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1.
World J Pediatr ; 2024 Aug 07.
Artículo en Inglés | MEDLINE | ID: mdl-39112809

RESUMEN

BACKGROUND: Childhood cancer entails a heavy burden for patients and their families. Recent advances in overall survival rates have increasingly brought long-term quality of life into focus. Animal-assisted activities (AAAs) have long been hypothesized to alleviate the burden on pediatric patients and their peers in the hospital setting. However, their use in inpatient pediatric oncology has been a sensitive issue mainly due to the fear of infections, resulting in a lack of studies. This study presents data on the feasibility, safety, and efficacy of AAAs from a single German center. METHODS: Between 2018 and 2022, 60 patients (median age = 10.3 years) diagnosed with malignancy and undergoing treatment were visited by an intervention dog (total visits = 100). Patients were screened for infections as per hospital policy, with additional microbiological testing performed based on symptoms. The dog was screened for human pathogens and zoonoses. Microbial data and hospitalizations were analyzed from two months prior to the first visit until two months after the last visit. Acceptance of being in the hospital, both with and without planned animal-assisted interventions and pre- and post-intervention state stress, were measured using a validated visual analogue scale (0-10). RESULTS: Patients benefited from AAAs, showing increased acceptance of being in the hospital (median: 7.25 vs. 4.50, P < 0.001) and decreased median state stress ratings one hour after the visit compared to one hour before the visit (1.00 vs. 4.25, P < 0.001). The intervention did not result in an increased number of infections or unplanned hospitalizations, and no zoonoses were detected. All microbial screening tests of the dog were negative. CONCLUSIONS: AAAs with visiting dogs in inpatient pediatric oncology are feasible and safe. Although they hold promise for enhancing patients' well-being, further prospective studies are needed. Supplementary file 2 (MP4 240076 KB).

2.
Horm Res Paediatr ; 94(3-4): 151-158, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34261073

RESUMEN

In ultra-rare bone diseases, information on growth during childhood is sparse. Juvenile Paget disease (JPD) is an ultra-rare disease, characterized by loss of function of osteoprotegerin (OPG). OPG inhibits osteoclast activation via the receptor activator of nuclear factor-κB (RANK) pathway. In JPD, overactive osteoclasts result in inflammatory-like bone disease due to grossly elevated bone resorption. Knowledge on the natural history of JPD, including final height and growth, is limited. Most affected children receive long-term antiresorptive treatment, mostly with bisphosphonates, to contain bone resorption, which may affect growth. In this study, we report the follow-up of height, growth velocity, and skeletal maturation in a 16-year-old female patient with JPD. The patient was treated with cyclic doses of pamidronate starting at 2.5 years of age and with 2 doses of denosumab at the age of 8 years, when pamidronate was paused. In the following years, a sustainable decline in a height z-score and a stunted pubertal growth spurt; despite appropriate maturation of the epiphyseal plates of the left hand, the proximal right humerus and both femora were observed. Whether this reflects the growth pattern in JPD or might be associated to the antiresorptive treatments is unclear, since there is very limited information available on the effect of bisphosphonates and denosumab on growth and the growth plate in pediatric patients. Studies are needed to understand the natural history of an ultra-rare bone disease and to assess the effects of antiresorptive treatment on the growing skeleton.


Asunto(s)
Denosumab/administración & dosificación , Fémur , Placa de Crecimiento , Húmero , Osteítis Deformante , Pamidronato/administración & dosificación , Adolescente , Niño , Preescolar , Femenino , Fémur/crecimiento & desarrollo , Fémur/metabolismo , Fémur/fisiopatología , Placa de Crecimiento/crecimiento & desarrollo , Placa de Crecimiento/metabolismo , Placa de Crecimiento/fisiopatología , Humanos , Húmero/crecimiento & desarrollo , Húmero/fisiopatología , Osteítis Deformante/tratamiento farmacológico , Osteítis Deformante/metabolismo , Osteítis Deformante/fisiopatología , Osteoprotegerina/metabolismo
3.
Bone Rep ; 14: 100762, 2021 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-33850973

RESUMEN

Juvenile Paget disease (JPD) is an ultra-rare disease, characterized by loss of function of osteoprotegerin. Osteoprotegerin inhibits osteoclast activation via the receptor activator of nuclear factor κB (RANK) pathway. Severely affected children suffer from bone deformities and pain and require long term anti-resorptive treatment. Due to the rarity of the disease, few long-term follow-up data on the clinical course in children are available. In this report, motor development during infancy and early childhood and the activity of the bone disease based on clinical, radiographic and biochemical parameters are reported in 2 children with severe forms of JPD during long term treatment (4 and 14 years) with bisphosphonates. Results of a bone biopsy in patient 1 after 10 years of treatment and video material of the motor development of patient 2 are provided. Doses per year of pamidronate ranged from 4 to 9 mg/kg bodyweight and were administered in 4-10 courses, yearly. Treatment was adjusted individually according to the presence of bone pain. Motor development was delayed in both children before treatment with bisphosphonates was commenced and improved thereafter. Bone histology revealed a significantly higher heterogeneity of mineralization which was mainly attributed to the increased percentage of low mineralized bone areas. Individualized intravenous treatment with pamidronate resulted in sufficient control of bone pain and suppression of bone turnover with few side effects over the observation period.

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