• Clinics in Shoulder and Elbow
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• v.16 no.1
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• pp.33-39
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• 2013

Purpose: The aim of this study is to evaluate the clinical result of intra-articular fractures of the distal humerus (AO type C) in elderly osteoporotic patients treated with double tension band osteosynthesis. Materials and Methods: From January 2006 to December 2010, 10 elderly osteoporotic patients(1 male, 9 females) with intra-articular fractures of the distal humerus (AO type C) were treated with double tension band osteosynthesis. The mean age of patients at the time of surgery was 74.6(66~84) years and the mean follow-up period was 39.2(20~74) months. The fracture union and complications were assessed and the functional result was evaluated by the rating system of Jupiter et al. and the Mayo elbow performance index. Results: Bone union was achieved in all patients with no secondary displacement. The mean time for union was 16.6(13~22) weeks. The average postoperative arc of elbow flexion was 119(100~140) degrees with a mean flexion contracture of 8.5(0~15) degrees. The recovery in two patients was rated as excellent, in 7 as good, and in 1 as fair in terms of the Mayo elbow performance index with average value of 82(70~90) points. Seven patients were rated as excellent, 1 as good, and 2 as fair in terms of the rating system of Jupiter et al. Changing tension band wiring was performed in one patient as skin irritation was noticed due to tension band knots. Heterotopic ossification developed in one patient but had no symptom. Conclusion: Double tension band osteosynthesis in intra-articular fractures of distal humerus (AO type C) in elderly osteoporotic patients can provide sufficient and secure stability to allow early rehabilitation.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.21 no.1
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• pp.7-14
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• 2021

Purpose: Pseudohypoparathyroidism (PHP) is caused by genetic and epigenetic alteration in the GNAS locus, and characterized by the resistance to multiple hormones and the Albright's hereditary osteodystrophy (AHO) phenotype. This study investigated the phenotypic characteristics and molecular features of PHP. Methods: Eight patients who diagnosed as PHP were enrolled at Pusan National University Children's hospital and clinical features, biochemical and genetic findings were retrospectively reviewed. Results: Of a total of 8 patients, 5 were diagnosed with PHP1a, and 3 were diagnosed with PHP1b. Patients with PHP1a had three different mutations in the GNAS gene, and patients with PHPIb had imprinting defect in differentially methylated regions (DMRs) of the GNAS locus. Two novel GNAS variants were identified in patients with PHP1a, including c.313-2A>T and c.1094G>A. All patients with PHP1a displayed AHO features; short stature (80%), brachydactyly (80%), a round face (80%), obesity (40%), heterotopic ossification (60%), and intellectual disability (60%), whereas only one patient (33.3%) with PHP1b showed AHO feature such as a round face. When phenotypic features between PHP1a and PHP1b patients were compared, patients with PHP1b showed a tendency of higher current height standard deviation scores (SDS) compared to patients with PHP1a, (-3.2±2.1 vs.-1.1±0.8; P=0.06) Conclusions: This study summarizes the phenotypic and genetic features of the PHP patients. Although we found considerable clinical overlap between PHP1a and PHP1b, further long-term follow-up is needed to evaluate the growth and development of children with PHP, as well as the effects of end-organ resistances to endocrine hormones.