Zinc in sickle cell disease: A narrative review.

Sickle cell disease (SCD) is an inherited disease caused by hemoglobin S mutated hemoglobin S. It is characterized by chronic hemolysis, intermittent vaso-occlusive crises followed by ischemia-reperfusion, and organ damage. These patients have an increased risk of multiple micronutrient deficiencies, such as zinc. The reduced zinc bioavailability in sickle cell patients may lead to several complications such as growth retardation, delayed wound healing, increased vaso-occlusive crises, and infections. This narrative review aims to analyze the literature concerning the zinc status in SCD and their possible consequences on the patients' clinical evolution. We found in children and adolescents a direct association between zinc insufficiencies/deficiencies with increased disease severity in SCD. Monitoring zinc status in children and adolescent SCD appears essential for reducing disease-associated morbidity and infections. Zinc supplementation is a safe therapeutic modality for treating SCD patients. New research must be carried out, especially for adults, to ensure more remarkable survival for this population.

[Treatment of chronic itch in systemic disease. Current standards]. / Therapie des Pruritus bei inneren Erkrankungen. Was ist gesichert?

Chronic itch (CI) is a frequent and sometimes tormenting symptom in many skin and systemic diseases. In systemic diseases, it mostly appears on primarily unaffected skin. As a sequelae of intense scratching, secondary skin lesions such as excoriations, scars, and prurigo nodularis may occur. Due to the lack of valid pathogenetic concepts and good clinical trials, the therapy of CI remains mostly symptomatic. In Europe almost all drugs used to treat CI are not approved for this indication. CI is frequent in patients with chronic kidney diseases in advanced stages. Gabapentin and pregabalin, anticonvulsants, and centrally acting calcium channel blockers have been shown to exert a profound effect in CI. Furthermore, UVB phototherapy has been proven to attenuate pruritus in uremic patients. Randomized controlled studies have recently shown that nalfurafine, a κ-opioid receptor agonist, is able to ameliorate itch in patients with uremic itch. In patients suffering from cholestatic itch, the anion exchange resin colestyramine and rifampicin are effective antipruritic drugs. Furthermore, µ-opioid receptor antagonists and sertraline may be used to alleviate CI in hepatic diseases. In refractory cases, naso-biliary drainage or albumin dialysis are effective invasive procedures. For the treatment of chronic itch in hematological diseases no controlled trials have been performed so far. The mainstay in these cases is to treat the underlying disease.

Iron deposits in the knee joints of a thalassemic patient.

The overall prognosis for patients with ß-thalassemia has improved considerably during the past decades mainly due to regular blood transfusions, improvements in chelation therapy, and enhanced surveillance with imaging studies examining iron overload and other clinical complications. However, the prolonged survival of these patients leads to the development of other health problems including degenerative diseases such as arthropathies, which require further attention since they have a significant impact on the quality of life. In the current case report, we present a 45-year-old white man with ß-thalassemia complaining of non-traumatic pain and restriction in the range of motion of both knees. Magnetic resonance imaging (MRI) revealed a tear in the medial meniscus of the left knee as well as iron deposits in both knees. Histological findings confirmed the presence of hemosiderin in both joints. To our knowledge, this is the first reported case of macroscopically documented iron deposits in the knee joints of a patient with ß-thalassemia using MRI.

Use of complementary and alternative medicine by patients with hematological diseases: experience at a university hospital in northeast Mexico

BACKGROUND: Complementary and alternative medicine includes a diverse group of medical and healthcare systems, practices and products not considered part of conventional medicine. Although there is information on unconventional practices in oncological diseases, specific data regarding the use of complementary and alternative medicine by hematology patients is scarce. OBJECTIVE: The aim of this study is to document the prevalence of this modality of unconventional therapy in patients with malignant and benign hematological diseases, particularly children with acute lymphoblastic leukemia. METHODS: An observational study of adult patients and guardians of children with malignant or benign hematological diseases was carried out by applying a structured questionnaire detailing the use and results of the most prevalent complementary and alternative medicine practices. RESULTS: One hundred and twenty patients were included; 104 had malignant and 16 had benign hematological diseases. The use of complementary and alternative medicine was greater in benign diseases but the difference was not statistically significant (64.7% versus 41.7%; p-value = 0.08). Patients and guardians with high school or college educations used these alternative practices more than patients with less schooling (60.7% versus 54.7%; p-value = 0.032). The use of folk remedies was most prevalent followed by herbal preparations and spiritual healing. Sixty-four percent of patients that used these unconventional practices reported improvement in their symptoms and increased capacity to perform daily activities. CONCLUSION: No significant difference was documented between patients with malignant or benign hematological diseases using these alternative practices. The majority of complementary and alternative medicine users reported improvement of the disease or chemotherapy-related symptoms.