ABSTRACT
The first documented case of mitochondrial neurogastrointestinal encephalomyopathy was described in 1962 by R. Luft. The variety and am-biguity of the clinical manifestations of the disease complicate its early diagnosis and treatment. The first clinical manifestations of the disease are associated with the pathology of the gastrointestinal tract. Low alertness and insufficient awareness of doctors delays the timely diagnosis of mitochondrial neurogastrointestinal encephalomyopathy. The aim of the work is to increase the alertness and awareness of narrow specialties about the possibility of differential diagnosis of an extremely rare detected disease on the base of our clinical observation.
Subject(s)
Gastrointestinal Diseases , Intestinal Pseudo-Obstruction , Mitochondrial Encephalomyopathies , Ophthalmoplegia , Humans , Intestinal Pseudo-Obstruction/diagnosis , Intestinal Pseudo-Obstruction/etiology , Masks , Ophthalmoplegia/etiology , Ophthalmoplegia/complications , Mitochondrial Encephalomyopathies/diagnosis , Mitochondrial Encephalomyopathies/complications , Diagnosis, Differential , Rare Diseases/complications , Rare Diseases/diagnosis , Gastrointestinal Diseases/diagnosisABSTRACT
Lipid metabolism was studied in 161 patients with renal diseases, 62 of them had nephrotic syndrome (NS). The measurements were made of cholesterol, triglycerides, dienic conjugates, B2-microglobulins in urine and blood, functional and morphological parameters, red cell resistance to peroxide hemolysis. It was found that NS patients, especially those with additional renal failure, displayed high-intensity lipoperoxidation (LPO). LPO intensity proved unrelated to NS etiology. NS-associated hyperlipidemia gives rise to multifunctional biomembrane instability responsible for antioxidant disorders.
Subject(s)
Lipid Peroxidation , Lipids/blood , Nephrotic Syndrome/blood , Adult , Antioxidants , Biomarkers/blood , Chronic Disease , Female , Humans , Lipid Peroxides/blood , Male , Nephrotic Syndrome/etiology , beta 2-Microglobulin/analysisABSTRACT
The nephrotic syndrome is characterized by proteinuria, hypoalbuminemia and hypercholesterolemia. Hypercholesterolemia is in some cases a risk factor for atherosclerosis in this group of patients. The lipid plasma spectrum was studied in 45 patients with the nephrotic syndrome. Most pronounced changes of the lipid composition of the plasma were revealed in patients with systemic lupus erythematosus and a special form of mesangio-proliferative glomerulonephritis which is characterized by a torpid course and rapid development of chronic renal failure. Plasma atherogenicity was calculated according to the index of plasma atherogenicity. A high atherogenicity index was revealed in patients with an association of the nephrotic syndrome and arterial hypertension. Plasma atherogenicity is determined mainly by the level of high-density-lipoprotein cholesterol.
Subject(s)
Arteriosclerosis/etiology , Lipids/blood , Nephrotic Syndrome/blood , Plasma/chemistry , Adult , Arteriosclerosis/blood , Chronic Disease , Female , Glomerulonephritis, Membranoproliferative/blood , Glomerulonephritis, Membranoproliferative/complications , Glomerulonephritis, Membranous/blood , Glomerulonephritis, Membranous/complications , Humans , Hypertension/blood , Hypertension/complications , Male , Nephrotic Syndrome/complicationsABSTRACT
The results are available of combined examination of the kidneys in 84 patients with manifest nephrotic syndrome varying in etiology: 57 had glomerulonephritis, 18 amyloidosis, 9 diabetic nephropathy. The study covered lipid metabolism, immunological status, lifetime morphological investigation of the kidneys. The latter procedure proved advantageous over biochemical and immunological studies in deciding upon nosological diagnosis of nephrotic syndrome and its prognosis. Destruction of podocytic miner processes revealed in all the syndrome cases can serve a uniform morphological substrate underlying massive proteinuria.
Subject(s)
Immunoglobulins/analysis , Kidney/physiopathology , Nephrotic Syndrome/diagnosis , T-Lymphocytes/immunology , Adult , Atrophy/etiology , Humans , Kidney/pathology , Kidney Function Tests , Leukocyte Count , Nephrotic Syndrome/immunology , Nephrotic Syndrome/pathology , Nephrotic Syndrome/physiopathologyABSTRACT
The author examined 78 patients with chronic glomerulonephritis and verified their diagnosis on the basis of the kidney biopsy data. With regard to the severity of the tubular interstitial component the patients were enrolled in three groups. It was stated that with the increasing changes developed in the tubular interstitial component, the levels of total cholesterol, cholesterol of high density lipoproteins, low density lipoproteins and triglyceride elevated. In the group of the patients with minimal changes in the tubular interstitial component, the number of those in whom plasma lipid levels were below the upper normal limits was higher than in two other groups. A direct proportional relationship between the levels of plasma lipids and severity of the tubular interstitial component indicated the immediate role of the renal tissue in the pathogenesis of lipid turnover disorders in patients with chronic glomerulonephritis.
