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With the advent of the information age, data storage has not only developed from paper information systems to electronic information system storage but also extended to cloud database storage methods. To date, we can see the application of big data and cloud in various fields and levels, which makes the induction, arrangement, transmission, and exchange of data easier and faster. The electronic and systematization of patient medical records in the medical environment is an example. The use of electronic medical records can help to improve the quality of medical care, reduce duplication of treatment, and reduce the waste of examination resources. It has many advantages, but also many risks. Medical institutions may abuse electronic medical data without the consent of patients leading to the leakage of patient privacy data and causing serious social impact. Therefore, this study proposes a medical record exchange and sharing mechanism based on the Hyperledger blockchain architecture combined with proxy re-encryption. When pharmacists need to obtain medical records for related research, patients can decide whether to share medical records, and hospitals can also revoke the access rights of pharmacists at any time to achieve more private, secure, and convenient sharing of medical records. The proposed scheme meets various blockchain security requirements, and the BAN (Burrows–Abadi–Needham) logic proof model is applied to evaluate the correctness of the proposed scheme. The proposed scheme performs well in terms of computational and communication costs.

Cystic fibrosis (CF) is one of the most common autosomal recessive inherited disorders among Caucasians. Comparatively, it is considered to be a rare disease among Asians. To date, only a few cases of Taiwanese CF have been published. We report four CF cases from three families. Case 1 was the first report of CF associated with a homozygosity for the CF transmembrane conductance regulator gene (CFTR gene) mutation 3849+10kb C->T in a Taiwanese patient. Cases 2 and 3 had heterozygous c. 1898+5 G->T and heterozygous p. I1023R (novel mutation) for the CFTR gene mutation. Case 4 was homozygous for the CFTR gene mutation R553X being reported in 2005 and complicated with cor pulmonale. These four patients had received 300 mg bid aerosolized tobramycin treatment every other month.

We report a 3-year-old girl with Kawasaki disease who presented with retropharyngeal edema and shock syndrome. This is the first reported case in Taiwan. The patient initially presented with fever, cough, and pyuria followed by rapidly progressive enlarged bilateral cervical lymphadenopathy. On the third day of the fever, computed tomography for airway compression sign found widening of the retropharyngeal space mimicking a retropharyngeal abscess. Later, an endotracheal tube was inserted for respiratory distress. A skin rash over her trunk was also noted. On the fifth day of the fever, the clinical course progressed to hypotension and shock syndrome. Because of more swelling of bilateral neck lymph nodes, computed tomography was arranged again and revealed partial resolution of the edematous changes in the retropharyngeal space. Edema of the hands and feet, bilateral bulbar conjunctivitis, and fissured lips were subsequently found. The diagnosis of Kawasaki disease was confirmed on the eighth day of fever. There was no evidence of bacterial infection. She was administered intravenous immunoglobulin (2 mg/kg) and high dose aspirin (100 mg/kg/day). One day later, the fever subsided, and her blood pressure gradually became stable. Heart echocardiography on the Day 13 revealed dilated left coronary artery and mitral regurgitation. Follow-up echocardiography six months later showed normal coronary arteries. To date, the patient has not experienced any complications. This case illustrates that retropharyngeal edema and shock syndrome can be present in the same clinical course of Kawasaki disease. Clinicians and those who work in intensive care units should be aware of unusual presentations of Kawasaki disease to decrease rates of cardiovascular complications.

The purpose of this study was to examine the role of career barriers in social cognitive career theory (R. W. Lent, S. D. Brown, & G. Hackett, 1994). The participants were 584 high school students in Taiwan, Republic of China. The gender differences in perceived career barriers and career self-efficacy were significant. Results of hierarchical regression analyses indicated that some of the perceived career barriers were significant in predicting individuals' career choice behavior after controlling for career self-efficacy in certain career types. Career counseling for high school students in Taiwan, Republic of China, is important because all the students need to declare their college majors when they apply for colleges and/or take the College Entrance Examination to get into colleges. (The College Entrance Examination is similar to the Scholastic Aptitude Test in the United States. It tests students' understanding in high school learning, including math, Chinese literature, English, natural science, and social science.) To understand factors related to their career choice behavior, empirical studies are needed to aid guidance counselors to effectively intervene to enhance students' educational status. Of the varieties of career models applied in Western culture, we believe the social cognitive career theory (SCCT) developed by Lent, Brown, and Hackett (1994) is the most appropriate one for career counseling practitioners to understand factors related to student career choice behavior. The SCCT was developed to explain the interplay among person and contextual variables within three important phases of the career development process: the formation of academic and vocational interest, selection and pursuit of career-related choices, and performance and persistence in educational and occupational endeavors. The purpose of the current study was to test the second segment of the models, that is, the choice model. Variables related to choice behavior include person variables such as self-efficacy, outcome expectation, and interest. Contextual variables, on the other hand, are mainly perceived barriers and supports. In our study, we examined the role of contextual barriers in the choice model because recent studies have shown that social-contextual factors might facilitate or impede career development in addition to the person variables such as self-efficacy and outcome expectations (Lent et al., 2001; Richie et al., 1997). In the past decade, the structure of the SCCT model has been tested in a variety of samples. More and more research has examined hypotheses involving social contextual variables in addition to the social cognitive variables. Lent et al. (2001) tested the model by using a sample composed of 111 college students. Findings indicated that self-efficacy and outcome expectations were joindy predictive of interests and choice intentions. Support and barrier percepts produced only weak direct relations to choice, although barrier percepts were found to moderate interest-choice relations. Lent et al. (2001) found that a model portraying barriers and supports as indirectly (via their impact on self-efficacy) linked to choice produced a better fit to the data than did a model specifying barriers and supports as directly linked to choice. In another study, Lent, Brown, Nota, and Soresi (2003) tested the predictions of the SCCT model. Findings based on 328 students in an introductory engineering course indicated good support for a model portraying contextual supports and barriers as indirectly linked to choice goals and actions through selfefficacy rather than direcdy as posited by SCCT. Lent et al. (2005) further examined the utility of SCCT in predicting engineering interests and major choice goals among women and men and among students at historically Black and predominandy White universities. Findings based on a sample of 487 students in introductory engineering courses at three universities indicated that the SCCT- based model of interest and choice goals produced a good fit to the data across gender and university type. …

X-linked agammaglobulinemia (XLA, also called Bruton's disease) is is an X-linked recessive disorder characterized by recurrent bacterial infections, usually occurring in the first few years of life. Here, we report the results of a BTK gene mutation screening study that was performed in Taiwanese families with the BTK gene defect to further understand the inheritance patterns of XLA patients in Taiwan and to avoid new cases of XLA within families.In this study, 52 members of 4 unrelated Taiwanese families with the BTK gene defect were enrolled. We studied the immunologic reports of 6 symptomatic living male patients with confirmed BTK gene defects and correlated the findings with their clinical symptoms. The genomic DNA of the subjects was subjected to direct sequencing mutation analysis.We screened 52 members of 4 unrelated Taiwanese families with the BTK gene defect for BTK gene mutation and found that there were 6 symptomatic living patients with a confirmed defect, 7 symptomatic deceased patients highly suspected to have had the defect and 11 asymptomatic female carriers.This is the first report in a series of the thorough screening for the BTK mutation and its carrier status in 4 unrelated Taiwanese families. One pedigree of our study comprises 4 generations. A complete BTK gene mutation study for the patient's family members is strongly suggested.

Object Currently, the effectiveness of minimally invasive evacuation of intracerebral hemorrhage (ICH) utilizing the endoscopic method is uncertain and the technique is considered investigational. The authors analyzed their experience with this method in terms of case selection, surgical technique, and long-term results. Methods The authors performed a retrospective analysis of the clinical and radiographic data obtained in 68 patients treated with endoscope-assisted ICH evacuation. Rebleeding, morbidity, and mortality were recorded as primary end points. Hematoma evacuation rate was calculated by comparing the pre- and postoperative CT scans. Glasgow Coma Scale scores and scores on the extended Glasgow Outcome Scale (GOSE) were recorded at the 6-month postoperative follow-up. The technical aspect of this report explains details of the procedure, the instruments that are used, the methods for hemostasis, and the role of hemostatic agents in the management of intraoperative hemorrhage. The pertinent literature was reviewed and summarized. Results All surgeries were performed within 12 hours of ictus, and 84% of the surgeries were performed within 4 hours. The mortality rate was 5.9%, and surgery-related morbidity occurred in 3 cases (4.4%). The hematoma evacuation rate was 93% overall—96% in the putaminal group, 86% in the thalamic group, and 98% in the subcortical group. The rebleeding rate was 1.5%. The mean operative time was 85 minutes, and the average blood loss was 56 ml. The mean GOSE score was 4.9 at 6-month follow-up. The authors acknowledge the limitations of these preliminary results in a small number of patients. Conclusions The data suggest that early endoscope-assisted ICH evacuation is safe and effective in the management of supratentorial ICH. The rebleeding, morbidity, and mortality rates are low compared with rates reported in the literature for the traditional craniotomy method. This study also showed that early and complete evacuation of ICH may lead to improved outcomes in selected patients. However, the safety and efficacy of endoscope-assisted ICH evacuation should be further investigated in a large, prospective, randomized trial.

Scleroderma is a chronic connective tissue disease characterized by hardened or scaly skin and widespread abnormalities of the viscera, which is rare in the pediatric age group.In this study, we retrospectively reviewed 23 pediatric patients suffering systemic (SSc) and localized (LS) scleroderma.Twenty-three patients were enrolled and were diagnosed with SSc or LS from March 1993 to September 2009 in the Department of Pediatrics at Mackay Memorial Hospital in Taipei, Taiwan. These diagnoses were based on the criteria of the American College of Rheumatology and the clinical manifestations of hard skin. Data recorded included sex, age-at-onset, age-at-diagnosis, laboratory data, family history, trauma history, treatment, and outcomes.Three patients suffered SSc and 20 patients had LS, including 16 girls and 7 boys. Mean age-at-onset was 6.55 +/- 3.28 years old. Antinuclear antibodies were positive in 15 patients. Tests for anti-Scl-70 antibodies were positive in 1 patient with SSc. One boy had en coup de sabre combined with a posterior fossa tumor. Twenty-two patients were treated with D-penicillamine. Oral prednisolone and methotrexate were added, if indicated. One girl with LS developed proteinuria after D-penicillamine treatment. All patients with localized disease ultimately documented a softening of their skin lesions.While scleroderma is rare in children, the prognosis of SSc is poor but better than for adults. The prognosis for LS is usually benign, however, the skin may become progressively indurated and it may not only be a skin disease. No progression from LS to SSc was observed in our study.

Pachydermodactyly (PDD), Greek for thick-skin-finger, is an infrequently recognized benign disorder characterized by painless fusiform swelling of the soft tissues around the proximal interphalangeal joints of the hands. Histopathologic features include increased dermal accumulation of collagen fibers. Young males are predominantly affected. PDD is quite rare with approximately 90 cumulative cases reported worldwide. We report three new cases of PDD in Taiwan, including two female patients. Except for patient 1 having the habit of cracking the knuckles, and patient 2 having a history of patent ductus ateriosus post catheterization, the histories of all three patients were unremarkable. X-ray of bilateral hands revealed no abnormal finding except for soft tissue swelling around proximal interphalangeal joints. Laboratory examinations all showed negative results. No local or systemic treatment was given to these patients to treat PDD, with the exception of non-steroidal anti-inflammatory drugs prescribed to one patient for a short period. Skin care with local irritation avoidance was explained to all three patients. The long-term outcome of PDD was benign.