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At least 1,261 records · Page 70Linked to original sources
PubMed2005

Liver involvement in sickle cell disease.

BACKGROUND/AIMS: Liver involvement in sickle cell disease may take place due to the primary disease itself or to secondary conditions such as iron overload, viral hepatitis and cholelithiasis. In the present study we have tried to evaluate the frequency of hepatic dysfunction and etiological factors in 48 patients with sickle cell disease. METHODS: Clinical and laboratory investigation including liver function tests, serological tests for viral hepatitis, and abdominal ultrasonography were performed in all of the patients. Additionally, liver biopsies were taken from 13 patients. RESULTS: Intrasinusoidal sickling and Kupffer cell hyperplasia were consistently seen in all of the biopsy specimens. Hepatomegaly was present in all patients, whereas liver function test abnormalities were seen in 27%. The prevalence of cholelithiasis was found as 35%. Serological tests demonstrated the presence of hepatitis B surface antigen in three, antibody to hepatitis B virus in 19 and antibody to hepatitis C virus in four of the patients. The most significant contributory finding was the presence of hemosiderosis in histological examination of liver specimens. CONCLUSION: Our data suggest that chronic liver injury in patients with sickle cell disease seems to be a multifactorial phenomenon depending mostly on overlapping factors such as iron overload and viral damage rather than primary disease itself.

Adolescent↗
PubMed1991

[Candida arthritis: report of two cases].

Candida arthritis is extremely rare. However, there has been a steady increase in the incidence of candida arthritis during the last decade because of increasing candida infections in immunocompromised hosts, drug addicts, premature babies, and those who have received prosthetic joint operations or intraarticular injections of steroids. We report herein two cases of candida arthritis: a 27-year-old man with aplastic anemia and transfusional hemosiderosis, and a 60-year-old man with osteoarthritis who received repeated intra-articular injections of steroids. Definite diagnoses were made after repeated smear examinations of synovial fluids. In the first case, candida arthritis occurred about 2 weeks after an antecedent candidemia. Although the arthritis relapsed once due to inadequate antifungal treatment, it was finally successfully treated with systemic amphotericin B, oral ketoconazole and surgical drainage of the synovial fluid. In the second case, it took several months to make the diagnosis. The patient was also treated with amphotericin B, ketoconazole, and surgery with a satisfactory outcome. We conclude that since candida arthritis is a slow, less destructive joint infection, it can almost always be resolved. Also, late orthopedic complications can be avoided by early diagnosis and prompt treatment, including aggressive antifungal therapy, adequate drainage and necessary surgical intervention.

Adult↗
PubMed2006

Randomized phase II trial of deferasirox (Exjade, ICL670), a once-daily, orally-administered iron chelator, in comparison to deferoxamine in thalassemia patients with transfusional iron overload.

BACKGROUND AND OBJECTIVES: Iron accumulation is an inevitable consequence of chronic blood transfusions and results in serious complications in the absence of chelation treatment to remove excess iron. Deferoxamine (Desferal, DFO) reduces morbidity and mortality although the administration schedule of slow, parenteral infusions several days each week limits compliance and negatively affects long-term outcome. Deferasirox (Exjade, ICL670) is an oral chelator with high iron-binding potency and selectivity. In a phase II study, the tolerability and efficacy of deferasirox were compared with those of DFO in 71 adults with transfusional hemosiderosis. DESIGN AND METHODS: Patients were randomized to receive once-daily deferasirox (10 or 20 mg/kg; n=24 in both groups) or DFO (40 mg/kg, 5 days/week; n=23) for 48 weeks. Results. Both treatments were well tolerated and no patient discontinued deferasirox due to drug-related adverse events. The reported frequency of transient, mild to moderate gastrointestinal disturbances was higher in the deferasirox group than in the DFO group, but these disturbances settled spontaneously without dose interruption in all patients. Decreases in liver iron concentration (LIC) were comparable in the deferasirox 20 mg/kg/day and DFO groups; baseline values of 8.5 and 7.9 mg Fe/g dw fell to 6.6 and 5.9 mg Fe/g dw, respectively, by week 48. Deferasirox showed a plasma elimination half-life of 8-16 hours, supporting its once-daily administration. INTERPRETATION AND CONCLUSIONS: Deferasirox at daily doses of 10 or 20 mg/kg was well tolerated and, at 20 mg/kg, showed similar efficacy to DFO 40 mg/kg in terms of decreases in LIC.

Adolescent↗
PubMed1991

Hemorrhagic pericardial effusion in beta-thalassemia major: report of a case.

Although sterile pericardial effusion occurs in about half of the patients with massive iron overload, hemorrhagic pericardial effusion is rarely seen in beta-thalassemia major patients. A 10-year-old girl with beta-thalassemia major who was diagnosed in her early infancy developed a massive hemorrhagic pericardial effusion. She was receiving blood transfusions every 4-6 weeks without chelating therapy with an average hemoglobin (Hb) level of 6-9 g/dL. Progressive hepatospenomegaly was noted during the course. She had complained of orthopnea with palpitation and bilateral leg edema before admission. After evaluation, a massive pericardial effusion was found and pericardiocentesis was performed twice, which revealed a bloody and uncoagulable effusion. Finally a pericardial window was performed to eliminate the bloody effusion. Negative etiological evaluations of blood and pericardial effusion were reported. Pathological examination of the pericardial biopsy revealed hemosiderosis with a few lymphocytic infiltrates. We report this case for its rarity and its necessity for urgent treatment.

Child↗
PubMedDate not supplied

[Reaction of various components of the erythrocyte system to periodic action of submaximal loads].

During repeated exposures of rats to hypergravity two stages of catabolic and adaptive developments in red blood were distinguished. The first stage that lasted about a week was characterized by decrease of resistance and hemolysis of red blood cells, hemosiderosis in the spleen, circulation hypoxia in the liver. The second stage was characterized by the predominance of adaptive processes, viz. increased activity of red blood cells, enhanced erythropoiesis in the spleen pulp, and appearance of highly active hepatocytes.

Acceleration↗
PubMed1991

[Diagnosis of granulomatous lung diseases].

The accumulated experience and the literature data allow for the separation of a group of granulomatous pulmonary diseases among pulmonary diseases of different etiology. Etiologically heterogeneous granulomatous diseases are united by the general signs: granuloma development, immunologic disturbances mainly within the cellular system and mediators as well as systemic vascular affection in the form of vasculitis. In our opinion, granulomatous diseases include disseminated tuberculosis, sarcoidosis of respiratory organs, exogenic and idiopathic fibrous alveolitis, Wegener's granulomatosis, histiocytosis X, primary hemosiderosis, Goodpasture's syndrome and some other rare diseases. Granulomatous diseases are diagnosed on the basis of the ++clinico-roentgenologic findings with an obligatory cytological and histological study of the bioptic specimen; immunologic diseases are diagnosed proceeding from the study of the immunologic status and detection of specific antibodies; of great significance for the diagnosis of pulmonary granuloma caused by infectious pathogens are microbiologic studies which provide for the detection of microorganisms and fungi. Study of the clinicoroentgenologic and laboratory data made it possible to distinguish a number of features typical for each disease and to unite them into diagnostic symptom complexes. Despite the different course of granulomatous pulmonary diseases they may end in recovery and granuloma resolution, development of lung fibrosis in a chronic course and in certain diseases in lung tissue destruction with cavity formation (tuberculosis, Wegener's granulomatosis). A fatal outcome may also ensue due to an acute or chronic course of the diseases.

Alveolitis, Extrinsic Allergic↗
PubMed1991

[Echocardiographic assessment of patients with sickle cell anemia].

One hundred and sixty four (164) patients were evaluated. Sixty (60) with Sickle cell disease (SSHg.) and ninety seven (97) with Trait (ASHg.); seventeen (17) were normal control group. The study confirmed that the incidence of cardiomyopathy in Trait (ASHg.) is greater than reported by other clinical investigations. Cardiac arrhythmia, atrial fibrillation, premature ventricular contractions, bundle branch blocks, and T and ST modifications with sub epicardial isquemia were most significant electrocardiographics changes. The possibility of myocardial infarction in SS patients with low or normal hemoglobin is significant. M-Mode and 2-D echo, demonstrated similar end diastolic volumes in AS and SS patients in which cardiomyopathy were diagnosticated. Patients with cardiac failure, treated with cardiotonics, diuretics and ACE were compensated most frequently. To prevent hemosiderosis, antioxydant (alfatocoferol and Ubiquinones) were used with satisfactory response.

Adolescent↗
PubMed1991

[Antioxidant activity of blood serum amd erythrocytes in patients with refractory anemia].

Iron metabolism was studied in patients with refractory anemia. It is shown that continuous transfusions of erythrocytes result in a sharp rise of iron reserves that plays an important role in the development of hemosiderosis. To prevent iron oxidative action it is recommended that the patients should be subjected to therapy with chelating agents and antioxidants simultaneously with transfusions of erythrocytes. A relationship has been noted between blood antioxidant capacity decrease and iron-overloading.

Aged↗
PubMedDate not supplied

[The endocrine complications in thalassemia major].

Based on a review of the literature and personal observation, the authors discuss the most frequent endocrine complication that occur in patients with thalassemia major given blood transfusion and chelating therapy. All the complication are attributed to iron overload in the endocrine glands. The authors conclude that an adequate chelating therapy protects against hemosiderosis and thus against endocrine pathologies.

Adolescent↗
PubMed1991

[Histopathologic study of cardiac myxoma. Apropos of 80 surgical cases].

The histological features of eighty cardiac myxomas removed by surgery between 1959 and 1988 were evaluated by the usual staining techniques and also by identification of immunohistological markers (vimentin, desmin, factor VIII, actin). Myxoma cells present various forms (polygonal, round, spindle, stellate) when isolated but are characterized by their tubular angioid structures: cellular masses, elementary tubes, organoid formations with several parietal layers and abundant "myxoid" matrix, denser around the angioid structures. Sometimes they are located on the surface forming crypts and vegetations, replaced in old myxomas by hyalinization. Other constituents such as vascular spaces, fibrinous exudate, hemosiderosis pigments, necrobiotic areas, various calcifications, plasma cell infiltrates are also frequently found. The insertion on the endocardium is usually an interesting structure. The presence of mucous epithelial structures and clusters of smooth muscle cells is uncommon but constitutes an additional argument in favour of the present explanation concerning its histogenosis i.e. a tumor derived from vestigial cells, usually confined to the floor of the fossa ovalis. On the basis of these various characteristics, myxomas can be classified as being active or inactive (more or less mummified), poorly differentiated or mature, which may correspond to the duration of the lesion prior to its resection. A precise histological examination of myxoma avoids confusion with more malignant myxomatous cardiac tumors.

Heart Neoplasms↗
PubMedDate not supplied

[Alcoholic liver disease in Venezuela. Clinical hepato-functional and histopathologic course].

24 patients with alcoholic intake were classified according to the amount of alcohol ingestion; clinical symptoms and signs, liver function tests (bilirubin, aminotransferases and prothrombin time) were analyzed. In all patients a percutaneous liver biopsy was performed and tissue stained by hematoxylin-eosin, wilder reticulin and Mallory trichromic. 9 Histologic criteria were analyzed. 4 groups according to the histology were identified. Group 1 (5 patients) hepatic fibrosis and/or fatty liver. Group 2 (5 patients) alcoholic hepatitis. Group 3 (10 patients) cirrhosis. Group 4 (4 patients) normal. 20% of patients with fatty liver, 80% of alcoholic hepatitis and 100% of cirrhotics referred ingestion or more than 160 g of ethanol and important correlation between liver histological damage and alcohol ingestion. Telangiectasia was the most common clinical finding and present in all hepatitis, 70% of cirrhotics and only 20% of fatty livers. Hemosiderosis was found in 60% of cirrhotics and in alcoholic hepatitis. Only 40% of patients with fatty liver and inflammatory cells while this was evident in all patients with alcoholic hepatitis and those with cirrhosis. Mallory bodies were identified in only 20% of cirrhotics and in none of the alcoholic hepatitis. The results suggest that there are significant differences from a histological and clinical point of view that distinguish alcoholic liver disease as seen in Venezuela from that reported in other countries.

Adult↗
PubMed1990

Hemoglobin H disease--ten years' experience.

Eighty-eight patients with Hemoglobin (Hb) H diagnosed in our hospital in the past decade were reviewed. Among them, 37 were males and 51 were females, and their age ranged from 10 to 77 years. In physical examination, 43.3% of them showed to have jaundice, 47% had splenomegaly, 14% had hepatomegaly and 19.3% had gall stone. Hb electrophoresis revealed the presence of Hb H (1.4-40%), Hb F (23/88, range: 0.5-7.5%) and decreased Hb A2 (mean = 1.23 +/- 1.2%). The study of iron status showed increased serum ferritin concentration (mean = 421.4 +/- 343.7 ug/dl) and transferrin saturation ratio (53.9% +/- 20.5%). Hemosiderosis was found in three out of four patients received liver biopsy. Although most patients did not receive blood transfusion frequently, iron overload was not uncommon in the patients with Hb H disease. Further study would be needed to explore the true relationship between iron overload and Hb H disease.

Adolescent↗
PubMed1990

[Correlation of CT and pathologic findings of pulmonary lymphangioleiomyomatosis].

The X-ray CT findings of two cases of pulmonary lymphangioleiomyomatosis were reported. The correlation between high-resolution CT findings and inflated biopsy specimens was studied. The X-ray CT findings are 1) multiple low attenuation areas, 2) diffuse areas of slightly increased density and 3) irregular enlargement of pulmonary vascular images. Each low attenuation are turned out to correspond to emphysematous lesions. Slightly increased densities on CT images seemed to be caused by a summation of many small nodules of a proliferation of smooth muscle cells located in the wall of respiratory bronchioles and alveolar ducts, with or without intraalveolar hemosiderosis. Some nodular lesions in bronchiolar walls were so close to neighboring vessels that they could not be separated from vascular images on CT, so peripheral vascular images were irregularly thickened. X-ray CT reflected more actual pathological findings than routine chest radiographs. As low attenuation areas on CT images have been reported to be representative of pulmonary emphysema, it is thought that the above CT findings must be differentiated from those of pulmonary emphysema. While pulmonary vascular images were thin and stretched on the CT in patients with emphysema, they were irregularly thickened on the CT of patients with LAM. Furthermore, while CT of emphysema often revealed overinflation or decreased density, diffuse areas of slightly increased density were never found in emphysema.

Adult↗
PubMedDate not supplied

[Computed tomography in the diagnosis of diffuse diseases of the liver].

Methods of CT, echotomography and scintigraphy of the liver were used to investigate 95 patients with diffuse liver diseases (fatty dystrophy, hepatitis, cirrhosis, hemosiderosis and amyloidosis). CT changes in these diseases were described. A high efficacy of CT was established in the detection of diffuse liver lesions and portal hypertension permitting one to give up in some cases invasive puncture biopsy of the organ.

Adult↗
PubMed1990

Experimental selenium poisoning in Nubian goats.

The toxicity of sodium selenite was studied in 28 Nubian goats, 20 of which died or were killed in extremis 2 h to 21 d after dosing. Single or repeated daily oral doses of 160, 80, 40, 20 and 5 mg sodium selenite/kg were toxic to goats while daily doses of selenite ranging from 0.25 to 1 mg/kg/d for 225 d were not toxic to this species of animals. The main signs of poisoning were uneasiness, inappetence, dyspnea, salivation, diarhea, paresis of the hind limbs, arching of the back, and recumbency. The main lesions were hemorrhages in the rumen, reticulum, osmasum and abomasum, hemorrhagic or catarrhal abomasitis and enteritis, fatty change and necrosis of the centrilobular hepatocytes and of the cells of the renal convoluted tubules, splenic hemosiderosis, pulmonary congestion, haemorrhage, edema and emphysema, accumulation of lymphocytes in the vital organs, and straw-colored fluid in the serous cavities.

Animals↗
PubMed1990

[Hemoglobin Shepherds Bush in a Sicilian family].

A case of Hb Shepherds Bush observed in a Sicilian family is reported. The iron status has been evaluated since chronic haemolysis may cause hemosiderosis. The paper also refers to the diagnostic workout for all chronic hemolytic anemias.

Adult↗
PubMedDate not supplied

[Physiopathology of iron metabolism and hemochromatosis: interrelationships, assessment and pathogenesis of iron overload, screening].

Fundamental aspects of iron metabolism relate to the dynamic processes of metal plasma transport as well as cell storage and efflux. Transferrin not only carries iron in the plasma but also delivers it to the various cells by binding to a diffuse specific cell receptor; it also acts by chelating cell iron. Ferritin co-operates by storing iron in the cell. By a still unknown regulatory mechanism, iron, from the ferritin pool, is redistributed in the cell to a cytosolic, easily chelatable, "transit" pool or to a degradative lysosomal hemosiderin pool from which it is slowly released outside the cell. Iron overload, such as that typical of hyperhemolysis or hemochromatosis, profoundly impairs its metabolism by saturating and/or altering transferrin and ferritin, by freeing iron from any regulated transport, thus allowing parenchymal deposition and damage. An important aspect still awaiting clarification relates to the different storage of excess iron in the parenchymal cells, as in hemochromatosis, or in the reticuloendothelial system such as in hemosiderosis. Studies using cellular models attempt to evaluate such differences in terms of altered properties of the iron proteins or their cell receptors, and of the different cell responsivity to non-transferrin iron. In the expectation of better knowledge, attention should be concentrated, from a clinical standpoint, on precise assessment of iron deposits in the tissues with the aim of preventing its excessive accumulation and parenchymal damage. In hemochromatosis, the risk of iron overload is evaluated by HLA typing.

HLA Antigens↗
PubMed1989

[An autopsy case of rectal cancer receiving cisplatin at total dose of 3.250 mg and platinum concentrations in autopsy tissue samples].

An autopsy case of a 57-year-old man who had received cisplatin, total dose of 3,250 mg (1,920 mg/m2) after proctectomy for advanced rectal cancer with hepatic metastasis is reported. Platinum concentrations in autopsy tissue samples are also reported. Cisplatin at a dose of 20-40 mg was administered intravenously once a week for 82 weeks, but renal failure did not occur. The patient died 529 days after his last cisplatin therapy, due to acute suppurative cholangitis. On autopsy, the hepatic hilar metastasis involved the bile duct, but the other metastasis of the liver became regeneration and necrosis, and the kidneys showed few findings but congestion and arteriosclerotic change. The spleen evidenced mild hemosiderosis. Tissue samples obtained at the time of autopsy were assayed for platinum using flameless atomic absorption spectrometry. The spleen had the highest concentration, about 2.7 times as high as that of the liver. The liver, the heart and the kidneys had high platinum concentrations (greater than 3.04 micrograms/g), but those of the jejunum and the ileum were low (less than 0.95 micrograms/g). Although cisplatin, FT 207 and krestin were effective for the rectal cancer, platinum was undetectable (less than 0.13 micrograms/g) in the ascending and the transverse colons.

Adenocarcinoma↗