Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P02794 (ferritin)
 17,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In an attempt to stimulate endogenous erythrocyte production and thereby provide an alternative to erythrocyte transfusions, we administered recombinant human erythropoietin (rHuEpo) in doses of 75 to 300 units/kg/wk to seven infants with the anemia of prematurity. Treatment was started between 21 and 33 days of life, maintained for 4 weeks, and was well tolerated. All the patients had low baseline serum erythropoietin levels. After rHuEpo therapy, the number of reticulocytes increased from a mean baseline count of 75 x 10(9)/L to 95, 141, and 165 x 10(9)/L on days 7, 10, and 14 of therapy, respectively. Correction or stabilization of the anemia was observed in six of seven patients, whose estimated total erythrocyte volume increased by 49% during therapy (vs a predicted increment of 18% in the absence of rHuEpo). In one patient, however, the hematocrit declined during the treatment, and in three of the responders a secondary fall in hematocrit was noted either during therapy or after its discontinuation. Serum iron and ferritin levels rapidly decreased after the initiation of rHuEpo therapy, and in most patients transient early thrombocytosis and late neutropenia were observed. These data suggest that rHuEpo may correct or stabilize the anemia of prematurity. Its effects, however, may be limited by a variety of factors, among which iron availability probably plays an important role. Controlled studies will be needed to confirm these preliminary observations.
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PMID:Effects of recombinant human erythropoietin in infants with the anemia of prematurity: a pilot study. 169 80

The iron status of 26 patients with essential thrombocythaemia (ET) was evaluated at diagnosis by means of bone marrow iron and blood studies, including serum ferritin determination. Nine patients were males, 17 females, and the mean age was 53 years (range 7-81). A decreased or absent iron level by semiquantitative estimation on bone marrow smears was observed in 77% of patients, and 81% had a low sideroblast score. Such a marrow pattern of iron depletion was equally distributed between both sexes. Contrasting with this, normal Hb, MCV, serum iron and serum ferritin were registered in the majority of cases. According to these results, absent or decreased marrow iron would be a common feature in ET, generally not reflecting true iron deficiency, as it occurs in the remaining chronic myeloproliferative disorders. Thus, in patients in whom ET is suspected, the diagnostic criterion of ruling out iron deficiency would be better served by serum ferritin measurement than by bone marrow iron estimation.
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PMID:Iron stores in essential thrombocythaemia. A study of 26 patients. 273 9

We examined hematopoietic disorders in 74 patients with various malignancies. The proportions of patients with anemia in the case of esophageal carcinoma, gastric carcinoma, colorectal carcinoma, hepatoma, and pancreatic carcinoma were 75%, 87.5%, 77%, 64% and 87.5%, respectively. Hypochromic microcytic anemia was mainly observed in showed patients with carcinoma of the gastro-intestinal tract which showed severe bleeding. The major proportion of patients with hepatoma and pancreatic carcinoma showed normochromic normocytic anemia. The WBC count was usually within the normal range except for patients with liver cirrhosis or generalized metastasis who showed decreased WBC counts. The mean lymphocyte count in the peripheral blood, which is thought to be correlated with the prognosis of cancer patients, was less than 1,500/microliter except for patients with gastric carcinoma, whose five-year survival rate was 28.6%. Monocytosis was mainly observed in patients with colorectal carcinoma and pancreatic carcinoma, accounting for 24.5% of the total cases. This finding may suggest some relationship between cancer and monocytes. Thrombocytosis was seen in patients with severe bleeding, but thrombocytopenia was seen in patients with liver cirrhosis. Most cancer patients showed normo-cellular marrow and normal M/E. We also examined the ferrokinetics and ferritin levels in cancer patients.
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PMID:[Hematological disorders in malignancy]. 301 96

We report on the progress of a new modified method of phlebotomy, erythrocytapheresis, as a means of fast therapeutic removal of erythrocytes from the circulation. We performed erythrocytapheresis in 65 patients. In 18 patients with central venous thrombosis of the eye, the beneficial effect of this procedure proved superior to the traditional approach. In 29 patients with primary or secondary polycythemia, hemoglobin, hematocrit, and blood viscosity could be lowered drastically for up to 11 months by a single erythrocytapheresis. We performed erythrocytapheresis in an effort to deplete the iron stores in patients with hemochromatosis (14 cases) and in patients with porphyria cutanea tarda (4 cases). Several consecutive erythrocytaphereses were necessary, however, to even slightly lower the amounts of stored iron as measured by serum iron, iron-binding capacity, and serum ferritin in these patients. The intervals between treatment were 2 to 11 months, thus much longer than the intervals between blood-lettings. We did not observe any adverse side effects. There was no significant influence on the clotting system, and no reactive thrombocytosis as described after phlebotomies.
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PMID:Erythrocytapheresis. A method for rapid extracorporeal elimination of erythrocytes. Results in 65 patients. 339 95

Coulter profiles with differential white cell counts, serum ferritin, and haptoglobin levels were determined in venous blood samples obtained from 90 males (M) and 25 females (F) immediately before and after completion of a competitive marathon (42.2 km) race. In an additional 20 male runners, the same measurements were performed serially during the 24 h following their completion of the race. In the pre-race samples from 90 M and 25 F, hypoferritinemia was present in 4/22 M and 1 F found to be mildly anemic. Neutropenia was detected in 4 M and 3 F and mild thrombocytopenia in 2 M. Haptoglobin levels were normal in all the female runners but reduced (less than 0.3 g/l) in 6 M. All post-race samples (88 M and 25 F) were characterized by a reactive neutrophilia and thrombocytosis including those with pre-race neutropenia or thrombocytopenia. An unexpected and incompletely explained sex difference in packed cell volume (PCV) response was observed. In males, the mean PCV increased from 0.425 +/- 0.021 to 0.444 +/- 0.028 (P less than 0.0001) whereas in females it decreased from 0.437 +/- 0.029 to 0.423 +/- 0.036 (P less than 0.05). In the post-race samples, anhaptoglobinemia was found in 13/88 M and 4/25 F. In the 20 male runners studied serially for 24 h after the race, the major changes involved a progressive increase in mean plasma volume (17.4% +/- 12.2% at 24 h) compared with the pre-race value, a progressive and significant increase in MCH and MCHC probably indicating a loss in red cell water and the gradual reversion of the reactive neutrophilia and thrombocytosis to basal levels.
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PMID:Hematological changes associated with marathon running. 355 78

The recombinant human erythropoietin (rHuEPO) by subcutaneous route has been considered the drug of choice for the correction of the anemia of chronic renal patients. PURPOSE--To evaluate the efficacy of a new preparation of rHuEPO in the correction of the anemia of chronic renal patients maintained by haemodialysis, exclusively administered by subcutaneous route, studying the adverse effects and searching for predictive factors for the response to this medication. METHODS--Twelve patients in regular haemodialysis were treated with freeze-dried rHuEPO by subcutaneous route during 18 months with initial doses of 20U/kg/dialysis. They were submitted to a careful clinical and laboratory monitoring for all this study. RESULTS--Eleven patients ended the study reaching the target hematocrit (Htc) of 30% and keeping it during the whole period of the study. The mean correction and maintenance doses of rHuEPO were 65U/kg/dialysis and 51U/kg/dialysis respectively. At the 12th week of the study a significative increase of Htc (18.4 +/- 3.5% vs. 25.4% +/- 3.8%, p < 0.05) was demonstrated. An increase of the erythrocytes and hemoglobin was concomitantly observed. Leucocytes and platelets increased significantly from the 24th week and kept steadily until the end of study. Just potassium increased in the biochemistry analysis of the patients at the 4th and the 12th week of the study returning to the basal values at the 24th week. The evolution of the iron metabolism parameters demonstrated an intermitent and statistically significant decrease of transferrin saturation at the 1st, 12th and 24th week, returning to the basal levels at the end of study. The serum ferritin did not change (582.7 +/- 700, 9ng/mL vs. 700.0 +/- 651, 6ng/nL). The weight and the blood pressure did not change either, although 2 normotensive patients became hypertensive and 2 others with controlled hypertension needed drug rearrange for blood pressure control (36%). A patient had a seizure episode with a full recovery. CONCLUSION--The rHuEPO has proved to be a safe and an efficient drug with easily controlled adverse effect.
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PMID:[Correction of anemia in chronic kidney failure with lyophilized recombinant human erythropoietin using a subcutaneous approach]. 782 Jan 45

Over a 9-year period, twelve patients (8 boys, 4 girls), from 3 to 14 years old, were diagnosed as having Still's disease. Intermittent spiking high fever, poly- or pauci- articular arthritis, and typical evanescent skin rash were the most prominent clinical features. Hemogram examinations showed that 36% of the patients had anemia, ninety-two percent had neutrophilic leukocytosis and 78% had thrombocytosis. Serologically, none had positive results of rheumatoid factor and anti-nuclear antibody. Serum ferritin level was obtained from six patients and all revealed marked elevation during active disease. C-reactive protein and erythrocyte sedimentation rate were both invariably elevated. Immunologically, elevated serum concentrations of IgG, IgA, and complements (C3, C4) were found in 33%, 20%, and 17%, respectively. Furthermore, eighty percent of patients showed an increased serum level of circulating immune complexes. Aspirin (ASA) was used in all patients, but 92% of them required non-steroid antiinflammatory drugs (NSAIDs) in combination to get a better response. Sixty-seven percent of patients needed corticosteroids to control the acute systemic manifestations. Other disease-modifying agents were also used in 33% of our patients. ASA-induced liver function impairment was found in two cases. In addition, one patient experienced an episode of upper gastrointestinal bleeding. Generally speaking, the overall prognosis was good. One patient (8%) died of internal bleeding after a needle liver biopsy.
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PMID:Still's disease: experience in 12 children. 823 59

Interleukin (IL)-6-associated laboratory parameters obtained at diagnosis on 17 children with histologically confirmed nodular sclerosing Hodgkin's disease (NSHD) are reported. When these patients were grouped as either symptomatic stage A or B, they were found to be similar in extent of disease, age, and gender. However, statistically significant differences between these two groups were observed for the means of the following IL-6-associated laboratory parameters: hematocrit (p = 0.019), platelet count (p = 0.009), serum albumin (p = 0.001), and ferritin (p = 0.037) concentrations. Moreover, trend analysis of abnormalcy revealed an increasing frequency of anemia, thrombocytosis, hypoalbuminemia, and hyperferritinemia between stage A and B patients and, when available, febrile controls (p values = 0.0012, 0.0009, 0.0406, and 0.0011, respectively). Correspondingly, IL-6 immunohistochemistry performed on archival material from representative cases in each group showed greater overall reactivity in specimens from stage B patients. A variety of cells accounted for this positivity for IL-6 antigen including Reed-Sternberg cells and their variants, lacunar cells, dendritic interdigitating cells, endothelial cells, fibroblasts, and vascular smooth muscle cells. In summary, greater and more frequent abnormalities in IL-6-associated laboratory parameters and increased immunohistochemical reactivity for IL-6 antigen coincide with the presence of fever in helping to identify children with clinical stage B NSHD.
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PMID:Interleukin-6-associated laboratory parameters and immunohistochemistry in symptomatic stage A and B nodular sclerosing Hodgkin's disease in children. 899 54

The purpose of the present study was to investigate the incidence of juvenile rheumatoid arthritis (JRA) among Japanese children and to evaluate the clinical features of this disease. A questionnaire was sent to the department of pediatrics of 1290 hospitals in Japan, in 1994, asking for the number of rheumatic patients during the past 10 years. Subsequently, a second questionnaire was sent asking for the type of onset, clinical features, treatment, and other details. The results of 570 cases were obtained. Of these, 310 cases (54%) were the systemic onset type, 140 cases were the polyarticular onset type (25%), and 120 cases (21%) were the pauciarticular onset type. Hence, in the present series of children, the proportion of the pauciarticular type was less than the other two types of JRA. In the laboratory findings of the systemic onset type, hyperferritinemia and thrombocytosis were noted, in addition to leukocytosis, positive C- reactive protein (CRP) and accentuated erythrocyte sedimentation rate (ESR). The rheumatoid factor was positive in 50% of patients with the polyarticular onset type. Chronic uveitis was recognized in 13 cases (10.8%) of the pauciarticular onset type. In four girls, uveitis started before the onset of arthritis. Non-steroidal anti-inflammatory drugs were used in almost one-third of cases, and methotrexate (MTX) was used in 12.8% of cases. The quality of life of children with JRA was disturbed in almost 20% of cases. Therefore, for the early and definitive diagnosis of the systemic type of JRA, diagnostic procedures including thrombocyte counts and serum ferritin level, should be performed. In order to obtain good results and to avoid side effects, a protocol for the use of disease modifying anti-rheumatic drugs and immunosuppressants, especially for the use of MTX, must be established.
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PMID:Clinical analysis of 570 cases with juvenile rheumatoid arthritis: results of a nationwide retrospective survey in Japan. 914 Dec 66

In higher eukaryotes, the expression of about 1 gene in 10 is strongly regulated at the level of messenger RNA (mRNA) translation into protein. Negative regulatory effects are often mediated by the 5'-untranslated region (5'-UTR) and rely on the fact that the 40S ribosomal subunit first binds to the cap structure at the 5'-end of mRNA and then scans for the first AUG codon. Self-complementary sequences can form stable stem-loop structures that interfere with the assembly of the preinitiation complex and/or ribosomal scanning. These stem loops can be further stabilized by the interaction with RNA-binding proteins, as in the case of ferritin. The presence of AUG codons located upstream of the physiological start site can inhibit translation by causing premature initiation and thereby preventing the ribosome from reaching the physiological start codon, as in the case of thrombopoietin (TPO). Recently, mutations that cause disease through increased or decreased efficiency of mRNA translation have been discovered, defining translational pathophysiology as a novel mechanism of human disease. Hereditary hyperferritinemia/cataract syndrome arises from various point mutations or deletions within a protein-binding sequence in the 5'-UTR of the L-ferritin mRNA. Each unique mutation confers a characteristic degree of hyperferritinemia and severity of cataract in affected individuals. Hereditary thrombocythemia (sometimes called familial essential thrombocythemia or familial thrombocytosis) can be caused by mutations in upstream AUG codons in the 5'-UTR of the TPO mRNA that normally function as translational repressors. Their inactivation leads to excessive production of TPO and elevated platelet counts. Finally, predisposition to melanoma may originate from mutations that create translational repressors in the 5'-UTR of the cyclin-dependent kinase inhibitor-2A gene.
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PMID:Translational pathophysiology: a novel molecular mechanism of human disease. 1082 6


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