Leigh's disease is a rare progressive neurological disorder that is characterized light microscopically by focal spongy necrosis in the brain and electron microscopically by mitochondriopathy. We report an autopsy case of Leigh's disease that showed abnormalities in the liver, kidney and skeletal muscle as well as the central nervous system. The patient was an 18-month-old girl who has carried a diagnosis of cerebral palsy ever since her birth to a 20-year-old mother. The baby was generally hypertonic and mentally retarded. She died of severe metabolic acidosis. Postmortem examination showed growth retardation, fatty liver, fatty kidney and soft brain. Brain section showed multifocal softenings in the brainstem, basal ganglia and periventricular areas. Microscopically increased capillaries with endothelial proliferation, vacuolar degeneration and mild gliosis were seen in the brain. The axons were relatively preserved. Liver and kidneys showed microvesicular fatty change. Myofiber degeneration of the skeletal muscle was also noted. Electron microscopic examination showed markedly increased mitochondria in the parenchymal cells of the brain, liver and kidney. The mitochondria showed round to ovoid ballooned appearance including electron-dense core-like structures and pseudoinclusions of glycogen granules.
Brain/pathology/ultrastructure ; Female ; Humans ; Infant ; Kidney/pathology/ultrastructure ; Leigh Disease/*pathology ; Liver/pathology/ultrastructure ; Mitochondrial Encephalomyopathies/pathology ; Muscles/pathology

Basement Membrane ; pathology ; ultrastructure ; Biopsy ; Glomerulonephritis, IGA ; pathology ; Humans ; Kidney ; pathology ; ultrastructure ; Kidney Glomerulus ; pathology ; ultrastructure ; Lupus Nephritis ; pathology ; Microscopy, Electron, Transmission ; Paraffin Embedding ; Specimen Handling ; methods

OBJECTIVETo investigate the effects of postburn fluid resuscitation on the pathohistological and ultrastructural changes of multiple organs with dysfunction in severely burned dogs.

METHODSForty - four mongrel dogs were randomly divided into four groups: (1) immediate infusion (II, n = 8), (2) delayed infusion (DI, n = 15), (3) no infusion (NI, n = 14), (4) normal control (NC, n = 7). The dogs were inflicted with 50% TBSA III degree flame burn produced by napalm in concentration of 30g/L burning for 30 seconds on the back. Small pieces of tissue samples of heart, lungs, liver, kidneys and gastrointestinal tract were taken from injured dogs at 72 postburn hours (PBHs) or moribund stage for the examination with light microscope (LM) and transmission electron microscope (TEM).

RESULTSDifferent degrees of blood circulation disturbance and degenerative changes were found in all above internal organs. These changes were more evident in DI than in II and NI groups.

CONCLUSIONDelayed postburn fluid resuscitation could induce multiple organ dysfunction in early postburn stage.

Animals ; Burns ; complications ; therapy ; Digestive System ; pathology ; ultrastructure ; Dogs ; Fluid Therapy ; Kidney ; pathology ; ultrastructure ; Liver ; pathology ; ultrastructure ; Lung ; pathology ; ultrastructure ; Multiple Organ Failure ; etiology ; pathology ; prevention & control ; Myocardium ; pathology ; ultrastructure ; Time Factors

To improve the understanding of lipoprotein glomerulopathy, the clinical data of a single case of lipoprotein glomerulopathy, diagnosed at the Second Xiangya Hospital, Central South University in June 2011, were retrospectively analyzed. The analysis included clinical manifestation, laboratory results, and pathological information on kidney biopsy. Nephrotic syndrome was seen as the major clinical feature in this patient. Levels of serum apoE and apoB were increased. Obvious expansion of glomerular capillary cavities, filled with a weakly stained thrombus-like substance, were observed. ApoE immunohistochemical staining showed positive staining in capillary luminal contents. Transmission electron microscopy revealed capillary lumina occluded by various foaming lipid depositions in clustered or layered arrangement. There were no special symptoms nor obvious abnormal laboratory results of the LPG patient. Diagnosis of LPG should be based on clinical manifestations and renal biopsy. Characteristic pathological performance plays an important role in the diagnosis of lipoprotein glomerulopathy.
Adult ; Biopsy, Needle ; Humans ; Kidney ; pathology ; ultrastructure ; Kidney Diseases ; diagnosis ; pathology ; Male

OBJECTIVETo investigate the ultrastructural changes of the extraintestinal organs of newborn mice with human retrovirus (RV) infection to probe into the mechanism and clinical diagnose and therapy of extraintestinal RV infection.

METHODSHuman RV was inoculated into the abdominal cavity of the newborn mice, and the ultrastructural changes of the heart, lung, livers, and kidneys of the infected and control mice were observed by transmission electron microscope.

RESULTSThe mice with intraabdominal RV injection showed pathological changes of the cells in the small intestinal villus, liver, and kidneys. Shortened small intestinal villus, nuclear membrane disorganization, massive vacuolization, mitochondrial swelling and rough endoplasmic reticulum dilation were observed in the cells of the small intestinal. In the liver of the mice, marked mitochondrial swelling and agglutination, cell nucleus pyknosis or collapse, presence of numerous lipid droplets and vacuoles were seen in the liver cells, with lymphocyte and plasmacyte infiltration. Obvious dilatation and shedding of the microvillus were seen in cholangioles. The mitochondria of the proximal convoluted renal tubule showed mild swelling, but the cells in the heart and lung did not display obvious changes.

CONCLUSIONThe small intestinal villi were highly susceptible to RV infection, and systemic spread of human RV may cause damage of various extraintestinal organs especially the liver, which can also be susceptible to RV.

Animals ; Animals, Newborn ; Female ; Intestine, Small ; ultrastructure ; virology ; Kidney ; ultrastructure ; virology ; Liver ; ultrastructure ; virology ; Lung ; ultrastructure ; virology ; Male ; Mice ; Microscopy, Electron, Transmission ; Rotavirus Infections ; pathology ; virology

Adult ; Aged ; Amyloidosis ; metabolism ; pathology ; Biopsy ; Female ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Immunoglobulin kappa-Chains ; metabolism ; Immunoglobulin lambda-Chains ; metabolism ; Intestinal Mucosa ; pathology ; ultrastructure ; Kidney ; metabolism ; pathology ; ultrastructure ; Kidney Diseases ; metabolism ; pathology ; Male ; Middle Aged ; Rectum ; pathology ; ultrastructure ; Retrospective Studies

Adult ; Fabry Disease ; pathology ; Female ; Follow-Up Studies ; Humans ; Kidney Glomerulus ; pathology ; ultrastructure ; Proteinuria

BACKGROUNDLipoprotein glomerulopathy (LPG), once recognized as a rare glomerular disease, has been reported around the world in recent years. In this study, we reported 8 patients of LPG and aimed to explore the clinical features and pathological characteristics of LPG under light microscope, immunofluorescence staining, and electron microscope.

METHODSClinical manifestations were recorded on the day of renal biopsy. Biochemical patterns of lipids and lipoproteins were detected by routine examination. Plasma concentrations of apo B and apo E were determined by radial immunodiffusion assays. Biopsy specimens were then processed for light microscopy, immunohistochemical staining for immunoglobulins and complement components, and electron microscopy. Glomerular deposition of apo A, B, and E were detected using monoclonal antibodies on cryostatic sections.

RESULTSAll of the eight patients presented with edema, microscopic hematuria, severe proteinuria, anemia, and enlarged kidney size. Biochemical profiles revealed high levels of triglycerides, apo B, and apo E. We noted increments of glomerular size and lipoprotein thrombi occupying capillary lumina in the glomeruli of all patients. Immunofluorescence staining showed that the thrombi were strongly positive for apo A, B, and E. Granules and various sizes of vacuoles were observed in the thrombi under electron microscope.

CONCLUSIONCompared with previous reports on LPG in other countries, unique clinical and pathological features were found in this group of Chinese LPG patients.

Adolescent ; Adult ; Child ; Female ; Humans ; Kidney Diseases ; pathology ; Kidney Glomerulus ; pathology ; ultrastructure ; Lipoproteins ; metabolism ; Male ; Microscopy, Fluorescence ; Middle Aged

OBJECTIVETo study the clinicopathologic features of membranous nephropathy coexisting with IgA nephropathy.

METHODSThe renal biopsies performed in Peking University First Hospital during the period from January, 1998 to April, 2006 were retrospectively reviewed. The clinicopathologic features of 11 cases of membranous nephropathy coexisting with IgA nephropathy were studied. Electron microscopy with immunogold labeling for IgG and IgA were also performed.

RESULTSThe mean age of patients was 39.9 years. The male-to-female ratio was 1:2.9. The patients mainly presented with proteinuria. Proteinuria of nephrotic level was seen in 7 cases (63.6%). Seven cases also had associated microscopic hematuria. None of them showed evidence of renal insufficiency. Cases with secondary diseases, such as hepatitis virus infection and systemic lupus erythematosus, were excluded from the study. Histologically, vacuolation and thickening of glomerular basement membrane was seen. There was also mild mesangial hypercellularity and increase in mesangial matrix. Occasional glomeruli with crescent formation were identified in 2 cases. Immunofluorescence study showed granular staining for IgG and C3 along glomerular capillary walls, in addition to clumps of IgA deposits in mesangium. Electron microscopy revealed subepithelial and mesangial electron-dense deposits. Immunogold labeling showed IgG and IgA localized in the subepithelial and mesangial deposits respectively.

CONCLUSIONMembranous nephropathy coexisting with IgA nephropathy possesses the clinicopathologic features of both components. It might be caused by independent occurrence of the two entities.

Adult ; Female ; Glomerular Basement Membrane ; immunology ; pathology ; ultrastructure ; Glomerular Mesangium ; immunology ; pathology ; ultrastructure ; Glomerulonephritis, IGA ; complications ; immunology ; pathology ; Glomerulonephritis, Membranous ; complications ; immunology ; pathology ; Humans ; Immunoglobulin A ; metabolism ; Immunoglobulin G ; metabolism ; Kidney Glomerulus ; immunology ; pathology ; ultrastructure ; Male ; Middle Aged ; Retrospective Studies

Percutaneous renal biopsy was performed on a 34 year old male patient with mild proteinuria and microhematuria. Histopathologic examination showed a focal mesangiopathic glomerulonephritis, simulating a "minimal change" disease pattern by light microscope. Granular deposits of IgA, C3, IgG, IgM, and fibrinogen were present in the glomerular mesangial area by immunofluorescent technique. A special prevalence of IgA was found. The intensity of immunofluorescent staining was correlated with the mesangial proliferative reaction by light microscopy. Electron microscopy showed electron dense granular deposits in the mesangial areas. The glomerulonephritis in this patient was related with the IgA antibody associated mesangial immune complex deposit disease mediated by the classic complement pathway. This glomerulonephritis is known to have a good prognosis. The antigenic nature, the reason of predominant immune deposits in the mesangium, and the mechanism of a special prevalence of IgA and IgM immunoglobulin classes are discussed, and special attention to the value of immunofluorescent study of renal diseases, with a review of the literature, is given.
Adult ; Case Report ; Glomerulonephritis/immunology* ; Glomerulonephritis/pathology ; Human ; Immunoglobulin A/analysis* ; Immunoglobulin G/analysis* ; Kidney/ultrastructure ; Male