Search results for "Kidney"
showing 10 items of 1514 documents
Microvascular injury and repair in acute human bacterial pyelonephritis.
1987
Acute inflammatory cell-capillary endothelial cell interactions, related to injury and repair, were investigated light and electron microscopically in acute human bacterial pyelonephritis. In inflammatory infiltrate-adjacent microvessels, the small capillaries were completely occluded by leukocyte plugs and the large capillaries were densely filled with acute inflammatory cells adhering to the endothelium. Severe damage to small and large capillaries was observed around endothelium adherent, degranulated neutrophil granulocytes containing phagocytosed bacteria. There were spaces in the endothelium, degradation of the vascular basement membrane, of the perivascular interstitial matrix and of…
Anderson-Fabry disease: clinical manifestations of disease in female heterozygotes.
2002
Anderson-Fabry disease is a rare, X-chromosomal lipid storage disorder caused by a deficiency of lysosomal alpha-galactosidase A. Clinical manifestations of Anderson-Fabry disease include excruciating pain in the extremities (acroparaesthesia), skin vessel ectasia (angiokeratoma), corneal and lenticular opacity, cardiovascular disease, stroke and renal failure, only renal failure being a frequent cause of death. Heterozygote female carriers have often been reported as being asymptomatic or having an attenuated form of the disease. To evaluate the spectrum of clinical signs in heterozygotes, a comprehensive clinical examination was performed on 20 carriers of Anderson-Fabry disease. This rev…
Intrahepatic IgG/IgM plasma cells ratio helps in classifying autoimmune liver diseases.
2010
Abstract Background/Aim Plasma cells infiltrate in the liver is a prototype lesion of autoimmune liver diseases. The possible role of plasma cells isotyping (IgM and IgG) in the liver in the diagnostic definition of autoimmune liver disease, and particularly in variant syndromes such as autoimmune cholangitis and the primary biliary cirrhosis/autoimmune hepatitis overlap syndrome, is less defined. Methods We analysed the clinical, serological and histological features of 83 patients with autoimmune liver disease (40 primary biliary cirrhosis, 20 autoimmune hepatitis, 13 primary sclerosing cholangitis, 4 autoimmune cholangitis and 6 overlap syndrome) compared to 34 patients with chronic hepa…
Pharmacokinetics, pharmacodynamics, and safety of moss-aGalactosidase A in patients with Fabry disease.
2019
Moss-aGalactosidase A (moss-aGal) is a moss-derived version of human α-galactosidase developed for enzyme replacement therapy in patients with Fabry disease. It exhibits a homogenous N-glycosylation profile with >90% mannose-terminated glycans. In contrast to mammalian cell produced α-galactosidase, moss-aGal does not rely on mannose-6-phosphate receptor mediated endocytosis but targets the mannose receptor for tissue uptake. We conducted a phase 1 clinical trial with moss-aGal in six patients with confirmed diagnosis of Fabry disease during a 28-day schedule. All patients received a single dose of 0.2 mg/kg moss-aGal by i.v.-infusion. Primary endpoints of the trial were safety and pharmaco…
Conservative Surgery of Renal Carcinoma: The EIRSS Experience
1981
Summary— Seventy-two patients with renal carcinoma (RC) in a solitary kidney or bilateral synchronous tumours underwent parenchyma-sparing excision of their neoplasms. This was performed in situin 51 and extracorporeally in 21 kidneys, with a complication rate respectively of 1 7 and 43%. The 5-year survival rates were 78% for unilateral disease, 48% for bilateral synchronous and 38% for bilateral asynchronous disease. Survival correlated with Pcategory, grade and diameter of the tumour. Vascular invasion and spindle cells indicated a poor prognosis; the position of the tumour, oncocytic elements, calcification, lymphocytic infiltration or the predominance of clear cell or granular cells di…
De novo mutation in a male patient with Fabry disease: a case report
2014
Abstract Background Fabry disease is an X-linked inherited metabolic condition where the deficit of the α-galactosidase A enzyme, encoded by the GLA gene, leads to glycosphingolipid storage, mainly globotriaosylceramide. To date, more than 600 mutations have been identified in human GLA gene that are responsible for FD, including missense and nonsense mutations, small and large deletions. Such mutations are usually inherited, and cases of de novo onset occur rarely. Case presentation In this article we report an interesting case of a 44-year-old male patient suffering from a severe form of Fabry disease, with negative family history. The patient showed signs such as cornea verticillata, ang…
The Influence of the Primary Tumor on the Long-term Results of Pulmonary Metastasectomy for Metastatic Renal Cell Carcinoma
2011
OBJECTIVE To investigate one of the primary tumor (PT) on pulmonary metastasectomy (PM) for metastatic renal cell carcinoma (RCC) and to define prognostic factors. METHODS Retrospective review of patients with pulmonary metastases from RCC from January 1999 through December 2008 was performed. All patients underwent PM with curative intend. TNM-classification, tumor stage and PT grade, disease-free-interval (DFI) from nephrectomy to the diagnosis of metastasis, systemic chemotherapy before surgical intervention, surgical procedures, morbidity, mortality, and survival were investigated. RESULTS One-hundred seven consecutive patients (age 61.5 ± 9.6 years) underwent PM. Morbidity and mortalit…
Metastasectomy With Standardized Lymph Node Dissection for Metastatic Renal Cell Carcinoma: An 11-Year Single-Center Experience
2013
Background Pulmonary metastasectomy (PM) for metastatic renal cell carcinoma is an established method of treatment for selected patients. The incidence of intrathoracic lymph node metastases (ITLNM) and outcomes remain controversial. The purpose of this study was to determine the incidence of ITLNM and long-term outcome of PM for metastatic kidney cancer. Methods From January 1999 to December 2009, 116 patients (82 men, age 61.7 ± 9.0 years) with metastases from kidney cancer underwent PM and systematic lymph node dissection with curative intent. Kaplan-Meier analyses, log-rank test, and Cox regression analyses were used to estimate survival and to determine prognosticators of survival. Res…
Hand-assisted laparoscopic living-donor nephrectomy versus open surgery: evaluation of surgical trauma and late graft function in 82 patients
2009
Abstract Objective We evaluated and quantified surgical trauma and late graft function in cases of hand-assisted laparoscopic living-donor nephrectomy (HALLDN) versus open living-donor nephrectom (OLDN). Methods This study is a retrospective nonrandomized single-center analysis. Between 1995 and January 2008, 82 patients with end-stage renal disease received kidney transplantations from living donors. Open living-donor nephrectomy was performed in 37 donors, and 45 underwent laparoscopic hand-assisted nephrectomy. Demographic data and perioperative and postoperative data, such as markers of acute phase (C-reactive protein; serum amyloid A) and biochemical markers of glomerular filtration (s…
Virtual Unenhanced Images at Dual-Energy CT: Influence on Renal Lesion Characterization
2019
Background Dual-energy (DE) CT allows reconstruction of virtual noncontrast (VNC) images from a single-phase contrast agent-enhanced examination, potentially reducing the need for multiphasic CT to characterize renal lesions. However, data regarding diagnostic performance of VNC images for the characterization of renal lesions are limited. Purpose To determine whether renal mass CT performed by using VNC images allows for reliable identification of renal lesions and differentiation of contrast-enhanced from unenhanced lesions, compared with unenhanced images. Materials and Methods This is a retrospective study of 293 patients (105 women [mean age, 65 years; age range, 18-91 years] and 188 m…