SKELETAL MANIFESTATIONS OF PRIMARY OXALOSIS

被引：25

作者：

SCHNITZLER, CM

KOK, JA

JACOBS, DWC

THOMSON, PD

MILNE, FJ

MESQUITA, JM

KING, PC

FABIAN, VA

机构：

[1] Department of Orthopaedic Surgery and M R C Mineral Metabolism Research Unit, University of the Witwatersrand, Parktown, Johannesburg, 2193

[2] Department of Medicine, University of the Witwatersrand, Parktown, Johannesburg, 2193

[3] Department of Paediatrics, University of the Witwatersrand, Parktown, Johannesburg, 2193

[4] School of Pathology, University of the Witwatersrand, Parktown, Johannesburg, 2193

来源：

PEDIATRIC NEPHROLOGY | 1991年 / 5卷 / 02期

关键词：

OXALOSIS; BONE; HISTOLOGY; PATHOLOGICAL FRACTURE;

D O I：

10.1007/BF01095951

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

We describe the clinical, radiographic and histological features of skeletal involvement in four patients with end-stage renal failure due to primary oxalosis. The clinical features were unrelenting bone pain, and in two patients multiple fractures. Radiographic features were, in chronological order: (1) radiodense metaphyses and other red marrow bone; (2) cortical defects in metaphyses; (3) spontaneous fracture-separations of epiphyses of long limb bones which healed poorly. The fractures occurred through crystal deposits, and fracture displacement was associated with extrusion of crystalline material from bone. On histological examination crystals were found to replace metaphyseal bone. Pericrystalline giant cell granulomata replaced bone marrow. Erosion surfaces near granulomas were increased. Subperisteal and intra-osseous tophi of calcium oxalate were seen. Calcium oxalate appears to precipitate with greater facility than does physiological mineral. Bone showed the features of mixed uraemic osteodystrophy in all four patients. We conclude that: (1) the fractures occurred through heavy crystal deposits; (2) ununited fractures and intra-osseous and subperiosteal tophi contributed to the pain; (3) spontaneous fractures are of poor prognostic significance. We recommend that unstable fractures be internally fixed.

引用

页码：193 / 199

页数：7

共 26 条

[1]

Willia H.E., Smith L.H., Primary hyperoxaluria, The metabolic basis of inherited disease, pp. 182-204, (1978)

[2]

Hug I., Mihatch J.M., Die primäre Oxalose, Fortschr Röntgenstr, 123, pp. 154-162, (1975)

[3]

Lagier R., Revell P., Schoenboerner A., Calcium oxalate deposition in growing bone: anatomical and radiological study in a case of primary oxalosis, Metab Bone Dis Rel Res, 4, pp. 49-59, (1982)

[4]

Benhamou C.L., Pierre D., Geslin N., Viala J.F., Maitre F., Chavassieux P., Edouard C., Meunier P.J., Case report. Primary bone oxalosis: the roles of oxalate deposits and renal osteodystrophy, Bone, 8, pp. 59-64, (1987)

[5]

Gherardi G., Poggi A., Sisca S., Calderaro V., Bonucci E., Bone oxalosis and renal osteodystrophy, Arch Pathol Lab Med, 104, pp. 105-111, (1980)

[6]

Branaccio D., Poggi A., Cicarelli C., Bellini F., Galmozzi C., Poletti I., Maggiore Q., Bone changes in end-stage oxalosis, A J R, 136, pp. 935-939, (1981)

[7]

Mathews M., Stauffer M., Cameron E.C., Maloney N., Sherrard D.J., Bone biopsy to diagnose hyperoxaluria in patients with renal failure, Ann Intern Med, 90, pp. 777-779, (1979)

[8]

Kalifa G., Dossans B., Gagnadoux M.F., Sauvegrain J., Aspects radiologiques de l'oxalose, J Radiol, 60, pp. 45-49, (1979)

[9]

Jahn H., Frank R.M., Voegel J.C., Schohn D., Scanning electron microscopy and X-ray diffraction studies of human bone oxalosis, Calcif Tissue Int, 30, pp. 109-119, (1980)

[10]

Jacobs C., Rottembourgh J., Reachi, Le Rain M., Terminal renal failure due to oxalosis in 14 patients, Proc Eur Dial Transplant Assoc, 18, pp. 359-365, (1974)

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