In most of the studies of patients with osteogenesis Imperfecta (OI), application of bisphosphonates led to a beneficial increase in bone density (measured by DXA). What is your experience in this topic?
Yes there is consistent evidence of improved bone density. My clinical experience, and I believe that of the worldwide experts, is of significant clinical benefit as well, although the publihsed clinical data is not so clear yet. See the recent Cochrane review: http://www.ncbi.nlm.nih.gov/pubmed/25054949
my colleague at the University of McGill Montreal Canada published a very good article with her experience in experience with Bisphosphonates in Osteogenesis Imperfecta? the results are very encouraging and here is the abstract
Bisphosphonates, Osteonecrosis, Osteogenesis Imperfecta and Dental Extractions: A Case Series Stephane Schwartz, DDS, MSD, FRCD(C); Clara Joseph, DMD, MSc;
Over the past 4 years, numerous cases of osteonecrosis of the jaw in patients treated with bisphosphonates have been reported. Since 1998, children and adolescents with osteogenesis imperfecta have received bisphosphonates to increase their bone density and reduce the incidence of bone fractures. The results have been convincing, but recent
reports of osteonecrosis of the jaw have caused great concern when these patients
require dental extractions. The dental records of 15 children and adolescents with
osteogenesis imperfecta, involving 60 dental extractions, mostly of primary teeth, done between 2001 and 2006, were reviewed. All patients but one had had or were having bisphosphonate treatment at the time of the extractions. No patient developed osteonecrosis.Further studies and data that allow clinicians to design adequate and safe treatment plans for this unique population are needed.The purpose of bisphosphonate therapy is to slow the rate of bone resorption.Mostly, this therapy is used to treat different types of cancers (myelomas, metastatic breast or prostate cancers), bone diseases or severe osteoporosis.1,2 Since 2003, when the link between bisphosphonate therapy and bone osteonecrosis, and more specifically osteonecrosis of the jaw, was established,3–5 many cases of osteonecrosis have been reported.Between September 2007 and May 2008, a search of PubMed yielded 141 articles and reviews on this subject. The authors of these articles caution clinicians to avoid oral surgery for patients undergoing bisphosphonate therapy and indicate that bone osteonecrosis,once established, is practically irreversible.6–9Bisphosphonates are also administeredto children and adolescents who have osteogenesisimperfecta. Osteogenesis imperfectais often associated with severe dental problems, such as dentinogenesis imperfecta(gray-brown friable teeth, bulky crowns and early calcification of the pulpal space) and mal occlusions (drastic open bites, impacted molars; Figs. 1 and 2). Bisphosphonate treatments contribute greatly to the well-being of these patients by reducing bone resorption and by controlling the pain associate with the condition. Bisphosphonate therapy has allowed many of these children to lead normal lives.10,11 Osteogenesis imperfecta is not a common syndrome. The prevalence of all types combined is about 0.5 per 10,000 births.The group of 15 patients whom were reviewed correspond to a population of 300,000 people,probably more, since our group comprised children and adolescents exclusively.
The use of bisphosphonates (B) in Osteognesis Imperfecta (OI) is well documented by many years.
The most tested B is pamidroate, but also zolendronate and neridronate are used and the choice is often made on the base of availability of the drug in each country. In Italy, for example, we use neridronate, they is the only officially approved for this use.
The administration of the medication is made intravenously with repeated cycles with a dose proportional to the weight and the age of the patient.
The long term effects are not so relevant, and, in any case less important than the fragility. Osteonecrosis of the jaws is one of the potential problems.
More recently there some studies about the use of teriparatide in OI.
see some citations
Luca Pietrogrande
Early bisphosphonate treatment in infants with severe osteogenesis imperfecta.
In our research, we found that Bisphosphonates increased oral bone volume in osteogenesis imperfecta. These findings corroborate with the findings in the medical field about the increase in bone volume with bisphosphonate treatment.