OBJECTIVETo analysis the clinical and pathological characteristics of children with dense deposit disease (DDD).

METHODS12 Children diagnosed as DDD by electron microscope were enrolled in this study. The clinical and pathological data were analyzed.

RESULTSOf the 12 cases, 7 were males and 5 females, mean age 9.1 +/- 3.9 (5-13) years at onset, the duration from onset to renal biopsy was 1 month to 5 years and the follow-up period was 1-9 years. All cases had heavy proteinuria >50 mg/(kg x d), and persistent microscopic hematuria with recurrent gross hematuria during the course. Seven cases had hypertension (> or = 140/100 mm Hg, 1 mm Hg =0. 133 kPa), 5 cases had transient or recurrent abnormal renal function, and mild to severe anemia were observed in 8 cases respectively. All the cases had lower serum C3 (0.15-0.55 g/L). Clinically, 10 cases were diagnosed as nephritic syndrome (one case had partial lipodystrophy at the same time), and 2 cases were diagnosed as acute nephritic syndrome. Immunofluorescence study showed intense deposition of C3 along GBM, TBM and the wall of Bowman's capsule in a ribbon-like pattern and in the mesangial regions as coarse granules in all the cases. Under light microscopy, 9 cases showed the feature of membrane proliferative glomerulonephritis (MPGN), 1 case with focal segmental glomerulosclerosis (FSGS), 1 case with endocapillary proliferative glomerulonephritis (EnPGN) and 1 case with proliferative sclerosis (PSGN). Crescents were seen in 3 cases. Under electron microscopy, ribbon-like or linear electron-dense intramembranous deposits were identified in the lamina dense of GBM, and often along TBM and the wall of Bowman's capsule. All patients showed steroid resistance. After methylprednisone treatment, some patients showed transient remission. During the follow- up stage of 1-9 years, 3 cases showed normal urinalysis, 5 cases showed partial remission, 2 cases progressed to end stage renal disease (ESRD) and 2 cases were lost.

CONCLUSIONDDD is an in dependently rare disease with pathological-clinical varieties. Children with DDD presented with persistently lower C3, heavy proteinuria, recurrent gross hematuria and anemia. The characteristic immunopathologic finding is intense deposition of C3 along the GBM. Under electron microscopy, ribbon-like or linear electron-dense deposits in the lamina dense of the GBM, TBM and the wall of Bowman's capsule. Electron microscopic examination to demonstrate the intramembranous dense deposits is definitive diagnosis, regardless of the finding of light microscopy. All of them showed steroid resistant. Patients with steroid and CTX treatment showed some clinical improvement of their urinalysis.

Adolescent ; Child ; Child, Preschool ; Female ; Glomerular Basement Membrane ; pathology ; Glomerulonephritis, Membranoproliferative ; diagnosis ; pathology ; therapy ; Humans ; Male

Morphological studies have an important role in establishing the timing of irreversible damage occurred in cryptorchid testis. The present study was undertaken to determine how soon definitive sign or testis pathology may appear in cyptorchid testis. Testicular biopsies were collected at the time of orchiopexy from 79 patients during the period from January, 1988 to June, 1990, and then we examined ultrastructural changes of cryptorchid testis with light and electron microscopy. Comparing with normal testis, following results were obtained ; l. The volume of cryptorchid testis became smaller than normal after age of 14. 2. Mean tubular diameter became smaller after age of 14 and basement membrane thickened prominently after age of 20. 3. The increase number of mitochondria appeared at age of 2 4. Cytoplasmic vacuolization became prominent after age of 6, but detachment and loss of ribosome appeared at age of 20. 5. Tunica propria showed marked thickening and collagenous degeneration after age of 14. With above results, we suggest that orchiopexy should be done before age of 2.
Basement Membrane ; Biopsy ; Collagen ; Cytoplasm ; Humans ; Microscopy, Electron ; Mitochondria ; Orchiopexy ; Pathology ; Ribosomes ; Testis*

BACKGROUND: Airway hyperresponsiveness is expressed as the provocative dose or concentration of the stimulus required to achieve bronchoconstriction, a 20% fall in FEV1 (PD20 and PC20, respectively). A decrease in PC20 may be due to a steeper dose-response curve (hyperreactivity) or to a shift in the curve to the left (hypersensitivity), or both. It has been suggested that many factors, such as genetic factor, airway inflammation, epithelial damage or airway remodeling, are involved in the airway hyperresponsiveness in asthma. OBJECTIVE: In this study, we analyzed the relationship of airway sensitivity and reactivity with bronchial inflammation and structural change in asthmatics. METHOD: The PC20 for methacholine, as the airway sensitivity parameter, and the slope between PC20 and PC40, as the airway reactivity parameter, were measured. Total cell counts and differential cell counts in BAL fluid, percentage of epithelial shedding (ES), basement membrane thickness (BMT) and depth of submucosal collagen deposition (SMC) on bronchial tissue were measured. The patients (n=27) were divided into two groups by median values of ES, BMT, or SMC (32%, 7.3 micrometer, 68 micrometer, respectively). RESULTS: The PC20 showed a significant correlation with baseline FEV1% (r=0.498, p<0.05), and was significantly lower in patients with over 32% of ES than in those with under 32% of ES (2.89+/-1.05 mg/ml vs 5.70+/-3.70 mg/ml, p<0.05). The slope was significantly steeper in patients with thicker BMT or SMC. CONCLUSION: These results suggest that airway hypersensitivity is affected by airway caliber, and airway hyperreactivity is affected by bronchial remodeling in asthma.
Airway Remodeling ; Asthma ; Basement Membrane ; Bronchoconstriction ; Cell Count ; Collagen ; Humans ; Hypersensitivity ; Inflammation ; Methacholine Chloride ; Pathology*

BACKGROUND AND OBJECTIVES: Chronic sinusitis has been closely related to bronchial asthma. Patients with both sinusitis and asthma have showed somewhat different mucosal appearance and pathology, compared to those without asthma. We investigated histopathological features of these patients. SUBJECTS AND METHOD: 19 sinusitis patients with asthma who had undergone endoscopic sinus surgery from April, 1995 through September, 1997, and 53 patients without asthma who had undergone surgery from January, 1997 through July, 1997 were evaluated. We compared the following 7 parameters of sinus mucosal histopathology between the asthma and non-asthma group by reviewing histopathological slides: basement membrane thickening, goblet cell hyperplasia, subepithelial edema, submucous gland formation, eosinophilic infiltration, lymphocyte infiltration, polymorphonuclear leukocyte infiltration. We also compared preoperative disease extent, evaluated by degree of polyposis and OMC CT findings, and presence of allergy, which might affect the sinus mucosal pathology. RESULTS: There revealed no statistical difference between two groups on presence of allergy, preoperative polyposis, and OMC CT scores. However, the asthma group showed significant basement membrane thickening, goblet cell hyperplasia, and eosinophilic infiltration, which was statistically significant. No difference was found between subepithelial edema, submucous gland formation, lymphocyte infiltration, and polymorphonuclear leukocyte infiltration. CONCLUSION: Significant histopathological features such as basement membrane thickening, goblet cell hyperplasia, and eosinophil infiltration characterized chronic sinusitis with asthma; however, there were no differences owing to the presence of allergy or the extent of preoperative disease. Adequate preoperative management, close attention during surgery and careful follow-up would be necessary.
Asthma* ; Basement Membrane ; Edema ; Eosinophils ; Goblet Cells ; Humans ; Hyperplasia ; Hypersensitivity ; Lymphocytes ; Neutrophils ; Pathology ; Sinusitis*

Epidermolysis bullosa (EB) comprises a collection of clinically diverse inherited blistering diseases that affect the skin and, in some subtypes, mucous membranes and other organs. Currently classified into four main subtypes (EB simplex, junctional EB, dystrophic EB, and Kindler syndrome, mainly based on the level of skin cleavage), the spectrum of EB extends to more than 30 clinical subtypes with pathogenic mutations in at least 18 distinct genes. This review focuses on three recent additions to variants of EB: all are autosomal recessive, and result from mutations in either DST-e (coding for epidermal dystonin, also known as the 230 kDa bullous pemphigoid antigen, BP230), EXPH5 (coding for exophilin-5, also known as Slac2-b), or ITGA3 (coding for the integrin alpha-3 subunit). Each of these new forms of EB is reviewed with respect to the initial gene discovery, clinical features, the current mutation database, and skin pathology. Awareness of these recently described forms of EB is helpful in the clinical evaluation of patients with EB and in defining genotype-phenotype correlation for inherited blistering skin diseases.
Basement Membrane ; Blister ; Epidermolysis Bullosa* ; Genetic Association Studies ; Hemidesmosomes ; Humans ; Mucous Membrane ; Pathology ; Pemphigoid, Bullous ; Skin ; Skin Diseases ; Transport Vesicles

Diffuse glomerular basement membrane (GBM) lamellation, reminiscent of Alport's syndrome, has rarely, and exclusively, been reported in renal allografts from pediatric donors to adult recipients. We report on a similar lesion, identified in a 42-year-old male, who received a kidney from an unrelated 21-year-old living male donor. The disease of the recipient was unknown. Renal allograft biopsies were performed 3.5 and 4.8 years after the renal transplantation, due to massive proteinuria and serum creatinine elevation. The histological features of both biopsies were similar, but more advanced in the second biopsy. Glomerular mesangium was widened and had an IgA deposit in the first biopsy. In addition to the presence of mesangial electron dense deposits, the GBM showed diffuse lamellation and splintering on the subepithelial side, but no definite deposits. In the second biopsy, IgA deposits were extended to the peripheral capillary walls, but electron microscopic examination was not available. Two months after the second biopsy, the patient returned for hemodialysis.
Adult ; Basement Membrane/*pathology ; Case Report ; Glomerulonephritis, IGA/*etiology/*pathology ; Human ; Kidney Glomerulus/*pathology ; *Kidney Transplantation/*adverse effects ; Male

Diffuse glomerular basement membrane (GBM) lamellation, reminiscent of Alport's syndrome, has rarely, and exclusively, been reported in renal allografts from pediatric donors to adult recipients. We report on a similar lesion, identified in a 42-year-old male, who received a kidney from an unrelated 21-year-old living male donor. The disease of the recipient was unknown. Renal allograft biopsies were performed 3.5 and 4.8 years after the renal transplantation, due to massive proteinuria and serum creatinine elevation. The histological features of both biopsies were similar, but more advanced in the second biopsy. Glomerular mesangium was widened and had an IgA deposit in the first biopsy. In addition to the presence of mesangial electron dense deposits, the GBM showed diffuse lamellation and splintering on the subepithelial side, but no definite deposits. In the second biopsy, IgA deposits were extended to the peripheral capillary walls, but electron microscopic examination was not available. Two months after the second biopsy, the patient returned for hemodialysis.
Adult ; Basement Membrane/*pathology ; Case Report ; Glomerulonephritis, IGA/*etiology/*pathology ; Human ; Kidney Glomerulus/*pathology ; *Kidney Transplantation/*adverse effects ; Male

OBJECTIVETo study the influence of alcohol on the liver sinusoids endothelial cell (LSEC) fenestrae of rats.

METHODSSetting up the rat model of alcoholic liver disease by orogastric administration of alcohol, then kill the experimental and control groups of rats at the end of 4 weeks, 8 weeks and 12 weeks after alcohol feeding, and also at the end of another 12 weeks after balance foods feeding succeeding with alcohol feeding for 12 weeks. Staining the liver tissue by means of HE method and observing the successive change of LSEC fenestrae by transmission electron microscope.

RESULTSThe normal LSEC was flat with nucleus and organelle arranged regularly. The distal cytoplasm displayed as lamina with many fenestrae, not accompanied by basement membrane (BM) formation under the endothelial cell. At the end of 4 weeks of alcohol feeding, fenestrae decreased at the partial distal LSEC cytoplasm, but no BM developed. At the end of 8 weeks, fenestrae decreased significantly, even disappeared, with the BM developed incompletely under the endothelial cell. Concomitantly, fibroblast with active function developed. At the end of 12 weeks, the changes became more obvious; the complete BM could even be seen. However, this kind of changes was mostly limited in the single or adjoining sinusoids, as well as with little widespread formation of fibrosis. At the end of 12 weeks of stopping alcohol feeding, defenestrae and development of BM attenuated obviously.

CONCLUSIONThe defenestrae and BM of LSEC develop gradually with the chronic alcohol stimulation. Sinusoid capillarization and liver fibrosis even form when significant changes happen. The early change of the limited defenestrae and capillarization may be the basis of alcohol periportal fibrosis formation. This kind of liver fibrosis can be reversible after stopping alcohol feeding.

Animals ; Basement Membrane ; pathology ; Endothelium ; drug effects ; pathology ; Ethanol ; Liver ; blood supply ; pathology ; Liver Cirrhosis, Experimental ; pathology ; Liver Diseases, Alcoholic ; pathology ; Male ; Rats ; Rats, Wistar

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

Adolescent ; Anti-Glomerular Basement Membrane Disease ; pathology ; Diagnosis, Differential ; Granulomatosis with Polyangiitis ; pathology ; Humans ; Kidney ; pathology ; Lung ; pathology ; Lupus Erythematosus, Systemic ; pathology ; Male