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Clinical Nephrology. Case Studies

Hisako Hirashima, Toshiyuki Komiya, Naoya Toriu, Shigeo Hara, Akira Matsunaga, Takao Saito, Eri Muso
A 79-year-old man was admitted to our hospital for proteinuria due to nephrotic syndrome. Renal biopsy revealed focal sclerosis and foam cell infiltration in the glomerulus. In addition, electron microscopic findings (EM) revealed peculiar electron-dense deposits (EDDs) in both sides of the glomerular basement membrane. Although subepithelial deposits had spike formation highly resembling those seen in membranous nephropathy (MN), immunoglobulins and complements were not identified by immunofluorescence study, and microbubbles appeared in high magnification of EM different from the immune disease...
2018: Clinical Nephrology. Case Studies
Laurence Gariépy-Assal, Rodney D Gilbert, Aleksas Žiaugra, Bethany Joy Foster
Denys-Drash syndrome (DDS), a condition caused by mutations in the tumor-suppressor gene WT-1, is associated with a triad of disorders: ambiguous genitalia, nephrotic syndrome leading to end-stage renal disease (ESRD), and Wilms' tumor. Given the variable disease course, management is challenging. We aimed to describe the evolution of DDS and the range of management strategies by summarizing the clinical courses of cases collected from a questionnaire sent to the international pediatric nephrology community...
2018: Clinical Nephrology. Case Studies
Michele Mirabella, Lucia Taramasso, Laura Ambra Nicolini, Rodolfo Russo, Claudio Viscoli, Antonio Di Biagio
In this report, we describe the coexistence of two rare and debated complications of hepatitis C virus (HCV) infection: interstitial nephritis, with associated focal glomerulosclerosis, and autoimmune hepatitis, in a 55-year-old HIV/HCV-coinfected woman. The patient was treated for the immune-mediated manifestations with mycophenolate mofetil, which she continued for 9 years, as symptoms relapsed at every attempt to discontinue immunosuppression. The patient finally cleared HCV infection thanks to new direct-acting agents and could discontinue immunosuppressive therapy maintaining stable conditions and laboratory parameters after 24-weeks follow-up...
2018: Clinical Nephrology. Case Studies
Masato Sawamura, Atsushi Komatsuda, Mizuho Nara, Masaru Togashi, Hideki Wakui, Naoto Takahashi
A 32-year-old woman presented with amenorrhea after a normal childbirth and subsequently developed nephrotic syndrome. Renal biopsy showed a thrombotic microangiopathy (TMA)-like glomerular lesion with deposits of immunoglobulins, complements, and fibrinogen. Increased serum levels of the beta subunit of human chorionic gonadotropin, abnormal uterine findings from imaging studies, and endometrial biopsy findings suggested gestational trophoblastic disease. She was diagnosed with a placental site trophoblastic tumor (PSTT) after hysterectomy and, following treatment, her proteinuria disappeared...
2018: Clinical nephrology. Case studies
Ramy M Hanna, Marian Kaldas, Farid Arman, Melissa Wang, Terrance Hammer, Deren Sinkowitz, Anjay Rastogi
. Sarcoidosis is a rare autoimmune disease resulting in formation of non-necrotizing "non-caseating" granulomas generally in the lung. The disease classically strikes African American females in their fourth and fifth decades. The resulting hypercalcemia is a result of 1-α hydroxylase overexpression in granulomas with increased 1,25-dihydroxy vitamin D levels. This phenomenon can also be observed in mycobacterial and fungal infections that produce granulomas in infected patients. Thus, chronic infectious diseases are part of differential diagnosis of granulomatous processes...
2018: Clinical Nephrology. Case Studies
Yuhei Noda, Yosuke Saka, Akihisa Kato, Tetsushi Mimura, Tomohiko Naruse
Thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly constitute TAFRO syndrome, a variant of Castleman disease. We describe a patient with TAFRO syndrome who underwent renal biopsy. A 79-year-old woman was referred to us with fever and leg edema. She also had thrombocytopenia, pleural effusion, ascites, and acute kidney injury, and was admitted to our hospital. Her response to initial therapy with corticosteroid and cyclosporine was poor. Therefore, she received 4 doses of rituximab per week, which resulted in clinical improvement, including recovery of thrombocytopenia...
2018: Clinical nephrology. Case studies
Haili Wang, Haiyu Zhan, Zhigang Yao, Qingwei Liu
Malignant renal epithelioid angiomyolipoma (EAML) is an extremely rare disease with a poor prognosis, and currently there are no uniform criteria for its biological behavior. Here, we present a case of malignant renal EAML with TFE3 gene amplification in a 53-year-old woman. Four months after surgery, unenhanced computed tomography scans showed recurrence as well as metastasis in the abdomen and lung. The patient succumbed to rapid neoplastic progression of the disease 6 months later.
2018: Clinical Nephrology. Case Studies
Mohammad Almeqdadi, Mohammed Al-Dulaimi, Aleksandr Perepletchikov, Kevin Tomera, Bertrand L Jaber
Retroperitoneal fibrosis (RPF) is a progressive fibroinflammatory disease that can be complicated by urinary obstruction. RPF can be the only manifestation of IgG4-related disease (IgG4-RD). Treatment of IgG4-related RPF is challenging and mostly consists of long-term glucocorticoids leading to significant side effects and treatment intolerance. Recent exploration of the role of rituximab as a B-cell depleting therapy in the treatment of IgG4-RD provides therapeutic potential as a well-tolerated alternative to glucocorticoids...
2018: Clinical Nephrology. Case Studies
Kenneth Lim, David Steele, Andrew Fenves, Ravi Thadhani, Eliot Heher, Amel Karaa
Not available.
2018: Clinical Nephrology. Case Studies
Rachele Del Sordo, Rachele Brugnano, Carla Covarelli, Gioia Fiorucci, Franca Falzetti, Giorgio Barbatelli, Emidio Nunzi, Angelo Sidoni
Primary myelofibrosis (PMF) is an uncommon form of myeloproliferative neoplasm (MPN) characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that, in fully-developed disease, is associated with reactive deposition of fibrous connective tissue, extramedullary hematopoiesis (EMH), and splenomegaly. Kidney involvement is rare and clinically presents with proteinuria, nephrotic syndrome, and renal insufficiency. Renal damage can be due to EMH and glomerulopathy. Renal EMH presents three patterns: infiltration of the interstitium with possible renal failure caused by functional damage of parenchyma and vessels, infiltration of capsule and pericapsular adipose tissue, and sclerosing mass-like lesions that can cause hydronephrosis and hydroureter with obstructive uropathy and renal failure...
2017: Clinical Nephrology. Case Studies
Douglas K Atchison, H David Humes
Renal dysfunction is a common comorbidity of multiple myeloma. However, tumor lysis syndrome is a rare cause of renal dysfunction in multiple myeloma. Elotuzumab is a newly US FDA-approved monoclonal antibody used in the treatment of refractory multiple myeloma. To our knowledge, elotuzumab has not been associated with a case of tumor lysis syndrome. We present the case of a patient who developed clinical tumor lysis syndrome 1 week after treatment with elotuzumab accompanied by renal failure with hyperphosphatemia, hyperkalemia, and profound hyperuricemia...
2017: Clinical Nephrology. Case Studies
Shannon B Palombo, Eric C Wendel, Laura R Kidd, Farshid Yazdi, Mihran V Naljayan
. INTRODUCTION: The association of hepatitis C virus (HCV), cryoglobulinemia, and membranoproliferative glomerulonephritis (MPGN) is well known. Treatment of underlying HCV infection has greatly improved in recent years with the introduction of direct-acting antivirals (DAA), which have demonstrated curative sustained viral response (SVR) rates for select viral genotypes with the added benefit of less drug side effects. However, a mainstay of newer DAAs is sofosbuvir, which is contraindicated in patients with severe renal impairment...
2017: Clinical Nephrology. Case Studies
Jian Wu, Xiao-Ling Pi, Zhi-Bin Ye
Although pre-B acute lymphoblastic leukemia (ALL) is the most common type of renal leukemic infiltration; the renal infiltration with leukemia cells as the initial manifestation of leukemia is very rare. Translocation (1;19)(q23;p13) is one of the most common chromosomal abnormalities in patients with ALL and is observed in 5 - 6% of children with pre-B ALL. However, the incidence of t(1;19) in adults is lower, not exceeding 3%, and the prognosis of adult patients is usually poor. Herein, we report a 52-year-old female patient with pre-B ALL who initially presented as bilateral renal enlargement...
2017: Clinical Nephrology. Case Studies
Keisuke Sugimoto, Satoshi Ueda, Mitsuru Okada, Tsukasa Takemura
Rosai-Dorfman disease is also known as sinus histiocytosis with massive lymphadenopathy. Extranodal Rosai-Dorfman disease has been reported in ~ 43% of cases; the most frequent extranodal sites - skin, soft tissue, bone, respiratory tract, and eye - are usually involved in association with lymphadenopathy. Lack of lymph node involvement is rare, especially when patients manifest renal disease. Here, we describe a patient who developed membranoproliferative glomerulonephritis when lymphadenopathy was absent...
2017: Clinical Nephrology. Case Studies
Robert Rope, Neeraja Kambham, Neiha Arora
BACKGROUND: Renal disease associated with paraproteinemias is classically predicated upon pathologic paraprotein deposition in the kidney. However, growing evidence suggests that paraproteins may be able to systemically activate complement or neutrophils to drive renal damage. This may provide an alternative pathologic mechanism for renal injury in rare cases. CASE REPORT: We report a case of a patient with crescentic pauci-immune glomerulonephritis presenting with rapidly progressive renal failure, polyarthropathy, and a purpuric rash in association with a monoclonal immunoglobulin G κ-light-chain producing multiple myeloma...
2017: Clinical Nephrology. Case Studies
Anawin Sanguankeo, Natanong Thamcharoen, Sikarin Upala
BACKGROUND: Calciphylaxis in a nondialysis patient is a rare condition and is characterized by calcific deposition in tissue. We present a case of calciphylaxis in a nondialysis patient who was diagnosed by clinical presentation and skin biopsy and was treated with sodium thiosulfate with improvement of skin lesions. CASE: A 43-year-old female with type 2 diabetes and atrial fibrillation taking oral anticoagulation medication presented with reddish drainage from the right buttock...
2017: Clinical Nephrology. Case Studies
Andrea Cervi, Dylan Kelly, Iakovina Alexopoulou, Nader Khalidi
PURPOSE: We report the case of a 59-year-old man with chronic hepatitis B and C infection presenting with acute kidney injury and enterococcus faecalis-infective endocarditis (IE). An elevated proteinase-3 (PR3)-ANCA and pauci-immune glomerulonephritis (GN) on renal biopsy were discovered, corresponding to ANCA-mediated GN. We conducted a literature review to assess the role of ANCA in IE and treatment implications. METHODS: On systematic review of the literature, we found five previous cases whereby IE caused by streptococcus and bartonella species were related to ANCA vasculitis-associated GN...
2017: Clinical Nephrology. Case Studies
Meredith Halpin, Olga Kozyreva, Vanesa Bijol, Bertrand L Jaber
We report the case of a 57-year-old man who presented with subacute bacterial endocarditis secondary to Streptococcus mutans complicated by biopsy-proven immune complex-mediated glomerulonephritis (ICGN). Despite initial treatment with antibiotics and a short course of corticosteroids, the kidney function further deteriorated, and plasmapheresis was introduced as third-line therapy to remove circulating immune complexes. Following 7 treatment sessions, the patient recovered kidney function. We discuss the potential merit of plasmapheresis for patients with subacute bacterial endocarditis who develop ICGN...
2017: Clinical Nephrology. Case Studies
Kenneth Lim, David Steele, Andrew Fenves, Ravi Thadhani, Eliot Heher, Amel Karaa
Primary mitochondrial diseases (MD) are complex, heterogeneous inherited diseases caused by mutations in either the mitochondrial or nuclear DNA. Glomerular diseases in MD have been reported with tRNA mutation m.3243A>G causing a syndrome of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS). We describe here a case of focal segmental glomerulosclerosis (FSGS) associated with a new tRNA mutation site. A 34-year-old man with a history of living related kidney transplantation, diabetes, hearing loss, and developmental delay presented to the outpatient clinic with complaints of new behavioral difficulties, worsening symptoms, and brain involvement on imaging...
2017: Clinical Nephrology. Case Studies
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