Background: Current international guidelines recommend against deep sedation as it is associated with worse outcomes in the intensive care unit (ICU). However, in Korea the prevalence of deep sedation and its impact on patients in the ICU are not well known. Methods: From April 2020 to July 2021, a multicenter, prospective, longitudinal, noninterventional cohort study was performed in 20 Korean ICUs. Sedation depth extent was divided into light and deep using a mean Richmond Agitation–Sedation Scale value within the first 48 hours. Propensity score matching was used to balance covariables; the outcomes were compared between the two groups. Results: Overall, 631 patients (418 [66.2%] and 213 [33.8%] in the deep and light sedation groups, respectively) were included. Mortality rates were 14.1% and 8.4% in the deep and light sedation groups (P = 0.039), respectively. Kaplan-Meier estimates showed that time to extubation (P < 0.001), ICU length of stay (P = 0.005), and death P = 0.041) differed between the groups. After adjusting for confounders, early deep sedation was only associated with delayed time to extubation (hazard ratio [HR], 0.66; 95% confidence inter val [CI], 0.55– 0.80; P < 0.001). In the matched cohort, deep sedation remained significantly associated with delayed time to extubation (HR, 0.68; 95% 0.56–0.83; P < 0.001) but was not associated with ICU length of stay (HR, 0.94; 95% CI, 0.79–1.13; P = 0.500) and in-hospital mortality (HR, 1.19; 95% CI, 0.65–2.17; P = 0.582). Conclusion In many Korean ICUs, early deep sedation was highly prevalent in mechanically ventilated patients and was associated with delayed extubation, but not prolonged ICU stay or in-hospital death.

Hereditary hemorrhagic telangiectasia (HHT) is an inherited disorder that is characterized by abnormal communication between the arteries and veins in the skin, mucosa, and various organs. HHT has been reported to show significant phenotypic variability and genetic heterogeneity with wide ethnic and geographic variations. Although mutations in the endoglin (ENG) and activin A receptor type II-like 1 (ACVRL1) genes have been known to cause HHT for more than 10 yr, little is known about the clinical features or genetic background of Korean patients with HHT. In addition, mutations in mothers against decapentaplegic homolog 4 (SMAD4) are also seen in patients with the combined syndrome of juvenile polyposis and HHT. This study examined five Korean patients with the typical manifestations of HHT such as frequent epistaxis and pulmonary arteriovenous malformations. Direct sequencing of the ENG and ACVRL1 genes revealed one known mutation, ENG c.277C>T, in one patient and two novel mutations, ENG c.992-1G>C and ACVRL1 c.81dupT in two patients, respectively. The remaining two patients with negative results were screened for SMAD4 mutations as well as gross deletions of ENG and ACVRL1 using multiple ligation-dependent probe amplification, but none was detected. Despite the small number of patients investigated, we firstly report Korean patients with genetically confirmed HHT, and show the genetic and allelic heterogeneity underlying HHT.
Activin Receptors, Type II/*genetics ; Adult ; Alleles ; Angiography ; Antigens, CD/*genetics ; Asian Continental Ancestry Group/*genetics ; Base Sequence ; Female ; Genetic Predisposition to Disease ; Humans ; Korea ; Male ; Middle Aged ; *Mutation ; Pedigree ; Receptors, Cell Surface/*genetics ; Smad4 Protein/*genetics ; Telangiectasia, Hereditary Hemorrhagic/diagnosis/*genetics/pathology ; Tomography, X-Ray Computed ; Young Adult

Congenital myotonic dystrophy is an autosomal dominantly inherited myotonic dystrophy, rare form, with an incidence estimated to be 13/100,000 liveborns. Affected newborns can present with intrauterine growth retardation, prematurity, birth asphyxia, respiratory distress, and always exhibit generalized muscular hypotonia. Feeding problems are common and an association with protein losing enteropathy, hydrops fetalis, and persistent pulmonary hypertension of the newborn has been described. Twenty-five percent of the affected infants die within the first 18 months of life. The molecular basis is an unstable DNA fragment consisting of a variable expansion of a CTG triplet, Dystrophia myotonica-protein kinase (DMPK) which is localized on chromosome 19q 13.3. The severity of the disease is directly correlated to the length of the CTG sequence. Women with idiopathic polyhydroamnios, decreased fetal movement, prematurity, hypotonia, should be counselled family, and mother, father and baby should be evaluated congenital myotonic dystrophy, as PCR (polymerase chain reaction). It is possible to diagnose congenital myotonic dystrophy, by PCR, antenatal test, such as CVS, amniocentensis. We experienced a case of recurrent congenital myotonic dystrophy, with neonatal death, twice, and report with a review of related literatures.
Asphyxia ; DNA ; Fathers ; Female ; Fetal Growth Retardation ; Fetal Movement ; Humans ; Hydrops Fetalis ; Hypertension, Pulmonary ; Incidence ; Infant ; Infant, Newborn ; Mothers ; Muscle Hypotonia ; Myotonic Dystrophy* ; Parturition ; Phosphotransferases ; Polymerase Chain Reaction ; Protein-Losing Enteropathies ; Triplets

BACKGROUND: Mycobacterium abscessus is the most common respiratory pathogen in rapidly growing mycobacteria and is resistant to all of the first-line antituberculosis drugs. This report describes the clinical and radiographic characteristics in patients with pulmonary disease caused by M. abscessus. MATERIALS AND METHODS: Twelve patients with pulmonary disease caused by M. abscessus who fulfilled the 1997 American Thoracic Society diagnostic criteria for a nontuberculous mycobacterial pulmonary infection were observed over a five-and-a-half year period. The clinical characteristics and chest radiographic findings were analyzed, retrospectively. RESULTS: The patients were predominantly female(11/12, 92%) and nonsmokers(12/12, 100%). Coughing (10/12, 83%), sputum(10/12, 83%) and hemoptysis(10/12, 83%) were the common symptoms and they had prolonged periods from the onset of symptoms to the diagnosis of their disease(median 6.5 years). Eleven (92%) patients had a previous history of being treated for pulmonary tuberculosis. The sputum specimens were acid-fast bacilli smear-positive in all patients. All patients were administered antituberculosis drugs. Six (50%) patients were treated with second-line antituberculosis drugs on account of persistent smear-positive sputum specimens. The chest radiographs showed that reticulonodular opacities(11/12, 92%) were the most common pattern of abnormality, followed by cavitary lesions(5/12, 42%). The computed tomography findings suggested bronchiolitis from the centrilobular nodules with a tree-in-bud appearances(9/10, 90%) and bronchiectasis (9/10, 90%) were the most common, followed by well-defined nodules smaller than 10-mm in diameter(7/10, 70%). CONCLUSIONS: M. abscessus pulmonary disease should be recognized as a cause of chronic mycobacterial lung disease, and respiratory isolates should be assessed carefully.
Bronchiectasis ; Bronchiolitis ; Cough ; Diagnosis ; Humans ; Korea ; Lung Diseases* ; Mycobacterium* ; Nontuberculous Mycobacteria ; Radiography, Thoracic ; Retrospective Studies ; Sputum ; Tuberculosis, Pulmonary

BACKGROUND: Mycobacterium avium complex(MAC) is the most common respiratory pathogen in nontuberculous mycobacterial pulmonary disease. This study described the clinical characteristics of the patients with pulmonary disease caused by MAC. MATERIALS AND METHODS: The clinical characteristics of 24 patients with pulmonary disease caused by the MAC, who fulfilled the 1997 American Thoracic Society diagnostic criteria for nontuberculous mycobacterial pulmonary disease, were retrospectively analyzed. RESULTS: Fourteen patients(58%) were male and the median age at diagnosis was 61 years(range 46-75). Of the 24 patients, 16(67%) had a M. intracellulare infection, 7(29%) had a M. avium infection and one patient was not identified. Coughing(92%) and sputum(88%) were most frequently observed symptoms. The sputum smear for acid-fast bacilli was positive in 17(71%) patients. Fourteen(58%) patients had the upper lobe cavitary form and 10(42%) patients had the nodular bronchiectatic form. In a comparison between the patients with the upper lobe cavitary form and those with the nodular bronchiectatic form, significant differences were found according to sex(male 86% vs. 20%, p=0.003), smoking history(79% vs. 10%, p=0.008), the presence of an underlying disease(64% vs. 20%, p=0.036), the pulmonary function(% forced vital capacity, median 71% vs. 88%, p=0.022; % forced expiratory volume in one second, median 69% vs. 89%, p=0.051) and bilateral disease at chest radiography(29% vs. 90%, p=0.005). The time from the onset of symptoms to diagnosis was longer in those with the nodular bronchiectatic form(median 72 months, range 8-132) than those with the upper lobe cavitary form(median 22 months, range 6-60) CONCLUSIONS: MAC pulmonary disease occurs in two distinct populations with two distinct clinical presentations. For a correct diagnosis of MAC pulmonary disease, knowledge of the diverse clinical and radiological findings is essential.
Diagnosis ; Forced Expiratory Volume ; Humans ; Korea ; Lung Diseases ; Male ; Mycobacterium avium Complex* ; Mycobacterium avium* ; Mycobacterium* ; Nontuberculous Mycobacteria ; Retrospective Studies ; Smoke ; Smoking ; Sputum ; Thorax ; Vital Capacity

BACKGROUND/AIMS: Despite of increasing numbers of reports on intraductal papillary mucinous tumor (IPMT), there is still difficulty in its' diagnosis, treatment and prediction of prognosis. The purpose of this multicenter study was to evaluate the clinico-pathological features of IPMT in Korea and suggest the prediction criteria of malignancy in IPMT. METHODS: We retrospectively reviewed the clinico-pathological data of 208 patients who underwent operations with IPMT between 1993 and 2002 at 28 institutes in Korea. RESULTS: Of the 208 patients with a mean age of 60.5+/-9.7 years, 147 were men and 61 were women. 124 patients underwent pancreatoduodenectomy, 42 distal pancreatectomy, 17 total pancreatectomy, 25 limited pancreas resection. Benign cases were 128 (adenoma (n=62), borderline (n=66)) and malignant cases were 80 (non-invasive (n=29), invasive (n=51)). A significant difference in 5-year survival was observed between benign and malignant group (92.6% vs. 65.3%; p=0.006). Of the 6 factors (age, location, duct dilatation, tumor appearance, main duct type, and tumor size) that showed the statistical difference in univariate analysis between benign and malignant group, we found three significant factors (tumor appearance (p=0.009), tumor size (p=0.023), and dilated duct size (p=0.010)) by multivariate analysis. CONCLUSION: Although overall prognosis of IPMT is superior to ordinary pancreatic cancer, more curative surgery is recommended in malignant IPMT. Tumor appearance (papillary), tumor size (> or =30 mm) and dilated duct size (> or = 12 mm) can be used as preoperative indicators of malig-nancy in IPMT.
Academies and Institutes ; Diagnosis ; Dilatation ; Female ; Humans ; Korea* ; Male ; Mucins* ; Multivariate Analysis ; Pancreas* ; Pancreatectomy ; Pancreatic Neoplasms ; Pancreaticoduodenectomy ; Prognosis ; Retrospective Studies

BACKGROUND AND OBJECTIVES: An array of materials have been used for rhinoplasty, however, the fate of these materials after rhinoplasty is not all clear. This study was performed to find post-operative gross and microscopic changes of the implants and grafts (cartilage, Gore-Tex , and Alloderm), and to guide selection of implants and graft. MATERIALS AND METHODS: We took the implants and grafts out from nine patients who underwent revision rhinoplasty for cosmetic problems, and studied gross and microscopic charaterizations. RESULTS: Autologous cartilages showed minor volume reduction and remodeling in gross examination, and mild degeneration, peripheral fibrosis, and fibroblast in-growth without inflammation under microscopic examination. Gore-Tex , which was a little difficulty to remove, had good volume preservation with focal in-growth of fibroblasts and inflammatory cells. A gross examination of Alloderm showed preserved volume, but thin, soft and weak appearance. A microscopic examination of Alloderm showed well preserved collagen materials without inflammation. CONCLUSIONS: Cartilages have good biocompatibility and stabilization, but it is necessary to study absorption and remodeling. We found that Gore-Tex is a good volume filler for dorsal augmentation, whereas Alloderm is a good material for camouflage.
Absorption ; Cartilage ; Collagen ; Fibroblasts ; Fibrosis ; Humans ; Inflammation ; Naphazoline ; Polytetrafluoroethylene ; Rhinoplasty* ; Transplants*

Incarceration of the gravid uterus is a rare but serious complication of pregnancy. Reported is the case of a gravid 2, para 0, abortus 1 with known uterine subserosal myoma(5.3 x5.5cm sized) 26-year-old woman presented with acute dysuria and urinary retention. The patient was 14 weeks and 3 days pregnant and presented several week history of urinary frequency and sensation of incomplete bladder emptying. Examination revealed a retroflexed uterus with cervical opening pointing toward the anterior abdominal wall. An ultrasound revealed a thin, elongated maternal bladder and a uterus incarcerated between the sacral promontary and the pubis. The incarceration was successfully reduced by tenaculum traction of the cervical posterior lip without surgical intervention and had a normal infant of appropriate weight at term.
Abdominal Wall ; Adult ; Dysuria ; Female ; Humans ; Infant ; Leiomyoma ; Lip ; Myoma* ; Pregnancy ; Sensation ; Traction ; Ultrasonography ; Urinary Bladder ; Urinary Retention ; Uterine Retroversion ; Uterus*

Krukenberg tumor of the primary breast carcinoma is rare and this is almost invasive lobular carcinoma. They are usually bilateral and frequent in premenopausal women. Diagnosis of symptomatic Krukenberg tumor is reported to be 1 or 2 year after the diagnosis of primary neoplasm. But sometimes it is discovered prior to the detection of the primary breast carcinoma. Unexpected ovarian micrometastasis was recognized after oophorectomy of normally appearing ovaries in breast cancer patients. Existence of Krukenberg tumor means advanced primary disease and possible metastasis to other organs, and have a poorer prognosis. We report a case of Krukenberg tumors occurred 3 years after the diagnosis of primary ductal type breast carcinoma.
Breast Neoplasms* ; Breast* ; Carcinoma, Lobular ; Diagnosis ; Female ; Humans ; Krukenberg Tumor* ; Neoplasm Metastasis* ; Neoplasm Micrometastasis ; Ovariectomy ; Ovary* ; Prognosis

BACKGROUND AND OBJECTIVES: Malignant lymphoma is the most common type of cancer in the immunologic system. There have been many reports about malignant lymphoma, but etiologies and prognosis of malignant lymphoma remain controversial. This study was designed to carry out an immunohistochemical classification of malignant lymphoma and to define the relationship between Epstein-Barr virus (EBV) and malignant lymphoma by using the polymerase chain reaction (PCR), which is known to be more sensitive than than any other methods for detection of EBV. MATERIALS AND METHODS: Thirty six cases of cervical nodal non-Hodgkin's lymphoma tissue and twenty five cases of normal neck node tissue were used. RESULTS: We observed 72.7% (26 cases/36 cases) B-cell lineage expression and 27.8% (10 cases/36 cases) T-cell lineage expression in the cervical nodal of non-Hodgkin's lymphoma. The detection rate of EBV in the malignant lymphoma tissue was 27.8% (10 cases/36 cases) and 7.1% (2 cases/36 cases) in the normal neck node tissue. Of the B-cell lineage expression, EBV was detected in 6 cases (23.1%) and 4 cases (40.0%) in T-cell league expression. There was no statistical significance between the malignant lymphoma group and the normal group in the detection rate of EBV. However, the detection rate of EBV of the T-cell malignant lymphoma group was significantly higher compared with the normal group (p<0.05). CONCLUSION: These results suggest that the T-cell expression group of cervical nodal non-Hodgkin's lymphoma may be related to EBV.
B-Lymphocytes ; Classification* ; Herpesvirus 4, Human* ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Neck ; Polymerase Chain Reaction ; Prognosis ; T-Lymphocytes