Updated recommendations for the treatment of light-chain amyloidosis from the Swiss Amyloidosis Network.

Since the publication of the first Swiss recommendations on systemic light-chain amyloidosis in 2020, treatment strategies have evolved. As a result of the third joint meeting of the Swiss Amyloidosis Network, a multidisciplinary and multicentre Swiss clinical consortium, in 2024, recommendations for the treatment of light-chain amyloidosis were updated. They discuss the role of the new standard first-line protocol Daratumumab, Cyclophosphamide, Bortezomib, Dexamethasone (Dara-CyBorD), the timing and indication of high-dose treatment and potential second-line strategies as well as emerging treatment options, with a special focus on multidisciplinary supportive care measures.

Unanticipated Global Emergence of Multiple Mutants Selected by Mycophenolic Acid Driving Increasing Outbreaks in Solid Organ Transplant Recipients.

Background: Classified by the WHO as one of the 19 most dangerous fungal pathogens, has been associated with increasing outbreaks of pneumonia (PCP) among solid organ transplant (SOT) recipients worldwide. Mycophenolic acid (MPA), an inosine monophosphate dehydrogenase (IMPDH) inhibitor commonly used as an immunosuppressant to prevent organ rejection, is a risk factor for PCP. However, MPA also displays antifungal activity, potentially protecting against PCP, despite not being used to treat it.

[Paraneoplastic limbic encephalitis - an important differential diagnosis in cases of unclear encephalopathy].

The diagnostics of autoimmune encephalitides have made significant progress with the recent discovery of numerous tumor-associated and non-tumor-associated autoantibodies targeting neuronal and glial cells. Nevertheless, physicians continue to face major diagnostic and therapeutic challenges posed by this complex disease. This review summarizes the key aspects of the clinical presentation, diagnostics, and therapy of the most common form of autoimmune encephalitis, limbic encephalitis, with a focus on the paraneoplastic forms.

Perinephric myxoid pseudotumor of fat - histopathological and molecular characterization of 3 cases after renal transplantation.

Background: Perinephric myxoid pseudotumor of fat (PMPF) is a rare benign pseudo-neoplastic proliferation of the perinephric and renal sinus adipose tissue. Its pathogenesis is thought to be a reactive process typically associated with neoplastic and non-neoplastic end-stage kidney disease. The distinctive histopathological feature of PMPF is a myxoid process comprising bland, spindled stromal cells interspersed with mature adipose tissue.

[Albumin-corrected calcium].

In order to correctly interpret laboratory findings, it is essential to be aware of the limitations and background of laboratory values and correction formulas for everyday clinical practice. The determination of total calcium is sufficient as a screening test, while the determination of ionised calcium is the clinical gold standard. An albumin correction can lead to misinterpretations, unnecessary additional diagnostics and therapies and should be critically questioned.

mTOR inhibitors after lung transplantation: a real-life experience.

Background: The mammalian target of rapamycin (mTOR) inhibitors in combination with calcineurin inhibitors (CNIs), antimetabolites and corticosteroids for immunosuppression after lung transplantation (TPL) have gained importance in patients with chronic kidney disease (CKD). The goal of this study was to characterize lung transplant recipients (LTR) treated with mTOR inhibitors, with a special focus on kidney function.

Methods: LTR transplanted at the University Hospital Zurich between December 1992 and April 2022 were analyzed.

Historic characteristics and mortality of patients in the Swiss Amyloidosis Registry.

Aims Of The Study: Systemic amyloidoses are rare protein-folding diseases with heterogeneous, often nonspecific clinical presentations. To better understand systemic amyloidoses and to apply state-of-the-art diagnostic pathways and treatment, the interdisciplinary Amyloidosis Network was founded in 2013 at University Hospital Zurich. In this respect, a registry was implemented to study the characteristics and life expectancy of patients with amyloidosis within the area covered by the network.

Stillbirth and fulminant postpartum haemolysis: COVID-19 or leptospirosis or both?

We report the case of a female patient with a SARS-CoV-2 infection first diagnosed at 32 2/7 weeks of gestation, resulting in stillbirth at 33 5/7 weeks of gestation. Post partum the patient presented with severe and persistent haemolysis, mild thrombocytopaenia, renal insufficiency and proteinuria as well as elevated liver enzymes and jaundice. Further investigations revealed a positive IgM for and proof of infection by PCR in the urine.

Selective Bcl-2 inhibition promotes hematopoietic chimerism and allograft tolerance without myelosuppression in nonhuman primates.

Hematopoietic stem cell transplantation (HSCT) has many potential applications beyond current standard indications, including treatment of autoimmune disease, gene therapy, and transplant tolerance induction. However, severe myelosuppression and other toxicities after myeloablative conditioning regimens have hampered wider clinical use. To achieve donor hematopoietic stem cell (HSC) engraftment, it appears essential to establish niches for the donor HSCs by depleting the host HSCs.

Clinical prediction model for prognosis in kidney transplant recipients (KIDMO): study protocol.

Background: Many potential prognostic factors for predicting kidney transplantation outcomes have been identified. However, in Switzerland, no widely accepted prognostic model or risk score for transplantation outcomes is being routinely used in clinical practice yet. We aim to develop three prediction models for the prognosis of graft survival, quality of life, and graft function following transplantation in Switzerland.

Serious Games and Gamification: Health Care Workers' Experience, Attitudes, and Knowledge.

Objective: With the rapid advancement of digital technology due to COVID-19, the health care field is embracing the use of digital technologies for learning, which presents an opportunity for teaching methods such as serious games to be developed and improved. Technology offers more options for these educational approaches. The goal of this study was to assess health care workers' experiences, attitudes, and knowledge regarding serious games in training.

Mammalian Target of Rapamycin Inhibitors and Kidney Function After Thoracic Transplantation: A Systematic Review and Recommendations for Management of Lung Transplant Recipients.

Background: Chronic kidney disease (CKD) after lung transplantation is common and limits the survival of transplant recipients. The calcineurin inhibitors (CNI), cyclosporine A, and tacrolimus being the cornerstone of immunosuppression are key mediators of nephrotoxicity. The mammalian target of rapamycin (mTOR) inhibitors, sirolimus and everolimus, are increasingly used in combination with reduced CNI dosage after lung transplantation.

Peripartum cardiomyopathy: a rare cause of acute heart failure.

A woman in her early 30s presented herself with acute dyspnoea and elevated D-dimers 5 weeks after delivery of her second child. Echocardiographic findings showed signs of acute left ventricular failure, and an MRI confirmed a non-ischaemic dilated left heart failure compatible with peripartum cardiomyopathy. The antihormonal therapy with bromocriptine during 6 weeks and an intensive heart failure therapy led to an amelioration of the heart function within 3 years, but full recovery was not yet observed.

[CME: Diagnostic Approach and Management of Thrombotic Microangiopathy].

CME: Diagnostic Approach and Management of Thrombotic Microangiopathy Thrombotic microangiopathies (TMA) are characterized by organ thrombosis induced by endothelial injury. They present with thrombocytopenia, hemolytic anemia and schistocytes. In case of an underlying disease-causing TMA, the treatment of the underlying disease is essential.

First Testing of Literature-Based Models for Predicting Increase in Body Weight and Adipose Tissue Mass After Kidney Transplantation.

Weight gain is a risk factor for poor clinical outcomes following kidney transplantation. This study's aim was a first testing of 2 models to identify patients early after kidney transplantation who are at risk for weight gain and increase in adipose tissue mass in the first year after kidney transplantation. The literature-based models were evaluated on longitudinal data of 88, respectively 79 kidney transplant recipients via ordinary and Firth regression, using gains ≥ 5% in weight and adipose tissue mass respectively as primary and secondary endpoints.

Impact of the COVID-19 pandemic on emergency outpatient consultations and admissions of non-COVID-19 patients (ECCO)-A cross-sectional study.

During the first year of the COVID-19 pandemic, healthcare facilities worldwide struggled to adequately care for the increasing number of COVID-19 patients while maintaining quality of care for all other patients. The aim of this study was to investigate the displacement and underuse of non-COVID-19 patient care in a medical department of a tertiary hospital in Switzerland. In this retrospective cross-sectional study, internal medicine admissions from 2017 to 2020, emergency outpatient visits from 2019 to 2020 and COVID-19 admissions in 2020 were analyzed and compared using a regression model.

Successful Induction of Specific Immunological Tolerance by Combined Kidney and Hematopoietic Stem Cell Transplantation in HLA-Identical Siblings.

Induction of immunological tolerance has been the holy grail of transplantation immunology for decades. The only successful approach to achieve it in patients has been a combined kidney and hematopoietic stem cell transplantation from an HLA-matched or -mismatched living donor. Here, we report the first three patients in Europe included in a clinical trial aiming at the induction of tolerance by mixed lymphohematopoietic chimerism after kidney transplantation.

Management of transthyretin amyloidosis.

Transthyretin amyloidosis (ATTR amyloidosis) is a disease caused by deposition of transthyretin fibrils in organs and tissues, which causes their dysfunction. The clinical heterogeneity of ATTR amyloidosis and the variable presentation of symptoms at early disease stages, historically meant treatment delays. Diagnostic tools and therapy options of ATTR amyloidosis have markedly improved in recent years.

[CME/Answers: Giant Cell Arteritis].

CME/Answers: Giant Cell Arteritis Giant cell arteritis (GCA) is the most common vasculitis among patients over the age of 50. Mainly large vessels are targeted. GCA can be differentiated into cranial and extra-cranial types; thus the symptoms can range from headache, blurred vision and jaw claudication to non-specific symptoms like fatigue, polymyalgia and fever.

[CME/Answers: Musculoskeletal Manifestations of Lyme Disease].

Lyme disease is the most common vector-borne illness in Switzerland and Lyme arthritis the most prevalent manifestation of late-stage Lyme disease. It presents as a monoarthitis or oligoarthritis in large joints, often involving the knee. Such a clinical presentation, together with positive Lyme serologies or polymerase chain reactions from synovial fluid/tissue, is considered diagnostic.

[CME: Giant Cell Arteritis].

CME: Giant Cell Arteritis Giant cell arteritis (GCA) is the most common vasculitis among patients over the age of 50. Mainly large vessels are targeted. GCA can be differentiated into cranial and extra-cranial types; thus the symptoms can range from headache, blurred vision and jaw claudication to non-specific symptoms like fatigue, polymyalgia and fever.