ABSTRACT
Up to 40% of patients with anti-glomerular basement membrane (GBM) disease, which is a rare autoimmune disorder usually manifesting as rapidly progressive glomerulonephritis (RPGN), are positive for circulating anti-neutrophil cytoplasmic antibody (ANCA). Many previous reports showed poor outcomes in these "double-positive" patients. We report a patient with perinuclear (p)-ANCA positive anti-GBM disease who presented with RPGN and required hemodialysis. Plasmapheresis and steroid and cyclophosphamide therapy were initiated following renal biopsy and resulted in normalization of anti-GBM antibody and p-ANCA titers, recovery of renal function, and discontinuation of hemodialysis. This case suggests that aggressive immunosuppression with plasmapheresis in patients who are p-ANCA positive with anti-GBM disease should be considered, even in those with severe renal dysfunction.
Subject(s)
Humans , Anti-Glomerular Basement Membrane Disease , Antibodies, Antineutrophil Cytoplasmic , Autoantibodies , Basement Membrane , Biopsy , Cyclophosphamide , Glomerulonephritis , Hemorrhage , Immunosuppression Therapy , Lung Diseases , Plasmapheresis , Renal DialysisABSTRACT
Heterotopic pancreas can be defined as the occurrence of pancreatic tissue at aberrant anatomic sites that lack vascular, neural and anatomic community with the pancreas. A minority of heterotopic pancreas elicit clinical signs or symptoms that can vary according to its location and size, and the involvement of the overlying mucosa. We report here on a case of heterotopic pancreas that was removed by pancreatoduodenectomy under the erroneous diagnosis of distal common bile duct cancer.
Subject(s)
Ampulla of Vater , Common Bile Duct , Diagnosis , Jaundice , Mucous Membrane , Pancreas , PancreaticoduodenectomyABSTRACT
Chronic active Epstein-Barr virus infection(CAEBV) is a nonfamilial syndrome that shows a specific immunodeficiency for the Epstein-Barr virus(EBV). CAEBV is characterized by fever, lymphadenopathy, splenomegaly, hepatitis, interstitial pneumonitis, interstitial nephritis, and uveitis. Cardiovascular complications are rare in EBV infection. Patients with CAEBV show characteristically high titers of anti-viral capsid antigen(VCA) IgG antibody and anti-early antigen(EA) antibody, as well as relatively low titer of anti-EB nuclear antigen(EBNA) antibody. We experienced a case of CAEBV with giant coronary aneurysms, who was a 6-year-old boy. He had 5 episodes of high fever and cervical lymphadenopathy and hepatosplenomegaly. The 6 mm sized bilateral coronary aneurysms were detected by echocardiography at second admission. IgG antibodies to EBV was positive, whereas negative for IgM antibody. Antibodies to EA and EBNA were also positive. The EBV was detected in lymph node tissue by in situ hybridization, and in the peripheral blood and bone marrow by the PCR. Treatment has been done with ganciclovir and interferon (IFN)-alpa for 5 weeks. The EBV-PCR of peripheral blood was converted to negative from 12th day of treatment. At present he has followed well for two years without fever and enlargements of lymphnode, except large coronary aneurysm.
Subject(s)
Child , Humans , Male , Antibodies , Bone Marrow , Capsid , Coronary Aneurysm , Echocardiography , Epstein-Barr Virus Infections , Fever , Ganciclovir , Hepatitis , Herpesvirus 4, Human , Immunoglobulin G , Immunoglobulin M , In Situ Hybridization , Interferons , Lung Diseases, Interstitial , Lymph Nodes , Lymphatic Diseases , Nephritis, Interstitial , Polymerase Chain Reaction , Splenomegaly , UveitisABSTRACT
Congenital lobar emphysema is a rare disease and a cause of respiratory distress in early infancy. The most common location of pulmonary involvement is the left upper lobe. We diagnosed a case of congenital lobar emphysema in a 5-month-old female infant, who had a complex heart disease using flexible bronchoscopy. In spite of palliative heart surgery, respiratory difficulty and cyanosis did not improve and weaning from artificial ventilation was not possible. Chest X-ray and CT scan showed persistent hyperinflation in the left upper lobe. So, we performed flexible bronchoscopy. The findings showed no cartilage and patent bronchus during inspiration, but complete collapse during expiration due to a check valve effect in the left upper lobar bronchus. After excision of the involved lobe, the patient improved from respiratory distress. The observed airway patency during inspiration, and dynamic airway collapse on expiration suggest that bronchomalacia contributed to lung overinflation in this case.
Subject(s)
Female , Humans , Infant , Bronchi , Bronchomalacia , Bronchoscopy , Cartilage , Cyanosis , Emphysema , Heart Diseases , Lung , Rare Diseases , Thoracic Surgery , Thorax , Tomography, X-Ray Computed , Ventilation , WeaningABSTRACT
We described a case of well differentiated papillary mesothelioma (WDPM) of the peritoneum in a 68-year-old man because of its rarity. It was incidentally found during operation for renal cell carcinoma. Grossly, the tumor appeared as multiple small granules or nodules on the entire peritoneal surface, including serosa of the intestine, omentum and mesentery. Microscopically, characteristic features are papillary, tubulopapillary or solid architecture lined by single layer of uniform, cytologically bland, cuboidal or polygonal mesothelial cells. The tumor cells were positive for EMA, cytokeratin and vimentin, but negative for CEA on immunohistochemical staining. Ultrastructural study showed characteristic long slender microvilli on the surface and intercellular junctions.