Haemolytic uraemic syndrome associated with pancreatitis: report of four cases and review of the literature.

Background: The incidence of acute kidney injury (AKI) in patients with acute pancreatitis ranges from 15% to 40% and is associated with poor prognosis. Haemolytic uraemic syndrome (HUS) in the setting of acute pancreatitis is an uncommon association with fewer than 30 cases reported in the literature. Methods: A retrospective review of the clinical records at our institution between January 1981 and December 2019 was carried out to identify patients with acute pancreatitis and HUS. Additionally, a literature review was conducted on this topic. The aims of the study were to describe the clinical course and outcomes of patients affected by this condition. Results: Four cases of HUS following an acute pancreatitis were identified. The mean (±SD) age of the study group was 30 ± 6 years, all of which were males. Excessive alcohol consumption was the main cause of acute pancreatitis in all four patients. HUS with progressive AKI developed in a median interval of 2 days from the onset of pancreatitis (range 1-3 days). All patients required kidney replacement therapy during the course of follow-up. A kidney biopsy was performed in two patients, showing typical thrombotic microangiopathic features. One case was treated with eculizumab, whereas the rest were treated with supportive care and/or plasma exchange. A normalization of haematological parameters and complete recovery of kidney function were observed in all patients at last follow-up, although this improvement was significantly faster in the patient treated with eculizumab. Conclusions: HUS may infrequently develop in patients with acute pancreatitis. An early identification of this complication is mandatory, and complement blockade with eculizumab may be associated with a faster kidney function recovery.

Erratum: Haemolytic uraemic syndrome associated with pancreatitis: report of four cases and review of the literature.

[This corrects the article DOI: 10.1093/ckj/sfaa245.][This corrects the article DOI: 10.1093/ckj/sfaa245.].

Gammapatías monoclonales de significado renal / Monoclonal gammopathies of renal significance

Bajo el término gammapatías monoclonales de significado renal (GMSR) se engloban un conjunto de enfermedades que se caracterizan patogénicamente por la proliferación de un clon de linfocitos B o células plasmáticas que sintetizan y segregan una inmunoglobulina monoclonal o uno de sus componentes (cadenas ligeras o pesadas), con capacidad para depositarse y producir daño a nivel glomerular, tubular, intersticial o vascular. La importancia de discriminar el término GMSR radica en poder indicar procedimientos diagnósticos y terapéuticos dirigidos al control de la síntesis y secreción de las proteínas monoclonales independientemente de los criterios clásicos vinculados con la expansión tumoral maligna. La patología renal asociada a las GMSR es muy heterogénea, lo que confiere a la biopsia renal una consideración de prueba diagnóstica clave. La correcta investigación diagnóstica de una GMSR debe incluir, además, la identificación en plasma u orina de la proteína monoclonal y un estudio hematológico completo que determine la naturaleza y extensión del clon celular. Los avances en el conocimiento de estas entidades han permitido mejorar el curso evolutivo y la supervivencia en varias formas de GMSR, aunque son necesarios más estudios y experiencia clínica para delinear protocolos terapéuticos más efectivos. En la presente revisión se resumen las principales características clínico-patológicas de las GMSR, se detalla la aproximación diagnóstica más adecuada, así como las opciones terapéuticas disponibles en el momento actual (AU)

The term monoclonal gammopathy of renal significance (MGRS) comprises a group of diseases pathogenetically characterised by proliferation of a B-cell or plasma cell clone that synthesises and secretes a monoclonal immunoglobulin or its components (light and/or heavy chains), that may deposit and cause glomerular, tubular, interstitial and/or vascular damage. The importance of differentiating the term MGRS from other monoclonal gammopathies lies in the fact that diagnostic and therapeutic procedures aimed at controlling monoclonal protein synthesis and secretion can be indicated, irrespective of the classic criteria based on malignant tumour expansion. Renal pathology associated with MGRS is highly heterogeneous, and therefore renal biopsy should be considered a key diagnostic tool. A precise diagnostic approach, however, must also identify the monoclonal protein in plasma and/or in urine, together with a complete haematological study in order to determine the nature and extension of cell clones. Recent advances in the understanding of these entities have resulted in significant improvements in clinical course and survival in several forms of MGRS, although more studies and clinical experience are needed in order to delineate more effective therapeutic strategies. In this review, we summarise the main clinical and pathological features of MGRS, highlighting the most appropriate diagnostic approach and current therapeutic options (AU)

Monoclonal gammopathies of renal significance. / Gammapatías monoclonales de significado renal.

The term monoclonal gammopathy of renal significance (MGRS) comprises a group of diseases pathogenetically characterised by proliferation of a B-cell or plasma cell clone that synthesises and secretes a monoclonal immunoglobulin or its components (light and/or heavy chains), that may deposit and cause glomerular, tubular, interstitial and/or vascular damage. The importance of differentiating the term MGRS from other monoclonal gammopathies lies in the fact that diagnostic and therapeutic procedures aimed at controlling monoclonal protein synthesis and secretion can be indicated, irrespective of the classic criteria based on malignant tumour expansion. Renal pathology associated with MGRS is highly heterogeneous, and therefore renal biopsy should be considered a key diagnostic tool. A precise diagnostic approach, however, must also identify the monoclonal protein in plasma and/or in urine, together with a complete haematological study in order to determine the nature and extension of cell clones. Recent advances in the understanding of these entities have resulted in significant improvements in clinical course and survival in several forms of MGRS, although more studies and clinical experience are needed in order to delineate more effective therapeutic strategies. In this review, we summarise the main clinical and pathological features of MGRS, highlighting the most appropriate diagnostic approach and current therapeutic options.