Multiple extremity necrosis in fatal calciphylaxis: Case report. 2021

Diego Ennes Gonzalez, and Renato Demarchi Foresto, and Ana Luiza Santos Maldonado, and Wallace Stwart Carvalho Padilha, and Fernanda Badiani Roberto, and Maria Eduarda Vilanova da Costa Pereira, and Marcelino de Souza Durão Junior, and Aluizio Barbosa Carvalho
Universidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM), Hospital do Rim, São Paulo, SP, Brasil.

BACKGROUND The clinical impact of vascular calcification is well established in the context of cardiovascular morbidity and mortality, but other clinical syndromes, such as calciphylaxis, although less frequent, have a significant impact on chronic kidney disease. METHODS Case report of a 27-year-old woman, who had complained of bilateral pain in her toes for 3 days, with the presence of small necrotic areas in the referred sites. She had a history of type 1 diabetes (25 years ago), with chronic kidney disease, on peritoneal dialysis, in addition to rheumatoid arthritis. She was admitted to the hospital, which preceded the current condition, due to exacerbation of rheumatoid arthritis, evolving with intracardiac thrombus due to venous catheter complications, when she started using warfarin. Ischemia progressed to her feet, causing the need for bilateral amputations. Her chirodactyls were also affected. Thrombophilia, vasculitis, endocarditis or other embolic sources were investigated and discarded. Her pathology report evidenced skin necrosis and superficial soft parts with recent arterial thrombosis, and Monckeberg's medial calcification. We started treatment with bisphosphonate and sodium thiosulfate, conversion to hemodialysis and replacement of warfarin with unfractionated heparin. Despite all the therapy, the patient died after four months of evolution. CONCLUSIONS Calciphylaxis is a rare microvasculature calcification syndrome that results in severe ischemic injuries. It has pathogenesis related to the mineral and bone disorder of chronic kidney disease combined with the imbalance between promoters and inhibitors of vascular calcification, with particular importance to vitamin K antagonism. CONCLUSIONS The preventive strategy is fundamental, since the therapy is complex with poorly validated effectiveness.

UI MeSH Term Description Entries
D007676 Kidney Failure, Chronic The end-stage of CHRONIC RENAL INSUFFICIENCY. It is characterized by the severe irreversible kidney damage (as measured by the level of PROTEINURIA) and the reduction in GLOMERULAR FILTRATION RATE to less than 15 ml per min (Kidney Foundation: Kidney Disease Outcome Quality Initiative, 2002). These patients generally require HEMODIALYSIS or KIDNEY TRANSPLANTATION. ESRD,End-Stage Renal Disease,Renal Disease, End-Stage,Renal Failure, Chronic,Renal Failure, End-Stage,Chronic Kidney Failure,End-Stage Kidney Disease,Chronic Renal Failure,Disease, End-Stage Kidney,Disease, End-Stage Renal,End Stage Kidney Disease,End Stage Renal Disease,End-Stage Renal Failure,Kidney Disease, End-Stage,Renal Disease, End Stage,Renal Failure, End Stage
D009336 Necrosis The death of cells in an organ or tissue due to disease, injury or failure of the blood supply.
D002115 Calciphylaxis Condition of induced systemic hypersensitivity in which tissues respond to appropriate challenging agents with a sudden local calcification. Calcific Uraemic Arteriolopathy,Calciphylaxes,Idiopathic Calciphylaxis,Arteriolopathy, Calcific Uraemic,Calcific Uraemic Arteriolopathies,Calciphylaxis, Idiopathic,Uraemic Arteriolopathy, Calcific
D005121 Extremities The farthest or outermost projections of the body, such as the HAND and FOOT. Limbs,Extremity,Limb
D005260 Female Females
D006435 Renal Dialysis Therapy for the insufficient cleansing of the BLOOD by the kidneys based on dialysis and including hemodialysis, PERITONEAL DIALYSIS, and HEMODIAFILTRATION. Dialysis, Extracorporeal,Dialysis, Renal,Extracorporeal Dialysis,Hemodialysis,Dialyses, Extracorporeal,Dialyses, Renal,Extracorporeal Dialyses,Hemodialyses,Renal Dialyses
D006493 Heparin A highly acidic mucopolysaccharide formed of equal parts of sulfated D-glucosamine and D-glucuronic acid with sulfaminic bridges. The molecular weight ranges from six to twenty thousand. Heparin occurs in and is obtained from liver, lung, mast cells, etc., of vertebrates. Its function is unknown, but it is used to prevent blood clotting in vivo and vitro, in the form of many different salts. Heparinic Acid,alpha-Heparin,Heparin Sodium,Liquaemin,Sodium Heparin,Unfractionated Heparin,Heparin, Sodium,Heparin, Unfractionated,alpha Heparin
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

Related Publications

Diego Ennes Gonzalez, and Renato Demarchi Foresto, and Ana Luiza Santos Maldonado, and Wallace Stwart Carvalho Padilha, and Fernanda Badiani Roberto, and Maria Eduarda Vilanova da Costa Pereira, and Marcelino de Souza Durão Junior, and Aluizio Barbosa Carvalho
February 1997, Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons,
Diego Ennes Gonzalez, and Renato Demarchi Foresto, and Ana Luiza Santos Maldonado, and Wallace Stwart Carvalho Padilha, and Fernanda Badiani Roberto, and Maria Eduarda Vilanova da Costa Pereira, and Marcelino de Souza Durão Junior, and Aluizio Barbosa Carvalho
January 2022, Journal of investigative medicine high impact case reports,
Diego Ennes Gonzalez, and Renato Demarchi Foresto, and Ana Luiza Santos Maldonado, and Wallace Stwart Carvalho Padilha, and Fernanda Badiani Roberto, and Maria Eduarda Vilanova da Costa Pereira, and Marcelino de Souza Durão Junior, and Aluizio Barbosa Carvalho
July 2018, The American journal of case reports,
Diego Ennes Gonzalez, and Renato Demarchi Foresto, and Ana Luiza Santos Maldonado, and Wallace Stwart Carvalho Padilha, and Fernanda Badiani Roberto, and Maria Eduarda Vilanova da Costa Pereira, and Marcelino de Souza Durão Junior, and Aluizio Barbosa Carvalho
June 2020, Medical archives (Sarajevo, Bosnia and Herzegovina),
Diego Ennes Gonzalez, and Renato Demarchi Foresto, and Ana Luiza Santos Maldonado, and Wallace Stwart Carvalho Padilha, and Fernanda Badiani Roberto, and Maria Eduarda Vilanova da Costa Pereira, and Marcelino de Souza Durão Junior, and Aluizio Barbosa Carvalho
September 2023, Urology case reports,
Diego Ennes Gonzalez, and Renato Demarchi Foresto, and Ana Luiza Santos Maldonado, and Wallace Stwart Carvalho Padilha, and Fernanda Badiani Roberto, and Maria Eduarda Vilanova da Costa Pereira, and Marcelino de Souza Durão Junior, and Aluizio Barbosa Carvalho
October 2023, Medicine,
Diego Ennes Gonzalez, and Renato Demarchi Foresto, and Ana Luiza Santos Maldonado, and Wallace Stwart Carvalho Padilha, and Fernanda Badiani Roberto, and Maria Eduarda Vilanova da Costa Pereira, and Marcelino de Souza Durão Junior, and Aluizio Barbosa Carvalho
January 2010, Urologia,
Diego Ennes Gonzalez, and Renato Demarchi Foresto, and Ana Luiza Santos Maldonado, and Wallace Stwart Carvalho Padilha, and Fernanda Badiani Roberto, and Maria Eduarda Vilanova da Costa Pereira, and Marcelino de Souza Durão Junior, and Aluizio Barbosa Carvalho
January 2008, Dermatologic therapy,
Diego Ennes Gonzalez, and Renato Demarchi Foresto, and Ana Luiza Santos Maldonado, and Wallace Stwart Carvalho Padilha, and Fernanda Badiani Roberto, and Maria Eduarda Vilanova da Costa Pereira, and Marcelino de Souza Durão Junior, and Aluizio Barbosa Carvalho
January 2013, Anais brasileiros de dermatologia,
Diego Ennes Gonzalez, and Renato Demarchi Foresto, and Ana Luiza Santos Maldonado, and Wallace Stwart Carvalho Padilha, and Fernanda Badiani Roberto, and Maria Eduarda Vilanova da Costa Pereira, and Marcelino de Souza Durão Junior, and Aluizio Barbosa Carvalho
March 2023, Cureus,
Copied contents to your clipboard!