1/27. Fibroglial proliferation in pars planitis.In a 51-year-old patient with long-standing pars planitis, light and electron microscopical examination of the intravitreal 'snowbank' revealed a fibrovascular layer adjacent to the hyperplastic nonpigmented epithelium of the pars plana and an extensive fibroglial proliferation within the vitreous base. The fibrovascular layer consisted of well-differentiated capillaries, probably emanating from the peripheral retina, interspersed with aggregated vitreous fibrils. The fibroglial portion of the 'snowbank' was composed of fibrous astrocyte-like cells which had secreted basement membranes and larger diameter collagen fibrils. This fibroglial tissue was in direct continuity with an ultrastructurally similar preretinal membrane. No significant choroiditis or cyclitis could be demonstrated. It is hypothesized that, in pars planitis, a primary inflammatory process of the peripheral retina and vitreous base may stimulate the observed preretinal and intravitreal fibroglial proliferation.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
2/27. Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin alpha3.BACKGROUND: The inflammatory variant of epidermolysis bullosa acquisita (EBA) may clinically closely resemble bullous or cicatricial pemphigoid. patients with inflammatory EBA have IgG autoantibodies against type VII collagen. patients with anti-epiligrin cicatricial pemphigoid have IgG autoantibodies against laminin 5. observation: We describe a patient with inflammatory EBA exhibiting nonscarring oral and vaginal involvement. Indirect immunofluorescence using skin substrate lacking an epidermal basement membrane molecule, direct immunoelectron microscopy, immunoblot, and immunoprecipitation studies revealed the simultaneous presence of circulating IgG autoantibodies against type VII collagen and laminin alpha3. A final diagnosis of EBA was based on the sublamina densa level of blister formation. CONCLUSION: This case illustrates the clinical and immunological overlap between EBA and anti-epiligrin cicatricial pemphigoid, a unique finding that may have developed as a consequence of epitope spreading.- - - - - - - - - - ranking = 0.5keywords = membrane (Clic here for more details about this article) |
3/27. Fetal varicella syndrome: disruption of neural development and persistent inflammation of non-neural tissues.Primary varicella zoster virus (VZV) infection during pregnancy is rare. If it occurs between the 8th and 20th week of gestation, fetal varicella syndrome results in 1-2% of the fetuses. We report about a varicella infection that affected a pregnant mother in the 12th week of gestation. At 33 weeks, a premature girl was born with destruction of neurons in spinal cord, spinal ganglia and plexus myentericus, and secondary developmental disturbance including mummification of one arm and segmental intestinal atresia. The brain did not show any abnormalities. However, VZV dna could be detected by PCR in tissues from the brain and spinal ganglia. Chronic necrotizing inflammation was found in the placenta, fetal membranes, and one ovary. These locations showed nuclear inclusions which by in-situ-hybridization were proven to be VZV derived. This case demonstrates that in the fetal age, 'neurotropism' of VZV signifies severe destruction but not necessarily persistent inflammation of neural tissue. However, due to the inefficient fetal immune system, inflammation can go on for weeks, preferentially in non-neural tissues.- - - - - - - - - - ranking = 0.5keywords = membrane (Clic here for more details about this article) |
4/27. Pulmonary involvement in a child with ligneous conjunctivitis and homozygous type I plasminogen deficiency.Ligneous conjunctivitis (LC) is a rare disorder characterized by a chronic course of recurrent membranous lesions at conjunctivae. Pseudomembranes of other mucous membranes have been reported in patients with LC, but to the best of our knowledge, no case with alveolar involvement has been described. Here, we report a 2.5-year-old girl with LC who had tracheoaveolar involvement and homozygous type I plasminogen deficiency. Tracheal involvement was diagnosed by bronchoscopic biopsy and alveolar involvement with postmortem biopsy. She was shown to be homozygous for frameshift mutation in plasminogen exon 14 (Gly565ins-G) with molecular genetic examination of dna which was obtained from parafin embedded postmortem lung tissue. Ligneous inflammation of the upper and lower respiratory tract must be considered in children with LC and recurrent respiratory tract problems.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
5/27. Perinatal cytomegalovirus infection complicated with pneumonitis and adrenalitis in a premature infant.cytomegalovirus causes pneumonia, hepatitis, thrombocytopenia, and hemolytic anemia. cytomegalovirus adrenalitis in premature infants, however, is rare. This report described a premature newborn who had progressively worsening hyperbilirubinemia, pancytopenia, and hepatosplenomegaly at the age of 4 days. The baby's mother had prolonged rupture of amniotic membrane for about 8 weeks. The infant received exchange blood transfusion, empiric antibiotics treatment, and mechanical ventilation. pneumonia and sepsis developed at the age of 18 days. serum anticytomegalovirus immunoglobulin m and urine virus culture were positive for cytomegalovirus. The baby died at the age of 22 days. autopsy showed cytomegalovirus infection complicated with interstitial pneumonitis and pulmonary edema, subacute bronchopulmonary dysplasia with interstitial fibrosis, and adrenalitis. We concluded that the functional status of the adrenal glands in cytomegalovirus-infected premature newborns who have unexplained electrolytes imbalance, fever, diarrhea, weight loss, or hypotension should be closely followed because of the possible involvement of adrenal glands.- - - - - - - - - - ranking = 0.5keywords = membrane (Clic here for more details about this article) |
6/27. Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor.Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal proliferation of transformed myofibroblasts, with a prominent inflammatory cell component, that can mimic other spindle cell processes such as nodular fasciitis, desmoid tumor, and gastrointestinal stromal tumor. Genetic analyses have recently demonstrated rearrangements of anaplastic lymphoma kinase (ALK), located at 2p23, in a subset of IMTs. Molecular characterizations have identified ALK fusions involving tropomyosin-3 and -4 (TPM-3 and -4), the clathrin heavy chain (CLTC), and the cysteinyl-tRNA synthetase (CARS) genes as fusion partners. Here we describe two IMTs with a novel ALK fusion that involves the Ran-binding protein 2 (RANBP2) gene at 2q13, which normally encodes a large (358-kDa) nucleopore protein localized at the cytoplasmic side of the nuclear pore complex. The N-terminal 867 residues of RANBP2 are fused to the cytoplasmic segment of ALK in the 1,430-amino acid RANBP2-ALK chimeric protein. myofibroblasts that express RANBP2-ALK exhibit nuclear membrane-associated ALK staining that is unique compared to the subcellular localization observed with other ALK fusions in IMT, presumably attributable to heteroassociation of the fusion with normal RANBP2 at the nuclear pore. These findings expand the spectrum of ALK abnormalities observed in IMT and further confirm the clonal, neoplastic nature of these lesions.- - - - - - - - - - ranking = 0.5keywords = membrane (Clic here for more details about this article) |
7/27. Histopathology of severe childhood asthma: a case series.BACKGROUND: To date, little has been published describing the pathology of severe childhood asthma. The currently accepted model of asthma holds that persistent airway inflammation leads to various symptoms of asthma, airway hyperresponsiveness, and airway remodeling that ultimately results in permanent loss of lung function. methods: Evaluation of six children referred to the National Jewish Medical and research Center with difficult-to-control asthma, despite aggressive anti-inflammatory therapy, who underwent bronchoscopy with endobronchial biopsy to better characterize their disease. RESULTS: In every case, endobronchial biopsies revealed changes consistent with airway remodeling characterized by thickening of the basement membrane, smooth-muscle hypertrophy, with varying degrees of goblet-cell and submucous gland hyperplasia. The degree of subbasement membrane thickening did not appear to correlate with baseline FEV(1), ultimate FEV(1) following aggressive therapy, or lability in lung function. In five of six cases, there was minimal to no histologic evidence for airway inflammation with mild and patchy submucosal lymphocytic infiltration noted; eosinophils and neutrophils were not present. Further, the majority of the patients achieved normal FEV(1) values despite significant subbasement membrane thickening, counter to the current beliefs regarding airway remodeling and irreversible loss of lung function. CONCLUSIONS: This case report highlights some of the shortcomings of the current inflammatory paradigm for severe asthma. Despite little evidence of ongoing airway inflammation, many of the subjects displayed significant lung function lability. The lack of inflammation argues against steroid resistance at a cellular level, although it could be argued that inflammation may have been distal to the site sampled. Additionally, normal to nearly normal lung function was achieved despite the presence of significant remodeling. These findings suggest the need to look beyond inflammation to fully treat severe asthma and ultimately alter its progression.- - - - - - - - - - ranking = 1.5keywords = membrane (Clic here for more details about this article) |
8/27. Highly anaplastic extraventricular ependymoma arising in an adult, mimicking metastatic adenocarcinoma with heavy stromal inflammation and emperiporesis.We report a case of extraventricular ependymoma arising in a 50-year-old woman that took an aggressive clinical course with recurrence three times. The initial tumor was a well-circumscribed nodule in the right temporal white matter measuring 2 cm in diameter. It showed variegated histological findings mimicking metastatic adenocarcinoma: an epithelioid arrangement of highly pleomorphic cells with pseudopapillary structures and perivascular pseudorosettes, and bizarre multinucleated giant cells with occasional emperiporesis surrounded by abundant mononuclear inflammatory cells, as well as a focal small area of conventional ependymoma. Emperiporesis and abundant mononuclear cell infiltration were not previously described in an ependymoma. The recurrent tumors predominantly showed an epithelioid pattern with frequent formation of astroblastoma-like pseudopapillary structures. Neoplastic cells were markedly atypical and had characteristic intracytoplasmic eosinophilic inclusion bodies. Much of the cells in both the initial and recurrent tumors showed a positive immunostaining for glial fibrillary acidic protein (GFAP) with accentuation to the cytoplasmic processes of the pseudorosettes and pseudopapillary structures. Epithelial membrane antigen (EMA) highlighted the epithelial differentiation of the tumor cells, while cytokeratin was completely negative. Although this tumor might be classified to at least WHO grade III from the histology and aggressive behavior, the exact grading is still controversial because of the rarity of such cases.- - - - - - - - - - ranking = 0.5keywords = membrane (Clic here for more details about this article) |
9/27. Nonfunctional abdominal complications of the distal catheter on the treatment of hydrocephalus: an inflammatory hypothesis? Experience with six cases.INTRODUCTION: The peritoneal cavity is the most common site of cerebrospinal fluid absorption in hydrocephalus treatment. Many distal catheter complications are the result of this type of treatment, and these have been extensively described in the neurosurgical literature. MATERIALS AND methods: In our study, six cases of distal catheter migration with visceral perforation and/or extrusion are presented: three through the umbilicus, two through the scrotum, and one through the anus. An extensive review of the literature was performed. RESULTS: The studies of peritoneal dialysis models for the treatment of chronic renal failure patients provide important data about solute absorption in the peritoneal cavity and reactivity of the peritoneal membrane. CONCLUSION: This model, when compared to distal catheter complications on a ventriculoperitoneal (VP) shunt, presents similarities that could help understand the mechanism of the nonfunctional complications of the distal VP catheter (complication with functional shunt), providing valuable data to support an inflammatory mechanism.- - - - - - - - - - ranking = 0.5keywords = membrane (Clic here for more details about this article) |
10/27. Unexpected disseminated herpes simplex infection in a newborn.Disseminated herpes simplex virus type 2 was unexpectedly discovered in a baby delivered by primary cesarean section at 36 weeks' gestation prior to rupture of the membranes. The mother was free of orogenital lesions at each prenatal examination. Examination of the placenta revealed chorioamnionitis implicating an ascending infection from the mother's infected genital tract.- - - - - - - - - - ranking = 0.5keywords = membrane (Clic here for more details about this article) |
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