1/259. Idiopathic oedema with increased cytokine production: a pathogenetic link?We describe four patients with idiopathic oedema and elevated cytokines. All patients shared increased serum concentrations of soluble interleukin-2 receptors (sIL-2R). In three patients tumour necrosis factor alpha (TNF-alpha), gamma interferon (IFN-gamma) and interleukin-2 (IL-2) were transiently elevated. There was no evidence for an underlying disease. Based on experimental and clinical data it is hypothesized that oedema formation in our patients is the consequence of cytokine induced alteration of endothelial cells.- - - - - - - - - - ranking = 1keywords = duct (Clic here for more details about this article) |
2/259. Successful use of alternate waste nitrogen agents and hemodialysis in a patient with hyperammonemic coma after heart-lung transplantation.BACKGROUND: Lethal hyperammonemic coma has been reported in 2 adults after lung transplantation. It was associated with a massive elevation of brain glutamine levels, while plasma glutamine levels were normal or only slightly elevated. In liver tissue, glutamine synthetase activity was markedly reduced, and the histologic findings resembled those of reye syndrome. The adequacy of therapy commonly used for inherited disorders of the urea cycle has not been adequately evaluated in patients with this form of secondary hyperammonemia. OBJECTIVE: To determine whether hemodialysis, in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy, would be efficacious in a patient with hyperammonemic coma after solid-organ transplantation. DESIGN: Case report. SETTING: A children's hospital. PATIENT: A 41-year-old woman with congenital heart disease developed a hyperammonemic coma with brain edema 19 days after undergoing a combined heart and lung transplantation. methods: Ammonium was measured in plasma. amino acids were quantitated in plasma and cerebrospinal fluid by column chromatography. The effectiveness of therapy was assessed by measuring plasma ammonium levels and intracranial pressure and performing sequential neurological examinations. RESULTS: The patient had the anomalous combination of increased cerebrospinal fluid and decreased plasma glutamine levels. To our knowledge, she is the first patient with this complication after solid-organ transplantation to survive after combined therapy with sodium phenylacetate, sodium benzoate, arginine hydrochloride, and hemodialysis. Complications of the acute coma included focal motor seizures, which were controlled with carbamazepine, and difficulty with short-term memory. CONCLUSIONS: The aggressive use of hemodialysis in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy may allow survival in patients after solid-organ transplantation. An acute acquired derangement in extra-central nervous system glutamine metabolism may play a role in the production of hyperammonemia in this illness that resembles reye syndrome, and, as in other hyperammonemic disorders, the duration and degree of elevation of brain glutamine levels may be the important determining factors in responsiveness to therapy.- - - - - - - - - - ranking = 0.25keywords = duct (Clic here for more details about this article) |
3/259. smith-lemli-opitz syndrome presenting with persisting nuchal oedema and non-immune hydrops.smith-lemli-opitz syndrome (SLO) is a recognized clinical entity with distinctive anomalies. Recently it has been shown that a specific defect in cholesterol metabolism, 7-dehydroxycholesterol reductase deficiency, causes the multiple abnormalities seen in SLO. There have been two reports of first-trimester nuchal translucency associated with SLO. We report two cases of SLO in the third trimester, one with persisting nuchal oedema and the other presenting with hydrops. These findings may explain a proportion of the perinatal loss associated with this syndrome.- - - - - - - - - - ranking = 0.25keywords = duct (Clic here for more details about this article) |
4/259. The cystic fibrosis conductance regulator gene exon sequence is normal in a patient with edematous eosinophilic nasal polyps.nasal polyps are the most common mass lesions found in the nose and their etiology is unknown. nasal polyps from cystic fibrosis (CF) patients are histologically distinct from nasal polyps from patients without CF. It has been suggested that a mutation (G551D) of the cystic fibrosis transmembrane conductance regulator (CFTR) gene may play a role in nasal polyp formation in patients without CF. To investigate the possibility that this or other CFTR gene exon mutations are required for nasal polyp formation, the CFTR gene exons were sequenced from peripheral blood dna derived from an adult patient with edematous eosinophilic nasal polyps and no personal or family history of CF. No mutations or deletions were identified in any of the CFTR exons. A single polymorphism (A or G) was found in exon 10, base pair 1540, amino acid 470. This polymorphism was detected in 11 of 16 subjects (69%) with edematous eosinophilic nasal polyps and 10 of 21 normal subjects (48%) without nasal polyps and was not statistically significant (p = 0.316). These results demonstrate that mutations of the CFTR coding region are not a prerequisite for the formation of edematous eosinophilic nasal polyps.- - - - - - - - - - ranking = 1.25keywords = duct (Clic here for more details about this article) |
5/259. Production of anterior segment ischemia.Anterior segment ischemia changes can occur without detachment of any muscles. The most common cause of such ischemic changes of the anterior segment is the removal of too many rectus muscles in one operation. Twenty dog eyes and eight monkey eyes were subjected to the disinsertion and detachment of various combinations of extraocular muscles. They were sacrificed at intervals from 30 to 90 days. During the observation period, they were observed for gross and slit lamp changes. The enucleated eyes were studied microscopically for signs of ischemic and necrotic changes. Two patients who were studied, observed, and treated for anterior segment ischemia following muscle surgery are described. The changes which occur after muscle surgery are extensive and include corneal edema, cataract, chemosis, corneal changes, decreases in intraocular pressure, decreases in outflow or glaucoma and frank necrosis. The variables which lead to this reaction is described in detail. Also, some unanswered queries, such as the duration of the reaction and the time interval of the reaction after multiple muscle surgeries, are discussed.- - - - - - - - - - ranking = 1keywords = duct (Clic here for more details about this article) |
6/259. Lipodermatosclerosis - report of three cases and review of the literature.We report 3 cases of lipodermatosclerosis (LDS) and discuss the nosology of similar disorders caused by venous insufficiency of the legs. These cases are characterized by (1) occurrence in middle-aged or aged woman, (2) painful, indurated erythema with hyperpigmented scleroderma-like hardening on the lower leg, (3) lobular panniculitis with membranocystic fat necrosis and various degrees of septal fibrosis. Although the designation LDS has been used particularly in the UK and in the USA, this entity is not familiar in other countries including japan. LDS clinically represents a wide spectrum from an acute, inflammatory phase to a chronic, fibrotic state. The clinicopathologic findings of LDS are similar or identical to the disease previously reported as chronic indurated cellulitis, hypodermitis sclerodermiformis, stasis panniculitis or sclerosing panniculitis. These diseases are probably related conditions, which depend upon the various stages.- - - - - - - - - - ranking = 65.84005999947keywords = stasis (Clic here for more details about this article) |
7/259. Complications of total abdominal and spleen irradiation in patients with lymphomas.Fifty patients with non-Hodgkin's lymphoma were treated with total abdominal irradiation to a dose of 3000 rad by anterior and posterior fields treated the same day. Fourteen patients developed complications, four with intestinal obstruction due to stenosis and three with total or partial intestinal irradiation damage. These bowel complications were only seen in patients who had previously had a laparotomy. The ways of preventing these complications are discussed. We have studied renal function by means of clinical examination, biologic and radiologic investigations, and scanning in 75 patients who have received splenic irradiation to a dose of 4000 rad. With a mean follow-up time of 36 months, this study has shown that there was no significant change in blood pressure or in biochemical measurements. However, at the 17th month, nephrotomograms demonstrated cortical atrophy of the upper role of the kidney; at the 8th month, low uptake in the irradiated area was seen on 197Hg neohydrine scanning and a decrease in renal plasma flow in the left kidney during dynamic studies with Hippuran.- - - - - - - - - - ranking = 22.736023424591keywords = obstruction (Clic here for more details about this article) |
8/259. Conjunctival edema and alopecia of the external third of the eyebrows in a patient with meige syndrome.PURPOSE: To describe a patient with meige syndrome in whom we observed the coexistence of hereditary lymphedema of the lower legs, conjunctival edema and alopecia of the lateral third of the eyebrows. methods: Case report. RESULTS: Histological examination of the conjunctival and skin specimens showed dermal edema and a slight reduction in the number of severely ectatic lymphatics in the reticular dermis. The vessel were identified as lymphatics on the basis of immunohistochemical evidence of discontinuity and/or absence of basement membrane. CONCLUSIONS: Clinical and histological findings suggest that the etiopathogenesis of the edema in meige syndrome is related to a structural ectatic defect of lymphatics. This anomaly seems to involve both skin and other sites, such as conjunctival mucosa.- - - - - - - - - - ranking = 0.25keywords = duct (Clic here for more details about this article) |
9/259. Solid facial edema preceding a diagnosis of retro-orbital B-cell lymphoma.Persistent solid facial edema is a rare condition of unknown cause. Although acute facial edema has been associated with numerous disease processes such as infections, neoplasms, immune disorders, inflammation, neuropathic processes, drugs, mechanical obstructions, and trauma, solid facial edema has most often been associated with acne vulgaris. We report the first case, to our knowledge, of solid facial edema preceding a diagnosis of a subcutaneous scalp and orbital/periorbital B-cell lymphoma.- - - - - - - - - - ranking = 22.736023424591keywords = obstruction (Clic here for more details about this article) |
10/259. Solid facial edema in a patient with rosacea.We report a 53-year old man with symmetrical nonpitting edema, conjunctivitis, and acneiform eruptions on the face. Histopathological examination showed perifollicular lymphohistiocytic infiltration and telangiectasias in the upper dermis. Loosely aggregated non-caseating granulomas were scattered through the dermis; some of them were seen in the perifollicular regions. The patient was treated with fleroxacin (100 mg/day, orally) for two weeks with a marked reduction of both solid facial edema and periorbital edema.- - - - - - - - - - ranking = 0.25keywords = duct (Clic here for more details about this article) |
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