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A 6-year-old girl is described with a unique, rare type of tibial hemimelia known as Carraro syndrome (OIMI 275230). There are only two previous reports of this condition in the literature. In our patient, the tibia anomaly was associated with other skeletal abnormalities, hemivertebra and tetralogy of Fallot. This association of malformations has not previously been reported. We conclude that this girl has either a variant of Carraro syndrome or a new syndrome.

We report on two siblings with a severe neonatal form of spondylometaphyseal dysplasia (SMD). Similar cases have been reported in four publications. Analysis of pedigree data from the original and present families suggests an autosomal recessive mode of inheritance, although parental gonadal mosaicism is also possible. The similarities in the phenotype between our patients and spondyloepimetaphyseal dysplasia congenita (SEMDC) and spondyloepimetaphyseal dysplasia Strudwick (SEMDS) type, indicated that these patients could have a defect in the COL2A1 gene. Molecular analysis of genomic DNA of these patients excluded this gene. Another potential candidate gene PTHR1, was also analyzed in the selected regions and no mutation was found. This gene is probably causative in the Jansen type of SMD, which shares some phenotypic features with the siblings whom we documented. Our results indicate that a new candidate gene for the reported form of SMD should be sought.

Between 1993-2003 the authors treated 5 girls with idiopathic chondrolysis of the hip joint. Their mean age at first symptoms were 9 years (range 6-11 years). In all initial symptoms were pain and a hip flexion contracture of a mean 33 degrees (range 30-40 degrees). All were treated with multiple stationary rehabilitation including traction, range of motion exercises, analgetic physical therapy and longlasting walking with crutches. At the follow up examination only one hip was I 10 degree. Remaining 4 children were painfree. In 2 of them an acetabular protrusion, hip joint space narrowing and degenerative changes were observed on anteroposterior radiographs and clinically the hips were stiff in functional position. In the remaining 2 children, the hip range of motion was limited, with 30 and 90 degree of flexion, but joint space became wider on radiographs. Results were bad, because we do not know neither effective causative non symptomatic methods of treatment. Maybe the new technics of treatment, like arthrodiastasis, will bring better results.

The method and results of treatment in 9 children with fibrous dysplasia of bone and 3 children with Albright syndrome are presented. Curettage and filling of the defect with autologous and/or lyophilized grafts failed in all 5 attempted cases. Due to pathological fracture or deformity Rush nailing combined with multilevel osteotomies has been performed within 5 femoral bones and 4 tibial bones. Healing was undisturbed, neither fracture nor deformity recurred. Valgus osteotomy has been done in 2 children with pseudoarthrosis of the femoral neck. In one case osteotomy healed, in another bony union is not completed yet but the neck-shaft angle remained unchanged. In authors' opinion curettage and grafting proved ineffective; Rush nailing rendered good results in both fracture and deformity treatment.

Reactions between ReH(7)(PPh(3))(2) and a series of benzylic imines, PhCHNR (R = Me, Ph, or Bn), produce the compounds ReH(4)[eta(2)-(1,2-C(6)H(4))CHNR](PPh(3))(2). One such compound, ReH(4)[eta(2)-(1,2-C(6)H(4))CHNPh](PPh(3))(2), was characterized by X-ray diffraction analysis. The compound ReH(4)[eta(2)-(1,2-C(6)H(4))CHNPh](PPh(3))(2) crystallizes in the P(&onemacr;) space group with the following unit cell dimensions: a = 12.422(3) Å, b = 12.696(3) Å, c = 13.604(5) Å, alpha = 93.26(2) degrees, beta = 90.66(2) degrees, gamma = 107.03(2) degrees, V = 2047.2 Å(3), and Z = 2. The structure was refined to R(F(o)) = 0.029 (R(w)(F(o)) = 0.034) for 5352 data with I > 3.0sigma(I). The structure determination indicates that ortho-metalation of the imine reactant occurs during the reaction of ReH(7)(PPh(3))(2) with PhCHNPh. The observation of four nu(Re-H) absorptions in the infrared spectrum of each ReH(4)[eta(2)-(1,2-C(6)H(4))CHNR](PPh(3))(2) compound indicates that the rhenium-bound hydrogen atoms, in these compounds, are better regarded as classical hydride ligands rather than as elongated dihydrogen ligands. Acidolysis reactions of the compounds ReH(4)[eta(2)-(1,2-C(6)H(4))CHNR](PPh(3))(2), with HBF(4).Et(2)O in acetonitrile, result in loss of the ortho-metalated imine ligands from the rhenium coordination spheres and in reduction of the imine functional groups to secondary ammonium cations. One hydride ligand is transferred to the imine carbon atom, during the course of each acidolysis reaction.