Effect of carnitine supplementation on cardiac function in hemodialyzed children

Thirteen carnitine-deficient children (mean age, 16.1 ±2.56 years) on a three-times-weekly hemodialysis program for at least 1 year, and 11 healthy age matched children were involved in the study. All the patients had stable blood pressure and hemoglobin (Hb) levels with a maintenance dose of erythropoetin and none were digitalized. The total carnitine (TC) and free carnitine (FC) plasma levels were sampled prior to hemodialysis (HD) before and after 3 months of carnitine supplementation. A free carnitine (FC) to acylcarnitine (AC) ratio less than 4 was defined as carnitine deficiency. Intravenous L-carnitine was injected at a dose of 20–4.0 mg/kg three times weekly at the end of each dialysis session for a 3-month period. Echocardiographic examination was performed the day following HD, before and after carnitine treatment. Systolic and diastolic functions of the left ventricle, including the ejection fraction, were measured. Almost all the parameters were significantly different in controls and hemodiaiyzed patients. In carnitine-deficient hemodiaiyzed patients. 3 months of L-carnitine supplementation resulted in a significant increase in blood carnitine levels and the FC/AC ratio, but this was not associated with any significant improvement of cardiac function. Furthermore no significant changes were observed in plasma triglycerides, total cholesterol or other lipoprotein parameters before or after carnitine supplementation. Although there was a moderate increase in mean hematocrit (Hct) and Hb levels, these also did not reach statistically significant levels. These results suggest that the 3 months of carnitine supplementation is not sufficient to ameliorate cardiac function or increase Hb levels in children.     

Viral hepatitis C infection among Egyptians the magnitude of the problem: epidemiological and laboratory approach

This study examines the relative importance of risk factors for viral hepatitis C infection and estimates the magnitude of HCV problem among Egyptians. It is a continuation of a recently performed cross-sectional study conducted on more than 5000 Egyptians. Serum samples (1945) were analyzed for liver enzymes (SGPT and SGOT) to evaluate the status of liver affection. One hundred and sixty nine samples (103 confirmed HCV seropositives and 66 seronegative) were analyzed for PCR HCV RNA to estimate the frequency of HCV viraemia among those individuals. Rates for HCV seropositivity by ELISA test in mass screening were corrected using predictive value of a positive test at prevalence between 5-50%. Attributable risk and population attributable risk estimates were calculated for those significant factors in logistic regression analysis. Overall number of HCV infected individuals was estimated for age groups of 15-65 years and the numbers of HCV related liver complications were estimated. HCV PCR RNA was positive for 50% of ELISA-seropositive samples and for 13.8% of the seronegative samples. However, only 5% of those individuals with HCV seropositivity and 3.7% of those with PCR RNA positivity Showed SGPT serum levels above 1.5 normal. The overall age, sex and urban/rural adjusted rate of true HCV seropositivity is estimated to be 15.6% among working Egyptians between 15-65 years. Attributable risk due to injections for the treatment of bilharziasis is estimated to be 47% (95% CL = 38%-55%) among exposed males. Blood transfusion was estimated to be responsible for 87% (95% CL = 57%-96%) of cases among previously transfused females. Population attributable risk for injections for treatment of bilharziasis among working urban and rural males is estimated to be 15% and 11% respectively. Blood transfusion and sharing contaminated needles contributed by 24% of cases (for each) among working urban females. Previous hospitalization contributed by 36% of cases among working rural females and by 10% among working urban males. As for the national estimate of cases of HCV seropositivity we estimated more than 5 million individuals with an expected number of chronic hepatitis of varying degrees of 3.5 millions. HCV viraemia with high probability for transmission is present in more than 50% of those individuals and liver cirrhosis cases expected to develop within an average of 20 years of infection are in the range of 350-700 thousand cases. The major proportions of population attributable risk are due to other undefined risk factors associated with age, male sex, living in rural areas and in lower and upper Egypt. Further research is needed to elucidate those factors prevailing in these areas, associated with increased risk of HCV infection.     

Sexual dysfunction in dermatological diseases

Decrease or loss of sexual function in many chronic diseases has recently attracted significant attention owing to its impact on quality of life. Generic and disease-specific quality-of-life questionnaires measure changes in work, school, social life and emotional status regarding the disease and its treatment. Specific questionnaires have been designed to evaluate changes in sexuality and sexual function. Sexual dysfunction, especially female sexual dysfunction, in different diseases became a popular and important health concern in recent years. There are a lot of studies about sexual dysfunction in the areas of other specialities of medicine, but there are only a few studies in dermatological diseases. In this paper, sexual dysfunction and the studies performed about this subject in dermatology will be reviewed.

Conflict of Interest

None declared.     

Molecular analysis of cutaneous B- and T-cell lymphomas

Among extranodal non-Hodgkin's lymphomas, primary cutaneous lymphomas (CLs) represent a consistent group of B- and T-cell malignancies. We investigated the arrangement of Ig and T-cell receptor (TCR) genes, together with the involvement of several oncogenes and the tumor- suppressor gene p53, in a panel of primary cutaneous B- and T-cell lymphomas (CBCLs and CTCLs). Southern blot analysis was performed to detect rearrangements of the Ig, c-myc, bcl-1, bcl-2, bcl-3, bcl-6, and the NFKB2/lyt-10 genes in 52 cases of CBCLs and of the TCR, bcl-3, and NFKB2/lyt-10 genes in 38 cases of CTCLs. tal-1 gene deletions were analyzed in CTCLs by means of polymerase chain reaction (PCR). p53 gene mutations were assayed using PCR, single-strand conformation polymorphism analysis, and direct DNA sequencing in CBCL and CTCL cases. Clonal rearrangements of Ig genes or oncogenes were found in 25 of the 52 CBCLs. In particular, we detected rearrangements of the bcl-1 locus (2 cases), the bcl-2 gene (2 cases), the NFKB2/lyt-10 gene (2 cases), and the bcl-6 gene (1 case); interestingly, 4 of these cases showed a germline arrangement of the Ig genes. Clonal rearrangements of TCR genes were detected in 37 of the 38 CTCLs. Rearrangements of the NFKB2/lyt-10 gene were present in 2 cases and tal-1 gene deletions in 3 CTCL cases; p53 gene mutations were detected in 1 CTCL case. Overall, our data indicate that (1) clonal rearrangement of Ig genes is frequently undetectable by means of Southern blot in CBCLs (60%); (2) genetic lesions are involved in a limited but significant fraction of primary CLs showing a molecular marker of clonality (13/62; 20%); and (3) rearrangements of the bcl-1, bcl-2, or bcl-6 loci, associated with specific subsets of nodal lymphoid neoplasias, are rarely observed in CBCLs. Moreover, our results suggest that tal-1 gene deletions may play a pathogenetic role in non-acute T-cell malignancies and that, in the context of lymphoid malignancies, CLs may represent a favorable target for the possible oncogenic potential of the NFKB2/lyt-10 gene.     

Transformed T lymphocytes infected by a novel isolate of human T cell leukemia virus type II

Human T cell leukemia virus type II (HTLV-II) has been isolated from a patient (Mo) with features of leukemic reticuloendotheliosis (LRE) and from a patient with acquired immunodeficiency syndrome (AIDS). We have obtained another isolate of HTLV-II from a patient (CM) with severe hemophilia A, pancytopenia, and a 14-year history of staphylococcal and candidal infections but no evidence of T cell leukemia/lymphoma, AIDS, or LRE. Fresh mononuclear cells and cultured lymphocytes from CM express retroviral antigens indistinguishable by molecular criteria from HTLV-IIMo. Leukocyte cultures from CM yield hyperdiploid (48,XY, +2, +19) continuous lymphoid lines; human fetal cord blood lymphocytes (CBL) are transformed by cocultivation with these CM cell cultures but retain normal cytogenetic constitution. Electron microscopic examination of the CM cultures and transformed CBL reveals budding of extracellular viral particles, intracellular tubuloreticular structures, and viral particles contained within intracellular vesicles. CM cell cultures and the transformed CBL do not require exogenous interleukin 2, have T cell cytochemical features and mature T helper phenotypes, and exhibit minimal T helper and profound T suppressor activity on pokeweed mitogen-stimulated differentiation of normal B cells. These characteristics, which are similar to those observed with the first HTLV-II isolate, may represent properties of all HTLV-II-infected T cells.