Primary hybrid total hip arthroplasty: an interim followup

The senior authors' initial experience with primary hybrid hip replacement in patients with osteoarthritis was studied to evaluate the efficacy of the procedure. Hybrid total hip arthroplasty (uncemented Harris-Galante acetabular component and cemented Iowa precoated femoral component) was performed in 131 consecutive, nonselected hips in 118 patients with the diagnosis of primary osteoarthritis. Followup was performed at 8 to 9 years after the procedure. The average age at the time of the procedure was 68 years (range, 45-87 years). There were 50 men (55 hips) and 68 women (76 hips). At final followup 19 patients (22 hips) had died. The femoral component had been revised for aseptic loosening in 8 hips (6.1%). One additional hip showed definite radiographic loosening. Hence, the prevalence of radiographic femoral failure was 6.9% (9 hips). No acetabular component had been revised for aseptic loosening and no acetabular component had migrated. The senior author continues to perform hybrid total hip arthroplasty in all patients with primary osteoarthritis. However, design modifications have been made in the femoral component that is used.     

Management of nasopharyngeal and oropharyngeal stenosis in children

Nasopharyngeal stenosis and oropharyngeal stenosis are rare and challenging problems in the pediatric population. The most common etiology is currently the surgical trauma associated with adenotonsillectomy. Stenosis can vary from a thin band to a complete obstructing cicatrix. Presenting symptoms range from mild hyponasal speech to severe airway obstruction. We present a series of eight children with varying degrees of stenosis and associated symptoms. Choice of treatment varied with the severity of disease. In our series, successful interventions included triamcinolone acetonide injection, lysis of adhesions, rotational and advancement mucosal flaps, and jejunal free flap. Preoperative evaluation and individualized surgical repair are essential for successful treatment.     

In-utero transplantation of parental CD34 haematopoietic progenitor cells in a patient with X-linked severe combined immunodeficiency (SCIDXI)

BACKGROUND: X-linked severe combined immunodeficiency (SCIDXI) is an inherited immune defect which leads to death in infancy from severe infections. The defect is caused by mutations of the IL-2RG gene that encodes for the common gamma chain shared by several cytokine receptors. The disease is characterised by lack of T and NK cells with normal numbers of B cells. SCIDXI can be cured by bone marrow transplantation (BMT) or prevented by abortion after prenatal diagnosis. METHODS: A male fetus was diagnosed as having SCIDXI by molecular, immunophenotypic, and functional analyses. The fetus was injected intraperitoneally under ultrasound guidance with CD34 haematopoietic progenitor cells purified from paternal bone marrow and T-cell depleted by E rosetting. Chimerism analysis was by HLA-DQ alpha typing and gamma-chain staining on cord blood. FINDINGS: A healthy 3.6 kg boy was delivered by caesarean section at 38 weeks of gestation with no clinical or laboratory signs of graft-versus-host disease. Engraftment of donor-derived CD2 cells was found at birth. At 3.5 months of age the infant is well and his T-cell counts and function are normal. INTERPRETATION: In-utero transplantation of haematopoietic progenitor cells allowed immune reconstitution of a fetus with SCIDXI and may be an alternative to elective abortion. Our report should encourage applications of this method to other inherited disorders curable by BMT.