Efficacy of denosumab therapy for neurofibromatosis type 1 with osteoporosis and history of fractures: a case report

Figures & data

Table 1 Patient characteristics and lumbar and total hip BMD before and at 6, 12, 18, and 24 months of denosumab therapy

Parameters	−22 months	−14 months	−3 months	0 month	6 months	12 months	18 months	24 months
Medication	ALN	ALN	ALN	Denosumab	Denosumab	Denosumab	Denosumab	Denosumab
Lumbar BMD (g/cm^2)	0.77	0.69	0.79	0.77	0.78	0.70	0.76	0.82
Lumbar T-score	−2.8	−2.9	−2.6	−2.8	−2.7	−3.4	−2.9	−2.4
Total hip BMD (g/cm^2)	0.52	0.48	0.53	0.47	0.52	0.53	0.54	0.52
Total hip T-score	−3.5	−3.5	−3.4	−3.9	−3.5	−3.3	−3.3	−3.5

Abbreviations: ALN, alendronate; BMD, bone mineral density.

Table 2 Serum Alb, Ca, bone turnover markers, 1,25(OH)₂D, and whole PTH values before and at 1, 3, 6, 12, 18, and 24 months of denosumab therapy

Parameters	−22 months	−12 months	0 month	1 month	3 months	6 months	12 months	18 months	24 months
Medication	ALN	ALN	Denosumab	Denosumab	Denosumab	Denosumab	Denosumab	Denosumab	Denosumab
Alb-corrected Ca (mg/dL)	8.8	9.1	9.1	8.2	8.5	8.6	9.3	9.5	8.6
BAP (μg/L)	76.6	79.5	59.6	54.2	36.2	79.8	56.3	53.8	15.4
PINP (ng/mL)	N/A	N/A	66.6	34.4	37.2	88.8	79.3	114	26.4
NTX (nmolBCE/mmol/CRE)	N/A	61.8	55.9	8.3	12.9	51.8	92	198	9.8
TRACP-5b (mU/dL)	N/A	N/A	688	83	84	355	775	1,070	150
1,25(OH)2D (pg/mL)	44.4	50.7	36.6	76.5	58.2	25.9	32.2	107	93.5
Whole PTH (pg/mL)	N/A	61.9	73.2	187	150	70.9	72.4	68.1	270

Abbreviations: ALN, alendronate; Alb, albumin; Ca, calcium; 1,25(OH)₂D, 1-alpha, 25-dihydroxyvitamin D₃; PTH, parathyroid hormone; BAP, bone-specific alkaline phosphatase; PINP, type I procollagen N-terminal propeptide; NTX, type I collagen amino-terminal telopeptide; TRACP-5b, tartrate-resistant acid phosphatase 5b; N/A, not available.