To investigate the mechanism of crescent formation, sequential pathologic changes from the New Zealand White rabbits with anti-GBM antibody induced GN by administration of guinea pig anti-GBM IgG were studied by light (LM), immunofluorescent (IF) and electron (EM) microscopy. Although no glomerular changes were observed in LM, swelling of the endothelial cells and the epithelial cells were noted in EM by day 2. By day 7, early and cellular crescents were evident. Proteinaceous materials and fibrins were noted in the glomerular capillary lumina (GCL) and Bowman's space (BS) associated with segmental hypercellularity. The GBM damage became progressively severe, followed by focal detachment of the visceral epithelial cells from the GBM. At day 14, fibrin strands, mononuclear cells and collagen fibrils were present between the proliferating extracapillary cells. At day 31, fibrocellular crescents were predominated. Elongated spindle cells, morphologically resembling myofibroblasts, were noted near the Bowman's capsule (BC). A degree of tubular atrophy, interstitial fibrosis, and inflammatory infiltrates increased as it did with fibrous organization of crescent. Intense linear IF staining for IgG and C3 were seen throughout the experiments along the GBM. In conclusion, the progression of crescent from an early "proteinaceous" stage through cellular, fibrocellular and fibrous stages was well documented in this study. Inflammatory cells and coagulation mechanism may activate the initiation of the GBM damage at the early stage. Activated periglomerular mononuclear cells may also cause disruption of BC which facilitates entry of activated periglomerular cells and fibroblasts into BS leading to progressive fibrous crescent formation.
Animals ; Atrophy ; Bowman Capsule ; Capillaries ; Collagen ; Endothelial Cells ; Epithelial Cells ; Fibrin ; Fibroblasts ; Fibrosis ; Glomerulonephritis ; Guinea Pigs ; Immunoglobulin G ; Microscopy ; Myofibroblasts ; Rabbits*

Myxofibrosarcomas (MFSs), which consist of multiple nodules with a variable cellular population in a myxoid matrix, are primarily located in subcutaneous tissue. Pleomorphic hyalinizing angiectatic tumors (PHATs) are rare soft-tissue tumors characterized by a proliferation of highly pleomorphic spindle or polygonal cells and abundant ectatic blood vessels in cellular or myxoid stroma. We present here an unusual case of a high-grade MFS with a PHAT-like appearance. A 67-year-old man presented with an asymptomatic subcutaneous mass in the right forearm. The tumor had myxoid, hypo-, and hypercellular areas with highly pleomorphic spindle or polygonal tumor cells that showed frequent mitoses and nuclear pseudoinclusions. Foci of punctuate necrosis and inflammatory infiltration were present throughout the tumor, and abundant ectatic, thick-walled vessels containing blood clots were noted. The tumor cells were immunohistochemically positive for vimentin but negative for CD34, S-100 protein, smooth muscle actin, desmin, and bcl-2.
Actins ; Aged ; Blood Vessels ; Desmin ; Forearm ; Humans ; Hyalin ; Mitosis ; Muscle, Smooth ; Necrosis ; S100 Proteins ; Sarcoma ; Subcutaneous Tissue ; Vimentin

Gastroesophageal reflux disease (GERD) and sleep disturbances are both common health problems. There is a significant association between disturbed sleep and GERD, and this may be bidirectional. Sleep disorders may induce gastrointestinal (GI) disturbances, while GI symptoms also may provoke or worsen sleep derangements. Reflux of gastric acid is a less frequent event during sleep, however, acid clearance mechanisms (including swallowing, salivation and primary esophageal motility) are impaired during sleep resulting in prolongation of acid contact time. Nighttime reflux can lead to sleep disturbance and sleep disturbance may further aggravate GERD by prolonged acid contact time and heightened sensory perception. This may facilitate the occurrence of complicated GERD and decreased quality of life. However, the interplay between sleep problems and GERD is complex, and there are still relatively limited data on this issue. Further investigation of sleep-related GERD may identify common pathophysiological themes and new therapeutic targets.
Aluminum Hydroxide ; Carbonates ; Deglutition ; Gastric Acid ; Gastroesophageal Reflux ; Quality of Life ; Salivation ; Sleep Wake Disorders

We report an autopsy case of amniotic band syndrome exhibiting microcephaly, asymmetric encephalocele, microphthalmia, nasal deformity, cleft lip and palate accompanied by left maxillary and zygomatic bone deformities. The amniotic membrane of the placenta was also attached to the herniated brain. The twenty-year-old primigravid mother had no history of taking drug, irradiation, infection or trauma before or during pregnancy.
Pregnancy ; Female ; Humans

Cytophagic histiocytic panniculitis is a rescently described histiocytic disorder. It is characterized by the presence of fever, pancytopenia, and subcutaneous nodules resulting from the infiltration of lympho-histiocytes in the dermis and subcutaneous adipose tissue. The characteristic findings is presence of bean-bag histiocytes containing phagocytized red blood cells, lymphocytes, and platelets. We experienced two cases of cytophagic histiocytic panniculitis with hard and erythematous subcutaneous nodules. These skin lesions exhibited infiltration of the subcutaneous tissue by large, benign histiocytes with cytophagic features. Hemophagocytic histiocytes were observed in the cervical lymph node in case 1, and bone marrow in case 2. One patient is alive, while the other one died with hemorrhagic complications.

A wide range of denomination has been used for inflammatory myofibroblastic tumor (IMT). IMT is not entirely homogeneous, even though it shows some overlapping histologic features such as haphazard proliferation of spindle cell and polymorphic chronic inflammatory cell infiltraion. The spindle cell is considered to be of myofibroblastic origin but follicular dendritic cell origin was reported recently. IMT is known as nonneoplastic, aberrant inflammatory response. However, IMT could show local invasion, recurrence, vascular invasion, and malignant transformation, and clonal characteristics and aneuploidy of IMT support the hypothesis that IMT may be a neoplastic process. In order to define the nature of spindle cell of IMT, immunohistochemical stains for smooth muscle actin (SMA), vimentin (VMT), lysozyme, S-100 protein, cytokeratin, CD21 were done. Additional immunohistochemical stains for MIB-1 for proliferating activity and LMP (latent membrane protein) for Epstein-Barr virus (EBV) were done. IMTs were composed of each 2 cases from lung, liver and lymph node and one case from common bile duct, maxillary sinus, bladder and thigh, and were histologically subclassified according to Coffin et al. Nine cases (90%) were positive for SMA and VMT, but no correlation between SMA and VMT immunoreactivity and histologic types was identified. Five cases (50%) were positive for lysozyme and S-100 protein, and histologic type III was negative for lysozyme and S-100 protein, and immunoreactivity for S-100 protein was different according to the histologic subtypes. All 11 cases were negative for CD21 and EBV LMP. MIB-1 labelling index was less than 1% in all cases. In summary, the spindle cell is regarded as myofibroblastic origin rather than follicular dendritic cell origin. Relationship with EBV is not clear, and negligible MIB-1 reaction suggests that IMT might have a good prognosis.
Actins ; Aneuploidy ; Coloring Agents ; Common Bile Duct ; Dendritic Cells, Follicular ; Herpesvirus 4, Human ; Immunohistochemistry ; Keratins ; Liver ; Lung ; Lymph Nodes ; Maxillary Sinus ; Membranes ; Muramidase ; Muscle, Smooth ; Myofibroblasts* ; Prognosis ; Recurrence ; S100 Proteins ; Thigh ; Urinary Bladder ; Vimentin

Ki-1 monoclonal antibody is a well known marker for Reed-Sternberg cells in Hodgkin's disease, but also occasionally reacts with activated lymphoid cells of either benign or malignant nature. Recently, Ki-1 antibody positive Non-Hodgkin's lymphoma, usually of large cell and/or polymorphous type, has been reported in the lymph nodes, skin, soft tissue, and stomach, but not in the bone. We report a case of multifocal primary bone lymphoma in a seven-year old body involving the left shoulder and right frontal bone, which proved to be a large cell, polymorphous lymphoma, helper T-cell type expressing Ki-1 antigen.
Child ; Male ; Female ; Humans

No abstract available.

Both idiopathic pulmonary fibrosis (IPF) and pulmonary involvement of collagen vascular disease(CVD) are well known cause of diffuse interstitial lung disease which lead to fibrosis and honeycombing. We analyzed HRCT findings of 33 patients with IPF and 14 patients with CVD in terms of predominant pattern, site of involvement, mediastinal lymph node enlargement, pleural change and pulmonary volume loss. Criteria of mediastinal lymph node enlargement and pleural thickening were 15mm in long diameter and 3mm, respectively. Volume loss of the lung was measured by using hilar height ratio (apex to hilum/hilum to diaphragmatic dome). Mean age was 61 years for IPF and 46 years for CVD and male: female ratio was 27:6, 4:10, respectively. Predominant HRCT pattern was honeycombing for IPF (63%), and ground-glass opacity for CVD (66%) (p=0.001). Predominantly, subpleural involvement was seen in 90% for IPF and 74% for CVD. Mediastinal lymph node enlargement was seen in 47% of the patient with IPF and 14% with CVD (p=0.004). pleural thickening was seen in 97% of the patients with IPF and 42% with CVD (P=0.002), Pleural effusion was seen in 10% of the patients with IPF and 36% with CVD (P=0.009). Hilar height ratio of more than 1.5 was seen in 84% of the patients with IPF and 29% with CVD. In conclusion, our study shows that patients with IPF are prone to have more progressed stage of pulmonary fibrosis than the patients with CVD on HRCT.
Collagen* ; Female ; Fibrosis ; Humans ; Idiopathic Pulmonary Fibrosis* ; Lung ; Lung Diseases, Interstitial ; Lymph Nodes ; Male ; Pleural Effusion ; Pulmonary Fibrosis

Objective: To evaluate circularity as a quantitative shape factor of small renal tumor on computed tomography (CT) in differentiating fat-poor angiomyolipoma (AML) from renal cell carcinoma (RCC). Materials and Methods: In 257 consecutive patients, 257 pathologically confirmed renal tumors (either AML or RCC less than 4 cm), which did not include visible fat on unenhanced CT, were retrospectively evaluated. A radiologist drew the tumor margin to measure the perimeter and area in all the contrast-enhanced axial CT images. In each image, a quantitative shape factor, circularity, was calculated using the following equation: 4 x π x (area ÷ perimeter 2 ). The median circularity (circularity index) was adopted as a representative value in each tumor. The circularity index was compared between fat-poor AML and RCC, and the receiver operating characteristic (ROC) curve analysis was performed. Univariable and multivariable binary logistic regression analysis was performed to determine the independent predictor of fat-poor AML. Results: Of the 257 tumors, 26 were AMLs and 231 were RCCs (184 clear cell RCCs, 25 papillary RCCs, and 22 chromophobe RCCs). The mean circularity index of AML was significantly lower than that of RCC (0.86 ± 0.04 vs. 0.93 ± 0.02, p < 0.001).The mean circularity index was not different between the subtypes of RCCs (0.93 ± 0.02, 0.92 ± 0.02, and 0.92 ± 0.02 for clear cell, papillary, and chromophobe RCCs, respectively, p = 0.210). The area under the ROC curve of circularity index was 0.924 for differentiating fat-poor AML from RCC. The sensitivity and specificity were 88.5% and 90.9%, respectively (cut-off, 0.90). Lower circularity index (≤ 0.9) was an independent predictor (odds ratio, 41.0; p < 0.001) for predicting fat-poor AML on multivariable logistic regression analysis. Conclusion Circularity is a useful quantitative shape factor of small renal tumor for differentiating fat-poor AML from RCC.