MO107CLINICAL CHARACTERISTICS OF A PATIENT POPULATION WITH ATYPICAL HAEMOLYTIC URAEMIC SYNDROME AND MALIGNANT HYPERTENSION: THE GLOBAL AHUS REGISTRY ANALYSIS

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Jean-Michel Halimi ◽  
Imad Al-Dakkak ◽  
Katerina Anokhina ◽  
Gianluigi Ardissino ◽  
Christoph Licht ◽  
...  
Keyword(s):  
Kidney Transplant ◽  
Haemolytic Uraemic Syndrome ◽  
Clinical Characteristics ◽  
Organ Damage ◽  
Malignant Hypertension ◽  
Symptom Presentation ◽  
Pathogenic Variants ◽  
Atypical Haemolytic Uraemic Syndrome ◽  
Before And After ◽  
History Of

Abstract Background and Aims Atypical haemolytic uraemic syndrome (aHUS) is a rare disease that manifests as complement-mediated thrombotic microangiopathy (TMA), which can lead to severe organ damage. Some patients with aHUS may present with malignant hypertension (MHT); both conditions can result in TMA. The objective of this analysis was to characterise patients with aHUS and MHT. Method In this analysis, patients from the Global aHUS Registry (NCT01522183) were included if they were diagnosed with MHT and were followed ≥90 days after initial aHUS symptom presentation or diagnosis date; patients were excluded if they withdrew from the registry or discontinued treatment with eculizumab due to a diagnosis other than aHUS. Demographics and clinical characteristics were evaluated. Results Seventy-one of 1903 registry patients were included in the analysis. Clinical characteristics are presented in the table. Seventeen patients (24%) had a paediatric (<18 years) onset of disease, and 54 (76%) were adults at aHUS diagnosis; female patients were slightly overrepresented (61%). Sixty-nine percent of patients were reported to have MHT at around the same time as aHUS diagnosis (+/-2 months), while 11% and 13% experienced MHT before and after aHUS diagnosis, respectively. aHUS triggering conditions were reported in 6/71 patients (8%) (Table). Cardiovascular (27%) and gastrointestinal (21%) symptoms were the most commonly reported extra-renal manifestations. Eight patients (11%) had a reported family history of aHUS and 40 patients (56%) had a complement pathogenic variant or an anti-CFH-antibody. Thirty-three patients (46%) had a kidney transplant; of these, 20 were prescribed eculizumab in the peri- or post-transplant period. Conclusion In this analysis of patients with aHUS and MHT, the observed high prevalence of pathogenic variants in complement genes or anti-CFH antibodies, alongside the high proportion of patients with extrarenal manifestations and/or requiring kidney transplant, indicate a high severity of presentation and poor prognosis of aHUS associated with MHT.

scholarly journals Successful Subsequent Pregnancy in a Woman Receiving Eculizumab for Pregnancy-Associated Atypical Haemolytic Uraemic Syndrome

2019 ◽  
Vol 2019 ◽  
pp. 1-4 ◽  
Author(s):  
Samantha Bateman ◽  
Maleeka Ladhani ◽  
Shilpanjali Jesudason
Keyword(s):  
Renal Function ◽  
Haemolytic Uraemic Syndrome ◽  
Rescue Therapy ◽  
Significant Risk ◽  
First Trimester ◽  
Subsequent Pregnancy ◽  
Baby Girl ◽  
Successful Pregnancy ◽  
Atypical Haemolytic Uraemic Syndrome ◽  
History Of

Atypical haemolytic uraemic syndrome (aHUS) is a form of thrombotic microangiopathy precipitated by unopposed complement activation, the treatment of which has been revolutionised by the availability of the monoclonal anti-complement (C5) antibody, eculizumab. Historically, women with aHUS would be unable to achieve a successful pregnancy due to the severity of their renal disease and for the few who could conceive, recurrence of aHUS was a significant risk. In spite of this, parenthood remains a priority for many. Experience with eculizumab use in the management of aHUS during pregnancy is growing and with it comes a significant change in the course of the disease. We present the case of a 28-year-old woman diagnosed with severe aHUS in the first trimester of her first pregnancy. She received rescue therapy with eculizumab and had a return to normal renal function. While this pregnancy was lost, she strongly desired a family. We managed her through a subsequent pregnancy while receiving eculizumab. This pregnancy was uncomplicated and carried to term and she birthed a healthy 2500 g baby girl. The complexities of managing a pregnancy in a woman with a history of aHUS are vast but not insurmountable, as demonstrated by this case.

scholarly journals Successful kidney transplant with eculizumab, thymoglobulin and belatacept therapy in a highly sensitised patient with atypical haemolytic uraemic syndrome due to factor H mutation

2018 ◽  
Vol 38 (4) ◽  
pp. 433-437
Author(s):  
John Fredy Nieto-Ríos ◽  
Mónica Zuluaga-Quintero ◽  
Diana Carolina Bello-Márquez ◽  
Arbey Aristizabal-Alzate ◽  
Catalina Ocampo-Kohn ◽  
...  
Keyword(s):  
Kidney Transplant ◽  
Haemolytic Uraemic Syndrome ◽  
Factor H ◽  
Atypical Haemolytic Uraemic Syndrome ◽  
Factor H Mutation

Carfilzomib-induced atypical haemolytic uraemic syndrome: a diagnostic challenge and therapeutic success

2021 ◽  
Vol 14 (2) ◽  
pp. e239091
Author(s):  
Alicia Darwin ◽  
Leonger Malpica ◽  
Jugraj Dhanoa ◽  
Hamza Hashmi
Keyword(s):  
Multiple Myeloma ◽  
Plasma Exchange ◽  
Haemolytic Anaemia ◽  
Haemolytic Uraemic Syndrome ◽  
Complement Protein ◽  
Diagnostic Challenge ◽  
Drug Induced ◽  
Therapeutic Success ◽  
Atypical Haemolytic Uraemic Syndrome ◽  
History Of

Haemolytic uraemic syndrome (HUS) is a thrombotic microangiopathy (TMA) that presents with renal insufficiency, thrombocytopaenia and microangiopathic haemolytic anaemia. Typical HUS is associated with Shiga toxin while atypical HUS (aHUS) is due to overactivation of the alternative complement pathway. aHUS has numerous causes, including drugs, with rare reports of carfilzomib, a proteasome inhibitor used in multiple myeloma, as causative agent. Cases vary in presentation, presenting a diagnostic challenge. Historically, TMAs were treated with plasma exchange. aHUS, however, is considered refractory to plasma exchange and best treated with eculizumab, a monoclonal antibody targeting C5, a terminal complement protein. We report a patient with history of multiple myeloma who presented with headaches, elevated blood pressure, petechiae, ecchymosis and haemolytic anaemia. His condition was determined to be carfilzomib-induced aHUS and he was successfully treated with eculizumab. Early detection and treatment of drug-induced aHUS is vital in reducing morbidity and mortality related to the condition.

scholarly journals Pregnancy-associated atypical haemolytic uraemic syndrome in the postpartum period: a case report and review of the literature

2011 ◽  
Vol 4 (2) ◽  
pp. 83-85 ◽  
Author(s):  
M Egbor ◽  
A Johnson ◽  
F Harris ◽  
D Makanjoula ◽  
H Shehata
Keyword(s):  
Case Report ◽  
Medical History ◽  
Postpartum Period ◽  
Haemolytic Uraemic Syndrome ◽  
Review Of The Literature ◽  
Atypical Haemolytic Uraemic Syndrome ◽  
History Of ◽  
Previous Medical History

Pregnancy has been reported to be a trigger in about 10% of all patients with atypical haemolytic uraemic syndrome (aHUS). However, in contrast to pregnancy-associated thrombotic thrombocytopaenic purpura, the presentation of pregnancy-associated aHUS remains ill defined and can therefore be difficult to diagnose and manage appropriately. Here we report a case of pregnancy-associated relapse of aHUS in a patient with a previous medical history of aHUS prior to pregnancy.

scholarly journals Primary Hyperaldosteronism: A Rare Cause of Malignant Hypertension with Thrombotic Microangiopathy in a Kidney Transplant Recipient

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Carolina Ormonde ◽  
Sara Querido ◽  
Nuno Rombo ◽  
Rita Roque ◽  
Belarmino Clemente ◽  
...  
Keyword(s):  
Transplant Recipient ◽  
Kidney Transplant ◽  
Thrombotic Microangiopathy ◽  
Viral Infections ◽  
Kidney Transplant Recipient ◽  
Calcineurin Inhibitors ◽  
Organ Damage ◽  
Primary Hyperaldosteronism ◽  
Malignant Hypertension ◽  
Antibody Mediated Rejection

Thrombotic microangiopathy (TMA) is a rare disease that presents with haemolysis and organ damage. The kidney is one of the main affected organs, and TMA is associated with serious complications and increased mortality. In transplanted patients, TMA is even less common and has a variety of possible causes, including thrombotic thrombocytopenic purpura (TTP) and haemolytic-uremic syndrome (HUS), infections, drugs, autoimmune disease, tumours, and malignant hypertension. Transplant-related causes, such as antibody-mediated rejection, calcineurin inhibitors, and viral infections, need to be considered as well. The authors report a rare case of TMA in a kidney transplant recipient, whose investigation revealed malignant hypertension secondary to primary hyperaldosteronism.

Study of Diabetic Mellitus and Knowledge of Lotion Foot Care on the Community

2018 ◽  
Vol 9 (3) ◽  
Author(s):  
Hariyadi DM ◽  
Athiyah U ◽  
Hendradi E ◽  
Rosita N ◽  
Erawati T ◽  
...  
Keyword(s):  
Blood Glucose ◽  
T Test ◽  
Small Scale ◽  
Scale Production ◽  
Foot Care ◽  
Diabetic Mellitus ◽  
Glucose Levels ◽  
Blood Glucose Levels ◽  
Before And After ◽  
History Of

The prevention of Diabetic Mellitus (DM) and its complications is the main aim of this study, in addition to the training of lotion foot care application and the development of small scale industry. The research team delivered knowledge in the form of training on Diabetic Mellitus, healthy food, treatment and prevention of complications, and small-scale production of cosmetic products. The aim of this study was to determine the correlation between training on diabetic and lotion foot care application as preventive measures against diabetic complications on the patient's blood glucose levels in the community of residents in Banyuurip Jaya, Surabaya. It was expected from this training that the knowledge of the residents increases and people living with diabetic undergo lifestyle changes and therefore blood sugar levels can be controlled. The parameters measured in this research were blood glucose levels, the anti diabetic drug types consumed, and compliance on diabetics. This study used the data taken from 60 patients with DM over a period of one month. Questionnaires and log books was used to retrieve data and changes in blood glucose levels in diabetic patients. The results showed the demographic data of patients with type 2 diabetic of 85% female and 15% male, with the range of patients aged of 61-70 years of 46.67% and had history of diabetic (90%). The history of drugs consumed by respondents was anti diabetic drugs such as metformin (40%), glimepiride (33.37%) and insulin (6.67%). In addition, the increased knowledge of DM patients after being given the training compared to before training was shown in several questions in the questionnaire. A statistical analysis using t-test analyzed a correlation between training provided in order to enhance understanding of the patient, as well as correlation with blood glucose levels. A paired T-test showed that there was a relationship between the knowledge of trainees before and after training (p less than 0.05). An interesting result was that there was no relationship between blood glucose levels before and after training provided (p> 0.05).

The Challenges in Dealing with Organized Crime and its Consequences in Modern Societies in Albania, Kosovo and Macedonia – the Balkan Peninsula

2015 ◽  
Vol 5 (1) ◽  
pp. 161
Author(s):  
Bashkim Selmani ◽  
Bekim Maksuti
Keyword(s):  
Organized Crime ◽  
Balkan Peninsula ◽  
Human Mind ◽  
Negative Consequences ◽  
The Public ◽  
Before And After ◽  
History Of ◽  
European Standards ◽  
Organized Crime Groups ◽  
Further Development

The profound changes within the Albanian society, including Albania, Kosovo and Macedonia, before and after they proclaimed independence (in exception of Albania), with the establishment of the parliamentary system resulted in mass spread social negative consequences such as crime, drugs, prostitution, child beggars on the street etc. As a result of these occurred circumstances emerged a substantial need for changes within the legal system in order to meet and achieve the European standards or behaviors and the need for adoption of many laws imported from abroad, but without actually reading the factual situation of the psycho-economic position of the citizens and the consequences of the peoples’ occupations without proper compensation, as a remedy for the victims of war or peace in these countries. The sad truth is that the perpetrators not only weren’t sanctioned, but these regions remained an untouched haven for further development of criminal activities, be it from the public state officials through property privatization or in the private field. The organized crime groups, almost in all cases, are perceived by the human mind as “Mafia” and it is a fact that this cannot be denied easily. The widely spread term “Mafia” is mostly known around the world to define criminal organizations.The Balkan Peninsula is highly involved in these illegal groups of organized crime whose practice of criminal activities is largely extended through the Balkan countries such as Kosovo, Albania, Macedonia, Serbia, Bosnia, Croatia, Montenegro, etc. Many factors contributed to these strategic countries to be part of these types of activities. In general, some of the countries have been affected more specifically, but in all of the abovementioned countries organized crime has affected all areas of life, leaving a black mark in the history of these states.

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