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[Calcinosis universalis with hyperphosphataemia--successful treatment with phosphorus deprivation]
Insights
A rare case of calcinosis universalis in a child with hyperphosphatemia showed significant reduction in calcified mass with aluminum hydroxide and probenecid treatment. This suggests a potential new therapeutic approach for this condition.
Area of Science:
- Pediatric Endocrinology
- Dermatology
- Nephrology
Background:
- Calcinosis universalis is a rare condition characterized by widespread calcification of soft tissues.
- Hyperphosphatemia, or high serum phosphorus, is a known factor in soft tissue calcification, commonly seen in tumoral calcinosis.
- The pathogenesis of soft tissue calcification in calcinosis universalis remains unclear.
Observation:
- A seven-year-old boy presented with calcinosis universalis and significantly elevated serum phosphorus levels.
- Treatment with aluminum hydroxide (up to 18g/day) for one year led to a remarkable reduction in calcified mass.
- Despite aluminum hydroxide treatment, serum phosphorus levels remained above the normal range.
Findings:
- Concomitant administration of probenecid alongside aluminum hydroxide demonstrated efficacy in both reducing serum phosphorus levels and decreasing the calcified mass.
- The case highlights hyperphosphatemia as a potential accelerating factor in soft tissue calcification within calcinosis universalis.
- This presentation is unique as it associates calcinosis universalis with hyperphosphatemia, a combination not previously reported.
Implications:
- This case suggests that a combination therapy of aluminum hydroxide and probenecid may be a viable treatment strategy for calcinosis universalis associated with hyperphosphatemia.
- Further research is warranted to elucidate the specific pathogenetic mechanisms linking hyperphosphatemia to calcinosis universalis.
- This unique case may represent a distinct subtype of calcinosis universalis requiring tailored therapeutic interventions.
Abstract:
Seven year old boy with calcinosis universalis associated with high serum phosphorus was reported. Over one year's treatment with aluminum hydroxide up to 18 grams per day have reduced calcified mass remarkably, while serum phosphorus levels were remaining still above the normal range. Concomitant use of probenecid seemed to be effective in reducing serum phosphorus levels as well as decreasing the calcified mass. Pathogenetic mechanisms of soft tissue calcification in calcinosis universalis is not clear, but in this case hyperphosphataemia is considered to be one important factor accelerating soft tissue calcification. Although hyperphosphataemia associated with tumoral calcinosis has been frequently observed, no report is yet available on calcinosis universalis associated with hyperphosphataemia. This case might represent one unique type of calcinosis.