ObjectivesThe development of tibiofemoral angle in children has shown ethnic
variations. However this data is unavailable for our population.MethodsWe measured the tibiofemoral angle (TFA) and intercondylar and
intermalleolar distances in 360 children aged between two and 18
years, dividing them into six interrupted age group intervals: two
to three years; five to six years; eight to nine years; 11 to 12
years; 14 to 15 years; and 17 to 18 years. Each age group comprised
30 boys and 30 girls. Other variables recorded included standing
height, sitting height, weight, thigh length, leg length and length
of the lower limb.ResultsChildren aged two to three years had a valgus angulation with
a mean TFA of 1.8° (sd 0.65) in boys and 2.45° (sd 0.87)
in girls. Peak valgus was seen in the five- to six-year age group,
with mean TFAs of 6.7° (sd 1.3) and 7.25° (sd 0.64)
for boys and girls, respectively. From this age the values gradually
declined to a mean of 3.18° (sd 1.74) and 4.43° (sd 0.68)
for boys and girls, respectively, at 17 to 18 years. Girls showed
a higher valgus angulation than boys at all age groups.ConclusionThis study defines the normal range of the TFA in south Indian
boys and girls using an easy and reliable technique of measurement
with a standardised custom-made goniometer.Cite this article: Bone Joint Res 2013;2:155–61.
Neurofibromatosis type 1 (NF1) is a commonly occurring genetic disorder in children. Mutation in the NF1 gene has its implication in poor osteoblastic capabilities. We hypothesised that pamidronate will enhance the osteoblastic potential of the mesenchymal stem cells (MSCs) derived from lipofibromatosis tissue of children with congenital pseudarthrosis tibia (CPT) associated with NF1. In this study, bone marrow MSCs (BM MSCs) and CPT MSCs were obtained from three patients undergoing salvage surgeries/bone grafting (healthy controls) and those undergoing excision of the hamartoma and corrective surgeries respectively. The effects of pamidronate (0, 10 nM, 100 nM and 1 μM) on cell proliferation, toxicity and differentiation potential were assessed and the outcome was measured by staining and gene expression. Our outcome showed that CPT MSCs had more proliferation rate as compared to BM MSCs. All 3 doses of pamidronate did not cause any toxicity to the cells in both the groups. The CPT MSCs showed less differentiation with pamidronate compared to the healthy control MSCs. This was quantitated by staining and gene expression analysis. Therefore, supplementation with pamidronate alone will not aid in bone formation in patients diagnosed with CPT. An additional stimulus is required to enhance bone formation.
Florid reactive periostitis is a pronounced periosteal reaction, usually affecting the hands and feet, for which there is no obvious cause. It is rare in children and in long bones. We report an unusual case of florid reactive periostitis in a ten-year-old girl that involved both bones of the forearm. The lesion resolved over a period of one year, leaving a residual exostosis. She developed a physeal bar in the distal ulna in the region of the lesion at one-year follow-up. This was thought to be a complication of the biopsy procedure and was treated by resection and proximal ulnar lengthening.
Although the differential diagnosis may include infantile cortical hypertrophy, child abuse, or a malignant tumor (e.g., osteosarcoma), the presence of typical clinical and radiographic features and characteristic gene mutation helps confirm the diagnosis of type-V osteogenesis imperfecta.
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