• Clinical and Experimental Pediatrics
• /
• 제60권2호
• /
• pp.31-37
• /
• 2017

Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers. Psychosocial issues frequently affect adherence to glucocorticoid treatment. Therefore, the safe transition of adolescents to adult care requires regular follow-up of patients by a multidisciplinary team including pediatric and adult endocrinologists. The major goals for management of adults with 21-hydroxylase deficiency are to minimize the long-term complications of glucocorticoid therapy, reduce hyperandrogenism, prevent adrenal or testicular adrenal rest tumors, maintain fertility, and improve quality of life. Optimized medical or surgical treatment strategies should be developed through coordinated care, both during transition periods and throughout patients' lifetimes. This review will summarize current knowledge on the management of adults with CAH, and suggested appropriate approaches to the transition from pediatric to adult care.

• 의료법학
• /
• 제10권2호
• /
• pp.203-249
• /
• 2009

It is the so-called Shinchon Severance Hospital Case brought to an end by the decision of the Supreme Court that opened the real discourse of withholding or withdrawing of LST (Life-Sustaining Treatment) in the legal profession as well as medical profession in Korea. Everyone has sympathy with the validity and necessity of legal regulation on withdrawing-including withholding-of LST save the requirements & procedure of withdrawing of LST. In this situation, the legislative bill of amendment to the Korean Civil Law introducing of adult guardianship was pre-announced by the Ministry of Justice on September 18th 2009. The adult guardianship is a guardianship system that supports an mentally handicapped adult to deal with his affairs by support of a guardian. The object of adult guardianship includes affairs of body or well-being as well as property of adult wards. In particular, affairs of medical matters are of importance in the duty and authority of adult guardians. So, the introduction of adult guardianship is of much importance de lege lata as well as de lege ferena in the discussion of withdrawing of LST as a medical treatment. Since the legislation on withdrawing of LST intents to protect the right of death with dignity on the basis of patients' autonomy, the ratio legis of withdrawing of LST is variant from that of adult guardianship. In this context, it seems reasonable to legislate the withdrawing of LST separately from the adultguardianship. In the meantime, the adult guardianship of the legislative bill of amendment to the Korean Civil Law is related to the withdrawing of LST, since the main purpose of adult guardianship is to protect patients' quality of lives and to regulate guardianship contracts based on patients' autonomy. In that context, it seems reasonable to incorporate the legislation of withdrawing of LST into the adult guardianship system. In the latter case, it is not easy to adopt the withdrawing of LST into the legislative bill of the Korean Civil Law for the bill is pre-announced already as previously stated. However, the legislation of withdrawing of LST is not inferior to the legislation of adult guardianship as a matter of urgency. Moreover, it is likely that the legislative bill of Amendment to the Korean Civil Law generates discrepancies in interpretation of the requirements & procedure of withdrawing of LST as the amended German Civil Law did. In short, it is desirable for the legislator to revise the legislative bill despite delay.

• Korean Circulation Journal
• /
• 제52권3호
• /
• pp.233-242
• /
• 2022

Background and Objectives: For adult men with congenital heart disease (ACHD), data on erectile dysfunction (ED) is limited. We aimed to assess the frequency of ED, its role in patient-physician communication and to identify parameters predicting ED. Methods: Male ACHD ≥18 years registered at the German National Register for Congenital Heart Defects were invited to participate in an online questionnaire about sexual health. Participants with presumed ED according to International Index of Erectile Function Score were compared to patients without ED. Results: The 371 patients responded to the questionnaire (83% with moderate to highly complex ACHD). The 43% presented with more than mild ED. When ED was present, patients complained about general anxiety to be sexually active more often (p<0.05) and underwent sexual activity less frequently compared to those without ED (p<0.05). Age ≥40 years (odds ratio [OR], 3.04; p=0.002), being single (OR, 6.82; p<0.0001), anxiety to be sexually active (OR, 2.64; p=0.0002) and psychiatric disease (OR, 4.33; p<0.0007) emerged as independent predictors for ED. Overall, patients sought medical advice in 6.7% of cases, whilst 29.6% would appreciate an active approach by the physician to address this sensitive topic. Conclusions: ED is affecting one third to one half of male ACHD according to a questionnaire-based analysis. Older age, being single, fear of sexual activity due to ACHD and psychiatric disorder emerged as independent predictors for ED. These parameters can easily be assessed to identify patients at risk. ED should be addressed proactively by health professionals.

• Journal of Trauma and Injury
• /
• 제22권1호
• /
• pp.103-107
• /
• 2009

Purpose: There are no reports on amusement-park-related injuries in Korea. Thus, the objective of this study was to describe traumatic injury patterns that occurring in an amusement park. Methods: The medical records of an infirmary were retrospectively reviewed. From January 1, 2008, to December 31 2008, patients who were transferred to the nearest emergency departments of hospitals for the purpose of further test and treatment were enrolled. Demographics, injury types and involved parts of the body were analyzed. Results: A total of 3,608 patients visited an infirmary for traumatic injury and about two-thirds had soft issue injuries. Of those, 191 patients (5.3%) were transferred to the emergency department of a hospital. Of the patients who were transferred to a hospital, laceration and contusion were the responsible injuries for about half. Laceration was the most common injury in pediatric patients, and a sprain or a strain was the most common in adult patients. The most commonly injured parts of the body were the extremities in adult patients. However, in pediatric patients, injuries of the head, face and neck were similar to injuries of the extremities. Conclusion: Soft tissue injury was the most common amusement-park-related injury. Laceration was the most common reason to transfer a patient to a hospital. There were differences in injury type and injured part of the body between adult and pediatric patients.

• 성인간호학회지
• /
• 제25권3호
• /
• pp.250-262
• /
• 2013

Purpose: This study was designed to develop, to implement and evaluate a fever management nursing protocol for adult patients. Methods: This study was conducted through seven steps following both the guideline development procedures of the Scottish Intercollegiate Guidelines Network and the Model of the Asan Medical Center Evidence-Based Practice, as follows: 1) identifying the clinical needs and composition of the protocol development group; 2) identifying and evaluating the evidence; 3) assessing the level of evidence and grading recommendations; 4) forming a protocol; 5) evaluating the protocol using an expert group validity test and identifying barriers to its implementation; 6) protocol development; and 7) evaluation of practical improvement measures following implementation of the protocol. Results: The evidence-based protocol for fever management in adult patients was completed and includes five domains and 15 items. The protocol had good content validity (CVI=.90) and nursing practice could be improved after implementation of the protocol. Conclusion: This nursing protocol can be used as a guide for nursing in febrile adult patients. We recommend that further guidelines be updated in an interdisciplinary manner in order to foster local adaptation of the best clinical practices.

• Journal of Trauma and Injury
• /
• 제27권4호
• /
• pp.101-107
• /
• 2014

Purpose: Recently, the population of elderly people has been increasing rapidly all over the world. The social activities of the aging population have increased, which has also increased the number of elderly patients injured in traffic accidents. Thus, we analyzed the characteristics of elderly patients involved in traffic accidents. Methods: This study was conducted retrospectively from July 2008 to March 2009 among trauma patients involved in traffic accidents who visited Wonju Severance Christian Hospital. Patients under 18 years of age and pregnant patients were excluded. We divided the patients in two groups, a geriatrics group and an adult group on the basis of an age of 65. We compared the types of traffic accidents, the locations of the accidents, the behaviors of the patients at the times of the accidents, the use of seat-belts, and alcohol consumption between the two groups. We calculated the Revised Trauma Score (RTS), Injury Severity Score (ISS), and Trauma and Injury Severity Score (TRISS) for each group. Results: Total number of the included patients was 903, and the number of elderly patients was 181 (mean age: $71.7{\pm}4.9$ years old). There were no significant differences in the initial vital signs, GCS (Glasgow Coma Scale), and RTS between the two groups. There were differences in the types and the locations of the crashes, the behaviors of the patients at the times of the accidents, the use of seat belts, and alcohol consumption between the two groups (p<0.05). The average ISS of the geriatric group was higher than that of the adult group ($9.66{\pm}10.11$ vs. $6.59{\pm}8.99$, p=0.004). The mortality was higher in the geriatric group (n=17,9%) than in the adult group (n=23,2%) (p=0.004). Conclusion: The numbers of mortalities and surgical procedures were greater within the elderly group than the adult group. The average ISS was higher in the geriatric group than in the adult group. The severity of injuries due to traffic accidents was higher in the geriatric group than it was in the adult group.

• Journal of Korean Neurosurgical Society
• /
• 제59권5호
• /
• pp.512-517
• /
• 2016

Objective : The pathophysiology of idiopathic Chiari malformation (CM) type 1 is disturbance of free cerebrospinal fluid (CSF) flow and restoration of normal CSF flow is the mainstay of treatment. Additional migration of the medulla oblongata in pediatric patients is referred to as CM type 1.5, but its significance in adult patients is unknown. This study is to compare surgical outcomes of adult idiopathic CM type 1.5 with that of type 1. Methods : Thirty-eight consecutive adult patients (M : F=11 : 27; median, 33.5; range, 18-63) with syringomyelia due to idiopathic CM type 1 were reviewed. Migration of the medulla oblongata was noted in 13 patients. The modified McCormick scale (MMS) was used to evaluate functional status before and one year after surgery. All patients underwent foramen magnum decompression and duroplasty. Factors related to radiological success (${\geq}50%$ decrease in the diameter of the syrinx) were investigated. The follow-up period was $72.7{\pm}55.6$ months. Results : Preoperative functional status were MMS I in 11 patients and MMS II in 14 of CM type 1 and MMS I in 8 and II in 5 of CM type 1.5. Of patients with MMS II, 5/14 patients in group A and 3/5 patients in group B showed improvement and there was no case of deterioration. Radiological success was achieved in 32 (84%) patients and restoration of the cisterna magna (p=0.01; OR, 46.5) was the only significant factor. Conclusion : Migration of the medulla oblongata did not make a difference in the surgical outcome when the cisterna magna was restored.

• Journal of Chest Surgery
• /
• 제28권9호
• /
• pp.811-816
• /
• 1995

Repairs of atrial septal defect utilizing cardiopulmonary bypass were performed in 50 adults, ranging age from 16 to 53 years, since April 1986 up to October 1994. They occupied 38.8% of all adult congenital heart disease operated in the same period. Preoperatively, 16 patients were functional class II, 12 patients class III and 4 patients class IV[New York Heart Association Classification , respectively. Five patients combined with atrial fibrillation and the remainders revealed regular sinus rhythm. Cardiac catheterizations were performed in 43 out of 50 patients, and revealed a systolic pulmonary arterial pressure in excess of 51 mmHg in 4 patients but none had reversed shunt. 40 patients[80% were repaired with patch closure and remainings were repaired with direct closure. 49 patients were followed up for 2 months up to 102 months[average 55 months . A comparison of the preoperative and postoperative functional class demonstrated a mean decrease of one NYHA functional level[2.5$\pm$0.63 to 1.4$\pm$0.56 . There was no operative mortality. One patient died during the follow-up period and the death was unrelated to heart disease. Operative treatment is indicated for repair of atrial septal defect with left to right shunt in the adult patient and a considerable clinical improvement can be anticipitated with low mortality.

• Journal of Chest Surgery
• /
• 제28권8호
• /
• pp.747-753
• /
• 1995

Patients over 14 years of age who have undergone a surgical correction of a congenital cardiac malformations during period of August 1959 through December 1993 have been reviewed. During this period, there were 6894 cases of congenital cardiac lesions consisting of 4576 acyanotic and 2318 cyanotic cases. Among them, a total of 1389 adults [20.1%with various congenital cardiac malformations, 1126 acyanotic group and 263 cyanotic group were operated on. 677 patients were male and 712 patients were female. There were 444 patients under 20 years of age, 365 patients between 20-24 years, 220 patients between 25-29 years, 138 patients between 30-34 years and 222 patients over 34 years. The most common defects were atrial septal defect which accounted for 500 cases [36.0%and another common malformations were ventricular septal defect [276 cases, 19.9% , patent ductus arteriosus [207 cases, 14.9% , tetralogy of Fallot [185 cases, 13.3%and pulmonary stenosis [44 cases, 3.2%in order of incidence. Overall operative mortality for this series was 2.7% [1.5% of acyanotic group and 7.9% of cyanotic groupcompared with 4.6% of operative mortality of total cases of congenital cardiac malformations [2.9% of acyanotic group and 16.7% of cyanotic group . This reviewed series reveals the incidence of operable congenital heart defects appearing in any adult life and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.

• Journal of Korean Neurosurgical Society
• /
• 제54권3호
• /
• pp.207-210
• /
• 2013

Objective : To compare spinopelvic parameters in young adult patients with spondylolysis to those in age-matched patients without spondylolysis and investigate the clinical impact of sagittal spinopelvic parameters in patients with L5 spondylolysis. Methods : From 2009 to 2012, a total of 198 young adult male patients with spondylolysis were identified. Eighty age-matched patients without spondylolysis were also selected. Standing lateral films that included both hip joints were obtained for each subject. Pelvic incidence (PI), sacral slope (SS), pelvic tilt, lumbar lordosis angle, sacral inclination, lumbosacral angle, and sacral table angle were measured in both groups. A comparative study of the spinopelvic parameters of these two groups was performed using SPSS 15.0 (SPSS Inc., Chicago, IL, USA). Results : Among the aforementioned spinopelvic parameters, PI, SS and STA were significantly different between patients with spondylolysis and those without spondylolysis. PI and SS were higher in the spondylolysis group than in the control group, but STA was lower in the spondylolysis group than in the control group. Conclusion : PI and SS were higher in the spondylolysis group than in the control group, but STA was lower in the spondylolysis group than in the control group. Patients with spondylolysis have low STA at birth, which remains constant during growth; a low STA translates into high SS. As a result, PI is also increased in accordance with SS. Therefore, we suggest that STA is an important etiologic factor in young adult patients with L5 spondylolysis.