Clinical Features and Metabolic and Autoimmune Derangements in Acquired Partial Lipodystrophy: Report of 35 Cases and Review of the Literature

Medicine

Volumn 83, Issue 1, 2004, Pages 18-34

  Misra, Anoop ^a   Peethambaram, Aparna ^a   Garg, Abhimanyu ^a,b  

^a UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER   (United States)

^b UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

2,4 THIAZOLIDINEDIONE DERIVATIVE; ANTIBIOTIC AGENT; COMPLEMENT COMPONENT C3; CORTICOSTEROID; CYTOTOXIC AGENT; NEPHRITIC FACTOR; POLYCLONAL ANTIBODY; ROSIGLITAZONE; TROGLITAZONE;

ACQUIRED PARTIAL LIPODYSTROPHY; ADULT; AUTOIMMUNE DISEASE; BODY FAT; BODY FAT DISTRIBUTION; CLINICAL ARTICLE; CLINICAL FEATURE; COMPLEMENT ACTIVATION; COMPLEMENT ALTERNATIVE PATHWAY; CORTICOSTEROID THERAPY; DERMATOMYOSITIS; DIABETES MELLITUS; DRUG EFFECT; DYSLIPIDEMIA; FEMALE; HISTOPATHOLOGY; HUMAN; HUMAN TISSUE; HYPOCOMPLEMENTEMIA; IMPAIRED GLUCOSE TOLERANCE; INFECTION SENSITIVITY; KIDNEY DISEASE; KIDNEY FAILURE; LABORATORY TEST; LIPOATROPHY; LIPODYSTROPHY; MALE; MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS; METABOLIC DISORDER; NUCLEAR MAGNETIC RESONANCE IMAGING; ONSET AGE; PATHOGENESIS; PLASTIC SURGERY; PREVALENCE; PRIORITY JOURNAL; PROGNOSIS; RECURRENT INFECTION; REVIEW; SEX DIFFERENCE; SYSTEMIC LUPUS ERYTHEMATOSUS;

ADIPOSE TISSUE; ADOLESCENT; ADULT; ANTILIPEMIC AGENTS; BODY COMPOSITION; CHILD; DIABETES COMPLICATIONS; DIABETES MELLITUS; FEMALE; GLOMERULONEPHRITIS, MEMBRANOPROLIFERATIVE; GLUCOSE INTOLERANCE; HUMANS; HYPERTRIGLYCERIDEMIA; LEPTIN; LIPODYSTROPHY; MAGNETIC RESONANCE IMAGING; MALE; MIDDLE AGED; RECONSTRUCTIVE SURGICAL PROCEDURES; TREATMENT OUTCOME;

EID: 1542642958     PISSN: 00257974     EISSN: None     Source Type: Journal    
DOI: 10.1097/01.md.0000111061.69212.59     Document Type: Review

References (194)

1. Agarwal AK, Arioglu E, de Almeida S, Akkoc N, Taylor SI, Bowcock AM, Barnes RI, Garg A. AGPAT2 is mutated in congenital generalized lipodystrophy linked to chromosome 9q34. Nat Genet. 2002;31:21-23.
2. Agarwal AK, Garg A. A novel heterozygous mutation in peroxisome proliferator-activated receptor-γ gene in a patient with familial partial lipodystrophy. J Clin Endocrinol Metab. 2002;87:408-411.
3. Agnello V. Complement deficiency states. Medicine (Baltimore). 1978;57:1-23.
4. Akhter J, Qureshi R. Partial lipodystrophy and successful pregnancy outcome. J Pak Med Assoc. 1995;45:24.
5. Allen BR. Partial lipodystrophy. Br J Dermatol. 1978;99(Suppl 16):48-49.
6. Alper CA, Bloch KJ, Rosen FS. Increased susceptibility to infection in a patient with type 11 essential hypercatabolism of C3. N Engl J Med. 1973;288:601-606.
7. American Academy of Pediatrics. National Cholesterol Education Program: Report of the Expert Panel on Blood Cholesterol Levels in Children and Adolescents. Pediatrics. 1992;89:525-584.
8. Aragona P, Quattrocchi P, Trombetta CJ, Ferlazzo E, Spinella R, Bonanno D. Retinal alterations in acquired partial lipodystrophy: a case report. Arch Ophthalmol. 2002;120:218-220.
9. Arioglu E, Duncan-Morin J, Sebring N, Rother KI, Gottlieb N, Lieberman J, Herion D, Kleiner DE, Reynolds J, Premkumar A, Sumner AE, Hoofnagle J, Reitman ML, Taylor SI. Efficacy and safety of troglitazone in the treatment of lipodystrophy syndromes. Ann Intern Med. 2000;133:263-274.
10. Barraquer L. Histoire clinique d'un cas d'atrophie du tissu celluloadipeux. Neurolog Zentralblatt. 1907;26:1072.
11. Baxter H, Entin MA, Drummond JA. Studies in progressive lipodystrophy. Can Med Assoc J. 1948;59:452-458.
12. Bennett WM, Bardana EJ, Wuepper K, Houghton D, Border WA, Gotze O, Schreiber R. Partial lipodystrophy, C3 nephritic factor and clinically inapparent mesangiocapillary glomerulonephritis. Am J Med. 1977;62:757-760.
13. Berger EH. Progressive lipodystrophy. Med Clin North Am. 1932;15:1431-1438.
14. Berger M, Balow JE, Wilson CB, Frank MM. Circulating immune complexes and glomerulonephritis in a patient with congenital absence of the third component of complement. N Engl J Med. 1983;308:1009-1012.
15. Berkan L, Chmielewski H, Spychalski E. Case of Barraquer-Simons disease. Wiad Lek. 1972;25:1705-1708.
16. Bernstein RS, Pierson RN III, Ryan SF, Crespin SB. Adipose cell morphology and control of lipolysis in a patient with partial lipodystrophy. Metabolism. 1979;28:519-526.
17. Biasi D, Caramaschi P, Carletto A, Bambara LM. A case of acquired partial lipodystrophy associated with localized scleroderma and undifferentiated connective tissue disease. Rheumatol Int. 1999;19:75-76.
18. Bier DM, O'Donnell JJ, Kaplan SL. Cephalothoracic lipodystrophy with hypocomplementemic renal disease: discordance in identical twin sisters. J Clin Endocrinol Metab. 1978;46:800-807.
19. Blake DR, Rashid H, McHugh M, Morley AR. A possible association of partial lipodystrophy with anti-GBM nephritis (Goodpasture's syndrome). Postgrad Med J. 1980;56:137-139.
20. Bocker FM, Weilbrecht WU, Neundorfer B. Progressive lipodystrophy (Barraquer-Simon syndrome): differential diagnosis and clinical aspects. Fortschr Neurol Psychiatr. 1986;54:59-67.
21. Boissonas L. La lipodystrophie progressive. Rev Med Suisse Rom. 1914;34:214-217.
22. Bossert-Rollet. Lipodystrophia progressiva. Mschr Kinderheilk. 1918;14:230-236.
23. Boston LN. Lipodystrophia progressiva superior. N Y Med J Med Rec. 1923;118:668-673.
24. Bouadjar B, Kaci A, Ysmail-Dahlouk M. Partial lipodystrophy, glomerulonephritis and hypocomplementemia. Ann Dermatol Venereol. 1991;118:826-827.
25. Branisteanu DD, Zbranca E. Barraquer-Simons syndrome. Report of a case and review of the literature. Rev Med Chir Soc Med Nat Iasi. 2000;104:155-158.
26. Brown EE. Progressive lipodystrophy following infection. Report of three cases. Arch Pediatr. 1958;75:227-233.
27. Bulbul M, Erdogan O, Demircin G, Altuntas B, Memis L, Oner A. Acute pancreatitis in a patient with partial lipodystrophy and membranoproliferative glomerulonephritis. Nephrol Dial Transplant. 2001;16:1930-1931.
28. Burden RP. Partial lipodystrophy and glomerulonephritis. Br J Dermatol. 1978;99(Suppl 16):49-51.
29. Campbell H. Disappearance, more or less complete, of the subcutaneous fat above the region of lower extremities. Trans Clin Soc Lond. 1907;40:272.
30. Capper A. Lipodystrophia progressiva. Arch Pediatr. 1932;49:155-164.
31. Caramaschi P, Biasi D, Lestani M, Chilosi M. A case of acquired partial lipodystrophy associated with POEMS syndrome. Rheumatology. 2003;42:488-490.
32. Carr A, Samaras K, Burton S, Law M, Freund J, Chisholm DJ, Cooper DA. A syndrome of peripheral lipodystrophy, hyperlipidaemia and insulin resistance in patients receiving HIV protease inhibitors. AIDS. 1998;12:F51-F58.
33. Charlesworth JA, Williams DG, Sherington E, Lachmann PJ, Peters DK. Metabolic studies of the third component of complement and the glycine-rich beta glycoprotein in patients with hypocomplementemia. J Clin Invest. 1974;53:1578-1587.
34. Chartier S, Buzzanga JB, Paquin F. Partial lipodystrophy associated with a type 3 form of membranoproliferative glomerulonephritis. J Am Acad Dermatol. 1987;16:201-205.
35. Chen D, Misra A, Garg A. Lipodystrophy in HIV-infected patients. J Clin Endocrinol Metab. 2002;87:4845-4856.
36. Chopra S, Isaacs R, Mammen K, Pawar B. Renal transplantation in a patient with Barraquer-Simons disease and mesangiocapillary glomerulonephritis type 11. Nephrol Dial Transplant. 2000;15:1723-1724.
37. Choy LN, Rosen BS, Spiegelman BM. Adipsin and an endogenous pathway of complement from adipose cells. J Biol Chem. 1992;267:12736-12741.
38. Christiansen V. Lipodystrophie progressivie. Rev Neurol (Paris). 1922;29:747-753.
39. Ciarpella E, Scatafassi S. I1 trattamento plastico della atrofia lipodistrofica della faccia nel morbo di Barraquer Simons. Policlinico [Prat]. 1968;75:980-984.
40. Cigtay OS, Benitez RP, Farzaneh NK. Unusual mammographic findings in a patient with partial lipodystrophy. AJR Am J Roentgenol. 1993;160:417-418.
41. Coessens BC, Van Geertruyden JP. Simultaneous bilateral facial reconstruction of a Barraquer-Simons lipodystrophy with free TRAM flaps. Plast Reconstr Surg. 1995;95:911-915.
42. Cronin CC, Higgins TJ, Molloy M. Lupus, C3 nephritic factor and partial lipodystrophy. Q J Med. 1995;88:298-299.
43. Currier FP, Davis DB. Progressive lipodystrophy appearing at menopause. Am J Med Sci. 1930;179:750-760.
44. Davis TM, Holdright DR, Schulenberg WE, Turner RC, Joplin GF. Retinal pigment epithelial change and partial lipodystrophy. Postgrad Med J. 1988;64:871-874.
45. Debora MJM, Martul MV, Peiro JLE, Perez JG, Rodriguez JL. Lipodistrofia partial y glomerulonefritis membranoproliferativa. A proposito de un caso. An Esp Pediatr. 1989;30:499-502.
46. De Leo T, Iacono G, Caccia Perugini I, Guala L. Levels and composition of serum depot lipids in Barraquer-Simons' disease. Folia Endocrinol. 1967;20:247-254.
47. Demetriou K, Kallikas I, Zouvani I, Kyriacou K, Pierides A. The pregnant patient with partial lipodystrophy developing acute renal failure-onset of de novo membranoproliferative glomerulonephritis. Nephrol Dial Transplant. 1998;13:2121-2124.
48. Ducours JL, Poizac P, Ardanza B, Modschiedler T, Caix P, Barraquer-Simons syndrome and facial lipo-filling. Apropos of a case. Rev Stomatol Chir Maxillofac. 1991;92:105-111.
49. Eisinger AJ, Shortland JR, Moorhead PJ. Renal disease in partial lipodystrophy. Q J Med. 1972;41:343-354.
50. Expert Panel on Detection, Evaluation and Treatment of High Blood Cholesterol in Adults. Executive summary of the third report of the National Cholesterol Education Program (NCEP) Expert Panel on detection, evaluation, and treatment of high blood cholesterol in adults (Adult Treatment Panel III). JAMA. 2001;285:2486-2497.
51. Fijen CA, Kuijper EJ, Te Bulte M, van de Heuvel MM, Holdrinet AC, Sim RB, Daha MR, Dankert J. Heterozygous and homozygous factor H deficiency states in a Dutch family. Clin Exp Immunol. 1996;105:511-516.
52. Finn JE, Mathieson PW. Molecular analysis of C3 allotypes in patients with nephritic factor. Clin Exp Immunol. 1993;91:410-414.
53. Fitch N, Tulandi T. Progressive partial lipodystrophy and third-trimester intrauterine fetal death. Am J Obstet Gynecol. 1987;156:1195-1196.
54. Fiumara A, Mazzarino MC, Di Stefano VA, Micali G, Mancuso GR, La Rosa M. Partial lipodystrophy: immunologic studies in a Sicilian girl. Ann Allergy. 1989;62:128-129.
55. Font J, Herrero C, Bosch X, Cervera R, Ingelmo M, Mascaro JM. Systemic lupus erythematosus in a patient with partial lipodystrophy. J Am Acad Dermatol. 1990;22:337-340.
56. Fowler PBS. Lipodystrophia progressiva and temporary hydronephrosis. Br Med. J. 1955;1:1249-1251.
57. Garg A. Gender differences in the prevalence of metabolic complications in familial partial lipodystrophy (Dunnigan variety). J Clin Endocrinol Metab. 2000;85:1776-1782.
58. Garg A. Lipodystrophies. Am J Med. 2000;108:143-152.
59. Garg A, Fleckenstein JL, Peshock RM, Grundy SM. Peculiar distribution of adipose tissue in patients with congenital generalized lipodystrophy. J Clin Endocrinol Metab. 1992;75:358-361.
60. Garg A, Misra A. Lipodystrophies and diabetes. In: Defronzo RA, Ferrannini E, Keen H, Zimmet P, eds. International Textbook of Diabetes Mellitus. Sussex: John Wiley; 2004 (in press).
61. Garg A, Peshock RM, Fleckenstein JL. Adipose tissue distribution pattern in patients with familial partial lipodystrophy (Dunnigan variety). J Clin Endocrinol Metab. 1999;84:170-174.
62. Garg A, Vinaitheerthan M, Weatherall P, Bowcock A. Phenotypic heterogeneity in patients with familial partial lipodystrophy (Dunnigan variety) related to the site of mis-sense mutations in Lamin A/C (LMNA) gene. J Clin Endocrinol Metab. 2001;86:59-65.
63. Gellis SS, Green S, Walker D. Chronic renal disease in children with lipodystrophy. AMA J Dis Child. 1958;96:605-606.
64. Gerhartz H. Lipodystrophia progressiva superior. Munch Med Wschr. 1916;63:823-825.
65. Gerstman J. Lipodystrophia progressiva. Wein Klin Wschr. 1916;29:1209-1212.
66. Gibson R. The occurrence of progressive lipodystrophy in mental defectives. Can Med Assoc J. 1957;77:217-219.
67. Goossens S, Coessens B. Facial contour restoration in Barraquer-Simons syndrome using two free TRAM flaps: Presentation of two case reports and long-term follow-up. Microsurgery. 2002;22:211-218.
68. Gorlin RJ, Cervenka J, Moller K, Horrobin M, Witkop J. Rieger anomaly and growth retardation (The S-H-O-R-T syndrome). Birth Defects. 1975;11:46-48.
69. Grigovich IN, Sharapov NV, Grigovich LN. Bolezn' Barrakera-Simonsa (progressiruiushchaia lipodistrofiia) u detei. Vopr Okhr Materin Det. 1977;22:83-84.
70. Guelinckx PJ, Sinsel NK. Facial contour restoration in Barraquer-Simons syndrome using two free anterolateral thigh flaps. Plast Reconstr Surg. 2000;105:1730-1736.
71. Guralnick R, Green H. Lipodystrophia facialis. N Engl J Med. 1939;220:553-556.
72. Gurbuz O, Yucelten D, Ergun T, Khalilazer R. Partial lipodystrophy. Int J Dermatol. 1995;34:36-37.
73. Habib R, Levy M, Gubler MC, Broyer M, Habib M. Lipodystrophie partielle, hypocomplementemie et glomerulonephrite. Arch Fr Pediatr. 1977;34(7 Suppl):197-212.
74. Hagiwara M, Fukaya H, Nagata Y, Hoshika A, Aritaki S. Partial lipodystrophy-a case report. Horumon To Rinsho. 1983;31(Suppl):63-66.
75. Halazonetis J. A case of progressive lipodystrophy. Barraquer-Simon's disease. Br J Oral Surg. 1968;6:130-133.
76. Harris JS, Reiser R. Lipodystrophy. Report of a case with metabolic studies. Am J Dis Child. 1940;59:143-166.
77. Hartston W. Lipodystrophia progressiva. Lancet. 1933;2:1416-1418.
78. Haxton MJ. Progressive partial lipodystrophy in association with intrauterine death and growth retardation. Am J Obstet Gynecol. 1983;147:837-838.
79. Hegasy GA, Manuelian T, Hogasen K, Jansen JH, Zipfel PF. The molecular basis for hereditary porcine membranoproliferative glomerulonephritis type 11: point mutations in the factor H coding sequence block protein secretion. Am J Pathol. 2002;161:2027-2034.
80. Herrman C. Progressive lipodystrophy. Arch Intern Med. 1916;17:516-524.
81. Hogasen K, Jansen JH, Mollnes TE, Hovdenes J, Harboe M. Hereditary porcine membranoproliferative glomerulonephritis type 11 is caused by factor H deficiency. J Clin Invest. 1995;95:1054-1061.
82. Hollander E. Ueber einen fall von fortschreitend fettgewebes und seinen kosmetisch menschenfett. Munch Med Wschr. 1910;7:1794-1795.
83. Houssin A, Saint-Andre JP, Buzelin F, Guenel J. Lipodystrophie partielle faciotronuclaire avec glomerulopathie asymptomatique du type maladie des depots denses. Ann Med Interne (Paris). 1981;132:44-47.
84. Hunter AM, Lawson AA, Thomson D. Partial lipodystrophy with nephrotic syndrome. Postgrad Med J. 1978;54:286-291.
85. Hurwitz PJ, Sarel R. Facial reconstruction in partial lipodystrophy. Ann Plast Surg. 1982;8:253-257.
86. Ipp MM, Minta JO, Gelfand EW. Disorders of the complement system in lipodystrophy. Clin Immunol Immunopathol. 1977;7:281-287.
87. Jackson AS, Pollock ML, Ward A. Generalized equations for predicting body density of women. Med Sci Sports Exerc. 1980;12:175-181.
88. Jasin HE. Systemic lupus erythematosus, partial lipodystrophy and hypocomplementemia. J Rheumatol. 1979;6:43-50.
89. Jolowicz E. Lipodystrophia progressiva. Neurol Zbl. 1915;34:930-934.
90. Jones E. Progressive Lipodystrophy. Br Med J. 1956;1:313-319.
91. Kumar R, Seema R, Aneja S, Seth A, Taluja V. Partial lipodystrophy in a boy. Indian Pediatr. 2000;37:93-96.
92. Laignel-Lavastine M, Viard M. Adipose segmentaire des membres inferieurs. Nouvelle Iconographies de la Salpetriere. 1912;25:473-482.
93. Lajouanine P, Canet J, Pesnel G, Rencki-Barre MJ. Les nephropathies dans la lipodystrophie progressive. Ann Pediatr. 1968;15:33-41.
94. Laxenaire M, Weber M, Tridon P. Familial epilepsy and progressive partial lipodystrophy. (Barraquer-Simons disease). Epilepsia. 1969;10:87-90.
95. Leipoldt CL. Notes on a case of lipodystrophia progressiva. Med J S Afr. 1920;15:161-165.
96. Lenane P, Murphy G. Partial lipodystrophy and renal disease. Clin Exp Dermatol. 2000;25:605-607.
97. Levy M, Halbwachs-Mecarelli L, Gubler MC, Kohout G, Bensenouci A, Niaudet P, Hauptmann G, Lesavre P. H deficiency in two brothers with atypical dense intramembranous deposit disease. Kidney Int. 1986;30:949-956.
98. Liashchuk PM. Barraquer-Simons syndrome. Klin Med (Mosk). 1984;62:130-131.
99. Liubashevskii RI, Erman LV. [Progressive lipodystrophy in a 7 and one half year old boy. (Simons-Hollander disease)]. Pediatriia. 1969;48:91-92.
100. Ljunghall S, Fjellstrom KE, Wibell L. Partial lipodystrophy and chronic hypocomplementemic glomerulonephritis. Acta Med Scand. 1974;195:493-497.
101. Mahieu P. Biochemical structure of glomerular basement membrane in chronic glomerulonephritis. 1. Lobular and membrano-proliferative glomerulonephritis. Kidney Int. 1972;1:115-123.
102. Mathieson PW, Wurzner R, Oliveria DB, Lachmann PJ, Peters DK. Complement-mediated adipocyte lysis by nephritic factor sera. J Exp Med. 1993;177:1827-1831.
103. a. McKusick VA. Mendelian Inheritance in Man: A Catalog of Human Genes and Genetic Disorders. 12th ed. Baltimore: Johns Hopkins University Press; 1998. The online version is accessible from the National Center for Biotechnology Information, National Library of Medicine, http://www.ncbi.nlm.nih.gov/omim/.
104. McLean RH, Hoefnagel D. Partial lipodystrophy and familial C3 deficiency. Hunt Hered. 1980;30:149-154.
105. McLean RH, Siegel NJ, Kashgarian M. Activation of the classic complement pathway in patients with the C3 nephritic factor. Nephron. 1980;25:57-64.
106. McNeill AD. Progressive lipodystrophy. Br J Surg. 1966;53:216-218.
107. Meri S, Koistinen V, Miettinen A, Tornroth T, Seppala IJ. Activation of the alternative pathway of complement by monoclonal lambda light chains in membranoproliferative glomerulonephritis. J Exp Med. 1992;175:939-950.
108. Merino J, Rodriguez-Valverde V, Lamelas JA, Riestra JL, Casanueva B. Prevalence of deficits of complement components in patients with recurrent meningococcal infections. J Infect Dis. 1983;148:331.
109. Mery JP, Kourilsky O, Morel-Maroger L, Adam C. Partial lipodystrophy and glomerulonephritis without complement activation. N Engl J Med. 1978;298:1034.
110. Meyrier A. The patient with glomerulonephritis and lipodystrophy. Nephrol Dial Transplant. 1997;12:226-227.
111. Misra A, Garg A. Clinical features and metabolic derangements in acquired generalized lipodystrophy: case reports and review of literature. Medicine (Baltimore). 2003;82:129-146.
112. Mitchell SW. Singular case of absence of adipose tissue matter in the upper half of the body. Am J Med Sci. 1885;90:105-106.
113. Murray I. Lipodystrophy. Br Med J. 1952;2:1236-1239.
114. Myers B, Chambers R, Anderson A, Barry R, Wallington T, Whicher J. C3 nephritic factor in an individual with recurrent viral infection and lipodystrophy. J Clin Lab Immunol. 1986;19:201-204.
115. Neel AV. Et Tilfaelde af lipodystrophia progressiva. Hospitalstidende. 1916;59:1253-1265.
116. Ng YC, Peters DK. C3 nephritic factor (C3NeF): dissociation of cell-bound and fluid phase stabilization of alternative pathway C3 convertase. Clin Exp Immunol. 1986;65:450-457.
117. O'Mahony D, O'Mahony S, Whelton MJ, McKieman J. Partial lipodystrophy in coeliac disease. Gut. 1990;31:717-718.
118. Orrell RW, Peatfield RC, Collins CE, Woodrow DF, Moss J, Press M, Lane RJ. Myopathy in acquired partial lipodystrophy. Clin Neurol Neurosurg. 1995;97:181-186.
119. Pangburn MK, Muller-Eberhard HJ. The C3 convertase of the alternative pathway of human complement. Enzymic properties of the bimolecular proteinase. Biochem J. 1986;235:723-730.
120. Parmalee AH. Lipodystrophy. A report of six cases in children. JAMA. 1932;98:548-552.
121. Perrot H, Delaup JP, Chouvet B. Barraquer and Simons lipodystrophy. Complement anomalies and cutaneous leukocytoclastic vasculitis. Ann Dermatol Venereol. 1987;114:1083-1091.
122. Peters DK, Charlesworth JA, Sissons JG, Williams DG, Boulton-Jones JM, Evans DJ, Kourilsky O, Morel-Maroger L. Mesangiocapillary nephritis, partial lipodystrophy, and hypocomplementaemia. Lancet. 1973;2:535-538.
123. Peters DK, Williams DG. Complement and mesangiocapillary glomerulonephritis role of complement deficiency in the pathogenesis of nephritis. Nephrol. 1974;13:188-197.
124. Pickering MC, Cook HT, Warren J, Bygrave AE, Moss J, Walport MJ, Botto M. Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H. Nat Genet. 2002;31:424-428.
125. Piscatelli RL, Vieweg WV, Havel RJ. Partial lipodystrophy. Metabolic studies in three patients. Ann Intern Med. 1970;73:963-970.
126. Pollock J, Wood B, Kelly JP. Membranoproliferative glomerulonephritis, type II and partial lipodystrophy in an adult. Am J Kidney Dis. 1986;8:274-276.
127. Power DA, Ng YC, Simpson JG. Familial incidence of C3 nephritic factor, partial lipodystrophy and membranoproliferative glomerulonephritis. Q J Med. 1990;75:387-398.
128. Premkumar A, Chow C, Bhandarkar P, Wright V, Koshy N, Taylor S, Arioglu E. Lipoatrophic-lipodystrophic syndromes: the spectrum of findings on MR imaging. AJR Am J Roentgenol. 2002;178:311-318.
129. Prodinger WH, Wurzner R, Erdei A, Dierich MP. Complement. In: Paul WR, ed. Fundamental Immunology. 4th ed. Philadelphia: Lippincott-Raven; 1999:967-995.
130. Pussell BA, Bourke E, Nayef M, Morris S, Peters DK. Complement deficiency and nephritis. A report of a family. Lancet. 1980;1:675-677.
131. Quecedo E, Febrer I, Serrano G, Martinez-Aparicio A, Aliaga A. Partial lipodystrophy associated with juvenile dermatomyositis: report of two cases. Pediatr Dermatol. 1996;13:477-482.
132. Rees TD, Ashley FL. Treatment of facial atrophy with liquid silicone. Am J Surg. 1966;111:531-535.
133. Rees TD, Ashley FL, Delgado JP. Silicone fluid injections for facial atrophy. A ten-year study. Plast Reconstr Surg. 1973;52:118-127.
134. Rees TD, Coburn RJ. Silicone treatment of partial lipodystrophy. JAMA. 1974;230:868-870.
135. Reichel W, Kobberling J, Fischbach H, Scheler F. Membranoproliferative glomerulonephritis with partial lipodystrophy: discordant occurrence in identical twins. Klin Wschr. 1976;54:75-81.
136. Reuben MS, Zamkin HO, Fox HR. Lipodystrophia progressiva. Arch Pediatr. 1924;41:480-488.
137. Richardson JS. Lipodystrophy. Proc R Soc Med. 1946;39:704.
138. Robertson DA, Wright R. Cirrhosis in partial lipodystrophy. Postgrad Med J. 1989;65:318-320.
139. Ruhl CE, Everhart JE. Leptin concentrations in the United States: relations with demographic and anthropometric measures. Am J Clin Nutr. 2001;74:295-301.
140. Russell JGB. Lipodystrophy progressiva and pregnancy. Postgrad Med J. 1958;34:530-534.
141. Sacks SH, Zhou W, Sheerin NS. Complement synthesis in the injured kidney: does it have a role in immune complex glomerulonephritis? J Am Soc Nephrol. 1996;7:2314-2319.
142. Sanchez Muros J. Lipodistrofia de Barraquer-Simons. Actas Dermosifiliogr. 1971;62:305-310.
143. Sapelkina LV, Isakova GL. [Progressive lipodystrophy (Barraquer-Simons' syndrome)]. Vopr Okhr Materin Det. 1971;16:73-75.
144. Schifferli JA, Blanc E. Partial lipodystrophy, meningococcal meningitis and nephritis. Dermatologica. 1986;173:9-12.
145. Schreiber RD, Medicus RG, Gitze O, Muller-Eberhard HJ. Properdinand nephritic factor-dependent C3 convertases: requirement of native C3 for enzyme formation and the function of bound C3b as properdin receptor. J Exp Med. 1975;142:760-772.
146. Schwenke J. Ein fall von lipodystrophia progressiva. Dtsch Med Wochenschr. 1922;48:292-293.
147. Schwingshandl J, Mache CJ, Rath K, Borkenstein MH. SHORT syndrome and insulin resistance. Am J Med Genet. 1993;47:907-909.
148. Scott DM, Amos N, Sissons JG, Lachmann PJ, Peters DK. The immunoglobulin nature of nephritic factor (NeF). Clin Exp Immunol. 1978;32:12-24.
149. Seip M, Trygstad O. Generalized lipodystrophy, congenital and acquired (lipoatrophy). Acta Paediatr Suppl. 1996;413:2-28.
150. Senior B, Gellis SS. The syndromes of total lipodystrophy and of partial lipodystrophy. Pediatrics. 1964;33:593-612.
151. Sensenbrenner JA, Hussels IE, Levin LS. CC-a low birthweight syndrome, Reiger syndrome. Birth Deffects. 1975;11:423-426.
152. Serra JM, Ballesteros A, Mesa F, Bazan A, Paloma V, Sanz J. Use of the temporal muscle flap in Barraquer-Simon's progressive lipodystrophy. Ann Plast Surg. 1993;30:180-182.
153. Simha V, Garg A. Body fat distribution and metabolic derangements in patients with familial partial lipodystrophy associated with mandibuloacral dysplasia. J Clin Endocrinol Metab. 2002;87:776-785.
154. Simons A. Lipodystrophia progressiva. Z ges Neurol Psychiat. 1911;5:29-38.
155. Singh A, Krishan I. Progressive lipodystrophy in a male. A case report. Indian J Med Sci. 1970;24:149-151.
156. Singhal PC, Sakhuja V, Jain SK, Chugh KS. Partial lipodystrophy, hypocomplementaemia and dense deposit disease. J Indian Med Assoc. 1983;81:201-202.
157. Sissons JG, West RJ, Fallows J, Williams DG, Boucher BJ, Amos N, Peters DK. The complement abnormalities of lipodystrophy. N Engl J Med. 1976;294:461-465.
158. Smak Gregoor PJ, van Bommel EF, Ketterings C, van Saase JL, Kramer P. Progressive lipodystrophy, a diagnosis at a glance. Nephrol Dial Transplant. 1998;13:507-509.
159. Smith HL. Lipodystrophia progressiva. Bull Johns Hopkins Hosp. 1921;32:344-350.
160. Smith PM, Morgans ME, Clark CG, Lennard-Jones JE, Gunnlaugsson O, Jonasson TA. Lipodystrophy, pancreatitis, and eosinophilia. Gut. 1975;16:230-234.
161. Sniderman AD, Cianflone K. The adipsin-ASP pathway and regulation of adipocyte function. Ann Med. 1994;26:388-393.
162. Sokolova LA. Free adipo-dermal grafts in the surgical treatment of progressive lipodystrophy. Acta Chir Plast. 1972;14:157-164.
163. Sorge G, Ruggieri M, Polizzi A, Scuderi A, Di Pietro M. SHORT syndrome: a new case with probable autosomal dominant inheritance. Am J Med Genet. 1996;61:178-181.
164. Spear IJ. Lipodystrophia progressiva. Arch Intern Med. 1918;21:39-47.
165. Spitzer RE, Vallota EH, Forristal J, Sudora E, Stitzel A, Davis NC, West CD. Serum C'3 lytic system in patients with glomerulonephritis. Science. 1969;164:436-437.
166. Steinberg T, Gwinup G. Lipodystrophy, a variant of lipoatrophic diabetes. Diabetes. 1967;16:715-721.
167. Strauch A. Progressive lipodystrophy. JAMA. 1922;78:1037-1038.
168. Taylor WB, Honeycutt WM. Progressive lipodystrophy and lipoatrophic diabetes. Arch Dermatol. 1961;84:31-36.
169. Teisner B, Elling P, Svehag SE, Poulsen L, Lamm LU, Sjoholm A. C3 nephritic factor in a patient with recurrent Neisseria meningitidis infections. Acta Pathol Microbiol Immunol Scand [C]. 1984;92:341-349.
170. Thompson RA, Yap PL, Brettle RB, Dunmow RE, Chapel H. Meningococcal meningitis associated with persistent hypocomplementaemia due to circulating C3 nephritic factor. Clin Exp Immunol. 1983;52:153-156.
171. Toriello HV, Wakefield S, Komar K, Higgins JV, Waterman DF. Report of a case and further delineation of the SHORT syndrome. Am J Med Genet. 1985;22:311-314.
172. Torrelo A, Espana A, Boixeda P, Ledo A. Partial lipodystrophy and dermatomyositis. Arch Dermatol. 1991;127:1846-1847.
173. van der Wal KG, Mulder JW. Facial contour reconstruction in partial lipodystrophy using two temporalis muscle flaps. A case report. Int J Oral Maxillofac Surg. 1998;27:14-16.
174. Vargas R, Thomson KJ, Wilson D, Cameron JS, Turner DR, Gill D, Chantler C, Ogg CS. Mesangiocapillary glomerulonephritis with dense "deposits" in the basement membranes of the kidney. Clin Nephrol. 1976;5:73-82.
175. Verger P, Guillard JM, Theodorides M, Guinard F. Lipodystrophie partielle (syndrome de Barraquer-Simmons incomplete). J Med Bord. 1967;144:723-728.
176. Walker UA, Kirschfink M, Peter HH. Improvement of acquired partial lipodystrophy with rosiglitazone despite ongoing complement activation. Rheumatology. 2003;42:393-394.
177. Walport MJ. Complement. First of two parts. N Engl J Med. 2001;344:1058-1066.
178. Walport MJ, Davies KA, Botto M, Naughton MA, Isenberg DA, Biasi D, Powell RJ, Cheung NT, Struthers GR. C3 nephritic factor and SLE: report of four cases and review of the literature. Q J Med. 1994;87:609-615.
179. Warin RP. Progressive lipodystrophy. Report of two cases. Lancet. 1950;2:55-57.
180. Watson WNB, Ritchie WT. Progressive lipodystrophy. Q J Med. 1925;18:225-239.
181. Wayte J, Bird G, Wilkinson JD. The clinical significance of partial lipoatrophy and C3 hypocomplementaemia: a report of two cases. Clin Exp Dermatol. 1996;21:131-134.
182. Weber FP. Lipodystrophia progressiva. Q J Med. 1916;10:131-139.
183. Weber P, Gunewardene TH. A new case of lipodystrophia progressiva. Br J Child Dis. 1919;16:89-91.
184. Wertham FI. Habitus lipodystrophicus with effective psychosis (hypomanic excitement). Arch Neurol Psychiat. 1929;22:714-718.
185. West CD, McAdams AJ. The alternative pathway C3 convertase and glomerular deposits. Pediatr Nephrol. 1999;13:448-453.
186. West RJ, Fosbrooke AS, Lloyd JK. Metabolic studies and autonomic function in children with partial lipodystrophy. Arch Dis Child. 1974;49:627-632.
187. White RT, Damm D, Hancock N, Rosen BS, Lowell BB, Usher P, Flier JS, Spiegelman BM. Human adipsin is identical to complement factor D and is expressed at high levels in adipose tissue. J Biol Chem. 1992;267:9210-9213.
188. Williams DG, Bartlett A, Duffus P. Identification of nephritic factor as an immunoglobulin. Clin Exp Immunol. 1978;33:425-429.
189. Williams DG, Scopes JW, Peters DK. Hypocomplementaemic membranoproliferative glomerulonephritis and nephrotic syndrome associated with partial lipodystrophy of the face and trunk. Proc R Soc Med. 1972;65:591.
190. Yamada A, Ohi H, Okano K, Watanabe S, Seki M, Hatano M, Koitabashi Y. Production of C3 nephritic factor by cultured lymphocytes derived from a patient with partial lipodystrophy. J Clin Lab Immunol. 1988;27:35-37.
191. Yuh WT, Collison JS, Sickels WJ, Barloon TJ, Brennan DC, Flanigan MJ. Partial lipodystrophy. Magnetic resonance findings in one case. J Comput Tomogr. 1988;12:287-291.
192. Zafarulla MY. Lipodystrophy: a case report of partial lipodystrophy. Br J Oral Maxillofac Surg. 1985;23:53-57.
193. Ziegler LH. Lipodystrophies: report of seven cases. Brain. 1928;51:145-167.
194. Ziegler LH, Prout CT. Neuropsychiatric aspects of lipodystrophic disturbances. Am J Psychiatry. 1928;7:709-714.