We found an error in this article. The author's name should be corrected as following: from "Younsuk Koh" to "Younsuck Koh".

BACKGROUND: Critical care physician staffing is a crucial element of the intensive care unit (ICU) organization, and is associated with better outcomes in ICUs. Adult ICUs in Korea have been suffering from inadequate full-time intensivists and nurses because of insufficient reimbursement rates (<50% of the original critical care cost) from the National Health Insurance System. Recently, full-time intensivists have been introduced as a prerequisite for adult ICUs of tertiary hospitals in Korea. The purpose of this study was to examine the perception of intensivist staffing among critical care program directors regarding the barriers and solutions when implementing an intensivist model of critical care in Korea. METHODS: An email survey of critical care program directors in designated teaching hospitals for critical care subspecialty training by the Korean Society of Critical Care Medicine was performed. The survey domains included vision, culture, resources, barriers, and potential solutions to implementing intensivist physician staffing (IPS). RESULTS: Forty-two critical care program directors were surveyed. A total of 28 directors (66.7%) responded to email queries. Of these, 27 directors (96.4%) agreed that IPS would improve the quality of care in the ICU, although half of them reported a negative perception of relevant clinical colleagues for the role of full-time intensivists and poor resources for IPS in their hospitals. Increased financial burden due to hiring full-time intensivists and concerns regarding exclusion from the management of their critically ill patients in the ICU, together with loss of income for primary attending physicians were stated by the respondents to be major barriers to implementing IPS. Financial incentives for the required cost from the health insurance system and enhancement of medical law relevant to critical care were regarded as solutions to these issues. CONCLUSIONS: Critical care program directors believe that intensivist-led critical care can improve the outcome of ICUs. They indicated the financial burden due to IPS and underestimation of a full-time intensivist's role to be major barriers. The program directors agreed that a partnership between hospital leaders and the Ministry of Health and Welfare was needed to overcome these barriers.
Adult* ; Critical Care ; Critical Illness ; Electronic Mail ; Hospitals, Teaching ; Humans ; Insurance, Health ; Intensive Care Units ; Jurisprudence ; Korea ; Motivation ; National Health Programs ; Personnel Staffing and Scheduling ; Surveys and Questionnaires ; Tertiary Care Centers

Ovarian Sertoli-Leydig cell tumors are rare sex cordstromal tumors, and these neoplasms account for less than 0.5% of all ovarian tumors. Those are more often encountered in young women between the ages of 20 and 30 years who usually become virilized. Recently, we experienced an unusual case of Sertoli-Leydig cell tumor with mucinous heterologous elements in a 71-year-old postmenopauseal woman. We present it with brief review of literatures.
Aged ; Cystadenoma, Mucinous ; Female ; Humans ; Mucins* ; Postmenopause ; Sertoli-Leydig Cell Tumor*

Myasthenia gravis is an autoimmune disease that results from an antibody-mediated reaction and occurs with thymoma in 15+ACU- of patients. It is very rarely associated with autoimmune hepatitis. Four cases of myasthenia gravis with autoimmune hepatitis have been reported in the world. We recently experienced a case of 30-year-old man with myasthenia gravis associated with thymoma and autoimmune hepatitis. This condition is the first case that has not been reported previously in Korea. We report this rare condition along with a brief review of the literature.
Adult ; Case Report ; HLA-DR Antigens/genetics ; Hepatitis, Autoimmune/etiology+ACo- ; Human ; Male ; Myasthenia Gravis/complications+ACo- ; Thymoma/etiology+ACo- ; Thymus Neoplasms/etiology+ACo-

OBJECTIVE: To evaluate the incidence, combined anomaly, and prognosis of prenatally diagnosed Single Umbilical Artery (SUA) by ultrasound. METHODS: From January 2001 to December 2005, a single umbilical artery (SUA) was observed in 41 cases out of 22,868 deliveries. Among 41 cases, 39 cases were examined by targeted imaging to rule out fetal anomalies in the mid trimester (intrauterine pregnancy 16-27 weeks). The remaining two cases were detected in the third trimester, which were transferred from a local clinic, and were examined by routine sonogram. Pregnancy and perinatal outcome data were retrieved by review of the medical records. RESULTS: The incidence of SUA in our population was 0.18%. Of 41 fetuses with SUA, 8 cases presented congenital malformations (19.5%) such as acrania (n=1), Tetralogy of Fallot (n=1), renal anomalies (unilateral renal agenesis n=2, pyelectasis n=1), esophageal atresia (EA) with tracheoesophageal fistula (TEF)(n=1), omphalocele with choroid plexus cyst (CPC)(n=1), and congenital diaphragmatic hernia with hypoplastic left heart syndrome (n=1). Of 33 fetuses with isolated SUA, 3 (9.1%) demonstrated growth restriction. Karyotype analysis was performed in three cases. Two were normal and omphalocele with CPC was Trisomy 18. CONCLUSION: During the prenatal period, the fetus with SUA by ultrasound examination must be carefully monitored with targeted prenatal ultrasound because of its frequent association with fetal congenital anomalies (19.5%). Isolated SUA without associated anomaly dose not affect the outcome of the pregnancy.
Choroid Plexus ; Esophageal Atresia ; Female ; Fetus* ; Hernia, Diaphragmatic ; Hernia, Umbilical ; Humans ; Hypoplastic Left Heart Syndrome ; Incidence ; Karyotype ; Medical Records ; Neural Tube Defects ; Pregnancy ; Pregnancy Trimester, Third ; Prognosis ; Pyelectasis ; Single Umbilical Artery* ; Tetralogy of Fallot ; Tracheoesophageal Fistula ; Trisomy ; Ultrasonography

PURPOSE: To evaluate the role of bile duct obstuction in the development of atrophy of the liver, using ananimal model. MATERIALS AND METHODS: Seven rabbits were divided into two groups : group 1(n=5), in which therewas selective bile duct ligation, and group 2(n=2), which underwent a sham operation. Each group was evaluated using CT for changes in hepatic volume after selective bile duct ligation or a sham operation. In group I, the diameter of dilated bile duct was measured 2, 4, 8, 12 and 16 weeks after bile duct ligation, while gross andhistologic change were evaluated in all cases. RESULTS: In group 1, bile duct dilatation was seen on CT two weeks after selective bile duct ligation, and did not change significantly during follow-up. In four of five cases, CT revealed no evidence of significant atrophy of the involved segment. Pathologic specimens, however, revealed dilatation of the bile duct, periductal fibrosis, infiltration of chronic inflammatory cells, and periportalfibrosis. One of five cases showed segmental liver atrophy after selective bile duct ligation. In addion to the above pathologic findings, there was obstruction of the portal vein by foreign body reaction. In group 2, no evidence of dilated bile duct or liver atrophy was revealed by CT or pathologic specimen after a sham operation. CONCLUSION: During long-term follow-up of 16 weeks, obstruction of the bile duct did not play a major role in the development of lobar atrophy in the rabbit.
Animals ; Atrophy ; Bile Ducts* ; Bile* ; Dilatation ; Fibrosis ; Follow-Up Studies ; Foreign-Body Reaction ; Ligation* ; Liver ; Portal Vein ; Rabbits

Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is an autosomal recessive hereditary metabolic disorder of mitochondrial fatty acid beta-oxidation. It is characterized by hypoketotic hypoglycemia, hyperammonemia, seizure, coma, and sudden infant death syndrome-like illness. The most frequently isolated mutation in the acyl-CoA dehydrogenase, medium-chain (ACADM) gene of Caucasian patients with MCADD is c.985A>G, but ethnic variations exist in the frequency of this mutation. Here, we describe 2 Korean pediatric cases of MCADD, which was detected during newborn screening by tandem mass spectrometry and confirmed by molecular analysis. The levels of medium-chain acylcarnitines, including octanoylcarnitine (C8), hexanoylcarnitine (C6), and decanoylcarnitine (C10), were typically elevated. Molecular studies revealed that Patient 1 was a compound heterozygote for c.449_452delCTGA (p.Thr150ArgfsX4) and c.461T>G (p.L154W) mutations, and Patient 2 was a compound heterozygote for c.449_452delCTGA (p.Thr150ArgfsX4) and c.1189T>A (p.Y397N) mutations. We detected asymptomatic patients with MCADD by using a newborn screening test and confirmed it by ACADM mutation analysis. This report presents evidence of the biochemical and molecular features of MCADD in Korean patients and, to the best of our knowledge, this is the first report of the c.461T>G mutation in the ACADM gene.
Acyl-CoA Dehydrogenase/chemistry/deficiency/genetics ; Asian Continental Ancestry Group/*genetics ; Base Sequence ; Biological Markers/blood ; Carnitine/analogs & derivatives/blood ; DNA Mutational Analysis ; Exons ; Female ; Gene Deletion ; Heterozygote ; Humans ; Infant, Newborn ; Lipid Metabolism, Inborn Errors/diagnosis/genetics ; Male ; Mutation ; Neonatal Screening ; Republic of Korea ; Tandem Mass Spectrometry

A case of Cushing's syndrome due to bilateral pigmented nodular adrenal disease in a 35-year-old male is presented. The adrenals showed multiple, black, variable sized nodules. Histologically the cells contained lipofuscin and either had a clear cytoplasm or an eosinophilic cytoplasm with a prominent nucleus. Lymphocytic infiltration and fatty metaplasia within the nodules are two of the prominent histological features. There is extreme internodular atrophy which suggests that primary pigmented nodular adrenocortical disease is a non-adrenocorticotropic hormone dependent condition. Since the disorder appears to involve primarily the cortex of both adrenals, the treatment of choice is bilateral adrenalectomy followed by steroid replacement. The characteristic clinicopathological manifestations that separate this diagnosis from other types of adrenal disease are also discussed. This is the first reported case in Korea to be documented with the pertinent clinicopathological findings.
Adipose Tissue/pathology ; Adrenal Cortex/chemistry/*pathology/radiography/secretion/ultrasonography ; Adrenalectomy ; Adult ; Atrophy ; Case Report ; Cushing Syndrome/*etiology/surgery ; Dexamethasone/diagnostic use ; Furosemide/diagnostic use ; Human ; Hydrocortisone/secretion ; Inflammation ; Lipofuscin/*analysis ; Male ; Metaplasia ; Organelles/ultrastructure

Although intestinal bleeding is known to occur in amyloidosis, it is rare as a presenting symptom or sole manifestation of the disease. We experienced a case of intestinal hemor-rhage in a 64-year old female patient with primary amyloidosis, kappa type. Antral muco-sal erosions were discovered and one shallow healing ulcer at the angle. Colonoscopy revealed multiple purplish nodules in the sigmoid colon and descending colon, as well as a large shallow ulcer with blood clots in the sigmoid colon which was suspected to be the intestinal hemorrhage site. Endoscopic biopsy established amyloidosis. Polarizing microscopy after Congo red staining and immunohistochemical staining identified primary amyloid, kappa type. A case of intestinal hemorrhage in patients with primary amyloidosis is presented and the literature is reviewed.
Amyloid ; Amyloidosis* ; Biopsy ; Colon, Descending ; Colon, Sigmoid ; Colonoscopy ; Congo Red ; Female ; Hemorrhage* ; Humans ; Microscopy ; Middle Aged ; Ulcer

3-methylcrotonyl-CoA carboxylase deficiency is an autosomal recessive disorder characterized by a defect in leucine catabolism. We report the case of an 80-day-old patient with 3-methylcrotonyl-CoA carboxylase deficiency who had elevated levels of 3-hydroxyisovalerylcarnitine (45.56 micromol/L; reference range, <0.65 micromol/L), which was detected using tandem mass spectrometry during newborn screening, and elevated levels of 3-hydroxyisovaleric acid (375.75 mmol/mol Cr) and 3-methylcrotonylglycine (502.36 mmol/mol Cr ), which were detected in urine organic acid analysis. We performed direct sequence analysis of all the exons of the MCCC1 and MCCC2 genes. No mutations were detected in the direct sequence analysis of MCCC1. However sequencing of the MCCC2 gene revealed a mutation caused by a heterozygous G to C transversion [c.313G>C (p.Gly105Arg)] at nucleotide position 313 and a mutation caused by a heterozygous A to T transversion [c.1252A>T (p.lle418Phe)] at nucleotide position 1252. Identification of these 2 novel MCCC2 gene mutations in our patient suggested that analysis of the MCCC1 and MCCC2 genes might prove useful in the diagnosis of 3-methylcrotonyl-CoA carboxylase deficiency.
Carnitine ; Exons ; Glycine ; Humans ; Infant, Newborn ; Leucine ; Mass Screening ; Reference Values ; Sequence Analysis ; Tandem Mass Spectrometry ; Valerates