BACKGROUND: Several factors, including clinical manifestations and laboratory data, have been used to evaluate the disease activity of Sjögren's syndrome (SS). We investigated saliva indicators of disease activity in primary SS patients. METHODS: We enrolled 138 Taiwanese patients with primary SS and 100 Taiwanese normal controls. Interleukin (IL)-6, IL-17A, tumor necrosis factor-alpha (TNF-α), and rheumatoid factor (RF)-IgA levels in saliva samples were measured using ELISA or fluorescent enzyme-linked immunoassay. Serum IgG, IgA, and IgM levels were measured by nephelometry. Erythrocyte sedimentation rate (ESR) was measured with an automatic ESR analyzer. The t-test and Pearson correlation test were used. RESULTS: IL-6 level was higher in primary SS patients than in normal controls (14.23±14.77 vs 9.87±7.32, P=0.012), but there were no significant differences in IL-17A, TNF-α, and RF-IgA levels. In primary SS patients, IL-6 level correlated weakly with ESR and IgG levels (r=0.252, P=0.015, and r=0.248, P=0.017, respectively), and TNF-α level correlated weakly with IgG level (r=0.231, P=0.024). CONCLUSIONS: IL-6 may play a role in SS pathogenesis. Saliva IL-6 might be an indicator of disease activity in primary SS patients.
Blood Sedimentation ; Enzyme-Linked Immunosorbent Assay ; Humans ; Immunoassay ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Interleukin-17 ; Interleukin-6 ; Interleukins ; Nephelometry and Turbidimetry ; Rheumatoid Factor ; Saliva ; Tumor Necrosis Factor-alpha

As diagnostic techniques have advanced, primary aldosteronism (PA) has emerged as the most common cause of secondary hypertension. The excess of aldosterone caused by PA resulted in not only cardiovascular complications, including coronary artery disease, myocardial infarction, arrhythmia, and heart failure, but also cerebrovascular complications, such as stroke and transient ischemic attack. Moreover, PA is associated more closely with these conditions than is essential hypertension. In this review, we present up-to-date findings on the association between PA and cerebrovascular diseases.
Aldosterone ; Arrhythmias, Cardiac ; Cerebrovascular Disorders* ; Coronary Artery Disease ; Heart Failure ; Hyperaldosteronism* ; Hypertension ; Ischemic Attack, Transient ; Myocardial Infarction ; Stroke

AIMTo determine the deletion junctions of infertile men in Taiwan with azoospermia factor region c (AZFc) deletions and to evaluate the genotype/phenotype correlation.

METHODSGenomic DNAs from 460 infertile men were examined. Bacterial artificial chromosome clones were used to verify the accuracy of polymerase chain reaction. Deletion junctions of the AZFc region were determined by analysis of sequence-tagged sites and gene-specific markers.

RESULTSComplete AZFc deletions, including BPY2, CDY1 and DAZ genes, were identified in 24 men. The proximal breakpoints were clustered between sY1197 and sY1192, and the distal breakpoints were clustered between sY1054 and sY1125 in all but one of the 24 men. The testicular phenotypes of men with complete AZFc deletion varied from oligozoospermia, to hypospermatogenesis, to maturation arrest.

CONCLUSIONWe identified a group of infertile men with uniform deletion junctions of AZFc in the Taiwan population. Despite this homogeneous genetic defect in the AZFc region, no clear genotype/phenotype correlation could be demonstrated.

Asian Continental Ancestry Group ; genetics ; Base Sequence ; Chromosomes, Human, Y ; genetics ; DNA Primers ; Deleted in Azoospermia 1 Protein ; Gene Deletion ; Genetic Loci ; Humans ; Infertility, Male ; genetics ; Male ; Nuclear Proteins ; genetics ; Oligospermia ; genetics ; Phenotype ; Polymerase Chain Reaction ; Proteins ; genetics ; RNA-Binding Proteins ; genetics ; Seminal Plasma Proteins ; genetics ; Taiwan ; Testis ; anatomy & histology

Objective: Hypoactivity in the reward system among patients with attention deficit hyperactivity disorder (ADHD) is a well-known phenomenon. Whether the activity in the reward pathway is related to harm avoidance, such as in sensitivity to punishment, is unclear. Evidence regarding the potential difference between ADHD patients and controls in terms of this association is scarce. Methods: Event-related functional magnetic resonance imaging was conducted on subjects performing the Iowa gambling test. Fourteen adults with ADHD and 14 controls were enrolled in the study. Results: Harm avoidance was found to be positively correlated with the activities of the bilateral orbitofrontal cortex and right insula in individuals with ADHD. A group difference was also confirmed. Conclusion Understanding the roles of harm avoidance and brain activation during risk tasks is important.

Hypoglycemia is a common finding in both daily clinical practice and acute care settings. The causes of severe hypoglycemia (SH) are multi-factorial and the major etiologies are iatrogenic, infectious diseases with sepsis and tumor or autoimmune diseases. With the advent of aggressive lowering of HbA1c values to achieve optimal glycemic control, patients are at increased risk of hypoglycemic episodes. Iatrogenic hypoglycemia can cause recurrent morbidity, sometime irreversible neurologic complications and even death, and further preclude maintenance of euglycemia over a lifetime of diabetes. Recent studies have shown that hypoglycemia is associated with adverse outcomes in many acute illnesses. In addition, hypoglycemia is associated with increased mortality among elderly and non-diabetic hospitalized patients. Clinicians should have high clinical suspicion of subtle symptoms of hypoglycemia and provide prompt treatment. Clinicians should know that hypoglycemia is associated with considerable adverse outcomes in many acute critical illnesses. In order to reduce hypoglycemia-associated morbidity and mortality, timely health education programs and close monitoring should be applied to those diabetic patients presenting to the Emergency Department with SH. ED disposition strategies should be further validated and justified to achieve balance between the benefits of euglycemia and the risks of SH. We discuss relevant issues regarding hypoglycemia in emergency and critical care settings.
Diabetes Mellitus/drug therapy ; Humans ; Hypoglycemia/blood/*chemically induced/*complications/epidemiology ; Hypoglycemic Agents/adverse effects/therapeutic use ; Insulin/adverse effects/therapeutic use

PURPOSE: The aim of this study is to explore non-steroid anti-inflammation drugs (NSAIDs) potency for pelvic floor muscle pain by measuring local concentration in a rat model. MATERIALS AND METHODS: We used nine NSAIDs, including nabumetone, naproxen, ibuprofen, meloxicam, piroxicam, diclofenac potassium, etodolac, indomethacin, and sulindac, and 9 groups of female Wister rats. Each group of rats was fed with one kind of NSAID (2 mg/mL) for three consecutive days. Thereafter, one mL of blood and one gram of pelvic floor muscle were taken to measure drug pharmacokinetics, including partition coefficient, lipophilicity, elimination of half-life (T1/2) and muscle/plasma converting ratio (Css, muscle/Css, plasma). RESULTS: Diclofenac potassium had the lowest T1/2 and the highest mean Css, muscle/Css, plasma (1.9 hours and 0.85+/-0.53, respectively). The mean Css, muscle/Css, plasma of sulindac, naproxen and ibuprofen were lower than other experimental NSAIDs. CONCLUSION: Diclofenac potassium had the highest disposition in pelvic floor muscle in a rat model. The finding implies that diclofenac potassium might be the choice for pain relief in pelvic muscle.
Animals ; Anti-Inflammatory Agents, Non-Steroidal/*therapeutic use ; Butanones/therapeutic use ; Chronic Pain/*drug therapy ; Diclofenac/therapeutic use ; Female ; Muscles/drug effects ; Naproxen/therapeutic use ; Pelvic Floor/*pathology ; Pelvic Pain/*drug therapy ; Piroxicam/therapeutic use ; Rats ; Rats, Wistar ; Thiazines/therapeutic use ; Thiazoles/therapeutic use

OBJECTIVE: Angiomyolipoma is the most common benign kidney tumor. However, literature describing FDG PET findings on renal angiomyolipoma (AML) is limited. This study reports the FDG PET and PET/CT findings of 21 cases of renal AML. MATERIALS AND METHODS: The study reviews FDG PET and PET/CT images of 21 patients diagnosed with renal AML. The diagnosis is based on the classical appearance of an AML on CT scan with active surveillance for 6 months. The study is focused on the observation of clinical and radiographic features. RESULTS: Six men and 15 women were included in our study. The mean age of the patients was 57.14 +/- 9.67 years old. The mean diameter of 21 renal AML on CT scans was 1.76 +/- 1.00 cm (Min: 0.6 cm; Max: 4.4 cm). CT scans illustrated renal masses typical of AMLs, and the corresponding FDG PET scans showed minimal FDG activities in the area of the tumors. None of the 21 AMLs showed a maximum standardized uptake value (SUVmax) greater than 1.98. No statistically significant correlation was present between SUVmax and tumor size. CONCLUSION: Renal AMLs demonstrate very low to low uptake on FDG PET and PET/CT imaging in this study. When a fat-containing tumor in the kidney is found on a CT scan, it is critical to differentiate an AML from a malignant tumor including an RCC, liposarcoma, and Wilms tumor. This study suggests that FDG PET or PET/CT imaging is useful for differentiating a renal AML from a fat-containing malignant tumor.
Angiomyolipoma/*radionuclide imaging ; Contrast Media/diagnostic use ; Ehlers-Danlos Syndrome ; Female ; Fluorodeoxyglucose F18/diagnostic use ; Humans ; Kidney Neoplasms/*radionuclide imaging ; Male ; Middle Aged ; *Positron-Emission Tomography ; *Positron-Emission Tomography and Computed Tomography ; Radiopharmaceuticals/diagnostic use ; Retrospective Studies

BACKGROUND/AIMS: Only moderate to severe Crohn's Disease (CD) patients without a satisfactory conventional therapy effect are eligible to get reimbursement from the National Health Insurance of Taiwan for using adalimumab. These are more stringent criteria than in many Western countries and Japan and Korea. We aim to explore the efficacy of using adalimumab in CD patients under such stringent criteria. METHODS: A retrospective analysis was conducted in nine medical centers in Taiwan and we collected the results of CD patients receiving adalimumab from Sep 2009 to Mar 2014. The clinical characteristics, response measured by CDAI (Crohn's Disease Activity Index), adverse events and survival status were recorded and analyzed. CR-70, CR-100, and CR-150 were defined as attaining a CDAI decrease of 70, 100 or 150 points compared with baseline. RESULTS: A total of 103 CD patient records were used in this study. Sixty percent of these patients received combination therapy of adalimumab together with immunomodulators. CR-70 was 68.7%, 74.5% and 88.4% after week 4, 8 and 12 of treatment, respectively. The steroid-free rate, complications and survival were 47.6%, 9.7% and 99% of patients, respectively. In considering the mucosal healing, only 25% patients achieve mucosal healing after treatment for 6 to12 months. Surgery was still needed in 16.5% of patients. Combination treatment of adalimumab with immunomodulators further decreased the level of CDAI at week 8 when compared with the monotherapy. CONCLUSIONS: Even under the stringent criteria for using adalimumab, the response rate was comparable to those without stringent criteria.
Adalimumab ; Crohn Disease* ; Humans ; Immunologic Factors ; Inflammatory Bowel Diseases* ; Japan ; Korea ; National Health Programs ; Retrospective Studies ; Taiwan*

AIMTo complete comprehensive haplotype analysis of USP26 for both fertile and infertile men.

METHODSTwo hundred infertile men with severe oligospermia or non-obstructive azoospermia were subjected to sequence analysis for the entire coding sequences of the USP26 gene. Two hundred men with proven fertility were genotyped by primer extension methods. Allele/genotype frequencies, linkage disequilibrium (LD) characteristics and haplotypes of fertile men were compared with infertile men.

RESULTSThe allele frequencies of five single nucleotide polymorphisms (370-371insACA, 494T>C, 576G>A, ss6202791C>T, 1737G>A) were significantly higher in infertile patients than control subjects. The major haplotypes in infertile men were TACCGA (28% of the population), TGCCGA (15%), TACCAA (8%), TGCCAA (6%), TATCAA (5%) and CATCAA (5%). The major haplotypes for the control subjects were TACCGA (58% of the population), CACCGA (7%), CATCGA (6%) and TGCCGA (5%). Haplotypes TGCCGA, TATCAA, CATCAA, CATCGC, TACCAA and TGCCAA were over-transmitted in patients with spermatogenic defect, whereas haplotypes TACCGA, CACCGA, and CATCGA were under-transmitted in these patients.

CONCLUSIONSome USP26 alleles and haplotypes are associated with spermatogenic defect in the Han nationality in Taiwan, China.

Adult ; Alleles ; Azoospermia ; epidemiology ; genetics ; Cysteine Endopeptidases ; genetics ; DNA Primers ; Gene Frequency ; Genetic Variation ; Genotype ; Haplotypes ; Humans ; Infertility, Male ; epidemiology ; genetics ; Linkage Disequilibrium ; Male ; Multigene Family ; Oligospermia ; epidemiology ; genetics ; Polymorphism, Genetic ; Spermatogenesis ; genetics ; physiology ; Taiwan ; epidemiology

PURPOSE: Aortic dissection (AoD) is one of the most common catastrophes involving the aorta. Nevertheless, early diagnosis remains to be a challenge in the Emergency Department (ED), particularly in young individuals. In this study, we attempted to identify the characteristics of acute AoD among young individuals, particular in patients with Marfan syndrome. MATERIALS AND METHODS: This was an retrospective chart-review study conducted in a tertiary referring hospital. The hospital database was queried for the combination of AoD and patients under age of 40 years. The medical charts were reviewed to obtain demographic data, clinical data and laboratory characteristics by using a standardized data collection sheet. A comparison between Marfan syndrome and non-Marfan syndrome patients was performed. RESULTS: During the 10-years period, 18 of 344 patients with acute AoD were younger than 40 years-old. Patients with Marfan syndrome developed acute AoD at a younger age than patients without Marfan syndrome. The mean diastolic blood pressure was significantly lower in patients with Marfan syndrome upon presenting to the ED than those without. Patients with Marfan syndrome had trends toward higher risk of development of type A AoD, increased recurrence rate and higher mortality rate than those without. However, statistical significance was not present. CONCLUSION: ED physicians should have high alert to acute AoD in young patients presenting with severe unexplained chest and back pain, particularly in those patients with a history of heart diseases, hypertension, and Marfan syndrome or featuring Marfanoid habitus. Acute coronary syndrome, unexplained abdominal symptoms, and sudden cardiac arrest could be the initial manifestation of AoD in young patients. A low threshold to perform enhanced computed tomography may facilitate early diagnosis and timely treatment in this patient population.
Adult ; Aneurysm, Dissecting/*complications/epidemiology/*pathology ; Aortic Aneurysm/complications/epidemiology/*pathology ; Asian Continental Ancestry Group ; Female ; Heart Defects, Congenital/complications/physiopathology ; Humans ; Hypertension/complications/physiopathology ; Male ; Marfan Syndrome/*complications/*physiopathology ; Middle Aged ; Retrospective Studies ; Young Adult