Effective and Elaborative Induction Program for Mitigating Myths and Misconceptions Linked to Hematopoietic Stem Cell Transplantation in a Resource Limited Setting.

Since the first transplant in 1957 and hematopoietic stem cell transplantation (HSCT) is the curative modality for numerous hematological disorders. Nevertheless, it is not available for all patients. Besides unavailability of matched donors a lot of factors could hinder HSCT in a resource limited setting, as financial and administrative factors. In our daily practice we noticed other factors that hinder HSCT in our center, the common myths and misconceptions about HSCT and donation. This quasi-experimental study assessed, for the first time, common myths and misconceptions about HSCT among 218 medical and nursing students before and after an interventional educational program. The study tool was an investigators' developed self-administered questionnaire. Participants' male to female ratio was 1:2.5, and FAS was middle in 52.7%. Pretest high myths scores were reported in 53.4% and 90% of medical and nursing students that was reduced to 0% and 4% post-test, respectively. Pretest, 26.3% and 7% of medical and nursing students welling to donate HSC, that increased to 66% and 39% post-test, respectively. Rural residency, low and middle FAS associated with higher myths scores. Myths score is an independent effector of willingness to donate HSC among participants. In conclusion medical/nursing students had significant myths and misconceptions about HSCT that was corrected with the educational program. Thus, wide based educational programs about HSCT are mandatory to correct myths and augment HSC donation. www.clinicaltrrial.gov: clinical trial ID NCT05151406. Supplementary Information: The online version contains supplementary material available at 10.1007/s12288-023-01634-5.

Are normolipidaemic patients with xanthelasma prone to atherosclerosis?

BACKGROUND: When patients with xanthelasma are found to have normal lipid levels, dermatologists usually proceed with their treatment without further investigations. However, there is some evidence that normolipidaemic patients with xanthelasma (NPX) have a similar cardiovascular risk to hyperlipidaemic patients with xanthelasma (HPX). AIM: To evaluate the risk of atherosclerosis in Egyptian NPX compared with HPX and controls. METHODS: In total, 20 NPX, 20 HPX and 40 normolipidaemic controls were enrolled. All participants were matched for age and sex. Diabetes was an exclusion factor. Carotid ultrasonography was used to measure intima-media thickness (IMT). Other risk factors of atherosclerosis such as high blood pressure, obesity and smoking were also assessed, as well as atherosclerotic markers, including total leucocytic count (TLC), C-reactive protein and lipoprotein a. RESULTS: Although still within the normal range, total cholesterol and triglycerides were significantly higher in NPX compared with controls. IMT was significantly higher in NPX compared with controls, but lower than that of HPX. The increased IMT in NPX was not related to any of the studied risk factors. Apart from significantly higher body mass index and TLC, NPX showed no significant differences from controls for other risk factors of atherosclerosis or for atherosclerotic markers. CONCLUSION: NPX seem to have a higher risk of atherosclerosis independent of lipid concentrations, and should therefore be fully investigated in order to allow detection and early management of such risk.

Acral lesions of vitiligo: why are they resistant to photochemotherapy?

BACKGROUND: Acral lesions of vitiligo are usually resistant to conventional lines of treatment as well as surgical interventions. OBJECTIVE: To clarify causes underlying resistance of acral lesions to pigmentation in vitiligo by studying some of the factors associated with mechanisms of repigmentation following photochemotherapy. METHODS: The study included twenty patients with active vitiligo. Skin biopsies were taken from lesional and perilesional skin of areas expected to respond (trunk and proximal limb) and skin of acral areas, before and after PUVA therapy. Sections were stained with H and E, Melan-A, MHCII, CD1a, SCF and c-kit protein. RESULTS: Before treatment acral areas showed significantly lower hair follicle density, melanocyte density, Langerhans cell (LC) density, epidermal MHCII expression, lesional SCF expression and perilesional c-kit expression. Following treatment with PUVA in both non-responsive acral and repigmenting non-acral lesions identical immunohistochemical changes in the form of significant decrease in LC density, epidermal MHC-II and SCF expression were observed. CONCLUSION: The surprisingly similar histochemical changes in response to PUVA in acral and non-acral lesions did not manifest with clinical repigmentation except in non-acral ones. Factors such as inherent lower melanocyte density, lower melanocyte stem cell reservoirs and/or lower baseline epidermal stem cell factor may be considered as possible play makers in this respect.

A large scale analytical study on efficacy of different photo(chemo)therapeutic modalities in the treatment of psoriasis, vitiligo and mycosis fungoides.

Psoriasis, vitiligo, and mycosis fungoides (MF) are among the most frequently treated dermatological diseases by photo(chemo)therapy. The objectives are to determine which photo (chemo) therapeutic modality could achieve the best response in the treatment of psoriasis, vitiligo, and MF. The design used in this study is retrospective analytical study. The study included 745 patients' records; 293 with psoriasis, 309 with vitiligo, and 143 with early MF, treated in the Phototherapy Unit, Dermatology Department, Kasr El-Aini Hospital, Cairo University by either psoralen and ultraviolet A (PUVA), narrow band ultraviolet B (NB-UVB), psoralen and narrow band UVB (P-NBUVB), broad band UVB (BB-UVB), or broad band UVA (BetaBeta-UVA). Data were retrieved from the computer database of the unit and statistically analyzed. In psoriasis, oral and topical PUVA and NB-UVB were found to be equally effective, whereas oral PUVA had significantly better results than both UVA and BB-UVB at the end of therapy. In generalized vitiligo, PUVA and P-NBUVB had significantly better results than NB-UVB alone. In early MF, there was no statistically significant difference between the response to oral PUVA and NB-UVB. PUVA and NB-UVB are good choices in patients with psoriasis and early stage MF, whereas PUVA appears the best choice in the treatment of vitiligo.

Narrow band Ultraviolet B 311 nm in the treatment of vitiligo: two right-left comparison studies.

AIM: Evaluation of narrow band ultraviolet B (NB UVB 311 nm) in the treatment of vitiligo by two independent studies. The first study compared NB UVB with a well-established therapeutic modality, psoralen ultraviolet A (PUVA), and the second study was conducted to find out whether psoralen might add to its efficacy. METHODS: In the first study, 15 patients were exposed on the left half of their body to UVB 311 nm and then exposed on their right half to UVA after ingestion of psoralen. In the second study, 20 patients were exposed to UVB 311 nm on the left side of the body, followed by ingestion of psoralen and exposure to NB UVB 311 nm 90 min later to the right side of the body. In both studies, while exposing one side, the other was protected by an UV-proof gown. Thus two right-left comparative studies were carried out simultaneously, namely: UVB 311 nm vs. PUVA and UVB 311 nm vs. PUVB 311 nm. RESULTS: In the first study, comparison of PUVA and NB UVB 311 nm showed no difference either in the degree of response or in the incidence of complications. In the second study, comparison of PUVB and UVB showed equal clinical improvement on both sides. The cumulative dose needed to achieve the same response on the PUVB side was lower than that on the UVB side, but the difference was not statistically significant. The incidence of phototoxic reactions was significantly higher on the PUVB treated body half. CONCLUSION: NB UVB 311 nm has similar repigmentary effects as PUVA. The addition of psoralen does not increase its efficacy.

Different low doses of broad-band UVA in the treatment of morphea and systemic sclerosis.

BACKGROUND: Numerous treatment modalities, some with potentially hazardous side effects, are currently used for morphea (M) and systemic sclerosis (SS) with limited success. Low-dose ultraviolet A (UVA) phototherapy (20 J/cm(2)) was found to be highly effective for sclerotic patches, even in patients with advanced and rapidly evolving lesions. OBJECTIVE: To determine the effectiveness of different low doses of UVA in treating patients with M and SS. METHODS: Sixty-three patients complaining of M and 15 patients complaining of SS received 20 sessions of UVA (320-400 nm) each. Patients were divided randomly into three groups that received 5, 10 and 20 J/cm(2), with cumulative UVA doses of 100, 200, and 400 J/cm(2), respectively. The efficacy of therapy was judged clinically (by sequential inspection and palpation) and histopathologically by morphometry in M cases. RESULTS: Obvious clinical improvement, with no comparable differences between various low UVA doses, was noted in patients with M and SS, accompanied by histopathological changes towards normalization of collagen. CONCLUSIONS: After 20 sessions, it appears that lower doses of UVA (5, 10 J/cm(2)) are as beneficial as the relatively higher dose (20 J/cm(2)) in the treatment of M and SS.

Suggested mechanisms of action of UVA phototherapy in morphea: a molecular study.

BACKGROUND: Ultraviolet A (UVA) phototherapy proved to be an efficient line of treatment of scleroderma. The mechanism through which it acts is still not clear. OBJECTIVES: To detect the mechanism of action of UVA phototherapy in morphea through measuring its effect on the levels of different parameters related to collagen metabolism. METHODS: Twenty-one cases of morphea were treated with low-dose broad-band UVA for 20 sessions. Twelve cases received 20 J/cm(2)/session with a cumulative dose of 400 J/cm(2) and nine cases received 10 J/cm(2)/session with a cumulative dose of 200 J/cm(2). The response was assessed clinically every week. Two skin biopsies were taken from the lesional skin of each patient before starting and after the end of therapy. Paraffin sections were examined for quantitative polymerase chain reaction measurement of collagen I, collagen III, collagenase, transforming growth factor-beta (TGF-beta) and interferon gamma (IFNgamma). RESULTS: Eighteen patients reported remarkable softening of the skin lesions, with variable degrees ranging from moderate in 57.1% of them good in 19% to very good response in 9.5%. After treatment, all the studied parameters revealed statistically significant changes. There was a significant decrease in collagen I, collagen III and TGF-beta and a significant increase in collagenase (MMP-1) and IFNgamma. The relative change was found to be greatest in collagenase, followed by IFNgamma then TGF-beta and finally collagen I. The changes in collagen I, collagenase, IFNgamma and TGF-beta were found to increase gradually with the degree of clinical response. In all the parameters studied the relative change was significantly higher in cases treated with 20 J/cm(2)/session in contrast to those treated with 10 J/cm(2)/session although no statistically significant difference could be detected in the clinical response to those doses. CONCLUSIONS: The efficacy of low-dose UVA phototherapy in the treatment of localized scleroderma is mainly obtained by the increased production of MMP-1 and IFNgamma, and to a lesser extent by decreasing TGF-beta and collagen production. Concerning the use of 10 or 20 J/cm(2)/session those effects are dose dependent, but the clinical response does not significantly differ.

PUVA and PUVB in vitiligo--are they equally effective?

BACKGROUND/AIMS: The combination of psoralens with different types of ultraviolet (UVL) sources in the treatment of vitiligo has led to different reports of success. The purpose of this trial is to compare in a random right-left comparison study the efficacy and side effects of oral 8-MOP plus UVA (PUVA) and oral 8-MOP plus UVB (broadband, 290-320 nm P-UVB) in the treatment of vitiligo. METHODS: The study included 24 cases of extensive vitiligo involving more than 30% of the body surface area in a bilateral symmetrical distribution. Each patient received 0.7 mg/kg 8-MOP orally 2 h before the light session. The right side of the body was exposed to UVA (320-400 nm), while the left half was exposed to UVB (290-320 nm). The patients received 3 sessions/week for a total of 30 sessions. RESULTS: Both PUVA and PUVB produced moderate (50-60%) improvement, with similar incidences of phototoxic reaction and skin thickening. However, the study revealed a significant difference in the number of sessions needed to improve produce erythema and perifollicular pigmentation as well as a moderate response, the response on the UVA side always being earlier. Furthermore, the amount of joules needed to achieve the same response was 10 times greater on the UVA side than on the UVB side. CONCLUSION: The use of psoralen plus broadband UVB is as effective as PUVA in the treatment of vitiligo. However, the long-term side effects of psoralen plus UVB are unknown.

Role of the free radical release process in the pathogenesis of morphea in contrast to systemic sclerosis.

Abstract The objective of this study was to assess the importance of the free radical release process in the pathogenesis of localized scleroderma and compare it with that in systemic sclerosis. The study was conducted on 20 randomly collected cases of morphea (4 single plaque, 7 linear, and 9 disseminated), 16 cases of systemic sclerosis, and 10 age- and sex-matched healthy volunteers. Blood samples and homogenized skin biopsies from lesional and nonlesional skin of patients and controls were examined for superoxide dismutase (SOD) activity using spectrophotometric assay, and for lipid peroxide level using the thiobarbituric acid assay. Morphea and systemic sclerosis cases showed significant elevation of blood, lesional, and nonlesional skin lipid peroxide levels and SOD activity compared with normal controls. When each of the subtypes of morphea were compared with the controls, a significant elevation of SOD was found in lesional skin in all groups, in plasma of linear and disseminated morphea, and in nonlesional skin of cases of disseminated morphea. A comparison of systemic sclerosis and morphea cases revealed no significant differences in blood or tissue SOD activity or lipid peroxide level. In both groups, the degree of skin induration could be correlated with changes in lesional SOD activity and lipid peroxide levels, respectively, but no correlation could be found between SOD or lipid peroxide and antinuclear antibody titer. The free radical release process is as important in the pathogenesis of morphea as it is in systemic sclerosis, where it appears to be involved in the development of skin induration.