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Dupuytren's Contracture

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  • Dupuytren's contracture

    Dupuytren's contracture (also known as morbus Dupuytren, or Dupuytren's disease and slang terms "Viking disease" or "Celtic hand), is a fixed flexion contracture of the hand due to a palmar fibromatosis, where the fingers bend towards the palm and cannot be fully extended (straightened). It is an inherited proliferative connective tissue disorder that involves the hand's palmar fascia. It is named after Baron Guillaume Dupuytren, the surgeon who described an operation to correct the affliction in the Lancet in 1831. The ring finger and little finger are the fingers most commonly affected. The middle finger may be affected in advanced cases, but the index finger and the thumb are not affected as frequently. Dupuytren's contracture progresses slowly and is often accompanied by some aching and itching. In patients with this condition, the palmar fascia thickens and shortens so that the tendons connected to the fingers cannot move freely. The palmar fascia becomes hyperplastic and contracts. Incidence increases after age 40; at this age, men are affected more often than women. Beyond 80 the gender distribution is about even. In Dupuytren's contracture, the palmar fascia within the hand becomes abnormally thick, which can cause the fingers to curl and can impair finger function. The main function of the palmar fascia is to increase grip strength, thus Dupuytren's contracture decreases patients' ability to hold objects. Patients often report pain, aching and itching. Those patients report pain along with the contractions. Dupuytren's disease often starts with nodules in the palm of the hand and it can extend to a cord in a finger. Suspected causes of Dupuytren's contracture include trauma, diabetes, epilepsy and therapy with phenytoin. No proven evidence links hand injuries or specific occupational exposures to a higher risk of developing Dupuytren’s. Some speculation links the condition or its onset may be triggered by, physical trauma such as sustained manual labor or over-exertion of the hands. However, the fact that Dupuytren's is not connected with handedness undermines this claim. The causative agents or the main risk factors of this disease are: smoking, alcohol consumption, frozen shoulder, epilepsy, diabetes mellitus, carpal tunnel syndrome, history of manual labor, and hand injury. All of these reports are controversial and frequently delivered based on selective data.

  • Dupuytren's contracture

    The prevalence of DD in all studies increased with age with a male to female ratio of approximately 5.9:1. Prevalence rates ranged from 0.2% to 56% in varying age, population groups, and methods of data collection. The highest prevalence rate was reported in a study group of epileptic patients. Although, only one study calculated the incidence (as opposed to prevalence) of DD to be equal to 34.3 per 100,000 men (0.03%). The prevalence of DD in different geographical locations is extremely variable, and it is not clear whether this is genetic, environmental, or a combination of both. The majority of the prevalence studies have been conducted in Scandinavia or the UK, and the vast changes in population structure, the changes in prevalence of associated diseases, and the change in diagnostic criteria of DD makes understanding the epidemiology of this condition difficult. The highest prevalence of this disease is in northern European countries. Of the 32,000 men and women (16,000 of each gender) aged 18 years and older who were randomly selected from the pool of KN panel members and invited to participate in phase 1, a total of 23,103 completed the screening survey for a response rate of 72%. Over 400 (n = 463) Dupuytren’s patients (197 of them reported a Dupuytren’s diagnosis and/or surgical/needle aponeurotomy treatment) were invited to complete a full survey in phase 2 of those, 376 (81%) responded to the survey invitation. Of the responders, 326 (87%) consented and completed the Dupuytren’s disease survey. Treatment involves one or more different types of treatment with some hands needing repeated treatment. Treatment can be of two types: i. Surgical ii. Non-surgical Surgical: Limited Fasciectomy, Wide-Awake Fasciectomy, Dermofasciectomy, Free Vascular Flaps. Minimally-Invasive Surgery: Segmental Fasciectomy with/without Cellulose, Percutaneous Needle Fasciotomy, Extensive Percutaneous Aponeurotomy and Lipografting. Non-surgical: Collagenase. Other Alternative Therapies are: i. Laser treatment: Low power red and infrared rays. ii. ii. Diet supplements: Quercetin, Bromelain, DMSO, Methylsulfonylmethan, Acetylcarnitine Hcl, Para amino benzoic acid, Nattokinase, Vitamin E, Copper, Vitamin C. The main categories listed by the International Dupuytren Society in order of stage of disease are Radiation Therapy, Needle Aponeurotomy (NA), Collagenase Injection (Xiaflex) and Hand Surgery. Radiation Therapy Dupuytren’s may be treated at the nodules, cords and early finger deformation stages with Radiation therapy. The effects of radiation treatment in the British Journal of Radiology in 1955. In Germany and parts of the U.S., radiotherapy is one of the main treatments.

  • Dupuytren's contracture

    The results of fractionated radiation therapy were published in studies in 1996 and 2001. In a 2012 study, Seegenschmiedt et al. showed comparisons with a control group. Shows the beam's eye view of the radiotherapy portal on the hand's surface with the lead shield cut-out placed in the machine's gantry. Dupuytrens radiotherapy treatment from Prof Seegenschmiedt, Strahlenzentrum Hamburg Radiotherapy has been reported to be effective for prevention of disease progression in early stages with only mild acute or late side effects. The effect of radiation therapy on a long-term outcome was evaluated by Betz et al. They conducted a follow up evaluation 13 years later for patients receiving radiation therapy. Treatment toxicity and objective symptom reduction in terms of stage change and numbers of nodules and cords were assessed. They concluded that radiotherapy is effective in prevention of disease progression and improves patients' symptoms in stage N, N/I. Seegenschmiedt, a german scientist stated that radiotherapy is an early stage treatment in which finger deformation should be 10 degrees or less. The most preferable state would be no deformation, with the hand diagnosed as an "active" state, in which nodules and cords are changing. During diagnosis the feet are checked for Ledderhose disease. The nodules and cords are irradiated for five days in a row with a dose of 3Gy fractions per day, totaling 15Gy for the week and is repeated after 12 weeks. X-Ray and more recently E-beam radiation are used. The purpose of radiotherapy is to stop disease progression. It has a documented success rate of 85%. The cell responsible for the disease is the myofibroblast. Liaquat S. Verjee et al. excised myofibroblasts from patients with the disease to explore the signaling pathways for their formation. They found significant numbers of immune cells, including classically activated macrophages and high levels of proinflammatory cytokines. They compared the effects of these cytokines on contraction and profibrotic signaling pathways in fibroblasts from the palmar and nonpalmar dermis of patients and palmar fibroblasts from controls. Addition of tumor necrosis factors (TNF), but not other cytokines, promoted differentiation of fibroblasts into myofibroblasts in Dupuytren's patients. Neutralizing antibodies to TNF inhibited the contractile activity of myofibroblasts, reduced their expression of α-smooth muscle actin, and mediated disassembly of the contractile apparatus.

 

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