Citations

Abstract The link between autoimmune diseases and primary immunodeficiency syndromes has been increasingly appreciated. Immunologic evaluation of a young man with autoimmune enterocolopathy and unexplained infections revealed evidence of immunodeficiency, including IgG subclass deficiency, impaired Ag-induced lymphocyte proliferation, reduced cytokine production by CD8+ T lymphocytes, and decreased numbers of NK cells. Genetic evaluation identified haploinsufficiency of NFAT5, a transcription factor regulating immune cell function and cellular adaptation to hyperosmotic stress, as a possible cause of this syndrome. Inhibition or deletion of NFAT5 in normal human and murine cells recapitulated several of the immune deficits identified in the patient. These results provide evidence of a primary immunodeficiency disorder associated with organ-specific autoimmunity linked to NFAT5 deficiency.

OBJECTIVE: The US Surgeon General has called for new approaches to close the mental health services gap for the large proportion of US children with significant mental health needs who have not received evaluation or services within the previous 6 to 12 months. In response, investigators sought to develop brief, easily understood, scientifically derived “warning signs” to help parents, teachers, and the lay public to more easily recognize children with unmet mental health needs and bring these children to health care providers' attention for evaluation and possible services. METHOD: Analyses of epidemiologic data sets from >6000 children and parents were conducted to (1) determine the frequency of common but severely impairing symptom profiles, (2) examine symptom profile frequencies according to age and gender, (3) evaluate positive predictive values of symptom profiles relative to Diagnostic and Statistical Manual of Mental Disorders diagnoses, and (4) examine whether children with 1 or more symptom profiles receive mental health services. RESULTS: Symptom-profile frequencies ranged from 0.5% to 2.0%, and 8% of the children had 1 or more symptom profile. Profiles generated moderate-to-high positive predictive values (52.7%–75.4%) for impairing psychiatric diagnoses, but fewer than 25% of children with 1 or more profiles had received services in the previous 6 months. CONCLUSIONS: Scientifically robust symptom profiles that reflect severe but largely untreated mental health problems were identified. Used as “action signs,” these profiles might help increase public awareness about children's mental health needs, facilitate communication and referral for specific children in need of evaluation, and narrow the child mental health services gap.

BACKGROUND: When surgeons perform total hip arthroplasty for hips with a high dislocation related to developmental dysplasia of the hip, obtaining long-term stable implant fixation and optimizing patient function remain challenges. The purpose of this paper was to evaluate the results of cementless arthroplasty with a simultaneous subtrochanteric shortening osteotomy in a group of patients with Crowe type-IV developmental dysplasia of the hip. METHODS: In a retrospective study, we evaluated the results and complications of twenty-eight consecutive primary cementless total hip arthroplasties in twenty-four patients (twenty women and four men), all of whom had Crowe type-IV developmental dysplasia of the hip. The arthroplasty was performed in combination with a subtrochanteric shortening osteotomy and with placement of the acetabular component at the level of the anatomic hip center. The patients were evaluated at a mean of 4.8 years postoperatively. RESULTS: The mean Harris hip score increased from 43 points preoperatively to 89 points at the time of final follow-up (p < 0.01). Twelve (43%) of the twenty-eight hips had an early or late complication or a reoperation. Two (7%) of the twenty-eight subtrochanteric osteotomies were followed by nonunion. There was one instance of isolated loosening of the femoral stem. One acetabular component loosened, and one acetabular liner disengaged. Four hips dislocated postoperatively. All remaining components were well-fixed at the time of the last radiographic follow-up. No sciatic neurapraxic injuries were identified. CONCLUSIONS: Cementless total hip arthroplasty combined with a subtrochanteric femoral shortening osteotomy in patients with a high hip dislocation secondary to developmental dysplasia was associated with high rates of successful fixation of the implants and healing of the osteotomy site and a mean postoperative Harris hip score of 89 points. The complication rate, however, was substantially higher than that associated with primary total hip arthroplasty in patients with degenerative arthritis. LEVEL OF EVIDENCE: Therapeutic Level IV . See Instructions to Authors for a complete description of levels of evidence. ORIGINAL ABSTRACT CITATION: “Total Hip Arthroplasty with Shortening Subtrochanteric Osteotomy in Crowe Type-IV Developmental Dysplasia” (2009;91:2213-21).