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1

Small, Juan E., Yasir N. Jassam, Kirstin M. Small, et al. "Barraquer-Simons Syndrome." American Journal of the Medical Sciences 352, no. 3 (2016): 280–84. http://dx.doi.org/10.1016/j.amjms.2016.05.007.

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2

Mansouri Hattab, N., S. Lahmiti, S. Aimadeddine, S. Fawzi, M. El Bouihi, and T. Fikry. "Syndrome de Barraquer-Simons." Revue de Stomatologie et de Chirurgie Maxillo-faciale 112, no. 3 (2011): 172–73. http://dx.doi.org/10.1016/j.stomax.2011.03.005.

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3

Bellon, N., F. Lioté, M. Bagot, E. Bourrat, and A. Petit. "Syndrome de Barraquer-Simons." Annales de Dermatologie et de Vénéréologie 140, no. 12 (2013): S603. http://dx.doi.org/10.1016/j.annder.2013.09.545.

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4

Dutta, Mita, Munira Afroz Siddika, Mohammad Aminul Islam, and Hurjahan Banu. "Barraquer–Simons syndrome: A case report." Bangabandhu Sheikh Mujib Medical University Journal 18, no. 2 (2025): e78654. https://doi.org/10.3329/bsmmuj.v18i2.78654.

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5

Murakhovskaya, E. V., and N. T. Starkova. "To the diagnosis of Barraquer-Simons syndrome." Problems of Endocrinology 44, no. 3 (1998): 29–31. http://dx.doi.org/10.14341/probl199844329-31.

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Barraquer-Simons syndrome is a rare disease characterized by the disappearance of subcutaneous fat in the upper half of the body with normal or excessive deposition of fat in the lower half of the body. There are other terms that refer to this disease: Hollander-Simons syndrome, progressive lipodystrophy, partial lipodystrophy, paradoxical lipodystrophy, progressive segmental lipodystrophy, i.e. the name emphasizes the disappearance of fat in certain parts of the body.
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6

Sedova, Tatiana G., Vladimir D. Elkin, Marina Y. Kobernik, and Elena N. Borodina. "A clinical case of Barraquer-Simons syndrome." Almanac of Clinical Medicine 49, no. 8 (2021): 564–70. http://dx.doi.org/10.18786/2072-0505-2021-49-067.

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Barraquer-Simons syndrome (SBS) belongs to the groupof lipodystrophy with complex etiology and pathophysiology and is characterized by progressive loss of subcutaneous fat, presumably related to autoimmune destruction of adipocytes. SBS is frequently associated with autoimmune disorders. Its first signs are found in childhood or puberty. It is characterized by gradual onset from the face with subsequent extension of the pathological process downwards without the involvement of the lower extremities. At the same time, there is a clear delimitation between the lipodystrophic and unaffected zones
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7

Tuzun, Binnur, Deniz Kircuval, Cuyan Demirkesen, Nadir Goksugur, Gaye Unal, and Yalcin Tuzun. "Barraquer-Simons syndrome with benign infundibulocystic proliferation." International Journal of Dermatology 42, no. 3 (2003): 210–12. http://dx.doi.org/10.1046/j.1365-4362.2003.01172.x.

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8

Requena Caballero, Celia, Miguel Ángel Navarro Mira, Isabel Febrer Bosch, José Miguel Fortea Bauxauli, and Adolfo Aliaga Boniche. "Barraquer-Simons lipodystrophy associated with antiphospholipid syndrome." Journal of the American Academy of Dermatology 49, no. 4 (2003): 768–69. http://dx.doi.org/10.1067/s0190-9622(03)00789-8.

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9

Simsek-Kiper, Pelin Ozlem, Emir Roach, Gulen Eda Utine, and Koray Boduroglu. "Barraquer-Simons syndrome: A rare clinical entity." American Journal of Medical Genetics Part A 164, no. 7 (2014): 1756–60. http://dx.doi.org/10.1002/ajmg.a.36491.

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10

Ferrarini, A., D. Milani, M. Bottigelli, G. Cagnoli, and Angelo Selicorni. "Two new cases of Barraquer-Simons syndrome." American Journal of Medical Genetics 126A, no. 4 (2004): 427–29. http://dx.doi.org/10.1002/ajmg.a.20623.

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11

FRANÇA, ANDRÉ LUIZ DE ARAUJO, LUÍS ALBERTO DE SOUZA LEITE, JEANINE MARIA TEJO CAMINHA, et al. "Barraquer-Simons syndrome: literature review and case report." Revista Brasileira de Cirurgia Plástica (RBCP) – Brazilian Journal of Plastic Sugery 31, no. 2 (2016): 281–86. http://dx.doi.org/10.5935/2177-1235.2016rbcp0046.

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12

Bouzaib, S., H. Benderradji, F. Devemy, et al. "Phénotype métabolique du syndrome de Barraquer–Simons (SBS)." Annales d'Endocrinologie 79, no. 4 (2018): 231. http://dx.doi.org/10.1016/j.ando.2018.06.108.

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13

Varghese, Abin, and Sukumaran Pradeep Nair. "Barraquer-Simons Syndrome Associated with Myopathy - A Rare Entity." Indian Dermatology Online Journal 15, no. 4 (2023): 665–67. http://dx.doi.org/10.4103/idoj.idoj_398_23.

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14

Lau, E., P. Freitas, M. J. Matos, and D. Carvalho. "P18. LIPODISTROFIA PARCIAL ADQUIRIDA– BARRAQUER-SIMONS SYNDROME: CASO CLÍNICO." Revista Portuguesa de Endocrinologia, Diabetes e Metabolismo 7, no. 2 (2012): 110–11. http://dx.doi.org/10.1016/s1646-3439(12)70144-x.

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15

de Lucas-Collantes, Carmen, Jesús Pozo-Román, Cristina Aparicio-López, Inmaculada de Prada-Vicente, and Jesús Argente. "Acquired partial lipodystrophy (Barraquer–Simons syndrome) and IgA nephropathy." Nefrología (English Edition) 36, no. 5 (2016): 556–58. http://dx.doi.org/10.1016/j.nefroe.2016.11.001.

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16

BALEVİ BATUR, Elif. "Barraquer-Simons Syndrome Occurred After Pregnancy: A Rare Case Report." Fiziksel Tıp ve Rehabilitasyon Bilimleri Dergisi 22, no. 1 (2019): 33–35. http://dx.doi.org/10.31609/jpmrs.2017-58127.

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17

Taj, Farhana Tahseen, and Vyshak BM. "Acquired partial lipodystrophy: Barraquer –Simons syndrome: A rare case report." Our Dermatology Online 10, no. 1 (2019): 59–61. http://dx.doi.org/10.7241/ourd.20191.15.

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18

Ben Ghorbel, I., T. Ben Salem, M. Lamloum, et al. "Syndrome de Barraquer-Simons au cours d’un lupus érythémateux systémique." La Revue de Médecine Interne 31, no. 5 (2010): 372–74. http://dx.doi.org/10.1016/j.revmed.2009.10.434.

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19

Gjukaj, Driton, Naser Musli, and Margarita G. Todorova. "Long-term Electrodiagnostic Changes in Barraquer-Simons Syndrome: A Case Report." Klinische Monatsblätter für Augenheilkunde 240, no. 04 (2023): 532–35. http://dx.doi.org/10.1055/a-2040-3480.

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20

Boucelma, F., N. Benzine, A. Rehaimia, I. Hannous, and S. Aitabderrahmane. "Le syndrome lipoatrophique partiel acquis (syndrome de Barraquer et Simons) : savoir y pensé." Annales d'Endocrinologie 85, no. 5 (2024): 505–6. http://dx.doi.org/10.1016/j.ando.2024.08.415.

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21

Rho, Nark-Kyoung, and Won-Serk Kim. "Acquired Partial Lipodystrophy (Barraquer-Simons Syndrome): Early Cosmetic Intervention with Autologous Fat." Annals of Dermatology 30, no. 5 (2018): 610. http://dx.doi.org/10.5021/ad.2018.30.5.610.

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22

Afra, T. P., Keshavamurthy Vinay, Muhammed Razmi T, Tarun Narang, and Sunil Dogra. "Hyaluronic acid fillers for correcting midface volume deficit in Barraquer‐Simons syndrome." Journal of Cosmetic Dermatology 18, no. 5 (2019): 1264–66. http://dx.doi.org/10.1111/jocd.12847.

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23

Spranger, Stephanie, Matthias Spranger, Abel-Jan Tasman, Wolfgang Reith, Theda Voigtländer, and Volker Voigtländer. "Barraquer-Simons syndrome (with sensorineural deafness): A contribution to the differential diagnosis of lipodystrophy syndromes." American Journal of Medical Genetics 71, no. 4 (1997): 397–400. http://dx.doi.org/10.1002/(sici)1096-8628(19970905)71:4<397::aid-ajmg5>3.0.co;2-r.

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24

Payapvipapong, Kittisak, Nucha Niumpradit, Artit Nakakes, and Kitti Buranawuti. "A rare case of acquired partial lipodystrophy (Barraquer-Simons syndrome) with localized scleroderma." International Journal of Dermatology 53, no. 1 (2013): 82–84. http://dx.doi.org/10.1111/j.1365-4632.2011.05435.x.

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25

Corvillo, Fernando, Laura González-Sánchez, Alberto López-Lera, et al. "Complement Factor D (adipsin) Levels Are Elevated in Acquired Partial Lipodystrophy (Barraquer–Simons syndrome)." International Journal of Molecular Sciences 22, no. 12 (2021): 6608. http://dx.doi.org/10.3390/ijms22126608.

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Complement overactivation has been reported in most patients with Barraquer–Simons syndrome (BSS), a rare form of acquired partial lipodystrophy. Complement Factor D (FD) is a serine protease with a crucial role in the activation of the alternative pathway of the complement system, which is mainly synthesized by adipose tissue. However, its role in the pathogenesis of BSS has not been addressed. In this study, plasma FD concentration was measured in 13 patients with BSS, 20 patients with acquired generalized lipodystrophy, 22 patients with C3 glomerulopathy (C3G), and 50 healthy controls. Gene
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26

Srinivasaraghavan, Rangan. "Barraquer Simons Syndrome - Rare Cause of Progressive Wasting in an Eight Year Old Girl." SBV Journal of Basic, Clinical and Applied Health Science 1, A4 (2018): 139–40. http://dx.doi.org/10.5005/jp-journals-10082-01138.

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27

Velter, C., N. Messaddeq, E. Levy, et al. "Abnormal lipid storage related to adipocyte shrinkage in acquired partial lipodystrophy (Barraquer–Simons syndrome)." Journal of the European Academy of Dermatology and Venereology 33, no. 11 (2019): 2188–91. http://dx.doi.org/10.1111/jdv.15366.

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28

Al-Jawad, Mohammad, Lina Mawaldi, Nada Mawaldi, Mariam Krimsty, and Silva Ishkanian. "Acquired partial lipodystrophy (Barraquer-Simons syndrome) with both-side axillary breasts: A case report." International Journal of Surgery Case Reports 129 (April 2025): 111202. https://doi.org/10.1016/j.ijscr.2025.111202.

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29

Jeon, Faith Hyun Kyung, Michelle Griffin, Carole Frosdick, and Peter Edward Michael Butler. "Lipotransfer provides effective soft tissue replacement for acquired partial lipodystrophy." BMJ Case Reports 13, no. 5 (2020): e232601. http://dx.doi.org/10.1136/bcr-2019-232601.

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We present a 48-year-old female patient who presented with features consistent with acquired partial lipodystrophy (APL) also known as ‘Barraquer-Simons syndrome’. It is a rare disease characterised by a gradual and progressive onset of lipoatrophy limited to the face, neck, upper limbs, thorax and abdomen and sparing the lower extremities. The resultant physical appearance can have significant psychosocial sequelae, further compounded by misdiagnosis and delay in recognition and management. Treatment is aimed at surgical correction of soft tissue destruction. Autologous fat transfer is an est
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30

Gautam, Anjali, Purnachandra Badabagni, Surender Singh, Archana Mavoori, Pranavi Katike, and Sravya Himasri Bhamidipati. "Barraquer–Simons syndrome: A rare case of acquired partial lipodystrophy after pregnancy with diabetes mellitus." Cosmoderma 4 (November 7, 2024): 136. http://dx.doi.org/10.25259/csdm_150_2024.

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Barraquer–Simons syndrome (BSS) is an uncommon acquired partial lipodystrophy (APL) characterized by gradual, progressive loss of subcutaneous fat limited to the upper part of the body. It is a rare condition of uncertain origin typically appearing in early adolescence with a higher incidence among females with autoimmunity and C3 hypocomplementemia. Approximately 250 cases were reported worldwide, we present a case of BSS that began after pregnancy. A biopsy of the affected areas reveals reduced adipocytes. Metabolic abnormalities are relatively uncommon in APL compared to other forms of lipo
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31

Gabura, Ancuta, and Laura Ciobanu. "From Barraquer-Simons syndrome to treatment perspectives for obesity and diabetes mellitus – a case report." Archive of Clinical Cases 01, no. 02 (2014): 41–47. http://dx.doi.org/10.22551/2014.02.0102.10010.

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32

Pradhan, Swetalina, and Kananbala Sahu. "Barraquer–Simons Lipodystrophy Syndrome in an Otherwise Normal Child: A Rare Case of Acquired Partial Lipodystrophy." Indian Journal of Paediatric Dermatology 22, no. 1 (2021): 87. http://dx.doi.org/10.4103/ijpd.ijpd_73_20.

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33

Mondal, Nirjhar, Sudip Mandal, Pramit Nandy, and Biswanath Naskar. "Barraquer-Simons Syndrome: An Unusual Form of Acquired Partial Lipodystrophy in a Child with Lupus Nephritis." Indian Dermatology Online Journal 14, no. 6 (2023): 890–92. http://dx.doi.org/10.4103/idoj.idoj_692_22.

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34

Corvillo, Fernando, Pilar Nozal, Alberto López‐Lera, et al. "Evidence of ongoing complement activation on adipose tissue from an 11‐year‐old girl with Barraquer–Simons syndrome." Journal of Dermatology 47, no. 12 (2020): 1439–44. http://dx.doi.org/10.1111/1346-8138.15570.

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35

Mun, Goo-Hyun. "One-Stage Transfer of 2 Paddles of Thoracodorsal Artery Perforator Flap with Vascular Anastomoses for Barraquer-Simons Syndrome." Journal of Craniofacial Surgery 23, no. 6 (2012): 1936. http://dx.doi.org/10.1097/scs.0b013e3182654338.

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36

José, Anthar Ávalos Narváez, Daniel Lupercio Romo Cesar, Cuadra Casillas Alondra, et al. "Surgical Management of Lipodystrophy in Barraquer-Simons Syndrome: Autologous Fat Redistribution and Reconstructive Strategies for Acquired Partial Lipodystrophy." International Journal of Medical Science and Clinical Research Studies 5, no. 05 (2025): 794–99. https://doi.org/10.5281/zenodo.15550516.

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<strong>Background:</strong> Barraquer-Simons Syndrome (BSS), a rare form of acquired partial lipodystrophy, is characterized by progressive adipose tissue loss in the cephalothoracic region, often with paradoxical fat accumulation in the lower body. This condition presents significant metabolic and aesthetic challenges, necessitating multidisciplinary management. Fat redistribution surgery (FRS) has emerged as a potential therapeutic approach to restore facial and upper body contour while addressing metabolic complications. <strong>Objective:</strong> This article reviews the surgical techniq
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37

Winhoven, S. M., A. Hafejee, and I. H. Coulson. "An unusual case of an acquired acral partial lipodystrophy (Barraquer-Simons syndrome) in a patient with extrinsic allergic alveolitis." Clinical and Experimental Dermatology 31, no. 4 (2006): 594–96. http://dx.doi.org/10.1111/j.1365-2230.2006.02137.x.

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38

Ben Ghorbel, I., T. Ben Salem, T. Larbi, et al. "Association d’un syndrome de Barraquer-Simons à un lupus érythémateux systémique et à une hypothyroïdie. À propos d’une observation." La Revue de Médecine Interne 29 (June 2008): S158. http://dx.doi.org/10.1016/j.revmed.2008.03.291.

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39

Okazaki, Mutsumi, Kentaro Tanaka, Satoshi Kodaira, Tsutomu Homma, and Hiroki Miyashita. "One-Stage Transfer of 2 Paddles of Thoracodorsal Artery Perforator Flap With 1 Pair of Vascular Anastomoses for Barraquer-Simons Syndrome." Journal of Craniofacial Surgery 23, no. 3 (2012): 883–85. http://dx.doi.org/10.1097/scs.0b013e31824ddf5a.

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40

Goossens, Sofie, and Bruno Coessens. "Facial contour restoration in Barraquer-Simons syndrome using two free tram flaps: Presentation of two case reports and long-term follow-up." Microsurgery 22, no. 5 (2002): 211–18. http://dx.doi.org/10.1002/micr.22508.

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41

Baracho, Maria Fatima, Maria Goreti Santos, Beatriz Amaral, et al. "Barraquer-Simons syndrome." Endocrine Abstracts, April 1, 2013. http://dx.doi.org/10.1530/endoabs.32.p276.

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42

Corvillo, Fernando, Giovanni Ceccarini, Pilar Nozal, et al. "Immunological features of patients affected by Barraquer-Simons syndrome." Orphanet Journal of Rare Diseases 15, no. 1 (2020). http://dx.doi.org/10.1186/s13023-019-1292-1.

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Abstract Background C3 hypocomplementemia and the presence of C3 nephritic factor (C3NeF), an autoantibody causing complement system over-activation, are common features among most patients affected by Barraquer-Simons syndrome (BSS), an acquired form of partial lipodystrophy. Moreover, BSS is frequently associated with autoimmune diseases. However, the relationship between complement system dysregulation and BSS remains to be fully elucidated. The aim of this study was to provide a comprehensive immunological analysis of the complement system status, autoantibody signatures and HLA profile in
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43

Oliveira, Joana, Paula Freitas, Eva Lau, and Davide Carvalho. "Barraquer–Simons syndrome: a rare form of acquired lipodystrophy." BMC Research Notes 9, no. 1 (2016). http://dx.doi.org/10.1186/s13104-016-1975-9.

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44

Acharya, Suman, Suraj Kandel, Suraj Shrestha, et al. "Barraquer‐Simons syndrome in systemic lupus erythematosus: A case report." Clinical Case Reports 9, no. 11 (2021). http://dx.doi.org/10.1002/ccr3.5132.

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45

Corvillo, Fernando, Giovanni Ceccarini, Pilar Nozal, et al. "Correction to: Immunological features of patients affected by Barraquer-Simons syndrome." Orphanet Journal of Rare Diseases 15, no. 1 (2020). http://dx.doi.org/10.1186/s13023-020-1350-8.

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46

Gowda, Shreya K., Kapil Baheti, Sonali Gupta, Amulya M. Lakshman, and Dinesh Asati. "Successful fat transplant in a rare acquired lipodystrophy Barraquer–Simons syndrome." Journal of Cutaneous and Aesthetic Surgery, September 27, 2024, 1–6. http://dx.doi.org/10.25259/jcas_89_23.

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Lipodystrophies can be generalized and partial/localized lack of subcutaneous tissue, which are uncommon heterogeneous illnesses that can be hereditary or acquired. Acquired partial lipodystrophy’ (APL) is the new name for lipodystrophia progressiva, also known as Barraquer–Simons syndrome (BSS). It could come after a severe viral infection like the measles. Young people and children both experience it. Typically, BSS or APL starts between the ages of 5 and 15. The principal feature of BSS is cephalothoracic lipodystrophy, which affects the face, neck, arms, thorax, and upper abdomen in a ceph
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47

Hamam, Meryem, Atilla Adnan Eyuboglu, and Mustafa Tonguc Isken. "Barraquer Simons Syndrome: Case Series and Review of Surgical Treatments for Facial Lipodystrophy." Aesthetic Plastic Surgery, March 24, 2025. https://doi.org/10.1007/s00266-025-04794-z.

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Abstract Background Barraquer Simons Syndrome (BSS) is a subtype of idiopathic acquired lipodystrophy, wherein patients—mostly female—lose subcutaneous fat in the upper half of the body starting in childhood or puberty. A disproportionate fat allocation can be seen in the trochanteric region and thighs. Methods Along with three cases of lipoatrophy treated at our hospital, we conducted a comprehensive review and presented the treatments in the literature for improving bilateral facial lipodystrophy. Results Flaps and autologous fat grafting procedures were favored across the literature, and re
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48

Kaewkrasaesin, Chatchon, Michael Hwang, Chandna Vasandani, Rebecca J. Brown, and Abhimanyu Garg. "Clinical features, metabolic and autoimmune derangements in acquired partial lipodystrophy (Barraquer-Simons Syndrome)." Journal of Clinical Endocrinology & Metabolism, May 28, 2025. https://doi.org/10.1210/clinem/dgaf315.

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Abstract: Introduction Acquired partial lipodystrophy (APL) is an ultra-rare disorder characterized by unique loss of subcutaneous fat affecting mostly the face, neck, trunk and upper extremities. The precise prevalence of metabolic derangements and other co-morbidities amongst patients with APL is not clear. Therefore, we report clinical features, metabolic and autoimmune derangements in a large cohort. Methods Seventy-seven females and 9 males with median age of 40 years (range 8-78 years) with APL from two tertiary referral centers, UT Southwestern and National Institute of Diabetes and Dig
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49

Narváez, José Anthar Ávalos, Cesar Daniel Lupercio Romo, Alondra Cuadra Casillas, et al. "Surgical Management of Lipodystrophy in Barraquer-Simons Syndrome: Autologous Fat Redistribution and Reconstructive Strategies for Acquired Partial Lipodystrophy." International Journal of Medical Science and Clinical Research Studies 05, no. 05 (2025). https://doi.org/10.47191/ijmscrs/v5-i05-20.

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Background: Barraquer-Simons Syndrome (BSS), a rare form of acquired partial lipodystrophy, is characterized by progressive adipose tissue loss in the cephalothoracic region, often with paradoxical fat accumulation in the lower body. This condition presents significant metabolic and aesthetic challenges, necessitating multidisciplinary management. Fat redistribution surgery (FRS) has emerged as a potential therapeutic approach to restore facial and upper body contour while addressing metabolic complications. Objective: This article reviews the surgical techniques, outcomes, and long-term effic
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50

CDA-AMC. "Metreleptin (Myalepta)." Canadian Journal of Health Technologies 4, no. 10 (2024). http://dx.doi.org/10.51731/cjht.2024.997.

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Reimbursement reviews are comprehensive assessments of the clinical effectiveness and cost-effectiveness, as well as patient and clinician perspectives, of a drug or drug class. The assessments inform non-binding recommendations that help guide the reimbursement decisions of Canada’s federal, provincial, and territorial governments, with the exception of Quebec. This review assesses metreleptin (Myalepta), 3 mg, 5.8 mg, and 11.3 mg, powder for solution, subcutaneous injection. Indication: As an adjunct to diet as a replacement therapy to treat the complications of leptin deficiency in lipodyst
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