Beyond Loss of Kidney Function: Patient Care in Autosomal Dominant Polycystic Kidney Disease.

Patients with autosomal dominant polycystic kidney disease benefit from specialized care over their lifetimes, starting with diagnosis of the condition with ongoing discussion of both the renal course and extra-renal issues. Both renal and extra-renal issues may continue to cause major morbidity even after successful kidney transplant or initiation of RRT, and extra-renal disease aspects should always be considered as part of routine management. In this review, we will focus on updates in pain/depression screening, cardiac manifestations, liver and pancreatic cysts, kidney stone management, and genetic counseling. In some instances, we have shared our current clinical practice rather than an evidence-based guideline. We anticipate more standardization of care after the release of the Kidney Disease Improving Global Outcomes guidelines for management in autosomal dominant polycystic kidney disease later this year.

Overview of, and Preparations for, Dialysis.

Chronic kidney disease (CKD) is a progressive condition which is defined by decreased kidney function evidenced by a glomerular filtration rate (GFR) less than 60 mL/min per 1.73 m2 or markers of kidney damage, or both, for at least 3 months, regardless of the underlying cause. The 5 stages of CKD are based on the estimated GFR. Patients with CKD have significantly higher rates of morbidity, mortality, hospitalization, and health care utilization. Renal replacement therapy in the form of dialysis or kidney transplant is the life-sustaining treatment for patients with kidney failure. Predialysis education helps patients make informed decisions and opt for a modality conducive with their lifestyle/values. It has also been associated with improvement in measurable outcomes such as delayed initiation of dialysis, cardiovascular complications, and mortality.

Thrombospondin Type 1 Domain-Containing 7A (THSD7A)-Associated Membranous Nephropathy Leading to Metastatic Neuroendocrine Carcinoma.

Idiopathic membranous nephropathy also known as primary membranous nephropathy (PMN) is a common cause of nephrotic syndrome often seen in nondiabetic adults worldwide, rising as high as 40% in adults over the age of 60. Most PMN is mediated by antibodies to the M-type phospholipase A2 receptor (anti-PLA2R) in nearly 70%-80% of individuals. Thrombospondin type 1 domain-containing 7A (THSD7A) accounts for 1%-5% of individuals with PMN. In these individuals, malignancies have a varying incidence of 6%-25%. We present a case of idiopathic membranous nephropathy with anti-PLA2R negative and THSD7A positive with an underlying metastatic neuroendocrine carcinoma. Our goal is to highlight the importance of cancer screening in individuals with THSD7A-positive PMN. In addition, although nonspecific, a negative anion gap may be an indicator of an underlying malignancy.

Hypertension-Induced Thrombotic Microangiopathy Leading to End-Stage Renal Disease.

Thrombotic microangiopathy (TMA) is a term used for a group of rare and life-threatening hematological conditions. Usually, these disease processes are characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and microthrombi leading to tissue or organ injury. We present a case of a 41-year-old male with TMA induced by uncontrolled hypertension leading to end-stage renal disease requiring hemodialysis. Our goal is to highlight the importance of distinguishing hypertension-induced thrombotic microangiopathy from other etiologies of TMA, particularly thrombotic thrombocytopenic purpura (TTP), and its effect on renal function despite treatment focused on blood pressure control. Thus, it is a challenging diagnosis for clinicians to determine whether to initiate plasmapheresis for prompt treatment of suspected TTP in the setting of severe hypertension with thrombocytopenia.

Granulicatella adiacens Subacute Bacterial Endocarditis Presenting as Diffuse Alveolar Hemorrhage and Infection-Related Glomerulonephritis.

We present a case of a 69-year-old male with a past medical history of prostate cancer, chronic mitral valve regurgitation, and recent dental cleaning who presented to the hospital with shortness of breath, anemia, and acute renal failure. Due to unexplained creatinine rise, a renal biopsy was obtained which was suspicious for infection-related glomerulonephritis (IRGN). Further workup confirmed subacute endocarditis according to modified Duke's criteria. The patient's blood culture became positive for Granulicatella adiacens, a nutritionally variant streptococcus. The patient later developed acute respiratory failure from diffuse alveolar hemorrhage (DAH). Subacute infective endocarditis can result in serious morbidity and mortality due to its insidious symptoms and subsequent fatal complications.

Immunotherapy-Induced Acute Tubulointerstitial Nephritis.

Due to its minimal side-effect profile, immunotherapy has become a popular choice for the treatment of advanced melanoma as compared to conventional chemotherapy. The most common side effects associated with immunotherapy include gastrointestinal, pulmonary, and dermatologic manifestations. However, there have been very few documented occurrences of nephrotoxic side effects. We present a case of a 73-year-old male with a past medical history of chronic kidney disease (CKD) stage 3A, metastatic uveal melanoma, and gastroesophageal reflux disease on pantoprazole who arrived at the intensive care unit with altered mental status and creatinine of 27 gm/dl (baseline creatinine of 3 gm/dl about one year prior), after receiving his first dose of ipilimumab and nivolumab approximately 21 days prior. Kidney biopsy demonstrated acute tubulointerstitial nephritis (ATIN). This case highlights the importance of recognizing acute tubulointerstitial nephritis as a side effect of immunotherapy for prompt diagnosis and early treatment.