Case Reports in Nephrology https://www.hindawi.com The latest articles from Hindawi © 2017 , Hindawi Limited . All rights reserved. Multiple Electrolyte and Metabolic Emergencies in a Single Patient Tue, 31 Jan 2017 09:33:11 +0000 http://www.hindawi.com/journals/crin/2017/4521319/ While some electrolyte disturbances are immediately life-threatening and must be emergently treated, others may be delayed without immediate adverse consequences. We discuss a patient with alcoholism and diabetes mellitus type 2 who presented with volume depletion and multiple life-threatening electrolyte and metabolic derangements including severe hyponatremia (serum sodium concentration [] 107 mEq/L), hypophosphatemia (“undetectable,” <1.0 mg/dL), and hypokalemia (2.2 mEq/L), moderate diabetic ketoacidosis ([DKA], pH 7.21, serum anion gap [] 37) and hypocalcemia (ionized calcium 4.0 mg/dL), mild hypomagnesemia (1.6 mg/dL), and electrocardiogram with prolonged QTc. Following two liters of normal saline and associated increase in by 4 mEq/L and serum osmolality by 2.4 mosm/Kg, renal service was consulted. We were challenged with minimizing the correction of (or effective serum osmolality) to avoid the osmotic demyelinating syndrome while replacing volume, potassium, phosphorus, calcium, and magnesium and concurrently treating DKA. Our management plan was further complicated by an episode of significant aquaresis. A stepwise approach was strategized to prioritize and correct all disturbances with considerations that the treatment of one condition could affect or directly worsen another. The current case demonstrates that a thorough understanding of electrolyte physiology is required in managing complex electrolyte disturbances to avoid disastrous outcomes. Caprice Cadacio, Phuong-Thu Pham, Ruchika Bhasin, Anita Kamarzarian, and Phuong-Chi Pham Copyright © 2017 Caprice Cadacio et al. All rights reserved. Kinetics of Rituximab Excretion into Urine and Peritoneal Fluid in Two Patients with Nephrotic Syndrome Tue, 24 Jan 2017 00:00:00 +0000 http://www.hindawi.com/journals/crin/2017/1372859/ Clinical observations suggest that treatment of Rituximab might be less effective in patients with nephrotic range proteinuria when compared to nonnephrotic patients. It is conceivable that the reason for this is that significant amounts of Rituximab might be lost in the urine in a nephrotic patient and that these patients require a repeated or higher dosage. However, this has not been systematically studied. In this case report we describe two different patients with nephrotic range proteinuria receiving Rituximab. The first patient received Rituximab for therapy resistant cryoglobulinemic membranoproliferative glomerulonephritis and the other for second line treatment of Felty’s syndrome. We employed flow cytometry to determine the amount of Rituximab excretion in both urine and peritoneal fluid specimens in these patients following administration of Rituximab. We found that a significant amount of Rituximab is lost from the circulation by excretion into the urine. Furthermore we saw a close correlation of the excretion of Rituximab to the excretion of IgG molecules suggesting selectivity of proteinuria as the determining factor of Rituximab excretion. Further larger scale clinical studies could have the potential to evaluate an optimal cut-off value of IgG urinary loss before a possible administration of Rituximab therefore contributing to a more individualized treatment approach in patients with nonselective and nephrotic range proteinuria. Klaus Stahl, Michelle Duong, Anke Schwarz, A. D. Wagner, Hermann Haller, Mario Schiffer, and Roland Jacobs Copyright © 2017 Klaus Stahl et al. All rights reserved. Porphyria Cutanea Tarda in a Patient with End-Stage Renal Disease: A Case of Successful Treatment with Deferoxamine and Ferric Carboxymaltose Sun, 22 Jan 2017 00:00:00 +0000 http://www.hindawi.com/journals/crin/2017/4591871/ Porphyria cutanea tarda (PCT) is a rare disease, with a strong association with hepatitis C virus. PCT is particularly problematic in end-stage renal disease patients as they have no renal excretion of porphyrins and these are poorly dialyzed. Also, conventional treatment of PCT is compromised in these patients as hydroxychloroquine is contraindicated, phlebotomies with the stipulated frequency are poorly tolerated in already anaemia-prone patients, and iron-chelating agents are less efficient in removing iron and contribute to worsening anaemia. The authors report a patient on haemodialysis, with hepatitis C infection, that is diagnosed with PCT. Despite the good clinical results with deferoxamine, she became dependent on blood transfusions because of her ferropenic state. Every time oxide iron was started, the patient developed clinical features of the disease, resolving after the suspension of the drug. A decision was made to start the patient on ferric carboxymaltose, which was well tolerated without disease symptoms and need of further blood transfusions. This case suggests that deferoxamine is efficient in treatment of porphyria cutanea tarda. Also, ferric carboxymaltose may be a valuable option for refractory anaemia in patients with this disease and end-stage renal disease, as it seems to provide iron without clinical relapse of the disease. Natacha Rodrigues, Fernando Caeiro, Alice Santana, Teresa Mendes, and Leonor Lopes Copyright © 2017 Natacha Rodrigues et al. All rights reserved. Successful Management of Refractory Type 1 Renal Tubular Acidosis with Amiloride Tue, 03 Jan 2017 06:52:56 +0000 http://www.hindawi.com/journals/crin/2017/8596169/ A 28-year-old female with history of hypothyroidism, Sjögren’s Syndrome, and Systemic Lupus Erythematosus (SLE) presented with complaints of severe generalized weakness, muscle pain, nausea, vomiting, and anorexia. Physical examination was unremarkable. Laboratory test showed hypokalemia at 1.6 mmol/l, nonanion metabolic acidosis with HCO3 of 11 mmol/l, random urine pH of 7.0, and urine anion gap of 8 mmol/l. CT scan of the abdomen revealed bilateral nephrocalcinosis. A diagnosis of type 1 RTA likely secondary to Sjögren’s Syndrome was made. She was started on citric acid potassium citrate with escalating dosages to a maximum dose of 60 mEq daily and potassium chloride over 5 years without significant improvement in serum K+ and HCO3 levels. She had multiple emergency room visits for persistent muscle pain, generalized weakness, and cardiac arrhythmias. Citric acid potassium citrate was then replaced with sodium bicarbonate at 15.5 mEq every 6 hours which was continued for 2 years without significant improvement in her symptoms and electrolytes. Amiloride 5 mg daily was added to her regimen as a potassium sparing treatment with dramatic improvement in her symptoms and electrolyte levels (as shown in the figures). Amiloride was increased to 10 mg daily and potassium supplementation was discontinued without affecting her electrolytes. Her sodium bicarbonate was weaned to 7.7 mEq daily. Patrick Oguejiofor, Robert Chow, Kenneth Yim, and Bernard G. Jaar Copyright © 2017 Patrick Oguejiofor et al. All rights reserved. Treatment of Light Chain Deposition Disease Using Bortezomib-Based Regimen Followed by Thalidomide-Based Regimen in a Saudi Male Mon, 19 Dec 2016 09:48:38 +0000 http://www.hindawi.com/journals/crin/2016/7485695/ Light chain deposition disease (LCDD) is a rare illness with, as yet, no clear evidence-based guidelines for its treatment. To the best of our knowledge, LCDD has not been previously reported from Saudi Arabia. We present in this report, a 38-year-old Saudi male who presented with clinical features suggestive of hypertensive nephropathy but kidney biopsy later revealed the diagnosis of LCDD. His serum creatinine at presentation was 297 μmol/L which came down to 194 μmol/L on treatment with Bortezomib, Cyclophosphamide and Dexamethasone. His 24-hour protein excretion at presentation was 6 g/L which also came down to less than 1 g/day. He was later placed on Cyclophosphamide, Thalidomide, and Dexamethasone regimen because of persistent high titres of serum free light chains. He went into remission with undetectable serum free light chains and remained so for three years at the time of writing this report. We conclude that LCDD, though rare, does occur in Saudi population. The treatment of LCDD is challenging but the use of Bortezomib, a proteosome inhibitor, is promising. However, suboptimal response may require further treatment with other therapeutic options such as chemotherapy with alkylating agents or high-dose Melphalan with autologous stem cell transplant. Bappa Adamu, Mushabab Al-Ghamdi, Mustafa Ahmad, and Khaled O. Alsaad Copyright © 2016 Bappa Adamu et al. All rights reserved. Acute Kidney Injury, Recurrent Seizures, and Thrombocytopenia in a Young Patient with Lupus Nephritis: A Diagnostic Dilemma Sun, 04 Dec 2016 09:27:48 +0000 http://www.hindawi.com/journals/crin/2016/7104098/ Introduction. Posterior reversible encephalopathy syndrome (PRES) is a constellation of clinical and radiologic findings. Fluctuations in blood pressure, seizures, and reversible brain MRI findings mainly in posterior cerebral white matter are the main manifestations. PRES has been associated with multiple conditions such as autoimmune disorders, pregnancy, organ transplant, and thrombotic microangiopathy (TMA). Case Presentation. A 22-year-old woman with history of Systemic Lupus Erythematous complicated with chronic kidney disease secondary to lupus nephritis class IV presented with recurrent seizures and uncontrolled hypertension. She was found to have acute kidney injury and thrombocytopenia. Repeat kidney biopsy showed diffuse endocapillary and extracapillary proliferative and membranous lupus nephritis (ISN-RPS class IV-G+V) and endothelial swelling secondary to severe hypertension but no evidence of TMA. Brain MRI showed reversible left frontal and parietal lesions that resolved after controlling the blood pressure, making PRES the diagnosis. Conclusion. PRES is an important entity that must be recognized and treated early due to the potential reversibility in the early stages. Physicians must have high suspicion for these unusual presentations. We present a case where performing kidney biopsy clinched the diagnosis in our patient with multiple confounding factors. Hector Alvarado Verduzco and Anjali Acharya Copyright © 2016 Hector Alvarado Verduzco and Anjali Acharya. All rights reserved. Adjustment of Eculizumab Dosage Pattern in Patients with Atypical Hemolytic Uremic Syndrome with Suboptimal Response to Standard Treatment Pattern Tue, 29 Nov 2016 11:01:49 +0000 http://www.hindawi.com/journals/crin/2016/7471082/ In patients with atypical hemolytic uremic syndrome (aHUS), complement blocking by eculizumab rapidly halts the process of thrombotic microangiopathy and it is associated with clear long-term hematologic and renal improvements. Eculizumab treatment consists of a 4-week initial phase with weekly IV administration of 900 mg doses, followed by a maintenance phase with a 1,200 mg dose in the fifth week and every days thereafter. We present three patients with aHUS and suboptimal response to eculizumab treatment at the usual administration dosage who showed hematologic and renal improvements after an adjustment in the eculizumab treatment protocol. Camino García Monteavaro, Carmen Peralta Roselló, Borja Quiroga, José María Baltar Martín, Lorena Castillo Eraso, Fernando de Álvaro Moreno, Alberto Martínez Vea, and María Teresa Visus-Fernández de Manzanos Copyright © 2016 Camino García Monteavaro et al. All rights reserved. Bullous Dermatosis in an End-Stage Renal Disease Patient: A Case Report and Literature Review Thu, 24 Nov 2016 09:37:25 +0000 http://www.hindawi.com/journals/crin/2016/6713807/ Patients with advanced chronic kidney disease including ESRD patients may present with a wide spectrum of cutaneous abnormalities, ranging from xerosis to hyperpigmentation to severe deforming necrotizing lesions. Skin problems are not uncommon in this population of patients, with a clinical presentation that can be quite bizarre, mandating a long list of differential diagnostic possibilities, and subsequent rise of a puzzling diagnostic challenge. We describe an ESRD patient who presented with blistering, nonhealing ulcerative lesions with a diagnostic skin biopsy revealing a mixed pattern of linear IgA bullous dermatosis and dermatitis herpetiformis. A clinical remission could be achieved with pulse intravenous steroids followed by oral maintenance in combination with dapsone, with no evidence of recurrence. Zeenat Yousuf Bhat, Marwan Abu Minshar, Nashat Imran, Andrew Thompson, and Yahya Osman Malik Copyright © 2016 Zeenat Yousuf Bhat et al. All rights reserved. A Case of Pulmonary-Renal Syndrome Leading to the Diagnosis of Legionnaires’ Disease Wed, 23 Nov 2016 06:08:18 +0000 http://www.hindawi.com/journals/crin/2016/4250819/ We report a case of a 51-year-old Caucasian man referred at our department due to acute renal failure (ARF) complicating respiratory failure during hospitalization in a regional hospital. The patient was previously started on steroids due to the suspicion of rapidly progressive glomerulonephritis (RPGN) in the context of Goodpasture syndrome. However, clinical and laboratory findings did not support this diagnosis; instead a careful evaluation limited differential diagnosis of the renal insult to acute tubular necrosis or acute interstitial nephritis (AIN) following respiratory infection. With lung function fully improved but renal function not recovering, a renal biopsy revealed AIN, a finding leading to further diagnostic testing and finally to the diagnosis of Legionnaires’ disease as a cause of this patient’s pulmonary-renal syndrome. The management consisted of progressive tapering of oral steroids associated with full recovery of the patient’s renal function. This is a rare case of Legionnaires’ disease causing immune-mediated AIN and highlights the possibility of Legionella infection as a cause of pulmonary-renal syndrome. Erasmia Sabani, Pantelis A. Sarafidis, Antonios Lazaridis, Theodora Kouloukourgiotou, Konstantinos Stylianou, Afroditi Pantzaki, Aikaterini Papagianni, and Georgios Efstratiadis Copyright © 2016 Erasmia Sabani et al. All rights reserved. A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis Sun, 06 Nov 2016 11:16:43 +0000 http://www.hindawi.com/journals/crin/2016/9340524/ Background. Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation. A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical history consisted of newly diagnosed but untreated rheumatoid arthritis, hypertension, and non-insulin-dependent diabetes mellitus. He was found to have acute renal failure, proteinuria, and hypoglycemia. Standard therapy, including intravenous fluids, did not improve his acute renal failure. A vasculitis workup resulted in a positive myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Renal biopsy revealed crescentic glomerulonephritis (GN) pauci-immune type, suggestive of MPO-ANCA-associated vasculitis (MPO-AAV). Treatment consisted of prednisone, cyclophosphamide, and seven cycles of plasmapheresis, in addition to hemodialysis for uremia. Upon discharge, he received hemodialysis for another week and continued treatment with cyclophosphamide and prednisone. Conclusion. Patients with longstanding rheumatoid arthritis may develop renal failure due to nonsteroidal anti-inflammatory medication use and AA type amyloidosis; however, necrotizing glomerulonephritis with crescent formation has been rarely reported. This stresses the importance of early recognition and swift initiation of treatment. Nathalie Foray, Tamer Hudali, Muralidhar Papireddy, and John Gao Copyright © 2016 Nathalie Foray et al. All rights reserved. Interferon Induced Focal Segmental Glomerulosclerosis Wed, 26 Oct 2016 09:51:09 +0000 http://www.hindawi.com/journals/crin/2016/6967378/ Behçet’s disease is an inflammatory disease of unknown etiology which involves recurring oral and genital aphthous ulcers and ocular lesions as well as articular, vascular, and nervous system involvement. Focal segmental glomerulosclerosis (FSGS) is usually seen in viral infections, immune deficiency syndrome, sickle cell anemia, and hyperfiltration and secondary to interferon therapy. Here, we present a case of FSGS identified with kidney biopsy in a patient who had been diagnosed with Behçet’s disease and received interferon-alpha treatment for uveitis and presented with acute renal failure and nephrotic syndrome associated with interferon. Yusuf Kayar, Nuket Bayram Kayar, Nadir Alpay, Jamshid Hamdard, Iskender Ekinci, Sebnem Emegil, Rabia Bag Soydas, and Birol Baysal Copyright © 2016 Yusuf Kayar et al. All rights reserved. Apolipoprotein C-II Deposition Amyloidosis: A Potential Misdiagnosis as Light Chain Amyloidosis Thu, 20 Oct 2016 14:18:44 +0000 http://www.hindawi.com/journals/crin/2016/8690642/ Hereditary amyloidoses are rare and pose a diagnostic challenge. We report a case of hereditary amyloidosis associated with apolipoprotein C-II deposition in a 61-year-old female presenting with renal failure and nephrotic syndrome misdiagnosed as light chain amyloidosis. Renal biopsy was consistent with amyloidosis on microscopy; however, immunofluorescence was inconclusive for the type of amyloid protein. Monoclonal gammopathy evaluation revealed kappa light chain. Bone marrow biopsy revealed minimal involvement with amyloidosis with kappa monotypic plasma cells on flow cytometry. She was started on chemotherapy for light chain amyloidosis. She was referred to the Mayo clinic where laser microdissection and liquid chromatography mass spectrometry detected high levels of apolipoprotein C-II, making a definitive diagnosis. Apolipoprotein C-II is a component of very low-density lipoprotein and aggregates in lipid-free conditions to form amyloid fibrils. The identification of apolipoprotein C-II as the cause of amyloidosis cannot be solely made with routine microscopy or immunofluorescence. Further evaluation of biopsy specimens with laser microdissection and mass spectrometry and DNA sequencing of exons should be done routinely in patients with amyloidoses for definitive diagnosis. Our case highlights the importance of determining the subtype of amyloidosis that is critical for avoiding unnecessary therapy such as chemotherapy. Sadichhya Lohani, Emily Schuiteman, Lohit Garg, Dhiraj Yadav, and Sami Zarouk Copyright © 2016 Sadichhya Lohani et al. All rights reserved. Systemic Toxicity of Intraperitoneal Vancomycin Thu, 20 Oct 2016 06:51:54 +0000 http://www.hindawi.com/journals/crin/2016/3968690/ Intraperitoneal vancomycin is used for empiric treatment of peritoneal dialysis peritonitis. It is dosed intermittently and a high systemic concentration is often achieved. Despite this, there are very few reports of systemic toxicity from intraperitoneal vancomycin. We report the course of a patient who developed a drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome after three weeks of intraperitoneal vancomycin. We review the literature and conclude that this is the first ever reported case of DRESS syndrome from intraperitoneal vancomycin. Teerath Kumar, Iris Teo, and Brendan B. McCormick Copyright © 2016 Teerath Kumar et al. All rights reserved. A Swelling in the Mouth in a Chronic Hemodialysis Patient Sun, 09 Oct 2016 13:29:48 +0000 http://www.hindawi.com/journals/crin/2016/4970702/ Oral manifestations of severe secondary hyperparathyroidism include maxillary and mandibular deformities, brown tumors, dental abnormalities, and metastatic calcification of soft tissues. We report on a chronic hemodialysis (HD) woman with severe, uncontrolled secondary hyperparathyroidism and a painful, nontender mass in the floor of her mouth. The most likely clinical diagnosis was a bone tumoral lesion of the oral cavity, secondary to renal osteodystrophy. Unexpectedly, pathological examination showed characteristic features of ossifying fibroma (OF) of the jaw, a rare, benign fibroosseous lesion characterized by the replacement of normal bone by collagen and fibroblasts containing varying amounts of mineralized substance. The occurrence of an OF in chronic HD patients is exceptional. Differential diagnosis must be made with bone tumoral lesions secondary to renal osteodystrophy. Surgical removal is the treatment of choice. The pathogenesis of OF in the setting of secondary hyperparathyroidism remains unknown. Parathyroidectomy may not be necessary to avoid OF recurrence after surgical removal. Arnaud Devresse, Alexandros Raptis, Anne-Sophie Claes, and Laura Labriola Copyright © 2016 Arnaud Devresse et al. All rights reserved. Interstitial Nephritis in a Patient with Inflammatory Bowel Disease Wed, 14 Sep 2016 12:08:37 +0000 http://www.hindawi.com/journals/crin/2016/4260365/ Tubulointerstitial nephritis in patients with inflammatory bowel disease has been linked to the use of 5-ASA derivatives. Various aspects of this theory have been challenged with a potential role for the underlying autoimmune disorder. Steroids are the mainstay of treatment and mycophenolate mofetil might be an effective alternative. We report a patient who responded well to mycophenolate despite continuing mesalamine, the suspected offending agent. Payaswini Vasanth, Michelle Parmley, Jose Torrealba, and Tamim Hamdi Copyright © 2016 Payaswini Vasanth et al. All rights reserved. Subdiaphragmatic Renal Ectopia: Case Report and Review of the Literature Wed, 07 Sep 2016 16:39:16 +0000 http://www.hindawi.com/journals/crin/2016/1084917/ Background. We report the case of a male infant whose right kidney migrated to an ectopic position after birth. The migration of a kidney in postnatal life without any symptoms has not been reported in literature so far. Case Presentation. In a series of antenatal and the first postnatal ultrasound scans, the right kidney was normally located within the right renal fossa. During the first 3 months of life, the kidney migrated to a subdiaphragmatic position. This was confirmed on MRI scan. The infant was asymptomatic with normal renal function and blood pressure. Conclusion. Postnatal migration of a kidney has been described in cases of diaphragmatic hernia or nephroptosis. In this report, we describe a case of kidney migration where there were no underlying anatomical defects to provide an explanation for the kidney migration. This is the first report in literature of a case of postnatal migration of a kidney. Eleftherios Zolotas and Rajesh G. Krishnan Copyright © 2016 Eleftherios Zolotas and Rajesh G. Krishnan. All rights reserved. An Atypical Presentation of a Male with Oral-Facial-Digital Syndrome Type 1 Related Ciliopathy Mon, 29 Aug 2016 09:51:00 +0000 http://www.hindawi.com/journals/crin/2016/3181676/ Background. Oral-facial-digital syndrome type 1 (OFD1) is a rare condition with X-linked dominant inheritance caused by mutations in the Cxorf5 (OFD1) gene. This gene encodes the OFD1 protein located within centrosomes and basal bodies of primary cilia. Approximately 15–50% of patients with OFD1 progress to end-stage kidney disease following development of polycystic changes within the kidneys. This condition almost always causes intrauterine lethality in males. Description of Case Diagnosis and Treatment. A Caucasian male aged 9 years and 9 months presented with increased urinary frequency, increased thirst, and decreased appetite. Physical examination demonstrated short stature, hearing loss, photophobia, murmur, and hypogonadism. He had no other dysmorphic features. Laboratory results revealed anemia, renal insufficiency, and dilute urine with microscopic hematuria but no proteinuria. Ultrasound showed small kidneys with increased echogenicity but no evidence of cystic changes. A Ciliopathy Panel showed a novel and likely pathogenic deletion, approximately 7.9 kb, in the OFD1 gene encompassing exons 16, 17, and 19 (c.1654+833_2599+423del). Brain MRI did not demonstrate typical OFD1 findings. He is currently on chronic hemodialysis awaiting transplant from a living donor. Conclusions. We present a male patient with OFD1 mutation who lacks the classic OFD1 phenotype who presented with end-stage renal disease without evidence of polycystic changes within the kidneys. Sheena Sharma, Jennifer M. Kalish, Ethan M. Goldberg, Francis Jeshira Reynoso, and Madhura Pradhan Copyright © 2016 Sheena Sharma et al. All rights reserved. SIADH Induced by Pharyngeal Squamous Cell Carcinoma: Case Report and Literature Review Mon, 22 Aug 2016 08:38:22 +0000 http://www.hindawi.com/journals/crin/2016/3186714/ Background. The Syndrome of Inappropriate Antidiuretic Hormone (SIADH) is considered to be the most common cause of euvolemic hyponatremia. The most common malignancy associated with SIADH is small cell lung cancer. We present a rare case of a patient with SIADH secondary to well differentiated squamous cell carcinoma of the naso-oropharynx. Case. A 46-year-old Caucasian woman presented to emergency department with four-week history of progressive dysphagia. On examination, she was found to have a pharyngeal mass. CT scan and MRI of neck confirmed a mass highly suspicious of carcinoma. Patient’s serum sodium level decreased to 118 mEq/L and other labs including serum and urine osmolality confirmed SIADH. She was started on fluid restriction and oral sodium tablets which gradually improved her serum sodium levels. Biopsy confirmed diagnosis of squamous cell carcinoma of pharynx. Conclusion. SIADH can be caused by squamous cell carcinoma. Appropriate management includes fluid restriction. Hafiz Muhammad Sharjeel Arshad, Aleida Rodriguez, and Faten Suhail Copyright © 2016 Hafiz Muhammad Sharjeel Arshad et al. All rights reserved. Tuberous Sclerosis and Bilateral Renal Angiomyolipomas: A Case Report and Literature Review of Emerging Treatment Strategies Mon, 25 Jul 2016 16:06:04 +0000 http://www.hindawi.com/journals/crin/2016/4595014/ Tuberous sclerosis complex is a rare multisystemic genetic disorder associated with the development of benign hamartomas. Angiomyolipomas are one such characteristic finding that may be seen in 55–80% of tuberous sclerosis complex patients. While being normally asymptomatic, they can also cause significant morbidity and mortality. We present the case of a patient with tuberous sclerosis complex and recently discovered bilateral renal angiomyolipomas, admitted for hematuria who underwent left renal artery embolization; however, worsening renal function necessitated subsequent nephrectomy. Despite still being mainstays of treatment, invasive interventions are now being recommended for specific patient populations as demonstrated in our case. Emerging strategies targeting the PI3K/AKT/mTOR pathway have been shown to reduce the size of angiomyolipomas and are now used to treat asymptomatic cases >3 cm. Our review discusses these treatment options with the intention of increasing awareness of current recommendations and hopefully leading to increased application of these novel therapies that will reduce the need for invasive interventions. Francois Jo-Hoy, Omar Tolaymat, Ryan Kunjal, and Leighton R. James Copyright © 2016 Francois Jo-Hoy et al. All rights reserved. Aldosterone Response in Severe Hypokalemia and Volume Depletion: A Case Report and Review of the Recent Research Wed, 20 Jul 2016 10:46:39 +0000 http://www.hindawi.com/journals/crin/2016/2036503/ We report a case of severe hypokalemia and volume depletion complicated by chronic watery diarrhea resulting from chronic alcoholism in a 57-year-old man. Prompt replacement of normal saline with potassium chloride and cessation of alcohol intake resulted in a favorable outcome. We discuss the pathophysiology of the case, emphasizing the response of aldosterone in both hypokalemia and volume depletion, and provide a review of recent research. Keiko Kai, Naoto Tominaga, Daisuke Uchida, Nanae Fukai, Yumie Matsuura, Susumu Uda, and Akio Yokochi Copyright © 2016 Keiko Kai et al. All rights reserved. Collapsing Glomerulopathy in a Child with Galloway-Mowat Syndrome Tue, 14 Jun 2016 11:44:45 +0000 http://www.hindawi.com/journals/crin/2016/4386291/ Galloway-Mowat syndrome (GMS) is an autosomal recessive disorder with a poor prognosis that was first defined as a triad of central nervous system involvement, hiatal hernia, and nephrotic syndrome. However, this syndrome is now known to have a heterogeneous clinical presentation. The nephrotic syndrome is steroid resistant and is responsible for the outcome. The combination of collapsing glomerulopathy and GMS is very rare. A 26-month-old boy presented with steroid-resistant nephrotic syndrome associated with neurologic findings, including microcephaly, psychomotor retardation, and nystagmus. Magnetic resonance imaging showed marked cerebral atrophy, optic atrophy, and hypomyelination. A renal biopsy was consistent with collapsing glomerulopathy. If collapsing glomerulopathy is associated with neurological abnormalities, especially with microcephaly, clinicians should consider GMS as a possible underlying cause. Cengiz Zeybek, Gokalp Basbozkurt, Salih Hamcan, Ayhan Ozcan, Davut Gul, and Faysal Gok Copyright © 2016 Cengiz Zeybek et al. All rights reserved. A Rare Cause of Acute Kidney Injury in a Female Patient with Breast Cancer Presenting as Renal Colic Tue, 17 May 2016 13:40:38 +0000 http://www.hindawi.com/journals/crin/2016/9565873/ Renal infarction is a rare cause of acute kidney injury which could lead to permanent loss of renal function. A prompt diagnosis is necessary in order to achieve a successful revascularization of the occluded artery. Given the rarity of the disease and the paucity of the reported cases in the previous literature a high index of suspicion must be maintained not only in the classical cardiac sources of systemic emboli (atrial fibrillation, dilated cardiomyopathy, or endocarditis), but also in the situations when a hypercoagulable state is presumed. The unspecific presenting symptoms often mask the true etiology of the patient’s complaints. We present here a rare case of renal infarction that occurred in the setting of a hypercoagulable state, in a female patient with a history of breast cancer and documented hepatic metastases. Roxana Jurubita, Bogdan Obrisca, and Gener Ismail Copyright © 2016 Roxana Jurubita et al. All rights reserved. Cystatin C Falsely Underestimated GFR in a Critically Ill Patient with a New Diagnosis of AIDS Tue, 17 May 2016 11:28:52 +0000 http://www.hindawi.com/journals/crin/2016/9349280/ Cystatin C has been suggested to be a more accurate glomerular filtration rate (GFR) surrogate than creatinine in patients with acquired immunodeficiency syndrome (AIDS) because it is unaffected by skeletal muscle mass and dietary influences. However, little is known about the utility of this marker for monitoring medications in the critically ill. We describe the case of a 64-year-old female with opportunistic infections associated with a new diagnosis of AIDS. During her course, she experienced neurologic, cardiac, and respiratory failure; yet her renal function remained preserved as indicated by an eGFR ≥ 120 mL/min and a urine output > 1 mL/kg/hr without diuresis. The patient was treated with nephrotoxic agents; therefore cystatin C was assessed to determine if cachexia was resulting in a falsely low serum creatinine. Cystatin C measured 1.50 mg/L which corresponded to an eGFR of 36 mL/min. Given the >60 mL/min discrepancy, serial 8-hour urine samples were collected and a GFR > 120 mL/min was confirmed. It is unclear why cystatin C was falsely elevated, but we hypothesize that it relates to the proinflammatory state with AIDS, opportunistic infections, and corticosteroids. More research is needed before routine use of cystatin C in this setting can be recommended. Caitlin S. Brown, Kianoush B. Kashani, Jeremy M. Clain, and Erin N. Frazee Copyright © 2016 Caitlin S. Brown et al. All rights reserved. Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy Wed, 11 May 2016 13:47:14 +0000 http://www.hindawi.com/journals/crin/2016/1075659/ Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN). The diagnosis was consistent with granulomatosis with polyangiitis (GPA). Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management. Ryan Kunjal, Raafat Makary, and Andreea Poenariu Copyright © 2016 Ryan Kunjal et al. All rights reserved. Febuxostat and Increased Dialysis as a Treatment for Severe Tophaceous Gout in a Hemodialysis Patient Thu, 21 Apr 2016 12:39:09 +0000 http://www.hindawi.com/journals/crin/2016/9106935/ Uric acid accumulates in renal failure and is thought to be a uremic toxin—that is, higher levels of uric acid are more damaging to the kidneys. Urate crystals can precipitate in the kidney tubules, cause urate stones, and promote inflammatory changes in the renal interstitium and vascular endothelium. Uric acid is also a small non-protein-bound molecule and therefore easily dialyzable. Here, we present the case of an anuric hemodialysis patient with severe tophaceous gout who regained some renal function and whose gout burden significantly decreased resulting in marked improvement in functional status using a new gout medication, febuxostat, and increased frequency of dialysis. Lynda Ann Frassetto and Suzanne Gibson Copyright © 2016 Lynda Ann Frassetto and Suzanne Gibson. All rights reserved. Suppression of Parathyroid Hormone in a Patient with Severe Magnesium Depletion Thu, 14 Apr 2016 08:15:38 +0000 http://www.hindawi.com/journals/crin/2016/2608538/ Hypomagnesemia is often associated with coexisting electrolyte abnormalities like hypokalemia and hypocalcemia. Hypocalcemia has been shown to be secondary to hypoparathyroidism induced by hypomagnesemia. Here, we discuss a case of a patient with severe hypomagnesemia and associated hypocalcemia. A 38-year-old lady was admitted to the hospital for weakness of lower extremities and an eventual fall. The exam was significant for decreased motor strength and some paresthesias. The laboratory data was significant for hypomagnesemia, hypokalemia, and low parathyroid level in the face of hypocalcemia. After replacing magnesium, the parathyroid hormone levels normalized and led to eventual correction of calcium levels without any additional calcium replacement therapy. There was complete symptom resolution with correction of electrolyte abnormalities. This case highlights the importance of looking for all associated abnormalities in a patient with hypomagnesemia and starting the replacement therapy by first replacing the magnesium and then the others as needed. Replacing the magnesium alone may correct the hypoparathyroidism and eliminate the need for calcium replacement. Sangeeta Mutnuri, Isaac Fernandez, and Tina Kochar Copyright © 2016 Sangeeta Mutnuri et al. All rights reserved. Reversible Fluindione-Induced Chronic Interstitial Nephritis Tue, 05 Apr 2016 10:52:39 +0000 http://www.hindawi.com/journals/crin/2016/9818195/ Fluindione is well known to induce acute drug-induced interstitial nephritis (IN). Most cases occurred soon after the onset of treatment. We report a unique case of severe subacute fluindione-induced IN diagnosed 2 years after the treatment was begun. Renal function dramatically improved after fluindione withdrawal and steroid therapy. Thomas Crepin, Jamal Bamoulid, Cécile Courivaud, Omar Dahmani, Sophie Felix, and Didier Ducloux Copyright © 2016 Thomas Crepin et al. All rights reserved. A Case of Primary Hypoparathyroidism Presenting with Acute Kidney Injury Secondary to Rhabdomyolysis Thu, 10 Mar 2016 13:17:07 +0000 http://www.hindawi.com/journals/crin/2016/3240131/ Hypoparathyroidism is the most common cause of symmetric calcification of the basal ganglia. Herein, a case of primary hypoparathyroidism with severe tetany, rhabdomyolysis, and acute kidney injury is presented. A 26-year-old male was admitted to the emergency clinic with leg pain and cramps, nausea, vomiting, and decreased amount of urine. He had been treated for epilepsy for the last 10 years. He was admitted to the emergency department for leg pain, cramping in the hands and legs, and agitation multiple times within the last six months. He was prescribed antidepressant and antipsychotic medications. He had a blood pressure of 150/90 mmHg, diffuse abdominal tenderness, and abdominal muscle rigidity on physical examination. Pathological laboratory findings were as follows: creatinine, 7.5 mg/dL, calcium, 3.7 mg/dL, alanine transaminase, 4349 U/L, aspartate transaminase, 5237 U/L, creatine phosphokinase, 262.000 U/L, and parathyroid hormone, 0 pg/mL. There were bilateral symmetrical calcifications in basal ganglia and the cerebellum on computerized tomography. He was diagnosed as primary hypoparathyroidism and acute kidney injury secondary to severe rhabdomyolysis. Brain calcifications, although rare, should be considered in dealing with patients with neurological symptoms, symmetrical cranial calcifications, and calcium metabolism abnormalities. Abdullah Sumnu, Zeki Aydin, Meltem Gursu, Sami Uzun, Serhat Karadag, Egemen Cebeci, Savas Ozturk, and Rumeyza Kazancioglu Copyright © 2016 Abdullah Sumnu et al. All rights reserved. Resin-Induced Colonic Pseudotumor: Rare Complication from Chronic Use of Potassium Binders in a Hemodialysis Patient Mon, 29 Feb 2016 11:55:26 +0000 http://www.hindawi.com/journals/crin/2016/3692086/ Potassium-binding resins are widely used in the treatment of hyperkalemia, mostly in the acute setting. Gastrointestinal adverse events, although reported, are not frequently seen due to its short course of use. This report describes a case involving an end-stage renal disease patient on hemodialysis who developed a colonic mass after being on sodium polystyrene sulfonate chronically for persistent hyperkalemia. Gastrointestinal symptoms developed late during the treatment rather than early as reported previously in the literature. This mass was mistaken for a carcinomatous lesion, which initiated an extensive work-up as well as hospitalization that nearly resulted in a subtotal colectomy. Mary Bui, Shyan-Yih Chou, Pierre Faubert, Pablo Loarte, and Ronny Cohen Copyright © 2016 Mary Bui et al. All rights reserved. Bile Nephropathy in Flucloxacillin-Induced Cholestatic Liver Dysfunction Wed, 24 Feb 2016 07:35:57 +0000 http://www.hindawi.com/journals/crin/2016/4162674/ Kidney injury in the context of cholestatic liver dysfunction is not uncommon; this has been historically referred to as cholemic nephrosis implying a direct deleterious renal effect of cholemia. However, scepticism about the exact role that bile and its constituents play in this injury has led to the disappearance of the term. We describe a case of severe AKI due to bile nephropathy with bile casts in flucloxacillin-induced liver dysfunction. We also discuss the recent literature reviving the concept of bile nephropathy. Basil Alnasrallah, John F. Collins, and L. Jonathan Zwi Copyright © 2016 Basil Alnasrallah et al. All rights reserved.