Intravenous pamidronate treatment in osteogenesis imperfecta.

J Pediatr

Istituto di Clinica Pediatrica, Carattere Scientifico Burlo Garofolo, Trieste, Italy.

Published: October 1997

Three children with osteogenesis imperfecta, severe osteopenia, and repeated fractures were treated with cyclic infusions of aminohydroxypropylidene bisphosphonate (pamidronate) for a period ranging from 22 to 29 months. A clear clinical response was shown, with a striking reduction of new fracture episodes and a marked improvement in the quality of the patients' lives. Bone mineral density increased significantly in two patients, and linear growth continued along the percentile at the start of treatment. There were no adverse effects of note during treatment, and further studies are warranted.

Download full-text PDF

Source
http://dx.doi.org/10.1016/s0022-3476(97)70074-xDOI Listing

Publication Analysis

Top Keywords

osteogenesis imperfecta
8
intravenous pamidronate
4
pamidronate treatment
4
treatment osteogenesis
4
imperfecta three
4
three children
4
children osteogenesis
4
imperfecta severe
4
severe osteopenia
4
osteopenia repeated
4

Similar Publications

The parental care-seeking behavior of children with osteogenesis imperfecta based on the Anderson's model: a qualitative study.

BMC Nurs

January 2025

Department of Orthopedic, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China.

Objective: This study aims to analyze the medical-seeking behavior of Osteogenesis Imperfecta(OI) children in Southwest China, summarize and analyze the issues in their medical process, and propose corresponding improvement strategies.

Methods: A phenomenological study involving semi-structured interviews with 20 OI caregivers at a tertiary centre for children from March to August 2021 was analyzed thematically, following Anderson's model.

Results: We identified eight themes in the data: 1)Regional disparities of OI management, 2)Big economic burden, 3)High-risk population, 4)Lack of health education, 5)Multiple treatments,6)Strict treatment indications,7)Disappointing therapeutic outcomes,8)Effective or ineffective treatment results.

View Article and Find Full Text PDF

Purpose: to characterize mastication and electrical activation of the masseter and anterior temporalis muscles in children and adolescents with osteogenesis imperfecta (OI), and relate results to guided occlusion and occlusal interference.

Methods: This observational, analytical cross-sectional study included 22 subjects divided into mild OI (MOI) (type 1) (n=15) and moderate-to-severe OI (MSOI) (types 3, 4, and 5) (n=7) groups. The Orofacial Myofunctional Evaluation with Scores (OMES) form was used to evaluate the clinical aspects of mastication.

View Article and Find Full Text PDF

Objective: Spinal orthoses are the most viable conservative treatment for scoliosis, and additive manufacturing techniques have shown huge perspective in producing patient-specific braces, reducing material waste, and production times. This pilot study aimed at determining whether 3D-printed braces could induce advantages or disadvantages compared to conventional braces in terms of mobility and gait, and at quantitatively evaluating the effects of braces on mobility and gait.

Methods: Ten participants were included in the study, eight with adolescent idiopathic scoliosis and two with osteogenesis imperfecta.

View Article and Find Full Text PDF

Timelapse imaging using high-resolution peripheral quantitative computed tomography (HR-pQCT) has emerged as a noninvasive method to quantify bone (re)modelling. However, there is no consensus on how to perform the procedure. As part of the ASTEROID phase-2b multicenter trial, we used 29 same-day repeated scans from adults with osteogenesis imperfecta (OI) to identify a method that minimized measurement error.

View Article and Find Full Text PDF

The trimeric intracellular cation channel B (TRIC-B), encoded by TMEM38B, is a potassium (K) channel present in the endoplasmic reticulum membrane, where it counterbalances calcium (Ca) exit. Lack of TRIC-B activity causes a recessive form of the skeletal disease osteogenesis imperfecta (OI), namely OI type XIV, characterized by impaired intracellular Ca flux and defects in osteoblast (OB) differentiation and activity. Taking advantage of the OB-specific Tmem38b knockout mouse (Runx2Cre;Tmem38b; cKO), we investigated how the ion imbalance affects the osteogenetic process.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!