anomalie scheletriche: condrodisplasia puntata (foci iperecogeni) interessante rotula e anca
anomalie del SNC: agenesia del corpo calloso
epatomegalia
Possono essere presenti ritardo di crescita, microcefalia, micrognazia, palato ogivale.
Bibliografia
Dancis, J., Hutzler, J. The significance of hyperpipecolatemia in Zellweger syndrome. Am. J. Hum. Genet. 38: 707-711, 1986. Ebberink, M. S., Mooijer, P. A. W., Gootjes, J., Koster, J., Wanders, R. J. A., Waterham, H. R. Genetic classification and mutational spectrum of more than 600 patients with a Zellweger syndrome spectrum disorder. Hum. Mut. 32: 59-69, 2010. Gilchrist, K. W., Gilbert, E. F., Shahidi, N. T., Opitz, J. M. The evaluation of infants with the Zellweger (cerebro-hepato-renal) syndrome. Clin. Genet. 7: 413-416, 1975. Naritomi, K., Hyakuna, N., Suzuki, Y., Orii, T., Hirayama, K. Zellweger syndrome and a microdeletion of the proximal long arm of chromosome 7. Hum. Genet. 80: 201-202, 1988. Naritomi, K., Izumikawa, Y., Ohshiro, S., Yoshida, K., Shimozawa, N., Suzuki, Y., Orii, T., Hirayama, K. Gene assignment of Zellweger syndrome to 7q11.23: report of the second case associated with a pericentric inversion of chromosome 7. Hum. Genet. 84: 79-80, 1989. Opitz, J. M., ZuRhein, G. M., Vitale, L., Shahidi, N. T., Howe, J. J., Chou, S. M., Shanklin, D. R., Sybers, H. D., Dood, A. R., Gerritsen, T. The Zellweger syndrome (cerebro-hepato-renal syndrome). Birth Defects Orig. Art. Ser. V(2): 144-160, 1969. Tamura, S., Okumoto, K., Toyama, R., Shimozawa, N., Tsukamoto, T., Suzuki, Y., Osumi, T., Kondo, N., Fujiki, Y. Human PEX1 cloned by functional complementation in a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I. Proc. Nat. Acad. Sci. 95: 4350-4355, 1998. Volpe, J. J., Adams, R. D. Cerebro-hepato-renal syndrome of Zellweger: an inherited disorder of neuronal migration. Acta Neuropath. 20: 175-198, 1972. Wanders, R. J. A., Schrakamp, G., van den Bosch, H., Tager, J. M., Schutgens, R. B. H. A prenatal test for the cerebro-hepato-renal (Zellweger) syndrome by demonstration of the absence of catalase-containing particles (peroxisomes) in cultured amniotic fluid cells. Europ. J. Pediat. 145: 136-138, 1986. Wanders, R. J. A., van Roermund, C. W. T., Schutgens, R. B. H., Barth, P. G., Heymans, H. S. A., van den Bosch, H., Tager, J. M. The inborn errors of peroxisomal beta-oxidation: a review. J. Inherit. Metab. Dis. 13: 4-36, 1990. Wilson, G. N., Holmes, R. G., Custer, J., Lipkowitz, J. L., Stover, J., Datta, N., Hajra, A. Zellweger syndrome: diagnostic assays, syndrome delineation, and potential therapy. Am. J. Med. Genet. 24: 69-82, 1986