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)

Of 567 children with neuroblastoma diagnosed between November 1984 and May 1993 in 21 Italian institutions, 235 (41%) have been evaluated for MYCN oncogene amplification. The amplification (3 or more copies of the gene) was found in 39 patients (17%) and was more frequent in patients aged more than one year, abdominal primary site of the tumor, advanced stages, normal urinary excretion of vanillylmandelic acid (VMA), and high level of LDH, NSE and ferritin. The five-year survival of the 235 patients (62%) was significantly better in patients with normal copy number of MYCN (69% versus 29%). By correlating genomic amplification with clinical and biochemical characteristics, MYCN amplification was found associated with a worse prognosis even when patients were subdivided for age (under and above one year), disease extension (localized operable, localized but inoperable, and disseminated) with exception for Stage IV-S, VMA and homovanillic acid excretion, serum levels of NSE and ferritin, but not of LDH. These data confirm the unfavourable prognostic meaning of MYCN amplification, but are unable to define if it represents a new independent variable.
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PMID:[The prognostic effect of amplification of the MYCN oncogene in neuroblastoma. The preliminary results of the Italian Cooperative Group for Neuroblastoma (GCINB)]. 797 42

HepG2 cell cultures and human hepatocyte primary cultures were used to develop appropriate hepatocytic in vitro models of iron load in order to further understand the pathophysiological mechanisms occurring in the liver of patients with hemochromatosis. The first step of this study was to obtain an efficient iron supply in conditions of minimal toxicity. It was demonstrated that iron complexed to citrate entered efficiently into HepG2 cells and human hepatocytes. This iron load was obtained with minimal toxicity in both culture models as evaluated by the intracellular LDH activity and the total protein content. The second step was to study the effect of iron on ferritin and transferrin receptor expression. In HepG2 cell cultures, intracellular and extracellular ferritin concentrations were strikingly increased by iron in dose- and time-dependent manners. However, the relative amounts of H and L ferritin mRNAs were not significantly affected by iron, suggesting that ferritin regulation occurred at a translational level. On the other hand, in human hepatocyte cultures, the increase of intracellular and extracellular ferritin concentrations was accompanied by an increase in the amounts of H and L ferritin mRNAs. In this model, iron-induced ferritin biosynthesis seemed to be more complex than in HepG2 cells and to be governed by transcriptional and/or post-transcriptional regulatory mechanisms. However, an additional translational level of regulation could not be excluded. In contrast, transferrin receptor expression was decreased by iron in HepG2 cells as well as in human hepatocyte cultures. This decrease was associated with a decrease in the mRNA steady-state level. In both culture models, transferrin receptor regulation seemed to occur at a transcriptional or post-transcriptional level. These results demonstrate that normal human hepatocytes in primary culture respond to iron in a manner close to that observed in vivo and thereby provide a promising experimental model for further understanding pathophysiological mechanisms involved in human hemochromatotic liver.
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PMID:Regulation of ferritin and transferrin receptor expression by iron in human hepatocyte cultures. 822 24

Management of variceal bleeding secondary to portal hypertension constitutes a challenging issue, particularly in child's C cirrhotic patients. Recently, transjugular placement of self-expanding metallic stents in the liver (TIPS), creating a shunt between the portal and hepatic branches has provided a safe and promising therapeutic approach in this clinical situation. We report here the case of a 66-year-old male cirrhotic patient who developed a moderately severe clinical picture of Coombsnegative hemolytic anemia (serum hemoglobin, 93 g/l, serum bilirubin 160.74 umol/L (9.4 mg/dl), indirect 6.3 mg/dl (107.73 umol/L); serum LDH 1220 mu/l, reticulocytes, 5.1%, serum ferritin 1221 micrograms/l, schistocytes in peripheral blood smear) the week after undergoing a TIPS, suggesting the development of microangiopathic hemolytic anaemia secondary to red blood cell disruption by passing through the metallic network of the stent.
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PMID:Transient hemolytic anemia after transjugular intrahepatic portosystemic stent shunt. 880 88

The present report describes the value of the plasma determination of TNF-alpha, at diagnosis, in 43 patients with non-Hodgkin's lymphoma (NHL), related to their clinical presentation and the new International Prognosis Index (IPI). We also compare the levels of TNF-alpha with those of LDH, beta-2-microglobulin (beta-2-m), and ferritin. At diagnosis, the mean values of the quotients between the marker values and the maximum value of normal (ratio:r-) are placed 7 times higher than normal for r-TNF-alpha, whereas those of r-beta-2-m and r-LDH are 2,4 and 1,4 times more respectively. We found a relationship between the value of r-TNF-alpha and the ECOG, Ann Arbor stage, the number of affected extranodal sites, and between the values of r-beta-2-m with r-LDH. The best correlation was obtained between the values of r-TNF-alpha and r-beta-2-m and IPI, however r-TNF-alpha best stratify the four risk groups in this prognosis index.
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PMID:Value of the determination of TNF-alpha in the plasma of patients with non-Hodgkins lymphoma. 883 7

We encountered seven children with hemophagocytic syndrome complicated with collagen diseases of whom five were JRA and two SLE, HPS was seen as one of initial symptoms in 6 cases excluding one case of JRA. Hematologically, cytopenia in 2 cell lines (RBC and platelets) was seen in most cases. Active hemophagocytosis by matured large histiocytes was observed in all cases. All patients had moderately to extremely high serum ferritin levels and LDH levels. Although both lives did not correlated with prognosis in HPS with collagen disease, the higher serum total cholesterol/serum triglyceride ratio, the worse was the prognosis. Those findings suggested that HPS in collagen diseases may be a different entity from other types of HPS.
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PMID:[Seven cases of hemophagocytic syndrome complicated with childhood collagen diseases]. 891 Oct 81

Serum soluble interleukin-6 receptor (sIL-6R) concentrations were measured in 50 patients with plasma cell dyscrasias using a commercially available immunoenzymatic assay kit. There were 40 patients with multiple myeloma (MM), 5 patients with monoclonal gammopathy of undetermined significance (MGUS), 3 patients with solitary plasmacytoma (SPC), 1 patient with chronic myelogenous leukaemia and multiple myeloma (CML/MM), and 1 patient with plasma cell leukaemia (PCL). We found that serum sIL-6R concentrations were higher in MM patients (62.53 +/- 38.85 ng/ml) than in 20 normal volunteers studied (36.75 +/- 13.79 ng/ml) (p < 0.01). The cut-off value of 65 ng/ml seen in 2 of our controls was arbitrarily taken as the upper limit of the control range for serum sIL-6R; according to this criterion, 14 patients with MM (35%), 1 patient with SPC, the unique patient with CML + MM, and the unique patient with PCL had elevated concentrations of the receptor. Patients with MGUS had normal sIL-6R values. In MM patients, serum sIL-6R levels correlated with the clinical phase of the disease: they were elevated in patients with early or late active disease and ranged within normal limits in patients with plateau-phase disease (p < 0.001). Thirteen of 27 patients with active MM had elevated serum sIL-6R values, i.e. 48.1%, but only 1 out of 13 patients with disease in the plateau phase, i.e. 7.7% (p < 0.05). Furthermore, in the entire group of MM patients, serum sIL-6R levels correlated with the concentrations of serum beta 2-microglobulin, (p < 0.02), CRP (p < 0.01), ferritin (p < 0.01) and LDH (p < 0.01), while they did not correlate with disease stage, haemoglobin levels, proportion of marrow myeloma cells, the values of serum IL-6, the levels of serum albumin, or the grade of bone lesions. We conclude that elevated serum sIL-6R levels should be related to the growth of myeloma cells and suggest that serum sIL-6R concentrations may be used as an indicator of disease activity.
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PMID:Serum levels of soluble IL-6 receptor in multiple myeloma as indicator of disease activity. 915 60

A 15-year-old girl was admitted to our hospital because of fever, diarrhea, and right lower abdominal pain on November 11, 1997. Computed tomographic and ultrasound studies of the abdomen disclosed enlarged mesenteric lymph nodes. Hematologic and serologic findings included WBC 3000/microliter, LDH 550 IU/l, IFN-gamma 264 pg/ml, IL-6 9.74 pg/ml, sIL-2R 781 U/ml, and ferritin 720 ng/ml. Although the patient was treated with antibiotics, high fever and abdominal pain persisted with progressive anemia and leucocytopenia (1800/microliter). Bone marrow aspiration specimens revealed an increase of histiocytes with phagocytosis. Appendectomy and lymphadenectomy were performed on November 21. A lymph node specimen showed necrosis with infiltration by large mononuclear cells. The resected appendix revealed reactive lymphoid hyperplasia. Based on these findings, a diagnosis of necrotizing lymphadenitis (NL) was made. The postoperative course was satisfactory and systemic symptoms resolved gradually without specific treatment. However, high fever and abdominal pain recurred with right cervical lymph node swelling on December 15. The patient's general condition improved after treatment with prednisolone. In NL, lymphadenopathy is usually observed in the cervical region, and the involvement of intra-abdominal lymph nodes is quite rare. Our findings indicated the possibility that IFN-gamma may play an important role in the pathogenesis of NL with hemophagocytic syndrome.
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PMID:[Necrotizing lymphadenitis presenting as mesenteric lymphadenopathy]. 1049 41

A 32-year-old woman in the 16th week of pregnancy was admitted to our hospital because of high fever. Laboratory findings disclosed pancytopenia and extremely elevated serum LDH and ferritin levels. Coagulation tests showed disseminated intravascular coagulation. Serum soluble interleukin-2 receptor, tumor necrosis factor-alpha, and interleukin-6 levels were high, but serum interferon-gamma was below the detectable limit. Reactive Epstein-Barr virus (EBV) infection was diagnosed on the basis of a high titer of IgG antibodies to the EBV capsid antigen and early antigen. EBV was demonstrated in the peripheral blood and bone marrow cells by polymerase chain reaction. Mature histiocytosis and hemophagocytosis were detected in the bone marrow. A diagnosis of EBV-associated hemophagocytic syndrome (EBV-AHS) was made. Neither prednisolone (PSL 30 mg/day, P.O.) nor methylprednisolone (m-PSL) pulse therapy (1,000 mg/day for 3 days) induced a response. Thereafter, treatment with m-PSL pulse therapy (1,000 mg/day for 3 days) and i.v. administrations of high-dose immunoglobulin (20 g/day for 3 days) in combination with acyclovir (750 mg/day) and gabexate mesilate (2 g/day) induced remission of the disease. Maintenance therapy consisted of PSL (5 mg/day, P.O.) and camostat mesilate (600 mg/day, P.O.). The patient delivered a healthy male infant in the 35th week of pregnancy via natural birth. Reports of pregnant women with EBV-AHS are rare, and the choice of therapy has not yet been established. The present case study suggested the above combination treatment is useful and safe, and capable of changing the fulminant course of EBV-AHS during pregnancy without the use of anticancer drugs.
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PMID:[Epstein-Barr virus-associated hemophagocytic syndrome during mid-term pregnancy successfully treated with combined methylprednisolone and intravenous immunoglobulin]. 1065 79

We report 2 cases of orbital non-Hodgkin's lymphoma (NHL) with hemophagocytic syndrome (HPS). Patient 1 was a 64-year-old man with a diagnosis of peripheral T-cell lymphoma originating in the right orbita (clinical stage: IV B). Epstein-Barr virus DNA was demonstrated in tissue specimens by polymerase chain reaction. Laboratory findings on admission were WBC: 4,700/microliter, Hb: 12.1 g/dl, Plt: 14.6 x 10(4)/microliter, LDH: 951 IU/l, sIL-2R: 2,553 IU/ml, and ferritin: 5998.1 ng/ml. Patient 2 was a 73-year-old man with a diagnosis of diffuse large B-cell lymphoma originating in the right orbita (Clinical stage: IV B). Laboratory findings on admission were WBC: 9,100/microliter, Hb: 7.7 g/dl, Plt: 15.4 x 10(4)/microliter, LDH: 1,043 IU/l, sIL-2R: 10,090 IU/ml, and ferritin: 2079.3 ng/ml. Both patients had high-grade fever and extremely high serum cytokine levels. Bone marrow aspiration disclosed many histiocytes with hemophagocytosis. In both cases, combined chemotherapy was transiently effective, but patient 1 died of relapse of HPS and patient 2 of cerebral bleeding. Orbital non-Hodgkin's lymphoma with HPS is rare. These cases were interesting in terms of the relationship between HPS and the primary site of lymphoma.
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PMID:[Two cases of orbital non-Hodgkin's lymphoma presenting with hemophagocytic syndrome]. 1069 99

After 2 years of steroid therapy that had effectively controlled her systemic lupus erythematosus, a 37-year-old woman presented with fever, erythema (face, upper chest), and low CH50. Increased oral steroid (prednisolone from 15 mg to 40 mg) and intravenous methylprednisolone (mPSL) (80 mg for 3 days) alleviated these symptoms except for the fever. Subsequently, the patient's fever worsened and leukocytopenia, abnormal liver function, lymphadenopathy (neck, axilla), and salivary gland swelling developed. Lymph node histology revealed features characteristic of Kikuchi-Fujimoto's disease (KFD). Laboratory examinations showed WBC 600/microliter, Hb 9.5 g/dl, platelets 90,000/microliter, GOT 766 IU/l, GPT 646 IU/l, LDH 4,228 IU/l, TG 1,622 mg/dl, and ferritin 6,330 ng/ml. Serum interferon gamma was also elevated (673 U/ml). Because a bone marrow smear revealed hemophagocytosis, mPSL pulse therapy (1 g for 3 days) was started for treatment of hemophagocytic syndrome. The fever promptly disappeared, and the patient's clinical symptoms resolved within 2 weeks. The abnormal laboratory data related to KFD and hemophagocytosis returned to normal within 4 weeks after the initiation of mPSL pulse therapy. We speculated that the hemophagocytosis and salivary gland involvement in this patient were also symptoms of KFD. This case indicated that corticosteroid pulse therapy is effective for KFD with serious clinical symptoms.
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PMID:[Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto's disease) accompanied by hemophagocytosis and salivary gland swelling in a patient with systemic lupus erythematosus]. 1069


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