Abstract
The first description of lipogranulomatosis was published by Farber in 1952 (8). On the basis of the findings in three children, two of whom were siblings he described the clinical and pathological features of this rare systemic disorder. At present eight additional cases, including the present one have been reported (1,3,4,6,7,17, 25). It has been suggested that the disease is inherited in an autosomal recessive manner (7).
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Abul-Haj, S.K., Martz, D.G., Douglas, W.F. and Geppert, L.J. Farber’s disease. Report of a case with observations on its histogenesis and notes on the nature of the stored material. J. Pediat. 61: 221 (1962).
Adams, C.W.M. Neurohistochemistry. Elsevier Publishing Co., Amsterdam, 1965.
Azanza, X. Une Nouvelle Neurolipidose. La Maladie de Farber (Thesis). Editions Bergerel, Bordeaux, 1969.
Bierman, S.M., Edgington, T., Newcomer, V.D. and Pearson, C.M. Farber’s disease: A disorder of mucopolysaccharide metabolism with articular respiratory and neurologic manifestations. Arthr. and Rheum. 9: 620 (1966).
Brady, R.O. Cerebral lipidoses. Amer. Rev. Med. 21: 317 (1970).
Clausen, J. and Rampini, S. Chemical studies of Farber’s disease. Acta neurol. scand. 46: 313 (1970).
Crocker, A.C., Cohen, J. and Farber, S. The “lipogranulomatosis” syndrome; Review, with report of patient showing milder involvement. In: Aronson, S.M. and Volk, B.W. (eds) Inborn Disorders of Sphingolipid Metabolism. Pergamon Press Ltd., Oxford, 1967, pp. 485–503.
Farber, S. A lipid metabolic disorder-disseminated “Lipogranulomatosis” - a syndrome with similarity to and important difference from, Niemann-Pick and Hand-Schuller-Christian disease. Amer. J. Dis. Child. 84: 499 (1952).
Farber, S., Cohen, J. and Uzman, L.L. Lipogranulomatosis. A new lipo-glyco-protein “storage” disease. J. Mt Sinai Hosp. 24: 816 (1957).
Gatt, S. Enzymatic aspects of sphingolipid degradation. In: Sweeley, C.C. (ed) Chemistry and Metabolism of Sphingolipids. North Holland Publishing Co., Amsterdam, 1970, pp. 235–249.
Hammarström, S. Gas-liquid chromatography-mass spectrometry of synthetic ceramides containing phytosphingosine. J. Lipid Res. 11: 175 (1970).
Hammarström, S., Samuelsson, B. and Samuelsson, K. Gas-liquid chromatography-mass spectrometry of synthetic ceramides containing 2-hydroxy acids. J. Lipid Res. 11: 150 (1970).
Ivemark, B.I. Lipid histochemical findings in juvenile lipogranulomatosis (Farber). Proceedings of the 5th annual meeting of the Scandinavian Society of Pediatric Pathology in Helsinki, June 10–12, 1971. Acta paediat. scand. In press.
Lauter, C.J. and Trams, E.G. A spectrophotometric determination of sphingosine. J. Lipid Res. 3: 136 (1962).
Morell, P., Costantino-Ceccarini, E., and Radin, N.S. The biosynthesis by brain microsomes of cerebrosides containing nonhydroxy fatty acids. Arch. Biochem. 141: 738 (1970). And references therein.
Moser, H.W., Prensky, A.L., Wolfe, H.J. and Rosman, N.P. Farber’s lipogranulomatosis. Report of a case and demonstration of an excess of free ceramide and ganglioside. Amer. J. Med. 47: 869 (1969).
Prensky, A.L., Ferreira, G., Carr, S. and Moser, H.W. Ceramide and ganglioside accumulation in Farber’s lipogranulomatosis. Proc. Soc. exp. Biol. (N.Y.) 126: 725 (1967).
Samuelsson, B. and Samuelsson, K. Gas-liquid chromatographic separation of ceramides as di-0-trimethylsilyl ether derivatives. Biochim. biophys. Acta (Amst.) 164: 421 (1968).
Samuelsson, B. and Samuelsson, K. Gas-liquid chromatography-mass spectrometry of synthetic ceramides. J. Lipid Res. 10: 41 (1969).
Samuelsson, K. Identification and quantitative determination of ceramides in human plasma. Scand. J. clin. Lab. Invest. 27: 371 (1971).
Samuelsson, K. and Zetterström, R. Ceramides in a patient with lipogranulomatosis (Farber’s disease) with chronic course. Scand. J. clin. Lab. Invest. 27: 393 (1971).
Sribney, M. Enzymatic synthesis of ceramide. Biochim. biophys. Acta (Amst.) 125: 542 (1966).
Sribney, M. and Kennedy, E.P. The enzymatic synthesis of sphingomyelin. J. Biol. Chem. 233: 1315 (1958).
Stoffel, W. Studies on the biosynthesis and degradation of sphingosine bases. In: Sweeley, C.C. (ed) Chemistry and Metabolism of sphingolipids. North Holland Publishing Co., Amsterdam, 1970, pp. 139–158.
Zetterström, R. Disseminated lipogranulomatosis (Farber’s disease). Acta paediat. (Uppsala) 47: 501 (1958).
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1972 Springer Science+Business Media New York
About this chapter
Cite this chapter
Samuelsson, K., Zetterström, R., Ivemark, B.I. (1972). Studies on a Case of Lipogranulomatosis (Farber’s Disease) with Protracted Course. In: Volk, B.W., Aronson, S.M. (eds) Sphingolipids, Sphingolipidoses and Allied Disorders. Advances in Experimental Medicine and Biology, vol 19. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-6570-0_36
Download citation
DOI: https://doi.org/10.1007/978-1-4757-6570-0_36
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4757-6572-4
Online ISBN: 978-1-4757-6570-0
eBook Packages: Springer Book Archive