Late gadolinium enhancement in cardiac magnetic resonance imaging is associated with high renal resistive index in patients with systemic sclerosis

Introduction: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by microvascular damage and fibrosis of the skin and internal organs. The major complications are lung fibrosis, pulmonary artery hypertension, scleroderma renal crisis, and cardiac involvement. Objective: The aim of this study was to assess renal and cardiac involvement in asymptomatic SSc patients using renal Doppler ultrasound (RDU) and cardiac magnetic resonance (CMR). Materials and Methods: We enrolled 26 consecutive SSc patients (21 female) according to 2013 ACR/EULAR criteria. Biochemical analysis, clinical evaluation, RDU with intrarenal hemodynamic parameters (renal resistive index [RRI], pulsatility index [PI], systolic/diastolic [S/D] ratio), and CMR with late gadolinium enhancement (LGE) were investigated at the time of enrollment. Results: The median PI value was significantly (p = 0.007) higher in SSc patients with LGE than in SSc patients without LGE (1.37 [1.28-1.58] vs. 1.12 [1.06-1.26]). The median RRI value was significantly (p = 0.002) higher in SSc patients with LGE than in SSc patients without LGE (0.68 [0.65-0.73] vs. 0.64 [0.63-0.65]). The median S/D ratio was significantly (p = 0.02) higher in SSc patients with LGE than in SSc patients without LGE (3.12 [2.83-3.76] vs. 2.78 [2.64-2.84]). Conclusions: Our study, although performed on a small SSc population, showed RRI and LGE as markers of vascular and fibrotic damage. Early detection of cardiorenal involvement in SSc patients without symptoms is important to avoid further complications

Acute renal failure and hypercalcemia as onset in splenic lymphoma

Hypercalcemia is a rare metabolic disorder in course of B cell lymphoma. The mechanism of hypercalcemia in patients with malignancy may include the increased extrarenal production of vitamin D from tumoral cells or neighboring macrophages, i-PTH or PTHrP from tumoral cells. In this case we reported a 34 years old caucasian woman with acute renal failure and hypercalcemia as onset of splenic lymphoma in absence of abnormal levels of serum vitamin D and PTHrP. Because of dramatic recovery of renal function and hypercalcemia after splenectomy, we can speculate that main mechanism of hypercalcemia is related to vitamin D production from neighboring lymphoma macrophages

NEPHROTIC SYNDROME AND STROKE

The hypercoagulability of patients with nephrotic syndrome could be an important trigger for arterial and venous thrombotic events. Arterial thromboses are less frequent than venous thromboses and the most common locations are femoral arteries. The association of stroke and nephrotic syndrome is extremely rare. Here we report the case of a patient with stroke as first manifestation of nephrotic syndrome. Ischemic stroke can be the first manifestation of nephrotic syndrome and should be considered as a possible complication of the syndrome, when the commonest causes of ischemic stroke are excluded and especially in presence of pre-existing glomerular disease

Ask-Upmark Kidney and Tubulointerstitial Nephritis in a Woman with Severe Renal Failure

Ask-Upmark kidney is a rare diagnosis of segmental hypoplasia in pediatric population clinically characterized by severe hypertension potentially treatable with partial to total nephrectomy. Although originally was described only as a congenital anomaly, recent data suggest to be caused by vesicoureteral reflux, either in utero or in early childhood and pyelonephritis. The case we reported indicates that Ask-Upmark kidney should be considered as potential cause of hypertension and renal failure both in children and adults. The renal biopsy is necessary for early diagnosis and may consent to normalize blood pressure with nephrectomy; however, if renal damage is severe and progressive with tubulointerstitial nephritis, surgical management is excluded and renal transplant should be considered

ACTH AND AZATHIOPRINE: ANTIPROTEINURIC AND LIPID-LOWERING EFFECT IN THE COURSE OF IDIOPATHIC MEMBRANOUS GLOMERULONEPHRITIS

Idiopathic membranous glomerulonephritis is a frequent cause of nephrotic syndrome and may have a variable course, from spontaneous remission to progression on renal failure. The therapy is based on alternating steroids and chlorambucil or cyclophosphamide (Ponticelli protocol) for six months. In absence of complete or partial remission after protocol, cyclosporine, adrenocorticotropic hormone, mycophenolate mofetil, rituximab can be used for potential therapy. We report here the case of a woman with idiopathic membranous glomerulonephritis unresponsive to the Ponticelli regimen and treated with adrenocorticotropic hormone in association with azathioprine, showing a dramatic decrease of proteinuria and beneficial effects on lipid profile. After 36 months, no relapse of disease has occurred. Although larger cohorts of patients are needed to evaluate the long-term effects, adrenocorticotropic hormone plus azathioprine in association could be a possible therapeutic option for unresponsive idiopathic membranous glomerulonephritis

Liver abscess

Liver abscess is a rare and severe infection. Incidence increases because of aging of population, advances in liver and biliary surgery including liver transplantation, and immunodeficiency factors. Diagnosis depends mainly on imaging and needle aspiration for microbiological identification. Treatment is based on antibiotics, percutaneous or surgical drainage, and control of the primary source

Cytokines expression in SLE nephritis

Renal involvement is a common manifestation in course of systemic lupus erythematous (SLE) and may occur at any time. In SLE nephritis, the pattern of glomerular injury is primarily related to the formation of the immune deposits in situ, due major to antidouble-stranded DNA (anti-dsDNA) antibodies and anti- C1q. Immune complexes deposits can induce the inflammatory response by activation of adhesion molecules on endothelium, resulting in the recruitment of pro inflammatory leukocytes. Activated and damaged glomerular cells, infiltrating macrophages, B and T cells produced cytokines that play a pivotal role as inflammatory mediators to extend renal injury. In serum of SLE patients, the concentrations of IL-6, IL-17, IL-12, INF-gamma, IL-18, IL-10 and TNF-alpha are higher than healthy people and this increase correlate with disease activity. It is well established possible correlation between urinary cytokines levels (IL-6, IL-10, INF-gamma and TGF-beta) and disease activity. In fact, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) correlate with cytokines over-expression, in particular IL-17, IL-10, TNF-alpha and the axis INF-gamma/IL-12. Recent studies are promising about proteinuria reduction and improving renal function through cytokine blockade therapy

Malnutrition is prevalent in patients with cardiorenal syndrome and negatively influences clinical outcome

Cardiorenal syndrome (CRS) describes the concurrent failure of cardiac and renal function, each influencing the other. Malnutrition and cachexia frequently develop in patients with heart failure or kidney failure. However, no information is currently available on the prevalence of malnutrition in CRS patients. We studied CRS patients admitted to an internal medicine ward during a 5-month period and evaluated their clinical characteristics and nutritional status. Malnutrition risk was assessed by using the validated screening tool NRS-2002 whilst body composition was assessed by bioimpedance analysis and muscle function was measured by handgrip (HG) strength. Cardiac mass was also recorded. Length of stay, hospital readmission and 6-month mortality were registered. During the study period, 22 CRS patients were studied. Twenty patients were diagnosed with either CRS type 1 or CRS type 5. In CRS patients, fat-free mass showed a trend toward representing a protective factor for 6-month mortality (OR=0.904; p=0.06). Also, fat-free mass correlated with HG strength and cardiac ejection fraction. Malnutrition risk was diagnosed in 45% of the patients, whereas 8 patients met the definition of cachexia. Even without statistical significance, CRS patients with malnutrition had lower BMI (Body Mass Index) (p=0.038) and fat-free mass (p= n.s.). However, CRS malnutrition was associated to higher 6-month mortality (p= 0.05), and appears to negatively influence the outcome in CRS (OR= 9; p= 0.06). Our results show that malnutrition is prevalent in CRS patients and influences the clinical outcome. The assessment of nutritional status, and particularly body composition, should be implemented in daily practice of patients with CRS

Nutritional status and intrarenal arterial stiffness in cardiorenal syndrome: a pilot study

Cardio-Renal Syndrome (CRS) is a condition, which is more frequently observed in clinical practice. The aim of this study is to explore nutritional status and intrarenal arterial stiffness in patients affected by CRS

Rhabdomyolysis after midazolam administration in a cirrhotic patient treated with atorvastatin.

The administration of statins in patients with liver disease is not an absolute contraindication. Hepatotoxicity is a rare and often dose-related event and in the literature there are only a few described cases of fatal rhabdomyolysis in patients with chronic liver disease after statin administration. During treatment with 3-hydroxy-3-methylglutaryl-coenzyme A reductase inhibitors, the factors responsible for myopathy may either be related to the patient, or due to interactions with other medications that are metabolic substrates of the same isozymes and therefore able to increase blood statin concentration. The most important side effects consist of increased transaminase levels, abdominal pain or muscle weakness, increased serum levels of creatine kinase and rhabdomyolysis. In this article we report a case of fatal rhabdomyolysis with acute renal failure after gastric endoscopy, where midazolam was used as a sedation agent in a patient with chronic liver disease treated with a high dose of atorvastatin. Therefore, we suggest paying particular attention to the potential risks of associating atorvastatin and midazolam in patients with chronic liver disease who need to undergo gastric endoscopy