scholarly journals Extracorporeal Treatment for the Acute und Long-Term Outcome of Patients with Life-Threatening Acquired Hemophilia

2012 ◽  
Vol 39 (4) ◽  
pp. 264-270 ◽  
Author(s):  
Heike Zeitler ◽  
Gudrun Ulrich-Merzenich ◽  
Darius Panek ◽  
Georg Goldmann ◽  
Natascha Vidovic ◽  
...  
2020 ◽  
Vol 07 (04) ◽  
pp. 13-15 ◽  
Author(s):  
BS Mahapatra ◽  

Multisystem Inflammatory Syndrome in Children (MIS-C) is a newly recognized multiorgan disease seen in children, adolescent and young adults presumed to be a delayed immune mediated complication of Corona virus 2 (SARS-CoV-2) infection leading to severe acute respiratory syndrome. MIS-C can be associated with life threatening organ dysfunction requiring complex multidisciplinary care. Early recognition is important in order to prevent complication and serious sequalae. Because it is a post infective complication, in most of the cases RT-PCR comes negative though antibodies to COVID-19 are positive. Although SARS-CoV-2 in children are generally mild and nonfatal, there is increasing evidence of MIS-C. Clinical and laboratory features of MIS-C are similar to those of Kawasaki disease like syndrome and Toxic Shock Syndrome. Pathophysiology of MIS-C is still unclear and mainly due to formation of autoantibody and immune complex which activates inflammation. Most of the MIS-C associated with COVID-19, need treatment with ionotropic agents and anticoagulants. The long-term outcome of MIS-C like coronary artery aneurysm formation remain unknown and needs close follow up.


2012 ◽  
Vol 2012 ◽  
pp. 1-2 ◽  
Author(s):  
Ole D. Wolthers

Case reports of women with the rare condition of human seminal plasma allergy have indicated that the condition may be associated with life-threatening anaphylactic reactions in relation to coitus. Few observations, if any, of long-term outcome of the condition are available. The aim of this paper was to present a case diagnosed in an 18-year-old girl who presented with generalized urticaria, nasal congestion and secretion, conjunctivitis, and periorbital and labial oedema 6–8 hours after coitus. During five years of followup the condition improved clinically significantly. Due to intimacy concerns and the low prevalence of the condition robust long term data on the natural course of the condition are difficult to obtain. The present case suggests that in some patients the condition may improve over time.


Blood ◽  
2010 ◽  
Vol 115 (16) ◽  
pp. 3231-3238 ◽  
Author(s):  
Michael H. Albert ◽  
Tanja C. Bittner ◽  
Shigeaki Nonoyama ◽  
Lucia Dora Notarangelo ◽  
Siobhan Burns ◽  
...  

Abstract A large proportion of patients with mutations in the Wiskott-Aldrich syndrome (WAS) protein gene exhibit the milder phenotype termed X-linked thrombocytopenia (XLT). Whereas stem cell transplantation at an early age is the treatment of choice for patients with WAS, therapeutic options for patients with XLT are controversial. In a retrospective multicenter study we defined the clinical phenotype of XLT and determined the probability of severe disease-related complications in patients older than 2 years with documented WAS gene mutations and mild-to-moderate eczema or mild, infrequent infections. Enrolled were 173 patients (median age, 11.5 years) from 12 countries spanning 2830 patient-years. Serious bleeding episodes occurred in 13.9%, life-threatening infections in 6.9%, autoimmunity in 12.1%, and malignancy in 5.2% of patients. Overall and event-free survival probabilities were not significantly influenced by the type of mutation or intravenous immunoglobulin or antibiotic prophylaxis. Splenectomy resulted in increased risk of severe infections. This analysis of the clinical outcome and molecular basis of patients with XLT shows excellent long-term survival but also a high probability of severe disease-related complications. These observations will allow better decision making when considering treatment options for individual patients with XLT.


2020 ◽  
Vol 9 (4) ◽  
pp. 903
Author(s):  
Michael Veith ◽  
Ibrahim El-Battrawy ◽  
Gretje Roterberg ◽  
Laura Raschwitz ◽  
Siegfried Lang ◽  
...  

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited disorder causing life-threatening arrhythmias. Long-term outcome studies of the channelopathy are limited. Objective: The aim of the present study was to summarize our knowledge on CPVT patients, including the clinical profile treatment approach and long-term outcome. Methods: In this single center study, we retrospectively and prospectively collected data from nine CPVT patients and analyzed them. Results: We reviewed nine patients with CPVT in seven families (22% male), with a median follow-up time of 8.6 years. Mean age at diagnosis was 26.4 ± 12 years. Symptoms at admission were syncope (four patients) and aborted cardiac arrest (four patients). Family history of sudden cardiac death was screened in five patients. In genetic analyses, we found five patients with ryanodine type 2 receptor (RYR2) mutations. Seven patients were treated with beta-blockers, and if symptoms persisted flecainide was added (four patients). Despite beta-blocker treatment, three patients suffered from seven adverse cardiac events. An implantable cardioverter defibrillator was implanted in seven patients (one primary, six secondary prevention). Over the follow-up period, three patients suffered from ventricular tachycardia (ten times) and five patients from ventricular fibrillation (nine times). No one died during follow-up. Conclusion: Our CPVT cohort showed a high risk of cardiac events. Family screening, optimal medical therapy and individualized treatment are necessary in affected patients in referral centers.


2019 ◽  
Vol 56 (2) ◽  
pp. 405-408
Author(s):  
Cristina Tudoran ◽  
Mariana Tudoran ◽  
Tudor Ciocarlie ◽  
Catalina Giurgi-Oncu ◽  
Dana Velimirovici ◽  
...  

Aortic aneurysm (AA) and especially dissecting aneurism (DAA) represent life threatening medical conditions and vascular reconstruction surgery with the insertion of a vascular prosthetic grafts is often required to save patients life. In this paper we debate over the long term outcome of 23 patients who underwent five to eight years ago an aortic reconstruction surgery with insertion of Dacron grafts. They attended, during 2018, the emergency room of the County Emergency Hospital Pius Brinzeu of Timisoara in terms of patency complications of the prosthesis. Despite slight dilatation of the Dacron graft and of the native aorta, presence of mural thrombi and/or progress of aortic regurgitation, their evolution was satisfactory, without severe complications.


2019 ◽  
Vol 6 (4) ◽  
pp. 1120
Author(s):  
Paresh Sodhiya ◽  
Zafar Ahmed Sheikh ◽  
Mukesh Sharma ◽  
Meen Morey

Background: Raised intracranial pressure because of abnormal accumulation of CSF with in the ventricles of brain is a potentially life-threatening condition. Ventriculoperitoneal shunt remains mainstay of treatment even today. However, shunt failure is not uncommon. Hence endoscopic procedures are now gaining popularity. The outcome of ETV with respect to age ,etiology and long-term outcome needs further elucidation.Methods: Thirty patients with hydrocephalus who underwent endoscopic treatment were analysed retrospectively with respect to age, diagnosis, surgical outcome, treatment failure and complications.Results: Total of 28 patients successfully underwent ETV and with an overall success rate of 78%. Moreover, the success rate of ETV was found to be 100% in the 0-1 and 80% in 1-2 years age groups. 2 patients required re operation in the form of VP shunt.Conclusions: ETV is a safe and effective surgical modality with comparable outcome in paediatric and adult populations. 


2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
C. Hornuss ◽  
M. Firsching ◽  
M. Dolch ◽  
A. Martignoni ◽  
A. Peraud ◽  
...  

A 47-year-old man with a history of heart transplant was admitted after severe traumatic brain injury and seizures. During mechanical ventilation, the patient developed bronchospasm that severely compromised respiratory function that led to cardiac arrest. After resuscitation, application of isoflurane through the Anaesthetic Conserving Device (AnaConDa) in the ICU successfully treated bronchospasm, provided adequate sedation, and enabled appropriate ventilation and diagnostic bronchoscopy. A subsequent bronchoalveolar lavage revealed a high amount of Herpes simplex DNA. Herpes simplex pneumonia was diagnosed and treated with acyclovir. Isoflurane treatment was applied for twelve days total without side effects on renal and cerebral function. The patient recovered quickly after the termination of sedation. At discharge, he was fully awake without focal neurological deficiency and his long-term outcome was excellent. This case demonstrates that isoflurane is a treatment option in life-threatening cases of bronchospasm and a safe option for long-term sedation.


1994 ◽  
Vol 5 (3) ◽  
pp. 242-245 ◽  
Author(s):  
Debbie Gearner Thompson

Perinatal asphyxia occurs in 3–9 of every 1,000 births. The risk for perinatal asphyxia is present in every pregnancy. When asphyxia is diagnosed in a newborn, the effects on the infant are potentially life-threatening. Management of the asphyxia focuses on initial stabilization and support based on identified organ system dysfunction as well as support for the infant’s family. Long-term outcome for the asphyxiated infant is related to the degree, duration, and resolution of organ system dysfunction


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