Impact of genotype on multi-organ iron and complications in patients with non-transfusion-dependent ß-thalassemia intermedia.

We evaluated the impact of the genotype on clinical and hematochemical features, hepatic and cardiac iron levels, and endocrine, hepatic, and cardiovascular complications in non-transfusion-dependent (NTD) ß-thalassemia intermedia (TI) patients. Sixty patients (39.09 ± 11.11 years, 29 females) consecutively enrolled in the Myocardial Iron Overload in Thalassemia project underwent Magnetic Resonance Imaging to quantify iron overload, biventricular function parameters, and atrial areas and to detect replacement myocardial fibrosis. Three groups of patients were identified: homozygous ß+ (N = 18), heterozygous ß0ß+ (N = 22), and homozygous ß0 (N = 20). The groups were homogeneous for sex, age, splenectomy, hematochemical parameters, chelation therapy, and iron levels. The homozygous ß° genotype was associated with significantly higher biventricular end-diastolic and end-systolic volume indexes and bi-atrial area indexes. No difference was detected in biventricular ejection fractions or myocardial fibrosis. Extramedullary hematopoiesis and leg ulcers were significantly more frequent in the homozygous ß° group compared to the homozygous ß+ group. No association was detected between genotype and liver cirrhosis, hypogonadism, hypothyroidism, osteoporosis, heart failure, arrhythmias, and pulmonary hypertension. Heart remodelling related to a high cardiac output state cardiomyopathy, extramedullary hematopoiesis, and leg ulcers were more pronounced in patients with the homozygous ß° genotype compared to the other genotypes analyzed. The knowledge of the genotype can assist in the clinical management of NTD ß-TI patients.

[Chronic wounds of the lower limbs: a guide for non-specialists]. / Plaies chroniques des membres inférieurs : un guide pour le non-spécialiste.

Chronic lower-extremity ulcers are a growing public health problem, resulting in significant costs for society and patients, and having a significant impact on the quality of life of patients and informal caregivers. As general practitioners are often solicited early on, the acquisition of basic knowledge regarding wound care management is therefore essential to initiate local care, to make an early diagnosis and identify emergencies and patients that need a referral. The CASE and TIMERS frameworks enable a holistic assessment of the patient and the wound, to propose a treatment of the wound based on its etiology combined with appropriate local wound care. This framework allows also to identify atypical, severe, or recalcitrant wounds requiring specialized advice.

Les plaies chroniques des membres inférieurs sont un problème grandissant de santé publique, occasionnant des dépenses conséquentes et entraînant une répercussion non négligeable sur la qualité de vie des patients et de leurs proches aidants. Les médecins de premier recours étant le plus souvent les premiers intervenants, l'acquisition d'un socle commun de connaissances est donc essentielle pour la bonne prise en soin initiale des plaies chroniques, obtenir un diagnostic précoce et identifier les urgences et les patients à référer. L'approche selon les principes CASE et TIMERS permet une évaluation holistique du patient et de sa plaie, et de proposer un traitement étiologique associé à des soins locaux adaptés. Celle-ci permet également d'identifier les plaies atypiques, sévères ou récalcitrantes, nécessitant un avis spécialisé.

Efficacy of maggot debridement therapy on refractory leg ulcers of Behçet disease: an open-label study.

BACKGROUND: Cutaneous ulcers of Behçet disease (BD) are rare but have high morbidity and resistance to conventional therapies. An important and essential aspect of ulcer management is debridement. Regarding maggot therapy (MT), excretions of the green bottle fly, Lucilia sericata, have been shown to have the ability to remove necrotic debris and promote healing. AIM: To evaluate the efficacy of MT for cutaneous ulcers of BD. METHODS: In this open-label trial, patients with BD with refractory leg ulcers suitable for MT were enrolled. Maggot application was performed until complete debridement was achieved, and all patients were followed up for 12 months afterwards to assess the total healing of ulcers. RESULTS: In total, 24 patients with 32 ulcers were enrolled. Using MT, 91.6% of all ulcers were completely debrided. Mean time to debridement was 14.9 days and mean number of cycles required was 5.3. Mean ulcer size was decreased by 23% with treatment. Time to debridement was positively correlated with pretreatment ulcer size and ulcer duration (P = 0.01 and P < 0.01) but not with ulcer depth, comorbidities, smoking, age or sex (P > 0.05 for all). During follow-up, 79.1% of all ulcers healed completely. Mean time required for total healing was positively correlated with ulcer duration, pretreatment and post-treatment ulcer area, ulcer depth and mean time to total debridement (P < 0.03, P = 0.00, P = 0.04 and P < 0.01, respectively). CONCLUSIONS: To our knowledge, the findings presented in this first and unique study may provide key answers about factors affecting success rate of MT in BD cutaneous ulcers.

Calciphylaxis in a renal transplant recipient with normal graft function: A case report and review of literature.

Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare, life-threatening complication of end-stage renal disease. However, it may also occur in patients without renal failure, the nonuremic calciphylaxis. Most patients present with painful skin ulcers. Delayed diagnosis can lead to sepsis-related morbidity and mortality. Aberrations in calcium, phosphate, and parathyroid hormone physiology are common and biopsy is diagnostic. Early diagnosis and correction of vascular and metabolic aberrations may assist in healing as may the use of sodium thiosulfate and hyperbaric oxygen therapy. We report on successful treatment of a case of calciphylaxis in a renal transplant recipient with normal allograft function.

SICKLE as a holistic treatment approach to sickle cell disease related ulcers.

This article introduces a simple approach to adopt in the treatment of sickle cell disease (SCD) related ulcerations based on the acronym SICKLE, standing for Skin assessment, Infection control, Compression, Keep moving keep debriding, Local strapping and Endless support. This case report explains and describes each step used in the treatment of this challenging disease in a 24-year-old patient, achieving complete healing in 6 months in long-lasting SCD ulceration previously treated without results with many other treatments. It has been replicated in further patients with excellent results. The SICKLE approach is an attempt to deliver a holistic way of treating patients with SCD-related leg ulcers. More studies are needed to better define the scientific validity of this method or to demonstrate any inconsistencies.

A Rare Cause of Leg Ulcer: Calcinosis Cutis as a Part of CREST Syndrome.

Leg ulcers are not a disease themselves, they are a symptom of a disease. To manage them properly, finding the correct diagnosis of the disease is mandatory. Our case is a model to underline that leg ulcers are a significant burden for the patient, because leg ulcer got ahead of the patient's other serious complaints such as Raynaud's phenomenon or sclerodactyly. Furthermore, our patient is also a model, and an attentive clinical evaluation is inevitable to find the correct diagnosis. We present our case aiming to underline the significance of full dermatologic examination in each ulceration to discover underlying disease.

Phototherapy with LED Shows Promising Results in Healing Chronic Wounds in Diabetes Mellitus Patients: A Prospective Randomized Double-Blind Study.

OBJECTIVE: The study examined the influence of phototherapy with light-emitting diodes (LEDs) on chronic diabetic wound healing. BACKGROUND: Chronic diabetic wounds are very difficult to treat due to underlying conditions such as angiopathy and neuropathy, resulting in slow healing rates. Conventional treatment options are often insufficient and do not provide satisfactory outcomes. Phototherapy with LED enhances the healing processes through mechanisms of energy exchange between incoming photons and their target, the main one being cytochrome-c oxidase in mitochondria. METHODS: A double-blind, randomized study included 60 patients with a chronic diabetic wound treated at the University Medical Center Ljubljana between October 1, 2012 and December 1, 2014. Patients were randomized into either an active group (LED group) or a control group (Co-group). The active group was treated with LED 2.4 J/cm2 (wavelengths 625, 660, 850 nm) three times a week for 8 weeks. The Co-group was treated with light that simulated LED. Healing was evaluated using the Falanga wound bed score and wound surface area. RESULTS: The average baseline wound surface before treatment was 1315 mm2 in the LED group and 1584 mm2 in the Co-group (p = 0.80). After 8 weeks, the mean surface in the LED group was 56% of the baseline surface and 65% in the Co-group (p > 0.05). Falanga score evaluation showed significantly faster wound bed healing in the LED group compared with the Co-group (p < 0.05). CONCLUSIONS: According to our results, LED significantly improves healing of chronic diabetic wounds and prepares the wound bed for further coverage options.

Hyperbaric oxygen therapy for chronic ulcers in systemic sclerosis - case series.

BACKGROUND: Digital and leg ulcers are extremely painful, hard to heal manifestations of systemic sclerosis (SSc). Many treatments have been tried for these challenging complications, but use of hyperbaric oxygen therapy (HBOT) is very limited. HBOT has been used as an adjunctive therapy for treating chronic wounds, and nonhealing SSc wounds, which have a hypoxic nature, may also benefit from it. METHODS: This is a retrospective analysis of six SSc patients who underwent HBOT for their ulcers. Patient demographics, ulcer properties, and details of treatments were evaluated. RESULTS: Three patients had digital ulcers, and the other three had leg ulcers. Three patients (two leg ulcers and one digital ulcer) had bilateral lesions. All patients had been treated for at least one and a half months with various modalities. After HBOT was applied, four patients' ulcers healed completely and two patients had near-complete healing. Amputation was not required for any. CONCLUSION: This case series, which has the largest patient population up to present, shows adjunctive HBOT may be helpful in the treatment of SSc ulcers.

[Leg ulcers in sickle cell disease: A retrospective study of 40 cases]. / Ulcères de jambe au cours de la drépanocytose: étude rétrospective de 40 cas.

BACKGROUND: In Africa, studies primarily devoted to chronic leg ulcer due to sickle cell disease are rare. The objectives of the study were to determine the epidemiology, diagnosis and progression of chronic leg ulcers in sickle cell disease. PATIENTS AND METHODS: A 5-year multicentre, retrospective study was conducted in three university hospitals in Dakar. We included all patients with chronic leg ulcers occurring in a setting of sickle cell disease. RESULTS: We identified 40 cases of chronic leg ulcers associated with sickle cell disease, representing 3.4% of the current population of sickle cell patients in our institutions. The average patient age was 25.9 years and the sex ratio was 2.33. Chronic leg ulcer was the presenting feature enabling diagnosis of sickle cell disease in one third of the cases. The average time to consultation from onset was 5.4 years. Pain was reported in 22 cases (48%). Ulcers were isolated in 76% and multiple in 24% of cases. The most common site was the medial malleolus (39%). A CBC allowed identification of anaemia in 35 cases. Haemoglobin electrophoresis was performed and homozygous sickle cell SS disease was identified in 39 cases and heterozygous SC disease in 1 case. Local treatments included physiologic serum, topical antibiotics and skin grafting. Systemic treatment included supplementation with folic acid in all patients, blood transfusion in 16 cases, vasodilators in 11 cases and antibiotics in 25 cases. The outcome was favourable in 61.8% of cases. DISCUSSION: In Dakar, sickle cell disease is a common cause of chronic leg ulcer and is frequently revealed by chronic leg ulcer.

Critical Reviews: How we treat sickle cell patients with leg ulcers.

The past five decades have seen an improvement in the mortality and morbidity of sickle cell disease (SCD) because of prophylaxis against infectious complications, improved and expanded red cell transfusions, implementation of hydroxyurea therapy, and advances in supportive care. Now that the majority of patients in the western hemisphere reaches adulthood, end organ diseases are frequent, which include vasculopathic complications such as chronic leg ulcers. The management of patients with leg ulcers requires the hematologist to lead a team of health care professionals, and investigates the presence of associated, but potentially still occult signs of vasculopathy, such as pulmonary hypertension, renal disease, priapism and retinopathy. These complications may be asynchronous, and long term careful screening is indicated, in order to ensure early diagnosis and intervention. It is crucial to address both the immediate consequences of pain, infection and disability, and long term effects on quality of life, employment and stigma associated with chronic ulceration. Recent insights into their pathophysiology may have practical implications. We propose a holistic approach to the management of patients' physical and emotional problems and mechanisms of ulcers formation and delayed healing. An overview of topical and systemic therapies for chronic ulcers is given, with the understanding that wound care therapy is best left to the wound specialists, medical and surgical, with whom the hematologist must keep an open line of communication. In the absence of evidence-based guidelines, our opinion is based on both a critical review of the literature and our personal clinical and research experience.

Successful Management of Severe Unilateral Lower Extremity Lymphedema in an Outpatient Setting.

BACKGROUND AND PURPOSE: Non-cancer-related lymphedema is frequently unrecognized until it has advanced and, therefore, presents substantial management challenges. Treatment for lymphedema reflects cancer research and not the distinct complexities of non-cancer-related lymphedema. This case report describes an outpatient intervention for a patient with non-cancer-related unilateral lower extremity lymphedema. CASE DESCRIPTION: The patient was a 42-year-old woman who was morbidly obese and had left lower extremity chronic stage III lymphedema. Massive disfiguring lymphedema of her lower leg caused chronic wounds, pain, difficulty ambulating, and the inability to lift her leg in transfers. The patient received complete decongestive therapy (CDT) in an outpatient setting. OUTCOMES: The volume of the patient's leg decreased more than 66%, so that her massive lower leg was transformed to nearly the same size as her unaffected limb. Proper compression management restored independent transfers and pain-free ambulation. DISCUSSION: Delayed diagnosis and treatment of non-cancer-related lymphedema result in increased severity and impaired mobility, making this a unique, complex condition requiring modifications to CDT in the outpatient setting. This case report demonstrates successful outpatient treatment of a patient with severe unilateral lower extremity lymphedema associated with large wounds and impaired mobility using CDT that was modified to accommodate her complex medical status, transportation barriers, and health insurance availability. Following 23 weeks of physical therapist interventions, the patient's affected lower extremity was the same size as the unaffected lower extremity, and she was independent in ambulation and stair climbing.

An introduction and guide to effective Doppler assessment.

Accurate and timely diagnosis of leg ulceration is an essential factor in making evidence-based, effective decisions regarding patient management with the aim of swift wound healing and/or referral to the appropriate specialty. Nurses are professionally responsible for ensuring that patients receive the appropriate assessment and evidence-based management. This article examines the most up-to-date guidance on Doppler ultrasound as a key element of this assessment. Approaches to assessment will be explored, with emphasis on the need to include a Doppler ultrasound as one key element of a larger, holistic assessment. An introduction to the ankle-brachial pressure index (ABPI) will be given, followed by a step-by-step guide to standard procedures for carrying out a full Doppler ultrasound. Alternative options for measuring ABPI are also provided.

Role of hyperbaric medicine for intractable leg ulcers: a case report.

We present a case report of intractable multifactorial leg ulcer that was treated successfully with multiple approaches including hyperbaric oxygen treatment (HBOT) to prepare for skin grafting. A 66-year-old female with a history of rheumatoid arthritis and Felty's syndrome presented with a non-healing ulcer on her left leg that was caused by a trauma. She failed multiple treatment options including debridement, different wound dressings, antibiotics, anti-inflammatories and vein closure procedure. She finally healed with skin graft following HBOT that prepared the wound bed before the procedure.

Arterial disease ulcers, part 2: treatment.

OBJECTIVES: The objectives of this article are to describe the therapeutic options available and develop an appropriate clinical approach suitable to the individual needs of the patient with arterial insufficiency, based on the wound bed preparation paradigm. This information will also assist in the integration of decision making regarding appropriate clinical intervention in an interprofessional team approach, according to the International Inter-professional Wound Caring Model 2012, with inclusion of patient-centered concerns within the patient's circle of care.