Effects of intravenous cyclophosphamide in severe proliferative lupus glomerulonephritis.
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Biomedical subjects
Publications and source records attributed to M T Vidal.
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The pattern and extent of disorganization of the liver architecture were studied in 25 children undergoing orthotopic liver transplantation for cirrhosis. Image analysis techniques based on mathematical morphology were used to define seven parameters for each case, including fibrosis index (percentage of Sirius-red-stained areas), three categories of regenerative nodules (< 0.8, 0.8-1.6 and > 1.6 mm in diameter) and three categories of fibrous septa (< 0.4, 0.4-1.2 and > 1.2 mm in width). Fibrosis index ranged from 10.2% to 51.9%. Percentage of small nodules of infralobular size (< 0.8 mm in diameter) varied from 31.4% to 98.2%. Percentage of large nodules, > 1.6 mm in diameter, in only four cases was > 15%. Multivariate clustering analysis classified the cases into three main groups. One of them included only cases of cirrhosis secondary to parenchymal disease or to inborn errors of metabolism. In their pattern, tiny nodules predominated, and the percentage of slender septa, < 0.4 mm in diameter, was very high, although the overall fibrosis index was relatively low. Patients with biliary cirrhosis were classified into two groups. In one the fibrosis index was low and the size of the nodules variable, with 12% large nodules. In the other the fibrosis index was high, and small nodules, < 0.8 mm in diameter, predominated. No relationship was found with age at transplantation or previous portoenterostomy. A presumptive explanation for this divergent evolution might be the occurrence of cholangitic episodes. Overall fibrosis that the liver can sustain without failure is apparently limited.(ABSTRACT TRUNCATED AT 250 WORDS)
Remodeling of the cirrhotic liver was studied retrospectively by mathematical morphologic methods in 75 autopsy cases (40 alcoholic, 17 hepatitis B virus (HBV)-related and 18 cryptogenetic cirrhosis), including 28 hepatocellular carcinomas. The aim was to obtain objective measurements of cirrhotic patterns that could be correlated with liver function evaluated by the Pugh-Child score, establish the relationship among different morphogenetic features and evaluate the implications of an objective classification of cases by numerical taxonomy in terms of their etiology, liver function and malignant transformation. The results indicate that the Pugh-Child score was closely related to the global amount of fibrosis or to the percentage of regenerative nodules < 0.8 mm in diameter. In contrast, the higher the percentage of lobular-sized regenerative nodules (0.8-1.6 mm), the better the functional score, suggesting that they are probably residual lobules, albeit completely surrounded by fibrous tissue, rather than true regenerative pseudolobules. The four groups of cases obtained by numerical taxonomy (cluster analysis) showed different distributions for alcoholic and HBV-related cirrhosis. The pattern of the latter was practically analogous to that in classically labeled cryptogenetic cirrhosis, suggesting its viral etiology. Taxonomic classification had functional implications. The Pugh-Child score showed a definite relationship with the different clusters obtained. The incidence of malignant transformation gradually decreased from group G1 to G4, with a steeper descent between G2 and G3. These results might contribute to a more dynamic concept of morphologic changes in liver biopsies from patients with cirrhosis.
A prospective, open and uncontrolled study of effectiveness and side effects of intravenous 6-methylprednisolone megadoses was carried out in 20 patients with systemic vasculitis with rapidly progressive renal failure. The results showed a favorable response of renal function in 80% of patients and a control of pulmonary hemorrhage in the patients in which it was present. Oliguria and the need for hemodialysis during the acute phase of the disease significantly reduced the response to treatment. Extracapillary proliferation in more than 80% of glomeruli resulted in a lesser degree of recovery of renal function. Severe side effects developed in 2 patients (10%), but they could be exclusively attributed to the 6-methylprednisolone megadose in 1 (5%). It was concluded that 6-methylprednisolone megadoses may be an effective therapeutic alternative for the treatment of these patients.
In the present study, 7 patients with secondary amyloidosis and renal involvement who developed rapidly progressive deterioration of renal function are reported. After the investigation of the possibly implicated clinicopathological factors a cause was found for 3 patients. In the remaining 4 patients, the rapid evolution to end stage renal disease was attributable to the rapid progression of amyloid deposits by itself. A correlation between the evolution of renal function, vascular amyloid deposits and interstitial fibrosis is reported.
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Necrotizing angiitis or vasculitis exhibits a wide clinical spectrum characterized by many different cutaneous manifestations. Diagnosis must be confirmed by histopathology. We studied in retrospect 25 patients whose conditions had been diagnosed by skin biopsy. Histologic examination revealed infiltration by polynuclear cells and fibrinoid necrosis of the walls of the blood vessels in the skin. The great variety of clinical manifestations and etiologies stands out in a review of the records of these patients. Necrotizing angiitis has been found associated with mixed cryoglobulinemia; administration of drugs, milliary tuberculosis, bacterial meningitis, rickettsiosis, staphylococcal sepsis, pharyngotonsillitis, and rheumatoid arthritis. Necrotizing angiitis is a group of diseases with a great variety of clinical manifestations, ranging from benign to fatal. The various entities described to date have been more like different clinical forms of the same disease that distinct conditions. In cases of necrotizing angiitis caused by basically immunological mechanisms, the walls of the blood vessels may be impaired in varying diffuse degrees. The prognosis of the disease depends on the intensity of the inflammation and its repercussions on the parenchymas of different organs. The kidney is the most susceptible organ in this case. Treatment should be directed toward the avoidance of predisposing and etiologic factors, detection of the immunological reaction, requiring careful and individual attention in every case.
The case of a 50-year-old woman with a retroperitoneal paraganglioma and nephrotic syndrome is presented. After the tumor was removed deposits of amyloid material were observed in the paraganglioma, kidneys, and liver. There was no thrombosis of the renal vein. The clinical picture (fever and nephrotic syndrome) was similar to that of patients with amyloidosis associated with neoplasms. The nephrotic syndrome disappeared 8 months after the operation. The patient has not had fever or nephrotic manifestations over the past 4 years and is asymptomatic at the present time. We believe this to be the first reported case of paraganglioma associated with amyloidosis. Nor have we found any references to clinical and biological remission of the nephrotic syndrome following surgical removal of the concomitant neoplasm.
Finding a lymphangioma in the upper digestive tract is a rather infrequent event. A case of a 64-year-old man is reported. Diagnosis was made in the course of an endoscopic oesophagogastroduodenal examination and tumor excision with a diathermy snare at the same time. Two years after resection there was no sign of recidivation of the tumor.
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Two patients are presented with lymphadenopathy, hepatosplenomegaly and infiltration of the bone marrow with plasmocytoid cells in whom an important heterogeneous hyperimmunoglobulinemia was detected (3.18 and 5.85 g/100/ml). Autopsy showed widespread poorly differentiated lymphocytic lymphoma with plasmocytoid differentiation in the first case and a well differentiated lymphocytic lymphoma with plasmocytoid differentiation involving lymph nodes, marrow, spleen, liver and kidney in the second case. High concentrations of IgM and IgG were detected in lymph node and spleen homogenates from this case. After splenectomy and cyclophosphamide the immunoglobulins decreased. It is suggested that both cases had a malignant lymphoma with plasmocytoid differentiation and polyclonal gammopathy.
The authors report nine cases of chronic glomerulonephritis with positive hepatitis B antigen (AgHB) (8 cases) and positive anti-AgHb (1 case). Five patients had membranous glomerulonephritis. The sub-type was ad in 6 cases and ay in 2. In 3 members of two families of patients, AgHB was positive with the same sub-type as the subject studied. In 5 cases, liver biopsy showed signs of chronic hepatitis. According to the authors, a positive AgHB in glomerulonephritis is often associated with clinically latent chronic hepatitis.