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We report an unusual form of chorioretinal anastomosis in a 20-year-old man with chronic recurrent Vogt-Koyanagi-Harada syndrome. Several subretinal optic disc-like lesions of subretinal fibrosis were found in the vicinity of the equator. No evidence of neovascularization or arteriovenous shunt was noted.  相似文献   
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Hyperoxaluria is frequently seen in patients with inflammatory bowel disease, or after resection of the ileum. It is assumed to be responsible for the development of nephrolithiasis, nephrocalcinosis (oxalate nephrosis) and progressive renal impairment in these patients. Steatorrhea may aggravate the severity of hyperoxaluria. A 60-year-old male underwent massive resection of the jejunum and ileum 10 years prior to admission, due to strangulation of the small bowel, with occlusion of the superior mesenteric artery. He remained well except for steatorrhea which developed two-and-a-half years prior to admission, when microhematuria, proteinuria and oxaluria developed progressively. Since that time, the nephrolithiasis, nephrocalcinosis and renal failure have continued to worsen despite therapy with oxalate restriction and oxalate-binding agents. A renal biopsy, performed late in the clinical course, showed severe changes in the renal parenchyma. The decline in renal function proved irreversible. The unusual metabolic consequences of massive resection of the small intestine and their mechanisms are discussed.  相似文献   
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Fuh JL  Wang SJ  Lu SR  Juang KD  Chiu LM 《Maturitas》2001,39(2):117-124
OBJECTIVES: This paper aims to report the methodology of a study of a cohort of middle-aged women in Taiwan, their age at menopause, and related factors and prevalence of menopausal symptoms, and to examine the relationships between symptoms and sociodemographic variables. METHODS: An epidemiological study of neuropsychological change during the menopausal transition among Chinese women aged 40-54 years old on the islet of Kinmen. RESULTS: Of a targeted population of 2256 individuals, 1497 (66%) participated in the study. The mean age at menarche was 15.6 years and that at menopause was 48 years. The hormone use rate at the time of study was 23% in surgical menopausal women, and 9% were past users. After excluding surgical menopausal and premenopausal women, 6% reported a current use of estrogen replacement therapy and 6% were past users. The most frequently reported discomforts for those women aged >45 were troubled sleep, backaches, and joint pain. Four symptom clusters: musculoskeletal, non-specific somatic complaints, urogenital, and vasomotor, were identified. After adjustment for age, the urogenital and vasomotor symptoms were significantly associated with menopausal status. CONCLUSIONS: The age at menopause did not differ much from Western studies, but the menopausal symptoms, especially the vasomotor symptoms, were much lower in our study population. Nevertheless, vasomotor symptoms were still significantly associated with menopausal status.  相似文献   
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IntroductionThere are many described technique to performing laparoscopic inguinal hernia repair in children. We describe our outcomes using a percutaneous internal ring suturing technique.MethodsA retrospective review of patients under 18 years old who underwent repair between January 2014 - March 2019 was performed. A percutaneous internal ring suturing technique, involving hydro-dissection of the peritoneum, percutaneous suture passage, and cauterization of the peritoneum in the sac prior to high ligation, was used. p < 0.05 was considered significant during the analysis.Results791 patients were included. The median age at operation was 1.9 years (IQR 0.37, 5.82). The median operative time for a unilateral repair was 21 min (IQR 16, 28), while the median time for a bilateral repair was 30.5 min (IQR 23, 41).In total, 3 patients required conversion to an open procedure (0.4%), 4 (0.6%) experienced post-operative bleeding, 9 (1.2%) developed a wound infection, and iatrogenic ascent of testis occurred in 10 (1.3%) patients. Twenty patients (2.5%) developed a recurrent hernia. All but two were re-repaired laparoscopically.ConclusionsThe use of percutaneous internal ring suturing for laparoscopic repair of inguinal hernias in the pediatric population is safe and effective with a low rate of complications and recurrence.  相似文献   
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Background

Information on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited.

Objectives

The purpose of this study was to describe their characteristics and management as well as risk factors for AE recurrence.

Methods

A total of 57 patients (age ≤20 years), all with BrS and AEs, were divided into pediatric (age ≤12 years; n = 26) and adolescents (age 13 to 20 years; n = 31).

Results

Patients’ median age at time of first AE was 14 years, with a majority of males (74%), Caucasians (70%), and probands (79%) who presented as aborted cardiac arrest (84%). A significant proportion of patients (28%) exhibited fever-related AE. Family history of sudden cardiac death (SCD), prior syncope, spontaneous type 1 Brugada electrocardiogram (ECG), inducible ventricular fibrillation at electrophysiological study, and SCN5A mutations were present in 26%, 49%, 65%, 28%, and 58% of patients, respectively. The pediatric group differed from the adolescents, with a greater proportion of females, Caucasians, fever-related AEs, and spontaneous type-1 ECG. During follow-up, 68% of pediatric and 64% of adolescents had recurrent AE, with median time of 9.9 and 27.0 months, respectively. Approximately one-third of recurrent AEs occurred on quinidine therapy, and among the pediatric group, 60% of recurrent AEs were fever-related. Risk factors for recurrent AE included sinus node dysfunction, atrial arrhythmias, intraventricular conduction delay, or large S-wave on ECG lead I in the pediatric group and the presence of SCN5A mutation among adolescents.

Conclusions

Young BrS patients with AE represent a very arrhythmogenic group. Current management after first arrhythmia episode is associated with high recurrence rate. Alternative therapies, besides defibrillator implantation, should be considered.  相似文献   
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Decoy receptor 3 (DCR3) halts both Fas ligand- and LIGHT-induced cell deaths, which are required for pancreatic beta cell damage in autoimmune diabetes. To directly investigate the therapeutic potential of DCR3 in preventing this disease, we generated transgenic nonobese diabetic mice, which overexpressed DCR3 in beta cells. Transgenic DCR3 protected mice from autoimmune and cyclophosphamide-induced diabetes in a dose-dependent manner and significantly reduced the severity of insulitis. Local expression of the transgene did not alter the diabetogenic properties of systemic lymphocytes or the development of T helper 1 or T regulatory cells. The transgenic islets had a higher transplantation success rate and survived for longer than wild-type islets. We have demonstrated for the first time that the immune-evasion function of DCR3 inhibits autoimmunity and that genetic manipulation of grafts may improve the success and survival of islet transplants.  相似文献   
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