of the patients at their follow up, and the model predicts well for the earlier follow up days.

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.

scholarly journals Robotic Radiosurgery for Persistent Postoperative Acromegaly in Patients with Cavernous Sinus-Invading Pituitary Adenomas—A Multicenter Experience

Cancers ◽  
2021 ◽  
Vol 13 (3) ◽  
pp. 537
Author(s):  
Felix Ehret ◽  
Markus Kufeld ◽  
Christoph Fürweger ◽  
Alfred Haidenberger ◽  
Paul Windisch ◽  
...  
Keyword(s):  
Disease Control ◽  
Effective Treatment ◽  
Cavernous Sinus ◽  
Treatment Options ◽  
Cavernous Sinus Invasion ◽  
Robotic Radiosurgery ◽  
Patient Morbidity ◽  
Biochemical Remission ◽  
Patients Experience

Background: The rates of incomplete surgical resection for pituitary macroadenomas with cavernous sinus invasion are high. In growth hormone-producing adenomas, there is a considerable risk for persistent acromegaly. Thus, effective treatment options are needed to limit patient morbidity and mortality. This multicenter study assesses the efficacy and safety of robotic radiosurgery (RRS) for patients with cavernous sinus-invading adenomas with persistent acromegaly. Methods: Patients who underwent RRS with CyberKnife for postoperative acromegaly were eligible. Results: Fifty patients were included. At a median follow-up of 57 months, the local control was 100%. The pretreatment insulin-like growth factor 1 (IGF-1) levels and indexes were 381 ng/mL and 1.49, respectively. The median dose and prescription isodose were 18 Gy and 70%, respectively. Six months after RRS, and at the last follow-up, the IGF-1 levels and indexes were 277 ng/mL and 1.14, as well as 196 ng/mL and 0.83, respectively (p = 0.0001 and p = 0.0002). The IGF-1 index was a predictor for biochemical remission (p = 0.04). Nine patients achieved biochemical remission and 24 patients showed biochemical disease control. Three patients developed a new hypopituitarism. Conclusions: RRS is an effective treatment for this challenging patient population. IGF-1 levels are decreasing after treatment and most patients experience biochemical disease control or remission.

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