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Disease Presenting (disease + presenting)
Selected AbstractsClinical Characteristics of Patients With Spontaneous or Inducible Ventricular Fibrillation Without Apparent Heart Disease Presenting with J Wave and ST Segment Elevation in Inferior LeadsJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 8 2000MASAHTKO TAKAGI M.D., Ph.D. Ventricular Fibrillation with J Wave in Inferior Leads. Introduction: The clinical characteristics of three patients with spontaneous or inducible ventricular fibrillation (VF) without apparent heart disease, who presented with J wave and ST segment elevation in inferior leads, are described. Methods and Results: All patients were male and experienced syncope. Their symptoms occurred at night or early in the morning. Holter ECG revealed infrequent premature ventricular complexes. Injection with disopyramide 2 mg/kg augmented ST segment elevation. Conclusion: These characteristics were very similar to those of patients with Brugada syndrome. These three patients with these specific features might have a variant of Brugada syndrome. [source] Infantile Crohn Disease Presenting with Diarrhea and Pyoderma GangrenosumPEDIATRIC DERMATOLOGY, Issue 1 2006James G. H. Dinulos M.D. We describe an infant with chronic diarrhea and failure to thrive who developed extensive ulcerations in the inguinal folds and perineum that were initially thought to be exclusively caused by local irritation. A cutaneous examination found signs consistent with those of pyoderma gangrenosum, leading to a diagnosis of infantile Crohn disease. Cutaneous signs can lead to the diagnosis of an underlying systemic disease in infants with chronic diarrhea and rash. Prompt diagnosis is especially important in infantile Crohn disease, since many infants require surgical resection of affected bowel, and 60% die from disease complications. This article reports a rare instance of an infant who developed pyoderma gangrenosum due to Crohn disease and reviews cutaneous signs of systemic disease in infants presenting with chronic diarrhea and rash. [source] Fatal Graft-Versus-Host Disease Presenting as Fever of Unknown Origin in a Pancreas-After-Kidney Transplant RecipientAMERICAN JOURNAL OF TRANSPLANTATION, Issue 4 2008F. L. Weng Acute graft-versus-host disease (GVHD) is a rare complication of pancreas transplantation. We describe a 54-year-old male with type 1 diabetes who received a zero-antigen mismatched pancreas-after-kidney transplant from a pancreas donor who was homozygous at the HLA-B, -Cw, -DR, and -DQ alleles. Starting on postoperative day (POD) #22, the patient developed persistent fevers. Workup was notable only for low-grade cytomegalovirus viremia, which was treated. The fevers eventually disappeared. On POD #106, the patient was noted to have a diffuse erythematous rash. A skin biopsy was consistent with GVHD. Short tandem repeat DNA analysis of both peripheral blood lymphocytes and skin demonstrated mixed chimerism, confirming the diagnosis of GHVD. Soon after diagnosis, the patient developed pancytopenia and fevers and died of multiorgan failure on POD #145. Transplant clinicians should consider GVHD as a possible, although admittedly rare, cause of fevers of unknown origin in recipients of pancreas transplants. [source] Use of the molecular adsorbents recirculating system as a treatment for acute decompensated wilson diseaseLIVER TRANSPLANTATION, Issue 10 2008Alexander Chiu Acute decompensated Wilson disease presenting as fulminant liver failure is a life-threatening condition for which liver transplantation is the ultimate treatment. It is listed as a status 1 indication according to the United Network for Organ Sharing classification. A massive amount of copper released during the attack induces hemolytic anemia and acute renal failure. Conventional chelating therapy attempting to remove copper from the patient is not satisfactory because there is inadequate time for these drugs to take action and patients are usually oliguric. The Molecular Adsorbents Recirculating System (MARS) is a form of modified dialysis that removes putative albumin-bound toxins associated with liver failure. It is believed that extracorporeal albumin dialysate absorbs the circulating copper molecules that are trapped in the patient's circulation. We report 2 patients with acute decompensated Wilson disease treated with MARS. In the first case, the patient was started on MARS once conventional treatment failed. A significant amount of copper was removed from her circulatory system, and her condition stabilized afterwards. The treatment gained her extra time, and she was eventually bridged to liver transplantation. In the second case, the patient was started on MARS treatment early in the course of his illness, and his condition soon stabilized after the treatment. He was able to return to his home country for liver transplantation. In both cases, MARS was used as a means of preventing deterioration rather than salvaging devastation. In conclusion, MARS may confer benefits to patients with acute decompensated Wilson disease if it is started early in the course of illness. Liver Transpl 14:1512,1516, 2008. © 2008 AASLD. [source] Sparing of the substantia nigra in sporadic Creutzfeldt-Jakob disease presenting as an acute corticobasal syndromeMOVEMENT DISORDERS, Issue 11 2007Wim Vandenberghe [source] Castleman's disease presenting within the cheek of a childORAL SURGERY, Issue 4 2008S. Katti Abstract A case is presented of an 11-year-old male with an eventual diagnosis of extranodal Castleman's disease (hyaline vascular type). This was difficult to diagnose as attempts were made to remove what was believed to be a haematoma, and then a reactive lymph node within the left cheek. Subsequent histopathology revealed the true nature of this swelling. [source] Rosai,Dorfman disease presenting as a solitary mediastinal massPATHOLOGY INTERNATIONAL, Issue 4 2009Akira I. Hida Rosai,Dorfman disease (RDD) involving an extranodal site is a diagnostic challenge. Reported herein is the case of a 67-year-old man who presented with a solitary superior mediastinal mass. The lesion was clinically suspected of malignancy including lymphoma because of its high uptake during a 67Ga-scintigram and 18F-fluorodeoxyglucose,positron emission tomography. There was no evidence of spread of the disease. Histology of thoracoscopic biopsy specimens indicated granulomatous lesion with infiltration of lymphocytes, plasma cells, and histiocytes with lymphocytes engulfed in their cytoplasm. The lesion did not contain lymph node or thymic elements. On immunohistochemistry the histiocytes were positive for S-100 protein, CD68, and CD163 but were negative for CD1a. These findings suggested a diagnosis of RDD. Despite lack of intervention, the lesion remained almost the same size for 3 years. To the best of the authors' knowledge this is the first case of RDD presenting as a solitary mediastinal mass. [source] Linear IgA disease presenting as prurigo nodularisBRITISH JOURNAL OF DERMATOLOGY, Issue 2 2006D. Torchia No abstract is available for this article. [source] A case of paraneoplastic pemphigus associated with Castleman's disease presenting as erosive lichen planusCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 8 2009H. L. Tey Summary Paraneoplastic pemphigus (PNP) is a debilitating chronic blistering mucocutaneous disease associated with an underlying neoplasm and a poor prognosis. We present a patient who had been diagnosed and treated for recalcitrant erosive mucocutaneous lichen planus for 3 years with little success. Further investigations established the diagnosis of PNP with underlying Castleman's disease. This case highlights the importance of repeated investigations to exclude PNP in patients with recalcitrant mucosal erosions, especially if atypical features are present. [source] | ||||||||||||||||||||||