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Biomedical subjects

A Avagnina

Publications and source records attributed to A Avagnina.

At least 19 recordsLinked to original sources

Liver infiltrating mononuclear cells in children with type 1 autoimmune hepatitis.

OBJECTIVE: To investigate infiltrating cells in the liver of children with type 1 autoimmune hepatitis (AH-1). METHODS: liver biopsies from 24 untreated AH-1 patients (14 children, 10 adults), five patients with hepatitis C virus related chronic hepatitis (HCV), and 10 control liver specimens (CL) were processed for immunohistochemical cell characterisation. RESULTS: Two different cell distribution patterns were detected in the liver of patients with AH-1: (1) CD4(+) and CD20(+) cells were found in the central areas of the portal tracts (portal distribution); (2) CD8(+) cells were observed at the periphery of the portal space (periportal distribution). Some cell subsets, like CD56, CD57, Fas-L, and Bak, showed a non-defined distribution pattern. The presence of two well defined patterns of cell distribution was not observed in HCV and CL (CD4(+), CD20(+), and CD8(+) cells were uniformly distributed in the portal space). In AH-1 and CL, the NK markers CD56 and CD57 were found scattered throughout the liver parenchyma. However, in HCV biopsies, CD56(+) cells were also clearly increased in both the portal and the periportal areas. Biopsies of AH-1 and HCV patients showed a uniform distribution of Fas-L and Bak in the portal and periportal areas, with Bak staining also detected in the hepatic parenchyma. CONCLUSIONS: Despite clinical and genetic differences, there was a similar distribution of liver infiltrating mononuclear cells in children and adults with AH-1. These results raise the possibility of reclassifying cryptogenic chronic hepatitis by immunohistochemical analysis of infiltrating liver cells.

CD4-Positive T-Lymphocytes↗

[Ischemic colitis after renal transplantation:etiology and pathogenesis].

Ischemic colitis is a well-recognized complication occurring in renal transplant recipients. It has often been associated with cytomegalovirus (CMV) vasculitis. However, the diagnosis of this pathology in the absence of CMV suggests that other etiological factors might be involved. Drugs inducing mesenteric vasoconstriction, such as non-steroidal anti-inflamatory drugs (NSAIDs) and cyclosporine could be related to this entity.

Colitis, Ischemic↗

[Influence of diet on lipoprotein profile in familial combined hyperlipidemia affected children].

BACKGROUND: Familial Combined Hyperlipidemia is an inherited disorder affecting cholesterol and triglycerides metabolism, well known myocardial infarction risk factors. The FCHL clinical presentation is usually silent until the third decade although children can be affected, and the more recent opinion is that precocious diagnosis is mandatory in preventing complications. Aim of this study is to examine the effectiveness of the diet therapy (Step-One-Diet) in a group of 13 children affected by Familial Combined Hyperlipidemia. METHODS: The patients have been submitted to a normocaloric diet, 30% fat of the total caloric daily intake according with the Dietary Intervention Study in Children (Step-One-Diet). The patients then have been submitted to a two year-follow-up and lipoprotein levels (total cholesterol, LDL-cholesterol, triglycerides and apolipoprotein B), nutritional status (macro- and micro-nutrients) as well as anthropometric data (height, weight, BMI) have been monitored. RESULTS: Results showed a 10% total cholesterol and 30% triglycerides decrease, Iron and Calcium intake show increased levels approaching to the normal ones after controlled diet, while cholesterol intake was correct on both regimen. The growth parameters show a decrease in weight only in two obese and two overweight patients. CONCLUSIONS: The present study confirmed the effectiveness and safety of the Step-One-Diet in children patients, allowing triglycerides normalization in 60% of the patients, and a 10% cholesterol decrease, in agreement with the complex genetic inheritance of the disease.

Adolescent↗

Primitive neuroectodermal tumor of the lung.

Primitive neuroectodermal tumors occur most frequently in bone and soft tissue but have been reported in other locations. Primary lung primitive neuroectodermal tumors without pleural or chest wall involvement are extremely rare. We present a case with immunohistochemical and ultrastructural studies and follow-up of the patient. An 18-year-old man presented with hemoptysis. Chest radiographs revealed a right middle lobe mass, and bronchoscopy showed an endobronchial tumor. The lesion was resected by middle lobectomy. After 2 years, a local recurrence was treated by pneumonectomy. The patient died after surgery. Histologically, the tumor was composed of uniform cells with round nuclei and scanty cytoplasm arranged in cohesive lobules with occasional rosette formation. Immunohistochemically, the tumor was positive for vimentin, CD99, neuron-specific enolase, and neurofilaments. Ultrastructural study revealed neurosecretory granules and cytoplasmic processes. Our case shows the value of immunohistochemistry and electron microscopy in the diagnosis of primitive neuroectodermal tumors in unusual locations.

Adolescent↗

[Diagnosis and treatment of Helicobacter pylori infection. Its relationship with gastrointestinal ulcer and antimicrobial resistance].

Reliable data regarding the efficacy of different schemes of triple therapy for the eradication of Helicobacter pylori in our country, are not available. Patients with Helicobacter pylori infection and non-ulcer dyspepsia or active peptic ulcer disease were randomized in three different groups for therapy with, omeprazole 20 mg, clarithromycin 500 mg and amoxicillin 1000 mg, twice daily for one week (OCA 1, 40 patients) and the same treatment but for two weeks in a second group (OCA 2, 40 patients). The third group received omeprazole 20 mg, clarithromycin 500 mg and metronidazole 500 mg twice daily during one week (OCM, 40 patients). The primary efficacy end point was the eradication of Helicobacter pylori as confirmed by negative urea breath test, 4 weeks after the completion of treatment. Of 120 patients enrolled in the study, 113 met the entry criteria. Of them, 103 completed the treatment. When analyzed by intention to treat, after 4 weeks of finishing the treatment, Helicobacter pylori was eradicated in 92.3% of patients in OCA 1, 89.7% in OCA 2, and 82.8% in OCM. There was no significant difference between the three groups, regarding the eradication efficacy. Side effects were observed more frequently in OCA 2 and OCM groups. Primary resistance to amoxicillin and clarithromycin was not demonstrated, while 20% of cultured strains were resistant to metronidazole. In patients with peptic ulcer disease or non-ulcer dysplasia, triple therapy with omeprazole and two antibiotics is highly effective in the eradication of Helicobacter pylori. One week of OCA therapy is as effective as two weeks of OCA or one week of OCM, with less side effects.

Adult↗

[Fatal acute hepatic failure with hepatocarcinoma presentation in a patient with renal transplant with asymptomatic chronic B and C hepatitis].

Hepatocellular carcinoma is a primary tumor complicating liver disease, associated with cirrhosis in 80-90% of the cases. A kidney transplant recipient with chronic B and C viral hepatitis was admitted because of general malaise, renal function impairment and positive AST, ALT and alkaline phosphatase tests, and very high alpha-fetoprotein levels. Ascites, spontaneous bacterial peritonitis and renal failure developed. A CT showed multiple liver masses. Renal failure required hemodialysis. The patient died 17 days after the initial symptoms with hepatic encephalopathy. A postmortem liver biopsy confirmed the diagnosis of cirrhosis and hepatocellular carcinoma (HCC). This report, as well as a few others, shows the accelerated evolution of chronic viral hepatitis in kidney transplant patients and questions the convenience of kidney transplantation and the adequate follow up in chronic viral hepatitis.

Acute Disease↗

[Distribution of the genotypes of hepatitis C virus in intravenous drug addicts in Argentina].

Intravenous drug addiction (IVD) is an unfrequent risk factor in Argentina, representing less than 10% of patients (pts) with chronic HCV infection seen in our Unit. In order to study the genotypes (Gt) in IVD and compare them with a non drug addicted control population, 68 pts with a history of IVD were enrolled in this study and compared with 68 non drug addict (NDA) pts with chronic HCV, with similar age and gender distribution. In all pts a liver biopsy was performed. Genotyping was done by INNO LiPA (Innogenetics, Belgium). Mean age in both groups was 35 +/- 7.8 years and 50 were males. No difference was observed between both groups in the prevalence of Gt1a, Gt2a/c and in those with mixed infections. The prevalence of Gt1b in IVD was 19.1% and in NDA 38.2% (p = 0.0228). A highly significant difference was also observed in the prevalence of Gt3a, of 42.6% in IVD and only 11.8% in NDA (p = 0.0001). Gt1a was the second most frequent genotype in IVD pts (26.5%). Simultaneous HIV infection was present in 8 IVD pts (11.8%) and in none of NDA group. Liver biopsies showed a higher prevalence of mild chronic hepatitis in NDA (57.3%) than in IVD (32.4%) (p = 0.0058). Severe chronic hepatitis with advanced fibrosis or cirrhosis was more frequent in the Gt3 of the group with IVD when compared with Gt3 of the NDA group. It can be concluded that in accordance with other geographical areas, Gt3a is far more prevalent in intravenous drugs addicts than in the general population in Argentina where Gt1b is more frequent. Mild forms of chronic hepatitis are less frequent in IVD. In spite of the relatively small group with HCV co-infection with HIV, it seems important to note that 2/8 (25%) showed severe hepatitis C or cirrhosis.

Adult↗

[Great vessel and temporal artery involvement in temporal arteritis].

The importance of this case report resides in the unusual initiation of an arterial inflammatory disease. The patient presented ischemic symptoms and signs with bilateral upper limb claudication and Raynaud's phenomenon, with ulcerations on her left hand. We describe the patient's clinical history, diagnosis and therapy.

Aged↗

[Primary ureteral amyloidosis].

OBJECTIVE: Two additional cases of primary ureteral amyloidosis are described. The current literature and clinical classification of the disease are reviewed. METHODS: Over a period of 18 months, we observed two cases of organic ureteral stenosis whose etiology was difficult to determine. The first case warranted a nephroureterectomy due to the condition of the compromised urinary tract. Analysis of the surgical specimen showed histological evidence of ureteral amyloidosis. In the second case, the previous experience allowed a preoperative diagnosis to be made, an exceptional situation that permitted conservative surgery. Characterization of the amyloid proteins was done in both cases with immunochemical study by Western Blot. RESULTS: Two patients with primary ureteral amyloidosis were surgically treated. The first case underwent complete excision of the urinary tract. The second case, who had a single kidney, was treated by conservative surgery. In both cases we identified a fragment of the lambda light chain of immunoglobulin by Western Blot characterization as previously reported in localized amyloidosis. CONCLUSIONS: Although ureteral amyloidosis is an infrequent disease, it should be seriously considered in the differential diagnosis of organic ureteral stenosis in order to avoid unnecessary excision. To our knowledge, these are the first cases of ureteral amyloidosis with immunochemical characterization of the amyloid protein.

Aged↗

Ketoconazole-induced liver damage.

Five cases (four females, one male) of ketoconazole-related liver damage are presented, two of whom died. All patients received ketoconazole (400 mg/day) for various mycoses. In the four women the first signs of hepatotoxicity appeared after four weeks of therapy. One fatal case developed massive necrosis with fulminant liver failure and the other, submassive necrosis. In four cases cholestasis was a prominent finding. Biochemical evidence of biliary stasis may persist for several months, as occurred in the three surviving patients of our series. The two fatal cases continued receiving the drug in spite of its adverse effects. Consequently, repeated evaluation is recommended to detect early signs of liver environment.

Adult↗

[Follicular dendritic cell sarcoma in a lymph node].

We report the case of a 39 years old man who presented with a rapidly enlarging, painless mass in his left side of the neck. The mass was excised and turned out to be a lymph node. Histologically, it showed a proliferation of oval to spindle cells growing in sheets and fascicles, occasionally forming a storiform pattern. Tumor cells showed large nuclei, dispersed chromatin and small evident nucleoli. Neoplastic cells were positive for immunohistochemical markers vimentin, CD 68, CD 21, CD 35 and R 4/23. Ultrastructural examination showed long cytoplasmatic processes joined by desmosomes. The patient was treated with local radiotherapy and is alive and well 18 months after the excision. Follicular dendritic cell sarcoma is a very unusual tumor that merits wider recognition.

Adult↗

[Cholestasis associated with ticlopidine].

The case of a 56-years-old male with cholestasis associated with ticlopidine is presented. Cholestasis is an infrequent adverse effect of this drug. The patient was admitted to hospital because of jaundice, choluria, and itching of one month of evolution. The patient had taken ticlopidine twice a day for 3 months up to one week prior to admission for peripheral arteriopathy. Biopsy was performed showing acinar cholestasis and portal inflammatory infiltrate compatible with cholestasis due to hypersensitivity. Ticlopidine was discontinued by the patient himself one week prior to admission. The drug was not readministered and the evolution of the clinical and biochemical parameters of cholestasis decreased. The patient was asymptomatic and laboratory data were normal 4 months later.

Biopsy↗

[Non alcoholic steatohepatitis].

A prospective study of 21 patients with the diagnosis of non-alcoholic steatohepatitis (NASH) was carried out. All patients had hepatomegaly and in 10 (48%) image studies were consistent with steatosis and/or fibrosis. Biochemically, there was increase of AST, ALT and cholesterol in 48%, of GGT in 52% and of alkaline phosphatase in 38%. 18 patients were obese, 2 of them diabetic, 2 others had a history of exposure to drugs (amiodarone and isopropilic alcohol) and the last one presented hypothyroidism. Liver biopsies were studied using a semiquantitative scale to evaluate the degree of steatosis, inflammation and fibrosis in a scale from 1 to 3. Results showed a medium score of 2.6 for steatosis, 1.5 for inflammation and 1.8 for fibrosis. Four patients had cirrhosis and Mallory bodies were found in 11 cases (52%). NASH is an oligosymptomatic disease that can be found in different clinical conditions, mainly obesity, and is more frequent in women. It is histologically indistinguishable from alcoholic steatohepatitis. It is frequently underdiagnosed clinically and must be taken into account as a possible cause of cryptogenetic cirrhosis.

Adult↗

[Cronkhite-Canada syndrome. Report of a case].

The case of a 71 year old woman who developed a Cronkhite-Canada syndrome is presented, with generalized gastrointestinal polyposis, skin hyperpigmentation, onychodystrophia and alopecia. She had severe diarrhea with hypoalbuminemia and unusual dermal and articular changes. The patient had a partial remission following important protein diet therapy supplemented with vitamins and antibiotics for the intestinal bacterial over-growth. This is the first report of this entity in Argentina.

Aged↗

[Undifferentiated carcinoma of the nose and paranasal sinuses. A clinicopathologic and immunohistochemical study].

A clinico-pathological and immunohistochemical study of 22 cases of undifferentiated carcinoma (UC) of the nose and paranasal sinuses is presented. There were 6 nasal and 16 sinusal lesions. Eleven were small cell, 7 of the intermediate type and 4 of the large cell type. All the large cell UC originated in the sinuses. Immunohistochemically all but three of the UC (all small cell) were positive for an epithelial marker. EMA was better in this study than cytokeratin and CEA (19, 14 and 8 positive cases). Neuroendocrine markers were positive in 5 cases of the large cell UC (Table I). Of the 18 cases with follow up, 3 patients died of their tumor, 9 were alive with disease and 5 were with no evidence of disease after an average of 36 months. Patients with UC of the intermediate variant and large cell type had a better prognosis than those of the small cell type (Table 2). Cases treated with surgery and radiation therapy with or without chemotherapy had a better prognosis than those treated with either one of those treatments alone. This study shows that many of the UC of the nose and sinuses have a neuroendocrine phenotype and although they are aggressive neoplasms they seem to be not as lethal as suggested in the literature.

Adult↗

[Cytologic changes in bronchoalveolar lavage in amiodarone treated patients].

The histologic evidence of amiodarone pulmonary toxicity is interstitial pneumonia with foamy alveolar macrophages, which ultrastructurally show lamellar inclusion bodies due to lipid storage. Bronchoalveolar lavage (BAL) fluid findings include foamy macrophages, considered characteristic, and, in certain patients, differential cell counts suggestive of active alveolitis, giving rise to an immunologic explanation for its origin. The present study was undertaken in order to investigate the findings in BAL fluid in nontoxic patients taking amiodarone and to evaluate their clinical relevance. Eleven patients taking amiodarone chlorhydrate for severe ventricular arrhythmias (345 +/- 129 mg/day during 46 +/- 31 months and an accumulated dose of 440 +/- 337 g) and without clinical or radiological evidences of pulmonary toxicity, were clinically evaluated and studied by BAL. As shown in Table 1, cough and pulmonary rales were common findings (64% and 36% respectively), chest X-Rays were normal or indicative of cardiac failure and arterial blood gases showed slight hypoxemia (PaO2 83 +/- 10). As these are usual findings in advanced cardiac diseases, the patients were considered as having no amiodarone toxicity. BAL was done and the fluid obtained was processed for cytologic study. In every patient foamy macrophages were seen with light microscopy and lamellar bodies were detected by electron microscopy. In 5/10 evaluable patients BAL fluid cell count disclosed an increase in lymphocytes, leukocytes or both, indicative of alveolitis. This group of patient had lower PaO2 and PaO2/PAO2 than "non alveolitic" patients (76 +/- 9 mmHg vs 89 +/- 5 mmHg and 0.72 +/- 0.1 vs 0.85 +/- 0.08 - p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

[Spindle cell carcinomas. Immunohistochemical analysis of 15 cases].

Fifteen spindle cell carcinomas, 9 from the larynx, 4 from skin, 1 from pharynx and 1 from the esophagus were studied with a variety of mesenchymal and epithelial markers: vimentin (VIM), smooth muscle actin (ACT), desmin (DESM), cytokeratin (AE1), polyclonal keratin (Q), and epithelial membrane antigen (EMA). Peroxidase-antiperoxidase and avidin-biotin complex techniques were used. Ten of the lesions were biphasic and the remaining 5 monophasic, showing only the spindle cell component. The spindle cell areas were positive for ACT (14/15), VIM (13/15), DESM (10/15), Q (10/15), AEi (14/15) and EMA (3/15). There were 12 cases in which the spindle cell component was stained with epithelial and mesenchymal markers. Areas of squamous cell carcinoma were positive with the epithelial markers and unstained with the mesenchymal markers. These results support the concept of a bidirectional differentiation in the pathogenesis of these lesions with a "mesenchymal" metaplasia of malignant epithelial cells.

Biomarkers, Tumor↗