2022 Clinical practice guidelines for central precocious puberty of Korean children and adolescents

Kim Su Jin,Kim Ji Hyun,Hong Yong Hee,Chung In Hyuk,Lee Eun Byoul,Kang Eungu,Kim Jinsup,Yang Aram,Rhie Young-Jun,Yoo Eun-Gyong,Shin Young-Lim,Choi Jin Ho,Kim Soo Young,Lee Ji Eun 대한소아내분비학회 2023 Annals of Pediatirc Endocrinology & Metabolism Vol.28 No.3

The Committee of Central Precocious Puberty of Korean Pediatrics and Adolescents of the Korean Society of Pediatric Endocrinology has newly developed evidence-based 2022 clinical practice guidelines for central precocious puberty in Korean children and adolescents. These guidelines provide the grade of recommendations, which includes both the strength of recommendations and the level of evidence. In the absence of sufficient evidence, recommendations are based on expert opinion. These guidelines have been revised and supplement the previous guidelines "Clinical Guidelines for Precocious Puberty 2011," and are drawn from a comprehensive review of the latest domestic and international research and the grade of recommendation appropriate to the domestic situation. This review summarizes the newly revised guidelines into 8 key questions and 27 recommendations and consists of 4 sections: screening, diagnosis, treatment, and long-term outcome of central precocious puberty.

Uniform Graphene Quantum Dots Patterned from Self-Assembled Silica Nanodots

Lee, Jinsup,Kim, Kyungho,Park, Woon Ik,Kim, Bo-Hyun,Park, Jong Hyun,Kim, Tae-Heon,Bong, Sungyool,Kim, Chul-Hong,Chae, GeeSung,Jun, Myungchul,Hwang, Yongkee,Jung, Yeon Sik,Jeon, Seokwoo American Chemical Society 2012 NANO LETTERS Vol.12 No.12

<P>Graphene dots precisely controlled in size are interesting in nanoelectronics due to their quantum optical and electrical properties. However, most graphene quantum dot (GQD) research so far has been performed based on flake-type graphene reduced from graphene oxides. Consequently, it is extremely difficult to isolate the size effect of GQDs from the measured optical properties. Here, we report the size-controlled fabrication of uniform GQDs using self-assembled block copolymer (BCP) as an etch mask on graphene films grown by chemical vapor deposition (CVD). Electron microscope images show that as-prepared GQDs are composed of mono- or bilayer graphene with diameters of 10 and 20 nm, corresponding to the size of BCP nanospheres. In the measured photoluminescence (PL) spectra, the emission peak of the GQDs on the SiO<SUB>2</SUB> substrate is shown to be at ∼395 nm. The fabrication of GQDs was supported by the analysis of the Raman spectra and the observation of PL spectra after each fabrication step. Additionally, oxygen content in the GQDs is rationally controlled by additional air plasma treatment, which reveals the effect of oxygen content to the PL property.</P><P><B>Graphic Abstract</B> <IMG SRC='http://pubs.acs.org/appl/literatum/publisher/achs/journals/content/nalefd/2012/nalefd.2012.12.issue-12/nl302520m/production/images/medium/nl-2012-02520m_0007.gif'></P><P><A href='http://pubs.acs.org/doi/suppl/10.1021/nl302520m'>ACS Electronic Supporting Info</A></P>

A Novel Mutation of KRT14 Gene in a Newborn with Epidermolysis Bullosa Simplex (Dowling-Meara Type): Case Report

( Sung-min Lim ),( Jin Hee Kim ),( Yeseul Kim ),( Seung Sam Paik ),( Jeong Eun Kim ),( Joo Yeon Ko ),( Jinsup Kim ),( Hyun-kyung Park ),( Chang-ryul Kim ),( Hyun Ju Lee ) 대한주산의학회 2020 Perinatology Vol.31 No.1

Epidermolysis bullosa simplex (EBS) is a group of inherited skin diseases characterized by intraepidermal blistering upon mild trauma. They are classified into three major types based on the severity and distribution of blisters, age of onset, and histological findings. These three major types are caused by KRT5 and KRT14 gene mutations. EBS Dowling-Meara (DM) is one of the most severe subtypes which mostly affects neonates. Electron microscopy is a primary diagnostic tool for EBS; however, mutation analysis has recently become more important for its diagnosis, prognosis, genetic counselling, and prenatal diagnosis. Several studies have reported that almost all mutations in EBS-DM patients are found in the highly conserved rod domains of the KRT5 and KRT14 genes and have also demonstrated a genotype-phenotype correlation. Here, we report an EBS-DM case diagnosed by mutation analysis in a newborn and a missense mutation not identified in humans previously.

Beneficial Effects of Growth Hormone Treatment in Prader-Willi Syndrome

Kim, Jinsup,Yang, Aram,Cho, Sung Yoon,Jin, Dong-Kyu Association for Research of MPS and Rare Diseases 2017 Journal of mucopolysaccharidosis and rare disease Vol.3 No.2

Prader-Willi syndrome (PWS) is a genetic disorder that is considered, especially on child, to cause poor feeding, hypotonia, failure to thrive, developmental delay and hypogonadism which is known to affect between 1 in 10,000 and 30,000 people. The children with PWS are viewed as affected by growth hormone (GH) insufficiency, although the exact mechanisms of GH deficiency are not fully understood. However, the benefits of GH treatment in children with PWS are well established. Myers, et al. (2006), Grugni, et al. (2016) indicated its positive effects on linear growth, body composition, motor function, respiratory function and psychomotor development. Despite of its effectiveness and advantages had been well known and proven in many other studies, there is only one recombinant GH product that is approved for PWS in Korea, $Genotropin^{(R)}$, till now. A phase III clinical study of GH treatment with $Eutropin^{TM}$, in 34 Korean PWS children is in progress, which is expected to have comparable effects and safety profile with the active control by assessing auxological changes such as height standard deviation score, body composition changes such as lean body mass and percent body fat, motor and cognitive development using Bayley scale, and safety profiles.

Fighting Corruption of Korea Customs Office

Jinsup Kim(김진섭) 한국관세학회 2004 관세학회지 Vol.5 No.3

Corruption thrives when public accountability is weak, where governments or political parties are inclined to pursue their own private agendas often disguising the true cost to their country, where there are big differences between public and private sector pay, and when there is impunity for the corrupt. The issue of fighting corruption should be addressed not by stop-gap measures and temporary remedies, but by systematic and comprehensive integrity enhancement measures. The Korea Customs Service(KCS) has diminished the opportunity of corruption through minimization of direct person-to-person contact between Customs officers and their clients by establishment of automated paperless Customs procedures.

Introduction to the New Version of PWS Application and It's Use in Medical Practice

Kim, Jinsup,Yang, Aram,Cho, Sung Yoon,Jin, Dong-Kyu Association for Research of MPS and Rare Diseases 2016 Journal of mucopolysaccharidosis and rare disease Vol.2 No.2

Today's mobile phones and tablet PCs offer a considerably wider range of functionalities than before. Mobile applications (apps) are increasingly used for managing various daily health tasks. Currently, more than 165,000 health-related apps are offered on all the stores of different platforms. Pf Jin and the Association for Research on MPS and Rare Diseases (AMARD) have helped Prader-Willi syndrome (PWS) families through medical information and family support since 2015. AMARD developed the first mobile application for Korean patients with PWS, which was released to a limited number of patients under the age of 3 and only provided to Android users. The first version of the PWS application focused on growth hormone therapy and the assessment of growth and development by parents in infant and early-childhood PWS patients. The 2016 version of the PWS application has been improved in many different ways. We have expanded the subjects of the application to late childhood and adolescent groups, changed the user interface accordingly, and made the application available for iOS users. We will show the specialized growth curves of older children with PWS. Therefore, patients with PWS over the age of 3 and their parents can assess the patients' growth. Additionally, we have upgraded the growth hormone therapy menu by improving the input system for the growth hormone therapy injection schedule and the daily growth profile (height and weight). We expect that the new version of the PWS application will help many PWS families cope with growth hormone therapy and evaluate the effects of growth hormones in better ways. Additionally, we are making a constant effort to provide more useful information about patients with PWS in many aspects.

Clinical and molecular characterization of Korean children with infantile and late-onset Pompe disease: 10 years of experience with enzyme replacement therapy at a single center

Kim, Min-Sun,Song, Ari,Im, Minji,Huh, June,Kang, I-Seok,Song, Jinyoung,Yang, Aram,Kim, Jinsup,Kwon, Eun-Kyung,Choi, Eu-Jin,Han, Sun-Ju,Park, Hyung-Doo,Cho, Sung Yoon,Jin, Dong-Kyu The Korean Pediatric Society 2019 Clinical and Experimental Pediatrics (CEP) Vol.62 No.6

Purpose: Pompe disease (PD) is an autosomal recessive disorder caused by a deficiency of acid alpha-glucosidase resulting from pathogenic GAA variants. This study describes the clinical features, genotypes, changes before and after enzyme replacement therapy (ERT), and long-term outcomes in patients with infantile-onset PD (IOPD) and late-onset PD (LOPD) at a tertiary medical center. Methods: The medical records of 5 Korean patients (2 male, 3 female patients) diagnosed with PD between 2002 and 2013 at Samsung Medical Center in Seoul, Republic of Korea were retrospectively reviewed for data, including clinical and genetic characteristics at diagnosis and clinical course after ERT. Results: Common initial symptoms included hypotonia, cyanosis, and tachycardia in patients with IOPD and limb girdle weakness in patients with LOPD. Electrocardiography at diagnosis revealed hypertrophic cardiomyopathy in all patients with IOPD who showed a stable disease course during a median follow-up period of 10 years. Patients with LOPD showed improved hepatomegaly and liver transaminase level after ERT. Conclusion: As ERT is effective for treatment of PD, early identification of this disease is very important. Thus, patients with IOPD should be considered candidates for clinical trials of new drugs in the future.

A STUDY ON INDUSTRIAL GAMMA RAY CT WITH ASINGLE SOURCE-DETECTOR PAIR

JINSUP KIM,정성희,김종범 한국원자력학회 2006 Nuclear Engineering and Technology Vol.38 No.4

Having its roots in medical applications, industrial gamma ray CT has opened up new roads for investigating and modelingindustrial processes. Using a line of research related to industrial gamma ray CT, the authors set up a system of single sourceand detector gamma transmission tomography for wood timber and a packed bed phantom. The hardware of the CT systemconsists of two servo motors, a data logger, a computer, a radiation source and a radiation detector. One motor simultaneouslymoves the source and the detector for a parallel beam scanning, whereas the other motor rotates the scan table at a presetprojection angle. The image is reconstructed from the measured projections by the filtered back projection method. The phantomwas designed to simulate a cross section of a packed bed with a void. The radiation source was 20mCi of Cs-137 and thedetector was a 1 inch 1 inch NaI (TI) scintillator shielded by a lead collimator. The experimental gamma ray CT imagehas sufficient resolution to reveal air holes and the density distribution inside the phantom. The system could possibly beapplied to a packed bed column or a pipe flow in a petrochemical plant.

A Study on Duopoly Game of International Trade Negotiation

Kim, Jinsup 한국협상학회 2000 협상연구 Vol.6 No.1

This paper examines the subgame perfect Nash equilibrium in a trade negotiation under the possibility of the Rotten Kid paradox. It is shown that the Nash equilibrium is the most efficient strategy to both payers compared with the others by analyzing welfare effects. under the assumption of Cournot duopoly. Also it can be said that if the Rotten Kid paradox holds, the agreement might not take effects since the offered country may violate the agreement at any time without sanction power.

Quality of Life in Prader-Willi Syndrome Patients

Kim, Jinsup,Cho, Sung Yoon,Jin, Dong-Kyu Association for Research of MPS and Rare Diseases 2015 Journal of mucopolysaccharidosis and rare disease Vol.1 No.2