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An 83-year-old female patient presented to our nephrology outpatient clinic with complaints of weakness, edema, abdominal pain, and constipation, with a preliminary diagnosis of chronic kidney failure related to heart failure. The patient had undergone mitral valve replacement surgery 10 years prior and was diagnosed with chronic renal failure six years prior. Laboratory tests revealed mild normochromic normocytic anemia, consistently high erythrocyte sedimentation rate (ESR) above 100 mm/h, and nephrotic-range proteinuria, prompting suspicion of multiple myeloma. Further investigations, including bone marrow aspiration, confirmed the diagnosis of multiple myeloma. During follow-up, the patient began to complain of difficulty swallowing and symptoms of microstomia. Upon further questioning, it was discovered that these symptoms had been present for more than 10 years. Immunoblot tests revealed positive centromere protein B (CENP-B), suggesting a diagnosis of scleroderma. Subsequently, during follow-up, bullous lesions appeared on the patient's chest. Biopsy samples confirmed a diagnosis of bullous pemphigoid (BP). The co-occurrence of scleroderma, multiple myeloma, and superimposed BP represents a rare and noteworthy case for publication.
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Hemolytic uremic syndrome (HUS), traditionally recognized in pediatric populations, is characterized by renal insufficiency, hemolytic anemia, and thrombocytopenia, often linked to Shiga-like toxin (SLT) exposure. While typically associated with enteric pathogens like Escherichia coli (E. coli) and Shigella, Aeromonas spp. has also been identified as potential SLT producers, posing a new challenge. This study presents an exceptional case of HUS in a 77-year-old female, implicating Aeromonas hydrophila as the causative agent. The patient's clinical trajectory, marked by acute kidney injury post-consumption of raw oysters, underscores the unorthodox manifestation of HUS in adults. Diagnostic confirmation via stool antigen testing and blood culture revealed the presence of SLT and Aeromonas hydrophila, respectively. This case underscores the evolving landscape of HUS etiology, stressing the importance of heightened clinical awareness to expedite therapeutic intervention and mitigate long-term renal complications.
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Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits (PGNMID) is a relatively rare diagnosis with variable presentation. When detectable, the disease is typically indolent rather than malignant and recurs in transplant cases. Here, we report a case of PGNMID, which presented clinically as rapidly progressive glomerulonephritis (RPGN). The patient presented to his primary care physician's office with diarrhea for one day and was admitted for acute kidney injury. Urine sediment was active, and the patient had nephrotic range proteinuria. Serologic workup was negative for any monoclonality: ANA, c-ANCA, and p-ANCA. Kidney biopsy showed diffuse proliferative and crescentic glomerulonephritis with IgG3-kappa restricted deposits, consistent with PGNMID. The patient required dialysis initiation, and corticosteroids were administered. The patient declined further immunomodulatory treatment and remains hemodialysis-dependent. This case highlights the potential for severe renal damage from monoclonal proteins despite an indolent or even undetectable hematologic clone. This entity needs further studies to better understand its immuno-physiological background and develop a standard treatment regimen.
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Fanconi syndrome is a multi-factorial disorder that involves diffuse malfunction of the proximal convoluted tubule in the kidney. Renal wasting of potassium, glucose, bicarbonate, amino acids, and phosphorus characterize the condition. We report a case of a 57-year-old female who presented to our emergency department with cardiopulmonary arrest. After successful resuscitation, she had extensive workup to uncover the cause of her cardiac arrest. She had extensive negative workup but was found to have severely low potassium, prompting further evaluation. She was noted to have elevated urine potassium, with a trans-tubular potassium gradient of 9. She was also found to have severe glycosuria, hypophosphatemia, proteinuria, and an elevated urine anion gap, suggesting proximal convoluted tubular dysfunction. The hypokalemia noted on admission was thought to have been the causative factor for the cardiopulmonary arrest and was thought to be due to proximal tubule dysfunction, with the major suspected diagnosis being a Fanconi-like syndrome. This report highlights the diagnosis and treatment of hypokalemia, the broad differential involved with hypokalemia, and the syndromes involved with renal potassium wasting. This report also seeks to raise awareness of the association of renal potassium wasting with cardiopulmonary arrest.
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This comprehensive narrative review aims to investigate the impact of virtual reality (VR) and social gaming on the quality of life, depression, and dialysis tolerance in patients with chronic kidney disease (CKD), a growing global health concern affecting the quality of life and increasing mortality rates. Through a combination of exercise and video games, exergaming, and digital game therapy (DGT), sedentary risks can be mitigated and therapy adherence enhanced. Despite potential side effects such as exhaustion and nausea, research indicates that virtual therapeutic games improve motivation and dialysis tolerance, and even reduce brain activity in pain-associated regions during procedures. These methods are superior to traditional distraction techniques like music, movies, and toys. Exergaming, social gaming, and VR gaming can improve various health factors including depressive symptoms, functional capacity, muscle strength, fatigue, physical activity, mental health, motivation, engagement, cognition, and overall quality of life for CKD patients. Representing a novel approach to CKD management, these interventions promote physical activity, cognitive stimulation, and social interaction. Continued research and innovation will facilitate the integration of VR-based therapies into routine CKD care.
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Hyperkalemia refractory to standard temporization measures can be life-threatening, and urgent hemodialysis is often utilized as a final resort. Our patient presented with hyperkalemia that was multifactorial in etiology, with acute kidney injury complicated by adrenal insufficiency. Her hyperkalemia was refractory to temporization and excretion agents, and hemodialysis was being considered. Given a recent infection, surgery, and borderline hypotension with low adrenocorticotropic hormone, there was a concern for adrenal insufficiency. However, a full investigation for secondary adrenal insufficiency via magnetic resonance imaging could not be conducted as the patient suffered from claustrophobia. Continued concern for adrenal insufficiency prompted the initiation of intravenous hydrocortisone, and the patient's hyperkalemia resolved within 24 hours. While suspected adrenal insufficiency is already a basis for stress-dose steroids in the setting of pathologies such as severe sepsis, clinicians should have a low threshold for considering refractory hyperkalemia alone as an indication for stress-dose steroids. When dialysis is being considered as an option, this treatment modality should be given even more consideration. Adopting this practice may not only lead to improved mortality from hyperkalemia but also lead to fewer patients being exposed to the risks of dialysis.
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Acute kidney injury (AKI) involves a rapid decline in kidney function, classified into prerenal, intrarenal, and postrenal causes. Drug-induced AKI's complex pathophysiology includes altered hemodynamics, inflammation, crystal deposition, hemolysis, and rhabdomyolysis. This report details a 42-year-old female with hypertension and diabetes who, following a dog bite, exhibited reduced kidney function (GFR: 16 ââmL/min/1.73m2; BUN/Cr: 23/3.23 mg/dL). A renal ultrasound revealed no stones or masses, and the recent use of tirzepatide was identified. Discontinuation of the drug, IV fluid maintenance, and close monitoring led to swift kidney function improvement. This case underscores the importance of recognizing drug-induced AKI, even in unrelated complaints, and highlights the need for vigilance and research into the adverse effects of medications such as glucagon-like peptide 1 (GLP-1) receptor agonists.
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Membranous nephropathy (MN) is an autoimmune disease resulting in nephrotic syndrome. Neural epidermal growth factor-like 1 protein (NELL-1) has been shown to cause a rare form of MN that is more likely to be associated with malignancy. We present a case of a 73-year-old female who was found to have a NELL-1-associated segmental MN. She presented complaining of generalized weakness, chills, and poor appetite, worsening over a one-week duration. Her kidney functions were noted to be markedly deranged, with a computed tomography scan of the abdomen showing evidence of chronic kidney disease. Further testing confirmed heavy proteinuria, although the etiology was still uncertain. A kidney biopsy revealed granular subepithelial immunoglobulin G deposits with subsequent immunohistochemical staining for NELL-1 antigen being positive. She improved with supportive care over the next few days. Despite an extensive workup, no underlying malignancy was found. NELL-1 is a rare yet recognized antigen target for the development of MN. Up to a third of patients with NELL-1-associated MN have associated cancer, thus requiring evaluation for underlying malignancy in this cohort.
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Immunotactoid glomerulopathy is an uncommon cause of glomerular disease that results from deposits derived from immunoglobulins. This rare disease can occur in native kidneys and in transplant patients. They are present only in 0.5% to 1.4% of native kidney biopsies. Treatment of this disease is directed at the underlying monoclonal gammopathy, infection, and B-cell lymphoproliferative disorders. Prognosis is very guarded with 50% of people developing ESRD within five years of diagnosis. We present an interesting, rare case of recurrent immunotactoid glomerulonephritis which responded appropriately to rituximab therapy.
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Catheter-related sheath (CRS) formation secondary to chronic indwelling central venous catheters (CVC) is a well-documented complication. When these fibrin sheaths calcify, they can form a "cast" surrounding the catheter. Upon removal of the CVC, a rare complication can occur where the calcified sheath remains in situ leaving behind an intraluminal catheter-shaped cast. This report describes a case of a 57-year-old dialysis-dependent woman who was found to have a right internal jugular vein cast during the evaluation and treatment of methicillin-resistant Staphylococcus aureus (MRSA) bacteremia. This case reviews and discusses the embolic complications suspected to be a result of this cast. Our case provides insight into the clinical course, diagnostic methods, and imaging identification of a rare pathology and its unique complications.
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Emphysematous pyelonephritis (EPN) is a severe, necrotizing infection of the renal parenchyma. It is commonly found as a complication of uncontrolled diabetes mellitus (DM). EPN has a terrible prognosis unless promptly identified and treated. In this case study, a 38-year-old man with type 2 diabetes mellitus (T2DM) was admitted due to complaints of excruciating abdominal pain, vomiting, and non-adherent to his insulin medication. The patient was subsequently diagnosed with EPN. For most patients, the current course of treatment includes nephrectomy along with antimicrobial medications. In this case report, however, the patient improved with conservative treatment such as IV fluids, antibiotics, and blood glucose control.
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Unilateral renal cystic disease has been mostly reported in older male patients; however, this case is novel as the youngest reported case in the literature and in a female patient. We present a 22-year-old female with no past medical history and no family history of renal disease that was incidentally found to have unilateral renal cystic disease on computed tomography imaging. The patient's renal function was not impaired and the cystic kidney was found to be functioning appropriately on an intravenous pyelogram. The unilateral cystic disease is benign but must be differentiated from autosomal dominant polycystic disease to prevent morbidity and mortality.
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Background Most patients with end-stage kidney disease begin hemodialysis (HD) in an unplanned fashion at a late stage, necessitating the commencement of HD with a temporary venous catheter, the least favorable option. Alternative modalities of kidney replacement therapy (KRT), peritoneal dialysis (PD), and preemptive transplant offer similar or better outcomes than HD at a lower overall cost, and yet they remain underutilized in Saudi Arabia. Early education may help prepare patients with advanced chronic kidney disease (CKD IV and V) to accept their disease and choose a KRT modality that minimizes complications and matches their lifestyle. The aim of the study is to assess the impact of a pilot educational class on therapy choices and outcomes. Methodology In a cross-sectional study, we conducted phone interviews and reviewed medical records of 81 attendees of the multidisciplinary monthly educational class about KRT that was held at the King Abdulaziz Medical City (KAMC) from January 2017 to October 2021. The interview was conducted at least one year after the participants attended the class. The study proposal, consent, and questionnaire were approved by the King Abdulaziz International Medical Research Center. Patient data was retrieved from KAMC electronic medical record system. Results Volunteer participation in the survey was high (62/81). For the respondents, a preemptive kidney transplant was the most preferred (48/62, 77%) option for KRT. Among the preferred fallback options, HD was the most frequently chosen (29/62, 47%) compared to PD (26/62, 41.9%). At the time of the interview, a great majority of the patients (54/62, 87%) was already on KRT, including about half (26/54, 48%) on HD via a catheter, and the rest about equally divided between those on HD via an arteriovenous (AF) fistula (13/54, 24%) and those on PD (15/54, 28%). Thus, half of the respondents on KRT (28/54, 51%) avoided urgent HD catheter commencement. However, because of an unfortunate shortage of donors, only a small minority (2/62, 3%) of patients received preemptive transplantation. Conclusion The KAMC CKD education class helped boost the fraction of patients, significantly above the national average, who accepted the diagnosis of kidney failure and pursued preemptive native HD access or enrolled in PD.
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Introduction Hemodialysis (HD) is a significant contributor to Medicare spending. Peritoneal dialysis (PD) is a lower-cost dialysis modality with non-inferior clinical outcomes. Recent initiatives at the federal level have emphasized shifting dialysis from in-center to home modalities, namely, PD. Such policy has been slow to impact the distribution of HD and PD due to multiple barriers, including at the provider level. Previous research has characterized the role of patient knowledge gaps and preferences in the under-utilization of PD. We sought to understand physician knowledge and attitudes toward PD to elucidate provider-level barriers to PD adoption. Methods We conducted a 10-question survey assessing physician comfort level, perceived knowledge, and objective knowledge of HD and PD that was distributed among the internal medicine faculty at the University of Texas Southwestern Medical Center, Dallas, TX. The survey respondents included nephrologists and non-nephrologists. Demographic information of respondents was collected. Survey responses were summarized and stratified by medical specialty. All statistical tests used 0.05 as the statistical significance level. Results Among 391 survey recipients, there were 83 respondents (21.2%). The mean age of respondents was 43 and 54% were women. With regard to specialty, 88% of respondents were non-nephrologists and 12% were nephrologists. All respondents reported an increased level of comfort and experience caring for patients receiving HD compared to PD. Regardless of specialty, respondents had a high incorrect response rate with regard to contraindications to PD. While nephrologists reported high perceived knowledge regarding PD, objective assessments revealed knowledge gaps with regard to PD candidacy. Non-nephrologists reported lower perceived knowledge but scored better on objective knowledge assessments regarding medical contraindications to PD. Both specialty groups held misconceptions regarding psychosocial barriers to PD. Discussion This physician survey demonstrated overall decreased confidence in knowledge and experience in the care of patients receiving PD compared to HD. Knowledge assessments revealed discordance between perceived knowledge and objective knowledge with regard to contraindications to PD. These findings highlight ongoing misconceptions across medical specialties regarding the applicability of PD. These findings demonstrate the need for increased training on PD candidacy among nephrologists and non-nephrologists alike. These findings demonstrate the need for education and advocacy around PD for providers to effectively meet federal priorities advocating for shifting dialysis to the home. Conclusion This study demonstrates the impact of physician knowledge and attitudes toward PD in the under-utilization of PD as a dialysis modality. These findings demonstrate a need for increased provider education around PD candidacy and the benefits of shifting dialysis care to the home. Novel models of dissemination are needed to increase the adoption of PD and meet federal policy goals of shifting dialysis care to home-based modalities.
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Hypertension is an important modifiable risk factor for cardiovascular disease and stroke. Most cases are diagnosed as essential hypertension however, in some patients, a secondary (and potentially curable) cause is identified. Selecting the right patient to screen for a secondary cause can be challenging and certain clinical and laboratory characteristics can guide work-up. We report a case of a 67-year-old man who presented with intracranial hemorrhage. He had a history of resistant hypertension for three decades and chronic hypokalemia while on a non-diuretic antihypertensive regimen. We discuss our approach to a hypertensive hypokalemic phenotype that led to the diagnosis of Liddle's syndrome with complete amelioration of hypokalemia with directed therapy. This case highlights the importance of accurate and early screening for causes of secondary hypertension in the outpatient community, and in doing so, preventing downstream catastrophic outcomes. It is imperative to develop a clear, concise approach to secondary hypertension, and raising awareness for the importance of early diagnosis as it can potentially avoid downstream sequela.
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Page kidney is a rare cause of hypertension and kidney injury; it results from extrinsic compression of the kidney due to fluid accumulation in the subcapsular space. Hypertensive crisis may be the only presenting clinical sign in patients with Page kidney. Urinomas are a very rare cause of Page kidney with very few cases reported in the literature. Urinoma should be suspected in patients presenting in hypertensive crisis who have a history of recent abdominal trauma, genitourinary malignancy, and renal instrumentation. Patients diagnosed with Page kidney from a urinoma should be managed with the least invasive means possible.
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Idiopathic hyperammonemia is a serious condition that can arise after induction of chemotherapy and is characterized by plasma ammonia levels greater than two times the normal upper limit but within the context of normal liver function. While this dangerous complication usually appears several weeks after the start of chemotherapy, we report a fatal case of idiopathic hyperammonemia that was detected only nine days after induction chemotherapy in a 22-year-old man with no liver pathology or other risks for hyperammonemia. The patient's initial emergent presentation was altered mental status. Laboratory workup showed acute monoblastic leukemia and radiological investigation showed cerebral hemorrhagic foci secondary to leukostasis. He received leukoreduction apheresis and he was started on induction chemotherapy with daunorubicin and cytarabine. On the ninth day of induction chemotherapy, it was noted that he developed worsening neurological findings. Investigations showed significant elevation in ammonia level and associated cerebral edema. Although hyperammonemia was mitigated, the patient's cerebral status worsened and he died 15 days after initial presentation. This case shows that critical hyperammonemia can occur quickly after chemotherapy induction and that strategies for preventing a rise in plasma ammonia are necessary.
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Purpose In the intensive care unit (ICU), acute renal failure is mostly part of multiple organ dysfunction syndromes with mortality ranging from 28%-90%, continuous renal replacement therapy (CRRT) is the predominant mode of RRT used in ICU. The main objective of the study was to evaluate the outcomes in patients with acute kidney injury (AKI) on CRRT in the ICU. Methods A retrospective chart review was conducted for all ICU patients with acute renal failure on CRRT in a tertiary care teaching hospital. A subgroup analysis was conducted between 15 days in hospital survivors and non-survivors to look for predictors of survival for patients on CRRT. Results Two-hundred twenty-six patients underwent CRRT from January 2007 to December 2013. The overall in-hospital mortality was 84.1%. Fifty-six patients (24.77%) survived to the 15-day post-CRRT mark. Acute respiratory failure requiring mechanical ventilation was associated with significantly increased mortality; 89.2% vs. 97.6% (P=0.008), ICU length of stay was significantly longer in the survivor group than the nonsurvivor group. Median±IQR; {20±24 vs 6±7(P: <0.0001)} and so were the ventilator-associated days {16±24 vs 4±6.5 (P: <0.0001)} and duration of CRRT {4.5±5.5 vs 2±2.0(P: <0.0001)}. Patients who survived had a lower incidence of metabolic acidosis {44.6% vs 62.9% (P: 0. 016)} and uremic encephalopathy {12.5% vs 26.5%; (P: 0.031)} but a greater incidence of volume overload {28.6% vs 15.9% (P: 0.031)} as compared to the non-survivor. Acute Physiology And Chronic Health Evaluation II (APACHE II) scores were significantly higher in the non-survivor group (mean SD) 26.9±28.0 vs. 23.9±25.8 (P: 0.0136). Conclusions This observational study in patients undergoing CRRT in an ICU setting revealed that the overall mortality was 84.1%. Fluid overload as an indication of CRRT was associated with improved 15 days' survival whereas higher APACHE II scores and the use of mechanical ventilation were associated with reduced 15 days' survival.
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PURPOSE: Arterial media calcification (AMC) is often the only vascular calcification (VC) present in young patients with chronic renal failure and its presence is associated with higher mortality rates. Currently, X-ray imaging (as a standard approach) is able to show AMC in areas without diffuse overlapping arterial intimal calcification (AIC), but X-ray imaging only allows us to identify this lesion when the vessel is widely calcified. The aim of this study was to evaluate the possibility of using ultrasonography as opposed to X-rays to visualize AMC in patients with chronic renal failure. Patients and Methods: In this cross-sectional study, we examined 105 patients (chronic kidney disease stage IV: 19 patients, hemodialysis: 48 patients, renal transplant: 26 patients; mean age: 54 ± 14 years; 65 males and 40 females); B-mode ultrasonography was performed to detect AMC or AIC on the superficial femoral artery (SFA). As a control, plain radiography of the thigh was performed in all patients. RESULTS: Upon ultrasonography investigation, 12 subjects were excluded due to diffuse VC on the SFA that did not permit a distinction between AMC and AIC. In the remaining 93 patients, AMC was detected on the SFA in 43 patients using ultrasonography and in 20 patients using the standard approach. The sensitivity and specificity of the standard approach for the detection of AMC on the SFA were 47 and 100%, respectively. The positive and negative predictive values of the standard approach were 1 and 0.68, respectively. CONCLUSION: Ultrasonography is able to detect AMC better than the X-ray approach, focusing on individuals at higher risk.