Novel Pathogenic Mutation of PNPLA1 Identified in Autosomal Recessive Congenital Ichthyosis: A Case Report / 中国医学科学杂志(英文版)

Autosomal recessive congenital ichthyosis (ARCI) is characterized by being born as collodion babies, hyperkeratosis, and skin scaling. We described a collodion baby at birth with mild ectropion, eclabium, and syndactyly. Whole exome sequencing showed a compound heterozygous variant c.[56C>A], p.(Ser19X) and c.[100G>A], p.(Ala34Thr) in the PNPLA1 gene [NM_001145717; exon 1]. The protein encoded by PNPLA1 acts as a unique transacylase that specifically transfers linoleic acid from triglyceride to ω-hydroxy fatty acid in ceramide, thus giving rise to ω-O-acylceramide, a particular class of sphingolipids that is essential for skin barrier function. The variant was located in the patatin core domain of PNPLA1 and resulted in a truncated protein which could disrupt the function of the protein. This case report highlights a novel compound heterozygous mutation in PNPLA1 identified in a Chinese child.

A Case of Self-healing Collodion Baby / 대한피부과학회지

No abstract available.

Preparation and immunogenicity of influenza virus-like particles using nitrocellulose membrane filtration

PURPOSE: Nitrocellulose membrane–based filtration system (NCFS) is widely used for protein concentration. In this study, we applied NCFS for production of virus-like particle (VLP) as a vaccine candidate and evaluated yield property and immunogenicity. MATERIALS AND METHODS: Influenza VLPs were generated by baculovirus-insect cell protein expression system. NCFS and sucrose gradient ultracentrifugation were used for purification of VLP. Immunogenicity of VLP was evaluated by animal experiment. RESULTS: Influenza VLPs expressing hemagglutinin (HA) and neuraminidase proteins derived from highly pathogenic influenza virus (H5N8) were effectively produced and purified by NCFS. HA activity of VLP which correlated with antigenicity was well conserved during multiple purification steps. This NCFS based purified VLPs induced influenza virus–specific antibody responses. CONCLUSION: Our results indicate that the influenza VLP vaccine could be prepared by NCFS without loss of immunogenicity and elicit antigen-specific immune responses.

Development of an immunochromatographic strip for detection of antibodies against porcine reproductive and respiratory syndrome virus

A simple and rapid immunochromatographic test strip incorporating a colloidal gold-labeled recombinant Nsp7 antigen probe was successfully developed for the detection of anti-porcine reproductive and respiratory syndrome virus (PRRSV) antibodies in swine. Recombinant Nsp7 protein of PRRSV labeled with colloidal gold was dispensed on a conjugate pad for use as the detector. Staphylococcal protein A and purified porcine anti-Nsp7 antibodies were blotted on a nitrocellulose membrane to form test and control lines, respectively. A comparison of the strip with standard diagnostic tests, enzyme-linked immunosorbent assays and immunoperoxidase monolayer assay, was also performed. The immunochromatographic test strip was shown to be of high specificity and sensitivity. Furthermore, the strip assay is rapid and easy to perform with no requirement for professional-level skills or equipment. It is suggested that the immunochromatographic test strip can be used to quickly and accurately detect PRRSV antibody and to be suitable for diagnostic purposes in the field.

Elution of urinary proteins preserved on nitrocellulose membrane with heating / 生物工程学报

The preservation of urinary proteins on a membrane plays a vital role in biomarker research, and the efficient elution of proteins preserved on nitrocellulose membrane (NC membrane) determines the application of this method. During the heating elution procedure, we raised the temperature to reduce the intense vortexing time, and kept gentle rotating while precipitation to prevent nitrocellulose reformation. We also used SDS-PAGE and LC-MS/MS to analyze the urinary proteins prepared by heating elution procedure, intense vortexing elution procedure and acetone precipitation method. There was no degradation of proteins prepared by heating elution procedure. Compared with proteins prepared by heating elution method and acetone precipitation method, the overlapping rates of the proteins was almost the same (92.6% versus 96.8%) and the ratios of CV values (< 20%) of the proteins were both high (85.2% and 94.4%). The heating elution procedure achieved good technical reproducibility, and was much simpler and more efficient than the previous one. It can facilitate the application of the preservation of urinary proteins on membrane.

Comparison of two urinary protein preparation methods: nitrocellulose membrane preservation and acetone precipitation / 生物工程学报

Nitrocellulose membrane based urinary protein preservation method is simple, fast and economic, but its advantage over the traditionally used acetone precipitation method is still unclear. In this work, we prepared urinary proteins by the two methods by LC-MS/MS. Then we used protein spectra counts to assess the reproducibility of the two methods. Proteins identified by the two methods were almost the same in number, spectral count distribution and distribution of coefficients of variation value. In conclusion, nitrocellulose membrane method is generally the same as acetone precipitation method. It can be used for large scale preservation of clinical urine samples.

The effect of additional etching and curing mechanism of composite resin on the dentin bond strength

PURPOSE: The aim of this study was to evaluate the effects of additional acid etching and curing mechanism (light-curing or self-curing) of a composite resin on the dentin bond strength and compatibility of one-step self-etching adhesives. MATERIALS AND METHODS: Sixteen human permanent molars were randomly divided into eight groups according to the adhesives used (All-Bond Universal: ABU, Clearfil S3 Bond: CS3), additional acid etching (additional acid etching performed: EO, no additional acid etching performed: EX), and composite resins (Filtek Z-250: Z250, Clearfil FII New Bond: CFNB). Group 1: ABU-EO-Z250, Group 2: ABU-EO-CFNB, Group 3: ABU-EX-Z250, Group 4: ABU-EX-CFNB, Group 5: CS3-EO-Z250, Group 6: CS3-EO-CFNB, Group 7: CS3-EX-Z250, Group 8: CS3-EX-CFNB. After bonding procedures, composite resins were built up on dentin surfaces. After 24-hour water storage, the teeth were sectioned to make 10 specimens for each group. The microtensile bond strength test was performed using a microtensile testing machine. The failure mode of the fractured specimens was examined by means of an optical microscope at x20 magnification. The data was analyzed using a one-way ANOVA and Scheffe's post-hoc test (alpha=.05). RESULTS: Additional etching groups showed significantly higher values than the no additional etching group when using All-Bond Universal. The light-cured composite resin groups showed significantly higher values than the self-cured composite resin groups in the Clearfil S3 Bond. CONCLUSION: The additional acid etching is beneficial for the dentin bond strength when using low acidic one-step self-etch adhesives, and low acidic one-step self-etch adhesives are compatible with self-cured composite resin. The acidity of the one-step self-etch adhesives is an influencing factor in terms of the dentin bonding strength and incompatibility with a self-cured composite resin.

Bond strength of veneer ceramic and zirconia cores with different surface modifications after microwave sintering

PURPOSE: To evaluate the effects of surface treatments on shear bond strength (SBS) between microwave and conventionally sintered zirconia core/veneers. MATERIALS AND METHODS: 96 disc shaped Noritake Alliance zirconia specimens were fabricated using YenaDent CAM unit and were divided in 2 groups with respect to microwave or conventional methods (n=48/group). Surface roughness (Ra) evaluation was made with a profilometer on randomly selected microwave (n=10) and conventionally sintered (n=10) cores. Specimens were then assessed into 4 subgroups according to surface treatments applied (n=12/group). Groups for microwave (M) and conventionally (C) sintered core specimens were as follows; M(C),C(C): untreated (control group), M1,C1:Al2O3 sandblasting, M2,C2:liner, M3,C3:Al2O3 sandblasting followed by liner. Veneer ceramic was fired on zirconia cores and specimens were thermocycled (6000 cycles between 5degrees-55degrees C). All specimens were subjected to SBS test using a universal testing machine at 0.5 mm/min, failure were evaluated under an optical microscope. Data were statistically analyzed using Shapiro Wilk, Levene, Post-hoc Tukey HSD and Student's t tests, Two-Way-Variance-Analysis and One-Way-Variance-Analysis (alpha=.05). RESULTS: Conventionally sintered specimens (1.06 +/- 0.32 microm) showed rougher surfaces compared to microwave sintered ones (0.76 +/- 0.32 microm)(P=.046), however, no correlation was found between SBS and surface roughness (r=-0.109, P=.658). The statistical comparison of the shear bond strengths of C3 and C1 group (P=.015); C(C) and M(C) group (P=.004) and C3 and M3 group presented statistically higher (P=.005) values. While adhesive failure was not seen in any of the groups, cohesive and combined patterns were seen in all groups. CONCLUSION: Based on the results of this in-vitro study, Al2O3-sandblasting followed by liner application on conventionally sintered zirconia cores may be preferred to enhance bond strength.

Debonding forces of three different customized bases of a lingual bracket system / 대한치과교정학회지

OBJECTIVE: The purpose of this study was to investigate whether extension of the custom base is necessary for enhancement of bond strength, by comparing the debonding forces and residual adhesives of 3 different lingual bracket systems. METHODS: A total of 42 extracted upper premolars were randomly divided into 3 groups of 14 each for bonding with brackets having (1) a conventional limited resin custom base; (2) an extended gold alloy custom base: Incognito(TM); and (3) an extended resin custom base: KommonBase(TM). The bonding area was measured by scanning the bracket bases with a 3-dimensional digital scanner. The debonding force was measured with an Instron universal testing machine, which applied an occlusogingival shear force. RESULTS: The mean debonding forces were 60.83 N (standard deviation [SD] 10.12), 69.29 N (SD 9.59), and 104.35 N (SD17.84) for the limited resin custom base, extended gold alloy custom base, and extended resin custom base, respectively. The debonding force observed with the extended resin custom base was significantly different from that observed with the other bases. In addition, the adhesive remnant index was significantly higher with the extended gold alloy custom base. CONCLUSIONS: All 3 custom-base lingual brackets can withstand occlusal and orthodontic forces. We conclude that effective bonding of lingual brackets can be obtained without extension of the custom base.

Comparative study of fracture strength depending on the occlusal thickness of full zirconia crown / 대한치과보철학회지

PURPOSE: The purpose of this study was to compare the fracture strength of traditional metal-ceramic crowns and full zirconia crowns according to the occlusal thickness. MATERIALS AND METHODS: A mandibular first molar resin tooth was prepared with 1.5 mm occlusal reduction, 1.0 mm rounded shoulder margin and 6degrees taperness in the axial wall. Duplicating the resin tooth, 64 metal dies were fabricated. 48 full zirconia crowns were fabricated using Prettau zirconia blanks by ZIRKONZAHN CAD/CAM and classified into six groups according to the occlusal thickness (0.5 mm, 0.6 mm, 0.7 mm, 0.8 mm, 0.9 mm, 1.0 mm). 16 metal-ceramic crowns were fabricated and classified into two groups according to the occlusal porcelain thickness (1.0 mm, 1.5 mm). All crowns were cemented on each metal die and mounted in a universal testing machine. The load was directed at the functional cusp of each specimen until catastrophic failure occurred. One-way ANOVA, Tukey multiple comparison test (alpha=.05) and t-test (alpha=.05) were used. RESULTS: The results were as follows. 1. The test 1 group (646.48 N) showed the lowest fracture strength (P.05). The value of test 6 group (1781.24 N) was significantly higher than those of the other groups (P<.05). 2. There were no significant differences of the fracture strength of metal ceramic crowns according to occlusal porcelain thickness 1.0 mm (2515.71 N) and 1.5 mm (3473.31 N) (P<.05). CONCLUSION: Full zirconia crown needs to be 1.0 mm or over in occlusal thickness for the posterior area to have higher fracture strength than maximum bite force.

Denaturing Gradient Gel Electrophoresis for Detection of Bacterial Population and Diversity in Amniotic Fluids and Neonatal Gastric Fluids

PURPOSE: Intra-amniotic infection (IAI) is often polymicrobial, and the 16S rDNA PCR assay has a major limitation that its interpretation is difficult in the presence of multiple 16S rDNAs. Denaturing gradient gel electrophoresis (DGGE) can overcome this limitation by separating PCR products based on sequence. We performed the DGGE analysis to investigate bacterial prevalence and diversity in amniotic fluids from pregnant women with preterm births and gastric fluids from their newborns. METHODS: DNA was extracted from bacterial cells in amniotic fluid (AF) and gastric fluid (GF) and was amplified with universal 16S rDNA primers. For DGGE analysis, the PCR products were loaded onto polyacrylamide gels that were made with denaturing gradients. RESULTS: Bacterial 16S rDNA was detected by PCR from all AF and GF samples. The bacterial species in AF samples were the following: Lactobacillus reuteri (87.0%), uncultured Enterococcus species (65.2%), Ureaplasma urealyticum (13.0%), and Enterococcus faecalis (4.3%). The bacterial species in GF samples were the following: Lactobacillus reuteri (95.2%), uncultured Enterococcus species (42.9%), and Ureaplasma urealyticum (4.8%). Two or more species were identified from 69.6% of AF and 47.6% of GF samples. CONCLUSION: We suggest that DGGE analysis allows improved understanding of microbial diversity and community in AF and GF.

Prevention of venous thromboembolism with enoxaparin in bariatirc surgery / 대한외과학회지

PURPOSE: Venous thromboembolism (VTE) after bariatric surgery is a significant cause of morbidity and mortality. Current modalities of thromboprophylaxis include subcutaneous injection of unfractionated or low-molecular-weight heparin (LMWH), pneumatic compression, elastic stockings, and inferior vena cava filters. Despite universal agreement on the need for thromboprophylaxis, no clear consensus has been reached regarding the best regimen and treatment duration of bariatric surgery. METHODS: From April, 2009 to December, 2011, we performed 200 bariatric surgery (191 with primary intent, 9 with revisional intent). There was no history of VTE prior to surgery. Clexane therapy was done with 4000 U SQ once daily for 2 weeks to the day before surgery. Development of VTE was assessed by direct interview, physical examination in out-patient clinic, and phone calls to patients for history taking if needed. The history taking was presented in questionnaire format. The patients were asked to state their symptoms of VTE by answering the questionnaire. The patients were followed up for a minimum of 6 months after surgery to determine the incidence of clinical VTE. RESULTS: Two-week Clexane therapy was completed in 193 patients. Clexane was stopped in 5 due to surgical related complications (4 bleeding, 1 reoperation due to leak), in 2 due to Clexane related complications (1 epistaxis, 1 metrorrhagia). Follow-up of out-patient clinic were 68%, those who could follow up by telephone were 89%. There was no evidence of VTE. CONCLUSION: A 2-week VTE prophylaxis regimen using LMWH is simple, effective and associated with a low incidence of complications.

Effects of contamination by either blood or a hemostatic agent on the shear bond strength of orthodontic buttons / 대한치과교정학회지

OBJECTIVE: To evaluate the effects of contamination by either blood or a hemostatic agent on the shear bond strength (SBS) of orthodontic buttons. METHODS: We used 45 freshly extracted, non-carious, impacted third molars that were divided into 3 groups of 15. Each tooth was etched with 37% phosphoric acid gel for 30 s. Human blood or the blood stopper agent was applied to the tooth surface in groups I and II, respectively. Group III teeth were untreated (controls). Orthodontic buttons were bonded to the teeth using light-curing composite resin. After bonding, the SBS of the button was determined using a Universal testing machine. Any adhesive remaining after debonding was assessed and scored according to the modified adhesive remnant index (ARI). ANOVA with post-hoc Tukey's test was used to determine significant differences in SBS and Fisher's exact test, to determine significant differences in ARI scores among groups. RESULTS: ANOVA indicated a significant difference between groups (p < 0.001). The highest SBS values were measured in group III (10.73 +/- 0.96 MPa). The SBS values for teeth in groups I and II were significantly lower than that of group III (p < 0.001). The lowest SBS values were observed in group I teeth (4.17 +/- 1.11 MPa) (p < 0.001). CONCLUSIONS: Contamination of tooth surfaces with either blood or hemostatic agent significantly decreased the SBS of orthodontic buttons. When the contamination risk is high, it is recommended to use the blood stopper agent when bonding orthodontic buttons on impacted teeth.

Shear bond strength of the three different kinds of resin cement on CAD/CAM ceramic inlay / 대한치과보철학회지

PURPOSE: The purpose of this study was to evaluate the bond strengths between the latest CAD/CAM ceramic inlay and various resin cements which are used primarily for esthetic restoration. MATERIALS AND METHODS: Cylindrical ceramic blocks(Height: 5 mm, diameter: 3 mm) were fabricated by using Cerec3 and bonded on the dentin of the ninety extracted caries-free molars using three different kinds of resin cement(Unicem(R), Biscem(R), and Variolink II(R)) according to the manufacturer's instructions. Ninety specimens were divided into 3 groups according to three different kinds of resin cement. Half of each group were conducted thermocycling under the conditions of the 5 - 55degrees C, 5,000 cycle but the other half of them weren't. All specimens were kept in normal saline 37degrees C, for 24 hours before measuring the bond strength. The shear bond strength was measured by Universal testing machine with a cross head speed of 0.5 mm/min. The results were analyzed statistically by t-test and one-way ANOVA. RESULTS: Unicem(R) group showed the highest shear bond strength despite a slight decline by thermocycling. The shear bond strength of Unicem(R) group and ValiolinkII(R) group were significantly influenced by thermocycling, whereas Biscem(R) group was not influenced (P<.05). There were no significant differences in the bond strength between the three groups without thermocycling, but there was significant differences between Unicem(R) group and Valiolink II(R) group with thermocycling(P<.05). CONCLUSION: It has been shown to be clinically effective when the self-adhesive resin cements Unicem(R) and Biscem(R) were used instead of the etch-and-rinse resin cement Valiolink II(R) during the bonding of CAD/CAM ceramic inlay restorations with teeth.

Cardiopulmonary resuscitation update

Sudden cardiac arrest is a growing medical issue in developed countries. Annually, more than 25,000 out-of-hospital cardiac arrests (OHCA) occur in Korea. Only 3% of victims with OHCA discharge alive from hospital and less than 1% of them survive neurologically intact. Major changes of recent guidelines for cardiopulmonary resuscitation (CPR) and emergency cardiac care includes modification of basic life support (BLS) sequence from A-B-C to C-A-B, an emphasis on minimally interrupted, high-quality chest compression, the introduction of chest compression-only CPR, and addition of integrated post-cardiac arrest care concept as the fifth chain in the Chain of Survival. Repetition of 2-minutes of CPR, rhythm check, and defibrillation if indicated is recommended as a universal algorithm during BLS. Defibrillation and drug administration including epinephrine should not be delayed to place an advanced airway during CPR. Important interventions during post-cardiac arrest care are comprised of the optimization of ventilation (arterial CO2 tension, 40 to 45 mmHg) and oxygenation (arterial O2 saturation, 94% to 98%), glucose control (blood glucose, 144 to 180 mg/dL), therapeutic hypothermia (body tem-perature, 32degrees C to 34degrees C) for unresponsive patients, and percutaneous coronary intervention for the patient with ST-segment elevation. Systemic approaches to increase public awareness of cardiac arrest and CPR, to spread CPR education to citizen, and to implement public access defibrillation are a prerequisite for improving survival from OHCA in the community. Effective advanced life support and integrated post-cardiac arrest care should be provided to increase neurologically intact survival among the patients resuscitated from cardiac arrest.

The current state of dyslipidemia in Korean children and adolescents and its management in clinical practice

Cardiovascular disease (CVD) is a leading cause of death worldwide including Korea. The risk factors of CVD are known as positive family history of early CVD, obesity, hypertension, diabetes, and dyslipidemia. Among those, dyslipidemia is one of modifiable risk factors. Dyslipidemia starts in childhood and progress to adulthood. Furthermore, dyslipidemia cause atherosclerosis and is closely related to other CVD risks. On the rationale that early identification and control of pediatric dyslipidemia will reduce the risk and severity of CVD in adulthood, the National Heart, Lung, and Blood Institute guidelines expanded to universal screening for lipid levels. However, there was no guideline for lipid screening and management in Korean children and adolescents yet. This review deals with the rationale of early identification and control of pediatric dyslipidemia along with the current Korean status of pediatric dyslipidemia. This review also deals with how to screen, diagnosis, and treatment of pediatric dyslipidemia.

A Case of Systemic Lupus Erythematosus Occurring after H1N1 Influenza Vaccination / 대한내과학회지

The recent worldwide outbreak of H1N1 has led to the universal administration of H1N1 influenza vaccination, including in South Korea. Several complications have been reported with use of H1N1 influenza vaccine, but systemic lupus erythematosus (SLE) has not been reported as a complication until now. Here, we report a case of SLE occurrence after H1N1 influenza vaccination. A 17-year-old girl who had not been diagnosed with SLE was hospitalized with fever, myalgia, and arthralgia after H1N1 influenza vaccination. Laboratory tests revealed increased levels of antinuclear antibody and anti-ds-DNA antibody, and decreased levels of C3 and C4 as well as proteinuria. The pathological findings confirmed a diagnosis of lupus nephritis. The patient was treated with high-dose corticosteroid and hydroxychloroquine. This is the first report of SLE following H1N1 influenza vaccination in South Korea.

Toward a universal influenza vaccine: from the perspective of protective efficacy

No abstract available.

Comparative fracture strength analysis of Lava and Digident CAD/CAM zirconia ceramic crowns

PURPOSE: All-ceramic crowns are subject to fracture during function. To minimize this common clinical complication, zirconium oxide has been used as the framework for all-ceramic crowns. The aim of this study was to compare the fracture strengths of two computer-aided design/computer-aided manufacturing (CAD/CAM) zirconia crown systems: Lava and Digident. MATERIALS AND METHODS: Twenty Lava CAD/CAM zirconia crowns and twenty Digident CAD/CAM zirconia crowns were fabricated. A metal die was also duplicated from the original prepared tooth for fracture testing. A universal testing machine was used to determine the fracture strength of the crowns. RESULTS: The mean fracture strengths were as follows: 54.9 +/- 15.6 N for the Lava CAD/CAM zirconia crowns and 87.0 +/- 16.0 N for the Digident CAD/CAM zirconia crowns. The difference between the mean fracture strengths of the Lava and Digident crowns was statistically significant (P<.001). Lava CAD/CAM zirconia crowns showed a complete fracture of both the veneering porcelain and the core whereas the Digident CAD/CAM zirconia crowns showed fracture only of the veneering porcelain. CONCLUSION: The fracture strengths of CAD/CAM zirconia crowns differ depending on the compatibility of the core material and the veneering porcelain.

Shear bond strength of resin cement to an acid etched and a laser irradiated ceramic surface

PURPOSE: To evaluate the effects of hydrofluoric acid etching and Er,Cr:YSGG laser irradiation on the shear bond strength of resin cement to lithium disilicate ceramic. MATERIALS AND METHODS: Fifty-five ceramic blocks (5 mm x 5 mm x 2 mm) were fabricated and embedded in acrylic resin. Their surfaces were finished with 1000-grit silicon carbide paper. The blocks were assigned to five groups: 1) 9.5% hydrofluoric-acid etching for 60 s; 2-4), 1.5-, 2.5-, and 6-W Er,Cr:YSGG laser applications for 60 seconds, respectively; and 5) no treatment (control). One specimen from each group was examined using scanning electron microscopy. Ceramic primer (Rely X ceramic primer) and adhesive (Adper Single Bond) were applied to the ceramic surfaces, followed by resin cement to bond the composite cylinders, and light curing. Bonded specimens were stored in distilled water at 37degrees C for 24 hours. Shear bond strengths were determined by a universal testing machine at 1 mm/min crosshead speed. Data were analyzed using Kruskal-Wallis and Mann-Whitney U-tests (alpha=0.05). RESULTS: Adhesion was significantly stronger in Group 2 (3.88 +/- 1.94 MPa) and Group 3 (3.65 +/- 1.87 MPa) than in Control group (1.95 +/- 1.06 MPa), in which bonding values were lowest (P<.01). No significant difference was observed between Group 4 (3.59 +/- 1.19 MPa) and Control group. Shear bond strength was highest in Group 1 (8.42 +/- 1.86 MPa; P<.01). CONCLUSION: Er,Cr:YSGG laser irradiation at 1.5 and 2.5 W increased shear bond strengths between ceramic and resin cement compared with untreated ceramic surfaces. Irradiation at 6 W may not be an efficient ceramic surface treatment technique.