Treatment-induced copper deficiency in two patients with Wilsons disease

Copper is an essential trace element that is vital to the health of all living cells. However, like all essential elements, its serum level must be kept within normal values; otherwise, conditions of toxicity or deficiency can result, each of which has its own unique set of adverse health effects. Wilson's disease [WD] is an inherited disease of copper accumulation that can cause liver and neurological affection. Its management depends on removal of excess copper using copper chelators such as D-penicillamine or trientine, which increase the urinary excretion of copper. In a more recent approach, zinc has been used to decrease copper accumulation. We present two WD cases that developed copper deficiency as a result of their treatment. These cases improve our understanding and management of copper deficiency in WD and highlight the importance of copper-level monitoring in WD

Study of some detection mechanisms for multidrug resistance P-gp and mRNA in acute leukemia and correlation to clinical outcome

Leukemic blast samples from 32 patients with acute leukemias [AML] were studied by / immunofluorescence/ flowcytometry [IFL/FCM] and by immunocytochemistry [ICH] with three monoclonal antibodies [MoAbs] to the multidrug resistance protein [P-glycoprotein, P-gp]. MDRI RNA levels in the same blasts were determined by RT-PCR. IFL/FCM staining with C219-FITC showed >5% positive cells in 14/32 cases [43.7%], the mean percentage of positive cells being 24.5%, In ICH staining, the percentages of positive cells varied with different MoAbs, but the results obtained with C219 and MRK16 correlated with those obtained with JSB1. No correlation could be demonstrated between the percentages of cells expressing P-gp and Mdrl-mRNA. Ninety one point seven% of patients with Mdrl RNA negative leukemia reached complete remission as compared to 9.3% expressing higher levels of MDRI mRNA [p<0.0001]. There was no correlation between the response to therapy and the results of IFL/ICH staining. We conclude that the results of immunostaining for P-gp must be interpreted cautiously and probably novel, more specific MoAbs should be raised before any therapeutic use can be made of the findings. Also, PCR detection of MDRI mRNA could be of prognostic value in AML

Macrophage inflammatory protein 1 - alpha [MIP -I]: its pathogenetic role in some connective tissue diseases

This study aimed to measure serum level of macrophage inflammatory protein 1- alpha [MIP-la] in some connective tissue diseases as rheumatoid arthritis [RA], systemic lupus erythematosus [SLE] and systemic sclerosis [SSc] in order to clarify its possible role in their immunopathogenesis. Fifteen RA patients [group I], 15 SLE patients [group II], 10 SSc patients [group III] and 10 healthy controls [group IV] were participated in this study. A full history taking, thorough clinical examination and routine rheumatological profile investigations were done. Serum MIP-1-alpha was measured in all patients and control groups using ELISA technique. In conclusion, the higher level of serum MIP-1-alpha in RA, SLE and SSc patients could reflect its immunopathogenetic role as an important stimulator of T cells, monocyte macrophage as well as its variable pro-inflammatory activates

Serum adrenomedullin level in rheumatoid arthritis compared with systemic lupus erythematosus

This study was conducted to measure the level of serum adrenomedullin [AM] in rheumatoid arthritis [RA] and systemic lupus erythematosus [SLE] to find out its role in their pathogenesis and its correlation with clinical and laboratory parameters of disease activity. Twenty-five RA patients [group I], 15 SLE patients [group II] and 10 healthy controls were included in this study. They were subjected to full history taking, thorough clinical examination and CBC, ESR, RF, ANA, Ant ds DNA investigations and measurement of serum adrenomedullin level with ELISA. An assessment of disease activity was done using Duke score for RA patients and SLE-DAI score for SLE. From the results obtained, it was concluded that serum level of AM was elevated in both RA and SLE [more in RA than SLE] patients and it was correlated with the disease activity parameter RA [only with ESR in SLE]. These findings emphasized that AM is involved in the pathogenesis of RA and SLE

Macrophage inflammatory protein 1-alpha [MIP-1alpha]: its pathogenic role in some connective tissue diseases

To measure serum level of macrophage inflammatory protein 1alpha [MIP-1alpha] in some connective tissue diseases as rheumatoid arthritis [RA], systemic lupus erythematosus [SLE] and systemic sclerosis [SSc] in order to clarify its possible role in their immunopathogenesis Fifteen RA patients [Group I], fifteen SLE patients [Group II], ten SSc patients [Group III] and 10 healthy controls [Group IV] were participated in this study. A full history taking, thorough clinical examination and routine rheumatological profile investigations were done. Serum MIP-1alpha was measured in all patients and control groups using ELISA technique. The three patients groups [RA, SLE and SSc] had a significantly higher level [p<0.001] of serum MIP-1alpha [118.9 +/- 31.4 pg/mL, 106.2 +/- 13.3 pg/mL and 94.2 +/- 4.6 pg/mL] respectively when compared to controls [70.3 +/- 5.4 pgm/mL]. The highest level was among RA patients. Furthermore, there was a significant positive [p<0.05] correlation between serum MIP-1alpha level and disease activity parameters [Duke score, ESR in RA patients; SLEDAI and ESR in SLE patients and only ESR in SSc patients]. The higher level of serum MIP-1alpha in RA, SLE and SSc patients could reflect its immunopathogentic role as an important stimulator of T cells, monocyte macrophage as well as its variable proinflammatory activites. Future theraputic researches should target MIP-1alpha which play an important immunopathogenetic role in chronic inflammatory diseases

Early effects of esophageal injection sclerotherapy and band ligation on coagulation and bleeding profile

The present study was performed with the aim of comparing the systemic coagulopathy and bleeding profile following endoscopic variceal sclerotherapy with 5% ethanolamine oleate versus that following band ligation, where they were estimated just before then one hour and 2 hours after the procedure. Twenty patients were randomly allocated into two groups, the ligation group [10 patients],with a mean age of [48.5 +/- 10.9 years] and the sclerotherapy group [10 patients],with a mean age of 44 +/- 8.5 years. In the ligation group, no statistically significant differences were obtained on comparing coagulation and bleeding parameters through out the study time.As regards the sclerotherapy group, on comparing these parameters one hour after the procedure to those just before it, there were significant decrease in platelet count, factor VIII concentration, prothrombin concentration and significant prolongation of prothrombin time, partial thromboplastin time. Non significant results were obtained on comparing the tested parameters 2 hours after the procedure with those one hour after it. While on comparing parameters 2 hours after sclerotherapy to those just before it, there were significant changes of the same parameters changed before, denoting that these changes occuring one hour after sclerotherapy are maintained till the second hour. On comparing both groups of patients with each other, regarding parameters one hour after the procedure to those just before it and also 2 hours after the procedure to those just before it, there were significant prolongation of prothrombin time, partial thromboplastin time and highly significant decrease in prothrombin concentration in the sclerotherapy group. In conclusion, only the group of patients undergoing elective intravariceal sclerotherapy using 5% ethanolamine oleate, showed mild changes in the coagulation and bleeding profile. These changes were found one hour after the procedure and were maintained up to the second hour. Thus, endoscopic variceal ligation appears to be a technique of less negative impact on coagulation and bleeding profiles when compared with sclerotherapy and may be considered as the procedure of choice for treatment of bleeding esophageal varices especially in patients with marked thrombocytopenia and coagulation abnormalities, because in such patients scierotherapy might worsen the preexisting coagulation abnormalities and precipitated haemorrhagic complication