Effects of hormonal changes on sarcopenia in chronic kidney disease: where are we now and what can we do?

Sarcopenia or muscle wasting is a progressive and generalized skeletal muscle disorder involving the accelerated loss of muscle mass and function, often associated with muscle weakness (dynapenia) and frailty. Whereas primary sarcopenia is related to ageing, secondary sarcopenia happens independent of age in the context of chronic disease states such as chronic kidney disease (CKD). Sarcopenia has become a major focus of research and public policy debate due to its impact on patient's health-related quality of life, health-care expenditure, morbidity, and mortality. The development of sarcopenia in patients with CKD is multifactorial and it may occur independently of weight loss or cachexia including under obese sarcopenia. Hormonal imbalances can facilitate the development of sarcopenia in the general population and is a common finding in CKD. Hormones that may influence the development of sarcopenia are testosterone, growth hormone, insulin, thyroid hormones, and vitamin D. Although the relationship between free testosterone level that is low in uraemic patients and sarcopenia in CKD is not well-defined, functional improvement may be seen. Unlike testosterone, it is known that vitamin D is associated with muscle strength, muscle size, and physical performance in patients with CKD. Outcomes after vitamin D replacement therapy are still controversial. The half-life of growth hormone (GH) is prolonged in patients with CKD. Besides, IGF-1 levels are normal in patients with Stage 4 CKD-a minimal reduction is seen in the end-stage renal disease. Unresponsiveness or resistance of IGF-1 and changes in the GH/IGF-1 axis are the main causes of sarcopenia in CKD. Low serum T3 level is frequent in CKD, but the net effect on sarcopenia is not well-studied. CKD patients develop insulin resistance (IR) from the earliest period even before GFR decline begins. IR reduces glucose utilization as an energy source by hepatic gluconeogenesis, decreasing muscle glucose uptake, impairing intracellular glucose metabolism. This cascade results in muscle protein breakdown. IR and sarcopenia might also be a new pathway for targeting. Ghrelin, oestrogen, cortisol, and dehydroepiandrosterone may be other players in the setting of sarcopenia. In this review, we mainly examine the effects of hormonal changes on the occurrence of sarcopenia in patients with CKD via the available data

The Mitochondrion: A Promising Target for Kidney Disease

Acute kidney injury; Chronic kidney disease; Mitochondrial dysfunctionLesión renal aguda; Enfermedad renal crónica; Disfunción mitocondrialLesió renal aguda; Malaltia renal crònica; Disfunció mitocondrialMitochondrial dysfunction is important in the pathogenesis of various kidney diseases and the mitochondria potentially serve as therapeutic targets necessitating further investigation. Alterations in mitochondrial biogenesis, imbalance between fusion and fission processes leading to mitochondrial fragmentation, oxidative stress, release of cytochrome c and mitochondrial DNA resulting in apoptosis, mitophagy, and defects in energy metabolism are the key pathophysiological mechanisms underlying the role of mitochondrial dysfunction in kidney diseases. Currently, various strategies target the mitochondria to improve kidney function and kidney treatment. The agents used in these strategies can be classified as biogenesis activators, fission inhibitors, antioxidants, mPTP inhibitors, and agents which enhance mitophagy and cardiolipin-protective drugs. Several glucose-lowering drugs, such as glucagon-like peptide-1 receptor agonists (GLP-1-RA) and sodium glucose co-transporter-2 (SGLT-2) inhibitors are also known to have influences on these mechanisms. In this review, we delineate the role of mitochondrial dysfunction in kidney disease, the current mitochondria-targeting treatment options affecting the kidneys and the future role of mitochondria in kidney pathology

Pleomorphic Carcinoma of the Lung with High Serum Beta-human Chorionic Gonadotropin Level and Gynecomastia

Although gynecomastia is a well-defined paraneoplastic syndrome in patients with non-small cell lung cancer, the association with pleomorphic carcinoma has not been reported. A 50-yr-old man presented with bilateral gynecomastia and elevated serum beta-human chorionic gonadotropin (βhCG) level. Chest tomography showed a mass in the right middle lobe. Right middle lobectomy and mediastinal lymph node dissection were performed. βhCG levels decreased rapidly after surgery. Histological examination revealed pleomorphic carcinoma with positive immunostaining for βhCG. Serum βhCG levels began to increase gradually on postoperatively 4th month. Computed tomography detected recurrence and chemotherapy was started. After second cycle of chemotherapy, βhCG levels decreased dramatically again and tomography showed regression in mass. Patient died 6 months later due to brain metastasis. βhCG expression may be associated with aggressive clinical course and increased risk of recurrence, also βhCG levels may be used to evaluate therapy response in patients with pleomorphic carcinoma

A familial Mediterranean fever patient with double homozygous mutations treated with anakinra after kidney transplantation

sevinc, mustafa/0000-0003-2804-4884; Hasbal, Nuri Baris/0000-0002-2229-5140WOS: 000581011100010PubMed: 31201047[No abstract available

Guillain-Barre Syndrome in a Patient with Primary Extranodal Intestinal Non-Hodgkin's Lymphoma: Paraneoplastic, Drug Induced or Coincidental?

Neurological involvement is observed in 5%-25% of patients with lymphoma being either the first presentation of the disease or emerging during its course. However, Guillain-Barre syndrome is rarely reported. In this article, we present a case with intestinal lymphoma developing Guillain-Barre syndrome during the course of the disease. A 66-year-old male patient with primary extranodal intestinal lymphoma developed quadriparesis, sensory deficits and autonomic dysfunction while receiving chemotherapy. The findings of clinical, electrophysiological and laboratory examinations were consistent with Guillain-Barre syndrome. Guillain-Barre syndrome can potentially be fatal and mimic chemotherapy-induced neurotoxicity, especially in patients with lymphoma, and therefore, must be considered in the differential diagnosis

Air embolism following removal of hemodialysis catheter

sevinc, mustafa/0000-0003-2804-4884; Sahutoglu, Tuncay/0000-0003-2015-4421; Hasbal, Nuri Baris/0000-0002-2229-5140WOS: 000393616400009PubMed: 27457403Introduction: Air embolism (AE) is a rare, but serious complication that can occur in the practice of hemodialysis. in contrast to careful techniques andmeticulous care during insertions and manipulations of the central catheters, awareness of the risk of AE following catheter removal is less. We aimed to analyze the clinical characteristics of the all case reports with AE after catheter removal and summarize the mechanisms, clinical consequences, treatment and prevention of AE. Methods: in addition to our case, MEDLINE database was searched for all case reports with AE following catheter removal, and the clinical, diagnostic and outcome data were analyzed. Findings: A total of 10 patients (including our case) (M/F 6/4; median age 50.5 years) were found for the analysis. Procedures for prevention of AE were reported in a few patients (Trendelenburg position 2, airtight dressing 1). the time that elapsed between catheter removals and onset of AEs was ranged from seconds to 6 hours. the most common findings were dyspnea (90%), hypoxemia (70%), and cerebral dysfunction (70%). the most common sites where air could be detected were the left ventricle (40%), pulmonary artery (30%) and right ventricle (30%). Mortality was reported in 4 (40%) cases and the remaining 6 patients had complete recovery. Blocking of air portal was not reported in any of the fatal cases. Discussion: AE following catheter removal carries a major risk of mortality. Great awareness and attention to preventive procedures and appropriate care after development of AE seemmandatory

CLINICAL MANIFESTATIONS RISK FACTORS AND PROGNOSIS OF ENCAPSULATED PERITONEAL SCLEROSIS

55th Congress of the European-Renal-Association (ERA) and European-Dialysis-and-Transplantation-Association (EDTA) -- MAY 24-27, 2018 -- Copenhagen, DENMARKWOS: 000433059801645…European Renal Assoc, European Dialysis & Transplant Asso

Unexpectedly High Prevalence of Low Alpha-Galactosidase A Enzyme Activity in Patients with Focal Segmental Glomerulosclerosis

Hasbal, Nuri Baris/0000-0002-2229-5140WOS: 000576771000001PubMed: 32997080OBJECTIVES: Fabry disease (FD) is a rare disease associated with sphingolipid accumulation. Sphingolipids are components of plasma membranes that are important in podocyte function and accumulate in various glomerular diseases such as focal segmental glomerulosclerosis (FSGS). Both FD and FSGS can cause podocyte damage and are classified as podocytopathies. in this respect, FD and FSGS share the same pathophysiologic pathways. Previous screening studies have shown that a significant proportion of end-stage renal disease (ESRD) patients receiving hemodialysis (HD) have unsuspected FD, and the prevalence of low alpha-galactosidase A (aGLA) enzyme activity in these patients is higher than that in the normal population. We aimed to compare aGLA enzyme activity in patients with biopsy-proven FSGS and ESRD receiving HD. METHODS: the records of 232 patients [62 FSGS (F/M: 33/29); 170 HD (M/F: 93/79)] were evaluated retrospectively. the screening was performed based on the aGLA enzyme activity on a dried blood spot, with the confirmation of plasma LysoGb3 levels, and the known GLA mutations were tested in patients with low enzyme activities. the two groups were compared using these parameters. RESULTS: the mean level of aGLA enzyme activity was found to be lower in FSGS patients than in the HD group (2.88 +/- 1.2 mmol/L/h versus 3.79 +/- 1.9 mmol/L/h, po0.001). There was no significant relationship between the two groups with regard to the plasma LysoGb3 levels (2.2 +/- 1.22 ng/ml versus 1.7 +/- 0.66 ng/ml, p: 0.4). in the analysis of GLA mutations, a D313Y mutation [C(937G4T) in exon p] was found in one patient from the FSGS group. CONCLUSIONS: We found that aGAL activity in patients with FSGS is lower than that in patients undergoing HD. the low enzyme activity in patients with FSGS may be explained by considering the similar pathogenesis of FSGS and FD, which may also lead to sphingolipid deposition and podocyte injury