
| 1. Am J Med Genet A. 2010 Apr;152A(4):970-6. Lipedema: an inherited condition. Child AH, Gordon KD, Sharpe P, Brice G, Ostergaard P, Jeffery S, Mortimer PS. Department of Cardiac and Vascular Sciences, St. George's, University of London, London, UK. achild@sgul.ac.uk Abstract Lipedema is a condition characterized by swelling and enlargement of the lower limbs due to abnormal deposition of subcutaneous fat. Lipedema is an under-recognized condition, often misdiagnosed as lymphedema or dismissed as simple obesity. We present a series of pedigrees and propose that lipedema is a genetic condition with either X-linked dominant inheritance or more likely, autosomal dominant inheritance with sex limitation. Lipedema appears to be a condition almost exclusively affecting females, presumably estrogen-requiring as it usually manifests at puberty. Lipedema is an entity distinct from obesity, but may be wrongly diagnosed as primary obesity, due to clinical overlap. The phenotype suggests a condition distinct from obesity and associated with pain, tenderness, and easy bruising in affected areas. (c) 2010 Wiley-Liss, Inc. PMID: 20358611 [PubMed - in process] --- 1. J Cancer Surviv. 2010 Apr 7. [Epub ahead of print] Upper extremity impairments in women with or without lymphedema following breast cancer treatment. Smoot B, Wong J, Cooper B, Wanek L, Topp K, Byl N, Dodd M. Department of Physical Therapy and Rehabilitation Science, University of California San Francisco, San Francisco, CA, USA, betty.smoot@ucsf.edu. Abstract INTRODUCTION: Breast-cancer-related lymphedema affects approximately 25% of breast cancer (BC) survivors and may impact use of the upper limb during activity. The purpose of this study is to compare upper extremity (UE) impairment and activity between women with and without lymphedema after BC treatment. METHODS: 144 women post BC treatment completed demographic, symptom, and Disability of Arm-Shoulder-Hand (DASH) questionnaires. Objective measures included Purdue pegboard, finger-tapper, Semmes-Weinstein monofilaments, vibration perception threshold, strength, range of motion (ROM), and volume. RESULTS: Women with lymphedema had more lymph nodes removed (p < .001), more UE symptoms (p < .001), higher BMI (p = .041), and higher DASH scores (greater limitation) (p < .001). For all participants there was less strength (elbow flexion, wrist flexion, grip), less shoulder ROM, and decreased sensation at the medial upper arm (p < .05) in the affected UE. These differences were greater in women with lymphedema, particularly in shoulder abduction ROM (p < .05). Women with lymphedema had bilaterally less elbow flexion strength and shoulder ROM (p < .05). Past diagnosis of lymphedema, grip strength, shoulder abduction ROM, and number of comorbidities contributed to the variance in DASH scores (R (2) of 0.463, p < .001). IMPLICATIONS FOR CANCER SURVIVORS: UE impairments are found in women following treatment for BC. Women with lymphedema have greater UE impairment and limitation in activities than women without. Many of these impairments are amenable to prevention measures or treatment, so early detection by health care providers is essential. PMID: 20373044 [PubMed - as supplied by publisher] 2. Support Care Cancer. 2010 Apr 6. [Epub ahead of print] Can ICF model for patients with breast-cancer-related lymphedema predict quality of life? Tsauo JY, Hung HC, Tsai HJ, Huang CS. -------------------- 1: Ridner SH, Dietrich MS, Kidd N RelatedArticles Breast cancer treatment-related lymphedema self-care: Education, practices, symptoms, and quality of life. Support Care Cancer. 2010 Apr 15. PMID: 20393753 [PubMed - Publisher] School and Graduate Institute of Physical Therapy, College of Medicine, National Taiwan University, Taipei, Taiwan. Abstract GOAL OF WORK: The aim of the study was to investigate if the International Classification of Functioning, Disability and Health (ICF) model with clinical data from patients with breast-cancer-related lymphedema can predict their health-related quality of life (HRQL). MATERIALS AND METHODS: Sixty-one patients with breast-cancer-related lymphedema were recruited. Data were collected from records, including age, type(s) of surgery, number of dissected lymph nodes and history of radiotherapy and/or chemotherapy, duration of lymphedema, and duration between surgery and enrollment. Excessive arm volume, average arm symptom, function of upper extremity (U/E), and HRQL were assessed four times during and after patients' treatment of lymphedema. RESULTS: The ICF model accounted for 20.5% to 55.6% variance in each domain of HRQL. Activity and participation reflected by U/E function were the most important factor, significantly predicting every domain of HRQL. Among measured impairments, average arm symptom was found to be most correlated with U/E function (r = 0.590, P < 0.05). CONCLUSION: The ICF model consisting of clinical measures for patients with breast-cancer-related lymphedema can predict their HRQL. Activity and participation were the most important component. Arm symptoms rather than arm volume significantly correlated with U/E function. This might suggest that reducing arm symptoms is relatively more important while treating patients with breast-cancer-related lymphedema. PMID: 20372972 [PubMed - as supplied by publisher] 3. Indian J Plast Surg. 2009 Jul;42(2):248-50. Lymphangiectasis of lower limb: A rare challenging case. Bhattacharya V, Mishra B, Barooah PS, Chaudhuri GR, Bhattacharya S. Department of Plastic Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005, U.P, India. Abstract Lymphangiectasis usually occurs in the viscera. Involvement of the lower limb is very rare. It is difficult to establish the diagnosis without detailed investigations. Clinical features are peculiar and may mimic lymphoedema of different origins which needs to be ruled out. Contrary to the expectation, the post-operative result is excellent in the long-term follow-up. PMID: 20368868 [PubMed - in process] 4. J Clin Invest. 2010 Apr 1. pii: 40101. doi: 10.1172/JCI40101. [Epub ahead of print] Direct transcriptional regulation of neuropilin-2 by COUP-TFII modulates multiple steps in murine lymphatic vessel development. Lin FJ, Chen X, Qin J, Hong YK, Tsai MJ, Tsai SY. Abstract The lymphatic system plays a key role in tissue fluid homeostasis. Lymphatic dysfunction contributes to the pathogenesis of many human diseases, including lymphedema and tumor metastasis. However, the mechanisms regulating lymphangiogenesis remain largely unknown. Here, we show that COUP-TFII (also known as Nr2f2), an orphan member of the nuclear receptor superfamily, mediates both developmental and pathological lymphangiogenesis in mice. Conditional ablation of COUP-TFII at an early embryonic stage resulted in failed formation of pre-lymphatic ECs (pre-LECs) and lymphatic vessels. COUP-TFII deficiency at a late developmental stage resulted in loss of LEC identity, gain of blood EC fate, and impaired lymphatic vessel sprouting. siRNA-mediated downregulation of COUP-TFII in cultured primary human LECs demonstrated that the maintenance of lymphatic identity and VEGF-C-induced lymphangiogenic activity, including cell proliferation and migration, are COUP-TFII-dependent and cell-autonomous processes. COUP-TFII enhanced the pro-lymphangiogenic actions of VEGF-C, at least in part by directly stimulating expression of neuropilin-2, a coreceptor for VEGF-C. In addition, COUP-TFII inactivation in a mammary gland mouse tumor model resulted in inhibition of tumor lymphangiogenesis, suggesting that COUP-TFII also regulates neo-lymphangiogenesis in the adult. Thus, COUP-TFII is a critical factor that controls lymphangiogenesis in embryonic development and tumorigenesis in adults. PMID: 20364082 [PubMed - as supplied by publisher ---- 1. PLoS Negl Trop Dis. 2010 Apr 20;4(4):e668. Feasibility and effectiveness of basic lymphedema management in Leogane, Haiti, an area endemic for bancroftian filariasis. Addiss DG, Louis-Charles J, Roberts J, Leconte F, Wendt JM, Milord MD, Lammie PJ, Dreyer G. Division of Parasitic Diseases, National Center for Infectious Diseases, U.S. Centers for Disease Control and Prevention, Atlanta, Georgia, United States of America. daddiss@fetzer.org Abstract BACKGROUND: Approximately 14 million persons living in areas endemic for lymphatic filariasis have lymphedema of the leg. Clinical studies indicate that repeated episodes of bacterial acute dermatolymphangioadenitis (ADLA) lead to progression of lymphedema and that basic lymphedema management, which emphasizes hygiene, skin care, exercise, and leg elevation, can reduce ADLA frequency. However, few studies have prospectively evaluated the effectiveness of basic lymphedema management or assessed the role of compressive bandaging for lymphedema in resource-poor settings. METHODOLOGY/PRINCIPAL FINDINGS: Between 1995 and 1998, we prospectively monitored ADLA incidence and leg volume in 175 persons with lymphedema of the leg who enrolled in a lymphedema clinic in Leogane, Haiti, an area endemic for Wuchereria bancrofti. During the first phase of the study, when a major focus of the program was to reduce leg volume using compression bandages, ADLA incidence was 1.56 episodes per person-year. After March 1997, when hygiene and skin care were systematically emphasized and bandaging discouraged, ADLA incidence decreased to 0.48 episodes per person-year (P<0.0001). ADLA incidence was significantly associated with leg volume, stage of lymphedema, illiteracy, and use of compression bandages. Leg volume decreased in 78% of patients; over the entire study period, this reduction was statistically significant only for legs with stage 2 lymphedema (P = 0.01). CONCLUSIONS/SIGNIFICANCE: Basic lymphedema management, which emphasized hygiene and self-care, was associated with a 69% reduction in ADLA incidence. Use of compression bandages in this setting was associated with an increased risk of ADLA. Basic lymphedema management is feasible and effective in resource-limited areas that are endemic for lymphatic filariasis. PMID: 20422031 [PubMed - in process]PMCID: PMC2857874 2. Support Care Cancer. 2010 Apr 25. [Epub ahead of print] Longitudinal changes in sexual problems related to cancer treatment in Korean breast cancer survivors: a prospective cohort study. Yang EJ, Kim SW, Heo CY, Lim JY. Int J Med Sci. 2010 Apr 15;7(2):68-71. Godoy & Godoy technique in the treatment of lymphedema for under-privileged populations. de Godoy JM, de Godoy Mde F. Stricto-Sensu and Lato-Sensu of Course in Medicine of Medical School in São José do Rio Preto- SP (FAMERP), Brazil. godoyjmp@riopreto.com.br Abstract The aim of this paper is to report new options in the treatment of lymphedema for under-privileged populations. Several articles and books have been published reporting recent advances and contributions. A new technique of manual lymph drainage, mechanisms of compression, development of active and passive exercising apparatuses and the adaptation of myolymphokinetic activities have been developed for the treatment of lymphedema. This novel approach can be adapted for the treatment of lymphedema in mass. PMID: 20428336 [PubMed - in process] Department of Rehabilitation Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital, 300 Gumi-dong Bundang-gu, Seongnam-si, Gyeonggi-do, 463-707, Republic of Korea. Abstract PURPOSE: The aims of the study were to investigate longitudinal changes in multiple domains of problems with sexual functioning in Korean breast cancer survivors in the first year after surgery for breast cancer and to determine which factor(s), including upper limb dysfunction, may influence sexual problems. METHODS: Women diagnosed with breast cancer (n = 191) were initially assessed at baseline before surgery and completed follow-ups at 3, 6, and 12 months after surgery. Survey items included sexual interest, sexual activity, satisfaction with sex life, feeling sexually attractive, body image, medical history, symptoms, upper limb dysfunction, and sociodemographics. RESULTS: The prevalence of sexually active women was 39.2% at 3 months, which increased to 48.2% at 6 months, and 50% at 12 months after surgery. Compared with pretreatment levels, considerably more women reported moderate or severe problems with sexual interest and sexual activity at 3, 6, and 12 months after surgery. Chemotherapy was related to sexual problems only early after treatment, and surgical procedure (extensive vs. conservative) had no significant effect on sexual problems. Low perceived sexual attractiveness in 3 months after surgery was related to greater overall sexual problems. Lymphedema was significantly related to sexual disinterest at all stages of follow-up and to sexual satisfaction at the 6 month follow-up after adjusting for other predicting factors. CONCLUSIONS: Although sexual activity gradually improved during the first year, more women reported moderate or severe problems with sexual interest and activity over time. Upper limb dysfunction, such as that caused by lymphedema, is a significant factor that may interfere with sexual functioning in breast cancer survivors. PMID: 20419495 [PubMed - as supplied by publisher] 3. J Indian Assoc Pediatr Surg. 2009 Oct;14(4):230-1. Saxophone penis due to primary lymphoedema. Jain VK, Singh S, Garge S, Negi A. Department of Surgery, SAIMS, Indore, India. Abstract Congenital lymphoedema is a rare disorder that may result in disfiguring edema of the male genitalia. The treatment of persistent lymphoedema is surgical and consists of meticulous excision of all subcutaneous layers of the affected skin, combined with reconstruction of the penis and or scrotum. PMID: 20419030 [PubMed - in process]PMCID: PMC2858891 4. Indian J Orthop. 2010 Apr;44(2):198-201. One-stage release of congenital constriction band in lower limb from new born to 3 years. Das SP, Sahoo P, Mohanty R, Das S. Swami Vivekananda National Institute of Rehabilitation Training and Research, Olatpur, Bairoi, Cuttack, Orissa-754 010, India. Abstract BACKGROUND: Congenital constriction band is the most common cause of terminal congenital malformation of a limb and lymphoedema. Superficial bands do not need any treatment, but deeper bands are managed with excision and Z-plasty. The circumferential bands are released in two to three stages to prevent vascular compromise. The purpose of this study was to present the outcome of one-stage release. MATERIALS AND METHODS: Nineteen children, 12 boys and 7 girls, with 24 congenital constriction bands constituted the clinical material. The mean age at presentation was 57 days (range 12 hours to 3 years) Band was unilateral in 14 and bilateral in five limbs. In unilateral cases, right side was involved in nine cases and left side in five. The constriction band is seen at the junction of middle and distal third. The patients having constriction bands in lower limbs and age less than 3 years were included in the study. One stage circumferential release of congenital constriction band was performed. Our youngest patient was operated at the age of six months. Club feet, (n=8) and lymphedema (n=7) were associated anomalies. Club feet and band were released in one stage in three limbs. The results were evaluated by criteria described by Joseph Upton and Cissy Tan. RESULTS: There were 18 excellent, six satisfactory results. No wound problem occurred. No vascular compromise was noted during or after the procedure. On follow-up, distal swelling reduced. CONCLUSIONS: One-stage circumferential release of congenital constriction band in lower limbs with or without lymphodema is a safe and easy procedure. PMID: 20419008 [PubMed - in process]PMCID: PMC2856396 1. SADJ. 2010 Feb;65(1):14, 16-8. Facial lymphoedema as an indicator of terminal disease in oral HIV-associated Kaposi sarcoma. Feller L, Khammissa RA, Wood NH, Jose RJ, Lemmer J. Department of Periodontology and Oral Medicine, School of Oral Health Sciences, University of Limpopo, Medunsa Campus, South Africa. lfeller@ul.ac.za Abstract Rapidly progressive facial lymphoedema developing concurrently with, or immediately after rapid enlargment of oral Kaposi sarcoma (KS) in HIV-seropositive highly active antiretroviral treatment (HAART)-naïve subjects, foretokens death. We present here an unusual case of HIV-KS in an 11-year-old HIV-seropositive HAART-naïve boy. Our patient's KS disease had had a fulminant course characterised by rapidly progressing oral HIV-KS, resorption of the mandibular alveolar bone process beneath some of the HIV-KS lesions, and rapidly progressive facial lymphoedema. He died 3 weeks after the onset of facial lymphoedema. PMID: 20411797 [PubMed - in process] 2. Hell J Nucl Med. 2010 Jan-Apr;13(1):6-10. Diagnostic application of lymphoscintigraphy in the management of lymphoedema. Sadeghi R, Kazemzadeh G, Keshtgar M. Nuclear Medicine, Mashhad University of Medical Sciences, Mashhad, Iran. Abstract Lymphoedema (LOE) is an under-diagnosed condition which can cause severe incapacitating swelling of the extremities. Misdiagno sis and/or delayed diagnosis are common and the goal of further evaluation is to confirm the cause and determine the type and site of lymphatic obstruction. Lymphoscintigraphy (LSG) is a minimally invasive way of evaluation of the lymphatic system and can be used in the management of the LOE patients. However, many aspects of this useful diagnostic procedure are not fully explained in the med ical literature. In this article we briefly explain the etiology and pathophysiology of LOE. Methodology and applications of LSG for the evaluation of this disease are extensively reviewed. PMID: 20411162 [PubMed - in process 3. Dermatol Online J. 2010 Apr 15;16(4):4. Papillary intralymphatic angioendothelioma of the thigh: A case report and review of the literature. Ward KA, Ecker PM, White RR, Melnik TE, Gulbahce EH, Wilke MS, Sangueza OP. University of Minnesota, USA. Abstract The term angiosarcoma, encompasses several neoplasms, all of which exhibit a malignant process derived from endothelial cells of the vessels. The most common form of angiosarcoma is highly aggressive, often fatal, and usually affects the head and neck region of elderly white men. Other low-grade forms of angiosarcoma, including papillary intralymphatic angioendothelioma, also known as Dabska tumor, are less invasive, affect a wider age range, and offer a better prognosis. There are several predisposing factors that increase the risk of angiosarcoma and include chronic lymphedema of the extremities, preexisting vascular lesions, and prior radiation, often as therapy for other malignancies. We report an unusual case of a very small, low-grade angiosarcoma on the thigh of an adult female with no known predisposing risk factors. PMID: 20409411 [PubMed - in process] 4. Int Wound J. 2010 Feb;7(1):14-26. The experience of children and families with lymphoedema--a journey within a journey. Moffatt CJ, Murray SG. University of Glasgow, Glasgow, UK. christine.moffatt@cricp.org.uk Abstract This paper reports on a study in the UK that explored the experience of children suffering with Lymphoedema and that of their families. Qualitative data was collected from 20 children between the ages of 6 and 18 and their respective parents. Single, semi-structured interviews were used in which children and their parents were asked to share how lymphoedema impacted on their family life. Children were asked about their school experience, their dreams and their aspirations. Three categories emerged. Firstly, the negotiation of the health care system. Themes included correct diagnosis, finding robust information and reaching a knowledgeable expert. The second category explored the complex role of the parents as advocates. Themes within this category included the dilemmas of parenting and the increasing challenges as children reached adolescence. The final category involved the impact on the family unit. The first theme concerned the integration of lymphoedema into daily activities and the intrusion on family time. The second explored the impact on siblings and the final theme the changing dilemmas as children moved through the stages of childhood and faced adulthood. PMID: 20409247 [PubMed - in process] 5. Br J Dermatol. 2010 Apr 16. [Epub ahead of print] High resolution cutaneous ultrasonography to differentiate lipoedema from lymphoedema. Naouri M, Samimi M, Atlan M, Perrodeau E, Vallin C, Zakine G, Vaillant L, Machet L. Université François Rabelais de Tours; UMR, Inserm U930, CNRS ERL 3106; Inserm CIC 202, Department of Dermatology, Department of Plastic Surgery, Department of Radiology, CHRU de Tours; France. Abstract Summary Introduction. Lipoedema is an accumulation of fat abnormally distributed in the lower limbs, and lymphoedema is edema caused by a deficiency of the lymphatic system. High-resolution ultrasound operating at 20 MHz makes it possible to characterise dermal oedema. The purpose of our study was to demonstrate that high-resolution ultrasound imaging of the skin was able to differentiate lipoedema from lymphoedema. Patients and method. Sixteen patients with lymphoedema (22 legs), 8 patients with lipoedema (16 legs) and 8 controls (16 legs) were included. Patients with lipolymphoedema were excluded. Ultrasound examinations were carried out with a real time high resolution ultrasound device on 3 different sites for each lower limb. The images were then anonymized and examined by an independent dermatologist who was blind to the clinical diagnosis. A new series of images was examined by 3 dermatologists to check inter-observer agreement. Results. A significant difference in dermal thickness was observed between lymphoedema and lipoedema patients and lymphoedema patients and controls. No significant difference in dermal thickness was shown between lipoedema and controls at the thigh or ankle. Dermal hypoechogenicity was evidenced on at least one of the three sites in 100% of lymphoedema patients, 12.5% of lipoedema patients and 6.25% of controls. Hypoechogenicity affected the entire dermis in all cases of lymphoedema except one. In cases of lipoedema and controls, hypoechogenicity was only localized at the ankle and prevailed in the upper dermis. The expert diagnosed all lower limbs with lymphoedema. No cases of lipoedema were diagnosed as lymphoedema. Exact inter-observer agreement was excellent (0.98). Conclusions. High-resolution cutaneous ultrasonography makes it possible to differentiate lymphoedema from lipoedema. Obtaining a reliable diagnosis through high resolution cutaneous ultrasonography might be valuable to improve the treatment of lipoedema and lymphoedema. PMID: 20408836 [PubMed - as supplied by publisher] |