Radiological and histological improvement of oxalate osteopathy after combined liver-kidney transplantation in primary hyperoxaluria type 1.
A 15-year-old patient with severe bone disease (with bilateral fractures of hips and shoulders) due to primary hyperoxaluria type 1 (PH1) was treated with combined liver-kidney transplantation after a 4-year hemodialysis period. Normalization of excessive oxalate synthesis brought in by the liver graft combined with the slow excretion of skeletal oxalate stores by the renal graft led to progressive improvement of clinical, radiological, and histological evidence of oxalate osteopathy. This allowed bilateral hip replacement 3 years after transplantation, which led to complete physical rehabilitation of the crippled patient. Combined liver-kidney transplantation constitutes the treatment of choice for end-stage renal failure due to PH1, even in the face of severe oxalate bone disease.
Toussaint
C
C
Cliniques Universitaires de Bruxelles, Hôpital Erasme, Belgium.
De Pauw
L
L
Vienne
A
A
Gevenois
P A
PA
Quintin
J
J
Gelin
M
M
Pasteels
J L
JL
D000293:Adolescent; D015519:Bone Density; D001851:Bone Diseases, Metabolic / Q000209:etiology* / Q000601:surgery; D001842:Bone and Bones / Q000737:chemistry / Q000473:pathology; D003404:Creatinine / Q000378:metabolism; D005260:Female; D005270:Femur Head / Q000737:chemistry / Q000473:pathology; D006620:Hip Fractures / Q000209:etiology; D006622:Hip Prosthesis; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000150:complications / Q000601:surgery*; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000601:surgery; D016030:Kidney Transplantation; D016031:Liver Transplantation; D010070:Oxalates / Q000032:analysis / Q000097:blood / Q000378:metabolism; D012784:Shoulder Fractures / Q000209:etiology
D010070:Oxalates; D003404:Creatinine