Marginal Zone Lymphoma Manifesting as Macrophage Activation Syndrome: A Case Report.

Macrophage activation syndrome (MAS) is a form of secondary hemophagocytic lymphohistiocytosis (HLH) when it occurs in the context of rheumatologic disorders. HLH is a rare and potentially life-threatening syndrome characterized by excessive immune system activation. It is mainly seen in children and can be genetic based or related to infections, malignancies, rheumatologic disorders, or immunodeficiency syndromes. MAS can present with nonspecific symptoms, leading to a delay in diagnosis. This report describes a case of a 64-year-old female with marginal zone lymphoma and systemic lupus erythematosus who presented with a purpuric rash and acute kidney injury. She underwent a kidney biopsy and was diagnosed with MAS. This case highlights the importance of promptly recognizing MAS's symptoms and signs, allowing timely diagnosis and early therapeutic intervention. This potentially fatal condition tends to respond well to rapid treatment initiation with corticosteroids and to address the underlying condition.

Calciphylaxis in a Patient With Lupus Nephritis and Acute Kidney Injury: A Rare Case Report and Literature Review.

Calciphylaxis is a rare and severe disease characterized by calcification, fibrosis, and thrombosis of small blood vessels. Although it primarily affects patients with end-stage renal disease (ESRD) on dialysis, limited cases have been reported of calciphylaxis in patients with acute kidney injury (AKI) and lupus. This case report describes the occurrence of calciphylaxis in a 35-year-old female recently diagnosed with lupus nephritis class IV and AKI requiring dialysis.

IgA nephropathy: a review of existing and emerging therapies.

IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis worldwide. Recently, there have been multiple advances in the understanding of IgAN pathophysiology and therapeutic options. Despite the advent of new treatment options, individual risk stratification of the disease course and choosing the best treatment strategy for the patient remains challenging. A multitude of clinical trials is ongoing, opening multiple opportunities for enrollment. In this brief review we discuss the current approach to the management of IgAN and highlight the ongoing clinical trials.

Global hemodialysis vascular access care: Three decades of evolution.

The development of vascular access for hemodialysis has come a long way since 1943 when the first hemodialysis treatment was performed in humans by connecting an artery and vein using an external glass canula. Since then, vascular access care has evolved robustly through contributions from numerous countries and professional nephrology societies, worldwide. To understand the global distribution and contribution of different specialties to medical literature on dialysis vascular access care, we performed a literature search from 1991 to 2021 and identified 2768 articles from 74 countries. The majority of publications originated from the United States (41.5%), followed by China (5.1%) and the United Kingdom (4.6%). Our search results comprise of observational studies (43%), case reports/series (27%), review articles (16.5%) and clinical trials (12%). A large proportion of articles were published in Nephrology journals (49%), followed by General Medicine (14%), Surgery (10%), Vascular Medicine (8%), and Interventional Radiology journals (4%). With the introduction of interventional nephrology, nephrologists will be able to assume the majority of the responsibility for dialysis vascular access care and above all maintain a close interdisciplinary collaboration with other specialties to provide optimum patient care. In this review article, we discuss the history, evolving knowledge, challenges, educational opportunities, and future directions of dialysis vascular access care, worldwide.

The Kidney Diet Challenge: An Experiential Educational Experience.

Background: Health care providers who care for patients with CKD must be able to provide effective counseling about a kidney-friendly diet. Nutrition is underemphasized in medical curricula, and the kidney diet is one of the most challenging diets. We hypothesized that participation in an experiential educational program in kidney diet would result in improved knowledge of the underlying principles behind it and provide concrete examples of how to explain this diet to patients. Methods: The first part of this study was a knowledge assessment administered to all US nephrology fellows during the 2020 National Board of Medical Examiners Nephrology In-Training Examination. We later opened the assessment to a broader, global audience via social media. Respondents included trainees, practicing nephrologists, dieticians, and other health professionals. Participants self-identified willingness to participate in the second part of the study, the Kidney Diet Challenge (KDC). The 5-day challenge included daily webinars by experts in nutrition. Daily surveys captured self-reported adherence to the diet. Social media was used to engage with participants. All participants received a follow-up knowledge assessment. Results: Among the nephrology fellows (n=317), the median pretest score was 2 out of 5 (40%) questions correct, and results did not differ by year of training (P=0.31). Of the participants (n=70) who completed the 5-day challenge and responded to the post-challenge survey, the distribution of the number of correct answers improved after the KDC (median [25th, 75th percentile]: 3 [2, 3] versus 3 [2, 4]; P<0.001). Statistics from our official hashtag for this study (#kidneydietchallenge) showed that we achieved 406,241 reaches and 1,004,799 impressions, with a total of 974 posts using this hashtag. Conclusions: The KDC is an immersive, experiential educational tool that enabled a global population to learn how to counsel their patients better about adherence to a complex kidney diet.

Glomerular Disease Education Experience across Nephrology Fellowship Programs: An International Survey.

Introduction: Glomerulonephritis (GN) education is an important, albeit a challenging, component of nephrology fellowship training. We hypothesized that trainee experience varies widely across programs, leading to differences in self-reported competency levels in the diagnosis and management of glomerular diseases. Methods: The Glomerular Disease Study & Trial Consortium (GlomCon) conducted an anonymous online survey to determine the educational experience of nephrology trainees. We used multiple-choice questions to obtain data about (a) curriculum-based education, (b) dedicated specialty clinic, and (c) exposure to pathology. We leveraged a visual analog scale of 1-100 (with a higher number indicating a higher comfort level) to assess self-reported levels of clinical competency. The survey was disseminated via email to the subscribing members of GlomCon and through Twitter. Results: In total, there were 109 respondents to our survey, and 56% were from training programs in the USA. Exposure to a specialized GN clinic was reported by 45%, while 77% reported the presence of an onsite nephropathologist at their training program. Self-reported competency scores were 59 ± 25 and 52 ± 25 for diagnosis and treatment of glomerular diseases, respectively. Days spent in a GN clinic per year, years of fellowship, and dedicated nephropathology didactics were associated with higher diagnosis and treatment competency scores. Conclusion: Trainees report a wide variation in glomerular disease education across fellowship programs. A lack of nephropathology exposure and a dedicated GN curriculum was associated with lower scores in self-reported clinical competency in caring for patients with glomerular disease.

Impact of the COVID-19 Pandemic on Nephrology Fellow Training and Well-Being in the United States: A National Survey.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic's effects on nephrology fellows' educational experiences, preparedness for practice, and emotional wellbeing are unknown. METHODS: We recruited current adult and pediatric fellows and 2020 graduates of nephrology training programs in the United States to participate in a survey measuring COVID-19's effects on their training experiences and wellbeing. RESULTS: Of 1005 nephrology fellows-in-training and recent graduates, 425 participated (response rate 42%). Telehealth was widely adopted (90% for some or all outpatient nephrology consults), as was remote learning (76% of conferences were exclusively online). Most respondents (64%) did not have in-person consults on COVID-19 inpatients; these patients were managed by telehealth visits (27%), by in-person visits with the attending faculty without fellows (29%), or by another approach (9%). A majority of fellows (84%) and graduates (82%) said their training programs successfully sustained their education during the pandemic, and most fellows (86%) and graduates (90%) perceived themselves as prepared for unsupervised practice. Although 42% indicated the pandemic had negatively affected their overall quality of life and 33% reported a poorer work-life balance, only 15% of 412 respondents who completed the Resident Well-Being Index met its distress threshold. Risk for distress was increased among respondents who perceived the pandemic had impaired their knowledge base (odds ratio [OR], 3.04; 95% confidence interval [CI], 2.00 to 4.77) or negatively affected their quality of life (OR, 3.47; 95% CI, 2.29 to 5.46) or work-life balance (OR, 3.16; 95% CI, 2.18 to 4.71). CONCLUSIONS: Despite major shifts in education modalities and patient care protocols precipitated by the COVID-19 pandemic, participants perceived their education and preparation for practice to be minimally affected.

Back off baclofen when the kidneys don't work.

Medication adverse events are common and often preventable. Patients with diminished kidney function are particularly susceptible to adverse events, especially if the medication's primary means of elimination is through the kidneys. Neurotoxicity from baclofen (80% excreted by the kidneys) is increasingly being recognized in patients with diminished kidney function. Chauvin et al. performed a large population-based retrospective cohort study in patients with end-stage kidney disease (ESKD) and showed a high rate of encephalopathy with hospitalizations shortly after baclofen exposure. This commentary discusses the high rate of altered mental status after baclofen exposure in patients with ESKD, potential reasons for the continued reports of this adverse event, and strategies to educate the health care community.

Bundled Payment Reform and Dialysis Facility Closures in ESKD.

BACKGROUND: In 2011, inclusion of injectable medications into an expanded ESKD payment bundle prompted concerns that dialysis facilities facing higher costs might close, disrupting care delivery and access to care. Whether this policy change influenced dialysis facility closures is unknown. METHODS: To examine whether facility closures increased after 2011 and whether factors influencing closures changed, we analyzed US Renal Data System registry data to identify all patients receiving in-center hemodialysis from 2006 through 2015 and to track dialysis facility closures. We used interrupted time series logistic regression models and estimated marginal effects to examine immediate and longer-term changes in the likelihood of being affected by facility closures following payment reform. We also examined whether associations between selected predictors of closures indicating populations at "high risk" of closure (patient characteristics, facility characteristics, and geography-related characteristics) and closures changed after payment reform. RESULTS: Dialysis facility closures were uncommon over the study period. In adjusted models, the relative odds of experiencing a closure declined by 37% (odds ratio [OR], 0.63; 95% confidence interval [95% CI], 0.59 to 0.67) immediately after payment reform and declined by an additional 6% (OR, 0.94; 95% CI, 0.91 to 0.97) annually thereafter, corresponding to a 0.3% lower absolute probability of closure in 2015 in association with payment reform. Patients who were black and who dialyzed at small, hospital-based facilities experienced slight increases in closures following payment reform, whereas Hispanic and Medicare/Medicaid dual-eligible patients experienced slight decreases in closures. CONCLUSIONS: Expansion of the ESKD payment bundle was not associated with increased closure of dialysis facilities, although the likelihood of closures changed slightly for some higher-risk populations.

Defining the best practice patterns for the neonatal systemic-to-pulmonary artery shunt procedure.

OBJECTIVES: To assess variation in outcome measures and their associations with shunt thrombosis prophylaxis regimens after systemic-to-pulmonary artery shunt surgery across centers in the United States participating in the Pediatric Health Information System database. METHODS: We reviewed data on neonates who underwent an off-pump shunt procedure at 39 centers between 2000 and 2011. The overall variation in rates of discharge mortality and shunt-related complications were assessed by funnel plots. Complications were defined as revision/repeat of the shunt procedure during the same admission, institution of extracorporeal membrane oxygenation after surgery, and catheter interventions after shunt surgery. Bayesian hierarchical modeling was used to identify outliers. Shunt thrombosis prophylaxis regimens including the time of initiation of aspirin were compared between high and low outliers. RESULTS: A total of 2058 index operations were identified. Funnel plots highlighting the outcomes from various centers allowed discrimination of discharge mortality and complication rates around an aggregate of 6.7% and 12.3%, respectively. Bayesian modeling showed the presence of substantial variation in complication rates between centers; 20% of them were identified as outliers. Aspirin was initiated significantly earlier during the hospital course in centers with a lower composite rate of complications than those with higher rates (median initiation day of 2 [interquartile range (IQR), 1-3] in low outliers vs 4 [IQR, 3-6] in high outliers; P < .001). CONCLUSIONS: A substantial variation was found between hospitals in the rate of shunt-related complications. Centers with best outcomes implement aspirin earlier in their postoperative shunt thrombosis prophylaxis regimen.

Spondylodiscitis: a rare complication of button battery ingestion in a 10-month-old boy.

Nowadays, button battery ingestion accounts for one of the common foreign body ingestions among pediatric population. Unexpected manifestations in this setting, particularly after late removal, are confusing and might be misleading and postpone the proper management. We report a case of button battery ingestion in a 10-month-old boy who was primarily considered uncomplicated. However, he was readmitted with neck stiffness, which later turned to be due to spondylodiscitis. Based on this report, we strongly recommend considering spondylodiscitis as a late but serious complication of button battery ingestion in any patient who presents with restricted neck movement following foreign body ingestion.

Bacillus Calmette-Guérin (BCG) complications associated with primary immunodeficiency diseases.

Primary immunodeficiency diseases (PIDs) are a group of inherited disorders, characterized by defects of the immune system predisposing individuals to variety of manifestations, including recurrent infections and unusual vaccine complications. There are a number of PIDs prone to Bacillus Calmette-Guérin (BCG) complications. This review presents an update on our understanding about the BCGosis-susceptible PIDs, including severe combined immunodeficiency, chronic granulomatous disease, and Mendelian susceptibility to mycobacterial diseases.

Upper endoscopic findings in children with recurrent abdominal pain: high prevalence of hiatus hernia.

OBJECTIVE: Recurrent abdominal pain (RAP) by itself is one of the common reasons in child-aged patients to refer to a clinician. Some of these patients are presented with more serious features, so-called the "red flag". The most important issue in management of RAP is to distinguish the type of it, whether it is functional or organic. In this study we aimed to assess the redundancy of red-flagged RAP with findings of esophago-gastro-deudonoscopy. METHODS: In a 2 year prospective study 150 consecutive children with RAP who showed red flags underwent esophago-gastro-deudonoscopy. The prevalence of each finding was recorded. Overall positive predictive value of predicting an endoscopic finding while having a red-flag was calculated. FINDINGS: Among all the patients, 126 cases showed at least a positive finding in their endoscopy that corresponded to the positive predictive value of 84% for predicting the presence of an endoscopic finding according to red flags. Interestingly, 20% of patients showed hiatus hernia when surveyed. CONCLUSION: Comprehensive physical examination is needed to avoid performing esophago-gastro-deudonoscopy without indication in patients with recurrent abdominal pain.

Disseminated BCG as a unique feature of an infant with severe combined immunodeficiency.

Severe combined immunodeficiency (SCID) is a rare primary immunodeficiency disease, which renders patients prone to recurrent severe infections in early childhood. Herein, we present a five-month-old boy with SCID who was referred to our center with recurrent diarrhea, respiratory infection and lymphadenopathy. Immunological studies showed hypogammaglobulinemia and low number of T-cells, which was compatible with the diagnosis of T- B+ SCID. An advanced cytomegalovirus pneumonitis was detected based on the results of lung necropsy. Cultures and polymerase chain reaction studies of bone marrow aspirates and spleen specimen were indicative of Mycobacterium bovis. This report emphasizes the importance of lymphadenopathy as a sentinel sign of immunological disorders. Underlying immunodeficiency diseases such as SCID should be considered in the differential diagnosis of an infant with infections and lymphadenopathy, particularly in the regions with routine national Bacillus Calmette-Guérin (BCG) vaccination.

BCGiosis as a presenting feature of a child with chronic granulomatous disease.

Bacillus Calmette Guerin (BCG) vaccine, which is administered to all newborns in some regions, could lead to serious complication ranging from local disease (known as BCGitis) to disseminated disease (BCGosis) in a group of patients with primary immunodeficiency diseases. We are reporting here a 3.5 year-old girl with a history of prolonged BCGitis, which developed to disseminated disease without any other special features. Immunological studies with nitro-blue tetrazolium test confirmed the diagnosis of chronic granulomatous disease in this patient. Chronic granulomatous disease should be considered in the list of differential diagnosis in all children with BCGosis, even in the absence of any other manifestations related to immunodeficiency.

BCGiosis as a presenting feature of a child with chronic granulomatous disease

Bacillus Calmette Guerin (BCG) vaccine, which is administered to all newborns in some regions, could lead to serious complication ranging from local disease (known as BCGitis) to disseminated disease (BCGosis) in a group of patients with primary immunodeficiency diseases. We are reporting here a 3.5 year-old girl with a history of prolonged BCGitis, which developed to disseminated disease without any other special features. Immunological studies with nitro-blue tetrazolium test confirmed the diagnosis of chronic granulomatous disease in this patient. Chronic granulomatous disease should be considered in the list of differential diagnosis in all children with BCGosis, even in the absence of any other manifestations related to immunodeficiency.