Osteopenia in a teenage boy presenting with previously undiagnosed guanidinoacetate methyltransferase (GAMT) deficiency and response to creatine supplementation

A 16 y.o. fully ambulant boy born to consanguineous Indian parents, presented for assessment of a fragility femoral neck fracture sustained against a background of autism and moderately severe intellectual disability. He had a past history of infantile eczema, and epilepsy treated with anticonvulsants from 2 to 10 years of age, with no further seizures following cessation of anticonvulsants. He had a thin body habitus (see Table 1) with long fingers and a high arched palate. He had no speech and negligible social interaction, but physical examination was otherwise unremarkable. Positive investigations revealed an undetectable serum creatinine and a urinary metabolic screen which showed an elevated GUA:Phe of 160 (< 36) and a decreased creatinine of 0.3 mmol/l (1.2–29.5) consistent with the diagnosis of guanidinoacetate methyltransferase(GAMT) deficiency. He was commenced on oral creatine 5 g three times daily. Despite improvement in physical activity, height and bone density, there was no discernable improvement in his intellectual functioning. Proton and phosphorous brain and leg magnetic resonance spectroscopy(MRS) was performed at baseline and showed an increased inorganic phosphorus peak and decreased phosphocreatine synthesis in brain and decreased creatine concentration in muscle. Following creatine treatment total brain creatine(1H-MRS) and phosphocreatine/ATP ratio (31P-MRS) content increased to 30% and 60% of control values, respectively. Brain GUA returned to normal levels.

Osteopenia and rickets in the extremely low birth weight infant: A survey of the incidence and a radiological classification

A review was carried out of the radiographs of twenty-five infants with birth weights under 1000 G, who survived for more than twenty-eight days; eighteen of these had enough suitable films for a survey of the progressive bone changes which occur in these infants, including estimation of humeral cortical cross-sectional area. The incidence of the changes has been assessed and a typical progression of radiographic appearances has been shown, with a suggested system of staging. All infants showed some loss of bone mineral, with frank changes of rickets occurring in forty-four percent. Aetiological factors are mainly concerned with the difficulty of supplying and ensuring absorption of sufficient bone mineral (calcium and phosphate) and vitamin D. Liver immaturity may be another factor. Disease states additional to prematurity accentuate the problem. Rib fractures occurring around 80–90 days post-nataEy commonly draw attention to the bone disorder and are probably the major clinical factor of importance; there is a high incidence of associated lung disease of uncertain pathology. Attention is drawn to possible confusion with other bone disorders in the post-natal period.

Osteopenia diabética e pós-menopausa: efeito metabólico do zinco na recuperação do volume ósseo trabecular

Diabetes mellitus has been associated with bone metabolism alterations, such as osteopenia and osteporosis. So, the search of new anabolic agents promote bone mass gain can be important to prevent osteoporosis. The aim of this study was evaluate zinc anabolic effect over bone in diabetic and post-menopausal osteopenic models. Diabetes was induced by STZ (45mg/Kg of body weight) administration and post-menopausal by bilateral ovariectomy. Adults female Wistar rats (n=65) were divided in 5 groups: control group (n=15), ovariectomized without (n=15) and with zinc supplementation (n=10) groups, diabetic and ovarioctomized without (n=15) and with zinc supplementation (n=10) groups. Studied periods had been untill 90 days. Diabetic condition was confirmed hiperglicemic state and alterations of state with polyuria, polyphagia, polydipsia and glucosuria. Histomorphometric analysis showed that zinc supplementation increased trabecular thickness and reduced trabecular distance significantly in diabetic groups with similar values to those showed in control group. Correlation analysis of histomorphometric parameters with serum glucose concentration showed that more time in hyperglycemia more bone damage, as well as, zinc supplementation contributed to prevent this damage. Elevated serum glucose caused hyperzincuria, phosphaturia and calciuria. Zinc supplementation promoted increased levels of calcium and phosphorous ions in 90th days diabetic group. No alteration was observed by ovariectomy in mineral (Ca, P and Zn) serum and urine concentrations. Total serum Alkaline Phosphatase activity increased in diabetic groups, supplemented or not, compared with control group. However, Tartarate-Resistant Acid Phosphatase, magnesium and serum zinc did not altered in studied groups. Serum albumin was reduced only in diabetic groups. Serum creatinine was unaltered. These results support the hypotesis that zinc can be used to prevent and treat diabetic and post-menopausal osteopenia

Influence of Osteopenia in Autogenous Bone Graft Healing With or Without Expanded Polytetrafluoroethylene Membranes: Histologic and Histomorphometric Study in Rats

Purpose: The aim of this study was to quantitatively evaluate and qualitatively describe autogenous bone graft healing with or without an expanded polytetrafluoroethylene (e-PTFE) membrane in ovariectornized rats. Materials and Methods: Eighty Wistar rats, weighing approximately 300 g each, were used. A graft was obtained from the parietal bone and fixed to the sidewall of each animal's left mandibular ramus. The animals were randomly divided into four experimental groups (n = 20 in each group): group 1, sham operated and autogenous bone graft only- group 2, sham operated and autogenous bone graft covered by e-PTFE membrane; group 3, ovariectornized (OVX) and autogenous bone graft only- group 4, OVX and autogenous bone graft covered by e-PTFE membrane. The animals were sacrificed at five different time points: immediately after grafting or at 7, 21, 45, or 60 days after grafting. Histologic examination and morphometric measurement of the sections were performed, and values were submitted to statistical analyses. Results: Both groups (sham and OVX) experienced loss of the original graft volume when it was not covered by the membrane, whereas use of the membrane resulted in additional bone formation beyond the edges of the graft and under the membrane. Histologic analysis showed integration of the grafts in all animals, although a larger number of marrow spaces was found in OVX groups. Conclusions: Association of bone graft with an e-PTFE membrane resulted in maintenance of its original volume as well as formation of new bone that filled the space under the membrane. Osteopenia did not influence bone graft repair, regardless of whether or not it was associated with e-PTFE membrane, but descriptive histologic analysis showed larger numbers of marrow spaces in the bone graft and receptor bed and formation of new bone in the OVX animals. INT J ORAL MAXILLOFAC IMPLANTS 2009;24:1074-1082

Influência da osteopenia na reparação do enxerto ósseo autógeno associado ou não por membrana de PTFE-e: estudo histológico e histomorfométrico em ratas

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Aplicação do ultrassom de baixa potência na prevenção de osteopenia em tíbias de ratos sob ausência de carga

Pós-graduação em Ciência Animal - FMVA

Prática de atividade física e qualidade de vida de pacientes com osteoporose e osteopenia

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Análise comparativa por meio da densidade óptica radiográfica entre o grau de osteopenia induzida pela fenitoína e ausência hormonal em ratos

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Efeitos da osteopenia no reparo ósseo alveolar de ratas

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

Contribuição da aplicação do ultrassom de baixa potência na prevenção de osteopenia em tíbias de ratos sob ausência de carga

Este trabalho objetivou verificar se o ultrassom de baixa potência (US) previne a ocorrência de osteopenia em tíbias de ratos sob ausência de carga. Foram utilizados 45 Rattus novergicus albinus, Wistar adultos, machos, distribuídos em cinco grupos iguais: C - animais-controle livres em gaiolas por 21 dias; S - animais suspensos pela cauda por 21 dias; ST - suspensos pela cauda por 21 dias e concomitantemente tratados com US; S→C - suspensos por 21 dias e depois permanecendo livres em gaiolas por mais 21 dias; S→CT - suspensos por 21 dias e depois permanecendo livres em gaiolas por mais 21 dias e concomitantemente tratados com US. O tratamento foi realizado com US de 1,5MHz, ciclo de trabalho 1:4, 30mW/cm², na tíbia direita, por 15 sessões de 20 minutos cada, cinco sessões por semana. Ainda vivos, os animais foram submetidos a exame de densitometria óssea para verificação da densidade mineral óssea (DMO) e do conteúdo mineral ósseo (CMO). Após a eutanásia dos animais, as tíbias foram desarticuladas, dissecadas e submetidas a ensaio mecânico destrutivo para análise da força máxima (Fmáx) e da rigidez (R). Foram avaliados também o comprimento (L) e o diâmetro (D) no ponto médio da tíbia. O grupo S apresentou valores de DMO, CMO, Fmáx, R, L e D menores em relação ao grupo C, demonstrando que a suspensão pela cauda é prejudicial a estas variáveis. O tratamento dos animais suspensos com o US, grupo ST, elevou os valores de CMO e DMO em relação aos do grupo S, igualando-os aos do grupo C. A Fmáx, R e L do grupo ST aumentou em relação ao grupo S e também em relação ao grupo C (p<0,05). Nenhuma diferença significativa foi encontrada entre as variáveis analisadas para os grupos S→CT e S→C (p>0,05). Os resultados obtidos neste estudo permitem concluir que o US de baixa potência contribuiu na prevenção e reversão da ocorrência da osteopenia nos animais submetidos à suspensão pela cauda, demonstrando que a ausência do estímulo mecânico causada pela impossibilidade da deambulação pode ser minimizada pela ação mecânica deste.

Exercício resistido no tratamento e prevenção de osteopenia de ratos machos jovens

Pós-graduação em Ciência Animal - FMVA

Graded doses of recombinant interleukin-1beta induce generalized osteopenia in rats without altering skeletal growth and joint integrity

Whereas a primary role of interleukin-1beta (IL-1beta) in local bone remodelling and articular inflammation has been well established, the effect of prolonged systemic administration of this cytokine on total skeletal Ca, somatic growth and joint tissue has not yet been investigated.

Non-invasive quantitative estimation of bone density in rats throughout the life cycle and in arthritic osteopenia: preliminary results

A longitudinal bone survey was conducted in 86 female Wistar rats in order to assess mineral density kinetics from young age (5 weeks: 115 g) till late adulthood (64 weeks: 586 g). In vivo quantitative radiographic scanning was performed on the caudal vertebrae, taking trabecular mass as the parameter. Measurements were expressed as Relative Optical Density (ROD) units by means of a high resolution densitometric device. Results showed a progressive increase in mineral density throughout the life cycle, with a tendency to level in the higher weight range, indicating that progressive mineral aposition occurs in rats in dependency of age. This phenomenon, however, should be always considered within the context of continuous skeletal growth and related changes typical of this species. Twelve different animals were also examined following induction of articular inflammation with Freund's adjuvant in six of them. Bone survey conducted 12 to 18 days after inoculation revealed a significant (P less than 0.01) reduction in trabecular bone mass of scanned vertebrae in comparison with the weight-matched untreated controls. It is concluded that the in vivo quantitative assessment of bone density illustrated in this report represents a sensitive and useful tool for the long-term survey of naturally occurring or experimentally induced bone changes. Scanning of the same part of the skeleton can be repeated, thereby avoiding sacrifice of the animal and time-consuming preparation of post-mortem material.

No evidence of osteopenia 5 to 8 years after ileal orthotopic bladder substitution

The use of bowel segments as bladder substitutes may result in chronic, impaired vitamin D and calcium metabolism, and ultimately in bone demineralization.