[A case of type 2N von Willebrand disease with homozygous R816W mutation of the VWF gene in a Nepalese woman].

Type 2N von Willebrand disease (vWD) can be confused with hemophilia A due to decreased factor VIII levels and a bleeding tendency, and differential diagnosis is of importance for providing the optimal treatment and genetic counseling. For the accurate diagnosis of type 2N vWD, von Willebrand Factor (vWF) function tests, multimer assay and gene mutation analysis are needed. The patient was a 38-yr-old Nepalese woman with a history of bleeding manifestations from childhood, such as hemarthrosis, intramuscular hematoma, and menorrhagia.

Early outcome of TKA with a medial pivot fixed-bearing prosthesis is worse than with a PFC mobile-bearing prosthesis.

Unlabelled: Although the design features of the Medial Pivot fixed-bearing prosthesis reportedly improve kinematics compared with TKAs using fixed-bearings, clinical improvements have not been reported. We asked whether the clinical and radiographic outcomes, ranges of motion of the knee, patient satisfaction, and complication rates would be better in knees with a Medial Pivot fixed-bearing prosthesis than in those with a PFC Sigma mobile-bearing prosthesis. We compared the results of 92 patients who had a Medial Pivot fixed-bearing prosthesis implanted in one knee and a PFC Sigma mobile-bearing prosthesis implanted in the other.

The results of a proximally-coated cementless femoral component in total hip replacement: a five- to 12-year follow-up.

This study reviewed the results of a cementless anatomical femoral component to give immediate post-operative stability, and with a narrow distal section in order not to contact the femoral cortex in the diaphysis, ensuring exclusively metaphyseal loading. A total of 471 patients (601 hips) who had a total hip replacement between March 1995 and February 2002 were included in the study. There were 297 men and 174 women.

[Usefulness of NMP22 BladderChek for the diagnosis and monitoring of bladder cancer].

Background: As bladder cancer is a superficial tumor with frequent recurrences, early detection and confirmation of recurrence are important. We evaluated the usefulness of NMP22 BladderChek (NMP22BC) for the diagnosis and monitoring of bladder cancer.

Methods: From July to December 2004, we enrolled in the study 670 patients who visited the urology clinic in Ewha Womans University, Dongdaemun Hospital with hematuria or dysuria and were tested with NMP22BC.

Alignment and orientation of the components in total knee replacement with and without navigation support: a prospective, randomised study.

Bilateral sequential total knee replacement was carried out under one anaesthetic in 100 patients. One knee was replaced using a CT-free computer-assisted navigation system and the other conventionally without navigation. The two methods were compared for accuracy of orientation and alignment of the components.

Clinical outcome and rate of complications after primary total knee replacement performed with quadriceps-sparing or standard arthrotomy.

We performed a prospective, randomised study to compare the results and rates of complications of primary total knee replacement performed using a quadriceps-sparing technique or a standard arthrotomy in 120 patients who had bilateral total knee replacements carried out under the same anaesthetic. The clinical results, pain scales, surgical and hospital data, post-operative complications and radiological results were compared. No significant differences were found between the two groups with respect to the blood loss, knee score, function score, pain scale, range of movement or radiological findings.

Long-term survivorship of the Charnley Elite Plus femoral component in young patients.

We studied prospectively the long-term results of the Charnley Elite-Plus femoral stem in 184 consecutive young patients (194 hips). There were 130 men and 54 women with a mean age of 49.1 years (21 to 60).

Changes in the bone mineral density in the acetabulum and proximal femur after cementless total hip replacement: alumina-on-alumina versus alumina-on-polyethylene articulation.

Our aim in this prospective study was to compare the bone mineral density (BMD) around cementless acetabular and femoral components which were identical in geometry and had the same alumina modular femoral head, but differed in regard to the material of the acetabular liners (alumina ceramic or polyethylene) in 50 patients (100 hips) who had undergone bilateral simultaneous primary total hip replacement. Dual energy X-ray absorptiometry scans of the pelvis and proximal femur were obtained at one week, at one year, and annually thereafter during the five-year period of the study. At the final follow-up, the mean BMD had increased significantly in each group in acetabular zone I of DeLee and Charnley (20% (15% to 26%), p=0.

Comparison of natural and Dumon airway stents for the management of benign tracheobronchial stenoses.

Objective And Background: To investigate the utility and safety of the 'Natural stent', a newly designed silicone airway stent, the authors compared clinical outcomes and complications in patients who underwent silicone airway stenting for the management of benign airway stenosis.

Methods: The medical records of 94 patients requiring the placement of 100 airway stents (43 Dumon and 57 Natural) were retrospectively reviewed in a tertiary referral hospital.

Results: Post-tuberculous stenosis was the leading indication for airway stenting (74%), followed by post-intubation stenosis (21%).

Use of silicone stents for the management of post-tuberculosis tracheobronchial stenosis.

The role of bronchoscopic management in post-tuberculosis tracheobronchial stenosis is not well defined. To investigate the role of bronchoscopic intervention, including silicone stenting, in the management of post-tuberculosis tracheobronchial stenosis, the current retrospective study was conducted at a tertiary referral hospital. Under rigid bronchoscopy, 80 patients underwent ballooning, neodymium-yttrium aluminium garnet laser resection and/or bougienation as first-line methods of airway dilatation between January 2000 and December 2003 inclusive, and were followed for a median of 41 months.

Bronchoalveolar lavage in fibrotic idiopathic interstitial pneumonias.

The purpose of this study was to assess the role of bronchoalveolar lavage (BAL) in differentiating usual interstitial pneumonia (UIP) from non-specific interstitial pneumonia (NSIP) and in predicting the prognosis in fibrotic idiopathic interstitial pneumonia (IIP). A retrospective review of 122 patients (age 58+/-8 years, 70 male) with UIP (n=87) and NSIP (n=35) was carried out. Prior to surgical lung biopsy, all of them underwent BAL and high-resolution-computed tomography (HRCT) of the chest.

Early onset severe and late-onset mild Charcot-Marie-Tooth disease with mitofusin 2 (MFN2) mutations.

Mutations in the mitofusin 2 (MFN2) gene, which encodes a mitochondrial GTPase mitofusin protein, have recently been reported to cause both Charcot-Marie-Tooth 2A (CMT2A) and hereditary motor and sensory neuropathy VI (HMSN VI). It is well known that HMSN VI is an axonal CMT neuropathy with optic atrophy. However, the differences between CMT2A and HMSN VI with MFN2 mutations remained to be clarified.

Comparison of anterior-posterior-glide and rotating-platform low contact stress mobile-bearing total knee arthroplasties.

Background: The anterior-posterior-glide Low Contact Stress mobile-bearing knee prosthesis was developed to approximate the natural kinematics of the knee more closely than the rotating-platform Low Contact Stress mobile-bearing knee prosthesis does. The purpose of the present study was to compare the results associated with these two prostheses in patients managed with simultaneous bilateral total knee replacement.

Methods: One hundred and ninety patients received an anterior-posterior-glide Low Contact Stress mobile-bearing prosthesis in one knee and a rotating-platform Low Contact Stress mobile-bearing prosthesis in the contralateral knee.

Crying patterns of Korean infants in institutions.

This study investigates whether the crying pattern of infants reared in institutions is different from those reared at home in the same cultural backgrounds. Ninety-five institutional infants and 143 home infants, aged 2-18 weeks were studied through diaries, recorded by children's care-givers or mothers. Results showed that the crying duration of the infants in institutions was almost double that of infants at home (86 vs 45 min/day) and contact period with the care-givers was half that of home infants (136 vs 279 min/day).