End-stage

Nonalcoholic fatty liver disease (NAFLD) is highly prevalent and can lead to cirrhosis, hepatocellular carcinoma, and end-stage liver disease. NAFLD comprises the spectrum from simple steatosis (nonalcoholic fatty liver, NAFL), to steatosis with inflammation (nonalcoholic steatohepatitis, NASH). Current primary therapy recommended for NAFLD is weight loss induced by lifestyle modification. The difficulty in achieving this has led to robust pharmacological therapy development. While new drugs may show efficacy in selected phase II/III clinical trial populations, their real-world effectiveness is unknown...

OBJECTIVE: Increased Wisp1 expression was previously reported in experimental and human osteoarthritis. Moreover, adenoviral overexpression of Wisp1 in naïve mouse knee joints resulted in early OA-like cartilage lesions. Here, we determined how the matricellular protein WISP1 is involved in the pathology that occurs in the complex osteoarthritic environment with aging and experimental osteoarthritis in wild type and Wisp1(-/-) mice. METHODS: Wild type and Wisp1(-/-) mice were aged or experimental osteoarthritis was induced with intraarticular collagenase injection, destabilization of the medial meniscus or anterior cruciate ligament transection...

BACKGROUND: Elbow reconstruction with vascularized composite allotransplantation (VCA) may hold promise in treating end-stage arthritis as no current treatment is both functional and durable. We describe the vascular and gross anatomy of the elbow in the context of VCA procurement and propose a step-by-step surgical technique for human elbow VCA. METHODS: We injected latex in the arterial tree of 16 fresh adult cadaveric upper extremities. We identified and measured arteries and nerves and their branch points relative to the medial epicondyle...

Insufficient is the evidence for safe use of elderly donors in adult-to-adult living donor liver transplantation (LDLT). The aim of this study was to evaluate the outcomes of right lobe LDLT by donor age (≥55 vs. <55 years). All living donors who underwent right hepatectomy at the authors' institution between March 2008 and December 2015 were divided into 2 groups: group A with an age ≥ 55 and group B with an age < 55. The selection criteria for elderly donor were preservation of middle hepatic vein, remnant liver volume ≥30%, and no or mild fatty liver...

Orthotopic liver transplant (OLT) remains the standard of care for end stage liver disease. To circumvent allo-rejection, OLT subjects receive gluococorticoids (GC). We investigated the effects of GC on endogenous mesenchymal stem (stromal) cells (MSCs) in OLT. This question is relevant because MSCs have regenerative potential and immune suppressor function. Phenotypic analyses of blood samples from 12 OLT recipients, at pre-anhepatic, anhepatic and post-transplant (2 h, Days 1 and 5) indicated a significant decrease in MSCs after GC injection...

AIM: To study the epidemiology, clinical characteristics, vascular access, and the short term survival of ESRD patients initiated on dialysis from Hospital Queen Elizabeth (HQE). BACKGROUND: The number of patients with ESRD is increasing in Sabah, Malaysia. Most patients present late and some live in remote areas with difficult access to healthcare services. Many therefore present with potentially fatal complications. METHODS: All the newly confirmed ESRD patients who were initiated on renal replacement therapy (RRT) from 1 January to 31 December 2014 were included...

BACKGROUND: Pulmonary hypertension (PH) in patients with hypertrophic cardiomyopathy (HCM) has been investigated in a small number of studies. Purpose of this study was to assess the prevalence and its association with outcome in a population of consecutive HCM outpatients. METHODS: We retrospectively analyzed data of 361 consecutive HCM outpatients in whom echocardiographic measurements of pulmonary artery systolic pressure (PASP) were available at initial and most recent evaluation...

BACKGROUND: The participation of the people in health decisions may be structured in various levels. One of these is participation in decisions for the treatment. "Advanced directives" is one of the examples for the participation in decisions for the treatment. AIM: We wanted to determine the decisions on advanced life support at the end-stage of life in case of a life-threatening illness for the people themselves and their first degree relatives and the factors effecting these decisions...

INTRODUCTION: Henoch-Schönlein Purpura (HSP; IgA vasculitis), the most common vasculitis of childhood, has a wide spectrum of clinical manifestations ranging from palpable purpura with abdominal pain and arthritis to the more morbid renal involvement. AIMS AND OBJECTIVES: To study and correlate the clinical presentation, laboratory values, skin and renal histopathology and immunofluoroscence findings in HSP. MATERIAL AND METHODS: A total of 44 cases of HSP from March 2011 to February 2014 were studied for the above features along with their clinical outcomes...

BACKGROUND: End-stage kidney disease is associated with poor prognosis. Health care professionals must be prepared to address end-of-life issues and identify those at high risk for dying. A 6-month mortality prediction model for patients on dialysis derived in the United States is used but has not been externally validated. AIM: We aimed to assess the external validity and clinical utility in an independent cohort in Canada. DESIGN: We examined the performance of the published 6-month mortality prediction model, using discrimination, calibration, and decision curve analyses...

BACKGROUND: Providing accurate and valid prognostic information significantly influences end-of-life care. Disclosing a poor prognosis can be among the most difficult of physician responsibilities, thus having appropriate knowledge during training is crucial for appropriate prognostication. OBJECTIVE: To provide internal medicine (IM) house staff with a pre- and posteducational survey to determine their ability to accurately prognosticate 5 common end-stage diseases...

BACKGROUND & AIMS: Mortality of cirrhotic patients after emergency care admission is high and prognostic factors can help in prioritizing patients. The aim of our study was to assess the association between levels of cardiac Troponin T (cTnT) and one-year mortality in patients with liver cirrhosis without known cardiac disease, who were admitted to the emergency department (ED). METHODS: All patients with cirrhosis presented to the ED from October 2009 until August 2015 who had an initial cTnT value measured with the first lab-panel were retrospectively analyzed with a follow-up of 365 days...

The autonomic nervous system (ANS) is the principal endogenous defense mechanism that maintains blood pressure in the setting of hypotension. Disruption of the ANS impairs this ability and can contribute to blood pressure instability, including hypotension and hypertension. In this narrative review, we provide an overview of the ANS and the consequences of its dysfunction in patients with end-stage kidney disease treated with dialysis. We also discuss possible mechanisms of this autonomic dysfunction that may need future investigation...

The authors present an overview of lower urinary tract obstruction (LUTO) in the fetus with a particular focus on the insult to the developing renal system. Diagnostic criteria along with the challenges in estimating long-term prognosis are reviewed. A proposed prenatal LUTO disease severity classification to guide management decisions with fetal intervention to maintain or salvage in utero and neonatal pulmonary and renal function is also discussed. Stage I LUTO (mild form) is characterized by normal amniotic fluid index after 18 weeks, normal kidney echogenicity, no renal cortical cysts, no evidence of renal dysplasia, and favorable urinary biochemistries when sampled between 18 and 30 weeks; prenatal surveillance is recommended...

AIM: To test whether muscle mass evaluated by creatinine excretion (EXCr) is maintained in patients with end-stage kidney disease (ESKD) treated by peritoneal dialysis (PD), we evaluated repeated measurements of EXCr in a PD population. METHODS: One hundred and sixty-six PD patients (94 male, 72 female) receiving the same PD dose for the duration of the study (up to approximately 2.5 years) had repeated determinations of total (in urine plus spent dialysate) 24-h EXCr (EXCr T) to assess the adequacy of PD by creatinine clearance...

Congestion is an integral component of cardiorenal syndrome and portends an adverse impact on the outcomes. Recent studies suggest that congestion has the ability of modulating the interactions between the kidney and the heart in this setting. Peritoneal dialysis (PD) is a home-based therapeutic modality that is not only offered to patients with end-stage renal disease to provide solute clearance and ultrafiltration, but it has also been used in patients with refractory heart failure and fluid overload to help optimize volume status...

Chronic obstructive pulmonary disease (COPD) is a heterogeneous disease in which the amount of emphysema and airway disease may be very different between individuals, even in end-stage disease. Emphysema formation may be linked to the involvement of the small pulmonary vessels. The NAPDH oxidase (Nox) family is emerging as a key disease-related factor in vascular diseases, but currently its role in hypoxia-induced pulmonary remodelling in COPD remains unclear.Here we investigate the role of p22phox, a regulatory subunit of Nox, in COPD lungs, hypoxic pulmonary vasoconstriction (HPV), hypoxia-induced pulmonary vascular remodelling and pulmonary hypertension...

Chronic kidney disease (CKD) is higher in elderly, but mortality outweighs the risk of end-stage renal disease (ESRD). Our aim was to identify prognostic markers for ESRD or death in elderly CKD, within a competing-risk analysis. This is a longitudinal study of consecutive newly referred patients with CKD ages 65 years, followed until the time of the first event (ESRD or death), using a competing-risk analysis. A modified Charlson Comorbidity Index (mCCI) was subdivided into subgroups (0-2, 3-4, ≥5). Patients were followed for hospitalizations that occurred prior to the outcomes...

INTRODUCTION: Proprotein convertase subtilisin/kexin type 9 (PCSK9) plays a key role in the cholesterol metabolism and is synthesized by the liver. It interacts with the LDL-receptor to promote its degradation. The model of end-stage liver disease (MELD) score is a well-established tool to estimate the risk of mortality in patients with end-stage chronic liver disease. The study aims to assess the associations between PCSK9, hypocholesterinemia, liver synthesis, cholestasis, MELD score and mortality in patients with end-stage liver disease...

Human ciliopathies are a class of multi-organ genetic disorders caused by defects of proteins expressed at the primary cilium, an organelle present on the cell surface of almost all cell types. Thus far, dozens of causative genes for ciliopathies have been identified and many of them are known to cause allelic disease. Of particular interest is the TMEM67 gene, encoding the transmembrane protein meckelin. The involvement of the mutant TMEM67 gene is known to be associated with a broad range of clinical presentations, namely Joubert syndrome 6 (JBTS6), nephronophthisis 11 (NPHP11), Bardet-Biedel syndrome (BBS), COACH syndrome, and lethal Meckel syndrome type 3 (MKS3)...