Background: and Purpose Thoroughly acquainting physicians with the effects of antiseizure medications (ASMs) is essential for developing appropriate therapeutic regimens for seizure management. This review summarizes the available evidence regarding patients receiving the antiseizure agent perampanel (PER) and its effects on the cognition, behavior, and psychological status of patients. Methods: The PubMed and Google Scholar databases were searched for all relevant articles published during 2015–2021 and without any other publication limitations, and also manually searched the reference lists in the identified articles. Outcomes of interest were changes in seizure frequency relative to baseline, 50% responder rate, seizure-free rate, and retention rate (proportion of participants continuing PER at study endpoints). Safety outcomes included adverse effects and the percentage of patients experiencing effects on cognitive, psychiatric, and behavioral symptoms. Results: We identified 139 studies, of which 28 were included after screening. Most studies found reduced seizure frequencies and satisfactory responder and retention rates, demonstrating the effectiveness and tolerability of PER. No negative effects were found for cognitive function, but a nonnegligible impact on aggressive behavior was noted when compared with other ASMs. Patients with previous psychiatric comorbidities had a greater risk of psychiatric side effects under PER treatment. PER induces an overall improvement in quality of life. Conclusions After synthesizing the study results, PER was a safe and effective choice as an additional therapy for patients with refractory epilepsy. A comprehensive evaluation of behavior and psychiatric risk is suggested before implementing PER.

Objectives: . Type 2 diabetes mellitus (T2DM) is a risk factor for deep neck infection (DNI) and leads to complications and poor outcomes. Our study aimed to investigate the risk, prognosis, and complications of peritonsillar abscess (PTA) in patients with T2DM. Methods: . We extracted data of patients newly diagnosed as having T2DM between January 2000 and December 2011 from Taiwan’s National Health Insurance Research Database. These patients were matched with patients without T2DM, and PTA incidence was compared between both cohorts. Results: . In total, 67,852 patients with and 135,704 patients without T2DM were enrolled. PTA incidence was significantly higher in patients with T2DM (incidence rate ratio, 1.91; P<0.001); moreover, PTA incidence was higher at 1 to 5 years after T2DM diagnosis than at <1 and >5 years after T2DM diagnosis. Cox regression analysis showed that patients with T2DM had an approximately 2-fold higher PTA risk (adjusted hazard ratio [aHR]: 1.89, P<0.001). Patients with a higher adapted Diabetes Complications Severity Index (aDCSI) had higher PTA risk than those with a lower aDCSI (aHRs: 2.17 for aDCSI ≥1, P=0.006 and 1.81 for aDCSI=0, P=0.002). T2DM patients with a high aDCSI (≥1) had a nonsignificantly longer hospitalization duration and a higher rate of DNI complications than did those with a low aDCSI (=0). Conclusion . In patients with T2DM, PTA incidence was relatively high, and it increased with T2DM severity. Moreover, T2DM patients should be particularly careful about PTA within 1 to 5 years after the diagnosis, and physicians should keep in mind that the prognosis of PTA was correlated with T2DM severity.

Objectives: . Type 2 diabetes mellitus (T2DM) is a risk factor for deep neck infection (DNI) and leads to complications and poor outcomes. Our study aimed to investigate the risk, prognosis, and complications of peritonsillar abscess (PTA) in patients with T2DM. Methods: . We extracted data of patients newly diagnosed as having T2DM between January 2000 and December 2011 from Taiwan’s National Health Insurance Research Database. These patients were matched with patients without T2DM, and PTA incidence was compared between both cohorts. Results: . In total, 67,852 patients with and 135,704 patients without T2DM were enrolled. PTA incidence was significantly higher in patients with T2DM (incidence rate ratio, 1.91; P<0.001); moreover, PTA incidence was higher at 1 to 5 years after T2DM diagnosis than at <1 and >5 years after T2DM diagnosis. Cox regression analysis showed that patients with T2DM had an approximately 2-fold higher PTA risk (adjusted hazard ratio [aHR]: 1.89, P<0.001). Patients with a higher adapted Diabetes Complications Severity Index (aDCSI) had higher PTA risk than those with a lower aDCSI (aHRs: 2.17 for aDCSI ≥1, P=0.006 and 1.81 for aDCSI=0, P=0.002). T2DM patients with a high aDCSI (≥1) had a nonsignificantly longer hospitalization duration and a higher rate of DNI complications than did those with a low aDCSI (=0). Conclusion . In patients with T2DM, PTA incidence was relatively high, and it increased with T2DM severity. Moreover, T2DM patients should be particularly careful about PTA within 1 to 5 years after the diagnosis, and physicians should keep in mind that the prognosis of PTA was correlated with T2DM severity.

We studied a family with Gorlin-Goltz syndrome. The novel mutations of our cases were located on the 21st exon of the PTCH1 gene (c.3450C>G). The father, who received a strategic 56-day vismodegib treatment for disease control, was the first patient with Gorlin syndrome treated with the hedgehog inhibitor in Taiwan. The lesions regressed gradually, with scar formation, and were subsequently removed via a wide excision. Further details are provided below.
Basal Cell Nevus Syndrome* ; Cicatrix ; Exons ; Fathers ; Hedgehogs ; Humans ; Taiwan

OBJECTIVETo detect the diversity of killer Ig-like receptor(KIR) gene content and the combination of haplotypes in Chinese Han population in Shanghai area.

METHODSDNA samples from 87 randomly unrelated healthy individuals in Shanghai Han population were genotyped with SSP/PCR method.

RESULTS(1) Frequencies of KIR genes: All of 18 known KIRs genes, such as 2DL1-5, 2DS1-5, 3DL1-3, 3DS1, KIR1D and the pseudogenes X, Xv and Z(KIR2DP1) were observed in Shanghai Hans. All individuals contain 3DL3, 2DL4, 3DL2 and 3DL1; the most common genes were 2DL3, Z, 2DL1 and X; the following were 2DS4, 1D, 2DL5, 2DS1, 3DS1 and 2DS5; the next were 2DS2, 2DL2, 2DS3 and Xv. (2) Frequencies of KIR gene haplotypes; there were 13 haplotypes detected in 87 Han individuals, among them, the most frequent one was type 2 (haplotypeA-2DS4). (3) Frequencies of KIR genotypes: 18 kinds of the combinations of the haplotypes were observed; the most frequent ones were AJ(2,2), AF (1,2). Also, In this study were identified five new genotypes FZ1 2 9 , FZ2 1 16 , FZ3 6 17 , FZ4 4 13 and FZ5 2 6 ,which had not been observed in Caucasians so far.

CONCLUSIONThese findings suggest that there are distinctive frequencies of KIR gene content, haplotype as well as genotype in Chinese Han population in Shanghai area.

China ; Gene Frequency ; Genetic Variation ; Genotype ; Haplotypes ; genetics ; Humans ; Receptors, Immunologic ; genetics ; Receptors, KIR ; Receptors, KIR2DL1 ; Receptors, KIR2DL3 ; Receptors, KIR2DL4 ; Receptors, KIR3DL1 ; Receptors, KIR3DL2 ; Receptors, KIR3DS1

BACKGROUND AND OBJECTIVES: Age is a traditional risk factor for open-heart surgery. The efficacy and safety of transcatheter edge-to-edge mitral valve repair, using MitraClip (Abbott Vascular), has been demonstrated in patients with severe mitral regurgitation (MR). Since octogenarians or older patients are usually deferred to receive open-heart surgery, the main interest of this study is to elucidate the procedural safety and long-term clinical impact of MitraClip in elderly patients. METHODS: Patients with symptomatic severe MR were evaluated by the heart team. For those with high or prohibitive surgical risks, transcatheter mitral valve repair was performed in hybrid operation room. Transthoracic echocardiography (TTE), blood tests, and six-minute walk test (6MWT) were performed before, 1-month, 6-months, and 1 year after index procedure. RESULTS: A total of 46 consecutive patients receiving MitraClip procedure were enrolled. Nineteen patients (84.2±4.0 years) were over 80-year-old and 27 (73.4±11.1 years) were younger than 80. Compare to baseline, the significant reduction in MR severity was achieved after the procedure and sustained. All the patients benefited from significant improvement in New York Heart Association functional class. The 6-minute walk test (6MWT) increased from 259±114 to 319±92 meters (p=0.03) at 1 year. The overall 1-year survival rate was 80% in the elderly and 88% in those <80 years, p=0.590. Baseline 6MWT was a predictor for all-cause mortality (odds ratio, 0.99; 95% confidence interval, 0.982–0.999; p=0.026) after the MitraClip procedure. CONCLUSIONS: Trans-catheter edge-to-edge mitral valve repairs are safe and have positive clinical impact in subjects with severe MR, even in advanced age.
Aged ; Aged, 80 and over ; Echocardiography ; Heart ; Hematologic Tests ; Humans ; Mitral Valve Insufficiency ; Mitral Valve ; Mortality ; Risk Factors ; Survival Rate

BACKGROUND AND OBJECTIVES: Age is a traditional risk factor for open-heart surgery. The efficacy and safety of transcatheter edge-to-edge mitral valve repair, using MitraClip (Abbott Vascular), has been demonstrated in patients with severe mitral regurgitation (MR). Since octogenarians or older patients are usually deferred to receive open-heart surgery, the main interest of this study is to elucidate the procedural safety and long-term clinical impact of MitraClip in elderly patients. METHODS: Patients with symptomatic severe MR were evaluated by the heart team. For those with high or prohibitive surgical risks, transcatheter mitral valve repair was performed in hybrid operation room. Transthoracic echocardiography (TTE), blood tests, and six-minute walk test (6MWT) were performed before, 1-month, 6-months, and 1 year after index procedure. RESULTS: A total of 46 consecutive patients receiving MitraClip procedure were enrolled. Nineteen patients (84.2±4.0 years) were over 80-year-old and 27 (73.4±11.1 years) were younger than 80. Compare to baseline, the significant reduction in MR severity was achieved after the procedure and sustained. All the patients benefited from significant improvement in New York Heart Association functional class. The 6-minute walk test (6MWT) increased from 259±114 to 319±92 meters (p=0.03) at 1 year. The overall 1-year survival rate was 80% in the elderly and 88% in those <80 years, p=0.590. Baseline 6MWT was a predictor for all-cause mortality (odds ratio, 0.99; 95% confidence interval, 0.982–0.999; p=0.026) after the MitraClip procedure. CONCLUSIONS Trans-catheter edge-to-edge mitral valve repairs are safe and have positive clinical impact in subjects with severe MR, even in advanced age.

Background/Aims: Finite nucleos(t)ide analog (NA) therapy has been proposed as an alternative treatment strategy for chronic hepatitis B (CHB), but biomarkers for post-treatment monitoring are limited. We investigated whether measuring hepatitis B core-related antigen (HBcrAg) after NA cessation may stratify the risk of subsequent clinical relapse (CR). Methods: This retrospective multicenter analysis enrolled adults with CHB who were prospectively monitored after discontinuing entecavir or tenofovir with negative HBeAg and undetectable HBV DNA at the end of treatment (EOT). Patients with cirrhosis or malignancy were excluded. CR was defined as serum alanine aminotransferase > two times the upper limit of normal with recurrent viremia. We applied time-dependent Cox proportional hazard models to clarify the association between HBcrAg levels and subsequent CR. Results: The cohort included 203 patients (median age, 49.8 years; 76.8% male; 60.6% entecavir) who had been treated for a median of 36.9 months (interquartile range [IQR], 36.5–40.1). During a median post-treatment follow-up of 31.7 months (IQR, 16.7–67.1), CR occurred in 104 patients with a 5-year cumulative incidence of 54.8% (95% confidence interval [CI], 47.1–62.4%). Time-varying HBcrAg level was a significant risk factor for subsequent CR (adjusted hazard ratio [aHR], 1.53 per log U/mL; 95% CI, 1.12–2.08) with adjustment for EOT HBsAg, EOT anti-HBe, EOT HBcrAg and time-varying HBsAg. During follow-up, HBcrAg <1,000 U/mL predicted a lower risk of CR (aHR, 0.41; 95% CI, 0.21–0.81). Conclusions Dynamic measurement of HBcrAg after NA cessation is predictive of subsequent CR and may be useful to guide post-treatment monitoring.