Oxidative stress and hepcidin expression in pediatric sickle cell anemia with iron overload.

BACKGROUND: Blood transfusion (BT) is essential in treating sickle cell disease (SCD); however, it leads to iron overload (IO) and oxidative stress. We studied the relationship between oxidative stress, iron status parameters, hepcidin mRNA gene expression, and IO in SCD patients. METHODS: We classified all SCD patients (n = 90) into two groups: Group I, 45 children (s.ferritin ≥ 938 ng/mL) and Group II, 45 children (s.ferritin < 938 ng/mL). A total of 55 children, age and sex matched, participated as a control group. Malondialdehyde (MDA), nitrite, s.iron, s.total iron-binding capacity (sTIBC), transferrin saturation %, s.ferritin, s.hepcidin, and hepcidin mRNA gene expression were assessed. RESULTS: Among SCD BT-dependent patients (>3 times/year), 63% were from Group I and 37% from Group II, p < .01. The two patient groups had significantly lower s.hepcidin and hepcidin gene expression than controls ( p < .001). TIBC, s.iron, s.ferritin, transferrin saturation %, ferritin/hepcidin ratio, and MDA levels were higher among SCD patients than controls ( p < .001). Group I had higher mean level of ferritin/hepcidin ratio and MDA than Group II ( p < .01). The higher level of MDA and increased frequency of BT were the significant predicting risk factors for IO ( p < .05). A receiver-operating characteristic curve indicates that MDA is the outstanding significant biomarker for high level of s.ferritin with subsequent IO progression. CONCLUSION: MDA may serve as a biomarker of oxidative stress and IO in SCD patients. This result paid attention for urgent initiation of antioxidant and chelation therapy on detecting increased MDA level.

Recipient site preparation by cryoblebbing in melanocyte keratinocyte transplantation procedure over the fingers in vitiligo: A pilot study.

Surgical treatment of vitiligo lesions over the fingers has poor outcome. In this intra-patient comparative study, 12 patients with stable non-segmental vitiligo (NSV) affecting the middle three fingers of one hand were included. Three variations were used in treatment of finger vitiligo lesions: minipuch grafting, melanocytes keratinocyte transplantation procedure (MKTP) preceded by cryoblebbing or full CO2 laser resurfacing of the recipient site. Liquid nitrogen was used to create blebs in one finger 24 hours before therapy. On the following day, the second finger was treated by minipunch grafting and the third finger was resurfaced by CO2 laser. A suspension was prepared and 0.1 mL was injected into each cryobleb. It was also applied to the resurfaced skin. All patients underwent topical PUVA therapy and were followed-up for 12 months. Ten cases with 52 lesions completed the follow-up period. About 4/18 lesions treated by cryoblebbing followed by MKTP showed ≥75% repigmentation while only 1/17 lesions treated by laser resurfacing + MKTP and 1/17 lesions treated by minipunch grafting showed 30% and 10% repigmentation, respectively. No complications occurred in MKTP treated lesions. Cryoblebbing of the recipient site seems to improve the outcome of MKTP in lesions over the fingers in stable NSV.

Bone Health and its Relation to Energy Intake, Fat Mass and its Distribution.

BACKGROUND AND OBJECTIVES: Osteoporosis and obesity are two of the most important inter-related diseases worldwide. This study aimed to investigate impact of fat mass and its distribution on bone health in relation to energy intake among sample of Egyptian women. MATERIALS AND METHODS: A cross-sectional study included 116 Egyptian women with age range 25-65 years old. They were classified according to the menopause into 2 groups: Pre-menopausal (n = 51) and post menopausal (n = 65). All participants have undergone anthropometric measurements, body composition, DEXA and laboratory investigations. RESULTS: Among overweight/obese women, pre-menopausal women had significant higher values of BMR and BMD at both lumbar spines, neck of femur and significant lower values of central obesity (waist/hip ratio, waist/height ratio, visceral fat) and C-terminal peptides than postmenopausal ones. Among pre and post-menopausal women, BMD at both sites had significant positive correlations with obesity markers (BMI, waist and hip circumferences), fat mass, BMR, in addition to fat distribution, visceral fat, leptin among pre-menopausal women and C-terminal peptide among postmenopausal women. Among pre-menopausal women, BMR significantly explained 56% of the variations in BMD at neck of femur, while at lumbar spines the best model was BMI, BMR and waist circumference, which significantly explain 33% of the variations in BMD. CONCLUSION: Bone health positively correlated with BMI, fat mass and its distribution and BMR, particularly at femur neck, among pre and post-menopausal Egyptian women. Overweight/obesity can be considered as a protective factor for bone health.

Studying the effect of adding growth factors to the autologous melanocyte keratinocyte suspension in segmental vitiligo.

Addition of different growth factors to the medium used in autologous melanocyte-keratinocyte transplantation procedure (MKTP) was reported in the literature. The aim of the current study was comparison of response to MKTP in segmental vitiligo (SV) with and without adding growth factors to the suspension medium. Eighteen cases with SV were randomly divided into two groups. In group A: Ham F12 medium was used for suspension and in group B: 5 ng/mL recombinant basic fibroblast growth factor (bFGF) and 25 mg/500 mL 3'5' cyclic adenosine monophosphate (cAMP) were added to the medium. All cases received NB-UVB twice weekly for 24 weeks. The area of vitiligo lesions was measured before and after therapy by point-counting technique and complications were recorded. Excellent response (90%-100% repigmentation) occurred in 5/9 cases (56%) in group A and 7/9 cases (78%) in group B (with growth factors). A significant decrease in the area of treated lesions before and after therapy was found in both groups A and B (P = .0012 and .0004, respectively), however, a higher percentage of reduction in area of vitiligo was seen in group B cases (70% in group A vs 90% in group B; P value: .028). Marginal halo was seen in five cases in group A and six in group B. In conclusion addition of bFGF and cAMP to MKTP medium improved the results of the procedure. It could be considered if economically feasible.

Genetic polymorphisms of fecundity genes in Watish Sudanese desert sheep.

BACKGROUND AND AIM: The Watish sheep is a strain of desert sheep of smaller size compared to other desert sheep ecotypes, and there is anecdotal evidence that it is endowed with high litter size. The present study was designed for screening for polymorphisms in the known fecundity genes (bone morphogenetic protein receptor type 1B A

MicroRNA-150 down Regulation in Acute Myeloid Leukaemia Patients and Its Prognostic Implication.

BACKGROUND: MicroRNAs (miRNAs) are small, non-coding RNAs that are important for post-transcriptional gene regulation in both healthy and morbid conditions. Numerous miRNAs promote tumorigenesis, while others have a tumour suppressive effects. Acute myeloid leukaemia (AML) is a heterogeneous group of genetically diverse hematopoietic malignancies with variable response to treatment. AIM: Our study aimed to investigate the possible role of miR-150 in de novo adult AML and the impact of its level on survival, and we used in the silicon analysis to predict the main target genes involved in miR-150 mediated cancer pathway. MATERIAL AND METHODS: We evaluated miR-150 expression profiling assay using TaqMan primer probes RT-PCR in the plasma of 50 adult AML patients, before the start of treatment and at day 28 of treatment, along with 20 normal adult control samples. miR-16 was used as an endogenous reference for standardisation. Follow-up of patients during treatment at day 28 of induction chemotherapy and after one year was done. RESULTS: In this study, we found a significantly lower level of miR-150 in AML patients when compared to controls (p = 0.005) with 0.62 fold change than in healthy controls. Patients were divided into two groups: the low miR-150 group (miR-150 < 1) and the high miR-150 group (miR-150 > 1). A statistically significant difference was found between the two groups regarding initial total leukocytic count and initial PB blast count while for the TLC, HB and PLT count at follow up. No difference in the overall survival between the low and the high miR-150 groups could be demonstrated. CONCLUSION: Our results suggest that miR-150 functions as a tumour suppressor and gatekeeper in inhibiting cell transformation and that its downregulation is required for leukemogenesis.

Increased tenascin C and DKK1 in vitiligo: possible role of fibroblasts in acral and non-acral disease.

Recently, multiple culprits-in addition to melanocytes-have been implicated in the pathogenesis of vitiligo. Among those factors are fibroblasts. However, their exact role has not been clearly elucidated. The aim of the study was to evaluate the possible role played by fibroblasts in vitiligo via studying the expression Tenascin C and DKK1 in acral versus non-acral vitiligo lesions. This case-control study included 19 non-segmental vitiligo patients and ten controls. All patients were subjected to thorough clinical evaluation. Both Tenascin C and DKK1 were measured in lesional and peri-lesional skin of acral and non-acral lesions using ELISA technique. The measured levels of Tenascin C and DKK1 were significantly higher in the vitiligo group when compared to controls in all assessed sites (P < 0.05). Tenascin C was found to be significantly higher in lesional areas compared to peri-lesional ones only in the acral sites. DKK1 was significantly higher in lesional areas in all assessed sites (P < 0.05). The current work suggests a malfunction of fibroblasts in vitiligo, through demonstrating significant up-regulation of two melanogenesis inhibitory products (Tenascin C and DKK1) in patients compared to controls. Larger scale studies are warranted to detect the possible implications of such findings on vitiligo treatment.

Propranolol and infantile hemangiomas: different routes of administration, a randomized clinical trial.

UNLABELLED: Oral propranolol has become the treatment of choice of infantile hemangiomas (IH)s. However, the safety of systemic propranolol is questioned. Topical therapy with 1% propranolol has been reported to be safe and effective. Intralesional (IL) administration may possibly allow safe delivery of higher drug dosages. AIM: To assess the efficacy and safety of two locally administered routes of propranolol (topical and IL), in comparison with its systemic oral use in the treatment of IHs. PATIENTS AND METHODS: 45 patients with IHs were randomly divided into 3 groups, A, B and C (n = 15 in each), receiving oral propranolol, 2 mg/kg/day, topical propranolol 1% ointment twice daily, IL propranolol, 1 mg of propranolol hydrochloride in 1 ml of injection once weekly, respectively. Follow up was done for 6 months after treatment was stopped. RESULTS: Excellent response was achieved in 9 patients in group A (60%), 3 in group B (20%) and 2 in group C (13.3%), (P value : 0.04). As regards safety, all 3 modalities proved safe with no major side effects apart from 1 patient in group A and 3 in group C who dropped out due to pain or inconvenience of therapy. CONCLUSIONS: Further work is needed to establish clear guidelines and reach best formulations. Nevertheless, in properly selected patients with IHs, we recommend the usage of oral propranolol. Topically administered propranolol could be considered in patients at risk of potential side effects from oral administration. As IL application did not offer any more benefits, it could not be recommended.

Role of student pharmacists in the identification and prevention of medication-related problems.

OBJECTIVES: To measure the total rate of attending physician, medical resident, and medical student acceptance of interventions recommended by third-year student pharmacists supervised by a licensed pharmacist and to determine the type of health care provider group that accepted the most interventions, type of interventions provided, and number of patients counseled on medication use. METHODS: This prospective unblinded pilot study followed single interventions documented by nine third-year student pharmacists enrolled at the University of Louisiana at Monroe College of Pharmacy. Success rates of suggested interventions made to family medicine attending physicians, pharmacists, medical residents, and medical students in three Louisiana outpatient clinics were documented. Student pharmacists performed medication histories, identified potential medication-related problems, discussed interventions with health care providers, and counseled patients on medication use. Outcomes assessed were intervention type and number, intervention acceptance rate, and number of patients counseled. RESULTS: Student pharmacists communicated with attending physicians, medical residents, and/or medical students regarding 32 patients. A total of 48 interventions were documented. Of these interventions, 34 were potential medication-related problems, 8 were pharmacotherapeutic recommendations for a new problem, 4 were responses to a health care provider's drug information requests, and 2 were referrals to a diabetes education class. The acceptance rate of student pharmacists' recommendations was 86%. Attending physicians accepted 93.8% of recommended interventions, medical residents 70%, and medical students 87.5%, Student pharmacists counseled 29 patients on medication use. CONCLUSION: Student pharmacists were able to recommend new drug regimens and discuss changes in therapy with health care providers. Attending physicians, medical residents, and medical students accepted the majority of students' recommendations. Third-year student pharmacists have the potential to serve an important role in the identification and prevention of medication-related problems by suggesting interventions to health care providers.

Oral propranolol: an effective, safe treatment for infantile hemangiomas.

Infantile hemangiomas (IH) are the most common childhood tumors. In 2008, Labreze reported the serendipitous effect of oral propranolol on hemangioma and since then it has overshadowed the use of other therapeutic modalities in the treatment of IH. The aim of this prospective, clinical study was to assess the efficacy and safety profile of oral propranolol at a fixed dose of 2 mgkg(-1) in the treatment of 30 patients with problematic IH. Propranolol treatment continued for a duration of 2-14 months where 60% of the patients (n=18) showed a final excellent response with complete resolution of the lesion (P<0.001). 20% (n=6) showed a good response with more than 50% reduction in the size of the IH. 16.6% showed a fair response (n=5) with less than 50% reduction in the size of the IH. Only one patient (3.3%) was resistant to treatment. Five patients (17.24%) showed evidence of rebound growth after cessation of therapy and responded well to re-treatment.We did not face any side effects related to the oral propranolol. In conclusion, propranolol therapy at a fixed dose of 2 mgkg(-1), given in three equally divided doses, is a very safe and effective regimen in the treatment of IH.