Structural and histomorphometric studies of iliac crest trabecular and cortical bone in autosomal dominant osteopetrosis: a study of two radiological types
J Bollerslev, T Steiniche, F Melsen, L Mosekilde - Bone, 1989 - Elsevier
J Bollerslev, T Steiniche, F Melsen, L Mosekilde
Bone, 1989•ElsevierCylindrical iliac crest biopsies were obtained from 16 patients with autosomal dominant
osteopetrosis after intravital double labeling with tetracycline, and compared with normal
age-and sex-matched controls. Ten patients had the radiological type I (5 women, 5 men,
aged 17–62 years, mean 42) characterized by diffuse, symmetrical osteosclerosis and
enlarged thickness of the cranial vault. Six patients had type II (2 women, 4 men, aged 22–
44 years, mean 36), where “Rugger Jersey Spine” and endobone are characteristic findings …
osteopetrosis after intravital double labeling with tetracycline, and compared with normal
age-and sex-matched controls. Ten patients had the radiological type I (5 women, 5 men,
aged 17–62 years, mean 42) characterized by diffuse, symmetrical osteosclerosis and
enlarged thickness of the cranial vault. Six patients had type II (2 women, 4 men, aged 22–
44 years, mean 36), where “Rugger Jersey Spine” and endobone are characteristic findings …
Abstract
Cylindrical iliac crest biopsies were obtained from 16 patients with autosomal dominant osteopetrosis after intravital double labeling with tetracycline, and compared with normal age- and sex-matched controls. Ten patients had the radiological type I (5 women, 5 men, aged 17–62 years, mean 42) characterized by diffuse, symmetrical osteosclerosis and enlarged thickness of the cranial vault. Six patients had type II (2 women, 4 men, aged 22–44 years, mean 36), where “Rugger Jersey Spine” and endobone are characteristic findings.
Structural studies of cortical and trabecular bone were performed, and trabecular bone resorption and formation rates were studied using dynamic histomorphometry. The total biopsy length (C. Wi) were increased in type I (p < 0.05), and unchanged in type II. Both types showed increased cortical width (Ct. Wi) (p < 0.01 and p < 0.05, respectively), and decreased fractional width of cancellous bone (Cn.Wi/C.Wi) (p < 0.01 and p < 0.05).
The fractional trabecular bone volume (BV/TV) and trabecular thickness (Tb. Th) were both significantly increased in type I (p < 0.05), while resorptive and formative indices of trabecular bone remodeling were normal. No difference was found in trabecular bone balance, which was slightly positive in both patients and controls.
In type II osteopetrosis the eroded surfaces (OS/BS) were significantly increased (p < 0.01), as was the total resorptive period RP) (p < 0.05). The resorption depth (R.D.) was normal, while the resorption rate (MRR) was insignificantly decreased. Many big multinucleated osteoclasts were seen in this type suggesting defective resorptive function.
It is concluded that there seems to be a modeling defect at the endosteal envelope in both types of osteopetrosis leading to defective endosteal bone resorption, while trabecular bone remodeling is normal in type I. The periosteal bone formation may be increased in this type. The trabecular bone remodeling is disturbed in type II, where the study demonstrates sign of an increased resorption area, but decreased sinle cell resorption rate.
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