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.

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.

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.

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.

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.