Therapy with pamidronate in children with osteogenesis imperfecta

Figures & data

Table 1 Clinical and anthropometric characteristics of the patients diagnosed with OI at first hospital admission

Case number	Age at admission	Sex	Height at first admission (cm)	SDS at first admission
1	17 years	F	113	−8.76
2	15 years	F	123	−6.91
3	5 months	M	53	−6.39
4	3 years	F	79	−5.13
5	4 months	M	59	−2.86
6	8 years	M	112	−3.10
7	5 months	F	51	−7.35
8	5 months	M	55	−7.02
9	2 weeks	F	46	−1.45

Abbreviations: F, female; M, male; OI, osteogenesis imperfecta; SDS, score of standard deviation (for height in our case).

Table 2 Clinical aspects of the study group

Case number	Age at first hospital admission	Type of OI	Sex	Number of fractures	Location of the fractures
1	17 years	III	F	8	Femurs and hips
2	15 years	III	F	7	Hips, right tibia and fibula, 1/3 distal of right femur, right and left humerus
3	5 months	III	M	39	Right and left humerus, right femur, ribs
4	3 years	V	F	6	Bilateral tibia
5	4 months	VIII	M	3	Bilateral tibia and left femur
6	8 years	IV	M	7	Left radius
7	5 months	II	F	3	Left femur and tibia
8	5 months	VI	M	1	Right femur
9	2 weeks	II	F	2	Ribs

Abbreviation: OI, osteogenesis imperfecta.

Figure 1 Newborn with triangle face.

Figure 2 Short stature due to limb fractures with large head and barrel-shaped rib cage.

Figure 3 Dentinogenesis imperfecta with brown–yellow spots, malposition and friability.

Figure 4 The evolution of Z-score in patients before and after 3 years of treatment with pamidronate.

Figure 5 Scatter diagram of serum alkaline phosphatase values and the number of fractures in patients before treatment.

Figure 6 Scatter diagram between the serum alkaline phosphatase and number of fractures in patients after 3 years of treatment.

Figure 7 The evolution of serum bone alkaline phosphatase before and after 3-year period of treatment with pamidronate.

Table 3 The serum levels of osteocalcin and β-crosslaps (β–CTx) in these patients before and after pamidronate treatment

Case	Age at first hospital admission	Sex	Osteocalcin (µg/L)			β-crosslaps (β–CTx) (ng/L)
			Before pamidronate treatment	After 6 months of pamidronate treatment	P-value	Before pamidronate treatment	After 6 months of pamidronate treatment	P-value
1	17 years	F	187	112	0.08	3,450	2,156	<0.05
2	15 years	F	161	103		4,150	1,980
3	5 months	M	134	103		875	672
4	3 years	F	128	101		1,560	1,198
5	4 months	M	104	84		2,372	1,754
6	8 years	M	152	129		973	713
7	5 months	F	124	96		2,280	1,703
8	5 months	M	136	87		1,206	750
9	2 weeks	F	123	89		1,350	981