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PubMedDate not supplied

[Ocular function in beta-thalassemia patients].

The eyes were examined in 118 inpatients with various thalassemia forms. The examinations included viso- and perimetry, biomicro- and ophthalmoscopy, fluorescent angiography of the fundus oculi and retinophotography. Patients with thalassemia were found to develop a variety of changes in the eye, whose manifestations depended on the length and severity of the underlying disease and on the treatment administered. Changes in the conjunctival and retinal vessels develop as early as in the initial stages of thalassemia, presenting as twisting, dilatation, and irregular calibre of the veins, dilatation of the arteries and reduction of their light reflex. Later dystrophic and atrophic changes develop: obliteration of the iris pattern and thinning of its peripheral zone, deterioration of blood circulation in the central retinal zone. Multiple blood transfusions and hemosiderosis lead to the development of hyperpigmentation of the limb, sclera, fundus oculi and appearance of retinal angioid strips. These findings should be taken into consideration when assessing the ocular status of thalassemia patients and defining the indications for treatment for angiopathic and dystrophic processes in the eye.

Adolescent↗
PubMed1992

[Blackfan-Diamond disease and malignancy: cause effect relationships?].

The authors report on a 24-year old patient with Blackfan-Diamond syndrome who developed a Hodgkin's disease. This patient became transfusion-dependent at the age of 10, after an initial period of corticosensitivity, and after failure of androgens. He developed hemochromatosis despite from parenteral chelation therapy. He died of infectious complications 4 months after the diagnosis of Hodgkin's lymphoma. A review of the literature shows an increased incidence of malignancies in Blackfan-Diamond syndrome (three cases of leukemia), and in similar disease (thalassemia and sickle cell disease), but not in other patients with hemosiderosis (primitive hemochromatosis, end-stage renal failure under dialysis). Etiopathogenic hypotheses are discussed.

Adult↗
PubMedDate not supplied

Morphophysiopathological correlations in pulmonary hypertension of cardiac origin.

100 patients with congenital malformation and acquired cardiac diseases were studied on preoperatory pulmonary biopsies to estimate the pulmonary hypertension by correlating the data obtained by modern multidisciplinary investigation: histological, histoenzymological, ultrastructural, physiological and clinical techniques. The morphological pictures show a range of vascular lesions similar to those found in Heath and Edwards' classification and they are especially thickenings of the intima, hypertrophies of the media, narrowings of vascular lumen and later some plexiform lesions and hemosiderosis. Progressive fibrosis processes as well as immune pulmonary reactions were made evident. The electron microscopical examinations provided new data regarding the thickening of the capillary and alveolary basal lamina, the activity of pneumocytes and macrophages that were also certified from the histoenzymological point of view by increase of peroxidases and acid phosphatases activity. The investigation carried out proved the importance of the cardiac preoperatory catheterism in order to establish the grade of the pulmonary hypertension while the concordance index is more increased than in the cases that were investigated only by non-bleeding techniques.

Capillaries↗
PubMed1991

[Twenty-eight-day repeated dose toxicity test of p-phenetidine in F344 rats].

A twenty-eight-day repeated dose toxicity test of p-phenetidine was carried out in male and female F344 rats at dose levels of 160, 40, 10 or 0 mg/kg/day. Thirty animals of both sexes were divided into 6 groups of equal number. All groups were treated daily by i.g. administration for 28 days, two extra groups of animals at dose levels of 160 and 0 mg/kg being used for investigation of recovery over 14 days. Hematological and urinary examinations revealed decrease in erythrocytes and increased serum reticulocytes and urinary urobilinogen in the 160 and 40 mg/kg groups of both sexes, and methemoglobinemia occurred in the 160 mg/kg group. Increase in spleen weight was noted in the 160 and 40 mg/kg groups. On histopathological examination, hemosiderosis, increased extramedullary hemopoiesis, congestion of the spleen and myeloid hyperplasia of the bone marrow were observed in the 160 and 40 mg/kg groups of both sexes. Repair of these lesions occurred within 14 days after the cessation of administration. Based on these findings, a no-observed-effect level for p-phenetidine would be concluded 10 mg/kg/day.

Animals↗
PubMed1992

Congenital sideroblastic anemia successfully treated by allogeneic bone marrow transplantation.

Allogeneic bone marrow transplantation (BMT) was carried out on a 34-month-old boy with congenital sideroblastic anemia. The patient had been red blood cell transfusion dependent since the age of 7 weeks. He did not respond to therapy with pyridoxine and developed secondary progressive hemosiderosis. The preparatory regimen consisted of busulfan (3.5 mg/kg for 4 days) and cyclophosphamide (50 mg/kg for 4 days). Full engraftment of donor bone marrow was achieved and effective hemopoiesis is still maintained 3 years after BMT.

Anemia, Sideroblastic↗
PubMedDate not supplied

[Liver pathology in patients with primary hemochromatosis--homozygous carriers of C282Y mutation ].

Liver pathology was studied in 3 patients with primary chemochromatosis. In two cases so-called iron free foci with signs of hepatocytes with feature of dysplasia were found. Many siderosomes were found ultrastructurally in the cytoplasma of hepatocytes. Histological markers of virus infection were absent in a patient with positive serum HbsAg and HCV-Ab. Alcohol did not produce typical histological changes. In this case grave liver reticuloendothelial hemosiderosis typical for secondary hemochromatosis and overloading with iron of spleen pulp according to MR imaging were observed.

Genetic Markers↗
PubMed2003

[Causes and diagnostic procedure of diffuse lung disease in 28 children].

OBJECTIVE: Diffuse lung disease comprises a large, heterogeneous group of pulmonary interstitial and parenchymal disease. It is therefore difficult to some extent to make etiologic diagnosis. Little information on clinical spectrum and diagnostic evaluation of pediatric diffuse lung disease is available in our country. The purpose of this study was to explore the causes of and diagnostic approach to diffuse lung disease in children. METHODS: Twenty-eight children with diffuse lung disease aged 2 months to 14 years were studied retrospectively. Their history, physical examination, radiographic findings, final diagnosis and diagnostic processes were reviewed. RESULTS: Confirmed diagnosis was established in 25 cases and suggestive diagnosis in 3 cases. Confirmed diagnoses included: mycoplasma pneumonia in 1 case, Chlamydia trachomatis pneumonia in 2 cases, Epstein-Barr virus pneumonia in 1, CMV pneumonia in 2, hematogenous disseminated pulmonary tuberculosis in 3, pulmonary cryptococcosis in 1, invasive pulmonary aspergillosis in 2, Staphylococcus aureus sepsis in 1, diffuse bronchiectasis in 2, idiopathic pulmonary hemosiderosis in 1, idiopathic pulmonary fibrosis in 1, extrinsic allergic alveolitis in 1, HIV-related lymphocytic interstitial pneumonitis in 1, Wegner's granulomatosis in 1, Langerhan's cell histiocytosis in 2, and lymphoma in 3. Suggestive diagnoses included Nocardia pneumonia in 1, Pneumocystis carinii pneumonia in 1, and juvenile rheumatoid arthritis-associated pulmonary fibrosis in 1. The diagnostic directions of 26 patients were conducted by radiographic features. In 17 of 26 cases, the diagnostic range was confined by history. The diagnosis of 14 cases was made by noninvasive tests including antibody detection, bacterial culture, those of 8 cases by examination of biopsy material, and those of 2 cases by autopsy. CONCLUSIONS: The causes of pediatric diffuse lung disease included pulmonary infectious disease, idiopathic pulmonary disease and pulmonary lesion associated with systemic diseases. The diagnosis may be made by radiography, history, physical examination, noninvasive tests in most cases, while in some cases invasive procedures were necessary.

Adolescent↗
PubMed1992

[Changes in the organ of vision in beta-thalassemia: clinical and morphologic parallels].

Ophthalmologic examination and morphologic investigations of the spleen were conducted in 10 patients with beta-thalassemia. Pathologic changes of vascular and dystrophic character were detected in the organ of vision, symptoms of angiopathy, hemosiderosis and spleno-cirrhosis were recorded in the spleen tissue. Clinical changes in the organ of vision were correlated with morphological shifts in the spleen tissue. A conclusion has been made that ophthalmologic examination of thalassemia patients could be useful for evaluation of severity of the main disease and effectiveness of the treatment conducted.

Adolescent↗
PubMedDate not supplied

[Functional and morphological characterization of rat thyroid gland at remote periods following single high and low dose radiation exposure].

A study of the morphological structure and functional activity of the rat thyroid gland was carried out after 22 months following a single exposure to external radiation. The 3-month-old animals were irradiated with doses of 0.25, 0.5, 1.0, 2.0 and 5.0 Gy. Blood was assayed for thyroxin (T4) and triiodothyronine (T3) levels, while liver tissue--for NADP-MDH activity and thyroid tissue--for thyroperoxidase activity. The thyroid was studied histologically, morphometrically and by electron microscope. The decreased T4 concentrations 2.59-fold in the 5.0 Gy group, the increased T3/T4 in the 2.0 and 0.25 Gy groups, the reduced diameter of cellular nuclei and follicles, the flat follicular epithelium and diminished number of thyrocyte ultrastructures indicate thyroid hypofunction in the irradiated animals. The morphological changes are characterized by enhanced diffuse and focal sclerotic changes in thyroid, most pronounced at high irradiation doses (1.0-5.0 Gy), whereas the hemosiderosis foci suggest that the structural changes are consequences of radiation-induced destructive injuries in the gland parenchyma. Two of the thyroids (0.5 Gy) demonstrate foci with pronounced lymphoid infiltration, while follicular carcinomas were detected in 4 thyroids (2.0 Gy), and in one thyroid (0.5 Gy) in one thyroid (5.0 Gy). The remote effects of radiation were dose-dependent destructive, sclerotic and atrophic processes, decreased functional activity, stimulation of development of autoimmune aggression and carcinogenesis in thyroid.

Animals↗
PubMed2004

Lead poisoning in cattle grazing pasture contaminated by industrial waste.

Lead poisoning is described in a herd of 120 Nelore cows of which 35 were affected. All affected cows died after a clinical course of 2-7 d with clinical signs related to cortical neurological disturbances. The source of Pb was fumes from a car battery recycling plant which has had a failure in its filtering system. Lead concentrations in liver and kidneys of 2 cows, in soil; and in grass where the cows were held were respectively 39 to 431 ppm, 147 to 431 ppm, and 245 ppm. No significant gross changes were observed. Histopathology revealed of neuronal necrosis, vacuolation of the neuropil and hypertrophy of the vascular endothelium in the cerebral cortices, degeneration of the epithelial cells of renal proximal tubules, and hemosiderosis of kidney, spleen and liver.

Animal Husbandry↗
PubMed2004

Diagnostic yield of adenosine deaminase in bronchoalveolar lavage.

Adenosine deaminase (ADA) activity rises in various body fluids in patients with tuberculosis. A prospective study was conducted to determine the diagnostic value of ADA activity in bronchoalveolar lavage. Between March 2001 and February 2003, 148 patients were enrolled in our study, mean age 55.6 years (SD 14.6), and a male to female ratio of 2.4:1. The mean duration of symptoms was 66.2 days. All patients were either sputum-smear negative for AFB or failed to produce sputum. The final diagnosis resulted in three patient groups: 43 with pulmonary tuberculosis, 70 malignancy, and 35 miscellaneous causes. The mean ADA activity in the bronchoalveolar lavage for the pulmonary tuberculosis, malignancy, and miscellaneous causes groups was 8.98 (95% CI, 3.79-14.17), 7.63 (95% CI, 4.12-11.14), and 11.61 U/l (95% CI, 3.59-19.62), respectively. No difference was detected in the ADA level in the pulmonary tuberculosis vs other groups (p=0.56, one-way ANOVA). A high level of ADA activity was found in non-tuberculous conditions such as bronchogenic carcinoma, pulmonary hemosiderosis, chronic pneumonia with empyema thoracis and chronic myeloid leukemia. We concluded that ADA activity in the bronchoalveolar lavage was not clearly diagnostic of smear-negative pulmonary tuberculosis. Early diagnosis required histopathology of biopsied transbronchial specimens obtained by fiberoptic bronchoscopy.

Adenosine Deaminase↗
PubMed2004

Impact of hepatitis C virus infection on short-term outcomes in renal transplantation.

Hepatitis C virus is an RNA virus with 6 known genotypes. Prevalence of hepatitis C virus infection in the world is almost 3%. In patients undergoing hemodialysis, prevalence of hepatitis C virus positivity is reported to be from 1%-54% depending on the methods used for detection. Liver disease in kidney transplant recipients has been attributed to hepatitis B virus, hepatitis C virus, Epstein-Barr virus, cytomegalovirus, ethanol, hemosiderosis, and drugs such as azathioprine and cyclosporine A. Hepatitis C virus infection is currently the main cause of chronic liver disease in this group, and it may affect allograft outcome. Whether hepatitis C virus infection after renal transplantation adversely affects graft and patient survival remains controversial. Several series have reported no impact on short- and long-term patient and graft survival. In fact, comparative studies using different immunosuppressive protocols are not available. The differences in the results of these studies may be explained by confounding factors, for example, differences in immunosuppressive protocols, study design, methodology of diagnosing hepatitis C virus infection, and differences in hepatitis C virus genotypes. Treatment protocols for hepatitis-C-virus--associated liver disease should be considered before renal transplantation. Nevertheless, transplantation is the best option for patients with hepatitis C virus with end-stage renal disease, and less hepatotoxic immunosuppressive agents may decrease the incidence of posttransplant liver disease in patients with hepatitis C virus. This review will discuss the studies with specific emphasis on the impact of hepatitis C virus infection on short-term outcome in renal transplantation.

Acute Disease↗
PubMed2005

Hemosiderin pigmentation of tumour cells in cerebellar pilocytic astrocytoma associated with post-traumatic hemorrhage in adults.

The pilocytic astrocytoma is only rarely associated with gross intratumoral hemorrhage despite rich vasculature and blood vessel changes, accompanied often by perivascular depots of hemosiderin. We report an unusual case of pigmented cerebellar pilocytic astrocytoma presenting with posttraumatic hemorrhage in a 38-year-old man with no history related to the tumor. CT and MRI examination after head injury demonstrated unexpectedly the cystic lesion of 2 cm in diameter in the region of the right cerebellar hemisphere and vermis. The lesion was associated with hematoma and it was surgically removed 3 weeks after trauma. Histopathological examination revealed pilocytic astrocytoma tissue with broad hemorrhagic changes and with an unusual pattern of massive pigmentation of the cytoplasm of pilocytic astrocytes, consistent with hemosiderosis. Positive stains for iron and ferritin and ultrastructural study confirmed deposition of hemosiderin granules in the tumour cells. There was no evidence of melanin or melanosomes. This finding of hemosiderin accumulation in the cytoplasm of neoplastic astroglia seems to be analogous to post-hemorrhagic pigmentation of the normal Bergmann glia and subpial astrocytes. In the literature, the examples of neuroepithelial tumors with hemosiderin pigmentation of tumor cells have been rarely documented. To our knowledge, this is the first reported case of pigmented pilocytic astrocytoma exhibiting extensive intracellular hemosiderin deposition.

Adult↗
PubMedDate not supplied

Pathological study of lead poisoning in whooper swans (Cygnus cygnus) in Japan.

During spring 1989, thirty-three whooper swans (Cygnus cygnus) died at Lake Miyajima in Hokkaido, Japan; 15 were examined. The birds were diagnosed as having subacute lead poisoning due to ingestion of spent lead shot. The main gross findings were bile-stained liver, edematous or gelatinous bone marrow, bile-stained lining with hyperkeratosis and lead pellets in the gizzard, and proventricular impaction. Histopathologically, there was lead-hemolytic jaundice of the liver, hemosiderosis in the liver and spleen, and hypoplasia of the bone marrow with increased numbers of polychromatic erythroblasts. Acid-fast intranuclear inclusion bodies were seen in kidneys of seven swans. Under electron microscopy, inclusion bodies had frayed contours and consisted of high-electron-dense fine granules. The lead concentration of the liver ranged from 5.5 to 44.3 mg/kg wet weight. It was suggested that these changes resulted from excess breakdown of erythrocytes, inhibition of heme synthesis, and impaired erythropoiesis caused by lead shot.

Animals↗
PubMed2004

Angiodysplasia of cardia of stomach--a case report.

Angiodysplasia of gastrointestinal tract is still thought to be an entity of unknown aetiology. This lesion is most commonly observed in elderly patients presenting with severe and persistent iron deficiency anaemia, following occult blood loss or acute episodes of haematemesis. In the stomach antral vascular ectasia is the most common presentation. We report an autopsy case of vascular ectasia in the cardia of stomach in a young patient with clinical symptoms of anaemia as the presentation and an associated secondary hemosiderosis of liver.

Adult↗
PubMed2006

Myelodysplastic syndromes: iron overload consequences and current chelating therapies.

Chronic red blood cell transfusion support in patients with myelodysplastic syndromes (MDS) is often necessary but may cause hemosiderosis and its consequences. The pathophysiologic effects of iron overload relate to increased non-transferrin bound iron generating toxic oxygen free radicals. Studies in patients with MDS and thalassemia major have shown adverse clinical effects of chronic iron overload on cardiac function in patients who underwent polytransfusion. Iron chelation therapy in patients with thalassemia who were effectively chelated has prevented or partially reversed some of these consequences. A small group of patients with MDS who had undergone effective subcutaneous desferrioxamine (DFO) chelation for 1 to 4 years showed substantial hematologic improvements, including transfusion independence. However, because chronic lengthy subcutaneous infusions of DFO in elderly patients have logistic difficulties, this chelation therapy is generally instituted late in the clinical course. Two oral iron chelators, deferiprone (L1) and deferasirox (ICL670), provide potentially useful treatment for iron overload. This article reviews data indicating that both agents are relatively well tolerated, were at least as effective as DFO for decreasing iron burdens in comparative thalassemia trials, and (for deferiprone) were associated with improved cardiac outcomes. These outcomes could potentially alter the tissue siderosis-associated morbidity of patients with MDS, particularly those with pre-existing cardiac disease.

Administration, Oral↗
PubMed2004

Prevalence of diabetes mellitus and impaired glucose tolerance in beta-thalassemia patients with and without hepatitis C virus infection.

INTRODUCTION: Impaired glucose tolerance and diabetes are well known complications in beta-Thalassaemic multitransfused patients (beta-Th). Iron overload and chronic liver disease, viral infections and/or genetic factors may play an important role in the development of glucose intolerance. The present study aimed to investigate whether in beta-thalassemic patients the hepatitis C virus (HCV) infection may be an additional risk factor for the development of diabetes mellitus. PATIENTS AND METHODS: The study included 98 beta-Th multitransfused patients, 50 females and 48 males (mean age 15.9 -/+ 4 years; range: 8-32 years). Forty six (47%) patients were seropositive for HCV by ELISA. Six patients were diagnosed as diabetic before the present study. In the remaining oral glucose tolerance test was performed according to the recommendations of National Diabetes Data Group. Prevalence of diabetes and impaired glucose tolerance were compared in HCV-seropositive and HCV-seronegative groups. RESULTS: The prevalence of diabetes in HCV (+) adult beta-Th patients was higher compared to HCV (-) patients (15.2% vs. 1.9%, p: 0.02). No difference was observed in HCV-RNA (-) in the younger group of beta-Th patients. CONCLUSIONS: The prevalence of diabetes in adult thalassemic patients is significantly increased by HCV infection, but there is no difference between HCV(+) group and HCV(-) group in age range of 8-15 years. It is probable that the existence of hemosiderosis makes the effect of HCV infection on glucose metabolism clinically more evident.

Adolescent↗
PubMed2004

Hypoparathyroidism and heart failure in thalassemic patients: a case report.

Hypocalcemic cardiomyopathy due to primary or secondary hypoparathyroidism is usually refractory to conventional treatment for cardiac failure but responds favorably to the restoration of normocalcemia. We report on a thalassemic patient with severe cardiac failure due to hypocalcemia. Cardiac echocardiography showed impaired biventricular performance and laboratory analyses revealed hypoparathyroidism due to hemosiderosis. Concomitant treatment for heart failure and calcium supplementation was initiated. Thereafter, clinical and laboratory improvement was achieved. In the literature of thalassemic disorders, this is the first case of heart failure associated with hypoparathyroidism in which the patient recovered significantly after the correction of hypocalcemia, providing strong evidence to support our hypothesis of hypocalcemic myocardiopathy.

Adult↗