Date of Presentation
5-2-2019 12:00 AM
College
School of Osteopathic Medicine
Poster Abstract
Renal failure, both acute and chronic, can present from many different etiologies and if diagnosed with diabetes mellitus, it is commonly assumed to be due to diabetic nephropathy. Monoclonal gammopathy of renal significance (MGRS) is a disorder that combines monoclonal gammopathy of undetermined significance with end stage renal disease. Monoclonal immunoglobulins are secreted by B or plasma cells leading to deposition in the kidney causing end organ damage. Several different subtypes fall under this category of renal diseases including, but not limited to, amyloidosis, C3 glomeruopathy, and light chain proximal tubulopathy, or better known as Fanconi syndrome1. Fanconi syndrome, which can be acquired or inherited, occurs due to toxicity of the proximal renal tubule in the kidney which leads to hypophosphatemia, hypokalemia, metabolic acidosis due to impaired bicarbonate absorption, impaired glucose and uric acid absorption, and proteinuria.
In this case, an 84 year old Caucasian male presented with shortness of breath and weakness. He denied chest pain, hemoptysis, polyuria, and hematuria. His past medical history includes chronic renal disease secondary to well-controlled type II diabetes mellitus (DM), perirenal lymphangiectasis, pulmonary fibrosis, hypertension, atrial fibrillation, hyperlipidemia, and anemia. He had a left radiocephalic AV fistula Permacath placement prior to admission for dialysis. A combined effort from general internal medicine, nephrology, hematology/oncology, cardiology, pulmonary, vascular, infectious disease, radiology, and pathology was made to investigate the patient’s illness over the course of his one-month stay.
Though type II DM played a role in the development of this patient’s chronic kidney disease, MGRS is a significant causal factor of the progression to end stage renal disease. Currently, treatment with chemotherapy that targets plasma or B cells can be used for MGRS. Several case studies reported improvement or stabilization of renal function in those treated with chemotherapeutic agents vs. no therapy. (4,5) Though renal transplantation can be done, recurrence post-transplantation is common. (6) The main goal in managing MGRS with Fanconi syndrome is slowing the progression of renal disease and preventing associated extra-renal complications. (7)
This case demonstrates the complexities of medical diagnostics and the step-wise progression involved in caring for a patient. It displays the value of interdisciplinary collaboration among different medical specialties to accurately provide patients with an etiology and treatment for their illness and related symptoms.
Keywords
monoclonal gammopathy of renal significance, MGRS, renal failure, Fanconi syndrome, interdisciplinary collaboration, collaborative care
Disciplines
Integrative Medicine | Medicine and Health Sciences | Nephrology | Pathological Conditions, Signs and Symptoms | Urology
Document Type
Poster
Included in
Integrative Medicine Commons, Nephrology Commons, Pathological Conditions, Signs and Symptoms Commons, Urology Commons
Chronic Renal Failure, But Why? A Case Report.
Renal failure, both acute and chronic, can present from many different etiologies and if diagnosed with diabetes mellitus, it is commonly assumed to be due to diabetic nephropathy. Monoclonal gammopathy of renal significance (MGRS) is a disorder that combines monoclonal gammopathy of undetermined significance with end stage renal disease. Monoclonal immunoglobulins are secreted by B or plasma cells leading to deposition in the kidney causing end organ damage. Several different subtypes fall under this category of renal diseases including, but not limited to, amyloidosis, C3 glomeruopathy, and light chain proximal tubulopathy, or better known as Fanconi syndrome1. Fanconi syndrome, which can be acquired or inherited, occurs due to toxicity of the proximal renal tubule in the kidney which leads to hypophosphatemia, hypokalemia, metabolic acidosis due to impaired bicarbonate absorption, impaired glucose and uric acid absorption, and proteinuria.
In this case, an 84 year old Caucasian male presented with shortness of breath and weakness. He denied chest pain, hemoptysis, polyuria, and hematuria. His past medical history includes chronic renal disease secondary to well-controlled type II diabetes mellitus (DM), perirenal lymphangiectasis, pulmonary fibrosis, hypertension, atrial fibrillation, hyperlipidemia, and anemia. He had a left radiocephalic AV fistula Permacath placement prior to admission for dialysis. A combined effort from general internal medicine, nephrology, hematology/oncology, cardiology, pulmonary, vascular, infectious disease, radiology, and pathology was made to investigate the patient’s illness over the course of his one-month stay.
Though type II DM played a role in the development of this patient’s chronic kidney disease, MGRS is a significant causal factor of the progression to end stage renal disease. Currently, treatment with chemotherapy that targets plasma or B cells can be used for MGRS. Several case studies reported improvement or stabilization of renal function in those treated with chemotherapeutic agents vs. no therapy. (4,5) Though renal transplantation can be done, recurrence post-transplantation is common. (6) The main goal in managing MGRS with Fanconi syndrome is slowing the progression of renal disease and preventing associated extra-renal complications. (7)
This case demonstrates the complexities of medical diagnostics and the step-wise progression involved in caring for a patient. It displays the value of interdisciplinary collaboration among different medical specialties to accurately provide patients with an etiology and treatment for their illness and related symptoms.