1/152. 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 = formation, production (Clic here for more details about this article) |
2/152. 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 = 0.16464368283338keywords = production (Clic here for more details about this article) |
3/152. 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 = 1.0242758059995keywords = formation (Clic here for more details about this article) |
4/152. infectious mononucleosis.infectious mononucleosis is a unique disease in its hematologic aspects; it is different from the frequently occurring acute microbial diseases in that it affects primarily the reticuloendothelial system; and it is interesting serologically because of the heterophil antibody reaction, as well as the multiplicity of antibodies which may be produced. The diagnosis should be suspected clinically before hematology is reported - by remembering the prototypes. In fact, a patient between 16 and 25 years old complains of sore throat and fever is more likely to have infectious mononucleosis than another disease; and if - in addition - he is jaundiced, a diagnosis of infectious mononucleosis is almost certain. Finally, a negative result of treatment with corticosteroid has the diagnostic significance mentioned above. Positive effect of treatment has no diagnostic significance.- - - - - - - - - - ranking = 1.8733802606333keywords = antibody (Clic here for more details about this article) |
5/152. spinal cord swelling preceding syrinx development. Case report.The pathophysiology of syrinx development is controversial. The authors report on a patient with progressive cervical myelopathy and a Chiari I malformation in whom spinal cord swelling preceded, by a few months, the development of a syrinx in the same location. The patient underwent a craniocervical decompressive procedure and duraplasty, and complete resolution of cord swelling and syringomyelia was achieved. This report is consistent with the theory that patients with Chiari I malformation have increased transmural flow of cerebrospinal fluid, which causes spinal cord swelling that later coalesces into a syrinx. The pathophysiology of syrinx development from spinal cord edema and the success of surgical decompressive treatments that do not invade the central nervous system support the prompt treatment of patients with spinal cord edema who are at risk for the development of a syrinx.- - - - - - - - - - ranking = 0.68285053733299keywords = formation (Clic here for more details about this article) |
6/152. Acute glomerulonephritis after human parvovirus B19 infection.We evaluated clinical and histological characteristics of four adult patients who presented with acute glomerulonephritic syndrome with serological confirmation of recent HPB19 infection. All patients had generalized edema with urinary abnormalities. body weight gain ranged from 3 to 10 kg. Three of the patients had contact with erythema infectiosum simultaneously with or within 10 days before development of flu-like symptoms. Two patients had an erythematous rash, and one patient had lower-extremity purpura. Joint pain was present in three of the patients. All patients had proteinuria and hematuria. Renal functions were normal except in one patient who had a serum creatinine of 3.2 mg/dL. Three of the patients had hypocomplementemia. All renal biopsy specimens were characterized by glomerular leukocyte infiltration and endothelial cell swelling. Mesangiolysis was seen in three of the patients. C3 was deposited in a coarse granular pattern along the capillary walls in all cases. Electron microscopic examination showed marked expansion of the subendothelial space of glomerular capillaries in all patients. Subendothelial electron-dense deposits were present in all patients. Immunohistochemical analysis using monoclonal anti-HPB19 antibody showed that one of the four patients had positive staining in the glomeruli. dna extracted from renal biopsy specimens contained HPB19 dna, as shown by polymerase chain reaction (PCR) analysis in all patients. PCR amplification of the renal dna generated a 104-bp product, which hybridized to an HPB19-specific probe. No control group subjects contained HPB19 dna as determined by PCR. This circumstantial evidence indicates that HPB19 infection may be one of the causes of acute glomerulonephritis in normal individuals.- - - - - - - - - - ranking = 1.8733802606333keywords = antibody (Clic here for more details about this article) |
7/152. Transient oedema of the cervical spinal cord.Transient but very intense oedema of the cervical spinal cord was observed in two patients with obstruction of the cerebrospinal fluid (CSF) pathways. Both presented with hydrocephalus, one due to an infratentorial obstructing mass and the other due to postmeningitic adhesive obstruction of the outlet foramina of the fourth ventricle. In animal experiments with obstruction of CSF pathways (due to outlet foramina obstruction or to downward tentorial herniation) flattening and stretching of the ependymal cells along the central canal is observed, followed by disruption and splitting of the ependymal lining and then by extracellular oedema of the subependymal tissue. Without treatment, frank cavity formation develops in a fourth stage. In our two patients, however, most probably because of appropriate decompressive therapy, the oedema disappeared completely without a residual spinal cord lesion.- - - - - - - - - - ranking = 0.34142526866649keywords = formation (Clic here for more details about this article) |
8/152. Massive pericardial and pleural effusion with anasarca following allogeneic bone marrow transplantation.A 10-year-old girl presented with massive pericardial/pleural effusion with anasarca 216 days after an allogeneic bone marrow transplantation from her HLA-matched sibling for relapsed acute lymphoblastic leukemia. She did not show any other symptoms of chronic graft-versus-host disease (GVHD). The antinucleolar antibody was elevated in the blood and the pleural fluid. The lymphocytes in the fluid were mostly CD8 /HLA-DR , and a majority of CD8 cells in the blood expressed CD57. These data suggested that she had chronic GVHD. Immunosuppressive therapy including prednisolone, cyclosporin A, high-dose methylprednisolone, tacrolimus (FK506), and methotrexate had no effect, and the patient died of aspergillus pneumonia 183 days after the presentation of the disease. Although it has not been described before, isolated serositis with edema should be recognized as a clinical feature of chronic GVHD.- - - - - - - - - - ranking = 1.8733802606333keywords = antibody (Clic here for more details about this article) |
9/152. When a mastoid swelling is not mastoiditis.A case is reported of swelling over the mastoid process due to subgaleal abscess possibly secondary to trivial cutaneous trauma. The diagnosis was difficult as subgaleal abscess is an extremely rare condition especially after the advent of the antibiotic era. The route of entry of the infection to the subgaleal space was unclear as there was no skin puncture. The absence of substantial trauma excluded subgaleal haematoma as a precondition. We would like to discuss the possible aetiologies and the management of this rare case in the light of the limited information available in the world literature.- - - - - - - - - - ranking = 0.34142526866649keywords = formation (Clic here for more details about this article) |
10/152. Hydrops of placental stem villi complicated with fetal congenital adrenal hyperplasia.The authors present a case report of hydrops of placental stem villi. Numerous small aechoic spaces were demonstrated by prenatal ultrasonography. The patient spontaneously delivered a female newborn at 26 weeks' gestation. The infant showed hypertrophied clitoris and urogenital sius, and had a normal 46, XX karyotype. Endocrinological examination revealed that 3beta-hydroxysteroid dehydrogenase deficiency caused the anomaly. To our knowledge, this is the first report that congenital genital malformation complicated the placental mesenchymal dysplasia.- - - - - - - - - - ranking = 0.34142526866649keywords = formation (Clic here for more details about this article) |
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