RESUMO
The aim of this study was to evaluate the effect of denosumab (Dmab) on bone mineral density (BMD) and bone turnover markers after 1 year of treatment. Additionally, the effect of Dmab in bisphosphonate-naïve patients (BP-naïve) compared to patients previously treated with bisphosphonates (BP-prior) was analyzed. This retrospective study included 425 postmenopausal women treated with Dmab for 1 year in clinical practice conditions in specialized centers from Argentina. Participants were also divided according to previous bisphosphonate treatment into BP-naïve and BP-prior. A control group of patients treated with BP not switched to Dmab matched by sex, age, and body mass index was used. Data are expressed as mean ± SEM. After 1 year of treatment with Dmab the bone formation markers total alkaline phosphatase and osteocalcin were significantly decreased (23.36% and 43.97%, resp.), as was the bone resorption marker s-CTX (69.61%). Significant increases in BMD were observed at the lumbar spine, femoral neck, and total hip without differences between BP-naïve and BP-prior. A better BMD response was found in BP-prior group compared with BP treated patients not switched to Dmab. Conclusion. Dmab treatment increased BMD and decreased bone turnover markers in the whole group, with similar response in BP-naïve and BP-prior patients. A better BMD response in BP-prior patients versus BP treated patients not switched to Dmab was observed.
RESUMO
Cammurati-Engelmann's Disease or Progressive Diaphyseal Dysplasia (PDD), is a rare autosomal dominant disorder, sometimes non hereditable, which begins in childhood, and is characterized by symmetrical excess of osseous apposition in diaphysis and metaphysis of long bones. In severe cases skull and vertebrae could be involved. Clinically, patients refer limb pain, muscular weakness and atrophy, easy fatigability and waddling gait. Later on S. Ribbing described an illness that he thought was a separate entity with sclerosis and enlargement of diaphysis of femora and tibiae, which begins after puberty, is less extensive, not always symmetric and without gait or neurological involvement. Some authors think it may be an adult form of the PDD. As no specific treatments are available we report one case of each entity, treated with the bisphosphonate pamidronate, by the oral route. A white female, 69 years old, with clinic and radiology of Ribbing's Disease, had positive scintigraphy in the affected areas and elevated bone biochemical markers: Serum alkaline phosphatase (SAP): 57 UKA. Total urinary hydroxyproline (THP): 60 mg/24 h. Bone Gla protein (BGP): 40 ng/ml. Considering the high bone turnover treatment with oral pamidronate, 400 mg/day plus Calcium 1g/day was started, dose was then progressively reduced. After two months pain almost disappeared, and THP became normal: 14 mg/24 h; with normalization of BGP values: 8 ng/ml, and a decrease of SAP: 21 UKA, 99mTc MDP uptake by affected bones decreased after 1 year of treatment. Because of these results we decided to begin treatment in a white female 17 years old, 32 kg weight, 1.47 m height with PDD characteristics and also a high bone turnover (THP: 95 mg/24 h. SAP: 32 UKA). After six months of Calcium 1 g/day, given with meals, and oral pamidronate 100 mg/day, she became painless with normal strength and gait, almost normalization of THP (48 mg/24 h). Although a small decrease of SAP, and no charges in scintigraphy. These results obtained with pamidronate suggest that it may be useful to treat dysplasias with high bone turnover.
