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A case of 17 alpha-hydroxylase deficiency

  • Kim, Sung Mee;Rhee, Jeong Ho
    • Clinical and Experimental Reproductive Medicine
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    • v.42 no.2
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    • pp.72-76
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    • 2015
  • $17{\alpha}$-hydroxylase and 17,20-lyase are enzymes encoded by the CYP17A1 gene and are required for the synthesis of sex steroids and cortisol. In $17{\alpha}$-hydroxylase deficiency, there are low blood levels of estrogens, androgens, and cortisol, and resultant compensatory increases in adrenocorticotrophic hormone that stimulate the production of 11-deoxycorticosterone and corticosterone. In turn, the excessive levels of mineralocorticoids lead to volume expansion and hypertension. Females with $17{\alpha}$-hydroxylase deficiency are characterized by primary amenorrhea and delayed puberty, with accompanying hypertension. Affected males usually have female external genitalia, a blind vagina, and intra-abdominal testes. The treatment of this disorder is centered on glucocorticoid and sex steroid replacement. In patients with $17{\alpha}$-hydroxylase deficiency who are being raised as females, estrogen should be supplemented, while genetically female patients with a uterus should also receive progesterone supplementation. Here, we report a case of a 21-year-old female with $17{\alpha}$-hydroxylase deficiency who had received inadequate treatment for a prolonged period of time. We also include a brief review of the recent literature on this disorder.

Anticoagulation Therapy, Thrombolytic Therapy, and Use of Blood Products in Neonates (신생아에서의 항응고요법, 혈전용해요법 및 혈액제제 사용)

  • Choi, Chang-Won
    • Neonatal Medicine
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    • v.18 no.1
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    • pp.34-41
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    • 2011
  • Neonatal coagulation disorders and thromboembolism require timely management. Failure to treat these conditions at the appropriate time may lead to death or the development of significant long-term sequelae. However, most current guidelines for managing neonatal coagulation disorders and thromboembolism are empiric and not based on randomized clinical trials. Thus, it is not easy to choose an appropriate management strategy for these conditions in clinical settings. In this review, therapeutic guidelines currently utilized in clinics and novel therapeutic options still under investigation are presented and reviewed.

Large-scale Synthesis of Uniform-sized Nanoparticles for Multifunctional Medical Applications

  • Hyeon, Taeg-Hwan
    • Proceedings of the Korean Vacuum Society Conference
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    • 2011.02a
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    • pp.1-1
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    • 2011
  • We developed a new generalized synthetic procedure, called as "heat-up process," to produce uniform-sized nanocrystals of many transition metals and oxides without a size selection process. We were able to synthesize uniform magnetite nanocrystals as much as 1 kilogram-scale from the thermolysis of Fe-oleate complex. Clever combination of different nanoscale materials will lead to the development of multifunctional nano-biomedical platforms for simultaneous targeted delivery, fast diagnosis, and efficient therapy. In this presentation, I would like to present some of our group's recent results on the designed fabrication of multifunctional nanostructured materials based on uniform-sized magnetite nanoparticles and their medical applications. Uniform ultrasmall iron oxide nanoparticles of <3 nm were synthesized by thermal decomposition of iron-oleate complex in the presence of oleyl alcohol. These ultrasmall iron oxide nanoparticles exhibited good T1 contrast effect. In in vivo T1 weighted blood pool magnetic resonance imaging (MRI), iron oxide nanoparticles showed longer circulation time than commercial gadolinium complex, enabling high resolution imaging. We used 80 nm-sized ferrimagnetic iron oxide nanocrystals for T2 MRI contrast agent for tracking transplanted pancreatic islet cells and single-cell MR imaging. We reported on the fabrication of monodisperse magnetite nanoparticles immobilized with uniform pore-sized mesoporous silica spheres for simultaneous MRI, fluorescence imaging, and drug delivery. We synthesized hollow magnetite nanocapsules and used them for both the MRI contrast agent and magnetic guided drug delivery vehicle.

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Useful effect of a clinical shoe insole, Mubal®, as orthotics

  • Park, Chan-Lee;Go, Ji-Hyun;Han, Na-Ra;Moon, Hong-Hee;Seo, Min-Jun
    • CELLMED
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    • v.5 no.2
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    • pp.10.1-10.3
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    • 2015
  • Arthritis is a major cause of joint pain, stiffness, and subsequent disability which adversely affects quality of life. Seriously, it can lead to long term social and psychological effects including loss of independence, depression, and anxiety. Arthritis is usually treated with joint replacement surgery or medications. However, the artificial joint is temporary and pharmacological measures have side effects, such as addiction or hypersensitivity. Thus, orthotics has been developed to improve arthritis as a nonpharmacological measure. The increased regional load across compartments of articular cartilage is an important factor in the cause of the arthritis. Mubal$^{(R)}$, a clinical shoe insole, has a sliding function to help people to walk straight and realign the body balance. The slide of Mubal$^{(R)}$ reduces the knee joint loading in patients with arthritis. In addition, pumping function of Mubal$^{(R)}$ can mitigate arthritis by stretching the squashed nerves from lumbar to cervical vertebral and actively circulating blood of pelvic limb. In addition, Mubal$^{(R)}$ could help to stimulate the growth plate. Therefore, Mubal$^{(R)}$ can be used for the child with short stature as well as patients with arthritis.

The Optimal Pyloric Procedure: A Collective Review

  • Kim, Dohun
    • Journal of Chest Surgery
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    • v.53 no.4
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    • pp.233-241
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    • 2020
  • Vagal damage and subsequent pyloric denervation inevitably occur during esophagectomy, potentially leading to delayed gastric emptying (DGE). The choice of an optimal pyloric procedure to overcome DGE is important, as such procedures can lead to prolonged surgery, shortening of the conduit, disruption of the blood supply, and gastric dumping/bile reflux. This study investigated various pyloric methods and analyzed comparative studies in order to determine the optimal pyloric procedure. Surgical procedures for the pylorus include pyloromyotomy, pyloroplasty, or digital fracture. Botulinum toxin injection, endoscopic balloon dilatation, and erythromycin are non-surgical procedures. The scope, technique, and effects of these procedures are changing due to advances in minimally invasive surgery and postoperative interventions. Some comparative studies have shown that pyloric procedures are helpful for DGE, while others have argued that it is difficult to reach an objective conclusion because of the variety of definitions of DGE and evaluation methods. In conclusion, recent advances in interventional technology and minimally invasive surgery have led to questions regarding the practice of pyloric procedures. However, many clinicians still perform them and they are at least somewhat effective. To provide guidance on the optimal pyloric procedure, DGE should first be defined clearly, and a large-scale study with an objective evaluation method will then be required.

Functional Anatomy of the Temporomandibular Joint and Pathologic Changes in Temporomandibular Disease Progression: A Narrative Review

  • Yeon-Hee Lee
    • Journal of Korean Dental Science
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    • v.17 no.1
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    • pp.14-35
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    • 2024
  • The temporomandibular joint (TMJ) is one of the most unique joints in the human body that orchestrates complex movements across different orthogonal planes and multiple axes of rotation. Comprising the articular eminence of the temporal bone and the condylar process of the mandible, the TMJ integrates five major ligaments, retrodiscal tissues, nerves, and blood and lymph systems to facilitate its function. Cooperation between the contralateral TMJ and masticatory muscles is essential for coordinated serial dynamic functions. During mouth opening, the TMJ exhibits a hinge movement, followed by gliding. The health of the masticatory system, which is intricately linked to chewing, energy intake, and communication, has become increasingly crucial with advancing age, exerting an impact on oral and systemic health and overall quality of life. For individuals to lead a healthy and pain-free life, a comprehensive understanding of the basic anatomy and functional aspects of the TMJ and masticatory muscles is imperative. Temporomandibular disorders (TMDs) encompass a spectrum of diseases and disorders associated with changes in the structure, function, or physiology of the TMJ and masticatory system. Functional and pathological alterations in the TMJ and masticatory muscles can be visualized using various imaging modalities, such as cone-beam computed tomography, magnetic resonance imaging, and bone scans. An exploration of potential pathophysiological mechanisms related to the TMJ anatomy contributes to a comprehensive understanding of TMD and informs targeted treatment strategies. Hence, this narrative review presents insights into the fundamental functional anatomy of the TMJ and pathological changes that evolve with TMD progression.

Effects of Isometric Exercise based MNIT Treatment on Body Composition, Serum Lipid, Leptin and C-reactive Protein Concentration in Middle-aged Obese Women (등척성 운동 기반의 MNIT 수기법이 중년 비만여성의 신체조성, 혈중지질, 렙틴 및 CRP 농도에 미치는 영향)

  • Oh, Sean-Ok;Seo, Dae Yun;Song, Kang-Young;Park, Se-Hwan
    • The Journal of the Korea Contents Association
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    • v.17 no.11
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    • pp.200-208
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    • 2017
  • The purpose of this study was to investigate the effects of isometric exercise based MNIT treatment on body composition, blood lipids, leptin and CRP in middle-aged obese women. For this purpose, middle-aged obese women (age: 40-50) with more 30% of body fat percentage in U city were recruited. They were divided into MNIT group(MG, n=9) and control group(CG, n=9). MNIT group was performed hand therapy program with abdomen exercise equivalent to RPE (9-11) (week 1-4) and exercise equivalent to RPE (11-12) (Week 5-8) and exercise equivalent to RPE (12-13) (week 9-12) 60 minutes a day for 3 days a week in 12 weeks. The results of this study were as follows: Body weight, fat mass, and body mass index had a significant lower in MG group than CG. There are no significant changes in blood profiles, and leptin. In conclusion, isometric based on MNIT may lead to development of an alternative therapy for middle-aged obese women.

Evaluation of Biocompatibility of Extracorporeal Circuit - Development of a Quantification Technique using in-vivo Injection of Tc99m Radioactive Platelets - (체외순환도관의 혈액적합성 평가 - 방사선 동위원소(Tc99m) 활성화 혈소판의 생체 내 주입을 이용한 정량분석법의 개발 -)

  • Lee, Sung-Ho;Sun, Kyung;Choi, Jai-Geol;Son, Ho-Sung;Jung, Jae-Seung;Ahn, Sang-Soo;Oh, Hye-Jung;Lee, Whan-Sung;Lee, Hye-Won;Kim, Kwang-Taik;Jeong, Yoon-Seop;Kim, Young-Ha;Kim, Hyoung-Mook
    • Journal of Chest Surgery
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    • v.35 no.3
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    • pp.171-176
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    • 2002
  • Background: Blood-foreign interaction cause activation of coagulation and inflammatory process that may lead to multiorgan dysfunction and determine the surgical outcomes. Of the methods for assessing the biocompatibility, the platelet adhesion study is considered as the most valuable evaluation step in blood-foreign interaction. As the most studies have used in-vitro or ex-vivo conditions, we have developed a technique of quantification for platelet adhesion on the blood contact surface by using in-vivo injection of radioactive platelets. Material and Method: A coupled bypass circuit was designed to connect the proximal and descending thoracic aorta in 6 piglets(20∼25 Kg). One side of the circuit tube was consisted of a heparin coated PVC tube(10mm in ID, n=6, Experimental group), and the other, a non-heparin coated PVC tube(10mm in ID, n=6, Control group). After cannulation, the blood was circulated through the circuit for 2 hours. Platelet concentrate was prepared from homologous pig blood 24 hours before the experiment. The platelet concentrate was incubated with Tc-99m-HMPAO for 30 min and then centrifuged for 10 min. The supernatant was discarded and the radio-labeling efficacy was measured. The radio-labeled platelet concentrate was mixed with the autologous plasma to make the volume 5 ml, and the mixture was injected intravenously into the experimental animal. After 2 hour circulation, 5 pieces of the specimen(10mm in length each) were obtained from each PVC tube. The radioisotopes were counted with a gamma counter(Cobra ll, Packard, USA), and the ratio of radioisotope count was compared between the control and experimental group. Result: The radioisotope count number was 537.3221.1 Ci/min in the control group and 311.1 184.5 Ci/min in the experimental group(p=0.0104). The ratio between the groups was 1 to 0.58 (p=0.004). Conclusion: In vivo quantification using technetium-99m-HMPAO labeled platelets is simple and reproducible in evaluating platelet adhesion on a foreign surface. We suggest this technique to be a useful tool for blood compatibility test.

Changes of Blood $Mg^{2+}$ and $K^+$ after Starvation during Molting in Laying Hens (환우(換羽, molting)에 의한 절식 후 산란계의 혈액 $Mg^{2+}$$K^+$ 변동)

  • Go, Hyeon-Kyu;Lee, Sei-Jin;Cho, In-Gook;Lee, Mun-Young;Park, Hye-Min;Mun, A-Reum;Kim, Jeong-Gon;Kim, Gi-Beum;Kim, Jin-Shang;Kang, Hyung-Sub;Kim, Shang-Jin
    • Journal of Veterinary Clinics
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    • v.28 no.6
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    • pp.581-585
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    • 2011
  • Either the fasting during natural molting or the starvation in induced molting would be a severe metabolic stress to laying hens. The metabolic stress during starvation and subsequent refeeding syndrome could lead to unbalance of mineral homeostasis, including $Mg^{2+}$, $K^+$ and P required by ATP synthesis. Since $Mg^{2+}$ is a fundamental ion for normal metabolic processes and stress may not only increase in demands of $Mg^{2+}$ but also produce consequence of $Mg^{2+}$ deficiency, we investigated the changes of blood ionized and total ions related to starvation during molting in laying hens. We founded the significant decrease in blood $Mg^{2+}$ and $K^+$ accompanied by the changes of biochemical parameters relating to increased metabolic stress after molting. These results suggested that appropriate $Mg^{2+}$ and $K^+$ supplements to laying hens could have beneficial effects during molting and subsequent refeeding that could produce a severe hypomagnesemia and hypokalcemia.

Rectal Injury Associated with Pelvic Fracture

  • Gwak, Jihun;Lee, Min A;Yu, Byungchul;Choi, Kang Kook
    • Journal of Trauma and Injury
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    • v.29 no.4
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    • pp.201-203
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    • 2016
  • Rectal injury is seen in 1-2% of all pelvic fractures, and lower urinary tract injury occurs in up to 7%. These injuries are rare, but if missed, can lead to a severe septic response. Rectal injury may be suspected by the presence of gross blood on digital rectal examination. However, this classic sign is not always present on physical examination. If an Antero-Posterior Compression type pelvic fracture is seen, we should consider the possibility of rectal and lower urinary tract injury. It is important to define the anatomic location of the rectal injury as it relates to the peritoneal reflection. Trauma to the intraperitoneal rectum should be managed as a colonic injury. Extraperitoneal rectal injury should be managed with fecal diversion regardless of primary repair. We present the case of a 46-year-old man who was referred to our hospital following a major trauma to the pelvis in a pedestrian accident.