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.

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: 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.

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.

Appropriate nutraceutical combinations may represent a valid approach to prevent vascular calcification associated with chronic kidney disease (CKD). In the present study, we tested the effect of a new nutraceutical combination named RenaTris®, containing MK-7, magnesium carbonate, and Sucrosomial® Iron, on vascular calcification in uremic rats. Rats were randomly divided into three groups, i.e. control (high-phosphate diet), uremic (high-phosphate diet containing 0.5% adenine), and supplemented uremic diet (0.5% adenine, MK-7, magnesium carbonate, and Sucrosomial® Iron). After six weeks, sera and vascular calcification were examined. The uremic diet increased creatinine and phosphate levels and induced extensive vascular calcification. The uremic condition also induced a mild hypercholesterolemic condition (+52% of total cholesterol; p < 0.05). The supplemented uremic diet did not reduce creatinine, phosphate levels, or vascular calcification, however, we observed a significant hypocholesterolemic effect (-18.9% in supplemental uremic vs. uremic diet; p < 0.05). Similar to simvastatin, incubation of cultured human hepatoma cells (Huh7) with MK-7 significantly reduced cholesterol biosynthesis (-38%) and induced 3-hydroxy-3-methyl-glutaryl-CoA (HMG-CoA) reductase and low-density lipoprotein receptor (LDLR) at both mRNA and protein levels. The effect of MK-7 on LDLR was counteracted by the co-incubation with squalene. Unlike simvastatin, MK-7 reduced PCSK9 in Huh7. These results indicated that the new nutraceutical combination significantly impacts cholesterol metabolism and its supplementation may help to control mild hypercholesterolemic conditions in CKD patients.

Pruritus is one of the most common and disturbing symptoms in hemodialysis (HD) patients. The pathogenesis of pruritus in HD patients is multifactorial; however, a little progress in understanding this pathogenesis has been achieved.
To evaluate the frequency and risk factors of pruritus among HD patients in Dakahlia, Egypt.
A total of 193 patients from 4 HD centers were included in this study. Pruritus intensity was assessed by visual analog scale. All patients were assessed by clinical and laboratory parameters, such as age, sex, duration of dialysis, dialysis adequacy, urea, calcium, phosphorus, parathyroid hormone, fasting insulin, fasting sugar, HOMA-IR, serum ferritin, high-sensitive C-reactive protein (hs-CRP) and hemoglobin. They were also investigated for hepatitis C virus (HCV) infection by HCV enzyme-linked immunosorbent assay, HCV-PCR for plasma and buffy coat for further detection of occult HCV infection.
Male gender, dialysis duration, inadequate dialysis, anemia, high ferritin, hyperphosphatemia, hypocalcemia, hs-CRP, and insulin resistance were characteristic features in pruritic patients (p = 0.01, 0.006, 0.0001, 0.047, 0.01, 0.0001, 0.024, 0.000, and 0.0001, respectively). Significant positive correlations were found between pruritus score and each of age (p = 0.002, r = 0.222), duration of dialysis (p = 0.03, r = 0.151), serum ferritin (p = 0.001, r = 0.213), serum phosphorus (p = 0.0001, r = 0.59), fasting insulin (p = 0.001, r= 0.273), and HOMA-IR (p = 0.0001, r = 0.349), while there was a negative correlation with Kt/V (p= 0.0001, r = -0.459). Linear multivariate regression analysis showed that age, duration of dialysis, serum phosphorus, Kt/v, and hs-CRP were good predictors for pruritic score in HD patients. All HCV-infected patients (who were positive for both plasma and buffy coat HCV-PCR) had pruritus with -significantly higher pruritus score than non-infected patients (p = 0.009), they also showed significantly higher fasting insulin, HOMA-IR, and hs-CRP levels (p = 0.0001).
Uremic pruritus (UP) is a serious problem in HD patients. hs-CRP, male gender, dialysis duration, insulin resistance, dialysis inadequacy, and hyperphosphatemia are positively correlated with the intensity of UP. HCV infection is associated with severe UP, insulin resistance, and inflammation.

UNASSIGNED: Uremic pruritus is a common and disturbing problem in hemodialysis patients. Although its pathogenesis is not completely understood, it is thought to be multifactorial. The aim of this study was to identify risk factors of uremic pruritus in hemodialysis patients.
UNASSIGNED: A total of 249 patients from four dialysis centers were included in this study. Data were collected using a questionnaire, the visual analogue scale, and the Hospital Anxiety and Depression Scale. We investigated whether socio-demographic and biochemical parameters were correlated to uremic pruritus.
UNASSIGNED: Pruritus was present in 53.4% of the hemodialysis patients. The mean visual analogue scale severity was 6.47 ± 1.56. Patients with white blood cell (WBC) counts > 6.7 × 103/μL had 1.73 times (95% confidence interval [CI], 1.360-2.888; P = 0.036) more pruritus than did those with WBC counts < 6.7 × 103/μL. Patients with dry skin were 0.2 times (95% CI, 0.070-0.182; P = 0.028) more likely to suffer from very severe pruritus than were those with normal skin.
UNASSIGNED: Uremic pruritus remains a serious problem in dialysis patients. The WBC level and presence of dry skin are thought to be among its causes. Therefore, data regarding the possible risk factors of uremic pruritus must be followed closely in patients at risk.

BACKGROUND: Systemic lupus erythematosus (SLE) is characterized by various clinical manifestations and immunologic abnormalities. Cardiovascular and respiratory system involvement are increasingly recognized as critical for patients\' prognosis. In this review, current knowledge concerning diagnosis, pathogenesis and treatment of the cardiac and pulmonary lupus manifestations are discussed.
METHODS: Review of the literature.
RESULTS: Although pericarditis is the most frequent heart manifestation in the context of lupus, valvular disease and less often myocarditis may be detected. In the latter, treatment should be prompt and aggressive to prevent chronic sequelae like congestive heart failure. Later on disease course, accelerated atherosclerosis is considered as one of the most important co-morbidities of SLE with cardiovascular events being one of the leading causes of death at relatively young ages. Stratification of the patients at risk and stringent management of the traditional risk factors are warranted. Respiratory system involvement affects all anatomic structures of the lungs, pleura and pulmonary vasculature while its severity ranges from asymptomatic pleural disease to acute respiratory failure. The most common features include pleuritis, interstitial lung disease and pulmonary embolism on the background of antiphospholipid syndrome. Less usual complications include lupus pneumonitis, diffuse alveolar hemorrhage, shrinking lung syndrome and pulmonary arterial hypertension.
CONCLUSIONS: There are no specific guidelines for the management of these manifestations and therapeutic approach remains empiric.