Diagnostik und Therapie der Osteogenesis imperfecta

Meurer, Andrea; Lewens, T.; Schmitt, DC; Vetter, Thomas R.; Drees, Philipp

doi:10.1007/s00132-008-1211-y

Cited by 2 publications

(2 citation statements)

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“…On cycle days 2 and 3, the dose was increased to 0.250-0.500 mg/kg body weight, respectively (12)(13)(14)(15) . Pamidronate was administered intravenously as a 4-hour physiological saline infusion, followed by ionised blood calcium measurement 4 hours after the end of the infusion (16)(17)(18)(19)(20) . An analysis of biochemical calcium and phosphate metabolism parameters in newborns revealed significant changes after the first pamidronate cycle (Tab.…”

Section: Resultsmentioning

confidence: 99%

Assessment of the health status and pharmacological treatment of neonates with osteogenesis imperfecta – 20-year single-centre observations

Jakubowska-Pietkiewicz,

Górczewska,

Nowicki

et al. 2023

Pediatr Med Rodz

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Introduction and objective: Osteogenesis imperfecta is a heritable bone dysplasia resulting in reduced bone mineral density. Fractures of long bones, which are the dominant symptom of osteogenesis imperfecta, can be diagnosed already in foetal life. Multiple fractures are associated with severe pain, which may cause deterioration of the newborn’s general condition, respiratory and circulatory disorders, and reduced general motor activity. The aim of the study was to summarise the experience resulting from many years of care and treatment of neonates with osteogenesis imperfecta types II and III. Materials and methods: The study included 53 newborns with osteogenesis imperfecta hospitalised at the Department of Paediatrics, Newborn Pathology and Bone Metabolic Diseases in the years 2001–2021. All children underwent a babygram and an assessment of their health status, including calcium and phosphorus metabolism parameters. Results: In 67.3% of cases, the mother was informed before delivery that she would give birth to a sick child. The general condition of the newborns was moderately severe to severe in 43.4%. Physical examination revealed abnormal body proportions related to previous fractures in 98.1% of patients, including long bones of limbs. More than 10 fresh bone fractures were diagnosed in 34% of children. Conclusions: Prenatal diagnosis of osteogenesis imperfecta indicates its severe course (multiple fractures) in the neonatal period. Therefore, it requires care in a specialised centre that will provide safe, symptomatic pharmacological treatment (bisphosphonates) to reduce both pain and the risk of further bone fractures.

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Section: Resultsmentioning

confidence: 99%

Assessment of the health status and pharmacological treatment of neonates with osteogenesis imperfecta – 20-year single-centre observations

Jakubowska-Pietkiewicz,

Górczewska,

Nowicki

et al. 2023

Pediatr Med Rodz

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show abstract

“…Trotz relativ hoher Komplikationsraten bezüglich einer Dislokation der Nägel wird meist die Vertikalisierung und oft das Gehen, wenn auch eingeschränkt, ermöglicht [2,48,49]. Bei schwer betroffenen OI-Patienten mit geringem Wachstum und sehr dünnem Markraum müssen Kompromisse eingegangen und auf Kirschner-Drähte oder Titan-elastische Nägel -TEN-oder ESIN-Nägelzurückgegriffen werden [2].…”

Section: Merkeunclassified

Angeborene Bindegewebserkrankungen mit skelettalem Phänotyp

Brenner¹,

Taurman²

2017

Orthopädie und Unfallchirurgie up2date

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Diagnostik und Therapie der Osteogenesis imperfecta

Cited by 2 publications

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Assessment of the health status and pharmacological treatment of neonates with osteogenesis imperfecta – 20-year single-centre observations

Assessment of the health status and pharmacological treatment of neonates with osteogenesis imperfecta – 20-year single-centre observations

Angeborene Bindegewebserkrankungen mit skelettalem Phänotyp

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