Biomedical subjects
Publications and source records attributed to L S Khodasevich.
Current literature on the pathogenesis of the hemolytic-uremic syndrome (HUS) is analyzed. A variety of etiologic factors and mechanisms of HUS development is considered. The emphasis is laid on the endotoxinemia, damage of the renal vessel and glomerular mesangium endothelium and other mechanisms.
An analysis of 4 cases of the thrombotic thrombocytopenia in children of 4 to 10 years of age is performed. The disease was characterized by fever, purpura, headache and abdominal pains, arterial hypertension, microangiopathic haemolytic anemia, thrombocytopenia, increase of blood urea and serum creatinine, micro-haematuria and proteinuria. The duration of the disease was from 4 days to 7 months. Anuria, gangrene of the ears, scrotum, penis and soft tissues of legs and feet were registered in a 5-year-old patient with a fulminant disease. The cause of death of other patients was heart failure with acute lung oedema, brain haemorrhages and haemorrhagic pancreonecrosis. The diagnosis of the thrombotic thrombocytopenia was confirmed by the finding in the autopsy material of thrombotic microangiopathy of small arteries, veins, arterioles, venules and capillaries in kidneys and other organs and tissues. Kidney damage in fulminant disease is complicated by segmentary cortical necrosis, in a more prolonged disease--by glomerulosclerosis or mesangio-capillary glomerulonephritis.
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7 cases of the generalized streptococcus infection in children aged from 1 year 8 months to 12 years are analyzed. The course of the disease was in all cases extremely rapid and terminated by death within 3-4 days. Results of the autopsy material investigation allowed to conclude that the pathology of the generalized streptococcus infection at present does not differ from that described in classical text-books. The attention is drawn to the connection between the generalized streptococcus infection and herpes-like infections since in all cases studied herpes stomatitis, genital herpes, varicella were noted clinically. The presence of herpes-like infections was confirmed morphologically in some cases.
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The attempt to establish the connection of the idiopathic arterial calcification (IAC) with calciphilaxy is made on the basis of the published data analysis and 11 observations of IAC in children. Apart from known morphological manifestations of IAC, the authors found mast cells in the cellular infiltrate, vesicular transformation of smooth cells in the arteries affected. This together with the elastic membrane changes in the arteries and massive iron and oxyapatite deposits in the arterial wall suggests the development of calciphylactic reaction. The similarity of the morphological and clinical manifestations, and IAK therapy in the clinics and experiment is shown. Special emphasis is made on the possibility of the calciphylaxy development in children under conditions of the vitamin D wide use for the prevention of rickets and iron preparations use for anemia treatment. The conclusion is drawn on the multicausal character of IAK and possibility to distinguish congenital, acquired and combined forms of the disease. The necessity of through analysis of each case of IAK is stressed in order to reveal iatrogenic factors capable to produce sensibilization and/or to provoke the disease.
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A course of leprechaunism is described in a 6-month-old girl who died of cachexia in the presence of generalized cytomegalia and myocardial metabolic derangement caused by cardiac arterial calcinosis. Proband sib aged 7 suffered from deaf-mutism and imbecility. There were multiple deformities of the face and body, skin hyperkeratosis with papillomatosis and melanoderma, hirsutism, pseudohermaphroditism, breast hyperplasia, normal serum glucose. Ovarian polycystosis and nephromegaly were detected at autopsy. Histologically, there appeared nesidioblastosis, Leydig's cells focal hyperplasia in the ovaries, cystic renal dysplasia calcinosis of cardiac arteries, those of mesentery of the small intestine, spleen, pancreas and thyroid gland. The final diagnosis was established on the basis of the whole complex of clinical and morphological signs.
Morphology of the kidneys in hemolytic-uremic syndrome is considered basing on autopsy findings obtained for 3 infants with 5-17-day history of acute renal failure. A newborn infant of 17 days developed the disease after feto-fetal hemotransfusion when macerated fetus-donor hemolysis products entered the circulation of the fetus-recipient through monochorionic placenta. The second case in an infant of 6 months was due to ADTP Vaccine. The last infant aged 16 months manifested the syndrome in the presence of Proteus-induced ulcerative colitis. Varying in etiology, renal morphology exhibited similar features: fibrin deposits in the lumens of glomerular capillary loops, afferent glomerular arterioles and intrarenal arteries; fibrinoid necrosis of the wall in the arterioles. The renal affection ranged from acute thrombotic glomerulonephritis to cortical necrosis, these variations being dependent on the degree of thrombogenesis, caliber of impaired intrarenal vessels and time from the onset of acute renal failure.
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Two cases of idiopathic Addison's disease affecting heterozygous twins who died from addisonian crisis at the ages of 7 and 10 years, are described, including descriptions of histologic findings in the adrenals, thymus, hypophysis, kidneys, esophagus, upper respiratory tract, and other viscera. The authors believe that autoimmunization in children with idiopathic Addison's disease develops in the presence of congenitally defective immunity and propose that activation of the juxtaglomerular complex and hypergranulation of interstitial cells in the inner renal medulla may be used as morphologic criteria in the diagnosis of addisonian crisis.
Two cases of this syndrome occurring in children aged 1.5 and 3 years are reported. In one case, no other abnormalities were present, and the deformity did not manifest itself clinically before the child had fallen from a height. In the other, a cyst of the fourth ventricle coexisted with other abnormalities in the brain which were responsible for neurologic symptoms from the very birth and also, probably, for the atresia of draining orifices.
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An observation of Reye's syndrome in a 4.5-years-old boy developing against the background of varicella and disturbed immune status is described. Ultra-acute evolution of the syndrome prevented clinical diagnosis. Brain oedema, fat degeneration of the liver, kidney, and heart were found at the necropsy.
Comparative morphometric study of the juxtaglomerular complex and interstitial cells of the corticomedullary zone of the kidney was performed on the autopsy material in benign (26 cases) and malignant hypertension; for control, 15 cases were examined. The state of intrarenal arteries, arterioles and the degree of medulla interstitium sclerosis were taken into consideration. No activation of the renin-producing system was observed morphometrically in "benign" hypertension while it was pronounced in malignant hypertension. The hypothesis is put forward on the reserve renin secretion by mesangial cells in malignant hypertension. It is assumed that the prostaglandine-synthesizing function of the interstitial cells is not damaged in benign hypertension, but it is significantly lower in malignant course of the disease. Relationship is shown between the alterations of intrarenal arteries and arterioles and the degree of the medulla sclerosis in malignant hypertension.
Two observations of the primary form of diffuse phlegmonous gastritis in men of 46 and 33 are described. In both cases the disease ran malignant courses with the development of peritonitis and bacterial shock. In the first case the causative agent of the disease was not established, however, it had a marked gas-producing capacity, in the other case it was Proteus.