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이형숙,김현주,조재현,이승원,김현아,최준혁,송영준,김대중,이관우,정윤석 대한내분비학회 2003 Endocrinology and metabolism Vol.18 No.5
연구배경 및 방법: 골형성부전증은 비교적 희귀한 유전병으로 교원질 대사 장애로 인한 골의 취약성과 다발성 골절 및 척추측만증 등을 특징으로 한다. 유전방식과 표현형의 발현 정도에 따라 다양한 임상 양상을 보이며, 임상적 중증도에 따라 4가지 형태로 분류된다. 지금까지 국내 보고는 분만과정이나 태아 진찰시 골격이상으로 발견된 증례보고가 주였다. 저자들은 비교적 경미한 임상 양상을 보이는 예를 포함한 다수의 골형성부전증 환자들의 전반적인 임상적 특징에 대해 보고하는 바이다. 결과: 2001년 6월부터 2003년 2월까지 골형성부전증으로 진단받은 6 가계, 10예를 대상으로 하였다. 평균 나이는 27.3(5∼56)세였고 소아가 2예였다. 모두 상염색체 우성으로 유전되었으며, 제 I형이 4예, 제 III형이 4예, 그리고 제 IV형이 2예였다. 전 예에서 다수의 골절 경험이 있었고, 골밀도 저하와 골피질 두께 감소 소견이 관찰되었다. 전신의 평균 골밀도는 0.690(0.421∼1.039) g/cm²였다. 골형성지표로 측정된 sAlk는 소아의 경우만 증가되어 있었고, 골흡수지표로 측정된 uDPD의 평균치는 12.9(4.4∼36.3) nM/mM Cr으로 증가된 소견을 보였다. mobility score는 대부분 3,4단계에 속해 있었다. 중증형일수록 진단 시의 mobility score가 낮은 경향을 보였다. 결론: 한국인 골형성부전증 환자의 임상적 특징을 살펴본 결과 기존의 보고된 II형 외에도 I, III, IV형이 다양하게 존재함을 알 수 있었으며, 모든 예에서 상염색체 우성으로 유전됨을 확인할 수 있었다. 또한 모든 예에서 증가된 골흡수로 인한 골밀도 저하와 골절을 확인할 수 있었다. Osteogenesis Imperfecta (OI) is a relatively rare hereditary disease, which is characterized by multiple bone fractures and spine scoliosis, due to the fragility of bone, and is often associated with blue sclerae, deafness and dentinogenesis imperfecta. Four types of OI can be distinguished, according to the clinical findings. Although mutations affecting type I collagen are responsible for the disease in most patients, the mechanism by which the genetic defects cause abnormal bone development remains to be fully understood. Here, the clinical characteristics of 10 OI patient cases are reported, with a review of the literature. All the cases, including 4 type I, 4 type III and 2 type IV, inherited OI as an autosomal dominant trait. All the subjects had multiple old fractures and decreased bone densities. In this study, the biochemical marker of bone formation, serum alkaline phosphatase, was found to be increased only in the pediatric OI patients, while the biochemical marker of bone resorption, urinary deoxypyridinoline, was increased in all cases. The mobility score was found to correlate with the severity of the type on diagnosis (J Kor Soc Endocrinol 18:496∼503, 2003).
김진호,문준성,문선중,이지은,최재원,은미정,천경아,조인호,윤지성,원규장,이경희 신덕섭,이형우 영남대학교 의과대학 2005 Yeungnam University Journal of Medicine Vol.22 No.2
Central diabetes insipidus (DI) is a syndrome characterized by thirst, polydipsia and polyuria. Langerhans cell histiocytosis is one of the etiologies of DI. Recently we experienced a central DI associated with Langerhans cell histiocytosis. The 44 years old female patient complained right hip pain polydipsia and polyuria. We carried out water deprivation test. After vasopressin injection, urine osmotic pressure was increased form 109mOsmol/Kg to 327mOsmol/Kg (300%). Brain MRI showed a thickened pituitary stalk and at hot bone CT.CT guided biopsy revealed abnormal histiocytes proliferation and abundant lymphocytes, The final diagnosis was central DI associated with systemic Langerhans cell histiocytosis invading hip bone, L-spine and pituitary stalk. Desmopressin and etoposide chemotherapy were performed to the patient.
Cho, Jae-Hyoung,Kim, Hun-Sung,Han, Jae-Hoon,Lee, Jin-Hee,Oh, Jeong-Ah,Choi, Yoon-Hee,Yoon, Kun-Ho Korean Diabetes Association 2010 Korean diabetes journal Vol.34 No.5
<P>New diabetes management systems based on interactive communication have been introduced recently, accompanying rapid advances in information technology; these systems are referred to as 'ubiquitous diabetes management systems.' In such ubiquitous systems, patients and medical teams can communicate via Internet or telecommunications, with patients uploading their glucose data and personal information, and medical teams sending optimal feedback. Clinical evidence from both long-term and short-term trials has been reported by some researchers. Such systems appear to be effective not only in reducing the levels of HbA1c but also in stabilizing glucose control. However, most notably, evidence for the cost-effectiveness of such a system should be demonstrated before it can be propagated out to the general population in actual clinical practice. To establish a cost-effective model, various types of clinical decision supporting software designed to reduce the labor time of physicians must first be developed. A number of sensors and devices for monitoring patients' data are expected to be available in the near future; thus, methods for automatic interconnections between devices and web charts were also developed. Further investigations to demonstrate the clinical outcomes of such a system should be conducted, hopefully leading to a new paradigm of diabetes management.</P>
Yoon Hyoung Pai,Chae Seong Lim,Kyung-Ah Park,Hyun Sil Cho,Gyu-Seung Lee,Yong Sup Shin,Hyun-Woo Kim,Byeong Hwa Jeon,Seok Hwa Yoon,Jin Bong Park 대한생리학회-대한약리학회 2016 The Korean Journal of Physiology & Pharmacology Vol.20 No.4
In addition to classical synaptic transmission, information is transmitted between cells via the activation of extrasynaptic receptors that generate persistent tonic current in the brain. While growing evidence supports the presence of tonic NMDA current (I<sub>NMDA</sub>) generated by extrasynaptic NMDA receptors (eNMDARs), the functional significance of tonic I<sub>NMDA</sub> in various brain regions remains poorly understood. Here, we demonstrate that activation of eNMDARs that generate INMDA facilitates the α-amino-3-hydroxy-5-methylisoxazole-4-proprionate receptor (AMPAR)-mediated steady-state current in supraoptic nucleus (SON) magnocellular neurosecretory cells (MNCs). In low-Mg<sup>2+</sup> artificial cerebrospinal fluid (aCSF), glutamate induced an inward shift in I<sub>holding</sub> (I<sub>GLU</sub>) at a holding potential (V<sub>holding</sub>) of –70 mV which was partly blocked by an AMPAR antagonist, NBQX. NBQX-sensitive IGLU was observed even in normal aCSF at V<sub>holding</sub> of –40 mV or –20 mV. IGLU was completely abolished by pretreatment with an NMDAR blocker, AP5, under all tested conditions. AMPA induced a reproducible inward shift in Iholding (I<sub>AMPA</sub>) in SON MNCs. Pretreatment with AP5 attenuated I<sub>AMPA</sub> amplitudes to ~60% of the control levels in low-Mg <sup>2+</sup> aCSF, but not in normal aCSF at V<sub>holding</sub> of –70 mV. I<sub>AMPA</sub> attenuation by AP5 was also prominent in normal aCSF at depolarized holding potentials. Memantine, an eNMDAR blocker, mimicked the AP5-induced I<sub>AMPA</sub> attenuation in SON MNCs. Finally, chronic dehydration did not affect I<sub>AMPA</sub> attenuation by AP5 in the neurons. These results suggest that tonic INMDA, mediated by eNMDAR, facilitates AMPAR function, changing the postsynaptic response to its agonists in normal and osmotically challenged SON MNCs.
Cho, Jung Jae,Chae, Jung-Il,Yoon, Goo,Kim, Ka Hwi,Cho, Jin Hyoung,Cho, Seung-Sik,Cho, Young Sik,Shim, Jung-Hyun Lychnia 2014 International journal of oncology Vol.45 No.2
<P>Licochalcone A (LCA), a chalconoid derived from root of Glycyrrhiza inflata, has been known to possess a wide range of biological functions such as antitumor, anti-angiogenesis, antiparasitic, anti-oxidant, antibacterial and anti-inflammatory effects. However, the anticancer effects of LCA on oral squamous cell carcinoma (OSCC) have not been reported. Our data showed that LCA inhibited OSCC cell (HN22 and HSC4) growth in a concentration- and time-dependent manner. Mechanistically, it was mediated via downregulation of specificity protein 1 (Sp1) expression and subsequent regulation of Sp1 downstream proteins such as p27, p21, cyclin D1, Mcl-1 and survivin. Here, we found that LCA caused apoptotic cell death in HSC4 and HN22 cells, as characterized by sub-G(1) population, nuclear condensation, Annexin V staining, and multi-caspase activity and apoptotic regulatory proteins such as Bax, Bid, Bc1-(xl), caspase-3 and PARR Consequently, this study strongly suggests that LCA induces apoptotic cell death of OSCC cells via downregulation of Sp1 expression, prompting its potential use for the treatment of human OS CC.</P>
Pai, Yoon Hyoung,Lim, Chae Seong,Park, Kyung-Ah,Cho, Hyun Sil,Lee, Gyu-Seung,Shin, Yong Sup,Kim, Hyun-Woo,Jeon, Byeong Hwa,Yoon, Seok Hwa,Park, Jin Bong The Korean Society of Pharmacology 2016 The Korean Journal of Physiology & Pharmacology Vol.20 No.4
In addition to classical synaptic transmission, information is transmitted between cells via the activation of extrasynaptic receptors that generate persistent tonic current in the brain. While growing evidence supports the presence of tonic NMDA current ($I_{NMDA}$) generated by extrasynaptic NMDA receptors (eNMDARs), the functional significance of tonic $I_{NMDA}$ in various brain regions remains poorly understood. Here, we demonstrate that activation of eNMDARs that generate INMDA facilitates the ${\alpha}$-amino-3-hydroxy-5-methylisoxazole-4-proprionate receptor (AMPAR)-mediated steady-state current in supraoptic nucleus (SON) magnocellular neurosecretory cells (MNCs). In $low-Mg^{2+}$ artificial cerebrospinal fluid (aCSF), glutamate induced an inward shift in $I_{holding}$ ($I_{GLU}$) at a holding potential ($V_{holding}$) of -70 mV which was partly blocked by an AMPAR antagonist, NBQX. NBQX-sensitive $I_{GLU}$ was observed even in normal aCSF at $V_{holding}$ of -40 mV or -20 mV. $I_{GLU}$ was completely abolished by pretreatment with an NMDAR blocker, AP5, under all tested conditions. AMPA induced a reproducible inward shift in $I_{holding}$ ($I_{AMPA}$) in SON MNCs. Pretreatment with AP5 attenuated $I_{AMPA}$ amplitudes to ~60% of the control levels in $low-Mg^{2+}$ aCSF, but not in normal aCSF at $V_{holding}$ of -70 mV. $I_{AMPA}$ attenuation by AP5 was also prominent in normal aCSF at depolarized holding potentials. Memantine, an eNMDAR blocker, mimicked the AP5-induced $I_{AMPA}$ attenuation in SON MNCs. Finally, chronic dehydration did not affect $I_{AMPA}$ attenuation by AP5 in the neurons. These results suggest that tonic $I_{NMDA}$, mediated by eNMDAR, facilitates AMPAR function, changing the postsynaptic response to its agonists in normal and osmotically challenged SON MNCs.
( Yun Kyeong Cho ),( Seung Ho Hur ),( Nam Hee Park ),( Sang Woong Choi ),( Ji Hyun Sohn ),( Hyun Ok Cho ),( Hyoung Seob Park ),( Hyuck Jun Yoon ),( Hyung Seop Kim ),( Chang Wook Nam ),( Yoon Nyun Kim 대한내과학회 2014 The Korean Journal of Internal Medicine Vol.29 No.1
Background/Aims: While drug-eluting stents (DESs) have shown favorable out-comes in ST-segment elevation myocardial infarction (STEMI) compared to bare metal stents (BMSs), there are concerns about the risk of stent thrombosis (ST) with DESs. Because intravascular ultrasound (IVUS) guidance may help optimize stent placement and improve outcomes in percutaneous coronary intervention (PCI) patients, we evaluated the impact of IVUS-guided BMS versus DES implan-tation on long-term outcomes in primary PCI. Methods: In all, 239 STEMI patients received DES (n = 172) or BMS (n = 67) under IVUS guidance in primary PCI. The 3-year incidence of major adverse cardiac events (MACEs) including death, myocardial infarction (MI), target vessel revas-cularization (TVR), and ST was evaluated. Results: There was no difference in all cause mortality or MI. However, the in-cidence of TVR was 23.9% with BMS versus 9.3% with DES (p = 0.005). Thus, the number of MACEs was significantly lower with DES (11.0% vs. 29.9%; p = 0.001). The incidence of definite or probable ST was not different (1.5% vs. 2.3%; p = 1.0). IVUS-guided DES implantation (hazard ratio [HR], 0.25; 95% confidence interval [CI], 0.08 to 0.78; p = 0.017), stent length (HR, 1.03; 95% CI, 1.00 to 1.06; p = 0.046), and multivessel disease (HR, 3.01; 95% CI, 1.11 to 8.15; p = 0.030) were independent predictors of MACE. Conclusions: In patients treated with primary PCI under IVUS guidance, the use of DES reduced the incidence of 3-year TVR versus BMS. However, all cause mor-tality and MI were similar between the groups. The incidence of ST was low in both groups.