Atypical hemolytic uremic syndrome (HUS) is extremely rare in adults. HUS is characterized by hallmark features of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal injury. Atypical HUS (aHUS) is caused by uncontrolled complement activation. The complement activation can be triggered by infections such as Streptococcus pneumoniae or influenza, pregnancy, malignancy, cytotoxic drugs, organ transplants, or autoimmune diseases. Genetic mutations and autoantibodies have been found to play a crucial role in the pathogenesis of dysregulated complement activity. The majority of cases of atypical HUS due to invasive S. pneumoniae infection are more commonly seen in children. We present a case of S. pneumoniae HUS (Sp-HUS) presenting with multiorgan failure, disseminated intravascular coagulation (DIC), and limb ischemia in an adult. This case highlights the importance of considering S. pneumoniae HUS (Sp-HUS) in the differential diagnosis of thrombotic microangiopathies (TMA) in adults.

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OBJECTIVE: Vascular calcification is a frequently occurring complication of end-stage renal disease (ESRD). This study focused on the significance of long non-coding RNA Fas cell surface death receptor-antisense 1(lncRNA FAS-AS1) in ESRD-related vascular calcification aiming to explore a potential biomarker for the detection.
METHODS: The study enrolled 65 healthy individuals, 79 ESRD patients (48 patients with vascular calcification), and 93 early-stage (I-IV) chronic kidney disease (CKD) patients. The expression of FAS-AS1 in serum was evaluated by real-time quantitative polymerase chain reaction (PCR). The diagnostic potential of FAS-AS1 was assessed in discriminating ESRD patients, vascular calcification, and the severity of vascular calcification. In vitro, the vascular smooth muscle cells (VSMCs) were treated with a hyperphosphatemia medium to evaluate the effect of FAS-AS1 on VSMCs calcification.
RESULTS: Elevated serum FAS-AS1 was observed in ESRD patients, which could discriminate from healthy individuals and early-stage CKD patients. FAS-AS1 was associated with the development of ESRD and the occurrence of vascular calcification. FAS-AS1 was also upregulated in vascular calcification patients, especially the patients with severe calcification, which showed diagnostic significance in evaluating vascular calcification degrees. Calcified VSMCs showed significantly increased levels of Ca2+, reactive oxygen species (ROS), tumor necrosis factor-α (TNF-α), and interleukin 6 (IL-6), which was attenuated by silencing FAS-AS1.
CONCLUSIONS: FAS-AS1 discriminated ERSD patients and was associated with the occurrence of vascular calcification. The knockdown of FAS-AS1 suppressed hyperphosphatemia-induced vascular calcification via alleviating oxidative stress and inflammation.

Gemcitabine-induced hemolytic uremic syndrome is an often-missed condition. We present a case outlining the successful management of a patient with metastatic cholangiocarcinoma treated with gemcitabine who subsequently developed hemolytic uremic syndrome. Early recognition and stopping gemcitabine are essential in this patient population. Complement inhibitors have been used, and our patient improved on eculizumab therapy.

UNASSIGNED: To investigate the predictive value of serum d-serine level for hearing impairment (HI) in uremic patients.
UNASSIGNED: In this study, 30 uremic patients with HI and 30 with normal hearing were selected. The basic conditions, biochemical indicators, and serum serine levels of the two groups were compared to analyze the influencing factors of HI.
UNASSIGNED: The age and d-serine levels were higher in the HI group, while the l-serine level was lower than uremia in the normal hearing group. Logistic regression analysis showed that d-serine level ≥10 μM and older age increased the risk of HI. The area of the receiver operating characteristic (ROC) curve drawn by the prediction probability of HI was 0.838, indicating that age, d-serine, and l-serine had predictive diagnostic values for HI (p < .001). Among these, the ROC curve area of d-serine in predicting HI in uremic patients was 0.822 (p < .001).
UNASSIGNED: Increased d-serine and age are two risk factors for HI, while l-serine is a protective factor. d-Serine level has a predictive value for HI in uremic patients. Uremic patients are recommended hearing assessment, estimation of d-serine levels, and early intervention.

UNASSIGNED: Xerosis and pruritus are common chronic dermatological disorders among dialysis and diabetic patients that are frequently underdiagnosed or neglected, which can impact the quality of life of these patients. This study aimed to evaluate the efficacy and safety of a specific dermo-cosmetic product in the treatment of dry skin and pruritus associated with dialysis and diabetes.
UNASSIGNED: Twenty-nine dialysis patients (mean age 62 years) and 40 diabetic patients (mean age 57 years, 88% type 2) were included in two different single-center open-label uncontrolled clinical trials. All patients presented skin dryness according to the Scaling Roughness Redness and Cracks (SRRC) scale, and pruritus and/or insomnia. They applied the dermo-cosmetic product Medi-Secure Atoderm Xereane (NAOS, Laboratoire Bioderma) once or twice a day. The clinical efficacy (SRRC, pruritus, and insomnia), the skin-related quality of life (Dermatological Life Quality Index, DLQI), and the subjective efficacy were assessed at the inclusion visit and after 28 days of product application, as well as the safety.
UNASSIGNED: After 28 days of application, the product significantly reduced the SRRC global score of 83% (0.9±0.8 vs 5.1±1.2) and 66% (1.4±1.2 vs 4.2±0.5), pruritus intensity of 76% (1.1±1.3 vs 4.6±2.1) and 78% (0.9±1.7 vs 4.2±2.6), and insomnia intensity of 61% (0.9±1.3 vs 2.4±2.3) and 82% (0.9±1.7 vs 4.8±2.7) in dialysis and diabetic patients, respectively. Furthermore, the product\'s application led to an improvement of the skin-related quality of life of 50% (5.4 vs 2.7; p<0.0001) in dialysis patients and 71% (6.6 vs 1.9; p<0.0001) in diabetic patients at D28. In addition, the product was greatly appreciated by all patients for its soothing, comforting, repairing, nourishing, and hydrating effects and was very well tolerated by the entire panels.
UNASSIGNED: This specific dermo-cosmetic product significantly reduces skin dryness, pruritus, and insomnia in dialysis and diabetic patients, thereby greatly improves their skin-related quality of life. By managing and avoiding bothersome symptoms associated with their disease or treatment, this ecobiological dermo-cosmetic can prevent serious complications that constitute a substantial burden on their daily life.

Uremic calciphylaxis is a rare disease that affects patients with chronic end-stage renal disease. It is a pathology of the microvessels of the dermis and hypodermis which are calcified and whose thrombosis leads to skin necrosis. Calciphylaxis lesions can be distal and axial. They lead to pain, infection and are associated with denutrition and in high mortality rate (40-80% at 1 year). This general review describes the clinical and para-clinical presentations of calciphylaxis. It summarizes the current knowledge on its pathogenesis and the therapeutical options that can be proposed to improve the management and attempt to reduce the mortality of patients with uremic calciphylaxis.

Skeletal changes are a common complication in patients with chronic kidney disease and traditionally labelled as renal osteodystrophy. Uremic leontiasis ossea is a rare and severe form of renal osteodystrophy with characteristic overgrowth of the craniofacial bones. Imaging, in particular computed tomography, is valuable for the diagnosis and management of such rare condition. Uremic leontiasis ossea has distinctive imaging features with significant overgrowth of the jaw and characteristic internal serpiginous tunneling. The recognition of its radiological appearance and abrupt management are essential to avoid its devastating esthetic and functional impairments.

While renal osteodystrophy is a common complication of chronic renal failure which is caused by secondary hyperparathyroidism, it is rare that the bony changes result in a severe progressive overgrowth of the bones of the face such that the patient is at risk for breathing and feeding difficulties. When this occurs, it is called uremic leontiasis ossea and patients who suffer from this rare, severe complication of renal osteodystrophy may go undiagnosed or be misdiagnosed resulting improper management due to its limited discussion in the literature. We report a case of a 42-year-old man with end-stage renal disease who was unable to receive dialysis consistently for many years who was found to have a large hard mass on the palate and palate ulcers.

Calciphylaxis is a rare thrombotic vasculopathy characterized by high morbidity and mortality. There is a paucity of studies examining longitudinal outcomes.
To assess mortality, days spent in the hospital, and amputations in patients with calciphylaxis.
A retrospective medical record review was conducted in 145 patients diagnosed with calciphylaxis at an urban tertiary care hospital from January 2006 to December 2018.
Six-month mortality was 37.2%, and 1-year mortality was 44.1%. Patients with nephrogenic calciphylaxis had worse survival than those with nonnephrogenic calciphylaxis (P = .007). This difference in survival disappeared when limiting mortality to deaths due to calciphylaxis. Age (P = .003) and end-stage renal disease (P = .01) were risk factors associated with 1-year mortality. Diabetes mellitus was associated with greater total hospitalization days (coefficient, 1.1; 95% confidence interval, 1.01-1.4); bedside debridement was associated with fewer hospitalization days (coefficient, 0.8; 95% confidence interval, 0.7-0.9). Amputations were not associated with any of the examined risk factors. The use of warfarin followed by a transition to nonwarfarin anticoagulation was associated with decreased hazard of death (P = .01).
Retrospective nature.
Calciphylaxis remains a complex, heterogeneous disease. Mortality is lower in patients with nonnephrogenic disease. These findings may be incorporated during discussions regarding the goals of care to facilitate informed shared decision making.