Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.1.3.1 (alkaline phosphatase)
 47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lipopigment (LP) deposition was studied in a series of 36 control and 79 pathological spleens. In the control group the LP deposition in SSE was rudimentary and did not display age-dependence. A varying degree of lysosomal and cytoplasmic siderosis was a frequent finding in haemolytic anemia without any significant LP induction. In the acquired secondary storage syndrome and in some inherited lysosomal enzymopathies, the amount of LP in splenic sinus endothelium (SSE) was significantly increased and in some instances its deposition reached very high values. As deposition was not accompanied by any detectable lysosomal lipid storage phenomenon in pulpar histiocytes, the pigmentogenesis is thought to be by a process resembling that for lipofuscin. In ceroid-lipofuscinosis group the SSE affection was of low degree, as seen in other viscera. The LP deposition seems thus to be a prominent, albeit variable feature of human SSE lysosomal pathology and may represent a monotonous response to various stimuli connected with increased demands on the SSE lysosomal system. Only in some lysosomal enzymopathies, typically in sphingomyelinase deficiency was SSE LP deposited progressively and concurrently with the stored lipid. LP deposition was accompanied by an increase in lysosomal enzyme activities but lacked the alkaline phosphatase induction in SSE described in lipid and mucopolysaccharide storage diseases. This and several other features which are reviewed clearly distinguish SSE from the pulpar histiocytes with which they have been often identified.
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PMID:Deposition of lipopigment--a new feature of human splenic sinus endothelium (SSE). Ultrastructural and histochemical study. 210 29

Renal osteodystrophy (ROD) is a multifactorial disease. Aluminium deposits have been implicated in its physiopathology but iron deposits have seldom been described. The purpose of this study was to investigate the presence of iron on the mineralization front, in 70 patients with ROD. Their mean age was 48+/-16 years, 36 were female, 34 male, 55 were admitted on peritoneal dialysis (78.5%) and 15 to haemodialysis (21.5%), for a period of 28+/-22 months. A bone biopsy was obtained from each patient after double tetracycline labelling. Blood samples were also obtained at the time of bone biopsy. The histomophometric analysis was performed following the criteria of Sherrard et al., with slight modifications; beside the usual stains, aluminium, iron and amyloid stainings were done on all bone specimens. Biochemical findings were: Ca 8.8+/-0.9 mg/dl, P 6.1+/-1.5 mg/dl; total alkaline phosphatase 197+/-258; PTHm 4.9+/-4.05ng/ml (normal 0.4-0.7 ng/ml), calcitonin 11+/-6 pg/ml (normal 1-26 pg/ml). Osteitis fibrosa was found in 31 patients (44.28%), mixed bone disease in two patients (2.28%); mild bone disease in 20 subjects (28.57%), adynamic bone lesion in 15 cases (21.42%) and osteomalacia in two patients (2.28%). Iron deposits were found on the mineralization front in 43 patients (61.4%); in 17, the percentage was <25 and, in 26, >25%. The iron deposits in the osteitis fibrosa group were highly significant (25/31). The aluminium deposit at the mineralization front was observed in eight patients (11.4%); in all but one, the percentage of this metal was <10%. Amyloid deposits were negative in all cases. The results show: (i) a Mexican population with ROD, present a highly significant incidence of siderosis on the bone mineralization front; (ii) in contrast, the aluminium deposits in this group of patients is lower than that reported in other series, and (iii) the spectrum of RO in this Mexican population is similar to that reported in other studies.
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PMID:High frequency of iron bone deposits in a Mexican population with renal osteodystrophy. 956 20

A 52-year-old female underwent autologous BMT because of acute myeloid leukaemia FAB M4 in second remission. Since the patient had no HLA-identical sibling she received a purged autologous BM transplant. On day +5 she developed signs of a sepsis syndrome with fluid retention and was treated with broad-spectrum antibiotic therapy. However, her body weight remained high, ascites and an increase of total serum bilirubin and alkaline phosphatase developed. The icterus worsened to a total bilirubin level of 25 mg/100 ml. Sonographic and endoscopic imaging showed a dilated gall bladder but disclosed a post-hepatic cause for the icterus. A transjugular liver biopsy on day +71 revealed severe cholestasis and siderosis. The patient remained aplastic with constantly increased bilirubin levels. On day +73 septic shock syndrome occurred and the patient died of multiorgan failure 3 days later. At autopsy, a highly differentiated bile duct adenocarcinoma at the porta hepatis, so-called Klatskin tumour, was found, explaining the fatal course with intractable cholestasis.
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PMID:Bile duct adenocarcinoma mimicking veno-occlusive disease after autologous bone marrow transplantation for acute leukaemia. 967 64

Patients with sickle cell disease (SCD) frequently have bone disorders of multifactorial aetiology. We attempted to analyse the relationships between bone mineral density (BMD) on the one hand and auxologic parameters, degree of siderosis, function of the growth hormone (GH)/insulin-like growth factor-I (IGF-I)/IGF-binding protein 3 (IGFBP3) axis, and calcium-phosphate balance in 28 prepubertal children with SCD and 15 age-matched children with constitutional short stature (CSS). Children with SCD had significantly decreased BMD (77.9 +/- 11.9 per cent of normal BMD for age and sex) and circulating concentrations of IGF-I (91 +/- 31 ng/ml) and IGFBP3 (1.7 +/- 0.44 mg/l) compared with the control group (BMD = 93.5 +/- 8.2 per cent of normal BMD for age and sex, IGF-I = 221 +/- 48 ng/ml, and IGFBP3 = 2.3 +/- 0.34 mg/ml). GH response to provocation was defective (peak below 10 micrograms/l) in 40 per cent of children with SCD. Those with SCD with defective GH secretion had significantly lower circulating IGF-I concentration and BMD than those with normal GH secretion. Serum calcium, phosphate and alkaline phosphatase concentrations were normal in all children with SCD. BMD was correlated significantly with height, weight, and body mass index as well as with the circulating concentrations of IGF-I and IGFBP3. It is suggested that increasing the circulating IGF-I concentration, either through increasing the caloric intake of subjects and/or via GH/IGF-I therapy, may improve growth and bone mineralization in these patients.
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PMID:Decreased bone mineral density in prepubertal children with sickle cell disease: correlation with growth parameters, degree of siderosis and secretion of growth factors. 971 3

The behavior of hepatitis C in states of immunodeficiency is poorly understood and it is still unclear whether the characteristics of hepatitis C virus (HCV) infection in renal transplant patients differ from those observed in immunocompetent subjects. The aim of this study was to compare the biochemical and histologic characteristics of chronic HCV infection between renal transplant and immunocompetent patients. Forty-one HCV-RNA-positive renal transplant patients and 41 immunocompetent controls matched for gender, age at infection and time of infection were included in the study. The groups were compared regarding laboratory and histologic variables. Renal transplant patients showed lower alanine aminotransferase (ALT) levels (p = 0.005) and higher levels of gamma-glutamyltransferase (p = 0.003), alkaline phosphatase (p < 0.001), and direct bilirubin (p < 0.001) when compared with controls. Histologic analysis revealed less intense portal (p < 0.001) and periportal (p = 0.046) inflammatory infiltrate in renal transplant patients but a larger proportion of cases with confluent necrosis (p = 0.043). No difference in the presence of septal fibrosis, hepatic steatosis, bile duct injury and siderosis was observed. However, there was a difference in the presence of lymphoid aggregates, which were less frequent in the renal transplant group (p < 0.001). In conclusion, the characteristics of hepatitis C in renal transplant patients differ from that observed in immunocompetent patients. In renal transplant patients, HCV infection is biochemically characterized by lower ALT levels and higher frequency of cholestasis. Regarding histology, despite lower frequency of lymphoid aggregates and less intense portal/periportal inflammatory infiltrate, a greater lobular damage was observed. The impact of these differences on the progression of fibrosis remains to be established.
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PMID:Hepatitis C virus infection in renal transplant patients: a comparative study with immunocompetent patients. 1631 22

Tamoxifen (TX), a drug used in the treatment of breast cancer, may cause hepatic changes in some patients. The consequences of its use on the liver tissues of rats with or without diabetes mellitus (DM) have not been fully explored. The purpose of this study was to evaluate the correlation between plasma hepatic enzyme levels and the presence of iron overload in the hepatic tissue of female Wistar rats with or without streptozotocin-induced DM and using TX. Female rats were studied in control groups: C-0 (non-drug users), C-V (sorbitol vehicle only) and C-TX (using TX). DM (diabetic non-drug users) and DM-TX (diabetics using TX) were the test groups. Sixty days after induced DM, blood samples were collected for glucose, alanine aminotransferase (ALT), aspartate aminotransferase (AST) alkaline phosphatase (ALP) and bilirubin measures. Hepatic fragments were processed and stained with hematoxylin and eosin, Masson's trichrome, Perls. The hepatic iron content was quantified by atomic absorption spectrometry. AST, ALT and ALP levels were significantly elevated in the DM and DM-TX groups, with unchanged bilirubin levels. Liver iron overload using Perls stain and atomic absorption spectrometry were observed exclusively in groups C-TX and DM-TX. There was positive correlation between AST, ALT and ALP levels and microscopic hepatic siderosis intensity in group DM-TX. In conclusion, TX administration is associated with liver siderosis in diabetic and non-diabetic rats. In addition, TX induced liver iron overload with unaltered hepatic function in non-diabetic rats and may be a useful tool for investigating the biological control of iron metabolism.
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PMID:Liver iron overload induced by tamoxifen in diabetic and non-diabetic female Wistar rats. 1763 94

Genetic hemochromatosis is an iron overload disorder, and osteopenic and osteoporotic. Femoral neck bone mineral density (BMD) appears to fall with rising hepatic iron concentrations. A critical role for iron in mediating tissue injury is played via hydroxyl radical formation in nephrotoxicity. We investigated the effects of a colloidal iron overload on renal function, organ siderosis, and femoral bone in male rats. Iron overload reduced body growth, and increased the weights of the liver and spleen. Marked deposition of iron was noted in liver and kidney. Activities of lactate dehydrogenase and alkaline phosphatase were decreased, and the concentrations of blood urea nitrogen and creatinine were increased with the reduction in plasma calcium and inorganic phosphorus levels, i.e. functions of the liver and kidney might be affected by reactive oxygen species such as the superoxide radical, H2O2, and the hydroxyl radical produced by overloaded iron. Damage to the proximal tubular epithelial cells of the kidney and a loss of connectivity of cancellous bone in the epiphysis and of trabecular bone in the metaphysis of the distal femur were observed in iron-overloaded rats with a reduction of femoral bone mineral density, i.e. reabsorption of calcium from the proximal tubular epithelial cells of the kidney might be affected and urinary discharge of calcium might be elevated. It was suggested that iron overload gave rise to osteoporosis combined with renal dysfunction and liver iron overload syndrome.
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PMID:Effects of colloidal iron overload on renal and hepatic siderosis and the femur in male rats. 1828 63